Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and... |
Purpose : Acute interstitial pneumonia (AIP) is a rare disease, but its prognosis is fatal because of lack of efficient treatment modality. Recently, it has been reported that there was epidemic AIP in Korea. This study aims to investigate the past and current status of AIP in Korea. Methods : We performed a nationwide survey and a prospective study. From August... |
Purpose : This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. Methods : The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival... |
Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary... |
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... |
Purpose : The purpose of this study is to make a diagnostic classification and discuss a diagnostic strategy of floppy infants by investigating clinical, neurological, electrophysiological, and genetic analysis of infants admitted to intensive care units with the complaint of hypotonia. Methods : A retrospective study was performed from Jan. 1993 to Dec. 2005 in neonatal and pediatric intensive care units... |
Mechanical ventilation in children has some differences compared to in neonates or in adults. The indication of mechanical ventilation can be classified into two groups, hypercapnic respiratory failure and hypoxemic respiratory failure. The strategies of mechanical ventilation should be different in these two groups. In hypercapnic respiratory failure, volume target ventilation with constant flow is favorable and pressure target ventilation... |
Purpose : Nephrocalcinosis in very low birth weight(VLBW) infants were known to be caused by a longer duration of furosemide use. However, etiologies, pathogenesis and risk factors remain unclear. Therefore, we examined the incidence and risk factors of nephrocalcinosis in VLBW infants retrospectively. Methods : Inborn babies of birth weights less than 1,500 gm were examined retrospectively. Data were reviewed on... |
Congenital methemoglobinemia is caused by NADH-methemoglobin reductase deficiency in more than half of the total reported cases. NADH-methemoglobin reductase deficiency is an uncommon hereditary disorder producing methemoglobinemia and cyanosis in the homozygous subject. A majority of the patients born with these abnormalities have only a cosmetic defect-asymptomatic cyanosis. Congenital methemoglobinemia due to NADH-methemoglobin reductase deficiency is an autosomal recessive disorder... |
Purpose : Surfactant replacement therapy significantly decreases neonatal complications and mortality in neonates with respiratory distress syndrome(RDS), but clinical responses to the treatment is not consistent. An analysis of the perinatal factors influencing the clinical response to the therapy is important for early detection of high risk and prognosis. The purpose of this study is to analyze the clinical... |
Purpose : Patent ductus arteriosus(PDA) of prematurity is very important disease to study because it causes many cases of perinatal morbidity and its incidence is now increasing. Nowadays indomethacin is the drug of choice for PDA closure, but its use has been limited due to its side effects. Therefore, we compared the effect and side effects of indomethacin according to... |
Purpose : Patent ductus arteriosus(PDA) of prematurity is very important disease to study because it causes many cases of perinatal morbidity and its incidence is now increasing. Nowadays indomethacin is the drug of choice for PDA closure, but its use has been limited due to its side effects. Therefore, we compared the effect and side effects of indomethacin according to the infusion method, continuous... |
Purpose : Most neonatal abdominal masses are benign in nature, but early detection and management is important to avoid the development of complications. So, we studied the causes of neonatal abdominal masses and the efficacy of ultrasonography for early detection and diagnosis. Methods : We reviewed 36 newborn infants with abdominal masses who had been admitted to the nursery and Neonatal... |
Purpose : Retinopathy of prematurity(ROP) continues to be a serious problem in infants who survived with neonatal intensive care. Nevertheless, there is neither available data about the incidence of ROP which may be the basis of the epidemiology, nor analysis of the risk factors of ROP in Korea. The purpose of this retrospective study was to evaluate the incidence of... |