An aged case of hereditary xanthinuria with xanthine urinary calculi

A 74-year-old female with hereditary xanthinuria and xanthine stones is reported. She has a family history of consanguineous parents and a past history of right side nephrectomy due to a xanthine renal stone and vesicolithotomy of 3 bladder stones approximately 5 X 4 X 4 cm in size at the age of 58 and 71, respectively. Her young brother exhibited a slightly elevated urinary excretion of oxypurines. Laboratory examination showed a low serum level (0.3 mg/dl) and urinary excretion (1.56 mg/day) of uric acid, and high plasma and urine levels of oxypurines. No xanthine oxidase activity was detectably in duodenal mucosa by biopsy specimen obtained by duodenofiberscopy. Now she has another stone approximately 5 X 4 X 4 cm in her bladder. There have been are few elderly cases of hereditary xanthinuria with recurrent giant urolithiasis.     

Pharmacokinetics of carboplatin and etoposide in a haemodialysis patient with Merkel-cell carcinoma

We present a Merkel-cell carcinoma patient with chronic renal failure requiring haemodialysis and evaluate the pharmacokinetics of carboplatin and etoposide during haemodialysis. The area under the concentration-time curve of carboplatin was increased by prolonging the interval between administration and haemodialysis. However, that of etoposide was not changed. Carboplatin showed good membrane permeability in haemodialysis, while etoposide showed no permeability. In conclusion, the pharmacokinetics of carboplatin could be controlled by haemodialysis and the interval between chemotherapy and haemodialysis. However, the pharmacokinetics of etoposide were not affected.     

Rheumatoid arthritis in patients aged above 75 years old at onset]

Elderly onset rheumatoid arthritis (RA) is difficult to diagnose definitively when the patients note their first symptoms of arthritis above the age of 75 years old. In this report, we reviewed the clinical features of elderly onset RA and its diagnosis. The subjects included 4 females, aged 78, 83, 84 and 93 years, respectively. The onsets were abrupt in 2 cases and more slowly arriving in the other 2. Shoulder joints and wrist joints were involved in all cases. Knee joints, finger joints and foot joints were involved in 3 cases and the elbow was involved in one case. Anti-RA treatment quickly attenuated the acute and severe arthritis and brought down the high CRP level associated with vivid inflammatory activity of RA. The RAPA value was very high in all but one of the cases. Severe destructive findings in radiography was undetectable in the major joints (e.g. shoulder, hip and knee joints). Radiographic findings in wrist and finger joints were also very difficult to differentiate from arthrosis and osteoporosis. On the other hand, RA involvement in the cervical spine was certain in the radiographs; 1 case had anterior atlantoaxial subluxation and 2 cases had subaxial intervertebral erosion. Pathological radiographic findings in the cervical spine are useful for the diagnosis of elderly onset RA.     

A case of ureteritis cystica]

We report a case diagnosed as bilateral ureteritis cystica by ureteroscopic examination. A 51-year-old female was admitted with pyuria and proteinuria. Retrograde pyelography revealed multiple, small, smooth and round punched-out defects in the bilateral lower ureters. Ureteroscopic examination and punch biopsy were performed with a suspected diagnosis of ureteritis cystica. The histological examination revealed chronic ureteritis. This patient was given conservative treatment (chemotherapy) and careful follow-up.     

The value and limitation of resting 201Tl reinjection after stress-redistribution imaging for the assessment of myocardial viability: comparison with wall motion improvement by revascularization]

Clinical value and limitation of resting reinjection of small dose of thallium (37 MBq) for the assessment of myocardial viability were evaluated. The results were compared with the degree of wall motion improvement by revascularization to infarcted myocardium supplied by chronic total vessels in 12 patients with old myocardial infarction. Thallium uptake was visually scored and judged as normal, reversible defect (Group 1), new fill in after reinjection (Group 2A), and no fill in even after reinjection (Group 2B). Among 53 segments with initial perfusion abnormality, 21 segments reverted to almost normal, while 32 segments remained abnormal on redistribution images. New fill in after reinjection was observed in 11 segments of 32 segments showing persisting defect on stress and delayed image (37%). Wall motion score index of Group 2A improved significantly higher than Group 2B (p less than 0.01) and almost equal to Group 1, suggesting the utility of reinjection for the assessment of tissue viability which may be underestimated by conventional imaging. But significant wall motion improvement (greater than or equal to 0.6 mean SD/chords) was observed in 6 segments (29%) of 21 segments showing neither redistribution nor fill in after reinjection. These data indicate that small dose of thallium reinjection may enhance detection of viable but jeopardized myocardium, although some underestimation of viability remained to be resolved.     

X-ray evaluation of radial shortening for Kienb?ck's disease.

The effect of inclination of the distal radius to subsequent lunate collapse is controversial. Therefore we evaluated the postoperative x-ray course of 10 patients with stage II, III, or IV Kienb?ck's disease who were treated only with radial shortening and followed up for an average of 25 months. The three patients who became worse as determined by x-ray evaluation had lunate fossa inclinations of less than 12 degrees after surgery. The four patients who improved had lunate fossa inclinations greater than 12 degrees. Appropriate radial shortening with adjunct wedge osteotomy to increase lunate fossa inclination may prevent further lunate collapse.     

Successful Norwood operation for single left ventricle associated with severe subaortic stenosis, coarctation of aorta and hypoplastic aorta]

A 4 month-old boy who had double-inlet left ventricle, severe subaortic stenosis, hypoplastic ascending aorta and coarctation of the aorta revealed severe respiratory failure. An urgent Norwood operation was done. The procedure consisted of enlargement of the ascending aorta and main pulmonary artery and a systemic-pulmonary shunt using 5 mm PTFE tube. Postoperative course was uneventful.