617 results match your criteria Hemoglobin C Disease


Point-of-care screening for sickle cell disease in low-resource settings: A multi-center evaluation of HemoTypeSC, a novel rapid test.

Am J Hematol 2019 Jan 31;94(1):39-45. Epub 2018 Oct 31.

Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Sickle cell disease (SCD) is a common, life-threatening genetic disorder that is best managed when diagnosed early by newborn screening. However, SCD is most prevalent in low-resource regions of the world where newborn screening is rare and diagnosis at the point-of-care is challenging. In many such regions, the majority of affected children die, undiagnosed, before the age of 5 years. Read More

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http://doi.wiley.com/10.1002/ajh.25305
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http://dx.doi.org/10.1002/ajh.25305DOI Listing
January 2019
10 Reads
3.800 Impact Factor

Sickle Cell Hemoglobin C Disease Patient Undergoing Coronary Artery Bypass Grafting with Complete Exchange Blood Transfusion during Cardiopulmonary Bypass.

J Extra Corpor Technol 2018 06;50(2):117-119

Department of Cardiac Surgery, Lexington Medical Center, West Columbia, South Carolina.

Sickle cell disorders are associated with increased risk of sickling and vaso-occlusive complications when undergoing cardiopulmonary bypass (CPB) surgery. Monitoring of certain parameters such as venous and arterial oxygen content, hematocrit, acid base homeostasis, and body temperature are required for a superior outcome. Furthermore, perioperative exchange transfusion has a positive effect on the outcome of surgery and on the survival of patients undergoing heart surgery. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6002650PMC
June 2018
2 Reads

Hemoglobinosis C in Morocco : A report of 111 cas.

Tunis Med 2017 Dec;95(12):229-233

Background: - Hemoglobin C is a hemoglobin variant encountered worldwide. The regionswith high prevalence are West Africa and South-East Asia.The objective of this study is to report cases of hemoglobin C disease brought together during these last twelve years in the Laboratory of Biochemistry and Toxicology of RabatMilitary Hospital Mohammed V (MHIMV). Read More

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December 2017
2 Reads

Proliferative retinopathy and maculopathy are two independent conditions in sickle cell disease: Is there a role of blood rheology?1.

Clin Hemorheol Microcirc 2018 May 25. Epub 2018 May 25.

Université des Antilles, CHU dePointe-à-Pitre, Guadeloupe, Université Sorbonne Paris Cité, Université Paris Diderot, Inserm, INTS, Unité Biologie Intégrée du Globule Rouge UMR_S1134, Paris, France.

Objective: Our study investigated the prevalence of retinopathy and maculopathy in sickle cell patients and tested the association between these two conditions. In addition, we tested whether hematological and hemorheological parameters, as well as genotype, were involved in the development of these two conditions.

Methods: Seventy sickle cell adult patients were recruited: 37 with sickle cell anemia (SCA) and 33 with sickle cell hemoglobin C disease (SCC). Read More

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http://www.medra.org/servlet/aliasResolver?alias=iospress&am
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http://dx.doi.org/10.3233/CH-180412DOI Listing
May 2018
11 Reads

Association between oxidative stress and vascular reactivity in children with sickle cell anaemia and sickle haemoglobin C disease.

Br J Haematol 2017 08 3;178(3):468-475. Epub 2017 May 3.

Laboratory LIBM EA7424, Team "Vascular Biology and Red Blood Cell", University of Lyon 1, Villeurbanne, France.

Oxidative stress and haemolysis-associated nitric oxide (NO) depletion plays a crucial role in the development of vasculopathy in sickle cell anaemia (SS). However it remains unknown whether oxidative stress and haemolysis levels influence vascular function in patients with sickle haemoglobin C disease (SC). Microvascular response to heat (using Laser Doppler flowmetry on finger), oxidative stress biomarkers, NO metabolites, endothelin-1 and haematological parameters were compared between patients with SS and SC. Read More

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http://dx.doi.org/10.1111/bjh.14693DOI Listing
August 2017
17 Reads

Cerebral and muscle microvascular oxygenation in children with sickle cell disease: Influence of hematology, hemorheology and vasomotion.

Blood Cells Mol Dis 2017 06 4;65:23-28. Epub 2017 Apr 4.

