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Background & aims: Studies have determined that people with genetically defined lactase non-persistence have lower dairy intake that may lead to an increase risk of various non-communicable diseases. Furthermore, lactase non-persistence itself has been associated with insulin resistance. However, data on lactase non-persistence status and dairy intake in developing countries are sparse. We therefore aimed to define 1) the prevalence of lactase non-persistence among individuals with diabetes and non-diabetes in Thai population and 2) the links between lactase non-persistence, milk consumption, and risk of diabetes mellitus. Methods: We conducted a case-control study from participants of the National Health Examination Survey. DNA was isolated from the blood for LCT −13910C>T (rs4988235) polymorphism and processed using the Bio-rad c1000 touch thermal cycler and MALDI-TOF Mass Spectrometry MassARRAY Typer v4.0 (Agena Bioscience, San Diego, CA, USA) at the Center for Medical Genomics, Faculty of Medicine Ramathibodi Hospital. Cases were participants with previously diagnosed diabetes mellitus or fasting plasma glucose ≥126 mg/dL (n = 1,756) vs. the controls (n = 2,380). Results: We included 4,136 participants, 62% female, and 98.8% were > 30 years old. Homozygous CC genotype (i.e., lactase non-persistence) was noted in 98.6% and only 1.4% carried heterozygous CT. Most (76%) consumed milk <1 portion/month. Participants with either CC or CT genotype had comparable milk consumption and the risk of diabetes mellitus. Males, older adults, and lower education had a lower chance of consuming milk at least one portion per month. Besides various baseline variables, we found that higher milk consumption was associated with a lower DM risk (P =.01). Conclusion: The prevalence of lactase non-persistence in Thai population is very high. A significant difference in milk consumption frequency in relation to the lactase non-persistence status was not found. However, higher milk consumption is associated with a lower risk of diabetes mellitus. [ABSTRACT FROM AUTHOR]

Aim: To evaluate whether pregnancy and birth‐related factors are associated with celiac disease (CD) in a large, United States (US)‐based mother‐child cohort. Methods: We analysed data gathering from the Massachusetts General Hospital Maternal Child Cohort (MMCC) of children born between 1998 and 2016. Data included the mode of delivery, maternal pregnancy and their offspring characteristics. We searched for CD cases by using diagnosis billing codes. Cox proportional hazard regression models were created to identify variables associated with CD. Results: We identified 44 539 mother‐child pairs who had at least one encounter by 5 years old and identified 173 children (0.4%) with CD diagnosis; median age at the diagnosis was 6 years. Overall, the adjusted hazard ratio (aHR) of caesarean delivery for CD was 1.39 (95% CI: 0.99, 1.96, p = 0.06) when compared to children born vaginally. After stratifying for the presence of labour, children born by Caesarean delivery without labour had a higher risk of CD (aHR 1.56; 95%CI: 1.01, 2.41; p = 0.046) while infants born by Caesarean delivery with labour did not (aHR 1.26; 95% CI: 0.83, 1.93; p = 0.28). Conclusion: Being born by Caesarean delivery without labour may be associated with an increased risk for CD in the US children. [ABSTRACT FROM AUTHOR]

Background: Many children respond to medical treatment for gastroesophageal reflux disease (GERD). However, some may require invasive intervention for refractory disease. Due to the lack of prognostic tools in children, this study aimed to develop a predictive model for refractory GERD. Methods: A retrospective review was performed in children with symptoms of GERD at a university hospital. Refractory GERD was defined as an unresponsive disease after optimal treatment with medication for >8 weeks. The predictive model was constructed based on clinical features and 24‐h multichannel intraluminal impedance‐pH (MII‐pH) monitoring results. Results: A total of 205 children were included with a median (IQR) age of 0.6 (0.3, 2.0) years. Over half of the patients (59.5%) had motor disabilities. Forty‐four children (21.5%) were diagnosed with refractory GERD and subsequently underwent fundoplication. Multivariable analysis suggested that the refractory disease was associated with motor disabilities (OR: 5.35; 95% CI: 2.06–13.91), recurrent aspiration pneumonia (OR: 2.78; 95% CI: 1.24–6.26), prematurity with an onset of GERD at a post‐conceptual age <40 weeks (OR: 6.76; 95% CI: 1.96–23.33), and abnormal total reflux episodes according to age (OR: 2.78; 95% CI: 1.24–6.19), but not the acid exposure time or symptom association analysis. The predictive model for refractory GERD based on associated factors revealed an area under the ROC curve of 76.8% (95% CI: 69.2%–84.3%) with a sensitivity of 77.3% and a specificity of 64% when applying a cutoff score of ≥2.5. Conclusions: The predictive model, using clinical features and MII‐pH, may be an additional tool to predict refractory GERD in young children. [ABSTRACT FROM AUTHOR]

