No abstract available.
Diagnosis* ; Facial Asymmetry*

No abstract available.
Diagnosis* ; Facial Asymmetry*

PURPOSE: Ataxia Telangiectasia (AT) is a hereditary multi-systemic disease resulting from mutations of AT gene and is characterized by progressive neurodegeneration, cancer, immune system defects, and hypersensitivity to ionizing radiation. AT gene has a homologue sequence of PI3-kinase. The activity and cellular function of PI3-kinase in AT gene remains unclear. This study was undertaken to evaluate the function of AT gene through the effect on cell survival and differentiation by the inhibition of AT gene expression. MATERIALS AND METHODS: NH2-terminal portion of AT gene was isolated from MCF-7 cells by RT-PCR. The isolated DNA fragment was ligated in reverse orientation in pcDNA3. This antisense ATM expression vector was transfected to PC-12 cells by calcium phosphate method, and the transformed cells were selected using G418 and immunohisto- chemistry. To analyze the cell survival and differentiation, cells were cultured in serum free medium supplemented with/without NGF. We performed the immunoprecipitation for the p53 induction of cells after ionizing radiation, and the FACS for the apoptosis of cells after the exposure of wortmanin. RESULTS: PC-12 cells which down-regulated AT gene (like ATM, AT mutated) showed decreased survival and ceased differentiation with NGF. Also, PC-12 (ATM) cells showed increased apoptosis with wortmanin and reduced or delayed p53 induction after ionizingradiation. CONCLUSION: Results obtained from these studies suggest that AT gene regulates survival and differentiation of PC-12 cells through PI3-kinase activity. It seems that apoptosis is induced by the inhibition of AT gene expression.
Apoptosis ; Ataxia Telangiectasia ; Calcium ; Cell Survival ; Chemistry ; DNA ; Gene Expression ; Hypersensitivity ; Immune System ; Immunoprecipitation ; MCF-7 Cells ; Nerve Growth Factor ; Phenotype* ; Phosphatidylinositol 3-Kinases ; Radiation, Ionizing

Facial asymmetry is the most frequent disease in craniofacial deformities. And the primary causing area of that is mostly placing in mandible. That is to say, it is known that primarily, mandible grows excessively or deficiently, and other facial region involving maxilla undergoes compensatory growth secondarily, so asymmetric face develops. In facial asymmetry, the surgical correction of undergrowth is more difficult than that of overgrowth and the reason of it is the postoperative relapse caused by stress of surrounding soft tissues. It means the stress of surrounding soft tissues occurring after bone lengthening and reducing above stress is the same meaning with reducing postoperative relapse. Among various areas, mandibular ramus is the most difficult area to lengthen vertically and maintain its length. The reason of it is considered by many authors as the stress of surrounding pterygomasseteric sling which is enveloping lower border of mandible and interrupting elongation of ramal height. So we applied two different surgical procedures in which pterygomasseteric slings have different stress respectively to monkeys which have similar masticatory function and anatomy to human being and compared relapse by radiographic film and observed periodically the histochemical change of masseteric muscle fiber. So we could see the following results. The relapse was less in EVRO group in which we separated pterygomasseric sling in inferior border and didn't approximate muscle sling after vertical lengthening to minimize the stress of soft tissues than IVRO group in which we elongated ramal height preserving pterygomassetric sling. Of course, we could see a problem in EVRO group such as bone resorption in inferior border caused by uncovering the periosteum of inferior border. But we expect that such problem will be solved by developing periosteum substitutes for covering the exposed bone and minimizing the surgical trauma. In histochemical study of masseteric muscle fiber, the fiber constituents of EVRO group in which we minimized soft tissue stress was changed immediately after operation and maintained it for 1 year, whereas that of IVRO group in which we preserved soft tissue stress was changed in more portion after operation and recovered it by 1 year . By the histochemical results, we can see that the recovery of fiber constituents reflect the recovery of muscle stress and it is closely related with relapse phenomenon.
Bone Lengthening ; Bone Resorption ; Congenital Abnormalities ; Facial Asymmetry ; Haplorhini ; Humans ; Mandible ; Maxilla ; Periosteum ; Recurrence* ; X-Ray Film

