Amyloidosis is a rare multisystem disorder characterized by extracellular accumulation of insoluble fibrils in various organs and tissues. The most common subtype in the pediatric population is systemic reactive amyloidosis, typically developing secondary to chronic inflammatory conditions and resulting in deposition of serum amyloid A protein in association with apolipoprotein HDL3. Clinical presentation is highly variable and is mostly influenced by specific organs involved, precursor protein type, and extent of amyloid deposition, often closely reflecting clinical features of the underlying disease. The most critical determinants of prognosis are cardiac and renal involvement. Diagnosis of amyloidosis is confirmed by tissue biopsy, which remains the gold standard, followed by precise amyloid fibril typing. The primary therapeutic approach is directed towards controlling underlying disease and reducing serum levels of precursor proteins to prevent further amyloid deposition. This study aims to highlight the main clinical characteristics of amyloidosis with onset in childhood, emphasizing the key differences compared to adult form.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11594867PMC
http://dx.doi.org/10.3390/jcm13226682DOI Listing

Publication Analysis

Top Keywords

clinical features
8
amyloid deposition
8
underlying disease
8
amyloidosis
5
amyloidosis childhood
4
childhood review
4
clinical
4
review clinical
4
features comparison
4
comparison adult
4

Similar Publications

The aim of this study is to investigate the impact of using probiotics with strains related to dopamine and gamma-aminobutyric acid production on clinical features of autism spectrum disorder (ASD) and/or attention deficit/hyperactivity disorder (ADHD). This randomized, controlled trial involved 38 children with ADHD and 42 children with ASD, aged 5-16 years, who received probiotics (Lactiplantibacillus plantarum and Levilactobacillus brevis 109/cfu/daily) or placebo for 12 weeks. Parent-reported symptoms were assessed using Conners' 3rd-Ed and the Social Responsiveness Scale Test, 2nd-Ed (SRS-2), and children completed the Conners Continuous Performance Test, 3rd-Ed (CPT 3) or Conners Kiddie CPT, 2nd-Ed (K-CPT 2).

View Article and Find Full Text PDF

IL-33, a neutrophil extracellular trap-related gene involved in the progression of diabetic kidney disease.

Inflamm Res

January 2025

Department of Nephrology, First Affiliated Hospital of Naval Medical University, Shanghai Changhai Hospital, Shanghai, China.

Background: Chronic inflammation is well recognized as a key factor related to renal function deterioration in patients with diabetic kidney disease (DKD). Neutrophil extracellular traps (NETs) play an important role in amplifying inflammation. With respect to NET-related genes, the aim of this study was to explore the mechanism of DKD progression and therefore identify potential intervention targets.

View Article and Find Full Text PDF

Purpose: This case report aims to present a rare case of endometrial carcinosarcoma, a highly malignant tumor with a poor prognosis. The primary objective is to describe this unique case's clinical presentation, multimodal magnetic resonance imaging (MRI) features, typical histopathological characteristics and surgical treatment.

Methods: A detailed analysis of the patient's medical history, preoperative imaging evaluation, and treatment approach was conducted.

View Article and Find Full Text PDF

Microglial polarization and ferroptosis are important pathological features in Alzheimer's disease (AD). Ghrelin, a brain-gut hormone, has potential neuroprotective effects in AD. This study aimed to explore the potential mechanisms by which ghrelin regulates the progression of AD, as well as the crosstalk between microglial polarization and ferroptosis.

View Article and Find Full Text PDF

Background: The mechanism underlying chronic drug-induced liver injury (DILI) remains unclear. Immune activation is a common feature of DILI progression and is closely associated with metabolism. We explored the immunometabolic profile of chronic DILI and the potential mechanism of chronic DILI progression.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!