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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)
Ji Na Jung,Kweon Heo,Myong Jo Kim,Ju Kyong Lee 한국육종학회 2008 한국육종학회지 Vol.40 No.4
In order to better understand the morphological differentiation of the two cultivated types of Perilla crop and their weedy types in Korea and Japan, we studied the variation of 62 accessions by examining 15 morphological characteristics. By using ANOVA (one-way analysis of variance), we determined that var. frutescens and var. crispa showed significant morphological differences in terms of plant height and seed weight. Furthermore, cultivated var. frutescens and var. crispa could also be clearly discriminated from one another using PCA (principal component analysis). Specifically, quantitative and qualitative characteristics such as plant height, seed weight, degree of pubescence, shape of leaf, color of leaf, fragrance of plant, color of flower, color of stem and seed size greatly contributed to differences seen in the positive and negative direction on the first axis. In our study, most accessions of cultivated var. frutescens and those of its weedy type could be clearly discriminated from one another, however, most accessions of cultivated and weedy types of var. crispa were not clearly discriminated by the ANOVA and PCA analyses. These results indicated that cultivated var. frutescens can be considered to be a domesticated form, while the cultivated var. crispa can not be considered to be a domesticated form in Korea and Japan. It is our belief that our results concerning the morphological variations among cultivated types of Perilla crop and their weedy types in Korea and Japan will help ensure the long-term success of breeding programs and maximize the use of the germplasm resources in Korea.
Clinical Outcomes in Patients with Asthma and Bronchiectasis
( Na Young Kim ),( Chang Hoon Lee ),( Kwang Nam Jin ),( Hyun Woo Lee ),( Seo-young Yoon ),( Tae Yun Park ),( Eun Young Heo ),( Deog Kyeom Kim ),( Hee Soon Chung ),( Jung Kyu Lee ) 대한결핵 및 호흡기학회 2020 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.128 No.-
Background Bronchiectasis and asthma share many clinical features and the overlap between two diseases may Result in more severe disease. We aimed to assess the clinical outcomes according to bronchiectasis in patients with asthma. Methods A retrospective study was conducted from January 2013 to December 2019 on patients with asthma who received chest computed tomography and pulmonary function tests. We evaluated radiologic findings, history of acute exacerbations, and longitudinal changes of lung function. Results Among 586 patients with asthma, there were 221 patients (37.7%) with bronchiectasis, who had lower lung functions and higher annual incidence of acute exacerbations than those without bronchiectasis. The existence of bronchiectasis was significantly higher risk of severe and moderate-to-severe acute exacerbations (aOR 1.74, P = 0.020; aOR 1.50, P = 0.027). Bronchiectasis severity measured by semi-quantitative bronchiectasis score significantly predicted the risk of development of severe and moderate-to-severe acute exacerbation (AUC 0.60, P = 0.033; AUC 0.55, P = 0.024). The existence and progression of bronchiectasis was not associated with the annual decline of lung function. Conclusion In patients with asthma, the existence and severity of bronchiectasis are associated with increased risk of severe and moderate-to-severe acute exacerbations.