myotonic dystrophy/triglyceride

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The direct relationship of proinsulin-insulin hypersecretion to basal serum levels of cholesterol and triglyceride in myotonic dystrophy.

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Excessive pancreatic insulin secretory responses to a variety of beta-cytotropic agents have been observed in 40 to 60 per cent of subjects with myotonic dystrophy; in addition, 25 to 30 per cent of affected persons manifest mild glucose intolerance, suggesting that circulating insulin may be

Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction: Detection by Cardiovascular Magnetic Resonance.

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Myotonic dystrophy type 2 (DM2) is a genetic disorder characterized by skeletal muscle symptoms, metabolic changes, and cardiac involvement. Histopathologic alterations of the skeletal muscle include fibrosis and fatty infiltration. The aim of this study was to investigate whether subclinical

The pleiotropic expression of the myotonic dystrophy protein kinase gene illustrates the complex relationships between genetic, biological and clinical covariates of male aging.

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Aging is a complex process modulated by multiple interactions between environmental and genetic factors. Myotonic dystrophy (DM1) is an autosomal dominant disorder caused by an unstable (CTG)n repeat expansion in the DM1 protein kinase (DMPK) gene. The affected male patients' life expectancy at

[Intra-abdominal visceral fat in myotonic dystrophy].

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We first clarified the relationship between abdominal visceral fat accumulation and impaired glucose and lipid metabolism in patients with myotonic dystrophy. Nineteen patients aged 44.2 +/- 11.4 years with a body mass index (BMI) of 20.5 +/- 2.8 kg/m2, and 18 controls aged 45.2 +/- 10.3 years, with

Tumor necrosis factor system activity is associated with insulin resistance and dyslipidemia in myotonic dystrophy.

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Myotonic dystrophy (MyD) is a multisystem autosomal dominant disorder associated with progressive muscle wasting and weakness. The striking metabolic abnormality in MyD is insulin resistance. The mechanism by which target tissues are insensitive to insulin action remains uncertain. In a recent

[Changes in brain stem potentials in dystrophia myotonica].

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In 15 patients with dystrophia myotonica brainstem auditory potentials (BAEP) were examined: in 8 patients (53%) pathological components in the BAEP's (such as increased latency of one peak) and in 80% a pathologic component in the neurography could be found. Comparing the latencies of the peak and

Open-label trial of recombinant human insulin-like growth factor 1/recombinant human insulin-like growth factor binding protein 3 in myotonic dystrophy type 1.

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OBJECTIVE To evaluate the safety and tolerability of recombinant human insulin-like growth factor 1 (rhIGF-1) complexed with IGF binding protein 3 (rhIGF-1/rhIGFBP-3) in patients with myotonic dystrophy type 1 (DM1). METHODS Open-label dose-escalation clinical trial. METHODS University medical

Hypertriglyceridemia and lower LDL cholesterol concentration in relation to apolipoprotein E phenotypes in myotonic dystrophy.

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Plasma lipid, lipoprotein levels and apolipoprotein apo E phenotypes were determined in 70 patients with myotonic dystrophy (MyD) and 81 controls. Marked differences were noticed in the apo E phenotype frequencies between the two groups. Plasma triglycerides and VLDL cholesterol were higher in MyD

Peripheral neuropathy in patients with myotonic dystrophy type 1.

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OBJECTIVE To assess the frequency and type of peripheral neuropathy (PNP) in patients with myotonic dystrophy type 1 (DM1), as well as to identify factors that may be associated with this abnormality. METHODS This study comprised 111 adult patients with DM1. Nerve conduction study was performed on

Increased levels of tPA antigen and tPA/PAI-1 complex in myotonic dystrophy.

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OBJECTIVE To assess the fibrinolytic system in myotonic dystrophy (DM1), a disease connected to features of the metabolic syndrome, including a prominent insulin resistance, increased body fat mass, and hypertriglyceridaemia. We hypothesized that abnormalities in the fibrinolytic system are linked

Frequency and predictors of nonalcoholic fatty liver disease in myotonic dystrophy.

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Nonalcoholic fatty liver disease (NAFLD) is a common chronic liver disease that is strongly associated with insulin resistance. Myotonic dystrophy (DM1) is the most common form of adult-onset muscular dystrophy, and there is a high frequency of insulin resistance due to insulin receptor mRNA

Decreased concentration of adiponectin together with a selective reduction of its high molecular weight oligomers is involved in metabolic complications of myotonic dystrophy type 1.

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OBJECTIVE The hormone adiponectin exerts beneficial pleiotropic effects on biological and metabolic processes. Although a well-recognized insulin sensitizer, its characteristic has yet to be clearly defined. Myotonic dystrophy type 1 (DM1) is a rare genetic disorder that features muscle wasting and

Development and Clinical Applications of Antisense Oligonucleotide Gapmers

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DNA-like molecules called antisense oligonucleotides have opened new treatment possibilities for genetic diseases by offering a method of regulating gene expression. Antisense oligonucleotides are often used to suppress the expression of mutated genes which may interfere with essential downstream

Alterations in lipid incorporation in Duchenne muscular dystrophy. Studies of fresh and cultured muscle.

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The incorporation of [3H] glycerol into lipids of fresh and cultured skeletal muscle obtained from patients with Duchenne muscular dystrophy (DMD), patients with myotonic dystrophy (My Dyst), controls, and aborted fetuses (10-12 weeks old) was studied. A significant increase of specific

The fatty acid composition of various lipid fractions isolated from erythrocytes and blood plasma of patients with Duchenne and congenital myotonic muscular dystrophy.

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Ten lipid fractions, both neutral and phospholipids, were isolateed from erythrocytes of patients with Duchenne and congenital myotonic dystrophy. These fractions were: phosphatidylethanolamine, phosphatidylcholine, phosphatidylserine, sphingomyelin, lysophosphatidylcholine, phosphatidic acid,

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