Extended total colonic aganglionosis and total intestinal aganglionosis: Challenging enemies.

AIM: Extended total colonic aganglionosis (ETCA) represents uncommon forms of Hirschsprung disease (HD), with aganglionosis extending into the proximal small bowel. ETCA management is challenging and associated with poor outcomes and high mortality. This study compares management and outcomes of ETCA to more common HD forms. METHODS: A retrospective cohort of HD patients (2012-2023) from two institutions. Three HD forms were compared: short-segment HD (SSHD, n = 19), long-segment HD or total colonic aganglionosis (LS/TCA, n = 9) and ETCA (n = 7). RESULTS: Normally innervated segments in ETCA patients ranged 0-70 cm. Median times to first surgery were; ETCA = 3 days versus TCA = 21 days (p = 0.017) and SSHD = 95 days (p < 0.001), respectively. Median number of surgeries were; ETCA = 4, versus TCA = 2 (p = 0.17) and SSHD = 1 (p = 0.002), respectively. All the patients underwent a definitive pull-through procedure, except four ETCA patients with a permanent jejunostomy and residual aganglionic segment of 57-130 cm. ETCA patients had 92% lower odds of enterocolitis (14%) compared to TCA patients (67%, p = 0.054), and comparable odds to SSHD patients (16%, p = 0.92). ETCA mortality was 14%. CONCLUSION: Extended total colonic aganglionosis patients require earlier and multiple interventions. Leaving an aganglionic segment may be advantageous, without increasing risk for enterocolitis. Tailored surgical treatment and rehabilitation programmes may prevent mortality and need for transplantation.

Acute pancreatitis in children with inflammatory bowel disease: Risk factors, clinical course, and prognosis.

OBJECTIVES: To characterize the clinical course of acute pancreatitis (AP) in pediatric inflammatory bowel disease (IBD) patients compared to children with AP without IBD and to identify risk factors associated with AP among IBD patients. METHODS: This retrospective, single-center study compared clinical characteristics of children (<19 years) with AP with and without concomitant IBD who were hospitalized 2005-2019. We also conducted a risk factor analysis of AP development in pediatric IBD. RESULTS: Sixty-eight (54% males) patients with 120 episodes of AP were admitted at a median age of 15.3 years. Thirteen patients (14 episodes) had a co-diagnosis of IBD, representing 4% of our IBD patient population. The AP-IBD patients presented with lower amylase levels compared to the non-IBD patients (160 [interquartile range, IQR: 83-231] vs. 418 [IQR: 176-874] U/L, p > 0.01), all had a mild pancreatitis, and none required invasive intervention. The presumed etiology for AP in all IBD patients was IBD-related: IBD flare-up in five, side effects of medications in two, and undetermined in seven. The only risk factor for AP development among IBD patients was IBD-associated arthritis (23% vs. 3% for IBD-non-AP, p = 0.04), while extracolonic Crohn's disease and induction therapy with nutrition were negative risk factors (15% vs. 51%, p = 0.05, and 8% vs. 44%, p = 0.04, respectively). Other parameters, including disease type and medications, were nonsignificant. CONCLUSION: The clinical course of AP in pediatric IBD patients is mild. Only IBD-associated arthritis emerged as a risk factor for the development of AP, while, unexpectedly, IBD medication did not.

Pediatricians' practices and knowledge of metabolic dysfunction-associated steatotic liver disease: An international survey.

