Eldad Rosenthal

Use of Saliva in Home Monitoring of Carbamazepine Levels

Summary: Total carbamazepine (CBZ) levels in serum of 61 epileptic children were compared with saliva levels. Both resting and stimulated saliva was analyzed. The salivary levels were 38.6% of serum CBZ levels. A highly significant correlation was noted (r= 0.89, p < 0.001). Stimulation had no effect on saliva CBZ levels (r= 0.97). Salivary and serum CBZ levels were not affected by storing the samples for 7 days at room temperature. The data indicate that salivary CBZ may provide a reliable alter‐native monitoring method to Tegretol therapy, especially in children, in whom blood sampling is difficult. Further‐more, the samples may be collected at home and delivered to the laboratory by mail.

SEVERE PULMONARY DISEASE IN ASSOCIATION WITH CROHN’S DISEASE IN A 13-YEAR-OLD GIRL

Pulmonary manifestations of Crohns disease are infrequent in adults and even less common in children. Our literature search found only a few cases of Crohns disease causing pulmonary manifestations in children. We report on the case of a 13‐year‐old girl in whom severe pulmonary disease was found four years after the onset of Crohns disease. Open lung biopsy uncovered bronchiolitis obliterans and granulomatous lung disease. Aggressive treatment has yielded gradual improvement. This case emphasizes the importance of recognizing the association, the differential diagnosis, and treatment implications. Pediatr Pulmonol. 2000; 29:151–154.

Progressive familial intrahepatic cholestasis among the Arab population in Israel.

BACKGROUND Progressive 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. METHODS Sixteen affected children from six Israeli Arab families were evaluated over 30 years. All were born to consanguineous parents. RESULTS Jaundice 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. CONCLUSIONS Progressive 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.

BACKGROUND To evaluate the effect of a therapeutic regimen of 7 days versus 14 days on the clinical manifestations of Helicobacter pylori gastritis in children. METHODS Ninety 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. RESULTS A 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. CONCLUSIONS A 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.

Human parvovirus B19 infection associated with idiopathic thrombocytopenic purpura in a child following liver transplantation

BACKGROUND/AIMS Idiopathic (autoimmune) thrombocytopenic purpura has been previously reported as a rare complication in children following parvovirus B19 infection. In the immunocompromised host who is unable to produce neutralizing antibody, an infection with parvovirus B19 can persist and cause chronic bone marrow failure. METHODS We describe a child who had undergone liver transplantation and who had idiopathic thrombocytopenic purpura, whose history and laboratory findings suggested parvovirus B19 infection. The infection disappeared without persistent viremia, and the thrombocytopenia responded completely to the administration of gamma globulin while the patient was undergoing chronic immunosuppression therapy. RESULTS/CONCLUSION Transplant physicians need to be aware of this complication, and parvovirus B19 infection should be included in the differential diagnosis of liver recipients presenting with severe thrombocytopenia.

Cholestatic pruritus: effect of phototherapy on pruritus and excretion of bile acids in urine.

Pruritus associated with hepatic cholestasis may cause significant morbidity and its correlation to retention of bile acids in skin is inconsistent. Available treatment modalities are only partially effective and can have several adverse effects. Phototherapy has recently been reported to improve cholestatic pruritus, but has not been evaluated previously in children, and its mechanism is still unclear. We report the outcome of multiple Daylite phototherapy treatments over two years in a seven‐year‐old child with chronic hepatic cholestasis that was resistant to other therapeutic modalities. Bile acid levels in urine were used as markers of effectiveness in parallel with clinical response. Night phototherapy alone increased the bile acids/creatinine ratio in urine from 1.54 & 0.04 pmol/mg at baseline to 2.07 ± 0.29 pmol/mg. Continuous phototherapy combined with night diuresis raised the ratio further to 2.28 ± 0.55 μmol/mg. Night diuresis alone had no effect. Continuous phototherapy combined with night diuresis raised the bile acids/creatinine ratio by 44% on the first day and by 61 % on the second day, but declined to baseline on the third day of treatment. A marked clinical improvement was noted for one week following two days of phototherapy. This schedule has been repeatedly effective in improving pruritus for approximately one year and may be due to the ability of phototherapy to enhance excretion of bile acids and other possible pruritogens into urine.

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.