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COMLEX 2 > Peds > Flashcards

Peds Flashcards

1
Q

First A in APGAR

A

Appearance, 0 - blue/pale, 1 - pink trunk, 2 - all pink

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2
Q

P in APGAR

A

Pulse, 0 - 0, 1 - 100

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3
Q

G in APGAR

A

Grimace with stimulation, 0 - None, 1 - Grimace, 2 - grimace and cough

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4
Q

Second A in APGAR

A

Activity: 0 - Limp, 1 - Some activity, 2 - Active

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5
Q

R in APGAR

A

Respirations: 0 - None, 1 - Irregular, 2 - Regular

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6
Q

If a child has mastoiditis, what is the next best step in management?

A

CT head and neck to reveal extent of infection

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7
Q

Down Syndrome cancer risks

A

Increase ALL, Decrease solid organ tumors

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8
Q

Childhood Disintegrative Disorder

A

Normal development in early childhood then loss of acquired skills. Onset 2-10 yrs. Boys

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9
Q

Rett Disorder

A

Normal development until 2 yrs. then loss of acquired skills. Onset 2-10 yrs. Girls. Seizures

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10
Q

Pertussis: tx

A

14 Days of Erythromycin (a macrolide) for pt. and close contacts

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11
Q

Tx for Kawasaki Dz?

A

IV Ig and ASA

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12
Q

Classic signs of peritonsillar abscess

A

Fever, Trismus (can’t open mouth), “Hot potato” voice, Peritonsillar swelling, Uvula deviation

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13
Q

Ambiguous genitalia ddx?

A

5-alpha-reductase deficiency, Androgen Insensitivity

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14
Q

Bedwetting Tx.

A

Behavior mod, DDAVP, Imipramine (tricyclic)

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15
Q

Jejunal atresia keywords

A

bilious, triple bubble, d/t vascular accident in utero causing intestinal necrosis

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16
Q

Duodenal atresia keywords

A

bilious 1st day of life, double bubble, d/t failure of duodenum recanalize, assoc. w/ polyhydramnios

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17
Q

Hirschprung disease keywords

A

bilious, delayed passage of meconium age >48 hrs, Dx: Rectal biopsy gold standard

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18
Q

Necrotizing enterocolitis keywords

A

bilious, bloody stool, thrombocytopenia and unstable vitals, Dx: XRAY showing intramural air

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19
Q

Pyloric stenosis

A

NON-bilious, projectile vomiting, 3-6 wks age, olive mass, Dx: US

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20
Q

What is the most common nutritional deficiency in children?

A

Iron

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21
Q

When can cow’s milk be given?

A

Not until 1 y.o.

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22
Q

Infectious mononucleosis keywords

A

EBV, splenomegaly, posterior cervical lymphadenopathy

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23
Q

Leukocyte adhesion deficiency

A

recurrent bacterial infections, delayed umbilical cord separation, CD18 deficient, No Neutrophils in infected tissues but levels increased in blood (cause they can’t migrate)

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24
Q

Adenosine Deaminase Deficiency

A

Auto Rec, deficient mature B cells and T cells d/t adenosine toxicity

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25
Q

Chronic Granulomatous disease

A

impaired respiratory burst –> ineffective intracellular killing, Dx: (-) Nitroblue Tetrazolium test –> yellow is seen (normally should be blue)

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26
Q

Cerebral Palsy keywords

A

equinovarus deformity, clasp knife resistance

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27
Q

Galactosemia

A

galactose-1-p- uridyl transferase deficiency causing: jaundice, hepatomegaly, catarcts, hypoglycemia, Tx: Soy formula

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28
Q

Congenital CMV keywords

A

microcephaly, cataracts/chorioretinitis, periventricular calcifications, sensorineural hearing loss

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29
Q

DiGeorge keywords

A

22q11 microdeletion, decrease T cell production, wide set eyes, small mandible, holosystolic murmur/VSD/Tetralogy of Fallot, neonate with hypocalcemic seizures

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30
Q

Klinefelter (47XXY) cancer risk?

