Prednisolone administration monitored by postoperative stool color achieves high jaundice clearance after laparoscopic portoenterostomy for biliary atresia.

PURPOSE: Stool color (SC) for monitoring prednisolone use in biliary atresia (BA) patients after laparoscopic portoenterostomy (LPE) was reviewed. METHODS: Subjects were 47 post-LPE BA patients given a reducing dose course of intravenous prednisolone. The course started at 4 mg/kg/day and gradually reduced, ultimately reaching a final total dose (TD) of 31.5 mg/kg. Normal SC indicated a course could progress until finished and was repeated until jaundice clearance (JC) was achieved. Abnormal SC persisting for two consecutive courses was the absolute indication for redo or liver transplantation (LTx). RESULTS: JC was achieved in 38/47 (80.9%) LPE cases and 4/6 redos to give an overall JC rate (JCR) of 42/47 (89.4%). Outcomes after one course (n = 5; JCR: 80.0%; median TD: 30.0 mg/kg, interquartile range [IQR: 26.0-31.5]), two courses (n = 10; JCR: 90.0%; median TD: 62.5 mg/kg [IQR: 60.8-66.0]), three courses (n = 13; JCR: 92.3%; median TD: 90.0 mg/kg [IQR: 86.0-90.0]), four courses (n = 10; JCR: 80.0%; median TD: 120.0 mg/kg [IQR: 116.7-123.3]), five courses (n = 7; JCR: 100%; median TD: 156.0 mg/kg [IQR: 154.3-157.5]), six courses (n = 1; JCR: 100%; TD: 189.0 mg/kg), ten courses (n = 1; JCR: 100%; TD: 308 mg/kg). CONCLUSION: Indications for repeat prednisolone and timing of redo/LTx based on SC monitoring appeared effective based on high JCR and successful redo/LTx. LEVELS OF EVIDENCE: III.

Comparison of robotic assistance and laparoscopy for pediatric choledochal cyst: advantages of robotic assistance.

AIM: Surgery for pediatric choledochal cyst (CC), complete excision (CE), and Roux-en-Y hepaticojejunostomy anastomosis (HJA) can be performed using laparoscopy (Lap), robotic-assistance (Rob; da Vinci Xi/Si), or both (Lap/Rob). METHODS: Lap was used exclusively between 2009 and 2021 (n = 31) and Rob was introduced in 2017 (n = 23). All subjects were matched for age, weight, BMI, and episodes of preoperative pancreatitis. For Rob, the first 15/23 were Lap-CE/Rob-HJA and the last 8/23 were Rob-CE/Rob-HJA. RESULTS: Total anastomotic time (TAT), TAT per suture during HJA, and time taken for dissection during CE were significantly shorter with less variance for Rob, although overall operative times were similar. Serum amylase on postoperative days 3, 5, and 7 were significantly higher for Lap. Times taken to ambulate, for return of bowel sounds, and discharge home were all significantly shorter for Rob. All postoperative complications occurred after Lap; HJA leak (n = 1; 3.2%), HJA stricture (n = 1; 3.2%), both treated by open re-HJA; and pancreatic fistula (n = 6; 19%), all treated conservatively. CONCLUSION: Dissection and recovery were faster with Rob while overcoming Lap-associated shortcomings to prevent complications associated with suturing. Both CE and HJA were safer and more reliable with Rob, a reflection of Rob's superiority.

Appropriate tension sensitivity of α-catenin ensures rounding morphogenesis of epithelial spheroids.

The adherens junction (AJ) is an actin filament-anchoring junction. It plays a central role in epithelial morphogenesis through cadherin-based recognition and adhesion among cells. The stability and plasticity of AJs are required for the morphogenesis. An actin-binding α-catenin is an essential component of the cadherin-catenin complex and functions as a tension transducer that changes its conformation and induces AJ development in response to tension. Despite much progress in understanding molecular mechanisms of tension sensitivity of α-catenin, its significance on epithelial morphogenesis is still unknown. Here we show that the tension sensitivity of α-catenin is essential for epithelial cells to form round spheroids through proper multicellular rearrangement. Using a novel in vitro suspension culture model, we found that epithelial cells form round spheroids even from rectangular-shaped cell masses with high aspect ratios without using high tension and that increased tension sensitivity of α-catenin affected this morphogenesis. Analyses of AJ formation and cellular tracking during rounding morphogenesis showed cellular rearrangement, probably through AJ remodeling. The rearrangement occurs at the cell mass level, but not single-cell level. Hypersensitive α-catenin mutant-expressing cells did not show cellular rearrangement at the cell mass level, suggesting that the appropriate tension sensitivity of α-catenin is crucial for the coordinated round morphogenesis.Key words: α-catenin, vinculin, adherens junction, morphogenesis, mechanotransduction.

