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1

Henoch-Schonlein purpura

IgA mediated leukocytoclastic vasculitis

sx: palpable purpura, arthritis/arthralgia, abd pain/intussusception, renal dz (IgA dephropathy)

--> nml plt, coags, CBC
--> normal or increased creatinine
--> hematuria +/- RBC casts and proteinuria

2

NF-1 vs. NF-2

NF1 = cafe au lait spots, macrocephaly, short stature, feeding problems, learning disabilities, tumors later in life

NF2 = Bilateral acoustic neuromas + cataracts

3

Pityriasis Rosea

+/- viral prodrome
annular, pink herald patch on trunk
-oval lesions in "christmas tree" pattern
-pruritis

TX = reassurance (resolve spontaneously in weeks to months)

tx itching with anthistamines

4

Selective Mutism

Refusal to speak in specific social situations, nml elsewhere

5

Congenital torticollis

head tilt to one side
-postural deformity
-palpably, well circumscribed, mass that does NOT transilluminate on inferior aspect of SCM

6

Legg-Calve-Perthes disease

idiopathic avascular necrosis of femur

-boys age 3-12
-insidious hip pain + limp
-decreased hip ABduction and internal rotation

TX: non-weight bearing, splinting, (?) surgery

7

Slipped capital femoral epiphysis

-limp + insidious hip pain
-OBESE adolescent
-XR --> posterior displacement of femoral head

8

Reye syndrome

2/2 peds ASA use w/influenza or varicella infection

--> Acute liver failure (microvesicular steatosis on liver bx)
--> elevated AST, ALT, PT, INR, PTT, and ammonia

TX: supportive

9

Eosinophilic esophagitis

chronic, immune-mediated esophageal inflammation

sx: dysphagia, epigastric pain, reflux/vomiting, food impaction, associated atopy

DX: endoscopy and esophageal biopsy with > 15 eos/hpf

TX: dietary modification +/- topical glucocorticoids

10

Gonnococcal vs. chlamydial neonatal conjunctivitis

Gonococcal: onset at 2-5 days
-marked eyelid swelling, profuse purulent discharge, corneal edema/ulceration
TX: single IM dose of 3rd gen cephalosporin

Chlamydial: mild eyelid swelling, watery/serosanguinous, or mucopurulent eye discharge
TX: PO macrolide

11

Leukocyte adhesion deficiency

-recurrent skin and mucosal bacterial infections
--> no pus (lack of neutrophils at inflammation site)
--> poor wound healing

-Delayed umbilical cord separation (>21 days)

-Marked peripheral leukocytosis

Pathophys: defective integrins on leukocyte surface --> neutrophils can't adhere to vascular endothelium and exit

12

Adenosine deaminase deficiency

AR
Severe combined immunodeficiency

-deficient formation of mature B and T lymphocytes
-Severe infections and failure to thrive
-Labs show marked lymphopenia

13

X-linked agammaglobulinemia

Defective B lymphocyte maturation due to mutation in Bruton tyrosine kinase

-Recurrent sinopulmonay and GI infections
-LOW B cell and Ig concentrations

14

Patellofemoral stress syndrome

what makes it worse?

overuse injury seen in runners

anterior knee pain that worsens upon descending steps or hills

pain localized to patella

15

Breastfeeding failure jaundice

presents in 1st week of life
-Lactation failure resulting in --> decreased bilirubin elimination, increased enterohepatic circulation

--> suboptimal breastfeeding, signs of dehydration

TX: increase frequency and duration of feeds to stimulate milk production and maintain adequate hydration

16

Breast milk jaundice

starts at 3-5 days, peaks at 2 weeks

-High levels of B-glucuronidase in breast milk deconjugates intestinal bilirubin and increases enterohepatic circulation

--> adequate breastfeeding w/ normal exam

17

Treatment of Croup (laryngotracheitis)

mild (no stridor at rest) --> humidified air +/- corticosteroids

Moderate/severe (stridor at rest) --> corticosteroids + nebulized epinephrine

18

Necrotizing enterocolitis (NEC)

risks: prematurity, very low birth weight
--> gut immaturity and exposure to bacteria from enteral feeds --> inflammation and damage to bowel wall

*Decreased rates of NEC in breastfed premature infants

DX: abd XR with air in bowel wall (pneumatosis intestinalis) and portal venous air --> can lead to perforation and pneumoperitoneum

w/leukocytosis and metabolic acidosis.

