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1.
Cancer Research and Treatment ; : 279-290, 2023.
Article in English | WPRIM | ID: wpr-966470

ABSTRACT

Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

2.
Cancer Research and Treatment ; : 269-276, 2022.
Article in English | WPRIM | ID: wpr-913833

ABSTRACT

Purpose@#Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. @*Materials and Methods@#Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. @*Results@#Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). @*Conclusion@#This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

3.
Pediatric Infection & Vaccine ; : 111-116, 2020.
Article | WPRIM | ID: wpr-837086

ABSTRACT

Purpose@#Human parvovirus B19 infection is widespread and has a heterogeneous clinical spectrum, ranging from asymptomatic infection to potentially life-threatening complications. We investigated the various clinical features of human parvovirus B19 infection during an outbreak of the virus in our community. @*Methods@#A retrospective chart review study was conducted at the Pusan National University Children's Hospital from December 2017 to April 2019. We investigated the clinical features of children with parvovirus B19 immunoglobulin M or parvovirus B19 DNA detected using polymerase chain reaction. @*Results@#A total of 24 children were diagnosed with parvovirus B19 infection. Twelve (50%) had lace form rashes, and four (16.7%) had petechial rashes. Two (8.3%) were diagnosed with fever without a focus. Six (25%) developed aplastic crisis as a complication of infection, of whom three were previously diagnosed with hereditary spherocytosis and three with acute lymphoblastic leukemia. @*Conclusions@#In addition to erythema infectiosum, the parvovirus B19 infection can present clinically with various types of rashes and fever without a focus. Furthermore, hematologic manifestations such as neutropenia and aplastic crisis can occur during infection.

4.
Journal of Korean Medical Science ; : e393-2020.
Article in English | WPRIM | ID: wpr-831569

ABSTRACT

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

5.
Clinical Pediatric Hematology-Oncology ; : 77-82, 2019.
Article in Korean | WPRIM | ID: wpr-763519

ABSTRACT

BACKGROUND: Immune thrombocytopenic purpura (ITP) in children less than one year of age is less well characterized compared to ITP in toddlers and school-age children. Since children of different ages may have differing clinical courses, better delineation of the natural history of ITP in infants is needed. METHODS: We retrospectively reviewed the admission records of 248 consecutive pediatric patients between 1 month and 15 years of age who were admitted and treated for acute ITP at Pusan National University Children's Hospital from 2009 through 2017. All patients less than 1 year of age were identified and enrolled in this study. We investigated their demographics, clinical features, laboratory examinations, response to treatment, and long-term outcomes and made a comparison to those of children aged 1 to 10 years of age. RESULTS: Ninety nine infants were identified. Male to female ratio was highest in infants and decreased with age. Seventy nine (79.8%) of the 99 infant were found to be under 6 months old. The median platelet counts at diagnosis was 6×10⁹/L. Minor bleeding (bleeding score 0–2) was significantly dominant in infant compared to older subjects. Eighty two (96.5%) out of 85 patients achieved complete remission after initial intravenous immunoglobulin (IVIG) treatment. The relapse rate after initial CR was significantly lower than older ages (P=0.003). The platelet count after IVIG treatment in infant showed more rapid response compared to older subjects (P=0.04). Follow up information at 12 months was available for 70 infants. Chronic ITP at 12 month was seen less frequently in infants than in children 1 to 10 years of age (1.4% vs. 20.2%, P<0.001). CONCLUSION: Infants with acute ITP respond more favorably to IVIG treatment and are less likely to develop chronic ITP compared to children 1 to 10 years of age.


Subject(s)
Child , Female , Humans , Infant , Male , Demography , Diagnosis , Follow-Up Studies , Hemorrhage , Immunoglobulins , Immunoglobulins, Intravenous , Natural History , Platelet Count , Purpura, Thrombocytopenic, Idiopathic , Recurrence , Retrospective Studies
6.
Clinical Pediatric Hematology-Oncology ; : 46-54, 2019.
Article in Korean | WPRIM | ID: wpr-763505

ABSTRACT

BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.