OBJECTIVE: Metabolic dysfunction-associated steatotic liver disease (MASLD) is the leading cause of chronic liver disease in children. It is associated with significant intra- and extrahepatic comorbidity. Current guidelines lack consensus, potentially resulting in variation in screening, diagnosis and treatment practices, which may lead to underdiagnosing and/or insufficient treatment. The increasing prevalence of MASLD and associated long-term health risks demand adequate clinical management and consensus in guidelines. This study aims to evaluate the daily practices of pediatricians in screening, diagnosis and treatment of MASLD in children. METHODS: An online survey with 41 questions (single/multiple response options) was sent to pediatricians (with/without subspecialty) in Europe and Israel, via members of the ESPGHAN Fatty Liver Special Interest Group, between June and November 2022. The 454 pediatricians were included in this study. RESULTS: 51% of pediatricians indicated using any guideline for diagnosis and treatment of MASLD, with 68% reporting to follow recommendations only partially. 63% is of the opinion that guidelines need revision. The majority of pediatricians screen for MASLD with liver function tests and/or abdominal ultrasound. A large variety of treatment options is utilized, including lifestyle management, supplements and probiotics, with a notable 34% of pediatricians prescribing pharmacotherapy. When asked how often pediatricians request a liver biopsy in children with MASLD, 17% indicates they request a liver biopsy in more than 10% of cases. CONCLUSIONS: There is limited awareness and considerable variation in screening, diagnosis and treatment practices among European pediatricians, and a clear demand for new, uniform guidelines for MASLD in children.

Factors associated with age of presentation of pediatric feeding disorder.

AIM: Understanding the association between pediatric feeding disorder (PFD) and age of presentation is limited. We aimed to investigate factors associated with PFD among different age groups. METHODS: Retrospective analysis of medical records of infants and toddlers diagnosed with PFD, according to the World Health Organization-based definition. We compared children aged 1-12 months to those aged 13-72 months. RESULTS: Included were 253 children with PFD (median [interquartile range] age 16.4 [9.5-33] months at diagnosis, 56% boys). Significantly more children in the younger age group were girls (52.6% vs. 34.4%, respectively, p = .03) and preterm (25% vs. 14%, p = .03). They had more hospitalizations (34% vs. 23%, p = .03) and needed more prescription medications (36% vs. 17%, p < .01). Additionally, disturbances in oral intake were primarily linked to feeding skills dysfunction in the younger group and nutritional dysfunction in the older group (39.6% vs. 23.7% and 55% vs. 38%, respectively, p = .02). CONCLUSIONS: Infants under 1 year old with PFD represent a distinct patient group with unique characteristics and outcomes. The age of presentation plays a significant role in children with PFD, necessitating tailored treatment strategies.

Impact of Dietary Protein on the Management of Pediatric Short Bowel Syndrome.

Essential amino acids (AAs) play a key role in stimulating intestinal adaptation after massive small gut resection. The nutritional effect of dietary amino acids during intestinal regrowth has received considerable attention in recent years. This review explores the significance of dietary amino acids in the nutritional management of infants and children with intestinal failure and short bowel syndrome (SBS) as reported in the medical literature over the last three decades. A literature search was conducted using electronic databases. Breast milk emerged as the first-line enteral regimen recommended for infants with SBS. Hydrolyzed formulas (HFs) or amino acid formulas (AAFs) are recommended when breast milk is not available or if the infant cannot tolerate whole protein milk. The superiority of AAFs over HFs has never been demonstrated. Although glutamine (GLN) is the main fuel for enterocytes, GLN supplementation in infants with SBS showed no difference in the child's dependence upon parenteral nutrition (PN). Circulating citrulline is considered a major determinant of survival and nutritional prognosis of SBS patients. Early enteral nutrition and dietary supplementation of AAs following bowel resection in children are essential for the development of intestinal adaptation, thereby eliminating the need for PN.

Pediatric feeding disorders among children with parental history of feeding disorders: a distinct group of patients with unique characteristics.

