Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myelodysplastic syndromes (MDS) are classified by the WHO as myeloid neoplasms, and are characterized by cytopenia and dysplasia in one or more myeloid cell lines. Recently, a flow cytometric score (FCM-score) was published capable of discriminating low-grade MDS from non-clonal cytopenias (Della Porta et al., 2012). We tested the applicability of the FCM-score in a patient population from a large peripheral teaching hospital in The Netherlands. The evaluation of the proposed FCM score in low-grade MDS showed a high sensitivity and specificity, and clinically significant positive and negative likelihood ratios. The use of CD10 and CD19 positivity to identify progenitor B-cell blasts provided a specific and precize method to separate progenitor B-cell blasts from myeloid blasts within the CD34+/low CD45+ population and may be more convenient compared to the published method using low SSC and CD45 expression. This study confirms the value of utilizing the FCM-score in our patient population.
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PMID:Translating the MDS flow cytometric score into clinical practice. 2549 Sep 72

Tufting enteropathy (TE), previously known as intestinal epithelial dysplasia, is a rare congenital enteropathy characterized by refractory diarrhea in the neonatal period. It presents clinical and histological heterogeneity and may be associated with birth defects and punctuate keratitis. The causative gene(s) have not yet been identfied making prenatal diagnosis unavailable. Although there are milder phenotypes most require parenteral nutrition for prolonged periods with the risk of complications. TE becomes an indication for intestinal transplantation. We report the case of a 4-month-old male, born full term with a normal weight. The parents consulted because of severe malnutrition and chronic watery diarrhea. Duodenal and rectal biopsy was negative. Because of poor tolerance gastroclysis was changed to parenteral nutrition. The infant had several catheter-related infections and died at 13 months from catheter-associated complications. Histopathological autopsy was performed. The material was fixed in paraffin and studied with routine techniques. PAS and immunohistochemistry for CD10 were performed. We observed villous atrophy with intestinal epithelial dysplasia and disorganization on the surface of epithelial cells resembling tufts in jejunal and ileal tissue. The objective of this study was to present a rare case of neonatal enteropathy, especially TE, describe the methodology used to study the biopsy, and discuss the differential diagnoses. TE is a rare neonatal enteropathy that is difficult to diagnose and manage. Children in whom TE is suspected should be referred to specialized pediatric centers, with the option of intestinal transplantation.
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PMID:[Tufting enteropathy: a case report, histopathological methodology, and differential diagnoses]. 2607 17

The present study aimed to identify the pathologic and genetic characteristics of intestinal subtype of intraductal papillary neoplasm of the bile duct (iIPNB) showing columnar cells with pseudostratified, cigar-shaped nuclei, and basophilic or amphophilic cytoplasm with the diffuse immunohistochemical expression of CK20 and/or CDX2. A total of 34 cases of iIPNB were pathologically examined according to their anatomic location (the bile duct) and were then compared with the intestinal subtype of intraductal papillary mucinous neoplasm (iIPMN) of the pancreas (n=22). Mutations of 26 somatic genes were examined in formalin-fixed paraffin-embedded tissue specimens from 21 cases of iIPNB using the TruSight Tumor 26 gene panel and next-generation sequencing. iIPNB cases were divided into intrahepatic (n=6) and extrahepatic (n=28) categories. Intrahepatic IPNBs showed a less-complicated villous-papillary pattern, while extrahepatic IPNBs showed a papillary pattern with tubular and/or villous components and predominant high-grade dysplasia with complicated architectures. MUC5AC was frequently and extensively expressed in intrahepatic iIPNBs and iIPMNs but not in extrahepatic iIPNBs. CD10 was frequently expressed in extrahepatic IPNBs but not in intrahepatic iIPNBs or iIPMN. Genetic mutations of TP53 and PIK3CA, which were infrequent or absent in iIPMNs, were frequently detected in extrahepatic iIPNBs, while KRAS and GNAS, which were commonly observed in iIPMNs, were frequently detected in intrahepatic iIPNBs. Intrahepatic iIPNBs showed villous-papillary growth with features reminiscent of iIPMNs, while extrahepatic iIPNBs showed papillary growth with tubular and/or villous components, complicated histology and variable differences from iIPMNs, suggesting differences in the tumorigenesis of iIPNBs along the biliary tree.
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PMID:The Pathologic and Genetic Characteristics of the Intestinal Subtype of Intraductal Papillary Neoplasms of the Bile Duct. 3116 2

Colitis-associated cancer (CAC) is a subtype of colon cancer that is driven by chronic inflammation and is prevalent in chronic ulcerative colitis patients. The development of CAC is associated with the inflammation-dysplasia-carcinoma pathway which is significantly different than adenoma-carcinoma pathway of sporadic colon cancer (CRC). Matrix Metalloproteinase 9 (MMP9) is a zinc-dependent endopeptidase against extracellular matrix (ECM) proteins expressed in the gastrointestinal tract during inflammation. We have previously shown that MMP9 plays a tumor suppressor role in CAC via "MMP9-Notch1-ARF-p53 axis" pathway. The aim of this study is to determine the role of MMP9 in maintaining genomic stability in CAC. Homozygous transgenic mice with constitutive-expression of MMP9 in the colonic epithelium (TgM9) with their wild-type littermates (WT) and stably transfected HCT116 cells with/without MMP9 were used for in vivo and in vitro experiments, respectively. As 'proof of concept' model, nanoparticles (NPs) loaded with MMP9 siRNA were used to examine the effect of MMP9 silencing in the colonic epithelium. In CAC, colonic epithelium of TgM9 mice exhibited lower amounts of reactive oxygen species (ROS), less DNA damage, and increased expression of mismatch repair genes compared to WTs. Our study showed that MMP9 expression correlates with the reduced ROS levels, decreased DNA damage, and upregulated mismatch repair pathway. This suggests that MMP9 expression is a natural biological way to suppress CAC by limiting ROS accumulation and DNA damage in the colon. Therefore, MMP9 inhibition could be deleterious for CAC patient.
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PMID:Matrix metalloproteinase 9 (MMP9) limits reactive oxygen species (ROS) accumulation and DNA damage in colitis-associated cancer. 3294 3


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