Subject(s)
Adult , Child , Humans , Diagnosis , Hemophilia A , Hemorrhage , Hospitals, University , Korea , Medical Records , Prevalence , Retrospective Studies , von Willebrand Diseases
7.
Clinical Pediatric Hematology-Oncology ; : 197-201, 2018.
Article in English | WPRIM | ID: wpr-717633

ABSTRACT

Spontaneous rupture with internal bleeding of solid tumors has rarely been described at the time of diagnosis or during chemotherapy. This rare event must be regarded as a life threatening condition. In these emergency situations, control of hemorrhage, which is life-saving, can be achieved by transcatheter arterial embolization (TAE) and/or surgical resection. This report describes two infants presenting with acute hemorrhagic shock due to spontaneous tumor rupture of hepatoblastoma and neuroblastoma during chemotherapy. TAE successfully arrested the tumor bleeding and a visibly reduced the tumor size in both children. Spontaneous rupture of solid tumors occur infrequently in children, but is a life threatening situation. Careful monitoring for the occurrence of this rare event especially in very young children presenting with a large tumor mass.


Subject(s)
Child , Humans , Infant , Diagnosis , Drug Therapy , Emergencies , Hemorrhage , Hepatoblastoma , Neuroblastoma , Rupture , Rupture, Spontaneous , Shock, Hemorrhagic
8.
Clinical Pediatric Hematology-Oncology ; : 43-49, 2018.
Article in English | WPRIM | ID: wpr-714199

ABSTRACT

BACKGROUND: Venous thromboembolism (VTE) is rare in pediatric patients compared to adults, but it's incidence is gradually increasing. The purpose of this study was to analyze the incidence, risk factors, and prognosis of pediatric patients with VTE in Korea. METHODS: Between January 2000 and July 2017, 249,312 medical records of the patients older than 1 year who were hospitalized in the department of pediatrics of 10 university hospitals in Yeungnam region were retrospectively reviewed. RESULTS: The overall incidence of VTE was 4.9 per 10,000 admissions. Of the total 123 patients, 80 (65.0%) were male and the median age was 10.8 years (range, 1.0–23.5 years). Magnetic resonance imaging was performed most frequently to confirm the diagnosis of VTE (43.1%). Thrombosis occurred in the cerebral vessels (46.3%), lower extremities (23.8%), pulmonary (19.5%), abdomen (9.8%), and upper extremities (4.1%). One hundred and six patients had underlying causes such as cancer (27.6%), infection (26.8%), intravenous catheter insertion (17.9%), and surgery (14.6%). Protein C was evaluated in 39 patients (31.7%), protein S in 40 (32.5%), antithrombin (AT) III in 52 (42.3%), and homocysteine in 21 (17.1%). Among them, one patient with a family history of AT III deficiency had SERPINC gene mutation. Seventy-seven patients (62.6%) started anticoagulation treatment. Most (52.0%) were treated for more than 90 days. CONCLUSION: Healthcare providers must be aware of the potential for VTE development in childhood. In the near future, a nationwide survey should be investigated to determine the incidence rate and the trends in VTE among Korean children.


Subject(s)
Adult , Child , Humans , Male , Abdomen , Catheters , Diagnosis , Epidemiology , Health Personnel , Homocysteine , Hospitals, University , Incidence , Korea , Lower Extremity , Magnetic Resonance Imaging , Medical Records , Pediatrics , Prognosis , Protein C , Protein S , Retrospective Studies , Risk Factors , Thrombosis , Upper Extremity , Venous Thromboembolism
9.
Electrolytes & Blood Pressure ; : 27-36, 2017.
Article in English | WPRIM | ID: wpr-149589

