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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.12
- 수록면
- 99 - 103 (5page)
- DOI
- 10.18214/jend.2021.00094
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초록· 키워드
오류제보하기
Transthyretin amyloidosis (aTTR) is a life-threatening type of systemic amyloidosis that has been associated with autosomal dominant mutations in the transthyretin gene. In this case series, we reviewed 3 patients initially treated for acquired demyelinating neuropathy that was later confirmed by pathologic testing and genetic analysis as aTTR. These patients had systemic symptoms and family records of sudden deaths that could not be explained by acquired demyelinating neuropathy. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable neuropathy; however, 20% to 33% of CIDP patients remain refractory to conventional immunotherapy, and incorrect diagnoses might be the cause of this therapeutic failure. This case series revealed that the electrophysiological findings of aTTR could resemble the findings of demyelinating neuropathy.
목차
등록된 정보가 없습니다.
참고문헌 (9)
참고문헌 신청
Ando Y / 2005 / Transthyretin-related familial amyloidotic polyneuropathy / Arch Neurol 62:1057~1062
Cocito D / 2010 / A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy / Eur J Neurol 17:289~294
Kaplan A / 2017 / Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy / Muscle Nerve 55:476~482
Allen JA / 2015 / CIDP diagnostic pitfalls and perception of treatment benefit / Neurology 85:498~504
Van den Bergh PY / 2010 / European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy : report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society-first revision / Eur J Neurol 17:356~363
Cocito D / 2010 / A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy / Eur J Neurol 17:289~294
Kaplan A / 2017 / Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy / Muscle Nerve 55:476~482
Allen JA / 2015 / CIDP diagnostic pitfalls and perception of treatment benefit / Neurology 85:498~504
Van den Bergh PY / 2010 / European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy : report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society-first revision / Eur J Neurol 17:356~363
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