Wiskott-Aldrich syndrome is a rare inheritable disease. Though there are many reported cases in the world, there are only 3 reported cases in Korea. We experienced a 7 years old boy with Wiskott-Aldrich syndrome. The initial presentation was generalized petechiae due to thrombocytopenia. We diagnosed him to have idiopathic thrombocytopenic purpura and followed up him for several years. During follow-up the patient have been suffered from croup, perianal abscess, bronchitis, and otitis media. One year ago, inguinal mass developed and 3 months ago, cervical and axillary masses developed. The fine needle aspiration cytology and biopsy revealed angioimmunoblastic lymphadenopathy like atypical lymphoproliferative disorders associated with congenital immune deficiency. So we diagonsed the patient to have Wiskott-Aldrich syndrome through his clinical course and laboratory findings including eczema, persistent low platelet counts, low mean platelet volume, monoclonal gammopathy on serum electrophoresis and biopsy finding.
Abscess ; Biopsy ; Biopsy, Fine-Needle ; Bronchitis ; Child ; Croup ; Eczema ; Electrophoresis ; Follow-Up Studies ; Humans ; Immunoblastic Lymphadenopathy ; Korea ; Lymphoproliferative Disorders ; Male ; Mean Platelet Volume ; Otitis Media ; Paraproteinemias ; Platelet Count ; Purpura ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia* ; Wiskott-Aldrich Syndrome*

Localized nodular myositis is an uncommon benign inflammatory myopathy of unkonwn cause affecting skeletal muscle and, presenting as a localized painful swelling within the soft tissue of an extremity. Histological examination reveals lymphocytic infiltration, scattered muscle fiber necrosis and regeneration, and interstitial fibrosis. MRI finding is an enhancement with increased signal intensity around the lesion. We report two cases of localized nodular myositis presenting as pseudothrobothrombophlebitis. We believe this is the first case report of localized nodular myositis in Korea.
Extremities ; Fibrosis ; Korea ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Myositis* ; Necrosis ; Regeneration

During the last ten years, 7.384 patients with diabetes mellitus were managed at Seoul National University Hospital. Among them 1,026 patients (13.9%) were diagnosed as diabetic neuropathy and 84 (1.1%) as diabetic neurogenic bladder with severe voiding dysfunction. We analyzed clinical informations. laboratory and radiologic findings. results of urodynamic study and treatment modalities and outcomes. Thirty one patients were eligible, including 13 men and 18 women. The mean duration of diabetes was 9.4 years before severe voiding dysfunctions developed. Significant amount of residual urine was detected in all patients ranging from 300cc to 1,900 cc. Urodynamic study was done in 16 patients, all showed flaccid neurogenic pattern with increased bladder capacity over 500cc. Bacteriuria was found in 24 patients (77.4%), including 8 mixed infections. Hydronephrosis was identified in 7 out of 11 radiologically evaluated patients. Other diabetic chronic complications were found in most of the patients. diabetic retinopathy in 24 (77.4%). nephropathy in 13 (41.9%). and peripheral neumpathy in 24 (77.4%). They were managed successfully with timed voiding and double voiding in 2 patients, and urecholine medication with or without phenoxybenzamine in 6. and intermittent catheterization in l8. Education of the patients and high suspicion and full recognition of medical persons about diabetic neurogenic bladder is necessary for decrement of the development of diabetic neurogenic bladder, for early detection and prevention of severe complication such as renal and bladder impairment.
Bacteriuria ; Bethanechol Compounds ; Catheterization ; Catheters ; Coinfection ; Diabetes Mellitus ; Diabetic Neuropathies ; Diabetic Retinopathy ; Education ; Female ; Humans ; Hydronephrosis ; Male ; Phenoxybenzamine ; Seoul ; Urinary Bladder ; Urinary Bladder, Neurogenic* ; Urodynamics

