IntroductionWith the advent of revolutionary information technology, most general medical information can be accessed by the community at large. However, the factual nature of information, its understandability, and actionability of diseases like Hemophilia are unknown to the general population. Hence the present study has been envisaged to assess the understandability and actionability of available video information on YouTube about Hemophilia.
MethodsA cross-sectional study was performed using the Patient Education Materials Assessment Tool for Audiovisual materials (PEMAT-AV) to assess the understandability and actionability of 50 videos shown by order of relevance utilizing three independent assessors. An online google survey was prepared using the PEMAT questionnaire as a basis and results were recorded and saved as a Microsoft Excel sheet for analysis. Data was analyzed using either Microsoft Excel or an online calculator as the case may be.
ResultsA total of 50 short videos on Hemophilia were assessed by three independent assessors using PEMAT. The data so obtained was rechecked by an independent reviewer before data analysis. Three videos were excluded due to non-English language while only two videos out of 50 showed 100% average understandability and actionability. Average understandability and actionability scores range between 34 to 100 percent and 11.1 to 100 percent, respectively. Most videos have higher average understandability than actionability (P value=0.003).
ConclusionOur study shows there are only a few high-quality short videos available as audio-visual patient education materials on YouTube about Hemophilia. There is a great need to develop content that is beneficial to patients as patient educational material.
Human leukocyte antigen (HLA) is complex immune system with many applications in transfusion and transplant medicine. A lot of clinical challenges such as febrile non-hemolytic transfusion reactions, transfusion-related acute lung injury, and graft-versus-host-disease are caused by HLA antibodies. Histocompatibility in transplant scenario is not the only function of HLA antigens but the main role is to present peptides to immune system and regulate cellular and humoral immunity for the fate of graft. HLA Class I (A, B, and C) and HLA Class II (DR, DQ, and DP) antigens are different in structure and function. Their typing methods have progressed from serology-based techniques to sequence-based typing to next-generation sequencing. Cross-matching techniques have also changed from complement-dependent cytotoxicity to microbead-based assay to flow cytometry. Finally, HLA and its disease association has long been established, particularly so in cases of autoimmune disorders. The article will be helpful for beginners to get introduced about this unique system and principles in transplant immunology.
Gilbert's syndrome (GS) is a benign hereditary disorder of bilirubin metabolism due to a mutation in the UDP glucuronosyltransferase family 1 member A1 (UGT1A1) gene which results in hyperbilirubinaemia and related complications mainly cholelithiasis. It can be co-inherited along with sickle cell anaemia, thalassaemias and other haemoglobinopathies including glucose-6-phosphate dehydrogenase deficiency, hereditary spherocytosis and cystic fibrosis. More than 100 mutations have been reported in UGT1A1 gene and the most common as insertion of extra (TA) nucleotides in the promoter region of TATA box. The more the number of TA repeats, the higher is the bilirubin levels. These mutations result in a 10%–35% reduction in the UGT1A1 enzyme activity resulting in mild to moderate unconjugated hyperbilirubinaemia and related complications. For diagnosis the mode of inheritance is more important than testing in the patients. However; the inheritance pattern of GS differs in ethnicities. For early diagnosis to prevent worsening of the symptoms and for timely management one should be aware of the inheritance pattern in patient. In this systemic analysis we studied the association between complications in GS with the genotypes and complications. It was found that TA7/7 is more significant in GS with sickle cell disease (SCD) group when compared to healthy controls with 2.2% chances of having this genotype in GS with SCD than healthy controls. The significance of having TA7/7 genotype is similar in GS with SCD and α-thalasaemia group. However, there is a high recommendation to carry out multicentre studies and conduct meta-analyses for establishing universal recommendations.
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