[Differential diagnosis of chest pain: a case of acute aortic syndrome]. / Diagnóstico diferencial del dolor torácico: un caso de síndrome aórtico agudo.

Chest pain is one of the most frequent reasons for consulting in any healthcare setting, however its diagnosis remains a challenge for both Primary Care and Emergency Department physicians. We report a case of an Acute Aortic Syndrome which was diagnosed late after an insidious course of chest pain, repetitive syncope, and in which the delay in diagnosis and treatment could be fatal. We also describe the definition, diagnosis, treatment, and outcome of this condition.

Diagnóstico diferencial del dolor torácico: un caso de síndrome aórtico agudo / Differential diagnosis of chest pain: a case of acute aortic syndrome

El dolor torácico es uno de los motivos de consulta más frecuentes en cualquier ámbito sanitario, no obstante sigue suponiendo un reto diagnóstico tanto en la consulta del médico de atención primaria como en el ámbito de los médicos que prestan atención en los servicios de urgencias. Describimos un caso de síndrome aórtico agudo que se presentó de forma insidiosa en forma de dolor torácico y síncope de repetición en el que el retraso en el diagnóstico y tratamiento pudo resultar fatal. Hacemos también un breve repaso en la definición, diagnóstico, tratamiento y pronóstico de esta entidad (AU)

Chest pain is one of the most frequent reasons for consulting in any healthcare setting, however its diagnosis remains a challenge for both Primary Care and Emergency Department physicians. We report a case of an Acute Aortic Syndrome which was diagnosed late after an insidious course of chest pain, repetitive syncope, and in which the delay in diagnosis and treatment could be fatal. We also describe the definition, diagnosis, treatment, and outcome of this condition (AU)

Mitochondrial damage results in a reversible increase in lysosomal storage material in lymphoblasts from patients with juvenile neuronal ceroid-lipofuscinosis (Batten Disease).

We have previously demonstrated reduced phospholipid fatty acid content in blood cells and cultured skin fibroblasts from patients with JNCL. This has led to an experimental treatment regimen consisting of dietary supplementation with polyunsaturated fatty acids (PUFAs). In order to study the effects of PUFA supplementation in vitro, we have developed a laboratory model based upon cultured lymphoblast cell lines. We have transformed lymphocytes from four JNCL patients in whom disease linkage to chromosome 16 was informative. Cells from patients and controls were cultured with and without antibiotic (50 micrograms/ml gentamycin) and with and without PUFA supplementation. None of the control cells demonstrated significant storage under any of the above conditions. In gentamycin treated cells, we observed that many of the mitochondria were damaged. In addition, cells from patients incubated with gentamycin demonstrated large accumulations of autofluorescent storage material. Disease cells grown in the presence of antibiotic and PUFAs did not demonstrate a significant accumulation of storage material; this suggests a direct relationship between mitochondrial damage and storage of autofluorescent material. Moreover, it appears that this storage (but not mitochondrial damage) is reversed by the addition of PUFAs.

Nonsyphilitic spirochetosis in second-trimester fetuses.

Four female fetuses (17-23 weeks) spontaneously aborted by young women (15-19 years old) showed spirochetal microorganisms predominantly in the intestinal lumen and mucosa and to a much lesser extent in other organs. Fetal tissues showed a brisk lymphocytic-plasmacytic response in intestinal mucosa, lungs, and meninges in some cases. In all instances the placenta had chorioamnionitis and severe chronic villitis, with villous vasculitis in some. One fetus had a concomitant cytomegalovirus infection. The observed lesions were reminiscent of Treponema pallidum infections; however, the spirochetes were morphologically different by light and ultrastructural microscopy from T. pallidum and did not react with a silver-enhanced, gold-labeled anti-T. pallidum antibody. In addition, serologic tests for syphilis of the women before or after the abortions were nonreactive. On the basis of clinical pathologic considerations as well as the absence of immunostaining, it is possible also to rule out infections caused by Lyme and relapsing fever Borrelia, Leptospira, and Campylobacter. The spirochetes' prominent tropism for the intestinal tract raises the possibility of a congenital infection with gastrointestinal spirochetal species described in recent years. The placental findings suggest an ascending transamniotic infection, with initial colonization of the intestinal tract and systemic dissemination of the organisms in the fetus and placental villi.

Identification of glyoxylate cycle enzymes in chick liver--the effect of vitamin D3: cytochemistry and biochemistry.

Information regarding the presence of the glyoxylate cycle in chick liver was sought. This metabolic pathway has long been thought to be absent from vertebrate tissues. Previous studies in other tissues have shown that, when present, this pathway is sensitive to vitamin-D. Thus, the effect of long-term vitamin-D deficiency and subsequent vitamin-D replacement on liver structure was studied by light microscopy. In addition, specific biochemical assays for the presence of glyoxylate cycle enzymes were performed. Light microscopy of lipid extracted tissues, light microscopic histochemistry, and quantitative histochemistry showed that the hepatocytes from vitamin-D-deficient animals contained primarily lipid. Hepatocytes from normal and vitamin-D-replete livers contained primarily carbohydrate as judged by their staining with periodic acid-Schiff (PAS). Also, malate synthase positive peroxisomes were seen in hepatocytes from normal and vitamin-D-treated chicks. Structures positive for this glyoxylate cycle enzyme were rarely seen in the hepatocytes from vitamin-D-deficient animals. Biochemical analyses showed the presence of the two unique glyoxylate cycle enzymes, isocitrate lyase and malate synthase, in chick hepatocytes. The activity of these enzymes was markedly increased in the vitamin-D-replete livers. In addition, chick hepatocytes demonstrated the capacity to oxidize fatty acid in the presence of cyanide. This activity, which is characteristic of peroxisomal B-oxidation rather than mitochondrial, was stimulated by vitamin-D treatment. Lastly, incubation of chick liver in the presence of a fatty acid substrate (palmitate) led to higher tissue glycogen content. The latter was further increased in liver from vitamin-D-replete animals. These data show the presence of glyoxylate cycle enzymes in a higher vertebrate and indicate that this tissue is endowed with the capacity to convert lipid to carbohydrate.

