Identification of a novel E-box binding pyrrole-imidazole polyamide inhibiting MYC-driven cell proliferation.

The MYC transcription factor plays a crucial role in the regulation of cell cycle progression, apoptosis, angiogenesis, and cellular transformation. Due to its oncogenic activities and overexpression in a majority of human cancers, it is an interesting target for novel drug therapies. MYC binding to the E-box (5'-CACGTGT-3') sequence at gene promoters contributes to more than 4000 MYC-dependent transcripts.

Clinical assessments in patients ten years after pylorus-preserving gastrectomy with or without preserving both pyloric and hepatic branches of the vagal nerve for early gastric cancer.

Background/aims: To clarify the differences in the postoperative quality of life (QOL) of patients after pylorus preserving gastrectomy (PPG) between those with preserved pyloric and hepatic branches of the vagal nerve (PHV) and those without PHV, we investigated the postoperative gastrointestinal symptoms at 10 years after PPG patients with or without PHV.

Methods: Twenty eight subjects who underwent PPG with D2 lymphadenectomy without preserving the PHV (group A: 18 male and 10 female subjects aged 38 to 70 years with a mean age of 60.2 years) were interviewed to inquire about gastrointestinal symptoms (appetite, weight loss, epigastric fullness, reflux esophagitis, and early dumping syndrome), and compared with 30 PPG patients with D1 lymphadenectomy with preserving PHV (group B: 20 male and 10 female subjects aged 33 to 72 years with a mean age of 61.

Are there any functional differences of the enteric nervous system between jejunum and ileum in normal humans?

Background/aims: Functional differences of the enteric nervous system (ENS) between the jejunum and ileum in humans are still unknown. To clarify the physiological differences of the ENS in the normal human jejunum and ileum, we investigated the enteric nerve responses on normal jejunum and normal ileum in vitro.

Methodology: Twenty-two preparations of normal jejunum were taken from 22 patients with gastric cancer (14 men and 8 women, aged 43 to 65 years with a mean age of 49.

Allogeneic hematopoietic stem cell transplantation against recurrent rhabdomyosarcoma.

The prognosis of high-risk rhabdomyosarcoma (RMS) with metastatic or recurrent disease remains poor. We report a 6-year-old girl who successfully underwent allogeneic hematopoietic stem cell transplantation against recurrent metastatic alveolar RMS. The disease recurred at distant lymph node metastasis with bone marrow involvement.

Usefulness of power Doppler ultrasonography and superparamagnetic iron oxide enhanced magnetic resonance imaging for diagnosis of focal nodular hyperplasia of the liver after treatment of neuroblastoma.

Focal nodular hyperplasia (FNH) of the liver is rare in children, and it is usually diagnosed through a biopsy of the liver or hepatectomy. The authors report a case of a 10-year-old girl with multiple focal nodular hyperplasia lesions of the liver after the completion of tumor therapy for advanced neuroblastoma, and review the usefulness of the combination of power Doppler ultrasonography (US) and superparamagnetic iron oxide (SPIO) enhanced magnetic resonance imaging (MRI) for the diagnosis of FNH without a biopsy of the liver or hepatectomy.

Mesenchymal hamartoma of the liver originating in the caudate lobe with t(11;19)(q13;q13.4): report of a case.

We herein report the case of a 35-month-old female child presenting with mesenchymal hamartoma of the liver (MHL), with t(11;19)(q13;q13.4) originating in the caudate lobe. This case is the eighth known description of a cytogenetic abnormality in mesenchymal hamartoma of the liver.

Studies on pudendal nerve terminal motor latency in patients after ileal J-pouch-anal anastomosis for ulcerative colitis and adenomatous coli in childhood.

Background/aims: To clarify the neurological function with respect to external anal sphincter (EAS) muscles in child patients with or without soiling after ileal J pouch anal anastomosis (IPAA), we examined the terminal motor latency in the pudendal motor nerves (PNTML).

Methodology: A total of nine patients after IPAA for UC (7 cases) and AC (2 cases) were studied (6 males and 3 females, 10 to 15 with a mean age of 13.8 years).

Are there any functional differences of enteric nervous system between the proximal and distal parts from the dentate line in the normal human internal anal sphincter?

