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Priapism in Sickle Cell Anemia

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1. Re.: A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial; the Priapism in Sickle Cell Study (PISCES study). (PubMed)

Re.: A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial; the Priapism in Sickle Cell Study (PISCES study). 22088730 2016 02 05 2018 12 01 1527-3792 185 5 2011 May The Journal of urology J. Urol. Re.: A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial; the Priapism in Sickle Cell Study (...) (PISCES study). 1837-8 10.1016/S0022-5347(11)60219-X Seftel Allen D AD eng Journal Article Comment United States J Urol 0376374 0022-5347 0 Adrenergic alpha-2 Receptor Agonists AIM IM J Androl. 2011 Jul-Aug;32(4):375-82 21127308 Adrenergic alpha-2 Receptor Agonists therapeutic use Anemia, Sickle Cell complications Humans Male Priapism drug therapy Stuttering complications 2011 11 18 6 0 2011 11 18 6 0 2016 2 6 6 0 ppublish 22088730 S0022-5347(11)60219-X 10.1016/S0022-5347(11)60219-X

2016 The Journal of urology

2. Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength (PubMed)

Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength To investigate the association between priapism in men with sickle cell anemia (SCA) and hemorheological and hemolytical parameters.Fifty-eight men with SCA (median age: 38 years) were included; 28 who had experienced priapism at least once during their life (priapism group) and 30 who never experienced this complication (control group (...) ). Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion. Hematological and biochemical parameters were obtained through routine procedures. The Laser-assisted Optical Rotational Cell Analyzer was used to measure red blood cell (RBC) deformability at 30 Pa (ektacytometry) and RBC aggregation properties (laser backscatter versus time). Blood viscosity was measured at a shear rate of 225 s-1 using a cone/plate viscometer

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2016 PloS one

3. Priapism in Sickle Cell Anemia

Priapism in Sickle Cell Anemia Priapism in Sickle Cell Anemia Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Priapism in Sickle Cell (...) Anemia Priapism in Sickle Cell Anemia Aka: Priapism in Sickle Cell Anemia From Related Chapters II. Epidemiology of Priapism in Sickle Cell Anemia: 89% by age 20 years old III. Management Duration: <2 hours s IV Fluids Do not transfuse for <4 hours Duration: 2-4 hours First Intracavernosal (1:1,000,000 dilution) or injection Other measures 10 mg (in repeated doses) Duration >4 hours Administer above measures if not already attempted Exchange transfusion (Risk of CVA symptoms) Urology indications

2018 FP Notebook

4. MKSAP: 24-year-old woman with sickle cell anemia

MKSAP: 24-year-old woman with sickle cell anemia In sickle cell disease, transfusion is not indicated for uncomplicated pregnancy MKSAP: 24-year-old woman with sickle cell anemia | | January 21, 2017 2 Shares Test your medicine knowledge with the , in partnership with the . A 24-year-old woman undergoes routine evaluation. She is pregnant at 12 weeks’ gestation. Medical history is notable for homozygous sickle cell anemia (Hb SS). She has had multiple uncomplicated painful crises treated (...) of the following is the most appropriate management? A: Erythrocyte transfusion to maintain hemoglobin level at 10 g/dL (100 g/L) B: Erythropoiesis-stimulating agent C: Exchange transfusion D: No transfusion at this time MKSAP Answer and Critique The correct answer is D: No transfusion at this time. The patient should not receive an erythrocyte transfusion at this time. Erythrocyte transfusion in sickle cell disease (SCD) is appropriate only for specific indications, including stroke, symptomatic anemia, acute

2017 KevinMD blog

5. Degree of Anemia, Indirect Markers of Hemolysis, and Vascular Complications of Sickle Cell Disease in Africa. (PubMed)

Degree of Anemia, Indirect Markers of Hemolysis, and Vascular Complications of Sickle Cell Disease in Africa. The hyperhemolysis paradigm that describes overlapping "hyperhemolytic-endothelial dysfunction" and "high hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North American studies. We performed a transversal study nested in the CADRE cohort to analyze the association between steady-state hemolysis and vascular complications of SCD among sub-Saharan (...) African patients. In Mali, Cameroon, and Ivory Coast, 2407 SCD patients (1751 SS or sickle β-zero-thalassemia [Sβ0], 495 SC, and 161 sickle β+-thalassemia [Sβ+]), aged 3 years old and over, were included at steady state. Relative hemolytic intensity was estimated from a composite index derived from principal component analysis, which included bilirubin levels or clinical icterus, and lactate dehydrogenase levels. We assessed vascular complications (elevated tricuspid regurgitant jet velocity [TRV