ABSTRACT

BACKGROUND: We investigated the effect of vitamin D deficiency on cardiovascular risk profiles in an Asian population with chronic kidney disease (CKD). METHODS: A total of 210 participants (62 non-dialysis CKD patients and 148 hemodialysis [HD] patients) were enrolled between December 2009 and February 2010. Vitamin D deficiency was determined using the serum 25-hydroxyvitamin D [25(OH)D] concentration. Blood pressure and arterial stiffness were measured. Subjects were divided into groups according to 25(OH)D concentration based on a cut-off of 13.5 ng/mL in non-dialysis CKD patients and 11.3 ng/mL in HD patients. RESULTS: The mean age was 61.7±12.3 years in non-dialysis CKD patients and 57.0±12.7 years in HD patients. In the non-dialysis CKD group, mean estimated glomerular filtration rate (eGFR) was 29.7±15.4 mL/min/1.73 m2. Mean 25(OH)D concentration was 13.6±7.8 ng/mL in non-dialysis CKD patients and 11.3±6.7 ng/mL in HD patients. More than half of the subjects had vitamin D deficiency (67.6% in non-dialysis CKD patients and 80.4% in HD patients). There were no significant differences in systolic blood pressure, pulse pressure, and arterial stiffness between higher and lower 25(OH)D groups among non-dialysis CKD and HD patients. Multivariate analysis revealed that female sex (odds ratio [OR]: 5.890; 95% confidence interval [CI]: 2.597–13.387; p<0.001) and presence of diabetes (OR: 2.434; 95% CI: 1.103–5.370; p=0.028) were significantly associated with lower serum 25(OH)D levels in HD patients. CONCLUSION: The prevalence of vitamin D deficiency was high in both nondialysis CKD patients and HD patients. Serum 25(OH)D concentration was not a significant factor associated with blood pressure and arterial stiffness among non-dialysis CKD and HD patients.


Subject(s)
Female , Humans , Asian People , Blood Pressure , Glomerular Filtration Rate , Multivariate Analysis , Prevalence , Renal Dialysis , Renal Insufficiency, Chronic , Vascular Stiffness , Vitamin D Deficiency
10.
Electrolytes & Blood Pressure ; : 1-11, 2017.
Article in English | WPRIM | ID: wpr-29650

ABSTRACT

Vitamin D has the pleiotropic effects in multiple organ systems, and vitamin D deficiency was suggested to be associated with high blood pressure according to previous reports. Several interventional studies have examined the effect of vitamin D supplementation on high blood pressure patients, but the results have been inconsistent. In this article, we examined the literature that have proposed a mechanism involving vitamin D in the regulation of blood pressure and review previous observational and interventional studies that have shown the relationship between vitamin D and hypertension among various populations.


Subject(s)
Humans , Blood Pressure , Hypertension , Physiology , Vitamin D Deficiency , Vitamin D , Vitamins
11.
Clinical Pediatric Hematology-Oncology ; : 120-126, 2015.
Article in Korean | WPRIM | ID: wpr-788558

ABSTRACT

BACKGROUND: Currently, data on the role of tacrolimus and mini-dose methotrexate (MTX) in pediatric unrelated hematopoietic stem cell transplantation (HSCT) is limited. We report the outcomes of unrelated hematopoietic stem cell recipients, evaluating engraftment status, incidence of acute and chronic graft-versus-host disease (GVHD) and toxicities after use of tacrolimus and mini-dose MTX for GVHD prophylaxis.METHODS: Thirty-five children who received tacrolimus and mini-dose MTX as prophylaxis from January 2004 to December 2013 were reviewed. All patients received tacrolimus beginning the day prior to transplant at a dose of 0.03 mg/kg/day by continuous intravenous infusion. MTX was administered at a dose of 5 mg/m2 IV on days 1, 3, 6 and 11.RESULTS: Median age at transplantation was 8.42 years (range 0.75-18.9 years). Seventeen patients received human leukocyte antigen (HLA) fully matched donor transplants and 18 received partially mismatched transplants. All but two patients who received unrelated cord blood transplants showed successful engraftment. The median time to ANC recovery was 12 days. The incidence of acute GVHD was 33.3% including 15.1% grade III-IV GVHD. Localized chronic GVHD developed in only 2 of 27 (7.4%) evaluable patients. Lower tacrolimus levels during days 1-21 were associated with a higher incidence of acute GVHD (P=0.033). The estimated 4-year event free survival and overall survival of the patients were 71.2% and 80.0%.CONCLUSION: Overall, the combination of tacrolimus and mini-dose MTX could be effectively administered in the setting of pediatric unrelated HSCT.


