Amyloidosis in a child with leucocyte adhesion deficiency type-1: an unusual association.

Indian J Pediatr

Allergy-Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.

Published: December 2011

Leucocyte adhesion deficiency type I (LAD I) is a rare autosomal recessive disorder of leucocyte function, characterized by delayed separation of the umblical cord, recurrent bacterial and fungal infections, defective wound healing and impressive constant blood neutrophilia. The authors report a child with genetic diagnosis of leucocyte adhesion deficiency (LAD) type I, who succumbed to his illness and developed amyloidosis preterminally. To the best of author's knowledge this is the first case of leucocyte adhesion deficiency type I associated with amyloidosis among the human LAD cases worldwide.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-011-0417-xDOI Listing

Publication Analysis

Top Keywords

leucocyte adhesion
16
adhesion deficiency
16
deficiency type
8
leucocyte
5
amyloidosis child
4
child leucocyte
4
adhesion
4
deficiency
4
deficiency type-1
4
type-1 unusual
4

Similar Publications

Erythrodermic psoriasis (EP) is a life-threatening variant of psoriasis. In this study, we contrasted the vascular endothelial cells (ECs) in EP lesions against those in psoriasis vulgaris and healthy controls. Utilizing single-cell RNA sequencing, immunofluorescence, and flow cytometry on human and mouse samples, we observed a marked increase and activation of EP ECs, which upregulated genes relative to angiogenesis, leukocyte adhesion and antigen presentation.

View Article and Find Full Text PDF

Background: Neutrophils are the most abundant leukocytes in human blood, and their recruitment is essential for innate immunity and inflammatory responses. The initial and critical step of neutrophil recruitment is their adhesion to vascular endothelium, which depends on G protein-coupled receptor (GPCR) triggered integrin inside-out signaling that induces β2 integrin activation and clustering on neutrophils. Kindlin-3 and talin-1 are essential regulators for the inside-out signaling induced β2 integrin activation.

View Article and Find Full Text PDF

Despite the unique properties of clay nanocomposites for cardiovascular applications, there are few data on the hemocompatibility of these nanomaterials. This study represents the first comprehensive investigation of the hemo/biocompatibility of clay nanocomposites . Nanocomposite coatings of polylactic acid (PLA)-polyethylene glycol (3 wt %)-Cloisite20A nanoclay (3 wt %) were produced using electrospraying technique as potential drug-eluting stent (DES) coatings.

View Article and Find Full Text PDF

Review on critical factor in monocyte adhesion: Nutrients.

Cytokine

January 2025

Department of Nutrition and Dietetics, Faculty of Health Sciences, Gazi University, Ankara 06490, Türkiye.

Endogenous and exogenous factors play a role in endothelial dysfunction. Inflammation, leukocyte adhesion-aggregation, abnormal vascular proliferation, atherosclerosis, and hypertension are among the endogenous factors. Another factor that affects endothelial dysfunction is exogenous factors such as drug treatments, smoking, alcohol, and nutrition.

View Article and Find Full Text PDF

The Integrin Receptors: From Discovery to Structure to Medicines.

Immunol Rev

December 2024

Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Innate immune cells perform vital tasks in detecting, seeking, and eliminating invading pathogens, thus ensuring host survival. However, loss of function of these cells or their overactive response to tissue injury often causes serious ailments. It is, therefore, crucial to understand at a basic level how these cells function in health and disease.

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!