No abstract available.
Idiopathic Pulmonary Fibrosis*

No abstract available.
Fanconi Anemia*

High tibial osteotomy is a useful procedure in the treatment of early stage osteoarthritis of the knee which is confined to the medial compartment. But long term follow-up study of high tibial osteotomy has not been provided yet and its prerequisite for a successful result still remains unrevealed. Since the osteoarthritis can progress after the osteotomy, it may result in the loss of correction and recurrence of pain and require conversion to total knee replacement arthroplasty. We studied clinically and radiologically the long term result of high tibial osteotomy in 11 patients(16 knees) who had been treated at the Department of Orthopaedic surgery of Seoul Paik Hospital during the period from January, 1978 to May, 1989, and follow-up for longer than 4 years. Preoperative mean varus angle was 6.4 degrees and immediate postoperative mean valgus angle was 8.6 degrees and final mean valgus angle was 4.7 degrees. The average loss of correction angle was 3.9 degrees. At 2-year follow-up, the results of 16 knees were as follows; excellent in six knees, good in seven knees. The final results at average 7.2-year follow-up were excellent in four knees, good in six knees and one knee was converted to total knee replacement arthroplasty. The results showed deterioration of clinical results and loss of correction with time after osteotomy. However, in more than half of the cases the results were good or excellent at the final follow-up, We, therefore, conclude that high tibial osteotomy is still a useful procedure in osteoarthritis of the knee if performed with a precise surgical technique and an adequate overcorrection more than 10 degrees.
Arthroplasty ; Arthroplasty, Replacement, Knee ; Follow-Up Studies ; Knee ; Osteoarthritis ; Osteotomy ; Recurrence ; Seoul

A case of systemic amyloidosis involving the upper and lower gastrointestinal tract is presented. The initial manifestation of this case was bloody diarrhea. On colonoscopy, multiple submucosal hematomas and irregular ulcerations of the sigmoid and descending colon were found. The pathologic diagnosis was confirmed by an endoscopic mucosal biopsy of the gastrointestinal tract and the specimen revealed massive amyloid deposits in the wall of the upper and lower intestinal tract. With intensive medical treatment, the submucosal hematoma disappeared and the ulcerations decreased in size. However, on the 29th day, the patient was expired due to unexpected sepsis.
Amyloidosis* ; Biopsy ; Colon, Descending ; Colon, Sigmoid ; Colonoscopy ; Diagnosis ; Diarrhea ; Gastrointestinal Tract ; Hematoma* ; Hemorrhage* ; Humans ; Lower Gastrointestinal Tract* ; Plaque, Amyloid ; Sepsis ; Ulcer

OBJECTIVE: Multipotent bone marrow stromal cells have the ability to differentiate toward a variety of connective tissue lineages including cartilage. The future use of adult mesenchymal stem cells (MSCs) for human therapies depends on the establishment of preclinical studies. Therefore, in this preclinical study we demonstrated the expression of MSC surface markers CD29, CD105, and CD44 on human bone marrow derived stromal cells during chondrogenic differentiation. METHODS: Adult human bone marrow was collected from the iliac crest of 7 donors following informed consent. Mononuclear cells were isolated, incubated in monolayers, and embedded in alginate beads for three-dimensional cultures. Cellualr viability was assessed by MTT assay. Flow cytometry of alginate bead cultures was performed on days 0, 7, 14, 21, and 28 using monoclonal antibody against surface molecules, CD105, CD29, CD44, CD34 and CD45. Total contents of collagen and glycosaminoglycan (GAG) of the alginate beads was measured. SPSS 11.0 was used for data analysis. RESULTS: After 7 days of culture, 89% of the cells expressed the human integrin beta 1 antibody, CD29. The CD29-positive cells remained elevated at 83% on days 28. However, while only 18% expressed the type II TGF-beta receptor endoglin, CD105 on day 7, the CD105-positive cells increased abruptly 65% on day 14 remaining elevated up to day 28. The expression of CD44 was maximal in the first passage cell (63%). High concentration of TGF-beta 3 (10 ng/mL) was more favorable for sustaining cell viability than a low concentration (0.5 ng/mL)(n=4, p= 0.002, day 21). The total contents of collagen and GAG in the MSC-alginate beads increased during the three-dimensional culture (n=4, p=0.02, p=0.006) suggesting its differentiation into a chondrogenic lineage. CONCLUSION: CD29 was expressed earlier than CD105 during chondrogenic differentiation of human bone marrow MSC. CD44 expression was highest in the first passage cells and gradually decreased afterwards.
Adult ; Bone Marrow* ; Cartilage ; Cell Survival ; Collagen ; Connective Tissue ; Flow Cytometry ; Humans* ; Informed Consent ; Mesenchymal Stromal Cells ; Receptors, Transforming Growth Factor beta ; Statistics as Topic ; Stromal Cells* ; Tissue Donors ; Transforming Growth Factor beta

