1.Clinical Course of Neutropenia in Previously Healthy Children.
Do Hee KIM ; Jae Hee LEE ; Hoi Soo YOON
Clinical Pediatric Hematology-Oncology 2018;25(2):87-96
BACKGROUND: Neutropenia can be easily found in previously healthy children associated with various medical conditions, and the clinical course ranges from transient benign to life threatening. This study aimed to investigate the etiology, clinical characteristics, and clinical courses of neutropenia in previously healthy children. METHODS: We evaluated 215 previously healthy children under aged 18 years who diagnosed with neutropenia in two hospitals. Clinical and laboratory features were analyzed retrospectively based on the medical records. RESULTS: Transient infectious neutropenia (TIN) accounted for 97.7% of cases and chronic neutropenia (CN), for 2.3%. An infectious agent was identified in 128/210 (61%) patients with TIN, and the most frequent agents were viruses (46.5%). The most common viral agent was respiratory syncytial virus (RSV) (29%). TIN subgroups exhibited no differences in severity according to infectious agent (virus, bacteria, Mycoplasma); however, neutropenia severity differed among viral agents [mild-to-moderate neutropenia in the RSV group (857.3±293.3/µL) and moderate-to-severe neutropenia in the parainfluenza group (567.3±198.1/µL); P=0.017]. All patients with CN had anti-neutrophil antibody positivity (autoimmune neutropenia, AIN), and moderate-to-severe neutropenia predominated. The median duration of TIN was 8 days (range, 3–286 days), and it was significantly longer for AIN at 330 days (range, 217–730 days) (P=0.000). The median duration of neutropenia was also different according to each viral agent, with 4 days (range, 3–11 days) for the RSV group and longer durations for 3 other groups (influenza, parainfluenza, other respiratory viruses) (P=0.015). CONCLUSION: Neutropenia in previously healthy children is usually of transient infectious origin, with mild-to-moderate severity, and it resolves spontaneously without complications.
Autoimmune Diseases
;
Bacteria
;
Child*
;
Humans
;
Medical Records
;
Neutropenia*
;
Paramyxoviridae Infections
;
Respiratory Syncytial Viruses
;
Retrospective Studies
;
Tin
2.Predictive Value of Endometrial Thickness for Anemia in Adolescent Girls with Abnormal Uterine Bleeding.
Ji Sung LEE ; Young Hwan CHO ; In Sang JEON
Clinical Pediatric Hematology-Oncology 2018;25(2):81-86
BACKGROUND: Immaturity of the endocrine system that controls the normal menstrual cycle frequently results in abnormal uterine bleeding (AUB) and elicits anemia in adolescent girls. This study was conducted to assess the predictive value of endometrial thickness (ET) for anemia in adolescent girls with AUB. METHODS: A retrospective chart review was performed for a cohort of adolescents (12–18 years old) with AUB who presented over a 10-year period. Complete blood count and ultrasonographic data of 115 adolescent girls with AUB were analyzed. Subjects were classified according to ET as group I (ET < 11 mm) and group II (≥11 mm), and the incidence of anemia was compared. Subjects were also classified according to age as group Y (12–15 years old of age) and group O (16–18 yr), and ET, hemoglobin (Hb), and incidence of anemia were compared. RESULTS: The incidence of anemia in all subjects was 67.8% and was significantly higher in group II than in group I (P < 0.001). The incidence of severe anemia was 56.9% in group II, which was higher than in group I (P=0.039). The incidence of anemia was not significantly different between groups Y and O. However, the incidence of severe anemia was significantly higher in group Y than in group O (P=0.001). CONCLUSION: AUB can result in severe anemia in adolescent girls particularly those who are close to menarche or have a thick endometrium. Early supervision of AUB is required in order to avoid anemia in adolescent girls with AUB.
Adolescent*
;
Anemia*
;
Blood Cell Count
;
Cohort Studies
;
Endocrine System
;
Endometrium
;
Female*
;
Humans
;
Incidence
;
Menarche
;
Menstrual Cycle
;
Organization and Administration
;
Retrospective Studies
;
Uterine Hemorrhage*
3.Anemia Can Be Associated with Isovaleric Acidemia.
Jong Hyung YOON ; Hong Jin LEE
Clinical Pediatric Hematology-Oncology 2018;25(1):76-79
No abstract available.
Anemia*
4.A Case of Successfully Treated Severe Heart Failure due to Cyclophosphamide Induced Cardiomyopathy.
