Cases reported "Metabolic Syndrome X"

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1/24. Detection of coronary microvascular disease by means of cardiac scintigraphy.

    A 76-year-old woman strictly defined as having cardiac syndrome X underwent cardiac scintigraphies. A reversible perfusion abnormality was identified by (201)thallium in the inferior segment of the left ventricle. (123)iodine meta-iodo-benzyl-guanigine uptake showed extremely diminished uptake in the inferior segment of the myocardium. (123)iodine labelled beta-methyl-iodophenyl pentadecanoic acid myocardial single photon emission computed tomography showed decreased uptake of the inferior segment in the early image, whereas the delayed images revealed significant fill-in of the tracer in the inferior segment of the myocardium. These findings suggest that coronary microvascular dysfunction causes regional myocardial ischemia, resulting in metabolic and sympathetic abnormality. ( info)

2/24. Dysmetabolic syndrome: multiple risk factors for premature adult disease in an adolescent girl.

    The clinical diagnosis of dysmetabolic syndrome in an adult defines a patient with abnormal glucose metabolism (or diabetes), hypertension, hyperlipidemia, and obesity. This disorder accelerates atherosclerosis and significantly raises the risk for cardiovascular events. With the marked rise in the prevalence of obesity in childhood, obesity-linked risk factors are being expressed at young ages. The case of a 12-year-old girl with dysmetabolic syndrome is described and discussed. Emerging clinical data now indicate that the presence of 1 risk factor for cardiovascular disease in an overweight child should prompt screening for additional clinical abnormalities, with the aim of finding treatable disorders. ( info)

3/24. A review of the literature of Bardet-Biedl disease and report of three cases associated with metabolic syndrome and diagnosed after the age of fifty.

    bardet-biedl syndrome (BBS) is a genetic autosomal-recessive disease (formerly grouped with Laurence-moon-Biedl syndrome but considered today as a separate entity) characterized by abdominal obesity, mental retardation, dysphormic extremities (syndactyly, brachydactyly or polydactyly), retinal dystrophy or pigmentary retinopathy, hypogonadism or hypogenitalism (limited to male patients) and kidney structural abnormalities or functional impairment. The expression and severity of the various clinical BBS features show inter- and intrafamilial variability. This study focuses on three cases of familial BBS--two sisters and one brother (66, 64 and 51 years of age, respectively)--with the main cardinal findings of the disease plus a classic 'metabolic syndrome' (characterized by abdominal obesity, atherogenic dyslipidaemia, raised blood pressure, insulin resistance with or without glucose intolerance, and prothrombotic risk and proinflammatory states). One female patient (not affected by reproductive dysfunction) had three healthy offspring, while the other two patients were unmarried. Another severely affected brother died at 70 years of age; two other brothers are lean but affected by nephropathy, retinopathy, slight mental retardation, polydactyly, hypertension and thrombotic diseases, and had healthy offspring. BBS is a rather rare but severe syndrome that is often mis- or undiagnosed. Ophthalmologists, endocrinologists and nephrologists should be aware of BBS because of its adverse prognosis--early onset of blindness, associated findings of metabolic syndrome and increased vascular risk, and severe renal impairment (the most frequent cause of reduced survival and death early in life). ( info)

4/24. The metabolic syndrome, type 2 diabetes, and cardiovascular disease: understanding the role of insulin resistance.

    The most common and clinically important complication in adults with diabetes is cardiovascular disease (CVD), which includes coronary heart disease, peripheral vascular disease, and stroke. Both type 2 diabetes and the insulin resistance syndrome are associated with a marked increase in the risk for CVD. The metabolic syndrome and the closely related insulin resistance syndrome have recently been recognized as important disorders, each being associated with an increase in CVD risk even in the absence of glucose intolerance. Given the significant public health burden of CVD, risk reduction has emerged as a significant clinical challenge for most practitioners. Diabetes and the insulin resistance syndrome are closely related disorders, with insulin resistance being more than a key pathogenic defect in type 2 diabetes. Even in the absence of glucose intolerance, these 2 disorders are both associated with a number of distinct pathologic findings, including hypertension, atherogenic dyslipidemia, a prothrombotic environment, and significant vascular and hemodynamic abnormalities that result from endothelial cell dysfunction. insulin resistance is now recognized to be closely associated with the development of each of these risk factors. This article uses a case-based approach to discuss the unique features of insulin resistance and type 2 diabetes considered to be key contributors to CVD risk. A systematic approach to both evaluation and management is proposed, with priority given to therapies of demonstrated clinical benefit. Because of its critical and central role in the development of many CVD risk factors, targeted treatment of insulin resistance will also be discussed as such therapy may prove to be a critical component of care in years to come. ( info)

5/24. recurrence of insulin resistant metabolic syndrome following liver transplantation.

