{"title":"The Impact of the \"Newborn Gang\" Scandal on Türkiye's Neonatal Healthcare Community.","authors":"Didem Yüksel","doi":"10.1080/15513815.2025.2469584","DOIUrl":"https://doi.org/10.1080/15513815.2025.2469584","url":null,"abstract":"","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-3"},"PeriodicalIF":0.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143532080","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}
Juan Cao, Yongxian Chen, Guocheng Yang, Xiaoxiao He, Hailun Chen, Zaoling Luo, Jingting Liao, Zhihui Huang, Weiguo Cao
{"title":"Clinicopathological Features of Diffuse Hemispheric Glioma, H3G34-Mutant.","authors":"Juan Cao, Yongxian Chen, Guocheng Yang, Xiaoxiao He, Hailun Chen, Zaoling Luo, Jingting Liao, Zhihui Huang, Weiguo Cao","doi":"10.1080/15513815.2025.2460560","DOIUrl":"https://doi.org/10.1080/15513815.2025.2460560","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the clinical manifestations, radiographic features, and pathological characteristics of three cases of diffuse hemispheric gliomas, H3G34-mutant (H3G34 DHG).</p><p><strong>Methods: </strong>This study used a single-center retrospective cohort approach to analyze 45 pediatric-type diffuse high-grade glioma cases.</p><p><strong>Results: </strong>Histologically, case 1 and case 2 had glioblastoma structures, and case 3 had primitive neuroectodermal tumor (PNET) morphology with ganglion cell differentiation. Immunohistochemical staining revealed diffuse expression of H3G34R and P53, but no expression of Olig2, ATRX, IDH1/2 and BRAF V600E in tumor cells in all three cases.</p><p><strong>Conclusions: </strong>H3 G34 DHG occurs more significantly in younger patients, and nearly all lesions are located in the cerebral hemisphere. MRI showed mass effects, edema and mild enhancement. The histological type showed glioblastoma structure and PNET morphology. Immunohistochemistry showed that the expression of H3G34R was more significant in PNET morphology.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-9"},"PeriodicalIF":0.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143532077","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":"Opening of the Mitochondrial Permeability Transition Pore Mediated Myocardial Damage After Perinatal Asphyxia in Neonatal Rats.","authors":"Zhixin Chen, Jianqin Chen, Yongheng Chen, Xiaoyi Fang","doi":"10.1080/15513815.2025.2466804","DOIUrl":"https://doi.org/10.1080/15513815.2025.2466804","url":null,"abstract":"<p><strong>Objectives: </strong>This study investigated the mechanisms underlying myocardial damage after perinatal hypoxia.</p><p><strong>Methods: </strong>An intrauterine hypoxia-ischemia model (I/U HI) and a hypoxia/reoxygenation (H/R) model were established. Myocardial damage, mitochondrial function, and mitochondria permeability transition pore (MPTP) opening were determined. The results, presented as means ± SD, were analyzed using SPSS.</p><p><strong>Results: </strong>Intrauterine hypoxia induced cardiac damage, mitochondrial dysfunction, and MPTP opening in neonatal rats. H/R led to apoptosis and MPTP opening. cTnI and apoptosis-inducing factor (AIF) levels were positively correlated with the degree of MPTP opening. The larger degree of MPTP opening combined with the significant increases in the Ca<sup>2+</sup>, ROS, and decreases in mitochondrial membrane potential and ATP levels. The larger degree of MPTP opening combined with the stronger release of cytochrome c and AIF.</p><p><strong>Conclusions: </strong>Increased MPTP opening may play a crucial role in perinatal asphyxia-induced myocardial damage in neonatal rats.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-19"},"PeriodicalIF":0.7,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484424","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}
Aileen Azari-Yam, Mohammad Vasei, Moeinadin Safavi
{"title":"Multinucleated Giants: Unveiling Pediatric Renal Epithelioid PEComa.","authors":"Aileen Azari-Yam, Mohammad Vasei, Moeinadin Safavi","doi":"10.1080/15513815.2025.2458677","DOIUrl":"https://doi.org/10.1080/15513815.2025.2458677","url":null,"abstract":"<p><p>Perivascular epithelioid cell tumors (PEComas) of the kidney are rare mesenchymal tumors that rarely occur in children. Individuals with tuberous sclerosis (TS) are at increased risk for these tumors. While classic PEComas are benign, the epithelioid variant could have malignant potential and metastasis capacity. We report a case of right kidney epithelioid PEComa with uncertain malignant potential in a 3-year-old girl who had no personal or family history of tuberous sclerosis. The tumor was found through ultrasound imaging. Sections from the radical nephrectomy specimen showed epithelioid and spindle cells with abundant granular eosinophilic or clear cytoplasm, mildly pleomorphic vesicular nuclei and distinctive perivascular arrangement. Abundant multinucleated giant cells were seen. No further therapy was suggested by the oncologist. The patient is doing well ten months post-surgery. We reviewed the literature and analyzed the features of pediatric renal PEComas reported so far.