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Mitochondrial diseases are a phenotype and genotype heterogeneous group of disorders that typically have a multisystemic involvement. The m.3243A>G pathogenic variant is the most frequent mitochondrial DNA defect, and it causes several different clinical syndromes, such as mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS), and the maternally inherited diabetes and deafness (MIDD) syndromes.Not frequently reported, renal involvement in these diseases is probably underestimated, yet it increases morbidity. It generally manifests as subnephrotic proteinuria and progressive deterioration of kidney function. Adult presentation of mitochondrial diseases is hard to recognize, especially in oligosymptomatic patients or those with exclusive kidney involvement. However, suspicion should always arise when family history, particularly on the maternal side, and multisystemic symptoms, most often of the central nervous system and skeletal muscles, are present.In this review we discuss the clinical diagnosis and approach of patients with renal manifestations in the context of the mtDNA m.3243A>G pathogenic variant. Resumen: Las enfermedades mitocondriales son un grupo heterogéneo de trastornos fenotípicos y genotípicos que típicamente tienen una afectación multisistémica. La variante patógena m.3243A>G es el defecto del ADN mitocondrial más frecuente y causa varios síndromes clínicos diferentes, como la síndrome MELAS (mitochondrial encephalopathy, lactic acidosis and stroke-like episodes), y la síndrome MIDD (maternally inherited diabetes and deafness).Aunque no se informa con frecuencia, la afectación renal en estas enfermedades probablemente se subestima, pero aumenta la morbilidad. Generalmente se manifiesta como proteinuria subnefrótica y deterioro progresivo de la función renal. La presentación en adultos de las enfermedades mitocondriales es difícil de reconocer, especialmente en pacientes oligosintomáticos o con afectación renal exclusiva. Sin embargo, la sospecha siempre debe surgir cuando hay antecedentes familiares, particularmente del lado materno, y síntomas multisistémicos, con mayor frecuencia del sistema nervioso central y los músculos esqueléticos.En esta revisión discutimos el diagnóstico clínico y el abordaje de pacientes con manifestaciones renales en el contexto de la variante patogénica mtDNA m.3243A>G.

For patients with end stage renal disease, kidney transplant offers significant survival and quality-of-life advantages compared with dialysis. But for patients seeking transplant who are highly sensitized, waiting times have traditionally been long and options limited. We present the case of a 34-year-old hypersensitized female who underwent renal retransplantation. Histocompatibility tests revealed a calculated panel-reactive antibody of 99.53% with multiple antibodies against class I and II human leucocyte antigens and an eplet analysis was performed. The donor’s potential unacceptable antigens were re-defined and the calculated panel-reactive antibody decreased to 88.38%. After one month the patient received a deceased-donor kidney transplant. Complement dependent cytotoxicity crossmatch was negative; virtual crossmatch and flow cytometry crossmatch with historical serum were positive. High-dose intravenous immunoglobulin and rituximab were added to the thymoglobulin-based induction immunosuppression. Three donor-specific antibodies were detected and plasmapheresis was performed. Renal allograft biopsy revealed no manifestations of rejection. Repeated testing observed a decrease in donor-specific antibodies median fluorescence intensity values. Four months post-transplant, the patient remained with normal graft function without proteinuria. She is receiving a standard maintenance immunosuppression regime with prednisolone, mycophenolate mofetil and tacrolimus. The careful discussion among the transplantation center and histocompatibility laboratory in association with intense immunosuppression and close laboratory monitoring allowed a successful human leukocyte antigen-incompatible deceased donor kidney transplantation in the most critical phase for the occurrence of humoral rejection. It is noteworthy that the new histocompatibility and immunogenetics methodologies provide a more affirmative and comprehensive assessment of mismatch acceptability

Idiopathic pulmonary hemosiderosis is an uncommon disease of childhood, manifesting with the triad of recurrent hemoptysis, diffuse parenchymal infiltrates on chest radiographs and iron deficiency anemia. Celiac disease is an enteropathy characterized by life-long intolerance to ingested gluten in genetically susceptible people. In 1971 Lane and Hamilton first described the association of idiopathic pulmonary hemosiderosis with celiac disease and since then a few isolated cases have been reported in literature. We report a child with pulmonary hemosiderosis presenting with hemoptysis, severe anemia, and diffuse alveolar infiltrates in whom we made a diagnosis of celiac disease. In our case, severe anemia and alveolar infiltrates markedly improved with gluten-free diet.

Neste trabalho objetivou-se relatar o uso de permanganato de potássio e azul de metileno (cloreto de metiltionina) no tratamento de lesão cutânea da pirarara exposta para visitação pública. Pirarara, nome comum para espécie Phractocephalus hemioliopterus, é originária da bacia amazônica e bastante valorizada na aqüicultura pelo fácil manejo, adaptação ao cativeiro e pela bela fisionomia e coloração, porém muito susceptível à infestação por ectoparasitas acarretando em mortalidades tanto no aspecto produtivo como no mercado pet. A adesão de anelídeos na pele desta espécie alocada no aquário público da cidade de Uberlândia, Minas Gerais, Brasil, levou à lesão cutânea grave e infecção secundária por fungos e bactérias necessitando de tratamento medicamentoso. O diagnóstico foi feito através de achados macroscópicos e das características fisiológicas e comportamentais do animal e o tratamento durou trinta e quatro dias. Após identificação da lesão, os ectoparasitas foram retirados manualmente e o peixe foi conduzido ao tanque escavado isolando-o de outros animais e sem acesso aos visitantes. Adotou-se, respectivamente, o tratamento tópico de 10 gramas e 2 gramas por metro cúbico de permanganato de potássio e azul de metileno até o desaparecimento total da ferida e cura do animal. Com recuperação clínica, o peixe retornou ao aquário exposto à visitação.

