• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Pediatric Infection and Vaccine
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• v.14 no.2
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• pp.171-178
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• 2007

Purpose : We investigated clinical characteristics of real-time PCR proven EBV viremia patients who were not serologically diagnosed but clinically suspected, and compared it to serologically proven EBV infected patients. Methods : The study population consisted of 45 patients, who were suspected acute EBV infection at Wonju Christian Hospital Department of Pediatrics, Yonsei University Wonju College of Medicine from Jan. 2004 to Dec. 2006. real-time PCR of cell free serum was performed to prove EBV viremia. Then we chose $102.5copies/{\mu}g$ DNA as a suitable cutoff level for EBV associated diseases. Results : There are 4 patients diagnosed as EBV infection by serologically and 15 patients diagnosed as EBV viremia by real-time PCR quantitative measurement. The most common presenting symptoms and signs of EBV viremia was fever in 11 cases (73%). Atypical lymphocytosis was found in 4 cases (27%). Increased AST, ALT levels were observed in 13 cases (87%), 12 cases (80%), respectively. We could diagnose 5 cases of EBV viremia younger than one year of age. They revealed clinical symptoms which could be found in EBV infection. The serologically diagnosed patients had hepatomegaly and splenomegaly in 3 cases (75%). All serologically confirmed patients have leukocytosis above $20,000/mm^3$, among them 2 cases (50%) had higher percentage (>15%) of atypical lymphocytes. The AST/ALT level above 50 IU/L were demonstrated in all cases. Conclusion : Serologically unproven real-time PCR EBV viremia patients revealed similar clinical findings with that of serologically proven EBV infected patients. So, it is meaningful to perform EBV real-time PCR for the diagnosis of EBV infection especially for the cases younger than 1 year of age.

• The Korean Journal of Nuclear Medicine
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• v.33 no.6
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• pp.493-501
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• 1999

Purpose: We conducted a comparative study to evaluate the diagnostic values of T1-201, Tc-99m MIBI and I-131 scans in the follow-up assessment after ablative I-131 therapy in differentiated thyroid cancer. Materials and Methods: The study population consisted of 20 patients who underwent surgical removal of thyroid cancer and ablative radioactive iodine therapy, and followed by one or mote times of I-131 retreatments (33 cases). In all patients, T1-201, Tc-99m MIBI, diagnostic and therapeutic I-131 scans were performed and the results were analyzed retrospectively. Also serum thyroglobulin levels were measured in all patients. The final diagnosis of recurrent or metastatic thyroid cancer was determined by clinical, biochemical, radiologic and/or biopsy findings. Results: Positive rates (PR) of Tc-99m MIBI, T1-201, diagnostic and therapeutic I-131 scans in detecting malignant thyroid tissue lesions were 70% (19/27), 54% (15/28), 35% (17/48) and 63% (30/48), respectively. The PR in the group of 20 cases (28 lesions) who underwent concomitant T1-201 and I-131 scans were in the order of therapeutic 131 scan 71%, T1-201 scan 54% and diagnostic I-131 scan 36%. There was no statistically significant difference between T1-201 and diagnostic I-131 scans (p>0.05). In the group of 20 cases (27 lesions) who underwent concomitant Tc-99m MIBI and I-131 scans, the PR were in the order of Tc-99m MIBI scan 70%, I-131 therapeutic scan 52% and I-131 diagnostic scan 33%. The PR of Tc-99m MIBI was significantly higher than that of diagnostic I-131 scan (p<0.05). Conclusion: Tc-99m MIBI scan is superior to diagnostic I-131 scan in detecting recurrent or metastatic thyroid cancer following ablation therapy in patients with differentiated thyroid cancer. T1-201 scan did not showed significantly higher positive rate than diagnostic I-131 scan. Instead of diagnostic I-131 scan before the I-131 retreatment, Tc-99m MIBI scan without discontinuing thyroid hormone replacement would be a prudent and effective approach in the management of these patients.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.736-741
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• 2008

Purpose : The incidence of thromboembolic episodes in children with nephrotic syndrome (NS) is low; however, these episodes are often severe. Moreover, both pulmonary thromboembolism (PTE) and renal vein thrombosis (RVT) rarely show clinical symptoms. This study was performed to determine the benefits of routine screening in the detection of thrombosis in childhood NS. Methods : Among 62 children with nephrotic syndrome, a total of 54 children (43 males, 11 females) were included in this study. When the patients experienced their first NS episode, we performed renal Doppler ultrasonography in order to detect RVT. To rule out the possibility of PTE, a lung perfusion scan was performed. Computed tomographic (CT) pulmonary angiography was recommended to patients who showed possible signs of PTE. All patients were evaluated for clinical signs of thrombosis, biochemical indicators of renal disease, as well as clotting and thrombotic parameters. Results : RVT or related clinical symptoms were not observed in any children. Based on the findings of the lung perfusion scans, 15 patients (27.8%) were observed with as a high probability of PTE. We were able to perform a CT pulmonary angiography only on 12 patients, and 5 patients were diagnosed with PTE (prevalence 8.1%). The serum fibrinogen level in the group with PTE was significantly higher ($776.7{\pm}382.4mg/dL$, P<0.05) than that in the group without PTE, and other parameters were not significantly different between each group. Conclusion : Further studies are required for clarifying the role of renal Doppler ultrasonography for the detection of RVT in NS. Children with NS who developed non-specific respiratory symptoms should be evaluated for the diagnosis of PTE. In the management of NS, a lung perfusion scan should be performed at the time of the initial episode of NS regardless of the pulmonary symptoms, since patients having PTE are either often asymptomatic, or present with nonspecific symptoms.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• 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.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.473-481
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• 2002

