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Biomedical subjects

J Rey

Publications and source records attributed to J Rey.

At least 145 records · Page 8Linked to original sources
PubMed1981

[Normal values of the main stool constituents from age 1 year to puberty (author's transl)].

The daily excretions of the main stool constituents have been measured during 97 balance periods performed in 73 children aged 1 to 17 years and whose digestive functions could be considered as normal. From 1 to 2, 2 to 6, and 6 to 17 years of age, the following values (mean and range) have been obtained: expressed in g/day, for stool wet weight: 61 (33-112), 79 (19-157), 76 (22-166); for nitrogen: 0.49 (0.26-1.06), 0.74 (0.3-2.13), 0.83 (0.3-1.52); for fat: 2 (1-3.6), 1.9 (0.9-3.2), 1.8 (0.6-4.5); expressed in mmol/day for volatile acids: 10.1 (5.2-18), 13.7 (1.8-23.7), 10.1 (3.5-27.5); for Na+ : 0.7 (0.3-1.2), 1 (0.1-3.2), 1.1 (0.1-4); for K+ : 4.4 (3-6.7), 5.1 (1.7-10.4), 5.9 (1.6-11). Only wet weight and nitrogen vary significantly from the first to the second period of age (p less than 0.05 and less 0.01 respectively). Wet weight is highly correlated with volatile acid excretion (p less than 0.001) but weakly linked to fecal fat which implies that the measurement of the percentage of fat in fresh stools is not a correct estimate of their absorption.

Adolescent↗
PubMed1980

[Horizontal optokinetic nystagmus in the pigeon: role of accessory optical system].

Some characteristics of the horizontal optokinetic nystagmus (OKN) have been studied in the pigeon using monocular and binocular stimulations. The OKN is asymmetrical, temporal to nasal stimulation eliciting a larger response than a nasal to temporal one. The role of the accessory optic system (AOS) was studied by means of unilateral or bilateral lesions of the primary relays, nuclei ectomamillaris (nEM) and superficialis synencephali (nSS). Results show the existence of a synergy between nEM and nSS on the same side and between nEM and nSS on opposite sides, whereas each nucleus has an inhibitory effect upon its contralateral homologue. Some evidence was obtained which suggests the existence of an ipsilateral retinal projection to the nEM.

Animals↗
PubMed1980

Biopterin defect in a normal-appearing child affected by a transient phenylketonuria.

A child diagnosed as having transient phenylketonuria was found to have reduced synthesis of tetrahydrobiopterin and an abnormal clearance of phenylalanine, but he remained clinically normal when on a normal diet. A small amount of 7,8-dihydrobiopterin was found in his serum; this distinguishes the case from that of malignant hyperphenylalaninaemia.

Biopterins↗
PubMed1980

[Polyacrylamide gel electrophoresis analysis of membrane proteins of microvilli and of a cytoplasmic fraction in 8 cases of congenital saccharose intolerance].

The protein composition of the brush border membrane and of a cytoplasmic fraction has been studied by polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulfate in 8 children with congenital sucrose intolerance. The sucrase-isomaltase complex was not found (6 times) or in trace amounts (2 times) among the brush border membrane proteins whereas an abnormal band was visible among the cytoplasmic proteins in the 4 cases where they have been studied. These results suggest that a structural mutation affecting the signal sequence necessary for the complex to be transferred on the luminal side of the endoplasmic reticulum membrane could be at the origin of the disease.

Adolescent↗
PubMed1980

[Cytogenetic study in malformed newborns. Evolution during different stages of life (author's transl)].

Authors report cytogenetic findings in 34 alive newborns presenting congenital malformations in the first week (Group A). These findings have been tested with Group B (100 malformated children up to 12 years old) and Group C (1,000 hospital patients of any age). Chromosomic anomalies in 44, 22 and 15% of the groups respectively are found. Performance of kariotype in any newborn with congenital malformations for detection of chromosomic aberrations or familial translocations in disbalance is needed.

Child↗
PubMed1980

[Perfusion study of intestinal sugar absorption after extensive intestinal resection (author's transl)].

Small bowel adaptation after extensive intestinal resection has been studied in 5 children. A segmental perfusion technique was used to evaluate the kinetics of glucose absorption and sucrose hydrolysis. The activity of 6 brush border hydrolases and the mean villus height were measured in intestinal biopsies. Glucose absorption was greater than in 3 control children of the same age and was almost complete. Sucrose hydrolysis was increased in the same proportion and the maximum capacity of the segment was 2 mmol/min/20 cm as compared to 1.2 mmol/min/20 cm in the control group. Absorption of the released monosaccharides was complete for glucose and as high as in controls for fructose. Brush border hydrolase activities were identical to those of controls whereas the mean villus height was increased (438 micrometer as compared to 342 micrometer) although the difference was not significant. These results demonstrate the functional adaptation of the remaining small bowel and confirm that it is due to compensatory hyperplasia.

Carbohydrate Metabolism↗
PubMed1980

[Johanson-Blizzard's syndrome: another cause of pancreatic lipomatosis (author's transl)].

