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Background Gallstones are uncommon in infants and children and Asian children are thought to have very low risk. Diagnoses have increased in recent years with the widespread use of ultrasonography (USG). This study aimed to review our experience with risk factors, complications, and treatment of pediatric gallstones in low-incidence populations. Methods We retrospectively reviewed patients younger than 18 years old diagnosed with gallstones using USG between November 2006 and December 2012 in a tertiary referral hospital in Taiwan. Demographic information including age and sex, follow-up period, USG findings, predisposing factors, complications, treatment approaches and outcomes were recorded. Results Ninety-eight children with gallstones diagnosed with USG were enrolled and reviewed in our study. Females comprised 55% of patients, with no specific gender tendency. No risk factor could be identified in 30.8% of patients. The most common risk factors were cephalosporin (CS) use, presence of a choledochal cyst (CC), and spherocytosis. CS use was not associated with a higher dissolution rate. The presence of type IVa CC implied a high rate of gallstone recurrence after Roux-en-Y hepaticojejunostomy. Complications were seen in 22.4% of patients, but only two needed emergency stone removal. Expectant management was performed in 61% of patients and 62.5% of them achieved spontaneous resolution; the stone dissolution rate was not lower than in the group treated with ursodeoxycholic acid (UDCA). Stone size was significantly decreased after UDCA use if resolution did not occur. Conclusions Pediatric gallstones showed high resolution rate, and the clinical course was largely benign. CS use was the most common risk factor and did not predict a higher dissolution rate. Type IVa CC was also an important risk factor associated with a high recurrence rate. Conservative treatment and oral UDCA may be reasonable strategies in most patients, unless complications are present.

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

Severe vitamin D deficiency may cause rickets in infants or children and osteomalacia in adults, though it is now uncommon in developed countries. However, subclinical vitamin D deficiency is more prevalent, and it is associated with osteoporosis and higher incidence of falls or fractures. It was reported that 96% children with rickets were breastfed, since breast milk contains inadequate vitamin D. The American Academy of Pediatrics 2008 recommended infants who were exclusively or partially breastfed required 400 international units vitamin D daily from the first few days of life. Furthermore, since vitamin D receptors are present all over the body, insufficient vitamin D status may correlate with several extra-skeletal effects, such as pregnancy-related complications and immune dysfunction. This paper discusses the researches regarding system-based vitamin D effects, the possible risk factors leading to vitamin D deficiency, and the recommendations of vitamin D requirements. It is well-known that vitamin D can be obtained by sun exposure or limited natural dietary sources. The American Academy of Dermatology declared ultraviolet radiation to be a known skin carcinogen, so it may not be safe or efficient to obtain vitamin D via sun exposure or other artificial sources. Therefore, many pediatricians and physicians recommend appropriate vitamin D supplementation to achieve optimal plasma concentration. Trials assessing the effects of vitamin D repletion and establishing its optimum serum level are ongoing. Medical advice for vitamin D supplementation should be individualized accordingly. Key Words: deficiency, insufficiency, supplementation, vitamin D

Background and Aims: Vitamin D (VD) plays an important role not only in mineral balance and skeletal maintenance but also in immune modulation. VD status was found correlated with the pathophysiology and severity of inflammatory bowel diseases and other autoimmune disorders. Epithelial barrier function is primarily regulated by the tight-junction (TJ) proteins. In this study, we try to establish an animal model by raising mice fed VD-deficient diet and to investigate the effects of VD-deficient diet on gut integrity and zonulin expression.Methods: Male C57BL/6 mice were administered either VD-deficient [VDD group, 25(OH)2D3 0 IU/per mouse] or VD-sufficient [VDS group, 25(OH)2D3 37.8 IU/per mouse] special diets for 7 weeks. Body weight and diet intake were recorded weekly. Serum VD levels were detected. After sacrifice, jejunum and colon specimens were collected. The villus length and crypt depth of the jejunum as well as mucosa thickness of the colon were measured. Various serum pro-inflammatory cytokines and intestinal TJ proteins were assessed. The serum level of zonulin and the mRNA expression of jejunum zonulin were also investigated.Results: We found that mice fed a VDD diet had a lower serum level of VD after 7 weeks (p < 0.001). VDD mice gained significant less weight (p = 0.022) and took a similar amount of diet (p = 0.398) when compared to mice raised on a VDS diet. Significantly decreased colon mucosa thickness was found in VDD mice compared with the VDS group (p = 0.022). A marked increase in serum pro-inflammatory cytokine levels was demonstrated in VDD mice. All relative levels of claudin (CLD)-1 (p = 0.007), CLD-3 (p < 0.001), CLD-7 (p < 0.001), and zonulin-1 (ZO-1, p = 0.038) protein expressions were significantly decreased in the VDD group when compared to the VDS group. A significant upregulation of mRNA expression of jejunum zonulin (p = 0.043) and elevated serum zonulin (p = 0.001) were found in the VDD group.Conclusions: We successfully demonstrated that VDD could lead to impaired barrier properties. We assume that sufficient VD could maintain intestinal epithelial integrity and prevent mucosal barrier dysfunction. VD supplementation may serve as part of a therapeutic strategy for human autoimmune and infectious diseases with intestinal barrier dysfunction (leaky gut) in the future. To our knowledge, this is the first study to demonstrate that VDD could lead to a significant upregulation in mRNA expression of the jejunum zonulin level and also a marked elevation of serum zonulin in a mouse model.

