scholarly journals A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Acute Infection ◽  
Organ Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk ◽  
Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

scholarly journals The Effect of Health Care Disparities on Complications and Mortality in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5886-5886
Author(s):  
Stuthi Pavani Perimbeti ◽  
Kevin Ye Hou ◽  
Sabarina Ramanathan ◽  
Adonas Woodard ◽  
Daniel Kyung ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Healthcare Cost ◽  
Sickle Cell ◽  
Significant Role ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
Insurance Status ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk

Abstract Introduction: Sickle cell disease (SCD) is a chronic and debilitating disorder that affects approximately 100,000 Americans and results in the development of significant complications, leading to high numbers of hospitalizations, healthcare cost and mortality. Despite the advent of newer therapies, the overall rate of complications has continued to rise. We aimed to study the prevalence of complications in SCD as well as its relation to differing insurance status. Methods: Patients with SCD were identified using ICD-9 codes 2826, 28260, 28261, 28262, 28263, 28264, 28268 and 28269 from the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample from 1999 to 2014. Admission with acute chest syndrome, acute myocardial infarction (AMI), avascular necrosis of the hip (AVN), end stage renal disease (ESRD), pneumococcal infections, splenic sequestration and stroke. Univariate and bivariate analyses were performed using the Chi square test. Cox proportional hazard regression was used to control for multiple confounders in calculating the hazard ratios of an event occurrence and mortality. Results: A total of 216,438 (Weighted=1,066,536) observations were identified from the years 1999 to 2014. The median age for male patients was 25 years and that for females was 27. Observing the trends from 1999 to 2014, the prevalence of acute chest syndrome increased from 1.22% to 8.82% (p=0.002), splenic sequestration from to 0.08 % to 1% (p=0.01) and AVN from 1 % to 8.8% (p=0.001). The prevalence of stroke and ESRD were unchanged over the interval studied. After controlling for confounding factors such as race, age, sex, income, comorbidities and insurance status, the hazard ratio of mortality for various complications is significantly elevated. Also, after controlling for multiple confounders, the patient's insurance status plays a significant role in the risk of developing a complication and subsequent mortality (Table 1). Discussion: The data indicates that the rate of complications from SCD have risen since 1999. With newer therapies and better understanding, the life expectancy of SCD patients has risen over time, nearly doubling from 1951 to 2018. The increased frequency of complications may be attributed to better survivorship and a rising number of older SCDs patients. However, our data also suggests that insurance status plays a significant role in the complication rate of SCD. The uninsured and patients with Medicaid have significantly increased risk of developing disease complications and resultant mortality. This could be the result of reduced access to care and health disparities due to race, socioeconomic status and insurance status. Disclosures No relevant conflicts of interest to declare.

Epidemiology of human parvovirus B19 in children with sickle cell disease

Blood ◽  
2004 ◽  
Vol 103 (2) ◽  
pp. 422-427 ◽  
Author(s):  
Kim Smith-Whitley ◽  
Huaqing Zhao ◽  
Richard L. Hodinka ◽  
Janet Kwiatkowski ◽  
Renèe Cecil ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Epidemiologic Study ◽  
Incidence Rates ◽  
Acute Chest Syndrome ◽  
Prevention Strategies ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Abstract Human parvovirus (HPV) B19 causes significant morbidity and mortality in children with sickle cell disease (SCD), but little data are published about the epidemiology of HPV B19 infection and its associated complications in this patient population. In this study, prevalence and incidence rates of HPV B19 were determined in 633 patients with SCD followed at The Children's Hospital of Philadelphia between November 1996 and December 2001. Thirty percent (30%) were HPV B19 immunoglobulin G (IgG) positive at first testing, and the 70% without evidence of past HPV B19 infection were tested annually. One hundred ten patients developed evidence of HPV B19 infection for an incidence rate of 11.3 per 100 patientyears. Sixty-eight episodes of HPV B19–induced transient red cell aplasia occurred with the following clinical events: fever (89.7%), pain (61.8%), acute splenic sequestration (19.1%), and acute chest syndrome (11.8%). Pain, fever, and acute splenic sequestration were more frequent events with acute HPV B19 infections compared with acute events in uninfected patients. The results of this epidemiologic study, the largest and most comprehensive to date, justify the development of HPV B19 prevention strategies to diminish the frequent and often severe complications associated with HPV B19 infections in patients with SCD.

