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sickle cell anaemia

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https://www.readbyqxmd.com/read/29114966/a-cross-sectional-clinic-based-study-exploring-whether-variants-within-the-glutathione-s-transferase-haptoglobin-and-uridine-5-diphosphoglucuronosyl-transferase-1a1-genes-are-associated-with-inter-individual-phenotypic-variation-in-sickle-cell-anaemia-in-jamaica
#1
Sharon Howell, Kwesi Marshall, Marvin Reid, Norma McFarlane-Anderson, Colin McKenzie
OBJECTIVES: To explore putative associations between specific variants in either the glutathione S-transferase (GST), haptoglobin (HP), or uridine 5'-diphospho-glucuronosyltransferase 1A1 (UGT1A1) genes and clinically important phenotypes in sickle cell anaemia (HbSS). METHODS: Three hundred and seventy-one HbSS participants were recruited from the Sickle Cell Clinic of the Sickle Cell Unit at the University of the West Indies, Kingston, Jamaica. Markers within four GST superfamily genes, the HP gene, and the UGT1A1 gene were analyzed using PCR-based assays...
November 7, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29099347/the-morbidity-pattern-of-children-with-sickle-cell-disorders-admitted-to-the-queen-elizabeth-hospital-barbados-2009-2013
#2
D Grannum, Paula M Lashley
A retrospective study was conducted by examining all the medical files of all the children with sickle cell anaemia (Hb SS) admitted from 1 January 2009 to 31 December 2013. A total of 220 admission notes (59 patients aged 5 months-16 years) were analysed. Of these, 53.2% were boys; 85.8% of the patients had Hb SS. The most common reason for admission was vaso-occlusive crisis. Blood transfusions were used in 39.7% of admissions There were no deaths during the study period. We note the number of sickle cell-related admissions and morbidity has increased, along with an increase in the number of blood transfusions...
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/29021902/significantly-elevated-foetal-haemoglobin-levels-in-individuals-with-glucose-6-phosphate-dehydrogenase-disease-and-or-sickle-cell-trait-a-cross-sectional-study-in-cape-coast-ghana
#3
Patrick Adu, Essel K M Bashirudeen, Florence Haruna, Edward Morkporkpor Adela, Richard K D Ephraim
BACKGROUND: Previously published data have demonstrated that sickle red blood cells produce twice as much reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and haemoglobin concentration in adults in steady state...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29019038/utilisation-and-safety-of-deferasirox-results-from-an-observational-cohort-study-in-england
#4
Vicki Osborne, Miranda Davies, Deborah Layton, Saad A W Shakir
INTRODUCTION: Deferasirox (EXJADE(®), Novartis, UK) is an oral iron-chelating agent primarily used to reduce chronic iron overload in patients receiving blood transfusions for various chronic anaemias and some non-transfusion dependant anaemias. OBJECTIVE: The aim of this study was to examine the utilisation and safety of deferasirox used in general practice in England. METHOD: A single exposure observational cohort study design was used...
October 10, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
https://www.readbyqxmd.com/read/28988427/hydroxyurea-therapy-in-uk-children-with-sickle-cell-anaemia-a-single-centre-experience
#5
Kate Phillips, Laura Healy, Louise Smith, Russell Keenan
INTRODUCTION: Despite the demonstrated efficacy of hydroxyurea therapy, children with sickle cell anaemia in the UK are preferentially managed with supportive care or transfusion. Hydroxyurea is reserved for children with severe disease phenotype. This is in contrast to North America and other countries where hydroxyurea is widely used for children of all clinical phenotypes. The conservative UK practice may in part be due to concerns about toxicity, in particular marrow suppression with high doses, and growth in children...
October 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28969108/evaluation-of-serum-zinc-and-antioxidant-vitamins-in-adolescent-homozygous-sickle-cell-patients-in-wardha-district-of-central-india
#6
Rina Raibhan Wasnik, Nilkanth Ramji Akarte
INTRODUCTION: Sickle cell anaemia is a condition characterized by haemolytic and vaso-occlusive crisis. Previous studies in different part of the world have reported deficiency of zinc, vitamin C and E but the role of their supplementation in sickle cell disease remains question. Nutritional factors may contribute to clinical manifestation in rural population of developing countries specially in adolescent age group. Thus, the present study was designed in rural population of Wardha district of Maharashtra in adolescent sickle cell homozygous patients in view to evaluate serum zinc and antioxidant vitamins C and E...
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28951650/atypical-osteomyelitis-and-concurrent-septic-arthritis-due-to-salmonella-in-immunocompetent-children
#7
Balasubramaniam Balakumar, Sangeet Gangadharan, Nithya Ponmudi, Satish Kumar, John Jude Prakash, Thomas Palocaren
OBJECTIVE: Salmonella osteomyelitis in immunocompromised individuals with sickle cell anaemia is well documented. Its occurrence in immunocompetent children is rare. METHODS: All pus culture positive cases of salmonella typhi between the period 2009 to 2014 were reviewed and only those children without sickle cell disease or trait were considered further. RESULTS: Eighty five patients had positive cultures. Of these only three children had culture positive Salmonella septic arthritis in the absence of sickle cell disease...
