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== Overview == | == Overview == | ||
[[Hereditary spherocytosis]] can present at any [[Ageing|age]] with any presentation from [[hydrops fetalis]] [[Uterus|inutero]] through [[diagnosis]] in the ninth decade of [[life]], and is reported worldwide in all [[Race|racial]] and [[Ethnic group|ethnic groups]]. It is most [[Common-cause and special-cause|common]] [[inherited]] [[anemia]] in northern european ancestry and north america. The reported [[incidence]] is 1 in 2000 [[Birth|births]]. Approximately 25% of all [[hereditary spherocytosis]] is [[autosomal recessive]]. It is most often [[Diagnosis|diagnosed]] in childhood or early [[Adult|adulthood]]. | |||
== Epidemiology and Demographics == | == Epidemiology and Demographics == | ||
[[ | * [[Hereditary spherocytosis]] is reported worldwide in all [[Race|racial]] and [[Ethnic group|ethnic groups]].<ref>{{Cite journal | ||
| author = [[Silverio Perrotta]], [[Patrick G. Gallagher]] & [[Narla Mohandas]] | |||
| title = Hereditary spherocytosis | |||
| journal = [[Lancet (London, England)]] | |||
| volume = 372 | |||
| issue = 9647 | |||
| pages = 1411–1426 | |||
| year = 2008 | |||
| month = October | |||
| doi = 10.1016/S0140-6736(08)61588-3 | |||
| pmid = 18940465 | |||
}}</ref> | |||
* It is the most [[Common-cause and special-cause|common]] [[inherited]] [[anemia]] in the northern European ancestry and north america.<ref>{{Cite journal | |||
| author = [[Sayeeda Huq]], [[Mark A. C. Pietroni]], [[Hafizur Rahman]] & [[Mohammad Tariqul Alam]] | |||
| title = Hereditary spherocytosis | |||
| journal = [[Journal of health, population, and nutrition]] | |||
| volume = 28 | |||
| issue = 1 | |||
| pages = 107–109 | |||
| year = 2010 | |||
| month = February | |||
| pmid = 20214092 | |||
}}</ref> | |||
* The reported [[incidence]] of [[hereditary spherocytosis]] is 1 in 2000 [[Birth|births]].<ref>{{Cite journal | |||
| author = [[Sayeeda Huq]], [[Mark A. C. Pietroni]], [[Hafizur Rahman]] & [[Mohammad Tariqul Alam]] | |||
| title = Hereditary spherocytosis | |||
| journal = [[Journal of health, population, and nutrition]] | |||
| volume = 28 | |||
| issue = 1 | |||
| pages = 107–109 | |||
| year = 2010 | |||
| month = February | |||
| pmid = 20214092 | |||
}}</ref> | |||
* It is less commonly seen in african american and southeast asian people.<ref name="PerrottaGallagher2008">{{cite journal|last1=Perrotta|first1=Silverio|last2=Gallagher|first2=Patrick G|last3=Mohandas|first3=Narla|title=Hereditary spherocytosis|journal=The Lancet|volume=372|issue=9647|year=2008|pages=1411–1426|issn=01406736|doi=10.1016/S0140-6736(08)61588-3}}</ref> | |||
=== Incidence === | === Incidence === | ||
* In the United States, the incidence of the disorder is approximately one case in 5000 people. | * In the [[United States]], the [[incidence]] of the [[Disorder (medicine)|disorder]] is approximately one case in 5000 people. | ||
* Given that approximately 25% of all | * Given that approximately 25% of all [[hereditary spherocytosis]] is [[autosomal recessive]], calculations indicate that 1.4% of the US population might be silent [[Carrier|carriers]] of [[hereditary spherocytosis]]. | ||
=== Prevalence === | === Prevalence === | ||
* In northern European, | * In northern European, [[hereditary spherocytosis]] affects as many as 1 in 2000 to 1 in 5000 ([[prevalence]], approximately 0.02 to 0.05 percent) [6,7,62,75]. | ||
* The frequency is thought to be lower in individuals from other parts of the world such as Africa and Southeast Asia, although comprehensive population survey data are unavailable. | * The [[frequency]] is thought to be lower in individuals from other parts of the world such as Africa and Southeast Asia, although comprehensive [[population]] [[Survey Research Methods|survey]] [[data]] are unavailable. | ||
=== Age === | === Age === | ||
