Venous thromboembolism in adults screened for sickle cell trait: a population-based cohort study with nested case-control analysis.

OBJECTIVE: To determine whether sickle cell carriers ('sickle cell trait') have an increased risk of venous thromboembolism (VTE). DESIGN: Cohort study with nested case-control analysis. SETTING: General population with data from 609 UK general practices in the Clinical Practice Research Datalink (CPRD). PARTICIPANTS: All individuals registered with a CPRD general practice between 1998 and 2013, with a medical record of screening for sickle cell between 18 and 75 years of age. MAIN OUTCOMES MEASURES: Incidence of VTE per 10 000 person-years (PY) among sickle cell carriers and non-carriers; and adjusted OR for VTE among sickle cell carriers compared with non-carriers. RESULTS: We included 30 424 individuals screened for sickle cell, with a follow-up time of 179 503 PY, identifying 55 VTEs in 6758 sickle cell carriers and 125 VTEs in 23 666 non-carriers. VTE incidence among sickle cell carriers (14.9/10 000 PY; 95% CI 11.4 to 19.4) was significantly higher than non-carriers (8.8/10 000 PY; 95% CI 7.4 to 10.4). Restricting analysis to confirmed non-carriers was non-significant, but performed on a small sample. In the case-control analysis (180 cases matched to 1775 controls by age and gender), sickle cell carriers remained at increased risk of VTE after adjusting for body mass index, pregnancy, smoking status and ethnicity (OR 1.78, 95% CI 1.18 to 2.69, p=0.006), with the greatest risk for pulmonary embolism (PE) (OR 2.27, 95% CI 1.17 to 4.39, p=0.011). CONCLUSIONS: Although absolute numbers are small, in a general population screened for sickle cell, carriers have a higher incidence and risk of VTE, particularly PE, than non-carriers. Clinicians should be aware of this elevated risk in the clinical care of sickle cell carriers, or when discussing carrier screening, and explicitly attend to modifiable risk factors for VTE in these individuals. More complete primary care coding of carrier status could improve analysis.

Fibromixoma odontogénico: a propósito de un caso servicio de pediatría médica: Hospital Central "Dr. José María Vargas" San Cristobal marzo 2007 Estado Táchira-Venezuela / Odontogenic fibromyxoma: a purpose of a case pediatric medical service Hospital Central

El fibromixoma odontogénico es una lesión infrecuente de naturaleza benigna y origen controversial que aparece en el esqueleto facial, afectando con mayor frecuencia a la mándibula. Ocurre principalmente en la segunda y tecera décadas de la vida y afecta de igual modo a ambos sexos. Escolar masculino de 8 años de edad natural y procedente de Mata de Bracera vía la Victoria estado Apure, cuya madre refiere inicio de su enfermedad actual de 5 años de evolución caracterizada por aumento de volumen en forma progresiva de hemicara derecha sin ningún otro concomitante. Fascie asimétrica con aumento de volumen de hemicara inferior derecha que se extiende hasta el cuello y mastoides. Ojos asimétricos protrucción ocular derecha, pupilas normoreactivos, ojo derecho con amaurosis; obstrucción del conducto auditivo externo derecho, cuello con adenomegalias cervicales y laterales. En la TAC de cráneo se observó una masa de aspecto tumoral hipodensa en hemicara derecha que engloba nervio óptico y parte de la órbita del lado derecho con aumento de volumen de parótida derecha. Se realiza biopsia de la lesión donde se aprecia histológicamente es un tejido blando gelatinoso que no tiene cápsula, constituido por células fusiformes y estrelladas largas con prolongaciones citoplásmicas. Tiene una estroma rica en mucopolisacáridos que confirma diagnóstico de fibromixoma odontogénico. El fibromixoma es una entidad infrecuente en la población pediátrica y en Venezuela no se había publicado ningún caso. Aunque se publicó caso de un niño de 3 años en Bogotá. Colombia en el año 2005.

Understanding sickle cell disorders.

More than 12,500 people in England have sickle cell disorders. This article explains how these disorders call be inherited and discusses the associated clinical problems. The author focuses on social, psychosocial and cultural aspects, and the implications for nursing practice are examined.

The role of cytokines in sickle cell disease.

Sickle cell disease (SCD) is characterized by chronic hemolysis, frequent infections, and recurrent occlusions of microcirculation, which cause painful crises and result in chronic organ damage and failure. Occlusions of the microcirculation and infections are important factors that stimulate the production of cytokines and acute-phase proteins. Cytokines seem to be involved with several possible mechanisms in the pathogenesis of vasoocclusive phenomena in SCD: vascular endothelial activation, induction of red-cell adhesiveness to vascular endothelium, induction of neutrophil adhesiveness to endothelium, development of vascular intimal hyperplasia, platelet activation, endothelin-1 production, and dysregulation of endothelial apoptosis. Cytokines are also thought to be involved in the regulation of hemopoiesis, the inhibition of immune functions, and the development of growth deficits. Investigation of cytokines in SCD patients will elucidate the pathogenesis of the disease and its complications and may help in assessing disease severity and prognosis.