Aim: Functional abdominal pain disorders (FAPDs) and functional constipation (FC) are the common functional gastrointestinal disorders in adolescents. We aimed to determine the prevalence of FAPDs and FC in adolescents using the Rome IV Questionnaire of Pediatric Gastrointestinal Symptoms and the factors associated with these two functional gastrointestinal disorders. Methods: A survey for the prevalence of FAPDs and FC in adolescents was carried out at two high schools. A translated and validated Thai version of Rome IV Questionnaire of Pediatric Gastrointestinal Symptoms was used. Potential associated factors were also collected. Psychosocial problems were evaluated by using the Strengths and Difficulties Questionnaire. Results: A total of 1700 adolescents (55.5% females) with a mean age (SD) of 16.1 (0.9) years were enrolled. The prevalence of FAPDs and FC was 5.3% and 8.1%, respectively. The subtypes of FAPDs were functional dyspepsia (4.7%; postprandial distress syndrome 3.9% and epigastric pain syndrome 0.8%), irritable bowel syndrome (0.6%), abdominal migraine (0.4%) and functional abdominal pain not otherwise specified (0.3%). Multiple logistic regression analysis revealed that FAPDs were associated with female gender (odds ratio (OR) 3.3, 95% confidence interval (CI): 1.7–6.4), underlying allergic diseases (OR 3.2, 95% CI: 1.6–6.6) and concomitant emotional problem (OR 2.7, 95% CI: 1.2–5.9). No significant associated factors with FC were found. Conclusion: FAPDs and FC are common in adolescents. Postprandial distress syndrome is the most common subtype of FAPD. Associated factors for FAPDs may suggest hormonal, immune‐related and psychological involvement in the disease pathogenesis. [ABSTRACT FROM AUTHOR]

• Graft-versus-host disease may mimic eosinophilic gastrointestinal disorders in a pediatric patient who underwent bone marrow transplantation. • Clues for graft-versus-host disease include persistent hypereosinophilia and gastrointestinal symptoms that were unresponsive to conventional treatment of eosinophilic gastrointestinal disorders. • Ruxolitinib might be a treatment of choice for patients with a steroid-refractory disease. Eosinophilic gastrointestinal disorders (EGIDs) are well-documented entities in pediatric solid organ transplantation. However, the diseases are rare after bone marrow transplantation (BMT). We present an adolescent male with hemoglobin E-β-thalassemia who underwent BMT and developed chronic graft-versus-host disease (GVHD) mimicking EGIDs. Initially, the patient presented with a presumed diagnosis of eosinophilic gastroenteritis (subserosal type) and received corticosteroids for 12 weeks. Six months after corticosteroids cessation, he again developed abdominal pain, treated with corticosteroids, azathioprine, and a six-food elimination diet. Still, he later had similar symptoms with persistent hypereosinophilia. The patient was subsequently diagnosed with chronic GVHD after excluding various potential causes. Ruxolitinib also led to significant clinical improvement and the disappearance of eosinophilia. The differential diagnosis of chronic GVHD should be a concern in BMT recipients with persistent gastrointestinal symptoms and eosinophilia. Ruxolitinib may be a treatment option in children with a steroid-refractory disease. [ABSTRACT FROM AUTHOR]

Cassia occidentalis toxicity is thought to be uncommon; however, several cases have been described with acute hepatomyoencephalopathy with a high-mortality rate. We report a previously healthy, 2-year-old girl who developed acute liver failure after fresh seed ingestion. Without a specific antidote, we decided to implement supportive measures and medications including lactulose, sodium benzoate and N-acetylcysteine. The patient also experienced with cardiogenic shock and transient distal renal tubular acidosis, which were all spontaneously resolved. The liver chemistries returned to normal 3 months after the ingestion, without receiving liver assisted device or liver transplantation. [ABSTRACT FROM AUTHOR]

Background: Although identifying cases in large administrative databases may aid future research studies, previous reports demonstrated that the use of the International Classification of Diseases, Tenth Revision (ICD-10) code alone for diagnosis leads to disease misclassification. Purpose: We aimed to assess the value of the ICD-10 diagnostic code for identifying potential children with biliary atresia. Methods: Patients aged <18 years assigned the ICD-10 code of biliary atresia (Q44.2) between January 1996 and December 2016 at a quaternary care teaching hospital were identified. We also reviewed patients with other diagnoses of code-defined cirrhosis to identify more potential cases of biliary atresia. A proposed diagnostic algorithm was used to define ICD-10 code accuracy, sensitivity, and specificity. Results: We reviewed the medical records of 155 patients with ICD-10 code Q44.2 and 69 patients with other codes for biliary cirrhosis (K74.4, K74.5, K74.6). The accuracy for identifying definite/probable/possible biliary atresia cases was 80%, while the sensitivity was 88% (95% confidence interval [CI], 82%-93%). Three independent predictors were associated with algorithm-defined definite/probable/possible cases of biliary atresia: ICD-10 code Q44.2 (odds ratio [OR], 2.90; 95% CI, 1.09-7.71), history of pale stool (OR, 2.78; 95% CI, 1.18-6.60), and a presumed diagnosis of biliary atresia prior to referral to our hospital (OR, 17.49; 95% CI, 7.01-43.64). A significant interaction was noted between ICD-10 code Q44.2 and a history of pale stool (P<0.05). The area under the curve was 0.87 (95% CI, 0.84-0.89). Conclusion: ICD-10 code Q44.2 has an acceptable value for diagnosing biliary atresia. Incorporating clinical data improves the case identification. The use of this proposed diagnostic algorithm to examine data from administrative databases may facilitate appropriate health care allocation and aid future research investigations. [ABSTRACT FROM AUTHOR]