A

breast ca

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31
Q

XYY males description

A

pectus exxcavatum, explosive tempers –> “Juvenile delinquents”

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32
Q

What is the most common form of inherited mental retardation?

A

Fragile X (CGG repeat), S&S: macrocephaly, long face/forehead, high arched palate, large ears, macro-orchidism after puberty

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33
Q

Methylamlonic acidemia keywords

A

defect of methylmalonyl-CoA –> Succinyl coA, vegan breast feeding mothers who need B12

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34
Q

Who do you avoid HCO3- infusion in DKA?

A

HypOkalemia, shifts O2 curve left, cerebral acidosis, edema

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35
Q

What is the routine care of a child with sickle cell dz?

A

Daily PCN by 2 mo., Daily Folate by 6 mo., Meningococcal and PPV23 @ 2 y.o., Morphine/Hydromorphone for pain

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36
Q

Intussusception keywords, Dx? Tx

A

Bilious, RUQ Sausage mass, Currant Jelly Heme + stool, Target sign, Cannot give Rotavirus vaccine, Dx AND Tx: Air contrast enema

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37
Q

Meckel Diverticulum

A

remnant of omphalomesenteric (vitelline) duct, 3-6 cm size, 50-75 cm from ileocecal valve in 2 y.o. male, of gastric and pancreatic tissues causing painless hematochezia, Dx: Meckel Radionuclide scan/T-99 pertechnetate

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38
Q

choanal atresia

A

CHARGE syndrome, pink when crying, blue when stops crying/is feeding

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39
Q

Tx: Chlamydial conjunctivitis

A

Erythromycin PO

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40
Q

Meconium ileus

A

failure to pass meconium w/in first 48 hrs, assoc. with Cystic fibrosis. Tx: IVF, NG tube, surgery

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41
Q

Electrolyte imbalance in Cystic Fibrosis

A

hypOnatremic/hypoChloremic met alkalosis

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42
Q

Cystic Fibrosis

A

Auto Rec F508 mutation leading to recurrent sinopulmonary infections, steatorrhea, nasal polyps, Dx: Quantitative Pilocarpine Iontophoresis and Sweat Cl-, Tx: Intranasal glucocorticoids, Vit. ADEK supplementation, Aminoglycosides for Pseudomonas infections

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43
Q

Hypothyroidism growth curve in children

A

normal childhood growth then abrupt falling off growth curve

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44
Q

Tetralogy of Fallot keywords

A

PROV (pulm stenosis, RV hypertrophy, Overriding Aorta, VSD), boot shaped heart, Tet spells (irritable, tachypnea, cyanosis), harsh systolic murmur @ LSB, Tx: Knee-chest which increases SVR, O2, surgical repair before 6 mo.

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45
Q

Rheumatic Fever keywords

A

Strep pyogenes (Group A beta hemolytic), J

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46
Q

Henoch-Schonlein purpura (anaphylactoid purpura)

A

IgA mediated vasculitis post viral URI with mesangium deposition

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47
Q

Bruton’s (X-linked) Agammaglobulinemia

A

X-linked recessive defect in B TyKinase, recurrent pyogenic (strep pneumo, H. flu) infections, Dx: Dec. Ig GAME and B cells

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48
Q

Common Variable Immunodeficiency (CVID)

A

acquired hypogammaglobulinemia, older than Bruton’s, Dx: Dec Ig GAME but normal B cell count

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49
Q

Beckwith-Wiedemann

A

macroglossia, R sided hemi-hypertrophy, umbilical hernia, 11p15

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50
Q

First line tx. for club foot

A

Stretching and manipulation THEN serial casting between 3-6 mo.

