• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.35-42
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• 2018

Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

• THE JOURNAL OF KOREAN ORIENTAL ONCOLOGY
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• v.4 no.1
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• pp.37-53
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• 1998

The sprig of Jinryungtang Gagambang has been used for curing as a traditional medicine without any experimental evidence to support the rational basis for their clinical use. This experiment was carried out to evaluate the possible therapeutic or antitumoral effects of Jinryungtang Gagambang extract against cancer, and to study some mechanisms responsible for its effect. The cytotoxic and antitumor effects were evaluated on human cell liens (A549, hep3B, Caki-1, Sarcoma 180) after exposure to Jinryungtang Gagambang extract using in ILS, colony forming efficency and SRB assay which were regarded as a valuable method for cytotoxic and antitumor effects of unknown compound on tumor cell lines. The results obtained in this studies were as follows. 1. As a result of exposure to Jinryungtang Gagambang extract, the proliferation of A549, hep3B, Caki-1, good correlations were shown from the results of SRB assay and those of clogenetic assay. 2. The oral administration of Jinryungtang Gagambang extract showed significant effects of increase of MST(mean survival time) and ILS(increased life span) depending on the increasing concentration. 3. Against squamous cell carcinoma induced by MCA, Jinryungtang Gagambang decreased not only the frequency of tumor production but also the number and weight of tumors per tumor bearing mice(TBM). Jinryungtang Gagambang also significantly suppressed the development of 3LL cell-implanted tumors by frequency and their size, and some developed tumors were regressed by the continuous treatment of Jinryungtang Gagambang extract into TBM. 4. Jinryungtang Gagambang extract also increased NK cell activities. According to the above results, it could be suggested that Jinryungtang Gagambang extract has prominent antiutmor effect.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.25-30
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• 2016

Purpose: We undertook this study to investigate discrepancies in C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values, and variations following intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD). Methods: A total of 123 KD patients were retrospectively enrolled. Patients were treated with IVIG 2 g/kg at 2 to 9 days after disease onset. We obtained white blood cell (WBC) count, percentage of neutrophils (% neutrophils), CRP, ESR, and N-terminal pro-brain natriuretic peptide (NT-proBNP) values before and 48 to 72 hours after IVIG treatment. Discrepancy was defined as $CRP{\geq}10mg/dL$ and ESR <50 mm/hr (Group 1), or CRP <10 mg/dL and $ESR{\geq}50mm/hr$ (Group 2). Results: Thirty-six of 123 subjects (29.2%) had a discrepancy: 25 (20.3%) in Group 1 and 11 (8.9%) in Group 2. In Group 1, 15 patients (60%) had fever for <5 days (early presenter) and 10 (40%) had fever for ${\geq}5days$ (late presenter). There were six early presenters (55%) and five late presenters (45%) in Group 2. Late presenters had higher ESR than early presenters ($34.3{\pm}21.0mm/hr$ vs. $26.3{\pm}19.3mm/hr$, P=0.029). After IVIG treatment, elevated WBC count, % neutrophils, CRP, and NT-proBNP levels normalized. In contrast, ESR increased from $37.4{\pm}21.9mm/hr$ to $48.0{\pm}22.7mm/hr$ (n=36, P=0.051). Conclusions: A discrepancy may be related to the duration of fever. Due to discrepancies in CRP and ESR values in acute KD, both should be measured to assess the degree of inflammatory activity before IVIG treatment. After IVIG treatment, the ESR should not be used as a marker of response to therapy in KD.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.221-227
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• 1985

