Chronic progressive radiation myelopathy(CPRM) is a rare but serious complication of radiation therapy. It's exact cause is unknown and the diagnosis is usually made based on the exclusion of other causes of myelopathy. Magnetic resonance imaging(MRI) with gadolinium- diethylenetriamine pentaacetic acid(DTPA) enhancement seems to be useful for the diagnosis of CPRM. There is no known effective treatment and the complication is irreversible. We report a case of CPRM after radiation therapy for subglottic cancer which was not respond to high-dose steroid therapy with review of literature.
Diagnosis ; Spinal Cord Diseases*

Prader-Willi syndrome(PWS) was first described by Prader et al in 1956. This syndrome is characterized by diminished fetal activity, low birth weight, infantile hypotonia with feeding problem, temperature instability, early onset of childhood hyperphagia with consequent obesity, short stature, hypogonadism and mental retardation. The deletion of chromosome 15(del 15(qll-13)) was reported by Ledbetter in 1981, which was thought to be of paternal origin. Recently, such micro- deletion may be diagnosed by fluorescence in situ hybridization(FISH) that recognizes specific DNA base sequence. We experienced a Prader-Willi syndrome confirmed by FISH in an infant that had hypotonia, growth retardation, feeding difficulty and FUO since 1 month of age. We report this case with a brief review and related literature.
Base Sequence ; DNA ; Fetal Movement ; Fluorescence ; Humans ; Hyperphagia ; Hypogonadism ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Muscle Hypotonia ; Obesity ; Prader-Willi Syndrome*

Fryns syndrome is a lethal syndrome of multiple congenital anomalies first described by Fryns et al in 1979. A recently developed major diagnostic criteria includes abnormal face, small thorax with widely spaced hypoplastic nipples, distal limb and nail hypoplasia, lung hypoplasia with diaphragmatic hernia, central nervous system anomalies and congenital heart disease. The pathogenesis of Fryns syndrome is not clear. Of the major immediate life-threatening abnormalities of this syndrome, lung hypoplasia associated with diaphragmatic hemia has usually proven to be fatal. We report a case of Fryns syndrome, which has the prenatal ultrasonographic findings of Dandy-Walker malformation and renal hypoplasia.
Central Nervous System ; Dandy-Walker Syndrome ; Extremities ; Heart Defects, Congenital ; Hernia, Diaphragmatic ; Lung ; Nipples ; Thorax

Cyclins are proteins that activate different cyclin-dependent kinases(CDKs) and promote the cell cycles. Their correlations with several human cancers have been identified. Cyclin E, as one of G1 cylins, produces DNA replication through the progression of cell cycle G1 --> S phase. In contrast, cyclin-dependent kinase inhibitors(CDKI) bound with cyclin E-cdk2 complex control the cell cycle and inhibit the cell proliferation. P21(WAF1) proteins, which are CDKIs, are transcripted by a p53 gene and participate in the cell cycle inhibition. Variant p53 proteins produced by a mutated p53 gene lose the ability to control of the cell cycle resulting in cell proliferation. This study is aimed to reveal the expressions of cyclin E, p21(WAF1) protein, p53 variant protein in colorectal adenomas and carcinomas, and also reveal their correlations in the process of carcinogenesis. Twenty-one colorectal adenomas or adenomatous polyps, and thirty colorectal carcinoma tissues were obtained by operative resections or endoscopic polypectomies. Immuno histochemical stains of the above-mentioned three proteins and a statistical analysis of their correlations were made. The results were as follows: 1. P21 proteins were expressed in the upper-one third layer of all normal colonic mucosa, but cyclin E and variant p53 protein were not identified. 2. Cyclin E was expressed in 23.8% of adenomas and 76.7% of carcinomas. Variant p53 protein was expressed in 71.4% of adenomas and 83.3% in carcinomas. The degree of positivity of variant p53 expression was correlated with cancer staging. P21 protein was expressed in all adenomas, similar to normal mucosa, but was not expressed in 43.3% of carcinomas. 3. Expression of cyclin E was increased as to the positivity of variant p53 proteins but the correlations of p21 proteins and cyclin E, and p21 proteins and variant p53 proteins were not identified. Cancer staging was not correlated with the expressions of the three proteins. In conclusion, it can be thought that the overexpression of cyclin E and variant p53 proteins, and the loss of p21 proteins are related with the colorectal carcinogenesis. We can also identify the relationship of cyclin E and variant p53 proteins.
Adenoma* ; Adenomatous Polyps ; Carcinogenesis ; Cell Cycle ; Cell Proliferation ; Colon ; Colorectal Neoplasms ; Coloring Agents ; Cyclin E* ; Cyclins* ; DNA Replication ; Genes, p53 ; Humans ; Mucous Membrane ; Neoplasm Staging ; Phosphotransferases ; S Phase

