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Alkali therapy is widely accepted as a treatment for recurrent calcium stone patients with distal renal tubular acidosis (dRTA). Nine patients, five with complete and four with incomplete type of dRTA, were treated with alkali agents for more than three years; an average period of 10.8 years. In five patients, new stone formation and stone growth were completely prevented by this treatment. Among the four failed cases, three did not take enough alkali agents (in spite of our medication), and the other had recurrent urinary tract infection resulting in infectious stone formation. In conclusion, the long-term efficacy of alkali therapy for prevention of recurrent stone formation with dRTA is confirmed when the patient takes enough alkali agents and the urinary tract infection is well controlled.

Gelatinous transformation of the bone marrow is a rare disease characterised by a focal marrow hypoplasia, fat atrophy and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid, which is often associated with extreme malnutrition and weight loss. There are only two reports describing its association with systemic lupus erythematosus (SLE). One described underlying diseases in 155 cases of gelatinous transformation of the bone marrow and found one case with clinical diagnosis of SLE, but no clinical details were provided. The other described three SLE patients with gelatinous transformation of the bone marrow; however, two of these were cachectic and one was diagnosed with concomitant tuberculosis. We describe one active SLE patient without other comorbidities whose pancytopaenia was histologically confirmed as gelatinous transformation. The combination of high-dose steroid, intravenous immunoglobulin and mycophenolate mofetil improved the peripheral blood cytopaenia and reversed the bone marrow abnormalities.

Simple mechanical disintegration of cystine calculi by extracorporeal shock wave lithotripsy and/or percutaneous nephrolithotomy is being performed widely around the world. However, it cannot be denied that the cystine calculus is one of the most difficult stones for mechanical disintegration. We previously reported the oral medical chemolysis of cystine calculi in 1982 and a third of the patients became stone-free. In another third of the patients the cystine component was replaced by apatite during the medical treatment and apatite stones formed in this manner are easily disintegrated. In view of the complications of mechanical disintegration, oral medical treatment for chemolysis should be recommended before simple destruction of cystine calculi.

This study was undertaken to evaluate destructive spondylarthropathy (DSA) in patients long-term dialysis. The patients were investigated by plain X-ray examinations twice with a three-year interval, and the changes in severity of the illness in 66 patients undergoing dialysis for over 5 years were defined. Group A (9 patients) consisted of patients with progression in grades during the three years, while group B (57 patients) consisted of patients without deterioration. There was no differences in the two groups in age, duration and the modality of dialysis. However, in group A, the level of plasma beta 2-microglobulin (beta 2M) was 33.5 +/- 14.6 micrograms/ml at the first observation, and 33.1 +/- 15.1 micrograms/ml at the second (NS). In group B, the level of plasma beta 2M was 45.6 +/- 8.13 micrograms/ml at the first observation, and 39.7 +/- 9.18 micrograms/ml at the second (p0.05). No improvement in DSA was observed. The result of this study suggests that DSA will not improve once it has become overt, and the management for lowering the level of beta 2M may prevent the occurrence and progression of DSA.

Coronary atherosclerosis and calcification score were assessed angiographically in 30 uremic patients receiving dialysis (8 patients with diabetes mellitus [DM dialysis group] and 22 patients without DM [non-DM dialysis group] who were suspected of having coronary artery disease because of their clinical symptoms and electrocardiographic findings. Thirty non-uremic subjects (11 with DM and 19 without DM) served as controls. Twelve dialysis patients (40%) did not have significant coronary artery disease, and the rate of significant coronary artery disease in dialysis patients overall was less than that in the control subjects. The calcification score of dialysis patients was significantly higher than that of the control patients (P0.0001), and also scores of the non-DM dialysis group and the DM dialysis group were higher than those of each group of control subjects. The scores were significantly correlated with the duration of dialysis in uremic patients as a group (r = 0.52; P0.01) and the non-DM dialysis group (r = 0.70; P0.01), but were not correlated with the duration of dialysis in the DM dialysis group. The scores were correlated with serum phosphate concentration, but not with serum calcium concentration, calcium phosphate product, alkaline phosphatase levels total cholesterol or age. Calcification scores were extremely high (21) in six uremic patients who had high serum c-terminal parathyroid hormone concentrations. These findings indicate that serum phosphate concentration, serum c-terminal parathyroid hormone concentration, and the duration of dialysis are closely associated with coronary calcification in long-term dialysis patients.

