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CAH/WH > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

WAGR syndrome

A

wilms’s tumor, aniridia, GU anomalies, mental retardation

PAX6+WT1

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2
Q

VACTERL abnormalities

A 
Vertebral defects
anal atresia
cardiacabnormalities
tracheoesophageal fistula
Renal anomalies
limb syndrome
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3
Q

Common variable immunodeficiency

A

antibody deficiencies in CVID may be as profound as in XLA but with normal numbers of circulating immunoglobulin-bearing B-lymphocytes

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4
Q

Henoch-Schonlein Purpura

A

1) petechial rash-buttocks, 2) arthritis, and 3) abdominal or renal complications

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5
Q

VSD

A

VSD holosystolic murmur next to sternum

think about fetal alcohol syndrome, TORCH, Down syndrome

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6
Q

ASD

A

fixed, split S2 and palpitation

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7
Q

Endocarditis prophylaxis

A

PDA, VSD, ASD, tetralogy except asymptomatic, sedundum type ASD.

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8
Q

duodenal atresia

A

DOWN Syndrome

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9
Q

MMR contraindicated

A

anaphylaxis to neomycin or gelatin, thrombocytopenia after first dose, recently immunoglobulin,

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10
Q

MMR not contraindicated

A

TB, breastfeeding, asymptomatic HIV infection, anaphylaxis to eggs

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11
Q

intussusception

A

Abdominal U/S

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12
Q

slipped capital femoral epiphysis

A

obese boys
limp and hip pain
avascular necrosis, femoral head and chondrolysis
immediate internal fixation with a single screw

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13
Q

DOWN syndrome

A 
endocardial cushion defect
duodenal atresia
hirschsprung's disease
hypothyroidism
Alzheimer's
antism, ADHD
acute leukemia
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14
Q

Sickle cell anemia:

A

Glutamic acid is changed into valine at 6th position of β chain
α2β2 is abnormal called Hb S, normal called Hb A

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15
Q

thalassemia

A

α problem, α-/– called Hb H disease (β4), –/– hydrops fetalis, Hb Barts(γ4)
β problem, minor and major, latter no β chain, a lot of α2γ2 (Hb F)

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16
Q

sick cell-beta zero thalassemia and sickle cell beta plus thalassemia

A

two beta chains, one is sickle, the other is thalassemia.

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17
Q

children constipation

A

magnesium hydroxide

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18
Q

turner syndrome

A

bicuspide aortic valve, coarotation of the aorta, horseshoe kidney

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19
Q

calcium supplementation

A

9-18yrs 1300mg
19-50yrs, 1000mg
51 and above 1200mg

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20
Q

developmental dysplasia of the hip

A

female, first born, breech position
barlow test positive
pavlik harness( frog leg positive)

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21
Q

unconjugated hyperbilirubinemia

A

Criggler-Najjar, Gilbert

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22
Q

conjugated hyperbilirubinemia

A

Rotor

Dubin-Johnson

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23
Q

IgA deficiency

A

anaphylaxis after Ig exposure

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24
Q

X-linked agammaglobulinemia

A

low or absent B cells and infections after 6 months

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25
Q

Digeorge syndrome

A

hypocalcemia and tetany in the first 24-48 h of life

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26
Q

severe combined immunodeficiency

A

adenosine deaminase deficiency
B and T cell defect
Thymus and lymph nodes absent

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27
Q

adenosine deaminase deficiency
B and T cell defect
Thymus and lymph nodes absent

A

eczema, thrombocytopenia, recurrent infection

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28
Q

chronic granulomatous disease

A

deficient nitroblue tetrazolium dye reduction by granulocytes

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29
Q

chediak-higashi syndrome

A

giant granules in neutrophils and oculocutaneous albinism

defect in microtubule polymerization

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30
Q

complement deficiencies

A

recurrent neisserial infection

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31
Q

chronic mucocutaneous candidiasis

A

associated with hypothyroidism

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32
Q

Hyper-IgE syndrome

A

recurrent staphy infection(skin), fair skin, red hair and eczema

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33
Q

streptococcus pyogens

A
  1. pharyngitis—rheumatic fever(heart) and glomerulonephritis
  2. scarlet fever—-glomerulonephritis
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34
Q

strabismus/amblyopia

A

correcting the associated amblyopia by covering the normal eye

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35
Q

child meningitis after antibiotics

A

check up for hearing loss

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36
Q

Neisseria meningitidis close contacts

A

antibiotic prophylaxis(rifampin, ceftriaxone or ciprofloxacin)

