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        윌름스종양

        박준은 대한소아혈액종양학회 2015 Clinical Pediatric Hematology-Oncology Vol.22 No.1

        Wilms' tumor, nephroblastoma is the most common renal tumor in children. Treatment outcomes have improved dramatically over the past few decades due to applying of multimodality therapy consisting of surgery, radiotherapy and combination chemotherapy. There are two representative protocols produced by SIOP (Internationl society of Paediatric Oncology) and COG (Children Oncology Group) which is previously NWTS (Nationlal Wilms' tumor study) group in treatment of Wilms' tumor. The current protocol used by the COG undergoes upfront surgery prior to initiation treatment of chemotherapy. But, the protocol used by the International Society of Paediatric Oncology (SIOP) performs staging after preoperative chemotherapy and then surgery and postoperative chemotherapy. Current protocols becomes conducting to incorporate biologic features to stratify patients for therapy. Treatment strategies focuses on minimzing late effects of treatment while producing an excellent survival. This review presents a summary of these advances and outline the current molecular markers, pahtoloic and radiologic diagnostic method and treatment of Wilms' tumor.

      • KCI등재

        A Case of Pediatric Acute Lymphoblastic Leukemia with Trisomy 5 as a Sole Chromosomal Anomaly: A Prognostic Significance

        조영환,전인상 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.2

        We present a case of Korean pediatric patient with pre-B cell type acute lymphoblastic leukemia (ALL) with trisomy 5 as a sole cytogenetic anomaly. Here, we compare and describe the present case with previous pediatric case reports and provide a review of the literature. This case report may help elucidate the poor prognostic impact of trisomy 5 as a sole cytogenetic anomaly in pediatric patients with ALL. Additional studies are needed to confirm this hypothesis.

      • KCI등재

        A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine)

        조민형,정현주,한재호,박준은 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.2

        Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.

      • KCI등재

        Two Cases of Cytomegalovirus Infection Developed in Pediatric Acute Lymphoblastic Leukemia Patients

        정나영,김동현,진희승,김순기 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.2

        A 14 year-old boy with acute lymphoblastic leukemia (ALL) on maintenance chemotherapy presented with vision-threatening cytomegalovirus (CMV) retinitis. Treatment with intavitreal ganciclovir injection (2 mg/0.1 mL) followed by oral ganciclovir resulted in successful resolution of CMV retinitis. Another 13 year-old boy with ALL on maintenance chemotherapy presented with prolonged fever with no response to antibiotics administration. CMV and real-time PCR revealed positive result and a titer of 2,618,700 copies/mL, respectively. Ganciclovir was used for more than the approved duration of treatment, but viral titer frequently recurred with elevated liver enzymes and fever. In these 2 cases of CMV infection, a high index of suspicion and prompt management is important in children receiving ALL chemotherapy.

      • KCI등재

        Embryonal Carcinoma of the Pineal Gland Developed in an Adolescent Boy with Klinefelter Syndrome

        최효진,김은아,이재민,장경미,최준혁 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.2

        Klinefelter syndrome (KS) is characterized by small testes, gynecomastia, tall stature, and hypergonadotropic hypogonadism. This condition is associated with extra X chromosomes. It is well known that these aneuploidies predispose individuals to the development of several cancers. Moreover, there are many case reports that show KS patients to have a higher relative risk for the development of malignancy. However, incracranial germ cell tumor (ICGCT) associated with KS is very uncommon. Herein, we report delayed diagnosis of KS in a 15-year-old boy with ICGCT, embryonal carcinoma of the pineal gland, after multimodality treatment in Korea.

      • KCI등재

        Various Complications after a Vascular Procedure in Patients with Hemophilia

        하연수,박영실 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.1

        Hemophilia, an inherited bleeding disorder, is caused by a deficiency of coagulation factor VIII or IX. Most of patients with hemophilia need vascular procedure, which can lead to complications. Even though these complications can also occur in normal people, hemophilia and coagulopathy are particular risk factors. We reviewed medical records of patients with hemophilia who underwent vascular procedures and investigated its complications. Vessel-related complications occurred in five patients. Three patients had pseudoaneurysms after radial arterial puncture. All patients underwent coagulation factor replacement or ultrasound-guided compression and showed improvement. Neuropathy developed in one patient due to a hematoma that occurred after blood sampling. The hematoma improved, but motor and sensory deficits remained and neuropathy was confirmed. One patient died of uncontrolled bleeding after angiography. Vascular procedures require more attention in patients with hemophilia. Caution and prevention of complications is essential, even before the patient is diagnosed with hemophilia.

      • KCI등재

        A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region

        김현주,심예지,이재민,임영탁,양유진,박경미,최희원,박은실,김효선,박지경,최은진,공섬김,김지윤,박상규 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.1

        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%). 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.

      • KCI등재

        Ewing Sarcoma

        주희영 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.1

        Ewing sarcoma is the second most frequently occurring malignant tumor of the bone and soft tissue in adolescents and young adults. Genetically, Ewing sarcoma is characterized by balanced chromosomal translocation in which a member of FET gene family is fused with an ETS transcription factor, with the most common fusion being EWSR1-FLI1 (85% of cases). Treatment of Ewing sarcoma is based on multidisciplinary approach (local surgery, radiotherapy and multiagent chemotherapy), which are associated with chronic late effects that may compromise quality of life of survivors. First line treatment includes combination of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin. The beneficial role of high dose chemotherapy has been suggested in high-risk localized Ewing sarcoma patients, and the studies are being performed to investigate the role in metastatic disease. The 5-year overall survival for localized Ewing sarcoma has improved to reach 65% to 75%. But patients with metastatic disease have a 5-year survival rate of <30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrent tumor have a dismal prognosis. Novel therapeutic strategies based on understanding of molecular mechanisms are needed to improve the outcome of Ewing sarcoma and to lessen the treatment-related late effects.

      • KCI등재

        What Should We Consider Carefully When Performing Survival Analysis?

        곽상규,최은진 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.1

        The survival data and the survival analysis are the data and analysis methods used to study the probability of survival. The survival data consist of a period from the juncture of a start event to the juncture of the end event (occurrence event). The period is called the survival period or survival time. In this way, the method of analysing the survival time of subjects and appropriately summarizing the degree of survival is called survival analysis. To understand and analyse survival analysis methods, researchers must be aware of some concepts. Concepts to be aware of in the survival analysis include events, censored data, survival period, survival function, survival curve and so on. This review focuses on the terms and concepts used in the survival analysis. It will also cover the types of survival data that should be collected and prepared when using actual survival analysis method and how to prepare them.

      • KCI등재

        Primary Cardiac Hemangioendothelioma in an Infant: A Case Report

        서정욱,송미경,박성혜,박혜은,박신애 대한소아혈액종양학회 2019 Clinical Pediatric Hematology-Oncology Vol.26 No.1

        Primary cardiac tumors are rare, with a prevalence of 0.001-0.2%. Among such tumors, cardiac hemangioendotheliomas are some of the most uncommon. In Korea, there have been no reports of hemangioendothelioma occurring in the heart of infants. We herein report a case of an infant that was admitted to our medical center and presented with cough and a runny nose. The initial diagnosis was acute bronchiolitis. Cardiomegaly was observed on chest radiography. Echocardiography revealed a tumor measuring 3.5×4.0 cm in the right atrium. The infant was transferred to a tertiary medical center for tumor excision. The excised lesion was 3.8×3×3.2 cm in size, and biopsy confirmed a diagnosis of hemangioendothelioma. In this case report, we describe our experience with a rare case involving cardiac tumor in an infant with an upper respiratory tract infection.

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