Gene expression and in vitro replication of bovine gammaherpesvirus type 4.

In vitro cell cultures are widely used models for dissecting cellular and molecular mechanisms that lead to certain physiological conditions and diseases. The pathogenesis of BoHV-4 in the bovine reproductive tract has been studied by conducting tests on primary cultures. However, many questions remain to be answered about the role of BoHV-4 in endometrial cells. The aim of this study was to compare the replication and gene expression of BoHV-4 in cell lines and bovine reproductive tract primary cells as an in vitro model for the study of this virus. We demonstrated that BoHV-4 strains differ in their in vitro growth kinetics and gene expression but have the same cell type preference. Our results demonstrate that BoHV-4 replicates preferentially in bovine endometrial cells (BEC). However, its replication capacity extends to various cell types, since all cells that were tested were permissive to BoHV-4 infection. The highest virus titers were obtained in BEC cells. Nevertheless, virus replication efficiency could not be fully predicted from the mRNA expression profiles. This implies that there are multiple cell-type-dependent factors and strain properties that determine the level of BoHV-4 replication. The results of this study provide relevant information about the in vitro behavior of two field isolates of BoHV-4 in different cell cultures. These findings may be useful for the design of future in vitro experiments to obtain reliable results not only about the pathogenic role of BoHV-4 in the bovine female reproductive tract but also in the development of efficient antiviral strategies.

Population structure of Squatina guggenheim (Squatiniformes, Squatinidae) from the south-western Atlantic Ocean.

Population genetic analyses based on both mitochondrial cytochrome b and the internal transcribed spacer 2 of recombinant (r)DNA genes were implemented to examine hypotheses of population differentiation in the angular angel shark Squatina guggenheim, one of the four most-widespread endemic species inhabiting coastal ecosystems in the south-western Atlantic Ocean. A total of 82 individuals of S. guggenheim from 10 sampling sites throughout the Río de la Plata mouth, its maritime front, the outer shelf at the subtropical confluence and the coastal areas of the south-west Atlantic Ocean, were included. The analysis of molecular variance (AMOVA) based on the second internal transcribed spacer (its-2) region supports that the samples from the outer shelf represent an isolated group from other sites. Historical gene flow in a coalescent-based approach revealed significant immigration and emigration asymmetry between sampling sites. Based on the low level of genetic diversity, the existence of a long-term population decline or a past recent population expansion following a population bottleneck could be proposed in S. guggenheim. This demographic differentiation suggests a degree of vulnerability to overexploitation in this endemic and endangered south-west Atlantic Ocean shark, given its longevity and low reproductive potential.

Mapping Rph20: a gene conferring adult plant resistance to Puccinia hordei in barley.

A doubled haploid (DH) barley (Hordeum vulgare L.) population of 334 lines (ND24260 × Flagship) genotyped with DArT markers was used to map genes for adult plant resistance (APR) to leaf rust (Puccinia hordei Otth) under field conditions in Australia and Uruguay. The Australian barley cultivar Flagship carries an APR gene (qRphFlag) derived from the cultivar Vada. Association analysis and composite interval mapping identified two genes conferring APR in this DH population. qRphFlag was mapped to the short arm of chromosome 5H (5HS), accounting for 64-85% of the phenotypic variation across four field environments and 56% under controlled environmental conditions (CEC). A second quantitative trait locus (QTL) from ND24260 (qRphND) with smaller effect was mapped to chromosome 6HL. In the absence of qRphFlag, qRphND conferred only a low level of resistance. DH lines displaying the highest level of APR carried both genes. Sequence information for the critical DArT marker bPb-0837 (positioned at 21.2 cM on chromosome 5HS) was used to develop bPb-0837-PCR, a simple PCR-based marker for qRphFlag. The 245 bp fragment for bPb-0837-PCR was detected in a range of barley cultivars known to possess APR, which was consistent with previous tests of allelism, demonstrating that the qRphFlag resistant allele is common in leaf rust resistant cultivars derived from Vada and Emir. qRphFlag has been designated Rph20, the first gene conferring APR to P. hordei to be characterised in barley. The PCR marker will likely be effective in marker-assisted selection for Rph20.

Effect of bovine viral diarrhea virus on subsequent infectivity of bovine gammaherpesvirus 4 in endometrial cells in primary culture: An in vitro model of viral co-infection.

