Paediatrics Flashcards
(175 cards)
1
Q
WAGR syndrome
A
wilms’s tumor, aniridia, GU anomalies, mental retardation
PAX6+WT1
2
Q
VACTERL abnormalities
A
Vertebral defects anal atresia cardiacabnormalities tracheoesophageal fistula Renal anomalies limb syndrome
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
4
Q
Henoch-Schonlein Purpura
A
1) petechial rash-buttocks, 2) arthritis, and 3) abdominal or renal complications
5
Q
VSD
A
VSD holosystolic murmur next to sternum
think about fetal alcohol syndrome, TORCH, Down syndrome
6
Q
ASD
A
fixed, split S2 and palpitation
7
Q
Endocarditis prophylaxis
A
PDA, VSD, ASD, tetralogy except asymptomatic, sedundum type ASD.
8
Q
duodenal atresia
A
DOWN Syndrome
9
Q
MMR contraindicated
A
anaphylaxis to neomycin or gelatin, thrombocytopenia after first dose, recently immunoglobulin,
10
Q
MMR not contraindicated
A
TB, breastfeeding, asymptomatic HIV infection, anaphylaxis to eggs
11
Q
intussusception
A
Abdominal U/S
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
13
Q
DOWN syndrome
A
endocardial cushion defect duodenal atresia hirschsprung's disease hypothyroidism Alzheimer's antism, ADHD acute leukemia
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
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)
16
Q
sick cell-beta zero thalassemia and sickle cell beta plus thalassemia
A
two beta chains, one is sickle, the other is thalassemia.
17
Q
children constipation
A
magnesium hydroxide
18
Q
turner syndrome
A
bicuspide aortic valve, coarotation of the aorta, horseshoe kidney
19
Q
calcium supplementation
A
9-18yrs 1300mg
19-50yrs, 1000mg
51 and above 1200mg
20
Q
developmental dysplasia of the hip
A
female, first born, breech position
barlow test positive
pavlik harness( frog leg positive)
21
Q
unconjugated hyperbilirubinemia
A
Criggler-Najjar, Gilbert
22
Q
conjugated hyperbilirubinemia
A
Rotor
Dubin-Johnson
23
Q
IgA deficiency
A
anaphylaxis after Ig exposure
24
Q
X-linked agammaglobulinemia
A
low or absent B cells and infections after 6 months
25
Digeorge syndrome
hypocalcemia and tetany in the first 24-48 h of life
26
severe combined immunodeficiency
adenosine deaminase deficiency
B and T cell defect
Thymus and lymph nodes absent
27
adenosine deaminase deficiency
B and T cell defect
Thymus and lymph nodes absent
eczema, thrombocytopenia, recurrent infection
28
chronic granulomatous disease
deficient nitroblue tetrazolium dye reduction by granulocytes
29
chediak-higashi syndrome
giant granules in neutrophils and oculocutaneous albinism
| defect in microtubule polymerization
30
complement deficiencies
recurrent neisserial infection
31
chronic mucocutaneous candidiasis
associated with hypothyroidism
32
Hyper-IgE syndrome
recurrent staphy infection(skin), fair skin, red hair and eczema
33
streptococcus pyogens
1. pharyngitis---rheumatic fever(heart) and glomerulonephritis
2. scarlet fever----glomerulonephritis
34
strabismus/amblyopia
correcting the associated amblyopia by covering the normal eye
35
child meningitis after antibiotics
check up for hearing loss
36
Neisseria meningitidis close contacts
antibiotic prophylaxis(rifampin, ceftriaxone or ciprofloxacin)
37
Roseloa infantum
human herpesvirus type 6
38
Erythema infectiosum (fifth disease)
slapped cheek rash, parvoviurs B19
39
kawasaki syndrome
1 fever> 5 days, 2 conjunctival infection, cervical lymph adenopathy, 3 watch for coronary artery aneurysms, 4 aspirin and IV immunolglobulin
40
sort throat, treated with amoxicillin ----rash
EBV infection
41
croup
1. parainfluenza virus, 2 barking cough 3 humidified oxygen, racemic epinephrine
42
Epiglottitis
1 No cough; 2. thumb sign for X ray 3. treat with antibiotics(3rd)
43
RSV
1 diffuse hyperinflation of the lung for X ray 2. ribavarin treatment 3. palivizumab prophylaxis
44
Rubella pregnancy
1. cardiovascular defects
45
CMV pregnancy
