UWorld Flashcards
What is an indication for prophylactic administration of anti-D Ig for RhD - patients
- <72 hours after delivery of RhD + infant
- <72 hours after spontaneous abortion
- ectopic pregnancy
- threatened abortion
- 2nd/3rd trimester bleeding
- at 28-32 weeks gestation
- hydatidiform mole
- chorionic villus sampling, amniocentesis
What labs/studies are performed during a initial prenatal visit?
- CBC
- Blood antibody and Rh typing
- Pap smear
- gonorrhea/chlamydia screening
- urinalysis
- RPR/VDRL
- rubella and varicella antibody titer
- hep B surface antigen
- HIV screening
What labs/studies done at 16-18 weeks prenatal screening?
Quadruple screening (AFP, hcG, unconjugated estriol, maternal serum inhibin A)
What condition has the following quadruple screen results: AFP low, estriol low, hCG high, inhibin A high
Down syndrome
What test is done at 18-20 weeks prenatal screening
Anatomy scan to check for gross fetal abnormalities
What test is done at 24-28 weeks prenatal screening
1 hr glucose challenge to screen for GDM
What study/test is done at 32-37 weeks prenatal visit?
- Gonorrhea/chlamydia cervical culture
- group B strep testing
What is the mnemonic for vaccine schedule for pediatric population
Birth- Hep B
2mo- Hep B, Rota, DTaP, HiB, PCV, IPV
4mo- Rota, DTaP, HiB, PCV, IPV
6mo- Hep B, Rota, DTaP, HiB, PCV, IPV
12mo- Varicella, MMR, Hep A, DTaP, HiB, IPV, PCV
4-6yr- Varicella, DTaP, IPV, MMR
B
B DR HIP
DR HIP
B DR HIP
Very MAD HIP-ster
Very DIM
What is the most common primary malignant tumor that is more common in adolescents?
Osteosarcoma
What are the risk factors for osteosarcoma
Paget disease of bone, p53 genetic mutations, familial retinoblastoma, radiation exposure, bone infarcts
What regions do osteosarcoma involve
Proximal tibia, proximal humerus, distal femur
How does osteosarcoma present clinically and what are the labs and imaging for it
Presents as deep bony pain
Labs- high alk phos, high ESR, high LDH
Dx- biopsy of bone
Imaging- x ray shows sunburst pattern and Codman triangle, chest CT commonly done to check for metastases
Treatment for osteosarcoma
Radical surgical excision, chemotherapy
What is a highly malignant cartilage tumor occurring in the diaphysis of long bones and is most common in children
Ewing sarcoma
How does Ewing sarcoma present clinically and what are labs and imaging for it show
Ewing sarcoma presents as bony pain, tissue swelling, fever, fatigue, fractures with minor trauma
Labs- high WBCs, low Hgb, high ESR
dx- biopsy
Radiology- large destructive lesions, onion skinning of bone
Treatment for osteosarcoma
radiation, adjuvant chemo, radical excision
What is the most common benign bony tumor in metaphysis of long bone
Osteochondroma
How does osteochondroma present and where is it commonly found
Presents as irritated soft tissue overlying mass, palpable hard mass
Occurs in lower femur or upper tibia
What does osteochondroma show on imaging
bony growth off metaphysis of long bone, cancellous portion of long bone continuous with interior of lesion
Treatment for osteochondroma
None, unless causing soft tissue irritation or neurovascular compromise or continued growth occurs (surgery)
What appears as sclerotic cortical lesion on imaging with a central nidus of lucency
Osteoid osteoma
How does osteoid osteoma present and how is it treated
Presents as pain worse at night and unrelated to activity
Treated with NSAIDS
What is the cause of sheehan syndrome
Obstetric hemorrhage complicated by hypotension
- causes postpartum anterior pituitary infarction
What are the clinical features of sheehan syndrome
- lactation failure (low prolactin)
- amenorrhea, hot flashes, vag atrophy (low FSH and LH)
- fatigue and brady (low TSH)
- anorexia, weight loss, hypotension (low ACTH)
- low lean body mass (low GH)
How do you diagnose Sheehan syndrome- what hormone levels do you check
Cortisol
Thyroxine
FSH
Prolactin
How do you treat Sheehan syndrome
Corticosteroids
Estrogen-progestin replacement therapy
Damage to endometrial basalis layer with D&C causes intrauterine adhesions and subsequent amenorrhea in what condition
Asherman syndrome
What causes acute adrenal insufficiency (adrenal crisis)
- Adrenal hemorrhage or infarction
- illness/injury/surgery
- pituitary apoplexy
What are the clinical features of adrenal insufficiency
- hypotension and shock
- nausea, vomiting, abdominal pain
- fever, generalized weakness
How do you treat adrenal crisis
- hydrocortisone or dexamethasone
- rapid IV volume repletion
Primary Adrenal Insufficiency vs Secondary Adrenal Insufficiency vs Tertiary Adrenal Insufficiency
1- due to impairment of adrenal glands (Addisons disease)
2- caused by impairment of pituitary gland
3- due to hypothalamic disease and decrease in CRF (corticotropin releasing factor)
Lab values in 1 vs 2 vs 3 adrenal insufficiency
1- low cortisol, low DHEAS, low androgens, high CRH, high ACTH
2- low cortisol, low DHEAS, low ACTH, high CRH
3- low cortisol, low DHEAS, low ACTH, low CRH
Causes of 1 adrenal insufficiency
Autoimmune > TB
drugs, infarction, hemorrhage, infiltration
SYMPTOMS
Salt cravings (because of low aldosterone)
Low libido (low androgens)
Low blood pressure
High melanin (hyperpigmentation)
Causes of 2 adrenal insufficiency
Adenoma, surgery, radiation, autoimmune
Symptoms of 2 adrenal insufficiency
Bitemporal hemianopsia is sometimes seen
What is the name of a rash that is described as diffuse red skin
Erythematous rash
Common febrile/toxic etiologies of erythematous rash
TEN (toxic epidermal necrolysis)
SSSS (staphylococcal scalded skin syndrome)
TSS (toxic shock syndrome)
Kawasaki (Pediatrics)
Scarlet Fever
Common afebrile erythematous rashes
Allergic reaction/ anaphylaxis
TEN (can be febrile or afebrile)
Macules (flat, red splotches) and papules (solid and raised) rash
Maculopapular rash
Common febrile/toxic maculopapular rashes
Viral exanthem (most common)
Lyme disease
RMSF
Meningococcemia
Syphilis
SJS
Erythema Multiforme
Afebrile etiologies of maculopapular rash
Drug reaction
Pityriasis Rosea
Scabies
Eczema
Psoriasis
Small, red (blood leak from capillaries), no blanching, may start in dependent areas
Petechial Rash
Febrile/toxic causes of petechial rash
Palpable:
RMSF
Meningococcemia
Endocarditis
Disseminated Gonoccoal
Afebrile Petechial Rash
ITP
Petechiae >0.5cm, no blanching, may be palpable
Pupuric rash
Common febrile/toxic causes of pupuric rash
Palpable: HSP
Non palpable: DIC, TTP
Afebrile causes of purpuric rash
TTP
Autoimmune vasculitis
Fluid filled lesions, vesicles <1cm, bullae >1cm
Vesiculobullous rash
Common febrile/toxic causes of vesiculobullous rashes
Disseminated:
varicella
smallpox
disseminated gonoccus
DIC
Localized
Hand, food and mouth
necrotizing fascitis
Afebrile causes of vesiculobullous rash
Diffuse:
Pemphigus vulgaris
Bullous pemphigoid
Localized:
Herpes zoster
Burns
Contact dermatitis
ITP symptoms
- thrombocytopenia (isolated low platelets)
- petechiae (non blanching red spots)
- can have bleeding from gums
- otherwise systemically well
ITP is commonly seen in what population
often in children
viral prodrome about 3 weeks prior
Treatment for acute ITP
Self limiting
IVIG/Steroids for active bleeding
platelet transfusion for life threatening bleeding
TTP symptoms
Neuro symptoms (AMS, seizure, stroke)
Fever
Hemolytic anemia
Thrombocytopenia
Renal involvement (proteinuria/renal failure)
TTP diagnosis
subendothelial and intraluminal deposits of fibrin and platelet aggregates in capillaries and arterioles (thrombotic events)
Treatment for TTP
consult hematology
plasma exchange with FFP
How is a pleural effusion described or is diagnosed on chest x ray
Pleural effusion is the accumulation of excess fluid between the layers of the pleura
- can present as shortness of breath, chest pain, cough
Pleural effusion on chest x-ray
pleural effusions appear white, contrasting with the air space which looks black
- small effusions are seen as a meniscus of increased density at the costophrenic angle
- large volume effusions cause pressure on the adjacent lung leading to collapse
- if the volume of effusion is greater than the degree of collapse, there will be accompanying mediastinal shift
What is transudative pleural effusion
Imbalance in the production and removal of pleural fluid due to systemic factors like heart failure or liver disease
What is exudative pleural effusion
Arising due to inflammation or damage to the pleura or nearby organs
Common causes of transudative pleural effusion
CHF
Cirrhosis
Nephrotic Syndrome
Atelectasis
peritoneal dialysis
Exudative Causes of Pleural Effusion
Pneumonia
Malignancies (cancer)
Tuberculosis
Pulmonary Embolism
Autoimmune Disorders (SLE, RA)
Pancreatitis (inflammation of pancreas can lead to fluid build up in pleural space)
Trauma (hemothorax or pneumothorax)
How do you treat a small pleural effusion
Observation
If pleural effusion is small and asymptomatic- just monitor
How do you treat large pleural effusions
For large effusions causing SOB, use thoracentesis to remove the fluid
What is lights criteria?
