Yehuda Nofech-Mozes MD, Yael Yuhas PhD, Elisabeth Kaminsky MSc, Abraham Weizman MD and Shai Ashkenazi MD MSc
Background: The pathogenesis of neurological symptoms, the most common extraintestinal complication ofchildhood shigellosis, is unclear. To elucidate the mechanisms involved, we developed an animal model and demonstrated that TNF alpha and IL-1 beta play a role.
Objectives: To determine whether TNF alpha and IL-1 beta genes are expressed in the brain following peripheral administration of Shigella dysenteriae 60R.
Methods: Expression of mRNA for TNF alpha and IL-1 beta was examined in the brain structures (hypothalamus and hippocampus) and peripheral organs by reverse transcriptase polymerase chain reaction, at different time points after intraperitoneal injection of S. dysenteriae sonicate.
Results: In our animal model of Shigella related seizures, TNF alpha and IL-1 beta mRNA were induced in the brain, spleen and liver already 1 hour after injection of S. dysenteriae sonicate. The expression of TNF alpha and IL-1 beta mRNA in spleen, hippocampus and hypothalamus decreased after 6 h and increased again at 18 h post-injection.
Conclusions: Local production of TNF alpha and IL-1 beta in the brain may be involved in the enhanced seizure response of mice after administration of S. dysenteriae. It is possible that intracerebral production of TNF alpha and IL-1 beta plays a role in neurological disturbances of human shigellosis.
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
Einat Birk MD, Alon Stamler MD, Jacob Katz MD, Michael Berant, Ovadia Dagan MD, Abraham Matitiau, Eldad Erez MD, Leonard C. Blieden and Bernardo A. Vidne
Background: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital malformation that presents a diagnostic challenge to the pediatrician and pediatric cardiologist. Although surgical repair is always indicated, the optimal technique has yet to be determined.
Objectives: To review our experience with the diagnosis of children with ALCAPA and to assess short to midterm surgical results.
Methods: Between 1992 and 1998, 13 infants and children (2 months to 15 years) were treated for ALCAPA at our medical center. Eight were diagnosed during the first year of life; all were symptomatic and had severe dysfunction of the left ventricle. The five patients diagnosed at an older age had normal myocardial function. Diagnosis was established by echocardiography alone in seven patients; six required catheterization (one infant and all older patients). Surgery was performed in 12 patients to establish dual coronary artery system: 7 underwent the Takeuchi procedure and 5 had re-implantation of the anomalous left coronary artery.
Results: One infant died shortly after diagnosis before surgical repair was attempted, and one died postoperatively. Four patients required additional surgery: three for late complications of the Takeuchi procedure and one valve replacement for mitral insufficiency. Recent evaluation revealed good global left ventricle function in all patients except for one, who is still within the recovery phase and shows gradual improvement. However, most patients who presented with severe myocardial dysfunction upon diagnosis still display abnormal features such as echo-dense papillary muscles or evidence of small akinetic segments. In this group, early repair was associated with faster myocardial recovery.
Conclusions: The diagnosis of ALCAPA remains a clinical challenge to the pediatrician and cardiologist. Diagnosis can be established echocardiographically, and early diagnosis and treatment may lead to faster myocardial recovery. The preferred surgical method appears to be re-implantation of the ALCA. The chance for good recovery of global ventricular function is high even in the sickest patients, nonetheless abnormal myocardial features can be identified even years after surgery.
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ALCAPA= anomolous origin of the left coronary artery from pulmonary artery.
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
Amir Kimia MD, Ilan Zahavi MD, Rivka Shapiro MD, Yoram Rosenbach MD, Akiva Hirsh MD1, Tamara Druzd MD, Jacob Yahav MD and Gabriel Dinari MD
Background: Recurrent abdominal pain is a common pediatric diagnostic problem. Endoscopy is sometimes performed as part of the evaluation. Although gastritis and/or Helicobacter pylori infection is often present, it is not known if they contribute to the symptomatology.
Objectives: To evaluate the role of either gastritis or H. pylori infection in the symptomatology of children with RAP.
Patients and Methods: We retrospectively studied two groups of patients, 70 children in each, who had undergone endoscopy. One group was evaluated endoscopically for RAP and the other was a heterogeneous group that underwent endoscopy for indications other than RAP. Biopsies were taken during endoscopy and Giemsa staining was performed for the presence of H. pylori. Triple therapy was given as indicated, and the children were followed for an average of 6 months.
Results: Microscopic gastritis was diagnosed in 39 patients (55.7%) of the RAP group and in 31 of the heterogeneous group (44.2%) (NS), and H. pylori was found in 32 patients of the RAP group and in 16 of the heterogeneous group (45.7% vs. 22.8%, P<0.01). All children with H. pylori, except one in the heterogeneous group, had accompanying gastritis. On the other hand, gastritis without H. pylori infection was seen in 7 children in the RAP group and in 15 of the other. Endoscopy revealed macroscopic abnormalities in 52 of the 70 children with microscopic gastritis. There was a clinical improvement after triple therapy in 28 of 33 children with H. pylori-associated gastritis (84.85%), in 4 of 8 children with gastritis unassociated with H. pylori (50%), and in 8 of 15 without gastritis or H. pylori (53.3%) (P<0.01 between the H. pylori-associated gastritis and each of the other groups).
Conclusions: H. pylori infection and gastritis may be associated with RAP in a selected subgroup of children. We recommend a complete work-up, including endoscopy and invasive or non-invasive diagnostic modalities for H. pylori, and treatment of the infection.
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RAP = recurrent abdominal pain
Idan Burstein, MD, Ran Steinberg, MD and Michael Zer, MD
Background: Small bowel obstruction with perforation is an unusual and rare complication of bezoars.
Objective: To describe our use of emergency laparotomy to treat intestinal obstruction caused by bizarre bezoars.
Conclusions: An aggressive surgical approach to intestinal obstruction in the pediatric disabled or mentally retarded population is recommended.
Michael Schwarz MD, Gadi Horev MD, Enrique Freud MD, Nizza Ziv MD, Amir Blumenfeld MD, Ran Steinberg MD and Liora Kornreich MD
Background: Multiple organ injury in children is an increasingly frequent phenomenon in the modern emergency room. Adrenal hemorrhage associated with this type of trauma has received little attention in the past.
Objectives: Using computed tomography, we sought to determine the rate and nature of adrenal gland injury in children following blunt abdominal trauma due to motor vehicular accident.
Methods: A total of 121 children with blunt abdominal trauma were examined and total body CT was performed in cases of multi-organ trauma or severe neurological injury.
Results: Of all the children who presented with blunt abdominal trauma over a 51 month period, 6 (4.95%) had adrenal hemorrhage. In all cases only the right adrenal gland was affected. Coincidental injury to the chest and other abdominal organs was noted in 66.7% and 50% of patients, respectively.
Conclusions: Traumatic adrenal injury in the pediatric population may be more common than previously suspected. Widespread application of the more sophisticated imaging modalities available today will improve the detection of damage to the smaller organs in major collision injuries and will help in directing attention to the mechanism of trauma.
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.
Kalman Katz MD, Liora Kornreich MD, Rami David MD, Gad Horev MD and Michael Soudry MD