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- 저자 : Si-Hoon Hahn (검색결과 16 건)
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박시훈(Si Hoon Park),이선주(Sun Ju Lee),한지숙(Jee Sook Hahn),고윤웅(Yun Woong Ko),김병수(Byung Soo Kim) 대한내과학회 1987 대한내과학회지 Vol.32 No.6
N/A The evaluation of bone marrow metastasis in cancer patients is essential for staging and establishing the treatment streategies. Hower, the bone marrow examination is generally not performed in recently diagnosed cancer patients unless they have obvious hematologic abnormalities, Though a number of parameters have been reported to correlate with bone marrow metastasis, no single clinical parameter has been considered to be highly predictive of it. The present study was designed to evaluate the parameters and to establish the criteria which best met the bone marrow metastasis, retrospectively in 60 cancer patients with bone marrow metastasis(subject group) and 41 without it (control group), underwent bone marrow aspiration and/or biopsy from Jan. 1977 to Dec. 1985. Stepwise logistic regression analysis was employed using a statistical package program, BMDPLR, at the younsei University Computer Center. Univariate anaylsis showed 11 significant parameters as follows; bone pain, nucleated RBC, fragmented RBC, immature WBC, platelet count, alkaline phosphatase (ALP), total bilirubin, SGOT, LDH, and uric acid. Stepwise logistic regression analysis revealed the most significant 4 variables as follows; bone pain, ALP>115 IU/L, platelet<100,000/mm³, and fragmented RBC. Then two sets of decision rule judging a patient to have bone marrow metastasis were introduced. Decision rule I: the calculated predicted probability greater than 0.426, by the equation Pr(M)=1-[1+exp {1.050 (bone pain)+2.594 (fragmented RRC)+1.115 (platelet<100,000/mm³)+1.411 (ALP) 115 IU/L)}], where positive and negative result of the each variable corresponds the value +1 and -1, respectively. Decision rule II for the convenience of clinicians; at least 2 out of 4 major correlates, or 1 major correlates and at least 2 out of 5 minor correlates, where major correlates are the above 5 variables of the most significance and minor correlates are immature WBC, nucleated RBC, LDH > 125IU/L, SGOT>30IU/L and hematocrit<30%. The sensitivity and specificity of the decision rule I and II are 90.0%, 85.7% and 92.5%, 70.6%, respectively. In conclusion, the above rules should aid the physicians in staging and establishing the treatment strategy for cancer patients with high probabilty of bone marrow metastasis.
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Sim, Se-Hoon,Yeom, Ji-Hyun,Shin, Choy,Song, Woo-Seok,Shin, Eunkyoung,Kim, Hong-Man,Cha, Chang-Jun,Han, Seung Hyun,Ha, Nam-Chul,Kim, Si Wouk,Hahn, Yoonsoo,Bae, Jeehyeon,Lee, Kangseok Blackwell Publishing Ltd 2010 Molecular microbiology Vol.75 No.2
<P>Summary</P><P>During the course of experiments aimed at identifying genes with ribonuclease III (RNase III)-dependent expression in <I>Escherichia coli</I>, we found that steady state levels of <I>bdm</I> mRNA were dependent on cellular concentrations of RNase III. The half-lives of adventitiously overexpressed <I>bdm</I> mRNA and the activities of a transcriptional <I>bdm‘–’cat</I> fusion were observed to be dependent on cellular concentrations of RNase III, indicating the existence of <I>cis</I>-acting elements in <I>bdm</I> mRNA responsive to RNase III. <I>In vitro</I> and <I>in vivo</I> cleavage analyses of <I>bdm</I> mRNA identified two RNase III cleavage motifs, one in the 5′-untranslated region and the other in the coding region of <I>bdm</I> mRNA, and indicated that RNase III cleavages in the coding region constitute a rate-determining step for <I>bdm</I> mRNA degradation. We also discovered that downregulation of the ribonucleolytic activity of RNase III is required for the sustained elevation of RcsB-induced <I>bdm</I> mRNA levels during osmotic stress and that cells overexpressing <I>bdm</I> form biofilms more efficiently. These findings indicate that the Rcs signalling system has an additional regulatory pathway that functions to modulate <I>bdm</I> expression and consequently, adapt <I>E. coli</I> cells to osmotic stress.</P>
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발달 지연으로 내원한 Cystathionine 뇨증 1례
변소훈(So Hoon Byun),이희선(Hee Sun Lee),피대훈(Dae Hun Pee),이기형(Kee Hyoung Lee),은백린(Baik-Lin Eun),한시훈(Si Houn Hahn) 대한소아신경학회 2003 대한소아신경학회지 Vol.11 No.1
