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1.
Molecular genetic studies have pointed to a relationship between congenital lipodystrophy syndromes and some cardiac disorders. For instance, mutations in LMNA cause either lipodystrophy or cardiomyopathy, indicating that different mutations in the same gene can produce these clinical syndromes. The present authors describe a 10-year-old female with Berardinelli-Seip congenital complete lipodystrophy (MIM 606158) caused by homozygosity for a frameshift mutation in BSCL2. In addition to the typical attributes of complete lipodystrophy, this subject had hypertrophic cardiomyopathy diagnosed in the first year of her life; its progress has been followed with non-invasive imaging. The mechanism underlying the hypertrophic cardiomyopathy in complete lipodystrophy is unclear. It may result from a direct effect of the mutant gene or it might be secondary to the effects of hyperinsulinemia on cardiac development. The variability of the associated cardiomyopathy in patients with complete generalized lipodystrophy may be caused by differential effects of mutations in the same gene or of mutations in different genes which underlie the lipodystrophy phenotype. 相似文献
2.
The cellular structure and lipid/protein composition of adipose tissue surrounding chronically stimulated lymph nodes in rats 下载免费PDF全文
To test the hypothesis that chronic immune stimulation of a peripheral lymph node induces the formation of additional mature adipocytes in adjacent adipose tissue, one popliteal lymph node of large male rats was stimulated by local injection of 10 microg or 20 microg lipopolysaccharide three times a week for 6 weeks. Adipocyte volumes in sites defined by their anatomical relations to the stimulated and homologous unstimulated popliteal lymph nodes were measured, plus adipocyte complement of the popliteal depot, and the lipid and protein content of adipocytes and adipose stroma. The higher dose of lipopolysaccharide doubled the mass of the locally stimulated lymph node and the surrounding adipose tissue enlarged by the appearance of additional mature adipocytes. Similar but smaller changes were observed in the popliteal adipose depot of the unstimulated leg and in a nodeless depot. The lipid content of the adipocytes decreased and that of the stroma increased dose-dependently in all samples measured but the changes were consistently greater in the depot surrounding the stimulated lymph node. The protein content of both adipocytes and stroma increased in samples surrounding the stimulated node. We conclude that chronic immune stimulation of lymphoid tissues induces the formation of more adipocytes in the adjacent adipose tissue. These findings suggest a mechanism for the selective hypertrophy of lymphoid-containing adipose depots in the HIV-associated adipose redistribution syndrome. 相似文献
3.
Lindegaard B Keller P Bruunsgaard H Gerstoft J Pedersen BK 《Clinical and experimental immunology》2004,135(2):273-279
This study tested the hypothesis that in patients with HIV-associated lipodystrophy, adiponectin levels were related to insulin resistance, TNF-alpha and IL-6 and treatment with nucleoside analogues. HIV seropositive men undergoing highly active antiretroviral treatment were enrolled into three predetermined clinical groups: lipodystrophy with central fat accumulation (n = 12); lipodystrophy without central fat accumulation (n = 15); no lipodystrophy (n = 15). HIV-negative healthy men served as controls (n = 12). Both lipodystrophic groups had a low percentage of limb fat compared to the two control groups. Patients with lipodystrophy with fat accumulation had increased truncal fat compared with controls. Levels of adiponectin did not correlate with either TNF-alpha or IL-6. Low levels of adiponectin were found in both lipodystrophic groups and were associated with current or previous treatment with stavudine. Furthermore, the adiponectin level correlated with the percentage of limb fat. Patients with lipodystrophy with fat accumulation were more insulin resistant, measured by HOMA-IR, compared with controls. However, HOMA-IR did no correlate to adiponectin or other cytokines. In conclusion, the finding of no difference between the two lipodystrophic groups with regard to adiponectin, indicates that low levels of adiponectin reflects fat atrophy, whereas the insulin resistance was best explained by increased truncal fat mass. 相似文献
4.