Potentiation of Muscarinic M3 Receptor Activation through a New Allosteric Site with a Novel Positive Allosteric Modulator ASP8302.

Muscarinic M3 (M3) receptors mediate a wide range of acetylcholine (ACh)-induced functions, including visceral smooth-muscle contraction and glandular secretion. Positive allosteric modulators (PAMs) can avoid various side effects of muscarinic agonists with their spatiotemporal receptor activation control and potentially better subtype selectivity. However, the mechanism of allosteric modulation of M3 receptors is not fully understood, presumably because of the lack of a potent and selective PAM. In this study, we investigated the pharmacological profile of ASP8302, a novel PAM of M3 receptors, and explored the principal site of amino-acid sequences in the human M3 receptor required for the potentiation of receptor activation. In cells expressing human M3 and M5 receptors, ASP8302 shifted the concentration-response curve (CRC) for carbachol to the lower concentrations with no significant effects on other subtypes. In a binding study with M3 receptor-expressing membrane, ASP8302 also shifted the CRC for ACh without affecting the binding of orthosteric agonists. Similar shifts in the CRC of contractions by multiple stimulants were also confirmed in isolated human bladder strips. Mutagenesis analysis indicated no interaction between ASP8302 and previously reported allosteric sites; however, it identified threonine 230 as the amino acid essential for the PAM effect of ASP8302. These results demonstrate that ASP8302 enhances the activation of human M3 receptors by interacting with a single amino acid distinct from the reported allosteric sites. Our findings suggest not only a novel allosteric site of M3 receptors but also the potential application of ASP8302 to diseases caused by insufficient M3 receptor activation. SIGNIFICANCE STATEMENT: The significance of this study is that the novel M3 receptor positive allosteric modulator ASP8302 enhances the activation of human M3 receptor by interacting with a residue distinct from the reported allosteric sites. The finding of Thr230 as a novel amino acid involved in the allosteric modulation of M3 receptors provides significant insight into further research of the mechanism of allosteric modulation of M3 and other muscarinic receptors.

Impact of sarcopenia in infants with liver transplantation for biliary atresia.

While sarcopenia is an important predictor of LT outcomes in adults, few studies have examined the association of sarcopenia with LT outcomes in pediatric patients. We investigated the clinical influence of sarcopenia on the post-transplant outcomes in infants with BA. To define sarcopenia in infants, the cross-sectional area of the tPMA in 93 healthy control infants was measured by computed tomography. Sarcopenia was defined as a tPMA lower than two standard deviations below the mean of healthy control infants. Eighty-nine infants with BA with a median age at LT of 7.6 months old were enrolled. The clinical characteristics and outcomes of LT were verified in the sarcopenia group (n = 21) and non-sarcopenia group (n = 68). The sarcopenia group had a significantly longer operation time and greater blood loss during LT than the non-sarcopenia group (P = .03 and 0.02). The incidence of portal vein stenosis and post-operative bloodstream infection was also significantly higher in the sarcopenia group than in the non-sarcopenia group (23.8% vs 4.4%, P = .02 and 28.6% vs 10.3%, P = .04, respectively). The total length of hospital stay did not differ significantly. The 1-year patient and graft survival rates tended to be lower in the sarcopenia group than in the non-sarcopenia group (90.5% vs 98.5%, P = .07 and 85.7% vs 97.1%, P = .05, respectively). Sarcopenia in infants with BA may be associated with the patient survival and serve as an effective marker for post-operative outcomes of LT.

Efficacy of intraoperative cine-portogram for complicated portal vein reconstruction in pediatric living donor liver transplantation.