To investigate factors associated with pediatric feeding disorders (PFD) among children of parents that reported to have had feeding disorders during their own childhood compared to children with PFD with no history of parental PFD. We retrospectively reviewed the medical records of children diagnosed with PFD according to the recent WHO-based definition. The demographic and clinical characteristics of children with PFD with a parental history of PFD were compared to those of children with a PFD with no history of parental PFD. Included were 231 children with PFD (median [interquartile range] age 10 months [5.5-29] at diagnosis, 58% boys) of whom 133 children had parents without PFD and 98 children had parents with PFD. Unexpectedly, children of parents without PFD had a higher rate of low birth weight (28% vs. 19%, respectively, p = 0.007), more delivery complications (10% vs. 2%, p = 0.006), more hospitalizations (33% vs. 17%, p = 0.004), more prescription medications (27% vs. 18%, p = 0.05), and a higher percent of gastrostomy tube use (6% vs. 0, p = 0.02). Moreover, more parents with PFD had academic background compared with parents without PFD (72% vs. 59%, p = 0.05). There were no significant group differences in sex, history of breastfeeding, parental marital status, or type of the child's feeding disorder.  Conclusion: PFD among children with a parental history of PFD comprise a distinct group of patients with unique characteristics and outcomes. Since parental feeding history may explain their child's PFD in highly differing ways, such information may help in devising a specific family-based and multidisciplinary treatment plan for those children. What is Known: • Pediatric feeding disorder (PFD) is relatively common and its prevalence is increasing. • Information on an association between parental PFD and their child's feeding disorder is limited. What is New: • PFD among children with a parental history of PFD comprise a distinct group of patients with various characteristics and outcomes. • The parents' feeding history during childhood may provide important clues to their child's PFD.

Glucagon-like peptide-1 analog therapy in rare genetic diseases: monogenic obesity, monogenic diabetes, and spinal muscular atrophy.

AIM: Implementing genetic analyses have unraveled rare alterations causing early-onset obesity and complications, in whom treatment is challenging. We aimed to report on the effects of adjuvant off-label therapy with liraglutide, glucagon-like peptide-1 analogue (GLP-1a), in rare genetic diagnoses. METHODS: Case scenarios and review of the literature. RESULTS: Case 1: Nine-year-old boy with early-onset severe obesity and nonalcoholic fatty liver disease (NAFLD) due to a homozygous mutation in the MC4R gene deteriorated under lifestyle change and metformin therapy [at 10.5 years: body mass index (BMI) 51.2kg/m2, 226% of the 95th percentile, fat percentage (FP) 65% and muscle-to-fat ratio (MFR) z-score of -2.41]. One year of liraglutide treatment halted progressive weight gain [BMI 50.3kg/m2, 212% of the 95th percentile, 63.7% FP and MFR z-score of -2.34], with biochemical improvement. Case 2: Twelve-year-old boy with obesity presented with diabetes and progressive NAFLD. Exome analysis revealed two heterozygous mutations compatible with monogenic diabetes (HNF1A) and familial hypercholesterolemia (LDLR). Lifestyle modifications resulted in clinical and laboratory improvement (BMI 87th percentile, 32.8% FP, MFR z-score of -1.63, HbA1c 5.5%) without the expected recovery in liver transaminases. Liraglutide treatment augmented the improvement in weight status (BMI 68th percentile, 22.6% FP, MFR z-score of -1.13) with normalization of liver transaminases. Case 3: Nineteen-year-old male with spinal muscular atrophy type 3 presented with sarcopenic obesity and comorbidities. Treatment strategy included dietary counseling and multiple drug therapies (metformin, anti-hypertensive and statins). Liraglutide therapy led to a gradual recovery of metabolic complications allowing tapering-down other medications. CONCLUSIONS: Considering the pleiotropic effects of GLP1-a beyond BMI reduction, this treatment modality may serve as a game changer in challenging cases.

Chronic Mucosal Inflammation in Pediatric Intestinal Failure Patients-A Unique Phenomenon.

INTRODUCTION/OBJECTIVES: As intestinal failure (IF) management improves and long-term survival rate increases, its physiological complications have become more apparent. The development of chronic intestinal inflammation resembling inflammatory bowel disease (IBD) in this population has been reported, but the literature describing it in detail is sparse. The present study was designed to characterize children with IF who developed chronic intestinal inflammation and identify the potential predisposing clinical factors. METHODS: This retrospective study was based on the electronic medical records of pediatric patients seen at the Cincinnati Children's Hospital Medical Center between January 2000 and July 2022. Demographic and medical history data were collected and compared between children with IF that developed chronic intestinal inflammation and children with IF that did not develop chronic intestinal inflammation. RESULTS: During the follow-up period, 23 children were diagnosed with chronic intestinal inflammation. Of these, 12 (52%) were males, with a median age of 4.5 (3-7) years at diagnosis. Nearly one-third of the patients had gastroschisis (31%), followed by necrotizing enterocolitis (26%), and malrotation and volvulus (21.7%). More children in the chronic intestinal inflammation group lacked an ileocecal valve (ICV) and adjoining distal ileum as compared to the short bowel syndrome (SBS)-IF control group (15 patients, 65% vs 8 patients, 33%). Moreover, more children in the chronic intestinal inflammation group had undergone a prior lengthening procedure than the SBS-IF control group (5 patients, 21.7% vs. 0, respectively). DISCUSSION: SBS patients are at risk of relatively early onset chronic intestinal inflammation. The absence of an ICV (and adjoin ileum) and prior lengthening procedures emerge as factors associated with the risk of IBD in these patients.

