Pub Date : 2023-10-31DOI: 10.1097/pg9.0000000000000377
Erin Hyer, Caitlin Alexander, Elizabeth B. Rand, Tamir Diamond
Fontan-associated liver disease (FALD) is a form of congestive hepatopathy resulting from Fontan palliation procedures in patients with single ventricle physiology. Although there is variation between pediatric centers, the surveillance for FALD may include liver biopsies for assessment of degree of fibrosis. Our report describes a 7-year-old girl with hypoplastic left heart syndrome who underwent Fontan palliation at age 2, and presented with disproportionate hepatomegaly, elevated liver enzymes, and increased stiffness on liver elastography. Liver biopsy showed diffuse hepatocellular cytoplasmic glycogenation, leading to the diagnosis of glycogen storage disease IX. This case demonstrates the importance of investigating unexpected physical exam findings and the potential for serendipitous benefit of liver biopsy in FALD.
{"title":"Liver Biopsy Leads to Serendipitous Diagnosis of Glycogen Storage Disease Type IX in a Patient With Fontan-Associated Liver Disease","authors":"Erin Hyer, Caitlin Alexander, Elizabeth B. Rand, Tamir Diamond","doi":"10.1097/pg9.0000000000000377","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000377","url":null,"abstract":"Fontan-associated liver disease (FALD) is a form of congestive hepatopathy resulting from Fontan palliation procedures in patients with single ventricle physiology. Although there is variation between pediatric centers, the surveillance for FALD may include liver biopsies for assessment of degree of fibrosis. Our report describes a 7-year-old girl with hypoplastic left heart syndrome who underwent Fontan palliation at age 2, and presented with disproportionate hepatomegaly, elevated liver enzymes, and increased stiffness on liver elastography. Liver biopsy showed diffuse hepatocellular cytoplasmic glycogenation, leading to the diagnosis of glycogen storage disease IX. This case demonstrates the importance of investigating unexpected physical exam findings and the potential for serendipitous benefit of liver biopsy in FALD.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"3 5","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135869554","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}
Pub Date : 2023-10-31DOI: 10.1097/pg9.0000000000000378
Abdullah Almasoud, Fernando Alvarez, Colette Deslandres
Objectives: Adenomyomatosis (ADM) of the gallbladder is a benign condition, which is characterized by mucosal hyperplasia of the gallbladder and formation of intramucosal invagination through the thickened mucosal layer. The pathogenesis is unclear. This condition is rare in children. The aim of this publication is to present the case of a teenager with ADM of the gallbladder and review the pediatric literature on this topic. Methods: A 17-year-old female presented with severe postprandial right upper quadrant abdominal pains. The abdominal ultrasound revealed ADM of the gallbladder. Results: A curative laparoscopic cholecystectomy was performed. Since 1998, eleven of the 13 pediatrics cases reported with ADM of the gallbladder were symptomatic and a cholecystectomy was curative in all of them. Conclusion: ADM of the gallbladder should be considered in the differential diagnosis of recurrent right abdominal upper quadrant pains in pediatrics. Abdominal ultrasound is the best diagnostic procedure. In symptomatic patients, a cholecystectomy is curative.
{"title":"Adenomyomatosis of the Gallbladder as a Cause of Abdominal Pain in Pediatrics: A Case Report of an Adolescent and a Literature Review","authors":"Abdullah Almasoud, Fernando Alvarez, Colette Deslandres","doi":"10.1097/pg9.0000000000000378","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000378","url":null,"abstract":"Objectives: Adenomyomatosis (ADM) of the gallbladder is a benign condition, which is characterized by mucosal hyperplasia of the gallbladder and formation of intramucosal invagination through the thickened mucosal layer. The pathogenesis is unclear. This condition is rare in children. The aim of this publication is to present the case of a teenager with ADM of the gallbladder and review the pediatric literature on this topic. Methods: A 17-year-old female presented with severe postprandial right upper quadrant abdominal pains. The abdominal ultrasound revealed ADM of the gallbladder. Results: A curative laparoscopic cholecystectomy was performed. Since 1998, eleven of the 13 pediatrics cases reported with ADM of the gallbladder were symptomatic and a cholecystectomy was curative in all of them. Conclusion: ADM of the gallbladder should be considered in the differential diagnosis of recurrent right abdominal upper quadrant pains in pediatrics. Abdominal ultrasound is the best diagnostic procedure. In symptomatic patients, a cholecystectomy is curative.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"9 4","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135870621","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}
Pub Date : 2023-10-30DOI: 10.1097/pg9.0000000000000371
Shivangi Tetarbe, Tsering Yangchen Dirkhipa, Parag Janardhan Karkera, Praveen Benjamin Dennis, Ira Shah
Trichobezoar, a rare condition of intragastric hair accumulation is commonly associated with an underlying psychological condition. Removal of the bezoar either endoscopically or surgically (laparoscopy or laparotomy) with concurrent psychiatric assessment and treatment is the mode of treatment. We present a 10-year-old child with recurrent trichobezoar, who was managed surgically the first time, and subsequently endoscopic removal was done on recurrence of bezoar after 3 months. We also present the difficulties encountered during endoscopic bezoar removal.
