Transthyretin amyloid cardiomyopathy disease burden is increasing daily due to advancements in diagnostic and imaging modalities in the modern world. Tafamidis is one of many therapeutic options. The main objective of this review is to study the role of Tafamidis in slowing the progression of transthyretin cardiomyopathy (TTR-CM) by analyzing randomized controlled trials (RCTs) and non-RCTs of Tafamidis. We searched for published papers of Tafamidis in the English language in electronic databases like Google Scholar, PubMed, Cochrane Library, and PubMed Central. We imported the resulting articles from our search to Mendeley software. Four reviewers removed the duplicates and performed title and abstract screening of the articles. The same reviewers obtained the full-text of relevant articles and did fulltext screening based on eligibility criteria. Finally, five reviewers performed a quality assessment of RCTs using the Cochrane risk of bias assessment and of non-RCTs by a checklist prepared by Downs and Black. Any disagreements about any process were resolved by a discussion with other authors. One RCT and five non-RCTs of Tafamidis were included in this systematic review. From the non-RCTs, stability was observed in different parameters like echocardiographic findings, cardiac biomarkers, and ECG in patients with transthyretin cardiomyopathy during the study duration with Tafamidis. ATTR-ACT (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial) trial demonstrated reduction of cardiovascular events and allcause mortality in the Tafamidis group in comparison to placebo. In both RCT and non-RCTs, Tafamidis was established as a safe and tolerable drug for patients with TTR-CM. Our study proved the role of Tafamidis in reducing cardiovascular events, all-cause mortality, and the progression of cardiac disease in TTR-CM patients. In addition to five non-RCTs, current evidence is based on the findings of only one RCT of Tafamidis. Hence, evidence from additional RCTs is required to strongly support the stability of parameters like echocardiographic findings, cardiac biomarkers, and ECG with Tafamidis use.
This case report highlights considering Behcet's disease as a diagnosis in orogenital ulcers and uveitis, although its prevalence is unknown in Nepal due to underreporting. Also, collaboration for patient care among relevant specialties is required.
Behcet’s Disease is a rare systemic vasculitis characterized by
recurrent episodes of acute inflammation affecting blood vessels of all
sizes. Symptoms include orogenital apthosis, cutaneous skin lesions, and
uveitis. We present the case of a 38-year-old Nepalese man with Behcet’s
Disease. In Nepal, Behcet’s Disease may still be under-reported.
Perimedullary spinal AVM is a rare type of spinal arteriovenous
malformations. We present a case of 70 yrs female who presented with
motor weakness in her bilateral limbs. Initial MRI was misinterpreted as
ependymal myxoma; however, histopathology revealed spinal AVM. MRA or
DSA should be conducted if AVM is suspected.
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