Evaluation of quality of life and its associations with clinical parameters in pediatric patients with familial Mediterranean fever

Yıldırım, Deniz Gezgin; Bakkaloğlu, Sevcan A.; Acar, A. Şebnem Soysal; Çelik, Bülent; Buyan, Necla

doi:10.14744/nci.2020.90093

Cited by 5 publications

(2 citation statements)

References 9 publications

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“…There is currently no score evaluating disease activity from both physician’s and patient’s perspective, taking into account the quality of life, which was a strong argument in our study for introducing IL-1 inhibitors. Several authors confirmed a poorer quality of life in children and adults with FMF ( 155 – 158 ) and a higher incidence of anxiety and depression ( 159 – 161 ) that may lead to more frequent attacks with higher rates of CRP and SAA ( 159 , 161 , 162 ). Van der Hilst et al.…”

Section: Discussionmentioning

confidence: 96%

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

et al. 2021

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BackgroundInterleukin (IL)-1 inhibitors represent the main treatment in patients with colchicine-resistant/intolerant familial Mediterranean fever (crFMF), mevalonate kinase deficiency (MKD), and tumor necrosis factor receptor-associated periodic syndrome (TRAPS). However, the reasons for the use of IL-1 inhibitors in these diseases are still not completely clarified.ObjectiveIdentify real-life situations that led to initiating anakinra or canakinumab treatment in hereditary recurrent fevers (HRFs), combining data from an international registry and an up-to-date literature review.Patients and MethodsData were extracted from the JIRcohort, in which clinical information (demographic data, treatment, disease activity, and quality of life) on patients with FMF, MKD, and TRAPS was retrospectively collected. A literature search was conducted using Medline, EMBASE, and Cochrane databases.ResultsComplete data of 93 patients with HRF (53.8% FMF, 31.2% MKD, and 15.1% TRAPS) were analyzed. Data from both the registry and the literature review confirmed that the main reasons for use of IL-1 blockers were the following: failure of previous treatment (n = 57, 61.3% and n = 964, 75.3%, respectively), persistence of disease activity with frequent attacks (n = 44, 47.3% and n = 1,023, 79.9%) and/or uncontrolled inflammatory syndrome (n = 46, 49.5% and n = 398, 31.1%), severe disease complication or associated comorbidities (n = 38, 40.9% and n = 390, 30.4%), and worsening of patients’ quality of life (n = 36, 38.7% and n = 100, 7,8%). No reasons were specified for 12 (16.4%) JIRcohort patients and 154 (12%) patients in the literature.ConclusionIn the absence of standardized indications for IL-1 inhibitors in crFMF, MKD, and TRAPS, these results could serve as a basis for developing a treat-to-target strategy that would help clinicians codify the therapeutic escalation with IL-1 inhibitors.

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Section: Discussionmentioning

confidence: 96%

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

et al. 2021

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“…All subscales were then linearly transformed to a 0–100 scale. Higher scores represent a better QoL [ 6 ]. Details about the questions can be found on the KINDL web site ( (accessed on 22 March 2023).…”

Section: Methodsmentioning

confidence: 99%

Ureterocystoplasty in Boys with Valve Bladder Syndrome—Is This Method Still up to Date?

et al. 2023

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Boys with valve bladder syndrome (PUV) require adequate treatment of the lower urinary tract to preserve renal function and improve long-term outcomes. In some patients, further surgery may be necessary to improve bladder capacity and function. Ureterocytoplasty (UCP) is usually carried out with a small segment of intestine or, alternatively, with a dilated ureter. Our aim was to evaluate the long-term outcomes after UCP in boys with PUV. UCP had been performed in 10 boys with PUV at our hospital (2004–2019). Pre- and postoperative data were evaluated in relation to kidney and bladder function, the SWRD score, additional surgery, complications, and long-term follow-up. The mean time between primary valve ablation and UCP was 3.5 years (SD ± 2.0). The median follow-up time was 64.5 months (IQR 36.0–97.25). The mean increase in age-adjusted bladder capacity was 25% (from 77% (SD ± 0.28) to 102% (SD ± 0.46)). Eight boys micturated spontaneously. Ultrasounds showed no severe hydronephrosis (grade 3–4). The SWRD score showed a median decrease from 4.5 (range 2–7) to 3.0 (range 1–5). No conversion of augmentation was required. UCP is a safe and effective approach to improve bladder capacity in boys with PUV. In addition, the possibility of micturating naturally is still maintained.

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The efficacy of a single-dose anakinra injection during disease attack in pediatric familial Mediterranean fever

et al. 2023

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Evaluation of quality of life and its associations with clinical parameters in pediatric patients with familial Mediterranean fever

Cited by 5 publications

References 9 publications

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

Ureterocystoplasty in Boys with Valve Bladder Syndrome—Is This Method Still up to Date?

The efficacy of a single-dose anakinra injection during disease attack in pediatric familial Mediterranean fever

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