22 research outputs found
Choque séptico por Pseudomonas aeruginosa associado a éctima gangrenosa em criança com agamaglobulinemia
Ecthyma gangrenosum (EG) due to Pseudomonas aeruginosa is a rare and invasive infection that can be associated with agammaglobulinemia. The cornerstone of the treatment is based on prompt recognition with appropriate antibiotic coverage and intravenous immunoglobulin. The authors report a case of EG emphasizing the clinical and therapeutic aspects of this condition.Ăctima Gangrenosa (EG) por Pseudomonas aeruginosa Ă© uma infecção rara e invasiva que pode ser associada com agamaglobulinemia. O tratamento fundamental Ă© baseado no pronto reconhecimento com cobertura de antibiĂłtico apropriada e imunoglobulina intravenosa. Os autores relatam caso de EG dando ĂȘnfase aos aspectos clĂnicos e terapĂȘuticos desta condição
Meningite neonatal por Streptococcus pyogenes e trombose de seio sagital: relato de caso
COVID-19 symptoms at hospital admission vary with age and sex: results from the ISARIC prospective multinational observational study
Background:
The ISARIC prospective multinational observational study is the largest cohort of hospitalized patients with COVID-19. We present relationships of age, sex, and nationality to presenting symptoms.
Methods:
International, prospective observational study of 60â109 hospitalized symptomatic patients with laboratory-confirmed COVID-19 recruited from 43 countries between 30 January and 3 August 2020. Logistic regression was performed to evaluate relationships of age and sex to published COVID-19 case definitions and the most commonly reported symptoms.
Results:
âTypicalâ symptoms of fever (69%), cough (68%) and shortness of breath (66%) were the most commonly reported. 92% of patients experienced at least one of these. Prevalence of typical symptoms was greatest in 30- to 60-year-olds (respectively 80, 79, 69%; at least one 95%). They were reported less frequently in children (â€â18 years: 69, 48, 23; 85%), older adults (â„â70 years: 61, 62, 65; 90%), and women (66, 66, 64; 90%; vs. men 71, 70, 67; 93%, each Pâ<â0.001). The most common atypical presentations under 60 years of age were nausea and vomiting and abdominal pain, and over 60 years was confusion. Regression models showed significant differences in symptoms with sex, age and country.
Interpretation:
This international collaboration has allowed us to report reliable symptom data from the largest cohort of patients admitted to hospital with COVID-19. Adults over 60 and children admitted to hospital with COVID-19 are less likely to present with typical symptoms. Nausea and vomiting are common atypical presentations under 30 years. Confusion is a frequent atypical presentation of COVID-19 in adults over 60 years. Women are less likely to experience typical symptoms than men
Pseudomonas aeruginosa septic shock associated with ecthyma gangrenosum in an infant with agammaglobulinemia
Ecthyma gangrenosum (EG) due to Pseudomonas aeruginosa is a rare and invasive infection that can be associated with agammaglobulinemia. The cornerstone of the treatment is based on prompt recognition with appropriate antibiotic coverage and intravenous immunoglobulin. The authors report a case of EG emphasizing the clinical and therapeutic aspects of this condition
AlteraçÔes da articulação temporomandibular e suas repercussÔes orofaciais em pacientes portadores de artrite idiopåtica juvenil Temporomandibular joint alterations and their orofacial complications in patients with juvenile idiopathic arthritis