OBJECTIVE: The purpose of this study was to determine the efficacy of short segment fixation following postural reduction for the re-expansion and stabilization of unstable burst fractures in patients with osteoporosis. METHODS: Twenty patients underwent short segment fixation following postural reduction using a soft roll at the involved vertebra in cases of severely collapsed vertebrae of more than half their original height. All patients had unstable burst fracture with canal compromise, but their motor power was intact. The surgical procedure included postural reduction for 2 days and bone cement-augmented pedicle screw fixations at one level above, one level below and the fractured level itself. Imaging and clinical findings, including the level of the vertebra involved, vertebral height restoration, injected cement volume, local kyphosis, clinical outcome and complications were analyzed. RESULTS: The mean follow-up period was 15 months. The mean pain score (visual analogue scale) prior to surgery was 8.1, which decreased to 2.8 at 7 days after surgery. The kyphotic angle improved significantly from 21.6+/-5.8degrees before surgery to 5.2+/-3.7degrees after surgery. The fraction of the height of the vertebra increased from 35% and 40% to 70% in the anterior and middle portion. There were no signs of hardware pull-out, cement leakage into the spinal canal or aggravation of kyphotic deformities. CONCLUSION: In the management of unstable burst fracture in patients with severe osteoporosis, short segment pedicle screw fixation with bone cement augmentation following postural reduction can be used to reduce the total levels of pedicle screw fixation and to correct kyphotic deformities.
Congenital Abnormalities ; Follow-Up Studies ; Humans ; Kyphosis ; Osteoporosis ; Spinal Canal ; Spine

OBJECTIVE: To investigate the effect of taping therapy on hemiplegic shoulder subluxation. METHOD: Sixty patients were randomly assigned to three groups. In study group, two parts of the first tape was shaped as letter Y and were attached from humeral insertion of deltoid muscle to extension line of the clavicle midline, following anterior and middle deltoid line, respectively, with 125% elasticity. And two parts of the second tape as same as the first tape were attached horizontally from head of greater tubercle to medial border of scapular, following supraspinatus and infraspinatus muscles, respectively, with 125% elasticity. The placebo group was applied by the tape with 100% and 150% elasticity and control group was not applied. The effects of strapping were evaluated by using visual analogue scale (VAS), passive range of movement (ROM), vertical distance (VD) and horizontal distance (HD) on radiologic findings of plain anteroposterior (AP) view and lateral distance (LD) on shoulder ultrasonography at entry (day 0), 1 and 2 days later. RESULTS: Repeated measured analysis of variances indicated that the study group showed corrective effect in the VAS (p<0.05). The control and placebo groups showed no significant change in any parameters of distance measurements and clinical findings. In every group, spasticity, MMT and Brunnstrom stage were not changed during study. CONCLUSION: The taping therapy with 125% elasticity may be helpful for patients with hemiplegic shoulder to reduce pain.

OBJECTIVE: To evaluate the autonomic nervous function in post-stroke patients. METHOD: A total of 58 subjects, 34 post-stroke patients (mean age of 61.35 years) and 24 healthy subjects (mean age of 34.42 years) were included. The sympathetic skin response (SSR), the R-R interval variation (RRIV) and orthostatic hypotension (OH) were evaluated. Bilateral sympathetic skin responses were recorded on the palm and sole with stimulation of both median and tibial nerves. The obtained SSRs were divided into normal response and abnormal response including no response. The R-R interval variations were estimated during rest, deep breathing and Valsalva maneuver respectively. RESULTS: In all healthy subjects, obtained SSRs were of normal response. Although the rate of abnormal SSR was 75.4% (205 of 272 waves) in stroke patients, the rate of abnormal SSR on affected side (81.6%, 111 of 136 waves) was significantly higher than unaffected side (69.1%, 94 of 136 waves) (p<0.05). The rate of abnormal SSR according to OH was not significantly different (with OH 75.0%, without OH 75.6%) (p>0.05). The RRIV of stroke patients with OH (1.09+/-0.06) was significantly lower than in control (1.14+/-0.05) during rest (p<0.01), and in stroke patients without OH, the RRIV (1.13+/-0.06) was also significantly lower than in control (1.27+/-0.22) during Valsalva maneuver (p<0.01). CONCLUSION: Evaluation of SSR and RRIV could be helpful in detecting dysfunction of autonomic nervous system in post-stroke patients.
Autonomic Nervous System ; Humans ; Hypotension, Orthostatic ; Respiration ; Skin ; Stroke ; Tibial Nerve ; Valsalva Maneuver