The thoracic fraction of road dust (PM10) was measured for the first time in Portugal in parking areas, both outdoors and indoors, with the aim of completing existing studies carried out in active lanes of various roads. An in situ resuspension chamber was used to collect a total of 23 samples in three parking areas of Aveiro, whilst the laboratory procedures included determination of carbonaceous content (OC and EC) by a thermo-optical technique, elemental composition by ICP-MS and ICP-OES after acid digestion, and the Aliivribrio fisherii bioluminescent bacteria ecotoxicity bioassay. Dust loadings (DL10) obtained were 18.5 ± 9.8 mg PM10 m−2, in outdoor parking, and 1.8–23.7 mg PM10 m−2 for indoor parking, corresponding to emission factors of 476 and 75–589 mg veh−1 km−1, respectively. OC represented 9–30 % of PM10 for the indoor parking areas. However, for the outdoor samples, the high iron oxide content jeopardised the OC-EC separation. In those samples, carbonates accounted for 10.0 ± 3.3% of the PM10 mass. The analysis of elemental components focused on major elements (Al, Ca, Fe, K, and Mg) as well as minor elements. The total mass fraction of element oxides accounted for 27.1% (outdoor) and 23.6–34.3% (indoor). ΣPAH calculated for all parking areas accounted for 8.38–36.9 μg g−1 PM10. The ecotoxicological bioassay showed that all aqueous solutions were toxic to bioluminescent bacteria, whereas no clear correlations could be made with specific component groups, with the exception of ΣPAH and EC50.

The reign of D. Manuel II (1908-1910) was characterized by the intense activity of political propaganda, with the aim of making known the new king and simultaneously stabilizing the kingdom following the Regicide of February 1, 1908. To this monarchical campaign, widely sponsored by Queen D. Amelia, the Portuguese Republican Party corresponded to an evaluation of the royal figure, centred on fundamental concepts such as youth and the inexperience of the king. The interview of Vila Viçosa, held in February 1909, opened new perspectives of approach and criticism to the Portuguese Republican Party through the two royal figures of the Iberian Peninsula. The objective of this work is the analysis of republican political discourse on Alfonso XIII and Manuel II between 1908 and 1910.

The presence of anhydrosugars and sugar alcohols in airborne articulate matter 10) samples collected between December 2018 and June 2019 was studied for two urban environments in Coimbra. Anhydrosugars were used to estimate the biomass burning contribution, and sugar alcohols were investigated regarding biological sources. Anhydrosugars contributed more than sugar alcohols to the total sugars, mainly levoglucosan. Higher levoglucosan concentrations were linked with the use of biomass-fueled heating appliances, mainly during cold periods. A significant contribution from biomass burning smoke was registered, accounting for 20% to 23% of the PM10 mass in the colder period. Xylitol presented higher concentrations in the colder period and was well correlated with levoglucosan, indicating a common origin. Mannitol and arabitol were well correlated with each other but did not present any kind of correlation with anhydrosugars or xylitol, suggesting a natural source. A quantitative estimation based on the concentration of ambient tracers (mannitol) was evaluated, and the results reveal that, for the two sites, the fungal spore relative contribution to PM10 (roadside site: 2.7% to 2.8%; urban background: 1.9% to 2.7%) and OC mass (roadside site: 6.2% to 8.1%; urban background: 3.9% to 7.5%) was significant and always higher in the warmer period.

Pollutants resulting from domestic combustion would have harmful effects on public health, causing disturbances in the atmosphere chemistry and the climate. In this work, the emissions from the combustion of pellets made of 80% poplar (organically fertilized) and 20% cereal straw (Triticum aestivum) have been analysed. Poplar wood from I-214 and AF-8 clones was obtained from fertilized and non-fertilized plots (CONTROL). Dairy wastewater treatment (MUD) and dehydrated sewage sludge (BIOSOLIDS) were the fertilizers employed. BIOSOLIDS-I214 pellets had lower quality standards, so its combustion was omitted. A flue gas sample was sampled together with the particles emitted. The I-214 MUD sample had the highest emitted CO value (1505 mg/MJ). Regarding HCl emission factors, there was a homogeneity in the results linked with the Cl content present in the raw material. A higher particle emission was associated with pellets with higher ash content. Particles were composed mainly for elemental carbon (26–80 mg/MJ), except I-214 MUD. I-214 pellets had the indicators of incomplete combustion and, therefore, major contaminants emission and major environmental impact. Thus, both the poplar clone and the organic fertilizer influenced the parameters and emissions analysed. Therefore, special attention must be paid to both characteristics.

ABSTRACT: Cysticercosis represents an important public health problem that can cause significant economic losses to the beef industry. The present study aimed to determine the prevalence rate of cysticercosis in cattle and estimate the economic losses to producers associated with this parasitic disease. Data were collected from the official sanitary inspection service of a slaughterhouse located in the city of Uberlândia, Minas Gerais, southeast Brazil, from 2009 to 2016. A total of 358,383 cattle from 46 cities in Minas Gerais, Brazil, were slaughtered, of which 4,243 were infected with cysticercosis (1.18%). A total of 5,194 cysticerci were detected in these carcasses, of which 4,548 (87.56%) were alive and 646 (12.44%) were calcified. Most of the cysticerci were located in the masseter and pterygoid masticatory muscles (72.41%). As to the classification of cysticercosis according to the degree of infection of organs and/or parts affected, mild infection was the most frequently observed (92.36%). Rural producers had a total of R$ 1,755,204.20 (US$ 537,526.80) of economic losses due to bovine cysticercosis during the time span of eight years covered by this retrospective study. The importance of the sanitary inspection of meat in the control of this major zoonotic diseases is emphasized.

RESUMO Este artigo analisa a diplomacia comercial portuguesa face à Espanha, no decurso da segunda metade do séc. XIX. Pretende, especialmente, acompanhar as várias negociações comerciais desenvolvidas entre os países ibéricos, nas décadas de 1860, 70, 80 e 90, as quais culminaram na celebração do tratado de comércio e navegação de 1893. Procura, igualmente, avaliar as tendências políticas nacionais sobre os aspectos positivos e negativos associados ao fortalecimento das ligações econômicas com a Espanha. Neste exercício, é possível identificar um papel renovado da Espanha na geopolítica portuguesa, como nas perspectivas de crescimento econômico subsequentes ao Ultimatum (1890) e à denúncia dos tratados de comércio e navegação celebrados com a Inglaterra (1842/1878).

