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Query: UMLS:C0020672 (hypothermia)
 17,327 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 5-year-old black girl with sickle cell anemia and G-6-PD deficiency is described. The child underwent successful repair of tetralogy of Fallot. Open-heart surgery can be safely performed on these patients if certain guidelines are observed, particularly the avoidance of hypoxia, hypothermia, acidosis, and dehydration. The patients should be prepared for the operation with transfusion of normal red cells. Routine preoperative testing for the above hemoglobinopathologic conditions is urged. The pertinent literature is reviewed.
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PMID:Complete repair of tetralogy associated with sickle cell anemia and G-6-PD deficiency. 13 81

Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.
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PMID:Surgical management of children with hemoglobinopathies. 144 Jan 54

Five patients with sickle cell haemoglobinopathies underwent open heart surgery. At the start of the cardiopulmonary bypass the patient's circulating blood volume was separately drained into a bag, spun down and retransfused into the patient at the end of the operation. Moderate hypothermia, aortic cross clamping, topical hypothermia and cold crystalloid cardioplegia were used in all patients. None of the patients had transfusions in the postoperative period. No macroscopic or microscopic evidence of haemolysis were seen, nor haematuria or other clinical evidence of sickling. There was no evidence of wound infection and all were discharged home at a mean of 12.6 days. It is concluded that in cases of sickle cell anaemia exchange transfusion at the beginning of cardiopulmonary bypass followed by retransfusion of the red cell free blood can be used safely, permitting the performance of standard open heart procedures.
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PMID:Cardiopulmonary bypass in sickle cell anaemia. Report of five cases. 201 34

Blood has a number of rheological properties which partially determine flow, especially at capillary level, and its capacity to deliver oxygen. It is non-Newtonian, pseudoplastic, thixotropic and viscoelastic. Viscosity can be studied with different types of viscosimeters (coaxial cylinder or capillary viscosimeters). It can be defined by the ratio of stress of deformation to rate of deformation. Viscosity depends on macrorheological parameters: hematocrit, serum proteins, especially fibrinogen and globulins, and also on microrheological parameters: degree of aggregation and red blood cell deformability. Viscosity rises when the temperature falls and decreases with the radius of the tube through which the blood flows (Fahraeus-Linqvist effects). Blood viscosity is studied clinically at different temperatures, and, above all, at different rates of deformation by carefully recording the hematocrit. Plasma viscosity, fibrinogen, albumia and immunoglobulin levels, the viscosity of blood cell suspensions in normal saline must also be taken into consideration. Special investigations (rheoscopy, filtrability) provide information about red cell aggregation and deformability. Hyperviscosity syndromes are observed with: --raised hematocrit (polycythemia and pseudopolycythemia), --conditions with raised serum proteins or changes in their composition (especially hyperfibrinogenemia, raised immunoglobulins, low albumin levels); inflammatory syndromes, dysglobulinemias (Fahey's syndrome of plasma hyperviscosity), --low temperature (hypothermia), --increased red cell aggregability (shock, fat embolism), --reduced red cell deformability due to various congenital and acquired conditions (sickle cell anemia, renal failure, hyperlipoproteinemia, thrombosis, diabetes). Conversely, hypoviscosity may occur with a low hematocrit, hypoproteinemia, hypofibrinogenemia, and hyperthermia. Increased viscosity results in a slowing of blood flow, stagnation of its constituents and in ischemia. Therapeutic interventions may be considered on the different components of the hyperviscosity syndrome: hemodilation, plasmapheresis, dispersion of aggregants, agents acting on red cell deformability.
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PMID:[Blood hyperviscosity syndromes. Classification and physiopathological understanding. Therapeutic deductions]. 636 7

Between July 1977 and October 1981, nine patients with sickle cell anemia underwent tonsillectomy or tonsillectomy with adenoidectomy without operative or postoperative morbidity or mortality. Appropriate management is necessary to prevent vaso-occlusive events. The current protocol for elective surgery used at the Medical College of Georgia, Augusta, consists of the following: (1) preoperative transfusion and hydration, (2) intraoperative prevention of hypothermia and maintenance of blood volume, and (3) postoperative oxygen therapy and hydration. The critical requirement of this protocol is the preoperative transfusion of these patients with packed cells consisting of hemoglobin A erythrocytes to lower the percentage of Hb S to less than 45% as determined by quantitative hemoglobin electrophoresis.
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PMID:Elective surgery in patients with sickle cell anemia. 711 88

