Journal of Pediatric Gastroenterology and Nutrition最新文献

Objective: Data on the relationship between body composition and strength in children with intestinal failure (IF) is lacking. The objectives were to (1) assess strength in children with IF compared to published norms; (2) examine the relationship between body composition and strength.

Methods: Children aged 4-18 years with IF, excluding those with significant developmental delay or mobility challenges, were included. Functional strength was assessed using the Bruininks-Oseretsky Test of Motor Proficiency-2 (BOT-2) and muscle strength was measured with a dynamometer for handgrip strength (HGS) and knee flexion/extension. Body composition was assessed using dual-energy X-ray absorptiometry. Two-sample t-test compared strength in IF patients to published norms, and the relationship between body composition and strength was analyzed using linear regression.

Results: Thirty-one children (71% male, mean age 9.9 years) were included. Twelve (39%) received parenteral nutrition (PN). Children with IF had significantly lower HGS (p = 0.004), knee flexion (p = 0.002), and extension (p < 0.001) compared to published norms. Specifically, 80.6% scored below the mean for HGS, 71.0% for knee flexion, and 74.2% for extension. BOT-2 revealed 26.9% had scores less than -1 SD. No significant differences in strength were found between those on enteral nutrition or PN. A significant positive relationship between functional strength and increased fat-free mass, and decreased fat mass was found (r² = 0.36, p = 0.002), with male sex strengthening the relationship (r² = 0.62, p < 0.001).

Conclusion: Children with IF, both on enteral nutrition and PN, are weaker than healthy literature controls. Decreased strength was associated with lower fat-free mass. This relationship was stronger in males than in females, highlighting the need to further investigate the mechanisms behind body composition and strength in this population.

Trial registration: ClinicalTrials.gov identifier: NCT04610918.

Objective: Extensively hydrolyzed formulas (eHF) are the first-line approach for cow's milk protein allergy (CMPA). This study evaluated the effects of eHF with synbiotic eHF (SeHF) on the general condition and symptoms of presumed CMPA infants during the initial 4-week exclusion diet period.

Methods: This observational prospective study (NCT04893330) recruited 114 patients (intention-to-treat [ITT] population; aged ≤8 months) with presumed CMPA. Patients were followed up for 28 days (D28) (per-protocol [PP], n = 80). Changes in general condition and presumed allergic symptoms were assessed using a 4-level Likert scale. Secondary endpoints included the time to symptom improvement, formula acceptability and consumption, and the parent's quality of life (QoL).

Results: The presumed allergic symptoms had improved/resolved for 86.3% of the PP population in 7-10 days on average. 86.8% of ITT cases had digestive symptoms at baseline and those reduced/disappeared in 93.2% of the PP cases at D28. The majority of parents agreed that the formula was suitable for the baby (78.8%). The QoL significantly improved from 60 ± 28 to 42 ± 24 (p < 0.001).

Conclusion: This study reflects the real-world management of presumed CMPA in our private practices using SeHF, which effectively improved CMPA symptoms. It also highlights the importance of waiting at least 2 weeks before reconsidering CMPA diagnosis.

Objectives: To describe the clinical and laboratory characteristics and outcomes of pediatric hepatopulmonary syndrome (HPS) secondary to portal hypertension (PH) in Brazil.

Methods: Fifty-four pediatric patients diagnosed with PH and HPS according to the European Respiratory Society criteria were included in this multicenter retrospective study. Clinical and laboratory data at the time of diagnosing the underlying disease, 12 months before diagnosing HPS, at the time of diagnosing HPS, and at the time of the last consultation were collected from the medical records.

Results: PH was cirrhotic in 87% of patients. Biliary atresia was the predominant etiology (35.2%). The median age at the time of diagnosis was 7.8 years (interquartile range [IQR]: 4.7-10.8). Partial arterial oxygen pressure (PaO₂) of asymptomatic patients (44.4%) was higher than that of symptomatic patients (p < 0.0001). Peripheral oxygen saturation (SpO₂) was ≥96% in eight patients, seven of whom had PaO₂ of <70 mmHg. The hemoglobin levels were elevated at the time of diagnosing HPS (p = 0.009), whereas the platelet count was decreased (p < 0.001). The survival rates of the 66.6% of patients who underwent liver transplantation (LT) at 2 months and 1 year post-LT were 90.3% and 84.6%, respectively. The severity of HPS did not affect the general post-LT survival (p = 0.787).

Conclusions: HPS remains asymptomatic during initial stages. SpO₂ may not be a reliable screening test in pediatric patients. Elevated hemoglobin levels in PH may be related to hypoxemia. The severity of hypoxemia at the time of diagnosis does not affect post-LT survival.

Objectives: To understand the clinical utility of small bowel video capsule endoscopy (VCE) based on indications, diagnostic yield, and management for pediatric patients suffering from disorders of the small bowel.

