Patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency can achieve their target height: the Leipzig experience.
BACKGROUND
Despite treatment, the mean final height (FH) of patients with classic congenital adrenal hyperplasia (CAH) is below the mean height of a normal population.


OBJECTIVE
To show that CAH patients can achieve their target height (TH), 39 adult subjects, whose therapy had started in infancy, were studied in a retrospective analysis. All height SDS were corrected so that they related to TH SDS.


METHODS
Group 1: patients born before 1975 (n = 13) had received prednisolone, at doses equivalent to hydrocortisone 39.4 +/- 15.6 mg/m2 BSA daily, together with DOCA in the first 2 years of life. Group 2: patients born from 1975 to 1986 (n = 26) received at this age lower hydrocortisone doses (16.4 +/- 6.9 mg/m2 BSA daily, divided 8 hourly; p < 0.001) combined with fludrocortisone, had outpatient visits every 3 months and bone age (BA) estimation every 6 months.


RESULTS
Patients of group 1 (FH SDS -1.2 +/- 1.0) had a poor outcome, whereas patients of group 2 (FH SDS 0.1 +/- 0.9; p = 0.01) achieved their TH.


CONCLUSIONS
Combined corticoid administration adjusted quarterly to keep height, BMI, blood pressure and BA within normal limits resulted in FH close to TH in patients with classic CAH.
Hoepffner
Wolfgang
W
University Children's Hospital, Leipzig, Germany. wolfgang.hoepffner@web.de
Kaufhold
Antje
A
Willgerodt
Helmut
H
Keller
Eberhard
E
D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000503:physiopathology; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D001827:Body Height / Q000187:drug effects*; D003900:Desoxycorticosterone / Q000008:administration & dosage*; D005260:Female; D005438:Fludrocortisone / Q000008:administration & dosage*; D005500:Follow-Up Studies; D005858:Germany; D006130:Growth Disorders / Q000188:drug therapy* / Q000201:enzymology / Q000503:physiopathology; D006785:Hospitals, University; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage*; D008297:Male; D008901:Mineralocorticoids / Q000008:administration & dosage*; D011239:Prednisolone / Q000008:administration & dosage*; D012189:Retrospective Studies; D013255:Steroid 21-Hydroxylase
D000893:Anti-Inflammatory Agents; D008901:Mineralocorticoids; D003900:Desoxycorticosterone; D011239:Prednisolone; D013255:Steroid 21-Hydroxylase; D005438:Fludrocortisone; D006854:Hydrocortisone