Peds Shelf 7

Front 1

Wide-based gait, decreased vibratory/position sense in legs, absent lower DTRs + inheritance pattern

Back 1

Friedrich's ataxia (AR)

Front 2

Most common non=CNS complication of Friedrich's ataxia

Back 2

Necrosis and degeneration of cardiac muscle fibers --> myocarditis, myocardial fibrosis, and cardiomyopathy (--> arrhythmias and CHF)

Front 3

Unilateral gynecomastia in a 14yo

Back 3

Pubertal gynecomastia: reassure

Front 4

Rx for platelets <30,000

Back 4

Corticosteroids (platelet transfusion only if actively bleeding, as can often cause further destruction of platelets; splenectomy if life-threatening bleed)

Front 5

Why does a hyperextended neck provide relief in epiglottitis?

Back 5

Maximizes the airway diameter

Front 6

Signs of chronic upper airway obstruction since birth, worse while supine, relieved with neck extension

Back 6

Vascular ring (rx: surgery)

Front 7

Cyanosis aggravated by feeding and improved with crying

Back 7

Choanal atresia

Front 8

Inspiratory stridor exacerbated by any exertion; symptoms appear w/in first 2 weeks of life with progressive increase in severity; no cyanosis

Back 8

Laryngomalacia

Front 9

Precocious puberty + signs of severe androgen excess (e.g. severe acne, rapid growth)

Back 9

Precocious pseudo-puberty, a gonadotropin-independent process, often caused by late-onset CAH

Front 10

Heart disease associated w/ neonatal lupus

Back 10

Congenital heart block

Front 11

Heart disease associated w/ congenital rubella

Back 11

PDA

Front 12

Heart disease associated w/ Edward's syndrome

Back 12

VSD

Front 13

Heart disease associated w/ Williams' Syndrome

Back 13

Supravalvular aortic stenosis

Front 14

Breathholding spells may be associated w/ this abnormality

Back 14

Anemia

Front 15

Craniotabes (ping-pong ball sensation over occiput), rachitic rosary (enlargement of costochondral junctions) and thickening of wrists and ankles

Back 15

Vitamin D deficiency rickets

Front 16

Lab findings in Vita D deficiency rickets

Back 16

Serum calcifediol level is decreased, alk phos is increased

Front 17

XR findings in rickets

Back 17

Cupping and fraying of distal ends of long bones; double contour along lateral outline of radius

Front 18

General tenderness, pseudoparalysis (frog position), bluish-purple and spongy/swollen gums over incisors, and depression of sternum

Back 18

Vita C deficiency

Front 19

XR findings in scurvy

Back 19

Pencil-point thinness of long bone cortex and sharply outlined epiphyseal ends

Front 20

How to differentiate autism and Asperger's

Back 20

Asperger kids are more socially aware and communicative; have normal language development

Front 21

Baby born at home with poor suckling and fatigue followed by rigidity, spasms, and opisthotonus + red and inflamed umbilical stump

Back 21

Neonatal tetanus

Front 22

Main causes of death in neonatal tetanus

Back 22

Apnea or septicemia

Front 23

Prevention of neonatal tetanus

Back 23

Maternal immunization

Front 24

Tests used for neonatal HIV diagnosis

Back 24

PCR, viral culture, p24 antigen testing (can't test antibodies b/c maternal Abs cross the placenta)

Front 25

Average survival with neonatal HIV

Back 25

AIDS by 1yo, death by 3yo (progresses more rapidly)

Front 26

Empiric Abx for septic arthritis

Back 26

IV nafcillin or IV cefazolin

Front 27

Mechanism for improvement of tet spells w/ squatting

Back 27

Increased systemic vascular resistance (--> increased blood flow ot pulmonary circulation)

Front 28

In child w/ epidural hematoma, features that necessitate emergent craniotomy

Back 28

GCS < 8, increased ICP, pupillary abnormalities, hemiparesis, or cerebellar signs

