Asphyxia is the most important perinatal cause of neurologic morbidity in infants. Recently, elevated levels of CK-BB have been demonstrated in serum after asphyxic insult in infants. To evaluate the possibility of using serial measurements of CK-BB activity as a diagnostic method and a predictor of neurologic outcome in asphyxiatic infants. We studied CK-BB activity in serum of asphyxiated infants (16 preterm babies, 41 term babies) and healthy infants (11 preterm babies, 12 term babies) from birth (cord) to 5 days of serially. The results were as follows 1) Serum CK-BB activites (cord 76.0IU/L, 12 hours 34.1 IU/L, 1 day 22.4 IU/L, 2 days 8.2 IU/L) of asphyxiated preterm infants were much higher than those (cord 4.4IU/L, 12 hours 2.4IU/L, 1 day 1.6 IU/L, 2 days 1.1 IU/L) of healthy preterm infants, but there were no significant differences (P values>0.05). 2) Serum CK-BB activities (cord 59.0 IU/L, 12 hours 23.6 IU/L, day 17.6 IU/L) of asphyxiated term infants were significantly higher than those (cord 2.4 IU/L, 12 hours 2.7 IU/L, 1 day 0.4 IU/L) of healthy term infants (P values<0.05) 3) There were no significant differences in serum CK-BB activities between asphyxiated preterm infants who showed late neurologic deficits (P values>0.05) 4) Serum CK-BB activity (131.7 IU/L) at birth (cord) of asphyxiated term infants who showed late neurologic deficits was significantly higher than that (49.8 IU/L) of asphyxiated term infants who did not show late neurologic deficits (P value<0.05) In conclusion, it is suggested that serial measurement of CK-BB activity in serum of asphyxiated term infants from birth (cord) to 1 day of age is an available test for diagnosis of neonatal asphyxia and for a prediction of neurologic outcome of neonatal asphyxia.
Asphyxia ; Diagnosis ; Humans ; Infant* ; Infant, Newborn ; Infant, Premature ; Neurologic Manifestations ; Parturition

This study was designed to investigate factors related to recovery from cerebrovascular accidents(CVA). Medical charts of 100 CVA patients(40 males and 60 females) who had been treated at Bul-Guang hospital in Teagu from June to December 1994 were reviewed to assess their recovery from CVA. Not only types of CVA, blood pressure and serum cholesterol and triglyceride were factors affecting recovery from CVA, but also smoking and drinking status and food preference seemed to be important factors. The percentages of recovered patients were higher in the order of cerebral thrombosis(83.3%), subarachnoid hemorrhage(57.1%), cerebral embolism(50.0%), and cerebral hemorrhage(26.7%). Recovery rates of patients with serum cholesterol below 200mg/dl, 200-239mg/dl, over 240mg/dl were 81.8%, 66.6%, and 16.6% respectively. Recovery rates of patients with serum triglyceride below 160mg/dl, 160-209mg/dl, and oover 210mg/dl were 84.6%, 72.8%, and 35.7% respectively. Patients with standard weight recovered better than those with overweight or obesity. Recovery rates of underweight, standard weight, overweight and obesity patients were 73.3%, 85.7%, 45.8%, and 31.6% respectively. Smoking and drinking seemed to be important factors which inhibited recovery from CVA. Patients preferring spiced foods were recovered better than those preferring salty or pungent foods.
Blood Pressure ; Cholesterol ; Drinking ; Food Preferences ; Humans ; Male ; Obesity ; Overweight ; Smoke ; Smoking ; Spices ; Stroke* ; Thinness ; Triglycerides

