• Title/Summary/Keyword: 혈액 질환

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The relationship between catechol-O-methyltransferase gene polymorphism and coronary artery abnormality in Kawasaki disease (가와사키병의 관상동맥 이상과 catechol-O-methyltransferase 유전자의 단일염기다형성)

  • Lee, Hyo Jin;Lee, Myung Sook;Kim, Ji Sook;Kim, Eun Ryoung;Kang, Sung Wook;Kim, Soo Kang;Chung, Joo Ho;Yoon, Kyung Lim;Han, Mi Young;Cha, Seong Ho
    • Clinical and Experimental Pediatrics
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    • v.52 no.1
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    • pp.87-92
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    • 2009
  • Purpose : Many gene polymorphisms are associated with coronary artery abnormalities in Kawasaki disease. Catechol-O- methyltransferase (COMT) plays an important role in the metabolism of catecholamines, catechol estrogen, and catechol drugs. Polymorphisms of the COMT gene are reported to be associated with myocardial infarction and coronary artery abnormalities. The aim of this study was to evaluate the relationship between COMT gene polymorphisms and coronary artery abnormalities in Kawasaki disease patients. Methods : One hundred and one Korean children with Kawasaki disease and 306 healthy Korean control subjects were enrolled in this study. The polymorphisms of the COMT gene were analyzed by direct sequencing. Results : There were no differences in the genotype and allelic frequency of the rs4680 and rs769224 polymorphic sites between Kawasaki disease and control subjects. Further, no significant difference was found in the rs4680 polymorphism between patients with coronary artery abnormalities and patients without coronary artery abnormalities (codominant P=0.32, dominant P=0.74, recessive P=0.13). However, the distribution of the rs769224 polymorphism was significantly different between patients with coronary artery abnormalities and patients without coronary artery abnormalities (codominant P= 0.0077, dominant P=0.0021, recessive P=0.16). Conclusion : Our results indicate that the polymorphisms of the rs769224 gene might be related to the development of coronary artery abnormalities in Kawasaki disease.

Antithrombin-III as an early prognostic factor in children with acute lung injury (급성 폐손상 소아 환자에서 조기 예후 인자로서의 antithrombin-III)

  • Lee, Young Seung;Kim, Seonguk;Kang, Eun Kyeong;Park, June Dong
    • Clinical and Experimental Pediatrics
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    • v.50 no.5
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    • pp.443-448
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    • 2007
  • Purpose : To evaluate the potential prognostic value of the antithrombin-III (AT-III) level in the children with acute lung injury (ALI), we analyzed several early predictive factors of death including AT-III level at the onset of ALI and compared the relative risk of them for mortality. Methods : Over a 18-month period, a total of 198 children were admitted to our pediatric intensive care unit and 21 mechanically ventilated patients met ALI criteria, as defined by American-European consensus conference, i.e., bilateral pulmonary infiltrates and $PaO_2/FiO_2$ lower than 300 without left atrial hypertension. Demographic variables, hemodynamic and respiratory parameters, underlying diseases, as well as Pediatric Risk of Mortality-III (PRISM-III) scores and Lung Injury Score (LIS) at admission were collected. AT-III levels were measured within 3 hours after admission. These variables were compared between survivors and non-survivors and entered into a multiple logistic regression analysis to evaluate their independent prognostic roles. Results : The overall mortality rate was 38.1% (8/21). Non-survivors showed lower age, lower lung compliance, higher PEEP, higher oxygenation index (OI), lower arterial pH, lower $PaO_2/FiO_2$, higher PRISM-III score and LIS, and lower AT-III level. PRISM-III score, LIS, OI and decreased AT-III level (less than 70%) were independently associated with a risk of death and the odds ratio of decreased AT-III level for mortality is 2.75 (95% confidence interval; 1.28-4.12) Conclusion : These results suggest that the decreased level of AT-III is an important prognostic factor in children with ALI and the replacement of AT-III may be considered as an early therapeutic trial.

