Yehezkel Naveh

Thiamine-dependent beriberi in the thiamine-responsive anemia syndrome

IN 1969 Rogers et al. described a case of unexplained anemia in association with diabetes mellitus and sensorineural deafness.1 The anemia responded only to pharmacologic closes of thiamine. Anothe...

Progressive familial intrahepatic cholestasis among the Arab population in Israel.

BACKGROUNDnProgressive familial intrahepatic cholestasis, which constitutes a heterogeneous group of imperfectly delineated syndromes and appears to be inherited as an autosomal recessive condition, has not been hitherto reported from the Middle East, in spite of the high rate of consanguineous marriage in this region.nnnMETHODSnSixteen affected children from six Israeli Arab families were evaluated over 30 years. All were born to consanguineous parents.nnnRESULTSnJaundice appeared during the first 3 weeks of life in 15 babies. When first referred, 10 had hepatomegaly and nine had splenomegaly. A progression toward cirrhosis was the rule. Serum levels of conjugated bilirubin, liver enzymes, and alkaline phosphatase were raised; gamma-glutamyl transpeptidase levels were normal in all three infants in whom it was examined, but elevated in two siblings of another family at ages 2 and 3 years. No abnormal bile acids were detected in the serum and urine of patients. Histologic examination of the liver showed giant-cell transformation, paucity of intrahepatic bile ducts, cholestasis, fibrosis, or cirrhosis. The pattern of liver pathology differed at times among affected members within the same family. Therapeutic trials with phenobarbital, cholestyramine, or ursodeoxycholic acid were ineffective. Survival of the patients was from 5 to 18 months in four families; in the other two families, three children received liver transplants, and one is awaiting liver transplantation.nnnCONCLUSIONSnProgressive familial intrahepatic cholestasis should be included in the differential diagnosis of infants with cholestatic jaundice of unknown etiology, especially those born to consanguineous Arab parents.

Efficacy of bismuth-based triple therapy in children with abdominal pain and Helicobacter pylori gastritis.

BACKGROUNDnTo evaluate the effect of a therapeutic regimen of 7 days versus 14 days on the clinical manifestations of Helicobacter pylori gastritis in children.nnnMETHODSnNinety children (age 2-19 years) who had abdominal pain and/or recurrent vomiting were determined to have H. pylori gastritis by endoscopy, histology, and a Giemsa stain positive for H. pylori. The patients were randomized to receive amoxicillin, metronidazole, and bismuth subcitrate for 7 days (group A; 45 children) or 14 days (group B; 45 children) and were observed clinically for 19 +/- 11.5 months. Resolution of all abdominal and gastrointestinal symptoms was considered a good response.nnnRESULTSnA good response was obtained in 36 (80%) children from group A, and in 37 (82%) from group B. A recurrence of symptoms occurred in four (11%) of the responders from group A, and in six (15.2%) from group B.nnnCONCLUSIONSnA 7-day course of bismuth-based triple therapy for H. pylori gastritis in children appears to be clinically as effective as a 14-day regimen. The feasibility of a shorter therapeutic regimen may enhance patient compliance and provide a better chance of clinical benefit.

Celiac disease–associated alopecia in childhood

We report the association of celiac disease and alopecia in 3 children. In one, the alopecia developed after 4 years nonadherence to a gluten-free diet; the other 2 patients presented with alopecia. Administration of a gluten-free diet resulted in partial regrowth of hair in the first child and complete hair growth in the others.

A prospective study of serum zinc concentration in children with celiac disease

Activation of human factor VII in plasma and m purified systems: Role of activated factor IX, Kallikrein and activated factor XII. J Clin Invest 64:1056, 1979. 7. Sardharwalla JB, Fowler B, Komrower GM: Homocystinuria: Cysteine levels in the plasma. In Bickel H, Stern J, editors: Inborn error of calcium and bone metabolism, Baltimore, 1976, University Park Press. 8. Radcliffe R, Bagdasarian A, Colman R, Ncmerson Y: Activation of bovine factor VII by Hageman factor fragments. Blood 50:6l 1, 1977. 9. Hilden M, Brandt N J, Nilsson IM, Schonheyder F: Investigations of coagulation and fibrinolysis in homocystinuria. Acta Med Scand 195:533, 1974, 10. Gershwin ME, Gude JK: Deep vein thrombosis and pulmonary embolism in congenital factor VII deficiency. N EngI J Med 288:141, 1973.

Serum Igg Antibodies to Gliadin in Children with Celiac Disease as Measured by an Immunofluorescence Method

Antigliadin antibodies (AGAs) were studied in sera from 190 patients divided into five clinical groups. Group I included 28 sera from children with newly diagnosed celiac disease on a normal diet. Group II consisted of 43 sera from children with celiac disease who were fed a gluten-free diet (GFD). Group III included 25 sera from children with celiac disease who had been in remission but exposed to a gluten-containing diet (GCD). Group IV consisted of 46 sera from children with chronic diarrheal disorders other than celiac disease. Group V included 43 sera from healthy children. The observed p values proved that (a) mean titer levels of AGAs in Groups I and III were significantly higher than the mean values for all other groups (p < 0.001), and (b) the mean titer level of AGAs in Group II was significantly higher than the mean values for Groups IV and V A good correlation between the AGA titers and the morphology of the duodenal mucosa was found in children with celiac disease. The examination of IgG AGAs by the immunofluorescence technique used in our study appears to be a useful tool in the follow-up of individual patients to determine adherence to a GFD.

