Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data / Journal of the Korean Cancer Association, 대한암학회지

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.

Korean clinical practice guidelines for the diagnosis of hereditary hemolytic anemia

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.

Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma:a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study

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.

Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study

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.

Clinical characteristics of children who visited the emergency department with extended-spectrum beta-lactamase-producing Escherichia coli urinary tract infection and its risk factors

Purpose@#To identify the differences in features between children with urinary tract infection (UTI) caused by extended-spectrum beta-lactamases (ESBL)-positive and -negative Escherichia coli, and analyze risk factors for the former infection. @*Methods@#We reviewed medical records of children younger than 36 months with E. coli UTI who visited the emergency department from January 2012 through January 2019. Differences in variables regarding clinical, laboratory, and microbiologic (i.e., ESBL-positive E. coli on urine culture) features, and outcomes between the ESBL-positive and -negative groups were identified. Factors associated with ESBL-positive E. coli infection were analyzed by logistic regression. @*Results@#The children were classified into the ESBL-positive (n = 151) and -negative (n = 40) groups. The former group showed higher frequency of prior UTI (P = 0.038) without other differences between the groups. The median counts of white blood cells, absolute neutrophils, and absolute lymphocytes were higher in the ESBL-positive group than in the other group (P = 0.009, 0.022, and 0.027, respectively). The former group showed longer median hospital length of stay (11.0 days [interquartile range, 8.9-12.0] vs. 6.0 [5.0-7.0]; P < 0.001), and more frequent recurrence per child (3.0 [2.5-3.0] vs. 1.0 [1.0-1.75]; P = 0.047) and presence of vesicoureteral reflux (27.5% vs. 13.2%; P = 0.001). Logistic regression showed leukocytosis (odds ratio, 12.85; 95% confidence interval, 1.04-157.69) and vesicoureteral reflux (4.00; 1.19-13.43) as the factors for ESBL-positive E. coli infection. @*Conclusion@#The ESBL-positive group showed significantly higher leukocyte count and rate of vesicoureteral reflux than the ESBL-negative group. For children with these features, empirical antibiotics should be chosen in consideration of the resistant bacteria.

Clinical characteristics of children who visited the emergency department with extended-spectrum beta-lactamase-producing Escherichia coli urinary tract infection and its risk factors

Purpose@#To identify the differences in features between children with urinary tract infection (UTI) caused by extended-spectrum beta-lactamases (ESBL)-positive and -negative Escherichia coli, and analyze risk factors for the former infection. @*Methods@#We reviewed medical records of children younger than 36 months with E. coli UTI who visited the emergency department from January 2012 through January 2019. Differences in variables regarding clinical, laboratory, and microbiologic (i.e., ESBL-positive E. coli on urine culture) features, and outcomes between the ESBL-positive and -negative groups were identified. Factors associated with ESBL-positive E. coli infection were analyzed by logistic regression. @*Results@#The children were classified into the ESBL-positive (n = 151) and -negative (n = 40) groups. The former group showed higher frequency of prior UTI (P = 0.038) without other differences between the groups. The median counts of white blood cells, absolute neutrophils, and absolute lymphocytes were higher in the ESBL-positive group than in the other group (P = 0.009, 0.022, and 0.027, respectively). The former group showed longer median hospital length of stay (11.0 days [interquartile range, 8.9-12.0] vs. 6.0 [5.0-7.0]; P < 0.001), and more frequent recurrence per child (3.0 [2.5-3.0] vs. 1.0 [1.0-1.75]; P = 0.047) and presence of vesicoureteral reflux (27.5% vs. 13.2%; P = 0.001). Logistic regression showed leukocytosis (odds ratio, 12.85; 95% confidence interval, 1.04-157.69) and vesicoureteral reflux (4.00; 1.19-13.43) as the factors for ESBL-positive E. coli infection. @*Conclusion@#The ESBL-positive group showed significantly higher leukocyte count and rate of vesicoureteral reflux than the ESBL-negative group. For children with these features, empirical antibiotics should be chosen in consideration of the resistant bacteria.

