No abstract available.

No abstract available.
Hand*

Trichorhinophalangeal syndrome (TRPS) was first described in 1966 by Giedion. It is a rare genetic disease and divided into two groups : TRPS type I and TRPS type II. TRPS type I is an autosomal dominant inherited disorder, defined by three characteristic findings : first, sparsely distributed brittle and slow-growing scalp hair, second, a bulbous pear-shaped nose with an elongated philtrum, third brachydactyla with angular deformities. The characteristic radiological features of the hands include cone shaped epiphysis of the proximal interphalangeal joints. Most TRPS type II is sporadic and shows multiple exosteosis, mental retardation and microcephaly in addition to the three chracteristic findings of TRPS type I . A 20-year-old female visited our department for the evaluation of her sparse, slow-growing scalp hair. She also had a bulbous pearshaped nose, ulnar deviated fingers and shortened both hallux. Some relatives of patients show similar symptoms of scalp hair. A Radiologic aly, histopathologic aly and chromosomal study were done, and we established the diagnosis, TRPS type I.
Congenital Abnormalities ; Diagnosis ; Epiphyses ; Female ; Fingers ; Hair ; Hallux ; Hand ; Humans ; Intellectual Disability ; Joints ; Lip ; Microcephaly ; Nose ; Scalp ; Young Adult

No abstract available.
Scalp* ; Superficial Back Muscles*

No abstract available.
Pregnancy*

Transvaginal cervical cerclage for the prevention of pregnancy loss in patients with cervical incompetence is well accepted. However, there is a small subject of patients with incompetence of cervix, congenital or surgical shortening of the cervix, previously failed transvaginal cerclage and deep cervical damage or tears in the fornices. We experienced a case of the transabdominal cervical cerclage. 'Ihe patient had a past history of deep cervical laceration due to vaginal delivery. Therefore, we present here a surgical technique of transabdominal cerdage and report it with brief review of literatures.
Cerclage, Cervical ; Cervix Uteri* ; Female ; Humans ; Lacerations ; Pregnancy

No abstract available.
Incontinentia Pigmenti*

Neurofibromatosis produces a broad spectrum of clinical manifestations. Cafe-au-lait spots, cutaneous neurofibroma and tumors of the central and peripheral nervous system are well known manifestations. One of the more serious aspects of the disease relates to the arterial involvement. Renal arterial disease with resultant hypertension has been paticularly well documented. However, cerebrovascular lesions in neurofibromatosis are uncommon. Cerebral arterial occlusive diseases with juxtabasilar telangiectasia (moyamoya disease) associated with neurofibromatosis have been documented in about 40 patients in the world literature. We report a case of neurofibromatosis associated with the moyamoya disease.
Arterial Occlusive Diseases ; Cafe-au-Lait Spots ; Humans ; Hypertension ; Moyamoya Disease* ; Neurofibroma ; Neurofibromatoses* ; Peripheral Nervous System ; Telangiectasis

Previous successful results of neocartilage formation using tissue engineering technique in immunocompromised nude mouse xenograft model were reported. For clinical application, autogenous cell is preferrable to allogenic or xenogenic cell for circumvention of immune rejection. This study evaluates the feasibility of producing a engineered cartilage using autogenous chondrocytes. Chondrocytes were isolated from the auricular cartilage of New Zealand White rabbit and seeded onto PGA polymer coated with polylactic acid in round pattern(diameter 0.7 cm, thickness 0.1 cm) at a concentration 7 x 10(7) chondrocytes per cm3. Each Autogenous Cell-polymer constructs were implanted subcutaneously into the left side of dorsum of twelve rabbits. Polymer templates not containg cells were implanted into the right side as a control. Twelve rabbits were sacrificed at the following intervals: 5 rabbits at nine weeks, 7 rabbits at twelve weeks New autogenous cartilage formation which retained the approximate dimensions of original round polymer template in 11 of 12 cell seeded implants. Histological examination using hematoxyline and eosin stain revealed vast majority of implants developed into mature cartilage. This study opens up the possibility of autologus cell transplant to construct autogenous cartilge.
Animals ; Cartilage* ; Chondrocytes* ; Ear Cartilage ; Eosine Yellowish-(YS) ; Hematoxylin ; Heterografts ; Mice ; Mice, Nude ; New Zealand ; Polymers ; Rabbits ; Tissue Engineering ; Transplants

No abstract available.
Progeria*