Jung Min PARK ; Seung Min HAHN ; Jung Woo HAN ; Chuhl Joo LYU
Clinical Pediatric Hematology-Oncology 2018;25(1):71-75
Cyclophosphamide-induced cardiotoxicity is an uncommon complication especially in patients who have never undergone mediastinal irradiation or cardiotoxic chemotherapy and do not have underlying cardiac diseases. Here, we describe the case of a 19-year-old female with chronic myeloid leukemia. She was previously treated with oral tyrosine kinase inhibitors and developed cardiomyopathy after receiving infusion of 60 mg/kg intravenous cyclophosphamide for two days with a conditioning regimen for allogenic hematopoietic stem cell transplantation. Severe thickening of the left ventricle and reduced ejection fraction without triggering agents were characteristic for cyclophosphamide-induced cardiomyopathy. Her NT-pro BNP and troponin T concentrations surged to >70,000 pg/mL (0=130 pg/mL) and 2,031 pg/mL (0-14 pg/mL), respectively, during the course of the therapy and multiple organ failure seemed imminent evidenced by unresponsive decline in blood pressure. However, with close monitoring and persistent conservative management which consisted of intravenous hydration, continuous hemodialysis, and mechanical ventilation, her condition recovered.
Blood Pressure
;
Cardiomyopathies*
;
Cardiotoxicity
;
Cyclophosphamide*
;
Drug Therapy
;
Female
;
Heart Diseases
;
Heart Failure*
;
Heart Ventricles
;
Heart*
;
Hematopoietic Stem Cell Transplantation
;
Humans
;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
;
Multiple Organ Failure
;
Protein-Tyrosine Kinases
;
Renal Dialysis
;
Respiration, Artificial
;
Troponin T
;
Young Adult
5.A Case of Erdheim-Chester Disease Developed during Treatment of Leukemia in a Child.
Joon Pyo HONG ; Won Ki AHN ; Joo Yeon LIM ; Jo Eun JUNG ; Seung Min HAHN ; Jung Woo HAN ; Chuhl Joo LYU
Clinical Pediatric Hematology-Oncology 2018;25(1):66-70
Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell histiocytosis disorder characterized by replacement of normal tissue by lipid-laden histiocytes affecting various organs. A few pediatric cases have been reported worldwide. Here we present a child with leukemia who was diagnosed as ECD. A 2-year and 11-month old boy diagnosed with high risk acute lymphoblastic leukemia (ALL) at the age of 17 months, received allogeneic hematopoietic stem cell transplantation (HSCT) at the age of 2 years old. Six months after the transplantation, the patient was admitted to the hospital with palpable left calf nodules. Bone marrow study suggested ECD without leukemia with complete chimerism status. Excisional biopsy of the left calf nodule showed ‘aggregation of non-Langerhan's cell type epitheloid histiocytes’; clinically suggestive of ECD. The patient was started on vinblastine and corticosteroid treatment.
Biopsy
;
Bone Marrow
;
Child*
;
Chimerism
;
Erdheim-Chester Disease*
;
Hematopoietic Stem Cell Transplantation
;
Histiocytes
;
Histiocytosis
;
Humans
;
Leukemia*
;
Male
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma
;
Vinblastine
6.Septic Arthritis and Infective Endocarditis in an Adolescent Hemophilia B Patient with an Inhibitor and a Central Venous Access Device.
Clinical Pediatric Hematology-Oncology 2018;25(1):61-65
Central venous access devices (CVAD) provide hemophilic patients, particularly children, with prolonged reliable venous access to promote routine factor replacement therapy. However, one of the significant complications of CVAD use is infection. We report the case of a severe hemophilia B patient with an inhibitor who developed septic arthritis and infective endocarditis associated with methicillin-resistant Staphylococcus aureus infection originating from a CVAD. Our patient had an underlying condition of congenital heart disease, one of the risk factors for infective endocarditis. Unfortunately, the antibiotic therapy did not have a significant effect. An echocardiogram revealed vegetation on the right ventricular moderate band and surgery was determined to be the best course of action. Septic arthritis and endocarditis rarely occur in hemophilia patients, however, they must be taken into account in hemophiliacs with continuing bacteremia.
Adolescent*
;
Arthritis, Infectious*
;
Bacteremia
;
Child
;
Endocarditis*
;
Heart Defects, Congenital
;
Heart Septal Defects, Ventricular
;
Hemophilia A*
;
Hemophilia B*
;
Humans
;
Methicillin-Resistant Staphylococcus aureus
;
Risk Factors
7.A Case of Jacobsen Syndrome Presenting with a Huge Cephalhematoma and Thrombocytopenia after Birth.
Juhee SHIN ; Gaeun KIM ; Rosie LEE ; Nani JUNG ; Ye Jee SHIM ; Jung Sook HA
Clinical Pediatric Hematology-Oncology 2018;25(1):56-60
Jacobsen syndrome (JS) is a contiguous gene syndrome resulting from a deletion of chromosome 11q, with various clinical manifestations. A post-term small for gestational age infant was born by normal vaginal delivery without trauma or vacuum extraction. On day 5, right parietotemporal scalp swelling developed, with petechiae on the right cheek and thrombocytopenia (platelets: 63,000/µL). A prominent forehead, wide-set eyes, short and upturned nose were noted. Karyotyping and microarray analysis demonstrated del(11)(q24q25), consistent with Jacobsen syndrome. Brain magnetic resonance imaging (MRI) revealed a huge cephalhematoma. The patient is scheduled to receive periodic evaluations for thrombocytopenia and heart, kidney, abdominal malformations, ophthalmologic and auditory problems. There are lots of newborns with cephalhematoma or petechiae after birth. Not all newborns with these symptoms need evaluations, but if they have these symptoms with suspect features or appearances, we need to go through further evaluations.