scholarly journals Identification of Clinical and Laboratory Parameters Associated with the Development of Acute Chest Syndrome during Vaso-Occlusive Episodes in Children with Sickle Cell Disease: A Preliminary Step before Assessing Specific and Early Treatment Strategies

2019 ◽  
Vol 8 (11) ◽  
pp. 1839
Author(s):  
Madhi ◽  
Kamdem ◽  
Jung ◽  
Carlier-Gonod ◽  
Biscardi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pain Score ◽  
Sickle Cell ◽  
Multivariate Model ◽  
Red Blood Cell Transfusion ◽  
Predictive Score ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Laboratory Parameters ◽  
Increased Risk

This prospective observational study sought to ascertain clinical and laboratory parameters associated with the development of acute chest syndrome (ACS) during vaso-occlusive episodes (VOE) in children with sickle cell disease (SCD). It was performed at the pediatric department of the university Intercommunal Créteil hospital. All children with SCD (all sickle genotypes) consecutively admitted from November 2013 to December 2016 for painful VOEs and no evidence of ACS were included. Clinical and laboratory parameters collected at admission and within 48 h after admission were compared for children in whom ACS developed or not. Variables that were statistically significant on univariate analysis or considered to be clinically relevant were included in a multivariable model to ascertain the risk factors associated with the development of ACS during a VOE. The variables retained in the multivariate model were used to construct a predictive score for ACS. For each included child and during the study period, only data from the first VOE and/or the first ACS were analyzed. Among 191 hospitalizations for painful VOEs, for 176 children with SCD, ACS developed in 35 during hospitalization. Mean hospital stay was longer for children with ACS versus VOEs alone (7.6 (±2.3) vs. 3.3 (±1.8) days, p < 0.0001), and all children with ACS versus 28/156 (17.9%) with VOEs alone received red blood cell transfusion (p < 0.0001). The multivariate model retained pain score (≥9/10), pain localization (abdominal or spinal pain or involving more than two limbs), and high reticulocyte (≥260 × 109/L) and neutrophil (>10 × 109/L) counts, at admission, as independently associated with ACS development. The area under the receiver operating characteristic curve for the ACS predictive score was 0.82 (95% CI: 0.74–0.89), and the negative predictive value was 97.7%. The evolution profiles during the first 48 h differed between children with ACS and VOEs alone, with a more rapid decline of pain score and leucocytosis in children with VOEs. Clinical and laboratory measurements at admission may be simple parameters to identify children with increased risk of ACS development during VOEs and to facilitate early diagnosis of this respiratory complication. Also, the persistent elevation of leukocyte count on day 2 may be considered a sign of evolving ACS.

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.

Polymorphisms in TNFα Are Associated with Cerebrovascular Events in Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1540-1540 ◽  
Author(s):  
Latorya A Barber ◽  
Allison E Ashley-Koch ◽  
Melanie E. Garrett ◽  
Karen L Soldano ◽  
Marilyn J. Telen
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Umbilical Vein ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Data Set ◽  
Clinical Complications ◽  
Cerebrovascular Events ◽  
Increased Risk

