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

A 4-year-old girl with hemoglobin SC disease died following general anesthesia. Autopsy showed widespread intravascular sickling; staining with hematoxylin-basic fuchsin-picric acid demonstrated newly developed massive myocardial necrosis, a rarely documented finding. Anesthesia may produce conditions that provoke "crises" in patients with sickle hemoglobinopathies. Preoperative identification of sickle states and careful attention to hydration and oxygenation may minimize anesthetic risks in these patients.
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PMID:Myocardial necrosis following general anesthesia in hemoglobin SC disease. 26 77

Two cases have been presented: one shows complications when a latent form of sickle cell disease was manifested after orthognathic surgery; the other illustrates a protocol for management of patients with abnormal hemoglobins who are candidates for orthognathic surgery. The protocol includes an initial screening test for all black patients who are hospitalized for elective orthognathic surgery. When the screening test is positive, afollow-up hemoglobin electrophoresis is obtained to identify the specific hemoglobinopathy. Transfusions, when indicated, are given to alter the percentages so that the patient has no greater than 40% abnormal hemoglobin. Precautions are taken during anesthesia and after surgery to ensure that the patient is well oxygenated and is not in a hypothermic state. Serial electrophoreses are performed and transfusions, if indicated, are given during the postoperative and healing phases according to the postoperative status.
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PMID:Sickle cell hemoglobinopathies: a protocol for management. 28 37

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

The pediatric patient with sickle cell disease risks having a vasoocclusive episode during adenotonsillectomy under general anesthesia. With proper patient selection and appropriate perioperative management, adenotonsillectomy can be accomplished safely in children with sickle cell disease. We review the management of 10 children with sickle hemoglobinopathies who had adenotonsillectomy. Indications for surgery were recurrent streptococcal infections in four and obstructive sleep apnea in six of these children. No complications resulted from any of these procedures, and the mean length of postoperative hospitalization was 2.4 days. The principal feature of preoperative management was the transfusion of red blood cells to suppress the patient's endogenous erythropoiesis and to reduce the concentration of sickle cell hemoglobin to less than 30%. Though a prospective, multi-institutional clinical trial will ultimately be required to settle the issue of the safest preoperative management of children with sickle cell disease, balancing the risks of transfusion-related complications against anesthesia-related complications, our experience supports the operative safety of hypertransfusion therapy in children with sickle cell disease.
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PMID:Adenotonsillectomy in children with sickle cell disease. 199 Apr 54

Intraoperative cyanosis is an utmost emergency for anesthesiologist. If the patient has adequate control ventilation, and normal cardiac pulmonary physiology, then methemoglobinemia must be considered. Reported here is a normal female with dark color lip on the second day after her second parturition and was undergoing tubal ligation. Twenty minutes after induction of general anesthesia and endotracheal intubation, dark blood at the incision site was noted by the operator. After emergent check up of the anesthesia machine, tubings, breathing sound and arterial blood gas, there was only one suspicion left. Methemoglobinemia was confirmed by the hematological examination. Methemoglobinemia is a product from the oxidation of the iron atom in the heme ring when oxygen dissociates from it. This process exists in nature, but can also be induced by nitrate or nitrite-containing drugs or foods or benzene-like organic compounds. Methemoglobinemia can be differentiated from normal hemoglobin by mass spectrometry. If acute illness develops, patients should be treated with methylene blue. Otherwise ascorbic acid will do. This case is reported to remind all anesthesia personnel about one of the rare but serious hemoglobinopathy.
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PMID:[Another reason for cyanosis--methemoglobinemia]. 803 75

Perioperative complications of surgical procedures are frequently encountered in patients with sickle cell disease. We have reviewed our series of patients with hemoglobinopathies who underwent cholecystectomy from 1978 to 1991 to evaluate their perioperative management and clinical outcome. Twenty-two children with major sickle hemoglobinopathy underwent cholecystectomy for symptomatic cholelithiasis. All 22 were transfused to achieve a hemoglobin (Hgb) level greater than 9 g/dL and hemoglobin S (HbS) less than 37%. Fourteen underwent immediate preoperative automated red cell exchange (ARCE). The median preexchange Hgb of these patients was 8.1 g/dL (range, 6.8 to 10.5). Their median HbS was 84% (range, 53% to 97%). These patients underwent placement of an apheresis catheter under local anesthesia followed by red cell exchange. The median volume of packed red blood cells (PRBC) exchanged was 28.1 mL/kg (range, 13.8 to 58.7). The median HbS after exchange was 21% (range, 16% to 37%) and the median Hgb was 10.6 g/dL (range, 6.5 to 16.7). Eight other patients underwent sequential transfusion (3 after an exchange for an acute pulmonary vasoocclusive crisis). These patients had been prepared over an interval of 2 to 8 weeks preoperatively and had received a median of 26.9 mL PRBC/kg (range, 12.8 to 95). Following sequential transfusion the median Hgb was 11.8 g/dL (range, 9 to 15.7) and the median HbS was 19% (range, 5% to 32%) at the time of surgery. All patients received extended antigen matched blood. Complications of preoperative transfusion were minor and included two febrile-/allergic reactions and one mild superficial catheter-induced phlebitis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Perioperative management for cholecystectomy in sickle cell disease. 842 77

