Transaldolase is a key enzyme in the pentose phosphate pathway, an alternative pathway for glucose metabolism. Transaldolase deficiency leads to the accumulation of metabolites such as polyols and seven-carbon sugars, particularly in urine. Transaldolase deficiency is a rare disorder with multisystem involvement, including liver dysfunction, hepatosplenomegaly, anemia, thrombocytopenia, endocrine abnormalities and dysmorphic features. We now report on two siblings with kidney involvement due to transaldolase deficiency. Kidney dysfunction is common in transaldolase deficiency and may progress to chronic kidney disease (CKD). The disease may have an early onset, but it can also progress without showing any sign of kidney impairment until adulthood. Transaldolase deficiency should be considered among the causes of proteinuria and CKD of unknown origin, and these patients should be carefully monitored for kidney function. Although the disease has been generally reported to manifest with tubular dysfunction, one of our patients had both tubular and glomerular involvement, which started at an earlier age than in her younger sister.

BACKGROUND:Primary immunodeficiencies (PIDs) and eosinophilic gastrointestinal diseases (EGIDs) may share common underlying defects. However, no studies have investigated the frequency of PIDs among patients with EGIDs. In this study, we aimed to assess the frequency and spectrum of PIDs among our pediatric EGIDs patients. METHOD:Patients were prospectively evaluated over a two-year period. All patients were questioned according to ten warning signs of the Jeffrey Model Foundation. Routine laboratory tests and basic immunologic tests [serum immunoglobulin levels, isohemagglutinin titres, anti-HBs and anti-rubella Ig G titres] were performed on all participants. Advanced immunologic workup was performed in selected cases. All PID diagnoses were established according to the European Society for Immunodeficiencies' criteria. Electronic health records were screened for EGIDs' specific features, comorbidities, and previous laboratory tests. RESULTS:A total of 88 EGID patients [76% male, mean age: 12.02 years, 78 patients (88.6%) with EoE] were included. Fourteen patients were diagnosed with predominantly antibody deficiencies [unclassified antibody deficiency (n = 11), selective Ig A deficiency (n = 2), transient hypogammaglobulinemia of infancy (n = 1)]. Eighteen patients had abnormal Ig levels. Comparisons across the groups revealed no statistically significant differences in demographic, endoscopic, and pathologic features. Only topical swallowed budesonide unresponsiveness was significantly higher among cases with PID (p = .004). CONCLUSION:PIDs may not be rare among patients with EGIDs. JMF's warning signs alone may be insufficient to identify affected patients in this population; therefore, additionally, basic immunologic tests as first step and advanced evaluation in suspected cases may be appropriate. Neither the key diagnostic endoscopic and pathological features nor the presence of strictures reliably distinguish PIDs.

Transaldolase deficiency is a rare autosomal recessive disease caused by biallelic mutations in the TALDO1 gene. This disorder is characterized by multisystem involvement, including liver disease. Here, we present 2 siblings with transaldolase deficiency and a novel homozygous mutation in the TALDO1 gene. The index case was presented with hepatosplenomegaly and elevated transaminases in infancy. The 11-year-old sibling, who was diagnosed through family screening, also had chronic liver disease with fibrotic changes, despite initially normal liver function tests. Both patients exhibited additional findings, including dysmorphic facial features, hypergonadotropic hypogonadism, proteinuria, and skeletal anomalies such as scoliosis. Liver biopsies revealed periportal and bridging fibrosis without necroinflammatory activity or malignant transformation. In the second case, a 6 mm T2-hyperintense nodular lesion detected on MRI prompted close monitoring for hepatocellular carcinoma. This report highlights the phenotypic variability of TALDO deficiency, the potential for progressive liver damage in asymptomatic patients, and the importance of liver biopsy and imaging in surveillance. Early diagnosis using genetic testing and family screening facilitates the timely management of complications. Given the multisystem nature of the disorder, multidisciplinary follow-up is essential.