Laparoscopic Sleeve Gastrectomy Versus One Anastomosis Gastric Bypass in Adolescents With Obesity.

OBJECTIVE: Large studies comparing outcomes between laparoscopic sleeve gastrectomy (LSG) and one anastomosis gastric bypass (OAGB) are scarce and involve adult populations. The aim of the study was to compare perioperative, early postoperative, and 1-year postoperative outcomes of adolescents with obesity who underwent LSG or OAGB surgery. METHODS: The medical records of adolescents with obesity who underwent LSG or OAGB at the Tel Aviv Sourasky Medical Center from January 2017 to January 2021 were retrospectively reviewed. Data on their gastrointestinal (GI) symptoms and postoperative quality of life were obtained by a telephone interview. RESULTS: Included were 75 adolescents (median [interquartile range, IQR] age 17.3 [16-18] years) of whom 22 underwent OAGB and 53 underwent LSG. There were no significant preoperative group differences in age, sex, and body mass index score. A low rate of perioperative (5.7% vs 0) and postoperative complication (15.1% vs 10%) with no statistical differences between LSG and OAGB group, respectively, was noted. At 12 months, the percent excessive weight loss + IQR was 42.40% [30.00, 45.00] and 38.00% [33.550, 44.20] in the LSG and OAGB group, respectively ( P = NS). The results of the Pediatric Quality of Life Inventory Gastrointestinal Symptoms scale revealed significantly less food limitation and heartburn after OAGB compared to LSG (food limitation 71.63 vs 53.85 and heartburn 83.654 vs 61.6, P = 0.03 and P = 0.029, respectively). CONCLUSIONS: Both surgeries are effective and safe for weight loss in the adolescent population. OAGB was associated with significantly fewer GI symptoms compared to LSG.

Body composition in pediatric celiac disease and metabolic syndrome component risk-an observational study.

BACKGROUND: Celiac disease (CD) in children and adolescents has been linked with increased susceptibility for cardiometabolic disease in adulthood. We explored the interaction between body composition and metabolic syndrome (MetS) components in pediatric CD. METHODS: We conducted a retrospective observational study of patients with CD followed at our Pediatric Endocrine and Gastroenterology Units between 1/2018-1/2022. Data on sociodemographic, clinical, laboratory, and body composition parameters (bioelectrical impedance analysis, BIA) were collected. RESULTS: Forty-four patients with MetS components and 67 patients without them were enrolled. The cohort's mean age at BIA assessment was 11.5 ± 3.6 years. Individuals with MetS components were older (P = 0.045), had higher BMI z-scores (P < 0.001), higher total and truncal fat percentage levels (P < 0.001), lower muscle-to-fat ratio z-scores (P = 0.018), higher sarcopenic indices (P = 0.05), higher systolic blood pressure percentiles (P = 0.001), higher triglycerides levels (P = 0.009), and higher triglycerides/HDL-c ratios (P < 0.001) than those without MetS components. A sex- and age-adjusted model revealed that the diagnosis of MetS components was positively associated with fat percentage (odds ratio = 1.087, confidence interval [1.010-1.171], P = 0.027), but not with BMI z-scores (P = 0.138). CONCLUSIONS: We found that fat percentage but not weight status is associated with risk for MetS components in individuals with childhood-onset CD. Preventive interventions should target an improvement in body composition. IMPACT: The literature on cardiometabolic risk in pediatric patients with celiac disease (CD) is sparse. Our analysis revealed that at least one metabolic syndrome (MetS) component was present in two out of every five children and adolescents with CD. An increase in fat percentage but not in body mass index z-scores predicted the presence of MetS components in our cohort. These findings suggest that the weight status of children and adolescents with CD does not mirror their risk for MetS components. Body composition analysis should be considered as an integral part of the clinical evaluation in young patients with CD.

