Mortality Rates and autopsy findings in fat embolism syndrome complicating sickle cell disease.

Fat embolism syndrome is a rare but underdiagnosed complication of sickle cell disease associated with high morbidity and mortality. It affects predominantly patients with a previously mild course of their illness and those of non-SS genotypes while there is possibly an association with infection with human parvovirus B19 (HPV B19). Here, we present the mortality rates and autopsy findings of all reported cases to date. A systematic review has revealed 99 published cases in the world literature with a mortality rate of 46%. Mortality varied greatly according to the time of reported cases with no survivors in the 1940s, 1950s or 1960s and no deaths since 2020. 35% of cases had previously undiagnosed sickle cell disease and the latter was only identified at autopsy after developing fat embolism with a fatal outcome. 20% of cases reported after 1986 tested positive for HPV B19 with an associated mortality of 63% whereas in cases that have not documented HPV B19 infection the mortality was 32%. The organs most often staining positive for fat were the kidneys, lungs, brain and heart whereas ectopic haematopoietic tissue was found in 45% of the examined lung specimens.

Rate of Dental Extractions in Patients with Sickle Cell Disease.

BACKGROUND: Sickle cell disease is an inherited disorder associated with chronic haemolysis and anaemia, recurrent episodes of pain and potentially multisystem end-organ damage. A lot less is known about the dental health of these patients. AIMS: To explore the incidence of severe dental disease leading to dental extraction in our sickle cell population. PATIENT/METHODS: We undertook an audit looking at the rate of dental extractions, as a composite marker of severe dental disease, among sickle cell patients over a 3-month period. The patients were unselected and approached during routine assessments. We analysed both clinical and laboratory data to look for possible associations between dental disease and sickle cell characteristics. RESULTS: 177 patients were interviewed between February 2022 and April 2022. Overall, 71% of the patients had at least one dental extraction with a median number of teeth extracted of three and a median age at first extraction of 26. More than half of the patients stated that they do not have regular dental check-ups. There were no significant associations with the severity of sickle cell phenotype, baseline Hb or markers of haemolysis. CONCLUSION: A large number of patients with sickle cell disease require dental extractions at a relatively young age. The lack of any correlation with disease severity suggests that poor engagement with dental services and the underestimation of the importance of dental health are the main factors behind the increased prevalence of severe dental disease. Actively enquiring about dental problems should be part of any routine consultation with these patients, both in primary and specialist care.