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

During an episode of pneumonia and multiple pulmonary thromboembolic events, an adult male patient with sickle cell-C hemoglobinopathy developed a severe anemia associated with rapidly increasing splenic size and diminished splenic uptake of technetium Tc 99m sulfur colloid, indicating diminished splenic function due to hypersequestration, a rare and noteworthy phenomenon in adult patients with sickle cell-C disease.
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PMID:Splenic sequestration with sickle cell-C disease. 62 59

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

Hemoglobinopathies are a major public health problem in Saudi Arabia. We studied the effect of splenectomy in 16 Saudi Arabian children with compound hemoglobinopathies. Seven patients no longer require regular blood transfusions, and transfusion requirements were decreased by 30 to 60% in the other eight patients. Three patients whose heights and weights were below the 5th percentile before splenectomy reached the 25th percentile 1 year after the surgery. In spite of preoperative pneumococcal vaccination and the penicillin prophylaxis after the surgery, one patient died of Haemophilus influenzae Group B bacteremia, and three others had six episodes of pneumonia.
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PMID:Splenectomy in compound heterozygous hemoglobinopathies in Saudi Arabia. 224 Apr 76

The light-microscope finding of red cell membrane fragments in the form of long filamentous processes and myelin bodies in the blood smears of a patient with sickle cell anemia has recently been described. This phenomenon has been termed erythrocytic ecdysis. We examined the blood smears of all sickle cell anemia patients admitted to the Cook County Hospital and those attending the hemoglobinopathy clinic between October 1979 and December 1981. Nine instances of erythrocytic ecdysis were uncovered. Associated clinical conditions included congestive heart failure, acute viral syndrome, pneumonia, and metastatic malignancy. Transient ecdysis associated with congestive heart failure was noted for one patient during two separate admissions one year apart. Ecdysis is a transient form of erythrocytic fragmentation occurring in sickle cell anemia. Its pathogenesis is unknown. The role of regional circulatory stasis and hypoxia in the induction of erythrocyte membrane damage in sickle cell anemia needs investigation.
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PMID:Erythrocytic ecdysis in smears of EDTA venous blood in eight patients with sickle cell anemia. 311 41

The DF given weekly by the RBIT in 14 thalassemia/hemoglobinopathy patients decreased the frequency of URIs and febrile illnesses in 5/10 patients. Five of the six patients had absence of frequent enteritis. There were no instances of pneumonia and otitis media in two patients with frequent infections before DF. Skin color was improved within 3 months. Most of the patients became more active at the same hematocrit level. The height and weight in eight patients was normal. The total number of blood transfusions in all patients was significantly decreased. It is advisable to use weekly DF infusion by RBIT in the patient who cannot afford to use the infusion pump and the full program of iron chelation.
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PMID:Rubber band technique for slow subcutaneous infusion of desferrioxamine (Desferal). 339 May 34

The diverse manifestations of human parvovirus B19 infection have been well established. Erythema infectiosum, fetal hydrops, adult arthropathy, and aplastic anemia in patients with hemoglobinopathies or underlying immunocompromise have been described. Recently we successfully treated a patient who, after heart transplantation, had fever, rash, and pneumonia with respiratory failure caused by human parovirus B19. Human parovirus B19 has not been reported previously as a pathogen causing pulmonary disease after pediatric heart transplantation, and we wish to report it at this time.
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PMID:Severe pneumonia after heart transplantation as a result of human parvovirus B19. 803 18

The acute chest syndrome (ACS), characterized by fever, chest pain, leukocytosis and a new infiltrate on chest roentgenogram, is a common complication of sickle hemoglobinopathies. The major differential diagnoses of ACS are pneumonia and pulmonary vaso-occlusive disease, which may occur simultaneously. Bacterial pulmonary infections are documented infrequently in ACS with the exception being in the pediatric population under 5 years of age. Because there are no clinical or laboratory parameters that clearly allow for distinction between pneumonia and vaso-occlusive disease, empiric use of antibiotics directed against S. pneumoniae and other pathogens commonly seen in community-acquired pneumonias remain a mainstay of therapy.
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PMID:The acute chest syndrome of sickle cell disease. 848 94