{"title":"Endoscopic Removal of a Recurrent Trichobezoar in an Adolescent: A Case Report","authors":"Shivangi Tetarbe, Tsering Yangchen Dirkhipa, Parag Janardhan Karkera, Praveen Benjamin Dennis, Ira Shah","doi":"10.1097/pg9.0000000000000371","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000371","url":null,"abstract":"Trichobezoar, a rare condition of intragastric hair accumulation is commonly associated with an underlying psychological condition. Removal of the bezoar either endoscopically or surgically (laparoscopy or laparotomy) with concurrent psychiatric assessment and treatment is the mode of treatment. We present a 10-year-old child with recurrent trichobezoar, who was managed surgically the first time, and subsequently endoscopic removal was done on recurrence of bezoar after 3 months. We also present the difficulties encountered during endoscopic bezoar removal.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"282 ","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136102477","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}
Pub Date : 2023-10-26DOI: 10.1097/pg9.0000000000000375
Priyanka Sahajwani, Megha Rustagi, Shivangi Tetarbe, Ira Shah
Plummer-Vinson syndrome (PVS), also called Patterson-Kelly-Brown syndrome, is a rare cause of dysphagia in children. This syndrome is associated with single or multiple webs in the upper esophagus with frequent iron deficiency. PVS usually occurs in adults, particularly in Caucasian middle-aged women, in the fourth to seventh decade of life, and is rare in childhood. There are various theories about what causes PVS. One theory suggests that iron deficiency plays a crucial role in its development. Iron repletion often improves dysphagia, although some patients require esophageal dilatation or bougienage. Herein, we describe the case of a 4-year-old male child, having complaints of difficulty in swallowing solid food, diagnosed with PVS.
{"title":"Plummer-Vinson Syndrome and Role of Endoscopic Balloon Dilatation in a 4-Year-Old Child","authors":"Priyanka Sahajwani, Megha Rustagi, Shivangi Tetarbe, Ira Shah","doi":"10.1097/pg9.0000000000000375","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000375","url":null,"abstract":"Plummer-Vinson syndrome (PVS), also called Patterson-Kelly-Brown syndrome, is a rare cause of dysphagia in children. This syndrome is associated with single or multiple webs in the upper esophagus with frequent iron deficiency. PVS usually occurs in adults, particularly in Caucasian middle-aged women, in the fourth to seventh decade of life, and is rare in childhood. There are various theories about what causes PVS. One theory suggests that iron deficiency plays a crucial role in its development. Iron repletion often improves dysphagia, although some patients require esophageal dilatation or bougienage. Herein, we describe the case of a 4-year-old male child, having complaints of difficulty in swallowing solid food, diagnosed with PVS.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"33 5","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135013166","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}
Pub Date : 2023-10-23DOI: 10.1097/pg9.0000000000000380
Regina J. Lee, Kathleen Lo, Rachel B. Schenker, Yuhua Zheng
