Aortitis as a Rare Cause of Aortic Aneurysm and Valve Regurgitation: Is Repair Precluded?

Aortitis is an infrequent cause of aortic root dilatation and aortic valve regurgitation. Valve-sparing procedures have been proposed, but there is not clear evidence of which is the treatment of choice. We report the case of a 38-year-old pregnant lady with a diagnosis of idiopathic aortitis associated with aortic root aneurysm and severe aortic valve regurgitation.

Cambio de personalidad como primera manifestación de una esclerosis múltiple / Personality change as an onset symptom of multiple sclerosis

Introducción. La esclerosis múltiple es una enfermedad neurológica autoinmune, de etiología compleja y de curso crónico, que afecta con mayor frecuencia a población adulta joven, sobre todo a mujeres. El cuadro clínico incluye toda una serie de síntomas neurológicos, cognitivos y psiquiátricos, que van a afectar a casi todas las áreas del funcionamiento del individuo, provocando una serie de discapacidades tanto físicas como mentales, que a su vez traerán como consecuencia importantes cambios emocionales y en el estilo de vida. El desarrollo de clínica psiquiátrica relacionada con el afecto y la conducta se presenta a lo largo de la evolución de la enfermedad, sobre todo en fases avanzadas, siendo poco frecuente su aparición en el debut de la esclerosis múltiple. Caso clínico. Se expone el caso de un paciente en el que el inicio de la esclerosis múltiple estaba asociado a la aparición de clínica psiquiátrica; en concreto, presentaba cambios en sus rasgos de personalidad, confabulaciones y alteraciones de conducta de 3 años de evolución que fueron atribuidos inicialmente a un trastorno psiquiátrico, lo que trajo como consecuencia un retraso en el diagnóstico de su enfermedad neurológica. Conclusiones. La aparición de clínica psiquiátrica atípica, o los cambios en la expresión clínica de la enfermedad psiquiátrica de un paciente, deben constituir un signo de alarma que conduzca a realizar las exploraciones complementarias necesarias para descartar la presencia de una enfermedad orgánica subyacente (AU)

Introduction. Multiple sclerosis is a chronic, autoimmune neurological disease. Its pathogenesis has been shown to be complex and it more often affects young females. Its symptoms include neurological, cognitive, and psychiatric manifestations that can cause physical and mental disabilities with great impact on emotional state and life style. The development of psychiatric symptoms related with affectivity and behaviour are usually observed in advanced stages, being infrequent in the initial stages of the disease. Clinical case. The case is presented on a patient with psychiatric symptoms since multiple sclerosis onset. These symptoms included change in personality traits, confabulations, and behavioural disorders. The patient suffered these manifestations for three years, and were initially attributed to a psychiatric disorder. As a result, there was a delay in the diagnosis of the neurological disease. Conclusions. The appearance of atypical psychiatric symptoms, or a change in the clinical expression of a psychiatric disease, must be a warning sign for all physicians, and requires performing supplementary tests in order to rule out potentially serious medical diseases (AU)

Péptidos natriuréticos e hipertensión arterial pulmonar / Natriuretic peptides and pulmonary arterial hypertension

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Prevalence of exercise pulmonary arterial hypertension in scleroderma.

OBJECTIVE: Pulmonary arterial hypertension (PAH) is a complication of scleroderma (systemic sclerosis, SSc); as soon as PAH develops, the patient's prognosis deteriorates rapidly. Early detection of PAH ensures timely treatment. We investigated the prevalence of exercise-induced PAH in a cohort of patients with SSc, and examined the relation between exercise-induced PAH and clinical characteristics and biochemical markers. METHODS: Patients with SSc and normal resting systolic pulmonary arterial pressure (sPAP) were studied. Eligible patients were asked to perform cycloergometer exercise until exhaustion, and exercise sPAP was measured. All patients had their pulmonary function tested and underwent echocardiography at rest. Brain natriuretic peptide (BNP) was also determined. RESULTS: Forty-one patients with SSc were studied. Mean sPAP at rest was 29.7 mm Hg, rising to a mean of 41.4 mm Hg on exercise. Eleven of 41 patients (26.8%) had sPAP post-exercise > 50 mm Hg and 8/41 (19.5%) > 55 mm Hg. A significant correlation was found between exercise sPAP and DLCO (p = 0.008) and between sPAP and BNP levels (p = 0.04). Pre-existing severe Raynaud's phenomenon was more prevalent (50% vs 20%), DLCO levels lower (78.9 vs 92.7 % predicted), and BNP levels higher (72.6 vs 42.1 pmol/ml) in patients with exercise sPAP > 55 mm Hg. CONCLUSION: The prevalence of exercise-induced PAH in patients with scleroderma is high. Patients with lower DLCO and higher levels of BNP are at higher risk of developing higher sPAP. Studies with longterm followup are required to evaluate the risk of developing resting PAH in these patients.