OBJECTIVE:The Pediatric Quality of Life Inventory™ (PedsQL™) Eosinophilic Esophagitis (EoE) Module is a specialized instrument designed to assess how EoE affects the quality of life in pediatric patients. This study assesses the Turkish cross-cultural adaptation of the 8-12-year-old version. MATERIALS AND METHODS:As part of the cross-cultural adaptation of the Turkish PedsQL™ EoE Module for children aged 8-12 (Tr-PedsQL™ EoE), linguistic validation, content validity (CnV), construct validity (CsV), and reliability testing were conducted. The CsV was evaluated by correlating Tr-PEESS v2.0 Frequency and Total scores with the Tr-PedsQL™ EoE Symptoms Total, Symptoms I, and Symptoms II scores. Reliability was assessed using Cronbach's alpha for internal consistency and intraclass correlation coefficients (ICC) for test-retest stability. RESULTS:The study included 25 children diagnosed with EoE and their parents (n=25). All items showed CnV indexes above 0.8. Good to excellent correlations (r=-0.459 to -0.948) between Tr-PEESS v2.0 (Frequency, Total) and Tr-PedsQL™ EoE Module (Total, Symptoms Total, Symptoms I-II) scores indicate CV. The 8-12-year-old version showed good to excellent internal consistency, with Cronbach's α values of 0.712-0.918 for children and 0.703-0.901 for parents. All ICCs indicated good to excellent test-retest reliability (0.777-0.905 for children, 0.801-0.941 for parents). CONCLUSION:Validity and reliability analyses confirmed that the Tr-PedsQL™ EoE Module is a psychometrically sound instrument for use in both children and their parents. It completed the cross-cultural adaptation process and is appropriate for implementation in both clinical practice and research contexts in Türkiye.

Shunt thrombosis is an important complication after proximal splenorenal shunt (PSRS) in children with extrahepatic portal vein thrombosis (EHPVT). We evaluated whether preoperative vascular parameters are associated with postoperative PSRS patency. Of 139 non-cirrhotic patients who underwent PSRS for EHPVT, 86 with adequate preoperative imaging and follow-up were included in this retrospective single-center study. The aortomesenteric angle, splenic and left renal vein diameter were measured on preoperative CT, and spontaneous splenorenal shunt presence was recorded. Shunt patency was assessed from postoperative imaging. Correlation, Firth penalized logistic regression, and ROC analyses were performed. Mean age was 114 ± 50 months; mean follow-up 81 ± 51 months. Patency was preserved in 69 patients (80.2

OBJECTIVES:Our aim was to identify the potential predictors of liver transplant in patients with autoimmune hepatitis based on data at initial presentation. MATERIALS AND METHODS:We retrospectively evaluated records of children diagnosed with autoimmune hepatitis between 2000 and 2025. We excluded 21 patients with de novo autoimmune hepatitis (n =5) and incomplete data (n = 16). We compared demographic, clinical, biochemical, and histopathological data in 2 study groups: transplanted (n = 9) and not transplanted (n = 81). RESULTS:Of 90 included patients, 63 (70% ) were female. Median age at diagnosis was 115 months (range, 8-215 months). Among included patients, 70 had type 1 autoimmune hepatitis, 13 had type 2 autoimmune hepatitis, and 7 had seronegative autoimmune hepatitis. Nine patients underwent liver transplant (5 with type 1 autoimmune hepatitis, 4 with type 2 autoimmune hepatitis). Rate of transplant was significantly higher in patients with type 2 autoimmune hepatitis (P = . ⁰³ ). Compared with the nontransplant group, patients in the transplant group were more likely to have type 2 autoimmune hepatitis; higher gamma-glutamyl transferase, bilirubin, international normalized ratio, and immunoglobulin levels; and lower albumin and platelet levels. In addition, this group was more likely to have relapsing disease and moderate-to-severe fibrosis at diagnosis. Multivariate analysis showed that moderate-to-severe fibrosis and total bilirubin level were significant predictors of liver transplant (odds ratio: 21.84, 95%CI, 1.34-355.25; P = . ⁰³⁰ and odds ratio: 1.22, 95% CI, 1.02-1.47; P = . ⁰²⁹, respectively). Autoimmune hepatitis type 2 was not found to be associated with outcome (P = . ⁸³⁹). ). CONCLUSIONS:Systematic risk assessment at diagnosis is important to identify high-risk patients who may benefit from close monitoring, early escalation of therapy, and timely referral for transplant evaluation. Tailoring management strategies based on these predictive factors may improve long-term outcomes in pediatric autoimmune hepatitis.