As inovações tecnológicas nas indústrias farmacêuticas e da medicina permitiram o prolongamento da vida sexual da população de idosos. Esse fator associado ao rompimento de tabus que envolvem a sexualidade na terceira idade tornam os idosos mais vulneráveis às infecções sexualmente transmissíveis (IST's). Um dos fatores que contribuem para a multiplicação dos casos é a falta de informação. O objetivo do estudo é analisar a vulnerabilidade às infecções sexualmente transmissíveis/Aids em idosos que frequentaram um espaço cultural de dança. Trata-se de uma pesquisa do tipo descritiva, com abordagem quantitativa. O local do estudo é um espaço cultural de dança localizado no município de Teresina-PI. A amostra é composta por 40 idosos, de ambos os sexos, que frequentavam o espaço cultural. Foi preenchido um questionário denominado ficha de coleta de dados que foi dividido em três partes. Com o presente estudo, percebeu-se nitidamente que a maioria dos idosos exercem atividade sexual com regularidade e possuem conhecimento da importância do uso do preservativo, porém acabam negligenciando sua utilização devido a sua condição de idoso. Com relação à atividade de dança, verificou-se que os idosos reconhecem a importância da mesma na melhoria da saúde e de sua qualidade de vida. Portanto, observa-se que a sexualidade deve ser debatida com os idosos e estimulada dentro de uma prática saudável e sem estigmas.

A denominada "crise de trabalho", resultante do conjunto complexo de transformações económicas e comerciais em curso nos finais do séc. XIX, constituiu um aspecto estruturante da realidade portuguesa desde os anos 80. Esta circunstância, uma determinante na conjuntura nacional, influenciou largamente a propaganda do Partido Republicano Português. Uma vez implantado o regime republicano, a construção do novo estado, discutido no contexto da Assembleia Nacional Constituinte, confrontava-se com as características de uma realidade social complexa, tanto no espaço urbano como rural, dominada pela "crise de trabalho", pelas reivindicações laborais acompanhadas pelo surto grevista, cujas consequências políticas e económicas conflituavam com a imagem de tranquilidade interna desejada no contexto, difícil, do reconhecimento externo do novo regime.

In order to evaluate the microbiological quality of the milk used in the production of “Minas” “Minas Frescal” cheese produced under Municipal Inspection in Uberlândia – MG Brasil, were collected between August and October 2013, eight samples of raw milk from two factories dairy products ("A" and "B") for carrying out microbiological analysis. At the same time there was a survey in the file of the Municipal Inspection Service - SIM from August 2012 to October 2013 to verify the results of microbiological analysis of samples of milk and cheese collected by the SIM in order to assess the quality and safety the production process of “Minas” cheese, totaling 14 samples of pasteurized milk and 17 “Minas” cheese. Microbiological analyzes consisted of total bacterial count, total and fecal coliforms, Salmonella sp. and coagulase positive Staphylococcus count, using as parameter Normative Instruction number 62 of December 29th, 2011 of the Ministério da Agricultura, Pecuária e Abastecimento (MAPA) for milk and the Resolution number 12 of January 2nd, 2001 permitted by the current legislation (Agência Nacional de Vigilância Sanitária- ANVISA) to cheese. The dairies "A" and "B" was 100% and 50% of raw milk samples satisfying respectively. Regarding the research done in the SIM file, all the samples of pasteurized milk analyzed were within the established legal standards. The analyzed cheese samples for the presence of fecal coliform, two (12%) were at odds with the standards established by ANVISA and, as the research Staphylococcus coagulase positive and Salmonella sp. 100% of the samples were within the required legal standards.

OBJECTIVE: To assess pulmonary function and the prevalence of atopy in school-age children who were very low birth weight as infants and to compare those who had bronchopulmonary dysplasia to those who did not. METHOD: We studied 85 (39 male and 46 female) at a mean age of 84 (range, 62 to 107) months who were very low birth weight infants. Bronchopulmonary dysplasia was defined as oxygen dependency at 36 weeks gestational age. We excluded 8 patients (4 for cerebral palsy and 4 for no collaboration). Detailed perinatal and clinical data were collected. Lung function was evaluated using conventional spirometry. Atopy (assessed by the allergy skin-prick test) was considered when at least one positive skin test occurred in a panel of the most common environmental allergens in the local region. Comparisons between the bronchopulmonary dysplasia and no bronchopulmonary dysplasia groups were performed using the Mann-Whitney, x2 and Fisher's exact tests. RESULTS: We compared the bronchopulmonary dysplasia (n = 13) and no bronchopulmonary dysplasia (n = 64) groups. Atopy was observed in 4 (30.8%) of the bronchopulmonary dysplasia patients and in 17 (26.6%) of the no bronchopulmonary dysplasia patients (p = 0.742). Two (15.4%) patients with bronchopulmonary dysplasia had a family history of atopy vs. 17 (26.6%) in the no bronchopulmonary dysplasia group (p = 0.5). Lung function tests showed airway obstruction in 2 (15.4%) of the bronchopulmonary dysplasia patients and in 10 (15.6%) of the no bronchopulmonary dysplasia patients (p = 1.0). Four (33.3%) of the bronchopulmonary dysplasia patients had small airway obstruction vs. 14 (22.2%) of the no bronchopulmonary dysplasia patients (p = 0.466). CONCLUSION: Our data showed no significant differences in lung function between bronchopulmonary dysplasia and no bronchopulmonary dysplasia patients at school age and no evidence of an association between atopy and bronchopulmonary dysplasia.

