PMID- 9294526
OWN - NLM
STAT- MEDLINE
DCOM- 19971001
LR  - 20190516
IS  - 0025-6196 (Print)
IS  - 0025-6196 (Linking)
VI  - 72
IP  - 9
DP  - 1997 Sep
TI  - Clinical spectrum of familial Hibernian fever: a 14-year follow-up study of the 
      index case and extended family.
PG  - 806-17
AB  - OBJECTIVE: To determine the clinical spectrum and natural history of the disease 
      "familial Hibernian fever" (FHF). DESIGN: We ascertained the disease status in 
      all 54 living members and 9 deceased members of the extended family and conducted 
      a detailed study of those affected. MATERIAL AND METHODS: All family members with 
      FHF were clinically assessed and investigated fully, including human leukocyte 
      antigen (HLA) typing. Medical records were studied for relevant clinical 
      features, drug therapy, and complications. All previously obtained histologic 
      specimens were reviewed. Three typical case histories are presented. RESULTS: The 
      updated family tree confirmed an autosomal dominant mode of inheritance in 16 
      living members with FHF. In addition to the febrile attacks, abdominal pain and 
      localized myalgias were almost invariably present. Episodic erythematous patches, 
      conjunctivitis, and unilateral periorbital edema were also notable features. Of 
      10 affected male family members, 8 had inguinal hernias (in comparison with 1 of 
      21 unaffected male family members). No association with HLA status was noted. 
      Secondary amyloidosis was found in one affected member. CONCLUSION: The 
      characteristic clinical features and natural history of FHF distinguish it from 
      other periodic fever syndromes. The discovery of amyloidosis related to FHF 
      alters the prognosis associated with this condition and emphasizes the need to 
      search for effective treatment strategies. The high prevalence of inguinal 
      herniation may provide clues about its pathogenesis.
FAU - McDermott, E M
AU  - McDermott EM
AD  - Clinical Immunology Unit, Queen's Medical Centre, Nottingham, United Kingdom.
FAU - Smillie, D M
AU  - Smillie DM
FAU - Powell, R J
AU  - Powell RJ
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Mayo Clin Proc
JT  - Mayo Clinic proceedings
JID - 0405543
SB  - IM
MH  - Adult
MH  - Diagnosis, Differential
MH  - Female
MH  - Fever of Unknown Origin/etiology/*genetics
MH  - Humans
MH  - Male
MH  - Pedigree
MH  - Precipitating Factors
EDAT- 1997/09/19 00:00
MHDA- 1997/09/19 00:01
CRDT- 1997/09/19 00:00
PHST- 1997/09/19 00:00 [pubmed]
PHST- 1997/09/19 00:01 [medline]
PHST- 1997/09/19 00:00 [entrez]
AID - S0025-6196(11)63494-0 [pii]
AID - 10.4065/72.9.806 [doi]
PST - ppublish
SO  - Mayo Clin Proc. 1997 Sep;72(9):806-17. doi: 10.4065/72.9.806.