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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 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.
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.
남궁미경,NamGoong, Mee-Kyung 대한소아신장학회 2011 Childhood kidney diseases Vol.15 No.2
급성신부전은 그 단어 의미에 약한 신장 손상을 포함하지 못한다. 또한 급성신부전에 대한 기준이 연구자마다 달라서 연구결과 해석상에 어려움을 주었다. 따라서 급성신부전보다는, 급성신손상이란 용어가, 신손상의 모든 정도를 포함하는 의미로 사용될 수 있다. 2002년에, 급성신손상을 자세히 분류하고 진단하기위해, 혈청크레아티닌, 사구체 여과율과 요량을 기준으로 하여, RIFLE 기준이 제안되었다. 2007년에는, RIFLE 기준을 변형하여, AKIN 기준이 제안되었다. 소아를 위해 소아-RIFLE 기준도 제안되었다. 저자는 여기서 이들 기준과 각각에 대한 비교를 기술하였다. 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.
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.