Pleural effusion is EXUDATIVE if
pleural fluid protein/serum protein >0.5
pleural fluid LDH/serum LDH >0.6
pleural fluid LDH is >2/3 upper limit of lab normal value for serum LDH
What does purulent pleural fluid indicate
Infection
What does bloody pleural fluid indicate
Malignancy, PE or trauma
What is the most common renal malignancy in children in ages 2-5
Wilms tumor (nephroblastoma)
What does WAGR stand for in association with nephroblastoma
WAGR
W- Wilms tumor
A- Aniridia
G- Genitourinary abnormalities
R- mental Retardation
Beckwieth-Wiedemann Syndrome
Denys Drash Syndrome
How does a nephroblastoma present
Usually asymptomatic
Unilateral abdominal mass
+/- abdominal pain, hypertension, hematuria
How do you treat nephroblastoma
Surgical excision
Chemotherapy
+/- radiation therapy
How do you diagnose Wilms tumor
Abdominal ultrasound 1st to differentiate Wilms tumor from other abdominal masses
Contrast- enhanced CT or MRI 2nd to evaluate the extent of the mass
CT of chest to identify pulmonary mets
What is a neuroblastoma
Tumor of neural crest cell origin that may arise in adrenal glands or sympathetic ganglia
What are risk factors for neuroblastoma
Neurofibromatosis
Tuberous Sclerosis
Pheochromocytoma
Beckwieth-Wiedemann Syndrome
Turner Syndrome
How does a neuroblastoma present clinically
abnormal distention and pain
weight loss
malaise
bone pain
diarrhea
abdominal mass
possible Horner syndrome and proptosis
What lab findings are seen with neuroblastoma
Possible increased vanillymandellic and homvanillic acids in 24hr urine collection used to detect catecholamine secreting tumors like NEUROBLASTOMA
What is 5-HIAA test used to diagnose
Carcinoid Syndrome
Symptoms of Carcinoid Syndrome
Serotonin secreting tumors- flushing, diarrhea, wheezing, heart problems
How is a neuroblastoma diagnosed
CT may locate adrenal or ganglion tumor
How is neuroblastoma treated
surgical resection
chemotherapy
radiation
When does erythema toxicum neonatorum present
birth to 3 days of age
What is the name of a benign neonatal rash that is described as pustules with erythematous base on trunk and proximal extremities
Erythema toxicum neonatorum
How do you treat erythema toxicum neonatorum
Observation
Usually resolves within a week
When do milia present
Birth
Firm white papules on face benign neonatal rash
Milia
How do you treat milia
Observation
Resolves within a month
Erythematous papular rash on occluded and intertriginous areas at any age (not at birth)
Miliaria rubra
Treatment for miliara rubra
Avoid overheating
If severe, topical corticosteroid
Nonerythematous pustules that evolve int hyperpigmented macules with collarette of scale
Diffuse, may involve palms and soles
Neonatal pustular melanosis
What is the treatment for neonatal pustular melanosis
Observation
Pustules resolve within days
Hyperpigmentation may last months
Erythematous papules and pustules on face and scalp only that appear around 3 weeks of age
Neonatal cephalic pustulosis
Treatment for neonatal cephalic pustulosis
Observation
Resolves in weeks to months
If severe, topical corticosteroid or ketoconazole
What is the ectopic implantation of endometrial glands
Endometriosis
What disease presents as dyspareunia, dysmenorrhea, chronic pelvic pain, infertility, dyschezia, cyclic dysuria, hematuria
Endometriosis
Physical examination findings of endometriosis
immobile uterus
Cervical motion tenderness
adnexal mass
rectovaginal septum, posterior cul-de-sac, uterosaral ligament nodules
How do you diagnose endometriosis
Direct visualization and surgical biopsy
What is the treatment for endometriosis
Oral contraceptive pills
NSAIDs
surgical resection
Inflammation inside the uterine cavity that can cause infertility by creating intrauterine adhesions that prevent pregnancy implantation
Endometritis
What are the symptoms of endometritis e
Commonly occurs after childbirth or miscarriage or cervical/uterine surgery (C-section)
Presents as fever, pelvic pain, vaginal bleeding or discharge, constipation, swelling in your abdomen
Benign growth of endometrial glands and stroma that present with painless intermenstrual spotting
Endometrial polyps
Heavy prolonged menstrual bleeding
Submucosal fibroids
Uterine fibroids are also known as
Uterine leiomyoma
Benign uterine masses composed of smooth muscle within myometrium, regress after menopause usually
Leiomyoma
Risk factors for leiomyoma
nulliparity
AA heritage
diet high in meats and alcohol
family hx
Leimyoma symptoms
possible menorrhagia, pelvis pressure or pain, palpable mass on examination
Imaging for leiomyoma
Transvaginal US or hysteroscopy
Treatment for uterine leiomyoma
Follow asymptopmatic fibroids with US to detect abnormal growth
GnRH agonists to reduce uterine bleeding and fibroid size
Myomectomy for resection of symptomatic fibroids in women wishing to maintain fertility
Hysterectomy in patients whose fertility is not a concern
Lining of uterus grows into walls of uterus
uterine adenomyosis
symptoms of adenomyosis
heavy menstrual period, pelvic pain, discomfort during sex
Treatment for adenomyosis
NSAIDS to soothe menstrual cramps
OCPs to lighten flow
IUD to help cramps and flow
Surgery - endometrial ablation, uterine artery embolization (affects fertility), hysterectomy
Rapid onset of periumbilic pain (severe) that is out of proportion to examination findings and late presentation of hematochezia
Acute mesenteric ischemia
Risk factors for acute mesenteric ischemia
Atherosclerosis
Embolic source (thrombus, cardiac vegetation)
Hypercoagulable disorder
Lab findings for AMI
leukocytosis
elevated amylase and phosphate level
metabolic acidosis
Diagnosis for Acute mesenteric ischemia
CT (preferred) or MR angiography
Age >50, bilateral pain and morning stiffness > 1 month, involving 2 of the following (neck or torso, shoulder or proximal arms, proximal thigh or hip, constitutional symptoms)
Polymyalgia rheumatica
PE findings on polymyalgia rheumatic
decreased active ROM in shoulders, neck and hips
Lab values in PMR polymyalgia rheumatica
High ESR
High CRP
normocytic anemia
Tx for polymyalgia rheumatica
Oral glucocorticoids (low dose- prednisone 10-20mg)
vs high dose glucocorticoids for patients with suspected GCA
SLE immunologic markers
ANA
Anti-dsDNA
Anti-Sm Ab
False positive RPR or VDRL
Immunologic markers for drug induced lupus
Antihistone antibodies
ANA
Immunologic markers for RA
RF
Anticitrullinated peptide antibodies (>90% specific)
ANA
HLA-DR4 common
Immunologic markers for polymyositis or dermatomyositis
ANA
Anti-Jo 1 antibodies in patients with interstitial lung disease
Immunologic marker for ankylosing spondylitis
HLA-B27
Immunologic markers for scleroderma
Anti-scl 70 ANA
immunologic markers for CREST syndrome
anticentromere antibodies
Immunologic markers for Sjogren syndrome
Anti-Ro (anti SSA) ANA
Anti-La (anti SSB) ANA
Rheumatoid arthritis causes
Autoimmune disease- leads to inflammation and joint damage
RA symptoms
Affects small joints in hands and feet
morning stiffness lasting >1hr, low fevers, rheumatoid nodules
RA risk factors
female
smoking
exposure to dangerous chemicals like asbestos or silica
OA causes
wear and tear on your joint cartilage causing cartilage break down due to daily use and aging
OA symptoms
Affects joints you use most (hands and spine) and weight bearing joints (hips and knees)
Pain, stiffness, swollen joints, joint noises during movement
OA risk factors
genetic
excess weight
older age, joint injury, overuse of joints
OA treatment
NSAIDs
non medicinal relief (hot or cold packs)
RA treatment
DMARDs (disease modifying antirheumatic drugs)- suppress immune system and reduce inflammation
Traditional DMARDs - methotrexate, sulfasalazine, hydroxychloroquine, leflunomide, azathioprine
Biologic DMARDs- target specific modules, cells and pathways causing inflammation
ALS tracts affected and symptoms
Corticospinal tract and ventral horn affected
Symptoms- spastic and flaccid paralysis (UMN and LMN symptoms)
Poliomyelitis tracts affected and symptoms
Ventral horn affected
Symptoms- flaccid paralysis (LMN lesion)
Tabes dorsalis tracts affected and symptoms
Dorsal columns affected
