Pediatric Conditions Flashcards

Question

Acute Laryngitis

Answer 1

* common, self limiting infection of the vocal cords (usually lasts \<3 weeks) * \*\*common bugs: **S. pneumoniae, HIB, Moraxella catarrhalis**\*\* * associated with previous URI and vocal strain * \*\*hoarseness * Viral: supportive care * Bacterial: **erythromycin, ceftriaxone, Augmentin**

Answer 2

* aka Croup * \*\*barking cough most commonly caused by **parainfluenzae virus**\*\* * abrupt onset of symptoms * \*\***Steeple Sign\*\*** * home treatment: symptomatic care maybe with some **dexamethasone** * **Nebulized epi with IV/oral/IM dexamethasone** * \*\*the WESLEY CROUP SCORE\*\* \>12 → send to the hospital * mild = 2 * Moderate 3-7 * severe \>/= 8 * impending respiratory failure \>/=12

Answer 3

* \*\*most common bugs: **adenovirus, coronavirus, rhinovirus** \*\* * coryza (inflammation of mucus membrane of nose), *cough*, and hoarseness *

Answer 4

* aka strep throat/ Scarlatina * EXUDATE, fever, rarely cough, scarlatina form rash (\*\*sandpaper rash\*\*), and STRAWBERRY TONGUE * tx: **Penicillin** is first choice but kids may not like it →**Amoxicillin ⇒ Augmentin** if you suspect resistance * alernative abx: **Cephalosporins, macrolides, clindamycin**

Answer 5

* caused by Epstein Barr Virus \*\*dsDNA (HHSV-4)\*\* infects B cells of the lymphoid tissues * fever, fatigue, pharyngitis, acute rupture of spleen due to trauma as after splenomegaly * higher lympocyte than neutrophil count → indicative of viral infection * Liver function test: higher number of aminotransferases ⇒ indicates that its mono not strep * diagnosis: **heteroantibody test** is gold standard * tx: supportive, maybe corticosteroids for throat and tonsillar swelling * **can return to sports 4 weeks after onset of symptoms** * ******Cold agglutination**

Answer 6

* acute = \< 4 weeks * subacute = \>4 weeks but \< 3 months * chronic = \>3months (12 weeks)

Answer 7

* lasts longer than 12 weeks/3 months \*\*\* * common bacteria: **pseudomonas, klebsiella pneumonia, enterobacter spp. e.coli, and S. aureus** * 4 cardinal signs: 1. mucopurulent drainage of the anterior or posterior portion of the nose 2. nasal obstruction/ congestion/blockage 3. facial/sinus pain, pressure, or fullness 4. loss of sense of smell or reduced sense of smell (anosmia or hyposmia ) * tx: single agent: **Augmentin** * double agent: * **metronidazole** plus cefdinir or bactrim 1. CRS with nasal polyposis (with nasal polyps) 2. Allergic fungal rhinosinusitis (more common in DM patients) 3. CRS without nasal polyposis (without nasal polyps)

Answer 8

ASA - aspirin

Answer 9

* **Acne Vulgaris** * **caused by Cutibacterium acnes** * Mild: comedones, small amounts of papules or pustules * tx: azelauc acid, salicylic acid, benzoyl peroxide, retinoids or topical abx * Moderate: comedones, large amounts of papules or pustules * tx: as above + oral antibiotics (minocycline or doxycyline) * Sever: nodular \>5mm or cystic acne * tx: Oral Isotretinoin

Answer 10

Acne Rosacea * acne-like rahs + central facial erythema, facial flushing, telangiectasias * \*\*NO COMEDONES\*\* * Rhinophyma (red enlarged nose with edema) * women age 30-50 = most common * tx: * **sunscreen (not chemical), avoid irritants** * mild/moderate: * topical **metronidazole** for papulopustules, azelaic acid, **ivermectin** for **demodex mite**) * Severe: * oral abx (tetracycline, doxycyline, minocycline) * Oral Isotretinoin can be used for refractory cases

Answer 11

Perioral dermatitis * monomorphic pink papules * hx of topical corticosteroid use * tingling/burning sensation * no comedones * tx: * clindamycin, sodium sulfacetamide (excellent for kids) * **oral abx**: doxycyline, azithromycin * in children: **amoxicillin** or azithromycin if PCN allergy

Answer 12

Hot tub folliculitis * caused by pseudomonas: * tx: abx soap, CLN, BPO and fix hot tub chlorine levels * Immune compromised? **fluoroquinolones**

Answer 13

folliculitis * inflammation/infection of air follicile * common cause: MRSA * tx: doxycycline or cephalexin * CLN wash (bleach wash) * topical mupirocen ointment

Answer 14

hydradentitis supparativa * \*\*double comedones\*\* * favors female \> males, smoking and obesity = risk factor * targets hair follicle and apocrine glands found in skin folds * tx: weight reduction * reduce friction and moisture * stop smoking * **topical clindamycin, intralesional injections of triamcinolone** * systemic: **tetracyclines, clindamycin, rifampin**

Answer 15

Paronychia * Most commonly caused by staph or strep * if recurrent consider HSV * acute tx: * cefalexin (Keflex) * doxycyline * Chronic: * avoid water and chemicals * topical steroids * topical antifungals

Answer 16

* cornification disorder * keratotic follicular papules * tend to affect upper arms, thighs, and lateral cheeks * tx: * hydrate skin with gentle cleansers and moisturizers * keratolytic agents * salicyclic acid, or retinoids consistently for **months**

Answer 17

* family hx of thryoid disease, DM, and vitiligo have increased risk of developing of vitiligo * Multifactorial cause: * 1. autoimmune * 2. self-destruction of melanocytes * 3. neurogenic: nerve ending that secrete a neurochemical mediatory that is **cytotoxic to melanocytes** (segmental vitiligao) * 4. oxidative stress, melanocyte separate from basement membrane * Generalized vitiligo (most common) vs localized vitiligo * **localized:** dermatomal pattern, rarely spread beyond dermatome * more common in children

Answer 18

**Ichthyosis vulgaris** * **Filaggrin** deficiency = impaired formation of cornified keratinocytes * increases your loss of epidermal water * **much more likely to have inflammatory rxn** when exposed to irritants or allergens * diagnosis: * clinical diagnosis * tx: * **Emollients or keratolytic agents**

Answer 19

**Keratosis pilaris** * keratotic follicular papules may have som emild erythema * **thigsh, lateral cheeks, upper arms** * diagnosis: * clinical * tx: * thick moisturizers (keep skin hydrated) * keratolytic agents: (must be used for months) * salicylic acid * retinoids * adapalene

Answer 20

**Pityriasis Alba** * \*\*commonly associated with children/adolescents, especially if they have atopic dermatitis\*\* * hypopigmented macules and patches with **subtle fine scales** * located on face\> shoulders/arms * diagnosis: * clinical * tx: * treat the atopic dermatitis * sunscreen * emollients * low grade topical steroids can help

Answer 21

Cellulitis * GAS and S. aureus = adults * HIB = in children \<3 yo * 4 factors: * 1. erythema/hyperpigmentation * 2. warmth * 3. Edema * 4. pain * Diagnosis in immunocompromised host: * skin cx * aspirate/blood cx * **mild leukocytosis with left shift** and a mild **increased sed rate** * ****tx: **empiric** * **cephalexin** (keflex) * non-betalactam * **clindamycin** * **tetracycline** * elevate lower legs * cold compresses for pain * **send to hospital if at risk for systemic disease**

Answer 22

Erysipelas * \*\*STREAKING\*\*, does not involve the deeper layer (subcutaneous tissue) * caused by GAS and s.aureus in adults, HIB in children \<3 yo * 4 factors: * 1.erythema * 2.warm * 3. pain * 4.edema * Diagnosis: * in immunocompromised host: * skin cx, aspirate/blood cx * **Mild leukocytosis** with a **left shift**, and a mild **increased sed rate** * tx: treat empirically * **cephalexin (keflex)** * non-beta-lactams * **clindamycin or tetracycline** * ****elevate legs and cold compresses * send to hospital if at risk for systemic infection

Answer 23

Impetigo * \*\*caused by Staph\*\* * erythema and Honey crusted * diagnosis: * gram stain and cx * tx: * acute: **mupirocin ointment** 2-3x/day for 10-14 days * oral abx: * **cephalexin** if concern for MRSA: doxycyline, **clindamycin** or BActrim

Answer 24

bullous impetigo * Caused by S.aureus * favor trunk, can have fever and diarrhea * tx: **mupirocen** 2-3x/day for 10-14 days * oral: * **cephalexin** * if concern for MRSA: **doxycyline, clindamycin,** or bactrim * RECURRENT: * **mupirocen BID for 2-3 weeks** * wash the area with CLN cleansers or dilute bleach baths ( 1/2 cup in a full bath)

Answer 25

Erythrasma * \*\*causative agent is **Corynebacterium minutissimum**\*\* (gram positive bacilli) * reddish, brown patch that is **uniformly scaly** * non-inflammatory border and **no advancing border** * not very pruritic * Diagnosis: * **Wood's lamp: coral-red fluorescence** * want to do KOH to r/o fungal * tx: * decrease moisture * topical **clindamycin or erythromycin**

Answer 26

Candidiasis * satellite pustules * \*\*affects the scrotum\*\* * erythematous patch * \*\*causative agent **candida albicans**\*\* * Diagnosis: * KOH wet prep * Tx: * **ketoconazole 2% cream**, **fluconazole** daily for 2-4 weeks * Mucosal: * **clotrimazole trouch 5x/day** * **Nystatin swish and swallow** * oral **fluconazole**

Answer 27

Tinea Corporis * \*\*causative agent: **Trichophyton rubrum and Trichophyton mentagrophytes**\*\* * Annular, scaly borders, advancing, erythematous * Diagnosis: * KOH wet prep * Fungal Cx * tx: * **Ketoconazole** 2% cream (imidazoles) or **Terbinafine** 1% cream (allyamine) * oral: * **fluconazole** * **terbinafine** (for tinea unguium 250 mg QID for 6-12 weeks vs once weekly for 52 weeks)

Answer 28

Tinea Capitis \*\*causative agent: Trichophyton rubrum and Trichophyton mentagrophytes\*\* * **Tinea capitis**: requires oral tx with **griseofulvin** plus topical medicated shampoo (**ketoconazole 2% shampoo**) * Diagnosis: * KOH wet prep * Fungal Cx * tx: * Ketoconazole 2% cream (imidazoles) or Terbinafine 1% cream (allyamine) * oral: * fluconazole * terbinafine (for tinea unguium 250 mg QID for 6-12 weeks vs once weekly for 52 weeks)

Answer 29

**Lice** * caused by \*\***Pediculus humanus capitis**\*\* * erythema, scaling, excoriations, lymphadenopathy * **High yield locations**: above the ears and lower occipital scalp * Diagnosis: * clinical * Tx: * **Permethrin shampoo** = first line * **Malathion** = second line * **Ivermectin** ⇒ DO NOT GIVE TO CHILDREN \<15kg * **7 days** in bag or washed in **HOT water**

Answer 30

**Scabies** * caused by **Sarcoptes scabiei hominis** * mite lives in the stratum corneum * **severe pruritus worse at night/ in shower** * Diagnosis: * delta-wingjet sign * Biopsy: but difficult to capture the organism * **Mineral Oil Prep**: very time consuming * Tx: * **Permethrin 5%** applied before bed twice, separated by one week * **10 days** of storage for clothing/bedding or washed in **HOT water** * pt ed: * pruritus and lesions will last 2-4 weeks after tx

Answer 31

**Herpes Simplex Virus** * dsDNA virus * small round vesicle on erythematous base * PAINFUL, burning **grouping of vesicles** * may become umbilicated or pustular * then experience erosion/ulceration and crust **with scalloped border****** * diagnosis: * Viral cx :but sensitivity = 50% * **PCR**: higher sensitivity than cx * **Tzank smear** = multinucleated giant cells * tx: * **valacyclovir** * **acyclovir** * protection from sun * consider prophylaxis

Answer 32

**Verrucae planae** * **flat warts, made worse by shaving** * caused by HPV (dsDNA) * diagnosis: * clinical * serologies * tx: 80% will resolve on their own * salicylic acid * cryotherapy * electro cauterizatiion/surgery * prevention: * HPV vaccine

Answer 33

**Molluscum contagiosum** * caused by \*\***DNA poxvirus\*\*** * spread via skin2skin contact * tends to appear near skin folds, lateral trunk, thighs, buttocks, genitals and FACE * diagnosis: * clinical diagnosis * serologies * tx: * should spontanously resolve in immunocompetent patients * tx underlying eczema * encourage daily skin hydration * **cantharidin** = creates more inflammation which triggers an immune response * can do curettage (VERY PAINFUL) * cryotherapy * retinoid

Answer 34

**Fifth's Disease** * \*\*caused by **Parvovirus B19**\*\* ssDNA aka slap cheek * **erythema infectiosum** * **Bright red, macular erythema on cheeks** 7-10 days after prodromal sxs * **lacy, reticulated pattern of erythematous macules** favors **extremities \> trunk** * diagnosis: * clinical * tx: * supportive care * complications: * **hydrops faecalis** * **arthralgias of the small joints of the hands**

Answer 35

**Hand, Foot and Mouth Disease** * caused by \*\***coxsackievirus A16**\*\* * ssRNA virus * **Enterovirus A71**: much more severe, can be fatal * msot common in **summer and fall** * **onychomadesis**: * fingernails/toenails fall off **1-2 months** later (especially with **coxsackie virus A16**) * diagnosis: * clinical * tx: * resolves within 1-2 weeks * complications: * **otitis media** * **pneumonia** * **encephalitis**

Answer 36

**Measles** * aka rubeola caused by **RNA paramyxovirus** (ssRNA) * Prodromal sxs: * **cough, coryza, conjunctivitis** * fever * **Koplik spots** * **exanthem that spreads cephalocaudally** * **Morbillaform rash** * diagnosis: * clinical * serologies * Tx: * supportive

Answer 37

Acquired or Congenital Nevi * acquired: **benign** pigmented or non-pigmented lesions that come from **melanocytes** * age 3-5 * **Larger congential melanocytic nevus** may be removed to reduce risk of malignant melanoma * **may undergo transition to malignant melanomas**

Answer 38

**Infantile Hemangioma** * aka superficial hemangiomas or strawberry hemangiomas * **femals \> males** * proliferation of mast cells that may **promote angiogenesis** * bright red papules and plaques with smal capillary projections * **usually only one lesion present on head, neck or trunk** * will begin to disappear 12-16 months of age * most will completely disappear by 5-9 years

Answer 39

**Arcochordon** * "skin tag" * **small, soft, common, benign pedunculated neoplasm** * often found in **obese** patients * skin colored or hyperpigmented * surface nodules or peduncules, or papillomas

Answer 40

* well-**circumscribed** area of **raised erythema and edema of the superificial dermis** * associated with **type I hypersensitivity rxns** * \*\***chronic urticaria \> 6 weeks**\*\* * _Angioedema_ = affects of the mucosal tissue of the face, lips, tongue, larynx, hands, feet, and genitalia * diagnosis: clinical, workup if unknown cause * tx: avoid food & medication triggers * **antihistamines**: **cetirizine** * **steroids for severe cases** * **epipen for SEVERE cases** * **omalizubmab for** _autoimmune etiology_ = inhibits activation of mast cells

Answer 41

**type IV hypersensitivity rxn** (delayed) most often caused by **HSV and then mycoplasma spp** * **target lesions** with 3 components: **dusky, central area or blister**, dark red inflammatory zone surrounded by a **pale ring of edema** and an **erythematous halo on the extreme periphery** of the lesion * **NO EPIDERMAL DETACHMENT** (Negative Nikolsky sign) * \*\*most common on extremities and trunk\*\* * Minor = no mucosal involvement, Major = mucosal involvement * diagnosis: clinical diagnosis/ **biopsy if diagnosis is not clear** * tx: discontinue the drug, antihistamines, analgesics and skin care * for Oral lesions: **corticosteroids + lidocain +diphenhydramine mouthwash** * if severe: **systemic corticosteroids**

Answer 42

\<10% BSA / TEN = \>30% BSA * **severe mucocutaneous rxn** * \*\* medications = most common cause\*\* **sulfa drugs, anticonvulsants,** and **lamotrigine, allopurinol, NSAIDs, antipsychotics, and abx** * Prodromal fever and URI sxs * **widespread flaccid bullae** beginning on the **trunk and face** * itchy **target lesions** with **purpuric centers** * **mucous involvement with NIKOLSKY SIGN**

Answer 43

associated with Lymes disease * T-cell mediated response * bull's-eye rash associated with myalgias and arthralgias * diagnosis: clinical diagnosis * tx: **lyme-disease abx: amoxicillin**

Answer 44

**IgE mediated type I hypersensitivity rxn** * \*\***_Filaggrin deficiency_**\*\* * _TH2 cell involvement_ : type of T-helper cell * **pruritus** * scaling, **non-demarcated, FLEXOR Creases** * **tx: topical steroid _ointment_** * long-term tx: topical **calcineurin inhibitors** * phototherapy narrow range UVB * **Dupilumab** = inhibits cytokines, $$$ but works very well

Answer 45

**Dyshidrotic Eczema** * most common onset is \<40 years * triggers: sweating, emotional stress, warm and humid weather, metals * \*\* sudden onset of pruritic clear, **tapioca-like** tense vesicles on the soles, palms, & fingers (the lateral digits) \*\* * Diagnosis: clinical * tx: **topical corticosteroid ointments preferred** * will usually resolve spontaneously * oral corticosteroids for severe cases

Answer 46

**Nummular Eczema** * **IgE mediated type I hypersensitivity rxn** * **Filaggrin gene mutation** * sharply defined **coin-shaped lesions** * (especially on the dorsal surfaces of the **hands, feet, and extensor surfaces**) * Diagnosis: clinical * tx: **topical corticosteroids** * **antihistamines for itching** * topical _calcineurin inhibitors_

Answer 47

**Seborrheic dermatitis** * caused by increased **sebaceous gland activity + hypersensitivity** rxn to **Malassezia furfur** * **greasy appearance of erythematous plaques with fine white scales** * tx: **SELENIUM SULFIDE** * **sodium sulfacetamide, zinc pyrithione** * **ketoconazole shampoo**

Answer 48

* small, erythematous "tear-drop" papules with fine scales, discrete lesions and confluent plaques * often appear after a **strep pharyngitis infx** * tx: \<3% = topical corticosteroids * 5-10% = phototherapy + refer to derm * \>10% = phototherapy + consider systemic tx + refer to derm * methotrexate, retinoids, & biologic * With Joint involvement: **Methotrexate** + refer to derm or rheum

Answer 49

**Pityriasis rosea** * associated with viral infections (HHSV 6 or 7) * **usually seen in older children and young adults** * will often start with a **herald patch** (single, salmon-colored macule) on the trunk followed by a general exanthem 1-2 weeks later * **smaller, very pruritic round or oval salmon-colored papules** * **CHRISTMAS TREE PATTERN** * tends to affect trunk and proximal extremities * tx: self resolving in 6-12 weeks * for pruritus: **antihistamines, topical corticosteroids or oatmeal baths**

Answer 50

bulging of the eyeball, anterior protrusion from orbit

Answer 51

Recession of the eyeball, posterior displacement within the orbit

Answer 52

* incorrect development of the eye's drainage system before birth * sxs: enlarged eyes, cloudiness of the cornea, and photosensitvity **autosomal recessive inheritance**

Answer 53

* clouding of the lens of the eye * part of many birth defects * Most important: * non-dysjunctions * Down syndrome (trisomy 21) * Trisomy 13

Answer 54

* autosomal dominant and autosomal recessive inheritance

Answer 55

misalignment of one or both eyes * stable ocular alignment not usually reached until ag 2-3mo, still persisting at 4-6 months? refer * types: hypertropia (upward), hypotropia (downward), esotropia (inward), exotropia (outward) * _dx_: cover-uncover test * _tx:_ **patch the normal eye**, eyeglasses, corrective surgery if severe

Answer 56

decreased visual acuity of one eye due to disuse during visual development * \*\*needs to be **treated before age 8** if you want to avoid SEVERE vision loss\*\* * _risk factors_: strabismus, refractive errors (astigmatism, myopia, hyperopia), congenital cataract * _s/sxs_: decreased visual acuity * _diagnosis_: early screening * _tx_: eyeglasses, patch the normal eye, cataract removal, tx of strabismus, atropine drops

Answer 57

Hyphema blood in the anterior chamber of the eye * referral, eye shield

Answer 58

Hypopyon collection of neutrophils and fibrin in the anterior chamber of the eye * often associated with **endophthalmitis** * referall to surgery and intravitreal abx

Answer 59

* _preseptal orbital cellulitis_ = outside of the orbit * _postseptal orbital cellulitis_ = inside the orbit (especially from sinus infection from the **ethmoid sinus** * _s/sxs_: * preseptal and postseptal = tenderness, edema, erythema, discoloration of eyelid and fever * postseptal = **decreased ocular mobility**, pain with eye movement, **proptosis**, and decreased visual acuity * _dx_: clinical, **high resolution CT scan** * _tx_: admission + IV abx * **vancomycin + ceftriaxone** or **cefotaxime** (3rd gen) * preseptal = outpatient if older than **_1 year_** oral **clindamycin** (to cover MRSA)

Answer 60

aka stye = abscess of the meibomian gland, gland of Moll, or Gland of Zeiss) * *staph aureus* * _s/sxs_: pain, warm, erythematous, nodule or pustule on eyelid * _diagnosis_: clinical * _tx:_ warm compresses, can use oral abx if accompanied by preseptal orbital cellulitis (**dicloxacillin** or **erythromycin**) * can add topical abx if actively draining (**erythromycin or bacitracin**)

Answer 61

non-infectious blockage of internal Meibomian sebaceous gland, or zeiss gland * often associated with acne rosacea * _s/sxs_: **non-tender localized eyelid swelling** on the conjunctival surface of the eyelid * often larger, firmer, slower growing, and less painful than hordeolum * diagnosis is clinical * _tx:_ warm compresses, abx eye drops * referral to optho for injection of glucocorticoid or incision + curettage if does not resolve

Answer 62

infection of the eyelids * _acute ulcerative_: *staph or HSV* * _acute non-ulcerative_: allergic * _chronic_: meibomian gland dysfunction or seborrheic dermatitis * _s/sxs_: **pruritus or burning of eyelid margin**, conjunctival irritation and tearing, photosensitivity, sensation of foreign body * _dx_: slit lamp * _tx:_ supportive * warm compresses, cleansing of eyelid, keratoconjuctivitis sicca tx PRN * can use abx for acute ulcerative and chronic or topical corticostosteroid of nonulcerative

Answer 63

infection of the lacrimal sac * often caused by *staph aureus and strep* * _s/sxs_: pain, erythema, edema around lacrimal sac * pressure on lacrimal sac may **exude mucoid or purulent material through the puncta** * diagnosis is clinical * _tx_: mild = warm compresses and **1st gen ceph,** or **penicillinase-resistant penicillin** (PO) * IV abx * not responding to tx? consider MRSA

Answer 64

abx ointment (**aminoglycoside** tobramycin, gentamicin), or **erythromycin** and pupillary dilation plus the **tetanus immunization**!!!

