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성인에서 발생한 보체 H 인자 돌연변이에 의한 가족성 비전형적 용혈성 요독 증후군
이현기 ( Hyun Kee Lee ),나희경 ( Hee Kyung Na ),이지영 ( Ji Young Lee ),장재원 ( Jai Won Chang ),양원석 ( Won Seok Yang ),김순배 ( Soon Bae Kim ),박정식 ( Jung Sik Park ),박수길 ( Su Kil Park ),권순길 ( Soon Kil Kwon ),정해일 ( Ha 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.3
The hemolytic uremic syndrome is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure. Atypical hemolytic uremic syndrome (aHUS) which is not usually associated with prodromal symptoms, especially diarrhea, has a higher mortality rate and a stronger tendency to progress to chronic renal failure. In approximately 30-50% of patients with aHUS, mutations have been detected in complement factor H, membrane cofactor protein or factor I. Mutations in the complement regulator factor H are the most frequent and have a very poor prognosis, with most patients developing ESRD. We have experienced a 33-year-old man with a family history of renal failure diagnosed as aHUS resulted from factor H mutation, for whom we carried out hemodialysis, plasmapheresis and other conservative management.
조정민 ( Jung Min Jo ),신미선 ( Mi Seon Shin ),김주희 ( Joo Hui Kim ),김민정 ( Min Jung Kim ),박현정 ( Hyun Jung Park ),나희경 ( Hee Kyung Na ),조경욱 ( Kyung Uk Jo ),김재중 ( Jae Joong Kim ),심태선 ( Tae Sun Shim ) 대한결핵 및 호흡기학회 2010 Tuberculosis and Respiratory Diseases Vol.69 No.3
Nontuberculous mycobacterial (NTM) diseases are increasing worldwide. However NTM lung disease in organ transplant recipients has been rarely reported. Here, we report 2 cases of NTM lung disease in heart transplant recipients. A 37-year-old man, who had undergone a heart transplant one year previous, was admitted to hospital due to a cough. Chest CT scan showed multiple centrilobular nodules in both lower lungs. In his sputum, M. abscessus was repeatedly identified by rpoB gene analysis. The patient improved after treatment with clarithromycin, imipenem, and amikacin. An additional patient, a 53-year-old woman who had undergone a heart transplant 4 years prior and who suffered from bronchiectasis, was admitted because of purulent sputum. The patient`s chest CT scan revealed aggravated bronchiectasis; M. intracellulare was isolated repeatedly in her sputum. Treatment was successfully completed with clarithromycin, ethambutol, and ciprofloxacin. NTM lung disease should be considered as a potential opportunistic infection in organ transplant recipients.