Successful Treatment for Hepatoblastoma in Trisomy 18: A Case Report.

Children with trisomy 18 tend to develop hepatoblastoma. Since the introduction of appropriate management for organ malfunction, individuals with trisomy 18 have come to have a longer life expectancy. However, the predisposition to hepatoblastoma becomes a significant issue for the quality of a case. Here, we present a rare multifocal hepatoblastoma involving predominantly Couinaud segments 5 and 7 in a 10-month-old boy with trisomy 18. Though the first-line cisplatin monotherapy resulted in unsatisfactory tumor shrinkage, the second-line neoadjuvant chemotherapy administrating irinotecan and vincristine gave rise to significant tumor reduction in volume, leading to the completion of partial resection of the liver without the microscopic residual disease. The patient has been free from recurrence for 44 months. Because anatomical right hepatectomy can cause circulatory instability, including acute onset of pulmonary hypertension in trisomy 18 patients, physicians should balance treatment benefits and potential adverse effects. Our successful experience utilizing a combination of efficacious and less cardiotoxic neoadjuvant chemotherapy followed by the partial hepatectomy encourages physicians to treat a patient with trisomy 18 and tackle hepatoblastoma with a genetic background.

Optimal Surgical Method and Timing for Low-birth-weight Esophageal Atresia Babies: Multi-institutional Observational Study.

AIM OF THE STUDY: Previous research has shown that low birth weight is one of the risk factors for esophageal atresia. However, there remains a paucity of evidence on the timing and the treatment method. METHOD: Data were collected using a multi-institutional observational study in 11 hospitals that performed surgeries on esophageal atresia babies whose birth weights were ≤1500 g from 2001 to 2020. RESULTS: Of the 46 patients analyzed, median birth weight was 1233 (IQR 1042-1412) g. Within 46 cases, 19 (41%) underwent definitive esophageal anastomosis at the median of age in 8 (IQR 2-101) days. Thirteen out of 19 experienced either closure of tracheoesophageal fistula, gastrostomy, or esophageal banding at the first operation, followed by esophageal anastomosis. Seven infants, including four cases of <1000 g, underwent anastomosis after one month of age to wait for weight gain (variously 2-3000 g). Twenty-one out of 27 infants (78%) who did not receive anastomosis died within one year of age, including 21 (78 %) with major cardiac anomalies and 24 (89%) with severe chromosomal anomalies (trisomy 18). Six survivors in this group, all with trisomy 18, lived with palliative surgical treatments. CONCLUSION: In our study, the definitive esophageal anastomosis was effective either at the first operation or as a later treatment after gaining weight. Although having severe anomalies, some infants receive palliative surgical treatments, and the next surgery was considered depending on their condition. EVIDENCE LEVEL: II.

Prospective evaluation of common hepatic duct histopathology at the time of choledochal cyst excision ranging from children to adults.

PURPOSE: To evaluate common hepatic duct just distal to the HE anastomosis (d-CHD) prospectively for mucosal damage, inflammation, fibrosis, dysplasia, carcinoma in situ, malignant transformation, effects of serum amylase, and symptoms at presentation in CC cases ranging from children to adults. METHODS: Cross-sections of d-CHD obtained at cyst excision 2018-2023 from 65 CC patients; 40 children (< 15 years old), 25 adults (≥ 15) were examined with hematoxylin and eosin, Ki-67, S100P, IMP3, p53, and Masson's trichrome to determine an inflammation score (IS), fibrosis score (FS), and damaged mucosa rate (DMR; damaged mucosa expressed as a percentage of the internal circumference). RESULTS: Mean age at cyst excision ("age") was 18.2 years (range: 3 months-74 years). Significant inverse correlations were found for age and DMR (p = 0.002), age and IS (p = 0.011), and age and Ki-67 (p = 0.01). FS did not correlate with age (p = 0.32) despite significantly increased IS in children. Dysplasia was identified in a 4-month-old girl with cystic CC. Serum amylase was elevated in high DMR subjects. CONCLUSIONS: High DMR, high IS, and evidence of dysplasia in pediatric CC suggest children are at risk for serious sequelae best managed by precise histopathology, protocolized follow-up, and awareness that premalignant histopathology can arise in infancy.

