Portal hyperflow in patients with hepatosplenic mansonic schistosomiasis

Cleva, Roberto de; Saad, William Abrão; Herman, Paulo; Pugliese, Vincenzo; Zilberstein, Bruno; Laudanna, Antônio Atílio; Gama-Rodrigues, Joaquim

doi:10.1590/s0041-87812004000100003

Cited by 49 publications

(65 citation statements)

References 20 publications

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“…are associated with intravascular hemolysis, and consequently there is the potential risk for the development of pulmonary hypertension, such as schistosomiasis (3,4), and iatrogenic hemolysis from mechanical heart valves (5, 6), left ventricular assist devices, and cardiopulmonary bypass procedures (7)(8)(9)(10)(11). Importantly, there are no case reports of pulmonary hypertension complicating iron deficiency anemia, the most common anemia in the world, suggesting a requirement for intravascular hemolysis for the development of this complication.…”

Section: What This Study Adds To the Fieldmentioning

confidence: 99%

Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Anthi¹,

Machado²,

Jison³

et al. 2007

Am J Respir Crit Care Med

229

228

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Rationale: Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population. Objectives: To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH. Methods: Patients with SCD with PH (n ϭ 26) were compared with control subjects with SCD but without PH (n ϭ 17), matched for age, hemoglobin levels, and fetal hemoglobin levels. Measurements and Main Results: Upon catheterization, 54% of the patients with PH had pulmonary arterial hypertension, and 46% had pulmonary venous hypertension. When compared with control subjects, patients with PH exhibited lower six-minute-walk distance (435 Ϯ 31 vs. 320 Ϯ 20 m, p ϭ 0.002) and oxygen consumption (50 Ϯ 3% vs. 41 Ϯ 2% of predicted, p ϭ 0.02), and also had mild restrictive lung disease and more perfusion abnormalities on radionuclide lung scans. The six-minute-walk distance in this population inversely correlated with tricuspid regurgitant jet velocity (r ϭ Ϫ0.55, p Ͻ 0.001), and mean pulmonary artery pressure (r ϭ Ϫ0.57, p Ͻ 0.001), and directly correlated with maximal oxygen consumption (r ϭ 0.49, p ϭ 0.004), even after adjustment for hemoglobin, supporting an independent contribution of increasing pulmonary artery pressures to loss of exercise capacity. Conclusions: Patients with SCD-associated PH have both pulmonary arterial and venous PH associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia. These data support the use of the six-minute-walk distance as an index of PH and cardiopulmonary function in patients with SCD.Keywords: sickle cell disease; pulmonary hypertension; six-minute walk; hemodynamics; echocardiogram Pulmonary arterial hypertension is an increasingly recognized complication of chronic hereditary and acquired hemolytic anemias, including sickle cell disease (SCD), thalassemia intermedia and major, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis and stomatocytosis, microangiopathic hemolytic anemias, pyruvate kinase deficiency, alloimmune hemolytic anemia, and possibly malaria (1, 2). In addition, certain conditions AT A GLANCE COMMENTARY Scientific Knowledge on the SubjectPulmonary hypertension is an emerging complication of sickle cell disease with high mortality. There are few data characterizing hemodynamics and cardiopulmonary function in this population. What This Study Adds to the FieldPatients with sickle cell disease-associated pulmonary hypertension have both pulmonary arterial and venous pulmonary hypertension associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia.are associated with intravascular hemolysis, and consequently there is the potential risk for the development of pulmonary hypertension, such as schistosomiasis (3, 4), and iatrogenic hemolysis from mechanical heart valves (5, 6), left ventricula...

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Section: What This Study Adds To the Fieldmentioning

confidence: 99%

Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Anthi¹,

Machado²,

Jison³

et al. 2007

Am J Respir Crit Care Med

229

228

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“…5 The most severe presentation of the disease is hepatosplenic schistosomiasis mansoni (HSM), which is characterized by splenomegaly, periportal fibrosis, portal hypertension, and upper digestive bleeding caused by rupture of esophageal varices. 6 Ectopic schistosomiasis infection of the central nervous system (CNS) is unusual. Cerebral lesions are caused primarily by S. japonicum, whereas spinal cord lesions are usually caused by S. mansoni and S. haematobium.…”

Section: Introductionmentioning

confidence: 99%

Brain Magnetic Resonance Imaging Findings in Young Patients with Hepatosplenic Schistosomiasis Mansoni without Overt Symptoms

Manzella

Borba-Filho²,

Brandt³

et al. 2012

The American Journal of Tropical Medicine and Hygiene

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Abstract. The purpose of this study was to describe the brain magnetic resonance imaging (MRI) findings in young patients with hepatosplenic schistosomiasis mansoni without overt neurologic manifestations. This study included 34 young persons (age range = 9-25 years) with hepatosplenic schistosomiasis mansoni who had been previously treated. Patients were scanned on a 1.5-T system that included multiplanar pre-contrast and post-contrast sequences, and reports were completed by two radiologists after a consensus review. Twenty (58.8%) patients had MRI signal changes that were believed to be related to schistosomiasis mansoni. Twelve of the 20 patients had small focal hyperintensities on T2WI in the cerebral white matter, and eight patients had symmetric hyperintense basal ganglia on T1WI. There was a high frequency of brain MRI signal abnormalities in this series. Although not specific, these findings may be related to schistosomiasis.