Université des Antilles, Inserm, Unité Biologie Intégrée du Globule Rouge, Laboratoire d'Excellence du Globule Rouge GR-Ex, Paris, France; Institut Universitaire de France (IUF), Paris, France; Université Claude Bernard Lyon 1, Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie Vasculaire et du Globule Rouge", Villeurbanne, France. Electronic address:

The present study investigated cerebral and muscle hemoglobin oxygen saturation (tissue oxygen index, TOI) in children with sickle cell anemia (SS), sickle cell hemoglobin C disease (SC) and healthy children (AA). TOI was measured by near-infrared spectroscopy (NIRS) and spectral analysis of the TOI variability was used to assess flowmotion and vasomotion. Arterial oxyhemoglobin saturation (SpO), hemorheological and hematological parameters were also measured in SS and SC children. Read More

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http://dx.doi.org/10.1016/j.bcmd.2017.03.015DOI Listing
June 2017
7 Reads

Micro- and macrovascular function in children with sickle cell anaemia and sickle cell haemoglobin C disease.

Blood Cells Mol Dis 2017 05 4;64:23-29. Epub 2017 Feb 4.

Laboratory ACTES (EA 3596), French West Indies University, Pointe-à-Pitre, Guadeloupe, France. Electronic address:

It is unclear whether vascular function is affected similarly in children with sickle cell anaemia (SS) and children with sickle haemoglobin C (SC) disease. Therefore, we compared micro and macrovascular functions in healthy (AA) children, children with SS and SC disease, and assessed their association with physical activity. Participants (24 SS, 22 SC and 16 AA), were compared in terms of 1) thermal hyperaemic response (finger pad warming to 42°C) measured by Laser Doppler techniques, 2) arterial stiffness determined by pulse wave velocity, 3) daily energy expenditure related to moderate and intense physical activities estimated by questionnaire and 4) fitness level, evaluated by the six-minute walk test. Read More

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http://dx.doi.org/10.1016/j.bcmd.2017.02.001DOI Listing
May 2017
31 Reads

Hemoglobin C trait accentuates erythrocyte dehydration in hereditary xerocytosis.

Pediatr Blood Cancer 2017 Aug 25;64(8). Epub 2017 Jan 25.

Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut.

A 17-year-old male presented with acute hemolysis with stomatocytosis, elevated mean corpuscular hemoglobin concentration (MCHC), and osmotic gradient ektacytometry consistent with marked erythrocyte dehydration. Erythrocytes from both parents also demonstrated evidence of dehydration with elevated MCHC and abnormal ektacytometry, but neither to the degree of the patient. Genetic studies revealed the patient had hereditary xerocytosis (HX) due to a novel PIEZO1 mutation inherited from his mother and hemoglobin C (HbC) trait inherited from his father. Read More

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http://dx.doi.org/10.1002/pbc.26444DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5858911PMC
August 2017
6 Reads

Abnormal expression of inflammatory genes in placentas of women with sickle cell anemia and sickle hemoglobin C disease.

Ann Hematol 2016 Oct 22;95(11):1859-67. Epub 2016 Aug 22.

Center for Molecular Biology and Genetic Engineering (CBMEG), University of Campinas - UNICAMP, Campinas, SP, Brazil.

Sickle cell disease (SCD) is a complex disease that is characterized by the polymerization of deoxyhemoglobin S, altered red blood cell membrane biology, endothelial activation, hemolysis, a procoagulant state, acute and chronic inflammation, and vaso-occlusion. Among the physiological changes that occur during pregnancy, oxygen is consumed by fetal growth, and pregnant women with SCD are more frequently exposed to low oxygen levels. This might lead to red blood cells sickling, and, consequently, to vaso-occlusion. Read More

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http://dx.doi.org/10.1007/s00277-016-2780-1DOI Listing
October 2016
24 Reads

Effect of Age on Blood Rheology in Sickle Cell Anaemia and Sickle Cell Haemoglobin C Disease: A Cross-Sectional Study.

PLoS One 2016 29;11(6):e0158182. Epub 2016 Jun 29.

Laboratoire Interuniversitaire de Biologie de la Motricité, Equipe "Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, Lyon, France.