* | * [[Hereditary spherocytosis]] can present at any [[Ageing|age]] and with any severity, with [[Case report|case reports]] describing a range of presentations, from [[hydrops fetalis]] [[Uterus|in utero]] through [[diagnosis]] in the ninth decade of [[life]].<ref name="pmid18940465">{{cite journal| author=Perrotta S, Gallagher PG, Mohandas N| title=Hereditary spherocytosis. | journal=Lancet | year= 2008 | volume= 372 | issue= 9647 | pages= 1411-26 | pmid=18940465 | doi=10.1016/S0140-6736(08)61588-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18940465 }}</ref><ref name="pmid1954389">{{cite journal| author=Whitfield CF, Follweiler JB, Lopresti-Morrow L, Miller BA| title=Deficiency of alpha-spectrin synthesis in burst-forming units-erythroid in lethal hereditary spherocytosis. | journal=Blood | year= 1991 | volume= 78 | issue= 11 | pages= 3043-51 | pmid=1954389 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1954389 }}</ref> | ||
* | * [[Hereditary spherocytosis]] is most often [[Diagnosis|diagnosed]] in [[Child|childhood]] or early [[Adult|adulthood]]. | ||
* Children diagnosed early in life usually have a severe form of | * [[Child|Children]] [[Diagnosis|diagnosed]] early in [[life]] usually have a severe form of [[hereditary spherocytosis]] that results in their early presentation. [[Jaundice]] is likely to be most prominent in [[Infant|newborns]]. The [[Magnitude (mathematics)|magnitude]] of [[Jaundice|hyperbilirubinemia]] may be such that [[exchange transfusion]] is required. Approximately 30-50% of [[Adult|adults]] with [[hereditary spherocytosis]] had a history of [[jaundice]] during the first week of [[life]]. Recognition of [[hereditary spherocytosis]] as a potential [[Causality|cause]] of [[Infant|neonatal]] [[anemia]] and [[Jaundice|hyperbilirubinemia]] and institution of prompt treatment may reduce the risk of [[bilirubin]]-induced [[Neurology|neurologic]] dysfunction in these [[Patient|patients]].<ref name="pmid26009624">{{cite journal| author=Christensen RD, Yaish HM, Gallagher PG| title=A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates. | journal=Pediatrics | year= 2015 | volume= 135 | issue= 6 | pages= 1107-14 | pmid=26009624 | doi=10.1542/peds.2014-3516 | pmc=4444801 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26009624 }}</ref> | ||
=== Race === | === Race === | ||
* | * [[Hereditary spherocytosis]] occurs in all [[Race|racial]] and [[Ethnic group|ethnic groups]] but is more common in northern Europeans, | ||
* | |||
=== Gender === | === Gender === | ||
* | * There is no significant [[data]] from US related to gender difference. In 2011, the number of cases was 114 reported from CBM(China Biology Medicine) [[database]], the [[male]]: [[female]] [[ratio]] was 1.04:1.<ref name="pmid25984425">{{cite journal| author=Wang C, Cui Y, Li Y, Liu X, Han J| title=A systematic review of hereditary spherocytosis reported in Chinese biomedical journals from 1978 to 2013 and estimation of the prevalence of the disease using a disease model. | journal=Intractable Rare Dis Res | year= 2015 | volume= 4 | issue= 2 | pages= 76-81 | pmid=25984425 | doi=10.5582/irdr.2015.01002 | pmc=4428190 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25984425 }}</ref> | ||
* In 2011, overall [[Literature and Medicine|literature]] reported [[prevalence]] of [[hereditary spherocytosis]] in China was estimated to be: 1.27 cases per 100,000 people in [[Male|males]] and 1.49 cases per 100,000 people in [[Female|females]] | |||
* | |||
=== Region === | === Region === | ||
* Hereditary spherocytosis occurs in 1 in 5,000 individuals of Northern European ancestry. This condition is the most common cause of inherited anemia in that population. The prevalence of hereditary spherocytosis in people of other ethnic backgrounds is unknown, but it is much less common. | * [[Hereditary spherocytosis]] occurs in 1 in 5,000 individuals of Northern European ancestry. This [[Disease|condition]] is the [[Common-cause and special-cause|most common]] [[Causality|cause]] of [[inherited]] [[anemia]] in that [[population]]. The [[prevalence]] of [[hereditary spherocytosis]] in people of other ethnic backgrounds is unknown, but it is much less common. | ||
=== Developed Countries === | === Developed Countries === | ||
Latest revision as of 21:17, 5 December 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Hereditary spherocytosis can present at any age with any presentation from hydrops fetalis inutero through diagnosis in the ninth decade of life, and is reported worldwide in all racial and ethnic groups. It is most common inherited anemia in northern european ancestry and north america. The reported incidence is 1 in 2000 births. Approximately 25% of all hereditary spherocytosis is autosomal recessive. It is most often diagnosed in childhood or early adulthood.
