Amianthoid transformation of costal cartilage matrix in children with pectus excavatum and pectus carinatum. 2021

Alexandr Kurkov, and Anna Guller, and Alexey Fayzullin, and Nafisa Fayzullinа, and Vladimir Plyakin, and Svetlana Kotova, and Petr Timashev, and Anastasia Frolova, and Nikita Kurtak, and Vyacheslav Paukov, and Anatoly Shekhter
Institute for Regenerative Medicine, Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia.

It is unclear if amianthoid transformation (AT) of costal cartilage extracellular matrix (ECM) has an impact on the development of pectus excavatum (PE) and pectus carinatum (PC). AT foci were examined in intrasurgical biopsy specimens of costal cartilages of children (8-17 years old) with PE (n = 12) and PC (n = 12) and in age-matching autopsy control samples (n = 10) using histological and immunohistochemical staining, atomic force and nonlinear optical microscopy, transmission and scanning electron microscopy, morphometry and statistics. AT areas were identified in the costal cartilage ECM in children with normal chest, PE and PC. Each type of the AT areas ("canonical", "intertwined", "fine-fibred" and "intralacunary") had a unique morphological pattern of thickness and alignment of amianthoid fibers (AFs). AFs were formed via lateral aggregation of collagen type II fibrils in the intact ECM. Foci of the AT were observed significantly more frequently in the PE and PC groups. The AT areas had unique quantitative features in each study group. AT is a structurally diverse form of ECM alteration present in healthy and pathological costal cartilage. PE and PC are associated with specific AT disorders.

UI MeSH Term Description Entries
D008297 Male Males
D002356 Cartilage A non-vascular form of connective tissue composed of CHONDROCYTES embedded in a matrix that includes CHONDROITIN SULFATE and various types of FIBRILLAR COLLAGEN. There are three major types: HYALINE CARTILAGE; FIBROCARTILAGE; and ELASTIC CARTILAGE. Cartilages
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005109 Extracellular Matrix A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. Matrix, Extracellular,Extracellular Matrices,Matrices, Extracellular
D005260 Female Females
D005660 Funnel Chest A developmental anomaly in which the lower sternum is posteriorly dislocated and concavely deformed, resulting in a funnel-shaped thorax. Pectus Excavatum,Chest, Funnel,Chests, Funnel,Excavatum, Pectus,Funnel Chests
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D066166 Pectus Carinatum A developmental anomaly characterized by abnormal anterior protrusion of the STERNUM and adjacent COSTAL CARTILAGE. Currarino-Silverman Syndrome,Pectus Carinatum, Arcuate,Pectus Carinatum, Chondrogladiolar,Pectus Carinatum, Chondromanubrial,Pouter Pigeon Breast,Arcuate Pectus Carinatum,Arcuate Pectus Carinatums,Breast, Pouter Pigeon,Breasts, Pouter Pigeon,Carinatum, Arcuate Pectus,Carinatum, Chondrogladiolar Pectus,Carinatum, Chondromanubrial Pectus,Carinatum, Pectus,Carinatums, Arcuate Pectus,Carinatums, Chondrogladiolar Pectus,Carinatums, Chondromanubrial Pectus,Carinatums, Pectus,Chondrogladiolar Pectus Carinatum,Chondrogladiolar Pectus Carinatums,Chondromanubrial Pectus Carinatum,Chondromanubrial Pectus Carinatums,Currarino Silverman Syndrome,Pectus Carinatums,Pectus Carinatums, Arcuate,Pectus Carinatums, Chondrogladiolar,Pectus Carinatums, Chondromanubrial,Pigeon Breast, Pouter,Pigeon Breasts, Pouter,Pouter Pigeon Breasts,Syndrome, Currarino-Silverman

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