Erratum: Correction of Affiliations in the Article "Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study".

This corrects the article on p. e393 in vol. 35, PMID: 33258329.

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, high-risk, 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.

Therapeutic Efficacy and Safety of Prolonged Macrolide, Corticosteroid, Doxycycline, and Levofloxacin against Macrolide-Unresponsive Mycoplasma pneumoniae Pneumonia in Children.

BACKGROUND: We aimed to compare the therapeutic efficacy of prolonged macrolide (PMC), corticosteroids (CST), doxycycline (DXC), and levofloxacin (LFX) against macrolide-unresponsive Mycoplasma pneumoniae (MP) pneumonia in children and to evaluate the safety of the secondary treatment agents. METHODS: We retrospectively analyzed the data of patients with MP pneumonia hospitalized between January 2015 and April 2017. Macrolide-unresponsiveness was clinically defined with a persistent fever of ≥ 38.0°C at ≥ 72 hours after macrolide treatment. The cases were divided into four groups: PMC, CST, DXC, and LFX. We compared the time to defervescence (TTD) after secondary treatment and the TTD after initial macrolide treatment in each group with adjustment using propensity score-matching analysis. RESULTS: Among 1,165 cases of MP pneumonia, 190 (16.3%) were unresponsive to macrolides. The proportion of patients who achieved defervescence within 48 hours in CST, DXC, and LFX groups were 96.9% (31/33), 85.7% (12/14), and 83.3% (5/6), respectively. The TTD after initial macrolide treatment did not differ between PMC and CST groups (5.1 vs. 4.2 days, P = 0.085), PMC and DXC groups (4.9 vs. 5.7 days, P = 0.453), and PMC and LFX groups (4.4 vs. 5.0 days, P = 0.283). No side effects were observed in the CST, DXC, and LFX groups. CONCLUSION: The change to secondary treatment did not show better efficacy compared to PMC in children with macrolide-unresponsive MP pneumonia. Further studies are needed to guide appropriate treatment in children with MP pneumonia.

Pediatric intracerebral histiocytic sarcoma with rhabdoid features: Case report and literature review.

A 16-year-old boy presented with marked weight loss, weakness of the left extremities and dizziness of 2 months duration and vomiting for 2 days. Brain MRI showed an approximately 6.5 × 5.3 cm-sized huge heterogeneous enhancing mass located in the corpus callosum, extending into the lateral ventricle. Open biopsy showed that the lesion consisted of lymphoplasmacytes and plump histiocytes with rhabdoid morphology, which were stained with S-100 protein, CD68 (KP1) and negative for CD1a. Histiocytic tumor was initially diagnosed. Chemotherapy using methotrexate, 6-mercaptopurine, vinblastine, interferon-alpha and dexamethasone was performed. After 5 months, partial removal was done. Microscopically, plump and bizarre tumor cells as well as rhabdoid features were found. Occasional spindle cells and necrosis were also found. These cells were positive for CD163, CD68, lysozyme, CD4, INI-1 and BRG1. BRAF V600E mutation was detected. The lesion was finally diagnosed as histiocytic sarcoma. Radiotherapy (6000 cGy in 30 fractions) was done. Both cerebral and extracerebral histiocytic sarcomas have long been diagnosed by unclarified criteria; its rarity as well as previously unclarified criteria can easily lead to a misinterpretation. Histiocytic sarcoma of the CNS is exceptionally rare in children, associated with an exceptionally poor prognosis. To date, only seven cases of pediatric cerebral histiocytic sarcomas have been reported. The present case is the first pediatric case showing BRAF V600E-mutated intracerebral histiocytic sarcoma.

Different clinical phenotypes in familial severe congenital neutropenia cases with same mutation of the ELANE gene.

