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Biomedical subjects

A M Weber

Publications and source records attributed to A M Weber.

At least 73 records · Page 4Linked to original sources
PubMed1986

Secretory diarrhea with protein-losing enteropathy, enterocolitis cystica superficialis, intestinal lymphangiectasia, and congenital hepatic fibrosis: a new syndrome.

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na+ 108 mEq/L, Cl- 85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

Anti-Bacterial Agents↗
PubMed1985

Bile acid metabolism in children with cystic fibrosis.

Recycling of bile acids through the enterohepatic cycle is very efficacious. Bile acids contribute to bile formation and, by forming micelles, participate in lipid solubilization and absorption. The small fraction which escapes in the feces, is synthesized daily by the liver to compensate for losses. In CF, bile acid malabsorption has been documented; these large losses are accompanied by an interruption in the enterohepatic circulation with concomitant reduction in bile acid pool and disturbances in biliary composition. The various intraluminal factors implicated in bile acid malabsorption include: unhydrolysed triglycerides and phospholipids, precipitation of bile acids in acidic duodenal content, adsorption to residues and modification of colonic microflora. A defect in bile acid ileal uptake has also been advocated. These disturbances in bile acid metabolism associated with CF might lead to aggravation of diarrhea and steatorrhea, cholelithiasis and perhaps liver disease.

Absorption↗
PubMed1983

Capacity to vary writing hand/posture in relation to the Levy and Reid model for control of writing.

Inherent in Levy and Reid's (1978) neurological model for the control of writing hand/posture were implications pertaining to the capacity of people to vary their hand/posture when writing. These implications were formulated into the writing variation hypothesis. The skill with which right-normal, left-inverted, and left-normal writers were able to write using hand/postures other than their preferred one was examined. The resulting performance was incompatible with Levy and Reid's neurological model. Whether or not subjects wrote with their preferred hand seemed more important than which posture they used.

Journal Article↗
PubMed1983

Relapsing pancreatitis in association with Crohn's disease.

Two adolescent girls, aged 15 and 18, in whom the diagnosis of Crohn's ileocolitis had been made 6 months and 3 years previously, developed acute pancreatitis with relapses of varying duration and severity. The younger patient's condition progressed to chronic relapsing pancreatitis with intractable pain despite partial pancreatectomy. The other has had recurrent acute attacks but has been well between bouts. No duodenal involvement could be found in the two cases and there was no evidence to implicate drugs as a factor responsible for this rare association between pancreatitis and Crohn's disease.

Acute Disease↗
PubMed1982

Hepatobiliary disease in cystic fibrosis: a survey of current issues and concepts.

The incidence of hepatobiliary complications of cystic fibrosis (CF) has been increasing in parallel with the rate of survival. Detection of hepatic involvement remains a problem, as liver function tests, serum bile acid determinations, and ultrasonography do not permit an early diagnosis. The pathogenesis of cholelithiasis has been elucidated in the past few years. However, the mechanism leading to the pathognomonic CF lesion, focal biliary cirrhosis, is still unknown. There are indications that mucus plugging may be the ancestral lesion and the triggering factor. The possibility that correction of the abnormalities of bile acid metabolism could slow the progress or prevent CF cirrhosis is discussed in light of recent experimental data.

Adolescent↗
PubMed1981

Severe familial cholestasis in North American Indian children: a clinical model of microfilament dysfunction?

Studies of 14 North American Indian children with a familial type of severe neonatal cholestasis are described. Jaundice occurred during the neonatal period in 9 children, but disappeared before the end of the 1st yr. Progressive liver damage was documented by the persistence of high levels of alkaline phosphatase, moderate elevation of transaminases, and severe pruritus. Serum bile acids were constantly elevated (3.0-119.5 microgram/ml). Early portal hypertension and variceal bleeding necessitated portal-systemic shunts in 7 children. By light microscopy, the early stage was characterized by hepatitis with giant-cell transformation and biliary stasis. Later on, portal fibrosis became evident and was followed by cirrhosis. By electron microscopy bile canaliculi appeared slightly dilated with preservation or only partial loss of microvilli. They were surrounded by a prominent pericanalicular filamentous web. Immunofluorescence studies indicated the presence of action-containing microfilaments. This group of children might represent a human model of microfilament dysfunction-induced cholestasis.

