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[Recurrent fevers in childhood]

Maryam Piram1, Isabelle Koné-Paut1

  • 1AP-HP, université Paris Sud, CHU de Bicêtre, centre de référence des maladies auto-inflammatoires de l'enfant (CeRéMAI)(1), service de rhumatologie pédiatrique, 78, rue du Général-Leclerc, 94270 le Kremlin-Bicêtre, France.

Presse Medicale (Paris, France : 1983)
|November 10, 2015
PubMed

Insights

Hereditary recurrent fevers (HRF) are rare genetic disorders causing repeated fever episodes. Early diagnosis, often aided by genetic testing, is crucial to manage complications like AA amyloidosis.

Area of Science:

  • Genetics
  • Immunology
  • Internal Medicine

Context:

  • Hereditary recurrent fevers (HRF) are rare Mendelian diseases.
  • These conditions involve inherited mutations affecting innate immunity pathways.
  • Recurrent fevers manifest as stereotypical febrile episodes over months or years.

Purpose:

  • To define and differentiate hereditary recurrent fevers from other febrile illnesses.
  • To highlight key diagnostic clues for suspecting HRF.
  • To underscore the importance of genetic diagnosis in monogenic HRF.

Summary:

  • Recurrent fevers are characterized by multiple, similar febrile episodes with symptom-free intervals.
  • Hereditary recurrent fevers stem from genetic mutations impacting innate immunity.
  • Key indicators include childhood onset, family history, and inflammatory symptoms, with AA amyloidosis as a common complication.

Impact:

  • Facilitates accurate diagnosis of rare genetic fever syndromes.
  • Distinguishes HRF from intermittent infectious fevers, improving patient management.
  • Emphasizes the role of genetic testing in confirming monogenic HRF.

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