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sickle cell crisis diagnosis

Sri L H Yeruva, Yonette Paul, Patricia Oneal, Mehdi Nouraie
Renal dysfunction in sickle cell disease is not only a chronic comorbidity but also a mortality risk factor. Though renal dysfunction starts early in life in sickle cell patients, the predictors that can identify sickle cell disease patients at risk of developing renal dysfunction is not known. We used the Truven Health MarketScan(®) Medicaid Databases from 2007 to 2012. Incidence of new acute renal failure (ARF) and chronic kidney disease (CKD) was calculated in this cohort. There were 9481 patients with a diagnosis of sickle cell disease accounting for 64,201 hospital admissions, during the study period...
September 18, 2016: Hemoglobin
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Deogratias Munube, Elly Katabira, Grace Ndeezi, Moses Joloba, Samden Lhatoo, Martha Sajatovic, James K Tumwine
BACKGROUND: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. METHODS: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in Kampala, Uganda...
2016: BMC Neurology
Erica Simon, Brit Long, Alex Koyfman
BACKGROUND: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention. OBJECTIVE: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications. DISCUSSION: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease...
October 2016: Journal of Emergency Medicine
William F Wright
Bacillus cereus typically presents as a gastrointestinal infection, but rarely manifests as systemic disease. This report describes a case of B. cereus-related endocarditis that presented as a sickle cell crisis and bacteremia. Initial clinical suspicion was for laboratory contamination of blood cultures. The case herein described is intended to demonstrate an uncommon presentation of B. cereus infection and highlights the value of an aggressive need to further investigate and interpret unexpected blood culture findings in clinical practice, early adequate antimicrobial therapy, prompt diagnosis, and consideration to urgent surgical interventions in such cases...
June 2016: Clinical Medicine & Research
John J Squiers, Anthony G Edwards, Alberto Parra, Sandra L Hofmann
A 70-year-old African American female with a past medical history significant for chronic bilateral shoulder pain and reported sickle cell trait presented with acute-onset bilateral thoracolumbar pain radiating to her left arm. Two days after admission, Hematology was consulted for severely worsening microcytic anemia and thrombocytopenia. Examination of the patient's peripheral blood smear from admission revealed no cell sickling, spherocytes, or schistocytes. Some targeting was noted. A Coombs test was negative...
January 2016: Journal of Investigative Medicine High Impact Case Reports
Carly S Gardner, Tracy A Jaffe
PURPOSE: The purpose of this study was to determine the incidence, specific imaging features, and outcome of gastrointestinal vaso-occlusive ischemia (GVOI) in sickle cell patients undergoing CT for acute abdominal pain. METHODS: This HIPAA-compliant, IRB-approved retrospective study evaluated sickle cell patients with an abdominal pain crisis and acute gastrointestinal abnormalities on CT from 1/2006 to 1/2014. CT findings were divided into those compatible and incompatible with bowel ischemia or clinical diagnosis of GVOI...
March 2016: Abdominal Radiology
Marzieh Nikparvar, Mohammad Reza Evazi, Tasnim Eftekhari, Farzaneh Moosavi
In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive crisis. The patient had manifestations of microangiopathic hemolytic anemia, including laboratory evidence of hemolytic anemia, thrombocytopenia, respiratory distress, fever, jaundice, and abnormal liver function and coagulation tests, accompanied by clot formation on the Eustachian valve of the inferior vena cava in the right atrium and also a long and worm-like thrombus in the right ventricle...
March 2016: Iranian Journal of Medical Sciences
Iwalokun Bamidele Abiodun, Afolabi Oluwadun, Aina Olugbenga Ayoola, Iwalokun Senapon Olusola
Asymptomatic malaria (ASM) has been implicated in the development of hemolytic crisis in infected sickle cell anemia (SCA) patients worldwide. This study surveyed steady state SCA Nigerian patients for ASM to investigate the influence of malaria prevention behaviors and age on parasitaemia and multiplicity of infection (MOI). A total of 78 steady SCA patients aged 5 - 27 years on routine care at three health facilities in Lagos were investigated for ASM by light microscopy and PCR with a multiplicity of infection determined by genotyping block 2 of merozoite surface protein 1 (msp1) gene of Plasmodium falciparum (P...
January 2016: Acta Medica Iranica
C Janssens, L Claeys, P Maes, T Boiy, M Wojciechowski
We present the case of a 17-year-old boy, known with homozygous sickle cell disease, who was admitted because of generalised pain. He developed bilateral periorbital oedema and proptosis, without pain or visual disturbances. In addition to hyperhydration, oxygen and analgesia IV antibiotics were started, to cover a possible osteomyelitis. Patients with sickle cell disease are at risk for vaso-occlusive crises, when the abnormally shaped red blood cells aggregate and block the capillaries. Such a crisis typically presents at a location with high bone marrow activity, as the vertebrae and long bones...