July 2017: Journal of Clinical Orthopaedics and Trauma
https://www.readbyqxmd.com/read/28922657/methods-employed-in-cytofluorometric-assessment-of-eryptosis-the-suicidal-erythrocyte-death
#8
REVIEW
Mohamed Jemaà, Myriam Fezai, Rosi Bissinger, Florian Lang
Suicidal erythrocyte death or eryptosis contributes to or even accounts for anemia in a wide variety of clinical conditions, such as iron deficiency, dehydration, hyperphosphatemia, vitamin D excess, chronic kidney disease (CKD), hemolytic-uremic syndrome, diabetes, hepatic failure, malignancy, arteriitis, sepsis, fever, malaria, sickle-cell disease, beta-thalassemia, Hb-C and G6PD-deficiency, Wilsons disease, as well as advanced age. Moreover, eryptosis is triggered by a myriad of xenobiotics and endogenous substances including cytotoxic drugs and uremic toxins...
2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28912951/the-frequency-and-severity-of-epistaxis-in-children-with-sickle-cell-anaemia-in-eastern-uganda-a-case-control-study
#9
Amina Nardo-Marino, Thomas N Williams, Peter Olupot-Olupot
BACKGROUND: There are a paucity of data on epistaxis as it pertains to sickle cell anaemia. Some case studies suggest epistaxis to be a significant complication in patients with sickle cell anaemia in sub-Saharan Africa; however, no robust studies have sought to establish the epidemiology or pathophysiology of this phenomenon. METHODS: We conducted a case-control study with the aim of investigating the importance of epistaxis among children presenting with sickle cell anaemia at the Mbale Regional Referral Hospital in eastern Uganda...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28910308/pulmonary-hypertension-among-5-to-18-year-old-children-with-sickle-cell-anaemia-in-nigeria
#10
Ogochukwu J Sokunbi, Ekanem N Ekure, Edamisan O Temiye, Roosevelt Anyanwu, Christy A N Okoromah
BACKGROUND: Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates. METHODS: In this cross sectional study, evaluation involved obtaining bio-data, history and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA...
2017: PloS One
https://www.readbyqxmd.com/read/28894559/pattern-of-cerebral-blood-flow-velocity-using-transcranial-doppler-ultrasonography-in-children-with-sickle-cell-disorder-in-lagos-state-nigeria
#11
Motunrayo Oluwabukola Adekunle, Adeola Barakat Animasahun, Ijeoma Nnenna Diaku-Akinwumi, Olisamedua Fidelis Njokanma
Cerebrovascular accident (CVA) is a common, devastating neurological complication of sickle cell disorder (SCD) with a high recurrent and mortality rate. The Stroke Prevention Trial in Sickle Cell Anaemia study (STOP) recommends routine screening with transcranial Doppler ultrasonography in children aged two to sixteen years with SCD. The present study assessed cerebral blood flow velocities of children with SCD in accordance with the recommendation of routine screening by the STOP study. METHODS: Transcranial Doppler ultrasonography was done for children with SCD that attended Sickle Cell Foundation, Nigeria between July and November 2015...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28881103/hla-polymorphisms-and-risk-of-red-blood-cell-alloimmunisation-in-polytransfused-patients-with-sickle-cell-anaemia
#12
C Rodrigues, A M Sell, G A S Guelsin, T T Higa, S Pagliarini E Silva, L C Macedo, E Â Sippert, J B de Alencar, Â Zanette, C R L Acorsi, L Castilho, J E L Visentainer
BACKGROUND: Red blood cell (RBC) alloimmunisation is an event that may occur due to factors such as numerous blood transfusions, age, gender and genetic factors such as human leukocyte antigen (HLA). AIMS/OBJECTIVES: The aim of the present study was to investigate the possibility of alloimmunisation to red blood cell group antigens associated with the HLA of individuals and to relate alloimmunisation to risk factors. METHODS: A total of 172 polytransfused patients with sickle cell anaemia (SCA) (44 alloimmunised, 128 non-alloimmunised) participated in this study...
September 7, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28880374/skeletal-and-myocardial-microvascular-blood-flow-in-hydroxycarbamide-treated-patients-with-sickle-cell-disease
#13
Vandana Sachdev, Stanislav Sidenko, Melinda D Wu, Caterina P Minniti, Hwaida Hannoush, Cynthia L Brenneman, Myron A Waclawiw, Andrew E Arai, Alan N Schechter, Gregory J Kato, Jonathan R Lindner
In sickle cell disease (SCD), abnormal microvascular function combined with chronic anaemia predisposes patients to perfusion-demand mismatch. We hypothesized that skeletal muscle and myocardial perfusion, normalized to the degree of anaemia, is reduced at basal-state compared to controls, and that this defect is ameliorated by hydroxycarbamide (HC; also termed hydroxyurea) therapy. Twenty-one SCD patients, of whom 15 were treated with HC, and 27 controls underwent contrast-enhanced ultrasound (CEU) perfusion imaging of the forearm as well as the myocardium...
