Purpose:Gastrointestinal integrity in young population warrants special attention. Infectious pathogens may induce changes in intestinal microbiota, facilitating gut permeability. This study aimed to investigate the gut integrity and inflammatory markers in children with parasitic and non-parasitic infection in Kupang and North Kodi, Indonesia. Methods:A cross-sectional study assessed the anthropometric measurement, socio-demographic factors and personal hygiene practices. Stool samples for helminthic and protozoan infections were analysed using standard microscopy, while blood samples for gut integrity (intestinal fatty acid-binding protein [I-FABP] and fatty acid-binding protein 6 [FABP6]) and inflammatory markers (soluble cluster of differentiation 14 [sCD14] and soluble cluster of differentiation 163 [sCD163]) were assessed using enzyme-linked immunosorbent assay kit. Results:As many as 80 stool samples taken from the children with age of 36-45 months to examine gut parasites in East Nusa Tenggara. Thirty-three from 80 children have intestinal parasites infection and 5 of them infected with 2 types of parasites. A total of 38 intestinal parasites were found with 47.37% protozoa and 52.63% helminths. The most predominant parasites found are Giardia lamblia (21.1%) for protozoans and Trichuris trichiura (26.3%) for helminths. Furthermore, significantly, the median levels of gut integrity biomarkers concentration were higher in non-parasitic group compared to parasitic group, as follows I-FABP 99.50 ng/mL (33.81-393.39); FABP6 56.13 ng/mL (2.43-234.69); sCD14 4.87 ng/mL (1.98-15.06) and sCD163 17.338 ng/mL (1.98-60.92) and significant in FABP6 and sCD14 (p=0.014; 0.001 respectively). Conclusion:In Kupang and North Kodi, intestinal parasitic infections remain a significant concern. The elevated markers of gut integrity and inflammation biomarkers in children with non-parasitic infection are quite concerning and need additional research to justify the causality.
Celiac disease (CD) is an autoimmune disorder triggered by gluten ingestion in genetically susceptible individuals. While gluten exposure is a necessary environmental trigger, its interaction with the intestinal microbiota has recently been recognized as a critical factor in modulating the onset and progression of the disease. Emerging evidence suggests that compositional and functional alterations in the microbial community may contribute to CD's pathogenesis by influencing intestinal barrier integrity, antigen presentation, and the balance between pro- and anti-inflammatory immune responses. Consequently, detailed characterization of microbial signatures holds promise for the identification of biomarkers for early diagnosis and development of microbiota-targeted interventions. This narrative review comprehensively analyzed observational human studies and controlled clinical trials to assess the role of microbiota in pediatric CD. Moreover, dysbiosis patterns across gastrointestinal niches in children with CD were compared and discussed. These findings have the potential for use in novel diagnostic and therapeutic strategies.
Purpose:Inflammatory bowel disease (IBD) is a chronic immune-mediated condition often beginning in childhood, impacting both physical and mental health. Children with IBD may face psychological distress, stigma, developmental challenges and general impact on quality of life (QoL). Residential camps offer holistic support by providing safe, inclusive spaces where children can connect, share experiences, and build resilience. Methods:The study aimed to review the impact and outcomes of residential camps for children with IBD, with regards to factors such as emotional well-being, social functioning and disease-specific knowledge. This review explored the evolution of these camps while considering the mechanisms of benefit, and implications for future practice and research. Comparisons were made to camps for children with other chronic diseases. Results:Studies of IBD camps across multiple countries show consistent psychosocial benefits, including improved QoL, social functioning, confidence, disease knowledge, and reduced isolation. Similar outcomes have also been reported in camps for other chronic illnesses such as diabetes, asthma, and haemophilia, supporting the value of structured, peer-based interventions in enhancing self-management and emotional well-being more generally. Conclusion:Structured routines, peer support and role modelling may be key contributors to the positive effects associated with residential camps for children with IBD. Though formal economic evaluations are limited, the multifaceted benefits of camps - psychological, educational, and potentially physiological - highlight their value in the comprehensive care of children with IBD.
