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At least 289 records · Page 16Linked to original sources
PubMed1989

Scintigraphic findings in congenital lipodystrophy.

The scintigraphic findings of a patient with characteristic clinical, laboratory, and radiographic features of congenital lipodystrophy were studied. Bone scan showed uniform increased bone uptake of Tc-99m MDP with markedly enhanced peri-articular activity and very prominent renal activity. Liver-spleen scan showed marked hepatosplenomegaly. This combination of scintigraphic abnormalities is unique in congenital lipodystrophy and constitutes a very interesting constellation of findings. Observed scintigraphic findings are compatible with previously described radiographic findings.

Adult↗
PubMed1990

Cytophagic histiocytic panniculitis evolving into total lipodystrophy.

A 15-year-old boy had persistent fever with severe neutropenia, thrombocytopenia, coagulation disorder, and marked elevation of lactate dehydrogenase values. A diagnosis of cytophagic histiocytic panniculitis was made after repeated skin biopsies. Three years after onset, he gradually lost 20 kg in body weight, and both skin and bone marrow specimens revealed degenerative changes of fatty tissue without any inflammatory cells. These findings suggest that total lipodystrophy syndrome results from the inflammatory destructive process of adipose tissue. To our knowledge, our report is the first to show, in a series of histologic studies, that an inflammatory destructive process may be involved in some cases of total lipodystrophy syndrome.

Adolescent↗
PubMed2003

Leptin replacement therapy but not dietary polyunsaturated fatty acid alleviates HIV protease inhibitor-induced dyslipidemia and lipodystrophy in mice.

A major complication associated with the use of protease inhibitors (PIs) in treatment of HIV-infected patients is lipid abnormalities including dyslipidemia, lipodystrophy, and liver steatosis. Previous studies revealed that these abnormalities are associated with PI-induced accumulation of activated sterol regulatory element binding proteins (SREBPs) in the nucleus of liver and adipose tissues, resulting in constitutive activation of lipid metabolism genes. This study used the mouse model to determine the potential of polyunsaturated fatty acid (PUFA) diet or leptin replacement therapy to alleviate these PI-induced metabolic abnormalities. Results showed that feeding C57BL/6 mice with a PUFA-rich diet failed to normalize plasma cholesterol and triglyceride levels in ritonavir-treated mice. The PUFA-rich diet also had no effect on ritonavir-induced interscapular fat accumulation and liver steatosis. In contrast, daily administration of leptin significantly reversed the elevated plasma cholesterol level induced by ritonavir. Leptin replacement therapy also significantly reduced the ritonavir-induced interscapular fat mass and improved liver steatosis. Taken together, these data suggest that PI-induced lipid abnormalities, especially dyslipidemia, lipodystrophy, and liver steatosis, may be reduced with leptin replacement therapy.

Adipose Tissue↗
PubMed2006

Lipodystrophy is an independent predictor of hypertriglyceridemia during pregnancy in HIV-infected women.

Lipid values were measured during pregnancy in HIV-infected, treatment-experienced women. A previous history of lipodystrophy was associated with significantly higher triglyceride values at all pregnancy trimesters. In multivariate analyses lipodystrophy independently increased the risk of hypertriglyceridemia by threefold at the first trimester, and by eightfold at the second and third trimesters. Protease inhibitor treatment was also independently associated with hypertriglyceridemia.

Adult↗
PubMed2005

HIV-associated cervicodorsal lipodystrophy: etiology and management.

