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Syndrome Refractory (syndrome + refractory)

Distribution by Scientific Domains

Medical Sciences	100%

Selected Abstracts

Successful unrelated cord blood transplantation in a 7-year-old boy with Evans syndrome refractory to immunosuppression and double autologous stem cell transplantation

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2006
Christian Urban
Abstract:, Evans syndrome is an autoimmunopathy characterized by thrombocytopenia and autoimmune hemolytic anemia with poor response to immunosuppression. A 2-yr-old boy with Evans syndrome showed only short-lasting responses to immunosuppressive treatment including double autologous peripheral stem cell transplantation (SCT). Intracranial bleeding required emergency splenectomy and external ventricular drainage. Unrelated umbilical cord blood was given following conditioning with busulfan, thiotepa, etoposide and antithymocyte globulin. One year after SCT the patient shows stable blood counts without immunosuppression. This is the first child reported with Evans syndrome successfully treated by means of unrelated cord blood transplantation. [source]

Octreotide therapy in dumping syndrome: analysis of long-term results

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 9 2006
P. DIDDEN
Summary Background Octreotide therapy is effective in controlling severe dumping symptoms during short-term follow-up but little is known about long-term results. Aim To report on the long-term results of patients with severe dumping syndrome treated at the Leiden University Medical Center with subcutaneous or depot intramuscular (long-acting release) octreotide. Methods Follow-up of 34 patients with severe dumping syndrome refractory to other therapeutic measures treated between 1987 and 2005 with octreotide subcutaneous/long-acting release. At regular intervals symptoms, quality of life, weight, faecal fat excretion and gallstone formation were evaluated. Results All patients had excellent initial relief of symptoms during octreotide subcutaneous therapy. However, during follow-up 16 patients stopped therapy because of side effects (n = 9) or loss of efficacy (n = 7). Four patients died. Fourteen patients (41%) remain using octreotide (follow-up 93 ± 15 months), seven are on octreotide subcutaneous and seven on octreotide long-acting release. Patients with severe dumping (both early and late) do better on subcutaneous than long-acting release despite the inconvenience of frequent injections. Dumping symptoms are reduced by 50% even in long-term users. Body weight continues to increase during therapy despite more pronounced steatorrhoea. Conclusion The long-term the efficacy of octreotide is much less favourable compared with short-term treatment. [source]

Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from meige syndrome refractory to bilateral thalamotomy,

MOVEMENT DISORDERS, Issue 4 2001
Daisuke Muta MD
Meige syndrome is an adult-onset dystonic movement disorder that predominantly involves facial muscles, while some patients with this syndrome develop spasmodic dysphonia and dystonia of the neck, trunk, arms, and legs. We report that all dystonic symptoms that had been refractory to both pharmacotherapy and bilateral thalamotomy were markedly alleviated by bilateral pallidal stimulation in a patient with segmental axial dystonia advanced from Meige syndrome. © 2001 Movement Disorder Society. [source]

Multiple organ failure and severe bone marrow dysfunction in two 18 year-old Caucasian patients: Epstein,Barr virus and the haemophagocytic syndrome

ANAESTHESIA, Issue 11 2008
P. A. Berry
Summary Haemophagocytic lymphohistiocytosis secondary to viral infection is an unusual but well recognised cause of bone marrow dysfunction and multiple organ failure in young patients. Two 18 year-old patients were admitted to a tertiary liver unit with features of acute liver failure, cardio-respiratory collapse and pancytopenia. Serological tests and bone marrow examination with in-situ hybridisation revealed severe acquired haemophagocytic lymphohistiocytosis secondary to acute Epstein,Barr virus infection. Both patients died despite full supportive therapy; the first due to pulmonary haemorrhage, the second due to acute respiratory distress syndrome refractory to high frequency oscillatory ventilation. The clinical spectrum, diagnostic features and current evidence based recommendations for treatment of this condition are explored. The diagnosis of haemophagocytic lymphohistiocytosis should be considered in young patients with marked bone marrow dysfunction and multiple organ failure. Further research into appropriate therapy for patients with acute severe forms of the disease who require intensive organ support is required. [source]

Safety, Efficacy, and Cost Advantages of Combined Coronary Angiography and Angioplasty

JOURNAL OF INTERVENTIONAL CARDIOLOGY, Issue 3 2003
CLAUDE LE FEUVRE M.D., F.E.S.C.
Aim: The safety and efficacy of ad hoc PTCA has been previously reported and this approach is performed in many angioplasty centers as a routine procedure. The aim of this study is to examine whether this approach reduces the length, and cost of hospital stay. Methods and results: To determine the hospital costs we studied 2,440 PTCAs over 11 years in our institution (1990,2000). Urgent PTCA for acute coronary syndromes refractory to medical treatment were excluded. In 1809 patients (74%) angioplasty was performed immediately after coronary angiography, while separate procedures were performed in 631 patients. Indication for PTCA was unstable angina in 1342 patients (55%). In the ad hoc PTCA group, 92% of the culprit lesions were successfully treated; complications included myocardial infarction (2%), urgent bypass surgery (0.6%) and death (0.9%). The rate of combined procedure progressively increased from 54% in 1990 to 88% in 2000, with a significant decrease in the rate of complications. After adjusting for clinical and angiographic differences between combined and separate procedures, angiographic success and complication rates were not statistically different in the two groups. Mean length of hospital stay decreased all along the years, and was 45% less in the ad hoc PTCA group (11.4 ± 6.9vs18.2 ± 7.7in 1990,5.4 ± 4.3vs10.8 ± 5.7in 2000,P < 0.0001). The cost was 40% lower in the ad hoc PTCA group. For patients with stable angina, the savings were 49%, and for those with unstable angina, they were 29%. Conclusion: In the era of coronary stenting, ad hoc PTCA can be performed in most of the patients as safely and successfully as a separate procedure. It reduces the length, and the cost of hospital stay in patients with stable or unstable angina. (J Interven Cardiol 2003;16:195,199) [source]