Health-related quality of life (HRQoL) of liver transplantation (LT) recipients has been widely studied because of increased patient longevity. However, studies in developing countries are scanty. We aimed to evaluate psychometric properties of the Thai version of the PedsQL Transplant Module and used it to determine the HRQoL in LT children. We included children aged 2 to 18 years who underwent LT more than 1 year previously. The phase 1 study included a cognitive interview with 20 LT children and their parents using the Thai version of PedsQL Transplant Module and revised the module accordingly. Then, the finalized version was evaluated for psychometric properties in 50 LT children. In phase 2, HRQoL was evaluated in 52 LT children who did not participate in the phase 1 study. In the Thai PedsQL Transplant Module, both parent report and child self-report had good internal consistency (α = 0.94 and 0.93, respectively) and substantial reliability (interclass correlation coefficient = 0.82 and 0.77, respectively). Median HRQoL scores from the parent report and child self-report were 80 (interquartile range [IQR], 70, 88) and 77 (IQR, 71, 88), respectively. From the parent report, lower HRQoL was associated with opportunistic viral infection (P =.004) and correlated with a greater number of immunosuppressive agents (ρ = –0.346 , P =. 014). However, none of these factors were significant according to the child self-report. The Thai PedsQL Transplant Module has satisfactory psychometric properties. Post-LT children have good HRQoL. To improve their HRQoL, specific attention should be focused on managing viral infection and optimizing immunosuppressive therapy. • The PedsQL Transplant Module is reported on with respective psychometric properties. • Most children who underwent liver transplantation had a good quality of life. • Immunosuppressive drugs and opportunistic infections were associated with quality of life. [ABSTRACT FROM AUTHOR]

Background: Previous studies have shown that physicians' perception and practice can differ from proposed guidelines with regards to constipation. Most studies were performed in developed countries but only a few in developing nations. We therefore aimed to study Thai pediatricians' perceptions of the management of constipation. Methods: We performed a national survey using an 37‐item online questionnaire that included questions about demographics, perceptions, and practice related to constipation in children. We used a five‐point scale to determine perception in each aspect (5 = strongly agreed; 1 = strongly disagreed). Results: We received 275 responses (response rate of 11.0%). Most were female (69.1%), subspecialists (55.6%) and worked in a government‐based setting (69.5%). We noted that only 51.8% considered disimpaction if physical examination is suggestive of fecal impaction. Most pediatricians used lactulose for both disimpaction and maintenance phases (83.4 and 91.9%, respectively); however, pediatricians perceived that the caregivers of constipated children were concerned about tolerance or dependence of most commonly used laxatives, ranged from 45.8 to 63.8%. We also found that recently graduated pediatricians were more likely than older individuals to provide advice on fluid intake, toilet training, and laxative use, and to consider polyethylene glycol for disimpaction (P = 0.003, 0.02, 0.004, and 0.02, respectively). Conclusions: The proportion of pediatricians who consider disimpaction remained suboptimal. Providing appropriate knowledge on pharmacological management, especially disimpaction and issues regarding laxatives, may be helpful for pediatricians and caregivers. Education may also need to be tailored to various pediatricians' experience and work settings. [ABSTRACT FROM AUTHOR]

