Peds D

Front 1

Traditionally, congenital LQTS has been characterized as 2 clinical entities:

Back 1

Romano-Ward syndrome, which is inherited in an autosomal dominant fashion and only has cardiac manifestations.Jervell and Lange-Nielsen syndrome, which is inherited in an autosomal recessive fashion and is associated with sensorineural deafness.

Front 2

Bazett formula

Back 2

measured QT by the square root of the R-R interval (<.45 s)

Front 3

North carolina and Rash

Back 3

Rickettsi rickettsii

Front 4

Rural with pneumonia/splenomegaly

Back 4

Coxiella brunetti

Front 5

Arkansas and LN

Back 5

Francisella Tularensis

Front 6

Gulf of Mexico and oyster

Back 6

Vibrio vulnificus

Front 7

Swimming pool and lesion/LN won't heal

Back 7

Mycobacterium marinum

Front 8

A swimming pool and conjunctivitis

Back 8

Adeno virus

Front 9

San Joaquin Valley and pneumonia

Back 9

Coccidiodes immitis

Front 10

Nantuckett and malaria like-illness

Back 10

Babesia microti

Front 11

Missouri and pancytopenia

Back 11

Ehrlichia chaffeenis

Front 12

Dessert Southwest and flea-bites

Back 12

Yersinia pestis

Front 13

Rickets (3 forms)

Back 13

1) Vitamin D–resistant form
(hypophosphatemia)
2) Renal form
3) Vitamin D deficiency form
widening of the cartilaginous growth plates, which
is the main radiographic feature of the disease in a
child.

Front 14

OI

Back 14

Type I (mildest 50%), is an autosomal dominant condition.
Type II most severe, freq death in infancy
Type III worst type of those who survive infancy
Type IV-VIII white sclerae variable sev and freq, present later

Front 15

ALL Musculoskeletal Sx

Back 15

20-40% pain and swelling in the joints and bone pain.
50-90% pos Skeletal surveys (many asymptomatic).

Front 16

idiopathic juvenile osteoporosis

Back 16

osteopenia at about seven or eight years of age, often accompanied
by bone pain and fractures, including vertebral
compression fractures. Spontaneous recovery occurs
at about the age of 15 years.

Front 17

ALL Statistics

Back 17

2500 cases/year
80% pre–B-cell
5% of the cases B-Cell
15% T-cell–precursor type

The current rate of cure among children with acute
lymphoblastic leukemia is about 80 percent.

Front 18

ALL
unfavorable prognostic indicators

Back 18

§ age <1 year or >10years,
§ male sex
§ black race,
§ mediastinal mass
§ hepatosplenomegaly or lymphadenopathy
§white-cell count >50,000
§T-cell phenotype

Front 19

Birth defects from phenytoin

Back 19

Orofacial clefts,
cardiac malformations,
genitourinary defects

Front 20

Second stage of Lyme disease

Back 20

early disseminated Lyme disease, consists of arthritis, which occurs in 50% to 60% of untreated cases, facial nerve palsy, aseptic meningitis,and carditis.

Front 21

most common of the urea cycle disorders

Back 21

OTC deficiency (X-linked)

Front 22

Teratogenicity of valproate

Back 22

One to 2 percent of fetuses exposed to valproate in utero develop neural-tube like defects (spina bifida aperta, open lumbosacral myelocele), a 10- to 20-fold increase over the general population

Front 23

most common type of pituitary adenoma

Back 23

prolactinoma

Front 24

Hodgkin's prurigo

Back 24

Hodgkin's disease has a well-known association with pruritus and secondary skin lesions

Front 25

Sturge-Weber

Back 25

ipsilateral leptomeningeal angioma

Front 26

bicycling, lip smacking, arm extension

Back 26

extra-pyramidal movements

Front 27

Third stage Lyme disease

Back 27

chronic stage, characterized by prolonged arthritis, encephalopathy,carditis and acrodermatitis chronica atrophicans.

Front 28

hypomelanosis of Ito

Back 28

"1) hypopigmentation that develops along the lines of Blaschko 2) Approximately 67% of patients have been reported to have an associated CNS, musculoskeletal, or ocular anomaly.