Bovine viral diarrhea virus (BVDV) and bovine gammaherpesvirus 4 (BoHV-4) infect the uterus of cattle, being responsible for huge economic losses. Most of the pathogenesis of BoHV-4 in the bovine reproductive tract has been elucidated by conducting tests on primary cultures. Thus, it is important to have optimal in vitro conditions, avoiding the presence of other pathogens that can alter the results. BVDV is one of the most frequent viral contaminants of cell cultures. Considering that non-cytopathic (NCP) BVDV biotype can generate persistently infected (PI) cattle, which are the major source for virus transmission in susceptible herds, it is important to check products derived from cattle that are intended to be used in research laboratories. The aim of this work was to evaluate how the natural infection of bovine endometrial cells (BEC) with a NCP BVDV strain (BEC + BVDV) affects BoHV-4 replication. We have demonstrated a delay in BoHV-4 gene expression and a decrease in viral load in the extracellular environment in BEC + BDVD cells compared to BEC (BVDV-free) cells. These results confirm that replication of BoHV-4 in BEC primary cultures is affected by previous infection with BVDV. This finding highlights the importance of ruling out BVDV infection in bovine primary cell cultures to avoid biological interference or misinterpretation of results at the time of performing in vitro studies with BoHV-4.

Colonization of the Residues of Diverse Plant Species by Gibberella zeae and Their Contribution to Fusarium Head Blight Inoculum.

The presence of Fusarium spp. was examined in the residues of wheat, barley, corn, sunflower, pasture, and gramineous weed species common in wheat and barley cropping systems collected from no-tillage and reduced-tillage plots from February 2001 to March 2003 in Uruguay. Gibberella zeae was recovered from residues of wheat, barley, corn, sunflower, fescue, and the gramineous weeds Digitaria sanguinalis, Setaria spp., Lolium multiflorum, and Cynodon dactylon, except from birdsfoot trefoil or white clover. Of the Fusarium spp. obtained, G. zeae was the most frequently recovered from wheat and barley residues, while other species were more common in other crops. G. zeae declined over time in all residues examined. Wheat and barley residues produced more ascospores of G. zeae than corn or other gramineous residues. Sunflower residue did not support ascospore production, indicating that it probably did not contribute to primary inoculum. Wheat and barley residues supported G. zeae colonization longer in no-till than in reduced-tillage production systems and, thus, may represent major contributors to Fusarium head blight (FHB) inoculum in Uruguay. The presence of G. zeae in the gramineous components of pastures, weed species, and sunflower should be considered when implementing control strategies for FHB.

[Neuropsychological study of 12 patients with pure degenerative cerebellar disease]. / Evaluación cognitiva en 12 pacientes con enfermedad cerebelosa degenerativa pura.

INTRODUCTION: The cerebellum has been traditionally associated with motor control learning and performance. However, since 1970 a growing body of clinical and experimental evidences has suggested that the cerebellum may be involved in nonmotor cognitive functions as well. OBJECTIVE: To explore the presence of eventual cognitive impairment in non-demented patients with isolated degenerative cerebellar diseases. PATIENTS AND METHODS: Twelve patients with the diagnosis of selective degenerative cerebellar disorders, either inherited or sporadic, were selected (mean age: 40.42 +/- 13.49 years; mean education level: 9.92 +/- 3.99 years; duration of illness: 12.13 +/- 11.27 years, MMSE: 26.75 +/- 1.5) and evaluated through a standardized neuropsychological tests battery. Normalized Z scores were estimated and compared against 0, employing the t test for one sample. RESULTS: Significant cognitive deficits were found in the following domains: executive, visuo-spatial, memory and attention functions. Performance on the Wisconsin test showed a significative number of perseverative errors. Memory deficits included verbal learning and free recall difficulties, with good recognition of the material presented. CONCLUSIONS: The findings of this study are consistent with the role of the cerebellum as modulator of mental functions. The cognitive deficits resulting from cerebellar pathology may be related with the disruption of cerebello-cortical connexions involving a complex network which includes the prefrontal region, suggesting that the cerebellum may process cortical information coming from different areas linked with the control of cognition.

Epidemic history of major genotypes of hepatitis C virus in Uruguay.

