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Biomedical subjects

J T Harries

Publications and source records attributed to J T Harries.

At least 55 records · Page 3Linked to original sources
PubMed1980

Yeast opsonisation in children with chronic diarrhoeal states.

Four patients with defective yeast opsonisation and protracted diarrhoea are reported. Plasma infusions improved the opsonising function in all 4 and the diarrhoea in 3. This immunological abnormality was assessed in 100 sequential patients with chronic diarrhoea associated with various gastrointestinal disorders; 52 with protracted diarrhoea and failure to thrive of undetermined cause, 26 with 'toddler diarrhoea', 8 with coeliac disease, 5 with chronic inflammatory bowel disease, and 9 with miscellaneous disorders. 23% of the patients with protracted diarrhoea of undetermined cause had defective opsonisation, a greater proportion (P less than 0.05) than that in 'toddler diarrhoea' or the remaining patients, in whom the frequency (4%) was similar to that (5%) in healthy populations. We suggest that yeast opsonisation be tested in children with protracted diarrhoea, as plasma infusions can be an effective form of treatment.

Child, Preschool↗
PubMed1980

Shwachman's syndrome. A review of 21 cases.

21 patients (10 male, 11 female) aged between 11 months and 29 years with Shwachman's syndrome are reviewed. All patients had exocrine pancreatic insufficiency. Haematological features included neutropenia in 19 (95%), anaemia in 10 (50%), and thrombocytopenia in 14 (70%); one patient developed erythroleukaemia. Severe infections occurred in 17 (85%) from which 3 (15%) died. Only one child exceeded the 3rd centile for height, and growth retardation was particularly evident in the older patients. All had skeletal abnormalities or delayed skeletal maturation, or both. Metaphyseal dyschondroplasia affected 13 of the older patients and was associated with skeletal deformities. Eight of 9 children under 2 1/2 years had rib abnormalities. Respiratory function tests in children under 2 years demonstrated reduced thoracic gas volume and chest wall compliance. Older patients had reduced forced expiratory volume and forced vital capacity. Neurological assessment showed developmental retardation or reduced IQ assessments, or both, in 85% of patients studied. Other neurological abnormalities included hypotonia, deafness, and retinitis pigmentosa. Neonatal problems had been present in 16 (80%) of the patients and 5 were of low birthweights. Hepatomegaly with biochemical evidence of liver involvement occurred in the younger patients and resolved with age. Other associated features included dental abnormalities, renal dysfunction, an icthyotic maculopapular rash in 13 (65%), delayed puberty, diabetes mellitus, and various dysmorphic features. These findings stress the diverse manifestations of the syndrome and extend knowledge on a number of aspects. Sibship segregation ratios support an autosomal mode of inheritance and an hypothesis for the pathophysiological basis of this syndrome is advanced.

Adolescent↗
PubMed1980

Symptomatic zinc deficiency in a breast-fed preterm infant.

A 2-month-old preterm boy who developed symptomatic zinc deficiency while being exclusively breast fed is described. Oral zinc supplements induced a complete remission but mucosal 65Zn uptake studies and metabolic balances conducted before and after withdrawal of the supplements excluded the diagnosis of acrodermatitis enteropathica. By age 12 months the boy was well and no longer required zinc supplements. Other possible causes of this patient's symptomatic zinc deficiency are discussed and these should be considered in the immediate and long-term management of preterm infants.

Breast Feeding↗
PubMed1980

Mucosal (Na+-K+)-ATPase and adenylate cyclase activities in children with toddler diarrhea and the postenteritis syndrome.

Assays for (Na+-K+)-ATPase and basal, fluoride stimulated and percentage of activation of adenylate cyclase have been established for small portions of human jejunal biopsies. Control means and ranges have been established for each activity in a group of children with failure to thrive but no gastrointestinal disease. Activities of (Na+-K+)-ATPase and percentage of activation and basal activity of adenylate cyclase are increased in children with toddler diarrhea. (Na+-K+)-ATPase activity is reduced in children with active postenteritis syndrome, and recovery from this syndrome is associated with an increase in this activity and of percentage of activation and basal activity of adenylate cyclase.

Acute Disease↗
PubMed1979

An inherited defect of neutrophil mobility in Shwachman syndrome.

Selected immunologic functions were assessed in 14 patients with the Shwachman syndrome. Nine patients were neutropenic and four had low levels of IgA or of IgM. Neutrophil mobility was significantly defective in the group of patients as a whole (in 12 it was below the lower limit of normal) and in their parents. No other consistent abnormality in immunity was found. These results suggest that the defective neutrophil mobility is a feature of Shwachman syndrome which may contribute to the vulnerability of these patients to frequent infections. The defect appears to be a primary genetic one, inherited as an autosomal recessive characteristic consistent with the assumed inheritance of Shwachman syndrome.