Front 29

small vessel vasculitides (6)

Back 29

WG, Churg-Strauss syndrome (CSS), microscopic polyangiitis(MPA), essential cryoglobulinemic vasculitis, cutaneous polyarteritis, and hypersensitivity vasculitis.

Front 30

hantavirus pulmonary syndrome (HPS)

Back 30

1) febrile illness with respiratory compromise
2) develops within 72 hours of hospitalization in a previously healthy person.
3) Characteristic noncardiogenic pulmonary edema and death

Front 31

First stage of Lyme disease:

Back 31

About 7 to 10 days (up to 36 days) after the bite, 60% to 80%of affected individuals develop
1) erythema migrans rash and 2) flulike symptoms (fever, arthralgias, and myalgias)

Front 32

Acrodynia (pink disease) 8 features

Back 32

hypersensitivity reaction to mercury that produces paresthesias, generalized pain, anorexia, irritable apathy, diaphoresis, hypertension, and tachycardia, accompanied by an acral rash

Front 33

side-effects SSRI

Back 33

Pulmonary HTN, W/D Sz

Front 34

Scurvy four H's:

Back 34

hemorrhage, hyperkeratosis, hypochondriasis, and hematological abnormalities.

Front 35

most common cANCA vasculitis

Back 35

Wegener's granulomatosis

Front 36

acetone-positive urine drug screen could be associated with...

Back 36

huffing

Front 37

posterior reversible encephalopathy syndrome PRES (3 features)

Back 37

syndrome characterized by
1) hypertension,
2) neurological findings such as headach altered alertness or behavior
3) neurological imaging changes

Front 38

DDX galactorrhea

Back 38

1) pregnancy or a terminated pregnancy
2) central nervous system (CNS) disorder such as pseudotumor cerebri, tumors, or neurocutaneous disorders
3) certain medications such as oral contraceptives, opiates, tetrahydrocannabinol, atypical antipsychotics, or amphetamines

Front 39

Pregnancy category SSRIs

Back 39

C

Front 40

ABVD chemotherapy (Hodgkins)

Back 40

adriamycin, bleomycin, vinblastine, and dacarbazine

Front 41

Rx Lyme disease

Back 41

amoxicillin, cefuroxime,or doxycycline

Front 42

AED with lowest incidence of teratogens

Back 42

carbamazepine was associated with the lowest rate of major congenital malformations: 2.2 percent of 900 pregnancies

Front 43

Lyme disease pathogen

Back 43

Borrelia burgdorferi

Front 44

Medial epicondylar apophysitis

Back 44

“Little Leaguer’s elbow”

Front 45

Sx Mercury poisining

Back 45

1) include tender and inflamed gums 2) Increased Salivation
3) Psychiatric changes

Front 46

presentation posterior urethral valves

Back 46

diurnal enuresis,urinary tract infection, and voiding difficulty.

Front 47

Presentation Guillan-Barre

Back 47

Gait abnormality, ataxia disproportionate to weakness, decreased DTRs

Front 48

Deficiencies in PEM

Back 48

B6 and B12, niacin,riboflavin, thiamine, zinc, and many fatty acids.

Front 49

Rx WG

Back 49

cyclophosphamide and steroids

Front 50

Functions of Vit C

Back 50

1) Cofactor in the hydroxylation of proline and lysine in procollagen.
2) hydroxylation of dopamine to norepinephrine and tryptophan to 5-hydroxytryptophan,
3) Cofactor in the metabolism of tyrosine, folic acid, histamine, and carnitine.

Front 51

Dx DIC

Back 51

Dx based on the constellation of thrombocytopenia, prolonged PT and aPTT, and elevated concentrations of fibrin split products. elevated FSP alone has a sensitivity of 90% to 100%

Front 52

AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE 

Back 52

microcysts 1 to 2 mm in diameter liver may be involved

Front 53

Birth defects phenobarbital

Back 53

heart, orofacial, and urogenital structures

Front 54

Rx OTC deficiency

Back 54

1) IV high-volume glucose, sodium benzoate, and sodium phenylacetate 2) Long-term management includes sodium phenylbutyrate and citrulline

