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Biomedical subjects

M H Fischer

Publications and source records attributed to M H Fischer.

31 records · Page 2Linked to original sources
PubMed1988

Bone status in nonambulant, epileptic, institutionalized youth. Improvement with vitamin D therapy.

This study was conducted to determine if the adverse effects of anticonvulsant drug therapy and nonambulancy on bone status could be overcome with vitamin D therapy in severely handicapped individuals. Six male and five female gastrostomy fed, nonambulant, epileptic, profoundly mentally retarded individuals ranging in age from 7 to 17 years were given vitamin D therapy at a dosage of 4,000 IU/m2 body surface area/day for 6 months. Photon absorptiometry and biochemical indices of bone status were measured to follow the effects of therapy. Bone mineral content expressed as a percentage of normal improved by 11 percent (p less than 0.01), from 59.6 to 66.1 percent. Tartrate-resistant acid phosphatase, total alkaline phosphatase, and the bone isoenzyme activities declined 11 percent, 18 percent, and 11 percent respectively. These reductions were not statistically significant but they were consistent with the improvements observed by photon absorptiometry. The results of our study suggest that a conservative supplement of vitamin D will improve the bone status of severely disabled youths.

Adolescent↗
PubMed1981

Urinary levels of blood group A trisaccharide: observations in two siblings with neuronal ceroid lipofuscinosis.

A readily detectable carbohydrate in the urine of two siblings with neuronal ceroid lipofuscinosis was found to be the blood group A trisaccharide. One child expired before blood typing was done while the other sibling was AB. This latter child excreted greater amounts of the trisaccharide than a group of blood type A subjects with different diagnoses when all the subjects were receiving whole or skim milk by nasogastric feedings. While the relevance of this observation to neuronal ceroid lipofuscinosis is unknown, it has been shown that the trisaccharide may be a major urinary carbohydrate depending on diet and blood type.

ABO Blood-Group System↗
PubMed1981

The composition of tuberous sclerosis grey matter.

The chemical analysis of grey matter from a tuberous sclerosis subject indicated the presence of two different types of grey matter based on two distinctly different ganglioside concentrations. Both samples had gangliosides of reduced concentration with an abnormal composition compared to normal grey matter as evidenced from their thin-layer chromatography profiles. Analyses of the glycoprotein fraction from both samples revealed increased glycoprotein content, particularly in total carbohydrate. This observation was consistent with previous studies of angiofibromas and angiomyolipomas.

Brain↗
PubMed1980

Tryptophan and lysine metabolism in alpha-aminoadipic aciduria.

Two brothers previously diagnosed as having alpha-aminoadipic aciduria (alpha-AA) were subjected to a tryptophan loading test to determine if their condition resulted from a defect in the alpha-aminoadipate aminotransferase (kynurenine aminotransferase) system. Normal increases in kynurenic and xanthurenic acids eliminated this possibility. Further analyses of their urines revealed that both boys had measurable amounts of previously undetected alpha-ketoadipic acid (alpha-KA) before and after the loading test. A reexamination of speciments from a prelysine and postlysine loading test reconfirmed the existence of alpha-KA in their urines at the time the original observation of alpha-AA was made. The response to the lysine load was a predictable increase in both alpha-AA and alpha-KA. The boy who had been referred to this institution with a learning defect responded to the tryptophan load with a slight decrease in alpha-AA and an unpredicted decrease in alpha-KA and 3-hydroxykynurenine. His mentally normal brother showed a significant decrease in alpha-AA and major increases in all other measured metabolites including alpha-KA. The latter results were compatible with a defect in the oxidative decarobxylation of alpha-KA. A comparison of the urinary alpha-AA and alpha-KA concentrations in our subjects with comparable data in mentally normal and mentally retarded patients with this condition suggested that the retardation may result from other causes.

2-Aminoadipic Acid↗
PubMed1977

Chemical analysis of an angiofibroma from a patient with tuberous sclerosis.

Amino-acid analyses on the acid hydrolysates of an angiofibroma and skin established that the former tissue contained less collagen than skin based on the reduced content of hydroxyproline, glycine, proline and alanine in the tumour. From lysosomal enzyme measurements it became evident that the specific activities of the hexosaminidases, beta-glucuronidase and beta-galactosidase were elevated. Analyses of the alcohol insoluble fraction following pronase digestion revealed that the tumour contained more acidic glycosaminoglycan (AG) than skin as assessed by uronic acid and hexosamine measurements. More outstanding, however, was the seven-fold increase in the total carbohydrate in the AG fraction of the tumour. The overall composition of this fraction was very similar to comparable material from foetal skin except that the tumour fraction contained increased sulphate concentration.

Adult↗
PubMedDate not supplied

Improved selenium, carnitine and taurine status in an enterally fed population.

Ten adult, male, nonambulant and gastrostomy-fed individuals had received commercially available enteral feedings containing negligible amounts of selenium, carnitine, and taurine for an average of 59 months. Blood levels of these three nutrients were below published normal ranges as were the urinary excretions of carnitine and taurine. After 9 weeks on a product that was fortified with 23 micrograms of selenium, 39 mg of carnitine, and 38 mg of taurine per 8 ounces, blood levels were significantly increased with the levels of selenium and carnitine being normalized.

Adolescent↗
PubMedDate not supplied

Dietary fiber and long-term large bowel response in enterally nourished nonambulatory profoundly retarded youth.

The objective of this year-long research was to determine the effects of three levels of a purified dietary fiber source, soy polysaccharide, on the long-term bowel function of 11 youth, aged 7 to 17 years, who were nonambulatory, profoundly disabled, constipated, and fed by gastrostomy. From day 1 to 60 the diet was a fiber-free enteral feeding; from day 61 to 300, 12 to 20 g/day total fiber was added to the diet; and from day 301 to 360, 18 to 25 g/day. Complete stool collections were made from day 51 to 60, day 111 to 120, day 291 to 300, day 321 to 330, and day 351 to 360. The second fiber addition significantly increased mean (+/- SD) daily stool frequency from 0.6 +/- 0.2 during the fiber-free diet to 1.1 +/- 0.5. The first addition of fiber compared to the fiber-free diet significantly increased stool moisture from 70 +/- 7% to 76 +/- 8% and wet stool weight from 30 +/- 13 g/day to 53 +/- 21; mean stool weight during days 351 to 360 was 87 +/- 45 g/day. Daily dry stool weight significantly increased with the second fiber addition. Soy polysaccharide fiber improved bowel function in this nonambulatory profoundly disabled population.

Adolescent↗