Pediatric Gastroenterology, Hepatology & Nutrition最新文献

{"title":"Clinician Experience with Using Hypoallergenic Formulas to Treat Infants with Suspected Cow's Milk Protein Allergy: A Secondary Analysis of a Prospective Survey Cohort.","authors":"Jesse Beacker,&nbsp;Jerry M Brown,&nbsp;Jared Florio,&nbsp;Jessica V Baran,&nbsp;Luke Lamos,&nbsp;Lea Oliveros,&nbsp;Jon A Vanderhoof,&nbsp;Panida Sriaroon,&nbsp;Michael J Wilsey","doi":"10.5223/pghn.2023.26.5.277","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.5.277","url":null,"abstract":"<p><strong>Purpose: </strong>Cow's milk protein allergy (CMPA) is a common condition in infants, but little is known about healthcare providers' clinical experience treating infants with CMPA. To address this gap, we analyzed prospectively collected data from healthcare providers (HCPs) who treated infants under six months old with suspected CMPA using hypoallergenic formulas. The study focused on a commercial extensively hydrolyzed formula containing Lactobacillus rhamnosus GG (ATCC53103) (eHF-LGG) or a commercial amino acid formula (AAF).</p><p><strong>Methods: </strong>In this secondary analysis of prospectively collected survey data, 52 HCPs treated 329 infants under six months old with suspected CMPA using hypoallergenic formulas. A series of two de-identified surveys per patient were collected by HCPs to assess short-term symptom relief in the patients and HCP's satisfaction with the management strategies. The initial survey was completed at the initiation of treatment of CMPA, and the second survey was completed at a follow-up visit.</p><p><strong>Results: </strong>The majority of HCPs (87%) in the study were general pediatricians, and most saw 2 to 10 CMPA patients weekly. Results showed that clinicians reported satisfaction with treatment in 95% of patients in the EHF cohort and 97% of patients in the AAF cohort and achieved expected clinical results in 93% and 97% of patients using eHF and AAF, respectively. Furthermore, few patients were switched from the hypoallergenic formula once initiated.</p><p><strong>Conclusion: </strong>The study provides new insights into HCP perspectives on treating infants with CMPA and supports using hypoallergenic formulas to manage this condition. However, additional prospective controlled studies are needed to confirm these initial findings.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/bc/75/pghn-26-277.PMC10509022.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41126369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anxiety, Depression, and Quality of Life in Parents of Adolescents with Inflammatory Bowel Disease: A Longitudinal Study.","authors":"Satomi Nomura,&nbsp;Yuri Hirano,&nbsp;Ichiro Takeuchi,&nbsp;Hirotaka Shimizu,&nbsp;Katsuhiro Arai","doi":"10.5223/pghn.2023.26.5.239","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.5.239","url":null,"abstract":"<p><strong>Purpose: </strong>The parents of adolescents with inflammatory bowel disease may experience impaired mental health and quality of life. This longitudinal study aimed to verify whether the mental health and quality of life of the parents of adolescents with inflammatory bowel disease declined when their children had active disease.</p><p><strong>Methods: </strong>Sociodemographic data, parental anxiety, depression, and quality of life were analyzed using validated questionnaires for each variable. After the baseline survey, the second and follow-up surveys were conducted at 3 and 12 months, respectively. The active disease group comprised eight parents whose children had active disease during the baseline and second surveys. The remission group comprised 14 parents whose children remained in remission during both surveys. The improved group comprised nine parents whose children experienced active disease at baseline and remission during the second survey. Parental mental health and quality of life were compared among the groups.</p><p><strong>Results: </strong>Significantly higher levels of anxiety were observed in the active disease group in all surveys (<i>p</i><0.050). Although depression levels and quality of life did not differ significantly among the three groups, pairing the active disease group with other groups showed some large effect sizes.