BackgroundKawasaki disease (KD) is a form of systemic febrile vasculitis that is complicated with coronary artery lesions (CAL). The tight junctions that maintain the intestinal barrier also play a role in systemic inflammatory diseases. Serum zonula occludens-1 (ZO-1) expression was found to be significantly lower in asthmatic patients, and another study reported that elevated systemic ZO-1 was positively correlated with inflammation in cirrhotic patients. A murine model of KD vasculitis demonstrated that vasculitis depended on intestinal barrier dysfunction, which is maintained by tight junctions. In this study, we aimed to investigate the role of the tight junction zonula occludens-1 (ZO-1) in the treatment response of intravenous immunoglobulin (IVIG) and the occurrence of CAL formation in KD patients.MethodsWe enrolled 40 KD patients, 12 healthy controls, and 12 febrile controls in this study. The serum levels of tight junction ZO-1 were determined by enzyme-linked immunosorbent assay.ResultsThe serum ZO-1 level was higher in the fever control group but did not reach a statistical significance. KD patients who received a second dose of IVIG treatment due to initial IVIG unresponsiveness had a higher serum levels of tight junction ZO-1, but without statistical significance (2.15 ± 0.18 vs. 2.69 ± 0.31 ng/mL,p = 0.058). KD patients who developed a CAL demonstrated a significant lower serum tight junction ZO-1 levels than KD without CAL formation (1.89 ± 0.16 vs. 2.39 ± 0.15 ng/mL,p = 0.027). After multiple logistic regression analysis, ZO-1 levels [(95% confidence interval (CI): 0.058 ~ 0.941, odds ratio (OR) = 0.235,p = 0.041)] showed as the risk factor for CAL formation.ConclusionSerum levels of tight junction ZO-1 levels were lower in KD patients than fever controls and associated with CAL formation.

Bacillus coagulans (PROBACI) bacteria have been examined for efficacy against infectious or inflammatory bowel diseases. The aim of this observational and cross-sectional study was to evaluate the effects of PROBACI against various functional bowel symptoms. Thirty-eight enrolled patients (36.5 ± 12.6 years) with functional bowel disorders in a gastrointestinal clinic were administered PROBACI (300-mg formulation containing 1 × 109 colony-forming units of B coagulans) twice/day over a 4-week period. Abdominal pain, abdominal distention, and global assessment were evaluated using a 5-point visual analog scale. The defecation characteristics, discomfort level, and effort required for defecation were recorded. The gut-microbiota composition in terms of the Firmicutes/Bacteroidetes ratio was analyzed by 16S-ribosomal RNA gene sequencing with stool samples at days 0, 14, and 28 post-treatment. The 38 patients achieved significant improvements in abdominal pain (2.8 ± 0.5 to 3.3 ± 0.7, P = .0009), abdominal distention (2.5 ± 0.7 to 3.2 ± 0.8, P = .0002), and global assessment (2.7 ± 0.6 to 3.6 ± 0.7, P = .0001) from days 0 to 14. Compared with the diarrhea group, the constipation group achieved greater improvements in terms of discomfort during defecation (2.5 ± 0.7 to 3.1 ± 0.7, P = .02) and normalization of defecation style (50% vs 7.1%, P = .007) by day 28. A difference was observed in the Firmicutes/Bacteroidetes ratio between the constipation-dominant group (118.0) and diarrhea-dominant group (319.2), but this difference was not significant. PROBACI provided control of abdominal pain, less discomfort during defecation, and a more normalized defecation style, especially in the constipation-dominant group.

OBJECTIVE Pediatric acute pancreatitis (AP) may be different from adult AP in various respects. This study focuses on the epidemiology and medical resource use of pediatric AP in Taiwan. METHODS Patients aged 0 to 18 years with AP were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases, Ninth Revision code of AP 577.0. The medical resource use was measured by length of hospital stay and hospital charges. RESULTS Between 2000 and 2013, a total of 2127 inpatient cases of pediatric AP were collected, which represented a hospitalization rate of 2.83 per 100,000 population. The incidence by age had 2 peaks, the first peak was at age 4 to 5 years old, and the second one started rising from 12 to 13 years old until adulthood. The incidence by year increased from 2.33 to 3.07 cases per 100,000 population during the study period. The average hospital stay is steady, but the medical cost is increasing. Girls have longer hospital stays, higher medical expenditures, more use of endoscopic retrograde cholangiopancreatography possibly due to more comorbidities with biliary tract diseases than boys (P

The physical positions that are adopted by women during childbirth significantly impact their childbirth outcomes and experiences. Literature studies have associated using a squatting position with reduced childbirth pain and increased comfort and pushing efficiency. However, the major disadvantage of the squatting position is that women may lack the muscular fitness and stamina necessary to sustain this position for a long period.The aim of this study was to compare the pushing experiences and birth outcomes of three different pushing positions during the second stage of labor.A randomized controlled trial was conducted at a regional teaching hospital in northern Taiwan. Data were collected from 168 primiparous women during the 38th to 42nd gestational weeks. None of the participants received epidural analgesia during labor, and all were free of pregnancy and labor-related complications. During labor, after full cervical dilation and when the fetal head had descended to at least the +1 station and had turned to the occiput anterior position, the experimental group was asked to push in the squatting position while using the ergonomically designed ankle support. For purposes of comparison, Comparison Group A was asked to push in the squatting position without the use of the support, and Comparison Group B was asked to push in a standard semirecumbent position. All of the participants completed a demographic and obstetrics data sheet, the short-form McGill Pain Questionnaire, and the Labor Pushing Experience scale within 4 hours postpartum.In terms of delivery time, the duration between the start of pushing to crowning for the experimental group (squatting with ankle supports) averaged 25.79 minutes less (F = 6.02, p.05) than the time for Comparison Group B (semirecumbent). The duration between the start of pushing to infant birth averaged 25.21 minutes less for the experimental group than for Comparison Group B (F = 6.14, p.05). Moreover, the experimental group had a lower average visual analog scale pain score (5.05 ± 3.22) than Comparison Group B (F = 42.67, p.001), and the average McGill pain score for the experimental group was lower than both comparison groups (F = 18.12, p.001). The participants in the group that delivered from a squatting position with ankle support had better labor pushing experiences than the comparison groups (F = 14.69, p.001).In comparison with both unsupported squatting and semirecumbent pushing, squatting with the aid of ergonomically designed ankle supports reduced pushing times, ameliorated labor pain, and improved the pushing experience. Thus, this intervention may reduce the caring needs of women during the second stage of labor. This intervention may be introduced in midwifery education programs and in clinical practice as a method to improve the care of women during the second stage of labor.