INTRODUCTION AND OBJECTIVES: Retinoic acid (RA) is known as a potent chemopreventive agent in bladder tumor. Recently, RA has gained attention for up-regulation of transduced gene expression via long terminal repeat (LTR) transcriptional promotion. In this study, we investigated the possible dual effect of RA, growth inhibition and up-regulation of transduced gene expression which contains LTR promoter in human bladder carcinoma cell lines. MATERIALS AND METHODS: Human bladder carcinoma cell lines CY-24, J-82, HT-1197, ATCC) were transduced with Moloney murine leukemia virus containing cDNA of TNF-alpha. The growth of transduced and parent cell line was measured by tetrazolium based colorimetric assay (MTF). Transduced TNF-alpha gene expression was determined by ELISA method. RESULTS: TNF-alpha production was increased approximately twofold after treatment with RA (10 uM) in all three cell lines. This increase was dependent on RA concentration. RA treatment of transduced and parent cell line resulted in dose dependent inhibition of cell proliferation(up to 80% inhibitionwith 10 uM RA) in all parental and transduced cell lines. CONCLUSIONS: These results indicate that RA shows dual effect in cytokine gene transduced bladder carcinoma cells with retroviral vector containing LTR promoter and could be a supplement to the gene therapy of bladder cancer.
Cell Line* ; DNA, Complementary ; Enzyme-Linked Immunosorbent Assay ; Gene Expression* ; Genetic Therapy ; Humans* ; Moloney murine leukemia virus ; Parents ; Terminal Repeat Sequences ; Tretinoin* ; Tumor Necrosis Factor-alpha* ; Up-Regulation ; Urinary Bladder Neoplasms* ; Urinary Bladder* ; Zidovudine

The incidence of synchronous gastric cancer is variously reported to be 2.07%, 5.22%, 6.5%, 8.6%, or 9% and has been increasing recently, probably with advance in the diagnosis of gastric cancer. Our case in presentation is a 70-year-old male patient who had total gastrectomy and esopbagiojunostomy done for synchronous four gastric cancer. Each has different histologic types, Some of these lesions are not suspected before operation and are found almost by chance during histologic examinations after surgery performed only to remove main lesion. So we report this case with a review of literatures.
Aged ; Diagnosis ; Gastrectomy ; Humans ; Incidence ; Male ; Stomach Neoplasms*

PURPOSE: To evaluate the antitumor effect of the proapoptotic Bax gene in prostate cancer cells, in vitro, using a plasmid vector expressing the human Bax gene. MATERIALS AND METHODS: cDNA of the human Bax gene, amplified by RT-PCR, was cloned to pCR@3.1. The expression of the cloned Bax (pCR3.1-Bax) was observed by RT-PCR and Western blot analyses. The efficacy of growth inhibition by the cloned Bax gene was tested, in vitro, on PC-3 and DU145 human prostate cancer cell lines using the MTT assay. Immunoblot analysis for the expressions of Bcl-2 and Bcl-xL were performed. Assays were also performed to evaluate the apoptosis, DNA fragmentation and CPP32. RESULTS: The Bax protein was expressed in the parental PC-3 cells, but not in the DU-145 cells. The expressions of Bax mRNA in the transfected PC-3 and DU-145 cells had increased by 24 hr, and those of Bax protein in the transfected PC-3 and DU-145 cells had increased by 48 and 24 hr, respectively, compared with the control cell lines. The cytotoxicity of pCR3.1-Bax on PC-3 and DU-145 cells increased significantly compared with an empty vector, pCR3.1 (p<0.05, respectively). An increased cytotoxicity of the Bax-transfected cell lines was associated with enhanced apoptosis. The Bcl-2 protein was not expressed in the transfected cells, and the levels of Bcl-xL protein expression in transfected cells were no different to those in the parenteral cells. The Bax/Bcl-xL ratio was increased by the transfection of the Bax expression vector. CONCLUSIONS: Our results show that the cloned Bax-expression plasmid vector efficiently inhibits the growth of PC-3 and DU145 human prostate cancer cell lines. These data suggest that exogenous Bax expression may have therapeutic applications in prostate cancer.
Apoptosis* ; bcl-2-Associated X Protein ; bcl-X Protein ; Blotting, Western ; Cell Line ; Clone Cells ; DNA Fragmentation ; DNA, Complementary ; Gene Expression* ; Genetic Therapy ; Humans ; Parents ; Plasmids ; Prostate* ; Prostatic Neoplasms* ; RNA, Messenger ; Transfection

