Right-sided Bochdalek hernia in an elderly patient: a case review of adult Bochdalek hernias from 1982 to 2015 in Japan.

Case: An 89-year-old woman was admitted to our hospital because of dyspnea following abdominal pain and vomiting. According to chest computed tomography findings, a diagnosis of acute respiratory failure due to right-sided Bochdalek hernia was made. Outcome: Emergency laparotomy revealed that a bowel loop from the terminal ileum to the transverse colon had herniated into the right thoracic cavity through the foramen of Bochdalek. The herniated organs were reduced to the abdominal cavity and the foramen was repaired by direct suturing. The patient had good recovery and was discharged 78 days later. Conclusion: Bochdalek hernias in elderly patients are rare, but may be complicated by strangulation of the herniated organ. This should be taken into account by acute care physicians. To prevent this fatal complication, regardless of symptoms, surgical treatment may be essential as soon as a diagnosis of adult Bochdalek hernia is made.

Resurgence of bovine ephemeral fever in mainland Japan in 2015 after a 23-year absence.

In September and October 2015, suspected cases of bovine ephemeral fever (BEF) were reported in the mainland region of Kagoshima Prefecture and on Tanegashima Island. The genome of the BEF virus (BEFV) was detected in the diseased cows and the cows that had recovered. The serum obtained from the affected cows contained high titers of BEFV-neutralizing antibody. In total, 18 affected cows were demonstrated to be infected with BEFV during the outbreak. Our findings showed evidence that BEF occurred in mainland Japan after a 23-year absence. Phylogenetic analysis based on the surface glycoprotein (G) gene revealed that BEFVs detected in the affected cows were genetically distinct from previous Japanese BEFVs, but were close to BEFVs circulating in Taiwan and mainland China in recent years. Amino acid substitution in the neutralizing epitope domains of the G protein was limited between the detected viruses and the vaccine strain (YHL isolate), and high titers of the neutralizing antibody against the YHL isolate were induced in the infected cattle during the disease occurrences. Therefore, current BEF vaccines probably elicit protective immunity against the BEFVs detected in 2015, although their effectiveness should be assessed. Since the BEFV vaccination rates are estimated to be low, a BEF outbreak should be considered a possibility in mainland Japan.

[A CASE OF URETERAL ENDOMETRIOSIS].

A 49-year-old woman was admitted to our hospital due to macroscopic hematuria. Contrast-enhanced computed tomography revealed left hydronephrosis, a tumor at her left ureter, pseudoaneurysm and ovarian cystoma. Prior to the operation, the tumorous lesion was considered as left ureteral cancer without metastasis (cT4N0M0; stage IV). Left nephroureterectomy was performed. After the surgery, pathological examination revealed that this lesion was extrinsic endometriosis originating from the ureter.We here report this case of ureteral endometriosis that presented with atypical clinical findings along with a review of the literature.

Delayed perforation of the sigmoid colon after endoscopic reduction of sigmoid volvulus.

Case: An 81-year-old woman was diagnosed with sigmoid volvulus. As there were no signs of peritoneal irritation, emergency endoscopic reduction was attempted and achieved successfully. Although she remained stable on the following day, she suddenly developed perforative pan-peritonitis 35 h after the procedure. Outcome: Emergency laparotomy revealed a 10-mm-diameter perforation at the anti-mesenteric wall of the top of the sigmoid loop, and sigmoid resection with Hartmann's procedure was carried out. Pathological examination revealed ischemic necrosis around the perforation site. She recovered well with intensive care, and was discharged 32 days later. Conclusion: Delayed sigmoid perforation after endoscopic reduction of sigmoid volvulus is a rare but important and life-threatening pitfall, and should always be considered by acute care physicians. To prevent this dangerous complication, evaluation of the viability of the entire sigmoid by direct vision and semi-emergency operation as soon as the patient's condition is optimized after endoscopic reduction may be essential.

[Two cases of adult T-Cell leukemia/lymphoma with main lesion in stomach treated by VCAP-AMP-VECP regimen].

We report two cases of adult T-cell leukemia/lymphoma(ATLL)having their main lesions in the stomach. Case 1 was a 74-year-old man, complaining of left upper abdominal mass and pain. Upper gastrointestinal endoscopy revealed an ulcerous lesion in the stomach. Histological analysis and southern blotting for HTLV-1 pro-viral DNA led us to our diagnosis of ATLL. There were no apparent lesions in the bone marrow and other organs. He died of tumor lysis and multi-organ failure shortly after treatment with the VCAP-AMP-VECP regimen. Case 2 was a 68-year-old man complaining of abdominal bloating and pain. Upper gastrointestinal endoscopy disclosed an irregularity of the gastric mucosa. A biopsy sample was diagnosed pathohistologically as non-Hodgkin's lymphoma. We conducted total gastrectomy. Based on the results from the histological study and southern blotting for HTLV-1 p ro-viral DNA in the resected specimen, a diagnosis of ATLL was made. We treated him with a VCAP-AMP-VECP regimen, but multiple bone metastases and pathologic fracture occurred, proving that the disease was progressive. ATLL having a main lesion in the stomach is rare, and requires an accumulation of cases analyzed with careful diagnostic approach to establish a standard therapy for it.

Depression of mitochondrial metabolism by downregulation of cytoplasmic deacetylase, HDAC6.

Mitochondria perform multiple functions critical to the maintenance of cellular homeostasis. Here we report that the downregulation of histone deacetylase 6 (HDAC6) causes a reduction in the net activity of mitochondrial enzymes, including respiratory complex II and citrate synthase. HDAC6 deacetylase and ubiquitin-binding activities were both required for recovery of reduced mitochondrial metabolic activity due to the loss of HDAC6. Hsp90, a substrate of HDAC6, localizes to mitochondria and partly mediates the regulation of mitochondrial metabolic activity by HDAC6. Our finding suggests that HDAC6 regulates mitochondrial metabolism and might serve as a cellular homeostasis surveillance factor.

[A case of complex (type IV) paraesophageal hiatal hernia with herniation of the small intestine].

A 78-year-old woman was admitted to our hospital because of abdominal pain. She was diagnosed with complex (type IV) paraesophageal hiatal hernia, for which an operation was performed. At laparotomy, a 50 cm long ileum was found to have herniated into the thoracic cavity through the esophageal hiatus along with the sliding hiatal hernia of the stomach. Both the organs were reduced to the abdominal cavity. The hiatal hernia defect (diameter, 7cm) was repaired with a direct suture, and the gastric fundus was sutured to the diaphragm. Presently, 6 years have passed since the operation, there is no sign of recurrence.

Current problems of chronic active antibody-mediated rejection.

The Banff 2007 classification allows chronic rejection to be differentiated based on clinicopathological characteristics evidenced by two independent immunologic mechanisms; chronic active antibody-mediated rejection and chronic active T-lymphocyte mediated rejection. However, several incompletely understood issues concerning chronic active antibody-mediated rejection remain. Chronic active antibody-mediated rejection is characterized by C4d deposition in the capillary basement membrane(PTC), the presence of circulating anti-donor antibodies(DSA), and morphologic evidence of chronic tissue injury such as glomerular double contours compatible with transplant glomerulopathy (TPG), PTC basement membrane multilayering, interstitial fibrosis/tubular atrophy, and fibrous arterial intimal thickening. PTC basement membrane multilayering correlates highly with TPG, and most of TPG have evidence of either C4d-positive staining or DSA. However, the proposed criteria do not apply to all situations of chronic active antibody-mediated rejection. C4d is not a magic marker for antibody-mediated rejection. C4d staining is not always highly sensitive for detecting antibody-mediated rejection. Multi-institutional studies should be conducted to better understand the clinicopathological context of chronic antibody-mediated rejection. These studies should include well-designed serial protocol biopsies with evaluation by electron microscopy, C4d staining performed on frozen sections, and assessment using sensitive DSA detection methods.

