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עמוד בית
Fri, 05.12.25

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June 2005
M. Arad, H. Lahat and D. Freimark
 Familial cardiomyopathies represent a substantial portion of idiopathic dilated cardiomyopathy in clinical practice. Diversity of clinical presentations and variability in penetrance lead to under-recognition of this disease entity as an inherited disorder. The mechanisms by which mutations in different genes perturb cardiac function and lead to pathologic remodeling help understand the molecular pathways in disease pathogenesis and define the potential targets for therapeutic interventions. Appreciating when DCM[1] is inherited might spare unnecessary diagnostic efforts and, instead, help give appropriate attention to the timely detection of subclinically affected family members. Establishing preventive therapy in asymptomatic family members showing early signs of cardiac dysfunction might prevent death and slow down progression to end-stage heart failure.


 





[1] DCM = dilated cardiomyopathy


October 2004
M. Korem, Z. Ackerman, Y. Sciaki-Tamir, G. Gino, S. Salameh-Giryes, S. Perlberg and S.N. Heyman
September 2004
August 2004
N. Lubezky, R. Nakache, M. Carmiel, R. Oren, P. Sorkin, J. Klausner and M. Ben-Haim

Background: The prognosis of patients with fulminant hepatic failure without timely liver transplantation is dismal. Given the limited availability of cadaveric organs for urgent transplantation in Israel, adult-to-adult living-donor segmental liver transplantation may be the only alternative.

Objectives: To report our initial experience with urgent lifesaving LDLT[1] in this unique scenario.

Methods: Three adult patients with FHF[2] (two of unknown etiology, one with paracetamol intoxication) were transferred from other institutions and admitted to our intensive care unit. Initial treatment and monitoring included intracranial pressure monitoring and hepatic dialysis using the Molecular Adsorbent Recirculating System. Expeditious potential donor selection included medical, psychosocial and surgical evaluation. Liver volume and vascular anatomic compatibility were assessed with computed tomography angiography.

Results: Between July and October 2003 we performed three procedures of urgent adult-to-adult LDLT. The donors (two uncles, one sister) underwent hepatic resection (two right lobes, one left lateral segment) and recovered well. The recipients underwent total hepatectomy with caval preservation, followed by lobar grafting. All recipients recovered and are alive with good liver function and without any neurologic complications.

Conclusions: Urgent adult-to-adult living-donor segmental liver transplantation can be performed safely and timely as a lifesaving procedure in the setting of comatose patients with FHF.






[1] LDLT = living-donor liver transplantation

[2] FHF = fulminant hepatic failure


June 2004
J. Kundel, R. Pfeffer, M. Lauffer, J. Ramon, R. Catane and Z. Symone

Background: The role of prostatic fossa radiation as salvage therapy in the setting of a rising prostate-specific antigen following radical prostatectomy is not well defined.

Objectives: To study the efficacy and safety of pelvic and prostatic fossa radiation therapy following radical prostatectomy for adenocarcinoma.

Methods: A retrospective review of 1,050 patient charts treated at the Sheba Medical Center for prostate cancer between 1990 and 2002 identified 48 patients who received post-prostatectomy pelvic and prostatic fossa radiotherapy for biochemical failure. Two patients were classified as T-1, T2A-9, T2B-19, T3A-7 and T3B-11. Gleason score was 2–4 in 9 patients, 5–6 in 22 patients, 7 in 10 patients and 8–10 in 7 patients. Positive surgical margins were noted in 28 patients (58%) of whom 18 had single and 10 had multiple positive margins. Radiation was delivered with 6 mV photons using a four-field box to the pelvis followed by two lateral arcs to the prostatic fossa.

Results: At a median follow-up of 34.3 months (25th, 75th) (14.7, 51,3) since radiation therapy, 32 patients (66%) are free of disease or biochemical failure. Exploratory analysis revealed that a pre-radiation PSA[1] less than 2 ng/ml was associated with a failure rate of 24% compared with 66% in patients with a pre-radiation PSA greater than 2 ng/ml (chi-square P < 0.006).

Conclusions: For patients with biochemical failure following radical prostatectomy early salvage radiation therapy is an effective and safe treatment option.






[1] PSA = prostate-specific antigen


January 2004
A. Zeidman, Z. Fradin, A. Blecher, H.S. Oster, Y. Avrahami and M. Mittelman

Background: Anemia is a known risk factor for ischemic heart disease. Based on knowledge of the physiologic role of oxygen delivery to the myocardium, anemia may be a cause of more severe cardiovascular diseases or a marker of other processes occurring in the body that induce more severe disease.

Objectives: To investigate the relationship between anemia and the clinical picture of IHD[1], including manifestations, severity and complications.

Methods: The population studied comprised 417 similarly aged patients with IHD and anemia. The patients were categorized into subgroups of IHD according to disease severity: namely, angina pectoris, acute ischemia, acute myocardial infarction, congestive heart failure or cardiac arrhythmias. Two populations served as control groups: patients with anemia but no IHD (C-I) and patients with IHD without anemia (C-II). A standard anemia workup was conducted in all patients with IHD and anemia and a correlation was made between the hematologic parameters and the manifestations and complications of IHD.

Results: The common presenting symptom was chest pain in the study group and in C-II (94% and 86% respectively) and weakness (90%) in C-I. Patients with IHD and anemia tended to suffer from a more advanced degree of IHD (80%) compared to patients with IHD alone who had milder disease (46%). Hematologic values including hemoglobin, mean cell volume, serum iron and total iron binding capacity correlated inversely with disease severity among anemic patients with IHD. There were significant differences between the study group and C-II regarding CHF[2] (31% and 18% respectively) and arrhythmias (41% and 16% respectively). The mortality rate was higher in patients with IHD and anemia than in patients with IHD alone (13% and 4% respectively).

Conclusions: Anemia is a significant risk factor in IHD. It correlates with advanced IHD, CHF, rhythm disturbance and higher mortality rate. An aggressive therapeutic and preventive approach might improve the outcome of this disease.







[1] IHD = ischemic heart disease



[2] CHF = congestive heart failure


May 2003
D.S Silverberg, D. Wexler, M. Blum, D.Schwartz, G. Keren, D. Sheps, and A. Iaina

Background: Congestive heart failure is extremely common in octogenarians and is associated with severe fatigue, shortness of breath, recurrent hospitalizations, and death. These patients, many of whom are anemic, are often resistant to standard CHF[1] therapy including angiotensin-converting enzyme inhibitors, beta-blockers and diuretics.

Objectives: To examine whether correction of the anemia (hemoglobin <12 g/dl) in CHF patients lowers their resistance to therapy.

Methods: Forty octogenarians with anemia and severe resistant CHF were administered a combination of subcutaneous erythropoietin and intravenous iron sucrose.

Results: This combination therapy led to a marked improvement in cardiac function, shortness of breath and fatigue, a marked reduction in the rate of hospitalization and a stabilizing of renal function.

Conclusion: Anemia appears to be an important but ignored contributor to the progression of CHF, and its correction may improve cardiac and renal status as well as the quality of life in elderly patients.






[1] CHF = congestive heart failure


January 2003
D. Kohelet

Background: High frequency oscillatory ventilation has proved valuable in recruiting and sustaining lung volume; the combined treatment may augment nitric oxide delivery to target vessels. NO[1] therapy lowers pulmonary resistance and improves oxygenation.

Objective: To retrospectively review data on changes in oxygenation – indicated by arterial/alveolar PO2 ratio, oxygenation index, and outcome – in a cohort of 10 infants with hypoxemic respiratory failure in whom nitric oxide inhalation was instituted in a compassionate-use protocol after deteriorated oxygenation.

Methods: NO inhalation was administered at a range of 0.12–122 days of life using the SensorMedics system in 10 infants who developed hypoxemic respiratory failure associated with a variety of lung diseases while on HFOV[2].

Results: The infants' birthweight was 1,717 ± 1,167 g and their gestational age 31.1 ± 6.5 weeks. Mean exposure to NO inhalation was 14.2 days and ranged from 3–59 days. Oxygenation index decreased from 39.3 ± 13.2 to 12.7 ± 6.9 (P < 0.0002) after NO therapy. Despite an initial prompt response to NO inhalation, two patients died of progressive intractable respiratory failure and one term infant died of extrapulmonary complications (hypoxic ischemic encephalopathy grade III and multiorgan failure).

Conclusion: Our results indicate that the combined treatment of HFOV and NO inhalation is superior to HFOV alone for improving oxygenation in a selected cohort of infants ventilated for a variety of lung diseases.






[1] NO = nitric oxide



[2] HFOV = high frequency oscillatory ventilation


September 2002
Alla Shnaider, MD, Anna Basok, MD, Boris Rogachev, MD and Marcus Mostoslavsky, MD
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