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Mitochondrial respiratory chain disorders (MRCD) constitute a highly heterogeneous group both with regard to clinical manifestations and underlying genetic/biochemical defects. Management of urological complications such as vesicoureteral reflux (VUR) has not been discussed in cases with MRCD. We report a pediatric case of MRCD with recurrent urinary tract infections (UTI) due to primary and secondary VUR.A 6-month-old boy with multiple malformations and a history of aspiration pneumonia was referred to our department for febrile UTI (fUTI). Voiding cystourethrography showed high-grade left VUR and a normal bladder. As a spontaneous resolution of VUR was not observed, he underwent left ureteral reimplantation concomitant with left orchidopexy at the age of 1 year and 7 months. Although he had fUTI immediately after surgery, no recurrence of the UTI occurred after discharge. At the age of 3 years, he had septic shock and cardiac arrest caused by aspiration pneumonia, as well as, encephalopathy following cardiopulmonary resuscitation. At this event, the diagnosis of MRCD was achieved by liver biopsy. Since the age of 4 years, he has had repeated fUTI. VCUG confirmed the bladder deformity and right VUR. He underwent vesico-cutaneostomy at the age of 6 years. The postoperative course was uneventful. No recurrence of UTI was observed at the one-year follow-up.

A 60-year-old woman was not accompanied by any symptom. She had a gallstone which was identified 20 years prior. Ultrasonography performed by a local doctor revealed that the gallbladder was filled with small stones, and the patient was referred to our department for further examination and treatment for gallbladder stone. Tumor markers are elevated. Contrast- enhanced CT revealed gallbladder stones and thickening in the gallbladder body. PET-CT showed abnormal accumulation of FDG-PET with SUVmax 3.6 in the body of the gallbladder. With a diagnosis of gallbladder cancer, extended cholecystectomy and gallbladder bed resection with regional lymph node dissection were performed. The tumor was diagnosed histologically as small cell type neuroendocrine carcinoma of the gallbladder(pT2a[SS], pN0, pStage ⅡA; Japanese society of hepato-biliary-pancreatic surgery, the 7th edition). The postoperative course was uneventful. This patient has been followed up for 8 years without obvious signs of recurrence. R0 resection and lack of lymph node metastasis can allow long- term survival.

(Objective) The etiology of acute epididymitis in children remains poorly understood. Several studies have demonstrated that urine tests are negative in the majority of children with acute epididymitis, and the condition is self-limiting. The need for radiological evaluation of the urinary tract in children with acute epididymitis is still debatable. The aim of this study was to describe clinical and imaging findings in children with acute epididymitis. (Methods) We identified 47 children with acute epididymitis at our institute between 2017 and 2021.We retrospectively reviewed their clinical features and radiological and laboratory data. All children underwent ultrasonography of the kidney and urinary tract. (Results) Median patient age was 9 years (range, 6 months-16 years) and 60% of the cases occurred between the ages of 7 and 12 years. Thirteen children (28%) had a past history of genitourinary malformations. The common malformations were hypospadias in eight children and bladder dysfunction in three. Ultrasound revealed no new urinary tract abnormalities in the remaining 34 children. Urinalysis were performed in 27 children, nine of whom (33%) had pyuria. Urine culture was positive in two children. Of the nine children with genitourinary malformations, eight had pyuria. All 18 children without genitourinary malformations had a negative urinalysis except for one patient (p< 0.0001). (Conclusions) Acute epididymitis is a common cause of acute scrotum in pediatric patients. In this study, one-third of acute epididymitis cases presented pyuria, and about 30% had a past history of genitourinary malformations. The presence of pyuria was associated with a past history of genitourinary malformations. For children with no previous genitourinary malformations, routine use of ultrasound for the detection of urinary tract abnormalities is questionable due to the low yield.

A 9-month-old boy, having a history of cardiac surgery in neonatal period, was referred to our department for evaluation of genital anomalies. The antenatal course was uneventful, except for unknown gender. His family history was unremarkable. He was delivered at full term, and his birth weight was 3,510 g. Physical examination revealed proximal hypospadias and a non-palpable testis on the left side. Chromosome studies showed a normal male karyotype with positive SRY. At the age of 14 months, he underwent hypospadias repair. Three months later, left testicular exploration was performed along with orchidopexy of an ascending testis on the contralateral side. As nothing was found through an inguinal incision on the left side, laparoscopy was indicated. Laparoscopic observation revealed a small dark reddish mass cranially connected to the left hypoplastic testis that was located high in the left iliac fossa. The epididymis and vas deference looked abnormal, and detachment to the testis was apparent. Testicular vessels were undifferentiated from the mass. Therefore, the left testis was excised with the mass. Histopathological examination confirmed the testis and spleen tissue, and the diagnosis of splenogonadal fusion was made postoperatively.

