Hedvig Björkman, Marcus Fager Ferrari, Alexandros Arvanitakis, Christian Kjellander
{"title":"[Sickle cell disease - common and dangerous complications].","authors":"Hedvig Björkman, Marcus Fager Ferrari, Alexandros Arvanitakis, Christian Kjellander","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Sickle cell disease is a genetic disorder affecting hundreds of thousands of people worldwide. This article will focus on the most common and dangerous acute complications to sickle cell disease. Vaso-occlusive crisis is the most common manifestation of sickle cell disease. Rapid pain relief and treating the underlying cause are cornerstones of the treatment. Acute chest syndrome is the most common cause for intensive care unit admission and death among adult patients with sickle cell disease. The condition is treated as vaso-occlusive crises, but patients often need blood transfusions and respiratory support. Patients with sickle cell disease have an increased risk of stroke. Treatment follows the usual guidelines for stroke with the addition of blood- or exchange transfusions. Splenic sequestration is a rare but acute and life-threatening complication, most commonly seen in children. The condition can quickly lead to hypovolemic shock and the treatment aims to maintain adequate circulation and to treat the underlying cause.</p>","PeriodicalId":17988,"journal":{"name":"Lakartidningen","volume":"121 ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Lakartidningen","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Sickle cell disease is a genetic disorder affecting hundreds of thousands of people worldwide. This article will focus on the most common and dangerous acute complications to sickle cell disease. Vaso-occlusive crisis is the most common manifestation of sickle cell disease. Rapid pain relief and treating the underlying cause are cornerstones of the treatment. Acute chest syndrome is the most common cause for intensive care unit admission and death among adult patients with sickle cell disease. The condition is treated as vaso-occlusive crises, but patients often need blood transfusions and respiratory support. Patients with sickle cell disease have an increased risk of stroke. Treatment follows the usual guidelines for stroke with the addition of blood- or exchange transfusions. Splenic sequestration is a rare but acute and life-threatening complication, most commonly seen in children. The condition can quickly lead to hypovolemic shock and the treatment aims to maintain adequate circulation and to treat the underlying cause.