Presentation
On cessation of HU the DM-LE clears avoiding unnecessary immunosuppression and demonstrating the importance of consideration of medication aetiology in DM presentations. We present a case report and review of the literature. [ncbi.nlm.nih.gov]
Presenters are required to disclose only those relationships that are relevant to their specific presentation. [hopkinsmedicine.org]
An 18-year-old woman with sickle cell anemia presents with recurrent painful crises, and treatment with hydroxyurea is recommended. Hydroxyurea causes a shift toward the production of red cells containing fetal hemoglobin. [doi.org]
Entire Body System
- Hypoxemia
Chronic hypoxemia is a common manifestation among patients with sickle cell anemia (SCA) who develop chronic lung disease. [ncbi.nlm.nih.gov]
There is observational evidence that hydroxycarbamide may improve hypoxemia, prevent cerebrovascular disease and reduce proteinuria. 18 The evidence for these applications is provisional, although encouraging in some cases. [doi.org]
Respiratoric
- Pleural Effusion
We describe intrathoracic and symptomatic pelvic EH masses in a 48-year-old woman and intrathoracic bilateral masses causing respiratory insufficiency with pleural effusion in a 42-year-old male, both affected by thalassaemia intermedia. [ncbi.nlm.nih.gov]
Psychiatrical
- Social Isolation
isolation (r s = -0.78, P = 0.02). [ncbi.nlm.nih.gov]
Workup
Serum
- Macrocytosis
The exact mechanism of action of HU is not known although the induction of Hb F synthesis and macrocytosis seem to be apparent possible mechanisms [ 7 - 10 ]. [omicsonline.org]
[…] patients develop some toxicity, which may occur at any time during a course of treatment: Leucopaenia (reduced white cell count) Anaemia (reduced red cell count) Thrombocytopenia (reduced platelet count) Pancytopaenia (reduction in all blood cell types) Macrocytosis [dermnetnz.org]
[…] effect with Siklos (seen in more than one patient in 10) is bone marrow suppression, causing neutropenia (low levels of neutrophils, a type of white blood cell), reticulocytopenia (low levels of reticulocytes, a type of immature red blood cell) and macrocytosis [emea.europa.eu]
- Liver Enzymes Abnormal
enzyme abnormalities as well [bilirubin 1.1 mg/dL, ALT 108-124 U/L, Alk P 104-140 U/L]). [livertox.nih.gov]
Pleura
- Pleural Effusion
We describe intrathoracic and symptomatic pelvic EH masses in a 48-year-old woman and intrathoracic bilateral masses causing respiratory insufficiency with pleural effusion in a 42-year-old male, both affected by thalassaemia intermedia. [ncbi.nlm.nih.gov]
Treatment
Complete remission was observed after 6 to 10 weeks of imiquimod 5% (10 mg/cm of skin cancer) treatment. [ncbi.nlm.nih.gov]
Prognosis
We know that patients who have intolerance to hydroxyurea, due to hematologic toxicity, are patients defined in a couple of studies as having a worse prognosis. [targetedonc.com]
Awareness of the possibility of necrotizing fasciitis following hip replacements is warranted in patients with MPNs on hydroxyurea as the prognosis is poor without early aggressive treatment with antibiotics and surgical debridement. [omicsonline.org]
Introduction Recent progress in the therapy of sickle cell disease, particularly the use of hydroxyurea, has considerably improved the prognosis of patients with this disease. 1, 2 Their mean life expectancy is currently about 50 years, 3 their quality [haematologica.org]
Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874–81. View Article PubMed Central PubMed Google Scholar Bjorkholm M, Hultcrantz M, Derolf AR. [ehoonline.biomedcentral.com]
Etiology
In males, copegus bijsluiter 40mg the etiology is more often related to alcohol; in females, it is more often related to biliary tract disease. [thebandhustle.co.uk]
The etiologies of DM-LE, HU-NMSC, and HUSD are not fully elucidated; however, they appear to share similar mechanisms involving direct effects of both HU and UVR. [jamanetwork.com]
Development of new painful oral ulcers, histo-pathologic findings of which were not unlike those in the colon led us to suspect that etiology of ulcers at these various sites was the same. [bmcgastroenterol.biomedcentral.com]
Acknowledgements Supported by the Office of the Secretary of Health for the State of Rio de Janeiro and the Brazilian Ministry of Health References Steinberg MH (2008) Sickle cell anemia, the first molecular disease: overview of molecular etiology, pathophysiology [omicsonline.org]
The underlying etiology is thought to be an infarctive event secondary to large-vessel stenosis or occlusion ( 4 ). [ajnr.org]
Epidemiology
Bronx, NY, United States. 3 Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, United States. 4 Children's Hospital at Montefiore, Department of Pediatrics, Division of Hematology/Oncology, Bronx, NY, United States. 5 Dept. of Epidemiology [ncbi.nlm.nih.gov]
Carrying out the Public Health Research Epidemiology and Surveillance for Hemoglobinopathies (PHRESH) project in California, Georgia and Mississippi, which will strengthen and improve upon the information gathering strategies developed in RuSH to help [cdc.gov]
Felix AA, Souza HM, Ribeiro SB (2010) Epidemiologic and social aspects of sickle cell disease (in Portuguese). Rev Bras Hematol Hemoter 32: 203-208. [omicsonline.org]
Epidemiology of psoriasis. In: Sharma VK, editor. Practical guidelines for the management of psoriasis. 1st ed. New Delhi: Department of Dermatology and Venereology, AIIMS, New Delhi; 2002. p. 1-3. 2. Kumar B, Saraswat A, Kaur I. [web.archive.org]
Pathophysiology
However, elucidation of the multiple pathophysiologic mechanisms leading to vaso-occlusion and tissue injury in SCD has now resulted in a burgeoning effort to identify new treatment modalities to prevent or ameliorate the consequences of the disease. [ncbi.nlm.nih.gov]
Inati A, Koussa S, Taher A, Perrine S (2008) Sickle cell disease: new insights into pathophysiology and treatment. Pediatr Ann 37: 311-321. [omicsonline.org]
Marvelle Brown, Sickle Cell Disease and Thalassaemia: Pathophysiology, Care And Management, Haematology Nursing, (117-149), (2013) ., Spezielle Arzneimitteltherapie in der Schwangerschaft, Arzneimittel in Schwangerschaft und Stillzeit, 10.1016/B978-3- [doi.org]
OBJECTIVES After participating in this activity, the participant will demonstrate the ability to: - Describe the basic pathophysiology of a vaso-occlusive crisis. - Describe the role of hemoglobin F in sickle cell disease. - Outline the evidence of hydroxyurea [hopkinsmedicine.org]
Prevention
Enzyme Inhibitors – Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction. [en.wikipedia.org]
[…] potentially lead to the formation of a malignant tumour, but DNA damage does not lead inevitably to the creation of cancerous cells. antimetabolite A substance which is structurally similar to a metabolite but which competes with it or replaces it, and so prevents [ebi.ac.uk]
HU initiation for disease severity prevention decreases the prevalence of abnormal TCDi velocities. © 2016 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]