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2017 Blood

6. Kidney Function in Sickle Cell Anemia

Kidney Function in Sickle Cell Anemia Kidney Function in Sickle Cell Anemia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Kidney Function in Sickle Cell Anemia The safety and scientific validity (...) characteristics for the worsening of kidney function and for a rapid decline in kidney function. "Funding Source - FDA OOPD" Condition or disease Sickle Cell Disease Kidney Failure, Chronic Detailed Description: Sickle cell disease is a severe monogenic disorder which affects approximately 80,000 patients in the US. It is characterized by a vasculopathy with involvement of multiple organs and resulting in complications such as ischemic stroke, pulmonary hypertension, autosplenectomy, priapism, as well

2017 Clinical Trials

7. Acute Crises and Complications of Sickle Cell Anemia Among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic Of Congo (PubMed)

Acute Crises and Complications of Sickle Cell Anemia Among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic Of Congo In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients (...) . This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1-24 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8-250 months). The median age at the first

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2017 Hematology reports

8. Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease

prevent priapism (2D) In children and adults with SS/Sβ 0 and symptomatic chronic anaemia that interferes with daily activities or quality of life, recommend treatment with hydroxycarbamide (1C) Use in other genotypes Hydroxycarbamide has been less well investigated in SCD phenotypes other than SS/Sβ 0 thalassaemia genotypes. Although there are no completed randomised clinical trials of hydroxycarbamide use in patients with sickle cell/HbC (SC) disease, cohort studies suggest a beneficial role (...) , making evidence‐based counselling of risk of developing sperm abnormalities or infertility challenging. Baseline sperm abnormalities exist in men with sickle cell disease. Berthaut et al ( ) carried out a retrospective multicentre study and evaluated the sperm parameters and fertility of 44 patients and the potential impact of hydroxycarbamide. At least 1 sperm parameter was found to be abnormal in 91% of patients pre‐treatment. This abnormality is seen whether or not clinical priapism or testicular

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2018 British Committee for Standards in Haematology

9. Red cell transfusion in sickle cell disease Part l

, R.J., McKie, V.C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., Abboud, M., Gallagher, D., Kutlar, A., Nichols, F.T., Bonds, D.R. & Brambilla, D. (1998) Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. The New England Journal of Medicine, 339, 5- 11. Alexy, T., Pais, E., Armstrong, J.K., Meiselman, H.J., Johnson, C.S. & Fisher, T.C. (2006) Rheologic behavior of sickle and normal red blood cell mixtures (...) ". Arch Intern Med, 111, 286-294. Aygun, B., Padmanabhan, S., Paley, C. & Chandrasekaran, V. (2002) Clinical significance of RBC alloantibodies and autoantibodies in sickle cell patients who received transfusions. Transfusion, 42, 37-43. Bachmeyer, C., Maury, J., Parrot, A., Bachir, D., Stankovic, K., Girot, R. & Lionnet, F. (2010) Rituximab as an effective treatment of hyperhemolysis syndrome in sickle cell anemia. Am J Hematol, 85, 91-92. Ballas, S.K. (2001) Iron overload is a determinant

2016 British Committee for Standards in Haematology

10. Red cell transfusion in sickle cell disease Part II

, A.K., Shehata, N., D'Souza, R., Kuo, K.H., Ward, R., Shah, P.S. & Murphy, K. (2015) Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood, 126, 2424-2435. McCarthy, L.J., Vattuone, J., Weidner, J., Skipworth, E., Fernandez, C., Jackson, L., Rothenberger, S., Waxman, D., Miraglia, C., Porcu, P. & Danielson, C.F. (2000) Do automated red cell exchanges relieve priapism in patients with sickle cell anemia? Ther Apher, 4, 256-258. McKinney (...) , C.M., Siringo, F., Olson, J.L., Capocelli, K.E., Ambruso, D.R. & Nuss, R. (2015) Red cell exchange transfusion halts progressive proliferative sickle cell retinopathy in a teenaged patient with hemoglobin SC disease. Pediatr Blood Cancer, 62, 721-723. Merritt, A.L., Haiman, C. & Henderson, S.O. (2006) Myth: blood transfusion is effective for sickle cell anemia-associated priapism. CJEM, 8, 119-122. Miller, S.T., Wright, E., Abboud, M., Berman, B., Files, B., Scher, C.D., Styles, L., Adams, R.J

