Skip to main content
Erschienen in: Pediatric Rheumatology 1/2013

Open Access 01.11.2013 | Meeting abstract

PW01-027 – Predictors and survival of FMF related amyloidosis

verfasst von: E Demirkaya, I Yilmaz, C Acikel, M Saglam, H Unal, M Gok, A Polat, H Cetinkaya, T Eyileten, S Sari, AO Yildirim, Y Oguz, A Vural, JJ Carrero, FMF Arthritis Vasculitis and Orphan disease Research in Pediatric Rheumatology (FAVOR)

Erschienen in: Pediatric Rheumatology | Sonderheft 1/2013

download
DOWNLOAD
print
DRUCKEN
insite
SUCHEN

Introduction

Amiloidosis is most fatal complication of FMF. Former studies recognize that endothelial functions were severely impaired in patients who have amyloidosis than the patients who have other glomerulopathies. Asymmetric dimethyl arginine (ADMA), the endogenous inhibitor of nitric oxide synthase is possibly a causative or predictive factor in endothelial dysfunction in humans.

Objectives

Compare the amyloidosis group to only proteinuria group for biochemical, demographic and some other features such as flow-mediated dilation (FMD), to understand which markers may affect or help prediction of amyloidosis. We also evaluated the effects of elevated ADMA levels and impaired FMD responses on the survival time of CVD free period in two distinct groups with severe proteinuria, secondary amyloidosis (SA) versus primary glomerulopathy. We proposed that increased ADMA synthesis in amyloidosis induced endothelial damage may contribute part of the mechanism by which proteinuria increases cardiovascular morbidity and mortality.

Methods

Study was part of a cohort study. The amiloidosis and proteiuria groups were followed up for predictive factors. All enrolled subjects were evaluated by standard physical examination, chest X-ray, baseline electrocardiogram, two-dimensional echocardiography, and routine biochemical laboratory tests, including liver and kidney function tests and 24-hour urinary protein measurements. FMD and venous blood samples were taken following a 2 week wash-out period, during which time no vasoactive drugs (including colchicines) were given. Measurements of serum ADMA and SDMA were done using HPLC.

Results

The data of 102 patients with proteinuria due to primary glomerulopathy and 98 patients with amyloidosis due to FMF were assessed. Median age of diagnosis in patients with amyloidosis was 16 (min.-max.: 6-25) and 71.4 % of patients were 18 or younger at the date of diagnoses of amyloidosis. Patients with amyloidosis provided higher levels of SDMA and ADMA (p < 0.01) and lower FMD percentage when compared to patients with glomerulonephritis (p < 0.01). Inflammatory markers such as high sensitivity C-reactive protein (hsCRP) and pentraxim-3 were statistically different between groups and higher among patients with amyloidosis.

Conclusion

Inflammatory markers such as hsCRP and pentraxim-3 were statistically different between groups and higher among patients with amyloidosis. The mortality and the cardiovascular event rate was much higher in patients with secondary amyloidosis.

Disclosure of interest

None declared
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://​creativecommons.​org/​licenses/​by/​2.​0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Metadaten
Titel
PW01-027 – Predictors and survival of FMF related amyloidosis
verfasst von
E Demirkaya
I Yilmaz
C Acikel
M Saglam
H Unal
M Gok
A Polat
H Cetinkaya
T Eyileten
S Sari
AO Yildirim
Y Oguz
A Vural
JJ Carrero
FMF Arthritis Vasculitis and Orphan disease Research in Pediatric Rheumatology (FAVOR)
Publikationsdatum
01.11.2013
Verlag
BioMed Central
Erschienen in
Pediatric Rheumatology / Ausgabe Sonderheft 1/2013
Elektronische ISSN: 1546-0096
DOI
https://doi.org/10.1186/1546-0096-11-S1-A80

Weitere Artikel der Sonderheft 1/2013

Pediatric Rheumatology 1/2013 Zur Ausgabe

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

23.04.2024 Typ-1-Diabetes Nachrichten

Wenn Kinder an Werktagen zum Arzt gehen, werden neu auftretender Typ-1-Diabetes und diabetische Ketoazidosen häufiger erkannt als bei Arztbesuchen an Wochenenden oder Feiertagen.

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

22.04.2024 Fehlsichtigkeit Nachrichten

Augentropfen mit niedrig dosiertem Atropin können helfen, das Fortschreiten einer Kurzsichtigkeit bei Kindern zumindest zu verlangsamen, wie die Ergebnisse einer aktuellen Studie mit verschiedenen Dosierungen zeigen.

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

18.04.2024 Spinale Muskelatrophien Nachrichten

Seit 2021 ist die Untersuchung auf spinale Muskelatrophie Teil des Neugeborenen-Screenings in Deutschland. Eine Studie liefert weitere Evidenz für den Nutzen der Maßnahme.

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

18.04.2024 Pädiatrische Notfallmedizin Nachrichten

Im Choosing-Wisely-Programm, das für die deutsche Initiative „Klug entscheiden“ Pate gestanden hat, sind erstmals Empfehlungen zum Umgang mit Notfällen von Kindern erschienen. Fünf Dinge gilt es demnach zu vermeiden.

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.