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Biomedical subjects

A Lerner

Publications and source records attributed to A Lerner.

150 records · Page 9Linked to original sources
PubMedDate not supplied

Effects of parenteral nutrition on whole body and extremity composition in children and adolescents with active inflammatory bowel disease.

The effect of parenteral nutrition (PN), combined with corticosteroid therapy, on body composition was evaluated in 22 pediatric patients with active and complicated inflammatory bowel disease (IBD). The patients were divided into two groups according to the duration of PN. Group A consisted of 14 patients who received PN for an average of 43 days (short term) whereas eight patients received PN for an average of 104 days (long term); group B. Nutritional assessments were performed before and following the PN periods. In addition, a follow-up assessment was performed 50 days after the cessation of PN for group A. Serial evaluations included; weight, height, disease activity score, arm anthropometry, subscapular skinfold (SSSF) thickness, 24-hr urinary creatinine, and computed tomography (CT) scan of the thigh. Following PN with a glucose-fat mixture used as a nonprotein energy source, there were significant (p less than 0.05) increases in weight, SSSF, and muscle mass (MM) in both groups. Disease activity declined in both groups and height increased in group B and at the follow-up assessment for group A. Considering extremity composition, both groups exhibited increases in midarm muscle area, midarm fat area, thigh muscle area, and thigh fat area. However, a differential distribution of incremental change was observed depending on the duration of PN. Fat deposition predominated in both the upper and lower extremities for group A. Group B patients, however, although showing a predominance of fat accumulation in the upper extremities, gained muscle and fat components equally in the lower extremities.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

Endomysial antibodies in the diagnosis of celiac disease and the effect of gluten on antibody titers.

Celiac disease, a common chronic gastrointestinal disorder, is gluten induced and is controlled with a gluten-free diet. While the management of CD with a gluten-free diet is quite effective, the diagnosis is rather difficult. The ESPGAN criteria for the diagnosis of CD seems to be tedious and time-consuming. Serological tests for IgA class endomysial antibodies, as detected by indirect immunofluorescence, on human and primate smooth muscles are specific and sensitive markers of celiac disease. Of all the specimens examined, endomysial antibodies were present in patients with gluten-sensitive enteropathy. These antibodies occurred in all active cases of celiac disease, in 90 percent suspected celiac patients where all the ESPGAN criteria has not been fulfilled. This contrasts to the presence of endomysial antibodies in 46 percent of confirmed and 17 percent of suspected celiac patients maintained on a gluten-free diet for various time intervals. Endomysial antibodies also occurred in all cases with chronic diarrhea and gut histology consistent with CD and 8% of asymptomatic family members of CD patients. None of the patients with other gastrointestinal and liver diseases had endomysial antibodies. These studies thus emphasize the specificity and sensitivity of endomysial antibodies for celiac disease.

Animals↗
PubMedDate not supplied

A single-base deletion in the 3'-coding region of glycogen-debranching enzyme is prevalent in glycogen storage disease type IIIA in a population of North African Jewish patients.

Glycogen storage disease type III (GSD III) is an autosomal recessive disease caused by the deficiency of glycogen-debranching enzyme (AGL). The overall incidence of the disease is about 1:100,000 life births in the USA; however, it is unusually frequent among North African Jews in Israel (prevalence 1:5,400, carrier prevalence 1:35). All North African Jewish GSD III patients examined have both liver and muscle involvement. While all patients showed the characteristic features related to the liver enzyme deficiency, the peripheral muscular impairment varied from minimal to severe, with neuromuscular involvement. A single mutation in the AGL gene, the deletion of T at position 4,455 (4,455delT) in homozygous form, was found in this patient population. The mutation 4,455delT results in the change of 17 amino acids at the carboxy terminus of the AGL protein (1,486-1,502) and truncation of the last 30 amino acids of the normal AGL 1,532 amino acids. The mutation appears to be ethnic specific as it was not seen in 18 patients of different ethnic origins. This is the first report of a mutation in the AGL gene affecting a considerable number of GSD III patients in a defined population.

Adult↗