[Prenatal diagnosis of Lesch-Nyhan syndrome].

Neurología : Publicación Oficial de la Sociedad Española de Neurología

The ten commandments of point-of-care ultrasound (POCUS).

CJEM

A strategy of administering a transfusion only when the hemoglobin level falls below 7 or 8 g per deciliter has been widely adopted. However, patients with acute myocardial infarction may benefit from a higher hemoglobin level.

In this phase 3, interventional trial, we randomly assigned patients with myocardial infarction and a hemoglobin level of less than 10 g per deciliter to a restrictive transfusion strategy (hemoglobin cutoff for transfusion, 7 or 8 g per deciliter) or a liberal transfusion strategy (hemoglobin cutoff, &lt;10 g per deciliter). The primary outcome was a composite of myocardial infarction or death at 30 days.

A total of 3504 patients were included in the primary analysis. The mean (&#xb1;SD) number of red-cell units that were transfused was 0.7&#xb1;1.6 in the restrictive-strategy group and 2.5&#xb1;2.3 in the liberal-strategy group. The mean hemoglobin level was 1.3 to 1.6 g per deciliter lower in the restrictive-strategy group than in the liberal-strategy group on days 1 to 3 after randomization. A primary-outcome event occurred in 295 of 1749 patients (16.9%) in the restrictive-strategy group and in 255 of 1755 patients (14.5%) in the liberal-strategy group (risk ratio modeled with multiple imputation for incomplete follow-up, 1.15; 95% confidence interval [CI], 0.99 to 1.34; P&#x2009;=&#x2009;0.07). Death occurred in 9.9% of the patients with the restrictive strategy&#x2009;and in 8.3% of the patients with the liberal strategy (risk ratio, 1.19; 95% CI, 0.96 to 1.47); myocardial infarction occurred in 8.5% and 7.2% of the patients, respectively (risk ratio, 1.19; 95% CI, 0.94 to 1.49).

In patients with acute myocardial infarction and anemia, a liberal transfusion strategy did not significantly reduce the risk of recurrent myocardial infarction or death at 30 days. However,&#xa0;potential&#xa0;harms&#xa0;of a&#xa0;restrictive transfusion strategy&#xa0;cannot be excluded. (Funded by the National Heart, Lung, and Blood Institute and others; MINT ClinicalTrials.gov number, NCT02981407.).

Restrictive or Liberal Transfusion Strategy in Myocardial Infarction and Anemia.

New England Journal of Medicine

Endogenous Cushing's syndrome results from excess glucocorticoid secretion, which leads to a myriad of clinical manifestations, comorbidities, and increased mortality despite treatment. Molecular mechanisms and genetic alterations associated with different causes of Cushing's syndrome have been described in the last decade. Imaging modalities and biochemical testing have evolved; however, both the diagnosis and management of Cushing's syndrome remain challenging. Surgery is the preferred treatment for all causes, but medical therapy has markedly advanced, with new drug options becoming available. Nevertheless, several comorbidities remain even after patient remission, which can affect quality of life. Accurate and timely diagnosis and treatment are essential for mitigating chronic complications of excess glucocorticoids and improving patient quality of life. In this Seminar, we aim to update several important aspects of diagnosis, complications, and treatment of endogenous Cushing's syndrome of all causes.

Cushing's syndrome.

Lancet

The influence of restrictive fluid resuscitation and the early administration of vasopressors on the clinical outcomes in patients with septic shock is not fully understood. The purpose of this study was to evaluate the effects of restrictive fluid management on mortality and organ dysfunction in patients with septic shock.

This study included consecutive patients with septic shock in need of fluid resuscitation. Based on the fluid management provided in the initial resuscitation phase, a comparison was made between a restrictive group and a standard fluid management group. The primary outcome was in-hospital death, while secondary outcomes included organ dysfunction and other adverse events.

A total of 238 patients were included in this study. Restrictive fluid management was administered to 59.2% of patients, while 40.8% received standard fluid management. Restrictive resuscitation was associated with a lower in-hospital mortality rate (24.8% vs. 52.6%), as well as a shorter median ICU stay (8.0 vs. 11.0 days). The restrictive strategy was associated with a significantly lower prevalence of new-onset acute kidney injury (25.5% vs. 51.5%) and a decrease in the incidence of renal replacement therapy (20.6% vs. 40.2%). The standard group had a higher risk of the need for mechanical ventilation and a significantly lower median number of days without a ventilator than the restrictive group. The median duration of vasopressor-free days in the restrictive group was significantly longer than that in the standard group (25.0 vs. 18.0). The administration rate of inotropes in the restrictive group was significantly lower than that in the standard group. A multivariate logistic regression model showed that restrictive fluid management (OR 0.312; 95% CI 0.098-0.994) and vasopressor-free days (OR 0.807; 95% CI 0.765-0.851) protect against in-hospital death, whereas APACHE II scores (OR 1.121; 95% CI 1.018-1.234) were independent risk factors for in-hospital death.

Restrictive fluid resuscitation and early vasopressor protocol in patients with septic shock are associated with better outcomes, indicating that this regimen is feasible and safe.

[Prenatal diagnosis of Lesch-Nyhan syndrome].

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[Prenatal diagnosis of Lesch-Nyhan syndrome].

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