[HTML][HTML] The clinical chameleon of autoinflammatory diseases in children

E Sangiorgi, D Rigante - Cells, 2022 - mdpi.com
The very first line of defense in humans is innate immunity, serving as a critical strongpoint in
the regulation of inflammation. Abnormalities of the innate immunity machinery make up a …

[HTML][HTML] Familial Mediterranean fever: assessing the overall clinical impact and formulating treatment plans

R Manna, D Rigante - Mediterranean journal of hematology and …, 2019 - ncbi.nlm.nih.gov
Recurrent self-limited attacks of fever and short-lived inflammation in the serosal
membranes, joints, and skin are the leading features of familial Mediterranean fever (FMF) …

[PDF][PDF] Limiting cholesterol biosynthetic flux spontaneously engages type I IFN signaling

AG York, KJ Williams, JP Argus, QD Zhou, G Brar… - Cell, 2015 - cell.com
Cellular lipid requirements are achieved through a combination of biosynthesis and import
programs. Using isotope tracer analysis, we show that type I interferon (IFN) signaling shifts …

A systematic approach to autoinflammatory syndromes: a spelling booklet for the beginner

D Rigante - Expert Review of Clinical Immunology, 2017 - Taylor & Francis
Introduction: Hallmark of autoinflammatory syndromes (AIS) is the periodic recurrence of
'sterile'inflammatory attacks characterized by fever and organ-or tissue-specific …

A comprehensive overview of the hereditary periodic fever syndromes

D Rigante, B Frediani, L Cantarini - Clinical reviews in allergy & …, 2018 - Springer
Innate immunity is a critical partner in the regulation of inflammation and some mutations in
genes implied in innate immunity pathways can cause genetic disorders characterized by …

[HTML][HTML] The everchanging framework of autoinflammation

R Manna, D Rigante - Internal and Emergency Medicine, 2021 - Springer
The innate immunity works as a defence bullwark that safeguards healthy tissues with the
power of detecting infectious agents in the human body: errors in the context of innate …

[HTML][HTML] The broad-ranging panorama of systemic autoinflammatory disorders with specific focus on acute painful symptoms and hematologic manifestations in …

D Rigante - Mediterranean Journal of Hematology and Infectious …, 2018 - ncbi.nlm.nih.gov
Systemic autoinflammatory disorders (SAIDs) are inherited defects of innate immunity
characterized by recurrent sterile inflammatory attacks involving skin, joints, serosal …

[HTML][HTML] Assessment of congenital neutropenia in children: common clinical sceneries and clues for management

I Lazzareschi, E Rossi, A Curatola… - … of Hematology and …, 2022 - ncbi.nlm.nih.gov
A disparate group of rare hematological diseases characterized by impaired maturation of
neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to …

[HTML][HTML] Hints for genetic and clinical differentiation of adult-onset monogenic autoinflammatory diseases

C Gaggiano, D Rigante, A Vitale… - Mediators of …, 2019 - hindawi.com
Monogenic autoinflammatory diseases (mAIDs) are inherited errors of innate immunity
characterized by systemic inflammation recurring with variable frequency and involving the …

[HTML][HTML] The pyrin inflammasome, a leading actor in pediatric autoinflammatory diseases

S La Bella, A Di Ludovico, G Di Donato… - Frontiers in …, 2024 - frontiersin.org
The activation of the pyrin inflammasome represents a highly intriguing mechanism
employed by the innate immune system to effectively counteract pathogenic agents. Despite …