Purpose:Functional abdominal pain disorders (FAPDs) affect 13.5% of children and are challenging to treat owing to their poor quality of life and chronicity. Although antidepressants are potential interventions, the evidence of their efficacy remains inconclusive. This study evaluated the effectiveness of antidepressants in pediatric patients with FAPDs and explored the differences in antidepressant type and treatment duration. Methods:A systematic review of antidepressant efficacy in pediatric FAPDs was conducted using PubMed, EMBASE, Web of Science, and Cochrane Central databases. Randomized controlled trials (RCTs) comparing antidepressants were included. Subgroup analyses assessed the differences according to the antidepressant type (amitriptyline vs. citalopram) and treatment duration (4 weeks vs. 12 weeks). Results:Three RCTs with 287 participants met the inclusion criteria. The pooled odds ratio (OR) for clinical response (symptom improvement) was 3.18 (95% confidence interval [CI], 0.67-15.00), which was not statistically significant (p=0.144). Individual ORs were 1.59 (95% CI, 0.76-3.32) for citalopram, 15.55 (95% CI, 7.07-34.20) for amitriptyline, and 1.30 (95% CI, 0.56-2.99) for amitriptyline. Subgroup analysis suggested a numerically higher effect estimate for longer treatment duration; however, the 12-week finding (OR, 15.55; 95% CI, 7.07-34.20), which was derived from a single study, should be interpreted cautiously. Conclusion:Although antidepressants did not demonstrate statistically significant overall efficacy in pediatric FAPDs, the observed effect estimates suggest a possible therapeutic benefit. Exploratory subgroup analyses indicated that treatment duration might influence symptom improvement; however, these findings were limited by substantial heterogeneity and the small number of trials available. Additional randomized controlled trials are warranted.
Purpose:This study aimed to characterize the clinical features of necrotizing enterocolitis totalis (NEC-T) in infants compared with NEC non-totalis (NEC-non-T) and to identify associated risk factors, thereby providing insights into its management for clinicians and parents. Methods:A retrospective study was conducted at the Guangzhou Women and Children's Medical Centre and included infants with Bell's Stage II or III NEC from January 2015 to March 2021. Infants were divided into NEC-T and NEC-non-T groups based on intraoperative findings. Clinical data were collected and compared between the two groups. Logistic regression was used to identify independent risk factors for NEC-T. Results:Among 195 infants who underwent surgery for NEC, 30 (15.4%) had NEC-T. Significant differences were observed in gestational age, time from onset to surgery (8 hours vs. 46 hours, p<0.001), survival, and intestinal failure. Risk factors for NEC-T included neonatal respiratory distress syndrome (NRDS) (OR, 12.85; p=0.004), ventilatory support after birth (OR, 13.36; p<0.001), bacteremia (odds ratio [OR], 10.98; p<0.001), transfusion (OR, 4.62; p=0.004), increased ventilatory support (OR, 7.34; p=0.001), and portal venous gas (PVG) (OR, 4.78; p=0.007). Conclusion:NEC-T in neonates is characterized by sudden onset, rapid progression, and high mortality. NRDS, ventilatory support after birth, bacteremia, transfusion, increased ventilatory support, and PVG significantly increased the risk of NEC-T. Aggressive surgical decision-making may be a crucial approach to improving survival rates in patients with NEC-T.
Purpose:Although biologics, including anti-tumor necrosis factor (TNF) drugs, are increasingly used for treating pediatric Crohn's disease (CD), the potential loss of response (LOR) to these drugs requires attention. This retrospective study investigated reactive therapeutic drug monitoring (TDM) and the clinical course of pediatric-onset CD in patients treated with adalimumab. Methods:Patients aged <18 years diagnosed with CD and treated with adalimumab were enrolled in this study. Reactive TDM levels, presence of anti-drug antibodies (ADAs), and LOR were evaluated from 2017 to 2019, and clinical outcomes were followed up until June 2022. Results:Thirty-two pediatric patients with CD were enrolled: 14 in the LOR group and 18 in the remission group. The median ages at CD diagnosis and first adalimumab injection were 13 and 14 years, respectively. The median duration of adalimumab injection was 12.5 months. Among 7 patients with therapeutic trough levels (TLs) and undetectable ADAs, 5 achieved clinical remission with continued adalimumab, whereas 2 remained refractory and subsequently switched to alternative biologics. Among 5 patients with subtherapeutic TLs (<5 µg/mL) and undetectable ADAs, 3 eventually underwent a switch to other biologic agents despite interval shortening, while the remaining 2 were ultimately diagnosed with monogenic inflammatory bowel disease (IBD) after further genetic evaluation. Conclusion:Reactive TDM and ADA categorizes the mechanisms of LOR to adalimumab in pediatric CD, guiding appropriate dose escalation or biologic switching. When these strategies fail, exploring underlying monogenic disorders is essential for optimal management.