Clinical Features of Henoch-Schönlein Purpura Gastroenteropathy without Purpura before Diagnosis (진단 전에 자반이 동반되지 않았던 Henoch-Schönlein 자반 위장병증의 임상적 고찰)

  • Oh, Jae Min;Park, Jae Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.54-60
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    • 2004
  • Purpose: Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a small-vessel vasculitic disease that most often affects the skin. Abdominal symptoms precede the typical purpuric rash of HSP in 14~36%. It is a challenge to diagnose HSP in the absence of a rash, because there are no biologic tests that can identify HSP with certainty, so we tried to find out the characteristic features of HSP gastroenteropathy without purpura before diagnosis. Methods: This study included 82 children with HSP who had been admitted or visited outward of the Department of Pediatrics, Pusan National University Hospital from 1995 to 2000. The cases that the onset of purpura preceded or coincided that of abdominal pain were defined as purpura-positive group. The cases that the onset of abdominal pain preceded purpura more than 1 week and purpura was not presented till diagnosed as HSP gastroenteropathy were defined as purpura-negative group. We compared and analyzed the clinical features of the two groups by reviewing the medical records retrospectively. To ensure the diagnosis of HSP gastroenteropathy, we conducted upper GI series, abdominal ultrasonogram, abdominal CT, endoscopy and/or skin biopsy. Results: The number of cases of purpura-positive group and purpura-negative group were 72 and 10, respectively. There is no difference between two groups in the incidence of clinical symptoms and laboratory findings. Children with HSP gastroenteropathy had characteristic erosive or ulcerative lesions in the stomach or duodenum on esophagogastroduodenoscopy, or mural thickening of the small bowel on abdominal ultrasonogram, CT or upper GI series. Skin biopsy revealed leukocytoclastic vasculitis in 3 of them, although biopsy specimen was taken from any areas of normal- appearing skin. In purpura-negative group, 9 patients improved by steroid therapy. Conclusion: In purpura-negative group, there is no diagnostic feature on the laboratory findings and clinical features. Therefore, to diagnose HSP gastroenteropathy in patients with abdominal pain in the absence of the characteristic rash, careful observation of clinical features and laboratory data, and prompt application of available diagnostic tools such as gastrointestinal endoscopy, radiologic study and skin biopsy are recommended. Early use of corticosteroid may reduce the suffering in these patients.

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Lower Extremity Edema in Terminal Cancer Patients (말기 암 환자에서의 하지 부종)

  • Shim, Byoung-Yong;Hong, Seok-In;Park, Ji-Chan;Hong, Sug-Hui;Choi, Gang-Heun;Cho, Hong-Joo;Kim, Seon-Young;Han, Sun-Ae;Lee, Ok-Kyung;Kim, Hoon-Kyo
    • Journal of Hospice and Palliative Care
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    • v.8 no.2
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    • pp.152-155
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    • 2005
  • Purpose: The lower extremity edema (LEE) is a common distressful symptom in advanced cancer patients and is hard to manage. We analyze the characteristics of LEE in patients with advanced cancer to provide the basic information of causes and adequate management. Methods: Physical examination, assessment of the location and severity of edema, blood chemistry (albumin, creatinine), Doppler Sono for patients with suspecting deep vein thrombosis (DVT), and abdomen CT scan for patient with suspecting lymph edema were performed. Severity of edema was classified according to NCI lymph edema scaling and improvement was defined as lowering at least 1 grade of edema after management. Results: Among 154 patient who had been admitted to Hospice Ward from Mar 2003 to Jan 2004, 33 had LEE, and 6 had both upper extremity edema and LEE except generalized edema. Their underlying cancers were stomach (7), lung (6), biliary tract (5), liver (5), colorectal (5), pancreas (2), and others (9). There were 12 patient with grade I, 20 patients with grade II, and 7 patients with grade III edema. The causes were hypoalbuminemia (11), lymph edema (10), DVT (7), obstruction of inferior vena cava (IVC) or portal vein (6), and dependent edema (5). The common managements were including leg elevation and diuretics. Elastic stocking was applied for patients with DVT and leg massage and pneumatic compression was used for lymph edema. The 2/3 patients were improved after management. Conclusion: The incidence of LEE in terminal cancer pts was high (25.3%) and their causes were variable including lymph edema, DVT, hypoalbuminemia and dependent edema. Active noninvasive management according to causes could result in good palliation.

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Clinical Features of Dietary Protein Induced Proctocolitis (식이 단백 유발 직결장염의 임상적 고찰)

