scholarly journals The Mortality of Adult Sickle Cell Patients at a Comprehensive Sickle Cell Center

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 971-971
Author(s):  
Jennifer A Afranie-Sakyi ◽  
Morgan L. McLemore ◽  
Fuad El Rassi
Keyword(s):  
Life Expectancy ◽  
Sickle Cell ◽  
Outpatient Visit ◽  
The United States ◽  
Early Mortality ◽  
Mortality Data ◽  
Acute Chest Syndrome ◽  
Genotype Distribution ◽  
Significant Difference ◽  
Age Of Death

Abstract Sickle cell disease (SCD) is the most common hemoglobinopathy affecting 100,000 people in the United States. Although without a national registry, the precise number remains an estimate and the mortality data remains unknown. The life expectancy of SCD has extended into adulthood with the advent of prophylactic penicillin use, antipneumococcal vaccines, newborn screenings, and use of hydroxyurea (HU). However as SCD patients age, comorbidities arise reducing the life expectancy. There is an evolving landscape regarding SCD therapies, with 3 FDA approved drugs since 2017 (L-glutamine, voxelotor, crizanlizumab) joining HU which was approved in 1998. It is paramount to understand the factors that lead to early mortality in SCD so that future therapies and cures can be accessible and benefit all SCD patients. We embarked a review of the mortality data in a healthcare system serving a significant number of SCD patients in Atlanta, GA. Methods: We conducted a retrospective chart review of the Georgia Comprehensive Sickle Cell Center at Grady Health system's electronic medical record for deceased sickle cell patients from 2013 to 2020. Patients were then grouped into 3 groups, those whom followed up at the Sickle Cell Center (defined as outpatient visit within 6 months prior to death-RF), those whom did not (defined as outpatient visit > 6 months-NF), and those with unknown status. Results: Overall, 72 patients were analyzed, with the following genotype distribution: SS n=49, SC n=14, Sbeta null thalassemia (Sβ0 thal) n=2, SBeta plus thalassemia (Sβ+ thal) n=7. Overall, the most common SCD complication was acute chest syndrome (ACS) (62.5%). The majority had hypertension (HTN) (52.8%), followed by reported heart failure (40.3%) and pulmonary HTN (pHTN) (40.3%), stroke (37.5%), and chronic kidney disease (CKD) (31.9%). 35% were current and/or former smokers. Median TR max velocity was 2.8 m/sec, median albumin/Cr ratio was 27.8 mg/g, and other characteristics are in Table 1. The median age of death was 44 (STD= 15.5) overall and by genotype it was: SS at age 38 (STD=14.6); Sβ0 thal at age 42.5 ( STD=2.12); Sβ+ thal at age 50 ( STD=14.7); SC at age 51 (STD=16.5). 46 patients had data of their final hospitalization available. 73.9% (n=34) received at least 1 simple transfusion with a only 17.4% (n=8) receiving an exchange transfusion. The majority were critically ill, with 84.8% in the ICU and 95.7% with multiorgan failure. 50 patients had routine follow up (RF) while 14 patients did not (NF), and 8 patients with unknown outpatient visit status. The median age of death for RF and NF were the same 43.5 (STD=15.53) & 43 (STD=15.71) years, respectively. The genotype distribution, and other characteristics are listed in Table 2. About 44% (n=22) of the RF group were prescribed HU, with 31.8% (n=7) were prescribed a dose > 15mg/kg/dose. This was not a significant difference from the NF group, where 50% (n=7) were prescribed HU, with 42.9% at > 15mg/kg/dose. The median MCV, percentage of HbF were similar. Significant majority in RF and NF groups were prescribed chronic opiates (86% and 78.6%, respectively) with 105 and 95 median MME per day. 42.9% of patients in the NF group had alloimmunization compared to 26% in the RF group. Discussion: Most of the patients had at least 1 severe complications from SCD, and most had other comorbidities (Table 1). This is commensurate with SCD literature showing that the manifestations of chronic end-organ damage increase as patients age which increases the likelihood of early mortality. In our analysis, the median age of death was similar for those in the RF and NF groups across genotypes. HU is a disease-modifying therapy that impacts survival and reduces complications and was FDA approved in 1998, however less than 50% of patients had a prescription. Median HbF and MCV were similar between the 2 groups, suggesting HU adherence was not a factor. This raises concern about accessibility of HU for patients which was not within the scope of this review. Improvement of access and education for patients and clinicians of these therapies is needed to see reduction in mortality. As more of the adult population starts to include those with access to HU since early childhood, future studies can examine what impact that will have on HU adherence, and life expectancy. With the advent of new therapies, it is imperative to study these agents on the patients with SCD complications to evaluate their efficacy in reducing mortality. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