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“…More than 200 million people in 74 countries are infected with Schistosoma spp., and approximately 5-15 million people chronically and recurrently infected with Schistosoma mansoni manifest PAH, a progressive and fatal pulmonary vascular disease (1)(2)(3)(4). Schistosomiasis-associated PAH shares common pulmonary vascular pathology with other forms of PAH, including remodeling of the vascular media and intima, resulting in vascular obstruction (5).…”

mentioning

confidence: 99%

Protective Role of IL-6 in Vascular Remodeling in Schistosoma Pulmonary Hypertension

Graham

Chabon²,

Kumar³

et al. 2013

Am J Respir Cell Mol Biol

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Schistosomiasis is one of the most common causes of pulmonary arterial hypertension worldwide, but the pathogenic mechanism by which the host inflammatory response contributes to vascular remodeling is unknown. We sought to identify signaling pathways that play protective or pathogenic roles in experimental Schistosomainduced pulmonary vascular disease via whole-lung transcriptome analysis. Wild-type mice were experimentally exposed to Schistosoma mansoni ova by intraperitoneal sensitization followed by tail-vein augmentation, and the phenotype was assessed by right ventricular catheterization and tissue histology, as well as RNA and protein analysis. Whole-lung transcriptome analysis by microarray and RNA sequencing was performed, and RNA sequencing was analyzed according to two bioinformatics methods. Functional testing of the candidate IL-6 pathway was determined using IL-6 knockout mice and the signal transducers and activators of transcription protein-3 (STAT3) inhibitor S3I-201. Wild-type mice exposed to S. mansoni demonstrated increased right ventricular systolic pressure and thickness of the pulmonary vascular media. Whole-lung transcriptome analysis determined that the IL-6-STAT3-nuclear factor of activated T cells c2(NFATc2) pathway was up-regulated, as confirmed by PCR and the immunostaining of lung tissue from S. mansoni-exposed mice and patients who died of the disease. Mice lacking IL-6 or treated with S3I-201 developed pulmonary hypertension, associated with significant intima remodeling after exposure to S. mansoni. Whole-lung transcriptome analysis identified the up-regulation of the IL-6-STAT3-NFATc2 pathway, and IL-6 signaling was found to be protective against Schistosomainduced intimal remodeling.Keywords: pulmonary hypertension; schistosomiasis; gene expression profiling; Schistosomiasis is one of the most common causes of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) worldwide. More than 200 million people in 74 countries are infected with Schistosoma spp., and approximately 5-15 million people chronically and recurrently infected with Schistosoma mansoni manifest PAH, a progressive and fatal pulmonary vascular disease (1-4). Schistosomiasis-associated PAH shares common pulmonary vascular pathology with other forms of PAH, including remodeling of the vascular media and intima, resulting in vascular obstruction (5). Studies aimed at identifying the pathogenesis of schistosomiasis-associated PAH have revealed key roles of T helper (Th)2-mediated inflammation, which may also participate in other forms of PAH in which inflammation likely contributes to the disease, including idiopathic PAH (IPAH), connective tissue diseaseassociated PAH (6), and hypoxic pulmonary hypertension (PH) (7).Mice exposed to S. mansoni develop experimental pulmonary hypertension (PH), which recapitulates several key features of the human disease, including increased right ventricular (RV) pressure, pulmonary vascular remodeling, and RV hypertrophy. RV hypertrophy in particular is assoc...

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Portal hyperflow in patients with hepatosplenic mansonic schistosomiasis

Abstract: Esophagogastric devascularization and splenectomy promote a significant reduction of the elevated portal pressure and flow in schistosomal portal hypertension. These data favor the hypothesis of portal hyperflow in the physiopathology of portal hypertension of schistosomiasis.

Cited by 49 publications

References 20 publications

Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension

Brain Magnetic Resonance Imaging Findings in Young Patients with Hepatosplenic Schistosomiasis Mansoni without Overt Symptoms

Protective Role of IL-6 in Vascular Remodeling in Schistosoma Pulmonary Hypertension

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