مستخلص: BACKGROUND: Untreated microangiopathic hemolytic anemia in pregnancy is associated with adverse maternal and perinatal outcomes. Accurate diagnosis is challenging owing to nonspecific clinical features and pathologic findings. Timely initiation of appropriate management is essential to optimize maternal and perinatal outcomes. CASE: A 26-year-old primiparous woman presented at 20 weeks of gestation with new-onset microangiopathic hemolytic anemia on a background of poorly controlled type 1 diabetes. She received eculizumab for presumed atypical hemolytic uremic syndrome. At 24 weeks of gestation, she developed superimposed early-onset preeclampsia; she delivered at 27 weeks of gestation after continuing eculizumab. CONCLUSION(S): Eculizumab may prolong pregnancy in early-onset preeclampsia. Additional research is needed to assess short-term and long-term maternal and newborn outcomes.Copyright © 2019 by the American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.

مصطلحات الفهرس: erythrocyte transfusion, estimated glomerular filtration rate, female, fetal well being, fetus lung maturation, fibrin deposition, fibrosing alveolitis, gene, generalized edema/dt [Drug Therapy], generalized edema/si [Side Effect], gestational age, glycemic control, headache/si [Side Effect], hemoglobin blood level, hemolytic uremic syndrome/di [Diagnosis], heterozygote, histopathology, home oxygen therapy, human, human tissue, hyperreflexia/si [Side Effect], hypertension/dt [Drug Therapy], insulin dependent diabetes mellitus/dt [Drug Therapy], insulin treatment, kidney biopsy, kidney function, kidney tubule, lactate dehydrogenase blood level, lethargy, macroalbuminuria, maternal smoking, meningococcosis, missense mutation, onset age, peripheral edema, placenta insufficiency, platelet count, preeclampsia/dt [Drug Therapy], preeclampsia/pc [Prevention], primipara, priority journal, proliferative diabetic retinopathy, prolonged pregnancy, protein urine level, proteinuria, restlessness/si [Side Effect], thrombocyte aggregation, thrombocytopenia, thrombotic thrombocytopenic purpura/di [Diagnosis], vaccination, acetylsalicylic acid/dt [Drug Therapy], albumin/ec [Endogenous Compound], antihypertensive agent/po [Oral Drug Administration], corticosteroid, creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], furosemide/dt [Drug Therapy], haptoglobin/ec [Endogenous Compound], hemoglobin/ec [Endogenous Compound], insulin/dt [Drug Therapy], lactate dehydrogenase/ec [Endogenous Compound], magnesium sulfate, nifedipine/dt [Drug Therapy], nifedipine/po [Oral Drug Administration], penicillin derivative, placental growth factor/ec [Endogenous Compound], prednisone, protein/ec [Endogenous Compound], urea/ec [Endogenous Compound], vasculotropin receptor 1/ec [Endogenous Compound], CFI gene, hemolytic uremic syndrome/dt [Drug Therapy], adult, antibiotic prophylaxis, antihypertensive therapy, article, atrophy, blurred vision/si [Side Effect], cannabis use, case report, Caucasian, cesarean section, cigarette smoking, clinical article, creatinine urine level, diabetic nephropathy/di [Diagnosis], end stage renal disease, erythrocyte concentrate, Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/35380Test
Obstetrics and Gynecology
LibKey Link

مستخلص: BACKGROUND: Untreated microangiopathic hemolytic anemia in pregnancy is associated with adverse maternal and perinatal outcomes. Accurate diagnosis is challenging owing to nonspecific clinical features and pathologic findings. Timely initiation of appropriate management is essential to optimize maternal and perinatal outcomes. CASE: A 26-year-old primiparous woman presented at 20 weeks of gestation with new-onset microangiopathic hemolytic anemia on a background of poorly controlled type 1 diabetes. She received eculizumab for presumed atypical hemolytic uremic syndrome. At 24 weeks of gestation, she developed superimposed early-onset preeclampsia; she delivered at 27 weeks of gestation after continuing eculizumab. CONCLUSION(S): Eculizumab may prolong pregnancy in early-onset preeclampsia. Additional research is needed to assess short-term and long-term maternal and newborn outcomes.Copyright © 2019 by the American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.

