William E. Berquist

Effect of theophylline on gastroesophageal reflux in normal adults

In a randomized, double-blind manner, 15 normal adults were given a single oral loading dose of anhydrous theophylline (6.5 +/- 0.9 mg/kg) and nine normal adults were given placebo. All subjects were tested for serum theophylline levels, acid reflux (using intraesophageal pH probe), and lower esophageal sphincter pressure (LESP) at 1.5, 4, and 8 hr after treatment. Of subjects with negative baseline acid reflux tests, eight of 13 (61.5%) given theophylline developed positive acid reflux tests compared with none of eight subjects given placebo. One of nine (11%) placebo subjects reported heartburn compared with 11 of 15 (73%) subjects given theophylline. The maximum mean percent change in LESP from baseline was +5.62 +/- 28.8% in the placebo group and -25.01 +/- 23.7% in the theophylline group (p = 0.01) at 4 hr after treatment. While only two of nine (22%) adults given placebo had at least a 14% reduction in LESP following treatment, all of the 15 subjects sustained a minimum of 14% relaxation in LESP. This study confirms that oral theophylline at therapeutic serum levels inhibits LESP and induces gastroesophageal reflux (GER) measured by acid reflux tests in most normal adults.

Sclerotherapy for extrahepatic portal hypertension in childhood

Ten children with extrahepatic portal hypertension who had major bleeding from esophageal varices were treated with sclerotherapy of esophageal varices by means of flexible fiberoptic endoscopy and intravenous sedation. Four had had no previous therapy, five had had previous surgery for variceal bleeding, and five had received propranolol orally. During therapy and follow-up monitoring of 1.4 to 7.1 years (mean 4.7 years), only two patients bled again from esophageal varices, one before complete obliteration of varices and one who temporarily defaulted on follow-up. The few complications were easily managed, and only three required any specific therapy. No child bled from gastric varices. Frequency of sclerotherapy sessions and quantity of sclerosant could be decreased with time, usually after 3 years of sclerotherapy, suggesting that the natural history of decreased bleeding with time in extrahepatic portal hypertension may be accelerated by sclerotherapy. Esophageal varices in children with extrahepatic portal hypertension may be treated safely with sclerotherapy, which is effective in preventing chronic and recurrent gastrointestinal bleeding.

Surgical Management of Chronic Intestinal Pseudo-obstruction in Infancy and Childhood

Chronic intestinal pseudo-obstruction is an uncommon cause of repeated obstruction in children of undetermined etiology, often leading to repeated laparotomies and early death. TPN combined with venting gastrostomy provides sufficient calories for growth and minimizes the need for operations and/or hospitalizations. Seventeen children managed with these techniques experienced a sixfold decrease in the number of hospitalizations, and more than a tenfold decrease in the number of laparotomies for obstruction compared to the period before routine use of TPN and gastrostomies. Oral feedings are encouraged whenever possible.

Gastric emptying of mixed solid-liquid meal in patients with intestinal pseudoobstruction

Gastric emptying of solids and liquids was evaluated simultaneously in 11 patients with intestinal pseudoobstruction. Despite a normal upper gastrointestinal series in most patients, over two thirds of the patients had abnormal gastric emptying of solids and/or liquids. Half these patients exhibited delayed emptying of solids and accelerated emptying of liquids. Alterations in motility patterns were observed in the four patients tested including decreased frequency or absence of migrating motor complexes originating in the stomach, shortening or absence of phase I, and loss of distinct fasted and fed patterns. Both basal values and mean postprandial increments in serum gastrin concentrations were lower in the patients, whereas no significant difference was found for pancreatic polypeptide concentrations when compared to normals. Our findings emphasize the functional heterogeneity of patients with intestinal pseudoobstruction. In addition, in contrast to previous reports which used barium meals to access gastric function, we observed a high prevalence of gastric emptying disturbances in these patients, suggesting that the motility disturbance is not restricted to the small intestine.

Somatic growth and developmental functioning in children receiving prolonged home total parenteral nutrition

Growth and psychomotor development in nine infants receiving prolonged home total parenteral nutrition (TPN) were studied longitudinally from infancy to 3 years of age. Although these children had received TPN for, on average, 79% of their lives, normalization of somatic growth occurred by 2 years of age in all of them. Three children maintained average or above average levels of developmental performance over the 3-year study period. Another four children had initially delayed development but average or above average developmental scores by 18 months. In two children the rate of development gradually slowed without obvious cause, so that by 3 years they functioned in the below average to mildly retarded range.

SZ phenotype alpha-1-antitrypsin deficiency with paucity of the interlobular bile ducts

Abstract A child is reported whose alpha‐1 ‐antitrypsin phenotype is SZ and who has chronic cholestatic liver disease that began in the neonatal period. Liver biopsy demonstrated paucity of the interlobular bile ducts, marked hepatocellular deposition in periportal areas of PAS‐positive, diastase‐resistant granules, and bridging portal fibrosis. The association of paucity of the interlobular bile ducts with SZ phenotype alpha‐1‐antitrypsin deficiency has not been reported previously.