• Genomics & Informatics
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• v.18 no.1
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• pp.6.1-6.9
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• 2020

Acute leukemia represents the most common pediatric malignancy comprising diverse subtypes with varying prognosis and treatment outcomes. New and targeted treatment options are warranted for this disease. Patient-derived xenograft (PDX) models are increasingly being used for preclinical testing of novel treatment modalities. A novel approach involving targeted error-corrected RNA sequencing using ArcherDX HemeV2 kit was employed to compare 25 primary pediatric acute leukemia samples and their corresponding PDX samples. A comparison of the primary samples and PDX samples revealed a high concordance between single nucleotide variants and gene fusions whereas other complex structural variants were not as consistent. The presence of gene fusions representing the major driver mutations at similar allelic frequencies in PDX samples compared to primary samples and over multiple passages confirms the utility of PDX models for preclinical drug testing. Characterization and tracking of these novel cryptic fusions and exonal variants in PDX models is critical in assessing response to potential new therapies.

• Clinical and Experimental Pediatrics
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• v.54 no.4
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• pp.169-175
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• 2011

Purpose: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies. Methods: We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, T-cell prolymphocytic leukemia, Langerhans cell histiocytosis, and rhabdomyosarcoma), who had undergone selective arterial embolization (SAE) of the internal iliac artery at the Chonnam National University Hwasun Hospital between January 2004 and December 2009. SAE was performed by an interventional radiologist using Gelfoam$^{(R)}$ and/or Tornado$^{(R)}$ coils. Results: The patients were 5 boys and 1 girl with median age of 6.9 years (range, 0.7-14.8 years) at the time of SAE. SAE was performed once in 4 patients and twice in 2, and the procedure was unilateral in 2 and bilateral in 4. The causes of hemorrhage were as follows: hemorrhagic cystitis (HC) in 3 patients, procedure-related internal iliac artery injuries in 2 patients, and tumor rupture in 1 patient. Initial attempt at conservative management was unsuccessful. Of the 6 patients, 5 (83.3%) showed improvement after SAE without complications. Conclusion: SAE may be a safe and effective procedure for controlling acute intractable hemorrhage in pediatric malignancy patients. This procedure may obviate the need for surgery, which carries an attendant risk of morbidity and mortality in cancer patients with critical conditions.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.59-63
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• 2001

The authors, over the last 6 months, have treated 2 patients with perforated typhlitis complicating acute lymphocytic leukemia (ALL) with good outcome. The first patient was a 13-year-old male who developed intermittent high fever, abdominal pain, abdominal distention and diarrhea during the course of maintenance chemotherapy. The peripheral leukocyte ranged from 230-470/$mm^3$. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal and ascending colonic walls and subsequent ragged perforation of the posterior wall of the cecum. He survived after treatment by right hemicolectomy and aggressive supportive measures. The patient case was a 3 year-old female who developed intermittent high fever, right lower abdominal pain, a mass, and watery diarrhea during the course of maintenance chemotherapy. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal wall (6-15mm in thickness) and subsequent small perforation of the posterior wall of the cecum with thick-walled localized abscess. She has recovered completely after aggressive medical management. We learned two lessons from our experience treating these patients:1) early diagnosis provided by a high index of suspicion and the use of ultra sonogram or CT scan is essential. And 2) although perforation is one of the surgical indications for the treatment of typhlitis, it is possible to manage the perforation nonoperatively in selected cases with localized abscess.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.11
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• pp.4609-4614
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• 2015

Background: In recent decades, the prognosis for childhood leukemia has improved, especially for acute lymphoblastic leukemia (ALL). In Thailand, though, the survival rate for ALL is unimpressive. In 2006, standard national protocols for childhood leukemia treatment were implemented. We herein report the outcome of the ALL national protocols and explanations behind discrepancies in outcomes between institutions. Materials and Methods: Between March 2006 and February 2008, 486 children with ALL from 12 institutions were enrolled in the Thai national protocols. There were 3 different protocols based on specific criteria: one each for standard risk, high risk and Burkitt's ALL. We classified participating centers into 4 groups of institutions, namely: medical schools in Bangkok, provincial medical schools, hospitals in Bangkok and provincial hospitals. We also evaluated supportive care, laboratory facilities in participating centers, socioeconomics, and patient compliance. Overall and event-free survival were determined for each group using the Kaplan Meier method. Statistical differences were determined using the log-rank test. Previous outcomes of Thai childhood ALL treatment between 2003 and 2005 served as the historic control. Results: Five-year overall survival of ALL treated using the Thai national protocol was 67.2%; an improvement from the 63.7% of the 12-institute historical control (p-value=0.06). There were discrepancies in event-free survival of ALL between centers in Bangkok and up-country provinces (69.9% vs 51.2%, p-value <0.01). Socioeconomics and patient compliance were key elements in determining the outcome (65.5% vs 47.5%, 59.4% vs 42.9%) (p-value < 0.02). Conclusions: Implementation of standard national protocols for childhood leukemia in Thailand did not significantly improve the outcome of ALL. Factors leading to better outcomes included (a) improvement of treatment compliance (b) prevention of treatment abandonment and (c) financial support to the family.

