scholarly journals Acute kidney injury during parvovirus B19-induced transient aplastic crisis in sickle cell disease

2018 ◽  
Vol 93 (8) ◽  
pp. E198-E200 ◽  
Author(s):  
Jamie Oakley ◽  
Rima Zahr ◽  
Inmaculada Aban ◽  
Varsha Kulkarni ◽  
Rakesh P. Patel ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Kidney Injury ◽  
Cell Disease ◽  
Aplastic Crisis ◽  
Transient Aplastic Crisis

scholarly journals Hemopexin deficiency promotes acute kidney injury in sickle cell disease

Blood ◽  
2020 ◽  
Author(s):  
Solomon Ofori-Acquah ◽  
Rimi Hazra ◽  
Oluwaseun O Orikogbo ◽  
Danielle Crosby ◽  
Bethany Flage ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Kidney Injury ◽  
Risk Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Inflammatory Process ◽  
Clinical Evidence ◽  
Kidney Injury ◽  
Red Cell ◽  
Cell Disease

Acute kidney injury (AKI) is a major clinical concern in sickle cell disease (SCD). Clinical evidence suggests that red cell alarmins may cause AKI in SCD however the sterile inflammatory process involved has hitherto not been defined. We discovered that hemopexin deficiency in SCD is associated with a compensatory increase in alpha-1-microglobulin (A1M) resulting in up to 10-fold higher A1M/hemopexin ratio in SCD compared to health controls. The A1M/hemopexin ratio is associated with markers of hemolysis and AKI in both humans and mice with SCD. Studies in mice showed that excess heme is directed to the kidneys in SCD in a process involving A1M causing AKI while excess heme in controls is transported to the liver as expected. Using genetic and bone marrow chimeric tools, we confirmed that hemopexin deficiency promotes AKI in sickle mice under hemolytic stress. However, AKI was blocked when hemopexin deficiency in sickle mice was corrected with infusions of purified hemopexin prior to the induction of hemolytic stress. This study identifies acquired hemopexin deficiency as a risk factor of AKI in SCD and hemopexin replacement as a potential therapy.

Myocardial Ischemia in Patients with Sickle Cell Disease: A Retrospective Review

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2189-2189
Author(s):  
Payal C. Desai ◽  
Nicole Kendel ◽  
Spero R Cataland ◽  
Eric H. Kraut ◽  
Ying Huang ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Chest Pain ◽  
Sickle Cell Disease ◽  
Myocardial Ischemia ◽  
Sickle Cell ◽  
Myocardial Injury ◽  
Kidney Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Troponin Elevation

Abstract Introduction: On an autopsy study of 306 patients with sickle cell disease (SCD), cardiovascular disease is observed in 58% of patients and myocardial microinfarcts were noted in 20% of patients. Previously data from our center indicates that approximately 13% (3/22) patients demonstrated cardiac microvascular disease in steady state. However, the incidence of myocardial ischemic injury in patients with SCD presenting with chest pain remains largely undefined. Methods: We conducted a single institution retrospective chart review from September 2009 through September 2014 to evaluate the incidence of elevated troponin-I (normal < 0.11ng/ml), which is a well-established biomarker of myocardial injury, in patients with SCD and chest pain. We further characterize each of these episodes with cardiac magnetic resonance imaging, if available, and clinical and laboratory findings at the time of the event. Kruskal-Wallis test was used to compare troponin measurement values among different groups of patients. Results: A total of 25 (10 female, 15 male) of the 352 (7%) of patients followed at the Ohio State Comprehensive Sickle Cell Center had troponin elevation over a 5 year period. They had a total of thirty-eight individual encounters with troponin elevations (range: 0.11-12.17) [72% patients with 1 elevation, 28% patients multiple troponin elevations) (range: 1-4 incidences)]. The median age at the time of troponin elevation was 36 (Range: 20.5-66 yrs). Troponin elevation was observed in patients of all genotypes (76% SS; 4% SBeta+; 20% SC). 6/25 (25%) of patients with troponin elevation in the past five years are now deceased. Thirteen patients (52%) had acute chest syndrome and ten patients (40%) had acute kidney injury at the time of troponin elevation. The degree of troponin elevation was not associated with concurrent acute chest syndrome, concurrent acute kidney injury or mortality. At the encounter level, median troponin at diagnosis was 0.3 (range: 0.11-12.2) and the median peak troponin was 0.4 (range: 0.11-38.1),. The median value of TR jet velocity at baseline was 2.8 (range: 1.1-3.7) (n=23) and the median TR jet velocity at the time of elevation was 3.0 (range: 1.7-4.6) (n=28). Median baseline hemoglobin was 8 (range: 4.5-12.6) and median hemoglobin at encounter was 7.5 (range: 4.3-12.5), resulting in a median change in hemoglobin of -0.4 (range: -6.2 - 2.5). Four of 10 MRI obtained at the time of troponin elevation showed myocardial ischemia and 3/10 patients showed late gadolinium enhancement indicating myocardial injury. Conclusion: Patients with SCD presenting with chest pain have myocardial ischemia and infarctions as demonstrated in our population by both troponin elevation and cardiac imaging. While, the long term implications of this finding are currently being studied in a multi-centered prospective study, further cardiac evaluations should be considered in patients with SCD presenting with chest pain. Disclosures Desai: Pfizer: Consultancy. Cataland:Ablynx: Consultancy. Raman:Siemens: Consultancy.

scholarly journals Acute kidney injury in children with sickle cell disease—compounding a chronic problem

