共查询到20条相似文献,搜索用时 0 毫秒
1.
2.
3.
4.
Initial Evaluation of the Pediatric PROMIS® Health Domains in Children and Adolescents With Sickle Cell Disease 下载免费PDF全文
Carlton Dampier MD Vaughn Barry PhD Heather E. Gross MEd Yang Lui PhD Courtney D. Thornburg MD Darren A. DeWalt MD Bryce B. Reeve PhD 《Pediatric blood & cancer》2016,63(6):1031-1037
5.
《Pediatric hematology and oncology》2013,30(5):432-436
We performed a retrospective chart review of children with sickle cell disease hospitalized for fever at our local institution. We reviewed 456 hospitalizations in 133 patients between January 2006 and June 2012. The prevalence of true bacteremia was 4%. The mean C-reactive protein values and temperatures were nonsignificantly higher in patients with positive blood cultures. The mean time to detection was 22.5 hours in bacteremia compared to 32.6 hours in blood cultures that grew contaminants (p = .034). Only two (0.4%) cases of pneumococcal bacteremia were reported and both occurred before May 2010, which marks the introduction of 13-valent pneumococcal vaccine (PCV13). Both patients with pneumococcal bacteremia had discontinued penicillin prophylaxis after the age of 5 years. The first patient was immunized but contracted a nonvaccine serotype (23B). The second patient was partially vaccinated and acquired a vaccine-preventable serotype (23F). Both serotypes were sensitive to ceftriaxone and vancomycin; one was resistant to penicillin. This is the first study reporting the prevalence of pneumococcal bacteremia since the introduction of PCV13. 相似文献
6.
Juanita Conkin DaleAuthor Vitae Cindy J. CochranAuthor Vitae Lonnie RoyAuthor Vitae Ethel JerniganAuthor Vitae George R. BuchananAuthor Vitae 《Journal of pediatric health care》2011,25(4):208
Objective
To assess health-related quality of life (HRQOL) in children and adolescents with sickle cell disease (SCD).Design, Setting, and Participants
The PedsQL 4.0 Generic Scales, a multidimensional self-report instrument that has been shown to be valid and reliable for use in children and adolescents with chronic illness, consists of 23 items that assess physical, emotional, social, and school functioning. Questionnaires were administered to 124 children and adolescents (ages 8 to 18 years, child self-report) with SCD (100 sickle cell anemia, 24 sickle β zero thalassemia) and their parents (parent-proxy report). Summary scores for children's and parents' ratings of overall HRQOL and psychosocial health and subscale scores for physical, emotional, social, and school functioning were compared with published data for healthy children. Both summary and subscale scores for children with SCD also were compared with those of their parents.Results
Children with SCD and their parents rated overall HRQOL and all subdomains of HRQOL lower than did healthy children and their parents (P < .001). Children with SCD rated their own HRQOL significantly better than their parents did for overall HRQOL and all subdomains (P < .001) except emotional functioning (P = .06).Conclusions
Children with SCD and their parents perceived overall HRQOL and all HRQOL subdomains to be lower than scores reported in healthy children. Therefore, successful therapeutic efforts to improve HRQOL could represent important advances in the health of children with SCD. 相似文献7.
Onyekwere OC Campbell A Teshome M Onyeagoro S Sylvan C Akintilo A Hutchinson S Ensing G Gaskin P Kato G Rana S Kwagyan J Gordeuk V Williams J Castro O 《Pediatric cardiology》2008,29(2):309-312
The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite
its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found
in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive
sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were
screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%)
SCD patients had increased PAP (i.e., TRV ≥2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV ≥3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86;
p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested
by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association
with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the
development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent
patients with SCD. 相似文献
8.
《Pediatric hematology and oncology》2013,30(3):228-232
Hybrid renal cell carcinoma (HRCN) is a rare type of renal tumor with characteristic pathologic features, including oncocytic and chromophobe cellular content, and shows more favorable prognosis than renal cell carcinoma. The early-stage tumors show favorable outcome, and postoperative regular clinical and radiological follow-up is adequate in most cases. However, close follow-up is mandatory for tumors with histopathologically coexistence of squamous, papillary, and/or eosinophilic RCC component. This report describes a 12-year-old girl with a stage I HRCN who was closely followed after nephrectomy with no further treatment. 相似文献
9.
