1,401 results match your criteria: "Pulmonary Venoocclusive Disease"
Combined CAR-T/HSCT approach in a patient with refractory acute lymphoblastic leukemia and cystic fibrosis.
Tumori
December 2024
Department of Pediatrics, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
Introduction: The association of acute lymphoblastic leukaemia (ALL) and cystic fibrosis (CF) is rare. We present the case of a paediatric patient affected by CF and refractory B-cell precursor (BCP) ALL, who was treated with combined chimeric antigen receptor T-cells (CAR-T) and allogeneic haematopoietic stem cell transplantation (HSCT).
Case Description: Autologous-CD19 targeting CAR-T allowed to achieve molecular remission and spare chemo-related toxicity.
Pulmonary veno-occlusive disease (PVOD) is a lethal variant of pulmonary hypertension. The degree of pulmonary arterial involvement varies. Here, we compare two PVOD patients who were transplanted at 8 years of age, whereof one is a homozygous mutation carrier.
View Article and Find Full Text PDFPosterior Spinal Cord Infarction Complicating a Bronchial Arterial Embolization.
Cureus
November 2024
Intensive Care Unit, Hospital Vila Franca de Xira, Vila Franca de Xira, PRT.
Massive hemoptysis is a life-threatening condition. Bronchial artery embolization (BAE) is an effective technique for controlling bleeding in cases of severe hemoptysis, with infrequent complications. While rare, spinal cord infarction is a serious potential complication of BAE.
View Article and Find Full Text PDFPulmonary veno-occlusive disease: A systematic review of risk factors, clinical presentation, diagnostic investigations, treatment outcomes and prognostic factors.
J Med Imaging Radiat Oncol
December 2024
Department of Cardiology, St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. We aimed to systematically evaluate published cases of PVOD to provide an overview of their clinical presentation, management and prognosis to assist early identification and treatment. We conducted a literature search of PubMed and Embase databases for adult cases of 'pulmonary veno-occlusive disease' and 'pulmonary capillary haemangiomatosis'.
View Article and Find Full Text PDFBurden of illness of non-hematopoietic stem cell transplant-related hepatic sinusoidal obstruction syndrome: A systematic review.
Heliyon
October 2024
Jazz Pharmaceuticals, Oxford, UK.
Article Synopsis
Case report: A finding of PVOD and PAH in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases.
Respirol Case Rep
November 2024
Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a rare pulmonary vascular condition that can be mistaken for pulmonary arterial hypertension (PAH), and they are believed to have different genetic causes.
- This report details a case of a woman diagnosed with PAH at 42, who unfortunately passed away despite treatment, and her son, who was diagnosed with PAH at 16 and required a heart-lung transplant due to worsening conditions.
- The autopsy of the mother showed evidence consistent with PAH, while the son's lung pathology confirmed PVOD, and neither had identifiable genetic mutations linked to these diseases.
Potential therapeutic uses of L-citrulline beyond genetic urea cycle disorders.
J Inherit Metab Dis
November 2024
George Washington University, Maryland, USA.
Article Synopsis
- L-citrulline, a non-essential amino acid involved in the urea cycle, is primarily known for its role in managing genetic urea cycle disorders, but recent studies reveal its potential in treating conditions linked to nitric oxide deficiency and oxidative stress.
- Citrulline may have beneficial effects in several medical conditions such as sickle cell disease, post-operative pulmonary hypertension, hepatic veno-occlusive disease, and bronchopulmonary dysplasia, by enhancing nitric oxide production and improving vascular health.
- While promising results suggest citrulline could serve as an adjunct therapy in these conditions, more research is needed to establish proper dosing and long-term safety and effectiveness.
Long-term sildenafil therapy for pulmonary veno-occlusive disease in association with melphalan therapy for multiple myeloma: A case report.
SAGE Open Med Case Rep
November 2024
Division of Pulmonology, Medical University of Graz, Graz, Austria.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) has a poorer prognosis than idiopathic pulmonary arterial hypertension (PAH), with median survival of only 1 year post-diagnosis.
- Treatments for PAH have shown limited effectiveness for PVOD, with risks such as pulmonary oedema and mixed results from case reports on medication effects.
- Lung transplantation is the best long-term treatment for suitable patients, but candidates over a certain age or with certain cancers are typically excluded; a case study describes a successful 5-year sildenafil treatment for a 59-year-old patient with PVOD and multiple myeloma.