2017 ◽  
Vol 32 (8) ◽  
pp. 1287-1291 ◽  
Author(s):  
Cherry Mammen ◽  
Mei Lin Bissonnette ◽  
Douglas G. Matsell
Keyword(s):  
Acute Kidney Injury ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Kidney Injury ◽  
Cell Disease ◽  
Chronic Problem

scholarly journals Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

2009 ◽  
Vol 53 (3) ◽  
pp. 479-481 ◽  
Author(s):  
Amber Mayfield Yates ◽  
Jane S. Hankins ◽  
Nicole A. Mortier ◽  
Banu Aygun ◽  
Russell E. Ware
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Aplastic Crisis ◽  
Transient Aplastic Crisis

scholarly journals Hemopexin Replacement Therapy Protects Sickle Cell Disease Mice from Acute Kidney Injury

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 78-78
Author(s):  
Samit Ghosh ◽  
Oluwaseun Orikogbo ◽  
Rimi Hazra ◽  
Bethany Flage ◽  
Danielle Crosby ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Kidney Injury ◽  
Sickle Cell Disease ◽  
Replacement Therapy ◽  
Sickle Cell ◽  
Bone Marrow Cells ◽  
Kidney Injury ◽  
Renal Tissue ◽  
Acute Chest Syndrome ◽  
Cell Disease

Acute kidney injury (AKI) is a major clinical concern during episodes of acute chest syndrome and vaso-occlusive crisis in sickle cell disease (SCD). AKI increases the risk of chronic kidney disease (CKD) and end stage renal disease (ESRD). We previously showed that alpha-1-microglobulin (A1M), a low affinity heme-binding protein that carries heme for renal clearance is elevated in patients and mice with homozygous SCD (SS). Hemopexin (Hx), which primarily scavenges circulating heme to the liver, is exhausted in SCD. In this study, we explored the idea that acquired Hx deficiency in SCD is a risk factor for AKI development in SCD. We studied mice with a global knockout of Hx with no detectable plasma Hx at baseline. Plasma A1M was significantly elevated in the Hx-/-mice compared to control (Hx+/+) mice (n=5; p&lt;0.01). We then transplanted whole bone marrow cells from SS mice into Hx+/+and Hx-/-mice to create bone marrow chimeric SSHx+/+and SSHx-/-mice with SCD phenotype. The chimeras had elevated plasma A1M compared to recipient littermates (Hx+/+and Hx-/-) and low baseline glomerular filtration rate (GFR). Modest elevation of circulating heme with infusion of hemin (20 μmoles/kg bw) worsened GFR and caused severe AKI. Next, to determine whether hemin induced AKI is attenuated by elevation of circulating Hx, we infused SS mice with purified Hx, and control SS mice with either purified A1M or vehicle immediately prior to the hemin challenge. We observed improved GFR in the Hx-treated SS mice, while vehicle and A1M-treated mice suffered 23% and 39% loss of GFR respectively compared to their baseline. In agreement with the GFR data, the levels of several AKI diagnostic markers, plasma creatinine (plasma Cr), urinary albumin-creatinine ratio (uACR) and kidney injury molecule (uKIM-1) were elevated significantly in vehicle and A1M treated mice following hemin challenge. In Hx-treated mice, these biomarkers remained unaltered. Importantly, Hx infusions re-directed excess heme in SS mice to the liver, while A1M infusion significantly increased total heme content in the kidneys. Two-way ANOVA analysis of GFR (p&lt;0.01) and plasma Cr (p&lt;0.001) revealed significant exacerbation of kidney injury in A1M treated SS mice compared to vehicle treated mice. Histopathology of renal tissue showed considerable tissue damage in vehicle and A1M infused SS mice, while Hx treated SS mice kidneys appeared relatively normal. This study provides genetic evidence that hemopexin deficiency promotes AKI development in SCD, and we provide proof-of-principle for hemopexin replacement therapy to treat AKI in SCD. Disclosures Ofori-Acquah: Shire Human Genetic Therapies Inc: Other: Financial Relationship.

scholarly journals Aplastic crisis caused by parvovirus B19 in an adult patient with sickle-cell disease

2000 ◽  
Vol 33 (5) ◽  
pp. 477-481 ◽  
Author(s):  
Sérgio Setúbal ◽  
Adelmo H.D. Gabriel ◽  
Jussara P. Nascimento ◽  
Solange A. Oliveira
Keyword(s):  
Sickle Cell Disease ◽  
Adult Patient ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Cell Disease ◽  
Aplastic Crisis

We describe a case of aplastic crisis caused by parvovirus B19 in an adult sickle-cell patient presenting with paleness, tiredness, fainting and dyspnea. The absence of reticulocytes lead to the diagnosis. Anti-B19 IgM and IgG were detected. Reticulocytopenia in patients with hereditary hemolytic anemia suggests B19 infection.

scholarly journals Acute kidney injury in paediatric patients with sickle cell disease is associated with increased morbidity and resource utilization

2020 ◽  
Vol 189 (3) ◽  
pp. 559-565
Author(s):  
Meghan McCormick ◽  
Troy Richardson ◽  
Bradley A. Warady ◽  
Enrico M. Novelli ◽  
Ramasubramanian Kalpatthi
Keyword(s):  
Acute Kidney Injury ◽  
Sickle Cell Disease ◽  
Resource Utilization ◽  
Sickle Cell ◽  
Kidney Injury ◽  
Cell Disease ◽  
Paediatric Patients
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