Diabetic cardiomyopathy is a well-recognized complication of diabetes, but its pathophysiology is unclear. We aimed to investigate the mechanisms underlying cardiac dysfunction in an elderly type 2 diabetic (T2DM) mouse model, using membrane proteomic analyses. Elderly mice were fed a high fat diet for 12 weeks to induce T2DM, and myocardial structure and function were assessed by echocardiography. Cardiomyocytes were isolated by Langendorff perfusion and subjected to iTRAQ-based quantitative membrane proteomic profiling, immunoblotting, and real-time quantitative reverse-transcriptase polymerase chain reaction. Compared to controls, elderly T2DM mice showed worse systolic function, more myocardial fibrosis and up-regulation of several heart failure markers (all p0.05). Cardiomyocyte membrane proteomic profiling revealed that 417 proteins had differential expressions related to perturbations in several biological processes in T2DM mice compared with the control. The most up-regulated proteins were involved in oxidative phosphorylation, whereas many down-regulated proteins were involved in cytoskeletal regulation. Differential protein expression correlated with myocardial systolic velocity by tissue Doppler. In addition, cardiomyocyte immunofluorescence staining showed greater disorganization of thick/parallel F-actin stress fibers and marked reduction in F-to-G-actin ratio in T2DM vs control (p0.05), which paralleled worsened myocardial systolic velocity. We concluded that cardiac contractile dysfunction in elderly T2DM mice was associated with impaired energetics and cytoskeletal disorganization.

Jui-Ming Liu,1–3,* Ren-Jun Hsu,3–5,* Fung-Wei Chang,6 Feng-Hsiang Chiu,7,8 Chia-Lun Yeh,1 Chun-Fa Huang,9,10 Shu-Ting Chang,11 Hung-Chang Lee,12 Hsin Chi,12,13 Chien-Yu Lin14,15 1Division of Urology, Department of Surgery, Taoyuan General Hospital, Ministry of Health and Welfare, Taoyuan, 2Department of Medicine, National Yang-Ming University, 3Graduate Institute of Life Sciences, 4Department of Pathology and Graduate Institute of Pathology and Parasitology, the Tri-Service General Hospital, National Defense Medical Center, 5Biobank Management Center of the Tri-Service General Hospital, 6Department of Obstetrics and Gynecology, Tri-Service General Hospital, National Defense Medical Center, Taipei, 7Department of Emergency Medicine, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan; 8Superintendent office, Lihuili Eastern Hospital, Ningbo, Zhejiang province, People’s Republic of China; 9School of Chinese Medicine, College of Chinese Medicine, China Medical University, 10Department of Nursing, College of Medical and Health Science, Asia University, Taichung City, 11Department of Gastroenterology, BinKun Women’s and Children’s Hospital, Taoyuan City, 12Department of Infectious Disease, MacKay Children’s Hospital, Taipei, 13Department of Medicine, MacKay Medical College, New Taipei city, 14Department of Pediatrics and Infectious Disease, Hsinchu MacKay Memorial Hospital, Hsinchu City, Taiwan; 15Division of Infection and Pathway Medicine, College of Medicine and Veterinary Medicine, The University of Edinburgh, Scotland, UK *These authors contributed equally to this work Objectives: Scabies is a common and annoying disorder. Pernicious anemia (PA) is a serious disease which, when untreated, leads to death. Mounting evidence suggests that immune-mediated inflammatory processes play a role in the pathophysiology of both diseases. The relationship between these two diseases has not been investigated. We conducted this study to explore the potential relationship between scabies and PA.Materials and methods: This nationwide, population-based study was conducted using the National Health Insurance Research Database of Taiwan. In total, 5,407 patients with scabies were identified as a study group and 20,089 matched patients were randomly selected as a control group. We tracked patients in both groups for a 7-year period to identify the incidence of PA. The demographic characteristics and comorbidities of the patients were analyzed, and Cox proportional hazards regression was used to calculate the hazard ratios for PA.Results: Of the 25,496 patients in this study, 183 (0.7%) patients with newly diagnosed PA were identified during the 7-year follow-up period; 71 of 5,407 (1.3%) from the scabies group and 112 of 20,089 (0.6%) from the control group. Patients with scabies had a higher risk of subsequent PA, with a crude hazard ratio of 2.368. After adjusting for covariates, the adjusted hazard ratio was 1.51 (95% confidence interval: 1.09–2.08).Conclusion: This study demonstrated an increased risk of PA (adjusted hazard ratio 1.51) among patients with scabies. Immune-mediated inflammatory processes may contribute to this association. Further studies are warranted to investigate the entire pathological mechanisms between these two diseases. Physicians should pay attention to patients with history of scabies presented with anemia. Further confirmative tests of PA may contribute to correct diagnosis and initiation of vitamin B12 supplement. Keywords: scabies, pernicious anemia, National Health Insurance Research Database, autoimmune gastritis, vitamin B12 deficiency, cobalamin

We report a 7-year-old Taiwanese girl with acute pancreatitis (AP) complicated with pseudocyst (PC). The girl was found to have a PC by ultrasonograpgy (USG) and magnetic resonance imaging 14 days after the onset of AP. The girl was discharged 21 days after management with an asymptomatic PC. The diagnostic criteria of AP included abdominal pain, serum amylase or lipase level, and imaging findings. Transabdominal USG after appropriate preparation with adequate fasting, intake of some liquids during the scanning, and right decubitus position enhance the demonstration of pancreas and peripancreatic structures. PC could be seen in up to 38% of pediatric AP patients. It can form within 2 weeks after the onset of symptoms, although most are late complications. Pancreatic PCs have to be differentiated from other intra-abdominal cysts on USG according to their image character and anatomic location. A well-prepared USG examination in combination with liquid intake and right decubitus position is of value in the diagnosis and follow-up of PC. Abbreviations: ANC, acute necrotic collection; AP, acute pancreatitis; APFC, acute peripancreatic fluid collection; CECT, contrast-enhanced computed tomography; CRP, C-reactive protein; CT, computed tomography; MRI, magnetic resonance imaging; PAP, pediatric acute pancreatitis; PC, pseudocyst; USG, ultrasonography.

