pediatrics Flashcards
patient with spiral fracture what to rule out
child abuse
Left to right shunt 3 D
ASD
VSD
PDA
shunt in non cyanotic conditions
left to right
shunt in cyanotic conditions
right to left
Apert’s syndrome(2)
cranial deformities
fusion of fingers and toes
syndrome with VSD
Apert Down cri du chat trisomies 13 trisomies 18
auscultation of ASD
fixed split of s2
quid of eisenmenger syndrome(3)
left to right shunt causes
pulmonary hyper tension
shunt reversal
xray and EKG in ASD(2)
increase pulmonary vascular markings
right axis deviation
secondary HTA in child
coarctation of oarta
disease with systolic BP higher in the upper extremities greater in the right arm than in the left
coarctation of aorta
advanced cases of coarctation of aorta(2)
well developped upper body
lower extremity wasting
xray of coarctation of aorta(2)
3 sign
rib notching
what is life sustaining in transposition of the great vessels(2)
PDA
Septal defect
why PDA and septal defect allow in case of transposition of great vessels
allow mixing of pulmonary and systemic blood flow
could a baby survive with transposition of the great vessels without a PDA or septal defect
it’s incompatible with life
transposition of the great vessels
eggshaped silhouette
quid of eggshaped silhouette(2)
narrow heart base
absence of of the main pulmonary artery segment
right to left shunt causes 5T
tetralogy transposition of great vesels truncus arteriosus tricuspid atresia total pulmonary venous return
tetralogy of fallot PROVE
pulmonary stenosis
RVH
overridding aorta
VSD
the most common cyanotic heart disease in infancy
transposition
the most common cyanotic heart disease in childhood
tetralogy
rx of tetralogy and transposition
PGE1
disease with tetralogy of fallot(3)
down
cri du chat
trisomie 13 and 18
xray of tetralogy de fallot
bootshaped
DTAP(5)
2 mois 4 mois 6 mois 15-18 months 4-6 ans
HIB vaccination(4)
2 mois
4 mois
6 mois
12-15 months
IPV vaccination(4)(inactivated polio vaccine)
2 mois
4 mois
6 mois
4-6 ans
PPV vaccination(4)
2 mois
4 mois
6 mois
12-15 mois
MMR
12-15 months
4-6 ans
Varicella
12-15 months
HAV
2 ans
age to take influenza vaccine
6 months
enfant with evidence of neglect and severe malnourishment CAT
hospitalisation
management of pulmonary manifestation sin cystic fibrosis(5)
chest physical activity bronchodilators antiinflammatory agents antibiotics DNASE
digestive management of cystic fibrosis(2)
pancreatic enzymes
fat soluble vitamins ADEK
patau syndrome
trisomy 13
clue for turner
pas de corps de Barr
quid of fabry disease
@ glycosidase deficiency
consequence of @ glycosidase deficiency
ceramide trihexoside
finding of Fabry
renal failure
heritance of Fabry
x-linked recessive
quid of krabbe disease
absence of galactosylceramide B galactoside
consequence of Krabbe disease
galactocerebroside in the brain
symptom of Krabbe(3)
atrophy
spasticity
early death
inheritance of krabbe
autosomal recessive
quid of gaucher disease
deficiency of B glucocerebrosidase
consequence of gaucher(3)
accumulation of glucocerebroside in brain
liver and
bone marrow
gaucher cells
crinkled paper enlarged cytoplasm
type 1 of gaucher
incompatible with life
inheritance of gaucher
autosomal recessive
quid of Nieman pick disease(2)
Deficiency of sphingomyelinase
buildup of sphingomyelin
cholesterol in reticuloendothelial and parenchymal cells and tissues
prognosis of nieman pick
death by age of 3
inheritance of nieman pick
autosomal recessive
Tay-Sachs disease
Absence of hexosaminidase A
GM2 ganglioside accumulation
Tay-Sachs disease prognosis
death by age of 3
symptom of tay sachs
Cherry-red spot visible
on macula.
people at risk Tay sachs disease
Carrier rate is 1 in 30 in Jews of European
descent (1 in 300 for others).
metachromatic leukodystrophy
Deficiency of arylsulfatase A
accumulation of sulfatide in
the brain, kidney, liver, and peripheral nerves.
inheritance of metachromatic leukodystrophy
Autosomal recessive
target organ in metachromatic leukodystrophy(5)
accumulation of sulfatide in the brain kidney liver peripheral nerves.
quid Hurler’s syndrome
Deficiency of α-L-iduronidase
inheritance of hurler syndrome
Autosomal recessive.
symptom of hurler(2)
corneal clouding
mental retardation
quid Hunter’s syndrome
Hunter’s syndrome Deficiency of iduronate sulfatase
hunter’s syndrome symptom(2)
mild form of hurler without corneal clouding
mild mental retardation
transmission of hurler(2)
X-linked recesive
Hunters aim for the X.