A previously healthy 18-month-old male presented to the emergency department for rectal prolapse. Parents endorsed 2 months of intermittent, worsening rectal bulging. History is notable for the daily consumption of 32 ounces of milk. The patient was stooling daily, eating and drinking well, and gaining weight appropriately. The successful manual reduction was administered in the ED. The patient was discharged on daily polyethylene glycol (PEG) 4.25 g daily. Prolapse improved on daily PEG. However, after PEG discontinuation, prolapse returned, significantly larger and more difficult to reduce (Figs. 1 and 2). Given the worsening prolapse, pediatric surgery performed a repeat manual reduction under sedation with flexible sigmoidoscopy. The procedure was effective, and no polyps or lead points were visualized. No biopsies were obtained during the procedure. After this manual reduction under anesthesia, the patient was adherent to a regimen of 17 g of PEG daily. No further prolapse recurrences occurred.Figure 1.: Large rectal prolapse before manual reduction.Figure 2.: Lateral view of rectal prolapse.Rectal prolapse is classified as partial/mucosal or complete prolapse (1). Our patient had the latter, which involves full-thickness rectal wall extrusion. Predisposing conditions include chronic constipation (most common), increased bowel motility, celiac disease, and cystic fibrosis (1–3). Additionally, there are case reports highlighting the relationship between cow’s milk protein allergy and chronic constipation, which may warrant further consideration for the reduction and/or elimination of cow’s milk in the diet (4). Since our patient’s prolapse was most likely secondary to constipation with excessive milk intake, an additional workup was not performed. In general, management for rectal prolapse involves (1) immediate manual reduction if instantaneous spontaneous reduction does not occur and (2) constipation bowel regimens. For most children, rectal prolapse resolves with a bowel regimen alone. There are no definitive indications for surgery, but it can be considered if prolapse persists despite conservative therapy or if there is difficulty in manual reduction (1–3,5). ACKNOWLEDGMENTS All attempts have been exhausted in trying to contact the parents or guardian for the purpose of attaining their consent to publish the Image.
先前健康的18个月大的男性直肠脱垂提交到急诊科。父母表示2个月间歇性，恶化的直肠鼓胀。历史上每天消耗32盎司的牛奶是值得注意的。患者每日便便，饮食正常，体重适当增加。在急诊科进行了成功的手动复位。患者出院时每天服用4.25 g聚乙二醇(PEG)。每日PEG可改善脱垂。然而，停用PEG后，脱垂复发，明显更大，更难以复位(图1和2)。鉴于脱垂恶化，儿科外科在镇静下使用弹性乙状结肠镜进行了重复的手动复位。手术是有效的，没有息肉或铅点可见。手术过程中未进行活检。在麻醉下手工复位后，患者坚持每日17 g PEG的治疗方案。无脱垂复发。图1所示。手法复位前直肠大脱垂。图2。:直肠脱垂侧位图。直肠脱垂分为部分/粘膜脱垂和完全脱垂(1)。本例患者为完全性脱垂，包括全层直肠壁挤压。易感条件包括慢性便秘(最常见)、肠蠕动增加、乳糜泻和囊性纤维化(1-3)。此外，有病例报告强调了牛奶蛋白过敏和慢性便秘之间的关系，这可能需要进一步考虑减少和/或消除饮食中的牛奶(4)。由于我们患者的脱垂很可能是继发于过量牛奶摄入引起的便秘，因此没有进行额外的检查。一般来说，直肠脱垂的治疗包括(1)如果没有发生即时的自发减少，立即手动减少(2)便秘肠方案。对于大多数儿童来说，直肠脱垂只需要排便即可解决。没有明确的手术指征，但如果保守治疗后脱垂仍然存在或手动复位困难，可以考虑手术(1 - 3,5)。我们已尽一切努力与家长或监护人联系，以获得他们对发布图片的同意。
{"title":"Recurrent Rectal Prolapse Successfully Treated With Polyethylene Glycol","authors":"Regina J. Lee, Kathleen Lo, Rachel B. Schenker, Yuhua Zheng","doi":"10.1097/pg9.0000000000000380","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000380","url":null,"abstract":"A previously healthy 18-month-old male presented to the emergency department for rectal prolapse. Parents endorsed 2 months of intermittent, worsening rectal bulging. History is notable for the daily consumption of 32 ounces of milk. The patient was stooling daily, eating and drinking well, and gaining weight appropriately. The successful manual reduction was administered in the ED. The patient was discharged on daily polyethylene glycol (PEG) 4.25 g daily. Prolapse improved on daily PEG. However, after PEG discontinuation, prolapse returned, significantly larger and more difficult to reduce (Figs. 1 and 2). Given the worsening prolapse, pediatric surgery performed a repeat manual reduction under sedation with flexible sigmoidoscopy. The procedure was effective, and no polyps or lead points were