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37
Q

Roseloa infantum

A

human herpesvirus type 6

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38
Q

Erythema infectiosum (fifth disease)

A

slapped cheek rash, parvoviurs B19

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39
Q

kawasaki syndrome

A

1 fever> 5 days, 2 conjunctival infection, cervical lymph adenopathy, 3 watch for coronary artery aneurysms, 4 aspirin and IV immunolglobulin

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40
Q

sort throat, treated with amoxicillin —-rash

A

EBV infection

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41
Q

croup

A
  1. parainfluenza virus, 2 barking cough 3 humidified oxygen, racemic epinephrine
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42
Q

Epiglottitis

A

1 No cough; 2. thumb sign for X ray 3. treat with antibiotics(3rd)

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43
Q

RSV

A

1 diffuse hyperinflation of the lung for X ray 2. ribavarin treatment 3. palivizumab prophylaxis

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44
Q

Rubella pregnancy

A
  1. cardiovascular defects
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45
Q

CMV pregnancy

A

deafness, cerebral calcification

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46
Q

duchenne

A

Gower sign

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47
Q

neonatal conjunctivitis

A
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48
Q

osgood-schlatter disease

A
  1. osteochondritis of the tibial tubercle 2. rest , activity restriction , NSAID
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49
Q

ADHD

A

modafinil, methylphenidate, dextroamphetamine

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50
Q

respiratory distress syndrome comlications

A

intraventricular hemorrhage, pneumothorax/bronchopulmonary dysplasia

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51
Q

cryptorchidism

A

wait until 1 year, surgical intervention for fertility, no use for cancer risk-screen

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52
Q

potter syndrome

A

bilateral renal agenesis—oligohydramnio in utero

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53
Q

port wine

A

sturge-Weber syndrome, evaluate for glaucome, give anticonvulsive

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54
Q

defect in the irs

A

CHARGE syndrome: coloboma, heart defects, atresia of the nasal choanae, growth retardation, genitourinary, ear

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55
Q

Imphalocele

A

trisomy 13, 18, 21

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56
Q

Gastroschisis

A

right to midline without sac

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57
Q

umbilical hernia

A

congenital hypothyroidism

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58
Q

hypospadias

A

urethral opening on ventral surface, undescended testes, do not circumcise

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59
Q

epispadias

A

urinary exstrophy

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60
Q

respiratory distress newborn treatment

A

1 initial diagnosis: chest X ray, 2 initial treatment: Oxygen, nasal CPAP(if no work, check heart defect),

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61
Q

meconium ileus

A
  1. diagnostic testing: X-ray, 2 treatment: gastografin enema
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62
Q

Development at 9, 12, 15, 18, 24, 36, 48 months.

A

9 creeps adn crawls, 12 say one or more words, 15 walks alone, 18 walks donw stairs, says 10 words, 24 runs well, says 2-3 sentences, 36 rides tricycle, knows age and sex, 48 tells stories, participates in group play

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63
Q

Retention

A

disimpaction, stool softerner and behavior intervention,

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64
Q

Nonretention

A

Behaviour only

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65
Q

respiratory distress in the newborn

A

1 preterm: respiratory distress dyndrome 2 term, C-section transient tachypnea of the newborn, 3. term: Meconium aspiration

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66
Q

jaundice—indirect, next test?

A

Coombs, if positive, RH/ABO, if negative, check Hgb

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67
Q

breast feeding and milk jaundice

A

former: dehydration,needs frequent feeding, latter, temporaty cessation.

