The level of agreement between the numerical rating scale and visual analogue scale for assessing pain intensity in adults with chronic pain.

The numerical rating scale and visual analogue scale are used to quantify pain intensity. However, it has not yet been explored whether these scores are interchangeable in adults with chronic pain. Data from the prospective multicentre cross-sectional INTERVAL study were used to evaluate the one-dimensionality and agreement between numerical rating scale scores and visual analogue scale scores in adults with chronic pain. Pain intensity scores using the numerical rating scale and visual analogue scale were provided by 366 patients with chronic pain for current, average, minimal and maximal pain. To evaluate whether pain intensity scales are completed in accordance with each other, the proportion of patients who satisfied the following condition was calculated: minimal pain intensity ≤ maximal pain intensity. A factor analysis confirmed the one-dimensionality of the pain measures. A significant difference was found between numerical rating scale and visual analogue scale scores for average, current, minimum and maximum pain. Intra-class correlation coefficient estimates ranged from 0.739 to 0.858 and all measures failed to show sufficient and acceptable agreement at the 95% level. The strength of agreement between pain severity categories was classified as 'moderate' for average and minimal pain and 'substantial' for current and maximal pain. The proportion of patients who scored minimal pain ≤ maximal pain was 97.5% for the numerical rating scale and 89.5% for the visual analogue scale. This study failed to show an acceptable agreement between the numerical rating scale and visual analogue scale when pain intensity was rated by adults with chronic pain, despite showing both scales measure the same information.

Paediatric mastocytosis: long-term follow-up of 53 patients with whole sequencing of KIT. A prospective study.

BACKGROUND: Mastocytosis is a heterogeneous group of clinical disorders characterized by the abnormal accumulation of mast cells. The adult and paediatric forms differ in their clinical and genetic features and outcomes. OBJECTIVES: To describe the clinical evolution of a well-characterized cohort of paediatric mastocytosis (PM), and to analyse the relationship between KIT mutation and the clinical course. METHODS: This was a prospective cohort study performed at the National Clinical Reference Center for Mastocytosis. Diagnosis was confirmed by identification of KIT mutation on lesional skin biopsy. Mastocytosis subtype, mast cell mediator-related symptoms (MC MRS) and clinical course were recorded. Fifty-three patients with PM and > 4 years of disease course were enrolled. The mean ± SD age at the final evaluation was 13·2 ± 4·8 years. The main outcome was the type of KIT mutation as a predictor of evolution and clinical characteristics. RESULTS: Patients presented with maculopapular cutaneous mastocytosis (n = 44), diffuse cutaneous mastocytosis (n = 6) or mastocytoma (n = 3). The mean duration of disease was 12·1 years. Substantial or partial cutaneous regression (18 of 53 and 16 of 53), stabilization or aggravation (16 of 53) and complete cutaneous regression (three of 53) were noted. MC MRS mainly regressed (21 of 53). For 22 patients, evolution of MC MRS and evolution of cutaneous lesions were different. No significant association between evolution and KIT mutation or between evolution and type of cutaneous mastocytosis was found. A late onset of the disease (after 2 years) is associated with worse evolution. CONCLUSIONS: PM is not systematically self-regressive. MC MRS manifestations and cutaneous lesions can persist or increase overtime. KIT mutation is not a predictor of evolution.

Wireless high-frequency dorsal root ganglion stimulation for chronic low back pain: A pilot study.

OBJECTIVE: Evaluation of the efficacy of a wireless high-frequency stimulator placed over selected DRG of exiting nerve roots for the treatment of chronic low back pain. DESIGN: Feasibility. SUBJECTS: Six subjects with chronic, intractable back pain refractory to standard medical treatment. METHODS: Four stimulators (Freedom-4A) provided by Stimwave Technologies, were implanted over the DRG exiting nerve roots, bilaterally at both the T9 and L2 vertebral levels. Subjects were asked to evaluate stimulation independently with the devices turned on at T9 and subsequently L2 for each of 2 weeks. Subjects were then monitored for 8 weeks with the preferred stimulator. Pain reduction with the Visual Analog Scale (VAS), functionality with the Oswestry Disability Index (ODI), Patient Global Impression of Change (PGIC) and medication usage were evaluated. RESULTS: Four subjects preferred T9 stimulation with only one subject preferring stimulation at L2. One subject dropped out of the study before conclusion of the 4-week evaluation and is not included in this report. Average pain levels (n = 5) at 12-week post-implantation decreased with 61% for back pain and 56% for leg pain with a significant reduction in pain medication, including a 100% reduction in opioid pain medications. The average reduction in disability was 12%. Subjects reported an average impression of change of 6 (1 = no change, 7 = great deal better). CONCLUSIONS: Wireless high-frequency stimulation of the DRG is a viable option to treat chronic low back pain. Preliminary results show a subject preference for stimulation at the T9 vertebral level.

Decreased tryptophan and increased kynurenine levels in mastocytosis associated with digestive symptoms.

