sickle cell trait; hemoglobin S trait
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Introduction
Carriers of a single hemoglobin S gene.
Pathology
- renal papillary necrosis accounts for 1/2 of hematuria
Clinical manifestations
- asymptomatic
- painless hematuria
Laboratory
- occasional hematuria
- occasional hyposthenuria
- hemoglobin electrophoresis: Hgb A 60%, Hgb S 40%
- HgbA1c may underestimate average blood glucose by ~5%[7]
Complications
- renal medullary carcinoma
- increased risk of renal disease
- chronic renal failure[4][9], including end-stage renal disease[9]
- hematuria (RR=2)
- albuminuria (RR=2)[8]
- splenic rupture at high altitudes
- venous thromboembolism[8]
- sudden death during extreme conditions
- exertional rhabdomyolysis
- similar changes in cardiovascular fitness
- no excess risk for hypertension, diabetes, or metabolic syndrome[6]
- higher prevalence of type 2 diabetes, hypertension, retinal disorders[9]
Management
- diagnostic workup should not differ from patients with normal hemoglobin
More general terms
References
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018.
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 648
- ↑ Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009 Jun;122(6):507-12 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19393983
- ↑ 4.0 4.1 Naik RP et al. Association of sickle cell trait with chronic kidney disease and albuminuria in African Americans. JAMA 2014 Nov 13 PMID: https://www.ncbi.nlm.nih.gov/pubmed/25393378
- ↑ Asplund CA, O'Connor FG. Challenging Return to Play Decisions: Heat Stroke, Exertional Rhabdomyolysis, and Exertional Collapse Associated With Sickle Cell Trait. Sports Health. 2016 Mar-Apr;8(2):117-25. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26896216
Saxena P, Chavarria C, Thurlow J. Rhabdomyolysis in a Sickle Cell Trait Positive Active Duty Male Soldier. US Army Med Dep J. 2016 Jan-Mar:20-3. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26874092
Nelson DA, Deuster PA, Carter R 3rd et al., Sickle Cell Trait, Rhabdomyolysis, and Mortality among U.S. Army Soldiers. N Engl J Med. 2016 Aug 4;375(5):435-42. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27518662 - ↑ 6.0 6.1 Liem RI, Chan C, Vu TT et al. Longitudinal association among sickle cell trait, fitness, and cardiovascular disease risk factors in African Americans in CARDIA. Blood 2016 Nov 16 PMID: https://www.ncbi.nlm.nih.gov/pubmed/27856464
- ↑ 7.0 7.1 Lacy ME, Wellenius GA, Sumner AE et al Association of Sickle Cell Trait With Hemoglobin A1c in African Americans. JAMA. 2017;317(5):507-515 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28170479 <Internet> http://jamanetwork.com/journals/jama/article-abstract/2600468
Bleyer AJ, Aloi JA Sickle Cell Trait and Interpretation of Hemoglobin A1c Levels. JAMA. 2017;317(5):481-482 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28170462 <Internet> http://jamanetwork.com/journals/jama/article-abstract/260044 - ↑ 8.0 8.1 8.2 Naik RP, Smith-Whitley K, Hassell KL et al. Clinical outcomes associated with sickle cell trait: A systematic review. Ann Intern Med 2018 Oct 30; PMID: https://www.ncbi.nlm.nih.gov/pubmed/30383109
- ↑ 9.0 9.1 9.2 9.3 Hulsizer J et al. Sickle cell trait and risk for common diseases: Evidence from the UK Biobank. Am J Med 2022 Aug; 135:e279. PMID: https://www.ncbi.nlm.nih.gov/pubmed/35461807 https://www.amjmed.com/article/S0002-9343(22)00282-0/fulltext