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Lorenzo's Oil Treatment in X-linked Adrenoleukodystrophy (X 염색체 연관 부신백질이영양증에서 Lorenzo Oil의 투여효과)

  • Kim, Hyo Jung;Kim, Eun Young;Woo, Young Jong;Kook, Hoon
    • Clinical and Experimental Pediatrics
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    • v.48 no.11
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    • pp.1232-1238
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    • 2005
  • Purpose : Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X-linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods : Four patients who were treated with Lorenzo's oil for at least 12 months were enrolled from 1996 to 2003. During treatment, changes of neurologic symptoms, brain magnetic resonance imaging(MRI) findings, and serum saturated very long-chain fatty acid(C26:0) were assessed. Results : Two patients with childhood cerebral ALD had progression of neurologic symptoms and MRI lesions during treatment. One asymptomatic patient developed childhood cerebral ALD after six year treatment of Lorenzo's oil. One "Addison only" patient remained neurologically intact after three years. During Lorenzo's oil therapy, serum C26:0 levels which had increased at diagnosis decreased in all four patients. Conclusion : Treatment with Lorenzo's oil did not prevent disease progression in childhood cerebral ALD patients who were already symptomatic. Long term follow up will be needed in asymptomatic ALD including "Addison only" to reveal the efficacy of Lorenzo's oil.

Voriconazole Therapeutic Drug Monitoring is Necessary for Children with Invasive Fungal Infection (소아에서 보리코나졸 치료적 약물 농도 모니터링의 임상적 의의)

  • Kang, Hyun Mi;Kang, Soo Young;Cho, Eun Young;Yu, Kyung-Sang;Lee, Ji Won;Kang, Hyoung Jin;Park, Kyung Duk;Shin, Hee Young;Ahn, Hyo Seop;Lee, Hyunju;Choi, Eun Hwa;Lee, Hoan Jong
    • Pediatric Infection and Vaccine
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    • v.21 no.1
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    • pp.9-21
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    • 2014
  • Purpose: To determine the clinical significance of voriconazole therapeutic drug monitoring (TDM) in the pediatric population. Methods: Twenty-eight patients with invasive fungal infections administered with voriconazole from July 2010 to June 2012 were investigated retrospectively. Fourteen received TDM, and 143 trough concentrations were analyzed. All 28 patients were assessed for adverse events and treatment response six weeks into treatment, and at the end. Results: Out of 143 samples, 53.1% were within therapeutic range (1.0-5.5 mg/L). Patients administered with the same loading (6 mg/kg/dose) and maintenance (4 mg/kg/dose) dosages prior to initial TDM showed highly variable drug levels. Adverse events occurred in 9 of 14 patients (64.3%) in both the TDM and non-TDM group. In the TDM group, voriconazole-related encephalopathy (n=2, 14.3%) and aspartate aminotransferase (AST) or alanine aminotransferase (ALT) elevation (n=8, 57.1 %) occurred with serum levels in the toxic range (>5.5 mg/L), whereas blurred-vision (n=2, 14.3%) occurred within the therapeutic range (1.18 mg/L and 3.9 mg/L). The frequency of voriconazole discontinuation due to adverse events was lower in the TDM group (0.0% vs. 18.2%, P =0.481). Overall, 57.2% of the patients in the TDM group versus 14.3% in the non-TDM group showed clinical response after 6 weeks (P =0.055), whereas 21.4% in the TDM group versus 14.3% in the non-TDM group showed response at final outcome (P =0.664). In the TDM group, >67.0% of the serum levels were within therapeutic range for the first 6 weeks; however 45.5% were within therapeutic range for the entire duration. Conclusion: Routine TDM is recommended for optimizing the therapeutic effects of voriconazole.

Antihepatotoxic effect of ethanol extracts from steam-dried ginseng berry on ᴅ-galactosamine/lipopolysaccharide-sensitized mice (ᴅ-galactosamine/lipopolysaccharide로 감작된 급성간독성 마우스 모델에서 인삼열매추출물의 간독성 개선 효과)