Backgrounds: To compare factor(s) contributing to aetiology, management and clinical outcome(s) of paediatric patients acquiring acute pancreatitis (AP) at two major university paediatric surgical centres in Liverpool and Bangkok. Methods: All patients (<18 years) with an index diagnosis of AP (ICD 10 coding) during 2006–2016 were studied. Results: 121 patients included n = 79 (65.3%) in Thailand versus n = 42 (34.7%) in the UK centre with no difference(s) in age at diagnosis at 10.4 ± 4.5 and 11.7 ± 6 years. (P = 0.12). Major AP aetiology(s) in Thailand were medications (39.2%) and choledochal cysts (8.9%). In the UK—gallstone disease (21.4%), and medications (16.7%) were leading factors (P < 0.01). Ultrasonography was deployed more frequently in the UK versus Thai centre (74.3% vs. 49.1%; P < 0.01). Pancreatitis was confirmed by imaging in 67.9% (Thai) and 62.9% (UK) patients (P = 0.47). Most patients at both centres had a mild‐grade pancreatitis illness (95% Thai vs. 90.5% UK; P = 0.28) while 12.7% of Thai and 19% of UK children developed pancreatitis‐related complication (P = 0.37). Overall mortality rate (%) was significantly higher in the Thai versus UK centre (27.8% vs. 9.5%; P = 0.02). Conclusions: Aetiology of acute pancreatitis appears to vary between UK and Thailand children. Timely early diagnosis and healthcare pathways may be driven by local patient‐related factor(s). The higher mortality (%) observed in Thailand versus UK in this comparative study was linked to underlying co‐existent chronic medical condition(s) in vulnerable patient cohorts. [ABSTRACT FROM AUTHOR]

PTLD is a rare but potentially life‐threatening condition, which shows a higher prevalence in children than in adults. From 129 children who underwent LT, we reported 5 cases with biopsy‐proven PTLD at a single teaching hospital. Four patients had shared clinical presentations including fever, lymphadenopathy, and splenomegaly. They were noted to be given a prolonged course of IS due to the management of comorbid complications such as acute cellular rejection or severe food allergy or eosinophilic gastrointestinal disease. The other one patient presented with upper gastrointestinal bleeding from gastric mass during an early post‐transplantation period. Notably, hypoalbuminemia was noted in all reported patients. Similar to previous studies, both EBV serology mismatch between the donor and recipient with high EBV viral load were noted in all except one case, whose EBV serology was unknown before LT. At least one episode of CMV reactivation was also observed in 3 of 5 patients prior to the PTLD diagnosis. The histopathology revealed 1 of 5 early PTLD, 1 of 5 polymorphic PTLD, and 3 of 5 monomorphic PTLD. The treatment included IS withdrawal, chemotherapy, and/or rituximab. One patient died of multiorgan dysfunction, one remains in complete remission, and three patients are either still on treatment or await response evaluation. Even though most of our reported PTLD cases had shared manifestations with fever, lymphadenopathy, splenomegaly, EBV serology mismatch, and high EBV viral load, various initial presentations such as respiratory symptoms, hypoalbuminemia, and prolonged use of IS from other causes such as significant food allergy were noted. [ABSTRACT FROM AUTHOR]

The prevalence of food allergy and food intolerance is increasing and it is an important public health problem affecting children. Food allergy results from an immunological reaction to certain food(s) and affects numerous organs in the body. Food intolerances are non-immunological reactions including metabolic, toxic, pharmacological and undefined mechanisms. Cow milk is the most common cause of food allergy and food intolerance, especially in young children. Food intolerance can present with similar symptoms to those of food allergy. Health-care personnel, patients and their caregivers often confuse food intolerance with food allergy. This review focuses on the clinical manifestations, diagnostic evaluation, treatment and prevention of food allergy and food intolerance. [ABSTRACT FROM AUTHOR]

Aim: Children with biliary atresia (BA) born in countries with temperate climate showed month-of-birth (MoB) predilection during cooler months. To date, no study on the MoB-BA association has been performed in a tropical country. Our aim was to define MoB variation in children with BA in a tropical country.Methods: We studied 150 children diagnosed with BA between January 1996 and April 2015 at a teaching hospital. MoB was defined by two categories based on the precipitation: rain and dry, and three categories based on the air temperature: high, average and low. We applied the country's population data on the number of births in each period as the expected proportions of birth.Results: A slightly higher proportion of BA children was born in the rainy months (52.7%); however, the difference was not significant compared to the general population's birth (P = 0.87). For the MoB based on the air temperature, no statistically significant difference was noted. Males with BA seemed to have a greater MoB variation compared to females, but this did not reach statistical significance.Conclusion: We could not find an association between MoB and BA in a tropical country. Multinational studies may aid in understanding the MoB-BA association in the tropical countries. [ABSTRACT FROM AUTHOR]

Many studies have investigated the aetiological roles of genetic and environmental factors in coeliac disease (CD) with the long-term goal of developing an effective primary prevention strategy. CD is a condition with dysregulated systemic and intestinal mucosal immune responses to dietary gluten proteins among genetically predisposed individuals. We recently described spring birth as a novel risk factor for CD in children. We believe that the association between season of birth and CD is due to seasonal differences in sunlight exposure and subsequent vitamin D status. Concomitant with global increases in CD prevalence, vitamin D deficiency also is increasingly recognized in children worldwide. Recent studies have shown that vitamin D deficiency can cause improper immune responses, abnormal intestinal mucosal integrity and impaired local defence to pathogenic microbial agents. In conjunction with other potential aetiological factors, we propose a hypothesis model of early-life vitamin D deficiency in the pathogenesis of childhood-onset CD. [ABSTRACT FROM AUTHOR]