Sickle cell trait as a risk factor for secondary hemorrhage in children with traumatic hyphema.

PURPOSE: To determine risk factors for secondary hemorrhage and poor visual outcome in children with traumatic hyphemas. METHODS: We reviewed 99 eyes of 97 children younger than 18 years who had been hospitalized for hyphema within 48 hours of blunt eye trauma. Inpatient records were examined for race, age, sickle cell trait status, size of hyphema and intraocular pressure at admission, secondary hemorrhage (rebleed of hyphema), and medications while hospitalized. Fifty-five eyes of 53 children had at least 1 month of follow-up or attained best-corrected visual acuity of 20/50 or better at their last outpatient visit. RESULTS: Among 99 eyes of 97 children with traumatic hyphema, secondary hemorrhage occurred in nine eyes (9%). Among 72 eyes of 70 African-American children, secondary hemorrhage occurred in nine eyes (14%), whereas in 27 eyes of 27 white children, there were no secondary hemorrhages. However, when the 14 eyes of 13 sickle cell trait-positive children were excluded from the African-American group, the 57 eyes of sickle cell trait-negative African-American and white children did not have any secondary hemorrhages. The sickle cell trait-positive group had secondary hemorrhages in nine of 14 eyes (64%), significantly (P < .005) different from the 0% rate in the 57 eyes of African-American sickle cell trait-negative and white children. The sickle cell trait-positive group also had higher intraocular pressure and permanent visual impairment. CONCLUSION: Sickle cell trait is a significant risk factor for secondary hemorrhage, increased intraocular pressure, and permanent visual impairment in children who have traumatic hyphemas following blunt trauma.

Removal of an infected right atrial mass in a patient with sickle cell disease.

The use of indwelling central catheters for hyperalimentation, chemotherapy, and long-term venous access is increasing. We report the successful removal of an infected right atrial mass associated with the use of a central catheter in an adult with sickle cell disease. The clinical options for the treatment of infected atrial thrombus as well as the challenge of performing cardiopulmonary bypass in patients with sickle cell disease are briefly discussed.

Orthopaedic manifestations of sickle-cell disease.

Sickle-cell disease is a well-recognized clinical entity. The pathophysiology of this hemoglobinopathy has been described in detail by numerous investigators since the first case report appeared in 1910. Orthopaedic manifestations of sickle-cell disease account for much of the morbidity associated with this disorder, including pain, osteonecrosis, arthritis, and sepsis. Effective management of these bone and joint sequelae reflect accurate diagnosis, understanding of this disorder's pathophysiology, and knowledge of available medical and surgical treatment alternatives. In this review, the authors summarize the major orthopaedic manifestations of sickle-cell disease with special emphasis placed upon osteonecrosis and osteomyelitis, since these conditions are the most disabling and serious complications in patients with sickle-cell disease.

Homozygous sickle cell disease and priapism in the eastern province of Saudi Arabia.

Two adult sickle cell homozygotes from the eastern oases of the Kingdom of Saudi Arabia presented with severe persistent priapism. Each patient had a high Hb F of 24%, and their red cells were neither hypochromic nor microcytic. Priapism probably occurs more frequently in homozygous sickle cell disease than reported previously from this region. It would appear that a high fetal haemoglobin alone without hypochromia and microcytosis--features suggestive of co-existing alpha-thalassaemia--does not protect against this agonizing vaso-occlusive event.

Sickle cell trait and hematuria associated with von Willebrand syndromes.

A von Willebrand syndrome was present in four patients with sickle cell trait and hematuria. The first two patients had severe anemia and active bleeding and received cryoprecipitate, with prompt cessation of hemorrhage. All of the patients had repetitive laboratory and clinical features; that is, reduced, but detectable, factor VIII-related antigen, heterogeneity of, and incongruities within, the coagulation studies performed by consistently defective platelet aggregation to ristocetin correctable only with normal plasma. Bleeding outside the genitourinary tract never was observed. Because of the focal nature of the hemorrhage, the hematuria may not have been perceived as part of a general hemorrhagic disorder and the diagnosis not pursued. These observations suggest that when sickle cell trait and hematuria occur together, a von Willebrand syndrome should be a major diagnostic consideration that ultimately may point toward a rational, effective, easily administered, and clinicially acceptable form of treatment with cryoprecipitate.