  • Jang, Su Kil;Park, Jun Sub;Ahn, Jeong Won;Jo, Boram;Kim, Hyun Soo;Kim, JeongHoon;Kim, Sang Yun;Park, Jung Youl;Lee, Do Ik;Park, Hee Yong;Joo, Seong Soo
    • Korean Journal of Food Science and Technology
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    • v.49 no.6
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    • pp.676-684
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    • 2017
  • The present study aimed to examine the hepatoprotective effects of ethanol extracts from steam-dried ginseng berry (SGBE) in both $\text\tiny{D}$-Galactosamine/Lipopolysaccharide ($\text\tiny{D}$-GalN/LPS)-sensitized mice and in vitro models. Our results clearly demonstrated that SGBE significantly reduced the level of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and lactate dehydrogenase in blood, and $TNF{\alpha}$ was normalized in 8 h after the treatment with $\text\tiny{D}$-GalN/LPS. Coincidently, major organs remained unimpaired when compared to $\text\tiny{D}$-GalN/LPS control group. Moreover, p38, which stimulates expression of NAFLD-associated cytokines, was markedly inhibited when treated with SGBE. In vitro analysis revealed that the main components of SGBE, linoleic acid and ginsenoside Re/Rd, may play a role in protecting liver from $\text\tiny{D}$-GalN/LPS-induced toxicity. Finally, we concluded that SGBE may be a promising therapeutic agent for preventing damage to the liver.

Etiology and Clinical Features of Viral Lower-respiratory Tract Infections in Children in Winter, 2003 (2003년 겨울철 소아의 바이러스성 하기도 감염증에 대한 원인 및 바이러스별 임상양상)

  • Yun, Byung Ho;Lee, Hee Chul;Chun, Jung Mi;Yoon, So Young;Lee, Woo Gill;Shin, Son Moon
    • Clinical and Experimental Pediatrics
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    • v.48 no.7
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    • pp.723-730
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    • 2005
  • Purpose : This study was performed to characterize the etiology and clinical features of acute viral lower-respiratory tract infections(LRI). Methods : Etiologic agents and clinical features of acute viral LRI were studied from October. 2003 through March. 2004 in hospitalized children with LRI(253 cases) at Samsung Cheil Hospital. The viruses were identified by indirect immunofluorescent method. Medical records of patients with proven viral LRI were reviewed retrospectively. Results : Ninety two cases(36.4%) were confirmed as viral infections. The identified pathogens were respiratory syncytial virus(RSV, 76.0%), adenovirus(ADV, 12.0%), influenza virus type A(INFA, 9.8 %), influenza virus type B(INFB, 1.1%) and parainfluenza virus(PIV, 1.1%). Eight four point eight% of patients were younger than 2 years of age. Clinical diagnosis of LRI were pneumonia(56.5%), bronchiolitis(35.9%), tracheobronchitis(4.3%) and croup(3.3%). The clinical symptoms and signs were cough(98.8%), rhinorrhea(82.6%), fever(70.7%), rale(67.4%), wheezing(29.3%), chest retraction(28.3%) and cyanosis(4.3%). The severe respiratory symptoms and signs were more common in RSV-infected patients, even cyanosis could be observed. Seventeen point four percent of patient had fever of $38.5^{\circ}C$ or higher and their most common etiologic agent was INFA(66.7%). Twenty three point nine percent had fever more than 5 days and common etiologic agent was INFA(77.8%). The elevated WBC count($>14{\times}10^3/{\mu}L$) was in 14.1%, and common etiologic agents were INFA(22.2%) and ADV(18.2%). C-reactive protein(CRP >4.0 mg/dL) was increased in 13.0%, and common in ADV(63.6 %). Increased aspartate aminotransferase(AST)/alanine aminotransferase(ALT) was detected in 10.9%, and the most common etiologic agent was RSV(12.9%). Conclusion : The common agents of acute viral LRI were RSV, ADV and INF, respectively. Because the etiologic agents present variable clinical features, it may be helpful to treat and to evaluate acute viral LRI that we should understand their etiologic variability.

Effect of ${\alpha}$-Interferon Treatment on Serum ALT Levels in Patients with Chronic Hepatitis C (만성 C형 간염에 대한 ${\alpha}$-interferon 치료 후 ALT치의 변화)

