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2.
Annu Int Conf IEEE Eng Med Biol Soc ; 2020: 5838-5841, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-33019301

RESUMO

Sickle Cell Disease (SCD) is a hereditary disorder of red blood cells in humans. Complications such as pain, stroke, and organ failure occur in SCD as malformed, sickled red blood cells passing through small blood vessels get trapped. Particularly, acute pain is known to be the primary symptom of SCD. The insidious and subjective nature of SCD pain leads to challenges in pain assessment among Medical Practitioners (MPs). Thus, accurate identification of markers of pain in patients with SCD is crucial for pain management. Classifying clinical notes of patients with SCD based on their pain level enables MPs to give appropriate treatment. We propose a binary classification model to predict pain relevance of clinical notes and a multiclass classification model to predict pain level. While our four binary machine learning (ML) classifiers are comparable in their performance, Decision Trees had the best performance for the multiclass classification task achieving 0.70 in F-measure. Our results show the potential clinical text analysis and machine learning offer to pain management in sickle cell patients.


Assuntos
Dor Aguda , Anemia Falciforme , Dor Aguda/diagnóstico , Anemia Falciforme/complicações , Contagem de Eritrócitos , Humanos , Manejo da Dor , Medição da Dor
3.
Niger J Clin Pract ; 23(10): 1426-1430, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33047701

RESUMO

Background: Osteoarthritis secondary to osteonecrosis of the femoral head is a common presentation in patients with sickle cell disease. Functional limitations with or without deformities from these complications of sickle cell disease often require Total Hip Arthroplasty (THA) to improve outcome. Aim: The aim of this study was to evaluate the postoperative outcome of THA for secondary osteoarthritis from osteonecrosis of the head of the femur in patients with sickle cell disease. Methodology: A retrospective study of outcome of THA in sickle cell disease patients between January 2010 and December 2015 is presented. Primary outcome was measured using the rates of complications and the Harris Hip scores at 6 weeks, 12 weeks, 6 months, and yearly. Results: A total of 68 THA in 56 patients with sickle cell disease was reviewed. The age range was 13-68 years, with a mean of 30.9 years. There were 15 males and 41 females (M: F ratio = 1:3). Approaches to the hip were direct lateral (64.3%), anterolateral (1.8%), and posterior (33.9%). 12 (21.4%) patients had bilateral total hip arthroplasty, and 44 (78.6%) had unilateral total hip arthroplasty. No patient required adductor tenotomy. Superficial surgical site infection was recorded in 7 (12.5%) patients. The postoperative functional outcome, measured by the Harris Hip score, was good or excellent in all patients, except 2 (2.9%) who required revision surgery following unacceptable results after primary hip arthroplasty. Conclusion: THA is a viable and effective treatment modality for sickle cell disease patients with osteonecrosis and secondary hip osteoarthritis. The choice of surgical approach does not affect the outcome.


Assuntos
Anemia Falciforme/complicações , Artroplastia de Quadril/métodos , Necrose da Cabeça do Fêmur/cirurgia , Osteoartrite do Quadril/cirurgia , Reoperação , Adolescente , Adulto , Idoso , Artroplastia de Quadril/psicologia , Feminino , Fêmur , Necrose da Cabeça do Fêmur/etiologia , Necrose da Cabeça do Fêmur/psicologia , Humanos , Masculino , Pessoa de Meia-Idade , Osteonecrose/etiologia , Complicações Pós-Operatórias , Período Pós-Operatório , Qualidade de Vida/psicologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
4.
J Opioid Manag ; 16(4): 267-275, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32885834

