[Cushing syndrome in children].
In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children.
Raux-Demay
M C
MC
Laboratoire d'Explorations Endocriniennes, Hôpital Trousseau, Paris.
Girard
F
F
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects; D000310:Adrenal Gland Neoplasms / Q000150:complications*; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002985:Clinical Protocols; D003131:Combined Modality Therapy; D003346:Corticotropin-Releasing Hormone; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D003907:Dexamethasone; D005260:Female; D006130:Growth Disorders / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008797:Metyrapone; D008939:Mitotane / Q000627:therapeutic use; D009347:Nelson Syndrome / Q000453:epidemiology / Q000209:etiology; D009765:Obesity / Q000209:etiology*; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology; D008083:beta-Lipotropin / Q000097:blood
D000305:Adrenal Cortex Hormones; D008939:Mitotane; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D008083:beta-Lipotropin; D008797:Metyrapone
Syndromes de Cushing de l'enfant.