Objectives: Blood rheology plays a key role in the pathophysiology of sickle cell anaemia (SS) and sickle cell haemoglobin C disease (SC), but its evolution over the lifespan is unknown.

Materials And Methods: Blood viscosity, red blood cell (RBC) deformability and aggregation, foetal haemoglobin (HbF) and haematocrit were measured in 114 healthy individuals (AA), 267 SS (161 children + 106 adults) and 138 SC (74 children + 64 adults) patients.

Results: Our results showed that 1) RBC deformability is at its maximal value during the early years of life in SS and SC populations, mainly because HbF level is also at its peak, 2) during childhood and adulthood, hydroxycarbamide treatment, HbF level and gender modulated RBC deformability in SS patients, independently of age, 3) blood viscosity is higher in older SS and SC patients compared to younger ones and 4) haematocrit decreases as SS patients age. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0158182PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4927160PMC
July 2017
23 Reads

Medullary Microvascular Thrombosis and Injury in Sickle Hemoglobin C Disease.

J Am Soc Nephrol 2016 May 6;27(5):1300-4. Epub 2015 Nov 6.

Departments of Pathology and

Sickle cell nephropathy is a common complication in patients with sickle cell hemoglobinopathies. In these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells, which can cause endothelial cell injury in the kidney that may lead to thrombus formation when severe or manifest by multilayering of the basement membranes (glomerular and/or peritubular capillaries) in milder forms of injury. As the injury progresses, the subsequent ischemia, tubular dysfunction, and glomerular scarring can result in CKD or ESRD. Read More

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http://dx.doi.org/10.1681/ASN.2015040399DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4849830PMC
May 2016
6 Reads

The effects of exchange transfusion for prevention of complications during pregnancy of sickle hemoglobin C disease patients.

Transfusion 2016 Jan 3;56(1):119-24. Epub 2015 Sep 3.

Hematology and Hemotherapy Center-University of Campinas/Hemocentro-UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, University of Campinas, Campinas, São Paulo, Brazil.

Background: Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease.

Materials And Methods: Patients were divided into two groups according to the type of transfusion support received: 10 women received prophylactic erythrocytapheresis or manual exchange transfusion at 28 weeks of gestation, and 14 received transfusions only on demand, due to acute complications, or received no transfusions at all. Read More

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http://dx.doi.org/10.1111/trf.13280DOI Listing
January 2016
10 Reads

Hemoglobin C disease.

Authors:
Barbara J Bain

Am J Hematol 2015 Feb;90(2):174

St Mary's Hospital Campus of Imperial College Faculty of Medicine, St Mary's Hospital, Praed Street, London, N1 1NY, UK.

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http://dx.doi.org/10.1002/ajh.23915DOI Listing
February 2015
2 Reads

Red blood cell nitric oxide synthase activation is increased in patients with sickle cell hemoglobin C disease.

Am J Hematol 2015 Feb;90(2):E30-2

UMR Inserm U1134, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.

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http://dx.doi.org/10.1002/ajh.23894DOI Listing
February 2015
12 Reads

Rheology of red blood cells in patients with HbC disease.

Clin Hemorheol Microcirc 2016 ;61(4):571-7

Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.

Patients with hemoglobin C disease (CC) usually do not develop severe complications in comparison with individuals with sickle cell anemia (SS) or with sickle cell hemoglobin C disease (SC). The present study compared the hematological, biochemical, hemorheological and clinical characteristics of CC patients to those of SS, SC and healthy individuals (AA). Blood viscosity was measured at 225 s(-1) with a cone plate viscometer. Read More

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http://dx.doi.org/10.3233/CH-141906DOI Listing
January 2017
10 Reads

Blood typing profile of a school-aged population of a North Togo township.

Hemoglobin 2014 ;38(5):316-8

Laboratory Hematology the CHU-Sylvanus Olympio , Lomé , Togo .

The aim of this study was the determination of hemoglobin (Hb) variants and ABO blood groups in a school population aged 6 to 9 years in the township of Agbandé-Yaka in North Togo. A cross-sectional study was carried out on 570 children of four primary schools at Agbande-Yaka, between March and July 2010. Hemoglobin characterization was done by alkaline buffer electrophoresis and the blood types ABO-Rhesus (Rh) D by immuno-hematological methods. Read More

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http://dx.doi.org/10.3109/03630269.2014.963234DOI Listing
June 2015
9 Reads

Effects of oxidative stress on red blood cell rheology in sickle cell patients.