مصطلحات الفهرس: erythrocyte transfusion, estimated glomerular filtration rate, female, fetal well being, fetus lung maturation, fibrin deposition, fibrosing alveolitis, gene, generalized edema/dt [Drug Therapy], generalized edema/si [Side Effect], gestational age, glycemic control, headache/si [Side Effect], hemoglobin blood level, hemolytic uremic syndrome/di [Diagnosis], heterozygote, histopathology, home oxygen therapy, human, human tissue, hyperreflexia/si [Side Effect], hypertension/dt [Drug Therapy], insulin dependent diabetes mellitus/dt [Drug Therapy], insulin treatment, kidney biopsy, kidney function, kidney tubule, lactate dehydrogenase blood level, lethargy, macroalbuminuria, maternal smoking, meningococcosis, missense mutation, onset age, peripheral edema, placenta insufficiency, platelet count, preeclampsia/dt [Drug Therapy], preeclampsia/pc [Prevention], primipara, priority journal, proliferative diabetic retinopathy, prolonged pregnancy, protein urine level, proteinuria, restlessness/si [Side Effect], thrombocyte aggregation, thrombocytopenia, thrombotic thrombocytopenic purpura/di [Diagnosis], vaccination, acetylsalicylic acid/dt [Drug Therapy], albumin/ec [Endogenous Compound], antihypertensive agent/po [Oral Drug Administration], corticosteroid, creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], furosemide/dt [Drug Therapy], haptoglobin/ec [Endogenous Compound], hemoglobin/ec [Endogenous Compound], insulin/dt [Drug Therapy], lactate dehydrogenase/ec [Endogenous Compound], magnesium sulfate, nifedipine/dt [Drug Therapy], nifedipine/po [Oral Drug Administration], penicillin derivative, placental growth factor/ec [Endogenous Compound], prednisone, protein/ec [Endogenous Compound], urea/ec [Endogenous Compound], vasculotropin receptor 1/ec [Endogenous Compound], CFI gene, hemolytic uremic syndrome/dt [Drug Therapy], adult, antibiotic prophylaxis, antihypertensive therapy, article, atrophy, blurred vision/si [Side Effect], cannabis use, case report, Caucasian, cesarean section, cigarette smoking, clinical article, creatinine urine level, diabetic nephropathy/di [Diagnosis], end stage renal disease, erythrocyte concentrate, Article

URL: Obstetrics and Gynecology
Click here for full text options
LibKey Link

مصطلحات الفهرس: letter, lung edema, malignant hypertension/dt [Drug Therapy], middle aged, multiple sclerosis/dt [Drug Therapy], priority journal, hemolysis, human tissue, kidney biopsy, acute kidney failure, adult, case report, clinical article, creatinine blood level, drug withdrawal, dyspnea, female, human, proteinuria, thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/si [Side Effect], antihypertensive agent/dt [Drug Therapy], beta1a interferon/ae [Adverse Drug Reaction], beta1a interferon/dt [Drug Therapy], beta1a interferon/sc [Subcutaneous Drug Administration], creatinine/ec [Endogenous Compound], teriflunomide/dt [Drug Therapy], Letter

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/36225Test
Internal Medicine Journal
Click here for full text options
LibKey Link

