sickle cell crisis diagnosis

Sickle cell disease is a well-known homozygous inherited hemoglobinopathy that causes vaso-occlusive phenomena and chronic hemolysis. Vaso-occlusion results in sickle cell crisis and can eventually lead to complications involving multiple organ systems. However, the heterozygous counterpart, sickle cell trait (SCT) has less clinical significance as these patients are generally asymptomatic. This case series examines three unrelated patients with SCT ranging from the age of 27 to 61 years, who presented with pain in multiple long bones...

KEY CLINICAL MESSAGE: In patients with sickle cell disease, we must be more cognizant of the possibility of multifocal osteomyelitis. Diagnosis can be challenging in this patient population as the symptoms mimic vaso-occlusive crisis. There is no gold standard in imaging. ABSTRACT: Osteomyelitis occurs more frequently in children with sickle cell disease. Diagnosis is challenging as it mimics vaso-occlusive crises, a common manifestation of sickle cell disease. We present a case of a 22-month-old girl with sickle cell disease and multifocal osteomyelitis...

INTRODUCTION: The aim of this study was to describe the clinical complications, treatment use, healthcare resource utilization (HCRU), and costs among patients with sickle cell disease (SCD) with recurrent vaso-occlusive crises (VOCs) in the US. METHODS: Merative MarketScan Databases were used to identify patients with SCD with recurrent VOCs from March 1, 2010, to March 1, 2019. Inclusion criteria were ≥ 1 inpatient or  ≥ 2 outpatient claims for SCD and ≥ 2 VOCs per year in any 2 consecutive years after the first qualifying SCD diagnosis...

MANAGEMENT OF ACUTE COMPLICATIONS OF SICKLE CELL DISEASE. Acute complications are the most frequent causes of hospitalization and morbidity in patients with sickle cell disease. Vaso-occlusive crisis are responsible of more than 90% of hospitalization, but numerous acute complications can affect multiples organ or function, that may be life-threatening. Thus, a single reason for hospitalization may include many complications such as worsening of an anemia, vascular disease (stroke, thrombosis, priapism), acute chest syndrome, liver or spleen sequestration...

Background Sickle cell disease (SCD) is the most common genetic blood disorder in Saudi Arabia. A limited number of studies have been conducted on SCD patients regarding their intensive care unit (ICU) admissions. We aimed to identify the cause of ICU admission in SCD patients and to identify predictors of mortality. Methodology We identified 64 patients with SCD, aged 14 years and older, who were admitted to the ICU of King Saud Medical City, Riyadh, Kingdom of Saudi Arabia, from January 1, 2017, to December 31, 2020...

Vaso-occlusive pain crisis is a debilitating complication of sickle cell disease (SCD) and it is the most common cause of hospitalization among these individuals. We studied the inpatient outcomes among patients admitted with sickle cell crisis based on the timing of red blood cell transfusion. In this retrospective study, we used the United States National Inpatient Sample (NIS) data for the year 2019, to identify adult patients hospitalized with the principal diagnosis of sickle cell crisis who received simple red blood cell transfusion during their hospitalization...

Sickle Cell Disease (SSD) can present with acute painful crises, most commonly manifesting as diffuse bony pain; however, rare presentations of acute coronary syndrome, acute papillary necrosis, or multi-organ failure may also present in these patients. TTP has been rarely described in conjunction with sickle cell pain crisis (SS crisis). In both TTP and sickle cell crises, widespread platelet activation is present with thrombocytopenia as a result. Thrombocytopenia can be utilized as a poor prognostic indicator in patients with SS crisis...

Pulmonic valve endocarditis is a rare and clinically elusive identity, commonly associated with congenital heart malformations and intravenous (IV) drug abuse. We describe a case of a 40-year-old male who has established sickle cell disease and presented with pain crisis, febrile episodes, and oxygen desaturation on room air. The clinical presentation and echocardiographic findings of a pulmonic mass were consistent with the diagnosis of pulmonic valve endocarditis. Due to the small size of the pulmonic valve vegetation, the patient was treated with antibiotics and discharged home on antibiotics and home oxygen...

A 24-year-old male sickle cell anemia patient presented with acute abdominal pain. Computed tomography (CT) demonstrated signs of bowel ischemia about the terminal ilium. He underwent bowel resection and anastomosis. Pathology of the resected bowel showed acute inflammation at the site of bowel perforation. This was thought to be secondary to bowel infarction from sickle cell vasculopathy. Despite the surgical intervention, the patient's symptoms continued to worsen. He also developed bilateral toe pain during the same hospital stay...

INTRODUCTION: Sickle cell disease (SCD) is the most common genetic disease found in Africa and throughout the world. It is responsible for a high rate of hemolysis, systemic inflammation, and modulation of the immune system with the involvement of immunological molecules, such as cytokines. IL-1β is a major inflammatory cytokine. IL-18 and IL-33, members of IL-1 family, also exhibit characteristics of inflammation-related cytokines. Thus, in order to contribute to the evaluation of the severity and prognosis of SCD in Africa, this study aimed to estimate the cytokine response, in particular the levels of cytokines of the IL-1 family, in sickle cell patients living in a Sub-Saharan country...