Amisulpride en los trastornos de ideas delirantes. Estudio observacional / Amisulpride in delusional disorder. An observational study

Introducción: Hay poca información científica sobre el tratamiento de los trastornos de ideas delirantes, y menos aún con los antipsicóticos atípicos. En este trabajo nos proponemos investigar la efectividad, tolerabilidad y efectos adversos del amisulpride en dichos trastornos. Metodología: Estudio piloto observacional, de cohorte y retrospectivo. La muestra son 16 pacientes diagnosticados de trastornos de ideas delirantes, tratados en nuestra Unidad Hospitalaria de Psiquiatría discrecionalmente con amisulpride como antipsicótico de elección, en las condiciones habituales. Resultados: De manera estadísticamente significativa el amisulpride resultó efectivo en los trastornos de ideas delirantes a las dos semanas de tratamiento, y siguió siéndolo a los tres meses; fue bien tolerado; como efectos secundarios se detectaron síntomas psíquicos y neurológicos en algún sujeto, de gravedad leve. Conclusiones: Nuestros resultados parecen apoyar el uso de un fármaco tan selectivo sobre los receptores D2/D3 como es el amisulpride en los trastornos de ideas delirantes. Sin embargo, son necesarios estudios de más adecuada calidad metodológica al respecto

Introduction: There is little e:xisting information on the treatment of delusional disorders, and even less on treatments using atypical antipsychotic drugs. Our objective is to investigate the effectiveness, tolerability, and adverse effects of amisulpride in the treatment of said disorders. Methodology: A pilot retrospective cohort observational study, the sample being 16 patients diagnosed with delusional disorders who were discretionally treated in our psychiatric unit, under habitual conditions, with amisulpride as chosen antipsychotic. Results: 1n a statistically significant way, amisulpride began to take effect two weeks after the start of treatment and continued to prove effective three months after; amisulpride was well tolerated; adverse effects detected in some subjects were psychical and neurological symptoms of a light nature. Conclusions: Our results seem to support the use of a very selective drug on D2/D3 receptors as amisulpride for treating delusional disorders. There is a need, however, for further studies of more appropriate methodological quality

Incidence and clinicobiologic characteristics of leukemic B-cell chronic lymphoproliferative disorders with more than one B-cell clone.

Leukemic B-chronic lymphoproliferative disorders (B-CLPDs) are generally believed to derive from a monoclonal B cell; biclonality has only occasionally been reported. In this study, we have explored the incidence of B-CLPD cases with 2 or more B-cell clones and established both the phenotypic differences between the coexisting clones and the clinicobiologic features of these patients. In total, 53 B-CLPD cases with 2 or more B-cell clones were studied. Presence of 2 or more B-cell clones was suspected by immunophenotype and confirmed by molecular/genetic techniques in leukemic samples (n = 42) and purified B-cell subpopulations (n = 10). Overall, 4.8% of 477 consecutive B-CLPDs had 2 or more B-cell clones, their incidence being especially higher among hairy cell leukemia (3 of 13), large cell lymphoma (2 of 10), and atypical chronic lymphocytic leukemia (CLL) (4 of 29). In most cases the 2 B-cell subsets displayed either different surface immunoglobulin (sIg) light chain (n = 37 of 53) or different levels of the same sIg (n = 9 of 53), usually associated with other phenotypic differences. Compared with monoclonal cases, B-CLL patients with 2 or more clones had lower white blood cell (WBC) and lymphocyte counts, more frequently displayed splenomegaly, and required early treatment. Among these, the cases in which a CLL clone coexisted with a non-CLL clone were older and more often displayed B symptoms, a monoclonal component, and diffuse infiltration of bone marrow and required early treatment more frequently than cases with monoclonal CLL or 2 CLL clones.