Acute hepatic encephalopathy and multiorgan failure in sickle cell disease and COVID‐19

Martone, Giulia M.; Nanjireddy, Priyanka M.; Craig, Robin; Prout, Andrew; Higman, Meghan A.; Kelly, Kara M.; Ambrusko, Steven J.

doi:10.1002/pbc.28874

Cited by 5 publications

(6 citation statements)

References 19 publications

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“…Pulmonary embolism complicated condition. n/a 0 (0) Martone et al [ 87 ] USA 1 (0/1) 19 HbSβ 0 BT (1) PT developed ACS with fever, decreased hemoglobin, but no hypoxemia, which was managed with BT and antibiotics. Four weeks later, pt.…”

Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

“…In the general population, children are less likely to experience severe COVID-19 outcomes as compared to adults [ 76 ], but only few studies [ 29 , 72 ] have examined children with SCD and SARS-CoV-2 infection in sufficiently large cohorts from which valid inference can be drawn, as the vast majority of studies were case reports with less than 10 patients [ 67 , 68 , 75 , [77] , [78] , [79] , [80] , [81] , [82] , [83] , [84] , [85] , [86] , [87] , [88] , [89] , [90] , [91] , [92] ].…”

Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

“…It was hypothesized that activation of the type I Interferon (IFN-I) pathway may have contributed to partial protection from SARS-CoV-2 severe illness and death seen in their pediatric sample. Among the smaller pediatric studies [ 67 , 68 , 75 , [77] , [78] , [79] , [80] , [81] , [82] , [83] , [84] , [85] , [86] , [87] , [88] , [89] , [90] , [91] , [92] ] that reported on a combined 42 children with SCD, there was 1 (2.4%) patient with a history of prior pulmonary and cardiac complications who died from COVID-19 disease.…”

Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

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Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature

et al. 2022

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Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

Section: Patients With Covid-19 and Sickle Cell Diseasementioning

confidence: 99%

See 1 more Smart Citation

Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature

et al. 2022

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“…35 Notwithstanding the variable outcomes reported, COVID-19 infection has been associated with severe disease in adult and pediatric patients with SCD. 31,32 VOE, 36 pulmonary embolism, 21 ACS, 19,22,37 splenic sequestration, 38 and acute hepatic encephalopathy with multiorgan system failure, 39 respiratory failure, intubation, and death have been all reported, 40,41 therefore, early and aggressive treatment is warranted.…”

Section: Discussionmentioning

confidence: 99%

SARS-CoV-2 Infection Presenting as Acute Chest Syndrome in a Child With Hemoglobin SD-Los Angeles Disease: A Case Report and Review of Literature

Calderwood

Sabir

Rao

et al. 2022

Journal of Pediatric Hematology/Oncology

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Hemoglobin D-Los Angeles is a variant of hemoglobin that can polymerize in the deoxygenated state. When co-inherited with Hemoglobin S (HbSD-Los Angeles disease) a severe sickling syndrome similar to HbSS can result. Corona virus infectious disease 2019 (COVID-19) is caused by the severe acute respiratory syndromecorona virus-2. It has been associated with acute chest syndrome (ACS) in individuals with sickle cell disease (SCD), but this complication has not previously been reported in patients with HbSD-Los Angeles. Dexamethasone has been shown to improve outcomes in non-SCD patients with severe acute respiratory syndrome-corona virus-2 pneumonia or acute respiratory distress syndrome; however, its use in SCD patients with ACS is controversial due to a reported increased risk of complications including vaso-occlusive painful episodes. Herein, we reported a patient with HbSD-Los Angeles and COVID-19-associated ACS whom we treated with dexamethasone without transfusion. The patient experienced a rapid recovery without sequelae from steroid use.To further evaluate the use of steroids, we conducted a literature review focusing on the management of pediatric SCD patients with COVID-19-associated ACS. We identified a total of 39 pediatric patients with SCD and COVID-19, of whom 21 (54%) had ACS. Packed red blood cell transfusion (n = 11), exchange transfusion (n = 4), or a combination of exchange transfusion and packed red blood cell transfusion (n = 4) were the most frequently reported treatment, with hydroxychloroquine (n = 5), remdesivir (n = 1), and tocilizumab (n = 1) also being reported. Three patients were treated with dexamethasone. All patients recovered and no adverse outcomes from steroid use were reported. Even though transfusion is considered the standard of care for children with ACS and steroids are not routinely recommended, our experience suggested that COVID-19-associated ACS may be an important exception, especially for patients who refuse transfusion or are in resourcepoor nations where blood transfusions may not be readily available. Further studies are warranted to confirm these observations.

