Centrifugal lipodystrophy.
We describe a case of centrifugal lipodystrophy occurring in a young English girl, and compare the clinical and histopathological features with previous reports from Japan.
Child, Preschool↗
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Lipodystrophy with hyperlipidaemia: the role of insulin in very low density lipoprotein over-synthesis.
A patient with partial lipodystrophy is described in whom hypertriglyceridaemia was accompanied by marked hyperinsulinaemia. The hyperlipidaemia was due to increased plasma levels of very low density lipoprotein (VLDL). Kinetic studies, performed after injection of autologous radioiodinated VLDL, indicated that the raised VLDL levels were associated with over-production of this lipoprotein. Administration of diazoxide led to a substantial fall in serum insulin levels, accompanied by reduction in VLDL production and in serum triglyceride concentration. The possible role of insulin in inducing hyperlipidaemia by causing over-production of VLDL is discussed.
Adult↗
Beta-cell dysfunction and low insulin clearance in insulin-resistant human immunodeficiency virus (HIV)-infected patients with lipodystrophy.
OBJECTIVE: To obtain a better understanding of the physiological aspects of glucose homeostasis in human immunodeficiency virus (HIV)-infected patients with lipodystrophy, we evaluated separately beta-cell function and insulin sensitivity after an oral glucose load. DESIGN: Beta-cell function was investigated during an oral glucose tolerance test (OGTT) (75 g, 180 min) in 16 lipodystrophic HIV-infected patients and in 15 age- and weight-matched nonlipodystrophic HIV-infected patients. All participants were sedentary Caucasian males, who were on highly active antiretroviral therapy with no history of diabetes mellitus or impaired glucose tolerance. Prehepatic insulin secretion rates were estimated by deconvolution of C-peptide concentrations. A composite measure of insulin sensitivity was derived from the OGTT. RESULTS: Beta-cell secretory capacity (i.e. the rate of change in insulin secretion per unit change in glucose concentration) was similar in lipodystrophic and nonlipodystrophic patients (6.2 +/- 1.0 mU kg(-1) min(-1) mg(-1) dl vs. 5.4 +/- 0.4, P > 0.4), but insulin sensitivity was reduced by 61% in lipodystrophic patients (P < 0.004). The disposition index (insulin capacity multiplied with insulin sensitivity) and insulin clearance rate were reduced in lipodystrophic patients (-55%, P < 0.003 and -31%, P < 0.004). Insulin clearance rate correlated strongly with insulin sensitivity (r = 0.82, P < 0.001). More lipodystrophic than nonlipodystrophic patients exhibited impaired glucose tolerance and diabetes mellitus (63%vs. 20%, P < 0.05). CONCLUSIONS: Our data support the concept that impaired glucose tolerance in lipodystrophic HIV-infected patients relates to a failure of the beta-cells to fully compensate for decrements in insulin sensitivity despite simultaneous reduction in insulin clearance.
Adult↗
Plasminogen activator inhibitor type 1 (PAI-1) in plasma and adipose tissue in HIV-associated lipodystrophy syndrome. Implications of adipokines.
BACKGROUND: PAI-1, an important inhibitor of fibrinolysis, is increased in obese subjects and has been shown to be an independent risk factor for cardiovascular disease. In the present study, we investigated the association between circulating levels of PAI-1 and locally produced PAI-1 in adipose tissue and body fat distribution and adipokines (TNF-alpha, TNF receptors, IL-6, IL-8) in patients with and without HIV-associated lipodystrophy syndrome (HALS). MATERIALS AND METHODS: Eighteen men with HALS and 18 men with HIV but without HALS were investigated. DEXA and computed tomography scan were performed to determine total body fat and visceral adipose tissue mass. Insulin sensitivity was determined by the euglycaemic clamp technique. Plasma levels of PAI-1 and cytokines were determined. In addition, PAI-1, TNF-alpha, IL-6 and IL-8 mRNA levels in subcutaneous adipose tissue were measured by real-time reverse transcriptase polymerase chain reaction. RESULTS: HALS patients were characterized by a 3-fold increased visceral adipose tissue (P < 0.001) and reduced limb fat (P < 0.01) as compared with non-HALS patients but with no difference in total fat mass between the groups. Plasma PAI-1 was increased in HALS patients (16.7 ng mL(-1) vs. 8.2 ng mL(-1), P < 0.05). Plasma PAI-1 was positively correlated with BMI (r = 0.74, P < 0.01), plasma TNF-alpha level (r = 0.64, P < 0.01), sTNFR-I (r = 0.38, P < 0.05), and visceral fat (r = 0.67, P < 0.01). Moreover, plasma PAI-1 was negatively associated with insulin sensitivity (r = -0.57, P < 0.01) and the percentage of limb fat (r = -0.57, P < 0.01). A positive correlation was found between plasma PAI-1 and TNF-alpha mRNA level. No association was, however, found between plasma PAI-1 and PAI-1 mRNA level in adipose tissue. CONCLUSION: Plasma PAI-1 is increased in HALS patients and it is suggested that dysregulation of the TNF-system (high TNFalpha and high sTNFR1) may play a role in up-regulating PAI-1 in HALS.
