Cryopyrin-associated periodic syndrome epidemiology and demographics: Difference between revisions

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==Overview==
==Overview==
The [[incidence]] cryopyrin-associated periodic syndrom is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide. The [[prevalence]] of cryopyrin-associated periodic syndrom is approximately 0.1 per 100,000 individuals worldwide. CAPS is usually first presented in the infancy. There is no racial predilection to cryopyrin-associated periodic syndrome. Cryopyrin-associated periodic syndrome affects men and women equally. The majority of CAPS cases are reported in Europe.
The [[incidence]] cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide. The [[prevalence]] of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide. CAPS is usually first presented in the [[infancy]]. There is no racial predilection to cryopyrin-associated periodic syndrome. Cryopyrin-associated periodic syndrome affects men and women equally. The majority of CAPS cases are reported in Europe.
==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Incidence===
===Incidence===
*The incidence cryopyrin-associated periodic syndrom is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide.<ref name="LainkaNeudorf2010">{{cite journal|last1=Lainka|first1=E.|last2=Neudorf|first2=U.|last3=Lohse|first3=P.|last4=Timmann|first4=C.|last5=Bielak|first5=M.|last6=Stojanov|first6=S.|last7=Huss|first7=K.|last8=Kries|first8=R. von|last9=Niehues|first9=T.|title=Analysis of Cryopyrin-Associated Periodic Syndromes (CAPS) in German Children: Epidemiological, Clinical and Genetic Characteristics|journal=Klinische Pädiatrie|volume=222|issue=06|year=2010|pages=356–361|issn=0300-8630|doi=10.1055/s-0030-1265181}}</ref>
*The [[incidence]] cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide.<ref name="LainkaNeudorf2010">{{cite journal|last1=Lainka|first1=E.|last2=Neudorf|first2=U.|last3=Lohse|first3=P.|last4=Timmann|first4=C.|last5=Bielak|first5=M.|last6=Stojanov|first6=S.|last7=Huss|first7=K.|last8=Kries|first8=R. von|last9=Niehues|first9=T.|title=Analysis of Cryopyrin-Associated Periodic Syndromes (CAPS) in German Children: Epidemiological, Clinical and Genetic Characteristics|journal=Klinische Pädiatrie|volume=222|issue=06|year=2010|pages=356–361|issn=0300-8630|doi=10.1055/s-0030-1265181}}</ref>
===Prevalence===
===Prevalence===
*The prevalence of cryopyrin-associated periodic syndrom is approximately 0.1 per 100,000 individuals worldwide.<ref name="MehrAllen2016">{{cite journal|last1=Mehr|first1=Sam|last2=Allen|first2=Roger|last3=Boros|first3=Christina|last4=Adib|first4=Navid|last5=Kakakios|first5=Alyson|last6=Turner|first6=Paul J|last7=Rogers|first7=Maureen|last8=Zurynski|first8=Yvonne|last9=Singh-Grewal|first9=Davinder|title=Cryopyrin-associated periodic syndrome in Australian children and adults: Epidemiological, clinical and treatment characteristics|journal=Journal of Paediatrics and Child Health|volume=52|issue=9|year=2016|pages=889–895|issn=10344810|doi=10.1111/jpc.13270}}</ref>
*The [[prevalence]] of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide.<ref name="MehrAllen2016">{{cite journal|last1=Mehr|first1=Sam|last2=Allen|first2=Roger|last3=Boros|first3=Christina|last4=Adib|first4=Navid|last5=Kakakios|first5=Alyson|last6=Turner|first6=Paul J|last7=Rogers|first7=Maureen|last8=Zurynski|first8=Yvonne|last9=Singh-Grewal|first9=Davinder|title=Cryopyrin-associated periodic syndrome in Australian children and adults: Epidemiological, clinical and treatment characteristics|journal=Journal of Paediatrics and Child Health|volume=52|issue=9|year=2016|pages=889–895|issn=10344810|doi=10.1111/jpc.13270}}</ref>
===Age===
===Age===
*CAPS is usually first presented in the infancy. However, due to the vague nature of the symptom, diagnosis may be delayed until childhood or longer.<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
*CAPS is usually first presented in the [[infancy]]. However, due to the vague nature of the [[symptom]], [[diagnosis]] may be delayed until childhood or longer.<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
===Race===
===Race===
*There is no racial predilection to cryopyrin-associated periodic syndrome.<ref name="CuissetJeru2010">{{cite journal|last1=Cuisset|first1=L.|last2=Jeru|first2=I.|last3=Dumont|first3=B.|last4=Fabre|first4=A.|last5=Cochet|first5=E.|last6=Le Bozec|first6=J.|last7=Delpech|first7=M.|last8=Amselem|first8=S.|last9=Touitou|first9=I.|title=Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France|journal=Annals of the Rheumatic Diseases|volume=70|issue=3|year=2010|pages=495–499|issn=0003-4967|doi=10.1136/ard.2010.138420}}</ref>
*There is no [[racial]] predilection to cryopyrin-associated periodic syndrome.<ref name="CuissetJeru2010">{{cite journal|last1=Cuisset|first1=L.|last2=Jeru|first2=I.|last3=Dumont|first3=B.|last4=Fabre|first4=A.|last5=Cochet|first5=E.|last6=Le Bozec|first6=J.|last7=Delpech|first7=M.|last8=Amselem|first8=S.|last9=Touitou|first9=I.|title=Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France|journal=Annals of the Rheumatic Diseases|volume=70|issue=3|year=2010|pages=495–499|issn=0003-4967|doi=10.1136/ard.2010.138420}}</ref>
===Gender===
===Gender===
*Cryopyrin-associated periodic syndrome affects men and women equally.<ref name="CuissetJeru2010">{{cite journal|last1=Cuisset|first1=L.|last2=Jeru|first2=I.|last3=Dumont|first3=B.|last4=Fabre|first4=A.|last5=Cochet|first5=E.|last6=Le Bozec|first6=J.|last7=Delpech|first7=M.|last8=Amselem|first8=S.|last9=Touitou|first9=I.|title=Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France|journal=Annals of the Rheumatic Diseases|volume=70|issue=3|year=2010|pages=495–499|issn=0003-4967|doi=10.1136/ard.2010.138420}}</ref>
*Cryopyrin-associated periodic syndrome affects men and women equally.<ref name="CuissetJeru2010">{{cite journal|last1=Cuisset|first1=L.|last2=Jeru|first2=I.|last3=Dumont|first3=B.|last4=Fabre|first4=A.|last5=Cochet|first5=E.|last6=Le Bozec|first6=J.|last7=Delpech|first7=M.|last8=Amselem|first8=S.|last9=Touitou|first9=I.|title=Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France|journal=Annals of the Rheumatic Diseases|volume=70|issue=3|year=2010|pages=495–499|issn=0003-4967|doi=10.1136/ard.2010.138420}}</ref>

