Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society最新文献

Histopathologic Features of Biliary Atresia and Outcome Predictors of Kasai Portoenterostomy: A 10-Year Retrospective Study of a Philippine Cohort. 胆道闭锁的组织病理学特征和开赛门肠造口术的预后预测因素：一项菲律宾队列的10年回顾性研究。

IF 1.3

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-31 DOI: 10.1177/10935266251359976

Pauline Mae Dy, Rachel Santos, Erick Martin Yturralde

{"title":"Histopathologic Features of Biliary Atresia and Outcome Predictors of Kasai Portoenterostomy: A 10-Year Retrospective Study of a Philippine Cohort.","authors":"Pauline Mae Dy, Rachel Santos, Erick Martin Yturralde","doi":"10.1177/10935266251359976","DOIUrl":"https://doi.org/10.1177/10935266251359976","url":null,"abstract":"Introduction: There is a dearth of information regarding the epidemiology of biliary atresia and Kasai portoenterostomy (KPE) outcomes in the Philippines. Here we describe the histopathologic features of biliary atresia and identify outcome predictors of KPE in a local cohort.Materials and methods: We performed a retrospective review of all KPEs done in our institution from 2013 to 2023, focusing on pertinent clinical and histologic features. Patients were categorized into having favorable or unfavorable outcomes based on a 3-month post-operative serum total bilirubin (≥2 mg/dL) or mortality.Results: Of the 71 patients who underwent KPE during this period, 41 had liver biopsies available for review. Fibrosis, ductular reaction, and portal tract cellular infiltrates were consistently present in all samples examined, with varying degrees of giant cell transformation, portal tract edema, and ductal plate malformation. An elevated AST to Platelet Ratio Index (APRI) was linked to poorer prognosis, while visible bile plugs in biopsies significantly correlated with unfavorable outcomes.Conclusions: This study demonstrated the extensive variability of clinical and histologic features in biliary atresia. Identifying significant laboratory and histologic predictors of liver survival is essential in management and prognostication, especially in resource-limited settings where liver transplantation is not readily available.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251359976"},"PeriodicalIF":1.3,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144755661","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

In Response to Dr. James R. Wright, Jr.'s Letter to the Editor. 回复小詹姆斯·r·赖特博士给编辑的信。

IF 1.3

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-30 DOI: 10.1177/10935266251357785

Elizabeth O Ferreira, Stefan Kostadinov, Camelia Stefanovici, Virginia Duncan

引用次数: 0

Comment on "Differences in Placental Pathologic Features by Trimester of Infection with SARS-CoV-2". 对“SARS-CoV-2感染不同妊娠期胎盘病理特征的差异”的评论

IF 1.3

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-29 DOI: 10.1177/10935266251357787

Hinpetch Daungsupawong, Viroj Wiwanitkit

引用次数: 0

H3F3A K36M-mutant Epithelioid Neoplasm: A Report of Two Novel Cases of a Non-chondrogenic H3K36-altered Mesenchymal Tumor. H3F3A k36m突变的上皮样肿瘤：两例非软骨性h3k36改变的间充质肿瘤报告

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-22 DOI: 10.1177/10935266251357098

Sze Jet Aw, Yingting Mok, Khurshid Merchant, Shiela Ramos, Robert Siddaway, Jian Yuan Goh, Amos Hong Pheng Loh, Kenneth Tou En Chang

{"title":"H3F3A K36M-mutant Epithelioid Neoplasm: A Report of Two Novel Cases of a Non-chondrogenic H3K36-altered Mesenchymal Tumor.","authors":"Sze Jet Aw, Yingting Mok, Khurshid Merchant, Shiela Ramos, Robert Siddaway, Jian Yuan Goh, Amos Hong Pheng Loh, Kenneth Tou En Chang","doi":"10.1177/10935266251357098","DOIUrl":"https://doi.org/10.1177/10935266251357098","url":null,"abstract":"Background: Identifying the genetic signatures in bone and soft tissue tumors enhances our understanding of tumor biology and aids in the subclassification of tumors for personalized treatment. Histone H3.3 alterations play a pivotal role in H3F3A/B K36M-mutant chondroblastomas and H3F3A G34W/L-mutant giant cell tumors of the bone.Methods and results: In this report, we describe 2 cases of a distinct epithelioid neoplasm with H3F3A K36M mutation but lacking features of chondroblastoma, which extends the spectrum of H3.3-mutant mesenchymal tumors. The 2 cases occurred in pediatric patients, had an aggressive clinical presentation, distinct epithelioid histomorphology with diffuse cytokeratin and TFE3 expression, and identical H3F3A K36M mutations. No gene fusions were identified. Methylation analysis using the DKFZ sarcoma classifier v12.3 pipeline did not classify these tumors with known entities, suggesting the existence of non-chondrogenic mesenchymal tumors within the H3.3-mutant tumor spectrum.Conclusions: The distinctive histological and molecular features of these 2 cases expand the spectrum of H3.3-mutant tumors and call for further investigation of the biological underpinnings of this group of tumors.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251357098"},"PeriodicalIF":0.0,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693127","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