Abstract Abstract 1540 Poster Board I-563 Tumor necrosis factor alpha (TNFα) is a pro-inflammatory cytokine that stimulates phagocytosis, neutrophil recruitment, and expression of adhesion molecule VCAM-1. Plasma levels of TNFα have been found to be increased in sickle cell disease (SCD), and in vitro studies show that TNFα causes increased adherence of sickle red blood cells to human umbilical vein endothelial cells. A polymorphism in the promoter region of the TNFα gene has previously been associated with stroke in children with SCD (Hoppe et al., 2007). The current study was designed to identify associations of additional TNFα single nucleotide polymorphisms (SNPs) with SCD clinical complications. We analyzed five SNPs in the TNFα gene in 509 DNA samples of SCD patients from Duke University, University of North Carolina at Chapel Hill, and Emory University. In our data set, cerebrovascular events (CVEs), including overt stroke, seizures, and transient ischemic attacks, occurred in 133 out of 509 SCD patients (26.1%). SNP genotyping was performed using Taqman genotyping assays from Applied Biosystems. Due to low minor allele frequencies (<0.05) for all the SNPs examined, genetic associations with SCD clinical complications were examined by using allele tests. After controlling for age, gender, and use of hydroxyurea, two of the five TNFα SNPs, rs2228088 and rs3093665, were significantly associated with CVEs (p=0.013 and 0.029, respectively). The odds that SCD patients with a G allele at rs2228088 suffered from CVEs were 0.485 times that for patients with the T allele, suggesting that the G allele had a protective effect. The odds of having the A allele at rs3093665 and suffering from CVEs was also reduced, at 0.45 compared to the C allele. Neither SNP was found to be in linkage disequilibrium (LD) with any of the other SNPs analyzed (r2≤0.002). There was also strong association of SNP rs2228088 with acute chest syndrome (ACS; p=0.003), occurring in 382 out of 509 SCD patients (75%). However, in this analysis, the G allele was associated with increased risk for ACS (OR=2.313). In addition to the association with CVEs, the SNP rs3093665 was also significantly associated with priapism (p=0.03), reported by 86 of 223 male SCD patients (38.6%). In this analysis, the A allele was protective, as had been observed for CVE (OR=0.188). Additionally, we found no difference in steady state plasma TNFα levels between genotypes for the two SNPs. The functional significance of these SNPs is presently unknown. SNP rs2228088 is a synonymous SNP located in the coding region, and rs3093665 is located in the 3' untranslated region of the TNFα gene. While the G to T change at SNP rs2228088 does not translate to a change in amino acid sequence, the A to C change at SNP rs3093665 may affect mRNA stability due to its location. It is also possible that one or both of these SNPs is in LD with another functionally relevant SNP. Our findings thus support previous data implicating TNFα polymorphisms in risk for central nervous system events. Interestingly, ACS has been previously associated with seizures, stroke and altered mental status in adults and children with SCD (Vinchinsky et al., 2000) and with silent cerebral infarcts and reversible posterior leukoencephalopathy syndrome in children with SCD (Henderson et al., 2003). However, in our dataset, ACS and the occurrence of CVEs were not associated (p=0.24). Further studies are required to elucidate these and other factors that potentially correlate with SCD clinical complications. Disclosures No relevant conflicts of interest to declare.

Acute human parvovirus B19 infection in a 6-year old girl with sickle cell disease: a case report

2014 ◽  
Vol 5 ◽  
pp. 1
Author(s):  
Daniele Don� ◽  
Federica Visentin ◽  
Eleonora Borgia ◽  
Stefania Scanferla ◽  
Carlo Giaquinto ◽  
...  
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Human Parvovirus B19 ◽  
Cell Disease ◽  
Parvovirus B19 Infection

scholarly journals Regulatory Genetic Variation at the S100B Gene Associates with Vaso-Occlusive Manifestations in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1063-1063 ◽  
Author(s):  
Xu Zhang ◽  
Wei Zhang ◽  
Santosh L. Saraf ◽  
Sergei Nekhai ◽  
Mark T Gladwin ◽  
...  
Keyword(s):  
Linkage Disequilibrium ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Research Funding ◽  
Genetic Regulation ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Thrombotic Risk ◽  
Increased Risk