We report a case of light chain deposition disease in a 59-yr-old female showing deposition of monoclonal light chain in the kidney and bone marrow accompanied with a schistocytosis, the morphologic finding of microangiopathic hemolytic anemia. The immunofluorescence examination of the kidney revealed strongly stained kappa-light chain deposits on the glomerular mesangium and capillary wall, tubules, and vessel wall. The electron microscopy demonstrated electron-dense deposits on the glomerular basement membrane and mesangium. Anemia was observed with schistocytosis and Howell-Jolly body in the peripheral blood smears. The immunohistochemical examination of the bone marrow showed the presence of kappa-light chain deposits in scattered plasma cells and thickened vessel wall in the absence of a prominent plasma cell proliferation. Although an immunofixation electrophoresis failed to detect a monoclonal gammopathy, the presence of monoclonal protein could be identified by an abnormal kappa/lambda ratio on the serum free light chain analysis.
Anemia, Hemolytic/complications/*diagnosis ; Bone Marrow/*pathology ; Female ; Glomerulonephritis/complications/*diagnosis/pathology ; Humans ; Immunoglobulin Light Chains/*analysis ; Kidney Glomerulus/*pathology/ultrastructure ; Middle Aged ; Paraproteinemias/complications/*diagnosis/immunology

PURPOSE: To evaluate the efficacy of amniotic membrane (AM) transplantation in symptomatic bullous keratopathy using cryo-preserved or lyophilized AM. METHODS: Eight bullous keratopathic patients, who experienced intractable pain despite medication and the application of a therapeutic lens, underwent permanent AM transplantation with 5 cryo-preserved and 3 lyophilized AMs following removal of epithelium using a trephine. The time to re-epithelization and the subsidence of pain were evaluated retrospectively. RESULTS: Mean follow-up was 252+/-171.0 days. Pain relief and re-epithelization were obtained in all eight patients. The times to complete relief of pain was 32.2+/-22.7 days in patients with cryo-preserved AM transplantations (n=5) and 22.0+/-18.0 days in those with lyophilized AM transplantations (n=3) (p=0.549, Mann-Whitney U test). Respective times to re-epithelization were 25.4+/-20.7 days and 23.0+/-25.2 days (p=0.297). There were no significant differences in the diameter of transplanted AMs between the two groups (7.6+/-0.2 vs. 7.3+/-0.3 mm, p=0.172). CONCLUSIONS: Permanent AM transplantation effectively reduced the intractable pain caused by epithelial bulla. Whether the AM was cryo-preserved or lyophilized did not affect the efficacy of the treatment.
Amnion* ; Epithelium ; Follow-Up Studies ; Humans ; Pain, Intractable ; Retrospective Studies ; Transplants*

PURPOSE: The diagnostic criteria of the biphenotypic acute leukemia (BAL) are based on the number and degree of the specificity of the immunological markers expressed on the leukemic blasts. The newer diagnostic criteria proposed by The European Group for the Immunological Classification of Leukaemias (EGIL) is now well accepted. We have recently evaluated our BAL patients using the EGIL criteria and analyzed the clinical characteristics, treatment and clinical outcome. METHODS: Fourteen children diagnosed with BAL among 193 childhood acute leukemia patients in our hospital from January 1995 to December 2001 were retrospectively reviewed. RESULTS: The incidence of BAL was 7.3% (14 out of 193 cases). Of these 14 patients, 12 were de novo and 2 were secondary. In the de novo group, the immunological marker studies showed myeloid/B-lymphoid phenotype in 6 (50%), myeloid/T-lymphoid in 3 and B-lymphoid/T-lymphoid in 1. In addition, two patients showed trilineage differentiation. Cytogenetic analysis revealed various abnormal karyotypes in the majority of the cases, showing normal karyotype only in 3 cases. Among the karyotype abnormalities, two were t (9; 22) and one was t (4; 11). Chemotherapy was mostly based on the induction regimen of acute lymphoblastic leukemia therapy (12 of 14 cases). One patient was treated with acute myeloid leukemia regimen and one patient received combination of both acute lymphoid and myeloid regimen. Induction of complete remission was achieved in 100% of the patients and the median duration of induction therapy to complete remission was 33 days. Five of the 12 de novo patients died during the median follow-up of 16.5 months (4 months to 37.5 months) and the 2 year survival rate was only 52%. CONCLUSION: The incidence of BAL in children is relatively rare and the most common immunophenotypic expression consists of myeloid and B-lymphoid coexpression. Most of the cases showed chromosomal abnormalities. The outcome of BAL treated mostly with the traditional acute lymphoblastic leukemia induc without stem cell transplantation should be sought especially in those expressing poor prognostic cytogenetic abnormalities or strong myeloid marker expression.
Abnormal Karyotype ; Child ; Chromosome Aberrations ; Classification ; Cytogenetic Analysis ; Drug Therapy ; Follow-Up Studies ; Humans ; Incidence ; Karyotype ; Leukemia ; Leukemia, Biphenotypic, Acute* ; Leukemia, Myeloid, Acute ; Phenotype ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Sensitivity and Specificity ; Stem Cell Transplantation ; Survival Rate

