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https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#1
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28228943/a-case-series-of-cholecystectomy-in-jamaican-sickle-cell-disease-patients-the-need-for-a-new-strategy
#2
Pierre-Anthony Leake, Marvin Reid, Joseph Plummer
: High morbidity rates related to cholecystectomy in sickle cell disease (SCD) patients have been previously reported in the region. This study serves to assess the current outcomes related to cholecystectomy in a Jamaican SCD population. METHODS: A retrospective chart review of SCD patients undergoing elective cholecystectomy at the University Hospital of the West Indies over a 6-year period was performed providing relevant information for analysis. Patients were grouped on an intention-to-treat basis into an open and laparoscopic group...
March 2017: Annals of Medicine and Surgery
https://www.readbyqxmd.com/read/28228042/haemoglobin-f-a2-and-s-levels-in-subjects-with-or-without-sickle-cell-trait-in-south-eastern-gabon
#3
Landry-Erik Mombo, Gaël Mabioko-Mbembo, Roland-Fabrice Kassa-Kassa, Emmanuel Ontsitsagui, Statiana Mboui-Ondo, Leatitia Nzé-Kamsi, Dieudonné Nkoghé, Jacques Elion
BACKGROUND: Infant mortality due to sickle cell disease in sub-Saharan Africa is high, necessitating a better understanding of the modulating factors of the disease in this region. METHODS: We assessed the hereditary persistence of foetal haemoglobin and α-thalassemia. We diagnosed 787 subjects, with or without sickle cell trait, by capillary electrophoresis in the Medical Diagnostic Laboratory of the CIRMF (Franceville, Gabon). RESULTS: Heterocellular and pancellular forms of hereditary persistence of foetal haemoglobin occurred at low rates of 10...
February 23, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28221286/sickle-cell-disease-a-natural-model-of-acute-and-chronic-pain
#4
Amanda M Brandow, Katherine J Zappia, Cheryl L Stucky
No abstract text is available yet for this article.
February 13, 2017: Pain
https://www.readbyqxmd.com/read/28221266/body-mass-index-and-the-association-with-vaso-occlusive-crises-in-pediatric-sickle-cell-disease
#5
Andrea Zivot, Nataly Apollonsky, Edward Gracely, Deepti Raybagkar
BACKGROUND: Children with sickle cell disease (SCD) historically have been underweight and have poor overall growth. Recent studies have demonstrated a trend toward obesity in pediatric SCD populations. MATERIALS AND METHODS: Through retrospective chart review of patients with SCD followed at our center, we collected patient's data, including body mass index (BMI), weight percentiles, sickle cell genotype, baseline hemoglobin, medical and psychiatric comorbidities, 25-hydroxy vitamin D level, treatment with hydroxyurea, and chronic transfusions...
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28221265/acute-liver-failure-during-deferasirox-chelation-a-toxicity-worth-considering
#6
Nathan Menaker, Katharine Halligan, Natasha Shur, John Paige, Matthew Hickling, Anne Nepo, Lauren Weintraub
This case report details a unique case of acute, reversible liver failure in a 12-year-old male with sickle cell anemia on chronic transfusion protocol and deferasirox chelation. There is substantial literature documenting deferasirox-induced renal injury, including Fanconi syndrome, but less documentation of hepatic toxicity and few reports of hepatic failure. The case highlights the importance of close monitoring of ferritin, bilirubin, and transaminases for patients on deferasirox.
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28221202/arginine-therapy-shows-promise-for-treatment-of-sickle-cell-disease-clinical-subphenotypes-of-hemolysis-and-arginine-deficiency
#7
Claudia R Morris
No abstract text is available yet for this article.