10.
The Benefits and Challenges of Preconsent in a Multisite,Pediatric Sickle Cell Intervention Trial 下载免费PDF全文
Mark Nimmer BA Jason Czachor MS Laura Turner BA Bobbe Thomas BA CCRC Ashley L. Woodford BS Karli Carpenter BSN MSN MBA CCRC Victor Gonzalez MD Robert I. Liem MD MS Angela Ellison MD MSc T. Charles Casper PhD David C. Brousseau MD MS for the sickle cell working group of the Pediatric Emergency Care Applied Research Network 《Pediatric blood & cancer》2016,63(9):1649-1652
Enrollment of patients in sickle cell intervention trials has been challenging due to difficulty in obtaining consent from a legal guardian and lack of collaboration between emergency medicine and hematology. We utilized education and preconsent in a pediatric multisite sickle cell intervention trial to overcome these challenges. Overall, 48 patients were enrolled after being preconsented. Variable Institutional Review Board policies related to preconsent validity and its allowable duration decreased the advantages of preconsent at some sites. The utility of preconsent for future intervention trials largely depends on local Institutional Review Board policies. Preeducation may also benefit the consent process, regardless of site differences. 相似文献
11.
Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease 下载免费PDF全文
Ziad Solh MD MSc Michael S. Taccone MD Samantha Marin MD Uma Athale MD MSc Vicky R. Breakey MD MEd 《Pediatric blood & cancer》2016,63(6):983-989
Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction. 相似文献
12.
13.
《Pediatric hematology and oncology》2013,30(2):124-133
Although most common in tropical regions, population migration has meant that sickle cell disease is now one of the most prevalent genetic diseases worldwide. The issues and challenges faced by physicians and patients have been discussed by an international group of experts representing 4 key regions: the USA, Europe, Latin America, and the Middle East/Africa. Conclusive evidence to support the use of transfusion therapy for the prevention of stroke has resulted in key changes to patient management in all regions, and increasing numbers of patients are benefiting from this management approach. However, it is apparent that transfusion therapy is still under-utilized, largely due to concerns over iron overload, alloimmunization, limited blood supplies, and, sometimes, due to parental refusal. Once transfused, assessment and management of body iron levels can be poor, particularly in patients who are intermittently transfused. Compliance with chelation therapy regimens is a significant challenge, but new therapeutic options are likely to overcome some of the current barriers. Key requirements in all regions were considered to be the following: to provide greater physician, patient, and family education; to ensure effective transition from pediatric to adult care; and to establish national guidelines in order to ensure best practice is consistently applied. 相似文献
14.
Reduction in Overt and Silent Stroke Recurrence Rate Following Cerebral Revascularization Surgery in Children with Sickle Cell Disease and Severe Cerebral Vasculopathy 下载免费PDF全文
Erin M. Hall MD Jeffrey Leonard MD Jodi L. Smith PhD MD FAANS Kristin P. Guilliams MD Michael Binkley MS Robert J. Fallon MD PhD Monica L. Hulbert MD 《Pediatric blood & cancer》2016,63(8):1431-1437
15.
16.
Platinum plus bortezomib for the treatment of pediatric renal medullary carcinoma: Two cases 下载免费PDF全文
Marcus A. Carden Stephen Smith Holly Meany Hong Yin Adina Alazraki Louis B. Rapkin 《Pediatric blood & cancer》2017,64(7)
Renal medullary carcinoma (RMC) was first described over two decades ago as the seventh sickle nephropathy. Survival after diagnosis with metastatic disease still rarely extends beyond 1 year, with recent reports of median overall survival in patients treated with platinum therapy being just 10 months. We describe our experience using platinum‐based chemotherapy plus the proteasome inhibitor bortezomib to treat metastatic RMC in two pediatric patients who had complete responses. One patient passed away 7 years after diagnosis, while another remains disease free nearly 2 years from diagnosis. 相似文献
17.
Elizabeth Petri Henske Paul Thorner Kathleen Patterson Zhengping Zhuang Jay Bernstein 《Pediatric and developmental pathology》1999,2(3):270-274
We report the clinical, pathologic, and genetic features of renal malignancy in two children with diffuse cystic hyperplasia.