RESUMO

OBJECTIVE: Vaso-occlusive crisis is the most common clinical feature requiring opioid analgesics in patients with sickle cell disease. We conducted a network meta-analysis to compare the drugs that can be used as add-on with opioids for vaso-occlusive crisis. DESIGN: Network meta-analysis of randomized clinical trials. PATIENTS: Sickle cell disease patients with vaso-occlusive crisis receiving adjuvants to opioids for pain management. MAIN OUTCOME MEASURES: A number of patients with complete pain relief and pain scores assessed either by visual ana-log or by a numerical rating scale were the primary outcomes. Adverse events and dose of opioids (in morphine equiva-lents) for pain alleviation between the treatment arms were the secondary outcome measures. RESULTS: Eleven studies evaluating the addition of ketorolac, magnesium sulfate, ketoprofen, ibuprofen, methadone, inhalational nitric oxide, methylprednisolone, and arginine with morphine were obtained. The pooled analysis showed a favorable effect in the pain reduction for the additions of arginine {-2 [-3.39, -0.61]} and ibuprofen {-1.7 [-3.26, -0.14]} with morphine. Arginine has high probability of being the "best" in the pool followed by ibuprofen. No significant differ-ences were observed in the risk of adverse events {ketoprofen-0.84 [0.42, 1.65]; magnesium sulfate-1.81 [0.64, 5.81]; and arginine-2.08 [0.18, 24.31]}. A significant lower dose of opioid was required when given adjunctive to argin-ine, inhalational nitric oxide, and methylprednisolone. CONCLUSION: We observed that arginine and ibuprofen could produce additional analgesic effects when combined with morphine in vaso-occlusive crisis.


Assuntos
Analgésicos Opioides , Anemia Falciforme , Manejo da Dor , Analgésicos Opioides/uso terapêutico , Anemia Falciforme/complicações , Humanos , Morfina , Metanálise em Rede , Medição da Dor
5.
Ann Emerg Med ; 76(3S): S12-S20, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928457

RESUMO

STUDY OBJECTIVE: This was a prospective, pre-post, 13-year observational study documenting the multiyear implementation of an observation unit sickle cell pathway for patients with uncomplicated vaso-occlusive events. METHODS: The sickle cell pathway begins with rapid triage to identify patients with uncomplicated vaso-occlusive events for immediate transfer to the observation unit and initiation of patient-controlled analgesia followed by repeated evaluations of pain and identification of other complications. Data were abstracted from the electronic medical record or observation unit database. The sickle cell pathway was initiated in April 2006. Major revisions of it were carried out in June 2009 (physician evaluation occurs in sickle cell pathway and only patient-controlled analgesia administration of medications) and October 2010 (multidisciplinary management and individual dosing). RESULTS: Annual ED visits ranged between 287 and 528. The preimplementation hospital admission rate was 33% (123/368), 3-day return rate 16% (60/368), and 30-day return rate 67% (248/368). Refinements to the sickle cell pathway have resulted in a decrease in admission rate to 20% (258/1276); 3-day return rate, to 3.6% (46/1,276); and 30-day return rate, to 41% (525/1,276) for the past 3 years. CONCLUSION: The use of a sickle cell pathway for the treatment of uncomplicated vaso-occlusive events has been effective in providing rapid treatment and reducing hospital admissions. However, it was not only the intervention and its refinement that made the sickle cell pathway successful. With the Consolidated Framework for Implementation Research, it was discerned that outer setting factors of organizational commitment to the care of patients with SCD, inner setting factors of learning climate and leadership engagement, individuals, and process contributed to the success of the sickle cell pathway.


Assuntos
Analgesia Controlada pelo Paciente/métodos , Anemia Falciforme/terapia , Unidades de Observação Clínica , Serviço Hospitalar de Emergência , Dor Aguda/tratamento farmacológico , Dor Aguda/etiologia , Adolescente , Adulto , Idoso , Anemia Falciforme/complicações , Estudos Controlados Antes e Depois , Procedimentos Clínicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Manejo da Dor/métodos , Estudos Prospectivos , Triagem , Doenças Vasculares/etiologia , Adulto Jovem
6.
Ann Emerg Med ; 76(3S): S21-S27, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928458

RESUMO

STUDY OBJECTIVE: Sickle cell disease (SCD) is an inherited hematologic disorder that affects approximately 100,000 US individuals and results in greater than 200,000 emergency department (ED) visits annually in the United States, with pain being the most common complaint. The objective of this retrospective study is to determine the effect of implementing individualized pain plans in the treatment of patients with SCD in the ED on time to first opioid, length of stay, and disposition. METHODS: At The Ohio State University Wexner Medical Center, a multidisciplinary group including hematologists and ED physicians was formed and enacted a protocol for using individualized pain plans, with the goal of decreasing time to treatment for patients with SCD who presented to the ED with chief complaint of pain. In this retrospective study, data from the year before through the year of implementation were gathered. Generalized linear models were fit to compare time to first opioid, length of stay, and disposition before and after protocol implementation. RESULTS: Data showed a 48% decrease in time to first opioid and a 22% decrease in length of ED stay after protocol implementation. No significant change was found in disposition or length of inpatient admission before and after protocol initiation. CONCLUSION: The use of individualized pain plans in the treatment of patients with SCD in the ED is a useful method of not only ensuring rapid and adequate treatment but also decreasing use of health care resources.