"What the Mind Does Not Know, the Eye Does Not See": Epididymal Melanotic Neuroectodermal Tumor of Infancy Misdiagnosed as Paratesticular Rhabdomyosarcoma-A Rare Case Report. “心所不知，眼所不见”：婴幼儿附睾黑色素样神经外胚层肿瘤误诊为睾丸旁横纹肌肉瘤一例罕见报告。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-10 DOI: 10.1177/10935266251355755

Neha Bakshi, Seema Rao, Manas Kalra, Shashi Dhawan, Laksita Joshi, Sonia Badwal

引用次数: 0

Umbilical Vessel Aneurysm Presenting a Large Placental Cyst: A Unique Case and Literature Review. 脐血管动脉瘤并发大胎盘囊肿：一例独特病例并文献复习。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-07-02 DOI: 10.1177/10935266251352897

Veronique Schiffer, Ina Thijs, Salwan Al-Nasiry, Stijn van Teeffelen, Carmen Severens-Rijvers

{"title":"Umbilical Vessel Aneurysm Presenting a Large Placental Cyst: A Unique Case and Literature Review.","authors":"Veronique Schiffer, Ina Thijs, Salwan Al-Nasiry, Stijn van Teeffelen, Carmen Severens-Rijvers","doi":"10.1177/10935266251352897","DOIUrl":"https://doi.org/10.1177/10935266251352897","url":null,"abstract":"We present a unique case of a 33-year-old gravida that was referred to our hospital with an umbilical vessel aneurysm presenting as a large placental cyst on ultrasound. Although the 20-week anomaly scan showed no structural abnormalities, routine fetal biometry scanning at 30 weeks of gestation revealed an abnormal placental cystic structure, located subchorionic under the umbilical cord insertion. Given the uncertainty of the origin of the structure's origin and its unpredictable evolution with possible adverse effect on the fetus, a cesarean section was performed delivering a healthy baby. Histopathological examination of the placenta showed an aneurysmal vein with thinning of the vessel wall and fragmented smooth muscle. Umbilical cord aneurysm represents an exceptionally rare placental anomaly, with umbilical vein aneurysms being associated with variable fetal mortality rates, ranging from those observed in uncomplicated pregnancies to 82% in documented cases. Therefore, a multidisciplinary approach is essential to optimize fetal outcomes.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251352897"},"PeriodicalIF":0.0,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144546827","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

A Retrospective Review of Autopsy Data From 6 North American Institutions Indicates Practice Variations That Could Be Leveraged to Address Declining Autopsy Rates. 对6家北美机构尸检数据的回顾性分析表明，可以利用实践变化来解决尸检率下降的问题。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-06-27 DOI: 10.1177/10935266251346988

Anita Nagy, Colleen Collins, Linda J Szymanski, Bruce R Pawel, Portia A Kreiger, Tricia Bhatti, Murillo Olivia, Erin Rudzinski, Jeanette Reyes, Melissa Blessing, Kudakwashe Chikwava, Juan Putra, Chrystalle Katte Carreon

引用次数: 0

Childhood Lupus-Associated Protein-Losing Enteropathy (LUPLE): A Case Report and Review of the Literature. 儿童狼疮相关蛋白丢失性肠病（LUPLE）： 1例报告及文献回顾。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-06-20 DOI: 10.1177/10935266251349494

Ozlem Sumer Cosar, Hakan Öztürk, Deniz Gezgin Yıldırım, Batuhan Küçükali, Buket Dalgıç, Ödül Eğritaş Gürkan

