Interdisciplinary management of FGF23-related phosphate wasting syndromes: a Consensus Statement on the evaluation, diagnosis and care of patients with X-linked hypophosphataemia.

X-linked hypophosphataemia (XLH) is the most frequent cause of hypophosphataemia-associated rickets of genetic origin and is associated with high levels of the phosphaturic hormone fibroblast growth factor 23 (FGF23). In addition to rickets and osteomalacia, patients with XLH have a heavy disease burden with enthesopathies, osteoarthritis, pseudofractures and dental complications, all of which contribute to reduced quality of life. This Consensus Statement presents the outcomes of a working group of the European Society for Clinical and Economic Aspects of Osteoporosis, Osteoarthritis and Musculoskeletal Diseases, and provides robust clinical evidence on management in XLH, with an emphasis on patients' experiences and needs. During growth, conventional treatment with phosphate supplements and active vitamin D metabolites (such as calcitriol) improves growth, ameliorates leg deformities and dental manifestations, and reduces pain. The continuation of conventional treatment in symptom-free adults is still debated. A novel therapeutic approach is the monoclonal anti-FGF23 antibody burosumab. Although promising, further studies are required to clarify its long-term efficacy, particularly in adults. Given the diversity of symptoms and complications, an interdisciplinary approach to management is of paramount importance. The focus of treatment should be not only on the physical manifestations and challenges associated with XLH and other FGF23-mediated hypophosphataemia syndromes, but also on the major psychological and social impact of the disease.

2021 EULAR recommendations for the implementation of self-management strategies in patients with inflammatory arthritis.

BACKGROUND: An important but often insufficient aspect of care in people with inflammatory arthritis (IA) is empowering patients to acquire a good understanding of their disease and building their ability to deal effectively with the practical, physical and psychological impacts of it. Self-management skills can be helpful in this regard. OBJECTIVES: To develop recommendations for the implementation of self-management strategies in IA. METHODS: A multidisciplinary taskforce of 18 members from 11 European countries was convened. A systematic review and other supportive information (survey of healthcare professionals (HCPs) and patient organisations) were used to formulate the recommendations. RESULTS: Three overarching principles and nine recommendations were formulated. These focused on empowering patients to become active partners of the team and to take a more proactive role. The importance of patient education and key self-management interventions such as problem solving, goal setting and cognitive behavioural therapy were highlighted. Role of patient organisations and HCPs in promoting and signposting patients to available resources has been highlighted through the promotion of physical activity, lifestyle advice, support with mental health aspects and ability to remain at work. Digital healthcare is essential in supporting and optimising self-management and the HCPs need to be aware of available resources to signpost patients. CONCLUSION: These recommendations support the inclusion of self-management advice and resources in the routine management of people with IA and aim to empower and support patients and encourage a more holistic, patient-centred approach to care which could result in improved patient experience of care and outcomes.

Volatile compounds and quality analysis in commercial medicinal plants of Camellia sinensis / Caracterização dos compostos voláteis e análise da qualidade de plantas medicinais comerciais de Camellia sinensis

ABSTRACT: The interest in the use of green tea (Camellia sinensis) (L.) Kuntze (Theaceae) products have increased in the last few years due to its medicinal properties. In the present study, we proposed that headspace solid phase microextraction, in combination with gas chromatography coupled to mass spectrometric could be an efficient method to assess the volatile compounds and to ensure the quality control of C. sinensis. We have also compared the anatomical leaf of different commercial green tea samples, analyzed the information in their labels and determined the presence of foreign materials, moisture content, total ashes and microorganisms. Among the 30 analyzed samples, six were identified as C. sinensis according to the anatomical study; 83.4% were not in accordance with the specified limits for foreign matter and 6.6% for moisture content. All samples presented total ashes in accordance with the current legislation. The analysis indicated the presence of several different volatile organic compounds, being terpenes, hydrocarbons and alcohols the major ones. Microbiological analysis showed that 13.3% and 16.6% of the samples exceeded the limits for counts of molds and yeasts, and for mesophilic bacteria, respectively. 73.3% presented E. coli above the established sanitary limits.