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68
Q

blue sclera

A

osterogenesis imperfecta

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69
Q

left upper quadrant anopsia, lower, with macular sparing

A

right temporal lobe, right parietal lobe, right occipital lobe

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70
Q

oculomotor

A

down and out

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71
Q

trochlear

A

cannot look donw when gaze is medial

72
Q

Hemolytic-uremic syndrome

A

uremia, thrombocytopenia and hemolytic anemia, treatment plasmapheresis

73
Q

sickle cell disease

A

aplastic crisis-blood transfusion, splenic sequestration crisis- splenectomy or hemolytic crisis.

74
Q

galactokinase deficiency, congenital rubella

A

cataracts

75
Q

SLE

A

false positive RPR test

76
Q

Hyper-IgM syndrome

A
  1. high IgM with deficiency of IgG, IgA 2 recurrent sinopulmonary infections and Pneumocystis carinii pneumonia
77
Q

Prolonged use of lithium in pregnancy

A

Ebstein’s anomaly (right heart defect)

78
Q

congenital HIV infection

A

needs PCR, viral culture and p24 antigen testing, not serology test

79
Q
A

midgut volvulus

80
Q

Acute ingestion of vitamin A

A

pseudotumor cerebri

81
Q

Iron poisoning

A

Deferoxamine

82
Q

heavy metals poisoning

A

Dimercapro

83
Q

Gaucher’s disease

A
  1. lack acid beta-glucosidase 2 jews 3 Erlenmeyer flask deformity of the distal femur
84
Q

Niemann-picks’ disease

A

1 lack sphingomyelinase 2 fatal in infant 2-3 years

85
Q

GM1 gangliosidosis(krabbe’s)

A
  1. lack beta-galactosidase 2 enlargement of the sella turcica
86
Q

GM2 gangliosidoses

A
  1. include Tay-Sachs’ disease 2 lack hexosaminidase 3 cherry-red spot in retina
87
Q

meconium ileus

A

1, cystic fibrosis transmembrane conductance regulator (CFTR) protein 2 a deletion of a three base pair encoding for phenylalanine(chro 7) (DÄ508)(70%)

88
Q

McCune-Albright syndrome

A

1 precocious puberty, cafe au lait spots and multiple bone defects 2 defect in the G-protein cAMP-kinase function

89
Q

Kartagener’s syndrome

A
  1. situs inversus, recurrent sinusitis, and bronchiectasis 2. heart-right chest
90
Q

respiratory distress newborn

A

1 most effective treatment: surfactant administration, 2 antenatal betamethasone, avoid prematurity and give tocolytics,

91
Q

respiratory distress newborn complications

A

retinopathy of prematurity, bronchopulmonary dsyplasia, intraventricular hemorrhage.

92
Q

chickenpox

A

rash within 24 hours

93
Q

Fanconi’s anemia

A

pancytopenia+hyperpigmentation on the trunk

94
Q

Diamond-Blackfan anemia

A
  1. congenital pure red cell aplasia in the first 3 months of life
  2. WBC and platelet counts are normal
95
Q

Guillain-Barré syndrome

A
  1. albumino-cytologic dissociation 2 plasmapheresis or human immunoglobulin
96
Q

cystic fibrosis

A

Hemophilus, Pseudomonas and Staphylococcus

97
Q

Cytomegalovirus, Mycobacterium avium intracellulare

A

CD4

98
Q

Pneumocystis carinii, Cryptococcus and Histoplasma pneumonia

A

CD4

99
Q

neuroblastoma, wilm’s tumor

A

former 3yrs

100
Q

Delayed separation of the umbilical cord (>3 weeks)

A
  1. leukocyte adhesion defect 2. recurrent bacterial infections and necrotic periodontal infection
101
Q

erythema migrans

A
  1. early-localized Lyme disease 2 9yrs, Doxycycline 4 both are contradictory, cefuroxime or erythromycin 5 3) Ceftriaxone or penicillin G for late stage
102
Q

Croup

A

Nebulized Racemic epinephrine and oral dexamethasone

103
Q

Diamond-Blackfan syndrome (DBS)

A

1 macrocytic anemia +congenital anomalies described (webbed neck) 2 no hypersegmentation

104
Q

Primary aortic coarctation and Recoarctation

A

surgery and balloon angioplasty.

