HISTIOCITOSE CEFÁLICA BENIGNA

Benign cephalic histiocytosis is a type of non-Langerhans histiocytitic disorder. Of unknown etiology, is a self-healing disease, arising in childhood and is characterized by papular lesions primarily affecting the face. We describe a 10-month-old girl who presented with 6 months of evolution, yellow-red papules asymptomatic, on her face. The histological and immunohistochemical study showed the presence of a diffuse infiltration of histiocytes, throu- ghout the dermis, negative for the protein S100 and CD1a and positive for CD68. Given the diagnosis of benign cepha- lic histiocytosis, we chose an expectant attitude verifying complete regression after one year of follow-up. Since its description by Gianotti, et al. in 1971, only 40 cases were reported in the literature. KEYWORDS – Histiocytosis; Non-Langerhans-cell; Facial dermatoses; Skin diseases. A histiocitose cefálica benigna faz parte do grupo das histiocitoses de células não-Langerhans. De etio- logia desconhecida, é uma doença autolimitada, que surge na infância e se caracteriza por lesões papulosas que afetam fundamentalmente a face. Descrevemos o caso clínico de uma criança de 10 meses de idade que apresentava desde há 6 meses lesões papu- losas, eritemato-amareladas, assintomáticas e de localização exclusiva à face. O estudo histológico e imuno-histo- químico revelou a presença de um infiltrado difuso de histiócitos em toda a derme, negativo para a proteína S100 e CD1a e positivo para CD68. Perante o diagnóstico de histiocitose cefálica benigna, optou-se por uma atitude expec- tante verificando-se regressão clínica completa após um ano de seguimento. Desde a sua descrição por Gianotti, et al. em 1971, apenas 40 casos foram descritos na literatura.PALAVRAS-CHAVE – Histiocitose cefálica benigna; Histiocitoses de células não-Langerhans; Doença autolimitada.

DERMATIOMIOSITE COM DOENÇA INTERSTICIAL PULMONAR - UMA ASSOCIAÇÃO COM ANTI-MDA-5

Dermatomyositis is a systemic autoimmune disease with cutaneous, muscular and pulmonary involvement. MDA-5 has recently been described as a specific dermatomyositis target antigen associated with a higher risk for interstitial lung disease. A 54-year-old female patient presented with proximal muscular weakness, myalgia and cutaneous signs of dermatomyositis. She presented with high aldolase and CK levels, and the muscular biopsy and electromyography were consistent with dermatomyositis. Pulmonary CT scan showed ground glass appearance. Autoimmunity revealed strong anti-MDA-5 positivity. No neoplasm was detected. She began treatment with prednisolone 20mg, hydroxicloroquine 400mg and methotrexate 20mg/week with very little improvement. She then began IVIG, with better clinical response. The authors report a case of dermatomyositis with interstitial lung disease associated with anti-MDA-5, an association unknown until recently.A dermatomiosite (DM) é uma doença auto-imune com atingimento cutâneo, muscular e pulmonar. O MDA-5 (melanoma differentiation-associated protein 5) foi recentemente descrito como alvo de uma resposta serológica específica da DM que está associada a maior risco de doença pulmonar. Doente do sexo feminino, 54 anos, com fraqueza muscular proximal, mialgias, incapacidade funcional marcada e sinais cutâneos de dermatomiosite. Analiticamente apresentava elevação da aldolase e CK, e electromiografia e biópsia muscular compatíveis com dermatomiosite. A TAC pulmonar revelou padrão em vidro despolido. O estudo de auto-imunidade mostrou anti-MDA-5 positivo forte. Não foi detectada neoplasia. O tratamento com prednisolona 20mg, hidroxicloroquina 400mg e metotrexato 20mg/semana levou a melhoria apenas discreta do quadro, pelo que se introduziu imunoglobulina endovenosa (IgIV), com melhor resposta. Descreve-se o caso de uma doente com DM e doença intersticial pulmonar grave, com anti-MDA-5 positivo, chamando a atenção para esta associação até há pouco desconhecida

Excess hospitalizations and mortality associated with seasonal influenza in Portugal, 2008–2018