Front 55

# Cases Lyme disease/year in US

Back 55

20,000

Front 56

lamotrigine birth defects

Back 56

cleft palate and/or cleft lip in infants exposed in the first trimester, of 8.9 per 1000 compared with the expected 0.7 to 2.5 per 1000

Front 57

most common lesion found to cause primary glomerulonephritis

Back 57

IgA nephropathy

Front 58

Causes of opisthotonos in the newborn

Back 58

intracerebral or intraventricular hemorrhage (from birth trauma, bleeding diathesis, arteriovenous malformations, tumors), kernicterus,tuberous sclerosis, hydrocephalus,neonatal tetanus, and Dandy-Walker

Front 59

hyperexplexia

Back 59

generalized muscular rigidity, hypertonia in flexion,and an exaggerated startle response, in the absence of other identified disorders,

Front 60

angiofibroma of nose

Back 60

"prolonged ""sinusitis"" epistaxis almost exclusively in
almost exclusively in
pubertal males and is an androgen hormone-dependent tumor.

Front 61

commonest cause of dilated cardiomyopathy

Back 61

neuromuscular disease (40%)

Front 62

commonest causes of viral myocarditis

Back 62

coxsackie and enteroviruses

Front 63

the most common helminthic infection in the United States

Back 63

Enterobiasis (pinworms)

Front 64

management CHF

Back 64

digoxin, diuretics, ACE inhibitors

Front 65

characteristic pain in pancreatitis

Back 65

worse supine

Front 66

cefadroxil

Back 66

1st gen

Front 67

rx sepsis

Back 67

vancomycin and piperacillin or meropenem and tobramycin

Front 68

ecthyma gangrenosum

Back 68

black ulceration=pseudomonas vasculitis

Front 69

rx "warm shock"

Back 69

fluids and vasopressors

Front 70

rx "cold shock"

Back 70

fluids and inotropes

Front 71

cause HTN in preadolescent

Back 71

70-80% renal dz, coarc, endocrinopathy, tumors

Front 72

Multiple Sleep Latency Test (MSLT)

Back 72

quantifies daytime sleepiness for dx narcolepsy

Front 73

Sx Narcolepsy (4)

Back 73

1)daytime sleepiness
2)cataplexy (drop attacks)
3) hypnagogic hallucinations 4)sleep paralysis

Front 74

"Drop Attacks"

Back 74

narcolepsy

Front 75

dx narcolepsy

Back 75

PSG and MSLT

Front 76

rx epiglottitis

Back 76

1) intubation 1)antistaph plus 3rd gen cephalosporin

Front 77

FMF (4)

Back 77

fever, myalgias, abd pain, diarrhea/constipation

Front 78

pathophysiology of FMF

Back 78

activation of PMNs on mucosal surfaces-MEFV gene on chromosome 16

Front 79

(TNF) receptor associated periodic syndrome (TRAPS)

Back 79

long, dramatic episodes of high fever; severe pain in the abdomen, chest, or joints; rash; inflammation in or around the eyes; and the developmentof amyloidosis.

Front 80

Diencephalic syndrome results from

Back 80

brain mass leads to FTT despite adequte caloric intake

Front 81

high on DDX renal stones

Back 81

hypercalcemia

Front 82

DDX Ansarca

Back 82

1) hepatic (cirrhosis, cystic fibrosis),
2) renal (nephrotic syndrome)3)gastrointestinal (protein losing enteropathy, allergic gastrointestinal reactions, gastroenteropathy).

Front 83

benign hypertrophic gastropathy of childhood.

Back 83

self-limited form of an adult disorder known as Menetrier disease hypertrophy of the mucus-secreting cells of the stomach

Front 84

stool alpha 1 antitrypsin

Back 84

sign of malabsorption

Front 85

pseudomembranous colitis (3 features)

Back 85

1) presence of toxin,
2) ongoing diarrhea and fever
3) in pt receiving abx

Front 86

IEM with acidosis

Back 86

urea cycle, aminoacidopathies, FA oxidation

Front 87

ingestion: ataxia and nystagmus

Back 87

1) alcohol
2) metals such as lead and lithium
3) anticonvulsants (phenytoin, phenobarbital,and carbamazepine).