Endometriosis is a common gynecologic disease, it occur not only pelvic organ but also anywhere in human body. However inguinal endometriosis is a very rare condition, its incidence is 0.4% of entire endometriosis. Most of inguinal endometriosis are found as inguinal masses in women in 30s and 40s who have history of gynecologic surgery. Majority of the cases are accompanied with pelvic endometriosis. Authors report a case of inguinal endometriosis in patients who has no history of gynecological surgery.
Endometriosis ; Female ; Genital Diseases, Female ; Gynecologic Surgical Procedures ; Human Body ; Humans ; Incidence

PURPOSE: To assess the usefulness of CT angiography(CTA) compared with compared with conventional angiography(CA) in the evaluation of intracranial occlusive vascular disease. MATERIALS AND METHODS: We evaluated 26 patients with clinically suspected intracranial occlusive vascular disease studied with both CTA and CA. Incases where there was no vascular lesion on CA, we used CTA to retrospectively review the detection rate and size of individual vessels, and compared the findings with those obtained by CA. In cases of occlusive vascular lesion, we evaluated the degree of stenosis on CTA and compared this with the CA findings. We also measured the time taken to use both modalities. RESULTS: Sixteen patients had no vascular lesion and ten patients had occlusive vascularlesions. Compared with CA, CTA detected 97%(124/128) of normal intracranial arteies ; their diameter measured on CTA was slightly smaller than that on CA. On CTA, the degree of stenosis was correctly estimated in eight lesions, underestimated in one and overestimated in one. Examination time ranged between 15 and 20 minutes with CTA and between 40 and 60 minutes with CA. CONCLUSION: Compared with CA, CTA shows good correlation in the delineation of intracranial normal and occlusive vessels around the Circle of Willis. CTA may be an additional tool for the evaluation of the Circle of Willis in patients with suspected intracranial occlusive vascular disease. CTA may, additionally, be used as a follow-up method in patients with acute cerebral infarctions after thrombolytic therapy.
Angiography* ; Cerebral Infarction ; Circle of Willis* ; Constriction, Pathologic ; Humans ; Retrospective Studies ; Vascular Diseases*

Tardy ulnar nerve palsy might develop secondary to nonunion, malunion, or elbow deformity after medial epicondylar fracture of the humerus. We report a case of tardy ulnar nerve palsy following medial epicondylar fracture, treated with excision of bony fragment, neurolysis and relocation of the ulnar nerve.
Congenital Abnormalities ; Dislocations ; Elbow ; Humerus ; Ulnar Nerve ; Ulnar Neuropathies

PURPOSE: To review the result of a radical nephrectomy for renal cell carcinomas and investigate whether an ipsilateral adrenalectomy, during a radical nephrectomy, has a favorable prognostic effect in patients with renal cell carcinomas. MATERIALS AND METHODS: The medical records of 365 patients, who underwent a radical nephrectomy, between January 1995 and December 1999, were retrospectively reviewed. All patients had unilateral renal cell carcinomas, and nephrectomies were performed either with (adrenalectomy group, 193 patients) or without (non-adrenalectomy group, 172 patients) an ipsilateral adrenalectomy. The survival rate was assessed using the Kaplan-Meier method. In conjunction with a univariate analysis, a multivariate analysis was performed, using a Cox regression analysis, to determine the independent prognostic factors. RESULTS: The ages of the adrenalectomy and non-adrenalectomy groups ranged from 21 to 78 (mean age: 54.7 years) and 28 to 77 years (mean age: 54.0 years), respectively. The durations of the follow-up periods in the adrenalectomy and non-drenalectomy groups were 62.4 and 62.6 months, respectively. There were no significant differences in the clinicopathological characteristics, including the T stage, between the two groups. The 3-year survival rates of the adrenalectomy and non-adrenalectomy group were 82.8 and 91.2%, respectively, and the 5-year survival rates were 75.4 and 85.3%, respectively (p=0.095). In the adrenalectomy group, distant metastasis, high T-stage and adrenal involvement were identified as prognostic factors by the multivariate statistical analysis (p=0.002, p=0.008 and p<0.001, respectively). CONCLUSIONS: Our results reveal that an ipsilateral adrenalectomy, during a radical nephrectomy, does not improve the prognosis of patients with renal cell carcinomas.
Adrenalectomy* ; Carcinoma, Renal Cell* ; Follow-Up Studies ; Humans ; Medical Records ; Multivariate Analysis ; Neoplasm Metastasis ; Nephrectomy* ; Prognosis ; Retrospective Studies ; Survival Rate