Sufficient PV flow is necessary to achieve successful PV reconstruction in pediatric LDLT. IOCP can be used to assess the severity of PV stenosis and to identify potential portosystemic collateral pathways. The present study reviewed the utility of IOCP and the outcomes of patients who underwent assessment with an IOCP. Consecutive primary LDLTs were performed in 488 pediatric recipients between November 2005 and October 2019. IOCP was used in patients who were unable to achieve sufficient PV flow after the ligation of collaterals. In total, 11 patients underwent IOCP to assess potential portosystemic collateral pathways. The median age and body weight was 8 months (IQR, 6-11 months) and 6.6 kg (IQR, 5.7-8.9 kg), respectively. The reasons for using the IOCP were recurrent PV thrombus in seven patients and insufficient PV flow in four patients. IOCP revealed remaining collaterals in six patients and residual hypoplastic PV in eight patients. Two patients required additional interruption of the potential collaterals under IOCP, which were unable to be recognized as a dominant portosystemic collateral pathway on preoperative imaging. All eight patients with residual hypoplastic PV required vein graft interposition for the complete removal of the hypoplastic PV. All the patients are currently doing well with a median follow-up period of 4.9 years (IQR, 2.2-5.6 years). IOCP can be an effective tool for precisely detecting occult portosystemic collateral pathways and for assessing the patency of the PV anastomosis in pediatric LDLT.

Impact of prolonged clamping of the portal vein during liver transplantation in infants with biliary atresia: renewed interest in a long-standing issue.

PURPOSE: Various approaches to portal vein (PV) reconstruction have been described; however, there is still scope to improve the safety and efficiency of how PV anastomosis is performed in infants. METHODS: We analyzed, retrospectively, the predictive factors for PV reanastomosis during living donor liver transplantation (LDLT) in 117 infants (< 1 year old) with biliary atresia (BA). RESULTS: Twenty-six infants required PV reanastomosis because of intraoperative PV thrombosis in 21 and insufficient PV flow in 5. Univariate analysis identified multiple previous laparotomies and a prolonged anhepatic phase (AHP) as significant risk factors for repeated PV anastomosis. The AHP cutoff value for identifying patients requiring PV reanastomosis was 134 min. Multivariate analysis revealed prolonged AHP > 134 min (odds ratio, 15.98; 95% confidence interval, 5.05-50.6; P < 0.001) as an independent risk factor for repeated PV anastomosis. The plasma D-dimer and fibrinogen degradation product concentration just after reperfusion were significantly higher in patients requiring PV reanastomosis (P < 0.001). Patients requiring reanastomosis of the PV had a higher incidence of PV complications after LDLT than those who did not need PV reanastomosis (P = 0.01). CONCLUSION: Attempting to minimize the AHP is indispensable for successful LDLT in infants with BA.

The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup "o" biliary atresia.

PURPOSE: The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA. METHODS: Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT. RESULTS: All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup "o", in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct. CONCLUSIONS: Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.

Comparative study of open and laparoscopic Kasai portoenterostomy in children undergoing living donor liver transplantation for biliary atresia.

PURPOSE: The effectiveness of laparoscopic Kasai portoenterostomy (Laparoscopic-KPE) for biliary atresia (BA) has been reported but remains controversial. We reviewed our own cases and cases described in previous studies of liver transplantation (LT) for BA after Laparoscopic-KPE to investigate the efficacy of Laparoscopic-KPE. METHODS: Subjects were children of ≤ 2 years old with LT for BA after KPE who underwent Laparoscopic-KPE (n = 10) or Open-KPE (n = 115) between 2009 and 2020. Propensity score matching was performed to reduce the effect of treatment selection bias. The clinical data regarding the preoperative characteristics and surgical results were compared. RESULTS: The rates of hypoplastic portal vein and retrograde portal vein flow were lower in the Laparoscopic-KPE group than in the Open-KPE group (0 vs. 40.0%, p = 0.02 and 0 vs. 35.0%, p = 0.04). There was no marked difference in the operation time or duration of hepatectomy. For portal vein reconstruction, a vein graft was not required in the Laparoscopic-KPE group (0 vs. 35.0%, p = 0.03). No patients in the Laparoscopic-KPE group developed portal vein complications or required re-laparotomy for bowel perforation or re-bleeding, in contrast to the Open-KPE group (0 vs. 15.0% and 0 vs. 10.0%, respectively). CONCLUSION: Laparoscopic-KPE may reduce postoperative complications that necessitate re-laparotomy in LT.

Living-donor liver transplantation for liver hemorrhaging due to peliosis hepatis in X-linked myotubular myopathy: Two cases and a literature review.

