title: Successful Treatment of an Unusual Case of FPLD2: The Role of Roux-en-Y Gastric Bypass-Case Report and Literature Review.
abstract: Familial partial lipodystrophy type 2 (FPLD2) is a rare disorder associated with LMNA gene mutations. It is usually marked by loss of subcutaneous fat on the limbs and trunk and severe insulin resistance. Scattered reports have indicated that Roux-en-Y bypass helps to control the diabetes mellitus in these patients. We present here a very unusual patient with FPLD2 who had life-threatening retroperitoneal and renal fat accumulation accompanied by bilateral renal cancers. Following cryotherapy of one renal cancer and a contralateral nephrectomy with debulking of the retroperitoneal fat, Roux-en-Y gastric bypass (RYGB) has successfully controlled the disease for 3 years. The clinical presentations and causes of FPLD are reviewed and the role of RYGB is discussed.
authors: Department of General Surgery, Digestive Disease Institute, Cleveland Clinic, Desk A100, Cleveland, OH, 44195, USA. grundfs@ccf.org.
authors: Sharon
authors: 0000-0001-8030-6138
authors: S
authors: Grundfest-Broniatowski
authors: Department of General Surgery, Digestive Disease Institute, Cleveland Clinic, Desk A100, Cleveland, OH, 44195, USA.
authors: JingLiang
authors: J
authors: Yan
authors: Department of General Surgery, Digestive Disease Institute, Cleveland Clinic, Desk A100, Cleveland, OH, 44195, USA.
authors: Matthew
authors: M
authors: Kroh
authors: Department of Endocrinology, Diabetes, and Metabolism, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors: Holly
authors: H
authors: Kilim
authors: Department of Urology, Glickman Urological Institute, Cleveland Clinic, Cleveland, OH, USA.
authors: Andrew
authors: A
authors: Stephenson
mesh_terms: D000713:Anastomosis, Roux-en-Y; D003920:Diabetes Mellitus / Q000209:etiology / Q000601:surgery*; D005260:Female; D015390:Gastric Bypass / Q000379:methods*; D006801:Humans; D050152:Intra-Abdominal Fat / Q000601:surgery*; D007680:Kidney Neoplasms / Q000150:complications / Q000628:therapy*; D052496:Lipodystrophy, Familial Partial / Q000150:complications / Q000601:surgery*; D008875:Middle Aged
keywords: LMNA mutation; Laminopathy; Partial familial lipodystrophy; Retroperitoneal mass; Roux-en-Y gastric bypass