2016 British Committee for Standards in Haematology

11. A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study. (PubMed)

A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study. Priapism is defined as a prolonged, persistent, and purposeless penile erection. It is a common (35%) but frequently understated complication in young men and adults with sickle cell disease. We had previously demonstrated an association between stuttering attacks (<4 hours) and an acute catastrophic event (...) ). None of the patients who completed the study required penile aspiration at study sites while on medical prophylaxis. Young men with sickle cell disease are not comfortable engaging with health care providers about issues relating to their sexual health. The full impact of an improved awareness campaign and early presentation to hospital merits further standardized study. Priapism still contributes seriously to the comorbidity experienced by this previously inaccessible group of patients and medical

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2011 Journal of andrology

12. CRACKCAST E121 – Anemia, Polycythemia, and White Blood Cell Disorders

] List 4 intrinsic and 4 extrinsic causes of hemolytic anemia Refer to box 112.8 for intrinsic and extrinsic causes of hemolytic anemia Intrinsic Causes of Hemolytic Anemia Enzyme defect (pyruvate kinase deficiency, G6PD deficiency) Membrane defect (spherocytosis, elliptostomatocytosis, paroxysmal nocturnal hemoglobinuria) Hemoglobin defect (Thalassemias, hemoglobin M, unstable hemoglobin, sickle cell) Extrinsic Causes of Hemolytic Anemia Immunologic (allo or autoantibodies) Mechanical (...) will have specific RBC morphologies suggesting etiology (bite cells, sickle cells, spherocytes, spur cells, Howell-Jolly bodies, etc.) Intravascular – peripheral smear will predominantly show schistocytes (RBC fragments) [17] Which types of hemolytic anemia tend to be intravascular? Which are extravascular? Extravascular – enzyme/membrane/hemoglobin defects, autoimmune hemolysis, toxins, hypersplenism Intravascular – MAHA (DIC, TTP, HUS), transfusion reactions, sepsis, heat injury, paroxysmal nocturnal

2017 CandiEM

13. Prevention of Ischemic Priapism in Sickle Cell Disease: Sildenafil. (PubMed)

Prevention of Ischemic Priapism in Sickle Cell Disease: Sildenafil. 26431892 2016 04 21 2018 12 02 1527-9995 86 6 2015 Dec Urology Urology Prevention of Ischemic Priapism in Sickle Cell Disease: Sildenafil: Commentary on: Randomized Controlled Trial of Sildenafil for Preventing Recurrent Ischemic Priapism in Sickle Cell Disease. 1055-6 10.1016/j.urology.2015.07.003 S0090-4295(15)00650-0 Shakeri Aria A Division of Urology, Department of Surgery, University of Toronto, Toronto, Ontario, Canada (...) . Van Asseldonk Brandon B Division of Urology, Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Elterman Dean S DS Division of Urology, Department of Surgery, University of Toronto, Toronto, Ontario, Canada. eng Journal Article Comment 2015 09 30 United States Urology 0366151 0090-4295 0 Phosphodiesterase 5 Inhibitors 0 Piperazines 0 Sulfones IM Am J Med. 2014 Jul;127(7):664-8 24680796 Anemia, Sickle Cell complications Humans Ischemia complications prevention & control Male

2015 Urology

14. Priapism in Sickle Cell Anemia

Priapism in Sickle Cell Anemia Priapism in Sickle Cell Anemia Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Priapism in Sickle Cell (...) Anemia Priapism in Sickle Cell Anemia Aka: Priapism in Sickle Cell Anemia From Related Chapters II. Epidemiology of Priapism in Sickle Cell Anemia: 89% by age 20 years old III. Management Duration: <2 hours s IV Fluids Do not transfuse for <4 hours Duration: 2-4 hours First Intracavernosal (1:1,000,000 dilution) or injection Other measures 10 mg (in repeated doses) Duration >4 hours Administer above measures if not already attempted Exchange transfusion (Risk of CVA symptoms) Urology indications

2015 FP Notebook

15. Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy. (PubMed)

Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy. Priapism describes a persistent erection lasting longer than 4 hours. Ischemic priapism and stuttering priapism are phenotypic manifestations of sickle-cell disease (SCD).To define the types of priapism associated with SCD, to address pathogenesis, and to recommend best practices.Literature review and published clinical guidelines.Priapism is a full or partial erection that persists more than 4 hours. There are three kinds (...) of priapism: ischemic priapism (veno-occlusive, low flow), stuttering priapism (recurrent ischemic priapism), and nonischemic priapism (arterial, high flow). Ischemic priapism is a pathologic phenotype of SCD. Ischemic priapism is a urologic emergency when untreated priapism results in corporal fibrosis and erectile dysfunction. The recommended treatment for ischemic priapism is decompression of the penis by needle aspiration and if needed, injection (or irrigation) with dilute sympathomimetic drugs

