ReviewMediterranean journal of hematology and infectious diseases2018
The Broad-Ranging Panorama of Systemic Autoinflammatory Disorders with Specific Focus on Acute Painful Symptoms and Hematologic Manifestations in Children.
Review in Mediterranean journal of hematology and infectious diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- Surgical management for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: a systematic review and meta-analysis.Jornal de pediatriaPooled it
- Highlights on the Contribution of Gut Microbiota to Immune-Mediated Diseases in Childhood.Mediterranean journal of hematology and infectious diseases · 2026Review
- R202Q prevalence in clinically diagnosed Familial Mediterranean Fever patients: 9 years of data analysis from 1570 patients living Central Black Sea region, Turkey.Irish journal of medical science · 2023Article
- Current Evidence on Vaccinations in Pediatric and Adult Patients with Systemic Autoinflammatory Diseases.Vaccines · 2023Review
- Review
- Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management.Mediterranean journal of hematology and infectious diseases · 2022Review
- The everchanging framework of autoinflammation.Internal and emergency medicine · 2021Review
- Overview of the rarest causes of fever in newborns: handy hints for the neonatologist.Journal of perinatology : official journal of the California Perinatal Association · 2021Review
- Children and Adults with PFAPA Syndrome: Similarities and Divergences in a Real-Life Clinical Setting.Advances in therapy · 2021Article
- [Clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: an analysis of 13 cases].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2021Article
- Efficacy of anti-interleukin-1 treatment in colchicine-resistant arthritis in patients with familial Mediterranean fever.European journal of rheumatology · 2021Article
- Chronic Ulcers and Malnutrition in an African Patient.Pediatrics · 2020Article
- The importance of Mediterranean fever gene in familial Mediterranean fever.European journal of rheumatology · 2020Article
- Kawasaki Disease as the Immune-Mediated Echo of a Viral Infection.Mediterranean journal of hematology and infectious diseases · 2020Review
- Interleukin-1 Blockade in Polygenic Autoinflammatory Disorders: Where Are We now?Frontiers in pharmacology · 2020Review
- Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report.Mediterranean journal of hematology and infectious diseases · 2020Article
- Novel Deleterious Sequence Change in the NLRP12 Gene in a Child with the Autoinflammatory Syndrome, Joint Hypermobility and Cutis Laxa from India.Mediterranean journal of hematology and infectious diseases · 2019Article
- Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases.Mediators of inflammation · 2019Review
- Familial Mediterranean Fever: Assessing the Overall Clinical Impact and Formulating Treatment Plans.Mediterranean journal of hematology and infectious diseases · 2019Review
- The Gut Microbiota-Host Partnership as a Potential Driver of Kawasaki Syndrome.Frontiers in pediatrics · 2019Review
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Systemic autoinflammatory disorders (SAIDs) are inherited defects of innate immunity characterized by recurrent sterile inflammatory attacks involving skin, joints, serosal membranes, gastrointestinal tube, and other tissues, which recur with variable rhythmicity and display reactive amyloidosis as a potential long-term complication. Dysregulated inflammasome activity leading to overproduction of many proinflammatory cytokines, such as interleukin-1 (IL-1), and delayed shutdown of inflammation are considered crucial pathogenic keys in the vast majority of SAIDs. Progress of cellular biology has partially clarified the mechanisms behind monogenic SAIDs, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome, mevalonate kinase deficiency, hereditary pyogenic diseases, idiopathic granulomatous diseases and defects of the ubiquitin-proteasome pathway. Whereas, little is clarified for the polygenic SAIDs, such as periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. The puzzle of symptomatic febrile attacks recurring over time in children requires evaluating the mixture of clinical data, inflammatory parameters in different disease phases, the therapeutic efficacy of specific drugs such as colchicine, corticosteroids or IL-1 antagonists, and genotype analysis in selected cases. The long-term history of periodic fevers should also need to rule out chronic infections and malignancies. This review is conceived as a practical template for proper classification of children with recurring fevers and includes tips useful for the diagnostic approach to SAIDs, focusing on the specific acute painful symptoms and hematologic manifestations encountered in childhood.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.