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Comparison of Rhinitis Treatments Using MASK-air ® Data and Considering the Minimal Important Difference
No full textBackground: Different treatments exist for allergic rhinitis (AR), including pharmacotherapy and allergen immunotherapy (AIT), but they have not been compared using direct patient data (i.e., "real-world data"). We aimed to compare AR pharmacological treatments on (i) daily symptoms, (ii) frequency of use in co-medication, (iii) visual analogue scales (VASs) on allergy symptom control considering the minimal important difference (MID) and (iv) the effect of AIT.
Methods: We assessed the MASK-air® app data (May 2015-December 2020) by users self-reporting AR (16-90 years). We compared eight AR medication schemes on reported VAS of allergy symptoms, clustering data by the patient and controlling for confounding factors. We compared (i) allergy symptoms between patients with and without AIT and (ii) different drug classes used in co-medication.
Results: We analysed 269,837 days from 10,860 users. Most days (52.7%) involved medication use. Median VAS levels were significantly higher in co-medication than in monotherapy (including the fixed combination azelastine-fluticasone) schemes. In adjusted models, azelastine-fluticasone was associated with lower average VAS global allergy symptoms than all other medication schemes, while the contrary was observed for oral corticosteroids. AIT was associated with a decrease in allergy symptoms in some medication schemes. A difference larger than the MID compared to no treatment was observed for oral steroids. Azelastine-fluticasone was the drug class with the lowest chance of being used in co-medication (adjusted OR = 0.75; 95% CI = 0.71-0.80).
Conclusion: Median VAS levels were higher in co-medication than in monotherapy. Patients with more severe symptoms report a higher treatment, which is currently not reflected in guidelines.info:eu-repo/semantics/publishedVersio
The Lockdown Impact on the Relations between Portuguese Parents and Their 1- to 3-Year-Old Children during the COVID-19 Pandemic
No full textMany countries have applied mandatory confinement measures in response to the COVID-19 pandemic, such as school and kindergarten closures, which confined families to their homes. The study concerns the impacts of the first COVID-19 lockdown on the relationships between Portuguese parents and their children, in a non-clinical population composed of fathers and mothers of children between the ages of 12 months and 3 years and 364 days. An online questionnaire (set by the research team) and the Parenting Daily Hassles Scale (PDHS) concerning the confinement period were applied between 17 June and 29 July 2020. To assess the impacts of the lockdown, outcomes regarding the impacts perceived by the parents, the potential regression in the development of children, and the willingness to promote changes in family routines in the future, were considered. Of the total sample (n = 1885), 95.4% of the parents (n = 1798) said that, after confinement, the relationship with their children had improved or remained similar to the pre-confinement period; 97.3% (n = 1835) noticed positive changes in the development of their children, and 63.7% (n = 1200) noted that the relationships with their children during the confinement period would lead to some changes in family routines in the future. Multivariate regression analyses showed that most of the sociodemographic variables chosen were not associated with the outcomes. However, significant levels of pressure over parenting and parental overload (reported by high scores in the PDHS intensity and frequency scales), challenging behaviors of the children, and the impacts they had on parental tasks had negative influences on the studied outcomes. On the contrary, the number of adults living with their children, the perceptions regarding the development of their children, and sharing new experiences with them were significant factors for positively-perceived impacts on the relationships between them or in the desire to bring about changes in family routines in the future. The impacts of the lockdown on the relationships between parents and children aged between 1 and 3 years old were more dependent on relational aspects and on the parents' sense of competence in exercising parental functions. We conclude that, despite the increased demands imposed by the lockdown, nearly all of the parents evaluated the quality of the relationship with their children as positive after this period.info:eu-repo/semantics/publishedVersio
Acquired Cytomegalovirus Infection in an Extremely Low-Birth Weight Infant Presenting with a Severe Sepsis-Like Syndrome
No full textCytomegalovirus infection is one of the most common congenital infections worldwide. Moreover, it seems to be an important cause of postnatally acquired infection. Perinatal transmission can occur intrapartum (from the birth canal), from a blood transfusion, via maternal breast milk, or from close contact with infected people. The risk of breast milk-acquired cytomegalovirus infection is higher in countries with a high prevalence of cytomegalovirus immunoglobulin G-positive women.
