Familial Chylomicronemia Syndrome: A Clinical Guide For Endocrinologists.

Objective: Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disorder caused by mutations in lipoprotein lipase, resulting in accumulation of chylomicrons in plasma and hypertriglyceridemia. Elevated triglycerides cause several complications in patients, the most serious being episodes of acute pancreatitis. This review focuses on expert guidance and opinion from an experienced lipidologist and endocrinologist as well as a current review of the literature, as there are no specific guidelines on FCS.

The role of lipoprotein(a) in clotting reactions during lipoprotein apheresis--A case report.

In individuals with familial hypercholesterolemia (FH) who are unable to reach a target low-density lipoprotein level on a drug regimen, lipoprotein apheresis (LA) may be the treatment of choice. Severe reactions involving clotting during LA are not well described in the literature. We report a case of a 63-year-old woman with FH and markedly elevated lipoprotein(a) (Lp[a]) levels who experienced such a reaction while undergoing LA with a dextran-sulfate cellulose column on the Kaneka MA-01 Liposorber system.

New treatments on the horizon for familial hypercholesterolemia.

Patients with familial hypercholesterolemia (FH) have higher baseline LDL cholesterol (LDLc) levels and are at high risk of developing premature cardiovascular disease. Disease is attributed to mutations in the LDLR gene, which encodes the LDL receptor protein and whose deficiency results in decreased uptake of apoB-containing cholesterol particles by the liver and elevated serum LDLc levels. Heterozygous FH is inherited in an autosomal-dominant pattern and has an incidence of 1:500 in the general population.

Randomized trial assessing the impact of a musculoskeletal intervention for pain before participating in a weight management program.

Purpose: Obesity increases the risk of developing physical disability and pain. Persons with a body mass index (BMI) of 30 kg/m or more have an increased risk for osteoarthritis compared with those with a BMI between 25 and 29 kg/m. The purpose of this study was to examine the effect of treatment directed at reducing musculoskeletal pain on weight loss in obese subjects prior to participation in a 6-month weight management (WM) program.

Dyslipidemia in South Asians living in a western community.

An increased prevalence of coronary heart disease (CHD) has been well documented in the South Asian population living worldwide. The prevalence of certain traditional CHD risk factors, like diabetes mellitus and tobacco use, have been on the rise in this ethnic group and likely contribute to the increase in CHD prevalence. Still, a disproportionate excess of CHD exists, and this may be linked to novel CHD risk factors.

Optimal management of lipids in diabetes and metabolic syndrome.

Patients with diabetes or metabolic syndrome frequently have higher triglycerides, lower high-density lipoprotein (HDL) cholesterol, and more particles containing apolipoprotein B (ApoB); this combination contributes significantly to their cardiovascular risk. Optimal management of dyslipidemia and increased atherosclerotic risk requires a fundamental understanding of diabetic dyslipidemia, the clinical evidence for different interventional strategies, and the potential benefit of achieving therapeutic targets. For this review, we considered guidelines, recent reviews, and clinical trial results.

Effect of a weight management program on the determinants and prevalence of metabolic syndrome.

Objective: As prevalence of obesity and metabolic syndrome (met synd) rises, establishing effective, community-based treatments is imperative. Our investigation sought to evaluate and report the effect of a weight management program on the prevalence and determinants of met synd, and the effect of participation level.

Methods And Procedures: Between 10 July 2001 and 17 November 2005, 339 of 574 individuals enrolled in and completed our 6-month weight management program at the McConnell Heart Health Center in Columbus, Ohio.

Cardiovascular disease risk of type 2 diabetes mellitus and metabolic syndrome: focus on aggressive management of dyslipidemia.

Type 2 diabetes mellitus and the closely related metabolic syndrome markedly increase the risk of cardiovascular disease a major contributor is the dyslipidemia. Recent studies and new national guidelines suggest these very high risk patients with cardiovascular disease achieve optional low density lipoprotein cholesterol (LDL-C) level of less than 70 mg/dl. In addition there may be no threshold to begin therapeutic lifestyle change and pharmacologic therapy to reduce LDL-C by 30-40%.

A systematic approach to improve lipids in coronary artery disease patients participating in a cardiac rehabilitation program.

Purpose: To determine the effectiveness of an intervention, directed toward the primary care physician (PCP), to improve the number of patients treated to low-density lipoprotein cholesterol (LDL-C) goal in a cardiac rehabilitation (CR) population.

Methods: A pre-post intervention cohort comparison using data collected from participants in a CR program with LDL-C > or =100 mg/dL at entry. The control cohort participated in CR between 1/00 and 10/02, 41.

Effect of a community-based weight management program on weight loss and cardiovascular disease risk factors.

Objective: The purpose of our retrospective database analysis was to describe and evaluate the outcomes of a weight loss intervention in a community medical wellness center.

Research Methods And Procedures: Four hundred eighteen overweight and obese adults entered the program between 2001 and 2004. Forty-seven percent completed the 6-month program designed using standards and recommendations established by the NIH, the American Dietetic Association, and the American Academy of Sports Medicine.

Cardiac rehabilitation is associated with an improvement in C-reactive protein levels in both men and women with cardiovascular disease.

Purpose: Inflammation is involved in the development of atherosclerotic plaque. The most studied indicator of inflammation in coronary heart diseases (CHD) is C-reactive protein (CRP) which has prognostic significance in those with CHD. The purpose of this study is to evaluate the effect of participation in cardiac rehabilitation (CR) on this marker of vascular inflammation, CRP.

Clinical profile and outcomes of diabetic and nondiabetic patients in cardiac rehabilitation.