Methods: A single-center retrospective chart review was performed at Children's Health in Dallas, Texas between 2004 and 2022. Pediatric patients who underwent small bowel VCE were identified by Medtronic's Rapid PillCam Reader Software, and studies were organized by their primary indication. Chi-squared analysis was used to compare the rates of positive studies and studies leading to therapy or diagnostic plan changes by indication.

Results: Our analysis included 478 completed VCE studies of which 245 (51.3%) were positive and 169 (35.4%) led to therapy or diagnostic plan changes. The median age of patients was 12 years (range: 10 months to 18 years; interquartile range 8, 15) with 58.8% male patients. Compared to other indications, VCE studies performed for abdominal pain are less likely to be positive (p = 0.0003) and lead to fewer therapy and diagnostic plan changes (p = 0.0037).

Conclusions: VCE is useful in diverse clinical settings to diagnose and monitor small bowel pathologies. It leads to fewer changes in clinical management when the primary indication is abdominal pain.

Objectives: Bioelectric devices such as gastric electrical stimulation (GES) are used to treat severe upper gastrointestinal motility disorders in both younger and older patients. We compared clinical, physiologic, quality of life, and full-thickness gastric biopsy results between younger and older patients undergoing GES.

Methods: We analyzed 245 patients (45 males, 200 females; median age 42 [range 2-80 years] 47.6% with delayed solid gastric emptying and 52.4% nondelayed). All patients underwent temporary GES trials, followed by full-thickness gastric biopsies and physiologic assessments during permanent GES placement. Histologic evaluation used standardized cellular markers. Validated patient-reported outcomes were assessed at baseline and 1 year, with a 1-point improvement as clinical success.

Results: Younger patients (n = 37; 9 males, 28 females; median age 17 [range 2-25 years]); reported less severe anorexia (3.0 vs. 3.5, p = 0.005) and bloating (2.0 vs. 3.5, p < 0.001) at baseline, while nausea and vomiting severity were similar to 208 older adults. Biopsy analysis showed fewer CD3+ and CD8+ cells in younger patients (p = 0.032 and p = 0.002) and fibrosis was more common in older adults (41.2% vs. 16.1%, p = 0.008).

Conclusion: Younger patients with gastroparesis (Gp) syndromes have full-thickness gastric biopsy abnormalities and clinical measures that are similar, but not identical to older patients. These findings indicate the need for broader use of tissue analysis and other clinical measures in severely symptomatic patients with Gp symptoms undergoing the bio-electric therapy of gastric electrical stimulation to optimize perioperative counseling and postoperative symptom management.

Objectives: We aim to categorize the mechanical properties of the esophagus in children with eosinophilic esophagitis (EoE) in different disease stages and based on different management modalities, using endoscopic functional luminal imaging probe (EndoFLIP) which has been established to objectively evaluate mechanical properties of the esophagus in patients with EoE.

Methods: We performed a retrospective analysis of children who completed EndoFLIP during sedated endoscopy over 2 years. Patients with EoE were categorized into EoE remission or active EoE based on published guidelines. Control subjects were those with normal endoscopy, esophageal biopsies, and EndoFLIP parameters. EndoFLIP studies were analyzed for distensibility index (DI) and maximum diameter (MD) at the esophagogastric junction (EGJ), as well as contractile response (CR).

Results: We included 130 subjects, 60 controls, and 70 with EoE (34 [49%] had active EoE and 36 [51%] were in remission). DI and MD were significantly lower in active EoE compared to controls (p < 0.001). DI and MD were significantly lower in initial (baseline) EoE compared to active EoE on therapy (p < 0.05) and EoE remission (p < 0.001). There was no statistically significant difference in DI and MD between EoE subgroups on different therapies.

Conclusion: Assessment of esophageal mechanical properties using EndoFLIP provides valuable information on disease severity in pediatric EoE. In our cohort, histological remission achieved with therapies such as proton pump inhibitors, topical steroids, and dupilumab was associated with improved distensibility and diameter at the EGJ. However, a subset of patients continued to demonstrate abnormal EndoFLIP findings despite histologic improvement.

Objectives: Timely availability of a suitable allograft helps offset pediatric liver transplant (pLT) waitlist mortality. Candidate-donor blood type matching (CDBM) is an important attribute for allograft allocation. Current policy allows for blood type (ABO) compatible non-identical transplants for pLT candidates with high acuity. Additionally, centers may use ABO incompatible allografts for pLT candidates when required.

Methods: We conducted a retrospective analysis of the United Network for Organ Sharing (UNOS) database. We analyzed pediatric (<18 years) candidates listed for deceased donor liver-only transplant (DDLT) during the time-period 1/2008 to 12/2022. Specifically, we analyzed the impact of CDBM, including ABO non-identical LT on outcomes.

Results: 72.4% of the 8976 pLT candidates included in our analyses underwent DDLT. 19.9% of pLTs were ABO nonidentical, and 4.9% were ABO incompatible. Nonidentical pLT recipients had significantly higher listing urgency and lower waitlist times. Nonidentical pLTs led to a net loss of allografts for blood type O and net gain of allografts for all other blood types. Candidates with blood type O had significantly higher waitlist time (62.5 vs. 48 days) and lower proportion of candidates undergoing DDLT (70.2% vs. 74.7%), when compared to blood type A.

