Claude L. Morin

Chronic intermittent elemental diet improves growth failure in children with Crohn's disease

Growth failure often complicates Crohns disease in pediatric patients and is principally due to inadequate caloric intake. To assess whether intermittent courses of an elemental diet could reestablish growth, 8 children (aged 9.8-14.2 yr) with Crohns disease and growth failure entered into a prospective trial. Each patient was studied during an observation year on standard therapy, then for an experimental year during which they received enteral elemental diet 1 out of 4 mo. An age- and disease-matched control group of 4 patients was treated by conventional medical therapy during both years. Elemental diet therapy was administered nocturnally, at home, by continuous nasogastric infusion and increased the daily caloric intake by 25% (p less than 0.01). Anthropometric measurements demonstrated significant height and weight gains in the elemental diet group vs. controls (p less than 0.01). Crohns disease activity index and prednisone intake decreased significantly in patients receiving elemental diet therapy when compared with themselves and with controls on conventional medical therapy (p less than 0.05). In contrast, the rate of pubertal development was similar in both groups irrespective of the treatment modality. This study demonstrates that chronic intermittent elemental diet effectively reverses growth arrest, while decreasing prednisone requirements and Crohns disease activity index in pediatric Crohns disease patients prior to puberty.

One-hour blood-xylose test in diagnosis of cow's milk protein intolerance.

In this prospective study, 18 consecutive children suspected of cows milk protein intolerance (C.M.P.I.) on clinical grounds were investigated before and after challenge with cows milk. One-hour blood-xylose, serum-IgE, eosinophil count, serum-complement (C3) and a jejunal biopsy specimen for histology and disaccharidase activity were obtained from all patients before challenge. Serum-complement was measured again 24 h after the beginning of the challenge and the first three tests were repeated on the fifth day. A second jejunal biopsy was obtained in 9 of the first 10 children. 15 of the 18 children were considered to have C.M.P.I. The one-hour blood-xylose test emerged as a simple and valuable test in the diagnosis of C.M.P.I. A significant drop (51--81%) in the one-hour blood-xylose level was observed in all 15 patients. In all patients but one it dropped below 25 mg/dl. Mean value (+/- S.D.) for the group was 47.5 +/- 11.1 mg/dl before and 17.2 +/- 4.2 mg/dl after cows milk ingestion.

Malabsorption of Bile Acids in Children with Cystic Fibrosis

Abstract Quantitative fecal bile acids (in milligrams per 24 hours per square meter of body-surface area) in 24 children with untreated pancreatic insufficiency secondary to cystic fibrosis were increased (743.2 ± 55.3 — mean ± S.E.) whereas those in 12 cases of celiac disease and in seven with chronic nonspecific diarrhea did not differ from values in 18 controls (110.0 ± 11.0). Bile acid loss in cystic fibrosis was comparable to that of six infants after ileal resection (957.7 ± 195.1). A large increase (p<0.01) in fecal bile acids occurred when pancreatic enzyme therapy was withdrawn in five patients with cystic fibrosis. These data and the good correlation (r = 0.78) found in cystic fibrosis and not in celiac disease between fecal fat and bile acids suggest that unhydrolyzed fat or other products of maldigestion may interfere with bile acid absorption. The daily fecal bile acid sequestration of 1.9 g and 3.7 g found in two cases of cystic fibrosis studied during pancreatic supplementation indicates th...

Hirschsprung's Disease in a Family with Multiple Endocrine Neoplasia Type 2

SummaryGastrointestinal symptoms are known to occur in association with multiple endocrine neoplasia (MEN) type 2. Patients with the variant 2b are at special risk and show diffuse intestinal ganglioneuromatosis. Megacolon is frequently an early X-ray finding and is associated with hyperplastic gang

Small intestinal and colonic changes induced by a chemically defined diet.

Three groups of rats were fed, respectively, a chemically defined diet intragastrically (IG), an equivalent diet intravenously (IV) and solid food orally (CH) for 8 days, and their small intestines and colons compared. All received equal calories. The small intestine was divided into equal proximal (A), middle (B), and distal (C) segments for measurements. Mucosal weight per cm in segments A and B of IG were, respectively, 65 and 38% higher than in IV (P<0.01), but 27 and 33% lower than in CH (P<0.01). However, in the distal segment, C, mucosal weight in IG was similar to IV and CH was 79% higher (P<0.01). DNA and protein followed the same pattern. Segment A sucrase activities were similar in CH and IG and were much higher than in IV (P<0.01). Sucrase in IG dropped very rapidly distally so that it became much lower than in CH (P<0.05) and similar to IV. Mucosal weight, DNA, and protein in the colon were not significantly different in IG and IV, which were both signficantly lower than in CH (P<0.01). The results indicate that a chemically defined diet maintains intestinal mass well in the proximal small intestine, but the effect diminishes rapidly in a distal direction so that distal small intestine and colon become atrophied and similar to those in intravenous feeding.

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.

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.

Malabsorption, hypocholesterolemia, and fat-filled enterocytes with increased intestinal apoprotein B

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.

Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis

Ten children with cystic fibrosis, aged 3.5 to 12 years, whose weights were lower than 90% of the expected weight for height, received high-calorie elemental enteral alimentation for four weeks. Clinical, anthropometric, and biochemical evaluations as well as blood gas analyses and chest radiograph scoring were performed in all. Pulmonary function tests were performed in the five older children, and progressive exercise tests in three. These evaluations were done before, immediately after, and two months after termination of therapy. Nutritional therapy resulted in an increase of caloric intake and in dramatic weight gain, which persisted only for a short time and was mainly related to adipose tissue accretion. No functional improvement accompanied the amelioration in nutritional status. This short-term nutritional therapy in malnourished children with cystic fibrosis was effective in increasing relative weight and energy stores, but there was no evidence of any long-term functional benefit.

Gastric Emptying and Lingual Lipase Activity in Cystic Fibrosis

Summary: 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 ≤0.05) in CF patients than in controls with values (± ± S.E.) of 34.48 ± 11.59 and 12.65 ± 5.60 μmole 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 ≤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.Speculation: In view of the normal gastric emptying time, high lingual lipase activity, and significant degree of hydrolysis of a fatty meal taking place in the stomach of CF patients, the use of acid-resistant lingual lipase supplements could be considered as a new approach to the treatment of the lipolytic phase defect associated with pancreatic insufficiency in cystic fibrosis.