Purpose:Collagenous gastritis (CG) is a rare pediatric disorder characterized by gastrointestinal symptoms and iron-deficiency anemia. Owing to its low prevalence and broad range of nonspecific manifestations, CG is frequently overlooked during diagnosis. This study aimed to propose a diagnostic algorithm for CG and systematically review the published literature. Methods:Four patients aged <18 years diagnosed with CG at a tertiary referral center in Korea between 2003 and 2023 were retrospectively analyzed. In addition, 73 pediatric cases reported worldwide between 1989 and 2024 were reviewed. Results:Among 77 cases, excluding one case without symptom data, 16 patients (20.8%) had no gastrointestinal symptoms other than anemia. Abdominal pain was reported in 37 patients (48.7%), gastrointestinal bleeding in five (6.6%), vomiting in nine (11.8%), and diarrhea in three (3.9%). Anemia was present in 62 patients, and 68 tested negative for Helicobacter pylori. Most patients demonstrated nodular or irregularly coarse gastric mucosa on esophagogastroduodenoscopy (EGD), although these findings were less apparent during the early disease stage. Conclusion:CG remains a diagnostically challenging condition that requires a high index of suspicion. Early EGD should be considered in pediatric patients with recurrent gastrointestinal symptoms and treatment-refractory iron-deficiency anemia who test negative for H. pylori. Careful evaluation for gastric mucosal nodularity and acquisition of additional histologic biopsies are recommended in these cases.
Purpose:This study aimed to evaluate the effect of histological fibrosis on liver biopsy at presentation on the decision-making and outcomes of Kasai portoenterostomy (KPE), as well as the subsequent timing of liver transplantation. Methods:A retrospective, cross-sectional study of children <13 years old with biliary atresia (BA) seen at the Royal Hospital between January 2007 and December 2021. Results:Forty children were diagnosed with BA, of whom 31 (77.5%) underwent a liver biopsy, and 26 (65.0%) underwent KPE. Thirty children (96.8%) had either complete or impending cirrhosis. Eleven children (42.3%) who underwent KPE received a liver transplantation at a median age of 26 months (interquartile range [IQR]=33-34). Four children did not undergo KPE, and seven (50.0%) of these underwent liver transplantation at a median age of 22 months (IQR=13-25). The age of liver transplantation did not differ statistically between the KPE group and the primary liver transplantation group (p=0.311). Three children (11.5%) were alive with their native liver following KPE at 5 years. The mortality rate was similar in the KPE and non-KPE groups (p=0.171). Conclusion:Of the 40 children with biliary atresia, 31 had a liver biopsy, with 30 (96.8%) exhibiting either complete or impending cirrhosis. Hepatic fibrosis did not impact the decision to perform KPE. The age of liver transplantation did not differ significantly between the KPE and non-KPE groups (median ages of 26 vs. 22 months, p=0.311).