December 2015: Acta Clinica Belgica
Dipti S Upadhye, Dipty L Jain, Yogesh L Trivedi, Anita H Nadkarni, Kanjaksha Ghosh, Roshan B Colah
BACKGROUND: Sickle cell disease (SCD) is a major health burden in India. The objective of the study was to establish a neonatal screening program and to understand the clinical course of children with SCD in central India. METHODS AND FINDINGS: Pregnant mothers were screened for sickle hemoglobin using the solubility test. Babies were screened by high performance liquid chromatography if the mother was positive for sickle hemoglobin. The diagnosis was confirmed by molecular analysis...
2016: PloS One
Aamir Malik, Chandni Merchant, Mana Rao, Rosemary P Fiore
BACKGROUND: Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality. CASE REPORT: We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice...
2015: American Journal of Case Reports
Stalin Ramprakash, Daniel Fishman
Juvenile fibromyalgia in children with sickle cell disease has not been reported in the literature. We report an adolescent patient with sickle cell whose pain symptoms progressed from having recurrent acute sickle cell pain crisis episodes to a chronic pain syndrome over several years. He was eventually diagnosed with juvenile fibromyalgia based on the clinical history and myofascial tender points and his pain symptoms responded better to multidisciplinary strategies for chronic fibromyalgia pain. Chronic pain in sickle cell disease is an area of poor research, and in addition there is inconsistency in the definition of chronic pain in sickle cell disease...
2015: BMJ Case Reports
Danielle M Graff, Erin Owen, Robert Bendon, Salvatore Bertolone, Ashok Raj
An adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggregated sickle cells and positive parvovirus immunostaining. The brain lesion both grossly and microscopically presented a distinct pathology of acellular fat emboli that led to the correct diagnosis of this increasingly recognized association of sickle hemoglobinopathies with fat embolism syndrome (FES)...
2015: Case Reports in Hematology
Onyekachi Henry Ogbonna, Yonette Paul, Hasan Nabhani, Adriana Medina
First described by Sir Nicholas Brodie in 1832, Brodie's abscess is a localized subacute or chronic infection of the bone, typically seen in the metaphases of long bones in children and adolescents. The diagnosis can prove to be enigmatic due to absence of clinical signs and symptoms of systemic disease. We report a very interesting case of Brodie's abscess masquerading as sickle cell vasoocclusive crisis in a 20-year-old female with sickle cell disease and review the literature.
2015: Case Reports in Medicine
Leonard Naymagon, Gopichand Pendurti, Henny H Billett
Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed...
2015: Hemoglobin
Andrew J Gangemi, Peter V Pickens
We report a case of TTP in a sickle cell/β+-thalassemia heterozygote with nonspecific complaints and a evidence of hemolysis, initially attributed to sickle crisis. Included in this case is a discussion of the development of functional hyposplenism, a rarely reported complication, limitation of ADAMTS-13 in diagnosis, and the use of platelet transfusion.
July 2015: Clinical Case Reports
Emily R Blauel, Lily T Grossmann, Madhav Vissa, Scott T Miller
In a patient with sickle cell disease receiving chronic transfusion, exacerbation of anemia with reticulocytopenia must prompt consideration of a delayed hemolytic transfusion reaction with hyperhemolysis, as further transfusion may worsen this condition; definitive diagnosis is sometimes difficult. Anemia evolving during parvovirus B19-induced erythroid hypoplasia (transient aplastic crisis) should be attenuated in chronic transfusion patients due to superior survival of transfused over endogenous red blood cells...
October 2015: Journal of Pediatric Hematology/oncology
Carole Janssens, Lieve Claeys, Philip Maes, Tine Boiy, Marek Wojciechowski
We present the case of a 17-year-old boy, known with homozygous sickle cell disease, who was admitted because of generalised pain. He developed bilateral periorbital oedema and proptosis, without pain or visual disturbances. In addition to hyperhydration, oxygen and analgesia IV antibiotics were started, to cover a possible osteomyelitis. Patients with sickle cell disease are at risk for vaso-occlusive crises, when the abnormally shaped red blood cells aggregate and block the capillaries. Such a crisis typically presents at a location with high bone marrow activity, as the vertebrae and long bones...
July 24, 2015: Acta Clinica Belgica
Carly S Gardner, Tracy A Jaffe
OBJECTIVE: The purposes of this article are to illustrate the CT appearance of gastrointestinal vasoocclusive crisis in patients with sickle cell disease (SCD), highlight potential complications, and review other conditions that may have similar findings. CONCLUSION: The gastrointestinal vasoocclusive crisis in SCD is rare but can result in potentially life-threatening ischemia and death. Knowledge of the spectrum of CT features of the gastrointestinal vasoocclusive crisis is important in making the diagnosis and potentially preventing complications...
May 2015: AJR. American Journal of Roentgenology
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