</p><p><strong>Conclusion: </strong>Parents tended to experience decreased mental health and quality of life when their adolescents experienced active inflammatory bowel disease. Consequently, our hypothesis was partially verified. Therefore, parents need support when their children have active disease; this finding highlights the need for parental support systems.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3d/c2/pghn-26-239.PMC10509017.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41179602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Human Milk Oligosaccharide Profiles and the Secretor and Lewis Gene Status of Indonesian Lactating Mothers.","authors":"Verawati Sudarma,&nbsp;Diana Sunardi,&nbsp;Nanis Sacharina Marzuki,&nbsp;Zakiudin Munasir,&nbsp;Asmarinah,&nbsp;Adi Hidayat,&nbsp;Badriul Hegar","doi":"10.5223/pghn.2023.26.5.266","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.5.266","url":null,"abstract":"<p><strong>Purpose: </strong>Human milk oligosaccharides (HMOs) may be genetically determined based on the secretor and Lewis status of the mother. This study aims to determine the HMO profile and the secretor and Lewis gene status of Indonesian lactating mothers.</p><p><strong>Methods: </strong>Baseline data of 120 mother-infant pairs between 0-4 months post-partum obtained from a prospective longitudinal study was used. The concentrations of 2'-fucosyllactose (2'FL), lacto-N-fucopentaose I (LNFP I), lacto-N-tetraose (LNT), lacto-N-neotetraose (LNnT), 3'-sialyllactose (3'SL), and 6'-sialyllactose (6'SL) were measured. Genetic analysis was performed for mothers using targeted next-generation sequencing and Sanger sequencing. Wild-type AA with the rs1047781 (A385T) polymorphism was categorized as secretor positive, while heterozygous mutant AT was classified as a weak secretor. The presence of rs28362459 (T59G) heterozygous mutant AC and rs3745635 (G508A) heterozygous mutant CT genes indicated a Lewis negative status, and the absence of these genes indicated a positive status. Subsequently, breast milk was classified into various groups, namely Group 1: Secretor+Lewis+ (Se+Le+), Group 2: Secretor-Lewis+ (Se-Le+), Group 3: Secretor+Lewis- (Se+Le-), and Group 4: Secretor-Lewis- (Se-Le-). Data were analyzed using the Mann-Whitney and Kruskal-Wallis rank tests, and a <i>p</i>-value of 0.05 indicated statistical significance.</p><p><strong>Results: </strong>A total of 58.3% and 41.7% of the samples had positive and weak secretor statuses, respectively. The proportion of those in Group 1 was 85%, while 15% were Group 3. The results showed that only 2'FL significantly differed according to the secretor status (<i>p</i>-value=0.018).</p><p><strong>Conclusion: </strong>All Indonesian lactating mothers in this study were secretor positive, and most of them had a Lewis-positive status.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a1/19/pghn-26-266.PMC10509021.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41176900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Endoscopic Primary Prophylaxis to Prevent Bleeding in Children with Esophageal Varices: A Systematic Review and Meta-Analysis.","authors":"Fatima Safira Alatas,&nbsp;Ervin Monica,&nbsp;Lukito Ongko,&nbsp;Muzal Kadim","doi":"10.5223/pghn.2023.26.5.231","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.5.231","url":null,"abstract":"<p><strong>Purpose: </strong>This systematic review and meta-analysis aimed to compare endoscopy as primary versus secondary prophylaxis to prevent future bleeding in children with esophageal varices.</p><p><strong>Methods: </strong>A systematic literature search using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses method was conducted using the Scopus, PubMed, and Cochrane databases for relevant studies on the outcome of rebleeding events after endoscopy in primary prophylaxis compared to that in secondary prophylaxis. The following keywords were used: esophageal varices, children, endoscopy, primary prophylaxis and bleeding. The quality of eligible articles was assessed using the Newcastle-Ottawa Scale and statistically analyzed using RevMan 5.4 software.