Thelansis

  Lipodystrophy Syndromes are a heterogeneous group of rare metabolic disorders of diverse etiology characterized by variable loss of body fat or selective deficiency of adipose tissue in the absence of nutritional deprivation or catabolic state. The partial or complete loss of body fat (lipoatrophy) may occur with a pathological accumulation of fat in other distinct body regions. Loss of fatty tissue frequently decreases leptin levels, which interferes with hunger-satiety signals and often leads to hyperphagia. Surplus calories are kept as fat in the liver and muscle tissue, resulting in various metabolic manifestations. The extent of adipose loss usually determines the severity of the associated clinical and metabolic manifestations. Expansion of spared adipose stores in partial lipodystrophies is one likely mechanism that leads to clinical and metabolic manifestations. It may affect nearly the entire body (generalized), only certain body regions (partial), or small areas under t...