Worldwide, more than 170 million people are chronically infected with the hepatitis C virus (HCV) and every year die more than 350,000 people from HCV-related liver diseases. Recently, HCV was reclassified into seven major genotypes and 67 subtypes. Some subtypes as 1a, 1b and 3a, have become epidemic as a result of the new parenteral transmission routes and are responsible for most HCV infections in Western countries. HCV 1a subtype have been sub-categorized into two separate sub clades. Recent studies based on the analysis of NS5B genome region, reveal that HCV epidemics in Argentina and Brazil are characterized by multiple introductions events of subtypes 1a, 1b and 3a, followed by subsequent local dispersion. There is no data about HCV genotypes circulating in Uruguay and their evolutionary and demographic history. To this end, a total of 153 HCV NS5B gene sequences were obtained from Uruguayan patients between 2005 and 2011. 86 (56%) sequences grouped with subtype 1a, 40 (26%) with subtype 3a and 27 (18%) with subtype 1b. Furthermore, subtype 1a sequences were distributed among both clades, 1 (n=62, 72%) and 2 (n=24, 28%). Four local HCV clades were found: UY-1a(I), UY-1a(II), UY-1a(III) and UY-3a; comprising a 39% of all HCV viruses analyzed in this study. HCV epidemic in Uruguay has been driving by multiple introductions of subtypes 1a, 1b and 3a and by local dissemination of a few country-specific strains. The evolutionary and demographic history of the major Uruguayan HCV clade UY-1a(I) was reconstructed under two different molecular clock rate models and displayed an epidemic history characterized by an initial phase of rapid expansion followed by a more recent reduction of growth rate since 2000-2005. This is the first comprehensive study about the molecular epidemiology and epidemic history of HCV in Uruguay.

First Report of Spot Type of Barley Net Blotch Caused by Pyrenophora teres f. sp. maculata in Uruguay.

In September 2003, leaves exhibiting spot-type lesions similar to those produced by Cochliobolus sativus Drechs. ex Dastur were widely observed in six commercial barley crops of cvs. Norteña Daymán, Norteña Carumbé, and MUSA 936 in Soriano and Río Negro provinces, the main barley production region in western Uruguay. Spot lesions were tan to dark brown, circular to elliptical, and 3 to 10 mm in diameter. Larger lesions were surrounded by a chlorotic margin of varying width. Affected leaf pieces (10 to 15) from each field were placed in a moist chamber for 2 days to promote sporulation. A fungus identified morphologically as Pyrenophora teres (Died.) Drechs. (1) was consistently isolated from infected leaves. However, symptoms did not correspond to the net-type lesions of net blotch commonly produced by P. teres f. sp. teres in Uruguay. Three monoconidial cultures were obtained by transferring single conidia to potato dextrose agar and then to 10% V8 juice agar and incubated at 20 to 22°C with a 12-h photoperiod for 10 days. Adding sterile water to each plate and gently rubbing the surface with a microscope slide prepared inoculum for pathogenicity tests. Conidia concentration was adjusted to 1 × 104 conidia per ml. Sixty-eight barley genotypes from Uruguay, ICARDA/CIMMYT, and North Dakota were grown in the greenhouse for 2 weeks at 20 to 22°C with a 14-h photoperiod. For each monoconidial isolate, three seedlings of each genotype were inoculated at the three-leaf stage 15 to 16 days after seeding with 0.4 ml of the inoculum suspension with an airbrush inoculator. A drop of Tween 20 was added per 40 ml of inoculum suspension. One set of each genotype was inoculated with sterile water as a control. Seedlings were placed in a dew chamber at 20°C and 100% relative humidity in the dark for 24 h and then returned to prior conditions. The first lesions developed after 7 to 9 days. Leaves two and three of the plants were visually rated for disease (3) 13 days after inoculation. Control plants were disease free. The most susceptible reactions were observed on cvs. Norteña Daymán, MUSA 936, and line CLE 230 (Uruguay). Symptoms were similar in shape and size to those observed in the fields. The most resistant infection types were observed on several Uruguayan and North Dakota advanced lines. The fungus was consistently reisolated from inoculated plants. On the basis of morphology and symptoms produced, the pathogen was identified (2) as P. teres. f. sp. maculata Smedeg. To our knowledge, this is the first report of this fungus causing spot-like symptoms of net blotch in Uruguay. References: (1) M. B. Ellis. Dematiaceous hyphomycetes, CABI, Oxon, UK, 1971. (2) V. Smedergaard-Petersen. Pages 124-144 in: R. Vet. Agr. Univ. Yearbook, Copenhagen, 1971. (3) A. Tekauz. Can. J. Plant Pathol.7:181, 1985.

Survival and Inoculum Production of Gibberella zeae in Wheat Residue.

