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Biomedical subjects

J P Buts

Publications and source records attributed to J P Buts.

At least 55 records · Page 3Linked to original sources

[Refractory diarrhea in infants: experimental basis of current therapeutic perspectives].

Intractable diarrhoea is a severe condition of early infancy producing prolonged food intolerance, massive watery diarrhoea and malnutrition. Although the etiology remains unknown in more than half of the cases, a basic physiopathological mechanism appears to be a variable inhibition of the maturation and differentiation of the crypt cell resulting in immature villus cells. Among the known nutritional and hormonal factors that control maturation of the enterocytes, polyamines (spermine, spermidine) emerge as the most trophic substances potentially useful for humans. Clinical studies are warranted to determine whether semi-elemental diets enriched in polyamines would provide some benefit for patients with intractable diarrhoea or with an adapted bowel after extensive enterectomy.

Cell Differentiation↗

Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy.

An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging. Combined immunosuppressive regimen with steroids and azathioprine led to prolonged clinical and biological remission with a respective 2 years and 7 months follow up. The girl, however, after 7 months developed a progressive encephalopathy of unknown aetiology, while liver and haematological disease were still under control. She died subsequently from severe recurrent seizures. We conclude that acute Coombs-positive giant cell hepatitis of infancy can be improved by sustained immunosuppressive therapy.

Acute Disease↗

Caustic burns of the upper digestive and respiratory tracts.

In a series of 51 children presenting with an accidental caustic burn, symptoms were analysed for their predictive value of significant i.e. necrotizing oesophageal lesions (grade II or III). For the whole group, the incidence of significant oesophageal lesions was 37%. Vomiting and/or respiratory distress were associated with high incidence of significant oesophageal burn (84% and 75% respectively). The particular location of each caustic burn was analysed for its association with caustic burns at other anatomical sites. Of the 18 patients with a laryngeal burn, 72% also had a grade II or III oesophageal burn. The 19 patients with a grade II or III oesophageal burn and the 18 patients with a laryngeal burn, all had lesions at other sites. In the group of 19 patients with a caustic lesion limited to one site, only 1 patient showed mild oesophagitis (grade I) without late sequelae.

Burns, Chemical↗

Persistence of a liver metabolic zonation in extra-hepatic biliary atresia cirrhotic livers.

To evaluate whether liver metabolic zonation persists in human biliary cirrhosis, we used quantitative cytochemistry to measure activities of glucose 6 phosphatase (G6P) and NADPH dehydrogenase (ND) in hepatocytes situated in different zones of liver cirrhotic nodules. Liver specimens were obtained from 13 children with extrahepatic biliary atresia with compensated cirrhosis. Activity and distribution were compared with zonal activities measured in 17 control human liver specimens obtained during reduction hepatectomies for orthotopic liver transplantation. In normal human liver, G6P was 1.86 times more active in the periportal than in the perivenular zone. On the contrary, ND activity was lower in the periportal zone (63% of perivenular activity). A metabolic zonation persisted in extra-hepatic biliary atresia with compensated cirrhosis. G6P activity was 1.56 times greater at the nodule periphery than at the nodule center, whereas ND activity was lower at the periphery (75% of nodule center activity). This metabolic zonation is the opposite of that observed in animal toxic (CCl4) cirrhosis, in which greater G6P activity is observed at the nodule center and greater ND activity at its periphery. This confirms our previous hypothesis that the type of cirrhotic metabolic zonation may depend on the site of initial liver damage.

Biliary Atresia↗

Stimulation of secretory IgA and secretory component of immunoglobulins in small intestine of rats treated with Saccharomyces boulardii.

Saccharomyces boulardii (S.b.) is largely used in Western European countries for the treatment of acute infectious enteritis and antibiotic-induced gastrointestinal disorders. To study the mechanisms of the protective effect of S.b. against enteral pathogen infection, we assessed the response of the intestinal secretion of secretory IgA (s-IgA) and of the secretory component of immunoglobulins (SC) to oral administration of high doses (0.5 mg/g body weight, three times per day) of S.b. cells in growing rats. S.b. cells (biological activity: 2.8 x 10(9) viable cells/100 mg) were administered daily by gastric intubation to weanling rats from day 14 until day 22 postpartum. Control groups received either 0.9% saline or ovalbumin following the same schedule. Expressed per milligram of cell protein, SC content was significantly increased in crypt cells isolated from the jejunum (48.5% vs saline controls, P less than 0.05) as it was in the duodenal fluid (62.8% vs saline controls, P less than 0.01) of rats treated with S.b. Oral treatment with S.b. had no effect on the secretion of SC by the liver. In the duodenal fluid of rats treated with S.b. cells, the mean concentration of s-IgA was increased by 56.9% (P less than 0.01) over the concentration of s-IgA measured in saline controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Liver transplantation in children less than 1 year of age.