A case of tacrolimus-associated thrombotic microangiopathy after ABO-blood-type-incompatible renal transplantation.

De novo thrombotic microangiopathy(TMA) is most commonly triggered by calcineurin inhibitors (CNI) and the prognosis is less severe than with recurrent TMA. However, it is difficult to distinguish de novo TMA from CNI toxicity and acute antibody-mediated rejection(AMR) soon after renal transplantation. We present a case of tacrolimus-associated TMA soon after ABO blood type incompatible renal transplantation that was difficult to differentiate from acute AMR. On day 9 his urine output decreased dramatically and the Scr level increased. His anti-blood type A antibody titer increased to ×16 postopratively and the tacrolimus trough level was higher than in our immunosuppressive regimen. Although we gave priority to anti-AMR treatment, adequate dose adjustment of tacrolimus after tacrolimus nephrotoxicity was diagnosed from graft biopsy could correct allograft dysfunction.

A rare case of esophageal cancer (T2N0) with a solitary cerebellar metastasis.

A 72-year-old man with a history of distal gastrectomy was diagnosed with esophageal cancer (EC). A subtotal esophagectomy and the residual total gastrectomy were performed via a right-sided thoracotomy and laparotomy with D2 lymph node dissection followed by reconstruction with a retrosternal right colonic interposition. The pathological diagnosis was Mt, 65 mm, moderately differentiated squamous cell carcinoma, pT2, ly0, v2, pN0, sM0, pStage II. The patient suddenly developed neurological symptoms 10 days after the operation, and brain magnetic resonance imaging detected a single solid left cerebellar tumor. This tumor was completely excised, and pathological diagnosis confirmed the tumor as an EC metastasis. He received adjuvant chemotherapy with cisplatin + 5-fluorouracil. Seven months later, he developed multiple brain metastases; however, no evidence of local recurrence or other metastatic sites was found. He died 8 months after the surgery. Solitary cerebellar metastasis from EC in which the primary tumor is T2N0 is rare, and the mechanism of this metastatic pattern is of particular interest. Our case study suggests that even if the primary tumor is in the limited stage and other metastatic sites are not identified at presentation, it seems reasonable to perform preoperative imaging of the brain for all patients with EC.

Benefits of cyclosporine absorption profiling in nephrotic syndrome: preprandial once-daily administration of cyclosporine microemulsion improves slow absorption and can standardize the absorption profile.

AIM: Cyclosporine is known to improve proteinuria in nephrotic syndrome (NS), but is also associated with drug-related renal impairment. In this case series, therapeutic drug monitoring using the absorption profile was applied to adults with NS to investigate the efficacy and safety of once-daily administration of cyclosporine microemulsion (CSAME). METHODS: Twenty patients received CSAME starting at 100-175 mg/day (1.4-3.1 mg/kg per day) once daily after breakfast. The area under the concentration-time curve up to 4 h after administration of cyclosporine (AUC(0-4 h)) was determined in each patient within 1 week after the start of CSAME treatment. Thereafter, the dose of CSAME was adjusted according to the absorption profile. RESULTS: After 6 months, treatment with CSAME improved efficacy test values compared with those prior to treatment, and the severe nephrotic state was eliminated in all patients. No changes in serum creatinine or blood urea nitrogen levels were observed. The dose of CSAME was adjusted so that AUC(0-4 h) and the peak level fell within the range of target values, resulting in a significant decrease in the mean dose of cyclosporine (P = 0.0001). Time of peak level was variable among patients, but when CSAME was administered before breakfast, good absorption was achieved in all patients. CONCLUSION: By monitoring the absorption profile in patients with NS, a once-daily administration of CSAME was used to achieve both efficacy and a reduction in total exposure to the drug. Preprandial administration provided a more stable absorption profile of cyclosporine. The authors hope this method will become standard procedure during cyclosporine treatment in these patients.

[A case of combination with proliferative cystitis and pelvic lipomatosis causing bilateral hydronephrosis].

