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-Chest X-ray showing prominence of the pulmonary artery trunk, as well as of the right pulmonary artery and its interlobar branch.
Source publication
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%) suffer from pulmonary arterial hypertension, which is associated with the hepatosplenic form of the disease....
Context in source publication
Context 1
... serology was negative for hepatitis B surface antigen (HBsAg), antibody to hepatitis B surface antigen (anti-HBs), antibody to hepatitis B core antigen (anti-HBc), and antibody to hepatitis C virus (anti-HCV). A chest X-ray showed dilated pulmonary arteries and right heart enlargement (Figure 1), both of pulmonary artery systolic pressure, calculated on the basis of the tricuspid regurgitant jet velocity and the right atrial pressure. (4,6) Schistosomiasis is the third most prevalent endemic parasitic disease in the world, surpassed only by malaria and amebiasis. ...
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Citations
... Diagnosis of chronic schistosomiasis is based on the identification of eggs in stool or urine by direct microscopy or rectal/bladder biopsy [1]. Chest roentgenogram shows enlargement of the right ventricle, dilatation of the pulmonary arteries and trunk as well as their interlobar branches that indicates pulmonary hypertension and cor pulmonale [176]. Bronchoscopic examination and tissue biopsy are unlikely to be useful because of the small amount of the tissue obtained and the sporadic distribution of the granulomas [166]. ...
... A short course treatment of steroids is effective in acute form before the beginning of praziquantel treatment and can be treated with artemether, an artemisinin derivative which acts on the juvenile forms of the schistosome [166]. A patient with schistosomiasis-associated pulmonary hypertension was reported of successful treatment with a phosphodiesterase-5 inhibitor [176]. ...
Parasitic infestations demonstrated a decline in the past decade as a result of better hygiene practices and improved socioeconomic conditions. Nevertheless, global immigration, increased numbers of immunocompromised people, international traveling, global warming, and rapid urbanization of the cities have increased the susceptibility of the world population to parasitic diseases. A number of new human parasites, such as Plasmodium knowlesi, in addition to many potential parasites, have urged the interest of scientific community. A broad spectrum of protozoal parasites frequently affects the respiratory system, particularly the lungs. The diagnosis of parasitic diseases of airway is challenging due to their wide varieties of clinical and roentgenographic presentations. So detailed interrogations of travel history to endemic areas are critical for clinicians and pulmonologists to manage this entity. The migrating adult worms can cause mechanical airway obstruction, while the larvae can cause airway inflammation. This paper provides a comprehensive treview of both protozoal and helminthic infestations that affect the airway system, particularly the lungs, including clinical and roentgenographic presentations, diagnostic tests, and therapeutic approaches.
... We read with interest the recent publications in the Brazilian Journal of Pulmonology highlighting the prevalence of pulmonary hypertension (PH) in Brazil, particularly as a complication of sickle cell anemia (1) and schistosomiasis, (2) and discussing the role of imaging methods in the evaluation of this disease. (3)(4)(5) We would like to report another uncommon cause of PH, pulmonary capillary hemangiomatosis (PCH), and describe the important role of imaging methods in establishing the diagnostic suspicion of this condition. ...
Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.
Parenteral prostacyclin therapy is first-line for Group 1 pulmonary arterial hypertension patients with severely limited functional status. There are two current pharmacologic options in this realm, intravenous epoprostenol and intravenous or subcutaneous treprostinil. In this chapter, we will discuss similarities and differences between the two formulations, present practical knowledge for prescribing these therapies, and outline the current indications and the data supporting those recommendations. We will dissect the components of the clinical trials that exist in this realm and controversies therein. Lastly, we will discuss the timing of initiation of therapy, as well as patient-focused concerns and challenges with parenteral therapy.
Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is a life-threatening complication of chronic hepatosplenic schistosomiasis. It is suggested to be the leading cause of pulmonary arterial hypertension (PAH) worldwide. However, pathophysiological data on Sch-PAH are scarce. We examined the hypothesis that there are pronounced similarities in pathophysiology, haemodynamics, and survival of Sch-PAH and idiopathic PAH (iPAH).
This systematic review and meta-analysis was registered in the PROSPERO database (identifier CRD42018104066). A systematic search and review of the literature was performed according to PRISMA guidelines for studies published between 01 January 1990 and 29 June 2018.