Front 29

Rx for pts with a small epidural hematoma and no focal neuro signs

Back 29

F/u CT in 24 hrs

Front 30

Kids with HSP are at risk for this GI problem

Back 30

Intussusception

Front 31

Holosystolic murmur over LLSB and rumbling diastolic murmur over apex

Back 31

VSD

Front 32

Rx for vaginal foreign body

Back 32

Irrigation with warmed fluid first; if unsuccessful, exam and removal with sedation or under general anesthesia

Front 33

Infants who are SGA are at risk for these 7 complications

Back 33

Hypoxia, perinatal asphyxia, meconium aspiration, polycythemia, hypothermia, hypoglycemia, hypocalcemia

Front 34

Paralysis of the left hand and ipsilateral Horner syndrome (miosis and ptosis) after delivery

Back 34

Klumpke paralysis (brachial plexus injury to C7, C8, and T1)

Front 35

Absent Moro reflex and intact grasp reflex of affected arm; adduction and internal rotation/ pronation of arm

Back 35

Erb-Duchenne palsy (C5 and C6)

Front 36

Craniofacial anomalies, fingernail hypoplasia, growth deficiency, dev't delay, cardiac defects, facial clefts

Back 36

Phenytoin use during pregnancy

Front 37

Structure affected in Guillain-Barre

Back 37

Peripheral nerves

Front 38

What test should always be done in patients w/ apparent subQ emphysema 2/2 severe coughing paroxysms

Back 38

CXR to r/o pneumothorax

Front 39

Rx for chronic granulomatous disease?

Back 39

Daily TMP-SMX and gamma-INF 3x/wk

Front 40

Coagulopathy, neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections (S. aureus) and progressive lymphoproliferative syndrome

Back 40

Chediak-Higashi Syndrome

Front 41

Findings diagnostic of Chediak-Higashi syndrome

Back 41

Neutropenia and giant lysosomes

Front 42

Chronic pruritic dermatitis, recurrent staph infections, eosinophilia, coarse facial features

Back 42

Hyper IgE (Job's) syndrome

Front 43

What must be done prior to surgery for pyloric stenosis?

Back 43

Stabilization w/ IVF and K+

Front 44

2 abnormalities where corrective surgery should be delayed until school age

Back 44

Umbilical hernia
ASD

Front 45

Neck swelling w/ rotation to one side, resisting rotation or flexion + diagnostic test

Back 45

Torticollis
Neck XR to r/o cervical spine fracture or dislocation

Front 46

3 most common causes of acquired torticollis

Back 46

URIs, minor trauma, cervical lymphadenitis

Front 47

How to differentiate HSV meningitis from bacterial meningitis

Back 47

HSV meningitis usually has focal neurologic signs, and fewer band forms on diff

Front 48

Sail sign on CXR

Back 48

Thymic shadow (appears very large in kids, but this is normal in kids <2yo)

Front 49

Infant with FTT, bilateral cataracts, jaundice, and hypoglycemia

Back 49

Galactosemia

Front 50

Enzyme deficiency in galactosemia

Back 50

Galactose-1-phosphate uridyl transferase deficiency

Front 51

Pts with galactosemia are at increased risk for this infection

Back 51

E. coli neonatal sepsis

Front 52

Cataracts in neonatal period

Back 52

Galactokinase deficiency

Front 53

Pertussis prevention for close contacts

Back 53

Erythromycin x 14 days (regardless of age, immunization, or symptoms)

Front 54

Dx and Rx for acute bacterial sinusitis

Back 54

Amoxicillin based on clinical, not radiographic findings

Front 55

Dx for muscular dystrophy

Back 55

Muscle biopsy

Front 56

What is postpericardiotomy syndrome?

Back 56

Reactive pericarditis w/ pericardial effusion that occurs after surgery for congenital heart disease

Front 57

Dx test for proteinuria w/o hematuria

Back 57

Repeat 2 more times (is most likely just transient proteinuria)

Front 58

Rx for Bruton's agammaglobulinemia

Back 58

IVIG infusion

Front 59

Rx for suspected congenital diaphragmatic hernia

Back 59

Orogastric tube w/ continuous suction to prevent bowel distension and further lung compression (followed by intubation)

Front 60

Rx for exposure to varicella

Back 60

Vaccine if within 3-5 days of exposure; varicella IG if immunodeficient

Front 61

When is surgery indicated for umbilical hernias?