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-10"},"PeriodicalIF":0.7,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143473182","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":"Two GNPTAB Variations Caused Mucolipidosis II Alpha/Beta in a Chinese Family.","authors":"Jingxin Yang, Chao Liu, Qian Geng, Liyuan Chen, Lei Zhang, Weiqing Wu","doi":"10.1080/15513815.2025.2466057","DOIUrl":"https://doi.org/10.1080/15513815.2025.2466057","url":null,"abstract":"<p><p><b>Introduction:</b> Mucolipidosis II alpha/beta (ML II) is an autosomal recessive disorder with craniofacial dysmorphism and bone deformities. The variants in <i>GNPTAB</i> are associated with ML II. <b>Materials and Methods:</b> A female pediatric patient presented with bone deformities, mental and motor developmental abnormalities and craniofacial dysmorphism. We performed clinical whole-exome sequencing (WES) and verified the variants <i>via</i> qPCR, gap-PCR and Sanger sequencing. <b>Results:</b> Clinical WES identified a point variant c.1090C > T (p.R364*) and a copy number variation (CNV) in <i>GNPTAB</i>. Compared with normal control, <i>GNPTAB</i> expression was reduced in blood of the proband. Using Gap-PCR and Sanger sequencing, we identified the break point of CNV (NC_000012.11:g.102136912_102142973del), and successfully performed prenatal diagnosis for the proband's mother. <b>Conclusion:</b> To our knowledge, this is the first report of this novel CNV associated with ML II. Our findings expand the genotypes related to ML II and contribute to the gene diagnosis of ML II.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-9"},"PeriodicalIF":0.7,"publicationDate":"2025-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143434309","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":"Early- and Late-Onset Intrahepatic Cholestasis of Pregnancy: A Comparison of Maternal and Neonatal Outcomes.","authors":"Meryem Hocaoglu, Ozgul Bulut, Irem Unal, Gokcem Inanc Karaman, Dilan Unsal Kaya, Abdulkadir Turgut","doi":"10.1080/15513815.2025.2463983","DOIUrl":"https://doi.org/10.1080/15513815.2025.2463983","url":null,"abstract":"<p><strong>Introduction: </strong>We investigated the maternal and neonatal outcomes of early- and late-onset intrahepatic cholestasis of pregnancy (ICP).</p><p><strong>Methods: </strong>A total of 198 pregnant women were recruited into this retrospective cohort study. Women with ICP (<i>n</i> = 84) were classified into two groups: (1) Early-onset ICP (<i>n</i> = 36): pregnancy duration at diagnosis <34 weeks; (2) Late-onset ICP (<i>n</i> = 48): pregnancy duration at diagnosis ≥34 weeks. Maternal and neonatal outcomes were compared among the three groups.</p><p><strong>Results: </strong>The assisted reproductive technology (ART) pregnancy rate and serum bile acid (SBA) levels were significantly higher in the early-onset ICP group with adverse perinatal outcome (APO) than those without. Notably, the birth weight was significantly lower among neonates in the early-onset group of ICP than among neonates in the late-onset ICP and control groups (<i>p</i> < 0.001). Birth weight (OR = 0.998, 95% CI: 0.997-0.999, <i>p</i> = 0.041)was associated with early-onset ICP, according to the multivariate analysis. Receiver-operating characteristic (ROC) analysis revealed that a cutoff value of 36.8 weeks for gestational age at diagnosis and 9.6 mmol/L for SBA can distinguish between ICP patients with APO and those without.</p><p><strong>Discussion: </strong>Early-onset ICP is associated with low birth weight. ART pregnancies and women with higher SBA concentrations needed to be closely monitor for possible adverse perinatal outcomes in early-onset ICP.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-17"},"PeriodicalIF":0.7,"publicationDate":"2025-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143392373","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}
P Sivathangam, D Srinivasamurthy, Vani Krishnamurthy