مصطلحات الفهرس: letter, lung edema, malignant hypertension/dt [Drug Therapy], middle aged, multiple sclerosis/dt [Drug Therapy], priority journal, hemolysis, human tissue, kidney biopsy, acute kidney failure, adult, case report, clinical article, creatinine blood level, drug withdrawal, dyspnea, female, human, proteinuria, thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/si [Side Effect], antihypertensive agent/dt [Drug Therapy], beta1a interferon/ae [Adverse Drug Reaction], beta1a interferon/dt [Drug Therapy], beta1a interferon/sc [Subcutaneous Drug Administration], creatinine/ec [Endogenous Compound], teriflunomide/dt [Drug Therapy], Letter

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/36225Test
Internal Medicine Journal
LibKey Link

مستخلص: Thrombotic microangiopathy (TMA) is a well-recognised complication following transplantation, often due to an underlying genetic predisposition, medications or rejection. The use of eculizumab in these settings has been previously described, but its role still remains to be clarified. A 45-year-old man, with a history of type 1 diabetes mellitus and subsequent end-stage kidney failure, presented for a simultaneous pancreas-kidney transplant. Immunologically, he was well matched with the donor, and he received standard induction immunosuppression including tacrolimus. His early transplant course was complicated by Haemophilus parainfluenzae paronychia and a Pseudomonas aeruginosa catheter-associated urinary tract infection. Within 1 week, he developed thrombotic microangiopathy with significant renal dysfunction and eventual dialysis dependence, without evidence of transplant rejection on biopsy. He was also noted to have antiphospholipid antibodies in moderate titres. The TMA did not resolve despite cessation of tacrolimus, and he was subsequently commenced on eculizumab. The patient achieved a partial remission from TMA, with ongoing biochemical evidence of haemolysis, although now with stable graft function, despite significant damage. His transplanted pancreas remained seemingly unaffected by TMA, and continues to function well. This case describes an unusual presentation of TMA post-transplantation and is the only described case of eculizumab use following pancreas-kidney transplant. It remains unclear in this case what the likely precipitant for TMA was, although it seems to be, at least in part, controlled by ongoing use of eculizumab, presumably by terminal complement inhibition.Copyright © 2017 Asian Pacific Society of Nephrology

مصطلحات الفهرس: drug dose reduction, drug substitution, drug withdrawal, end stage renal disease/su [Surgery], end stage renal disease/th [Therapy], fever/co [Complication], gastroscopy, graft versus host reaction/dt [Drug Therapy], graft versus host reaction/pc [Prevention], Haemophilus parainfluenzae, hemolysis/di [Diagnosis], hemolysis/si [Side Effect], HLA matching, human, hypercholesterolemia, hypertension, hypotension/co [Complication], hypotension/dt [Drug Therapy], immunosuppressive treatment, intensive care, kidney biopsy, kidney cortex necrosis/di [Diagnosis], kidney pancreas transplantation, leg thrombosis/di [Diagnosis], leg thrombosis/dt [Drug Therapy], leukocyte count, low drug dose, male, medical history, middle aged, pancytopenia/si [Side Effect], paronychia/co [Complication], paronychia/dt [Drug Therapy], peritoneal dialysis, plasma exchange, Pseudomonas infection/co [Complication], Pseudomonas infection/dt [Drug Therapy], reflux esophagitis/di [Diagnosis], remission, review, sigmoidoscopy, thrombocyte count, thrombosis/dt [Drug Therapy], thrombosis/pc [Prevention], thrombosis prevention, thrombotic thrombocytopenic purpura/co [Complication], thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/dt [Drug Therapy], thrombotic thrombocytopenic purpura/th [Therapy], triacylglycerol lipase blood level, urinary tract infection/co [Complication], urinary tract infection/dt [Drug Therapy], vomiting/si [Side Effect], acetylsalicylic acid/dt [Drug Therapy], basiliximab/cb [Drug Combination], basiliximab/dt [Drug Therapy], beta2 glycoprotein 1/ec [Endogenous Compound], cardiolipin antibody/ec [Endogenous Compound], corticosteroid/cb [Drug Combination], corticosteroid/dt [Drug Therapy], creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], enoxaparin/dt [Drug Therapy], everolimus/dt [Drug Therapy], flucloxacillin/dt [Drug Therapy], flucloxacillin/iv [Intravenous Drug Administration], HLA antibody/ec [Endogenous Compound], inotropic agent/dt [Drug Therapy], leukocyte antigen/ec [Endogenous Compound], mycophenolic acid/cb [Drug Combination], mycophenolic acid/dt [Drug Therapy], novel erythropoiesis stimulating protein/dt [Drug Therapy], phospholipid antibody/ec [Endogenous Compound], piperacillin plus tazobactam/dt [Drug Therapy], prednisolone/dt [Drug Therapy], tacrolimus/cb [Drug Combination], tacrolimus/dt [Drug Therapy], thymocyte antibody/dt [Drug Therapy], triacylglycerol lipase/ec [Endogenous Compound], warfarin/dt [Drug Therapy], catheter/am [Adverse Device Effect], human tissue, adult, anemia/di [Diagnosis], anemia/dt [Drug Therapy], antibody titer, bacterium identification, biochemical analysis, bone marrow biopsy, bone marrow suppression/di [Diagnosis], case report, catheter, creatinine blood level, deep vein thrombosis/di [Diagnosis], deep vein thrombosis/dt [Drug Therapy], diabetic retinopathy, diarrhea/co [Complication], diarrhea/si [Side Effect], Doppler flowmetry, drug dose increase, Review