</p><p><strong>Results: </strong>A total of 174 children were included from four eligible articles. All four studies were considered of high-quality based on the Newcastle-Ottawa Quality Assessment Scale. Patients who received primary prophylaxis had 79% lower odds of bleeding than those who received secondary prophylaxis (odds ratio, 0.21; 95% confidence interval [CI], 0.07-0.66; I²=0%, <i>p</i>=0.008). Patients in the primary prophylaxis group underwent fewer endoscopic procedures to eradicate varices than those in the secondary prophylaxis group, with a mean difference of 1.73 (95% CI, 0.91-2.56; I²=62%, <i>p</i><0.0001).</p><p><strong>Conclusion: </strong>Children with high-risk varices who underwent primary prophylaxis were less likely to experience future bleeding episodes and required fewer endoscopic procedures to eradicate the varices than children who underwent secondary prophylaxis.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/01/5b/pghn-26-231.PMC10509019.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41168595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of Small Bowel Transit Time for Capsule Endoscopy in Children with Inflammatory Bowel Disease.","authors":"Itsuhiro Oka,&nbsp;Rie Funayama,&nbsp;Hirotaka Shimizu,&nbsp;Ichiro Takeuchi,&nbsp;Shuko Nojiri,&nbsp;Toshiaki Shimizu,&nbsp;Katsuhiro Arai","doi":"10.5223/pghn.2023.26.4.181","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.181","url":null,"abstract":"<p><strong>Purpose: </strong>The development of assistive devices has allowed for the performance of capsule endoscopy in children. Anticipating the capsule's transit time could affect the efficacy of the investigation and potentially minimize the fasting period. This study determined the predictors of small bowel transit time for small-bowel capsule endoscopy in children and adolescents with inflammatory bowel disease.</p><p><strong>Methods: </strong>We retrospectively examined children and adolescents with inflammatory bowel disease who underwent capsule endoscopy by the age 18 at a Japanese tertiary care children's hospital. Small bowel transit time predictors were analyzed using multiple regression with explanatory variables.</p><p><strong>Results: </strong>Overall, 92 patients, aged 1-17 years, with inflammatory bowel disease (63 Crohn's disease and 29 ulcerative colitis cases) were examined for factors affecting small bowel transit time. In the simple regression analysis, diagnosis, age, height, weight, serum albumin, general anesthesia, and small intestine lesions were significantly associated with small bowel transit time. In the multiple regression analyses, serum albumin (partial regression coefficient: -58.9, <i>p=</i>0.008), general anesthesia (partial regression coefficient: 127, <i>p<</i>0.001), and small intestine lesions (partial regression coefficient: 30.1, <i>p=</i>0.037) showed significant associations with small bowel transit time.</p><p><strong>Conclusion: </strong>Hypoalbuminemia, the use of general anesthesia for endoscopic delivery of the capsule, and small intestine lesions appeared to be predictors of prolonged small bowel transit time in children and adolescents with inflammatory bowel disease. Expecting the finishing time may improve examination with a fasting period reduction, which benefits both patients and caregivers.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e2/66/pghn-26-181.PMC10356975.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9918955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Proven Cytomegalovirus Colitis Associated with Dasatinib Administration in Two Pediatric Allogeneic Hematopoietic Stem Cell Transplantation Recipients.","authors":"Jeong Min Kim,&nbsp;Hyun Mi Kang,&nbsp;In Hyuk Yoo,&nbsp;Dong-Gun Lee,&nbsp;Nack-Gyun Chung,&nbsp;Bin Cho","doi":"10.5223/pghn.2023.26.4.224","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.224","url":null,"abstract":"<p><p>Gastrointestinal (GI) bleeding is a rare adverse event of dasatinib, which is known to be caused by dasatinib-induced colitis, severe thrombocytopenia, and platelet dysfunction. We present two cases of pediatric patients who developed hematochezia during treatment with dasatinib after hematopoietic stem cell transplantation (HSCT). A colonic tissue biopsy was performed to differentiate the cause of GI bleeding. Both patients were diagnosed with proven cytomegalovirus (CMV) colitis, but only one was treated with ganciclovir. The patient who did not receive antiviral therapy experienced recurrent GI bleeding during dasatinib administration, leading to multiple treatment interruptions. During dasatinib therapy after HSCT, patients with GI bleeding and confirmed CMV colitis may benefit from antiviral therapy to reduce interruptions in dasatinib therapy.