On the Uses of Disinformation to Legitimize the Revival of the Cold War: Health in the U.S.S.R.

1982 ◽  
Vol 12 (3) ◽  
pp. 481-496 ◽  
Author(s):  
Albert Szymanski
Keyword(s):  
Child Care ◽  
Soviet Union ◽  
Infant Mortality ◽  
Life Expectancy ◽  
Public Health Education ◽  
The United States ◽  
Mortality Data ◽  
The Soviet Union ◽  
Employment Of Women ◽  
Child Care Facilities

During the 1970s the Soviet Union experienced rising infant mortality rates and a corresponding levelling off of earlier increases in life expectancy. Several Western critics have misrepresented or exaggerated these statistics, suggesting that these trends indicate a general breakdown in the Soviet health care system as well as the failure of the Soviet form of socialism. This paper examines life expectancy and infant mortality data by Soviet republic, showing that rates are not uniform throughout the U.S.S.R. and in many cases compare favorably with those in Western European countries and the United States. It is suggested that the infant mortality problem in the U.S.S.R. is a temporary negative consequence of rapid progress in the areas of industrialization, employment of women, and socialization of child care. It is concluded that improvements in public health education, the quality of child care facilities, and the manufacture and distribution of infant formula will contribute to the rapid resolution of this problem.

scholarly journals Predictors of Maternal Morbidity Among Participants Enrolled in the Sickle Cell Disease Implementation Consortium Registry

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 3-3
Author(s):  
Rita V Masese ◽  
Dominique Bulgin ◽  
Liliana Preiss ◽  
Mitchell Knisely ◽  
Eleanor Stevenson ◽  
...  
Keyword(s):  
Board Of Directors ◽  
Sickle Cell ◽  
Research Funding ◽  
Transfusion Requirement ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Maternal Outcomes ◽  
Maternal Complications ◽  
Advisory Committees ◽  
Increased Risk

