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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.
Blood Research ; : 86-94, 2022.
Article in English | WPRIM | ID: wpr-937252

ABSTRACT

Although the prevalence of hereditary hemolytic anemia (HHA) is relatively low in Korea, it has been gradually increasing in recent decades due to increment in the proportions of hemoglobinopathies from immigrants of South East Asia, raising awareness of the disease among clinicians, and advances in diagnostic technology. As such, the red blood cell (RBC) Disorder Working Party (WP), previously called HHA WP, of the Korean Society of Hematology (KSH) developed the Korean Standard Operating Procedures (SOPs) for the diagnosis of HHA in 2007. These SOPs have been continuously revised and updated following advances in diagnostic technology [e.g., flow cytometric osmotic fragility test (FOFT) and eosin-5-maleimide (EMA) binding test], current methods for membrane protein or enzyme analysis [e.g., liquid chromatography-tandem mass spectrometry (LC-MS/MS), ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS), high-performance liquid chromatography (HPLC)], and molecular genetic tests using next-generation sequencing (NGS). However, the diagnosis and treatment of HHA remain challenging as they require considerable experience and understanding of the disease. Therefore, in this new Korean Clinical Practice Guidelines for the Diagnosis of HHA, on behalf of the RBC Disorder WP of KSH, updated guidelines to approach patients suspected of HHA are summarized. NGS is proposed to perform prior to membrane protein or enzyme analysis by LC-MS/MS, UPLC-MS/MS or HPLC techniques due to the availability of gene testing in more laboratories in Korea. We hope that this guideline will be helpful for clinicians in making diagnostic decisions for patients with HHA in Korea.

3.
Cancer Research and Treatment ; : 378-388, 2021.
Article in English | WPRIM | ID: wpr-889726

ABSTRACT

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

4.
Cancer Research and Treatment ; : 378-388, 2021.
Article in English | WPRIM | ID: wpr-897430

ABSTRACT

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

5.
Blood Research ; : 6-16, 2021.
Article in English | WPRIM | ID: wpr-874333

ABSTRACT

Venous thromboembolism (VTE), which includes pulmonary embolism and deep vein thrombosis, is a condition characterized by abnormal blood clot formation in the pulmonary arteries and the deep venous vasculature. It is often serious and sometimes even fatal if not promptly and appropriately treated. Moreover, the later consequences of VTE may result in reduced quality of life. The treatment of VTE depends on various factors, including the type, cause, and patient comorbidities. Furthermore, bleeding may occur as a side effect of VTE treatment. Thus, it is necessary to carefully weigh the benefits versus the risks of VTE treatment and to actively monitor patients undergoing treatment. Asian populations are known to have lower VTE incidences than Western populations, but recent studies have shown an increase in the incidence of VTE in Asia. A variety of treatment options are currently available owing to the introduction of direct oral anticoagulants.The current VTE treatment recommendation is based on evidence from previous studies, but it should be applied with careful consideration of the racial, genetic, and social characteristics in the Korean population.

6.
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.

7.
Journal of Korean Medical Science ; : e279-2020.
Article | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

8.
Cancer Research and Treatment ; : 438-445, 2020.
Article | WPRIM | ID: wpr-831054

ABSTRACT

Purpose@#Loss of heterozygosity (LOH) at chromosomes 1p and 16q is a poor prognostic factor infavorable histology Wilms tumor (FHWT). This study investigated the prevalence of LOH at1p and 16q and evaluated its prognostic value in Korean children with FHWT. @*Materials and Methods@#We analyzed 101 FHWT patients who were diagnosed between 1996 and 2016 in KoreanSociety of Pediatric Hematology Oncology Group hospitals. Using paraffin-embedded kidneytissue samples sent from each center, we reviewed LOH at 1p and 16q in each patient andassessed the prognostic value of LOH status for clinical parameters affecting event-freesurvival (EFS). @*Results@#Of the 101 patients, 12 (11.9%) experienced recurrence; the 3-year EFS was 87.6%. LOHat 1p or 16q was detected in 19 patients (18.8%), with five having LOH at both 1q and 16q.The frequency of LOH at 1p was higher among younger patients (p=0.049), but there wasno difference in LOH prevalence according to tumor stage. In the multivariate analysis, LOHat 16q was a significant negative prognostic factor affecting EFS (3-year EFS, 73.7% vs.91.1%; hazard ratio, 3.95; p=0.037), whereas LOH at 1p was not (p=0.786). @*Conclusion@#LOH at 16q was a significant negative prognostic factor affecting outcome in Korean pediatricFHWT patients. Due to the small sample size of this study, large-scale multicenter trialsare warranted to investigate the prognostic value of LOH at 1p and 16q in Korean childrenwith FHWT.