The Yield of Routine Tissue Sampling in Pediatric Gastrointestinal Endoscopy.

OBJECTIVES: Societies' guidelines suggest routine tissue sampling in all children undergoing esophagogastroduodenoscopy and ileocolonoscopy, even in the absence of visible endoscopy abnormalities. We aimed to determine the agreement between endoscopic and histopathological findings in pediatric endoscopy and to assess the yield of routine biopsies from all sites. METHODS: Since January 2019, our endoscopy institute protocol has included routine biopsies sampling from the esophagus, stomach, duodenum, ileum, and colon in all diagnostic procedures. Agreement between tests was done using the kappa coefficient ( κ ). The study included all endoscopies performed during 2019. RESULTS: In total, 541 diagnostic endoscopies were done during the study period with 434 (80%) esophagogastroduodenoscopy and 107 (20%) were ileocolonoscopy. Compared to histology, endoscopic findings performance were: esophagus-sensitivity 33%, specificity 98%; stomach-sensitivity 60%, specificity 89%; duodenum-sensitivity 50%, specificity 97%; duodenal bulb-sensitivity 47%, specificity 89%; terminal ileum-sensitivity 82%, specificity 100%; colon-sensitivity 84%, specificity 96%. Assessment of concordance between endoscopic and histopathologic findings reveals an overall low level of agreement in esophagogastroduodenoscopy ( κ of 0.39, 0.51, 0.53, and 0.24 for the esophagus, stomach, duodenal second part, and bulb, respectively), and good agreement in ileocolonoscopy ( κ of 0.88 and 0.81 for the ileum and colon, respectively). CONCLUSIONS: Endoscopy findings are highly specific for histologic pathology, whereas the absence of findings correlates poorly with histologic findings. Ileocolonoscopy shows better agreement than esophagogastroduodenoscopy. Our data support routine tissue sampling in pediatric endoscopy.

Anemia, Hepcidin, and Vitamin D in Healthy Preterm Infants: A Pilot Study.

OBJECTIVE: The etiology of anemia in premature neonates is multifactorial and may involve anemia of inflammation mediated by hepcidin. Hepcidin expression is suppressed by vitamin D. We aimed to investigate the interrelationship between hepcidin, anemia, and vitamin D status in preterm infants. STUDY DESIGN: Preterm infants aged 1 to 5 weeks were prospectively recruited at the neonatal intensive care unit of the Dana Dwek Children Hospital. Blood counts and serum levels of hepcidin, ferritin, iron, 25-hydroxyvitamin D [25(OH)D] and C-reactive protein (CRP) were measured and compared between anemic and nonanemic preterm infants. RESULTS: Forty-seven preterm infants (mean ± standard deviation gestational age at birth 32.8 ± 1.1 weeks, 66% males) were recruited. In total, 36% of the preterm infants were vitamin D deficient [25(OH)D < 20 ng/mL] and 15% were anemic. Hepcidin levels were significantly higher in anemic premature infants than in the nonanemic group (55.3 ± 23.9 ng/mL vs. 30.1 ± 16.3 ng/mL, respectively, p < 0.05). No differences were found in iron, ferritin, 25(OH)D, and CRP levels between anemic and nonanemic premature newborn infants. A positive correlation was found between hepcidin and ferritin (R 2 = 0.247, p = 0.02) and a negative correlation was found between 25(OH)D and CRP (R 2 = 0.1, p = 0.04). No significant correlations were found between 25(OH)D and hepcidin, iron, ferritin, or CRP. CONCLUSION: Anemia of prematurity was associated with high hepcidin serum levels. The exact mechanisms leading to anemia and the role of vitamin D warrant further investigation. KEY POINTS: · Hepcidin levels were significantly higher in anemic premature infants.. · A positive correlation was found between hepcidin and ferritin.. · Negative correlation was found between 25(OH)D and CRP..

