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Gitelman 증후군 환자에서 면역조직화학법으로 확인한 원위세관 sodium-chloride cotransporter (NCCT)의 결손
장혜련 ( Hye Ryoun Jang ),허남주 ( Nam Ju Heo ),손민정 ( Min Jung Son ),이재욱 ( Jay Wook Lee ),이정환 ( Jeong Hwan Lee ),전은실 ( Un Sil Jeon ),신성준 ( Sung Jun Shin ),나기영 ( Ki Young Na ),주권욱 ( Kwon Wook Joo ),이정상 ( Jun 대한내과학회 2005 대한내과학회지 Vol.69 No.6
목적 : Gitelman 증후군은 저포타시움혈증, 대사성 알칼리증, 고레닌혈증, 고알도스테론혈증, 요 중 칼슘 배설의 저하 및 저마그네슘혈증을 특징으로 하는 유전성 질환이다. 이는 원위세관 sodium-chloride cotransporter (NCCT)의 유전자 돌연변이에 의하여 발생하는 것으로 알려져 있으나 사람의 신조직에서 NCCT 결손이 증명된 바는 없었다. 방법 : 저자들은 임상적으로 Gitelman 증후군이 의심되는 환자에서 이뇨제를 이용한 신청소율 검사와 유전자 검사를 시행하였고, 이를 통하여 감별진단한 Gitelman 증후군 환자의 신조직에서 인간 NCCT에 대한 토끼 다클론 항체를 이용한 면역조직화학법을 시행하였다. 신세포암으로 신적출술을 시행 받은 환자의 정상 신조직과 전해질 이상이 없었던 사구체신염 환자의 신조직을 각각 정상 대조군과 질환 대조군으로 하였다. 결과 : 대상 환자는 저포타시움혈증과 대사성 알칼리증, 저마그네슘혈증 및 요 중 칼슘 배설의 저하를 보였다. Bartter 증후군과 감별을 위하여 furosemide 및 hydrochlorothiazide를 이용한 신청소율 검사를 시행하였다. 수분 부하를 시행한 기저치(86.1%)에 비해서 furosemide를 투여한 후 distal fractional chloride reabsorption이 감소하였으나(9.7%) hydrochlorothiazide 투여 후에는 변화가 없었다(81.4%). 유전자 검사 결과 SLC12A3 유전자의 돌연변이(S967F)가 발견되었다. 신조직에서 면역조직화학법을 시행한 결과 정상 및 질환 대조군에서는 원위세관 세포의 내강 막 쪽에 NCCT가 뚜렷이 염색되었으나, Gitelman 증후군에서는 원위세관 세포의 NCCT에 대한 면역 반응성이 관찰되지 않았다. 반면에 Na/K-ATPase, Na-K-2Cl cotransporter, calbindin-D28K는 대조군과 대상 환자의 신조직에서 모두 관찰되었다. 결론 : 기능적 검사로 진단된 Gitelman 증후군 환자의 신조직에서 NCCT의 결함을 면역조직화학법으로 확인하였다. Background : Gitelman`s syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. It is known to be caused by a mutation of SLC12A3 gene coding the sodium-chloride cotransporter (NCCT) in the distal tubule. The defect of NCCT in human renal tissues has not been investigated, and we tested whether the defect of NCCT can be detected in renal tissue of a patient with Gitelman`s syndrome by using immunohistochemistry. Methods : In an adult patient with Gitelman`s syndrome, blood and urine samples were collected for measurement of biochemical parameters. Renal clearance study and gene analysis were performed. Immunohistochemistry was performed on the renal tissue of the patient using a rabbit polyclonal antibody directed against a synthetic peptide corresponding to a portion in the amino terminal tail for human NCCT. Normal human renal tissues from surgical nephrectomy due to renal cell carcinoma and renal biopsy tissues from patients with glomerulonephritis but without any electrolyte disturbance were used as controls. Results : The patient had hypokalemic metabolic alkalosis, hypocalciuria and hypomagnesemia. Renal clearance study revealed a decrease in distal fractional chloride reabsorption after the administration of furosemide. SLC12A3 gene mutation (S967F) was found by direct sequencing method. Immunohistochemistry showed the absence of NCCT staining in the renal tissue of the patient. On the other hand, the immunostaining of other transporters was all positive in renal tissues from both Gitelman`s syndrome patients and controls. Conclusions : We report the absence of intact NCCT in the renal tissue of a Gitelman`s syndrome patient.(Korean J Med 69:642-650, 2005)
증례 : 광방기에 의해 발생한 Chinese Herb Nephropathy 1예
이재욱 ( Jay Wook Lee ),손민정 ( Min Jung Sohn ),허남주 ( Nam Joo Heo ),주권욱 ( Kwon Wook Joo ),정윤철 ( Yoon Chul Jung ),이정상 ( Jung Sang Lee ),한진석 ( Jin Suk Han ) 대한내과학회 2006 대한내과학회지 Vol.71 No.2
