Does primary salt wasting occur in 11-beta-hydroxylase deficiency?
Renal salt wasting secondary to 11-beta-hydroxylase deficiency (11-beta-OHD) has been described in a few patients. This report describes an infant with 11-beta-OHD initially thought to be in adrenal crisis with renal salt wasting. Subsequently the sodium loss was found to be due to a secretory diarrhea, and this prompted us to critically review the literature of the previous case reports. In none has the entity of renal salt wasting secondary to 11-beta-OHD been unequivocally confirmed. The entity should remain in doubt until proven by appropriate studies in the future.
Singer-Granick
C
C
Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, PA 19134-1095, USA.
Hill
I D
ID
Rolston
R K
RK
D000312:Adrenal Hyperplasia, Congenital; D003681:Dehydration / Q000209:etiology / Q000628:therapy; D003967:Diarrhea / Q000150:complications; D005440:Fluid Therapy; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007010:Hyponatremia / Q000209:etiology* / Q000628:therapy; D007223:Infant; D007668:Kidney / Q000378:metabolism*; D008297:Male; D012964:Sodium / Q000378:metabolism*
D012964:Sodium; D006854:Hydrocortisone