scholarly journals Risk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease

2015 ◽  
Vol 172 (2) ◽  
pp. 274-284 ◽  
Author(s):  
Veronica van der Land ◽  
Henri J. M. M. Mutsaerts ◽  
Marc Engelen ◽  
Harriët Heijboer ◽  
Mark Roest ◽  
...  
Keyword(s):  
Factor Analysis ◽  
Risk Factor ◽  
White Matter ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
White Matter Hyperintensities ◽  
Risk Factor Analysis ◽  
Cerebral White Matter ◽  
Cell Disease

scholarly journals Longitudinal evaluation of cerebral white matter hyperintensities lesion volume in children with sickle cell disease

2016 ◽  
Vol 176 (3) ◽  
pp. 485-487 ◽  
Author(s):  
Renzo Manara ◽  
Giacomo Talenti ◽  
Patrizia Rampazzo ◽  
Mario Ermani ◽  
Maria Montanaro ◽  
...  
Keyword(s):  
White Matter ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
White Matter Hyperintensities ◽  
Cerebral White Matter ◽  
Lesion Volume ◽  
Cell Disease ◽  
Longitudinal Evaluation

scholarly journals Volume of white matter hyperintensities is an independent predictor of intelligence quotient and processing speed in children with sickle cell disease

2014 ◽  
Vol 168 (4) ◽  
pp. 553-556 ◽  
Author(s):  
Veronica van der Land ◽  
Channa T. Hijmans ◽  
Marieke de Ruiter ◽  
Henri J. M. M. Mutsaerts ◽  
Marjon H. Cnossen ◽  
...  
Keyword(s):  
White Matter ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Processing Speed ◽  
Intelligence Quotient ◽  
White Matter Hyperintensities ◽  
Independent Predictor ◽  
Cell Disease

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.

Sickle Cell Disease Is a Risk Factor for Obstructive Sleep Apnea

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 322A
Author(s):  
JoAnn Eng ◽  
George A. Apergis ◽  
Samir Fahmy
Keyword(s):  
Obstructive Sleep Apnea ◽  
Risk Factor ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

Hydroxyurea Use Is Associated with Avascular Necrosis of the Femoral Head among Children with Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2477-2477 ◽  
Author(s):  
Kris Michael Mahadeo ◽  
Suzette Oyeku ◽  
Karen Moody ◽  
Swapmil N. Rajpathak ◽  
Abraham Groner ◽  
...  
Keyword(s):  
Risk Factor ◽  
Femoral Head ◽  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Demographic Characteristics ◽  
Cell Disease ◽  
Health Maintenance ◽  
Laboratory Values