Orlandi B. Baldassarre M. Camponozzi F. A. Stanislao C. Di Poccia G. Donatis D. De 《The Italian Journal of Neurological Sciences》1992,13(2):161-164
We report the case of a 22 year old woman with congenital generalized lipodystrophy who presented a left brachiocrural pyramidal hemisyndrome, bilateral cerebellar signs and a left cranial nerve VI deficit. The clinical pattern had a tendency to regress. MRI brainscan, CSF examination and clinical features led to the diagnosis of probable demyelinating syndrome. Published data on CNS involvement in patients with congenital generalized lipodystrophy are few and we have found no cases in which a demyelinating syndrome is associated. In the case we report it is tempting to see the disorder of the lipid metabolism underlying the congenital generalized lipodystrophy as underlying the myelin disorder as well.
Sommario Descriviamo il caso di una paziente di 22 anni, affetta da Lipodistrofia Congenita Generalizzata, che ha presentato una sintomatologia caratterizzata da emisindrome piramidale brachiocrurale sinistra, segni cerebellari bilaterali e deficit del VI nervo cranico di sinistra. Il quadro clinico ha avuto una tendenza alla regressione. La RMN encefalo, l'esame del liquor e la clinica hanno fatto porre diagnosi di probabile sindrome demielinizzante. I dati della letteratura relativi al coinvolgimento del SNC in pazienti con Lipodistrofia Congenita Generalizzata sono scarsi ed in particolare non abbiamo rilevato casi di associazione con sindrome demielinizzante. Nel caso descritto è suggestivo che la turba del metabolismo lipidico alla base della Lipodistrofia Congenita Generalizzata possa essere in rapporto all'interessamento della mielina.相似文献
5.
Dr. T. Matsuo M. Suetsugu M. Eguchi M. Sasaki M. Tsuneyoshi 《European archives of psychiatry and clinical neuroscience》1982,231(2):123-130
Summary The case is described of a 35-year-old housewife diagnosed as having membranous lipodystrophy (as described by Nasu et al. in 1970 and called lipomembranous polycystic osteodysplasia by Hakola in 1972). The main symptom of this patient was a slowly progressive dementia. Skeletal symptoms were not seen. The computerized tomogram of the brain showed calcification of bilateral basal ganglia and the plain roentgenograms of the bones revealed cystic radiolucent areas at the distal end of the bones of the patient's extremities. Histological examination of the curetted material from the right talus revealed a membranocystic pattern. The fatty tissue curetted from the cyst of the talus and the lysosomal enzymes of the white blood cells were biochemically normal. A possible relationship between this disease entity and connective disorders is considered. 相似文献
6.
Sehar Sajid Mohammed Gulrez Zariwala Richard Mackenzie Mark Turner Theo Nell Srikanth Bellary Derek Renshaw 《Nutrients》2022,14(11)
Obesity is a multifactorial disease and is associated with an increased risk of developing metabolic syndrome and co-morbidities. Dysregulated expansion of the adipose tissue during obesity induces local tissue hypoxia, altered secretory profile of adipokines, cytokines and chemokines, altered profile of local tissue inflammatory cells leading to the development of low-grade chronic inflammation. Low grade chronic inflammation is considered to be the underlying mechanism that increases the risk of developing obesity associated comorbidities. The glucocorticoid induced protein annexin A1 and its N-terminal peptides are anti-inflammatory mediators involved in resolving inflammation. The aim of the current study was to investigate the role of annexin A1 in obesity and associated inflammation. To achieve this aim, the current study analysed data from two feasibility studies in clinical populations: (1) bariatric surgery patients (Pre- and 3 months post-surgery) and (2) Lipodystrophy patients. Plasma annexin A1 levels were increased at 3-months post-surgery compared to pre-surgery (1.2 ± 0.1 ng/mL, n = 19 vs. 1.6 ± 0.1 ng/mL, n = 9, p = 0.009) and positively correlated with adiponectin (p = 0.009, r = 0.468, n = 25). Plasma annexin A1 levels were decreased in patients with lipodystrophy compared to BMI matched controls (0.2 ± 0.1 ng/mL, n = 9 vs. 0.97 ± 0.1 ng/mL, n = 30, p = 0.008), whereas CRP levels were significantly elevated (3.3 ± 1.0 µg/mL, n = 9 vs. 1.4 ± 0.3 µg/mL, n = 31, p = 0.0074). The roles of annexin A1 were explored using an in vitro cell based model (SGBS cells) mimicking the inflammatory status that is observed in obesity. Acute treatment with the annexin A1 N-terminal peptide, AC2-26 differentially regulated gene expression (including PPARA (2.8 ± 0.7-fold, p = 0.0303, n = 3), ADIPOQ (2.0 ± 0.3-fold, p = 0.0073, n = 3), LEP (0.6 ± 0.2-fold, p = 0.0400, n = 3), NAMPT (0.4 ± 0.1-fold, p = 0.0039, n = 3) and RETN (0.1 ± 0.03-fold, p < 0.0001, n = 3) in mature obesogenic adipocytes indicating that annexin A1 may play a protective role in obesity and inflammation. However, this effect may be overshadowed by the continued increase in systemic inflammation associated with rapid tissue expansion in obesity. 相似文献
7.