Br J Haematol 2014 Aug 23;166(4):601-6. Epub 2014 Apr 23.

Inserm U 1134, Pointe-à-Pitre, Guadeloupe, France; Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France; Laboratory of Excellence GR-Ex, Paris, France.

Sickle cell anaemia (SS) and sickle cell-haemoglobin C disease (SC) patients exhibit severe red blood cell (RBC) rheological alterations involved in the development of several complications. The contribution of oxidative stress in these haemorheological abnormalities is still unknown. We compared RBC reactive oxygen species (ROS) and glutathione (GSH) content, and the haemorheological profile of SS (n = 11), SC (n = 11) and healthy subjects (n = 12) at baseline and after in-vitro treatment with t-butyl hydroperoxide (TBHP). Read More

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http://dx.doi.org/10.1111/bjh.12912DOI Listing
August 2014
7 Reads

Rapid screening for sickle cell disease by polymerase chain reaction-high resolution melting analysis.

Mol Med Rep 2014 Jun 9;9(6):2479-84. Epub 2014 Apr 9.

Medical College of Shantou University, Shantou, Guangdong 515041, P.R. China.

Each year, ~300,000 individuals with sickle cell disease (SCD), a hemoglobinopathy caused by β-globin gene mutation, are born, and >75% of those are in Africa. The present study examined 511 individuals on the island of Bioko (Equatorial Guinea) and attempted to establish a method for rapid sickle cell disease screening. Following DNA extraction and polymerase chain reaction (PCR) amplification, high resolution melting (HRM) analysis was used to assess the specificity of fluorescence signals of the PCR products and to differentiate various genotypes of these products. Read More

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http://dx.doi.org/10.3892/mmr.2014.2130DOI Listing
June 2014
5 Reads

The impact of sickle cell disease on oral health-related quality of life.

Pediatr Dent 2014 Jan-Feb;36(1):24-8

Associate professor, Division of Pediatric Dentistry, The Ohio State University, Nationwide Children's Hospital Columbus, Ohio, USA.

Purpose: The purpose of this study was to characterize the impact of sickle cell disease (SCD) on oral health and examine its impact on quality of life.

Methods: Fifty-four study subjects were recruited from the sickle cell clinic and 52 control subjects from the adolescent medicine clinic at Nationwide Children's Hospital, Columbus, Ohio. A dental exam was performed to determine each participant's caries burden. Read More

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January 2015
3 Reads

Brain Perfusion Impairment in Neurologically Asymptomatic Adult Patients with Sickle-Cell Disease Shown by Voxel-Based Analysis of SPECT Images.

Front Neurol 2013 20;4:207. Epub 2013 Dec 20.

Hematology and Hemotherapy Center, University of Campinas , Campinas , Brazil.

Cerebrovascular lesions are frequently observed in patients with sickle-cell disease (SCD) and these structural lesions are preceded by insidious perfusion deficits. Our aim was to investigate the presence of brain perfusion deficits in neurologically asymptomatic SCD patients, especially affecting microvessels. For this study, 42 SCD patients [33 sickle-cell anemia (HbSS), 6 sickle hemoglobin C disease (HbSC), and 3 sickle β-thalassemia disease (HbSβ)] with mean hematocrit of 25. Read More

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http://dx.doi.org/10.3389/fneur.2013.00207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3869238PMC
January 2014
10 Reads

HbC disorders.

Blood 2013 Nov;122(22):3698

Department of Medicine, Boston University School of Medicine, Center of Excellence for Sickle Cell Disease, Boston Medical Center, Boston, MA.

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http://dx.doi.org/10.1182/blood-2013-09-526764DOI Listing
November 2013
3 Reads

Homozygous hemoglobin C disease.

Blood 2013 Sep;122(10):1694

H. Lee Moffitt Cancer and Research Institute, USA.

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September 2013
4 Reads

Autoimmune haemolysis in unexpectedly mild sickle cell/hemoglobin C disease.

Am J Hematol 2013 Sep 23;88(9):817. Epub 2013 Jul 23.

