Relationship between salt-inducible kinase 2 (SIK2) and lymph node metastasis in colorectal cancer patients complicated with chronic schistosomiasis / 浙江大学学报·医学版

To investigate the relationship between salt-inducible kinase 2 (SIK2) and lymph node metastasis in colorectal cancer patients complicated with chronic schistosomiasis. Tissue specimens were collected from 363 patients who were diagnosed as colorectal cancer by clinical and pathological examination in Wuhu Second People's Hospital from June 2015 to June 2020. Fifty-six patients were colorectal cancer complicated with schistosomiasis (CRC-S) and 307 patients were colorectal cancer not complicated with schistosomiasis (CRC-NS). The clinical and pathological data of the patients were analyzed to explore the relationship between chronic schistosomiasis and colorectal cancer. Immunohistochemistry and Western blotting were used to detect the distribution and expression of SIK2 in colorectal cancer specimens. The relationship between SIK2 and lymph node metastasis of CRC-S was analyzed. The rate of lymph node metastasis in CRC-S group was significantly higher than that in CRC-NS group (62.5% vs. 47.2%, <0.05). In CRC-S patients with lymph node metastasis, schistosome eggs were distributed mainly in tumor tissues (25/35, 71.4%), while in patients with CRC-S without lymph node metastasis, schistosome eggs were distributed mainly in paracancerous tissues (17/21, 81.0%) (14.243, <0.01). The SIK2 was mainly located in cytosol, and its expression in tumor tissues was higher than that in paracancerous tissues. Compared with CRC-NS patients, the expression of SIK2 in CRC-S patients was significantly increased; the expression of SIK2 in patients with lymph node metastasis was higher than that in patients without lymph node metastasis; and the expression of SIK2 in patients with schistosome eggs in cancer tissues was higher than that in patients with schistosome eggs in paracancerous tissues (all <0.01). Lymph node metastasis is more likely to be occurred in colorectal cancer patients with schistosomiasis, especially in those with schistosome eggs in tumor tissues. The expression of SIK2 may be correlated with chronic schistosomiasis, egg distribution and lymphatic metastasis.

Demographic and clinical characteristics of pulmonary arterial hypertension caused by schistosomiasis are indistinguishable from other etiologies

Abstract INTRODUCTION: Pulmonary arterial hypertension (PAH) is a serious pulmonary circulation disease caused by several etiologies, including schistosomiasis. The present study retrospectively evaluated the clinical and hemodynamic characteristics of patients with schistosomal PAH (PAH-Sch) compared to those of non-Sch PAH patients (non-Sch PAH). METHODS: Patients treated at the Pronto-Socorro Cardiológico de Pernambuco and diagnosed by right cardiac catheterization were divided into PAH-Sch and non-Sch PAH groups. Their socio-demographic and clinical characteristics, N-terminal-pro B-type natriuretic peptide (NT-proBNP), and echocardiography and hemodynamic parameters were retrospectively reviewed. RESULTS: Among the included 98 patients (mean age, 45 ± 14 years; 68 women [69.4%]), we found 56 PAH-Sch and 42 non-Sch PAH. The age distribution was heterogeneous in the PAH-Sch group, with patients predominantly ranging from 50-59 (p <0.004). Dyspnea was the most common symptom, reported by 92 patients (93.8%), and commonly present for over two years prior to diagnosis. Clinical symptoms were similar in both groups, with no differences in functional class, pulmonary artery systolic pressure (p = 0.102), 6-minute walk test score (p = 0.234), NT-proBNP serum levels (p = 0.081), or hemodynamic parameters. CONCLUSIONS: Patients with PAH-Sch present clinical, laboratory, and hemodynamic profiles similar to those with PAH resulting from other etiologies of poor prognosis. PAH is an important manifestation of schistosomiasis in endemic regions that is often diagnosed late.

Obstrução extrínseca do tronco da coronária esquerda causada por dilatação da artéria pulmonar associada à esquistossomíase / Extrinsic obstruction of the left main coronary artery due to pulmonary artery dilation associated with schistosomiasis

Relatamos o caso de um paciente portador de hipertensão pulmonar de origem esquistossomótica, que procurou pronto atendimento por apresentar quadro de dor torácica em repouso. A apresentação clínica e os demais dados referentes ao caso levantaram a suspeita de insuficiência coronariana aguda, e foi diagnosticada uma obstrução grave do tronco da coronária esquerda. O relato do caso teve por objetivo destacar a necessidade do diagnóstico diferencial da queixa de dor torácica nestes pacientes e ressaltar a opção da intervenção coronariana percutânea como tratamento eficaz e seguro neste cenário.

This report describes a patient with pulmonary hypertension secondary to schistosomiasis, who sought emergency care due to chest pain at rest. The clinical presentation and other information related to the case raised the suspicion of acute coronary failure, and a severe obstruction was identified in the left main coronary artery. The case report aimed to highlight the need for a differential diagnosis of chest pain complaints in these patients, and emphasizes the choice of percutaneous coronary intervention as an effective and safe treatment in this scenario.

