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Sickle Cell Crisis

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https://www.readbyqxmd.com/read/29152307/coagulation-profile-of-sudanese-children-with-homozygous-sickle-cell-disease-and-the-effect-of-treatment-with-omega-3-fatty-acid-on-the-coagulation-parameters
#1
Shiekh Awoda, Ahmed A Daak, Nazik Elmalaika Husain, Kebreab Ghebremeskel, Mustafa I Elbashir
Background: It has been reported that patients with SCD do have an abnormal coagulation profile. Coagulopathy is thought to be one of the key factors that contribute to the vaso-occlusive crisis that characterises sickle cell disease (SCD). In this study, we investigated whether Sudanese sickle cell patients have an abnormal coagulation profile. In addition, the effect of treatment with either omega-3 fatty acids or hydroxyurea on coagulation profile was assessed. Methods: Homozygous SCD patients untreated (n = 52), omega-3 treated (n = 44), hydroxyurea (HU) treated (n = 8) and healthy (HbAA) controls (n = 52) matched for age (4-20 years), gender and socioeconomic status were enrolled...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29134776/acute-chest-syndrome-among-children-hospitalized-with-vaso-occlusive-crisis-a-nationwide-study-in-the-united-states
#2
Takuto Takahashi, Yusuke Okubo, Atsuhiko Handa
PURPOSE: Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication. METHODS: Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database...
November 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#3
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29099347/the-morbidity-pattern-of-children-with-sickle-cell-disorders-admitted-to-the-queen-elizabeth-hospital-barbados-2009-2013
#4
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/29078924/sickle-cell-disease-in-the-emergency-department
#5
REVIEW
Paris B Lovett, Harsh P Sule, Bernard L Lopez
Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. EPs should be aware of cognitive biases that may misdirect the diagnostic process. Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29076161/use-of-the-blood-substitute-hboc-201-in-critically-ill-patients-during-sickle-crisis-a-three-case-series
#6
Jonathan M Davis, Nura El-Haj, Nimish N Shah, Garry Schwartz, Margaret Block, James Wall, Mark Tidswell, Ernest DiNino
BACKGROUND: Red blood cell (RBC) transfusion is an important treatment modality during severe sickle cell crisis (SCC). SCC patients who refuse, or cannot accept, RBCs present a unique challenge. Acellular hemoglobin (Hb)-based oxygen carriers (HBOCs) might be an alternative for critically ill patients in SCC with multiorgan failure due to life-threatening anemia. HBOC-201 (HbO2 Therapeutics) has been administered to more than 800 anemic patients in 22 clinical trials, but use of any HBOCs in critically ill sickle cell patients with organ failure is exceedingly rare...
October 26, 2017: Transfusion
https://www.readbyqxmd.com/read/29051184/novel-use-of-hydroxyurea-in-an-african-region-with-malaria-noharm-a-trial-for-children-with-sickle-cell-anemia
#7
Robert O Opoka, Christopher M Ndugwa, Teresa S Latham, Adam Lane, Heather A Hume, Phillip Kasirye, James S Hodges, Russell E Ware, Chandy C John
Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29039292/an-innovative-short-stay-health-care-model-for-treatment-of-uncomplicated-vaso-occlusive-crisis-in-adult-sickle-cell-disease-patients-in-canada-to-reduce-emergency-department-utilization
#8
Andrew Binding, Richard Ward, Chai Phua, Veronique Naessens, Tara O'Brien, Sacha Bhatia, Hayley Baranek, Husayn Marani, Geetha Mukerji
OBJECTIVES: Patients with sickle cell disease (SCD) with vaso-occlusive crises (VOC) often visit the emergency department (ED) for management of painful episodes. The primary objective of this pilot study was to evaluate the acceptability of a short-stay model for treatment of VOC in SCD outside of the ED in Toronto, Canada. Secondary objectives were to assess patient satisfaction of this model, barriers to its use and comparison of clinical outcomes to a historical control. METHODS: Adult SCD patients with symptoms of an uncomplicated VOC between October 2014 to July 2016 were managed according to best practice recommendations in a short-stay unit as an alternative to the local emergency room...
