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Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele.This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes.Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies.The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus.

Extraosseous Ewing's sarcoma is a rare tumor which is aggressive with poor prognosis; it can occur anywhere in the body, but scantily in the pancreas. Pancreatic Ewing's sarcoma is not reported commonly, with inconsistent clinical manifestations. In this regard, early recognition of this disease is very important for the patient's sake.A 16-year-old boy presented with left lower quadrant abdominal pain for 2 months, and left flank pain with dysuria for 1 month.Abdominal and renal ultrasonography found a mass between the spleen and left kidney as well as left renal pelvic dilatation. Abdominal computed tomography found a heterogenous mass derived from the tail of the pancreas. Serial examinations revealed that the mass was a pancreatic Ewing's sarcoma. Furthermore, no metastasis was documented.The tumor was totally excised after 6 months of chemotherapy, which included 10 courses of neoadjuvant chemotherapy with vincristine, epirubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. The patient completed consolidation chemotherapy with vincristine, epirubicin, and cyclophosphamide, alternating with ifosfamide and etoposide for 5 courses. Radiotherapy was applied to the tumor-involved region and tumor bed.To date, the malignancy has not recurred since the treatment was completed 4 years ago. There are no complications from the treatment for the patient.The pancreas is a very rare extraosseous location for Ewing's sarcoma. Pancreatic extraosseous Ewing's sarcoma should be regarded as a differential diagnosis of non-urinary originated left flank pain with dysuria in adolescents.

Background and aim Intestinal mucositis remained one of the most deleterious complications in cancer patients undergoing chemotherapy. 5-FU treatment was reported to affect the abundance of gut microbiota and cause mucositis, which might be ameliorated by probiotics. We investigate the potential changes of 5-FU treatment and the modulations of probiotics on gut microbiota in a mouse model. Methods Male BALB/c mice received either 5-FU or saline (S). They were separated and fed saline, Lactobacillus casei variety rhamnosus (Lcr) and Lactobacillus reuteri DSM 17938 (BG). Lcr and BG were simultaneously administered with 5-FU for 5 days. Stool specimens were collected for DNA extraction and pyrosequenced for bioinformatic analysis. Results Fecal microbial communities were obviously diverse. Bacteroides and Bacteroidaceae were the most abundant microbiota in FU.BG group while S24_7 was the most in S.S group. At phylum and class levels, abundances of Betaproteobacteria, Erysipelotrichi, Gammaproteobacteria, and Verrucomicrobia were significantly increased in the FU groups. Probiotics supplementation did increase the abundances of Enterobacteriales and Turicibacterales. We demonstrated that probiotics did modulate the abundance and diversity of gut microbiota. Bacterial motility proteins were found enriched and upregulated in the S.BG group. No mortality was noted. No bacterial translocation was found in spleen and blood among the six groups. Conclusion Gut microbiota of mice undergoing chemotherapy exhibited a distinct disruption in bacterial composition. Probiotic did modulate the abundance and diversity of gut microbiota. This is the first study to analyze the effects and safety of Lactobacillus strains on 5-FU-induced mucositis systematically and assess changes in the intestinal microbiota after probiotic intervention.

Background: For chemotherapy patients, intestinal mucositis is a frequent complication. Previously, we evaluated the beneficial effect of oral probiotics in 5-Fluorouracil (5-FU) induced mucositis in BALB/c mice. Here, we used SCID/NOD mice instead to simulate the immunodeficiency of chemotherapy patients: first, to evaluate the safety of probiotic supplementation and second, to determine the probiotic effect in response to 5-FU intestinal mucositis. Methods: Thirty-six SCID/NOD mice were injected with saline (three control groups) or 5-FU (three experimental groups) intraperitoneally daily for five days. Mice were given either oral saline daily, probiotic suspension of Lactobacillus casei variety rhamnosus (Lcr35, Antibiophilus™, France) or Lactobacillus acidophilus and Bifidobacterium bifidum (LaBi, Infloran™, Italy). Blood, liver, spleen, and lymph node tissue samples were evaluated for probiotic translocation via culture and Q-PCR. Weight change, diarrhea score, jejunal villus height (VH) and crypt depth (CD), and serum cytokine levels of TNF-α, IFNγ, IL-1β, IL-6, IL-4, IL-10, IL-13, and IL-17 were also assessed. Results: No weight loss was found in the SCID control group. Mean weight loss of 10.63 ± 0.87% was noted by day five in 5-FU group without probiotics but it was only 6.2 ± 0.43% if mice were given Lcr35 (p

