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A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common iliac lymph nodes. Abdominal CT scan revealed a possible periaortic mass. Small intestinal biopsies demonstrated normal villous architecture with dilated lacteals, and electron microscopy revealed enterocytes with normal as well as blunted microville, enlarged, dilated lacteals and intercellular vacuoles. An exploratory laparatomy was performed and revealed no masses but dilated serosal lymphatic vessels. Medical treatment, including marked restriction of long chain triglyceride intake, resulted in loss of peripheral edema, weight stabilization, and normal activity.

Thirty-eight consecutive children with recurrent abdominal pain underwent lactose tolerance tests; 28 of these were also given sucrose tolerance tests. Abdominal pain and abnormal lactose tolerance tests were noted in 11 of 38, while none of the 28 had an abnormal sucrose tolerance test; however, 1 had abdominal pain. Elimination diet for 4 weeks produced significant or total pain relief in 10 of 11 (lactose free) and 0 of 1 children (sucrose free). Lactose intolerance seems to play a contributory role in recurrent abdominal pain in children, while sucrose intolerance does not.

Fiberoptic colonoscopy was performed on 15 patients between the ages of 1 1/2 years and 16 years. Ten patients were hospitalized and five were outpatients. Of 12 with frank or microscopic blood in stools, fiberoptic colonoscopy revealed single polyps in six patients, ulcerative colitis in two and negative results in four with prior nondiagnostic radiographic studies, colonoscopy revealed ulcerative colitis in one, granulomatous colitis in one and negative findings in one. Polypectomy through the colonoscope was accomplished in all six patients with polyps. Perforation of the sigmoid colon during polypectomy with the snare loop was the single complication encountered. Lower intestinal endoscopy should be selectively considered for diagnosis and therapy of unexplained bleeding or recurrent lower abdominal pain in children in whom proctosigmoidoscopic laboratory and radiographic examinations do not achieve a diagnosis.

Fiberoptic endoscopy of the upper gastrointestinal tract was performed on 53 patients without incident, ranging in age between two months and 18 years, of whom 35 were out patients at the time of examination. Of 27 patients with recurrent abdominal pain and normal upper gastrointestinal series, eight had abnormal findings at endoscopy: a duodenal ulcer in four, a gastric ulcer in two and duodenitis in two. Of 18 patients with hematemesis and/or melena, esophageal varices were demonstrated both by endoscopy and x-ray in two, gastric ulcer by endoscopy in three and x-ray in one, duodenal ulcer by endoscopy in three and by x-ray in two, esophagitis by endoscopy only in one patient, erosive gastritis by endoscopy in five and by x-ray in two and duodenitis by endoscopy in three and by x-ray in two. Of the remaining eight patients with abnormal x-rays findings and other symptomatology, endoscopy demonstrated foreign bodies in two (coins, esophagus and stomach), duodenitis in two, a gastric ulcer in one, a duodenal ulcer in one and normal examination in two. The data indicate that fiberoptic endoscopy significantly improves diagnostic accuracy in the evaluation of disorders of the upper gastrointestinal tract in children and is a safe and effective procedure in ambulatory pediatric patients.

The reported patient represents an example of extensive colonic disease at an uncommon age of involvement. The significant response to salicylazosulfapyridine therapy with this degree of involvement at this age is uncommon. This case suggests the value of an initial trial of medical therapy with salicylazosulfapyridine before the use of steroids or colectomy is contemplated in infants with ulcerative colitis.

Intestinal coccidiosis in a 6-mo-old infant terminated fatally after 30 wk of continuous total parenteral nutrition, and proved refractory to treatment with antibiotics, hydrocortisone, and antimetabolic agents. Intestinal biopsies obtained at laparotomy revealed flattened mucosa infiltrated with coccidia at various stages of the parasites' life cycle. The course was characterized by severe diarrhea due to a cholera-like hypersecretion of intraluminal fluid. This case suggests that intestinal coccidiosis may be included among the small number of conditions responsible for authentic "intractable diarrhea of infancy."

Serum IgE levels were measured in 25 consecutive children with recurrent abdominal pain (RAP) of undetermined cause and 25 consecutive controls of similar age and sex without evidence of RAP, allergy, or parasitic infection. No significant difference in serum IgE levels was demonstreated in children with and without RAP.

