Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 48-year-old woman with malabsorption and type V hyperlipoproteinemia developed hypervitaminosis A with a total plasma vitamin A level of 871 micrograms/dL during therapy with an oral dosage of 18,000 retinol equivalents (60,000 IU) daily. Twelve percent of the total plasma retinol was found to be transported in the chylomicron-very low density lipoprotein (VLDL) fraction, which does not contain retinol-binding protein. For comparison, concentrations of retinyl esters and retinol were determined in nine patients with type V hyperlipoproteinemia and nine control subjects, none of whom were using vitamin A supplements. Both retinyl esters and retinol were significantly elevated in the group with hyperlipoproteinemia (p less than 0.0005 in both cases). Eight of these nine patients had retinol present in the chylomicron-VLDL fraction, whereas retinol was not detectable in this fraction in any of the nine normal controls. The data suggest that patients with severe hypertriglyceridemia associated with type V hyperlipoproteinemia are at increased risk for hypervitaminosis A.
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PMID:Increased risk for vitamin A toxicity in severe hypertriglyceridemia. 377 11

The National Heart, Lung, and Blood Institute is currently sponsoring a multicenter clinical trial to evaluate the long-term efficacy of partial ileal bypass in the prevention of recurrent myocardial infarction in hypercholesterolemia patients. Thus we felt that a report of our clinical results with this intervention at the Montreal Heart Institute during the last 11 years would be of interest. Twenty patients with type II hyperlipoproteinemia and a mean age of 38 (range 25-54) years underwent partial ileal bypass between March 1971 and April 1978. This intervention was associated with aortocoronary bypass surgery in 11 patients. All patients were followed at regular intervals. The mean survival time was 70.7 (range 1-123) months. Two deaths were observed during follow-up, one from an acute myocardial infarction and the other from ventricular fibrillation, respectively, 1 month and 1 and one-half years after partial ileal bypass. The ileal bypass was undone twice because of gastrointestinal problems including a malabsorption syndrome and repeated episodes of subocclusion. A progressive decrease of the effects of the operation on serum cholesterol was noted, from a 33 per cent reduction at 3 months to 43 per cent at 2 years and 16 per cent at 6 years. Two patients presented an acute myocardial infarction respectively 3 and 4 years after the operation, respectively, and one patient suffered a right-sided hemiplegia at age 30, 12 months after the operation. Of 14 patients with angina pectoris preoperatively (class III in 10), eight remained symptomatic postoperatively (class I and II angina in five).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Partial ileal bypass in type II familial hypercholesterolemia. Eleven-year experience at the Montreal Heart institute. 636 76

Several vitamins have been demonstrated to interfere with the pathogenesis of some metabolic diseases, mainly by three different mechanisms: 1) vitamin malabsorption, 2) errors in vitamin metabolism, 3) vitamin dependent syndromes. The latter is due to a deficiency of the apoenzyme whose coenzyme is the vitamin itself. In this case pharmacological, instead of nutritional doses of the vitamin may be needed. The vitamins which interfere with inborn metabolic errors are reviewed; for each vitamin the corresponding diseases which may be treated are indicated. The vitamins are: 1) thiamine (leucinosis); b) nicotinic acid (hyperlipoproteinemia); c) biotin (beta-methyl-crotonyl-glycinuria, propionic aciduria); d) pyridoxine (infantile convulsions, familial pyridoxine responsive anemia, homocystinuria, cystathioninuria, xanthurenicaciduria); e) cobalamins (congenital intrinsic factor deficiency, cobalamin malabsorption, transcobalamin deficiency, methylmalonic aciduria) f) folic acid (congenital folic acid malabsorption, formimino-transferase deficiency, methylenetetrahydrofolic reductase deficiency, Lesch-Nyhan syndrome); g) vitamin D (phosphatic diabetes, Prader's type rickets, Albright's syndrome; essential hereditary hypophosphatemia, etc). It is noteworthy that the vitamin therapy of these diseases, not only corrects the metabolic errors, but can also promote the healing or the amelioration of the psycho-physical growth, of central nervous system alterations and of other lesions.
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PMID:[Vitamins in metabolic diseases]. 702 68

The cells' malabsorption of three classes of lipoproteins--chylomicrons and lipoproteins of low and very low density,--form under electrophoresis six phenotypes of hyperlipoproteinemia. In phylogenesis, cells absorb lipoproteins in a consecutive way by apoE/B-48, apoB-100 and apoE/B-100 receptor endocytosis. The domain-ligand in lipoproteins of very low density is forming when apoB-100 takes active conformation "deformed bilayer apoprotein-lipid" in association with domain apoE apoE/B-100 ligand is formed. Another active conformation apoB-100 in domain is globule with lipids in "pocket" forming apoB-100 ligand In blood 9 subclasses are formed: pre-ligand and post-ligand chylomicrons, lipoproteins with low density and lipoproteins with very low density. The ligand lipoproteins bind receptors of membrane and absorb cells. Both pre-chylomicrons, pre-lipoproteins with low density, pre-lipoproteins with very low density and post-chylomicrons, post-lipoproteins with low density, post-lipoproteins with very low density remain in blood. The sub-classes of lipoproteins form at electrophoregram 6 phenotypes of hyperlipoproteinemia: phenotype I-pre-chylomicrons + pre-lipoproteins with very low density; phenotype IIa--post-lipoproteins with low density; phenotype IIb--pre-lipoproteins with very low density; phenotype III--post-chylomicrons + pre-lipoproteins with very low density; phenotype IV--pre-lipoproteins with very low density; phenotype V--pre-chylomicrons + post-chylomicrons + pre-lipoproteins with very low density + post-lipoproteins with very low density. The formation under electrophoresis of primary phenotypes and secondary types of hyperlipoproteinemia occurs according single algorithm. In aphysiological sense, the major mass of palmitic and oleic lipoproteins with very low density absorb cells without transformation into lipoproteins with low density. Only linoleic and linolenic lipoproteins with very low density which are formed after binding of apoB-100 of triglycerides the same name and which are not much in blood acquire density of lipoproteins with low density physiologically. Under high content of triglycerides in blood main mass of lipoproteins with low density consists of aphysiologic palmitic lipoproteins with very low density with hydrated density lipoproteins with low density, the cause of hyperlipoproteinemia of phenotype III is genotype e21e2 apoE; hyperlipoproteinemia of phenotype V--genotype e4/e4 and probably toxic inhibition of activity (synthesis) phylogenetically late stearil-KoA-desaturase-2.
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PMID:[The conformation of apoB-100 in phylogenetically and functionally different lipoproteins of low and very low density: algorithm of formation of phenotypes of hyper lipoproteinimia (a lecture)]. 2506 20