  • Lee, Heon-Ju
    • Journal of Yeungnam Medical Science
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    • v.10 no.1
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    • pp.190-196
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    • 1993
  • The prognosis of chronic hepatitis C is very variable. In some, the disease is progressive and cirrhosis can develop from chronic hepatitis C. Hepatitis C virus(HCV) may act as a trigger towards hepatocellular carcinoma in patients with cirrhosis. Interferon has been used for the treatment of chronic hepatitis C in abroad, 16 patients with chronic C liver disease were treated with ${\alpha}$-interferon (alfa-2b; "Intron A" Schering Corp. Kenilworth. NJ). All patients were given ${\alpha}$-interferon in subcutaneous doses of 3 million units three times weekly for 1 to 9 months. During therapy, CBC and ALT levels were checked weakly to monthly. After therapy, patients were followed for 1 to 8 months. Among 16 patients treated with ${\alpha}$-interferon, progressive decrease of ALT levels was observed in 14(87.5%). In 11 patients(68.8%), ALT levels fell into the normal range during therapy, and in 9 of 11, within one month after therapy, 6 months after the completion of therapy in 4 of 9 patients(44.4%) whose ALT levels were in the normal range, ${\alpha}$-interferon seems to have effect in controlling disease activity in patients with chronic hepatitis C. But the changes in the usage of ${\alpha}$-interferon, dose and duration, long term follow up and more convenient and simple tests for HCV detection are recommended for the better effect and the exact evaluation on the effect of ${\alpha}$-interferon therapy in patients with chronic hepatitis C.

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Health status of children in low socioeconomic conditions (공부방을 이용하는 저소득층 소아들의 건강상태에 대한 조사)

  • Choi, Hee Kyoung;Her, Jeong A;Jang, Seong Hee;Kim, Dal Hyun;Yoon, Kyoung Lim;Ahn, Young Min
    • Clinical and Experimental Pediatrics
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    • v.49 no.1
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    • pp.24-28
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    • 2006
  • Purpose : The purpose of this study was to investigate the health status and nutritional condition of children living in a low-income community through anthropometric, laboratory evaluation. Methods : A community-based survey identified children below 15 years living in a low-income community. Their weight, height, visual acuity, hearing level and dental status were measured. Blood sample were obtained on June and July, 2004. Hemoglobin, serum cholesterol, Hepatitis B antigen/antibody, AST and ALT were measured. Results : A total of 285 students(M : F=141 : 144) aged 6 to 14 years were included in this study. The heights and weights in some grades were smaller than controls. The prevalence of obesity was 10.6 percent in males and 10.7 percent in females. The prevalence of abnormal visual acuity, hearing impairments and dental carries were 20.5 percent, 0.3 percent and 69.4 percent. The prevalence of anemia was 10.1 percent. Serum total cholesterol was over 200 mg/dL in 7 percent. They complained of abdominal pain(22.1 percent) and headache(17.1 percent). Hyperthyroidism, cataract, neurofibromatosis, severe atopic dermatitis, ventricular septal defect, strabismus and inguinal hernia were newly diagnosed. Conclusion : Mean heights and weights of children in the low-income community were smaller than controls. The prevalence of abnormal visual acuity, hearing impairment and dental carries were higher than in the 2003 national health survey. Additional research is needed to evaluate the health status of the low-income community.

Clninical Features of Rotaviral Gastroenteritis in Neonates (신생아에서 로타바이러스 위장관염의 임상양상에 대한 고찰)

  • Park, Shin-I;Kwon, Hae Oak;Lee, Jun Ho;Jung, Su Jin
    • Clinical and Experimental Pediatrics
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    • v.48 no.10
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    • pp.1121-1125
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    • 2005
  • Purpose : Rotavirus is the main cause of infantile diarrheal disease worldwide, especially in patients 3-24 months of age. Infants younger than 3 months of age are relatively protected by transplacental antibody. So the purpose of this study is to evaluate the clinical features and severity of neonatal rotaviral gastroenteritis less than 1 month of age. Methods : A retrospective chart review was established of 62 neonates less than 1 month of age and with a diagnosis of rotaviral gastroenteritis who had been admitted to Pochon CHA University between June 2002 through July 2004. The rotavirus was examined by stool latex agglutination. Results : During 2 years, the total number of admitted patients for rotaviral gastroenteritis was 688 and among these, less than 1 month of age accounted for 9%(62). The occurrence was generally even distribution from January to July($7.14{\pm}1.0$) but since then decreased($2.4{\pm}1.8$). The most common chief complaint was mild fever(46%) when admitted which subsided within 1 hospital day in most patients. 4 patients had seizure and cyanosis with no typical symptoms of rotaviral gastroenteritis. During admission, all the patients had diarrhea. 17% of the patients had leukocytosis and positive C-reactive protein. In one patient, stool occult blood test was positive but there was no necrotizing gastroenteritis evidence. The mean period of hospital day was $5.8{\pm}2.5$ and breast-milk feeding was 62.9%. Conclusion : Neonatal rotaviral gastroenteritis is not a rare disease. Most patients have fever and diarrhea and improve through conservative therapy but a few patients may have severe complications so we must be more cautious about the hygiene for prevention.