A 70-year-old man consulted our hospital complaining of gross hematuria and bilateral hydronephrosis. Cystoscopic findings suggested non-papillary sessile tumor at the bladder neck. CT findings revealed bilateral hydronephrosis caused by the stricture of lower ureters. Tumorous structure existed between bladder and prostate. Abundant fatty tissue was observed around bladder and rectum, the shape of the bladder was distorted to inverted tear-drop and the bladder was transferred anteriorly, showing findings of pelvic lipomatosis. Urethrocystography revealed elongation of prostatic urethra and anterior displacement of the bladder. Transurethral tumor resection was performed under spinal anesthesia. Pathological diagnosis was proliferative cystitis and no malignant cells were observed. Transperineal tumor biopsy also revealed no malignant cells. The patient was followed under administration of "Saireitou" (chinese medicine) and cetirizine hydrochloride, followed by antibiotics and anti-inflammatory enzyme preparations.

Preferable operative age of choledochal dilation types to prevent patients with pancreaticobiliary maljunction from developing biliary tract carcinogenesis.

BACKGROUND: Pancreaticobiliary maljunction (PBM), which frequently accompanies choledochal dilation, is a high risk factor for biliary tract (gallbladder, bile duct) carcinoma because of the continuous reflux of pancreatic juice into the biliary tract. The aim of this study was to clarify the preferable operative age in PBM patients for the prevention of biliary tract carcinogenesis, with reference to the dilation types of bile ducts. METHODS: There were 165 PBM patients in total studied, including 92 pediatric patients (< or =15 y) (cystic, 63; spindle-like, 29; nondilation, 0) and 73 adult patients (>15 y) (cystic, 45; spindle-like, 18; nondilation, 10) who underwent operative excision of extrahepatic bile ducts or cholecystectomy. We investigated incidence by age of biliary tract malignancies and the risk according to types of dilation. RESULTS: In the pediatric group, no carcinoma case could be found preoperatively or postoperatively (mean follow-up period, 11.7 y). In the adult group, bile duct carcinomas could be detected in 6 cases of a cystic type (6 of 45; 13.3%) (3 preoperative, 3 postoperative). Among the bile duct carcinoma cases, the youngest patient was a 21-year-old woman who had undergone excision of an extrahepatic bile duct 3 years previously. Gallbladder carcinomas were detected in 16 patients: 3 of 45 cystic (6.7%), 6 of 18 spindle-like (33.3%), and 8 of 10 nondilation (80.0%), in whom the youngest patient was a 41-year-old woman with a spindle-like type. CONCLUSIONS: To prevent biliary tract carcinogenesis in PBM patients, cystic-dilated choledochus should be excised in childhood before the development to a precancerous stage. In spindle-like and nondilation types, cholecystectomy is absolutely necessary in early adulthood before age 40.

Optimal strategy of preoperative transcatheter arterial chemoembolization for hepatoblastoma.

PURPOSE: To investigate the optimal strategy of preoperative transcatheter arterial chemoembolization (TACE) for hepatoblastoma. METHODS: Between 1992 and 2001, 7 children with hepatoblastoma (aged 9 months to 13 years) underwent preoperative TACE. The chemoembolic agent used was an emulsion of pirarubicin and lipiodol. Four patients without distant metastasis underwent "primary" TACE without systemic chemotherapy. The other 3 with distant metastases underwent "delayed" TACE following systemic chemotherapy. These patients were all examined retrospectively using clinical data. RESULTS: The average dosage of lipiodol was 0.6 ml per tumor maximal diameter (cm). All the primary cases showed a significant decrease in alpha-fetoprotein (AFP) and a reduction in the tumor size. They consequently underwent a complete surgical resection and are now disease free. All the delayed cases showed a slight decrease in AFP and underwent complete surgical resection; however, two of them died of lung metastases, and the other died of a second malignancy. Regarding complications, liver dysfunction and pulmonary embolism occurred in one patient each. CONCLUSIONS: For patients without distant metastasis, regardless of the resectability of the primary tumor, TACE may be considered the initial and only preoperative treatment, and it may be repeated. For patients with distant metastases, their complete eradication with systemic chemotherapy prior to TACE is essential.