Subject(s)
Child , Humans , Disease-Free Survival , Fetal Blood , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Hematopoietic Stem Cells , Incidence , Infusions, Intravenous , Korea , Leukocytes , Methotrexate , Tacrolimus , Tissue Donors
12.
Clinical Pediatric Hematology-Oncology ; : 181-185, 2015.
Article in English | WPRIM | ID: wpr-788547

ABSTRACT

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.


Subject(s)
Adolescent , Adult , Child , Humans , Male , Bone Marrow , Dendritic Cells , Diagnosis , Hematologic Neoplasms , Hematopoietic Stem Cell Transplantation , Induction Chemotherapy , Lymph Nodes , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Skin
13.
Journal of Korean Medical Science ; : 162-166, 2015.
Article in English | WPRIM | ID: wpr-141159

ABSTRACT

Associations between body mass index (BMI), body fat percentage (BF%), and health risks differ between Asian and European populations. BMI is commonly used to diagnose obesity; however, its accuracy in detecting adiposity in Koreans is unknown. The present cross-sectional study aimed at assessing the accuracy of BMI in determining BF%-defined obesity in 6,017 subjects (age 20-69 yr, 43.6% men) from the 2009 Korean National Health and Nutrition Examination Survey. We assessed the diagnostic performance of BMI using the Western Pacific Regional Office of World Health Organization reference standard for BF%-defined obesity by sex and age and identified the optimal BMI cut-off for BF%-defined obesity using receiver operating characteristic curve analysis. BMI-defined obesity (> or =25 kg/m2) was observed in 38.7% of men and 28.1% of women, with a high specificity (89%, men; 84%, women) but poor sensitivity (56%, men; 72% women) for BF%-defined obesity (25.2%, men; 31.1%, women). The optimal BMI cut-off (24.2 kg/m2) had 78% sensitivity and 71% specificity. BMI demonstrated limited diagnostic accuracy for adiposity in Korea. There was a -1.3 kg/m2 difference in optimal BMI cut-offs between Korea and America, smaller than the 5-unit difference between the Western Pacific Regional Office and global World Health Organization obesity criteria.


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Adipose Tissue/physiology , Adiposity/physiology , Body Mass Index , Cross-Sectional Studies , Nutrition Surveys , Obesity/diagnosis , ROC Curve , Reference Standards , Republic of Korea
14.
Journal of Korean Medical Science ; : 162-166, 2015.
Article in English | WPRIM | ID: wpr-141158

ABSTRACT

Associations between body mass index (BMI), body fat percentage (BF%), and health risks differ between Asian and European populations. BMI is commonly used to diagnose obesity; however, its accuracy in detecting adiposity in Koreans is unknown. The present cross-sectional study aimed at assessing the accuracy of BMI in determining BF%-defined obesity in 6,017 subjects (age 20-69 yr, 43.6% men) from the 2009 Korean National Health and Nutrition Examination Survey. We assessed the diagnostic performance of BMI using the Western Pacific Regional Office of World Health Organization reference standard for BF%-defined obesity by sex and age and identified the optimal BMI cut-off for BF%-defined obesity using receiver operating characteristic curve analysis. BMI-defined obesity (> or =25 kg/m2) was observed in 38.7% of men and 28.1% of women, with a high specificity (89%, men; 84%, women) but poor sensitivity (56%, men; 72% women) for BF%-defined obesity (25.2%, men; 31.1%, women). The optimal BMI cut-off (24.2 kg/m2) had 78% sensitivity and 71% specificity. BMI demonstrated limited diagnostic accuracy for adiposity in Korea. There was a -1.3 kg/m2 difference in optimal BMI cut-offs between Korea and America, smaller than the 5-unit difference between the Western Pacific Regional Office and global World Health Organization obesity criteria.