This review by a multidisciplinary panel of pediatric gastroenterology, pediatric neurology, and pediatric oncology experts aimed to address the standard of care in pediatric malnutrition in a context of ABCDs: A- Anthropometric assessment, B- Etiology-based evaluation, C- Nutritional Intervention & Treatment and D- Individualization & Restoration. The proposed standard of care in pediatric malnutrition involves routine assessment of growth and development at every pediatric visit, timely diagnosis and etiological classification of malnutrition, selection of optimal nutritional product meeting specific energy and protein requirements (such as energy- and protein-rich formulas with proteins constituting at least 10% of total calories), and implementation of appropriate nutritional intervention strategies tailored to the type and severity of malnutrition. These strategies may include stabilization, catch-up growth, nutritional rehabilitation, and restoration treatment using peptide-based enteral formulas, depending on the clinical context.

Introduction: Celiac disease (CD)-related antibody positivity in children with type 1 diabetes (T1D) may fluctuate and become negative spontaneously. There are uncertainties about the optimal tissue transglutaminase IgA (tTG-IgA) titre and timing of endoscopy in the diagnosis of CD, and this study aimed to contribute to the debate on the tTG-IgA threshold titre for endoscopy decisions in children with T1D. Methods: The data of 991 children with T1D who had undergone serologic evaluation for CD were analysed retrospectively. The tTG-IgA positivity rate and the upper limit of normal (ULN) tTG-IgA positivity were assessed. Participants were grouped according to the frequency, course, and test results of tTG-IgA tests. Those with and without histopathologic diagnosis of CD by endoscopic biopsy were compared in terms of tTG-IgA screening time and tTG-IgA predictive values. Results: In 10.2% (n:101) of all cases, tTG-IgA antibody was positive and endoscopic biopsy was performed in 68.3% (n:69) of these cases. Of all cases, 4.3% (n:43) were diagnosed with CD by endoscopic biopsy. A tTG-IgA titre of 7xULN and above was found to be the best predictive value for the diagnosis of CD with 79.1% sensitivity, 80.8% specificity 87.2% positive predictive value, and 70% negative predictive value. Conclusions: Approximately 10% of antibody positive cases showed fluctuating and low-titre positivity, and no CD was detected by endoscopic biopsy in the group with fluctuating antibody course. The results of our study suggest that endoscopy in children with tTG-IgA levels 7xULN or above may prevent both false-positive results and missed cases.

INTRODUCTION:Neonatal cholestasis is a group of disorders characterised by conjugated hyperbilirubinemia in the newborns and young infants. Advances in genetic testing have facilitated the identification of specific aetiology. This study examines the genetic and clinical profiles of neonates with cholestasis, focusing on genotype-phenotype correlations and diagnostic outcomes. METHODS:A retrospective review of children with neonatal cholestasis treated between 1997 and 2024 was conducted. Extrahepatic causes were excluded, and genetic testing, including a targeted cholestasis panel and whole exome sequencing (WES), was employed. Clinical and biochemical data, including gamma-glutamyl transferase (GGT) levels, were collected. RESULTS:Genetic disorders were identified in 28.0% of 378 cases, including mutations in ATP8B1, ABCB11, ABCB4, DCDC2, DGUOK, KIF12, USP53, and genes related to bile acid synthesis (HSD3B7, PEX1). GGT levels played a significant role in diagnosis: patients with low or normal GGT were frequently diagnosed with progressive familial intrahepatic cholestasis (PFIC)1 and 2, or bile acid synthesis defects, while high GGT levels were associated with PFIC3, alpha-1 antitrypsin deficiency, and cystic fibrosis. Consanguinity was noted in 56.0% of genetically diagnosed cases. After 2010, 35.5% of patients received a genetic diagnosis, compared to 18.2% before 2010. CONCLUSION:Genetic diseases are a major cause of neonatal cholestasis, and GGT levels serve as a useful diagnostic tool in differentiating subtypes. The increasing availability of genetic testing has improved early diagnosis and personalised management. Expanded genetic testing in clinical practice is critical for timely and accurate diagnosis of these rare disorders.

