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Pediatrics Flashcards

1
Q

What is lymphadenitis, and the most common cause?

A

An enlarged, tender and erythematous LN.
Acute unilateral – bacterial infection, norm S. aureus and GAS #2. Often febrile in kids <5y, with node >5cm
MAC is a common cause of unilateral subacute/chronic lymphadenopathy (normally nontender, afebrile, and has thin overlying violaceous skin). Kids <5y w/node <5cm

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2
Q

Presentation of Langerhans cell histiocytosis:

A

Lytic bone lesions (skull, jaw, femur) seen in diaphysis of long bones
Skin lesions (purplish papules, eczematous rash)
Lymphadenopathy, hepatosplenomegaly
Pulmonary cysts/nodules
Central DI

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3
Q

First step in evaluating primary amenorrhea:

A

Pelvic US

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4
Q

Gold standard for dx of Muscular Dystrophy:

A

Genetic testing

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5
Q

What are contraindications to admin of the DTaP?

A

Anaphylaxis, unstable neurologic disorders, and encephalopathy (coma, decreased consciousness, prolonged seizures).
Uncomplicated seizures aren’t a contraindication

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6
Q

What radiologic signs are characteristic of pyloric stenosis?

A

String sign on barium swallow/upper GI series
Shoulder sign – filling defect in antrum d/t inward prolapse of mm.
Mushroom sign – hypertrophic pylorus against duodenum
Railroad track sign – excess mucosa in pyloric lumen, gives 2 columns of barium

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7
Q

Metabolic disturbance seen in pyloric stenosis:

A

Hypochloremic, hypokalemic, metabolic alkalosis – d/t vomiting

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8
Q

CHARGE Syndrome:

A 
Coloboma -- defects in the lens, iris or retina
Heart Defects
Atresia choanae
Retardation of growth/development
Genito-urinary anomalies
Ear abnormalitites/deafness
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9
Q

Tx of choice for foreign body ingestion:

A

Flexible endoscopy – can visualize and retrieve object.

If the object is non-toxic and smooth in a child that is asx, 24hr observation may be done first.

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10
Q

Features and risk-factors for Milk/Soy-protein-induced colitis:

A

Vomiting/Regurg by 2-8 weeks
Often have painless bloody stools and eczema as well.
Risk Factors: Fhx of allergies, eczema or asthma
Spontaneously resolves by 1 year

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11
Q

What is a diffusely narrow colon a/w failure to pass meconium likely a result of?

A

Cystic Fibrosis

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12
Q

When do you see microvesicular fatty changes in the liver?

A

In Reye syndrome

Macrovesicular fatty changes are seen in EtOH liver disease and NASH.

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13
Q

What is tethered cord syndrome and what is it a/w?

A

It is a syndrome of weakness, decreased sensation, urinary incontinence, and hyporeflexia.
The cord is affected below T12/L1 and therefore UMN sxs are not seen
Commonly a/w Spina bifida.

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14
Q

What are common sxs seen after traumatic injury to the carotid aa.?

A

May get hemiparesis, facial droop, and aphasia d/t dissection or thrombus formation which may occur over hours or days.
Neck pain, thunderclap HA, and sxs of ischemic stroke are also common.
Often d/t penetrating trauma, seemingly minor oropharyngeal trauma, or neck strain/manipulation.

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15
Q

Most common source of infection for Staphylococcal Scalded Skin Syndrome (SSSS) in neonates:

A

The umbilicus or circumcision site.

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16
Q

Most common source of infection for Staphylococcal Scalded Skin Syndrome (SSSS) in older children:

A

Nasopharyngeal colonization or a primary skin lesion (pustule).

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17
Q

Common abnormal PE findings a/w strabismus (6):

A 
Constant strabismus at any age
Eye deviation after 4mo of age
Asymmetric corneal light reflexes
Asymmetric intensity of red reflexes
Deviation on cover test
Torticolis or head tilt
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18
Q

Gold standard for diagnosing VUR:

A 
Voiding cystourethrogram (VCUG).
Should only be done in cases of recurrent UTIs, not after the first UTI as this can lead to false negative.
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19
Q

Clinical features of Iron poisoning:

A

W/in 30min-4d: AbdP, Vomiting (hematemesis), diarrhea (melena), HoTive shock, anion gap Metabolic acidosis.
w/in 2d: Hepatic necrosis
W/in 2-8 weeks: Pyloric stenosis
**See Radiopaque pills on XR

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20
Q

Features of Cephalohematoma:

A

Subperiosteal hemorrhage occurring d/t birth trauma.
Appears as a scalp swelling that is firm, nontender, does not cross suture lines, and no skin discoloration.
Often not seen until several hrs after birth.

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21
Q

Features of Caput succedaneum:

A

Scalp swelling superficial to the periosteum that crosses the suture lines.
Edema is usually in the portion of the head that presents during vertex delivery and is present at birth.

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22
Q

What is the post exposure pphx for pertussis?

A

Macrolides for all close contacts
<1mo: Azithromycin x 5d
>1mo: Azithromycin x 5d, Clarithromycin x 7d, or
Erythromycin x 14d

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23
Q

Mgmt for Androgen Insensitivity Syndrome:

A

Elective gonadectomy (for malignancy prevention) and gender identity/assignment counseling.

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24
Q

What is hyposthenuria and what are its sxs?

A

Inability of the kidneys to concentrate urine.
The hypoxic, hyperosmolar conditions in the renal medulla cause RBCs to sickle in the vasa recta and impairs free H2O reabsorption and countercurrent exchange.
Sxs: polyuria and nocturia despite fluid restriction.
Urine osmolality is low with normal serum Na+.
Often seen in SCD and SCT

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25
Q

Complications of Sickle Cell Trait:

A 
Hematuria/papillary necrosis
Hyposthenuria
Splenic infarction (at high Alts), Venous thromboembolisms
Priapism
Exertional rhabdomyolysis
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26
Q

What is erythema toxicum neonatorum?

