Paeds Cases · rheumatology-musculoskeletal-and-sports
Periodic fever and autoinflammatory syndromes — OSCE
OSCE communication and clinical reasoning station for a six-year-old of Lebanese background with familial Mediterranean fever, and parents frightened by the father's amyloidosis and renal transplant.
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MRCPCH ClinicalRACP DCE
Prompt
You have 8 minutes with the parents of a six-year-old boy of Lebanese background who has had two-day attacks of fever, severe abdominal pain and a swollen knee for two years, and is completely well between attacks. His father had a renal transplant at thirty-two for amyloidosis. Explain the likely diagnosis, the lifelong treatment, the surveillance for amyloidosis, and the genetic implications, and agree a clear plan and safety-net.
Station brief (candidate)
- Acknowledge the parents' fear, given the father's own experience of amyloidosis and a renal transplant, and validate it as entirely reasonable.
- Explain that the pattern of short attacks of fever with the abdominal pain and the swollen knee, in a child of Lebanese background who is completely well between attacks, points to familial Mediterranean fever, the same condition that affected the father.
- Outline the management: a lifelong daily tablet called colchicine that reduces the attacks and is the only treatment proven to prevent the amyloidosis that caused the father's kidney failure, with regular monitoring of the urine for protein and the kidney function.
- Explain the genetic and the family implications honestly: it is inherited, it runs in families, and there are reproductive considerations for the future.
- Agree a clear, specific safety-net for when to return immediately (new persistent swelling, severe abdominal pain that does not settle, or any change in the urine). [3][4]
References3ShowHide
- [3]Livneh A, Langevitz P, Zemer D, et al Criteria for the diagnosis of familial Mediterranean fever Arthritis Rheum, 1997.PMID 9336425
- [4]Ozen S, Demirkaya E, Erer B, et al EULAR recommendations for the management of familial Mediterranean fever Ann Rheum Dis, 2016.PMID 26802180
- [1]Gattorno M, Hofer M, Federici S, et al Classification criteria for autoinflammatory recurrent fevers Ann Rheum Dis, 2019.PMID 31018962