Abstract
Tetrahydroaldosterone (3α, 5β) is excreted in urine as glucuronide and is the major known metabolite of aldosterone. Estimation of this parameter gives a valuable information on diurnal aldosterone production compared to a single estimation, of plasma aldosterone concentration.
Urine samples collected during hospitalization for steroid profiling from 25 untreated newborns suspected of and proven to have salt-wasting 21-hydroxylase deficiency (CAH) were analyzed for Tetrahydroaldosterone 3α, 5β -glucuronide (THALDO). The children were from 1 day to 4 months old, 12 were females, 13 males. The females were all virilized and the boys were either admitted to hospital due to salt-wasting crisis or sibship to children with 21-hydroxylase deficiency (3 cases). In the lowest age group with CAH 1 day - 8 days old, THALDO excretion was 0, 2-7 μg/day (mean 1,31 ± 1,8 μg, n=13) compared to 4-223 μg/day (mean 77 ± 72 μg, n=24) in normal newborns. In the CAH-group 14 days-1 month the excretion was 0,8-5 μg/day (mean 2,7 ± 1,3 pg, n=10) compared to 11-29 μg (mean 20 ± 8 μg, n= 6) in the controls. Two children with CAH, 2 and 4 month, old excreted 4 and 5 μg THALDO compared to 2-24 μg (mean 13 ± 9 μg, n=5) in normals.
In conclusion all 25 children with untreated salt-wasting 21-hydroxylase deficiency had very low aldosterone production irrespective of age. In the normal controls the wellknown increased activity in aldosterone production during the first week of life was demonstrated.
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Damkjær Nielsen, M., Petersen, K. 157 URINARY EXCRETION OF TETRAHYDRUALDOSTERONE IN NEW-BORNS WITH SALT-WASTING 21-HYDROXYLASE DEFICIENCY. Pediatr Res 24, 543 (1988). https://doi.org/10.1203/00006450-198810000-00178
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DOI: https://doi.org/10.1203/00006450-198810000-00178