9.
Brain Tumor Research and Treatment ; : 1-9, 2019.
Article in English | WPRIM | ID: wpr-739672

ABSTRACT

BACKGROUND: There has been no practical guidelines for the management of patients with central nervous system (CNS) tumors in Korea for many years. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, started to prepare guidelines for CNS tumors from February 2018. METHODS: The Working Group was composed of 35 multidisciplinary medical experts in Korea. References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of keywords. RESULTS: First, the maximal safe resection if feasible is recommended. After the diagnosis of a glioblastoma with neurosurgical intervention, patients aged ≤70 years with good performance should be treated by concurrent chemoradiotherapy with temozolomide followed by adjuvant temozolomide chemotherapy (Stupp's protocol) or standard brain radiotherapy alone. However, those with poor performance should be treated by hypofractionated brain radiotherapy (preferred)±concurrent or adjuvant temozolomide, temozolomide alone (Level III), or supportive treatment. Alternatively, patients aged >70 years with good performance should be treated by hypofractionated brain radiotherapy+concurrent and adjuvant temozolomide or Stupp's protocol or hypofractionated brain radiotherapy alone, while those with poor performance should be treated by hypofractionated brain radiotherapy alone or temozolomide chemotherapy if the patient has methylated MGMT gene promoter (Level III), or supportive treatment. CONCLUSION: The KSNO's guideline recommends that glioblastomas should be treated by maximal safe resection, if feasible, followed by radiotherapy and/or chemotherapy according to the individual comprehensive condition of the patient.


Subject(s)
Humans , Brain , Central Nervous System , Chemoradiotherapy , Diagnosis , Drug Therapy , Glioblastoma , Korea , Radiotherapy
10.
Cancer Research and Treatment ; : 357-367, 2019.
Article in English | WPRIM | ID: wpr-719418

ABSTRACT

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.


Subject(s)
Humans , Anthracyclines , Cardiotoxicity , Dexrazoxane , Disease-Free Survival , Follow-Up Studies , Incidence , Korea , Multivariate Analysis , Neoplasms, Second Primary , Risk Factors , Stem Cell Transplantation
11.
Brain Tumor Research and Treatment ; : 63-73, 2019.
Article in English | WPRIM | ID: wpr-763112

ABSTRACT

BACKGROUND: There was no practical guideline for the management of patients with central nervous system tumor in Korea in the past. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, developed the guideline for glioblastoma successfully and published it in Brain Tumor Research and Treatment, the official journal of KSNO, in April 2019. Recently, the KSNO guideline for World Health Organization (WHO) grade III cerebral glioma in adults has been established. METHODS: The Working Group was composed of 35 multidisciplinary medical experts in Korea. References were identified by searches in PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL databases using specific and sensitive keywords as well as combinations of keywords. Scope of the disease was confined to cerebral anaplastic astrocytoma and oligodendroglioma in adults. RESULTS: Whenever radiological feature suggests high grade glioma, maximal safe resection if feasible is globally recommended. After molecular and histological examinations, patients with anaplastic astrocytoma, isocitrate dehydrogenase (IDH)-mutant should be primary treated by standard brain radiotherapy and adjuvant temozolomide chemotherapy whereas those with anaplastic astrocytoma, NOS, and anaplastic astrocytoma, IDH-wildtype should be treated following the protocol for glioblastomas. In terms of anaplastic oligodendroglioma, IDH-mutant and 1p19q-codeletion, and anaplastic oligodendroglioma, NOS should be primary treated by standard brain radiotherapy and neoadjuvant or adjuvant PCV (procarbazine, lomustine, and vincristine) combination chemotherapy. CONCLUSION: The KSNO's guideline recommends that WHO grade III cerebral glioma of adults should be treated by maximal safe resection if feasible, followed by radiotherapy and/or chemotherapy according to molecular and histological features of tumors.


