scholarly journals Abnormal Pulmonary Function and Associated Risk Factors in Children and Adolescents With Sickle Cell Anemia

2014 ◽  
Vol 36 (3) ◽  
pp. 185-189 ◽  
Author(s):  
Manuel Arteta ◽  
Andrew Campbell ◽  
Mehdi Nouraie ◽  
Sohail Rana ◽  
Onyinye C. Onyekwere ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Function ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Associated Risk Factors ◽  
Abnormal Pulmonary Function

Epidemiology and Risk Factors for Pain In Children and Adolescent with Sickle Cell Anemia.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1651-1651 ◽  
Author(s):  
Deepika Darbari ◽  
Onyinye Onyekwere ◽  
Mehdi Nouraie ◽  
Gregory J. Kato ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Risk Factors ◽  
Confidence Interval ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Odds Ratio ◽  
Severe Pain ◽  
History Of ◽  
Pain Crisis ◽  
Thalassemia Trait

Abstract Abstract 1651 Background: Pain crises are the most common symptom experienced by individuals with sickle cell anemia (SCA). Frequency of pain crises varies significantly and high rate is a risk factor for higher mortality in adults with SCA. The risk factors for pain crises in children and adolescents with SCA are not completely understood. To determine factors associated with frequent severe pain crises, we analyzed the cohort of children and adolescents with SCA who were enrolled in the prospective study “The Pulmonary Hypertension and the Hypoxic Response in SCD (PUSH)”. All children were evaluated in their steady, non-crisis state. Methods: Family-reported history of number of severe pain crises in the preceding 12 months was recorded prospectively in 365 children and adolescents with SCA. Severe pain crises were defined as painful vaso-occlusive episodes requiring evaluation in Emergency Department (ED) or in-patient hospitalization. Lifetime history of red cell transfusions, echocardiography, and laboratory studies were obtained. Clinical and laboratory characteristics of study subjects who had ≥3 severe pain crises in the preceding year were compared to subjects with < 3 severe pain crises. Results: Study subject ranged in age from 3–20 years and 175 (48%) were female. Seventy two children (20 %) had ≥3 severe pain crises in the preceding year (frequent pain crisis group); 293 (80%) children had < 3 severe pain crises (infrequent pain crisis group), including 224 (61%) subjects who had no admissions/ ED visits for pain. Associated factors for frequent pain crisis included older age (odds ratio 1.2; 95% confidence interval 1.12–1.35; P <0.001) and α-thalassemia trait (odds ratio 3.22; 95% confidence interval 1.55–6.69; P =0.002) while higher steady state serum lactate dehydrogenase (LDH) was associated with infrequent pain crisis (odds ratio 0.35; 95% confidence interval 0.13–0.98; P =0.045). In a group of patients without α-thalassemia trait, older age and low LDH were linked to frequent pain crisis. Subjects in the frequent pain crisis group had higher median hemoglobin (9.0 vs. 8.5 gm/dL; p=0.003) and higher ferritin (median 455 vs. 191 ng/mL; p=0.008). Higher ferritin in the frequent pain crisis group was mirrored by the higher percentage who reported >10 lifetime transfusions (42% vs. 22%; p=0.001). Median tricuspid regurgitation jet velocity (TRV) was higher in the frequent pain crisis group (2.41 vs. 2.31; p= 0.001) but the proportion of children with TRV>2.5 was not different (19.4% vs.11.5% in infrequent crises group; p=0.09). Hydroxyurea use was not different between the groups (51% vs. 40%; p=0.08) nor was fetal hemoglobin (10% vs. 12%; p=0.2). Conclusions: The occurrence of severe pain crisis varies among children and adolescents with SCA with a large number of children experiencing no severe painful episodes. Consistent with the Cooperative Study of Sickle Cell Disease report, the risk of severe pain crisis increases with age. Individuals with α-thalassemia trait are likely to experience more frequent pain crises possibly related to higher hemoglobin concentration and viscosity. However, even after controlling for α-thalassemia trait, children and adolescents who reported more frequent severe pain crises showed evidence of less hemolysis, consistent with a hypothetical model associating the hemolytic subphenotype of SCA with less frequent vasoocclusive pain crises. Further studies are indicated to identify the molecular mechanisms of pain in sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.

