Twenty years' experience of midgut malrotation and volvulus in a tertiary center in northern Taiwan: A retrospective study.

BACKGROUND: Early diagnosis and surgical intervention for midgut malrotation with bowel obstruction are crucial. We aimed to identify risk factors for adverse outcomes in infants with midgut malrotation and to develop a prediction model. METHODS: We reviewed the operation records of infants surgically diagnosed with midgut malrotation at Chang Gung Children's Medical Center between January 2000 and December 2020. Patients were classified into the poor-outcome group (PO) if they underwent bowel resection or experienced mortality; all others were categorized as the favorable-outcome group (FO). Data on demographics, initial presentations, laboratory results, radiographic or sonographic findings, maternal conditions, and outcomes were collected and analyzed. Fisher's exact test, the independent sample t-test, and the Mann-Whitney test were utilized for comparative analysis when suitable. RESULTS: The study included 103 infants. Eleven were in the PO group, and 92 were in the FO group. Initial presentations such as respiratory distress, poor activity, and shock status were notably more prevalent in the PO group. The INR, hemoglobin, HCO3, base excess, and aspartate transaminase values showed significant variation between the two groups. Multivariate analysis identified that lower hemoglobin (OR 0.677, p = 0.043) and higher AST (OR 1.036, p = 0.044) were independent predictors of adverse outcomes. An AST/Hb ratio of <3.78 demonstrated a high negative predictive value (98.6%) for an adverse outcome in midgut malrotation. CONCLUSIONS: Prompt diagnosis and surgical treatment of midgut malrotation are vital to prevent bowel resection or mortality. The independent predicting factors for poor outcomes include low hemoglobin and elevated AST levels.

The trends of pediatric duodenal ulcer and predictors of recurrence.

BACKGROUND: Duodenal ulcer (DU) causes various symptoms in children. The prevalence of Helicobacter pylori (Hp)-associated DU has been reducing in some regions, yet the updated trend in Taiwan is unknown. Risk factors of DU recurrence have not been comprehensively investigated in children. METHODS: This retrospective study included children diagnosed with DU to evaluate the demographics, symptoms, diagnostics, treatment, and outcomes. Specific populations (infant, surgery required) were sorted for subgroup analysis. Predictors of DU recurrence was analyzed in patients who received endoscopic follow-ups. RESULTS: A total of 488 children were included. Most patients were male (72.5%), school-aged (11.3 ± 4.8 years old), and with varied underlying diseases in one-fifth. The annual incidences were around 3-5%, with a declining trend of case numbers and the Hp-positive proportion. Hp infection, concurrent gastric ulcer, perforation, and mortality were noted in 32.7%, 16%, 1.6%, and 1% of patients. Patients with or without Hp infection showed different clinical features but similar outcomes. The characteristics of subpopulations were depicted respectively. Male sex, lower Hb level, and perforation were independent risk factors associated with recurrence. CONCLUSIONS: Hp-positive DU seems to wane. Patients with male sex, lower Hb level, or perforation at diagnosis carried a higher risk of recurrence, which may warrant active surveillance and endoscopic follow-up.

Clinical significance of incidental common bile duct dilatation in children: A 10-year single medical center experience.

BACKGROUND: With the widespread use of abdominal ultrasonography (US), incidental detection of common bile duct (CBD) dilatation is common in pediatric populations. This study investigated the causes and clinical significance of CBD dilatation in children without biliary symptoms, jaundice, or causative lesions in US. METHODS: We retrospectively reviewed pediatric patients with CBD dilatation from July 2013 to June 2023. All cases were detected via abdominal US. We analyzed the patients' clinical manifestations, laboratory data, diagnosis, underlying diseases, and clinical course. RESULTS: In a total of 687 patients enrolled, 338 met inclusion criteria (90 in hepatobiliary, 248 in CBD dilatation group). Of 128 patients with incidental CBD dilatation who underwent regular US examinations, 91 (71.1%) experienced resolution during follow-up. The proportion of patients with intrahepatic duct dilatation was significantly higher in the non-resolution group (p = 0.038). General health examination group had significant smaller CBD diameter compared to the gastrointestinal and infection groups. Correlation analysis found starting point of resolution decline at 3.24 mm (all-inclusive) and 2.51 mm (infant group) CBD diameter. CONCLUSIONS: Most children with incidental CBD dilatation did not have abnormal hepatobiliary function or other sonographic abnormalities. They usually remained asymptomatic and experienced uneventful clinical courses.