Renal complications of lipodystrophy: A closer look at the natural history of kidney disease 下载免费PDF全文
Baris Akinci Sadiye Mehtat Unlu Ali Celik Ilgin Yildirim Simsir Sait Sen Banu Nur Fatma Ela Keskin Basak Ozgen Saydam Nilufer Kutbay Ozdemir Banu Sarer Yurekli Bekir Ugur Ergur Melda Sonmez Tahir Atik Atakan Arslan Tevfik Demir Canan Altay Ulku Aybuke Tunc Tugba Arkan Ramazan Gen Erdal Eren Gulcin Akinci Aslihan Arasli Yilmaz Habib Bilen Samim Ozen Aygul Celtik Senay Savas Erdeve Semra Cetinkaya Huseyin Onay Sulen Sarioglu Elif Arioglu Oral 《Clinical endocrinology》2018,89(1):65-75
8.
《Expert opinion on pharmacotherapy》2013,14(8):1055-1064
Exenatide is a glucagon-like peptide 1 receptor agonist, which has recently received FDA approval in the US for the treatment of Type 2 diabetes. Exenatide is an incretin mimetic that improves glycaemic control in patients with diabetes through acute mechanisms, such as glucose-dependent stimulation of insulin secretion, suppression of inappropriate glucagon secretion and slowing of gastric emptying, as well as chronic mechanisms that include enhancement of β-cell mass in rodent studies and weight loss and inhibition of food intake in humans. This article reviews the mechanisms of exenatide action, as well as its efficacy in the treatment of Type 2 diabetes. 相似文献
9.
Jason R. Jaggers Vivek K. Prasad Wesley D. Dudgeon Steven N. Blair Xuemei Sui Stephanie Burgess 《AIDS care》2014,26(11):1387-1392
Recent data show that people living with HIV/AIDS (PLWHA) are at a greater risk of cardiovascular disease (CVD), which could possibly be explained by an increased prevalence of metabolic syndrome (MetSyn) due to the known toxicities associated with antiretroviral therapy (ART). The purpose of this study is to examine the relationships between physical activity (PA) and components of MetSyn in a sample of PLWHA taking ART. A total of 31 males and 32 females living with HIV and currently taking ART were enrolled in a home-based PA intervention aimed to reduce risk factors for CVD. Clinical assessments included measures of resting blood pressure (BP), waist circumference, height, weight, PA levels via accelerometer, and a fasted blood draw. Components of MetSyn were divided into three clusters (1 = 0–1; 2 = 2; 3 = 3 or more). A one-way analysis of variance was used to determine differences between clusters. Multiple linear regressions were used to identify significant associations between moderate intensity PA (MPA) and sedentary time among components of MetSyn. MPA was significantly lower across MetSyn clusters (p < 0.001), whereas sedentary time was significantly higher (p = 0.01). A multiple linear regression showed MPA to be a significant predictor of waist circumference after controlling for age, race, gender, and sedentary time. Routine PA can be beneficial in helping PLWHA reduce waist circumference ultimately leading to metabolic improvements. This in turn would help PLWHA self-manage known components of MetSyn, thus reducing their risk of CVD and mortality. 相似文献
10.
Hua-dong Fan Shao-peng Chen Yu-xiang Sun Shao-hai Xu Li-jun Wu 《Acta pharmacologica Sinica》2015,36(4):497-506