Introduction Pregnancy in sickle cell disease (SCD) is associated with an exacerbation of SCD-related complications and an increased risk of maternal complications. The increased risk is partly due to physiologic adaptations in pregnancy, which include increased metabolic demands and a hypercoagulable state. The maternal death rate for SCD is 629 per 100,000 deliveries, compared to 12 per 100,000 deliveries in black women and 6 per 100,000 deliveries in the general population (Raider et al., 2016). Studies on maternal and perinatal outcomes of patients with SCD present inconsistent and conflicting results. Some studies have reported an increase in maternal complications such as pre-eclampsia, acute chest syndrome and thromboembolic events, while other studies have reported no significant risk in adverse maternal outcomes. The inconsistent findings reported in prior studies may be attributed to small sample sizes and single-centered sites. Our study aims to determine the prevalence and predictors of maternal morbidity among participants enrolled in the SCD Implementation Consortium (SCDIC) registry, which is the largest, most geographically diverse SCD participant sample in the United States. Methods This cross-sectional study included women enrolled in the SCDIC registry who had at least one pregnancy event. The SCDIC is composed of eight academic SCD centers across the United States and one data-coordinating center. Participants were enrolled in the SCDIC registry if they were 18 to 45 years of age and had a confirmed diagnosis of SCD. Enrolled participants completed a series of surveys that collected sociodemographic information, SCD and pregnancy history and data abstractions of participants' medical records was completed. Medical complications queried during pregnancy included: vaso-occlusive episodes, acute chest syndrome, blood transfusion requirement, preeclampsia, maternal diabetes and deep venous thrombosis. Descriptive analysis of sociodemographic, clinical and maternal characteristics was conducted. Bivariate analysis was performed using Chi-Square test, Mann-Whitney U test, t-test, and logistic regressions, as appropriate. A p-value of ≤ 0.05 was considered statistically significant for all analysis. Results The study sample included 743 women who had at least one pregnancy event, and a total of 1066 live births. Almost all women (96.3%) were African American, with a median age of 21 years (inter-quartile range of 19 to 23 years) at first birth. The majority had Hb SS SCD genotype (69.5%; 513 of the 738 with SCD genotype data). Of all reported pregnancies, participants did not use hydroxyurea during conception (78%), and pregnancy (84.5%). Only 2.7 % of the women reported using fertility drugs or assisted reproductive procedures. Seventy five percent of the pregnancies that ended in live births had maternal complications. The leading complications were vaso-occlusive episodes (61.2%), pregnancy requiring blood transfusion(s) (33.2%), preeclampsia (15.4%), deep venous thrombosis (5.6%) and acute chest syndrome (7.7%). When the pregnancies were stratified by SCD genotype, women with Hb SS had a higher occurrence of acute chest syndrome (63.4% vs. 26.7%), transfusion requirement (70.8% vs. 21%) and preeclampsia (66.7% vs 22.4%). In the univariate logistic regressions, multiparous women, with a history of adverse maternal outcomes in a previous pregnancy, had higher odds of vaso-occlusive episodes (OR: 3.42; 95% CI: 2.42-4.94) acute chest syndrome (OR:4.99; 95% CI:2.56- 9.48), transfusion requirement (OR:3.86; 95% CI:2.64- 5.69), and pre-eclampsia (OR:3.36; 95% CI:2.05-5.45). Conclusion In this large multicenter registry, we found pregnant women with SCD have significant maternal complications. Early antenatal care by healthcare providers knowledgeable about risk factors for adverse maternal outcomes in SCD is essential improve maternal and fetal outcomes and reduce the maternal death rate for SCD. Disclosures Hankins: Novartis: Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; MJH Life Sciences: Consultancy, Patents & Royalties; UptoDate: Consultancy; National Heart, Lung, and Blood Institute: Honoraria, Research Funding; LINKS Incorporate Foundation: Research Funding; American Society of Pediatric Hematology/Oncology: Honoraria. Treadwell:Global Blood Therapeutics: Consultancy; UpToDate: Honoraria. King:Amphivena Therapeutics: Research Funding; Bioline: Consultancy; Celgene: Consultancy; Cell Works: Consultancy; Incyte: Consultancy; Magenta Therapeutics: Membership on an entity's Board of Directors or advisory committees; Novimmune: Research Funding; RiverVest: Consultancy; Tioma Therapuetics: Consultancy; WUGEN: Current equity holder in private company. Gordeuk:CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Imara: Research Funding; Ironwood: Research Funding; Novartis: Consultancy. Kanter:SCDAA Medical and Research Advisory Board: Membership on an entity's Board of Directors or advisory committees; AGIOS: Membership on an entity's Board of Directors or advisory committees; BEAM: Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy; GLG: Honoraria; Jeffries: Honoraria; Cowen: Honoraria; Wells Fargo: Honoraria; NHLBI Sickle Cell Advisory Board: Membership on an entity's Board of Directors or advisory committees; Medscape: Honoraria; Guidepoint Global: Honoraria; bluebird bio, inc: Consultancy, Honoraria; Sanofi: Consultancy. Glassberg:Pfizer: Research Funding; Global Blood Therapeutics: Consultancy; Eli Lilly and Company: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; CSL Behring: Consultancy; Bluebird Bio: Consultancy; Global Blood Therapeutics: Consultancy, Research Funding, Speakers Bureau.

scholarly journals Pregnancies and Neonatal Outcomes in Patients with Sickle Cell Disease (SCD): Still a (High-)Risk Constellation?