Severe congenital neutropenia (SCN) is a heterogeneous group of disorders with a defect in granulopoiesis causing marked neutropenia and severe bacterial infections. A 17-month-old girl (patient 1) was admitted due to cervical lymphadenitis caused by methicillin-resistant Staphylococcus aureus, with neutropenia. She had Pseudomonas aeruginosa sepsis and peritonitis with perforated appendicitis at 8-month of age. Her sister, a 37-month-old girl (patient 2), had recurrent stomatitis with profound neutropenia, and her mother, a 32-yr-old woman (patient 3), had had recurrent stomatitis until her early 20s with neutropenia. We found an ELANE gene mutation (c.597+1G > A) from them in direct DNA sequencing analysis. Patients 1 and 2 did not respond to granulocyte colony stimulating factor and patient 1 was treated with prolonged antibiotics and excision. We demonstrated inherited SCN cases showing different severity even with the same mutation of the ELANE gene in a family.

Respiratory viruses in neonates hospitalized with acute lower respiratory tract infections.

BACKGROUND: The burden of respiratory syncytial virus (RSV) in neonates has not been clearly studied. The aims of this study were to determine the overall distribution of respiratory viruses in neonates hospitalized with acute lower respiratory tract infectiosns (ALRI) and to describe the clinical characteristics of RSV infections in these neonates. METHODS: From January 2009 through May 2010, neonates aged <1 month who were hospitalized with ALRI and did not have underlying disease were included in the study. Viruses were identified on multiplex reverse transcription polymerase chain reaction using nasal swab samples. Clinical variables were evaluated between the RSV and non-RSV infection groups. RESULTS: Of the 108 infants included in the study, 46 (42.6%) had RSV; human rhinovirus (18.5%), human parainfluenza virus 3 (7.5%), and human metapneumovirus (3.7%) were the next most common infections. Codetections accounted for 8.3% of the cases. Crowding increased the risk of RSV infection compared to the non-RSV group (OR, 16.5; P = 0.001). The RSV group had a greater incidence of dyspnea (P = 0.027), pneumonia (P < 0.001), requirement for oxygen (P < 0.001), and prolonged hospitalization (P = 0.011) than the non-RSV group. CONCLUSIONS: RSV was the most common viral etiology in neonates without underlying diseases who were hospitalized with ALRI. The disease severity of RSV infection was worse than that of other detected viral infections. Strict prevention strategies should be considered in overcrowded situations.

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data.

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.

Atypical hydroa vacciniforme-like epstein-barr virus associated T/NK-cell lymphoproliferative disorder.

Epstein-Barr virus (EBV)-associated T-cell/natural killer (NK)-cell lymphoproliferative disorders (EBV-T/NK-LPDs) accompany severe chronic active EBV infection (CAEBV) or comprise the CAEBV disease entity. The CAEBV disease entity has the common feature of lymphoproliferation of T or NK cells (primarily), and B cells (rarely), with chronic activation of EBV infection. The disease is rare and seems to be more prevalent in East Asian countries. The CAEBV disease entity encompasses heterogenous disorders, including hydroa vacciniforme (HV), hypersensitivity to mosquito bites, EBV-associated hemophagocytic syndrome, NK/T-cell lymphoma, and NK-cell leukemia. Atypical HV-like eruptions are present on sun-exposed and nonexposed areas with facial edema, fever, and hepatosplenomegaly, unlike classic HV. Recently, it has been suggested that classic HV and atypical HV-like eruptions are variants within the same disease spectrum of EBV-T/NK-LPD. We report a Korean boy with an atypical HV-like eruption and various systemic manifestations, including fever, sore throat, abdominal pain, headaches, seizures, and hematologic abnormalities for 2 years. After the initial mild eruption, which resembled a viral exanthem, ulceronecrotic skin lesions gradually developed and were associated with a high-grade fever and constitutional symptoms. He had a CAEBV infection, which showed a predominant proliferation of NK cells with high EBV DNA levels in the peripheral blood. However, in the skin lesions, there were nonneoplastic CD4 T-cell infiltrations predominantly showing a monoclonal T-cell receptor-γ gene rearrangement and positive EBV in situ hybridization.

Norovirus infections in asymptomatic food handlers in elementary schools without norovirus outbreaks in some regions of Incheon, Korea.