A rare case of a toddler with unilateral cryptorchidism-related cranial suspensory ligament remnant.

Key clinical message: Several animal experiment studies have shown that insufficient testicular descent to the scrotum can be caused by persistence of cranial suspensory ligament (CSL). We report a case of right cryptorchidism in a male toddler surgically treated with an orchidopexy possibly associated with CSL persistence based on intraoperative and pathological findings. This case would be a precious source to further investigate the etiopathogenesis of cryptorchidism. Abstract: The CSL anchors embryonic gonads to the dorsal abdominal wall during antenatal mammalian development. Although its persistence appears to cause cryptorchidism in animal models, it has never been proven in humans. A 1-year-old boy with right cryptorchidism underwent right orchidopexy. Intraoperatively, a band-like structure running from the right testis into the retroperitoneum and up to the right side of the liver was noticed and resected. The pathological findings of the specimen showed fibrous connective tissues, smooth muscles, and blood vessels but no tissues suggestive of a testis, a spermatic cord, an epididymis, or liver. Immunohistochemical analysis for an androgen receptor antibody did not detect any signal in the specimen. The right cryptorchidism in this case was possibly caused by CSL persistence, which is the first such human case, to our knowledge.

Circulating maternal chimeric cells have an impact on the outcome of biliary atresia.

Introduction: We aimed to quantify the DNA of maternal chimeric (MC) cells in the peripheral blood of the BA patients and investigated the impact on the outcome. Methods: Patients with progressive jaundice because of no bile flow, which necessitated liver transplantation, or who showed inadequate bile flow with or without episodes of cholangitis and progressive hepatic fibrosis and portal hypertension were classified into the poor group. Those with adequate bile flow with completely normal liver function tests beyond 2 years were classified into the good group. The qPCR were separately carried out in buffy coat samples and plasma samples, targeting the non-inherited maternal HLA alleles in the DNA samples. Results: MC-DNA was present in the buffy coat (10-328 gEq per 106 host cells) in seven patients. There was no MC-DNA in the remaining five patients. MC-DNA (214-15,331 gEq per 106 host cells) was observed in the plasma of five patients. The quantity of MC-DNA in the buffy coat showed a significant difference between the two prognostic groups (p = 0.018), whereas there was no significant difference in the quantity of MC-DNA in plasma (p = 0.205). MC-DNA in the buffy coat was significantly associated with the outcome (p = 0.028), whereas MC-DNA in the plasma did not influence the outcome (p = 0.56). Conclusions: Poor outcomes in BA were correlated with circulating maternal chimeric lymphocytes.

Quality of life after type-A esophageal atresia surgery: changes over time and effect of pre-anastomotic elongation.

AIM: To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients. METHODS: Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS). RESULTS: There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant. CONCLUSION: Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

Changes in postoperative quality of life of pediatric total colonic aganglionosis patients: effect of pull-through technique.

AIM: To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients. METHODS: Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF. RESULTS: There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores. CONCLUSION: MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

Acute renal failure due to severe bilateral ureteropelvic junction obstruction treated by urinary drainage in a 2-year-old infant.

INTRODUCTION: Conscientious follow-up is essential for bilateral grade 4 hydronephrosis with ureteropelvic junction obstruction to ensure optimal surgical timing. We have reported a case of a male infant who required emergent urinary drainage due to severe bilateral ureteropelvic junction obstruction-derived acute renal failure. CASE PRESENTATION: Bilateral grade 4 hydronephrosis was diagnosed in a male neonate. Vesicoureteral reflux was ruled out. Two years after the initial diagnosis, he developed acute renal failure and underwent bilateral emergent urinary drainage, followed by multiple urinary tract reconstructions against left ureterovesical junction stenosis and bilateral ureteropelvic junction obstruction. The postoperative renogram demonstrated a bilateral nonobstructive pattern. CONCLUSION: Bilateral emergency drainage for acute renal failure was successful without hemodialysis. Unilateral drainage or pyeloplasty should be planned early for bilateral grade 4 hydronephrosis with ureteropelvic junction obstruction to avoid lethal events if the obstruction pattern with decreased split renal function is <40% or if it is symptomatic.