2021 ◽  
Vol 11 (9) ◽  
pp. 870
Author(s):  
Pia Proske ◽  
Laura Distelmaier ◽  
Carmen Aramayo-Singelmann ◽  
Nikolaos Koliastas ◽  
Antonella Iannaccone ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neonatal Outcomes ◽  
University Hospital ◽  
High Rate ◽  
Successful Outcome ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
Tract Infections

Background: This monocentric study conducted at the University Hospital of Essen aims to describe maternal and fetal/neonatal outcomes in sickle cell disease (SCD) documented between 1996 to 2021 (N = 53), reflecting the largest monocentric analysis carried out in Germany. Methods/Results: 46 pregnancies in 22 patients were followed. None of the patients died. In total, 35% (11/31) of pregnancies were preterm. 15 pregnancies in eight patients were conceived on hydroxycarbamide (HC), of which nine had a successful outcome and three were terminated prematurely. There was no difference regarding the rate of spontaneous abortions in patients receiving HC compared to HC-naive patients prior to conception. In patients other than HbS/C disease, pregnancies were complicated by vaso-occlusive crises (VOCs)/acute pain crises (APCs) (96%, 23/24); acute chest syndrome (ACS) (13%, 3/24), transfusion demand (79%, 19/24), urinary tract infections (UTIs) (42%, 10/24) and thromboembolic events (8%, 2/24). In HbS/C patients complications included: VOCs/APCs (43%, 3/7; ACS: 14%, 1/7), transfusion demand (14%, 1/7), and UTIs (14%, 1/7). Independent of preterm deliveries, a significant difference with respect to neonatal growth in favor of neonates from HbS/C mothers was observed. Conclusion: Our data support the results of previous studies, highlighting the high rate of maternal and fetal/neonatal complications in pregnant SCD patients.

Newborn Screening for Sickle Cell Disease and Other Hemoglobinopathies

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 813-814
Author(s):  
DORIS WETHERS ◽  
HOWARD PEARSON ◽  
MARILYN GASTON
Keyword(s):  
Sickle Cell Disease ◽  
Early Diagnosis ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Case Fatality ◽  
The United States ◽  
Early Mortality ◽  
Cell Disease ◽  
Newborn Period ◽  
The Past

Hemoglobinopathies represent one of the major health problems in the United States and constitute the most common genetic disorders in some populations. Sickle cell disease (SS, SC, S-β-thalassemia) alone affects about one in 400 American black newborns, as well as persons of African, Mediterranean, Asian, Caribbean, Middle Eastern, and South and Central American origins. For the past 20 years, the medical profession has known that children with sickle cell anemia have an increased susceptibility to severe bacterial infection, particularly due to Streptococcus pneumoniae. The risk of major infection and death posed by this organism is greatest in the first 3 years of life and can occur as early as 3 months of age. In fact, this infection may be the first clinical manifestation of disease. The infection can be fulminant, progressing from the onset of fever to death in a matter of hours, and the case fatality rate is reported as high as 30%. In addition, acute splenic sequestration, another acute catastrophic event, contributes to early mortality in children with sickle cell anemia and may occur as early as 5 months of age. It has been proposed that early diagnosis to identify infants with major sickle hemoglobinopathies, who have a high risk of early mortality and morbidity, is essential to institute appropriate ongoing care and effective measures of prophylaxis and intervention. Early diagnosis of hemoglobinopathies should be in the newborn period. Even though the technology to screen infants in the newborn period has been available for the past 15 to 20 years, screening has not received widespread acceptance.

scholarly journals Equity and length of lifespan are not the same

2016 ◽  
Vol 113 (30) ◽  
pp. 8420-8423 ◽  
Author(s):  
Benjamin Seligman ◽  
Gabi Greenberg ◽  
Shripad Tuljapurkar
Keyword(s):  
Life Expectancy ◽  
Health Equity ◽  
Causes Of Death ◽  
The United States ◽  
Past Century ◽  
The Past ◽  
The United Kingdom ◽  
Mortality Improvement ◽  
Over Time ◽  
Age Of Death