Symptoms- impaired proprioception and pain
Spinal artery syndrome tracts affected and symptoms
Corticospinal tract, spinothalamic tract, ventral horn, lateral gray matter affected- DORSAL COLUMNS SPARED
Symptoms-
bilateral loss of pain and temperature one level below lesion
bilateral spastic paresis (below lesion)
bilateral flaccid paralysis (level of lesion)
Vit B12 def tracts affected and symptoms
Dorsal columns and corticospinal tract
Symptoms- bilateral loss of vibration and discrimination and bilateral spastic paresis affecting legs before arms
Syringomyelia tracts affected and symptoms
Ventral horn
Symptoms- bilateral loss of pain and temperature one level below lesion and bilateral flaccid paralysis at level of lesion
Brown Sequard Syndrome tracts affected and symptoms
all tracts on one side of cord affected
Symptoms- ipsilateral loss of vibration and discrimination
ipsilateral spastic paresis
ipsilateral flaccid paralysis
contralateral loss of pain and temeparature
UMN symptoms
Hypertonic, spastic paralysis, hyperreflexia, Babinski sign (upgoing plantars)
LMN symptoms
Hypotonia, flaccid paralysis, fasciculations, downgoing plantars (absent Babisnki)
Post Op Fever Days 1-2
Wind
- atelectasis #1
- pneumonia
Diagnosis - CXR, sputum culture if pneumonia is suspected
Tx- incentive spirometry, antibiotics (vanc + pip/tazo)
Post Op Fever Days 3-5
Water
UTI
Dx- urinalysis (nitrite and leukocyte esterase +)
Tx- antibiotics
Post op Fever Days 5-6
Walking
DVT
Thrombophlebitis (IV site infection)
Dx- Doppler US
Tx- anticoagulation- heparin then warfarin
Replace IVs
Post Op Fever Day 7
Wound
Incision Site wound
Infectious Cellulitis
Dx- physical exam: erythema, pus, swelling
Tx- abscess incision/drainage, antibiotics
Post Op Fever Day 8-15
Wonder Drugs
Drug Reaction
Deep Abscess
Dx- D/C likely medication, CT scan
Tx- drainage of abscess
Tuberculosis Symptoms
Chronic cough >3 weeks
weight loss
night sweats
fatigue
sometimes hemoptysis
can affect other organs- pleuritic chest pain, neuro symptoms in TB meningitis
Pneumonia symptoms
Sudden onset of high fever
productive cough with yellow or green sputum
Chest pain
SOB
Fatigue
Common causes of TB
Mycobacterium tuberculosis cause
- spreads through airborne droplets when infected person coughs or sneezes
Pneumonia causes
Commonly bacteria (S. pneumo)
Viruses (flu, RSV)
Imaging findings for TB
Irregular patches (lesions) in lungs especially in upper lobes
Cavitation is a characteristic feature
TB infections tends to start in the top of the lungs
Pneumonia imaging features
Consolidated areas (infiltrates) in the lungs typically in the lower lobes
Effusions can also be seen
Diagnosis for TB
Skin test (tuberculin test
Blood test
Chest x ray for characteristic lesions
Sputum test to detect TB in mucus
Diagnosis for pneumonia
Chest X ray to see infiltrates
Blood test to assess inflammation and infection markers
TB treatment
latent TB: treated with isoniazid for 3-4 months active TB: RIPE protocol (rifampin, isoniazid, pyrazinamide, ethambutol)
Pneumonia Treatment
antibiotics specific to causative agent
Supportive care
Primary Hyperaldosteronism (CONN SYNDROME) causes
Caused by issues within the adrenal glands
- Adrenal adenomas (benign tumors)
- Bilateral Adrenal Hyperplasia (enlargement of both adrenal glands)
Secondary Hyperaldosteronism causes
Occurs due to problems outside of the adrenal glands (reduced kidney blood flow, heart failure, liver cirrhosis)
- activation of RAAS secondary to perceived low blood pressure in the kidneys (renal artery stenosis, heart failure, cirrhosis, nephrotic syndrome)
Presenting symptoms of hyperaldosteronism
headache, weakness, paresthesia, recalcitrant HTN, tetany
Lab findings in hyperaldosteronism
decreased K+ (because of high aldosterone)
metabolic alkalosis
mildly increased Na_
increased 24 hour urine aldosterone
high ratio of plasma aldosterone concentration to plasma renin activity (PAC: PRA) ratio indicated primary hyperaldosteronism
Imaging findings for hyperaldosteronism
CT or MRI may detect adrenal mass
Treatment for hyperaldosteronism
surgical resection of tumor (primary hyperaldosteronism)
treat underlying disorder causing RAA hyperactivity (secondary hyperaldosteronism)
aldosterone antagonists (spironolactone) improve hypokalemia until definitive therapy administered
Primary Adrenal Insufficiency causes
mineralocorticoid (aldosterone) deficiency
glucocorticoid (cortisol) deficiency
caused by adrenal disease or ACTH insufficiency
Addison Disease pathophysiology
Primary adrenal insufficiency
-autoimmune destruction of adrenal cortices caused by autoimmune disease
Secondary corticoadrenal insufficiency causes
insufficient ACTH production by pituitary
Tertiary corticoadrenal insufficiency causes
insufficient corticotropin releasing hormone (CRH) secretion by hypothalamus (commonly due to chronic corticosteroid use)
Symptoms of adrenal insufficiency
weakness
fatigue
anorexia
weight loss
nausea and vomiting
arthralgias
decreased libido in women
memory impairment
depression
HYPOTENSION
possible INCREASE IN SKIN PIGMENTATION
Lab values in adrenal insufficiency
Low Na+ and high K+ due to low aldosterone, eosinophilia and decreased cortisol
High ACTH with Addison disease
Low ACTH with secondary or tertiary insufficiency
Low cortisol that increases following ACTH analog (cosyntropin) administration in secondary or tertiary insufficiency but not in Addison disease
Treatment for adrenal insufficiency
Treat underlying disease
Glucocorticoid replacement (hydrocortisone, dexamethasone, prednisone)
Mineralocorticoid replacement
DHEA
hydration to achieve adequate volume status
Complications of adrenal insufficiency
Addisonian crisis- severe weakness, fever, mental status changes, vascular collapse caused by stress and increased cortisol need
- treat with IV glucose and hydrocortisone or vasopressors
Causes of Hematuria
Nephrolithiasis
UTI
BPH
Bladder Inflammation (cystitis)
Kidney disease
Trauma or injury
Endometriosis
Sickle Cell Disease
Bladder Cancer epidemiology and population at risk
> 90% risk with urothelial carcinoma
High risk with smokers and exposure to industrial carcinogens
Symptoms of bladder cancer
Painless hematuria throughout micturition
Irritative voiding symptoms (frequency, urgency, dysuria)
Regional pain
Dx for bladder cancer
GOLD STANDARD- flexible cystoscopy with biopsy
Urine cytology
Staging of bladder cancer
TURBT
Upper urinary tract imaging
Tx for bladder cancer
No muscle invasion: TURBT and intravesical immunotherapy
Muscle invasion: radical cystectomy and systemic chemotherapy
metastatic bladder cancer- systemic chemotherapy and immunotherapy
Nephritic Syndrome symptoms
Acute hematuria and proteinuria secondary to glomerular inflammation
Commonly see oliguria and gross hematuria
Nephritic Syndrome labs
increased BUN
increased Cr
hematuria and proteinuria on UA
24 hr urine collection- protein <3.5g/day
Nephrotic Syndrome symptoms
Significant proteinuria associated with hypoalbuminemia and hyperlipidemia
Edema
Foamy urine
Dyspnea
Hypertension
Ascites
Nephrotic Syndrome labs
Decreased albumin and hyperlipidemia
proteinuria >3.5g/day seen on 24hr urine collection
Nephritic Syndromes
PSGN
IgA nephropathy
Goodpasture Syndrome
Alport Syndrome
RPGN
Lupus nephritis
Wegener granulomatosis
Nephrotic Syndromes
Minimal change disease
FSGN
Membranous nephropathy
MPGN
Diabetic Nephropathy
Amyloidosis
PSGN pathology, symptoms, labs, treatment
PSGN- caused by Group A strep infection
Symptoms- recent infection, oliguria, edema, brown urine, hypertension, common in children
Labs- hematuria and proteinuria in UA, high ASO titer, subepithelial humps of IgG and C3 on renal basement membrane on electron microscopy
Treatment- self limited, supportive care
IgA nephropathy (Berger disease) pathology, symptoms, labs, treatment
Deposition of IgA immune complexes in mesangial cells
Commonly few days after infection while PSGN is weeks after infection
symptoms- hematuria, flank pain, low grade fever
labs- high serum IgA, mesangial cell proliferation on electron microscopy
Tx- occasional self limited, ACE-I and statins for persistent proteinuria