Thirty-four patients with suspected ectopic pregnancy whose serum hCG levels had been measured by beta-hCG EIA before surgery were evaluated retrospectively. The results were as follows: 1. Final diagnosis of thirty-four patients with suspected ectopic pregnancy comprised twenty- eight tubal pregnancy, five ruptures of hemorrhagic corpus luteum and, one tubo-ovarian abscess. One of the five patients with rupture of hemorrhagic corpus luteum was accompanied by missed abortion. 2. Range of serum hCG levels in twenty-eight patients with tubal pregnancy was 59-21,980 mIU/ml and that of four patients with rupture of hemorrhagic corpus luteum and one patient with tubo-ovarian abscess was 0.6-6.6mIU/ml. Serum hCG level of a patient with rupture of hemorrhagic corpus luteum who was accompanied by missed abostion was 200 mIU/ml. 3. Serum hCG levels in twenty-two of twenty-eight patients with tubal pregnancy were lower than 3,000 mIU/ml. Low serum hCG level below 100mIU/ml and high serum hCG level above 6,500 mIU/ml were noticed in four and six patients with tubal pregnancy, respectively. 4. Mean serum hCG levels (${\pm}SD$) of twelve patients with tubal pregnancy who had intra-abdominal free blood of less than 500ml and sixteen patients with tubal pregnancy who had intra-abdominal free blood of more than 500ml were 4,131 (${\pm}7,801$) mIU/ml and 3,208 (${\pm}5,081$) mIU/ml, respectively. There was no statistical difference in the mean level of serum hCG between both group (P>0.05). 5. Mean serum hCG levels (${\pm}SD$) of thirteen patients with unruptured tubal pregnancy and fifteen patients with ruptured tubal pregnancy were 2,628 (${\pm}5,448$) mIU/ml and 4,449 (${\pm}6,938$) mIU/ml, respectively. Mean level of serum hCG was statistically higher in ruptured tubal pregnancy (P<0.01). 6. Positive rate of urine pregnancy test in the diagnosis of ectopic pregnancy was 64% (16/25) and mean range of serum hCG level of nine patients with false negative result were 353 mIU/ml and 59-933 mIU/ml.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.1
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• pp.11-18
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• 2004

The eruption of permanent teeth represents the movement in the alveolar bone before appearance in oral cavity, to the occlusal plane after appearance in oral cavity, and additive movement after reaching th the occlusal plane. Tooth eruption is mostly controlled by genetic signals. The eruption stage is divided to preeruptive alveolar stage, alveolar bone stage, mucosal stage according to the process of growth and development. If the disturbance is occured in any stage of eruption, tooth does not erupt. The cause of eruption disturbance are ectopic position of the tooth germ, obstruction of the eruption path and defects in the follicle or PDL. In the treatment of eruption disturbance, surgical procedures are commonly used. There are three kind of surgical procedure ; surgical exposure, surgical repositioning, surgical exposure and traction Surgical exposure is basic procedure. This involves removal of mucosa, bone, lesion that are surrounding the teeth, dental sac when necessary to maintain a patent channel between the crown and the normal eruptive path into the oral cavity. To ensure this patency, many techniques including cementation of a celluloid crown, packing with gutta-percha or zinc oxide-eugenol, or a surgical pack, are used. When surgical exposure is conducted, operators should not expose any part of cervical root cement and not injure periodontium or root of adjunct tooth. After surgical exposure, tooth should be surrounded by keratinized gingiva. There is direct relationship between the extent of development of pathophysiologic aberrations and the intensity of the manipulative injury inflicted on the tooth by surgical treatment, so operator should consider this thing. In these cases, surgical exposure is conducted on Maxillary 1st milars that have a eruption disturbance and improve the eruption disturbance effectively.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.80-87
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• 1999

Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.

• Journal of Chest Surgery
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• v.37 no.8
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• pp.691-701
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• 2004

Background: Tissue hypoxia is a characteristic of many human malignant neoplasms, and hypoxia inducible factor-1 (HIF-1) plays a pivotal role in essential adaptive response to hypoxia, and activates a signal pathway for the expression of the hypoxia-regulated genes, resulting in increased oxygen delivery or facilitating metabolic adaptation to hypoxia. Increased level of HIF-1 a has been reported in many human malignancies, but in esophageal squamous cell carcinoma, the influence of HIF-1 a on tumor biology, including neovascularization, is not still defined. Material and Method: The influence of HIF-1 a expression on angiogenic factors, correlation between the tumor proliferation and HIF-1 a expression, interaction of HIF-1 a expression and p53, and correlation between HIF-1 a expression and clinicopathological prognostic parameters were investigated, using immunohistochemical stains for HIF-1 a, VEGF, CD34, p53, and Ki-67 on 77 cases of resected esophageal squamous cell carcinoma. Result: HIF-1 a expression in cancer cells was found in 33 of 77 esophageal squamous cell carcinoma cases. The 33 cases (42.9%) showed positive stain for HIF-1 a. High HIF-1 a expression was significantly associated with several pathological parameters, such as histologic grade (p=0.032), pathological TMN stage (p=0.002), the depth of tumor invasion (p=0.022), regional lymph node metastasis (p=0.002), distant metastasis (p=0.049), and lymphatic invasion (p=0.004). High HIF-1 a expression had significant VEGF immunoreactivity (p=0.008) and Ki-67 labeling index (p<0.001), but was not correlated with microvascular density within tumors (p=0.088). The high HIF-1 a expression was correlated with aberrant p53 accumulation with a marginal significance (p=0.056). The overall 5-year survival rate was 34.9%. The survival rate of patients with a high HIF-1 a expression was worse than that of patients with low-expression tumors (log-rank test, p=0.0001). High HIF-1 a expression was independent unfavorable factors although statistical significance is marginal in multivariate analysis. Conclusion: It is suggested that (1) high HIF-1 a expression in esophageal squamous cell carcinoma is associated with tumor hypoxia, or with genetic alteration in early carcinogenesis and progressive stages, (2) high HIF-1 a expression may be associated with intratumoral neovascularization through HIF-VEGF pathway, and (3) high HIF-1 a expression is associated with poor prognosis in patients with esophageal squamous cell carcinoma and may playa role as biomarker for regional lymph node metastasis.

