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Management of IgA vasculitis nephritis (Henoch- Schonlein purpura nephritis) in Children
남궁미경 대한소아신장학회 2020 Childhood kidney diseases Vol.24 No.1
Immunoglobulin (Ig)A vasculitis nephritis (IgAVN), also referred to as Henoch- Schönlein purpura nephritis, is a relatively benign disease in children. However, two 24-year European cohort studies have reported high sustained rates of hypertension, severe proteinuria, and renal dysfunction in patients with IgAVN. Notably, the incidence and exacerbation rates of proteinuria, hypertension, and renal dysfunction during pregnancy were high even in women who recovered from IgAVN before pregnancy. Patients with IgAVN need lifelong care. Trials have been performed to investigate early biomarkers and genes associated with poor prognosis to identify high-risk patients in whom IgAVN may progress to severe renal disease. Urinary IgA/cr, IgM/cr levels, and HLAB35 and angiotensinogen gene expression were shown to be predictors of progression of IgAVN to severe renal dysfunction. The 2019 Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative group published guidelines for pediatric IgAVN, following the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines established in 2012. Compared with the KDIGO guidelines, the SHARE guidelines recommend earlier corticosteroid administration in cases of mild proteinuria (>0.5 g/d). Clinical trials of targeted budesonide delivery to the distal ileum, monoclonal antibody targeting C5, eculizumab and anti-CD20 monoclonal antibody administration, among others are currently underway in patients with IgA nephropathy. It is expected that newer therapeutic agents would become available for IgAVN in the near future. This review summarizes IgAVN with emphasis on recently published literature, including possible preventive strategies, predictive biomarkers for progression of IgAVN, and various treatments.
신증후군에서 스테로이드 반응성과 재발할 환자를 예측할 수 있을까?
남궁미경 대한소아청소년과학회 2005 Clinical and Experimental Pediatrics (CEP) Vol.48 No.10
Pediatric nephrotic syndrome is a well-known illness for its high relapsing rate. If we can predict the relapsing rate and the responses to the steroid therapy of individual patients with nephrotic syndrome, the predictability will be helpful in building a therapeutic plan. Here is my review of research articles on the risk factors for the prediction of relapsing nephrotic syndrome.
남궁미경,NamGoong, Mee-Kyung 대한소아신장학회 2011 Childhood kidney diseases Vol.15 No.2
Acute renal failure means that the word does not contain a mild kidney injury. In addition, the criteria for acute renal failure per researcher are different, and it is difficult in interpreting the results of research on acute renal failure. Therefore, rather than acute renal failure, a new term "acute kidney injury" meaning to include all the levels of injury is introduced. In 2002, to diagnose by means of serum creatinine, glomerular filtration rate and urine output, a detailed classification of acute kidney injury, the RIFLE criteria has been proposed. In 2007, the RIFLE criteria by transforming, AKIN criteria has been proposed. The pediatric RIFLE criteria for children has also been proposed. The author reviews here these criteria by comparing them.
남궁미경,NamGoong, Mee-Kyung 대한소아신장학회 2007 Childhood kidney diseases Vol.11 No.1
Many children with microscopic hematuria have been found on school screening examinations. There are not, however, nation-wide criteria for us(specifically, pediatric nephrologists) how to take care of them. Recently, quite a few research papers concerning microalbuminuria with microscopic hematuria, which can predict the renal pathological findings, are published. Here I have reviewed articles on microalbuminuria which gives us the information how to manage microscopic hematuria.
남궁미경(MK Namgoong),윤영옥(YO Yoon),남숙현(SH Nam),홍승길(SK Hong),나중열(JW Rha) 대한산부인과학회 1993 Obstetrics & Gynecology Science Vol.36 No.9
Many parturients and patients who have uterine diseases suffer form low back pain coin-cidently Whether the contraction of the longissimus muscle can be evoked by uterine pain is the purpose of this study. Recordings have been made from 34single efferent nerve fibers of longissimus muscle at L level of the a-chloralose anesthetized cat. The obtained results are summerized as follows; 1. Impulse discharges of 34 muscular efferents were recorded. Among them 21 fibers were a-motor fibers and 13 fibers were y-motor fibers 2. 21 a-motor fibers revealed the increased impules dischasrges to the injected brady-kinin adn potassium chloride into rterus. 3. 13 y-motor fibers had increased responses to intra-arterially injected bradykinin adn potassium chloride into uterus 4. The excitation response of bradykinin had logner duration and less small peak discharge in comparison with that of potassium chlorde. These results suggest that the signal of the uterine pain which was transmitted to the spinal cord gy way of the hypogastric nerve activated the back muscle efferents which elicited the contraction of the back muscle. This reflexive responses may explain the back pain by which uterine pain is accompanied.
이도윤,남궁미경,김황민,임백근,Lee Do-Yoon,NamGoong Mee-Kyung,Kim Hwang-Min,Lim Baek-Keun 대한소아신장학회 1997 Childhood kidney diseases Vol.1 No.2
Cyclosporine is an immunosuppressant usually used to prevent renal transplantation rejection. Nephrotoxicity and hypertension are considered as the most frequent side effects of cyclosporine treatment. The neurotoxic effects of cyclosporine such as agitation, anxiety, delirium, depression and psychosis have recently been found. Methylprednisolone may increase as well plasma concentration of cyclosporine, which leads to side effects. Here we report a $Henoch-Sch\"{o}nlein$ nephritis patient treated with cyclosporine and methylprednisolone who has experienced psychosis including visual and auditory hallucination and convulsion.
신생검에서 급성 사구체신염 양상을 보인 Henoch-$Sch{\ddot{o}}nlein$ 자반증 2례
이상흔,남궁미경,차병호,김종수,Lee Sang Heun,Namgoong Mee Kyung,Cha Byung Ho,Kim Jong Soo 대한소아신장학회 1998 Childhood kidney diseases Vol.2 No.2
[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.
폐 동정맥 기형과 비장 동정맥 기형을 동반한 유전 출혈 모세혈관 확장증 1례
김미현,남궁미경,김영주,정순희,어영,이해용 대한 소아알레르기 호흡기학회 2012 Allergy Asthma & Respiratory Disease Vol.22 No.4
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a rare autosomal dominant vascular disorder involving arteriovenous malformation (AVM) due to angiodysplasia. HHT is characterized by mucocutaneous hemorrhagic telangiectasia and AVM of various different organs. The mutated genes in HHT encode proteins that mediate signaling by the transforming growth factor-β superfamily, leading to migration and proliferation of endothelial cells to finally result in AVMs of various organs. Amongst various affected organs, pulmonary AVM is the most common. This 11-year-old female patient with a past and familial history of frequent epistaxis visited the hospital for abdominal pain and vomiting which developed on the same day. Nodular opacity was found in chest X-ray. Subsequently, AVMs in the lungs and the spleen were recognized in chest computed tomography along with detection of ENG gene mutation. Here we report a case of hereditary hemorrhagic telangiectasia diagnosed by incidental chest X-ray findings followed by genetic and radiologic studies. 유전 출혈 모세혈관 확장증은 혈관 생성 유전자의 이상에 의해 나타나는 상염색체 우성 질환으로, 국내에서는 산발적으로 보고되고 있다. 저자들은 평소 비출혈이 있으며 우연히 발견된 폐 결절이 있는 환아에서 영상 소견과 유전자 검사로 폐 및 비장 동정맥 기형을 동반한 유전 출혈 모세혈관 확장증을 진단하고 치료하였기에 보고하는 바이다.