Survival and inoculum production of Gibberella zeae (Schwein.) Petch (anamorph Fusarium graminearum (Schwabe)), the causal agent of Fusarium head blight of wheat and barley, was related to the rate of wheat (Triticum aestivum L.) residue decomposition. Infested wheat residue, comprising intact nodes, internodes, and leaf sheaths, was placed in fiberglass mesh bags on the soil surface and at 7.5- to 10-cm and 15- to 20-cm depths in chisel-plowed plots and 15 to 20 cm deep in moldboard-plowed plots in October 1997. Residue was sampled monthly from April through November during 1998 and every 2 months through April to October 1999. Buried residue decomposed faster than residue placed on the soil surface. Less than 2% of the dry-matter residue remained in buried treatments after 24 months in the field, while 25% of the residue remained in the soil-surface treatment. Survival of G. zeae on node tissues was inversely related to the residue decomposition rate. Surface residue provided a substrate for G. zeae for a longer period of time than buried residue. Twenty-four months after the initiation of the trial, the level of colonization of nodes in buried residue was half the level of colonization of residue on the soil surface. Colonization of node tissues by G. zeae decreased over time, but increased for other Fusarium spp. Ascospores of G. zeae were still produced on residue pieces after 23 months, and these spores were capable of inducing disease. Data from this research may assist in developing effective management strategies for residues infested with G. zeae.

Loss of motor units in Alzheimer's disease.

Little attention has been paid in the literature to the state of the peripheral nervous system (PNS) in Alzheimer's disease (AD). We conducted a comprehensive electrophysiological study in 15 AD patients looking for functional abnormalities within their PNSs. A reduction of the number of functioning motor units (MU) was found in the thenar and soleus muscles of most of these patients without enlargement of the remaining MUs territories, while the motor and sensory conduction velocities of the peripheral nerves were preserved. These results suggest dysfunction of the spinal motoneurones in patients afflicted with this condition.

Cognitive impairment in human chronic Chagas' disease.

UNLABELLED: We proposed to investigate subclinical cognitive impairment secondary to chronic Chagas' disease (CCD). No similar study was previously done. The neuropsychological performance of 45 chronic Chagasic patients and 26 matched controls (age, education place and years of residency in endemic area) was compared using the Mini Mental State Exam (MMSE), Weschler Memory Scale (WMS) and the Weschler Adult Intelligent Scale (WAIS). Non-parametric tests and Chi2 were used to compare group means and multivariate statistics in two way frequency tables for measures of independence and association of categorical variables with the disease. RESULTS: Chagasic patients showed lower MMSE scores (p < .004), poor orientation (p < .004), and attention (p < .007). Lower WMS MQ were associated with CCD (Chi2 5.9; p < .01; Fisher test p < .02). Lower WAIS IQ were associated with CCD (Chi2 6.3, p < .01; Fisher test p < .01) being the digit symbol (p < .03), picture completion (p < .03), picture arrangement (p < .01) and object assembly (p < .03) subtests the most affected. The impairment in non-verbal reasoning, speed of information processing, problem solving, learning and sequencing observed in chronic Chagas disease patients resembles the cognitive dysfunction associated with white matter disease.

Pénfigo vulgar refractario a tratamientos convencionales, con respuesta positiva a Rituximab: presentación de 5 casos / Positive Outcome in Treatment of Patients with Pemphigus Vulgaris Refractory to Conventional Treatments: A Report of Five Cases

Resumen El pénfigo vulgar es una enfermedad ampollar crónica de etiología autoinmune, en la cual el diagnóstico y tratamiento precoz disminuye la morbimortalidad de los pacientes. Actualmente se utilizan corticosteroides de primera línea asociados a inmunosupresores. Sin embargo,se ha visto que el uso de Rituximab como terapéutica de primera elección en los países desarrollados ha disminuido las reacciones adversas, con lo que mejora la calidad de vida de los pacientes. Se presentan cinco pacientes con diagnóstico de pénfigo vulgar refractario a tratamientos convencionalesque requirieron tratamiento con Rituximab.

Abstract Pemphigus vulgaris is an autoimmune chronic blistering disease. Its early diagnosis and treatment help reduce patient mortality and morbidity. Corticosteroids associated with immunosuppressants remain the standard treatment for pemphigus vulgaris. However, the use of Rituximab as first-line therapy in developed countries has been effective in reducing adverse effects improving the quality of life of patients. We report five patients with a diagnosis of pemphigus vulgaris refractory to conventional treatments requiring treatment with Rituximab.