visualized. No biopsies were obtained during the procedure. After this manual reduction under anesthesia, the patient was adherent to a regimen of 17 g of PEG daily. No further prolapse recurrences occurred.Figure 1.: Large rectal prolapse before manual reduction.Figure 2.: Lateral view of rectal prolapse.Rectal prolapse is classified as partial/mucosal or complete prolapse (1). Our patient had the latter, which involves full-thickness rectal wall extrusion. Predisposing conditions include chronic constipation (most common), increased bowel motility, celiac disease, and cystic fibrosis (1–3). Additionally, there are case reports highlighting the relationship between cow’s milk protein allergy and chronic constipation, which may warrant further consideration for the reduction and/or elimination of cow’s milk in the diet (4). Since our patient’s prolapse was most likely secondary to constipation with excessive milk intake, an additional workup was not performed. In general, management for rectal prolapse involves (1) immediate manual reduction if instantaneous spontaneous reduction does not occur and (2) constipation bowel regimens. For most children, rectal prolapse resolves with a bowel regimen alone. There are no definitive indications for surgery, but it can be considered if prolapse persists despite conservative therapy or if there is difficulty in manual reduction (1–3,5). ACKNOWLEDGMENTS All attempts have been exhausted in trying to contact the parents or guardian for the purpose of attaining their consent to publish the Image.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"20 11","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135367223","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}
Pub Date : 2023-10-09DOI: 10.1097/pg9.0000000000000374
Tu Cam Nguyen, Annie Robert, Thu Hien Anh Pham, Khoa Hoang Vo, Loc Duc Le, Ha Tu Ma, My Huynh Thao Le, Thai Hoang Che, Hiep Thanh Nguyen, Dinh Quang Truong, Patrick Bontems, Phuong Ngoc Van Nguyen
Objectives: This study assessed the diagnostic value of a monoclonal immunoassay stool antigen test (HpSA) for Helicobacter pylori (H. pylori ) infection and the eradication outcomes. Methods: Children undergoing digestive endoscopy at 2 Children’s Hospitals in Ho Chi Minh City were recruited. Treatment was offered to H. pylori -infected children. Stool samples were collected on the same day as the endoscopy procedure and after 6 weeks post-treatment for HpSA. Diagnostic value and optimal cutoff of HpSA were assessed using biopsy-based tests as the gold standard. Eradication was defined as a negative HpSA post-treatment. Ethical approval was obtained, and informed consent was signed by the participants. Results: In total, 394 patients participated in the study. The most common symptoms were epigastric pain (74.6%) and vomiting (37.3%). H. pylori status was positive in 78% of patients (306/394), doubtful in 10.1%, and negative in 12.2%. HpSA was positive in 73.2% (142/194). Excluding doubtful infections, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of HpSA were 87.4%, 95.2%, 99.2%, 51.3%, and 88.4%, respectively. The optimal cutoff value of 0.148 provided similar accuracy to the recommended cutoff. The eradication rate was 56.1% in per-protocol analysis and 27.9% in intention-to-treat analysis. Treatment success was higher in boys, but lower among malnourished children and those infected with cagA+ strains. Conclusions: The HpSA is reliable for identifying H. pylori infection in epidemiological studies and assessing eradication outcomes. The low eradication rate highlights the need for an appropriate intervention strategy in Vietnamese children.
