{"title":"Expression of Calretinin in the Cecal Muscularis Interna: Observation and Hypothetical Relevance to Appendicitis.","authors":"Raj P Kapur","doi":"10.1177/10935266241235507","DOIUrl":"10.1177/10935266241235507","url":null,"abstract":"<p><strong>Background: </strong>The unexpected observation of calretinin immunoreactivity in smooth muscle cells in the muscularis propria of the cecum led to a more detailed examination of calretinin expression and its possible relationship to propulsive contractile activity around the vermiform appendix.</p><p><strong>Methods: </strong>Immunohistochemistry and RNA in situ hybridization were performed to analyze calretinin expression in intestinal samples from 33 patients at ages ranging from mid-gestation fetuses to adults, as well as in some potentially relevant animal models. Dual immunolabeling was done to compare calretinin localization with markers of smooth muscle and interstitial cells of Cajal.</p><p><strong>Results: </strong>Calretinin expression was observed consistently in the innermost smooth muscle layers of the muscularis interna in the human cecum, appendiceal base, and proximal ascending colon, but not elsewhere in the intestinal tract. Calretinin-positive smooth muscle cells did not co-express markers located in adjacent interstitial cells of Cajal. Muscular calretinin immunoreactivity was not detected in the ceca of mice or macaques, species which lack appendices, nor in the rabbit cecum or appendix.</p><p><strong>Conclusions: </strong>Localized expression of calretinin in cecal smooth muscle cells may reduce the likelihood of retrograde, calcium-mediated propulsive contractions from the proximal colon and suppress pro-inflammatory fecal stasis in the appendix.</p>","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":" ","pages":"241-254"},"PeriodicalIF":1.3,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140319932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrea C Bakker, Steven J Fishman, Marilyn G Liang, Alyaa Al-Ibraheemi, Harry P Kozakewich, John B Mulliken, Jonathan C Slack
{"title":"Immunohistochemical Expression of Lymphatic Endothelial Markers in Blue Rubber Bleb Nevus Syndrome.","authors":"Andrea C Bakker, Steven J Fishman, Marilyn G Liang, Alyaa Al-Ibraheemi, Harry P Kozakewich, John B Mulliken, Jonathan C Slack","doi":"10.1177/10935266241228930","DOIUrl":"10.1177/10935266241228930","url":null,"abstract":"<p><strong>Introduction: </strong>Blue rubber bleb nevus syndrome (BRBNS) is an uncommon vascular anomaly characterized by multifocal cutaneous, visceral, and other soft tissue or solid organ venous malformations. We observed that BRBNS lesions express immunohistochemical markers of lymphatic differentiation.</p><p><strong>Methods: </strong>BRBNS histopathologic specimens assessed at our institution during the past 27 years were reviewed. Slides from 19 BRBNS lesions were selected from 14 patients (9 cutaneous, 9 gastrointestinal, and 1 hepatic). We recorded the involved anatomical compartments and presence/absence of thrombi or vascular smooth muscle. Immunohistochemical endothelial expression of PROX1 (nuclear) and D2-40 (membranous/cytoplasmic) was evaluated semi-quantitatively.</p><p><strong>Results: </strong>Endothelial PROX1 immunopositivity was noted in all specimens; the majority (89.5%) demonstrated staining in more than 10% of cells. D2-40 immunopositivity was present in one-third (33%) of cutaneous lesions and only 1 gastrointestinal lesion.</p><p><strong>Conclusion: </strong>Endothelial cells in BRBNS almost always express 1 or more immunohistochemical markers of lymphatic differentiation.</p>","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":" ","pages":"228-234"},"PeriodicalIF":1.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140186326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rohit Josyabhatla, Mary Lauren Wood, Amber Gafur, Nina Tatevian, Amanda S Tchakarov, Syed Shahrukh Hashmi, Jon Marc Rhoads, Melissa Renee Van Arsdall
{"title":"Rising Prevalence of Mild Chronic Gastritis in Children: A Single Center Experience.","authors":"Rohit Josyabhatla, Mary Lauren Wood, Amber Gafur, Nina Tatevian, Amanda S Tchakarov, Syed Shahrukh Hashmi, Jon Marc Rhoads, Melissa Renee Van Arsdall","doi":"10.1177/10935266241238625","DOIUrl":"10.1177/10935266241238625","url":null,"abstract":"<p><strong>Objectives and methods: </strong>We analyzed upper endoscopic and histological findings in 3 cohorts of children undergoing upper gastrointestinal endoscopy over a 10-year period. Five hundred seventy-nine patients were identified, with 244 (42%), 199 (35%), and 136 (23%) in the 2011, 2015, and 2019 cohorts, respectively. The most common symptoms and signs were abdominal pain, vomiting, failure to thrive, and diarrhea.</p><p><strong>Results: </strong>The number of patients who had histological evidence of chronic gastritis increased from 2011 (n = 70, 29%) to 2015 (n = 106, 53%) and 2019 (n = 92, 68%; <i>P</i> < .001). The prevalence of \"normal\" endoscopic gastric findings was higher in controls (n = 247, 90%) compared to cases (n = 201, 76%; <i>P</i> < .001). There was a small but statistically significant difference in endoscopic esophageal grading (<i>P</i> = .008) over time, with lower grades being more prevalent in 2011 compared to 2015 (<i>P</i> = .026) and 2019 (<i>P</i> = .001). Crude comparisons of the predictors (sex, weight percentile, payor type, month of endoscopy, symptom duration, PPI exposure, and endoscopic stomach findings) yielded no difference between cases and controls.