BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

PURPOSE:Maxillay sinus grafting is an effective treatment procedure to improve bone height in the posterior maxillar area for implant installation. Beta-tricalciumphosphate(beta-TCP) was introduced to be grafting substitute material, providing a reasonable bio-degradation time, no need for harvesting procedure. The purpose of this study is to evaluate bone healing and regeneration phase using histomorphometric and immunohistochemical analysis. MATERIAL AND METHODS:Sixteen rabbits were divided into 4 groups. Bi-lateral maxillary sinus membranes were elevated at each rabbits, beta-TCP was augmented in left sinus, autogenous bone was augmented in right sinus. The rabbits were sacrificed at 2, 4, 8 and 12 weeks. We investigated the bone regeneration and growth factor expression. RESULTS: 1. The mean new bone volume formation was 28.99+/-6.55%, 49.54+/-5.47%, 69.09+/-8.90% in autogenous grafted area, and 22.86+/-5.56%, 24.00+/-4.09%, 34.11+/-3.37% in beta-TCP area at 4, 8, 12 weeks. Therefore, new bone formation in autogenous bone was significantly higher than beta-TCP (p<0.05). 2. The BMP 2/4 expression in autogenous bone grafted area was higher at 4, 8 weeks. 3. There was no difference in expression pattern of BMP-7/PDGF/VEGF during grafted bone regeneration. CONCLUSION:The authors we conclude that the autogenous bone graft was faster than beta-TCP in bone regeneration, and the BMP 2/4 were more important in graft bone regeneration.
Bone Regeneration* ; Maxillary Sinus* ; Membranes ; Osteogenesis ; Rabbits ; Regeneration ; Transplants*

PURPOSE:Maxillay sinus grafting is an effective treatment procedure to improve bone height in the posterior maxillar area for implant installation. Beta-tricalciumphosphate(beta-TCP) was introduced to be grafting substitute material, providing a reasonable bio-degradation time, no need for harvesting procedure. The purpose of this study is to evaluate bone healing and regeneration phase using histomorphometric and immunohistochemical analysis. MATERIAL AND METHODS:Sixteen rabbits were divided into 4 groups. Bi-lateral maxillary sinus membranes were elevated at each rabbits, beta-TCP was augmented in left sinus, autogenous bone was augmented in right sinus. The rabbits were sacrificed at 2, 4, 8 and 12 weeks. We investigated the bone regeneration and growth factor expression. RESULTS: 1. The mean new bone volume formation was 28.99+/-6.55%, 49.54+/-5.47%, 69.09+/-8.90% in autogenous grafted area, and 22.86+/-5.56%, 24.00+/-4.09%, 34.11+/-3.37% in beta-TCP area at 4, 8, 12 weeks. Therefore, new bone formation in autogenous bone was significantly higher than beta-TCP (p<0.05). 2. The BMP 2/4 expression in autogenous bone grafted area was higher at 4, 8 weeks. 3. There was no difference in expression pattern of BMP-7/PDGF/VEGF during grafted bone regeneration. CONCLUSION:The authors we conclude that the autogenous bone graft was faster than beta-TCP in bone regeneration, and the BMP 2/4 were more important in graft bone regeneration.
Bone Regeneration* ; Maxillary Sinus* ; Membranes ; Osteogenesis ; Rabbits ; Regeneration ; Transplants*