Localized cystic disease of the kidney (LCDK) is a rare, non-hereditary, non-progressive benign cystic renal condition. It is found primarily in adults, and is rarely reported in children. To date, only 5 prepubertal cases of LCDK have been reported in the literature. In this report, we present a case of LCDK that was difficult to differentiate from malignant renal tumor in a 6-year-old girl. Nephron-sparing surgery could not be performed.A 6-year-old girl with no past medical history presented with gross hematuria and right-sided abdominal pain. An abdominal ultrasound at a local hospital showed multiple variable-sized cysts throughout the right kidney. She was referred to our hospital for further evaluation. Computed tomography demonstrated that the region between cysts was slightly enhanced in some part of the lower pole and was diagnosed III in the Bosniak classification. Nephrectomy was performed for possible cystic renal neoplasm. After surgery, a diagnosis of LCDK was made by histopathological examination.

Stent encrustation is one of the most serious complications of using double J stents. The management of encrusted stent is challenging, especially in children. To date, only two pediatric cases of stent encrustation, at the expected timing of removal after pyeloplasty, have been reported. In this report, we present a case of ureteral stent encrustation after laparoscopic retroperitoneal pyeloplasty in an 11-year-old girl. She underwent dismembered pyeloplasty for pelvi-ureteric junction obstruction on the left side using a double J stent (6 F, 24 cm, Polaris ™ Ultra). Postoperative course was uneventful, except for mild bladder irritability and asymptomatic pyuria. Nine weeks later, an attempt to remove the stent was made under general anesthesia. This attempt was unsuccessful as the renal coil got stuck in the anastomotic position. A retrograde 6 F flexible ureteroscopy revealed an encrusted stent with calculi. After transurethral lithotripsy with a holmium: yttrium aluminum garnet (YAG) laser, the stent was extracted. Stone analysis showed struvite. At 8-months follow-up, she was stone-free and doing well with resolution of hydronephrosis.

Since 2011, endoscopic correction of vesicoureteral reflux using dextranomer-hyaluronic acid copolymer (Deflux ® ) has been widely accepted in Japan due to its safety and minimally invasive nature. However, long-term complications are unknown. We present a case of Deflux ® implants calcification mimicking distal ureteral calculi in a 12-year-old boy with a history of Deflux ® injection performed at three years of age for primary VUR. We should be aware of this complication to avoid misdiagnosis and unnecessary invasive examination such as radiological imaging or endoscopy.

Fibrous hamartoma of infancy (FHI) is a rare, benign soft tissue tumor arising from subcutaneous tissue in children during the first two years of life. The tumor is commonly found in the axilla, shoulder and upper arm. Paratesticular FHI is extremely rare. To date, only a case of paratesticular FHI has been reported in Japan. We present a case of paratesticular FHI in an 11-month-old boy who eventually needed orchiectomy due to local recurrence one month after the excision of the tumor. Ten months postoperatively, there was no sign of recurrence.

A 59-year-old man presented with epigastralgia. A diagnosis of advanced gastric cancer MLU, Circ, Type 3, 160 mm, tub2, cT4b (SI: panc), cN1, cM0, cH0, cP0, cCY0, cStage ⅢB was made. Because of difficulty with oral intake due to malignant outlet obstruction and tumor bleeding, endoscopic self-expanding metallic stent placement was performed. We administered chemotherapy involving docetaxel, cisplatin, and S-1(DCS). After 2 courses of chemotherapy, the primary lesion and regional lymph nodes had reduced in size. His response was judged as SD according to the RECIST criteria. The patient elected to undergo explorative laparotomy for assessment of the gastric cancer. The intraoperative findings showed that there was no pancreatic invasion, peritoneal dissemination, or distal metastasis, so a total gastrectomy and D2 lymph node dissection was performed. The pathological findings showed that there were very few cancer cells in the primary lesion, and a lymph node metastasis was found. The final stage was gastric cancer MLU, Circ, Type 3, 100 mm, muc, ypT4a(SE), ypN3a (13/51), ypM0, ypH0, ypP0, ypCY0, ypStage ⅢC. The therapy evaluation was Grade 1b. In summary, we encountered a patient with gastric cancer in whom curative surgery was made possible by undergoing chemotherapy and metallic stent placement.

A 67-year-old man visited an urological clinic with a chief complaint of urination pain. Cystourethroscopy and magnetic resonance imaging (MRI) examination revealed a bladder tumor (cT3bN0M0). Marked leukocytosis and respiratory distress with pleural effusion appeared. Pulse steroid therapy improved the general condition partially. The patient was sent to our hospital for further examination. Serum granulocyte colony-stimulating factor (G-CSF) and interleukin-6 (IL-6) were high and the pathological findings of bladder tumor obtained by transurethral resection (TUR) revealed an urothelial carcinoma that produced G-CSF and IL-6. Neoadjuvant systemic chemotherapy was performed along with use of steroid and sivelestat, which ameliorated the respiratory distress. After three courses of systemic chemotherapy, serum G-CSF and IL-6 normalized and cystoprostatectomy was performed. The patient has been in good health at 20 months after the surgery with no evidence of recurrence.