X-linked myotubular myopathy (MTM) (OMIM 310400) is a severe neuromuscular disorder caused by mutations in the myotubularin (MTM1) gene. Liver hemorrhaging due to peliosis hepatis (PH) is a fatal complication. We herein report 2 successful cases of living-donor liver transplantation (LDLT) for MTM patients due to liver hemorrhaging caused by PH and review previous reports. A boy who was 9 years and 4 months old initially underwent left lateral segmentectomy due to massive hepatic and intraperitoneal hemorrhaging. As bleeding from the remnant liver continued after hepatectomy, this patient emergently underwent LDLT using a left lateral segment graft from his father. Another boy who was 1 year and 7 months old underwent transcatheter arterial embolization due to hepatic hemorrhaging and was referred to our hospital for LDLT using a left lateral segment graft from his father. The pathological findings in both cases showed sinusoidal dilatation with degenerative changes in reticular fiber and hematoma in the explanted liver, which were consistent with PH associated with MTM. LT should be considered as a treatment option for patients with episodes of hepatic hemorrhaging due to MTM in order to protect against fatal bleeding.

Outcome for Pediatric Recipients of Macrosteatotic Liver Grafts From Living Donors.

In deceased donor liver transplantation, a donor liver with moderate (>30%) macrosteatosis used to be considered inappropriate for grafting. We examined the outcomes of children who underwent living donor liver transplantation (LDLT) at the National Center for Child Health and Development whose donor livers had moderate-to-severe macrosteatosis. Twelve children were enrolled who had received a moderate-to-severe macrosteatotic liver graft and underwent liver biopsy soon after LDLT. The primary diseases were biliary atresia in 7 patients, acute liver failure in 3 patients, glycogen storage disease type 1 in 1 patient, and primary sclerosing cholangitis in 1 patient. Median age was 11 months. There were 4 recipients who received grafts from their fathers, and 8 received grafts from their mothers. Median donor age was 35.5 years. We compared the degree of donor liver steatosis with the results of graft liver biopsies that were collected 4-105 days after LDLT. The levels of donor liver macrovesicular steatosis were moderate (34%-66%) in 9 patients and severe (>66%) in 3 patients. The nonalcoholic fatty liver disease activity score was 3 in 7 patients and 4 in 5 patients. Shortly after LDLT, 11 of 12 patients showed improvement in steatosis compared with the donor livers. One biopsy specimen taken 22 days after LDLT showed 60% macrosteatosis, which was the same as that in the donor liver. However, this patient was alive and well 6 years after LDLT. One patient died after LDLT because of infection and respiratory failure. The levels of steatosis of the donor liver grafts improved soon after LDLT in children, and the outcomes of children receiving a moderate-to-severe macrosteatotic liver from their parents were excellent.

Longterm Outcomes of Living Donor Liver Transplantation for Glycogen Storage Disease Type 1b.

Glycogen storage disease (GSD) type 1b (Online Mendelian Inheritance in Man [OMIM] 232220) is an autosomal recessive inborn error of carbohydrate metabolism caused by defects in glucose-6-phosphate translocase. GSD1b patients have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, and neutropenia. Liver transplantation (LT) has been indicated for severe glucose intolerance, poor metabolic control (PMC), and poor growth (PG). We retrospectively reviewed 11 children with GSD1b who underwent living donor liver transplantation (LDLT) at the National Center for Child Health and Development in Tokyo, Japan. Between November 2005 and December 2018, 495 children underwent LDLT with an overall 10-year patient and graft survival of 90.6% and 88.9%, respectively. Of these, LT was indicated for 11 patients with GSD1b. All patients are doing well with the stabilization of glucose intolerance and decreased hospitalization for infectious complications. Demand for granulocyte colony-stimulating factor significantly decreased. However, although LT stabilized the blood glucose level, the platelet function was not improved. The posttransplant developmental quotient (DQ) remained similar to the pretransplant DQ without deterioration. LDLT is a feasible procedure for GSD1b patients with regard to the longterm prognosis. LT should be considered for patients with severe glucose intolerance to protect the cognitive function against hypoglycemic encephalopathy and to ameliorate PMC and PG.

Sequentially timed all-optical mapping photography boosted by a branched 4f system with a slicing mirror.

We present sequentially timed all-optical mapping photography (STAMP) with a slicing mirror in a branched 4f system for an increased number of frames without sacrificing pixel resolution. The branched 4f system spectrally separates the laser light path into multiple paths by the slicing mirror placed in the Fourier plane. Fabricated by an ultra-precision end milling process, the slicing mirror has 18 mirror facets of differing mirror angles. We used the boosted STAMP to observe dynamics of laser ablation with two image sensors which captured 18 subsequent frames at a frame rate of 126 billion frames per second, demonstrating this technique's potential for imaging unexplored ultrafast non-repetitive phenomena.