Assuntos
Dor Aguda/terapia , Anemia Falciforme/terapia , Serviço Hospitalar de Emergência , Manejo da Dor/métodos , Medicina de Precisão/métodos , Dor Aguda/etiologia , Adulto , Idoso , Analgésicos Opioides/administração & dosagem , Analgésicos Opioides/uso terapêutico , Anemia Falciforme/complicações , Estudos Controlados Antes e Depois , Feminino , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Ohio , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
7.
Ann Emerg Med ; 76(3S): S28-S36, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928459

RESUMO

STUDY OBJECTIVE: We provide an updated assessment of trends in sickle cell disease (SCD)-related mortality, a significant source of mortality in the United States among black persons, using 1979 to 2017 US mortality data. METHODS: SCD-related deaths were identified with International Classification of Diseases codes. Because SCD-related death is rare in other races, the analysis focused on black decedents. Age-specific and average annual SCD-related death rates were calculated. Causes of death codes were categorized into 20 groups relevant to SCD outcomes. SCD-related deaths were compared with non-SCD-related deaths after matching on race, sex, age group, and year of death. RESULTS: There were 25,665 SCD-related deaths reported among blacks in the United States from 1979 through 2017. During that period, the annual SCD-related death rate declined in children and increased in adults, and the median age at death increased from 28 to 43 years. Acute causes of death, such as infection and cerebrovascular complications, were more common in younger age groups. Chronic complications were more common in adults. SCD-related deaths were more likely to be related to acute cardiac, pulmonary, and cerebrovascular complications; acute infections; and chronic cardiac and pulmonary complications and renal disorders; and less likely to be related to drug overdose and chronic infections than non-SCD-related deaths. CONCLUSION: These data indicate SCD-related deaths are now more likely to be related to chronic complications of the disease than to acute complications. More research regarding prevention and treatment of chronic complications of SCD is necessary because persons with SCD are living longer.


Assuntos
Anemia Falciforme/mortalidade , Adolescente , Adulto , Fatores Etários , Anemia Falciforme/complicações , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/mortalidade , Causas de Morte , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , Estados Unidos/epidemiologia , Adulto Jovem
8.
Ann Emerg Med ; 76(3S): S37-S45, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928460

RESUMO

STUDY OBJECTIVE: We determine the association between use of specific cephalosporins and macrolides and hospital length of stay in patients with sickle cell disease (SCD) who are admitted with acute chest syndrome, and determine treatment risk factors for acute chest syndrome-related 30-day readmission. METHODS: Patients admitted to 48 US hospitals within the Pediatric Health Information System between January 2008 and December 2016 with associated International Classification of Diseases, Ninth Revision (ICD-9) or ICD-10 diagnoses of SCD and acute chest syndrome were included. Primary outcomes were hospital length of stay and acute chest syndrome-related and all-cause 30-day readmission. Data were analyzed with t tests, ANOVA, and bivariable and multivariable linear and logistic regressions. RESULTS: In 21,126 visits (representing 8,856 patients), median age was 11.2 years (interquartile range 6.1 to 16.5 years), 53.5% were male patients, and 77.2% had hemoglobin SS genotype. Median length of stay was 4 days (interquartile range 2 to 6 days; mean 4.76 days [SD 4.62 days]). Ceftriaxone alone (length of stay 4.75 days [SD 4.66 days]; P<.001) or the combination of ceftriaxone and azithromycin (length of stay 4.84 days [SD 4.74 days]; P<.001) was associated with the shortest length of stay and a reduced risk of acute chest syndrome-related readmission (ceftriaxone odds ratio [OR] 0.31; 95% confidence interval [CI] 0.27 to 0.35; ceftriaxone+azithromycin OR 0.20; 95% CI 0.17 to 0.24). Albuterol (OR 0.97; 95% CI 0.96 to 0.98) and RBC transfusion (OR 0.60; 95% CI 0.43 to 0.83) were also associated with decreased rates of acute chest syndrome-related 30-day readmission. All-cause 30-day readmission rate was 16.7% (95% CI 16.2% to 17.3%). CONCLUSION: Guideline-compliant therapy for acute chest syndrome could preferentially include ceftriaxone and azithromycin. All-cause 30-day readmission for acute chest syndrome is lower than that reported for all-cause readmissions for SCD and more consistent with rates of readmission for pneumonia in the general population.