Management of variceal hemorrhage: current concepts / Tratamento da hemorragia digestiva alta por varizes esofágicas: conceitos atuais

INTRODUCTION: The treatment of portal hypertension is complex and the the best strategy depends on the underlying disease (cirrhosis vs. schistosomiasis), patient's clinical condition and time on it is performed (during an acute episode of variceal bleeding or electively, as pre-primary, primary or secondary prophylaxis). With the advent of new pharmacological options and technical development of endoscopy and interventional radiology treatment of portal hypertension has changed in recent decades. AIM: To review the strategies employed in elective and emergency treatment of variceal bleeding in cirrhotic and schistosomotic patients. METHODS: Survey of publications in PubMed, Embase, Lilacs, SciELO and Cochrane databases through June 2013, using the headings: portal hypertension, esophageal and gastric varices, variceal bleeding, liver cirrhosis, schistosomiasis mansoni, surgical treatment, pharmacological treatment, secondary prophylaxis, primary prophylaxis, pre-primary prophylaxis. CONCLUSION: Pre-primary prophylaxis doesn't have specific treatment strategies; the best recommendation is treatment of the underlying disease. Primary prophylaxis should be performed in cirrhotic patients with beta-blockers or endoscopic variceal ligation. There is controversy regarding the effectiveness of primary prophylaxis in patients with schistosomiasis; when indicated, it is done with beta-blockers or endoscopic therapy in high-risk varices. Treatment of acute variceal bleeding is systematized in the literature, combination of vasoconstrictor drugs and endoscopic therapy, provided significant decline in mortality over the last decades. TIPS and surgical treatment are options as rescue therapy. Secondary prophylaxis plays a fundamental role in the reduction of recurrent bleeding, the best option in cirrhotic patients is the combination of pharmacological therapy with beta-blockers and endoscopic band ligation. TIPS or surgical treatment, ...

INTRODUÇÃO: O tratamento da hipertensão portal é complexo e a definição da melhor estratégia depende da causa subjacente (cirrose vs. Esquistossomose), da condição clínica e do momento em que é realizado (episódio agudo de hemorragia ou como profilaxia pré-primária, primária ou secundária). Com o advento de novas opções medicamentosas e o desenvolvimento da endoscopia e radiologia intervencionista, o tratamento da hipertensão portal tem sofrido grande transformação nas últimas décadas. OBJETIVO: Avaliar os avanços e as estratégias empregadas no tratamento emergencial e eletivo da hemorragia varicosa em pacientes cirróticos e esquistossomóticos. MÉTODO: Revisão bibliográfica nas bases de dados PubMed, Embase, Lilacs, SciELO e Cochrane até junho de 2013, com os descritores: portal hypertension, esophageal and gastric varices, variceal bleeding, liver cirrhosis, schistosomiasis mansoni, surgical treatment, pharmacological treatment, secondary prophylaxis, primary prophylaxis, pré-primary prophylaxis. CONCLUSÃO: Com relação à profilaxia pré-primária não existem estratégias específicas; a melhor recomendação é tratamento da doença de base. A proflaxia primária em pacientes cirróticos deve ser feita com betabloqueadores ou terapêutica endoscópica com ligadura elástica. Existe controvérsia quanto à efetividade da profilaxia primária em pacientes esquistossomóticos; quando indicada, faz-se com betabloqueadores ou terapêutica endoscópica nas varizes de maior risco. O tratamento do sangramento agudo é o com melhor sistematização e mais alto nível de evidência; a associação de drogas vasoconstritoras e terapia endoscópica ...

Renal autotransplantation to treat renal artery aneurysm: case report / Autotransplante renal para tratamento de aneurisma de artéria renal: relato de caso

CONTEXT: Renal artery aneurysm (RAA) is uncommon and usually asymptomatic, but complications like rupture or thromboembolism of the aneurysm can occur, with consequent renal infarction. Most of the clinical findings are found incidentally through imaging examinations, in investigating other diseases. Renal autotransplantation (RAT) is an alternative treatment for complex RAA, with satisfactory results described in the literature. CASE REPORT: The patient was a 48-year-old man with a history of systemic arterial hypertension, thrombocytopenia and advanced hepatosplenic schistosomiasis. He complained of right lumbar pain, which was investigated through imaging examinations (computed tomography and angiotomography). These revealed right RAA of 2.5 cm in diameter. Evaluation by the vascular surgery team found that this was untreatable using endovascular methods. The treatment performed was open right nephrectomy with kidney preservation in solution, followed by aneurysmectomy, suturing of the injured artery and kidney reimplantation in the right iliac fossa with anastomosis of the iliac vessels and ureter. The durations of the surgery and kidney ischemia were 385 and 140 minutes, respectively. The patient was discharged on the 20th postoperative day, with creatinine concentration of 1.4 mg/dL, urea 41 mg/dL, urine volume 1400 mL/24 h and ascites treated with diuretics. CONCLUSION: RAT is indicated basically in three situations: extracorporeal reconstruction of complex aneurysms of the renal pedicle, extensive ureteral injury, and conservative kidney cancer surgery in patients with a single kidney. This study presents a case of a patient with advanced liver disease and RAA that was untreatable using endovascular methods and was successfully treated using RAT. .

CONTEXTO: O aneurisma de artéria renal (AAR) é incomum e, em geral, assintomático, mas podem ocorrer complicações como rotura ou embolia de trombos do aneurisma com consequente infarto renal. A maioria dos achados clínicos é encontrada acidentalmente por exames de imagem na investigação de outras doenças. O autotransplante renal (ATR) constitui-se em alternativa de tratamento de AAR com resultados satisfatórios descritos na literatura. RELATO DE CASO: Paciente masculino, 48 anos, com histórico de hipertensão arterial sistêmica, plaquetopenia e esquistossomose hepatoesplênica avançada. Referia dor lombar direita que após exames de imagem (tomografia computadorizada e angiotomografia) revelou AAR direita com 2,5 cm de diâmetro não tratável por via endovascular após avaliação da equipe de cirurgia vascular. O tratamento realizado foi uma nefrectomia aberta direita com preservação renal em solução, seguida de aneurismectomia, sutura da artéria lesada e reimplante do rim na fossa ilíaca direita com anastomoses dos vasos ilíacos e do ureter. O tempo cirúrgico e de isquemia renal foram de 385 e 140 minutos, respectivamente. Recebeu alta hospitalar no vigésimo dia do pós-operatório, com concentrações de creatinina de 1,4 mg/dL, ureia de 41 mg/dL, volume urinário de 1400 mL/24 h e ascite tratada com diuréticos. CONCLUSÃO: O ATR está indicado basicamente em três casos: reconstrução extracorpórea de aneurismas complexos do pedículo renal, lesão ureteral extensa e cirurgia conservadora de câncer renal em pacientes com único rim. Este estudo apresenta caso de paciente com doença hepática avançada e AAR intratável por método endovascular e tratado com sucesso por ATR. .