quid of lysosomal storage disease(8)
Hurler hunter gaucher krabbe fabry nieman pick tay sachs Metachromatic leukodystrophy
most common cause of bowel obstruction in the first two years of life
intussuception
quid of Digeorge syndrome Catch 22
CATCH-22 Congenital heart disease Abnormal facies Thymic aplasia Cleft palate Hypocalcemia 22q deletion
B cell deficiency(3)
bruton(x linked agammaglobulinemia)
common variable immunodeficiency
Ig A deficiency
the most common immunodeficiency
IGA deficiency
common variable immunodeficiency clue
immunoglobulin levels drop at 20s et 30 s
infectious risk in brutons
pseudomonas
risk in common variable immunodeficiency
↑ pyogenic upper and lower respiratory infections
risk of lymphoma and autoimmune disease
t cell immunodefiency
digeorge syndrome
risk infectieux in digeorge(2)
PCP
fungi
rx of digeorge(2)
marrow transplantation
Intraveinous immunoglobulin
combined immunodeficiency(3)
Ataxia-telangiectasia
severe combined immunodeficiency
wiskott aldrich
symptom in ataxia telangiectasia(2)
Oculocutaneous telangiectasias
cerebellar ataxia.
physiopatho of ataxia telangiectasia
Caused by a DNA
repair defec
risk in ataxia telangiectasia(2)
non-Hodgkin’s
lymphoma
gastric carcinoma
Severe combined immunodeficiency
Severe lack of B and T cells
risk in severe combined immunodeficiency(2)
opportunistic organism infection
PCP infection
quid of wiskott aldrich(3)
eczema
low platelets
reccurent otitis media
immunoglobulin in wiskott aldrich(2)
↑↑ IgE/IgA,
↓↓ IgM
risk in wiskott aldrich(4)
atopic disorder
lymphoma
leukemia
infection
bugs causing infection in wiskott
S pneumoniea
S aureus
H influenza type B
Phagocytic problem causing immunodeficiency(2)
CGD
chediak higashi
inheritance of CGD
x linked
risk in CGD(3)
Chronic pulmonary, GI, and urinary infection
osteomyelitis
hepatitis
bugs causing infection in CGD
catalase + organism
dx test for CGD
Nitroblue tetrazolium test
problem in chediak higashi
defect in neutrophils chemotaxis
syndrome in chediak(4)
oculocutaneous
albinism,
neuropathy
neutropenia
bugs in chediak higashi(3)
S. pyogenes,
S. aureus,
Pseudomonas spp
complement deficiency(2)
C1 esterase deficiency( angioneurotic hereditary edema)
terminal complement deficiency (c5-c9)
inheritance of chediak
autosomal recessive
symtom in c1 esterase deficiency
recurrent episodes of angioedema in stress and trauma
inheritance of C1 esterase deficiency
autosomal dominant
complication of C1 esterase inhibitor
airway edema
dx of C1 esterase deficiency
total hemolytic complement( CH50)
prophylactic rx in c1 esterase deficiency
danazol
problem in terminal complement deficiency
inability to form membrane attack complex
consequence of terminal complement deficiency(4)
recurrent meningocal
gonoccocal infection
lupus
glomerulonephritis
Mesure a prendre in case of terminal complement deficiency
meningoccal vaccines
Kawasaki disease
symptoms—CRASH AND BURN”
Kawasaki disease symptoms— “CRASH AND BURN” Conjunctivitis Rash Adenopathy Strawberry tongue Hands and feet (red, swollen, flaky skin) AND Burn = fever > 40°C for ≥ 5 days
fisr cause of bronchiolitis
RSV
criteria to hospitalize brochiloitis(9)
marked respiratory distress, O2 saturation of < 92%, toxic appearance, dehydration/poor oral feeding, a history of prematurity (< 34 weeks), age < 3 months, underlying cardiopulmonary disease unreliable parents.
RSV prophylaxis
poly or monoclonal antibodies
example of poli or mono clonal antbodies(2)
respigam
synagis
first bug involved in croup
PIV 1(para influenza type 1)
bugs causing tracheitis
s aureus
xray in croup
“Steeple sign” on AP film
xray in epiglotitis
“Thumbprint sign” on lateral film
xray in tracheitis
Subglottic narrowing
indication of racemic epinephrine
croup
what to not do in epiglotitis and why
throat examination
laryngospasm and airway obstruction
bug in epiglotitis
h influenza
quid of direct hyperbilirubinemia
always pathologic in baby
quid of erythma infectyosum 5th disease(2)
slapped cheek
worse with fever and sun exposure
bug causing erythema infectyosum
parvovirus B19
bugs causing roseola infantum
HHV-6
rash in roseola infantum(3)
Maculopapular rash appears as fever breaks begins on the trunk and quickly spreads to the face and extremities last< 24 h
tear drop vesicules
varicella
bug causing hand fot and mouth disease
cocksakie A
rash in hand foot mouth syndrome
Oral ulcers
maculopapular vesicular rash
on hands feet and sometimes buttocks
associated defect with esophageal atresia(VACTERL)
VACTERL Vertebral cardiac anal tracheal esophageal renal limb
is gastrocisis emergent
surgical emergency
rule of 2 for meckel(4)
2 times as many males affected;
2 feet from the ileocecal valve (most common);
2 types of mucosa (gastric, pancreatic)
2% of people affected
failure to pass meconium in the first 24 hours of life
hirshprung disease
jaundice in neonate witn abnormal vital signs
workup for sepsis
Patients with complex febrile may require what?
chronic
anticonvulsant therapy.
Top childhood
cancers:(4)
- Leukemia (ALL)
- CNS tumors
- Lymphoma
- Neuroblastoma
association of wilms tumor(2)
aniridia
hemihypertrophy
