We report a case of drug eruption and liver damage due to diaminodiphenyl suIfone(DDS) ingestion in a 17-year-old female. This patient had taken DDS for 20 days, 100mg-200mg daily to treat an unknown skin disease. Thereafter, she had generalized erythematous eruption on whole body, icteric sclera and fever. Liver function test showed abnormality (SGOT 514 unit, SGPT 710 unit, alkarine phosphotase 4. 3 B.U., total biIirubin 7. Oml/dl, direct bilirubin 4. 8mg/dl). The adverse reactiions to DDS are gastrointestinal intolerance, hemolytic, anemia, methemoglobinemia, agranulocytoais, hepatitis, neuritis, psychosis and a skin rash described as a fixed drug eruption, erythema multiforme, exfoliative dermatitis and toxic epidermal necrolysis. In Korea, DDS has been used for the treatment of skin diseases of various types for a long time without prescription, especially in rural areas. This trend is a significant sociomediical problem in Korea.
Adolescent ; Alanine Transaminase ; Anemia ; Bilirubin ; Dermatitis, Exfoliative ; Drug Eruptions* ; Eating ; Erythema Multiforme ; Exanthema ; Female ; Fever ; Hepatitis ; Humans ; Korea ; Liver Function Tests ; Liver* ; Methemoglobinemia ; Neuritis ; Prescriptions ; Psychotic Disorders ; Sclera ; Skin Diseases ; Stevens-Johnson Syndrome

No abstract available.

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.
Abdomen ; Adult ; Catheters ; Child ; Diagnosis ; Epidemiology ; Health Personnel ; Homocysteine ; Hospitals, University ; Humans ; Incidence ; Korea* ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Medical Records ; Pediatrics ; Prognosis ; Protein C ; Protein S ; Retrospective Studies ; Risk Factors ; Thrombosis ; Upper Extremity ; Venous Thromboembolism*

BACKGROUND: Clinical trials that utilize imaging findings as surrogate endpoints are considered to be cost-effective. However, unlike numeric data, magnetic resonance imaging (MRI) findings are not quantifiable. Thus, we have begun to develop a software package that is able to convert qualitative MRI findings into quantifiable data. METHODS: Computer software (DUIH_Image) was created with which every patient's MRI data can be registered on a standard brain template. Interuser and intrauser reliabilities for the registration were measured, and then a proof-of-principle experiment was conducted to determine whether the system could identify factors that were associated with a greater National Institutes of Health Stroke Scale (NIHSS) score at admission. We studied 40 consecutive patients [65.1+/-14.2 years old (mean+/-SD); 22 males and 18 females] with first-ever acute lacunar infarction of the corona radiata, who were divided into two groups according to their NIHSS score (i.e., low: 0-2; high: > or =3). The following parameters were compared between these two groups: (1) data retrieved from clinical profiles, including demographic and risk factor variables; and (2) accumulated diffusion MRI lesions mapped on a standard template. RESULTS: Modest levels of interuser and intrauser reliability were observed (p<0.05, R(2)=0.63-0.84, Pearson correlations). Regarding the clinical profiles, no significant difference was found for the numeric data sets or infarct size between the two groups. However, on the accumulated lesion map image, the lesion area that overlapped the most was located more posterolaterally in the high NIHSS score group than in the low NIHSS score group. CONCLUSIONS: In this pilot study we have demonstrated the potential usefulness of the DUIH_Image software. We plan to update this software to enable its utilization in actual clinical trials.
Biomarkers ; Brain ; Computer Systems ; Diffusion Magnetic Resonance Imaging ; Humans ; Magnetic Resonance Imaging ; Male ; National Institutes of Health (U.S.) ; Pilot Projects ; Risk Factors ; Software ; Stroke ; Stroke, Lacunar

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.
Adult ; Child ; Diagnosis ; Hemophilia A ; Hemorrhage ; Hospitals, University ; Humans ; Korea ; Medical Records ; Prevalence ; Retrospective Studies ; von Willebrand Diseases

Inflammatory bowel disease (IBD) is an idiopathic chronic inflammation of the intestines. IBD treatment may require anti-inflammatory agents such as sulfasalazine or 5-aminosalicylate (5-ASA) and immunomodulators to control the symptoms. However, these agents have a variety of common adverse effects such as nausea, vomiting, skin rash, leukopenia, thrombocytopenia, and infections. Moreover, rare side effects such as nephrotic syndrome, pneumonitis, and pericarditis can occur. A 21-year-old male was admitted to the hospital due to acute chest pain, fever, and sweating. The patient had a history of Crohn's disease and had been taking mesalazine for 3 weeks. Chest x-ray, echocardiography, and clinical manifestations revealed that the patient had acute pericarditis. However, we did not recognize the relationship between these findings and 5-ASA at that time. Two years later, the patient took 5-ASA again, and similar symptoms occurred, which led us to confirm that he suffered from pericarditis induced by this drug. We report a case of acute recurrent pericarditis that developed after taking 5-ASA for IBD treatment with a review of the literature.
Anti-Inflammatory Agents ; Chest Pain ; Colitis, Ulcerative ; Crohn Disease ; Echocardiography ; Exanthema ; Fever ; Humans ; Immunologic Factors ; Inflammation ; Inflammatory Bowel Diseases ; Intestines ; Leukopenia ; Male ; Mesalamine ; Nausea ; Nephrotic Syndrome ; Pericarditis ; Pneumonia ; Sulfasalazine ; Sweat ; Sweating ; Thorax ; Thrombocytopenia ; Vomiting ; Young Adult

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

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.

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.