Background. Intestinal mucositis remains one of the most deleterious side effects in cancer patients undergoing chemotherapy. We hypothesize that the probiotics could preserve gut ecology, ameliorate inflammation, and protect epithelia via immune modulations of enterocytes and intestinal stem cells. Our aim is to characterize these changes and the safety of probiotics via a 5-fluorouracil- (5-FU-) induced intestinal mucositis mouse model. Methods. 5-FU-injected BALB/c mice were either orally administrated with saline or probiotic suspension of Lactobacillus casei variety rhamnosus (Lcr35). Diarrhea scores, serum proinflammatory cytokines, and T-cell subtypes were assessed. Immunostaining analyses for the proliferation of intestinal stem cells CD44 and Ki67 were processed. Samples of blood and internal organs were investigated for bacterial translocation. Results. Diarrhea was attenuated after oral Lcr35 administration. Serum proinflammatory cytokines were significantly increased in the 5-FU group and were reversed by Lcr35. A tremendous rise of the CD3+/CD8+ count and a significant decrease of CD3+CD4+/CD3+CD8+ ratios were found in the 5-FU group and were both reversed by Lcr35. 5-FU significantly stimulated the expression of CD44 stem cells, and the expression was restored by Lcr35. 5-FU could increase the number of Ki67 proliferative cells. No bacterial translocation was found in this study. Conclusions. Our results showed that 5-FU caused intestinal inflammation mainly via Th1 and Th17 responses. 5-FU could stimulate stem cells and proliferation cells in a mouse model. We demonstrate chemotherapy could decrease immune competence. Probiotics were shown to modulate the immune response. This is the first study to analyze the immune modulation effects and safety of Lactobacillus strain on enterocytes and intestinal stem cells in a mouse model.

Background Intestinal mucositis remained one of the most deleterious side effects in cancer patients undergoing chemotherapy. 5-Fluorouracil (5-FU) treatment was reported to affect the abundance of gut microbiota. In this study, we hypothesize that the probiotics could preserve gut ecology, ameliorate inflammation and protect epithelium by maintaining the tight junction integrity via immune modulations of enterocytes and intestinal stem cells. Our aim is to characterize these changes and to investigate the immune modulation effects and safety of probiotic via a 5-FU-induced intestinal mucositis mouse model.Methods 5-FU-injected BALB/c mice were used. They were either orally administrated saline or probiotic suspension of Lactobacillus casei variety rhamnosus (Lcr35). Diarrhea score, serum pro-nflammatory cytokines, intestinal histology and T-cells subtypes were assessed. Immunostaining analysis for intestinal stem cells CD44 and Ki67 proliferation were processed. Samples of blood and internal organs were investigated for bacteria translocation.Results Diarrhea was attenuated significantly after oral Lcr35 administration. Serum pro-inflammatory cytokines were significantly increased in 5-FU group and were reversed by Lcr35. There was a tremendous rise of CD3+/CD8+ count in the 5-FU group. The CD8+ count was reversed in the 5-FU+Lcr35 group. 5-FU caused a significant decrease of CD3+CD4+/CD3+CD8+ ratio and was reversed by Lcr35. 5-FU significantly stimulated the expression of CD44 stem cells and was restored by Lcr35. We also found 5-FU could increase the number of Ki67 proliferative cells. No bacterial translocation was found in this study.Conclusions Our results showed 5-FU caused intestinal inflammation via Th1 and Th17 responses. 5-FU could stimulate stem cells and proliferation cells in a mouse model. We demonstrated chemotherapy could decrease immune competence. Probiotics were shown to modulate immune response. This is the first study to analyze the immune modulation effects and safety of Lactobacillus strains on enterocytes and intestinal stem cells in a 5-FU-induced mucositis mouse model. The model therefore seems well suited to study the effects of different probiotics on chemotherapy-induced mucositis, prior to performing clinical human studies.

