JPGN Reports最新文献

{"title":"Gastrointestinal Bleeding in Children: The Role of Endoscopy and the Sheffield Scoring System in a Resource-Limited Setting","authors":"Oluwafunmilayo Funke Adeniyi, Olufunmilayo Adenike Lesi, Emuobor Aghoghor Odeghe, Ganiyat Oyeleke, Nicholas Croft","doi":"10.1097/pg9.0000000000000369","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000369","url":null,"abstract":"Objective: To document the clinical presentation, endoscopic diagnosis, and Sheffield scores of children with gastrointestinal (GI) bleeding who were referred for endoscopy at the Lagos University Teaching Hospital. The participants who needed endoscopy based on clinical criteria and according to the Sheffield scores were also documented. Methods: This study analyzed the records of 111 children with GI bleeding retrospectively from January 2013 to January 2021, while 9 children were recruited prospectively from February 2021 to March 2022. Receiver operating curves and area under the curve were generated to test the ability of the Sheffield scores to predict rebleeds, mortality, and the need for endoscopic intervention for upper GI bleeds. Results: One hundred and twenty participants were recruited. Ninety-one (75.8%) presented with upper GI bleeding (UGIB), while 29 (24.2%) had lower GI bleeding (LGIB). Only 70 (58.3%) (53 UGIB and 17 LGIB) had endoscopy performed. For UGIB, 5 (9.4%) had no source of the bleeding identified at endoscopy, 12 (22.6%) had variceal bleeding, and 36 (67.9%) had nonvariceal bleeding. Colonoscopy revealed juvenile polyps in 5 (29.4%), indeterminate colitis in 5 (29.4%), ulcerative colitis in 4 (23.5%), Crohn’s disease in 1 (5.9%), and hemorrhoids in 2 (11.8%) participants, respectively. The Sheffield score was ≥8 in 42 (46.1%) of the participants who presented only with UGIB (hematemesis and melena). The scores were significantly related to the type of bleeds, rebleeds, and deaths ( P = 0.00). Conclusion: The clinical and endoscopic findings in this study are similar to those reported previously. The Sheffield scoring was useful in assessing Nigerian children. However, due to limited access and other restraints, endoscopy was not performed on all the study participants even when the scoring system was suggestive. The availability, and therefore, utility of GI endoscopy in this setting are still suboptimal. The need for the provision of adequate equipment and resources and the training of personnel is thus recommended.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"58 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134975801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful Treatment of Recurrent Pyloric Stenosis Using Balloon Dilation","authors":"Jesseca R. A. Pirkle, David Deutsch","doi":"10.1097/pg9.0000000000000364","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000364","url":null,"abstract":"Infantile hypertrophic pyloric stenosis is a common surgical disease in infants, with an incidence of 2 to 5 cases per 1000 live births. It often presents with nonbilious projectile vomiting after feeding and a mid-epigastric mass in infants between the third and eighth weeks of life. Ramstedt pyloromyotomy remains the gold standard of treatment. Postoperative emesis is common; however, further evaluation for incomplete pyloromyotomy and recurrent pyloric stenosis should be conducted with prolonged, or new-onset postoperative emesis. While repeat pyloromyotomy is the standard of care for infants presenting with incomplete pyloric stenosis, treatment for the rare development of recurrent pyloric stenosis is not clearly outlined. Here, we report a successful balloon dilation procedure in an 8-week-old female with recurrent pyloric stenosis three and a half weeks after the initial laparoscopic pyloromyotomy.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"32 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135814466","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retching Without Vomiting With Acute Abdominal Distension: A Clinical Cue","authors":"Zahabiya Nalwalla, Tsering Yangchen Dirkhipa, Rishabh Jain, Ira Shah, Pradnya Bendre","doi":"10.1097/pg9.0000000000000363","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000363","url":null,"abstract":"Gastric volvulus leading to acute gastric dilatation is a rare presentation of congenital diaphragmatic hernia. Urgent detorsion with gastropexy and closure of the diaphragmatic defect are essential to prevent further complications and recurrence. We present a rare case of an infant with acute gastric dilatation due to acute gastric volvulus secondary to congenital diaphragmatic hernia.