Venous Thromboembolism Incidence and Risk Factors in Patients Undergoing Hematopoietic Stem Cell Transplantation.
Transplant Cell Ther
November 2024
Department of Cancer Pharmacology & Pharmacogenomics, Levine Cancer Institute, Atrium Health, Charlotte, North Carolina.
Article Synopsis
Survival outcomes in EIF2AK4 mutation-associated pulmonary arterial hypertension: seeking clarity in contrast.
Eur Heart J Case Rep
November 2024
National Pulmonary Hypertension Centre, Royal Brompton Hospital, Part of GSTT Foundation Trust, Sydney Street, London SW3 6NP, UK.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH) linked to mutations in the EIF2AK4 gene, particularly presenting severe outcomes for those with homozygous mutations.
- This case study highlights two patients with homozygous EIF2AK4 mutations who responded exceptionally well to pulmonary vasodilator therapy and showed stable health over a 2 to 10-year follow-up period.
- The findings suggest that intrapulmonary shunts (IPS) might help relieve pressure on the right ventricle while maintaining oxygen levels, indicating a potentially better prognosis for PVOD patients compared to classical PAH cases.
Late-Onset Noninfectious Pulmonary Complications after Hematopoietic Stem Cell Transplantation.
Transplant Cell Ther
September 2024
Division of Pulmonary, Critical Care and Sleep Medicine, Lenox Hill Hospital, Northwell Health, New York, New York. Electronic address:
GCN2 kinase activation mediates pulmonary vascular remodeling and pulmonary arterial hypertension.
JCI Insight
September 2024
Program for Lung and Vascular Biology, Section for Injury Repair and Regeneration Research, Stanley Manne Children's Research Institute, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
Article Synopsis
Recurrent Pulmonary Veno-Occlusive Disease after Lung Transplantation.
Am J Respir Crit Care Med
December 2024
Department of Pulmonary Medicine and.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a rare and severe type of pulmonary hypertension that obstructs blood flow in pulmonary arteries and veins, leading to high pressure and right heart failure with a poor prognosis.
- Research shows that the chemotherapy drug mitomycin C (MMC) activates certain stress response pathways (eIF2 kinase PKR and the integrated stress response) that worsen vascular issues in PVOD.
- Aged rats respond worse to MMC treatment compared to younger ones due to higher basal stress response activity, and blocking this stress response may offer new therapeutic options for treating PVOD.
The spectrum of systemic sclerosis-associated pulmonary hypertension: Insights from the ASPIRE registry.
J Heart Lung Transplant
October 2024
Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK; Division of Clinical Medicine, School of Medicine and Population Health, University of Sheffield, Sheffield, UK. Electronic address:
Background: There are limited data assessing the spectrum of systemic sclerosis-associated pulmonary hypertension (PH).
Methods: Data for 912 systemic sclerosis patients assessed between 2000 and 2020 were retrieved from the Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre (ASPIRE) registry and classified based on 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines and multimodality investigations.
Results: Reduction in pulmonary vascular resistance (PVR) diagnostic threshold to >2WU resulted in a 19% increase in precapillary PH diagnoses.
Article Synopsis
- Treatment for pulmonary arterial hypertension (PAH) helps improve patients' quality of life and walking ability but does not fix the underlying vascular issues.
- A patient with connective tissue disease and PAH, who was on triple therapy, developed metastatic lung cancer but showed significant improvement in her PAH symptoms after receiving a combination of chemotherapy and immunotherapy.
- The case indicates that combining cancer treatment may enhance the efficacy of PAH therapies, but the patient’s condition worsened after stopping cancer treatment, highlighting the challenges in managing both conditions simultaneously.
Reversal of pulmonary veno-occlusive disease phenotypes by inhibition of the integrated stress response.
Nat Cardiovasc Res
July 2024
Cardiovascular Research Institute, University of California, San Francisco, San Francisco, CA, USA.
Article Synopsis
Persistent hypoxemia in a child with medulloblastoma following a third autologous stem cell transplant.
Pediatr Pulmonol
December 2024
Department of Pediatric Hematology, Oncology, and Bone Marrow Transplant, Mayo Clinic, Rochester, Minnesota, USA.
Pediatric Lung Transplantation for Pulmonary Vascular Diseases: Recent Advances and Challenges.
Clin Chest Med
September 2024
Division of Pulmonary Medicine, Harvard Medical School, Boston Children's Hospital, 300 Longwood Avenue, BCH 3121, Boston, MA 02115, USA.