Subject(s)
Adult , Humans , Astrocytoma , Brain , Brain Neoplasms , Central Nervous System , Drug Therapy , Drug Therapy, Combination , Glioblastoma , Glioma , Isocitrate Dehydrogenase , Korea , Lomustine , Oligodendroglioma , Radiotherapy , World Health Organization
12.
Brain Tumor Research and Treatment ; : 74-84, 2019.
Article in English | WPRIM | ID: wpr-763111

ABSTRACT

BACKGROUND: There was no practical guideline for the management of patients with central nervous system tumor in Korea for many years. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has developed the guideline for glioblastoma. Subsequently, the KSNO guideline for World Health Organization (WHO) grade II cerebral glioma in adults is established. METHODS: The Working Group was composed of 35 multidisciplinary medical experts in Korea. References were identified by searching PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL databases using specific and sensitive keywords as well as combinations of keywords regarding diffuse astrocytoma and oligodendroglioma of brain in adults. RESULTS: Whenever radiological feature suggests lower grade glioma, the maximal safe resection if feasible is recommended globally. After molecular and histological examinations, patients with diffuse astrocytoma, isocitrate dehydrogenase (IDH)-wildtype without molecular feature of glioblastoma should be primarily treated by standard brain radiotherapy and adjuvant temozolomide chemotherapy (Level III) while those with molecular feature of glioblastoma should be treated following the protocol for glioblastomas. In terms of patients with diffuse astrocytoma, IDH-mutant and oligodendroglioma (IDH-mutant and 1p19q codeletion), standard brain radiotherapy and adjuvant PCV (procarbazine+lomustine+vincristine) combination chemotherapy should be considered primarily for the high-risk group while observation with regular follow up should be considered for the low-risk group. CONCLUSION: The KSNO's guideline recommends that WHO grade II gliomas should be treated by maximal safe resection, if feasible, followed by radiotherapy and/or chemotherapy according to molecular and histological features of tumors and clinical characteristics of patients.


Subject(s)
Adult , Humans , Astrocytoma , Brain , Central Nervous System , Drug Therapy , Drug Therapy, Combination , Follow-Up Studies , Glioblastoma , Glioma , Isocitrate Dehydrogenase , Korea , Oligodendroglioma , Radiotherapy , World Health Organization
14.
Nutrition Research and Practice ; : 214-221, 2019.
Article in English | WPRIM | ID: wpr-760608

ABSTRACT

BACKGROUNDS/OBJECTIVES: Cancer treatment may lead to significant body composition changes and affect growth and disease outcomes in pediatric cancer patients. This prospective study aimed to evaluate short- and long-term body compositions changes focused on body fat during the first year of cancer treatment in children. SUBJECTS/METHODS: A prospective study was conducted in 30 pediatric cancer patients (19 hematologic malignancies and 11 solid tumors) and 30 age- and sex-matched healthy controls. Anthropometric measurements and body composition analysis using whole body dual energy X-ray absorptiometry were performed at baseline and 1, 6, and 12 month(s) of cancer treatment. Kruskal-Wallis tests, Wilcoxon paired t tests, and generalized estimation equation (GEE) were applied for statistical analysis. RESULTS: At baseline, no differences in weight, height, body mass index, abdominal circumferences, body fat, and fat-free mass were observed between 30 controls and 30 pediatric cancer patients. Total fat mass (P < 0.001) and body fat percentage (P = 0.002) increased significantly during the first month, but no changes were observed from 1 to 12 months; however, no changes in the total mass were observed during the first year of cancer treatment. Meanwhile, the total fat-free mass decreased during the first month (P = 0.008) and recovered between 6 and 12 months of follow-up (P < 0.001). According to GEE analysis, there was a significant upward trend in body fat percentage during the first year, especially the first month, of cancer treatment in children with hematologic malignancies, but not in those with solid tumors. CONCLUSIONS: Our results indicate that cancer treatment is related to significant body composition changes and rapid body fat gain, particularly during the first month after initiating cancer treatment, in children with hematologic malignancies. Therefore, individualized dietary strategies to prevent excessive fat gain are needed in pediatric cancer patients for better outcomes.