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"35 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135814631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Looking Beyond the Growth Curve: A Retrospective Study on Nutrient Deficient Diets in Children with Severe Food Selectivity","authors":"Amy K. Drayton, Rachel M. Knight, Heather Shepard, Ashley S. Andersen, Daniel Shriver","doi":"10.1097/pg9.0000000000000365","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000365","url":null,"abstract":"Severe food selectivity places children at risk for nutrient deficiencies and long-term medical complications, if unaddressed. However, poor nutrition in highly selective eaters is often overlooked when considering other behavioral or medical concerns. Additionally, studies regarding food selectivity are sparse and limited to children with developmental delays. This study further investigates the nutritional deficiencies and growth characteristics of children with severe food selectivity to assist pediatricians in the earlier identification of patients for nutrition screening. A retrospective chart review was completed for 13 patients admitted to a pediatric feeding program solely for table-textured food selectivity. Nutrition and anthropometric data from the medical record were analyzed. All patients were determined to be following an age-appropriate growth curve but had multiple micronutrient deficiencies. Additionally, 6 children were typically developing outside of the feeding context. Results suggest that children with food selectivity may require more intensive and earlier nutrition screening beyond their growth patterns.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"15 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135552745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multiorgan Failure in a Malnourished Infant: Inappropriate Use of Goat Milk, Dilution, or Both?","authors":"Andrea Gosalvez-Tejada, Sara Mapa, Ruba Azzam, Ankur Chugh","doi":"10.1097/pg9.0000000000000367","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000367","url":null,"abstract":"Goat milk is gaining popularity in the United States as an alternative to cow and soy milk. The milk is presented as a healthier and less allergenic alternative, with casein and more MCT oil. The interest in goat milk has increased significantly with the recent formula shortage. Goat milk is available in many forms in the United States, including liquid and powdered formulations. However, there are no approved infant formulas in the United States that are goat milk-based. This case describes an infant who became critically ill due to family confusion over goat milk feeds, highlighting the importance of understanding the nutritional components and safety of various goat milk formulations.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134911038","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Central Venous Catheter Replacement After Candida in Pediatric Intestinal Failure Patients","authors":"Daphna Katz, Natalia Jelen, Alana Xavier de Almeida, Alberto Cruz, Brandon Chatani, Amanda Fifi","doi":"10.1097/pg9.0000000000000358","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000358","url":null,"abstract":"Background: Deferred central venous catheter (CVC) replacement places children with intestinal failure (IF) at risk of complications. We hypothesized that early CVC replacement after uncomplicated candidemia is safe and beneficial. Methods: We performed a retrospective review of children with IF. Patients were divided into early (<7 days after their first negative culture), and late (≥7 days after their first negative culture) CVC replacement following uncomplicated candidemia. We calculated the median time to CVC removal, clearance of infection, CVC replacement or exchange, and duration of the initial hospitalization. The proportion of patients readmitted within 30 days was also calculated, taking note of the number of candida reinfections. Results: Early replacement occurred in 18 encounters and late replacement in 21 encounters. The median time in both groups to CVC removal was 3 days ( P = 0.949), and clearance of infection was 4 days ( P = 0.466). The median time to CVC replacement or exchange in the early group was 4 days, compared to 10 days in the late group ( P < 0.001). The median duration of the hospitalization in the early group was 12 days compared to 21 days in the late group ( P = 0.011). In total 39% of patients from the early group were readmitted within 30 days compared to 57% from the late group ( P = 0.359). None of the patients were reinfected with candida within 30 days. Conclusion: Early CVC replacement after uncomplicated candidemia in children with IF decreases hospital stay without increased risk of readmission or reinfection.