Introduction: Cobalamin J disease (CblJ) is an ultrarare autosomal recessive disorder of intracellular cobalamin metabolism associated with combined methylmalonic academia and homocystinuria (MAHCJ; 614857). Case Presentation: A new patient with MAHCJ, representing the eighth documented instance, is reported here. A novel homozygous missense variant c.1591C>T (p.Arg531Trp) in exon 17 of ABCD4 (NM_005050.4) was identified. The patient, a 15-year-old male of Azerbaijani descent, presented with severe abdominal pain beginning at the age of 1 year. These episodic pain attacks were accompanied by hypotonia, pallor, nausea, and vomiting. Initial evaluations were inconclusive. At the age of 8 years, the patient developed megaloblastic anemia due to vitamin B12 deficiency, leading to the initiation of replacement therapy. The pain attacks ceased during treatment but recurred whenever vitamin B12 levels dropped after discontinuation. The patient exhibited no dysmorphology, skin hyperpigmentation, somatic abnormalities, seizures, or neurodevelopmental delays and remains in remission with ongoing vitamin B12 treatment. Discussion: This patient is the oldest diagnosed with MAHCJ and has the longest documented clinical course. This report expands the known clinical and molecular spectrum of this rare disease. We recommend remembering cobalamin defects in the differential diagnosis of unresolved abdominal pain attacks.

INTRODUCTION:Portal vein thrombosis (PVT) has been increasingly diagnosed in pediatric patients owing to the widespread use of non-invasive radiological techniques. Although the prevalence of PVT in adults with cirrhosis and intrahepatic non-cirrhotic portal hypertension ranges from 0.6 to 26 % and 13 to 46 %, respectively, no available data exist in the pediatric population. The prevalence of PVT in children with cirrhotic and intrahepatic non-cirrhotic portal hypertension was evaluated in this study. METHODS:This retrospective study included children with cirrhosis and intrahepatic non-cirrhotic portal hypertension, which consisted of congenital hepatic fibrosis (CHF) and idiopathic noncirrhotic portal hypertension (INCPH). Patients with extrahepatic portal venous obstruction were excluded from the study. The presence of PVT was evaluated using abdominal Doppler ultrasonography and/or CT. Etiological, clinical, and laboratory findings were compared between the groups. RESULTS:One hundred and forty-two patients with cirrhosis (mean admission age: 64.6 months ± 66.4, mean follow-up duration: 46.8 months ± 45.6) and 41 patients with non-cirrhotic patients (CHF=16, INCPH = 25, mean admission age: 126 months ± 64.2) were enrolled in this study. The prevalence of PVT was not significantly different between cirrhotic (8.5 %) and non-cirrhotic (9.7 %) patients. The incidence of PVT was significantly higher in patients with biliary atresia than in those with other etiologies in the cirrhotic group (p = 0.022). The frequency of PVT was higher in patients who had Child-Pugh score ≥7 in the cirrhotic group, but the difference was not statistically significant (p = 0066). The PVT group required more liver transplantations than the non-PVT group (p = 0.038). CONCLUSION:The prevalence of PVT was similar in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension in our cohort, which is compatible with adult studies. Biliary atresia is found to be an important risk factor for PVT in our pediatric population. It might be associated with rapid progression of the disease, ascending cholangitis, and embryological abnormalities. These patients should be routinely evaluated to identify portal vein complications and early warning signs during follow-up.