A new case of Johanson-Blizzard's syndrome is reported. It concerns a boy born to consanguineous parents and who died at the age of 10 months from malnutrition. Anal imperforation, alar agenesia, hair anomalies, mental retardation and external pancreatic failure were associated. Neither deafness nor hypothyroidism appeared to be present. Autopsy revealed lipomatous hypoplasia of the exocrine pancreas, hitherto unobserved in this syndrome, and probably responsible for the external pancreatic failure noted in published cases. The variability within a given family of the Johanson-Blizzard malformative syndrome is illustrated by two other cases reported in the anamnesis, one involving a brother who had died earlier with cutaneous aplasia at the fontanella and lacrimal canal malposition and one involving a second cousin who presented with isolated anal imperforation.

Abnormalities, Multiple↗
PubMed1980

[Neonatal primary chronic adynamic bowel (author's transl)].

Three cases of a primary disorder of intestinal motility which was responsible for neonatal functional intestinal obstruction are reported. This serious condition affected the small intestine and the colon and was difficult to treat. Early operation and prolonged intravenous alimentation was necessary. It is distinct from Hirschsprung's disease because of the presence of ganglion cells and because of the abnormalities of the myenteric plexus on silver staining. However the clinical symptoms and the decreased intraparietal cell V.I.P. concentration in pathological areas are similar in both conditions.

Female↗
PubMed1979

The diagnosis of coeliac disease. A commentary on the current practices of members of the European Society for Paediatric Gastroenterology and Nutrition (ESPGAN).

In 1977, 53 members of ESPGAN completed a questionnaire on their current practice in diagnosing coeliac disease. The usefulness of the 'Interlaken' criteria enumerated 9 years previously was reassessed. Details were obtained about the initial diagnostic approach, the acceptable histological criteria of the initial jejunal biopsy, and the timing, technique, response, and interpretation of early and late rechallenges with gluten. Answers indicated that, although the initial mucosal lesion is usually 'flat' at the time of diagnosis, a few infants may present at a time when the mucosal lesion is less completely damaged. Furthermore, the degree of histological change after gluten challenge that is acceptable as a positive response may vary according to the state of the mucosa before challenge. It was noted that there are still no generally agreed criteria by which the histological lesions may be described, so that (after further discussions at the Third International Coeliac Conference in Galway) a European panel has been set up to make recommendations. In the experience of ESPGAN members, most coeliac children will have a histological relapse within 2 years of reintroduction of gluten. But a small number of unorthodox cases were reported that suggest that (a) histological relapse may take longer than 2 years to appear, or (b) the degree of sensitivity to gluten may vary at different ages. Very long-term follow-up will be needed to explain these anomalies. Meanwhile the search continues for 'the basic defect'.

Age Factors↗
PubMed1979

Prinzmetal's angina:reflex cardiovascular response during episode of pain.

Previous angiographic studies have shown that coronary spasm occurs in association with the variant angina described by Prinzmetal, confirming his original hypothesis. In this work we recorded the heart rate changes and the incidence of arrhythmias during variant angina. The patients were divided into two groups: anterior, with electrocardiographic signs of anterior ischaemia, and inferior, with changes in the inferior leads. There was a significant increase of heart rate during pain in anterior myocardial ischaemia and a significant decrease when the ischaemia was inferior. The incidence of ectopic arrhythmias during pain was significantly greater in patients with anterior ischaemia, but there was a high incidence of atrioventricular block in patients with inferior ischaemia. We suggest that these findings can be explained by different responses of the automatic nervous system to anterior and inferior acute myocardial ischaemia.

Adult↗
PubMed1979

[Circulating somatomedin activity and growth hormone secretion. Changes during late diagnosed celiac disease and effects of treatment].

Sixteen children, aged 5-19 years with late diagnosed coeliac disease who presented with significant growth delay were studied. Plasma somatomedin was decreased in all patients (mean 0.28 +/- 0.1) but the circulating growth hormone levels were normal in 12 patients. No inhibitory activity could be detected. Somatomedin activity was partially corrected in six patients who were re-examined after 3 months on a gluten free diet. This suggests that low somatomedin levels may be the result of the nutritional abnormalities during the active phase of the disease.

Adolescent↗
PubMed1979

["Transitory" phenylketonuria. A permanent deficit].

Three cases are reported with hyperphenylalaninaemia greater than 1.8 micrometer/ml-1 in the neonatal period, becoming tolerant of a normal regime (3 g protein per kg) without plasma levels of phenylalanine exceeding 0.2 to 0.3 micrometer/ml-1. Atypical kinetics (zero order) of phenylalanine clearance after intravenous perfusion were shown in the three cases at the age of one year and the persistence of the disorder was again demonstrated at the age of five years in two cases by the study of an oral load of phenylalanine. Examination of the parents showed normal fasting levels of phenylalanine and a normal phenylalanine/tyrosine ratio. The observations draw together several previous publications from diverse authors and a new defined entity, "transitory" phenylketonuria, is proposed. It does not always appear to be a homogenous condition, as a partial defect in biopterin synthesis has been shown in the one case. In retrospect no anomaly of this kind was discovered in the other two cases where the mechanism was not elucidated.

Biopterins↗