Pulmonary Function in Children with Sickle Cell Anemia Following Treatment with Hydroxyurea.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1435-1435 ◽  
Author(s):  
Elizabeth Record ◽  
Tamara New ◽  
Randall Brown ◽  
LeRoy Graham ◽  
R. Clark Brown
Keyword(s):  
Pulmonary Function ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pulmonary Function Tests ◽  
Pulmonary Complications ◽  
Acute Chest Syndrome ◽  
P Value ◽  
Chronic Injury ◽  
History Of ◽  
Abnormal Pulmonary Function

Abstract Background: Pulmonary function is particularly susceptible to acute and chronic injury occurring in patients with sickle cell anemia (SCA). Acute Chest Syndrome (ACS), a common and potentially fatal complication of SCA, can be the cause or the consequence of abnormal pulmonary function (Siddiqui & Ahmed, 2003). Use of hydroxyurea therapy (HU) is increasing for children with recurrent pulmonary complications, following the findings that HU reduces risk of ACS in adults (Charache et al, 1995). How HU may improve pulmonary function in children with SCA is still ill-defined. The purpose of this study was to assess the change observed on serial pulmonary function tests (PFT) for children prescribed HU for abnormal pulmonary function. Methods: Over 240 children with pulmonary complications, such as acute chest syndrome, reactive airway disease, and chronic hypoxia, have been evaluated in the Sickle Cell Pulmonary Clinic at Children’s Healthcare of Atlanta since beginning in July 2000. As part of the Clinic’s standard of care, PFT were routinely attempted on children over the age of 5. A retrospective review of PFT results was done for the children with SCA and prior abnormal PFT. Children were included in the HU cohort (+HU) if repeat PFTs were available following &gt; 3 months on HU therapy and if time on HU did not include frequent RBC transfusions. Children without history of HU therapy (−HU) were selected for comparison. PFTs were performed on a standard plethysmograph. Paired t-test was used to evaluate observed differences. Results: Thirty-one children with prior abnormal PFT had test before and following prescription of HU. Their mean age was 12.6 (range 6–20) years and mean duration of HU was 21 (range 4–47) months at the time of the repeat PFT. Hematologic changes expected on HU occurred for all 31 children. Twenty-four children followed for abnormal PFT had no history of HU (−HU) and matched the +HU group by gender, age (mean 12.3, range 7–19 years), duration followed at time of repeat PFT (mean 19, range 3–66 months; p=0.36), and initial hematologic parameters. Spirometry findings changed for +HU group, and remained stable for −HU group. Mean FVC and FEV1 values improved significantly on HU, when compared to initial (PreHU) PFT and to −HU controls. Conversion to a normal PFT, as interpreted by a pediatric pulmonologist masked to treatment status, occurred in 18 (58%) and 2 (8%) children in the +HU and −HU groups, respectively. Table 1: Serial PFT and hematologic parameters according to HU exposure −HU (n=24) +HU (n=31) Initial Repeat # PreHU Repeat 1 % predicted value for age, sex and height of subject. 2Mean (SD) #No significant changes compared to Initial, p &gt; 0.05. *P value &lt; 0.001 compared to PreHU; ++P value &lt; 0.001 compared to −HU repeat. PFT parameter 1 TLC 88 (13.6)2 85 (11.7) 85 (13.4) 90 (13.5) FVC 78 (9.8) 79 (9.9) 75 (12.6) 90 (13.2)*,++ FEV1 75 (9.6) 74 (12.2) 72 (11.4) 86 (10.2)*,++ FEF 25-75 75 (22) 72 (28.0) 77 (23.4) 79 (26.8) FEV1/FVC (%) 85(6.7) 82 (9.8) 87 (11.8) 86 (11.1) Pulse oximetry (%) 95 (3.1) 96 (2.4) 94 (4.4) 97 (2.8)* WBC (x103/ul) 14 (3.8) 13 (2.9) 13.02 (2.9) 9.0 (2.1) *,++ Hb (g/dl) 8.0 (0.1) 8.0 (0.9) 7.8 (1.1) 9.1 (1.3) *,++ MCV (fl) 84 (9.2) 85 (10.0) 87.3 (8.1) 101.2 (10.1) *,++ HbF (%) 4.3 (1.5) 5.2 (5.2) 6.0 (3.8) 14.0 (7.3) *,++ Conclusions: Serial PFTs showed improved pulmonary function, following initiation of HU therapy in children with prior abnormal PFT. Routine PFT assessments of children prescribed HU may serve as an objective measure of clinical response in children with SCA and pulmonary complications. These results will help in the design of future prospective studies examining the clinical benefits of HU therapy for children at risk for long-term pulmonary complications.

