• Pediatric Infection and Vaccine
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• v.27 no.3
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• pp.198-204
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• 2020

Cytomegalovirus (CMV) disease is rare in children who receive anticancer chemotherapy and have no history of stem cell transplantation (SCT). We report a case of CMV retinitis that developed during maintenance chemotherapy for acute leukemia. A 7-year-old boy developed decreased visual acuity and persistent pancytopenia during maintenance chemotherapy. Laboratory investigations initially showed significant CMV antigenemia (51 positive cells/200,000 leukocytes); however, antiviral therapy was not deemed necessary in this patient who had no history of SCT. CMV antigenemia worsened to 170 positive cells/200,000 leukocytes over 3 weeks. Ophthalmological examination revealed multiple bilateral retinal infiltrates and granular lesions. He was diagnosed with CMV retinitis and was treated with a 4-week course of intravenous ganciclovir and intravitreal injection of ganciclovir 6 times, followed by a 1-month course of orally administered valganciclovir. A CMV antigenemia assay showed negative results, and follow-up fundoscopy revealed lesser retinal infiltration after the sixth intravitreal ganciclovir injection. Future studies should focus on the development of standardized screening methods and preemptive therapeutic strategies for CMV disease in high-risk children.

• Laboratory Medicine Online
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• v.1 no.2
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• pp.110-114
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• 2011

There have been a few reports of hemophagocytic lymphohistiocytosis (HLH) with chromosomal abnormalities. Clonal chromosomal abnormalities in HLH patients are usually found in association with hematologic malignancies and rarely with epstein-barr virus (EBV) infection. Here, we report a fatal case of HLH with clonal karyotype abnormalities. A 75-yr-old man was admitted with persistent anorexia and high fever. Laboratory data revealed pancytopenia, hypofibrinogenemia, hyperferritinemia, prolonged prothrombin time and activated partial thromboplastin time, and marked elevated level of serum transaminases. In real time-PCR using whole blood, EBV DNA was not detected but cytomegalovirus (CMV) DNA was detected. The bone marrow aspiration smear showed hyperplasia of mature histiocytes with prominent hemophagocytosis. In chromosomal analysis of bone marrow aspirates, complex chromosomal abnormalities were found. In spite of steroid pulse therapy and antibiotic treatment, he died of disseminated intravascular coagulopathy.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Tuberculosis and Respiratory Diseases
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• v.43 no.1
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• pp.22-29
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• 1996

Background: Abnormalities of the peripheral blood are frequent and varied in patients with miliary tuberculosis. Anemia, leukopenia, thrombocytopenia, pancytopenia, monocytosis, basophilia, eosinophilia and leukemoid reactions have been reported. These abnormalities are more frequent in patients with positive bone marrow study. In this report, we evaluated clinical, hematological and immunological features in patients with miliary tuberculosis in order to know whether difference is existed between "bone marrow biopsy positive group(pathologically proven to miliary tuberculosis)" and "negative group". Method: Clinical evaluation, serum ADA, sIL-2R, and T-lymphocyte subsets were measured in 40 patients with miliary tuberculosis who received bone marrow biopsy. Results: 1) The average age of patients was 39 year-old. There were 23 male and 17 female patients. Associated extrapulmonary tuberculosis are 9 CNS tuberculosis, 6 joint tuberculosis, and 2 tuberculous pleurisy. 2) Sixteen of the 40 patients were positive bone marrow biopsy(60%). 3) Sixteen of the 40 patients(60%) had anemia(11 positive patients: 13 negative patients). Leukopenia occurred in 12 per cent(4:1). Thrombocytopenia was noted in 10%(3:1). 4) The mean value of serum ADA was 83 U/L(90 U/L: 70.6 U/L, p=0.23). 5) The mean activity of Soluble IL-2 receptor was 4,643 pmol/L($6840{\pm}7446\;pmol/L$: $1,897{\pm}1,663\;pmol/L$, p=0.06). 6) In the T lymphocyte subsets, the percent of T-lymphocytes was 64%(62%:73%, p=0.2). In some patients(9), $T_4$ and $T_8$ ratio in BAL fluid($1.97{\pm}1.2$) was higher than that in the peripheral blood($1.16{\pm}0.5$). Conclusion: Bone marrow examination are diagnostic in 60% of cases of miliary tuberculosis. Percents of the total T lymphocyte and helper T cell in BAL are more elevated than in peripheral blood. There was no significant difference in peripheral blood abnormalities and marker of T lymphocyte activation between the bone marrow biopsy positive and negative group.

