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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Committee on Infectious Diseases of the American Academy of Pediatrics, and the Advisory Committee on Immunization Practices of the Center for Disease Control for many years have recommended the routine use of influenza vaccine in various hemoglobinopathies including sickle cell disease. This recommendation, however, has not been included in the patient care protocols of the Comprehensive Sickle Cell Centers program of NIHLB. Most clinicians have not used yearly influenza vaccine for their patients with sickle cell disease. This article reports a case of a 5-year-old boy with sickle cell disease who had not received influenza vaccine. He developed pneumonitis and acute myositis during a serologically confirmed influenza B virus infection. The incapacitating and protracted course of his illness presented diagnostic and management problems. His case strongly supports the recommendation of the two infectious disease committees.
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PMID:Sickle cell disease with complicated influenza B virus infection. 160 65

Sickle cell disease represents a spectrum of inherited hemoglobin disorders. The pathophysiology involves abnormalities not just in red blood cells but also vascular endothelium, white blood cell function, coagulation, and inflammatory response. Known sequelae of sickle cell disease include invasive infections, painful episodes, acute chest syndrome, strokes, and chronic pulmonary hypertension. Preventive strategies that decrease the risk of infection are the routine use of daily antibiotics until five years of age, immunization of children with the 7-valent pneumococcal conjugate vaccine in addition to the 23-valent polysaccharide pneumococcal vaccine, annual influenza vaccination after six months of age, and meningococcal vaccination after two years of age. A significant advance in stroke prevention is the use of transcranial Doppler ultrasonography to identify asymptomatic, at-risk children who should be considered for chronic blood transfusions. Chronic transfusion therapy for primary or secondary stroke prevention requires careful surveillance for iron overload and chelation therapy. Patients with chest pain, fever, or respiratory symptoms and new pulmonary infiltrates require aggressive medical management for acute chest syndrome. Pain management still represents an important area for aggressive treatment using sickle cell disease-specific guidelines. Newer treatments include hydroxyurea therapy to decrease the frequency of painful episodes and associated comorbidities, and hematopoietic cell transplantation for a limited subset of patients. Family physicians play a crucial role in instituting evidence-based preventive care strategies, initiating timely treatment of acute illness, recognizing life-threatening episodes, and providing a medical home for multidisciplinary management.
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PMID:Opportunities to improve outcomes in sickle cell disease. 1688 29

Infectious diseases and underlying medical conditions common to Africa may affect influenza frequency and severity. We conducted a systematic review of published studies on influenza and the following co-infections or co-morbidities that are prevalent in Africa: dengue, malaria, measles, meningococcus, Pneumocystis jirovecii pneumonia (PCP), hemoglobinopathies, and malnutrition. Articles were identified except for influenza and PCP. Very few studies were from Africa. Sickle cell disease, dengue, and measles co-infection were found to increase the severity of influenza disease, though this is based on few studies of dengue and measles and the measles study was of low quality. The frequency of influenza was increased among patients with sickle cell disease. Influenza infection increased the frequency of meningococcal disease. Studies on malaria and malnutrition found mixed results. Age-adjusted morbidity and mortality from influenza may be more common in Africa because infections and diseases common in the region lead to more severe outcomes and increase the influenza burden. However, gaps exist in our knowledge about these interactions.
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PMID:Potential Impact of Co-Infections and Co-Morbidities Prevalent in Africa on Influenza Severity and Frequency: A Systematic Review. 2606 16

The coronavirus disease 2019 (COVID-19) is an emerging infectious disease that has become a global public health concern after being first reported in China and has subsequently spread worldwide. It causes mild to severe respiratory illness with some flu-like symptoms. The causal virus behind this disease, SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), conceivably attacks the receptors of the respiratory system of the human body but has no strict evidence of attacking the blood cells yet. However, patients with hemoglobin disorders (e.g., sickle cell anemia, thalassemia) are vulnerable to this global health situation due to their clinical complications. Such patients are generally more prone to viral and bacterial infections, which can worsen their physical condition. Some of these patients present immunocompromised conditions, e.g., splenectomized or post-transplant patients. Therefore, they should follow some preventive steps such as shielding as well as the general guidelines for the COVID-19 pandemic. Transfusion dependent patients require regular monitoring for iron overload, and iron chelation therapy may be stopped by the physician depending on the situation. This article reviews the management strategies and provides some crucial recommendations for people in the corner with hemoglobin disorders.
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PMID:Management of Hemoglobin Disorders During the COVID-19 Pandemic. 3258 45