Norovirus (NV) has caused large outbreaks of gastroenteritis in schools. Studies of NV epidemiology in schools related to NV outbreaks have been frequently reported. However, reports of that in schools without outbreaks are not found. Presently, NV molecular epidemiology surveillance was carried out in asymptomatic food handlers working at nonoutbreak elementary schools in Incheon, Korea, in March, April and December, 2009. NV prevalence was examined by real-time reverse transcription-PCR (RT-PCR) and the positive products were re-evaluated by conventional RT-PCR for sequencing. Fecal samples (n = 776) were collected from 776 food handlers in 60 schools. NV was detected in 26 of them (3.4%). Of these, 17 (65%) were positive for NV GII and 10 (38%) were positive for NV GI. Of the 26 samples, 19 were positive by conventional RT-PCR. Sequencing of these 19 strains revealed GII/4 (n = 5), GI/6 (n = 3), GI/14 (n = 2), GII/8 (n = 2), GI/2 (n = 2), GI/10 (n = 1), GII/1 (n = 1), GII/3 (n = 1), GII/7 (n = 1), and GII/16 (n = 1). In this survey, the food handler population unrelated to NV outbreaks was found to normally contain asymptomatic carriers of NV. The excretion of NV from asymptomatic food handlers should be an infection source of NV outbreaks.

Effectiveness and Safety of Clofarabine Monotherapy or Combination Treatment in Relapsed/Refractory Childhood Acute Lymphoblastic Leukemia: A Pragmatic, Non-interventional Study in Korea.

PURPOSE: Effectiveness and safety of clofarabine (one of the treatment mainstays in pediatric patients with relapsed/refractory acute lymphoblastic leukemia [ALL]) was assessed in Korean pediatric patients with ALL to facilitate conditional coverage with evidence development. MATERIALS AND METHODS: In this multicenter, prospective, observational study, patients receiving clofarabine as mono/combination therapy were followed up every 4-6 weeks for 6 months or until hematopoietic stem cell transplantation (HSCT). Response rates, survival outcomes, and adverse events were assessed. RESULTS: Sixty patients (2-26 years old; 65% B-cell ALL, received prior ≥ 2 regimen, 68.3% refractory to previous regimen) were enrolled and treated with at least one dose of clofarabine; of whom 26 (43.3%) completed 6 months of follow-up after the last dose of clofarabine. Fifty-eight patients (96.7%) received clofarabine combination therapy. Overall remission rate (complete remission [CR] or CR without platelet recovery [CRp]) was 45.0% (27/60; 95% confidence interval [CI], 32.4 to 57.6) and the overall response rate (CR, CRp, or partial remission [PR]) was 46.7% (28/60; 95% CI, 34.0 to 59.3), with 11 (18.3%), 16 (26.7%), and one (1.7%) patients achieving CR, CRp, and PR, respectively. The median time to remission was 5.1 weeks (95% CI, 4.7 to 6.1). Median duration of remission was 16.6 weeks (range, 2.0 to 167.6 weeks). Sixteen patients (26.7%) proceeded to HSCT. There were 24 deaths; 14 due to treatment-emergent adverse events. CONCLUSION: Remission with clofarabine was observed in approximately half of the study patients who had overall expected safety profile; however, there was no favorable long-term survival outcome in this study.

Chemotherapy and interferon-alpha treatment of Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis of an unknown origin. The prognosis of ECD is variable, and it mainly depends on the involved anatomic sites. The treatment modalities have not been standardized. Interferon-alpha (IFN) has been reported to be effective in the management of ECD. We report here on an uncommon case with ECD in a 17-year-old female who had multiple lesions in the whole body and she was treated with chemotherapy and IFN. She has remained disease-free for 2 years after the completion of treatment.

A case of posterior reversible encephalopathy syndrome in a child with myelodysplastic syndrome following allogenic bone marrow transplantation.

