[Bone and visceral manifestations of lipoatrophic diabetes. Apropos of a case].
Lipoatrophic diabetes, known by pediatricians as Lawrence-Seip disease or Berardinelli lipodystrophy syndrome, is an infrequent condition of which approximately one hundred cases have been published to date. A case in a 24-year-old female with a fifteen-year follow-up is reported. Manifestations included acanthosis nigricans, generalized lipoatrophy, hirsutism, muscle hypertrophy, and intellectual impairment. Biologic tests revealed insulin-resistant diabetes mellitus with major diet-dependent type V hypertriglyceridemia. The patient had nephrotic syndrome (focal and segmental endocapillary proliferative glomerulonephritis without dense deposits). Phosphorus and calcium determinations were normal, as were the endocrinologic tests. Roentgenograms of the bones disclosed increased density of axial bones and large epiphyseal defects with increased bone density as determined by osteodensitometric studies. The bone manifestations of this syndrome have been documented but are often overshadowed by the severe metabolic alterations.
de Wazieres
B
B
Service de Médecine Interne, Immunologie Clinique et Gériatrie, CHU Jean Minjoz, Besançon.
Wendling
D
D
Fest
T
T
Morin
G
G
Dupond
J L
JL
D000015:Abnormalities, Multiple; D000052:Acanthosis Nigricans / Q000150:complications; D000328:Adult; D001848:Bone Diseases, Developmental / Q000175:diagnosis / Q000209:etiology*; D003923:Diabetes Mellitus, Lipoatrophic / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006628:Hirsutism / Q000150:complications; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications; D015228:Hypertriglyceridemia / Q000150:complications; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008607:Intellectual Disability / Q000150:complications; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology*; D013577:Syndrome
D007334:Insulin-Like Growth Factor I
Manifestations osseuses et viscérales du diabète lipoatrophique. A propos d'un cas.