Abstract In sickle cell disease (SCD) polymerization of hemoglobin S under deoxygenated conditions causes vaso-occlusion, which can manifest as acute pain crisis and progressive bone/organ damage. Molecular studies have attributed vaso-occlusion to elevated vascular adhesion and inflammatory responses, whereas the genetic regulation has only recently been assessed. Genomic DNA isolated from peripheral blood mononuclear cells (PBMCs) was hybridized to Illumina Human 610-Quad SNP array for the PUSH and Walk-PHaSST cohorts and to Affrymetrix SNP 6.0 array for the Howard SCD expression cohort. Single nucleotide polymorphisms (SNPs) for 381 PUSH, 525 Walk-PHaSST, and 55 Howard patients were imputed to 1000 genomes project phase 3 data. Messenger RNA from PBMCs was profiled using Affymetrix Human Exon 1.0 ST Array for the Howard expression cohort and Affymetrix Human gene 2.0 ST array for the UIC expression cohort. Patients within the PUSH and Walk-PHaSST cohorts were classified to four groups according to a cumulative pain score, calculated based on pain frequency and questionnaire description of pain intensity. Pain grouping was examined for correlation with other SCD complications using Cochran Armitage test. History of acute chest syndrome (ACS, PUSH P=3.8×10-9, Walk-PHaSST P=2.4×10-5) and avascular necrosis (AVN, PUSH P=4.1×10-4, Walk-PHaSST P=3.7×10-5) were the most significant clinical manifestations that consistently associated with pain in the two cohorts. To investigate the genetic control of vaso-occlusive manifestations with appropriate power, we leveraged genetic association of pain, ACS, and AVN with genetic regulation of disease-specific gene expression. We mapped expression quantitative trait loci (eQTL) in the Howard expression cohort for SNPs<1 Mb away from gene ends per expression trait. At a permutation based false discovery rate of 5%, 1004 independent eQTL (linkage disequilibrium r2 ≤0.3 per trait) were identified for 880 genes. Focusing on 129 genes whose expression was altered in PBMCs in sickle cell anemia by at least 1.5-fold [1], we identified six eQTL for five differential genes (up-regulated: OSBP2, SLC14A1, RNF182, CCRL2; down-regulated: S100B). The six eQTL were assessed for association with pain, ACS, and AVN, using the Walk-PHaSST cohort for discovery and the PUSH cohort for validation. At a significance of Bonferroni corrected P=0.05 (nominal P=0.0083), an eQTL of S100B (rs2154586) significantly associated with AVN in the Walk-PHaSST cohort (OR=1.8, P=0.00061) and the association was replicated in the PUSH cohort (OR=2.7, P=0.0052). The A allele of the eQTL (frequency=0.18) associated with increased risk for AVN and increased expression level of S100B in the Howard expression cohort (β=0.40, P=1.6 ×10-6). In an additional 64 sickle cell anemia patients without hydroxyurea treatment from the UIC expression cohort, expression levels of S100B were significantly elevated in the individuals with AVN (β=0.28, P=0.029). The 24 SNPs in linkage disequilibrium with the eQTL (r2 >0.7) constituted the third most significant peak in a meta-analysis of genome-wide association of AVN in the PUSH and Walk-PHaSST cohorts. To test the hypothesis that genes involved in vaso-occlusion in SCD may affect thrombotic risk in non SCD individuals, we examined the association of the locus with venous thromboembolism (VTE) in the ARIC, JHS and CHS cohorts from dbGaP. The locus was imputed in African Americans and VTE was defined as being told by a doctor to have a blood clot in the leg or lung as answered in questionnaires during medical exams. The SNPs were associated with VTE using logistic linear regression adjusting for age, gender, enrollment site, and the first 15 principal components per cohort. The risk allele of the leading SNP for AVN consistently associated with increased risk of VTE across the cohorts, with a combined P=0.0041 and OR=1.4. S100B encodes a calcium sensor that appears to intervene in a variety of biological functions. S100B can mediate the inflammatory effects of damage-associated molecular pattern molecules (DAMPs) produced by erythrocyte hemolysis [2, 3]. Serum concentration of S100B correlates with LDH and with TCD-determined peak velocity of the left middle cerebral artery in thalassemia patients[4]. Polymorphisms of S100B that lead to increased serum levels are associated with increased risk of ischemic stroke in the Chinese population[5]. Disclosures Nekhai: NIMHD, NIH: Research Funding; NHLBI, NIH: Research Funding; NIAID, NIH: Research Funding.

scholarly journals Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

2008 ◽  
Vol 50 (5) ◽  
pp. 1006-1012 ◽  
Author(s):  
John J. Strouse ◽  
Clifford M. Takemoto ◽  
Jeffrey R. Keefer ◽  
Gregory J. Kato ◽  
James F. Casella
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Increased Risk

Children with Sickle Cell Disease on Chronic Red Cell Transfusion Experience Fewer Hospitalizations for Acute Vaso-Occlusive Episodes Irrespective of the Indication for Transfusion

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4282-4282
Author(s):  
Carmen C Wallace ◽  
Hilda Mata ◽  
Nancy J Wandersee ◽  
J. Paul Scott ◽  
Amanda M Brandow ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Admission Rate ◽  
T Test ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Red Cell Transfusion ◽  
The Impact