Background: Patients with diabetes mellitus have increased risk of cardiovascular disease; however, there are limited data addressing cardiac rehabilitation in these patients. This study assessed the effectiveness of participation in cardiac rehabilitation on clinical outcomes after myocardial infarction and/or revascularization procedures in diabetic and nondiabetic patients.

Methods: Analysis on 1505 patients completing a minimum of 7 weeks of a 12-week cardiac rehabilitation program included fasting lipid profile and glucose, body mass index, and metabolic equivalent time in patients with diabetes (n = 292) and without diabetes (n = 1213).

Independent effect of cardiac rehabilitation on lipids in coronary artery disease.

Purpose: We evaluate the effects cardiac rehabilitation (CR) participation independent of using lipid-altering agents (LAAs) on low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, non-high-density lipoprotein cholesterol, total cholesterol, and triglyceride. Measurements included absolute and relative change in lipids and increases in percent of patients achieving goals.

Methods: Analysis of 766 patients who participated in CR between 2000 and 2003 was performed.

Evaluation and prevention of diabetic neuropathy.

Diabetic neuropathy is a debilitating disorder that occurs in nearly 50 percent of patients with diabetes. It is a late finding in type 1 diabetes but can be an early finding in type 2 diabetes. The primary types of diabetic neuropathy are sensorimotor and autonomic.

Unusual manifestations of cushing's syndrome in a multiple endocrine neoplasia type I kindred.

Objective: To describe two unusual cases of Cushing's syndrome in a kindred with multiple endocrine neoplasia type I (MEN I).

Methods: The clinical and laboratory data as well as follow-up course of the two patients are reviewed in detail.

Results: Our first patient with a history of MEN I had hypercortisolism and computed tomographic evidence of a right adrenal mass (8.

Therapeutic approaches to dyslipidemia in diabetes mellitus and metabolic syndrome.

Type 2 diabetes mellitus and the closely related metabolic syndrome are associated with significant risk for cardiovascular disease. Recent evidence suggests that both conditions are increasing in epidemic proportions. Dyslipidemia is characterized by increased triglyceride-rich lipoproteins; low high-density lipoprotein cholesterol; small, dense low-density lipoprotein particles; increased postprandial lipemia; and abnormal apolipoprotein A1 and B metabolism.

Acute psychological stress reduces plasma triglyceride clearance.

Acute stress elevates blood lipids, with the largest increases among men and postmenopausal women. The mechanisms for the effect are unknown, but may be due to altered lipid metabolism. This study investigated if acute stress induces transient reductions in triglyceride clearance in middle-aged men and women, and determined if gender and menopause affect triglyceride metabolism.

Metastatic pure papillary thyroid carcinoma presenting as a toxic hot nodule.

In the majority of cases, carcinoma of the thyroid presents as a cold nodule by radioiodine and Tc-99m sodium pertechnetate scintigraphy. Whereas the presence of a hot nodule usually implies a benign entity, it does not provide complete assurance against thyroid malignancy. Presented is a rare case of metastatic pure papillary thyroid carcinoma appearing as a hot nodule on Tc-99m sodium pertechnetate and I-123 sodium iodide scintigraphy.

Inhibition of low-density lipoprotein oxidation by oral herbal mixtures Maharishi Amrit Kalash-4 and Maharishi Amrit Kalash-5 in hyperlipidemic patients.

Low-density lipoprotein (LDL) oxidation is central to the pathogenesis of atherosclerosis. We have shown previously that the herbal mixtures Maharishi Amrit Kalash-4 (MAK-4) and Maharishi Amrit Kalash-5 (MAK-5) inhibit LDL oxidation induced by cupric ions (Cu+2) and endothelial cells in vitro and that MAK-4 reduces atherosclerosis in Watanabe heritable hyperlipidemic rabbits that were fed this herbal mixture. This study evaluates the antioxidant activity of MAK-4 and MAK-5 in vivo.

Both hypothyroidism and hyperthyroidism enhance low density lipoprotein oxidation.

Hypothyroidism is frequently associated with hypercholesterolemia and an increased risk for atherosclerosis, whereas hyperthyroidism is known to precipitate angina or myocardial infarction in patients with underlying coronary heart disease. We have shown previously that L-T4 functions as an antioxidant in vitro and inhibits low density lipoprotein (LDL) oxidation in a dose-dependent fashion. The present study was designed to evaluate the changes in LDL oxidation in subjects with hypothyroidism and hyperthyroidism.

RET mutation screening in MEN2 patients and discovery of a novel mutation in a sporadic medullary thyroid carcinoma.

RET germline mutations were found to predispose to the development of three variants of multiple endocrine neoplasia type 2, MEN2A, MEN2B, and familial medullary thyroid carcinoma (FMTC). We have screened for RET mutations at exons 10, 11, 13, and 16 in leukocyte DNA extracted from 37 individuals, and have identified RET germline mutations in 12 affected individuals from 9 unrelated families. No RET germline mutation was found in 19 individuals with apparent sporadic diseases.

Fibrosing pseudotumor of the sella and parasellar area producing hypopituitarism and multiple cranial nerve palsies.

We present an unusual patient with a medical history of a fibrosing pseudotumor of the left orbit that had been stable for 8 years who presented with acute anterior hypophyseal failure. During the next 10-month period, sequential magnetic resonance scans showed a rapid growth of a plaque-like sellar and parasellar mass extending into the right cavernous sinus, right Meckel's cave, along the dural surfaces of the clivus, dens, and body of the second cervical vertebra. A transsphenoidal biopsy revealed sphenoid and intrasellar pseudotumor that invaded the adenohypophysis and had microscopic features identical to those of the previously excised orbital pseudotumor.