%0 Case Reports %T Retinopathy and Uveitis in Congenital Generalized Lipodystrophy with Hypertriglyceridemia and Uncontrolled Diabetes (Berardinelli-Seip Syndrome). %A Rubaie KA %A Raef H %A Stone DU %A Kozak I %J Middle East Afr J Ophthalmol %V 26 %N 4 %D Oct-Dec 2019 %M 32153340 暂无%R 10.4103/meajo.MEAJO_94_19 %X Congenital lipodystrophy syndromes are characterized by a paucity of adipose tissue and are associated with metabolic abnormalities including insulin resistance, diabetes mellitus, and severe hypertriglyceridemia. Herein, we present a case of proliferative diabetic retinopathy with an attack of anterior uveitis in a young female with congenital generalized lipodystrophy - Berardinelli-Seip syndrome. To the best of our knowledge, this is the first description of ocular involvement in Berardinelli-Seip syndrome.