Immunological risk and complement genetic evaluations in early onset de novo thrombotic microangiopathy after living donor kidney transplantation: A Japanese multicenter registry.

BACKGROUND: Thrombotic microangiopathy (TMA) after kidney transplantation (KTx), particularly early onset de novo (dn) TMA, requires immediate interventions to prevent irreversible organ damage. This multicenter study was performed to investigate the allogeneic clinical factors and complement genetic background of dnTMA after KTx. METHODS: Perioperative dnTMA after KTx within 1 week after KTx were diagnosed based on pathological or/and hematological criteria at each center, and their immunological backgrounds were researched. Twelve aHUS-related gene variants were examined in dnTMA cases. RESULTS: Seventeen recipients (15 donors) were enrolled, and all dnTMA cases were onset within 72-h of KTx, and 16 of 17 cases were ABO incompatible. The implementation rate of pre-transplant plasmaphereses therapies were low, including cases with high titers of anti-A/anti-B antibodies. Examination of aHUS-related gene variants revealed some deletions and variants with minor allele frequency (MAF) in Japan or East Asian genome databases in genes encoding alternative pathways and complement regulatory factors. These variants was positive in 8 cases, 6 of which were positive in both recipient and donor, but only in one graft loss case. CONCLUSIONS: Although some immunological risks were found for dnTMA after KTx, only a few cases developed into TMA. The characteristic variations revealed in the present study may be novel candidates related to dnTMA in Japanese or Asian patients, but not pathogenic variants of aHUS. Future studies on genetic and antigenic factors are needed to identify factors contributing to dnTMA after KTx.

[A Case of Metanephric Adenoma Treated with Robotic-Assisted Laparoscopic Partial Nephrectomy].

A 53-year-old female patient was diagnosed with a left renal mass incidentally detected on an abdominal computed tomography (CT) scan. Further examination revealed a slightly contrast-enhancing mass 2.0 cm in diameter, in the left kidney on a contrast-enhanced CT scan. A diagnosis of left renal cell carcinoma (cT1aN0M0) was made and a robotic-assisted laparoscopic partial nephrectomy was performed. The excised tissue specimen exhibited a clearly circumscribed tumor. On hematoxylin eosin staining, the small uniform tumor cells appeared organized in glandular luminal arrangements, with lacking nuclear atypia and any malignant features. Immunostaining confirmed the diagnosis as metanephric adenoma, as indicated by positive results for WT1 and negative results for alpha-methylacyl-CoA race mase. Metanephric adenoma is an uncommon benign epithelial tumor of the kidney, which frequently poses a challenge in differential diagnosis with renal carcinoma on preoperative imaging. Pathologically, it can be challenging to differentiate from papillary renal cell carcinoma, and immunostaining can be used to effectively differentiate between the two entities.

[A Case of Bilateral Ureteral Stenosis Treated by Upper Urinary Tract Reconstruction Using an Ileal Ureter].

A 41-year-old female who suffered local recurrence of cervical cancer after receiving chemoradiotherapy underwent radical hysterectomy, radical vaginal resection, and pelvic and paraaortic lymph node dissection. After surgery, bilateral hydronephrosis due to right ureteral stenosis and left uretero-vaginal fistula occurred. We therefore placed a bilateral ureteral stent. Thereafter, we continued to replace the bilateral ureteral stent once every 3 months, but the replacement of the right ureteral stent became impossible three years after the initial placement. We thus performed bilateral upper urinary tract reconstruction using an ileal ureter with the aim of both eliminating the left ureteral vaginal fistula and resolving the right ureteral stricture.

[Robot-Assisted Partial Nephrectomy for Renal Cell Carcinoma Complicated by Leriche Syndrome : A Case Report].

A 71-year-old man was referred to our hospital for treatment of a 2 cm-sized right renal mass incidentally found by computed tomography (CT) and was diagnosed with right renal cell carcinoma cT1aN0M0. Contrast-enhanced CT revealed that the aorta was completely occluded below the inferior mesenteric artery origin, and Leriche syndrome was diagnosed. CT angiography showed several collateral arteries along the abdominal wall. A robot-assisted laparoscopic partial nephrectomy was performed to treat renal cell carcinoma. Preoperatively, we marked the collateral arteries using ultrasonography to avoid injury during trocar insertion. We did not observe any decrease in blood flow in the right leg during the operation. The pathological diagnosis was clear cell renal cell carcinoma. Leriche syndrome is a chronic occlusive disease involving the infrarenal aorta and the iliac arteries. Since lower limb blood flow is dependent on collateral circulation, it is important to avoid injuring the collateral arteries during surgery.

[Primary Ureteral Small Cell Carcinoma with Multiple Recurrences and Metastases in the Early Postoperative Period : A Case Report].

