• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.67-72
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• 2008

Purpose : In this study, we retrospectively analyzed the clinical outcomes of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) grafted from HLA-matched parents. Methods : Seven children with acute leukemia (4 acute lymphoblastic leukemia, 3 acute myeloid leukemia) in first complete remission received allogeneic HSCT from their respective parents at the St. Marys Hospital between April, 1999 and October, 2005. The median age of patients at transplantation was 5 years (range, 1-11 years; 2 male, 5 female) and the median age of donors was 35 years (range, 30-41 years; 5 male, 2 female). We investigated the clinical outcomes such as engraftment, acute and chronic graft-versus-host disease (GVHD), transplant-related morbidity and mortality, relapse and survival. Results : Median time from transplantation to last follow-up was 69.5 months (range, 18.8-96.5 months). All patients were successfully engrafted, with a median time of 11 days (range, 10-16 days) and 26 days (range, 13-39 days) for neutrophil and platelet recovery, respectively. Grade II acute GVHD occurred in 3, and grade III acute GVHD in 1 of 7 recipients. Extensive chronic GVHD developed in 2, and limited chronic GVHD in 1 of 7 recipients. Death from transplant-related complications occurred in 1, and relapse occurred in 1 of 7 recipients. Estimated 5-year overall survival was $83{\pm}15%$. Conclusion : The clinical outcomes of recipients who underwent HSCT from HLA-matched parents were comparable to those of patients who received HSCT grafted from HLA-matched sibling donors in childhood leukemia. HLA typing of parents, as well as siblings will increase the likelihood of finding an HLA-matched family donor for patients who need HSCT.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1158-1166
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• 2006

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 of Seoul National University Children's Hospital. Clinical features and all tests related to hypotonia were investigated. Results : There were 21 cases of floppy infants admitted to intensive care units. Final diagnosis was classified as centra (7 cases[33.3 percent]), peripheral (11 cases [52.4 percent]), and unspecified (3 cases [14.3 percent]). Among the central group, three patients were diagnosed as hypoxic ischemic encephalopathy, two patients as Prader-Willi syndrome, one patient as chromosomal disorder, and one patient as transient hypotonia. Among the peripheral group, four patients were diagnosed as myotubular myopathy, three patients as SMA type 1, two patients as congenital myotonic dystrophy, one patient as congenital muscular dystrophy, and one as unspecified motor-neuron disease. Motor power was above grade 3 on average, and deep tendon reflex was brisk in the central group. Among investigations, electromyography showed 66 percent sensitivity in the peripheral group, and muscle biopsy was all diagnostic in the peripheral group. Brain image was diagnostic in the central group, and Prader-Willi FISH or karyotyping was helpful in diagnosis in central group. Morbidity and mortality was more severe in the peripheral group Conclusion : Classification of diagnosis by clinical characteristics in this study, and application of investigations step by step, may provide an effective diagnostic strategy.

• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.112-120
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• 2004

Purpose : Methicillin Resistant-Coagulase Negative Staphylococcus(MR-CNS) infection has become an increasingly important cause of morbidity in NICU infants. We investigated the c linical characteristics of MR-CNS sepsis. Methods : This study included 40 neonates with MR-CNS sepsis who were admitted to the neonatal intensive care unit of Kangnam Sacred Heart Hospital, Hallym University from January 1998 to July 2002. MR-CNS sepsis was defined as MR-CNS recovery from blood with clinical symptoms and signs of infection. Retrospective analyses of the medical records of patients with MR-CNS sepsis were performed. The analyses included demographic findings, clinical features, hospital courses, risk factors for infection including invasive procedures and mortality. Results : From 1998 to 2002, there were 40 cases of MR-CNS sepsis, comprising 17.7% of late onset infections in NICU of Kangnam Sacred Heart Hospital. The male/female ratio was 1.5 : 1. The mean gestational age of infected babies was $32.4{\pm}4.3$ weeks at birth. And the first positive MR-CNS culture was done in the day $10.6{\pm}9.3$ after birth. Clinical symptoms such as fever, dyspnea, cyanosis, grunting, bradycardia, vomiting and diarrhea were frequent in MR-CNS. Mechanical ventilation was applied in 12 cases and catheter was inserted in 11 cases. The mortality(12.5%) directly attributable to MR-CNS sepsis was similar to other late onset infections. Conclusion : MR-CNS is a pathogen responsible for most late onset and nosocomial infections. And it will be life-threatening in high-risk neonate. Awareness of increasing infections due to MR-CNS in NICU is important not only for infection control but also placing a great limit in use of antibiotics and invasive procedures, especially in premature infants.