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d6/37/pghn-26-224.PMC10356971.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Value of Nutritional Screening Tools Versus Anthropometric Measurements in Evaluating Nutritional Status of Children in a Low/Middle-Income Country.","authors":"Shaimaa Sayed,&nbsp;Mortada H F El-Shabrawi,&nbsp;Eman Abdelmonaem,&nbsp;Nehal El Koofy,&nbsp;Sara Tarek","doi":"10.5223/pghn.2023.26.4.213","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.213","url":null,"abstract":"<p><strong>Purpose: </strong>Pediatric patients in low-income countries are at a high risk of malnutrition. Numerous screening tools have been developed to detect the risk of malnutrition, including the Subjective Global Nutritional Assessment (SGNA), Pediatric Yorkhill Malnutrition Score (PYMS), Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP), and Screening Tool for Risk of Nutritional Status and Growth (STRONGkids). However, anthropometry remains the main tool for assessing malnutrition. We aimed to identify the value of four nutritional screening tools versus anthropometry for evaluating the nutritional status of children.</p><p><strong>Methods: </strong>We conducted a cross-sectional study of 1,000 children aged 1-12 years who visited the outpatient clinic of Cairo University Pediatric Hospital. Each participant was evaluated using anthropometric measurements (weight, length/height, and weight for length/height) as well as the PYMS, STAMP, STRONGkids, and SGNA screening tools. The sensitivities and specificities of these four tools were assessed using anthropometry as the gold standard.</p><p><strong>Results: </strong>Of the patients, 1.7% were underweight, 10.2% were wasted, and 35% were stunted. STRONGkids demonstrated the highest sensitivity (79.4%) and a high specificity (80.2%) for detecting malnutrition compared with weight for height, followed by STAMP, which demonstrated lower sensitivity (73.5%) but higher specificity (81.4%). PYMS demonstrated the lowest sensitivity (66.7%) and the highest specificity (93.5%), whereas SAGA demonstrated higher sensitivity (77.5%) and lower specificity (85.4%) than PYMS.</p><p><strong>Conclusion: </strong>The use of nutritional screening tools to evaluate the nutritional status of children is valuable and recommended as a simple and rapid method for identifying the risk of malnutrition in pediatric patients.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/90/b5/pghn-26-213.PMC10356972.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association Between Pediatric Gastroesophageal Reflux Disease Symptom and Quality of Life Questionnaire Score, Endoscopy and Biopsy in Children with Clinical Gastroesophageal Reflux Disease: A Prospective Study.","authors":"Fatima Safira Alatas,&nbsp;Dian Wulandaru Sukmaning Pertiwi,&nbsp;Muzal Kadim,&nbsp;Pramita Dwipoerwantoro,&nbsp;Hanifah Oswari,&nbsp;Badriul Hegar,&nbsp;Yvan Vandenplas","doi":"10.5223/pghn.2023.26.4.173","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.173","url":null,"abstract":"<p><strong>Purpose: </strong>Gastro esophageal reflux disease (GERD) is a burdensome disease affecting many children. A clinical examination is reported to be unreliable to diagnose GERD in children. This study aimed to investigate the relationship between the Pediatric Gastroesophageal Reflux Disease Symptom and Quality of Life Questionnaire (PGSQ) and endoscopic and histopathological findings in children with symptoms suggesting GERD. Changes in the PGSQ score in children with esophagitis as response to one month therapy were recorded as secondary outcome.</p><p><strong>Methods: </strong>This is a prospective cohort study in the pediatric outpatient clinic in an Indonesian tertiary hospital. Children aged 2-17 years old with clinical symptoms suspected of GERD are included in the study. Blinded endoscopic and histopathological examination was performed in all patients before one month proton pump inhibitors (PPI) therapy. The PGSQ information was collected at inclusion and after one month PPI treatment.