October 17, 2017: CJEM
https://www.readbyqxmd.com/read/29036363/predictive-ability-of-intermittent-daily-sickle-cell-pain-assessment-the-pisces-project
#9
Wally R Smith, Donna K McClish, James Levenson, Imoigele Aisiku, Bassam Dahman, Viktor E Bovbjerg, Susan Roseff, John Roberts
Background: Pain diary assessment in sickle cell disease (SCD) may be expensive and impose a high respondent burden. Objective: To report whether intermittent assessment could substitute for continuous daily pain assessment in SCD. Design: Prospective cohort study. Setting: Academic and community practices in Virginia. Patients: A total of 125 SCD patients age 16 years or older in the Pain in Sickle Cell Epidemiology Study...
September 26, 2017: Pain Medicine: the Official Journal of the American Academy of Pain Medicine
https://www.readbyqxmd.com/read/29033725/assessment-of-patient-controlled-analgesia-versus-intermittent-opioid-therapy-to-manage-sickle-cell-disease-vaso-occlusive-crisis-in-adult-patients
#10
Alaa Al-Anazi, Lowloa Al-Swaidan, Maha Al-Ammari, Tariq Al-Debasi, Abdulmalik M Alkatheri, Shmeylan Al-Harbi, Aiman A Obaidat, Abdulkareem M Al-Bekairy
BACKGROUND: Vaso-occlusive crisis (VOC) is one of the acute complications of sickle-cell disease (SCD). Treatment mainly relies on hydration and pain control by analgesics. The specific aim of this study was to assess potential health outcomes within the first 72 h of admission between intermittent and patient-controlled analgesia (PCA) by opioids among VOC patients. METHODS: A retrospective chart review study was conducted to determine SCD patients with VOC. Using the hospital electronic system, the following data were collected: patient's age, gender, blood pressure, heart rate, respiratory rate, oxygen saturation, and pain score on admission and daily for 3 days as well as the cumulative opioid analgesic dose for 72 h which is reported as morphine equivalent...
October 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29026475/human-parvovirus-b19-in-children-with-sickle-cell-disease-poking-the-spleen
#11
Ashraf Abdellah Saad, Ismail Beshlawi, Abdul Hakim Al-Rawas, Mathew Zachariah, Hanan Fawzy Nazir, Yasser Wali
Parvovirus is a known culprit of transient red cell aplasia (TRCA) in children with sickle cell disease (SCD). Few reports have previously described the association between the virus and acute splenic sequestration crisis (ASSC) in the same patient. Here, we are shedding light on such a potentially serious combination by reporting two cases of siblings with SCD complicated with concurrent ASSC and TRCA and presenting a review of the relevant literature.
September 2017: Oman Medical Journal
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
#12
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/28953196/transcranial-doppler-screening-in-50-patients-with-sickle-cell-hemoglobinopathies-in-iran
#13
Susan Zamani, Afshin Borhan Haghighi, Sezaneh Haghpanah, Mehran Karimi, Mohammad R Bordbar
BACKGROUND: As previous studies had discordant results with regard to the correlation of transcranial Doppler (TCD) screening and brain MRI, the aim of this study was to find the correlation between TCD values and silent ischemia in sickle cell disease (SCD) patients. METHOD AND MATERIALS: In this cross-sectional study, 50 patients with proven diagnosis of sickle cell hemoglobinopathies based on their hemoglobin electrophoresis were included. Demographic data, their physical exam, information with regard to crises history, and their laboratory data were recorded...