Purpose:Disaccharidase deficiencies, including sucrase-isomaltase deficiency, can cause chronic gastrointestinal symptoms in children. While duodenal biopsies remain the diagnostic gold standard, results may be confounded by specimen handling variability or secondary mucosal injury. The noninvasive 13C sucrose breath test (13CSBT) accurately detects sucrase deficiency, including congenital sucrase-isomaltase deficiency (CSID), while the Trio-Smart® breath test (BT) identifies small intestinal bacterial overgrowth (SIBO), a potential cause of secondary enzyme deficiency. Methods:We conducted a retrospective review of 25 pediatric patients with disaccharidase deficiencies on duodenal biopsy and normal villous architecture. Patients underwent 13CSBT and/or Trio-Smart® BT to evaluate for true CSID or SIBO. Clinical outcomes and treatment responses were assessed. Results:Of 21 patients with low sucrase activities who completed 13CSBT, only 7 (33.3%) had abnormal results consistent with CSID. Six patients received sacrosidase, with three reporting symptom improvement. Of 15 patients who underwent Trio-Smart® breath testing, 9 (60.0%) had abnormal results suggestive of SIBO and responded to antimicrobial treatment. Two patients had abnormal results on both tests. Interestingly, low palatinase levels were associated with abnormal 13CSBT in some cases, though not consistently. Conclusion:Biopsy-based diagnosis may overestimate true CSID due to secondary causes or technical artifacts. Combined use of the 13CSBT and Trio-Smart® BT provides a noninvasive strategy to help distinguish primary or secondary sucrase deficiency, improving diagnostic accuracy and avoiding unnecessary lifelong enzyme therapy. We propose a diagnostic algorithm that integrates biopsy and BT results to guide evaluation and reduce misclassification of CSID.
Purpose: To evaluate the micronutrient status of infants at 1 and 6 months after gastrointestinal surgery and identify the associated factors. Methods: This prospective descriptive study was conducted between February 2023 and August 2024 at the Vietnam National Children's Hospital. Infants who underwent gastrointestinal surgery were monitored for micronutrient deficiency. A total of 120 infants were enrolled, 60 of whom had complete data available for analysis. Among the remaining patients, 12 died from severe infections, and 38 discontinued participation for various reasons, including the high cost of re-examination, lack of time, and follow-up elsewhere. Additional cases were excluded owing to incomplete data for the 6-month follow-up period. Results: The study included 60 infants (58.3% male), all of whom underwent their first gastrointestinal surgery before 3 months of age. Over time, the prevalence of most micronutrient deficiencies decreased, except for zinc and iron deficiencies. One month post-surgery, anemia and vitamin D deficiency were the most common micronutrient deficiencies (55% and 57%, respectively). At 6 months post-surgery, zinc deficiency was the most prevalent (47%), followed by iron deficiency (33%). The 6-month prevalence of zinc deficiency was significantly higher in infants with intestinal failure than in those without it (p<0.008). The rate of hyponatremia was significantly higher in infants who underwent enterostomy than in those who did not (p<0.001). Conclusion: Infants undergoing gastrointestinal surgery are at risk of persistent micronutrient deficiencies, particularly zinc, vitamin D, and iron deficiencies. Notably, zinc deficiency was more common in infants with intestinal failure than in those without.
Purpose:The prevalence of steatotic liver disease (SLD) in adolescents and young adults (AYA) is poorly understood. While the recognition of the importance of this condition as a precursor to serious liver disease is growing, the impact on young people is not well documented. The majority of SLD data is derived from older subjects undergoing a clinically indicated procedure. This study seeks to better describe the prevalence and associated factors for SLD in AYA. Methods:A convenience sample of 241 patients aged 14-30 in Northern California was recruited. Demographic, dietary, exercise and biometric data were collected. Logistic regression models were applied to assess the association between collected data and body mass index (BMI) categories with SLD presence. A diagnostic cut-off of controlled attenuation parameter ≥238 dB/m on transient elastography was used to define SLD. Results:The prevalence of SLD was 59/241 (24.8%). BMI was a predictor of SLD, with 0.0% (0/12) of underweight, 8.5% (11/129) of normal, 28.1% (18/64) of overweight and 83.3% (30/36) of obese participants having hepatic steatosis in this sample. Conclusion:The prevalence of SLD was unexpectedly high, particularly among subjects who were overweight or obese. The increasing diagnosis of SLD in young populations requires a better understanding of factors that may predict or protect from developing fatty liver. Similar to previous reports, BMI was the main predictor of SLD in our population. This study highlights a possible concerning finding of SLD starting at a young age and for consideration of standardized guidelines for screening in AYA.