{"title":"Helicobacter pylori Eradication Rate Using Stool Antigen Test in Vietnamese Children: A Prospective Multicenter Study","authors":"Tu Cam Nguyen, Annie Robert, Thu Hien Anh Pham, Khoa Hoang Vo, Loc Duc Le, Ha Tu Ma, My Huynh Thao Le, Thai Hoang Che, Hiep Thanh Nguyen, Dinh Quang Truong, Patrick Bontems, Phuong Ngoc Van Nguyen","doi":"10.1097/pg9.0000000000000374","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000374","url":null,"abstract":"Objectives: This study assessed the diagnostic value of a monoclonal immunoassay stool antigen test (HpSA) for Helicobacter pylori (H. pylori ) infection and the eradication outcomes. Methods: Children undergoing digestive endoscopy at 2 Children’s Hospitals in Ho Chi Minh City were recruited. Treatment was offered to H. pylori -infected children. Stool samples were collected on the same day as the endoscopy procedure and after 6 weeks post-treatment for HpSA. Diagnostic value and optimal cutoff of HpSA were assessed using biopsy-based tests as the gold standard. Eradication was defined as a negative HpSA post-treatment. Ethical approval was obtained, and informed consent was signed by the participants. Results: In total, 394 patients participated in the study. The most common symptoms were epigastric pain (74.6%) and vomiting (37.3%). H. pylori status was positive in 78% of patients (306/394), doubtful in 10.1%, and negative in 12.2%. HpSA was positive in 73.2% (142/194). Excluding doubtful infections, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of HpSA were 87.4%, 95.2%, 99.2%, 51.3%, and 88.4%, respectively. The optimal cutoff value of 0.148 provided similar accuracy to the recommended cutoff. The eradication rate was 56.1% in per-protocol analysis and 27.9% in intention-to-treat analysis. Treatment success was higher in boys, but lower among malnourished children and those infected with cagA+ strains. Conclusions: The HpSA is reliable for identifying H. pylori infection in epidemiological studies and assessing eradication outcomes. The low eradication rate highlights the need for an appropriate intervention strategy in Vietnamese children.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135146455","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}
Δ 4 -3-Oxosteroid 5β-reductase ( AKR1D1 ) deficiency typically causes severe cholestasis occurs in newborns, leading to death unless patients are treated with primary bile acids. However, we encountered an AKR1D1 deficiency patient treated with only ursodeoxycholic acid who had cholestasis until about 1 year of age but then grew up healthy without further treatment. We also have been following other healthy patients with AKR1D1 mutation who have never developed cholestasis and have not been treated. However, reports are few, involving 3 patients. To better understand and clinically manage a diverse group of patients with AKR1D1 mutation who do not develop potentially fatal cholestasis in the neonatal period, ongoing accumulation and study of informative cases is needed.
{"title":"Healthy Patients With AKR1D1 Mutation Not Requiring Primary Bile Acid Therapy: A Case Series","authors":"Akihiko Kimura, Jun Mori, Anh-Hoa Nguyen Pham, Kim-Oanh Bui Thi, Hajime Takei, Tsuyoshi Murai, Hisamitsu Hayashi, Hiroshi Nittono","doi":"10.1097/pg9.0000000000000372","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000372","url":null,"abstract":"Δ 4 -3-Oxosteroid 5β-reductase ( AKR1D1 ) deficiency typically causes severe cholestasis occurs in newborns, leading to death unless patients are treated with primary bile acids. However, we encountered an AKR1D1 deficiency patient treated with only ursodeoxycholic acid who had cholestasis until about 1 year of age but then grew up healthy without further treatment. We also have been following other healthy patients with AKR1D1 mutation who have never developed cholestasis and have not been treated. However, reports are few, involving 3 patients. To better understand and clinically manage a diverse group of patients with AKR1D1 mutation who do not develop potentially fatal cholestasis in the neonatal period, ongoing accumulation and study of informative cases is needed.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"139 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135093079","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}
Pub Date : 2023-10-09DOI: 10.1097/pg9.0000000000000370
Shivangi Tetarbe, Suhani Jain, Ira Shah
Gut inflammation and defect in the gut mucosal barrier appear to have a correlation with skin diseases and vice versa. The coexistence of hereditary ichthyosis with active colitis has never been reported. We present a 17-year-old female with ichthyosis since birth, abdomen pain for 3 months, with acute colitis. After the initial diagnosis, the patient was started on antituberculous therapy (ATT), steroids, and mesalamine. She followed up with us for 1 year where there was resolution of symptoms. Steroids were stopped after 16 weeks, mesalamine was stopped after 20 weeks in view of low absolute neutrophil counts and ATT was stopped after 1 year. She was asymptomatic post 18 months of stopping ATT.