105
Q

Henoch-Schonlein Purpura (HSP)

A
  1. intestinal intussusceptions, usually ileo-ileal. 2 ultrasonogram: doughnut sign 3. surgical reduction
106
Q

severe tricyclic antidepressant intoxication

A

seizure, hypotension, and QRS complex prolongation on ECG

107
Q

Parinaud’s syndrome

A
  1. paralysis of vertical gaze 2 poor pupillary reaction to light 3 rostral midbrain 4 germinomas and pinealomas
108
Q

deficient in Vitamin A

A

1 night blindness 2 follicular hyperkeratosis

109
Q

Vitamin B1 (thiamine) deficiency

A

1 In dry beriberi 2 wet beriberi

110
Q

Homocystinuria

A

1 cystathionine synthase deficiency, 2 lens dislocated to downwards (marfan is upwards) 3 marfan+mental+thromoembolic event vit B6 treatment

111
Q

Lateral epicondylitis (tennis elbow)

A

1 cystathionine synthase deficiency, 2 lens dislocated to downwards (marfan is upwards) 3 marfan+mental+thromoembolic event vit B6 treatment

112
Q

Von-Gierkes’ disease

A

1 glucose-6-phosphatase lack 2 Type I glycogen storage disease

113
Q

Pompes’ disease

A

1 Type II Glycogen storage disease 2 glycogen storage enzyme acid maltase lack 3 macroglossia

114
Q

Cori’s disease

A

1 Type III glycogen 2 glycogen debranching enzyme activity

115
Q

parotiditis (mumps)

A

Paramyxovirus

116
Q

inability of the patient to release the handshake

A

myotonic muscular dystrophy (MMD)

117
Q

Friedreich ataxia

A

1 unstable gait and speech difficulty 2 scoliosis 3 concentric hypertrophic cardiomyopathy 4 trinucleotide repeat

118
Q

phenylketonuria

A

1 urine has a mousy odor 2 phenylalanine hydroxylase lack 3 screen: blood phenylalanine levels 4

119
Q

Lesch-Nyhan syndrome

A

1 Self-mutilation 2 hypoxanthine-guanine phosphoribosyl transferase lack 3 a boy with gout

120
Q

Toxic epidermal necrolysis

A
  1. secondary to medication, such as sulfa drugs, anticonvulsants, and NSAID 2 a variant of Stevens-Johnson syndrome
121
Q

Neurofibromatosis

A

1 autosomal dominant 2 optic gliomas 3 iris hamartomas 4 ophthalmologic and neurologic evaluation

122
Q

Tuberous sclerosis

A

1 renal angiomyolipomas 2 cardiac rhabdomyosarcomas

123
Q

most common cause of meningitis in infants

A

Group-B streptococcus

124
Q

pathological jaundice

A
  1. 5 mg/dL/24 hours 2. >12 mg/dL in full-term or 10-14 mg/dL in preterm 3. direct >2 mg/dL
125
Q

Cataract

A
  1. TORCH, 2 galactosemia 3 Down’s syndrome, Turner’s syndrome 4 glucocorticoid use
126
Q

Trachoma

A

erythromycin or tetracycline

127
Q

systemic form of juvenile rheumatoid arthritis (Still’s disease)

A
  1. high-grade fever,maculopapular rashes, hepatosplenomegaly, lymphadenopathy, knee pain 2 aspirin and steroid
128
Q

absence epilepsy

A

Ethosuximide

129
Q

Heinz bodies

A

G6PD deficiency and thalassemia

130
Q

Helmet cells

A

HUS and TTP

131
Q

Necrotizing enterocolitis

A

X rays will reveal intramural air

132
Q

Bordetella pertussis infection

A

14-day erythromycin therapy

133
Q

hemophilia

A

factor VIII level, PTT longer

134
Q

21-hydroxylase deficiency

A

normal DHEA levels

135
Q

Cushing’s disease or Cushing’s syndrome

A

DHEA increase

136
Q

pertussis immunization

A

2, 4 and 6 months; one dose between 15 and 18 months; and a last one between 4 and 6 years.

