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Related Concept Videos

Types of Fever01:25

Types of Fever

Fever can be triggered by several factors, including infections, nervous system disorders, certain cancers, blood diseases like leukemia, embolism, thrombosis, heatstroke, dehydration, surgical trauma, crushing injuries, and allergic reactions.
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Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

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[Periodic fever with increased IgD].

J Ammar1, H Abid, S Yaalaoui

  • 1Service de pneumopédiatrie, hôpital A.-Mami, 2080 Ariana, Tunisie. jamelammar2000@yahoo.fr

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|July 27, 2010
PubMed
Summary
This summary is machine-generated.

Periodic fevers, like familial Mediterranean fever (FMF), involve recurrent inflammatory attacks. This case highlights a child with FMF-like symptoms and elevated IgD, despite negative FMF gene mutations and normal HIDS markers.

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Area of Science:

  • Pediatrics
  • Immunology
  • Genetics

Background:

  • Periodic fevers are recurrent inflammatory conditions, with Familial Mediterranean Fever (FMF) and Hyperimmunoglobulinemia D Syndrome (HIDS) being recognized entities.
  • These conditions are characterized by intermittent inflammatory attacks, necessitating accurate diagnosis for effective management.

Observation:

  • A 6-year-old boy presented with recurrent fevers, chest pain, pneumonitis, and pleuritis since age 5.
  • Laboratory findings included leukocytosis, elevated ESR and CRP, with persistently high serum IgD levels.
  • Episodes occurred stereotypically every 4-6 weeks, with infectious, malignant, and autoimmune causes excluded.

Findings:

  • Common FMF gene mutations (M694V, M680I, V726A, E148Q, M694I, A744S) were negative in this Tunisian patient.
  • Urinary leukotriene E4 levels, often elevated in HIDS, were normal.
  • Mevalonate kinase activity was not assessed, leaving a diagnostic gap for HIDS.

Implications:

  • The clinical presentation and ethnic background suggest FMF, complicated by elevated IgD, rather than typical HIDS.
  • Lack of response to colchicine and negative FMF mutation screening present diagnostic challenges.
  • This case underscores the complexity of diagnosing periodic fever syndromes and the need for broader genetic and biochemical analyses.