Current Diagnosis and Treatment, and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Youth

Current Diagnosis and Treatment, and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Youth J Clin Res Pediatr Endocrinol. 2019 Aug 22;: Authors: Ozen S, Akinci B, Oral EA Abstract Lipodystrophy is a heterogeneous group of disorders characterized by lack of body fat, which can be genetic or acquired. Lipodystrophy is associated with insulin resistance that can develop in childhood and youth, and usually leads to severe metabolic complications. Diabetes mellitus, hypertriglyceridemia, and hepatic steatosis ordinarily develop in these patients, and most girls suffer from menstrual abnormalities. Severe complications develop at a relatively young age, which include episodes of acute pancreatitis, renal failure, cirrhosis, and complex cardiovascular diseases, and they all are associated with serious morbidity. Treatment of lipodystrophy consists of medical nutrition therapy, exercise, and the use of anti-hyperglycemic and lipid-lowering agents. New treatment modalities such as metreleptin replacement offer a great benefit in the treatment of metabolic abnormalities secondary to lipodystrophy. Current challenges in lipodystrophy management in children and youth include, but not limited to, (1) establishing specialized centers with experience in providing care for lipodystrophy presenting in childhood and adolescence; (2) optimizing algorithms that can provide some guidance for the use of standard and novel...
Source: JCRPE Journal of Clinical Research in Pediatric Endocrinology - Category: Endocrinology Tags: J Clin Res Pediatr Endocrinol Source Type: research