{"title":"Childhood Lupus-Associated Protein-Losing Enteropathy (LUPLE): A Case Report and Review of the Literature.","authors":"Ozlem Sumer Cosar, Hakan Öztürk, Deniz Gezgin Yıldırım, Batuhan Küçükali, Buket Dalgıç, Ödül Eğritaş Gürkan","doi":"10.1177/10935266251349494","DOIUrl":"10.1177/10935266251349494","url":null,"abstract":"Protein-losing enteropathy (PLE) is a rare condition characterized by clinical findings such as edema, ascites, pleural effusion, and diarrhea due to excessive protein loss from the gastrointestinal system. Although systemic lupus erythematosus (SLE) is rare in childhood, PLE can be the first presenting feature; this condition is referred to as lupus-associated protein-losing enteropathy (LUPLE). Protein-losing enteropathy (PLE) is an uncommon condition resulting from excessive protein loss in the gastrointestinal system. Our case shows that PLE can be the initial presentation of SLE, which is a rare manifestation in childhood. PLE, a rare complication of lupus, tends to be more severe in children, and the diagnostic process can be challenging. This case report presents a 7-year-old girl who presented with abdominal distension, generalized edema, chronic diarrhea, and weakness. Despite treatment, the recurrence of symptoms and the addition of new joint findings led to further investigations, which revealed positive anti-dsDNA and low complement levels, resulting in a diagnosis of systemic lupus erythematosus. The patient's clinical condition improved with steroid, azathioprine, and hydroxychloroquine treatments. This case highlights the importance of considering SLE in the differential diagnosis of PLE and underscores the significance of recognizing the rare presentations of childhood lupus.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251349494"},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144334830","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

Neuropathologic Findings in Lennox-Gastaut Syndrome (SCN2A-Related): A Case Report and Review of Literature. lenox - gastaut综合征（scn2a相关）的神经病理表现：1例报告及文献复习。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-06-19 DOI: 10.1177/10935266251345684

Conner Thompson, Katsiaryna Khatskevich, Cynthia T Welsh, Tiffany G Baker, Daniel C Butler

{"title":"Neuropathologic Findings in Lennox-Gastaut Syndrome (SCN2A-Related): A Case Report and Review of Literature.","authors":"Conner Thompson, Katsiaryna Khatskevich, Cynthia T Welsh, Tiffany G Baker, Daniel C Butler","doi":"10.1177/10935266251345684","DOIUrl":"https://doi.org/10.1177/10935266251345684","url":null,"abstract":"Lennox-Gastaut syndrome is a form of severe childhood epilepsy caused by a variety of etiologies including structural abnormalities, inflammatory conditions affecting the brain, malignancy, injury, or unknown causes. Sodium channelopathies have been linked to multiple seizure disorders, including Lennox-Gastaut syndrome, due to their role in action potential propagation in the brain. SCN2A is one such voltage gated sodium channel found primarily in the central nervous system. We present a case of a 6-year-old female with Lennox-Gastaut syndrome and a heterozygous SCN2A variant who became unresponsive during a supervised bath and later died. The neuropathologic exam was remarkable for dentato-olivary dysplasia, among other gross and microscopic abnormalities. We present this case highlighting rarely documented neuropathologic findings in Lennox-Gastaut syndrome associated with a channelopathy (SCN2A variant) as well as a review of literature of previously reported brain abnormalities in patients with SCN2A variants.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251345684"},"PeriodicalIF":0.0,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328470","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

Umbilical Cord Weight Index Correlated with Fetal Birth Weight and Associated with Placental and Umbilical Cord Abnormalities. 脐带重量指数与胎儿出生体重相关，并与胎盘和脐带异常相关。

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Pub Date : 2025-06-17 DOI: 10.1177/10935266251349487

Peilin Zhang, Octavio Armas, Art Mendoza, Chris Wixom, Omid Bakhtar, Raymond Redline, Jenelle Coon, Lana Kabakibi

{"title":"Umbilical Cord Weight Index Correlated with Fetal Birth Weight and Associated with Placental and Umbilical Cord Abnormalities.","authors":"Peilin Zhang, Octavio Armas, Art Mendoza, Chris Wixom, Omid Bakhtar, Raymond Redline, Jenelle Coon, Lana Kabakibi","doi":"10.1177/10935266251349487","DOIUrl":"https://doi.org/10.1177/10935266251349487","url":null,"abstract":"Background: Umbilical cord consists of 2 arteries and 1 vein embedded in Wharton's jelly. Umbilical cord weight has not been examined for fetal growth and placental pathology.Materials and methods: We have assessed umbilical cord by measuring the weight and the length during placental examination. The cord weight index (CWI) was defined as an average weight of 10 cm cord, and the normal ranges of cord diameter and CWI were established as 10th to 90th percentile.Results: A total of 520 cases of umbilical cord were examined. Normal ranges of 10th to 90th percentile of cord diameter and CWI were established. Light cord was defined as less than 10th percentile and heavy cord was greater than 90th percentile of CWI. Light cord was significantly associated with decreased cord diameter, lower placental and fetal birth weight, female sex, nuchal cord, and IUGR, while heavy cord was significantly associated with increased cord diameter, increased BMI, male sex, diabetes mellitus, and decreased maternal and fetal inflammatory responses.Conclusion: Our data demonstrated that CWI is a novel and superior measure of cord development to diameter, and CWI can add value, providing preliminary evidence for more rigorous study and for incorporating CWI in placental examination.","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"10935266251349487"},"PeriodicalIF":0.0,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311207","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