Assuntos
Síndrome Torácica Aguda/tratamento farmacológico , Anemia Falciforme/tratamento farmacológico , Antibacterianos/uso terapêutico , Tempo de Internação/estatística & dados numéricos , Readmissão do Paciente/estatística & dados numéricos , Síndrome Torácica Aguda/etiologia , Adolescente , Anemia Falciforme/complicações , Cefalosporinas/uso terapêutico , Criança , Feminino , Humanos , Modelos Lineares , Modelos Logísticos , Macrolídeos/uso terapêutico , Masculino , Estudos Retrospectivos , Fatores de Risco , Estados Unidos
9.
Ann Emerg Med ; 76(3S): S46-S55, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928462

RESUMO

STUDY OBJECTIVE: Acute chest syndrome is a leading cause of mortality in patients with sickle cell disease (SCD). Because early detection of acute chest syndrome is directly tied to prognosis, young patients with SCD undergo countless chest radiography screenings throughout their lifetime for commonly occurring acute chest syndrome risk factors such as fever, chest pain, or cough. Chest radiography is not an ideal screening method because it is associated with radiation exposure, which accumulates with repeated imaging. Point-of-care lung ultrasonography is a nonradiating imaging modality that has been used to identify other lung pathology and may have a role in SCD. The goal of this study was to determine the accuracy of point-of-care lung ultrasound to identify an infiltrate suggestive of acute chest syndrome in patients with SCD compared to chest radiography as the gold standard. METHODS: This was a prospective observational study in 2 urban pediatric emergency departments to evaluate the accuracy of point-of-care lung ultrasonography in identifying patients with SCD who were aged 0 to 21 years and had an infiltrate suggestive of acute chest syndrome compared with chest radiography. Clinicians and trainees with point-of-care lung ultrasonographic training obtained informed consent and performed investigational point-of-care lung ultrasonography to evaluate for lung consolidation. A blinded point-of-care lung ultrasonographic expert reviewed results for quality assurance and agreement. Accuracy, sensitivity, specificity, likelihood ratios, and positive and negative predictive value were calculated for point-of-care lung ultrasonography test performance characteristics, with chest radiography as a reference standard. RESULTS: Point-of-care lung ultrasonography was performed on 191 SCD patients with a mean age of 8 years; 41% were female patients, and there was a 17% prevalence of acute chest syndrome. Accuracy of point-of-care lung ultrasonography to detected acute chest syndrome was 92%, sensitivity was 88%, and specificity was 93% compared with that for chest radiography. CONCLUSION: Point-of-care lung ultrasonography is a feasible alternative to chest radiography for screening for acute chest syndrome in young patients with SCD. Further studies are needed to determine how this test performs within clinical practice.


Assuntos
Síndrome Torácica Aguda/diagnóstico por imagem , Anemia Falciforme/complicações , Testes Imediatos , Ultrassonografia , Síndrome Torácica Aguda/diagnóstico , Síndrome Torácica Aguda/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Radiografia Torácica , Reprodutibilidade dos Testes , Ultrassonografia/métodos
10.
Ann Emerg Med ; 76(3S): S6-S11, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928464