Actins↗
PubMed1981

Ménétrier's disease in children: report of a patient and review of sixteen other cases.

Ménétrier's disease is one of the rarer causes of protein-losing enteropathies seen in childhood. At onset of symptoms, the present case and 15 others reported so far were younger than 10 yr of age. Edema was usually more conspicuous than gastrointestinal symptoms. In contrast to the usual chronicity of the disease in adults, the illness is self-limited, transient, and benign.

Child↗
PubMed1981

Separation of sulfated from non-sulfated serum bile acids without the use of Sephadex columns.

Methods currently in use for the quantitative measurement of sulfated serum bile acids usually lead to low recoveries. The present technique uses two internal standards and involves enzymatic hydrolysis followed by extraction of the non-sulfated fraction in ether at pH 4.6. The sulfated fraction is then solvolysed before gas-liquid chromatographic analysis of the individual bile acids of both fractions. Using 200 microliters of serum, recoveries of more than 81% of sulfated bile acids were achieved. This method is of particular significance in view of recent evidence showing that certain sulfated bile acids are cholestatic.

Amidohydrolases↗
PubMed1980

Gastric emptying and lingual lipase activity in cystic fibrosis.

To identify gastric factors likely to contribute to fat maldigestion and malabsorption in cystic fibrosis (CF), gastric emptying time, secretion rate, and preduodenal lipolytic activity were studied. Gastric emptying of a liquid test meal and gastric acid secretion were determined in five CF teenagers with pancreatic insufficiency and in five healthy controls. During the first hr, the rate of gastric emptying exhibited a linear pattern in both CF patients and controls. Neither the emptying time nor the gastric secretion rate was different. Lingual lipase activity was measured in eight other CF patients with pancreatic insufficiency and in eight controls. Lipase activity was higher (P less than 0.05) in CF patients than in controls with values (mean +/- S.E.) of 34.48 +/- 11.59 and 12.65 +/- 5.60 mumole butyric acid min-1 ml-1, respectively. No correlation with age or body surface was observed. Intragastric lipolysis of a butterfat triglyceride test meal was fast in both groups, but more extensive (P less than 0.05) in CF patients than in controls. The data show that in CF with pancreatic insufficiency, gastric factors contributing to the first step of fat digestion are preserved. In fact, lingual lipase activity was found to be increased, and a more complete intragastric lipolysis was documented.

Adolescent↗
PubMed1977

Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes.

Because of the increased incidence of gallstones in cystic fibrosis we compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls. Eighteen of the cystic fibrosis group had cholecystograms, and only one had gallstones. In 14 patients with cystic fibrosis who had stopped taking pancreatic enzymes for one week molar percentage of lipid composition accounted for by cholesterol (mean +/- S.E., 16.3 +/- 2.9) and saturation index (2.0 +/- 0.3) were comparable to values of the cholelithiasis group and higher (P less than 0.01) than those of controls. In 12 patients with cystic fibrosis taking pancreatic enzymes, molar percentage of cholesterol (8.6 +/- 1.7) and saturation index (1.0 +/- 0.1) did not differ from those of controls; in cystic fibrosis there was a preponderance of cholic over chenodeoxycholic acid both off (1.7 +/- 0.2) and on (1.9 +/- 0.3) therapy as compared to the cholelithiasis (0.7 +/- 0.1) and control (0.8 +/- 0.0) groups. The glycine/taurine ratio of conjugated bile acids were lower in enzyme-treated patients with cystic fibrosis (3.7 +/- 0.6) than in patients off treatment (6.4 +/- 1.0), but was higher (P less than 0.01) than in controls (1.8 +/- 0.2). Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes.

Bile↗