Goodpasture syndrome pathology, symptoms, labs, treatment
Pathology- deposition of antiglomerular and antialveolar basement membrane antibodies
Symptoms- dyspnea, hemoptysis, myalgias, hematuria
labs- serum IgG antiglomerular basement membrane Ab, anemia, LINEAR PATTERN OF IgG AB DEPOSITION
Tx- plasmapheresis, corticosteroids, immunosuppressive agents
Alport Syndrome pathology, symptoms, labs, treatment
Hereditary defect in collage IV in basement membrane
Sx- hematuria, high frequency hearing loss, eye disease (CANT PEE, CANT SEE, CANT HEAR A BEE)
Labs- red cell casts, split basement membrane on electron microscopy
tx- ace-I may reduce proteinuria
renal transplant may be complicated by alport related development of goodpasture syndrome
RPGN pathology, symptoms, labs, treatment
Rapidly progressive renal failure from idiopathic causes or associated with other glomerular diseases or systemic infection
sx- sudden renal failure, weakness nausea, weight loss, dyspnea, hemoptysis, myalgias, fever, oliguria
labs- deposition of inflammatory cells in Bowman capsule and crescent formation
- pauci immune RPGN is ANCA1
tx- poor prognosis, tx with corticosteroids plasmapharesis and immunosuppressives, renal transplant frequently required
Lupus nephritis pathology, symptoms, labs, tx
Complication of SLE involving proilferation of endothelial and mesangial cells
sx- possible hypertension or renal failure
labs- ANA, Anti-DNA antibodies, hematuria and possible proteinuria
tx- corticosteroids or immunosuppressives
ace-I and statins to help reduce proteinuria
Wegeners granulomatosis pathology, symptoms, labs, tx
Similar to crescentic disease with addition of pulm involvement- granulomatous inflammation of airways and renal vasculature
symptoms- weight loss, resp symptoms, hematuria, fever
labs- cANCA,, deposition of immune complexes in renal vessels on electron microscopy
tx- corticosteroids, cytotoxic agents (cyclophosphamide)
Minimal change disease pathology, symptoms, labs, tx
Idiopathic cause, may involve effacement of podocytes on basement membrane
symptoms= possible HTN, increased frequency of infections, most common cause of nephrotic syndrome in children
labs- HLD, hypoalbuminemia, proteinuria, flattening of podocytes on electron microscopy
tx- corticosteroids
FSGN pathology, symptoms, labs, tx
Associated with drug use of HIV, systemic sclerosis of glomeruli
Sx- possible HTN, most common cause of nephrotic syndrome in adults
Labs- HLD, hypoalbuminemia, hematuria, high proteinuria on UA, sclerotic changes in glomeruli
tx- corticosteroids, Ace-I, statins
Membranous nephropathy pathology, sx, labs, tx
Idiopathic or associated with infection, SLE, neoplasm or drugs causes, thickening of basement membrane
Symptoms- edema, dyspnea, hx of infection or medication use, associated with Hep B and hep C
labs- HLD, hypoalbuminemia, proteinuria on UA, spike and dome basement membrane thickening on electron microscopy
tx- corticosteroids, ace-I, statins
Membranoproliferative glomeruloneprhitis pathology, sx, labs, tx
idiopathic or associated with infection o autoimmune disease
thickening of basement membrane, associated with HepB and HepC, SLE< and subacute bacterial endocarditis
sx- edema, HTN, hx of systemic infection or autoimmune condition
labs- basement membrane thickening with double layer tram track appearance on electron microscopy
Diabetic nephropathy pathology, sx, labs, tx
basement membrane and mesangial thickening related to diabetic vascular changes
sx- hx of DM, hypertension, progressive renal failure
labs- nephrotic symptoms, basement membrane thickening, round nodules (kimmelstein wilson nodules) in glomeruli
tx- treat underlying DM and dietary protein restriction, Ace-I and tight BP control
Amyloidosis pathology, sx, labs, tx
Deposition of amyloid protein fibrils in glomeruli and/or renal vasculature, may also involve other tissues
Sx- edema, may progress to renal failure
labs- nephrotic symptoms, congo red stain of biopsy shows apple green birefringence on polarized light
tx- melphalan, hematopoietic stem cell transplant
Unintentional
Inattentive
Distractible
Disorganized
Increased Motor Activity
2+ settings
>6 months of symptoms
ADHD
Treatment for ADHD
Behavioral therapy first line
Atomoxetine or stimulant (methylphenidate, amphetamine) improve ability to focus and control behavior
Alpha 2 agonists and TCAs may be used in refractory cases
Deceitful for gain
Violates others
Destructive
Intentionally breaks law
Truancy
Conduct disorder
Conduct disorder < 18
What disorder >18
Antisocial personality disorder
Treatment for conduct disorder
Psychotherapy, psychostimulants helpful when comorbid ADHD diagnosed, mood stabilizers in severe cases
Sensitive/Touchy
Willful
Spiteful Vindictive
Defensiveness, excuse making, potential outbursts
Oppositional defiant disorder
OCPs (combined) - side effects and ocntraindications
possible nausea, headache and bloating
increased risk for DVT
contraindicated in heavy smokers, previous hx of DVT, estrogen related cancer, liver disease or hypertriglyceridemia
Progestin only OCP side effects
- used if pt has contraindication for estrogen
- can cause breakthrough bleeding
- has to be taken at same time everyday
Depo Provera shot
- progestin analog every 3 months, inhibits ovulation and endometrial development
- SE- nausea, headache, weight gain, osteoporosis, irregular bleeding
Progestin Implant
subcutaneous implant that slowly releases progestin over 3 years
SE: irregular bleeding, breast pain
Copper IUD
Object inserted into uterus by physician with slow release of copper to prevent fertilization and interfere with sperm production
- 10 years
can be placed soon after intercourse as emergency contraception
Risks- small risk of spont abortion or uterine perfortation and menorrhagia
Progestin releasing IUD
Left in place for 5 years
- small risk of spont abortion and uterine perf
Inflammation of cornea
Infectious keratitis
Causes of infectious keratitis
Bacterial- staph, strep, pseudomonas
- central round ulcer, stromal abscess, mucopurulent discharge
Vital- HSV or herpes Zoster
- branched dendritic ulcerations, decreased corneal sensation, watery discharge, recurrent episodes
Fungi- candida
- ulcerations with feathery margins and satellite lesions, mucopurulent discharge, indolent course (vs acute course with bacteria)
Contaminated Water
Symptoms of infectious keratitis
Eye redness, pain, blurred vision, excess tears, photophobia, difficulty opening your eyelid due to pain or irritation
Lens dislocation (ectopia lentis)
Commonly caused by trauma- direct blow to eye or blunt trauma to head
Also Marfans Syndrome and homocystinuria
Symptoms of lens dislocation
Painless vision loss
Tremulous iris with eye movement
lens displacement on slitlamp examination
Marfan Syndrome symptoms
Caused by defective fibrillin which is an essential component of ligaments
Features
- unlike homocystinuria, has normal levels of homocysteine and methionine
- Ectopia lentis
- Characteristic marfanoid habitus (tall stature, increased arm to height ratio, joint hypermobility) not usually apparent in young children
- Scoliosis
- High myopia (nearsightedness)
- Aortic root disease (dilation, dissection) = major cause of morbidity and REQUIRES MARFAN PT TO GET ECHO
Homocystinuria Symptoms
Caused by mutations in CBS (cystathionine beta synthase) which converts homocysteine into cysteine
- high levels of homocysteine present
- symptoms- lens dislocation, nearsightedness, long limbs and slender fingers, neuro complications, increased risk of blood clots
Tx- Vit B6
Ehlers Danlos symptoms
Affects connective tissues in body
- causes joint hypermobility which can lead to joint dislocation
- stretchy, fragile and prone to bruising skin
- muscle pain and fatigue
heart valve problems and blood vessel fragility
Mutation in COL5A1 or COL5A2 gene causes what condition
Ehlers Danlos (classic syndrome)
- these genes encode proteins essential for forming Type V collagen
12-18 month children stop breathing
Breath Holding Spells
Cyanotic vs Pallid Breath Holding Spell
Cyanotic- child turns blue bc of lack of O2 in blood- provoked by emotional upset, last less than a minute
