Israel Medical Association Journal

Hemophagocytic lymphohistiocytosis is thought to occur as a primary (familial) form or secondary to infection or malignancy. Recently, several defects in genes important for immune functions were identified in patients with HLH[1]. These include mutations in perforin, the gamma common chain, the receptor for interleukin-2, Slap and purine nucleoside phosphorylase. Since abnormal function of these genes is associated with a wide clinical spectrum, HLH is probably another manifestation of immune deficiency and a thorough immune evaluation should be done in all such patients.

Background: Domestic violence is a prevalent problem with serious consequences, including a 30% risk of death. The lifetime prevalence ranges from 21 to 34%, with 8–14% of them reporting abuse in the previous year. The incidence seen in primary care practice is about 8%. Despite this high rate, domestic violence is under-diagnosed in primary care.

Objectives: To estimate the prevalence of domestic violence among women visiting a primary care center, to characterize them and to evaluate a screening tool.

Methods: A brief anonymous questionnaire (in Hebrew and Russian) for self-completion was used as a screening tool. During October 1998 we distributed the questionnaires in a primary care clinic in Beer Sheva to all women aged 18–60 years whose health permitted their participation. A woman was considered at high risk for domestic violence when she gave a positive answer to at least one of the three questions related to violence. The risk factors for domestic violence were calculated by odds ratio with 95% confidence intervals.

Results: The response rate was 95.7%. We found 41 women (30.8%) at high risk for violence. Women preferred talking about this issue with their family physician. Women at highest risk were older than 40 years, had emigrated from the former Soviet Union during the last 10 years, were living alone, and were unemployed. None of the women visited the Domestic Violence Center during the study period and 2 months thereafter. Only three women tore off the address and phone number of the center that were attached to the questionnaire.

Conclusions: The anonymous questionnaire was well accepted and had a high compliance rate. Its disadvantages are that respondents must be literate and that it permits the woman to continue with her “secret-keeping” behavior. A high prevalence of domestic violence among women visiting a primary care clinic should convince family physicians to be more active in diagnosing the problem accurately among their patients, providing treatment and preventing further deterioration and possible danger. Further effort should be directed at improving the clinic staff's ability to detect domestic violence among patients, and in developing management programs in the health system to help combat domestic violence.

Background: Bloodletting is practiced in Ethiopia. Physicians in Israel engaging in transcultural encounters with Ethiopian immigrants are generally unaware of these ethnomedical beliefs and practices.

Objective: To assess the past and present use of bloodletting among Ethiopian immigrants in Israel.

Methods: We interviewed a sample of 50 adult patients of Ethiopian origin about present and past use of bloodletting. A second consecutive sample of 10 adult patients of Ethiopian origin who often asked their doctors to perform blood tests were identified and interviewed. Data analysis was performed by "immersion-crystallization" analysis.

Results: More than half of the interviewed patients reported the use of bloodletting. Scars were commonly present on their upper extremities. A qualitative analysis identified the different reasons for the use of bloodletting, the technique used and its appreciated efficacy. We also found an unexpected cultural synergy between traditional bloodletting and western medical blood sampling.

Conclusions: Some Ethiopian immigrants continue to perform traditional bloodletting in their new country of residency, a practice that local physicians may not be aware of. Bloodletting-type scars on the upper extremities may be common in these patients. Patients may ask for blood sampling as a culturally accepted way to perform bloodletting (synergy).
 

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

Background: Protruding aortic atheromas are a potential source of stroke and systemic emboli. The single modality currently available for their detection has been transesophageal echocardiography. However, TEE does not allow full visualization of the upper part of the ascending aorta and proximal aortic arch.

Objectives: To investigate whether double helical computerized tomography- both with and without contrast injection - may represent a useful technique for noninvasive detection of PAA in stroke patients.

Methods: Forty consecutive patients ≥50 years of age who sustained a recent ischemic stroke and/or systemic emboli (within 15 days after the onset of the event) were enrolled in the study and underwent TEE and DHCT without contrast injection using thin slice acquisition (3.2 mm thickness and 1.5 mm reconstruction increment). In addition, the last eight consecutive patients, after obtaining an unenhanced scan, underwent a contrast-enhanced DHCT following peripheral intravenous injection of a small amount of contrast material (15 ml of diatrizoate).

Results: PAAs were demonstrated by TEE in 18 patients (45%); in 16 of them (89%) the atheromas were recognized by DHCT. Of the 22 patients without PAA on TEE, DHCT confirmed their absence in 18 (82%). DHCT yielded a sensitivity of 89%, a specificity of 82%, and an overall accuracy of 85%. The total number of protruding plaques detected by TEE was 43, of which 41 (95%) were correctly identified by DHCT. The mean thickness of the plaques was 5.6±2.4 mm on TEE, and 5.4±2.3 on DHCT (P=NS), with a good correlation between the modalities (γ=0.84). Contrast-enhanced DHCT scans demonstrated absolute equivalence to TEE in aortic areas defined as "clearly visualized by TEE." DHCT detected PAA between the distal ascending aorta and the proximal arch in seven patients; these atheromas were not included in the comparative analysis. In these "occult" areas, DHCT may be superior to TEE.

Conclusions: DHCT without contrast injection using thin slice acquisition may become a useful modality for rapid noninvasive detection of PAA. Contrast-enhanced DHCT scans significantly improve imaging quality and may be superior to TEE in the upper ascending aorta and the proximal arch (areas not well visualized by TEE).

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TEE= transesophgeal echocardiography

PAA= protruding aortic atheroma

DHCT= dual helical computerized tomography
 

Background: The etiology of bone marrow failure, a prominent feature of paroxysmal nocturnal hemoglobulinuria, is presently unknown.

Objectives: To evaluate the possible influence of cellular immune mechanisms in the bone marrow failure of PNH.

Methods: We studied marrow erythroid colony formation in a patient with paroxysmal nocturnal hemoglobinuria without hypoplastic/aplastic marrow complications.

Results: In vitro assays revealed a pronounced inhibition of primitive erythroid (BFU-E) progenitor cell growth by marrow T lymphocytes. Removal of T cells prior to culture resulted in a 4.5-fold enhancement of BFU-E numbers. Reevaluation of in vitro erythropoiesis during steroid administration indicated a persistent, albeit less prominent, T cell inhibitory effect.

Conclusion: Our findings provide the first direct evidence for a cellular immune inhibitory phenomenon accompanying PNH.

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PNH= paroxysmal nocturnal hemoglobinuria