RESUMO

STUDY OBJECTIVE: The National Heart, Lung, and Blood Institute evidence-based guidelines for timeliness of opioid administration for sickle cell disease (SCD) pain crises recommend an initial opioid within 1 hour of arrival, with subsequent dosing every 30 minutes until pain is controlled. No multisite studies have evaluated guideline adherence, to our knowledge. Our objective was to determine guideline adherence across a multicenter network. METHODS: We conducted a multiyear cross-sectional analysis of children with SCD who presented between January 1, 2016, and December 31, 2018, to 7 emergency departments (EDs) within the Pediatric Emergency Care Applied Research Network. Visits for uncomplicated pain crisis were included, defined with an International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 code for SCD crisis and receipt of an opioid, excluding visits with other SCD complications or temperature exceeding 38.5°C (101.3°F). Times were extracted from the electronic record. Guideline adherence was assessed across sites and calendar years. RESULTS: A total of 4,578 visits were included. The median time to first opioid receipt was 62 minutes (interquartile range 42 to 93 minutes); between the first and second opioid receipt, 60 minutes (interquartile range 39 to 93 minutes). Overall, 48% of visits (95% confidence interval 47% to 50%) were guideline adherent for first opioid. Of 3,538 visits with a second opioid, 15% (95% confidence interval 14% to 16%) were guideline adherent. Site variation in adherence existed for time to first opioid (range 22% to 70%) and time between first and second opioid (range 2% to 36%; both P<.001). There was no change in timeliness to first dose or time between doses across years (P>.05 for both). CONCLUSION: Guideline adherence for timeliness of SCD treatment is poor, with half of visits adherent for time to first opioid and one seventh adherent for second dose. Dissemination and implementation research/quality improvement efforts are critical to improve care across EDs.


Assuntos
Analgésicos Opioides/uso terapêutico , Anemia Falciforme/terapia , Fidelidade a Diretrizes/estatística & dados numéricos , Manejo da Dor/métodos , Dor Aguda/tratamento farmacológico , Dor Aguda/etiologia , Adolescente , Analgésicos Opioides/administração & dosagem , Anemia Falciforme/complicações , Estudos Transversais , Serviço Hospitalar de Emergência/normas , Serviço Hospitalar de Emergência/estatística & dados numéricos , Feminino , Humanos , Masculino , Manejo da Dor/normas , Sistema de Registros , Fatores de Tempo , Estados Unidos
12.
BMC Infect Dis ; 20(1): 638, 2020 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-32854639

RESUMO

BACKGROUND: Sickle cell disease (SCD) is a multisystem disorder characterized by a wide spectrum of clinical manifestations and severity. Studies investigating potential effects of co-morbid human immunodeficiency virus (HIV) and SCD have produced conflicting results, and additional investigations are needed to elucidate whether the interaction between the two disease states might impact both HIV and SCD clinical outcomes. The association of HIV infection with clinical and laboratory characteristics of patients with SCD was assessed. METHODS: This nested case-control study included individuals with SCD with HIV treated at six Brazilian SCD centers. Clinical and laboratory data were abstracted from medical records. HIV positive participants were compared to age, gender, center, and SCD genotype matched HIV negative participants (ratio 1:4). Individual clinical outcomes as well as a composite outcome of any SCD complication and a composite outcome of any HIV-related complication were compared between the two groups. RESULTS: Fifteen HIV positive participants were included, 12 (80%) alive and 3 (20%) deceased. Most of the HIV positive patients had HbSS (60%; n = 9), 53% (n = 8) were female, and mean age was 30 ± 13 years. The frequency of individual SCD complications of acute chest syndrome/pneumonia, sepsis/bacteremia, pyelonephritis, ischemic stroke, hemorrhagic stroke, abnormal transcranial Doppler (TCD), and pulmonary hypertension was higher in HIV positive participants when compared to HIV negative, although analyzed individually none were statistically significant. HIV positive participants had significantly higher risk of any SCD complication and of a composite HIV-related complication compared to the HIV negative group (HR = 4.6; 95%CI 1.1-19.6; P = 0.04 and HR = 7.7; 95%CI 1.5-40.2; P = 0.02, respectively). There was a non-significant trend towards higher risk of any infections in participants with HIV positive (HR = 3.5; 95%CI 0.92-13.4; P = 0.07). Laboratory parameters levels were not significantly different in individuals with and without HIV. CONCLUSIONS: In summary, our study in SCD patients shows that those with HIV have an increased risk of any SCD complication and HIV-related complications, as well as a suggestive but not significantly increased risk of infections.