137
Q

frequent acute painful episodes of sickle

A

Hydroxyurea-increases Hb F, Folic acid supplementation

138
Q

infant of diabetic mother

A

Transposition of great vessels, Duodenal atresia, Anencephaly and neural tube defects

139
Q

acute otitis media

A

Streptococcus pneumoniae

140
Q

Cyanosis, aggravated by feeding and relieved by crying

A
  1. choanal atresia 2 CT scan with intranasal contras 3 first management-placing an oral airway and lavage feeding
141
Q

communicating hydrocephalus

A

subarachnoid hemorrhag

142
Q

acute stroke syndrome

A

trauma–internal carotid artery dissection(10-24 h delay)

143
Q

Prader-Willi syndrome (PWS)

A

father original, q15 deletion

144
Q

cystic fibrosis infection treatment

A

ceftazidime (or a penicillin derivative, like ticarcillin) and an aminoglycoside (amikacin or gentamycin).

145
Q

Impetigo

A

1 associated with post streptococcal glomerulonephritis 2 Topical mupirocin 3

146
Q

asthma+allergic rhinitis and eczema

A

mast cell stabilizers -Sodium cromolyn

147
Q

congenital hypothyroidism

A

thyroid dysgenesis, T4 and TSH, big tongue

148
Q

congenital toxoplasmosis

A

chorioretinitis, hydrocephalus, and intracranial calcifications

149
Q

Congenital rubella syndrome

A

deafness, cataracts, and cardiac defects

150
Q

HUS

A
  1. thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure 2. prodrome of diarrhea
151
Q

TTP

A

thrombocytopenia, microangiopathic hemolytic anemia, neurologic disturbances, renal dysfunction and fever.

152
Q

Von Willebrand’s disease

A

1 bleeding time, PTT and Von Willebrand factor activity (ristocetin cofactor) abnormal 2 paltelet count normal

153
Q

congenital diaphragmatic hernia

A

first step is immediate placement of an orogastric tube and continuous suction

154
Q

a holosystolic murmur at the left, lower sternal border and a single S2

A

tricuspid atresia associated with ventricular septal defect, PGE1 to keep ductus arteriosus open to survival

155
Q

21-hydroxylase deficiency

A
  1. ACTH stimulation test 2 hyponatremia, hyperkalemia, hypoglycemia, and metabolic acidosis
156
Q

varicella (chickenpox)

A

1.contamination period: from before 48 hours to vesicles are crusted 2

157
Q

high-risk persons exposed to varicella

A
  1. immunocompromised, Newborn(mother -5 to +2) 2. hospital contact, contiunous household contact, playmate >1h
158
Q

infant botulism

A

gag reflex impaired

159
Q

transient synovitis

A

U/S

160
Q

cellulitis local anesthetis

A

no work because acid environment by bacterial

161
Q

cystic fibrosis

A

Tobramycin+ticarcillis-clavulanate

162
Q

infant hemolysis most common

A

G-6-PD

163
Q

croup

A

viral infection, oral corticosteroid

164
Q

rheumatic fever

A

synenham’s chorea

165
Q

cat bite

A

amoxicillin/clavulanica

166
Q

anorexia nervosa nutritional therapy 2-3w, heart failure

A

refeeding syndrome

167
Q

TB meningitis

A

Treat for 12 Months

168
Q

intussusception

A

U/S abdomen, doughnut and target sign

169
Q

RSV Bronchitis

A

rapid detection of antigen in nasal secretion, complication asthma

170
Q

bacterial pneumonia, outpatient,

A

Amoxicillin

171
Q

post transplant varicella vaccination

A

safe

172
Q

viral meningitis or encephalitis in pediatian

A

enterovirus an darborivus, animal vector in rural area

173
Q

adult meningitis

A

HSV

174
Q

Lead Poisoning

A

mild:70 EDTA+dimercaprol

175
Q

bat contact

A

antirabies IgG +5 doze of vaccine

CAH/WH (2 decks)

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