Recovery of bowel function after transperitoneal or retroperitoneal laparoscopic pyeloplasty. A multi-center study.

AIM: To document the recovery of bowel function (BF) in children after transperitoneal (TP) or retroperitoneal (RP) laparoscopic pyeloplasty. METHODS: Data were obtained retrospectively from four centers between 2008 and 2019 for TP (n = 51) and RP (n = 58). Each surgeon chose which technique to perform. RESULTS: Subject demographics were not significantly different. Differences in operative times were not significant (RP: 241 min versus TP: 225 min). Mean duration/requirement for postoperative epidural/intravenous analgesia were not significantly different (TP: 1.4 days versus RP: 1.3 days) and (TP: 66.7% versus RP: 67.2%), respectively. Postoperative nasogastric (NG) intubation was more common in RP (TP: 19.6% versus RP: 44.8%; p < .05). NG aspiration (TP: 0.15 mL/kg/hr versus RP: 0.16 mL/kg/hr), nausea (TP: 31.4% versus RP: 17.2%), and vomiting (TP: 19.6% versus RP: 15.5%) were not significantly different. There were no perioperative complications (including ileus). Abdominal distention was problematic in one case per group (TP: 2.0% versus RP: 1.7%). Times for oral liquid (TP: 0.69 day versus RP: 0.83 day), solid food (TP: 0.88 day versus RP 1.07 days), and the first bowel movement (TP: 2.86 days versus RP: 2.79 days), were not significantly different. CONCLUSIONS: BF recovery would appear to be consistent, independent of technique.

Successful closure of a large abdominal wall defect using endoscopic component separation technique in an infant with a giant ventral hernia after staged surgery for omphalocele.

BACKGROUND: The management of large abdominal wall defects, such as omphalocele or gastroschisis, remains a challenge for pediatric surgeons. Though several techniques have been described to repair those conditions, there is no procedure considered to be the standard worldwide. We report an infant girl with a giant ventral hernia after staged surgery for omphalocele in whom delayed closure of a large abdominal wall defect was performed successfully using "endoscopic component separation technique (ECST)" without serious complications. CASE PRESENTATION: A baby girl was admitted to our hospital because of a giant omphalocele, which had been prenatally diagnosed. The omphalocele was supraumbilical and included the entire liver. After staged surgery, a large abdominal wall defect was closed by skin, creating a giant ventral hernia. We performed endoscopic separation component technique (ECST) for the closure of her abdominal wall defect when she was 11 months of age. ECST was initiated with placement of a 5.0-mm port just above the inguinal ligament and under the external oblique muscle. The space between the external and internal oblique muscles was created by the insufflation pressure, and a second 5.0-mm port was placed at 1.0 cm below the inferior edge of the rib into the space. As the further dissection was carried, the aponeurosis of the external oblique muscle was identified as a white line, running vertically from the epigastrium to inguinal ligament. It was transected longitudinally using electrocautery over its full length. The same procedure was performed on the contralateral side and the abdominal wall was successfully closed. Postoperative course was uneventful. CONCLUSIONS: The technique of ECST, described here, is simple and safe for infants, and the cosmetic result is satisfactory.

Distinct clonal evolution in a case with anaplastic embryonal rhabdomyosarcoma.

