Asplenia epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kalpana Giri, MBBS[2]
Overview
The incidence of congenital asplenia is approximately 1/10,000 to 1/40,000 live births per 100,000 individuals worldwide. Heterotaxy syndrome with asplenia and right atrial isomerism occurring approximately in 1 in 10,000-40,000 births. The prevalence of asplenia is vary among different conditions. The prevalence of Isolated congenital asplenia is 0.51 per million births, in alcoholic liver disease, is about 37-100%, celiac disease 33-76% , Whipple’s disease 47% and in bone marrow transplantation 40% , and in other cases the frequency of hyposplenism is relatively low such as in systemic lupus erythematosus around 7%. The mortality remains high, at greater than 60%, in asplenic patients who are at risk for overwhelming infection and when they are complicated by invasive infection. Patients younger than 16 years old are considered to be at higher risk of OPSI due to their immature immune system. Asplenia occurs slightly more often in males than in females.
Epidemiology and Demographics
Incidence
- The incidence of congenital asplenia is approximately 1/10,000 to 1/40,000 live births per 100,000 individuals worldwide.[1]
- The incidence of overwhelming post-splenectomy infection syndrome (OPSI) is 50% higher in splenectomised patients compared to healthy individuals.[2]
- Heterotaxy syndrome with asplenia and right atrial isomerism occurring approximately in 1 in 10,000-40,000 births, which is the most frequent one of these syndromes.[3]
Prevalence
- The prevalence of asplenia is vary among different conditions.[4]
- The prevalence of Functional hyposplenism in Sickle cell disease, almost 100% of cases, and overwhelming post-splenectomy infection syndrome(OPSI) occur more frequently.[5]
- The prevalence in alcoholic liver disease, is about 37-100%, celiac disease 33-76% , Whipple’s disease 47% and in bone marrow transplantation 40% , and in other cases the frequency of hyposplenism is relatively low such as in systemic lupus erythematosus around 7%.
- The prevalence of Isolated congenital asplenia is 0.51 per million births, indicated by French nationwide study.[1]
Mortality
- Asplenic patients are at risk for overwhelming infection and when they are complicated by invasive infection, the mortality remains high, at greater than 60%.[6]
Age
- Patients younger than 16 years old are considered to be at higher risk of OPSI due to their immature immune system.[5]
Gender
References
- ↑ 1.0 1.1 Mahlaoui N, Minard-Colin V, Picard C, Bolze A, Ku CL, Tournilhac O; et al. (2011). "Isolated congenital asplenia: a French nationwide retrospective survey of 20 cases". J Pediatr. 158 (1): 142–8, 148.e1. doi:10.1016/j.jpeds.2010.07.027. PMID 20846672.
- ↑ Hansen K, Singer DB (2001). "Asplenic-hyposplenic overwhelming sepsis: postsplenectomy sepsis revisited". Pediatr Dev Pathol. 4 (2): 105–21. doi:10.1007/s100240010145. PMID 11178626.
- ↑ Erdem SB, Genel F, Erdur B, Ozbek E, Gulez N, Mese T (2015). "Asplenia in children with congenital heart disease as a cause of poor outcome". Cent Eur J Immunol. 40 (2): 266–9. doi:10.5114/ceji.2015.52841. PMC 4637402. PMID 26557043.
- ↑ LIPSON RL, BAYRD ED, WATKINS CH (1959). "The postsplenectomy blood picture". Am J Clin Pathol. 32: 526–32. doi:10.1093/ajcp/32.6.526. PMID 14417436.
- ↑ 5.0 5.1 Holdsworth RJ, Irving AD, Cuschieri A (1991). "Postsplenectomy sepsis and its mortality rate: actual versus perceived risks". Br J Surg. 78 (9): 1031–8. doi:10.1002/bjs.1800780904. PMID 1933181.
- ↑ Uchida Y, Matsubara K, Wada T, Oishi K, Morio T, Takada H; et al. (2012). "Recurrent bacterial meningitis by three different pathogens in an isolated asplenic child". J Infect Chemother. 18 (4): 576–80. doi:10.1007/s10156-011-0341-z. PMID 22147274.
- ↑ Rose V, Izukawa T, Moës CA (1975). "Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis". Br Heart J. 37 (8): 840–52. doi:10.1136/hrt.37.8.840. PMC 482884. PMID 1191445.