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Paediatric cardiac anaesthesia in sickle cell disease : a case series
Sickle cell disease (SCD) is the most common inherited haematological disorder, producing a mutation of the haemoglobin molecule known as haemoglobin S (HbS). The presence of HbS in the erythrocyte makes it prone to sickling - a process which may lead to vaso-occlusive injury, haemolysis and a hyper...
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| Format: | Thesis |
| Language: | English |
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Department of Anaesthesia
2015
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| _version_ | 1867614456273436672 |
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| access_status_str | Open Access |
| author | Janse van Rensburg, P J |
| author2 | Thomas, Jenny |
| author_browse | Janse van Rensburg, P J Thomas, Jenny |
| author_facet | Thomas, Jenny Janse van Rensburg, P J |
| author_sort | Janse van Rensburg, P J |
| collection | Thesis |
| description | Sickle cell disease (SCD) is the most common inherited haematological disorder, producing a mutation of the haemoglobin molecule known as haemoglobin S (HbS). The presence of HbS in the erythrocyte makes it prone to sickling - a process which may lead to vaso-occlusive injury, haemolysis and a hypercoagulable state. Sickling is precipitated by dehydration, hypoxia, hypothermia, acidosis and low flow states. Over time, multi-organ damage develops with significant morbidity and mortality. Paediatric patients with SCD and congenital heart defects may require anaesthesia for corrective cardiac surgery on cardiopulmonary bypass (CPB). During the perioperative period these high-risk patients may suffer significant complications when exposed to the conditions that favour erythrocyte sickling. This case series details our experience of four paediatric patients with SCD patients who underwent corrective cardiac surgery at Red Cross War Memorial Children’s Hospital. The pathophysiology is discussed and the perioperative management of transfusion, cardiopulmonary bypass and temperature regulation is highlighted. |
| format | Thesis |
| id | oai:open.uct.ac.za:11427/13790 |
| institution | University of Cape Town (South Africa) |
| language | eng |
| last_indexed | 2026-06-10T12:52:19.896Z |
| license_str | Not specified — see source repository |
| provenance_str_mv | Harvested via OAI-PMH from UCTD — University of Cape Town Open Access Repository |
| publishDate | 2015 |
| publishDateRange | 2015 |
| publishDateSort | 2015 |
| publisher | Department of Anaesthesia |
| publisherStr | Department of Anaesthesia |
| record_format | dspace |
| source_str | UCTD — University of Cape Town Open Access Repository |
| spelling | oai:open.uct.ac.za:11427/13790 Paediatric cardiac anaesthesia in sickle cell disease : a case series Janse van Rensburg, P J Thomas, Jenny Anaesthesia Sickle cell disease (SCD) is the most common inherited haematological disorder, producing a mutation of the haemoglobin molecule known as haemoglobin S (HbS). The presence of HbS in the erythrocyte makes it prone to sickling - a process which may lead to vaso-occlusive injury, haemolysis and a hypercoagulable state. Sickling is precipitated by dehydration, hypoxia, hypothermia, acidosis and low flow states. Over time, multi-organ damage develops with significant morbidity and mortality. Paediatric patients with SCD and congenital heart defects may require anaesthesia for corrective cardiac surgery on cardiopulmonary bypass (CPB). During the perioperative period these high-risk patients may suffer significant complications when exposed to the conditions that favour erythrocyte sickling. This case series details our experience of four paediatric patients with SCD patients who underwent corrective cardiac surgery at Red Cross War Memorial Children’s Hospital. The pathophysiology is discussed and the perioperative management of transfusion, cardiopulmonary bypass and temperature regulation is highlighted. 2015-08-27T12:31:44Z 2015-08-27T12:31:44Z 2015 Master Thesis Masters MMed http://hdl.handle.net/11427/13790 eng application/pdf Department of Anaesthesia Faculty of Health Sciences University of Cape Town |
| spellingShingle | Anaesthesia Janse van Rensburg, P J Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| thesis_degree_str | Master's |
| title | Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| title_full | Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| title_fullStr | Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| title_full_unstemmed | Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| title_short | Paediatric cardiac anaesthesia in sickle cell disease : a case series |
| title_sort | paediatric cardiac anaesthesia in sickle cell disease a case series |
| topic | Anaesthesia |
| url | http://hdl.handle.net/11427/13790 |
| work_keys_str_mv | AT jansevanrensburgpj paediatriccardiacanaesthesiainsicklecelldiseaseacaseseries |