Intolerance to lactose and cow's milk protein was prospectively evaluated in 56 infants with apparent colic. A complete blood cell count, sedimentation rate, test for quantitative IgE level, radioallergosorbent test (performed in 15 patients), and at least three stool examinations for pH, presence of reducing substances, and occult blood were performed in 56 infants (46, appropriate size for gestational age; ten, small for gestational age) and results were within normal limits for age. This study suggests that tolerance to lactose and cow's milk protein does not have a significant role in infantile colic.

Recurrent abdominal pain (RAP) is a common, frustrating problem in childhood. A commonly mentioned cause has been acid hypersecretion without evidence of actual ulceration. Recently, a radioimmunoassay specific for group I pepsinogens (PgI), one of two immunochemically distinct groups of human pepsinogens or precursor zymogens of pepsin, has been developed. Serum PgI levels have been demonstrated to reflect the acid secretory capacity of gastric mucosa, specifically the maximal and peak acid outputs (MAO, PAO), as well as the basal acid output (BAO), thus providing an accurate, tubeless determination of acid secretion. The present study of children with and without RAP has revealed no significant difference in serum PgI levels in these groups. These results suggest that acid hypersecretion cannot be demonstrated in RAP: therefore its relationship to RAP is questionable.

In a series of 123 children with disorders of defecation, constipation was the main problem in 89 and fecal incontinence in 34. All but three of the latter also had constipation. The principal physical findings were abdominal protuberance with palpable stool in the bowel and the presence of a fecal mass in the rectal ampulla. Laboratory and radiologic findings were nonspecific except in two of the three patients found to have congenital megacolon (Hirschsprung's disease). Rectal suction biopsy, performed in 69 patients, was diagnostic in all three with Hirschsprung's disease. Anorectal manometry, done in 11 patients, was of limited value. In general, treatment consists of patience and understanding on the part of physician and family, a regimen of orally administered (noncathartic) laxatives with dose gradually reduced over weeks to months, frequent telephone communication between physician and parents, and occasional office visits. Patients with nonretentive fecal incontinence require an intensive psychologic approach. A patient, empathetic, and available physician is one of the most important ingredients in the treatment regimen.

A 5-year-old boy with known severe combined immunodeficiency disease presented with chronic diarrhea, malabsorption and retarded growth. Candida albicans was found in distal duodenal fluid, and invading the intestinal mucosa. Chronic diarrhea persisted after antimycotic therapy, but responded to treatment with cholestyramine. Repeated courses of cholestyramine resin over a 6-month period were required for complete resolution of the gastrointestinal symptomatology.

The sensitivity of the string test for detection of gastroesophageal reflux was assessed in 15 infants and children, 5 months to 11 years of age (mean, 2.8 years), with vomiting, regurgitation, or lower respiratory tract symptoms, and compared with the barium esophagram, esophageal manometry, and the acid reflux test (pH probe). Barium esophagram showed reflux in 4/15 patients, pH probe in 7/15, and string test in 6/15. The string test is a simple, rapid, safe, and efficient screening method for patients with suspected gastroesophageal reflux.

A four-year old boy with achalasia of the esophagus was successfully treated with pneumatic dilatation, using the standard Browne-McHardy dilator, without incident. His subsequent course over 10 months has been uneventful.

To determine the efficacy in assessing bacterial overgrowth, the string test (EnteroTest-Pediatric) and duodenal aspiration were compared in 18 children with chronic diarrhea. Isolation and significance of organisms in the small intestine were identical with both methods in all patients. These data suggested that the string test is an accurate, simple, and safe method for assessment of small-intestinal microflora.

The effect of topical acid on duodenal pepsinogen secretion was studied in the anesthetized rat. Perfusion of a 5-cm segment of the proximal duodenum with normal saline or buffered saline (pH 7.2 or 6.0) elicited no detectable pepsinogen response. Perfusion with 10, 25, and 100 mN HCl resulted in a graded increase in pepsinogen output. Acetylcholine bromide, 500 microgram/ml, in buffered saline, pH 7.2, also stimulated pepsinogen secretion. The pepsinogen response to 100 mN HCl and to acetylcholine was completely abolished by atropine. Secretin, 2 units/kg, did not alter pepsinogen output during perfusion with buffered saline or acid, while secretin, 75 units/kg, increased pepsinogen output. These observations suggest that topical acid stimulates duodenal pepsinogen secretion through a cholinergic reflex and that secretin is not a significant stimulant of duodenal pepsinogen secretion in the rat within the dose range employed (1--2 units/kg).