Pallid- child appears pale, resemble fainting, caused by sudden fright or minor pain, last longer than a minute
Systemic sclerosis vs scleroderma
Scleroderma- thickening and hardening of skin due to excessive collagen production
Systemic sclerosis- scleroderma affects skin and internal organs
Anti-topoisomerase (Scl-70) antibody seen in
diffuse systemic sclerosis and linked to lung involvement
Anticentromere antibody seen in
More common in limited systemic sclerosis and is associated with elevated blood pressure in the pulmonary system
Spur cell RBC (acanthocyte)
Irregular narrow base sharp projections of red cell membrane (spaced out projections)
- seen in:
- cirrhosis
- abetalipoproteinemia
- post splenectomy
- microangiopathic hemolytic anemia (DIC, TTP, HUS)
Burr Cell RBC (echinocyte)
regular broader-base short projections of red cell membrane
seen in:
- uremia (chronic liver disease)
- liver disease
- hyperlipidemia
Bite cells (degmacytes)
Seen in oxidative hemolysis- G6PD deficiency - uncontrolled oxidative stress causes hemoglobin to denature and form Heinz bodies
Schistocyte (helmet cell)
Seenin DIC, hemolytic anemia, frequently a consequence of mechanical artificial heart valves, HUS, TTP, MAHA
Dacrocyte (teardrop cell)
Commonly seen in primary myelofibrosis, myelodysplastic syndromes
SLE nephritis
presents as foamy urine, edema in legs ankles and face, high blood pressure, kidney dysfunction (elevated creatinine)
- increases risk of B cell lymphoma
Molar pregnancy/Hydatidiform Mole
Symptoms- vaginal bleeding, severe nausea and vomiting, uterine enlargement, rapidly rising hCG levels
Dx- hcG, transvaginal ultrasound
Choriocarcinoma symptoms
Rare and aggressive cancer that develops from cells that were part of placenta during pregnancy - occurs in uterus or ovaries
symptoms:
Irregular vaginal bleeding
pelvic pain
if it spreads to other parts of body can cause coughing, trouble breathing, headaches, abdominal pain
dx- pelvic exam, blood test to measure hCG levels, liver function and kidney function, US, CT
tx- chemotherapy, surgery may be needed
Lab values seen in choriocarcinoma
High levels of beta hCG
AFP is elevated
Elevated LDH levels can indicate tumor activity
Fluid filled swelling behind the knee joint in the popliteal fossa
Bakers cyst- extension of synovial membrane
Symptoms of bakers cyst
Visible bulge behind the knee, pain when bending or straightening knee, stiffness and limited range of motion
Dx- US and MRI
tx- aspiration of cyst fluid, cortisone injections into knee to reduce inflammation, self care
Prepatellar Bursitis
- located in prepatellar bursa
- commonly known as housemaid’s knee
- symptoms- pain, swelling and tenderness at front of the knee
- causes- repetitive kneeling or direct trauma
Pes anserine bursitis
affects bursae on the lower inner side of knee
- commonly seen in people who engage in activities like running or cycling
Infrapatellar Bursitis
- involves the bursae located just under the patella
- due to overuse, repetitive movements or direct impact
Suprapatellar Bursitis
Involves the bursae located just above the kneecap
- often seen in OA and RA
Internal rupture of respiratory system, chest wall intact
Closed pneumothorax
Types of closed pneumothorax
Spontaneous, COPD, TB, blunt trauma
Passage of air through opening in chest wall
Open pneumothorax
Types of open pneumothorax
Penetrating trauma, iatrogenic (central line placement, thoracentesis, biopsy)
Open pneumothorax, ball valve” condition allows air to enter but not leave pleural space
Tension pneumothorax
Unilateral chest pain, dyspnea, decreased chest wall movement, unilateral decreased breath sounds, increased resonance to percussion, decreased tactile fremitus, respiratory distress, hypotension
Pneumothorax
Deviation of trachea
Tension PTX
CXR of pneumothorax
lung retraction and mediastinal shift away from affected side
Tension PTX- shows tracheal deviation
Diagnosis of PTX
CXR and ultrasound
Tx of PTX
small PTX- supplemental O2
Large PTX- chest tube placement
open PTX with small wound- tx with chest tube and occlusive dressing
tension PTX- requires immediate needle decompression and chest tube placement
Collection of blood in pleural space caused by trauma, malignancy, TB or pulmonary
Hemothorax
CXR for hemothorax
resembles that for pleural effusion
blunting of costophrenic angles
upright CXR preferred
Tx for hemothorax
supplemental O2
chest tube placement
thoracotomy recommended if there is drainage of >1500mL after initial chest tube insertion or continuous drainage of 200mL/hr over 4 hours
Moderate abdominal pain and tenderness
Hematochezia, diarrhea
leukocytosis, lactic acidosis
Colonic ischemia
Colonic ischemia pathophysiology
Nonocclusive, “watershed” ischemia
- due to underlying atherosclerotic disease
- state of low blood flow (hypovolemia)
- repair of AAA is a common precipitating event as patients are often older and have extensive atherosclerotic vascular disease
Dx for colonic ischemia
CT scan- colonic wall thickening, fat stranding
Endoscopy: edematous and friable mucosa
Tx for colonic ischemia
IV fluids and bowel rest
Antibiotics with enteric coverage
Colonic resection if necrosis develops
Immature central respiratory center in preterm infants
Apnea of prematurity
Clinical features of apnea or prematurity
Intermittent apnea (cessation of respiration for >20 seconds) start at age 2-3 days
- associated bradycardia and desaturation sometimes seen
- well-appearing in between episodes
Tx for apnea of prematurity
Caffeine
Noninvasive ventilation
Resolves by expected due date
Obstructive apnea caused by severe bronchopulmonary dysplasia
occurs after 28 days
causes chronic hypoxia
tx- dexamethasone and albuterol
Premature infant with respiratory distress and normal CXR
apnea of prematurity
sepsis
intraventricular hemorrhage
hypoglycemia
hypothermia
narcosis
Premature infant with respiratory distress and abnormal CXR
RDS
TTN (premature or full term)
Pneumonia
Pneumothorax
Congenital Abnormality
Not premature baby with respiratory distress
- Meconium aspiration- meconium in amniotic fluid
- infectious- sepsis or pneumonia
- non infectious- RDS, TTN, pneumothorax, congenital abnormality
Cyanotic Heart Defects 5Ts
1 T- truncus arteriosus- one great vessel leaving heart
2 T- transposition of great arteries- pulmonary artery and aorta are transposed
3 T- tricuspid atresia- tricuspid valve fails to form
4 T- tetralogy of fallot - tetrad of cardiac defects- pulm stenosis, RVH, overriding aorta, VSD
5 T- total anomalous pulmonary venous return
5 words- pulmonary veins do not connect to left atrium
Hyperacute transplant rejection time, etiology, morphology
minutes to hours after transplant
- caused by antidonor antibodies in recipient
- gross mottling and cyanosis, arterial fibrinoid necrosis and capillary thrombotic occlusion
- untreatable, should be avoided by proper cross matching
Acute transplant rejection
occurs 6 days to 1 year after transplantation (usually <6 months)
- caused by antidonor T cell proliferation in recipient
- tx- frequently reversible through immunosuppressive agents
Chronic transplant rejection
Occurs >1 year after transplantation
- caused by multiple cellular and humoral immune reactions to donor tissue
- morphology- vascular wall thickening and luminal narrowing, interstitial fibrosis and parenchyma atrophy
tx- immunosuppression may serve some role
CMV colitis
occurs in patients with IBD, particularly those with steroid refractory colitis
- histology with immunochemistry for diagnosis
- tx- antiviral treatment
Microscopic colitis
Inflammatory disorder characterized by chronic diarrhea
- detected through histologic evaluation with mucosal biopsy
- unlike UC, microscopic colitis requires microscopic evaluation of colon tissue samples
- sx- abdominal pain and frequent stools but not typically bloody or rectal bleeding
Deceleration that begins near the onset of a uterine contraction with the lowest point occurring at the same time as the peak of the contraction
Early decelerations
Cause of early decelerations
compression of fetus head during a uterine contraction