Urothelial carcinoma of the urinary bladder mixed with squamous differentiation or squamous cell carcinoma in areas with schistosomiasis is showing high risk of recurrence and poor survival

In schistosomiasis haematobium areas endemic, bladder cancer is the first cause of malignancy in men and fourth in women. The chronic schistosomiasis would lead to variant histologic patterns which manifest in squamous cell carcinoma [SCC] or squamous differentiation [SqD]. This study evaluated the clinical outcome after radical cystectomy [RC] in patients with urothe-lial carcinoma [UC] mixed with SCC or SqD, Comparison was done with two arms of pure UC and pure SCC, indication for RC was muscle-invasive-disease, and evaluation included recurrence, metastases, and overall survival. The data of patients treated with RC for muscle-invasive-disease, selection was revised for 127 patients with urothelial carcinoma mixed with SCC/SqD, two comparative arms were 100 patients with pure UC, and 100 patients had pure SCC. Follow up was on 8 months, Syears, and 5 years to detect recurrence, metastasis, and overall survival in the three groupsThe results showed that by comparison of disease aggressiveness in the three groups regarding recurrence, metastasis, and overall survival was analysed. Overall survival with mixed tumours was significantly lower than pure UC or SCC, recurrence and metastases were higher in mixed tumour which was an independent factor for poor prognosis and low survival

Relação da pressão sistólica em artéria pulmonar com gênero, menopausa e gravidez em portadores de hipertensão arterial pulmonar associada à esquistossomose / Pulmonary artery pressure, gender, menopause, and pregnancy in schistosomiasis-associated pulmonary hypertension

FUNDAMENTO: A hipertensão arterial pulmonar associada à esquistossomose (HPAE) é uma grande preocupação no mundo todo. No entanto, o papel de fatores contribuintes específicos do gênero em HPAE é desconhecido. OBJETIVO: Investigamos os valores da pressão arterial pulmonar sistólica (PAPS) e a presença de elevação grave na PAPS relacionado ao gênero, presença de menopausa e histórico de gravidez em pacientes com HPAE. MÉTODOS: Setenta e nove pacientes diagnosticados com HPAE de 2000 a 2009 foram avaliados e 66 foram incluídos no estudo. As informações referentes à idade, status da menopausa, gravidez, PAPS derivada da ecocardiografia, e pressão arterial pulmonar média invasiva (PAPm) foram coletadas de registros médicos. A relação entre os valores de PAPS e PAPm e a correlação para doença grave foram avaliados. Os modelos de regressão avaliaram a associação de gênero, status da menopausa e histórico de gravidez com valores de PAPS e a presença de PAPS severa. RESULTADOS: Houve correlação moderada entre PAPm e PAPS, com boa concordância para classificação de doença grave. Os valores de PAPS foram semelhantes para homens e mulheres. Uma tendência a valores maiores de PAPS foi encontrada para mulheres não menopausadas em comparação a homens. Valores superiores de PAPS foram encontrados para mulheres menopausadas em comparação a mulheres não menopausadas; os valores não foram significativos após o ajuste de idade. O histórico de gravidez não teve relação com a PAPS. Presença de menopausa e passado de gravidez não mostraram associação com valores de PAPS. CONCLUSÃO: Em pacientes com HPAE, nem o gênero, nem o status da menopausa nem o histórico de gravidez apresentou uma correlação independente com valores de HPAE avaliados pela ecocardiografia.

BACKGROUND: Schistosomiasis-associated pulmonary arterial hypertension (SPAH) is a major concern worldwide. However, the role of gender-specific contributing factors in SPAH is unknown. OBJECTIVE: We investigated how systolic pulmonary artery pressure (SPAP) values and the presence of severe SPAP relate to gender, menopausal status, and pregnancy history in SPAH patients. METHODS: Seventy-nine patients diagnosed with SPAH from 2000 to 2009 were assessed and 66 were enrolled in the study. Information about age, menopausal status, pregnancy, echocardiography-derived SPAP, and invasive mean pulmonary artery pressure (mPAP) was collected from medical records. The relation between values of SPAP and mPAP and their agreement for severe disease were assessed. Regression models assessed the association of gender, menopausal status, and pregnancy history with SPAP values and the presence of severe SPAP. RESULTS: Moderate correlation and good agreement for severe disease were found between mPAP and SPAP. Mean SPAP values were similar for men and women. A trend toward higher values of SPAP was found for non-menopausal women compared to men. Higher SPAP values were found for menopausal compared to non-menopausal women; the values were non-significant after adjustment for age. Pregnancy history had no association with SPAP. Menopause and positive pregnancy had no association with severe SPAP. CONCLUSION: In SPAH patients, neither gender, nor menopausal status, nor pregnancy history showed independent correlation with SPAP values assessed by echocardiography.

