PubMed Health⌕ Search

Biomedical subjects

Hidehiko Kawabata

Publications and source records attributed to Hidehiko Kawabata.

5 recordsLinked to original sources
PubMed2006

Spine and rib abnormalities and stature in spondylocostal dysostosis.

STUDY DESIGN: A retrospective study of radiographic and clinical findings of spondylocostal dysostosis. OBJECTIVE: To determine the features of spondylocostal dysostosis diagnosed using consistent diagnostic criteria. SUMMARY OF BACKGROUND DATA: To our knowledge, no clear definition of spondylocostal dysostosis exists, and little information is available regarding its clinical or radiographic features. METHODS: We defined spondylocostal dysostosis as a congenital spinal disorder consisting of >or=2 vertebral anomalies associated with rib anomalies, without crab-like chest. For 30 patients, including 12 males and 18 females, who met these criteria, we evaluated vertebral and rib anomalies, birth and present body height, and associated anomalies. There were only 2 familial cases. RESULTS: Features of spondylocostal dysostosis were: (1) anomalies involved the thoracic region in all cases; many also involved the cervical spine; (2) most patients had >or=4 vertebral anomalies; (3) frequent vertebral anomalies were butterfly vertebra, hemivertebra, complete block, and unilateral bar, which were associated with both rib absence and fusion; (4) short stature was not always present at birth; and (5) complete block was 1 factor identified as being related to short stature after 12 years of age. CONCLUSION: Several features of sporadic spondylocostal dysostosis disorder were determined, including new findings related to body height.

Abnormalities, Multiple↗
PubMed2003

Open treatment of syndactyly of the foot.

The aim of reconstruction of the webs for syndactyly of the foot is cosmetic improvement, so skin grafting should be avoided. We present our long-term results of 19 feet with simple cutaneous syndactyly in 15 patients who were treated by an open technique. The bottom of the web was covered with a dorsal rectangular flap and the remaining skin defect was left open to epithelialise spontaneously. This took about 4 weeks. After a mean follow-up of 5.7 years (range 3-9), no hypertrophic scar or pigmentation of the skin had developed. Creeping of the web was seen only during the first postoperative year. The final depth of the web satisfied the families of all patients. As the aim of web reconstruction for syndactyly of the foot is purely cosmetic, we conclude that this simple method is reasonable treatment.

Female↗
PubMed2002

Multiple trigger fingers associated with geleophysic dysplasia.

We report an infant with geleophysic dysplasia (MIM 231050) who developed multiple trigger fingers. The condition was progressive and distinct from trigger thumb, which is generally seen in infants without any underlying metabolic disease.

Abnormalities, Multiple↗
PubMedDate not supplied

Differential expression of aggrecan mRNA isoforms by chondrosarcoma cells.

BACKGROUND: Aggrecan, the large aggregating proteoglycan, is a major component of the cartilage extracellular matrix. The C-terminal globular domain (G3) of the aggrecan molecule exhibits a pattern of alternative splicing that involves three exons. Each corresponds to a subdomain, epidermal growth factor 1(EGF1), EGF2, and a short complement repeat (SCR). MATERIALS AND METHODS: In the present study, we compared aggrecan mRNA splicing variants in osteochondrogenic tumor cells and normal cartilage using RT-PCR. RESULTS: Normal cartilage expressed two mRNA isoforms, both containing the SCR exon, but with or without the EGF1 exon. Benign cartilaginous tumors showed the same splicing pattern as normal cartilage. However, malignant chondrosarcomas expressed additional splicing isoforms, one of which lacked all three alternative exons and another with EGF2 and SCR exons. The human chondrosarcoma cell line (Ch-1 cells) expressed further isoforms, one containing either EGF1 or EGF2 exon only, and another with all the variant exons. CONCLUSION: These results imply that a change in the pattern of alternative aggrecan mRNA splicing is associated with malignant transformation of chondrocytes.

Aggrecans↗