Article Synopsis
- - Pediatric lung transplantation for pulmonary vascular diseases has advanced significantly, with new medical therapies, surgical options, and bridging techniques like ECMO enhancing treatment possibilities.
- - Key challenges in this area include patient adherence to post-transplant care, managing complications such as graft dysfunction and rejection, and the need for more research on rare conditions.
- - The review article will discuss these advancements, trends, and ongoing challenges related to pediatric lung transplantation for pulmonary vascular diseases.
Inhalation of hydrogen gas protects against mitomycin-induced pulmonary veno-occlusive disease.
Respir Res
July 2024
State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangdong Key Laboratory of Vascular Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, 510120, China.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a severe form of pulmonary hypertension that currently has no effective treatments, prompting research into the potential benefits of inhaled hydrogen gas.
- In a study with female rats, hydrogen inhalation showed positive effects by improving heart function, reversing heart enlargement, and preventing changes in pulmonary blood vessels when administered either before or after a harmful substance (mitomycin C).
- The use of hydrogen gas decreased markers of oxidative stress and inflammation in lung tissue and serum, while also promoting protective signaling pathways, suggesting that hydrogen inhalation could help counteract the damaging effects associated with PVOD.
Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.
BMC Pulm Med
July 2024
1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland.
Article Synopsis
- Sarcoidosis-associated pulmonary hypertension (SAPH) is classified as Group 5 pulmonary hypertension and often develops from advanced lung fibrosis, but about 30% of patients show no significant fibrosis, indicating other causes like vascular lesions.
- A case study of a 69-year-old woman highlighted that her SAPH resulted from pulmonary artery stenosis due to enlarged lymph nodes, despite lacking thrombi.
- Effective early detection strategies for pulmonary hypertension in sarcoidosis patients are necessary, as well as identifying the specific phenotype of the condition to guide treatment.
Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent.
Iran J Med Sci
June 2024
Shaheed Rajaie Cardiovascular Medical and Research Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Despite its rarity, pulmonary capillary hemangiomatosis (PCH) presents a significant diagnostic challenge. Due to its similarity to other pulmonary vascular diseases, such as pulmonary veno-occlusive disease, it is characterized by abnormal pulmonary capillary proliferation, which is a rare cause of primary pulmonary hypertension. This case was the first reported instance of PCH in Shahid Rajaee Heart Hospital in Tehran, Iran, in 2023, which was confirmed by genetic testing.
View Article and Find Full Text PDFIs anticoagulative therapy in systemic sclerosis to be reconsidered?
J Scleroderma Relat Disord
June 2024
Department of Medical Sciences and Public Health, Hemostasis and Thrombosis Unit, University of Cagliari and AOU of Cagliari, Cagliari, Italy.
Article Synopsis
- - Systemic sclerosis is a rare and serious autoimmune disease characterized by vascular and tissue fibrosis, leading to complications like increased clotting and pulmonary hypertension.
- - The condition triggers endothelial damage, causing immune and platelet activation, which contributes to blood clot formation and potential vascular obstruction.
- - Research is ongoing for new anticoagulant treatments, including anti-factor XI drugs and various molecular types, aimed at improving patient outcomes and minimizing bleeding risks.
Venous retrograde approach for endovascular angioplasty in chronic total pulmonary vein occlusion -a case report.
BMC Cardiovasc Disord
June 2024
Heart, Lung and Vessels Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, 610072, Sichuan, China.
Article Synopsis
- * A case was presented of an older patient with FM that caused pulmonary hypertension, diagnosed using echocardiography and CT imaging, which showed occlusion and stenosis in the pulmonary vessels.
- * The treatment involved a two-stage endovascular intervention; initially unsuccessful, they switched to a retrograde approach and successfully opened the occluded pulmonary vein, with a stent placement, offering new insights into managing similar cases.
Efficacy of Drug-Coated Balloon Angioplasty in Pulmonary Vein Stenosis or Total Occlusion.
JACC Clin Electrophysiol
August 2024
Heart, Vascular, and Thoracic Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA. Electronic address:
Article Synopsis
- Current treatments for pulmonary vein stenosis (PVS) and total occlusion (PVTO) face challenges with high rates of restenosis, prompting a study on a new method.
- This study compares drug-coated balloon (DCB) angioplasty and stenting to traditional angioplasty and stenting, analyzing data from two patient groups over different time periods.
- Results show that the DCB method significantly lowers the chances of restenosis and need for additional procedures compared to standard care, highlighting its effectiveness and safety.