A

An asymptomatic/benign rash. Often scattered, erythematous papules and pustules.
Does not need diagnostic workup or treatment.

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27
Q

Contraindications to Rotavirus vaccine:

A

Anaphylaxis
Hx of Intussusception or uncorrected congenital Malformation of the GIT (meckel’s etc)
SCID

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28
Q

CoD in different types of muscular dystrophy:

A

Duchenne – respiratory failure by 20-30yr
Becker – heart failure by 40-50 yr
Myotonic dystrophy – either one depending on age of onset

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29
Q

Fanconi syndrome:

A

Type 2 RTA, Glucosuria, aminoaciduria and phosphaturia

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30
Q

Features of Type 1 RTA:

A

Poor H+ secretion into the urine – alkalotic urine (pH>5.5)
Will have low-normal serum K+
Often a genetic disorder a/w nephrolithiasis.
Can be 2/2 Rx toxicity, or AI disorders (Sjogren, RA)

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31
Q

The primary defect in different types of RTA:

A

Type 1 – poor H+ secretion into urine
Type 2 – poor HCO3 resorption
Type 4 – aldosterone resistance
Type 1 will have alkalotic urine, types 2 and 4 will be acidotic

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32
Q

EMG patterns in different types of muscular dystrophy:

A

Both DMD and BMD display a myopathic pattern on EMG.

Myotonic dystrophy will display a myotonic pattern.

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33
Q

What is the primary cause of abnormal uterine bleeding in adolescents?

A

Immaturity of the Hypothalamic-pituitary-ovarian axis

Tx w/ progestin only or combined OCs to help regulate menses.

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34
Q

When should an infant be responding to its own name?

A

By 12 months. If it doesn’t happen by then, it may be an early sign of autism.

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35
Q

XR findings of Ewing Sarcoma (4):

A

Central lytic lesion
“Onion skinning” (lamellated periosteal rxn)
“Moth-eaten” appearance
Periosteal elevation (Codman triangle)

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36
Q

Typical EM findings of Alport’s syndrome:

A

Alternating areas of thinned and thickened capillary loops w/splitting of the GBM.

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37
Q

Pathophys of Guillain-Barre:

A

Immune-mediated demyelination of peripheral nerve fibers

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38
Q

Tx of slipped capital femoral epiphysis:

A

Prompt surgical pinning of the slipped epiphysis where it lies – will lessen the risk of avascular necrosis and chondrolysis.

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39
Q

First step in evaluating precocious puberty:

A

Determine bone age

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40
Q

How to differentiate causes of precocious puberty w/Normal bone age:

A

Isolated breast development – premature thelarche

Isolated pubic hair development – premature adrenarche

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41
Q

How to differentiate causes of precocious puberty w/advanced bone age:

A

Low basal LH and low LH after GnRH stimulation test – peripheral precocious puberty (McCune)
High or low basal LH plus high LH after GnRH stimulation – central precocious puberty

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42
Q

Features and causes of central precocious puberty:

A

Advanced bone age, elevated FSH & LH, and true precocious development (breast development etc)
Hypothalamic glioma
Pituitary hamartoma
Idiopathic precocious puberty

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43
Q

What is premature adrenarche?

A

Caused by early activation of adrenal androgens, more common in obese children.
Presents w/precocious development of pubic and axillary hair, acne, and body odor w/normal bone age.

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44
Q

What is pathognomonic for abusive head trauma in a child?

A

Retinal hemorrhages.

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45
Q

How to differentiate preseptal v. orbital cellulitis:

A

Both will have eyelid erythema and swelling, and chemosis.
Only orbital cellulitis will have pain w/EOM, proptosis +/- ophthalmoplegia w/diplopia.
Preseptal only need oral abx, orbital need IV abx +/- surgery.

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46
Q

How to differentiate Niemann-Pick from Tay-Sach:

A

Both have loss of motor milestones, hypotonia, feeding difficulties, and cherry-red macula.
Tay-sach: Hyperreflexia, no organomegaly
Niemann-pick: Areflexia, and hepatosplenomegaly

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47
Q

What tx might reduce risk of morbidity and mortality in Measles pts?

A

Vitamin A – given to all hospitalized Measles patients.

It helps promote formation of Ab-producing cells and regeneration of epithelial cells

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48
Q

Tx of acute airway obstruction 2/2 mono:

A

IV corticosteroids

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49
Q

Potential ENT cxs of Mono:

A

Peritonsilar abscess, and acute airway obstruction

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50
Q

Findings of congenital syphilis:

A

Rhinorrhea, abnormal long-bone radiographs, enlarged placenta w/edematous umbilical cord and desquamating or bullous rash (can be on palms and soles), plus the features present in all congenital infections.

Most pts asx at birth and start displaying sxs 1-3mos

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51
Q

Clinical findings present in all the congenital (TORCH) infections:

A

Intrauterine growth restriction
Hepatosplenomegaly
Jaundice
Blueberry muffin spots

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52
Q

Features unique to congenital CMV infection:

A

Periventricular calcifications chorioretinitis, and microcephaly in severe cases

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53
Q

Features unique to congenital toxo infection:

A

Diffuse intracerebral calcifications
Severe chorioretinitis
Hydrocephalus

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54
Q

Features unique to congenital rubella infection:

A

Sensorineural hearing loss, cataracts and heart defects (PDA)

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55
Q

What causes the blueberry muffin spots in congenital infections?

A

Extramedullary hematopoiesis

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56
Q

What causes the IUGR in congenital infections?

A

Fetal inflammation

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57
Q

Features, sxs and tx of osteoid osteoma:

A

Benign bone-forming tm. most common at proximal femur.
Have pain worse at night, relieved by NSAIDs and unrelated to activity.
XR: small, round lucency
Tx: NSAIDs and monitor for spontaneous resolution

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58
Q

Pathophys of Intussusception 2/2 viral illness:

A

Hypertrophy of Peyer’s patches in the ileum serves as a nidus for telescoping.

