Is the performance of the international severity scoring system for familial mediterranean fever in children better than other scoring systems?

AIMS: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterised by recurrent episodes of fever and serosal inflammation with elevated acute phase reactants. Assessing the severity of the disease may be useful in identifying colchicine-resistant patients. The aim of this study is to determine the disease severity of FMF patients according to the Pras, Mor, and International Severity Scoring System for Familial Mediterranean Fever (ISSF) scoring systems and to evaluate the consistency of these three systems. METHODS: The medical records of patients with FMF were retrospectively reviewed. Demographic features, family history of FMF, clinical characteristics at disease onset, laboratory features, Mediterranean fever genetic mutations, treatment regimens, and disease courses were recorded. RESULTS: A total of 205 patients (116 girls) were included in the study. The mean age of the patients was 13.3 ± 4.0 years. The Pras, Mor, and ISSF scores were inconsistent with each other, and there was poor fit between them (generalised Kappa: 0.140 ± 0.029; P < .001). In the receiver operating characteristic (ROC) analysis performed by accepting the clinician's opinion as the gold standard, the ISSF was found to be more sensitive and specific than the other two systems. CONCLUSION: Evaluation of disease severity according to the ISSF in paediatric patients is more sensitive and specific than the Pras and Mor scoring systems.

Función predictiva del índice de neutrófilos/linfocitos y del volumen plaquetario medio en la púrpura de Henoch-Schönlein relacionada con compromiso renal y gastrointestinal / Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement

Antecedentes: La púrpura de Henoch-Schönlein (PHS) es una vasculitis sistémica de vasos pequeños. El objetivo fue evaluar el índice de neutrófilos/linfocitos (INL) en sangre y el volumen plaquetario medio (VPM) en la PHS e investigar la relación con el compromiso renal y gastrointestinal.Métodos: Se incluyeron niños con PHS y controles sanos. Se evaluaron concentración de hemoglobina, recuento de leucocitos, recuento de trombocitos, INL, VPM, velocidad de sedimentación globular y proteína C-reactiva.Resultados: El INL fue significativamente mayor en los pacientes con PHS con hemorragia gastrointestinal (p < 0,001). El valor ideal de corte del INL para predecir la hemorragia gastrointestinal fue 2,05, con 93 % de sensibilidad y 62 % de especificidad. El VPM fue significativamente mayor en los pacientes con PHS con compromiso renal (p = 0,027).Conclusiones: El INL en sangre y el VPM podrían ser útiles para identificar el compromiso renal y gastrointestinal en la PHS

Background: Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement.Methods: Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated.Results: There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027).Conclusions:Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.

The association between obesity, hypertension and left ventricular mass in adolescents.

BACKGROUND: Obesity and hypertension (HT) are well known cardiac risk factors. Our goal was to show that even if arterial blood pressure (BP) measurements of obese adolescents are normal during clinical examination, ambulatory blood pressure monitoring (ABPM) can be high, may include cardiac involvement and can also detect left ventricular mass indices (LVMI) value for obese adolescents to diagnose left ventricular hypertrophy (LVH). METHODS: This study included 130 children (57 obese hypertensive, 36 obese normotensive, 14 normal weight hypertensive and 23 normal weight normotensive). Adolescents whose BP was measured during clinical examination, after 24-h BP was detected using ABPM, were examined with echocardiography for calculation of LVMI to determine cardiac risk factors for LVH. RESULTS: There was a significant difference between the LVMI of obese-normotensive and obese-hypertensive adolescents, which showed the effect of obesity on LVMI independent of HT. Twenty (35.7%) of 56 obese adolescents with HT detected with ABPM had normal BP measurements during clinical examination. Dipper and nondipper features of obese adolescents were significantly higher in ABPM than those with normal body mass index. When the cutoff LVMI value for LVH was set at ≥38 g/m2.7, 38.9% of obese-normotensive and 50.9% of obese-hypertensive subjects had LVH; however, when the cutoff value was set at ≥51 g/m2.7, the rates were 2.8% and 19.3%, respectively. CONCLUSIONS: Obesity is a risk factor for LVH independent of HT. To identify masked HT, 24-h ABPM and cardiac examination should be routinely performed in obese adolescents. Using a limit of LVMI ≥38 g/m2.7 in evaluating LVH secondary to HT in obese individuals may lead to an overestimated diagnosis rate of LVH.

Acute tubulointerstitial nephritis: a case series and long-term renal outcomes.

Acute tubulointerstitial nephritis (TIN) is a common cause of acute renal impairment, characterized by the infiltration of inflammatory cells in the interstitium of the kidney. We retrospectively reviewed the medical records of 19 acute TIN patients attended to our Pediatric Nephrology department between April 1999 and April 2014. Nineteen patients (7 boys and 12 girls) were evaluated. The median age was 14 years (range 7-19). Five were diagnosed as TIN histopathologically, fourteen patients were diagnosed as clinically. Six patients were treated with steroids, thirteen patients were treated symptomatically. All patients showed a rapid recovery at longest in one month. TIN is a common cause of acute renal impairment. Renal biopsy is recommended for persistent cases. Renal outcome is mostly good with symptomatic treatment but steroids could be preferred in severe nephritis however long-term follow up showed no differences between the treated and non-treated group.