Israel Medical Association Journal

Background: Arterial involvement in Behçet's syndrome is rare. Aneurysms are common among the arterial lesions, affecting various arteries but mostly the abdominal aorta. Surgical interposition graft insertion is the treatment of choice for large aneurysms. However, vasculitis in these patients is the reason for the notorious surgical complications that result in up to 50% false aneurysms in anastomotic sites. Recently, endovascular repair for abdominal aortic aneurysms has been established.

Objectives: To learn more about vascular Behçet and, specifically, to compare the results of surgical treatment and endovascular repair of AAA[1] in patients with Behçet's syndrome.

Methods: We retrieved the medical records of all 53 patients with Behçet disease admitted to Rambam Medical Center during the years 1985 and 2001 and analysed the results and follow-up of open surgery versus endovascular repair of AAA in patients with known Behçet's syndrome.

Results: Of the 53 patients with Behçet's disease 18 had vascular manifestations (34%). AAAs were encountered in 8 patients (15%) and 5 were treated. Open surgery (group 1), under general anesthesia, lasted less than 3 hours with an average aortic clamping time of 34 minutes (range 26–41 min) after which the patients were transferred to the intensive care unit for 24–48 hours. Endovascular treatment (group 2), although lasting about the same time without the need for intensive care, necessitated contrast media and fluoroscopy. The length of hospital stay was considerably shorter for patients after endovascular repair compared to open surgery (3 days vs. 6 days). Combined mortality and morbidity was higher in patients who underwent open surgery compared to endovascular repair (one death, one major amputation and three anastomotic pseudoaneurysms compared to one temporary contrast-induced nephropathy).

Conclusions: Vasculo-Behçet patients with AAA are better candidates for endovascular treatment than atherosclerotic patients. Combined morbidity (especially anastomotic pseudoaneurysms) and mortality of Behçet patients after endovascular repair is considerably lower than after open surgery.

Patients with graft-versus-host disease suffer from xerostomia, oral infections and mucosal pathologies. The continuous increase in the number of patients treated worldwide with bone marrow transplants, combined with improved survival statistics result in a concomitant increase in the number of GVHD[1] patients. the pathogenesis of GVHD is based on donor graft T lymphocytes that recognize antigenic disparities between donor and recipient, and on the disregulation of a broad panel of cytokines. Consequently, various tissues and organs, including the mucosa of the oral and gastrointestinal tract, are damaged via cytotoxicity caused by infiltrating T cells. Since the salivary glands are a known major target of GVHD and their secretions significantly contribute to preserving mucosal integrity, this mucosal insult is further enhanced by the reduced quantity and altered quality of saliva. GVHD occurs in 40–70% of patients treated by bone marrow and peripheral blood stem cell transplantation. limited studies suggest that a large percentage of GVHD patients are affected and that the induced salivary dysfunction occurs rapidly following transplantation, affecting both major and minor salivary glands and reflecting the severity of the disease. Moreover, profound sialochemical alterations may be diagnostic of GVHD. an additional reason for the vast amount of research is that GVHD, as an autoimmune-like disease, seems to be an appropriate model for studying a much more prevalent, well-known and studied autoimmune disease involving salivary glands, namely, sjögren’s syndrome. The present review describes the GVHD-related sialometric and sialochemical data available in the literature for both major and minor salivary glands in both human and rodent models, and discusses a possible mechanism.

Background: Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition associated with inflammatory bowel disease. PG[1] occurs rarely in IBD[2] patients and there are insufficient data on the clinical manifestations of this disease with IBD.

Objective: To determine the incidence, clinical manifestations and treatment of PG in patients with IBD and the connection to IBD, its activity and extent.

Methods: All patients hospitalized with IBD at a university hospital during a 20 year period were evaluated for the occurrence of PG.

Results: Of 986 patients hospitalized for IBD 6 suffered from PG (0.6% incidence). Their average age was 37 with equal sex distribution and equal distribution of Crohn’s disease and ulcerative colitis. PG appeared 6.5 years on average after diagnosis of IBD in all patients. The development of PG correlated with significant clinical exacerbation of IBD, the majority having active colitis at the onset of the PG. Extra-intestinal manifestations of IBD occurred in half the patients (sacroiliitis, peripheral arthritis and erythema nodosum). Pathergy was not elicited in any patients. Four patients had multiple skin lesions, frequently on the lower extremities. Diagnosis was made by skin biopsy in four patients. There was little correlation between amelioration of IBD and the skin lesions. Treatment consisted of high dose steroids and immunomodulatory drugs (cyclosporine, azathioprine and dapsone) in conjunction with topical treatment.

Conclusions: PG is a rare extra-intestinal manifestation of IBD that coincides with the exacerbation of the intestinal disease but does not always respond to treatment of the bowel disease.

Background: Screening for celiac disease is based on the sequential evaluation of serologic tests and intestinal biopsy; an optimal screening protocol is still under investigation. The screening policy of one of the main healthcare providers in Israel (Maccabi) consists of measuring total immunoglobulin A and tissue transglutaminase IgA[1] antibodies and confirming positive results by endomysial antibodies. For IgA-deficient patients antigliadin IgG is measured.

Objectives: To evaluate the use of tTGA[2] as a first-level screening test in patients suspected of having celiac disease

Methods: The results of tTGA and EMA[3] tests over a 3 month period were obtained from the laboratory computer. Letters were sent to the referring physicians of patients with positive tests, requesting clinical information and small intestinal biopsy results. tTGA was performed using an anti-guinea pig tTG-IgA enzyme-linked immunosorbent assay kit.

Results: Overall, 2,505 tTGA tests were performed: 216 (8.6%) were tTGA-positive of which 162 (75%) were EMA-negative (group 1) and 54 (25%) EMA-positive (group 2). Clinical information was obtained for 91 patients in group 1 and 32 in group 2. Small intestinal biopsy was performed in 33 (36%) and 27 patients (84%) in groups 1 and 2, respectively. Celiac disease was diagnosed in 4 biopsies (12%) in group 1 and 23 (85%) in group 2 (P < 0.0001). The positive predictive value was 45% for tTGA and 85% for EMA.

Conclusions: Symptomatic patients with positive tTGA and negative EMA have a low rate of celiac disease compared to those who are tTGA-positive and EMA-positive. Confirmation with EMA is advised when tTGA is performed as a first-level screening for suspected celiac disease.

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.

Background: Cardiac surgery is being performed with increasing frequency in patients aged 80 years and older.

Objectives: To examine the long and short-term results of surgery in this age group.

Methods: We retrospectively investigated 202 consecutive patients aged 80 years or older who underwent cardiac surgery between 1991 and 1999. Ninety-six operations (48%) were urgent.

Results: The study group comprised 140 men (69%) and 62 women (31%) with a mean age of 82.1 years (range 80–89). Preoperatively, 120 patients (59%) had unstable angina, 37 (18%) had left main coronary artery disease, 22 (11%) had renal failure, 17 (8.5%) had a history of stroke, and 13 (6.5%) had previous cardiac surgery. Hospital mortality for the whole group was 7.4%. Postoperative complications included: re-exploration for bleeding in 15 (7.4%), stroke in 8 (4%), sternal wound infection in 3 (1.5%), low cardiac output in 17 (8.4%), new Q wave myocardial infarction in 5 (2.5%), renal failure in 17 (8.5%), and atrial fibrillation in 71 (35%). The actuarial survival for patients discharged from the hospital was 66% at 5 years and 46% at 8 years. The type of surgical procedure was significantly associated with increased early mortality (coronary artery bypass grafting only in 2.9%, CABG[1] + valve in 16.1%, valve only in 16.7%; P = 0.01). Significant predictors (P < 0.05) for late mortality included type of surgical procedure, congestive heart failure, and postoperative low cardiac output.

Conclusions: When appropriately applied in selected octogenarians, cardiac surgery can be performed with acceptable mortality and good long-term results.