Efforts to understand the dramatic declines in mortality over the past century have focused on life expectancy. However, understanding changes in disparity in age of death is important to understanding mechanisms of mortality improvement and devising policy to promote health equity. We derive a novel decomposition of variance in age of death, a measure of inequality, and apply it to cause-specific contributions to the change in variance among the G7 countries (Canada, France, Germany, Italy, Japan, the United Kingdom, and the United States) from 1950 to 2010. We find that the causes of death that contributed most to declines in the variance are different from those that contributed most to increase in life expectancy; in particular, they affect mortality at younger ages. We also find that, for two leading causes of death [cancers and cardiovascular disease (CVD)], there are no consistent relationships between changes in life expectancy and variance either within countries over time or between countries. These results show that promoting health at younger ages is critical for health equity and that policies to control cancer and CVD may have differing implications for equity.

scholarly journals Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
The United States ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cooperative Study ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

Hospitalization Rates in Patients with Sickle Cell Disease (SCD) in the State of Maryland (MD): No Change Since Approval of Hydroxyurea (HU).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 107-107
Author(s):  
Sophie Lanzkron ◽  
George J. Dover
Keyword(s):  
Sickle Cell ◽  
Census Data ◽  
Eligible Patient ◽  
Johns Hopkins Hospital ◽  
Acute Chest Syndrome ◽  
Total Hospital ◽  
Significant Difference ◽  
Hospital Days ◽  
Number Of Individuals ◽  
Total Charges

Abstract Background : In 1998 the FDA approved the use of HU for use in patients (pts) with SCD. The trial on which the approval was based demonstrated that adult pts that were on HU had fewer hospitalizations, fewer episodes of acute chest syndrome and required fewer transfusions than those patients that were not on HU. The authors of this original study were able to calculate a cost savings of over 20 million dollars a year if every eligible patient in the US were taking HU. There is currently no literature that addresses the use of HU outside the setting of a clinical study. Methods: We reviewed the inpatient and outpatient charts of all adults with SCD, admitted to Johns Hopkins Hospital (JHH) in FY2003. We also reviewed hospital admission data for MD in FY2003 and FY1995, using data from the MD Health Services Cost Review Commission. Data was pulled using the following ICD9 codes 28260,28261,28263,28269,28262. An estimate of total number of individuals with SCD in MD was made using 1990 and 2000 census data and a prevalence of SCD of 1 in 400 African Americans (AA). Fisher’s exact test was used to compare proportions. As readmission data was unavailable we assumed that the rates of readmission were similar for 1995 and 2003. Results : Review of the JHH data (Table 1) showed that 25% of pts with SCD accounted for half of the hospital days for all pts with SCD and almost 40% of total charges. 66% of eligible pts with Hgb SS were not receiving HU. The reasons for not being on HU varied; 4/9 did not have regular outpatient follow-up, 1 was pregnant, 2 had compliance issues related to side effects, 1 patient refused to take HU and 1 patient started during FY2003. For the pts with hgb SC disease, the indications for use of HU are not established and in our pt cohort those that had received HU in the past did not attain significant benefit without toxicity. Based on US census data the number of individuals of African decent, 18 years and older with SCD in MD for 2003 was 2760. By comparison the number estimated to be in MD in 1995 was 2361. The number of adult admissions for the diagnosis of SCD to hospitals in MD in FY1995 was 1313 and in FY2003 it was 1961. The number of admissions per estimated AA adult with SCD in MD for 1995 was .56 and for 2003 was .71. (p<0.001) The annual costs of caring for hospitalized adult sickle cell pts in MD in 1995 was $6.7 million compared to over $10 million for FY2003. The percent of total hospital expenditures spent on SCD admissions in MD was 0.12% in 1995 and doubled to 0.21% in 2003. Pediatric data over the same time period demonstrated an increase in number of hospitalizations 909 in 1995 compared to 1202 in 2003. When the ratios of admissions per estimated number of pediatric sickle cell pt. in MD were compared for the two time periods there was no statistically significant difference. Conclusions : Hospital generated data for MD demonstrates that admissions and costs of caring for adult sickle cell pts have increased significantly since the approval of HU by the FDA. Data from JHH shows that a large number of eligible patients are not taking HU. Further investigation into the issues affecting hospitalizations of individuals with SCD and the use of HU in this patient population is warranted. Characteristics of SCD Patients Admitted to JHH All SCD patients ≥ 3 admissions/yr (%) Total Patients 109 27(25) Total Hospital days 1266 615(49) Total Admits 225 124 (55) Total Charges ($) 2,796,088 1,090,348(39) Number on HU Unknown 4

