• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.453-473
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• 1992

Background: National survey was performed to estimate the incidence of sarcoidosis in Korea. The clinical data of confirmed cases were analysed for the practice of primary care physicians and pulmonary specialists. Methods: The period of study was from January 1991 to December 1992. Data were retrospectively collected by correspondence with physicians in departments of internal medicine, dermatology, ophthalmology and neurology of the hospitals having more than 100 beds using returning postcards. In confirmed and suspicious cases of sardoidosis, case record chart for clinical and laboratory findings were obtained in detail. Results: 1) Postcards were sent to 523 departments in 213 hospitals. Internal medicine composed 41%, dermatology 20%, ophthalmology 20% and neurology 19%. 2) Postcards were returned from 241 departments (replying rates was 48%). 3) There were 113 confirmed cases from 50 departments and 10 cases. The cases were composed from internal medicine (81%), dermatology (13%), ophthalmology (3%) and neurology (3%). 78 confirmed cases were analysed, which were composed from department of internal medicine (92%), dermatology (5%), and neurology (3%). 4) The time span for analysed cases was 1980 to 1992. one case was analysed in 1980 and the number gradually increased to 18 cases in 1991. 5) The majority of patients (84.4%) were in the age group of 20 to 49 years. 6) The ratio of male to female was 1 : 1.5. 7) The most common chief complains were respiratory symptoms, dermatologic symptoms, generalized discomforts, visual changes, arthralgia, abdominal pains, and swallowing difficulties in order. 16% of the patients were asymptomatic. 8) Mean duration between symptom onset and diagnosis was 2 months. 9) The most common symptoms were respiratory, general, dermatologic, ophthalmologic, neurologic and cardiac origin in order. 10) Hemoglobin, hematocrits and platelet were in normal range. 58% of the patients had lymphopenia measuring less than 30% of white cell count. The ratio of CD4 to CD8 lymphocytes was $1.73{\pm}1.16$ with range of 0.43 to 4.62. ESR was elevated in 43% of the cases. 11) Blood chemistry was normal in most cases. Serum angiotensin converting enzyme (S-ACE) was $66.8{\pm}58.6\;U/L$ with the range of 8.79 to 265 U /L. Proteinuria of more than 150 mg was found in 42. 9% of the patients. 12) Serum IgG was elevated in 43.5%, IgA in 45.5%, IgM in 59.1% and IgE in 46.7%. The levels of complement C3 and C4 were in the normal range. Anti-nuclear antibody was detected in 11% of the cases. Kweim test was performed in 3 cases, and in all cases the result was positive. 13) FVC was decreased in 17.3%, FEV1 in 11.5%, FEV1/FVC in 10%, TLC in 15.2%, and DLco in 64.7%. 14) PaO2 was decreased below 90 mmHg in 48.6% and PaCO2 was increased above 45 mmHg in 5.7%. 15) The percentage of macrophages in BAL fluid was $51.4{\pm}19.2%$, lymphocytes $44.4{\pm}21.1%$, and the ratio of CD4 to CD8 lymphocytes was $3.41{\pm}2.07$. 16) There was no difference in laboratory findings between male and female. 17) Hilar enlargement on chest PA was present in 87.9% (bilaterally in 78.8% and unilaterally in 9.1%). 18) According to Siltzbach's classification, stage 0 was 5%, stage 158.3%, stage 228.3%, and stage 38.3%. 19) Hilart enlargement on chest CT was present in 92.6% (bilaterally 76.4% and unilaterally in 16.2%). 20) HRCT was done in 16 cases. The most common findings were nodules, interlobular thickening, focal patchy infiltrations in order. Two cases was normal finding. 21) Other radiologic examinations showed bone change in one case and splenomegaly in two cases. 22) Gallium scan was done in 12 cases. Radioactivity was increased in hilar and mediastinal lymph nodes in 8 cases and in parenchyme in 2 cases. 23) The pathologic diagnosis was commonly performed by transbrochial lung biopsy (TBLB, 47.3%), skin and mediastinal lymph nodes biopsy (34.5%), peripheral lymph nodes biopsy (23.6%), open lung biopsy (18.2%) and bronchial biopsy in order. 24) The most common findings in pathology were non·caseating granuloma (100%), multi-nucleated giant cell (47.3%), hyalinized acellular scar (34.5%), reticulin fibrin network (20%), inclusion body (10.9%), necrosis (9.1%), and lymphangitic distribution of granuloma (1.8%) in order. Conclusion: Clinical, laboratory, radiologic and pathologic findings were summarized. This collected data will assist in finding a test for detection and staging of sarcoidosis in Korea in near future.

