JPGN Reports最新文献_第7页

Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report. 小儿急性肝衰竭继发于自身免疫性肝炎婴儿伴血小板减少-桡骨缺失(TAR)综合征:1例报告。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000325

Rebecca Mercedes, Kalyani Patel, Henry Shiau, Krupa R Mysore, Wenly Ruan, Daniel H Leung, Mary Elizabeth M Tessier, Dana Cerminara, Sarah Nicholas, Kelby Fuller, Marielle Faraone, N Thao N Galvan, John Goss, Anna M Banc-Husu

{"title":"Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report.","authors":"Rebecca Mercedes, Kalyani Patel, Henry Shiau, Krupa R Mysore, Wenly Ruan, Daniel H Leung, Mary Elizabeth M Tessier, Dana Cerminara, Sarah Nicholas, Kelby Fuller, Marielle Faraone, N Thao N Galvan, John Goss, Anna M Banc-Husu","doi":"10.1097/PG9.0000000000000325","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000325","url":null,"abstract":"Thrombocytopenia absent radius (TAR) syndrome is a rare genetic disorder that has been associated with food protein-induced allergic proctocolitis and transient leukemoid reactions, among other manifestations. There has been no prior reports of its association with autoimmune disease, more specifically, autoimmune hepatitis (AIH) or the development of pediatric acute liver failure (PALF). We present a case of an 8-month-old infant with TAR syndrome who presented with PALF, secondary to AIH with elevated liver-kidney microsomal antibody (>1:2560). She received a liver transplant and had a very complicated postoperative course including severe T-cell-mediated rejection, infection, biliary stricture, persistently elevated liver-kidney microsomal antibodies, and antibody-mediated rejection. Ultimately, these complications led to graft failure, severe sepsis, and death. This case highlights a new association of TAR syndrome with AIH and PALF and a potentially aggressive nature of AIH both pre- and post-transplant.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e325"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10049475","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}

引用次数: 0

Hypereosinophilic Syndrome with Pulmonary Involvement in Ulcerative Colitis. 溃疡性结肠炎伴肺部受累的嗜酸性粒细胞增多综合征。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000320

Anam Bashir, Jose M Cabrera, Mariko Suchi, Barry J Pelz

引用次数: 0

Delayed Hypersensitivity Reaction to Infliximab Due to Mammalian Meat Allergy. 哺乳动物肉类过敏致英夫利昔单抗迟发性超敏反应。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000322

Esthermari González Polanco, Stephen Borowitz

引用次数: 0

Resolution of Pruritus in a Child With Alagille Syndrome Treated With Maralixibat for Seven Years: Durable Response and Discontinuation of Other Medications. 用马拉利西他治疗7年的Alagille综合征患儿瘙痒的解决:持久的反应和停药。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000335

Amy Garcia, Evelyn Hsu, Henry C Lin

引用次数: 0

A Diagnostic Conundrum: A Case of Pediatric Autoimmune Pancreatitis. 诊断难题:儿童自身免疫性胰腺炎1例

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000326

Naseem Ravanbakhsh, Nick Shillingford, Travis L Piester

引用次数: 0

Food for Thought: Remission of Perianal Pediatric Crohn's Disease on Specific Carbohydrate Diet Monotherapy. 思考的食物:特定碳水化合物饮食单一疗法缓解小儿肛周克罗恩病。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000343

David Simon, Kalyani Patel, Prakash Masand, Richard Kellermayer

引用次数: 1

Pediatric Colonic Xanthomas, a Previously Unreported Colonoscopic Finding. 儿童结肠黄疸，以前未报道的结肠镜检查发现。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000329

Jon A Vanderhoof, Jeffrey D Goldsmith, Rosemary Pauley, Victor L Fox

引用次数: 0

Agenesis of the Dorsal Pancreas: Case Report and Review of Age-Related Differences in Presentation. 胰腺背面发育不全:病例报告和年龄相关表现差异的回顾。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000337

Callie A Grey, Ajinkya Desai, Michael J Nowicki, Natalie Bhesania

引用次数: 0

Crohn's Disease in a Patient With Bardet-Biedl Syndrome: Random Anomaly or Rare Phenotypic Trait? Bardet-Biedl综合征患者的克罗恩病:随机异常还是罕见的表型特征?

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000333

Margot L Zuidhof, Tim G J de Meij, Sietse Q Nagelkerke, Anne M Smets, Ilan J N Koppen

{"title":"Crohn's Disease in a Patient With Bardet-Biedl Syndrome: Random Anomaly or Rare Phenotypic Trait?","authors":"Margot L Zuidhof, Tim G J de Meij, Sietse Q Nagelkerke, Anne M Smets, Ilan J N Koppen","doi":"10.1097/PG9.0000000000000333","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000333","url":null,"abstract":"Bardet-Biedl syndrome (BBS) is an autosomal recessive multisystem nonmotile ciliopathy. There are anecdotal reports of the co-occurrence of BBS and autoimmune diseases, including inflammatory bowel disease (IBD). We present the first case report of a child with BBS7 who developed Crohn disease, adding to the evidence on the association between BBS and IBD. A 13-year-old girl with BBS7 presented with abdominal pain and significant weight loss (-13%), but without other classical symptoms of IBD, such as diarrhea and blood loss. Fecal calprotectin was elevated, but on gastroscopy and colonoscopy, no macroscopic abnormalities were found. Ultrasound and MRI revealed an intestinal stenosis which was treated surgically. Histopathological examination confirmed the diagnosis Crohn disease. In conclusion, the reported co-occurrence of BSS and autoimmune diseases and the atypical presentation of IBD in this patient warrant a low threshold to perform diagnostic tests for IBD in patients with BBS and gastrointestinal symptoms.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e333"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10051613","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}

引用次数: 0

Unexpected Etiology of Intussusception in an Adolescent Patient. 一例青少年患者肠套叠的意外病因。

JPGN Reports Pub Date : 2023-08-01 DOI: 10.1097/PG9.0000000000000342

Yasir Alshareefy, Ali Alshareefy

{"title":"Unexpected Etiology of Intussusception in an Adolescent Patient.","authors":"Yasir Alshareefy, Ali Alshareefy","doi":"10.1097/PG9.0000000000000342","DOIUrl":"https://doi.org/10.1097/PG9.0000000000000342","url":null,"abstract":"Intussusception is a common cause of acute and subacute small bowel obstruction in children, young, and older patients; however, despite increasing awareness of the condition and the number of patients diagnosed with it across all ages, its clinical and diagnostic approach remains challenging. A 17-year-old girl attended our gastroenterology outpatient department complaining of a 6-month history of recurrent right iliac fossa pain associated with nausea and vomiting at times with no past medical history of note. Initial blood tests revealed a slightly raised CRP (9.1 mg/L) and a significantly elevated fecal calprotectin (>1000 µg/g). Computed axial tomography scan of the abdomen and pelvis revealed ileocecal intussusception with no evidence of small or large bowel obstruction. On subsequent colonoscopy a 5-cm mass protruding through the ileocecal valve was identified and multiple biopsies were taken for histological analysis, which confirmed a diagnosis of Burkitt's lymphoma. The lesion was surgically resected and plans for adjuvant chemotherapy were discussed. The learning lessons to take from this case are to widen the list of differential diagnoses of unexplained recurrent abdominal pain to include intussusception and to actively rule it out with an appropriate diagnostic approach that addresses its potential malignant etiology across all ages.","PeriodicalId":17618,"journal":{"name":"JPGN Reports","volume":"4 3","pages":"e342"},"PeriodicalIF":0.0,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10046617","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}

引用次数: 0