Positive coeliac serology with normal villous morphology ( NVM) indicates potential coeliac disease ( CD). Few studies have compared characteristics of NVM vs villous atrophy in patients with positive serology. Our aim was to determine the independent clinical predictors of NVM in children with positive CD serology. We performed a structured medical record review of patients aged 1-19 years who presented for an initial CD evaluation between 2000 and 2010 at a large teaching hospital. Data collection included demographics, medical history, prior history of gluten avoidance, CD-specific serology, oesophagogastroduodenoscopy and histopathology. Predictors of NVM (vs Marsh III) were determined using multivariable logistic regression. Among 320 patients with positive serology, we identified 62 patients (19%, 95% CI 15-24) with NVM (i.e. potential CD). Younger children may have been more likely to exhibit NVM (p = 0.06). Three significant predictors of NVM were prior gluten avoidance ( OR 4.17, 95% CI 1.02-17.13), positive tissue transglutaminase antibody but <100 U/mL ( OR 14.75, 95% CI 3.33-65.30), and absence of gross duodenal abnormalities (OR 3.48, 95% CI 1.51-8.03). Among children with positive CD serology, prior gluten avoidance predicts NVM. Future studies are warranted on the impact of gluten intake and CD testing in children without prior established CD diagnosis. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To evaluate the value of ICD-9-CM code for identifying celiac disease (CD). Methods: We searched administrative data to identify all adults with ICD-9-CM diagnosis code 579.0 (CD) at three teaching hospitals between 2000 and 2010. We then stratified patients according to the presence/absence of relevant serology and endoscopy codes into four groups: None, serology, endoscopy, and both. A diagnostic algorithm was applied to define CD status. Results: Through random sampling and appropriate weighting, the 1200 reviewed patients represented a cohort of 8,122 cases. The overall positive predictive value (PPV) of the ICD-9-CM code was 15% (95% confidence interval [CI], 13%–17%). Case identification by a diagnosis code alone had a PPV of 4%, whereas the group with diagnosis code plus both serology and endoscopy testing had a PPV of 49%. Independent predictors of CD were non-Hispanic white, ICD-9-CM–coded patient group, total number of a diagnosis code, and receiving a diagnosis code by a gastroenterologist. The model had an area under the curve of 0.87 (95% CI, 0.84–0.89). Conclusions: The performance of ICD-9-CM 579.0 alone for identifying CD is extremely poor. Adding other readily available administrative data significantly improves CD case identification. The proposed case finding strategy via administrative databases may facilitate future research on CD. [Copyright &y& Elsevier]

Aims Studies on the role of duodenal bulb biopsy (DBB) in coeliac disease (CD) evaluation have increased in recent years; growing evidence suggests that the disease can present solely in the duodenal bulb. Moreover, recent CD guidelines recommend obtaining a DBB. The study aim was to examine DBB performance in children undergoing CD evaluation and to identify independent predictors of DBB performance. Methods The authors performed a structured chart review of children aged <18 years who underwent CD evaluation between 2000 and 2010 at a large teaching hospital. The authors collected data including demographics, serology, endoscopy and histopathological findings. Predictors of DBB performance (obtained vs not obtained) were determined using multivariable logistic regression. Results Among 616 children who underwent endoscopy, DBB was performed in 103 children (17%, 95% CI 14% to 20%) with an increasing trend in the more recent years (2008-2010, 25%; 2004-2007, 16%; and 2000-2003, 5%, p<0.001). Three independent predictors of DBB performance were older age at endoscopy (OR 1.05 per year of age), gross gastric antral abnormalities (OR 2.81) and gross duodenal abnormalities (OR 5.55). Regarding the DBB histological findings, patchiness of CD was found in 15%. Positive Marsh III biopsy presented solely on the DBB in 6/103 (6%, 95% CI 2% to 12%) children. Conclusions The authors found a significant increase in DBB performance over time, but the overall performance remains suboptimal. Improving education on obtaining a DBB for CD evaluation is crucial, especially among those children in whom DBB is more likely to be omitted. [ABSTRACT FROM AUTHOR]

The article discusses the medical case of an 18-year-old obese Hispanic male with fever, jaundice and liver failure and diagnosed to have concomitant infections with hepatitis E and EpsteinBarr virus (EBV). The medical background of the patient is discussed. Results of physical and laboratory examinations performance on the patient are also discussed. The patient was conservatively managed, discharged and enrolled in a weight loss and exercise program for obesity.

