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
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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
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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.

مصطلحات الفهرس: eculizumab, monoclonal antibody/ad [Drug Administration], monoclonal antibody/dt [Drug Therapy], preeclampsia/dt [Drug Therapy], adult, case report, complication, diabetes mellitus, differential diagnosis, female, hemolytic uremic syndrome/di [Diagnosis], hemolytic uremic syndrome/dt [Drug Therapy], human, preeclampsia/di [Diagnosis], pregnancy, pregnancy complication, prenatal diagnosis, prolonged pregnancy, second trimester pregnancy, Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/35522Test
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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.

مصطلحات الفهرس: eculizumab, monoclonal antibody/ad [Drug Administration], monoclonal antibody/dt [Drug Therapy], preeclampsia/dt [Drug Therapy], adult, case report, complication, diabetes mellitus, differential diagnosis, female, hemolytic uremic syndrome/di [Diagnosis], hemolytic uremic syndrome/dt [Drug Therapy], human, preeclampsia/di [Diagnosis], pregnancy, pregnancy complication, prenatal diagnosis, prolonged pregnancy, second trimester pregnancy, Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/35522Test
Obstetrics and Gynecology
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مستخلص: Aims: To describe the baseline characteristics and treatment of Australian patients diagnosed with atypical haemolytic uraemic syndrome (aHUS) reported to the Global aHUS Registry. Method(s): Descriptive analysis of the Australian cohort with aHUS (n = 106) was undertaken for demographics, disease characteristics and prior treatment with eculizumab; comparing with the global cohort (n = 1688) for certain pre-specified disease characteristics. Result(s): In Australia, almost two-thirds of patients diagnosed with aHUS were female and over 80% of patients were Caucasians, with similar proportions reported in the global cohort. Less than 6% of patients in the Australia and global cohorts were reported to have a history of autoimmune disease (4% vs 2%, respectively; P =.21) or cancer (5% vs 5%, respectively; P =.93), conditions that have been associated with secondary HUS. In the Australian cohort, 26% had received a kidney transplant and 68% of patients had received eculizumab. Kidneys were the most common organ involvement, followed by gastrointestinal tract (26%) and cardiovascular system (19%), with 35% of patients reported to have had at least two organs involved within 6 months prior to baseline visit or entry into the registry. Complement factor H was the most common pathogenic complement gene variant in the Australian patients. Conclusion(s): Data from the aHUS registry confirms and defines region-specific disease characteristics among a selected group of Australian children and adults with aHUS reported to the registry. Ongoing and more inclusive data will provide further information about temporal trends and treatment outcomes, representing a unique opportunity for clinicians and researchers to further develop knowledge surrounding this rare disease.Copyright © 2020 Asian Pacific Society of Nephrology

مصطلحات الفهرس: controlled study, demography, disease registry, ethnic difference, family history, female, gastrointestinal disease, genetic variability, hemodialysis, hemolytic uremic syndrome/dt [Drug Therapy], human, kidney disease/su [Surgery], kidney disease/th [Therapy], kidney graft, kidney transplantation, major clinical study, complement factor H/ec [Endogenous Compound], Caucasian, clinical feature, cohort analysis, priority journal, sex difference, trend study, cardiovascular disease, eculizumab/dt [Drug Therapy], adult, age distribution, article, Australian, autoimmune disease, male, malignant neoplasm, medical history, Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/29108Test
Nephrology
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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
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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
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مستخلص: Background/Aim: This study aimed to report the clinical characteristics and outcomes of Australian patients treated with eculizumab for atypical haemolytic uraemic syndrome (aHUS). Method(s): A retrospective cohort study was undertaken of all patients in Australia treated with eculizumab provided in a compassionate access programme for a clinical diagnosis of aHUS using prospectively collected clinical data. Result(s): A total of 10 patients with a median age of 23.5 years (interquartile range (IQR) 24.83 years) received compassionate access eculizumab for aHUS in Australia. Eight patients were female, and three had a family history of aHUS. Three received eculizumab for an initial acute aHUS presentation, three for relapsing and refractory acute aHUS, two for de novo aHUS post-renal transplantation, and one each for aHUS recurrence post-transplantation and facilitation of transplantation with a history of aHUS. The median duration of eculizumab therapy has been 911.5 days (IQR 569 days) with a cumulative exposure of 9184 days. At baseline all patients had renal and extra-renal aHUS involvement, with up to three non-renal organs affected. All but one patient, who died from uncontrollable gastrointestinal aHUS manifestations, have continued. The nine continuing patients achieved remission of aHUS. Two of the four patients requiring renal replacement therapy (RRT) at eculizumab commencement subsequently ceased RRT. Clinical events occurring in this cohort while on eculizumab treatment included neutropenia (two), posterior reversible encephalopathy syndrome (one), cardiomyopathy (one), pulmonary embolus (one), antibody-mediated rejection resulting in renal graft failure (one), iron deficiency (one), gastrointestinal haemorrhage (one) and death (one). Conclusion(s): Eculizumab has been an effective therapy for aHUS in this cohort, including when other therapies have failed.Copyright © 2015 Royal Australasian College of Physicians.

