Alexandr Kurkov1,2, Anna Guller1,3, Alexey Fayzullin1,4, Nafisa Fayzullinа5, Vladimir Plyakin6, Svetlana Kotova1,7, Petr Timashev1,4,7,8, Anastasia Frolova1, Nikita Kurtak9, Vyacheslav Paukov2, Anatoly Shekhter1. 1. Institute for Regenerative Medicine, Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia. 2. A.I. Strukov Department of Anatomical Pathology, Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia. 3. The Graduate School of Biomedical Engineering, University of New South Wales, Sydney, New South Wales, Australia. 4. World-Class Research Center "Digital Biodesign and Personalized Healthcare", Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia. 5. National Medical Research Center for Obstetrics, Gynecology and Perinatology Named After Academician V.I. Kulakov of the Ministry of Healthcare of the Russian Federation, Moscow, Russia. 6. Clinical and Research Institute of Emergency Pediatric Surgery and Traumatology, Moscow, Russia. 7. Department of Polymers and Composites, N.N. Semenov Institute of Chemical Physics, Moscow, Russia. 8. Chemistry Department, Lomonosov Moscow State University, Moscow, Russia. 9. FSBI "Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs", Ministry of Health of the Russian Federation, Moscow, Russia.
Abstract
BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSION: 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.
BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSION: 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.