- harmless decelerations and do not affect fetal O2
Deceleration that begins just after a contraction with the lowest point occurring after the peak of the contraction
Late decelerations
Cause of late deceleration
uteroplacental insufficiency which reduces blood flow to the placenta- leading to decreased O2 and nutrient transfer to the fetus
- causes- maternal hypotension from epidural, dehydration, anemia, rapid uterine contractions, placental abruption, fetal hypoxia
Decelerations that vary in shape, duration and intensity and don’t necessarily correlate with uterine contractions
Variable decelerations
Cause of variable decelerations
umbilical cord compression
- if decelerations become repetitive, blood delivered to fetus can be reduced and cause fetal hypoxia and acidosis
Side effects of second generation antipsychotics
metabolic syndrome- weight gain, dyslipidemia, hyperglycemia
highest risk drugs- clozapine and olanzapine
monitoring guidelines- BMI, fasting glucose and lipids, blood pressure, waist circumference
Ischemic Hepatitis symptoms
aka shock liver
- characterized by acute liver injury caused by insufficient blood flow and insufficient oxygen delivery to the liver
- due to low blood pressure or shock but also due to blood clot in hepatic artery
sx- weakness, fatigue, mental confusion, low urine production
- jaundice or hepatic coma also possible
Symptoms of primary sclerosing cholangitis
fatigue and pruritis with progressive disease
- associated with IBD, particularly UC
Lab findings on Primary sclerosing cholangitis
cholestasis (high ALP, GGT, bilirubin)
multifocal strictures/dilation of bile ducts on MRCP
Complications of PSC
cholangitis, cholangiocarcinoma, colon cancer
fat soluble vitamin deficiencies
Acute Cholangitis symptoms
RUQ pain, fever, jaundice, hypotension, altered mental status (Reynolds pentad)
- commonly occurs due to infection of extrahepatic biliary system that usually occurs due to biliary obstruction which predisposes patients to bacterial invasion
Symptoms of magnesium toxicity
Mild: nausea, flushing, headache, hyporeflexia
Moderate: areflexia, hypocalcemia, somnolence
Severe: respiratory paralysis, cardiac arrest
Tx of magnesium toxicity
Stop magnesium
Give IV calcium gluconate
Leakage of urine with coughing, sneezing, lifting
Stress incontinence
- caused by decreased urethral sphincter tone
- urethral hypermobility
Sudden overwhelming urge to urinate
Urge incontinence
- detrusor overactvity
Incomplete emptying and persistent involuntary dribbling
Overflow incontinence
- impaired detrusor contractility
- bladder outlet obstruction
Pyogenic liver abscess symptoms
Fever, RUQ pain, leukocytosis, elevated liver function studies
- rounded hypoattenuating lesion in the liver
Causes of pyogenic liver abscess
Direct extension from biliary tract infections
Penetrating trauma
hematogenous spread from the system (IE) or portal circulation (intrabdominal infection)
Diagnosis of liver abscess
well defined hypoattenuating rounded lesion often surrounded by a peripherally enhancing abscess membrane on CT scan
Treatment for liver abscess
Blood cultures
Antibiotics
PERCUTANEOUS ASPIRATION AND DRAINAGE- both diagnostic and therapeutic
Symptoms of arthropathy of hereditary hemochromatosis
onset < 40
chronic pain and bony swelling
most common at 2nd and 3rd MCP joints
occasional acute flare
X ray signs of arthropathy of hereditary hemochromatosis
joint space narrowing
chondrocalcinosis
hook-shaped osteophytes at metacarpal heads
Treatment for arthropathy of hereditary hemochromatosis
NSAIDs
therapeutic phlebotomy
What age does Hodkin lymphoma affect
Bimodal peak incidence: age 15-35 and >60
- association with EBV in immunosuppression
Symptoms of Hodkins lymphoma
Painless lymphadenopathy
Mediastinal mass
B symptoms
Pruritis
How do you diagnose hodgkin lymphoma
Lymph node biopsy
Reed Sternberg cells on histology
Most common childhood cancer in ages 2-5 years old
Acute lymphoblastic leukemia
Symptoms of ALL
Nonspecific systemic symptoms (fever, weight loss)
Leukemic cells overcrowd bone marrow- anemia, thrombocytopenia, bone pain
Extramedullary spread- LAD, hepatosplenomegaly, testicular enlargement
mediastinal mass- airway compression and/or superior vena cava syndrome
leptomeningeal spread: neuro symptoms
Diagnosis of ALL
CBC
Bone marrow biopsy (>20% blasts is diagnostic) with flow cytometry
Lumbar puncture to evaluate for CNS involvement
What is the cause of high altitude illness
Reduced PiO2 at high altitude (>2500m or 8000ft)
Complications of high altitude illness
AMS- altered mental status- headache, fatigue nausea
High altitude cerebral edema- increased cerebral blood flow, lethargy, confusion, ataxia
High altitude pulmonary edema- uneven hypoxic vasoconstriction, dyspnea, cough +/- hemoptysis, respiratory distress
Treatment for high altitude illness
supplemental oxygen
acetazolamide for AMS
dexamethasone for high altitude cerebral edema
descent to lower altitude
What is the cause of Zenker diverticulum
Impaired UES relaxation (cricopharyngeus muscle) leading to increased intraluminal pressure and herniation causing a PSEUDODIVERTICULUM
Clinical manifestations of Zenker diverticulum
Age > 60
Men
Insidious, progressive dysphagia
Halitosis, gurgling, crepitus
Regurgitation of undigested food
Aspiration
Diagnosis and Treatment of Zenker diverticulum
Diagnosis: swallow study with contrast esophagography
Treatment: surgery: cricopharyngeal myotomy and/or diverticulectomy
Clinical indicators of clostridium difficile infection progression that warrant surgery
Signs of peritonitis: diffuse abdominal tenderness, rebound tenderness
- peritonitis indicates bowel perforation and is a definitive indication for surgical exploration- laparotomy
Colonic dilation: megacolon (colonic diameter >6cm) on abdominal x-ray with associated loss of smooth muscle tone (decreased diarrhea)
Increased serum lactate: possible marker of colonic ischemia
Indications for colonic resection (total abdominal colectomy) in c diff colitis patients
necrosis
perforation
abdominal compartment syndrome
Precipitating factors for hepatic encephalopathy
drugs (sedatives, narcotics)
hypovolemia (diarrhea)
electrolyte changes (hypokalemia)
increased nitrogen loan (gi bleeding)
infection (pneumonia, UTI, SBP)
portosystemic shunting (TIPS)
Indications for TIPS (transjugular intrahepatic portosystemic shunting)
when a patient has ascites that does not respond to medical therapy (diuretics) or has ongoing active or recurrent variceal bleeding even after appropriate treatment with upper endoscopy
Clinical signs of idiopathic intracranial hypertension
Headache + vision changes
headache quality (positional)
weight changes/elevated BMI
neuro exam
What do you do when you suspect increased intracranial pressure
do a visual assessment
- visual acuity
- visual fields
- funduscopy
If visual assessment shows papilledema what do you do next
Evaluate for mass lesions
- urgent imaging (CT/MRI)
- consider venography
if mass lesion evaluation is negative then what
Lumbar puncture
- if opening pressure >250 and no signs of infection- likely IIH
What does a empty sella sign on imaging indicate
Increased CSF pressure flattening the pituitary and filling the sella space with fluid
What does flattening of the posterior sclera of the eye indicate
Transmitted pressure onto the posterior side of the globe which also compresses the optic nerve- the optic nerve sheath is an extension of the arachnoid- leading to papilledema
Howell Jolly bodies
round dark blue inclusions in red blood cells
- retained RBC nuclear remnants that are typically removed by the spleen
- the presence of them indicates either physical absence of spleen (asplenia) due to congenital absence or surgical removal or functional hyposplenism
Twin pregnancy increases risk for what maternal complications
- hyperemesis gravidarum
- preeclampsia
- gestational diabetes mellitus
- iron deficiency anemia
Twin pregnancy increases the risk for what fetal complications
- congenital anomalies
- fetal growth restriction
- preterm delivery
- malpresentation
- monochorionic twins- twin twin transfusion syndrome