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51
Q

Kawasaki Dz (Mucocutaneous Lymph node syndrome)

A

Fever >5d, polymorphous rash, cervical lymphadenopathy, bilateral non-exudative conjunctivitis, Inc. ESR, CRP, platelets, Tx: ASA + IVIg

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52
Q

Nurse Maid’s elbow

A

Radial head dislocation, Tx: Hyperpronation

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53
Q

Lesch-Nyhan

A

X-Recessive HGPRT deficiency –> mutilation, gouty arthritis

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54
Q

Gilbert Syndrome

A

Dec UDP glucuronosyl Transferase causing disorder of bilirubin metabolism

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55
Q

Criggler Najjar

A

Dec. UDP transferase., Inc. bili and encephalopathy.

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56
Q

What is the most common cause of neonatal sepsis from birth to 3 mo. of age?

A

Group B. Strep

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57
Q

Transient Tachypnea of Newborn

A

incomplete evacuation of fetal lung fluid in full term infants leading to respiratory condition, assoc. w/ C-sections, Tx: supplemental O2 PRN

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58
Q

Atrioventricular Canal Defect

A

common in Down Syn., systolic and diastolic heart mumur, split loud S2, EKG with RV hypertrophy, Cyanosis when nursing

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59
Q

Sturge Weber

A

seizures + MR + port wine stain along CNV territory, Dx: “tramline” gyriform intracranial calcifications on skull xray, Tx: seizure control, laser therapy for skin lesions

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60
Q

Measles “Rubeola”

A

fever 104, 4 “C”s, Tx: Vit. A will dec. morbidity and mortality

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61
Q

Erythema Toxicum Neonatorum

A

Benign papules/pustules spares palms and soles that shows up first 2 wks of life, Tx: Spontaneous resolution

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62
Q

SCID

A

no lymph nodes, no tonsils, no thymic shadow, Dec. B, T, NK cells, - recurrent sinopulmonary infections, oral candidasis, persistent diarrhea, opportunistic and viral infections

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63
Q

Wiskott Aldrich

A

X-Recessive WASP gene., Classically a young male with eczema, thrombocytopenia, purpura, and encapsulated infections, Dx: Dec. IgM, T, platelets, but Inc. IgA and E

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64
Q

Most common cause of 2ndary HTN in children?

A

Fibromuscular dysplasia, “string of beads” on renal a. angiogram (Right > Left)

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65
Q

Rubella “German Measles” S&S, Tx?

A
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66
Q

Congenital Diaphragmatic Hernia sequelae

A

pulmonary hypoplasia and HTN, polyhydramnios

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67
Q

S&S congenital diaphragmatic hernia

A

concave scaphoid abdomen, barrel shaped chest, dx: prenatal US, Tx: endotracheal intubation

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68
Q

Diamond-blackfan Anemia “Congenital hypoplastic anemia”

A

progenitor defect causing inc. RBC apoptosis, S&S: pallor, short, web neck, shielded chest, long thumbs, Dx: inc. fetal Hgb, dec. retic., macrocytic anemia, Tx: Corticosteroids

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69
Q

Klumpke’s Paralysis

A

C8-T1 injury, Claw hand: wrist extended, MCP jts. hyperextended, interphalangeal jts. flexed, Horner syndrome

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70
Q

Erb-Duchenne “Waiter’s Tip”

A

C5-C6 (Tip me “$5”), Dec. moro reflex, Intact grasp reflex

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71
Q

Most common primary bone tumor

A

Osteosarcoma, @ metaphysis of long bones, Dx: spiculated sunburst, periosteal elevation (Codman’s Triangle), Inc. AlkPhos, Inc. LDH, Tx: Chemo and Surgical excision

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72
Q

Refeeding Syndrome explained

A

Carbs –> insulin release –> intracellular movement of K, phos, Mg –> arrythmias and cardiopulmonary failure

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73
Q

Transposition of Great arteries keywords

A

Egg on a string, Narrow mediastinum, Cyanosis

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74
Q

Parvo B19

A

targets RBC precursors (dx: 0 Retics), lacy rash

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75
Q

Cephalohematoma

A

sub-periosteal hemorrhage (limited to surface of one cranial bone –> does not cross suture lines), no scalp discoloration