Effect of Coffee and Tea Consumption on Adolescent Weight Control: An Interventional Pilot Study.

Background: Both catechin polyphenols and caffeine have been shown to have beneficial effects on weight control in the adult population. However, the influence of tea or coffee supplementation on body weight in adolescents has never been tested. The aim of the present study was to investigate the effect of tea and coffee consumption on body weight and body fat in adolescents with obesity. Methods: Randomized clinical trial comparing three weight-loss interventions composed of similar family-based counseling sessions on nutritional education with coffee (2 cups per day, total amount 160 mg caffeine), green tea (3 cups per day, total amount 252 mg catechin and 96 mg caffeine), or herbal tea (as placebo, 3 cups per day). Nutritional intake, BMI, and fat percentage, as measured by bioelectrical impedance, were compared between the groups at 3 and 6 months. Results: Forty-eight children were included in the final analysis: 18 in the coffee arm, 17 in the green tea arm, and 13 in the placebo arm. Nineteen (39.6%) children were males, with a median (interquartile range) age of 13 (11-14) years. There were no significant group differences in age, sex, and BMI (absolute number and percent of the 95th percentile) upon study entry. Comparison between the three interventions in total change in BMI from baseline revealed a significant advantage for coffee consumption compared with green tea and placebo (-9.2% change in BMI in the coffee group compared with -2.3% and 0.76% in the green tea and placebo group, respectively, p = 0.002). Conclusions: Dietary recommendations combined with coffee intake and, to a lesser extent, tea catechins may be associated with reduced weight and adiposity among adolescents. Clinical trial registration number: NCT05181176.

Factors Associated With Treatment Adherence to a Lifestyle Intervention Program for Children With Obesity: The Experience of a Large Tertiary Care Pediatric Hospital.

Early dropout and treatment adherence are main challenges in the treatment of children with obesity. The aim of this study was to identify factors associated with attrition and adherence to weight loss intervention program. We retrospectively reviewed the medical records of all the children who entered the program for pediatric weight loss over 5 years and retrieved demographic and clinical characteristics. Attrition and adherence were documented. Ninety-two of the 248 enrolled children (52% women, mean age 11.1 ± 3.9 years, mean body mass index 31.1 ± 7 kg/m2) dropped out (37%). Dropping out correlated with male sex, low parental education, and self-referral to the clinic (P < .05 for all). Adherence correlated with older age and the mother's healthy lifestyle (P < .05 for all). Sex, parental education, and referral source may predict treatment attrition. Early recognition of children at risk of attrition may help to facilitate better care of those children.

The role of gastrostomy feeding during intestinal rehabilitation for children with short bowel syndrome.

PURPOSE: Following extensive bowel resection, many children with short bowel syndrome (SBS) are routinely offered a placement of gastrostomy tube (G-tube) for feeding. This nutritional pathway is aimed to accommodate the gastric and small bowel motor disturbances related to SBS, and to promote weaning off parenteral nutrition (PN) to achieve enteral autonomy (EA). The aim of this study was to investigate the effect of gastrostomy feeding in outcomes of children with SBS. METHODS: A retrospective cohort of all SBS children managed at our multidisciplinary Intestinal Rehabilitation Center as part of an Intestinal Rehabilitation Program. SBS was defined as PN dependence for more than six weeks following extensive bowel resection. Patients treated with G-tube feeding were compared with patients without G-tube in terms of PN duration, reaching EA, physical development, and surgical parameters. RESULTS: A total of 36 SBS patients diagnosed between 2003 and 2022 were included. The most common etiologies included congenital intestinal atresia (31%) and necrotizing enterocolitis (25%). SBS-G-tube (group A) contained 20 children, and SBS (group B) contained 16 children. A total of 21 children reached EA (58%); ten from group A (50%), and 11 from group B (69%) (p > 0.05). Within EA patients, mean PN duration was 49 ± 44 months in group A, and 24 ± 33 months in group B (p > 0.05). Patients who reached EA had 22% longer residual small bowel when compared with PN-dependent patients (p = 0.003). However, the outcomes were adjusted for residual small and large bowel length and percentages, a residual ileocecal valve, and a colon in continuity with no differences between the groups. Two-thirds of children from group A reported G-tube related complications (mechanical, bleeding, or infections). We did not find differences in mean height and weight percentiles between the groups (p > 0.05). CONCLUSION: We did not find significant advantage of gastrostomy feeding in reaching EA. Because there are surgical and mechanical complications related to this procedure, further prospective studies are required to determine G-tube relevance for children with SBS.

