Ilan Zahavi MD, Olga Rosezki MD, Yerah Stolkarts MD, Raanan Shamir MD, Bruria Heckelman BSc, Hedva Marcus MSc and Gabriel Dinari MD
Background: Cholestasis is a frequent problem in patients on total parenteral nutrition. Cisapride has a prokinetic effect on the biliary system, but its effect on hepatic excretory function is unknown.
Objectives: To study the effect of cisapride on TPN-induced cholestasis in a rat model.
Methods: Bile flow and bile salt secretion rate were measured in rats given TPN. There were four groups of 8 to 13 animals each. After a one hour baseline period during which all four groups received i.v. saline infusion, two groups received a TPN solution for another 2 hours, while saline was infused in the two control groups.
At the beginning of the second hour, 2 mg/kg cisapride was injected i.v. as a bolus into one experimental and one control group. Bile was collected from the common bile duct.
Results: At the end of the third hour, TPN caused a significant reduction in bile flow (P<0.02) and bile salt secretion rate (P<0.001) (61.24 vs. 50.74 µl/min/kg, and 1.173 vs. 0.799 µmol/min/kg, respectively). Addition of cisapride abolished the cholestatic effect of TPN.
Conclusions: Cisapride has a protective effect against TPN-associated cholestasis. This may have clinical significance, and further studies are warranted.
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TPN= total parenteral nutrition
Yitzhak Lotem MD, Asher Barak MD, Huda Mussaffi MD, Mordechai Shohat MD, Michael Wilschanski MD, Yakov Sivan MD and Hannah Blau MD
Background: Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in Caucasians. Typically it is a multisystem disease diagnosed by increased chloride levels on sweat testing, with mortality due mainly to progressive respiratory disease. The clinical spectrum of CF has recently been much expanded.
Genetic testing for mutant CF transmembrane regulator has revealed atypical cases where sweat test results are borderline or normal. In other patients, genetic mutations cannot be identified but abnormal CFTR function is shown using nasal potential difference measurement.
Objectives: To highlight the diagnostic and therapeutic dilemmas in cases of atypical cystic fibrosis.
Methods: We reviewed patients with atypical CF and widely varying phenotype who are managed at Schneider Children’s Medical Center of Israel.
Results: Two patients had severe lung disease but little expression in other organs. Accurate diagnosis was essential to enable aggressive therapy in a specialized center. Four other patients are in excellent general health but have symptoms limited to male infertility, heat exhaustion, pancreatitis or transient liver dysfunction, while lung disease is minimal. For these patients, careful counseling is needed to avoid unnecessary upheaval, inappropriately aggressive management, and the psychosocial implications of a CF diagnosis. These dilemmas have increased considerably in our center, as in others worldwide.
Conclusion: It is our obligation as clinicians - at the level of both primary physician and referral center - to maintain an ever higher index of suspicion for CF, tempered by a rational program of counseling and management appropriate to the individual.
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CF= cystic fibrosis
CFTR= CF transmembrane regulator
Erez Sharoni MD, Jacob Katz MD, Ovadia Dagan MD, Avraham Lorber MD, Rafael Hirsch MD, Leonard C. Blieden, Bernardo A. Vidne MD and Einat Birk MD
Background: The need for aortic valve replacement in children and young adults poses a special problem to cardiologists and surgeons. Replacing the sick aortic valve with the patient’s pulmonary valve as described by Ross has proven to be a good option in this special age group.
Objective: To review our initial experience in order to assess the short-term results.
Methods: From January 1996 to June 1999, 40 patients (age 8 months to 41 years) underwent aortic valve replacement with pulmonary autograft. Indications for surgery were congenital aortic valve disease in 30 patients, bacterial endocarditis in 5, rheumatic fever in 3, and complex left ventricular outflow tract obstruction in 3. Trans-esophageal echocardiography was performed preoperatively and post-bypass in all patients, and transthoracic echocardiography was done prior to discharge and on follow-up.
Results: There was no preoperative or late mortality. All patients remain in functional class I (New York Heart Association) and are free of complications and medication. None showed progression of autograft insufficiency or LVOT obstruction. Homograft insufficiency in the pulmonary position has progressed from mild to moderate in one patient, and three developed mild homograft stenosis.
Conclusions: The Ross procedure can be performed with good results in the young population and is considered an elegant surgical alternative to prosthetic valves and homografts.
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LVOT = left ventricular outflow tract
Lutfi Jaber MD, Bella Eisenstein MD and Mordechai Shohat MD
Background: Hypertension is one of the most prevalent vascular diseases in the adult population. It is an important determinant of atherosclerosis in adolescents and young adults. There is to date no information on blood pressure in children of the Israeli Arab population.
Objectives: To study blood pressure in Israeli Arab children and adolescents.
Methods: Blood pressure measurements were taken in the supine position in 4,488 Israeli Arab children and adolescents of both sexes aged 6–17 years. Height and weight were also determined. Correlation was investigated between systolic and diastolic blood pressure, body mass index, gender, and age.
