LIVIVO - Search results -

Search results

Result 1 - 9 of total 9

Search options

Article: Biology of anemia, differential diagnosis, and treatment options in human immunodeficiency virus infection.

Claster, Susan

2002 Volume 185 Suppl 2, Page(s) S105–9

Abstract: Anemia is the most common hematologic manifestation of human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome. The causes of HIV-related anemia are multifactorial and include direct and indirect effects of HIV infection. HIV- ... ...

Abstract	Anemia is the most common hematologic manifestation of human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome. The causes of HIV-related anemia are multifactorial and include direct and indirect effects of HIV infection. HIV-related anemia generally is due to reduced red blood cell (RBC) production, secondary to a variety of causes, but it may also involve nutritional deficiencies, increased RBC destruction, or a combination of these problems. Evaluation of hemoglobin level, reticulocyte count, bilirubin, and mean corpuscular volume value and review of the peripheral blood smear are necessary for diagnosis. Treatment of HIV-related anemia should address the correctable underlying causes of this disorder, such as modifications of offending medications, nutritional deficiencies, and parvovirus infection. Patients with HIV infection have a blunted erythropoietin response to anemia. Therapeutic modalities for anemia that is not amenable to correction include blood transfusion and recombinant human erythropoietin (epoetin alfa).
MeSH term(s)	Acquired Immunodeficiency Syndrome/complications ; Anemia/diagnosis ; Anemia/etiology ; Anemia/therapy ; Anti-HIV Agents/therapeutic use ; Diagnosis, Differential ; HIV Infections/complications ; HIV Infections/drug therapy ; Humans
Chemical Substances	Anti-HIV Agents
Language	English
Publishing date	2002-05-15
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	3019-3
ISSN	1537-6613 ; 0022-1899
ISSN (online)	1537-6613
ISSN	0022-1899
DOI	10.1086/340202
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Uh II Zs.50: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
Zs.MO 445: Show issues

Order via subito

This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects.

Kanter, Julie / Smith, Wally R / Desai, Payal C / Treadwell, Marsha / Andemariam, Biree / Little, Jane / Nugent, Diane / Claster, Susan / Manwani, Deepa G / Baker, Judith / Strouse, John J / Osunkwo, Ifeyinwa / Stewart, Rosalyn W / King, Allison / Shook, Lisa M / Roberts, John D / Lanzkron, Sophie

Blood advances

2020 Volume 4, Issue 16, Page(s) 3804–3813

Abstract: Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD ... ...

Abstract	Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.
MeSH term(s)	Adult ; Anemia, Sickle Cell/therapy ; Child ; Health Services Accessibility ; Hematologic Diseases ; Humans ; United States
Language	English
Publishing date	2020-08-12
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2915908-8
ISSN	2473-9537 ; 2473-9529
ISSN (online)	2473-9537
ISSN	2473-9529
DOI	10.1182/bloodadvances.2020001743
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 7153: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Managing sickle cell disease.

Claster, Susan / Vichinsky, Elliott P

BMJ (Clinical research ed.)

2003 Volume 327, Issue 7424, Page(s) 1151–1155

MeSH term(s)	Adolescent ; Adult ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/therapy ; Blood Transfusion/methods ; Bone Diseases/etiology ; Central Nervous System Diseases/etiology ; Child ; Eye Diseases/etiology ; Female Urogenital Diseases/etiology ; Forecasting ; Humans ; Lung Diseases/etiology ; Male Urogenital Diseases ; Pain/prevention & control ; Splenic Diseases/etiology
Language	English
Publishing date	2003-11-13
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1362901-3
ISSN	1756-1833 ; 0959-8154 ; 0959-8146 ; 0959-8138 ; 0959-535X ; 1759-2151
ISSN (online)	1756-1833
ISSN	0959-8154 ; 0959-8146 ; 0959-8138 ; 0959-535X ; 1759-2151
DOI	10.1136/bmj.327.7424.1151
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ua VI Zs.10: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Pitfalls of using administrative data sets to describe clinical outcomes in sickle cell disease.

Claster, Susan / Termuhlen, Amanda / Schrager, Sheree M / Wolfson, Julie A / Iverson, Ellen

Pediatric blood & cancer

2013 Volume 60, Issue 12, Page(s) 1936–1939

Abstract: Background: Administrative data sets are increasingly being used to describe clinical care in sickle cell disease (SCD). We recently used such an administrative database to look at the frequency of acute chest syndrome (ACS) and the use of transfusion ... ...

Abstract	Background: Administrative data sets are increasingly being used to describe clinical care in sickle cell disease (SCD). We recently used such an administrative database to look at the frequency of acute chest syndrome (ACS) and the use of transfusion to treat this syndrome in California patients from 2005 to 2010. Our results revealed a surprisingly low rate of transfusion for this life-threatening situation. Procedure: To validate these results, we compared California OSPHD (Office of Statewide Health Planning and Development) administrative data with medical record review of patients diagnosed with ACS identified by two pediatric and one adult hospital databases during 2009-2010. Results: ACS or a related pulmonary process accounted for one-fifth of the inpatient hospital discharges associated with the diagnosis of SCD between 2005 and 2010. Only 47% of those discharges were associated with a transfusion. However, chart reviews found that hospital databases over-reported visits for ACS. OSHPD underreported transfusions compared to hospital data. The net effect was a markedly higher true rate of transfusion (40.7% vs. 70.2%). Conclusions: These results point out the difficulties in using this administrative data base to describe clinical care for ACS given the variation in clinician recognition of this entity. OSPHD is widely used to inform health care policy in California and contributes to national databases. Our study suggests that using this administrative database to assess clinical care for SCD may lead to inaccurate assumptions about quality of care for SCD patients in California. Future studies on health services in SCD may require a different methodology.
MeSH term(s)	Acute Chest Syndrome/etiology ; Acute Chest Syndrome/therapy ; Anemia, Sickle Cell/complications ; Blood Transfusion ; California ; Data Collection/standards ; Databases, Factual/standards ; Hospitals ; Humans ; State Health Planning and Development Agencies ; Treatment Outcome ; United States
Language	English
Publishing date	2013-08-26
Publishing country	United States
Document type	Journal Article
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.24747
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 1131: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Nutritional deficiencies in iron overloaded patients with hemoglobinopathies.

Claster, Susan / Wood, John C / Noetzli, Leila / Carson, Susan M / Hofstra, Thomas C / Khanna, Rachna / Coates, Thomas D

American journal of hematology

2009 Volume 84, Issue 6, Page(s) 344–348

Abstract: One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies ... ...

Abstract	One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and trace mineral levels has never been performed in chronically transfused SCD or TM patients. As vitamins and trace minerals may be consumed as a result of chronic oxidative stress; we hypothesized that levels of these compounds would correlate with surrogates of iron overload, hemolysis, and inflammation in chronically transfused patients. Using a convenience sample of our group of chronically transfused patients we studied 43 patients with SCD (17 male, 26 female) and 24 patients with TM (13 male and 11 female). The age range for our patients varied from 1.5 to 31.4 years. Levels of vitamins A, thiamin, B6, B12, C, D, E as well as selenium, zinc, copper, and ceruloplasmin were measured. We found that 40-75% of the patients were deficient in A, C, D and selenium and 28-38% of the patients had low levels of B vitamins and folate. There was little association with iron overload, hemolysis, or inflammation. Although the precise mechanism of these deficiencies is unclear, they may contribute to the morbidity of chronically transfused hemoglobinopathy patients.
MeSH term(s)	Adolescent ; Adult ; Anemia, Sickle Cell/metabolism ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Iron Overload/metabolism ; Male ; Minerals/metabolism ; Nutritional Status ; Vitamins/metabolism ; Young Adult ; beta-Thalassemia/metabolism
Chemical Substances	Minerals ; Vitamins
Language	English
Publishing date	2009-05-05
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.21416
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 1251: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Vitamin D deficiency, cardiac iron and cardiac function in thalassaemia major.

Wood, John C / Claster, Susan / Carson, Susan / Menteer, J D / Hofstra, Thomas / Khanna, Rachna / Coates, Thomas

British journal of haematology

2008 Volume 141, Issue 6, Page(s) 891–894

Abstract: Vitamin D25-OH and D1-25OH levels were compared with cardiac R2* (1/T2*), left ventricular ejection fraction (LVEF), age, ferritin and liver iron in 24 thalassaemia major patients. Vitamin D25-OH levels were reduced in 13/24 patients while vitamin D1- ... ...

Abstract	Vitamin D25-OH and D1-25OH levels were compared with cardiac R2* (1/T2), left ventricular ejection fraction (LVEF), age, ferritin and liver iron in 24 thalassaemia major patients. Vitamin D25-OH levels were reduced in 13/24 patients while vitamin D1-25OH levels were often elevated. Vitamin D25-OH levels decreased with age (r(2) = 0.48) and with liver iron (r(2) = 0.20). Cardiac R2 was inversely related with the ratio of D25-OH to D1-25OH levels (r(2) = 0.42). LVEF was also proportional to the D25-OH/D1-25OH ratio (r(2) = 0.49). Vitamin D deficiency may be associated with cardiac iron uptake and ventricular dysfunction in thalassaemia major patients.
MeSH term(s)	Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Iron/analysis ; Iron/metabolism ; Iron Overload/etiology ; Iron Overload/metabolism ; Liver/chemistry ; Male ; Myocardium/metabolism ; Stroke Volume ; Transfusion Reaction ; Ventricular Dysfunction, Left/etiology ; Ventricular Function, Left ; Vitamin D Deficiency/complications ; Vitamin D Deficiency/metabolism ; Vitamin D Deficiency/physiopathology ; beta-Thalassemia/complications ; beta-Thalassemia/metabolism ; beta-Thalassemia/physiopathology ; beta-Thalassemia/therapy
Chemical Substances	Iron (E1UOL152H7)
Language	English
Publishing date	2008-03-26
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/j.1365-2141.2008.07135.x
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Uh III Zs.182: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article: von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.

Sahud, Mervyn A / Claster, Susan / Liu, Lucy / Ero, Michael / Harris, Kathryn / Furlan, Miha

British journal of haematology

1999 Volume 116, Issue 4, Page(s) 909–911

Abstract: Antibodies that inhibit von Willebrand Factor (VWF)-cleaving protease activity occur in patients with acute thrombotic thrombocytopenic purpura (TTP) and often persist in the chronic phase. A deficiency of this protease is likely to be responsible for ... ...

Abstract	Antibodies that inhibit von Willebrand Factor (VWF)-cleaving protease activity occur in patients with acute thrombotic thrombocytopenic purpura (TTP) and often persist in the chronic phase. A deficiency of this protease is likely to be responsible for the generation of ultrahigh VWF multimers and influence the formation of intra-arterial platelet aggregates that result in microangiopathic haemolytic anaemia, thrombocytopenia and end in organ failure. This report demonstrates complete deficiency of VWF-cleaving protease and the presence of a concentration-dependent IgG1 inhibitor in the plasma of a patient with acquired immunodeficiency syndrome (AIDS). These data may contribute to understanding the pathophysiology of human immunodeficiency syndrome (HIV)-related TTP.
MeSH term(s)	ADAM Proteins ; ADAMTS13 Protein ; Acquired Immunodeficiency Syndrome/complications ; Acquired Immunodeficiency Syndrome/immunology ; Autoantibodies/blood ; Clinical Enzyme Tests ; Humans ; Immunoglobulin G/blood ; Male ; Metalloendopeptidases/analysis ; Metalloendopeptidases/immunology ; Middle Aged ; Purpura, Thrombotic Thrombocytopenic/complications ; Purpura, Thrombotic Thrombocytopenic/immunology
Chemical Substances	Autoantibodies ; Immunoglobulin G ; ADAM Proteins (EC 3.4.24.-) ; Metalloendopeptidases (EC 3.4.24.-) ; ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87)
Language	English
Publishing date	1999-08-11
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1046/j.0007-1048.2002.03349.x
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Uh III Zs.182: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article: Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease?