• Pediatric Infection and Vaccine
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• v.27 no.2
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• pp.134-139
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• 2020

Staphylococcus epidermidis is a part of the normal skin flora of humans. However, it can cause serious infections in people exposed to foreign bodies or in immunocompromised patients. A 13-year-old boy was hospitalized with fever and myalgia. Painful nodular lesions were detected on the scalp, arms, and legs. Pancytopenia and blasts were present in the peripheral blood. He was diagnosed with acute myeloid leukemia. Magnetic resonance imaging of the whole body showed multiple peripheral rim-enhancing, cyst-like lesions. Ultrasonography showed echogenic nodules inside the cystic lesions in the intramuscular space of the arms and legs. Therefore, cysticercosis was strongly suggested initially. However, an abscess was confirmed on sono-guided biopsy and S. epidermidis was isolated from a microbial culture of the tissue. We report a case of multiple disseminated lesions caused by S. epidermidis in a leukemia patient, initially mistaken for cysticercosis.

• Journal of Korean Neurosurgical Society
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• v.50 no.3
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• pp.235-239
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• 2011

Radiation therapy has been widely applied for cancer treatment. Childhood acute lymphocytic leukemia (ALL), characterized by frequent central nervous system involvement, is a well documented disease for the effect of prophylactic cranio-spinal irradiation. Irradiation, however, acts as an oncogenic factor as a delayed effect and it is rare that glioblastoma multiforme develops during the remission period of ALL. We experienced a pediatric radiation-induced GBM patient which developed during the remission period of ALL, who were primarily treated with chemotherapeutic agents and brain radiation therapy for the prevention of central nervous system (CNS) relapse. Additionally, we reviewed the related literature regarding on the effects of brain irradiation in childhood and on the prognosis of radiation induced GBM.

• Clinical and Experimental Pediatrics
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• v.55 no.7
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• pp.249-253
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• 2012

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma- leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.

• Journal of radiological science and technology
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• v.38 no.1
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• pp.17-21
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• 2015

Total body irradiation(TBI) and chemotherapy are the pre-treatment method of a stem cell transplantations of the childhood leukemia. in this study, we evaluate the Quantitative human body dose prior to the treatment. The MCNPX simulation program evaluated by changing the material of the tissue compensators with imitation material of pediatric exposure in a virtual space. As a result, first, the average skin dose with the material of the tissue compensators of Plexiglass tissue compensators is 74.60 mGy/min, Al is 73.96 mGy/min, Cu is 72.26 mGy/min and Pb 67.90 mGy/min respectively. Second, regardless of the tissue compensators material that organ dose were thyroid, gentile, digestive system, brain, lungs, kidneys higher in order. Finally, the ideal distance between body compensator and the patient were 50 cm aparting each other. In conclusion, tissue compensators Al, Cu, Pb are able to replace of the currently used in Plexiglass materials.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.278-281
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• 2009

Bilateral renal enlargement is a very rare manifestation as the primary presenting feature of acute lymphoblastic leukemia. We are reporting an unusual clinical picture of a 9-month-old male patient diagnosed as precusor B-cell lymphoblastic leukemia, who showed bilateral nephromegaly without any hepatosplenomegaly at the time of initial presentation.

• Pediatric Infection and Vaccine
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• v.19 no.3
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• pp.162-167
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• 2012

Respiratory syncytial virus (RSV) is the major cause of lower respiratory tract infection in infants. Life-threatening RSV infection is often reported in young children and immunocompromised hosts. Since there is no report on ribavirin therapy for RSV pneumonia in pediatric cancer patients in Korea, we report one case of RSV pneumonia that developed in an infant with acute lymphoblastic leukemia (ALL). Despite administration of oral ribavirin and intravenous immunoglobulin, the patient's respiratory distress worsened and admission to an intensive care unit was necessary. Chest x-ray showed multifocal consolidation, pneumothorax, and pneumomediastinum. Treatment with aerosolized ribavirin led to significant clinical improvement. The role of aerosolized ribavirin is still controversial, but it might have a therapeutic potential for severe RSV pneumonia in children with leukemia.