Rationale: Infantile inflammatory bowel disease (IBD) is an extremely rare subgroup of IBD that includes patients whose age of onset is younger than 2 years old. These patients can have more surgical interventions, and a severe and refractory disease course with higher rates of conventional treatment failure. Monogenic defects play an important role in this subgroup of IBD, and identification of the underlying defect can guide the therapeutic approach. Patient concerns: In 2007, a 4-month-old girl from a nonconsanguineous family presenting with anal fistula, chronic diarrhea, and failure to thrive. She underwent multiple surgical repairs but continued to have persistent colitis and perianal fistulas. Diagnosis: Crohn's disease was confirmed by endoscopic and histologic finding. Intervention: Conventional pediatric IBD therapy including multiple surgical interventions and antitumor necrosis factor alpha agents were applied. Outcomes: The patient did not respond to conventional pediatric IBD therapy. Interleukin-10 (IL-10) receptor mutation was discovered by whole-exome sequencing and defective IL-10 signaling was proved by functional test of IL-10 signaling pathway by the age of 12. The patient is currently awaiting hematopoietic stem cell transplantation. Lessons: Early detection of underlying genetic causes of patients with infantile-IBD is crucial, since it may prevent patients from undergoing unnecessary surgeries and adverse effects from ineffective medical therapies. Moreover, infantile-IBD patients with complex perianal disease, intractable early onset enterocolitis and extraintestinal manifestations including oral ulcers and skin folliculitis, should undergo genetic and functional testing for IL-10 pathway defect.

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

Adjuvant 5-fluorouracil (5-FU)-based chemotherapy, including FOLFOX (5-FU, leucovorin, and oxaliplatin), is recommended for colorectal cancer. However, intestinal mucositis remains a common adverse effect for which no effective preventive strategies are available. To develop a convenient and novel way to alleviate mucositis, we investigated the effect of Lactobacillus casei variety rhamnosus (Lcr35) on FOLFOX-induced mucosal injury. BALB/c mice subcutaneously injected with syngeneic CT26 colorectal adenocarcinoma cells were orally administered Lcr35 daily before, during, and after 5-day injection of FOLFOX regimen, for 14 days. The following methods were used: diarrhea score for toxicity, ELISA for cytokine production, histopathology for intestinal injury, immunohistochemistry for apoptosis/proliferation and regulatory proteins, RT-PCR for cytokine mRNA expression, and DNA sequencing for fecal gut microbiota. FOLFOX administration to colorectal cancer-bearing mice significantly inhibited tumor growth and the accompanying marked diarrhea and intestinal injury histologically characterized by the shortening of villi and destruction of intestinal crypts. Preventive administration of Lcr35 dose-dependently reduced the severity of diarrhea and intestinal mucositis without affecting the anti-tumor effect of FOLFOX. The numbers of apoptotic, NF-κB-, and BAX-activated cells increased after FOLFOX, and these responses were mitigated by Lcr35. TNF-α and IL-6 upregulation by FOLFOX treatment was attenuated by Lcr35. The fecal gut microbiota composition of Firmicutes and Bacteroidetes disturbed by FOLFOX was significantly reversed by Lcr35 toward a preferential profile. In conclusion, the oral probiotic Lcr35 prevented FOLFOX-induced intestinal mucositis in colorectal cancer-bearing mice. The putative mechanism might involve modulation of gut microbiota and proinflammatory responses with suppression of intrinsic apoptosis in intestinal injury.

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.

Advancements in diagnostic modalities have improved the diagnosis of meconium peritonitis (MP) both in utero and ex utero. This study aimed to determine the efficacy of prompt prenatal and postnatal diagnoses of MP on the postnatal outcomes of these patients.We conducted a retrospective chart review of neonates with MP admitted to the Mackay Memorial Hospital Systems from 2005 to 2016. The prenatal diagnoses, postnatal presentations, surgical indications, operative methods, types of MP, operative findings, associated anomalies, morbidities, patient outcomes, and survival rates were analyzed. Morbidities included postoperative adhesion ileus, bacteremia, and short bowel syndrome. We also performed subgroup analyses of the morbidity and survival rates of prenatally versus postnatally diagnosed patients, as well as inborn versus outborn neonates.Thirty-seven neonates with MP were enrolled. Of this number, 24 (64.9%) were diagnosed prenatally. Twenty-two (59.5%) were born preterm. The most common prenatal sonographic findings included fetal ascites followed by dilated bowel loops. Abdominal distention was the most frequent postnatal symptom. Thirty-four (91.9%) neonates underwent surgery, whereas 3 were managed conservatively. Volvulus of the gastrointestinal tract was the most frequent anatomic anomaly. The total morbidity and survival rates were 37.8% and 91.9%, respectively. The morbidity and survival rates did not differ significantly between prenatally and postnatally diagnosed patients (37.5% vs 33.3%, P = 1.00; 91.7% vs 92.3%, P = 1.00, respectively). Inborn and outborn patients did not differ in terms of morbidity and survival rates (27.3% vs 53.3%, P = .17; 100% vs 80.0%, P = .06, respectively).Although not statistically significant, inborn MP neonates had higher survival rates when compared with outborn MP neonates. Prompt postnatal management at tertiary centers seemed crucial.

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: The Identification and Management of Feeding Difficulties (IMFeD™) tool was introduced in 2011 for management of children with feeding difficulties and has been used in various countries around the world. This study aimed to assess its acceptance and effectiveness in clinical practice in Taiwan. Methods: This was a prospective, observational, multicenter study where subjects were followed over 3 months. Outpatients identified as picky eaters, having poor appetite, poor weight gain, or weight loss were enrolled. The acceptance and effectiveness of IMFeD tool were evaluated by caregivers and pediatricians. Results: Among 423 children who completed the study (235 boys, 188 girls; 78.8% aged

Objective DDX3 has diverse biological functions in translation control, cell growth regulation, and tumor progression. Oral squamous cell carcinoma (OSCC) is a common malignant tumor worldwide with a poor clinical prognosis. The impact of DDX3 expression in OSCC is seldom discussed. Materials and Methods Tumor tissues and adjacent normal tissues were obtained from 324 patients with OSCC. In this study, we used immunohistochemical staining methods to investigate the associations between DDX3 expression and the clinicopathological characteristics of OSCC. Results Low/negative DDX3 expression in tumor cells was significantly associated OSCC patient characteristics including male gender (P