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“…The drug is used parentally for treatment of chronic iron overload occurs due to chronic blood transfusions used in treatment of B-thalassemia, sickle cell anemia and myelodysplastic syndrome [11]. The mechanisms of Deferasirox-induced hepatotoxicity may involve production of reactive intermediates by cytochrome P450, mitochondrial injury, and disruption of calcium homeostasis, stimulation of autoimmunity, canalicular injury and apoptosis [12].…”

Section: Introductionmentioning

confidence: 99%

The Natural Antioxidants Curcumin and Silymarin Afforded Hepatoprotective Effects against Deferasirox-Induced Hepatotoxicity

Osman

Abo-Elnasr

Zaher

et al. 2021

Preprint

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Deferasirox belongs to a new is a bishydroxyphenyltriazoles iron chelator used for treatment of chronic iron overload. The use of Deferasirox is associated with hepatotoxicity. Silymarin is a benzo gamma-pyrones flavonoid which affords hepatoprotection and preserves hepatocyte membranes by its antioxidant effect. Curcumin is a poly-phenol compound naturally concentrated in the herb Curcuma longa. The present study was conducted to evaluate and compare the hepatoprotective effects of both silymarin and curcumin against deferasirox-induced hepatotoxicity in rats and to explore the potential mechanisms account for their hepatoprotective effects. The present study was carried out using 32 male wistar randomly assigned into 4 groups (8 rats each) as follows; Group1: served as normal control group in which rats received distilled water (0.5ml per rat) by oral gavage. Group II (Deferasirox group) in which hepatotoxicity was induced by oral administration of deferasirox in a dose of 100 mg/kg once daily for 4 weeks dissolved in distilled water for a concentration of 35mg/ml. Group III (deferasirox+ curcumin) in which rats received curcumin in a dose of 100 mg/kg daily dose by oral gavage for 4 weeks suspended in distilled water for a concentration of 35 mg/ml one hour before administration of deferasirox. Group IV (deferasirox+ silymarin) in which rats received silymarin in dose of 7.56 mg/kg once daily by oral gavage for 4 weeks suspended in distilled water for a concentration of 2.7mg/ml one hour before administration of deferasirox. At the end of the treatment period, all rats were sacrificed by cervical dislocation, blood samples were collected for measurement of ALT, AST, ALP and total bilirubin. Liver samples were used for measurement of MDA, GSH and IL-6. Histopathological and immunohistochemistry were also done. The present data revealed that rats pretreated with curcumin or silymarin exhibited significant reduction in ALT, AST, ALP, total bilirubin, IL-6 and MDA levels with significant elevation of GSH level compared to deferasirox group. In-between group comparison revealed that there was a more significant reduction in ALT, AST, ALP, total bilirubin, IL-6 and MDA in group IV compared to group III. In conclusion, both curcumin and silymarin represent natural protective agents against Deferasirox-induced hepatotoxicity due to their antioxidant and anti-inflammatory effects.

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Acute hepatic encephalopathy and multiorgan failure in sickle cell disease and COVID‐19

Cited by 5 publications

References 19 publications

Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature

Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature

SARS-CoV-2 Infection Presenting as Acute Chest Syndrome in a Child With Hemoglobin SD-Los Angeles Disease: A Case Report and Review of Literature

The Natural Antioxidants Curcumin and Silymarin Afforded Hepatoprotective Effects against Deferasirox-Induced Hepatotoxicity

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