Brain
;
Cheek
;
Forehead
;
Gestational Age
;
Heart
;
Humans
;
Infant
;
Infant, Newborn
;
Jacobsen Distal 11q Deletion Syndrome*
;
Karyotyping
;
Kidney
;
Magnetic Resonance Imaging
;
Microarray Analysis
;
Nose
;
Parturition*
;
Purpura
;
Scalp
;
Thrombocytopenia*
;
Vacuum
8.Effects of Low- or Moderate-dose Whole Body-X-ray Radiation on the Immune System of C57BL/6 Mice.
Yunmi KO ; Yeon Ho JEONG ; Jun Ah LEE
Clinical Pediatric Hematology-Oncology 2018;25(1):50-55
PURPOSE: Increase in the use of diagnostic imaging or occupational exposure to radiation have brought upon concerns on the safety and biological effects of low- or moderate-dose radiation. However, limited information is available on the effects of low or moderate dose radiation on human health. METHODS: Using C57BL/6 mice, we aimed to evaluate the biological effects of low- and moderate-dose radiation on the immune system. X-rays was chosen as a radiation source and we analyzed complete blood counts, various lymphocyte subsets and various cytokine levels after single fraction x-ray exposure (0.1 Gy, 1 Gy). RESULTS: No significant changes in the immunologic parameter of C57BL/6 mice were observed after radiation, except LIX (a cytokine equivalent to human CXCL5), that showed higher level after 0.1 Gy radiation compared to the control. CONCLUSION: We observed that a single fraction of low or moderate dose of X-ray radiation does not cause significant changes in the immune system of C57BL/6 mice. Further studies are necessary to elucidate the mechanism underlying our results.
Animals
;
Blood Cell Count
;
Diagnostic Imaging
;
Humans
;
Immune System*
;
Lymphocyte Subsets
;
Mice*
;
Occupational Exposure
;
Radiation Dosage
9.Childhood Venous Thromboembolism in Yeungnam Region in Korea: Multicenter Study.
Soram LEE ; Jong Hyuk YOUN ; Jae Young LIM ; Hee Won CHUEH ; Jae Min LEE ; Jin Kyung SUH ; Ji Yoon KIM ; Eu Jeen YANG ; Kyung Mi PARK ; Young Tak LIM ; Jikyoung PARK ; Eun Mi CHOI ; Ye Jee SHIM ; Heung Sik KIM ; Sang Kyu PARK ; Seom Gim KONG ; Eun Jin CHOI ; Eun Sil PARK
Clinical Pediatric Hematology-Oncology 2018;25(1):43-49
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*
10.Educational Interventions to Enhance Adherence to Prophylactic Treatment in Korean Hemophilia Patients.
Ji Hyun NA ; Ki Young YOO ; Ji Yoon KIM ; Sang Kyu PARK ; Soon Ki KIM ; Eun Jin CHOI
Clinical Pediatric Hematology-Oncology 2018;25(1):38-42
BACKGROUND: A patient's adherence to prophylactic treatment is one of the most significant factors to achieve desired outcomes, in regards to the quality of life and treatment cost-effectiveness. The aim of this study is to evaluate the effectiveness of educational interventions in enhancing adherence to prophylactic treatment in Korean hemophilia patients. METHODS: The Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro) was used to measure adherence. The study design consisted of two groups. One group was not educated with the education card which presented the morning administration of clotting factor concentrates and self-infusion skill. The other group was educated with the card. The scores of each subscale in the two groups and scores focused on the ‘Timing’ subscale were compared. RESULTS: Participants were recruited from five hemophilia treatment centers in Korea with 95 eligible patients forming the uneducated group and 123 patients in the educated group. The mean total score was 40.4 and 39.9, respectively. The subscale mean scores of the uneducated group and educated group were 8.43 and 7.90 (Timing), 6.12 and 5.88 (Dosing), 6.43 and 6.33 (Planning), 5.99 and 6.35 (Remembering), 6.22 and 6.25 (Skipping), and 7.23 and 7.24 (Communicating), respectively. CONCLUSION: Results showed that education programs on prophylactic treatment for hemophilia patients need to be well-designed with precise and subject-appropriate contents. Although the mean total scores of VERITAS-Pro could not be improved, the appropriate timing of injection with the educational intervention was administered. Results suggest that this whole process can enhance Korean hemophilia patients' adherence to prophylactic treatment.
Education
;
Hemophilia A*
;
Humans
;
Korea
;
Quality of Life