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is one of the most important armamentarium against various hematologic malignancies or some solid tumors. We investigated the number of patients who might need transplants and compared with that of actual transplants to conceptualize current status and circumstances of HSCTs in Korean children. METHODS: Questionnaires were sent to Korean Society of Hematopoietic Stem Cell Transplantation (KSHSCT) members who were taking care of children with malignancies or hematologic diseases. Almost all of the newly diagnosed patients between Jan, 1st and Dec, 31st, 2003 were enrolled in the study. RESULTS: Seven hundred forty eight children (male to female ratio = 1.4:1) were enrolled. The median age was 6.1 years old (8 days~28.8 years old). Malignant diseases consisted of 695 cases (92.9%), and among them almost half were hematologic malignancies. The participating members speculated that HSCTs should be indicated in 285 children (38.1%) which included 209 allogeneic, and 76 autologous transplants. In reality, however, allogeneic HSCTs were performed only in 140 children (67.0%) with the median interval of 5.9 month, and autologous transplants in 44 children (57.9%) with 8.3 month. In autologous setting, all the patients received peripheral blood stem cells (PBSCs), whereas bone marrow (61%), cord blood (34%), and PBSC (5%) were used in allogeneic HSCTs. Donor types were as follows: unrelated donor (37%), cord blood (34%), sibling donor (25%), and family (4%). The reasons for not performing HSCTs were unfavorable disease status or death, no availability of suitable donor, economical situation, and refusal by parental preferences. Under the strict insurance regulations, many transplants were not covered by insurance. More autologous transplants were performed without insurance coverage than allogeneic HSCTs (P=0.013). Those cases were advanced cases and HLA mismatch transplants for allogeneic setting, and relatively rare diseases still awaiting favorable results of transplants for autologous setting. CONCLUSION: HSCTs are essential part of treatment strategies for children with various diseases. Unfortunately, however, a third of patients who were in need of transplants did not receive HSCTs due to various reasons. It is necessary to expand unrelated donor pool or cord blood banks for the cases lacking HLA-identical sibling donors. Also medical insurances should cover HSCTs for rare diseases as well as for less favorable but novel situations where there are no suitable alternatives.
Autografts ; Bone Marrow ; Child* ; Disulfiram ; Female ; Fetal Blood ; Hematologic Diseases ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Insurance ; Insurance Coverage ; Parents ; Rare Diseases ; Siblings ; Social Control, Formal ; Stem Cells ; Tissue Donors ; Unrelated Donors ; Surveys and Questionnaires

PURPOSE: Surgery remains the treatment of choice for a hepatocellular carcinoma (HCC) confined within the liver. When there is no underlying liver disease, resection is the preferred option. In cases of HCC with cirrhosis, impaired hepatic reserve often precludes safe resection. Recently, acceptable transplantation outcomes have been shown in selected HCC patients. The aim of this study was to review the results of liver transplantation for HCC at the Asan Medical Center. METHODS: 73 HCC patients were treated by liver transplantation between August 1992 and April 2001. There were 7 in-hospital mortalities. The mean age of the patients was 51 years. The period of the median follow-up was 22 months. By reviewing the patients' medical records, we investigated tumor size, and number, TNM stage, survival rates, and recurrences. Statistical analysis was performed using Statistica 5.1 and SPSS 9.0. RESULTS: Among 67 patients, 8 (12%) developed a tumor recurrence or distant metastasis following the liver transplantation. The 3 year and 5 year survival rate were 88 and 57%, respectively. There were 12 incidentalomas. The 1 year and 3 year disease free survival rates of 54 cases, with the exception of the incidentalomas, were 80 and 50%, respectively. There were no statistically significant differences in the survival rates between the groups, with and without preoperative TACE (P=0.70). Also, there were no statistically significant differences in the survival rates between cadaveric donor liver transplantations (CDLT) and living donor liver transplantations (LDLT). CONCLUSION: We assume that transplantation for HCC, in carefully selected patients, may be the solution to HCC in cirrhotic livers. If the donor safety with a LDLT can be ensured, its application to patients with cirrhosis and early HCC may be a solution to the donor shortage, which could improve the survival of this group of patients.
Cadaver ; Carcinoma, Hepatocellular* ; Chungcheongnam-do ; Disease-Free Survival ; Fibrosis ; Follow-Up Studies ; Hospital Mortality ; Humans ; Liver Diseases ; Liver Transplantation* ; Liver* ; Living Donors ; Medical Records ; Neoplasm Metastasis ; Recurrence ; Survival Rate ; Tissue Donors

