scholarly journals The use of partial exchange blood transfusion and anaesthesia in the management of sickle cell disease in a perioperative setting: two case reports

2010 ◽  
Vol 4 (1) ◽  
Author(s):  
Rhett Jaeckel ◽  
Matthias Thieme ◽  
Elke Czeslick ◽  
Armin Sablotzki
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Case Reports ◽  
Cell Disease ◽  
Exchange Blood Transfusion

scholarly journals A Five Year Experience of Acute Intrahepatic Cholestasis in Patients with Sickle Cell Disease at a Large Teaching Hospital in London

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3416-3416
Author(s):  
Anne Nkirote Mwirigi ◽  
Chengetai Muzah ◽  
Liz Odeh ◽  
Abid Suddle ◽  
Swee Lay Thein ◽  
...  
Keyword(s):  
Liver Disease ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Autoimmune Hepatitis ◽  
Sickle Cell ◽  
Intrahepatic Cholestasis ◽  
Liver Imaging ◽  
Cell Disease ◽  
Radiological Features ◽  
Exchange Blood Transfusion

Abstract Background: Acute intrahepatic cholestasis (AIC) is a rare but severe form of sickle hepatopathy, caused by sickling within hepatic sinusoids, leading to vascular stasis, hypoxic damage and can lead to acute hepatic failure. It is characterised by a combination of clinical, laboratory and radiological features. There is tender hepatomegaly, hyperbilirubinaemia without evidence of extrahepatic biliary obstruction, coagulopathy and thrombocytopenia. Transaminitis, if present is mild or moderate. AIC has a high fatality rate if not treated promptly. Although there is a dearth of evidence on how to treat AIC, the most frequently recommended intervention is by exchange blood transfusion. We report our experience of managing patients with this rare yet serious complication of sickle cell disease at a large tertiary centre over the course of 5 years. Methods: A retrospective review of adult patients who attended the sickle cell service from 1st January 2010 to 30th June 2015 was undertaken. All patients who had had a bilirubin level greater than 300umol/L were identified. Patients with evidence of 'secondary' causes of hyperbilirubinaemia such as biliary sepsis, acute biliary or extrahepatic duct dilatation were excluded. For the remaining patients, clinical notes were reviewed for history of tender right upper quadrant, peak bilirubin levels, coagulation studies, ferritin, ultrasonography or other liver imaging, pre-existing co-morbidities and immediate and long-term clinical outcome were recorded. Results: We identified 16 cases of AIC in 15 patients, out of an excess of 582 patients who attended the Sickle Cell Department over the course of 5 years. In three of these cases, there was also an associated generalised vaso-occlusive crisis. There were 10 male and five female, all of whom had sickle cell anaemia (HbSS), with a median age of 30years (20-48 years). In terms of pre-existing liver disease, one patient had autoimmune hepatitis; another two had chronically deranged liver functions with radiological features suggestive of cirrhosis.Table 1.AgeSexPeak bilirubin; conjugated%INRAPTRThrombocytopeniaConcurrent crisis (VOC)Outcome25F398; 65%1.281.17NoNoAlive25F386; 61%1.221.97NoNoAlive36M299; 69%1.281.33NoYesAlive20M299; 63%1.491.22NoNoAlive37M693; 74%1.371.54NoNoAlive38F671; 100%1.211.41YesNoAlive25F686; 83%1.071.53YesNoAlive25M585; 78%1.251.14NoNoAlive34M450; 88%1.121.58YesNoAlive30M694; 96%2.593.77YesNoDeceased48M719; 66%3.002.33YesNoDeceased22M975; 98%4.0>5NoNoDeceased25F310; N/A1.472.06YesYesAlive25M1043; 99%3.43>5YesNoDeceased44F478; 100%1.331.40NoNoAlive46M644; 91%2.081.2YesNoDeceased The findings on ultrasonography included increased liver reflectivity, hepatomegaly and in two cases, cirrhosis. Choledolithiasis was present in 6 cases, with no evidence of gall bladder infection, inflammation or duct obstruction. Cholecystectomy had been performed in 3 cases; two of these patients had chronically dilated intrahepatic ducts. All patients received transfusions to lower the Haemoglobin S% to less than 30%. Eleven patients received exchange blood transfusions within 72hours of presentation. The remainder, who had received transfusion recently (majority at local hospital) received further transfusions within a week of admission to keep HbS% <20%. Seven of the 11 patients who recovered now have normal transaminases with mildly elevated bilirubin levels. Three patients have markedly elevated transaminases and bilirubinaemia, two of whom had previous cholecystectomy and chronically dilated intrahepatic ducts. The third has a heterogenous liver with features of sickle hepatopathy and probable early features of liver fibrosis, while the fourth went on to have a successful liver transplant. There were 5 deaths; all in male patients aged between 22 and 48 years. Two had established cirrhosis; one also had autoimmune hepatitis and CMV viraemia. A third patient had liver biopsy evidence of fibrosis, while a fourth had a CT description of liver with a nodular contour. The fifth patient,was previously fit and well and adenoviraemia was diagnosed. Discussion: Our experience supports the view that AIC can successfully be reversed by timely exchange blood transfusion, and suggests that underlying liver disease and viraemia confer a poor outcome, irrespective of age. Disclosures No relevant conflicts of interest to declare.

scholarly journals Outcomes following kidney transplantation in patients with sickle cell disease: The impact of automated exchange blood transfusion

PLoS ONE ◽  
2020 ◽  
Vol 15 (8) ◽  
pp. e0236998
Author(s):  
Joanna C. Willis ◽  
Moji Awogbade ◽  
Jo Howard ◽  
Cormac Breen ◽  
Allifia Abbas ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cell Disease ◽  
Exchange Blood Transfusion ◽  
The Impact

Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
J. Michael Taylor ◽  
Paul Horn ◽  
Heidi Sucharew ◽  
Todd A Abruzzo ◽  
Jane Khoury
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Care Utilization ◽  
Cell Disease ◽  
Common Procedure ◽  
Transfusion Therapy ◽  
Stroke Population ◽  
Clinical Classification Software ◽  
Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

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