Ehlers-Danlos syndrome is an inherited disorder of connective tissue in which joint hypermobility, hyperelasticity of skin, bleeding tendency, and scar formation are the most prominent features. It is a generalized disease with essentially universal involvement. A 3-year-old female child had velvety skin, skin hyperextensibility, joint hypermobility, subcutaneous mobile masses on the left shin, many scars on both knees, subcutaneous hematoma, and thick and very folded skin on both palms and soles, but no evidence of internal disorder. Cutaneous histopathologic findings were nonspecific without increase of dermal elastic fibers on Verhoeff stain. Diagnosis was confirmed by clinical and histopathological findings as Ehlers-DanIos syndrome.
Child ; Child, Preschool ; Cicatrix ; Connective Tissue ; Diagnosis ; Ehlers-Danlos Syndrome ; Elastic Tissue ; Female ; Hematoma ; Hemorrhage ; Humans ; Joint Instability ; Knee ; Skin

Most of cataract surgeons have made a great effort to minimize or nullify corneal 'astigmatism resulted from the incision and closure, but a completely ideal wound system still eludes us. The scleral pocket incision and continuous single knotted shoelace suture of it has been known as one of the technique for reducing postoperative astigmatism. Recently, horizontal suture closure of scleral pocket incisior has been introduced because it doesn't cause suture induced wound compression. We implanted standard PMMA intraocular lenses in the bag of 400 patients through 7mm scleral pocket incision following CCC (continuous circular capsulorhexis) and bimanual phacoemulsification. The incision was closed with either the shoelace or horizontal suture. The keratometric measurement was maded at postoperative 1 day, 1 week, 1 month, 2 month, 3 month and 6 month. In shoelace suture group, the preoperative corneal astigmatism appeared -0.14 +/- 0.15D of with the rule astigmatism (WTR); at one day postoperatively -2.75 +/- 1.61 of WTR, at 3 month 0.18 +/- 1.14D of against the rule astigmatism (ATR), 6 month 0.3 +/- 1.22D of ATR. In horizontal suture group, the preoperative corneal astigmatism appeared -0.22 +/- 1.22D of WTR; at one day postoperatively -1.05 +/- 1.28 of WTR, at 2 month 0.57 +/- 1.0 of ATR, at 6 month 0.72 +/- 1.91 of ATR. The difference between two suture methods was statistically significant (p<0.01 until 3 month, p<0.05 until 3-6 month).
Astigmatism* ; Cataract ; Humans ; Lenses, Intraocular ; Phacoemulsification ; Polymethyl Methacrylate ; Sutures* ; Wounds and Injuries

The authors studied EBV genome expression in 40 conventionally processed samples of nasopharyngeal carcinomas (NPC), using in situ hybridization for EBERs and immunohistochemistry for LMP, Bcl-2 and p53 proteins. The NPCs consisted of 6 keratinizing squamous cell carcinomas (KSCs), 13 nonkeratinizing carcinomas (NKCs) and 21 undifferentiated carcinomas (UCs). The results were summarized as follows: 1) EBERs were expressed in 80.0% of all the NPCs (32/40). As for the subtypes, they were detected in 92.3% of NKCs (12/13), in 90.5% of the UCs (19/21), and in 16.7% of the KSCs (1/6). In positive cases, the nuclei of tumor cells displayed uniformly strong staining. 2) LMP was expressed in 10.0% of all the NPCs (4/40), all of which were UC. The LMP expression in the UCs was not correlated to the expression of EBERs, Bcl-2 and p53 proteins. 3) Bcl-2 protein was detected in 85.0% of all the NPCs (34/40). As for the subtypes, they were detected in 92.3% of the NKCs (12/13), in 90.5% of the UCs (19/21), and in 50.0% of the KSCs (3/6). 4) p53 protein was detected in 75.0% of all the NPCs (30/40). As for the subtypes, they were detected in 81.0% of the UCs (17/21), in 69.2% of the NKCs (9/13), and in 66.7% of the KSCs (4/6). 5) In the NPCs the expression of EBER showed a significantly positive correlation with that of p53 or Bcl-2 protein. The above results indicate that the association of EBV with NPC is chiefly with poorly differentiated and undifferentiated carcinomas. Additionally, carcinomas commonly display widespread, strong immunoreactivity of Bcl-2 and p53 proteins over tumor cells. In conclusion, these observations indicate that the EBV-association in NPC appears to contribute to the overexpression of tumor-related genes during carcinogenesis.
Carcinogenesis ; Carcinoma ; Carcinoma, Squamous Cell ; Genome ; Herpesvirus 4, Human* ; Immunohistochemistry ; In Situ Hybridization