- monoamniotic twins- conjoined twins or cord entanglement
What are the causes of decreased central respiratory drive in hypercapnia
Drugs (opioids, benzos)
CNS trauma, stroke, encephalitis
What are the causes of decreased respiratory neuromuscular function in hypercapnia
Spinal cord lesions
ALS
myasthenia gravis
What are the causes of decreased thoracic cage or pleural function in hypercapnia
Obesity hypoventilation syndrome
Pneumothorax
Rib fractures, flail chest
What are the causes of airway obstruction in hypercapnia
OSA (upper airway)
COPD (lower airway)
What are the causes of impaired gas exchange in hypercapnia
Cardiogenic pulmonary edema
Interstitial lung disease
Immune etiology of hydrops fetalis
RhD alloimmunization
Nonimmune causes of hydrops fetalis
Parvovirus B19 infection
Fetal aneuploidy
Cardiovascular abnormalities
Thalassemia (hemoglobin Barts)
Clinical features of hydrops fetalis
pericardial effusion
pleural effusion
ascites
skin edema
placental edema
polyhydramnios
How is congenital CMV transmitted
bodily fluids (urine, saliva)
- main risk factor- caring for young children
Clinical features of congenital CMV
growth restriction and microcephaly
periventricular calcifications
hepatosplenomegaly
thrombocytopenia
diagnosis and treatment for congenital CMV
dx- PCR testing, viral culture of urine/saliva
Treatment- antiviral therapy (valganciclovir) if symptomatic
Causes of malignant pericardial effusion
Common primary tumors: lung, breast, GI, lymphoma, melanoma
Clinical features of malignant pericardial effusion
Progressive dyspnea, chest fullness, fatigue
ECG: low QRS voltage and or electrical alternans
CXR: enlarged cardiac silhouette and clear lung fields
ECHO: large effusion and signs of tamponade
Treatment for malignant pericardial effusion
Acute management: pericardiocentesis, cytologic fluid analysis
Prevention of recurrence: prolonged drainage (catheter, pericardial window)
Epidemiology of progressive multifocal leukoencephalopathy
JC virus reactivation
Severe immunosuppression (untreated AIDS)
How does progressive multifocal leukoencephalopathy present
confusion, paresis, ataxia, seizure
DX and TX of progressive multifocal leukoencephalopathy
Dx- MRI/CT of brain with contrast- shows asymmetric white mater lesions, no enhancement/edema
Lumbar puncture= CSF PCR positive for JC virus
Brain biopsy (rarely needed)
Treatment- often fatal
if HIV + antiretroviral therapy
What antihypertensive are associated with reduced insulin sensitivity and increased risk of developing type 2 DM
Beta blockers (metaprolol atenolol- beta 1 specific or propnaolol-beta 1 and beta 2)
Beta blockers with combined vasodilatory alpha 1 receptor blocking properties (carvedilol, labetalol) not associated with reduced insulin sensitivity
Cushing syndrome signs
Cushing syndrome- hypercortisolism
- weight gain
- proximal muscle weakness
- hypertension
- easy bruisability
- dermal atrophy
- wide purple striae
- hyperpigmentation (ACTH dependent Cushing syndrome)
- increased incidence of cutaneous fungal infections (tinea versicolor, onychomycosis)
- hyperandrogenism (menstrual irregularities, acne, hirsutism)
Closed spinal dysraphism
Failure of posterior vertebral arc fusion
Spinal cord anomalies (lipoma, cyst)
Stretch- induced distal spinal cord dysfunction (tethered cord)
Symptoms- cutaneous or lumbosacral anomalies (hair tuft, mass)
Tethered cord syndrome:
- neurologic: LMN signs
- urologic: incontinence/retention, recurrent UTI
- orthopedic: back pain, scoliosis, foot deformities
Management- MRI of the spine
surgical detethering of cord if symptomatic
What causes a focal seizure
Neuronal discharge begins in 1 cerebral hemisphere
- symptoms may be motor, sensory or autonomic
- Motor: twitching, sensory: paresthesias, autonomic: sweating
- underlying structural abnormality (tumor)more likely than with a generalized seizure
What are the two categories of focal seizures
No impairment of awareness
- occurs when seizure remains localized to 1 hemisphere
Impairment of awareness
- occurs when seizure spreads to the other hemisphere
- often associated with automatisms (eg: chewing, picking)
Diagnosis: EEG, Brain MRI
What is Todds paralysis
Postictal paresis or paralysis
Adolescents with myoclonic jerks immediately on wakening
Juvenile myoclonic epilepsy
By age 5, intellectual disability, severe siezures of varying types (atypical absence, tonic)
Lennox Gastaut syndrome
- Interictal EEG demonstrates a slow spike and wave pattern
What pathogens are airborne and what are isolation precautions for airborne pathogens
Bacterial: tuberculosis
Viral: primary VZV (chickenpox), disseminated VZV reactivation (shingleszoster) in immunocompromised patients, COVID19, measles
Requirements: negative pressure room, N95 respirator
What pathogens are contact spread and what are isolation precautions for contact pathogens
MRSA, VRE, Extended spectrum beta lactamase producing (ESBL producing
Bacterial: C.diff, E.coli 0157:H7
Viral: RSV, primary VZV (chickenpox), disseminated VZV reactivation (shingles/zoster), dermatomal ZVZ reactivation (shingles/zoster)
Requirements: gowns and gloves, single-use equipment (stethoscope)
What pathogens are spread by droplets and what precautions are needed
Bacterial: Neisseria meningitides, Hflu Type B, Mycoplasma pneumoniae
Viral: influenza virus, adenovirus
Requirements: mask within 3-6ft of patient
What are the clinical features of paroxysmal nocturnal hemoglobinuria
Hemolysis -> fatigue
Cytopenias -> impaired hematopoiesis
Venous thrombosis -> intraabdominal, cerebral veins
Workup for paroxysmal nocturnal hemoglobinuria
CBC (hypoplastic/aplastic anemia, thrombocytopenia, leukopenia)
elevated LDH and low haptoglobin (signs of hemolysis)
indirect hyperbilirubinemia
urinalysis (hemoglobinuria)
flow cytometry (absence of CD55 and CD59)
Treatment for paroxysmal nocturnal hemoglobinuria
Iron and folate supplementation
Eculizumab (monoclonal antibody that inhibits complement activation)
Causes of chronic pancreatitis
Alcohol use
Cystic fibrosis (common in children)
Ductal obstruction (malignancy, stones)
Autoimmune
Clinical presentation of chronic pancreatitis
Chronic epigastric pain with intermittent pain-free intervals
Malabsorption: steatorrhea, weight loss
Diabetes mellitus
Laboratory results/imaging of chronic pancreatitis
Amylase/lipase can be normal and nondiagnostic
CT scan or MRCP can show calcifications, dilated ducts and enlarged pancreas
Treatment of chronic pancreatitis
Pain management
Alcohol and smoking cessation
Frequent small meals
Pancreatic enzyme supplements
What does fecal elastase check for
In patients with chronic pancreatitis= destruction of pancreatic islet and acinar cells leads to endocrine and exocrine insufficiency resulting in protein and fat malabsorption, steatorrhea, weight loss, fat soluble vitamin deficiencies
Fecal elastase- noninvasive test for severe pancreatic exocrine insufficiency
- alternate noninvasive test s is serum trypsinogen
What markers are seen in inflammatory bowel disease
Increase in both fecal calprotectin and fecal leukocytes
What markers are seen in celiac disease
Celiac disease presents as steatorrhea and weight loss which can result from malabsorption
- typically celiac disease patients have IDA and other autoimmune comorbidities
Tissue transglutaminase antibodies are elevated in celiac disease
Hard signs of traumatic arterial injury
Require immediate surgery
Distal limb ischemia (paralysis, pain, pallor, poikilothermy)
Absent distal pulse
Active hemorrhage or rapidly expanding hematoma
Bruit or thrill at site of injury
Soft signs of traumatic arterial injury
Require further imaging
Diminished distal pulses
Unexplained hypotension
Stable hematoma
Documented hemorrhage at time of injury
Associated neurologic deficit
What is the diagnostic modality of choice for traumatic arterial injury
CT angiography - high sensitivity and specificity and rapid procedure time
Uncomplicated fractures of the middle third of the clavicle- how are they treated
Nonoperatively with rest, ice and either a sling or a figure of eight bandage
Fractures of the distal third of the clavicle are treated how?
