Abstract
The fourteenth reported patient with Murk Jansen's metaphyseal chondrodysplasia is presented, with a remarkable followup from birth to the age of 15 years. Numerous invasive procedures were performed in pursuit of erroneous provisional diagnoses. Five of these patients presented in infancy with radiographic metaphyseal changes similar to rickets, but with preservation of the provisional zone of calcification. Following infancy, these patients reveal the more typical short-limbed dwarfism, with fusiform joints and bowed extremities.
Similar content being viewed by others
References
Jansen M (1934) Über atypische Chondystrophie (Achondroplasie) und über eine noch nicht beschriebene angeborene Wachstumsstörung des Knochensystems: Metaphysaere Dysostosis. Z Orthop Chir 61: 253
Hass UHD, Boer W, Griffioen (1969) Metaphyseal dysostosis: A late followup of the first reported case. J Bone Joint Surg [Br] 51: 290
Cameron JAP, Young WB, Sissons HA (1954) Metaphyseal dysostosis. J Bone Joint Surg 36: 622
Weil S (1957) Die metaphysaeren Dysostosen. Z Orthop 89: 1
Gram PB, Fleming JL, Frame B (1959) Metaphyseal chondrodysplasia of Jansen. J Bone Joint Surg [Am] 41: 951
Silva E (1966) Disostosi metafisiaria tipo Jansen. Chir Organi Mov 54: 400
Holt JF (1969) Jansen's metaphyseal dysostosis. Birth Defects 5: 73
Ozonoff MB (1969) Metaphyseal dysostosis of Jansen. Radiology 93: 1047
Arroyo-Scotoliff H (1973) Metaphyseal dysostosis, Jansen type. J Bone Joint Surg [Am] 55: 623
Gordon SL, Varano LA, Alandete A, Maisels MJ (1976) Jansen's metaphyseal dysostosis. Pediatrics 58: 556
Kikuchi S, Hasue M, Watanabe M, Hasebe K (1976) Metaphyseal dysostosis (Jansen Type). Report of a case with long follow-up. J Bone Joint Surg [Br] 58: 102
Nazara Z, Hernandez A, Corona-Rivera E, et al. (1981) Further clinical and radiological features in metaphyseal chondrodysplasia Jansen Type. Radiology 140: 697
Lenz WD (1969) Murk Jansen type of metaphyseal dysostosis. Birth Defects 5: 71
Holthusen W, Holt JF, Stoeckenius M (1975) The skull in metaphyseal chondrodysplasia type Jansen. Pediatr Radiol 3: 137
Raisz LJ (1970) Bone formation and resorption in tissue culture. Arch Int Med 126: 887
Spranger JW, Langer LO, Wiedemann HR (1974) Bone dysplasia: an atlas of constitutional disorders of skeletal development. Saunders, Philadelphia, p 68
Sillence DO, Horton WA, Rimoin DL (1979) Morphologic studies in the skeletal dysplasias. A review. Am J Pathol 96: 814
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Silverthorn, K.G., Houston, C.S. & Duncan, B.P. Murk Jansen's metaphyseal chondrodysplasia with long-term followup. Pediatr Radiol 17, 119–123 (1987). https://doi.org/10.1007/BF02388087
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02388087