Primary biliary cholangitis with features of autoimmune hepatitis in a 19-year-old adolescent with 14q24.1q24.2 deletion: a case report.

Introduction: Primary biliary cholangitis (PBC) is a rare and chronic autoimmune liver disease characterized by the progressive destruction of small intrahepatic bile ducts that may eventually lead to cirrhosis. PBC with features of autoimmune hepatitis (AIH) has rarely been reported in pediatric patients with genetic defects. We present the case of an adolescent with chromosome 14q24.1q24.2 deletion who was given the diagnosis of stage IV PBC with features of AIH. Case presentation: A 19-year-old male adolescent with multiple congenital abnormalities and an intellectual disability presented with abnormal liver enzymes levels and pruritus for more than 5 years. Laboratory examinations revealed elevated levels of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and gamma-glutamyl transpeptidase. After the exclusion of viral hepatitis, alpha-1 antitrypsin deficiency, Wilson's disease, and other genetic cholestatic liver diseases by laboratory tests and whole exome sequencing, a liver biopsy was performed and stage IV PBC was diagnosed. Notably, features of AIH were also noted in the histopathological report, indicating the presence of PBC with AIH features. The patient responded well to a combination therapy of ursodeoxycholic acid and steroids. Array comparative genomic hybridization analysis performed to study the congenital abnormalities revealed a 3.89 Mb 14q24.1q24.2 deletion. Conclusion: PBC with AIH features has rarely been reported in an adolescent with a chromosomal abnormality. The present case can increase awareness for early-onset PBC and its possible correlation with chromosomal defects.

Zinc Deficiency and Therapeutic Value of Zinc Supplementation in Pediatric Gastrointestinal Diseases.

The benefits of zinc in treating certain gastrointestinal (GI) diseases have been recognized for over two decades. This review aims to explore zinc deficiency (ZD) and the potential therapeutic value and safety of zinc supplementation in pediatric GI diseases. A systematic review of published articles on ZD and zinc as adjuvant treatments for GI diseases was conducted using various databases. Children with inflammatory bowel disease (IBD), celiac disease, and those receiving long-term proton pump inhibitor treatments are particularly susceptible to ZD. ZD in children with celiac disease and IBD is attributed to insufficient intake, reduced absorption, and increased intestinal loss as a result of the inflammatory process. Zinc plays a crucial role in maintaining the integrity of the gastric mucosa and exerts a gastroprotective action against gastric lesions. Although considerable evidence supports the use of zinc as adjuvant therapy for certain GI diseases in adults, its use is unspecified in children except for infectious diarrhea. Current evidence suggests that zinc supplementation with well-documented dosages helps reduce the duration of diarrhea in children with acute or persistent diarrhea, while there are no specific guidelines for zinc supplementation in children with IBD and celiac disease. Zinc supplementation appears to be beneficial in peptic ulcer disease or gastroesophageal reflux disease. The available evidence highlights the need for intervention programs to enhance zinc status and reduce the morbidity of certain GI diseases in children.

Adenovirus infection is a risk factor for recurrent intussusception in pediatric patients.

BACKGROUND: Human adenoviruses are the most common pathogens to be isolated from cases of pediatric intussusception. However, the specific clinical characteristics of pediatric intussusception associated with adenovirus infection are poorly known. METHODS: We reviewed the medical records of pediatric patients (≤18 years of age) with intussusception treated between January 2014 and December 2020. We enrolled patients with febrile episodes, 27 with and 29 without adenovirus infections (the latter serving as control group). The demographic data, clinical characteristics, and the diagnoses and management strategies were evaluated. RESULTS: The adenovirus group exhibited a significantly longer febrile duration (4.3 ± 1.9 vs. 3.3 ± 1.1 days, p = 0.020) than the control group, with an odds ratio (OR) of 5.098 (95% confidence interval [CI] 1.223-21.254, p = 0.025). The recurrence rates were 48.1% and 13.8% in the two groups (OR 5.804; 95% CI: 1.585-21.245, p = 0.008). Most adenoviruses were non-enteric (85.2%). CONCLUSION: Adenovirus-related intussusception is associated with a longer febrile period and a higher rate of intussusception recurrence. It is recommended that patients suspected of adenovirus-related intussusception should be observed for longer than others prior to discharge.