Abnormal Pulmonary Function in Adults with Sickle Cell Disease: Association of Decreased DLCO with Systemic Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 316-316 ◽  
Author(s):  
Elizabeth S. Klings ◽  
Diego F. Wyszynski ◽  
Vikki G. Nolan ◽  
Martin H. Steinberg
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Function ◽  
Sickle Cell ◽  
Pulmonary Function Tests ◽  
Systemic Disease ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Restrictive Physiology ◽  
Abnormal Pulmonary Function

Abstract Pulmonary complications of sickle cell disease (SCD), including acute chest syndrome, pulmonary hypertension (PH) and pulmonary fibrosis, are common. Dyspnea and hypoxemia are equally common in this population. It is likely that pulmonary function tests (PFT) are abnormal in the SCD population, however, no extensive study has been reported to date. Moreover, the relationship between abnormal pulmonary function and other manifestations of SCD, such as PH, is unclear. We hypothesized that abnormalities of pulmonary function, particularly a low diffusion capacity for carbon monoxide (DLCO), may be associated with other complications of SCD. The Cooperative Study of Sickle Cell Disease (CSSCD) enrolled and followed more than 4,000 SCD patients who had visited one of 23 participating clinical centers across the United States between 1978 and 1998. Data were collected on many complications of the disease, and standardized collection of PFTs were part of the protocol. From the more than 1300 CSSCD patients who had the results of PFTs recorded, 310 adults (age≥ 20 years of age) homozygous for the Hb S gene without coincident α thalassemia and with sufficient data were identified. Predicted values for FEV1, FVC, FEV1/FVC, TLC, RV and DLCO were calculated using algorithms that accounted for gender, age, and height in the African American population (using STATA, version 9); data are presented as percent predicted. Based on criteria established by the American Thoracic Society, subjects were sub-classified into 7 groups: obstructive physiology; restrictive physiology; mixed obstructive/restrictive disease; low lung volumes with normal spirometry (LLV); LLV with a low DLCO, isolated low DLCO, or normal. The association of blood counts and serum chemistries between patients with low DLCO compared with those with a normal DLCO was assessed by multivariate linear regression (using SAS software version 8.2). Normal PFTs were present in only 31 of 310 (10 %) SCD patients. Overall, the adult SCD population was characterized by decreased total lung capacities (70.2 + 14.7% predicted) and DLCO (64.5 + 19.9 % predicted adjusted for hemoglobin concentration). The most common PFT patterns observed were restrictive physiology (35.8%), LLV with normal spirometry (34.2% of patients), and an isolated low DLCO (12.9%). The presence of a low DLCO was associated with an elevated platelet count (p=0.05), hepatic dysfunction [elevated ALT (p=0.07) with elevated AST (p=0.01)] and renal dysfunction [elevated BUN and creatinine (p=0.05, 0.07)]. Restrictive disease is marginally associated with a decrease in hematocrit (p=0.07) and Hb F levels (p=0.07). Pulmonary function is abnormal in 90% of adult SCD patients. Common abnormalities include restrictive physiology, LLV with normal spirometry and a decreased DLCO. The presence of a decreased DLCO may be a marker of more severe systemic disease that includes impaired renal and hepatic function and possibly complications of hemolytic anemia such as PH.