Wai-Tao Chan,1–3 Cheng-Che Liu,4 Jen-Shiu Chiang Chiau,5 Shang-Ting Tsai,6 Chih-Kai Liang,6 Mei-Lien Cheng,5 Hung-Chang Lee,7,8 Chun-Yun Yeung,1,3,9 Shao-Yi Hou2,6 1Department of Pediatric Gastroenterology, Hepatology and Nutrition, MacKay Children’s Hospital, 2Graduate Institute of Engineering Technology, National Taipei University of Technology, 3Mackay Medicine, Nursing, and Management College, 4Institute of Preventive Medicine, National Defense Medical Center, Taipei, 5Department of Medical Research, MacKay Memorial Hospital, Hsinchu, 6Graduate Institute of Biochemical and Biomedical Engineering, National Taipei University of Technology, Taipei, 7Department of Pediatrics, MacKay Memorial Hospital, Hsinchu, 8Department of Pediatrics, Taipei Medical University, Taipei, 9Department of Medicine, Mackay Medical College, New Taipei, Taiwan, Republic of China Abstract: Silica nanoparticles (SiNPs) are being studied and used for medical purposes. Asnanotechnology grows rapidly, its biosafety and toxicity have frequently raised concerns. However, diverse results have been reported about the safety of SiNPs; several studies reported that smaller particles might exhibit toxic effects to some cell lines, and larger particles of 100nm were reported to be genotoxic to the cocultured cells. Here, we investigated the in vivo toxicity of SiNPs of 150nm in various dosages via intravenous administration in mice. The mice were observed for 14days before blood examination and histopathological assay. All the mice survived and behaved normally after the administration of nanoparticles. No significant weight change was noted. Blood examinations showed no definite systemic dysfunction of organ systems. Histopathological studies of vital organs confirmed no SiNP-related adverse effects. We concluded that 150nm SiNPs were biocompatible and safe for in vivo use in mice. Keywords: in vivo, mice, silica nanoparticle, nanotoxicity

PURPOSE The long-term impact of Helicobacter pylori infection is complex, and concerns about the need for eradication exist. We conducted this case control study to investigate the association between H. pylori infection and failure to thrive (FTT). PATIENTS AND METHODS From January 2009 to December 2011, 53 children with FTT group and matched children with the same sex and age and similar socioeconomic status without FTT (control group) were enrolled. A questionnaire was administered to the parents/guardian, and a 13C-urea breath test was performed to detect H. pylori infection. RESULTS We found that the total prevalence of H. pylori infection was 29.2% and that there was no association between FTT and H. pylori infection (FTT group: 32%; control group: 26.4%; P=0.67). Short stature was more common in the FTT group and abdominal pain in the control group (FTT group: 37.7%; control group: 11.3%; P=0.003). In a comparison between the H. pylori-positive and -negative groups, abdominal pain (87.1% vs 64%; P=0.032) and the frequency of endoscopy (74.2% vs 32%; P

Rationale: Menkes disease (MD), also known as Menkes kinky hair disease, is a fatal neurodegenerative disease caused by a defect in copper metabolism. The symptoms involve multiple organ systems, such as the brain, lung, gastrointestinal tract, urinary tract, connective tissue, and skin. There is currently no cure for this disease entity, and patients with the classic form of MD usually die from complications between 6 months and 3 years of age. Intracranial hemorrhage secondary to tortuous intracranial arteries is a well-known complication of MD, but spontaneous retroperitoneal hemorrhage, to the best of our knowledge, has never been reported in a patient with MD. Herein, we describe the first case of retroperitoneal hematoma as a complication of MD in a 4-year-old boy. Patient concerns: A 4-year-old Taiwanese male patient with MD was referred to the hospital and presented with a palpable epigastric mass. Diagnoses: On the basis of the findings of ultrasonography and enhanced computed tomography, the diagnosis was retroperitoneal hematoma. Interventions: Interventions included laparotomy with evacuation of the hematoma, manual compression, and suture of the bleeding vessels. Outcomes: There were no postoperative complications. Lessons: This case emphasizes that bleeding in patients with MD is possible at any site in the body owing to the unstable structure of the connective tissues. Timely diagnosis with proper imaging studies can lead to prompt and appropriate management and save patients from this life-threatening condition.