• Journal of the Korean Society of Food Science and Nutrition
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• v.32 no.8
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• pp.1357-1363
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• 2003

This study was conducted to investigate the effect of chicken egg containing IgY against H. pylori in patients with gastritis. Sixty three H. pylori-infected volunteers (20∼43 year, Male Female=49 : 14) were randomized into four groups which were treated with one chicken egg containing IgY b.i.d. (IgY group; n=17) or omeprazole 20 mg b.i.d., amoxicillin 1.0 g b.i.d. and clarithromycin 500 mg b.i.d. (OAC group; n=17) or omeprazole 20 mg b.i.d., amoxicillin 1.0 g b.i.d., clarithromycin 500 mg b.i.d. and one chicken egg containing IgY b.i.d. (OAC with IgY group; n=16) for 2 weeks or lyophilized IgY 1 g b.i.d (lyophilized IgY group) for 1 month. $\Delta$$^{13}$ $CO_2$ before and after treatment, the eradication rate of H. pylori and histologic change including H. pylori density, acute and chronic inflammation activity, intestinal metaplasia and glandular atrophy by updated sydney system were evaluated. Eradication rate of OAC with IgY group (94%) was higher than IgY group (0%), lyophilized IgY group (0%) and OAC group (88%). $\Delta$$^{13}$ $CO_2$at 2 weeks after treatment in one patient of IgY group was decreased. But that was not changed in the other patients. $\Delta$$^{13}$ $CO_2$ at 1 week after treatment in 15 patients of OAC with IgY group was significantly lower than pretreatment level (p<0.05), and $\Delta$$^{13}$ $CO_2$ at 1 week and 2 week after treatment was decreased in the other patient. Acute inflammation activity at antrum was significantly decreased after treatment in IgY and lyophilized IgY group (p<0.01), H. pylori density at antrum was significantly decreased after treatment in IgY and lyophilized IgY group (p<0.05). Chronic inflammation activity at body was decreased after treatment in lyophilized IgY group. Intestinal metaplasia and glandular atrophy at antrum and body were not changed after treatment in IgY group. Mild intestinal metaplasia in one patient of lyophilized IgY group changed to normal after 1 month treatment. Gandular atrophy at antrum and body were not changed after treatment in lyophilized IgY group.

• 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.

• Journal of Yeungnam Medical Science
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• v.22 no.1
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• pp.52-61
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• 2005

Background: Guillain-Barre syndrome is defined as a recognizable clinical entity that is characterized by rapidly evolving symmetric limb weakness, the loss of tendon reflexes, absent or mild sensory signs, and variable autonomic dysfunctions. This study evaluated the clinical and electrophysiological findings retrospectively. Materials and Methods: Forty-five patients with Guillain-Barre syndrome, who were admitted to the Yeungnam University Hospital for six years from Jan. 1994 to Dec. 1999 were investigated. The correlation between the clinical manifestation and the electrophysiological study was evaluated. Results: The male to female ratio was 1.8:1 and there was a peak seasonal incidence in the winter. A preceding illness was noted in 66.7 % of cases, and an upper respiratory tract infection was the most common one. The most common clinical manifestations were a loss of tendon reflex and ascending muscle weakness and paralysis. The cerebrospinal fluid examinations revealed, albuminocytologic dissociation in 33 cases (73.3 %). Intravenous immunoglobulin therapy was performed in 29 cases (64.4 %). The sequential electrophysiological abnormalities were most marked at 2 to 4 weeks after onset. At that time the most significant change was a decrease in the compound muscle action potential amplitude. These 45 patients with Guillain-Barre syndrome were subclassified using the clinical and electrophysiological data. Conclusion: The result in this study, concured with other research on the clinical and electrophysiological data of Guillain-Barre syndrome. However, an extensive and dynamic investigation is necessary to determine the reason for the peak seasonal incidence in winter.