The purpose of this study was to describe the CT findings of focal organizing pneumonia and to compare the findings with pathology. CT findings of histologically proven focal organizing pneumonias in 26 consecutive patients were analyzed. In 17 patients who had undergone surgical resections, the findings were correlated with pathology. Focal organizing pneumonias appeared as a nodule (n= 13) or a mass (n=13), ranging from 9 mm to 66 mm in diameter. Ground-glass opacity was seen in 6/13 (46%) nodules and 6.5/13 (50%) masses (k=.48) with an extent ranging from 5% to 75% (mean, 16%). In 4/26 (15%) patients, the extent was more than 50% of the lesion. They showed smooth (n=4), lobulated (n=8), spiculated (n=1), or lobulated and spiculated margin (n=13). On correlative analysis, nodule or mass on CT consisted histologically of intraalveolar exudate or microabscess, chronic inflammatory cell infiltration, fibrotic nodules, and polypoid granulation tissue in the alveolar or bronchiolar spaces. Ground-glass opacity consisted of interstitial fibrosis and chronic inflammatory cell infiltration and intraalveolar polypoid granulation tissue. Focal organizing pneumonia may simulate a lung cancer with variable appearances on CT and the findings reflect underlying histopathology of the disease.
Adult ; Aged ; Aged, 80 and over ; Female ; Human ; Male ; Middle Age ; Pneumonia/pathology/*radiography ; *Tomography, X-Ray Computed

Tracheo-innominate artery fistula (TIAF) is rare, yet the most fatal complication after tracheostomy. In the absence of immediate diagnosis and surgical management, the mortality rate is very high, because the complication can lead to sudden massive tracheal hemorrhage. Tracheal obstruction and hypovolemic shock are the major life threatening conditions. The 46-year-old woman received tracheostomy tube insertion after stroke. Three months later, there was occurrence of active bleeding at the site of tracheostomy in the patient, who participated in comprehensive rehabilitation program. Immediately, the patient received an endotracheal tube insertion into the tracheostomy site and thus massive bleeding was controlled. The patient was transferred to the intensive care unit, where her breathing was maintained by mechanical ventilation. Based on computed tomography and laryngoscopy, no remarkable findings about TIAF were detected. Nevertheless, transfemoral angiography findings revealed that innominate artery made small luminal outpouching to trachea at the carotid artery and at the subclavian artery bifurcation level, based on which a diagnosis of TIAF was made. She had an operation for TIAF, tracheoplasty with bypass graft. Subsequently, she was discharged after 15 weeks. In the present report, we describe a case of TIAF, which can occur in the patients with tracheostomy tube during rehabilitation.
Angiography ; Arteries ; Brachiocephalic Trunk ; Carotid Arteries ; Female ; Fistula ; Hemorrhage ; Humans ; Intensive Care Units ; Laryngoscopy ; Phenobarbital ; Respiration ; Respiration, Artificial ; Shock ; Stroke ; Subclavian Artery ; Trachea ; Tracheostomy ; Transplants

PURPOSE: A fistula in ano is a granulating track between the anorectum and the perianal region or perineum. A typical fistula may consist of the track, the primary (internal) opening and secondary (external) opening. Its hallmark is frequent discharge through external opening. Sometimes this track become occluded and a sinus remains. Therefore abscence of an external opening should not be taken as evidence that there is no fistula, and then this study is aimed to make clear perianal sinus as another form of fistula in ano. METHODS: This study is a clinical analysis of 17 patients with perianal sinus, who were treated at the Department of General Surgery of Pundang-CHA hospital from June, 1995 to December, 1998. As comparative group, 91 patients of typical fistula in ano were also taken in same period. RESULTS: The ratio of male to female was 4.6:1 in sinus group, 4.1:1 in fistula group. The prevalent ages were in the third decade in both group. The main symptoms of sinus group were a palpable mass (94.1%), pain & discomfort (35.3%) but that of fistula group were purulent discharge (87.9%) and pain (28.6%). The most frequent duration of illness was 6 months~1 year (52.9%) in sinus group, within 6 months (40.7%) in fistula group. The distributions of sinus size were 7 cases (41.2%) in <1 cm, 8 cases (47.0%) in 1~2 cm and 2 cases (11.8%) in >2 cm. The most common type was intersphincteric in both group. The operative procedures included excision with fistulectomy in 4 cases (23.5%), lay open of sinus with fistulotomy in 11 cases (64.7%), seton procedure in 2 cases (11.8%). The mean hospital stay was 5.0 day in sinus group, 4.9 day in fistula group. CONCLUSIONS: There was no distinct difference in the clinical features or characteristics between the both groups, therefore perianal sinuscould be taken as fistula in ano and treated similar procedures.
Female ; Fistula* ; Humans ; Length of Stay ; Male ; Perineum ; Surgical Procedures, Operative