[THE VALUE OF MUSCLE BIOPSY IN PATIENTS WITHOUT WEAKNESS, HIGH CREATINE KINASE OR MYOPATHY ON ELECTROMYOGRAPHY].

INTRODUCTION: Muscle biopsy is an important diagnostic procedure for the evaluation of neuromuscular disorders, commonly employed when patients present with muscle weakness, high creatine-kinase or electromyography which suggest myopathy. The diagnostic value of this procedure when these are normal is unclear. AIMS: To characterize the pathology in muscle biopsies of patients without clinical, laboratory or electromyographic suggestion for myopathy. METHODS: Retrospective chart and pathology review of consecutive patients who were evaluated by muscle biopsy at Sheba Medical Center. RESULTS: Of 109 patients, 12 (11%) had no indication for myopathy prior to biopsy. Pathology was identified in 2/3 of cases. Inflammation was detected in 5 cases (42%), with a perivascular infiltrate in four, and endomysial in one. A mild myopathy was present in 3/5 of these cases. Type-2 muscle fiber atrophy as the primary or only pathology was seen in 2 cases (17%) and mild neurogenic changes in one (8%). A history of systemic disease, additional laboratory tests or imaging suggestive for inflammation were predictive for inflammatory pathology in 4/5 of cases (P = 0.006). CONCLUSIONS: Perivascular inflammation without significant muscle fiber damage is common and meaningful in patients with inflammatory conditions in spite of normal evaluation for myopathy. DISCUSSION: Muscle disease is characterized by damage to muscle fibers, connective tissue or vessels. In the absence of fiber damage, muscle strength, creatine-kinase and electromyography may remain normal. These tests therefore do not rule-out perivascular inflammation and mild myopathy. Muscle biopsy is effective for the detection of inflammation in patients with inflammatory conditions in spite of normal strength creatine-kinase and electromyography.

Social and utilitarian considerations for allocating organs within a national organ sharing system: a computerized simulation model for policy decision-making.

BACKGROUND: An organ-sharing system should achieve fairness and optimal graft longevity. Balancing between social and utilitarian considerations is a sensitive ethical, public and medical issue that requires a means to examine the consequences of any allocation policy or planned changes thereof. OBJECTIVE: To evaluate the performance and applicability of a computerized simulation model by examining the impact of two opposing organ allocation policies (social or utilitarian) on predicted organ distribution regarding age, waiting time, recipient sensitization measured by panel reactive antibody level, and overall donor-recipient tissue matching (measured by the number of HLA antigen mismatches). METHODS: Using a computerized simulation model, virtual donors and recipients were emulated and organs were allocated according to either social algorithms or utilitarian policies. The resulting number of HLA mismatches, PRA, age, and waiting time distributions were compared between allocation strategies. RESULTS: Simulating allocation of 7,000 organs to 17,000 candidate recipients and implementing social policies yielded donor-recipient compatibility comparable to utilitarian policies (0-1 mm: 19.4% vs. 28%) while allocating 66.7% of organs to long waiters (>48 months). CONCLUSION: This computerized simulation model is a valuable tool for decision-makers establishing or modifying organ allocation policies.

Focused, minimally invasive radio-guided parathyroidectomy: a feasible and safe option for elderly patients with primary hyperparathyroidism.