PURPOSE: Childhood acute immune thrombocytopenic purpura (ITP) is a benign hematologic disease. Therapy does not affect the natural history of the illness. We evaluated the clinical and laboratory findings, treatment and prognosis of childhood acute ITP in Korea through a retrospective multicenter study. METHODS: We analyzed retrospectively the data of 1, 829 children with acute ITP through survey of 33 hospitals among 43 hospitals in Korea from Sep. 1992 to Aug. 2001. RESULTS: Male to female ratio was 1.3: 1 and the median age at the diagnosis of ITP was 2.9 (0.1 17) years. Median duration of follow up was 6 months. One hundred and forty nine cases of the total 1, 829 patients (8.1%) received no treatment. The initial median platelet count of the non-treated group was 42, 500/mm3. Among the 861 cases who were followed up over 6 months, 315 cases (36.6%) progressed into chronic ITP. Associated with this high rate of chronicity of childhood acute ITP patients in Korea, we must consider the fact that acute ITP patients with fast improvement in the first episode tend not to follow up. Considering that fact, the rate of chronicity becomes 17.2% of the 1, 829 acute ITP patients. The treated group used many kinds of treatment methods. Intravenous immunoglobulins (IVIG) with or without prednisolone (PD) (67.5%) were the most commonly used regimens. In the group treated with IVIG alone, the platelet count began to rise above 50, 000/mm3 at 2.6 days, 100, 000/mm3 at 3.7 days and 150, 000/mm3 at 4.9 days. Four hundred and twenty two cases of the 1, 686 (25.0%) cases followed up after first episode of ITP relapsed. The relapse rate was significantly higher in older patients and in girls than in younger patients and in boys (P< 0.05). The chronicity of ITP statistically increased with age (P< 0.05) and that was the only valuable factor. CONCLUSION: Despite the fact that childhood acute ITP is a pretty common disaese, there is no agreement on the best treatment method for this disease. The establishment of Korean treatment guideline of childhood acute ITP, based on an analysis of multicenters, seems to be needed.
Child ; Diagnosis ; Female ; Follow-Up Studies ; Hematologic Diseases ; Humans ; Immunoglobulins, Intravenous ; Korea* ; Male ; Natural History ; Platelet Count ; Prednisolone ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic* ; Recurrence ; Retrospective Studies

PURPOSE: Although there has been recent progress in surgical techniques, such as perioperative management, immunosuppresive regimen and intervention radiology, a liver retransplantation remains as the only therapeutic option for patients with a failing liver allograft. The purpose of this study was to review our clinical experiences of liver retransplantation, performed at the Asan Medical Center. METHODS: Between August 1992 and March 2001, 400 cases of liver transplantations, including 331 in adults and 69 in pediatrics, were performed. Of the 331 adult cases, 10 cases of liver retransplantation, during the same period, were retrospectively analyzed. RESULTS: In the 331 cases of adult liver transplantation, 232 cases of living donor and 99 of cadaveric liver transplantations were carried out. The 331 adult cases also included 10 liver retransplantations. Therefore, the overall liver retransplantation rate was 3%. Primary non-function (PNF) was the leading cause of retransplantation. The conversion of living donor liver transplantation to a cadaveric liver retransplantation was the most common type of retransplantaion, with a cadaveric to cadaveric type the second most common. The in-hospital mortality was 40%. The causes of in-hospital mortality were hepatic artery pseudoaneurysm rupture, Aspergillus pneumonia, and multiple organ failure, initiated by jejuno-jejunostomy site bleeding and massive hepatic necrosis. CONCLUSION: In the current era of extreme organ shortage, retransplantation is the only therapeutic alternative for irreVersible graft failure, especially if the patient has no multiple organ failure (MOF) prior to the operation. Therefore, the careful selection of patients for a retransplantation is required. They should be given superurgent priority if the circumstances permit, and living donor liver transplantation (LDLT) offer a promising alternative.
Adult ; Allografts ; Aneurysm, False ; Aspergillus ; Cadaver ; Chungcheongnam-do ; Hemorrhage ; Hepatic Artery ; Hospital Mortality ; Humans ; Liver Transplantation ; Liver* ; Living Donors ; Massive Hepatic Necrosis ; Multiple Organ Failure ; Pediatrics ; Pneumonia ; Retrospective Studies ; Rupture ; Transplants

