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We describe a 45-year-old Taiwanese man with specific features of Brugada syndrome but no clinical features of structural heart disease. He was successfully treated with an implantable cardioverter-defibrillator. His electrocardiogram (ECG) patterns changed intermittently. Alpha-adrenoceptor stimulation and beta-adrenoceptor blockade augmented the characteristic ST-segment elevation, whereas alpha-adrenoceptor blockade and beta-adrenoceptor stimulation mitigated the ST-segment elevation. Intravenous procainamide administration did not aggravate ST-segment elevation when ECG had shown coved ST elevation in the right precordial leads. Molecular study did not reveal the same mutations in the cardiac sodium channel gene (SCN5A) as previously reported in Brugada syndrome. This case demonstrates the genetic heterogeneity of SCN5A in Brugada syndrome.

We evaluated the effects of zinc deficiency on the activities of endogenous antioxidant enzymes and lipid peroxidation in rat glomerular cells (GCs). Male Sprague-Dawley rats (n = 48) were fed a zinc-deficient diet and deionized distilled water for 1 week to induce zinc deficiency. Half of the rats (zinc-deficient group) continued on this diet for 4 weeks, and the other half (zinc-replete group) were maintained on the same diet but with zinc-supplemented water (150 mg/Lzinc sulfate solution). Half of each group underwent five-sixths nephrectomy, while the other half underwent a sham operation. Another 12 normal rats (controls) were fed standard rat chow (containing 23.4% protein and 70 ppm zinc) and drank deionized distilled water. The zinc-deficient rats, including sham and five-sixths nephrectomized rats, showed severe growth retardation and poor appetite. Their mean plasma zinc concentrations were half that of normal control rats, but their plasma copper concentration was significantly higher than that of the control rats. Zinc supplementation corrected the abnormality of plasma zinc and copper concentrations and the loss of body weight in zinc-deficient rats. Zinc-deficient rats exhibited lower renal creatinine clearance and higher GC-malondialdehyde (GC-MDA) than zinc-replete rats. The remnant kidney of all five-sixths nephrectomized rats, including zinc-deficient and zinc-replete rats, showed a compensatory elevation in renal creatinine clearance and increased GC-MDA concentrations. Zinc concentrations in the renal cortex were decreased in zinc-deficient rats and the activities of GC-superoxide dismutase and GC-glutathione peroxidase were increased, while zinc-replete rats exhibited normal activities of GC-superoxide dismutase and GC-glutathione peroxidase. We suggest that zinc deficiency enhances the formation of reactive oxygen species but does not affect the activities of endogenous antioxidant enzymes in glomerular cells.

Two girls with unilateral hematocolpos are reported. In both cases, a small amount of blood which had accumulated in the partially obstructed hemivagina was detected by real-time high resolution ultrasonography, and was confirmed by magnetic resonance imaging. Both patients were asymptomatic, and were regularly followed up at an outpatient clinic. To date, the hematocolpos persists but continues to be small.

Seventeen patients who received radioiodine (131I) therapy for Graves' hyperthyroidism had serial blood samples taken before therapy and after therapy for a period of at least 1 year. At 1 year post-therapy, six patients were hypothyroid. Seven patients were euthyroid, and four patients were hyperthyroid. Prior to isotope administration, 14 patients had detectable serum thyrotropin-binding inhibiting immunoglobulin (TBII) and 16 patients had detectable serum thyroperoxidase antibody (TPOAb). Three to 6 months after therapy, 11 of 14 TBII-positive patients demonstrated a marked increase (10%) in serum TBII activity. Four patients out of 11 developed hypothyroidism and six of the 11 developed euthyroidism. A decrease in TBII was observed in three patients who developed hyperthyroidism. In the three patients with undetectable TBII prior to therapy, two had high titers of TPOAb. Seven patients demonstrated a marked increase in TPOAb 3 to 6 months after therapy. Of these, four developed hypothyroidism and three developed euthyroidism, whereas three of the four patients who had a marked decrease in TPOAb developed hyperthyroidism. This study demonstrated that an increase in serum TBII and TPOAb activity 3 to 6 months after 131I therapy, may be useful in predicting which patients may develop euthyroidism or hypothyroidism after 1 year of 131I therapy.