We investigated the effectiveness of prophylactic FOLFOX after curative resection of synchronous metastases in patients with colorectal cancer (CRC). Clinicopathological information including postoperative chemotherapy, such as a therapeutic regimen, relapse-free survival (RFS), site of recurrence, etc., was retrospectively analyzed in 116 CRC patients with synchronous distant metastases, and 63 patients with metachronous metastases who had received surgery in our hospital between 2000 and 2009. Fifty-three patients (84%) out of 63 without adjuvant chemotherapy, and 38 (83%) out of 46 patients that received oral or intravenous 5-fluorouracil (5-FU) (alone or with leucovorin (LV)or isovorin) developed recurrent tumor(s) afterwards. The median RFSs were 119 and 281 days, respectively. By contrast, a single patient among 6 who underwent FOLFOX (up to 12 therapeutic courses) showed recurrence 476 days after surgery. The RFS of the FOLFOX was significantly higher than that of the 5-FU (+LV) or surgery alone (p=0. 03, p=0. 007, respectively). In conclusion, the FOLFOX regimen is more beneficial for CRC patients with synchronous metastasis as adjuvant chemotherapy than 5-FU (+LV) or other followup strategies.

Late side effects of ileal conduit are uncommon. Here we report a case of ileal conduit hemorrhage in a 78-year-old woman 8 years after radical cystectomy and ileal conduit diversion. The patient presented with gross hematuria and abdominal dynamic computed tomography showed extravasation of contrasts in ileal conduit and the patient was diagnosed with ileal conduit hemorrhage. Clipping hemostasis was performed under gastrointestinal endoscope and revealed that Dieulafoy's ulcer was the cause of ileal conduit hemorrhage. This is the first case of Dieulafoy's ulcer occurred in ileal conduit. Hemorrhage from ileal conduit is an important late side effect.

28-year-old female received dialysis treatment due to chronic renal failure caused by polycystic kidney disease. Later, she underwent a laparoscopic splenectomy and ABO incompatible living kidney transplantation successfully following bilateral renal contraction therapy with renal transcatheter arterial embolization (renal TAE). A unilateral or bilateral native nephrectomy of a massively enlarged kidney performed at the time of renal transplantation is a common treatment in polycystic kidney patients scheduled for transplantation. On the other hand, when treated with renal TAE, such patients can avoid a laparotomy, which provides several advantages when undergoing peritoneal dialysis in the future or a laparoscopic splenectomy prior to ABO incompatible kidney transplantation. Furthermore, we consider that bilateral renal TAE is necessary for polycystic kidney patients prior to renal transplantation for a variety of reasons, including problems associated with contrast nephropathy if renal TAE for left kidney is remained after renal transplantation.

The research performed at the Department of Urology Osaka University Graduate School of Medicine is overviewed. Renal ischemia-reperfusion (I/R) injury is inevitable in transplantation and is related to longterm graft function. MF-1, a bifunctional hepatocyte growth factor-macrophage stimulating protein chimera, was found to prevent apoptosis. In our study, MF-1 directly guarded cultured proximal tubular epithelial cells from hypoxia-induced necrosis and apoptosis in vitro, and MF-1 treatment ameliorated renal dysfunction by preventing apoptosis in rat I/R injury model. The erythropoietin molecule modified by carbamylation (CEPO) has been identified and was demonstrated to protect several organs without increasing the hemoglobin concentration. The therapeutic effect of CEPO was evaluated using an endothelial tube formation assay, and a rat ischemia-reperfusion injury model. CEPO treatment induced more capillarylike formation than EPO. CEPO-treated kidneys showed minimal tubular apoptosis with increased peritubular capillary endothelial cells. We identified a new therapeutic approach using CEPO to protect the kidney from ischemia-reperfusion injury by promoting angiogenesis.

A retrospective clinicopathological study of 82 patients with invasive urothelial carcinoma treated with radical cystectomy was performed. There were 62 men and 20 women, median age at operation was 68-years-old and the 5-year overall cancer-specific survival rate was 82.5%. Univariate analyses demonstrated that female patients had poor prognosis, and that lymphovascular invasion was a predictor of recurrence. Multivariate Cox regression analyses including age, grade, and vascular invasion, showed that vascular invasion was an independent predictor of recurrence (HR4.46, P=0.008).

A 52-year-old male presented with left intermittent abdominal pain, and was subsequently diagnosed with a tumor in the hilum of the left kidney based on computed tomography (CT) and magnetic resonance imaging (MRI) findings. Under suspicion of left renal cancer, we performed a retroperitoneoscopic left nephrectomy. Histopathological features of the resected specimen were compatible with leiomyosarcoma originating from the left renal vein. Immunohistologically, the tumor cells were spindle-shaped, arranged in bundles, and stained positive for a-smooth muscle actin and desmin. The patient was free from recurrence 2 years after surgery. The prognosis of leiomyosarcoma arising from the renal vein has been considered poor. Herein, we provide details of our case and also review 16 cases of leiomyosarcoma of the renal vein in Japan. We conclude that radical tumor resection is necessary for long-term survial.

