Israel Medical Association Journal

Background: Persistent creatine kinase elevation is occasionally encountered in subjects without any clinical manifestation of a neuromuscular disorder or any condition known to be associated with increased serum CK[1] levels. It is still unresolved whether extensive investigations and specifically a muscle biopsy should be performed in clinically normal individuals with elevated CK levels.

Objective: To study the muscle pathology of patients with asymptomatic or minimally symptomatic hyperCKemia.

Methods: The clinical and laboratory data of patients with persistent hyperCKemia and normal neurologic examination were reviewed and their muscle biopsies evaluated.

Results: The study group included 40 patients aged 7–67 years; the male to female ratio was 3:1. Nineteen patients were completely asymptomatic, 20 had mild non-specific myalgia, and 1 had muscle cramps. Electromyography was performed in 27 patients and showed myopathic changes in 7 (26%). Abnormal muscle biopsy findings (e.g., increased variation in fiber size, increased number of central nuclei and occasional degenerating fibers) were detected in 22 of the 40 patients (55%). No fat or glycogen accumulation was detected. Immunohistochemistry demonstrated abnormal dystrophin staining in 3 patients (8%), resembling the pathologic changes of Becker muscular dystrophy. No abnormal findings were detected on immunohistochemical staining for merosin, dysferlin, caveolin 3, or alpha and gamma sarcoglycans. The EMG[2] findings did not correlate with the pathologic findings.

Conclusions: Abnormal muscle biopsies were found in 55% of patients with asymptomatic or minimally symptomatic hyperCKemia. Specific diagnosis of muscular dystrophy, however, was possible in only 8% of the patients.

Background: The osteoporosis-pseudoglioma syndrome is a rare autosomal recessive disorder characterized by severe juvenile-onset osteoporosis and congenital or early-onset blindness. Other manifestations include muscular hypotonia, ligamentous laxity, mild mental retardation and seizures. The gene responsible was recently identified to be the low density lipoprotein receptor-related family member LRP5 on chromosome 11q11-12.

Objective: To measure bone density in two siblings with the OPPG[1] syndrome as well as in their family members (parents and siblings).

Methods: Bone mineral density was determined in the lumbar spine (antero-posterior), femoral neck, two-thirds distal forearm (>95% cortical bone) and ultradistal forearm (predominantly trabecular bone) by dual-energy X-ray absorptiometry.

Results: The studies revealed osteoporotic changes both in the patients and the carriers.

Conclusion: The findings demonstrate that OPPG carriers have reduced bone mass, which is a risk factor for development of early osteoporotic changes.

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Background: Trauma is viewed by many as a global problem. The phenomenon of similar outcomes within differing healthcare delivery systems can illuminate the strengths and weaknesses of various trauma systems as well as the effects of these characteristics on patient outcome.

Objectives: To compare and contrast demographic and injury characteristics as well as patient outcomes of two urban/suburban trauma centers, one in Israel and the other in the United States.

Methods: Study data were obtained from the trauma registries of two trauma centers. Demographic variables, injury characteristics and outcomes were compared statistically between registries.

Results: Significant differences between the registries were found in demographic variables (age), injury characteristics (Injury Severity Score and mechanism of injury), and outcome (mortality and length of stay). Age and Injury Severity Score were found to be significant predictors of outcome in both registries. The Glasgow Coma Score was found to contribute to patient outcomes more than the ISS[1]. Differences were found in the relative impact of injury and demographic factors on outcomes between the registries. After including the influence of these factors on patient outcomes, significant differences still remained between the outcomes of the trauma centers.

Conclusions: Despite possible explanations for these differences, true comparisons between centers are problematic.

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Background: Fasting is required by the Jewish and Islamic religions, and may sometimes be necessary for non­religious reasons as well. Very little empiric data are available on the effect of 24 hours of food and water deprivation.

Objectives:  To compare the effects of the dietary composition of different pre-fast meals on subjective discom­fort and various other parameters of a 24 hour food and water fast.

Methods: Thirteen volunteers of both genders participated in a non-randomized crossover study. Each consumed three different equicaloric pre-fast meals in which the main source of calories was protein (49% of calories), carbohydrate (86%), or fat (69%). Weight, heart rate, blood pressure, blood and urine were tested before and after 24 hours of fasting, and the subjective evaluations of the discomfort during the three fasts were compared.

Results: After the protein-rich meal greater discomfort and more side effects were reported. Weight and blood pressure decreased at the end of the fasts that followed each of the three meals heart rate increased after the high fat and carbohydrate meals but not after the protein meal. The main laboratory findings were a 40% increase in blood urea nitrogen and higher urine osmolarity after the protein-rich meal than after the other meals.

Conclusion: A protein-poor pre-fast meal is likely to be followed by easier fasting.