The interactions between the tumor microenvironment and tumor cells determine the behavior of the primary tumors. Whether cancer-associated fibroblasts (CAF) have a tumor progressive or a protective role likely depends on the type of tumor cells and the CAF subpopulation. In the present study, we analyzed the prognostic significance of CAF subpopulations in colorectal cancer (CRC). CAF phenotypes were analyzed in 302 CRC patients by using antibodies against podoplanin (PDPN), alpha-smooth muscle actin (alpha-SMA), and S100A4. The relationship between the CAF phenotypes and 11 clinicopathological parameters were evaluated and their prognostic significance was analyzed from the disease-free and overall survival times. We observed that at the tumor invasive front, PDPN CAFs were present in 40% of the cases, and S100A4 or alpha-SMA CAFs were detected in all the cases. PDPN/S100A4 and alpha-SMA/S100A4 dual-stained CAFs were observed in 10% and 40% of the cases, respectively. The PDPN+ CAFs were associated with 6 favorable clinicopathological parameters and prolonged disease-free survival time. The PDPN-/alpha-SMA(high) CAFs were associated with 6 aggressive clinicopathological parameters and tended to exhibit shorter disease-free survival time. On the other hand, the PDPN-/S100A4(high) CAFs were associated with 2 tumor progression parameters, but not with disease prognosis. The PDPN+ CAF phenotype is distinct from the alpha-SMA or S100A4 CAFs in that it is associated with less aggressive tumors and a favorable prognosis, whereas the PDPN-/alpha-SMA(high) or PDPN-/S100A4(high) CAFs are associated with tumor progression in CRC. These findings suggest that CAFs can be a useful prognostic biomarker or potential targets of anti-cancer therapy in CRC.
Actins/immunology/*metabolism ; Adult ; Aged ; Aged, 80 and over ; Antibodies/immunology ; Carcinoembryonic Antigen/blood ; Colorectal Neoplasms/*diagnosis/mortality/pathology ; Disease-Free Survival ; Female ; Fibroblasts/cytology/metabolism ; Humans ; Immunohistochemistry ; Lymphatic Metastasis ; Male ; Membrane Glycoproteins/immunology/*metabolism ; Middle Aged ; Neoplasm Staging ; Phenotype ; Prognosis ; S100 Proteins/immunology/*metabolism ; Tumor Markers, Biological/metabolism

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency in children caused by an abnormal function of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells, which results in an increased susceptibility to severe bacterial and fungal infections. Prophylactic trimethoprim-sulfamethoxazole improves medium-term survival, but cannot prevent inflammatory sequelae. It still shows high morbidity and mortality. Bone marrow transplantation (BMT) is currently the only curative treatment for CGD. We report on a 29-month-old boy with CGD who was successfully treated with allogeneic BMT from an HLA-identical unrelated donors following a conditioning regimen consisting of busulfan and cyclophosphamide. One year after post-transplantation, the boy is in excellent clinical and hematological condition with complete chimerism and normal nitroblue tetrazolium (NBT) test.
Bone Marrow Transplantation ; Busulfan ; Child ; Child, Preschool ; Chimerism ; Cyclophosphamide ; Granulomatous Disease, Chronic* ; Humans ; Male ; Mortality ; NADP ; Nitroblue Tetrazolium ; Oxidoreductases ; Phagocytes ; Stem Cell Transplantation* ; Stem Cells* ; Trimethoprim, Sulfamethoxazole Drug Combination ; Unrelated Donors

The myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis associated with multilineage cytopenias leading to serious morbidity or mortality, and the additional risk of leukemic transformation. The management of patients with MDS can be very complex and varies according to both the clinical manifestations in individual patients as well as the presence of complicating medical conditions. However, therapeutic dilemmas still exist for MDS due to the multifactorial pathogenetic features of the disease, its heterogeneous stages, and the elderly patient population. For these reasons, proper guidelines for management are necessary. This review describes the proper diagnosis for MDS, decision-making approaches for optimal therapeutic options that are based on a consideration of patient clinical factors and risk-based prognostic categories, and the use of recently available biospecific drugs such as hypomethylating agents that are potentially capable of abrogating the abnormalities associated with MDS. Proper indications and methods for transplantation, response criteria, management for iron overload for highly transfused patients and specific considerations for MDS in childhood are also described. All of these topics were discussed at the third symposium of AML/MDS working party on 3 March, 2007.
Aged ; Diagnosis ; Hematopoiesis ; Humans ; Iron Overload ; Mortality ; Myelodysplastic Syndromes* ; Transplantation

PURPOSE: To investigate the color vision defect in diabetic patients using the SNU computerized color test (SCCT). METHODS: From May to September 2003, diabetic patients with visual acuity 0.6 or better underwent various examinations including biomicroscopy, fundus photography, Ishihara color test, Hardy?Rand?Rittler (HRR) test, Seohan computerized hue test (SCHT), and SNU computerized color test. The SCCT was developed by using the Matlab 6.0 program. RESULTS: A total of 160 eyes of 82 diabetic patients were included. Thirty-two patients had no diabetic retinopathy, 19 had mild nonproliferative diabetic retinopathy (NPDR), 12 had moderate NPDR, 12 had severe NPDR, and 7 had proliferative diabetic retinopathy (PDR). In the all diabetic patients, the average total error score (TES) of SCHT was 189 and that of SCCT was 8.5; in patients without diabetic retinopathy, the scores were 125 and 3.64; in patients with mild NPDR, 185 and 8.16; in patients with moderate NPDR, 209 and 11.1; in patients with severe NPDR, 288 and 15.6 ; and in patients with PDR, 324 and 17.6 respectively. On the HRR test, patients without diabetic retinopathy had 1 tritan defect; those with mild NPDR 2 tritan, 2 protan, and 2 deutan defects: those with moderate NPDR, no color defects ; and those with severe NPDR, 2 tritan, and 2 protan defects, and 1 deutan defect. CONCLUSIONS: In diabetic patients, TES of SCHT and SCCT was higher according to the severity of diabetic retinopathy. SCHT and SCCT were more useful than HRR test.
Color Vision Defects* ; Color Vision* ; Diabetic Retinopathy* ; Humans ; Photography ; Visual Acuity

PURPOSE: This study was designed to investigate the characteristics and classification of congenital color vision deficiency (CVD) by the SNU computerized color test (SCCT) that was developed to sufficiently utilize the advantages of a computer. METHODS: Hardy-Rand-Rittler test (HRR test), Nagel anomaloscope and SCCT were performed on 60 eyes of 30 CVD patients and 30 normal subjects and the results were compared. RESULTS: In normal subjects, the error scores were all zero at all colors by SCCT. By SCCT protan color defectives showed a peak at hue 0 red in 7 eyes (29.2%), at hue 150 green in 3 eyes (12.5%), at hue 180 green in 18 eyes (75%), and at hue 330 red in 2 eyes (8.3%). By SCCT, deutan color defectives showed a peak at hue 0 red in 2 eyes (5.6%), at hue 150 green in 24 eyes (66.7%), at hue 180 green in 2 eyes (5.6%), and at hue 330 red in 23 eyes (63.9%). CONCLUSIONS: SCCT showed specific axes in CVD patients, with accuracy and high sensitivity to diagnosis. SCCT appears to be useful clinically as a color vision test to diagnose and classify CVD patients.
Classification* ; Color Vision Defects* ; Color Vision* ; Diagnosis ; Humans