Sphingomonas mucosissima Bacteremia in patient with sickle cell disease.
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ABSTRACT:
SUBMITTER: Angelakis E
PROVIDER: S-EPMC2660692 | biostudies-literature | 2009 Jan
REPOSITORIES: biostudies-literature
Publications
Sphingomonas mucosissima Bacteremia in patient with sickle cell disease.
Angelakis Emmanouil E Roux Véronique V Raoult Didier D
Emerging infectious diseases 20090101 1
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Project description:Patients with sickle cell disease (SCD) have an increased risk of invasive bacterial infection because of hyposplenism. Bordetella holmesii is a recently described Gram-negative coccobacillus with an apparent predilection for asplenic hosts. We report two patients with SCD and B. holmesii bacteremia. Fastidious growth in culture and a typically uncomplicated clinical course distinguish B. holmesii infection from other invasive bacterial infections in SCD. Providers for patients with SCD should be aware of this pathogen and ensure that their microbiology laboratories are capable of isolating and identifying this organism.
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Project description:Microbacterium species are non-spore-forming, Gram-positive rods rarely associated with human disease. In this report, we describe the first case of bacteremia caused by Microbacterium binotii in a patient with sickle cell anemia. The utility of using 16S rRNA gene sequence analysis along with phenotypic methods for identification is shown.
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Project description:Individuals living with sickle cell disease (SCD) experience severe recurrent acute and chronic pain. In order to develop novel therapies, it is necessary to better understand the neurobiological mechanisms underlying SCD pain. There are many barriers to gaining mechanistic insight into pathogenic SCD pain processes, such as differential gene expression and function of sensory neurons between humans and mice with SCD, as well as the limited availability of patient samples. These can be overcome by utilizing SCD patient-derived induced pluripotent stem cells (iPSCs) differentiated into sensory neurons (SCD iSNs). Here, we characterize the key gene expression and function of SCD iSNs to establish a model for higher-throughput investigation of intrinsic and extrinsic factors that may contribute to increased SCD patient pain. Importantly, identified roles for C-C Motif Chemokine Ligand 2 (CCL2) and endothelin 1 (ET1) in SCD pain can be recapitulated in SCD iSNs. Further, we find that plasma taken from SCD patients during acute pain increases SCD iSN calcium response to the nociceptive stimulus capsaicin compared to those treated with paired SCD patient plasma at baseline or healthy control plasma samples. Together, these data provide the framework necessary to utilize iSNs as a powerful tool to investigate the neurobiology of SCD and identify potential intrinsic mechanisms of SCD pain which may extend beyond a blood-based pathology.
| S-EPMC9882050 | biostudies-literature
Project description:Hematopoietic stem cell transplant (HSCT) is the only cure for patients with sickle cell disease (SCD). Although most SCD patients experience progressive end-organ damage and shortened lifespans, not all patients follow the same disease course, tempo, or outcome. Therefore, the dilemma facing physicians is weighing the selection of patients and timing for the procedure against donor type and transplant-related mortality and morbidity that go up with increasing age. On the other hand, the dilemma facing the patients and families is how acceptable HSCT that carries some mortality risks to them. We have analyzed the chronic conditions due to SCD in 449 patients to determine whether SCD-related multiple chronic conditions (MCC) can be risk-stratified to identify the group of patients predicted to not only have shortened lifespans but also functional limitation and poor quality of life so that these at-risk patients can be offered HSCT early and before MCC develops. We identified that the age of onset of the first SCD-related chronic conditions strongly predicted for the risks for disease-related MCC. SCD patients who suffered their first disease-related chronic condition before age 30 years developed MCC at a rate of 19.1 times faster than those at a later age. These patients are therefore high-risk patients and should be offered HSCT early in the course of their disease before multiple organ damage intervenes, even if matched-related donors are not available. This patient selection and timing approach provides a forum for an easy-to-understand and real-time discussion, including the choice of donor type, with SCD patients and families when considering HSCT.