Back 61

If persist to 3-4yo, >2cm, symptomatic, strangulated, or progressively enlarges

Front 62

Rx for absence seizures

Back 62

Ethosuximide or valproic acid

Front 63

2 toxoid vaccines

Back 63

Tetanus and diphtheria

Front 64

2 live attenuated vaccines

Back 64

MMR and chicken pox

Front 65

Children with congenital heart disease are at increased risk of this CNS pathology

Back 65

Brain abscess

Front 66

5 risk factors for brain abscess dev't

Back 66

Congenital heart disease, head trauma, infection of jaw/mouth/face/scalp, meningitis, cranial instrumentation

Front 67

Trauma to soft palate + stroke symptoms

Back 67

Internal carotid artery dissection

Front 68

How to distinguish Niemann-Pick from Tay Sachs

Back 68

Hepatomegaly and cervical lymphadenopathy only in NP

Front 69

Pathophys of infantile botulism

Back 69

NOT ingestion of preformed toxin; organisms enter GI tract, where they produce the toxin (a protease that blocks ACh release)

Front 70

Dilation of entire ventricular system w/ distinct enlargement of subarachnoid space over cerebral cortex

Back 70

Communicating hydrocephalus 2/2 subarachnoid hemorrhage (e.g. from intraventricular hemorrhage)

Front 71

Imaging difference btwn Dandy-Walker anomaly and Chiari malformation

Back 71

Dandy-Walker: cystic expansion of fourth ventricle
Chiari: protrusion of structures of posterior fossa thru foramen magnum

Front 72

Diagnostic test for pyloric stenosis

Back 72

Abdominal ultrasound

Front 73

Cause of edema in Turner Syndrome

Back 73

Abnormal development of lymphatic network

Front 74

Distinction btwn the arthritis in JRA and in rheumatic fever

Back 74

Migratory arthritis in rheumatic fever

Front 75

Scalp swelling limited to surface of one cranial bone

Back 75

Cephalohematoma (subperiosteal hemorrhage)

Front 76

Diffuse swelling that crosses suture lines

Back 76

Caput succedaneum

Front 77

Pain, pallor, poikilothermia, paresthesias, pulselessness, paralysis

Back 77

Compartment syndrome

Front 78

Increased gastric residual volume in a preterm neonate

Back 78

NEC

Front 79

Diarrhea, fever, and erythematous rash on abdomen (Rose spots)

Back 79

Salmonella poisoning

Front 80

Common organisms:
G+ diplococci
G+ cocci in clusters
G+ rods
G- cocci
G- rods

Back 80

Strep pneumo
Staph
Listeria and Bacillus
Neisseria
Pseudomonas, Haemophilus, Klebsiella, Legionella

Front 81

Neonate w/ irritability, high-pitched cry, poor sleeping, seizures, sweating, sneezing, tachypnea, V/D

Back 81

Neonatal abstinence syndrome (from opioid withdrawal)

Front 82

Drooling, abdominal distension, rattling breath sounds + atelectasis and gastric distension on XR

Back 82

Esophageal atresia

Front 83

Rx for epiglottitis

Back 83

Intubation w/ preparation for possible tracheostomy

Front 84

Slowly developing back pain, neuro dysfunction (e.g. incontinence), and palpable "step-off" at lumbosacral area

Back 84

High-grade spondyloisthesis

Front 85

Normal at birth, then apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, umbilical hernia

Back 85

Congenital hypothyroidism

Front 86

Test for CF if can't colelct enough sweat during pilocarpine iontophoresis

Back 86

DNA testing for CFTR mutations

Front 87

Lims shorter than trunk and head disproportionately large

Back 87

Achondroplasia

Front 88

Short but with normal proportions

Back 88

GH deficiency

Front 89

Signs of congenital hypothyroidism

Back 89

Constipation, prolonged jaundice, sluggishness, poor feeding, apnea, choking, macroglossia, excessive sleepiness