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/39529Test
Nephrology
Click here for full text options
LibKey Link

مستخلص: A 25-year-old man presented with microangiopathic haemolytic anaemia and acute kidney injury. With a normal ADAMTS-13 level, negative faecal shiga-toxin test and strong family history of atypical haemolytic uremic syndrome, he was commenced on eculizumab to good clinical response. Subsequent genetic testing revealed a heterozygous complement factor H mutation. Eculizumab was discontinued after 44 months of treatment, and he relapsed within 6 months, with the first sign being downtrending haptoglobin levels, with no other markers of haemolysis or thrombocytopaenia, 5 weeks prior to development of acute kidney injury. He was recommenced on eculizumab and to date still remains on it. This case highlights the unusual pattern of relapse and discusses the considerations for eculizumab discontinuation in patients with stable atypical haemolytic uremic syndrome receiving maintenance therapy.Copyright © 2017 Asian Pacific Society of Nephrology

مصطلحات الفهرس: hemolytic uremic syndrome/th [Therapy], hemolytic uremic syndrome/dt [Drug Therapy], human, kidney biopsy, lethargy/co [Complication], maintenance therapy, male, malignant hypertension/co [Complication], malignant hypertension/dt [Drug Therapy], nausea/co [Complication], nonhuman, Norovirus, plasmapheresis, relapse, review, seizure/co [Complication], thrombocyte count, thrombocytopenia/di [Diagnosis], thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/th [Therapy], treatment duration, urea blood level, vomiting, complement factor H/ec [Endogenous Compound], creatinine/ec [Endogenous Compound], eculizumab/dt [Drug Therapy], haptoglobin/ec [Endogenous Compound], hemoglobin/ec [Endogenous Compound], labetalol/dt [Drug Therapy], lactate dehydrogenase/ec [Endogenous Compound], lercanidipine/dt [Drug Therapy], ramipril/dt [Drug Therapy], von Willebrand factor cleaving proteinase/ec [Endogenous Compound], complement factor H gene, fecal shiga toxin test, helmet cell, acute kidney failure/di [Diagnosis], adult, case report, cells, diarrhea, erythrocyte, erythrocyte disorder, family history, feces analysis, gene mutation, genetic screening, headache/co [Complication], hematuria/di [Diagnosis], hemodialysis, hemolysis, Review

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/39530Test
Nephrology
Click here for full text options
LibKey Link

