Abstract
Long term observations and studies on two unrelated children with generalized lipodystrophy suggest a pituitary-endrocrine disorder with abnormal melanotropic growth-hormone-like secretion as the probable cause. Our patients have the advanced characteristics of generalized lipodystrophy which include loss of all body fat, skeletal and muscal overgrowth, hepatomegaly due to neutral fat infiltration, insulin resistant hyperglycemia, hyperlipemia, hyperpigmentation and greatly elevated levels of immunoreactive insulin.
We subjected a 13-year-old girl with generalized lipodystrophy to total surgical hypophysectomy. The pituitary gland was normal in size and appearance. subsequent histo-cytologic studies showed no anatomic abnormalities. Usual doses of endocrine explacement medications were sufficient.
Post-hypophysectomy, the patient rapidly improved. At three months, the following changes have occurred: (CLINICAL) 1) skin less pigemented, 2) acanthosis nigricans much improved, 3) liver about half former size, 4) beginning subcutaneous fat deposition. (LABORATORY) 1) serum no longer lipemic, with neutral fat levels returned to normal, 2) liver function tests returned to normal, 3) hyperinsulinism and insulin resistance less severe.
Our pre-hypophysectomy studies showed immunoreactive growth hormone levels to be low normal with no significan increase following intravenous insulin or arginine. These pre- and post-hypophysectomy findings suggest that the central problem involves the pituitary's secretion of an abnormal hormone with melanotrophic and growth hormone properties.
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Mabry, C., Hollingsworth, D. Generalized lipodystrophy (lipoatrophic diabetes): Evidence for abnormal pituitary function. Pediatr Res 5, 397–398 (1971). https://doi.org/10.1203/00006450-197108000-00111
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DOI: https://doi.org/10.1203/00006450-197108000-00111