Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

PURPOSE:It is important to evaluate the healing process of avascular necrosis (AVN) involving femoral head after treatment. The purpose of this study was to assess the usefulness of pinhole bone scintigraphy in the AVN of femoral head after surgery. MATERIALS AND METHODS: We analyzed the changing pattern of pinhole bone scintigram in 21 femoral heads of 16 patients (14 lesions/11 male, 7 lesions/5 female, mean age: 39.4 yrs) before and after multiple drilling or vascularized bone grafting for AVN of the femoral head. In all patients, pre-operative scintigrams were obtained at 1 to 3 months before treatment and the first post-operative scintigrams were obtained at 1 to 3 months after treatment. All patients were followed for 2 to 4 years after operation. RESULTS: The findings of the pinhole scintigrams were divided into three patterns: 1) curvilinear, 2) scattered spotty and 3) undetermined. The 10 of 11 lesions with curvilinear pattern had good postoperative clinical and radiological follow-up findings. However, all 6 lesions with scattered spotty pattern showed poor postoperative findings, which necessitated total hip joint replacement. Of the 4 lesions with undetermined pattern, 2 required total hip joint replacement. There was significant difference in postoperative prognosis between the curvilinear and scattered spotty patterns (p<0.05). CONCLUSION: We conclude that the pattern of pinhole bone scintigram obtained within 1 to 3 months after multiple drilling or vascularized bone graft operation is a useful prognostic indicator in the AVN of femoral head.
Bone Transplantation ; Female ; Follow-Up Studies ; Head* ; Hip Joint ; Humans ; Male ; Necrosis* ; Prognosis* ; Radionuclide Imaging* ; Technetium Tc 99m Medronate ; Transplants*

PURPOSE: To evaluate the effectiveness of digital rectal-compression immediately after transrectal prostate biopsy (P-bx) for improving the accuracy of prostate cancer (PCa) staging. MATERIALS AND METHODS: Between July 2008 and June 2010, 94 consecutive patients who had a radical prostatectomy were included in our retrospective analysis. The exclusion criteria included a history of previous P-bx and surgery, a biopsy performed in another hospital, a number of biopsy cores different from 12, or a condition interfering with bleeding assessment. The subjects were divided into two groups, compression and non-compression. All enrolled patients took magnetic resonance imaging (MRI) for PCa staging. RESULTS: The compression and non-compression groups were comparable with respect to several baseline characteristics. However, the total hemorrhage score of intraprostatic bleeding was significantly different between the groups, even with adjustment for the time from biopsy to MRI (compression:15.4+/-2.32, non-compression: 24.9+/-2.43, p<0.001). The intra-prostatic cancer location matching rate was higher in the compression group (78.0%) than in the non-compression group (70.2%) (p=0.011). Overall accuracy of staging in compression and non-compression groups was 84.7% and 77.3%, respectively. CONCLUSION: Our results demonstrate that digital rectal compression performed immediately after prostate biopsy to reduce intraprostatic hemorrhage improves the accuracy for detection of PCa using MRI.
Aged ; *Biopsy ; Biopsy, Needle ; *Digital Rectal Examination ; Hemorrhage/prevention & control ; Hemostasis ; Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Staging/*methods ; Pressure ; Prostate/pathology ; Prostatic Neoplasms/*diagnosis/*pathology ; Reproducibility of Results ; Retrospective Studies

No abstract available.
Chest Pain* ; Emergencies* ; Emergency Service, Hospital* ; Thorax*

No abstract available.
Acute Kidney Injury* ; Edema* ; Hemofiltration*

No abstract available.
Acute Kidney Injury* ; Edema* ; Hemofiltration*

We reviewed the magnetic resonance imaging (MRI) of intracranial teratoma to detect the characteristic findings. Five patients with intracranial teratoma were evaluated with MRI from January 1988 to December 1990. Four male and one female patients with age ranging from 6 to 14 years were surgicelly confirmed MRI was done with 2.0T superconducting system(Spectro 20000, Goldstar, Seoul) and gadolinium enhancement was done in 4 patients. We retrospectively reviewed the clinical, operative, pathologic, and radiologic findings. Intracranial teratomas showed fatty component, calcification and cystic component filled with sebum or serous fluid. Peripheral rim enhancement was seen in three and irregular enhancement in two cases. There were hydrocephalus in all teratoma, but neither surrounding edema nor surrounding tissue invasion was seen. Low signal intensity rim was noted around the solid component of all tumors and they were not enhanced. MRI showed multicomponent of intracranial teratoma including the solid and cystic component, and low singal rim which might be the characteristic findings of the intracranial teratoma.
Edema ; Female ; Gadolinium ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging* ; Male ; Retrospective Studies ; Sebum ; Teratoma*

