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respiratory complications of sickle cell disease

Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Nan Nitra Than, Han Ni, Jaspal Singh, Abdul Razzak Bin Mohd Said, Ifeyinwa Osunkwo
BACKGROUND: Sickle cell disease is a genetic chronic haemolytic and pro-inflammatory disorder. The clinical manifestations of sickle cell disease result from the presence of mutations on the beta globin genes that generate an abnormal haemoglobin product (called haemoglobin S) within the red blood cell. Sickle cell disease can lead to many complications such as acute chest syndrome, stroke, acute and chronic bone complications (including painful vaso-occlusive crisis, osteomyelitis, osteonecrosis and osteoporosis)...
January 20, 2017: Cochrane Database of Systematic Reviews
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
Radhika Gangaraju, Vishnu V B Reddy, Marisa B Marques
Bone marrow necrosis with subsequent embolization of the fat and necrotic tissues into the systemic circulation causing fat embolism syndrome and multiorgan failure is a rare complication of patients with hemoglobinopathies. The exact etiology of this condition is not known. Because it occurs more often in patients with compound heterozygous conditions than in sickle cell disease, some patients are unaware of their predisposition. The initial symptoms are nonspecific, such as back and/or abdominal pain, fever, and fatigue, which may rapidly progress to respiratory failure and severe neurologic compromise...
September 2016: Southern Medical Journal
John J Squiers, Anthony G Edwards, Alberto Parra, Sandra L Hofmann
A 70-year-old African American female with a past medical history significant for chronic bilateral shoulder pain and reported sickle cell trait presented with acute-onset bilateral thoracolumbar pain radiating to her left arm. Two days after admission, Hematology was consulted for severely worsening microcytic anemia and thrombocytopenia. Examination of the patient's peripheral blood smear from admission revealed no cell sickling, spherocytes, or schistocytes. Some targeting was noted. A Coombs test was negative...
January 2016: Journal of Investigative Medicine High Impact Case Reports
Jeffrey D Lebensburger, Prasannalaxmi Palabindela, Thomas H Howard, Daniel I Feig, Inmaculada Aban, David J Askenazi
BACKGROUND: Patients with sickle cell disease are at risk for developing chronic kidney disease (CKD). Acute kidney injury (AKI) has been linked to progression to CKD, but limited data exist to determine its role in acute complications of sickle cell disease. We hypothesized that AKI occurs in pediatric patients admitted for acute chest syndrome (ACS) and prolongs hospitalization. METHODS: We conducted a 6-year retrospective review of pediatric patients with ACS admitted to a single medical institution...
August 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Marzieh Nikparvar, Mohammad Reza Evazi, Tasnim Eftekhari, Farzaneh Moosavi
In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive crisis. The patient had manifestations of microangiopathic hemolytic anemia, including laboratory evidence of hemolytic anemia, thrombocytopenia, respiratory distress, fever, jaundice, and abnormal liver function and coagulation tests, accompanied by clot formation on the Eustachian valve of the inferior vena cava in the right atrium and also a long and worm-like thrombus in the right ventricle...
March 2016: Iranian Journal of Medical Sciences
Igho J Onakpoya, Gail Hayward, Carl J Heneghan
BACKGROUND: Lower respiratory tract infections (LRTIs) in young children account for 1.4 million deaths annually worldwide. Antibiotics could be beneficial in preventing LRTIs in high-risk children, and may also help prevent school absenteeism and work days missed by children and/or carers. While it is well documented that the efficacy of antibiotic prophylaxis for RTIs decreases over time, there are no reviews that describe the use of antibiotic prophylaxis to prevent LRTIs in high-risk children aged 12 years and under...
September 26, 2015: Cochrane Database of Systematic Reviews
Angela Barrier, Simo Willy, Jeremy S Slone
We present the case of a 7-year-old Cameroonian girl with sickle cell disease (SCD) who presented with progressive abdominal distension, fever, severe anemia, respiratory distress, and fatigue. Abdominal ultrasound showed a 15.3 cm × 11.5 cm × 15.5 cm solid echogenic mass within the left lobe of the liver. Fine-needle aspiration showed features of extramedullary hematopoiesis (EMH). Despite transfusions, antibiotics, and initiation of hydroxyurea the patient died of respiratory failure during the hospital stay...