  • Im, Sun Ju;Kim, Seong Heon;Bae, Sang Nam;Park, Jae Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.157-163
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    • 2005
  • Purpose: Dietary protein induced proctocolitis (DPIPC) can be considered as a cause of rectal bleeding or blood streaked stool in otherwise healthy-looking infants in the first several months of life. Failure to appreciate this entity may lead to inappropriate diagnostic or therapeutic intervention. This study aimed to ascertain the clinical features, treatment and prognosis of DPIPC. Methods: We reviewed 13 infants retrospectively, presented with bloody stool in early infancy. They were diagnosed as DPIPC clinically in Pusan National University Hospital from May 2002 to June 2004. Results: Seven males and six females were included. The mean age at onset of bleeding was $96.8{\pm}58.8days$. The mean frequency of hematochezia was $2.6{\pm}2.5$ times a day. Duration from onset of symptom to diagnosis was $35.5{\pm}55.0days$ and duration from onset of symptom to resolution of bleeding was $58.7{\pm}67.0days$. Nine (69.2%) were exclusively breast-fed infants and two (15.4%) were formula-fed infants. All but one infant did not have family history of other allergic diseases. A dietary history of ingestion of cow's milk, nut or shellfish was present in three mothers. Peripheral eosinophil count was normal to slightly elevated (total WBC count $10,555{\pm}3,145/mm^3$, relative eosinophil count $6.3{\pm}3.0%$, absolute eosinophil count $659.0{\pm}532.2/mm^3$). Sigmoidoscopy revealed lymphonodular hyperplasia with surrounding hemorrhagic spots in the rectosigmoid colon in 6 infants. Histopathologic finding of colonic biopsies in 5 infants showed chronic inflammation with lymphoid follicular hyperplasia (5 infants), crypt abscess (3 infants), or mild infiltration of eosinophils (less than 20/high power field) in the lamina propria. Spontaneous resolution of rectal bleeding occurred in all infants without dietary change or medicine. Conclusion: Most infants with DPIPC experience a very benign course and have spontaneous resolution of rectal bleeding without changes in the mother's diet. In the case of strong evidence for DPIPC we suggest deferring further invasive investigation and continuing breast feeding.

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Hemophagocytic Syndrome Presenting as Severe Acute Hepatitis (중증 급성 간염으로 발현한 혈구탐식증후군에 관한 연구)

  • Ryu, Jeong Min;Chang, Soo Hee;Kim, Joon Sung;Lee, Joo Hoon;Lee, Mi Jeong;Park, Kie Young;Kim, Kyung Mo;Seo, Jong Jin;Moon, Hyung Nam;Ghim, Thad;Chi, Hyun Sook
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.213-221
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    • 2005
  • Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

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Adenovirus Pneumonia with Respiratory Failure in Pediatric Patients (소아에서 발생한 호흡부전이 동반된 아데노 바이러스 폐렴)

  • Do, Sung Suk;Ma, Sang Hyeok;Park, Jae Sun;Lee, Young Ho;Lee, Hwan Jong;Lee, Gyu Man
    • Pediatric Infection and Vaccine
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    • v.5 no.2
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    • pp.258-266
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    • 1998
  • Purpose : Cases of adenoviral penumonia with rapidly progressive clinical course were experienced. We reviewed these patients in viewpoint of clinical manifestation and adenoviral serotypes. Methods : Culture and indirect immunofluorescence for respiratory viruses including respiratory syncytial virus, influenza virus, parainfluenza virus, adenovirus was done with nasopharyngeal aspirates from patients who admitted due to respiratory infections in Fatima Hospital, Masan from Nov. 1996 to Jul. 1997. Cultured adenovirus was serotyped by both neutralization and hemagglutination inhibition test. Medical records were reviewed for 5 patients with respiratory failure from adenovirus was isolated and serotyped. Results : The total number of examined patients was 460 patients. We isolated respiratory viruses in 143(30.9%) patients. Adenovirus was isolated from 66 out of 143(46.2%) patients. During Jan 1997 to May 1997, five patients with ages of 18 days to 11 months who were infected by adenovirus and had high fever with dyspnea and required assisted mechanical ventilation. One patients discharged against doctor's advice then died. Two of four patients had complications of disseminated intravascular coagulation; two had bronchiolitis obliterans. Two isolates were serotype 7, and one was serotype 5, and two were untyped. Conclusion : Severe pneumonia caused by serotype 7 continued to occur in 1997 following the epidemic in 1996, and severe pneumonia may also be caused by serotype 5 and other serotypes.

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A Comparison of Clinical Findings According to the Duration of Pyuria in Infants with Urinary Tract Infections (영아 요로 감염에서 농뇨의 지속기간에 따른 임상적 비교)

