A case of mandible Paget's disease of the bone treated with intravenous neridronate

Paget's disease of bone (PDB) is a focal disorder of osteoclasts, leading to chaotic bone remodelling, and it is characterized by the presence of focal areas of excessive bone formation alongside with areas of focal bone resorption. The typical radiographic feature is the cotton wool appearance. To date, bisphosphonates are the mainstay of the treatment. We hereby report the case of a young woman presenting with mandible PDB, with a relevant diagnostic delay and mistakenly treated for five years with chronic oral corticosteroids. After our evaluation, the patient received treatment with intravenous neridronate (an amino-bisphosphonate licensed in Italy for the treatment of this disease), with achievement of clinical remission

Insight into the WNT system and its drug related response.

The WNT signalling pathway is a complex system for transferring information for DNA expression from the cell surface receptors to cytoplasm and then to the nucleus. It is based on several proteins that work together as agonists and antagonists in order to maintain homeostasys and to promote anabolic processes. The WNT system acts on all cellular lines involved in bone resorption and formation. WNT pathway can mainly be triggered by two different signalling cascades. The first is well known and is the so-called WNT-beta catenin system (or the canonical pathway), the second is known as the non canonical WNT pathway. WNT proteins form a superfamily of secreted glycoproteins. The association with surface receptors, called Frizzled, that are members of the G protein-coupled receptors superfamily and co receptors like low-density lipoprotein receptor-related proteins 5 and 6 (LRP5/6) complete the WNT system. LRP5/6 show high affinity for WNT antagonists that modulate the activity of this pathway: DKK1 and sclerostin (SCL), that play a crucial role in modulating the WNT system. The WNT-pathway and in particular its antagonists SCL and DKK1 seems to play a key role in the regulation of bone remodeling during treatment with bone active agents such as bisphosphonates, but not only. Their effects become relevant especially in the course of long-term treatments

Radiological features of knee joint synovial chondromatosis

Synovial chondromatosis (SC) is a rare condition with a very variable clinical presentation, thus making the diagnosis not immediate. We report a case of massive primary SC of the knee, properly evaluated with X-rays, ultrasonography and magnetic resonance imaging and successfully treated with an arthroscopic approach

Different fracture risk profile in patients treated with anti-osteoporotic drugs in real-life

In this retrospective study, we intended to investigate the baseline fracture risk profile in patients who started treatment with different anti-osteoporotic medications. We analyzed retrospectively the fracture risk calculated with DeFRA, a validated FRAX derived tool, in women who started an anti-osteoporotic treatment from 2010 to 2017. We analyzed baseline data of 12,024 post-menopausal women aged over 50 years. Teriparatide initiators had a baseline 10-year risk of major osteoporotic fracture of 82.1% with a Standard Deviation (SD) of 66.5%. Denosumab initiators and zoledronic acid initiators had a greater 10-year baseline risk of fracture (54.3%, SD 46.5% and 47.0%, SD 42.0 respectively) than patients initiated on alendronate (24.9%, SD 34.6%) and patients initiated on risedronate (23.9%, SD 24.1%). Using DeFRA, a FRAX™ derived tool, we showed significantly different fracture risk profiles in women who were started on various therapeutic agents for the treatment of osteoporosis in routine clinical practice

Association between exposure to fine particulate matter and osteoporosis: a population-based cohort study

Long-term environmental air pollution exposure was associated with osteoporosis' risk in a cohort of women at high risk of fracture. Cortical sites seemed to be more susceptible to the exposure's effect

RHINASTHMA-Adolescents: a new quality of life tool for patients with respiratory allergy

Background: Specific instruments for health-related quality of life (HRQoL) assessment in adolescents with rhinoconjunctivitis or asthma are available. None of them evaluates rhinitis and asthma together, although they often coexist. Our aim was to validate a HRQoL questionnaire for adolescents with rhinoconjunctivitis, asthma, or both. Methods: A pool of 38 items covering the main symptoms and problems related to respiratory allergy was generated based on literature review, clinical experience, and unstructured interviews to 54 adolescents. The items were randomly listed and presented to 88 consecutive outpatients (44 M; mean age 15.2 3.1). Patients had to indicate which item they had experienced and, for each selected item, its importance on a four-point scale (1 = not at all; 4 = very much). Twelve items were excluded from the list, because of low importance. In the validation phase, 102 patients (54 M; mean age 15.36 1.12) completed the KINDL, a generic HRQoL tool, and the new questionnaire (RHINASTHMA-Adolescents). Results: Factor analysis revealed a five-dimensional structure, which explained up to 71.23% of the total variance. Association between RHINASTHMA-Adolescents and KINDL scores was all in the expected direction. Internal consistency for the extracted factors was satisfactory: Upper Airways (0.81), Lower Airways (0.89), Emotions (0.85), Social Relationship (0.79), Daily life management (0.74). Reliability was good for all factors with a Pearson coefficient ranged from 0.91 to 0.99. Conclusions: RHINASTHMA-Adolescents is the first tool for evaluating HRQoL in patients with rhinitis and/or asthma. It provides a simple assessment and met the standards of validity, internal consistency, and reliability

Alterações químicas, bioquímicas e da qualidade do café submetido a diferentes formas de processamento de secagem.

Devido ao elevado teor de umidade em que é colhido, o café requer secagem adequada a fim de preservar sua qualidade. Nesta etapa, o teor de umidade é reduzido de aproximadamente 60% (b.u.) para 11% (b.u.), sendo que o processo de secagem em terreiros, em secadores mecânicos ou a combinação destes são os métodos mais utilizados. Pesquisas recentes têm indicado várias alterações na integridade das membranas celulares, processo de germinação, conteúdo de ácidos e açúcares, devido ao estresse provocado aos grãos, ao longo do processamento e secagem. A elevação da temperatura de secagem promove danos aos grãos, o que reduz sensivelmente a qualidade da bebida. Este trabalho teve como objetivo verificar a influência da secagem lenta e secagem rápida sobre alguns parâmetros físicoquímicos do grão e sobre a qualidade do café. Os cafés utilizados neste experimento foram da cultivar Catuaí Vermelho IAC 44 produzidos na Fazenda Experimental de Machado-FEMA, da EPAMIG. Foram avaliadas três formas de preparo: café natural, desmucilado e despolpado. Após a obtenção destas três formas de preparo os cafés foram então submetidos à secagem lenta, sendo secados em telados suspensos à sombra ou secados por meio de secagem rápida em secadores de camada fixa com controle de temperatura de secagem de 35ºC, até atingirem cerca de 11% de umidade (b.u.). Depois do processo de secagem (lenta ou rápida), os cafés foram beneficiados e submetidos às seguintes análises: condutividade elétrica, lixiviação de potássio, atividade enzimática da polifenoloxidase, acidez total titulável e análise sensorial. Por meio dos resultados observados, verificam-se menores valores de condutividade elétrica, lixiviação de potássio, acidez total titulável, maior atividade enzimática da polifenoloxidase e melhor qualidade nos cafés submetidos à secagem lenta, ou seja, secagem à sombra, independente da forma de preparo utilizada. Em relação ao tipo de preparo, observa-se que os cafés naturais apresentaram maiores valores de condutividade elétrica e lixiviação de potássio quando submetidos à secagem rápida, ou seja, em secadores mecânicos, o que denota uma maior probabilidade desses cafés de perderem qualidade