Purpose:Published data on pediatric gastroenterology, hepatology, and nutrition (PGHN) training centers in the Asia-Pacific region are limited. This study aimed to evaluate the infrastructure, resources, and training opportunities in PGHN centers across the region to inform future development of training programs. Methods:We conducted an international multicenter, cross-sectional survey among Asia-Pacific nations between August 2023 and July 2024. Results:A total of 43 responses were received from 11 countries. Most centers (58.1%) operated with ≤3 specialists, and trainees in these centers were less likely to receive formal supervision or participate in research. Procedural exposure varied significantly: although endoscopy was widely available, 50% of centers reported fewer than 100 colonoscopies performed annually. Access to training in specialized procedures was limited (pH/impedance, 48.8%; high-resolution manometry, 34.9%; intestinal ultrasound, 41.9%), with radiologists performing most liver biopsies and intestinal ultrasounds in many centers. Conclusion:This first comprehensive survey of PGHN training in the Asia-Pacific region identified considerable variations, with key challenges in training infrastructure and procedural exposure. Most centers operate with limited specialist numbers, impacting supervision and research opportunities, and many struggle to meet international volume-based training requirements for essential procedures. Enhanced regional collaboration and alternative training approaches may help address these gaps.
Purpose:Chronic liver disease (CLD) in children often leads to portal hypertension and varices. Upper GI endoscopy (UGIE) is gold standard for variceal detection; however, it is invasive and requires anesthesia. Liver stiffness measurement (LSM) has emerged as a promising non-invasive alternative for variceal screening. This systematic review and meta-analysis was conducted to evaluate diagnostic accuracy of LSM for predicting any grade of esophageal and/or gastric varices (Vx) and high-risk varices (HRVx) in pediatric CLD. Methods:A systematic search of PubMed, Scopus, and Embase was conducted to include studies reporting LSM-based diagnostic accuracy data for Vx/HRVx prediction in CLD patients ≤18 years, with UGIE as reference standard. Pooled sensitivity, specificity, diagnostic odds ratio (DOR), and area under receiver operating characteristic curve (AUC) were estimated. Results:Twelve studies (n=647) were included. For prediction of Vx (six studies, n=327), LSM demonstrated pooled sensitivity of 0.85 (95% confidence intervals [CI]: 0.78-0.90), specificity of 0.74 (95% CI: 0.67-0.80), DOR of 16.35 (95% CI: 7.06-40.24), and AUC of 0.847. For HRVx (seven studies, n=369), pooled sensitivity, specificity, DOR and AUC were 0.81 (95% CI: 0.74-0.87), 0.79 (95% CI: 0.73-0.84), 14.19 (95% CI: 5.14-35.98) and 0.862, respectively. Considerable heterogeneity (I2=73.5%) was noted in specificity for HRVx. Subgroup analyses identified LSM modality, cutoff values, ethnicity, and variceal prevalence as sources of heterogeneity. Conclusion:LSM holds promise as a non-invasive tool for variceal screening in pediatric CLD. However, it should be regarded as a complement rather than a replacement for endoscopy until standardized protocols with prospectively validated thresholds are established.
Purpose:Data on treatment response in children with celiac disease (CeD), particularly with respect to changes in tissue transglutaminase IgA antibodies (tTGA-IgA), cytokines, intestinal fatty acid-binding protein (I-FABP), and quantitative histology, remain limited. To evaluate the utility of non-invasive parameters (NIPs), including I-FABP, tTGA-IgA, and cytokines, as potential markers of mucosal recovery in pediatric CeD. Methods:This single-center, prospective, observational follow-up study included children with newly diagnosed CeD. Blood and duodenal biopsy samples were obtained at baseline and after 1 year of follow-up. Results:Seventy-nine children who underwent follow-up biopsy at 1 year were analyzed. Significant improvements were observed in anthropometric measures, histological parameters, tTGA-IgA levels, cytokine profiles (tumor necrosis factor-alpha [TNF-α], interferon-gamma [IFN-γ], interleukin [IL]-15, IL-6, and IL-10), and I-FABP levels. tTGA-IgA showed strong correlations with histological indices, including intraepithelial lymphocyte (IEL) count (r=0.554, p<0.001), villous height (r=-0.557, p<0.001), crypt depth (r=0.553, p<0.001), and villous-to-crypt ratio (VCR; r=-0.561, p<0.001). TNF-α demonstrated weak correlation with crypt depth (r=0.24, p=0.04) and VCR (r=-0.22, p=0.05). IL-15 levels (r=0.34, p=0.003) and Δcycle threshold (Ct) scores correlated with IEL counts (r=-0.22, p=0.048). IFN-γ ΔCt scores also correlated with IEL counts (r=-0.34, p=0.001). Conclusion:Among the NIPs evaluated, tTGA-IgA showed the strongest correlation with mucosal recovery at 1 year. TNF-α, IL-15, and IFN-γ demonstrated weaker but significant correlations and may have the potential as adjunctive NIP of mucosal healing in pediatric CeD.