Assuntos
Anemia Falciforme/complicações , Infecções por HIV/complicações , Adolescente , Adulto , Brasil , Estudos de Casos e Controles , Feminino , Genótipo , Humanos , Masculino , Adulto Jovem
14.
West Afr J Med ; 37(4): 412-417, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32835405

RESUMO

BACKGROUND: Sickle cell anaemia (SCA), one of the causes of morbidity and mortality in children is associated with a large spectrum of systemic complications including sickle cell nephropathy (SCN). Microalbuminuria has been used as a marker of preclinical glomerular damage in these patients. This study aimed at detecting early, renal injury in children with SCA aged 1-17 years, highlighting associations and predictors of microalbuminuria in these children. METHODS: 102 known HbSS children aged 1-17yrears in steady state were recruited into a cross-sectional study. Socio-demographic and clinical findings were recorded. Albuminuria was assayed with spot urine using a quantitative method. Urine creatinine concentration was estimated using the Roche reflotron test strips for quantitative determination of creatinine in blood, serum, plasma and urine. Albumin to creatinine ratio (ACR) was then calculated. Microalbuminuria was defined as ACR of 30-300mg/g. RESULTS: Microalbuminuria was detected in 22.5% of SCA patients in our cohort. Age (p=0.001), gender (p=0.000), packed cell volume (p=0.047) showed a significant relationship with the occurrence of microalbuminuria in this study. Increasing age (OR=1.72, CI=1.22-2.44, p=0.002), female gender (OR=0.09, CI=0.01-0.95, p=0.04) and lower packed cell volume (OR=0.49, CI=0.26-0.90, p=0.02) emerged as independent risk factors associated with the occurrence of microalbuminuria in the study population. CONCLUSION: Renal injury occurs in a high proportion of patients with SCA. Routine screening of all patients with SCA as part of their follow up is therefore recommended to identify patients with early renal injury for proper management.


Assuntos
Albuminúria , Anemia Falciforme , Adolescente , Albuminúria/etiologia , Anemia Falciforme/complicações , Criança , Pré-Escolar , Creatinina , Estudos Transversais , Feminino , Humanos , Lactente , Rim , Masculino , Nigéria
15.
Niger J Clin Pract ; 23(8): 1079-1086, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32788485

RESUMO

Background: Poor growth and nutritional status are common features of sickle cell anemia (SCA) in children. The rising trend of obesity in children in developing countries has been reported despite a huge burden of undernutrition in these settings. In SCA, overweight/obesity is being increasingly reported. Aims: To evaluate the nutritional status and its determinants in children with SCA and to compare the same with hemoglobin AA (HbAA) controls of similar age, gender, and socioeconomic status. Methods: The study was a cross-sectional analytical study involving 175 subjects and controls aged 1-18 years who met the inclusion criteria. Weight and height were measured and body mass index (BMI) was calculated. Z scores were computed for the anthropometric measurements using the World Health Organization (WHO) standard reference. Hemoglobin concentration was determined using HemoCue Hb201+ Analyzer. Results: Subjects had significantly lower Z- scores for weight, height, and BMI compared with controls. Stunting, wasting, and overweight/obesity were observed in 10.9%, 24.6%, and 5.1% of subjects compared with 2.3%, 5.7%, and 9.7% respectively in controls. Wasting, stunting and overweight/obesity in SCA were significantly associated with age while overweight/obesity was significantly associated with upper social class (P = 0.001). Conclusions: Poor growth and nutritional status are still prevalent while overweight and obesity are emerging comorbidities among children with SCA in our environment. Regular nutritional assessment of children with SCA should be encouraged while those at risk of under/over-nutrition should receive adequate nutritional rehabilitation to prevent possible complications.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Desnutrição/epidemiologia , Estado Nutricional , Obesidade Pediátrica/epidemiologia , Adolescente , Índice de Massa Corporal , Peso Corporal , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Transversais , Feminino , Transtornos do Crescimento/complicações , Humanos , Lactente , Masculino , Nigéria/epidemiologia , Avaliação Nutricional , Sobrepeso/complicações , Sobrepeso/epidemiologia , Obesidade Pediátrica/complicações , Prevalência , Classe Social , Fatores Socioeconômicos
16.
PLoS One ; 15(8): e0236998, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32790687