VACTERL association is a non-random association of birth defects, which may include anomalies of the vertebral column, limbs, kidneys, and heart; anal atresia; tracheoesophageal fistula; and esophageal atresia. The presence of two or more of the defects establishes the diagnosis. The aim of our study is to describe the functional independence of children with VACTERL association and compare the results to unaffected children. These results will enable clinicians to provide more realistic prognostic information to parents and families. We used the WeeFIM questionnaire to assess the functional skills of 23 patients who had been diagnosed with VACTERL association at Mackay Memorial Hospital, Taipei, Taiwan, from June 1994 to June 2009. The total WeeFIM scores and sub-scores for three domains (self-care, mobility, and cognition) correlated significantly with age (P

Background The pandemic novel influenza H1N1 (swine) influenza A virus (H1N1v) infection has caused large-scale community infection in Taiwan. Anxiety developed in the general public and physicians faced a huge challenge in many aspects. We conducted this prospective study to develop a scoring system based on the clinical manifestations for predicting the results of influenza rapid testing, as a surrogate of influenza rapid testing, to lower the anxiety and decrease the burden for the test. Methods From September 1, 2009 to October 5, 2009, pediatric patients who received influenza rapid tests were enrolled, and questionnaires were recorded and analyzed in the first 2 weeks. A further scoring system was conducted to predict the results of influenza rapid tests and validated in the next 3 weeks. Results Eight hundred and forty-five children were enrolled in our study. In the first phase, data from 506 patients showed that those with age ≥ 5 years, fever ≥ 38°C, contact history of influenza A infection, myalgia, lethargy, sore throat, cough, and headache had a higher risk of positive results (odds ratio: 1.1–2.53). A scoring system was designed, with ≥5 points indicating acceptable sensitivity (69.5%) and specificity (63.6%). Three hundred and thirty-nine patients in the second phase were enrolled to validate the scoring system and the positive and negative predictive values were 52.0% and 73.8%. Conclusion The emergence of H1N1v infection is not only an important medical issue, but also a socioeconomic problem. Based on easily available clinical information, we develop a scoring system as a preliminary screening tool for the general public and first-line health care providers to evaluate the possibility of influenza virus infection. Although this study was limited by the sensitivity of rapid tests, this type of model may be a surrogate weapon when faced with overwhelming pandemic infection in the future, especially in areas with scarce medical resources.

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 Magnetic resonance cholangiopancreatography (MRCP) is an innovative and noninvasive technique for evaluating the biliary tree and pancreatic duct in children. The aim of this study was to assess the usefulness of MRCP as a noninvasive method to evaluate the biliary system in children. Methods We retrospectively reviewed the records of patients undergoing MRCP between October 2002 and May 2007 for suspected biliary system abnormalities. MRCP findings were compared with other imaging modalities, operative findings, and clinical endpoints. Results Complete data were available for 60 patients (35 girls, 25 boys; mean age 2 years, 33 children less than 1 year old). Ultrasound was performed in all 60 patients. Twenty-two patients had choledochal cyst, and 19 had a thin or invisible gall bladder. Endoscopic retrograde cholangiopancreatography was done in two patients. The sensitivities and specificities of MRCP for diagnosing choledochal cyst and biliary atresia were 100.0% and 100.0% and 86.7% and 100.0%, respectively. Surgery was performed in 37 patients, including 21 with a choledochal cyst, 14 with biliary atresia, and 1 with a pancreatic duct stone. Conclusion MRCP is useful method for evaluation of the pancreaticobiliary system in pediatric patients. It yields a high degree of accuracy in the diagnosis of biliary atresia and choledochal cyst.

OBJECTIVES: The pathogenesis of biliary atresia (BA) is unclear, but epidemiological studies may help to elucidate possible causes. The goals of this study were to identify BA incidence changes in Taiwan in 2004–2009 and to survey the factors that might influence incidence changes to elucidate the possible causes of BA. METHODS: A Taiwan national registry system for BA has been established since 2004. By using data from the national registry system for BA, we identified BA incidence changes in 2004–2009. We also evaluated the correlations between BA incidences and estimated rotavirus vaccine coverage rates and between BA incidences and the gross domestic product. RESULTS: A total of 185 patients with BA were identified in 2004–2009 in Taiwan, whereas the number of live births was 1 221 189. Compared with the incidence of BA in 2004–2006 (1.79 cases per 10 000 live births), the incidence of BA in 2007–2009 (1.23 cases per 10 000 live births) was decreased significantly (P = .01). BA incidences were negatively correlated with the gross domestic product (P = .02) and marginally negatively correlated with rotavirus vaccine coverage rates (P = .07). CONCLUSIONS: A significant decrease in BA incidence in Taiwan since 2007 has been noted and may be related to improvements in the general socioeconomic status and the popularity of rotavirus vaccination. Although more evidence is needed to establish a direct correlation, this phenomenon may shed light on possible causes of and preventive interventions for BA.

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.

In Taiwan, a screening system using an infant stool color card to promote the early diagnosis of biliary atresia (BA) was established in 2002. This study aimed to investigate the 5-year outcome of BA before and after using the screening program. BA patients were divided into three cohorts according to their birth dates. The patients in cohort A (n = 89) were born before the stool card screening program (1990-2000); those in cohort B (n = 28) were screened by the stool card regional screening program (2002-2003); and those in cohort C (n = 74) were screened by the stool card universal screening program (2004-2005). The relative odds ratios were computed using logistic regression to compare the different factors affecting survival time. The rate of age at Kasai operation

To evaluate the prevalence of obstructive sleep apnea (OSA) and to clarify sleep characteristics in patients with mucopolysaccharidoses (MPS), we performed overnight polysomnographic studies in 24 patients (22 males and 2 females; 3 with MPS I, 15 with MPS II, 1 with MPS III, 1 with MPS IV, and 4 with MPS VI; mean age, 10.8 ± 6.0 years; age range, 2.0-23.7 years; 2 patients ≥18 years of age). The nadir arterial oxygen saturation (SaO(2) ) was 74.5 ± 12.3%, and the average percentage of sleep time with an SaO(2) of95% was 39.4%. The percentages of total sleep time spent in sleep stages N1, N2, N3, and R were 18.6 ± 10.8%, 50.3 ± 7.6%, 14.8 ± 8.1%, and 15.3 ± 4.6%, respectively. The respiratory disturbance index (RDI) was 21.8 ± 20.4/hr, and obstructive apnea-hypopnea index (OAHI) and central apnea index were 21.4 ± 19.9/hr and 0.4 ± 0.6/hr, respectively. The desaturation index was 17.6 ± 17.8/hr. All patients had some degree of OSA. For 22 children, the disorder was mild (OAHI 1.5-5) in 2, moderate (OAHI 5-10) in 7, and severe (OAHI 10) in 13. Two patients with MPS II who received enzyme replacement therapy had reductions in RDI after treatment (38.9-10.8 and 3.5-2.0, respectively). The prevalence of moderate to severe OSA was 88% (21/24) in patients with MPS. The overnight polysomnography will help to determine the abnormalities of breathing during sleep more precisely and urge the clinicians to take necessary action for patients with severe manifestations.