Conclusion: ABO nonidentical allografts result in timely transplant for pLT candidates with high acuity. Blood type O candidates are disadvantaged, while blood type A candidates are advantaged by ABO nonidentical allografts. The observations of our study, which differ from those of prior adult studies, could inform future policy changes pertaining to organ allocation based on CDBM.

Objectives: Approximately 9% of children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP) have pancreatogenic diabetes during childhood; lifetime risk approaches 50%. To date, data are limited on pathophysiology and biomarkers identifying those at highest risk. This pilot study investigated glycemic physiology in children with ARP or CP.

Methods: Children (5-21 years) with an established diagnosis of CP or ARP, and participants of INSPPIRE-2 (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) were enrolled. Mixed meal tolerance testing (MMTT) measured glucose, insulin, C-peptide, glucagon, glucagon-like peptide-1 (GLP-1) and pancreatic polypeptide (PP) at -5, -1, 0, 30, 60, 90, 120 min before/after a Boost HP beverage. Other measures included hemoglobin A1c, continuous glucose monitoring (CGM, Dexcom Pro), HLA haplotype, and diabetes autoantibodies. Glycemic variability metrics were calculated using "cgmanalysis." Dysglycemia was defined by fasting glucose ≥100 mg/dL or HbA1c ≥ 5.7%.

Results: Twenty participants were enrolled (mean age 16.3 years; 65% female, 60% non-Hispanic white, 2 with pre-existing diabetes). Mean HbA1c was 5.7% (range 5.0-8.9); 7/20 had dysglycemia, 1 with previously unrecognized diabetes. Those with dysglycemia differed from normoglycemic participants by having greater insulin resistance, lower GLP-1, and trend toward lower insulin and C-peptide but higher PP on MMTT.

Conclusions: In this small study, 35% of children with pancreatitis had dysglycemia, which may be mechanistically related to insulin resistance. Other trends associated with dysglycemia included impaired insulin secretion, reduced GLP-1, and unexpectedly elevated PP.

Objectives: Celiac disease (CeD) is an autoimmune disease induced by ingestion of gluten. Patients may present with stereotypical gastrointestinal symptoms, including bloating and diarrhea, but can also present with nongastrointestinal symptoms such as migraines or rashes. The clinical presentation is well described in older pediatric and adult populations; however, the clinical picture is less clear for younger, preschool-aged pediatric patients. This retrospective chart review aims to describe the clinical presentation and serological values seen in patients diagnosed with CeD whose symptoms began before 5 years of age.

Methods: A retrospective review of a single pediatric CeD institution was conducted, and 82 children diagnosed with CeD with symptom onset before 5 years of age were identified.

Results: Children with symptom onset on or before 24 months of age were significantly more likely to have higher symptom burden (5 vs. 4, p = 0.0120). Most notable are vomiting (52.5% vs. 19%, p = 0.0024), mood changes (40% vs. 7.1%, p = 0.0005), poor weight gain (67.5% vs. 41.5%, p = 0.0164), and poor linear growth (35% vs. 11.9%, p = 0.0182). This subgroup was more likely to be hospitalized within 3 months of diagnosis (12.5% vs. 0, p = 0.0241). Children who had symptom onset after 24 months were more likely to have abdominal pain (30% vs. 59.5%, p = 0.0086) and sparser extraintestinal symptoms (p = 0.0224). No matter the age of symptom onset, there was no difference in the time interval to diagnosis.

Conclusions: This study adds to the literature evidence of the extraintestinal presentation of CeD that is seen in toddler-aged patients, as well as increased incidence of hospitalization at the time of diagnosis in this age group.

Objectives: Chronic intestinal failure (CIF) is a rare and complex disease, requiring home parenteral nutrition (HPN) to sustain growth and development. The impact on parents taking care for children with HPN remains underexplored. This qualitative study aimed to elucidate the experiences and challenges faced by parents in caring for HPN-dependent children.

Methods: Parents of children aged 0-18 years, diagnosed with CIF receiving HPN in the Emma Children's Hospital-Amsterdam UMC, were eligible. One-to-one semistructured interviews were conducted with parents at their home with open-ended questions regarding experiences around diagnosis, experiences with healthcare (both home care and at the hospital), mental health, holidays, social functioning and leisure time, work, relationship with the partner and family, fear for the future, and overall impact.

Results: Parents of 24 children were invited. Thirteen parents (four fathers and nine mothers) of 10 children (four females, median age 9 years) with HPN agreed to participate and were interviewed. Most important recurring themes among all parents, emerging from open-ended questions, were as follows: control in caregiving and reluctance to trust others to provide care over their child, social isolation due to limited time and flexibility, and importance of maintaining their own identity by self-fulfillment activities such as work, and physical or social activities.

Conclusions: This qualitative study underlines the profound impact of managing a child with HPN on daily life, relationships and well-being. There is need for tailored support and interventions to help families face the burden of having a child with HPN.