Department of Haematology, Imperial College Healthcare NHS Trust, Hammersmith Hospital, Du Cane Road, London, UK.

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http://dx.doi.org/10.1002/ajh.23512DOI Listing
September 2013
4 Reads

Human bulbar conjunctival hemodynamics in hemoglobin SS and SC disease.

Am J Hematol 2013 Aug 12;88(8):661-4. Epub 2013 Jun 12.

Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL 60612, USA.

The known biophysical variations of hemoglobin (Hb) S and Hb C may result in hemodynamic differences between subjects with SS and SC disease. The purpose of this study was to measure and compare conjunctival hemodynamics between subjects with Hb SS and SC hemoglobinopathies. Image sequences of the conjunctival microcirculation were acquired in 9 healthy control subjects (Hb AA), 24 subjects with SC disease, and 18 subjects with SS disease, using a prototype imaging system. Read More

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http://dx.doi.org/10.1002/ajh.23475DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040222PMC
August 2013
2 Reads

Indirect viscosimetric method is less accurate than ektacytometry for the measurement of red blood cell deformability.

Clin Hemorheol Microcirc 2015 ;59(2):115-21

UMR Inserm 665, Pointe-à-Pitre, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France Laboratoire ACTES (EA 3596), Département de Physiologie, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, Paris, France.

The aim of this study was to test the accuracy of viscosimetric method to estimate the red blood cell (RBC) deformability properties. Thirty-three subjects were enrolled in this study: 6 healthy subjects (AA), 11 patients with sickle cell-hemoglobin C disease (SC) and 16 patients with sickle cell anemia (SS). Two methods were used to assess RBC deformability: 1) indirect viscosimetric method and 2) ektacytometry. Read More

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http://dx.doi.org/10.3233/CH-131727DOI Listing
November 2015
22 Reads

The distribution of haemoglobin C and its prevalence in newborns in Africa.

Sci Rep 2013 ;3:1671

Spatial Ecology and Epidemiology Group, Tinbergen Building, Department of Zoology, University of Oxford, South Parks Road, Oxford OX1 3PS, United Kingdom.

Haemoglobin C (HbC) is one of the commonest structural haemoglobin variants in human populations. Although HbC causes mild clinical complications, its diagnosis and genetic counselling are important to prevent inheritance with other haemoglobinopathies. Little is known about its contemporary distribution and the number of newborns affected. Read More

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http://dx.doi.org/10.1038/srep01671DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3628164PMC
October 2013
5 Reads

Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease.

Haematologica 2013 Jul 28;98(7):1039-44. Epub 2013 Mar 28.

UMR Inserm 665, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France.

The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the six-minute walk test. Read More

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http://dx.doi.org/10.3324/haematol.2013.083576DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3696606PMC
July 2013
6 Reads

Relationships between systemic vascular resistance, blood rheology and nitric oxide in children with sickle cell anemia or sickle cell-hemoglobin C disease.

Clin Hemorheol Microcirc 2014 ;58(2):307-16

Inserm U665, Pointe-à-Pitre, F-97159 Guadeloupe, Université des Antilles et de la Guyane, Guadeloupe.

Vascular function has been found to be impaired in patients with sickle cell disease (SCD). The present study investigated the determinants of systemic vascular resistance in two main SCD syndromes in children: sickle cell anemia (SCA) and sickle cell-hemoglobin C disease (SCC). Nitric oxide metabolites (NOx), hematological, hemorheological, and hemodynamical parameters were investigated in 61 children with SCA and 49 children with SCC. Read More

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http://dx.doi.org/10.3233/CH-121661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4930491PMC
August 2015
7 Reads

Normal muscle oxygen consumption and fatigability in sickle cell patients despite reduced microvascular oxygenation and hemorheological abnormalities.

PLoS One 2012 20;7(12):e52471. Epub 2012 Dec 20.

Inserm 665, Université des Antilles et de la Guyane, Pointe-à-Pitre, Guadeloupe, France.

Background/aim: Although it has been hypothesized that muscle metabolism and fatigability could be impaired in sickle cell patients, no study has addressed this issue.

