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Biomedical subjects

J Batten

Publications and source records attributed to J Batten.

32 records · Page 2Linked to original sources
PubMed1977

Cystic fibrosis: Evidence for a genetic compound from a family study in cell culture.

Although the majority of patients with cystic fibrosis (CF) show a typical clinical course, a minority with the same clinical phenotype at the time of initial diagnosis have an atypical (mild) course. Skin fibroblast cultures were established from 49 members of the family of one such atypical CF adult patient, previously identified (Danes et al. 1976) as CF Class II (ametachromatic and no metabolic cooperation with CF Class I fibroblasts), the offspring of Class I (metachromatic, metabolic cooperation with normal fibroblasts)/Class II mating. The culture phenotype for Class I was traced on the maternal side and for Class II on the paternal side through consecutive generations and the culture phenotype of each class segregated. This family study added experimental evidence to support the hypothesis that the atypical (mild) clinical features and course of this adult CF patient were due to two different CF genes combining to produce a genetic compound expressing a mild form of CF.

Adult↗
PubMed1976

Cystic fibrosis: a cell culture study on an adult patient population.

Skin fibroblast cultures from an adult population of cystic fibrosis (CF) patients have been studied to determine any possible genetic or clinical significance of the two classes described for cultured CF skin fibroblasts (Danes 1973). On the basis of clinical course, the 46 adult patients from 43 unrelated families studied were divided into two groups (40 typical, 6 atypical). The cultured fibroblasts from 37 of the 40 patients with typical CF were Class I (metachromatic, cystic fibrosis factor activity (CFFA) in the culture medium, and metabolic cooperation with normal fibroblasts), and the cultures from the remaining three patients were Class II (ametachromatic, no CFFA in the culture medium and no metabolic cooperation with metachromatic CF fibroblasts). During the 4-year research period one of the 37 patients from Class I and all three Class II patients with typical CF succumbed. The cultured skin fibroblasts from two of the six patients who had an atypical course for CF were Class I and four were Class II. When the parents of three of these atypical CF Class II patients were studied, the cultured fibroblasts from one parent in each family in each family were Class I and the other Class II. The affected offspring from such Class I-II matings may represent genetic compounds.

Adolescent↗
PubMed1970

Comparative trial of Nutrizym in chronic pancreatic insufficiency.

A cross-over trial of pancreatic replacement therapy was carried out in 12 adults with chronic pancreatic insufficiency. The standard enteric-coated preparation, Pancrex V forte, was compared with Nutrizym, which has an enteric-coated core of pancreatic extract and a shell of bromelains-a mixture of proteolytic enzymes derived from the stem of the pineapple.Nutrizym was significantly more effective than Pancrex V forte in improving fat absorption, and reduced faecal weight. Protein digestion was assessed by measuring the urinary excretion of hydroxyproline after a gelatin meal. Nutrizym produced an earlier and significantly higher peak in hydroxyproline excretion than Pancrex V forte, but the cumulative effect was similar. The value of bromelains was investigated by including a period on the Nutrizym core alone. This was similar to Pancrex V forte in improving fat absorption but had less effect on protein digestion, suggesting that the beneficial effect of Nutrizym compared with Pancrex V forte was due to the added bromelains, and not to differences in enzyme content or enteric coating.

Adolescent↗
PubMed1969

Sudden death in a young asthmatic.

A 14-year-old boy with severe asthma was treated with prednisone and disodium cromoglycate with considerable subjective improvement, though there was persistent airways obstruction. After several weeks' freedom from symptoms he died in a severe attack of nocturnal asthma. Necropsy findings suggested that hypersecretion had been occurring in die medium-sized airways for several days before death.

Adolescent↗