Glycogen storage disease type 1 (GSD1), which is categorized into GSD1a and GSD1b, is caused by disease-causing genetic variants in G6PC or SLC37A4 genes, respectively. The aim of this study was to present clinical characteristics, novel phenotypic and molecular features as well as long-term complications of the largest cohort of patients in Turkey and one of the largest cohorts in the world. The demographic, clinical, and molecular data of GSD1a or 1b patients who were followed up between 2000 and 2024 were collected retrospectively from patients’ medical records. A total of 39 GSD1a patients were enrolled, and four different variants in the G6PC gene, c.247C > T (p.R83C), c.809G > T (p.G270V), c.562G > C (p.G188R), c.480G > A (p.W160*) were revealed. The most common variant among Turkish GSD1a patients was the c.247C > T (p.R83C) variant with an allele frequency of about 90

Objective: Although a limited number of studies have assessed the impact of the Coronavirus disease 2019 (COVID-19) pandemic on adults with eosinophilic esophagitis (EoE), there are no data on children. This study aimed to assess the impact of the COVID-19 pandemic on children with EoE, including long-term follow-up, treatment adherence, COVID-19 infection, and vaccination status. Materials and Methods: Treatment adherence, symptoms, and endoscopic-pathological findings were compared at the beginning and the end of the first and second years of the pandemic. The COVID-19 infection and vaccination status were also assessed. Results: The study included 66 children (median age 13.2 years) with EoE. Both treatment adherence and endoscopic follow-up decreased significantly during the pandemic compared to the beginning (P < .001 and P < .001, respectively). No strictures were observed. Twentytwo patients underwent endoscopy both before and during the pandemic, showing increased total eosinophilic esophagitis endoscopic reference score (EREFS) and peak eosinophil counts (P = .045 and P = .08, respectively). Among children aged 12 and older, 66% were vaccinated against COVID-19. Infection with COVID-19 was detected in 24 children (36.3%), with asymptomatic or mild symptoms in 95.8% of cases. Conclusion: No strictures developed during the first 2 years of the pandemic in children with EoE. However, increased tissue eosinophilia and EREFS scores suggest a possible risk of fibrostenosis if treatment adherence remains low. Eosinophilic esophagitis does not seem to pose an increased risk for COVID-19 infection in children.

BACKGROUND:Celiac disease is a chronic autoimmune disorder of the small intestine for which the sole effective treatment is a lifelong gluten-free diet (GFD). Gluten immunogenic peptides (GIP) serve as biomarkers for recent gluten intake and can be utilized to assess gluten consumption levels. The aim of this study was to compare levels of fecal GIP with levels of tissue transglutaminase IgA (tTG-IgA), as well as dietary compliance, during follow-up. METHODS:This prospective, non-randomized, single-center study took place between August 2019 and August 2021 at Pediatric Gastroenterology Clinic at Gazi University Hospital in Ankara with the participation of 24 newly diagnosed celiac patients between 2 and 18 years (17 females, 7 males). Participants received GFD training from an expert dietitian, while any dietary transgressions were determined and assessed at 3 and 6 months. Levels of fecal GIP and blood tTG-IgA were analyzed at diagnosis, and again in follow-ups, using a sandwich enzyme-linked immunosorbent assay kit. Compliance with GFD was evaluated using a structured approach in terms of levels of GIP, tissue transglutaminase (tTG), Biagi score, as well as 24-hour food consumption records kept for 3 days (2 weekdays and 1 weekend). RESULTS:The mean age of participants was 8.3 ± 4.70 years. 23 patients (95.8%) initially had detectable GIP levels, while serum tTG-IgA was determined to be positive for all. After starting the GFD, GIP detection rates were measured as being 37.5% at 3 months, and 25% at 6 months, while tTG-IgA positivity rates were determined as being 41.7% and 37.5% respectively. While no significant correlation was found between GIP and tTG-IgA positivity. GIP detection at 3 months was moderately associated with dietitian assessments and Biagi scores (P < 0.05) but with no association at 6 months. CONCLUSIONS:Expert dietitian training with regular monitoring increases celiac disease patients complying with GFD. Repeated fecal GIP analysis demonstrated any dietary nonadherence or unintentional gluten exposure that had occurred during the previous 2 to 7 days. It is suggested that a combination of these two tools can improve assessment of dietary compliance in celiac patients.