RESUMO

There are over 12,000 people with sickle cell disease (SCD) in the UK, and 4-12% of patients who develop Sickle Cell Nephropathy (SCN) progress to End Stage Renal Disease (ESRD). Renal transplantation offers the best outcomes for these patients with but their access to transplantation is often limited. Regular automated exchange blood transfusions (EBT) reduce the complications of SCD and may improve outcomes. However, concerns over alloimmunisation limit its widespread implementation. In this retrospective multicenter study, data were collected on 34 SCD patients who received a kidney transplant across 6 London Hospitals between 1997 and 2017. 20/34 patients were on an EBT program, pre or post renal transplantation. Overall patient and graft survival were inferior to contemporaneous UK data in the ESRD population as a whole, a finding which is well-recognised. However, patient survival (CI 95%, p = 0.0032), graft survival and graft function were superior at all time-points in those who received EBT versus those who did not. 4/20 patients (20%) on EBT developed de novo donor specific antibodies (DSAs). 3/14 patients (21%) not on EBT developed de novo DSAs. The incidence of rejection in those on EBT was 5/18 (28%), as compared with 7/13 (54%) not on EBT. In conclusion, our data, while limited by an inevitably small sample size and differences in the date of transplantation, do suggest that long-term automated EBT post renal transplant is effective and safe, with improvement in graft and patient outcomes and no increase in antibody formation or graft rejection.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/cirurgia , Transfusão Total , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Adulto , Anemia Falciforme/terapia , Terapia Combinada , Feminino , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Falência Renal Crônica/terapia , Londres , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
17.
Soins Pediatr Pueric ; 41(314): 35-41, 2020.
Artigo em Francês | MEDLINE | ID: mdl-32771206

RESUMO

The effect of a standardized musical intervention for adolescents with sickle cell disease was studied. Two groups were evaluated using the visual analog scale of pain and the anxiety-state inventory before and after a standardized musical intervention or breathing intervention. A significant decrease in scores was observed, most notably for the group benefiting from the standardized musical intervention. This intervention could be integrated into the overall management of adolescents with sickle cell disease.


Assuntos
Anemia Falciforme/terapia , Ansiedade/prevenção & controle , Musicoterapia , Manejo da Dor/métodos , Dor/prevenção & controle , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/psicologia , Ansiedade/etiologia , Humanos , Dor/etiologia , Resultado do Tratamento
18.
Pan Afr Med J ; 36: 81, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32774640

RESUMO

Sickle cell disease is a major concern of public health significance in Africa. Nearly 2/3rd of the global burden of sickle cell disease (SCD) is found to be in sub-Saharan Africa. There is increased mortality risk in sickle cell disease patients in Africa due to associated complications such as acute chest syndrome, asthma, pulmonary emboli and sepsis. Sickle cell disease management is the major contributor of financial burden on the government. Moreover, there is a shortage of medical specialists in Africa. COVID-19 pandemic has further led to devastating impact on economy and health globally. The chances of SCD patient contracting COVID-19 infections are higher as these patients are immunocompromised and may be at a higher risk of mortality. Practicing preventive measures including isolation and social distancing by these patients will prevent mortality rates as well as economic burden on government in the present unprecedented COVID-19 pandemic.


Assuntos
Anemia Falciforme/epidemiologia , Infecções por Coronavirus/epidemiologia , Efeitos Psicossociais da Doença , Pneumonia Viral/epidemiologia , África ao Sul do Saara/epidemiologia , Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Infecções por Coronavirus/economia , Infecções por Coronavirus/prevenção & controle , Humanos , Hospedeiro Imunocomprometido , Pandemias/economia , Pandemias/prevenção & controle , Pneumonia Viral/economia , Pneumonia Viral/prevenção & controle , Saúde Pública , Isolamento Social
19.
Ann R Coll Surg Engl ; 102(9): e1-e2, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32777927

RESUMO

Haemoglobin SC (HbSC) disease accounts for 30% of cases of sickle cell disease in the United Kingdom and the United States. Unlike other sickle cell carriers, who are relatively asymptomatic, people with HbSC disease have a combination of genotypes with the potential to cause considerable morbidity due to intracellular water loss. Patients can present with acute pain, acute chest syndrome, proliferative retinopathy, splenic and renal complications, or stroke. We present a young man with HbSC disease who developed acute compartment syndrome. This is only the second report of this syndrome in a patient with HbSC disease. This is a very rare complication in HbSC disease, but it can have serious implications.


Assuntos
Anemia Falciforme/complicações , Hipertensão Intra-Abdominal/etiologia , Dor Abdominal/etiologia , Doença Aguda , Anemia Falciforme/patologia , Evolução Fatal , Humanos , Hipertensão Intra-Abdominal/diagnóstico , Hipertensão Intra-Abdominal/patologia , Masculino , Adulto Jovem
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