November 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28833998/association-of-sickle-cell-haemoglobinopathies-with-dental-and-jaw-bone-abnormalities
#14
Sfc Souza, Hlcc de Carvalho, Cps Costa, Ebaf Thomaz
OBJECTIVE: To estimate the association between sickle cell anaemia and trait with dental and jaw bone abnormalities. SUBJECTS AND METHODS: Subjects (n = 369) were allocated to three groups: sickle cell anaemia, trait and control. Dental shape, number, size and position and changes in pulp chamber, root and periapex were analysed by intra-oral periapical radiographs. Integrity of lamina dura, quality of cancellous bone and bone trabeculation were also evaluated...
August 20, 2017: Oral Diseases
https://www.readbyqxmd.com/read/28774421/thalassaemia
#15
REVIEW
Ali T Taher, David J Weatherall, Maria Domenica Cappellini
Inherited haemoglobin disorders, including thalassaemia and sickle-cell disease, are the most common monogenic diseases worldwide. Several clinical forms of α-thalassaemia and β-thalassaemia, including the co-inheritance of β-thalassaemia with haemoglobin E resulting in haemoglobin E/β-thalassaemia, have been described. The disease hallmarks include imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic haemolytic anaemia, compensatory haemopoietic expansion, hypercoagulability, and increased intestinal iron absorption...
July 31, 2017: Lancet
https://www.readbyqxmd.com/read/28771666/how-i-manage-sickle-cell-patients-with-high-transcranial-doppler-results
#16
REVIEW
John Brewin, Banu Kaya, Subarna Chakravorty
Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non-attenders...
November 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28764173/clinical-and-laboratory-predictors-of-frequency-of-painful-crises-among-sickle-cell-anaemia-patients-in-nigeria
#17
Angela Ogechukwu Ugwu, Obike Godswill Ibegbulam, Theresa Ukamaka Nwagha, Anazoeze Jude Madu, Sunday Ocheni, Iheanyi Okpala
INTRODUCTION: The severity of Sickle Cell Anaemia (SCA) in terms of frequency of painful Vaso-Occlusive Crises (VOC) may be affected by clinical and haematological parameters amongst others. Elucidation of these factors in a given disease prevalent environment is necessary for prompt and effective management of patients with frequent painful VOC. AIM: This study aimed at determining the clinical and laboratory predictors of frequency of painful VOC among SCA patients in Enugu, Southeastern Nigeria...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28764172/clinico-haematological-profile-of-hereditary-haemolytic-anaemias-in-a-tertiary-health-care-hospital-in-south-india
#18
Chaitra Venkataswamy, Am Shanthala Devi
INTRODUCTION: Hereditary haemolytic anaemia is a common inherited disorder causing varying degree of morbidity and mortality. This includes disorders due to haemoglobin defect, membrane defect, and enzyme defect. Among them haemoglobinopathies, a single gene disorder, constitutes the major part of the disorder and is distributed worldwide with an incidence of 5%. These inherited disorders pose a major public health problem and increase the burden both on the patient and the society. Presently, these disorders are not curable but can only be prevented...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28731851/the-frequency-of-occurrence-of-fish-shaped-red-blood-cells-in-different-haematologic-disorders
#19
Christoph Robier, Carolin Körber, Franz Quehenberger, Manfred Neubauer, Albert Wölfler
BACKGROUND: Red blood cells (RBC) resembling the silhouette of a fish are rarely observed in peripheral blood (PB) smears. In this study, we determined the frequency of occurrence of fish-shaped RBC in different haematologic diseases. METHODS: We examined PB smears of patients with iron deficiency anaemia (IDA) (n=23), β-thalassaemia minor (BTM) (n=30), sickle cell disease (SCD) (n=7), autoimmune haemolytic anaemia (AIHA) (n=13), microangiopathic haemolytic anaemia (MAHA) (n=11), hereditary sphaerocytosis (HS) (n=4), hereditary elliptocytosis (HE) (n=3), vitamin B12 and folate deficiency (n=15), anaemia in liver disease (LD) (n=17), myelodysplastic syndrome (MDS) (n=15), acute myeloid leukaemia (AML) (n=29), chronic myeloid leukaemia (CML) (n=18), primary myelofibrosis (PMF) (n=12), chronic myelo-monocytic leukaemia (CMML) (n=15) and 21 healthy controls by light microscopy for the occurrence of fish-shaped erythrocytes...
July 21, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28692445/normal-cerebral-vascular-pulsations-in-humans-changes-with-age-and-implications-for-microvascular-disease
#20
Mi O Kim, Yan Li, Fangfei Wei, Jiguang Wang, Michael F O'Rourke, Audrey Adji, Alberto P Avolio
BACKGROUND: Cerebral syndromes in older humans, secondary stroke in younger persons following trauma, and sickle cell anaemia in children, are linked by unexplained microvascular damage and high cerebral pressure or flow pulsations. The aim of this study was to characterize age-related pressure and flow waveforms patterns entering the brain, to explain these in terms of disturbed physiological function, and to consider clinical implications. METHOD: Blood flow velocity waves were measured in four cerebral vascular territories by transcranial Doppler of 1020 apparently normal patients (497 men, 21-78 years)...
November 2017: Journal of Hypertension
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