Background: Thrombotic microangiopathy is a pathological condition comprised of microvascular thrombosis involving any organ of the body leading to thrombocytopenia, Coombs-negative hemolytic anemia, and end-organ damage. The most common forms of thrombotic microangiopathies are Shiga toxin-producing Escherichia colimediated hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome. The atypical hemolytic uremic syndrome occurs due to genetic and acquired mutations in complement regulatory factors and to complement activation factors in the immune system, mainly the alternative pathway. Clinical manifestations and outcomes differ with the prevalent mutations of the patient. Currently, available treatment modalities are therapeutic plasma exchange and a monoclonal antibody against C5, eculizumab. We report a case of a Sri Lankan girl diagnosed with atypical hemolytic uremic syndrome complicated with septicemia, hemolytic anemia, acute kidney injury, pulmonary hemorrhage with respiratory failure, and hypertension who had a complete remission following long-term (30 months) therapeutic plasma exchange. Case presentation: A 15-year-old Sri Lankan girl was transferred from a local hospital with the features of septicemia and acute kidney injury for specialized management. She had high blood pressure (180/100 mmHg) on admission. She underwent appendicectomy based on suspicion of acute appendicitis as the cause of sepsis. Following surgery, her condition deteriorated, and intensive care unit management was warranted because she developed pulmonary hemorrhages and respiratory failure requiring mechanical ventilation and renal replacement therapy in the form of hemodialysis. Her blood investigations showed microangiopathic hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase, and reduced human complement C3 levels, together with a normal coagulation profile. She was diagnosed with atypical hemolytic uremic syndrome and was initiated on therapeutic plasma exchange and other supportive therapy, including corticosteroids. Following a lengthy course of plasma exchange, complete recovery was achieved. Conclusion: The atypical hemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose. It is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical hemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy.
Background: Red cell transfusion is indicated to increase the oxygen delivering capacity and it cannot be replaced by any pharmacological agent. Although blood transfusion is generally safe, adverse effects can occur; hence, benefits must always overweigh risks of transfusion before decision to transfuse is made. Information about the aims of transfusion, benefits and risks of transfusion, must be explained to the patient and written consent must be obtained.Objective: To assess the awareness about blood transfusion among red cell recipients. Materials and Methods:This prospective descriptive study was carried out at a tertiary care hospital in Sri Lanka. Red cell recipients were questioned about the transfusion episode after been transfused.Results: There were 252 patients in the study, 82 from surgical units and 170 from medical units. 37 (45.12%) patients from surgical units and 79 (46.47%) patients from medical units were explained about the transfusion, before they were being transfused. 66% of patients from surgical units and 55% from medical wards knew their blood group. 77% and 57% of patients were aware about the reason for transfusion in surgical and medical wards, respectively. Moreover, 39% of patients from surgical units and 35% of patients from medical units knew about transfusion transmitted infections.Conclusions: Awareness about the own blood group, reason for transfusion, benefits and risks of transfusion was significantly low in the study group. Therefore, patient education should be improved to understand the benefits and risks to the patient.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.