“Life After Death” – What happens when someone survives Ebola

NPR posted on their website an interactive webpage detailing the experiences of survivors of Ebola and those who lost their family members to the infection in the African village of Barkedu. The webpage consists of photos and audio interviews with members of the village. Click on the link below to check it out:

Life After Death

Periodic Fever Syndromes

Periodic Fever Syndromes
  • Familial Mediterranean Fever (FMF)
  • TNF Receptor-1 Associated Periodic Syndrome (TRAPS)
  • Hyperimmunoglobulin D syndrome (HIDS)
  • Periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA)
  • Cryopyrin-associated periodic syndrome (CAPS)
    • Muckle-Wells syndrome (MWS)
    • Neonatal-onset multisystem inflammatory disorder (NOMID)
    • Familial cold autoinflammatory syndrome (FCAS)
PFS

Answer to Case Challenge #4

Case challenge # 4 presented a 21 yo white male with recurrent fevers and joint pains. Symptoms started at age 10, episodes occur every 6 months. Fever episodes last about 2 weeks, also associated with periorbital edema and spreading rash on extremities. Father has similar symptoms and also developed renal failure and CHF in his 40s. On exam, he had a fever of 102.3 F, periorbital edema, patchy macular rash on extremities.

What is the most likely diagnosis in this case?

A) Familial Mediterranean Fever

B) TNF Receptor-1 associated periodic syndrome

C) Hyper IgD syndrome

D) Trichinosis

E) Cryopyrin-associated periodic syndromes

Here are the results of the voting:
Screen Shot 2015-02-20 at 9.57.49 AM
The patient has TNF Receptor-1 Associated Periodic Syndrome (TRAPS).
  • Autosomal dominant, one of periodic fever syndromes
  • First described in pt of Irish (Hibernian) origin
  • Due to genetic defect in TNF receptor gene (TNFR1)
  • Presents with fever of longer duration (5-20 days) plus abdominal pain, characteristic rash, conjunctivitis and periorbital edema
  • 25% develop amyloidosis with renal or cardiac dz.
Look forward to a more extensive review of TRAPS. There will be a new case challenge next week!