Results: The systolic and diastolic blood pressures were similar in both sexes for all age groups up to 14 years of age. Systolic blood pressure was significantly (P<0.001) higher in males aged 15–17 years (120±13 vs. 111±12 mmHg, 123±13 vs. 113±14.0 mmHg, and 123±18 vs. 111±14 mmHg, respectively). Diastolic blood pressure was higher in males aged 15–17, with a statistically significant difference for age 17 only (75±12 vs. 69±13 mmHg). Blood pressure was elevated in 322 students in the initial screening (7.17%), with a decrease to 2.18% when this group was rescreened 2 weeks later. The systolic blood pressure in our group is higher than that in Jewish Israeli children of Asian and North African origin, and in American children. It is similar to the systolic blood pressure of European children and Jewish children born in Israel. The diastolic blood pressure in our group is higher than that in all groups of Israeli Jewish children and American children of different ethnic groups.
Conclusions: Israeli Arab children and adolescents have higher blood pressure levels than their Israeli Jewish counterparts. Further studies are required to confirm this observation.
Arie Levine MD, Yoram Bujanover MD, Shimon Reif MD, Svetlana Gass, Nurit Vardinon, Ram Reifen MD and Dan Lehmann PhD
Background: Anti-endomysial antibodies are sensitive and specific markers for celiac disease. This antibody has recently been identified as an antibody to tissue transglutaminase, an enzyme that cross-links and stabilizes extracellular matrix proteins.
Objectives: To evaluate the clinical usefulness of an enzyme-linked immunoassay for anti-transglutaminase antibodies, and to compare the results with those of AEA, the current gold standard serological test for celiac disease.
Methods: Serum samples were collected from 33 patients with biopsy-proven celiac disease and AEA tests were performed. Control samples for anti-transglutaminase were obtained from 155 patients. An ELISA test for immunoglobulin A anti-transglutaminase utilizing guinea pig liver transglutaminase was developed and performed on all sera. Cutoff values for the test were performed using logistic regression and receiver operating curves analysis.
Results: An optical density cutoff value of 0.34 was established for the assay. The mean value was 0.18±0.19 optical density for controls, and 1.65±1.14 for patients with celiac disease (P<0.001). Sensitivity and specificity of the assay were both 90%, while AEA had a sensitivity and specificity of 100% and 94%, respectively.
Conclusions: A tissue transglutaminase-based ELISA test is both sensitive and specific for detection of celiac disease.
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AEA = anti-endomysial antibody
Tal Eidlitz-Markus, MD, Avinoam Shuper, MD and Jacob Amir, MD
Background: In our experience, secondary enuresis nocturna is a common complaint among children after a motor vehicle accident. However, as these children are often brought for examination as part of an insurance compensation claim, this complaint is not always reliable.
Objective: To describe a series of children in whom secondary enuresis occurred after a motor vehicle accident.
Methods and Results: Five children were brought to our clinic for evaluation of secondary nocturnal enuresis. Review of past history revealed a car accident preceding the onset of the enuresis. All but one had additional behavioral symptoms typical of post-traumatic stress disorder. Four children had evidence of head trauma, and one had psychological but no physical trauma.
Conclusions: Nocturnal enuresis can occur after a motor vehicle accident due either to purely psychological trauma or organic head trauma. While nocturnal enuresis is generally attributed to organic causes, psychological mechanisms also play a significant role.
Yona Amitai MD, Yedidia Bentur MD, Matityahu Lifshitz MD, Pinhas Fainmesser MD, David Applebaum MD, Yehezkel Waisman MD, Nadine Cohen and Samuel D. Oman PhD
Background: Extensive cleaning of homes in Israel before Passover may result in increased exposure of children to cleaning substances.
Objectives: To evaluate the potential danger of Passover cleaning to children, and to study the risk factors in order to identify areas for prevention.
Methods: All cases of poison exposure in Jewish and Arab children under the age of 15 years reported to the Israel Poison Information Center during 1990–95 (n= 5,583) were analyzed for the 6 weeks before and 6 weeks after Passover. Poison exposures in Jewish children <15 years old were studied in seven pediatric emergency rooms for the 2 weeks before and 6 weeks after Passover (n=123).
Results: The IPIC data showed a highly significant 38% increase in the average weekly poison exposure rate for the 2 weeks before Passover compared with the remaining 10 weeks. Data recorded by the pediatric emergency rooms showed a twofold increase in cleaning substance poisoning during the 2 weeks before Passover compared with the following 6 weeks. The rise in exposures to cleaning substances was observed among children from secular, religious and ultra-orthodox families. In these exposures, the substance was found in open containers in 70% of cases.
Conclusions: The extensive cleaning of homes among Jewish families in preparation for Passover poses the danger to young children of cleaning substance poisoning. Increasing public awareness, closer observation of children, and keeping these substances in closed containers should increase children’s safety during this annual cleaning.
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IPIC = Israel Poison Information Center
Raz Somech MD, Vera Zakuth MSc, Ayala Assia MD, Uri Jurgenson MD and Zvi Spirer MD
Background: Previous reports on the behavior of procalcitonin blood levels in diverse clinical conditions suggest that it is part of the activation of cellular immunity and is another acute-phase reactant.