PURPOSE: To evaluate the usefulness of spiral CT in predicting the depth of tumor invasion in patients with gastric cancer by comparing with histopathological finding. MATERIALS AND METHODS: We studied spiral CT scans of forty-eight patients, in whom gastric cancer was proven by gastrofibroscopic biopsy and surgery. After distending the stomach with 400ml tap water or effervescent granules, 100ml of contrast media was given intravenously at a rate of 3ml/sec. CT scanning was started at 45 sec after administration of the contrast material. Gastric tumors were subdivided into five types according to enhancing pattern from the inner layer of gastric wall to the outer layer. These pattern were correlated with histopathologic results. RESULT: The tumor masses were detected on CT scan in 10(77%) of 13 patients with early gastric cancer, while, the tumor masses were seen on CT scan in all patients with advanced gastric cancer. Of 9 patients with type 1 or type 2 enhancing pattern, early gastric cancer(T1) were proven in 7 patients(78%), serosal invasion (T3) in 2 patients(22%). In contrast, among 29 patients with type 4 and type 5, 22 patients(76%) were proven as serosal invasion(T3). Of 7 patients with type 3, 3 patients(43%) were proven as serosal invasion(T3), three(43%) as subserosal invasion(T2), one as early gastric cancer(T1). CONCLUSION: Analysis of morphological enhancing pattern on spiral CT is useful in predicting the depth of tumor invasion in patients with gastric cancer.
Biopsy ; Contrast Media ; Humans ; Stomach ; Stomach Neoplasms ; Tomography, Spiral Computed* ; Tomography, X-Ray Computed ; Water

This study is concerned with a comparison of lower tibial and fibular epiphyseal growth and deformities of the ankles in normal and paralysed children. The data were based on 87 children with residual paralysis due to poliomyelitis an 20 non-paralysed children who were admitted to Severance Hospital and Sam Yook Children's Rehabilitation Center. The difference in level between the lower tibial and fibular ends and between the lower epiphysial lines of the tibia and fibula in X-rays of the ankles were measured in order to investigate the growth pattern of the lower tibio-fibular epiphysis. The difference between the level of the lower ends of the fibula was an avergage of 15.3mm in the normal and 6.2mm in paraysed children. Thus the fibular ends in paralysed limbs was an average of 9.1mm less than that of the normal. The difference between the level of the lower epiphyseal lines of the tibia and the fibula was an average of 13.7mm in normal and 5.5mm in paralysed children. Therefore, the level of the fibular epiphyseal lines in paralysed limbs was sn average of 8.2mm less than that of normal. In paralysed limbs, the lower tibial epiphyses showed lateral epical wedge shaped deformities. The tilting angle of the ankles and the wedging angle of the of the lower tibial epiphyses were measured in erder to investigate the degree of deformities of the ankles and the lower tibial epiphyses in paralysed limbs. The tilting angle of the ankles averaged 1.3° in normal and 7.6° in paralysed limbs. The wedging angle of the lower tibial epiphyses averaged 3.7° in normal and 11.5° in paralysed limbs. In paralysed children, muscle strength examination was performed to clarify the relationship between the degree of growth disturbance and the severity of the muscular paraysis. This lead us to conclude that: 1. In paralysed children, the growth disturbance of the lower fibular epiphyses was greater than that of the lower tibial epiphyses. 2. The difference of the growth disturbance between the tibial and fibular epiphyses was not due to muscle imbalance of the foot invertors and evertors but to muscle weakness. 3. In paraysed children, the shape of the lower tibial epiphyses changed to a wedge shape, a deformity which might be considered secondary to growth disturbance of the fibula. 4. In paralysed children, the lower tibial ends were tilted upward and laterally. This was thought to be the cause of valgus deformities of the ankles. 5. The relationship between the degree of muscle paralysis and the deformities of the ankles was not proved in this study.
Ankle ; Child ; Congenital Abnormalities ; Epiphyses ; Extremities ; Fibula ; Foot ; Humans ; Muscle Strength ; Muscle Weakness ; Paralysis ; Poliomyelitis ; Rehabilitation Centers ; Tibia

PURPOSE: There are few data regarding the epidemiology of hereditary or familial prostate cancer (PCa) in East Asians, especially in Korean men. Therefore, we evaluated the incidence of familial and hereditary PCa and the relation between socioeconomic status and the incidence of nonsporadic prostate cancer (NSPC). MATERIALS AND METHODS: We collected data from all patients who were treated for PCa at our center between November 2009 and January 2010. All patients were either newly diagnosed or had been diagnosed with PCa and seen as outpatients during the study period. RESULTS: In a sample of 218 patients with PCa; 25 (11.5%) were NSPC patients, and 193 (88.6%) were sporadic PCa sporadic prostate cancer (SPC) patients. Overall, 11.5% of the patients had a positive family history. There was one hereditary PCa family (three patients, 1.4%) and 11 familial PCa families (22 patients, 10.1%). Patients were divided into three different age groups. Of these, 18 (9.3%) SPC patients and 6 (24%) NSPC patients were diagnosed with the disease at the age of 55 years or younger (p=0.02). Prostate-specific antigen (PSA) levels in the NSPC group were significantly higher than in the SPC group (7.2+/-3.2 versus 6.3+/-4.9 ng/ml, p=0.042). SPC patients had larger waist circumferences than did NSPC patients (p=0.041). There were no significant differences between the SPC and NSPC groups in terms of socioeconomic status, Gleason score, pathological stage, or pathologic Gleason grade. CONCLUSIONS: East Asian NSPC patients are diagnosed at earlier ages than are SPC patients, even though the incidence of NSPC in the East Asian population is lower than in Western men.
Asian Continental Ancestry Group ; Humans ; Incidence ; Male ; Neoplasm Grading ; Outpatients ; Passive Cutaneous Anaphylaxis ; Pilot Projects ; Prostate ; Prostate-Specific Antigen ; Prostatic Neoplasms ; Siblings ; Social Class ; Waist Circumference