Front 88

Marfans armspan:height

Back 88

>1.05

Front 89

Acute retinal necrosis (ARN)

Back 89

HSV or VZV

Front 90

Postseptal (orbital) cellulitis

Back 90

often complicated by ethmoid sinusitis

Front 91

Nasal gliomas (NG)

Back 91

also known as nasal cerebral heterotopias or glial heterotopias, are rare, benign masses of glial tissue located near the nasal glabella that may be extranasal (60%), intranasal (30%), or both (10%).1

Front 92

Zosyn (piperacillin and tazobactam).

Back 92

aspiration pneumonia

Front 93

Lemierre’s syndrome

Back 93

acute oropharygeal infection with a secondary thrombophlebitis of the internal jugular vein and metastatic septic emboli.

Front 94

macroglossia, microsomia, abdominal wall defects , ear creases or pits, renal abnormalities and hemihypertrophy

Back 94

BWS

Front 95

hypoglycemia,
hepatomegaly
lactic acidosis
G6p-tase def,

Back 95

GDS 1 (von Gierke)

Front 96

enthesitis

Back 96

inflammation where ligament and tendon meet bone (ankylosing spondylitis)

Front 97

microspherocytosis

Back 97

Ab-mediated hemolytic disease

Front 98

physiologic anemia

Back 98

8-12 weeks

Front 99

descending pattern of paralysis

Back 99

botulism

Front 100

ascending pattern of paralysis

Back 100

Guillen Barre syndrome

Front 101

Charcot-Marie-Tooth (3 features)

Back 101

1)peroneal muscular atrophy (PMA), control sensory information and muscle function of the foot/lower leg and hand/forearm.
2) slowly progressive, causing deterioration of peripheral nerves
3) AD inheritance

Front 102

Genetic defect in Marfan Syndrome

Back 102

fibrillin 1 gene on XS 15

Front 103

chalazion

Back 103

chronic lipogranuloma of Meibomian gland

Front 104

hordeolum

Back 104

infection (bacterial) of a sebaceous gland

Front 105

familial adenomatous polyposis (FAP)
6 features

Back 105

multiple colonic polyps, long bone osteomas,lipomas,cysts, dermoids, AD

Front 106

adenosine site of action

Back 106

AV node

Front 107

isotretinoin anomalies (4)

Back 107

CNS, ear, heart, thymus

Front 108

Best Abxs for meningitis (combo of 2)

Back 108

(cefotaxime or ceftriaxone) and vancomycin

Front 109

pathogens in viral meningitis (3)

Back 109

non-polio enteroviruses, herpes, arboviruses

Front 110

Witch Hazel

Back 110

An extract of the leaves and bark of the Hamamelis virginnia (an elm)

Front 111

Current earliest age of thelarche

Back 111

5-6 years AA, 6-7 Caucasian

Front 112

pathologic CNS causes of precocious puberty (4)

Back 112

hamartomas, hydrocephalus, arachnoid/ventricular cysts, post-inflammatory

Front 113

familial adenomatous polyposis (FAP)

Back 113

multiple colonic polyps,
long bone osteomas,
lipomas,cysts,
dermoids, AD

Front 114

adenosine site of action

Back 114

AV node

Front 115

isotretinoin anomalies

Back 115

CNS, ear, heart, thymus

Front 116

Best Abx for meningitis

Back 116

(cefotaxime or ceftriaxone) and vancomycin

Front 117

pathogens in viral meningitis

Back 117

non-polio enteroviruses, herpes, arboviruses

Front 118

Witch Hazel

Back 118

An extract of the leaves and bark of the Hamamelis virginnia (an elm)

Front 119

Current earliest age of thelarche

Back 119

5-6 years AA, 6-7 Caucasian

Front 120

CNS causes of precocious puberty

Back 120

hamartomas, hydrocephalus, arachnoid/ventrivcular cysts, post-inflammatory

Front 121

abnormal urinary protein/creatinin ratio

Back 121

>.2 on first voided specimen

Front 122

Chiari crisis (4 features)

Back 122

hoarseness, stridor, apnea, sudden death

Front 123

Spina-bifida occulta

Back 123

abnormal sacral reflexes, absent wink

Front 124

Marfan Dx

Back 124

2 major
Pectus excavatum that requires surgery or pectus carinatum



Pectus excavatum of moderate severity.
Pectus excavatum of moderate severity
Reduced upper-to-lower body segment ratio (0.85 vs 0.93) or arm span–to-height ratio greater than 1.05:
Positive wrist (Walker)
Positive thumb (Steinberg) sign.