BACKGROUND: Clonal evolution of malignancy is a complex process related to intratumoral heterogeneity, as recent studies have also demonstrated in rhabdomyosarcoma. The purpose of this study is to present a distinct clonal feature of a case with anaplastic embryonal type rhabdomyosarcoma (ERMS) using molecular analysis. METHODS: A five-year-old girl developed a metastatic pelvic tumor. We cultured neoplastic cells isolated from the biopsy sample. Next, to characterize the current case, we analyzed the biopsy sample, autopsy sample, and established cell line using combined modalities, including histopathological, cytogenetic, and molecular assay. We also undertook the backtrack mutation-specific polymerase chain reaction to reveal clonal composition. RESULTS: The histology of the biopsy sample was consistent with ERMS with focal anaplasia. We established a permanently growing cell line, ICH-ERMS-1, from the biopsy sample. On molecular analysis, the biopsied tissue revealed a missense mutation at codon 245 of TP53. In contrast, the autopsy tumor tissue and the cell line established from the biopsied tissue showed a missense mutation at codon 248. A backtrack study using mutation-specific polymerase chain reaction detected a TP53 codon 248 mutation in the original biopsy sample. All the specimens examined had a missense mutation at PTPN11 codon 69. CONCLUSIONS: This study highlights intratumoral heterogeneity and distinct clonal change related to the functional context in our anaplastic ERMS case, supporting the concept of intratumoral heterogeneity and clonal evolution. It requires further case collection to reveal whether p14ARF-p53-MDM2 tumor suppressor pathway alteration, considered a late event in ERMS tumorigenesis, is responsible for anaplasia in ERMS.

Perforation in pediatric non-complicated appendicitis treated by antibiotics: the real incidence.

PURPOSE: The incidence of perforation during antibiotic therapy (AT) of children triaged as non-complicated acute appendicitis (NC-Ap) was investigated. METHODS: Abdominal ultrasonography (US) and/or computed tomography (CT) scans from cases of perforation identified at appendectomy for failed AT were reassessed blindly by a panel of board-certified specialists for any evidence of pre-AT morbidity suggestive of perforation. RESULTS: Of 521 cases triaged as NC-Ap, symptoms resolved with AT in 452 cases (86.8%). All 69/521 (13.2%) cases with persistent symptoms had urgent appendectomy, and 12/521 (2.3%) were found to have perforated. Blind reassessment of US and/or CT scans from these cases identified seven with evidence of perforation when they were triaged as NC-Ap. Thus, the actual incidence of perforation during AT for NC-Ap was actually 12-7 = 5/521 (0.95%). CONCLUSIONS: Perforation is generally believed to be a complication of AT, but inappropriate triaging of cases for AT can bias results by artificially inflating the number of perforations, in this study, by more than double. We are the first to assess the unbiased incidence of perforation during AT for NC-Ap, by reassessing pre-AT US and/or CT scans. The incidence of perforation during AT is actually negligible.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

Histological significance of hepatitis-like findings in biliary atresia: An analysis of 34 Japanese cases.

BACKGROUND: Hepatocellular injury including multinuclear changes are common histological features in biliary atresia (BA), as well as in neonatal hepatitis. To date, however, no reports have examined how those findings correlate with the prognosis of BA. We clarified the clinical implications of hepatitis-related changes in BA on histological analysis. METHODS: We retrospectively reviewed 34 cases of BA treated over the past 30 years at Ibaraki Children's Hospital. Liver biopsy specimens during Kasai procedures were evaluated for hepatocyte multinuclear change, ballooning, and acidophilic body, hereby defined as hepatitis-like findings (HLF). Each finding was semi-quantitatively scored as 0-2, and their sum was defined as the HLF score, ranging from 0 to 6. We examined the correlation between HLF score and total bilirubin (T-Bil), direct bilirubin (D-Bil), and other liver function test results at the Kasai procedure, as well as 1 week, and 1, 3, and 6 months after the Kasai procedure. Subsequently, HLF score was compared between native liver survivors (NLS; n = 16) and non-NLS (n = 18) for long-term analyses. RESULTS: Hepatitis-like findings score except for aspartate aminotransferase (AST), had no correlation with the preoperative data. HLF score was positively correlated, however, with T-Bil, D-Bil, and AST at 1 week and 1 month after the Kasai procedure (1 week: P = 0.009, 0.023, and 0.019; 1 month: 0.022, 0.019, and 0.013, respectively). HLF score was not significantly different between the NLS and non-NLS groups. CONCLUSION: Higher HLF score at Kasai procedure is an indicator of poor liver function at short-term follow up.