BACKGROUND: Primary hyperparathyroidism in elderly patients is usually associated with additional co-morbidity that increases operative risk, and thus many geriatric patients are denied the benefit of surgery for a single parathyroid adenoma. OBJECTIVES: To evaluate the safety and efficacy of accurate single photon emission computed tomography sestamibi scintigraphy, enabling precise localization of a single adenoma, in the geriatric population. METHODS: Twenty-two patients aged 70 years and over with biochemically proven PHPT and with a single parathyroid adenoma identified by localization studies (sestamibi SPECT scan and ultrasonography) underwent 23 operations over 29 months (out of a total of 140 patients operated upon during the same period). Immediate preoperative sestamibi scintigraphy and marking of focal adenoma uptake followed by intraoperative hand-held gamma probe were used for the removal of the parathyroid adenoma by unilateral minimal access surgery. Associated major co-morbid conditions and pre- and postoperative calcium, phosphorus and parathormone levels were recorded. Indications for surgery were listed and operative and postoperative complications were noted. The patients were followed for a mean period of 17.7 months using the same parameters. RESULTS: The 22 patients with PHPT had a mean age of 76.3 +/- 5.9 years (range 70-88 years) and a female to male ratio of 13:9. Associated co-morbidity included ischemic heart disease (n = 15), hypertension (n = 22), non-insulin-dependent diabetes mellitus (n = 9), chronic obstructive pulmonary disease (n = 3), and previous neck surgery (n = 3). Mean preoperative serum calcium, phosphorous and PTH were 11.7 +/- 1.3 mg/dl, 2.5 +/- 0.5 mg/dl and 160.9 +/- 75.4 pg/ml respectively. In 20 of the 22 patients, surgery was successful in curing PHPT (91%). One patient had persistent hypercalcemia due to a missed adenoma, and repeat operation (by focused minimal accesss surgery) was successfully performed 2 weeks later. There were no complications and no morbidity postoperatively. Mean postoperative serum calcium, phosphorous and PTH were 9.6 +/- 1.2 mg/dl, 3.0 +/- 0.5 mg/dl and 35.2 +/- 24 pg/ml respectively. In all patients, serum calcium levels remained normal (9.7 +/- 1.3 mg/ml) after long-term follow-up (mean 17.7 +/- 9.6 months). CONCLUSIONS: Minimally invasive, radio-guided focused parathyroidectomy for a single adenoma is a safe and effective method to cure hyperparathyroidism in the elderly. Success of surgery is directly related to the surgeon's experience and to the precise localization marking provided by sestamibi scintigraphic SPECT localization and concurrent sonographic findings.

Detrimental effects of cyclosporin A on long-term graft survival in familial Mediterranean fever renal allograft recipients: experience of two transplantation centers.

BACKGROUND: Cyclosporin A has been associated with severe toxic side effects in patients with familial Mediterranean fever who underwent renal transplantation. Nevertheless, the impact on graft function and survival is not well documented. OBJECTIVE: To compare long-term graft function and survival, between CsA-based vs. CsA free immunosuppressive protocols in FMF recipients of renal allograft. METHODS: Data of FMF recipients were analyzed retrospectively. Graft survival and function and the incidence of acute rejection were correlated to graft source (living donor vs. cadaveric donor), colchicine dose, presence of proteinuria, and immunosuppression protocol (CsA-based triple drug therapy vs. azathioprine-prednisone alone). RESULTS: There were 35 FMF patients with primary renal grafts (13 from living donors and 22 from cadaveric donors). Mean follow-up was 10.6 +/- 6.05 years. Sixteen patients were on CsA-based triple drug therapy and 19 patients on AZA-Pred alone. Mean overall graft survival was 11.2 +/- 0.6 years and 9.4 +/- 1.36 vs. 11.6 +/- 0.4 years for CsA-treated and AZA-Pred groups respectively (P = 0.05). One-year survival was 94% and 96.6% for CsA-treated vs. non-CsA patients (not significant), but 5 and 10 years survival were 76% and 46%, compared to 94.5% and 86% respectively (P = 0.05 at 5 years and 0.001 at 10 years). Mean serum creatinine at time of data collection was 2.3 +/- 1.5 mg/dl in the CsA group vs. 1.6 +/- 0.7 mg/dl in the AZA-Pred group (P = 0.02). There were 14 and 13 reversible rejection episodes in the AZA-Pred and CsA groups respectively (not significant). CONCLUSION: It is suggested that CsA exerts detrimental effects on long-term renal graft function and survival in FMF patients.