مستخلص: Thrombotic microangiopathy (TMA) is a well-recognised complication following transplantation, often due to an underlying genetic predisposition, medications or rejection. The use of eculizumab in these settings has been previously described, but its role still remains to be clarified. A 45-year-old man, with a history of type 1 diabetes mellitus and subsequent end-stage kidney failure, presented for a simultaneous pancreas-kidney transplant. Immunologically, he was well matched with the donor, and he received standard induction immunosuppression including tacrolimus. His early transplant course was complicated by Haemophilus parainfluenzae paronychia and a Pseudomonas aeruginosa catheter-associated urinary tract infection. Within 1 week, he developed thrombotic microangiopathy with significant renal dysfunction and eventual dialysis dependence, without evidence of transplant rejection on biopsy. He was also noted to have antiphospholipid antibodies in moderate titres. The TMA did not resolve despite cessation of tacrolimus, and he was subsequently commenced on eculizumab. The patient achieved a partial remission from TMA, with ongoing biochemical evidence of haemolysis, although now with stable graft function, despite significant damage. His transplanted pancreas remained seemingly unaffected by TMA, and continues to function well. This case describes an unusual presentation of TMA post-transplantation and is the only described case of eculizumab use following pancreas-kidney transplant. It remains unclear in this case what the likely precipitant for TMA was, although it seems to be, at least in part, controlled by ongoing use of eculizumab, presumably by terminal complement inhibition.Copyright © 2017 Asian Pacific Society of Nephrology

مصطلحات الفهرس: drug dose reduction, drug substitution, drug withdrawal, end stage renal disease/su [Surgery], end stage renal disease/th [Therapy], fever/co [Complication], gastroscopy, graft versus host reaction/dt [Drug Therapy], graft versus host reaction/pc [Prevention], Haemophilus parainfluenzae, hemolysis/di [Diagnosis], hemolysis/si [Side Effect], HLA matching, human, hypercholesterolemia, hypertension, hypotension/co [Complication], hypotension/dt [Drug Therapy], immunosuppressive treatment, intensive care, kidney biopsy, kidney cortex necrosis/di [Diagnosis], kidney pancreas transplantation, leg thrombosis/di [Diagnosis], leg thrombosis/dt [Drug Therapy], leukocyte count, low drug dose, male, medical history, middle aged, pancytopenia/si [Side Effect], paronychia/co [Complication], paronychia/dt [Drug Therapy], peritoneal dialysis, plasma exchange, Pseudomonas infection/co [Complication], Pseudomonas infection/dt [Drug Therapy], reflux esophagitis/di [Diagnosis], remission, review, sigmoidoscopy, thrombocyte count, thrombosis/dt [Drug Therapy], thrombosis/pc [Prevention], thrombosis prevention, thrombotic thrombocytopenic purpura/co [Complication], thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/dt [Drug Therapy], thrombotic thrombocytopenic purpura/th [Therapy], triacylglycerol lipase blood level, urinary tract infection/co [Complication], urinary tract infection/dt [Drug Therapy], vomiting/si [Side Effect], acetylsalicylic acid/dt [Drug Therapy], basiliximab/cb [Drug Combination], basiliximab/dt [Drug Therapy], beta2 glycoprotein 1/ec [Endogenous Compound], cardiolipin antibody/ec [Endogenous Compound], corticosteroid/cb [Drug Combination], corticosteroid/dt [Drug Therapy], creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], enoxaparin/dt [Drug Therapy], everolimus/dt [Drug Therapy], flucloxacillin/dt [Drug Therapy], flucloxacillin/iv [Intravenous Drug Administration], HLA antibody/ec [Endogenous Compound], inotropic agent/dt [Drug Therapy], leukocyte antigen/ec [Endogenous Compound], mycophenolic acid/cb [Drug Combination], mycophenolic acid/dt [Drug Therapy], novel erythropoiesis stimulating protein/dt [Drug Therapy], phospholipid antibody/ec [Endogenous Compound], piperacillin plus tazobactam/dt [Drug Therapy], prednisolone/dt [Drug Therapy], tacrolimus/cb [Drug Combination], tacrolimus/dt [Drug Therapy], thymocyte antibody/dt [Drug Therapy], triacylglycerol lipase/ec [Endogenous Compound], warfarin/dt [Drug Therapy], catheter/am [Adverse Device Effect], human tissue, adult, anemia/di [Diagnosis], anemia/dt [Drug Therapy], antibody titer, bacterium identification, biochemical analysis, bone marrow biopsy, bone marrow suppression/di [Diagnosis], case report, catheter, creatinine blood level, deep vein thrombosis/di [Diagnosis], deep vein thrombosis/dt [Drug Therapy], diabetic retinopathy, diarrhea/co [Complication], diarrhea/si [Side Effect], Doppler flowmetry, drug dose increase, Review