Answer 65

benign growth of the conjunctiva that results from chronic actinic irritation * more common in sunny, dry, hot climates * _s/sxs_: **fleshy, trianngular growth** of the _bulbar conjunctiva_ that can spread across the cornea and induce astigmatism/affect vision * **pinguecula** doe NOT affect the cornea * diagnosis is clinical * _tx:_ arifical tears or short course of topical corticosteroids, reassurance * referral if starting to affect vision for surgery

Answer 66

* aka post-infectious cough * no specific etiological agent * cough lasting from 3-8 weeks following a viral URI or bronchitis * usually normal CXR

Answer 67

* cough lasting \> 8 weeks in adults * cough lasting \>4 weeks in children * can be sign of underlying condition: * GERD * ACE inhibitor use (lisinopril) * asthma * upper airway cough sundrome * sarcoidosis, TB, cancer * CXR to r/o other causes * if cause can't be identified: * consider chest CT scan * pft * refer to pulmonologist

Answer 68

Most commonly caused by **RSV** in fall and winter * common in infants and children * S/sxs: **wheezing**, tachypnea, respiratory distress, fever * often have _prodromal viral sxs_ (fever, uri) for 1-2 days **followed by respiratory distress** * PE: expiratory wheezes, may have normal serous nasal discharge * Diagnosis: CXR = normal * test for influenza, **RSV** (antigen test or nasal washing monocloncal antibody test) * Tx: Supportive tx → humidifed air, antipyretics, **beta-agonists**, nebulized **racemic epi** * **_oxygen = mainstay of tx_** * Palivizumab prophylaxis (Synagis) for immunocompromised, premature infants etc

Answer 69

**cough \> 5 days**, can last 1-3 weeks most often _viral (95%)_, but bacterial = Moraxella, S. pneumo, chlamydia pneumoniae * S/sxs: cough \>5 days, low fever, malaise, dyspnea, **URI sxs** * may have **hemoptysis** (most common cause of hemoptysis, followed by carcinoma) * PE: less severe than PNA (normal vitals) **no crackles or egophany** * may have **rhonchi or wheezing** * → _rhonchi that clears with cough_ * Dx: clinical, can obtain CXR if uncertain * Tx: supportive → fluids, rest, **corticosteroids** if underlying RAD * **Dextromethrophan (Tessalon Pearls)** * **Guaifenesin (robitussin)** * SABAs for wheezing * antipyrettics * **Ribavirin** if severe lung or heart disease * if O₂\<96% on RA→ hospitalize

Answer 70

Medical Emergency → usually caused by Hflu Males\> females, DM =risk factor in adults; most common in age 3mo-6yr * S/sxs: fever, odynophagia (pain with swallowing), _Tripoding_ , dyspnea * 3Ds: **drooling, dysphagia** (difficulty swallowing), Respiratory **distress** * PE: **_inspiratory stridor**_, muffled hot-potato voice, hoarseness, _**Thumb print sign_** * Diagnosis: **laryngoscopy** * tx: secure airway then cx for Hflu * intubate if necessary, supportive care * **ceftriaxone** (or 2nd or 3rd gen cephs)

Answer 71

* aka Croup * \*\*barking cough most commonly caused by **parainfluenzae virus**\*\* * abrupt onset of symptoms * \*\***Steeple Sign\*\*** * home treatment: symptomatic care maybe with some **dexamethasone** * **Nebulized epi with IV/oral/IM dexamethasone** * \*\*the WESLEY CROUP SCORE\*\* \>12 → send to the hospital * mild = 2 * Moderate 3-7 * severe \>/= 8 * impending respiratory failure \>/=12

Answer 72

sxs \< 2x/month * Controller: * TAke ICS whenever SABA is taken * Reliever: * SABA

Answer 73

short acting beta agonist

Answer 74

sxs 2x/month + but less than 4-5days/week * Controller: * low dose maintenance ICS * Reliever: * PRN SABA

Answer 75

inhaled corticosteroid

Answer 76

sxs most days, or waking with asthma once/week + * Controller: * low dose maintenance ICS-LABA * Reliever: * PRN SABA

Answer 77

long acting beta 2 agonist

Answer 78

leukotriene receptor antagonist i.e. singulair (montelukast)

Answer 79

sxs most days, or waking with asthma once/week+, or low lung function * Controller: * medium/high dose maintenance ICS-LABA * Reliever: * PRN SABA

Answer 80

* Controller: * add on LAMA * refer for phenotypic assessment * +/- anti-IgE, anit-IL5/5R, anti-IL4R * consider high dose ICS-LABA * Reliever: * PRN SABA

Answer 81

6-11 years old

Answer 82

sxs \<2x/month * Controller: * low dose ICS whenever SABA is taken; or daily low dose ICS * Reliever: * PRN SABA

Answer 83

sxs 2x/month+ but less than daily * Controller: * daily low dose ICS * other: * daily LTRA, or low dose ICS taken whenever SABA taken * Reliever: * PRN SABA

Answer 84

sxs most days, or waking with asthma 1x/week+ * Controller: * low dose maintenance ICS-LABA or medium dose ICS * Other: * low dose ICS + LTRA * Reliever: * PRN SABA

Answer 85

sxs most days or waking with asthma 1x/week + AND low lung function * Controller: * medium dose ICS-LABA and **refer for expert advice** * other: * high dose ICS-LABA, or add on tiotropium or add on LTRA * Reliever: * PRN SABA

Answer 86

* Controller: * Refer for phenotypic assessment + add-on therapy. e.g. anti-IgE * other: * add-on anti-IL5, or add on low dose OCS but consider side-effects * Reliever: * PRN SABA

Answer 87

Short acting beta-2 agonists * albuterol * levalbuterol * metaproterenol * Terbutaline

Answer 88

SABA: MDI and neb * _dosing_: 2puffs Q4-6hours (90mcg/puff) * stimulates beta-2 receptors = bronchial muscle relaxation * _SEs_: **_HypoK_** especially during continuous neb * beta-2 stimulation causes cellular uptake of K+ = decreased srum K+ * also **tachycardia** (because not very selective and will stimulate beta-1 receptors)

Answer 89

long acting beta-2 agonists * salmeterol DPI (Serevent Diskus) * Formoterol DPI (Foradil) * Arformoterol (Brovana) * not for kids * Indacaterol (Arcapta) * not for kids * Olodaterol (striverdi Respimat) * (**Some Fish Are Inherently Odorous)**

Answer 90

* Fluticasone/Salmeterol (Advair) DPI * Fluticasone/Vilanterol (Breo Ellipta) * +3A4 inhibitors (both advair and breo) = increased LABAs = **QT prolongation** * Budesonide/Formoterol HFA (Symbicort) * Mometasone/Formoterol (Dulera) * _SEs_: Thrush * dysphonia * pharyngitis * HA * nausea * tremor

Answer 91

ssRNA Hemagglutinin spikes→ allow virus to bind to host(targeted by IZs) , neuraminidase→ allow virus to bud (targeted by meds) * S/sxs: **sudden onset** high fever, chills, malaise, sore throat, headache, and coryza, **myalgias** (especially legs and lumbosacral), non-productive cough * **_classic triad: FEVER, DRY COUGH, MYALGIAS_** * PE: ill appearance, mild pharyngeal edema, cervical adenopathy +/-, serous nasal drainage * Dx: **rapid antigen test**, serology = more accurate, WBC does not correlate well with severity → often normal or low (if \>15K → suggests secondary bacterial) * Tx: mild disease & healthy? → supportive tx (acetaminophen, fluids, and rest) * hospitalized or at-risk? → neuraminidase inhibitors (Oseltamivir or Zanamivir (Dr. Oz txs the flu)) → need to be initiated within 48 hours of onset * empiric antiviral tx: children \<2, pregnant, 65+, women up to 2 weeks post-partum, chronic or immunosuppressed

Answer 92

aka Whooping Cough → causative agent **bordetella pertussis** → gram -, coccobacillus common in children \<2 yrs, respiratory droplet transmission * S/sxs: consider in adults with cough \> 2weeks * _catarrhal stage_: cold like sxs, poor feeding and sleeping * _paroxysmal stage_: high-pitched **inspiratory whoop** *between coughing fits* → post-tussive emesis is highly indicative of this infx * _convalescent stage_: residual cough (up to 100 days) * Dx: nasopharyngeal swab for cx and PCR * Tx: supportive care +/- steroids/SBAS * **Macrolides** (clarithromycin, azithromycin) * DTaP, TDap Izs * Complications: **PNA**, encephalopathy, otitis media, sinusitis, seizures

Answer 93

Causative Agents: **S. Pneumo (rusty color sputum**, common in after splenectomy), **S. aureus (salmon colored sputum**, lobar, after influenza), Hflu, **Klebsiella (alcohol abuse; currant jelly sputu**m, aspiration); atypicals → **mycoplasma (young ppl living in dorms**, (+) cold agglutination), **chlamydia (college kids**, sore throat), **legionella (air conditioning**, low Na⁺, GI sxs and high fever) * S/sxs: tachycardia, tachypnea, **fever**, pleuritic chest pain, dyspnea +/- rigors (associated with strep pneumo) * PE: CXR: **infiltrate/consolidation** * Typical: percussion → dull, increased tactile fremitus, egophony and crackles * atypical: pulmonary exam is often normal (may have crackles) * Dx: CXR, **blood cxs x 2** , sputum gram stain * Tx: outpt → **doxycycline, macrolides (500mg 1st day, then 250mg for days 2-5)** * CURB-65: Confusion, uremia (BUN \>19), RR \>30, BP \<90/\<60, age 65+ * inpatient tx: * **ceftriaxone + azithromycin, respiratory fluoroquinolones** (Moxi, levo)

Answer 94

\*\*\*most common cause of lower respiratory tract infx in children world wide\*\*\* leading cause of pna and bronchiolitis * S/sxs: rhinorrhea, **wheezing/coughing**, persists for months, low grade fever, nasal flaring/ retractions, often prodomal sxs for 1-2 days before **respiratory distress** * PE: visible retractions * Dx: nasal washing, **RSV antigen test** * CXR can show diffuse infiltrates * Tx: oxygen \<95-96% → hospitalize, same with tachypnea, difficulty feeding * supportive tx * should resolve in 5-7 days * albuterol via neb, humidified O₂

Answer 95

* gram + diplococci, ENCAPSULATED * causes pneumonia: * rusty mucus * productive cough * fever * malaise * sudden onset chills and rigors (violent shaking) * causes: bacteremia and meningitis * tx: **Ceftriaxone**

Answer 96

most common cause in adults: **Influenza;** most common cause in children **RSV, Parainfluenza virus** * S/sxs: **persistent** sxs of sore throat, HA, myalgias, malaise for **more than 3-5 days** then **_new respiratory sxs_** _-→_ dyspnea, cyanosis * Dx: CXR: **bilateral lung involvement with interstitial infiltrate** * Rapid antigen test for influenza * _RSV_ nasal swab * _cold agglutinin titer_ to r/o Mycoplasma (should be negative with this) * Tx: flu → oseltamivir, zanamivir * RSV → **Ribavirin**

Answer 97

**reversible** chronic, inflammatory airway disease → recurrent attacks of breathlessness and wheezing that affects the trachea to the terminal bronchioles * caused by **mast cell mediators** * diminished FEV₁ that is improved with inhaler * s/sxs: dyspnea, wheezing, cough, chest tightness (NOT PAIN) * worse at night * increased mucus production * PE: wheezing, prolonged expiratory phase * Diagnosis: * **GOLD STANDARD**: FEV₁/FVC \< 75-80% in adults, below 85% in children * \>12% increase in FEV₁ after bronchodilator * exhaled nitric oxide = can be used to measure eosinophilic airway inflammation * if pt \<5 yo = RAD, not asthma * GINA guidelines

Answer 98

**_Autosomal Recessive Disorder_** mutation in the CFTR gene → abnormal production of mucus by most exocrine glands → pulm infx most common cause of death (staph and HIB acute, chronic = pseudomonas) * lungs normal at birth then begin to develop pulmonary disease often during infancy or childhood * _infancy_: **meconium ileus**, failure to thrive (FTT), diarrhea from malabsorption (can lead to rectal prolapse) * _pulmonary:_: CF = **most common cause of bronchiectasis in the US** (dilation and destruction of the bronchi due to chronic infx and inflammation * PE: rhonchi and crackles * CXR may show hyperinflation, mucus pluggung and focal atelectasis * Dx: elevated sweat chloride test on **two different days** * use pilocarpine → NaCL \>60mEq/L * Tx: **CFTR genotyping** to see if they are approved for CFTR modulator therapy * hypertonic saline and chest physiotherapy → clears the secretions from the airways * tx infections * replacement of pancreatic enzymes: **supplement fat-soluble enzymes (A, D, E, K)**

Answer 99

usually lodges in the larynx or trachea can lead to chronic, recurrent infx if left for a while→ PNA, ARDS, asphyxia * Risk factors: \>85 yo, \<2 yo, poor dentition, alcohol use, sedative use, institutionalization * S/sxs: Inspiratory stridor * Dx: CXR first * tx: rigid bronchoscopy = preferred for children * soft or rigid bronchoscopy for adults * surgical removal * Cxs if PNA is suspected

Answer 100

aka Hyaline membrane disease * affects **preterm infants** when they have not produced enough **surfactant** → poor lung compliance and atelectasis * \*\*\*most common cause of respiratory distress in pre-term infants\*\*\* * Risk factors: caucasion, male, multiple births, maternal diabetes * S/sxs: * respiratory distress at birth (tachypnea \>60 /min, tachycardia, chest wall retractions, grunting, nasal flaring, cyanosis) * Dx: CXR: diffuse bilateral atelectasis that cause a **ground glass appearance** and air bronchograms * Tx: * antenatal steroids within 24-48 hours of birth → **betamethasone IM x 2** * **artificial surfactant** via ET tube * mechanical ventilation with positive pressure

Answer 101

* _Etiology:_ **immune-related response** * due to: * **drugs**: PCN, sulfa (bactrim), NSAIDs, phenytoin, Diuretics, etc * **immunologic & infx disease**: strep (get an ASO antibody), SLE, CMV, Sjogren's, sarcoidosis

Answer 102

* \*\*\*type of intrinsic AKI\*\*\* * _S/sxs_: oliguria, increased SCr * _Dx_: **urinalysis = WBC cats, WBCs, and eosinophils** * acute azotemia (accumulation of nitrogenous waste, BUN) * **diagnosed with RENAL BIOPSY** → interstitial inflammatory cell infiltrates * _Tx_: d/c offending drug, **corticosteroids, dialysis PRN** * → usually self-limiting if caught early * most people recover kidney function within 1 year

Answer 103

* glomerular damage results in **higher loss of proteins in the urine** * _Most common_ **_primary_** _causes_: * **membranous nephropathy**: most common in non-DM adults associated with *malignancy* * **MINIMAL CHANGE DISEASE**: most common cause in children, idiopathic nephrotic syndrome sxs improve after tx * **focal segmental glomerulosclerosis**: obese pts, heroin, and HIV (+) black males * _Most common Secondary Cause:_ * **lupus** * **DM**

Answer 104

* _S/sxs_: peripheral or **periorbital edema**, ascites, weight gain, fatigue, and HTN, **_frothy urine_** * _Dx_: serologic testing and renal biopsy * **proteinuria \>3.5g/day = diagnostic** ( 24h urine collection) * _urinalysis_: **free lipid or oval fat bodies** or **fatty casts** → lipiduria * **Hypoalbuminemia** \< 3.5g/dL * **hyperlipidemia** LDL \> 130mg/dL, Triglycerides \> 150mg.dL * _Tx_: * tx the causative disorder, **corticosteroids**

Answer 105

* inflammation of the glomeruli due to blockage from immune complexes → **immune response causes this** * _Post-Infectious Group A strep_ → diagnosed with **ASO titers** and low serum complement * _IgA Nephropathy_ **_(berger disease_**_)_: *Most common cause of acute glomerulonephritis* * young males **after URI or GI infx** (within 24-48 hours) → IgA immune complexes are first line defense in respiratory/GI secretions so infx → overproduction which damages the kidneys * more common in asian population * _Membranoproliferative Glomerulonephritis_: caused by SLE, viral hepatitis (Hep C) * secondary to **immune-complex deposition** or **complement mediated mechanism**

Answer 106

* _S/sxs_: edema + HTN + hematuria + **RBC casts**, jaundice, HTN * _Dx_: urinalysis = **hematuria \>3 RBCs/HPF + RBC casts + proteinuria (1-3.5g/day)** * ASO titer for post-strep * **serum complement = decreased** (not always * **RENAL BIOPSY = GOLD STANDARD** * _Tx:_ steroids and immunosuppressive drugs to control inflammation due to immune response * dietary management = salt and fluid restrictions * **Dialysis if symptomatic azotemia** * **ACEI/ARBs (enalapril** or **losartan**) are renoprotective → BP goal \<130/80 * use meds to control hyperkalemia

Answer 107

aka renovascular HTN * **HTN caused by renal artery stenosis** in one or both kidneys * \*\*\*MOST COMMON cause of _secondary HTN_\*\*\* * _Pathophys_: decreased renal blood flow leads to activation of RAAS * _Etiologies_: **atherosclerosis** = most common in elderly, **fibromuscular dysplasia** = most common cause in women \<50 * _S/sxs_: * suspect in pts with **headache & HTN \<20 years** * or **\>50 years, severe HTN** or **HTN** resistant to 3+ drugs * or **abdominal bruits** * or it pt develops **AKI after the initiation of ACE-I therapy** * _Dx_: * non-invasive option: **CT angiography**, MR angiography, Duplex doppler (duplex doppler = less sensitive, specific) * **Renal Catheter Arteriography = GOLD STANDARD and definitive** → revascularization can be performed during the same procedure if stenosis is found (not used in pts with renal failure) * _Tx_: * **Revascularization = definitive management** * **angioplasty with stent** → performed if creatinine \>4.0, increased creatinine with ACE-I tx, or \>80% renal stenosis * Bypass if angioplasty is not successful * Medical Management: * **ACE-I or ARBs** (BUT these are contraindicated in pts with bilateral stenosis or solitary kidney b/c can cause AKI due to ischemia

Answer 108

synthetic analogue of ADH Act on V2-receptors at the collecting duct → reabsorption of water used to tx central diabetes insipidus → the underlying pathophys behind isovolemic hypernatremia

Answer 109

* **causes rapidly progressive Glomerulonephritis (nephritic syndrome)** * _anti-glomerular basement membrane_ * _presentation_: * lungs/kidneys hemorrhage * teenagers & \>50 years * rapidly progressive→ _fatal_ * _Pathology:_ * antibodies against the glomerular basement membranne * often associated with crescent formation * _Tx_: * **cyclophosphamide + corticosteroids + plasmapheresis** * due to high fatality → START RX while waiting for dx

Answer 110

* _Presentation_: * E.coli O157:H7 (foodborne), Salmonella, etc. → undercooked meat consumption * *bloody diarrhea that has resolved* * **fever; low platelets; AKI** * Dx: * often via serum assays * _Treatment_: symptomatic manage,ent * HUS may require dialysis, 10% death rate

Answer 111

* when the testes do not descend aka **undescended testicle** * _Risk factors_: * **premature infants** (30%) vs full term infants (5%) * most common in R testicle * **increases risk of cancer and infertility** * _Dx_: * referral made to urology if testicles have not descended by **3 months of age** * surgery between 6months - 1 year of age (**orchiopexy**) *

Answer 112

urine flow **retrograde** or backward from the bladder **up the ureters and into the kidney** * *Two Types*: * _primary vesicoureteral reflux:_ most common type → when child is born with defect **at the ureterovesical junction** * _secondary vesicoureteral reflux_: * obstruction that causes increased pressure and backflow; most commonly caused by **recurrent UTIs** * _At risk_: * **young females** with hx of **pyelonephritis** or **recurrent cystitis** → evaluate for VUR * _S/sxs_: * fever * urine cx with **E.coli** * _Dx_: * **VCUG (voiding cystourethrography**) and **serial U/S** * _Tx_: * mild to moderate VUR = usually resolves on its own * more serious = **surgery** * recently diagnosed: **give prophylactic abx** that are administered nightly at ½ the normal dosage

Answer 113

infx of bladder * _Most common organisms_: **E.coli**, Klebsiella, proteus, enterobacter, citrobacter * _S/sxs_: * hematuria, dysuria, increased urinary frequency, nocturia * **no fever, chills or back pain** * _PE_: NO CVA TENDERNESS * _Dx_: * **urine dipstick: nitrites, leukocyte esterase** * _urinalysis_: **pyuria** (WBCs in urine), bacteriuria, +/- blood, +/- nitrites * **Urine Cx = GOLD STANDARD** * → but do not need for uncomplicated cystitis * (non-pregnant woman) * _Tx_: * uncomplicated UTIs: * **_trimethoprim -sulfamethoxazole (BACTRIM)_** _x 3 days_ * **Nitrofurantoin x 5 days** * **fluoroquinolones x 3 days** * _Lower UTI in pregnancy_: * **nitrofurantoin** x 7 days * **Cephalexin (Keflex)** x 7 days * _Pediatric Cystitis_: * **1st gen ceph (Keflex**) for low risk of renal involvement * 2nd gen ceph (**cefuroxime)** or 3rd gen ceph (**cefixime, cefdinir, ceftibuten**) for those with high likelihood of renal involvement

Answer 114

* **Mumps = most common cuase in kids** * orchitis _without epididymitis_ = very uncommon in adults * _S/sxs_: unilateral scrotal pain * _PE_: * tender, swollen testicle * shininess of the overlying skin * scrotal edema with erythema * _Dx_: * r/o testicular torsion with **u/s with doppler** * **urinalysis with cxs**: _pyuria_ and _bacteriuria_ with cxs positive for suspected organisms * _Tx_: * rest, NSAIDS, scrotal support, ice, and abx (if bacterial) * Age \<35 or sexuallya ctive post-pubertal males → tx like epididymitis * → **ceftriaxone** IM + **doxycycline** * Age ≥ 35 (STI not suspected) →**levofloxacin**

Answer 115

infx of the kidneys usually by **E. coli** * _S/sxs_: * **dysuria + fever + flank pain** +/- nausea/vomting * _PE_: **flank pain** * _Dx_: **urinalysis**: bacteria and **WBC casts** * _Tx_: * outpatient: **cipro/levo +/- ceftriaxone IM** * inpatient: **cipro/levo** or **imipenem** for more severe disease * **admit all pregnant patients with pyelo!**

Answer 116

infx of the urethra * _Etiology_: **STIs: chlamydia, N. gonorrhoeae, trichomonas vaginalis** & **HSV** = common cause in both sexes * _Sxs_: **dysuria** * in men: **urethral discharge** → can be purulent, whitish, or mucoid * _Dx_; **first void** or **first-catch** urine sometimes with cx * **positive leukocyte esterase** on urine dipstick * or **≥ 10WBCs/HPF** * **nucleic acid amplification test** = allows for identification of N. gonorrhoeae, C. trachomatis * _Tx_: **should treat empirically for STDs** in sexually active pts pending test results * →**ceftriaxone 500mg IM + doxycycline 100mg PO BID x7 days** * → can consider replacing doxy with **azithromycin 1g PO** if compliance in question or pregnancy

Answer 117

* **Most common solid renal tumor of childhood** * arises from otherwise healthy kid's kidneys \< **4 years old** * _Risk factors_: family hx, horseshoe kidney * _Associated Conditions_: **WAGR syndrome** * Wilms Tumor * Aniridia (no iris) * GU abnormalities * Retardation * _PE_: **palpable, nontender mass on the lateral abdomen** * mass feels smooth and firm and does NOT cross the midline * _Dx_: **U/S** and **CT of the abdomen** followed by biopsy or resection. * CXR to look for metastases * **should _NEVER PALPATE_ the abdomen of a child with Wilms tumor** → increases risk of rupturing the encapsulated tumor → metastasis * _Tx_: **surgical resection and chemo** * → most cases are curable \*\*\*\*

Answer 118

* **_Hypospadias_**: when the urethral meatus open onto the **ventral** (bottom/underside) of the **penile shaft** * genetic heritability * **IVF** has been associated with increased risk of **hypospadias** * **_Epispadias_**: when the urethral meatus opens onto the **dorsal (topside)** of the penile shaft * _Dx_: usually made during the newborn exam but **imaging studies (excretory urogram)** can be helpful * _Tx_: **surgical repair** before 1-2 years of age * **DO NOT CIRCUMCISE** → may use foreskin in surgical repair

Answer 119

* entrapment of the foreskin in the retracted position → **Medical Emergency** * **Para**phimosis needs a **Para**medic * \*\*\*always remember to reduce the foreskin after urethral catheterization\*\*\* * _Dx_: clinical * _Tx_: firm circumferential compression of the glans with the hand may reduce the edema enough to allow the foreskin back to its normal position * → if not successful, **dorsal slit using local anesthetic** temporarily relieves the problem → **CIRCUMCISION** after edema is resolved

Answer 120

* foreskin in normal position and **cannot be retracted** * adult phimosis often caused from scarring **after trauma, infx** (such as balanitis) or **prolonged irritation** * _Dx_: clinical * _Tx_: in children, will normally resolve by age 5 * tx not usually required in absence of other issues such as balanitis, UTIs, urinary obstruction * **betamethasone cream 0.05% BID-TID** * gently stretch the foreskin

Answer 121

* mass of fluid-filled congenital remnants of the tunica vaginalis * infants: will usually close within the 1st year of life * _PE_: * **painless scrotal swelling** (most common cause of this) * **+ transillumination** vs tumor or varicocele which both do not transilluminate * _Dx_: **Scrotal U/S** * _Tx_: in infants → will usually close in the 1st year of life, but may require surgery if clinically indicated * **have parents practice watchful waiting for 1 year**

