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Sickle lung

Ursula David Alves, Agnaldo José Lopes, Maria Christina Paixão Maioli, Andrea Ribeiro Soares, Pedro Lopes de Melo, Roberto Mogami
OBJECTIVE: To describe and quantify the main changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease, as well as to evaluate the radiologist accuracy in determining the type of hemoglobinopathy. MATERIALS AND METHODS: A prospective study involving 44 adult patients with sickle cell disease who underwent inspiration and expiration computed tomography of the chest. The frequency of tomography findings and the extent of involvement are reported...
July 2016: Radiologia Brasileira
Elena Cela, Ana G Vélez, Alejandra Aguado, Gabriela Medín, José M Bellón, Cristina Beléndez
BACKGROUND AND OBJECTIVE: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. PATIENTS AND METHODS: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life...
September 29, 2016: Medicina Clínica
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Bum-Yong Kang, Kathy Park, Jennifer M Kleinhenz, Tamara C Murphy, Roy L Sutliff, David Archer, C Michael Hart
Pulmonary hypertension (PH), a serious complication of sickle cell disease (SCD), causes significant morbidity and mortality. While recent study determined that hemin release during hemolysis triggers endothelial dysfunction in SCD, the pathogenesis of SCD-PH remains incompletely defined. This study examines peroxisome proliferator-activated receptor gamma (PPARγ) regulation in SCD-PH and endothelial dysfunction. PH and right ventricular hypertrophy (RVH) were studied in Townes humanized sickle cell (SS) and littermate control (AA) mice...
September 9, 2016: American Journal of Respiratory Cell and Molecular Biology
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
Chen Li, Yu Zhou, Anastacia Loberg, Stanley M Tahara, Punam Malik, Vijay K Kalra
Previous studies showed higher levels of placenta growth factor (PlGF) correlated with increased plasma levels of endothelin-1 (ET-1), a potent vasoconstrictor, in sickle cell disease (SCD). PlGF mediated transcription of ET-1 occurred by activation of HIF-1α, and post-transcriptionally by miR-199a2, which targets the 3' -UTR of HIF-1α mRNA. However, relatively less is known how PlGF represses the expression of miR-199a2 located in the DNM3os transcription unit. Here we show PlGF induces the expression of ATF3, which in association with accessory proteins (JDP2, ATF2 and HDAC6), as determined by proteomic analysis, bind to the DNM3os promoter...
August 29, 2016: Molecular and Cellular Biology
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
Robyn T Cohen, Mark Rodeghier, Fenella J Kirkham, Carol L Rosen, Jane Kirkby, Michael R DeBaun, Robert C Strunk
BACKGROUND: Significance of exhaled nitric oxide (FeNO) levels in children with sickle cell anemia (SCA) is unclear, but increased levels may be associated with features of asthma and thus increased morbidity. OBJECTIVES: To determine factors associated with FeNO and whether FeNO levels are associated with increased rates of acute chest syndrome (ACS) and pain. METHODS: All participants had SCA, were part of the prospective, observational Sleep and Asthma Cohort study, and had the following assessments: FeNO, spirometry, blood samples analyzed for hemoglobin, white blood cell count, eosinophils and total serum IgE, questionnaires about child medical and family history, and review of medical records...
August 9, 2016: Journal of Allergy and Clinical Immunology
D Alan Nelson, Patricia A Deuster, Robert Carter, Owen T Hill, Vickee L Wolcott, Lianne M Kurina
BACKGROUND: Studies have suggested that sickle cell trait elevates the risks of exertional rhabdomyolysis and death. We conducted a study of sickle cell trait in relation to these outcomes, controlling for known risk factors for exertional rhabdomyolysis, in a large population of active persons who had undergone laboratory tests for hemoglobin AS (HbAS) and who were subject to exertional-injury precautions. METHODS: We used Cox proportional-hazards models to test whether the risks of exertional rhabdomyolysis and death varied according to sickle cell trait status among 47,944 black soldiers who had undergone testing for HbAS and who were on active duty in the U...
August 4, 2016: New England Journal of Medicine
Anastassios C Koumbourlis
No abstract text is available yet for this article.
August 2016: Annals of the American Thoracic Society
Manickam Niraimathi, Rakhee Kar, Sajini Elizabeth Jacob, Debdatta Basu
Vaso-occlusive crisis in sickle cell anaemia is one of the commonest presentations and a leading cause of death. Death can be sudden and unexpected. Herein we present three cases of sickle cell anaemia with sudden death within 3 days of hospitalisation. All the three cases presented with fever and jaundice. Two cases presented consecutively in the same year within a span of 5 months while the other case had presented 2 years prior to these two cases. Infection was the precipitating event in two cases and pregnancy with infection in one...
