scholarly journals Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia

2015 ◽  
Vol 53 (3) ◽  
Author(s):  
Monique J.M. de Groot ◽  
Karijn J. Pijnenburg-Kleizen ◽  
Chris M.G. Thomas ◽  
Fred C.G.J. Sweep ◽  
Nike M.M.L. Stikkelbroeck ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Term Treatment ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Classic Congenital Adrenal Hyperplasia ◽  
Paediatric Patients ◽  
Long Term Treatment ◽  
Target Values ◽  
21 Hydroxylase Deficiency

AbstractTreatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency can be monitored by salivary androstenedione (A-dione) and 17α-hydroxyprogesterone (17OHP) levels. There are no objective criteria for setting relevant target values or data on changes of 17OHP and A-dione during monitoring.We evaluated A-dione and 17OHP levels in nearly 2000 salivary samples collected during long-term treatment of 84 paediatric patients with classic 21-hydroxylase deficiency.A-dione and 17OHP levels and its ratio 17OHP/A-dione remained constant from 4 to 11 years with no sex-related differences. During puberty, A-dione and 17OHP levels both increased, starting at earlier age in girls than in boys. The ratio 17OHP/A-dione declined. Normalised A-dione concomitant with elevated 17OHP [1.43 nmol/L (0.46–4.41) during prepuberty; 2.36 nmol/L (0.63–8.89) for boys and 1.99 nmol/L (0.32–6.98) for girls during puberty] could be obtained with overall median glucocorticoid doses of 11–15 mg/mNormalised A-dione consistent with 17OHP three times URL during prepuberty and normalised A-dione consistent with 4–6 times URL during puberty could be obtained by moderate glucocorticoid dosages. A constant 17OHP/A-dione ratio during prepuberty suggested absence of adrenarche. During puberty, a higher percentage of samples met the criteria for undertreatment, especially of boys.

MASSIVE ADRENOCORTICAL ADENOMA FOLLOWING LONG-TERM TREATMENT OF 21-HYDROXYLASE DEFICIENCY

2002 ◽  
Vol 167 (3) ◽  
pp. 1390-1391 ◽  
Author(s):  
SAYURI TAKAHASHI ◽  
SHIGERU MINOWADA ◽  
KYOUICHI TOMITA ◽  
NORIYUKI KATUMATA ◽  
TOSHIAKI TANAKA ◽  
...  
Keyword(s):  
Term Treatment ◽  
Adrenocortical Adenoma ◽  
Hydroxylase Deficiency ◽  
Long Term Treatment ◽  
21 Hydroxylase Deficiency

scholarly journals Long-Term Health Outcomes of Korean Adults With Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

2021 ◽  
Vol 12 ◽  
Author(s):  
Seung Gyun Lim ◽  
Young Ah Lee ◽  
Han Na Jang ◽  
Sung Hye Kong ◽  
Chang Ho Ahn ◽  
...  
Keyword(s):  
Dehydroepiandrosterone Sulfate ◽  
Metabolic Risk ◽  
Menstrual Irregularity ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Classic Congenital Adrenal Hyperplasia ◽  
Increased Risk ◽  
21 Hydroxylase Deficiency ◽  
Age And Sex

There is a lack of studies regarding the long-term outcomes of Asian adults with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. We hypothesized that adults with CAH are at higher metabolic risk than their age-, and sex-matched controls. We further investigated the long-term health outcome-related factors in adults with CAH. We compared metabolic risk between adults with CAH (71 men, 93 women) and age-, and sex-matched controls (190 men, 261 women) from the Korean National Health and Nutrition Examination Survey data. The presence of obesity, testicular adrenal rest tumors (TARTs), and menstrual irregularity was assessed. Hormone status and treatment regimens were compared according to the presence of adverse outcomes. The median age was 27.0 y and 28.0 y for men and women, respectively. Adults with CAH had a higher waist circumference (88.0 vs. 82.3 cm in men, and 83.5 vs. 72.3 cm in women), and blood pressure (125.0 vs. 113.0 mmHg in men, and 120.0 vs. 104.0 mmHg in women) than age- and sex-matched controls (P<0.05 for all). The 2.7-fold increased risk for hypertension (men) and 2.0-fold increased risk for obesity (women) was significant in patients with CAH (P<0.05 for both). Obese adults with CAH showed significantly higher adrenal limb thicknesses (men) and 17-hydroxyprogesterone and dehydroepiandrosterone sulfate levels (women) (P<0.05 for both). TARTs occurred in 58.1% of men and did not differ by hormone or treatment regimen. Irregular menstruation was observed in 57.1% of women, with higher dehydroepiandrosterone sulfate levels in those with irregular periods. Adults with CAH had a higher metabolic risk than the general population. Poor disease control may increase their risk of metabolic morbidity and menstrual irregularity.

LONG-TERM TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA WITH CORTISOL AND PREDNISOLONE ACETATE IN AQUEOUS SUSPENSION

1962 ◽  
Vol 39 (3) ◽  
pp. 323-327 ◽  
Author(s):  
Bernardo Léo Wajchenberg ◽  
J. Schnaider ◽  
R. D. Federico ◽  
A. Gelman ◽  
Virgílio G. Pereira ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Linear Growth ◽  
Aqueous Suspension ◽  
Term Treatment ◽  
Prednisolone Acetate ◽  
Adrenal Hyperplasia ◽  
Bone Maturation ◽  
Intramuscular Injections ◽  
Long Term Treatment

ABSTRACT Treatment of congenital adrenal hyperplasia with cortisol or prednisolone acetate, by infrequent intramuscular injections, is quite adequate as shown by arrest of precocious bone maturation and excessive linear growth, despite the intermittent adrenal depression as shown by the 17-ketosteroid levels.

Cardiac function in paediatric patients with congenital adrenal hyperplasia due to 21 hydroxylase deficiency

2018 ◽  
Vol 88 (3) ◽  
pp. 364-371 ◽  
Author(s):  
Christiaan F. Mooij ◽  
Milanthy S. Pourier ◽  
Gert Weijers ◽  
Chris L. de Korte ◽  
Zina Fejzic ◽  
...  
Keyword(s):  
Cardiac Function ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Paediatric Patients ◽  
21 Hydroxylase Deficiency
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