[Seip-Lawrence syndrome associated with polyostotic fibrous dysplasia. Report of a case]. / Síndrome de Seip-Lawrence asociado a displasia fibrosa poliostótica. A propósito de un caso.

We report the case of a 28-year-old woman attending for hirsutism and diabetes mellitus. Diabetes was a casual finding 2 years before consulting and was treated with diet and antidiabetic drugs. Acromegalic appearance, facial acne, penty, curled and rude hair, hypertrichosis, ade I diffuse goitre, prominent abdomen with umbilical hernia, severe hepatomegaly, prominent muscles and veins with normal genitalia appeared in the physical examination. No other abnormalities were found. Hypophysis, thyroid, suprarenal and ovaric hormonal functional studies were normal. An insulin-resistant diabetes mellitus was found in the metabolic study. Ultrasound and TAC showed severe diffuse hepatomegaly and visceral fat lack. Bone radiographies showed diffuse lesions compatible with polyostotic dysplasia. Subcutaneous, hepatic and bone biopsy revealed lack of fat tissue, hepatic steatosis and osteal fibrosis. Patient s diagnosis was Berardinelli-Seip syndrome, Seip-Lawrence or lipoatrophic diabetes associated with polyostotic fibrotic dysplasia. Case is studies and bibliographic references are reviewed.

[Diabetic ketoacidosis as the first manifestation of acromegaly]. / Cetoacidosis diabética como primera manifestación de una acromegalia.

Diabetes mellitus is a frequent manifestation in patients with acromegaly and it can show up even before the latter manifests. Typically, the diabetes mellitus in these patients does not tend to develop ketosis. We present the case of a patient hospitalized due to diabetic ketoacidosis associated to a perianal abscess. Diabetes mellitus type I was rejected and the presence of insulin-resistance was verified. She required insulin therapy just for three months. The clinical, hormonal and radiological evaluation showed, three years latter, the presence of a hypophyseal tumor producing growth hormone. After performing a trans-sphenoidal adenomectomy, the insulin-therapy could be removed with a normal glycemic profile. This case of acromegaly demonstrates the relationship between growth hormone and glucose metabolism. It can be considered that the ketoacidosis was the first manifestation of the acromegaly, which is exceptional.