OBJECTIVES: To familiarize the otolaryngologist with the evaluation and management of cervicodorsal manifestations of lipodystrophy in patients who have been treated with HIV protease inhibitor medications. In addition, to share the benefits obtainable with ultrasonic tumescent liposuction treatment. STUDY DESIGN: Retrospective chart review of patients presenting to the senior author with symptomatic hypertrophic cervicodorsal fat pad attributable to HIV infection and HIV protease inhibitor use. RESULTS: Eight patients presented for evaluation of hypertrophic cervicodorsal fat pads between January 1, 2002 and December 31, 2004. All patients had been on protease inhibitors in the past and had minimal resolution after discontinuing offending agent. Most common presenting problems include disfigurement, limited range of upper extremity and neck motion, neck and back discomfort, and difficulty with sleep including sleep-study-confirmed obstructive sleep apnea. Five of eight patients underwent ultrasonic tumescent liposuction. Three patients had satisfactory improvement of symptoms after the first surgery, whereas the other two required additional operative sessions. No complications of hematoma, seroma, infection, prolonged pain, or re-accumulation of fat pad were encountered. The primary obstacle in the three nonoperative patients was insurance denial on the basis of deemed lack of established necessity. CONCLUSIONS: Cervicodorsal lipodystrophy is a well-recognized outcome of prolonged HIV infection and side effect of certain HIV medications. Patients may present with both esthetic and functional issues related to the excess tissue. Although cessation of associated medications may halt further progression, this alone does resolve the symptoms. Ultrasonic tumescent liposuction is shown in this study to be a well-suited modality for reduction of this fibrous adipose tissue. Multiple sessions may be necessary to achieve satisfactory results because of the tenacity of the tissue. It is important for the otolaryngologist to be familiar with the head and neck issues relevant to this disorder and its treatment.

Adult↗
PubMed2005

Cellular mechanisms of insulin resistance, lipodystrophy and atherosclerosis induced by HIV protease inhibitors.

Accumulating clinical evidence now links HIV protease inhibitors (HPIs) to the pathogenesis of insulin resistance, dyslipidaemia, lipodystrophy and atherosclerosis associated with highly active anti-retroviral therapy. Here we briefly describe the evidence for a distinct causative role for HPIs, and explore the cellular mechanisms proposed to underlie these side-effects. Acute inhibition of GLUT4-mediated glucose transport, and defective insulin signalling induced by chronic exposure to nelfinavir, are described as cellular mechanisms of insulin resistance. Interference with adipogenesis and adipocyte apoptosis and nelfinavir-induced activation of lipolysis are discussed as potential mechanisms of HPI-induced lipodystrophy. HPI-induced free radical production, apoptosis and increased glucose utilization in vascular smooth muscle cells are presented as possible novel mechanisms for atherosclerosis. Common pathways and cause-effect relationships between the various cellular mechanisms presented are then discussed, with emphasis on the role of insulin resistance, free radical production and enhanced lipolysis. Understanding the cellular mechanisms of HPI-induced side-effects will enhance the search for improved anti-retroviral therapy, and may also shed light on the pathogenesis of common forms of insulin resistance, dyslipidaemia and atherosclerosis.

Adipocytes↗
PubMed1975

Plasma free fatty acid turnover in total lipodystrophy.

Basal free fatty acid (FFA) turnover was found to be elevated or at the upper limit of normal in a patient with total lipodystrophy. The decrease in plasma FFA concentration which occurred after intravenous administration of insulin was shown to be primarily due to a fall in the inflow of FFA into the circulation. These results provide support for the suggestion that adipocytes in total lipodystrophy are able to synthesize triglycercide, but that they are unable to store it because of rapid lipolysis.

Adult↗
PubMed1990

Partial lipodystrophy syndromes--a further male case.

Details are presented of a boy with partial lipodystrophy. Only one male case has previously been described with this condition. The spectrum of partial lipodystrophy syndromes and the inheritance thereof are discussed in relation to our case.

Genetic Counseling↗
PubMed2006

Diabetes with partial lipodystrophy following sclerodermatous chronic graft vs. host disease.