Methods: We compared muscle metabolism and function (muscle microvascular oxygenation, microvascular blood flow, muscle oxygen consumption and muscle microvascular oxygenation variability, which reflects vasomotion activity, maximal muscle force and local muscle fatigability) and the hemorheological profile at rest between 16 healthy subjects (AA), 20 sickle cell-hemoglobin C disease (SC) patients and 16 sickle cell anemia (SS) patients.

Results: Muscle microvascular oxygenation was reduced in SS patients compared to the SC and AA groups and this reduction was not related to hemorhelogical abnormalities. Read More

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https://hal.archives-ouvertes.fr/docs/00/84/90/35/PDF/2013_W
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http://dx.plos.org/10.1371/journal.pone.0052471
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0052471PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3527490PMC
June 2013
8 Reads

Peripapillary retinal nerve fiber layer thickness in sickle-cell hemoglobinopathies using spectral-domain optical coherence tomography.

Am J Ophthalmol 2013 Mar 4;155(3):456-464.e2. Epub 2012 Dec 4.

Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL 60612, USA.

Purpose: To determine whether patients with a sickle-cell hemoglobinopathy without glaucoma have peripapillary retinal nerve fiber layer (RNFL) thinning by spectral-domain optical coherence tomography.

Design: Prospective study.

Methods: All patients with a sickle cell hemoglobinopathy (sickle-cell disease, sickle-cell hemoglobin C disease, and sickle-cell thalassemia) and age-similar, race-matched controls underwent a comprehensive eye examination and spectral-domain optical coherence tomography of the macula and optic nerve head using the Heidelberg Spectralis (Heidelberg Engineering, Inc, Carlsbad, California, USA). Read More

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http://dx.doi.org/10.1016/j.ajo.2012.09.015DOI Listing
March 2013
12 Reads

Prophylactic penicillin after 5 years of age in patients with sickle cell disease: a survey of sickle cell disease experts.

Pediatr Blood Cancer 2013 Jun 28;60(6):935-9. Epub 2012 Nov 28.

Division of Hematology-Oncology, University of Texas Southwestern Medical Center, Dallas, TX 75390-9063, USA.

Background: Since the publication of the Prophlyactic Penicillin Study II in 1995, the management of penicillin prophylaxis for children with sickle cell disease (SCD) after 5 years of age has been controversial. In this study, we sought to describe current practice patterns of pediatric hematologists related to cessation of penicillin prophylaxis for children with SCD after 5 years of age.

Procedure: We performed a cross-sectional, electronic survey of pediatric hematologists with expertise in SCD to examine practices regarding penicillin prophylaxis in children with SCD after 5 years of age. Read More

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http://dx.doi.org/10.1002/pbc.24395DOI Listing
June 2013
5 Reads

Severe proliferative retinopathy is associated with blood hyperviscosity in sickle cell hemoglobin-C disease but not in sickle cell anemia.

Clin Hemorheol Microcirc 2013 Jan;55(2):205-12

Service d'ophtalmologie, CHU de Pointe á Pitre/Abymes, route de Chauvel, Pointe á Pitre, Guadeloupe, France.

Little is known about the impact of blood rheology on the occurrence of retinopathy in sickle cell disease (SCD). Fifty-nine adult SCD patients in steady-state condition participated to the study: 32 with homozygous SCD (sickle cell anemia; SCA) and 27 with sickle cell hemoglobin-C disease (SCC). The patients underwent retinal examination and were categorized according to the classification of Goldberg: 1) no retinopathy (group 1), 2) non-proliferative or proliferative stage I-II retinopathy (group 2) and 3) proliferative stage III-IV-V retinopathy (group 3). Read More

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http://dx.doi.org/10.3233/CH-2012-1622DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5225266PMC
January 2013
8 Reads

Hemorheological alterations, decreased cerebral microvascular oxygenation and cerebral vasomotion compensation in sickle cell patients.

Am J Hematol 2012 Dec 22;87(12):1070-3. Epub 2012 Aug 22.

UMR Inserm 665, Pointe-à-Pitre, F-97159 Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, F-97157, France.