deafness, cerebral calcification
46
duchenne
Gower sign
47
neonatal conjunctivitis
48
osgood-schlatter disease
1. osteochondritis of the tibial tubercle 2. rest , activity restriction , NSAID
49
ADHD
modafinil, methylphenidate, dextroamphetamine
50
respiratory distress syndrome comlications
intraventricular hemorrhage, pneumothorax/bronchopulmonary dysplasia
51
cryptorchidism
wait until 1 year, surgical intervention for fertility, no use for cancer risk-screen
52
potter syndrome
bilateral renal agenesis---oligohydramnio in utero
53
port wine
sturge-Weber syndrome, evaluate for glaucome, give anticonvulsive
54
defect in the irs
CHARGE syndrome: coloboma, heart defects, atresia of the nasal choanae, growth retardation, genitourinary, ear
55
Imphalocele
trisomy 13, 18, 21
56
Gastroschisis
right to midline without sac
57
umbilical hernia
congenital hypothyroidism
58
hypospadias
urethral opening on ventral surface, undescended testes, do not circumcise
59
epispadias
urinary exstrophy
60
respiratory distress newborn treatment
1 initial diagnosis: chest X ray, 2 initial treatment: Oxygen, nasal CPAP(if no work, check heart defect),
61
meconium ileus
1. diagnostic testing: X-ray, 2 treatment: gastografin enema
62
Development at 9, 12, 15, 18, 24, 36, 48 months.
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
63
Retention
disimpaction, stool softerner and behavior intervention,
64
Nonretention
Behaviour only
65
respiratory distress in the newborn
1 preterm: respiratory distress dyndrome 2 term, C-section transient tachypnea of the newborn, 3. term: Meconium aspiration
66
jaundice---indirect, next test?
Coombs, if positive, RH/ABO, if negative, check Hgb
67
breast feeding and milk jaundice
former: dehydration,needs frequent feeding, latter, temporaty cessation.
68
blue sclera
osterogenesis imperfecta
69
left upper quadrant anopsia, lower, with macular sparing
right temporal lobe, right parietal lobe, right occipital lobe
70
oculomotor
down and out
71
trochlear
cannot look donw when gaze is medial
72
Hemolytic-uremic syndrome
uremia, thrombocytopenia and hemolytic anemia, treatment plasmapheresis
73
sickle cell disease
aplastic crisis-blood transfusion, splenic sequestration crisis- splenectomy or hemolytic crisis.
74
galactokinase deficiency, congenital rubella
cataracts
75
SLE
false positive RPR test
76
Hyper-IgM syndrome
1. high IgM with deficiency of IgG, IgA 2 recurrent sinopulmonary infections and Pneumocystis carinii pneumonia
77
Prolonged use of lithium in pregnancy
Ebstein's anomaly (right heart defect)
78
congenital HIV infection
needs PCR, viral culture and p24 antigen testing, not serology test
79
midgut volvulus
80
Acute ingestion of vitamin A
pseudotumor cerebri
81
Iron poisoning
Deferoxamine
82
heavy metals poisoning
Dimercapro
83
Gaucher's disease
1. lack acid beta-glucosidase 2 jews 3 Erlenmeyer flask deformity of the distal femur
84
Niemann-picks' disease
1 lack sphingomyelinase 2 fatal in infant 2-3 years
85
GM1 gangliosidosis(krabbe's)
1. lack beta-galactosidase 2 enlargement of the sella turcica
86
GM2 gangliosidoses
1. include Tay-Sachs' disease 2 lack hexosaminidase 3 cherry-red spot in retina
87
meconium ileus
1, cystic fibrosis transmembrane conductance regulator (CFTR) protein 2 a deletion of a three base pair encoding for phenylalanine(chro 7) (DÄ508)(70%)
88
McCune-Albright syndrome
1 precocious puberty, cafe au lait spots and multiple bone defects 2 defect in the G-protein cAMP-kinase function
89
Kartagener's syndrome
1. situs inversus, recurrent sinusitis, and bronchiectasis 2. heart-right chest
90
respiratory distress newborn
1 most effective treatment: surfactant administration, 2 antenatal betamethasone, avoid prematurity and give tocolytics,
91
respiratory distress newborn complications
retinopathy of prematurity, bronchopulmonary dsyplasia, intraventricular hemorrhage.