BACKGROUND: The importance of adipose tissue in metabolism, as a target for insulin action and a secretor of metabolic regulatory proteins, is increasingly recognized. Lipodystrophic conditions are often associated with significant insulin resistance. The commonest acquired form occurs with highly active antiretroviral therapy (HAART) for human immunodeficiency virus infection. Other medical conditions and drugs also have the potential to cause chronic subcutaneous fat damage. CASE REPORT: We describe an unfamiliar partial lipodystrophy in a young woman, associated with markedly insulin-resistant diabetes, acquired following allogeneic bone marrow transplantation for childhood leukaemia complicated by late sclerodermatous chronic graft vs. host disease (GVHD). Clinical examination revealed scarring and lipodystrophy affecting mainly legs, thighs, buttocks and forearms but sparing her face, neck and thorax. Her serum adiponectin level was markedly reduced. CONCLUSIONS: However, although thiazolidinediones lower insulin resistance and increase subcutaneous peripheral fat in Type 2 diabetes, pioglitazone treatment had little effect on either serum adiponectin, glycaemic control or the lipoatrophy. In this case, effective glycaemic control was best achieved using a combination of metformin and highly concentrated soluble insulin injections.

Adolescent↗
PubMedDate not supplied

Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases.

A 27-year-old woman and a 13-year-old girl diagnosed with juvenile dermatomyositis in childhood developed clinical findings of partial lipodystrophy 10 years after diagnosis. Exhaustive clinical and laboratory examinations showed an association with other abnormalities: hypertrichosis, steatohepatitis, and an abnormal insulin response to the glucose loading test in the first patient. Hypertrichosis, steatohepatitis, insulin-resistant diabetes mellitus, and acanthosis nigricans were observed in the second patient. Renal function was normal in both patients. Although a localized form of lipodystrophy has been reported associated with connective tissue disease (connective tissue lipoatrophy), the partial form has been infrequently described in association with juvenile dermatomyositis.

Adolescent↗
PubMed1996

Cardiac findings in generalized lipodystrophy.

The cardiac findings in eight patients, two of whom were female, with total lipodystrophy (Berardinelli-Seip's disease) are reported. One of them had the acquired form of the disease. Four patients died at a mean age of 32 years. As far as we know, at least three of them most likely died for cardiac reasons, one shortly after recovering from an attempted suicide. All eight patients had hypertrophic hearts, mostly with deranged diastolic, but also systolic, function. One had pulmonary hypertension. We conclude that generalized lipodystrophy is a serious disease with cardiac affection leading to cardiac dysfunction and early death. There is no specific cardiac treatment, and the treatment should be according to the general guidelines for patients with hypertrophic, dysfunctioning hearts.

Adult↗
PubMed1996

Radiological features in generalized lipodystrophy.

Seven patients with congenital generalized lipodystrophy and one patient with acquired generalized lipodystrophy have been followed up for 38 years. Various radiological techniques have been used, and the major findings are summarized. All patients had an advanced skeletal age. A sclerotic skeleton was a major feature in childhood in the congenital form, later turning into osteolytic lesions or more distinct sclerotic patches in adolescence or early adulthood. In the acquired form the skeleton was slightly osteoporotic without cystic changes. Ultrasonography and computerized tomography examinations revealed hepatosplenomegaly with fatty infiltration in both types. Lack of subcutaneous and intraabdominal fat was easily confirmed with computerized tomography. Five patients with the congenital for underwent pneumoencephalography, which revealed dilated brain ventricles and basal cisterns. Cardiomegaly and large kidneys were also a major feature.

Adolescent↗
PubMed2025

Linear ubiquitination prevents lipodystrophy and obesity-associated metabolic syndrome.

Adipocyte hypertrophy during obesity triggers chronic inflammation, leading to metabolic disorders. However, the role of adipocyte-specific inflammatory signaling in metabolic syndrome remains unclear. The linear ubiquitin chain assembly complex, LUBAC, is an E3-ligase that generates nondegradative linear ubiquitination (Lin-Ub). LUBAC regulates NF-κB/MAPK-driven inflammation and prevents cell death triggered by immune receptors like TNF receptor-1. Here, we show that mice lacking HOIP, the Lin-E3 ligase catalytic subunit of LUBAC, in adipocytes (HoipA-KO) display lipodystrophy and heightened susceptibility to obesity-induced metabolic syndrome, particularly metabolic dysfunction-associated steatotic liver disease (MASLD). Mechanistically, loss of HOIP attenuates TNF-induced NF-κB activation and promotes cell death in human adipocytes. Inhibiting caspase-8-mediated cell death is sufficient to prevent lipodystrophy and MASLD in HoipA-KO obese mice. HOIP expression in adipose tissue positively correlates with metabolic fitness in obese individuals. Overall, our findings reveal a fundamental developmental role for Lin-Ub in adipocytes by mitigating cell death-driven adipose tissue inflammation and protecting against obesity-related metabolic syndrome.