Changes of BNP Level and Pulmonary Arterial Pressure during An Acute Sickle Cell Crisis, Comparison with Steady State

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2503-2503
Author(s):  
Aref Agheli ◽  
Chenthil Rathnasabapathy ◽  
Ashish Sangal ◽  
Zili He ◽  
William Steier ◽  
...  
Keyword(s):  
Steady State ◽  
Right Ventricular ◽  
Sickle Cell ◽  
Ventricular Dysfunction ◽  
Right Ventricular Dysfunction ◽  
Acute Chest Syndrome ◽  
Cardiac Complications ◽  
Significant Difference ◽  
Sickle Cell Crisis ◽  
Pulmonary Arterial

Abstract Background: The heart is frequently involved in Sickle Cell Anemia (SCA). Cardiomegaly is a usual finding, significant arrythmias and sudden death are common, and 30% of patients with both homozygous and heterozygous SCA develop Pulmonary Arterial Hypertension (PAH), a major risk factor for higher mortality in this population. Brain Natriuretic Peptide (BNP) and echocardiographic data could provide important prognostic and diagnostic information about PAH in SCD. High levels of BNP, which is released from ventricular cardiomyocytes in response to their stretch, reflect cardiac chamber volume and pressure overload in various conditions. In patients with PAH, BNP levels correlate with the severity of Pulmonary Artery Pressure (PAP) elevation and right ventricular dysfunction. In human, the half life of BNP is 20 minutes, reflecting the fluctuation of BNP levels during different stages of any acute cardiac pathology. Methodology: The hypothesis of this prospective IRB approved study was to investigate the BNP level and PAP elevation during an acute Sickle Cell Crisis (SCC), in particular in those with intrathoracic structures involvement. Between December 2006 and July 2008, 81 patients were registered after a written informed consent was obtained. We collected the BNP levels and echocardiographic data of patients with SCD and compared them in two group; those who were admitted with Sickle Cell Crisis (SCC) and those who returned to clinic in Steady State (SS) for follow up. The data were obtained on the first day of admission in SCC group. The primary endpoint was the elevation of the BNP level and the secondary endpoint was elevation of the PAP during a SCC, which were compared with SS patients. The inclusion criterion was age above 18 and having one of the sickle cell syndromes, requiring hospital admission. Results: Forty nine patients (59%) were female, and 34 (41%) patients were male. Their ages ranged from 19 to 65, mean (SD) 30.2 (9.7) years. The mean (SD) levels of BNP were significantly higher in patients who were admitted with one of the acute complications or vaso-occlusive crisis of sickle cell, [177.3 (23.4) pg/ml], when compared with its levels in SS, [34.17 (6.1) pg/ml], (95% CI 61.4 to 225.0, p<.001) (Figure 1). An elevated BNP level was defined as levels more than 100 pg/ml. A further subgroup analysis revealed that the BNP levels were even more significantly higher in patients with acute chest syndrome or other intrathoracic events [(n= 17, mean (SD) 363.6 (121.3) pg/ml], when compared with those of simple acute sickle cell crisis, [(n= 35, mean (SD) 167.7 (26.8) pg/ml] (p=.038) (Figure 1). Topographic data about heart chambers’ sizes, volumes, and pressures were obtained by Echocardiography and compared in two groups. While only 23.1% of patients in SS group had elevated PAP with a mean (SD) of 43 (2.1) mmHg, 41.1% (n=21) of patients with SCC had elevated PAP with mean (SD) 45.9 (2.1) mmHg, with no significant difference between two groups with PAH (p=.608). Conclusion: Patients with either homozygous or heterozygous forms of SCA can have cardiac complications, such systolic and diastolic dysfunction. Hypoxemia leads to raised levels of BNP production. In patients with SCA, an elevated BNP level largely reflects the severity of right ventricular dysfunction associated with PAH. Our data revealed that BNP level and PAP are increased during vaso-occlusive crisis of SCA, in particular during those life-threatening complications, such acute chest syndrome. These changes seem to be temporary and with clinical improvement, the majority of patients’ BNP levels and PAP return to the baseline, although some will never normalize. Figure Figure