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76
Q

Tx. Pinworm

A

Scotch tape test, Metronidazole

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77
Q

Esophageal atresia/Tracheoesophageal fistula

A

Dx: Polyhydramnios on prenatal US, distended stomach and intestines, choking and regurg with initial feeding immediately after birth, risk for chronic GERD

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78
Q

Friedreich ataxia

A

AutoRec excess trinucleotide repeat sequences, Abnormal Tocopherol transfer protein, S&S: wide-based gait, dec. vibration/proprioception, no DTRs on LE, high plantar arches

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79
Q

What is the earliest manifestion of vaso-occlusion in sickle cell anemia?

A

dactylitis (AKA Hand-foot syndrome)

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80
Q

McCune Albright

A

defect in G-protein cAMP-kinase, 3 P’s: precocious puberty, pigmentation (cafe au lait spots), polyostotic fibrous dysplasia

81
Q

Craniopharyngioma keywords

A

Rathke’s pouch, suprasellar and inferior to optic chiasm

82
Q

Myotonic Dystrophy

A

AutoDom CTG trinucleotide repeat in DMPK gene on Chromosome 19q13.3, Facial weakness, hand grip myotonia, inverted V-shaped lip

83
Q

Duchenne Muscular Dystrophy

A

X-Rec deletion of Dystrophin gene on chrom Xp21, Dx: DNA blood analysis then mm. biopsy if blood samples not diagnostic, S&S: Trendelenburg waddling gait, progressive proximal mm. weakness (Gower’s), calf pseudohypertrophy, death from respiratory failure

84
Q

Becker Muscular dystrophy

A

X-Rec dystrophin gene deletion from Xp21.

85
Q

IgA Deficiency

A

recurrent sinopulmonary and GI infections and anaphylactic transfusion reactions (episodic diarrhea secondary to Giardia)

86
Q

Posterior Vermis Syndrome

A

unbalanced gait, trunk dystaxia, horizontal nystagmus, papilledema, HA/vomiting, d/t Medulloblastoma

87
Q

Fetal Alcohol Syn keywords

A

small palpebral fissues, smooth philtrum, small gestational age, septal defects

88
Q

Congenital adrenal hyperplasia (CAH)

A

21-OH-lase deficiency leading to Inc. 17a-OH-progesterone, S&S: ambiguous genitalia in girls, hyponatremia, hypotension

89
Q

Brick Red Urate Crystals in diapers

A

sign of dehydration

90
Q

Tx. Lead Poisoning

A

Chelation: EDTA(IM) or DMSA(PO)

91
Q

Berger IgA Nephropathy, Dx?

A

recurrent painless hematuria preceded by URI, Dx: Immune complex deposition in mesangium

92
Q

Post Strep Glomerulonephritis, d/t, Dx?, Tx?

A

d/t GABHS (strep throat or impetigo), Dx: Dec. C3, normal C4. ASO +, anti-DNAse Abs, Tx: Fluids, diuretics, CCB for HTN

93
Q

Rapidly Progressive Glomerulonephritis Dx

A

Crescentic glomeruli

94
Q

Goodpasture Syndrome S&S, d/t?

A

nephritis + pulm hemorrhage, d/t SLE, HSP

95
Q

What is the most common cause of chronic stridor? S&S? Dx?

A

Laryngomalacia, inspiratory stridor worse when supine and when crying/feeding, Dx: laryngoscopy showing Omega shaped epiglottis

96
Q

Retropharyngeal abscess, Dx?

A

fever, drooling, dysphagia, stridor, and muffled voice which is often polymicrobial, Dx: widened pre-vertebral space on lateral neck xrays

97
Q

Croup (laryngotracheobronchitis) keywords

Tx?