Urinary metabolites identified using metabolomic analysis as potential biomarkers of nocturia in elderly men.

PURPOSE: To investigate the association between nocturia and urinary metabolites in elderly men using metabolomic analysis. METHODS: We recruited 66 men aged 65-80 years. The 3-day frequency volume chart (FCV), International Prostate Symptom Score (IPSS), and quality of life score were used to assess micturition behavior. Participants with the total IPSS > 0 and ≥ 1.5 micturition on an average for three nights were included in the nocturia group. Participants with the total IPSS < 8 and < 1.5 micturition at night were included in the control group. We conducted a comprehensive metabolomic analysis of urine samples. Metabolites were compared between the groups using an unpaired t test. A multivariable logistic regression analysis was used to determine the relationship between nocturia and these metabolites. RESULTS: The nocturia and control groups consisted of 45 and 21 men, respectively. There were no differences in the background factors between the groups except for receiving anticholinergic drug and having life style-related diseases. The FVC revealed that nocturnal urine volume, 24 h micturition frequency, and nocturnal micturition frequency were significantly higher in the nocturia group than in the control group. The metabolomic analysis revealed 16 metabolites, which were differentially expressed between the groups. The multivariate analysis showed that increased serotonin level and decreased 3-hydroxypropionic acid and 3-indoleacetonitrile levels were associated with nocturia. CONCLUSIONS: These findings suggest that abnormal urinary metabolites including serotonin, 3-hydroxypropionic acid, and 3-indoleacetonitrile are involved in the pathogenesis of nocturia in elderly men.

Etiology of liver dysfunction after liver transplantation in children with metabolic disorders.

AIM: It has been reported that the long-term outcome for children with metabolic disorders after liver transplantation (LT) is excellent. However, there are several reports citing LT patients with metabolic disorders developing liver dysfunction early after LT. We examined the pathogenesis of liver dysfunction observed after LT in recipients with metabolic disorders at the National Center for Child Health and Development. METHODS: Of 106 children (aged <18 years) with metabolic disorders who underwent LT at our center, 36 patients who underwent liver biopsy within 60 days after LT were enrolled. The underlying diseases were urea cycle disorders (14 patients), methylmalonic acidemia (11 cases), Wilson's disease (3 patients), mitochondrial hepatopathy (3 patients), and others (5 patients). The median age was 1 year 2 months at LT. The reasons for biopsy were liver dysfunction (31 patients) and ascites (5 patients). RESULTS: The main findings of graft liver biopsy were diffuse steatosis (21 patients), rejection (8 patients), infection (3 patients), and others (4 patients). Of 21 patients who received graft biopsy showing steatosis, all the donor livers originally showed no steatosis or only mild steatosis. The liver function improved immediately after biopsy in 18 of 21 patients that showed diffuse steatosis. CONCLUSIONS: The major cause of liver dysfunction after LT in recipients with metabolic disorders was steatosis and the risk of rejection was low. It is important to take a liver biopsy and examine the cause of liver dysfunction to avoid administration of excess immunosuppressant, and select the right therapy for recipients with metabolic disorders.

Late T cell-mediated rejection may contribute to poor outcomes in adolescents and young adults with liver transplantation.

Although poor long-term graft survival in LT in AYA is recognized, detailed epidemiological data are still lacking. L-TCMR may have poor outcomes. This study aimed to provide a detailed, epidemiological assessment of the association between AYA age and rejection. L-TCMR was defined in this study as TCMR with central vein or perivenular inflammation occurring later than 3 months after LT. A total of 342 patients who survived for at least 3 months after LT between 2005 and 2015 were enrolled. The AYA group (10-24 years) was compared with the C group (less than 10 years), and the incidence and outcomes of L-TCMR were analyzed. In total, 342 patients had LT; 38 of these were AYA with the mean follow-up period of 6.7 years. A total of 304 patients in C group had a mean follow-up period of 6.3 years (P = .28). The incidence of L-TCMR in AYA group was significantly higher than in C group (15.8% vs 4.6%, P = .006). The time to L-TCMR after LT was significantly shorter in AYA group (P = .01). Neither patient survival nor the incidence of non-adherence differed significantly between the groups (P = .18 and P = .89). The number of additional immunosuppressants after L-TCMR was significantly higher in the AYA group (P = .04). A high incidence of L-TCMR was observed in AYA group irrespective of non-adherence. AYA patients with L-TCMR should be followed carefully due to the poor results of post-treatment biopsy and the need for intensive immunosuppressive therapy.