Risk Factor Analysis for Secondary Malignancy in Dexrazoxane-Treated Pediatric Cancer Patients / Journal of the Korean Cancer Association, 대한암학회지

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.

Current Assessment and Management of Retinoblastoma / 임상소아혈액종양

Retinoblastoma is the most common intraocular malignancy in childhood. Diagnosis is currently made by ophthalmologists under general anesthesia as it is the gold standard for intraocular assessment. However, evaluations for extraocular disease are also necessary. Treatment strategies vary according to the disease status. If a single eye is involved, the treatment goal is oriented to the removal of the tumor and prevention of relapse. In bilateral retinoblastoma, the main treatment goal is to save monocular vision and save life. This article will explore the available treatment options for retinoblastoma including enucleation, radiotherapy, local therapy, intravenous chemotherapy, intra-arterial injection and intra-vitreal injections. There were recent advances in our understanding on the genetic pathophysiology of the retinoblastoma protein gene in tumorigenesis, which may help developing future treatment. Early detection of retinoblastoma is important for prolonging survival and improving quality of life.

A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region / 임상소아혈액종양

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.

Iodine status and characteristics of Korean adolescents and their parents based on urinary iodine concentration: a nationwide cross-sectional study

PURPOSE: Iodine is a major component of thyroid hormones. Both deficiency and excess of iodine are major risk factors for thyroid disease, making it important to accurately assess iodine level in the human body. Urinary iodine concentration (UIC) is a commonly used measure of iodine status. However, there is little research on iodine status and related characteristics in Korean adolescents. METHODS: Using data from the sixth Korea National Health and Nutrition Examination Survey (KNHANES VI) for the years 2013–2015, we analyzed UIC and thyroid function test results in adolescents aged 10–18 years and their parents. We also investigated the influence of socioeconomic factors and family history of thyroid disease on iodine status. RESULTS: Mean UIC in Korean adolescents was 963.5±55.7 μg/L. Among evaluated subjects, 6.6%±1.0%, 29.8%±1.7%, and 63.9%±1.9% had low (UIC<100 μg/L), adequate (UIC: 100–299.9 μg/L), and excess (UIC≥300 μg/L) iodine concentrations, respectively. Based on regional trends, the incidence of iodine deficiency exceeded 10% in several regions, even though there was a dominance of regions with excess iodine. Parental UIC, female sex, average monthly income, and expenditure were all found to affect the iodine status of Korean adolescents. CONCLUSIONS: Although regional differences exist, the iodine status in Korean adolescents is mainly affected by the eating habits of their families and socioeconomic factors. Therefore, monitoring of iodine status is necessary in this population.

Family History as a Risk Factor for Iron Deficiency Anemia among Korean Adolescents: Data from the Fifth Korea National Health and Nutrition Examination Survey (KNHANES) / 임상소아혈액종양

BACKGROUND: Iron deficiency anemia (IDA) is a disease prevalent throughout the world. However, there is limited information regarding whether familial factors are associated with the risk of adolescent IDA. METHODS: This study evaluated the association between adolescent IDA and family history of IDA using data from the fifth Korea National Health Nutrition Survey (2010–2012). Data from 10-18-year-old children who underwent laboratory testing were analyzed. RESULTS: The overall prevalence of IDA was 3.1% (95% confidence interval [CI]: 2.4−4.1%), with prevalence of 0.5% among boys (95% CI: 0.2−1.3%) and 6.2% among girls (95% CI: 4.6−8.3%). The prevalence of IDA was associated with female sex (odds ratio [OR]: 13.43, 95% CI: 4.92−36.65; P < 0.001) and a family history of IDA (OR: 3.12, 95% CI: 1.11−8.76; P=0.03). Other risk factors for IDA were receiving social welfare support (OR: 3.31, 95% CI: 1.45−7.56; P=0.031), low maternal education (OR: 3.12, 95% CI: 1.39−6.99; P=0.006), receiving charitable food support (OR: 2.27: 95% CI: 0.9−5.44; P=0.04), poor body-image (OR: 2.14, 95% CI: 1.16−3.93; P=0.026), and weight-loss efforts (OR: 2.42, 95% CI: 1.27−4.61; P=0.01). Nutritional supplementation protected against IDA (OR: 0.40, 95% CI: 0.19−0.82; P=0.007), although adolescents with awareness of nutritional labels had a high IDA prevalence (OR: 8.06, 95% CI: 1.71−38.05; P < 0.001). CONCLUSION: A family history of IDA was an independent risk factor for IDA. Further studies are needed to determine whether family-level educational interventions can reduce the risk of adolescent IDA.