We report a case of giant left renal arteriovenous fistula (AVF). A 36-year-old man was diagnosed with chronic glomerulonephritis (CGN) by biopsy on the left kidney 11 years ago. He had been receiving hemodialysis for end-stage kidney disease from CGN since 10 years ago. A left renal cystic lesion was found and he was referred to our department for examination and treatment. He was diagnosed as having left AVF using imaging techniques (computed tomography, magnetic resonance imaging and Color Doppler ultra sonography). He underwent embolization of left renal artery using microcoils. After the surgery, there were no major complications, and there were no signs reccurence. AVF in a long-term dialysis patient is rare. We report this case and summarize the cases reported in Japan.

A78-year-old female was admitted to our hospital with anorexia due to ascites. We were not able to diagnose by cytological diagnosis. For a definite diagnosis she underwent an open biopsy. Histological findings revealed observable papillary adenocarcinoma with psammoma body, carcinoma cells were positive for Ber-EP4 on immunostaining, and ovaries were normal. Thus, we made a diagnosis of peritoneal serous papillary adenocarcinoma. She was treated by intraperitoneal administration of CDDP(20 mg/day: day 1, every four weeks). Ascites vanished and CA125 was within the normal range. There was no recurrence after 3 years, but Virchow's lymph node metastasis occurred. The patient is still alive 12 months after metastasis with CDDP intraperitoneal administration.

We analyzed 95 patients with renal pelvic and ureteral cancers with total nephroureterectomy at our Department fromJanuary, 1997 to April, 2007. The patients were composed of 60 males and 35 females, between 41- and 90-years-old with a median age of 69-years-old. Histologically, there were 94 transitional cell carcinomas (TCC) and 1 TCC with squamous cell carcinoma. Fifty five cases (57.9%) were less than pT2, and 41 cases (42.1%) were more than pT3. Findings of lymphatic and venous invasion were present in 35 cases and 24 cases respectively. The overall survival rate at 5 years was 76.6%. In this series, pathological stage, infiltration pattern, lymphatic and/or venous invasion and the regional lymph nodes indicated a significantly poor prognosis. On the other hand, multivariate analysis using Cox proportional hazards regression revealed the presence of pathological stage as the most significant predictor of survival. Furthermore, we investigated histopathological prognostic factors of the patients separating asymptomatic macrohematuria group (AM group) and non-asymptomatic macrohematuria group (non-AM group). According to this analysis, non-AM group had worse pathological stage, grade and lymphatic invasion than AM group.

A-46-year-old male with advanced-stage IV multicentric gastric cancer was treated with S-1/CDDP as neoadjuvant chemotherapy. S-1 (initially 100 mg/day, up to 120 mg/day) was orally administered for 3 weeks (day 1-21) followed by 1 drug-free week as a course, and CDDP (initially 60 mg/day, up to 100 mg/day) was administered by intravenous drip on day 8. After the fourth course, a significant tumor reduction was obtained and curative surgery was performed. Thereafter, S-1 therapy was continued. There has not been any recurrence for 19 months postoperatively.

A 21-year-old man with bilateral hypoplastic kidneys underwent laparoscopic splenectomy and ABO-incompatible living renal transplantation. After the splenectomy, pancreatic leak occurred and it caused intra-abdominal bleeding on the 10th and 16th postoperative day, so emergency operations were performed. Then the graft function had become stable and he was discharged on the 75th postoperative day. Although intra-abdominal bleeding due to pancreatic leak often causes a fatal condition, he was successfully treated by suppression of pancreatic external secretion using octreotide and appropriate drainage.

We report a case of renal cell carcinoma in which interferon-a therapy was effective in reducing the tumor thrombus extending into the inferior vena cava. A 66-year-old man was referred to our hospital with a complaint of macroscopic hematuria, cough and general fatigue. We made a diagnosis of a left renal cell carcinoma with tumor thrombus by imaging examination. Because his performance status was 3, a radical operation was considered risky. Twenty-two months after the start of interferon-a therapy, the tumor thrombus was markedly reduced in size, and the clinical response was evaluated as partial response by the response criteria for urological cancer treatrment. Because of improvement of the performance status and downsizing of tumor thrombus, we performed radical nephrectomy. Pathological examinations revealed that viable renal cell carcinomas were found in the primary lesion and the tumor thrombus. In some cases, interferon-alpha therapy is useful and safe in the treatment of the tumor thrombus. Furthermore, radical nephrectomy and complete resection of the tumor thrombus prolongs postoperative survival.

Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the bladder is a rare disease, and the most effective therapeutic procedure remains unknown. We report a case of primary MALT lymphoma of the bladder regressed after rituximab in combination with CHOP chemotherapy (R-CHOP). The patient, a 84-year-old woman, presented with general fatigue and loss of weight. Computed tomography (CT) showed a solitary mass at the bladder. Histological diagnosis of the tissue obtained by transurethral biopsy was extranodal marginal zone B cell lymphoma of MALT. The patient was in complete remission after only one cycle of R-CHOP. This is the fourteenth case of MALT lymphoma of the bladder in Japan.

