AA amyloidosis complicating hyperimmunoglobulinemia D with periodic fever syndrome: A report of two cases

2006 ◽  
Vol 54 (6) ◽  
pp. 2010-2014 ◽  
Author(s):  
Helen J. Lachmann ◽  
Hugh J. B. Goodman ◽  
Peter A. Andrews ◽  
Hugh Gallagher ◽  
James Marsh ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Aa Amyloidosis

scholarly journals MO961KIDNEY TRANSPLANTATION AS A TREATMENT OF CHOICE FOR AA AMYLOIDOSIS DUE TO PERIODIC FEVER SYNDROME

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Ivana Dedinska ◽  
Karol Graňák ◽  
Matej Vnučák
Keyword(s):  
Early Childhood ◽  
Kidney Transplantation ◽  
Clinical Course ◽  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Aa Amyloidosis ◽  
Periodic Fever Syndromes ◽  
Post Transplant ◽  
Fever Syndromes ◽  
End Stage

Abstract Background and Aims Renal AA amyloidosis is the most serious complication of periodic fever syndrome, which, inadequate suppression, due to persistent inflammation, leads to nephrotic syndrome and renal failure over several years. In most cases, periodic fever syndromes begin to manifest clinically in early childhood. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment for end-stage chronic kidney disease (CKD) based on AA amyloidosis. Method We present cases of two patients after deceased donor kidney transplantation, who have been diagnosed with adult periodic fever syndrome. Conclusion Periodic fever syndromes are diseases beginning to manifest clinically in early childhood in 60–90% of cases, most often in the case of familial Mediterranean fever around 4 years of age, in the case of CAPS usually only a few months after birth. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment of end-stage CKD based on AA amyloidosis associated with periodic fever syndrome. Adequate targeted treatment against IL-1 or TNF is important and appears to be safe during the post-transplant period, with regular monitoring of renal function, acute phase inflammatory reactants, and histological findings by protocol graft biopsies. It will be important and necessary to assess the development of diseases in the post-transplant period in the long term.

scholarly journals First report of systemic reactive (AA) amyloidosis in a patient with the hyperimmunoglobulinemia D with periodic fever syndrome

2004 ◽  
Vol 50 (9) ◽  
pp. 2966-2969 ◽  
Author(s):  
Laura Obici ◽  
Carlo Manno ◽  
Andrea Onetti Muda ◽  
Paolo Picco ◽  
Andrea D'Osualdo ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Aa Amyloidosis ◽  
First Report

Kidney transplantation as a treatment of choice for AA amyloidosis due to periodic fever syndrome

2020 ◽  
Author(s):  
K. Graňák ◽  
M. Jeseňák ◽  
Matej Vnučák ◽  
P. Skálová ◽  
Ľ. Laca ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Aa Amyloidosis

scholarly journals A Novel Unstable Duplication Upstream of HAS2 Predisposes to a Breed-Defining Skin Phenotype and a Periodic Fever Syndrome in Chinese Shar-Pei Dogs

PLoS Genetics ◽  
2011 ◽  
Vol 7 (3) ◽  
pp. e1001332 ◽  
Author(s):  
Mia Olsson ◽  
Jennifer R. S. Meadows ◽  
Katarina Truvé ◽  
Gerli Rosengren Pielberg ◽  
Francesca Puppo ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Periodic Fever Syndrome

Making a diagnosis of periodic fever syndrome: Experience from a single tertiary centre

2021 ◽  
Author(s):  
Peter McNaughton ◽  
Sophie Willcocks ◽  
Su Han Lum ◽  
Ben Whitehead ◽  
Jane Peake ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Tertiary Centre

TNFRSF1A Mutations in Italian Patients Affected by Apparently Sporadic Periodic Fever Syndrome

2004 ◽  
pp. 182-184
Author(s):  
G Palladini ◽  
L Obici ◽  
G Merlini ◽  
S Marciano ◽  
F Lavatelli ◽  
...  
Keyword(s):  
Periodic Fever ◽  
Periodic Fever Syndrome

scholarly journals Two cases of periodic fever syndrome with coexistent mevalonate kinase and mediterranean fever gene mutations

2017 ◽  
Vol 59 (4) ◽  
pp. 467 ◽  
Author(s):  
Mustafa Çakan ◽  
Nuray Aktay-Ayaz ◽  
Gonca Keskindemirci ◽  
Şerife Gül Karadağ
Keyword(s):  
Periodic Fever ◽  
Gene Mutations ◽  
Mevalonate Kinase ◽  
Periodic Fever Syndrome ◽  
Mediterranean Fever
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