JPGN reports最新文献_第6页

Achalasia in Klinefelter syndrome: A suspected pediatric case as well as prevalence analysis suggesting increased risk in this population 克莱恩费尔特综合征中的 Achalasia：一例疑似儿科病例以及患病率分析表明该人群患病风险增加

JPGN reports Pub Date : 2024-05-22 DOI: 10.1002/jpr3.12084

Lacey Miller, Hyung‐Gyo Cho, Charlotte Banayan, Vivian Vega Lemus, Shagun Sharma, Thomas Wallach

引用次数: 0

Umbilical arterial catheter duration as risk factor for Bell's Stage III necrotizing enterocolitis in preterm neonates 脐动脉导管持续时间是早产新生儿发生贝尔氏 III 期坏死性小肠结肠炎的风险因素

JPGN reports Pub Date : 2024-05-20 DOI: 10.1002/jpr3.12081

R. Lalitha, Matthew Hicks, Mosarrat Qureshi, Kumar Kumaran

{"title":"Umbilical arterial catheter duration as risk factor for Bell's Stage III necrotizing enterocolitis in preterm neonates","authors":"R. Lalitha, Matthew Hicks, Mosarrat Qureshi, Kumar Kumaran","doi":"10.1002/jpr3.12081","DOIUrl":"https://doi.org/10.1002/jpr3.12081","url":null,"abstract":"To explore risk factors for Stage‐III necrotizing enterocolitis (NEC‐III) in preterm neonates.This was a retrospective case‐control study of neonates born <33 weeks gestational age (GA) who were admitted to a tertiary neonatal intensive care unit, between 2015 and 2018. NEC‐III cases were compared with Stage‐II NEC (NEC‐II) and non‐NEC controls. Two to four non‐NEC controls were matched by GA ± 1 week and date of birth ± 3 months, to one NEC‐III case. Univariate and multivariate analyses were used to examine risk factors for NEC‐III.Of 1360 neonates born <33 weeks, 71 (5.2%) had NEC‐II and above, with 46% being NEC‐III. Mean age of onset of NEC‐III was 13.7 days versus 23.9 days for NEC‐II (p = 0.01). Neonates with NEC‐III were of lower GA (NEC‐III 25.4 weeks, NEC‐II 27.3 weeks, and non‐NEC 26 weeks; p = 0.0008) and had higher Score for Neonatal Acute Physiology Perinatal Extension‐II scores (NEC‐III 47.5, NEC‐II 28.4 and non‐NEC 37, p = 0.003). Multivariate analysis showed duration of umbilical arterial catheter (UAC) >5 days was significantly associated with the development of NEC‐III with adjusted odds ratio (AOR) 3.8; 95% confidence interval (CI) (1.05–13.66) for NEC‐III versus non‐NEC and AOR 5.57; 95% CI (1.65–18.73), p = 0.006 for NEC‐III versus NEC‐II. Rupture of membranes (ROM) >1 week was associated with NEC‐III (AOR 6.93; 95% CI [1.56–30.69] vs. non‐NEC and AOR 11.74; 95% CI [1.14–120.34] vs. NEC‐II).The increased association of NEC‐III with duration of UAC and ROM could be further examined in prospective studies, and an upper limit for UAC duration could be considered in NEC prevention bundles.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"74 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141121272","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}

引用次数: 0

Autoimmune hepatitis presenting as severe anemia. 表现为严重贫血的自身免疫性肝炎。

JPGN reports Pub Date : 2024-04-29 eCollection Date: 2024-08-01 DOI: 10.1002/jpr3.12076

Brandon J Calley, Alexandra Polovneff, Kathryn Henry, Paula North, David C Moe, Cara L Mack

{"title":"Autoimmune hepatitis presenting as severe anemia.","authors":"Brandon J Calley, Alexandra Polovneff, Kathryn Henry, Paula North, David C Moe, Cara L Mack","doi":"10.1002/jpr3.12076","DOIUrl":"10.1002/jpr3.12076","url":null,"abstract":"Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"5 3","pages":"402-406"},"PeriodicalIF":0.0,"publicationDate":"2024-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11322014/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141989882","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