Abstract Hydroxyurea therapy is associated with reduced morbidity among patients with sickle cell disease (SCD). Avascular necrosis of the femoral head (AVN) is one potentially debilitating complication of SCD. In this study, we examined the relationship between hydroxyurea use and the prevalence of AVN among children with SCD. We performed a retrospective chart review of 202 children with SCD, aged 10–21 years, followed in the pediatric hematology program at the Children’s Hospital at Montefiore (Bronx, NY) between July 2007 and 2008. Abstracted data included age, ethnicity, SCD genotype, frequency of hospitalization, hip radiograph results, laboratory data and hydroxyurea use. Hip radiographs were performed prospectively as part of SCD health maintenance from 2005–2008. Forty-four patients were excluded because they did not have a screening hip radiograph. Descriptive statistics were calculated for independent variables. T-tests and chi-square tests were used to compare clinical and demographic characteristics of children with and without AVN. Multivariate logistic regressions were used to estimate the odds ratio of having AVN among SCD patients. Our final sample consisted of 158 patients whose demographic characteristics are listed in Table 1. The prevalence of AVN was 16.5% (n=26). Of the clinical variables analyzed, we identified significant associations between the presence of AVN and hydroxyurea use (p=.005), as well as older age (p=.013) (Table 1.) Children with AVN had significantly lower mean lactic dehydrogenase levels (LDH) (p=.04) and higher mean corpuscular volumes (MCV) (p=.012). (Table 2.) After controlling for gender, ethnicity, sickle cell genotype, and frequency of hospitalizations, age was also found to be associated with AVN (OR 1.15, 95% confidence interval (CI): 1.01,1.31, p=0.033). SCD patients on hydroxyurea had higher odds of having AVN compared to non-users (OR 3.51, 95% CI: 1.31, 9.38, p= 0.013). Laboratory values (MCV, Hemoglobin, LDH and Hematocrit) had a high degree of collinearity and were removed from the final model. In summary, the prevalence of AVN in our sample was 16.5%. This is substantially higher than the prevalence of approximately 6% reported by the Cooperative Study of Sickle Cell Disease for comparative age groups in a prospective study1. SCD patients exposed to hydroxyurea were three times more likely to have AVN than those not exposed to this drug. Vaso-occlusive pain crisis is a recognized risk factor for AVN, thus we could expect a higher rate of AVN among patients on hydroxyurea. However, the odds ratio of 3.5 is unexpectedly high and warrants further investigation into the role of hydroxyurea as a risk factor for AVN. Nonetheless, these preliminary results suggest that more stringent screening regimens for AVN may be indicated among this subset of patients. Table 1. Clinical characteristics of patients with and without avn *p<0.05 **p<0.01 No AVN (N =132) AVN (N = 26) Age * 15.7 years 17.4 years Sex Male 64 (49%) 17 (65%) Ethnicity Black 110 (83%) 23 (88%) Hispanic 22 (17%) 3 (12%) HgbSS 84 (64%) 20 (77%) HgbSC 38 (29%) 4 (15%) HgbSBthal0 5(3.8%) 2 (8%) Hgb SC HgbSBthal+ 5 (3.8%) 0 On Hydroxyurea** 38 (29%) 15 (58%) # Hospitalizations 0 60 (45%) 10 (38%) 1–5 64 (49%) 14 (54%) >5 8 (6%) 2 (8%) Table 2. Mean Laboratory Values for Patients With And Without AVN No AVN AVN *p<0.05 (N =132) (N = 26) WBC 10.7 k/uL 10.5 k/uL Hgb 9.4 gm/dL 9.6 gm/dL MCV* 83 fL 89 fL Platelets 381 k/uL 376 k/uL Reticulocyte 7.70% 8.10% Ferritin 369.8 ng/mL 438.7 ng/mL LDH* 471.6 U/L 389 U/L Creatinine 0.6 mg/dL 0.6 mg/dL Hgb F 9.80% 11.30%

scholarly journals Disseminated Mycobacterium avium infection in a young woman with sickle cell disease and iatrogenic iron overload

2021 ◽  
Vol 8 (2) ◽  
pp. 5
Author(s):  
Pouria Hosseini ◽  
Yogamaya Mantha ◽  
Shannon J Koh ◽  
Gebre K Tseggay ◽  
Jyothi K Baby ◽  
...  
Keyword(s):  
Risk Factor ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Case Reports ◽  
Recurrent Pain ◽  
Cell Disease ◽  
Increased Risk ◽  
Avium Infection ◽  
Immunosuppressed Patients

A 29-year-old woman with known sickle cell disease (SCD) and iatrogenic iron overload presented to the emergency department with a recurrent pain crisis and fever. Blood cultures obtained at a recent prior admission for the same complaints grew M avium. Bone marrow biopsy revealed non-caseating granulomas, but stains for mycobacteria and fungi were negative. Disseminated non-tuberculous mycobacterial infections (NTMIs) occur almost exclusively in immunosuppressed patients. SCD is not considered a risk factor for the development of disseminated NTMIs, making diagnosis challenging in this population. However, a number of case reports describing disseminated NTMIs in patients with SCD have been published. This case adds to the current literature, suggesting SCD with iatrogenic iron overload is a possible risk factor for disseminated NTMIs. Potential mechanisms for this increased risk include 1) functional asplenia, 2) iatrogenic iron overload, 3) chronic indwelling central venous catheters, and 4) hydroxyurea use. Further investigation is required to describe the strength and mechanism of the relationship between SCD and disseminated NTMIs.

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