Subject(s)
Child , Humans , Absorptiometry, Photon , Adipose Tissue , Body Composition , Body Fat Distribution , Body Height , Follow-Up Studies , Hematologic Neoplasms , Prospective Studies
15.
16.
Clinical Pediatric Hematology-Oncology ; : 162-168, 2017.
Article in English | WPRIM | ID: wpr-788602

ABSTRACT

Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Although it may develop in any organs, ES/PNET originating from small intestine is exceedingly rare. We experienced a 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia. Imaging work-up showed multiple masses in the small bowel and omentum with disseminated peritoneal seeding nodules, indicating lymphoma as the most likely diagnosis. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising small round cells with CD99 expression and EWS-FLI1 translocation leading to the diagnosis of ES/PNET. Tumor burden decreased gradually during five consecutive cycles of systemic chemotherapy. The patient received segmental resection of jejunum, followed by adjuvant chemotherapy. This is the first pediatric case of ES/PNET found in small intestine in Korea.


Subject(s)
Child , Female , Humans , Abdominal Pain , Anemia, Iron-Deficiency , Biopsy , Chemotherapy, Adjuvant , Diagnosis , Drug Therapy , Intestine, Small , Jejunum , Korea , Lymphoma , Melena , Neural Plate , Neuroectodermal Tumors , Neuroectodermal Tumors, Primitive , Omentum , Pediatrics , Sarcoma, Ewing , Tumor Burden
17.
Clinical Pediatric Hematology-Oncology ; : 1-10, 2017.
Article in Korean | WPRIM | ID: wpr-788601

ABSTRACT

Although the incidence of venous thromboembolism (VTE) is significantly lower than in adults, recognition of VTE in children is increasing as advanced medical care enhances treatment intensity in pediatric patients. VTE in children usually develops as a secondary complication of underlying clinical conditions such as venous catheterization, malignancy, infections, trauma, surgery, and inherited or acquired thrombophilia, of which venous catheterization poses the highest risk. Neonates are at the greatest risk for VTE with a second peak in incidence during adolescence. There is some debate regarding which patients should have testing for inherited risk factors such as factor V Leiden, prothrombin G20210A, protein C-, protein S- and antithrombin deficiency. Guidelines for diagnosis and treatment of VTE in children are mostly extrapolated from adult data, despite the uniqueness of pediatric hemostatic system. The most common treatment is unfractionated heparin or low molecular weight heparin with vitamin K antagonist, whereas newly developed direct oral anticoagulants are under discussion and have been evaluated in only a small number of clinical trials in pediatric patients. Prospective multicenter collaborative research is necessary to develop validated guidelines for the diagnosis, antithrombotic therapy, prevention and follow-up monitoring of pediatric VTE.


Subject(s)
Adolescent , Adult , Child , Humans , Infant, Newborn , Anticoagulants , Catheterization , Catheters , Diagnosis , Factor V , Follow-Up Studies , Heparin , Heparin, Low-Molecular-Weight , Incidence , Prospective Studies , Prothrombin , Risk Factors , Thrombophilia , Venous Thromboembolism , Vitamin K
18.
Clinical Pediatric Hematology-Oncology ; : 1-10, 2017.
Article in Korean | WPRIM | ID: wpr-110912

ABSTRACT

Although the incidence of venous thromboembolism (VTE) is significantly lower than in adults, recognition of VTE in children is increasing as advanced medical care enhances treatment intensity in pediatric patients. VTE in children usually develops as a secondary complication of underlying clinical conditions such as venous catheterization, malignancy, infections, trauma, surgery, and inherited or acquired thrombophilia, of which venous catheterization poses the highest risk. Neonates are at the greatest risk for VTE with a second peak in incidence during adolescence. There is some debate regarding which patients should have testing for inherited risk factors such as factor V Leiden, prothrombin G20210A, protein C-, protein S- and antithrombin deficiency. Guidelines for diagnosis and treatment of VTE in children are mostly extrapolated from adult data, despite the uniqueness of pediatric hemostatic system. The most common treatment is unfractionated heparin or low molecular weight heparin with vitamin K antagonist, whereas newly developed direct oral anticoagulants are under discussion and have been evaluated in only a small number of clinical trials in pediatric patients. Prospective multicenter collaborative research is necessary to develop validated guidelines for the diagnosis, antithrombotic therapy, prevention and follow-up monitoring of pediatric VTE.