</p><p><strong>Results: </strong>Fifty-eight subjects were included. Esophagitis was found in 60.9% of subjects according to endoscopy and 58.6% according to histology. There was no significant relationship between the PGSQ score and endoscopic (<i>p</i>=0.781) nor biopsy (<i>p</i>=0.740) examinations. The PGSQ showed a low diagnostic value compared to endoscopy and biopsy (area under the curve [AUC] 0.477, <i>p</i>=0.477, 95% confidence interval [CI] 0.326-0.629 and AUC 0.474, <i>p</i>=0.740 (95% CI 0.321-0.627 respectively). The PGSQ improved significantly post one month of PPI treatment.</p><p><strong>Conclusion: </strong>The PGSQ cannot be used to diagnose esophagitis in children with clinical symptoms suggesting GERD. However, the PGSQ can be used to monitor the treatment response in children with esophagitis.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/47/4c/pghn-26-173.PMC10356974.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9918959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Immunodeficiencies in Children Initially Admitted with Gastrointestinal/Liver Manifestations.","authors":"Murat Cakir,&nbsp;Nalan Yakici,&nbsp;Elif Sag,&nbsp;Gulay Kaya,&nbsp;Ayşenur Bahadir,&nbsp;Alper Han Cebi,&nbsp;Fazil Orhan","doi":"10.5223/pghn.2023.26.4.201","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.201","url":null,"abstract":"<p><strong>Purpose: </strong>The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs.</p><p><strong>Methods: </strong>The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded.</p><p><strong>Results: </strong>The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%).</p><p><strong>Conclusion: </strong>Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f3/98/pghn-26-201.PMC10356973.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Utility of Contrast Enhanced Ultrasound and Elastography in the Early Detection of Fibro-Stenotic Ileal Strictures in Children with Crohn's Disease.","authors":"Sarah D Sidhu,&nbsp;Shelly Joseph,&nbsp;Emily Dunn,&nbsp;Carmen Cuffari","doi":"10.5223/pghn.2023.26.4.193","DOIUrl":"https://doi.org/10.5223/pghn.2023.26.4.193","url":null,"abstract":"<p><strong>Purpose: </strong>Crohn's disease (CD) is a chronic, idiopathic bowel disorder that can progress to partial or complete bowel obstruction. At present, there are no reliable diagnostic tests that can readily distinguish between acute inflammatory, purely fibrotic and mixed inflammatory and fibrotic. Our aim is to study the utility of contrast enhanced ultrasound (CEUS) in combination with shear wave elastography (SWE) to differentiate fibrotic from inflammatory strictures in children with obstructive CD of the terminal ileum.</p><p><strong>Methods: </strong>Twenty-five (19 male) children between 2016-2021 with CD of the terminal ileum were recruited into the study. Among these patients, 22 had CEUS kinetic measurements of tissue perfusion, including wash-in slope (dB/sec), peak intensity (dB), time to peak intensity (sec), area under the curve (AUC) (dB sec), and SWE. In total, 11 patients required surgery due to bowel obstruction. Histopathologic analysis was performed by a pathologist who was blinded to the CEUS and SWE test results.</p><p><strong>Results: </strong>Patients that underwent surgical resection had significantly higher mean area under the curve on CEUS compared to patients responsive to medical therapy (<i>p</i>=0.03). The AUC also correlated with the degree of hypertrophy and the percent fibrosis of the muscularis propria, as determined by histopathologic grading (<i>p</i><0.01). There was no difference in the mean elastography measurements between these two patient groups.</p><p><strong>Conclusion: </strong>CEUS is a useful radiological technique that can help identify pediatric patients with medically refractory obstructive fibrotic strictures of the terminal ileum that should be considered for early surgical resection.</p>","PeriodicalId":19989,"journal":{"name":"Pediatric Gastroenterology, Hepatology & Nutrition","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/29/e1/pghn-26-193.PMC10356970.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865165","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}