ObjectiveThis study aims to investigate the association of serum vitamin D (VD) levels with the severity of liver fibrosis (LF) in post Kasai's portoenterostomy biliary atresia (PKBA) patients living with their native liver.MethodsIn this cross-sectional study, carried out in a tertiary Children's Hospital in Taipei, Taiwan, PKBA patients living with their native liver were enrolled. Liver biochemistry data, serum 25-hydroxyvitamin D (25-OHVD), acoustic radiation force impulse (ARFI), and scores of Pediatric Quality of Life questionnaire (PedsQL) were collected.ResultsAll the enrolled 33 PKBA patients (36.4% males), aged 1-23 years, possessed 25-OHVD less than 30ng/ml. An inverse correlation was detected between serum 25-OHVD and ARFI (r2 = 0.175; p = 0.024). We selected a cutoff value of 23ng/mL to divide PKBA patients into two groups, as the p-value was the most significant at this point when comparing the median ARFI of two groups (p = 0.003). Ten (30.3%) had 25-OHVD≥23ng/ml (HVD group), whereas 23(69.7%) had 25-OHVDConclusionVD levels are inversely associated with severity of LF in PKBA patients with native liver.

Background.Lactobacillusshows beneficial anti-inflammatory effects onSalmonellainfection. The maintenance of the tight junction (TJ) integrity plays an importance role in avoiding bacterial invasion. WhetherLactobacilluscould be used to regulate the TJ protein expression and distribution in inflamed intestinal epithelial cells was determined.Methods. Using the transwell coculture model,Salmonellalipopolysaccharide (LPS) was apically added to polarized Caco-2 cells cocultured with peripheral blood mononuclear cells in the basolateral compartment. LPS-stimulated Caco-2 cells were incubated with variousLactobacillusstrains. TJ integrity was determined by measuring transepithelial electrical resistance across Caco-2 monolayer. Expression and localization of TJ proteins (zonula-occludens- (ZO-) 1) were determined by Western blot and immunofluorescence microscopy.Results. Various strains ofLactobacilluswere responsible for the different modulations of cell layer integrity. LPS was specifically able to disrupt epithelial barrier and change the location of ZO-1. Our data demonstrate thatLactobacilluscould attenuate the barrier disruption of intestinal epithelial cells caused bySalmonellaLPS administration. We showed thatLactobacillusstrains are associated with the maintenance of the tight junction integrity and appearance.Conclusion. In this study we provide insight that live probiotics could improve epithelial barrier properties and this may explain the potential mechanism behind their beneficial effectin vivo.

Background To assess whether constipation or fecal incontinence is a major late complication after posterior sagittal anorectoplasty in patients with anorectal malformation (ARM). Methods We retrospectively enrolled 188 children, 85 low-type ARM (L-ARM) and 103 high-type ARM (H-ARM), who had complete medical records of bowel habits and medication histories after posterior sagittal anorectoplasty for anorectal malformation in Mackay Memorial Hospital. Stool characteristics as well as physical and medication history were evaluated. The symptom severity (SS) scoring system was used to assess changes in bowel habits. Results During a mean follow-up period of 4.3 years, constipation was found to be the most common late complication in both groups of patients (64.5% in the L-ARM group and 78.6% in the H-ARM group). Compared to constipation, stool incontinence was much less frequent, with 4.7% in L-ARM and 3.9% in H-ARM. There was no significant difference in mean SS scores between the two groups. Conclusion Constipation was the most common late sequela in children after correction of ARM in our study.

Background and Objective: Biliary atresia (BA) is a destructive inflammatory obliterative cholangiopathy of neonates that affects both intrahepatic and extrahepatic bile ducts. Although the etiology is unknown, immunologically mediated injury of the bile ducts triggered by as yet unidentified infectious agents is likely to play a critical role. Interleukin-18 (IL-18) is a proinflammatory cytokine that plays an important role in immune, infectious, and inflammatory diseases because of its induction of interferon-gamma. In this study, we investigated whether polymorphisms of the IL18 gene were associated with susceptibility to BA. Patients and Methods: Genomic DNA was extracted from whole-blood samples of 50 Taiwanese children with BA and 1117 ethnically matched healthy controls. The IL18 ―1297 T/C, ―607 C/A, ―137 G/C, and +105 A/C polymorphisms were genotyped using the TaqMan assay. Results: No statistically significant differences of genotype, allele, carrier, and haplotype frequencies of these IL18 gene variants were found between children with BA and healthy controls. Conclusions: Our data suggest that the IL18 gene does not play a major role in BA predisposition in Taiwanese children.