A 79-year-old woman who presented with right hydronephrosis was referred to our hospital. Abdominal computed tomography (CT) showed a right ureteral tumor (cT3N0M0). Right nephroureterectomy and partial cystectomy were performed. Pathological examination revealed small cell carcinoma (mixed type ; INFb, pT3, ly1, v1, u-rt0, ur0, RM0). Cystoscopy showed intravesical recurrence of the tumor 3 months after the surgery. Transurethral resection was performed, and histopathological examination revealed small cell carcinoma (pT2). We recommended postoperative chemotherapy ; however, the patient and her family refused consent for chemotherapy. Liver and lymph node metastases developed, and the patient died 2 months after the transurethral resection.

[Rituximab Chemotherapy for Primary Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma of the Urinary Bladder : A Case Report].

An 84-year-old man consulted a local physician for asymptomatic macrohematuria. Abdominal ultrasonography revealed thickening ofthe bladder wall from the triangular part ofthe bladder to the posterior wall, and he was referred to our department. Cystoscopy showed extensive bladder wall thickening with edema ofthe mucosa. Abdominal contrast-enhanced computed tomography (CT) showed extensive bladder wall thickening and right external iliac lymphadenopathy accompanied by a contrast effect suspected ofbeing extravesical invasion. We performed transurethral resection ofthe bladder tumor and made the diagnosis ofmucosa associated lymphoid tissue (MALT) lymphoma. Our diagnosis made from positron emission tomography-CT performed after surgery was primary MALT lymphoma of the bladder and metastasis to the right external iliac lymph node. We administered rituximab 375 mg/m2 once a week for four times in total. CT after rituximab administration showed that the tumor and right external iliac lymph nodes had shrunk significantly, and no recurrence was present at 18 months after treatment.

[A Case of Pericardial Effusion Induced by Nivolumab for Metastatic Renal Cell Carcinoma].

A 72-year-old female with left renal cell carcinoma and lymphadenopathy had undergone hand-assisted laparoscopic left nephrectomy and dissection of the lymph node (papillary renal cell carcinoma, type 2, pT3a pN2 M1). She had been treated with adjuvant chemotherapy with sunitinib, temsirolimus and pazopanib. However, the patient was started on nivolumab due to disease progression. After receiving 5 cycles of nivolumab, she was admitted to our emergency room for chest discomfort and appetite loss. Since computed tomographic (CT) scan showed pericardial effusion, we performed pericardiocentesis. Cytological examination of the pericardial effusion demonstrated leukocytes and no malignant cells. CT scan two weeks after cardiocentesis showed no recurrent pericardial effusion. She became stable with nivolumab, but the administration of nivolumab was discontinued and she started receiving axitinib.

[Two Cases of Clear Cell Adenocarcinoma Arising in Urethral Diverticulum].

We report two cases of clear cell adenocarcinoma arising in the urethral diverticulum. Case 1 occurred in a 79-year-old woman presenting with complaints of frequent micturition. Magnetic resonance imaging (MRI) revealed a localized urethral diverticular tumor. Transurethral resection of the tumor was performed, and the final histopathological diagnosis was clear cell adenocarcinoma. Anterior pelvic exenteration was performed. She had no recurrence 15 months after surgery. Case 2 occurred in a 79-year-old woman presenting with urinary incontinence. As in Case 1, MRI and histopathological findings of transurethral resection of the tumor revealed clear cell adenocarcinoma in the urethral diverticulum. Anterior pelvic exenteration and ileal conduit formation were performed. She had no recurrence 16 months after surgery. Clear cell adenocarcinoma in the urethral diverticulum is very rare. We review 17 cases of clear cell adenocarcinoma arising in the urethral diverticulum in Japan.

High-pressure carbon monoxide preserves rat kidney grafts from apoptosis and inflammation.

Renal ischemia-reperfusion (I/R) injury is unavoidable in kidney transplantation (KTx) and frequently influences both short- and long-term allograft survival. Carbon monoxide (CO) has attracted attention as a medical gas with anti-inflammatory and anti-apoptotic effects. We investigated a new strategy for organ preservation using ex vivo application of high-pressure CO in an experimental rat KTx model. We preserved kidney grafts using a high-pressure chamber filled with mixed gases composed of CO and O2. We found that cold I/R injury resulted in progressive deterioration of renal graft function in University of Wisconsin solution, whereas CO significantly improved renal function. We confirmed that CO decreased oxidative stress and mRNA expression of proinflammatory cytokines and inhibited tubular apoptosis in the early phases. Western blot analysis demonstrated that CO increased phosphatidylinositol-3 kinase and phosphorylation of Akt and p38 mitogen-activated protein kinase. Furthermore, CO significantly alleviated tubular injury scores and suppressed the development of interstitial fibrosis at 100 days after KTx. Thus, high-pressure mixed CO and O2 gases successfully preserved rat kidney grafts for 24 h by protecting tubular epithelial cells from apoptosis and inhibiting inflammation.