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but distinctive clinical-radiologic entity characterized by headache, seizures, visual disturbance, and altered mental function associated with reversible white matter edema affecting the posterior parietal and occipital lobes of the brain. Although PRES is caused by a variety of conditions, acute elevation of blood pressure, fluid retention, and treatment with immunosuppressive drugs and/or anti-neoplastic agents are the main causes. A few cases of PRES associated with hematopoietic stem cell transplantation (HSCT) in children have been reported. Early recognition of PRES and appropriate management are needed to reduce the risk of permanent neurologic disability. The authors report a case of PRES in a girl who received an HLA-identical sibling bone marrow transplantation for myelodysplastic syndrome to emphasize the importance of early recognition and institution of appropriate management of PRES during HSCT.

Poor adherence and reasons for nonadherence to the asthma guidelines among pediatricians in Korea.

BACKGROUND: Information is limited regarding adherence to the asthma guidelines in Asia, especially for children. OBJECTIVE: The aim of the study was to estimate adherence to the asthma guidelines, and investigate reasons for nonadherence. METHODS: We conducted a cross-sectional questionnaire survey on the primary care pediatricians practicing in Incheon, Korea. RESULTS: Surveys were returned by 81 of 131 eligible primary care pediatricians for a response rate of 61.8%. Almost all respondents (98.8%) had heard of the asthma guidelines, and most (93.8%) had read or received education about them. The classification of asthma severity was well understood with the accuracy of 85.7%. Correct responses for treatments were 51.3% for intermittent, 68.5% for mild persistent and 56.9% for moderate persistent asthma. For severe persistent asthma, the accuracy was high (85.7%). Only 21.5% of the respondents reported routine use of office peak flow measurement for patients with moderate or severe persistent asthma for diagnosis, and fewer (10.3%) used spirometry. A written action plan was reported to be given to only 5.2% of patients with asthma. The principal barrier to adherence was the presence of external barriers (lack of time, equipment, supporting staff etc.). Different barriers were prominent for different types of guideline components. CONCLUSIONS: There is poor adherence to the asthma guidelines in several aspects of their recommendations. Tailored interventions that address the current state of barriers need to be designed and implemented.

Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus.

Acute lymphoblastic leukemia (ALL), a primary hematologic malignancy that is especially common in childhood, occurs relatively rarely as a secondary malignant neoplasm. Available data indicate that ALL often follows chemoradiotherapy for soft tissue sarcoma. Perivascular epithelioid tumor (PEComa), a primitive mesenchymal tissue origin, can be classified as a soft tissue sarcoma. An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa. ALL, FAB L2, and immunophenotypically pro-B developed 16 months after the final chemotherapy treatment. Moreover, a cytogenetic study of lymphoblasts showed t(1;11)(p32;q23). Herein, the authors report a case of secondary ALL that might be related to a previously used intercalating DNA topoisomerase II inhibitor (anthracycline) for a very rare sarcoma, PEComa.

A single dose of anti-D immunoglobulin raises platelet count as efficiently as intravenous immunoglobulin in newly diagnosed immune thrombocytopenic purpura in Korean children.

OBJECTIVE: The aim of this study is to compare the efficacy and safety of a single dose of anti-D immunoglobulin (anti-D) at 50 mug/kg to intravenous immunoglobulin (IVIG) in Korean children with acute immune thrombocytopenic purpura (ITP). METHODS: We performed this study prospectively by randomly administering 2 consecutive doses of IVIG at a dose of 1.0 g/kg/dor a single dose of anti-D at 50 microg/kg to children upon initial diagnosis of acute ITP. The platelet count and adverse events, including hemoglobin concentration, were then serially evaluated, and the responses were compared. RESULTS: The likelihood of having a platelet count greater than 20x10/mm after 3 days of treatment in the IVIG and anti-D group was 93% and 92%, respectively. In addition, hemoglobin concentration in the anti-D group had declined more than that of the IVIG group (1.49 g/dL vs. 0.80 g/dL, P=0.014) 3 days after treatment. Fever, chills, and headache occurred less frequently in the anti-D group than the IVIG group, however, this difference was not statistically significant (25% vs. 45%, P=0.494). CONCLUSIONS: A single dose of 50 microg/kg of anti-D raised platelet count as efficiently as IVIG in newly diagnosed cases of ITP in Korean children. Although 50 microg/kg of anti-D had a greater effect on the hemoglobin concentration than IVIG, the adverse effects were found to be acceptable, and no serious events were observed.