| S-EPMC8504222 | biostudies-literature
Project description:Understanding patient experiences, quality of life, and treatment needs in individuals with sickle cell disease (SCD) is essential in promoting health and well-being. We used measures from the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me), Patient Reported Outcomes Measurement Information System (PROMIS), and Quality of Life in Neurological Disorders (NeuroQol) to evaluate pain impact, sleep impact, social functioning, depressive symptoms, tiredness, and cognitive function (collectively, patient reported outcomes [PROs]) and to identify associated demographic and clinical characteristics. Participants (n = 2201) between 18 and 45 years were recruited through the eight Sickle Cell Disease Implementation Consortium (SCDIC) sites. In multivariate models, PROs were significantly associated with one another. Pain impact was associated with age, education, employment, time since last pain attack, hydroxyurea use, opioid use, sleep impact, social functioning, and cognitive function (F = 88.74, P < .0001). Sleep impact was associated with household income, opioid use, pain impact, social functioning, depressive symptoms, and tiredness (F = 101.40, P < .0001). Social functioning was associated with employment, pain attacks in the past year, autoimmune/inflammatory comorbidities, pain impact, sleep impact, depressive symptoms, tiredness, and cognitive function (F = 121.73, P < .0001). Depressive symptoms were associated with sex, sleep impact, social functioning, tiredness, and cognitive function (F = 239.51, P < .0001). Tiredness was associated with sex, education, sleep impact, social functioning, depressive symptoms, and cognitive function (F = 129.13, P < .0001). These findings reflect the baseline PRO assessments among SCDIC registry participants. Further research is needed to better understand these outcomes and new targets for interventions to improve quality of life and function in people with SCD.
| S-EPMC8141351 | biostudies-literature
Project description:Sickle cell disease (SCD) is a chronic and prevalent hemoglobin disorder with various manifestations and complications depending on the organs involved. Red cell transfusion either simple or exchange is crucial due to its prophylactic and therapeutic roles. We present a case showing serologic discrepancy between the red cells phenotype and the developed alloantibodies to emphasize the crucial role of molecular testing in SCD patients requiring chronic blood transfusion.
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Project description:BackgroundAdults with sickle cell disease (SCD) constitute a unique and vulnerable patient population with complex healthcare needs including routine follow-up visits and acute care evaluations. The COVID-19 pandemic accelerated healthcare systems' transition to providing telemedicine care. The purpose of this qualitative study was to elicit the perspectives of adults with SCD about their experience with telemedicine during the COVID-19 pandemic and to understand their preferences with respect to future telemedicine care.MethodsAdults with SCD who had a telemedicine visit between March August 2020 and were cared for at our SCD center were eligible to participate in a one-time interview. Interviews were audio taped, transcribed, and analyzed using NVIVO software.ResultsAmong 30 interviewed subjects, 28 transcripts were available for analysis. Analysis identified that participants compared telemedicine to in-person care across several domains including (a) how time is used, (b) personal safety, (c) pain management, and (d) maintaining caring relationships. Participants agreed that telemedicine care was most appropriate for follow-up care and less useful for painful crises or urgent needs. They expressed concerns about the need to expand telemedicine to other specialities and to ensure that privacy and technical support are provided.ConclusionsTelemedicine appeals to adults with SCD for maintenance SCD care. Decisions about in-person or telemedicine care need to be made in discussion with the patient with particular attention to pain management preferences. Ultimately, telemedicine is an option that adults with SCD would like to see continue and that has the potential to expand access to care to more geographically distant regions.