The neutrophil-to-lymphocyte ratio as a predictor of intravesical recurrence in patients with upper urinary tract urothelial carcinoma treated with radical nephroureterectomy.

BACKGROUND: The neutrophil-to-lymphocyte ratio (NLR) is a marker of systemic inflammation associated with recurrence and poor prognosis in numerous cancer types. The aim of this study was to evaluate the use of the NLR as a biomarker for intravesical recurrence (IVR) in patients undergoing radical nephroureterectomy (RNU) for upper urinary tract urothelial carcinoma (UTUC) for the first time. METHODS: We retrospectively analyzed the records of 100 patients with UTUC who had undergone RNU between 1999 and 2015 at our institution. The association between the preoperative NLR and IVR were assessed using multivariate models. RESULTS: Among the 100 patients enrolled in the study, 33 developed IVR during a median follow-up of 34 months. The receiver operating characteristic analysis revealed that the optimum cut-off value for the preoperative NLR was >3.8. A high preoperative NLR (n = 21) was associated with a significantly increased risk of lymph node involvement (p = 0.036) and IVR (p = 0.034) compared with a low preoperative NLR (n = 79). IVR-free survival in patients with a high preoperative NLR was significantly worse than that of patients with a low preoperative NLR (p = 0.018). On multivariate analysis, the preoperative NLR [hazard ratio (HR) 2.49; p = 0.015] and tumor multifocality (HR 2.96; p = 0.024) were independent risk factors predictive of IVR. CONCLUSION: In our study population of patients with UTUC who had undergone RNU the preoperative NLR was associated with a significantly increased risk of IVR, suggesting that the NRL could be a useful biomarker for predicting IVR.

[Seminal Vesicle Abscess Associated with Zinner Syndrome].

We report a case of seminal vesicle abscess associated with Zinner syndrome. A 26-year-old male was admitted to our hospital because of fever and right scrotal swelling. Ultrasound showed an enlarged epididymis and color Doppler ultrasound showed increased vascularity in the epididymis. We diagnosed the case as acute epididymitis and started intravenous antibiotic therapy. Four days after admission, remittent fever persisted and blood culture was positive for Staphylococcus aureus. We performed computed tomography, which showed a right seminal vesicle cyst abscess and right renal agenesis. Our diagnosis was an abscess associated with Zinner syndrome. Transrectal ultrasound-guided transperineal drainage was performed 30 days after admission because seminal vesicle abscess could not be controlled by conservative treatment. Eight days after the procedure, the patient was discharged. One year after the procedure, recurrence of seminal vesicle abscess has not been observed.

[A CASE OF ABO INCOMPATIBLE LIVING KIDNEY TRANSPLANTATION IN A PATIENT WITH SELECTIVE IgA DEFICIENCY].

We report a case of ABO incompatible living kidney transplantation in a 45-year-old man with selective IgA deficiency. He has been on hemodialysis for 1 year due to chronic renal failure. Although the serum anti-IgA antibody was negative, he has experienced anaphylactic reaction to red blood cell product in the past. Before the transplantation, we performed double filtration plasmapheresis for two times, but didn't performed plasma exchange, considering the possibility of producing anti-IgA antibody and anaphylactic reaction. He underwent kidney transplantation from his mother without anaphylactic reaction, following to the recirculation of renal blood flow. He was discharged on the 29th postoperative day when the serum creatinine level was 1.2 mg/dL. The graft function was stable at 8 months after transplantation with no evidence of rejection and infection.

[A CASE OF PELVIC FRACTURE URETHRAL INJURY RECONSTRUCTED BY DEFERRED URETHROPLASTY].

We report a case of pelvic fracture urethral injury reconstructed by anastomotic urethroplasty. A 24-year-old male was referred to our hospital because of pelvic trauma accompanying ischial fracture. Retrograde urethrography showed urethral disruption and suprapubic catheter was inserted. One week later, we underwent endoscopic realignment. Three months later, we removed the Foley balloon catheter after we had checked that there was no stricture by the voiding cystourethrogram. However, 5 days after that, he came to our hospital because of urinary retention. Cystoscopy detected urinary stricture between bulbar and membranous urethra. We decided to do deferred urethroplasty. Five months after that we performed anastomotic urethroplasty. He was discharged 31 days after the operation. No stricture has been detected for 7 months postoperatively.

Impact of prior abdominal surgery on the outcomes after robotic - assisted laparoscopic radical prostatectomy: single center experience.