Of 139 children who received an orthotopic liver transplant in our center between March 1984 and July 1989, a total of 17 patients (12%) had transplants before their first birthday (mean age 10.3 months; range 8 to 11). The mean weight was 7.3 kg (range 5.2 to 13). Nine retransplantations were performed in five children because of primary nonfunction (three children), hepatic artery thrombosis (four), or rejection (two). A reduced donor liver was used for 11 of 26 transplants. Baseline immunosuppression included cyclosporine, prednisone, and azathioprine with OKT3 or anti-thymocyte globulin for steroid-resistant rejection episodes. Survivors were discharged after a mean hospital stay of 47 days (range 22 to 87), and nonsurvivors died within a mean of 40 days (range 0 to 120). The 1 year actuarial survival rate was 64.7%, in comparison with 75.8% in the whole series. One patient died perioperatively, two died from primary nonfunction, one from adenovirus infection, two from rejection, and one from bone marrow aplasia. Eighteen rejection episodes, of which 11 were steroid resistant, occurred in 11 patients. Our series shows that liver transplantation can be successful in this age group.

Biliary Atresia↗

Unifocal stricture of the common bile duct in two children: a localized form of primary sclerosing cholangitis.

A 4-year-old boy and a 14-year-old girl referred for a cholestatic syndrome were found to have a unifocal stricture of the extrahepatic bile duct. Both radiological (endoscopic-retrograde-choledocopancreatography) and histological findings were suggestive of primary sclerosing cholangitis. There was no past history of chronic inflammatory bowel disease nor any other disorder. Serum autoimmune markers were negative. Complete remission was observed after surgical drainage, and both patients remained symptom-free after a follow-up of 12 months and 8 years, respectively. Such unifocal form of the disease may have a better prognosis than the plurifocal disease. The absence of associated disorders and autoimmunity may also be an indicator of better prognosis in these patients.

Adolescent↗

Refeeding after starvation in the rat: comparative effects of lipids, proteins and carbohydrates on jejunal and ileal mucosal adaptation.

To compare the tropic effect of different dietary nutrients on mucosal adaptation in the jejunum and ileum, adult rats were submitted to a 96-h period of starvation and refed isocaloric liquid diets (1.5 kcal ml-1) containing either protein (casein), carbohydrate (starch) or lipids. In the jejunum, 4 days of starvation caused mucosal hypoplasia, villus and crypt shortening and a decrease in the total activity of disaccharidases with the exception of lactase which was markedly enhanced. In contrast, mucosal hypoplasia was incomplete in the ileum which exhibited an increase in crypt depth and in the specific and total activities of disaccharidases and of aminopeptidase. Compared with protein and carbohydrates, lipids exerted the strongest stimulatory effect for mucosal regeneration. In the jejunum as well as in the ileum, mucosal mass parameters, villus length, crypt depth and lactase activity did reverse towards their initial value within 1-3 days of refeeding lipids, even though the animals received only one-third of their normal daily caloric intake. Our results indicate that the pattern of response to fasting differs between the proximal and distal small intestine, and that the intestinal changes induced by starvation are rapidly reversed by refeeding small amounts of a diet rich in fat.

Animals↗

Hormonal regulation of the rat small intestine: responsiveness of villus and crypt cells to insulin during the suckling period and unresponsiveness after weaning.

To further document the effect of insulin on intestinal maturation, suckling rats were treated either with exogenous insulin (12.5 mU.g body wt, intraperitoneally, twice daily) or with saline from d 8 to 12 postpartum. Sucrase activity in brush border membrane extracts was precociously induced by insulin, whereas the activities of other brush border membrane enzymes (maltase, aminopeptidase, and neutral lactase) were enhanced (+ 30 to + 131%, p less than 0.01 versus controls). The lysosomal enzyme, N-acetyl-beta-glucosaminidase, which normally declines at weaning was significantly (p less than 0.025) decreased in both villus (-51%) and crypt cells (-57%) isolated from the jejunum of insulin-treated rats. The microsomal enzyme, sulfatase C, and the cytosolic enzyme, lactate dehydrogenase, were also sensitive to insulin with decreases in activity ranging from -37 to -63% (p less than 0.05) compared to saline-treated control rats. Insulin at doses of 0.5 or 12.5 mU did not influence plasma total corticosterone levels, which were about 9-fold lower in suckling than in 25-d-old weaned rats. In weaned rats (from d 25 to 32) insulin treatment (12.5 mU) failed to influence the activity of brush border membrane hydrolases or of lysosomal, microsomal, and cytosolic enzymes. The synthesis rate of mature sucrase-isomaltase, measured in weaned rats (32 d) by the incorporation of 14C-leucine into the enzyme precursor protein, was equivalent in both groups. These data demonstrate that the immature enterocyte of the suckling rat is responsive to insulin, whereas the mature enterocyte of the weaned rat is unresponsive.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Intensive care for children after orthotopic liver transplantation.