Childhood Venous Thromboembolism in Yeungnam Region in Korea: Multicenter Study / 임상소아혈액종양

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.

Erratum: The effect of first nocturnal ejaculation timing on risk and sexual behaviors of Korean male adolescents

The ethical statement was omitted unintentionally.

The effect of first nocturnal ejaculation timing on risk and sexual behaviors of Korean male adolescents

PURPOSE: This study evaluated the effect of first nocturnal ejaculation timing on risk and sexual behaviors of Korean male adolescents. METHODS: We analyzed data from the 10th edition of the Korea Youth Risk Behavior Web-based survey that was conducted with male high school adolescents in grades 10–12. The survey included 17,907 adolescents, and 10,326 responded their experience of first nocturnal ejaculation. Of these, 595 had their first nocturnal ejaculation in ≤grade 4 (“early puberty”) and 9,731 had their first nocturnal ejaculation in ≥grade 5 (“normal puberty”). We analyzed differences between these 2 groups in risk and sexual behaviors. RESULTS: Early first nocturnal ejaculation showed a positive association with sexual intercourse (odds ratio [OR], 3.27; 95% confidence interval [CI], 2.56–4.17), sexual debut at elementary school age (OR, 7.45; 95% CI, 5.00–11.10), and having had a sexually transmitted disease (OR, 6.60; 95% CI, 3.94–11.08). After a multiple logistic regression to adjust for socio-demographic variables, early first nocturnal ejaculation was still positively associated with sexual intercourse (OR, 2.73; 95% CI, 2.03–3.69), sexual debut at elementary school age (OR, 5.96; 95% CI, 3.47–10.22), and having had a sexually transmitted disease (OR, 5.17; 95% CI, 2.52–10.20). Early first nocturnal ejaculation was positively associated with alcohol consumption, smoking, and substance use. However, this was not statistically significant after adjusting for several socio-demographic variables. CONCLUSION: There is a positive association between early nocturnal ejaculation and sexual behaviors in male adolescents. Proactive education about sexual behaviors is required for adolescents who reach sexual maturity early.

Metabolic syndrome induced by anticancer treatment in childhood cancer survivors

The number of childhood cancer survivors is increasing as survival rates improve. However, complications after treatment have not received much attention, particularly metabolic syndrome. Metabolic syndrome comprises central obesity, dyslipidemia, hypertension, and insulin resistance, and cancer survivors have higher risks of cardiovascular events compared with the general population. The mechanism by which cancer treatment induces metabolic syndrome is unclear. However, its pathophysiology can be categorized based on the cancer treatment type administered. Brain surgery or radiotherapy may induce metabolic syndrome by damaging the hypothalamic-pituitary axis, which may induce pituitary hormone deficiencies. Local therapy administered to particular endocrine organs directly damages the organs and causes hormone deficiencies, which induce obesity and dyslipidemia leading to metabolic syndrome. Chemotherapeutic agents interfere with cell generation and growth, damage the vascular endothelial cells, and increase the cardiovascular risk. Moreover, chemotherapeutic agents induce oxidative stress, which also induces metabolic syndrome. Physical inactivity caused by cancer treatment or the cancer itself, dietary restrictions, and the frequent use of antibiotics may also be risk factors for metabolic syndrome. Since childhood cancer survivors with metabolic syndrome have higher risks of cardiovascular events at an earlier age, early interventions should be considered. The optimal timing of interventions and drug use has not been established, but lifestyle modifications and exercise interventions that begin during cancer treatment might be beneficial and tailored education and interventions that account for individual patients' circumstances are needed. This review evaluates the recent literature that describes metabolic syndrome in cancer survivors, with a focus on its pathophysiology.