We investigated factors affecting net transperitoneal calcium balance (Ca-BL) and the level of parathyroid hormone in relation to stepwise changes in serum calcium, by short PET (peritoneal equibrium test during 240 min: using 2000 ml of 2.5% dextrose dialysate containing 1.75 mmol/L Ca) in uremic patients undergoing stable CAPD. We calculated Ca-BL (mg/effluent/PET) of 244 effluents obtained from 90 patients receiving calcium carbonate as a phosphate binder without vitamin D supplementation. Their serum calcium level corrected with albumin (cSCa), alkaline phosphatase activity (ALP) and intact-PTH level was 9.7 +/- 0.9 mg/dl, 236 +/- 83 mIU/ml and 153.0 +/- 172.4 pg/ml, respectively. We proposed two statistic significant regression lines between Ca-BL and total drainage effluent volume (Ca-BL = 133X - 0.056: r = 0.981, P0.001), cSCa (Ca-BL = -12.9X + 123.6: r = 0.941, P0.01). Total drainage volume (TDV) and cSCa were two major factors affecting Ca-BL. A TDV level of 2430 ml/240 min-PET or more was required for positive Ca-BL in cases with 9.5-10.0 mg/dl of cSCa, using this linear regression analysis. A cSCa level of 9.6 mg/dl or more was also required for positive Ca-BL in cases with 2400-2600 ml/240min-PET. We also proposed a significant linear regression line between the intact-PTH level and cSCa (i-PTH = -90.5X + 1015.8, r = 0.973, P0.01). This line suggest that 200 pg/ml of intact PTH was obtained by 9.0 mg/dl or less of cSCa level in 90 CAPD uremic patients.(ABSTRACT TRUNCATED AT 250 WORDS)

The effects of parathyroidectomy on left ventricular muscle volume and mechanical performance were evaluated echocardiographically in 24 patients with secondary hyperparathyroidism due to chronic renal failure and in 7 with primary hyperparathyroidism. Intraventricular septum and posterior wall thickness, left ventricular end-diastolic diameters, shortening fraction, ejection fraction, and left ventricular mass index were measured by M mode recording by the parasternal short axis view prior to parathyroidectomy as baseline and repeated 12 months after parathyroidectomy. Serum basal carboxyterminal parathyroid hormone levels in patients with secondary hyperparathyroidism (34.4 +/- 13.7 ng/ml) were significantly higher than in those with primary hyperparathyroidism (3.4 +/- 5.1 ng/ml; p0.0001). At 12 months after parathyroidectomy, intraventricular septum and posterior wall thickness, left ventricular end-diastolic diameter and left ventricular mass index were reduced from 11.8 +/- 3.1 mm, 10.9 +/- 1.7 mm, 53.8 +/- 6.3 mm, 200.8 +/- 57.1 g/m2 to 10.0 +/- 2.1 mm (p0.05), 9.8 +/- 1.9 mm (p0.05), 50.7 +/- 7.2 mm (p0.05), 149.6 +/- 38.7 g/m2 (p0.0001), in patients with secondary hyperparathyroidism. In patients with primary hyperparathyroidism, all echocardiographic parameters remained in the normal range and did not show any significant changes before or after parathyroidectomy. From this study, parathyroid hormone at extremely high concentrations as seen in secondary hyperparathyroidism appears to be a cardiotoxic substance. Therefore, all patients with secondary hyperthyroidism should be examined by echocardiography and parathyroidectomy should be considered if myocardial hypertrophy is present.

To evaluate the left ventricular early diastolic function after dipyridamole infusion or during exercise, the first pass radionuclide angiocardiography (FPRA) by a multicrystal gamma camera (SIM-400) was performed in 32 patients with coronary artery disease (CAD) and 14 normal subjects. Dipyridamole was administered intravenously (0.56 mg/kg in 4 min). The first third filling fraction (FF 1/3) and the first third mean filling rate (MFR 1/3) were calculated from the time-activity curve. FF 1/3 decreased and MFR 1/3 increased in both normal subjects and patients with CAD during exercise. After dipyridamole infusion, FF 1/3 and MFR 1/3 unchanged in normal subjects, in contrast, FF 1/3 and MFR 1/3 decreased in patients with CAD. Thus, dipyridamole infusion FPRA is more sensitive method to detect early diastolic dysfunction in patients with CAD than FPRA during exercise.

Summary— Between March 1981 and March 1990, 15 patients with locally advanced transitional cell carcinoma of the bladder were treated concurrently with cisplatin and radiotherapy. Treatment comprised a radiation dose of 40-50 Gy in 20-25 fractions over 4-5 weeks and intravenous infusion of cisplatin with hydration during days 1-5 and 22-26. The total scheduled dose of cisplatin was 200 mg. A complete response (CR) was seen in 3 patients (2 T2 tumours and 1 T3) and the other 12 were regarded as partial responders. Two of the 12 partial responders (1 T2 tumour and 1 T4) underwent cystectomy after treatment, but 9 patients (2 T2,6 T3 and 1 T4) underwent only transurethral resection. The remaining patient (with a T4 tumour) died from systemic disease, further treatment not being possible because of unrelated heart failure. In 3 CR patients and 9 with a partial response (PR), bladder function was preserved and they have survived for a mean of 18.3 months (range 5-47) after therapy. Although 4 patients in this group had recurrent bladder tumours and 1 died from cancer in another part of the body, 7 have survived with normal bladder function and no recurrence. It is concluded that concurrent cisplatin and radiation therapy is a safe and viable regimen and may be considered as a means of preserving the bladder in patients with locally advanced transitional cell carcinoma.

