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Subjects/Pediatrics/AIIMS 2019
AIIMS 2019
medium
smile Pediatrics

A 3 week neonate with ambiguous genitalia presented with Na+ 127 meq/L, K+ 7.2 meq/L wit BP 52/24 mm Hg and he was managed with IV fluids. What is the next step of management?

A. Spironolactone
B. Hydrocoisone administration
C. Broad spectrum antibiotics
D. Calcium gluconate

Explanation

Karyotype 46 XX Physical appearance - male pattern Precocious pubey at 2-4 yrs. of age. Uterus, cervix and fallopian tubes are present (bcz 44 XX) - Mullerian duct development is normal. Metabolic problems: Hyponatremia - aldosterone deficiency Hyperkalemia Hypotension Diagnosis: 1) USG Normal 2) Barr body present 3) Biochemistry - 17 hydroxyprogesterone || > 800 ngm/dl. Management: DOC - Dexamethasone - to pregnant women and fetus both having CAH. Hydrocoisone - pregnant women alone having CAH but fetus is normal. Neonate with CAH. Labioscrotal folds may be separated to folds may be fused at the midline, giving an appearance of a scrotum Labioscrotal folds with increased pigmentation suggest the possibility of increased coicotropin levels as pa of adrenogenital syndrome

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