The main metabolism pathway of tryptophan is protein formation, but it can also be metabolized into serotonin and kynurenine. Indoleamine 2,3-dioxygenase (IDO) is the enzyme that catalyzes the degradation of tryptophan into kynurenine. Mastocytosis is a heterogeneous disease characterized by mast cell accumulation in various tissues with 57% of patients having gastrointestinal involvement. We studied tryptophan metabolism in mastocytosis patients displaying or not gastrointestinal features and healthy subjects (n = 26 in each group). Mastocytosis patients with digestive symptoms displayed significantly increased kynurenine level and IDO activity as compared to healthy controls and mastocytosis patients without digestive symptoms. This could be linked to mast cell-mediated digestive inflammation among patients with mastocytosis. This work is the first focusing on kynurenine pathway in a mast cell disease and could help to understand the pathogenesis of digestive features in mastocytosis as well as in other mast cell-mediated diseases.

FDG-PET/CT findings in systemic mastocytosis: a French multicentre study.

INTRODUCTION: Mastocytosis is a clonal haematological disease characterized by uncontrolled proliferation and the activation of mast cells. The value of FDG-PET/CT (FDG-PET) in mastocytosis has yet to be determined. METHODS: We retrospectively identified patients with an established diagnosis of systemic mastocytosis (SM), according to the WHO criteria, who underwent PET using the French Reference Centre for Mastocytosis database. Semi-quantitative and visual analysis of FDG-PET was performed and compared to the clinico-biological data. RESULTS: Our cohort included 19 adult patients, median age 65 years [range 58-74], including three with smouldering SM (SSM), three with aggressive SM (ASM), 10 with an associated clonal haematological non-mast-cell lineage disease (SM-AHNMD), and three with mast cell sarcoma (MCS). FDG-PET was performed at the time of the SM diagnosis (15/19), to evaluate lymph node (LN) activity (3/19) or the efficacy of therapy (1/19). FDG uptake was observed in the bone marrow (BM) (9/19, 47%), LN (6/19, 32%), spleen (12/19, 63%), or liver (1/19, 5%). No significant FDG uptake was observed in the SSM and ASM patients. A pathological FDG uptake was observed in the BM of 6/10 patients with SM-AHNMD, appearing as diffuse and homogeneous, and in the LN of 5/10 patients. All 3 MCS patients showed intense and multifocal BM pathological uptake, mimicking metastasis. No correlation was found between the FDG-PET findings and serum tryptase levels, BM mast cell infiltration percentage, and CD30 and CD2 expression by mast cells. CONCLUSIONS: FDG uptake does not appear to be a sensitive marker of mast cell activation or proliferation because no significant FDG uptake was observed in most common forms of mastocytosis (notably purely aggressive SM). However, pathological FDG uptake was observed in the SM-AHNMD and in MCS cases, suggesting a role of FDG-PET in their early identification and as a tool of therapeutic assessment in this subgroup of patients.

FES kinases are required for oncogenic FLT3 signaling.

The closely related non-receptor tyrosine kinases FEline Sarcoma (FES) and FEs Related (FER) are activated by cell surface receptors in hematopoietic cells. Despite the early description of oncogenic viral forms of fes, v-fes, and v-fps, the implication of FES and FER in human pathology is not known. We have recently shown that FES but not FER is necessary for oncogenic KIT receptor signaling. Here, we report that both FES and FER kinases are activated in primary acute myeloid leukemia (AML) blasts and in AML cell lines. FES and FER activation is dependent on FLT3 in cell lines harboring constitutively active FLT3 mutants. Moreover, both FES and FER proteins are critical for FLT3-internal tandem duplication (ITD) signaling and for cell proliferation in relevant AML cell lines. FER is required for cell cycle transitions, whereas FES seems necessary for cell survival. We concluded that FES and FER kinases mediate essential non-redundant functions downstream of FLT3-ITD.

[Evoked oto-acoustic potentials as auditory screening tool in school health services]. / De geëvokeerde otoakoestische emissies als gehoorscreeningsinstrument binnen het medisch schooltoezicht.

The use of ILO 88 Otodynamic Otoacoustic Analyser in school health services is investigated. The technique in normal and hearing-impaired children for which audiometric data were known, is compared to the experimental group consisting of children, difficult to test with the conventional hearing screening methods. Several minimal practical criteria, advantages and limitations of the evoked otoacoustic emission technique are defined.

Evoked otoacoustic emissions in infant hearing screening.

The main application of the ILO88 otodynamic otoacoustic analyser is clearly in detecting whether the peripheral auditory system is normal or pathological. The evoked otoacoustic emission technique in normal and hearing-impaired children for which audiometric data were known (n = 422 ears) was first performed. The correlation was excellent. The actual experimental group (n = 258 ears) consisted of children who are difficult to test with the conventional screening methods in school health services. The use of evoked otoacoustic emissions as screening instrument is new, and therefore several minimal practical criteria, advantages and limitations were defined. The evoked otoacoustic emission test tool is especially applicable in difficult to test children using conventional screening methods.