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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aetiopathogenesis and clinical picture of 3 cases of priapism are described, along with the treatment employed. The first patient suffered from thalassaemia minor, whereas in the second case priapism was a manifestation of aneurysm of the abdominal aorta perforated in the cava. In the last case, erection was a sequela of surgery for hydrocoele performed under peridural anaesthesia.
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PMID:[Priapism. Etiopathogentic, clinical and therapeutic considerations]. 67 17

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Partial splenic embolization. An effective alternative to splenectomy for hypersplenism. 226 5

From 1978 to 1988, The Cooperative Study of Sickle Cell Disease observed 3,765 patients with a mean follow-up of 5.3 +/- 2.0 years. One thousand seventy-nine surgical procedures were conducted on 717 patients (77% sickle cell anemia [SS], 14% sickle hemoglobin C disease [SC], 5.7% S beta zero thalassemia, 3% S beta zero + thalassemia). Sixty-nine percent had a single procedure, 21% had two procedures, and the remaining 11% had more than two procedures during the study follow-up. The most frequent procedure was abdominal surgery for cholecystectomy or splenectomy (24% of all surgical procedures, N = 258). Of these, 93% received blood transfusion, and there was no association between preoperative hemoglobin A level and complication rates (except reduction in pain crisis). Overall mortality within 30 days of a surgical procedure was 1.1% (12 deaths after 1,079 surgical procedures). Three deaths were considered to be related to the surgical procedure and/or anesthesia (0.3%). No deaths were reported in patients younger than 14 years of age. Sickle cell diseases (SCD)-related complications after surgery were more frequent in SS patients who received regional compared with general anesthesia (adjusted for risk level of the surgical procedure, patient age, and preoperative transfusion status, P = .058). Non-SCD-related postoperative complications were higher in both SS and SC patients who received regional compared with those who received general anesthesia (P =.095). Perioperative transfusion was associated with a lower rate of SCD-related postoperative complications for SS patients undergoing low-risk procedures (P = .006, adjusted for age and type of anesthesia), with crude rated of 12.9% without transfusion compared with 4.8% with transfusion. In SC patients, preoperative transfusion was beneficial for all surgical risk levels (P = .009). Thus, surgical procedures can be performed safely in patients with SCD.
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PMID:Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. 757 33

Fifteen patients, 13 male and two female, known to be carrying the sickle-cell gene (12 HbSS and 3 HbAS), who were undergoing operations requiring a bloodless field, were included in the study. Of the 12 with HbSS, seven had haemoglobin A1 component of between 11 and 27%, three had fetal haemoglobin ranging from 5.7 to 29% and the remaining two had increased haemoglobin A2 concentrations suggesting a beta non-thalassaemia combination. All had a tourniquet applied to the appropriate limb and were given general anaesthesia with moderate hyperventilation throughout the procedure. The tourniquet inflation time was 61.7 +/- 27.5 min. The mean PaO2 remained above 200 mmHg, mean PaCO2 was less than 37 mmHg, and pH ranged between 7.40 and 7.45. There were no clinically important changes in BP or ECG. All patients made uneventful recoveries and none developed sickle-cell crises. It is suggested that it is safe to use tourniquet in patients with sickle-cell disease provided optimum acid-base status and oxygenation are maintained throughout the procedure.
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PMID:Use of a tourniquet in patients with sickle-cell disease. 842 39

The epidemiological characteristics of 359 difficult intubations resulted from retrospectively collected data in a series of 4.742 individual anaesthetic case records of consecutive adult patients (2392 men, 2350 women) undergoing general anaesthesia for routine surgery. The preoperative assessment of each patient's airway was performed using standardized guidelines. Mandibular length, body weight and mobility of jaw, head and neck were evaluated with simple measurements. The presence of protruding maxilla or upper teeth, tongue tumor or cyst, long narrow mouth, short muscular neck and deviation of larynx or trachea was based on clinical and/or radiological examination. Severity of difficulty in intubation was estimated on a five-point scale grades (0-4) according to the view obtained at laryngoscopy and the final outcome of airway management. Difficult intubation (grades 1-4) was confirmed in 359 cases (7.6%) of which 208 were anticipated (57.2%). The effectiveness of preoperative evaluation had a positive correlation with the level of difficulty since, the greater the grade of difficulty the greater the sensitivity. The prevalence of failed intubation (grade 4) was 0.5%. Risk factors which had been correlated with the relative size of the tongue proved to have low probability and level of difficulty of intubation. The most important risk factor was limited jaw movement (median grade of difficulty: 4, positive predictive value: 75.0%). Large endemic goitre and thalassaemia constitute RFs contributing occasionally to difficult airway. The positive predictive value was 61.5% and 40.9% respectively. The combination of risk-factors had a cumulative impact contributing to the high level of difficulty.
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PMID:Evaluating the difficult airway. An epidemiological study. 891 85

