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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical aspects of Hemoglobinopathy S in children of a black family from Zair living in Belgrade are discussed in the paper. The parents and two brothers are heterozygous carriers for patologyc hemoglobines; those children had, sauf permanent anaemia, the crysis of dyspnea, cyanosis, cough, evidence of subperiosteal bone formation, associated with vitamin D deficiency. The Youngest child, girl two years of age, is homozygous with complete Sickle cell disease: hand-foot syndrome, heterotopic paravertebral hematopoetic tissue, lung infarctions, cardiomegaly, severe drepanocytic anaemia; she succumbed in an a attack after many episodes of severe hypoxia.
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PMID:[Hemoglobinpathy S--clinical manifestations in children in a Zairian family]. 61 14

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

Hemoglobinopathies are diseases involving abnormalities of the structure or production of hemoglobin. Examples include sickle cell disease, the thalassemias, and rare hemoglobin variants producing cyanosis. Recent advances in the understanding of the consequences of hemoglobin dysfunction on nitric oxide signaling have led to a reassessment of the pathophysiology of sickle cell disease and thalassemia. Chronic vascular inflammation and damage is now recognized as playing an important role in disease expression. Hemoglobinopathies may present to the anesthesiologist as the primary cause of a surgical procedure, as an incidental complicating factor of a surgical patient, or with a problem arising from the disease itself. This article reviews the common types of hemoglobinopathies, presents a basic summary of the pathophysiology relevant to anesthesia, and outlines current perioperative management.
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PMID:Anesthesia and hemoglobinopathies. 1970 79