Background Duplication cyst of the duodenum is rare. This study describes a case of duodenal duplication and evaluates its clinical features through a literature review. Methods A case of duodenal duplication is reported, and related articles published from 1999 to 2009 on PubMed were reviewed. Clinical manifestations, diagnostic examinations, and methods of management were analyzed. Results Including this report, there had been 38 citations in literature that provide adequate descriptions of 47 cases of duodenal duplication cysts. Nineteen (40.4%) were discovered before 10 years of age, whereas 10 (21.3%) were found in the second decade. The remaining 18 patients (38.3%) were older than 20 years. The case number decreased as age increased. Overall, 80% of cases presented with abdominal pain, and 53% were complicated with pancreatitis. Conclusions The most common symptom in duodenal duplication cysts is abdominal pain with or without nausea or vomiting. The most common complication is pancreatitis. Differential diagnoses of pancreatitis, hepatitis, cholestasis, or intussusception should include duplication cyst of the duodenum.

In this study, we investigated the anti-inflammatory and reinforcing barrier effects of Lactobacillus casei subsp. rhamnosus (Lcr35) on Caco-2 intestinal epithelial cells already exposed to Salmonella LPS. Using the Transwell co-culture model, Salmonella LPS was apically added to polarized Caco-2 cells co-cultured with peripheral blood mononuclear cells (PBMCs) in the basolateral compartment. LPS-stimulated Caco-2 cells were incubated with Lcr35 for 1, 6, 24 or 48 h. Apical inoculation of Lcr35 after 48 h significantly inhibited the basolateral secretion of interleukin-8 (IL-8) in the Caco-2/PBMC co-culture. The PCR analysis showed that Lcr35 significantly downregulated mRNA expression of monocyte chemoattractant protein 1 (MCP-1) (PP=0.05), but did not alter mRNA expression of transforming growth factor-β1 in LPS-stimulated Caco-2 cells at 48 h after addition of Lcr35. Compared to non-LPS-pretreated controls, transepithelial electrical resistance (TEER) of the polarized Caco-2 cell monolayers pretreated with LPS for 48 h was decreased by 9.9 % (PPPPSalmonella LPS-pretreated inflamed intestinal epithelium in vitro.

Background Gastrointestinal polyps are common in children. The purpose of this study was to review the clinical manifestations, diagnostic procedures, endoscopic findings, management, pathology, and recurrence of gastrointestinal polyps in children at Mackay Memorial Hospital. Methods We retrospectively reviewed the charts of 50 children with a diagnosis of gastrointestinal polyps managed at Mackay Memorial Hospital between January 1984 and April 2007. Demographic data; clinical features; polyp size, number and location; endoscopic findings; management; pathology; and information on recurrences were extracted from the clinical records. Results The distribution of polyps in the 50 patients included gastric (4 patients), duodenal (2), ileocecal (4) and colorectal polyps (40). All patients with gastric polyps presented with vomiting, and three of the four patients with ileocecal polyps presented with intussusception. The mean age of the 40 patients with colorectal polyps was 6.8 years. The majority of those polyps were in the rectosigmoid colon; 36 patients presented with hematochezia. Solitary polyps were identified in 33 patients and multiple polyps were identified in seven patients. Most of the colorectal polyps were less than 2 cm in diameter. Histologically, the most frequent type was juvenile polyp. Conclusion Gastrointestinal polyps in children are usually benign. Pediatricians treating a child with a gastrointestinal polyp should pay attention to the immediate complications of the polyps, such as intussusception or bleeding, the extraintestinal manifestations and long-term risk for malignancy.