• Pediatric Infection and Vaccine
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• v.10 no.2
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• pp.200-207
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• 2003

Purpose : Malaria is known to have been eradicated for a few decades through persistent national health program in South Korea. However, malaria caused by Plasmodium vivax has started to reappear incidiously among military personnel near to DMZ since 1993. After then, the number of malarial cases have been increased abruptly year by year. We analyzed the children of indigenous malaria who were diagnosed by peripheral blood smear and malarial antibody test with regards to epidemiologic and clinical manifestations. Methods : The study 13 cases below 15 years of age, who were confirmed as vivax malaria during from January 2000 to August 2003. We retrospectively analyzed epidemiologic data, clinical manifestations, laboratory findings and therapeutic responses. Results : All of 13 cases were indigenous and tested positive for Plasmodium vivax. Of 13 patients, 9 were male and 4 were female. Mean age of onset was $9.5{\pm}3.6$ years old. Ilsan(n=9) was the most prevalent area, the most patients(n=11) were presented in summer (from June to August). A characteristic feature of periodic 3 day fever in patients with P.vivax infection was reported in only 2 among 13 cases. Thrombocytopenia was most prominent findings, which was accompanied by 12 of 13 patients and pancytopenia was appeared in 3 patients on this study. The therapeutic responses of hydroxycholoquine were very good in all cases, and abnormal laboratory findings were recovered and no relapse during follow-up period. Conclusion : Vivax malaria is indigenous in Korea near to DMZ, but recently endemic area seemed to be extended southward. Plasmodium vivax is the cause of indigenous malaria of children. As for children with high fever accompanying thrombocytopenia in endemic area of Korea, malaria must be included in differential diagnosis whether the type of fewer is periodic 3 day fever or not. Malaria antibody test is helpful as a screening test for malaria.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.545-558
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• 2003

Adult respiratory distress syndrome (ARDS) is of particular interest because of its severity of the associated lung injury and its high mortality. However, the pathophysiologies of ARDS in infant and childhood groups are still not well clarified inspite of many previous investigations. To investigate the time course of pathophysiology of ARDS in infant and childhood groups, this study was designed with experimental endotoxin-induced ARDS model using young rabbits (8 week-old). Material and Method: Rabbits were divided into the control group (n=8) and the endotoxin-treated group (n=32). The endotoxin group was subdivided into 4 groups by the sampling times as 3, 6, 12 and 24 hr-groups (G- $E_{3,6,12,24,}$ each n=8). The experimental ARDS was made by a bolus injection of endotoxin (Escherichia coli serotype 055 : B5, 0.50 mg/kg) via rabbit ear vein. For evaluation of the hematologic and inflammatory markers, and superoxide dismutase (SOD) concentrations, the blood samples were taken from the heart. The bronchoalveolar lavage fluid (BALF) were obtained for analysis of the leukocytes and protein concentration. With biopsy of the lung, histopathologic changes of the lung were also evaluated. Result: In the endotoxin groups, significant leukopenia (owing to pancytopenia) occurred in 3 and 6-hr groups, which was followed by significant leukocytosis (owing to neutrophilia) in the 12 and 24-hr groups (p<0.05). Serum levels of tumor necrosis factor-$\alpha$ (TNF-$\alpha$) and interleukin-1 $\beta$ (IL-1 $\beta$) in the endotoxin groups were higher than those of control group (p<0.05). Serum levels of superoxide dismutase (SOD) of G- $E_{3}$ and G- $E_{6}$ were higher than those of control group, whereas those of G- $E_{12}$ were lower than those of control groups (p<0.05). Total leukocyte counts and protein con-centrations in BALF were significantly elevated in the endotoxin groups compared to the control group (p < 0.05). The hemorrhagic pattern of BALF showed occurred in the endotoxin groups. The endotoxin groups (in G- $E_{6}$) had severe infiltration of inflammatory cells (lymphocyte and monocyte) in the pulmonary interstitium and parenchyma, migrations of neutrophil and eosinophil into alveolar spaces and interstitial widening, which are the evidences of acute lung injury. In the endotoxin groups, there were significant positive correlations between the BALF findings and the immunologic markers (TNF-$\alpha$, IL-1$\beta$, SOD) (p<0.05). Conclusion: Severe acute lung injury occurred in all the endotoxin-treated rabbits. The pathophysiologic findings were so progressive until 6-hr by time dependant pattern, and then recovered slowly, Variable hematologic, immuno-logic, and pathologic factors were well correlated in the development and progression of endoxin-induced lung injury. The pathophysiologic responses were sensitive and rapid in young rabbit Young rabbit seemed to be a useful experimental animal model for infant and childhood groups.roups.