Abstract While there is strong evidence that chronic red cell transfusion is effective in preventing primary stroke and reducing the risk of recurrent stroke in sickle cell disease (SCD), it is less clear whether chronic transfusions will prevent admissions for other acute vaso-occlusive complications, including pain, priapism and/or acute chest syndrome. To our knowledge, no study to date has investigated the effect of chronic transfusion on the frequency of admissions for acute vaso-occlusive complications in children with all diagnoses of SCD and treated with chronic transfusion for a variety of indications. In addition, this study included a special focus on the effect of chronic transfusion on children who were transfused specifically for recurrent vaso-occlusive episodes. We performed a single-site retrospective chart review. We selected subjects from all children aged 0 to 19 years who were treated (lived in the Milwaukee area) and followed by the Wisconsin Sickle Cell Center at Children’s Hospital of Wisconsin from 1984 to May 2014 (n=695 subjects). Data was extracted from any individual who was enrolled in a chronic transfusion program for a minimum of six months. Data on admissions for painful vaso-occlusive crises, acute chest syndrome (ACS), other SCD complications as well as sickle diagnosis, age at time of transfusion, CBC, reticulocyte count, and percent sickle hemoglobin (HbS%) were collected for 24 months prior to onset of transfusion and for all months during transfusion until the age of 19 yrs. Unless otherwise indicated, all statistical analyses on extracted data were done by paired Student’s t-Test. We extracted data from 103 unique subjects for 108 chronic transfusion programs (as defined above); 5 subjects were chronically transfused twice, separated by at least 4 years without chronic transfusion. 55% were female; average age was 8.6 ± 5.6 (mean ± SD) years and the sickle diagnosis included 94% SS, 3% SC, 2% Sβ°-Thalassemia and 1% SD. The indication for transfusion included pain (n=31), priapism (n=6), ACS (n=5), central nervous system complications (n=37, including stroke, TIA, and abnormal TCD), splenic sequestration (n=25), pulmonary hypertension (n=2), retinopathy (n=1) and osteomyelitis (n=1). The hemoglobin level increased from a baseline of 7.6 ± 2.2 gm/dL to 9.6 ± 0.8 gm/dL during transfusion (p<0.0001, paired t-Test). HbS% was also reduced from a baseline of 84.2 ± 10.8% to 35.8 ± 0.3% during transfusion (p<0.0001). We found that rate of admissions for acute painful episodes, including priapism, dropped from 2.2 ± 2.9 admits/yr during the 24 months pre-transfusion to 1.0 ± 1.9 admits/yr during transfusion (p<0.0001). Similarly, the rate of admission for ACS decreased from 0.3 ± 0.5 admits/yr for 24 months pre-transfusion to 0.1 ± 0.3 admits/yr during transfusion (p=0.0001). Subanalyses were performed on specific indications for transfusion. For children transfused due to frequent acute vaso-occlusive complications (pain, priapism and ACS were arbitrarily included in this group), the average age at initiation of transfusion was 11.9 ± 4.4 yr, and admissions for acute painful episodes dropped from 4.0 ± 3.2 admits/yr during the 24 months pre-transfusion to 2.1 ± 2.6 admits/yr during transfusion (p=0.003). When the indication for transfusion was splenic sequestration (age 2.3±2.7 yr), the admission rate for acute painful episodes did not change (0.8±1.7 vs 0.3±0.5 admits/yr, p= 0.14). For children transfused for CNS complications (age 8.5±4.6 yr), the admission rate for pain improved from 0.9±1.3 to 0.2±0.5 admissions/yr (p=0.007). In agreement with previous studies, our data also showed an increase in the rate of admissions for pain (nontransfused) as subjects aged (r2=0.19, p<0.0001). Thus, the significant improvement in admission rate for pain during transfusion, while the child continues to age, further accentuates the impact of transfusion on the natural history of pain in SCD. In summary, our data suggest that chronic transfusion reduces hospital admissions for pain and acute chest syndrome in children with SCD. Our data also support the notion that chronic transfusion is an effective treatment to prevent not only stroke, but also other painful, life-threatening and life-limiting complications of sickle cell disease. Disclosures No relevant conflicts of interest to declare.

scholarly journals Acute human parvovirus B19 infection and nephrotic syndrome in patients with sickle cell disease

2010 ◽  
Vol 149 (2) ◽  
pp. 289-291 ◽  
Author(s):  
Lynn Quek ◽  
Claire Sharpe ◽  
Neelanjana Dutt ◽  
Sue Height ◽  
Marlene Allman ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Human Parvovirus B19 ◽  
Cell Disease ◽  
Parvovirus B19 Infection
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