Os pacientes com artrite idiopĂĄtica juvenil (AIJ) podem apresentar alteraçÔes no metabolismo Ăłsseo e no crescimento esqueletal, alĂ©m de danos na articulação temporomandibular (ATM), o que pode gerar alteraçÔes extraorais e/ou intraorais, acarretando distĂșrbios craniofaciais. Nosso objetivo Ă© promover uma revisĂŁo da literatura sobre as alteraçÔes orofaciais em pacientes com AIJ. Dentre os distĂșrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular, geradas por disfunçÔes na regiĂŁo da ATM, parecem de grande prevalĂȘncia nesses pacientes. As alteraçÔes mais encontradas sĂŁo: retrognatismo, micrognatia, mordida aberta anterior, apinhamento dentĂĄrio, assimetria facial e limitação de abertura de boca. Assim, o reumatologista torna-se uma peça-chave na detecção precoce dessas disfunçÔes, ajudando no encaminhamento do paciente ao dentista. O diagnĂłstico, por sua vez, deve ser realizado pelo ortodontista, utilizando exames clĂnico e de imagem, o que possibilita tratamento precoce e prognĂłstico favorĂĄvel. DistĂșrbios na ATM devem ser tratados de forma multidisciplinar, incluindo tratamento farmacolĂłgico para o controle da dor e tratamento odontolĂłgico por meio de aparelhagem funcional e/ou ortodĂŽntica, fisioterapĂȘutico e por vezes fonoaudiolĂłgico. ConcluĂmos que dentre os distĂșrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular geradas por disfunçÔes na regiĂŁo da ATM parecem de grande prevalĂȘncia. Tais disfunçÔes podem promover, principalmente, mordida aberta, retrusĂŁo mandibular, micrognatia, apinhamento dentĂĄrio e assimetria facial. O reumatologista pode detectar tais alteraçÔes precocemente, encaminhando o paciente de imediato a uma equipe que deve ser, preferencialmente, multidisciplinar, composta por ortodontista, fisioterapeuta e fonoaudiĂłlogo, visando reduzir futuras complicaçÔes oclusais e de crescimento mandibular.<br>Patients with juvenile idiopathic arthritis (JIA) can have alterations in bone metabolism and skeletal growth, as well as damage to the temporomandibular joint (TMJ), which can generate extra and/or intraoral alterations, resulting in craniofacial disorders. Our goal is to carry out a review of the literature on orofacial alterations in patients with JIA. Among the orofacial disorders in patients with JIA, alterations in mandibular growth, caused by dysfunctions in the TMJ region, seem highly prevalent in these patients. The most often found alterations are: retrognathia, micrognathia, anterior open bite, dental crowding, facial asymmetry and mouth opening limitation. Thus, the rheumatologist becomes a key agent in the early detection of these disorders, helping with patient referral to a dentist. The diagnosis, in turn, should be performed by the orthodontist, using clinical examination and imaging methods, allowing early treatment and a favorable prognosis. TMJ disorders should be treated by a multidisciplinary team, including pharmacological treatment for pain control and dental care through functional appliance and/or orthodontic therapy, physical therapy and sometimes, speech therapy. We conclude that among the orofacial disorders in patients with JIA, alterations in mandibular growth generated by dysfunctions in the TMJ region seem highly prevalent. Such dysfunctions can cause mainly open bite, mandibular retrusion, micrognathia, dental crowding and facial asymmetry. The rheumatologist can detect these alterations at an early stage, with immediate patient referral to a team that should preferably be a multidisciplinary one, consisting of an orthodontist, physical therapist and speech therapist, to reduce future occlusal and mandibular growth complications
Perfil da doença de Kawasaki em crianças encaminhadas para dois serviços de reumatologia pediåtrica do Rio de Janeiro, Brasil
OBJETIVOS: Descrever uma população de crianças com diagnĂłstico de doença de Kawasaki (DK) atendida em centros de reumatologia pediĂĄtrica do Rio de Janeiro. Analisar o perĂodo de atraso no diagnĂłstico e inĂcio do tratamento, devido Ă dificuldade de distinguir DK de outras doenças febris comuns da infĂąncia; e o impacto deste atraso na frequĂȘncia de sequelas coronarianas. MĂTODOS: Os dados analisados incluĂram: nome, sexo, idade, data do inicio dos sintomas e da admissĂŁo no serviço especializado, sintomatologia, evolução clĂnica, uso de Imunoglobulina Endovenosa (IGEV) e complicaçÔes coronarianas. RESULTADOS: Dos 125 casos estudados, 63% eram meninos. 