June 2016: Indian Journal of Hematology & Blood Transfusion
Michael R DeBaun, Robert C Strunk
Acute chest syndrome is a frequent cause of acute lung disease in children with sickle-cell disease. Asthma is common in children with sickle-cell disease and is associated with increased incidence of vaso-occlusive pain events, acute chest syndrome episodes, and earlier death. Risk factors for asthma exacerbation and an acute chest syndrome episode are similar, and both can present with shortness of breath, chest pain, cough, and wheezing. Despite overlapping risk factors and symptoms, an acute exacerbation of asthma or an episode of acute chest syndrome are two distinct entities that need disease-specific management strategies...
June 18, 2016: Lancet
Valérie Ghilain, Elsa Wiame, Edward Fomekong, Marie-Françoise Vincent, Dana Dumitriu, Marie-Cécile Nassogne
INTRODUCTION: Lysinuric protein intolerance (LPI) is a form of inherited aminoaciduria caused by a deficiency in the cationic amino acid transport process on the basolateral membrane of enterocytes and renal tubular cells. Clinical signs include gastrointestinal symptoms, failure to thrive, hepatosplenomegaly, osteoporosis, episodes of coma, intellectual deficiency, lung and renal involvement, bone marrow abnormalities, as well as altered immune response. Moyamoya disease is a cerebrovascular disorder predisposing sufferers to stroke through progressive stenosis of the intracranial internal carotid arteries and their proximal branches...
September 2016: European Journal of Paediatric Neurology: EJPN
Elissa Z Faro, C Jason Wang, Suzette O Oyeku
Extensive variation exists in the follow-up of positive screens for sickle cell disease. Limited quality indicators exist to measure if the public health goals of screening-early initiation of treatment and enrollment to care-are being achieved. This manuscript focuses on the development of quality indicators related to the follow-up care for individuals identified with sickle cell disease and trait through screening processes. The authors used a modified Delphi method to develop the indicators. The process included a comprehensive literature review with rating of the evidence followed by ratings of draft indicators by an expert panel held in September 2012...
July 2016: American Journal of Preventive Medicine
Robyn T Cohen, Robert C Strunk, Mark Rodeghier, Carol L Rosen, Fenella Jane Kirkham, Jane Kirkby, Michael R DeBaun
RATIONALE: Patient factors associated with development of abnormal lung function in children with sickle cell anemia (SCA) have not been fully characterized. OBJECTIVES: To characterize lung function abnormalities among children with SCA and to determine whether these steady-state lung function results were associated with morbidity before or after testing among children with SCA. METHODS: This study was part of the prospective National Institutes of Health-funded Sleep and Asthma Cohort Study...
August 2016: Annals of the American Thoracic Society
Gladys B Asiedu, Sharonne N Hayes, Karen Patricia Williams, Matthew R Bondaryk, Michele Y Halyard, Monica W Parker, Joyce E Balls-Berry, Vivian W Pinn, Carmen Radecki Breitkopf
OBJECTIVE: African American women bear a disproportionate burden of cardiovascular disease (CVD) and cancer. The purpose of this study was to identify prevalent health concerns among African American women who are members of The Links, Incorporated (Links), a large national service organization with health programming for communities of color. METHODS: Survey data (n = 391) were collected during the 2012 Links National Assembly. Twenty-six health issues were presented within five groups: cancer, CVD, pulmonary disease, chronic conditions, and behavioral health...
December 22, 2015: Journal of Racial and Ethnic Health Disparities
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Ian R Hambleton, Gavin Cho
BACKGROUND: Sickle cell disease is a genetic haemoglobin disorder, which can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Sickle cell disease is one of the most common severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. These complications can lead to morbidity (such as reduced exercise tolerance) and increased mortality...
2016: Cochrane Database of Systematic Reviews
Samit Ghosh, Chibueze A Ihunnah, Rimi Hazra, Aisha L Walker, Jason M Hansen, David R Archer, Amma T Owusu-Ansah, Solomon F Ofori-Acquah
The prevention of organ damage and early death in young adults is a major clinical concern in sickle cell disease (SCD). However, mechanisms that control adult progression of SCD during the transition from adolescence are poorly defined with no cognate prophylaxis. Here, we demonstrate in a longitudinal cohort of homozygous SCD (SS) mice a link between intravascular hemolysis, vascular inflammation, lung injury, and early death. Prophylactic Nrf2 activation in young SS mice stabilized intravascular hemolysis, reversed vascular inflammation, and attenuated lung edema in adulthood...
2016: JCI Insight
Dina D Daswani, Vaishali P Shah, Jeffrey R Avner, Deepa G Manwani, Jessica Kurian, Joni E Rabiner
OBJECTIVES: The objective was to determine the test performance characteristics for point-of-care lung ultrasonography (LUS) performed by pediatric emergency medicine (PEM) physicians compared with radiographic diagnosis of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) and fever. METHODS: This was a prospective study of patients up to 21 years with SCD and fever requiring chest X-ray (CXR) evaluation for ACS. Before obtaining CXR, a blinded PEM physician performed LUS using a standardized scanning protocol...
August 2016: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
Anastassios C Koumbourlis
No abstract text is available yet for this article.
July 2016: Pediatric Pulmonology
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