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

The incidence cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide. The prevalence of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide. CAPS is usually first presented in the infancy. There is no racial predilection to cryopyrin-associated periodic syndrome. Cryopyrin-associated periodic syndrome affects men and women equally. The majority of CAPS cases are reported in Europe.

Epidemiology and Demographics

Incidence

  • The incidence cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide.[1]

Prevalence

  • The prevalence of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide.[2]

Age

  • CAPS is usually first presented in the infancy. However, due to the vague nature of the symptom, diagnosis may be delayed until childhood or longer.[3]

Race

  • There is no racial predilection to cryopyrin-associated periodic syndrome.[4]

Gender

  • Cryopyrin-associated periodic syndrome affects men and women equally.[4]

Region

  • The majority of CAPS cases are reported in Europe.[3]

References

  1. Lainka, E.; Neudorf, U.; Lohse, P.; Timmann, C.; Bielak, M.; Stojanov, S.; Huss, K.; Kries, R. von; Niehues, T. (2010). "Analysis of Cryopyrin-Associated Periodic Syndromes (CAPS) in German Children: Epidemiological, Clinical and Genetic Characteristics". Klinische Pädiatrie. 222 (06): 356–361. doi:10.1055/s-0030-1265181. ISSN 0300-8630.
  2. Mehr, Sam; Allen, Roger; Boros, Christina; Adib, Navid; Kakakios, Alyson; Turner, Paul J; Rogers, Maureen; Zurynski, Yvonne; Singh-Grewal, Davinder (2016). "Cryopyrin-associated periodic syndrome in Australian children and adults: Epidemiological, clinical and treatment characteristics". Journal of Paediatrics and Child Health. 52 (9): 889–895. doi:10.1111/jpc.13270. ISSN 1034-4810.
  3. 3.0 3.1 Kastner, D. L. (2005). "Hereditary Periodic Fever Syndromes". Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
  4. 4.0 4.1 Cuisset, L.; Jeru, I.; Dumont, B.; Fabre, A.; Cochet, E.; Le Bozec, J.; Delpech, M.; Amselem, S.; Touitou, I. (2010). "Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France". Annals of the Rheumatic Diseases. 70 (3): 495–499. doi:10.1136/ard.2010.138420. ISSN 0003-4967.

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