PURPOSE: The major limitation of adult-to-adult living donor liver transplantation (A-A LDLT) is the adequacy of the graft size. As an alternative, dual grafts from two living donors can solve the problem of graft-size insufficiency and guarantee the donor safety in many occasions. The present study aims to introduce the usefulness of dual-grafts A-A LDLT by review of our single center experience. METHODS: After the first successful pediatric LDLT in December 1994 and A-A LDLT in February 1997, 392 LDLTs including 73 pediatric and 319 adult cases were performed at Asan Medical Center until December 2001. Among 319 A-A LDLTs, 20 recipients implanted dual grafts were retrospectively analysed from March 2000 to December 2001. RESULTS: The ratio of graft volume to standard liver volume of the recipients ranged from 46.6% to 78.9%. More than 50% of the standard liver volume of the recipients was implanted in 16 patients. There was acute rejection episode in two patients, which were responded by pulsed steroid therapy. There were 3 in- hospital mortality (<3 month posttransplantation). CONCLUSION: In LDLT, the donor safety is the major concern. Although the donor has a large right lobe of liver that is adequate as a graft for large-size recipient, the remaining left lobe of liver is sometimes too small to endanger the donor safety. In this circumstance, the donor cannot be accepted to donate his or her right or left lobe of liver. Dual grafts from two living donors can help to alleviate the problem of small-for-size graft and secure the donor safety.
Adult ; Chungcheongnam-do ; Hospital Mortality ; Humans ; Liver Transplantation* ; Liver* ; Living Donors* ; Retrospective Studies ; Tissue Donors ; Transplants*

Wolff-Parkinson-White(WPW) syndrome is characterized by electrographic evidence of ventricular preexcitation, which predisposes to supraventicular arrhythmias. Familial occurrence of WPW syndrome is uncommon. We observed two affected siblings in a family. Five members of the family underwent 12-lead electrocardiography and echocardiography. Although known genetic abnormality of the 7q34-q36(PRKAG2) for the familial WPW syndrome was evaluated, the mutation was not detected in this family. Other unknown mutations responsible for this familial WPW syndrome were suggested.
Arrhythmias, Cardiac ; Echocardiography ; Electrocardiography ; Humans ; Siblings ; Wolff-Parkinson-White Syndrome*

PURPOSE: Living donor liver transplantation (LDLT) has become an evolving option to overcome the shortage of cadaveric donor organ in adults as well as in children. The purpose of this study was to determine the incidence, timing, sites, and risk factors of infection after adult-to-adult LDLT. METHODS: The authors performed 104 adult-to-adult LDLT in 103 patients during the period of February 1997 and December 1999. The major indications for transplantation were chronic hepatitis B (53), hepatocellular carcinoma (27), and fulminant hepatitis (10). Right hepatic lobe was used in 54 cases and left lobe in 50. Graft weight-to-standard liver volume of the recipient ranged from 28.91% to 77.43% (mean 47.60%). No patient died during surgery. The incidence, timing, sites, and risk factors of infection after adult- to-adult LDLT were investigated retrospectively. RESULTS: A total of 114 cases of infection, including 85 bacterial, 3 mycobacterial, 16 fungal and 10 viral infection, developed in 65 (63.1%) patients. Seventy-one cases of infection occurred within 1 month after surgery. Intra-abdominal infection (31), hepato-biliary infection (19), primary bacteremia (12), and pneumonia (10) were the frequent ones, which developed mainly within 1 month after transplantation. Eight of 9 patients with pneumonia that developed early in the postoperative course died. Since January 1999, the incidence of pneumonia declined significantly from 20.0% (7/35) to 2.9% (2/68). Most fungal infection, including 7 cases of intra-abdominal infection, also occurred within 1 month after surgery (13/16). In contrast, all the 10 cases of viral infection developed after 2 months postoperatively. One case each of recurrent hepatitis B, recurrent hepatitis C, and posttransplant lymphoproliferative disorder died. Patients with infection showed significantly lower survival rate than those without infection (66.2% vs. 97.4%, p=0.0009). The indication for transplantation, amount of intraoperative RBC transfusion, and value of prothrombin time at the 7th day after surgery were significant risk factors for early serious infection on multivariate analysis. Urgency of operation was the only significant risk factor for early fungal infection on univariate analysis. CONCLUSION: For the prevention of early serious infection after liver transplantation, efforts to reduce the amount of intraoperative transfusion and to protect the graft from perioperative insults should be executed. Preemptive anti-fungal therapy is suggested in cases of emergent operation.
Adult ; Bacteremia ; Cadaver ; Carcinoma, Hepatocellular ; Child ; Hepatitis ; Hepatitis B ; Hepatitis B, Chronic ; Hepatitis C ; Humans ; Incidence ; Intraabdominal Infections ; Liver Transplantation* ; Liver* ; Living Donors* ; Lymphoproliferative Disorders ; Multivariate Analysis ; Pneumonia ; Prothrombin Time ; Retrospective Studies ; Risk Factors ; Survival Rate ; Tissue Donors ; Transplants