A

Steeple sign (“Up” the steeple”), barking cough, parainfluenzae virus

Tx: Racemic Epinephrine

98
Q

Wilm’s tumor arises from? keywords

A

metanephros, mass that doesn’t cross midline

99
Q

What does the mesonephros give rise to?

A

Guy parts “EVES”: Ejaculatory ducts, Vas deferens, Epididydymis, Seminal vesicles

100
Q

What does the paramesonephron give rise to?

A

Female parts: Fallopian tubes, Uterus, Vagina

101
Q

NF-1 keywords

A

cafe au lait, MACROcephaly, feeding and learning problems

102
Q

NF-2

A

bilateral acoustic neuromas, cataracts

103
Q

Osteogenesis imperfecta keywords

A

COL1A1 mutation, osteopenia, blue sclera, hearing loss

104
Q

If IV access isn’t possible in pediatric emergencies what do you do?

A

Intraosseous access, often proximal tibia

105
Q

Hypertrophic cardiomyopathy keywords

A

AutoDom, systolic @ LSB, increases with valsalva, decreases with hand grip/passive leg raise, Tx: BB

106
Q

Vesicoureteral Reflux (VUR) Grading

A

I: undilated ureter, II: into ureter and collecting system, III: dilation of ureter and collecting system, IV: blunted calyces, V: tortuosity of ureter

107
Q

Vesicoureteral reflux (VUR) often leads to? dx? tx?

A

UTIs, Voiding cystourethrogram showing retrograde flow, Tx. TMP-SMX, Nitrofurantoin

108
Q

Epiglottitis keywords

A

abrupt stridor, Thumb’s sign “(epigloTTiTis Thumb), Haemophilus influenza type B,

Tx: Intubation. Hyperextension of neck, IV PCN, Cephalosporins, Carbapenems

109
Q

Prader Willi keywords

A

hyperphagia and obesity

110
Q

Chronic Granulomatous Disease, Tx?

A

-defective NADPH oxidase phagocytosis causing susceptibility to Catalase + organism (staph aureus, serratia, burkholderia, klebsiella, aspergillus), Tx: TMP-SMX and y-IFN 3x/wk

111
Q

Tx. Neonatal meningitis

A

Ampicillin + Ceftriaxone/3rd gen ceph

112
Q

Salmonella keywords

A

poultry, eggs, turtles, watery/bloody diarrhea, gastroenteritis

113
Q

Shigella keywords

A

Gram (-) Motile NON-lactose fermenter, watery diarrea –> bloody stool

114
Q

Trichuris Trichiura “Whipworms” Dx? Tx?

A

often found in South East US, whipworms cause rectal prolapse, stool for ova and parasites, Tx: Albendazole

115
Q

Which strains are covered by Gardasil

A

6, 11 –> genital warts, 16, 18 –> cervical ca.

116
Q

Febrile seizure: when does it occur? Different types? Tx?

A

between 6 mo. and 6 y.o., Simple: 15 minutes with possible lateralizing signs, Tx: Lorazepam (if seizures last >5 min) and Fosphenytoin if that doesn’t work

117
Q

If a child beyond neonatal period (e.g. 5 y.o.) has herpetic lesions, what should you suspect?

A

Possible sexual abuse

118
Q

Tx: Atopic dermatitis

A

topical corticosteroids (but not on face, genitalia, intertriginous), oral antihistamines

119
Q

Seborrheic dermatitis (“cradle cap”) keywords, tx?

A

greasy brown scales, Tx: Ketoconazole, low potency steroids

120
Q

Neuroblastoma keywords, Dx?