Low T3 syndrome in Kawasaki disease: Relation to serum levels of tumor necrosis factor-α, interleukin-6 and NT-proBNP (가와사끼병에서의 저 T3 증후군 : 혈청 tumor necrosis factor-α, interleukin-6 및 NT-proBNP 농도와의 관계)

  • Cho, Hye Kyung;Sohn, Jin A;Kim, Hae Soon;Sohn, Sejung
    • 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.

Use of herbal medicine in epileptic children (소아 간질 환자에서 한약 복용 실태)

  • Lee, Jee Yeon;Choi, Wook Sun;Eun, So-Hee;Eun, Baik-Lin;Hong, Young Sook;Lee, Joo Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.4
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    • pp.415-419
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    • 2008
  • Purpose : Herbal medicine is thought to be widely used by children with epilepsy, but there have been few studies. This study aimed to investigate the perceptions and the actual conditions of usage of herbal medicine by children with epilepsy. Methods : From July to August 2007, three hundred seventy eight questionnaires were completed by parents of epileptic children who visited pediatric neurology clinic of Korea University hospital and Kwangmyung-Sungae hospital. Demographic data and patterns of usage of herbal medicine were investigated. Results : Among 378 patients, sixty five (17.2%) reported using herbal medicine. Major reasons for using herbal medicine were 'to enhance general health' and 'belief that it has fewer side effects'. Majority of respondents did not notify their physician about using herbal medicine because 'they did not need to share this with doctor' and 'they thought their doctor would disapprove'. After taking herbal medicine, 33.3% of patients reported that it did not benefit their seizure but they felt healthier. Usage of herbal medicine was significantly related to number of AEDs (antiepileptic drugs), duration of AED treatment, association with psychosomatic disorders and motivation by other people or by mass media. Conclusion : Considerable numbers of epileptic children were using herbal medicine during treatment with AED and did not inform their physician about usage of it. Physicians should be aware of benefits and harms of herbal medicine and actively intervene in the usage of herbal medicine by epileptic patients.

Effect of Fermented (Oenanthe stolonifera DC) Extract on the Activity of Enzymes Related to Liver Function of Alcohol-administered Rats and Mice (밭미나리 발효액이 알콜투여 흰쥐의 간기능관련효소활성에 미치는 영향)

  • Whang, Tay-Eak;Lim, Hyun-Ock;Lee, Jae-Wa
    • Korean Journal of Medicinal Crop Science
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    • v.7 no.2
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    • pp.107-114
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    • 1999
  • It has long been recognized that dropwort contains specific funtional subtances for protecting human liver and preventing and curing its diseases, and thus it has been widely utilized in traditional folk remedy. In the present study. fresh of fermented extract of dropwort shoots grown on dryland and fresh extract of those grown on flooded fie1d were fed to the rats suffering from acute, subacute or chronical toxication induced by alcohol administration, and their affects were investigated. Administration of alcohol to rats and mice for 2 days at 5ml of 30% EtOH/kg/day raised total cholesterol and total glyceride which were, however, great1y supressed when alcohol was administered to the laboratory animals previously fed on fresh or fermented extract of dryland dropwort, or fresh extract of flooded field-grown dropwort for 20 days, without significant differences among the extracts. The levels of alanine aminotransferase and aspartate aminotransferase which were raised by alcohol adminstration were also lowered by feeding dropwort extract, among which that of fermented dryland-grown one was more effective than the other two. Chronic alcohol toxication was induced to rats by administering 10% alcohol for 10 months and fermented dropwort extract or tap water was fed to the rats for 5 days. The rats fed on fermented dropwort extract were lower in total cholesterol by 40% and in tota1 glyceride by 60% than the control. Alanine aminotransferase and aspartate aminotransferase in the rats fed on fermented dropwort extract were decreased by 87.2% and 91.7%, respectively, compared to the control, and the rats recovered almost to normal. Activity of alkaline phosphatase, catalase, superoxide dismutase or glutathione peroxidase changed greatly by alcohol administration in the rats suffering from chronic as well as acute toxication. The extract of fermented dry land dropwort significantly lowed the activity of those enzymes, especially, alkaline phosphatase and superoxide dismutase. The present results suggesting the possible medicinal effect of fermented dropwort extract to liver diseases.

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