We report the diagnosis and treatment for carcinoma in situ of the bilateral upper urinary tracts after total cystectomy and ileal conduit by intentionally inducing hydronephrosis. A 75-year-old man whose chief complaints were macroscopic hematuria and bladder irritabilities was diagnosed carcinoma in situ of the bladder and underwent total cystectomy and ileal conduit. 26 months after the operation, the cytological examination of ileal conduit urine revealed urothelial carcinoma. Since the radiographic findings in the upper urinary tracts and ileal conduit were negative, we constructed bilateral percutaneous nephrostomies by intentionally inducing hydronephrosis. Since two serial cytological examinations of the urine sampling from the bilateral pyeloureteral systems revealed urothelial carcinoma, we performed the percutaneous Bacillus Calmatte-Guerin perfusion of the bilateral upper urinary tracts. The therapy was repeated at weekly intervals for a total of 11 perfusions and the cytological examination of ileal conduit urine became negative 4 months after the beginning of the therapy. Intentionally induced hydronephrosis is useful for the diagnosis and treatment for carcinoma in situ of the bilateral upper urinary tracts after total cystectomy and ileal conduit.

Soft tissue sarcomas (STS) are rare tumors that comprise only 1% of all malignancies. The retroperitoneum is an uncommon site of origin for them, accounting for about 10% of all STS. In case 1, a 30-year-old man complaining of chest pain was found to have a retroperitoneal tumor around the left kidney by computed tomography (CT). The tumor was removed along with the left kidney. Histologically, it was fibrosarcoma 3,000 g in weight. In case 2, a 31-year-old woman complaining of abdominal distension was found to have a retroperitoneal tumor by CT. The tumor was removed in the same way. It was undifferentiated sarcoma 2,000 g in weight. In case 3, a 73-year-old woman, who had no complaint was found to have mass in the left lateral region during follow up for cardiac failure. She was referred to our department for treatment of retroperitoneal tumor revealed by ultrasound and CT. The tumor was dedifferentiated liposarcoma 2,420 g in weight. All three patients had local recurrences, and two of them underwent a second surgical procedure.

Sarcomas and related proliferative lesions of specialized prostatic stroma are rare. Lesions have been classified into prostatic stromal tumor of uncertain malignant potential (P-STUMP) and prostatic stromal sarcoma based on the degree of stromal cellularity, presence of mitotic figures, necrosis, and stromal overgrowth. STUMPs are considered neoplastic, based on the observations that they may diffusely infiltrate the prostate gland and extend into adjacent tissues, and often recur. Although most cases of STUMPs do not behave in an aggressive fashion, occasional cases have been documented to recur rapidly after resection and a minority have progressed to stromal sarcoma. Here we describe a case of P-STUMP. A 57-year-old male went to his family doctor because of pollakisuria. Digital examination revealed abnormal findings in the prostate, then he was referred to our medical center. The mass was palpable in the left lobe of the prostate; it was elastic hard, surface smooth, about 2 cm in diameter. Serum PSA was elevated slightly (5.42 mg /dl). We diagnosed firstly leiomyosarcoma by transrectal ultra sound guided needle biopsy of the prostate. Then we performed radical prostatectomy. Finally we made the pathological diagnosis of P-STUMP. After 11 months, there is no sign of metastasis or recurrence.

Malignant metastasis along the needle tract, called "needle tract implantation" (NTI), has been demonstrated for numerous carcinomas, including prostate, colon and liver. We report a case of NTI of ureteral carcinoma following percutaneous nephrostomy tube replacement. A 57-year-old woman was found to have a subcutaneous mass at the site of nephrostomy. She had a past history of right nephroureterectomy for ureteral carcinoma about one year earlier, and a nephrostomy tube had been inserted before diagnosis postoperatively. We resected the subcutaneous mass. Pathological diagnosis was urothelial carcinoma closely resembling right ureteral carcinoma. To our knowledge, this is the seventh reported case of NTI of upper urinary tract urothelial carcinoma. Percutaneous manipulation carries a potential risk of local tumor spillage and NTI in the nephrostomy tract.

The "lifestyle-related disease" has been increasing in Japan as the population advances in age and the food culture becomes westernized. Although prevention, treatment and therapy for this disease have been attempted using certain kinds of food and nutritive elements, so-called "health foods" such as DHA and EPA, which are mostly contained in fish oil, have been a special focus within these attempts. There have been many reports regarding the pharmacological functions and the mechanisms of DHA and EPA. Also, in the past few years, it has become possible to produce ingestible DHA and EPA oils, oils for chemical compounds, oils for animal feed, and highly purified DHA and EPA for medical and pharmaceutical use. EPA ethyl ester has a wide market as a preventive medicine in Japan. Initially in 1990, this medicine was administered in cases of arterisclerosis obliterans, using its anti-platelet aggregation ability. Four years later, in 1994, its effectiveness in triglyceride reduction was recognized, and its application was extended to cases of hyperlipidemia, which has remarkably broadened its market. Clinical studies with DHA have shown improvement in senile dementia (cerebral thrombosis, Alzheimer's disease), atopic dermatitis, and the ability to focus on moving objects, as well as control of aggressiveness against others caused by stress, and prevention of hyperlipidemia, hypertension, and cancer.