</p><p><strong>Conclusions: </strong>There has been a significant rise in the prevalence of mild chronic gastritis or non-specific gastritis over the last decade in our population.</p>","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":" ","pages":"235-240"},"PeriodicalIF":1.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11088214/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140319933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rebecca Green, Adeeb Ahmed, Ben Fleming, Anna-May Long, Sam Behjati, Jamie Trotman, Patrick Tarpey, James C Nicholson, Nicholas Coleman, C Elizabeth Hook, Matthew J Murray
{"title":"Wilms Tumor With Raised Serum Alpha-Fetoprotein: Highlighting the Need for Novel Circulating Biomarkers.","authors":"Rebecca Green, Adeeb Ahmed, Ben Fleming, Anna-May Long, Sam Behjati, Jamie Trotman, Patrick Tarpey, James C Nicholson, Nicholas Coleman, C Elizabeth Hook, Matthew J Murray","doi":"10.1177/10935266231213467","DOIUrl":"10.1177/10935266231213467","url":null,"abstract":"<p><p>Wilms tumor (WT) is the commonest cause of renal cancer in children. In Europe, a diagnosis is made for most cases on typical clinical and radiological findings, prior to pre-operative chemotherapy. Here, we describe a case of a young boy presenting with a large abdominal tumor, associated with raised serum alpha-fetoprotein (AFP) levels at diagnosis. Given the atypical features present, a biopsy was taken, and histology was consistent with WT, showing triphasic WT, with epithelial, stromal, and blastemal elements present, and positive WT1 and CD56 immunohistochemical staining. During pre-operative chemotherapy, serial serum AFP measurements showed further increases, despite a radiological response, before a subsequent fall to normal following nephrectomy. The resection specimen was comprised of ~55% and ~45% stromal and epithelial elements, respectively, with no anaplasia, but immunohistochemistry using AFP staining revealed positive mucinous intestinal epithelium, consistent with the serum AFP observations. The lack of correlation between tumor response and serum AFP levels in this case highlights a more general clinical unmet need to identify WT-specific circulating tumor markers.</p>","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":" ","pages":"260-265"},"PeriodicalIF":1.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11088205/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138812764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Addressing Chatbots as Artificial Intelligence Aids in Pediatric Pathology.","authors":"Casey Schukow, Van-Hung Nguyen","doi":"10.1177/10935266231212340","DOIUrl":"10.1177/10935266231212340","url":null,"abstract":"","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":" ","pages":"278-279"},"PeriodicalIF":1.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138048812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Renal Pathology of Ciliopathies","authors":"Thivya Sekar, Neil J. Sebire","doi":"10.1177/10935266241242173","DOIUrl":"https://doi.org/10.1177/10935266241242173","url":null,"abstract":"Renal ciliopathies are a group of genetic disorders that affect the function of the primary cilium in the kidney, as well as other organs. Since primary cilia are important for regulation of cell signaling pathways, ciliary dysfunction results in a range of clinical manifestations, including renal failure, cyst formation, and hypertension. We summarize the current understanding of the pathophysiological and pathological features of renal ciliopathies in childhood, including autosomal dominant and recessive polycystic kidney disease, nephronophthisis, and Bardet-Biedl syndrome, as well as skeletal dysplasia associated renal ciliopathies. The genetic basis of these disorders is now well-established in many cases, with mutations in a large number of cilia-related genes such as PKD1, PKD2, BBS, MKS, and NPHP being responsible for the majority of cases. Renal ciliopathies are broadly characterized by development of interstitial fibrosis and formation of multiple renal cysts which gradually enlarge and replace normal renal tissue, with each condition demonstrating subtle differences in the degree, location, and age-related development of cysts and fibrosis. Presentation varies from prenatal diagnosis of congenital multisystem syndromes to an asymptomatic childhood with development of complications in later adulthood and therefore clinicopathological correlation is important, including increasing use of targeted genetic testing or whole genome sequencing, allowing greater understanding of genetic pathophysiological mechanisms.","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":"23 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140591267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Michaela O’Neil, Sasha K. Demeulenaere, Phillip J. DeChristopher, Emily Holthaus, Walter Jeske, Loretto Glynn, Aliya Husain, Jonathan Muraskas