Sickle cell anemia (SS) is characterized by a reduced cerebral microvascular oxygen saturation (cerebral TOI), which is not associated with hemoglobin concentration. Cerebral TOI has never been studied in sickle cell-hemoglobin C disease (SC). We focused on the relationships between hemorheological alterations and cerebral TOI in sickle cell patients with no cerebral vasculopathy and on the usefulness of TOI variability to assess the cerebral vasomotion activity. Read More

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http://dx.doi.org/10.1002/ajh.23318DOI Listing
December 2012
7 Reads

Elevated plasma levels and platelet-associated expression of the pro-thrombotic and pro-inflammatory protein, TNFSF14 (LIGHT), in sickle cell disease.

Br J Haematol 2012 Sep 6;158(6):788-97. Epub 2012 Jul 6.

INCT de Sangue, Haematology and Haemotherapy Centre, School of Medicine, University of Campinas -UNICAMP, Campinas, SP, Brazil.

Chronic vascular inflammation and endothelial activation may initiate vaso-occlusion in sickle cell disease (SCD). TNFSF14 (CD258; LIGHT), a recently-identified pro-thrombotic and pro-inflammatory tumour necrosis factor (TNF)-superfamily cytokine, has a potent activating effect on endothelial cells. We evaluated whether TNFSF14 production is altered in SCD and whether platelets contribute to this production. Read More

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http://dx.doi.org/10.1111/j.1365-2141.2012.09218.xDOI Listing
September 2012
12 Reads

Prevalence of transcranial Doppler abnormalities in Nigerian children with sickle cell disease.

Am J Hematol 2012 May 28;87(5):544-7. Epub 2012 Mar 28.

Department of Paediatrics, College of Medicine, University of Ibadan, Ibadan, Nigeria.

Transcranial Doppler (TCD) ultrasonography helps to identify children with sickle cell disease (SCD) who are at an increased risk of stroke,making primary stroke prevention a reality. A cross-sectional study of145 Nigerian children aged ≥3 years with SCD was carried out to describe the pattern of cerebral blood flow (CBF) abnormalities. The mean time-averaged mean velocity (TAMV) was 152 ±27. Read More

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http://dx.doi.org/10.1002/ajh.23152DOI Listing
May 2012
7 Reads

Hemoglobins S and C interfere with actin remodeling in Plasmodium falciparum-infected erythrocytes.

Science 2011 Dec 10;334(6060):1283-6. Epub 2011 Nov 10.

Department of Infectious Diseases, Parasitology, Heidelberg University, 69120 Heidelberg, Germany.

The hemoglobins S and C protect carriers from severe Plasmodium falciparum malaria. Here, we found that these hemoglobinopathies affected the trafficking system that directs parasite-encoded proteins to the surface of infected erythrocytes. Cryoelectron tomography revealed that the parasite generated a host-derived actin cytoskeleton within the cytoplasm of wild-type red blood cells that connected the Maurer's clefts with the host cell membrane and to which transport vesicles were attached. Read More

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http://dx.doi.org/10.1126/science.1213775DOI Listing
December 2011
3 Reads
72 Citations
31.480 Impact Factor

Transfusion Med Illustrated: hemoglobin C acquired through exchange transfusion.

Transfusion 2012 Mar 27;52(3):466. Epub 2011 Oct 27.

Hematopathology and Transfusion Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada.

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http://dx.doi.org/10.1111/j.1537-2995.2011.03405.xDOI Listing
March 2012
3 Reads

Clinical and radiologic findings of inner ear involvement in sickle cell disease.

AJNR Am J Neuroradiol 2011 Dec 29;32(11):2160-4. Epub 2011 Sep 29.

Department of Radiology, Boston University School of Medicine, Boston, MA, USA.

Background And Purpose: SCD has been reported to involve the inner ear and result in LH and LO. Our purpose was to examine the prevalence of inner ear involvement and to assess the relationship between clinical and imaging findings in patients with SCD.

Materials And Methods: Review of our institution's imaging data base for patients with SCD who underwent imaging of the brain or head and neck region or both by CT, MR, or both from 2004 to 2008 was performed. Read More

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http://dx.doi.org/10.3174/ajnr.A2720DOI Listing
December 2011
2 Reads

Variant hemoglobin phenotypes may account for differential erythropoiesis-stimulating agent dosing in African-American hemodialysis patients.

Kidney Int 2011 Nov 17;80(9):992-999. Epub 2011 Aug 17.

