[Craniofacial strategy for syndromic craniosynostosis]. / Stratégie craniofaciale pour les faciocraniosténoses.

The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…).

On the role of sterol in the formation of the amphotericin B channel.

Amphotericin B is an antimycotic agent that has been studied for a long time, both because of its pharmacological action and the interest in understanding how this ionic channel works. It has been proposed that the channel is formed by a barrel of monomers, and that the presence of sterol is needed for the formation of such a barrel. As a matter of fact this need of a sterol has been used as a guiding idea in attempts to design derivatives more efficient in the discrimination of the cholesterol containing membranes, as compared to the ergosterol containing ones, henceforth diminishing the unwanted side effects in its pharmacological use. In this work we show that unitary channels that appear in a cholesterol containing membrane also appear when this membrane is free of cholesterol. We prove this to be the case for two membranes, a biological one, asolectin, and a synthetic one, DMPC. We then advance the idea that the role of sterols in the formation of the amphotericin B channel is related to the effects they have on the structure of the membrane itself, rather than to a direct involvement in the channel formation. We further look into the effect that different cholesterol concentrations in the membrane produce on the single channel properties.

Contribution of the spanish agency for medicines and healthcare products to the European committee for the evaluation of medicinal products for human use. / Contribución de la Agencia Española de Medicamentos y Productos Sanitarios al Comité Europeo de Evaluación de Medicamentos de Uso Humano.

The centralized procedure for registering medicinal products involves a joint assessment by all regulatory agencies of European Union member states, which are coordinated by the European Medicines Agency. Since its implementation in 1995, the Spanish Agency for Medicines and Healthcare Products (AEMPS) has actively contributed to the committee on medicinal products for human use. The therapeutic areas in which AEMPS has the greatest presence are cardiovascular, sensory organs (mainly ophthalmology) and genitourinary/sexual hormones. The technical staff of AEMPS contributes their expertise and extensive experience to this task, as do the practitioners of the Spanish healthcare system who act as external experts, providing their clinical vision and bringing the daily clinical practice to the evaluation of medicinal products. As with other European decision spaces, the joint participation of the member states is not homogeneous, with a minority of countries still heading the majority of assessments for the committee on medicinal products for human use, although all member countries take part in the final decision.

Enthalpic and entropic contributions to the conformational free energies of methylthio, methylsulfinyl, methylsulfonyl, phenylthio, phenylsulfinyl, and phenylsulfonyl

A variable-temperature NMR study of (cis-4-methylcyclohexyl)methyl sulfide (1), sulfoxide (2), and sulfone (3), as well as (cis-4-methylcyclohexyl)phenyl sulfide (4), sulfoxide (5), and sulfone (6) allowed determination of the thermodynamic parameters, DeltaH degrees and DeltaS degrees, for the title groups. Reproduction of the experimental results with Allinger's MM3 program was successfully accomplished in the case of the sulfoxide and sulfide groups. Nevertheless, modification of the original force field torsional parameters was required in order to adequately reproduce the experimentally observed behavior of the sulfonyl derivatives. Rationalization of the enthalpic and entropic contributions to DeltaG degrees [S(O)(n)()R, n = 0, 1, 2; R = CH(3), Ph] is advanced in terms of the steric characteristics of these sulfur-containing groups and the resulting rotameric populations in the axial and equatorial monosubstituted cyclohexanes.

Synthesis of the new alpha- and beta-adrenergic antagonist 1-[1-(2-benzodioxanylmethyl)-4-piperidyl]amino-3-(1-naphthoxy)-2- propanol.

The synthesis and in vitro alpha- and beta-adrenergic blocking potency of 1-[1-(2-benzodiaxanylmethyl)-4-piperidyl]amino-3-(1-naphthoxy-2-pr opanol (I) are described. Thus, N-benzyl-4piperidone was protected and debenzylated to the carbamate (V), which upon alkaline hydrolysis and acylation gave benzodioxanic amide (IX). Reduction of the amide group, deprotection of the ketone function of (X), and reductive amination gave the 4-aminopiperidine (XIII), which was finally condensed with the appropriate epoxide to yield the aminopropanol (I). Compound (I) is formally derived from a combination of piperoxan (II) and propranolol (III), and was approximately 10 times less potent than each one of these drugs, as an alpha- and beta-adrenergic blocker respectively.

Escala de desesperanza de Beck como instrumento útil para detectar riesgo de suicidio en universitarios chilenos / Beck Hopelessness Scale as a suicide risk screening tool among Chilean university students

Resumen El objetivo del presente estudio fue determinar la estructura factorial y las características psicométricas de la Escala de Desesperanza de Beck (BHS), validando su utilización en Chile como instrumento útil para la pesquisa de riesgo suicida en jóvenes universitarios. Método: La muestra estuvo compuesta por 200 estudiantes de primer año de la Universidad Austral de Chile, quienes respondieron voluntariamente el consentimiento informado, la BHS y los otros cuestionarios que permitieron verificar la validez concurrente y discriminante de la escala. Se realizaron análisis de consistencia interna, correlacionales y de componentes principales. Resultados: Los distintos procedimientos de análisis permiten sostener que la escala BHS es un instrumento confiable y valido para detectar riesgo de suicidio en población universitaria chilena. Asimismo, los análisis arrojaron un modelo de cuatro factores, el que varía de lo propuesto por los autores de la BHS, aunque es concordante con lo encontrado en otros estudios internacionales. Conclusiones: La presente investigación permite suponer que la BHS es un instrumento confiable y válido para detectar riesgo suicida en estudiantes universitarios chilenos. Este instrumento breve y fácil de administrar permitiría detectar eficaz y tempranamente suicidalidad, siendo un aporte a la prevención de este fenómeno en nuestro país.

Introduction: The aim of present study is to determine the psychometric properties and the factorial structure of the Beck Hopelessness Scale (BHS), validating its use in Chile as a suicide risk screening tool among university students. Method: The sample included 200 college freshmen undergraduate belonging to the Austral University of Chile, who through the website of the university, answered voluntary the Informed Consent and the questionnaires that allowed verifying the concurrent and discriminant validity of the BHS scale. The internal consistency of this scale was evaluated by Cronbach's alpha internal consistency. The data were analyzed through correlational and principal component analyzes. Results: The different Statistical analysis revealed a high level of internal consistency in the BHS scale, being a reliability and valid instrument to detect suicide risk in Chilean university population. Likewise, Principal Component Analysis showed a four-factor model, which varies from that proposed by the BHS authors, although it is consistent with that found in other international studies. Conclusions: The present investigation allows us to suppose that the BHS is a reliable and valid instrument to detect suicidal risk in Chilean university students. This brief and easy to administer instrument would allow to detect effective and early suicidality, being a contribution to the prevention of this phenomenon in our country.

Post-cardiotomy circulatory support with the Terumo Baby-Rx oxygenator in a newborn.

Some patients with severe post-cardiotomy ventricular dysfunction are unable to be separated from cardiopulmonary bypass (CPB). In this setting, extracorporeal circulatory support (ECS) should be instituted to obtain eventual myocardial recovery. We present a newborn in whom an ECS was established with the oxygenator used for the surgical correction.