scholarly journals Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

2006 ◽  
Vol 173 (11) ◽  
pp. 1264-1269 ◽  
Author(s):  
Elizabeth S. Klings ◽  
Diego F. Wyszynski ◽  
Vikki G. Nolan ◽  
Martin H. Steinberg
Keyword(s):  
Pulmonary Function ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Abnormal Pulmonary Function

scholarly journals Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

2016 ◽  
Vol 42 (6) ◽  
pp. 409-415 ◽  
Author(s):  
Ana Karine Vieira ◽  
◽  
Cristina Gonçalves Alvim ◽  
Maria Cristina Marquez Carneiro ◽  
Cássio da Cunha Ibiapina ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Pulmonary Function Tests ◽  
Cell Disease ◽  
Restrictive Lung Disease ◽  
Minute Walk ◽  
Abnormal Pulmonary Function

ABSTRACT Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Prevalence and risk factors of root resorption of adjacent teeth in maxillary canine impaction, among untreated children and adolescents

2018 ◽  
Vol 41 (5) ◽  
pp. 447-453 ◽  
Author(s):  
Frédéric Rafflenbeul ◽  
Catherine-Isabelle Gros ◽  
François Lefebvre ◽  
Sophie Bahi-Gross ◽  
Raphaëlle Maizeray ◽  
...  
Keyword(s):  
Risk Factors ◽  
Children And Adolescents ◽  
Root Resorption ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Data Sets ◽  
Maxillary Canine ◽  
3D Data ◽  
Potential Risk Factors ◽  
Associated Risk Factors

Summary Objectives The aim of this retrospective study was to assess in maxillary canine impaction cases both the prevalence of root resorption of adjacent teeth among untreated children and adolescents, and its associated risk factors. Subjects and methods Sixty subjects (mean age 12.2 years; SD 1.9; range 8–17 years) with 83 displaced maxillary canines and without any past or ongoing orthodontic treatment were included in this study. The presence of root resorption was evaluated on images from a single cone beam computed tomography (CBCT) unit. Potential risk factors were measured on the CBCT images and on panoramic reconstructions of the 3D data sets. The sample was characterized by descriptive statistics and multiple logistic regressions were performed to predict root resorption. Results Root resorption of at least one adjacent tooth was detected in 67.5 per cent of the affected quadrants. It was found that 55.7 per cent of the lateral incisors, 8.4 per cent of the central incisors, and 19.5 per cent of first premolars were resorbed. Of the detected resorptions, 71.7 per cent were considered slight, 14.9 per cent moderate, and 13.4 per cent severe. Contact between the displaced canine(s) and the adjacent teeth roots was the only identified statistically significant risk factor, all teeth being considered (odds ratio [OR] = 18.7, 95% confidence interval: 2.26–756, P < 0.01). An enlarged canine dental follicle, a peg upper lateral, or an upper lateral agenesis were not significantly associated with root resorption of adjacent teeth, nor were age nor gender. Conclusions Root resorption of adjacent teeth was detected in more than two-thirds of a sample of sixty untreated children and adolescents.

