Israel Medical Association Journal

Background: Recently, cognitive assessments of patients with Fabry disease highlighted neurocognitive impairment using test batteries that are time and labor intensive.

Objectives: To introduce a user-friendly self-administered tool for cognitive testing in patients with Fabry disease.

Methods: We used a computerized system requiring about 1 hour for patient follow-up. All patients with enzymatic and/or molecular diagnosis of Fabry disease seen in our clinic underwent assessment with the Fabry-specific Mainz Severity Score Index (MSSI) with subscores (neurological, renal, cardiac, and general) and a Mindstreams neurocognitive battery for mild impairment, evaluating memory, executive function, attention, information processing, visual spatial processing, verbal function, and motor skills. A Global Cognitive Score (GCS) was also computed.

Results: Ten patients (3 males, 7 females) were tested (mean age 41.5, range 25–56 years). Males were younger, had moderate nephropathy and no cerebrovascular accident (CVA); their Mindstreams GCS was 85.6–107 points. Three females had mild-moderate (8,10,15 points) neurological MSSI subscores (two CVA); all females had Mindstreams GCS of 59–107.7 points. Below-average performance was prevalent, particularly in information processing and motor skills consistent with mild impairment. Average GCS in females (90.3 points) was lower than in males (98.2 points). For individual patients, performance was poorest in information processing (n=4), attention (n=2), motor skills (n=2), verbal function (n=1), and visual spatial processing (n=1).

Conclusions: MindStreams may simplify cognitive assessment monitoring in Fabry disease.
 

Background: Atherosclerosis is a chronic inflammatory process resulting in coronary artery disease.

Objectives: To determine the relationship between inflammatory markers and the angiographic severity of CAD[1].

Methods: We measured inflammatory markers in sequential patients undergoing coronary angiography. This included C-reactive protein, fibrinogen, serum cytokines (interleukin-1 beta, IL-1[2] receptor antagonist, IL-6, IL-8, IL-10) and tumor necrosis factor-alpha), all measured by high sensitivity enzyme-linked immunoabsorbent assay.

Results: There was a significant correlation between TNFα[3] and the severity of CAD as assessed by the number of obstructed coronary vessels and the Gensini severity score, which is based on the proximity and severity of the lesions. Patients had more coronary vessel disease (> 70% stenosis) with increasing tertiles of serum TNFα; the mean number of vessels affected was 1.15, 1.33, and 2.00 respectively (P < 0.001). IL-6 correlated with the Gensini severity score and coronary vessel disease (> 70% stenosis). A weaker correlation was present with IL-1 receptor antagonist. A significant correlation was not found with the other inflammatory markers. After adjustment for major risk factors, multivariate analyses showed that significant independent predictors of CAD vessel disease were TNFα (P < 0.05) and combined levels of TNFα and IL-6 (P < 0.05). IL-6 levels were independently predictive of Gensini coronary score (P < 0.05).

Conclusion: TNFa and IL-6 are significant predictors of the severity of coronary artery disease. This association is likely an indicator of the chronic inflammatory burden and an important marker of increased atherosclerosis risk.

Background: The Beer Sheva Psoriasis Severity Score is a novel instrument for the assessment of psoriasis severity, designed for use in routine clinical conditions.

Objective: To identify the main factors of the BPSS[1].

Methods: The sample used to study the BPSS comprised 70 patients with psoriasis vulgaris treated by climatotherapy at the Dead Sea. Psoriasis severity was assessed using BPSS and PASI (Psoriasis Area and Severity Index). Factor analysis was used to identify the main factors of BPSS. Internal consistency analysis was performed. Correlation matrices were generated to compare BPSS factors.

Results: Factor analysis demonstrated that BPSS included six factors that explained 74.0% of the variance as follows: patient assessment 26.0%; physician assessment 13.2%; palms and soles involvement 11.9%; genitals, nails, and pruritus 9.0%; face involvement 7.3%; and scalp involvement 6.6%. Total scale Cronbach’s alpha was 0.76; alpha for the factors ranged between 0.39 and 0.81.

Conclusion: The major factors of BPSS were identified. BPSS may be used as a comprehensive tool for measuring psoriasis severity.

