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

A case of sickle cell disease diagnosed postmortem is described. A 37-year-old black woman presented with anemia, respiratory distress, and abdominal and back pain. Death followed an intramuscular injection of iron, and anaphylaxis was clinically diagnosed. At autopsy, massive fat and necrotic bone marrow embolization of pulmonary and renal vessels was found. In the vertebral column, multifocal areas of ischemic necrosis were present, and proved to be the source of this embolization. Sickled red cells appeared in bone marrow sinusoids, and signs of disseminated intravascular coagulation were present.
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PMID:Massive fat and necrotic bone marrow embolization in a previously undiagnosed patient with sickle cell disease. 230 55

We performed exchange transfusions, utilizing the technique of automated erythrocytapheresis, for the treatment of patients with sickle cell anemia. In an attempt to determine guidelines for the use of erythrocytapheresis, we studied the use of this procedure in three distinct clinical situations in nine patients with sickle cell disease. Patients with dangerous complications of sickle cell disease such as acute respiratory distress and priapism responded well to erythrocytapheresis, showing marked improvement within 24-48 hours. Patients with prolonged painful vasoocclusive crises showed only variable improvement after erythrocytapheresis therapy, insufficient to justify exposing the patient to the risks of the procedure. Patients treated to decrease the frequency of painful crises demonstrated no prolongation in symptom-free intervals between crises. Therefore, erythrocytapheresis has its main value in the management of acute, dangerous complications of sickle cell disease.
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PMID:Use of erythrocytapheresis in the treatment of patients with sickle cell anemia. 654 85

Exchange transfusion is a well-established procedure for the treatment of severe complications of sickle cell anemia. However, large-volume exchange is a difficult, time-consuming technique, and therefore rarely used. Exchange red blood cell pheresis, using automated equipment, can accomplish red blood cell exchange more rapidly and efficiently, and can be easily performed by a skilled team of nursing personnel. The recent introduction of a pediatric centrifuge bowl allows this technique to be applied to pediatric patients. This procedure was used in a 13-year-old male with sickle cell anemia, who presented with bilateral pulmonary infiltrates, respiratory distress, and hypoxemia, unresponsive to antibiotics and other means of conservative therapy. A one and one-half volume red blood cell exchange reduced the hemoglobin S concentration to 13 per cent. The patient experienced dramatic improvement within 24 hours, progressing to complete recovery within a few days.
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PMID:Exchange red blood cell pheresis in a pediatric patient with severe complications of sickle cell anemia. 726 69

Pathogenic organism can be considered as pro-oxidant agents because they produce cell death and tissue damage. In addition organism can be eliminated by specific cell defense mechanism which utilize in part, reactive oxygen radicals formed by oxidative stress responses. The cause of the necessarily defense process results in cell damage thereby leading to development of inflammation, a characteristic oxidative stress situation. This fact shows the duality of oxidative stress in infections and inflammation: oxygen free radicals protect against microorganism attack and can produce tissue damage during this protection to trigger inflammation. Iron, a transition metal which participates generating oxygen free radicals, displays also this duality in infection. We suggest also that different infectious pathologies, such as sickle cell anemia/malaria and AIDS, may display in part this duality. In addition, it should be noted that oxidative damage observed in infectious diseases is mostly due the inflammatory response than to the oxidative potential of the pathogenic agent, this last point is exemplified in cases of respiratory distress and in glomerulonephritis. This review analyzes these controversial facts of infectious pathology in relation with oxidative stress.
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PMID:[Oxidative stress and infectious pathology]. 779 23

Chest pain is a common complaint among children of all ages. It rarely is due to cardiac disease, but deserves careful evaluation for this possibility, with laboratory tests performed in limited cases. The child who has pain of acute onset that interferes with sleep, is precipitated by exercise, or is associated with dizziness, palpitations, syncope, or shortness of breath should be evaluated with the aid of laboratory tests. This includes at least a chest radiograph and electrocardiogram. Also, pain in the child who has a history of coin ingestion, trauma, previous cardiac disease, or conditions that put him or her at risk for developing cardiac pathology deserve further study. Likewise, those who have a history of conditions such as asthma, Marfan syndrome, or sickle cell disease warrant special consideration. Finally, most of those who have an abnormal physical examination (fever, respiratory distress, abnormal breath sounds, cardiac murmur, abnormal rhythm or heart sounds, palpable subcutaneous air, or obvious trauma) also require a chest radiograph and an electrocardiogram. However, the child who has chronic chest pain normal physical examination with no worrisome history needs reassurance and careful follow-up rather than extensive studies.
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PMID:Consultation with the specialist. Chest pain in children. 911 17