There is little information on the possible value of screening children who are liable to haemoglobinopathies as part of pre-operative assessment for dental treatment under general anaesthesia. The present retrospective investigation examined, first, the number of patients having low haemoglobin levels among 1000 patients who had undergone haematological investigation prior to general anaesthesia in a dental outpatient unit, and, secondly, the subsequent clinical management of these patients. Haemoglobin levels of 10.0 g/dl or less were found in 31 children: 13 Asian, 7 Afro-Caribbean, 5 Mediterranean, 3 Arabic, 1 white Caucasian and 2 Oriental children. In addition, 17 patients had sickle-cell trait and 2 had beta-thalassaemia trait, but there was no relationship between the presence of haemoglobinopathy and low levels of haemoglobin. The planned general anaesthesia was undertaken for 22 of the 31 children who had low levels of haemoglobin and for the 19 children with haemoglobinopathy. Only 6 children ultimately did not undergo general anaesthesia, all failing to return. It is concluded that pre-anaesthetic haematological assessment of children needing minor dental surgery is rarely of any significant clinical value.
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PMID:The prevalence of clinically significant anaemia and haemoglobinopathy in children requiring dental treatment under general anaesthesia: a retrospective study of 1000 patients. 960 Dec 37

We reported a case of a thrombosis of the retinal and central artery and vein in a 44-year-old patient, with high-grade T-cell lymphoma. Cardiovascular family history and a medical history of cerebral ischemia caused by in situ fibrinolysis, led to perform thorough hematologic and genetic examinations. These disclosed a prothrombin gene G 20210 a variant in a homozygous state. In a heterozygous state, this mutation leads to an increase in plasma prothrombin rate of the order of 30%. Therefore, it is considered to be a major risk factor for venous thrombotic disease. Otherwise, acute leukemia or non-Hodgkin's lymphoma may induce thrombosis of retinal artery and vein by means of an optic nerve infiltration, as well as beta-thalassemia, ocular trauma and retrobulbar anesthesia. Retinal central artery and vein are seldom both involved; whereas occlusions of their branches are more frequent.
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PMID:[Mixed retinal thrombosis in a patient carrying prothrombin gene mutation in a homozygote state]. 1060 75

Beta Thalassemia patients suffer from a high incidence of gallstones as well as systemic complications of iron overload. We performed laparoscopic cholecystectomy in 8 beta thalassemia patients and describe their specific features. Diagnosis was based on patients' clinical presentation and sonography. Trocars were readjusted due to the hepatomegaly. No intraoperative cholangiograms were performed. Follow-up included clinical assessment and abdominal sonography. All procedures were completed laparoscopically. Pigment cirrhosis and fibrosis around the cystic duct were noted in all patients. No biliary injury occurred. Post operatively, 4 patients suffered fluid leakage through the trocar site, treated conservatively. No major cardiopulmonary complications occurred. During a mean follow-up time of 65 months, all patients are symptom free and without evidence of biliary lithiasis. Thalassemia patients are difficult and often high-risk patients. Improvement in anesthesia and monitoring enables better management of these patients. Laparoscopic cholecystectomy should be advised in carefully selected thalassemia patients.
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PMID:Unique features of laparoscopic cholecystectomy in Beta thalassemia patients. 1457 Nov 67

A 37-year-old man with beta-thalassemia intermedia (betaTI), a rare disease caused by partial or complete deficiency of beta-globin chain synthesis, fell into a hemolytic crisis. Severe anemia persisted despite frequent transfusions. Therefore, he was scheduled for splenectomy to alleviate the anemia. The preoperative laboratory data showed marked anemia and liver dysfunction. Echocardiography revealed hyperkinetic left ventricular motion and increased cardiac index (CI), indicating a compensatory hyperdynamic circulation induced by persistent, severe anemia. Our strategy during general anesthesia was to keep the hyperkinetic cardiovascular system steady. Hence, the hemodynamic parameters including the CI were measured using a Swan-Ganz catheter, and other physiological parameters were monitored perioperatively. Anesthesia was maintained with balanced anesthesia: isoflurane at low concentrations and fentanyl to avoid cardiovascular depression. Throughout the operation, vital signs were kept stable and the lactate/pyruvate ratio was unchanged, indicating that anaerobic metabolism did not increase. We report successful anesthetic management with attention to hemodynamic changes in a patient with betaTI.
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PMID:Anesthetic management of a patient with beta-thalassemia intermedia undergoing splenectomy: a case report. 1603 56

beta thalassaemia is one of the world's most wide-spread monogenetic disorders. Advances in the management of beta thalassaemia major by extensive blood transfusions and chelation therapy have improved survival of patients into adult life. Due to the prolonged life expectancy and improvements in quality of life, pregnancy has now become an important issue for patients and clinicians. We report a case of a pregnant patient with beta thalassaemia major who underwent a successful caesarean section under spinal anaesthesia. The multidisciplinary approach to management of beta thalassaemia major and pregnancy is discussed.
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PMID:Management of pregnancy in a patient with beta thalassaemia major. 1614 4


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