We present a case of 29-year-old female who underwent an ABO-incompatible living kidney transplantation from her father. The serum creatinine (s-Cr) level of this patient was stabilized about 1.1-1.2 mg/dl during the first 3 months after the transplantation. Thereafter, the function of allograft was deteriorated gradually. A biopsy performed on post-transplant day (PTD) 520 to evaluate a rise in creatinine revealed an interstitial nephritis and chronic renal allograft nephropathy. The renal function worsened persistently, although we increased the dosage of immunosuppressant subsequently. The following biopsy performed on PTD 630 showed a suspicion of BK virus nephropathy, with a mass of tubular epithelial nuclear inclusions and an interstitial nephritis. The diagnosis of BK virus nephropathy was confirmed on the immunohistochemistry staining using anti-SV40 antibody and PCR analysis. Despite reducing the immunosuppressants, the function of the allograft worsened progressively and was lost on PTD 912.

There are several lines of evidence indicating that membrane AA correlates with the ability of aged rats to sustain LTP. The age-related decrease in membrane AA seems to be triggered by increased lipid peroxidation, which is involved with the decline of LTP. The chronic treatment of DHA could decrease membrane AA without an increase in lipid peroxidation. We have thus investigated the effect of chronic treatment of DHA on hippocampal LTP to assess whether the decrease in membrane AA could directly affect the induction of LTP. The effects of daily supplementation of DHA for 3 months on membrane AA, LTP, and Ca2+ response were evaluated using hippocampal slices from 26-month-old Wistar rats. Chronic treatment of DHA reduced the hippocampal AA significantly, but did not change the amplitude of LTP. Neither 30 mM K+ nor 500 microM NMDA-induced Ca2+ response was affected by chronic treatment of DHA, while the 500 microM carbachol-induced Ca2+ response was reduced. From these results, the reduction of membrane AA might suppress the carbachol-induced Ca2+ response by inhibiting the muscarinic receptor function, IP3 formation and/or Ca2+ release from Ca2+ stores by IP3. However, the reduction of membrane AA is not likely to be a main causal factor of the decline of LTP.

Background: We report on a 3 year-old boy who was first diagnosed with retinal detachment and macular hole and received surgical treatment. X-linked juvenile retinoschisis was determined by DNA analysis., Case: Past or family history was not recognized. There was left macular hole but no typical spoke-like foveal retinoschisis was observed in either eye. We could not diagnose the case as X-linked juvenile retinoschisis because there was no family history of it, central foveal reflex was observed in right eye with corrected visual acuity of 1.2, and no abnormality was recorded in the electroretinogram. High molecular weight DNA was extracted from peripheral leukocytes, and the XLRS 1 gene was analyzed. Hemizygous missense mutation, Arg102Gln, was detected. We diagnosed the disease as X-linked juvenile retinoschisis because the Arg102Gln mutation was detected in a family in Germany, two families in the United Kingdom, and two families in the USA., Conclusion: XLRS 1 gene analysis is useful if the diagnosis is difficult clinically due to atypical clinical findings.

Previously, we reported the short-term immunological effects of postoperative adjuvant interferon-gamma (IFN-gamma) administration to renal cell carcinoma patients as determined by three-color flow cytometry. We now report the results of a long-term study on a larger number of subjects. Thirty-three patients with renal cell carcinoma received a prophylactic intramuscular injection of IFN-gamma (300 x 10(4) units per week) after nephrectomy. We evaluated immunological changes by measuring peripheral blood lymphocyte subsets including activated cytotoxic T lymphocytes (ACTL), cytotoxic T lymphocytes (CTL), activated suppressor T lymphocytes (ASTL), helper T lymphocytes (HTL), activated suppressor-inducer T lymphocytes (AITL), and suppressor-inducer T lymphocytes (SITL). We also estimated the natural killer (NK) activity by a cytolytic test. All 33 patients were examined for at least 12 months after the start of IFN-gamma injection, and 18 patients were examined for 30 months including the 6-month period following discontinuation of IFN-gamma injection. We found significant enhancement of the ACTL subset from the second week to the sixth month after the start of IFN-gamma injection. On the other hand, we found a significant decrease in the percentage of the HTL and SITL subsets for a long time after the start of injection. NK activity significantly increased throughout the period of administration, and it continued to increase for six months after discontinuation of IFN-gamma injection.

