Pediatrics Flashcards
Is gonnorhea on vag cx of a kid evidence for child abuse?
Is chlamydia?
Gonn - yes
Chlamydia - no. Could have gotten it at birth from mom, it can take up to 3 years to present
What type of shunt?
cyanotic congenital heart disease
acyanotic congenital heart disease
cyanotic = R-->L shunt acyanotic = L-->R shunt (VSDs, ASD's, PDA's)
What type of heart defect is associated with: Rubella? Turners? Kawasaki's? SLE? Lithium? maternal DM?
Rubella = PDA Turners = Coarctation of Aorta Kawasaki's = Coronary Artery aneurysms SLE = Congenital heart block Lithium = Ebsteins anomoly maternal DM = Asymmetric septal hypertrophy AND transposition of great vessels
What is the tx for a PDA?
When do you not treat?
Indomethacin (closes the PDA) - can use up to 6-8 months old
Keep open w/ PGE! Don’t close it in case of transposition of great vessels, coarctation of aorta, tetralogy of Fallot, hypoplastic left heart disease, or if indomethacin is contraindicated (intraventricular hemorrhage.)
Where does a coarctation of aorta normally occur>?
Just below the subclavian artery (causes HTN in upper extremities and normal BP below the coarctation
Interestingly, more than 2/3 these pts have a bicuspid aortic valve
What are the risk factors for transposition of great vessels?
WHat additional abnormalities must these kids have to stay alive?
DM and Digeorge Syndrome
Must have an ASD/VSD and a PDA
If kid turns blue after a few years vs few hours?
years = tetralogy of fallot hours = transposition of vessels
What are the risk factor for tetralogy of fallot?
DiGeorge and maternal PKU
Infant is asymptomatic until 4 months when he is diaphoretic with feeding and tachypnic
Dx?
Tx?
Tetrology of Fallot
CXR shows Boot Shaped Heart
Tx with PGE (like transposition of great vessels) but will ultimately need surgery.
Explain tet spells?
kids squat to increase vascular resistance so that they can breath with tet of fallot. (pulmonary stenosis, overriding aorta, RVH, VSD)
What 2 genetic abnormalities can cause down syndrome?
Other issues to worry about in DS?
#1 = meiotic nondisjunction #2 (4%) = robertsonian translocation
Duodenal atresia, Hirschprung’s disease, atrioventricular cushion defects, ALL, hypothyroidism, Alzheimers
What genetic abnormality do PKU kids have? Sx?
decreased phenylalanin hydroxylase or decreased THB cofactor.
After a few normal months, the kids present w. MR, fair skin, blonde, eczema, musty urine. Tx w/ tyrosine and low phenylalanine
Match disease w/ deficiency: Fabry's disease absent galactosylcermidase Gaucher's disease absent sphingomyelinase Tay-Sachs absent Arylsulfatase A Hurler's Absent iduronate sulfatase
Fabry’s disease = galactosidase A (telangiectasias, angiokeratomas)
absent galactosylcermidase = Krabbe’s disease (CNS issues)
Gaucher’s disease = Glucocerebrosidase (crinkled cells, TCP, anemia)
absent sphingomyelinase = Neimann-Pick (cherry spot and HSM)
Tay-Sachs = hexosidase (cherry spot, no HSM)
absent Arylsulfatase A = Metachromatic Leukodystrophy (ataxia)
Hurler’s = a-L-iduronidase (corneal cloud, MR, gargoyl)
Absent iduronate sulfatase = Hunter’s (no corneal cloud, X-linked)
Colicky pain in healthy kid. Flexed knees and vomiting. Current jelly stool w. sausage shaped RUQ mass. Dx? Risk factors? Tx?
Intussusception
Risks = lymphoma, LAD, H.u.S., Meckels Diverticulum
Tx: air contrast barium enema
What Abx can be a risk factor for pyloric stenosis?
What abnormal acid state is seen in PS?
Erythromycin
pt will have hypochloremic hypokalemic metabolic alkalosis (persistent emesis of HCl)
Healthy kid with sudden, intermittent painless rectal bleeding. Dx?
Meckel’s diverticulum, can lead to diverticulitis, volvulus, or intussuss.
FAILURE OF OMPHALOMESENTERIC/VITELLINE DUCT TO DISAPEAR.
Use the 99 scan to check for ectopic gastric tissue
What GI disease is associated with MEN type 2?
Hirschprungs Disease
Dx needs rectal bx (will show absence of myenteric (aurbachs) plexus and submucosal (meissners) plexus
What is the dx of choice for malrotation (kid shows up with bilious vomiting)
An EGD, although an XR will show a birds beak
Newborn with feeding intolerance that has intramural air bubbles in the GI?
Necrotizing Entericolitis (bubbles are made by bacteria)
Tx for: Brutons Agammaglobulinemia CVID IgA def DiGeorge
Brutons Agammaglobulinemia = IVIG (B’s are low, tonsils maybe gone)
CVID = IVIG (Low T’s and plasma’s. B’s normal)
IgA def = tx infections (no IVIG as it can cause anti-IgA Abs)
DiGeorge = BM transplants & IVIG (thymic aplasia()
Tx for:
Ataxia-Telangiectasia
SCID
Wiskott-Aldrich
Ataxia-Telangiectasia = no specific (DNA repair defect) SCID = BM transplant, PCP prophylaxis, IVIG (bubble boy) Wiskott-Aldrich = supportive, die by adults. (infections, purpura, eczema, low IgM, TCP)
Tx of: Chronic Granulomatous Disease Leukocyte Adhesion Deficiency Chediak Higashi Syndrome Job's Syndrome C1 esterase def (hereditary angioma) Terminal Complement Def (c5-c9)
-Chronic Granulomatous Disease = nitroblue test, tx: TMPSMX (cat+ infections)
-Leukocyte Adhesion Deficiency: BM transplant. (umbilical cord)
-Chediak Higashi Syndrome: BM Tx (occulo-albinism, neuropathy, neutropenia.)
Job’s Syndrome: Abx & IVIG (FATED - facies, abscesses, small teeth, hyper-IgE, dermatologic)
C1 esterase def (hereditary angioma): C1 esterase. (edema)
Terminal Complement Def (c5-c9): mening vaccine & Abx (recurrent N.Men infections.)
Kid with painless conjunctivitis and painful unilateral LAD with truncal rash and strawberry tongue w/ desquam of fingertips. Dx? Tx?
Kawasaki Disease
Tx = high dose ASA, IVIG
SCARLET FEVER LOOKS LIKE THIS TOO, BUT HAS NORMAL LIPS AND NO CONJUNCTIVITIS
WHAT will labs look like for Juvenile Idiopathic Arthritis?
Tx?
ANA+
RF-
Tx = NSAIDs and strengthening exercises
