Israel Medical Association Journal

Background: Cystic fibrosis is no longer a terminal illness of childhood and mean survival is now over 30 years. Adult patients with atypical CF[1] are increasingly being diagnosed. In Israel, all patients are still followed in pediatric centers.

Objectives: To describe our experience with adult CF, stressing the importance of adult-related health and psychosocial issues.

Methods: Twenty-five CF patients aged 20–50 years, constituting 44% of the 57 patients followed at our center, were analyzed for pulmonary and extrapulmonary features and management.

Results: Nineteen were diagnosed as children and 6 as adults. Nineteen were pancreatic-insufficient and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 ± 21% (mean ± SD) and no difference between pancreatic-sufficient and insufficent patients. The latter had more hemoptysis, Pseudomonas infection, intestinal obstruction, liver disease and diabetes. Two patients died of malignancy and two of advanced lung disease. A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had children (none with CF). Patients diagnosed as adults had mild multisystem disease or isolated severe lung disease.

Conclusions: CF adults generally have a good quality of life. Advances in understanding the CF defect and a plethora of new treatment modalities bode well for the future. Patients must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in CF.

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Background: Radiofrequency ablation has recently become a viable treatment option for unresectable primary or secondary lesions confined to the liver.

Objective: To study the local therapeutic efficacy, side effects and complications of radiofrequency ablation for the treatment of hepatocellular carcinoma and liver metastases This is the first reported experience of radiofrequency ablation for treating malignant hepatic tumors in Israel.

Methods: Fifteen consecutive patients, aged 53–73 years, with 23 lesions (8 patients with HCC[1] and 7 with secondary liver tumors) underwent radiofrequency ablation under general anesthesia. RITA nine-array 5 cm thermal ablation catheter and the model 1500 generator were used. The mean diameter of all tumors was 4.28 cm (range 1–10 cm). Three lesions were 1–3 cm in diameter (small), 17 lesions measured 3.1–5 cm (medium), and 3 measured 5.1–10 cm (large).

Results: Complete necrosis was found in 8 (66%) of 12 HCCs by computed tomography scan. Of the remainder, diffuse tumor recurrence was demonstrated in three lesions (25%) after lipiodol injection and there was one local tumor recurrence. In the metastases group complete necrosis was found in 5 of 11 lesions (45%). One major complication (peritonitis) was treated with antibiotics and four (26%) minor complications (right pleural effusion, small subcapsular hematoma) were monitored.

Conclusions: Radiofrequency ablation appears to be an effective, safe and relatively simple procedure for the treatment of liver tumors.

Background: Lower limb critical ischemia is a major problem in dialysed patients.

Objective: To evaluate the results of revascularization procedures, amputations and prosthetic rehabilitation in dialysed amputees.

Methods: Major amputation was carried out in 48 patients (4.5% of the dialysis population), and 24 patients entered the rehabilitation program. Widespread arterial calcification was common and led to falsely elevated ankle-brachial pressure indices in 9 of 14 limbs. Eight patients underwent revascularization. Subsequent major amputation was carried out 4 ± 4.5 months after the revascularization (above knee in 5 patients and below knee in 3). Of the 16 patients who underwent primary amputation, only 2 were above-knee amputees. Seven patients with toe or metatarsal amputation went on to a major amputation 1.8 ± 1.2 months after the distal amputation.

Results: No differences were found between diabetic and non-diabetic patients regarding the number of revascularization operations performed, the level of major amputation, or overall survival. Prosthetic rehabilitation was considered successful in 12 patients, partially successful in 8, and failed in 4 patients. Patient survival time was shortest in those patients with failed rehabilitation. A younger age confirmed favorable rehabilitation results, while long-standing diabetics and bilateral amputees were poor rehabilitation candidates. Patients who underwent primary amputation had more successful rehabilitation. A comparison between 24 dialysed amputees and 138 non-uremic amputees revealed similar rehabilitation results, although hospitalization time was longer in the dialysed patients.

Conclusions: Early definitive therapy is essential when dealing with critical ischemia. After diagnostic angiography, proximal revascularization should be performed where feasible. Primary amputation is indicated in patients with extensive foot infection or gangrene. Prosthetic rehabilitation is warranted in most dialysed amputees.
 

Diffuse idiopathic skeletal hyperostosis is often incorporated into osteoarthritis. Although DISH[1] often coexists with OA, patients affected by this disorder differ from patients with primary OA in several aspects: prevalence in the general population, gender distribution, anatomic site of primary involvement, magnitude and distribution in the spine and the peripheral joints. DISH is a distinct clinical entity. Its recognition as such should stimulate clinicians and researchers to focus on its pathogenesis, treatment and prevention.

Sepsis is an infection-induced inflammatory syndrome that results in a complex network of adaptive and maladaptive alterations in homeostatic mechanisms. Severe sepsis, defined as sepsis associated with acute organ failure, is a serious disease with a mortality rate of 30–50%. The coagulation system, through complex interactions, has an important role in the final outcome of the sepsis-induced inflammatory cascade. A fine and delicate balance that normally exists between anticoagulant mechanisms and the procoagulant response is altered in sepsis. Activated protein C, an endogenous vitamin K-dependent anticoagulant, plays a major role in the down-regulation of the procoagulant arm. It also possesses anti-inflammatory properties. Endothelial damage during sepsis impairs the endothelium-dependent activation of protein C, thus shifting the balance towards thrombosis. This shift may contribute to the development of sepsis-related multi-organ failure. Evidence suggesting that activation of the coagulation system may contribute to sepsis-related morbidity and mortality has led to extensive research attempting to correct the hemostatic defects seen in septic patients. Indeed, a recent randomized controlled trial demonstrated a reduction in overall mortality in patients with severe sepsis treated with APC[1]. In this review we discuss the pathogenesis of the coagulopathy of sepsis, as well as the new therapeutic approaches aimed at correcting the defects in the coagulation system.

The induced sputum technique allows sampling of the airways in a non-invasive manner and thus offers a unique opportunity to identify biomarkers of potential clinical utility in respiratory medicine. Sputum cells were originally examined in stained smears and the procedure was applied in both research and clinical settings from the 1950s through the 1970s. The cells, recovered from spontaneous coughing, were used to study lung cancer and respiratory infections and, later on, to diagnose Pneumocystis carinii pneumonia in patients infected with human immunodeficiency virus. The method was largely improved by the induction of sputum with aerosol of hypertonic saline and was extended to become part of the assessment of airway inflammation in bronchial asthma and chronic obstructive pulmonary disease. It was recently shown that induced sputum can be used to study interstitial lung diseases and, more specifically, sarcoidosis, non-granulomatous ILD[1], occupational lung diseases and other systemic diseases with lung involvement.

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