Beta thalassemia syndromes are among the most common monogenic disorders that occur globally and are generally inherited as autosomal recessive diseases. The beta globin gene HBB is found on the short arm of chromosome 11, and to date, more than 200 pathogenic HBB mutations have been identified. Beta globin and alpha globin combine to create the adult hemoglobin tetramer, which comprises 2 chains of each; however, reduced or absent synthesis of beta globin chains can create an imbalance that leads to accumulation and precipitation of free alpha chains in the red blood cell precursors. The result is anemia, which then stimulates erythropoietin synthesis that results in intense proliferation of bone marrow, skeletal deformities, dysregulated iron homeostasis, and increased levels of reactive oxygen species in the erythroid cells. Based on the severity of the phenotype, beta thalassemias are classified as either transfusion-dependent (TDT) or nontransfusion-dependent (NTDT).
Advances in understanding the pathophysiology of beta thalassemia have substantially improved patient management and led to a robust increase in life expectancy. However, longer survival brings its own set of issues. Moreover, the underlying pathophysiology of the syndromes has not been eliminated, and current treatments, although effective, are associated with complications. Improvements in survival have also been associated with age-related disorders that occur in the general population.
In a review article published in Expert Review of Hematology, Irene Motta, MD, of the department of clinical sciences and community health at the Universit degli Studi di Milano in Italy, and colleagues discussed the management of common complications that have been observed in patients with beta thalassemia.
Cardiovascular diseases remain a significant cause of morbidity and the primary cause of death in patients with both TDT and NTDT. More than 70% of deaths in this patient population are due to cardiovascular disease, and arrhythmias are becoming more prevalent in adults who have experienced previous iron overload.
All patients transitioned to adult care are monitored closely for these complications, said Hayley Merkeley, MD, MSc, FRCPC, of the division of hematology at the University of British Columbia in Canada. With respect to cardiac concerns, patients receive echocardiograms every 1 to 5 years depending on their symptoms, as well as a cardiac [magnetic resonance imaging scan] every 6 [to] 12 months.
Arrhythmias should be treated as in the general population. Although ablation strategies can be considered in select cases, there are currently no specific recommendations for it.
Adrenal insufficiency is another complication of thalassemia, although the researchers noted that among all of the thalassemia-related endocrinopathies, adrenal insufficiency in particular has been poorly studied and underreported in adult patients. Because it tends to develop gradually over time, and symptoms may be nonspecific and attributed to chronic anemia, undetected adrenal insufficiency may eventually present as a life-threatening acute crisis.
It is recommended, therefore, that the annual endocrinological evaluation for patients with both TDT and NTDT who are older than 18 years should always include basal adrenal function evaluation (adrenocorticotropic hormone [ACTH] and cortisol basal levels), and replacement therapy with cortisone acetate should be given if a diagnosis of adrenal insufficiency is made. For patients with low or borderline basal cortisol levels, an ACTH stimulation test should be performed in order to identify adrenal insufficiency.