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59
Q

Difference in tx for AOM and otitis externa:

A

AOM requires oral abx (amoxicillin)

Otitis externa is tx’d w/ototopical abx

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60
Q

Most common causes of myocarditis in children:

A

Coxsackie B and Adenovirus

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61
Q

When should chelation therapy be used to tx lead poisoning?

A

When venous lead levels are >45 ug/dL
Dimercaptosuccinic acid (succimer) is used when levels are 45-69
Dimercaprol (British anti-Lewisite) + EDTA is used in emergency when levels are >70 or there are signs of acute encephalopathy.

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62
Q

How is bone age evaluation done?

A

Radiographic assessment of the hand and wrist to assess skeletal maturation.

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63
Q

When is the rotavirus vaccine given?

A

2-8 months only. If it is missed do not give past 8mo.

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64
Q

When is visual acuity testing performed?

A

Routinely at age 4. Can be performed as early as age 3 if the child is cooperative.

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65
Q

How to differentiate b/w Fe-deficiency anemia and thalassemia:

A

Fe-deficiency will have increased RDW, and low RBC count
Thalassemia will have Normal RDW, normal-high RBC count and likely high reticulocytes
Both will have microcytic cells. Fe-deficient will be hypochromic and Thals will have target cells.
Thals may also have elevated serum Fe and ferritin d/t increased RBC turnover.

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66
Q

When is varicella vaccine given?

A

2 doses – one at age 1 and 4 years

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67
Q

Postexposure pphx of varicella:

A

If completely immunized – nothing
Incompletely immunized and immunocompetent – VZV vaccine
Incompletely immunized and IMCPd – VZIG

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68
Q

What is laryngomalacia, its sxs, dx and mgmt?

A

It is increased laxity of supraglottic structures which causes inspiratory stridor worse when supine.
Peaks at 4-8mos.
Dx: normally clinical, confirmed w/flexible laryngoscopy for mod-severe cases
Mgmt: Reassurance (w/ spontaneous resolution by 18mo), or supraglottoplasty for severe sxs.

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69
Q

Cxs of SGA infants:

A 
Hypoxia
Perinatal asphyxia
Meconium aspiration
Hypothermia
Hypoglycemia
Hypocalcemia
Polycythemia
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70
Q

Most common cause of congenital hypothyroidism:

A

Thyroid dysgenesis

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71
Q

Features of congenital varicella syndrome:

A

Limb hypoplasia, cataracts, and distinctive skin lesions (scarring). NO heart defects.

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72
Q

Most common risk factor for orbital cellulitis:

A

Sinusitis

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73
Q

Cause and findings of Beckwith-Wiedemann syndrome:

A

Dysregulation of imprinted gene in chromosome 11p15. Overproduction of IGF-2 – fetal hypoglycemia & hyperinsulinemia
Presents w:
Fetal macrosomia & rapid growth until late childhood
Omphalocele/umbilical hernia
Macroglossia
Hemihyperplasia (limbs larger on one side)

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74
Q

Cxs of Beckwith-Wiedemann and surveillance for them:

A

Wilms tm – abdominal US every 3 mo from birth to 8yr, then renal US from 8yr through adolescence.
Hepatoblastoma – Serum AFP every 3mo from birth to 4yr

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75
Q

Most common organisms and tx of septic arthritis in kids <3mo:

A

Staph, GBS, and GNR
Tx: Joint aspiration + abx
Abx – antistaph (nafcillin or vanc) + gentamicin or cefotaxime
(Blood and synovial fluid cultures should both be obtained before starting abx)

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76
Q

Most common organisms and tx of septic arthritis in kids >3mo:

A

Staph, GAS, and S. pneumo
Abx – Nafcillin, clindamycin, cefazolin, or vancomycin
**Blood and synovial fluid cultures should both be obtained before starting abx

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77
Q

Most common cause of osteomyelitis:

A

S. aureus

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78
Q

Perianal streptococcus features and tx:

A

Caused by S. pyogenes (GAS), common in school-age children.
Get bright, sharply demarcated erythema over perianal/perineal area. May have pruritis, pain w/stooling and fissures causing blood-streaked stool.
Tx w/oral abx (beta lactams – penicillin, amoxicillin)

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79
Q

Perianal dermatoses in infants:

A

Contact dermatitis #1 – spares creases/skin folds, tx w/barrier ointment or paste
Candida dermatitis #2 – beefy-red rash involving skinfolds w/satellite lesions. Tx w/topical antifungals

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80
Q

Most common predisposing factor of acute bacterial sinusitis:

A

Viral URI

1st line tx of bacterial sinusitis: amoxicillin + clavulonate

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81
Q

What is the pathogenesis of Minimal Change Disease?

A

T-cell mediated injury to podocytes causing increased molecular permeability to albumin.

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82
Q

Primary side effect of Hydroxyurea:

A

Myelosuppression

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83
Q

How to differentiate Marfan syndrome from Homocystinuria:

A

Both have pectus deformity, tall stature (increased arm:height and decreased upper:lower body), arachnodactyly, joint hyperlaxity, skin hyperelasticity and scoliosis
Marfan: AD, normal IQ, aortic root dilation, Upward lens dislocation
Homocystinuria: AR, decreased IQ, thrombosis, downward lens dislocation, megaloblastic anemia, fair complexion (blonde, blue eyes)

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84
Q

How to differentiate Ehlers-Danlos from Marfan/Homocystinuria:

A

All have joint laxity, skin hyperelasticity, but ED does NOT have tall stature, lens probs, or hypercoagulability.
ED does have scoliosis

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85
Q

What type of fluid is preferred for resuscitation in burn victims?

A

Lactated Ringers – it is considered a balanced solution and will help correct acidosis and maintain normal blood pH.
NS is an unbalanced solution and can cause hyperchloremic metabolic acidosis and hypocoagulability.

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86
Q

Do children w/febrile seizures need to be admitted for observation?

A

No. Only children with a post-ictal state who haven’t returned to baseline rapidly need admittance.

