Nihon Hinyokika Gakkai zasshi. The japanese journal of urology最新文献

{"title":"[THREE CASES OF DENYS-DRASH SYNDROME WITH GONADOBLASTOMA].","authors":"Mimu Ishikawa, Naoya Tomomasa, Shutaro Yamamoto, Takahiro Kimura, Yuichi Hasegawa","doi":"10.5980/jpnjurol.115.104","DOIUrl":"https://doi.org/10.5980/jpnjurol.115.104","url":null,"abstract":"<p><p>Denys-Drash syndrome (DDS) is characterized by rapidly progressing nephropathy, Disorders of Sex development, and Wilms tumor. Gonadal removal is recommended owing to the increased risk of developing gonadal tumors; however, the optimal timing remains uncertain. Herein, we report three cases with gonadoblastoma discovered in excised gonads. Case 1 involves a 13-year-old girl for whom peritoneal dialysis (PD) was initiated at 6 months of age. The left gonad removal at 9 months revealed hypoplastic testicular tissue. At 2 years and 8 months, kidney transplantation and bilateral nephrectomy were performed. A living kidney transplant was received at the age of 10 years. Subsequently, virilization signs and right gonadal swelling led to a diagnosis of gonadoblastoma during right gonadectomy at 13 years and 7 months. Case 2 involves a 6-year-old girl for whom PD was initiated 1 month after birth. Left nephrectomy was performed at 1 years and 11 months, followed by right nephrectomy at 5 years and 4 months. At the age of 6 years and 1 months, living kidney transplantation and simultaneous right gonadectomy revealed gonadoblastoma with a dysgerminoma component, without metastasis. Left gonadectomy 3 months post-transplantation revealed testis pathology. Case 3: involves a 4-year-old girl for whom PD was initiated 9 days after birth. Bilateral nephrectomy was performed at 1 year of age. Bilateral gonadectomy at 4 years and 5 months revealed bilateral ovotestis with a left gonadoblastoma component. While gonadoblastoma has limited malignant findings, it serves as the precursor of highly malignant gonadal tumors, highlighting the importance of timely excision. In DDS cases, determining the optimal timing for gonadectomy remains complex owing to variations in nephropathy progression. The priority given to PD initiation and Wilms' tumor prevention further complicates excision timing. Notably, the case of dysgerminoma in our 6-year-old patient appears to be one of the youngest cases in the literature.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"115 2","pages":"104-107"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144048858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[UPFRONT DOCETAXEL WITH ANDROGEN DEPRIVATION THERAPY IN MALES WITH HIGH-VOLUME METASTATIC CASTRATION-SENSITIVE PROSTATE CANCER: RESULTS OF A SINGLE-INSTITUTION SERIES].","authors":"Kenichi Hata, Masaki Hashimoto, Yusuke Takahashi, Shun Saito, Ayaka Kawaharada, Yuki Enei, Masatoshi Tanaka, Keigo Sakanaka, Kazuhiro Takahashi, Akira Hisakane, Takafumi Yanagisawa, Shunsuke Tsuzuki, Mariko Honda, Akira Furuta, Kenta Miki, Takahiro Kimura","doi":"10.5980/jpnjurol.115.11","DOIUrl":"10.5980/jpnjurol.115.11","url":null,"abstract":"<p><p>(Objective) This study aimed at evaluating the efficacy and safety of upfront docetaxel (DTX) treatment and androgen deprivation therapy (ADT) in male patients with high-volume metastatic castration-sensitive prostate cancer (HV-mCSPC). (Methods) This retrospective study was conducted using the medical records of 30 patients treated for HV-mCSPC by using upfront DTX treatment along with ADT at Atsugi City Hospital between December 2015 and December 2022. The patient characteristics, demographics, oncological outcomes, adverse events, and sequential therapy were evaluated. (Results) Thirty patients were included in the final analysis. The median patient age and prostate-specific antigen at diagnosis were 73 years (range, 53-83 years) and 250 mg/ml (range, 0.54-3,817 ng/ml), respectively. The completion rate of six cycles of upfront DTX treatment was 86.7%. The median progression-free survival was 24 months; the median overall survival was not reached, and the 5-year survival rate was 71.5%. Alopecia was the most frequent non-hematological adverse event (60%) followed by fatigue (53.3%). Overall, adverse events of grade 3 or higher occurred in 46.7% of the patients, with neutropenia being the most frequent. The incidence of neutropenia of grade 3 or higher was significantly lower in the group receiving primary prophylaxis with long-acting granulocyte colony-stimulating factor (7.7% vs. 75%, P = 0.009). Abiraterone was the most frequently administered sequential treatment in 12 patients (60%). (Conclusion) In the triplet combination treatment era, upfront DTX treatment and ADT for patients with HV-mCSPC was safe as primary prophylaxis for severe neutropenia and effective as an upfront treatment. However, it should be selected if its effectiveness is superior to triplet treatment considering adverse events, cost-effectiveness, and quality of life.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"115 1","pages":"11-20"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[IMPACT OF ADJUVANT CHEMOTHERAPY AFTER RADICAL CYSTECTOMY FOR PATIENTS WITH LOCALLY ADVANCED BLADDER CANCER].","authors":"Akinori Minato, Ikko Tomisaki, Rieko Kimuro, Katsuyoshi Higashijima, Mirii Harada, Kazumasa Jojima, Takuro Sakamoto, Tomohisa Takaba, Daichi Ohno, Keisuke Kuretake, Kentarou Tanigawa, Takuo Matsukawa, Kenichi Harada, Naohiro Fujimoto","doi":"10.5980/jpnjurol.114.99","DOIUrl":"https://doi.org/10.5980/jpnjurol.114.99","url":null,"abstract":"<p><p>(Objective) The study aimed to retrospectively evaluate the therapeutic effects of adjuvant chemotherapy (AC) in patients following radical cystectomy (RC) in locally advanced bladder cancer. (Methods) A single-center-derived database registered 227 patients diagnosed with muscle-invasive bladder cancer and treated with RC and pelvic lymphadenectomy between March 2003 and December 2021. Of these, patients diagnosed with non-organ-confined diseases were classified as either pT3-T4 or pN-positive without distant metastasis. Platinum-based AC was administered for the following categories: two courses for patients with pT3-T4 and pN-negative and three courses for those with pTany and pN-positive. The primary endpoint was the disease-free survival (DFS) and overall survival (OS) between the patients receiving and not receiving AC. (Results) Among all patients, 90 were diagnosed with non-organ-confined disease: 43 (47.8%) were treated with AC and the remaining 47 (52.2%) were left untreated. The methotrexate, vinblastine, doxorubicin, and cisplatin regimen; the gemcitabine and cisplatin regimen; and the gemcitabine and carboplatin regimen were administered to 14 (32.6%), 25 (58.1%), and 4 (9.3%) patients, respectively. With a median follow-up period of 26 months, the groups that received and did not receive AC had 2-year DFS rates of 36.3% and 25.9% (median DFS time: 15 vs. 8 months, p=0.026) and 2-year OS rates of 64.3% and 41.4% (median OS time: 38 vs. 18 months, p=0.064), respectively. In patients with pT3-T4 and pN-negative, no significant difference in the median DFS and OS between the AC and non-AC groups was observed. However, in patients with pTany and pN-positive, the DFS (median: 14 vs. 4.5 months, p=0.002) and OS (38 vs. 11.5 months, p=0.009) were longer in the AC than those in the non-AC group, respectively. The multivariate Cox regression analysis revealed that AC administration was an independent predictor for DFS (hazard ratio: 0.44, 95% confidence interval: 0.24-0.79, p=0.006). (Conclusion) Platinum-based AC following RC significantly improved DFS in pN-positive patients with locally advanced bladder cancer.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 4","pages":"99-107"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142485163","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[EFFECTS OF COMBINATION THERAPY WITH VIBEGRON FOR ANTICHOLINERGIC-RESISTANT PEDIATRIC NEUROGENIC BLADDER].","authors":"Shotaro Yamamoto, Takayuki Hirano, Chiaki Nakamura, Kohei Mori, Atsushi Hamano","doi":"10.5980/jpnjurol.114.81","DOIUrl":"https://doi.org/10.5980/jpnjurol.114.81","url":null,"abstract":"<p><p>(Objective) We report the effectiveness of combination therapy with vibegron in pediatric patients with neurogenic bladder inadequately responding to