Motility disorders in children with intestinal failure: a national tertiary referral center experience.

PURPOSE: Intestinal dysmotility (ID) problems are common in patients with pediatric-onset intestinal failure (IF) and short bowel syndrome (SBS), leading to significant morbidity and delays in the advancement of enteral nutrition (EN). We aimed to investigate the clinical features and complications of ID in children with IF and SBS. METHODS: Retrospective chart review of all children with IF and/or SBS who required parenteral nutrition (PN) > 6 weeks or small-intestinal resection ≥ 50%. Patients were divided into SBS and non-SBS groups. SBS group was divided into two subgroups: with and without ID. Patients with ID were identified (clinically, radiologically and functionally) and analyzed with regard to demographics, intestinal anatomy, complications and outcomes (short and long term). RESULTS: A total of 42 children with IF were treated in our institution during 2003-2022. In non-SBS group (n = 10), ID was the most common cause of IF (80%). SBS-group included 32 children; 18 children (56%) developed ID. The clinical profile of SBS-ID patients (vs SBS) was: female gender (56%), remaining small bowel length ≤ 55 cm, estimated residual small bowel ≤ 28% (p = 0.045) and absence of ICV (56%). Common symptoms of the SBS-ID group were: food intolerance (61%), abdominal distension (50%), vomiting (44%), malabsorption and severe constipation. Complications included FTT (67%) (p = 0.003), bacterial overgrowth with subsequent bloodstream infection (33%) (p = 0.75), and lactic acidosis (11%). Lengthening procedure (STEP) was performed in 11 SBS-ID patients (61%) (p = 0.002). In all patients, STEP operation "rescued" their dysfunctional intestine. Eight of these patients (73%) were weaned from TPN. Survival rate was 100%; however, one SBS-ID patient is a candidate for combined intestinal and liver transplantation. CONCLUSIONS: ID is the most common complication of SBS and is the most common cause of IF in non-SBS patients. ID has a high morbidity rate and various clinical manifestations. Successful treatment of these infants may be achieved with the use of tapering enteroplasty.

Demographic and clinical parameters are comparable across different types of pediatric feeding disorder.

Knowledge and understanding of risk mechanisms associated with pediatric feeding disorder (PFD) remain limited. We aimed to investigate factors associated with PFD and their relation to specific PFD types according to the recent consensus WHO-based definition. We retrospectively reviewed the medical records of children with PFD and retrieved their demographic and clinical characteristics. Healthy age- and sex-matched children served as controls. Included were 254 children with PFD [median (interquartile range) age 16.4 (9.5-33) months at diagnosis] and 108 children in the control group [median age 24.85 (14.5-28.5) months]. According to the WHO-based definition, disturbances in oral intake were predominantly related to nutritional dysfunction in 118 (46.6%), feeding skill dysfunction in 83 (32.3%), medical conditions in 42 (16.7%) and psychosocial dysfunction in 11 (4.4%). In multivariate analysis, children with PFD had a higher risk for lower socioeconomic background (P < 0.01) and low birth weight (26.8% compared to 7.4%, P < 0.001). Moreover, significantly fewer children in the PFD group were breastfed (75% versus 89%, P = 0.003). There were no significant differences in any of those variables between PFD types. In conclusion, low socioeconomic status, lack of breastfeeding, and low birth weight were significantly more frequent in children with PFD. PDF manifest as multiple dysfunctions, thus highlighting the need to offer these children and their families multidisciplinary care.