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Adipose Tissue/physiology , Adiposity/physiology , Body Mass Index , Cross-Sectional Studies , Nutrition Surveys , Obesity/diagnosis , ROC Curve , Reference Standards , Republic of Korea
15.
Clinical Pediatric Hematology-Oncology ; : 120-126, 2015.
Article in Korean | WPRIM | ID: wpr-71734

ABSTRACT

BACKGROUND: Currently, data on the role of tacrolimus and mini-dose methotrexate (MTX) in pediatric unrelated hematopoietic stem cell transplantation (HSCT) is limited. We report the outcomes of unrelated hematopoietic stem cell recipients, evaluating engraftment status, incidence of acute and chronic graft-versus-host disease (GVHD) and toxicities after use of tacrolimus and mini-dose MTX for GVHD prophylaxis. METHODS: Thirty-five children who received tacrolimus and mini-dose MTX as prophylaxis from January 2004 to December 2013 were reviewed. All patients received tacrolimus beginning the day prior to transplant at a dose of 0.03 mg/kg/day by continuous intravenous infusion. MTX was administered at a dose of 5 mg/m2 IV on days 1, 3, 6 and 11. RESULTS: Median age at transplantation was 8.42 years (range 0.75-18.9 years). Seventeen patients received human leukocyte antigen (HLA) fully matched donor transplants and 18 received partially mismatched transplants. All but two patients who received unrelated cord blood transplants showed successful engraftment. The median time to ANC recovery was 12 days. The incidence of acute GVHD was 33.3% including 15.1% grade III-IV GVHD. Localized chronic GVHD developed in only 2 of 27 (7.4%) evaluable patients. Lower tacrolimus levels during days 1-21 were associated with a higher incidence of acute GVHD (P=0.033). The estimated 4-year event free survival and overall survival of the patients were 71.2% and 80.0%. CONCLUSION: Overall, the combination of tacrolimus and mini-dose MTX could be effectively administered in the setting of pediatric unrelated HSCT.


Subject(s)
Child , Humans , Disease-Free Survival , Fetal Blood , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Hematopoietic Stem Cells , Incidence , Infusions, Intravenous , Korea , Leukocytes , Methotrexate , Tacrolimus , Tissue Donors
16.
Clinical Pediatric Hematology-Oncology ; : 181-185, 2015.
Article in English | WPRIM | ID: wpr-71723

ABSTRACT

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.


Subject(s)
Adolescent , Adult , Child , Humans , Male , Bone Marrow , Dendritic Cells , Diagnosis , Hematologic Neoplasms , Hematopoietic Stem Cell Transplantation , Induction Chemotherapy , Lymph Nodes , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Skin
17.
Allergy, Asthma & Respiratory Disease ; : 208-212, 2014.
Article in Korean | WPRIM | ID: wpr-17993

ABSTRACT

PURPOSE: Necrotizing pneumonia (NP) is a complication of invasive pneumonia. Few studies have reported on the clinical features of NP in children. METHODS: Nineteen patients with NP who were admitted to Pusan National University Hospital and Pusan National University Children's Hospital between December 2008 and March 2013, were studied in terms of sex, age at diagnosis, clinical characteristics, detected organisms, radiologic findings, treatments and clinical outcomes. RESULTS: Twelve patients (63.2%) were males and seven patients (36.8%) were female. Eight patients (44.4%) were younger than 5 years of age, seven patients (38.9%) were at 6-10 years of age and four patients (21.1%) were older than 11 years of age. Most patients had fever and cough at the time of diagnosis. Minorganism were identified in 9 cases (47.4%): Mycoplasma pneumonia in 4 (21.1%), Streptococcus pneumonia in 1 (5.3%), Staphylococcus aureus in 1 (5.3%), G+cocci in 1 (5.3%), and M. pneumonia + S. pneumoniae coinfection in 2 (10.5%). The diagnosis of NP was established by computerized tomography. Cavitary necrosis was shown in all patients, and pleural effusion was shown in 15 patients (78.9%). Six patients (31.6%) presented local atelectasis and 2 patients (10.5%) presented pneumothorax. Five patients (26.3%) treated with only antibiotics were cured completely. Invasive treatment was needed by 14 patients (73.7%): 10 patients (52.6%) needed chest tubing, and 5 patients (26.3%) needed surgery. Only one patient (5.3%) expired. CONCLUSION: NP is a rare complication of pneumonia in children and has a good prognosis, typically resolving completely with medical treatment alone. However, patients who present with the worsening of clinical status or pleural complications despite the use of appropriate antibiotics should be evaluated for surgical indications.