Living donor liver transplantation for congenital hepatic fibrosis in children.

Congenital hepatic fibrosis (CHF) often accompanies autosomal recessive polycystic kidney disease (ARPKD), which stems from a PKHD1 gene mutation. The aim of this study was to clarify the prognosis of children with CHF who received living donor liver transplantation (LDLT) from donors who might be heterozygous carriers of a hepatorenal fibrocystic disease. Fourteen children with CHF who underwent LDLT at our center were enrolled. Eight and two patients had ARPKD and nephronophthisis, respectively. Eight of the donors were the recipients' fathers, and six donors were their mothers. We examined the histological and radiological findings of the donor livers and complications in the recipients following the liver transplantation. Seven of the donor livers presented morphological abnormalities of the bile ducts. Abdominal computed tomography revealed liver cysts in eight donors. One recipient underwent re-LT for graft failure due to rejection. Three patients presented with rejection, and one presented with sepsis. The overall survival rate was 100% and the original graft survival rate was 93%. In conclusion, the prognosis of recipients who received a LDLT from their parents for CHF was excellent. However, the morphology of half the donor livers was abnormal. Careful follow-up is needed to ensure long-term graft survival.

Atypical rectoscrotal and rectoperineal fistula in male anorectal malformations: a case series of non-detaching strategies for preventing urethral injury.

Anorectal malformations (ARM) represent a broad spectrum of patients with different level of the rectum and type of a fistula. Standardized approaches are usually successful, but patients occasionally present with an unusual course of fistula which requires a modified surgical strategy. We present here three male ARM patients with an atypical fistula which did not have connection with the urinary tract, but ran near the fistula. Case 1 has a low-type ARM with a rectoscrotal fistula running deep and partly involved in the corpus spongiosum. Anorectoplasty was performed through an anterior sagittal incision and the anterior wall of the fistula was laid open leaving the posterior wall undetached. Case 2 was diagnosed with an intermediate-type ARM with a long rectoscrotal fistula running near and parallel the urethra. Posterior sagittal anorectoplasty (PSARP) was performed leaving the fistula untouched. Case 3 presented with an intermediate-type ARM with a rectoperineal fistula adherent to the urethra. The patient was treated by PSARP leaving the fistula and part of the muscle coat of the rectum in situ. All the cases were smoothly discharged and no urological complication nor problem associated with the residual fistula was observed at the latest follow-up (17 months-2 years). Preoperative distal colostography with the aid of diverting colostomy was importantly useful for deciding surgical procedure.

Technical Considerations in Liver Transplantation for Biliary Atresia With Situs Inversus.

In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. We evaluated the surgical procedures and the outcomes in our patients and in the previously reported patients undergoing LT for BA with SI. Between November 2005 and October 2018, 235 children underwent LT with an overall 10-year graft survival of 94.7%. Of these, 6 (2.6%) patients received LT for BA with SI. Living donor liver transplantation (LDLT) was applied in all patients. Vascular anomalies at our center included the absence of the inferior vena cava (IVC; n = 5), a preduodenal portal vein (PV; n = 4), and an aberrant hepatic artery (HA; n = 1). Hepatic vein (HV) outflow obstruction occurred in 1 patient with an absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in 1 patient with a preduodenal PV at 5 months after LDLT. The overall survival of our patients was 80.0% at 3 years. In the literature, 49 patients with LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction (2.0%), PV complications (10.2%), and HA thrombosis (4.1%). All PV complications occurred in LDLT patients with a preduodenal PV (5/23; 21.7%). In conclusion, the detailed assessment of the surgical procedures and the systematic literature review demonstrated excellent outcomes after LT for BA with SI. Liver recipients with a preduodenal PV are exposed to a higher risk for vascular complications after a very challenging LT procedure with overall good outcomes.

Elliptic neutron-focusing supermirror for illuminating small samples in neutron reflectometry.

This paper details the development of a precise assembly of two supermirrors for neutron-focusing, designed for installation in neutron reflectometer SOFIA at BL16 in J-PARC MLF to intensify the illumination for small samples. The supermirrors are sputtered on two metal substrates, whose surfaces are coated with amorphous Ni-P plating, and are figured by diamond cutting and polished to subnanometer roughness. Special care is taken while polishing the substrates to reduce waviness and surface roughness for achieving a sharp focusing spot and uniform neutron reflectivity. The supermirror could converge the neutrons into a focal spot with a width of 0.13 mm in the full width at half maximum.