Adrenocorticotropic hormone unresponsiveness: Report of a girl with excessive growth and review of 16 reported cases
| Autor: | Maurice D. Kogut, Thomas F. Roe, Ann K. Kershnar |
|---|---|
| Rok vydání: | 1972 |
| Předmět: |
Adult
Male medicine.medical_specialty Obtundation Hydrocortisone Adrenocorticotropic hormone Hypoglycemia Arginine chemistry.chemical_compound Adrenocorticotropic Hormone Seizures Corticosterone Internal medicine Humans Insulin Medicine Child Desoxycorticosterone Aldosterone Growth Disorders business.industry Adrenal cortex Diet Sodium-Restricted medicine.disease Hyperpigmentation Glucose medicine.anatomical_structure Endocrinology chemistry Growth Hormone Pediatrics Perinatology and Child Health Vomiting Female medicine.symptom business Pigmentation Disorders Adrenal Insufficiency |
| Zdroj: | The Journal of Pediatrics. 80:610-619 |
| ISSN: | 0022-3476 |
| DOI: | 10.1016/s0022-3476(72)80058-1 |
| Popis: | A 4 9/12-year-old girl is described who developed vomiting, obtundation, convulsions, hypoglycemia, hyperpigmentation, and excessive growth after 11 months of age. She failed to elaborate cortisol following ACTH stimulation but could conserve salt and increase aldosterone production following salt deprivation. Secretory rates of desoxycorticosterone and corticosterone were normal on a normal salt diet. An explanation for her excessive growth was not evident. A sister, who had hypoglycemia and hyperpigmentation, died suddenly at age 2 1/2 years. The disorder is probably due to a failure in utero of the adrenal glands to respond to ACTH and to differentiate into the normal zones of the adrenal cortex. The clinical features of 16 reported pediatric cases are summarized. Evidence concerning the pathogenesis of this disease is reviewed. |
| Databáze: | OpenAIRE |
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