Hematological Indices in Pediatric Diagnosed with Familial Mediterranean Fever

NCT ID: NCT06583304

Last Updated: 2024-09-04

Basic Information

• Recruitment Status: NOT_YET_RECRUITING
• Total Enrollment: 93 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2024-10-13
• Study Completion Date: 2026-03-31

Brief Summary

To investigate the relationship between the hematological indices and subclinical inflammation in FMF patients during their attack-free period, additionally this study aims to assess the potential of these indices as predictive markers for ongoing inflammation in these patients population at Assuit university hospital of pediatrics.

Detailed Description

Familial Mediterranean fever (FMF) is the most common hereditary auto inflammatory disease in the world. FMF is caused by gain of function mutations in the MEFV gene, which encodes a protein called pyrin which has regulatory functions on the innate immune system. [1] Mutations interfere with the function of the pyrin domain, initiating an uninterrupted inflammatory cascade [2] Familial Mediterranean fever affects the populations located on the Mediterranean basin. It is virtually limited to Armenians, Turks, Arabs and nonAshkenazi Jews. However, some cases have been described in European countries, including Italy, France, Spain, Portugal and Greece. [3] The clinical presentation of FMF can be variable, likely depending on its genetic heterogeneity and environmental factors. However, the clinical picture is usually very suggestive of the underlying disease. It is typically characterized by recurrent episodes of fever and systemic inflammation with (pleural and peritoneal) serositis and arthritis. Starting in childhood, patients present shortlasting, self-resolving attacks of fever and abdominal, chest, or joint pain with systemic inflammation. Periodicity is not strict and episodes may occur from once a week up to once every three to four months or more in untreated patients. These events are usually very disabling and in clear contrast with complete well-being in attack-free periods [4]. The diagnosis of FMF based on clinical findings is supported by ethnic origin, family history and genetic testing. Several criteria had been developed for the diagnosis of FMF: the most widely used Tel-Hashomer criteria, Most recently, adult and pediatric rheumatology experts have created a new set of classifcation criteria (Eurofever/PRINTO classifcation criteria) [5].

During FMF attacks, many acute phase reactants such as C-reactive protein (CRP), serum amyloid A, fibrinogen, and erythrocyte sedimentation rate (ESR) increase. It is known that a subclinical inflammation continues in FMF patients during the inter attack period and this increases the risk of developing amyloidosis. [6]. Therefore, the use of additional serum markers to evaluate disease activity may provide beneficial advantages for monitoring and preventing further complications. Systemic inflammation also causes changes in the number and composition of circulating blood cells [7] [8]. Neutrophils, lymphocytes and platelets play major roles in systemic inflammation [9]. Neutrophils and platelets secrete proinflammatory cytokines that augment of more neutrophils and platelets [10]. Microparticles released from activated platelets interact with neutrophils through platelet-like lipoxygenase expression and activation of eicosanoid pathway [11]. The platelet count, mean platelet volume (MPV) are the most important indices of platelet function and activation . MPV, a component of routine complete blood count (CBC) tests, is inversely affected by systemic inflammation. MPV levels were shown to be lower in active inflammatory bowel diseases, rheumatoid arthritis (RA) and ankylosing spondylitis (AS) and increased MPV values were reported after treatment. Additionally, the neutrophil-to-lymphocyte ratio (NLR) and the platelet-to-lymphocyte ratio (PLR), which are easily obtained from differential CBC, have been shown to not only increase in inflammation, but also, be correlated with disease activity and prognosis [12] We discuss the clinical criteria and the association of hematological indices with subclinical inflammation and to evaluate their usability as predictors of ongoing inflammation in FMF patients during their attack-free period

Conditions

Familial Mediterranean Fever (FMF )

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: CROSS_SECTIONAL

Eligibility Criteria

Inclusion Criteria

1. Age at enrollment less than18 years.

2. Both sexes.

3. Diagnosed as familial Mediterranean fever according to Tel-Hashomer criteria in pediatrics and Eurofever /PRINTO criteria

Exclusion Criteria

1- Patients with acute or chronic active infections. 2- Patients with pre-existing illness as chronic lung, liver or renal diseases. 3- Patients with diabetes mellitus, atherosclerotic vascular disease. 4- Patients with malignancy

-

• Minimum Eligible Age: 1 Year
• Maximum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Assiut University

OTHER

Sponsor Role: lead

Responsible Party

Khaled Mohamed Ahmed Aboelsaud

71515,Assiut

Responsibility Role: PRINCIPAL_INVESTIGATOR

Central Contacts

khaled mohamed Ahmed Aboelsaud, Bachelor of Medicine

Role: CONTACT

Km5651466@gmail.com

01111520361

Naglaa Samy Mohamed Osman, Lecturer of Pediatrics

Role: CONTACT

Naglaaosman84@edu.eg

01002673103

References

Lancieri M, Bustaffa M, Palmeri S, Prigione I, Penco F, Papa R, Volpi S, Caorsi R, Gattorno M. An Update on Familial Mediterranean Fever. Int J Mol Sci. 2023 May 31;24(11):9584. doi: 10.3390/ijms24119584.

Reference Type: BACKGROUND

PMID: 37298536 (View on PubMed)

Assouad E, El Hage S, Safi S, El Kareh A, Mokled E, Salameh P. Familial Mediterranean fever research activity in the Arab world: the need for regional and international collaborations. East Mediterr Health J. 2021 Oct 27;27(10):984-992. doi: 10.26719/emhj.21.036.

Reference Type: BACKGROUND

PMID: 34766324 (View on PubMed)

Tufan A, Lachmann HJ. Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review. Turk J Med Sci. 2020 Nov 3;50(SI-2):1591-1610. doi: 10.3906/sag-2008-11.

Reference Type: BACKGROUND

PMID: 32806879 (View on PubMed)

Other Identifiers

familial Mediterranean fever

Identifier Type: -

Identifier Source: org_study_id