August 2015: Pediatrics International: Official Journal of the Japan Pediatric Society
David Baltierra, Tiffany Harper, Matthew Page Jones, Konrad C Nau
Sickle cell disease, the most common inherited hemoglobinopathy in the United States, is a group of autosomal recessive red cell disorders resulting from hemoglobin S. Hemoglobin S forms rigid polymers when deoxygenated that give red blood cells their sickle crescent shape. Increased viscosity and cell adhesion result in vasoocclusion. Universal screening of US newborns enables early detection. Prophylactic penicillin through age 5 years and pneumococcal immunization lower the risk of serious pneumococcal infections...
June 2015: FP Essentials
Jianhui Zhang, Xiaolei Kang
BACKGROUND: Plastic bronchitis (PB) is a rare disease characterized by formation of bronchial casts. It is usually associated with congenital heart disease, sickle cell disease, lymphoma, and lung diseases such as asthma and pneumonia. OBJECTIVES: To report 14 cases of PB with influenza A or influenza B infection. METHODS: We analyzed the clinical manifestations, bronchoscopic and histologic findings, clinical courses, and outcomes. RESULTS: These cases indicate that PB is a life-threatening complication of severe influenza...
April 2015: International Journal of Pediatric Otorhinolaryngology
Henry E Rice, Brian R Englum, Jennifer Rothman, Sarah Leonard, Audra Reiter, Courtney Thornburg, Mary Brindle, Nicola Wright, Matthew M Heeney, Charles Smithers, Rebeccah L Brown, Theodosia Kalfa, Jacob C Langer, Michaela Cada, Keith T Oldham, J Paul Scott, Shawn St Peter, Mukta Sharma, Andrew M Davidoff, Kerri Nottage, Kathryn Bernabe, David B Wilson, Sanjeev Dutta, Bertil Glader, Shelley E Crary, Melvin S Dassinger, Levette Dunbar, Saleem Islam, Manjusha Kumar, Fred Rescorla, Steve Bruch, Andrew Campbell, Mary Austin, Robert Sidonio, Martin L Blakely
The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery)...
March 2015: American Journal of Hematology
David C Reed, David Sarraf
PURPOSE: To report a case of multiple bilateral branch retinal artery occlusions associated with vancomycin infusion in an African American patient in acute sickle crisis. METHODS: Single case report. RESULTS: A 30-year-old man with complicated sickle cell disease presented with pain, fever, and hypoxia. During infusion of vancomycin, he became diaphoretic, hypotensive, and unresponsive, and exhibited respiratory distress and a disseminated red skin rash consistent with a severe allergic reaction referred to as the red man syndrome...
2014: Retinal Cases & Brief Reports
Inês Vaz Silva, Ana Filipa Reis, Maria João Palaré, Anabela Ferrão, Teresa Rodrigues, Anabela Morais
BACKGROUND: Sickle cell disease (SCD) has extremely variable phenotypes, and several factors have been associated with the severity of the disease. OBJECTIVES: To analyze the chronic complications of SCD and look for predictive risk factors for increased severity and number of complications. METHODS: Retrospective study including all children followed for SCD in the Paediatric Haematology Unit of a tertiary hospital in Portugal, who completed 17 yr old between the years 2004 and 2013...
February 2015: European Journal of Haematology
S Terzi, C Griffoni, L Babbi, G Barbanti Brodano
Sickle cell disease (or drepanocytosis) is a hemoglobinopathy characterized by an increase in viscosity and adhesivity of the typically sickle-shaped erythrocytes. The pathological osteo-articular involvement in the course of drepanocytosis is secondary to the avascular necrosis of the bone marrow, caused by vaso-occlusive episodes in the microcirculation during acute painful crises. Osteoporosis and extramedullary hematopoiesis are also consequences of the disease. The involvement of the spine is common, with clinical features ranging from simple changes in spinal morphology ("fish-mouth" appearance) up to vertebral bodies fractures with kyphotic deformity...