Subject(s)
Adolescent , Adult , Child , Humans , Infant, Newborn , Anticoagulants , Catheterization , Catheters , Diagnosis , Factor V , Follow-Up Studies , Heparin , Heparin, Low-Molecular-Weight , Incidence , Prospective Studies , Prothrombin , Risk Factors , Thrombophilia , Venous Thromboembolism , Vitamin K
19.
Clinical Pediatric Hematology-Oncology ; : 162-168, 2017.
Article in English | WPRIM | ID: wpr-23100

ABSTRACT

Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Although it may develop in any organs, ES/PNET originating from small intestine is exceedingly rare. We experienced a 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia. Imaging work-up showed multiple masses in the small bowel and omentum with disseminated peritoneal seeding nodules, indicating lymphoma as the most likely diagnosis. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising small round cells with CD99 expression and EWS-FLI1 translocation leading to the diagnosis of ES/PNET. Tumor burden decreased gradually during five consecutive cycles of systemic chemotherapy. The patient received segmental resection of jejunum, followed by adjuvant chemotherapy. This is the first pediatric case of ES/PNET found in small intestine in Korea.


Subject(s)
Child , Female , Humans , Abdominal Pain , Anemia, Iron-Deficiency , Biopsy , Chemotherapy, Adjuvant , Diagnosis , Drug Therapy , Intestine, Small , Jejunum , Korea , Lymphoma , Melena , Neural Plate , Neuroectodermal Tumors , Neuroectodermal Tumors, Primitive , Omentum , Pediatrics , Sarcoma, Ewing , Tumor Burden
20.
Blood Research ; : 164-170, 2016.
Article in English | WPRIM | ID: wpr-209258

ABSTRACT

BACKGROUND: While venous thromboembolism (VTE) is uncommon, its incidence is increasing in children. We aimed to evaluate the incidence, risk factors, treatment, and outcome of pediatric VTE cases at a single tertiary hospital in Korea. METHODS: We retrospectively analyzed the records of consecutive pediatric VTE patients admitted to the Seoul National University Bundang Hospital between April 2003 and March 2016. RESULTS: Among 70,462 hospitalizations, 25 pediatric VTE cases were identified (3.27 cases per 10,000 admissions). Fifteen patients (60%) were male, 8 were neonates (32%), and the median age at diagnosis was 10.9 years (range, 0 days‒17 yr). Doppler ultrasonography was the most frequently used imaging modality. Thrombosis occurred in the intracerebral (20%), upper venous (64%), lower venous (12%), and combined upper and lower venous systems (4%). Twenty patients (80%) had underlying clinical conditions including venous catheterization (24%), malignancy (20%), and systemic diseases (12%). Protein C, protein S, and antithrombin deficiencies occurred in 2 of 13, 4 of 13, and 1 of 14 patients tested, respectively. Six patients were treated with heparin followed by warfarin, while 4 were treated with heparin or warfarin. Thrombectomy and inferior vena cava filter and/or thrombolysis were performed in 5 patients. Two patients died of pulmonary embolism, and 2 developed a post-thrombotic syndrome. CONCLUSION: Compared with the reports from Western countries, VTE occurrence was lower in the Korean pediatric population under study, although similar clinical characteristics including bimodal age distribution, underlying diseases, treatment pattern, and outcomes were observed.


Subject(s)
Child , Humans , Infant, Newborn , Male , Age Distribution , Catheterization , Catheters , Diagnosis , Epidemiology , Heparin , Hospitalization , Incidence , Korea , Protein C , Protein S , Pulmonary Embolism , Retrospective Studies , Risk Factors , Seoul , Tertiary Care Centers , Thrombectomy , Thrombosis , Ultrasonography, Doppler , Vena Cava Filters , Venous Thromboembolism , Warfarin
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