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/39529Test
Nephrology
Click here for full text options
LibKey Link

مستخلص: A 25-year-old man presented with microangiopathic haemolytic anaemia and acute kidney injury. With a normal ADAMTS-13 level, negative faecal shiga-toxin test and strong family history of atypical haemolytic uremic syndrome, he was commenced on eculizumab to good clinical response. Subsequent genetic testing revealed a heterozygous complement factor H mutation. Eculizumab was discontinued after 44 months of treatment, and he relapsed within 6 months, with the first sign being downtrending haptoglobin levels, with no other markers of haemolysis or thrombocytopaenia, 5 weeks prior to development of acute kidney injury. He was recommenced on eculizumab and to date still remains on it. This case highlights the unusual pattern of relapse and discusses the considerations for eculizumab discontinuation in patients with stable atypical haemolytic uremic syndrome receiving maintenance therapy.Copyright © 2017 Asian Pacific Society of Nephrology

مصطلحات الفهرس: hemolytic uremic syndrome/th [Therapy], hemolytic uremic syndrome/dt [Drug Therapy], human, kidney biopsy, lethargy/co [Complication], maintenance therapy, male, malignant hypertension/co [Complication], malignant hypertension/dt [Drug Therapy], nausea/co [Complication], nonhuman, Norovirus, plasmapheresis, relapse, review, seizure/co [Complication], thrombocyte count, thrombocytopenia/di [Diagnosis], thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/th [Therapy], treatment duration, urea blood level, vomiting, complement factor H/ec [Endogenous Compound], creatinine/ec [Endogenous Compound], eculizumab/dt [Drug Therapy], haptoglobin/ec [Endogenous Compound], hemoglobin/ec [Endogenous Compound], labetalol/dt [Drug Therapy], lactate dehydrogenase/ec [Endogenous Compound], lercanidipine/dt [Drug Therapy], ramipril/dt [Drug Therapy], von Willebrand factor cleaving proteinase/ec [Endogenous Compound], complement factor H gene, fecal shiga toxin test, helmet cell, acute kidney failure/di [Diagnosis], adult, case report, cells, diarrhea, erythrocyte, erythrocyte disorder, family history, feces analysis, gene mutation, genetic screening, headache/co [Complication], hematuria/di [Diagnosis], hemodialysis, hemolysis, Review

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/39530Test
Nephrology
Click here for full text options
LibKey Link