Division of Nephrology and Hypertension, Department of Medicine, UNC Kidney Center, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

African-American patients with end-stage renal disease have historically lower hemoglobin concentrations and higher requirements of erythropoiesis-stimulating agent (ESA). While disparities in health-care access may partially explain these findings, the role of variant hemoglobin, such as sickle trait, has not been investigated. To clarify this, we evaluated 154 African-American patients receiving in-center hemodialysis with available hemoglobin phenotyping. Read More

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http://dx.doi.org/10.1038/ki.2011.247DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3311876PMC
November 2011
4 Reads

Paravertebral mass in a patient with hemoglobin C disease.

Joint Bone Spine 2011 Oct 23;78(5):534-5. Epub 2011 May 23.

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http://dx.doi.org/10.1016/j.jbspin.2011.04.003DOI Listing
October 2011
3 Reads

Pregnancy outcomes in sickle cell disease: a retrospective cohort study from two tertiary centres in the UK.

Obstet Med 2010 Sep 17;3(3):110-2. Epub 2010 Sep 17.

Women's Health Department, Guy's and St Thomas' Hospital NHS Foundation Trust.

The objective of this retrospective cohort study from two tertiary centres in the UK was to describe the pregnancy outcomes of women with sickle cell disease (SCD) who booked at these centres between 2004 and 2008, and to compare this with historical data. The study population comprised 122 singleton pregnancies in women with SCD: homozygous sickle cell disease 64, sickle cell haemoglobin C disease 45, sickle b plus thalassaemia 11, sickle cell haemoglobin E disease 1 and sickle cell delta disease 1 from 2004 to 2008 managed in the joint haematology/obstetric antenatal clinics in two tertiary teaching hospitals. The main outcome measures were the frequency of sickle cell crises and obstetric complications. Read More

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http://dx.doi.org/10.1258/om.2010.100026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4989596PMC
September 2010
5 Reads

Forecasting hemoglobinopathy burden through neonatal screening in Omani neonates.

Hemoglobin 2010 Jan;34(2):135-44

Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.

To evaluate the incidence of hemoglobinopathies in Omani subjects and to forecast its future burden on health resources, we initiated a prospective neonatal screening program in two major cities of the Sultanate of Oman. Consecutive cord blood samples from a total of 7,837 neonates were analyzed for complete blood counts and for hemoglobin (Hb) profile by high performance liquid chromatography (HPLC). No case with Hb H (beta4) was detected. Read More

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http://dx.doi.org/10.3109/03630261003677213DOI Listing
January 2010
5 Reads
14 Citations
0.960 Impact Factor

Differing effects of HbS and HbC traits on uncomplicated falciparum malaria, anemia, and child growth.

Blood 2010 Jun 15;115(22):4551-8. Epub 2010 Mar 15.

Infectious Disease Epidemiology, Bernhard Nocht Institute for Tropical Medicine, Hamburg, Germany.

The high prevalence of hemoglobin S (HbS) in Africa and hemoglobin C (HbC) in parts of West Africa is caused by the strong protection against severe falciparum malaria during childhood. Much less is known about the effect of HbS and especially HbC on Plasmodium falciparum infection, uncomplicated malaria, and anemia. A total of 1070 children from the Ashanti Region, Ghana, were enrolled at the age of 3 months and visited monthly until 2 years of age. Read More

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http://dx.doi.org/10.1182/blood-2009-09-241844DOI Listing
June 2010
6 Reads

Rheumatoid arthritis in patient with homozygous haemoglobin C disease.

Rheumatol Int 2011 Jun 25;31(6):801-3. Epub 2009 Oct 25.

Department of Rheumatology, El Ayachi University Hospital, Sale, Morocco.

To date, few cases of hemoglobinopathies in patients with rheumatoid arthritis (RA) have been reported (Marino and Mcdonald in J Rheumatol 17:970-972, 1990; Gladman and Bombardier in Arthritis Rheum 30:1065-1068, 1987; Michel et al. in Semin Arthritis Rheum 38:228-240, 2008). These haemoglobin diseases are associated with characteristics abnormalities of the skeleton. Read More

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http://dx.doi.org/10.1007/s00296-009-1230-yDOI Listing
June 2011
3 Reads