Epidemiologicalaaspects of hepatocellular carcioma in a referral center of minnas gerais, Brazil / Aspectos epidemiologicos do carcinoma hepatocelular em centro de referencia de Minas Gerais

Context Studies on epidemiology of hepatocellular carcinoma and modalities of therapy used to treat this condition are scarce in Brazil. Our aim was to characterize hepatocellular carcinoma according to etiology of the underlying chronic liver disease, and treatment modalities, in a referral center in Brazil. Methods All cases of hepatocellular carcinoma registered in the Department of Pathology during a 12-year period (1998-2010) were included. Demographic data, etiology of the underlying liver disease and treatment performed were collected. Results This case series included 215 patients, mean age 57.3 (± 14.1) years, 164 (76.2 %) male. Virus C and virus B infection were detected in 88 (43%) and 47 (23%) patients, respectively. Ethanol abuse alone or combined with other etiologies was identified in 64 (32%) individuals. Schistosomiasis was found in 18 (9%) patients. Liver transplantation was the treatment of choice in 112 (51%) patients. This procedure was more frequently performed in hepatitis C virus-related hepatocellular carcinoma (70%) than B virus-related hepatocellular carcinoma (17%). Tumor resection was performed in 40 (18%) individuals, ethanol injection or thermo ablation in 18 (14%), and chemoembolization in 14 (7%). In 40 (19.4%) patients no treatment was performed and this percentage remained constant over the years. Conclusions Chronic hepatitis C, followed by ethanol abuse and chronic hepatitis B were the leading causes of underlying chronic liver disease associated with hepatocellular carcinoma. The results show a trend of increasing incidence of hepatocellular carcinoma; however, the proportion of untreated patients remained constant over the analyzed period. .

Contexto Estudos sobre epidemiologia do carcinoma hepatocelular e terapias utilizadas em seu tratamento são escassos no Brasil. O objetivo foi caracterizar o carcinoma hepatocelular quanto à etiologia da hepatopatia crônica subjacente e modalidades terapêuticas empregadas, em um centro de referência no Brasil. Método Todos os casos de carcinoma hepatocelular registrados no Departamento de Patologia em período de 12 anos (1998-2010) foram incluídos. Dados demográficos, etiologia da hepatopatia crônica e tipo de tratamento realizado foram coletados dos prontuários. Resultado Esta série de casos incluiu 215 pacientes, com média de idade 57,3 (± 14,1) anos, 164 (76,2%) do sexo masculino. Infecções virais C e B foram detectadas em 88 (43%) e 47 (23%), respectivamente. Abuso de etanol isoladamente ou associado a outras causas foi identificado em 64 (32%) indivíduos. Esquistossomose foi encontrada em 18 (9%) indivíduos. Transplante hepático foi o tratamento de escolha em 112 (51%) pacientes. Esse procedimento foi mais frequentemente realizado em carcinoma hepatocelular relacionado com hepatite C (70%) do que hepatite B (17%). Ressecção cirúrgica foi realizada em 40 (18%) indivíduos, alcoolização ou termo-ablação em 18 (14%) e quimioembolização em 14 (7%). Em 40 (19,4%) pacientes nenhum tratamento foi empregado e esta porcentagem permaneceu constante ao longo dos anos do estudo. Conclusões Hepatite viral C seguida de abuso de etanol e hepatite viral B foram as causas de hepatopatia crônica mais frequentemente associadas ao carcinoma hepatocelular. Os resultados mostram tendência a aumento da incidência do carcinoma hepatocelular; no entanto, a proporção de pacientes não-tratados permaneceu constante ...

Glomerulopatia esquistossomótica: comportamentos das alterações em camundongos infectados pelo schistosoma mansoni, antes e depois do tratamento com praziquantel / Schistosomal glomerulopathy: behavior changes in mice infected with schistosoma mansoni before and after treatment with praziquantel

A esquistossomose é um grave problema de saúde pública causado pelo helminto Schistosoma mansoni, sendo uma das endemias parasitárias de maior prevalência no Brasil. Apresenta diversas formas clínicas passíveis de serem reproduzidas em modelos experimentais. A lesão renal da glomerulopatia esquistossomótica está associada ao antígeno parasitário que, em consequência da presença de hipertensão portal e formação da circulação colateral, permitem que complexos imunes sejam depositados em glomérulos renais, desencadeando um processo inflamatório progressivo. O objetivo do trabalho foi avaliar o comportamento das alterações renais em camundongos infectados pelo S. mansoni, antes e após o tratamento com praziquantel, a fim de observar a resposta à presença dos complexos imunes e ao processo inflamatório formado na glomerulopatia. Foram organizados 3 grupos de camundongos BALB/c de ambos os sexos: grupo controle intacto (10 animais), grupo de reinfectados tratados (35 animais) e grupo de reinfectados não tratados (35 animais). A avaliação da estrutura renal foi analisada através da microscopia óptica, observando a histologia glomerular com as colorações de HE, PAS e PIFG, através da imunofluorescência marcando IgG e antígeno de Nash, e da microscopia eletrônica de transmissão com emprego da morfometria para avaliar áreas de depósito de complexos imunes. Foram analisadas, também, a carga parasitária, o peso do fígado, a presença dos vermes nas veias mesentéricas e a estrutura física dos órgãos. Os resultados demonstraram significativa redução da carga parasitária, do peso do fígado, das alterações estruturais nas observações histológicas e dos depósitos de complexos imunes em área e marcação no tecido renal, após tratamento com praziquantel. O tratamento específico reverteu os processos iniciais de agressão renal na glomerulopatia do modelo experimental, embora em alguns poucos camundongos, após a quimioterapia, a doença se manteve. Os achados deste trabalho reforçam a possibilidade de que o estado normal de estrutura renal pode ser restabelecido com o tratamento antiparasitário, desde que ministrado logo que se instala a glomerulopatia esquistossomótica clínica.