INTRODUCTION: Considering that alternative antigens for diagnosing neurocysticercosis continue to be a challenge because of the increasing difficulty in obtaining parasites from naturally infected pigs for preparation of Taenia solium homologous antigen, the aim of the present study was to evaluate the detergent (D) and aqueous (A) fractions from saline extract of Taenia saginata metacestodes for diagnosing neurocysticercosis. METHODS: Taenia saginata was obtained from naturally infected bovines in the Triângulo Mineiro region, State of Minas Gerais, Brazil. The carcasses came from cold storage units and had been slaughtered in accordance with the inspection technique recommended by the Federal Inspection Service. The D and A fractions were obtained by using Triton X-114 (TX-114). Serum samples were obtained from 40 patients with a diagnosis of neurocysticercosis, 45 with other parasitic diseases and 30 from apparently normal individuals. IgG antibody levels were evaluated using the ELISA and immunoblotting assays. RESULTS: The ELISA sensitivity and specificity were 95% and 73.3%, when using saline extract; 95% and 82.6% for the D fraction; and 65% and 61.3% for the A fraction, respectively. The immunoblotting assay confirmed the ELISA results, such that the D fraction was more efficient than the other extracts, and the 70-68kDa component was immunodominant among neurocysticercosis patients. CONCLUSIONS: These results demonstrated that the D fraction from Taenia saginata metacestodes obtained using TX-114 can be used as a heterologous antigenic fraction in the immunoblotting assay for serologically diagnosing human neurocysticercosis, given its ability to select immunodominant antigens.INTRODUÇÃO: Considerando que antígenos alternativos para o diagnóstico da neurocisticercose (NC) continua sendo um desafio devido ao aumento da dificuldade em se obter parasitas de suínos naturalmente infectados, para a preparação do antígeno homólogo de Taenia solium, o objetivo do presente estudo foi avaliar frações detergente (D) e aquosa (A), do extrato salino de metacestódeo de Taenia saginata para diagnóstico da NC. MÉTODOS: Bovinos, naturalmente infectados com Taenia saginata, procedentes da região do Triângulo Mineiro, Estado de Minas Gerais, Brasil, foram obtidos de frigoríficos e abatidos de acordo com a técnica de inspeção recomendada pelo Serviço de Inspeção Federal. As frações D e A foram obtidas utilizando Triton X-114 (TX-114). Amostras de soro foram obtidas de 40 pacientes com diagnóstico de NC, 45 com diagnóstico de outras doenças parasitárias e 30 de indivíduos aparentemente normais. Níveis de IgG foram avaliados pelos testes ELISA e Imunoblotting. RESULTADOS: A sensibilidade e especificidade do teste ELISA foram 95% e 73,3%, quando utilizado o extrato salino, 95% e 82,6% para fração D, e 65% e 61,3% para a fração A, respectivamente. O ensaio Imunoblotting confirmou os resultados do teste ELISA, sendo a fração D mais eficiente que os outros extratos, observando-se que o componente 70-68kDa se comportou como imunodominante para os pacientes com NC. CONCLUSÕES: Estes resultados demonstraram que a fração D de metacestódeo de Taenia saginata obtida com TX-114 pode ser utilizada como fração antigênica heteróloga pelo Imunoblotting para o diagnóstico sorológico da NC humana, considerando sua habilidade para selecionar antígenos imunodominantes.

Nos últimos anos tem sido descrita, no mundo ocidental, uma redução da incidência da pneumonia aguda da comunidade (PAC) nas crianças, parodoxalmente associada a maior gravidade dos casos internados. O presente estudo pretendeu caracterizar os casos de PAC internados e compará-los com um estudo anterior a 2001. Recolhemos dados referentes a 63 internamentos, durante seis meses. Houve predomínio do grupo dos 0-2 anos. Na radiografia torácica, em 58 casos (92,1%) detectou-se condensação/atelectasia e em 17 (27,0%) derrame pleural (DP). Onze dos DP corresponderam a empiema (17,4% dos internamentos). Isolou-se agente bacteriano em cinco casos: Streptococcus pyogenes (dois, líquido pleural), Streptococcus pneumoniae (dois, hemocultura) e Haemophilus influenzae (um, hemocultura). Foi prescrita antibioticoterapia em 61 (96,8%) crianças. A duração mediana de internamento foi de cinco dias. Constatou-se, nos casos de DP, idade superior, maior duração de febre, proteína C reactiva mais alta e maior duração de internamento e de antibioticoterapia endovenosa. Comparativamente com o estudo anterior, constatou-se haver maior gravidade dos casos internados, traduzida por maior incidência de DP e empiema; paradoxalmente, registou-se menor duração da febre em meio hospitalar e menor duração do internamento. No estudo actual houve menor prescrição antibiótica prévia à admissão e maior prescrição de ampicilina durante o internamento. Na literatura, a maior gravidade das pneumonias internadas nos últimos anos tem sido associada à emergência de serotipos mais agressivos de Stretococcus pneumoniae não integrados na vacina heptavalente, assumindo interesse crescente a introdução de vacinas que os incluam. As pneumonias complicadas devem ser orientadas para centros de referência com experiência no seu diagnóstico e tratamento.The past few years have seen a decline in community acquired pneumonia (CAP) in children in the western world, although this has gone hand-in-hand with more serious cases needing hospital admission. Our study characterises cases of CAP admitted to hospital and compares this data with a 2001 study. We collected data on 63 admissions over a six-month period. The majority were aged 0-2 years old. Chest X-ray showed consolidation/atelectasy in 58 (92.1%) and pleural effusion (PE) in 17 (27.0%), of which 11 were empyema (17.4% of all admissions). The bacterial agent was isolated in five cases: Streptococcus pyogenes (two, pleural fluid), Streptococcus pneumoniae (two, blood culture) and Haemophilus influenzae (one, blood culture). Sixty-one children (96.8%) were prescribed antibiotherapy. The median length of hospital stay was five days. Patients with PE were older, had a longer course of fever, higher inflammatory parameters, longer hospital stay and longer course of iv antibiotics. Compared to the prior study we found greater severity of CAP, with higher prevalence of PE and empyema. Nevertheless there was a shorter course of fever during hospital stay and shorter hospital stay. We also noticed less antibiotic prescription prior to admission and greater prescription of ampicillin during hospital stay. In the literature, the higher severity of CAP has been partially attributed to the emergence of more aggressive serotypes of Stretococcus pneumoniae not included in the heptavalent vaccine. There is therefore a greater interest in new vaccines containing them. Complicated CAP should be referred to centres specialising in its diagnosis and management.

Simultaneous measurements of outdoor and indoor pollution were performed at three schools in Lisbon. Volatile organic compounds (VOCs), formaldehyde and NO2 were passively monitored over a two-week period. Bacterial and fungal colony-forming units and comfort parameters were also monitored at classrooms and playgrounds. The highest indoor levels of CO2 (2666 μg/m³), NO2 (40.3 μg/m³), VOCs (10.3 μg/m³), formaldehyde (1.03 μg/m³) and bioaerosols (1634 CFU/m³), and some indoor/outdoor ratios greater than unity, suggest that indoor sources and building conditions might have negative effects on air indoors. Increasing ventilation rates and use of low-emission materials would contribute towards improving indoor air quality.