anticholinergic agents. (Subjects and methods) This retrospective study involved 13 pediatric patients with neurogenic bladder treated with anticholinergics at our department from November 2019 to January 2021 who had an inadequate response and received combination therapy with vibegron. Changes in the volume of urinary incontinence before and after the use of vibegron reported during interviews from the 13 patients were compared. In addition, bladder capacity at the end of examination, bladder capacity at the end of examination/expected bladder capacity (EBC), and bladder compliance were compared using the Wilcoxon signed rank test in 9 patients for whom urodynamics (UDS) or video urodynamics (VUDS) was performed before and after introduction of vibegron. (Results) The 13 patients comprised 8 boys and 5 girls. The median age was 13 years (range, 5-18 years). Underlying diseases included 9 cases of spina bifida, 1 case of Hinman syndrome, 1 case of cervical vertebra injury, 1 case of idiopathic cervical epidural hematoma combined with spina bifida, and 1 case of spinal cord infarction. Eight of the 13 patients experienced decrease in urinary incontinence after the introduction of vibegron. All 9 patients who underwent UDS or VUDS before and after introduction of vibegron displayed significant differences in bladder capacity at the end of the examination, bladder capacity at the end of the examination/EBC, and bladder compliance, indicating improvement. (Conclusion) Combination therapy with vibegron is effective for pediatric patients with neurogenic bladder who have inadequately responded to anticholinergic agents.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 3","pages":"81-85"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141736315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[COMPARING LAPAROSCOPIC AND ROBOT-ASSISTED PYELOPLASTY FOR URETERO-PELVIC JUNCTION OBSTRUCTION: INITIAL EXPERIENCE FROM A SINGLE CENTER].","authors":"Tomoyuki Tatenuma, Hiroki Ito, Mitsuru Komeya, Yusuke Ito, Kentaro Muraoka, Hisashi Hasumi, Narihiko Hayashi, Kazuhide Makiyama","doi":"10.5980/jpnjurol.114.1","DOIUrl":"10.5980/jpnjurol.114.1","url":null,"abstract":"<p><p>(Objective) To compare the initial results of robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP) for uretero-pelvic junction obstruction (UPJO). (Methods) Between April 2008 to October 2021, we identified 104 cases of UPJO where LP was performed and 18 cases where RALP was performed at our hospital. We retrospectively analyzed their perioperative outcomes. Furthermore, we recorded the operative times for each cases of LP and RALP. (Results) The median operative time for RALP was 141 minutes, which was significantly shorter than that for LP (204 minutes). No patient in the RALP group demonstrated any Clavien-Dindo complications (≥grade 3). During the observation period, improvement of symptoms was observed in all cases. The median suturing time in RALP was 38 minutes. Compared with the last 20 cases of LP, the time to expose the uretero-pelvic junction, the time of renal pelvis incision, and suturing time were significantly shorter in RALP. In addition, the console and suturing times were stable since the initial stage. In cases with a high grade of hydronephrosis, there was a large variation in the time to expose the uretero-pelvic junction and suture the renal pelvis and ureter in LP; however, this variation was smaller in RALP. (Conclusion) At our hospital, RALP for UPJO is considered to be a safe procedure. In the future, it is necessary to consider the long-term results and effectiveness of RALP.