Epidemiology and Demographics
- Hereditary spherocytosis is reported worldwide in all racial and ethnic groups.[1]
- It is the most common inherited anemia in the northern European ancestry and north america.[2]
- The reported incidence of hereditary spherocytosis is 1 in 2000 births.[3]
- It is less commonly seen in african american and southeast asian people.[4]
Incidence
- In the United States, the incidence of the disorder is approximately one case in 5000 people.
- Given that approximately 25% of all hereditary spherocytosis is autosomal recessive, calculations indicate that 1.4% of the US population might be silent carriers of hereditary spherocytosis.
Prevalence
- In northern European, hereditary spherocytosis affects as many as 1 in 2000 to 1 in 5000 (prevalence, approximately 0.02 to 0.05 percent) [6,7,62,75].
- The frequency is thought to be lower in individuals from other parts of the world such as Africa and Southeast Asia, although comprehensive population survey data are unavailable.
Age
- Hereditary spherocytosis can present at any age and with any severity, with case reports describing a range of presentations, from hydrops fetalis in utero through diagnosis in the ninth decade of life.[5][6]
- Hereditary spherocytosis is most often diagnosed in childhood or early adulthood.
- Children diagnosed early in life usually have a severe form of hereditary spherocytosis that results in their early presentation. Jaundice is likely to be most prominent in newborns. The magnitude of hyperbilirubinemia may be such that exchange transfusion is required. Approximately 30-50% of adults with hereditary spherocytosis had a history of jaundice during the first week of life. Recognition of hereditary spherocytosis as a potential cause of neonatal anemia and hyperbilirubinemia and institution of prompt treatment may reduce the risk of bilirubin-induced neurologic dysfunction in these patients.[7]
Race
- Hereditary spherocytosis occurs in all racial and ethnic groups but is more common in northern Europeans,
Gender
- There is no significant data from US related to gender difference. In 2011, the number of cases was 114 reported from CBM(China Biology Medicine) database, the male: female ratio was 1.04:1.[8]
- In 2011, overall literature reported prevalence of hereditary spherocytosis in China was estimated to be: 1.27 cases per 100,000 people in males and 1.49 cases per 100,000 people in females
Region
- Hereditary spherocytosis occurs in 1 in 5,000 individuals of Northern European ancestry. This condition is the most common cause of inherited anemia in that population. The prevalence of hereditary spherocytosis in people of other ethnic backgrounds is unknown, but it is much less common.
Developed Countries
There is no particular relation of FA with developed countries.
Developing Countries
There is no particular relation of FA with developing countries.
References
- ↑ Silverio Perrotta, Patrick G. Gallagher & Narla Mohandas (2008). "Hereditary spherocytosis". Lancet (London, England). 372 (9647): 1411–1426. doi:10.1016/S0140-6736(08)61588-3. PMID 18940465. Unknown parameter
|month=ignored (help) - ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Perrotta, Silverio; Gallagher, Patrick G; Mohandas, Narla (2008). "Hereditary spherocytosis". The Lancet. 372 (9647): 1411–1426. doi:10.1016/S0140-6736(08)61588-3. ISSN 0140-6736.
- ↑ Perrotta S, Gallagher PG, Mohandas N (2008). "Hereditary spherocytosis". Lancet. 372 (9647): 1411–26. doi:10.1016/S0140-6736(08)61588-3. PMID 18940465.
- ↑ Whitfield CF, Follweiler JB, Lopresti-Morrow L, Miller BA (1991). "Deficiency of alpha-spectrin synthesis in burst-forming units-erythroid in lethal hereditary spherocytosis". Blood. 78 (11): 3043–51. PMID 1954389.
- ↑ Christensen RD, Yaish HM, Gallagher PG (2015). "A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates". Pediatrics. 135 (6): 1107–14. doi:10.1542/peds.2014-3516. PMC 4444801. PMID 26009624.
- ↑ Wang C, Cui Y, Li Y, Liu X, Han J (2015). "A systematic review of hereditary spherocytosis reported in Chinese biomedical journals from 1978 to 2013 and estimation of the prevalence of the disease using a disease model". Intractable Rare Dis Res. 4 (2): 76–81. doi:10.5582/irdr.2015.01002. PMC 4428190. PMID 25984425.