Background/aims: To clarify the functional differences of the enteric nervous system in the human internal anal sphincter (IAS) between the proximal and distal parts from the dentate line, we investigated the enteric nerve responses of normal proximal and distal IAS in vitro.

Methodology: Normal IAS specimens derived from 20 patients with lower rectal cancer (14 men and 6 women aged from 48 to 77 years, average 66.5 years) were used.

Mouse embryonic stem cells give rise to gut-like morphogenesis, including intestinal stem cells, in the embryoid body model.

Embryonic stem (ES) cells have been proposed as candidates for cell replacement therapy in patients with intestinal failure because these cells can be expanded indefinitely without losing their pluripotent phenotype. We investigated the differentiation capacity of mouse ES cells into gut-like structures, including intestinal stem cells, and defined culture conditions for efficient induction of formation of these structures. ES cell-derived gut-like structures (ES-guts) were reproducibly induced in developing embryoid bodies (EBs) by day 21 of differentiation culture.

Effects of mosapride citrate on patients after vagal nerve, lower esophageal sphincter, and pyloric sphincter-preserving nearly total gastrectomy reconstructed by jejunal J pouch interposition, and postoperative quality of life.

Background/aims: Vagal nerve and pylorus-preserving nearly total gastrectomy reconstructed by interposition of a jejunal J pouch (hereinafter called NTGP) is a function-preserving operation for early gastric cancer. However, some patients after NTGP have suffered from postprandial food stasis in the substitute stomach, and postprandial stasis leads to abdominal symptoms. To clarify the clinical effect of mosapride citrate (hereinafter called MS) for prevention of food stasis in the substitute stomach for patients after NTGP, we studied the clinical effects of MS before and after administration of MS.

A case series of children with high-risk metastatic neuroblastoma treated with a novel treatment strategy consisting of postponed primary surgery until the end of systemic chemotherapy including high-dose chemotherapy.

The aim of this study was to clarify the feasibility of a novel treatment strategy consisting of postponed primary surgery till the end of systemic chemotherapy including HDC without interruption by local therapy for neuroblastoma patients at a high risk for relapse. After induction chemotherapy, patients received double conditioning HDC consisting of thiotepa and melphalan. Radical surgery was applied to local lesions.

A rare case of multiple duodenal perforations in early infancy.

Duodenal perforation in early infancy is an uncommon condition. We describe a case of duodenal perforation from suspected ulcer. A premature boy was born at the gestational age of 26 weeks with a birth weight of 764 g.

Effect of FTY720 and ex vivo graft irradiation in rat small bowel transplantation: expression of mucosal addressin cell adhesion molecule-1.

Purpose: We performed a semiquantitative analysis of the expression of Mucosal addressin cell adhesion molecule-1 (MAdCAM-1) and gut-associated tissues (GALT) during small bowel graft rejection in the rat to confirm the effect of FTY720 and ex vivo graft irradiation.

Methods: Small bowel transplantations (SBT) were performed from BN rats to LEW rats. Four groups of SBT animals were studied on days 3, 5, and 7 after operations (untreated, FTY720, ex vivo graft irradiation, FTY720+ex vivo graft irradiation).

Intraoperative radiation therapy for advanced neuroblastoma: the problem of securing the IORT field.

The purpose of this study is to evaluate the efficacy of intraoperative radiation therapy (IORT) and the problem of securing the IORT field in advanced pediatric neuroblastoma. Between 1996 and 2005, 12 children received IORT for advanced pediatric neuroblastoma patients. Electron beam energies ranged from 10 to 12 MeV and median dose was 10 Gy (8-12 Gy).

Usefulness of color doppler sonography and 99m Tc-RBC scintigraphy for preoperative diagnosis of a venous malformation of the small intestine in a 2-year-old child.

Venous malformations of the small intestine are rare in children, and the preoperative diagnosis of a venous malformation in the small bowel can be very difficult. We report the case of a 2-year-old girl with a solitary cavernous venous malformation of the small intestine that caused gastrointestinal bleeding and anemia and review the usefulness of the combination of color Doppler sonography and 99m Tc-RBC scintigraphy for the diagnosis of venous malformation of the small intestine.

Treatment of fistula-in-ano in infants with a seton.