A practice parameter has been developed to assist physicians in the therapeutic use of red blood cell transfusions. The developers of this parameter used the best available information from the medical literature, as well as clinical experience and the extensive reality testing required by the College of American Pathologists for approval. In acute anemia, a fall in hemoglobin values below 6 g/dL or a rapid blood volume loss of more than 30% to 40% requires red blood cell transfusions in most patients. However, tissue oxygenation provides a better indication of physiologic need in situations where invasive monitoring provides this information. When these data are not available, heart rate and blood pressure measurements and the nature of bleeding (active, controlled, uncontrolled) supplement the hemoglobin value in guiding the transfusion decision. In sickle cell disease and thalassemias, red blood cells are transfused to prevent acute or chronic complications. Red blood cell transfusions are used in chronic anemias unresponsive to pharmacologic agents based on the patient's symptoms. Guidelines must be altered for neonates who require an increase in hematocrit to above 0.30 to 0.35 when respiratory distress is present. Indications for red blood cell transfusion for the pregnant or postpartum patient are similar to those for the nonpregnant patient. Risks of transfusion, particularly transmissible disease and incompatibility, remain but have been reduced. Thus, red blood cell transfusion continues to be a powerful therapeutic tool when used judiciously and carries less risk than in the recent past.
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PMID:Practice parameter for the use of red blood cell transfusions: developed by the Red Blood Cell Administration Practice Guideline Development Task Force of the College of American Pathologists. 949 55

A 19-year-old black male with previous history of mild bronchial asthma presented sudden dyspnea and died in a state of respiratory distress in spite of resuscitation attempts. Autopsy showed typical lesions of acute bronchial asthma in a context of long-standing asthma associated with a massive and disseminated intravascular sickling. Hemoglobin electrophoresis diagnosed heterozygous sickle cell disease. This rare case raises the problem of the accuracy of death certificate, especially the difficulties to distinguish postmortem lesions as the underlying cause of death or the contributory cause of death.
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PMID:Postmortem revelation of sickle cell disease following fatal episode of acute bronchial asthma. 1195 32

Severe acute chest syndrome afflicts patients with sickle cell disease and can cause hypoxemia refractory to conventional treatments. Obstructive mucus plugging and the development of acute respiratory distress syndrome may underlie the pathophysiology of refractory hypoxemia in acute chest syndrome. Although high-frequency oscillatory ventilation (HFOV) is well established in the treatment of pediatric acute respiratory distress syndrome, there is no support in the literature for its role in managing hypoxemia in acute chest syndrome. In disease processes with high airways resistance and obstructive mucus plugging, HFOV may predispose to air-trapping and increased morbidity secondary to air leak syndromes. We report the first successful HFOV management of pediatric patients suffering from severe acute chest syndrome and hypoxic respiratory failure. These cases suggest that HFOV should be strongly considered for patients with severe acute chest syndrome that is refractory to conventional mechanical ventilation.
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PMID:Successful treatment of acute chest syndrome with high-frequency oscillatory ventilation in pediatric patients. 1498 46

Experimenta naturae, like the glucose-6-phosphate dehydrogenase deficiency, indicate that malaria parasites are highly susceptible to alterations in the redox equilibrium. This offers a great potential for the development of urgently required novel chemotherapeutic strategies. However, the relationship between the redox status of malarial parasites and that of their host is complex. In this review article we summarise the presently available knowledge on sources and detoxification pathways of reactive oxygen species in malaria parasite-infected red cells, on clinical aspects of redox metabolism and redox-related mechanisms of drug action as well as future prospects for drug development. As delineated below, alterations in redox status contribute to disease manifestation including sequestration, cerebral pathology, anaemia, respiratory distress, and placental malaria. Studying haemoglobinopathies, like thalassemias and sickle cell disease, and other red cell defects that provide protection against malaria allows insights into this fine balance of redox interactions. The host immune response to malaria involves phagocytosis as well as the production of nitric oxide and oxygen radicals that form part of the host defence system and also contribute to the pathology of the disease. Haemoglobin degradation by the malarial parasite produces the redox active by-products, free haem and H(2)O(2), conferring oxidative insult on the host cell. However, the parasite also supplies antioxidant moieties to the host and possesses an efficient enzymatic antioxidant defence system including glutathione- and thioredoxin-dependent proteins. Mechanistic and structural work on these enzymes might provide a basis for targeting the parasite. Indeed, a number of currently used drugs, especially the endoperoxide antimalarials, appear to act by increasing oxidant stress, and novel drugs such as peroxidic compounds and anthroquinones are being developed.
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PMID:Oxidative stress in malaria parasite-infected erythrocytes: host-parasite interactions. 1503 4

A 36-year-old male with a known history of sickle cell disease (SCD) and acute chest syndrome (ACS) was treated in our hospital. Gas exchange deteriorated and the patient was transferred to our intensive care unit (ICU). Low dose inhaled nitric oxide (iNO) during pressure controlled mechanical ventilation (pcMV) induced a clinically relevant increase in arterial oxygenation. The patient was weaned from pcMV after five days and discharged home 14 days later. ACS evolving to acute respiratory distress syndrome (ARDS) is a rare but severe complication. In ACS iNO should be considered a beneficial therapeutic option.
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PMID:Inhaled nitric oxide for ARDS due to sickle cell disease. 1510 20


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