Objective: To compare procalcitonin with C-reactive protein, a well-known acute-phase reactant, in a series of acutely febrile pediatric patients and to review recent literature on procalcitonin.
Methods: Procalcitonin and CRP levels were evaluated in 38 blood samples of pediatric patients who were admitted to the Dana Children’s Hospital for evaluation of unexplained fever or for sepsis work-up.
Results: The parallelism between procalcitonin and CRP was found to be highly significant (P<0.01).
Conclusion: The rise of procalcitonin blood levels in febrile pediatric patients suggests that it is part of the acute-phase reaction, parallel with the CRP reaction.
Ronit Neudorf-Grauss MD, Yoram Bujanover MD, Gabriel Dinari MD, Efrat Broide MD,Yehezkiel Neveh MD, Ilan Zahavi MD and Shimon Reif MD
Objective: To describe the clinical and epidemiological features of hepatitis B virus infection in Israeli children, and to evaluate their response and compliance to therapy.
Methods: We retrospectively studied 51 patients (34 males, 17 females), aged 2–18 years, from several medical centers in Israel.
Results: Of the 51 patients, 38 with elevated transaminase, positive hepatitis B e antigen and/or HBV DNA, and histologic evidence of liver inflammation were treated. Interferon was administered by subcutaneous injections three times a week for 3-12 months (dosage range 3–6 MU/m2). Only 16% were native Israelis, while 78% of the children were of USSR origin. A family history of HBV infection was recorded in 25 of the 51 patients (9 mothers, 16 fathers or siblings). Five children had a history of blood transfusion. The histological findings were normal in 3 patients, 24 had chronic persistent hepatitis, 14 had chronic active hepatitis and 2 had chronic lobular hepatitis. Five children also had anti-hepatitis D virus antibodies. Twelve of the 38 treated patients (31.5%) responded to IFN completely, with normalization of the transaminase levels and disappearance of HBeAg and HBV DNA. In no patient was there a loss of hepatitis B surface antigen. The main side effects of IFN were fever in 20 children, weakness in 10, headaches in 9, and anorexia in 6; nausea, abdominal pain, and leukopenia were present in 3 cases each. The response rate was not affected by age, country of origin, alanine/aspartate aminotransferase levels, or histological findings. However, a history of blood transfusion was a predictor of good response, 60% vs 27% (P<0.05).
Conclusions: We found IFN to be a safe and adequate mode of treatment in children with chronic HBV infection, regardless of their liver histology and transaminase levels. Therefore, in view of the transient side effects associated with this drug, we recommend considering its use in all children with chronic hepatitis B.
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HBV = hepatitis B virus
IFN = interferon
HBeAg = hepatitis B e antigen
Matti Erlichman MD, Ruth Litt MD, Zachi Grossman MD, Ernesto Kahan MD MPH and IPROS Network
Background: Streptococcal pharyngotonsillitis remains a common illness in children and can lead to serious complications if left untreated.
Objective: To evaluate the diagnostic and management approach of a sample of primary care physicians in the largest sick fund in Israel to streptococcal pharyngotonsillitis in children.
Methods: A questionnaire was mailed to all physicians who treat children and are employed by the General Health Services (Kupat Holim Klalit) in the Jerusalem District. The questionnaire included data on demographics, practice type and size, and availability of throat culture and rapid strep test; as well as a description of three hypothetical cases followed by questions relating to their diagnosis and treatment.
Results: Of the 188 eligible physicians, 118 (62.5%) responded, including 65 of 89 pediatricians (73%) and 53 of 99 family and general practitioners (53.5%). Fifty-six physicians (47.4%) had more than 18 years experience, and 82 (70%) completed specialization in Israel. Mean practice size was 950 patients. Fifty-three physicians (43%) worked in Kupat Holim community clinics, 25 (21%) worked independently in private clinics, and 40 (34%) did both. A total of 91 (77%) had access to laboratory facilities for daily throat culture. The time it took for the results to arrive was 48 to 72 hours. For the three clinical scenarios, 90% of the physicians accurately evaluated case A, a 1-year-old with viral pharyngotonsillitis, and 100 (85%) correctly diagnosed case C, a 7-year-old with streptococcal infection. As expected, opinions were divided on case B, a 3-year-old child with uncertain diagnosis. Accordingly, 75 (65.3%) physicians did not recommend treatment for case A, compared to 109 (92.5%) for case C. For case B, 22 (19%) said they would always treat, 43 (36%) would sometimes treat, and 35 (30%) would await the result of the throat culture. For 104 (88%) physicians the antibiotic of choice for case C was penicillin, while only 9 (7.5%) chose amoxicillin. However, the recommended dosage regimens varied from 250 to 500 mg per dose, and from two to four doses daily. For case C, 110 physicians (93%) chose a 10 day duration of treatment.
Conclusions: The primary care physicians in the sample (pediatricians, general practitioners and family physicians) accurately diagnosed viral and streptococcal pharyngotonsillitis. However, there was a lack of uniformity regarding its management in general, and the dosage regimen for penicillin in particular.