A plastic ankle foot orthosis (AFO) was developed, referred to as functional ankle foot orthosis Type 2 (FAFO (II)), which can deal with genu recurvatum and the severe spastic foot in walking. Clinical trials were successful for all varus and drop feet, and for most cases of genu recurvatum. Electromyogram studies showed that the FAFO (II) reduced the spasticity of gastrocnemius and hamstring muscles and activated the quadricep muscles. Gait analysis revealed a reduction of the knee angles in the stance phase on the affected side when using the FAFO (II). Mechanical stress tests showed excellent durability of the orthosis and demonstrated its effectiveness for controlling spasticity in comparison with other types of plastic AFOs.

Clinical results of ESWL for renal and ureteral stone with MPL-9000 were presented. We also reviewed correlation between stone burden and shock wave energy. We studied 210 cases (295 sessions) of renal stone and 109 cases (154 sessions) of ureteral stone that were treated from November 1988 till January 1989 and from November 1989 till May 1990. Of those sessions, 76 sessions of renal stone cases and 53 of ureteral stone cases were carried out ambulatorily. Successful disintegration (within 4 mm) rate was 86% of renal stones, 84% of ureteral stones, and stone free rate after 6 months was 72% of renal stones and 84% of ureteral stones. As for the complications, brain infarction occurred in one case, subcapsular hematoma of the kidney in one, and arythmia in 5. No other severe side effects were observed. Between shock wave energy (the number of shock wave shots x voltage) and stone burden (long axis x short axis of the stone), a significant correlation was found in renal stone cases, but not in ureteral stone cases. E/B (shock wave energy/stone burden) was 33,100 (kv.shots/cm2) for the renal stone and 56,000 (kv.shots/cm2) for the ureteral stone. These results suggest that there is still another unknown factor, which is not related with renal stone, for successful disintegration of ureteral stone. We also confirmed that more energy is necessary for destruction of ureteral stone than for that of renal stone.(ABSTRACT TRUNCATED AT 250 WORDS)

Carcinoma of the urachus is a rare disease with poor prognosis. This is probably due to the difficulty of preoperative diagnosis. Radiological modalities have been considered to be of little value, but in many cases, preoperative diagnosis can be established with the aid of CT scan. In patients with urachal carcinoma, CT scan visualize the primary tumor and its extension beyond the bladder wall into the space of Retzius. Although about 150 cases of urachal tumor have been reported in the English literature, computerized tomographic findings have not been established. We present two cases and emphasize the usefulness of CT scan in preoperative diagnosis.

Eighteen patients were identified in whom ultrasound (US) of the kidney demonstrated a hyperechoic medulla. Diagnoses in the patients included gout in seven; Sjogren syndrome in two; medullary sponge kidney in two; primary aldosteronism in two; and Lesch-Nyhan syndrome, hyperparathyroidism, glycogen storage disease type XI, Wilson disease, and pseudo-Bartter syndrome in one each. The pathogenesis of the echogenicity was evaluated by comparing the findings from computed tomography and conventional radiography. It appears that a hyperechoic medulla is caused by hyperuricemia, medullary nephrocalcinosis, or hypokalemia. US is considered to be useful in evaluating renal involvement in patients with these diseases.

Olfactory neuroblastoma is a malignant, slow growing, neurogenic tumor that originates in the olfactory mucosa of the upper nasal cavity. We report a case of olfactory neuroblastoma that presented with exuberant hyperostosis of the facial bone during the course. This unusual radiological finding has been discussed with literature review.

Locally advanced bladder cancer (clinical stage T2-T4) of 11 patients and ureteral cancer (clinical stage T3) of 3 patients was treated concurrently by CDDP and irradiation. CDDP, total 200 mg, was administered 20 mg/day X5 day on the 1st week and 4th week combined with 2 GY/day irradiation, total doses 40 GY. CDDP was given on one or 2 hours after irradiation on the day. The results were significantly improved that the tumor could be controlled by TUR, and the pathological specimens showed marked tumor necrosis or degeneration. In 63.6% of 11 invasive bladder cancers, bladder function was preserved without any evidence of tumor recurrence or metastasis during 15-37 months. From these results, it is concluded that cis-radiation therapy may preserve the bladder with locally invasive cancer.