Response to treatment of congenital adrenal hyperplasia in infancy
Article Abstract:
Adrenal hyperplasia is the enlargement of the adrenal gland, which results in abnormal levels of important adrenal hormones. The standard method of treatment for congenital (present at birth) adrenal hyperplasia involves giving high initial doses of glucocorticoids, followed by lower maintenance doses, but some clinicians believe this regimen leads to overtreatment and reduced adult height. This study was designed to follow plasma levels of hormones and the long-term growth of infants with congenital adrenal hyperplasia, who were receiving low doses of replacement hormones, specifically hydrocortisone, without first being given a high initial dose. Blood samples were collected and growth patterns were charted every three months up to age three years for five boys and four girls. The plasma concentration of adrenal steroid hormones was greater than normal levels at birth (indicative of adrenal hyperplasia), but decreased to the normal range by three months of age. The results of this study show that biochemical control and appropriate growth rates can be established in infants with congenital adrenal hyperplasia using low replacement doses of hormone from the start of therapy, in contrast to the standard high initial doses followed by maintenance doses. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
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Normal postnatal androgen production and action in isolated micropenis and isolated hypospadias
Article Abstract:
The development of male sex organs begins during pregnancy and is influenced by genetic and hormonal factors. The development of the penis is controlled by testosterone, a male hormone, and its metabolic product, 5-alpha-dihydrotestosterone. Micropenis, or a very small penis, and hypospadias, a defect in the opening to the urethra, may result from defects in the production of androgens, male hormones that influence the development of male sexual characteristics. The effect of defects in androgen production or action on development of micropenis and hypospadias was assessed in 18 boys with micropenis and 19 boys with hypospadias. The binding of androgens to their receptors, which are cell membrane proteins that specifically attach androgens, was examined. In addition, the production of testosterone and gonadotrophins, which are hormones that stimulate the development of the sex glands in the embryo, were measured. The production of gonadotrophins and testosterone was normal in boys with micropenis, whereas androgen binding was normal in all subjects. Micropenis and hypospadias may result from transient abnormalities in the production and/or action of androgens during the development of the embryo. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1991
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Dexamethasone treatment for congenital adrenal hyperplasia
Article Abstract:
Congenital adrenal hyperplasia (CAD) is actually a group of diseases arising from defective biosynthesis of corticosteroids. The treatment of this condition usually requires the use of a glucocorticoid preparation in daily doses. In infancy and childhood, the probable drug of choice is hydrocortisone. In adults and adolescents who have stopped active growth, a satisfactory alternative medication is dexamethasone. Ten patients (three male and seven female, between ages 12 and 29) with the salt-losing form of CAD participated in a study to examine the effects of substitution of dexamethasone for their usual medication. Patient responses varied with the time of day the medication was taken and the strength of the medication. Dosage regulation was difficult to accomplish. No uniform dosage schedule could be assigned to all patients, as each patient has to have a personalized dosage schedule worked out to meet his or her specific need. It was found that a change to dexamethasone appears to offer an easier compliance option for each patient. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
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