Schistosomiasis is an important public health problem due to infection with the helminth Schistosoma mansoni, which is also one the parasitic diseases listed as highly prevalent in Brazil. It presents several clinical forms, which are susceptible of experimental reproduction in mice. The lesion involving the kidney – Schistosomal glomerulopathy – is related to the presence of circulating parasite antigens, which can be attached to circulating antibodies to form antigen-antibody complexes with glomerular deposition. However, in the presence of portal hypertension and collateral circulation, these antigen complexes, can bypass macrophage clearance and reach the systemic circulation, thus coming to be deposited within the renal glomeruli, starting a progressive inflammatory process. The aim of this research was to evaluate renal changes in mice infected with S. mansoni, before and after treatment with praziquantel, through the presence of immune complexes in the glomeruli, and its correlation with the glomerular changes shown. BALB/c mice infected, infected and treated mice and non-infected groups were evaluated. Besides the evaluation of the renal changes by optical microscopy (HE, PAS, PIFG), immunofluorescence (IgG and Nash antigen) and transmission electron microscopy, a morphometric study of the areas containing immune complexes was also attempted. Besides parasite burden, liver weigh, number of worm pairs within mesenteric veins, and organs physical structure, were also evaluated.

Perfil hemodinâmico de gravidade ao teste de vasorreatividade pulmonar em esquistossomóticos / Hemodynamic profile of severity at pulmonary vasoreactivity test in schistosomiasis patients

FUNDAMENTO: A taxa de vasorreatividade pulmonar da Hipertensão Arterial Pulmonar Esquistossomótica (HAPE) não é conhecida. Dados hemodinâmicos obtidos pelo cateterismo cardíaco associam-se aos critérios clínicos de gravidade mais utilizados. OBJETIVO: Estimar o percentual de vasorreatividade positiva ao óxido nítrico em hipertensão arterial pulmonar esquistossomótica; verificar nesses pacientes a associação de parâmetros hemodinâmicos com a classificação funcional da OMS e teste de caminhada de seis minutos. MÉTODOS: Foram selecionados 84 pacientes portadores de hipertensão pulmonar de etiologia esquistossomótica de um banco de dados, submetidos ao cateterismo cardíaco direito e esquerdo com realização do teste de vasorreatividade pulmonar com óxido nítrico. Foram coletados os dados da classificação funcional da OMS e do teste de caminhada de seis minutos para fim de comparação com os dados invasivos. RESULTADOS: Dos 84 pacientes portadores de HAPE, três (3,5%) apresentaram os critérios para vasorreatividade pulmonar positivo. O aumento da resistência vascular pulmonar esteve associado significativamente à menor capacidade de esforço aferida pelo teste de caminhada de seis minutos (p = 0,045) e maiores sintomas de gravidade por meio de maiores classificações funcionais da OMS (classes III/IV) (p = 0,013). A diminuição da saturação de oxigênio na artéria pulmonar esteve associada significativamente a maiores classificações funcionais (p = 0,041). CONCLUSÃO: A taxa de resposta pulmonar ao teste de vasodilatação dos pacientes esquistossomóticos encontra-se abaixo dos valores encontrados para hipertensão pulmonar de etiologia idiopática. A resistência vascular pulmonar e a saturação de oxigênio na artéria pulmonar são dados hemodinâmicos que podem ser utilizados como marcadores de gravidade na hipertensão pulmonar esquistossomótica.

BACKGROUND: The pulmonary vasoreactivity rate of Schistosomal Pulmonary Artery Hypertension (SPAH) is not known. Hemodynamic data obtained by cardiac catheterization are associated with the most commonly used clinical criteria of severity. OBJECTIVE: To estimate the percentage of positive vasoreactivity to nitric oxide in schistosomal pulmonary arterial hypertension and verify the association of hemodynamic parameters with WHO functional classification and the six-minute walk test in these patients. METHODS: A total of 84 patients with schistosomal pulmonary hypertension were selected from a database, who had been submitted to the right and left cardiac catheterization and pulmonary vasoreactivity test with nitric oxide. Data on WHO functional classification and six-minute walk test were collected for comparison with invasive data. RESULTS: Of the 84 patients with SPAH, 3 (3.5%) had positive criteria for pulmonary vasoreactivity. The increase in pulmonary vascular resistance was significantly associated with lower exercise capacity measured by the six-minute walk test (p = 0.045) and greater symptom severity by higher functional classifications (WHO class III/IV) (p = 0.013). The decrease in oxygen saturation in the pulmonary artery was significantly associated with higher functional classifications (p = 0.041). CONCLUSION: The pulmonary response rate to the vasodilation test of schistosomiasis patients is below the values found for idiopathic pulmonary hypertension. Pulmonary vascular resistance and oxygen saturation in the pulmonary artery are hemodynamic data that can be used as markers of severity in schistosomal pulmonary hypertension.