Biliary atresia (BA) is a devastating neonatal hepatobiliary disease characterized by bile duct inflammation and fibrosis. The pathogenesis remains unclear, but immunologically mediated injury to bile ducts following an infectious insult is likely to play a critical role. Interferon-gamma (IFN-gamma) is a key cytokine that affects immune-mediated inflammatory responses.This study aims to investigate whether polymorphisms of the IFN-gamma (IFNG) gene were associated with susceptibility to BA.The IFNG -1615 C/T, -183 G/T, +874 A/T, and +2197 A/G polymorphisms were genotyped using the TaqMan assay, and CA repeat microsatellite was analyzed using capillary electrophoresis in 50 children with BA and 788 ethnically matched healthy controls.The distribution of genotype, allele, and haplotype frequencies of these IFNG gene variants did not differ significantly between children with BA and controls.Polymorphisms of the IFNG gene do not appear to play a major role in the genetic predisposition to BA in Taiwanese children.

Background Anterior displacement of the anus has been reported to be a cause of chronic constipation amongst young children. However, the normal position of the anus has not been studied in Taiwan. Patients and Methods Normal anal positioning was studied in 200 neonates (100 males and 100 females). All of these individuals were non-constipated. The position of the anus was numerically defined by the anal position index (API), which is the distance from the center of the anus to the vagina or scrotum divided by the distance between the vagina or scrotum and the coccyx. Thirty non-constipated infants aged between 5 and 19 months (15 males and 15 females) were included in the study to compare the index between neonates and older infants. Results The API values were 0.54±0.03 (95% CI, 0.48–0.60) for newborn males and 0.40±0.04 (95% CI, 0.32–0.47) for newborn females, 0.53±0.02 (95% CI, 0.49–0.57) for young boys and 0.39±0.06 (95% CI, 0.28–0.51) for young girls. The difference in indices between males and females was significant, but there was no significant difference in the indices between newborns and older infants. Conclusions The API is independent of age, and provides a constant and reliable parameter for determining the position of the anus.

Background Scrub typhus is a clinically important endemic disease in Taiwan. The aims of this study were to analyze the clinical manifestations, laboratory data and complications of pediatric scrub typhus in eastern Taiwan. Patients and Methods We searched medical records for all patients with scrub typhus who were hospitalized between 1992 and 2002 at the Taitung branch of Mackay Memorial Hospital, Taiwan. Records of children under the age of 18 with a confirmed diagnosis were selected for retrospective review. Results During the study period, 145 patients fulfilled the diagnostic criteria for scrub typhus, of whom 106 (73%) were adults and 39 (27%) were children. The mean age of the children was 7.6 ± 4.6 years. The most common clinical manifestations of pediatric scrub typhus were fever ( n = 39; 100%), cough ( n = 28; 72%), anorexia (72%), eschar (69%), chill (67%) and lymphadenopathy (64%). The most common complications were hepatic dysfunction (77%) and pneumonitis (54%). Three children (8%) required intensive care, but the overall survival rate was 97%. One child died with multi-organ failure within 8 hours after admission. Conclusion Scrub typhus should be considered in children with fever and hepatic dysfunction, particularly in those with a history of environmental exposure in an endemic area for scrub typhus. The presence of an eschar offers an important diagnostic clue, but not for all cases. Children with scrub typhus may develop serious complications and may even die if appropriate treatment is not given. Doxycycline is an effective antibiotic for pediatric scrub typhus in Taiwan.

To describe the ages at diagnosis and operation of biliary atresia (BA) and its incidence over a 15-year period in Taiwan.This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases, Ninth Revision (ICD-9) code of BA 751.61 plus Kasai operation (ICD-9 procedure code 51.37) or liver transplantation (LT, ICD-9 procedure code 50.5). The patients' characteristics including sex, age at diagnosis, age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts: (1) 1997 to 2001; (2) 2002 to 2006; and (3) 2007 to 2011.There were a total of 540 BA cases (275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9, 55.6 and 52.6 d. A linear regression model demonstrated a decreasing trend of the mean age at diagnosis (1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189 (35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age, respectively. All patients who did not undergo a Kasai operation eventually required LT.The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates, but without certain trend.

