Quiz case. Macrodystrophia lipomatosa.
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
An atrial septal mass was identified by echocardiogram in a patient with multiple subcutaneous lipomas. In order to differentiate the benign condition of lipomatous hypertrophy from myxoma, thrombus and other tumors, nuclear magnetic resonance and computed tomographic imaging were performed. Both techniques identified the adipose nature of the tissue noninvasively, consistent with the diagnosis of lipomatous hypertrophy. Pathologically demonstrated lipomatous hypertrophy in a postmortem heart was similarly characterized by nuclear magnetic resonance imaging.
Explore the source record for details and available documents.
A rare case of macrodactylia involving three of the four limbs was observed in a two and a half year old child. A diffuse swelling developed in the right axilla and on the right chest wall. The pathological diagnosis for the tumor was benign lipoblastomatosis. The relationship between the two disorders was discussed.
Radiographs and magnetic resonance (MR) imaging of three patients with macrodystrophia lipomatosa are presented. Radiographs of involved extremities and digits demonstrated prominent soft tissues and bony hypertrophy. MR images showed hypertrophy of fatty tissue, cortical bone thickening and fatty infiltration of muscle.
Explore the source record for details and available documents.
Madelung's disease is a benign disorder characterized by the presence of lipomata in the head and neck areas. It is disfiguring for the sufferer and is usually asymptomatic. Dysphagia and airway obstruction are rare presentations. Early surgical excision is recommended to exclude sinister pathology and to improve patient function. This is the first report (according to our literature search) of a patient who presented with dysphagia and obstructive sleep apnoea and in whom surgical intervention resulted in an improvement in both these symptoms.
Explore the source record for details and available documents.
BACKGROUND: In chronic venous insufficiency (CVI) III typical changes of skin, subcutaneous tissue, fascia and muscle can be found. It was believed, that the thickening of the fascia is irreversible and it has been postulated that the degeneration of muscles is causative for the persistence of recalcitrant leg ulcers. PATIENTS AND METHODS: By computed tomography (CT) and magnetic resonance imaging (MRI) 8 patients with therapy resistant venous ulcers were examined. Changes of fascia and muscles were determined preoperatively and 12 months after successful shave therapy. RESULTS: By CT a thinning of the preoperatively thickened fascia could be demonstrated in all patients one year after surgery. Before shave therapy MRI showed that the fascia was not only thickened and blurred, but also had fluid accumulations perifascial. 12 months after therapy the fascia was thinner and sharply demarcated. The fluid was either absent or distinctively reduced. Referring to the thickness of fascia an average decrease of 0.084 cm (0.03-0.17 cm) was observed. Before surgery the muscles showed a fatty degeneration as a sign of atrophy; with CT and MRI no changes could be demonstrated after one year in all patients. CONCLUSION: The results after one year demonstrate for the first time, that the changes of the fascia in CVI III are reversible and that there is no direct correlation between the degeneration of muscles and the persistence of venous leg ulcers.
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 microg/liter; controls, 37.18 microg/liter) and the insulin responsiveness index was also comparable in the two groups (LBS group, 3.47 micromol/kg. minute; controls, 3.79 micromol/kg. 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.
In humans and most animal models, the development of obesity leads not only to increased fat depots in classical adipose tissue locations but also to significant lipid deposits within and around other tissues and organs, a phenomenon known as ectopic fat storage. The purpose of this review is to explore the possible locations of ectopic fat in key target-organs of cardiovascular control (heart, blood vessels and kidneys) and to propose how ectopic fat storage can play a role in the pathogenesis of cardiovascular diseases associated with obesity. In animals fed a high-fat diet, cardiac fat depots within and around the heart impair both systolic and diastolic functions, and may in the long-term promote heart failure. Accumulation of fat around blood vessels (perivascular fat) may affect vascular function in a paracrine manner, as perivascular fat cells secrete vascular relaxing factors, proatherogenic cytokines and smooth muscle cell growth factors. Furthermore, high amounts of perivascular fat could mechanically contribute to the increased vascular stiffness seen in obesity. Finally, accumulation of fat in the renal sinus may limit the outflow of blood and lymph from the kidney, which would alter intrarenal physical forces and promote sodium reabsorption and arterial hypertension. Taken together, ectopic fat storage in key target-organs of cardiovascular control may impair their functions, contributing to the increased prevalence of cardiovascular diseases in obese subjects.
1. Lipoprotein lipase activity and hormone-sensitive lipase activity were investigated in subcutaneous lipomas removed from two patients and compared with the enzyme activities in subcutaneous adipose tissue from two normal subjects. 2. Confirmation was obtained of the presence of lipoprotein lipase activity in lipomas with an activity fifteen to forty-five times that in the two control samples. 3. Hormone-sensitive lipase activity was demonstrated in lipomas under basal conditions of assay as well as in the presence of adrenaline plus theophylline. However, compared with the non-lipomatous fat samples, these activities were lower, as was the magnitude of the lipolytic response to adrenaline plus theophylline. 4. The significance of these measurements of enzyme activity and their role in the pathogenesis of lipomas are briefly discussed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.