Effect of the coronavirus disease 2019 pandemic on pediatric emergency department visits for acute gastroenteritis evaluated using a validated clinical severity score.

BACKGROUND: The coronavirus disease 2019 (COVID-19) outbreak that began in late 2019 has significantly affected quality of life and healthcare. Approaches to prevent the spread of COVID-19 have also affected the prevalence of other diseases. This retrospective review evaluated pediatric emergency department (PED) volume, in terms of children with acute gastroenteritis (AGE), and changes in AGE severity before versus during the COVID-19 pandemic in a tertiary medical center in Taiwan. METHODS: Patients who visited the PED and were diagnosed with AGE during the 70-day COVID-19 lockdown in 2021, or the identical period in 2020, were compared using a clinically validated AGE severity score, the modified Vesikari score (MVS), and additional parameters. RESULTS: During the COVID-19 outbreak, there was a 61.4% reduction in the number of children with AGE visiting the PED. In that period, the AGE severity score was similar compared to the pre-pandemic period (9.00 vs. 8.57, p = 0.273). The mean C-reactive protein (CRP) level (55.7 vs. 40.6 mg/L, p < 0.001) and rate of antibiotics use (48% vs. 23.5%, p < 0.001) were higher during the outbreak than the pre-pandemic period. CONCLUSION: The number of children with AGE visiting the PED decreased during the COVID-19 outbreak, while disease severity was unchanged compared to the pre-pandemic period. The use of antibiotics during the COVID-19 pandemic warrants further investigation.

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Quantitative ultrasound envelope statistics imaging as a screening approach for pediatric hepatic steatosis and liver fibrosis: using biomarker and transient elastography as reference standards.

Quantitative ultrasound (QUS) envelope statistics imaging is an emerging technique for the assessment of hepatic steatosis in adults. Blood tests are currently recommended as the screening tool for pediatric hepatic steatosis, a condition that can lead to liver fibrosis in children. This study examined the utility of QUS envelope statistics imaging in grading biomarker-diagnosed hepatic steatosis and detecting liver fibrosis in a pediatric population. A total of 173 subjects was enrolled (Group A) for QUS envelope statistics imaging using two statistical distributions, Nakagami and homodyned K (HK) models, and information entropy. QUS parameter values were compared with the hepatic steatosis index (HSI) and steatosis grade (G0: HSI <30; G1: 30 ≤ HSI <36; G2: 36 ≤ HSI <41.6; G3: ≥41.6). An additional cohort of 63 subjects (Group B) was recruited to undergo both QUS envelope statistics imaging and liver stiffness measurements (LSM) obtained from the transient elastography (Fibroscan), with a cutoff value set at 5 kPa to indicate liver fibrosis. The diagnostic performances were evaluated using the area under the receiver operating characteristic curve (AUROC). QUS envelope statistics imaging generated the AUROC values for steatosis grading at levels ≥ G1, ≥ G2, and ≥ G3 ranged from 0.94 to 0.97, 0.91 to 0.93, and 0.83 to 0.87, respectively, and produced an AUROC range of between 0.82 and 0.84 for identifying liver fibrosis. QUS envelope statistics imaging integrates the benefits of both biomarkers and elastography, enabling the screening of hepatic steatosis and detection of liver fibrosis in a pediatric population.

Application of Argon Plasma Coagulation for Gastrointestinal Angiodysplasia in Children- Experience From a Tertiary Center.

Background: Argon plasma coagulation (APC) has been applied in adults to treat various diseases, including vascular lesions in the gastrointestinal (GI) tract. However, angiodysplasia (AD) is an uncommon cause of pediatric GI bleeding, while the experience of treating AD with APC was rarely reported. Methods: Five children with AD in the GI tract successfully treated with APC were reviewed. Results: Three of the five patients were girls, and the age at diagnosis ranged from 1.5 months to 10.5 years of age. One patient with gastric AD manifested with tarry stool, and the rest had colonic AD, which caused various degrees of bloody stool. Three patients had evident anemia. All patients received an endoscopic diagnosis, and two had compatible findings in radiographic exams. Each patient underwent one APC treatment session, and none encountered procedure-related complications or re-bleeding. Conclusion: AD can be an etiology of GI bleeding even in neonates. APC is an effective and safe therapy for symptomatic AD in children.