Scoliosis greater than 20°
Reduced extension of the elbows
Protrusion acetabulum


1 minor
Pectus excavatum of moderate severity
Scoliosis less than 20°
Thoracic lordosis
Joint hypermobility

Front 125

Marfan arm span

Back 125

arm/height>1.05

Front 126

Incidence of no FH in Marfan Syndrome

Back 126

1/3

Front 127

neonate with depressed sensorium and AG

Back 127

measure NH4

Front 128

Greatest risk factor for CP

Back 128

prenatal infection

Front 129

scybalous

Back 129

pebble-like (stools)

Front 130

osteopetrosis

Back 130

1. Recurrent fractures with
brittle bones. 2. Failure to thrive with macrocephaly,
gingival hypertrophy, and
neurologic complications such as blindness and deafness caused by nerve entrapment.
3. Bone marrow failure caused by absence of the bone marrow cavity leading to anemia, thrombocytopenia,
extramedullary hematopoiesis,
and hepatosplenomegaly,
4. Impaired immune function
leading to recurrent infections andchronic rhinitis in conjunction with the frequently observed choanal stenosis.
Leukocytosis, usually present
in the early disease stage, can revertto leukocytopenia later.
5. Hypocalcemia sometimes resulting in tetanic seizures and secondary hyperparathyroidism.
6. Abnormal craniofacial appearance.
Most patients become blind or
anemic before 6 months of age

Front 131

histiocytosis X=LCH (3 diseases)

Back 131

1. solitary and indolent eosinophilic granuloma
2. Hand-Schüller-Christian 3.disseminated fulminant Letterer-Siwe disease.

Front 132

histiocytosis X,

Back 132

a spectrum of one disease caused by clonal proliferation of Langerhans cells, the antigen presenting cells of the epidermis (CD1a-positive cells) Electron microscopy reveals cytoplasmic Birbeck granules.

Front 133

DDX Papulopustular dermatoses (7)

Back 133

LCH, Seborrheic dermatitis, Erythema toxicum neonatorum, Atopic dermatitis, Mastocytosis, Wiskott-Aldrich syndrome Eosinophilic pustular folliculitis

Front 134

Wiskott-Aldrich syndrome

Back 134

eczema-thrombocytopenia-immunodeficiency syndrome

Front 135

% of U.S. adolescents eligible for LDL-lowering treatment

Back 135

Less than 1%

Front 136

6 Ps of Lichen Planus

Back 136

planar, polygonal, purple, pruritic. papules and plaques.

Front 137

Churg-Strauss Syndrome

Back 137

(allergic granulomatosis) medium and small vessel autoimmune vasculitis, leading to necrosis.
Mainly lungs (it begins as a severe type of asthma) gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys

Front 138

The most common neoplasm that presents with back pain in children is:

Back 138

osteoid osteoma

Front 139

Other tumors that can present in the spine

Back 139

leukemia, lymphoma, Ewing sarcoma, neuroblastoma, osteoblastoma, osteosarcoma, neurofibroma, and Langerhans cell histiocytosis (eosinophilic granuloma).

Front 140

Goldenhar Syndrome

Back 140

anomalies of the ear, hemifacial microsomia, and vertebral defects. Associated findings include anomalies of the eye, brain, and developmental delay.

Front 141

TRAPS

Back 141

Focal myalgias, sometimes migratory
Conjunctivitis
Periorbital edema
Abdominal pain
Monoarticular arthritis
Rash

The diagnosis of TRAPS is confirmed by genetic testing for common mutations in the TNFR1 gene. Testing is available commercially

Front 142

Chancroid

Back 142

Hemophilus ducreyi is a highly infectious bacterium.

"school of fish" appearance

The classic genital presentation of chancroid is with a deep, undermined, purulent ulcer that may be associated with painful inguinal lymphadenitis.
special culture media that is not widely available

PCR, may lead to improvements in the diagnosis of chancroid

erythromycin, trimethoprim-sulfamethoxazole, ciprofloxacin, ceftriaxone, and azithromycin

Front 143

Granuloma inguinale

Back 143

(donovanosis) is Klebsiella granulomatis,

cannot be cultured. Diagnosis requires visualization of dark-staining Donovan bodies on tissue crush preparation or biopsy.
Tetracycline or doxycycline, Sulfamethoxazole, Gentamicin,
Streptomycin, Ciprofloxacin, and Erythromycin.

Front 144

LGV

Back 144

L1, L2, and L3 serovars of Chlamydia trachomatis

Azithromycin

Front 145

Vomitting 2/2 fried rice left at room temperature

Back 145

B. cereus

Front 146

20% of patients with meningococcal disease have

Back 146

complement deficiency

Front 147

becteremic periorbital cellulitis 2/2:

Back 147

pneumococcus

Front 148

contacts of persons exposed to invasive strains of H. influenza should receive:

Back 148

rifampin

Front 149

tx plague

Back 149

TCN or quinolones

Front 150

chitterlingd

Back 150

Y. enterocolitica-pseudoappendicitis

Front 151

pneumonia and delirium

Back 151

Legionella
azithro or quinolones

Front 152

tx Francisella

Back 152

streptomycin, gentamycin, TCN

Front 153

rx Bartonella

Back 153

Azithromycin
rifampin

Front 154

Citrobacter

Back 154

look for brain abcess

Front 155

Q fever

Back 155

rickettsia- like
Coxiella burnetti
exposure to slaughter or products of conception TCN
doxycycline
chloramphenicol

Front 156

tick-bite and pancytopenia

Back 156

Ehrlichosis
doxycyclin/TCN

Front 157

cutaneous larva migrans-pathogens

Back 157

Strongyloides stecoralis
Ancyclostoma braziliensis
Ancyclostoma canium

Front 158

CLM rx

Back 158

albendazole or ivermectin

Front 159

DDX vaginal masses

Back 159

mucocolpos
urethral prolapse
prolapsed ectopic yreterocele
rhabdosarcoma
paraurethral cyst

Front 160

myosistis ossificans

Back 160

heterotropic calcification

Front 161

secretory diarrhea and anti-enerocyte antibodies

Back 161

autoimmune enteropathy

Front 162

loss of teeth with roots

Back 162

hypophosphatasia

Front 163

weight-gain, fever,. nl MRI

Back 163

Rapid Onset Obesity with Hypothalamic Dysfunction (ROHHAD)

Front 164

E Nodosum

Back 164

pertussis
GC
meningococcus
brucella
yersenia
salmonella
shigella Campylobacter
syphylis,
LGV
hepatitis
TB mycoplasma chlamydia.......

Front 165

Hill Sachs lesion

Back 165

results from impaction
of the posterolateral aspect
of the humeral epiphysis against theanterior glenoid rim with anterior
shoulder dislocation

Front 166

neonatal
adrenoleukodystrophy (NALD)

Back 166

mutation in one of several different
peroxisomal genes

Front 167

XALD

Back 167

mutation in the ABCD1 gene,
which causes a defect in peroxisomal
oxidation and accumulation
of long chain fatty acids

Front 168

Pleconaril

Back 168

anti-enteroviral activity, not on market

Front 169

Neonatal hemochromatosis

Back 169

is the most frequently
recognized cause of neonatal liver failure

Front 170

neonatal hemochromatosis

Back 170

an
alloimmune disease caused by an
autoantibody directed against a hepatocyte
surface protein. This leads
to marked structural damage

Front 171

metabolic causes liver failure in neonate

Back 171

tyrosinemia, galactosemia,
and hereditary fructose intolerance

Front 172

Lymphedema-distichiasis

Back 172

rare, autosomal, dominantly inherited
syndrome. Onset of lymphedema
usually starts at or just after puberty,

Front 173

lymphedema-distichiasis syndrome.
1

Back 173

subcutaneous and intramuscular
depot octreotide and a diet of MCT