Funding Information: The BARI study was funded by Sanofi. Funding Information: CG, MM, HB, and CDC are Sanofi employees. MC, HL, and GB are IQVIA employees. FF, JCDS, CR, JFR, and CRC have received fees from Sanofi. JCDS reports Advisory Board from Boehringer Ingelheim; personal fees and Advisory Board from GSK; grants, personal fees, and Advisory Board from AstraZeneca; personal fees and Advisory Board from Bial; non-financial support from Mundipharma; personal fees from Sanofi; Advisory Board from Novartis, outside the submitted work. FF reports Advisory Board and personal fees from Sanofi, Pfizer, MSD, Gilead and personal fees or non-financial support from Bial, AstraZeneca, GSK, Novartis, Boehringer Ingelheim, Tecnifar, Lilly, Bayer, and Roche outside the submitted work. Publisher Copyright: © 2022, The Author(s).Background: Influenza can have a domino effect, triggering severe conditions and leading to hospitalization or even death. Since influenza testing is not routinely performed, statistical modeling techniques are increasingly being used to estimate annual hospitalizations and deaths associated with influenza, to overcome the known underestimation from registers coded with influenza-specific diagnosis. The aim of this study was to estimate the clinical and economic burden of severe influenza in Portugal. Methods: The study comprised ten epidemic seasons (2008/09–2017/18) and used two approaches: (i) a direct method of estimating the seasonal influenza hospitalization incidence, based on the number of National Health Service hospitalizations with influenza-specific International Classification of Diseases (ICD) codes (ICD-9: 487–488; ICD-10: J09-J11), as primary or secondary diagnosis; (ii) an indirect method of estimating excess hospitalizations and deaths using broader groups of ICD codes in time-series models, computed for six age groups and four groups of diagnoses: pneumonia or influenza (ICD-9: 480–488, 517.1; ICD-10: J09–J18), respiratory (ICD-9: 460–519; ICD-10: J00–J99), respiratory or cardiovascular (R&C, ICD-9: 390–459, 460–519; ICD-10: I00–I99, J00–J99), and all-cause. Means are reported excluding the H1N1pdm09 pandemic (2009/10). Results: The mean number of hospitalizations coded as due to influenza per season was 1,207, resulting in 11.6 cases per 100,000 people. The mean direct annual cost of these hospitalizations was €3.9 million, of which 78.6% was generated by patients with comorbidities. Mean annual influenza-associated R&C hospitalizations were estimated at 5356 (min: 456; max: 8776), corresponding to 51.5 cases per 100,000 (95% CI: 40.9–62.0) for all age groups and 199.6 (95% CI: 163.9–235.8) for the population aged ≥ 65 years. The mean direct annual cost of the estimated excess R&C hospitalizations was €15.2 million for all age groups and €12.8 million for the population aged ≥ 65 years. Mean annual influenza-associated all-cause deaths per 100,000 people were estimated at 22.7 for all age groups. Conclusions: The study findings suggest that there is an under-detection of influenza in the Portuguese population. A high burden of severe influenza remains to be addressed, not only in the elderly population but also in younger people.publishersversionpublishe

Is diet partly responsible for differences in COVID-19 death rates between and within countries?

Correction: Volume: 10 Issue: 1 Article Number: 44 DOI: 10.1186/s13601-020-00351-w Published: OCT 26 2020Reported COVID-19 deaths in Germany are relatively low as compared to many European countries. Among the several explanations proposed, an early and large testing of the population was put forward. Most current debates on COVID-19 focus on the differences among countries, but little attention has been given to regional differences and diet. The low-death rate European countries (e.g. Austria, Baltic States, Czech Republic, Finland, Norway, Poland, Slovakia) have used different quarantine and/or confinement times and methods and none have performed as many early tests as Germany. Among other factors that may be significant are the dietary habits. It seems that some foods largely used in these countries may reduce angiotensin-converting enzyme activity or are anti-oxidants. Among the many possible areas of research, it might be important to understand diet and angiotensin-converting enzyme-2 (ACE2) levels in populations with different COVID-19 death rates since dietary interventions may be of great benefit.Peer reviewe

The Emerging Role of Menstrual-Blood-Derived Stem Cells in Endometriosis

The human endometrium has a complex cellular composition that is capable of promoting cyclic regeneration, where endometrial stem cells play a critical role. Menstrual blood-derived stem cells (MenSC) were first discovered in 2007 and described as exhibiting mesenchymal stem cell properties, setting them in the spotlight for endometriosis research. The stem cell theory for endometriosis pathogenesis, supported by the consensual mechanism of retrograde menstruation, highlights the recognized importance that MenSC have gained by potentially being directly related to the genesis, development and maintenance of ectopic endometriotic lesions. Meanwhile, the differences observed between MenSC in patients with endometriosis and in healthy women underlines the applicability of these cells as a putative biomarker for the early diagnosis of endometriosis, as well as for the development of targeted therapies. It is expected that in the near future MenSC will have the potential to change the way we manage this complex disease, once their long-term safety and effectiveness are assessed

Fotoprotecção na Criança

Resumo: Do espectro da radiação solar que atinge a superfície terrestre, a radiação ultravioleta (UVA e UVB) é a principal responsável pelas reacções cutâneas benéficas e nefastas. Os efeitos biológicos dos raios UV sobre a pele dividem-se em fenómenos precoces, como acção térmica, anti-raquítica, pigmentação imediata e acção anti depressiva, fenómenos tardios, como o eritema actínico ou queimadura solar, pigmentação retardada, hiperplasia epidérmica, imunossupressão, e efeitos a longo prazo, como heliodermia e fotocarcinogénese. Vários agentes interferem na transmissão da radiação UV à pele humana. De entre estes, destacam-se os agentes fotoprotectores naturais existentes na atmosfera e na pele, agentes fotoprotectores físicos e os filtros UV presentes nos protectores solares de aplicação tópica. A acção carcinogénica da radiação UV é actualmente reconhecida e indiscutível. A exposição a este tipo de radiação é a principal causa para o desenvolvimento de cancro cutâneo não melanoma, existindo também uma associação entre o desenvolvimento de melanoma maligno e exposição solar intensa e curta, que resulta em queimaduras solares, em idade pediátrica. Deste modo, a promoção de programas de fotoeducação e fotoprotecção é importante na prevenção da redução do cancro cutâneo, sendo premente a necessidade de sensibilização não só dos prestadores de cuidados de saúde, mas também da população em geral, nomeadamente pais, educadores e as próprias crianças, para que adquiram hábitos de convívio saudável com o sol