RESUMO: O interesse no chá verde comercial (Camellia sinensis) (L.) Kuntze (Theaceae) tem aumentado nos últimos anos devido suas propriedades medicinais. No presente estudo utilizamos a microextração em fase sólida, em combinação com a cromatografia gasosa acoplada com espectrometria de massas, como um eficiente método de caracterização de compostos voláteis e efetuamos o controle de qualidade de C. sinensis. Comparamos também a anatomia das folhas de diferentes amostras comerciais de chá verde, as informações de seus rótulos e determinamos a presença de matérias estranhas, de microrganismos, o teor de umidade e de cinzas totais. Entre as trinta amostras analisadas, seis foram identificadas como C. sinensis de acordo com o estudo anatômico; 83,4% não estavam de acordo com os limites especificados para matérias estranhas e 6,6% para o teor de umidade. Todas as amostras apresentaram cinzas totais de acordo com a legislação vigente. As análises evidenciaram diversos compostos orgânicos voláteis distintos, sendo majoritários terpenos, hidrocarbonetos e álcoois. As análises microbiológicas mostraram que 13,3 e 16,6% das amostras excedeu os limites de contagem para bolores e leveduras, e para bactérias mesófilas, respectivamente. Por fim, 73,3% das amostras apresentou Escherichia coli acima dos limites sanitários estabelecidos.

Manual lymphatic drainage and multilayer compression therapy for vulvar edema: a case series.

BACKGROUND AND OBJECTIVE: Vulvar edema is a condition rarely reported and without defined treatment that may result in functional limitation. The aim of the report is to describe a case series of patients with disabling vulvar edema of different etiologies that were treated with manual lymphatic drainage (MLD) and multilayer compression therapy (MCT). CASE SERIES: Four cases of vulvar edema are described: one in a woman with cervical cancer; one in a woman in the postoperative period of bilateral adrenalectomy for pheocromocytoma; and two in pregnant women with preeclampsia. All cases were treated with MLD and MCT during hospitalization. OUTCOMES: Total resolution of the edema occurred in 2 to 5 d of treatment. CONCLUSION: The present case series is the first to report the use of the MLD and MCT in the successful management of female genital edema. This report suggests that the vulvar edemas for these four patients treated with MLD and MCT seem to resolve faster than expected based on previously reported untreated edemas or edemas treated with different therapeutic approaches.

The influence of maternal prenatal and early childhood nutrition and maternal prenatal stress on offspring immune system development and neurodevelopmental disorders.

The developing immune system and central nervous system in the fetus and child are extremely sensitive to both exogenous and endogenous signals. Early immune system programming, leading to changes that can persist over the life course, has been suggested, and other evidence suggests that immune dysregulation in the early developing brain may play a role in neurodevelopmental disorders such as autism spectrum disorder and schizophrenia. The timing of immune dysregulation with respect to gestational age and neurologic development of the fetus may shape the elicited response. This creates a possible sensitive window of programming or vulnerability. This review will explore the effects of maternal prenatal and infant nutritional status (from conception until early childhood) as well as maternal prenatal stress and anxiety on early programming of immune function, and how this might influence neurodevelopment. We will describe fetal immune system development and maternal-fetal immune interactions to provide a better context for understanding the influence of nutrition and stress on the immune system. Finally, we will discuss the implications for prevention of neurodevelopmental disorders, with a focus on nutrition. Although certain micronutrient supplements have shown to both reduce the risk of neurodevelopmental disorders and enhance fetal immune development, we do not know whether their impact on immune development contributes to the preventive effect on neurodevelopmental disorders. Future studies are needed to elucidate this relationship, which may contribute to a better understanding of preventative mechanisms. Integrating studies of neurodevelopmental disorders and prenatal exposures with the simultaneous evaluation of neural and immune systems will shed light on mechanisms that underlie individual vulnerability or resilience to neurodevelopmental disorders and ultimately contribute to the development of primary preventions and early interventions.

