title: Rib cartilage characterization in patients affected by pectus excavatum.
abstract: Pectus excavatum (PE) is the most frequent anterior chest deformity which may be frequently associated with connective tissue disorders. We performed microscopic analyses to better understand cartilage behavior and obtain clues on its pathogenesis. In 37 PE patients, none with Marfan syndrome, we analyzed costal cartilage by light microscopy, immunohistochemistry and transmission electron microscopy. Control tissue specimens were harvested from four patients without any connective tissue disease. In both control and PE patients, chondrocytes were on the average <15 µm in diameter and occupied <10% of tissue volume; in most cases the extracellular matrix was stained by alcian blue, instead of safranin; no difference between PE and control samples was significant. All samples showed an uneven collagen type II immunolabeling both within the cells and pericellular matrix, and occasionally of the territorial matrix. In all cases numerous cells underwent apoptosis accompanied by matrix condensation as shown by electron microscopy. Our results suggest that matrix composition and the cell number and size of costal cartilage are dependent on the subject and not on the disease; the microscopic organization of cartilage is correlated with the stabilization of the defective shape rather than with the onset of the deformity.
authors.affiliation: Department of Pediatric Surgery, University of Florence and Children's University Hospital A. Meyer, Florence, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Tocchioni
authors.forename: Marco
authors.initials: M
authors.lastname: Ghionzoli
authors.forename: Laura
authors.initials: L
authors.lastname: Calosi
authors.forename: Daniele
authors.initials: D
authors.lastname: Guasti
authors.forename: Paolo
authors.initials: P
authors.lastname: Romagnoli
authors.forename: Antonio
authors.initials: A
authors.lastname: Messineo
mesh_terms: D000293:Adolescent; D000328:Adult; D002356:Cartilage / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D016022:Case-Control Studies; D002648:Child; D019902:Chondrocytes / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D015331:Cohort Studies; D005260:Female; D005660:Funnel Chest / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D046529:Microscopy, Electron, Transmission; D012272:Ribs / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D055815:Young Adult
keywords: fibrillin; fibrillinopathies; marfan; pectus excavatum; rib cartilage