Transient pseudohypoaldosteronism with complex malformation of internal genitalia. A case report.
At the age of 3 weeks, a girl presenting with acute dehydration was admitted to our hospital. Clinical and laboratory findings revealed malformations of the genitourinary tract, an acute urinary infection and electrolyte disturbances (severe hyponatremia at 115 mmol/l and mild hyperkalemia at 5.6 mmol/l). According to anamnestic data, the child was born to healthy, nonconsanguineous parents. Vaccum extraction was done in the 38th gestational week due to pathological cardiotocography (CTG) findings. Auxological parameters were within the normal range for gestational age. Normal values for 17-OH progesterone and ACTH ruled out congenital adrenal hyperplasia (CAH). Pathologically high aldosterone and plasma renin activity (PRA) confirmed the diagnosis of pseudohypoaldosteronism with salt-wasting crisis which proved to be transient.
Iliev
D I
DI
Paediatric Endocrinology Section, University Children's Hospital, Tübingen, Germany.
Petruch
U R
UR
Ranke
M B
MB
Binder
G
G
Leriche
C
C
Strotbek
G
G
Wollmann
H A
HA
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000450:Aldosterone / Q000097:blood; D019072:Antibiotic Prophylaxis; D003681:Dehydration / Q000209:etiology; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D005865:Gestational Age; D006801:Humans; D007010:Hyponatremia / Q000209:etiology; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities; D010053:Ovary / Q000002:abnormalities; D011188:Potassium / Q000097:blood; D011546:Pseudohypoaldosteronism / Q000150:complications / Q000175:diagnosis*; D012083:Renin / Q000097:blood; D012964:Sodium / Q000097:blood; D014463:Ultrasonography; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities
D000450:Aldosterone; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D012964:Sodium; D012083:Renin; D011188:Potassium