Assessment of neutrophil to lymphocyte ratio and mean platelet volume in pediatric familial Mediterranean fever patients.

BACKGROUND: Blood neutrophil to lymphocyte ratio (NLR) and mean platelet volume (MPV) both have been used as a simple marker of inflammation in many disorders. Here, we aimed to investigate the relationship between NLR, MPV, and familial Mediterranean fever (FMF). MATERIALS AND METHODS: In this retrospective study, the files of FMF patients in pediatric rheumatology outpatient clinic were reviewed. There were 160 participants (68.4%) in the FMF patient group and 74 participants (31.6%) in the control group. Ninety of patients were in attack-free period, and 70 were in attack period. RESULTS: The highest values of NLR were found in the patients at attack period. Patients in attack-free period and the participants in control group had similar levels of NLR (1.71 ± 0.83 and 1.91 ± 1.86 respectively) (P = 0.457), and they had lower ratios than the patients did at attack period (4.10 ± 3.11) (P < 0.001 for both). There was no significant difference between MPV values of attack patients (8.35 ± 4.91) and attack-free patients (8.43 ± 1.15) (P = 0.074). MPV values of attack patients and attack-free patients were significantly higher than control group (7.99 ± 0.81) (P < 0.001 for both). CONCLUSION: NLR ratio may indicate FMF attack period. Since there was no significant difference between attack-free patients and control groups, NLR ratio cannot be used as a subclinical inflammation marker. However, NLR could be a useful predictor of inflammation in FMF patients. On the other hand, since our attack and attack-free patients have similar MPV values and both had greater MPV values than control group, we suggest that MPV may be used to show subclinical inflammation.

Interleukin-1 targeting treatment in familial Mediterranean fever: an experience of pediatric patients.

OBJECTIVES: The aim of this report was to evaluate and discuss treatment of pediatric familial Mediterranean fever (FMF) patients with anti-interleukin1 (IL-1) agents. METHODS: Refractory or colchicine unresponsive FMF was described as severe and frequent attacks and/or having high acute phase reactance levels despite having a maximum dose of colchicine (2 mg/day). Disease course, adverse effects, duration of follow-up, treatment protocols, responses to the therapies were discussed. RESULTS: Eight patients (6 male, 2 female) having refractory FMF were identified. Mediterranean fever (MEFV) gene analyses revealed homozygous M694V mutations in six patients and heterozygote M694V mutations in one patient and no mutation in one patient. They were all treated with anakinra and/or canakinumab. The use of anti-IL-1 drugs was beneficial to all patients. None of them had any severe adverse effects due to the therapy. CONCLUSIONS: Anakinra and canakinumab were effective in patient refractory to colchicine treatment as shown both in our series and in the literature. Therefore, controlled trials are needed to evaluate the safety and long-term efficacy of IL-1 targeting agents in colchicine resistant patients.

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease.

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium-sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium-sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis-related PAN facilitates early diagnosis, and instigation of appropriate treatment.

Determination of Factors to Distinguish MIS-C from Acute Appendicitis in Children with Acute Abdominal Pain.

INTRODUCTION: The aim of this study was to make the differential diagnosis between acute appendicitis and multisystem inflammatory syndrome in children (MIS-C) for patients presenting with the complaint of acute abdominal pain (AAP) and to identify the determining factors for the diagnosis of MIS-C. MATERIALS AND METHODS: Eighty-one children presenting with AAP/suspected AAP were evaluated. Of these, 24 (29.6%) were included in the MIS-C group (MIS-C/g) and 57 were included in the suspected appendicitis group (S-A/g), which consisted of two subgroups: appendicitis group (A/g) and control observation group (CO/g). RESULTS: Comparing MIS-C/g, A/g, and CO/g, duration of abdominal pain (2.4, 1.5, 1.8 days), high-grade fever (38.8, 36.7, 37°C), severe vomiting, and severe diarrhea were higher in MIS-C/g. Lymphocytes count (LC) was lower, while values of C-reactive protein (CRP), ferritin, and coagulopathy were higher in MIS-C/g (p < 0.05). The optimal cutoffs for the duration of abdominal pain was 2.5 days; the duration of fever, 1.5 days; peak value of fever, 39°C; neutrophil count, 13,225 × 1,000 cell/µMoL; LC, 600 × 1,000 cell/µMoL; ferritin, 233 µg/L; and D-dimer, 16.4 mg/L (p < 0.05). The optimal cutoff for CRP was 130 mg/L (sensitivity 88.9, specificity 100%, positive predictive value 100%, NPV, negative predictive value 92.5%, p < 0.001). All patients in MIS-C/g tested positive by serology by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). CONCLUSION: The duration of abdominal pain, presence of high-grade and prolonged fever, and evaluation of hemogram in terms of high neutrophil count and low LC exhibit high sensitivity and negative predictive value for MIS-C presenting with AAP. In case of doubt, inflammatory markers such as CRP, ferritin, D-dimer, and serology for SARS-CoV-2 should be studied to confirm the diagnosis.