Behcets syndrome is a multisystem disorder presenting with recurrent oral and genital ulcers as well as uveitis, Gastrointestinal involvement associated with ileal ulcer is rare, but its outcome may by fatal. We have recently experienced a 43-year-old man who had Behcets syndrome with ileal ulcer penetrated to 3rd portion of the duodenum. He had been updergone right hemicolectomy and ileocolostomy for ileal inflammation of unknown etiology 20 years ago. Thereafter he had had relapsing oral and genital ulcers, and abdominal pain intermittently. From 15 days ago before visiting our hospital, he had severe abdominal pain and dark blood-colored stool. The colonofiberscopic finding revealed a large, deep penetrating ulcer and multiple shallow ulcers on the ileum. Deal segmental resection, gastro-jejunostomy and jejunal patch at 3rd portion of the duodenum were performed with marked improvement.
Abdominal Pain ; Adult ; Behcet Syndrome ; Duodenum* ; Humans ; Ileum ; Inflammation ; Ulcer* ; Uveitis

This study examined, among others, the relationship between the use of anticonvulsant and the bone mineral metabolism(measured via DR-XRA and biochemical study) in 46 adult epileptic outpatients who had been on anticonvulsant for more than one year at Seoul Red Cross Hospital. The study finding wet as follows. 1) There was statistically significant difference between index group(i.e. 46 adult epileptic outpatients) and control group(i.e. 46 healthy adults without previous exposure to anticonvulsant) in terms of bone mineral density. Specifically, the bone mineral density in the epileptic outpatients was significantly lower than that in the healthy adult. Although there was no statistically significant difference between male and female outpatients, their bone mineral density tended to decrease with the increase of age. 2) The bone mineral density in the epileptic outpatients was negatively correlated to both I) the age when the outpatients stared anticonvulsant treatment and ii) the period for which the outpatients were on anticonvulsant 3) Index group was further classified into three subgroup(i.e. low, normal and high subgroup) based on the mean and standard deviation of the bone mineral density in healthy adults control group. 24 outpatients(52.5%) out of 46 fell into low subgroup, of which 10 were male. Overall, there was no statistically significant difference between male and female outpatients in terms of their bone mineral density. 4) Low subgroup's average age(38.6 with SD of 12.5) was significantly higher than those of the other two subgroup. Low subgroup stained anticonvulsant treated when their average age was 24.8(SD 15.0) years, which was higher than those of the other two subgroups. Low subgroup was on anticonvulsant, on average, far 13.3(SD 7.6) years, which was also higher than those of the other two subgroups although there was no significant difference among the subgroups. 5) A series of comparison regarding the possible impact of various types of anticonvulsan(e.g. PHT, CBZ, FB, SV) in terms of i) overall dosage and ii) daily average dosage on bone mineral density showed no significant difference across the three subgroups. 6) There was no significant difference in terms of bone mineral density between the epileptic outpatients on only one type of anticonvulsant and those on two or more types of anticonvulsant. 7) A biochemical study showed that i) each subgroup fell into normal range in terms of blood serum Ca, F, and ALF with no significant difference among the subgroups, and ii) in low subgroup, there were 6 outpatients(25%) with Ca decrease, 2(9.5%) with P decrease, and 5(20.8%) with ALF increase.
Adult ; Bone Density ; Female ; Humans ; Male ; Outpatients* ; Radiography* ; Red Cross ; Reference Values ; Seoul ; Serum

