muscular atrophy/fedme

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Weight-Loss Cognitive-Behavioural Treatment and Essential Amino Acid Supplementation in a Patient with Spinal Muscular Atrophy and Obesity.

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Spinal muscular atrophy is a genetic neuromuscular disease characterised by muscle atrophy, hypotonia, weakness, and progressive paralysis. Usually, these patients display increased fat mass deposition and reductions in fat-free mass and resting energy expenditure-an unfavourable condition that

Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy.

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Body composition is sparsely described in spinal muscular atrophy (SMA). Body (BMI, mass/height in m(2)), fat-free (FFMI, lean mass/height in m(2)) and fat (FMI, fat mass/height in m(2)) mass indexes were estimated in 25 children (aged 5-18) with SMA (2 type I, 13 type II, 10 type III) using

Multimodal anesthesia management of a morbidly obese spinal muscular atrophy patient with obstructive sleep apnea.

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Musculoskeletal pain among postmenopausal women in Nigeria: Association with overall and central obesity.

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Menopausal women experience musculoskeletal changes such as muscle atrophy, muscle weakness and osteoporosis-symptoms associated with advancing age coupled with depletion of the female sex hormone, estrogen. Estrogen is important in the maintenance of the integrity of the

Correlation of insulin resistance and motor function in spinal and bulbar muscular atrophy.

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This study aimed to evaluate various metabolic parameters in patients with spinal and bulbar muscular atrophy (SBMA), to investigate the association between those indices and disease severity, and to explore the underlying molecular pathogenesis. We compared the degree of obesity, metabolic

Bioelectrical impedance analysis can be a useful screen for excess adiposity in spinal muscular atrophy.

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Accurate, noninvasive measures of body composition are needed for management of patients with spinal muscular atrophy. Fat mass index (fat mass/height(2) in kg/m(2)) was measured in 16 subjects with spinal muscular atrophy using 5 bioelectrical impedance analysis equations and compared with a

Characteristics of bone mineral density and soft tissue composition of obese Japanese women: application of dual-energy X-ray absorptiometry.

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We studied the characteristics of bone mineral density (BMD) and soft tissue composition in obese Japanese women using dual-energy X-ray absorptiometry. Eighty-nine women, aged 45-85 years, were divided into three groups according to their body mass index (BMI): a thin group (n = 38: BMI < 21), a

[Minimally invasive dorsal decompression-stabilization surgery in patients with overweight and obesity].

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Spinal surgery in patients with overweight and obesity is associated with an increased risk of perioperative complications. Minimally invasive (MIS-TLIF) and traditional (O-TLIF) techniques of rigid stabilization are extensively used, but the advantages and disadvantages of MIS-TLIF in patients with

Exercise Therapy in Spinobulbar Muscular Atrophy and Other Neuromuscular Disorders.

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There is no curative treatment for most neuromuscular disorders. Exercise, as a treatment for these diseases, has therefore received growing attention. When executed properly, exercise can maintain and improve health and reduce the risk of cardiovascular disease, obesity, and diabetes. In persons

Hyperleptinemia in children with autosomal recessive spinal muscular atrophy type I-III.

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BACKGROUND Autosomal-recessive proximal spinal muscular atrophies (SMA) are disorders characterized by a ubiquitous deficiency of the survival of motor neuron protein that leads to a multisystemic disorder, which mostly affects alpha motor neurons. Disease progression is clinically associated with

Responses to Fasting and Glucose Loading in a Cohort of Well Children with Spinal Muscular Atrophy Type II.

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OBJECTIVE To examine the impact of fasting and glucose tolerance on selected metabolic variables in children with spinal muscular atrophy (SMA) type II in a well state, secondary to reports of glucose regulation abnormalities in SMA. METHODS In this prospective pilot study, 6 children aged 7-11

Feeding the critically ill obese patient: a systematic review protocol.

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UNASSIGNED The objective of this review is to identify effective enteral nutritional regimens targeting protein and calorie delivery for the critically ill obese patient on morbidity and mortality.More specifically, the review question is:In the critically ill obese patient, what is the optimal

The endocrine manifestations of spinal muscular atrophy, a real-life observational study.

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The introduction of nusinersen, the first therapeutic modality for Spinal Muscular Atrophy (SMA) patients has raised hopes and led to construction of a multi-professional medical SMA service, including pediatric endocrinology. Our study aimed to provide a comprehensive description of the endocrine

TIA1 is a gender-specific disease modifier of a mild mouse model of spinal muscular atrophy.

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Spinal muscular atrophy (SMA) is caused by deletions or mutations of Survival Motor Neuron 1 (SMN1) gene. The nearly identical SMN2 cannot compensate for SMN1 loss due to exon 7 skipping. The allele C (C +/+) mouse recapitulates a mild SMA-like phenotype and offers an ideal system to monitor the

Adiposity is increased among high-functioning, non-ambulatory patients with spinal muscular atrophy.

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The relationship between body composition and function in spinal muscular atrophy (SMA) is poorly understood. 53 subjects with SMA were stratified by type and Hammersmith functional motor scale, expanded score into three cohorts: low-functioning non-ambulatory (type 2 with Hammersmith score < 12,

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