Pendekatan Klinis Pasien Transthyretin Cardiac Amyloidosis (ATTR-CA) - Nur Adiba Purba (Cermin Dunia Kedokteran, 2021)
Transthyretin Cardiac Amyloidosis (ATTR-CA) adalah penumpukan fragmen protein transthyretin di jaringan interstisial miokardium akibat salah lipat protein, menyebabkan kardiomiopati restriktif dan infiltratif karena penebalan dan kekakuan dinding jantung. Transthyretin sebagian besar diproduksi di hati, berbentuk tetramer dan berfungsi sebagai protein pembawa tiroksin (T4) serta holoretinol binding protein (HRP). Saat ini, pemeriksaan diagnostik metode non-invasif telah dikembangkan meskipun biopsi endomiokardium masih menjadi gold standard diagnosis. Diagnosis akurat sedini mungkin sangat berpengaruh terhadap keberhasilan serta penurunan angka morbiditas dan mortalitas. Transthyretin Cardiac Amyloidosis (ATTR-CA) is the accumulation of transthyretin protein fragment in myocardium interstitial tissue induced by misfolded protein, lead to thickening and stiffness cardiac muscles, causing restrictive and infiltrative cardiomyopathy. Transthyretin is produced mainly in the liver as tetramer and bonded both with thyroxine (T4) and holoretinol binding protein (HRP) as carrier protein. Diagnostic test with non-invasive methods have been developed recently although endomyocardial biopsy is still the gold standard. Early targeted diagnosis has huge impact for therapy management and decreasing patient’s morbidity and mortality.