Protein-losing enteropathy (PLE) is a rare condition characterized by clinical findings such as edema, ascites, pleural effusion, and diarrhea due to excessive protein loss from the gastrointestinal system. Although systemic lupus erythematosus (SLE) is rare in childhood, PLE can be the first presenting feature; this condition is referred to as lupus-associated protein-losing enteropathy (LUPLE). Protein-losing enteropathy (PLE) is an uncommon condition resulting from excessive protein loss in the gastrointestinal system. Our case shows that PLE can be the initial presentation of SLE, which is a rare manifestation in childhood. PLE, a rare complication of lupus, tends to be more severe in children, and the diagnostic process can be challenging. This case report presents a 7-year-old girl who presented with abdominal distension, generalized edema, chronic diarrhea, and weakness. Despite treatment, the recurrence of symptoms and the addition of new joint findings led to further investigations, which revealed positive anti-dsDNA and low complement levels, resulting in a diagnosis of systemic lupus erythematosus. The patient's clinical condition improved with steroid, azathioprine, and hydroxychloroquine treatments. This case highlights the importance of considering SLE in the differential diagnosis of PLE and underscores the significance of recognizing the rare presentations of childhood lupus.

INTRODUCTION:Hereditary cholestatic liver diseases are a group of disorders caused by different gene mutations encoding proteins involved in bile production, transport, or secretion. New genetic disorders have been identified in cholestatic patients through an increased understanding of these proteins, evolving genetic technology and more widespread genetic testing. Recently, mutations in the Lipolysis-Stimulated Lipoprotein Receptor (LSR) gene have been identified as a novel cause of infantile intrahepatic cholestasis. To date, only two cases have been reported in the literature. Here, we present two children with GGT normal cholestasis with LSR-gene variants. METHODS:Two pediatric patients with GGT-normal cholestasis underwent genetic analysis, including whole-exome sequencing. Clinical, laboratory, and histopathological findings were reviewed, and genetic variants were evaluated. RESULTS:Both patients exhibited progressive cholestasis with normal GGT levels, and genetic analysis identified homozygous missense variants in the LSR gene. These cases' clinical presentation and disease course shared similarities with previously reported LSR-related cholestasis, suggesting a common pathogenic mechanism. CONCLUSIONS:These findings further support the LSR gene as a novel cause of GGT normal cholestasis. Identifying additional cases provides crucial insights into the genotype-phenotype correlation, aiding in the early diagnosis and management of affected patients. Expanding the number of reported cases will contribute to a better understanding of disease progression and potential therapeutic strategies.

Celiac Disease (CD)-related antibody positivity in children with Type 1 Diabetes (T1D) may fluctuate and become negative spontaneously. There are uncertainties about the optimal tTG-IgA titre and timing of endoscopy in the diagnosis of CD, and this study aimed to contribute to the debate on the tTGA-IgA threshold titre for endoscopy decisions in children with T1D. The data of 991 children with T1D who had undergone serologic evaluation for CD were analysed retrospectively. The tTG-IgA positivity rate and the upper limit of normal (ULN) tTG-IgA positivity were assessed. Participants were grouped according to the frequency, course and test results of tTG-IgA tests. Those with and without histopathologic diagnosis of CD by endoscopic biopsy were compared in terms of tTG-IgA screening time and tTG-IgA predictive values. In 10.2% (n:101) of all cases, tTG-IgA antibody was positive and endoscopic biopsy was performed in 68.3% (n:69) of these cases. Of all cases, 4.3% (n:43) were diagnosed with CD by endoscopic biopsy. A tTG-IgA titre of 7xULN and above was found to be the best predictive value for the diagnosis of CD with 79.1% sensitivity, 80.8% specificity 87.2% positive predictive value and 70% negative predictive value. Approximately 10% of antibody positive cases showed fluctuating and low titre positivity, and no CD was detected by endoscopic biopsy in the group with fluctuating antibody course. The results of our study suggest that endoscopy in children with tTG-IgA levels 7xULN or above may prevent both false positive results and missed cases.