92
chickenpox
rash within 24 hours
93
Fanconi's anemia
pancytopenia+hyperpigmentation on the trunk
94
Diamond-Blackfan anemia
1. congenital pure red cell aplasia in the first 3 months of life
2. WBC and platelet counts are normal
95
Guillain-Barré syndrome
1. albumino-cytologic dissociation 2 plasmapheresis or human immunoglobulin
96
cystic fibrosis
Hemophilus, Pseudomonas and Staphylococcus
97
Cytomegalovirus, Mycobacterium avium intracellulare
CD4
98
Pneumocystis carinii, Cryptococcus and Histoplasma pneumonia
CD4
99
neuroblastoma, wilm's tumor
former 3yrs
100
Delayed separation of the umbilical cord (>3 weeks)
1. leukocyte adhesion defect 2. recurrent bacterial infections and necrotic periodontal infection
101
erythema migrans
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
Croup
Nebulized Racemic epinephrine and oral dexamethasone
103
Diamond-Blackfan syndrome (DBS)
1 macrocytic anemia +congenital anomalies described (webbed neck) 2 no hypersegmentation
104
Primary aortic coarctation and Recoarctation
surgery and balloon angioplasty.
105
Henoch-Schonlein Purpura (HSP)
1. intestinal intussusceptions, usually ileo-ileal. 2 ultrasonogram: doughnut sign 3. surgical reduction
106
severe tricyclic antidepressant intoxication
seizure, hypotension, and QRS complex prolongation on ECG
107
Parinaud's syndrome
1. paralysis of vertical gaze 2 poor pupillary reaction to light 3 rostral midbrain 4 germinomas and pinealomas
108
deficient in Vitamin A
1 night blindness 2 follicular hyperkeratosis
109
Vitamin B1 (thiamine) deficiency
1 In dry beriberi 2 wet beriberi
110
Homocystinuria
1 cystathionine synthase deficiency, 2 lens dislocated to downwards (marfan is upwards) 3 marfan+mental+thromoembolic event vit B6 treatment
111
Lateral epicondylitis (tennis elbow)
1 cystathionine synthase deficiency, 2 lens dislocated to downwards (marfan is upwards) 3 marfan+mental+thromoembolic event vit B6 treatment
112
Von-Gierkes' disease
1 glucose-6-phosphatase lack 2 Type I glycogen storage disease
113
Pompes' disease
1 Type II Glycogen storage disease 2 glycogen storage enzyme acid maltase lack 3 macroglossia
114
Cori's disease
1 Type III glycogen 2 glycogen debranching enzyme activity
115
parotiditis (mumps)
Paramyxovirus
116
inability of the patient to release the handshake
myotonic muscular dystrophy (MMD)
117
Friedreich ataxia
1 unstable gait and speech difficulty 2 scoliosis 3 concentric hypertrophic cardiomyopathy 4 trinucleotide repeat
118
phenylketonuria
1 urine has a mousy odor 2 phenylalanine hydroxylase lack 3 screen: blood phenylalanine levels 4
119
Lesch-Nyhan syndrome
1 Self-mutilation 2 hypoxanthine-guanine phosphoribosyl transferase lack 3 a boy with gout
120
Toxic epidermal necrolysis
1. secondary to medication, such as sulfa drugs, anticonvulsants, and NSAID 2 a variant of Stevens-Johnson syndrome
121
Neurofibromatosis
1 autosomal dominant 2 optic gliomas 3 iris hamartomas 4 ophthalmologic and neurologic evaluation
122
Tuberous sclerosis
1 renal angiomyolipomas 2 cardiac rhabdomyosarcomas
123
most common cause of meningitis in infants
Group-B streptococcus
124
pathological jaundice
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
Cataract
1. TORCH, 2 galactosemia 3 Down's syndrome, Turner's syndrome 4 glucocorticoid use
126
Trachoma
erythromycin or tetracycline
127
systemic form of juvenile rheumatoid arthritis (Still's disease)
1. high-grade fever,maculopapular rashes, hepatosplenomegaly, lymphadenopathy, knee pain 2 aspirin and steroid
128
absence epilepsy
Ethosuximide
129
Heinz bodies
G6PD deficiency and thalassemia
130
Helmet cells
HUS and TTP
131
Necrotizing enterocolitis
X rays will reveal intramural air
132
Bordetella pertussis infection
14-day erythromycin therapy
133
hemophilia
factor VIII level, PTT longer
134
21-hydroxylase deficiency
normal DHEA levels
135
Cushing's disease or Cushing's syndrome
DHEA increase
136
pertussis immunization
2, 4 and 6 months; one dose between 15 and 18 months; and a last one between 4 and 6 years.