Left-right judgment of haptic stimuli representing the human hand

The handedness recognition of visually perceived body parts engages motor representations that are constrained by the same biomechanical factors that limit the execution of real movements. In the present study, we used small plastic cutouts that represented the human hand to investigate the properties of mental images generated during their haptic exploration. Our working hypothesis was that any handedness recognition task that involves body parts depends on motor imagery. Forty-four blindfolded, right-handed volunteers participated in a handedness evaluation experiment using their index finger to explore either the back or palm view of a haptic stimulus that represented the human hand. The stimuli were presented in four different orientations, and we measured the subjects' response times. Our results showed that stimulus configurations that resemble awkward positions of the human hand are associated with longer response times (p < .006), indicating that the haptic exploration of stimuli that represent body parts also leads to motor imagery that is constrained by biomechanical factors.(AU)

Left-right judgment of haptic stimuli representing the human hand

The handedness recognition of visually perceived body parts engages motor representations that are constrained by the same biomechanical factors that limit the execution of real movements. In the present study, we used small plastic cutouts that represented the human hand to investigate the properties of mental images generated during their haptic exploration. Our working hypothesis was that any handedness recognition task that involves body parts depends on motor imagery. Forty-four blindfolded, right-handed volunteers participated in a handedness evaluation experiment using their index finger to explore either the back or palm view of a haptic stimulus that represented the human hand. The stimuli were presented in four different orientations, and we measured the subjects' response times. Our results showed that stimulus configurations that resemble awkward positions of the human hand are associated with longer response times (p < .006), indicating that the haptic exploration of stimuli that represent body parts also leads to motor imagery that is constrained by biomechanical factors

Use of intravaginal electrical stimulation for the treatment of chronic pelvic pain: a randomized, double-blind, crossover clinical trial.

OBJECTIVE: To assess the efficacy of intravaginal electrical stimulation (IVES) or placebo in women with chronic pelvic pain (CPP) with no apparent cause. STUDY DESIGN: A double-blind, crossover, randomized clinical trial in which 26 women were randomly allocated to active (group I, n = 15) or placebo (group II, n = 11) IVES. All women underwent 10 30-minute, twice-weekly sessions. The groups were then crossed over for a further 10 sessions. Pain was evaluated using a visual analog scale (VAS) before and after each series. RESULTS: At the end of the first series, 5 of 11 women who initiated with the placebo had a VAS pain score > 3 (p = 0.0253); however, when they crossed over to active IVES, only 1 had a VAS pain score > 3 at the end of the series (p = 0.0143). In the 15 women who initiated with active IVES, 2 had a score > 3 at the end of the series (p = 0.0005); however, when they crossed over to the placebo, 3 had a VAS pain score > 3 at the end of treatment (p = 0.0833). After the 2 sessions, 54.6% who initiated with placebo and 80% who initiated with active IVES had a VAS pain score < 3. CONCLUSION: Intravaginal electrical stimulation was more effective than a placebo in alleviating pain in women with CPP.

Management of stress urinary incontinence with surface electromyography-assisted biofeedback in women of reproductive age.

BACKGROUND AND PURPOSE: Although surgery has been widely accepted as the treatment of choice for stress urinary incontinence (SUI), there has recently been an increased interest in the conservative management of this condition. The aims of this study were to test the ability of a biofeedback-assisted pelvic-floor muscle exercise (PFME) program to affect symptoms of SUI in premenopausal women and to evaluate a training program that might lead to successful outcomes in a relatively limited number of sessions. SUBJECTS: Twenty-six women with SUI were treated with PFME with surface electromyography (sEMG)-assisted biofeedback. All participants were of reproductive age and were treated individually for 12 sessions. METHODS: results were evaluated with a 7-day voiding diary, a 1-hour pad test, pelvic-floor muscle strength measurements, sEMG amplitudes, a leakage index, and a quality-of-life questionnaire. These variables were compared before and after the intervention. RESULTS: The frequency of urine loss, the occurrence of nocturia, and the number of pads required decreased significantly after the intervention. Objective cure was found in 61.5% of women. There was a significant improvement in the quality of life, in pelvic-floor muscle strength, and in the sEMG amplitudes of all contractions throughout the intervention. DISCUSSION AND CONCLUSION: A relatively short-term intervention of PFME with sEMG-assisted biofeedback appeared to be helpful in relieving symptoms of SUI in premenopausal women and represents a reasonable conservative management option.