This study's purpose was to investigate the effectiveness of ultrasound-guided percutaneous drainage (US-PD) as treatment modality for pediatric pyogenic liver abscess when compared with other modalities. Ten consecutive patients, aged from 2 months to 22 years, were enrolled in this study. In addition to antibiotics, US-PD was attempted in each case unless the procedure was judged unsuitable. Six patients were treated with US-PD while four were not, for various reasons. US-PD was performed under an intravenous anesthesia and with the aid of real-time sonography. An indwelling pigtail catheter was usually inserted during US-PD. Fever subsided within one to six days, but the abscess cavity closed later. Failure to respond to US-PD may relate to the huge size of abscess cavity; surgical drainage provides an option. A trend toward a shorter hospital stay and defervescence day was found in the US-PD group than in the non-US-PD group. Prognosis was generally good and none in this series died of this disease or the procedure. Klebsiella pneumoniae was the pathogen most frequently encountered. Seven of the ten patients had underlying disease, and hemoglobinopathy was frequently associated. In conclusion, adequate drainage is recommended as the most effective treatment modality for pediatric liver abscess, and US-PD is the first choice.
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PMID:Ultrasound-guided percutaneous drainage of liver abscess in children. 860 58

To determine the effects of sickle cell disease on the glenohumeral joint, 28 shoulders in 14 patients with SS sickle cell hemoglobinopathy were studied clinically and roentgenographically. patients were randomly selected; their mean age was 46 years (range, 22 to 63 years). Pain, stability, and function of the shoulders were assessed, and roentgenograms were evaluated for osteonecrosis. All 28 shoulders had some degree of pain with activity, but functional range of motion was maintained despite symptoms. Seventy-one percent of the patients had had total hip arthroplasty and 21% had had total knee arthroplasty for osteonecrosis; there was a mean of 1.5 previous joint implants per patient. Our study results show that, in patients with sickle cell hemoglobinopathy, symptoms of humeral head osteonecrosis are better tolerated than those of osteonecrosis in the lower extremities, delaying the need for surgical intervention. With severe pain and functional limitations, shoulder arthroplasty is the procedure of choice in this patient population. However, the risks are greater for patients with sickle cell disease than for other patients who have humeral head osteonecrosis, and thorough preoperative medical and anesthesia evaluations are necessary. These patients require perioperative transfusion or plasmapheresis and sufficient intraoperative hydration and oxygenation to avoid precipitating a sickle cell crisis; in addition, use of methyl methacrylate should be avoided.
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PMID:Osteonecrosis of the humeral head in sickle cell disease. 879 12

This detailed review of sickle hemoglobinopathies covers their incidence and epidemiology, pathophysiology, diagnosis, clinical manifestations, complications, and organ changes. Issues such as pregnancy and birth control in patients with sickle cell disease, surgery and anesthesia, and medical management are also discussed.
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PMID:Advances in the Treatment of Adolescents with Sickle Hemoglobinopathies. 1035 77

Recent advances in the pathophysiology, clinical investigations, and management of sickle hemoglobinopathies enables all physicians to better manage these disease states and their sequelae. Patients with sickle cell disease SCD are living longer and are thus more likely to contract unrelated diseases that require surgery and anesthesia. Patients with SCD continue to be a challenge to all branches of medicine particularly in obstetrics, surgery and anesthesia; however, the armamentarium of new knowledge and practice places a different perspective on the care of this old disease. In general, the literature to date suggests that neither prophylactic transfusion of pregnant sicklers nor the selection of an anesthetic in labor have a major impact on patient outcome; however, perioperative management can greatly affect the consequences. A thorough knowledge of the impact of the disease on clinical status can determine how, when, and why to manage parturients with SCD.
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PMID:Sickle cell disease in pregnancy. Obstetric and anesthetic management perspectives. 1504 61


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