Alaaddin YORULMAZ, Hikmet AKBULUT, Şükrü ARSLAN

Uzamış Febril Myalji Sendromu ile başvuran Ailesel Akdeniz ateşi: 9 Hastanın Klinik, Laboratuvar ve Demografik Özellikleri

Amaç: Bu çalışmanın amacı AAA’li hastalarda uzamış febril myalji sendromlu bir grup hastayı geriye dönük olarak incelemek ve klinik tecrübemizi paylaşmaktır. Gereç ve Yöntemler: UFMS'li dokuz hasta Selçuk Üniversitesi Tıp Fakültesi Pediatri Acil Servisi ve Pediatrik Romatoloji Polikliniği (Eylül 2016- Mayıs 2018) elektronik veri tabanından tanımlandı. Klinik ve laboratuvar verileri toplandı. Hastaların yaşı, cinsiyet, eşlik ettiği semptomlar, AAA’un eski tanı ise takip süresi, tanı konulduktan sonraki takip süresi, hastanede yatış süresi, semptomların kaç gün öncesinden başladığı, tam kan sayımı, akut faz reaktanları gibi veriler kaydedildi. Bulgular: Çalışmada 9 hastanın 7'si (% 77.8) kadın, 2'si (% 22.2) erkekti. Hastalarımızın yaşları 39 ay ile 192 ay arasında değişmekte olup median ortalaması 145 ay idi. Hastaların sadece 2’sine (%22,2) daha önce tanı konulmuş olup kolşisin tedavisi almaktaydı. Hastaların 7’sinde (%77,78) AAA’inin ilk belirtisi FMS idi. Hastaların genetik analizine bakıldığında 4 hastada (%44,44) M694V homozigot tespit edildi. Tüm hastalarda akut faz reaktanları yüksek seviyede idi. Kreatin kinaz düzeyi tüm hastalarda normal sınırlardaydı. Pulse kortikosteroid tedavisi, 10 mg / kg dozunda uygulandı. Tüm hastalarda tedavi sonrası tam iyileşme görüldü. Sonuç: AAA kriterlerini karşılamayan, yüksek ateşle birlikte akut faz reaktanı yüksekliğinin olduğu ve uzun süren myalji varlığında UFMS düşünülmelidir.

Anahtar Kelimeler:

Ailesel Akdeniz ateşi; Uzamış febril myalji; Çocuk

Familial Mediterranean fever with Prolonged Febrile Myalgias Syndrome: Clinical, Laboratory and Demographic Characteristics of 9 Patients

Objective: The aim of this study is to retrospectively review a group of patients with prolonged febrile myalgias syndrome in patients with FMF and to share their clinical experience.Materials and Methods: Nine patients with prolonged febrile myalgias syndrome were identified from the electronic database of a Selçuk University Medical Faculty Pediatric Emergency Department and Pediatric Rheumatology Polyclinic (September 2016-May 2018). Their clinical and laboratory data were collected. Data such as age, sex, accompanying symptoms of FMF, previous follow-up of FMF, duration of follow-up after diagnosis, hospital stay, number of days before symptoms, complete blood count and acute phase reactants were recorded. Pulse corticosteroid therapy was administered at a dose of 10mg/kg.Results In the study, 7 of 9 patients (77.8%) were females and 2 (22.2%) were male. The ages of our patients ranged from 39 months to 192 months and the mean median was 145 months. Only 2 (22.2%) of the patients had previously been diagnosed and received colchicine treatment. The first sign of FMF in 7 of patients (77.78%) was PFMS. When genetic analysis of the patients was examined, M694V homozygous was detected in 4 patients (44.44%). Acute phase reactors were at high level in all patients. Creatine kinase levels were within normal limits in all patients. The patients received intravenous methylprednisolone. In all patients, complete recovery was seen after treatment. Conclusion: PFMS should be considered in the presence of high fever, acute phase reactant height, and long-standing myalgia that does not meet FMF criteria.

Keywords:

Familial Mediterranean fever; protracted febrile myalgia; child,

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