Israel Medical Association Journal

Background: Long-term follow-up in apical hypertrophic cardiomyopathy is rare.

Objective: To study the natural history of the disease.

Methods: We followed 11 patients, 5 women and 6 men, for 5-20 years.

Results: At presentation all 11 patients had typical features of apical hypertrophic cardiomyopathy, with dyspnea in 3 and chest pains in 8, of whom 5 were typical of angina and 3 had myocardial infarction. R-wave voltage and T-wave negativity progressively decreased in magnitude at serial electrocardiograms in four patients. Perfusion defects were detected on thallium myocardial scintigraphy in three, increased apical uptake in two, and normal in one patient. Apical aneurysm with normal coronary arteries developed in a patient who had sustained ventricular tachycardia. All of the 10 catheterized patients had normal coronaries except for one with significant left anterior descending artery stenosis and another with a minor lesion. Symptomatic sustained ventricular tachycardia was found in two patients, one of whom required the implantation of an internal cardioverter-defibrillator.

Conclusions: Apical hypertrophic cardiomyopathy may develop morphologic and electrocardiographic changes with life-threatening arrhythmias necessitating close follow-up and treatment.

Background: Leptin is a 16 kDa hormone synthesized by adipocytes and involved in body weight regulation.

Objectives: To determine serum leptin concentrations in heart, liver and kidney transplant recipients.

Methods: We investigated 57 patients: 18 male heart transplant recipients (age 25-69 years) at 1-66 months after transplantation, 6 female and 8 male liver transplant recipients (age 33-70) at 11-73 months after transplantation, and 10 female and 15 male kidney transplant recipients (age 20-61) at 3-138 months after transplantation. All recipients were receiving immunosuppressive therapy, including prednisone 0-20 mg/day, azathioprine 75-125 mg/day, cyclosporin 100-250 mg/day or tacrolimus 2-10 mg/day. The results were compared to those of 10 female and 10 male healthy controls. Morning serum concentrations of leptin were measured with a commercial radioimmunoassay (Linco Research Inc., USA), and serum insulin and cortisol levels were measured by radioimmunoassay.

Results: Patients (both men and women) after heart, liver and kidney transplantation exhibited significantly higher serum concentrations of leptin and leptin/body mass index ratios than controls. Serum leptin concentrations were significantly higher in women than in men and correlated very significantly with BMI[1] in all cases. The multivariate stepwise analyses showed that among parameters including BMI, gender, age, time after transplantation, prednisone dose, hematocrit, serum concentrations of glucose, albumin, creatinine, cortisol and insulin, only BMI, gender, cortisol and insulin were significant independent determinants of serum leptin levels in these patients.

Conclusions: This is the first report showing that, in addition to body mass index and gender, basal cortisol and insulin levels affect the hyperleptinemia in transplant patients. The clinical relevance of hyperleptinemia in these patients will require further investigation.

Background: At the start of the Bone and Joint Decade 2000-2010, a paleopathologic study of the physically disabled may yield information and insight on the prevalence of crippling disorders and attitudes towards the afflicted in the past compared to today.

Objective: To analyze “The procession of the Cripples,” a representative drawing of 31 disabled individuals by Hieronymus Bosch in 1500.

Method: Three specialists - a rhumatologist, an orthopedic surgeon and neurologist - analyzed each case by problem-solving means and clinical reasoning in order to formulate a consensus on the most likely diagnosis.

Result: This iconographic study of cripples in the sixteenth century reveals that the most common crippling disorder was not a neural form of leprosy, but rather that other disorders were also prevalent, such as congenital malformation, dry gangrene due to ergotism, post-traumatic amputations, infectious diseases (Pott’s, syphilis), and even simulators. The drawings show characteristic coping patterns and different kinds of crutches and aids.

Conclusion: A correct clinical diagnosis can be reached through the collaboration of a rheumatologist, an orthopedist and neurologist. The Bone and Joint Decade Project, calling for attention and education with respect to musculoskeletal disorders, should reduce the impact and burden of crippling disease worldwide through early clinical diagnosis and appropriate treatment.
 

Background: Transfer to an emergency room and hospitalization of nursing home residents is a growing problem that is poorly defined and reported.

Objectives: To assess the clinical effectiveness of a pilot project involving hospitalization of nursing home residents directly to an acute-care geriatric department.

Methods: We retrospectively compared the hospitalization in an acute-care geriatric unit of 126 nursing home residents admitted directly to the unit and 80 residents admitted through the emergency room. The variables measured included length of stay, discharge disposition, mortality, cause of hospitalization, chronic medical condition, cognitive state, functional status at admission, and change of functional status during the hospital stay. Follow-up data were obtained from medical records during the 2 year study.

Results: No significant differences between the groups were found for length of stay, mortality, discharge disposition and most characteristics of the hospital stay. The only significant difference was in patients’ mean age, as emergency room patients were significantly older (86 vs. 82.9 years). The most common condition among nursing home patients admitted via the emergency room was febrile disease (36.9%) ,while functional decline was the most common in those coming directly from the nursing home (32.5%). The prevalence of functional dependence and dementia were similar in both groups. Functional status did not change throughout the hospital stay in most patients.

Conclusions: Treatment of selected nursing home residents admitted directly from the nursing home to an acute- care geriatric unit is feasible, medically effective, results in the safe discharge of almost all such patients and provides an alternative to transfer to an emergency room. This study suggests that quality gains and cost-effective measures may be achieved by such a project, although a randomized controlled trial is necessary to support this hypothesis.
 

Sjogren’s syndrome is a chronic inflammatory process invol­ving primarily the exocrine glands. Its association with lymphoma is well documented. A low grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the commonest lymphoid neoplasia in Sjogren’s syndrome. We review the literature and comment on the molecular, clinical, histopathologic and therapeutic aspects of these tumors in Sjogrens syndrome.

Background: A previous study on Hodgkin's lymphoma in southern Israel found that Bedouin patients had an increased rate of Epstein-Barr virus expression in their tumor cells.

Objectives: To determine the influence of the patients' communities on the pattern of disease in HL.

Methods: We compared the clinical features, demographic data, stage at diagnosis, treatment modality and outcome, as well as laboratory findings, in four community-based subgroups. These groups comprised kibbutz residents (n=11), Bedouin (n=19), new immigrants from the former USSR (n=22), and town-dwellers (n=82).

Results: The Bedouin patients differed significantly from the new immigrants and town-dwellers, particularly regarding the rate of EBV sequences in the tumor tissues, and a poorer response to treatment. The kibbutz patients did not differ significantly from the other populations regarding most of the parameters studied, but showed an intermediate expression of EBV antigens compared to Bedouin patients and the rest of the cohort.

Conclusions: This study indicates that HL may behave differently in different population groups in a given geographic area. Notably, the Bedouin patients showed markedly different clinical and biological patterns of this malignancy. 

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HL= Hodgkin's lymphoma

EBV= Epstein-Barr virus