Resumo: Introdução: Os derrames pleurais podem complicar as pneumonias na população pediátrica. Assumem especial importância pelas implicações na duração do internamento, geralmente prolongado, e pela morbilidade associada.Objectivos: Caracterizar uma população de doentes com pneumonia complicada de derrame; determinar os possíveis factores de prognóstico a partir de dados clínicos, radiológicos e analíticos na admissão; e avaliar a influência das intervenções terapêuticas na evolução da doença.Métodos: Revisão casuística dos doentes com idade inferior a 18 anos e derrame pleural parapneu mónico, internados de Julho de 1997 a Junho de 2004 (7 anos).Resultados: Foram estudados 118 casos, 60% do sexo masculino, com idade média 7 anos. A incidência de derrame pleural aumentou ao longo do período do estudo. Verificou-se maior incidência de casos no Outono e no Inverno. Na admissão, 60% dos doentes apresentavam sinais de dificuldade respiratória e 39% dor torácica. Em 40% dos doentes foram detectados septos pleurais na admissão, o que se associou a maior duração de internamento e de antibioticoterapia e mais frequente necessidade de cirurgia. Em 72% dos doentes foi efectuada toracocentese (pH médio: 7,24). Em 17% foi possível isolar o agente: Streptococcus pneumoniae (cinco), Staphylococcus aureus (quatro) e Streptococcus pyogenes (quatro). Foram submetidos a drenagem pleural 52% e necessitaram de cirurgia 18%. A mediana da duração de internamento foi de 15 dias e a média de 16,4 dias (2 a 51).Discussão: Associam-se a pior prognóstico a presença de: sinais de dificuldade respiratória; septos; empiema; baixo valor no líquido pleural de pH, glicose ou proteínas; desidrogenase láctica elevada no líquido pleural e proteína C-reactiva sanguínea aumentada. A drenagem pleural e/ou cirurgia mais precoces provavelmente diminuem o tempo de doença e de internamento.Conclusão: Os derrames parapneumónicos complicados são tratados com êxito em centros de referência com experiência nos diferentes tipos de intervenção que poderão ser necessários.Rev Port Pneumol 2009; XV (2): 241-259 Abstract: Introduction: Pleural effusions can complicate pneumonias in children and adolescents and are usually associated with a long hospital stay and increased morbidity.Aims: To characterise a population of patients with parapneumonic pleural effusion and to establish possible prognostic factors on admission based on clinical, imaging and analytical data. To correlate treatment options with the outcome.Methods: Case review of patients under 18 years old with parapneumonic pleural effusion, admitted between July 1997 â June 2004 (7 years).Results: 118 patients were included, 60% male, with mean age 7 years. The incidence of pleural effusion increased throughout the period of the study. The admissions occurred predominantly in autumn and winter. On admission 60% of patients had respiratory distress and 39% chest pain. In 40% loculations were found on admission and were associated with longer hospital stay, longer course of antibiotic therapy and more frequent need for surgery. Thoracentesis was performed in 72% of patients (mean pH pleural fluid 7.24). The aetiologic agent was identified in 17% of cases: Streptococcus pneumoniae (five), Staphylococcus aureus (four) and Streptococcus pyogenes (four). In our study, 52% of patients underwent pleural drainage and 18% surgery. Median length of hospital stay was 15 days with mean 16.4 days (2 â 51).Discussion: Factors associated with worse prognosis were respiratory distress, loculations, empyema, low pH in pleural fluid, glucose or proteins in pleural fluid, high lactic dehydrogenase level in pleural fluid and high serum C-reactive protein. Pleural drainage and/or surgery can shorten hospital stay and improve outcome.Conclusion: Complicated parapneumonic pleural effusions are managed successfully in centres with experience in the different types of procedure that might be necessary.Rev Port Pneumol 2009; XV (2): 241-259 Palavras-chave: Pneumonia, derrame pleural, drenagem, cirurgia, prognóstico, Key-words: Drainage, pleural effusion, pneumonia, prognosis, surgery

Apesar dos avanços nas técnicas de ventilação não invasiva, a traqueostomia continua a ter indicação em crianças com perturbação grave da via aérea ou com necessidade de ventilação prolongada. Sendo um procedimento de execução fácil, não é isenta de riscos, pelo que se justifica sempre a ponderação prévia de alternativas à decisão de traqueostomizar. Foram objectivos deste estudo a identificação das crianças traqueostomizadas com seguimento num serviço de pediatria de hospital terciário, a caracterização da doença primária e indicações conducentes à decisão de traqueostomizar e a avaliação das complicações registada neste grupo de doentes. Procedeu -se à revisão dos processos clínicos das dezasseis crianças traqueostomizadas, com uma mediana de idade à data da realização da traqueostomia de 4,5 meses, cinco com doença neuromuscular, três com paralisia cerebral e sete com doença pulmonar ou das vias aéreas. A indicação para a traqueostomia foi a necessidade de ventilação invasiva prolongada em doze casos, estenose subglótica ou traqueal em três e laringomalacia num outro. As complicações mais frequentes foram a colonização bacteriana persistente, a descanulação transitória acidental e a obstrução transitória da cânula. Verificou-se um óbito relacionado com a traqueostomia. Nove crianças mantêm traqueostomia, cinco foram descanuladas (uma das quais veio a falecer) e outras duas faleceram ainda com traqueostomia. Tal como sublinhado na literatura, as crianças e adolescentes traqueostomizados têm habitualmente patologias múltiplas que exigem apoio multidisciplinar. A complexidade das situações clínicas e a exigência de recursos determinam a necessidade de acompanhamento em centro especializado de referência.Despite advances in non-invasive ventilation techniques, tracheostomy is still indicated in children with serious airway obstruction or with the need for long-term ventilation. Alternatives should be studied before deciding to tracheostomise as complications exist, despite the simple procedure. The aims of this study were the identification of tracheostomised children followed in a tertiary care paediatric department, the characterisation of primary disease, the indications leading to tracheostomy and any complications. We present a case review of tracheostomised children followed in our department. Sixteen tracheostomised children were followed, median age of 4.5 months, neuromuscular disease - 5, cerebral palsy - 3 and pulmonary or airway disease - 7. Indications were long-term invasive ventilation - 12, subglottic or tracheal stenosis - 3 and laryngomalacia - 1. The most frequent complications found were persistent bacterial colonisation, accidental decannulation and obstruction. There was a tracheostomy-related death in 1 child. Outcome: 9 children maintained tracheostomy, 5 were successfully decannulated and 2 children died while on tracheostomy. Tracheostomised children usually have complex disease that requires a multidisciplinary team and should be followed-up at a specialised reference centre.