Answer 122

venous varicosity within the spermatic vein * _PE_: feels like a "**bag of worms**' superior to the testicles * **dilation worse when the pt is upright or with valsalva** → decrease in size with elevation of the scrotum or supine position * negative transillumination → chronic non-tender mass that does not transilluminate * _Dx_: **Scrotal U/s** * _Tx_: **surgical repair** if varicocele is painful or appears to be cause of **infertility**

Answer 123

* _Risk Factors_: **after vigorous activity** or minor trauma * usually **post-pubertal boys** (65% in boys age 10-20) * more common in pts with a hx of **cryptorchidism** * _S/sxs_: * *severe, acute onset lower abdominal pain***,** sharp pain that may radiate into thigh * vomiting * _PE_: **negative phren's sign** * loss of **cremasteric reflex** ( elevation of the testes in response to stroking of the inner thigh) * **Blue dot sign**: tender nodule 2-3mm in diameter of the upper pole of the testicle * _Dx_: **U/s with doppler** = best initial test * **Radionuclide scan** demonstrates decreased uptake in the affected testes **→ GOLD STANDARD** * _Tx_: * need to de-torse the testicles in **_\< 6 hours_** (90% salvage rate) * \>24 hours? \<10% salvage * **orchiopexy** (permanent fixation of the testicle)

Answer 124

* most commonly affects **prepubertal girls** and **post-menopausal women** * _S/sxs_: * vaginal bleeding = most common presenting symptom * _PE_: doughnut-shaped protrusion * _Dx_: **clinical** and is often found during a routine exam * _Tx_: **estrogen creams**, vaseline, and sitz baths * **surgical excision** for young patients with symptomatic urethral prolapse or with recurrent urethral prolapse

Answer 125

* narrowing of the urethra caused by: * injury, instrumentation (TURP), infx etc * _S/sxs_: **weak urine flow**, sudden, frequent urges to urinate, **UTI** * **_hesitancy_** (stopping and starting) * _Dx_: cystourethroscopy, retrograde urethrogram (RUG), voiding cystourethrogram (VCUG) * **RUG** → helps to find **location** and **length of stricture** to guide tx * _Tx_: **urethral dilation** or **stent placement**

Answer 126

generally applied to kids ≥ 5 years of age, **meds for kids \>6 years only** * often hereditary * 3 main causes: * nocturnal polyuria * detrusor overactivity * increased arousal thresholds * _Minor Enuresis:_ **_we can tx_** * daytime frequency * giggle incontinence * stress incontinence * post void-dribbling * nocturnal enuresis * _moderate enuresis:_ **_referral to uro_** * underactive bladder * overactive bladder * dysfunctional elimination syndrome

Answer 127

**anticholinergic** used for _overactive bladder_ in children \> 6years

Answer 128

X-linked recessive inheritance _clotting factor disorder_ * _Hemophilia A_: **factor VIII** decrease → “Aight” * _Hemophilia B_: decreased clotting **factor IX** * Normal Factor Activity: 50-150% * _mild_: 6-49% (Bleeding during surgery) * _moderate_: 1-5% (occasional bleeding) * _Severe_: \<1% (spontaneous bleeding) * _S/sxs_: * **hemarthrosis** (bleeding in joint) * main sxs = excessive bleeding * _Diagnosis_: **increased PTT**, normal PT, normal platelets and function * → corrected with **mixing study** * if PTT is NOT corrected with mixing studies = indication of lupus anticoagulant or factor *inhibitor* * most specific test: **functional assay for factor VIII or IX** * _Tx_: replacement for depleted factors * avoid situations that cause bleeding * avoid certain drugs that interfere with platelet function (NSAIDs and aspirin)

Answer 129

Autosomal Dominant → **most common genetic bleeding disorder** _clotting factor disorder_ * missing protein for platelet function (usually hangs out in the **endothelium** of the **blood vessels** and is important for factor VIII transport → **platelets cannot adhere to vessel at site of injury** → bleeding doesn't stop as quickly as it should * hormonal changes, stress, pregnancy, inflammation, and infection can stimulate vWF production * _S/sxs_: hx and family hx of bleeding, bruising easily, increased menstrual bleeding, **no hemarthrosis**, petechiae, bleeding with minor injury * _Dx_: * **decreased vWF** and **decreased Factor VIII** * normal CBC, normal platelet count, and increased bleeding time * → _normal or prolonged PTT_ with normal PT * _Tx_: **DDVAP (desmopressin)** * if excessive bleeding → transfusion of concentrated blood clotting factors containing vWF

Answer 130

_Clotting Factor Disorder_ Vitamin K does not cross the placenta and it is a fat soluble vitamin for bone calcification and activation of coagulation factors * Vitamin K activates: factors II, VII (half-life = 4-6 hours), IX, X, Protein C and Protein S * **prolonged PT** * _causes:_ malnutrition, abx, fat malabsorption, **hemorrhagic disease of the newborn** * _S/sxs_: * bleeding, mucosal bleeding, epistaxis, GI hemorrhage, menorrhagia, and hematuria * _Dx_: * **prolonged PT** or **elevated INR** that improves with **_phytonadione_** (generic name for vitamin K1) * _Tx_: Oral or SQ phytonadione

Answer 131

Most common genetic **_hypercoagulable state_** mutations in factor V (of the common pathway) make it resistant to normal inactivation by activated **protein C** and predispose the pt to venous thrombosis * **have high suspicion in pts with recurrent thromboembolic events, thromboembolism in young pts, or in pts with NO risk factors; _previous miscarriages_** * _S/sxs_: hx of multiple DVTs, CBC is normal, PT and PTT are normal * _Dx_: * Factor V leiden Assay * Protein CC * _Tx_: anticoagulation * **antiplatelet therapy**: aspirin, clopidogrel, prasugrel or dipyridamole/aspirin * **oral anticoagulant**: Vitamin K antagonist, warfarin, direct thrombin inhibitor (**dabigatran**) or factor Xa blocker **(rivaroxaban**)

Answer 132

aka immune thrombocytic purpura * _Epidemiology_: **autoimmune rxn to platelets** usually after a *viral illness* → **splenic platelet destruction** often after an acute infx * _Caused by_: viral infx, SLE, lymphoma, medications * _s/sxs_: * mucosal bleeding, purpura, rashes, easy ecchymosis, petechial rashes * **chronic in adults**, **self-limiting in children** (usually) * _dx_: * diagnosis of exclusion (clinical diagnosis) * **isolated thrombocytopenia (very low)** with a **normal CBC** and **normal peripheral blood smear** * (+) Direct Coombs Test * **Normal PT and aPTT** * _Primary ITP_: isolated thrombocytopenia (\<100K) without a known cause * _Secondary ITP_: isolated thrombocytopenia (\<100K) with an underlying condition (e.g. HIV) * _Tx_: * steroids (**prednisone**) → blocks production of antibody * IVIG (IV Immune globulin) * Rituxan (**Rituximab**) → thrombopoietin receptor *agonist* * **Splenectomy** → reserved for pts with severe thrombocytopenia (\<15K)

Answer 133

Low MCV (\<80): a Tic is small (microcytic) * Thalassemia * Iron Deficiency * Chronic Disease * Lead poisoning

Answer 134

Microcytic Anemia: most common cause of anemia worldwide * _Pathophys_: decreased RBC production due of lack of iron and decreased iron stores (ferritin) normally stored in the bone marrow, liver and spleen * _Etiology_: * **chronic blood loss** = most common cause in the US, excessive menstruation, occult GI blood loss, _decreased absorption_: diet, celiac, bariatric surgery, *H.pylori* * _Risk Factors:_ * increased metabolic requirements → children, pregnant and lactating women * → **cow milk ingestion in young children**: infants fed cow's milk \<1 yr of age, toddlers fed large quantities of cow's milk

Answer 135

Microcytic (\<80) anemia * _S/sxs_: weakness, fatigue, exercise intolerance, dyspnea * **pagophagia**→ craving for ice * **Pica**: appetite for non-foods (clay, starch) * _PE_: **koilonychia**: spooning of the nails * **angular cheilitis** : inflammation of the corner of the mouth * tachycardia, glossitis (smooth tongue), signs of anemia (pallor) * _Dx_: * CBC: **microcytic hypochromic anemia = CLASSIC** * _Increased RDW_, decreased reticulocytes * _Iron Studies_: **decreased ferritin (pathognomonic), increased TIBC** (transferrin), **decreased transferrin saturation** * _Tx_: **iron replacement**: results in increased reticulocytes (within 5-10days), correction of anemia (6-8weeks), repletion of iron stores (1-3 months) * **increased absorption**: take iron replacement with vitamin C (ascorbic acid) with water or orange juice on an empty stomach * _severe life threatening anemia tx_: rbc transfusion (e.g.myocardial ischemia)

Answer 136

* acute onset of pallor from anemia * **splenomegaly** * jaundice with **high indirect bilirubin** → too much RBC breakdown for the liver to keep up with * **_increased LDH_**: hemolytic anemias will have increased LDH b/c it is part of the RBC membrane * **increased reticulocytes** * _Dx_: * **positive Coombs test if autoimmune etiology** * _G6PD deficiency_: * **episodic hemolytic anemia associated with sulfa drugs, fava beans, infx** * *HEINZ BODIES* * _Hereditary Spherocytosis_: * (+) osmotic fragility test: mixes RBCs with varying saline, abnormally shaped RBCs will be fragile and fall apart * _Thalassemia_: very low MCV + normal TIBC and ferritin * _Sickle Cell Anemia_: very high reticulocyte count + pain

Answer 137

Megaloblastic Macrocytic Anemia _Folate needed for DNA synthesis_: decreased folate → abnormal synthesis of DNA, **folate stores last 2-4 months** * _Etiology_: most common: inadequate intake (**alcohols, unbalanced diet**) * → increased requirements: **pregnancy, infancy, hemolytic anemias, psoriasis** (increased skin turnover) * → impaired absorption: *celiac disease, inflammatory bowel disease, chronic diarrhea, anticonvulsants* (phenytoin, phenobarbital, carbamazepine) * → impaired metabolism: **methotrexate, trimethoprim** * → Loss: dialysis

Answer 138

Megaloblastic Macrocytic (\>100) Anemia * _S/sxs_: anemia sxs **similar to B12 deficiency but _without neurologic abnormalities_** * fatigue, exercise intolerance, pallor, **chlorosis** (pale, faintly green complexion) * Glossitis, aphthous ulcers, diarrhea * Maternal Deficiency = risk of neural tube birth defects * _Dx_: decreased Folate, increased MCV * what to order: **CBC, serum vitamin B12, and folate** * _CBC with peripheral smear_: * **increased MCV +** megaloblastic anemia **(hypersegmented neutrophils, macroovalocytes)**, low reticulocytes * **_decreased serum folate (\<3)_**, increased LDH, increased homocysteine, *normal methylmalonic acid* (distinguishes from B12 deficiency) * _Tx_: * folate supplements (oral folic acid): **400-1000 micrograms PO daily** * d/c offending med and limit alcohol consumption * CDC recommendation and pregnancy: 4000 mcg each day 1 month before becoming pregnant, and during 1st 3 months of pregnancy

Answer 139

Sources of B12: mainly animal products (meats, eggs, dairy) * _Absorption_: B12 is released by the acidity of the stomach & _combines with_ **intrinsic factor** where it is mainly absorbed in the ***distal ileum*** * _Pathophys_: B12 deficiency → **abnormal synthesis of DNA** ; B12 normally needed to convert homocysteine to methionine for DNA synthesis. * _Etiology_: * **Pernicious Anemia = most common cause →** lack of intrinsic factor due to parietal cell antibodies leading to gastric atrophy (_parietal cells secrete acid and intrinsic factor_) * **Crohn's Disease**, **gastric bypass,** chronic alcohol use, celiac disease, pancreatic insufficiency * Meds: **H2 blockers, PPIs** (decreased acidity of stomach = less absorption); **metformin** (decreased nucleic acid synthesis) * **Vegans → decreased intake**

Answer 140

Megaloblastic macrocytic (\>100) anemia * _S/sxs_: anemia sxs similar to folate deficiency → fatigue, exercise intolerance, pallor, weight loss, **glossitis**, diarrhea * _neurologic sxs_: **symmetric paresthesias = most common initial sxs** (especially the legs) * _lateral and posterior spinal cord demyelination and degeneration_: ataxia, weakness, **vibratory, sensory and proprioception deficits, decreased deep tendon reflexes, + babinski** * _Dx_: * CBC with peripheral smear: * **increased MCV** + megaloblastic anemia (**hyper segmented neutrophils**), low reticulocytes * **increased homocysteine levels and methylmalonic acid** → distinguishes B12 from folate deficiency * _Tx_: * B12 replacement * for symptomatic anemia or neuro findings: **start with IM B12**, if adult IM = cyanocobalamin injection weekly until deficiency is corrected then once monthly) * **pts with pernicious anemia require lifelong monthly IM therapy** (or high dose oral therapy) * dietary deficiency = oral B12 replacement

Answer 141

Hemolytic Anemia * **X-linked recessive** that can cause **episodic hemolytic anemia** * _Risk Factors_: **primarily males** (x-linked), **10-15% of African-American males** * _Pathophys_: * G6PD catalyzes NADP to NADPH, which protects RBCs from oxidative injury * → loss of the NADPH causes the oxidative injury to the RBCs which leads to **denatured HGB that will then precipitate as a _Heinz Body_** * _Exacerbating Factors_: * **infection = most common cause** * fava beans * Medications: **dapsone, primaquine, methylene blue, nitrofurantoin, phenazopyridine**

Answer 142

X-linked recessive episodic Hemolytic Anemia * _S/sxs_: **most pts are asymptomatic until times of oxidative stress** * sxs begin 2-4 days after exposure → **back or abd -pain, sxs of anemia, jaundice**, transient splenomegaly * neonatal jaundice * _Dx_: **G6PD assay** * **Heinze bodies + bite cells** * increased LDH, increased indirect bilirubin, decreased haptoglobin * _Tx_: * **avoidance of trigger and supportive care** * during acute hemolysis, tx is supportive; **transfusion is** ***rarely*** **needed** * neonatal jaundice: phototherapy

Answer 143

**Autosomal Recessive** → causes defective beta chain that leads to RBCS **to sickle under conditions of low oxygen** * HbA + HbS; _HbSA = trait_ * avoid hypoxia and dehydration * **no anemia**, resistance to malaria * **Hematuria** * _Dx_: **sickled cells on peripheral smear + HbS + HbA** on hgb electrophoresis

Answer 144

**Autosomal Recessive Normocytic Anemia** HbSS = disease, causes a defective beta chain * almost exclusively in AAs, caused by homozygous inheritance of HbS → sickle-shaped RBCs clog capillaries, causing organ ischemia and infarcts * _S/sxs_: anemia, jaundice, arthralgias, fever, **painful** (severe abd and joint pain) * **slow healing ulcers**, N/V, cranial nerve palsies, **hepatosplenomegaly**, cholelithiasis * _Dx_: * **(+) HgbS on hemoglobin electrophoresis** = Definitive (no Hgb A) * **normochromic, normocytic cells**, increased bili, and LDH * hgb: approx 8-10, HCT 20-30% * **reticulocytosis** (increased reticulocytes) * **sickle cells** * **Howell Jolly Bodies** (nuclear remnants that have not been phagocytosed due to reduced splenic function) * _Tx_: * **crises are tx with analgesics and other supportive measures** (oxygen, hydration) * folic acid (penicillin prophylaxis in children) * vaccines prolong survival * **antisickling agents**: * _Hydroxyurea_ may decrease the frequency of crises * _voxeolotor_: increased oxygen affinity of HbS * _Crizanlizumab_: prevents cell adhesion * RBC transfusion to minimize hypoxia → Hgb \>10 * **Stem cell transplant and gene therapy = curative** * **Median survival 60yo: infx, CVA, renal failure, pulm HTN**

Answer 145

Autosomal Recessive hemoglobinopathy **Hypochromic microcytic anemia** * _S/sxs_: * **carrier**: 1 deletion = asymptomatic * **alpha thalassemia minor**: 2 deletions = mild anemia, microcytic hypochromic * cis deletion for *asians* * trans deletions for *AAs* * **alpha thalassemia major**: 3 deletions: _symptomatic_ → SPLENOMEGALY, MCHC, anemia, some need transfusion periodically * **Hemoglobin Bart's Hydrops Fetalis**: 4 deletions = incompatible with life * _Dx_: **decreased MCV, low hgb, _HIGH RBC_,** normal ferritin, normal TIBC and a **(+) HgB electrophoresis** * _peripheral smear_: **target, heinz bodies** “Golf ball” looking cells and teardrop cells, microcytic * _hgb electrophoresis_: normal hgb rations in adults with 1-2 gene deletions, detects **hgbH with 3 gene deletion** * _Tx_: for alpha thal minor → no tx * for alpha thal major → can do splenectomy if severe anemia or splenomegaly

Answer 146

**Autosomal recessive** hemoglobinopathy hypochromic microcytic anemia * _Beta Thal major_: 2 of 2 beta genes damaged = significant organ damage, transfusion dependent anemia, excess alpha chains, stable as monomers, no precipitation in RBC * _Beta Thal Minor_: 1 of 2 beta genes are damaged = mild-mod anemia * _S/sxs_: * thrombosis = vascular disease * ineffective erythropoiesis → **splenomegaly, hepatomegaly, bone pain** * hemolysis = iron deposition and gallstones → **endocrine dysfunction, cirrhosis, heart failure** * _Dx_: * **_very high RBC_, low MCV, _very hypochromic_**, normal ferritin, normal to low reticulocyte count, normal RDW * _peripheral smear_: target and **teardrop cells**, basophilic stippling; nucleated RBCs → suspect thalassemia in pts with microcytosis and normal iron with increased RBC count * _hgb electrophoresis_: * **trait (minor)**: increased HgA2 or increased HgbF and ***decreased HgbA*** * **Major (Cooley's)**: increased HgA2 or ***increased HgbF*** (up to 90%) and little to no HgbA * _Tx_: * beta thal major: transfusion hgb 11-12 * folic acid daily * **luspatercept**: RBC maturation agent * splenectomy * bone marrow transplant (*for severe cases*) * iron chelation daily (need to prevent iron overload)

Answer 147

alpha and gamma hemoglobin most prevalent just before birth, beta hgb rises just before birth 4 alpha, 2 beta in normal HgbA

Answer 148

2alpha and 2 beta chains normal adult Hgb

Answer 149

2 alpha and 2 gamma chains seen in **beta thal**

Answer 150

2 alpha and 2 delta chains ## Footnote **beta thal**

Answer 151

_4 beta chains_ ## Footnote **alpha thal**

Answer 152

* onset over few weeks * symptomatic * immature WBCs * decreased hgb and platelets * need tx * risk of death

Answer 153

* onset over months -years * minor sxs * excess mature WBCs * normal hgb and platelets * may need tx * chronic disease

Answer 154

* _Population_: **children**, boys\>girls, hispanics, 2-5yo * _Pathophys_: overpopulation of immature WBCs (**lymphoblasts**) overtake normal hematopoiesis * **_Pearl_**: *CHILD+Lymphadenopathy + bleeding + fever + bone marrow \> 20% blasts* * _S/sxs_: **Pancytopenia →** fever & infx (leukopenia); bleeding, petechiae, purpura (thrombocytopenia); Pallor & fatigue (Anemia) * _Dx_: **bone marrow aspiration \>20% blasts** * **flow cytometry test**: tells you subtype of leukemia * WBCs 5-100K, decreased hgb and platelet * _Tx_: **combination chemo** → highly responsive with a remission \>90% * can do stem cell transplant if relapse

Answer 155

associated with lead poisoning → microcytic anemia

Answer 156

* _Definition_: persistent communication between the descending thoracic aorta and the main pulmonary artery after birth → **L to R Shunt** * _Pathophys_: * continued **Prostaglandin E1 production** & low arterial oxygen content promotes patency. → Enlarged R atrium, R Ventricle, & L Ventricle * _Risks_: * **Prematurity**, female, fetal hypoxia

Answer 157

* _S/sxs_: * Most are asymptomatic: * but some have → poor feeding, weight loss, frequent URIs * if they experience Eisenmenger Syndrome → left-to-right shunt switches to a right-to-left shunt → **Cyanosis** * _PE_: * **Continuous Machinery Murmur** loudest at the Left upper sternal border (*PDA → personal digital assistant = machine*) * **wide pulse pressure** (bounding pulses)

Answer 158

* _Dx_: **ECHO = best initial test** * _Tx_:**I****ndomethacin, ibuprofen** (inhibits prostaglandin) * surgical: left thoracotomy * clip the premature babies * ligate the neonates & infants * divide and oversew the toddlers & infants * Catheter based: * coil device → for older infants & children

Answer 159

* Pulmonary HTN & Cyanotic heart disease occuring when a left-to-right shunt shunt switches to a right-to-left shunt → **Cyanosis**

Answer 160

Most common **cyanotic** congenital heart disease (right-to-left shunt) * _4 components_' 1. **RV outflow obstruction** (sometimes call pulm stenosis) 2. **R ventricular hypertrophy** 3. **Overriding Aorta** (shifted to the R; sits right over the VSD) 4. **Ventricular Septal Defect**

Answer 161

* _S/sxs_: * **infants:** **Cyanosis** * **Older Children**: TET spells: paroxysms of cyanosis relieved with squatting or pulling legs up * (increased peripheral vascular resistance causes the shunt to switch to left-to-right shunt ) * _PE_: * **Harsh systolic murmur at left mid-to-upper sternal border** changes similar to HOCM (louder with decrease in ventricular volume- blue, softer with increase in ventricular volume -pink) → **children will squat or pull legs to chest to increase peripheral vascular resistance** * R. ventricular heave * digital clubbing * cyanosis * \*Often dynamic (TOF spells) & progressive

Answer 162

* _Dx_: **Echo = test of choice** * CXR: **Boot shaped heart** (prominent R ventricle) * EKG: RVH, R. Atrial Enlargement * _Tx_: * DO NOT LET THEM GET DEHYDRATED → TOF spells (need to treat gastroenteritis aggressively) * **surgical repair by 3-6 months** or sooner if blue

Answer 163

Boot Shaped Heart Associated with **Tetralogy of Fallot** demonstrates prominent **R ventricle** due to **RV outflow obstruction**

Answer 164

* _Definition_: * abnormal opening in the ventricular septum, associated with a left-to-right shunt. **Most common type of congenital heart defect!!** * Most common defect in **Trisomy 21 (Down Syndrome)** * _Types_: * **Membranous =** **most common type**, hole in the LV outflow tract near tricuspid valve (higher up on the septum) * Muscular: multiple holes in “swiss cheese” pattern → these may resolve on their own

Answer 165

* _S/sxs_: * **Small VSDs**: may be asymptomatic * **Large VSDs**: symptoms manifest within 6 months of age: * **Failure to thrive** (have to pump more blood, breath harder → higher caloric demand, and decreased ability to feed) * **Poor growth** * **Tachypnea** * **GERD** * (liver enlarges due to heart failure and pushes on stomach) * **Frequent URIs** * (due to wet lungs) * _PE_: * high pitched **holosystolic murmur** best heard at the LLSB * **smaller VSDs are louder with more palpable thrills** * normal pulses

Answer 166

* _Dx_: * **Echo =** determines size & location of VSD * CXR: cardiomegaly &/or congestion * _Tx_: * Diuretics, ACEI +/- Digoxin * some may close or get smaller on their own * **Indications for Surgery**: * unmanageable heart failure * failure of medication management * shunt greater than 1.5-2x with or without sxs (babies hearts can accommodate 1.5-2x the volume often before symptoms) * \***\*Want to try to get them to 3-6 months of age before surgery, but not necessary if FTT or other issues\*\***

Answer 167

* _Definition_: * congenital **narrowing of the aortic lumen** at the **juxtaductal area** (insertion of ductus arteriosus distal to left subclavian artery) * Often associated with **Turner syndrome** and bicuspid aortic valve * _Pathophys_: * narrowing of the aorta most commonly → HTN in the arteries proximal to the lesion with hypotension in the lower extremities * overtime, the body develops collaterals around the coarctation

Answer 168

* _S/sxs_: * **Neonates**: Failure to thrive * poor feeding * tachypnea, irritability * CHF * \*\*Severe Coarctation: LV failure & Shock after birth when the _ductus closes_\* * **Older children/Teens**: * headache * nosebleeds * absent or diminished femoral pulses * **Unexplained HTN\*\*\*** * _PE_: * **upper extremity systolic HTN with lower extremity hypotension** * **Diminished or delayed lower extremity pulses** (vs. VSD that has normal pulses * Systolic murmur at LUSB with radiation to the **scapula** (back) * _Dx_: * _CT-angiography_ = **Gold standard** * CXR: cardiomegaly * **posterior rib notching** (due to increased intercostal artery collateral flow) after several years * **3 sign** (narrowed aorta looks like the notch of the number 3) * ECG: LVH * Echo: useful but difficult to visualize coarctation

Answer 169

* _Dx_: * CXR: cardiomegaly * **posterior rib notching** (due to increased intercostal artery collateral flow) after several years * **3 sign** (narrowed aorta looks like the notch of the number 3) * ECG: LVH * Echo: useful but difficult to visualize coarctation * _Tx_: * **surgical**: left thoracotomy with extended end-to-end anastomosis; stents reserved for recurrent coarctations * **Prostaglandin E1 (****Alprostadil**) can be used to open the ductus arteriosus and relax the narrowed aortic segment * Early referral saves lives!