[1] BPSS = Beer Sheva Psoriasis Severity Score

Background: Trauma management includes the care provided both in hospital and by emergency medical systems in the community. In many cases it is the parents who decide where to take an injured child for care, depending on the circumstances and severity of the injury, the personal characteristics of the injured or the carer and the availability and accessibility of services.

Objectives: To examine the use of pre-hospitalization services and reasons for their use by children and adolescents according to the injury and personal characteristics.

Methods: The study group comprised 924 Israeli citizens aged 0–17 years hospitalized for injuries in six hospitals across Israel. Carers were interviewed in the hospital regarding the circumstances of the injury event, the use of pre-hospitalization services, and sociodemographic characteristics. Data on the cause and nature of the injury were obtained from the hospital records.

Results: The proportion of severe injuries (Injury Severity Score 16+) was higher in Arab children than Jewish children (15% and 9% respectively). Sixty-three percent of the Arab children and 39% of the Jewish children used community services prior to hospitalization. The odds ratio of proceeding directly to the hospital was 0.44, 95% confidence interval 0.29–0.69, for the Arab compared to the Jewish children, controlling for severity, cause and nature of the injury, sociodemographic characteristics, and the reported availability of ambulance services.

Conclusions: More Arab than Jewish carers tended to seek care in the community for an injured child, but the effect of personal characteristics on seeking care was similar in both population groups. Issues of availability and accessibility of services may explain the differences.

Background: The arrival of 610,000 new immigrants to Israel from the former Soviet republics accounted for 58% of the population growth in the early 1990s.

Objective: To compare the coronary angiographic findings and risk factors between the new immigrants and local Jewish and Arab patients in this era of cost containment.

Methods and Results: A total of 550 consecutive patients - 314 Jews, 95 new immigrants and 141 Arabs - were catheterized and analyzed during a 5 month period in 1995. Of this group 403 were males (73%). The mean age was 63.6±10.2 years among new immigrants, 62.4±9.4 among Jews, and 55.1±10.9 among Arabs (P<0.05). Immigrants, including those under age 60, had the highest prevalence of multivessel disease (88.7%). Arabs had a high prevalence of single vessel disease (34.6%) and a low prevalence of multivessel (65.4%) and left main coronary disease (5.6%). Age, gender, risk factors and ethnic origin in descending order were determinants of the extent of coronary angiographic disease as revealed by multiple regression analysis.

Conclusion: New immigrants had the most extensive angiographic coronary involvement, while Arab patients were younger and had less severe coronary artery disease. More intensive risk factor modification may have a major impact on disease progression particularly in the new immigrant subgroup. 

Objectives: To suggest a simple outpatient technique for evaluating the response of arterial oxygen saturation to exercise for use as a marker of disease severity.

Patients and methods: Ninety-six patients with various degrees of COPD¹ were divided into three groups: mild (forced expiratory volume in 1 sec >65%), moderate (FEV1² between 50 and 65%), and severe (FEV1 <50%). Using continuous oximeter recording we measured oxygen saturation during 15 steps of climbing, and quantified  oxygen desaturation by measuring the “desaturation area”, defined as the area under the curve of oxygen saturation from the beginning of exercise through the lowest desaturarion point and until after recovery to the baseline level of oxygen percent saturation. Desaturation was correlated to spirometry, lung gas volumes, blood gas analysis, and 6 min walking distance.

Results A good correlation was found between severity of COPD and baseline SaO2³, lowest SaO2, recovery time, and desaturation area.  A negative correlation was found between desaturation area and FEV1 (r=-0.65), FEV1/forced vital capacity (r=-0.58), residual volume to total lung capacity (r=0.52), and diffusing lung capacity for carbon monoxide (r=-0.52). In stepwise multiple regression analysis only FEV1 correlated significantly to desaturation area.  A good correlation was noted between 6 min walking distance and desaturation area with the 15 steps technique (r=0.56).

Conclusions: In patients with severe COPD, arterial hypoxemia during exercise can be assessed by simple 15 steps oximetry. This method can serve both as a marker for disease severity and to determine the need for oxygen supplementation.

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COPD = chronic obstructive pulmonary disease

FEV1 = forced expiratory volume in 1 sec

SaO2 = arterial oxygen saturation