Read more here:
Managing Complications Associated With Increased Longevity in Beta Thalassemia - Hematology Advisor
- Inside the longevity lab that aims to help people live longer - TODAY - February 27th, 2024
- I'm a longevity expert these are the 4 supplements I take every day to fight aging - New York Post - February 27th, 2024
- The Longest Living People In the World All Abide by the 'Power 9' Rule - GQ - February 27th, 2024
- Im a longevity expert and there's six things you should do daily if you want to live longer' - Daily Record - February 27th, 2024
- Reducing the metabolic burden of rRNA synthesis promotes healthy longevity in Caenorhabditis elegans - Nature.com - February 27th, 2024
- Presidio Federal's Thad Anderson Receives WashingtonExec's Longevity Award - WashingtonExec - February 27th, 2024
- Faces of wealth, intellect and longevity, as revealed by veteran face reader - The Korea Herald - February 27th, 2024
- I'm a longevity expert here are the best foods to eat for a long and healthy life - New York Post - February 27th, 2024
- Danny Care: 'Pilates Has Helped with the Longevity of My Career' - Men's Health UK - February 27th, 2024
- Give Me This Mountain | Tips for Spiritual Longevity - Harvest.org - February 27th, 2024
- Chris Hemsworth did a 4-day fast to reverse aging. His trainer played a cheeky joke to test his willpower. - Yahoo Canada Shine On - February 27th, 2024
- Pet longevity is a spark about to turn into a wildfire - Longevity.Technology - February 27th, 2024
- The Longevity of Bitcoin's Bull Run; Polkadot and Up-and-Coming Memecoin Draw Crowds - Analytics Insight - February 27th, 2024
- 'This is weird': Experts 'shocked' by record-breaking longevity of Death Valley's phantom lake - Livescience.com - February 27th, 2024
- Understanding the Longevity of SARS-CoV-2 Antibody Responses to Infection and Vaccination - Medriva - February 27th, 2024
- Experts agree: The fasting-mimicking diet may be the key to longevity - Longevity.Technology - February 27th, 2024
- Daily habits of a longevity expert : TED Radio Hour - NPR - December 30th, 2023
- Why does fruitcake last so long? Ingredients and preparation of the dessert play into its longevity - Fox News - December 30th, 2023
- Interviews of 2023: the longevity companies - Longevity.Technology - December 30th, 2023
- Nancy Wilson Discusses Longevity of Heart & Family Drama Within the Band Ahead of New Year's Eve Show in Seattle - American Songwriter - December 30th, 2023
- What Will It Take To Make Super-Aging Not So Super-White? - Well+Good - December 30th, 2023
- Arteta on Moyes and longevity in coaching | Quotes | News - Arsenal.com - December 30th, 2023
- New Years resolution guide: 5 steps to a healthier 2024 from a longevity expert - Fox News - December 30th, 2023
- Interviews of 2023: the longevity visionaries - Longevity.Technology - December 30th, 2023
- The Top 11 Longevity Tips Of 2023, From The mindbodygreen Podcast | mindbodygreen - mindbodygreen - December 30th, 2023
- Why LeBron James' longevity is unmatched in the NBA | Good Word with Goodwill - Yahoo Sports - December 30th, 2023
- Short vs long fasts: What is the ideal length for longevity? - Longevity.Technology - December 30th, 2023
- Centenarians share the secrets to living a long, healthy life - Fox Business - December 30th, 2023
- Rob Lowe on Turning 60 Soon & His Secret to Career Longevity (Exclusive) - Extra - December 30th, 2023
- Investigating the longevity and diversity of South Asian miniature traditions - STIRworld - December 30th, 2023
- The Silent Threat of Hypochondria: Impact on Longevity and Health - Medriva - December 30th, 2023
- Longevity: What lifestyle habits could help you live to 100? - December 22nd, 2023
- Aiming for longevity - Harvard Health - December 22nd, 2023
- How Long Can We Live? - The New York Times - December 22nd, 2023
- This Was the Year of Obsessing Over Longevity - GQ - December 22nd, 2023
- Menopause Age Looms Over Women. Efforts to Prevent it Could Extend Fertility. - Bloomberg - December 22nd, 2023
- Longevity: Is exercise the key to a longer, healthier life? - Medical News Today - December 22nd, 2023
- The #1 Sign You'll Live to 100, According to Longevity Experts - Gwinnettdailypost.com - December 22nd, 2023
- Cowboys Take On Dolphins, How Longevity Factors Into Hall of Fame Election and More - The 33rd Team - December 22nd, 2023
- Longevity Hacks Are Coming to Your Gym - WSJ - The Wall Street Journal - December 22nd, 2023
