Rotation thromboelastometry and the hypercoagulable state in Cushing's syndrome.

INTRODUCTION: Rotation thromboelastometry (ROTEM®) can be used for hypercoagulability evaluation. Cushing's syndrome (CS) is associated with hypercoagulability; however, ROTEM® has never been evaluated in this setting. OBJECTIVE: To evaluate hypercoagulability in CS using ROTEM® and to correlate these parameters with coagulation markers and with the presence of deep vein thrombosis. DESIGN AND METHODS: Thirty patients with active CS (26 women) and 30 controls matched for age, sex, body mass index, diabetes mellitus, arterial hypertension, ABO blood group and smoking were included. We measured levels of activated partial thromboplastin time (aPTT), platelets, fibrinogen, D-dimer, factor VIII (FVIII), von Willebrand factor (vWF) and C-reactive protein. ROTEM® was used to evaluate the intrinsic (INTEM), extrinsic (EXTEM) and fibrinogen (FIBTEM) pathways. Doppler ultrasonography was performed to search for lower limbs deep vein thrombosis. RESULTS: INTEM clotting time using ROTEM® was shorter in patients than in controls (P = 0·04). Other ROTEM® parameters were not different. Mean aPTT was shorter in patients than in controls (P = 0·001). The FVIII, vWF and D-dimer levels were higher in patients than in controls (P = 0·001, 0·001 and 0·02, respectively). Obese CS patients presented higher levels of platelets and alterations in maximum clot formation (MCF), alpha angle and maximum speed of clot formation of INTEM (P = 0·03, 0·02 and 0·02, respectively) and an increase in the MCF of FIBTEM (P = 0·02). No deep vein thrombosis was found. CONCLUSIONS: Although FVIII and vWF were abnormal in CS patients, only the initiation clot formation was different in the ROTEM® methodology and no deep vein thrombosis was found.

Brazilian Guidelines for transcranial doppler in children and adolescents with sickle cell disease

BACKGROUND: Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used. OBJECTIVE: To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so that related health policies can be expanded, and thus contribute to reduce morbidity and mortality. METHODS: The guidelines were formulated in a consensus meeting of experts in transcranial Doppler and sickle cell disease. The issues discussed were previously formulated and scientific articles in databases (MEDLINE, SciELO and Cochrane) were carefully analyzed. The consensus for each question was obtained by a vote of experts on the specific theme. RESULTS: Recommendations were made, including indications for the use of transcranial Doppler according to the sickle cell disease genotype and patients age; the necessary conditions to perform the exam and its periodicity depending on exam results; the criteria for the indication of blood transfusions and iron chelation therapy; the indication of hydroxyurea; and the therapeutic approach in cases of conditional transcranial Doppler. CONCLUSION: The Brazilian guidelines on the use of transcranial doppler in sickle cell disease patients may reduce the risk of strokes, and thus reduce the morbidity and mortality and improve the quality of life of sickle cell disease patients.

Cor triatriatum em paciente adulta acompanhada durante a gestação / Clinical follow-up of a pregnant woman with cor triatriatum

Cor triatriatum (CT) é uma cardiopatia congênita rara que geralmente cursa com sintomas nos primeiros anos de vida. Na ausência de outras malformações cardíacas associadas e na dependência do grau de comunicação entre a câmara superior e o átrio esquerdo (AE), os pacientes podem atingir a idade adulta. Relatamos um caso de uma paciente adulta assintomática portadora de CT diagnosticado pelo ecocardiograma transtorácico (ETT) e acompanhada clinicamente durante a gestação.

Cor triatriatum (CT) is a rare congenital cardiac anomaly that usually becomes symptomatic in the first years of life. If the condition is not associated with other cardiac defects, and depending on the degree of communication between the upper chamber and left atrium (LA), patients may reach adulthood. We report a case of an asymptomatic, adult, female patient with CT diagnosed by transthoracic echocardiogram (TTE) and followed-up during pregnancy.