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Periodic fever syndromes.

P R Scholl1

  • 1Department of Pediatrics, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois 60614, USA. prscholl@northwestern.edu

Current Opinion in Pediatrics
|December 6, 2000
PubMed
Summary

Periodic fever syndromes encompass disorders with recurrent fevers. This review covers advances in genetics, molecular pathology, clinical features, and treatments for these conditions.

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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Periodic fever syndromes are a group of autoinflammatory disorders characterized by recurrent episodes of fever.
  • Historically, the term was narrowly defined but now broadly includes conditions with non-strictly periodic fevers.
  • Examples include cyclic neutropenia, PFAPA syndrome, familial Mediterranean fever, autosomal dominant familial fevers, and hyperimmunoglobulin D syndrome.

Purpose of the Study:

  • To provide a comprehensive review of recent advancements in periodic fever syndromes.
  • To discuss the genetic and molecular underpinnings of these diseases.
  • To outline current clinical characterization and treatment strategies.

Main Methods:

  • Literature review of recent research on periodic fever syndromes.
  • Analysis of genetic and molecular pathology findings.
  • Synthesis of clinical data and treatment outcomes.

Main Results:

  • Significant progress has been made in understanding the genetic basis of various periodic fever syndromes.
  • Molecular pathology reveals diverse inflammatory pathways involved.
  • Clinical characterization highlights overlapping and distinct features across syndromes.

Conclusions:

  • Periodic fever syndromes represent a spectrum of autoinflammatory conditions with evolving genetic and molecular insights.
  • Accurate diagnosis and tailored treatment are crucial for managing these complex disorders.
  • Continued research is essential for further understanding and therapeutic development.

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