Alloimmunization Does Not Modify the Clinical Profile of Sickle Cell Disease Patients from Salvador-Brazil

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4799-4799
Author(s):  
Angela Zanette ◽  
Karla O. Mota ◽  
Marilda Souza Goncalves ◽  
Laise Vilasboas Schettini ◽  
Lais Magalhaes Aguiar ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcomes ◽  
Sickle Cell ◽  
Laboratory Data ◽  
Clinical Profile ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Hemoglobin C ◽  
Significant Difference

Abstract Introduction: The hemoglobinopathies are the most common monogenic disorders known. A mutation in the gene for β globin gave origin to hemoglobin S, an abnormal hemoglobin originated in Africa. Sickle cell disease (SCD) is characterized by the presence of hemoglobin S, which results in vasoocclusion episodes and hemolytic anemia throughout patients life. Vascular occlusion leads to acute events and progressive disabling organ damage. Sickle cell anemia is the homozygous state SS, while hemoglobinopathy SC is a doubly heterozygous state, where hemoglobin S occurs in combination with hemoglobin C. Brazil has a prominent African ancestry and SCD is highly prevalent in some regions of the country. In Bahia State, for example, neonatal screening data have shown that, from every 650 children born alive, one has SCD, mostly homozygous SS. Among other therapeutic measures, packed red blood cells (RBC) play a prominent role in SCD management. In situations such as acute chest syndrome (ACS), primary and secondary prevention of stroke, splenic or hepatic sequestration crisis, severe anemia, complicated pregnancy, isquemic organ damages and others, the transfusions may save lives. Although RBC may contribute to reduce morbidity and improve quality of life in SCD patients, there still are risks. Among other risk categories, alloimmunization may result from transfusions and occurs in 5 % to 50 % of SCD patients. It is still not known whether allosensibilization significantly affects the clinical outcomes in SCD. Objecive: The purpose of this study was to compare the clinical profile of multitransfused adult SCD patients who developed alloantibodies (ALO) to patients with the same disease, coming from the same population who did not become alloimmunized (non-ALO). Methods: This is a cross sectional study where medical records of SCD patients, referred to a reference center of Salvador, the capital of Bahia State, Brazil, were reviewed. Only SCD patients 18 years of age or older were included. They had received at least 3 RBC transfusions from 2004 to 2007, or had any alloantibody identified during this period. Patient characteristics, clinical findings, number of transfusions, frequency and specificity of alloantibodies, laboratory data, and the main clinical outcomes were reviewed. Results: a hundred and eight patients were included: 105 SS and 3 SC. The pre-transfusional RBC matching was done to ABH, D,C,c,E,e and Kell antigens. 56 patients developed alloantibodies (53 SS and 3 SC). Anti-E, anti-K, and anti-C were the most prevalent alloantibodies identified (39,3 %, 21,4 % and 16,1 %, respectively). Among the variables addressed in this study, age (higher in non-ALO, .041) and antiglobulin test positivity, more prevalente in ALO (.0001), depicted statistically significant difference. A few patients developed immune hemolysis, controlled successfully with corticosteroids. Alloimmunization was more prevalent among women, although no statistically significant difference was reached between ALO and non-ALO Other variables such as number of transfusions, hematological profile, biochemical data and complications such as stroke, leg ulcers, osteonecrosis, renal disease, abnormal cardiac features, and pulmonary hypertension did not show significant difference between both groups. Conclusion: his study shows that, although alloimmunization is a potential dangerous consequence of RBC transfusions, it did not modify the clinical profile of SCD alloimmunized patients. The concomitance of allosensibilization and autoantibodies in SCD leads to additional difficulties in the RBC matching for transfusion and may exacerbate hemolysis. In order to address autoimmunity in SCD, prospective studies with larger samples are needed.

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