γ-cystathionase 결핍으로 인해 생긴 cystathioninuria는 상염색체 열성 유전질환으로, 다양한 임상양상을 보이며, 이 질환에 특징적으로 연관된 임상양상은 없는 것으로 추측되며, 다수에서 pyridoxine 경구 투여에 의해 호전된다. 저자들은 발달 지연을 주소로 내원하여 소변 아미노산 분석 검사 결과 시스틴뇨증으로 진단되고 pyridoxine 경구 투여로 호전된 1례를 경험하고, 우리나라에서 첫 증례라고 사료되어 이에 문헌 고찰과 함께 보고하는 바이다. Cystathionine is well-known intermediate in the metabolism of methionine. It is cleaved to cysteine and homoserine by γ-cystathionase. This enzyme utilize pyridoxal 5'-phosphate as coenzyme. γ-cystathionase deficiency leads to persistent excretion of large amount of cystathionine in urine, as well as to accumulation of cystathionine in body tissues and fluids. It is inherited as an autosomal recessive trait and shows wide variety of clinical manifestations. No clinical abnormality seems to be specifically associated with γ-cystathionase deficiency. The majority of patients responded to high dose administration of pyridoxine. We report the first case of cystathioninuric patient in Korea, 19 months of female with developmental delay. In brain MRI, there was generalized mild brain atrophy. There were several times of brief paroxysmal generalized polyspike and wave discharges in electroencephalography(EEG). In amino acid analysis of urine, there was elevated level of cystathionine. She was treated with high dose of pyridoxine. In follow up analysis of urinary amino acid, the cystathionine level was markedly decreased to normal range, and EEG was normalized. Her development shows improvement.
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Successful treatment of a child with citrullinemia
Lee, Key-Hyoung,Park, Moon-Sung,Hahn, Si-Hoon Korean Society of Medical Genetics 1997 대한의학유전학회지 Vol.1 No.1
The amino acids formed by degradation of proteins ingested produce ammonia. The ammonia which is broken down and excreted as urea through a process known as the Klebs-Hensleit cycle or the urea cycle (Rezvani, 1995). The urea cycle consists of five enzymes necessary for the synthesis of carbamyl phosphate, citrulline, argininosuccinate, arginine, and urea: carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinate synthetase (AS), argininosuccinate lyase (AL), and arginase (ARG) (Lloyd, 1992). Congenital deficiencies of the enzymes involved in the urea cycle are diseases that are almost fatal without treatment, showing symptoms like vomiting, lethargy, dyspnea, and coma due to hyperammonemia coming from the accumulation of ammonia and metabolic precursors resulting from the deficiency of one of these enzymes (Batshaw and Brusilow, 1983). Among these, the disease manifested by the congenital deficiency of argininosuccinate synthetase (AS) which is associated with the formation of argininosuccinate in citrulline is called argininosuccinate synthetase deficiency or citrullinemia. There have been two reports on this so far in Korea; one in July 1987 by Kim et al. and the other by Park et al. in 1995. We are to report a case of successful treatment of a child with citrullinemia who was transferred to our hospital due to dyspnea, lethargy, feeding difficulties, convulsions and cyanosis together with some document studies related to this case.
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Successful treatment of a child with citrullinemia
Key-Hyoung Lee,Moon-Sung Park,Si-Hoon Hahn 대한의학유전학회 1997 대한의학유전학회지 Vol.1 No.1
The amino acids formed by degradation of proteins ingested produce ammonia. The ammonia which is broken down end excreted as urea through a process known as the Kiebs-Hensleit cycle or the urea cycle (Rezvani, 1995). The urea cycle consists of five enzymes necessary for the synthesis of carbamyl phosphate, citrulline, argininosuccinate, arginine, and urea: carbomyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinate synthetase (AS), argininosuccinate lyase (AL), and arginase (ARG) (Lloyd, 1992). Congenital deficiencies of the enzymes involved in the urea cycle are diseases that are almost fatal without treatment, showing symptoms like vomiting, lethargy, dyspnea, and coma due to hyperammonemia corning from the accumulation of ammonia and metabolic precursors resulting from the deficiency of one of these enzymes (Batshaw and Brusilow, 1983). Among these, the disease manifested by the congenital deficiency of argininosuccinate synthetase (AS) which is associated with the formation of argininosuccinate in citrulline is called argininosuccinate synthetase deficiency or citrullinemia. There have been two reports on this so for in Korea; one in July 1987 by Kim et al. and the other by Park et al. in 1995. We are to report a case of successful treatment of a child with citrullinemia who was transferred to our hospital due to dyspnea, lethargy, feeding difficulties, convulsions and cyanosis together with some document studies related to this case.
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