{"title":"Hereditary Ichthyosis With Gastrointestinal Manifestations: A Case Report","authors":"Shivangi Tetarbe, Suhani Jain, Ira Shah","doi":"10.1097/pg9.0000000000000370","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000370","url":null,"abstract":"Gut inflammation and defect in the gut mucosal barrier appear to have a correlation with skin diseases and vice versa. The coexistence of hereditary ichthyosis with active colitis has never been reported. We present a 17-year-old female with ichthyosis since birth, abdomen pain for 3 months, with acute colitis. After the initial diagnosis, the patient was started on antituberculous therapy (ATT), steroids, and mesalamine. She followed up with us for 1 year where there was resolution of symptoms. Steroids were stopped after 16 weeks, mesalamine was stopped after 20 weeks in view of low absolute neutrophil counts and ATT was stopped after 1 year. She was asymptomatic post 18 months of stopping ATT.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"34 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135094609","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}
Objectives: According to our previous study, the 2-dose-BNT162b2 vaccination is less effective against the Omicron variant. This study aimed to assess the safety and efficacy of a 3-dose-BNT162b2 vaccination in liver-transplanted (LT) and healthy adolescents. Methods: LT and healthy adolescents who met the inclusion criteria received a third dose of the BNT162b2 vaccine (30 µg). Antireceptor-binding domain immunoglobulin and T-cell-specific responses to severe acute respiratory syndrome coronavirus 2 spike peptides were assessed 3 months before the third dose (Visit −1) and 0 (Visit 0), 1 (Visit 1), and 2 months (Visit 2) after the third dose. Antinucleocapsid immunoglobulin and neutralizing antibodies were assessed at Visits 0 and 1. Adverse events (AEs) were monitored. Results: Eleven LT and 14 healthy adolescents aged 14.64 (13.2, 15.7) years (44.2% male) had antireceptor-binding domain immunoglobulin geometric mean titers of 1412.47 (95% confidence interval [CI], 948.18–2041.11) and 1235.79 (95% CI, 901.07–1705.73) U/mL at Visit −1 but increased to 38 587.76 (95% CI, 24 628.03–60 460.18) and 29 222.38 (95% CI, 16 291.72–52 401.03) U/mL ( P < 0.05) at Visit 1, respectively. This was consistent with neutralizing antibodies (42.29% and 95.37% vs 44.65% and 91.68%, P < 0.001) and interferon-γ-secreting cells in LT and healthy adolescents at Visit 0 versus Visit 1, respectively. For serious AEs, an LT girl with autoimmune overlap syndrome died 5 months postvaccination from acute liver failure. Conclusions: In both LT and healthy adolescents, humoral and cellular immune responses were high after the 3-dose-BNT162b2 vaccination. However, serious AEs were suspected in LT adolescents with autoimmune diseases.
{"title":"Safety and Efficacy of a Third Dose of the BNT162b2 Vaccine in Liver-Transplanted and Healthy Adolescents","authors":"Palittiya Sintusek, Supranee Buranapraditkun, Siriporn Khunsri, Thanunrat Thongmee, Preeyaporn Vichaiwattana, Warunee Polsawat, Yong Poovorawan","doi":"10.1097/pg9.0000000000000373","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000373","url":null,"abstract":"Objectives: According to our previous study, the 2-dose-BNT162b2 vaccination is less effective against the Omicron variant. This study aimed to assess the safety and efficacy of a 3-dose-BNT162b2 vaccination in liver-transplanted (LT) and healthy adolescents. Methods: LT and healthy adolescents who met the inclusion criteria received a third dose of the BNT162b2 vaccine (30 µg). Antireceptor-binding domain immunoglobulin and T-cell-specific responses to severe acute respiratory syndrome coronavirus 2 spike peptides were assessed 3 months before the third dose (Visit −1) and 0 (Visit 0), 1 (Visit 1), and 2 months (Visit 2) after the third dose. Antinucleocapsid immunoglobulin and neutralizing antibodies were assessed at Visits 0 and 1. Adverse events (AEs) were monitored. Results: Eleven LT and 14 healthy adolescents aged 14.64 (13.2, 15.7) years (44.2% male) had antireceptor-binding domain immunoglobulin geometric mean titers of 1412.47 (95% confidence interval [CI], 948.18–2041.11) and 1235.79 (95% CI, 901.07–1705.73) U/mL at Visit −1 but increased to 38 587.76 (95% CI, 24 628.03–60 460.18) and 29 222.38 (95% CI, 16 291.72–52 401.03) U/mL ( P < 0.05) at Visit 1, respectively. This was consistent with neutralizing antibodies (42.29% and 95.37% vs 44.65% and 91.68%, P < 0.001) and interferon-γ-secreting cells in LT and healthy adolescents at Visit 0 versus Visit 1, respectively. For serious AEs, an LT girl with autoimmune overlap syndrome died 5 months postvaccination from acute liver failure. Conclusions: In both LT and healthy adolescents, humoral and cellular immune responses were high after the 3-dose-BNT162b2 vaccination. However, serious AEs were suspected in LT adolescents with autoimmune diseases.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"46 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135146452","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}