Purpose:Current World Health Organization guidelines recommend F-75/F-100 for inpatients and ready-to-use therapeutic foods (RUTF) for outpatients with severe acute malnutrition (SAM). In Indonesia, F-100 continues to be administered after discharge because of limited evidence of RUTF's acceptability and effectiveness in a local setting. Home-prepared F-100 carried the risk of improper mixing and bacterial contamination. Therefore, ready-to-drink oral nutritional supplements (RTD ONS) may serve as alternatives to F-75/F-100. This study aimed to investigate the safety and effectiveness of ONS in hospitalized children with SAM. Methods:This study was a pilot randomized controlled trial comparing the gastrointestinal tolerance of F-75/F-100, high-energy ONS, and standard-energy ONS in 108 hospitalized subjects aged 12-60 months old with complicated SAM. Subjects were monitored for gastrointestinal symptoms and refeeding syndrome. Results:The incidence of vomiting due to the formula was not significantly different among the three groups (p=0.362), as was the incidence of diarrhea, which was 13.9% (F-75/F-100), 16.7% (high-energy ONS), and 11.1% (standard-energy ONS) (p=0.939). The incidences of refeeding syndrome were 13.9%, 5.6%, and 2.8% in the F-75/F-100, standard-energy ONS, and high-energy ONS groups, respectively (p=0.169). The high-energy ONS showed the highest weight increment (8.5±3.2 g/kg/day) among the three groups. Conclusion:Ready-to-drink ONS is a safe and effective therapeutic nutritional option for children with complicated SAM who live in areas with poor sanitation. This study provides preliminary results and requires further investigation to address the limitations of a small sample size and short study timeframe.
Purpose:Endoscopic variceal hemostasis is a critical, life-saving intervention frequently performed by pediatric gastroenterologists. To ensure safe and effective practices, the Asian Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition Endoscopy Scientific Subcommittee developed a consensus-based position statement. Methods:A comprehensive literature review was conducted with an emphasis on pediatric evidence. Nineteen draft statements were formulated and refined through electronic votes and virtual meetings. Statements with <80% agreement were revised until consensus was reached. Levels of evidence certainty were evaluated to support the final recommendations. Results:Nineteen position statements, covering screening, acute management, and surgical options, were endorsed. Although noninvasive tools such as elastography and platelet-based indices may assist in risk stratification, endoscopy remains the diagnostic and therapeutic gold standard. Endoscopic variceal ligation was identified as the preferred modality for both prophylaxis and treatment in children ≥10 kg, outperforming sclerotherapy and non-selective beta-blockers. Standard acute management incorporates octreotide and restrictive transfusion strategies, with endoscopy performed within 24 hours. In refractory cases, the Meso-Rex stunt or transjugular intrahepatic portosystemic shunt may be considered depending on anatomy and available expertise. Conclusion:These findings provide an evidence-based framework for the management of pediatric variceal bleeding. Individualized care is emphasized while highlighting the pressing need for high-quality pediatric studies to strengthen future recommendations.