For Sch-PAH, 18 studies evaluating pathophysiological mechanisms, eight studies on haemodynamics (n=277), and three studies on survival (n=191) were identified. 16 clinical registries reporting data on haemodynamics and survival including a total of 5792 patients with iPAH were included for comparison. Proinflammatory molecular pathways are involved in both Sch-PAH and iPAH. The transforming growth factor (TGF)-β signalling pathway is upregulated in Sch-PAH and iPAH. While there was no difference in mean pulmonary artery pressure (54±17 mmHg versus 55±15 mmHg, p=0.29), cardiac output (4.4±1.3 L·min ⁻¹versus 4.1±1.4 L·min ⁻¹ , p=0.046), and cardiac index (2.6±0.7 L·min ⁻¹ ·m ⁻²versus 2.3±0.8 L·min ⁻¹ ·m ⁻² , p<0.001) were significantly higher in Sch-PAH compared to iPAH, resulting in a lower pulmonary vascular resistance in Sch-PAH (10±6 Woods units versus 13±7 Woods units, p<0.001). 1- and 3-year survival were significantly better in the Sch-PAH group (p<0.001).
Sch-PAH and iPAH share common pathophysiological mechanisms related to inflammation and the TGF-β signalling pathway. Patients with Sch-PAH show a significantly better haemodynamic profile and survival than patients with iPAH.
Background:
Pulmonary schistosomiasis may complicate urinary or intestinal infestations. Pulmonary pathology is either in the acute or chronic form. The chronic form of the disease may result in granuloma formation. This study presents 20 years of experience in surgical management of pulmonary bilharziomas.
Methods:
A retrospective review was undertaken of 17 consecutive patients who had surgery for lung bilharziomas from 1996-2016. Demographics, clinical presentation, underlying lung disease, investigations performed, operative procedure, and outcome were retrieved and reviewed.
Results:
All patients were males, with ages ranging from 22-52 years (median 33 years). Haemoptysis was the main presentation (53%). Coexisting lung tuberculosis was present in five (29.4%) patients. Indications for surgery were solitary shadows in 12 (70.6%) patients and persistent tuberculous cavities in five (29.4%) patients. Segmentectomy was performed in one (5.9%) patient, lingulectomy in one (5.9%) patient, lobectomy in 14 (82.3%) patients, and bi-lobectomy in one (5.9%) patient. The histologic nature of the infestation was: bilharzial ova with extensive granulomatous reaction and suppuration in eight cases (47%); both tuberculosis and bilharzial ova within a granulomatous tissue reaction in five cases (29.4%); and bilharzial ova within malignant tissue in four cases (23.6%). There was no operative mortality. One (1) patient (5.9%) developed postoperative bronchopleural fistula after left upper lobectomy; surgical repair of the fistula and omental flap buttress was needed after failure of conservative management.
Conclusion:
Pulmonary schistosomiasis is not an uncommon infestation and occurs more frequently in patients with underlying tuberculosis. It may predispose to granulomatous parenchymatous lung masses or even malignancy, which necessitate surgical intervention with a good outcome. However, predisposition of pulmonary schistosomiasis for the development of bronchogenic carcinoma warrants further studies.
Schistosomiasis is the most common parasitic disease associated with pulmonary hypertension. It induces remodelling via complex inflammatory processes, which eventually produce the clinical manifestation of pulmonary hypertension. The pulmonary hypertension shows clinical signs and symptoms that are not distinguishable from other forms of pulmonary arterial hypertension.
Schistosomiasis is the third most common endemic disease and has been considered the most common cause of pulmonary arterial hypertension (PAH) in the world. Around 6 % of people with chronic schistosomiasis in endemic areas are affected. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is classified by the World Health Organization as group 1 pulmonary arterial hypertension because it has clinical, pathological, and hemodynamic characteristic that are similar to idiopathic pulmonary arterial hypertension. Sch-PAH is a severe disease that affects mainly middle-aged women. Symptoms and signs of right-heart insufficiency may be present, such as dyspnea, syncope, and peripheral edema. It has been demonstrated that Sch-PAH has a more benign clinical course and a better survival than idiopathic pulmonary hypertension. There are few reports of any beneficial effect from specific therapy directed toward group 1 PAH in this population of patients. The mechanisms responsible for the development of pulmonary hypertension in patients with schistosomiasis are unknown. There is growing evidence that vessel obstruction and granulomatous reaction to pulmonary embolization of eggs is not the sole mechanism that causes the disease. Further studies are needed to better elucidate the pathogenesis of Sch-PAH and to develop targeted therapies for this devastating disease.
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.
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.