The clinical pattern of 119 children with recurrent abdominal pain, located most commonly in the periumbilical region, revealed no distinct features. The most common associated symptoms were pallor, tiredness and anorexia. The most important socioenvironmental and behavioral factors were marital turmoil in 44%, school activity in 32%, and perfectionism in 30%. Laboratory and radiologic and fiberoptic endoscopic studies were uniformly unremarkable. Counseling on a continuing basis helped 105 of these children.

Basal and histalog-stimulated gastric acid secretion and serum gastrin levels before and after a standard protein meal were compared in eight children with active duodenal ulcer (DU), four with active gastric ulcer (GU), and in seven children with recurrent abdominal pain (RAP) of undetermined etiology. There was no discernible difference in the pattern of abdominal pain in DU, GU, and RAP. Basal acid output, peak and maximal acid output, whether expressed as milliequivalents per hour or as milliequivalents per kilogram per hour, were comparable in children with DU, GU, and RAP. In contrast, serum gastrin levels, 1 and 2 hours after standard protein meal, were significantly higher in the DU children than in the GU or RAP group. These studies have suggested that hypersecretion of gastric acid may not be associated with duodenal ulcer or gastric ulcer disease in children, and that increased gastrin secretion and possible reduced acid responsiveness coexist in children with duodenal ulcers.

Clinically inapparent persistent chronic liver disease in a 15-month-old male patient with Shwachman-Diamond syndrome is presented. Cryptic hepatic involvement may be an unrecognized feature of the syndrome and should be evaluated in all cases.

The antigenic relationships and cellular localization of cat and dog pepsinogens were investigated by electrophoretic analysis, immunodiffusion, immunoelectrophoresis, immunoabsorption, and by immunofluorescence, respectively. Rabbit antiserum to human and hog group I (Pg I) and group II pepsinogens (Pg II) had been previously prepared. Electrophoretic analysis revealed at least eight distinct proteases in extracts of gastric and proximal duodenal mucosa, resistant to alkalinization but destroyed by sequential accidification and neutralization. Rabbit antiserum to Pg I (anti-Pg I) and Pg II (anti-Pg II) produced a single precipitin arc against each extract forming a line of nonidentity. Immunoelectrophoresis of extracts produced a single precipitin arc against anti-Pg I or anti-Pg II. The specificity of the antibodies for the group I or group II pepsinogens was confirmed by immunoabsorption. By immunofluorescnece, both Pg I and Pg II were present in mucous neck and chief cells in fundic mucosa, in the pyloric gland cells in antral mucosa, and Brunner's glands in the proximal duodenum. The results indicate that canine and feline pepsinogens are electrophoretically heterogenous, that canine and feline Pg I share antigenic determinants with each other but not with Pg II, that a similar positive relationship exists for Pg II, and that both Pg I and Pg II are localized to the peptic cell mass, consisting of four types of cells.

A case of upper gastrointestinal bleeding in the first 24 hours of life is discussed. The application and importance of upper gastrointestinal fiberoptic endoscopy in establishing a precise diagnosis in this age group, not previously reported, is demonstrated.

Serum group I pepsinogen (PG I) levels have been determined by radioimmunoassay in 924 subjects. The mean levels in 300 healthy control subjects and in 389 hospitalized controls were 110.6 and 100.0 ng per ml, respectively. The "normal" range is estimated to be between 50 and 175 ng per ml. The mean level of serum PG I in 7 patients with Zollinger-Ellison syndrome was 503.9 ng per ml; values ranged between 315 and 921 ng per ml. The 77 patients with duodenal ulcer had a mean serum PG I level of 221.3 ng per ml; 49 (63.6%) had values greater than 175 ng per ml. The distribution of serum PG I values was bimodal in the patients with duodenal ulcer whereas it was unimodal in both groups of control subjects. Mean serum PG I levels in 13 patients with both duodenal and gastric ulcer and in 18 patients with prepyloric ulcer were, respectively, 177.2 and 179.4 ng per ml. Approximately one-half of these patients had high values. The 28 patients with gastric ulcer had a mean serum PG I level of 116.6 ng per ml; 6 (21.4%) had high values. With the exception of 3 patients with gastric ulcer, none of the 136 patients with peptic ulcer had a low (less than 50 ng per ml) level of serum PG I. In 37 patients with chronic alcoholism the mean level of serum PG I was 73.4 ng per ml. The observed gradient in the mean level of serum PG I among the groups of patients studied is similar to that which has been reported for maximally stimulated gastric acid output. This finding suggests that the secretory potential of the fundic gland mucosa of the stomach may be reflected by the level of PG I in serum.