Rationale: Septo-optic dysplasia (SOD) is a rare congenital disorder that may cause jaundice in infants. However, it is usually prone to neglect and misdiagnosis in infants with cholestasis because endocrine disorder such as panhypopituitarism is rare in the cause of infantile cholestasis. We report a case of SOD concurrent with acquired cytomegalovirus (CMV) infection, who presented with prolonged jaundice as the first clinical sign. Patient concerns: The patient was a 2-month-old male infant who presented with cholestasis, combined with fever and panhypopituitarism. Diagnoses: He was diagnosed with SOD and acquired CMV infection. Interventions: He was treated with hormone replacement therapy and ganciclovir. Outcomes: After correction of the pituitary hormone deficiency and ganciclovir treatment, significant improvements of cholestasis, retinal lesions, and growth rate were seen in our patient. Lessons: Although an endocrine disorder such as panhypopituitarism is rare in the cause of neonatal or infantile cholestasis, we must keep this reason in mind.

Objectives. The purpose of this study was to investigate the clinical features of Meckel’s diverticula at different ages, genders, and pathology in order to serve as a reminder to clinicians when evaluating potential cases and to help obtain an early diagnosis. Methods. We collected information of patients with Meckel’s diverticulum diagnosed at Mackay Memorial Hospital in Taiwan from 1984 to 2009. After performing a thorough review of their charts, the clinical features of the Meckel’s diverticula were analyzed according to age groups, gender, and pathology. Result. A total of 126 patients, with 90 males and 36 females, were enrolled in this study. Seventy-five patients were symptomatic and 51 Meckel’s diverticula were found incidentally during surgery for other diseases. Among symptomatic patients, 39% of pediatric patients and 5% of adult patients had intestinal hemorrhage. Twenty-eight percent of pediatric patients and 67% of adult patients had inflammation of Meckel’s diverticulum. Forty-six percent of males and 16% of females had inflammation. Conversely, 27% of males and 58% percent of females had intestinal obstruction. When Meckel’s diverticulum had ectopic gastric mucosa, it tended to cause intestinal hemorrhage when the patient is young. Conclusions. Age, gender, and pathology affect the clinical presentations of Meckel’s diverticula.

Background. This retrospective study reviewed 213 asymptomatic neonates with common bile duct (CBD) dilatations diagnosed via ultrasound to evaluate their incidence and outcomes.Materials and Methods. From August 2001 to July 2010, 18,230 abdominal ultrasound scans were performed as newborn screening. There were 213 (1.17%) cases of CBD dilatation. Dilatation of neonatal CBD was defined when its diameter was ≥2 mm. The neonates’ birth history, CBD size, and follow-up results were analyzed.Results. In the 213 infants, four cystic dilatations (1.88%, 4/213) that were eventually diagnosed as choledochal cysts (CC). Among 209 neonates with fusiform dilatations (size 2.0–6.7 mm), 77 had ultrasound follow-up and 87% of them resolved spontaneously which were diagnosed as transient CBD dilatation (TCBDD). Eighty percent of TCBDDs resolved within 6 months. Patients with initial CBD size ≥3 mm had significantly lower resolution rate and neonates whose mothers are older than 35 years took longer time to resolve.Conclusion. The incidence of CBD dilatation in asymptomatic neonates was 1.17%. Eighty percent of TCBDDs resolved within 6 months. Regular ultrasound follow-up every 6 months may be appropriate for asymptomatic neonates with fusiform CBD dilatations to ensure resolution or progression.

