Wide spectrum of novel and rare hemoglobin variants in the multi-ethnic Indian population: A review.

The hemoglobin (Hb) variants are qualitative abnormalities due to production of structurally abnormal globin proteins. They are categorized based on the type of mutation present in the α1, α2, ß, Gγ, Aγ and δ globin genes. So far, more than 1550 Hb variants are reported in the database. They could lead to Hb polymerization, Hb instability, altered oxygen affinity and decreased oxygen-carrying capacity of Hb or have no clinical manifestations. In India, ethnic diversity, consanguinity, regional variations and migration result in the presence of different Hb variants. We have compiled all the variants of α, ß and δ globin chains in heterozygous, homozygous and in compound heterozygous forms reported from India in the last 52 years. Of the 63 rare and novel hemoglobin variants reported from India, 22 were α-globin chain variants, 37 were ß-globin chain variants and 4 were δ-globin chain variants. Twelve novel Hb variants (Hb J Rajappan, Hb Koya Dora, Hb Rampa, Hb Godavari, Hb Chandigarh, Hb D Agri, Hb Lucknow, Hb Vellore, Hb Midnapore, Hb Bijnor, Hb A2Tianhe and Hb A2Saurashtra) were identified among persons of Indian origin. Majority of them were picked up on HPLC. Some of the variants like Hb Titusville, Hb Shimonoseki, Hb Chandigarh, Hb D Agri, Hb Yaizu and Hb Vellore eluted in the HbS window whereas variants like HbD Iran, Hb St. Louis, Hb G Coushata, HbM Saskatoon, Hb Lucknow, Hb Grange-Blanche and Hb Tianshui showed falsely elevated HbA2. Hence, careful and systematic investigations are required to identify them.

Link between individual resilience and aggressiveness in dental students and the mediating effect of spirituality: A path analysis.

BACKGROUND: The role of spirituality in people's lives, particularly the lives of health professionals, as well as its impact on mental health issues like anger and aggressiveness and resilience, are particularly significant. Therefore, the purpose of this study was to examine the link between the propensity for violence and personal resilience in dental students by taking into account the function of spirituality as a mediator. MATERIALS AND METHODS: In this cross-sectional questionnaire survey, 211 volunteer dentistry students participated from a government institute in Jammu and Kashmir, India using a stratified sample procedure. The Spiritual Orientation Scale, Buss and Perry Aggression Questionnaire, and Connor-Davidson Resilience Scale were used to gather the data. SPSS version 20.0 was used for conducting a bivariate analysis to ascertain the directionality connection between the research variables. In addition; structural equation modelling analysis was conducted by Smart PLS. RESULTS: According to the findings, resilience and spirituality have a substantial and positive link (r = 0.468 r = 0.023). Aggressiveness and resilience revealed a statistically significant inverse link (r = 0.325, P = 0.04), but aggression and spirituality had no significant correlation. Spirituality played a substantial mediation influence in the indirect pathway of violence on resilience (P = 0.001). CONCLUSION: According to the study's findings, spirituality can help pupils become more resilient as individuals and can act as a helpful intermediary between aggressiveness and resilience.

Role of hydroxyurea therapy in the prevention of organ damage in sickle cell disease: a systematic review and meta-analysis.

BACKGROUND: Hydroxyurea is an affordable drug that reduces vaso-occlusive crises and transfusion requirements in sickle cell disease. However, its effectiveness in preventing chronic organ damage is still unclear. This systematic review and meta-analysis aimed to evaluate the role of hydroxyurea in preventing organ morbidity. METHOD: We included original articles published in English from 1st January 1990 to 31st January 2023, reporting hydroxyurea therapy and organ damage from PubMed, Google Scholar, Scopus, and CrossRef databases. A total of 45 studies with 4681 sickle cell disease patients were evaluated for organ damage. RESULTS: Our analysis showed that hydroxyurea intervention significantly lowered transcranial Doppler and tricuspid regurgitant velocity, with a standardized mean difference of - 1.03 (- 1.49; - 0.58); I 2 = 96% and - 1.37 (CI - 2.31, - 0.42); I 2 = 94%, respectively. Moreover, the pooled estimate for albuminuria showed a beneficial effect post-hydroxyurea therapy by reducing the risk of albuminuria by 58% (risk ratio of 0.42 (0.28; 0.63); I 2 = 28%). CONCLUSION: Our study found that a hydroxyurea dose above 20 mg/kg/day with a mean rise in HbF by 18.46% post-hydroxyurea therapy had a beneficial role in reducing transcranial doppler velocity, tricuspid regurgitant velocity, albuminuria, and splenic abnormality. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42023401187.