본 증례의 환자는 근위부 신세뇨관 산증, 진행성 만성 신부전증, 세뇨관 위축 및 간질 섬유화 등 CHN에 잘 맞는 임상 증상 및 조직 소견을 보였고, 복용하던 한약재에서 AA가 검출되어 AA에 의한 CHN 또는 AAN으로 진단할 수 있었다. 이러한 환자의 신조직에서 AA-DNA adduct를 추가로 검출할 수 있다면 진단의 정확성을 더욱 높일 수 있을 것으로 보인다. Chinese herb nephropathy (CHN) is characterized by progressive tubulointerstitial nephritis and development of renal failure in a couple of years after diagnosis. Aristolochic acid (AA) is believed to be associated with the development of CHN. The authors report a case of CHN in which AA in the herb regimen was identified by high-performance liquid chromatography (HPLC). A 32-year-old female presented with nausea, vomiting and generalized weakness. She had been taking Chinese herbs for symptomatic care. Clinical and laboratory examinations revealed Fanconi syndrome, renal failure, and severe anemia. Renal biopsy showed severe tubulointerstitial nephritis with moderate tubular atrophy and interstitial fibrosis. She developed end-stage renal failure 4 months after diagnosis. The herb she had been taking was Aristolochia fangchi. HPLC technique was used to identify AA and to measure its concentration in the herb. From the clinical and laboratory data, the patient was diagnosed with CHN caused by aristolochic acid.(Korean J Med 71:224-228, 2006)
기능성식품의 구매 및 섭취 실태와 아로니아 첨가식품 개발에 대한 소비자의 인식
박민규, 주신윤, 허남주, 이홍미 충북대학교 생활과학연구소 2008 생활과학연구논총 Vol.24 No.2
This study was conducted to provide the basic information in order to develop the aronia added functional foods by determining the consumers’s needs and status of consuming functional foods and those with aronia. The subjects were 43 men and 142 women over 20 years of age who had regular income residing Seoul and Gyeonggi area. The information were obtained by using self-administered questionnaire and data were analysed by SPSS 25.0. The proportion of the subjects who had experienced eating aronia and aronia added foods were only 64.9% and 35.1%, suggesting insufficient supply to market yet as of 2019. The subjects evaluated the importance of 8 factors when they purchase the functional foods; physiological active function and easiness to consume the highest (4.19, respectively); patent or certification (4.03), price (3.79), recommendation (3.65), brand (3.61), advertisement or broadcasting (2.75), and design (2.64). More over, ‘physiological function’ was evaluated higher by women, by those graduated with food and nutrition major, by those with more concern of their own health and by those who buy functional foods for their own or family members’ health; ‘the easiness to consume’ by the subjects who had experience of purchasing functional foods; ‘price’ by younger subjects under 30 years of age; ‘brand’ by those with experience of buying the functional foods than by their counterparts, respectively (p<0.05). The most answered reason for dissatisfaction with the foods with aronia was ‘unsatisfactory taste’ (52.4%) and followed by ‘unsatisfactory effectiveness’ (33.3%) and the most wanted type of foods with aronia was beverage (41.8%) followed by jam (26.9%), jelly (11.5%), bakery (10.4%), and sauce such as gochujang (7.1%), which should be reflected in developing new functional foods with aronia.