Neutrophil to Lymphocyte Ratio (NLR) as a Predictive Marker for Prognosis in the Patients with Immune Thrombocytopenic Purpura (ITP) / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting low platelet count in peripheral blood without any other abnormalities in other blood cell lineage. There were many studies that investigated the mechanism of ITP and the factors related to its chronicity, but much remains controversial. Neutrophil to lymphocyte ratio (NLR) is considered an indicator representing the severity of acute or chronic inflammation. The aim of this study was to assess whether NLR can predict the outcome of ITP and its chronicity.METHODS: Patients who were diagnosed as ITP at Dong-A University Hospital between March 2000 and March 2016 were analyzed retrospectively. NLR results and other medical records at diagnosis and after treatment were analyzed and compared between two groups; patients with single episode and chronic ITP group.RESULTS: Of a total of 67 patients, 39 patients (58.21%) were chronic ITP. Patients with single episode showed low NLR at diagnosis than patients with chronic ITP (1.23±1.38 vs. 1.54±1.15, P=0.42), but NLR after recovery were significantly high (1.58±1.71 vs. 1.31±0.76, P=0.21). However, patients with chronic ITP showed a trend of high NLR at the time of relapse.CONCLUSION: Chronic ITP patients showed higher NLR at the time of diagnosis compared with the patients with single episode. Also, the NLR at the time of relapse showed higher level compared to those at diagnosis. Relationship between NLR and chronic ITP need to be further studied.

Treatment and Management of Late Complications in Hereditary Hemolytic Anemia / 임상소아혈액종양

Hereditary hemolytic anemia is a very heterogeneous disorder in which abnormalities of red blood cell structural protein, globin protein, or enzyme defect lead to shortened life span. There has been much progress in revealing its pathophysiology and genetic backgrounds, but the lifelong plans for caring these patients are not well established yet. All patients with hereditary hemolytic anemic have three common problems: transfusion dependency, iron overload and iron chelation therapy. Patients with hereditary spherocytosis (HS) usually manifest severe anemia in neonatal period and infancy, but transfusion requirements may decrease in adulthood. But patients with thalassemia or sickle cell disease usually transfusion-dependent throughout life. Maintaining the optimal hemoglobin (Hb) levels in these patients is crucial because correction of anemia and dilution of abnormal Hb helps prevent certain complications that frequently occur in these patients. Frequent transfusion leads to transfusion-mediated infection and hemochromatosis. Iron chelation therapy should be started early to prevent permanent organ damage. Folate therapy can be helpful in patients with hereditary spherocytosis. Regular evaluations for cholestasis should be started at age 5, and splenectomy with concurrent cholecystectomy can be considered if the patient has cholecystitis. Hydroxyurea can be used to reduce transfusion requirements and prevent complications in patients with β-thalassemia and sickle cell disease. Consensus on long-term management of patients with hereditary hemolytic anemia is lacking, especially for adult patients. But further efforts to build guidelines for long-term follow-up and management of the patients with hereditary hemolytic anemia in the context of Korean society are needed.

Treatment and Management of Late Complications in Hereditary Hemolytic Anemia / 임상소아혈액종양