No abstract available.
Graves Disease* ; Hypothyroidism*

PURPOSE: To evaluate clinical and radiological results after screw fixation of the scaphoid and lunotriquetral ligament repair using a dorsal approach in the treatement of trans-scaphoid perilunate fracture dislocations. MATERIALS AND METHODS: From May 2003 to August 2007, 11 patients who underwent operative management of a trans-scaphoid perilunate fracture dislocation were included in this study. Average follow up period was 58 months. In all patients, screw fixation of the scaphoid and lunotriquetral ligament repair with a suture anchor after open reduction was performed. Clinical evaluation was done by measuring range of motion and grip power and disabilities of arm, shoulder and hand (DASH) score evaluation for functional recovery at the last follow up. Union of scaphoid, change in lunotriquetral distance, and development of any instability and arthritis of wrist joint were radiographically assessed. RESULTS: In clinical outcomes, 89.3% recovery of grip power and 87.5% recovery of range of motion compared to healthy side were observed at the last follow-up. Average range of motions of extension, flexion, ulnar deviation, radial deviation, supination and pronation were 51.8degrees, 58.4degrees, 21.2degrees, 16.2degrees, 74.3degrees, 75.1degrees respectively. Average DASH score was 13.2. Bony union of scaphoid was achieved in all cases at the average of postoperative 19.3 weeks. Lunotriquetral distance after the operation and at the last follow up were 1.9 mm and 2.0 mm, respectively. There were no radiographic evidence of instability or arthritis. CONCLUSION: Dorsal approach allows reduction of carpal bone, scaphoid fixation and lunotriquetral ligament repair in the treatment of trans-scaphoid perilunate fracture dislocations, providing satisfactory clinical and radiological results.
Arm ; Arthritis ; Carpal Bones ; Dislocations ; Follow-Up Studies ; Hand ; Hand Strength ; Humans ; Ligaments ; Pronation ; Range of Motion, Articular ; Shoulder ; Supination ; Suture Anchors ; Wrist Joint

PURPOSE: To determine the detection rate of MRI in the diagnosis of adenomyosis and ascertain the accuracy of MRI in assessing the extent and depth of the myometrial invasion. MATERIALS AND METHODS: By retrospective analysis of MR images of the pelvis in 65 pathologically proven cases of uterine adenomyosis, we investigated the detection rate and differences of in the detection rate when this was based on (a) the extent, and (b) the depth of myometrial invasion. The condition was classified as diffuse or focal according to the extent of invasion, and mild, moderate, or severe according to its depth. RESULTS: Pathologically, there were 35 cases of focal adenomyosis (53.8%), and 30 of diffuse adenomyosis (46.2%). Among patients with the focal variety of this condition, 12 cases were mild, 14 were mild, 14 were moderate, and nine were severe, while among those with the diffuse variety, two were mild, ten were moderate and 18 were severe. A total of 48 cases (73.8%) were detected on MR images; the detection rate of focal adenomyosis was 60.0% (21/35) and that of diffuse adenomyosis was 90.0% (27/30). The detection rates of mild, moderate, and severe adenomyosis were 42.9%(6/14), 79.2%(19/24), and 85.2% (23/27), respectively. Among the 48 cases detected on MR images, the pathologic and MR findings were consistent with regard to both the extent and depth of myometrial invasion in 26(54.2%). For diffuse adenomyosis, the consistency rate was higher than for the focal variety (81.5%, 22/27; compared with 19%, 4/21). The extent of myometrial invasion was correctly evaluated in 32 cases (66.7%); the consistency rate for the diffuse form was higher than for the focal form [96.3% (26/27), compared with 28.6% (6/21)]. In 42 cases (87.5%), the depth of invasion was correctly evaluated, though differences in this depth were not significant. CONCLUSION: MR imaging was a useful imaging modality in the diagnosis of uterine adenomyosis. With regard to the extant of mymetrial invasion, the detection rate was higher among cases of the diffuse form than of the focal form, and with regard to depth, the rate was higher among moderate and severe cases than among mild ones. These findings may be useful for preoperative diagnosis based on clinical symptoms, and provide important yardstick for decisions as to whether hysterectony should be replaced by an alternative therapy.
Adenomyosis* ; Diagnosis ; Humans ; Magnetic Resonance Imaging* ; Pelvis ; Retrospective Studies ; Uterus

No abstract available.
Arteriovenous Malformations*