Regional differences in species composition and toxigenic potential among Fusarium head blight isolates from Uruguay indicate a risk of nivalenol contamination in new wheat production areas.

Members of the Fusarium graminearum species complex (FGSC) are the primary cause of Fusarium head blight (FHB) of wheat, and frequently contaminate grain with trichothecene mycotoxins that pose a serious threat to food safety and animal health. The species identity and trichothecene toxin potential of 151 FGSC isolates collected from wheat in Uruguay were determined via multilocus genotyping. Although F. graminearum with the 15ADON trichothecene type accounted for 86% of the isolates examined, five different FGSC species and all three trichothecene types were identified in this collection. This is the first report of Fusarium asiaticum, Fusarium brasilicum, Fusarium cortaderiae, and Fusarium austroamericanum from Uruguay. In addition, we observed significant (P<0.001) regional differences in the composition of FGSC species and trichothecene types within Uruguay. Isolates of F. graminearum with the 15ADON type were the most prevalent in western provinces (95%), while F. asiaticum (43%) and the NIV type (61%) predominated in the new wheat production zone in Cerro Largo along Uruguay's eastern border with Brazil. F. graminearum isolates (15ADON type) were significantly (P<0.005) more aggressive on wheat than were isolates from the other species examined (NIV or 3ADON types). However, F. graminearum isolates (15ADON type) were significantly (P<0.05) more sensitive to tebuconazole than isolates from other species (NIV type). These results document substantial heterogeneity among the pathogens responsible for FHB in Uruguay. In addition, the regional predominance of the NIV trichothecene type is of significant concern to food safety and indicates that additional monitoring of nivalenol levels in grain may be required.

Genomic analysis of bovine herpesvirus type 4 (BoHV-4) from Argentina: high genetic variability and novel phylogenetic groups.

Bovine herpesvirus 4 (BoHV-4) is a γ-herpesvirus that has been isolated both from apparently healthy animals and from cattle with a variety of clinical signs, including post-partum endometritis and abortion. BoHV-4 causes either a persistent or a latent infection in cells of the monocyte/macrophage lineage. Two groups of BoVH-4 strains have been defined based on their restriction patterns: the Movar-like strains (European prototype) and the DN 599-like strains (American prototype). The purpose of the present study was to genetically characterize wild type BoHV-4 strains isolated from vaginal discharges of aborted cows in Argentina. The virus was identified by isolation and nested PCR in all vaginal discharge samples from aborted cows, either as a sole agent or in association with other pathogens. Restriction enzyme profiling and phylogenetic analysis demonstrated that there is a high genetic variability among the studied field isolates. The existence of three groups of strains, which were designated as genotypes 1, 2 and 3, is described. Genotypes 1 and 2 possibly correspond to the Movar-like and DN 599-like groups, respectively, whereas Genotype 3 corresponds to a novel group. Two viral strains did not cluster into any of these three groups, indicating that other genotypes could be circulating in Argentina. These results suggest a complex epidemiological background for the Argentinean BoHV-4 strains, probably influenced by independent events of genetic drift. This hypothesis cannot be rejected based on the available data. However, there is no direct evidence supporting this possibility. Thus, it seems speculative to suggest that interspecific jumps are responsible for the observed phylogenetic grouping. On the other hand, our analyses suggest a geographical structure for the observed viral genotypes, since genotypes 1 and 2 included the European (Movar-like) and American (DN599-like) reference strains, respectively. Geographic dispersion is known to be a driver of herpes viruses diversification, and independent evolution in geographical isolated places ensures the emergence of particular mutations in each location due to genetic drift (Compans, 2007; Zong et al., 1999). Therefore, at this point, the genetic drift hypothesis is the one that requires less ad-hoc considerations and thus, to our understanding, is the one that fits to the findings from this study. The involvement of this genetic variability in the detection and pathogenesis of BoHV-4 remains to be investigated.

Immune response to Neospora caninum native antigens formulated with immune stimulating complexes in calves.