مصطلحات الفهرس: genetic counseling, adult, article, Australia, Australian, cardiomyopathy/si [Side Effect], clinical article, cohort analysis, compassionate use, creatinine blood level, death, drug efficacy, drug fatality/si [Side Effect], female, gastrointestinal hemorrhage/si [Side Effect], hemolytic uremic syndrome/dt [Drug Therapy], hemolytic uremic syndrome/su [Surgery], hemolytic uremic syndrome/th [Therapy], human, infant, iron deficiency anemia/si [Side Effect], kidney function, kidney graft rejection/co [Complication], kidney transplantation, lung embolism/si [Side Effect], male, neutropenia/si [Side Effect], posterior reversible encephalopathy syndrome/si [Side Effect], priority journal, recurrent disease, remission, renal replacement therapy, retrospective study, thrombotic thrombocytopenic purpura, treatment response, complement inhibitor/dt [Drug Therapy], creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/do [Drug Dose], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/40687Test
Internal Medicine Journal
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مستخلص: Background/Aim: This study aimed to report the clinical characteristics and outcomes of Australian patients treated with eculizumab for atypical haemolytic uraemic syndrome (aHUS). Method(s): A retrospective cohort study was undertaken of all patients in Australia treated with eculizumab provided in a compassionate access programme for a clinical diagnosis of aHUS using prospectively collected clinical data. Result(s): A total of 10 patients with a median age of 23.5 years (interquartile range (IQR) 24.83 years) received compassionate access eculizumab for aHUS in Australia. Eight patients were female, and three had a family history of aHUS. Three received eculizumab for an initial acute aHUS presentation, three for relapsing and refractory acute aHUS, two for de novo aHUS post-renal transplantation, and one each for aHUS recurrence post-transplantation and facilitation of transplantation with a history of aHUS. The median duration of eculizumab therapy has been 911.5 days (IQR 569 days) with a cumulative exposure of 9184 days. At baseline all patients had renal and extra-renal aHUS involvement, with up to three non-renal organs affected. All but one patient, who died from uncontrollable gastrointestinal aHUS manifestations, have continued. The nine continuing patients achieved remission of aHUS. Two of the four patients requiring renal replacement therapy (RRT) at eculizumab commencement subsequently ceased RRT. Clinical events occurring in this cohort while on eculizumab treatment included neutropenia (two), posterior reversible encephalopathy syndrome (one), cardiomyopathy (one), pulmonary embolus (one), antibody-mediated rejection resulting in renal graft failure (one), iron deficiency (one), gastrointestinal haemorrhage (one) and death (one). Conclusion(s): Eculizumab has been an effective therapy for aHUS in this cohort, including when other therapies have failed.Copyright © 2015 Royal Australasian College of Physicians.

مصطلحات الفهرس: genetic counseling, adult, article, Australia, Australian, cardiomyopathy/si [Side Effect], clinical article, cohort analysis, compassionate use, creatinine blood level, death, drug efficacy, drug fatality/si [Side Effect], female, gastrointestinal hemorrhage/si [Side Effect], hemolytic uremic syndrome/dt [Drug Therapy], hemolytic uremic syndrome/su [Surgery], hemolytic uremic syndrome/th [Therapy], human, infant, iron deficiency anemia/si [Side Effect], kidney function, kidney graft rejection/co [Complication], kidney transplantation, lung embolism/si [Side Effect], male, neutropenia/si [Side Effect], posterior reversible encephalopathy syndrome/si [Side Effect], priority journal, recurrent disease, remission, renal replacement therapy, retrospective study, thrombotic thrombocytopenic purpura, treatment response, complement inhibitor/dt [Drug Therapy], creatinine/ec [Endogenous Compound], eculizumab/ae [Adverse Drug Reaction], eculizumab/do [Drug Dose], eculizumab/dt [Drug Therapy], eculizumab/iv [Intravenous Drug Administration], Article

URL: https://repository.monashhealth.org/monashhealthjspui/handle/1/40687Test
Internal Medicine Journal
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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

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