Familial Juvenile Nephronophthisis in Two Siblings — Histological Findings at an Early Stage —

We present two female siblings with familial juvenile nephronophthisis (FJN) which was diagnosed at the early stage of renal failure. Diagnosis was made during the investigation of anemia in case 1 and by a subsequent family survey in case 2. Most patients with FJN are not identified until the terminal stage of renal failure and such cases have rarely been reported in Japan. Case 2 had a reduction in the maximum urinary concentration ability but no azotemia, and among the FJN patients reported in Japan so far she has the least advanced renal disease. Histological examination of the renal biopsy in case 1 showed typical findings of FJN, such as thickening and lamination of the tubular basement membrane (TBM), interstitial fibrosis, and round cell infiltration of the interstitium. In case 2, renal biopsy revealed an irregular marked thickening of the TBM with trivial interstitial changes and a normal glomerular appearance. The histology of these two cases suggests that the TBM may be the primary site affected in FJN.     

99mTc(V)-DMSA and99mTc-MDP uptake and no67Ga-citrate uptake in a case of primary pulmonary leiomyosarcoma

Tumor scintigraphy with 67Ga-citrate, 99mTc(V)-DMSA and 99mTc-MDP were performed on a patient with rare primary pulmonary leiomyosarcoma. While 67Ga-citrate accumulation to the tumor was not recognized, 99mTc(V)-DMSA and 99mTc-MDP scintigraphy showed relatively intense localization of the tracers in the lesion, and were very useful in suggesting the characteristics of the tumor.     

Ventriculoperitoneal shunt using a continuously variable-resistance valve for management of hydrocephalus, especially for cases mimicking simple brain atrophy]

Twenty-two hydrocephalic patients with a ventriculoperitoneal shunt using a continuously variable flow resistance valve (Orbis-Sigma valve system; Cordis Corporation, USA) were reviewed to discuss usefulness of the shunt system. We divided the cases into two groups according to the demonstration of the cortical sulci in computed tomography (CT) as follows; A: progressive hydrocephalic cases mimicking simple brain atrophy, B: acute or subacute hydrocephalic cases with evidence of increased intracranial pressure. Patients in Group A had begun to present slowly progressive loss of activity, dementia and gait disturbance of various degrees after a long quiescent period following primary central nervous system injury. CT findings of these cases showed they were mimicking simple brain atrophy. Although the period of their deterioration was very long, ranging from 1 month to nine years, clinical signs improved in all cases after the shunt. None showed complications. Group B cases consisted of those with signs of increased intracranial pressure such as consciousness disturbance, nausea and vomiting. These cases showed poor demonstration of the cortical sulci in CT. Eleven out of thirteen cases showed satisfactory clinical improvement, though the size of the ventricle showed a slight decrease following shunt procedure. Only one case showed unilateral subdural effusion. In conclusion, this shunt system is useful for the management of ventricular enlarged cases mimicking simple brain atrophy as well as acute hydrocephalic cases. Troublesome consequences such as subdural effusion and hemorrhage rarely occur even in cases with low cerebrospinal pressure.     

Body temperature monitoring in subjects with delayed sleep phase syndrome

To clarify the circadian aspects of delayed sleep phase syndrome (DSPS) in 4 patients with DSPS, we recorded polysomnograms and rectal temperature before and after chronotherapy. The time interval (2.7 h) between sleep onset and rectal temperature minimum before chronotherapy was shorter than the time interval after chronotherapy (5.3 h). Before chronotherapy, the period of rectal temperature rhythm was 24.7 h. After chronotherapy, the period of rectal temperature rhythm was 24.0 h. These findings lead to the conclusion that in DSPS there is a weakened mechanism of entrainment similar to that in non-24-hour sleep-wake syndrome.     

Comparison of anticonvulsant effects of valproic acid entrapped in positively and negatively charged liposomes in amygdaloid-kindled rats

Intraperitoneal injection of free valproic acid (VPA) suppressed amygdaloid-kindled seizure 1 h after injection in rats, but had no effect at 24 h. VPA entrapped in positively charged liposomes showed a prolonged anticonvulsant effect lasting for 2 days, while the effect evaluated at 1 h was not different from that with free VPA. VPA entrapped in negatively charged liposomes exerted a significantly stronger effect at 1 h than did free VPA, while it had no significant effect at 24 h. These results suggest that surface charges on liposomes play an important role in modifying the anticonvulsant effect of VPA.     

Prostatectomy by transurethral balloon Laserthermia™ (PROSTALASE™) in the canine

Prostatectomy by transurethral balloon Laserthermia (PROSTA-LASE?) was performed in a canine model. This balloon device monitored by transrectal ultrasound can cylindrically irradiate with a laser beam. The treatment was performed in 8 canines using 15 watts for 20 minutes at 60°C at a 5 mm depth of the prostate from the urethral surface. Immediately following the laser therapy, an area of coagulation necrosis was observed around the urethra to a depth of 4–5 mm from the surface. After 1 week, cavitation formation was seen in 3 or 4 canines by ultrasound, and the urethral reepithelialization was shown in 1 of 2 canines. After 2 weeks, cavitation formation was observed in both canines, and the urethra was completely reepithelialized in one canine. There was no tissue damage in the bladder neck of urethral sphincter and no urinary incontinence in any animal. This system is thought to be simple and, satisfactorily performed, is an effective transurethral prostatectomy. © 1994 Wiley-Liss, Inc.