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87
Q

How to diagnose developmental dysplasia of the hip:

A

Age <4mos – hip US

Age >4mos – hip XR

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88
Q

What does squatting do to help reduce cyanosis in ToF?

A

It increases systemic vascular resistance/afterload and decreases the R-to-L shunting across the VSD, which then increases flow across the RVOT.

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89
Q

How does the timing of pain differentiate b/w the differentials for non-traumatic joint swelling?

A

Infectious is an acute onset with constant pain.
Inflamm/Rheum is subacute/chronic with pain worse in the morning.
Neoplastic is subacute/chronic and has pain worse in the evening/night

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90
Q

Laboratory findings of the differentials for non-traumatic joint swelling:

A

Infectious – increased WBCs, platelets, and inflammatory markers
Inflamm/Rheum – Increased WBCs, platelets, and inflammatory markers. Decreased RBCs
Neoplastic – decreased WBCs and platelets

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91
Q

What is the common presentation of systemic-onset juvenile arthritis?

A

Chronic oligoarthritis (worse in the morning)
Daily fever (quotidian fever >2wks)
Rash (pink, macular, worsens during fever)
Possible lymphadenopathy and hepatosplenomegaly

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92
Q

Characteristic lab findings in sJIA:

A

Leukocytosis, thrombocytosis, and markedly increased inflammatory markers.
May also have anemia d/t chronic disease or Fe-deficiency

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93
Q

What should be investigated in someone w/subQ emphysema in anterior chest?

A

Pneumothorax – an emergent CXR should be obtained to rule it out.
SubQ emphysema often occurs 2/2 severe coughing paroxysms which can also lead to pneumothorax.

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94
Q

What is the tx of Croup/laryngotracheitis?

A

Mild (no stridor at rest): Humidified air +/- steroids.
Mod/Severe (stridor at rest): Steroids + nebulized racemic Epi
Shouldn’t intubate/ventilate unless they have failed the above txs or have impending resp. failure (poor inspiratory effort, severe hypoxemia, etc.)

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95
Q

What are the initial steps in evaluating Primary amenorrhea?

A

Pelvic US to confirm presence of uterus.
If uterus is absent, do Karyotype.
If uterus is present then check FSH levels.

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96
Q

What are the differentials of Primary amenorrhea w/absent uterus on US?

A

Androgen Insensitivity

Mullerian agenesis

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97
Q

What are the differentials of Primary amenorrhea w/present uterus on US and how are they differentiated?

A

Differentiate based on FSH levels:
Normal FSH – imperforate hymen
High FSH – Primary ovarian insufficiency (46, XX), Turner syndrome (45, XO)
Low FSH – Hypothyroidism, Prolactinoma, Functional HoThalamic amenorrhea (normal TSH, prolactin)

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98
Q

What is the mgmt. of scrotal hydrocele in a neonate?

A

Reassurance and observation up until 1 year. These are common and should resolve on their own by 1 year.

99
Q

Most common cause of mastoiditis and its mgmt:

A

S. pneumo.
Complication of AOM.
Tx w/IV abx and drainage

100
Q

What abx should be used to tx neonatal sepsis?

A

Ampicillin and Gentamicin

101
Q

What is the acidified glycerol lysis test used to diagnose?

A

Aka the osmotic fragility test. Used to dx Hereditary Spherocytosis

102
Q

What are the lab findings of Hereditary Spherocytosis(5)?

A 
Increased MCHC
Spherocytes on peripheral smear
Negative Coombs test
Increased osmotic fragility on acidified glycerol lysis test
Abnormal eosin-5-maleimide binding test
103
Q

How do pineal gland masses often present?

A

With Parinaud/Dorsal midbrain syndrome: limited upward gaze, upper eyelid retraction, pupils reactive to accommodation but not light.
Obstructive hydrocephalus: papilledema, HA, vomiting, and ataxia.

104
Q

What are the common cxs of Bronciolitis?

A

Apnea (esp. infants <2mos)
Respiratory failure.
Risk of sepsis or bacterial pneumonia are extremely rare (<1%)

105
Q

How do “flow” murmurs typically sound?

A

Grade I/II midsystolic ejection murmurs.

106
Q

Most common cause of vaginal discharge in neonatal period?

A

Maternal withdrawal of estrogen. Presents w/bleeding as well. Lasts <1week.

107
Q

Features of PKU:

A

AR mutation in phenylalanine hydroxylase.
Presents with severe intellectual disability, seizures, musty body odor, hypopigmentation of skin, hair, eyes and brain nuclei.
Dx via newborn screen or quantitative amino acid analysis (increased Phe)

108
Q

What can provoke an absence seizure?

A

Hyperventilation

109
Q

What comorbid conditions are often a/w Tourette syndrome?

A

ADHD and OCD

110
Q

Most common cause of Sepsis in SCD?

A

S. pneumo – pts w/SCD should receive pphx penicillin until at least age 5 to avoid this.

111
Q

Most common cause of Acute Bacterial Rhinosinusitis:

A

S. pneumo or non-typeable H. influenzae

112
Q

Cxs of Bacterial meningitis in children:

A

~50% of children have long-term neurologic sequelae
Intellectual/behavioral disabilities
Hearing loss (2/2 inflammatory damage to the cochlea)
Cerebral palsy
Epilepsy

113
Q

What type of virus is coxsackie?

A

An enterovirus

114
Q

How is pertussis diagnosed?

A

Via pertussis culture or PCR. Will also see lymphocyte-predominant leukocytosis on CBC.

115
Q

Most common congenital heart defect in patients w/Down syndrome:

A

Complete atrioventricular septal defect.

116
Q

Most common cause of hearing impairment in children:

A

Conductive hearing loss d/t repeated ear infections.

117
Q

Why are men with CF infertile?

A

Bc inspissated mucus collects and obstructs the developing vas deferens in utero and prevents them from forming.

118
Q

What are the effects of untreated primary HoThyroidism on puberty?

A

Can cause peripheral/gonadotropin-dependent precocious puberty.

