Cardiovascular function and treatment in ß-thalassemia major: a consensus statement from the American Heart Association.

This aim of this statement is to report an expert consensus on the diagnosis and treatment of cardiac dysfunction in ß-thalassemia major (TM). This consensus statement does not cover other hemoglobinopathies, including thalassemia intermedia and sickle cell anemia, in which a different spectrum of cardiovascular complications is typical. There are considerable uncertainties in this field, with a few randomized controlled trials relating to treatment of chronic myocardial siderosis but none relating to treatment of acute heart failure. The principles of diagnosis and treatment of cardiac iron loading in TM are directly relevant to other iron-overload conditions, including in particular Diamond-Blackfan anemia, sideroblastic anemia, and hereditary hemochromatosis. Heart failure is the most common cause of death in TM and primarily results from cardiac iron accumulation. The diagnosis of ventricular dysfunction in TM patients differs from that in nonanemic patients because of the cardiovascular adaptation to chronic anemia in non-cardiac-loaded TM patients, which includes resting tachycardia, low blood pressure, enlarged end-diastolic volume, high ejection fraction, and high cardiac output. Chronic anemia also leads to background symptomatology such as dyspnea, which can mask the clinical diagnosis of cardiac dysfunction. Central to early identification of cardiac iron overload in TM is the estimation of cardiac iron by cardiac T2* magnetic resonance. Cardiac T2* <10 ms is the most important predictor of development of heart failure. Serum ferritin and liver iron concentration are not adequate surrogates for cardiac iron measurement. Assessment of cardiac function by noninvasive techniques can also be valuable clinically, but serial measurements to establish trends are usually required because interpretation of single absolute values is complicated by the abnormal cardiovascular hemodynamics in TM and measurement imprecision. Acute decompensated heart failure is a medical emergency and requires urgent consultation with a center with expertise in its management. The first principle of management of acute heart failure is control of cardiac toxicity related to free iron by urgent commencement of a continuous, uninterrupted infusion of high-dose intravenous deferoxamine, augmented by oral deferiprone. Considerable care is required to not exacerbate cardiovascular problems from overuse of diuretics or inotropes because of the unusual loading conditions in TM. The current knowledge on the efficacy of removal of cardiac iron by the 3 commercially available iron chelators is summarized for cardiac iron overload without overt cardiac dysfunction. Evidence from well-conducted randomized controlled trials shows superior efficacy of deferiprone versus deferoxamine, the superiority of combined deferiprone with deferoxamine versus deferoxamine alone, and the equivalence of deferasirox versus deferoxamine.

Epilepsy in transition from child care to adult service: a missing link in sub-Saharan Africa.

Epilepsy is a common chronic neurological disease which predominantly affects children and young adults. The disease is highly stigmatised and transition from child care to adult services is not routine in many low- and middle-income countries. Where a treatment system follows routines which cannot be sustained in such countries because of specialised manpower challenges, it becomes imperative that appropriate care models be sought for patients whose seizures fail to remit in childhood. In teaching hospitals, well-organised, multidisciplinary meetings and a planned transfer between paediatric and adult neurologists will be feasible. However, an alternative model is advocated at the community level where the majority of the patients reside which involves task shifting to general practitioners and community healthcare workers. The latter can organise home visits to ensure management compliance. This will ensure better seizure outcomes and a good quality of life for epileptic patients.

Randomized controlled trial of remote ischemic preconditioning and atrial fibrillation in patients undergoing cardiac surgery.

AIM: To study whether remote ischemic preconditioning (RIPC) has an impact on clinical outcomes, such as post-operative atrial fibrillation (POAF). METHODS: This was a prospective, single-center, single-blinded, randomized controlled study. One hundred and two patients were randomized to receive RIPC (3 cycles of 5 min ischemia and 5 min reperfusion in the upper arm after induction of anesthesia) or no RIPC (control). Primary outcome was POAF lasting for five minutes or longer during the first seven days after surgery. Secondary outcomes included length of hospital stay, incidence of inpatient mortality, myocardial infarction, and stroke. RESULTS: POAF occurred at a rate of 54% in the RIPC group and 41.2% in the control group (P = 0.23). No statistically significant differences were noted in secondary outcomes between the two groups. CONCLUSION: This is the first study in the United States to suggest that RIPC does not reduce POAF in patients with elective or urgent cardiac surgery. There were no differences in adverse effects in either group. Further studies are required to assess the relationship between RIPC and POAF.

Considerações psicanalíticas sobre o tratamento do outro no autismo / Psychoanalytical considerations on the treatment of the other on the autism / Consideraciones sobre el tratamiento psicoanalítico del otro en el autismo

O presente artigo busca discutir os avanços teóricos de Rosine e Robert Lefort sobre a clínica do autismo sublinhando, sobretudo a formulação de uma clínica onde não há Outro mediante a apresentação de fundamentos da teoria psicanalítica de orientação lacaniana. Para tal, o estudo de componentes estruturais do autismo torna-se essencial visto que a publicação de Daniel Tammet sobre seu funcionamento subjetivo esclarece elementos fundamentais, a saber: encapsulamento, objetos autísticos, duplos e construção de uma língua própria. Enfim, uma vez que a tentativa da construção de tais componentes pelo sujeito autista opera-se, um tratamento do Outro se torna possível.

The current article has the aim to discuss the theoretical advances of Rosine and Robert Lefort where there isn't Other based on the fundaments' presentation of psychoanalyses' theory of lacanien orientation. For that, the study of structural components of autism becomes essential due to the discussion of a clinical study case - the recent publication of Daniel Tammet and its subjective functionality - based on fundamental elements such as: self-absorbed, autistic objects, doubles and construction of a single and own language. To conclude, once the construction of such components is tried and done by the person itself, an Other's treatment is possible.

Este artículo tiene como objetivo discutir los avances teóricos de Rosine y Robert Lefort en la clínica del autismo destacando la formulación de una clínica en la que no hay Otro, basada en la presentación de las fundaciones de la teoría psicoanalítica de orientación lacaniana. Así, el estudio de los componentes estructurales del autismo es esencial ya que la publicación de Daniel Tammet y su funcionamiento subjetivo clarea elementos básicos: encapsulado, objetos autistas, duplos y la construcción de un lenguaje propio. Por último, si la construcción de estos componentes por sujeto autista opera, el tratamiento del Otro se hace posible.