Carbohydrate content of human milk is affected by seasonal variations: a retrospective observational study.

OBJECTIVE: The influence of seasonal variation upon human milk macronutrient content has not been elucidated. This study aimed to compare the macronutrient content of HM produced by lactating mothers during the winter and the summer seasons. STUDY DESIGN: Macronutrient content of colostrum milk samples collected from lactating mothers of healthy term infants between March 2012 and February 2016 was measured by mid-infrared spectroscopy and compared. RESULT: The carbohydrate content of the colostrum was significantly higher in the summer season than in the winter season (6.2 ± 1.3 vs. 5.5 ± 1.4, p-value < 0.001). Protein, fat, and energy contents were similar in summer and winter in both groups (protein 2.7 ± 2.1 vs. 2.6 ± 2.2 g/100 ml, fat 2.6 ± 1.9 vs. 2.35 ± 1.9 g/100 ml, and energy 62 ± 19.1 vs. 60.5 ± 21 kcal/100 ml, respectively). CONCLUSION: The carbohydrate content in colostrum obtained from mothers of term infants was affected by seasonal variations.

Heart rate variability as a predictor of disease exacerbation in pediatric inflammatory bowel disease.

OBJECTIVES: Heart rate variability (HRV), a marker of the parasympathetic vagal activity, was reportedly significantly lower in patients with inflammatory bowel disease (IBD) compared to healthy controls. The aim of this study was to evaluate HRV as a predictor of clinical outcomes in pediatric IBD. METHODS: This was a prospective study. Children (12-18 years of age) with IBD were prospectively recruited. Each patient underwent two 10-min HRV measurements by means of a photoplethysmograph finger sensor. The square root of the mean squared differences of successive R-R pulse intervals (RMSSD), an indirect index of vagal activity, was calculated. Clinical data, including demographic variables, disease activity and course, medications, and laboratory results were collected during a follow-up of 12 months. The relation between RMSSD and clinical outcomes was examined, adjusting for confounders. RESULTS: A total of 34 children with IBD were included. Patients in clinical remission had a significantly higher RMSSD compared to patients with active disease (67.72 ± 27.81 versus 45.76 ± 22.04, respectively, P = 0.022). A multivariate analysis revealed that a higher RMSSD was a significant and independent predictor of lower risk of IBD exacerbation (odds ratio = 0.941, 95% confidence interval 0.887-0.998, p = 0.044). CONCLUSION: HRV correlates with IBD activity and may also serve as an independent predictor of disease exacerbation in pediatric IBD.

The Glycemic Response to Infant Formulas: A Randomized Clinical Trial.

BACKGROUND: Commercial infant formulas attempt to imitate human milk's unique composition. However, lactose-free and milk protein-free formulas are often chosen due to medical reasons or personal preferences. The aim of this study was to determine the glycemic and insulinemic indices of a variety of infant formulas. METHODS: We conducted a three-arm, randomized, double-blind, crossover study. Participants were 25-40-year-old healthy adults. Three commercial infant formulas (cow's milk protein-based ["standard"], soy protein-based, and lactose-free) were randomly given to each participant. Glycemic and insulinemic responses were determined and compared between the three formulas. RESULTS: Twenty subjects were enrolled (11 females/9 males, mean age 32.8 ± 2.9 years). No significant difference was found in the glycemic index between the three formulas (21.5, 29.1, and 21.5 for the standard, soy protein-based, and lactose-free formulas, respectively, p = 0.21). However, maximal glucose levels were significantly higher for the soy protein-based formula compared to both the standard and lactose-free formulas (111.5 compared to 101.8 and 105.8 mg/dL, respectively, p = 0.001). CONCLUSION: Cow's milk protein-based, soy protein-based, and lactose-free formulas have a similar glycemic index. However, soy protein-based formula produced a significantly higher increase in postprandial glucose levels. The implication and biological significance of these results have yet to be determined.