Asplenia epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Anum Dilip, M.B.B.S[2]
Overview
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.