Usually, acquired cytomegalovirus infection is asymptomatic, especially in term infants. However, preterm infants can present with a sepsis-like syndrome and multiple organ involvement. A high index of suspicion is required to make an early diagnosis.
Therapeutic guidelines for symptomatic postnatal cytomegalovirus infection are not yet available. More studies are required to understand the long-term sequelae of postnatally acquired cytomegalovirus infection and know which is the best strategy to avoid cytomegalovirus post-natal transmission.info:eu-repo/semantics/publishedVersio
Primary Anti-Phospholipid Antibody Syndrome: Real-World Defining Features of Rethrombosis in the Course of Disease
No full textObjective: We aimed to identify features that allow differentiation of primary antiphospholipid syndrome (PAPS) patients that suffer recurrent thrombotic events (RTE) despite anticoagulation, from the other diagnosed PAPS patients.
Methods: This was an exploratory study of anticoagulated PAPS patients attending an Autoimmune Diseases Unit (1998-2018). From 2016, anti-phospholipid antibodies and lupus anticoagulant were determined for each patient at consecutive visits, collected together with retrospective clinical characteristics, laboratory, and therapeutic markers and compared according to the occurrence of thrombotic events during follow-up.
Results: Overall, two thirds of the patients were female, 93% were Caucasian, with a median age of 40 years at diagnosis, for a median time of 11.5 years in follow-up. Out of 54 patients, 10 were identified with RTE. There were no significant differences among the RTE and non-RTE patients as far as classical risk factors for clotting disorders. The RTE group was characterized by a higher proportion of younger patients, male sex and positivity for all laboratory markers, and initially and over follow-up as well as a sustained high-risk profile based on APS laboratory markers. Anticardiolipin IgG at onset was the only statistically significant marker of the RTE group. At the end of follow-up, consistent reversion to negative status was a rare event, observed in 20% of RTE vs. 25% of non-RTE patients.
Conclusions: Despite therapy, we were able to identify features associated to thrombotic events in patients with PAPS. Prospectively regular clinical and laboratory monitoring might be warranted in order to treat APS more assertively.info:eu-repo/semantics/publishedVersio
Spontaneous Intracranial Hypotension and Multi-Level Cervical and Lumbar Epidural Blood Patches: A Case Report
No full textSpontaneous intracranial hypotension (SIH) is a neurologic condition where the intracranial pressure is reduced due to a loss of cerebrospinal fluid from its reservoir, the intrathecal space, to surrounding tissues. It is commonly characterized by an incapacitating headache, phono-photophobia, nausea, and vomiting, commonly refractory to medical treatment and requires further investigation. We describe the case of a healthy young man who presented to the emergency room with a postural headache, accompanied by nausea, vomiting, and phono-photophobia. Brain computed tomography (CT) imaging study was unremarkable and he was initially treated symptomatically. Because of persisting pain even on medical treatment, additional imaging studies, including a myelo-CT scan, were performed and a diagnosis of multi-level cerebrospinal fluid fistulas was made. To treat the underlying cause, a first epidural blood patch (EBP) was initially performed at C7-T1 with 20 mL of autologous blood, but failed to provide complete symptomatic relief. Months later, a second EBP was conducted at C6-C7 with higher volume (30 mL) but as in the first EBP this procedure too did not result in total resolution of the