• Investigative Magnetic Resonance Imaging
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• v.5 no.1
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• pp.43-48
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• 2001

Purpose : To evaluate changes in rabbit liver parenchyma on MR images following percutaneous Holmium-166 injection, and to correlate those changes with histologic findings. Materials and methods. Holmium-166 (10-25 mCi) was percutaneously injected into the liver of rabbit (n=12) under sonographic guidance. MR images were obtained between one to two weeks (acute phasea) after the injection in four rabbits, and between two to four weeks (subacute phase) after the injection in four rabbits. Tissue specimens of these eight rabbits were obtained immediately after MR imaging. Tissue specimens were obtained without MR imaging in four rabbits (between one to two weeks in one rabbit and between three to four weeks in three rabbits). Results : Tissue specimens showed central liquefactive necrosis and peripheral coagulative necrosis containing deposition of small particles and hemorrhage. The peripheral margin of the lesions showed formation of the granulation tissue with fibrosis, which tended to be more prominent in subacute phase. The area of the necrosis tended to correlate with the dose of the radioactive Holmium-166. On MR images, the central portion of the necrosis showed hyperintensity on 72-weighted image, hypointensity on the precontrast T1-weighted images, and no enhancement on the dynamic MR images. The peripheral portion of the necrosis showed hypointensity on T2-weighted images, iso or mild hypointensity on the T1-weighted images, and mild peripheral enhancement on the delayed dynamic MR images. The peripheral margin of the lesion showed hypointensity on both T1- and T1-weighted images with increased enhancement on the delayed phase images of the dynamic MR images. Conclusion : After percutaneous Holmium-166 injection into rabbit liver parenchyma, the central portion showed liquefactive necrosis, the peripheral portion showed coagulative necrosis with granulation, fibrosis, hemorrhage and depostition of small granules. MR imaging may be helpful in evaluation of the histological change of the liver after percutaneous Holmium-166 treatment.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.9-14
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• 2001

Purpose : This study was undertaken to investigate clinical aspects of nutcracker syndrome in children including sexual distribution, age of onset, the ratio of peak flow velocity between the narrowed and dilated portions in the left renal vein and prevalence of hematuria or proteinuria. Materials and Method : The subjects included 30 patients diagnosed with nutcracker syndrome using Doppler sonography from September 1999 to January 2001 in Severance Hospital, Department of Pediatric Nephrology. The diagnostic criteria for nutcracker syndrome was a peak flow velocity of ratio is more than 5.0. Results : Patients consisted of 14 males and 16 females. The mean age was 9.19${\pm}$2.31 years old and the ratio of peak flow velocity was 8.52${\pm}$2.24. Age of onset us higher in males 9.68${\pm}$2.82 year old than in females, 8.76${\pm}$1.72 year old respectively(P<0.05). But there was no difference in the ratio of peak flow velocities ; 8.45${\pm}$2.31 in the male group , 8.58${\pm}$2.25 in the female group(P>0.05). There was no difference in the age of onset and the ratio of peak flow velocity between patients with hematuria and without hematuria. Among patients with nutcracker syndrome, 24 cases of hematuria, 3 cases of proteinuria only, and 3 cases of hematuria with proteinuria. Conclusion : In children of school age, early diagnosis of nutcracker syndrome and consistent follow up is worthy of consideration, especially ill the prevention of sequelae. (J. Korean Soc Pediatr Nephrol 5 : 9-14, 2001)

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1324-1328
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• 2006

Purpose : The object of this study was to determine the difference between two methods for myocardial performance index(MPI) in children, using the conventional and pulsed Doppler echocardiography. Methods : A total of 27 children with anatomically normal hearts were enrolled for the study. all were examined by conventional and pulsed Doppler echocardiography at Gangneung Asan Hospital between December, 2005 and February, 2006. First, we measured the time interval(a1) between the mitral inflows from apical 4-chamber view, and the ejection time(ET1) from apical 5-chamber view. And then, we calculated MPI1, isovolumic contraction time(ICT1) and isovolumic relaxation time (IRT1). Secondly, we measured ICT2, ET2 and IRT2 from apical 5-chamber view with a Dopper signal placed at just below junction between mitral and aortic valve at the same cardiac cycle. And then, we calculated MPI2. We compared MPI1 to MPI2. All MPIs were calculated by using the formula, MPI=(ICT+IRT)/ET. Results : The mean age was $5.7{\pm}2.2years$ old(M:F=15:12). The MPI2 was higher than MPI1: $0.277{\pm}0.083$ vs. $0.428{\pm}0.081$(MPI1 vs MPI2, P=0.000). Also, the ICT2 was higher than ICT1: $56{\pm}15msec$ vs $97{\pm}18msec$(ICT1 vs ICT2, P=0.000) and the IRT2 was higher than IRT1: $42{\pm}8msec$ vs $53{\pm}9msec$(IRT1 vs IRT2, P=0.000). But, the ET2 was lower than ET1: $260{\pm}16msec$ vs $254{\pm}14msec$ (ET1 vs ET2, P=0.01). There was, as well, positive linear correlation between MPI1 and MPI2. Conclusion : This study showed that there is a difference between MPI1 and MPI2 in connection with estimating methods. However, the two MPIs had a positive linear correlation. Judging from our results, the MPI of the new method might be a useful index of venticular global function in children.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.655-660
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• 2009

