Prevalence of hypersensitivity reactions in various forms of mastocytosis: A pilot study of 2485 adult patients with mastocytosis collected in the ECNM registry.

BACKGROUND: Hypersensitivity reactions (HR) are common in mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on Mastocytosis (ECNM) enables reliable studies in a larger cohort of mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with mastocytosis in the ECNM registry. METHODS: Data were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow-up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18-91 years). RESULTS: Nine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti-inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy-sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug-induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow-up. CONCLUSIONS: HR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.

Asthma management, focused on the use of oral corticosteroids: the opinions of Italian asthmatic patients.

OBJECTIVE: Patients' perceptions of asthma symptoms, and attitudes regarding diagnosis and management, can affect their ability to reach good asthma control. The aim of the study was to explore patients' perceptions of asthma management, with focus on treatment with oral corticosteroids (OCS). METHODS: A DOXAPHARMA survey was conducted. A questionnaire with 46 multiple choice questions was completed by 50 patients with severe uncontrolled asthma, and 258 with mild-moderate controlled or partly controlled asthma. Participants were representative of Italian asthmatic patients-with medium age, long asthma duration, delayed diagnosis, poor asthma control, and frequent exacerbations. RESULTS: Many asthmatics reported inadequate pharmacologic treatment. The majority but not all patients regularly used ICS/LABA. Oral treatment was common, mainly with OCS, particularly in severe asthmatics. One-fourth of patients did not regularly use inhaled therapy, and adherence was poor, resulting in frequent OCS use to treat exacerbations, which were common in mild-moderate cases. Patients were fairly satisfied with asthma therapies, but many had concerns about long-term corticosteroid use. Patients complained about poor management of comorbidities associated with asthma and OCS use, but were generally satisfied with their patient/doctor relationships. Many patients failed to achieve optimal health-related quality of life (HRQoL), mainly those with severe asthma who used OCS treatment and emphasized how OCS therapy impacted QoL. CONCLUSIONS: The survey results confirmed many problems related to mild-moderate and severe asthma management in Italy and highlighted the overuse of OCS rather than more effective and safe treatments, which had strong negative effects on HRQoL.

Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM.

ABSTRACT: Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most specific serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, ß2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of ≥260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308µg/L vs 146µg/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to ß2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; ≥1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry profiling is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.

A visão do paciente com insuficiência renal crônica sobre a importância da saúde bucal – pesquisa de campo / The opinion of patients with chronic kidney disease about the importance of oral health – field research

A insuficiência renal crônica (IRC) é uma doença sistêmica caracterizada pela perda progressiva e irreversível das funções renais. Manifestações sistêmicas causadas pela IRC podem acometer o meio bucal, favorecendo a instalação ou a progressão de processos infecciosos. Assim, o objetivo desse estudo foi avaliar o grau de conhecimento dos pacientes com IRC a respeito do laudo odontológico sobre as manifestações das infecções bucais. Foi selecionada uma amostra de 390 pacientes com IRC e aplicado um questionário durante as sessões de hemodiálise. Os dados obtidos foram organizados no programa Excel 2000 e submetidos à análise estatística pelo programa Bioestat 5.0. A grande maioria dos pacientes sabia que infecções poderiam prejudicar o sucesso do transplante, mas 32,82% não reconhecia cárie e sangramento gengival como infecções bucais. A cada três pacientes, somente um possuía o laudo odontológico, no entanto, parte deles relataram sintomas que exigem reavaliação do dentista. Entre os sintomas pesquisados, a xerostomia (50,51%) e alterações no hálito (45,64%) foram os mais relatados. Deste modo, há necessidade de conscientizar as equipes de profissionais e da pessoa com IRC sobre a importância de prevenir e/ou controlar as infecções bucais para a realização e o sucesso do transplante renal

The chronic kidney disease (CKD) is a systemic disease characterized by irreversible and progressive loss of kidney functions. Systemic manifestations caused by CKD may affect the oral environment, propitiating the installation of infectious processes. The aim of this study was to evaluate the level of knowledge of CKD patients about the dental report on oral infections. A sample of 390 patients with CKF was selected. Data collection was performed through a questionnaire, answered by the patients during hemodialysis sessions. Data were organized using Excel 2000 and later submitted to statistical analysis by Bioestat 5.0. The great majority of the patients knew that infections could compromise the success of the transplant, but 32.82% did not recognized caries and gingival bleeding as oral infections. One in each three patients had a dental report; however some of them have reported symptoms that require a reevaluation of the dentist. Among the studied symptoms, the xerostomia (50,51%) and alteration of the halitus (45.64%) were the most reported. It was verified that it is necessary to aware the professional teams and patients with CKD about de importance to prevent and/or control oral infections to perform successful kidney transplant