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.

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.

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 Sonography is used to diagnose infantile hypertrophic pyloric stenosis, but little information is available about the appearance of postoperative sono-graphs. The purpose of this study was to evaluate the morphology of the pylorus in association with an obstruction before and after pyloromyotomy. Methods Pyloric length, diameter, muscle thickness and intermuscular space were measured sonographically at diagnosis and daily after pyloromyotomy until discharge in 12 infants with infantile hypertrophic pyloric stenosis. The ratios of pyloric wall thickness and intermuscular space to the entire pyloric diameter were measured. Results The pylorus still appeared hypertrophied after pyloromyotomy on the sono-grams. The traditional measurement of linear dimensions of the pylorus was not significantly changed from the preoperative values by the time of discharge, except for muscle thickness. The intermuscular space increased from 4.8 ± 0.8 mm preoperatively to 7.3 ± 2.1 mm by postoperative day 3 ( p = 0.10). Lowe's pyloric ratio at diagnosis was a mean of 0.32, decreasing to 0.29 on postoperative day 3 and 0.29 on the day of discharge ( p = 0.82). The alternative pyloric ratio increased significantly by postoperative day 2 (0.24 ± 0.09 on day 2 vs . 0.11 ± 0.07 preoperatively, p = 0.02). Conclusion The pyloric ratio appears to be a reliable parameter in evaluating the regression of pyloric stenosis after pyloromyotomy, and also aids in the diagnosis of pyloric stenosis.

BackgroundGastric perforation among neonates is a rare but frequently fatal condition of uncertain etiology. The aim of this study was to review the clinical course of neonatal gastric perforation and to evaluate possible prognostic factors.MethodsWe retrospectively analyzed the medical records of 15 patients with neonatal gastric perforation over a 19-year period. Another 97 patients described in the medical literature, for whom the gestational ages and birth weights were clearly stated, were also reviewed.ResultsIn our series, there were three girls and 12 boys, nine of whom were fullterm infants and six preterm infants. The most common initial manifestations were poor activity, abdominal distension, and respiratory distress. The overall mortality was 47% (7/15). Prematurity was the only statistically significant risk factor; 83% (5/6) of premature infants died compared with 22% (2/9) of term babies (p < 0.05). Combining our series with the patients reported in the literature, there were a total of 50 premature infants and 62 term infants. Gastric perforation occurred on postnatal days 2–7 and presented with nonspecific manifestations. The mortality was significantly higher in premature than in term infants (31/50, 62% vs. 16/62, 26%; p < 0.001). A trend towards higher mortality in infants with lower birth weights was observed (> 2500 g, 28%; 1501–2500 g, 52%; 1000–1500 g, 60%; < 1000 g, 100%). Infants with birth weights < 2500 g had a significantly higher mortality than infants with birth weights > 2500 g (32/58, 55% vs. 15/54, 28%; p < 0.05).ConclusionNeonatal gastric perforation is associated with high mortality, particularly in premature infants. There is also a trend towards higher mortality in lowerbirth-weight infants.

Biliary atresia is the most common cause of death from liver disease in children. Although the Kasai operation before 60 days of age can significantly improve prognosis, delay in referral and surgery remains a formidable problem worldwide because of difficulties in differentiating it from benign prolonged neonatal jaundice. We established a universal screening system using an infant stool color card to promote the early diagnosis and treatment of biliary atresia. After a pilot regional study in 2002–2003, a national stool color screening system was established by integrating the infant stool color card into the child health booklet given to every neonate in Taiwan since 2004. Within 24 hours of the discovery of an abnormal stool color, this event is reported to the registry center. The annual incidence of biliary atresia per 10,000 live births in 2004 and 2005 was 1.85 (40/216,419) and 1.70 (35/205,854), respectively. The sensitivity of detecting biliary atresia using stool cards before 60 days of age was 72.5% in 2004, which improved to 97.1% in 2005. The national rate of the Kasai operation before 60 days of age increased from 60% in 2004 to 74.3% in 2005. The jaundice-free rate (

Choledochal cyst is defined as a cystic dilatation of the biliary tree. The most common lesion reported is in the common bile duct (choledochus), hence the term choledochal cyst. Ultrasonography is the imaging modality of choice in diagnosis. It has been stated that sonographic findings of a visible intrahepatic biliary duct and/or a common bile duct diameter wider than 2 mm in infancy, greater than 4 mm in childhood, and greater than 7 mm after adolescence were abnormal. Using this diagnostic criteria, a study found that all patients with intrahepatic duct dilatation and those with extra- and intrahepatic biliary dilatation were finally diagnosed with choledochal cyst. In contrast, only 73% of patients with extrahepatic biliary dilatation had biliary anomalies. Choledochal cysts in infancy must be evaluated carefully, because patients with biliary atresia have a worse outcome than those without biliary atresia. Excluding cases associated with biliary atresia, the accuracy of diagnosing choledochal cysts in extrahepatic biliary dilatation was 71% and 97%, using cutoff points of 7 mm and 10 mm as the minimum diameters, respectively. With ultrasonography and under the investigation of an experienced sonographic examiner, choledochal cysts can be easily diagnosed.

We describe the prenatal sonographic findings in a case of Klippel-Trénaunay-Weber syndrome including fetal ascites and subcutaneous cystic lesions associated with a relatively low level of maternal serum alpha-fetoprotein and a relatively high level of maternal serum beta-human chorionic gonadotrophin. Klippel-Trénaunay-Weber syndrome may present prenatally with fetal ascites and an abnormal maternal serum screening result in addition to fetal limb hypertrophy and subcutaneous cystic lesions.