Considerable differences in management of cytomegalovirus infection in patients with biliary atresia. 对胆道闭锁患者巨细胞病毒感染的管理存在巨大差异。

JPGN reports Pub Date : 2024-04-04 eCollection Date: 2024-08-01 DOI: 10.1002/jpr3.12068

Ulrika Liliemark, Afrodite Psaros Einberg, Jan F Svensson, Björn Fischler

{"title":"Considerable differences in management of cytomegalovirus infection in patients with biliary atresia.","authors":"Ulrika Liliemark, Afrodite Psaros Einberg, Jan F Svensson, Björn Fischler","doi":"10.1002/jpr3.12068","DOIUrl":"10.1002/jpr3.12068","url":null,"abstract":"Objectives: Patients with biliary atresia (BA) and ongoing cytomegalovirus (CMV) infection may have poorer outcomes after Kasai portoenterostomy than uninfected patients. Still, there is no consensus on the usefulness of viral testing and antiviral treatment (AVT). This study aims to explore the need for future research on AVT for CMV infection by assessing how CMV infection in BA patients is managed in different centers.Methods: An online questionnaire with 10 questions was offered to participants at an international congress on BA, organized in collaboration with the European Reference Network for rare liver diseases in 2022. Answers to questions were either dichotomic or multiple choices of different numeric intervals. Ongoing CMV infection was defined by detecting cytomegalovirus-immunoglobulin M (CMV-IgM) in serum or cytomegalovirus-deoxyribonucleic acid (CMV-DNA) by polymerase chain reaction in blood or urine.Results: There were 43 respondents from 36 centers in 26 countries. The total number of BA patients per year was between 208 and 380 from centers with 0-5 to >20 BA patients yearly (median 6-10). CMV infection was tested in 27 centers (75%), of which 18 (67%) use AVT. The rate of CMV infection varied between 0%-5% and 40%-50% (median 5%-10%). Willingness to treat the infection did not differ between centers with low and high rates of CMV infection.Conclusions: Most centers test for CMV infection, and a considerable proportion use AVT despite the lack of evidence of its benefits. A future randomized study on treating CMV infection in BA patients is necessary and feasible.","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"5 3","pages":"303-308"},"PeriodicalIF":0.0,"publicationDate":"2024-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11322013/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141989884","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

Visceral leishmaniasis as a rare cause of granulomatous hepatitis 作为肉芽肿性肝炎罕见病因的内脏利什曼病

JPGN reports Pub Date : 2024-03-26 DOI: 10.1002/jpr3.12059

Dalal Ben Sabbahia, Meriem Atrassi, Halima Msaaf, Imane Chahid, A. Khoaja, Nissrine Bennani, Mehdi Karkouri, Abdelhak Abkari

引用次数: 0

Letter to the editor in response to distinguishing autoimmune hepatitis from steatohepatitis in adolescents with obesity and positive screening alanine aminotransferase, appearing in JPGN rep 2023 feb 28;4(2):E292 致编辑的信，回应《区分肥胖和丙氨酸氨基转移酶筛查阳性青少年的自身免疫性肝炎和脂肪性肝炎》，刊登于《JPGN Rep 2023 feb 28;4(2):E292》。

JPGN reports Pub Date : 2024-02-28 DOI: 10.1002/jpr3.12050

C. Mandato, Angelo Colucci, Pietro Vajro

引用次数: 0

Resolution of metabolic dysfunction‐associated steatohepatitis with estradiol in a transgender female: A case report 变性女性服用雌二醇后，代谢功能障碍相关性脂肪性肝炎得到缓解：病例报告

JPGN reports Pub Date : 2024-02-26 DOI: 10.1002/jpr3.12054

Athreya Steiger, Nathan T. VanderVeen, Esther H. Kang, Amy K. Weimer