Clinical and Molecular Evaluation of Childern With Familial Meditterranean Fever and Their Siblings

Familial Mediterranean Fever Clinical Trial

Official title:

Clinical and Molecular Evaluation of Childern With Familial Meditterranean Fever and Their Siblings

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT05488561
• Other study ID #: Soh_Med_22_07_20
• Status: Not yet recruiting
• Phase: N/A
• Start date: August 15, 2022
• Est. completion date: August 2023

Study information

• Verified date: August 2022
• Source: Sohag University
• Contact: nahla a fawy, resident
• Phone: 01015681398
• Email: nahlaabdelaziz[@]med.sohag.edu.eg
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

Familial Mediterranean fever (FMF ,recurrent polyserositis ,periodic disease) is an autosomal recessive auto inflammatory disease which primarily affect population surrounding the Mediterranean basin (Arabs , Turks ,Armenians, Jews ).Despite its striking symptoms pattern FMF was first described as distinct entity only in 1945. It is characterized by recurrent attacks of fever , peritonitis ,pleurisy , arthritis , or erysipelas like skin disease. The most dangerous complication of this disease is secondary amyloidosis . FMF diagnosis is mainly clinical, and the genetic testing is indicated to support it . Uncommonly, amyloidosis may develop in individuals carrying two Familial Mediterranean fever gene (MEFV ) mutations without overt clinical symptoms of FMF, a condition designated as phenotype II. Furthermore, two MEFV mutations may be harbored without signs or symptoms of FMF nor of reactive amyloidosis. This 'silent' homozygous or compound heterozygote state is termed phenotype III.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 50
• Est. completion date: August 2023
• Est. primary completion date: August 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 1 Day to 18 Years

Eligibility

Inclusion Criteria:

A- all children diagnosed as FMF according to Tel hashomer criteria aged below 18 years:

The presence of at least 2 of the following 5 criteria after exclusion of other causes can

diagnose FMF with high sensitivity:

1. Fever axillary temperature of >38?C, 6-72 h of duration, =3 attacks

2. Abdominal pain 6-72 h of duration =3 attacks

3. Chest pain 6-72 h duration= 3 attacks

4. Arthritis 6-72 h duration =3 attacks, oligoarthritis

5. Family history of FMF*(11) B-sisters and brothers of a child with FMF with clinical or subclinical manifestation of FMF.

Exclusion Criteria:

1. Children with other auto inflammatory diseases, or with other diseases.

2. Persons above 18 years old.

Related Conditions & MeSH terms

• Brucellosis
• Familial Mediterranean Fever
• Fever
• Hereditary Autoinflammatory Diseases

Intervention

Diagnostic Test:
• CBC with differential ,ESR ,CRP, Amyloid level , FMF gene
leucocytosis , high ESR and CRP ,amyloid level indicates inflammation positive FMF gene indicates having the disease .

Locations

Country	Name	City	State
Egypt	Sohag University Hospital	Sohag

Sponsors (1)

Lead Sponsor	Collaborator
Sohag University

Country where clinical trial is conducted

Egypt, 

References & Publications (4)

Alghamdi M. Familial Mediterranean fever, review of the literature. Clin Rheumatol. 2017 Aug;36(8):1707-1713. doi: 10.1007/s10067-017-3715-5. Epub 2017 Jun 18. Review. — View Citation

Çakan M, Karadag SG, Tanatar A, Sönmez HE, Ayaz NA. The Value of Serum Amyloid A Levels in Familial Mediterranean Fever to Identify Occult Inflammation During Asymptomatic Periods. J Clin Rheumatol. 2021 Jan 1;27(1):1-4. doi: 10.1097/RHU.0000000000001134. — View Citation

Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. 2019 Feb;48(1 Pt 2):e61-e76. doi: 10.1016/j.lpm.2018.08.014. Epub 2019 Jan 25. Review. — View Citation

Talaat HS, Sheba MF, Mohammed RH, Gomaa MA, Rifaei NE, Ibrahim MFM. Genotype Mutations in Egyptian Children with Familial Mediterranean Fever: Clinical Profile, and Response to Colchicine. Mediterr J Rheumatol. 2020 Jun 15;31(2):206-213. doi: 10.31138/mjr.31.2.206. eCollection 2020 Jun. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	CBC	leucocytosis	12 month
Primary	amyloid level	high in untreated patients	12 month
Primary	FMF gene	positive or negative	12 month