Israel Medical Association Journal

Background: New drugs have significantly improved the prognosis and quality of life of patients with pulmonary arterial hypertension. However, PAH[1] associated with autoimmune disease, particularly progressive sclerosis, remains a very serious problem

Objectives: To evaluate whether the course of the disease and survival is significantly different in patients with PAH related to autoimmune disease as compared to other patients with PAH and to determine the prognostic factors in these patients.

Methods: We retrospectively compared 24 patients with PAH associated with autoimmune disease to 42 patients with other causes of PAH. We focused on the clinical and hemodynamic parameters and on the outcome.

Results: The early mortality rate was slightly higher in patients with PAH associated with autoimmune disease (13% after the first year, 25% after the fifth year). The prognostic factor was a shorter distance on the 6 minutes walking distance test (r = 0.2, P = 0.01).

Conclusions: The early detection of PAH associated with autoimmune disease should encourage earlier and more aggressive treatment than in idiopathic PAH.

Background: Metastatic melanoma is an aggressive and highly malignant cancer. The 5 year survival rate of patients with metastatic disease is less than 5% with a median survival of only 6–10 months. Drugs like dacarbazin (DTIC) as a single agent or in combination with other chemotherapy agents have a response rate of 15–30%, but the duration of response is usually short with no impact on survival. Interleukin-2-based immunotherapy has shown more promising results. The National Institutes of Health recently reported that lymphodepleting chemotherapy, followed by an adoptive transfer of large numbers of anti-tumor specific tumor-infiltrating lymphocytes, resulted in an objective regression in 51% of patients.

Objectives: To introduce the TIL[1] technology to advanced metastatic melanoma patients in Israel.

Methods: We generated TIL cultures from tumor tissue, choosing those with specific activity against melanoma and expanding them to large numbers.

Results: TIL cultures from nine patients were established and examined for their specific activity against the patients' autologous tumor cells. Twelve TIL cultures derived from 5 different patients showed the desired anti-tumor activity, making those 5 patients potential candidates for the therapy.

Conclusions: Pre-clinical studies of the TIL technology in a clinical laboratory set-up were performed successfully and this modality is ready for treating metastatic melanoma patients at the Sheba Medical Center's Ella Institute.

Objectives: To review the current status of MAS[1] in a pediatric tertiary care center in Israel, in terms of feasibility, safety, and effect on standard practices.

Methods: We reviewed the files of all children who underwent a MAS procedure in our department during the period April 2002 to July 2004, and compared the findings with those of children treated by standard practices.

Results: A total of 301 procedures were performed in 271 patients: 107 thoracoscopic and 194 laparoscopic. There were no major intraoperative complications. The total conversion rate was 3.65%: 0 for thoracoscopy and 5.6% for laparoscopy (11/194). Twenty-four types of procedures were performed during the study period. The thoracoscopies accounted for 92.24% of all thoracic procedures in the department (107/116), and routine abdominal laparoscopic procedures replaced open surgery in 30–100% of cases (total 44.8%, 194/433).

Conclusions: MAS procedures appear to be safe for a wide range of indications in children. In our center they currently account for a significant percentage of pediatric surgeries. We suggest that the integration of MAS training in the residency programs of pediatric surgeons be made a major long-term goal. The creation of a pediatric MAS study group, which would allow for multi-institutional studies, is especially important in Israel where a relatively large number of pediatric surgery departments handle a small annual number of patients.

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[1] MAS = minimal access surgery

Background: Restorative proctocolectomy eliminates the risk of colorectal cancer in patients with familial adenomatous polyposis. Complications and extra‑intestinal manifestations are inherent to the procedure.

Objectives: To evaluate operative procedures, complications, early and late results and long-term functional outcome in FAP[1] patients operated in our department.

Methods: The study group included all patients with FAP who were operated between 1988 and 2003. Demographic data, length of follow‑up, complications, colorectal cancer, pouch function and extracolonic manifestations were recorded.

Results: Median age at surgery was 33 years (range 13–61 years). The final operative breakdown was: 48 proctocolectomies, 41 ileal pouch-anal anastomoses, 2 Kock’s pouch, 5 permanent ileostomies, and 2 colectomies with ileorectal anastomosis. There was no perioperative mortality. Early and late complications occurred in 20 and 9 patients, respectively. Twelve patients required re‑operation. Colorectal carcinoma was diagnosed in eight patients, three of whom were in an advanced stage. The mean follow‑up was 74 months (range 3–288 months). Four patients were lost to follow‑up. Extracolonic manifestations developed in 38 patients, including desmoid tumors (in 12), duodenal adenomas (in 9), pouch adenomas (in 5), and rectal stump adenomas (in 3). Two patients died (4%) because of desmoid tumor and malignant fibrous histiocytoma. At last follow‑up, 37 IPAA[2] patients have (median) six bowel movements/24 hours and good fecal control.

Conclusions: Restorative proctocolectomy can be performed with low mortality, acceptable morbidity, and good functional results. Patients should be closely followed after surgery for development of other manifestations of the syndrome. Relatives of the affected patients should be referred to a specialist multidisciplinary clinic.

Objectives: To evaluate operative procedures, complications, early and late results and long-term functional outcome in FAP[1] patients operated in our department.

Methods: The study group included all patients with FAP who were operated between 1988 and 2003. Demographic data, length of follow‑up, complications, colorectal cancer, pouch function and extracolonic manifestations were recorded.

Results: Median age at surgery was 33 years (range 13–61 years). The final operative breakdown was: 48 proctocolectomies, 41 ileal pouch-anal anastomoses, 2 Kock’s pouch, 5 permanent ileostomies, and 2 colectomies with ileorectal anastomosis. There was no perioperative mortality. Early and late complications occurred in 20 and 9 patients, respectively. Twelve patients required re‑operation. Colorectal carcinoma was diagnosed in eight patients, three of whom were in an advanced stage. The mean follow‑up was 74 months (range 3–288 months). Four patients were lost to follow‑up. Extracolonic manifestations developed in 38 patients, including desmoid tumors (in 12), duodenal adenomas (in 9), pouch adenomas (in 5), and rectal stump adenomas (in 3). Two patients died (4%) because of desmoid tumor and malignant fibrous histiocytoma. At last follow‑up, 37 IPAA[2] patients have (median) six bowel movements/24 hours and good fecal control.

Conclusions: Restorative proctocolectomy can be performed with low mortality, acceptable morbidity, and good functional results. Patients should be closely followed after surgery for development of other manifestations of the syndrome. Relatives of the affected patients should be referred to a specialist multidisciplinary clinic.

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[1] FAP = familial adenomatous polyposis

[2] IPAA = ileal pouch-anal anastomosis

Background: Burn trauma occurs mostly in young children. Burn injury in the pediatric age group has multiple-aspect sequelae.

Objectives: To characterize the profile of the injured pediatric burn patient, thus targeting the most vulnerable pediatric group.

Methods: Between 1 January and 31 December 1996, a total of 9,235 pediatric patients were admitted for various traumatic injuries (burns, lacerations, fractures, etc.) to the Emergency Medicine Department of Schneider Children’s Medical Center. We conducted a retrospective study of the patients’ charts, including demographic data, which were stored in a computerized database, for statistical evaluation. The characteristics of pediatric burn patients were examined and compared with other pediatric trauma patients.

Results: Of the total patient population, 282 (3.1%) suffered from burns (37% females, 63% males). The most frequent burn injury was scald burn (58%). The pediatric group that was most exposed to burns was 13–18 month old males.

Conclusions: Having identified the high risk group among the pediatric burn patients, we suggest that prevention programs be directed towards this group in order to reduce further risk of burn injury.