40% tinham menos de 2 anos no momento do diagnĂłstico. O intervalo mĂ©dio entre o inicio dos sintomas e o diagnĂłstico de DK foi de 12 dias (duração mĂ©dia da febre = 14 dias). Dos casos estudados, 22,4% receberam o diagnostico de DK antes do atendimento em serviço especializado; nos demais, as hipĂłteses diagnosticas iniciais incluĂam: infecçÔes bacterianas (60%), virais (12%), outras doenças reumatolĂłgicas (4%) e reaçÔes adversas Ă vacinação (1,6%). Em 85.6 % dos casos registrou-se o tratamento realizado, sendo administrada IGEV em 46,7%, e a partir do 10Âș dia em 21,5% dos casos. Dos 20 pacientes apresentando sequelas coronarianas, 9 tiveram diagnĂłstico tardio, incluindo 3 iniciando tratamento apĂłs o 10Âș dia e 6 sem tratamento. NĂŁo encontramos associação significativa entre a frequĂȘncia de sequelas coronarianas e: sexo; idade; critĂ©rios clĂnicos; tratamento com IGEV antes ou depois do 10Âș dia de doença. CONCLUSĂES: O diagnĂłstico de DK pode ser atrasado pela dificuldade em diferenciĂĄ-lo de outras doenças febris da infĂąncia
Automatic Quantification of Interstitial Lung Disease From Chest Computed Tomography in Systemic Sclerosis
Background: Interstitial lung disease (ILD) is a common complication in patients with systemic sclerosis (SSc), and its diagnosis contributes to early treatment decisions. Purposes: To quantify ILD associated with SSc (SSc-ILD) from chest CT images using an automatic quantification method based on the computation of the weight of interstitial lung opacities. Methods: Ninety-four patients with SSc underwent CT, forced vital capacity (FVC), and carbon monoxide diffusion capacity (DLCO) tests. Seventy-three healthy individuals without radiological evidence of lung disease served as controls. After lung and airway segmentation, the ratio between the weight of interstitial opacities [densities between -500 and +50 Hounsfield units (HU)] and the total lung weight (densities between -1,000 and +50 HU) was used as an ILD indicator (ILD[%] = 100 Ă [LW(-500 to +50HU)/LW(-1, 000 to +50HU)]). The cutoff of normality between controls and SSc was determined with a receiver operator characteristic curve. The severity of pulmonary involvement in SSc patients was also assessed by calculating Z scores of ILD relative to the average interstitial opacities in controls. Accordingly, SSc-ILD was classified as SSc Limited-ILD (Z score < 3) and SSc Extensive-ILD (Z score â„ 3 or FVC < 70%). Results: Seventy-eight (83%) SSc patients were classified as presenting SSc-ILD (optimal ILD threshold of 23.4%, 0.83 sensitivity, 0.92 specificity, and 0.94 area under the receiver operator characteristic curve, 95% CI from 0.89 to 0.96, 0.93 positive predictive value, and 0.81 negative predictive value, p < 0.001) and exhibited radiological attenuations compatible with interstitial pneumonia dispersed in the lung parenchyma. Thirty-six (38%) patients were classified as SSc Extensive-ILD (ILD threshold â„ 29.6% equivalent to a Z score â„ 3) and 42 (45%) as SSc Limited-ILD. Eighteen (50%) patients with SSc Extensive-ILD presented FVC < 70%, being only five patients classified exclusively based on FVC. SSc Extensive-ILD also presented lower DLCO (57.9 ± 17.9% vs. 73.7 ± 19.8%; p < 0.001) and total lung volume (2,916 ± 674 vs. 4,286 ± 1,136, p < 0.001) compared with SSc Limited-ILD. Conclusion: The proposed method seems to provide an alternative to identify and quantify the extension of ILD in patients with SSc, mitigating the subjectivity of semiquantitative analyzes based on visual scores.This research was supported by the Brazilian Council for Scientific and Technological Development (Conselho Nacional de Desenvolvimento CientĂfico e TecnolĂłgico-CNPq) and the Rio de Janeiro State Research Supporting Foundation (Fundação de Amparo Ă Pesquisa do Estado do Rio de Janeiro-FAPERJ)info:eu-repo/semantics/publishedVersio
Brazilian multicentre study of Takayasuâs arteritis in children and adolescents â preliminary results of a clinical, imaging and therapeutic study
DEVELOPMENT AND INITIAL VALIDATION OF THE SYSTEMIC JADAS, A NEW COMPOSITE DISEASE ACTIVITY SCORE FOR SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
Background: Juvenile Arthritis Disease Activity Score (JADAS) has gained increasing popularity for the measurement of the level of disease activity in patients with juvenile idiopathic arthritis (JIA). However, so far the JADAS has been validated only in children with the non-systemic categories of JIA.