Animals↗
PubMed1997

Congenital total lipodystrophy and peripheral pulmonary artery stenosis.

Multiple peripheral pulmonary artery stenoses were detected in three patients with congenital generalised lipodystrophy. This association, which has not been described before, may be clinically important in patients with lipodystrophy who present with impaired exercise tolerance or heart murmurs.

Adolescent↗
PubMed2002

Genotype-phenotype relationships in Berardinelli-Seip congenital lipodystrophy.

Generalised lipodystrophy of the Berardinelli-Seip type (BSCL) is a rare autosomal recessive human disorder with severe adverse metabolic consequences. A gene on chromosome 9 (BSCL1) has recently been identified, predominantly in African-American families. More recently, mutations in a previously undescribed gene of unknown function (BSCL2) on chromosome 11, termed seipin, have been found to be responsible for this disorder in a number of European and Middle Eastern families. We have studied the genotype/phenotype relationships in 70 affected subjects from 44 apparently unrelated pedigrees of diverse ethnic origin. In all subjects, hepatic dysfunction, hyperlipidaemia, diabetes mellitus, and hypertrophic cardiomyopathy were significant contributors to morbidity with no clear differences in their prevalence between subjects with BSCL1 or BSCL2 and those with evidence against cosegregation with either chromosome 9 or 11 (designated BSCLX). BSCL2 appears to be a more severe disorder than BSCL1 with a higher incidence of premature death and a lower prevalence of partial and/or delayed onset of lipodystrophy. Notably, subjects with BSCL2 had a significantly higher prevalence of intellectual impairment than those with BSCL1 or BSCLX (p<0.0001, OR 17.0, CI 3.6 to 79.0). The higher prevalence of intellectual impairment and the increased risk of premature death in BSCL2 compared to BSCL1 emphasise the importance of molecular diagnosis of this syndrome and have clear implications for genetic counselling.

Adolescent↗
PubMed1988

Retinal pigment epithelial change and partial lipodystrophy.

Cuticular drusen and retinal pigment epithelial changes were found incidentally in a 27 year old Lebanese woman during assessment of partial lipodystrophy. Her vision was normal despite involvement of both maculae. The patient had hypocomplementaemia, but serum C3 nephritic factor was absent and renal function was normal. She had impaired glucose tolerance and a continuous infusion of glucose with model assessment (CIGMA) test revealed low normal tissue insulin sensitivity and high normal pancreatic beta cell function. Mild fasting hypertriglyceridaemia (2.0 mmol/l) may have been secondary to impaired insulin sensitivity. Endocrine function was otherwise normal apart from a completely absent growth hormone response to adequate hypoglycaemia. The simultaneous occurrence of partial lipodystrophy and retinal pigmentary epithelial and basement membrane changes appears to be a newly recognized syndrome.

Adult↗
PubMed2002

Does increased aromatase activity in adipose fibroblasts cause low sexual desire in patients with HIV lipodystrophy?

There is evidence from our own unit and other workers that many patients who have lipodystrophy on HAART given for HIV disease also have raised oestrogen levels and complain of low sexual desire. This hypothesis paper discusses a possible pathological mechanism for these changes--an increase in the number of fibroblasts and macrophages present in lipoatrophic areas that could convert testosterone to oestrogen by intracellular aromatisation. This process is known to be enhanced by increased levels of tumour necrosis factor, interleukin 6 (IL-6), and hydroxycorticosteroids present in many patients with HIV lipodystrophy. Treatment options are discussed, including aromatase inhibitors and testosterone.

Adipose Tissue↗