{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T16:09:23Z","timestamp":1792166963919,"version":"4.4.0"},"reference-count":15,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2002,1,1]],"date-time":"2002-01-01T00:00:00Z","timestamp":1009843200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2002,1]]},"abstract":"<jats:p> A morphological study of the skeletal system in a case of short rib polydactyly syndrome type III (SRPS-III) documented a \u201cbajonet\u201d deformity of the ribs for misalignment and overlap of cartilaginous and bony ends. This deformity resulted from a \u2018tandem\u2019 change in endochondral bone formation that is, arrested orthotopic cartilage maturation and etherotopic perichondral cartilage differention and ossification. At the cartilaginous end, cartilage maturation and vascular invasion were absent. At the bony end, longitudinal bone growth occurred by a perichondral ectopic growth plate. \u2018Miniature\u2019 versions of this \u2018tandem\u2019 change were also demonstrated in the long bones of the limbs and included focally arrested orthotopic cartilage maturation at the growth plates, perichondral cartilage differentiation, and ossification within cartilage canals. Our morphological study indicates that a generalized loss of syncrony in cartilage removal and osteogenic differentiation occurs in all growth plates, albeit with varied expressivity, and represents, at tissue level, the mechanism by which the SRPS-III skeletal phenotype develops. <\/jats:p>","DOI":"10.1007\/s10024-001-0122-2","type":"journal-article","created":{"date-parts":[[2003,12,2]],"date-time":"2003-12-02T21:39:20Z","timestamp":1070401160000},"page":"91-96","source":"Crossref","is-referenced-by-count":8,"title":["Short Rib Polydactyly Syndrome Type III: Histopathogenesis of the Skeletal Phenotype"],"prefix":"10.1177","volume":"5","author":[{"given":"Alessandro","family":"Corsi","sequence":"first","affiliation":[{"name":"Department of Experimental Medicine, University of L'Aquila, Via Vetoio, Coppito II, 67100 L'Aquila, Rome, Italy"},{"name":"Department of Experimental Medicine and Pathology, Division of Pathology, \u201cLa Sapienza\u201d University, Policlinico Umberto I, Viale Regina Elena 324, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara","family":"Riminucci","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine, University of L'Aquila, Via Vetoio, Coppito II, 67100 L'Aquila, Rome, Italy"},{"name":"Department of Experimental Medicine and Pathology, Division of Pathology, \u201cLa Sapienza\u201d University, Policlinico Umberto I, Viale Regina Elena 324, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Roggini","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Radiology, \u201cLa Sapienza\u201d University, Policlinico Umberto I, Viale Regina Elena 324, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Bianco","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine and Pathology, Division of Pathology, \u201cLa Sapienza\u201d University, Policlinico Umberto I, Viale Regina Elena 324, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2002,1,1]]},"reference":[{"key":"bibr1-s10024-001-0122-2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01959352"},{"key":"bibr2-s10024-001-0122-2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00444756"},{"key":"bibr3-s10024-001-0122-2","first-page":"1","volume":"19","author":"Spranger J","year":"1990","journal-title":"Adv Hum Genet"},{"key":"bibr4-s10024-001-0122-2","first-page":"474","volume":"17","author":"Sillence DO","year":"1987","journal-title":"I. 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