119
Q

What are the features and management of Jervell Lange-Nielsen syndrome?

A

AR inheritance of molecular defects in K+ channels.
Features: long QT syndrome, and congenital sensorineural deafness.
Tx: BB (propranolol) + pacemaker if there’s hx of syncope.
Must also focus on maintaining normal levels of Ca2+, K+, and Mg2+

120
Q

When should an infant be referred for audiology evaluation?

A

If they are not responding to their own name, babbling or saying small nonspecific words by 9mos.

121
Q

Difference in presentation of sporadic and inheritable retinoblastoma:

A

Sporadic presents as unilateral disease.

Hereditary is often bilateral

122
Q

When is ceftriaxone used in Lyme disease?

A

IV ceftriaxone is reserved for Lyme meningitis and heart block (manifestations of early dissem’d Lyme)

123
Q

Who can’t receive tetracyclines?

A

Kids <8 years and pregnant women.

124
Q

Tx of Lyme in tetracycline contra’d pts:

A

Oral amoxicillin or cefuroxime (2nd-gen enteral Ceph)

125
Q

What is Mupirocin?

A

Belongs to monoxycarbolic acid class of abx.
Has activity against most G+ cocci including MRSA and most strep (but not enterococci)
ONLY topical/intranasal agent w/MRSA coverage.
Used to tx impetigo and eradication of S. aureus nasal colonization

126
Q

Alternatives to stimulants in ADHD tx:

A

Atomoxetine (selective NE-RI) is 1st line

Others: a-2 adrenergic agonists (clonidine, guanfacine), Bupropion, TCAs

127
Q

What type of exudative effusion will have increased TGs?

A

Chylothorax – often from disruption of the thoracic duct.

128
Q

Transmission of Measles:

A

Airborne

129
Q

Most common posterior fossa tumors in children:

A 
#1 – Cerebellar astrocytoma (occur in lateral cerebellar hemispheres -- tremor, dysdiadokinesia etc.)
#2 – Medulloblastoma (occur in the cerebellar vermis -- truncal ataxia, gait instability)
130
Q

What are the features of hemiplegic migraine?

A

Migraine HAs accompanied by motor weakness during the preceding aura phase.
Common in adolescents and typically do not cause LoC.

131
Q

What is Todd paralysis?

A

Self-limited, focal weakness or paralysis that occurs after a focal or generalized seizure.
Presents in the post-ictal period w/hemiparesis or complete hemiplegia involving one side of the body.

132
Q

What Rxs are used in the tx of Tourette?

A

First line are Antidopaminergic (Tetrabenzine – DoC, Risperidone, and Haloperidol).
A-2 adrenergic agonists (guanfacine, and clonidine) can be used as alternatives.

133
Q

What is epidemic keratoconjunctivitis?

A

A more serious form of conjunctivitis caused by adenovirus.
Characterized by fever, eye pain and inflammation, and preauricular lymphadenopathy that’s followed by development of painful corneal opacities.
Self-limited, but highly contagious and occurs in outbreaks.

134
Q

What disease is a/w 12/21 gene translocation?

A

Most common form of ALL

135
Q

What is the definition of a concussion?

A

Transient alteration in consciousness following head trauma w/out intracranial imaging abnormality.

136
Q

What are some features of Wiskott-Aldrich syndrome?

A 
Eczema 
Increased IgE and IgA, decreased IgM, 
Micro-thrombocytopenia (small and low platelets) -- can get petechiae, purpura, hematuria, mucosal bleeding, intracranial hemorrhage
B and T cell dysfxn.
X-linked.
137
Q

How is lice treated?

A

With permethrin lotion

138
Q

What should all patients w/SLE be prescribed?

A

Hydroxychloroquine.

It is a DMARD for SLE and improves survival and may be beneficial enough to let the patient stop taking steroids.

139
Q

What do all patients with central precocious puberty need?

A

A brain MRI

140
Q

Clinical Manifestations of Rickets (5):

A

Craniotabes (“ping-pong ball” skull)
Delayed fontanel closure
Enlarged: skull (frontal bossing) and long-bone joints (wrist widening)
Costochondral joint hypertrophy (“rachitic rosary”)
Genu Varum (femoral and tibial bowing)

141
Q

Tx of radial head subluxation:

A

Hyperpronation of forearm or Supination of forearm w/flexion of elbow.

142
Q

What may be an early sign of Rett syndrome?

A

Deceleration of head growth

143
Q

What is Landau-Kleffner syndrome?

A

Deterioration/regression of language skills d/t severe epileptic attacks. Typically begins to deteriorate at age 3-6 years.

144
Q

Features of Scarlet Fever:

A

Caused by S. pyogenes w/erythrogenic exotoxins
Fever & Pharyngitis w/ tonsillar exudates, palatal petechiae and strawberry tongue
Tender, shotty anterior cervical lymphadenopathy
Sandpaper rash most pronounced in skin folds – will desquamate and cause peeling of hands and feet.
Rash on cheeks may seem to cause circumoral pallor
Tx w/Penicillin (amoxicillin)

145
Q

What are txs for nocturnal enuresis?

A

Enuresis alarm therapy and Desmopressin are 1st line

Should not be implemented before age 5 as this is normal.

146
Q

Features and tx of tinea capitis:

A

Dermatophyte infection most common in AfAm children.
Transmission: Direct contact or from fomite (sharing combs)
Clinical feats: Scaly, erythematous patch w/hair loss, +/- black dots in area, +/- tender lymphadenopathy (often occipital and post-auricular)
Tx: Oral griseofulvin or terbinafine

147
Q

What are features of Congenital lymphedema and what is it commonly a/w?

A

Presents at birth with NON-pitting edema 2/2 lymphatic network dysgenesis – get protein-rich ISF in the hands, feet and neck.
Severe obstruction can result in cystic hygroma in the neck and fetal hydrops
Often a/w Turner Syndrome

148
Q

What is the tx for Strawberry hemangioma?