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 1","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139512718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[INFECTIOUS AORTITIS-RELATED MORTALITY AFTER ILEAL NEOBLADDER SUBSTITUTION IN A PATIENT WITH DIABETES MELLITUS].","authors":"Akihisa Taniguchi, Kazushi Hirakawa, Ikumi Mayama","doi":"10.5980/jpnjurol.114.26","DOIUrl":"10.5980/jpnjurol.114.26","url":null,"abstract":"<p><p>A 78-year-old man, who underwent total cystectomy with ileal neobladder substitution for bladder cancer 5 years ago, had a fever since the beginning of May 2022. He was hospitalized in an internal medicine ward of another hospital and was diagnosed with febrile urinary tract infection (UTI). Escherichia coli with sensitivity to almost all antibiotics was cultured in urine. Computed tomography (CT) showed that the distended neobladder with bilateral hydronephrosis contained gas and the severely athelosclerotic aorta. Even after using four antibiotics, the UTI could not be controlled. After 3 weeks of hospitalization, CT showed periaortic lymphatic swelling. Therefore, he was transferred to our hospital on 6 June due to uncontrollable UTI and lymphatic metastasis of bladder cancer. However, CT revealed that the neobladder remained distended and showed thickening of the periaortic soft tissue with gas. He was diagnosed with advanced infectious aortitis. Furthermore, he had poorly controlled diabetes mellitus of HbA1c 8.4%. Immediately after admission, an exchange of the urethral catheter and antibiotics, and blood sugar control strengthening were performed. On the second day, the patient was close to defervescence. However, on the third day, abrupt onset of loss of consciousness and abdominal swelling occurred. CT showed retroperitoneal hematoma caused by the rupture of the aorta. Then, bradycardia and respiratory arrest occurred, ventilator management and blood transfusion were performed, and the patient survived. However, his condition worsened, and he died 2 days later. The patient had undergone ileal neobladder substitution, but had infectious aortitis and died of an aortic rupture due to distended neobladder-induced UTI, poorly controlled diabetes mellitus and the severely athelosclerotic aorta.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 1","pages":"26-29"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139512895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[NONRISING BLADDER PARAGANGLIOMA SUCCESSFULLY TREATED BY THE OPEN BLADDER SURGERY: A CASE REPORT].","authors":"Shuji Kita, Maki Yamakawa, Reina Kono, Kai Soumiya, Yuichiro Atagi, Shigeo Nakanishi, Yutaka Yanagihara, Iku Ninomiya, Kenjirou Okamoto, Sadamu Yamashi, Masaharu Kan","doi":"10.5980/jpnjurol.114.16","DOIUrl":"10.5980/jpnjurol.114.16","url":null,"abstract":"<p><p>The patient is a 47-year-old female. MRI revealed a well-defined submuscular mass in the bladder muscle layer. Bladder paraganglioma was suspected based on MRI findings. Endocrinologic Testing showed no significant elevation. ¹²³I-MIBG scintigraphy of the mass showed a significant uptake, and we made diagnosis of bladder paraganglioma. The mass was nonrising and showed no color differentiation making its location undetectable. Using MRI with a ureteral stent and urethral catheter in place, we were able to determine its location. The possibility of damage to the ureteral or internal urethral opening was feared. We chose open bladder surgery, emphasizing ease of operation and visualization. Although a transient increase in blood pressure was observed during the operation, the mass was resected as a single mass from all layers of the bladder without damaging the ureteral or internal urethral opening. Histopathological examination revealed a paraganglioma.MRI (ureteral stent and urethral catheter placement) and open bladder surgery were useful for identifying the location and resecting this case of this otherwise undetectable bladder paraganglioma.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 1","pages":"16-20"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139513338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A RETROSPECTIVE ANALYSIS OF SURGICAL POSITIONING INJURY USING INCIDENT REPORTING SYSTEM].","authors":"Kosuke Shibamori, Toshiaki Tanaka, Takuto Ogasawara, Junko Sasaki, Yukiko Hori, Tetsuya Shindo, Yuki Kyoda, Kohei Hashimoto, Ko Kobayashi, Naoya Masumori","doi":"10.5980/jpnjurol.114.116","DOIUrl":"https://doi.org/10.5980/jpnjurol.114.116","url":null,"abstract":"<p><p>(Purpose) Surgical positioning injury (SPI) is a cutaneous, musculoskeletal, neurological, or vascular injury resulting from the position of the patient during surgery. We performed a retrospective