Blood has particular rheological properties which partly condition its flow, especially in capillary vessels, and its ability to deliver oxygen. It is not subject to gravitation, pseudoplastic, thixotropic and visco-elastic. Blood viscosity depends upon macroscopic factors, such as erythrocyte aggregation and deformability. Hyperviscosity is observed in cases of increased haematocrit (polycythaemia and relative polycythaemia), increased serum proteins and changes in protein balance (e.g. rise in fibrinogen and immunoglobulins, fall in albumin) as seen in inflammation and dysglobulinaemia, drop in temperature (hypothermia), increased erythrocyte aggregation (shock, fat embolism) or imparied deformability due to various acquired or inherited disorders of red cell membrane or cytoplasma (e.g. sickle cell anaemia, renal failure, hyperlipoproteinaemias, thrombosis, diabetes). The various factors may be combined, as in diabetes. Conversely, hypoviscosity may result from decreased haematocrite, fall in blood proteins and fibrinogen, or hyperthermia. Hyperviscosity can be corrected by acting on its various constituents. Treatments include haemodilution, plasmapheresis, anti-aggregants and drugs improving red cell deformability.
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PMID:[Blood viscosity. Measurement and applications (hyper--and hypoviscosity syndromes) (author's transl)]. 723 52

As survival improves in patients with sickle cell anemia, the prospects of performing cardiac surgical procedures on older patients with this genetic defect increase. We describe the successful management of a 52-year-old patient with sickle cell disease (homozygous for hemoglobin S) and a history of multiple sickle crisis undergoing cardiopulmonary bypass for mitral valve repair. Preoperative partial exchange transfusion followed by total exchange transfusion at the time of operation was performed to reduce the level of hemoglobin S to 5.4% during bypass. Other management strategies included high-flow normothermic bypass with aortic crossclamping, topical hypothermia, and cold crystalloid cardioplegia.
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PMID:Mitral valve reconstruction in sickle cell disease. 865 4

Patients with sickle cell disease have been reported to have an increased risk of thromboembolism and pulmonary hypertension. Some of these patients may benefit from pulmonary thromboendarterectomy (PTE), a procedure that requires profound hypothermia, cardiopulmonary bypass, and periods of circulatory arrest, factors that may potentially increase the risk of sickling. Two patients with sickle cell disease (sickle-thalassemia [Hb S/beta+] and Hb SS) presented to the Pulmonary Vascular Center of UCSD Medical Center with significant shortness of breath and limitation of daily activities. Both of these patients were found to have surgically accessible chronic thromboembolic disease with pulmonary hypertension. PTE was performed in both patients using exchange transfusion, with avoidance of anemia, hypoxia, and acidosis. A successful outcome with resolution of pulmonary hypertension was achieved in both cases. To our knowledge this is the first report of patients with sickle cell disease who successfully underwent PTE for chronic thromboembolic pulmonary hypertension.
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PMID:Successful pulmonary thromboendarterectomy in two patients with sickle cell disease. 960 56

To minimize sickling during cardiopulmonary bypass, exchange transfusion is frequently recommended peri-operatively for patients with homozygous sickle cell anaemia to reduce the circulating concentration of HbS. We report the successful management of two children both aged 12 years with sickle cell anaemia (homozygous SS), several sickle cell crises and multiple blood transfusions who underwent cardiopulmonary bypass for mitral valve replacement. No pre- or intra-operative exchange transfusions were employed in the two cases. Furthermore, moderate hypothermia in both cases was not associated with adverse sequelae.
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PMID:Cardiopulmonary bypass in sickle cell anaemia without exchange transfusion. 986 Feb 13

Intracranial aneurysms are a common complication of sickle cell disease. The management of a patient with multiple intracranial aneurysms and sickle cell disease is described. The English language literature is reviewed. Neuroanesthetic management has traditionally been based on the avoidance of factors said to lead to erythrocyte sickling; however neuropathology typically arises from arterial intimal damage, not from venous sickling. Neuroanesthesia should be based on an appreciation of this pathophysiological model. Consideration of precipitants of vaso-occlusive crises, such as hypothermia, dehydration and possibly altered hemodynamics, should influence management.
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PMID:Management of multiple intracranial aneurysms: neuroanesthetic considerations of sickle cell disease. 1114 87


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