[Transvaginal electrical stimulation of the pelvic floor in the treatment of stress urinary incontinence: clinical and ultrasonographic assessment]. / Eletroestimulação transvaginal do assoalho pélvico no tratamento da incontinência urinária de esforço: avaliações clínica e ultra-sonográfica.

BACKGROUND: The purpose of this study was to evaluate the efficacy of transvaginal electrical stimulation on the treatment of women with stress urinary incontinence (SUI) in selected cases. METHODS: Twenty two women with primary stress urinary incontinence diagnosed by urodynamic study were submitted to transvaginal electrical stimulation. The mean age was 49 years old (range 22 to 74). The treatment consisted of two weekly sessions of stimulation, each 20 minutes long, during 8 weeks. Women were evaluated through history, clinical and urogynecological exam, weekly registration of frequency of urinary leakage, stress test and transperineal ultrasound before and after treatment. A pulse of 700 sec. was used, frequency of 50 Hz and intensity varying from 12 to 53 mA according to the women's sensitivity. RESULTS: Eighteen of 22 subjects (81.7%) were satisfied with electrical stimulation and frequency of urinary incontinence reduced significantly (p<0.01). Stress test was negative in 77.2% of the women after treatment. Valsalva leak-point pressure was higher in 14 (63.3%) women after treatment, although not reaching statistical significance (p=0.37). Bladder neck mobility did not change significantly before and after electrical stimulation (p=0.30). CONCLUSION: Transvaginal electrical stimulation of the pelvic floor is an effective and safe treatment to women with SUI without sphincter deficiency, with significant reduction of the frequency of urinary leakage.

Eletroestimulação transvaginal do assoalho pélvico no tratamento da incontinência urinária de esforço: avaliações clínica e ultra-sonográfica / Transvaginal electrical stimulation of the pelvic floor in the treatment of stress urinary incontinence: clinical and ultrasonographic assessment

OBJETIVO: Verificar o efeito da eletroestimulação transvaginal no tratamento de mulheres com incontinência urinária de esforço (IUE) adequadamente selecionadas. MÉTODOS: Vinte e duas mulheres com IUE, diagnosticadas através de estudo urodinâmico, foram submetidas à eletroestimulação transvaginal do assoalho pélvico. A média das idades foi de 49 anos (22 a 74 anos). Foram realizadas duas sessões semanais, com duração de 20 minutos cada, durante oito semanas. Todas as mulheres foram avaliadas por anamnese, exames clínico e uroginecológico, registro semanal dos episódios de perda urinária, teste de esforço e ultra-sonografia transperineal antes e após o tratamento. Os parâmetros elétricos utilizados foram: pulso de 700 microssegundos, freqüência de 50 Hertz e intensidade com variação entre 12 e 53 miliamperes, de acordo com a sensibilidade da mulher. RESULTADOS: Consideraram-se satisfeitas com a eletroestimulação 18 mulheres (81,7 por cento), havendo redução significativa do número de perdas urinárias (p<0,01). O teste de esforço foi negativo em 77,2 por cento das mulheres após o tratamento. A pressão de perda sob esforço aumentou em 14 mulheres (64 por cento), não alcançando, entretanto, valor com significância estatística (p = 0,37). A avaliação ultra-sonográfica não demonstrou diferença significativa na mobilidade do colo vesical antes e após as sessões de eletroestimulação (p = 0,30). CONCLUSÃO: A eletroestimulação transvaginal do assoalho pélvico representou uma alternativa terapêutica efetiva e segura às mulheres com IUE sem defeito esfincteriano, com diminuição significativa dos episódios de perda urinária