19

Edwards Syndrome (trisomy 18)

microcephaly, micrognathia, overlapping fingers, absent palmar creases, rocker-bottom feet

Increased risk of VSD (holosystolic murmur at LL sternal border)

20

Ewing's sarcoma

malignant tumor in LE > UE

typically in metaphysis and diaphysis of femur

very aggressive, early mets to lungs and lymph nodes

on XR has onion skin periosteal reaction or moth-eaten/mottled appearance and extension into soft tissue

21

Causes of QT prlongation

1. hypocalcemia
2. hypokalemia
3. hypomagnesmia
4. Meds - abx, psychotropics, opiods, antiemetics, antiarrhythmics

5. Inherited

22

Inherited disorders of QT prlongation

1. Jervell and Lange-Nielsen
2. Romano-Ward syndrome

23

Jervell and Lange-Nielsen

AR, congenital long QT due to defect in potassium channels

-associated with congenital deafness

-increased risk of syncope, ventricular arrhythmias, sudden death, and torsades

TX: B-blockers, normal electrolyte levels, avoid QT prolonging medications, avoid rigorous exercise

*if h/o syncope, add pacemaker

24

Methemoglobinemia

caused by exposure to oxidizing agent (anesthetics, nitrites, dapsone)

Fe2+ --> Fe3+ (ferric state) has increased O2 affinity --> decreased oxygen delivery to peripheral tissues

SX: cyanosis, dark chocolate blood, low pulse ox, PaO2 is NORMAL

25

Minimal change disease

most common cause of nephrotic syndrome in children (age 2-3 yrs, most <10yrs)

Path: T-cell mediated injury to podocytes causes increased molecular permeability to albumin

SX: edema, fatigue, no hematuria, hypoalbuminemia, proteinuria

TX: corticosteroids

26

Rotavirus vaccine is given....

given between 2-8 months

27

McCune-Albright Syndrome

SX: precocious puberty (FSH, LH), irregular cafe-au-lait macules, recurrent fractures due to polyostotic fibrous dysplasia
-can also cause thyrotoxicosis (TSH), acromegaly (GH), cushings (ACTH)

GNAS gene mutation --> G protein activation and overproduction of pituitary hormones

28

Immune Thrombocytopenia

-antecedent viral infection
-asymptomatic petechiae and ecchymosis
-mucocutaneous bleeding
-isolated thrombocytopenia w/peripheral smear showing megakaryocytes

TX in kids:
-skin manifestations only --> observe
-bleeding --> IVIG or glucocorticoids

29

Laryngomalacia

increased laxity of supraglottic structures --> inspiratorry stridor that worsens when supine

-peaks at age 4-8 months

DX - direct laryngoscopy

TX - reassurance vs. supraglottoplasty

30

Riboflavin deficiency (B2)

-due to poor intake, poor absoption, probenacid, oral contraceptive use.

-SX: cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis

31

cheliosis, glossitis, ocular problems (keratitis, conjunctivits, corneal vascularization), and seborrheic dermatitis

which deficiency am I?

B2, riboflavin

32

Vitamin B6 (pyridoxine) deficiency

-enzyme deficiency vs. poor absorption vs. isoniazid, steroids, anticonvulsants

SX: seizures, peripheral neuritis, dermatitis, microcytic anemia

33

seizures, peripheral neuritis, dermatitis, microcytic anemia

which deficiency am I?

Vitamin B6 (pyridoxine) deficiency

34

Folate deficiency

due to poor intake/absorption, high demand (e.g. sickle cell), inborn errors of metabolism, MTX, phenytoin

SX: megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity

35

megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity

which deficiency am I?