A

adrenal gland tumor, raccoon eyes, lymphadenopathy, bluish skin, Dx: CT, MRI, Increased VMA and Homovanillic acid (catecholamines)

121
Q

VATER

A

Vertebral abnormality, Anus imperforate, Tracheoesophageal fistula, Radial/Renal anomalies

122
Q

Malrotation and volvulus, d/t, Dx? Tx

A

bilious vomiting, blood per rectum, incomplete intestinal rotation during development, Using upper GI series Contrast: Lig. of Treitz on R side of abdomen (Malrotation), Corkscrew/birds beak of duodenum (Volvulus); Tx: Stop enteral feeds, use NG, LADD procedure

123
Q

Tx. Comedones

A
  1. Topical Benzoyl peroxide or Retin A, 2. Isoretinoin if severe
124
Q

What is the most common cause of UTIs in boys? dx? Tx?

A

Posterior Urethral valves causing dribbling, poor stream, palpable kidneys, VCUG and postnasal USG, Bladder catheterization then Endoscopic Transurethral valve ablation

125
Q

Tx ADHD. Children with ADHD often have what else?

A

Methylphenidate, Dextroamphetamine, Atomoxetine (SNRI), Oppositional defiant disorder, conduct disorder, anxiety, and depression

126
Q

Slipped Capital Femoral Epiphysis puts obese children at risk for?

A

Avascular necrosis

127
Q

Klippel-Feil keywords

A

torticollis, cervical vertebrae fusion, short neck

128
Q

Waardenburg Syndrome keywords

A

Think xmen’s rogue is deaf: Sensorineural hearing loss, Partial albinism/forelock of gray hair, Heterochromic iris

129
Q

Blount Disease (Idiopathic Tibia Vara) keywords

A

Abnormal medial Tibial epiphysis, Prominent Beaking and calcification

130
Q

Legg-Calve-Perthes keywords

A

painless “antalgic” limp gait d/t idiopathic avascular, femoral head necrosis, often boys, limited int. rotation and hip abduction

131
Q

Reye Syndrome S&S, Labs?

A

encephalopathy and acute liver failure (mitochondrial hepatopathy), Increased AST/ALT, PT/INR, PTT, Ammonia

132
Q

Catarrhal Stage (of Pertussis) S&S

A

mild cough and rhinitis for 1-2 wks

133
Q

Paroxysmal Stage (of Pertussis) S&S

A

coughing fits, inspiratory “whoop”, “staccato” cough, emesis, apnea, cyanosis. all for 2-6 wks

134
Q

Convalescent Stage (of Pertussis) S&S

A

resolution of cough and post-tussive emesis that comes after weeks to months

135
Q

Pertussis Tx. and Post-exposure ppx

A

1 mo: Azithromycin x 7d or Erythromycin x 14d

136
Q

Neimann Pick keywords

A

AutoRec, sphingomyelinase, no reflexes, hepatomegaly

137
Q

Tay Sachs keywords

A

AutoRec, Hexoaminidase, hyper-reflexia

138
Q

Krabbe keywords

A

Autorec, Galacto-cerebrosidase lysosomal storage disorder

139
Q

Gaucher keywords

A

Gluco-cerebrosidase deficiency, anemia, thrombocytopenia, hepatomegaly

140
Q

Hurler Syndrome keywords

A

Hydrolase deficiency lysosomal storage disorder, Coarse facies, hernias, cloudy cornea, hepatosplenomegaly

141
Q

Biliary Atresia S&S, Dx, Tx

A

conjugated hyperbilirubinemia –> jaundice, dark urine, pale stool, Dx: US, Hepatobiliary Scintigraphy, Liver biopsy, Intraoperative cholangiogram is the Gold standard, Tx: Kasai Hepato-porto-enterostomy procedure

142
Q

Von-Gierkes keywords

A

Type I Glycogen storage disease, G-6-Phosphatase deficiency, hypoglycemia, lactic acidosis, hyperuricemia, doll-like face

143
Q

Pompe (acid maltase deficiency) Dz. keywords

A

Type II, Floppy baby, macroglossia, heart failure

144
Q

Tx. Cyanide poisoning

A

Hydroxycobalamin

145
Q

Adverse effect of Lindane (Gamma-Benzene Hexachloride) lotion for scabies

A

Neurotoxicity in small infants

146
Q

What is a contraindication for all vaccines?