Answer 170

**3 Sign** associated with **Coarctation of the Aorta** narrowed aorta looks like the notch of the number 3

Answer 171

Coarctation-Rib Notching Associated with **Coarctation of the Aorta** due to increased intercostal artery collateral flow after several years

Answer 172

Hyperoxia test

Answer 173

* truncus did not separate into aorta and pulmonary artery * **Large VSD** → R-L shunt * repair: VSD closure, RV-PA conduit, usually operate in first 2 weeks of life * associated with DiGeorge Syndrome 22q11

Answer 174

Benign Murmur * SYSTOLIC, grade 1-2/6 * best heard at APEX of heart & LL sternal border * best heard with BELL of stethoscope * represents the normal sound of blood gushing out _into the aorta during contraction_ * healthy 3-7 year old children

Answer 175

* anything DIASTOLIC * anything \>II/VI * → get an echo

Answer 176

* _Rotation_: * tetralogy of fallot * transposition of the great arteries (TGA) * _Septation_: * ventricular septal defects * atrial septal defects * truncus arteriosus * _Migration_: * total anomalous pulmonary venous drainage (TAPVR)

Answer 177

**autosomal dominant** * _Pathophys_: * subaortic **outflow obstruction** d/t **asymmetrical septal hypertrophy** & systolic **anterior motion of the mitral valve** * \*\*Hypercontractile\*\* * can have myocardial ischemia d/t increased muscle mass and increased demand for O2 * _Risk Factors_: * young age, fam hx of sudden death, gene mutations prone to SCD, sustained VT or SVT, recurrent syncope, brady arrhythmias * **#1 cause of sudden death in competitive athletes \<35 yo**

Answer 178

* _S/sxs_: * **dyspnea, syncope** * angina * palpitations * dizziness * fatigue * **sudden cardiac death** * _PE_: * _Hypertrophic Obstructive Cardiomyopathy Murmur:_ **Crescendo-decrescendo systolic murmur** (best heard at R sternal border) * **increased with decreased venous return** (valsalva or standing) * → d/t less volume in L ventricle meaning that the obstruction from the septum is more severe, (vs when it has more volume it pushes the septum to its “normal” place making the murmur less severe) * loud S4 (di-a-stol-ic) * mitral regurg * _Dx_: * Echo: * **asymmetrical ventricular wall thickness** * systolic anterior motion of the mitral valve * small LV chamber * EKG: * **Left ventricular hypertrophy** * Septal Q-waves * R. atrial enlargement

Answer 179

* _Tx_: * **Beta-blockers** = 1st line, or CCB * Diuretics if CHF symptoms * surgery (if gradient \>50 mmHg at rest or class III-IV heart murmur) * alcohol septal ablation * dual chamber pacing * cardiac transplant * Pt should avoid dehydration, extreme activity, **& competitive sports** (low-risk older pts can speak with a cardiologist) * _Complications_: * can progress to dilated cardiomyopathy * a.fib, stroke * clinical deterioration is slow

Answer 180

aka Gastrinoma * _Definition_: **gastrin–secreting neuroendocrine tumor** → parietal cells release excess HCl → Severe PUD & diarrhea. Most commonly seen in the **duodenum** & pancreas * _S/sxs_: * **severe Peptic Ulcer Disease** **refractory to tx** * **chronic diarrhea** * weight loss * _Dx_: * **elevated serum gastrin levels** * _Tx_: * tumor resection if local * if metastatic = lifelong high-dose PPIs * \*Liver & abdominal lymph nodes = most common sites for METS

Answer 181

* _Pathophys_: * overgrowth/hypertrophy of the pylorus → stomach contents are unable to pass through the gastric outlet → leads to increased pressure → forced vomiting may occur * _S/sxs_: * 3-6 week old baby with non-bilious “projectile vomiting” after meals * -after vomiting the child cries from hunger * -dehydration * _PE:_ * Pyloric sphincter may be palpable→ described as **olive shaped** * _Dx_: * **pyloric US** * labs to evaluation for dehydration and electrolyte status * _Tx_: * Pyloromyotomy

Answer 182

* _Definition_: * inflammation of the small bowel secondary to the ingestion of gluten-containing food * villous atrophy occurs as a result of this autoimmune disease trigger → decreased absorptive area → malabsorption (of fat) * _General Info_: * **HLA-DQ2** and/or **DQ8** loci * **_Autoimmune disorder_** * → runs in families * Female \> Male usually 10-40 years old * Most common in N European ancestry * _S/sxs_: * **diarrhea, steatorrhea** (will have floating stools), **flatulence, weight loss** * weakness, and abd distention * infants and children present with FTT * _Malabsorption symptoms_: * weight loss, poor growth, severe anemia, neuro disease from B12 deficiency *

Answer 183

* _PE_: * **dermatitis herpetiformis:** herpes -like rash of grouped vesicles that's intensely pruritic INTENSELY ITCHY (like poison oak, but not in streaks) * _Dx_: * **ALL TESTING SHOULD BE PERFORMED ON A GLUTEN RICH DIET** * **IgA antiendomysial (EMA)** and **Antitissue transglutaminase (anti-TTG) antibodies** * **Tissue transglutaminase antibodies** **(TTG-IgA)**: the TTG-IgA test will be positive in about 98% of patients with celiac disease who are on a **gluten-containing diet** * **endoscopic intestinal mucosal biopsy = definitive diagnosis** * if biopsy & serum tests disagree = DQ2 & DQ8 genotyping * _Tx_: * Gluten free diet → caution with oats (no wheat, barley, rye) * supplementation may be needed: **iron, vitamin B12, folic acid, calcium, Vitamin D**

Answer 184

* _Definition_: * the **invagination** of a **proximal segment of the bowel into** the **portion just distal to it** * _Risk factors:_ * ⅔ of cases seen in age 6-18mo of age, esp esp males, esp after viral infections * adults = think NEOPLASM! * _S/sxs_: * **_Classic Triad_**: * 1. Vomiting * 2. Abd pain * 3. Passage of blood per rectum **“currant jelly” stool** (stool mixed with blood & mucus) * **sudden onset** of significant **colicky abd pain** that recurs Q 15-20 min often with vomiting * _PE_: * **sausage shaped mass in RUQ** * _Dx_: * _For kids_: **barium** or **air enema** can be both **diagnostic & therapeutic** * Best initial test = abd U/S looking for target or donut sign * then Abd XRay → Crescent sign or Bull's eye target * _tx_: * Children: barium or air enema * surgery if refractory * Adults = surgery

Answer 185

* _Definition_: * partial or complete mechanical block of the small intestine * _S/sxs_: * **_4 hallmark sxs_**_:_ * 1.Crampy abdominal pain, 2. abdominal distention, 3.vomiting (Bilious), and 4.obstipation * _PE_: * **High-pitched tinkling bowel sounds with visible peristalsis or ABSENT (SEVERE)** * _Dx_: * **Abdominal KUB Xray**: **multiple air-fluid levels** in a **“step-ladder” appearance, dilated bowel loops**. * _Tx_: * Non-strangulated: * NPO (bowel rest), IV fluids & electrolytes. Bowel decompression if severe vomiting * Strangulated: * surgical intervention

Answer 186

Small bowel obstruction

Answer 187

* _definition_: * obstruction of the lumen of the appendix, results in inflammation & bacterial overgrowth * _Risks_: * 10-30 yrs * _Etiology_: * **Fecalith & lymphoid hyperplasia = most common** * inflammation, malignancy or foreign body * Lymphoid hyperplasia due to infx = most common cause in kids * _PE_: * Rebound tenderness at **McBurney’s Point** * **Rovsing sign**: RLQ pain with palpation of LLQ * **Obturator sign**; RLQ pain with internal rotation of hip * **Psoas sign**: RLQ pain with hip extension while in Left lateral decubitus position * _Dx_: * clinical diagnosis * In adults: **CT scan of abdomen/pelvis** = imaging of choice with u/s and MRI reserved for radiosensitive populations (pregnant women, children) * **CBC** - neutrophilia * _Tx_: * **appendectomy**

Answer 188

⅓ between ASIS to umbilicus RLQ deep tenderness = appendicitis * Also where you test for rebound tenderness * release the pressure quickly!!

Answer 189

Palpate on the LLQ and the pt will feel pain on the RLQ suggests appendicitis

Answer 190

Have pt lie supine and lift one, straight leg against resistance of your hand * stretches the psoas muscle which puts pressure on the appendix * sign is **Positive** if pt feels more abd pain when lifting leg

Answer 191

When the pt lies supine and you bend the pts leg moving the knee laterally * this stretches the obturator muscle = puts more pressure on appendix * this sign is positive if abd pain increases

Answer 192

Press firmly upward on the RUQ and ask pt to take a deep breath * if gall bladder is enlarged it will push down on the hand and elicit pain. * sign is **positive** if pt stops breathing due to pain

Answer 193

feeling for splenomegaly * percuss at castell's spot and have pt inhale and exhale * if you hear dullness on percussion during this inhale/exhale = **positive indication** for splenomegaly

Answer 194

* _Source_: usually caused by **invasive or toxin producing bacteria** that **damage** the large intestinal mucosa * _S/sxs_: * Smaller volume diarrhea with blood, mucus +/- leukocytes * Tenesmus: “Dry heaving from your butt” * abd pain, fever * _Tx_: * supportive care and disease specific * DO NOT GIVE antimotility agents as the toxins will remain present longer and worsen the disease

Answer 195

* _Source_: enterotoxins increase GI secretion of electrolytes → causes secretory diarrhea * → no cell destruction or mucosal invasion * _S/sxs_: * **large volume of diarrhea** * vomiting * no fever or blood in stool * _Tx_: * supportive care and disease specific tx PRN

Answer 196

* invasive/inflammatory diarrhea * **\*\*Most common bacterial cause of diarrhea in the US\*\*** * Most common antecedent of **Guillain Barre Syndrome** * **Consumption of undercooked poultry = MCC;** **Raw milk consumption & Puppies!** * _S/sxs_: * bloody diarrhea, fever, abd pain * **guillain barre syndrome: ascending paralysis, loss of DTRs** * _Dx_: * stool cx with S or comma shaped **gram neg** rods * **PCR** = most practical and most commonly done (but not the answer on BOARDS) * _Tx_: * usually self limiting * abx can shorten duration → **Macrolides (Azithromycin)**

Answer 197

* invasive/inflammatory diarrhea * _Sources_: * contaminated **pork products =** most common, milk, water, **tofu** * _S/sxs_: * of inflammatory diarrhea * mesenteric lymphadenitis → abd tenderness & guarding known as **pseudoappendicitis** * smaller volume diarrhea blood, mucus +/- leukocytes * Tenesmus: “Dry heaving from your butt” * abd pain * fever * _Dx_: * **stool culture = preferred** * “safety pin appearance” of gram neg bacilli * _Tx_: * fluids/electrolytes * severe diarrhea → **fluoroquinolones** or **TMP/SMX** **(Bactrim)**

Answer 198

* invasive/inflammatory diarrhea) * Produces **Shiga toxin** → damages the endothelial lining and leads to hemorrhage * Most common in **elderly** **&** **children** * _Sources_: * undercooked ground beef, unpasteurized milk or cider, day care centers * _s/sxs_: * Smaller volume diarrhea with blood, mucus +/- leukocytes * Tenesmus: “Dry heaving from your butt” * abd pain, fever * Watery diarrhea that later becomes **bloody + vomiting** * _Dx_: * stool cx * PCR * _Tx_: * fluid/electrolytes * \*\***avoid abx in children due to risk of** **_hemolytic uremic syndrome_****\*\***

Answer 199

* invasive/inflammatory diarrhea * _Sources_: * undercooked or raw poultry, eggs, milk, fresh product * contact with reptiles (Turtles) * _S/sxs_: * Short incubation: 1-3 days * sxs of inflammatory diarrhea + vomiting * Smaller volume diarrhea with blood, mucus +/- leukocytes * Tenesmus, abd pain, fever * _Dx_: * stool cx * _Tx_: * Fluid/electrolytes * severe diarrhea → **Fluoroquinolones**

Answer 200

* invasive/inflammatory diarrhea * Most common cause = traveling to or living in an underdeveloped nation * _Source_: * fecal-oral * humans are only reservoir for typhoid * _S/sxs_: * _Classic (but rare_): **fever with relative bradycardia** * “**Pea soup diarrhea”** * **Rose spots (**faint pink/salmon colored macular rash) * _Dx_: * blood cx & Stool cx * _Tx_: * fluid/electrolytes * Severe diarrhea → **f****luoroquinolones****x****2 weeks** (2x as long as other diarrhea tx

Answer 201

* invasive/inflammatory diarrhea * Produces **shiga toxin** which is neurotoxic, cytotoxic, enterotoxic * _Sources_: * fecal-oral, raw veggies * _s/sxs_: * abrupt onset of explosive, watery diarrhea that becomes progressively bloody * tenesmus, abd pain, +/- fever, chills, anorexia, malaise, HA * _Dx_: * Stool cx → positive fecal WBC/RBC * CBC with WBC \> 50,000 (**super high white count** * _Sigmoidoscopy_: **punctate areas of ulceration** * _Tx_: * fluids/electrolytes * severe diarrhea → **fluoroquinolones**

Answer 202

* (invasive/inflammatory diarrhea) * Entameba histolica → protoza rarely seen in the US, but seen in travelers from endemic areas * _Source_: * ingestion of cysts from food/water contaminated with feces * _S/sxs_; * asymptomatic but may develop **_liver abscess_** * _Dx_: * O&P shows cysts and RBCs * → cysts are not consistently shed so must obtain samples on 3 different days * ELISA (rapid & highly sensitive) * _Tx_: * **Metronidazole** or **tinidazole**

Answer 203

* _General info_: associated with immunocompromised pts * -fungus * transmission: fecal-oral * **Most common cause of chronic diarrhea in AIDS patients** * _Dx_: stool O & P * _Tx_: ketoconazole, control the AIDs

Answer 204

* Non-inflammatory/non-invasive diarrhea * **Most common cause of gastroenteritis in adults** * _Source_: * fecal-oral route, contaminated food.water, fomite → associated with **cruise ships**, hospitals, and restaurants * _S/sxs_: * **_Vomiting_** + diarrhea * _Dx_: PCR * _Tx_: fluids/electrolytes

Answer 205

* Non-inflammatory/non-invasive diarrhea * **Most common cause of diarrhea in unvaccinated infants and young children** * _source_: * fecal-oral route, **contaminated food/water**, fomite → associated with outbreaks in childcare centers * _S/sxs_: * child presenting with fever, vomiting and non-bloody diarrhea * \*\***Associated with severe dehydration in babies\*\*** * _Dx_: PCR * _Tx_: * fluids/electrolytes * *Prevention*: **Rotavirus vaccine**

Answer 206

* Non-inflammatory/non-invasive diarrhea * **Most common cause of traveler’s diarrhea** * _Source_: * drinking water/ice, washed unpeeled fruit * _S/sxs_: * abrupt onset of symptoms of non-inflammatory diarrhea * _Dx_: * Stool cx, gram stain * _Tx_: * usually self limited but can prescribe **cipro +/- loperamide** or bismuth

Answer 207

* Non-inflammatory/non-invasive diarrhea * Exotoxin → hypersecretion of water/ions → severe dehydration→ shock and death * _Risk factors_: * overcrowding in areas of poor sanitation * occurs in outbreaks usually in developing countries * _Source_: contaminated food or water * _S/sxs_: * voluminous watery diarrhea * rice water stools, mucus flecks, no WBCs No fecal odor, blood, or pus * SECRETORY DIARRHEA : inhibition of water, sodium and chloride absorption * progressing to **SHOCK**/dehydration within hours, NO FEVER * _PE_: * rice water-like stools * _Dx_: * stool cultures show gram negative, comma shaped rods * _Tx_: * Oral rehydration solution * **Doxycycline or cipro** for mod-severe cases

Answer 208

* Non-inflammatory/non-invasive diarrhea * **aka. the 24 hour stomach bug** * _source_: * processed/**deli meats**, hotdogs, soft cheese, pates * _s/sxs_: * acute, watery diarrhea * _tx_: * oral rehydration, self-limited * Except in pregnancy → **STAT Ampicillin** **(preggos told not eat those foods to avoid the risk)**

Answer 209

* Non-inflammatory/non-invasive diarrhea * **MOST common intestinal protozoan parasite in the US** * _Source_: * streams and wells, constamined still water * → associated with **beavers** * _S/sxs_: * frothy/greasy/foul smelling diarrhea (Steatorrhea) * weight loss and low-grade fever is common * trophozoite attachment can cause inflammation and villus damage in small intestine and extremely large numbers may lead to a **direct physical blockage** of nutrient uptake such as Vitamin B12 * _Dx_: * O & P cx: trophozoites/cysts in stool * _Tx_: * Oral rehydration + **metronidazole**, **tinidazole****,**or**albendazole**

Answer 210

* _Risk Factors_: Antibiotics suppress normal flora → especially **Clindamycin** in adults, **amoxicillin** in children * _complications:_ * toxic megacolon * _S/sxs_: * **Mild disease:** * **Water diarrhea \> 3x/24 hours (Cardinal symptom)** * cramping, abd pain * +/- fever, bloody or mucoid stools, anorexia * **Severe disease**: (plus the above) * diffuse abd pain & distention * hypovolemia * lactic acidosis * marked leukocytosis * _Dx_: * stool testing (PCR, nucleic acid amplification test) * _Tx_: * CONTACT PRECAUTIONS * **oral vanco** (IV is not effective) * **Fidaxomicin** = also expensive but $$$$ * **Metronidazole** = 1st line in children

Answer 211

* INFLAMMATORY BOWEL DISEASE * _Where_: can occur anywhere in GI tract, from mouth to anus (**RECTAL SPARING**) **most common = terminal ileum!!** * _Who_: * Females \> males, _ashkenazi Jews_ (Eastern European Jewish Ancestry) * _Bimodal Peaks_: 15-30, 60-70 * _S/sxs_: * abd pain (Esp RLQ due to terminal ileum being most common) * diarrhea (usually not bloody) * **weight loss** **(lots of weight loss)** * vitamin deficiency * **aphthous ulcers** * _Complications_: * fistulas, abscess, bowel perf, granulomas * _Dx_**_:_** * **Colonoscopy/Endoscopy** → **Transmural** lesions, **skip lesions, cobblestoning. (DIAGNOSTIC)** * Labs Cannot diagnose but can point in right direction: * +ASCA * ESR/CRP (vague identification) * _Tx_: * **Mesalamine (nsaid)** for mild disease * steroids for acute flairs only * then 6MP/AZA, then anti-TNF = immunomodulators → tend to be most successful txs * Surgery is NOT curative in crohn’s (only used when meds fail)

Answer 212

* INFLAMMATORY BOWEL DISEASE * ONLY OCCURS IN THE COLON : Begins in rectum and then spreads through the colon * M = F, most common = caucasian, but increasingly fast in asian/hispanic populations * _S/sxs_: * abd pain (most commonly in LLQ) * tenesmus * **bloody diarrhea** → due to ulcerations * -mucoid stools * prei-anal tags * _Complications_: * toxic megacolon * primary sclerosing cholangitis, colon cancer: need to get an annual colonoscopy starting 10 years after initial diagnosis * _Dx_: * **Sigmoidoscopy** → uniform inflammation starting in the rectum, submucosal ulcerations, pseudopolyps * **Colonoscopy & barium enema CT in acute disease** * Labs: * P-ANCA + * ESR/CRP * Barium Studies → loss of haustral markings (**lead pipe sign**) * _Tx_: * **smoking helps in ulcerative colitis** → decreases immune response * **Sulfasalazine** works in colon only * steroids for acute flairs only * 6MP/AZA if sulfalasize does not work * Anti-TNF = immunomodulators → tend to be most successful treatments **Surgery = curative in UC** (only used when other tx fail)

Answer 213

* _Definition_; * chronic, functional, **idiopathic pain disorder** with no organic cause, not an inflammatory process * _Epidemiology_: * onset most common in late teens, early twenties **Most commonly diagnosed GI illness (10-15% of population** * _Risks_: * **gastric bypass surgery** * _S/sxs_: * **abd pain:** postprandial (occuring after a meal) * **altered bowel habits**: diarrhea, constipation, or alteration between both * **abd distention** (Gas pockets) * _Alarm sxs:_ * Gi bleed * anorexia/weight loss * fever * family hx of GI cancer, IBD * persistent diarrhea → dehydration * severe constipation/impaction * _PE_: * \*Normal * **stable weight** (vs IBD which has weight loss)

Answer 214

* _Dx_: * **Diagnosis of exclusion** **(after work-up with colonoscopy, abd CT)** * **_Rome IV Criteria_:** * \***Recurrent abd pain at least 1day/week for at least 3 months with ⅔ criteria:** * related to defecation * onset associated with change in stool frequency * onset associated with change in stool form * _Tx_: * lifestyle & dietary changes (low **FODMAP**) are first line (low fat, high fiber, unprocessed foods) * avoid drinks with sorbitol, or fructose * sleep, exercise, smoking cessation _Constipation symptoms_: - prokinetics: fiber, psyllium polyethylene glycol - No response: lubiprostone, linaclotide _Diarrhea symptoms_: - **loperamide, eluxadoline,** **rifaximin,** **bile acid sequestrants** (cholestyramine) - Anticholinergics, antispasmodics (Dicyclomine)

Answer 215

rash, intractable vomiting, liver damage, dilated pupils caused by taking **aspirin** after a **viral illness** or by kids **\<18 taking aspirin**

Answer 216

* _Definition_: acute viral infx of the liver due to HEV infx * _Transmission_: * **fecal-oral route**, unhealthy lifestyle, alcoholism * _S/sxs_: * pts are mostly asymptomatic * malaise, anorexia, fever, N/V, abd pain * _PE:_ * **Hepatomegaly** * **Jaundice** * _Dx_: * **LFTs**: elevated ALT, AST, bilirubin * **serology**: **Acute: anti-HEV** **IgM** * _Tx_: * **No tx needed** * **\*****HIGHEST****mortality due to fulminant hepatitis during****pregnancy****(esp 3rd trimester)\***

Answer 217

* _Definition_: * **autoimmune progressive cholestasis** → diffuse fibrosis of intra- and extrahepatic biliary ducts * _Risks_: * **Ulcerative colitis**, men 20-40yo * _S/sxs_: * **Jaundice, Pruritus****, Fatigue** * RUQ pain * hepatomegaly, splenomegaly * _Dx_: * **Increased alkaline phosphatase** & GGT * **positive P-ANCA** **(hallmark)** * **ERCP: beaded appearance of the biliary ducts** * liver biopsy: rarely used * _Tx_: * stricture dilation for symptom relief * Pruritus: **Cholestyramine** (binds to bile acids which are the cause of itchiness) * **Definitive = liver transplant**

Answer 218

* Most Common): Passage of intestine through the **internal inguinal ring down the inguinal canal,** may pass into **the scrotum.** Often congenital and will present before age one. * **Remember**: Indirect goes through the Internal Inguinal Ring (an “I” for an “I”)

Answer 219

Passage of intestine through the **external inguinal ring at Hesselbach triangle, rarely enters the scrotum**

Answer 220

Often from previous abdominal surgery, obesity. Abdominal mass noted at site of previous incision