Resumo: Na criança imunocompetente, a bronquiolite obliterante (BO) é uma doença rara que geralmente ocorre após uma infecção vírica das vias aéreas inferiores. O diagnóstico de BO era, tradicionalmente, confirmado por biópsia pulmonar. Contudo, a identificação de lesão pulmonar prévia, aliada à evolução clínica típica, radio-grafia e tomografia computorizada sugestivas, substi-tuíram a necessidade de procedimentos mais invasivos.Os autores fizeram uma revisão dos processos clínicos das crianças, com o diagnóstico de BO (n=10) em seguimento numa consulta externa de pneumo-logia pediátrica, entre Janeiro de 1997 e Dezembro de 2002, com o objectivo de determinar etiologia, apresentação clínica, alterações imagiológicas, tratamento e evolução mais frequentes.Todas as crianças com o diagnóstico de BO apresen-tavam tosse e/ou pieira persistentes após o episódio agudo inicial. Na altura, 80% das crianças tinham má evolução ponderal. A idade média de diagnóstico foi de 16 meses. O evento inicial foi uma pneumonia e, em 9 casos, identificou-se o agente causal (cinco adenovírus, três vírus sincicial respiratório, um parain-fluenzae e um desconhecido). Na nossa casuística, ne-nhuma das crianças efectuou biópsia pulmonar, por a apresentação clínica e radiológica ser típica de BO.O seguimento, de 36 meses em média, revelou resolução clínica em 3 crianças e sintomas persistentes em 6. Uma criança morreu por falência respiratória progressiva.O reconhecimento rápido do diagnóstico e o tratamento de suporte, que inclui oxigenioterapia e plano nutricional agressivo, contribuíram para melhorar o estado clínico destas crianças.Rev Port Pneumol 2007; XIII (4): 495-509 Abstract: Bronchiolitis obliterans (BO) is a rare disease in immunocompetent children that usually occurs after infection of the lower airways. While a diagnosis of BO was usually confirmed by lung biopsy, identification of prior lung lesion plus a typical clinical course and a suggestive chest X-ray and CT scan have replaced the need for more invasive procedures.The authors reviewed the clinical records of 10 BO patients, followed in the Outpatients Paediatric Pulmonology Unit from January 1997 to December 2002, to identify the most common aetio-logy, clinical and radiological profiles, treatment and course.All patients maintained cough and/or wheezing after the initial acute episode. 80% had failure to thrive at the time of the diagnosis, mean age 16 months. Viral pneumonia was the main initial event (5 adenovirus, 3 respiratory syncytial virus, 1 parainfluenza virus, 1 unknown). Lung biopsies were not performed as clinical and radiological presentations were typical of BO. The follow-up (mean 36 months) revealed clinical resolution in 3 children and persistent symptoms in 6. One patient had progressive respiratory failure and died.Prompt recognition of the diagnosis with supportive treatment that included oxygen therapy and an aggressive nutrition plan helped to improve the clinical state of the children.Rev Port Pneumol 2007; XIII (4): 495-509 Palavras-chave: Bronquiolite obliterante, adenovírus, etiologia, diagnóstico, evolução, criança, Key-words: Bronchiolitis obliterans, adenovirus, aetiology, diagnosis, evolution, children

A abordagem do derrame pleural parapneumónico complicado, em idade pediátrica, permanece controversa. As opções terapêuticas incluem antibioticoterapia e drenagem pleural contínua, instilação intrapleural de fibrinolíticos, videotoracoscopia e toracotomia com descorticação. O objectivo deste estudo foi rever, avaliar e actualizar a abordagem ao derrame pleural complicado. Procedeu-se à revisão retrospectiva dos processos clínicos das crianças internadas na UPP por derrame pleural complicado entre 1992 e 2003. Foram incluídos 25 doentes, com idade média (±DP): 37,4 (± 37,0) meses, sendo 15/25 (60%) do sexo masculino. A identificação do agente foi possível em 17/25 (68%) casos [S. Aureus 6/17 (35%), St. pneumoniae 5/17 (29%)], no líquido pleural em 16/17 (94%) casos. Todos os doentes realizaram toracocentese e antibioticoterapia sistémica. A drenagem pleural contínua foi instituída em 22/25 (88%) casos com duração média (±DP): 14,2 (± 7,8) dias; em 1 caso houve instilação de fibrinolíticos intrapleurais e em 11/25 (44%) realizou-se toracotomia com descorticação. Um doente foi submetido a videotoracoscopia primária. A duração média de internamento (±DP) foi de 30,4 (± 15,1) dias e não ocorreram óbitos. A experiência do centro é determinante na abordagem escolhida e na rapidez de actuação. Provavelmente ambas influenciam o prognóstico imediato.Pediatric management of complicated pleural effusion (CPE) remains controversial. Different approaches include antibiotics and chest tube drainage alone or the use of fibrinolitics, videothorascoscopy (VTC) and surgical decortication through thoracotomy. The aim of the present study was to review, evaluate and update technical approach to CPE. We retrospectively reviewed the clinical files of children admitted to the Pediatric Respiratory Ward between 1992 and 2003 with the diagnosis of CPE. Twenty-five patients were included [15 male (60%)]. Mean (±SD) age was 37,4 (±37,0) months. Bacteria were identified in 17/25 (68%) [S. aureus in 6/17 (35%), St. pneumoniae in 5/17 (29%)], 16/17 (94%)in the pleural fluid. Twenty-five children were treated with antibiotics and thoracocentesis (100%). Chest tube drainage was required in 22/25 (88%) with mean (±DP) duration of 14,2 (±7,8) days. Fibrinolitics were employed in 1 only case and surgical decortication in 11/25 (44%). One patient (4%) was submitted to primary VTC. Median length of stay was 30,4 (±15,1) days and no deaths were recorded. Center skills in CPE management are critical on the choice of the technique and the timing of approach. This seems to influence immediate prognosis