Prior studies have reported disparate clinical presentations between children with celiac disease and type 1 diabetes mellitus and those with celiac disease alone. Studies focusing on differences in endoscopic and histopathological findings, however, are limited. We reviewed children aged 2-18 years, presenting for an initial evaluation between January 2000 and December 2010. Data on medical history, serologic markers, upper endoscopy, and histopathology were collected. Only the children with positive celiac serology who had upper endoscopy performed within 3 months of the initial visit were included. We identified 294 children who fulfilled the criteria, 21 of whom had diagnosed type 1 diabetes mellitus. Diabetic children were more likely to have absence of gastrointestinal symptoms. Erythematous duodenal and esophageal mucosa on endoscopy, and histopathology suggestive of reflux esophagitis were more common in the diabetes group. Diabetic children with positive celiac serology had different histopathological features as compared with their non-diabetic counterparts. [ABSTRACT FROM AUTHOR]

Bloody diarrhea in children is usually due to either infectious or inflammatory etiology, but infection is far more common than inflammatory bowel disease in children worldwide. If, however, the patient has unfavorable response to antibiotics and a definite infectious agent has yet to be identified; colonoscopy should be performed. The current patient presented with acute onset of mucous bloody diarrhea. Stool culture was initially identified as Vibrio mimicus and later identified as Aeromonas schubertii but the biochemistry did not fit well with either organism. After a prolonged course of hematochezia despite i.v. antibiotics, colonoscopy was performed that showed inflammation in the rectosigmoid area. Meanwhile, the final biochemistry tests and 16s rRNA sequencing of the organism confirmed Chromobacterium haemolyticum infection. Twelve weeks after the initial colonoscopy, repeat colonoscopy showed post-infectious colitis. Herein we report on the first pediatric case of C. haemolyticum infection causing proctocolitis. [ABSTRACT FROM AUTHOR]

The article presents two case studies of two infants with abetalipoproteinemia (ABL); one with the classic manifestation and the other with an atypical predominant presentation. Topics discussed include histopathology of the duodenal biopsy in infants, the response of infants to dietary treatment, and ABL in an infant or achild with post-prandial vomiting, steatorrhea and substantial growth failure of unclear etiology.

The article presents a case study of a 5-year-old boy who presented with a month-long history of upper-respiratory symptoms and subsequently developed nonbloody loose stools. On admission he had persistent abdominal pain and bloody stools. Colonoscopy was ordered which confirmed the diagnosis of ischemic colitis. The patient was treated with intravenous hydration, intravenous antibiotics, and pain management; colonoscopy 4 weeks after discharge showed complete histopathological resolution.

Cytomegalovirus (CMV) infection is a major opportunistic infection after liver transplantation (LT) that necessitates monitoring. Because of the lack of studies in children, we aimed to investigate CMV-specific T cell immune reconstitution among pediatric LT recipients. The recipients were monitored for CMV infection and CMV-specific T cells from the start of immunosuppressive therapy until 48 weeks after LT. Clinically significant CMV viremia (csCMV) requiring preemptive therapy was defined as a CMV load of >2000 IU/mL. Peripheral blood CMV-specific T cells were analyzed by flow cytometry based on IFNγ secretion upon stimulation with CMV antigens including immediate early protein 1 (IE1) Ag, phosphoprotein 65 (pp65) Ag, and whole CMV lysate (wCMV). Of the 41 patients who underwent LT, 20 (48.8%) had csCMV. Most (17/20 patients) were asymptomatic and characterized as experiencing CMV reactivation. The onset of csCMV occurred approximately 7 weeks after LT (interquartile range: 4–12.9); csCMV rarely recurred after preemptive therapy. Lower pp65-specific CD8+ T cell response was associated with the occurrence of csCMV (p = 0.01) and correlated with increased viral load at the time of csCMV diagnosis (ρ = −0.553, p = 0.02). Moreover, those with csCMV had lower percentages of IE1-specific CD4+ and wCMV-reactive CD4+ T cells at 12 weeks after LT (p = 0.03 and p = 0.01, respectively). Despite intense immunosuppressive therapy, CMV-specific T cell immune reconstitution occurred in pediatric patients post-LT, which could confer protection against CMV reactivation. [ABSTRACT FROM AUTHOR]