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations.

Hydrometrocolpos (HMC) is a rare condition where fluids or secretions accumulate in the vagina (hydrocolpos) or up to the uterus (hydrometrocolpos). This case series study reports three infants with different etiologies and presentations of HMC and aims to review literature for proper workup upon initial diagnosis. The first neonate antenatally presented with a huge cystic mass. HMC secondary to imperforate hymen was proved, and hymenotomy was performed at 2 days of age. The second participant presented with persistent urogenital sinus and hematopoietic chimerism, possibly due to transfusion from her twin brother via placenta anastomoses. At 2 months of corrected age, she had difficult defecating, and sonogram revealed HMC with normal appearance of uterus and ovaries. Regular follow-ups and surgical reconstruction will be conducted before puberty. The third patient had cloacal malformation and multiple congenital anomalies at birth. Vesicovaginal fistula-related HMC was detected and managed with surgical drainage in the neonate stage. The girl began menstruation with dysmenorrhea at 12 years. The image studies demonstrated hematometrocolpos secondary to left-side hemivaginal septum, uterine didelphy, and ipsilateral renal agenesis, indicating Herlyn-Werner-Wunderlich syndrome. HMC can be diagnosed easily via sonogram. Careful external genitalia examinations help to identify persistent urogenital sinus or cloacal malformation. Occasionally, the HMC may be part of syndrome manifestations or associated with sex chromosome anomalies. Clinicians may conduct surveillance of renal, cardiac, and skeletal systems as well as chromosome study for early diagnosis and management.

Factors associated with pyloric hypertrophy severity and post-operative feeding and nutritional recovery in infantile hypertrophic pyloric stenosis.

BACKGROUND: To examine factors that affect the severity of pyloric hypertrophy, post-operative feeding and nutritional recovery in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Medical records of infants diagnosed with IHPS at a single tertiary center between 2009 and 2018 were retrospectively reviewed. Clinical characteristics, biochemistry data and outcome were assessed for their association with the severity of pyloric hypertrophy and post-operative recovery. Nutritional recovery was assessed using weight-for-age status improvement after surgery. RESULTS: Eighty-five patients were recruited in this study. The mean pre-operative weight-for-age percentile was 18.2. Elevated bicarbonate was positively correlated with symptom duration (p = 0.007). Pyloric muscle thickness was significantly correlated with age, weight, and symptom duration (p = 0.004, 0.003, 0.008, respectively). The mean weight-for-age percentile increased to 41.6 by post-operative weeks 6-8. Pyloric muscle thickness was negatively correlated with nutritional recovery by post-operative weeks 6-8 (p = 0.003). In multivariable analysis, pyloric length related to nutritional recovery at week 1-2 postoperatively (OR = 1.42, p = 0.030, 95% CI = 0.03-1.94), and pyloric muscle thickness related to nutritional recovery at week 6-8 postoperatively (OR = 4.08, p = 0.032, 95% CI = 1.13-14.7). CONCLUSION: Our study indicated that favorable nutritional outcome and successful weight gain was observed 6-8 weeks after surgery in children with IHPS. Pyloric muscle thickness positively correlated with age, weight, symptom duration, and favorable nutritional recovery. Serum bicarbonate showed a positive correlation with symptom duration.

Primary endoscopic variceal ligation reduced acute variceal bleeding events but not long-term mortality in pediatric-onset portal hypertension.