{"title":"Efficacy of Frozen Section Technique as a Rapid Diagnostic Tool in the Diagnosis of Funisitis in Premature Deliveries.","authors":"P Sivathangam, D Srinivasamurthy, Vani Krishnamurthy","doi":"10.1080/15513815.2025.2457617","DOIUrl":"https://doi.org/10.1080/15513815.2025.2457617","url":null,"abstract":"<p><strong>Introduction: </strong>Presence of leucocytic infiltration in perivascular area or in Wharton jelly indicates funisitis. While conventional histopathological examination is the gold standard, its time delay hampers timely intervention. The frozen section technique offers a rapid alternative, enabling clinicians to promptly manage preterm early onset sepsis.</p><p><strong>Methods: </strong>This is a prospective study of 18 months. 125 preterms analyzed. Frozen sections and conventional sections of umbilical cord prepared and examined for inflammation by two researchers. Diagnostic accuracy of frozen sections versus conventional sections evaluated for sensitivity and specificity in detecting funisitis.</p><p><strong>Results: </strong>The frozen section of the umbilical cord was 85.5% sensitive and 86.7% specific in diagnosing funisitis. The inter-rater agreement for diagnosing funisitis was 72.2%. Funisitis on frozen section was 82.2% sensitive and 26.3% specific in diagnosing neonatal sepsis.</p><p><strong>Conclusion: </strong>Frozen section examination of umbilical cord has good diagnostic ability in detecting funisitis. The inter-rater agreement is substantial.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-8"},"PeriodicalIF":0.7,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143371173","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}
Mai He, Kayla Hoerschgen, Amy E Armstrong, Lili Zhang, Patrick Dillon, Frances White, Louis P Dehner
{"title":"Yolk Sac Tumor of the Liver: An Important Differential for Liver Mass with Elevated Serum Alpha-Fetoprotein in the Pediatric Population.","authors":"Mai He, Kayla Hoerschgen, Amy E Armstrong, Lili Zhang, Patrick Dillon, Frances White, Louis P Dehner","doi":"10.1080/15513815.2025.2457616","DOIUrl":"https://doi.org/10.1080/15513815.2025.2457616","url":null,"abstract":"<p><p><b>Introduction:</b> Yolk sac tumor (YST) is a malignant germ cell tumor with 10-15% arising in extragonadal sites. <b>Methods:</b> A search through our institution's database from January 01, 1990, to December 31, 2020, for \"yolk sac tumor\" or \"endodermal sinus tumor\" and \"liver\". <b>Results:</b> Our search yielded three cases. A 20-month-old girl with a liver mass and serum alpha-fetoprotein (AFP) level of 46558.0 ng/mL. The neoplasm was papillary with Schiller-Duval bodies. A 2-year-old boy with hepatic masses and adrenal mass with a serum AFP of 106,604.5 ng/mL. Numerous Schiller-Duval bodies were present. A 7-month-old girl with a liver mass, lung nodules, and retroperitoneal masses. Serum AFP was in the 800s ng/mL. Hepatoid and microcystic YST were mixed with hepatoblastoma (HBL). All three cases were positive for CAM5.2, SALL4, Glypican-3, beta-catenin, and AFP. <b>Conclusion:</b> Hepatic yolk sac tumor should be considered in the differential of a liver mass in pediatric patients with elevated AFP.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-8"},"PeriodicalIF":0.7,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143191148","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":"Testicular Torsion in a 14-Year-Old with Sertoli Cell Granular Cell Change and Sertoli Nodules.","authors":"Randall Craver","doi":"10.1080/15513815.2025.2458665","DOIUrl":"https://doi.org/10.1080/15513815.2025.2458665","url":null,"abstract":"<p><p><b>Introduction:</b> Sertoli eosinophilic granular change and Sertoli cell nodules are incidental findings. This details focal Sertoli eosinophilic granular and Sertoli cell only changes coincident with Sertoli cell nodules in a pubertal testis with acute torsion and bell clapper deformity. <b>Case Report:</b> A 14-year-old with bell clapper deformity underwent orchiectomy for torsion. There was acute interstitial hemorrhage. Seminiferous tubules contained only Sertoli cells in ∼10%, 2-5% of tubules demonstrated eosinophilic granular cell changes. There were several Sertoli cell nodules. <b>Discussion:</b> Sertoli cell nodules and eosinophilic granular change have not been described together. The focality of Sertoli cell only changes is unusual. The combination of focal Sertoli cell only and eosinophilic granular cell changes with Sertoli nodules in a testis with bell clapper abnormality may reflect an abnormal testicular environment, either physical and/or molecularly. This combination raises concerns for future fertility if seminiferous tubular abnormalities exist and progress in the remaining testis.</p>","PeriodicalId":50452,"journal":{"name":"Fetal and Pediatric Pathology","volume":" ","pages":"1-6"},"PeriodicalIF":0.7,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143060989","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}