Cystic fibrosis and AA amyloidosis: a survey in the French cystic fibrosis network

Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries. Some studies offer insights into pubertal growth and non-pulmonary complications, such as liver disease, nephropathy, and cancer. Pediatr Pulmonol. 2017;52:390-398. © 2016 Wiley Periodicals, Inc.

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“…French registry gave information about nine cases of AA Amyloidosis . In these patients mainly the kidneys were affected.…”

Section: Discussionmentioning

confidence: 99%

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore

Buzzetti

Mastella

2016

Pediatric Pulmonology

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“…Regarding other causes, some genetic diseases responsible for recurrent pulmonary infections can be complicated by AA amyloidosis, in part because of secondary bronchiectasis. Therefore, more than 50 cases of AA amyloidosis associated with cystic fibrosis have been reported 108‐115 . Amyloidosis was found in 0.5% of patients with cystic fibrosis in an Italian study in 1985, 116 accounting for approximately one‐quarter of the causes of kidney diseases 112 and for 1.3% of the causes of death in cystic fibrosis in Italy 117 .…”

Section: Infections Associated With Aa Amyloidosismentioning

confidence: 99%

Infections and AA amyloidosis: An overview

et al. 2021

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Background Amyloidoses are a heterogeneous group of systemic diseases characterised by extracellular accumulation of insoluble amyloid fibrils derived from unfolded proteins. Inflammatory (AA) amyloidosis can complicate various inflammatory disorders that are associated with a sustained acute phase response and serum amyloid A (SAA) protein overproduction. Chronic infections were the first recognised cause of amyloidoses. However, with the better management of underlying diseases, the frequency of AA amyloidosis is decreasing. Purpose The aim of this overview was to discuss the several infections associated with AA amyloidosis and the relative frequency of infections as aetiological factors. Methods A search of the literature was performed using the PubMed database using the MeSH terms “Amyloidosis” and “Infections,” from inception to December 31st, 2019. Articles written in other languages than English or French were excluded. Results The frequency of AA amyloidosis secondary to infections decreased from more than 50% to less than 20% after the 2000s, with a parallel increase in the frequency of AA amyloidosis secondary to inflammatory diseases and to an unknown cause. Conclusion Whereas new antibiotics have been developed and sanitary conditions are better, infections still represent 5%‐30% of the causes of AA amyloidosis, including in developed countries. These data argue for better screening of chronic infections to prevent AA amyloidosis and the development of new strategies to manage recurrent infections.

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Impact of CF on the Kidneys

Prayle

Quon

2020

Respiratory Medicine

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Cystic fibrosis and AA amyloidosis: a survey in the French cystic fibrosis network

Abstract: AA amyloidosis is a rare but morbid complication of CF. Renal involvement is predominant.

Cited by 8 publications

References 23 publications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Infections and AA amyloidosis: An overview

Impact of CF on the Kidneys

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