Both presented with massive bilateral nephromegaly. Neither had a family history or clinical findings suggestive of tuberous
sclerosis or von Hippel–Lindau disease. The kidneys of both children were extensively replaced by tubulocystic hyperplasia
with large eosinophilic epithelial cells. The masses of hyperplastic tissue were nodular, compressing remnants of uninvolved
renal parenchyma. Tubulopapillary carcinoma was present in both children, one of whom had bilateral multicentric carcinoma.
No loss of heterozygosity was detected in the tumors at the TSC1, TSC2, or VHL gene regions, and no alterations in the VHL gene were detected using single-strand conformation polymorphism analysis. These cases of bilateral renal enlargement with
diffuse cystic hyperplasia appear to represent a new clinical syndrome that may warrant bilateral nephrectomy because of the
risk of malignancy.
Received February 13, 1998; accepted May 8, 1998. 相似文献
18.
Anaplastic Ependymoma in a Child With Sickle Cell Anemia: A Case Report Highlighting Treatment Challenges for Young Children With Central Nervous System Tumors and Underlying Vasculopathy 下载免费PDF全文
Erin E. Crotty MD Emily R. Meier MD Elizabeth M. Wells MD Eugene I. Hwang MD Roger J. Packer MD 《Pediatric blood & cancer》2016,63(3):547-550
A 3‐year‐old boy with sickle cell anemia (SCA) presented with progressive daily emesis and was found to have an anaplastic ependymoma. Radiation therapy and chemotherapy are usually employed after subtotal resections of anaplastic ependymomas, although the benefits from chemotherapy are unclear. To mitigate the risks of adjuvant treatment in this patient at risk for SCA‐associated vasculopathy, renal impairment, and other end‐organ damage, proton beam irradiation without chemotherapy was chosen. Scheduled packed red blood cell transfusions were instituted to maintain sickle hemoglobin levels less than 30%. This case highlights treatment complexities for malignant brain tumors in patients predisposed to treatment‐related adverse effects. 相似文献
19.
Introduction: Angiogenesis has been investigated in different kinds of anemia. However, its role as a marker of angiogenesis has not been investigated in thalassemia or sickle cell disease (SCD). Objectives: We aimed to investigate serum angiogenin level in children and adolescents with beta thalassemia or SCD and its relation to possible risk factors of angiogenesis. Materials and Methods: This study included; 32 β-thalassemia major (β-TM) patients aged 14.2 ± 3.8 years, 20 β-thalassemia intermedia (β-TI) patients aged 14.3 ± 4.8 years, 20 SCD patients aged 14.1 ± 2.4 years; 8 with (HbSS) and 12 with sickle thalassemia (HbS/β-thalassemia) and 35 age and sex-matched controls. Data collected regarding; age, sex, disease duration, blood transfusion frequency, transfusion index, chelation type and duration, CBC, Hb electrophoresis, serum ferritin and serum angiogenin level (by ELISA). Results: Angiogenin level was significantly higher in patients with SCD [250 (100–300) pg/mL] compared to β-TM [180 (140–230) pg/mL] and controls [89 (80–103) pg/mL] (P < .001) especially those with HbSS (P = .06). There was a significant negative correlation between serum angiogenin and age of patients, age of onset and duration of chelation in β-TM (P < .01, P < .001, P = .003) and β-TI (P = .009, P = .03, P < .001) and with serum ferritin in β-TI group (r = ?0.573, P = .008). In SCD, angiogenin level was negatively correlated with both frequency of blood transfusion (r = ?0.731, P < .001) and duration of hydroxyurea therapy (P = .017). Conclusions: High angiogenin level detected among patients with SCD may be negatively influenced by regular blood transfusion and hydroxyurea therapy, while; early onset of chelation therapy may decrease angiogenin level in β-TM. 相似文献
20.
Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity 下载免费PDF全文
David J. Martyres BSc Abi Vijenthira MD Nick Barrowman PhD Sydney Harris‐Janz RD Christine Chretien RN Robert J. Klaassen MD FRCPC 《Pediatric blood & cancer》2016,63(6):1060-1064