- 106-year-old woman shares hobbies, habits she says helped longevity - Business Insider - December 22nd, 2023
- Tips to Improve Your Health & Longevity in the New Year - KATU - December 22nd, 2023
- NN Group gets 13bn longevity risk transfer with Prudential and Swiss Re - Reinsurance News - December 22nd, 2023
- A fresh perspective on longevity - savannahmagazine - December 22nd, 2023
- Este Lauder targets age reversal with Stanford Center on Longevity partnership - Cosmetics Business - December 22nd, 2023
- Longevity means taking a proactive approach to prevention - Longevity.Technology - December 22nd, 2023
- Decoding Longevity: Centenarian Traits and Aging Research - BNN Breaking - December 22nd, 2023
- Brain Age: The Science of Cognitive Longevity - BNN Breaking - December 22nd, 2023
- The Role of Exercise in Longevity: A Holistic Approach to Health and Fitness - Medriva - December 22nd, 2023
- Get interventions to market and to people faster and cheaper - Longevity.Technology - December 22nd, 2023
- Humans Could Live up to 150 Years, New Research Suggests - May 17th, 2023
- The ingredients for a longer life - BBC Future - May 17th, 2023
- Life Expectancy by State 2023 - World Population Review - May 17th, 2023
- About Us - Fountain Life - May 17th, 2023
- Longevity: The Keys to Slow the Aging Process - Dr. Axe - March 29th, 2023
- "I got a 20-year contract. Giannis is going to play for 20 more years" - Mike Budenholzer on the longevity of Giannis Antetokounmpo and the... - January 23rd, 2023
- "I think Nadal has been doing a great job of that in the last four or five years": McEnroe believes Murray needs to shorten points for... - January 23rd, 2023
- Jon Voight is 'blessed' to have had such a long Hollywood career - Yakima Herald-Republic - November 7th, 2022
- USD Index to sink to 103 by end-2023 amid greater certainty and longevity of Dollar downtrend Westpac - FXStreet - November 7th, 2022
- Retirement Calculator | Northwestern Mutual - October 15th, 2022
- The Longevity Investors Conference Gstaad brought together longevity experts and deep-pocketed investors - Cointelegraph - October 15th, 2022
- Do you think ageing can be reversed? Here are 12 longevity startups to watch - Sifted - October 15th, 2022
- 'SNL' Gig Was a Risk Rob Lowe Credits for His Longevity: 'I Had People in My Life Telling Me - TheWrap - October 15th, 2022
- Wellness and longevity brand Serotonin Centers to open in Nashville - Tennessean - October 15th, 2022
- Lesley Stahl on longevity of '60 Minutes': 'That clock is still there' - USA TODAY - October 15th, 2022
- 4 Recipes With Corn, Beans, and SquashThe Three Sisters Ingredients That Blue Zone Centenarians in Costa Rica Eat Every Day - Well+Good - October 15th, 2022
- The Search for a Pill That Can Help Dogsand HumansLive Longer - WIRED - October 15th, 2022
- Supontis vs Dogecoin, Which Cryptocurrency Has Greater Longevity In The Market? - Coinpedia Fintech News - October 15th, 2022
- Louisville Zoo says goodbye to one of world's oldest gorillas - Evening News and Tribune - October 15th, 2022
- 'Prolong your lifespan': 5 'cheapest' foods that can boost longevity - Express - October 15th, 2022
- FOXO Technologies Announces First Distribution Partner and Begins Product Rollout in California; Expands Executive Team With Insurance Industry Talent... - October 15th, 2022
- Timeless: USM Arts and Sciences Faculty Members Ball, Meade Cite Love for Students, Teaching as Keys to Longevity in Profession - The University of... - October 15th, 2022
- The Best Workout To Slow Aging and Promote Longevity, Science Reveals Eat This Not That - Eat This, Not That - September 4th, 2022
- Share your story of loss or survival as part of The Longevity Project series on cancer - Summit Daily - September 4th, 2022
- Drinking two cups of tea a day is linked to boosted longevity - Women's Health UK - September 4th, 2022
- How the Asphalt Industry Can Improve Pavement Longevity - For Construction Pros - September 4th, 2022
- Longevity: Playing the Long Game in Ministry - The Gospel Coalition - September 4th, 2022
- Know 3 longevity secrets of the last USSR President Mikhail Gorbachev who died at the ripe age of 91 - Times Now - September 4th, 2022
- Tyrese Maxey Lauds LeBron James, Serena Williams, And Tom Brady For Their Longevity: "What Is In The Water Bron, Serena, And TB12... - September 4th, 2022
- 'There Should be Some Reward for Longevity in This Game'-Kiwi Pacer Wants Change In Central Contracts - News18 - September 4th, 2022