Fifty-five colorectal cancer patients who had continuous intraportal chemotherapy between 1990 and 1993 (treated group) and 130 colorectal cancer patients who did not have portal chemotherapy between 1982 and 1993 (untreated group) were studied to clarify the effects of continuous intraportal chemotherapy on the prognosis. After a catheter was placed in the portal vein through the right gastroepiploic vein at the time of radical operation, 10 mg of MMC was continuously infused for 4 hours at operation and 500 mg/day of 5-FU was continuously infused for 7 days postoperatively. The toxicities of this therapy were not serious. The five-year survival rate was 65.3% in the treated group and 65.6% in the untreated group. The five-year disease-free survival rate was 69.8% in the treated group and 58.6% in the untreated group, with no significant difference. In stage II patients, however, the five-year disease-free survival rate in the treated group was slightly higher than in the untreated group (90.0% vs 70.3%, p = 0.073), and the rate of hepatic recurrence in the treated group was significantly lower than in the untreated group. These results suggest that continuous intraportal chemotherapy may prevent metachronous hepatic metastases in stage II colorectal cancer patients.

The susceptibilities of clinical isolates of Candida spp. to fluconazole (FLCZ), itraconazole (ITCZ) and ketoconazole (KCZ) were determined by a new method, E-test (AB BIODISK, Sweden) and compared with those obtained by the microbroth dilution method. The MIC values of the 3 azoles obtained by the two methods were, in general, in good agreement. While MICs of FLCZ and KCZ demonstrated higher essential agreement rates and better correlation by both methods, MIC values of ITCZ by the E-test had a tendency to be higher than those of the microbroth dilution method in azole resistant strains. Potential usability of E-test as a broth dilution method for susceptibility tests for clinically isolated Candida spp. including azole resistant strains was discussed.

The patient was a 67-year-old male. Preoperative DSA study revealed a right subclavian aneurysm, a brachiocephalic aneurysm, stenosis of the left common carotid artery and the left subclavian artery, and occlusion of the left vertebral artery. An intraoperative occlusion test of the right vertebral artery showed a decline of the brainstem auditory evoked responses (BAER) seven minutes after the start of the procedure. Cardiopulmonary bypass was established by femoral arterial and bicaval cannulation. Under selective cerebral perfusion by cannulation of both common caroid arteries, a partial aortic arch replacement was performed using a graft patch with 3 branches. These three branches were connected to the right subclavian artery and to both common carotid arteries, respectively. Under deep hypothermia the right vertebral artery was reconstructed with the saphenous vein graft. Proximal anastomosis between the saphenous vein graft and the right caroid graft was done perfusion from a branch of the saphenous vein graft in order to shorten ischemic time of basilar arterial region. The patient was weaned from bypass without problems. After weaning the BAER test was again performed and found to be normal. The postoperative course was uneventful. Postoperative DSA examination showed good blood flow in all the reconstructed vessels. This study suggests that deep hypothermia and shortening of ischemic time of the basilar arterial region are effective in preventing ischemic injury of the brain stem and the cerebellum.

A 62-year-old man visited our hospital with asymptomatic gross hematuria. On cystoscopy, two diverticula were identified at the dome of the urinary bladder and one of them was packed with a tumor. Biopsy showed squamous cell carcinoma (SCC), G1, but malignancy was not detected in the rest of the vesical mucosa. We selected bladder-preserving therapy. Pathological diagnosis after partial cystectomy was SCC, G1 > G2, pT3b. Postoperatively, 3 courses of adjuvant chemotherapy with methotrexate, bleomycin and cisplatin were performed. The patient has remained free of recurrence for 4.5 years.

Acute necrotizing encephalopathy (ANE) of childhood is a newly proposed disease entity characterized by symmetrically distributed necrotic brain lesions in the thalamus, cerebral white matter, brainstem, and cerebellum. We report a 4-year-old girl with severe psychomotor delay and horizontal gaze palsy as sequelae of ANE at 17 months of age. The computed tomography showed bilaterally symmetrical low density areas in the thalamus and low density areas in the left middle cerebral artery (MCA) region and the posterior cerebral artery (PCA) region. MRI (T 1-weighted) revealed unevenly distributed small low signal intensity areas with scattered high intensity regions in the thalamus bilaterally. The T2-weighted images showed multiple small low intensity areas around high intensity areas, and low signal intensity areas in the left middle cerebral artery (MCA) region and the posterior cerebral artery (PCA) region. In addition to severe psychomotor delay, the patient exhibited a peculiar eye movements. Horizontal ocular movement was impaired, but vertical ocular movement was almost completely normal. As clearly shown by MRI of the brain, the pontine tegmentum, including bilateral abducens nucleus, paramedian pontine reticular formation (PPRF), medial longitudinal fasciculus (MLF), and the facial nerve were affected, but the thalamo-mesencephalic junction, including the rostral interstitial medial longitudinal fasciculus (riMLF) and the nucleus of Cajal, was spared. To our knowledge, this is the first case of ANE associated with this selective ocular movement disorder ever reported. Because of the multiple symmetrical lesions and pons and the asymmetrical lesions of the MCA and PCA regions in the present case, occlusion of a single vessel could not account for the pathology. The pathophysiological mechanism of ANE is unknown. We postulate that some toxic or vasoactive agent caused vasospasm and subsequent breakdown of the blood-brain barrier, especially in the thalamus and pons, resulting in the unique distribution of the lesions and the rare eye movement disorder observed in the present case.