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"64 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135553061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gluten-Free Diet Knowledge and Adherence in Adolescents with Celiac Disease: A Cross-Sectional Study.","authors":"Katherine Pohoreski,&nbsp;Simonne L Horwitz,&nbsp;Dominica Gidrewicz","doi":"10.1097/PG9.0000000000000330","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000330","url":null,"abstract":"<p><strong>Objectives: </strong>This study examined the relationship between knowledge of, and adherence to, the gluten-free diet (GFD) in a local population of adolescents with celiac disease (CD). The secondary objectives were to identify information sources used to learn about the GFD and to compare adolescents' and parents' knowledge of the GFD.</p><p><strong>Methods: </strong>Adolescents (12-17 years) with CD and their parents from pediatric gastroenterology clinics in Calgary, Alberta, completed an online survey containing a knowledge assessment (Gluten-Free Diet Quiz [GFD-Q]), an adherence scale, questions about GFD information sources, and demographic/clinical information. GFD-Q scores were deemed \"sufficient knowledge\" with correct identification of 3/3 gluten-containing foods, ≥4/7 gluten-free foods, and ≥ 4/7 foods that may contain gluten; otherwise, scores were termed \"insufficient knowledge\".</p><p><strong>Results: </strong>Of the 40 adolescent-parent pairs, 15 of 40 adolescents (37%) had sufficient knowledge, and 25 of 40 adolescents (63%) had insufficient knowledge. Within the insufficient knowledge group, 14 of 25 (56%) did not correctly identify enough allowed gluten-free foods. Parents scored higher on the GFD-Q (67% had sufficient knowledge). Adolescents reported overall adherence to the GFD (88%), with adherence being similar between the sufficient and insufficient knowledge groups (80% versus 92%). The most helpful information sources included physicians, another person with CD, parent(s), and Google; apps were infrequently used.</p><p><strong>Conclusion: </strong>Adolescents report good adherence; however, they struggle with GFD knowledge, particularly in identifying gluten-free foods. Further research is required to explore GFD educational tools, including mobile apps and dietician-led teaching sessions.</p>","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e330"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10049479","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":"Hypocoagulability in Children With Decompensated Chronic Liver Disease and Sepsis: Assessment by Thromboelastography.","authors":"Vignesh Vinayagamoorthy,&nbsp;Anshu Srivastava,&nbsp;Indranil Das,&nbsp;Anupam Verma,&nbsp;Prabhakar Mishra,&nbsp;Moinak Sen Sarma,&nbsp;Ujjal Poddar,&nbsp;Surender Kumar Yachha","doi":"10.1097/PG9.0000000000000324","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000324","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the coagulation status of children with decompensated chronic liver disease (DCLD) and infection and factors affecting it using thromboelastography (TEG).</p><p><strong>Methods: </strong>Coagulation status of children admitted with DCLD and infection was assessed by international normalized ratio (INR), platelet count, and TEG [reaction time (R), kinetic time (K), α-angle (AA), maximum amplitude (MA), coagulation index (CI), and lysis index (LY30)] at admission and at 7-14 days after treatment. CI < -3 represents hypocoagulable state. Clinical profile including systemic inflammatory response syndrome (SIRS), infection severity, bleeding, treatment response, and outcome were noted.</p><p><strong>Results: </strong>Thirty children (21 boys, median (IQR) age 78 [15.7-180] months) were studied prospectively. At admission, 29 (96.7%) had prolonged INR, 24 (80%) had thrombocytopenia, and 17 (56.6%) were hypocoagulable by TEG. Nine of 30 (30%) had normal TEG but deranged INR and platelets. Nineteen (63.3%) cases had SIRS, 11 (36.6%) had severe sepsis, and 8 (26.6%) had bleeding. Hypocoagulable state was common in severe sepsis than sepsis/infection (81.1% versus 42.1%; <i>P</i> = 0.05) and persistent (n = 4) versus recovered SIRS (n = 15, 100% versus 33%; <i>P</i> = 0.03). Bleeders had prolonged R-time (7.8 versus 5.4 min; <i>P</i> = 0.03), smaller MA (30.2 versus 47 mm; <i>P</i> = 0.05), and α-angle (40.4 versus 62.9; <i>P</i> = 0.03) but similar INR and platelets than nonbleeders. Six patients (20%) had poor in-hospital outcomes; R-time ≥8.5 min predicted mortality with high sensitivity (83%) and specificity (100%).</p><p><strong>Conclusions: </strong>Fifth-seven percent of children with DCLD and infection were hypocoagulable by TEG. Severe sepsis and persistent SIRS worsened the coagulation status. TEG identifies bleeders better than INR and platelet count. R-time ≥8.5 min predicts a poor hospital outcome.