PH Is Possible Complication of Bone Marrow Transplants in Children, Study Shows - Pulmonary Hypertension News
PH Is Possible Complication of Bone Marrow Transplants in Children, Study Shows - Pulmonary Hypertension News |
| Posted: 04 Dec 2019 08:30 AM PST Children who undergo bone marrow transplants could develop pulmonary hypertension (PH), which could lead to severe complications if not diagnosed and treated promptly, a retrospective study found. The study, "Pulmonary hypertension after bone marrow transplantation in children," was published in the European Respiratory Journal. Hematopoietic stem cell transplantation (HSCT), or bone marrow transplant, is a common treatment for blood cancers in adults and children. However, it can be associated with different complications. Children who undergo this procedure can develop a form of PH that seems to be different from idiopathic pulmonary hypertension. The causes and risk factors for this form of PH remain unknown, but it could be associated with the treatment given patients before receiving the transplant. To learn more, researchers in France performed a retrospective study designed to better understand the relationship between bone marrow transplant and PH in children. The team analyzed the clinical records of 366 children who underwent HSCT between January 2008 and December 2015. Patients who developed unexplained respiratory symptoms, such as rapid breathing, low oxygen levels (hypoxia), or difficulty breathing, were examined by echocardiogram to measure their pulmonary blood pressure non-invasively. Overall, 70 children presented unexplained respiratory symptoms, 22 of whom were later diagnosed with PH. Patients started receiving treatment immediately after diagnosis, with the specific treatment dependent on the individual symptoms of each child. One child died before doctors could start treatment. The remaining children were treated with calcium channel blockers, phosphodiesterase-5 inhibitors, or endothelin receptor antagonists (ERA), either alone or in combination. Initially, 12 children received one therapy, five received a combination of two therapies, and four received a combination of three therapies. After two days of treatment, seven children started receiving an additional therapy because they were not getting better. Seven patients (32%) died, six due to PH complications, and one due to infection. The remaining 15 are alive after a median follow-up of 7.5 years. Pulmonary pressure went back to normal in all survivors after approximately three weeks of treatment, and remained normal during follow-up. All survivors stopped taking PH medication after a median of 5 months. The results showed that late diagnosis was significantly associated with a higher risk of death. Children who died were diagnosed in a median 33.5 days after the first respiratory symptoms appeared, whereas survivors were diagnosed after a median of one week. The team also noted that treatment and time of diagnosis improved with time, and that the percentage of surviving children increased considerably in the last years of the study, with no deaths reported after 2012. "Even if the pathophysiology of PH is still unclear in [children who undergo bone marrow transplant], our experience indicates that rapid treatment with oxygen and potent specific treatments of PH allow improvement and pulmonary pressure normalization in all our recent cases," the researchers said. Overall, the team concluded that "pulmonary hypertension is a severe complication of HSCT with an underestimated incidence and high mortality," and that "aggressive and timely up-front combination therapy allowed normalisation of pulmonary pressure and improved survival." The researchers emphasized that doctors performing bone marrow transplants should be aware of this possible complication, and "seek prompt consultation with an experienced cardiologist whenever PH is suspected."  Alejandra has a PhD in Genetics from São Paulo State University (UNESP) and is currently working as a scientific writer, editor, and translator. As a writer for BioNews, she is fulfilling her passion for making scientific data easily available and understandable to the general public. Aside from her work with BioNews, she also works as a language editor for non-English speaking authors and is an author of science books for kids. × Alejandra has a PhD in Genetics from São Paulo State University (UNESP) and is currently working as a scientific writer, editor, and translator. As a writer for BioNews, she is fulfilling her passion for making scientific data easily available and understandable to the general public. Aside from her work with BioNews, she also works as a language editor for non-English speaking authors and is an author of science books for kids. Latest Posts  |
| Acute Pulmonary Embolism Clinical Practice Guidelines (ESC, 2019) - Medscape Posted: 03 Dec 2019 01:38 PM PST  Guidelines for the diagnosis and management of acute pulmonary embolism were published in 2019 by the European Society of Cardiology (ESC).[1] Perform bedside transthoracic echocardiography as an immediate step to differentiate suspected high-risk pulmonary embolism (PE) from other acute life-threatening situations in patients presenting with hemodynamic instability. If acute PE is suspected, institute anticoagulation therapy as soon as possible, unless the patient is bleeding or has absolute contraindications. Use recommended, validated diagnostic algorithms for PE, including standardized assessment of (pre-test) clinical probability and D-dimer testing. Consult a radiologist and/or seek a second opinion if the computed tomography pulmonary angiogram (CTPA) report suggests single subsegmental PE. In a patient without hemodynamic instability, confirmation of PE must be followed by further risk assessment involving clinical findings and comorbidity along with evaluation of the size and/or function of the right ventricle (RV), and with laboratory biomarkers if appropriate. As soon as you diagnose (or strongly suspect) high-risk PE, select the best reperfusion option (systemic thrombolysis, surgical embolectomy, or catheter-directed treatment), considering the resources and expertise available at your hospital. Reperfusion is not a first-line treatment for patients with intermediate–high-risk PE. When oral anticoagulation is started in a patient with PE who is eligible for a novel oral anticoagulant (NOAC) (apixaban, dabigatran, edoxaban, or rivaroxaban), a NOAC is recommended in preference to a vitamin K antagonist (VKA). A reduced dose of the NOACs apixaban (2.5 mg b.i.d.) or rivaroxaban (10 mg o.d.) should be considered if extended oral anticoagulation is decided after PE in a patient without cancer. There is a lifelong risk of venous thromboembolism (VTE) recurrence after the first episode of PE, therefore the patient should be re-examined after the first 3–6 months of anticoagulation. If PE is suspected in a pregnant patient, utilize formal diagnostic pathways and algorithms, including CTPA or ventilation–perfusion lung scan if needed, which can be used safely during pregnancy. Follow-up imaging is not routinely recommended in an asymptomatic patient, but it may be considered in patients with risk factors for the development of chronic thromboembolic pulmonary hypertension (CTEPH). For more Clinical Practice Guidelines, go to Guidelines. For more information, go to Acute Pulmonary Embolism.  |
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