137
frequent acute painful episodes of sickle
Hydroxyurea-increases Hb F, Folic acid supplementation
138
infant of diabetic mother
Transposition of great vessels, Duodenal atresia, Anencephaly and neural tube defects
139
acute otitis media
Streptococcus pneumoniae
140
Cyanosis, aggravated by feeding and relieved by crying
1. choanal atresia 2 CT scan with intranasal contras 3 first management-placing an oral airway and lavage feeding
141
communicating hydrocephalus
subarachnoid hemorrhag
142
acute stroke syndrome
trauma--internal carotid artery dissection(10-24 h delay)
143
Prader-Willi syndrome (PWS)
father original, q15 deletion
144
cystic fibrosis infection treatment
ceftazidime (or a penicillin derivative, like ticarcillin) and an aminoglycoside (amikacin or gentamycin).
145
Impetigo
1 associated with post streptococcal glomerulonephritis 2 Topical mupirocin 3
146
asthma+allergic rhinitis and eczema
mast cell stabilizers -Sodium cromolyn
147
congenital hypothyroidism
thyroid dysgenesis, T4 and TSH, big tongue
148
congenital toxoplasmosis
chorioretinitis, hydrocephalus, and intracranial calcifications
149
Congenital rubella syndrome
deafness, cataracts, and cardiac defects
150
HUS
1. thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure 2. prodrome of diarrhea
151
TTP
thrombocytopenia, microangiopathic hemolytic anemia, neurologic disturbances, renal dysfunction and fever.
152
Von Willebrand's disease
1 bleeding time, PTT and Von Willebrand factor activity (ristocetin cofactor) abnormal 2 paltelet count normal
153
congenital diaphragmatic hernia
first step is immediate placement of an orogastric tube and continuous suction
154
a holosystolic murmur at the left, lower sternal border and a single S2
tricuspid atresia associated with ventricular septal defect, PGE1 to keep ductus arteriosus open to survival
155
21-hydroxylase deficiency
1. ACTH stimulation test 2 hyponatremia, hyperkalemia, hypoglycemia, and metabolic acidosis
156
varicella (chickenpox)
1.contamination period: from before 48 hours to vesicles are crusted 2
157
high-risk persons exposed to varicella
1. immunocompromised, Newborn(mother -5 to +2) 2. hospital contact, contiunous household contact, playmate >1h
158
infant botulism
gag reflex impaired
159
transient synovitis
U/S
160
cellulitis local anesthetis
no work because acid environment by bacterial
161
cystic fibrosis
Tobramycin+ticarcillis-clavulanate
162
infant hemolysis most common
G-6-PD
163
croup
viral infection, oral corticosteroid
164
rheumatic fever
synenham's chorea
165
cat bite
amoxicillin/clavulanica
166
anorexia nervosa nutritional therapy 2-3w, heart failure
refeeding syndrome
167
TB meningitis
Treat for 12 Months
168
intussusception
U/S abdomen, doughnut and target sign
169
RSV Bronchitis
rapid detection of antigen in nasal secretion, complication asthma
170
bacterial pneumonia, outpatient,
Amoxicillin
171
post transplant varicella vaccination
safe
172
viral meningitis or encephalitis in pediatian
enterovirus an darborivus, animal vector in rural area
173
adult meningitis
HSV
174
Lead Poisoning
mild:70 EDTA+dimercaprol
175
bat contact
antirabies IgG +5 doze of vaccine