</p>","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e324"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435032/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10046614","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":"Fibrosing Colonopathy Presenting in a Patient with Cystinosis.","authors":"Dominic Fiore,&nbsp;Vidhur Sohini,&nbsp;Elizabeth Mileti,&nbsp;Nicholas Fiore","doi":"10.1097/PG9.0000000000000321","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000321","url":null,"abstract":"<p><p>Fibrosing colonopathy is a unique pathology characterized by long segment stricture, usually of the ileocecal region. Historically, it is most commonly described in patients with cystic fibrosis (CF). Fibrosing colonopathy is felt to be secondary to excessive doses of exogenous lipase medication. This condition is rarely seen in the last decade. In this case presentation, fibrosing colonopathy was identified in a patient with the lysosomal storage disorder of cystinosis. Fibrosing colonopathy has not previously been described in patients with cystinosis. The patient was found to have fibrosing colonopathy after perforation of the colon during a colonoscopy for bloody diarrhea. This case report aims to draw attention to a noteworthy case of fibrosing colonopathy in a patient who does not have cystic fibrosis, but rather cystinosis.</p>","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e321"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/99/65/pg9-4-e321.PMC10435035.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10049476","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":"Outcomes of Tofacitinib Use in an Irish Pediatric Cohort.","authors":"Niamh Ryan,&nbsp;Sarah Cooper,&nbsp;Anna Dominik,&nbsp;Shoana Quinn,&nbsp;Annemarie Broderick,&nbsp;Billy Bourke,&nbsp;Séamus Hussey","doi":"10.1097/PG9.0000000000000332","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000332","url":null,"abstract":"<p><strong>Background: </strong>Pediatric ulcerative colitis (UC) is typically more extensive and severe at diagnosis compared with adult disease. Tofacitinib, a Janus kinase inhibitor, has been used since 2018 to induce and maintain remission in UC. There are limited pediatric data regarding its use, either as a monotherapy or in combination with other treatments.</p><p><strong>Objectives: </strong>To determine the real-world experience and outcomes of tofacitinib therapy in the Irish national cohort with pediatric UC.</p><p><strong>Methods: </strong>A retrospective study of tofacitinib outcomes was undertaken at Ireland's single national center for pediatric inflammatory bowel disease. All patients commenced on tofacitinib since its availability in 2019 were included. Baseline and follow-up clinical characteristics, phenotype, Pediatric Ulcerative Colitis Activity Index (PUCAI) scores, and treatments before and after tofacitinib commenced were recorded. The primary outcome was remission by 8 weeks, with other clinical outcomes being recorded to maximal available follow-up.</p><p><strong>Results: </strong>Between November 1, 2019 and June 30, 2022, 15 children (M:F 1:2) were prescribed tofacitinib, 5 as monotherapy. Thirteen had baseline pancolitis at diagnosis and all patients had prior infliximab exposure. The mean time from diagnosis to starting tofacitinib was 381 days (±SD 265). Dual therapy included 5 with infliximab, 4 with vedolizumab, and 1 with adalimumab. The average length of treatment on tofacitinib was 232 days (±SD 170) with 2 patients transitioning to adult services while in remission on tofacitinib therapy. The mean PUCAI score was 48.7 (±SD 14.1) pre-tofacitinib, 16.7 (±SD 15.6) at week 8, and 22.5 (±SD 29.6) by week 16, with a significant reduction in PUCAI by week 16 (<i>P</i> = 0.0004). Eight patients (3 monotherapy) achieved clinical remission, with 4 of the 5 dual therapy patients on infliximab. There were no significant outcome differences between those on mono- or dual therapy. Three patients with combined vedolizumab therapy did not achieve remission, 2 of whom required colectomy by week 24. There were no malignancies, 1 patient developed shingles and another developed herpangina post-tofacitinib. Failure to achieve clinical remission by week 16 was seen in all children who progressed to colectomy (n = 4).</p><p><strong>Conclusion: </strong>Combining tofacitinib with other biologics is effective in select children with refractory UC. Early responders were more likely to achieve a sustained response at week 16. Failure to achieve remission by week 16 of tofacitinib therapy was strongly associated with progression to colectomy.</p>","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e332"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/91/85/pg9-4-e332.PMC10435039.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10051610","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}