  • Lee, Jeong-Eun;Lee, Seung-Woo;Park, So-Hyun;Kim, Jong-Hyun;Koh, Dae Kyun
    • Pediatric Infection and Vaccine
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    • v.17 no.1
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    • pp.23-29
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    • 2010
  • Purpose : Urinary tract infection (UTI) in children is the most common disease during the infantile period, therefore early diagnosis and treatment are important. Pyuria is a useful clinical parameter for the initial diagnosis of a UTI. In this study we aimed to compare the clinical, laboratory, and imaging findings in relation to the duration of pyuria in infants with UTIs. Methods : Three hundred seventy-four infants <12 months of age who were admitted between January 1995 and December 2005 for the first episode of a febrile UTI were retrospectively reviewed. Patients were divided into two groups according to the duration of pyuria as follows: group 1, pyuria resolved <3 days after initial treatment; and group 2, pyuria lasted at least 3 days after initial treatment. Results : There were no significant differences between the two groups in relation to gender, age, total duration of fever, and organisms in the urine. Group 2 had a significantly higher peripheral blood leukocyte count ($14,360.86{\pm}5,526.16cells/mm^3$ vs. $11,822.55{\pm}5,687.26cells/mm^3$, P <0.001), erythrocyte sedimentation rate ($32.81{\pm}19.34mm/hr$ vs. $23.74{\pm}20.43mm/hr$, P <0.001), and C-reactive protein ($6.84{\pm}5.68mg/dL$ vs. $3.78{\pm}3.99mg/dL$, P <0.001) than group 1. There was a significantly higher incidence of hydronephrosis and a higher grade of vesicoureteral reflux (VUR) in group 2 compared to group 1. Conclusion : In infants with UTI, pyuria of longer duration is related to severe UTI and higher grade VUR, therefore aggressive radiologic studies may be necessary.

Quality and Affecting Factor of Care for Patients Hospitalized with Pneumonia (폐렴 입원환자 진료과정의 질적 수준과 이에 영향을 미치는 요인: 임상질지표를 중심으로)

  • Moon, Sangjun;Lee, Jin-Seok;Kim, Yoon;You, Sun-Ju;Choi, Yun-Kyoung;Suh, Soo Kyung;Kim, Yong-Ik
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.4
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    • pp.300-308
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    • 2009
  • Background: The quality of care for patients with community acquired pneumonia needs to be improved; the factors affecting this care need to be analyzed. The objectives of this study were used to measure the performance of care processes of for patients with pneumonia and to determine those patient and hospital characteristics are associated with quality care. Methods: The analysis was performed using data from 21 hospitals that had over 500 beds for 1,001 patients, who were sampled randomly. All patients were born before 31 December 1989, and discharged between the two months' August 2006 and October 2006. Performance process indicators were measured by respective hospital, and multivariate logistic regression was used to calculate associations between patients and hospital characteristics using 4 process indicators. Results: Performance rates in timely assessment of oxygenation assessments and blood cultures, correct administration of antibiotic medications, and blood culture performed prior to initial antibiotics were 69.4%, 79.1%, 82.5% and 60.5%, respectively. Age had a positive affect on oxygenation assessment within 24 hours. Bed number, number of nurses per bed, annual number of emergency department visits, average percentage of beds filled, location and arrival time, and site were factors associated with process indicators. Conclusion: It is necessary to make up for the weak points in the process of care for patients with community acquired pneumonia, by enforcing quality assurance. To reduce performance rate variation among hospitals, improvement in care protocols is required for hospitals that have poor quality of care levels.

Database study for clinical guidelines of children with pneumonia who visited an emergency department (응급의료센터에 내원한 소아 폐렴의 진료 지침을 위한 기초 자료 연구)

  • Hong, Dae Young;Lee, Kyung Mi;Kim, Ji Hye;Kim, Jun Sig;Han, Seung Baik;Lim, Dae-Hyun;Son, Byoung Kwan;Lee, Hun Jae;Lee, Kyung-Hee
    • Clinical and Experimental Pediatrics
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    • v.49 no.7
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    • pp.757-762
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    • 2006
  • Purpose : Pneumonia is one of the most common infections in children who visit emergency departments(ED), but standard clinical guidelines for children with pneumonia in Korea have not been studied. This study was performed to collect and evaluate a data-base of children with pneumonia for establishing clinical guidelines in ED. Methods : This study reviewed 304 children who were diagnosed and treated for pneumonia in the ED at one tertiary hospital between January 2003 and December 2003 retrospectively by reviewing the charts and analyzing the clinical characteristics, laboratory findings, and radiologic findings between an admission group and a discharge group. Results : The 2 year-5 year age group was the top of age distribution and the peak incidence of monthly distribution was December. Two hundred forty seven(81.3 percent) children were hospitalized(admission group), and the mean length of hospitalization was $7.24{\pm}3.24$ days. The most common indications of admission were fever, tachypnea and an age of less than three months. There was statistical differences in the outpatient department follow-up between the two groups(85.8 percent in admission group vs 35.1 percent in discharge group). Conclusion : More prospective studies are needed to establish clinical standard guidelines for children with pneumonia. This will be helpful in ED management and will aid the prevention of pneumonia.