A

Most require no tx.
Topical Propranolol is used for disfiguring, ulcerating, disabling (eyelid – strabismus), or life-threatening (those by airway)

149
Q

What causes hyper-IgM syndrome?

A

X-linked defect in CD40-ligand on T-cells. Prevents class switching.

150
Q

Tx of Immune Thrombocytopenia in children:

A 
If only cutaneous sxs present – observation (norm resolves w/in 3mo).
If bleeding (decreased Hb/Hct, epistaxis, hematuria etc) – glucocorticoids, IVIG, or anti-D
151
Q

Tx of Immune thrombocytopenia in adults:

A

If only cutaneous sxs and platelets >30K – observation

If bleeding or platelets <30K – glucocorticoids, IVIG, or anti-D

152
Q

Features of Neuroblastoma:

A

Neural crest origin, involves adrenal medulla and sympathetic chain.
Often presents <2yrs, w/abdominal mass. Can get Horner syndrome if in SNS chain
Other features: periorbital ecchymoses (orbital mets), spinal cord compression from epidural invasion, opsoclonus-myoclonus syndrome (rapid vertical and horizontal nystagmus w/myoclonus), HTN, flushing and diaphoresis
Dx: elevated CAs (VMA, HVA), n-myc amplification, small round blue cells on histo.

153
Q

How should height and weight change in an infant by 12mos?

A

Weight should triple and height should increase by 50% from birth weight and height.

154
Q

Most common causes and risk factors of Otitis Externa:

A

P. aeruginosa and S. aureus
Risk Factors: Water exposure, Trauma (cotton swab), Foreign material (hearing aid, headphones), derm conditions (eczema, contact dermatitis)

155
Q

Tx of Otitis Externa:

A

Remove debris, topical abx (fluoroquinolone), +/- topical glucocorticoid

156
Q

What CNS cxs are NF1 patients most at risk of developing?

A

Optic pathway gliomas
Astrocytomas
Brainstem gliomas
The risk carries into adulthood

157
Q

PE findings in Von Gierke disease:

A

Doll-like face w/rounded cheeks, thin extremities, short stature, and protuberant abdomen d/t hepatomegaly.
Spleen and heart are not involved

158
Q

Laboratory findings in Von Gierke (Type I glycogen storage) disease:

A

Presents around 3-4 mo w/glycogen accumulation, hypoglycemia (causes seizures), lactic acidosis, hyperuricemia, and hyperlipidemia.

159
Q

Enzyme deficient in Von Gierke:

A

Glucose-6-phosphatase

160
Q

Enzyme deficient in Gaucher disease:

A

Glucocerebrosidase

161
Q

What causes Wiskott-Aldrich?

A

XR defect in WAS protein gene – impaired cytoskeleton changes in leukocytes and platelets.
The actin cytoskeleton in WBCs in abnormal, so there’s immune dysfxn d/t impaired cellular migration and immune synapse formation.
Get bacterial, viral and fungal infections.

162
Q

What is Werdnig-Hoffman syndrome?

A

An AR disorder – degeneration of anterior horn cells & cranial nerve motor nuclei causes “floppy baby” syndrome.

163
Q

Risk factors for neonatal respiratory distress syndrome:

A 
#1: Prematurity
Others: Male sex, perinatal asphyxia, maternal DM, c-section.
164
Q

What are some factors that might decrease the risk of neonatal RDS?

A

IUGR, maternal HTN, and prolonged rupture of membranes.

ie: any type of intrauterine stress

165
Q

Features of Leukocyte adhesion deficiency:

A

Delayed umbilical cord separation (>3 weeks), recurrent skin and mucosal bacterial infections (w/out purulence), and severe periodontal disease.
CBC shows marked leukocytosis w/NP predominance.

166
Q

What should be suspected in a pt. w/continued ear drainage for several wks. despite abx tx?

A

Cholesteatoma

167
Q

Common causes of pediatric stroke:

A 
Sickle Cell
Prethrombotic disorders
Congenital cardiac disease
Bacterial meningitis
Vasculitis
Focal cerebral arteriopathy
Head/neck trauma
168
Q

What causes the primary insult in refeeding syndrome and what are the sxs?

A

Rapid increase in insulin

Get arrhythmia, CHF, seizures, and Wernicke encephalopathy.

169
Q

What is the CoD in Friedreich ataxia?

A

Cardiac dysfxn by 30-40 yrs

Arrhythmia, CHF etc.

170
Q

Common presentation of Friedreich ataxia:

A

Progressive gait ataxia, dysarthria, loss of vibration/proprioception, absent DTRs, HOCM, scoliosis and DM.
Presents in adolescence/early adulthood

171
Q

Features of ataxia-telangiectasia:

A

AR disorder of DNA repair – have immunodeficiency, progressive cerebellar ataxia, ocular and cutaneous telangiectasias and increased risk of malignancy.

172
Q

What are the likely HbF levels to be in an SCD patient on Hydroxyurea?

A

> 15%

173
Q

Features of Sturge-Weber syndrome:

A

Congenital NON-inherited anomaly of neural crest derivatives d/t activating mutation of GNAQ
Port wine stain in V1/V2 distribution (nevus flammeus)
Leptomeningeal capillary-venous malformation
Seizures +/- hemiparesis
Intellectual disability
Visual field defects
Glaucoma

174
Q

What is Kippel-Trenaunay syndrome?

A

A syndrome of capillary, venous or lymphatic malformation in combination w/limb overgrowth.
They have port wine stains on the lower extremities
No neuro defects/abnormalities

175
Q

Where are ependymomas most likely located?

A

In children most common site is the 4th ventricle in the posterior fossa.
They arise from ependymal cells lining the ventricles and spinal cord.

176
Q

What are the most common Supratentorial CNS tms in children?

A

Pilocytic astrocytoma > Craniopharyngioma > Glioblastoma.

177
Q

Most common cx of Mumps?

A

Aseptic meningitis – get HA, fever and nuchal rigidity.