Association of MICA gene polymorphisms with liver fibrosis in schistosomiasis patients in the Dongting Lake region

Major histocompatibility complex class I chain-related A (MICA) is a highly polymorphic gene located within the MHC class I region of the human genome. Expressed as a cell surface glycoprotein, MICA modulates immune surveillance by binding to its cognate receptor on natural killer cells, NKG2D, and its genetic polymorphisms have been recently associated with susceptibility to some infectious diseases. We determined whether MICA polymorphisms were associated with the high rate of Schistosoma parasitic worm infection or severity of disease outcome in the Dongting Lake region of Hunan Province, China. Polymerase chain reaction-sequence specific priming (PCR-SSP) and sequencing-based typing (SBT) were applied for high-resolution allele typing of schistosomiasis cases (N = 103, age range = 36.2-80.5 years, 64 males and 39 females) and healthy controls (N = 141, age range = 28.6-73.3 years, 73 males and 68 females). Fourteen MICA alleles and five short-tandem repeat (STR) alleles were identified among the two populations. Three (MICA*012:01/02, MICA*017 and MICA*027) showed a higher frequency in healthy controls than in schistosomiasis patients, but the difference was not significantly correlated with susceptibility to S. japonicum infection (Pc > 0.05). In contrast, higher MICA*A5 allele frequency was significantly correlated with advanced liver fibrosis (Pc < 0.05). Furthermore, the distribution profile of MICA alleles in this Hunan Han population was significantly different from those published for Korean, Thai, American-Caucasian, and Afro-American populations (P < 0.01), but similar to other Han populations within China (P > 0.05). This study provides the initial evidence that MICA genetic polymorphisms may underlie the severity of liver fibrosis occurring in schistosomiasis patients from the Dongting Lake region.

Sleep-related breathing disorders in patients with Schistosomal cor-pulmonale

Schistosmiasis has long been an endemic disease in Egypt and an important cause of pulmonary hypertension. We aimed to investigate the clinical and polysomnographic features of sleep-related breathing disorders [SRBD] in patients with schistosomal cor-pulmonale and to evaluate their effects on pulmonary hemodynamics. We studied 10 stable patients diagnosed with schistosomal pulmonary hypertension [7 males and 3 females their mean age was 43.7 +/- 8.04] and 10 healthy volunteers matched for age, sex and BMI. Patients' exclusion criteria were: smoking, morbid obesity, other secondary causes of pulmonary hypertension, systemic hypertension, ischemic or rheumatic heart disease or left heart failure. All patients underwent overnight polysomnography or ambulatory cardiorespiratory sleep studies, spirometry, ECG and echocardiography. Daytime sleepiness was also assessed using the Epworth sleepiness scale [ESS]. The mean AHI in patients group was 20.0 +/- 11.34/h while in the control group it was 2.3 +/- 1.16/h. 80% of the patients were found to have an AHI > 10/h and 60% had moderate to severe sleep apnea [AHI >/= 15/h]. In addition, the majority of the patients [80%] spent > 30% of the night with an arterial oxygen saturation <90%. SRBD were not correlated with anthropometric measures, spirometry nor with the typical symptoms of SA such as excessive sleepiness as assessed by ESS. More importantly, SRBD were significantly associated with measures of pulmonary hypertension severity, and patients with moderate to severe SA had more impaired cardiovascular function as indicated by more severe right ventricular dilatation [p = 0.036] than patients with mild sleep apnea. SRBD are highly prevalent in patients with schistosomal pulmonary hypertension [PH]. Also, the SA severity was correlated with more advanced PH and more severe cardiovascular impairment. Therefore in the evaluation of patients with schistosomal PH, polysomnography or an ambulatory cardiorespiratory sleep study seems justified to identify potentially treatable SRBD that may additionally challenge the already compromised cardiovascular system in these patients

Tratamento da hipertensão pulmonar esquistossomótica / Treatment of schistosomiasis-associated pulmonary hypertension

A esquistossomose mansoni é a terceira doença parasitária endêmica mais prevalente do mundo. Estima-se que mais de 200 milhões de pessoas estejam infectadas com uma das espécies do parasita Schistosoma. Dessas, 270.000 pessoas (4,6 por cento) são portadoras de hipertensão arterial pulmonar, que é associada à forma hepatoesplênica da doença. Essa alta prevalência coloca a hipertensão pulmonar esquistossomótica como a causa mais frequente de hipertensão pulmonar no mundo. Entretanto, o tratamento dirigido especificamente ao acometimento vascular pulmonar não está ainda estabelecido. Relatamos o caso de uma paciente portadora dessa doença que foi tratada com um inibidor de fosfodiesterase-5 (sildenafil) com resultados satisfatórios.

Schistosomiasis mansoni is the third most prevalent endemic parasitic disease in the world. It is estimated that over 200 million people are infected with parasites belonging to one of the Schistosoma species. Of those, 270,000 people (4.6 percent) suffer from pulmonary arterial hypertension, which is associated with the hepatosplenic form of the disease. This high prevalence makes schistosomiasis-associated pulmonary hypertension the leading cause of pulmonary hypertension worldwide. However, no specific treatment for the pulmonary vascular component of the disease has yet been devised. We report the case of a patient with schistosomiasis-associated pulmonary hypertension who was treated satisfactorily with a phosphodiesterase-5 inhibitor (sildenafil).

Leucemia linfoblástica aguda diagnosticada na vigência de parto, simulando síndrome Hellp / Acute lymphoblastic leukemia diagnosed in delivery force, simulating Hellp syndrome

Este artigo relata a necessidade de realização de cesariana de emergência em paciente de 15 anos de idade, com 29 semanas de gestação, portadora de Leucemia linfoblástica aguda (LLA), pré-eclampsia e suspeita de síndrome HELLP. Possui a LLA baixa incidência durante a gravidez sendo, em geral, sua possibilidade diagnóstica pouco aventada. Neste relato, o diagnóstico de LLA não foi realizado de forma precoce no pré-natal, só tardiamente no pré-operatório da cesariana. A paciente evoluiu com instabilidade hemodinâmica, necessitando de terapia intensiva.

