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Fever, familial lifelong persistent

MedGen UID:
346443
•Concept ID:
C1856788
•
Disease or Syndrome
Synonym: Fever, Familial Lifelong Persistent
 
Monarch Initiative: MONDO:0009225
OMIM®: 228400

Recent clinical studies

Etiology

Nutcracker syndrome: a potentially underdiagnosed cause of proteinuria in children with familial Mediterranean fever.
Avar-Aydin PO, Ozcakar ZB, Cakar N, Fitoz S, Karakas HD, Yalcinkaya F
Pediatr Nephrol 2022 Jul;37(7):1615-1621. Epub 2021 Nov 18 doi: 10.1007/s00467-021-05337-9. PMID: 34796389
Previous dengue or Zika virus exposure can drive to infection enhancement or neutralisation of other flaviviruses.
Oliveira RA, de Oliveira-Filho EF, Fernandes AI, Brito CA, Marques ET, Tenório MC, Gil LH
Mem Inst Oswaldo Cruz 2019;114:e190098. Epub 2019 Aug 12 doi: 10.1590/0074-02760190098. PMID: 31411310Free PMC Article
A 62-year-old women with persistent severe asthma, skin rash, and eosinophilia.
Lataifeh AR, Deas S, Shalin SC, Khasawneh KR
Chest 2014 Aug;146(2):e52-e55. doi: 10.1378/chest.14-0347. PMID: 25091763
Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group.
Drenth JP, Haagsma CJ, van der Meer JW
Medicine (Baltimore) 1994 May;73(3):133-44. PMID: 8190036

Diagnosis

Nutcracker syndrome: a potentially underdiagnosed cause of proteinuria in children with familial Mediterranean fever.
Avar-Aydin PO, Ozcakar ZB, Cakar N, Fitoz S, Karakas HD, Yalcinkaya F
Pediatr Nephrol 2022 Jul;37(7):1615-1621. Epub 2021 Nov 18 doi: 10.1007/s00467-021-05337-9. PMID: 34796389
A 62-year-old women with persistent severe asthma, skin rash, and eosinophilia.
Lataifeh AR, Deas S, Shalin SC, Khasawneh KR
Chest 2014 Aug;146(2):e52-e55. doi: 10.1378/chest.14-0347. PMID: 25091763
Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group.
Drenth JP, Haagsma CJ, van der Meer JW
Medicine (Baltimore) 1994 May;73(3):133-44. PMID: 8190036

Therapy

Familial life-long persistent fever of unknown origin responding to dexamethasone and uronic acids.
Herman RH, Overholt EL, Hagler L
Am J Med 1969 Jan;46(1):142-53. doi: 10.1016/0002-9343(69)90066-7. PMID: 4951423

Prognosis

A 62-year-old women with persistent severe asthma, skin rash, and eosinophilia.
Lataifeh AR, Deas S, Shalin SC, Khasawneh KR
Chest 2014 Aug;146(2):e52-e55. doi: 10.1378/chest.14-0347. PMID: 25091763
Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group.
Drenth JP, Haagsma CJ, van der Meer JW
Medicine (Baltimore) 1994 May;73(3):133-44. PMID: 8190036

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