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.

Background Prior to the introduction of rotavirus vaccines, rotavirus was the leading cause of severe gastroenteritis in infants and young children, and it continues to be the leading cause in countries without vaccination programs. Rotavirus gastroenteritis results in substantial economic burden and has a pronounced effect on the family of those who are ill. Both in Taiwan and in Vietnam, rotavirus illness is viewed as a priority disease. This study assessed, in Taiwan and Vietnam, the impact of rotavirus gastroenteritis on the family among a group of parents whose children had recently been hospitalized for this illness. Methods In the first half of 2013, parents of children who had been hospitalized due to rotavirus infection were recruited from hospitals in Taiwan (n = 12) and Vietnam (n = 22), and participated in focus group sessions or in-depth ethnographic interviews. Results In both countries, the results point to a substantial burden on the parents concerning emotions and logistics of daily tasks, and to considerable disruptions of the family routine. Taiwanese parents reported satisfaction with the health care system, a great deal of effort to suppress emotions, a fair amount of knowledge about rotavirus, and little extra costs related to the illness. On the other hand, parents in Vietnam expressed concern about the emotional well-being of and the health care treatments for their children, were less knowledgeable regarding rotavirus infection, and experienced a substantial financial burden due to indirect costs that were related to accessing treatment. Conclusions Families in Taiwan and Vietnam suffer from a considerable economic and emotional burden related to rotavirus gastroenteritis. One way to substantially reduce this burden is to provide universal and affordable rotavirus vaccination to susceptible children, especially since cost-effectiveness studies have demonstrated that universal vaccination would be safe and efficacious against severe rotavirus gastroenteritis in these countries.

Intussusception in children may recur after surgical correction. Ileopexy has popularly been used as a procedure to prevent recurrent intussusception, but its effect has not been well evaluated. The present study compared the incidence of recurrent intussusception after several different surgical procedures for intussusception in children. The charts of 278 children undergoing surgical reduction of ileocolic intussusception over 17 years were retrospectively reviewed. The incidence of recurrent intussusception was compared among the three surgical procedures: simple manual reduction, manual reduction plus ileopexy, and segmental resection. Simple manual reduction was performed in 67 (24.1%) of the 278 patients, manual reduction plus ileopexy in 186 (66.9%), and resection of the involved segment in 25 (9.0%). There were four episodes of recurrence in three (4%) patients who had had manual reduction, nine episodes in eight (4%) patients who had undergone ileopexy, and none after segmental resection. There was no statistical difference in recurrent rate among the three groups (reduction vs. ileopexy, P = 0.95; reduction vs. resection, P = 0.28; ileopexy vs. resection, P = 0.29). Ileopexy is not better than simple manual reduction in preventing recurrence of ileocolic intussusception in children.

OBJECTIVE. We aimed to detect biliary atresia (BA) in early infancy to prevent additional liver damage because of the delay of referral and surgical treatment and to investigate the incidence rate of BA in Taiwan. METHODS. A pilot study to screen the stool color in infants for the early diagnosis of BA was undertaken from March 2002 to December 2003. We had designed an “infant stool color card” with 7 numbers of different color pictures and attached it to the child health booklet. Parents were then asked to observe their infant's stool color by using this card. The medical staff would check the number that the parents chose according to their infant's stool color at 1 month of age during the health checkup and then send the card back to the stool color card registry center. RESULTS. The average return rate was ∼65.2% (78184 infants). A total of 29 infants were diagnosed as having BA, and 26 were screened out by stool color card before 60 days of age. The sensitivity, specificity, and positive predictive value were 89.7%, 99.9%, and 28.6%, respectively. Seventeen (58.6%) infants with BA received a Kasai operation within 60-day age period. The estimated incidence of BA in screened newborns was 3.7 of 10000. CONCLUSIONS. The stool color card was a simple, efficient, and applicable mass screening method for early diagnosis and management of BA. The program can also help in estimating the incidence and creating a registry of these patients.

BackgroundThis study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan.MethodsWe retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 ± 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients).ResultsBABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p < 0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 ± 23 days and 158 ± 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization.ConclusionThe possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (< 8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.

Trisomy 18 is the second most common autosomal trisomy in newborns. The birth prevalence of this disorder is approximately 1 in 3,000 to 1 in 8,000, and the life span of the majority of patients is less than 1 year. As information regarding outcome in trisomy 18 is rather fragmentary in the literature, this study is aimed at investigating the survival and natural history of trisomy 18. We also evaluated the survival age and management of trisomy 18 in two different periods, before and after the implementation of National Health Insurance (NHI) program. Thirty-nine cases of trisomy 18 were collected in Mackay Memorial Hospital in a 17-year period, from 1988 to 2004. Delivery data, survival age, management before and after the implementation of NHI program, structural defects, image findings and cytogenetic results were analyzed by medical and nurse's records. The diagnosis of trisomy 18 was based on the prenatal amniocentesis or postnatal chromosome analysis. Three patients had trisomy 18 mosaicism. Since cardiovascular and central nervous systems are the most common organ systems involved in this disorder, 31 patients received brain ultrasonography and heart ultrasonography for evaluation of their multiple anomalies after admission. All patients except one died in their first year due to severe malformations of the cardiovascular or central nervous systems. The median survival age was 6 days. We found a longer survival with female patients than with male patients (P < 0.05). Implementation of NHI program in the more recent decade of this study period was associated with longer survival of trisomy 18 (P < 0.05). The three most common structural defects were clenched hands (95%), rocker bottom feet (90%), and low set or malformed ears (90%). Low birth weight was present in 90%. By cardiac ultrasonography, the top four heart defects were ventricular septal defect (94%), patent ductus arteriosus (77%) and atrial septal defect (68%). However, ten cases (32%) had complex congenital heart defects. By brain ultrasonography, the most common brain lesion was cerebellar hypoplasia (32%), followed by brain edema (29%), enlarged cisterna magna (26%) and choroid plexus cysts (19%). Although most patients with trisomy 18 die within the first few weeks after birth, it is important to recognize that a small but notable percentage of these patients will survive the first year. When prenatal or postnatal decisions need to be made, the possibility of long-term survival should be included in any discussion to enable families to make the most appropriate decision.