A

severe allergic rxn

147
Q

S&S Fetal Dilantin Exposure

A

midface hypoplasia, low nasal bridge, ocular HyPERtelorism, accentuated Cupid’s bow of upper lip

148
Q

S&S Small for Gestational Age baby

A

hypoglycemia, polycythemia –> Hyperviscosity syndrome

149
Q

What is the tx. for Hyperviscosity Syndrome in SGA baby

A

Partial exchange transfusion with saline/LR

150
Q

d/t, Dx, S&S? Tx

A

d/t surfactant deficiency secondary to prematurity,

151
Q

S&S of Donor Twin in Twin-Twin Transfusion

A

Oligohydramnios, anemia, hypovolemia, shock

152
Q

S&S of Recipient Twin in Twin-Twin Transfusion

A

Polyhydramnios, larger size

153
Q

Potter Sequence keywords

A

pulmonary hypoplasia secondary to oligohydramnios, wide spread eyes, low set ears, broad nose

154
Q

Caput succedaneum keywords

A

soft tissue swelling often presenting portion, ecchymotic, cross suture lines

155
Q

Atrial Septal Defect (ASD) keywords

A

wide, fixed split S2 @ LSB, pulmonary stenosis murmur, thrombocytopenia

156
Q

Ventricular Septal Defect (VSD) keywords

A

harsh, blowing, holosytolic @ LSB, diastolic apical rumble

157
Q

Ebstein anomaly keywords

A

downward displacement of tricuspid valve causing: quadruple rhythm S3 and S4 (Triscupid Regurg), mid-diastolic murmur @ LSB, R atrial hypertrophy

158
Q

Total Anomalous Pulm. Venous Return (TAPVR) keywords

A

obstruction to pulm. venous return, snowman venous congestion, fluffy CXR

159
Q

Noonan Syndrome aka Male Turner Syndrome

A

short, web neck, shield chest, cryoptorchidism, pulm stenosis, pectus excavatum

160
Q

What cardiac defect would you most likely see with Ehlers Danlos, Klinefelter?

A

Mitral Valve Prolapse, midsystolic click, @ apex

161
Q

Teeth development

A
  1. Mandibular central incisors, 2. Maxillary central incisors
162
Q

Tx. of Phenobarbital OD

A

activated charcoal

163
Q

Prune Belly Syndrome S&S

A

pulm. hypoplasia, wrinkly abd wall, oligohydramnios

164
Q

What is the most common hereditary nephritis?

A

Alport Syndrome (hematuria, hearing loss, ocular anomalies)

165
Q

Homocystinuria keywords

A

AutoRec, Cystathione B. Synthase deficiency, dislocated lenses, arachnodactyly

166
Q

Werdnig-Hoffman (Spinal mm. atrophy type I) S&S

A

floppy baby, defective survivor motor neuron gene destroying neurons in brain and spine

167
Q

Tuberous Sclerosis keywords

A

Ash-leaf hypopigmentation, Shagreen path over sacrum,

168
Q

Prehn Sign, significance?

A

Lifting of scrotum, Pain gets worse –> torsion, Pain gets better –> epididymitis

169
Q

Eisenmenger Syndrome

A

cyanosis and dyspnea secondary to L to R shunting seen in large VSDs

170
Q

Anemia of Prematurity, d/t

A

normocytic, normochromic, low retic anemia often seen in hospitalized, premature, LBW infants, d/t transition of erythropoiesis sites of neonate

171
Q

Roseola Infantum (Exanthem subitum) d/t

A

abrupt high fever THEN centrifugal rash to extremities,

172
Q

Parinaud syndrome, d/t

A

limited upward gaze, ptosis, Collier’s sign of upper eyelid retraction, d/t pinealoma

173
Q

Trachoma conjunctivitis: d/t, Dx:, Tx:

A

follicular conjunctivitis and neovascularization (pannus formation) in cornea, Dx: giemsa stain of scrapings,

174
Q

Edwards Syndrome keywords

A

Trisomy “E”ighteen for EDWARDS, LBW, microcephaly

175
Q

Patau Syndrome keywords

A

Trisomy “ThirTeen” for paTau, cleft lip, polydactyly

176
Q

Developmental Dysplasia of Hip, Dx? Tx?