Answer 221

Very common, generally is congenital and appears at birth. Many umbilical hernias resolve on their own and rarely require intervention. **Refer to surgery if an umbilical hernia persists \>2 years of life.**

Answer 222

* _definition_: * **catecholamine secreting adrenal tumor** which secretes norepi & epi autonomously and intermittently * 90% = benign, 10% = malignant * Associated with *neurofibromatosis type 1*, MEN 2A/2B, and Von Hippel-Lindau Disease * _etiology_: * women = men, but women have more symptoms * _S/sxs_: * Episodic: **5Ps** **→** * **1.Pressure (HTN)** * **2.Pain (headache)** * **3.Perspiration** * **4.Palpitations (tachycardia)** * **5.Pallor** * **TRIAD: HA, diaphoresis, tachycardia** * _Dx_: * **24-hour catecholamines (in urine)** including metabolites (metanephrine and vanillylmandelic acid) * MRI or CT of abdomen to visualize catecholamine secreting adrenal tumor * _Tx_: * **complete adrenalectomy** * Preoperative non-selective alpha-blockade: **phenoxybenzamine** **or** **phentolamine** **x 7-14 days followed by beta blockers to control HTN** * **\*\* Refer to Endo \*\*** * DO NOT initiate therapy with beta blockade to prevent unopposed alpha constriction during catecholamine release triggered by surgery, which could lead to life-threatening HTN

Answer 223

* young pts with HTN * family hx of pheochromocytoma * radiologic incidentalomas * shock after anesthesia, surgery, invasive procedure * other: **cardiomyopathy**

Answer 224

* _Primary_: * _etiology:_ * aldosterone-producing adenoma: 35% * bilateral idiopathic hyperplasia: 60% * unilateral idiopathic hyperplasia: 2% * **Most common cause of endocrine HTN!!!!!!!!!!!!** Prevalence: 5-10% * _Secondary_: * Pathophys: increased renin → increased aldosterone via RAAS * _etiology_: * renal artery stenosis, renal artery hypoperfusion * _S/sxs_: * **HTN**: HA or facial flushing * _When to consider testing for_ **_Primary Hyperaldosteronism:_** * HTN & hypokalemia * Resistant HTN (3+ drugs) * Adrenal incidentaloma + HTN * onset of HTN at young age \<30 * severe HTN (160/100) * any consideration of secondary HTN * _Dx of primary_: * plasma aldosterone concentration (PAC): high (\>15) * plasma renin activity (PRA): low (\<1) * **PAC-PRA ratio: \> 20ng/dL** * **Potassium: usually normal**, may be hypokalemic (but indicates very far along) * _Tx_: * **refer to endo** for further testing & eval → * oral salt loading test or IV saline infusion test * if hyperaldosteronism is established then adrenal CT or MRI may be done * **Spironolactone**: blocks aldosterone from binding to mineralocorticoid receptors in the renal CD (increases aldosterone level since it is completing with spironolactone for the receptor, but lower BP since it blocks the effect of aldosterone) * _Unilateral Adrenalectomy_: if adrenal vein sampling lateralizes site of overproduction

Answer 225

* _Definition_: * disorder resulting from excess growth hormone in **children** (when they have open growth plates) → excessive long bone growth * _S/sxs:_ * excessively fast growth in height and weight * DM * Carpal Tunnel Syndrome * _PE_: * macrocephaly, projection of lower jaw, soft tissue swelling, increased size of organs * _Dx_: * **Serum IGF-1 to screen**: must interpret ranges of age/sex * \*if positive then → **oral glucose suppression test**: 75g PO glucose → measure serum GH levels at 30 & 60 min (normal \<1g) * _Tx_: * refer to endo * **Surgery****: removal of active tumors** * Somatostatin analogs: **octreotide** or **lanreotide**, b/c somatostatin inhibits GH release

Answer 226

* _Definition_: * inability of the kidney to concentrate urine → production of large amounts of dilute urine caused by **no production of AVP** → water leaves collecting duct→ urine output increases * _Etiology_: * idiopathic, destruction of posterior pituitary, head trauma, CNS tumor, infx * _S/sxs_: * increased urinary frequency, polyuria, nocturia +/- enuresis, polydipsia * _PE_: * normal, may show signs of dehydration * _Dx_: * _Urine output_: increased (\>40mL/kg/24hours) * _Thirst_: increased * _Serum AVP_: low (\<1pg/mL) * _Urine Osmolality_: low (\<300 mOsm/L) * _Serum Sodium_: high * _Serum osmolality_: high * **Water deprivation Test**: patient produces large amounts of dilute urine (even though they aren't drinking water) * **MRI**: * evaluate posterior pituitary for **“****bright spot”****(if absent → likely central DI)** & pituitary stalk for trauma, hypophysitis, mass * _Tx_: * assess pt's ability to keep up with hydration * **endocrine referral: for discussion of water deprivation testing** and use of **DDAVP**

Answer 227

* _Definition_: * inability of kidney to concentrate urine → production of large amounts of dilute urine due to **partial or complete renal insensitivity to AVP** at the level of the kidney * _Etiology_: * congenital, pregnancy, meds (**lithium**, demeclocycline) * _S/sxs_: * urinary frequency * polyuria, nocturia +/- enuresis, polydipsia * **severe**: neurologic sxs of hypernatremia: confusion, lethargy, seizures, coma * _PE_: * normal (may show signs of dehydration: poor skin turgor, dry mucous membranes, drinking lots of water) * _Dx_: * _Urine Output:_ increased (**\>40** mL/kg/24 hours) * _Thirst_: increased * _Serum AVP:_ high (\>1pg/mL) * _Urine Osmolality_: low (\<300 mosmol/L) * _Serum Sodium_: high * _Serum Osmolality_: high * **\*\*MRI has no role in dx** * _Tx_: * **Thiazide diuretic** **(****hydrochlorothiazide****) &/or** **amiloride** **with low sodium diet** * identify cause: d/c offending med if possible * Nephro consult * Pregnant: consult with OB before meds

Answer 228

* _Definition_: * **excessive AVP production** that impairs urinary water excretion & predisposes the pt to **euvolemic hyponatremia** if water intakes is not reduced * _Pathophys_: * impaired urine dilution often caused by defect in osmotic suppression of AVP * _Etiology_: * malignancy, brain trauma, stroke, subarachnoid hemorrhage, PNA, lung CA, **meds** * _S/sxs_: * **Severe**: hyponatremia: confusion, lethargy, disorientation, seizures, coma * _PE_: * normal * weight gain: increased in total body water by up to 10% * _Dx_: * _Urine Output_: normal * _Thirst_: normal * _Serum AVP_: not measured * _Urine Osmolality:_ high (\>300 mOsmol/L) * _Urine Sodium_: high (\>40 mEq/L) * _Serum sodium_: low (125-130) * _Serum Osmolality_: low * _Tx_: * identify cause: d/x med if possible, look for malignancy & tx, look for lung (**small cell lung cancer**) or brain pathology & tx * **free water restriction (\<2 L/day)** * _endocrine or nephrology consult_ if no resolution: may need hypertonic (3%) NS infusion or **tolvaptan/conivaptan** **(V2 agonists)**

Answer 229

MCC of hyperthyroidism in the US * _Pathophys_: **autoimmune disease in which TSH-R ab target and STIMULATE** the TSH-R on the thyroid gland → increased in thyroid hormone production → hyperthyroidism * _S/sxs_: * s/sxs of hyperthyroidism * **Graves Orbitopathy:** **proptosis, exophthalmos, lid lag****, diplopia** * **Graves dermopathy:** **pretibial myxedema** (swollen red or brown patches with non-pitting edema) * _PE_: * **Diffusely enlarged but non-tender goiter/thyroid** * **Thyroid Bruit** * _Dx_: * **Decreased TSH, Increased T4** * **TSH-Receptor Ab: positive** * Thyrotropin binding inhibitor immunoglobulin (TBII): positive * Thyroid stimulating immunoglobulin (TSI): positive * **Thyroid Uptake & Scan: diffuse iodine uptake that is HIGH** * _Tx_: * Radioactive iodine (131Iodine Ablation): MOST COMMON * Antithyroid drugs: **Methimazole** or **Propylthiouracil** * Surgery: total thyroidectomy * beta blockers to alleviate tremor: **Propranolol** * **Smoking cessation** (tobacco worsens TED: thyroid eye disease)

Answer 230

* aka **Autoimmune Lymphocytic Thyroiditis** * _Definition_: * **autoimmune lymphocytic infiltration** of the thyroid → atrophy of thyroid follicles & fibrosis. 90% of thyroid gland gets destroyed before overtly become hypothyroid * _Triggers_: * pregnancy, radiation exposure (external beam radiation, nuclear disasters), medications * **Most common cause of hypothyroidism in the US.** **Women** \> men * _S/sxs_: * s/sxs of hypothyroidism * _PE_: * thyroid gland may be normal or atrophic/hypertrophic * bradycardia * loss of outer third of eyebrow * myxedema * _Dx_: * **Increased TSH + decreased Free T4** * **Thyroid Peroxidase (TPO) Ab: Positive (90%)** * Anti-thyroglobulin Ab: positive * Thyroid US: **heterogeneous echotexture “ patchwork quilt”** * pseudonodules, septations, mildly enlarged anterior cervical lymph nodes * Thyroid Uptake & Scan: diffusely decreased iodine uptake * _Tx_: * **Levothyroxine** **= first line tx** * Synthetic T4, SEs = osteoporosis & CV effects * Monitor TSH levels at 6 week intervals when initiating or changing dose

Answer 231

* _Definition_: * untreated congenital hypothyroidism * _Etiology_: * lack of maternal **iodine** during fetal development in developing countries, dysgenesis of the thyroid gland, acquired (TSH-R Ab passed across placenta) * _S/sxs_: * developmental delays * short stature * hypothyroid sxs * goiter sxs: hoarseness, dyspnea * _PE_: * **coarse facial features, macroglossia, umbilical hernia** * hypotonia (decreased DTRs) * jaundice, feeding problems * _Dx_: * TSH = increased * T4/T3 = decreased * _Tx_: * **Levothyroxine** (synthetic T4)

Answer 232

* _Definition_: * autoimmune disease causing absolute (to near absolute) **insulin deficiency** due to **pancreatic beta cell destruction** **→** uncontrolled blood sugar & ketoacids. * _Pathophys_: * Genetic predisposition with **HLA markers** + exposure to trigger → autoimmune response directed against pancreatic islet cells → # of beta cells decreases→ progressive impairment in insulin release results in DM when **≥ 80% of mass destroyed**; honeymoon phase is first 1-2 years after onset of DM associated with reduced insulin requirements * _Stages_: * STage 1: genetic predisposition * Stage 2: beta cell injury after immune trigger with multiple antibody positive * Stage 3: diabetes, beta cell mass \< 80% * _S/sxs_: * **polyuria, polydipsia, polyphagia** * fatigue, poor wound healing, **weight loss** * blurred vision

Answer 233

* _Screening_: * only recommended in the setting of a research trial or in 1st degree family members of a probland with T1DM * _Labs_: * Anti-Islet Autoantibodies * HLA: genetic markers * DR & DQ * _Criteria for Dx of T1DM_: * Presence of 1+ autoimmune markers: islet cell autoantibodies, insulin autoantibodies, GAD (GAD 65 or glutamic acid decarboxylase antibody), IA-2 (tyrosine phosphatase antibodies), ZnT8 (zinc-transporter 8) * _Management_: * multiple daily injections of prandial & basal insulin or continuous SQ insulin infusion * Use of rapid-acting insulin to reduce hypoglycemia risk (**Glulisine, Aspart, Lispro)** * Education pt on how to match prandial insulin doses to carb intake, premeal blood glucose & anticipated physical activity * **Pramlintide** can be added to insulin for A1C reduction & weight loss * SGLT-2 not currently recommended & may increase ketoacidosis * _When to monitor_: * blood glucose monitor: * before meals, before bedtime, 2 hours postprandial if testing insulin dose response

Answer 234

* **BMI ≥ 25 with 1+ Risk Factors**: * 1st degree relative with DM * high-risk race * CVD, HTN, hyperlipidemia * PCOS (polycystic ovarian disease) * physical inactivity * Prediabetes: test yearly * Women with gestational diabetes: test q 3 years for the rest of their life * **All pts ≥ 45 yo q 3 years** * Youth \> 85th percentile weight with 1+ risk factor

Answer 235

* _Definition_: * **painful menstruation** that affects normal activities * _Types_: * **Primary: due to increased** **prostaglandin** **(PGF2a) production by endometrium** → painful uterine wall contractions; teens/early 20s * **Secondary: due to pelvis or uterus pathology** (i.e. endometriosis, PID, adenomyosis, leiomyomas (fibroids)); common with increased age * _S/sxs_: * recurrent cramping at the midline of the lower abdomen **at the onset of menses** that gradually diminishes over 12-72 hours (*primary*) or throughout menses (*secondary*) * _PE_: * \*\*Usually normal * **Normal pelvic exam, no physical findings that support symptoms →** **primary dysmenorrhea** * Asymmetry or irregular uterine enlargement → fibroid (leiomyomas) * Tender, asymmetrical enlarged “boggy” uterus → adenomyosis * Cervical Motion Tenderness, adnexal pain, inguinal lymphadenopathy, fever, purulent cervical drainage → PID * _Dx_: * Clinical diagnosis * Labs/imaging may be needed if pelvic disease is suspected * _Note_: childbearing has no effect on primary or secondary dysmenorrhea * _Tx_: * Supportive: hot compresses * **Primary**: **NSAIDs** * -Estrogen-Progestin OCPs * -Laparoscopy: if unresponsive to 3 cycles of initial therapy to r/o secondary causes * **Secondary**: **tx the underlying condition** * (ex. Combined OCPs for endometriosis)

Answer 236

* _Definition_: cluster of physical, behavioral, and mood changes with cyclical occurrence during the of the **luteal phase** menstrual cycle * _Epidemiology_: * **very common** (premenstrual symptoms in 75-85% of women, 5-10% have PMS) * _S/sxs_: * **\*****symptoms occurring 1-2 weeks before menses & relieved with onset of menses** * Physical: abd bloating, fatigue, breast tenderness/swelling, weight gain, headache, changes in bowel habits * Emotion: **irritability**, tension, depression, anxiety, libido changes * Behavioral: food cravings, poor concentration, noise sensitivity * _PE_: * have pts record a diary of symptoms for multiple cycles * _Dx_: * subjective Dx * _PMDD Criteria_ * _Tx_: * Lifestyle mods: stress reduction & exercise, reduction of caffeine/EtOH/salt * **PMS:** **NSAIDs** **(for dysmenorrhea), OCPs (1st line for some)** * **PMDD:** **SSRIs** (fluoxetine, sertraline, citalopram) are gold standard

Answer 237

* \*5 of the following with at least 1 from the first 4 1. Depressed mood or hopelessness 2. anxiety or Tension 3. Lability (strong emotions) 4. Increased or Persistent Anger, irritability of conflicts * decreased interest, difficulty concentrating, lethargy, change in appetite, hyper/insomnia, overwhelmed

Answer 238

* _Definition_: * **failure of menarche onset by age 16** (in the presence of secondary sex characteristics) or by age 13yo (in the absence of secondary sex characteristics) * _S/sxs_: * No menstrual bleeding * _PE_: * consider eval by age 15 or no menstruation within 3 years of thelarche (onset of breast development) * _Dx_: * hCG to r/o pregnancy if sexually active * FSH, TSH, Prolactin * _Tx_: * tx the underlying problem

Answer 239

* _Definition_: * **absence of menses for \>3 months** in a pt with a previously normal menstruation cycle or for the duration of 3 typical menstrual cycles for the patient with oligomenorrhea * _Etiology_: * **Pregnancy = MCC (95%)** * **Hypothalamus dysfx: function** (poor nutrition/stress alters feedback to the brain that regulates the menstrual cycle), drug induced, psychogenic (anorexia), head injury * **Pituitary Dysfnx**: prolactinoma or pituitary infarct (Sheehan syndrome) * **Ovarian Dysfnx**: decreased estrogen & increased LH/FSH; PCOS, premature ovarian failure, Turner Syndrome, savage syndrome * **Outflow tract dysfxn**: **Asherman’s syndrome (**acquisition of scar tissue in uterus that causes blockage often d/t surgery or cancer tx) , imperforate hymen, no uterus/vagina * _S/sxs_: * no menstrual bleeding * _Female Athlete Triad_: * -hypothalamic amenorrhea, eating disorder, osteoporosis * \*Note: you need to be ~115 lbs to maintain cycle * _Dx_: * hCG = 1st test to r/o pregnancy * \*if hCG negative then order: * → serum prolactin, FSH, LH, TSH, estrogen * → testosterone if evidence of hirsutism or hyperandrogenism

Answer 240

* _Definition_: * unexplained abnormal bleeding in nonpregnant woman in regard to quantity, frequency, or duration. **Usually occurs just after menarche or in perimenopausal period** * _Etiology_: * Pregnancy * **Uterine-Structural** (**PALM)**: polyp, adenomyosis, leiomyoma, malignancy (any postmenopausal bleed is cancer until proven otherwise) * **Uterine-nonstructural** (**COEIN**): coagulopathy (von Willebrand), ovulatory dysfunction (PCOS, stress, adrenal hyperplasia, thyroid probs), endometrial, iatrogenic (OCPs, warfarin), not yet known/classified * _S/sxs_: * abnormal bleeding * _Types_: * **Anovulatory = most common**: unpredictable bleeding with variable flow/duration d/t chronic estrogen production unopposed by progesterone → endometrium proliferation that eventually outgrows its blood supply & is necrotic * **Ovulatory**: cyclic but heavy/prolonged bleeding d/t uterine structural conditions, normal gonadotropin/sex steroid levels * _PE_: * Skin: hirsutism, acne, striae, acanthosis, nigricans, easy bruising * Breast: galactorrhea * Thyroid * Abdomen: uterine fibroids, enlarged uterus, splenomegaly * Pelvic: atrophic changes, cervicitis, cervical carcinoma * _Dx_: * **hCG to r/o pregnancy** * _3 question algorithm?_ * Is she pregnant? * Is the bleeding from the uterus or elsewhere? * If bleeding is from the uterus, is she ovulating or having anovulatory bleeding? * CBC: hgb & hct * TSH, PAP screen, Prolactin, FSH/LH, STI screen, serum androgens & testosterone: if hirsutism * Endometrial biopsy to r/o endometrial biopsy to r/o endometrial carcinoma in all women \> 35 yo with obesity, HTN, or DM * \*if ovulatory: do US to check for polyps or fibroids * _Tx_: * **Tx the underlying etiology** (i.e. surgery for structural lesions) * **Progestin based treatment**: **medroxyprogesterone acetate** **x 10 days** (1st line), OCPs or IUD as alternative * Acute heavy menstrual bleeding: high-dose estrogen & progestin therapy OR OCP 3 pills/day x1 week + Tranexamic acid → ongoing therapy with IUD, OCPs, progestins, tranexamic acid, & NSAIDs

Answer 241

* Any post-menopausal bleeding * 45 yo to menopause: any AUB that is frequen, heavy or prolonged * \< 45 yo: any AUB with **chronic ovulatory dysfunction**, exposure to estrogen unopposed by progesterone, failed medical management bleeding, Lynch or Cowden syndrome * **Premenopausal: with amenorrhea/anovulatory \> 6 months**

Answer 242

* _Definition_: * tear of the anterior cruciate ligament * _Mechanism_: * **deceleration, hyperextension**, non-contact pivoting injury * _General Info_: * **Most common knee ligamental injury**. 70% sports related & more common in females. Usually **accompanied by a MCL or meniscus tear.** * _S/sxs:_ * \*\*Sudden onset * Knee pain associated with “**pop” & swelling** followed by **hemarthrosis** * may develop knee buckling, inability to bear weight * _PE_: * **Lachman test: most sensitive**, knee placed at 20 degrees in supine & tibia is pulled forward to test laxity * **Anterior Drawer Test**: knee placed at 90 degrees while pt is supine & tibia is pulled forward to test laxity * **Pivot Shift test:** knee is internally rotated, valgus force is applied, & knee is slowly flexed * _Dx_: * Xray: initial test to r/o fracture, usually only positive for effusion, **segond fracture** **is pathognomonic for ACL tear** (avulsion of lateral tibial condyle) * MRI: best test to access ACL tear * **_Unhappy triad_**: injury to ACL, medial collateral ligament, medial meniscus * _Tx_: * \*\*Most patients require surgery * non-operative: protected weight bearing, hinged knee brace, early gentle AROM, PT, NSAIDs * **Surgery: ACL reconstruction (not repair)** * → long rehab

Answer 243

* _Definition_: * tear of the posterior cruciate ligament. PCL is the primary restraint to posterior motion of the tibia * _Mechanism_: * **Direct anterior blow, fall on flexed knee,** **dashboard injury** (anterior force to proximal tibia with knees flexed) * _S/sxs_: * **Posterior knee pain** * **Anterior bruising** * Large effusion * instability bearing weight * _PE_: * **Posterior Sag:** patient’s hip flexed to 90 degrees while examiner support leg under lower calf & looks for posterior sag of the tibia * **Posterior Drawer Test**: posterior translational movement of the tibia * _Dx_: * MRI = best test to access PCL tear * _Tx_: * **\*Most pts do NOT require surgery** * Non-operative: RICE therapy, NSAIDs, knee immobilization * Surgery: may be necessary if acute or associated with multiple injuries

Answer 244

* _MCL_: * resists **Valgus force** on the knee * _Mechanism_**_:_** * **Lateral Trauma** * **MCL injury more common than LCL** * _S/sxs_: * localized medial knee pain, swelling, ecchymosis, stiffness * pain & laxity with **valgus stress** * _PE_: * evaluate for associated injuries and assess stability * palpate for entire ligament, but most injuries occur at midpoint * apply valgus/varus stress * **McMurry, Apley, Ober, Thessaly** * _Dx_: * _MRI:_ can detect collateral ligament injuries & severity * _Grading_: * -Grade 1: stretch, no tear * -Grade 2: partial tear * -Grade 3: complete tear (unrestricted motion) * _Tx_: * usually do not need surgery (instability is uncommon) * **incomplete tear (grades 1-2)**: pain control, PT, RICE, NSAIDs, knee immobilization * **complete tear (grade 3)**: surgical repair

Answer 245

* _LCL_: resists **varus force** on the knee * _Mechanism_: * **medial trauma** * _S/sxs_: * localized lateral knee pain, swelling, ecchymosis, stiffness * pain & laxity with **varus** stress * assess stability * palpate for entire ligament, but most injuries occur at midpoint * apply valgus/varus stress * **McMurry, Apley, Ober, Thessaly** * _Dx_: * _MRI:_ can detect collateral ligament injuries & severity * _Grading_: * -Grade 1: stretch, no tear * -Grade 2: partial tear * -Grade 3: complete tear (unrestricted motion) * _Tx_: * usually do not need surgery (instability is uncommon) * **incomplete tear (grades 1-2)**: pain control, PT, RICE, NSAIDs, knee immobilization * **complete tear (grade 3)**: surgical repair

Answer 246

* _Definition_: * tear of the medial or lateral meniscus. Menisci are fibrocartilaginous pads that function as shock absorbs between the femoral condyles & tibial plateau * _Mechanisms_: **Twisting Injury** * Medial is more common than lateral because the **lateral meniscus is smaller & less mobile** * _S/sxs_: * **Popping**, “giving way” during ambulation, climbing or descending stairs * locking or catching * **Effusion** after activities * Pain along the medial or lateral joint line worse with twisting or squatting * _PE_: * **Most common finding: joint tenderness over the medial or lateral joint line** * **McMurray sign**: pop or click when the knee is flexed & then externally rotated & extended * **Apley test**: pt is prone with knee flexed to 90 degrees then rotate knee medially & laterally, check for pop/click/pain * **Thessaly test: most sensitive**, pain/locking at medial or lateral joint with patient standing on affecting leg, knee flexed to 20 degrees, twist side to side x **3 times** * _Dx_: * MRI: most sensitive & specific * Transverse tear = most amenable to repair because it has best blood supply * _Tx_: * **\*May heal without surgery but follow closely** * *_Non-operative_*: Abstinence from weight bearing, rest with knee flexion, ice, compression dressing, NSAIDs * *_Surgery:_* indicated if young patient, high energy injury, repeat injury, large effusion, mechanical symptoms, high demand occupation, failure to improve * **arthroscopic repair**: **\<3mm**, stable, recent injury, young patient, later return to play * **partial meniscectomy**: **\> 3mm**, mobile, old injury, older patient, sooner return to play