Sickle cell beta-thalassemia (S/β) is a rare inherited variant of sickling disorders, usually occurring due to the inheritance of two abnormal genes, namely, the sickle cell gene, and the beta-thalassemia gene. There are two types of sickle cell β-thalassemia: (S/β+) and (S/β0), based on a decrease or complete absence of beta-globin synthesis, respectively. Skeletal complications, such as osteonecrosis, osteomyelitis, and septic arthritis, are well-known sequelae in sickle cell patients due to vaso-occlusive events...

UNLABELLED: Although risk factors such as cholelithiasis and vaso-occlusive crises exist in sickle cell disease, acute pancreatitis and its recurrence are considered rare complications manifesting as acute abdomen. CASE PRESENTATION: A 33-year-old female with sickle cell disease and established cholelithiasis presented to the center with acute abdomen. After examination, investigation, and contrast enhanced computed tomography, acute pancreatitis was diagnosed. Conservative management was done and cholecystectomy was planned but delayed due to low hemoglobin...

INTRODUCTION: Sickle cell disease mainly affects African Americans, and studies on racial differences in sickle cell disease outcomes are scanty. This study examined racial and ethnic differences in sickle cell disease prevalence, comorbidities, and outcomes. METHODS: Using the Nationwide Inpatient Sampling (NIS) database from 2016 to 2018, we identified patients' records with a diagnosis of sickle cell disease using the International Classification of Diseases, Tenth Revision codes (ICD-10)...

OBJECTIVE: To evaluate the association between sickle cell disease (SCD) and severe maternal morbidity (SMM) in a contemporary cohort of deliveries by non-Hispanic Black people. METHODS: We retrospectively examined SMM by using electronic health record data on deliveries by non-Hispanic Black patients between 2011 and 2020 at a single tertiary, public institution. Sickle cell disease was identified during the delivery admission by using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) and International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) codes...

PURPOSE: There is little research on care-seeking preferences during active pain crises for sickle cell disease (SCD) patients and their caregivers. The objective of this study was to identify relevant, patient or caregiver narratives of the pain crisis experience, to understand the factors that contribute to care-seeking during a pain crisis, and to identify preferences when making care-seeking decisions during a pain crisis. PATIENTS AND METHODS: Qualitative semi-structured interviews were conducted with Canadian residents with a self-reported SCD diagnosis, who were either ≥18 years of age or an adolescent between the ages of 12-18...

A real-world analysis was conducted in Italy among sickle cell disease (SCD) patients to evaluate the epidemiology of SCD, describe patients' characteristics and the therapeutic and economic burden. A retrospective analysis of administrative databases of various Italian entities was carried out. All patients with ≥1 hospitalization with SCD diagnosis were included from 01/2010-12/2017 (up to 12/2018 for epidemiologic analysis). The index date corresponded to the first SCD diagnosis. In 2018, SCD incidence rate was 0...

Patients with sickle cell disease frequently present to the hospital for pain control secondary to vaso-occlusive crises (VOCs). Diagnostic challenges exist for healthcare providers in distinguishing joint pain secondary to a VOC from an intraarticular infection at initial presentation due to the lack of established clinical markers exclusive to one or the other. We present a 35-year-old female with sickle cell disease and avascular necrosis of bilateral hips and the right shoulder with several previous admissions for VOC pain control complaining of a "different" kind of pain in her shoulder...

The aim of this study was to characterize the acute complications that required hospitalization in children under 5 years with Sickle Cell Disease. The population included 70 patients, 64% of African ascendency and 95.7% homozygotic for the HbS. We analyzed data from 337 hospitalizations. The first hospitalization occurred before 12 months in 38.6% and the average hospital admission per child was 3.92. The mean duration per hospitalization was 5.36 days. The most common diagnosis was vaso-occlusive crisis (36...

BACKGROUND: Musculoskeletal findings are common in sickle cell patients and may be confused with inflammatory arthritis. In addition, complications such as frequent infections may create difficulties while choosing drugs such as steroids, methotrexate, or antiTNFs. Our aim is to reveal whether the treatment is different in sickle cell patients with rheumatic diseases such as rheumatoid arthritis. METHODS: Patients followed by Rheumatology and Hematology divisions of Hatay Mustafa Kemal University Hospital were retrospectively screened...

INTRODUCTION: The most common cause of death among sickle cell disease (SCD) patients is acute chest syndrome (ACS). Since SCD is a common condition in the Eastern province of the Kingdom of Saudi Arabia (KSA), we aimed to provide a detailed description of the clinical characteristics and ACS management. METHODS: We retrospectively studied pediatric (<14 years) patients with SCD diagnosis who were admitted with ACS or developed ACS after admission from January 2002 to December 2020...