Adipose Tissue↗
Congenital generalized lipodystrophy: profile of the disease and gender differences in two siblings.
Congenital generalized Lipodystrophy (BSCL) or Berardinelli-Seip syndrome (Mendelian inheritance in man, catalog no. 269700) is a rare autosomal recessive syndrome characterized by paucity of body fat since birth and insulin resistance. The pathophysiology of this condition is unclear, but defects in insulin function and impaired adipogenesis have been described as important factors in the etiology of the disease. Recently, two gene loci have been identified to harbor the mutations causing this disorder: BSCL1 mapped to human chromosome 9q34 (1, 2) and BSCL2 mapped to human chromosome 11q13 (1, 3). This report describes the natural history of the disease in two siblings (female and male) of Lebanese origin who have mutations in the BSCL2 locus (669delGTATC).
Child↗
Partial face-sparing lipodystrophy (Köbberling-Dunnigan syndrome): report of a sporadic case.
A sporadic case is presented of a 20-year-old female with partial face-sparing lipodystrophy, also known as the Köbberling-Dunnigan syndrome with the Type 1 clinical phenotype. This disorder affects the limbs and spares the face. The trunk is spared in the Type 1 phenotype but is affected in the Type 2 phenotype. The patient had the associated features of this syndrome including the hyperinsulinaemia, acanthosis nigricans and mildly raised triglycerides, but not diabetes mellitus.
Acanthosis Nigricans↗
Diastasis recti abdominis in HIV-infected men with lipodystrophy.
Diastasis recti abdominis is a condition in which the rectus abdominus muscle separates in the midline at the linea alba producing a ventral herniation. We have observed the occurrence of this condition in HIV-infected men attending an osteopathic clinic. Two such cases are described in detail. An apparent association with HIV-associated lipodystrophy syndrome and implications for management are explored.
Abdominal Muscles↗
The role of diet, exercise and smoking in dyslipidaemia in HIV-infected patients with lipodystrophy.
BACKGROUND: Lipodystrophy in HIV-infected (LDHIV) patients receiving protease inhibitors (PIs) is associated with dyslipidaemia. Whether lifestyle factors play a role in dyslipidaemia in LDHIV subjects on PIs is not well characterized. METHODS: A total of 45 LDHIV male and six LDHIV female patients on PIs were recruited, and data were collected on smoking, exercise, diet (by 3-day food record), and fasting levels of serum lipids and lipoproteins. The relationships between lifestyle factors and metabolic variables were analysed in male patients by Spearman's correlation test and the significant relationships were further analysed by adjusting for age, PI duration, and waist circumference by Spearman's partial correlation test. RESULTS: In men, mean (+/-standard deviation) serum concentrations of total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), triglycerides (TG), and non-HDL-C were 212+/-70, 35+/-7.3, 325+/-230 and 169+/-44 mg/dL, respectively. Sixty-seven percent of the men exercised regularly and 31.1% smoked. The reported diet was high in cholesterol (390+/-212 mg) and percentage energy from saturated (12.2+/-3.3%) and trans (2.4+/-1.2%) fats, and low in soluble fibre (6.9+/-2.3 g) compared with recent dietary guidelines. Following adjustments for the confounding variables, percentage energy intake from total protein and animal protein was positively related to TC (r=0.44, P<0.01 and r=0.37, P<0.05, respectively), TG (r=0.40, P<0.01 and r=0.46, P<0.01, respectively) and non-HDL-C (r=0.56, P<0.001 and r=0.49, P<0.01, respectively), that from trans fat was positively related to TG (r=0.34, P<0.05), and soluble fibre was negatively related to non-HDL-C (r=-0.41, P<0.01). Moderate to heavy aerobic exercise tended to be associated with higher HDL-C (r=0.30, P=0.07) whereas smoking was not associated with any of the metabolic variables. CONCLUSIONS: Increased intake of total protein, animal protein and trans fat, and reduced soluble fibre consumption contribute to dyslipidaemia in LDHIV subjects on PIs.