Laryngo fiberscopy-guided suspension procedure for an ectopic lingual thyroid obstructing airway.

BACKGROUND: Currently, there is no consensus regarding the optimal therapeutic strategy for the management of an ectopic lingual thyroid. A surgical approach is suggested when airway obstructive symptoms cannot be tolerated at all, or when bleeding or malignancy occurs. However, for patients in whom ectopic thyroid is the only functioning thyroid tissue, complete surgical excision needs to be followed by lifelong hormone replacement therapy. We report the case of an infant with ectopic lingual thyroid obstructing the airway that was treated using our novel surgical procedure. CASE PRESENTATION: A 10-day-old male infant presented with symptoms of airway obstruction and subclinical hypothyroidism. Imaging tests revealed an ectopic lingual thyroid and the absence of a normal pretracheal thyroid gland. We administered oral levothyroxine to lower his thyroid stimulating hormone (TSH) level and reduce the volume of the lingual mass; however, his airway symptoms did not improve. Subsequently, we performed a surgical intervention when he was 2 months old. We split the hyoid bone, and then suspended the lingual thyroid by suturing it to the hyoid bone to elevate the epiglottis. We confirmed the degree of suspension using intraoperative laryngo fiberscopy. After the surgery, the symptoms of airway obstruction were resolved and the patient was clinically euthyroid on low-dose oral levothyroxine. CONCLUSIONS: Our laryngo fiberscopy-guided suspension procedure can be an effective surgical procedure for the treatment of ectopic thyroid. This relatively simple surgical procedure could completely preserve the patient's thyroid tissue and resolve airway obstruction.

Incidence of ureterovesical obstruction and Cohen antireflux surgery after Deflux® treatment for vesicoureteric reflux.

AIM: The purpose of this study was to determine the incidence of ureteric obstruction (UB) and requirement for Cohen antireflux surgery (CAS) after DefluxⓇ treatment (DT) for vesicoureteric reflux (VUR). METHODS: Between 2011 and 2017, 494 ureters (VUR severity ≤ grade III: N=291 or >grade IV: N=203) were treated by DT at a mean age of 4.5 (range: 0.2-24) years. Epidural Catheter Assistance (ECA) was used to exclude UB by injecting diluted indigo carmine solution (1-3mL) into an epidural catheter inserted into a ureter after DT and confirming dye flow within 15min. ECA+: N=181 ureters; ECA-: N=313 ureters. RESULTS: In ECA+, UB was detected in 5/181 (2.7%) ureters (grade II: N=1, III: N=3, and IV: N=1) treated by leaving the ECA catheter in situ overnight (N=4) or double J stent (DJS) insertion for 1month (N=2). After mean follow-up of 1.9years, one grade III DJS case has residual grade II VUR. In ECA-, 3/313 (0.9%) cases developed UB. One resolved, and one required DJS. CAS was required for 17/494 (3.4%) ureters and hindered by DT in 5/17 (29.4%) ureters. All are sequelae-free after mean follow-up of 1.8years. CONCLUSIONS: UB may be more frequent than reported (3.3% versus 0.6%-1.8%). ECA identifies potential UB. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Aggressive gastrointestinal food allergy in neonates and its possible relationship to necrotizing enterocolitis.