2012 Journal Of Sexual Medicine

16. A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe Sickle Cell Disease

: Plerixafor Phase: Phase 1/2 Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Busulfan Plerixafor octahydrochloride Alkylating Agents Molecular Mechanisms of Pharmacological Action Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antineoplastic Agents, Alkylating Antineoplastic Agents Myeloablative Agonists Anti-HIV Agents Anti (...) A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe Sickle Cell Disease A Study to Assess the Safety, Tolerability, and Efficacy of BIVV003 for Autologous Hematopoietic Stem Cell Transplantation in Patients With Severe Sickle Cell Disease - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms

2018 Clinical Trials

17. Sickle cell disease

. Has a sudden severe infection. Presents with features of an acute crisis (or a history of features consistent with an acute crisis). Presents with a chronic complication of sickle cell disease (such as chronic anaemia or chronic sickle lung). A sickle cell crisis should be suspected if there is a sudden onset of pain, infection, anaemia, or other symptoms (such as a stroke or priapism). There is often a history of a previous crisis. All people with sickle cell disease will be followed up regularly (...) complications in people with sickle cell disease, and it occurs in people of all ages and genotypes [ ]. It is most commonly caused by Salmonella species, Gram-negative enteric bacteria, and Staphylococcus aureus . Recurrent bone infarcts may lead to the formation of bone sequestra. These may become secondarily infected, causing osteomyelitis. There may be local warmth, swelling, tenderness, and fever (an identical presentation to acute painful crisis). Acute priapism This is a persistent painful erection

2017 NICE Clinical Knowledge Summaries

18. A Blood Stem Cell Transplant for Sickle Cell Disease

Sickle Cell Disease Sickle Cell Disorder Hemoglobinopathies Thalassemia Anemia, Sickle Cell Drug: Cyclophosphamide Drug: Pentostatin Drug: Rabbit anti-thymocyte globulin Drug: Tacrolimus Drug: Mycophenolate mofetil Biological: CD4+ T-cell-depleted Haploidentical Hematopoietic Transplant Phase 1 Detailed Description: This is a pilot study to determine the safety and feasibility of the COH-MC-17 regimen and ability of the regimen to induce a mixed chimeric status in severe sickle cell disease patients (...) : City of Hope Medical Center ClinicalTrials.gov Identifier: Other Study ID Numbers: 16453 First Posted: August 15, 2017 Last Update Posted: January 15, 2019 Last Verified: January 2019 Layout table for additional information Studies a U.S. FDA-regulated Drug Product: Yes Studies a U.S. FDA-regulated Device Product: No Keywords provided by City of Hope Medical Center: Sickle Cell Disease Sickle Cell Disorders Hemoglobinopathies Thalassemia Anemia, Sickle Cell Haploidentical Transplant

2017 Clinical Trials

19. Nonmyeloablative Stem Cell Transplant in Children With Sickle Cell Disease and a Major ABO-Incompatible Matched Sibling Donor

of priapism in males history of osteonecrosis pulmonary hypertension as documented by tricuspid regurgitation jet velocity (TRV) > 2.5 m/s on echocardiogram red cell allo-immunization (≥ 2 antibodies) during long term transfusion therapy Sickle complications should be present despite the use of hydroxyurea, but this is not an absolute requirement, if the treating team considers the patient to be at high risk for further crisis episodes. Exclusion Criteria: Patients who are unable to comply with or follow (...) Drug Product: No Studies a U.S. FDA-regulated Device Product: No Product Manufactured in and Exported from the U.S.: No Keywords provided by Tony H. Truong, University of Calgary: sickle cell disease stem cell transplant red blood cell engraftment nonmyeloablative pure red cell aplasia Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Red-Cell Aplasia, Pure Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases

2017 Clinical Trials

20. Study of HLA-Haploidentical Stem Cell Transplantation to Treat Clinically Aggressive Sickle Cell Disease

Healthy Volunteers: No Criteria Patient Eligibility: Patients with sickle cell disease are eligible if they have any of the following complications: 1.1 Stroke or central nervous system event lasting longer than 24 hours 1.2 Frequent vaso-occlusive pain episodes, defined as ≥ 3 per year requiring emergency room, acute care center, hospital admissions, or home bedrest leading to absence from work or school. 1.3 Recurrent episodes of priapism, defined as ≥ 2 per year requiring emergency room visits 1.4 (...) , Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Cyclophosphamide Fludarabine phosphate Sirolimus Everolimus Thymoglobulin Fludarabine Mycophenolic Acid Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antirheumatic Agents Antineoplastic Agents, Alkylating Alkylating Agents Molecular Mechanisms of Pharmacological Action Antineoplastic Agents Myeloablative Agonists Antimetabolites

2017 Clinical Trials

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