Purpose : Ascending cholangitis is the most common complication after Kasai operations. The aim of this study is to evaluate the therapeutic efficacy of cefotaxime as an empirical antibiotic on ascending cholangitis after Kasai operations. Methods : Thirty-nine episodes of cholangitis in twenty-nine children who underwent Kasai operations at Seoul National University Children's Hospital from January 1991 to December 2000 were included in this study. Empirical cefotaxime treatments were divided into three groups : cefotaxime and amikacin treatment group(CA group), cefotaxime and gentamicin treatment group(CG group) and cefotaxime treatment group(C group). A diagnosis of cholangitis was made on the basis of unexplained fever(>$38^{\circ}C$) and either development of acholic stool or elevation of serum total bilirubin (>1.5 mg/dL). Therapeutic efficacy was judged by elimination of fever up to 72 hours, 120 hours, and 168 hours after antibiotic treatment. Results : There were therapeutic responses in 51%(20/39) up to 72 hours after antibiotic treatment : 54%(13/24) in CA group, 43%(3/7) in CG group and 50%(4/8) in C group. There were therapeutic responses in 69%(27/39) up to 120 hours, in 79%(31/39) up to 168 hours and in 82%(32/39) up to 2 weeks. There were no differences in therapeutic efficacy among the three regimens. In 12 of 39 episodes, the etiologic pathogens including Escherichia coli and enterococcus were cultured from the blood. Conclusion : Cefotaxime can be tried as an initial antibiotic in Korean children with ascending cholangitis after Kasai operation prior to the identification of microorganism on culture. However, further evaluation of pathogen and its resistant strain to cefotaxime should be done.

• Tuberculosis and Respiratory Diseases
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• v.59 no.6
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• pp.656-663
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• 2005

Background : The B-type natriuretic peptide (BNP) is a cardiac neurohormone that is specifically secreted from the ventricles in response to volume expansion and a pressure overload. Differentiating congestive heart failure from the pulmonary causes of dyspnea is very important for patients presenting with acute dyspnea. Methods : A retrospective study was carried out on 261 patients who were admitted to the emergency department of Hanyang University Hospital due to acute dyspnea from March to July 2004. The serum BNP levels of the patients were measured using the ELISA method. Results : The BNP levels were 382, 111-1140 pg/ml (median, interquartile range) in the heart failure group (n=119) and 29, 7-81 pg/ml in the non-heart failure group (n=142). The BNP levels according to the subgroups of heart failure were 820, 354-1620 pg/ml, 1650, 239-1990 pg/ml, and 378, 106-1120 pg/ml for the chronic obstructive pulmonary disease (COPD) with combined left heart failure (n=5), cor pulmonale (n=3), and left heart failure groups (n=111), respectively. The BNP levels according to the subgroups of non-heart failure were 39, 21-101 pg/ml, 59, 10-129 pg/ml, and 15, 6-47 pg/ml for the COPD (n=20), other pulmonary diseases (n=56), and other causes groups (n=66), respectively. The BNP levels were significantly different according to the underlying etiology (p<0.001), and were significantly higher in the COPD patients with left heart failure than in those without (p=0.002). When the patients with no cardiovascular risk factor such as diabetes mellitus, hypertension, smoking or renal failure were analyzed, the BNP levels were also significantly higher in the patients with heart failure than in those without(p<0.001). When 133 pg/ml was designated as the BNP cut-off level the sensitivity for predicting heart failure was 73 percent and the specificity was 87 percent. Conclusion : BNP measurements are useful for determining the presence of heart failure in the patients presenting with acute dyspnea. The possibility of heart failure must be seriously considered in patients with high BNP levels even when they have a pre-existing pulmonary disease such as COPD or no risk factors for heart failure.

• Tuberculosis and Respiratory Diseases
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• v.66 no.6
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• pp.451-456
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• 2009

Paragonimiasis is a parasitic infection that occurs following the ingestion of infectious Paragonimus metacercariae, which occurs as a result of eating raw or undercooked freshwater crabs or crayfish. Pulmonary paragonimiasis is the most common clinical manifestation of this infection. Human paragonimiasis occurs sporadically. We experienced a case of pulmonary paragonimiasis in a 35-year-old woman with left lower chest pain. The patient had hypereosinophilia and a pleural effusion. The diagnosis was confirmed by positive ELISA (Enzyme-linked immunosorbent assay) that detected Paragonimiasis westermani antibody in the serum. We treated the patient with praziquantel for two days at a daily dosage of 75 mg/kg. Left pleuritic pain and pleural effusion improved after treatment. However, similar symptoms and pleural effusion developed recurrently for the first 3 courses of treatment with praziquantel. Upon the fourth round of treatment, the patient made a full recovery.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.964-970
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• 2008

Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.