BACKGROUND/PURPOSE: Esophageal variceal bleeding (EVB) is a medical emergency in patients with portal hypertension (PHT). However, studies on the long-term outcomes of prophylactic endoscopic variceal ligation (EVL) in pediatric-onset PHT are lacking. METHODS: Between 1999 and 2020, patients who received EVL in the Electronic Report System of the Pediatric Endoscopy Unit were included in this retrospective study. EVL was classified as primary prophylaxis when it was performed for esophageal varices (EVs) without previous bleeding. If it was implemented in acute EVB, the subsequent EVL was classified as secondary prophylaxis. RESULTS: Fifty-eight patients aged 10 months to 33 years with 31 males were included. Thirty-eight patients were classified as primary prophylaxis group, and twenty, secondary prophylaxis group. The primary prophylaxis group experienced fewer 5-year EVB events than the secondary prophylaxis group (cumulative risk: 14.4% versus 32.4%). Still, it didn't significantly affect overall survival and biliary atresia transplant-free survival. Long-term mortality was significantly associated with higher serum direct bilirubin levels (≥0.55 mg/dL) and lower albumin levels (≤2.54 mg/dL) at the first EVL. Aspartate aminotransferase-to-platelet ratio index (APRI) with a cut-off value of 1.24 helped to predict EV presence at the initial esophagogastroduodenoscopy (EGD) (AUROC = 0.762, sensitivity 75.0%, and specificity 66.7%). CONCLUSION: Primary prophylactic EVL, despite reducing acute EVB, may not change overall survival and biliary atresia transplant-free survival. APRI > 1.24 may predict EV presence at the first EGD and help to schedule a surveillance EGD. Higher direct bilirubin and lower albumin levels at the first EVL may relate to long-term mortality.

Clinical Presentations, Diagnosis, and Management for Pediatric Antral Web-A 20-Year Experience of a Referral Center.

Background: Antral web is a rare cause of gastric outlet obstruction in children. The presentation is diverse, depending on the degree of obstruction. Unfortunately, the guidance of management is still lacking. Methods: This study retrospectively evaluated the presentations, management, and outcomes of the pediatric antral web based on a 20-year experience in a referral center. Results: A total of 23 cases were included. The median age of diagnosis was 10 months (interquartile range, IQR, 0.8-23 months). Main presentations comprised vomiting (83%) and upper gastrointestinal (UGI) bleeding (48%). Concurrent gastric ulcers were common (68%). A total of 13 cases (57%) underwent interventional treatment. The median duration from diagnosis to intervention (DtI) was 10 days, but five with longer DtI, ranged from 30 to 755 days. Among the 15 cases with concurrent gastric ulcers, 10 patients received intervention, immediately in six but delayed in four. Surgical treatments (N = 12) achieved a cure in 11, with one rescued by endoscopic treatment. Conclusions: Children who suffer from early gastric ulcers with outlet obstruction shall raise the suspicion of the antral web. Complete obstruction madates early intervention. Around half of the cases with adequate feeding and growth need no intervention. Recurrent obstructive symptoms or adjacent ulcers justify a switch from observation to intervention to avoid complications or growth faltering.

Comparison of Body Mass Index and Fat Indices in Predicting the Severity of Nonalcoholic Fatty Liver Disease Among Children Who Are Overweight and Obese.

Background: Information of the relationships between body mass parameters and the severity of fatty liver is deficient in pediatric nonalcoholic fatty liver disease (NAFLD). Methods: The relationships between body mass parameters (waist circumference [WC], body mass index [BMI], and abdominal subcutaneous fat thickness [ASFT]) and the severity of fatty liver were prospectively evaluated in pediatric patients who are overweight or obese, suffering from NAFLD. Ultrasonography was performed to assess fatty liver and its severity on a three-grade scale (low-grade fatty liver [LGFL], grade 1 or 2; high-grade fatty liver [HGFL], grade 3). Results: A total of 110 subjects (55 LGFL and 55 HGFL) aged 6.2-17.9 years were included. The WC, BMI, and ASFT values were significantly higher in the HGFL group compared to those in the LGFL group (p = 0.00004, 0.01, and 0.04, respectively). WC had the greatest power to predict HGFL under receiver-operating characteristic curve analyses and was positively correlated with the severity of fatty liver in subjects aged 6-12-year old and 13-17-year old (p = 0.007, and 0.0039, respectively). ASFT showed a positive correlation with the severity of fatty liver in subjects aged 13-17-year old (p = 0.04). Conclusions: WC, BMI, and ASFT are predictive of severe NAFLD among children who are overweight and obese; particularly, WC has the most predictive accuracy. Among the parameters, WC and ASFT are predictive in specific age groups.