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Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, non-malignant hematologic disease characterized by complement-mediated hemolysis (with or without hemoglobinuria), fatigue, increased susceptibility to thrombosis, and bone marrow dysfunction. The development of complement inhibitors has transformed outcomes for patients with PNH, but patients may still experience pharmacodynamic breakthrough hemolysis (BTH), which can be caused by exposure to a complement amplifying condition (CAC), such as vaccination, infection, or surgery.
Materials And Methods: A 13-member expert panel used a validated methodology (a RAND/UCLA modified Delphi panel) to develop consensus on how to classify pharmacodynamic BTH in patients with complement-inhibitor treated PNH.
Management of paroxysmal nocturnal hemoglobinuria with low-level hemolysis in pregnancy- a report of two cases.
Ann Hematol
November 2024
Department of Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria.
Article Synopsis
- Pregnant women with paroxysmal nocturnal hemoglobinuria (PNH) face high risks of serious blood clots, but eculizumab, a complement inhibitor, can help reduce these risks and improve pregnancy outcomes.
- This study examines two pregnant patients with PNH showing low-level hemolysis, managed solely with preventive anticoagulation and no complement inhibition.
- Both patients had successful pregnancies without thromboembolic issues, suggesting that careful monitoring and anticoagulation might be a viable management approach for certain PNH cases during pregnancy, though more research is necessary.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder characterized by the absence of complement regulatory proteins on the surface of erythrocytes, leading to intravascular hemolysis and thrombosis. Managing PNH during pregnancy poses significant challenges due to increased risks of morbidity and mortality. This case report describes the detailed obstetric course of a 44-year-old woman with PNH and additional hereditary protein C deficiency who had previously experienced multiple thrombotic events and adverse pregnancy outcomes (two early miscarriages and one stillbirth at 25 weeks gestation [WG]), treated with eculizumab (terminal C5 inhibitor) and optimal anticoagulation management.
View Article and Find Full Text PDF[Paroxysmal nocturnal hemoglobinuria during pregnancy: a case report and literature review].
Zhonghua Xue Ye Xue Za Zhi
September 2024
Huashan Hospital Affiliated to Fudan University, Shanghai 200040, China.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, and a standardized treatment and follow-up management strategy for pregnant women with this disease are crucial for ensuring the safety of both the mothers and their newborns. This study includes a case of pregnant women with PNH. In accordance with the guidelines, the patient was successfully delivered after treatment with eculizumab.
View Article and Find Full Text PDFA familial case of diffuse periventricular nodular heterotopia identified prenatally: Filamin A defect as the probable cause.
Radiol Case Rep
November 2024
Department of Medical Genetics, Hospital de Santa Maria, Unidade Local de Saúde Santa Maria, Av. Professor Egas Moniz, Lisboa 1649-035, Portugal.
Article Synopsis
- Periventricular nodular heterotopia (PNH) is a brain condition caused by faulty neuron migration, leading to the formation of abnormal gray matter near the lateral ventricles, primarily affecting females due to its genetic link to the Filamin A gene on the X chromosome.
- A case study involving a 28-year-old woman with a history of respiratory issues and a family background of asthma and epilepsy showed signs of PNH in her female fetus at 20 weeks of pregnancy through ultrasounds and neurosonography.
- Genetic testing confirmed the PNH diagnosis in both the fetus and the mother, highlighting the importance of advanced imaging and genetic analysis in prenatal care for managing hereditary conditions.
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