[A patient with stage IIIc ovarian cancer with massive ascites who was chemotherapy resistant showed complete response with intraperitoneal hyperthermic chemoperfusion].

Intraperitoneal hyperthermic chemoperfusion (IHCP) was performed on a stage IIIc ovarian cancer patient with massive ascites who had been chemotherapy resistant. Complete response was observed after the treatment. A 59-year-old woman with advanced ovarian cancer complained of massive refractory ascites after 2 years' history of chemotherapy. The patient received IHCP combined with surgery to remove the primary tumors and omentum. Soon after the treatment, the massive ascites disappeared and CA125 level decreased to normal. The patient remained free of ascites for 14 months after the treatment.

Nephron-sparing surgery for multilocular cyst of the kidney in a child.

Multilocular cyst of the kidney is an uncommon benign renal neoplasm. Because of its benign nature, this lesion is best managed by nephron-sparing surgery. This report describes a child who underwent a partial nephrectomy for a multilocular cyst of the kidney. After reviewing other cases, we discuss the clinical characteristics of multilocular cyst of the kidney and emphasize the cystic partially differentiated nephroblastoma as a differential diagnosis.

Endoscopic internal biliary drainage in a child with malignant obstructive jaundice caused by neuroblastoma.

We describe a 13-year-old girl who underwent insertion of a Flexima biliary stent for obstructive jaundice due to compression of the extrahepatic bile duct by an enlarged lymph node secondary to neuroblastoma. This novel endoscopic internal biliary drainage procedure was safe and effective even for a child, and improved her quality of life. We further review other treatment options available for malignant obstructive jaundice in children.

A case of ileosigmoid knotting in a child.

Ileosigmoid knotting is an unusual condition producing a strangulated ileus in which a loop of the ileum encircles the loop of the sigmoid colon and then knots on itself. It is relatively common in many parts of Africa and tends to occur in adults; however, it is rare in developed countries, especially in children. Only 3 previous cases have been reported in children, and the authors report the fourth in a 6-year-old boy, which is the first case reported in Japan.

Strategy of management for congenital biliary dilatation in early infancy.

PURPOSE: The aim of this study was to establish the optimal management strategy of congenital biliary dilatation (CBD) in early infancy. METHODS: Over the last 15 years, 14 patients with CBD in early infancy (within 5 months), including 3 antenatally diagnosed patients, were treated in the authors' department. Of the 14 patients, 7 (50%) underwent early definitive surgery (E group), and the other 7 (50%) underwent delayed primary definitive surgery after percutaneous transhepatic cholangiodrainage (PTCD; D group). Both groups were compared retrospectively using clinical data. RESULTS: Pretreatment status and backgrounds of the patients were clinically homogeneous between the 2 groups. The total length of hospital stay was significantly longer in the D group. As short-term complications, 1 patient in the E group was compromised with hepatolithiasis, and 3 patients in the D group were compromised with catheter-related complications. Other clinical data, such as age at definitive surgery, blood loss, pathologic fibrosis of the liver, jaundice-free day, and long-term complications were not significantly different between the 2 groups. CONCLUSIONS: The authors propose that the standard of management should be early definitive surgery with wide anastomosis before 2 months of age. However, PTCD might be used under strict consideration of indication and careful management for patients with extremely poor surgical risk.

Endosonographic evaluation in two children with esophageal stenosis.

The authors report the successful use of endoscopic ultrasonography (EUS) for finding the etiology and subsequent treatment strategy for esophageal stenosis in 2 children. In case 1, EUS showed anterior wall thickening and multiple low echoic regions in the mp layer. These regions were believed to be cartilage. Esophageal resection therefore was performed. In case 2, EUS showed disruption of the sm and mp layers at the stenosis, leading us to speculate that the stenosis was caused by gastroesophageal reflux. After balloon dilatation, he underwent antireflux surgery of Nissen's fundoplication. EUS was useful for determining the etiology of esophageal stenosis and, thus, the appropriate treatment strategy.