    Insulin resistant metabolic syndrome is a major clinical disorder including hyperlipidaemia, hypertension, impaired glucose tolerance and/or type 2 diabetes and central obesity, which are well established cardiovascular risk factors. We report the case of a 61-year-old woman who developed severe hypercholesterolaemia and hypertriglyceridaemia after liver transplantation. In her forties she had hypertension, mixed hyperlipidaemia, mild hyperglycaemia and moderate abdominal obesity, suggesting the presence of the metabolic syndrome. She had liver enzyme elevation and severe steatosis and hepatomegaly at ultrasonography. At age 52, cryptogenic liver cirrhosis was diagnosed and rapidly progressing liver failure developed. In 1992 she underwent liver transplantation. Seven years after transplant the patient had abdominal obesity, high blood pressure, marked hypercholesterolaemia, hypertriglyceridaemia and moderate elevation of alanine aminotransferase. She also had impaired glucose tolerance and markedly increased basal and post-glucose load plasma insulin levels. Steatohepatitis was demonstrated by serial liver biopsies. This is the first case that reports the recurrence of the metabolic syndrome following liver transplantation. We postulate that metabolic syndrome may have promoted fatty liver and subsequent progression to end stage liver disease. We also stress the need for careful management of the metabolic syndrome in order to decrease the long-term risk for cardiovascular disease. ( info)

6/24. obesity and the metabolic syndrome.

    The prevalence of marked obesity is increasing rapidly among adults and has more than doubled in 10 years. Sixty-one percent of the adult population of the united states is overweight or obese. Americans are the fattest people on earth. Paradoxically these increases in the numbers of persons who are obese or overweight have occurred during recent years when Americans have been preoccupied with numerous dietary programs, diet products, weight control, health clubs, home exercise equipment, and physical fitness videos, each "guaranteed" to bring rapid results. overweight and obesity are also world problems. The world health organization estimates that 1 billion people around the world are now overweight or obese. Westernization of diets has been part of the problem. Fruits, vegetables, and whole grains are being replaced by readily accessible foods high in saturated fat, sugar, and refined carbohydrates. Since class 3 obesity (morbid or extreme obesity) is associated with the most severe health complications, the incidence of hypertension, stroke, heart disease, diabetes, and peripheral vascular disease will increase substantially in the future. Recently, obesity alone has been implicated in the development of cardiac hypertrophy and CHF. The metabolic syndrome associated with abdominal obesity, which includes insulin resistance, dyslipidemia, and elevated CRP levels, identifies subjects who have an increase in cardiovascular morbidity and mortality. Twenty to 25% of the adult population in the united states have the metabolic syndrome, and in some older groups this prevalence approaches 50%. The prevalence of overweight children in the united states has also been increasing dramatically, especially among non-Hispanic blacks and Mexican-American adolescents. overweight children usually become overweight adults. atherosclerosis begins in childhood. The degree of atherosclerotic changes in children and young adults can be correlated with the presence of the same risk factors seen in adults. As health providers, our direction is obvious! ( info)

7/24. Human metabolic syndrome resulting from dominant-negative mutations in the nuclear receptor peroxisome proliferator-activated receptor-gamma.

    We previously reported a syndrome of severe hyperinsulinemia and early-onset hypertension in three patients with dominant-negative mutations in the nuclear hormone receptor peroxisome proliferator-activated receptor (PPAR)-gamma. We now report the results of further detailed pathophysiological evaluation of these subjects, the identification of affected prepubertal children within one of the original families, and the effects of thiazolidinedione therapy in two subjects. These studies 1) definitively demonstrate the presence of severe peripheral and hepatic insulin resistance in the affected subjects; 2) describe a stereotyped pattern of partial lipodystrophy associated with all the features of the metabolic syndrome and nonalcoholic steatohepatitis; 3) document abnormalities in the in vivo function of remaining adipose tissue, including the inability of subcutaneous abdominal adipose tissue to trap and store free fatty acids postprandially and the presence of very low circulating levels of adiponectin; 4) document the presence of severe hyperinsulinemia in prepubertal carriers of the proline-467-leucine (P467L) PPAR-gamma mutation; 5) provide the first direct evidence of cellular resistance to PPAR-gamma agonists in mononuclear cells derived from the patients; and 6) report on the metabolic response to thiazolidinedione therapy in two affected subjects. Although the condition is rare, the study of humans with dominant-negative mutations in PPAR-gamma can provide important insight into the roles of this nuclear receptor in human metabolism. ( info)

8/24. diabetes mellitus type 2 in aviators: a preventable disease.