August 4, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28938324/bilateral-subperiosteal-hematoma-and-orbital-compression-syndrome-in-sickle-cell-disease
#14
Cem Sundu, Erdem Dinç, Ayça Sari, Selma Ünal, Özer Dursun
A 14-year-old boy with sickle cell disease presented with preseptal cellulitis findings as proptosis, eyelid edema, and hyperemia. His best corrected visual acuity in the right eye was 20/20 and 16/20 in the left eye. He had limited ductions in vertical and lateral gazes in both eyes. Bilateral venous tortuosity was observed in posterior segment examination. Orbital bone infarction and subperiosteal hematoma were seen in magnetic resonance imaging. He was diagnosed as having orbital compression syndrome secondary to vaso-occlusive crisis of sickle cell disease and was treated with intravenous ampicilin-sulbactam and methylprednisolone...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28928925/the-fortuitous-repair-of-a-common-bile-duct-injury-following-placement-of-a-percutaneous-transhepatic-cholangiogram-catheter
#15
Branum G Griswold, Jared A White
Common bile duct injuries are associated with a high rate of morbidity and mortality and are discussed frequently in the literature. These injuries may be difficult to diagnose intraoperatively and are often challenging to repair, necessitating referral to hepatobiliary surgery specialists at academic institutions. This case report highlights the management of a completely disrupted common bile duct identified post-operatively using a percutaneous transhepatic cholangiography (PTC) catheter to bridge the gap between the proximal and distal ductal injury prior to operative repair...
September 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28924974/incidence-and-predictive-score-for-delayed-hemolytic-transfusion-reaction-in-adult-patients-with-sickle-cell-disease
#16
David Narbey, Anoosha Habibi, Philippe Chadebech, Armand Mekontso-Dessap, Mehdi Khellaf, Jean-Daniel Lelièvre, Bertrand Godeau, Marc Michel, Frédéric Galactéros, Rachid Djoudi, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. We conducted a prospective single-center observational study over 30 months in adult sickle cell patients. We included 694 transfusion episodes (TEs) in 311 patients, divided into occasional TEs (OTEs: 360) and chronic transfusion program (CTEs: 334)...
September 19, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28868627/hydroxyurea-prescription-availability-and-use-for-children-with-sickle-cell-disease-in-italy-results-of-a-national-multicenter-survey
#17
Raffaella Colombatti, Giovanni Palazzi, Nicoletta Masera, Lucia Dora Notarangelo, Elisa Bonetti, Piera Samperi, Angelica Barone, Silverio Perrotta, Elena Facchini, Maurizio Miano, Giovanni Carlo Del Vecchio, Maria Elena Guerzoni, Paola Corti, Federica Menzato, Simone Cesaro, Maddalena Casale, Paolo Rigano, Gian Luca Forni, Giovanna Russo, Laura Sainati
BACKGROUND: The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population-based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe. POPULATION AND METHODS: The Hydroxyurea in SCD: A Large Nation-wide Cohort Study from Italy was a retrospective cohort study of adult and pediatric patients with SCD attending 32 centers...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28868180/acute-and-chronic-hepatobiliary-manifestations-of-sickle-cell-disease-a-review
#18
REVIEW
Rushikesh Shah, Cesar Taborda, Saurabh Chawla
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis...
August 15, 2017: World Journal of Gastrointestinal Pathophysiology
https://www.readbyqxmd.com/read/28853040/state-of-the-art-management-of-acute-vaso-occlusive-pain-in-sickle-cell-disease
#19
REVIEW
Latika Puri, Kerri A Nottage, Jane S Hankins, Doralina L Anghelescu
Acute vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD). Multiple complex pathophysiological processes can result in pain during a VOC. Despite significant improvements in the understanding and management of SCD, little progress has been made in the management of pain in SCD, although new treatments are being explored. Opioids and non-steroidal anti-inflammatory drugs (NSAIDs) remain the mainstay of treatment of VOC pain, but new classes of drugs are being tested to prevent and treat acute pain...
August 29, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28852282/the-bone-pain-crisis-of-sickle-cell-disease-and-malaria-observations-from-gujarat-india
#20
Jyotish Patel, Bharati Patel, Graham R Serjeant
BACKGROUND: Sickle cell disease is a common problem across central India, but its clinical features may differ from that in African populations. There is a need to define the features of sickle cell disease in India, and the current study addresses some features of the bone pain crisis. OBJECTIVES: The objective of the study was to describe the epidemiology of the bone pain crisis of sickle cell disease in Gujarat and explore the relationship with infection by Plasmodium vivax...
July 2017: Indian Journal of Community Medicine
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