The ALL (acute lymphoblastic leukemia) is a disease with low incidence during pregnancy. This case report an emergency caesarean section in a patient of 15 years with 29 weeks of gestation, ALL, pré-eclâmpsia and suspect of HELLP Syndrome. It was difficult to diagnose ALL during prenatal and pre-operative period, which may be associated with low incidence of this disease during pregnancy. The patient developed hemodynamic instability, had to stay in the ICU and the ALL was diagnosed in the postoperative period. This study shows the differential diagnosis in ALL, the difficulties to the diagnosis, clinical reasoning and the therapeutic.

Tratamento com stent em tronco de artéria coronária esquerda por compressão do tronco da artéria pulmonar em paciente com hipertensão pulmonar esquistossomótica / Stent treatment for a left main coronary artery compression by a dilated pulmonary trunk in a patient with schistosomotic pulmonary hypertension

A hipertensão arterial pulmonar frequentemente está associada a dor torácica com características anginosas e sua etiologia é desnconhecida. A compressão extrínseca do tronco de artéria coronária esquerda pela artéria pulmonar é uma causa tratável e deve ser considerada. É apresentado o caso de uma paciente com hipertensão arterial pulmonar de etiologia esquistossomótica, com angina do peito decorrente de compressão do tronco de artéria coronária esquerda, que foi tratada com stent intracoronário. Os sinais e sintomas isquêmicos foram completamente resolvidos e a ultrassonografia intracoronária, realizada na evolução tardia, mostrou a consolidação do resultado angiográfico.

Pulmonary hypertension is frequently associated to chest pain with anginous characteristics and its etiology is uncertain. Extrinsic compression of the left main coronary artery by the pulmonary artery is a treatable cause and should be taken into consideration. We present the case of a patient with pulmonary hypertension of schistosomotic etiology presenting angina due to compression of the left main coronary artery, who was treated with an intracoronary stent. Signs and symptoms of ischemia were completely resolved and a late follow-up intravascular ultrasound confirmed the vessel patency.

Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor?

INTRODUCTION: Chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the United States. The incidence rate in Brazil, however, is unknown. The estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. Pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. In Brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. OBJECTIVES: To evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in Brazil. METHODS: From December 2006 to November 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. The subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. RESULTS: Thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. Two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). The majority of patients were categorized in functional classes III and IV. Hemodynamic data showed a mean pulmonary vascular resistance (PVR) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 L/min. Linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. Paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). Brain natriuretic peptide serum levels were elevated in 19 of 27 patients. CONCLUSIONS: In a referral center for pulmonary hypertension in Brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterectomy had a hemodynamically severe status and had elevated brain natriuretic peptide serum levels. There was a predominance of females in our cohort, and the prevalence of hematological disorders and schistosomiasis was low (less than 10 percent).

Esquistossomose: quebrando o ciclo / Schistosomiasis: breaking the Cycle

O combate à esquistossomose esbarra nas dificuldades geradas pelas condições sanitárias a que muitas pessoas estão expostas, especialmente na região nordeste, devido à desigualdade social que ainda persiste no Brasil. Gravado na região metropolitana de Recife e Cabo de Santo Agostinho/PE, os principais objetivos deste vídeo são: oferecer aos profissionais do Sistema Único de Saúde (SUS) uma visão ampliada sobre a doença; contribuir para o conhecimento do seu ciclo; e orientar quanto ao reconhecimento do agente etiológico, bem como os fatores que mantêm a transmissão da doença.

message from the past: splenectomy and devascularisation with salvage sclerotherapy in the treatment of bleeding schistosomal oesophageal varices

To study the short and long term results of splenectomy and esophagogastric devascularisation in the prevention of bleeding from esophageal varices secondary to schistosomal portal hypertension. This is a retrospective prospective study of patients, who underwent splenectomy and devascularisation for schistosomal bleeding varices during the period 1980 to 1990 at Soba university hospital. They had clinical, laboratory, and endoscopic evaluation. They were followed up for a period of 10 years [mean 4.5 years]. At follow up special emphasis was laid on recurrence of variceal hemorrhage and, or mortality. Patients with recurrent variceal bleeding received emergency treatment in the form of resuscitation, balloon tamponade when necessary, and flexible endoscopic sclerotherapy. Splenectomy and oesophagogastric devascularisation was performed in 185 patients, and devascularisation only in 5 patients. Early postoperative mortality occurred in 8 patients [4.2%]. Early recurrence of variceal bleeding occurred in 4 patients who responded to emergency treatment. Transient of treatable ascitis developed in 21% of patients, transient jaundice in 12.6%, and liver failure in 1.6%. Major postoperative septic complications occurred in 6.4%. The rate of recurrence of variceal bleeding was 18.1% within the first 5 years, and 23.6% by 10 years follow-up. Late mortality occurred in 8 patients [4.4%]. The overall mortality of the procedure was 8.4%. Splenectomy and oesophagogastric devascularisation for the treatment of schistosomal portal hypertension can be associated with a high rate of variceal rebleeding. However, when coupled with sclerotherapy for recurrence, it carries an acceptable mortality rate

Frequência etiológica da hipertensão pulmonar em ambulatório de referência na Bahia / Etiological frequency of pulmonary hypertension in a reference outpatient clinic in Bahia, Brazil / Frecuencia etiológica de la hipertensión pulmonar en un ambulatorio de referencia en Bahia