مستخلص: Background: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA). In 2009, the Australian TTP/TMA registry was established to collect data on patients presenting with TTP/TMA throughout Australia. Aim(s): To summarise information on the diagnosis and management of patients with TTP collected in the first 5years (2009-2014) of the Australian TTP registry. Method(s): Registry data from June 2009 to October 2014 were reviewed. Result(s): Fifty-seven patients were identified with TTP (defined as ADAMTS13 activity <10%), accounting for 72 clinical episodes. ADAMTS13 inhibitor testing was performed in nine out of 57 patients (16%), reflecting the limited availability of accredited testing facilities. Sixty-seven out of 72 episodes were treated with therapeutic plasma exchange (PEx) using cryodepleted plasma (40% of episodes), fresh frozen plasma (36%) or a mixture (22%). Median exposure to plasma products was 55.9L. PEx was commenced >=2days from stated diagnosis in 15% of episodes. Adverse reactions to PEx were common with documented allergic reactions (including life threatening) in 21% of episodes. Adjunctive immunosuppression was documented in 76% of episodes (corticosteroid 71% and rituximab 39%). Platelet transfusion was administered in 15% of episodes. Conclusion(s): Data from the Australian TTP/TMA registry suggest a heterogenous approach to the diagnosis and management of TTP in Australia over the assessed period. These observations highlight areas for improvement and standardisation of practice, including comprehensive diagnostic testing, more immediate access to PEx and a more uniform approach to adjunctive immunosuppression and supportive care.Copyright © 2016 Royal Australasian College of Physicians.

مصطلحات الفهرس: thrombotic thrombocytopenic purpura/ep [Epidemiology], thrombotic thrombocytopenic purpura/th [Therapy], treatment duration, corticosteroid/dt [Drug Therapy], cyclophosphamide/dt [Drug Therapy], rituximab/dt [Drug Therapy], vincristine/dt [Drug Therapy], von Willebrand factor cleaving proteinase/ec [Endogenous Compound], medical history, adult, allergic reaction/co [Complication], article, Australia, Australian, disease association, disease registry, female, human, immunosuppressive treatment, laboratory diagnosis, major clinical study, male, patient information, plasma transfusion, plasmapheresis, practice guideline, priority journal, thrombocyte transfusion, thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/dt [Drug Therapy], Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/39661Test
Internal Medicine Journal
LibKey Link

مستخلص: Atypical hemolytic uremic syndrome (aHUS) is a very rare, life-threatening, progressive disease that frequently has a genetic component and in most cases is triggered by an uncontrolled activation of the complement system. Successful treatment of aHUS with plasma infusions and therapeutic plasma exchange (TPE) is well reported. TPE has been the treatment of choice in most adult patients with aHUS. However, due to severe hemolysis, which is common among aHUS patients, there are some technical challenges that can affect TPE treatment such as the continuous activation of the blood leak alarm due to hemolysis. Our experience shows that such patients can be managed better on a centrifuge based TPE machine compared to a membrane based TPE machine. © 2013 Zimbudzi.

مصطلحات الفهرس: fever, flow rate, follow up, glomerulus filtration rate, hematocrit, hemolytic uremic syndrome/dt [Drug Therapy], hemolytic uremic syndrome/th [Therapy], hospital discharge, human, human cell, kidney dysfunction, lactate dehydrogenase blood level, lipoprotein blood level, medical device, medical device complication/co [Complication], normochromic normocytic anemia/di [Diagnosis], plasmapheresis, potassium blood level, protein blood level, relapse, remission, sore throat, thrombocyte count, thrombocytopenia/di [Diagnosis], thrombotic thrombocytopenic purpura/di [Diagnosis], urea blood level, urine color, vital sign, C reactive protein/ec [Endogenous Compound], creatinine/ec [Endogenous Compound], eculizumab/dt [Drug Therapy], haptoglobin/ec [Endogenous Compound], hemoglobin/ec [Endogenous Compound], lactate dehydrogenase/ec [Endogenous Compound], low density lipoprotein/ec [Endogenous Compound], potassium/ec [Endogenous Compound], urea/ec [Endogenous Compound], blood leak alarm/co [Complication], centrifuge based therapeutic plasma exchange machine, membrane based therapeutic plasma exchange machine, hemolysis, adult, arteriovenous fistula, article, biochemistry, blood examination, blood vessel catheterization, case report, creatinine blood level, drug bioavailability, erythrocyte, erythrocyte count, female, Article

URL: Click here for full text options
LibKey Link