Sonographic evaluation of intra-abdominal organs in children with familial Mediterranean fever

Year 2021, Volume: 4 Issue: 5, 662 - 665, 05.09.2021

Ergün Sönmezgöz , Fitnet Sönmezgöz

https://doi.org/10.32322/jhsm.948957

Abstract

Objective: The reticuloendothelial system is rarely assessed in patients with familial Mediterranean fever (FMF). We aimed to evaluate the size of the liver and spleen by ultrasonography in children and adolescents with FMF and compare it to their healthy peers.
Material and Method: Patient data were evaluated by height, weight, and age and compared with those of healthy controls.
Results: A total of 86 children with FMF diagnosed using the Turkish Pediatric Criteria and 54 healthy children were included. The extent of splenomegaly was 27.9% in the FMF group. The mean spleen length was 99.84±17.4 mm in patients and 93.44+15.49 mm in controls (p=0.03). The mean liver length was 122.61±17.4 mm in patients and 117.71±16.04 mm in controls (p=0.104). FMF appears to affect spleen length independently of anthropometric data (t=2.182; p=0.031). Splenomegaly was accompanied by the M694V (32.55%, n=17) and E148Q (3.4%, n=3) mutations.
Conclusion: FMF affects spleen length independently of anthropometric data. Splenomegaly may reflect subclinical inflammatory activity in FMF patients in remission. Spleen size can serve as a marker of subclinical inflammation during remission.

Keywords

Familial Mediterranean fever, ultrasonography, splenomegaly, children, inflammatory markers

References

• Ozen S, Bilginer Y. A clinical guide to autoinflammatory diseases: familial Mediterranean fever and next-of-kin. Nature Reviews Rheumatology 2014; 10: 135-47.
• Yalcinkaya F, Cakar N, Acar B, et al. The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study. Rheumatology International 2007; 27: 517-22.
• La Regina M, Ben Chetrit E, Gazparyan AY, Livneh A, Ozdogan H, Manna R. Current trends in colchicine treatment in familial Mediterranean fever. Clin Exper Rheumatol 2013; 31: 41-6.
• Aharoni D, N Hiller, Hadas-Halpern I. Familial Mediterranean fever: abdominal imaging findings in 139 patients and review of the literature. Abdominal Imaging 2000; 25: 297-300.
• Barakat MH, Karnik AM, Majeed HWA, El-Sobki NI, Fenech FF. Familial Mediterranean fever (recurrent hereditary polyserositis) in Arabs—a study of 175 patients and review of the literature. Q J Med 1986; 60: 837–47.
• Inal A, Yilmaz, M Kendirli, SG, Altintas, DU. Karakoc, GB. The clinical and genetical features of 124 children with Familial Mediterranean fever: experience of a single tertiary center. Rheumatol Int 2009; 29: 1279–85.
• Yalcinkaya F, Ozen S, Ozçakar ZB, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford) 2009; 48: 395-8.
• Ozen S, Aktay N, Lainka E et al. Disease severity in children and adolescents with familial Mediterranean fever: a comparative study to explore environmental effects on a monogenic disease. Ann Rheum Dis 2009; 68: 246-8.
• Rimon D, Meir Y, Cohen L, Retroperitoneal lymphadenopathy in familial Mediterranean fever. Postgrad Med J 1989; 65: 776-8.
• Oncu K, Yazgan Y, Tanoglu A, et al; Can serum fetuin-A be regarded as an inflammatory marker among patients with familial Mediterranean fever? Dig Dis Sci 2013; 58: 3212-7.
• Akyol T, Düzenli T, Tanoğlu A. Evaluation of serum CXC chemokine ligand 16 (CXCL16) as a novel inflammatory bio- marker or familial Mediterranean fever disease. Turk J Med Sci 2021; 51: 813-8.
• Lachmann H.J, Sengül B, Yavuzşen TU, et al; Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford) 2006; 45: 746-50.
• Bilginer Y, Akpolat T, Ozen S. Renal amyloidosis in children. Pediatr Nephrol, 2011; 26: 1215-27.
• Ben-Zvi I, Livneh A. Chronic inflammation in FMF: markers, risk factors, outcomes and therapy. Nat Rev Rheumatol 2011;7:105-12.
• Baykal Y, Saglam K, Yilmaz MI, Taslipinar A, Akinci SB, Inal A. Serum sIL-2r, IL-6, IL-10 and TNF-alpha level in familial Mediterranean fever patients. Clin Rheumatol 2003; 22: 99-101.
• Marzouk H, Lotfy HM, Farg Y, Rashed LA, El-Garf K. Mean platelet volume and splenomegaly as useful markers of subclinical activity in Egyptian children with Familial Mediterranean Fever: a cross-sectional study. Int J Chronic Dis 2015; 2015: 152616.
• Livneh A, Langevitz P, Zemer D, et al. The changing face of familial Mediterranean fever. Semin Arthritis Rheum 1996; 26: 612-27.
• Korkmaz C, Ozdogan H, Kasapçopur O, yazici H. Acute phase response in familial Mediterranean fever. Ann Rheum Dis 2002; 61:79-81.
• Aharoni D, Hiller N, Hadas-Halpern I. Familial Mediterranean fever: abdominal imaging findings in 139 patients and review of the literature. Abdom Imaging 2000; 25: 297-300.
• Dursun I, Gok F, Babacan O, et al. Are mean platelet volüme and splenomegaly subclinical inflammatory marker in children with familial Mediterranean fever? Health 2010; 2: 692–695.
• Ureten K, Gönülalan G, Akbal E, et al. Demographic, clinical and mutational characteristics of Turkish familial Mediterranean fever patients: results of a single center in Central Anatolia. Rheumatol Int 2010; 30: 911-5.
• Tunca M, Akar S, Onen F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 2005; 84: 1-11.
• Dusunsel, R, Dursun I, Gündüz Z, et al. Genotype-phenotype correlation in children with familial Mediterranean fever in a Turkish population. Pediatr Int 2008; 50: 208-12.
• Moradian MM, Sarkisian T, Amaryan G, et al. Patient management and the association of less common familial Mediterranean fever symptoms with other disorders. Genet Med 2014; 16: 258-63.
• Koné Paut I, Dubuc M, Sportouch J, Minodier P, Garnier JM, Touitou I. Phenotype-genotype correlation in 91 patients with familial Mediterranean fever reveals a high frequency of cutaneomucous features. Rheumatology (Oxford) 2000; 39: 1275-9.
• Safak AA, Simsek E, Bahcebasi T. Sonographic evaluation of the normal limits and percentile curves of liver, spleen, and kidney dimensions in healthy school-aged children. J Ultrasound Med 2005; 24: 1359-64.
• Konus OL, Ozdemir A, Akkaya A, Erbaş G, Celik H, Işik S. Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography. AJR Am J Roentgenol 1998; 171: 1693-8.
• Warnakulasuriya DTD, Pathirennahalage P, Peries UC, et al. Ultrasonographic parameters of the liver, spleen and kidneys among a cohort of school children in Sri Lanka. Bmc Pediatrics 2017; 17: 192.
• Dogan TH, Başak M, Karatağ O, Değirmenci H, Ozkurt H. Sonographic evaluation of liver, spleen and kidney sizes in healthy children aged 0-14 years. Journal of Child Health and Diseases 2004; 47: 107-13