Pub Date : 2023-10-05DOI: 10.1097/pg9.0000000000000353
Adolfo Bautista-Casasnovas, Federico Argüelles-Martín, Benjamín Martín-Martínez, María Jose Domínguez-Otero, Marta Tavares, Jorge Amil-Dias
Background: Constipation is a common clinical problem in children, for which the first-line therapeutic options are osmotic laxatives, mainly polyethylene glycol (PEG). These treatments are often prescribed for short or limited periods, with progressive treatment withdrawal often resulting in relapses. However, there are a few studies into the long-term use (≥6 months) of PEG 3350 with electrolytes (PEG+E) in terms of the patients’ clinical evolution. Objectives: To assess bowel movement and other relevant symptoms in children with constipation receiving PEG+E (≥6 months), as well as parent/caregiver satisfaction with this treatment. Methods: A retrospective, observational, descriptive, longitudinal, and multicentre study was carried out on 74 children diagnosed with functional constipation (ROME IV criteria) who had received PEG+E (≥6 months). Bowel control was assessed using the Bristol stool scale, and the parent’s/caregiver’s perception of the treatment was also evaluated employing a nonvalidated questionnaire. Results: Children with an average duration of constipation >1 year experienced a significant improvement in bowel movements and stool consistency when using PEG+E. The mean duration of use was 18.6 (±13.4) months, without the need to adjust the dose for weight. All clinical symptoms improved significantly except bloating, and all the parents/caregivers confirmed these clinical improvements. Conclusions: Children treated with PEG+E (≥6 months) normalised their bowel movements, improving the clinical symptoms related to constipation in the absence of serious advert events or the need for dosage adjustments due to weight gain. Parents/caregivers reported good satisfaction with PEG+E treatment.
{"title":"Multicentre Study Into the Use of Polyethylene Glycol With Electrolytes Over at Least 6 Months to Treat Constipation in Paediatric Populations","authors":"Adolfo Bautista-Casasnovas, Federico Argüelles-Martín, Benjamín Martín-Martínez, María Jose Domínguez-Otero, Marta Tavares, Jorge Amil-Dias","doi":"10.1097/pg9.0000000000000353","DOIUrl":"https://doi.org/10.1097/pg9.0000000000000353","url":null,"abstract":"Background: Constipation is a common clinical problem in children, for which the first-line therapeutic options are osmotic laxatives, mainly polyethylene glycol (PEG). These treatments are often prescribed for short or limited periods, with progressive treatment withdrawal often resulting in relapses. However, there are a few studies into the long-term use (≥6 months) of PEG 3350 with electrolytes (PEG+E) in terms of the patients’ clinical evolution. Objectives: To assess bowel movement and other relevant symptoms in children with constipation receiving PEG+E (≥6 months), as well as parent/caregiver satisfaction with this treatment. Methods: A retrospective, observational, descriptive, longitudinal, and multicentre study was carried out on 74 children diagnosed with functional constipation (ROME IV criteria) who had received PEG+E (≥6 months). Bowel control was assessed using the Bristol stool scale, and the parent’s/caregiver’s perception of the treatment was also evaluated employing a nonvalidated questionnaire. Results: Children with an average duration of constipation >1 year experienced a significant improvement in bowel movements and stool consistency when using PEG+E. The mean duration of use was 18.6 (±13.4) months, without the need to adjust the dose for weight. All clinical symptoms improved significantly except bloating, and all the parents/caregivers confirmed these clinical improvements. Conclusions: Children treated with PEG+E (≥6 months) normalised their bowel movements, improving the clinical symptoms related to constipation in the absence of serious advert events or the need for dosage adjustments due to weight gain. Parents/caregivers reported good satisfaction with PEG+E treatment.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"81 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":"135480896","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}
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