Purpose:Excessive sodium intake in children can increase the risk of noncommunicable diseases in adulthood. Globally, children below 5 years of age consume approximately 2,000 mg of sodium daily, exceeding the recommended limit. However, studies on sodium intake among Indonesian children, especially those below 2 years of age, are limited. The aim of this study was to evaluate daily sodium intake and its associated risk factors. Methods:In this cross-sectional study, the daily sodium intake and its associated risk factors were assessed in 85 healthy children aged 6-24 months in Jakarta between June and August 2024. Sodium intake was measured using urinary excretion (International Study on Salt and Blood Pressure formula), and dietary analysis was conducted using the Food Frequency Questionnaire. High sodium intake was defined per the Indonesian Pediatric Society guidelines. Results:Results showed that 64% of children aged 6-11 months consumed over 1,200 mg/day of sodium (mean: 1,289.20±639.25), while 37% of children aged 12-24 months had high sodium intake (mean: 1,242.03±584.35). Family socioeconomic status significantly correlated with high sodium intake (r=0.176). Specific food types were identified as risk factors for excessive sodium consumption. In this study, it was concluded that almost all children aged 6-24 months had sodium levels above the recommended levels. Notably, all children aged 6-11 months had a higher sodium intake than approximately one-third of children aged 12-24 months. Conclusion:These findings highlight the need for targeted interventions to reduce sodium intake among young children, especially among families with higher socioeconomic status.
Purpose:Congenital hepatic fibrosis (CHF) and/or autosomal recessive polycystic kidney disease (ARPKD) represent rare and complex clinical conditions in childhood. Diagnostic challenges often arise due to heterogeneity in clinical manifestations. Methods:This study included pediatric patients diagnosed with CHF and/or ARPKD who were followed by the Pediatric Gastroenterology and Pediatric Nephrology Departments. Patient records were reviewed retrospectively. Results:A total of 23 patients were included in the study. The median age of the cohort was 12.7±4.8 years, and the median age at diagnosis was 0.6±3.4 years. Thirteen patients had combined CHF and ARPKD, while 10 had isolated ARPKD. The diagnosis was incidental in 13 patients (56.5%), whereas five patients (21.7%) presented with an abdominal mass. Most patients had mutations in the polycystic kidney and hepatic disease 1 gene. Bilateral kidney enlargement and multiple millimetric cysts were identified in the majority of cases. Three patients required organ transplantation during follow-up. Two patients who underwent liver or kidney transplantation experienced no complications, whereas the patient who received combined liver and kidney transplantation developed kidney failure secondary to reflux nephropathy. Except for one patient who died in infancy, disease progression was generally mild in the cohort. Conclusion:Although kidney involvement is often predominant, hepatic complications may develop over time, particularly in patients with combined disease. A collaborative, multidisciplinary approach is essential for effectively managing the complex manifestations of these ciliopathies.
Purpose:Cyclic vomiting syndrome (CVS), is a diagnosis of exclusion within the disorders of the gut-brain interaction, consisting of recurrent vomiting episodes unexplained by another medical condition or its triggers. When a patient presents with a recurrent vomiting episode, a broad differential is considered. The aim of this systematic review is to provide a hierarchal differential diagnostic approach to children with known or possible recurrent vomiting. Methods:This study followed the Preferred Reporting Items in Systematic Reviews and Meta-Analyses (PRISMA) guidelines. PubMed, EMBASE and Scopus databases were searched using the following criteria: (1) Subjects aged 18 or under with vomiting spells classified as cyclic vomiting syndrome according to Rome IV criteria or equivalents for older publications and; (2) experimental, observational, or cross-sectional studies of 10 or more patients. A comprehensive literature search was performed with specific search criteria that included the diagnosis of cyclic vomiting syndrome. The results were analyzed using a Bayesian methodology to determine the rate estimates and 95% credible intervals for each disease. Results:Seventeen studies (1,375 patients and 1,415 events) were recovered. 80.4% of the differential included: psychological stress (26.2%; 95% credible intervals [CredI], 24-28.5), infection (22%; 95% CredI, 19.9-24.2), motion sickness (19.3%; 95% CredI, 17.3-21.4) and migraine (12.9%; 95% CredI, 11.2-14.7). Surgical causes (4.8%; 95% CredI, 3.6-6.2) and mitochondrial diseases (0.4%; 95% CredI, 0.1-0.8) were uncommon. Conclusion:Many triggers and diagnoses are associated with recurrent vomiting and CVS: psychological stress, infection, motion sickness, and migraine accounted for most cases. Serious surgical or metabolic diseases were less common.