We report our experience in the management of children after orthotopic liver transplantation (OLT). From 03/84 to 04/87 50 patients (pts) were transplanted. Mean age was 4 3/12 years (8/12 to 13 2/12) and mean body weight 14.7 kg (5.8 to 40). Hospital mortality was 14%. Problems related to the surgery included: Abdominal complications: bleeding (8 pts), infection (18 pts), ascites and fistula (1 pt), need for secondary abdominal surgery (10 patients). Respiratory problems: lobar atelectasis (11 pts), right diaphragmatic paralysis (2 pts) and right pleural effusion (11 pts). Problems related to immunosuppression included: Bacterial infection (29 pts) fungal infection (5 pts), one patient died of disseminated cytomegalovirus infection. Side effect of cyclosporin A (CsA) were systemic blood hypertension (S.B.H.) (47 pts), sinusal bradycardia (37 pts), associated to SBH (24 pts), hypertensive encephalopathy (2 pts). Generalized seizures (2 pts in the absence of SBH). Renal side effects of CsA were hypercreatininemia, decreased sodium bicarbonate and hyperkaliemia. The nephrotoxicity of CsA was favoured by the use of other nephrotoxic drugs such as aminoglycosides, amphotericin B. Edematous pancreatitis was observed in 3 patients and related to the use of large doses of steroids. Problems related to the functioning of the graft included: Primary non-function of the graft (4 pts), hepatic artery thrombosis (8 pts) and severe acute rejection unresponsive to therapy (1 pt); these situations needed to be recognised early in order to organize a second OLT. Other causes of hepatic dysfunction were: portal vein thrombosis (1 pt), biliary tract obstruction (2 pts), angiocholitis (3 pts), right hepatic lobe necrosis (2 pts). Acute hepatic insufficiency in 7 children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Changes in serum and intestinal diamine oxidase (DAO) activity after proximal enterectomy in rats. Correlation of DAO activity with mucosal mass parameters.

To determine whether serum and mucosal DAO activity reflects quantitative changes in the small bowel mucosal mass, we have chosen an experimental model of mucosal hyperplasia which is known to occur in the rat after enterectomy. A 50% proximal enterectomy or a single transection was performed in 20 growing rats weighing 145-160 g. Ten days following surgery, we determined mucosal mass parameters (weight, protein, and DNA content), sucrase activity, and DAO activity in the duodenum (segment A), proximal ileum (segment B), and distal ileum (segment C) of the remaining small intestine. Mucosal hyperplasia was demonstrated by the finding that in each segment, mucosal weight, protein, and DNA content per centimeter of gut length were significantly (P less than 0.01) higher (+38 to + 78%) in the resected group than in transected controls. In segments B and C of resected rats, the changes in DAO activity expressed per gram of mucosa paralleled the changes in mucosal mass, the activity being increased by +69% and +49% (P less than 0.05) compared to the values recorded in transected controls. Expressed per centimeter of gut length, total DAO activity was also enhanced by +141% in segment B (P less than 0.05 vs controls) and by +87% in segment C (P less than 0.01 vs controls) of resected rats. In the duodenum, the changes in DAO activity were small (+36%) and not significant.(ABSTRACT TRUNCATED AT 250 WORDS)

Amine Oxidase (Copper-Containing)↗

Ontogeny of secretory component in rat liver.

The ontogeny of the expression of secretory component (SC), the receptor for transepithelial transport of polymeric immunoglobulins, in the rat liver was assessed by measuring the SC concentration in liver homogenates of rats killed from birth to 60 days of age, and by quantifying the daily SC secretion of in vitro cultured hepatocytes isolated from rats similarly killed from 2 days before birth to 60 days of age. With both methodologies, only very small amounts of SC were produced by the rat liver up to 20 days of age, whereafter expression of SC markedly increased. The development of SC production in the rat liver was co-ordinate with that previously reported in the rat small intestine, preceding the appearance of large numbers of intestinal IgA plasmacytes. These data further emphasize the importance of the liver contribution, via the bile, to the intestinal secretory IgA system of the rat.