headache and accompanying symptoms. Since there was no surgical indication from Orthopedics and Neurosurgery and the symptoms persisted, a third EBP was carried out, this time at a lumbar level (L2-L3) with infusion of 60 mL of blood so the upper dorsal and cervical epidural space was reached. This resulted in a better symptom relief, allowing the patient to now carry out his normal activities with only residual pain. The need for repeat procedures is one of the pitfalls of the blood patching technique. If possible, it should be performed at the level of the documented fistula, but always with safety in mind and by experienced hands, especially when cervical levels are concerned. A consensus has not been reached regarding the blood volume to be administered; however, any discomfort or pain reported by the patient should be seen as warning sign and the procedure should be interrupted. Although not being a perfect solution, EBP can completely or partially resolve SIH symptoms, without the need for surgical intervention.info:eu-repo/semantics/publishedVersio
Horner's Syndrome as a Complication of Ultrasound-Guided Central Cannulation: A Case Report
No full textCannulation of the internal jugular vein is often necessary for the management of critically ill patients. Despite being a very common procedure and performed more and more safely, several complications still occur. Horner's Syndrome (HS) is one of those complications described before the use of ultrasound as a method of guidance. HS is caused by functional interruption of sympathetic nerve supply to the eye, leading to a classic triad of ipsilateral ptosis, miosis, and anhidrosis. We present the case of a patient, in need of emergent surgery to control the hemorrhagic focus after delivery, with a transient HS secondary to internal jugular vein cannulation under real-time ultrasound guidance.info:eu-repo/semantics/publishedVersio
Suggestive Evidence for an Antidepressant Effect of Metreleptin Treatment in Patients with Lipodystrophy
No full textIntroduction: Lipodystrophy (LD) syndromes are rare heterogeneous disorders characterized by reduction or absence of subcutaneous fat, low or nondetectable leptin concentrations in blood and impaired hunger/satiety regulation. Metreleptin treatment reverses metabolic complications and improves eating behavior in LD. Because depression in anorexia nervosa (AN), which is also characterized by hypoleptinemia, improves substantially upon treatment with metreleptin, we hypothesized that metreleptin substitution may be associated with an antidepressant effect in patients with LD, too.
Methods: In this ancillary study, 10 adult patients with LD were treated with metreleptin. To assess depressive symptoms, the self-rating questionnaire Beck's Depression Inventory (BDI) was filled in at preestablished time points prior (T1) and after initiation of metreleptin (T2: 1 week; T3: 4 weeks; T4: 12 weeks) dosing. The differences between time points were tested with nonparametric Friedman's analysis of variance. Sensitivity analyses were performed upon exclusion of the BDI items addressing appetite and weight changes.
Results: According to their BDI scores, 4 patients had mild depression and 2 had moderate depression at baseline. Friedman's test revealed significant differences in BDI scores between the four time points. Post hoc analyses revealed that the difference between T1 and T3 was significant upon Bonferroni correction (p = 0.034, effect size r = 0.88). The sensitivity analyses upon exclusion of the appetite and weight change items again revealed a significant Friedman's test and significant Bonferroni corrected differences in the revised BDI scores between T1 versus T2 (p = 0.002, r = 0.99) and T1 versus T3 (p = 0.007, r = 0.79).
