Renal amyloidosis in children

Abstract

Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases. Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number of new treatments for amyloid per se are in the pipeline.

Serum Amyloid A Level in Egyptian Children with Familial Mediterranean Fever

“…These entire laboratory parameters usually return to normal in the attack-free periods [7]. However, it has been reported that subclinical inflammation may continue during the attack-free periods in some patients and may lead to the development of amyloidosis [8, 9] that maybe complicated by end-stage renal disease [10]. …”

Is Neutrophil/Lymphocyte Ratio Associated with Subclinical Inflammation and Amyloidosis in Patients with Familial Mediterranean Fever?

“…This type of subclinical inflammation has a chronic course and leads to the development of the most mortal complication named amyloidosis. And amyloidosis can be controlled with the inhibition of this inflammation [6]. …”

Cochlear involvement in Familial Mediterranean Fever: A new feature of an old disease

“…Vasculitis is a newly defined entity in FMF [4,9,10]. Cochlear involvement has been shown to be a component of the diseases in which vasculitis is predominant in the physiopathological mechanism.…”

“…Since vasculitic diseases and amyloidosis are seen frequently during the course of FMF and the disease involves many organs as kidneys, central nervous system, heart, respiratory and gastrointestinal tracts by amyloidosis or vasculitis, cochleovestibular involvement related to the course of the disease is probable [4,5]. To our knowledge, otological involvement in FMF has not been studied until now.…”