INTRODUCTION: The incidence of gastrointestinal food allergy (FA) in neonates is increasing. Despite this, cases of patients with gastrointestinal FA who develop necrotizing enterocolitis (NEC) requiring laparotomy are extremely rare. PRESENTATION OF CASE: We describe two cases that presented with bloody stool with a probable diagnosis of FA as eosinophils were positive in the stool at onset. Both cases failed conservative treatment. Jejunostomy and ileostomy were performed in both cases due to secondary NEC with underlying acute FA. Post-surgery, raised peripheral blood eosinophil count, presence of cow's milk-specific IgE antibody and positive allergen-specific lymphocyte stimulation test were found. Stoma closure were performed 3 and 1 months later in both cases. Postoperative recovery was uneventful. DISCUSSION: A few reports have not identified risk factors for NEC secondary to FA. Thrombocytopenia and rise in C-reactive protein (CRP) levels 2days after the development of FA may be suggestive of FA with NEC. Methicillin-resistant Staphylococcus aureus (MRSA) was detected in the fecal culture of both patients at the time of the onset of NEC. The toxic antigen produced by MRSA may cause activation of milk-protein-primed T cells and exacerbate FA. CONCLUSION: The decrease of platelet levels and rise in CRP may indicate the development of secondary NEC in patients with FA. Additionally, MRSA detected in the fecal culture also may be a risk factor for NEC through the activation of cellular immunity reaction pathways.

Timing and outcome of stoma closure in very low birth weight infants with surgical intestinal disorders.

PURPOSE: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders. METHODS: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders. RESULTS: We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI (p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI (p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g (p = 0.614) for MRI. There were no significant differences among the three groups. CONCLUSIONS: The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases.

Long-term follow-up for anicteric survival with native liver after redo Kasai: a first report.

PURPOSE: We present a first report of the long-term follow-up of biliary atresia (BA) patients who became anicteric with the native liver (ANL; total bilirubin <1.5mg/dL) after redo-Kasai. METHODS: Forty-six redo-Kasai cases (1984-2015) were the subjects for this study. ANL ratios were determined using the Kaplan-Meier estimate. RESULTS: BA type was I (n=3), II (n=1), and III (n=42). Mean ages (initial/redo) at Kasai were 60.3/231.9days, respectively. Jaundice persisted after the initial Kasai in 24/46 cases while 22 had recurrence of jaundice after initially becoming anicteric. After redo, 5/24 of the persistent jaundice cases and 14/22 of the initially anicteric cases became anicteric (p<.05). Of these 19, 7 (one type I, six type III) are currently ANL while the remaining 12 had LTx or died. Morbidity/Complications documented in the 7 post-redo ANL cases after a mean follow-up of 16.7years (range: 10.0-31.1) included 4 episodes of cholangitis, 3 episodes of portal hypertension, 4 episodes of esophageal varices, 3 episodes of splenomegaly, one splenectomy, and 4 episodes of thrombocytopenia. CONCLUSIONS: Ours is the first long-term follow-up study of redo-Kasai cases. We found that the ANL ratio after redo-Kasai was low at 7/46 (15.2%) and that ANL were prone to multiple morbidity. LEVEL OF EVIDENCE: Retrospective comparative study, level III.

Identification and molecular docking studies for novel inverse agonists of SREB, super conserved receptor expressed in brain.

The identification of novel synthetic ligands for G protein-coupled receptors (GPCRs) is important not only for understanding human physiology, but also for the development of novel drugs, especially for orphan GPCRs for which endogenous ligands are unknown. One of the orphan GPCR subfamilies, Super conserved Receptor Expressed in Brain (SREB), consists of GPR27, GPR85 and GPR173 and is expressed in the central nervous system. We report herein the identification of inverse agonists for the SREB family without their agonists. We carried out an in vitro screening of 5472 chemical compounds from the RIKEN NPDepo chemical library. The binding of [(35) S]GTPγS to the GPR173-Gsα fusion protein expressed in Sf9 cells was measured and resulted in the identification of 8 novel GPR173 inverse agonists. The most potent compound showed an IC50 of approximately 8 µm. The identified compounds were also antagonists for other SREB members, GPR27 and GPR85. These results indicated that the SREB family could couple Gs-type G proteins, and SREB-Gsα fusion proteins showed significant constitutive activities. Moreover, a molecular model of GPR173 was constructed using the screening results. The combination of computational and biological methods will provide a unique approach to ligand identification for orphan GPCRs and brain research.