{"title":"Syndecan-1 Level, a Marker of Endothelial Glycocalyx Degradation, Is Associated With Fetal Exposure to Chorioamnionitis and Is a Potential Biomarker for Early-Onset Neonatal Sepsis","authors":"Michaela O’Neil, Sasha K. Demeulenaere, Phillip J. DeChristopher, Emily Holthaus, Walter Jeske, Loretto Glynn, Aliya Husain, Jonathan Muraskas","doi":"10.1177/10935266241235504","DOIUrl":"https://doi.org/10.1177/10935266241235504","url":null,"abstract":"The goal of this investigation was to identify the association between Syndecan-1 (S1) serum levels in preterm newborns exposed to chorioamnionitis (CA) in utero and the potential of S1 as a biomarker of early-onset neonatal sepsis. A cohort of preterm newborns born <33 weeks gestational age was recruited. Within 48 hours of birth, 0.5 mL of blood was drawn to obtain S1 levels, measured via ELISA. Placentas were examined and classified as having (1) no CA, (2) CA without umbilical cord involvement, or (3) CA with inflammation of the umbilical cord (funisitis). S1 levels were compared between preterm newborns without exposure to CA verus newborns with exposure to CA (including with and without funisitis). Preterm newborns exposed to CA were found to have significantly elevated S1 levels compared to those unexposed. Although S1 levels could not differentiate fetal exposure to CA from exposure to CA with funisitis, the combined CA groups had significantly higher S1 levels compared to those not exposed to CA. S1 level has the potential to become a clinically useful biomarker that could assist in the management of mothers and preterm newborns with CA and funisitis. Furthermore, S1 level could aid in the diagnosis and treatment of early-onset neonatal sepsis.","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":"5 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140591842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ananda van der Kamp, Tomas J. Waterlander, Thomas de Bel, Jeroen van der Laak, Marry M. van den Heuvel-Eibrink, Annelies M. C. Mavinkurve-Groothuis, Ronald R. de Krijger
{"title":"Reply to: “Addressing Chatbots as Artificial Intelligence Aids in Pediatric Pathology”","authors":"Ananda van der Kamp, Tomas J. Waterlander, Thomas de Bel, Jeroen van der Laak, Marry M. van den Heuvel-Eibrink, Annelies M. C. Mavinkurve-Groothuis, Ronald R. de Krijger","doi":"10.1177/10935266241237904","DOIUrl":"https://doi.org/10.1177/10935266241237904","url":null,"abstract":"","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":"8 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140591265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Stillbirth Associated With Anomalous Origin and Course of the Left Coronary Artery: A Report of 2 Cases","authors":"Erica Price, Kristen M. Thomas, Linda M. Ernst","doi":"10.1177/10935266231223278","DOIUrl":"https://doi.org/10.1177/10935266231223278","url":null,"abstract":"Coronary artery anomalies and their potential sequelae are not well studied in association with stillbirth. Herein, we report the autopsy findings in two term stillborn fetuses with coronary artery anomalies. Both fetuses showed identical findings consisting of an abnormal origin of the left coronary artery from the right sinus of Valsalva and an interarterial course of the left coronary artery. Histologic vascular and myocardial changes were also present. These coronary artery findings are associated with sudden death in adults and neonates, and therefore, their potential to be a cause and/or contributor to fetal death is suspected.","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":"53 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140591372","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Frida Waldheim, Meeli Sirotkina, Karin Pettersson, Marius Kublickas, Nikos Papadogiannakis
{"title":"Reference Values for Placental Weight and Placental:Fetal Weight Ratio in a Swedish Population","authors":"Frida Waldheim, Meeli Sirotkina, Karin Pettersson, Marius Kublickas, Nikos Papadogiannakis","doi":"10.1177/10935266241239505","DOIUrl":"https://doi.org/10.1177/10935266241239505","url":null,"abstract":"Introduction:There is important clinical information from placental weight and its ratio to the fetal weight. The aim with this study was to establish reference values for the placental weight and the placental:fetal weight ratio for gestational weeks 13–43 in a Swedish population.Materials and Methods:Cases were retrospectively collected from the database used at the Pathology Department at Karolinska University Hospital and information about the placental weight, fetal weight, and gestational age was retrieved. Conditions, which could affect the placental- or fetal weight were excluded. Thereafter percentile curves were calculated for the placental weight and the placental:fetal weight ratio for gestational weeks.Results:A total of 730 cases were included and percentile curves for the placental weight for gestational week 13–43 and placental:fetal weight ratio for gestational week 18–43 are presented.Conclusions:Reference values for post fixation placental weight and its ratio to fetal weight for a Swedish population are presented. The reference values are lower than the current reference values used in our institution, and this will be of importance when interpreting findings after placental examination.","PeriodicalId":54634,"journal":{"name":"Pediatric and Developmental Pathology","volume":"107 1","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140591370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}