Bacterial infections remain a major cause of morbidity and mortality among young children with sickle cell disease. Susceptibility to infections is mainly observed in homozygous sickle cell disease. The incidence of bacteremias in children under 3 years of age is approximately 8 events/100 patient-years among homozygous subjects and approximately S events/100 patient-years among those with SC hemoglobinopathy. Pneumococci and Salmonellae are the most frequently isolated bacteria. Severe clinical manifestations include septicemia, meningitis, osteomyelitis and pneumonia. M. Pneumoniae and C. Pneumoniae infections may be severe and may induce acute chest syndrome. The high incidence and severity of bacterial infections in these children justify prevention efforts by antibiotic prophylaxis and vaccination. The efficacy of oral penicillin prophylaxis against pneumococcal infections has been well demonstrated and is now recommended from 3 months of age. The antipneumococcal conjugate vaccine has been shown to be safe and immunogenic in young infants.
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PMID:[Infections and antibiotic prophylaxis in sickle cell disease]. 1513 1

The erythropoietic system plays a major role in tissue oxygenation because the erythrocytes are the primary carriers of oxygen in the form of oxyhemoglobin. Therefore, clinical entities such as abnormal hemoglobins, polycythemia, anemia, and significant changes in blood volume frequently produce alterations in various respiratory functions. The pulmonary manifestations can vary from mild respiratory illness to life-threatening emergencies with high mortality rates. Among the hemoglobinopathies, sickle cell disease is clinically the most important and commonly associated with serious pulmonary consequences, including acute chest syndrome, pneumonia, infarction due to in situ thrombosis, bone marrow fat embolism of pulmonary vasculature, bone marrow infarction, pulmonary hypertension, and other abnormalities. Hemoglobinopathies with high and low affinity for oxygen and other abnormal hemoglobinopathies occasionally cause clinically significant respiratory complications by interfering with normal tissue oxygenation. Acquired methemoglobinemia can cause alarming cyanosis and medical emergency. Erythrocyte disorders are associated with pulmonary complications, including pulmonary hypertension, alveolar fibrosis, and pulmonary dysfunction. Coagulation disorders, both the inherited and acquired types, have the potential to affect the respiratory system in the form of hemorrhage from the airways, lung parenchyma, or pulmonary hypertension. The following paragraphs describe the common pulmonary complications and manifestations associated with hemoglobinopathies, erythrocyte disorders, and coagulation abnormalities.
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PMID:Lungs in hemoglobinopathies, erythrocyte disorders, and hemorrhagic diatheses. 1626 4

Bacterial infections are considered a major cause of morbidity and mortality in patients, particularly children, with sickle cell disease. Infections including pneumonia, meningitis, osteomyelitis, pyelonephritis and general sepsis are more prevalent in patients with these genetic abnormalities than in normal individuals. Generally, infections are more prevalent in children than in older patients. The most common cause of severe infections in hemoglobinopathies include Diplococcus, Staphylococcus, Pneumococcus, Salmonella and Streptococcus. Several investigations have been conducted to determine the possible defects in the host defense mechanisms. Functional asplenia, defects in alternate pathway and in opsonic activity and phagocytosis of Streptococci, Staphylococci and Salmonella in sickle cell anemia patients are considered important factors predisposing these patients to bacteremia. On the other hand, a beneficial association has been demonstrated between the sickle cell gene and malaria. The hemoglobin S (Hb S) provides a natural resistance against the malarial parasite resulting in an improvement in fitness and survival over the normal (Hb AA) individuals. This communication reviews infections in sickle cell disease with a comparison of results in various populations.
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PMID:Infections in sickle cell disease. 2116 37


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