Answer 247

* _Definition_: * avulsion fracture of the proximal tibial lateral plateau margin * associated with **anterior cruciate ligament** & posterolateral corner knee injuries

Answer 248

* _Definition_: * patella is tilted laterally or predisposed to lateral subluxation * _S/sxs_: * retropatellar pain exacerbated with stairs * pain with prolonged sitting * _PE:_ * Gait: patella pointed inward with knock-knee alignment * _Dx_: * Radiography: lateral patellar subluxation or lateral patellar tilt * _Tx_: * Exercises to strengthen quads, elastic braces, PT * Srugery

Answer 249

* _Definition_: * constellation of problems characterized by diffuse, aching anterior knee pain that increases with activities that place additional loads across the patellofemoral joint. No clear trauma, malalignment or instability * _Etiology_: * multifactorial, overuse & overloading of the patellofemoral joint * _Presentation_: * **adolescent female****, recent increase in activity level** * _Risks_: * running, squatting, climbing stairs, **dynamic valgus**, females, foot abnormalities (**p****es planus**→ flat feet), overuse or sudden increase in physical activity level, patellar instability,**quad (VMO) weakness, hyperlaxity syndrome** * _S/sxs_: * **Disabling** **anterior knee pain** behind or around the patella, **worsened with knee hyperflexion (squats, stairs)** * Sense of instability or retropatellar catching sensation * _Pe_: * Compression of the patella during knee extension will produce symptoms or anticipated pain * **Patellar grind test**: patient in supine position with knee extended, examinar displaces the patella inferiorly into the trochlear groove, asks patient to contract quads while examiner palpates patella → pain * _Patellar instability test_: * bend knee 20-30 degrees & place finger inside patella to see how far you can displace it laterally * _Dx_: * good hx and thorough exam for dx * Xray: **Merchant’s View** * _Tx_: * \*usually self-limiting * **Activity modification**: no jumps, squats, or running * -PT: strengthen VMO * -Icing after physical activity & NSAIDs * -Brace/sleeve/tape: for patellar stabilization * -Arch Supports * \*Refer if sxs do not improve (may need surgery)

Answer 250

* _Definition_: * overuse or overload syndrome involving either the quadriceps tendon at its insertion or the patellar tendon at its insertion * _Risks_: * **young adults** **involved in jumping or kicking sports** (“Jumper’s Knee”) * _S/sxs_: * -**Anterior Knee Pain (Hallmark) that begins immediately after exercise** * -Exacerbated by climbing, descending stairs, running, jumping, or squatting * _PE_: * Tender to palpation at the bony attachments of the quadriceps tendon or patellar tendon * Increased heat, mild swelling & soft tissue crepitus * Normal motion but painful * _Dx_: * Xray: usually normal, but may show small **enthesophytes** (calcifications of the tendinous insertions) * _Tx_: * Goals: rest & pain control, restoring pain-free range of motion/strength, resuming activities * Period of relative rest, NSAIDs, analgesic creams, Ice/heat * \*patients with a possible rupture of the extensor mechanism require prompt referral to ortho

Answer 251

* _Definition_: * inflammation of the patellar tendon at the insertion of the **tibial tubercle**. * _Etiology_: * **overuse** or small avulsions from repetitive knee extension & quadriceps contraction * _Risks_: * **10-15 yo males****, during growth spurts, athletes** * _S/sxs_: * **Anterior knee pain & swelling** **worse with activity****, relieved with rest** * _PE_: * prominence, swelling, & **tenderness to the anterior tibial tubercle** * _Dx_: * Imaging not needed in classic presentation (bilateral) * Xray: may be normal; elevation, ossification, &/or bone fragmentation of the tibial tuberosity * _Tx_: * \*\*Resolves in 12-24 months * **Conservative: avoid provocative activities**, RICE, NSAIDs, quadricep stretching * Surgery: if refractory, after growth plate closure

Answer 252

* _Definition_: * **form of arthritis that primarily affects the spine** * _Pathophys_: * inflammation of the spinal joint → new bone formation in the spine (ankylosis) →**sections of the spine fuse** in a fixed, immobile position * _Risks_: * **young males 15-30 yo****,****HLA-B27 gene** * Can also cause inflammation, pain, & stiffness in other areas of the body (shoulders, hip, ribs, heels, & small joints), as well as uveitis. * **Hallmark feature of AS is** **involvement of the SI joints****.** * **“Forward stooping” : shopping cart presentation** * _S/sxs_: * \*\*insidious onset (\> 3 months) * **pain & stiffness in the lower back & hips****: worse in the morning**& after periods of inactivity,**decreases with activity** * **Limited ROM of spine** * -Inflammation of multiple areas of the body (eyes, shoulder, ribs) * -**Bilateral SI joint inflammation** * -Stooped (hunched over) posture due to early compression fractures * -Fatigue, fever, weight loss * -Enthesis involvement: soft tissue pain (Achilles tendon) * -Acute uveitis, IBD * _Dx_: * Spine xray: **bamboo spine** (straightening of the spine + fusion of the vertebrae), sclerosis, erosions * SI Joint xray: **sacroiliitis** (narrowing of the joint) * MRI, CT, Increased ESR/CRP * _Tx_: * \*for symptomatic only * **NSAIDs** **= first line** * -TNF blockers: if no response to NSAIDs. → **Remicade (infliximab), Enbrel (etanercept), Humira (adalimumab), Cimzia (certolizumab pegol) and Simponi (golimumab)** * PT: to increase ROM & flexibility * Joint replacement surgery (THRA)

Answer 253

* _ASAS Classification_: * \*Sacroiliitis on imaging + 1 SpA feature or HLA-B27 (+) + 2 SPA * **SPA:** back pain, arthritis, enthesitis, uveitis, dactylitis, psoriasis, Crohn’s, family hx, HLA-B27, elevated CRP * _Complications_: * Predisposes patients to major fracture/dislocation injuries “**chalk stick fractures**”: spine becomes very stiff so if the patient falls or has trauma → **snaps like a chalk stick**

Answer 254

* _Definition_: * condition due to damage of the cauda equina. (cauda equina affects bowels/bladder) * _Etiology_: * **herniated disc = most common**, spinal stenosis, cancer, trauma, epidural abscess, epidural hematoma * _Anatomy_: * cauda equina is a bundle of nerves at the end of the spinal cord before it splits into the nerve roots. It contains nerves that innervate the pelvic organs & lower limbs (both sensory & motor components) * _S/sxs_: * \*\*RAPID ONSET * **severe lower back pain** * severe radicular pain into lower extremities (unilateral or bilateral) * loss of motor funcx/sensation of lower extremities * **loss of bowel/bladder funx** * loss of sexual function * _PE_: * Neuro exam * **Rectal exam: reveals “****saddle anesthesia****”** &/or **loss of rectal tone** * _Dx_: * **STAT MRI (noncontrast) of the lumbar spine** * CT myelogram: if patient cannot have an MRI * _Tx_: * **MEDICAL EMERGENCY** * **Immediate compression surgery** * If surgery absolutely must be held (ie patient is on an anticoagulant that cannot be reversed) → start IV steroids ASAP to reduce nerve inflammation

Answer 255

* _Definition_: * abnormal S-shaped or C shaped **curvature of the spine** * _Etiology_: * **most are unknown** * **-occurs during the growth spurt just before puberty**, muscular dystrophy, cerebral palsy, trauma, infection, birth defects, tumors * _Risks_: * males = females (but females are more likely to need surgery) * _S/sxs_: * \*usually identified during school screening or routine exam * **back pain** * \*severe curves can reduce the amount of space within the chest → difficult for lungs to funx properly & harder for the heart to pump → **cardiopulmonary decompensation** * _PE_: * uneven shoulders, uneven hips, head not centered on pelvis * obvious curvature of spine * **Adams forward bend test**: most sensitive, palpate spine + evaluate height of shoulders & iliac wings * _Dx_: * **Xray (36” cassette): full spine scoliosis films q 4-6 months,** **Cobb’s angle \>10 degrees** * MRI: if rapid curve progression * CT * _Tx_: * most are mild & don't require surgery * _Mild_: observation * _Moderate-Severe_: **scoliosis brace** to stop progression (custom molded, needed until bones stop growing, worn day & night) * _Surgical correction_: if curve progression worsens, neuro deficit present, or **difficulty breathing** ; growing rod if progressing rapidly at a young age * _Adults_: * NSAIDs, water therapy, aerobic activity, strength exercises, surgery (rare)

Answer 256

* _Definition_: * **separation of the pars interarticularis** (a small bony arch in the back of the spine between the facet joints) * _Risks_: * **athletes,** **adolescent males** (involvement in strenuous activity at a young age) * _Etiology_: * unknown but contributed to by physical sports, repetitive activity, & obesity * **Most common at L5-S1** (but can occur in the cervical vertebrae) * _S/sxs_: * \*\*Most are asymptomatic * **Low back pain**: unilateral, **aggravated with lumbar spine hyperextension** * _Dx_: * **Oblique radiograph**: **Scotty dog sign** **= 1st line** * Lateral radiograph: radiolucent defect in pars * MRI or CT (non-contrast for both) * bone scan * _Tx_: * **Activity restriction for 3-6 months** * **Anti-lordotic brace** * PT for core strengthening * Spinal fusion: for severe spondylolisthesis/instability

Answer 257

* _Definition_: * anterior, posterior, or lateral slippage of one vertebra over another → central or foraminal stenosis. Usually secondary to spondylolysis * _Etiology_: \*\*Many types * Degenerative: facet arthritis, joint remodeling * -Traumatic: rare, results from acute fractures * -Dysplastic: congenital abnormalities * -Isthmic: defect in pars interarticularis * -Pathologic: infx or malignancy * -Post-surgical: complications * _S/sxs_: * **low back pain** * Nerve compression: * radicular pain * -weakness of lower extremities * -numbness & tingling of lower extremities * -Loss of bowel/bladder function * -Hyperreflexia * _Grading_: * Grade 1: 25% slip * -Grade 2: 50% slip * -Grade 3: 75% slip * -Grade 4: 100% slips * -Grade 5: completely off * _Dx_: * Xray: slipping of vertebrae, lateral views can measure slip angle * MRI (non contrast) for neuro sxs * _Tx_: * Unstable but can heal in a slipped state * **Conservative: activity restriction, brace, PT** * Nerve sxs: steroids, epidural steroid injections, neuropathic pain meds * Spinal fusion: for unstable or refractory sxs * **Grade 2+ = requires surgery** * **Grade 1 = observation**

Answer 258

* Most common joint to dislocate. **Anterior is the most common type** of dislocation (95%) * _Risks_: young male with violent force/trauma (sports), older patient with lower mechanism * _PE_: * **Distal neurovascular eval _before**_ & _**after_ reduction** * -**sulcus sign**: elbow grasped and traction applied inferiorly→ depression below the acromion) * _Tx:_ * **conscious sedation, reduction & immobilization** (keeping upper extremity internally rotated across chest) * -Repeat neurovascular exam * -Post-reduction x-rays * -PT & referral to ortho * _Complications_: * -**axillary nerve injury**: this is why we do a neurovascular exam

Answer 259

* _Mechanism_: * most common after a blow to an abducted, externally rotated, & extended extremity * _S/sxs_: * shoulder pain * _PE_: * Inspect: arm held in **abduction & external rotation**, loss of deltoid contour * Palpate: sunken anterior soft tissues +/- humeral head palpable * _Dx_: * _Xray_: \*\*STAT * **\*axillary & scapular “Y” view** * -Hill-Sachs lesion: groove fracture of the humerus * -Bankart lesion: glenoid rim fracture * _Tx_: see glenohumeral dislocations overview

Answer 260

* _Mechanism_: * result from a fall onto the tip of the lateral shoulder with the arm tucked in (bicycle, football) * _Anatomy_: * the acromioclavicular (AC) ligament provides horizontal stability, the coracoclavicular (CC) ligament provides vertical stability * _Types_: * Type I: AC/CC intact, no separation * -Type II: Partial AC/CC intact, some widening * -Type III: both AC/CC torn, complete separation * -Type IV-VI: involves deltoid, trapezius, periosteal injury (rare) * _S/sxs_: * pain over AC joint, **pain with lifting arm,** obvious deformity with types III-IV * _PE_: * pt supporting arm in adducted position * **tenderness to palpation, swelling, & deformity over AC joint** * crepitus, instability * _Dx_: * xray: * mild separations (may need to compare both shoulders( * with type I → strain, so xray will be normal * _Tx_: * Type I-II: sling immobilization x 2-3 weeks, ice, NSAIDs, activity modification, recheck before resuming sports * -Type III: may respond to conservative tx, but if young/heavy demand patient consider surgery * -Type IV-VI: surgical referral

Answer 261

* **Most common bone injury** (esp in children, adolescents, & newborns during birth). * _Classifications_: * -**Group I: middle ⅓ (****most common****)** * -Group II: lateral (distal) third * -Group III: proximal (medial) third (rare usually caused by major trauma) * _Etiology_: * trauma, direct impact or fall, MVA * _S/sxs_: * pain & swelling @ clavicle * deformity at site * pain with ROM * _PE_: * Inspect: deformity, swelling, skin tenting, open wound * -Palpate: tenderness, crepitance, ROM * -Check sternocleidomastoid & AC joint * -Check chest, ribs, lungs * -Distal neurovascular exam * _Dx_: * Xray confirms fracture * _Tx_: * **Middle ⅓:** non-operative, **sling immobilization**, figure 8 splint in children x 3-4 weeks in children or x 6-8 weeks in adults → repeat films, PT * **\*****\*Surgical indications:** severely shortened, displaced, segmental, open, young athlete requesting surgical consult, delayed union, persistent pain * Proximal ⅓ = ortho consult *

Answer 262

* _Definition_: * inflammation of the tendon insertion of the **extensor carpi radialis brevis muscle** d/t repetitive pronation of the forearm & excessive wrist extension * _Risks_: * 35-50 yo, racquet sports, screwdriver/ hand tool work, painting * _S/sxs_: * \*Gradual onset * -Lateral elbow pain: worse with gripping, forearm pronation * -May radiate down forearm * _PE_: * Palpate tenderness over epicondyle & distal tendon * -Pain reproduced by performing **wrist** **extension** **against resistance** * _Dx_: * Xray to r/o OA, loose bodies, mass * MRI if dx is uncertain * _Tx_: * **Conservative: ice/heat, stabilizing band, activity mods,** **NSAIDs,** **PT** * -Corticosteroid injections (controversial b/c may be a degenerative process and not inflammatory process so could make it worse) * -Surgery if refractory to tx

Answer 263

****_M_**ini **_G_**olf is **_F_**un; M = medial; golf = golfers; F = flexion** * _Definition_: * inflammation of the **pronator teres-flexor carpi radialis muscles** d/t repetitive overuse & stress at the tendon insertion of the flexor forearm muscles * _Risks_: * 40-60yo, golfers, household chores * _S/sxs_: * \*Gradual onset * -Tenderness over the medial epicondyle: worse with pulling activities * _PE_: * Palpate: tenderness over epicondyle & distal tendon * -Pain reproduced by performing **wrist** **flexion** **against resistance** * _Dx_ * Xray to r/o OA, loose bodies, mass * MRI if dx is uncertain * _Tx_: * **Conservative: ice/heat, stabilizing band, activity mods,** **NSAIDs****, PT** * -Corticosteroid injections (controversial b/c may be a degenerative process and not inflammatory process so could make it worse) * -Surgery if refractory to tx

Answer 264

* Most commonly occur on the fingers of the dominant hand of children. 10% dog bites, 30-50% cat bites, 5% other. * Risk of infection is 5-10% for dog bites, 30-50% for cat bites (deeper puncture wounds) * _Organisms_: * ***Pasteurella. multocida**, S. aureus, Strep sp.* * _S/sxs_: * S/sxs of infection: pain, swelling & redness around puncture wound * → nerve damage: loss of sensation & motion * _PE_: * **Need to complete a neurovascular exam before using an anesthetic** (bites often associated with nerve/tendon injuries) * _Dx_: * Xray to r/o fracture * -Labs: CBC, ESR, CRP (to follow the injury) * -Gram Stain * _Tx_: * **Debridement, wound irrigation with saline solution, & outpt abx** (Ceftriaxone/Augmentin) * -Loose closure * -Suturing is controversial (only if wound is clean after debridement) * -Tetanus prophylaxis * -Referral & close f/u

Answer 265

* _Definition_: * finger **flexed at the** **PIP joint** & hyperextended at the DIP joint * _Mechanism_: * central portion of the extensor tendon ruptures at its insertion onto the middle phalanx → PIP joint flexes from the unopposed pull of the flexor tendon * _S/sxs_: * \*history of trauma * -Finger held partially flexed at the PIP joint & extended at the DIP joint * -Painful tender * _Dx_: * Xray to r/o fracture * _Tx_: * **Splint PIP in extension x 4-6 weeks** * -Hand surgeon f/u

Answer 266

* Typically occur in the pulp (palmar tip) of the finger (felon) & in the soft tissues directly surround the fingernail (paronychia) * _Types_: * Felon: puncture wound * -Paronychia: ingrown nail * -Herpetic Whitlow: HSV * _S/sxs_: * -Felon: severe pain & swelling in the pad of the fingertip * -Paronychia: swelling of the tissues around the fingernail (usually on one side) * -Herpetic whitlow: vesicles with clear fluid * _Dx_: * clinical dx * _Tx_: * **Medical Emergency** * -Felon: **surgical drainage & oral abx** * -Paronychia: warm moist soaks & oral abx if severe, may need to remove nail plate (if so, should place a cover at the proximal edge of the nail bed so that * -Herpetic whitlow: observation

Answer 267

* _Mechanism_: * fall on outstretched hand (FOOSH) * Most commonly fractured carpal bone. Often missed → delayed treatment. Healing time: 12 weeks * Poor blood supply (comes from distal to proximal) so it has poor healing outcomes * _S/sxs_: * Pain along the radial surface of the wrist with anatomical **snuffbox tenderness** * _Dx_: * _Xray_: * -Need scaphoid view * -Fracture may not be evident for 2 weeks, but **if snuffbox tenderness is present then tx as fracture** (d/t complications) * _Tx_: * Nondisplaced fracture or snuffbox tenderness: **thumb spica splint or cast with repeat xray q 4 weeks** * -Displaced \> 1mm: ORIF

Answer 268

Fx of the Distal Radius * _Definition_: distal radius fracture with dorsal angulation * _Mechanism_: FOOSH with wrist extension * _S/sxs_: * Wrist pain worse with passive motion * _PE_: * swelling, deformity, discoloration around the wrist * **dinner fork deformity** * _Dx_: * _Xray_: * -**dorsally displaced** or angulated extraarticular fracture of the distal radius on lateral view * _Tx_: * -Stable: closed reduction & sugar tong splint or cast * -Unstable: ORIF

Answer 269

* _Definition_: * distal radius fractural with ventral angulation * _Mechanism_: * FOOSH with wrist flexion * _S/sxs_: * wrist pain worse with passive motion * _PE_: * swelling, deformity, & discoloration around the wrist * **garden spade deformity** * _Dx_: * XR **ventrally displaced** or angulated extra articular fracture of the distal radius on lateral view * _Tx_: * -Stable: closed reduction & sugar tong splint or cast * -Unstable: ORIF

Answer 270

* _Organism_: * ***Eikenella corrodens (*****anaerobes in the mouth)***, Staph, Strep* * _s/sxs_: * \*History of injury (may be hard to identify) * -Laceration * -Warmth, swelling, pain & purulent discharge * -Lack of extension or flexion (d/t tendon damage) * _PE_: * Measure & record location of laceration * -Assess function of flexor/extensor tendons & sensory nerves * _Dx_: * Xray to r/o fracture or presence of foreign body * -WBC, ESR, CRP (to follow injury) * _Tx_: * **Debridement, wound irrigation, & outpt abx; do not close wound** * -Referral * -Tetanus prophylaxis * \*Wounds that involve the joint, tendon, nerve, or bone require further eval

Answer 271

* _Definition_: * inger **flexed at the DIP joint** & unable to actively extend at the DIP joint * _Mechanism_: * avulsion of the extensor tendon after sudden blow to tip of finger → forced flexion of an extended finger * _S/sxs_: * -unable to actively extend the DIP joint → usually caused by jamming your finger * _Dx_: * Xray = normal or avulsion fracture of the distal phalanx at the tendon insertion site * _Tx_: * Nonoperative: uninterrupted extension **splint of DIP x 6-8 weeks with f/u** * -Surgery: closed reduction & percutaneous pinning

Answer 272

* Distal radius/Clavicle = Most common * Clavicle = boards; distal radius = Emond * Usually do well * Children are not small adults * Frequent trauma = frequent fractures * _S/sxs_: * Kids don’t sprain ligaments → think about physeal fracture: especially wrists * _When to Refer_: * Pattern of injury unclear * -Abuse suspected * -All open fractures * -All fractures involving joints * -All swollen elbows/knees * -All physeal injuries * -All displaced or dislocated injuries

Answer 273

* More aggressive than osteomyelitis * _S/sxs_: * Pain & tenderness, Fever, malaise * Limp * Erythema, effusion, decreased ROM * **_Kocher Criteria (Hip)_**: * -Temp \> 101.3F * -WBC \> 12,000 * -ESR \> 40, normal = 0-20 * -inability to ambulate * \*C-reactive protein \>2.5 mg/L * \*used to accurately predict likelihood of septic hip in a **child with a limp** * _Dx_: * Labs: CBC, ESR, CRP, Cultures * Arthrocentesis: gram stain * \*\*\*arthrocentesis before abx * _Tx_: * Seek assistance immediately

Answer 274

* aka Coxa Plana * _Definition_: * idiopathic **avascular osteonecrosis** of the proximal femoral head in children due to **ischemia** **of capital femoral epiphysis** * _Risks_: * **males 4-8yo _(L_ittle **_C_**ids)****,**delayed skeletal maturity (**short stature**), family hx, low birth weight, breech presentation * _S/sxs_: * **limping for weeks** (worse with continued activity) * -**hip or knee pain** (\*\*hip problems can present as knee pain in kiddos\*\*) * _PE_: * Effusion around the hip * -**Restricted ROM** (loss of abduction & internal rotation) * -**Trendelenburg sign**: stability of the hip and particularly the ability of the hip abductors (gluteus medius and gluteus minimus) to stabilize the pelvis on the femur * _Dx_: * _Xray_: \*\***must get frog-leg lateral (in all kids you work up for hip pain**)\*\* * -Early: may be normal, increased density of the femoral epiphysis, widening of the cartilage space * -Advanced: **crescent sign** (microfractures with collapse of the bone) * _Tx_: * Earlier diagnosis = better outcome, if caught early = good outcome * -**Observation**: activity restriction with ortho f/u (self-limiting with revascularization), PT, brace/cast, NSAIDs * -Surgical: **pelvic osteotomy** if advanced disease

Answer 275

* _Definition_: * **displacement of the femoral neck from the femoral head (epiphysis)** through the physis d/t weakness of the perichondrial ring. Bilateral 25% of the time. * _Risks_: * **African-American**, **males 10-15yo** (during growth spurt), kids that are big for their age, **obese**, family hx * _S/sxs_: * **Painful limp** **that worsens with activity** * -Ipsilateral dull, achy **hip, thigh, knee or groin pain** * _PE_: * Externally rotated leg on the affected side * -Loss of internal hip rotation * -Upper BMI * _Dx_: * _Xray_: * **\*\*****Must get frog-leg lateral** * -Anterior displacement of femoral neck with external rotation * _Tx:_ * **Refer to ortho for surgery → may require pinning** * -Non-weight bearing with crutches followed by **internal fixation** with pinning

Answer 276

* _Definition_: * acute, nonspecific, **inflammation of the joint synovium,** **MCC of acute hip pain in children** **3-10yo** * _Etiology_: * **post-viral URI**, allergic, trauma * _S/sxs_: * \*Acute Onset * **-****Limp** * -Hip pain +/- spasms * -**Afebrile** or low-grade fever (will not meet Koch’s criteria) * _PE_: * Limited ROM of the hip * _Dx_: * Dx of exclusion. Need to r/o septic arthritis * _Diagnostics_: * -Labs: ESR \< 20, WBC may be elevated * -Xray: normal * _Tx_: * Refer to ortho for aspiration or inpatient observation * -Supportive care with NSAIDs, RICe, and activity restriction