In post-pubertal males orchitis may also occur and cause impaired fertility.

178
Q

How does Mumps present?

A

Fever and parotitis – facial swelling with tenderness.

Swelling often obscures the angle of the mandible.

179
Q

Features of Craniopharyngiomas:

A

Calcified, intracranial tumors in the suprasellar region.

Present w/bitemporal hemianopsia and pituitary hormonal deficiencies (DI, GH deficiency)

180
Q

What is the DoC for hemodynamically stable pts w/heavy vaginal bleeding?

A

High-dose combined OCs or IV estrogen.

These stabilize the endometrium and stop acute bleeding.

181
Q

What are Brushfield spots?

A

Whitish-grey spots on the periphery of the iris – often present in Down syndrome pts.

182
Q

What are some features of von Hippel-Lindau syndrome?

A

Inherited disorder – causes both benign and malignant tumors.
Benign hemangioblastomas (high vascularity w/hyperchromatic nuclei) occur in the retina, brain stem, cerebellum and spine.
Angiomatosis – cavernous hemangiomas in skin, mucosa and organs (even CNS – seizures)
Bilateral RCCa
Pheochromocytomas

183
Q

Disinhibited social engagement disorder:

A

Overfamiliarity and an unhesitant approach to unfamiliar adults.
Often seen in children of early neglect.

184
Q

Reactive attachment disorder:

A

Often develops in young children of abuse, neglect, institutionalization or inconsistent care.
The children rarely seek comfort and don’t respond to attempts to comfort them, have lack of social response, lack of positive emotions, and episodes of unexpected irritability or sadness.

185
Q

What would an EKG in tricuspid atresia show?

A

L axis deviation (normal neonates have R axis deviation)
Small/absent R waves in precordial leads (V1-V3)
Tall, peaked P waves (bc increased flow into RA from ASD)

186
Q

Features of tricuspid atresia:

A

Lack of tricuspid valve – requires ASD or VSD for survival.
Lack of blood flow to RV and pulmonary outflow tract – hypoplastic R heart and underdevelopment of pulmonary valve and/or a.
Decreased pulm circulation – decreased pulm markings on CXR.

187
Q

Mgmt of vaginal foreign bodies in chdlren:

A

Warm irrigation and vaginoscopy under sedation/anesthesia

188
Q

What is the most common cause of Pneumonia in CF children v. adults?

A

In children, S. aureus is the most common and Pseudomonas is in adults.
S. aureus starts to decline around age 20 whereas pseudomonas begins to peak.
Both pathogens should be covered in tx of pneumonia in CF children.

189
Q

What renal disease are HBV+ people most at risk of getting?

A

Membranous nephropathy.

Normally uncommon in children, but can occur in those w/HBV

190
Q

What cyanotic heart diseases of the newborn present with a single S2?

A

Transposition – Single S2 +/- VSD murmur
Tricuspid atresia – Single S2 + VSD murmur
Truncus arteriosus – Single S2 + systolic ejection murmur

191
Q

Most common congenital cyanotic heart disease in the neonatal period:

A

Transposition of the great vessels.

192
Q

Tx of Ringworm (Tinea Corporis):

A

1st line/localized disease - -topical clotrimazole or terbinafine
2nd line/extensive disease – oral terbinafine or griseofulvin

193
Q

What are the features of Diamond-Blackfan anemia?

A

Disease of congenital erythroid aplasia.
Clinical: Craniofacial abnormalities (webbed neck, cleft palate, short stature), triphalangeal thumbs, and increased risk of malignancy
a/w polyhydramnios on prenatal US
Lab: Macrocytic anemia, reticulocytopenia, normal platelets and WBCs.
Tx: steroids and RBC transfusions

194
Q

What kind of anemia is Diamond-Blackfan?

A

Pure red cell aplasia, macrocytic.
Caused by defect in RBC progenitor cells that increases RBC apoptosis and causes profound anemia
Will also see reticulocytopenia, but will have normal platelets and WBCs

195
Q

What organisms likely cause pseudoappendicitis and how are they differentiated?

A

Y. enterocolitica – also has erythema nodosum (1-2cm red nodules tender to palpation)
C. jejuni – no erythema nodosum

196
Q

What additional vaccines should HbSS children receive at age 2?

A

PPSV 23 (should have already received PCV13)
MCV4 (meningococcal)
Also influenza if they haven’t had it yet that year

197
Q

When should pap smears be recommended?

A

Only age 21 and later – then to all women regardless of sexual hx

198
Q

Tx of Kawasaki:

A

IVIG and HIGH-dose aspirin

199
Q

Why do Kwashiorkor children have large bellies?

A

Ascites
Dilated loops of bowel
Enlarged, fatty liver – d/t decreased apolipoprotein synth and decreased fat exportation

200
Q

What kind of organism is Listeria?

A

Gram + rod

201
Q

What is West Syndrome and how is it treated?

A

A pediatric seizure disorder characterized by the triad of:
Infantile spasms
Developmental regression
Hypsarrhythmia on EEG – pathognomonic, disorganized background w/high-A waves and spikes. Seen interictally as well.
Tx: ACTH (DoC). Vigabatrin may be considered as an alternative

202
Q

What test should be done repeatedly in pts. w/Kawasaki?

A

Echo

203
Q

What is the most specific sx that would suggest leukemia in the setting of pancytopenia?

A

Bone Pain

204
Q

How do infants w/urea cycle defects typically present?

A

W/in 24-48hrs with poor feeding, tachypnea, and lethargy.
Hyperammonemia will cause central hyperventilation leading to Respiratory alkalosis.
CBC, lactate, electrolytes, glucose and ketones will all likely be normal.

205
Q

How to differentiate pyruvate metabolism disorders from urea cycle defects:

A

Both will have hyperammonemia, but Pyruvate will also have lactic acidemia.

206
Q

What will be seen on CXR in an infant w/meconium aspiration?

A

Coarse, irregular infiltrates and hyperexpansion.