We report the 5th case of renal cell carcinoma associated with renal artery aneurysm in the Japanese literature. A 57-year-old woman was admitted to our hospital for further examination of a right renal mass. Computerized tomography and magnetic resonance imaging revealed a 6 x 5 cm tumor at the upper pole of the right kidney and an aneurysm of the right renal artery. An angiography showed duplicated renal arteries and a saccular aneurysm 2.1 x 2.5 cm occurring at the first bifurcation of one renal artery. A transperitoneal radical nephrectomy was performed.

A 69-year-old man presented for further examination for left renal mass. Computed tomography and magnetic resonance imaging revealed a heterogenous mass on the left side of a horseshoe kidney accompanied with renal vein thrombus. Aortography showed a hypervascular mass in the left kidney and aberrant arteries to the isthmus originating from the aorta. A left nephrectomy with the division of the isthmus was performed through an abdominal transperitoneal approach. Histological evaluation revealed grade 2, stage T3b, renal cell carcinoma. The patient was free of tumor, but died of intracerebral hemorrhage 5 months after the operation.

Cardiac performance in 54 patients with total anomalous pulmonary venous connection was investigated by cardiac catheterization before and after surgery. 51 patients underwent intracardiac repair, and 17 of them died during or immediately after operation. According to the preoperative study, the left ventricular ejection fraction (LVEF) of surviving patients was significantly higher than that of patients who died, and the pulmonary arterial mean pressure of surviving patients was significantly lower than that of patients who died. However, there was no significant difference between the left ventricular end-diastolic volume (LVEDV), right ventricular ejection fraction (RVEF), and right ventricular end-diastolic volume (RVEDV) in surviving patients and those who died. Post-operative catheterization studies showed significant increases of LVEF and LVEDV compared to pre-operative figures. RVEF and RVEDV and pulmonary arterial mean pressure decreased significantly after surgery. It was concluded that preoperative cardiac performance of surviving patients was better than that of those who died, and post-operative cardiac performance of surviving patients was basically normal.

We evaluated the validity of body surface mapping (MAP) in assessing noninvasively the degree of myocardial ischemia in patients with significant coronary arterial stenosis following Kawasaki disease. Delay of ventricular depolarization was examined by a departure map (DM) using mean QRS map, and the sensitivity of this method in detecting myocardial ischemia was evaluated based on the findings of coronary arteriography (CAG). The other noninvasive measures were also evaluated. MAP was obtained in 29 patients with significant coronary arterial stenotic lesions, including coronary occlusion, segmental stenosis and localized stenosis of 75% or greater. Mean QRS map was obtained based on MAPs in 41 children without organic heart disease and in 22 patients with significant stenotic lesions. The departure index (DIi) was calculated by subtracting potentials at each lead from those of the mean QRS maps and divided by the standard deviation. Departure area (Da) was defined as an area with DIi of -2 or less. Each MAP was subdivided into nine sections, and Da greater than or equal to one-eighth of the anterior wall section, Da greater than or equal to one-third of the posterior and inferior wall sections or Da greater than or equal to half of the other sections were regarded as ischemic areas. Sensitivity in detecting ischemia by MAP was assessed by the CAG findings, which was also compared to the sensitivity of the electrocardiograms (ECG), ECG with dipyridamole administration (Dp-ECG), vectorcardiograms (VCG), Holter ECG (Holter), treadmill test (TM), Master's double step test (MD), two-dimensional echocardiography (2DE) and thallium myocardial imaging (TMI).(ABSTRACT TRUNCATED AT 250 WORDS)

In vitro antibacterial activity of 30 beta-lactam antibiotics including 2 beta-lactamase inhibitors (clavulanic acid and sulbactam) against 2 major pathogenic Nocardia, i.e. Nocardia asteroides group and Nocardia brasiliensis was studied. Among the antibiotics tested, a newly developed carbapenem antibiotic, imipenem (IPM), was found to be the most active, followed by oxacephem group antibiotic flomoxef (FMOX). IPM exhibited activity against only N. asteroides group (N. asteroides, Nocardia farcinica and Nocardia nova). On the other hand, FMOX showed activity against all pathogenic Nocardia tested. A 2- to 30-fold decreases in minimum inhibitory concentration (MIC) for N. asteroides, N. brasiliensis and N. farcinica was noted when antibiotics and beta-lactamase inhibitors were combined compared to antibiotics alone. Further combination and enzymatic studies indicated that all pathogenic Nocardia possess beta-lactamase except for a half of the strains of N. nova. These species' specific sensitivity patterns of pathogenic Nocardia are discussed in this paper with references to their taxonomic positions.