study using incident reporting system to examine the incidence of SPI at our hospital. (Materials and methods) Among anesthesiology managed surgical cases, SPI cases reported in the incident reporting system between 2012 and 2017 were examined. The primary endpoint was the incidence of SPI. (Results) Of 35,400 anesthesiology managed cases, 59 (0.2%) had SPI reported in the incident reporting system. Forty-four (75%) were male patients. Median age and BMI were 60 and 23.6 years, respectively. Forty-four (75%) were not supine position. Median operative time and blood loss were 419 minutes and 220 ml, respectively. Nurses reported incident reports in 52 (88%) cases, and only 7 (12%) by physicians. Skin injuries were reported in 42 cases (71%) and neurovascular injuries in 17 cases (29%). Of the neurovascular injuries, 4 (7%) were lower extremity compartment syndrome. Three cases of the 4 received the fasciotomy. (Conclusions) The incidence of SPI reported in the incident report system was 0.2%. Four cases had compartment syndrome. This result suggested the importance of intraoperative and postoperative observation in addition to proper correct positioning.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 4","pages":"116-121"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142485160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[DIAGNOSIS OF GENETIC VARIANT CARRIERS IN A PATIENT WITH ASYMPTOMATIC BIRT-HOGG-DUBÉ SYNDROME: A CASE REPORT].","authors":"Shogo Watari, Takaharu Ichikawa, Akira Hirasawa, Hiromasa Shiraishi, Moto Tokunaga, Risa Kubota, Norihiro Kusumi, Tomoyasu Tsushima, Yoko Shinno, Mitsuko Furuya","doi":"10.5980/jpnjurol.114.61","DOIUrl":"https://doi.org/10.5980/jpnjurol.114.61","url":null,"abstract":"<p><p>Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder caused by germline mutations in the folliculin gene (FLCN). It is characterized by skin tumors, multiple lung cysts, and renal tumors. Active genetic testing and appropriate periodic examinations of family lines of patients with BHD syndrome have not been widely performed. In this report, we present our experience regarding the diagnosis of asymptomatic family members with BHD syndrome. The proband was a 65-year-old female with a family history of colorectal cancer and spontaneous pneumothorax that affected her father. Computed tomography revealed an approximately 10 cm-sized tumor protruding from the upper pole of the left kidney, a buried tumor approximately 1.5 cm in length in the right kidney, and multiple pulmonary cysts. The patient underwent laparoscopic radical left nephrectomy. Pathological examination indicated that the resected tumor was a chromophobe renal cell carcinoma. After the surgery, there was no evidence of local recurrence or metastasis. The size of the tumor in the right kidney was monitored, but it did not increase. On FLCN genetic examination, targeted next generation sequencing revealed a partial deletion of exon 14, thus confirming the diagnosis of the patient to be BHD syndrome that caused the previously unreported pathogenic variant. Three years after the surgery, we conducted genetic counseling for the proposita and her three children. Genetic examination, performed at the request of the second daughter, confirmed that she carried the same genetic variant as her mother. This diagnosis prompted the second daughter to begin managing her health via periodic imaging tests.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 2","pages":"61-65"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140863079","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[MITOCHONDRIAL RESPIRATORY CHAIN DISORDERS WITH VESICOURETERAL REFLUX: A PEDIATRIC CASE REPORT].","authors":"Shinta Suenaga, Satoko Matsuyama, Futoshi Matsui, Koji Yazawa, Fumi Matsumoto","doi":"10.5980/jpnjurol.114.89","DOIUrl":"https://doi.org/10.5980/jpnjurol.114.89","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":101330,"journal":{"name":"Nihon Hinyokika Gakkai zasshi. The japanese journal of urology","volume":"114 3","pages":"89-92"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141736317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}