Anal fistula in infants is a common disease. Although many are recovered by conventional treatment, there are some patients who are not repaired easily. We performed the seton method to an anal fistula and report the good result that we obtained.

Silencing of MYCN by RNA interference induces growth inhibition, apoptotic activity and cell differentiation in a neuroblastoma cell line with MYCN amplification.

Although it has been suggested that the MYCN oncoprotein functions may influence tumorigenesis and patient survival in neuroblastoma, the mechanism of these functions remains unclear. To elucidate such molecular and biological mechanisms, we performed knock-down of MYCN expression using RNA interference (RNAi) method. MYCN-siRNAs (MYCN-siRNA) were transfected into the MYCN-amplified cell line NB-1.

Artificially accumulated beta-catenin inhibits proliferation and induces neurite extension of neuroblastoma cell line NB-1 via up-regulation of trkA.

Recent evidence suggests an association between up-regulation of beta-catenin/Wnt signaling pathway and neuronal differentiation of neuroblastoma. We overexpressed beta-catenin into a human neuroblastoma cell line NB-1 and observed its effect on cellular morphology, growth potential and alteration in a known differentiation related gene, trkA. Expression plasmids containing wild-type and mutated forms of beta-catenin gene were transfected into NB-1 cells, using liposome-based transfection method.

Mutations and polymorphisms of Hirschsprung disease candidate genes in Thai patients.

Mutation and polymorphism data for Hirschsprung disease (HSCR) varies among ethnic groups. Single nucleotide polymorphisms (SNP) of RET proto-oncogene (RET) were recently shown to be associated with the disease, and with disease severity, in different populations. In this study, comprehensive analysis of RET, GDNF, EDNRB, ET-3, and SOX-10 genes among sporadic HSCR in Thailand was conducted by standard PCR-SSCP, RFLP, and sequencing methods.

Non-random p53 mutations in pediatric undifferentiated (embryonal) sarcoma of the liver.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare hepatic tumor in children. Its pathogenesis is largely unknown, but lines of evidence suggest common links to that of mesenchymal hamartoma of the liver (MH). Previously, we found a p53 mutation in a case of pediatric USL.

Living-related liver transplantation with removal of inferior vena cava for unresectable hepatoblastoma.

We report a case of a two-yr-old boy with hepatoblastoma resectable only by total hepatectomy including the vena cava. Successful LTx was performed with a living donor segment without vena cava reconstruction. The tumor was located in the bilateral lobe, surrounding the IVC.

Staged approach to the urachal cyst with infected omphalitis.

Embryonal urachus exists as a cord-like structure between the urinary bladder and the umbilicus. In some cases of urachal cysts at the level of the navel, no special symptoms are detected during childhood, but spontaneous drainage at the navel may occur after adolescence, which is called an infected urachal cyst. Especially in cases accompanied by infected omphalitis, no constant opinion has been established to choose either initially curative resection or staged incision.

In vitro RNA interference against beta-catenin inhibits the proliferation of pediatric hepatic tumors.

Mutations of beta-catenin have been identified in the majority of pediatric hepatic malignancies, including hepatoblastoma (HB) and hepatocellular carcinoma (HCC), suggesting its important contribution in hepatic tumorigenesis in this age group. However, the role of beta-catenin/canonical Wnt signaling pathway in the neoplastic growth of cancer cells has not been directly studied. To address beta-catenin's capability in maintaining the malignant phenotype in established pediatric HB and HCC cell lines, HuH-6 and HepG2, harboring mutated and overexpressed beta-catenin, we carried out a series of in vitro analyses through a transfection of short interfering RNAs (siRNAs) to generate a loss-of-function model.

Novel mutation of Endothelin-B receptor gene in Waardenburg-Hirschsprung disease.

Homozygous mutations of EDNRB in human have been reported to result in Waardenburg-Hirschsprung disease (WS4), while mutated heterozygotes manifested isolated Hirschsprung disease in lower penetrance. We investigated a case of WS4 together with all members of her nuclear family for the alteration of the EDNRB gene by using PCR-SSCP and direct sequencing technique. The index patient, who was born to a family with no history of Hirschsprung disease, presented total colonic aganglionosis with small bowel extension, sensorineural hearing loss and generalized cutaneous pigmentary defects.