Sickle Cell Disease

Steinberg, Martin H.

doi:10.1017/cbo9780511596582.026

Cited by 6 publications

(8 citation statements)

References 756 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The environment of the renal medulla is characterized by hypoxia, acidosis, and hypertonicity that promote hemoglobin S polymerization and red cell sickling which ultimately leads to glomerular malfunction manifested initially as proteinuria and potentially progressing to chronic renal failure [14,15].…”

Section: Discussionmentioning

confidence: 99%

Circumstances of death in adult sickle cell disease patients

et al. 2006

View full text Add to dashboard Cite

The purpose of the study was to analyze clinical and/or autopsy findings at the time of death among adults with sickle cell disease (SCD) at Howard University in Washington, DC over a 25-year period. A single physician recorded circumstances of death among 141 adult SCD patients he treated and knew well from 1976 to 2001. These findings were determined by autopsy report and/or clinical assessment. In a subset of 31 patients, autopsy records were reviewed for reports of iron deposition in liver and heart and of organ pathology. One hundred and fourteen (80.9%) of the patients had SS phenotype and 66 (46.8%) were female. The mean ± SD age at death was 36 ± 11 years. Leading circumstances of death included pulmonary hypertension (PHT) (26.2%), sudden death (23.4%), renal failure (22.6%), infection (18.4%), thromboembolism (14.9%), cardiac diagnoses (12.0%), cirrhosis (11.3%), pneumonia or acute chest syndrome (9.9%), bleeding (7.8%), and iron overload (7.0%). When circumstances of deaths that occurred after 1991 (n = 69) were compared to those that occurred in 1991 or earlier (n = 72), PHT (36.2% vs. 16.6%; P < 0.01) was significantly more common in 1992 or later. Significant associations were found between PHT and thromboembolism and between cirrhosis and iron overload. In this proportional mortality study of adults with SCD, PHT was the leading finding at the time of death. Thromboembolism was associated with PHT, and iron overload was associated with cirrhosis. Am. J. Hematol. 81:858-863, 2006. V V C 2006 Wiley-Liss, Inc.

show abstract

Section: Discussionmentioning

confidence: 99%

Circumstances of death in adult sickle cell disease patients

et al. 2006

View full text Add to dashboard Cite

show abstract

“…Please see Table 1 for the different genotypes and their clinical laboratory measures. (Steinberg, 2011) Every type of SCD has at least 50% of Hb S (Steinberg, 2011). SCA and Hb S-β 0 have the highest percentage of Hb S and are considered the most clinically severe forms of SCD.…”

Section: Potential Disease-specific Influencing Factorsmentioning

confidence: 99%

“…Patients with SCA and Hb S-β 0 have the highest incidences of vaso-occulsive pain crises, acute chest syndrome and stroke (Steinberg, 2011). These patients are also more likely to receive blood transfusion and hydroxyurea therapy, the only approved medication for prevention of complications in SCD with multiple beneficial effects with patients with SCD (Quinn, 2013;Steinberg, 2011).…”

Section: Potential Disease-specific Influencing Factorsmentioning

confidence: 99%

See 1 more Smart Citation

Health Literacy in Adolescents With Sickle Cell Disease

Perry

Carter

Becker

et al. 2017

Journal of Pediatric Nursing

View full text Add to dashboard Cite

“…170 Also, all blacks and others are tested routinely for sickle cell trait at birth since 1987, following a recommendation from the National Institutes of Health. 171 Concerns have been raised regarding the NCAA screening program, including potential infringement on individual privacy and liberty and the effects of such testing on self-image and employability in professional sports. 170 As yet, there are no data that support sickle cell trait testing as lifesaving, although awareness has been raised regarding this potential problem in sports.…”

Section: Other Screening Initiativesmentioning

confidence: 99%