207
Q

What should be expected in a patient who has an acute decompensation on mechanical ventilation?

A

Pneumothorax.

208
Q

What is the major life-threatening cx of ALL?

A

Infection – so in any child w/fever in the setting of suspected ALL abx should be started asap before confirming diagnosis.

209
Q

What valve abnormalities does Acute rheumatic fever cause?

A

FIRST mitral regurg

Then years later the valve may become stenotic.

210
Q

What is the best tx of Von Gierke disease?

A

Uncooked cornstarch – it is a glucose polymer.

211
Q

Why isn’t ceftriaxone used in infants?

A

It increases the risk of hyperbilirubinemia by binding to albumin and displacing the BR.

212
Q

Characteristic finding on AXR of NEC:

A

Pneumatosis intestinalis – air in the bowel wall.

213
Q

What is the most sensitive and specific test for iron-deficiency anemia?

A

Serum ferritin levels.

Should order these over Fe levels.

214
Q

What is the best way to asses urine protein excretion?

A

To determine the mg of protein/mg of Creatinine ratio.
This is easier than measuring 24hr proteins and such.
Nephrotic range proteinuria is considered >3mg protein/mg creatinine on a spot urine sample.

215
Q

How will classic galactosemia present and what will be seen on labs?

A

Within days-weeks after introduction of formula/breastmilk babies develop lethargy, poor feeding, jaundice, cataracts, and metabolic acidosis.
Labs: direct hyperbilirubinemia, elevated transaminases, prolonged PT and aPTT, hypoglycemia and aminoaciduria.

216
Q

First line tx of thrush:

A

Topical Nystatin

Fluconazole can be used in cases unresponsive to Nystatin

217
Q

What disease is characterized by a defective production of neutrophil superoxide?

A

Chronic Granulomatous disease – defect in phagocyte NADPH oxidase.

218
Q

What are significant developmental milestones seen at 18mos?

A

Walk backwards, run, walk up steps, scribble, build a tower of 2 cubes, point to at least one body part, say six words, remove garments.
This is the first age of autism screening, and then second is at 24 mos.

219
Q

When should autism screening be done?

A

18 and 24 mos

220
Q

When should hearing and vision screening be done?

A

Vision – 3 and 4 years old

Hearing – 4 years old

221
Q

When does BP become a routine part of the physical exam?

A

After age 3yr

222
Q

When should lead screening be done?

A

At age 1 and 2 years.

223
Q

What ocular abnormalities are seen in Alport syndrome?

A

Lens dislocation, posterior cataracts, and corneal dystrophy

224
Q

Features of Fanconi Anemia:

A

Inherited DNA repair defect – bone marrow failure
Clinical: short stature, hypo-/hyperpigmented macules, abnormal (hypoplastic or absent) thumbs, and genitourinary malformations
Labs: pancytopenia, + chromosomal breakage testing.

225
Q

What type of anemia is typically seen in Fanconi anemia?

A

Macrocytic anemia and pancytopenia d/t bone marrow failure

226
Q

What type of shunt does a PDA cause?

A

An extracardiac L-to-R shunt aka it shunts blood from the systemic to pulmonary circulation.

227
Q

What organism causes tinea capitis?

A

Most commonly in the US – Trichophyton tonsurans

228
Q

What procedure should be done to tx severe (stage 4) GERD?

A

Fundoplication

229
Q

What Rx would be best to give a patient with signs of Cardiogenic shock w/decreased renal fxn?

A

Dopamine.

230
Q

Possible complication of topical anesthetic use?

A

Methemoglobinemia.

Dapsone and nitrites may cause this as well.

231
Q

What are helmet cells?

A

Schistocytes

232
Q

What are Howell-Jolly bodies?

A

Normal nuclear remnants of RBCs – normally removed by the spleen, so seen in ppl w/hx of splenectomy or functional asplenia (SCD).

233
Q

How would SLE likely present in childhood?

A

With neuropsychiatric sxs, immune-mediated anemia (Coombs +), thrombocytopenia, arthritis, and nephritis

234
Q

What screening exams should be performed regularly on children with JIA?

A

Slit lamp exams to check for ocular involvement – anterior uveitis etc.

235
Q

What is physiologic jaundice?

A

Unconjugated hyperbilirubinemia that occurs >24hrs (norm about 72hrs) and goes away by one week.
D/t immature liver conjugation enzymes.
ALL JAUNDICE <24hrs IS PATHOLOGICAL

236
Q

What kind of medication is Clotrimazole?

A

Topical anti-fungal

237
Q

What kind of medication is Triamcinolone?

A

Topical corticosteroid

238
Q

What Immunodeficiency disorder may present after administration of BCG vaccine?

A

IL-12 receptor deficiency – get disseminated mycobacterial and fungal infections, and decreased IFN-y

239
Q

What Immunodeficiency disorder has increased risk of gastric ca?

A

CVID – also increased risk of lymphoma.

240
Q

What are the primary disorders of B-cells only?

A

XLA – bacterial and enteroviral infections. Absent B cells in peripheral blood.
IgA deficiency – Airway and GI infections. A/w atopy, AI, and anaphylaxis. Only IgA decreased
CVID – defect in B-cell differentiation. Increased risk of AI dxs, bronchiectasis, lymphoma, gastric ca., and sinopulmonary infections. Decreased plasma cells and Igs. Presents later.

241
Q

Primary disorders of T-cells only:

A

DiGeorge
IL-12R deficiency
Hyper-IgE aka Job syndrome
Chronic mucocutaneous candidiasis

242
Q

Immunodeficiencies of B&T cell dysfxn:

A

SCID
Ataxia-telangiectasia
Hyper-IgM syndrome
Wiskott-Aldrich syndrome

243
Q

Immunodeficiences of phagocyte dysfxn:

A

LAD
Chediak-Higashi
Chronic granulomatous disease

244
Q

Most common type of SCID:

A

IL-2R gamma chain defect – X-linked

2nd – ADA deficiency, AR

Step 2 Stuff (6 decks)

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