Folate deficiency

36

Kawasaki disease

acute vasculitis of small and medium arteries

-typically age <5, asian
-prolonged high fever, unresponsive to antipyretics

-Conjunctivitis (BL, nonexudative)
-strawberry tongue
-rash
erythema, edema, desquamation of hands and feet
-cervical lymphadenopathy
-elevated ESR/CRP, thrombocytosis, sterile pyuria

**can get coronary artery aneurysms

TX: ASA + IVIG

37

evaluation of Malrotation --> _____

upper GI series abd XR

38

DDX of bilious emesis

meconium ileus
Hirschsprung disease
malrotation
duodenal atresia

39

Evaluation of meconium ileus and hirschsprung disease?

contrast enema

40

evaluation of duodenal atreasia

abd XR --> double bubble sign

41

Iron poisoning --> what sx?

abdominal pain, vomiting, diarrhea, hypotensive shock

*Anion gap metabolic acidosis
--> hepatic necrosis and pyloric stenosis

TX: deferoxamine, whole bowel irrigation

42

Di George Syndrome

CATCH -22

Conotruncal cardiac defects
Abnormal facies
Thympic aplasia/hypoplasia
Cleft palate
Hypocalcemia

*Defective development of pharyngeal pouches
Chr 22 q11.2 deletion

43

Sickle cell disease patients are at high risk for what bugs causing sepsis?

1) Strep pneumoniae
2) Haemophilus influenzae
3) Neisseria meningitidis

44

Wilms tumor is associated with what syndromes?

WAGR
Beckwith Wiedemann syndrome
Denys-Drash syndrome

45

WAGR

wilms tumor, aniridia, GU abnormalities, intellectual disability (retardation)

46

Wilms tumor sx

asymptomatic, firm, smooth abdominal mass that does not cross midline

47

Types of renal tubular acidosis

1) distal - poor hydrogen secretion into urine
2) proximal - poor bicarbonate resorption
4) aldosterone resistance

48

Type 1 distal RTA

poor hydrogen secretion into urine

urine pH > 5.5
low-normal serum potassium

CAUSES:
-genetic disorders
-med toxicity
-autoimmunie disorders

49

Type 2 (proximal) RTA

poor bicarbonate resorption

urine pH < 5.5
low normal potassium

CAUSES:
-fanconi sydrome (glucosuria, phosphaturia, aminoaciduria)

50

Type 4 RTA

aldosterone resistance

urine pH < 5.5

HIGH serum potassium

CAUSES:
-obstructive uropathy
-CAH

51

Vitamin A deificiency

impaired adaptation to darkness
photophobia
dry scaly skin
dry conjunctiva (zerosis conjunctiva)
dry cornia (xerosis cornea)
keratomalacia
bitot spots
follicular hyperkeratosis

52

Major Jones Criteria for rheumatic fever

Joints - polyarthritis
O (Heart) - carditis
Nodules - subcutaneous nodules
Erythema marginatum
Syndam chorea --> this is alone enough to make dx of RF

Polyarthritis is the most common finding

53

murmur of VSD vs. ASD

VSD = harsh, blowing holosystolic murmur best heard alonf the left lower sternum

ASD = fixed splitting of S2

54

Fanconi syndrome

diminished reabsorption of solutes by the proximal tubule

SX:
-dehydration
-polyuria, polydipsia
-hypokalemia
-proteinuria
-hypophosphatemia, hypocalcemia
-hyperchloremic metabolic acidosis (defect in bicarb reabsorption) --> high urine pH

55

Minimal change disease

sx?
what does renal bx look like?

T cell mediated injury to podocytes
-most common cause of nephrotic syndrome in children age 2-3

DX: renal biopsy without microscopic changes --> can see diffuse effacement of foot processes of podocytes on EM

TX: steroids

56

Thickened BM and subepithelial "spikes"

what am I?

Membranous glomerulonephritis

-common nephrotic syndrome in adults, rare in young children

57

McCune-Albright Syndrome

gonadotropin-independent precocious pubery

presents with irregular cafe-au-lait macules and fibrous dysplasia of bone

58

Presentation of transposition of the great vessels in a new born

SINGLE S2 +/- VSD

cyanosis within 24hrs of live

59

Trachoma

chlamydia serotypes A, B, and C

-leading cause of blindness world wide

SX: follicular conjunctivitis with concomitant nasopharyngeal infection

TX: azithromycin

60

EBV infection

fever
tonsillitis/pharyngitis +/- exudates
posterior or diffuse cervical lymphadenopathy
fatigue
+/- hepatosplenomegaly
+/- rash after AMOXiCILLIN