Platelet-reduced glutathione (GSH), superoxide dismutase (SOD) and zinc content as well as malondialdehyde (MDA) formation were studied in 55 healthy volunteers as controls and 49 patients with chronic renal failure. Uremic patients had a significantly lower GSH and SOD content as well as an abnormal MDA formation in their platelets. Their plasma zinc level was sharply below the normal value, but their platelet zinc content was similar to that of normal controls. We also found that the plasma zinc and platelet zinc level was positively correlated to the platelet GSH content in normal controls and uremic patients. Therefore, the results suggest that in uremic patients the decrease in platelet antioxidant agents (GSH and SOD) may be partially related to their low plasma zinc level.

Using a specific citrate lyase method, renal excretion of citrate was studied in 32 normal Chinese males, 30 nondialysed uremic male patients and 35 male subjects who had a history of nephrolithiasis. Patients with uremia or nephrolithiasis were found to have a lower urinary citrate excretion. Tubular reabsorption of citrate was markedly decreased in uremic patients, but in stone patients, the increased renal tubular reabsorption of citrate was only found in patients with hypocitraturia, whose renal citrate excretion was below 650 mumol/day and whose urinary magnesium was also low. Hypocitraturia was found in 45% (16/35) of the patients with renal stones whether their filtered load of citrate was normal or subnormal. Urinary citrate excretion was correlated with renal creatinine clearance in both normal subjects and in patients with renal stones or chronic renal failure. However, urinary phosphate correlating with urinary citrate was only found in normal subjects and in patients with kidney stones. In normal subjects, we found a positive correlation between urinary citrate and phosphate, but in stone patients, we found a negative correlation. Hypercalciuria and hyperoxalaturia were noted in some stone formers, who had, moreover, a lower urinary citrate and ascorbate excretion level. Mean urinary ascorbate excretion in patients with renal stones was markedly below that in normal subjects. Thus, we suggest that low urinary citrate excretion may be prevalent in patients with renal stones or chronic renal failure, and that hypocitraturia can be found in some stone formers, whose tubular reabsorption of citrate may be increased.

This study was carried out in order to investigate renal oxalate excretion in a group of normal subjects (n = 40), a group of patients with uremia (n = 52) and a group with nephrolithiasis (n = 34). We found that the mean concentrations of oxalate in the 24-hour urine specimens of both patient groups were below the normal range. Although the renal creatinine clearance (CCR) was significantly decreased in some stone patients (n = 14), decreased renal oxalate clearance was noted only in those patients with severe renal failure. Thus, plasma oxalate was found to be elevated only in patients with chronic renal failure (mean +/- SD, 49.7 +/- 12.4 mumol/l), while the normal value was 17.0 +/- 6.7 mumol/l. The mean tubular excretion fraction of oxalate was also found to increase markedly in uremia with a mean of 26.3 +/- 17.3% (in normal subjects, 11.7 +/- 7.5%), but their mean daily urinary excretion of oxalate decreased to 63.2 mumol/day (mean value of 232.6 mumol/day in normal subjects). A positive correlation was observed between oxalate and creatinine, and between oxalate and calcium excretion, which was not found in normal subjects or patients with kidney stones. In nephrolithiasic patients, the daily excretion of oxalate, calcium and phosphate had no discernible increment and the mean excretory ratio of oxalate, calcium or phosphate to creatinine was all within normal limits. But when the CCR of stone patients was below 80 ml/min, their daily excretion of oxalate and calcium decreased significantly (p less than 0.01) and the excretory ratio of phosphate to creatinine markedly increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Though uremic patients have been reported to have subnormal zinc levels in plasma and an abnormal zinc metabolism, data on their renal excretion of zinc are still unavailable. In our study, 55 nondialyzed uremic patients had a markedly lower concentration of zinc in plasma and a lower urinary zinc excretion. They had a higher excretory fraction of zinc in the renal tubules and a higher ratio of zinc to creatinine excretion than normal subjects. We found that the total daily excretion of zinc, calcium, phosphate and ammonia was reduced, and that the ratio of calcium or ammonia to creatinine excretion decreased in uremic patients. Urinary zinc excretion was correlated with renal creatinine clearance (r = 0.50, p less than 0.001) in both normal subjects and uremic patients, but ammonia excretion was correlated with zinc excretion in normal subjects only. In uremia, urinary zinc excretion was correlated with urinary calcium (r = 0.55, p less than 0.001) and urinary phosphate excretion (r = 0.30, p less than 0.05). We conclude that the increased ratio of zinc to creatinine excretion and the excretory fraction of zinc in the renal tubules of uremic patients may increase the urinary zinc excretion, but a severe diminution of the glomerular filtered load of zinc may be the major factor in decreasing the urinary zinc excretion in uremia.