The aim was to investigate conjugated and unconjugated hyperbilirubinaemia in association with urinary tract infection (UTI) in young infants.Fifty infants aged3 mths who developed prolonged jaundice among 2128 infants with UTI from 1984 to 2004 were enrolled retrospectively. They were divided into conjugated (n=22) and unconjugated (n=28) hyperbilirubinaemia groups and the clinical variables between the two were compared.Compared with the unconjugated group, the conjugated hyperbilirubinaemia group had statistically significantly lower haemoglobin (1.57 vs 1.80 micromol/L), higher aspartate aminotransferase (96 vs 32.5 U/L) and alanine aminotransferase (81.5 vs 16 U/L), were older on admission (48.0 vs 32.5 days), had a longer duration of jaundice before treatment (43.5 vs 30 days) and a higher incidence of E. coli infections (19/22 vs 15/28). The direct/total bilirubin ratio was linearly correlated with duration of jaundice before treatment (p=0.004). The most significant cut-off value for the duration of jaundice vis-à-vis the type of jaundice was 38 days (p=0.007). Patients who on presentation had had jaundice for44 days (p=0.007) were unlikely to have unconjugated hyperbilirubinaemia.Infants with UTI may present with unconjugated hyperbilirubinaemia in the early stage. After 6 weeks, it is always conjugated hyperbilirubinaemia and is frequently associated with anaemia, elevated hepatic aminotransferases and E. coli infections.

Background: Type I glycogen storage disease (GSD-I) is an inherited disorder affecting glycogenolysis and gluconeogenesis. The characteristic manifestations are hepatomegaly, hypoglycemia, hyperlacticacidemia, hyperuricemia, and hyperlipidemia. Renal disease is regarded as a long-term complication and is reported mainly in older patients. Objective: We report the renal manifestations and renal ultrasonographic findings of GSD-I in infancy and early childhood in order to assess the role of renal sonography in the diagnosis of GSD-I. Materials and methods: We retrospectively reviewed our hospital’s database for patients with GSD-I from January 1993 to September 2004. The records of five patients were reviewed for this study. These five patients were diagnosed when they were younger than 3 years old. Data extracted from the charts included the initial extrarenal and renal manifestations, laboratory data, and imaging studies. We analyzed the indications for, and results of, renal sonography. Results: In addition to the clinical presentations and laboratory abnormalities, all five children had nephromegaly and increased echogenicity on ultrasonography on their first visit, although only a minor degree of tubular dysfunction was noted clinically. Three of these five patients had nephrocalcinosis or renal stones or both. Conclusion: Hyperechoic large kidneys, nephrocalcinosis, and renal stones are common in GSD-I. They can be present in early infancy. Abnormalities on renal sonography might suggest GSD-I in a patient with suspected inborn errors of metabolism.

OBJECTIVE Although the nutritional value of human milk has been thoroughly studied, few reports describing its free amino acid (FAA) content have been published. Although infant formulas are designed to approximate the nutrient composition of human milk, the content and concentration of free amino acids are unknown. We compared the FAA concentrations of milk from mothers of preterm and full-term infants with those in several infant formulas. METHOD Human milk was obtained during three different stages of lactation (colostral, transitional and mature milk). Sixty-seven samples were collected from 44 healthy mothers of term infants and 23 mothers of premature infants 29 to 36 weeks gestation (mean 33 weeks). Two brands of powdered term formula (TF-A and TF-B) and two brands designed for preterm infants (PTF-A and PTF-B )were also studied. Ion exchange chromatography was used for free amino acid analysis. RESULTS The mean concentration of total FAA in human milk was significantly higher than any of the infant formulas (8139 micromol/L for pre-term human milk; 3462 micromol/L for full term human milk; TF-A, 720 micromol/L; TF-B, 697 micromol/L; PTF-A, 820 micromol/L; PTF-B, 789 micromol/L) (P

Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. Fever and dyspnea occurred 4 days prior to admission. Ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc99m -diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group ( P

We retrospectively reviewed our experience of Epstein-Barr virus (EBV)-associated acute renal failure. Of 165 previously healthy children hospitalized with serologically proven primary EBV infection, 8 had acute renal failure, of whom 5 (group A) did not have virus-associated hemophagocytic syndrome (VAHS), while 3 (group B) did have VAHS. All had complications in four or more organ systems. Two patients in group A had renal biopsies showing acute tubulointerstitial nephritis, and the clinical and laboratory findings in the other 3 group A patients were consistent with acute tubulointerstitial nephritis. Acyclovir was used in 1 patient, but she died of hepatic failure and pulmonary hemorrhage. The other 4 spontaneously recovered renal function after supportive care, including hemodialysis in 1 patient. Our experience does not support the routine use of corticosteroids or antiviral agents in these patients. Children in group B had a relatively normal urinalysis. Renal biopsies were not performed, but their presentations were compatible with acute tubular necrosis. We conclude that EBV should be considered as a possible etiological agent in all children presenting with acute renal failure of unknown cause. The diagnosis depends on a high index of suspicion and careful serological evaluation in atypical cases.

Perinephric abscess commonly arises from rupture of an intrarenal abscess into the perinephric space. It rarely results from gastrointestinal pathology. We report two pediatric patients with retrocecal appendicitis that presented with perinephric abscess. A 3-year-old girl presented with high fever and right flank pain for more than 1 week. Ultrasonography showed a right perinephric fluid collection with normal renal parenchyma and collecting system. A perinephric abscess extending from a ruptured retrocecal appendix was diagnosed by abdominal computed tomographic (CT) scan. Her hospital course was complicated with empyema, peritonitis, and pericardial effusion. A 6-year-old girl had lower abdominal pain for 3 days and high fever on the day of admission. Ultrasonography showed a right perinephric abscess with a normal renal contour and a fecalith in the enlarged appendix in the right lower quadrant of the abdomen. Appendectomy and drainage of the perinephric abscess were performed in both cases. We suggest that a ruptured retrocecal appendix must be considered in cases of perinephric abscess, especially in patients with gas bubbles in the abscess and a normal urogenital appearance. Ultrasonography and abdominal CT scan are the preferred diagnostic tools. Prolonged antibiotics and drainage of the abscess are mandatory to decrease morbidity and mortality.