Adolescent↗
PubMed1979

A defect in zinc uptake by jejunal biopsies in acrodermatitis enteropathica.

1. In a system in vitro, 65Zn accumulation by jejunal mucosal biopsies from patients with acrodermatitis enteropathica was found to be markedly reduced compared with controls. 2. We suggest that defective uptake of zinc by enterocytes is the primary abnormality responsible for the zinc deficiency underlying this disorder.

Acrodermatitis↗
PubMed1979

Subcellular fractionation studies of the intestinal mucosa in congenital sucrase--isomaltase deficiency.

1. Jejunal biopsy specimens from three children with congenital sucrase-isomaltase deficiency were assayed for disaccharidase activity and were subjected to analytical subcellular fractionation with enzymic microanalysis. 2. By use of the highly sensitive fluorigenic modification of the disaccharidase assay, brush-border sucrase and isomaltase activities were depressed but nevertheless detectable in each child. 3. Apart from the expected decrease in brush-border alpha-glucosidase activity, the other enterocyte marker-enzyme activities were normal. 4. There were no abnormalities in the enterocytes of any child on analytical subcellular fractionation or on electron microsocopy.

Acetylglucosaminidase↗
PubMed1979

Intestinal bile salts in cystic fibrosis: studies in the patient and experimental animal.

The quantitative and qualitative distribution of bile salts in the duodenal juice of 13 patients with cystic fibrosis (CF) was studied after a test meal. The effects of triolein (TO), bovine serum albumin (BSA), and ricinoleic acid (RA) on the absorption of taurocholate (TCA) in the distal ileum of the rat in vivo was also studied. The mean (and ranges) of total bile salt concentrations, glycine: taurine conjugate ratios, and percentage of dihydroxy bile salts in the patients with CF and pancreatic insufficiency were 3.5 (1.3--6.6) mmol/l, 8.6 (greater than 10-3.1), and 37 (10--60) compared with control values of 7.4 (3.0--16.0) mmol/l, 3.0 (1.3--4.5), and 61 (52--70) respectively. The differences between the control and CF values were statistically significant (P less than 0.01--P less than 0.001). Three of the 13 CF patients had total bile salt concentrations less than 2 mmol/l, 8 had much higher glycine: taurine ratios, and 8 had a reduced percentage of dihydroxy bile salts. In 2 patients with normal pancreatic enzyme activities, duodenal bile salts were both quantitatively and qualitatively normal. TO (10 and 30 mmol/l), BSA (3%), and RA (5 mmol/l) had no inhibitory effect on the ileal absorption of TCA. These results show pronounced abnormalities of duodenal juice bile salts in CF with pancreatic insufficiency consistent with a broken enterohepatic circulation (EHC); such abnormalities may contribute to defective lipid absorption in CF. The data in the experimental animal do not support the suggestion that unhydrolysed dietary substrates play a role in the pathophysiology of the broken EHC.

Adolescent↗
PubMed1979

Studies in primary hypomagnesaemia: evidence for defective carrier-mediated small intestinal transport of magnesium.

A 4 year old male with primary hypomagnesaemia was studied using balance and steady-state perfusion techniques. Magnesium balance was negative and could be accounted for by increased faecal losses, renal conservation being normal; calcium balance was normal. After oral magnesium therapy magnesium balance became positive. The perfusion studies demonstrated net loss of magnesium into the intestinal lumen when low concentrations (1 and 2 mmol/l) of magnesium were perfused in contrast with control subjects; whereas at high concentrations (10 mmol/l a net absorption of a magnitude similar to control values was observed. In the control subjects sequential perfusion of increasing concentrations of magnesium demonstrated a curvilinear relationship between rates of absorption and the lower concentrations (1, 2, and 4 mmol/l) with an apparent Km and Vmax of 4.5 mmol/l and 91 nmol/min/cm respectively. At the higher concentrations (6 and 10 mmol/l) the relationship was linear. These data suggest that two separate transport systems participate in the absorption of magnesium from the proximal small intestine; a carrier-mediated system which saturates at low intraluminal concentrations, and a simple diffusional process. The possibility of the second transport system being a carrier-mediated process with a very much higher Km cannot be excluded. In primary hypomagnaesaemia the results suggest that the primary abnormality is a defect in carrier-mediated transport of magnesium from low intraluminal concentrations of magnesium.

Biological Transport↗