• Sleep Medicine and Psychophysiology
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• v.12 no.1
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• pp.58-63
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• 2005

Objectives: REM sleep behavior disorder (RBD), characterized by excessive motor activity during REM sleep, is associated with loss of muscle atonia. In recent years, it has been reported that RBD has high co-morbidity with CNS disorders (especially, Parkinson's disease, dementia, multiple system atrophy, etc.). We aimed to assess differences in clinical and polysomnographic findings among RBD patients, depending on the presence or absence of central nervous system (CNS) disorders. Methods: The medical records and polysomnographic data of 81 patients who had been diagnosed as having RBD were reviewed. The patients were classified into two groups: associated RBD (aRBD, i.e., with a clinical history and/or brain MRI evidence of CNS disorder) and idiopathic RBD (iRBD, i.e., without a clinical history and/or brain MRI evidence of CNS disorder) groups. Twenty-one patients (25.9%) belonged to the aRBD group and 60 patients (74.1%) belonged to the iRBD group. The clinical characteristics and polysomnographic findings of the two groups were compared. Results: Periodic limb movement disorder (PLMD), i.e., PLMI (periodic limb movement index)>5, was observed more frequently in the aRBD group than in the iRBD group (p<0.001, Fisher's exact test). Also, obstructive sleep apnea syndrome (OSAS), i.e., RDI (respiratory disturbance index)>5, was found more frequently in the aRBD group (p=0.0042, Fisher's exact test). The percentages for slow wave sleep and sleep efficiency were significantly lower in the aRBD group than in the iRBD group. Conclusion: We found that 1 out of 4 RBD patients had associated CNS disorders, warranting more careful neurological evaluation and follow-up in this category of RBD. In this category of RBD patients, we also found more frequent PLMD and OSAS. These patients were also found to have lower slow wave sleep and sleep efficiency. In summary, RBD patients with associated CNS disorders suffer from more disturbed sleep than those without them.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.474-480
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• 2008

Purpose : Stress hyperglycemia is common in critically ill adult patients. It is known as a predictor of increased mortality, and intensive insulin therapy has been shown to improve the prognosis in such patients. We have investigated the relationship between early stress hyperglycemia and clinical outcomes in preterm infants. Methods : In this study, 141 preterm infants with a gestational age of less than 30 weeks were enrolled. The hyperglycemic group was defined as that having maximum glucose of more than 150 mg/dL (n=61) during the first 48 h of life, and the non-hyperglycemic group was defined as that having maximum glucose of less than 150 mg/dL (n=80). Perinatal history, severity of illness using the Clinical Risk Index for Babies (CRIB) score, clinical outcomes, and mortality of the two groups were compared. Results : There was no significant difference in the gestational age between the two groups, but the birth weight (P<0.001) was significantly lower, and the CRIB score (P<0.001) was significantly higher in the hyperglycemic group. Disseminated intravascular coagulation (P<0.001) and clinically suspected sepsis (P=0.046) were more common in the hyperglycemic group. Mortality was markedly higher in the hyperglycemic group (11.3% vs. 41.0%, P<0.001). On performing a stepwise multiple logistic regression analysis, hyperglycemia (OR 3.787; 95% CI 1.324 to 10.829), the CRIB score (OR 1.252; 95% CI 1.047 to 1.496) and birth weight (OR 0.997; 95% CI 0.994 to 1.000) was independently associated with higher mortality. Conclusion : Stress hyperglycemia within the first 48 h of life is independently related to increased morbidity and mortality in preterm infants.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.532-539
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• 1993

Background: Physician's estimates of patient survival often influence clinical decisions, especially those near the end of life. In addition. clinical decisions frequently reflect trade-offs between morbidity and length of survival. As a result, accurate estimates of survival can be extremely useful in clinical decision. When the episode of acute respiratory failure in chronic obstructive lung disease, evaluation of the severity of the condition and short term prognosis is difficulit based on the available clinical or paraclinical data at the time of admission. Method: In this study, we performed a retrospective study in Chung Ang University Hospital, 74 patients (51 males, 23 females), who were hospitalized with chronic obstructive lung disease with acute respiratory failure from 1980 to 1992. We evaluated these patients to determine lung prognostic factors at time of admission in the Intensive Care Unit (ICU) that predict short term survival, and to determine the possible application of the Simplified Acute Phsiology Score (SAPS) to this population, All patients were treated with similar regimen during the hospitalization. Results: The results were as follows: 1) Hospital mortality was 34%(25/74 patients) and surival rate was 66%(49/74 patients) in COPD with acute respiratory failure. The prognosis of the older age was much poorer than those of the young age. 2) There was no difference in mortality according to the results of basal pulmonary test and arterial blood gas analysis. 3) The SAPS at admission was higher in those patients who expired(10.8) than the survived(6.5), and there was positive correlation between SAPS and mortality (r=0.91, p<0.05). 4) Prognostic factors in acute respiratory failure complicating COPD which were identifiable at time of admission to the ICU were as follows: cachexia, encephalopathy, serum creatinine and phosphate. Conclusion: In conclusion, the SAPS might have a good prognostic value for determination of short term survival among chronic obstructive lung disease with acute respiratory failure.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.495-502
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• 2010

Purpose : Sepsis is a significant cause of morbidity and mortality in the newborn, particularly in preterm. The objective of this study was to analyze the incidence rate, causative pathogens and clinical features of neonatal sepsis in one neonatal intensive care unit (NICU) for 6 years. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics including sex, gestational age, birth weight, Apgar score, length of hospitalization, length of total parenteral nutrition, presence of central venous catheter, underlying diseases, laboratory findings, microorganisms isolated from blood culture, complications and mortality in 175 patients between January 2003 and December 2008. Results : 1) Sepsis was present in 175 of 3,747 infants for 6 years. There were more gram-positive organisms. 2) The gram-negatives were more prevalent in preterm. There were no significant differences of other clinical features between two groups. 3) Underlying diseases were found in 73.7%, and the most common disease was cardiovascular disease. The most common organisms of gram-positives and gram-negatives were methicillin resistant Staphylococcus aureus (MRSA) and Serratia marcescens. 4) There was statistically significant difference on platelet counts between two groups (P<0.05). 5) Complications were found in 18.3% and septic shock was the most common. MRSA was the most common pathogen in sepsis with complication. 6) The mortality rate was 7.4%. 7) There were differences in monthly blood stream infection/1,000 patient-days. Conclusion : The studies about the factors that can influence neonatal sepsis will contribute to decrease the infection rates in NICUs.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.746-752
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• 2007

Purpose : Total hydroperoxide (TH), free radical-mediated oxidation product can be used as a measure of free radical injury. The aim of the present study was to see if preterm newborns are at increased risk for oxidative stress compared with term newborns, and to determine whether oxidative stress during postnatal first 1 week is associated with clinical outcomes in preterm infants. Methods : Serum TH levels of preterm infants (n=39) were compared with those of term infants (n=24) on the postnatal day 1. Among the preterm infants, serum TH levels of uncomplicated group (n=23) were also compared with those of complicated group (n=16) who developed oxygen radical related diseases on the postnatal day 1 and 7. Retrospective analysis was performed to find out risk factors for oxygen radical injuries based on birth history, laboratory data, neuroimaging findings and clinical progress in two preterm groups. Results : Serum TH levels on postnatal day 1 were higher in the preterm infant group than the term infant group. Serum TH levels on postnatal day 1 in the complicated preterm infant group were significantly higher compared with uncomplicated group, but there was no significant difference in serum TH levels on postnatal day 7. Also, there was no significant difference in serum TH levels between uncomplicated preterm infants and term infants. Serum TH level on postnatal day 1 was independently associated with higher morbidity after adjusting for gestational age, Apgar score (5 min), arterial blood gas analysis. Conclusion : Complicated preterm newborns are at increased risk for oxidative stress compared with uncomplicated newborns and term newborns. Oxidative injury during the prenatal or postnatal day 1 is associated with adverse outcomes in preterm infants. Elevated TH levels on postnatal day 1 may have a value to predict clinical outcomes in preterm infants.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.157-165
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.