Discussion/conclusion: Our study for the first time revealed suggestive evidence for an antidepressant effect of metreleptin in patients with LD. Metreleptin caused a rapid drop in depression scores within 1 week of treatment. A reduction of the depression score was also observed in 2 of the 3 LD patients whose BDI scores were in the normal range before start of the treatment. The reduction in total scores of BDI was still apparent after 3 months (T4) of dosing. This observation matches findings obtained in clinical case studies of AN patients, in whom depression scores also dropped during the first week of metreleptin treatment. It needs to be noted that by the nature of this observational study without a placebo group, nonspecific treatment expectation affecting mood cannot fully be ruled out.info:eu-repo/semantics/publishedVersio
Acute Disseminated Panniculitis Associated with Alpha-1 Antitrypsin Deficiency
No full textAlpha-1 antitrypsin deficiency, although one of the most common genetic diseases, is a very rare and often undiagnosed cause of panniculitis. The authors present a case characterized by an acute involvement of several areas in the thorax, abdomen, and limbs, occurring after repetitive trauma of the perineal area caused by a long period of cycling. After performing the differential diagnosis and establishing etiology, the patient was started on augmentation therapy with plasma-derived synthetic human alpha-1 proteinase inhibitor and the disease has been under control since then. We recommend lifelong treatment with this medication. At the end of a 10-year follow-up, there has been no evidence of pulmonary emphysema or liver disease. The authors perform a concise review of the genetic and pathogenic mechanisms behind this disease, with a special focus on panniculitis and its treatment.info:eu-repo/semantics/publishedVersio
A Realidade da Fisioterapia Vestibular em Portugal: Análise Preliminar
No full textIntrodução: A Reabilitação Vestibular (RV) é uma abordagem terapêutica dirigida aos sinais e sintomas causados por disfunção vestibular, com o objetivo de acelerar o processo natural de compensação baseada nos mecanismos centrais de neuroplasticidade. Visando maximizar a autonomia e independência funcional, os fisioterapeutas integraram as equipas com os primeiros exercícios descritos em 1940 por Cawthorne & Cooksey, a manobra de diagnóstico da vertigem posicional paroxística benigna em 1952 por Dix Hallpike, muito embora a grande evolução desta área tenha ocorrido com as técnicas desenvolvidas por A.
Sémont na década de 1970. Como área de intervenção muito específica importa caracterizar a oferta que existe a nível nacional.
Objetivo: Identificar e caracterizar os Fisioterapeutas que intervêm na área de RV em Portugal.
Métodos: Estudo de coorte transversal, realizado através de questionário online divulgado pela Associação Portuguesa de Fisioterapeutas e redes sociais. Foram incluídos Fisioterapeutas com prática clínica e/ou formação na área de RV. A participação voluntária foi validada com o consentimento informado. A análise dos dados foi realizada através de métodos analíticos, não se verificando missing data.
Resultados: 44 (36 mulheres) Fisioterapeutas responderam ao questionário, permitindo caracterizar a formação académica, a formação específica e a Intervenção dos Fisioterapeutas nesta área. No que respeita ao grau académico, 1 é doutorado, 11 são mestres, 31 são licenciados e 1 é bacharel. 84.1% dos Fisioterapeutas responderam ter formação na área de RV dos quais 18.2% desde a licenciatura e 77.3% com formação pós graduada. 29.5% dos Fisioterapeutas realizaram estágios profissionais na área. Foi
reportada uma mediana de 4±8 (0.08 27) anos de prática clínica em RV. A região Norte, Centro e Lisboa e Vale do Tejo concentram a grande maioria dos profissionais (85.4%). 68.2% identificaram como insuficiente a formação especifica na área de RV a nível nacional, sugerindo cursos de atualização, formação contínua e/ou pós graduada.
Conclusão: A RV é identificada como uma área específica de interesse do fisioterapeuta, com necessidades de formação básica e avançada. A maioria dos fisioterapeutas que intervém nesta área apresenta formação específica, muito embora salientem que a mesma não seja de fácil acesso. Apesar de reduzida e não representativa, esta amostra identifica a necessidade de disponibilização de formação no ensino pré e pós graduado. Tendo sido demonstrado um crescente investimento na sensibilização para a área nos planos de
estudos do ensino pré graduado dos cursos de Fisioterapia, não se refletindo ainda neste estudo. Desta forma, o reduzido número de profissionais a intervir nesta área em todo o país e as necessidades de formação/atualização expressas nesta área, justificam a aposta na formação na área de RV.info:eu-repo/semantics/publishedVersio
Tumor Inflamatório Miofibroblástico da Via Biliar Principal
No full textInflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and
metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)].
Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia
and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology
showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with
an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His
liver function tests have normalized and follow-up tests are unremarkable.info:eu-repo/semantics/publishedVersio