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INTRODUCTION: Determination of outcomes after adrenalectomy for primary aldosteronism (PA) is limited by the lack of standardized definitions of cure. The Primary Aldosteronism Surgical Outcomes (PASO) group recently established new consensus definitions for biochemical and clinical cure of PA. We hypothesize that utilization of PASO definitions will better stratify patient outcomes after surgery compared to original and current criteria utilized to document cure. MATERIALS AND METHODS: Patients undergoing adrenalectomy for PA from 1996 to 2016 were studied. Clinical data were reviewed. Three different sets of criteria (original, current, and PASO) were evaluated for differences in documentation of cure. Demographic data were reported as median (range). Comparisons were made using the Mann-Whitney U test; p < 0.05 is significant. RESULTS: A total of 314 patients with PA were identified. Ninety patients (60 males) elected to proceed with surgery. In Group 1 (35 patients), 30 patients had clinical follow-up and 29 (97%) were cured using original criteria. In Group 2 (55 patients), cure was recorded in 98% when original criteria for cure were applied, 89% cured applying current criteria, and 6% had complete biochemical and clinical cure by PASO criteria. Aldosterone rose 3.6 ng/dL (0.1-34.8) in five patients during extended follow-up, with two patients changing from complete to partial or missing biochemical success. CONCLUSION: Significant heterogeneity exists in outcomes criteria utilized to document cure or clinical improvement after adrenalectomy for primary aldosteronism. Aldosterone levels change over time after adrenalectomy. PASO definitions of cure appear to allow for improved stratification of short- and long-term outcomes.

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Primary aldosteronism (PA) is the most common cause of endocrine hypertension. Although adrenal venous sampling (AVS) is recommended as the gold standard procedure for subtype classification in PA, it is a specialized technique with limited availability. The objective of this study was to develop a scoring system that predicted PA subtype using clinical characteristics. Seventy-one patients with PA were studied. The subjects were diagnosed as having either unilateral (n=32) or bilateral disease (n=39) based on AVS, surgery and/or the postoperative clinical course. Variables associated with laterality in the univariate analysis were entered into multivariable logistic regression models and the regression coefficients were used to construct a subtype prediction score. The diagnostic significance of the score was then evaluated using receiver operating characteristic (ROC) curve analysis. The subtype prediction score was calculated as follows: serum potassium ⩽3.4 mEq l(-1), 2 points; plasma aldosterone concentration ⩾165 pg ml(-1), 3 points; and aldosterone to renin ratio ⩾1000 in a post-captopril challenge test (plasma renin activity in ng ml(-1) h(-1)), 3 points. ROC curve analysis for the ability to discriminate between unilateral and bilateral PA showed that a score of 5 points had 75% sensitivity and 95% specificity, and a score of 3 points had a sensitivity of 97% and a specificity of 59%. The area under the ROC curve was 0.920 (95% confidence interval, 0.859-0.979). Our subtype prediction score could discriminate between unilateral and bilateral PA and is useful for selecting patients who should undergo AVS before surgery.

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In a living renal transplantation, the recipients are administered an immunosuppressive agent preoperatively. The drug exhibits a high incidence of side effects of special note. We examined the side effects of tacrolimus to evaluate the postoperative management of living renal transplantation. Hypertension, hyperglycemia, tachycardia and chest pain were found as the side effects. The blood concentration should be measured frequently to maintain the effective blood concentration and to prevent the side effect.

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We report a rare male case of homocystinuria probably due to methylenetetrahydrofolate-reductase deficiency. The onset of his disorder was at 19 years of age, and he had no family history. He initially developed gait disturbance, and then generalized seizure in several months, which made him admitted to our hospital. Neurological examinations revealed mental dysfunction, spastic paraplegia, cerebellar ataxia, and sensory disturbance in his feet. MRI showed multiple increased intensities on T2-weighted images in the cerebral white matter. EMG revealed neurogenic changes. These symptoms and signs slowly progressed, and he then developed thrombophlebitis in his lower extremities. Thrombin-antithrombin III complex (TAT) and D-dimer remained high continuously, and plasma homocysteine level was more than ten times higher than the normal range. Plasma cystathionine level was high and methionine level was low. The serum folic acid, vitamin B12, and methylmalonic acid in the urine were normal. Megaloblastic anemia was not seen. Based on these data, he was diagnosed to have homocystinuria probably due to methylenetetrahydrofolate-reductase deficiency. Treatment with high doses of folic acid, pyridoxine and cobalamin normalized plasma cystathionine and methionine levels, and markedly decreased plasma homocysteine, although it remained about three times higher than the normal range. Thereafter, both TAT and D-dimer levels also markedly decreased. The administration of folic acid reduced elevated plasma homocysteine as well as the coagulation--fibrinolysis factors. This implies that they may serve as useful markers for effective treatment of this disease.

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