February 17, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28220744/burden-genotype-and-phenotype-profiles-of-adult-patients-with-sickle-cell-disease-in-cape-town-south-africa
#8
G D Pule, K Mnica, M Joubert, S Mowla, N Novitsky, A Wonkam
BACKGROUND: An exponential increase in the number of sickle cell disease (SCD) patients in paediatric services in Cape Town, South Africa, has been reported. The trend in adult/adolescent services has not been investigated. OBJECTIVES: To evaluate epidemiological trends of SCD and the profile of patients affected by SCD attending the Haematology Clinic at Groote Schuur Hospital (GSH), Cape Town. METHODS: (i) A retrospective review of the number of SCD patients over the past 20 years; (ii) a cross-sectional analysis of clinical and haematological characteristics of SCD patients; and (iii) molecular analysis of the haemoglobin S mutation, the haplotype in the β-globin-like genes cluster, the 3...
January 30, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28220719/protector-effect-of-%C3%AE-thalassaemia-on-cholecystitis-and-cholecystectomy-in-sickle-cell-disease
#9
Robéria M Pontes, Elaine S Costa, Patrícia F R Siqueira, Jussara F F Medeiros, Andréa Soares, Fabiana V de Mello, Maria C Maioli, Isaac L S Filho, Liliane R Alves, Marcelo G P Land, Marcos K Fleury
OBJECTIVES: Cholecystitis is one of the complications of symptomatic cholelithiasis responsible for high levels of morbidity of sickle cell disease (SCD) patients. Here, we investigated the possible protective role of single gene deletions of α-thalassaemia in the occurrence of cholelithiasis and cholecystitis in SCD patients, as well as the cholecystectomy requirements. METHODS: The α-globin genotype was determined in 83 SCD patients using the multiplex-polymerase chain reaction and compared with clinical events...
February 21, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28220143/venous-thromboembolism-in-children-with-cancer-and-blood-disorders
#10
REVIEW
Richard H Ko, Courtney D Thornburg
Venous thromboembolism (VTE) in children is multifactorial and most often related to a combination of inherited and acquired thrombophilias. Children with cancer and blood disorders are often at risk for VTE due to disease-related factors such as inflammation and abnormal blood flow and treatment-related factors such as central venous catheters and surgery. We will review risk factors for VTE in children with leukemia, lymphoma, and solid tumors. We will also review risk factors for VTE in children with blood disorders with specific focus on sickle cell anemia and hemophilia...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28218952/glomerular-hyperfiltration-is-strongly-correlated-with-age-in-congolese-children-with-sickle-cell-anaemia
#11
Michel Ntetani Aloni, René Makuala Ngiyulu, Pépé Mfutu Ekulu, Fiston IkwaNdol Mbutiwi, Jean Robert Makulo, Jean Lambert Gini-Ehungu, Nazaire Mangani Nseka, François Bompeka Lepira
AIM: Glomerular hyperfiltration is an early marker of sickle cell nephropathy and can lead to microalbuminuria and renal failure. Our aim was to identify the associated risk factors, as these could be of preventative importance. METHODS: We recruited 150 children with sickle cell anaemia (SCA), aged 2-18 years and living in Kinshasa, the Democratic Republic of Congo. Hyperfiltration and microalbuminuria were defined as an estimated glomerular filtration rate of less than 140 ml/min/1...
February 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28214498/vasculitic-and-autoimmune-wounds
#12
REVIEW
Victoria K Shanmugam, Divya Angra, Hamza Rahimi, Sean McNish
OBJECTIVE: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions...
March 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28212878/evaluation-of-macular-vascular-abnormalities-identified-by-optical-coherence-tomography-angiography-in-sickle-cell-disease
#13
Ian C Han, Mongkol Tadarati, Katia D Pacheco, Adrienne W Scott
PURPOSE: To evaluate macular vascular flow abnormalities identified by optical coherence tomography angiography (OCT-A) in patients with various sickle cell genotypes. DESIGN: Prospective, observational case series. METHODS: This is a single institution case series of adult patients with various sickle cell genotypes. All patients underwent macular OCT-A (Avanti RTVue XR, Optovue Inc, Fremont, CA). Images were analyzed qualitatively for areas of flow loss and quantitatively for measures of foveal avascular area, parafoveal flow, and vascular density...