Spontaneous intracranial hypotension (SIH) is a rare disease with a symptom of a postural headache, which is aggravated by the erect position and relieved by the supine position. This persistent headache is dramatically improved with an epidural blood patch. C6-7 is the most common site of leakage of cerebrospinal fluid (CSF) from the subarachnoid space in SIH. The diagnosis of SIH is based on a typical history. It is supported by low CSF pressure in a lumbar puncture and diffuse dural enhancement on a brain MRI, and confirmed by a CSF leakage site on a radioisotope cisternography or on a myelography. However, we had two patients of SIH whose CSF leakage site was not found on cisternography. We tried an epidurogram just before an injection of autologous blood for an epidural blood patch, and found specific finding on the epidurogam suggesting the CSF leakage site, such as a filling defect on C6-7 during dye injection, and delayed washout of dye around the filling defect on the epidurogram performed at C7-T1/C6-7. Therefore, we suggest that an epidurogram is occasionally a useful diagnostic tool when looking for the site of CSF leakage in SIH.
Blood Patch, Epidural ; Brain ; Cerebrospinal Fluid ; Diagnosis ; Headache ; Humans ; Intracranial Hypotension* ; Magnetic Resonance Imaging ; Myelography ; Rare Diseases ; Spinal Puncture ; Subarachnoid Space ; Supine Position

PURPOSE: To observe the hemodynamic change in brain tumors and peritumoral edemas after steroid treat-ment, and then nvestigate the clinical usefulness of perfusion MRI. MATERIALS AND METHODS: We acquired conventional and perfusion MR images in 15 patients with various in-tracranial tumors (4 glioblastoma multiformes, 4 meningiomas, 3 metastatic tumors, 1 anaplastic ependymo-ma, 1 anaplastic astrocytoma, 1 hemangioblastoma, and 1 pilocytic astrocytoma). For perfusion MR imaging, a 1.5T unit employing the gradient-echo EPI technique was used, and further perfusion MR images were ob-tained 2-10 days after intravenous steroid therapy. After processing of the raw data, regional cerebral blood volume (rCBV) maps were reconstructed. The maps were visually evaluated by comparing relative perfusion in brain tumors and peritumoral edemas with that in contralateral white matter. Objective evaluations were performed by comparing the perfusion ratios of brain tumors and peritumoral edemas. RESULTS: Visual evaluations of rCBV maps, showed that in most brain tumors (67%, 10/15), perfusion was high before steroid treatment and showed in (80%, 12/15) decreased afferwards. Objective evaluation, showed that in all brain tumors, perfusion decreased. Visual evaluation of perfusion change in peritumoral edemas revealed change in only one case, but objective evaluation indicated that perfusion decreased signifi-cantly in all seven cases. CONCLUSIONS: rCBV maps acquired by perfusion MR imaging can provide hemodynamic information about brain tumors and peritumoral edemas. Such maps could prove helpful in the preoperative planning of brain tumor surgery and the monitoring of steroid effects during conservative treatment.
Astrocytoma ; Blood Volume* ; Brain Neoplasms* ; Brain* ; Edema* ; Glioblastoma ; Hemangioblastoma ; Hemodynamics ; Humans ; Magnetic Resonance Imaging* ; Meningioma ; Perfusion* ; Steroids

PURPOSE: To observe the hemodynamic change in brain tumors and peritumoral edemas after steroid treat-ment, and then nvestigate the clinical usefulness of perfusion MRI. MATERIALS AND METHODS: We acquired conventional and perfusion MR images in 15 patients with various in-tracranial tumors (4 glioblastoma multiformes, 4 meningiomas, 3 metastatic tumors, 1 anaplastic ependymo-ma, 1 anaplastic astrocytoma, 1 hemangioblastoma, and 1 pilocytic astrocytoma). For perfusion MR imaging, a 1.5T unit employing the gradient-echo EPI technique was used, and further perfusion MR images were ob-tained 2-10 days after intravenous steroid therapy. After processing of the raw data, regional cerebral blood volume (rCBV) maps were reconstructed. The maps were visually evaluated by comparing relative perfusion in brain tumors and peritumoral edemas with that in contralateral white matter. Objective evaluations were performed by comparing the perfusion ratios of brain tumors and peritumoral edemas. RESULTS: Visual evaluations of rCBV maps, showed that in most brain tumors (67%, 10/15), perfusion was high before steroid treatment and showed in (80%, 12/15) decreased afferwards. Objective evaluation, showed that in all brain tumors, perfusion decreased. Visual evaluation of perfusion change in peritumoral edemas revealed change in only one case, but objective evaluation indicated that perfusion decreased signifi-cantly in all seven cases. CONCLUSIONS: rCBV maps acquired by perfusion MR imaging can provide hemodynamic information about brain tumors and peritumoral edemas. Such maps could prove helpful in the preoperative planning of brain tumor surgery and the monitoring of steroid effects during conservative treatment.
Astrocytoma ; Blood Volume* ; Brain Neoplasms* ; Brain* ; Edema* ; Glioblastoma ; Hemangioblastoma ; Hemodynamics ; Humans ; Magnetic Resonance Imaging* ; Meningioma ; Perfusion* ; Steroids

No abstract available.

BACKGROUND AND OBJECTIVES: Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. SUBJECTS AND METHODS: A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Children's Hospital. RESULTS: Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). CONCLUSION: Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.
Aneurysm ; Aorta, Thoracic ; Arteries ; Cardiovascular Abnormalities ; Deglutition Disorders ; Early Diagnosis ; Heart Diseases ; Humans ; Magnetic Resonance Imaging ; Prognosis ; Pulmonary Artery ; Retrospective Studies ; Risk Factors ; Subclavian Artery ; Tracheal Stenosis