The immunohistochemical localization of superoxide dismutase activity in the avian epithelial growth plate.

Superoxide dismutase (SOD) is a 'scavenger' enzyme which catalyses the dismutation (reduction-oxidation) of the superoxide anion (O2-.), a toxic free radical generated during normal cellular respiration. Light microscopy employing immunohistochemistry was utilized for localizing SOD activity in the chick epiphyseal cartilage. Antibodies to mammalian liver CuZn-SOD were prepared and the avidin-biotin-peroxidase technique (ABC complex) was utilized to localize activity for this enzyme in the growth plate cartilage. The localization of enzyme activity varied in accordance with the characteristic zonation pattern of the growth plate (zone of proliferation, zone of maturation, zone of cell hypertrophy and zone of matrix calcification). In the upper regions of the epiphyseal cartilage (the zones of proliferation and maturation), where the vascularity is poor and the oxygen tension low, SOD activity was localized within the chondrocytes. No extracellular activity was observed. However, in the lower regions of the growth plate (the zones of cell hypertrophy and matrix calcification), where both the vascularity and the oxygen tensions are increased, SOD activity was intense in both the chondrocytes and the surrounding extracellular matrix. Thus, the distribution of SOD enzyme activity in this tissue seems to vary in accordance with the level of oxygen present. The significance of the extracellular SOD activity, seen in the lower aspects of the growth plate cartilage, may indicate the sensitivity of matrix components, especially collagen, to toxic free radicals such as the superoxide anion.

The immunocytochemical localization of superoxide dismutase in the enterocytes of the avian intestine: the effect of vitamin D3.

Both light microscopical and electron microscopical immunocytochemical techniques were utilized to localize CuZn-superoxide dismutase (SOD) in the duodenum of normal, rachitic and vitamin-D3-replete chicks. This enzyme catalyses the dismutation of the superoxide anion, a toxic free radical generated during the normal aerobic metabolism of most respiring cells. Light microscopy showed no SOD activity associated with the duodenal enterocytes of normal and rachitic chicks. However, in rachitic animals subsequently treated with vitamin D, i.e. vitamin-D-replete chicks, intense immunoreactivity for the enzyme was seen in association with the apical border of the duodenal absorptive cells. Immunostaining for SOD was not seen in goblet cells. With electron microscopy, immunostaining for SOD activity was identified in association with the apical microvilli and, to a lesser degree, with the terminal web, a well as in association with both lysosomes and peroxisomes. From this report it appears that there is a physiological relationship between vitamin D, SOD and the intestinal absorptive cell. However, the precise relationship must await further clarification.

The ultrastructural immunocytochemical localization of superoxide dismutase in the amphibian urinary bladder: effect of aldosterone.

Transmission electron microscopy and immunohistochemistry, the latter employing the avidin-biotin-peroxidase (ABC complex) technique, were utilized to localize copper-zinc superoxide dismutase (CuZn-SOD) enzyme activity in the epithelial cells of the toad urinary bladder mucosa. This 'scavenger' enzyme catalyses the dismutation (reduction-oxidation) of the superoxide anion (O2-), a toxic free radical generated during normal cellular respiration. In unstimulated epithelial cells, enzyme activity was seen in the cytosol of granular, mitochondrial-rich and goblet cells. The basal cells were generally devoid of enzyme activity. In addition to the cytosol, SOD activity was also seen in association with the apical plasma membrane of the epithelial cells. In the presence of the steroid hormone aldosterone (10(-7)M, 30 min-6h), CuZn-SOD activity was markedly increased along the luminal mucosal membrane of granular, mitochondrial-rich and goblet cells. This increase was seen as early as 30 min after the addition of hormone, and as long as 6h after treatment. The cytosolic reaction was usually decreased or absent under these conditions. From the data presented, it appears that CuZn-SOD is involved in electrolyte (sodium) transport in the epithelial cells of the toad urinary bladder. The latter may involve hormone-induced alterations in luminal cell membrane structure and chemistry.

The glyoxylate cycle in rat epiphyseal cartilage: the effect of vitamin-D3 on the activity of the enzymes isocitrate lyase and malate synthase.

The effect of vitamin-D deficiency and subsequent vitamin-D replacement on the metabolism of rat epiphyseal growth plate cartilage was studied. Biochemical analyses showed the presence of the two unique glyoxylate cycle enzymes isocitrate lyase and malate synthase in cartilage. The activity of these enzymes was markedly increased after treatment with the vitamin. Additionally, rat cartilage showed the capacity to oxidize fatty acid in the presence of cyanide. This cyanide-insensitive fatty acid oxidation is characteristic of peroxisomal B-oxidation rather than mitochondrial B-oxidation. Vitamin-D treatment also increased fatty acid oxidation. Lastly, incubation of rat cartilage in the presence of a fatty acid substrate such as palmitate, resulted in a higher tissue glycogen content. Tissue glycogen was further elevated by vitamin-D. Such data indicate the presence of glyoxylate cycle enzymes in a vertebrate tissue and raise the possibility that mammalian cartilage has the capacity to convert lipid to carbohydrate.