Fundamento: A hipertensão pulmonar (HP) resulta de diversas etiologias com possíveis influências geográficas, porém poucos trabalhos descrevem a frequência etiológica da HP, especialmente em nosso meio. Não está claro se existe associação entre etiologia e nível de pressão pulmonar ou intensidade da sintomatologia. Objetivo: 1) descrever a prevalência etiológica da HP na Bahia; 2) avaliar se a etiologia é fator determinante no nível de pressão pulmonar; 3) avaliar se a etiologia é fator determinante na classe funcional; 4) identificar os demais preditores do nível de pressão pulmonar e da classe funcional. Métodos: Estudo observacional, de corte transversal, em portadores de HP atendidos no Ambulatório Magalhães Neto, entre junho de 2005 e dezembro de 2007. A HP foi definida como pressão sistólica da artéria pulmonar (PSAP) > 40 mmHg pelo ecocardiograma. Pacientes com doença pulmonar obstrutiva crônica ou doença ventricular esquerda não foram incluídos. Resultados: Foram estudados 48 pacientes com PSAP de 86 ± 24 mmHg. Quanto à etiologia, 42 por cento dos pacientes foram classificados como idiopáticos, seguidos de 25 por cento de esquistossomose, 19 por cento de cardiopatia congênita e 10 por cento de embolia pulmonar crônica. O maior tempo de doença prediz pressões mais elevadas em cardiopatas. Indivíduos com esquistossomose tiveram melhor desempenho funcional quando comparados aos demais (456 ± 58 vs. 299 ± 138 metros, p = 0,005). Etiologia esquistossomótica e idades menores apresentam melhor classe funcional. Conclusão: 1) Prevalece a classificação idiopática, sendo a esquistossomose a causa específica mais frequente em nosso meio; 2) pacientes com etiologia esquistossomótica e de idades menores apresentam melhor classe funcional; 3) cardiopatia congênita resulta em maior nível de pressão na artéria pulmonar, provavelmente devido ao maior tempo de doença.

Background: Pulmonary hypertension (PH) results from several etiologies, with possible geographic influences; however, few studies have described the etiological frequency of PH, especially in our country. It is not clear whether there is an association between etiology and pulmonary pressure level or symptom intensity. Objectives: 1) to describe the etiological prevalence of PH in the state of Bahia, Brazil; 2) to evaluate whether the etiology is a determinant factor for the pulmonary pressure level; 3) to evaluate whether the etiology is a determinant factor for functional class; 4) to identify the other predictors of pulmonary pressure level and functional class. Methods: The present was an observational, cross-sectional study that analyzed individuals with PH treated at the Magalhaes Neto Outpatient Clinic, between June 2005 and December 2007. PH was defined as pulmonary artery systolic pressure (PASP) > 40 mmHg measure at the echocardiogram. Patients with chronic obstructive pulmonary disease or ventricular disease were excluded from the study. RESULTS: A total of 48 patients with PASP = 86 ± 24 mmHg were studied. Regarding the etiology, 42 percent of the patients were classified as idiopathic, followed by 25 percent as schistosomatic, 19 percent as congenital cardiopathy and 10 percent of chronic pulmonary embolism. A longer time of disease predicted higher pressures in patients with cardiopathy. Individuals with schistosomiasis had the best functional performance when compared to the others (456 ± 58 vs. 299 ± 138 meters, p=0.005). The schistosomatic etiology and younger age presented better functional class. Conclusions: 1) the idiopathic classification is the most prevalent, with schistosomiasis being the most frequent specific cause in our country; 2) patients with schistosomatic etiology and of younger age present better functional class. 3) the congenital cardiopathy results in a higher pressure level in the pulmonary artery, ...

Fundamento: La hipertensión pulmonar (HP) es la resultante de diversas etiologías con posibles influencias geográficas, pero pocos trabajos describen la frecuencia etiológica de la HP, especialmente en nuestro medio. No está claro si existe asociación entre etiología y nivel de presión pulmonar o intensidad de la sintomatología. Objetivo: (1) Describir la prevalencia etiológica de la HP en Bahía; (2) evaluar si la etiología es factor determinante en el nivel de presión pulmonar; (3) evaluar si la etiología es factor determinante en la clase funcional; (4) identificar los demás predictores del nivel de presión pulmonar y de la clase funcional. Métodos: Estudio observacional, de corte transversal, en portadores de HP atendidos en el Ambulatorio Magalhães Neto, entre junio de 2005 y diciembre de 2007. La HP fue definida como presión sistólica de la arteria pulmonar (PSAP) > 40 mmHg por ecocardiograma. No se incluyó a los pacientes con enfermedad pulmonar obstructiva crónica o deterioro del ventrículo izquierdo. Resultados: Se estudiaron a 48 pacientes con PSAP de 86±24 mmHg. En cuanto a la etiología, el 42 por ciento de los pacientes fue clasificado como idiopático, seguido del 25 por ciento por esquistosomiasis, el 19 por ciento por cardiopatía congénita y el 10 por ciento por embolia pulmonar crónica. El mayor tiempo de enfermedad predice presiones más elevadas en cardiópatas. Los individuos con esquistosomiasis tuvieron mejor desempeño funcional cuando se los comparó con los demás (456±58 vs. 299±138 metros, P = 0,005). Pacientes con etiología esquistosomiásica y edades más jóvenes cursan con mejor capacidad funcional. Conclusión: (1) Prevalece la clasificación idiopática, y la esquistosomiasis constituye la causa específica más frecuente en nuestro medio; (2) pacientes con etiología esquistosomiásica y de edades menores presentan mejor clase funcional; (3) la cardiopatía congénita resulta en mayor nivel de presión en la arteria pulmonar, ...