The aim of this study was to compare the immune responses to live Neospora caninum tachyzoites and N. caninum native antigens formulated with immune stimulating complexes matrix (ISCOM-matrix) in calves. Fifteen calves were used in this study: 3 were intravenously inoculated with 1 × 10(8) live tachyzoites (Group A), 3 were inoculated twice with N. caninum native antigens formulated with ISCOMs (Group B); 3 with N. caninum native antigens in phosphate-buffered saline (PBS) (Group C); 3 received ISCOM-matrix (ISCOMs without antigen) (Group D) and 3 were negative controls receiving PBS (Group E). The last four groups were inoculated subcutaneously. The specific total IgG and its subtypes were analyzed by an indirect enzyme-linked immunosorbent assays (ELISAs) and by Western blot. IFN-γ levels in plasma was quantified using a commercial kit. All calves were challenged intravenously with 1 × 10(8) live tachyzoites at week 11 after receiving the first dose. Parasitemia was assessed in plasma samples by semi-nested PCR. Neospora-specific antibodies were detected in animals from Groups A and B in the week 2 after inoculation. The ELISA OD values were higher in Group B compared with Group A from weeks 6 to 11 (P<0.05). Analysis of the subisotype specific antibodies in experimentally infected calves revealed a predominant IgG(2) response; however, a predominant IgG(1) response was observed in animals inoculated with N. caninum native antigens formulated with ISCOM-matrix. Control calves remained seronegative until challenge infection. The pattern of bands by Western blot was similar when testing sera from animals in Groups A and B. The levels of IFN-γ production after respective immunization schedules were similar between Groups A and B. Neospora-DNA was detected in plasma samples shortly after intravenous challenge in calves from all groups including those receiving the experimental vaccine formulation. The duration of the parasitemia was similar in all groups.

Influence of feelings of burden on the caregiver's perception of the patient's functional status.

We studied the correlation between the caregiver's feelings of burden and the cognitive, behavioral and functional impairment of demented patients. We attempted to show the influence of caregiver's feelings of burden on their perception of the patients' functional status and to establish the predictors of feelings of burden. Twenty-five probable Alzheimer disease patients (NINCDS-ADRDA criteria) were assessed on cognitive measures and functional status (DAFS). The caregiver's index of burden (CIB), obtained from an adapted version of the Zarit Burden Interview, was correlated with the caregiver's report on the patient's instrumental activities of daily living (IADL) and physical self-maintenance functions (ADL); total and partial DAFS scores, and patient's cognitive and behavioral problems rated with the Functional Dementia Scale (FDS). The CIB correlated with the caregiver's report on the patient's behavioral disturbances (r = 0.71, p < 0.001) and physical self-maintenance activities (r = 0.62, p < 0.001), but not with cognitive impairment, IADL and the DAFS. The patients showed better functional performance in the direct assessment than in that reported by their caregivers (mainly in shopping, feeding, dressing, and ambulation). Thus, burden may foster a growing intolerance of the caregiver, inducing an underestimation of the patient's actual functional competence. The caregiver's report on the patient's behavioral problems was the best predictor of feelings of burden (P = 19,9, p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Production of pectinases by Aspergillus niger in solid state fermentation at high initial glucose concentrations.

Exopectinase (exo-p) and endopectinase (endo-p) production by Aspergillus niger CH4 in solid state culture was studied at initial glucose concentrations of 100, 250, 350 and 450 g/l. The highest activity of exo-p (35 U/g) was produced at 72 and 120 h in the medium containing 100 and 250 g glucose/l, respectively. The maximum endo-p activity (9 U/g) was produced at 72 h in the medium with 250 g glucose/l. The reduction in pectinase production at 350 and 450 g/l initial glucose concentration was due neither to repression of the synthesis of the enzyme nor to the glucose consumption rate of the strain but due to a drastic drop in pH of the medium.

Gonadoblastoma bilateral en una niña con síndrome de Turner: reporte de un caso / Bilateral gonadoblastoma in a Turner syndrome girl: a case

Objetivo: Presentar un caso de gonadoblasgotoma bilateral y su manejo en una niña portadora de Síndrome de Tumer con antígeno SRY positivo Material y métodos: Niña de 6 años con el Síndrome de Tumer en cuyo cariotipo se encuentra un mosaicismo 45 XI SRY, con antígeno SRY positivo por lo que realiza estudio para descartar o confirmar la presencia de Gonadoblastoma consistente en Biopsia por laparoscopia de ambas gónadas. Resultados: Se encontró al estudio anatomo-patológico Gonadoblastoma bilateral, realizándose posteriormente gonadectomia bilateral, cuya evolución fue favorable. El Gonadoblastoma es un raro tumor compuesto por una combinación de células germinales y cor- dones sexuales-estroma que afecta exclusivamente a pacientes con disgenesia gonadal, siendo mas frecuente en niñas y adultas jóvenes fenotipicamente femeninas, que usual- mente tienen signos de virilización. Su . cuencia en gónadas disgenéticas es estimado en un 30 por ciento. Conclusión: Se recomienda la gonadecto-mía profiláctica en los casos de pacientescon síndrome Tumer con antígeno SRY positivo.

