1/5. Launois-Bensaude syndrome in a female with type 2 diabetes.BACKGROUND: The Launois-Bensaude syndrome (LBS) is a rare disease characterized by an accumulation of multiple lipomata with a preponderance at the thorax, abdomen, upper arms and the thighs. Frequently, the condition is associated with past or present alcohol abuse with no clear temporal coincidence between the onset and termination of lipomata growth and onset or termination of alcohol consumption. Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia. CASE REPORT: A 79-yr-old female observed an increase of fat mass especially at the upper arms, the thighs and the thorax in combination with a weight gain of 19 kg within 2 years without any changes in the nutrition habits. The unique features confirmed the diagnosis of LBS. Interestingly, she had diabetes mellitus, hypertension and hyperlipidemia before the manifestation of LBS and without any history of heavy alcohol consumption. Furthermore, the condition predominantly affects males. In her very case, treatment with sultanol, as successfully performed in some cases, could not be recommended due to her cardiac insufficiency. A surgical approach is of limited value due to the frequent relapses of the lipomata. CONCLUSIONS: Establishing the diagnosis of LBS is an important step for patients confronted with an inexplicable physical disfigurement that is not related to excessive nutrition. The disease is often not diagnosed because of its rareness, but its features are unique and easily to be distinguished from 'simple' truncal obesity.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/5. airway management: a patient with Madelung disease.This case study describes a 51-year-old, ASA physical status II Hispanic man who underwent liposuction to the anterior neck and resection of adipose tissue of the posterior neck. The patient was diagnosed with multiple symmetric lipomatosis, better known by its eponym, Madelung disease. This disease is characterized by abnormal adipose tissue growth primarily in the neck and shoulder regions. As a result of this pathology, a complete airway assessment was challenging. The patient's Mallampati classification was class III with visualization of the soft palate only. Other elements of a full assessment such as thyromental distance, sternomental distance, and tracheal orientation were impossible to evaluate because of the extreme circumferential girth of the adipose tissue in the neck. In addition, flexion and extension of the neck was limited by a minimum of 20 degrees because of the pathology. The patient was deemed as having a difficult airway, and an awake fiberoptic intubation was planned and used successfully. The subsequent surgical procedure progressed without incident, and the patient was transferred to a stepdown unit for monitoring of his airway postoperatively.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/5. Benign symmetric lipomatosis associated with alcoholism.A 46-year-old man was diagnosed as having benign symmetric lipomatosis (BSL) based on the grotesque physical examination findings and subcutaneous fat tissue biopsy. Although markedly overweight, the glucose tolerance was normal and insulin levels indicated no remarkable insulin resistance on the 75 g oral glucose tolerance test. Furthermore his visceral fat tissue was very slight and the circulating adiponectin concentration was high those which suggesting a high insulin sensitivity. In addition, the relevance of alcohol in the onset of BSL is strongly suggested based on alcoholic hepatopathy and the history of the development of grotesque physical appearance associated with increased alcohol consumption.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
4/5. Mitochondrial respiratory function in multiple symmetrical lipomatosis: report of two cases.Madelung's disease is a rare benign disorder characterized by symmetrical deposition of adipose tissue on the neck and shoulders. The cause of Madelung's disease is obscure and may be associated with mitochondrial dysfunction. In this communication, we report two cases encountered at Cardinal Tien Hospital during 1992. Besides physical check-up and laboratory examination, adipose tissue and muscle biopsies from the biceps brachii were performed. As lipid metabolism is closely related to mitochondrial respiration, biochemical studies may be of great value in clarifying the pathogenetic mechanism of Madelung's disease. We thus determined the activities of mitochondrial respiratory enzymes (NADH cytochrome c reductase, succinate cytochrome c reductase, cytochrome c oxidase) in muscle and blood cells and revealed that the electron transport functions were all elevated. Molecular analysis of mitochondrial dna from the muscle and blood cells of both patients failed to find large-scale deletion or point mutations.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/5. A case of the elephant man phenotype with giant rhinophyma and benign symmetric lipomatosis.We described an unusual male patient with two disfiguring diseases, benign symmetric lipomatosis and giant rhinophyma, which gave him a grotesque appearance. His physical appearance had isolated him from the rest of society and was probably the main reason for his alcohol abuse.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |