Long-term treatment of a child with congenital adrenal hyperplasia

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.

Download Full-text

A case of adrenocortical adenoma following long-term treatment in a patient with congenital adrenal hyperplasia

Korean Journal of Pediatrics ◽

10.3345/kjp.2007.50.3.302 ◽

2007 ◽

Vol 50 (3) ◽

pp. 302

Author(s):

Seung Rim Lho ◽

So Hyun Park ◽

Min Ho Jung ◽

Byung Churl Lee

Keyword(s):

Congenital Adrenal Hyperplasia ◽

Term Treatment ◽

Adrenocortical Adenoma ◽

Adrenal Hyperplasia ◽

Long Term Treatment

Download Full-text

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

Clinical Chemistry and Laboratory Medicine (CCLM) ◽

10.1515/cclm-2014-0375 ◽

2015 ◽

Vol 53 (3) ◽

Cited By ~ 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.

Download Full-text

INFECTIONS AND VIRILIZING ADRENAL HYPERPLASIA

PEDIATRICS ◽

10.1542/peds.25.4.563 ◽

1960 ◽

Vol 25 (4) ◽

pp. 563-564

Author(s):

MARY L. VOORHESS ◽

LYTT I. GARDNER

Keyword(s):

Infectious Diseases ◽

Replacement Therapy ◽

Adrenal Glands ◽

Term Treatment ◽

Adrenal Hyperplasia ◽

Long Term Treatment ◽

Children With Asthma

PHYSICIANS who treat children with virilizing adrenal hyperplasia frequently question the proper course of therapy that should be followed when these patients are exposed to infectious diseases. Misconceptions have developed because of a tendency to confuse these patients, who need adrenocorticosteroids only for replacement therapy, with patients having other syndromes who are receiving suppression therapy. Children with virilizing adrenal hyperplasia are given long-term treatment with physiologic amounts of adrenocorticosteroids to replace those steroids which their adrenal glands are unable to produce. It is hoped that their abnormally large adrenal glands can thus be reduced to a normal size. This is replacement therapy. Children with asthma, leukemia, nephrosis, etc., may be given large doses of steroids in an empiric effort to induce remissions in these diseases.

Download Full-text