Wilson's disease is associated with abnormal deposition of copper in the brain, liver, kidneys and other body tissues, apparently due to an inherited defect in copper metabolism. Clinically the disorder is manifested by signs and symptoms of basal ganglia disease, postnecrotic hepatic cirrhosis, Kayser-Fleischer rings, hypoceruloplasminemia, hypocupremia, hypouricemia, cupruresis, and aminoaciduria. The authors experienced a case of Wilson's disease showing the characteristic signs such as Kayser-Fleischer rings, dysarthria, dysphagia and muscular rigidity, without any signs of liver involvement. A review of this case is provided with the literature.
Basal Ganglia Diseases ; Brain ; Copper ; Deglutition Disorders ; Dysarthria ; Hepatolenticular Degeneration* ; Kidney ; Liver ; Liver Cirrhosis ; Metabolism ; Muscle Rigidity

PURPOSE: Acute immume thrombocytopenic purpura (ITP) is relatively common hematologic disease in children. Most acute ITP is recovered within 6 month spontaneously and the complication is rare. But 10~20% of the ITP patient became a chronic form. Infection with Epstein-Barr virus (EB virus) in developing country usually occurs during infancy and early childhood. Acute ITP associated with EB virus is likely to develop chronic ITP in current literatures. We studied the pattern of laboratory findings in long term follow up of ITP with EB virus infection. METHODS: One hundred and seventy nine patients diagnosed with ITP admitted to the division of pediatric hematology, Gachon Medical Center and Hanyang University Hospital between Mar. 1991 and Jun. 2001 were reviewed retrospectively. Serologic test for EB virus was available for 57 patients and 25 of them were follow up at least 6 months. Evidence of acute EB virus infection was defined as a positive Viral Capsid Antigen (VCA) IgM or positive Anti VCA IgG and negative Ebstein-Barr virus Nuclear Antigen (EBNA). Complete remission (CR) was defined as a recovery of platelet count of more than 100 109/L and partial remission (PR) as a recovery of platelet count of 50~100 109/L, maintained for at least 6 months. RESULTS: Sixteen out of 57 patients were associated acute EB virus infection. Of this group, 8 patients were follow up at least 6 months. Forty one of 57 with no evidence of acute EB virus infection, 17 were follow up at least 6 months. The clinical and laboratory data was not different significantly in children with and without acute EB virus infection in admission. In EB virus infection group of 6 months follow up, platelet count was significantly lower than control group in 6 months follow up (P=0.006). Five patients of 8 (63%) with acute EB virus infection had chronic ITP and 2 of 17 (12%) with no evidence of EB virus infection had chronic ITP in follow up 6 months. CONCLUSION: Patients with EB virus associated ITP tended to resolved more slowly than those without EB virus infection and also showed tendency to become chronic ITP.
Capsid ; Child ; Developing Countries ; Follow-Up Studies ; Hematologic Diseases ; Hematology ; Herpesvirus 4, Human ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Platelet Count ; Purpura, Thrombocytopenic ; Purpura, Thrombocytopenic, Idiopathic* ; Remission, Spontaneous ; Retrospective Studies ; Serologic Tests

PURPOSE: Recent literature has shown that lymph node ratio is superior to the absolute number of metastatic lymph nodes in predicting the prognosis in several malignances other than colorectal cancer. The aim of this study was to evaluate the prognostic significance of the lymph node ratio (LNR) in patients with stage III colorectal cancer. METHODS: We included 186 stage III colorectal cancer patients who underwent a curative resection over a 10-year period in one hospital. The cutoff point of LNR was chosen as 0.07 because there was significant survival difference at that LNR. The Kaplan-Meier and the Cox proportional hazard models were used to evaluate the prognostic effect according to LNR. RESULTS: There was statistically significant longer overall survival in the group of LNR > 0.07 than in the group of LNR < or = 7 (P = 0.008). Especially, there was a survival difference for the N1 patients group (LN < 4) according to LNR (5-year survival of N1 patients was lower in the group of LNR > 0.07, P = 0.025), but there was no survival difference for the N2 group (4 > or = LN) according to LNR. The multivariate analysis showed that the LNR is an independent prognostic factor. CONCLUSIONS: LNR can be considered as a more accurate and potent modality for prognostic stratifications in patients with stage III colorectal cancer.
Colorectal Neoplasms ; Humans ; Lymph Nodes ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models