Resumo: A abordagem do derrame pleural parapneumónico complicado, em idade pediátrica, permanece controversa. As opções terapêuticas incluem antibioticoterapia e drenagem pleural contínua, instilação intrapleural de fibrinolíticos, videotoracoscopia e toracotomia com descorticação. O objectivo deste estudo foi rever, avaliar e actualizar a abordagem ao derrame pleural complicado. Procedeu-se à revisão retrospectiva dos processos clínicos das crianças internadas na UPP por derrame pleural complicado entre 1992 e 2003. Foram incluídos 25 doentes, com idade média (±DP): 37,4 (± 37,0) meses, sendo 15/25 (60%) do sexo masculino. A identificação do agente foi possível em 17/25 (68%) casos [S. aureus6/17 (35%), St. pneumoniae 5/17 (29%)], no líquido pleural em 16/17 (94%) casos. Todos os doentes realizaram toracocentese e antibioticoterapia sistémica. A drenagem pleural contínua foi instituída em 22/25 (88%) casos com duração média (±DP): 14,2 (± 7,8) dias; em 1 caso houve instilação de fibrinolíticos intrapleurais e em 11/25 (44%) realizou-se toracotomia com descorticação. Um doente foi submetido a videotoracoscopia primária. A duração média de internamento (±DP) foi de 30,4 (± 15,1) dias e não ocorreram óbitos. A experiência do centro é determinante na abordagem escolhida e na rapidez de actuação. Provavelmente ambas influenciam o prognóstico imediato.Rev Port Pneumol 2007; XIII (1): 53-70 Abstract: Pediatric management of complicated pleural effusion (CPE) remains controversial. Different approaches include antibiotics and chest tube drainage alone or the use of fibrinolitics, videothorascoscopy (VTC) and surgical decortication through thoracotomy.The aim of the present study was to review, evaluate and update technical approach to CPE. We retrospectively reviewed the clinical files of children admitted to the Pediatric Respiratory Ward between 1992 and 2003 with the diagnosis of CPE. Twenty-five patients were included [15 male (60%)]. Mean (±SD) age was 37,4 (±37,0) months. Bacteria were identified in 17/25 (68%) [S. aureus in 6/17 (35%), St. pneumoniae in 5/17 (29%)], 16/17 (94%)in the pleural fluid. Twenty-five children were treated with antibiotics and thoracocentesis (100%). Chest tube drainage was required in 22/25 (88%) with mean (±DP) duration of 14,2 (±7,8) days. Fibrinolitics were employed in 1 only case and surgical decortication in 11/25 (44%). One patient (4%) was submitted to primary VTC. Median length of stay was 30,4 (±15,1) days and no deaths were recorded. Center skills in CPE management are critical on the choice of the technique and the timing of approach. This seems to influence immediate prognosis.Rev Port Pneumol 2007; XIII (1): 53-70 Palavras-chave: Derrame pleural, empiema pleural, criança, Key-words: Pleural effusion, pleural empyema, child

RESUMO: Os autores descrevem o caso de uma adolescente de 15 anos com internamentos sucessivos por quilotórax bilateral desde os primeiros meses de vida. A clínica e as imagens da tomografia axial computorizada estabeleceram o diagnóstico de linfangiectasia pulmonar. A maioria dos casos de linfangiectasia pulmonar, descritos na literatura, apresenta muito mau prognóstico, sendo geralmente letal no período neonatal. Neste caso, apesar dos múltiplos internamentos hospitalares, tem-se verificado razoável qualidade de vida entre as exacerbações. Para além desta manifestação clínica, encontramos linfedema congénito, alterações faciais (face inexpressiva, edemas palpebrais, boca pequena, alterações dentárias) e atraso ligeiro de desenvolvimento. Esta constelação de manifestações permite-nos encará-lo como o primeiro caso português de síndroma de Hennekam. O recurso a estudos moleculares poderá ajudar a explicar muitas das manifestações clínicas encontradas nestes doentes e a englobar outros casos na mesma entidade clínica.REV PORT PNEUMOL 2004; X (3): 253-258 ABSTRACT: The authors describe the clinical case of a 15 year-old adolescent with recurrent bilateral chylothorax leading to multiple hospitalizations. As in other cases described the diagnosis was made on the basis of classical clinical features and characteristic CT scan. Most cases of pulmonary lymphangiectasis, described in the literature, are lethal in the neonatal period. In this case the girl has a reasonable life quality, in spite of multiple hospitalizations. Lower limb lymphedema, facial anomalies (flat face, palpebral edema, tooth anomalies and small mouth) and mild development retardation allow us to consider our case as the first portuguese case of Hennekam Syndrome. Molecular studies will allow, in the future to explain most of the clinical manifestations and to include other patients in the same clinical entity.REV PORT PNEUMOL 2004; X (3): 253-258 Palavras-chave: quilotórax, linfangiectasia pulmonar, síndroma de Hennekam, Key-words: chylothorax, pulmonary lymphangiectasis, Hennekam syndrome

RESUMO: O abcesso pulmonar é uma doença rara na infância, mas condiciona elevada morbilidade, obrigando a internamento hospitalar prolongado. Surge geralmente em crianças com factores de risco ou co-morbilidades associadas e responde bem à antibioterapia, raramente sendo necessário o recurso a técnicas de diagnóstico ou terapêutica invasivas.Os autores apresentam dois casos de abcesso pulmonar de grandes dimensões, em crianças, uma delas com risco de aspiração associado, que resolveram completamente com tratamento antibiótico, e fazem uma revisão sobre esta entidade clínica na infância.REV POR PNEUMOL 2003; IX (2): 161-172 ABSTRACT: Lung abscess is a rare entity in childhood, but leads to high morbidity and long length of hospitalisation. It appears commonly in children with risk factors or other disease, have a good response to antibiotic therapy and it is rarely necessary the use of invasive techniques.Authors present two cases of lung abscess in children, one of them with risk factors to aspiration, that improved with antibiotic therapy and make a revision about this entity in infancy.REV POR PNEUMOL 2003; IX (2): 161-172 Palavras-chave: Abcesso pulmonar, Criança, Key-words: Lung abscess, Child

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement.