A case of myeloid sarcoma presenting with an orbital mass, hearing loss, and multiple cranial neuropathies.

Lim SH, Nam HN, Lim KI, Jeon IS. A case of myeloid sarcoma presenting with an orbital mass, hearing loss, and multiple cranial neuropathies. Turk J Pediatr 2018; 60: 322-325. Primary myeloid sarcoma occurring in multiple sites; orbit, ear, brain, and spinal cord is a rare clinical entity. A 15-year-old male adolescent presented with bilateral orbital mass, hearing difficulty, and clinical signs of multiple cranial nerves palsy. Approximately 6 weeks later, acute myeloid leukemia was confirmed. This case alerts us that in patients with diverse sarcomatous lesions, acute myeloid leukemia presenting as myeloid sarcoma should be considered.

Characteristics of Upper Gastrointestinal Tract Involvement in Korean Pediatric Crohn's Disease: A Multicenter Study.

PURPOSE: Crohn's disease (CD) can involve any site of the gastrointestinal tract (GIT). However, the characteristics of upper GIT involvement in CD are unclear, especially in the Eastern pediatric population. This study aimed to estimate the prevalence of upper GIT involvement and identify the clinical features of Korean children with CD. METHODS: This was a retrospective multicenter cohort study that included 52 pediatric patients with CD who underwent esophagogastroduodenoscopy and biopsy. The clinical symptoms and endoscopic and histologic features of the upper GIT were identified according to the presence or absence of upper gastrointestinal symptoms. RESULTS: Among the 52 patients, upper GIT involvement was noted in 50.0% (26/52). The mean age at CD diagnosis was 14.1±2.1 years. Gastric ulcer was the most common lesion (19.2%) found on upper GIT endoscopy, followed by duodenal ulcers (15.4%). Chronic inflammation was the most common histopathologic feature (75.0%), followed by gastric erosion (17.3%). Granuloma was found in 9.6% of patients. Helicobacter pylori infection was identified in 5.8% of patients. Endoscopic and histologic findings were not significantly different, but the mean values of erythrocyte sedimentation rate (60.7±27.1 vs. 43.0±27.6 mm/h, p=0.037) and C-reactive protein (16.5±28.2 vs. 6.62±13.4 mg/dL, p=0.014) were significantly different between patients with and without upper gastrointestinal CD symptoms. CONCLUSION: Upper GIT involvement was relatively common in pediatric patients with CD irrespective of upper gastrointestinal symptoms, and H. pylori infection was relatively uncommon. The results of this study should aid the establishment of regional guidelines for upper GIT examination.

Early onset of colorectal cancer in a 13-year-old girl with Lynch syndrome.

Lynch syndrome is the most common inherited colon cancer syndrome. Patients with Lynch syndrome develop a range of cancers including colorectal cancer (CRC) and carry a mutation on one of the mismatched repair (MMR) genes. Although CRC usually occurs after the fourth decade in patients with Lynch syndrome harboring a heterozygous MMR gene mutation, it can occur in children with Lynch syndrome who have a compound heterozygous or homozygous MMR gene mutation. We report a case of CRC in a 13-year-old patient with Lynch syndrome and congenital heart disease. This patient had a heterozygous mutation in MLH1 (an MMR gene), but no compound MMR gene defects, and a K-RAS somatic mutation in the cancer cells.

Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children.

BACKGROUND AND OBJECTIVES: In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase. SUBJECTS AND METHODS: A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups-IVIG responders vs. non-responders-based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD. RESULTS: Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%). CONCLUSION: Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.