Insulin Resistance, Hyperleptinemia, and Obstructive Sleep Apnea in Launois‐Bensaude Syndrome

Abstract

HARSCH, IGOR A., SIMIN POUR SCHAHIN, FLORIAN S. FUCHS, ECKHART G. HAHN, TOBIAS LOHMANN, PETER C. KONTUREK, AND JOACHIM H. FICKER.Insulin resistance, hyperleptinemia, and obstructive sleep apnea in Launois-Bensaude Syndrome. Obes Res. 2002; 10:625-632. Objective: Launois-Bensaude Syndrome (LBS) is a very rare cause of obesity, characterized by a symmetrical accumulation of a very large number of lipomata in different regions of the body, excluding the face, the forearms, and the shanks. Obesity is known to be closely associated with insulin resistance, hyperleptinemia, and obstructive sleep apnea (OSA). We were interested in studying whether these conditions are also present in patients with obesity due to LBS with a similar frequency as in patients with "simple" truncal obesity. Research Methods and Procedures:We performed polysomnography and hyperinsulinemic euglycemic clamp studies and measured serum leptin in three patients with LBS and in six patients with "simple" truncal obesity, matched for sex and body mass index (LBS group, 36.39 kg/m 2 ; controls, 35.82 kg/m 2 ). Results: Polysomnography revealed severe OSA in one LBS patient with marked "horsecollar lipomata." In the other LBS patients, no OSA could be demonstrated. The leptin levels of the two groups were comparable (LBS group, 36.39 g/liter; controls, 37.18 g/liter) and the insulin responsiveness index was also comparable in the two groups (LBS group, 3.47 mol/kg ⅐ minute; controls, 3.79 mol/kg ⅐ minute). Discussion: Patients with LBS demonstrated similar metabolic features in terms of insulin sensitivity and hyperleptinemia as patients with "simple" truncal obesity. LBS is not strictly associated with OSA.

A case of obstructive sleep apnea syndrome: unusual comorbidity

“…The narrowing is not only explained by the posterior fatty infiltration, but also by a thickening of the muscular pharyngeal wall and a reduction of the lateral diameter of pharynx. These findings have been reported in some patients with OSAS without LBS (5). A metabolic syndrome is frequent in OSAS (6) as well as in LBS, and could be another link between these two syndromes.…”

Madelung's Disease in a Patient With Diabetes Mellitus

“…The lipomata show no tendency toward malignancy. 12 Weight loss and abstinence from alcohol are still recommended; however, they do not reverse or stop the progression of the disease once it is established. Therefore, it is not known whether the life expectancy of patients with Madelung's disease is normal or decreased.…”

Metabolische Besonderheiten bei Patienten mit Multipler Symmetrischer Lipomatose

“…Eine Insulinresistenz als eine Gemeinsamkeit mehrerer Facetten des MS fand sich allerdings bei den meisten von uns untersuchten Patienten. Diese hatten wir an einzelnen Patienten auch schon mit dem sensitiveren hyperinsulinämisch-euglykämischen Clampverfahren demonstriert [12].…”