Subject(s)
Child , Female , Humans , Male , Anti-Bacterial Agents , Coinfection , Cough , Diagnosis , Fever , Necrosis , Pleural Effusion , Pneumonia , Pneumonia, Mycoplasma , Pneumothorax , Prognosis , Pulmonary Atelectasis , Staphylococcus aureus , Streptococcus , Thorax
18.
Tuberculosis and Respiratory Diseases ; : 511-515, 2011.
Article in English | WPRIM | ID: wpr-117507

ABSTRACT

The fat-forming variant of solitary fibrous tumors (SFTs) is a rare soft tissue neoplasm that was previously referred to as a lipomatous hemangiopericytoma (L-HPC). The most common affected site is deep soft tissue. Here, we present the first case, worldwide, of a fat-forming variant of SFT of the pleura. A 74-year-old man presented with left lower chest pain. Chest radiographs showed a mass-like lesion at the left lower lung field and chest computed tomography revealed a 12 cm fat-containing enhancing mass that was well-separated, lobulated and inhomogeneous. Radiology findings suggested a liposarcoma. Percutaneous needle biopsy was performed and pathological diagnosis of the mass was a fat-forming variant of SFT. Surgical resection was carried out and there has been no recurrence to date. So, a benign fat-forming variant of SFT must be considered as one of the differential diagnoses of lipomatous tumors of the pleura.


Subject(s)
Aged , Humans , Biopsy, Needle , Chest Pain , Diagnosis, Differential , Hemangiopericytoma , Lipoma , Liposarcoma , Lung , Pleura , Rectal Neoplasms , Recurrence , Soft Tissue Neoplasms , Solitary Fibrous Tumors , Thorax
19.
Journal of Korean Medical Science ; : 461-465, 2011.
Article in English | WPRIM | ID: wpr-84668

ABSTRACT

Polymorphism of 5,10-methylenetetrahydrofolate reductase (MTHFR) C677T is one of the suggested risk factors for atherosclerosis. However, few studies have reported on the relationship between MTHFR C677T polymorphism and vascular calcification (VC) in chronic hemodialysis patients. We investigated the relationship between the MTHFR C677T polymorphism and VC in 152 chronic hemodialysis patients. Patients with a TT genotype exhibited significantly higher VC scores than patients expressing CC and CT (P = 0.002). The prevalence of peripheral vascular disease increased with the incidence of MTHFR C677T mutations for all patients, and the incidence of cerebrovascular accidents also increased with the presence of mutations for young patients (< or = 60 yr) (P < 0.05). Patients with CT and TT genotypes had adjusted odds ratios for VC of 1.39 and 1.58, respectively (P < 0.05). In summary, these data suggest that the MTHFR C677T polymorphism affects the degree of VC in chronic hemodialysis patients.


Subject(s)
Aged , Humans , Middle Aged , Calcinosis/genetics , Genetic Predisposition to Disease , Kidney Failure, Chronic/genetics , Methylenetetrahydrofolate Reductase (NADPH2)/genetics , Polymorphism, Single Nucleotide , Renal Dialysis , Risk Factors , Vascular Diseases/genetics
20.
Genomics & Informatics ; : 77-79, 2006.
Article in English | WPRIM | ID: wpr-96576

ABSTRACT

In order to identify novel proapoptotic genes, we screened approximately 1,000 hypothetical genes whose functions are completely unknown. After these genes were transiently expressed in HeLa cells, their nuclei images were captured using automated high-speed fluorescence microscope, through which the ratio of apoptotic nuclei was estimated. We selected genes that induce greater than 3-fold increase in apoptotic nuclei compared to that of the vector control. The candidate proapoptotic genes were sequenced and their effects on cell death were further confirmed by the additional assay, DNA fragmentation ELISA. Finally, we were able to identify 4 full-length hypo-thetical genes with proapoptotic activity.


Subject(s)
Humans , Apoptosis , Cell Death , DNA Fragmentation , Enzyme-Linked Immunosorbent Assay , Fluorescence , HeLa Cells
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