Hereditary hemolytic anemia is a very heterogeneous disorder in which abnormalities of red blood cell structural protein, globin protein, or enzyme defect lead to shortened life span. There has been much progress in revealing its pathophysiology and genetic backgrounds, but the lifelong plans for caring these patients are not well established yet. All patients with hereditary hemolytic anemic have three common problems: transfusion dependency, iron overload and iron chelation therapy. Patients with hereditary spherocytosis (HS) usually manifest severe anemia in neonatal period and infancy, but transfusion requirements may decrease in adulthood. But patients with thalassemia or sickle cell disease usually transfusion-dependent throughout life. Maintaining the optimal hemoglobin (Hb) levels in these patients is crucial because correction of anemia and dilution of abnormal Hb helps prevent certain complications that frequently occur in these patients. Frequent transfusion leads to transfusion-mediated infection and hemochromatosis. Iron chelation therapy should be started early to prevent permanent organ damage. Folate therapy can be helpful in patients with hereditary spherocytosis. Regular evaluations for cholestasis should be started at age 5, and splenectomy with concurrent cholecystectomy can be considered if the patient has cholecystitis. Hydroxyurea can be used to reduce transfusion requirements and prevent complications in patients with β-thalassemia and sickle cell disease. Consensus on long-term management of patients with hereditary hemolytic anemia is lacking, especially for adult patients. But further efforts to build guidelines for long-term follow-up and management of the patients with hereditary hemolytic anemia in the context of Korean society are needed.

Neutrophil to Lymphocyte Ratio (NLR) as a Predictive Marker for Prognosis in the Patients with Immune Thrombocytopenic Purpura (ITP) / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting low platelet count in peripheral blood without any other abnormalities in other blood cell lineage. There were many studies that investigated the mechanism of ITP and the factors related to its chronicity, but much remains controversial. Neutrophil to lymphocyte ratio (NLR) is considered an indicator representing the severity of acute or chronic inflammation. The aim of this study was to assess whether NLR can predict the outcome of ITP and its chronicity. METHODS: Patients who were diagnosed as ITP at Dong-A University Hospital between March 2000 and March 2016 were analyzed retrospectively. NLR results and other medical records at diagnosis and after treatment were analyzed and compared between two groups; patients with single episode and chronic ITP group. RESULTS: Of a total of 67 patients, 39 patients (58.21%) were chronic ITP. Patients with single episode showed low NLR at diagnosis than patients with chronic ITP (1.23±1.38 vs. 1.54±1.15, P=0.42), but NLR after recovery were significantly high (1.58±1.71 vs. 1.31±0.76, P=0.21). However, patients with chronic ITP showed a trend of high NLR at the time of relapse. CONCLUSION: Chronic ITP patients showed higher NLR at the time of diagnosis compared with the patients with single episode. Also, the NLR at the time of relapse showed higher level compared to those at diagnosis. Relationship between NLR and chronic ITP need to be further studied.

Incidence trends and associated factors of diabetes mellitus in Korean children and adolescents: a retrospective cohort study in Busan and Gyeongnam

PURPOSE: This study investigated the incidence trends and associated factors of type 1 (T1DM) and type 2 diabetes mellitus (T2DM) in children and adolescents under 15 years of age in Busan and Gyeongnam, Korea from 2001 to 2010. METHODS: Medical records of newly diagnosed diabetes patients (n=328; 160 males, 168 females) were collected in questionnaire form from 5 tertiary and 42 general hospitals in Busan and Gyeongnam. RESULTS: The average crude incidence rate of T1DM and T2DM was 2.01/100,000 (95% confidence interval [CI], 1.76-2.28) and 0.75/100,000 (95% CI, 0.60-0.92), respectively. The incidence rate ratio (IRR) of T1DM was 1.31 (95% CI, 1.01-1.69), and that of T2DM was 1.97 (95% CI, 1.25-3.11) in the latter half-decade (2006 to 2010) compared to the early half-decade (2001 to 2005). There were gradually increasing incidence trends in both T1DM and T2DM over the 10-year period (P for trend: T1DM, 0.0009; T2DM, <0.0001). Age-specific IRR was highest in the 10- to 14-year-old group, regardless of diabetes type. In particular, a rapid increase in incidence of T2DM occurred in the 10- to 14-year-old group. IRR for females was 1.07 (95% CI, 0.83-1.38) for T1DM and 1.56 (95% CI, 1.01-2.41) for T2DM. IRR for Busan (urban) was 1.41 (95% CI, 1.09-1.83) for T1DM and 1.49 (95% CI, 0.96-2.30) for T2DM. CONCLUSION: T1DM and T2DM incidence both increased over time in youth under age 15 living in Busan and Gyeongnam; in particular, the incidence of T2DM in adolescents increased more rapidly.