A

Normal female baby with abnormal acetabulum resulting in hip instability and uneven leg creases, Ortolani and Barlow tests, Ultrasound to confirm, Tx: Pavlik harness to splint hip in flexion and abduction

177
Q

Pt. with cryptorchidism, hypospadias, aniridia, retardation should raise suspicion for what?

A

Wilms Tumor. Baby has WAGR (Wilms, Aniridia, GU, Retardation)

178
Q

When is HepB Vaccine given?

A

Birth, 1-2, 6-18

179
Q

When is Rotavirus vaccine given?

A

2,4,6

180
Q

When is DTaP vaccine given?

A

2,4,6, 15-18, 4-6y

181
Q

When is Hib and Pneumoccocal vaccine given?

A

2,4,6, 12-15

182
Q

When is Inactivated Polio vaccine given?

A

2,4, 6-18, 4-6y

183
Q

When is influenza vaccine given?

A

yearly from 6 mo onward

184
Q

When is MMR and Varicella vaccine given?

A

12-15, 4-6

185
Q

When is Meningococcal Vaccine given?

A

2-6y

186
Q

When is Tdap given? (Tetanus)

A

Substitute 1 time dose of Tdap for Td booster, then boost with Td q10y

187
Q

Wilson’s Disease inheritance?

pathophys?

S&S?

Tx?

A

Auto Recessive

reduced Copper excretion from liver leading to:

Kayser-Fleischer rings, parkinsonism, icterus, elevated AST/ALT, neurologic S&S

Tx: Penicillamine, Screen 1st Degree relatives

188
Q

Tx. Lead poisoning

A

EDTA

189
Q

Tx. Mercury

A

Dimercaprol

190
Q

Tx. Iron poisoning

A

Deferoxamine

191
Q

Subgaleal hemorrhage keywords

A

crosses suture lines

grows slowly over first day of life

Hemorrhagic shock!

192
Q

Lab abnormalities in baby born to diabetic mother

A

Hypoglycemia

Hypocalcemia (d/t low PTH)/Hypomagnesemia

Hyperphosphatemia

193
Q

Meconium Aspiration Syndrome

What would you see on CXR?

What complication can arise?

A

Term Baby with persistent respiratory distress (retractions, grunting, flaring)

CXR with bilateral patchy opacities, possible hyperinflation or pneumothorax

Persistent Pulmonary HTN –> acidosis and hypoxia and death

194
Q

Posterior Urethral Valves

S&S

Dx

A

male infant with hydronephrosis/cortical thinning, bilateral flank mass AND an abdominal mass, may also have Potter facies and pulmonary hypoplasia

Dx to confirm: Voiding Cystourethrogram

195
Q

Langerhans Histiocytosis X keywords

A

solitary, lytic painful long bone lesions in children

eosinophilic

Tx: Resolve spontaneously

196
Q

Precocious Puberty Tx

A

Leuprolide (GnRH agonist) will negative feedback on GnRH

197
Q

McCune-Albright

S&S

Tx

A

precocious puberty

Polyostotic fibrous dysplasia

Unilateral café au lait spots “Coast of maine border” that doesn’t cross midline

Tx: Ketoconazole (estrogen synthesis blocker), Tamoxifen (anti-estrogen)

198
Q

Henoch-Schonlein Purpura (HSP)

S&S

Tx

A

Vasculitis affecting children, 50% after URI

IgA and C3 deposition in small vessels

palpable purpura on legs, buttocks, abdominal and joint pain, hematuria

Tx: Supportive care

COMLEX 2 (19 decks)

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