Answer 277

* _Definition_: * subluxation of the radial head. Most common elbow injury in children \< 5 yo * _Pathophys_: * associated with increased ligamentous laxity * _Etiology_: * pulling on the forearm when the elbow is extended & the forearm is pronated → annular ligament slips proximally & becomes stuck between the radius & ulna * _S/sxs_: * Child will react & cry after injury, but then the pain subsides * -**child is reluctant to use the arm, but is not in distress** * _PE_: * Extremity held by the side with elbow slightly flexed & forearm pronated * -**Tenderness over the radial head** * -Resistance on attempted supination * _Dx_: * XR will be normal * _Tx_: * **Reduction**: examiner’s thumb is placed over the radial head & the forearm fully supinated → snap * -Immobilization is not required

Answer 278

* _Definition_: **mild traumatic brain injury** leading to alteration in mental status +/- LOC * _Return to Play_: * law requires student athletes with possible concussion to be removed from play & receive a written note from a provider before return. Increase risk of concussion if full recovery from previous concussion was not completed. **No sooner than** **7 days** **after concussion diagnosis**. * _Complications_: * in the days following a concussion the brain cells are vulnerable & the brain doesn’t function normally temporarily * _S/sxs_: * \*Some may appear immediately, some may take time to develop/appear later * -HA, disorientation, slow or slurred speech, poor balance/coordination * -Poor concentration * -Photophobia/phonia * -Drowsiness, change in sleep pattern * -Fatigue * -Sadness or nervousness * _Dx_: * \*No definitive diagnostic test * -MRI/CT: no visible damage * *Assessment Tools*: * SCAT-2 or SCAT-5 * _Key History_: * seen by provider within 72 H, date of injury, mechanism of injury, LOC, location of impact, retrograde or anterograde amnesia, immediate or delayed onset of sxs * Most concussions **r****esolve within 3-4 weeks**. Return to normal is a stepwise process

Answer 279

* _First Response:_ * Remove from any activity, monitor s/sxs, do not give meds, evaluation by provider, no return to play until cleared * _First 24-48 hours_: * rest, avoid strenuous strenuous activity, do not drive x 24H, no EtOH, **acetaminophen** **= okay** (no ASA or NSAIDs), no sports * _Return to Learn_: * 1. Daily activities at home * 2. Homework/reading * 3. Part-time school * 4. Full-time school * \*Parachute’s Protocol: move forward to next stage only when sx free for 24H, if sxs reappear they should go back to previous stage, contact provider immediately if sxs worse * _Return to Play_: * 1. Daily activities * 2. Light aerobic activity * 3. Sport-specific activity * 4. Non-contact training drill * 5. Full contact practice * 6. Return to sport * **\*Each step takes a** **minimum of one day**

Answer 280

* _Definition_: * concussion sxs lasting beyond the expected recovery period after initial injury * _Etiology_: * unknown; structural damage, psychological factors * \*\*Usually goes away within a few days/weeks. However, persistent sxs may last for months-years * _S/sxs_: * -Mood swings, irritability * -Anxiety, depression * -Foggy thinking * -Memory Issues * -Difficulty with attention * -Dizziness, nausea * _PE_: * Tinnitus * -Balance problems * -difficulty making decisions * -changes in sleeping patterns * -Mild headaches * -Photophobia/phonia * _Dx_: * clinical dx * _Tx_: * Headache: sleep/rest, take a break from activities requiring concentration, pain relievers (APAP) * Memory Issues: write things in notebook, have family/friends remind you of important things

Answer 281

**Eyes opening (4)**; 4. spontaneously, 3. to speech, 2. to pain, 1. none - **Best Verbal (5)**: 5. oriented, 4. confused, 3. inappropriate word, 2. incomprehensive sounds, 1. none - **Best Motor (6)**: 6. follows commands, 5. localizes pain stimulus, 4. withdraws from pain, 3. flexion to pain, 2. extension to pain, 1. none → **MOST IMPORTANT SCORE** - Mild (80%): GCS 13-15, need repeat eval - Moderate (10%): GCS 9-12, need admission - Severe (10%): GCS 3-8

Answer 282

* _Dx_: * Evaluate damage & severity: x-rays, CT, MRI * -CT guidelines: LOC, severe HA, vomiting, age \>65yo, drug/EtOH intoxication, GCS \< 14, signs of basilar skull fracture, neuro deficits, amnesia, seizure, dangerous mechanism (ie significant MVA),, coagulopathy * _Tx_: * ABCs: Airway, Breathing, Circulation, Disability, Exposure/Environment * \*\*No drugs protect the delicate tissue of the brain * **Limit secondary damage**: maintain oxygen supply to the brain, prevent seizures (give **Keppra [levetiracetam**], prevent fever (meds if \> 39C), reducing swelling/inflammation/pressure * _Surgery:_ * if needed to remove blood clots or reduce pressure, need to remove entire skin flap * _Epidural hematoma_: * **operate within an hour of ipsilateral dilated pupil** or \> 30cm3, \>15mm thick, or \> 5 mm midline shift * **_Transient hyperventilation_**: * decreases ICP by causing vasoconstriction in the brain → more room to swell * **_Hyperosmolar therapy_**: * mannitol or hypertonic saline, fluid shift from intracellular to extracellular → low ICP * \*\*Steroid are contraindicated

Answer 283

* Start within 24 hours of injury & continue x 7 days * -Phenytoin, **Levetiracetam** (Keppra)

Answer 284

* F\>M. 1-6yo. Closely related to systemic sclerosis. * _S/sxs_: * **Fever** * **-****migratory salmon-pink rash** * -Arthritis * _PE:_ * HSM, lymphadenopathy * -growth delays * -pericarditis, pleuritis * _Complications_: * -tamponade, pulmonary HTN * -Macrophage activation syndrome *

Answer 285

* _Definition_: * **medium blood vessel** necrotizing vasculitis where the immune system attacks arteries, damaging endothelial cells (especially of the **coronary arteries**) * _Pathophys_: * unidentified respiratory agent or viral pathogen with a propensity towards vascular tissue * _Risks_: * **males \< 5yo**, asians * _S/sxs_: * _Warm & CREAM_: * -Fever \> 5 days * -**C**onjunctivitis * -**R**ash * -**E**xtremity changes: edema, erythema, or desquamation * **-A**denopathy * -**M**ucositis: **strawberry tongue**, lip swelling, fissures * _Dx_: * -Labs: elevated WBCs & platelets, anemia, increased ESR/CRP * -Echo * -ECG * _Tx_: * **IV immunoglobulin &** **aspirin** (for fever & coronary complications) * _Complications_: * -**coronary vessel arteritis**: coronary artery aneurysms, MI, pericarditis, myocarditis

Answer 286

* _Definition_: * \<1% of HAs; more common in young/middle aged **males** * _Triggers_: * **Night** **(sleep)**, alcohol, stress * Usually have 1-2 cluster periods per year (each lasting weeks to months) * _S/sxs_: * **severe unilateral** **periorbital** **pain**, \<2 hours with spontaneous remission, **sharp, excruciating, searing or piercing pain** * \*Repetitive Clusters of pain * _PE_: * **_Ipsilateral Dysautonomia_**: * -Ptosis (droopy eyelid) * -**Lacrimation** * -Conjunctival injection * -**Rhinorrhea** * -Nasal Congestion * -Restlessness * _Dx_: * Clinical Dx * _MRI_: to r/o lesion * _Tx_: * “Suicide Headache” because so severe * **100 %** **oxygen** **at 12-15L/min for 15-20 min via non-rebreather → provides relief within 15 minutes** * -Can give in combo with **sumatriptan** 6mg SQ , can repeat in hour PRN(Triptan; if possible) * For **long-term prophylaxis** = verapamil, lithium, topiramate, Divalproex, or a combo * Anti-migraine medications: Sumatriptan, Ergotamine/dihydroergotamine (MOA: 5HT-1b/d receptor agonists)

Answer 287

* _Chronic Migraine_: * **15+ days/month x 3 months** * _Pathophys_: * trigeminal nerve releases **neuropeptides** **(CGRP****)**→ dural inflammation & pain that is transmitted by the same nerve;**aura is an abnormal yet benign electric wave** that travels slowly across the cortex to cause sxs * _Risks_: * **women (3x)**, teens, genetics, obesity, frequent HAs, head injury, **med overuse (\>10day/month)**, stress, sleep apneas * _Epidemiology_: * 12% of HAs but more likely to be seen in clinic than tension-type because more severe sxs; 1-4 per month on average * _S/sxs_: * Headache: **unilateral**, **throbbing**, moderate-severe, increased with exertion, **4-72 Hr duration** * N/V * -Without Aura = most common 80% * -**photophobia** * **-Phonophobia**

Answer 288

* _Dx_: * **Diagnosis is clinical** * \*\*If worrisome features: * -MRI * -Labs * -Lumbar Puncture * _Tx_: * **_Holistic approach_**: regular sleep, physical activities, & meals; HA diaries, stress management * -**Mild to moderate attacks**: * → **NSAIDs** such as ibuprofen, naproxen, and diclofenac are generally inexpensive and effective in up to 60% of cases * →**Excedrin Migraine** (aspirin 500 mg-acetaminophen 500 mg-Caffeine 130 mg) combo is an inexpensive and FDA-approved tx for an acute migraine * **More Severe Attacks:** * **Abortive:** **Triptans** Sumatriptan 25-100 mg PO x1, may repeat dose x1 after 2 hours; 1-6mg SC x1 , may repeat dose after 1 hour. (do not use in Ischemic Heart disease), **ergotamine/dihydroergotamine** (do not use in pregnant women) * -Prophylaxis: first line agents = **Divalproex, topiramate (**generalized anti-seizure meds**), propranolol, metoprolol,** & **timolol**

Answer 289

* _Risks_: * stress, sleep deprivation, eye strain * _Epidemiology_: * **38% of headaches** **(most common)** but less likely to be seen in clinic than migraine * _s/sxs_: * Headache: **diffuse, dull, “band-like”**, non-throbbing * **No accompanying symptoms like N/V, and photophobia/phonia** * Bilateral, squeezing sensation, mild to moderate with dull pain * _PE_: * normal * _Dx_: * Dx is based on characteristic sxs and a normal physical exam → normal Neuro * _Tx_: * **1st line - NSAIDs****:** ibuprofen (400mg PO q 4-6hrs PRN) , naproxen (250-500mg PO q 12 hours, max dose: 1250mg) * 2nd line: caffeine-containing agents (Excedrin alone can be very effective) * Opiates should be avoided * **Prevention:** Tricyclic antidepressants (TCAs) (particularly **amitriptyline** - 10-100mg PO qhs (at bed time) )

Answer 290

* _Definition_: * inflammation of the brain parenchyma * _Etiology_: * arboviruses, **HSV-1 (most common****)**, HSV-2, HZV, EBV, CMV, rabies,**West nile virus** * _HSV Encephalitis:_ * lesions limited to the temporal lobe, necrotizing encephalitis * _S/sxs_: * -**Alteration of consciousness** * **-Focal neurologic deficits**: hemiparesis, sensory deficits, CN palsies * -Seizures, * -Fever * -HA * -**Change in personality**: hallucinations & bizarre behavior may precede neurological deficits * _PE_: * _HSV Encephalitis_: * -Dysphagia * -Seizures * _West Nile Virus Encephalitis_: * -**Flaccid paralysis** with a clear sensorium * _Dx_: * -**_MRI/CT_**: medial temporal & inferior frontal grey matter involvement * -**_EEG_**: asymmetric sharp waves * -**_Lumbar puncture_**: normal glucose, lymphocytes, elevated RBCs * -**_PCR testing_**_:_ HSV identification * **\*\*Neuroimaging must be done!!** * _Tx_: * HSV: **IV** **acyclovir** **x 14 days**

Answer 291

* _Definition_: * a localized, walled-off collection of pus surrounded by a fibrous capsule within the brain parenchyma * _Etiology_: * bacteria (most common), fungi, protozoa; *NO viruses* * _Predisposing Factors_: * otitis media, sinusitis, dental infection, trauma, neurosurgery, neutropenia, HIV infx * _S/sxs_: * focal neuro deficits * _Dx_: * head CT * _Tx_: * Abx: **Ceftriaxone + metronidazole + vancomycin** * -If prior neurosurgical patient → get MRSA coverage * -Steroids: only if cerebral edema * -Neurosurgery consult for possible drainage.

Answer 292

* _Definition_: * bacterial infection of the meninges * _Etiology_: * May be bacterial, viral, or subacute * -***Strep Pneumoniae***: **most common cause in adults & young kids**, G+ cocci * -***Neisseria meningitidis*****:**Most common in older children (**10-19yo**), G- diplococci * -Group B Strep: most common neonates * -***Listeria monocytogenes***: increased incidence neonates & infants, risk for preggos * -***Haemophilus influenzae***: reduced d/t HIB iz, G- coccobacillus * _Risks_: * age (babies), large group gatherings (college) microbiologists, travel (sub-saharan African, mecca) * _S/sxs_: * **_Clinical Triad_**_:_ * -**Fever** * **-Headache** * **-Nuchal Rigidity** * -decreased LOC * -Seizures * -Increased intracranial pressure * -photosensitivity * -Bulging fontanelles in babies * **_Meningismus_**: 1. **Nuchal Rigidity (stiff neck)** 2. **Photophobia** 3. **Headache** * _PE_: * **Brudzinski**: neck flexion produces knee/hip flexion * -**Kernig**: inability to extend the knee/leg with hip flexion * _Dx_: * -CSF: **PMN leukocytosis****, decreased glucose, increased protein**, increased opening pressure, turbid * -**CSF pathogen panels: tests for the 14 most common pathogens** * -Cultures, empirical therapies, neuroimaging * \*Work-up order: blood cultures, steroids, abx, CT, LP * _Tx_: * **Broad Spectrum Abx:** **Vanco + Ceftriaxone** +/- dexamethasone * -Droplet precautions for 24 hours * -Post-exposure prophylaxis: Ciprofloxain or Rifampin * -**Listeria****: add****ampicillin**; prevent by cooking foods & pasteurizing dairy products

Answer 293

* CT scan doesn't have to be done before LP if \< 60yo, * immunocompetent, * no hx of CNS disease, * no recent seizure, * normal sensorium & cognition, * no recent major trauma, * no papilledema

Answer 294

* _Etiology_: * **Enteroviruses**, varicella-zoster, HSV2, Epstein-Barr Virus, Arthropod-borne viruses * _S/sxs_: * **_Clinical Triad_**_:_ 1. **Fever** 2. **Headache** 3. **Nuchal Rigidity** * _PE_: * Decreased LOC, malaise, myalgias, anorexia, n/v/d, abd pain * _Dx_: * CSF exam: **lymphocytes****,****normal glucose & protein**, clear * -viral culture * -Serologic studies

Answer 295

* _Etiologies_: * M. Tuberculosis, C. neoformans, H. capsulatum, C. immitis, T. Palladium * _S/sxs_: * -low-grade fever * -headache * -nuchal rigidity * -Lethargy

Answer 296

* _Definition_: * clinical & lab evidence of meningitis where the organisms are not detected in the CSF * _Etiologies_: * viral (**enteroviruses most common**), bacteria, parasitic, drugs, autoimmune, malignancy, post-infectious * _S/sxs_: * \*Classic sxs of meningitis but may be milder * -Fever * -HA * -Nuchal rigidity * _Dx_: * Dx of exclusion. R/o bacterial meningitis * CSF: normal glucose, lymphocyte * _Tx_: * supportive: antipyretics, IV fluids, analgesics

Answer 297

* _Definition_: * abnormal neuronal discharge from **one discrete section of one hemisphere** * _Classification – Level of Awareness_: * Intact awareness (simple): consciousness fully maintained * -Impaired awareness (complex): consciousness impaired * _Classification – Onset:_ * -Motor: tonic-clonic, atonic, myoclonic * -Non-motor * -Unclassified: not enough info to determine onset (unwitnessed seizure) * _S/sxs_: * Types are dependent on brain area * _PE_: * **Focal Seizures** with **retained awareness** → **no alteration in consciousness**, but abnormal movements or sensations (used to be called *simple partial seizure*) * **Focal Seizures** with a **loss of awareness** → **altered LOC**, automatisms (i.e. lip smacking) (used to be called *complex partial seizure*) * → Present with a **postictal state (confusion & memory loss**) which helps to **differentiate them from absence seizures** * _Dx_: * Initial workup to r/o reversible causes * Electrolytes: Na+, Ca2+, Mg * -Serum glucose * -Pregnancy test → can affect type of antiepileptic therapy the patient receives * -toxicology screen * -ECG * -EEG * -Neuroimaging: CT or MRI of the head → should be done all adults with their first seizure * _Tx_: * May evolve into generalized tonic-clonic seizures * Tx: **phenytoin**, and **carbamazepine** = drugs of choice

Answer 298

* _Focal Motor_: * jerking movements of the face, one foot one arm, or another part * _Focal Sensory_: * hearing problems, distorted olfactory sense, or hallucination * _Focal Autonomic_: * changes in HR or rhythm, BP, bowel or bladder function * _Focal Psychic_: * triggers emotion or memories causing fear, anxiety, or deja vu

Answer 299

aka Grand Mal Seizures * _Definition_: * simultaneous neuronal discharge of **both hemispheres** (diffuse brain involvement) * Always have some level of impaired awareness * _Classification – Onset_: * -Motor: tonic-clonic, atonic, myoclonic * -Non-motor: absence * \*\*Tonic-clonic is most commonly seen in metabolic derangements, drug withdrawal, & head trauma * _S/sxs_: * **_Tonic Clonic_**: * -**Generalized body stiffness & rigidity** * -Arched back * -Jerking movements of the trunk, extremities, & head * -Associated findings: tongue biting, **incontinence**, frothing at the mouth, eye blink, **cyanosis** * -Post-ictal phase * _Dx_: * \*\*Initial workup to r/o reversible causes * Seizures that develop during **adolescence** and **adult life** are usually caused by **tumor**, trauma, drug use, or alcohol withdrawal → in addition to blood work you should obtain a **CT scan** * _Tx_: * Those at lower risk may not need medications until further evaluation is completed (or another seizure occurs) * **-Tx the underlying cause** * **-Anti-seizure meds**: phenytoin, fosphenytoin, levetiracetam, Valproic acid, phenobarbital; loading dose → daily dosing

Answer 300

aka Petit Mal → type of generalized seizure * _Definition_: * generalized seizure character by a **brief (4-20 sec) loss of environmental awareness without loss of body tone** **(Type of Generalized seizure)** * _Risks_: * provoked by hyperventilation or **flashing lights** * _Age of Onset_: * **~4-10 yo** (Childhood) * _S/sxs_: * -**Sudden, marked impairment of consciousness** without loss of body tone * -May be accompanied by simple automatisms: eyelid twitching, lip smacking * -”Staring into space” * **-No post-ictal confusion** & no memory of the event * _PE:_ * May have dozens of seizures per day which often go unrecognized * _Tx_: * **1st line =** **ETHOSUXIMIDE** * 2nd line = Valproic acid

Answer 301

* _Definition_: * **5+ minutes** **of continuous seizure activity** OR recurrent seizure activity without return to baseline OR **2+ seizures within a 5 minute period**. * _Etiologies_: * metabolic, sepsis, CNS infx, stroke, TBI, drugs, cardiac arrest, encephalopathies, autoimmune encephalitis, breakthrough seizures, chronic EtOH abuse, CNS tumors, remote CNS pathologies * _S/sxs_: Two types: * 1.**Convulsive status epilepticus**: presents with a regular pattern of contraction and extension of the arms and legs * 2. **Non-Convulsive Status Epilepticus**: includes **complex partial status epilepticus** and **absence status epilepticus** * _PE_: * Untreated generalized **seizures lasting** **\>60 min may result in permanent brain damage**; longer-lasting seizures may be fatal * _Dx_: * _Initial Assessment_: * -ABCs * -Trauma Assessment * -Fingerstick Blood Glucose (FSGS) * _Tx_: * **Medical Emergency** * Protect from injury: do not restrain patient & do not put anything in the mouth (but have suction available), * -Place patient in **left lateral decubitus position** (suppressed gag reflex ⇒ prone to aspiration of gastric contents) * -**Immediate seizure control treatment: IV/IO** **Benzodiazepines** (**LORAZEPAM,** midazolam, diazepam) initially after which you give **phenytoin** * -Closely monitor patient until recovery * -Post-Ictal: positioning, airway control, labs & imaging, longer-acting anti-seizure meds, EEG monitoring.

Answer 302

* **Key Hx Points:** * description of aura, duration of seizure, nature of motor activity, loss of awareness, duration of postictal state, triggers, current meds, EtOH/drug use * **Labs**: * rapid POC glucose, CBC/CMP/UA, toxicology, lactate, hCG, CT/MRI, ECG, EEG, LP (if infection suspected) * **All pts suffering a 1st seizure should be evaluated by a neurologist** (EEG, MRI, risk-stratification, start anti-seizure drug)

Answer 303

* _Definition_: * chronic condition characterized by **impaired muscle tone, strength,** and **coordination** of movements * _Pathophys_: * **cerebral injury** often **before birth** but can also occur **during birth** or in the **perinatal** and **postnatal** period. Maternal infections &/or fetal hypoxia is often root cause * _S/sxs_: * -Spasticity (still muscles) * -Dyskinesia (uncontrollable movements) * -Ataxia (poor balance & coordination) * * May also have: intellectual disability; seizures; problems with vision, hearing or speech; changes in spine (scoliosis); or joint problems (contractures) * _Dx_: * Cranial **MRI** * * CP can rarely be confirmed during early infancy and the specific syndrome **cannot be characterized until around age 2** * -High risk kids (e.g. kids with evidence of asphyxia, stroke, periventricular abnormalities seen on cranial ultrasonography in premature infants, jaundice, meningitis, neonatal seizures, hypertonia, hypotonia, or reflex suppression) should be followed closely * _Tx_: * **Early intervention** for children age 0-3 years is very important!! * PT and OT * **Tx the spasticity**: braces, constrains, surgery and meds * -Benzodiazepines → myorelaxation, anxiety relief * -Spasmolytics → muscle-spasticity relief * -Anticonvulsants → seizure tx, prevention * -pain meds * -Botulinum toxin injections * -Intrathecal baclofen

Answer 304

* _Definition_: * an **autoimmune disease of the CNS** characterized by chronic inflammation, **demyelination**, gliosis (plaques or scarring), & neuronal loss of the white matter * _Risks_: * **Upper Northern Hemisphere** (less sunlight), women, smoking, **Vitamin D deficiency**, family hx, **African Americans** (increasing) * _Epidemiology_: * most common permanently disabling disorder of the CNS in young adults; **mean age onset 28-31yo** * _S/sxs_: * **-paresthesias** in the extremities * **-Ataxia** * **-Fatigue** * **-Focal motor symptoms** * -**weakness** * -Spasticity * First visit usually associated with vision symptoms followed by weakness in distal limbs, ataxia, or paresthesias over several years * _PE_: * **Lhermitte’s Sign**: electrical shock sensation in the limbs & torso brought on by movement (usually flexion) of the neck. * _Dx_: * **MRI** looking for **plaques** associated with MS (McDonald’s Diagnostic Criteria) → **MRI of the brain & spinal cord** * If MRI is non-diagnostic then next best test = **lumbar puncture** which would show **oligoclonal bands** * **_McDonald’s Diagnostic Criteria_**: * -**2 episodes of neurologic deficits** (focal weakness, sensory disturbances) separated in time & space * \*Must occur in absence of a fever, infection or other etiologies * \*Attacks must last \>24 hours. * _Tx_: * Average lifespan = 25-35 years after diagnosis * **_Acute Exacerbations_:** * -**Corticosteroids** = tx of choice, PO = IV * -Plasmapheresis: if unresponsive to steroids, every other day x 14 days * **_Chronic Management_**: * \*Disease-modifying agents suppress T-cell autoimmune cascade → EARLY treatment instigated by neurologist * -**Disease modifying agents:** **interferon beta, Glatiramer**, fingolimod, teriflunomide, dimethyl fumarate, natalizumab, alemtuzumab, ocrelizumab, mitoxantrone * -Symptom-Specific tx for neurogenic bladder (oxybutynin) * Tx of **spasticity (****Baclofen, tizanidine, diazepam, dantrolene**)

