William M. Liebman

Recurrent Abdominal Pain in Children A Retrospective Survey of 119 Patients

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 per cent, school activity in 32 per cent, and perfectionism in 30 per cent. Laboratory and radiologic and fiberoptic endoscopic studies were uniformly unremarkable. Counseling on a continuing basis helped 105 of these children.

Primary Intestinal Lymphangiectasia

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, stea

Shwachman-Diamond Syndrome and Chronic Liver Disease

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.

Cholestyramine Treatment of Chronic Diarrhea Associated with Immune Deficiency Syndrome

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 treat ment with cholestyramine. Repeated courses of cholestyramine resin over a 6-month period were required for complete resolution of the gastrointestinal symptomatology.

Effect of topical acid on duodenal pepsinogen secretion in the rat.

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 μg/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).

Fetal Pepsinogens in Human Amniotic Fluid

The presence of group I (Pg I) and group II (Pg II) pepsinogens was determined in 59 samples of human amniotic fluid between 11 and 40 weeks of gestation. Pg I was present in all of the samples, while Pg II was present only in samples of gestational age 32 weeks or older. No differential pattern of the fractions was present, although the first fraction of Pg I was not present in any of the samples. The sequential appearance of Pg I and Pg II suggests their fetal origin and that the synthesis of Pg I by fetal gastric mucosa may precede that of Pg II, thus serving as a potential marker of fetal maturity.

Crinophagic inclusions in gastric chief cells of mice with Chediak-Higashi syndrome

Abstract Immunostaining for pepsinogen with an immunoglobulin-peroxidase bridge procedure has been undertaken in conjunction with cytochemical staining for complex carbohydrate to investigate the composition and nature of large inclusions in gastric chief cells of beige mice with an analog of the human Chediak-Higashi syndrome (CHS). By these methods to stain chief cells and immunostaining for carbonic anhydrase to distinguish parietal cells, stomachs of the beige mouse were compared with those of normal black mice from which the genetic defect arose. The staining has confirmed that the chief cells are involved in CHS and has affirmed that one type of megabody in CHS chief cells contains both group I and group II pepsinogens and apparently arises in a process akin to crinophagy.

607 BIOTIN DEFICIENCY: A NOVEL COMPLICATION OF PARENTERAL ALIMENTATION

All reported cases of biotin deficiency in man have been associated with prolonged ingestion of substantial amounts of raw egg white. A 12-month-old girl developed facial and perineal rash, alopecia totalis, waxy pallor, hypotonia, and irritability while receiving total parenteral alimentation (TPA) for short gut syndrome. Deficiencies of zinc and essential fatty acids (EFA) were ruled out. Biotin deficiency was documented by biotin levels and urinary excretion of organic acid.The clinical and biochemical abnormalities resolved with biotin supplementation alone and did not recur with supplementation at normal biotin requirements (0.1 mg/day). Acquired deficiency of biotin, as well as zinc and EFA, must now be considered when rash and alopecia appear in patients receiving TPA.

Serum Group I Pepsinogens in Children with Recurrent Abdominal Pain

Recurrent abdominal pain (RAP) is a common, frustrating problem in child hood. 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 Pgl levels in these groups. These results suggest that acid hypersecretion cannot be demonstrated in RAP; therefore its relationship to RAP is questionable.