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Fat Malabsorption (fat + malabsorption)

Distribution by Scientific Domains

Medical Sciences	75%

Selected Abstracts

Ataxia with isolated vitamin E deficiency: A clinical, biochemical and genetic diagnosis

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 5 2000
G Alex
Abstract: A case of ataxia with isolated vitamin E deficiency, in conjunction with supportive genetic studies, is reported. This is a neurodegenerative condition that involves a mutation in the tocopherol (,) transfer protein gene (TTPA). Measurement of serum vitamin E concentration should be included as part of the investigations in children with progressive ataxia, even in the absence of fat malabsorption. Early treatment with vitamin E may protect such patients against further neurological damage. [source]

Systematic review: efficacy and safety of pancreatic enzyme supplements for exocrine pancreatic insufficiency

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 1 2010
J. R. TAYLOR
Summary Background, Pancreatic enzyme supplements are standard therapy for fat malabsorption in patients with exocrine pancreatic insufficiency. The FDA determined that published data are insufficient to support the efficacy and safety of these agents. Aim, To determine if pancreatic enzyme supplements are: (i) superior to placebo for treating fat malabsorption and (ii) superior to other supplements based on randomized cross-over trials. Methods, A computer-assisted search of MEDLINE and EMBASE was performed to identify relevant studies. Data extraction on study design, improvement in coefficient of fat absorption, diarrhoea and adverse events using prespecified forms. Results, A total of 12 manuscripts met inclusion criteria. Most studies (10/12) compared pancreatic enzyme supplements that used different delivery systems, while using similar quantities of enzymes. These studies found no consistent difference in fat malabsorption or gastrointestinal symptoms between different active treatments. Two small placebo-controlled trials (n = 65 patients) demonstrate that pancreatic enzyme supplements are superior to placebo for fat absorption. Data are inadequate to determine if pancreatic enzyme supplements lead to weight gain or improvement in diarrhoea. Conclusions, Based on data from randomized cross-over trials, pancreatic enzyme supplements appear to improve fat malabsorption. No specific branded product or specific delivery system is superior for treatment of fat malabsorption in patients with exocrine pancreatic insufficiency. [source]

Systematic review: pancreatic enzyme treatment of malabsorption associated with chronic pancreatitis

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 3 2009
A. K. WALJEE
Summary Background, Pancreatic enzyme supplementation is standard treatment for malabsorption caused by chronic pancreatitis. The FDA recently required all manufacturers to submit New Drug Applications to continue to market these agents because published data demonstrated variation in formulation, bioavailability and shelf-life while providing limited data about efficacy and safety. Aim, To review systematically the design and results of randomized, parallel-design trials of pancreatic enzyme supplements in chronic pancreatitis patients with steatorrhea. Methods, A computer-assisted search of MEDLINE and EMBASE was performed to identify relevant studies. Two authors performed duplicate data extraction on study design, improvement in coefficient of fat absorption (CFA), diarrhoea and adverse events using pre-specified forms. Agreement between investigators for data extraction was greater than 95%. Results, Of 619 articles found through literature searching, 20 potentially relevant articles were identified and four manuscripts met inclusion criteria. No studies performed head-to-head comparisons of different supplements. Enzyme supplementation is more likely to improve CFA compared with placebo, but fat malabsorption remained abnormal. Important differences in patient population, study endpoint, study design, pancreatic enzyme dosage and measurement of CFA were present across trials, which precluded comparison of different agents. Conclusions, Enzyme supplementation improves CFA compared to placebo, but may not abolish steatorrhoea. [source]

Variable phenotypic expression of homozygous familial hypobetalipoproteinaemia due to novel APOB gene mutations

CLINICAL GENETICS, Issue 3 2008
E Di Leo
Homozygous familial hypobetalipoproteinaemia (Ho-FHBL) is a rare co-dominant disorder characterized by extremely low levels of low-density lipoprotein cholesterol (LDL-C) and apolipoprotein B (apoB). Most patients with Ho-FHBL have mutations in APOB gene resulting in truncated apoBs. Some patients are asymptomatic, while others have fatty liver, intestinal fat malabsorption and neurological dysfunctions. We investigated three adult subjects with severe hypobetalipoproteinaemia and a family history of FHBL. Proband FHBL-47 had liver cirrhosis with hepatocarcinoma and a renal carcinoma but no clinical manifestations related to FHBL. He was a compound heterozygote for a 7-bp deletion in exon 21 and a base insertion in exon 26 resulting in truncated apoBs (apoB-22.46/apoB-66.51). Proband FHBL-53, with severe hepatic steatosis and fibrosis, had a nonsense mutation in exon 19 resulting in a truncated apoB (apoB-20.61) and a rare nucleotide substitution in intron 14 (c.2068-4T>A). The latter was also present in her daughter, found to have low plasma LDL-C and apoB. Proband FHBL-82 had chronic diarrhoea and steatorrhoea. She was found to be homozygous for a nonsense mutation in exon 24 resulting in a truncated apoB (apoB-26.65). In adult subjects, the presence of chronic liver disease and chronic diarrhoea, when associated with severe hypobetalipoproteinaemia, may lead to the diagnosis of Ho-FHBL. [source]