Effect of zinc-deficient diet of varying duration on intestinal disaccharidase activity in the rat.

To determine whether zinc has a specific role on weight gain and intestinal disaccharidase activity, 42 male Sprague-Dawley rats were assigned to one of seven groups (n =6 each). These were a baseline control group (O) that was killed to analyze initial intestinal disaccharidase (sucrase and maltase) activity, a second group (A) fed a zinc-deficient diet for 1 week, a third group (B) pair-fed control for A, a fourth group (C) fed a zinc-deficient diet for 2 weeks, a fifth group (D) pair-fed control for C, a sixth group (E) fed a zinc-deficient diet for 3 weeks, and a seventh group (F) pair-fed control for E. All experimental groups received distilled deionized drinking water, whereas control groups received zinc-enriched (25 μg of zinc/ml) distilled deionized water. Water was given ad libitum. After killing, the mucosa of the proximal half of the small intestine was analyzed for protein and disaccharidase activity, and liver, kidney, and heart were analyzed for zinc concentration. Protein content and disaccharidase activity of the jejunal mucosa in the experiment and control groups did not differ significantly. However, animals on the zinc-deficient diet demonstrated mildly depressed growth rates that were proportional to the duration of the experiment, and significantly lower zinc concentration in the kidney in the experimental groups. The data indicate that administration of a zinc-deficient diet for up to 3 weeks did not result in significant changes in intestinal mucosa protein content or in disaccharidase activity.

Clinical zinc deficiency during zinc-supplemented formula.

A 2 1/2-month old preterm infant had failure to gain weight with high caloric intake, and had generalized persistent dermatitis and mild diarrhea. The patient was being fed zinc-supplemented cows-milk-based formula (Osterfeed). High caloric intake of 8 weeks and topical treatment of 11 weeks duration were futile. A thorough investigation revealed low serum zinc concentration. Administration of zinc sulfate 150 mg/day resulted in brisk weight gain and complete clearing of skin lesions. The infant maintained normal levels of zinc 4 months after zinc therapy was discontinued, while being fed unmodified cows milk and a diet of corn flour. The probability that zinc-supplemented formulas do not meet the high zinc requirements of premature infants is raised. The importance of plasma or serum zinc examination in preterm infants with slow growth velocity or failure to gain weight despite adequate caloric intake, with or without skin lesions and diarrhea, is emphasized.

Effect of Histamine H2 Receptor Antagonists on the Secretion of Cerebrospinal Fluid in the Cat

Abstract: Following a recent report that epithelial cells of the choroid plexus possess histamine H2 receptors, the effect of cimetidine and ranitidine, histamine H2 receptor antagonists, on the secretion and electrolyte content of CSF was examined. Fifty cats were divided into one control (n = 6) and six experimental groups. CSF was collected by puncture of the cisterna magna following pentobarbital anesthesia, and its volume, concentrations of Na+, K+, Cl‐, and pH were determined. Cimetidine or ranitidine (50, 20, or 10 mg/kg) was injected intravenously 2 h after the start of the test, and their concentrations were measured in hourly blood samples and in 30‐min aliquots of CSF in the 50 mg/kg experimental groups. Whereas the secretion of CSF did not change over 6 h in the control group, it decreased significantly by 30–60 min after injection of cimetidine or ranitidine and remained low for the following 61/2 h in all experimental groups except the 10‐mg ranitidine group. Peak cimetidine and ranitidine concentrations in CSF in the 50‐mg experimental groups were noted 60 and 90 min, respectively, after intravenous injection. CSF electrolyte concentrations and pH did not change during the test in any group. We conclude that intravenous cimetidine or ranitidine can significantly reduce CSF secretion in the cat, possibly by competitive inhibition of the histamine effect on H2 receptors located on the choroid plexus epithelial cell, or by a direct effect on the capillaries of the choroid plexus.

Coexistence of Celiac Disease and Eosinophilic Gastroenteropathy

The average incidence of celiac disease in Europe is 1 case in every 1,000 live births, ranging from 1 in 250 observed in Sweden to 1 in 4,000 observed in Denmark (1). The prevalence of celiac disease in the United States is probably comparable with that of Europe (1). The idiopathic form of eosinophilic gastroenteropathy (EG) is a relatively uncommon disorder, usually affecting the upper gastrointestinal tract, that may present at any age and that often requires steroid therapy (2). Review of the literature did not reveal any association between these diseases. We describe a teenager with near-fatal asthmatic crisis at presentation.