Purpose : To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. Methods : Live-born infants with SUA (n=59) detected by physical examination from among 15,193 live births in seven university hospitals in Korea between January 1, 2004, to August 1, 2007, were reviewed retrospectively, with 236 normal infants serving as the control group. Results : A statistical difference was observed between the groups in birth weight and in vitro fertilization. The incidence of infants with SUA was 0.37%. Congenital malformations were observed in 21 infants with cardiovascular (n=15, 25.4%), gastrointestinal (n=2, 3.4%), genitourinary (n=9, 15.3%), neuromusculoskeletal (n=6, 10.2%), central nervous system (n=1, 1.7%), chromosomal (n=1, 1.7%), and other (n=3, 5.1%) abnormalities. There were 49 (83.1%) infants with isolated SUA in this study population; among them, the associated congenital malformations were cardiovascular (n=6, 12.2%) and genitourinary (n=6, 12.2%) abnormalities. Two infants with cyanotic heart disease were operated and four infants with acyanotic heart disease showed improvements without any treatment. Six infants with genitourinary abnormalities on renal ultrasound had mild hydronephrosis without further consequences. Conclusion : The incidence of structural abnormalities in the cardiovascular and genitourinary systems is high and the genitourinary anomalies associated with isolated SUA have relatively good prognosis.

• Journal of Chest Surgery
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• v.42 no.1
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• pp.9-13
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• 2009

Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

• Journal of Nuclear Fuel Cycle and Waste Technology(JNFCWT)
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• v.5 no.3
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• pp.199-212
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• 2007

If a type IP-2 transport package were to be subjected to a free drop test and a penetration test under the normal conditions of transport, it should prevent a loss or dispersal of the radioactive contents and a more than 20% increase in the maximum radiation level at any external surface of the package. In this paper, we suggested the analytic method to evaluate the structural safety of a type IP-2 transport package using a thick steel plate for a structure part and a bolt for tying a bolt. Using an analysis a loss or dispersal of the radioactive contents and a loss of shielding integrity were confirmed for two kinds of type IP-2 transport packages to transport radioactive waste drums from a waste facility to a temporary storage site in a nuclear power plant. Under the free drop condition the maximum average stress at the bolts and the maximum opening displacement of a lid were compared with the tensile stress of a bolt and the steps in a lid, which were made to avoid a streaming radiation in the shielding path, to evaluate a loss or dispersal of radioactive waste contents. Also a loss of shielding integrity was evaluated using the maximum decrease in a shielding thickness. To verify the impact dynamic analysis for free drop test condition and evaluate experimentally the safety of two kinds of type IP-2 transport packages, free drop tests were conducted with various drop directions. For the tests we examined the failure of bolts and the deformation of flange to evaluate a loss or dispersal of radioactive material and measured the shielding thickness using a ultrasonic thickness gauge to assess a loss of shielding integrity. The strains and accelerations acquired from tests were compared with those by analyses to verify the impact dynamic analysis. The analytic results were larger than the those of test so that the analysis showed the conservative results. Finally, we evaluated the safety of the type IP-2 transport package under the stacking test condition using a finite element analysis. Under the stacking test condition, the maximum Tresca stress of the shielding material was 1/3 of the yielding stress. Two kinds of a type IP-2 transport package were safe for the free drop test condition and the stacking test condition.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.381-387
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• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.234-241
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• 2009

Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-${\alpha}$), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-${\alpha}$, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute febrile illness (control group). TNF-${\alpha}$ and IL-6 were determined by sandwich enzyme-linked immunosorbent assay (ELISA). Echocardiography was performed to detect coronary artery lesions (CAL) in KD patients. Results : Low $T_3$ syndrome occurred in 63.5% of KD patients. $T_3$ in the acute phase of KD was lower than that in the control. In KD patients, $T_3$ was lowered in the acute phase and elevated in the subacute phase, whereas TNF-${\alpha}$, IL-6 and NT-proBNP were elevated in the acute phase and decreased in the subacute phase. NT-proBNP, and IL-6 were higher in patients with low $T_3$ than in those with normal $T_3$. In addition, $T_3$ inversely correlated with IL-6 and NT-proBNP. Of the 4 patients with CAL, 3 had very low $T_3$. Compared with intravenous immunoglobulin (IVIG)-responsive patients, IVIG-resistant patients had lower $T_3$ and higher IL-6 and NT-proBNP. Conclusion : $T_3$ decreases in the acute phase of KD and normalizes in the subacute phase without thyroid hormone replacement. Low $T_3$ may be partially induced by IL-6 rather than TNF-${\alpha}$, and is strongly associated with high NT-proBNP. $T_3$ in KD may be used for the differential diagnosis, monitoring the activity of the disease, and predicting the severity of inflammation.