Front 90

Newborn w/ macrosomia, macroglossia, abdominal wall defects, linear ear creases, organomegaly, hypoglycemia

Back 90

Beckwith-Wiedemann Syndrome

Front 91

Infants w/ Beckwith-Widemann Syndrome are at increased risk of

Back 91

Malignancies (esp wilms, hepatoblastoma, and gonadoblastoma)

Front 92

Born normal and then loss of milestones, umbilical hernia, kyphoscoliosis, deafness, cloudy corneas, claw hand deformity (disease + deficiency)

Back 92

Hurler syndrome (alpha-L-iduronidase eficiency --> deposition of dermatan and heparan sulfate)

Front 93

What is the bone age in kids with constitutional short stature?

Back 93

Few years behind chronological age (indicating still time for a growth spurt)

Front 94

Inheritance of achondroplasia

Back 94

AD

Front 95

Short w/ delayed bone age, mental retardation, increased bone density esp in skull, brachydactyly, obesity w/ round facies and short neck, subcapsular cataracts, cutaneous and subQ calcifications, perivascular calcifications of basal ganglia

Back 95

Pseudohypoparathyroidism (Albright hereditary osteodystrophy)

Front 96

Lab findings in pseudohypoparathyroidism

Back 96

High PTH w/ low Ca and high phosphorous (receptors are resistance to PTH)

Front 97

Constipation, anorexia, vomiting, polyuria, lethargy after long-term immobilization b/c of fracture

Back 97

Immobilization hypercalcemia

Front 98

Dx test for immobilization hypercalcemia

Back 98

Serum ionized calcium and urinary calcium to creatinine ratio

Front 99

Complicaitons of immobilization hypercalcemia (and resultant hypercalciuria)

Back 99

Nephropathy, nephrocalcinosis, hypertensive encephalopathy, convulsions

Front 100

Hyponatremia, hyperkalemia, hypoglycemia in first 15 days of life

Back 100

CAH

Front 101

Urine that turns black when exposed to air (disease + deficiency)

Back 101

Alkaptonuria (AR), deficiency of homogentisic acid oxidase

Front 102

Rx for alkaptonuria

Back 102

None needed; supplemental ascorbic acid may delay onset of arthritis in adults

Front 103

Cholestasis, elevated LFTs, bleeding into CNS, GI tract, or at umbilical stump

Back 103

alpha-1 antitrypsin deficiency

Front 104

Hypoglycemia in a preemie is due to

Back 104

Decreased glycogen and fat stores

Front 105

Lab values in nutritional rickets

Back 105

Normal serum calcium (decreased absorption, but released from bone and decreased excretion), low phosphate (due to phosphaturia), high PTH (stimulated by transient hypocalcemia, causing the other findings), elevated alk phos

Front 106

Lab findings in vita D resistant rickets + pathophys and inheritance

Back 106

Low phosphate, normal calcium (genetic abnormality in reabsorption of phosphate), X-linked

Front 107

Numbness, tingling, seizures

Back 107

Hypoparathyroidism (--> hypocalcemia and hyperphosphatemia)

Front 108

Obesity, mental retardation, hypogonadism, polydactylyl, retinitis pigmentosa w/ night blindness

Back 108

Laurence-Moon-Biedl syndrome (AR)

Front 109

Sodium and potassium values in pts with central/nephrogenic diabetes insipidus

Back 109

High Na (losing water), somewhat high K+

Front 110

Lab findings in hyperaldosteronism

Back 110

Hypernatremia, hypokalemia (enhancing sodium-potassium exchange), hyperchloremia, alkalosis

Front 111

Lab findings in pts with hyperlipidemia (e.g. von Gierke's)

Back 111

Pseudo hyponatremia and hypokalemia

Front 112

Most common congenital anomaly in Ehlers-Danlos

Back 112

MVP

Front 113

Abnormalities of upper extremities, hypoplastic radii, thumb abnormalities, cardiac anomalies, missing pec major (chest wall musculature)

Back 113

Holt-Oram syndrome