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Project description:IntroductionWhite Blood Cell (WBC) count, %HbF, and serum creatinine (Cr), have been identified as markers for increased mortality in sickle cell anemia (SCA) but no studies have examined the significance of longitudinal rate of change in these or other biomarkers for SCA individuals.MethodsClinical, demographic and laboratory data from SCA patients seen in 2002 by our hospital system were obtained. Those who were still followed in 2012 (survival cohort) were compared to those who had died in the interim (mortality cohort). Patients lost to follow-up were excluded. Age adjusted multivariable Cox proportional hazards models were constructed to assess hazard ratios of mortality risk associated with the direction and degree of change for each variable.Results359 SCA patients were identified. Baseline higher levels of WBC, serum creatinine and hospital admissions were associated with increased mortality, as were alkaline phosphatase and aspartate aminotransaminase levels. Lower baseline levels of %HbF were also associated with increased mortality. When longitudinal rates of change for individuals were assessed, increases in Hb or WBC over patient baseline values were associated with greater mortality risk (HR 1.54, p = 0.02 and HR 1.16, p = 0.01 with negative predictive values of 87.8 and 94.4 respectively), while increasing ED use was associated with decreased mortality (HR 0.84, p = 0.01). We did not detect any increased mortality risk for longitudinal changes in annual clinic visits or admissions, creatinine or %HbF.ConclusionsAlthough initial steady state observations can help predict survival in SCA, the longitudinal course of a patient may give additional prognostic information.
| S-EPMC5072581 | biostudies-literature
Project description:Hydroxyurea (HU) has been widely used in sickle cell disease. Its potential long-term risk for carcinogenesis or leukemogenic risk remains undefined. Here, we report a 26 y old African-American female with Sickle Cell Disease (SCD) who developed refractory/relapsed acute myeloid leukemia (AML) 6 months after 26 months of HU use. That patient's cytogenetics and molecular genetics analyses demonstrated a complex mutation profile with 5q deletion, trisomy 8, and P53 deletion (deletion of 17p13.1). P53 gene sequence studies revealed a multitude of somatic mutations that most suggest a treatment-related etiology. The above-mentioned data indicates that the patient may have developed acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) as a direct result of HU exposure.
| S-EPMC6804808 | biostudies-literature
Project description:ImportanceBacteremia is a major cause of morbidity and mortality in children and young adults with sickle cell disease (SCD), but among those presenting to the emergency department (ED) with fever, the absolute risk of, risk factors associated with, and outcomes of bacteremia are poorly defined.ObjectiveTo obtain contemporary data on the absolute risk of, risk factors associated with, and outcomes associated with bacteremia in children and young adults with SCD presenting to the ED with fever.Design, setting, and participantsA multicenter retrospective cohort study was conducted of individuals with SCD younger than 22 years (young adults) presenting to EDs within the Pediatric Health Information Systems database from January 1, 2016, to December 31, 2021, with fever (identified by diagnostic codes for fever or the collection of blood samples for cultures and intravenous antibiotic administration). Data analysis was performed from May 17 to December 15, 2022.Main outcomes and measuresThe risk of bacteremia (defined by diagnostic coding) was identified in these children and young adults, and univariate analyses and multivariable regression were used to examine patient-level factors and bacteremia.ResultsA total of 35 548 encounters representing 11 181 individual patients from 36 hospitals were evaluated. The median age of the cohort was 6.17 (IQR, 2.36-12.11) years and 52.9% were male. Bacteremia was present in 405 encounters (1.1%, 95% CI, 1.05%-1.26%). A history of bacteremia, osteomyelitis, stroke, central line-associated bloodstream infection (CLABSI), central venous catheter, or apheresis was associated with the diagnosis of bacteremia, while age, sex, hemoglobin SC genotype, and race and ethnicity were not. In the multivariable analysis, individuals with a history of bacteremia (odds ratio [OR], 1.36; 95% CI, 1.01-1.83), CLABSI (OR, 6.39; 95% CI, 3.02-13.52), and apheresis (OR, 1.77; 95% CI, 1.22-2.55) had higher odds of bacteremia.Conclusions and relevanceThe findings of this large cohort study suggest that bacteremia in children and young adults with SCD presenting with fever is rare. A history of invasive bacterial infection, CLABSI, or a central line appears to be associated with bacteremia, while age and SCD genotype are not.
| S-EPMC10282882 | biostudies-literature