Aging↗

Intestinal development in the suckling rat: effect of insulin on the maturation of villus and crypt cell functions.

The influence of insulin on the postnatal development of intestinal functions linked to villus cells (sucrase, lactase, maltase and aminopeptidase) and crypt cells (secretory component of immunoglobulins, SC) has been studied in suckling and weanling rats. At 9 days of age, the animals received a daily injection of insulin 12.5 mU g-1 body weight day-1 for 4 days. Compared with saline-treated controls, insulin had no effect on the development of the intestinal mucosal mass parameters determined in the jejunum, ileum and colon. A premature appearance of sucrase was noted in isolated jejunal villus and crypt cells, the level of activity reached by the enzyme being dependent of the amount of insulin injected. By 6 and 12 h after a single injection of the hormone (12.5 mU g-1 body weight), sucrase activity was detected in all the cell fractions along the villus-crypt axis. In villus cells of insulin-treated rats, maltase, lactase and aminopeptidase activities were significantly (P less than 0.001) increased (+201%, +50%, +207%, respectively, vs. controls), whereas the concentration of SC measured by a sensitive immunoradiometric assay was enhanced over the controls by 75% in villus cells, 83% in crypt cells and 172% in the liver. Weanling rats treated from day 10 to day 20 postpartum with 12.5 mU insulin also exhibited a higher intestinal production of SC (+93%, P less than 0.01) than did saline controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Aminopeptidases↗

Double-blind controlled study on the efficacy of sodium alginate (Gaviscon) in reducing gastroesophageal reflux assessed by 24 h continuous pH monitoring in infants and children.

We studied the effects of an alginate compound (Gaviscon) on the frequency and the duration of gastroesophageal reflux (GOR) episodes in children. Twenty infants and children with characteristic symptoms of GOR were divided at random into two groups which were given either Gaviscon (ten patients, mean age: 21 months) or a placebo (ten patients, mean age: 35 months). A continuous pH probe monitoring of the lower oesophageal third was performed in all the patients before and after 8 days of treatment. Before the trial, sensitive pH monitoring variables of acid reflux (Euler-Byrne index, percentage of total reflux time per 24 h, mean duration and percentage of reflux time during sleep, total number of reflux episodes per 24 h and number of reflux episodes per 2 h post-cibal periods) were abnormal in all the patients tested. The oesophagram revealed a GOR in 13 of the 20 patients; none of the children who underwent an endoscopy had evidence of oesophagitis. Episodes of regurgitation reported by the parents decreased during Gaviscon therapy while no clinical improvement was noticed in the placebo group. No adverse effects were observed. After 8 days of treatment with Gaviscon, results of all the pH monitoring variables were significantly (P less than 0.05) reduced between -35% and -61% of the initial values recorded. In the placebo treated group, the mean values remained little changes (-9.5 to +8.2% of initial values). These data suggest that Gaviscon may prove useful in the medical management of GOR in infants and children.

Alginates↗

Malabsorption, hypocholesterolemia, and fat-filled enterocytes with increased intestinal apoprotein B. Chylomicron retention disease.

Eight infants presented with a malabsorption syndrome, normal fasting triglycerides, hypocholesterolemia (64.3 +/- 10.0 mg/dl), and deficiency of vitamins A and E. Plasma low-density lipoprotein, apolipoprotein B, and apolipoprotein A-I were decreased. After a fatty meal, plasma triglycerides did not increase and chylomicrons could not be identified. Lipoprotein composition was characterized by normal apoproteins, high phospholipids, and low cholesterol. Increased triglycerides were present in low-density lipoproteins. Immunoperoxidase localization of apolipoprotein B on fasting biopsy specimens showed increased staining of the lipid-laden intestinal epithelial cells compared to normals. On electron microscopy after a fat load, the enterocytes contained large numbers of fat particles vesiculating the endoplasmic reticulum. These particles, morphologically similar to chylomicrons, were also present as aggregates of well-individualized lipid droplets within dilated vesicles in the Golgi zone, but were not seen in the intercellular spaces and lacteals. This recessively transmitted condition differs from abetalipoproteinemia and from the homozygous form of hypobetalipoproteinemia and may be caused by a defect in the final assembly of chylomicrons or in the mechanism of their exocytosis.

Apolipoproteins B↗