Adult↗
A randomized, open-label study to compare the effects of two different doses of recombinant human growth hormone on fat reduction and fasting metabolic parameters in HIV-1-infected patients with lipodystrophy.
BACKGROUND: Several studies have shown beneficial effects of recombinant human growth hormone (r-hGH) in reducing visceral adipose tissue (VAT) in HIV-1-infected patients with lipodystrophy. METHODS: Patients were randomized to r-hGH 4 mg daily (group A) or three times per week (group B) over 12 weeks, followed by a 2 mg daily maintenance dose for 12 weeks. Magnetic resonance imaging (MRI) scans were performed to assess body composition. RESULTS: A total of 26 subjects were included in the study. VAT was reduced overall by 35.1 cm(2) (29.5%) at week 12 and by 49 cm(2) (39.9%) at week 24, respectively, compared with baseline (P<0.001 for both comparisons). By week 12, VAT was reduced by 27 and 29% (A vs B; P=0.47) while facial fat was reduced by 3.3 and 2.6 cm(2) in groups A and B, respectively (P=0.96). Over 24 weeks, VAT was reduced by 42 and 38% (P=0.35) and facial fat by 3.2 and 2.4 cm(2) in groups A and B, respectively (P=0.91), compared with baseline. There was a greater increase in high-density lipoprotein (HDL) in group A than in group B (4.9 vs 2.4 mg/dL in week 12 and 7.1 vs -0.4 mg/dL in week 24; P=0.03). Fasting insulin levels increased, whereas glucose and insulin measured in oral glucose tolerance tests remained unchanged. Drug-related side effects were transient and reversible, but more common in group A (67%) than in group B (29%). CONCLUSIONS: This study confirms reports that r-hGH effectively reduces VAT, with a relatively small reduction of facial and limb fat.
Adipose Tissue↗
Lipodystrophy following hepatitis infection: a causative relationship?
Progressive partial lipodystrophy is a clinical syndrome characterized by progressive loss of subcutaneous fat. A 27-year-old female developed progressive loss of fat from the face and upper part of the torso associated with increased bulkiness of the body below the pelvis following an attack of hepatitis. She had been persistently hepatitis B surface antigen positive and her serum was positive for IgG antibodies to hepatitis B core antigen. Although the woman had hypocomplementaemia of the C3 component of complement, there was no evidence of renal involvement.
Adult↗
Cutaneous myospherulosis and membranous lipodystrophy: extensive presentation in a patient with severe steroid-induced dermal atrophy.
Myospherulosis is a rare disorder that may develop in various organs including the skin. It is characterized by a typical histological presentation resulting from the interaction between erythrocytes and lipids. We present a case of cutaneous myospherulosis associated with membranous lipodystrophy. The lesions were supervening on severe steroid-induced skin atrophy upon which ointments had been applied.
Atrophy↗
Use of hyaluronic acid for soft tissue augmentation of HIV-associated facial lipodystrophy.
BACKGROUND: Lipodystrophy syndrome is a devastating complication of antiretroviral therapy in individuals with human immunodeficiency virus (HIV). The appearance of the associated facial lipoatrophy can be demoralizing and stigmatizing for the affected individuals to a point at which it may compromise their compliance with antiretroviral medication. OBJECTIVE: We describe the use of hyaluronic acid as an intradermal filler for correction of this disfiguring problem. METHODS: We treated five patients with grade 2 to 3 facial lipoatrophy. Each patient received approximately 5 to 6 cc in total of hyaluronic acid in the malar area via intradermal injection. RESULTS: There were no adverse events. We found that this technique provided a good cosmetic result with high patient satisfaction. At 6-month follow-up, sustained longevity was observed. CONCLUSIONS: We propose the use of hyaluronic acid for HIV-associated facial lipoatrophy as an efficacious and safe, but temporary, option for this problem until a more cost-effective option is available.
