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.
Arteriovenous Malformations*

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

The incidence rate of calcaneal fracture consists about 2% of all fractures, and, of the fracture, calcaneal tubercle avulsion fracture is known to be rare. To treat non-displaced calcaneal tubercle avulsion fracture, conservative treatment such as cast fixation is applied. However, most cases accompany displacement of the avulsion fragment, and, usually, surgery is necessary to treat the displaced fracture. Although surgical fixation simply by cancellous screw or tension wire is widely used, fixation failure is potential complication in this method. Thus, this study wants to introduce a prospective and useful method that further strengthens the calcaneal fixation by using both cannulated screw and tension band wiring.
Displacement (Psychology) ; Incidence

In the study of craniofacial deformity, it is very important that identifying the factor which can affect the morphology and which is closely related to the morphology, because it can not only improve the comprehension of growth and developmental process but also be applied in growth prediction and treatment modality. Several investigators have already mentioned the characterstics of head posture and airway space in relations to morphologic difference. But it is very meaningful work in clarifying the correlation between morphology, head posture and airway space that observing the change of head posture after morplologic change caused by operation and the change of airway space after same procedure. To investigate above correlation, I selected normal group which is consisted of 43 adults and mandibular prognathism group which is consisted of 47 adults who had been operated by sagittal split ramus osteotomy and were followed up more than 1 year. With their lateral skull radiograghs, reference lines which can evaluate each measuring points and areas without effect of postural change were first determined. And using above reference lines, change of airway space, positional change of tongue and hyoid, change of cranial and cervical angulations were measured. The results obtained from the study were as follows 1. In the change of head posture, the position of tongue and hyoid neighboring to pharynx is more closely related to the reference line of cervical column than to reference line of cranium. 2. After mandibular setback operation, the airway dimension was decreased to 81.6% of preoperative state at 1 month postoperatively and was slightly increased to 89.7% at 1 year postoperatively. 3. Posterior movement of tongue plays important role in decrease of airway dimension and inferior movement of hyoid was closely correlated with posterior movement of tongue. 4. Postoperative anterior movement of mandible, namely, morphologic relapse had correlation with relapse phenomenon of airway dimension. 5. Craniocervical angulation increased postoperatively. Especially in the postoperative early state, there was increased foreward inclination of cervical angulation rather than increase of cranial angulation. But at postoperative 1 year it was observed that cervical inclination was returned to preoperative state and cranial angulation was increased gradually. 6. Increase rate of airway dimension was correlated with the increase of cranial angulation from postoperative 1 month to 1 year. In conclusion, relapse tendency of airway dimension following increase of cranial angulation was found after mandibular setback operation and it is considered that increase of cranial angulation is one of compensatory mechanism in airway maintenance.
Adult ; Comprehension ; Congenital Abnormalities ; Growth and Development ; Head ; Humans ; Mandible ; Osteotomy, Sagittal Split Ramus ; Pharynx ; Posture ; Prognathism ; Recurrence ; Research Personnel ; Skull ; Tongue