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[Clinicopathological and molecular genetic features of Crohn's disease].
Gong, Y X; Chen, C N; Yang, Y F; Sun, S N; Shao, Y; Zhu, L Q; Shi, Y Q; Li, X; Han, X; Zhang, Z H.
Afiliación
  • Gong YX; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
  • Chen CN; Department of Pathology, the Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an 710004, China.
  • Yang YF; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
  • Sun SN; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
  • Shao Y; Nanjing Geneseeq Technology Company Limited, Nanjing 210000, China.
  • Zhu LQ; Nanjing Geneseeq Technology Company Limited, Nanjing 210000, China.
  • Shi YQ; Nanjing Geneseeq Technology Company Limited, Nanjing 210000, China.
  • Li X; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
  • Han X; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
  • Zhang ZH; Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
Zhonghua Bing Li Xue Za Zhi ; 53(4): 351-357, 2024 Apr 08.
Article en Zh | MEDLINE | ID: mdl-38556818
ABSTRACT

Objective:

To investigate the clinicopathological and molecular genetic characteristics of Crohn's disease (CD).

Methods:

A retrospective analysis was conducted on 52 CD patients who underwent surgical resection at the First Affiliated Hospital of Nanjing Medical University between January 2014 and June 2023. Clinical presentations and histopathological features were assessed. Whole-genome sequencing was performed on 17 of the samples, followed by sequencing and pathway enrichment analyses. Immunohistochemistry was used to assess the expression of frequently mutated genes.

Results:

Among the 52 patients, 34 were males and 18 were females, male-to-female ratio was 1.9∶1.0, with a median age of 45 years at surgery and 35 years at diagnosis. According to the Montreal classification, A3 (51.9%,27/52), B2 (61.5%, 32/52), and L3 (50.0%,26/52) subtypes were the most predominant. Abdominal pain and diarrhea were the common symptoms. Histopathological features seen in all 52 patients included transmural inflammation, disruption of cryptal architecture, lymphoplasmacytic infiltration, varying degrees of submucosal fibrosis and thickening, increased enteric nerve fibers and neuronal proliferation. Mucosal defects, fissure ulcers, abscesses, pseudopolyps, and adenomatous proliferation were also observed in 51 (98.1%), 38 (73.1%), 28 (53.8%), 45 (86.5%), and 28 (53.8%) cases, respectively. Thirty-one (59.6%) cases had non-caseating granulomas, and 3 (5.8%) cases had intestinal mucosal glandular epithelial dysplasia. Molecular analysis showed that 12/17 CD patients exhibited mutations in at least one mucin family gene (MUC2, MUC3A, MUC4, MUC6, MUC12, MUC17), and MUC4 was the most frequently mutated in 7/17 of cases. Immunohistochemical stains showed reduced MUC4 expression in epithelial cells, with increased MUC4 expression in the epithelial surface, particularly around areas of inflammatory cell aggregation; and minimal expression in the lower half of the epithelium.

Conclusions:

CD exhibits diverse clinical and pathological features, necessitating a comprehensive multidimensional analysis for diagnosis. Mutations and expression alterations in mucin family genes, particularly MUC4, may play crucial roles in the pathogenesis of CD.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Crohn Límite: Female / Humans / Male / Middle aged Idioma: Zh Revista: Zhonghua Bing Li Xue Za Zhi Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: China

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Crohn Límite: Female / Humans / Male / Middle aged Idioma: Zh Revista: Zhonghua Bing Li Xue Za Zhi Año: 2024 Tipo del documento: Article País de afiliación: China Pais de publicación: China