No abstract available.
Humans*

BACKGROUND: In eczematous skin it is expected that altered defense mechanisms are employed against an injury, which mechanisms include alterations in the stratum corneum barrier function and lipid contents. OBJECTIVE: To investigaten aspect of pathophysiology of hand eczema by comparing the water holding capacities and transepidermal water loss(TEWL) after injuries on the skin and by comparing the epidermal lipid contents between hand eczema and normal subjects. METHODS: The water holding capacities were checked by corneometer, and the TEWL were checked after epidermal barrie! destruction with acetone or adhesive tapes. The epidermal lipids were extracted with acetone, ther (1: 1 v/v) mixture solvent and analysed by HP- TLC. RESULTS: The water holdingapacities were decreased in eczema of the palm, but the recoveries of TEWL and the lipid content did not differ between hand eczema and normal subjects. CONCLUSION: The water holding capacities were decreased in the eczematous skin. But there were no significant differencies in the recovery of destructed epidermal barrier and the epidermal lipid contents.
Acetone ; Adhesives ; Defense Mechanisms ; Eczema* ; Hand* ; Skin ; Water

Refractory deformity in ankylosing spondylitis is caused by loss of normal lordotic curvature in lumbar spine. For the correction of deformity, monosegmental osteotomy, intracorporal decancellation and multisegmental osteotomy are used. Among them, multisegmental osteotomy is reported to be safe because of its small correction amount at each level. Since 1989, authors treated 5 cases of ankylosing spondylitis with severe kyphotic deformity by multisegmental osteotomy and transpedicular instrumentation. All were young males between 22 and 35 years of age. Preoperative kyphotic deformity was 80°, 105°, 72°, 35°, 55° (Av. 70°) and amount of correction was 55°, 105°, 72°, 20°, 40° (Av. 58°) respectively. Levels for osteotony were 4 to 8 segments and correction at a level was 5° to 13° (Av. 8.3°). Normal standing upright posture and vision for straight forward were obtained in all. Instrumentation was Zielke in three Cotrel-Dubousset in two. One case of Zielke instrumentation showed rod failure. However, all showed solid bony union without any loss of correction or pseudoarthrosis. From the above experience, multisegmental osteotomy for the treatment of kyphotic deformity in ankylosing spondylitis was believed to be a safe and effective method of treatment.
Congenital Abnormalities ; Humans ; Male ; Methods ; Osteotomy ; Posture ; Pseudarthrosis ; Spine ; Spondylitis, Ankylosing

No abstract available.
Tonsillectomy*

The authors analyzed 174 consecutive shunting procedures for hydrocephalus at Severance Hospital, Yonsei University. There were a total of 65 complications (48.5%) among 134 patients. The most common complications were blockage of the catheters (26.2%) and shunt-related infections (15.4%). Among the different types of shunting technique ventriculoatrial, ventriculoperitoneal and ventriculocisternal routes were most commonly used. The ventriculoatrial route had a greater complication rate (40%) than ventriculoperitoneal shunting (21.8%). Flushing devices are critically reviewed and the disadvantages are discussed. The authors suggested a need for a standardized prospective national data collection system where Procedures and compilations may be assembled in a comparable form.
Bacterial Infections/etiology ; Catheterization/adverse effects ; Cerebrospinal Fluid Shunts*/adverse effects ; Female ; Human ; Hydrocephalus/surgery* ; Infant ; Male

No abstract available.
Sarcoma* ; Uterus*

Papillary serous carcinoma of the peritoneum (PSCP) is a primary tumor of peritoneal lining (mesothelioma) of the abdomen and is histologically difficult to differentiate from papillary serous carcinoma of the ovary. It is very rare tumor that involves the surface of the pelvic and/or abdominal peritoneum. Most patients with this tumor have been treated with optimally surgical cytoreduction and postoperative chemotherapy. However, long term survival has not been achieved in many studies. In recent years, platinum-paclitaxel combination therapy was reported as a effective initial therapy for recurrent PSCP. We have experienced one case of recurrent PSCP which was successfully treated with heptaplatin and paclitaxel. We report the toxicity and long term result of the patient.
Abdomen ; Drug Therapy ; Drug Therapy, Combination* ; Female ; Humans ; Ovary ; Paclitaxel ; Peritoneum