2014: European Review for Medical and Pharmacological Sciences
Jérôme Cecchini, François Lionnet, Michel Djibré, Antoine Parrot, Katia Stankovic Stojanovic, Robert Girot, Muriel Fartoukh
OBJECTIVE: Sickle cell disease is associated with a decreased life expectancy, half of the deaths occurring in the ICU. We aimed to describe the characteristics of sickle cell disease patients admitted to ICU and to identify early predictors of a complicated outcome, defined as the need for vital support or death. DESIGN: Retrospective observational cohort study of sickle cell disease patients over a 6-year period. SETTING: ICU of a French teaching hospital and sickle cell disease referral center...
July 2014: Critical Care Medicine
Sara Christina Sadreameli, Megan E Reller, David G Bundy, James F Casella, John J Strouse
BACKGROUND: Respiratory syncytial virus (RSV) is a cause of acute chest syndrome (ACS) in sickle cell disease (SCD), but its clinical course and acute complications have not been well characterized. We compared RSV to seasonal influenza infections in children with SCD. PROCEDURE: We defined cases as laboratory-confirmed RSV or seasonal influenza infection in inpatients and outpatients <18 years of age with SCD from 1 September 1993 to 30 June 2011. We used Fisher's exact test to compare proportions, Student's t-test or Wilcoxon rank-sum test to compare continuous variables, and logistic regression to evaluate associations...
May 2014: Pediatric Blood & Cancer
Dimitris A Tsitsikas, Giorgio Gallinella, Sneha Patel, Henry Seligman, Paul Greaves, Roger J Amos
Fat embolism syndrome (FES) due to extensive bone marrow necrosis (BMN) in sickle cell disease (SCD) is a potentially under-diagnosed complication associated with severe morbidity and mortality. We identified 58 cases reported in the world literature to date. Typically, patients presented with a seemingly uncomplicated vaso-occlusive crisis (VOC) and subsequently deteriorated rapidly with a drop in their haemoglobin and platelets, development of respiratory failure, encephalopathy and varying degrees of involvement of other systems...
January 2014: Blood Reviews
Anastassios C Koumbourlis
Although some of the most severe complications of Sickle Cell Disease (SCD) tend to be acute and severe (e.g. acute chest syndrome, stroke etc.), the chronic ones can be equally debilitating. Prominent among them is the effect that the disease has on lung growth and function. For many years the traditional teaching has been that SCD is associated with the development of a restrictive lung defect. However, there is increasing evidence that this is not a universal finding and that at least during childhood and adolescence, the majority of the patients have a normal or obstructive pattern of lung function...
March 2014: Paediatric Respiratory Reviews
Adebola E Orimadegun, Babatunde O Ogunbosi, Shannon S Carson
BACKGROUND: Hypoxaemia is a potentially harmful complication of both acute lower respiratory tract infections (ALRI) and non-ALRI in children but its contribution to burden and outcomes of hospital admissions in Africa is unclear. We investigated prevalence and predictors of hypoxaemia in ALRI and non-ALRI according to age and primary diagnoses in emergently ill children in south western Nigeria. METHODS: In 1726 emergently ill children admitted to a tertiary hospital in Ibadan, south western Nigeria, oxygen saturation was measured shortly after admission...
November 2013: Transactions of the Royal Society of Tropical Medicine and Hygiene
Kurtis T Sobush, Courtney D Thornburg, Judith A Voynow, Stephanie D Davis, Stacey L Peterson-Carmichael
INTRODUCTION: This is the first published report of a young girl with co-inherited sickle cell-β+ thalassemia and cystic fibrosis. Although a small subset of patients with co-inherited cystic fibrosis and other hemoglobinopathies have been reported, this patient developed early hematologic and pulmonary complications that were more severe than the previous cases. To assess pulmonary co-morbidities, we used infant pulmonary function testing through the raised volume rapid thoracoabdominal compression technique as both an established study of early cystic fibrosis and also as a newer study of mechanism for early sickle cell lung disease...
2013: Journal of Medical Case Reports
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