PURPOSE: To evaluate the influence of prior abdominal surgery on the outcomes after robotic-assisted laparoscopic radical prostatectomy (RALP). MATERIALS AND METHODS: We retrospectively analyzed patients with prostate cancer who underwent RALP between June 2012 and February 2015 at our institution. Patients with prior abdominal surgery were compared with those without prior surgery while considering the mean total operating, console, and port-insertion times; mean estimated blood loss; positive surgical margin rate; mean duration of catheterization; and rate of complications. RESULTS: A total of 203 patients who underwent RALP during the study period were included in this study. In all, 65 patients (32%) had a prior history of abdominal surgery, whereas 138 patients (68%) had no prior history. The total operating, console, and port-insertion times were 328 and 308 (P=0.06), 252 and 242 (P=0.28), and 22 and 17 minutes (P=0.01), respectively, for patients with prior and no prior surgery. The estimated blood losses, positive surgical margin rates, mean durations of catheterization, and complication rates were 197 and 170 mL (P=0.29), 26.2% and 20.2% (P=0.32), 7.1 and 6.8 days (P=0.74), and 12.3% and 8.7% (P=0.42), respectively. Furthermore, whether prior abdominal surgery was performed above or below the umbilicus or whether single or multiple surgeries were performed did not further affect the perioperative outcomes. CONCLUSIONS: Our results suggest that RALP can be performed safely in patients with prior abdominal surgery, without increasing the risk of complications.

[Removal of a Foreign Body in the Urinary Bladder in a Patient Four Times in the Past Thirty Years].

We report a case of recurring foreign bodies in the urinary bladder. A 67-year-old male inserted a foreign body into the urinary bladder during masturbation. Eight months later, he experienced a fever and went to a hospital where ultrasonography revealed a foreign body in his urinary bladder. Then, he was referred to our hospital for surgical treatment. The patient's surgical record indicated that he had undergone the same operation 3 times in the past thirty years. The inserted foreign body was successfully removed by suprapubic cystotomy, and he was discharged 13 days after the operation. He was also evaluated by psychiatrists, but they diagnosed that he had no mental disorder. To our knowledge, this is the first report on the removal of a foreign body in the urinary bladder four times in the same patient.

[Two Cases of Penile Fracture Diagnosed by Magnetic Resonance Imaging].

We report two cases of penile fracture. Case 1 was in a 22-year-old male. He heard a cracking sound during urination and experienced acute penile pain and detumescence. He was admitted to our hospital on that day. Case 2 was in a 52-year-old male. He heard a cracking sound during sexual intercourse and experienced detumescence. He was admitted to our hospital on the next day. In both cases, magnetic resonance imaging (MRI) showed disruption of the tunica albuginea. We performed immediate surgical repair through localized incision. They had no perioperative complications. Several months after surgery, they reported subjectively good erection without penile curvature or pain. We found that MRI is a useful tool for the assessment of location of the tunica rupture and minimization of the surgical incision.

[A Case of Steroid Sulfatase Deficiency Complicated by Bilateral Undescended Testis].

Steroid sulfatase (STS) deficiency is one of the causes of ichthyoses. STS genes on the X chromosome is responsible for this disease. Therefore, STS deficiency is also called X-linked ichthyosis. Herein we report a case of STS deficiency complicated by bilateral undescended testis. A5-year-old-boy with STS deficiency was referred to our hospital because of bilateral undescended testis. We performed bilateral orchiopexy.

[A Case of Prenatal Testicular Torsion].

We report a prenatal case of neonatal testicular torsion. A 0-day-old boy with left scrotal swelling from the time of birth was referred to us. The physical examination revealed left intrascrotal hard mass. The ultrasonography with doppler color flow showed heterogeneous parenchymal echogenicity and the lack of blood flow to the affected testis. Testicular torsion was suspected and emergent surgical exploration was performed. The left testis was necrotic with extravaginal torsion of the spermatic cord. Left high orchiectomy was performed, considering the possibility of inguinal hernia. The histopathological examination confirmed the necrosis of the left testis.

[The Vaginal Metastasis of Ureteral Carcinoma after Left Nephroureterectomy: A Case Report].

69-year-old woman underwent left nephroureterectomy for left ureteral cancer (urothelial carcinoma (UC), high grade, pT3pN0) in September 2013. She returned to our hospital presenting with asymptomatic macrohematuria in July 2014. Cystoscopy showed tiny papillary tumors in the bladder. We also found genital bleeding from multiple papillary tumors on the vaginal wall. We performed transurethral resection of the bladder tumor and a biopsy of the vaginal wall demonstrated non-invasive UC, high grade. Pelvic magnetic resonance imaging after the operation showed no infiltration outside the bladder wall and vaginal wall. Therefore, we performed endoscopic excision of the vaginal tumor. However we could not resect all vaginal tumors. Irradiation of the vagina and uterus was performed under the diagnosis of metastasis of UC tovagina. Vaginal UC is extremely rare and this is the 26th case report in the literature.