Objectives: To develop and validate the systemic JADAS (sJADAS), a new version of the JADAS specific to systemic JIA (sJIA).
Methods: The sJADAS is made up by adding a fifth item, named Systemic Manifestation Score (SMS), to the four items included in the original tool (physician global assessment of disease activity, parent/patient global assessment of well-being, active joint count and erythrocyte sedimentation rate). The SMS ranges from 0 to 10 and is aimed to quantify the activity of systemic features. The sJADAS score ranges from 0 to 50. The validation sample included patients with definite and possible/probable sJIA with active systemic manifestations, which should comprise fever, who were assessed at baseline and then at a subsequent visit, 2 weeks to 3 months after initial evaluation. Validation procedures included assessment of concurrent, construct and discriminant validity, internal consistency and responsiveness to clinical change.
Results: A total of 161 patients, 86.7% (n=137) with definite sJIA and 13.3% (n=21) with possible/probable sJIA, assessed at disease onset
(n=90; 56.6%) or at time of a disease flare (n=69; 43.4%), were enrolled at 57 centers in 10 countries from February 2017 to December 2018.
Median age at disease onset was 5.0 years (interquartile range, IQR 2.8 - 8) and median age at study entry was 6.9 years (IQR 3.8â10.8).
Median disease duration from onset to study entry was 0.2 years (IQR 0.1â1.9). The median sJADAS at baseline visit was 28.2 (IQR 22.8â
35.0). The sJADAS correlated strongly with JADAS10 (rs=0.98) and clinical JADAS10 (cJADAS10) (rs=0.91); moderately with functional ability
scales (JAFS and CHAQ) (rs=0.69; rs=0.62) and total score (rs=0.57) and physical (rs=0.60) and psychosocial (rs=0.40) subscale scores of the
health-related quality of life tool (PRQL) and with pain VAS (rs=0.57); and mildly with the CRP (rs=0.42). The sJADAS discriminated well
between patients with or without morning stiffness (p<0.0001), with different levels of disease activity defined subjectively by the physician
(p<0.0001) and with different degrees of pain (p<0.0001). The internal consistency was good (Cronbachâs alpha=0.64) and comparable to that of JADAS10 (Cronbachâs alpha=0.59,) and cJADAS10 (Cronbachâs alpha=0.63). Responsiveness to change, measured on all patients
(SRM=2.23) and on patients classified as improved at second visit (SRM=2.41), was strong and superior to that of JADAS10 (SRM=1.92
and 2.14, respectively).
Conclusion: The sJADAS was found to be a valid instrument for the assessment of disease activity in sJIA. This composite score is feasible
and easily applicable in standard clinical practice, which should result in its widespread acceptance and use. The strong responsiveness to clinical change over time indicates that the sJADAS is suitable to assess therapeutic response in sJIA clinical trials