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답은 MMPH 입니다.
Multifocal micronodularpneumocyte hyperplasia
환자분은 TSC2 gene (+) 보였으나
그외 tuberous sclerosis complex 의
clinical triad of seizures, mental retardation and skin lesions 는 보이지
않았습니다.
further evaluation 위해 서울대 병원 임상유전학과로
transfer 한 상태입니다.
As pulmonary manifestation, lymphangioleiomyomatosis(LAM) and Multifocal micronodularpneumocyte hyperplasia (MMPH) have been reported less than 2 percent of TSC . The majority of patients with MMPH in TSC are younger or middle-aged women co-exsited with LAM , but there was no evidence of LAM in our patient. Previous reports suggested that MMPH remain unchanged or slowly progressive after long-term follow up , so the prognosis of MMPH is relatively good . Since MMPH is generally asymptomatic , and it is often difficult to detect multiple nodules on chest X-rays, the incidence of TSC may be underestimated without other symptoms. TSC should be suspected when the patients has multiple lung nodules.
참고하였던 저널 같이 첨부합니다.
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