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Sponastrime dysplasia. A radiologic-pathologic correlation

Journal Article:

Abstract

The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.).
Publication Date:
Jul 01, 1989
Product Type:
Journal Article
Reference Number:
DEN-89-008778; EDB-89-118358
Resource Relation:
Journal Name: Pediatr. Radiol.; (Germany, Federal Republic of); Journal Volume: 19:6/7
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; CONGENITAL MALFORMATIONS; DIAGNOSIS; SKELETON; BIOMEDICAL RADIOGRAPHY; HEREDITARY DISEASES; IMAGES; MORPHOLOGY; PATIENTS; SKELETAL DISEASES; X-RAY RADIOGRAPHY; BODY; DIAGNOSTIC TECHNIQUES; DISEASES; INDUSTRIAL RADIOGRAPHY; MALFORMATIONS; MEDICINE; NUCLEAR MEDICINE; ORGANS; PATHOLOGICAL CHANGES; RADIOLOGY; 550602* - Medicine- External Radiation in Diagnostics- (1980-)
OSTI ID:
5813583
Research Organizations:
California Univ., Los Angeles, Torrance, CA (USA). Dept. of Radiology; California Univ., Los Angeles, Torrance, CA (USA). Dept. of Pediatrics; Erlangen-Nuernberg Univ., Erlangen (Germany, F.R.). Inst. fuer Pathologie; Mainz Univ. (Germany, F.R.). Inst. fuer Paediatrie und Medizinische Genetik
Country of Origin:
Germany
Language:
English
Other Identifying Numbers:
Journal ID: CODEN: PDRYA
Submitting Site:
DEN
Size:
Pages: 417-424
Announcement Date:

Journal Article:

Citation Formats

Lachman, R S, Stoss, H, and Spranger, J. Sponastrime dysplasia. A radiologic-pathologic correlation. Germany: N. p., 1989. Web.
Lachman, R S, Stoss, H, & Spranger, J. Sponastrime dysplasia. A radiologic-pathologic correlation. Germany.
Lachman, R S, Stoss, H, and Spranger, J. 1989. "Sponastrime dysplasia. A radiologic-pathologic correlation." Germany.
@misc{etde_5813583,
title = {Sponastrime dysplasia. A radiologic-pathologic correlation}
author = {Lachman, R S, Stoss, H, and Spranger, J}
abstractNote = {The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.).}
journal = {Pediatr. Radiol.; (Germany, Federal Republic of)}
volume = {19:6/7}
journal type = {AC}
place = {Germany}
year = {1989}
month = {Jul}
}