    INTRODUCTION: The current epidemic of obesity and resultant diabetes mellitus type 2 (DMT2) is a tsunami that will impact healthcare worldwide and lap over into aerospace medicine. Metabolic syndrome (MBS) is the major link between obesity and DMT2. methods: A review of U.S. Air Force Aeromedical Consult Service (ACS) records was accomplished looking at aviators with a diagnosis of DMT2. case reports of three flyers with DMT2 are presented and discussed. Other aeromedical agencies were contacted regarding their experiences and this information was summarized. A literature review on DMT2, obesity, and metabolic syndrome was accomplished. RESULTS: Of 70 charts for flyers identified with diabetes mellitus at the ACS between 1975 and 2000, over 95% were for DMT2. The mean body mass index for these aviators was 26.2. Currently, all services grant restricted waivers for some aviators with DMT2, none in high performance, single-seat aircraft. The FAA is currently allowing most flyers with stable DMT2 to operate aircraft in all categories with specific restrictions. DISCUSSION: obesity and metabolic syndrome are becoming increasingly prevalent in the aviation community. Aggressive actions to limit weight gain and identify those at risk for developing DMT2 must be considered for all populations. ( info)

9/24. Metabolic syndrome.

    The metabolic syndrome is characterized by diabetes mellitus, obesity, hypertension, hyperlipidaemia and polycystic ovary syndrome. The lipid profiles of patient with metabolic syndrome is often characterized by the appearance of hypertrygliceridaemia and small, dense LDL-cholesterol, together with low HDL-cholesterol. patients with these abnormalities are at an increased risk for premature coronary artery disease. Treatment is a multifactorial process and includes modification of lifestyle factors such as diet and physical activity, weight reduction, correction of dyslipidemia, meticulous blood pressure and glycemic control. The case of a 36-year-old woman who develops metabolic syndrome is discussed. ( info)

10/24. Unilateral adrenalectomy improves insulin resistance and diabetes mellitus in a patient with ACTH-independent macronodular adrenal hyperplasia.

    ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered to be a standard therapy for AIMAH, although lifetime replacement of glucocorticoids is necessary after the procedure. This paper describes a subject with AIMAH who underwent unilateral adrenalectomy of the predominantly enlarged gland and subsequently displayed an improvement in insulin resistance and diabetes mellitus, the cardinal symptoms before the operation, concomitant with alleviation of abnormal cortisol secretion. The patient was a 61-year-old man with a body mass index of 25.6 kg/m2. He was diagnosed as having diabetes mellitus, hypertension, and hyperlipidemia at 50 years of age. Eight years after diagnosis, bilateral enlargement of the adrenal glands was revealed by chance upon computed tomography of the abdomen. Typical manifestations of Cushing's syndrome were not demonstrated. Basal levels of serum and urinary cortisol had not increased, although the serum cortisol level displayed no circadian rhythm and no response to the administration of dexamethasone. Despite sulfonylurea treatment, the patient's HbA1C level was as high as 7.6% (normal range 4.3-5.8%). fasting insulin concentration was increased to 42.6 microU/ml, and the homeostasis model insulin resistance index (HOMA-R) was calculated to be 15.5 (with a normal range of less than 2.5), indicating severe insulin resistance. Unilateral adrenalectomy of the predominantly enlarged gland revealed that the resected gland consisted of multiple nodules of various sizes. Based on endocrinological, radiological, and pathological findings, a diagnosis of AIMAH was made. Ten months after the unilateral adrenalectomy, cortisol circadian rhythms were restored, and serum cortisol concentration was suppressed in response to the administration of low doses of dexamethasone, suggesting an improvement in the cortisol secretory pattern. Levels of HbA1C, fasting insulin, and HOMA-R decreased to 5.7%, 12.7 microU/ml, and 2.2, respectively. An improvement in hyperlipidemia was also observed. insulin resistance and glucose intolerance are recognized as features of mild hypercortisolism. In the present case, unilateral adrenalectomy was effective in ameliorating insulin resistance and improving glycemic control. Unilateral adrenalectomy might be an alternative therapy for improvement of glucose and lipid metabolism in subjects with AIMAH. ( info)
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