RESUMO: Introdução: As pneumonias necrosantes são uma complicação rara da pneumonia lobar, sobretudo na era pós-antibiótica. Recentemente tem-se assistido no nosso hospital ao acréscimo da incidência destas complicações não sendo claro o motivo que lhe está subjacente.Material e Métodos: Estudo retrospectivo dos casos de pneumonias necrosantes em crianças com menos de 13 anos de idade e previamente saudáveis, ocorridos num período de 2 anos num hospital terciário. Carac-terização clínica, imagiológica e microbiológica e da evolução no internamento e após alta.Resultados: Entre as 392 crianças internadas por pneumonia no período do estudo, 15 crianças (3,4â2,4 anos; 9 rapazes) sem doença prévia conhecida apresentavam evidência de necrose pulmonar na TAC pulmonar. Apesar da antibioticoterapia, a febre persistiu 13â7 dias. Dez doentes desenvolveram pneumatocelos e 3 abcessos pulmonares. Doze doentes apresentavam derrames pleurais associados, tendo nove deles necessitado de drenagem torácica. Foi isolado o agente infectante em 3 doentes (2 S. aureus e 1 Enterobacter cloacae).Conclusões: Os resultados encontrados não permitem esclarecer o aumento recente das pneumonias necrosantes. A baixa taxa de isolamento do agente infeccioso implicado poderá estar relacionada com o frequente uso prévio de antibióticos. As imunodeficiências primárias deverão ser sempre pesquisadas nas pneumonias graves mesmo em crianças previamente saudáveis. Apesar da evolução clínica arrastada e da frequente necessidade de toracotomia, o resultado final foi bom na maioria dos doentes.REV PORT PNEUMOL 2002; VIII (1): ABSTRACT: Background: Necrotizing pneumonia used to be rare in the postantibiotic era. Its incidence appears to be rising in our hospital although there is no clear explanation for that.Patients and Methods: Retrospective analysis of the clinical presentation and evolution of necrotizing pneumonia in previously healthy children admitted at our hospital during two years.Results: Among 392 admissions for pneumonia in the study period, 15 patients (3,4â2, 4 years; 9 boys) had evidence of lung necrosis on thoracic computed tomography. Despite appropriate antibiotic therapy, fever persisted for 13â7 days. Ten patients deve loped pneumatoceles and three lung abscesses. Twelve patients had parapneumonic pleural effusions (4 empyemas), nine of whom need closed chest drainage. An agent was isolated from pleural fluid or blood culture in 3 children (2 S. aureus and 1 Enterobacter cloacae); in two further, bacteria were seen on Gram-stained pleural fluid. Two patients had an immunodefficiency state previously unknown (IgA deficiency and hiper-IgE syndrome). Thoracotomy were performed in 9 patients: 2 pleural debridement, 6 decortication, 1 ressection of a giant pneumatocele, 1 abscess drainage and 2 lobectomies. The mean length of hospital stay was 30â11 days and there were no deaths. During the follow-up period (18â9 months) 3 patients required surgery for recurrent infection or fibrothorax. In the remaining, asymptomatic children, 2 have pleural ticknening, 1 a residual pneumatocele and 1 bronchiectasis.Conclusions: The results of this study do not explain the recent increase of the suppurative complications of community acquired-pneumonia in children. The low rate of infectious agent isolation was probably related with frequent previous antibiotic use. Although the final outcome was generally good, the clinical course was very prolonged and surgery was frequently needed. In order to clarify the reason for the increase of this kind of pneumonia complications a prospective multicenter study is warranted.REV PORT PNEUMOL 2002; VIII (1): Palavras-chave: pneumonia adquirida na comunidade, necrose pulmonar, pneumatocelos, abcesso pulmonar, Key-words: community acquired pneumonia, lung necrosis, pneumatoceles, lung abscess

Introdução: Oxigenoterapia domiciliária de longa duração está indicada em doentes com hipoxémia crónica. Pretendemos descrever a população em programa de oxigenoterapia domiciliária de longa duração acompanhada numa Unidade de Pneumologia Pediátrica de Hospital Terciário entre 2003-2012 e comparar com revisão de 1991-2000; verificar conformidade com orientações nacionais e internacionais, refletindo sobre necessidade de orientações nacionais especificamente pediátricas, inexistentes em Portugal. Material e Métodos: Estudo retrospetivo, descritivo e comparativo por consulta de processo clínico. Pesquisa de orientações sobre oxigenoterapia em idade pediátrica. Resultados: Incluímos 86 doentes (59,3% rapazes). A idade mediana de início da oxigenoterapia foi 0,0 (0,0-216,0) meses e a duração mediana de 15,0 (3,0-223,0) meses. O diagnóstico mais frequente foi displasia broncopulmonar (53,5%), seguindo-se bronquiolite obliterante (14,0%), doença neurológica (10,5%), fibrose quística (8,1%), síndromes polimalformativas (5,8%), doença de células falciformes (3,5%), outras doenças pulmonares neonatais (2,3%) e doenças pulmonares intersticiais (2,3%). Mantêm acompanhamento 53 (61,6%) doentes, 38 mantendo oxigenoterapia; 12 (13,9%) faleceram. O tempo mediano de seguimento foi 39,5 (1,0-246,0) meses, mínimo nas outras doenças pulmonares neonatais e máximo na fibrose quística. Comparativamente ao estudo anterior revela aumento relativo dos lactentes com bronquiolite obliterante e displasia broncopulmonar, aumento da duração nestes últimos e inclusão de doentes neurológicos e hematológicos. Discussão: A prescrição de oxigenoterapia domiciliária de longa duração em pediatria ocorre sobretudo em doenças específicas dos lactentes e idade pré-escolar. Doentes neurológicos e hematológicos são novos grupos de prescrição, à semelhança da literatura internacional. Conclusão: O conhecimento da realidade nacional e orientações pediátricas são relevantes para organização de cuidados e prescrição racional. Palavras-chave: Criança; Cuidados de Longa Duração;Insuficiência Respiratória; Oxigenoterapia.