Adult↗
The renal lesion associated with total lipodystrophy.
This report concerns a 41-year-old man with total lipodystrophy associated with a specific renal disorder. He had massive proteinuria, mild azotemia and a normal level of serum complement in addition to the generalized loss of subcutaneous fatty tissue. Results of 1 50-gm glucose tolerance test indicated a high fasting insulin level with an exaggerated response to glucose. Hypoglycemic responses to exogenous insulin were reduced. Renal biopsy revealed changes consistent with glomerular lipidosis. This case is unique in that the renal lesion was not the Kimmelstiel-Wilson pathologic change atributable to abnormal glucose metabolism.
Adult↗
Localized lipodystrophy with panniculitis: light and electron microscopic studies.
A 41-year-old woman with localized lipodystrophy histologically showed lymphohistiocytic inflammation within adipose tissue at the periphery and loss of adipose tissue in the center of the lesion. Ultrastructurally, various degrees of lipophagocytosis by macrophages and giant cells were seen, starting with the projection of cytoplasmic processes into the fat droplet and engulfing pieces of the fat droplet which finally were digested. Laboratory investigations revealed a high serum gamma-globulin level and a positive antinuclear antibody, suggestive of a connective tissue disease or autoimmune disease.
Adipose Tissue↗
Congenital generalized lipodystrophy.
Six patients with congenital generalized lipodystrophy are described. They had generalized paucity of fat tissue, acanthosis nigricans, prominent superficial veins and muscle hypertrophy. They were mentally retarded. Three had corneal opacities. They had normal external genitalia and none was tall for age. Their bone age was advanced and some had minor skeletal anomalies and nephromegaly. The muscle histology on light microscopy was normal. The majority had elevated serum aldolase and to a lesser degree serum lactic dehydrogenase and creatinine phosphokinase. Four of five examined had a myopathic electromyogram. They had normal or deranged liver function tests. The fatty liver infiltration in one seems to be progressive. Four had a normal and two an abnormal metyrapone test. They had an age-dependent abnormality of growth hormone, insulin and carbohydrate homeostasis.
Acanthosis Nigricans↗
Cirrhosis in partial lipodystrophy.
A patient with partial lipodystrophy, fatty liver and cirrhosis, and autoimmune thyroid disease is described. Treatment with thyroxine led to partial improvement in the hepatic abnormality.
Adult↗
Hepatic lipodystrophy of pedigree Galloway calves.
Hepatic lipodystrophy has been recognised in pedigree Galloway calves since 1965. Between 1975 and 1984 15 cases from five farms were examined. The calves initially appear normal and in good bodily condition but invariably die by five months of age. The characteristic clinical and neurological changes lead to body tremors, opisthotonus, and dyspnoea before the animals become recumbent and die. On postmortem examination the most significant finding in all cases was an enlarged, pale and mottled liver weighing up to 2.75 kg. Limited histopathological examinations of the brain and liver revealed changes suggestive of hepatic encephalopathy. Exhaustive investigations of the farms failed to reveal any significant findings and the small number of cases made it impossible to determine whether the disease was genetically determined; limited evidence suggests that a 'storage disease' cannot be excluded.
Animals↗
HIV-associated lipodystrophy: pathogenesis, prognosis, treatment, and controversies.
Potent antiretroviral agents markedly suppress HIV and have dramatically improved the clinical course, prognosis, and survival of HIV-infected patients. Unfortunately, highly active antiretroviral therapy is often compromised by metabolic complications, including insulin resistance, dyslipidemia, and fat redistribution. Together these changes have been termed the HIV-lipodystrophy syndrome, which is estimated to affect a majority of patients treated with potent combination antiretroviral therapy. Routine testing of fasting glucose is recommended for all HIV-infected patients, particularly those who are obese, have a family history of diabetes mellitus, or are receiving protease inhibitor therapy. Preliminary investigations have demonstrated the potential utility of insulin-sensitizing agents and lipid-lowering therapies to ameliorate these metabolic disturbances. Patients with HIV infection who demonstrate fat redistribution and develop hyperinsulinemia and dyslipidemia may be at increased risk of cardiovascular disease. However, the long-term effects on cardiovascular disease have not yet been determined.
HIV-Associated Lipodystrophy Syndrome↗