Background: Cytomegalovirus (CMV) infection is the most common infection following pediatric liver transplantation (LT). Preemptive therapy (PET) is an approach to initiate antiviral treatment for asymptomatic early CMV viremia detected by surveillance testing. However, data on CMV infection after PET are scarce, and the optimal cut‐off remains controversial. This study aimed to evaluate the incidence, risk factors, and consequences of CMV infection in pediatric LT using 2 different viral load (VL) cut‐offs. Methods: We retrospectively reviewed patients aged 0–18 years who underwent LT at Ramathibodi Hospital between March 2001 and August 2020. Demographic data, CMV infection, CMV treatment, and consequences of CMV infection were collected. CMV viremia was monitored by a quantitative nucleic acid amplification test. Clinical outcomes were compared after starting antiviral therapy at a low (>400 but <2000 IU/mL) and a high VL cut‐off (≥2000 IU/mL). Results: A total of 126 patients were included. CMV infection was 71% (90/126), with an incidence rate of 5.5 per 1000 patient‐day. Higher tacrolimus and prednisolone dosages were associated with CMV infection with an adjusted hazard ratio of 1.2 (95%CI 1.0–1.4, p =.02) and 2.4 (95%CI 1.9–3.4, p <.001), respectively. The consequences of CMV infection did not differ significantly for the low and high CMV VL cut‐off groups. Conclusion: CMV infection in LT recipients is common and is associated with higher tacrolimus and corticosteroid dosage. Additionally, using the CMV VL cut‐off at 2000 IU/mL to initiate antiviral therapy is practical and effective in preventing CMV disease. [ABSTRACT FROM AUTHOR]

Pediatric inflammatory bowel disease (PIBD) is rising rapidly in many industrialized and affluent areas in the Asia‐Pacific region. Current available guidelines, mainly from Europe and North America, may not be completely applicable to clinicians caring for children with PIBD in this region due to differences in disease characteristics and regional resources constraints. This position paper is an initiative from the Asian Pan‐Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition (APPSPGHAN) with the aim of providing an up‐to‐date, evidence‐based approach to PIBD in the Asia‐Pacific region, taking into consideration the unique disease characteristics and financial resources available in this region. A group of pediatric gastroenterologists with special interest in PIBD performed an extensive literature search covering epidemiology, disease characteristics and natural history, management, and monitoring. Gastrointestinal infections, including tuberculosis, need to be excluded before diagnosing IBD. In some populations in Asia, the Nudix Hydrolase 15 (NUD15) gene is a better predictor of leukopenia induced by azathioprine than thiopurine‐S‐methyltransferase (TPMT). The main considerations in the use of biologics in the Asia‐Pacific region are high cost, ease of access, and potential infectious risk, especially tuberculosis. This position paper provides a useful guide to clinicians in the medical management of children with PIBD in the Asia‐Pacific region. [ABSTRACT FROM AUTHOR]

Background Southeast Asia is the endemic area of hepatitis E virus (HEV) infection. We aimed to determine the seroprevalence of the virus, its association, and the prevalence of chronic infection after pediatric liver transplantation (LT). Methods A cross-sectional study was performed in Bangkok, Thailand. Patients aged <18 years who had LT for >2 years underwent serologic and real-time polymerase chain reaction (rt-PCR) tests. Acute HEV infection was defined by the presence of positive anti-HEV immunoglobulin (Ig)M and HEV viremia from the rt-PCR. If the viremia persisted for >6 months, chronic HEV infection was diagnosed. Results A total of 101 patients had a median age of 8.4 years [interqartile range (IQR): 5.8–11.7]. The seroprevalence of anti-HEV IgG and IgM was 15% and 4%, respectively. Positive IgM and/or IgG were associated with a history of elevated transaminases with an unknown cause after LT (p  =   0.04 and p  =   0.01, respectively). The presence of HEV IgM was associated with a history of elevated transaminases with an unknown cause within 6 months (p  =   0.01). The two patients (2%) diagnosed with chronic HEV infection did not fully respond to the reduction of immunosuppression but responded well to ribavirin treatment. Conclusions Seroprevalence of HEV among pediatric LT recipients was not rare in Southeast Asia. Since HEV seropositivity was associated with elevated transaminases of an unknown cause, investigation for the virus should be offered in LT children with hepatitis after excluding other etiologies. Pediatric LT recipients with chronic HEV infection may receive a benefit from a specific antiviral treatment. [ABSTRACT FROM AUTHOR]

Sarcopenia is common in cirrhotic adults and associated with waitlist mortality and worse outcome after liver transplantation. Psoas muscle mass has been used to define sarcopenia. Therefore, we aimed to determine the association between psoas muscle mass and waitlist mortality as well as post‐transplant outcome in children with end‐stage liver disease. Medical records and abdominal imaging of pediatric liver transplant candidates during 2010–2019 were reviewed. A subset of images was measured by two radiologists to determine inter‐rater reliability. Psoas muscle surface area was determined at intervertebral lumbar disk 3–4 (L3‐4) and 4–5 (L4‐5) levels. PMI was calculated by psoas muscle surface area divided by height squared. We included 105 children, most with biliary atresia (84%). Patients with waitlist mortality had lower PMI compared to the ones who survived to transplantation (PMI at L3‐4 levels 352.8 ± 162.5 vs. 416.8 ± 136.2 mm2/m2 and at L4‐5 levels 497.3 ± 167.8 vs. 571.4 ± 163.4 mm2/m2, both p =.04), but not in the multivariate analyses. For transplanted patients (n = 75), a higher rate of re‐operation (39% vs. 15%, p =.03) and longer hospital stay (53 vs. 45 days, p =.02) were found in patients with lower PMI. Lower PMI is associated with higher re‐operation rate and longer hospital stay following transplantation, but not waitlist mortality. PMI may be taken into consideration with other biomarkers to predict post‐transplant complications. [ABSTRACT FROM AUTHOR]