Morris, Claudia R / Morris, Sidney M / Hagar, Ward / Van Warmerdam, Jane / Claster, Susan / Kepka-Lenhart, Diane / Machado, Lorenzo / Kuypers, Frans A / Vichinsky, Elliott P

American journal of respiratory and critical care medicine

2003 Volume 168, Issue 1, Page(s) 63–69

Abstract: Pulmonary hypertension is a life-threatening complication of sickle cell disease. L-Arginine is the nitrogen donor for synthesis of nitric oxide, a potent vasodilator that is deficient during times of sickle cell crisis. This deficiency may play a role ... ...

Abstract	Pulmonary hypertension is a life-threatening complication of sickle cell disease. L-Arginine is the nitrogen donor for synthesis of nitric oxide, a potent vasodilator that is deficient during times of sickle cell crisis. This deficiency may play a role in pulmonary hypertension. The enzyme arginase hydrolyzes arginine to ornithine and urea, and thus, it may compete with nitric oxide synthase, leading to decreased nitric oxide production. Nitric oxide therapy by inhalation has improved pulmonary hypertension associated with acute chest syndrome in sickle cell disease, and several studies demonstrate therapeutic benefits of arginine therapy for primary and secondary pulmonary hypertension. We sought to determine the effects of arginine therapy on pulmonary hypertension in patients with sickle cell disease. Arginase activity was also determined. Oral arginine produced a 15.2% mean reduction in estimated pulmonary artery systolic pressure (63.9 +/- 13 to 54.2 +/- 12 mm Hg, p = 0.002) after 5 days of therapy in 10 patients. Arginase activity was elevated almost twofold (p = 0.07) in patients with pulmonary hypertension and may limit arginine bioavailability. With limited treatment options and a high mortality rate for patients with sickle cell disease who develop pulmonary hypertension, arginine is a promising new therapy that warrants further investigation.
MeSH term(s)	Administration, Oral ; Adolescent ; Adult ; Amino Acids/blood ; Anemia, Sickle Cell/complications ; Arginase/blood ; Arginase/drug effects ; Arginine/metabolism ; Arginine/therapeutic use ; Biological Availability ; Case-Control Studies ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary/drug therapy ; Hypertension, Pulmonary/etiology ; Hypertension, Pulmonary/metabolism ; Hypertension, Pulmonary/physiopathology ; Male ; Middle Aged ; Nitric Oxide/metabolism ; Ornithine/blood ; Oximetry ; Pulmonary Wedge Pressure/drug effects ; Treatment Outcome
Chemical Substances	Amino Acids ; Nitric Oxide (31C4KY9ESH) ; Arginine (94ZLA3W45F) ; Ornithine (E524N2IXA3) ; Arginase (EC 3.5.3.1)
Language	English
Publishing date	2003-07-01
Publishing country	United States
Document type	Clinical Trial ; Controlled Clinical Trial ; Journal Article ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	1180953-x
ISSN	1535-4970 ; 1073-449X ; 0003-0805
ISSN (online)	1535-4970
ISSN	1073-449X ; 0003-0805
DOI	10.1164/rccm.200208-967OC
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Uh II Zs.80: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article: Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.

Steinberg, Martin H / Barton, Franca / Castro, Oswaldo / Pegelow, Charles H / Ballas, Samir K / Kutlar, Abdullah / Orringer, Eugene / Bellevue, Rita / Olivieri, Nancy / Eckman, James / Varma, Mala / Ramirez, Gloria / Adler, Brian / Smith, Wally / Carlos, Timothy / Ataga, Kenneth / DeCastro, Laura / Bigelow, Carolyn / Saunthararajah, Yogen /

Telfer, Margaret / Vichinsky, Elliott / Claster, Susan / Shurin, Susan / Bridges, Kenneth / Waclawiw, Myron / Bonds, Duane / Terrin, Michael

JAMA

2003 Volume 289, Issue 13, Page(s) 1645–1651

Abstract: Context: Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality.: Objective: To determine whether ... ...

Abstract	Context: Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality. Objective: To determine whether hydroxyurea attenuates mortality in patients with SCA. Design: Long-term observational follow-up study of mortality in patients with SCA who originally participated in the randomized, double-blind, placebo-controlled Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH), conducted in 1992-1995, to determine if hydroxyurea reduces vaso-occlusive events. In the MSH Patients' Follow-up, conducted in 1996-2001, patients could continue, stop, or start hydroxyurea. Data were collected during the trial and in the follow-up period. Setting: Inpatients and outpatients in 21 sickle cell referral centers in the United States and Canada. Patients: Two-hundred ninety-nine adult patients with frequent painful episodes enrolled in the follow-up. Follow-up data through May 2001 were complete for 233 patients. Intervention: In the MSH, patients were randomly assigned to receive hydroxyurea (n = 152) or placebo (n = 147). Main outcome measure: Mortality, HbF levels, painful episodes, acute chest syndrome, and blood cell counts. The randomized trial was not designed to detect specified differences in mortality. Results: Seventy-five of the original 299 patients died, 28% from pulmonary disease. Patients with reticulocyte counts less than 250 000/mm3 and hemoglobin levels lower than 9 g/dL had increased mortality (P =.002). Cumulative mortality at 9 years was 28% when HbF levels were lower than 0.5 g/dL after the trial was completed compared with 15% when HbF levels were 0.5 g/dL or higher (P =.03 ). Individuals who had acute chest syndrome during the trial had 32% mortality compared with 18% of individuals without acute chest syndrome (P =.02). Patients with 3 or more painful episodes per year during the trial had 27% mortality compared with 17% of patients with less frequent episodes (P =.06). Taking hydroxyurea was associated with a 40% reduction in mortality (P =.04) in this observational follow-up with self-selected treatment. There were 3 cases of cancer, 1 fatal. Conclusions: Adult patients taking hydroxyurea for frequent painful sickle cell episodes appear to have reduced mortality after 9 of years follow-up. Survival was related to HbF levels and frequency of vaso-occlusive events. Whether indications for hydroxyurea treatment should be expanded is unknown.
MeSH term(s)	Adult ; Anemia, Sickle Cell/drug therapy ; Anemia, Sickle Cell/mortality ; Anemia, Sickle Cell/physiopathology ; Antisickling Agents/therapeutic use ; Blood Cell Count ; Cause of Death ; Double-Blind Method ; Fetal Hemoglobin/metabolism ; Follow-Up Studies ; Humans ; Hydroxyurea/therapeutic use ; Morbidity ; Risk Assessment ; Survival Analysis
Chemical Substances	Antisickling Agents ; Fetal Hemoglobin (9034-63-3) ; Hydroxyurea (X6Q56QN5QC)
Language	English
Publishing date	2003-04-02
Publishing country	United States
Document type	Clinical Trial ; Journal Article ; Multicenter Study ; Randomized Controlled Trial ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	2958-0
ISSN	1538-3598 ; 0098-7484 ; 0254-9077 ; 0002-9955
ISSN (online)	1538-3598
ISSN	0098-7484 ; 0254-9077 ; 0002-9955
DOI	10.1001/jama.289.13.1645
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ua VI Zs.88: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

To top

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito