Unstable angina secondary to left main coronary artery vasculitis as a rare initial manifestation of systemic lupus erythematosus. A case report.

We present the case of a woman in her third decade of life, known to have difficult-to-control mixed headaches and polycystic ovary syndrome, under hormonal treatment. Without any other manifestation, the patient debuted with an acute coronary syndrome classified as unstable angina. Electrocardiogram showed anterior and lateral ST segment depression and ST elevation in aVR. Coronary computer tomography and coronary angiography showed evidence of significant obstruction of the left main coronary artery. The patient was diagnosed with systemic lupus erythematosus (SLE), and was classified as vasculitis secondary to SLE as an unusual initial manifestation.

Se presenta el caso de una mujer en la tercera década de la vida, con cefalea mixta de difícil control y síndrome de ovario poliquístico bajo tratamiento hormonal, sin ninguna otra manifestación, la cual debutó con un síndrome coronario agudo tipo angina inestable. El electrocardiograma mostró infradesnivel en cara anterior y lateral, así como supradesnivel del ST en aVR. La angiotomografia de coronarias y la coronariografía mostraron evidencia de obstrucción importante del tronco de la coronaria izquierda. Tras el abordaje se llegó al diagnóstico de vasculitis secundaria a lupus eritematoso sistémico como manifestación inicial poco habitual.

Store-operated channels in the pulmonary circulation of high- and low-altitude neonatal lambs.

We determined whether store-operated channels (SOC) are involved in neonatal pulmonary artery function under conditions of acute and chronic hypoxia, using newborn sheep gestated and born either at high altitude (HA, 3,600 m) or low altitude (LA, 520 m). Cardiopulmonary variables were recorded in vivo, with and without SOC blockade by 2-aminoethyldiphenylborinate (2-APB), during basal or acute hypoxic conditions. 2-APB did not have effects on basal mean pulmonary arterial pressure (mPAP), cardiac output, systemic arterial blood pressure, or systemic vascular resistance in both groups of neonates. During acute hypoxia 2-APB reduced mPAP and pulmonary vascular resistance in LA and HA, but this reduction was greater in HA. In addition, isolated pulmonary arteries mounted in a wire myograph were assessed for vascular reactivity. HA arteries showed a greater relaxation and sensitivity to SOC blockers than LA arteries. The pulmonary expression of two SOC-forming subunits, TRPC4 and STIM1, was upregulated in HA. Taken together, our results show that SOC contribute to hypoxic pulmonary vasoconstriction in newborn sheep and that SOC are upregulated by chronic hypoxia. Therefore, SOC may contribute to the development of neonatal pulmonary hypertension. We propose SOC channels could be potential targets to treat neonatal pulmonary hypertension.

Long-term exposure to high-altitude chronic hypoxia during gestation induces neonatal pulmonary hypertension at sea level.

We determined whether postnatal pulmonary hypertension induced by 70% of pregnancy at high altitude (HA) persists once the offspring return to sea level and investigated pulmonary vascular mechanisms operating under these circumstances. Pregnant ewes were divided into two groups: conception, pregnancy, and delivery at low altitude (580 m, LLL) and conception at low altitude, pregnancy at HA (3,600 m) from 30% of gestation until delivery, and return to lowland (LHL). Pulmonary arterial pressure (PAP) was measured in vivo. Vascular reactivity and morphometry were assessed in small pulmonary arteries (SPA). Protein expression of vascular mediators was determined. LHL lambs had higher basal PAP and a greater increment in PAP after N(G)-nitro-L-arginine methyl ester (20.9 ± 1.1 vs. 13.7 ± 0.5 mmHg; 39.9 ± 5.0 vs. 18.3 ± 1.3 mmHg, respectively). SPA from LHL had a greater maximal contraction to K(+) (1.34 ± 0.05 vs. 1.16 ± 0.05 N/m), higher sensitivity to endothelin-1 and nitroprusside, and persistence of dilatation following blockade of soluble guanylate cyclase. The heart ratio of the right ventricle-to-left ventricle plus septum was higher in the LHL relative to LLL. The muscle area of SPA (29.3 ± 2.9 vs. 21.1 ± 1.7%) and the protein expression of endothelial nitric oxide synthase (1.7 ± 0.1 vs. 1.1 ± 0.2), phosphodiesterase (1.4 ± 0.1 vs. 0.7 ± 0.1), and Ca(2+)-activated K(+) channel (0.76 ± 0.16 vs. 0.30 ± 0.01) were greater in LHL compared with LLL lambs. In contrast, LHL had decreased heme oxygenase-1 expression (0.82 ± 0.26 vs. 2.22 ± 0.44) and carbon monoxide production (all P < 0.05). Postnatal pulmonary hypertension induced by 70% of pregnancy at HA promotes cardiopulmonary remodeling that persists at sea level.

Enfermedad de niemann pick error innato del metabolismo de los lípidos: reporte de caso

Se hace una revisión de la enfermedad de Niemann-Pick que es una alteración innata del metabolismo de los fosfolípidos, la ESPINGOMIELINA, que se acumula en los lisosomas, constituyendo la enfermedad lisosomal, debido a una alteración cualitativa o cuantitativa de la ESZPINGOMIELINASA. La enfermedad es transmitida en forma autosómica recesiva, alterando los diversos órganos, especialmente el sistema nervioso y el fondo de ojo en el cual se observa en la mácula una mancha de color cereza. A continuación se presenta el caso problema en un indígena puro del Cañar-Ecuador (años anteriores se encontró otro caso que fue descrito por su extrema rareza), existe consanguinidad; hepatoesplenomegalia, mancha roja en mácula y células espúmosas en médula ósea. Un hermano falleció con identicas características clínicas.