scholarly journals A assistência de enfermagem em crianças e adolescentes portadores de anemia falciforme

2012 ◽  
Vol 2 (5) ◽  
pp. 5 ◽  
Author(s):  
Aline Barbosa Soares ◽  
Débora Rita Gobbi ◽  
André Moreno Silva ◽  
Gisele Duarte da Silva ◽  
Isabel Cristina Gomes Leite de Siqueira ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Sickle Cell ◽  
Nursing Care ◽  
Sickle Cell Anemia ◽  
Childhood And Adolescence ◽  
Nursing Interventions ◽  
Crisis Prevention ◽  
Calidad De Vida ◽  
High Prevalence ◽  
Prevention And Treatment

Anemia Falciforme é uma doença autossômica recessiva com elevada prevalência e morbimortalidade no Brasil. Apresenta uma variedade de complicações que se manifestam de forma peculiar em cada paciente, principalmente na infância e na adolescência. Levantar os diagnósticos e intervenções de enfermagem mais relevantes às crianças e aos adolescentes portadores da doença para proporcionar uma melhor qualidade de vida. Realizou-se o levantamento bibliográfico sobre o tema nas bases de dados SciELO, LILACS e AAFESP, além de dados sobre mortalidade da doença no portal DATASUS. Em 2008, 38,6% das mortes por Anemia Falciforme no Brasil compreendeu a faixa etária entre 0 e 19 anos, evidenciando a necessidade de uma assistência de enfermagem específica, baseada na prevenção e no tratamento das principais complicações da doença. O preparo do enfermeiro é fundamental para individualizar essa assistência junto às crianças e adolescentes portadores da doença para que se obtenha sucesso na prevenção das crises e no tratamento para amenizar os sintomas.Descritores: Enfermagem, Anemia Falciforme, Crianças e Adolescentes. Nursing care in children and adolescents with sickle cell anemiaAbstract: Sickle cell anemia is an autosomal recessive disease with a high prevalence, morbidity and mortality in Brazil. Features a variety of complications that manifest in a peculiar way in each patient, especially in childhood and adolescence. Raise the diagnoses and nursing interventions most relevant to children and adolescents with the disease to provide a better quality of life. We carried out the literature on the subject in the databases SciELO, LILACS and AAFESP, plus data on mortality rates in DATASUS portal. In 2008, 38.6% of deaths from sickle cell disease in Brazil comprised the age group between 0 and 19 years, highlighting the need for specific nursing care based on prevention and treatment of major complications of the disease. The preparation of nurses is critical to individualize this intervention with children and adolescents with the disease in order to achieve success in crisis prevention and treatment to alleviate the symptoms.Descriptors: Nursing, Sickle Cell Anemia, Children and Adolescents. Enfermería de atención a la infancia y la adolescencia con anemia de células falciformesResumen: La anemia falciforme es una enfermedad autosómica recesiva, con una alta prevalencia, morbilidad y mortalidad en Brasil. Cuenta con una variedad de complicaciones que se manifiestan de una manera peculiar en cada paciente, especialmente en la infancia y la adolescencia. Elevar los diagnósticos e intervenciones de enfermería más relacionados con los niños y adolescentes con la enfermedad de proporcionar una mejor calidad de vida. Hemos llevado a cabo la literatura sobre el tema en la bases de datos SciELO, LILACS y AAFESP, además de datos sobre las tasas de mortalidad en DATASUS portal. En 2008, el 38,6% de las muertes por la enfermedad de células falciformes en Brasil comprende el grupo de edad entre 0 y 19 años, destacando la necesidad de cuidados de enfermería específicos basados en la prevención y tratamiento de las principales complicaciones de la enfermedad. La preparación de las enfermeras es fundamental para individualizar la intervención con niños y adolescentes que padecen la enfermedad con el fin de lograr el éxito en la prevención de crisis y el tratamiento para aliviar los síntomas.Descriptores: Enfermería, Anemia de Células Falciformes, la Niñez y la Adolescencia.

Sign in / Sign up

Export Citation Format

Share Document