open reduction and internal fixation to prevent nonunion
Diamon Blackfan anemia
- congenital erythroid aplasia
- craniofacial abnormalities
- TRIGPHALANGEAL THUMBS
- increased risk of malignancy
- macrocytic anemia
- reticulocytopenia
- normal platelets, white blood cells
TREATMENT- corticosteroids, RBC transfusions
How does Diamond Blackfan anemia present in infancy
Progressive pallor and poor feeding due to anemia
Heart rate increases to meet oxygen demands and a faint systolic ejection murmur due to increased turbulence across the valves
Fanconi anemia signs
Pathogenic variants in genes involved in DNA repair
- pancytopenia- FA leads to low levels of RBCs, WBCs and platelets (WHILE DIAMOND BLACKFAN IS SELECTIVE DECREASE IN RBC FORMATION)
Asymptomatic Hypercalcemia
- normal renal function
- high-normal PTH level
- low urinary calcium excretion
FAMILIAL HYPOCALCIURIC HYPERCALCEMIA
- benign autosomal dominant disorder caused by a mutation of the calcium sensing receptor
- normally high calcium levels suppress PTH secretion by parathyroid glands
- in FHH higher calcium concentrations are required to suppress PTH release
- defective CaSR leads to increased reabsorption of calcium in renal tubules
Primary hyperparathyroidism vs Familial hypocalciuric hypercalcemia
Primary hyperPTH- increased urinary calcium excretion due to excessive mobilization of calcium from bones
UCCR >0.02
FHH- low urinary calcium levels (<100mg24hr)
UCCR <0.01
Side effects of calcineurin inhibitors (tacrolimus, cyclosporine)
- inhibit the transcription of Il2 to reduce T lymphocyte activity and are important component of chronic immunosuppression following solid organ transplantation
Side effects:
- nephrotoxicity: can lead to reversible AKI or slow progressive chronic kidney injury that is irreversible
- hyperkalemia, hyperuricemia with increased rates of gout
- HTN
- neurotoxicity- tremor, visual disturbance, seizures
- glucose intolerance
- gingival hypertrophy, hirsutism, alopecia
- GI disturbance
Vaginal cancer- risk factors, features, diagnosis and treatment
Risk factors- age >60, HPV infection, tobacco use, in utero DES exposure (clear cell adenocarcinoma only)
Clinical features- vaginal bleeding, malodorous vaginal discharge, irregular vaginal lesion
Diagnosis- vaginal biopsy
Management- surgery +/- chemoradiation
Neurofibromatosis Type 1- chromosome 17
Cafe au lait macules
Scoliosis
Axillary and inguinal freckling
Neurofibromas
Pseudoarthrosis
Optic glioma
Lisch nodules
Increased risk of neurologic disorders (cognitive deficits, learning disabilities, seizures and intracranial neoplasms)
AUTOSOMAL DOMINANT NEUROCUTNEOUS DISORDER
Neurofibromatosis Type 2- chromosome 22
Acoustic neuromas (vestibular schwannomas)
Do not have cafe au lait macules or axillary or inguinal freckling
Tuberous Sclerosis
Ash lead spots
Angiofibromas
Shagreen patches
Seizures (infantile spasms)
Developmental delay
Behavior problems- hyperactivity, self injury
White patches on the retina
Intracardiac rhabdomyomas
Fragile X syndrome
Testicular enlargement in the setting of seizures
X-linked disorder
Intellectual disability
Large ears
Long and narrow face
Macroorchidism
Sturge Weber Syndrome
Port wine stain on the face
Ocular disease (visual deficits or glaucoma)
leptomeningeal capillary-venous malformations- increased risk for seizures
Unilateral vs bilateral vestibular schwannoma
Unilateral- vestibular schwannoma
Bilateral- NF Type 2
Cholesteatoma
Erosive, expansile mass of keratin debris in the middle ear
Presents with unilateral conductive hearing loss (bone conduction > air conduction, lateralization to the affected ear)
Otorrhea
Pearly white mass in the middle ear
Legg Calve Perthes Disease
- avascular necrosis (fragmented, collapsed epiphysis)
- commonly affects boys age 5-7 and presents with insidious onset of hip pain and limp
- X ray may be normal in early disease or show fragmentation of the femoral head
SCFE
- anteriolateral and superior displacement of the proximal femur along the physis
- during periods of accelerated growth (growth spurts in tall thin children) or obesity
- classic presentation- insidious onset of dull hip pain and limp
- minor trauma can exacerbate symptoms
- patients hold affected hip in passive external rotation and exhibit decreased internal rotation, abduction and flexion
- hip radiographs- diagnostic
- treatment- immediate stabilization of the physis with surgical fixation
TTP
- low ADAMTS13 level- leads to platelet trapping and activation
- hemolytic anemia (high LDH, low haptoglobin) with schistocytes
- thrombocytopenia (high bleeding time, normal PT/PTT)
- sometimes with renal failure, neuro manifestations, fever
Tx- plasma exchange, glucocorticoids, rituximab, caplacizumab
Incisional Hernia
- breakdown of prior fascial closure
- commonly seen in obesity, tobacco smokers, poor wound healing, vertical or midline incision, surgical site infection
features: abdominal mass that enlarges with valsalva
- palpable fascial edges in nonobese patients
- possible delayed presentation
dx- clinical, CT abdomen
Androgen insensitivity Syndrome
X linked mutation in androgen receptor
Genotypically male (46 XY)
Phenotypically female
Breast development
Absent or minimal axillary and pubic hair
Female external genitalia
Absent uterus, cervix, and upper one third of vagina
Cryptorchid testes
Management- gender identity/assignment counseling and gonadectomy (malignancy prevention)
Primary Adrenal insufficiency
destruction of bilateral adrenal cortex
- caused by autoimmune adrenalitis or infection or malignancy
Low cortisol
low aldosterone
high ACTH
Hypovolemia
Hyperkalemia
Hyponatremia
Hyperpigmentation
Secondary adrenal insufficiency
Disruption of hypothalamic pituitary axis
Due to chronic glucocorticoid therapy, infiltrative disease or ischemia of anterior pituitary gland
Low cortisol
NORMAL aldosterone
low ACTH
euvolemia
minimal electrolyte disturbance
no hyperpigmentation
less severe symptoms
Migratory superficial thrombophlebitis (aka Trousseau syndrome)
Chronic disseminated intravascular coagulopathy
Recurrent superficial venous thrombosis at unusual sites (arm, chest)
STRONGLY associated with occult visceral adenocarcinoma (pancreas, lung)
complications include venous thrombosis, verrucous endocarditis, ischemic stroke, arterial emboli
DX- CT of the abdomen if epigastric pain despite antacid therapy
TX- heparin therapy to prevent future clots
Metatarsus adductus
Medial deviation of the forefoot
- neutral position of hindfoot
- flexible positioning typical
Tx- reassurance
Clubfoot
- medial/upward deviation of forefoot and hindfoot
- hyperplantar flexion of the foot
- rigid position typical (congenital clubfoot)
Tx- serial manipulation and casting; surgery for refractory cases
Acute postoperative mediastinitis
Direct microbial contamination of operative sites which complicates 1-2% of cardiothoracic operations involving sternotomy
presents 1-2 weeks after operation as sepsis (fever, tachy, leukocytosis), signs of sternal wound infection (drainage and tenderness) and chest x ray showing localized mediastinal fluid or air
dx- characteristic clinical presentation and surgical exploration revealing pus in mediastinum
tx- IV abx and surgical debridement
Miliary tuberculosis
fever, cough, lethargy and respiratory failure with numerous micronodular lesions in the liver, lungs, and spleen
caused by lymphohematogenous spread of Mycobacterium tuberculosis
- rare but serious complication of primary TB in hosts with poor T cell function (infants and immunocompromised)
Risk factors for cerebral palsy
Prematurity
low birth weight
intrauterine infection
perinatal complications (placental abruption, hypoxic-ischemic injury, stroke)
Clinical features of cerebral palsy
Spastic
- affects 1 or more limbs
- hypertonia
- motor delay
- commando crawling- diplegia
- early hand preference
- contractures
Dyskinetic (choreoathetotic, dystonic): involuntary movements, dysarthria
Ataxia: hypotonic, incoordination, jerky speech
Acute left renal vein thrombosis
Membranous neprhopathy is a common cause of nephrotic syndrome
- occlusion of L renal vein increases glomerular pressure and causes hematuria and L sided flank pain
- L gonadal vein drains into the L renal vein, obstruction also causes L gonadal vein dilation
- Risk factors for RVT- nephrotic syndrome, renal malignancy and trauma
Brown recluse spider bite
Common when people are putting their clothes on
- small ulcer developing at site of a recent bite
- many cases result in a small papule that heals in days to weeks
- in some cases, a deep skin ulcer develops at the bite site over the course of a few days with an erythematous halo and a necrotic center than can progress to an eschar
Black widow spider bites
Lead to more pronounced local and systemic manifestations due to the effects of the toxin
- muscle pain, abdominal rigidity, and muscle cramps
- ulceration is uncommon
- patients commonly develop nausea and vomiting within hours of the bite
Snake bites
hemoglobinuria, bleeding, muscle paralysis
severe local pain, swelling, and discoloration within hours of the bite
Treatment of preeclampsia with severe features
Severe features- hypertension, headache, visual changes
- in patients without MG, treat with magnesium sulfate sor seizure prophylaxis
- in patients with MG, magnesium sulfate is CONTRAINDICATED because it may trigger a myasthenic crisis (oropharyngeal muscle weakness, respiratory failure requiring intubation) due to inhibition of acetylcholine release at the NMJ
- IN THESE PATIENTS SEIZURE PROPHYLAXIS IS WITH VALPROIC ACID