ObjectiveUse of peptide-based formulas supplemented with medium chain triglycerides (MCTs) is considered a beneficial strategy to decrease the tube-feeding associated gastrointestinal tolerance. In children with cerebral palsy (CP), overall effects of enteral tube feeding as well as the utility of peptide-based specialized enteral formulas in those with gastrointestinal intolerance have not been extensively studied. This study aimed to evaluate the utility of enteral tube feeding via specialized peptide-based formula containing MCTs in children with CP in terms of gastrointestinal intolerance, anthropometrics, defecation characteristics and parental satisfaction with enteral formula.MethodsChildren with CP who received enteral tube feeding via specialized peptide-based formula containing MCTs were included in this prospective observational study. Anthropometrics (z scores for weight for age [WFA], weight for height [WFH], triceps skinfold thickness [TSFT] and mid-upper arm circumference [MUAC]), gastrointestinal intolerance symptoms, defecation frequency and stool patterns and formula satisfaction were recorded at baseline and during 6-month follow up.ResultsA total of 96 children with CP (mean ± SD age: 5.6 ± 3.2 years, 56.3% were boys) were included. Significant improvements were noted in MUAC, TSFT and WFH z scores at the 6th month visit. The rate of “severe symptoms” and the likelihood of Type-1/Type-2 (constipation) stool pattern were significantly decreased. Majority of parents were satisfied with the study formula.ConclusionOur findings revealed favorable efficacy and safety of using a specialized peptide-based formula containing MCT in provision of enteral tube feeding among children with CP in terms of improved anthropometrics, amelioration of gastrointestinal intolerance symptoms and normalization of bowel movements along with a high parental satisfaction.

IntroductionCeliac disease (CD) is a chronic autoimmune disorder that requires strict adherence to a gluten-free diet (GFD) initiated after diagnosis. This limited diet may lead to nutritional deficiencies. The aim of this study was to evaluate nutritional intake and dietary adequacy of children with CD having good adherence to a GFD compared with their healthy peers and to assess the contribution of commercial gluten-free products on the daily energy and macronutrient intakes.MethodsThis cross-sectional case-control study included children with CD (age range, 2–18 years) and age- and sex-matched healthy controls. Demographic characteristics, anthropometric measurements and food consumption (3-day food record) were recorded. The groups were compared for dietary compositions, dietary adequacy, and anthropometric parameters.ResultsThe study compared 51 patients with 54 controls. The patients had significantly lower height-for-age Z-scores and body mass index-for-age Z-scores (p < 0.05). The dietary daily energy, protein, fat and fiber intakes were significantly lower in the patients than in the healthy controls (p < 0.05). The mean nutrient adequacy ratio (NAR) for protein, thiamine, calcium, magnesium, iron, zinc and fiber was significantly lower in the patients for both sexes (p < 0.05 for all) and the mean NAR for vitamin A and folate was lower in the patients in females (p < 0.05 for all). The mean nutrient adequacy ratio (MAR) of protein, thiamine, calcium, magnesium, iron, zinc and fiber was lower in the patients than in the controls (p < 0.05 for all).ConclusionA comprehensive dietary assessment for patients with CD may enhance their adaptation to healthy nutrition and facilitate their optimal growth.