UNLABELLED A rapid identification of Salmonella, one of the most common foodborne pathogens worldwide, in clinical patients can enable better rational managements and prevent further outbreaks. The traditional immunochromatography using antibody-gold nanoparticles (Ab-AuNPs), such as the home pregnancy test, has been used for the Salmonella detection. In this study, we developed a new and rapid method using DNA probe-AuNPs for the detection of 16s ribosomal DNA of Salmonella. To evaluate the proposed method in clinical specimens, we performed a clinical test by identifying 159 stool samples on Hektoen agar containing black or crystalloid colonies using the method and the VITEK 2 system for confirmation. Eighty of the isolates were correctly identified as Salmonella to achieve 100% sensitivity. Seventy-five samples were correctly identified as non-Salmonella spp., but four were incorrectly identified as Salmonella. The specificity was 94·93%. The assay time is about 30 min after the DNA purification. The time-consuming and labour-intense biochemical tests can be replaced. We demonstrated that this assay is a rapid, convenient and cost-effective tool for Salmonella identification of clinical faecal samples, which is worth for further promotion and clinical use. This is the first application of using 16s ribosomal DNA probe-Au-NPs and immunochromatography on clinical samples. SIGNIFICANCE AND IMPACT OF THE STUDY This is the first application of using 16s ribosomal DNA probe-gold nanoparticles and immunochromatography method on clinical samples with sensitivity 100% and specificity 94·93%. The assay time is about 30 min after the DNA purification. We find this assay a rapid, convenient, sensitive and inexpensive tool for Salmonella identification of clinical faecal samples, which is worth further promotion and clinical use and can replace the traditional time-consuming and labour-intense biochemical tests. The potential benefit of this approach is to develop a rapid point-of-care test that provides results while the patient is still at the doctors' office.

The purpose of the present study was to review the different clinical manifestations of primary gastrointestinal (GI) malignancies and benign tumors in pediatric patients.The demographic data, duration to diagnosis, clinical features, laboratory data, location and pathology of the tumors, and outcomes of pediatric patients from January 1984 to December 2009 were retrospectively reviewed.A total of 24 GI malignancies and 62 benign tumors were diagnosed. Patients in the benign group were mostly diagnosed in the first decade of life (73%), whereas majority of patients with malignancies were older than 10 years (79%). The most common tumor location in the benign group was the colorectum, whereas in the malignant group, tumors were distributed equally in the small intestine and colorectum. The median duration from onset to diagnosis in the benign group was longer than that of the malignant group (P00.05). There was statistically significant increase in the presence of hematochezia in patients with benign tumor compared with those with malignancy (P00.05). Among the malignancies, 79% presented with abdominal pain, followed by weight loss (25%), anorexia (25%), palpable mass (25%), and fever (21%), with statistically significant differences compared with the benign group (P00.05). Anemia was found in 47% to 63% of patients in both groups (P00.05). Two patients with polyposis subsequently developed malignancy.Different manifestations of GI malignancies and benign tumors may help pediatricians to detect these early. Patients with polyposis should be aware of the risk of malignant change.

The biliary trees descend from the canaliculi at the hepatocytes in the liver. Bile drains from this level into intrahepatic ducts, smaller ducts combine to form the segmental bile ducts which within the liver gradually enlarge and merge to the right and left hepatic ducts. The right and left hepatic ducts join together to form the common hepatic duct (CHD) at porta hepatis. The common bile duct (CBD) is formed by the junction of the cystic duct with the CHD. The CBD traverses through the head of the pancreas entering the duodenum through the Sphincter of Oddi at the Ampulla of Vater. Prior to draining into the duodenum, the CBD is joined by the pancreatic duct. A smaller accessory pancreatic duct and sphincter is usually present (but rarely visible by sonography ) from the dorsal pancreatic embryologic rotation. (Fig. 1)

Biliary atresia (BA) is a neonatal cholangiopathy of unknown etiology that leads to biliary cirrhosis and is the most common cause of liver transplantation in children. A still undetermined hepatobiliary viral infection may elicit an uncontrollable autoimmune response against the biliary epithelial cells in genetically predisposed children and culminates in atresia of the biliary trees. Interleukin 4 (IL4) is crucial for the differentiation of naive T helper cells into the T helper 2 effector cells that promote humoral immunity. This study aims to investigate whether polymorphisms of the IL4 gene are associated with susceptibility to BA. Genomic DNA was extracted from whole blood samples of 53 Taiwanese children with BA and 904 ethnically-matched healthy controls. The IL4 –590 C/T, –33 C/T, and 8375 A/G polymorphisms were genotyped using the Pre-Developed TaqMan Allelic Discrimination Assay in a real-time polymerase chain reaction system. No significant difference between children with BA and healthy controls were found when comparing genotype, allele, carrier, and haplotype frequencies of these IL4 gene variants. These results suggest that the tested polymorphisms of IL4 gene are unlikely to contribute significantly to BA susceptibility in Taiwanese children.

Background Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed. Methods We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH. Results Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality. Conclusion Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.