Answer 305

* **_Pathophys_**: * -Plaques develop at different times & in different CNS locations * -**T cells & B cells** cross damaged BBB & release inflammatory mediators & *immunoglobulins* that target myelin nerve sheath * -**Macrophages** expose axonal surfaces & release NO & oxygen free radicals * -Surviving oligodendrocytes partially remyelinate → **gliotic plaques (Sclerosis)** * **_Course of Disease_**: * **Relapsing-Remitting (90%)**: discrete attacks evolving over days-weeks followed by recovery * -Secondary progressive: initial relapsing-remitting followed by gradual deterioration * -Primary progressive: steady functional decline from onset

Answer 306

* Common during childhood, increasing in frequency, & incidence during adolescence * _Epidemiology_: * 40% of children experience a HA by 7 years old & 75% will by 15 * \*Migraines more difficult to diagnose in children because they are expressed differently * HA diaries * \*Not all HA meds used in adults are approved in children

Answer 307

* Etiology & criteria for diagnosis of epilepsy are much the same as in adults. Febrile seizures more common in pediatrics * _Tx_: * Med dosage & serum drug levels will need to be careful monitored/adjusted with child growth * -Ketogenic diet * -Surgery: if refractory to meds & other treatments

Answer 308

* _Definition_: * specific type of seizure seen in epilepsy syndrome known as **WEST SYNDROME**. Onset 4-8 months. * **Poor intellectual prognosis** d/t neurological impairment prior to onset of spasms * _S/sxs_: * \*Happen in clusters * -Infantile spasms: occur just before sleep or upon awakening * -Developmental regression * -Specific EEG changes * _PE_: * Brief Symmetric contractions of extremities, trunk, neck muscles: sudden bending forward of the body with stiffening of the arms/legs, some arched backs at they extend * _Dx_: * EEG: hypsarrhythmia pattern (chaotic waveforms) * -MRI: preferred test * _Tx_: * Spasms usually resolve by mid-childhood but \> 50% will develop other types of seizures * -ACTH, vigabatrin, ketogenic diet

Answer 309

* _Definition_: * stroke occuring from age **1mo -18 years.** Rare but also top 10 cause of death in childhood * _Types_: * -Ischemic: 50% * -Hemorrhagic: 50% * _Perinatal Stroke_: * -occurs during later stages of pregnancy through 30 days of age; Red flags: congenital heart disease, infections, placental disorders * _PE_: * thorough neurological exam * _Dx_: * Often unrecognized for a long period of time * CT/MRI: early imaging = important!! * _Tx_: * Overall mortality rate: 3-20% (x2 in black children) * -Referral to pediatric neurology &/or specialty center * -Supportive tx * -Hemorrhagic: surgical & endovascular tx * -\*Thrombolytics are being investigated for pediatric stroke

Answer 310

* _Pathophys_: * defective closure of the neural tube at the end of week 4 of the gestational period. → leads to anomalies of the lumbosacral vertebrae or spinal cord * _Epidemiology_: * most frequent permanently disabling birth defect * _Forms_: * **Meningomyelocele**: most severe, spinal cord or nerve roots exposed on back in sac * -**Meningocele**: rare, onl meninges exposed * \*Both associated with latex allergy * _S/sxs_: * \*Range of severity from physical defects to total paralysis & incontinence * -Simple dimple or tuft of hair over affected area * -Cyst-like structures +/- nerve involvement * -**Meningomyelocele:** area covered with sores +/- CSF leaks, leg paralysis, & incontinence. * -**Meningocele:** underlying Spinal Cord is anatomically & functionally intact, no neuro deficits * _Tx_: * **_PREVENTION =**_ _**FOLIC ACID_** * -Meningomyelocele: immediate surgical closure * -Meningocele: surgical repair

Answer 311

* _Definition_: * cerebellum herniation through the foramen magnum * _S/sxs_: * Occipital headache that radiates upward and worsens with valsalva * -Visual or balance problems * -Cranial Nerve compression * -Muscle Weakness * _PE_: * Associated with hydrocephalus d/t compression of 4th ventricle * _Tx_: * Pain management vs surgical decompression

Answer 312

* _Definition_: * slow accumulation of CSF over weeks to months → ventricular distention & increased ICP * _Etiology_: * obstruction vs overproduction of CSF * _S/sxs_: * **Increased head circumference** * -Bulging anterior fontanelle * -**Frontal bossing** * -CN III or CN VI dysfunction * -Papilledema * _Pe_: * -Bradycardia * -HTN * -Altered respiration * _Dx_: * -CT scan: if head circumference \> 2nd percentile lines or if symptomatic * _Tx_: * Symptomatic relief: loop diuretics, acetazolamide * Surgery: remove obstruction, ventriculo-peritoneal shunt

Answer 313

* _Definition_: * a severe, progressive, muscle-wasting disease that leads to difficulties with movement and, eventually, to the need for assisted ventilation and premature death. The disease is caused by mutations in DMD (encoding dystrophin) that abolish the production of dystrophin in muscle * _Etiology_: * **X-linked** recessive disorder (affects males) * _Epidemiology_: * most common hereditary neuromuscular disease * _Progression_: * starts at 3 yo & fully expressed by 5-6yo, wheelchair bound by 10yo, death by 18-20yo * _Complications_: * **Cardiomyopathy** * _S/sxs_: * Progressive weakness: **waddling gait**, inability to rise from ground easily * **Gower’s sign**: use hands to push up from floor to standing * -Intellectual impairment * Proliferation of connective tissue in muscle: hypertrophy of calves * _PE_: * Slower to develop motor milestones: walking, climbing stairs * _Dx_: * -Muscle Biopsy: muscle fiber degeneration & regeneration with connective tissue * -Absence of dystrophin (Ig Protein) * -EMG: assesses muscle weakness * -Serum CPK: markedly elevated * _Tx_: * Symptomatic: glucocorticoids * -New therapies: gene therapy, creatine, aminoglycosides

Answer 314

* _Definition_: * **Autosomal dominant** genetic disorder that causes tumors (neurofibromas) to form on nerves * _Types_: * Type 1: more common, peripheral type * -Type 2: central type, incurable disease * _PE_: * **_cafe au lait spots_**: flat, irregularly shaped, light brown macules present at birth * -**Neurofibromas**: soft or firm pedunculated tumors along nerve * -Axillary/groin freckling * -Learning disability (40%) * -short stature, macrocephaly

Answer 315

* Primary malignant CNS tumors are the 2nd most common childhood malignancies (after heme). Leading cause of death from childhood cancer. * _S/sxs_: * Ha * -N/V * -Loss of balance * -Speech problems * -sleepiness * -personality changes * -Seizures * -increased head circumference * _PE_: * \*findings may be subtle &/or nonspecific & vary with child’s age & tumor location * _Dx_: * CT/MRI

Answer 316

* -**_Lithium_**: * 1st line, narrow therapeutic window (toxicity), euphoric mania, weight gain, decreases suicide risk -Valproic Acid/Divalproex: mixed episodes, rapid cycling, weight gain, liver/pancreas inflammation, thrombocytopenia * _Anticonvulsants_: * Carbamazepine, Lamotrigine (watch for rash→ SJS) * _Atypical antipsychotics_: * potential metabolic effects, monitor fasting glucose, cholesterol panels, weight * _Interpersonal Therapy:_ * psychoeducation, focuses on interpersonal context * _Cognitive therapy:_ * focuses on automatic negative thoughts about self/world/future * _NAMI-group therapy_: * for families & patient -Case-management → reduces hospitalizations * Family therapy

Answer 317

* _Definition_: * requires lifetime experience of **at least one manic episode**. Psychosis and major depressive disorder not required. * _Risks_: * family hx * _Epidemiology_: * 1.2% of adult population, onset 20-30s * _Criteria_: * At least **_1 manic**_ or _**mixed episode_** * -symptoms not due to medical condition or substance use * **_Manic Episode DSM-V_:** * **\*3+, 4+** **if mood irritable only** * -Abnormal & persistent **elevated, expansive, or irritable mood** lasting at least **one week** * -increased goal-directed activity * -grandiosity * -decreased need for sleep * -more talkative or pressured speech * -flight of ideas * -distractibility * -impulsivity * _Dx_: * _Screening Tools_: * -**Mood Disorder Questionnaire (MDQ): positive if endorse 7 items** * Manic Episodes with severe and marked impairment in functioning **may necessitate hospitalization** to prevent harm to self or others. Psychosis may be present.

Answer 318

* _Definition_: * requires lifetime experience of **at least one episode of** **major depression** **& at least one** **hypomanic** **episode**. No manic episodes * _Epidemiology_: * 5% of the population * _Criteria_: * -At least 1 hypomanic episode * -at least 1 major depressive episode * -No manic episodes * **_DSM-V of Hypomanic Episode_:** * **\*****3+, 4+****if mood irritable only** * -Abnormal & persistent **elevated, expansive, or irritable mood** lasting at least **_4 days_** * -increased goal-directed activity * -grandiosity * -decreased need for sleep * -more talkative or pressured speech * -flight of ideas * -distractibility * -excessive involvement in activities with consequences ($, sex, business involvement) * _Dx_: * **Mood Disorder Questionnaire (MDQ): positive if endorse 7 items** * Hypomanic episodes have a change in behavior that is **not severe enough for hospitalization**. No psychosis.

Answer 319

* _Definition_: * excessive worry or anxiety **occurring more days than not** for at least **6 months** → significant distress or impairment in social, occupational, or other important areas of life * _Epidemiology_: * average onset early 20s, more common in females * **_DSM-V Criteria_:** * \*\*3+ of the following: * -Restlessness * -Fatigue * -Difficulty concentrating * -Irritability * -Muscle Tension * -Sleep disturbance * _Dx_: * Clinical dx * r/o thyroid problems * _Tx_: * **SSRIs** = mainstay of treatment, (high dose for anxiety, low dose for depression) * **Cognitive Behavioral Therapy:** mainstay of tx * Benzos: as needed, not for ongoing treatment * Buspirone (Wellbutrin): may be used with SSRIs * Off label: propranolol, hydroxyzine, neurontin, seroquel, risperdal

Answer 320

* _Definition_: * marked fear or anxiety about **1+** social situations in which the individual is exposed to possible scrutiny by others (i.e. observed by others, eating in front of others) * _Epidemiology_: * most common type of phobia (public speaking) * _S/sxs_: * Anxiety provoked by social situations * Fear that they will act in a way or show anxiety & be negatively evaluated: out of proportion to threat posed * Avoidance of social situations * Impairment in social functioning * _Dx_: * Clinical Dx * r/o other causes * _Tx_: * **SSRIs** = mainstay of treatment, (high dose for anxiety, low dose for depression) * **Cognitive Behavioral Therapy:** mainstay of tx * Benzos: as needed, not for ongoing treatment * Buspirone: may be used adjacent to SSRIs * Off label: propranolol, hydroxyzine, neurontin, seroquel, risperdal

Answer 321

* _Definition_: * an abrupt surge of intense fear or discomfort that reaches a peak within 10 minutes * **_DSM-V Criteria_** * **\*\*****4+****of the following:** * -Palpitations * -Sweating * -Trembling, shaking * -SOB * -Feelings of Choking * -Chest pain or discomfort * -Nausea, abdominal distress * -feeling dizzy * -chills or heat sensations * -Paresthesias * -Depersonalization * -Fear of losing control * _Dx_: * Need to r/o potentially life-threatening conditions (MI, thyrotoxicosis) * _Tx_: * Acute Attacks: benzos →1st line, alprazolam or clonazepam

Answer 322

* _Definition_: * **recurrent, unexpected panic attacks** (at least 2). * **_DSM-V Criteria_** * **\*\*****4+****of the following:** * -Palpitations * -Sweating * -Trembling, shaking * -SOB * -Feelings of Choking * -Chest pain or discomfort * -Nausea, abdominal distress * -feeling dizzy * -chills or heat sensations * -Paresthesias * -Depersonalization * -Fear of losing control * **_DSM-V Criteria_**: * At least one of the attacks has been followed by 1 month of one or both of the following: * persistent concerns or worry about having additional panic attacks * a significant maladaptive change in behavior related to the attacks * _Tx_: * **SSRIs** = mainstay of treatment, (high dose for anxiety, low dose for depression) * **Cognitive Behavioral Therapy:** mainstay of tx * Benzos: as needed, not for ongoing treatment * Buspirone: may be used adjacent to SSRIs * Off label: propranolol, hydroxyzine, neurontin, seroquel, risperdal

Answer 323

* _Definition_: * presence of obsessions, compulsions, or both * _Specifiers_: * with good or fair insight: recognizes OCD beliefs are not true or may not be true * With poor insight: thinks OCD beliefs are probably true * With absent insight or delusional beliefs: completely convinced OCD beliefs are true * _Patterns_: * contamination, pathologic doubt, symmetry/precision, intrusive/obsessive thoughts * _Obsessions_: * **Recurrent & persistent thoughts**, urges, or imagines that are experienced at some time during the disturbance (intrusive & unwanted) * causes stress & anxiety * _Compulsions_: * **Repetitive behaviors** (hand washing, ordering, checking), or mental acts (counting, repeating words), patient feels drive to perform according to certain rules * -Aimed at preventing or reducing the anxiety or distress * _General Info_: * obsessions & compulsions are time consuming or cause significant distress in social or occupational functioning. Not attributable to a medical condition or better explained by another mental disorder. * _Dx_: * Clinical Dx * _Tx_: * low dose for depression) * **Cognitive Behavioral Therapy:** mainstay of tx * Benzos: as needed, not for ongoing treatment * Buspirone: may be used adjacent to SSRIs * Off label: propranolol, hydroxyzine, neurontin, seroquel, risperdal

Answer 324

* _Definition_: * persistent intense fear or anxiety of a specific situation, object, or place * **_DSM-V Criteria_:** * -Persists for at least **6 months** * -Exposure to situation triggers an immediate response * -Fear out of proportion to any real danger * -**Everyday activities impaired by distress or avoidance** * _Tx_: * **1st line = exposure & desensitization therapy** * Short term therapy: benzos or beta-blockers

Answer 325

* _Definition_: * **maladaptive emotional or behavioral rxn to an identifiable stressor** (job loss, physical illness, divorce, etc.) or a non-life threatening event that causes a disproportionate response that would normally be expected **within 3 months** of a stressor * _S/sxs_: * Marked distress out of proportion to the severity of stressors * -significant impairment in areas of functioning * -depressed mood, anxiety * Pt may try to self-medicate with alcohol or drugs * _Tx_: * **Psychotherapy = 1st line**

Answer 326

* _Definition_: * exposure to **traumatic event at any time in the past →** **sxs \> 1 month** * **_DSM-V Criteria_:** * **Stressor: exposure** or threat of death, serious injury, sexual violence * **Intrusion Symptoms**: traumatic event persistently re experienced (via nightmares, flashbacks, intrusive memories) * **Avoidance**: persistent avoidance of distressing trauma-related reminders after the event * **Negative Alterations in Mood**: negative beliefs, distorted blame, diminished interest * **Alterations in Arousal/Reactivity**: irritability, aggression, “on guard”, exaggerated startle, disturbed sleep, poor concentration, reckless or self-destructive behavior * **Duration \> 1 month** * _Dx_: * _Screening:_ * -PTSD Checklist-17 (PCL-17) * -National Stressful Events Survey PTSD Short Scale (NSESSS) * -Post Traumatic Cognitions Inventory (PTCI) * -Generalized Anxiety Disorder Screen: * → GAD-7 (score over 8) * → GAD-2 (score over 3) * _Tx_: * **SSRIs = 1st line** * **→** Paroxetine and sertraline are FDA approved, may take longer to be effective (up to 24 weeks) (**P**T**S**D) * Clonidine and Prazosin can help hypervigilance and nightmares * Benzos not encouraged * non-pharmacologic: * social engagement, exercise, journaling, lifestyle changes * CBT

Answer 327

* _Definition_: * sxs similar to PTSD except the **traumatic event occurred** **\< 1 month ago** and the **sxs last \< 1 month** * _S/sxs_: * Intrusive sxs * -avoidance * -increased arousal * -negative alterations in thought & mood * _Tx_: * Counseling & psychotherapy = 1st line * \*If sxs persist \>1 month then tx as PTSD

Answer 328

* _Definition_: * failure to maintain a normal body weight; fear & preoccupation with body image * _Etiology_: * **genetic**, trauma (50%), family dynamic \*False misconception that it is a disease of privilege in wealthy white women * _Epidemiology_: * **deadliest illness in psychiatry**, peak incidence **13-18yo** (but growing risk in group 8-12yo), 0.4% F population, 10:1 F:M; **chronic illness** with remission for many * _S/sxs_: * -**Thin** (usually) * -Relentless pursuit of thinness as a method of control * -Intense fear of becoming fat * -significantly underweight * -severe caloric restriction & new diets (vegetarianism) * -Ritualistic eating behaviors & habits * -**Preoccupied with food** (recipes, hoarding) * -May involve purging behaviors, vomiting, laxative abuse, exercise * **_DSM-V Criteria_** * A) **Restriction of energy intake** relative to requirements leading to significantly low body weight * B) **Intense fear of gaining weight** or becoming fat, even though underweight * C) **Disturbance in the way in which one’s body weight or shape is experienced**, undue influence of body shape or weight on self-evaluation, or denial of the seriousness of current low body weight. * _Dx_: * -UA * -Electrolyte panel * -CBC, TSH, ESR * -LFTs, Amylase * -EKG * -B-hcg, LH, FSH, prolactin, estradiol * -**Bone density** * **\*\* In pts who only restrict it is common that all lab tests are NORMAL** * _Tx_: * **Mortality of 5-7%** (as high as 18% in some studies) * **Want to restore/maintain weight, normalize eating, normalize psychological & family functioning** * **Family based therapy & nutrition rehab (1st line):** * **_Maudsley approach_ → families assume responsibility for healthy eating** * **Meds are not effective**, other than treating sxs → Can try SSRIs or benzos * Inpatient indications: severe malnutrition, physiologic instability (brady 50, hypotensive 80/50, hypothermic 96F, cardiac dysrhythmia, arrested growth, failure of outpatient tx, acute food refusal)

Answer 329

severe malnutrition, physiologic instability (brady 50, hypotensive 80/50, hypothermic 96F, cardiac dysrhythmia, arrested growth, failure of outpatient tx, acute food refusal)

Answer 330

starvation, **suicide**, electrolyte imbalance, **amenorrhea**, osteoporosis, bradycardia, hypotension, constipation, acid-base imbalance, pedal edema, **hypokalemia**

Answer 331

* _Definition_: * eating disorder characterized by frequent **binge eating** combined with **compensatory behavior** to prevent weight gain * _Comorbidities_: * depression, anxiety, substance abuse, borderline personality disorder, PTSD * _Epidemiology_: * 0.9-3% of population, 40% of college-aged women have disordered eating, onset later at **13-18yo** * _S/sxs_: * **Normal weight** (usually) * -recurrent episodes of binge eating * -Recurrent inappropriate compensatory behaviors: purging (90%), laxative abuse (33%), diuretics, abnormal amounts of exercise * -Self-eval focused on body shape & weight * _PE_: * **parotid gland enlargement** * **-Russel’s Sign**: calluses on dorsum of hand from self-induced vomiting * **_DSM-V Criteria_** * A) **Recurrent episodes of binge eating** * B) **Recurrent inappropriate compensatory behavior** to prevent weight gain * C) **At least 1x/week for 3 months** * D) Self-evaluation is unduly influenced by body shape/weight * E) This disturbance does not occur exclusively during episodes of AN * _Tx_: * Psychotherapy * **Fluoxetine (Prozac)**: only FDA-approved med

Answer 332

**hypokalemia**, **suicide/suicidal ideation**, gastric esophageal, bowel abnormalities, erosion of dental enamel, parotid enlargement

Answer 333

* _Definition_: * sxs that cause clinical distress and/or impairment in social, occupational, or other important areas of functioning, but **do not meet full criteria** for other eating disorders. * _Dx_: * Insufficient information to make a more specific diagnosis * _Tx_: * Psychotherapy, **CBT = best** * Dependent on sxs–antidepressants may be helpful

Answer 334

* _Definition_: * neurodevelopmental disorder characterized by deficits in intellectual functioning, such as reasoning, problem solving, planning, abstract thinking, judgment, academic learning, & learning from experience * _Types_: * **Mild** (85%): academic skills to 6th grade, minimum self-support * **Moderate**: academic skills to 3rd grade, provide self-care hygiene * **Severe:** rudimentary communication, simple skills * **Profound:** sensory motor problems * **_DSM-V Criteria_:** * Deficits in intellectual functions, such as reasoning, problem solving, planning, abstract thinking, judgment, academic learning, learning from experience, confirmed by assessment & testing * Impairment in adaptive functioning; failure to meet developmental goals * onset of intellectual & adaptive deficits – developmental period * Children's IQ no longer used

Answer 335

* _Definition_: * spectrum of developmental disorders characterized by impairment in social interaction, communication, restricted repetitive stereotyped behaviors, and other signs leading to impaired social functioning * _Etiology_: unknown * NOT d/t poor maternal bonding/parenting or immunizations * Hypotheses: obstetric complications, infection, genetics, & toxic exposures * _Risks_: * **age of parent**, maternal use of **valproate** during pregnancy, **sibling with ASD** * _S/sxs_: * **Developmental regression at** 15-30 months * Absence of pointing * -Abnormal reaction to environmental stimuli (not easily consoled) * **-Absence of symbolic play & social interaction** (ex. Stop playing with blocks) * -**Repetitive behavior** (fixated interest, resistance to change) * _Deficits in Social Interaction_: * Social/emotional reciprocity deficits (ex. Smiling back) * Social non-verbal communication deficits * Deficits in understanding, developing, & maintaining social relationships * Language development & verbal IQ more positive prognosis * _Screening Tools_: * **Autism screening checklist** **(MCHAT)**: 19-item screening, quick yes/no for parents * _Tx_: * **\*Refer** to specialty practice for neuropsychological testing, behavioral modification strategies, and medications * Special education * -behavior modification * -pivotal response treatment * -neuroleptics for aggression or self-injury * -long-term parental support & psychoeducation

Answer 336

* _Definition_: * neurodevelopmental disorder characterized by problems paying attention, impulsivity, & hyperactivity that is not age appropriate * _S/sxs_: * -Trouble paying attention * -Hard to stay still * -Trouble finishing schoolwork * -Hard not to blurt out things or interrupt * -Trouble finding or keeping track of things * -Trouble finishing chores/tasks at home * -got into arguments or fights with others * -got into trouble for not listening * **_DSM-V Criteria_:** * -**Inattention (****6+ for 6+ months**) * fails to pay attention, difficulty sustaining attention in task, does not seem to listen, does not follow through on activities, difficulty organizing tasks, avoid tasks, loses items for tasks, easily distracted, forgetful in daily activities * **Hyperactivity & Impulsivity (****6+ for 6+ months**): * fidgets, leaves seat when sitting is expected, runs or climbs when not appropriate, unable to engage in leisure activities, often “on the go”, talk excessively, blurts out answers, difficulty waiting for turn, interrupts frequently

Answer 337

* _Evaluation of ADHD in Primary Care_: * dx takes time: minimum of 60 minute eval may be over 3+ sessions * Dx tool include Vanderbilt or Conners forms from multiple settings (home, school, etc.) * Neuropsychiatric EEG-Based Assessment Aid (NEBA) system * _Screening Tools_: * Vanderbilt * Conners Forms * _Prognosis_: * ⅔ persist into adolescence, ⅓-⅔ persist as adults * _Tx_: * Refer to specialty care for assessment (takes a long time to complete) * **Medication: 1st line treatment**; stimulants, alpha-agonists, Strattera, Wellbutrin (bupropion) Strattera & wellbutrin are for pts refractory to tx * _Psychosocial Tx:_ * help with organization, family understanding, tips for teachers

Answer 338

* _Types_: * Specific phobias: 15% lifetime prevalence * Social Anxiety Disorder: 9% lifetime prevalence * Separation Anxiety: 2-4%, F \> M * Generalized Anxiety Disorder: 1% lifetime * Selective Mutism: 0.5-0.75% * _Risks_: * behavioral inhibition, access to support system, insecure attachment, cognitive factors, developmental events, traumatic events * _Tx_: * Psychotherapy = 1st line, child & parent counseling, **CBT** * Pharmacotherapy: **SSRI = 1st line**, trazodone, hydroxyzine, buspirone, mirtazapine * 2nd line); avoid benzos & atypical antipsychotics * Normalize sxs: * framing sxs in positive manner (“it’s your body getting ready to take care of you if something scary happens”) * Wellness: * sleep, nutritional intake, physical activity, social support

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