February 14, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28211104/dat-positive-plasmodium-ovale-malaria-presenting-in-a-child-with-sickle-cell-anemia
#14
Nicholas S Whipple, Jason R Schwartz, Kerri A Nottage
No abstract text is available yet for this article.
February 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28210918/nutcracker-syndrome-and-sickle-cell-trait-a-perfect-storm-for-hematuria
#15
Amier Ahmad, Samuel K McElwee, Ryan R Kraemer
We describe the case of a 27-year-old woman with a history of sickle cell trait (SCT) who presented with several months of hematuria and was found to have nutcracker syndrome (NCS). While SCT is a common cause of hematuria resulting from renal papillary necrosis, our patient had concomitant abdominal pain and anemia, prompting further evaluation and the subsequent diagnosis of NCS. Interestingly, the anoxia in the left renal vein from NCS predisposes patients with SCT to sickling. Our case highlights key clinical features of both NCS and SCT and the relationship between the two disease processes...
February 16, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/28210360/management-of-sickle-cell-disease-super-utilizers
#16
EDITORIAL
Gary A Johnson
No abstract text is available yet for this article.
February 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28209642/orbital-infarction-with-haematoma-in-sickle-cell-disease
#17
Matthew R Edmunds, Lucilla Butler
No abstract text is available yet for this article.
February 16, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28209096/increased-oxidative-stress-alters-nucleosides-metabolite-levels-in-sickle-cell-anemia
#18
Lívia Gelain Castilhos, Juliana Sorraila de Oliveira, Stephen Adeniyi Adefegha, Luana Pereira Magni, Pedro Henrique Doleski, Fatima Husein Abdalla, Cínthia Melazzo de Andrade, Daniela Bitencourt Rosa Leal
OBJECTIVES: This study was conducted to assess the markers of oxidative stress, myeloperoxidase (MPO), acetylcholinesterase (AChE) and xanthine oxidase (XO) activities as well as the levels of nucleotide metabolites in sickle cell anemia (SCA) patients. METHODS: Fifteen SCA treated patients and 30 health subjects (control group) were selected. The markers of oxidative stress (levels of reactive oxygen species (ROS), plasma proteins, carbonyl content, lipid peroxidation (TBARS), total thiols (T-SH), glutathione and catalase activity), MPO, AChE and XO activities as well as the levels of nucleotide metabolites were measured in SCA patients...
February 16, 2017: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/28208888/haemoglobinopathies-and-%C3%AE-thalassaemia-among-the-tribals-working-in-the-tea-gardens-of-assam-india
#19
Anju Barhai Teli, Rumi Deori, Sidhartha Protim Saikia
INTRODUCTION: Prevalence of haemoglobinopathies and β-thalassaemia are very high in India but information about its status among the tribals working in the tea gardens of Assam is very less. AIM: The present study was carried out to determine the prevalence of haemoglobinopathies and β-thalassaemia among the tribals working in the tea gardens of Assam. MATERIALS AND METHODS: A total 1204 samples from the tribals working in tea gardens of Assam were analysed for both Complete Blood Count (CBC) and High Pressure Liquid Chromatography (HPLC) for detection of haemoglobinopathies and β-thalassaemia...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207871/correction-inflammation-in-sickle-cell-disease-differential-and-down-expressed-plasma-levels-of-annexin-a1-protein
#20
Lidiane S Torres, Jéssika V Okumura, Danilo G H Silva, Kallyne K O Mimura, Édis Belini-Júnior, Renan G Oliveira, Clarisse L C Lobo, Sonia M Oliani, Claudia R Bonini-Domingos
[This corrects the article DOI: 10.1371/journal.pone.0165833.].
2017: PloS One
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