Liver transplant (LT) is a lifesaving treatment providing excellent clinical outcomes. However, data regarding behavioral and cognitive issues after LT are sparse in Asia. This study aimed to investigate behavior and cognitive problems among pediatric LT recipients. We used the Child Behavior Checklist (CBCL) to evaluate behavior problems and/or the Wechsler Intelligence Scale for Children, Third Edition (WISC-III) to assess cognitive function. Participants were children aged 2 to 18 years who were treated with LT for at least 2 years. We included 77 children with a median age of 7.8 years (interquartile range, 5.0-10.9). All children were evaluated with the CBCL, and 26 children were available for WISC-III assessment. Approximately one-third (34%) of the children had a total CBCL score above a clinical cutoff for significant behavior problems, and 29% of school-aged children had abnormal competence scores in a clinically significant range. Most of the evaluated children (17 of 26; 65%) had a normal full-scale intelligence quotient. Lower cognitive function was associated with having a single parent (P <.01). Higher behavior problems were associated with lower parental education level (P =.01) and correlated with longer post-transplant duration (Spearman's rho = 0.443; P <.001). Significant numbers of children have behavior problems after LT. Most children have normal cognitive function, although a larger sample size is required to confirm this result. Long-term support for cognitive and behavior problems after LT should be implemented, particularly in children with single parents and lower parental education level. • A significant number of children who underwent liver transplant had behavior problems. • Most common problems were affective problems in young children and somatic problems in children aged over 6 years. • Although more behavior problems were related to lower parental education level and longer post-transplant duration, lower cognitive function was associated with having a single parent. [ABSTRACT FROM AUTHOR]

Background: A major aim of the i2b2 (informatics for integrating biology and the bedside) clinical data informatics framework aims to create an efficient structure within which patients can be identified for clinical and translational research projects.Our objective was to describe the respective roles of the i2b2 research query tool and the electronic medical record (EMR) in conducting a case-controlled clinical study at our institution.Methods: We analyzed the process of using i2b2 and the EMR together to generate a complete research database for a case-control study that sought to examine risk factors for kidney stones among gastrostomy tube (G-tube) fed children.Results: Our final case cohort consisted of 41/177 (23%) of potential cases initially identified by i2b2, who were matched with 80/486 (17%) of potential controls. Cases were 10 times more likely to be excluded for inaccurate coding regarding stones vs. inaccurate coding regarding G-tubes. A majority (67%) of cases were excluded due to not meeting clinical inclusion criteria, whereas a majority of control exclusions (72%) occurred due to inadequate clinical data necessary for study completion. Full dataset assembly required complementary information from i2b2 and the EMR.Conclusions: i2b2 was critical as a query analysis tool for patient identification in our case-control study. Patient identification via procedural coding appeared more accurate compared with diagnosis coding. Completion of our investigation required iterative interplay of i2b2 and the EMR to assemble the study cohort. [ABSTRACT FROM AUTHOR]

Late allograft fibrosis in LT recipients can cause graft dysfunction and may result in re‐transplantation. TE is a non‐invasive tool for the assessment of liver fibrosis. We aimed to evaluate the prevalence of allograft fibrosis in pediatric LT recipients, identify factors associated with allograft fibrosis, and determine the diagnostic value of TE, compared to histology. All children who underwent LT for ≥3 years were included. TE was performed for LSM in all patients. LSM of ≥7.5 kPa was considered as abnormal and suggestive of allograft fibrosis. Percutaneous liver biopsy was performed when patients had abnormal LSM and/or abnormal LFTs. Histological fibrosis was diagnosed when METAVIR score ≥F1 or LAF scores ≥1. TE was performed in 43 patients and 14 (32.5%) had abnormal LSM suggestive of allograft fibrosis. Histological fibrosis was identified in 10 of the 15 patients (66.7%) who underwent percutaneous liver biopsy and associated findings included chronic active HBV infection (n = 3), and late acute rejection (n = 3). Multivariate analysis showed that graft age was significantly associated with allograft fibrosis (OR = 1.22, 95% CI: 1.05‐1.41, P = 0.01). In conclusion, late allograft fibrosis is common in children undergoing LT for ≥3 years and associated with graft age. HBV infection and late acute rejection are common associated findings. Abnormal TE and/or LFTs may guide physicians to consider liver biopsy for the detection of late allograft fibrosis in LT children. [ABSTRACT FROM AUTHOR]