Chemotherapy Improved Pulmonary Arterial Hypertension in a Patient with Chronic-Active Epstein-Barr Virus Infection.
Chemotherapy Improved Pulmonary Arterial Hypertension in a Patient with Chronic-Active Epstein-Barr Virus Infection. Int Heart J. 2020 Jan 17;: Authors: Akagi S, Miki T, Sando Y, Fujii N, Sarashina T, Nakamura K, Ito H Abstract Chronic-active Epstein-Barr virus infection (CAEBV) is a rare disease that can lead to pulmonary arterial hypertension (PAH). However, the treatment for CAEBV-associated PAH has not been established. We discuss a case of improved pulmonary hypertension after chemotherapy in a patient with CAEBV-associated PAH. A 44-year old man was admitted to our hospital because of an abnormal electrocardiogram and liver dysfunction detected by annual medical examination. Echocardiography showed a dilated right ventricle and an estimated right ventricular systolic pressure of 92 mmHg. Right heart catheterization revealed a mean pulmonary arterial pressure of 45 mmHg and pulmonary vascular resistance of 9.8 Wood units. Laboratory examination showed granular lymphocytes and 91% natural killer cells in lymphocyte subsets in peripheral blood. We diagnosed the patient as having CAEBV-associated PAH. After two cycles of chemotherapy without PAH-specific drugs, echocardiography showed improvement in the dilated right ventricle and an estimated right ventricular systolic pressure of 59 mmHg. Right heart catheterization revealed a mean pulmonary arterial pressure of 27 mmHg and pulmonary vascular resistance of 2.4 Wood units. Chemotherapy may improve pulm...
Suprasystemic pulmonary hypertension (PH) is highly predictive of pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH).
ConclusionThe absence of PSGL ‐1 induces a reduction in Treg cells, NO production, and ERα expression and causes an increase in Ang II in the lungs of female mice, favoring the development of PAH.
This article reviews the current drug therapies and their use in the management of PAH in children.
This article reviews the current drug therapies and their use in the management of PAH in children. PMID: 31960361 [PubMed - as supplied by publisher]
AbstractPulmonary arterial hypertension is a rare disease, with drug-induced causes even more uncommon, accounting for only 10% of cases in large registry series. Predisposing factors for drug-induced PAH have not been completely defined. This review summarizes drugs with definite, possible, or likely association to pulmonary hypertension and possible mechanisms involved in the occurrence of pulmonary hypertension. Controversies on mechanisms and on their role in pathophysiology were also shown. The possible synergism between drug abuse and HIV was discussed and the possible interactions of antiretroviral therapy in HIV su...
We present five cases of TBX4 related DPLD/PH who survived beyond infancy with different disease progression. Registry-based follow-up is mandatory to understand genotype-phenotype correlation and improve treatment strategies and prognosis for this rare disease.
ConclusionHSS is a rare type of vasculitis, which can present with arterial, venous and/or intracardiac thrombosis even without other systemic manifestations. HSS can present without venous involvement. HSS require aggressive immune suppressive treatment, however anticoagulation and surgery can be attempted when indicated.
ConclusionPSGL ‐1 absence induces reduction of Treg, NO production and ERα expression and the increase of Angiotensin II in the lungs of female mice, favoring the development of PAH
Systemic sclerosis (SSc) is a rare disease characterized by widespread collagen deposition resulting in fibrosis. Although skin involvement is the most common manifestation and also the one that determines the classification of disease, mortality in SSc is usually a result of respiratory compromise in the form of interstitial lung disease (ILD) or pulmonary hypertension (PH). Clinically significant ILD is seen in up to 40% of patients and PH in up to 20%. Treatment with either cyclophosphamide or mycophenolate has been shown to delay disease progression, whereas rituximab and lung transplantation are reserved for refractory cases.
Sickle cell disease (SCD) is the most common inherited blood disorder in the United States, afflicting nearly 100,000 Americans. Patients with SCD have sickle-shaped red blood cells, which have trouble moving through the blood vessels and thus cause blockages or slow blood flow and oxygen to parts of the body. SCD is life-threatening, due to potential complications from blocked blood vessels, which can include stroke, difficulty breathing, pulmonary hypertension and other organ damage.