Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A female, term neonate presented with generalized edema, heavy proteinuria, hypoalbuminemia and hyperlipidemia in the second week of life. The clinical and laboratory features were compatible with the diagnosis of congenital nephrotic syndrome. Treatment included albumin infusion, empirical penicillin, steroid and continuous arterio-venous hemofiltration. Intestinal perforation developed at the 19th day of age and led to a fatal outcome. At autopsy, thrombosis of the superior mesenteric artery and its branches was noted, and histology of the kidney was compatible with congenital nephrotic syndrome of the Finnish type. The risk of thromboembolism, arterial or venous, should be considered in patients with nephrotic syndrome, even in the neonatal period. Preventive measures, including avoiding volume depletion and femoral arterial/venous puncture, are essential in managing these patients. Prophylactic anticoagulation and infusion of fresh frozen plasma may be warranted to avoid such potentially lethal complications of thromboembolism.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Mesenteric arterial thrombosis complicating congenital nephrotic syndrome of Finnish type: report of one case. 859 34

As other developed countries of the world, obesity has become a major health problem of children and adolescents in Taiwan. According to an island-wide survey, the prevalence of obesity varied from 4.3% to 17.4% in children aged three to nineteen years. Obesity is defined as an excess of body fat. Since the standard references of weight-for-height and body mass index for Taiwanese are not available, a weight-for-length index has been introduced. We have found that its easy computation and interpretation with high reliability make it a desirable measure of relative weight either in clinical practice or in research. The family background of the obese children has been investigated by many workers. In our own series, the obese children were about five times as likely to have an obese parent as control. There was also a significant difference in the chance of having an obese sibling between the obese and control groups. Several health sequelae of childhood obesity have been studied in Taiwan. Obese children appear to have high prevalence of hypertension, hyperlipidemia, and abnormal glucose metabolism. Of several cutaneous manifestations of obesity, colored striae were sometimes overlooked. In our experience, 40% of the moderate to severe obese cases have such striking marks prominently located on the thighs, arms and abdomen. A few pediatric weight-losing programs have been carried out in Taiwan. In our Pediatric Anti-Obesity Club, four fundamental components: diet, exercise, behavior modification and parent involvement were included in the treatment program. In family-based parent-directed program, 11% of the participants showed decreases in degree of obesity after one year follow-up, compared to 3% of a control group. In another individualized outpatient counseling clinic, the success rate was 59% after one year. Although the long-term effectiveness cannot determined, it seems rational that a realistic and culturally sensitive weight reduction program should be developed for countries, such as Taiwan, with increasing prevalence of childhood obesity.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Childhood obesity in Taiwan. 947 15

Cyclosporin A (CsA) is an immunosuppressant that inhibits protein phosphatase 2B (PP2B/calcineurin) and is associated with hyperlipidemia, decreased cholesterol efflux via ATP-binding cassette transporter A1 (ABCA1), and increased risk of atherosclerosis. Apolipoprotein E (apoE) is an important regulator of lipid metabolism and atherosclerosis, the secretion of which from human macrophages is regulated by the serine/threonine protein kinase A (PKA) and intracellular calcium (Ca(2+)) (Kockx, M., Guo, D. L., Huby, T., Lesnik, P., Kay, J., Sabaretnam, T., Jary, E., Hill, M., Gaus, K., Chapman, J., Stow, J. L., Jessup, W., and Kritharides, L. (2007) Circ. Res. 101, 607-616). As PP2B is Ca(2+)-dependent and has been linked to PKA-dependent processes, we investigated whether CsA modulated apoE secretion. CsA dose- and time-dependently inhibited secretion of apoE from primary human macrophages and from Chinese hamster ovary cells stably transfected with human apoE and increased cellular apoE levels without affecting apoE mRNA. [(35)S]Met kinetic modeling studies showed that CsA inhibited both secretion and degradation of apoE, increasing the half-life of cellular apoE 2-fold. CsA also inhibited secretion from primary human Tangier disease macrophages and from mouse macrophages deficient in ABCA1, indicating that the effect is independent of the known inhibition of ABCA1 by CsA. The role of PP2B in mediating apoE secretion was confirmed using additional peptide and chemical inhibitors of PP2B. Importantly, kinetic modeling, live-cell imaging, and confocal microscopy all indicated that CsA inhibited apoE secretion by mechanisms quite distinct from those of PKA inhibition, most likely inducing accumulation of apoE in the endoplasmic reticulum compartment. Taken together, these results establish a novel mechanism for the pro-atherosclerotic effects of CsA, and establish for the first time a role for PP2B in regulating the intracellular transport and secretion of apoE.
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PMID:Cyclosporin A decreases apolipoprotein E secretion from human macrophages via a protein phosphatase 2B-dependent and ATP-binding cassette transporter A1 (ABCA1)-independent pathway. 1958 83