Objectíve: To present a case of bilateral nadoblastoma and its management in a Tumer Síndrome girl with positive SRY antigen. Material and methods: A six-year-old girl who was diagnosed as Tumer Syndrome and her karyotype was 45 XI 46 XY, with positive antigen, then she was performed a 46 XY laparoscopic biopsy looking for gonadoblastoma. Results: We found histopathologic evidenences of bilateral Gonadoblastoma, and weperformed bilateral gonadectomy, and postoperative recovery was uneventful. Gonadoblastoma is an uncommon tumor which has germ cells, which affects to patients who were diagnosed as gonadal dysgenesis, it is more I frequent in children and young women phenotypically female, who used to have virilism signs. Its frequency in dysgenetic gonads is calculated in 30 percent. Conclusion: We suggest prophylactic gonadectomy in patients with Tumer Syndrome and evidence of y chromosome material.

Administración de vacunas y casos de muerte súbita del lactante en el Perú, 2001. ¿Asociación o coincidencia temporal? / Immunization and sudden death in infants in Peru, 2001. Is it an association or a temporary coincidence?

Objetivos: Describir las características clínicas, socioeconómicas, y patológicas de nueve casos de lactantes que fallecieron horas después de administrárseles vacunas antipolio y DPT junto con anti Haemophilus influenzae b o asociada con antihepatitis B o BCG. Materiales y métodos: Revisión de la historia clínica, entrevista con el equipo de salud a cargo de la vacunación y con los padres del lactante fallecido. Revisión de los informes del protocolo de autopsia e informes de anatomía patológica y entrevista con los médicos legistas y patólogos. Evaluación de controlde calidad de las vacunas administradas. Realización de exámenes de inmunohistoquímica de tejidos pulmonarespara el diagnóstico de virus. Resultados: Entre agosto y diciembre de 2001 se notificaron en el Perú, nueve casos de fallecimiento de lactantes entre ocho horas y tres días después de la aplicación de vacunas DPT y antipolio. Después de la vacunación, los síntomas iniciaron entre 30 minutos a tres horas y la muerte se produjo entre ocho a 78 horas. Los síntomas más frecuentes fueron irritabilidad 8/9, llanto persistente 6/9, somnolencia 5/9, sangrado por nariz y boca5/9. Todos los casos procedieron de familias pobres o muy pobres. El control de calidad se corroboró que las vacunas cumplieron con los estándares establecidos por la OMS. Las causas de la muerte reportadas en las necropsias fueron neumonía en dos casos y edema pulmonar en cinco casos, los informes de anatomía patológica mostraron una neumonitis intersticial y meningitis linfocitaria. No se observaron efectos citopáticos virales en los tejidos pulmonares y los estudios toxicológicos fueron negativos. Conclusiones: No existe evidencia de una asociación causal entre los eventosfatales y la administración de las vacunas.

Objectives: To describe clinical, socio-economic, and pathological characteristics of nine infants who died hours after receiving anti-polio and DPT vaccines, together with anti-Haemophilus influenzae b or in association to anti-hepatitis B vaccine or BCG. Materials and methods: Review of the clinical record, interview with the healthcare team in charge of immunization, and with parents of the deceased infant. Review the autopsy and pathology reports, as well as an interview with pathologists and forensic physicians. Quality control assessment of vaccines used immunohistochemistry examinations for virus diagnosis. Results: Between August and December 2001, nine cases of infant death were reported in Peru, between 8 hours and 3 days after receiving DPT and anti-polio vaccines. After immunization, symptoms appeared from thirty minutes to three hours, and death occurred between 8 to 78 hours. Most frequently reported symptoms were: irritability (8/9), persistent weeping (6/9), somnolence (5/9), nose and mouth bleeding (5/9). All cases occurred in poor or very poor families. Quality control assessments corroborated that vaccines complied with standards set by the World Health Organization. Causes of death reported in autopsies were pneumonia in 2 cases and pulmonary edema in 5 cases; pathology reports indicated the presence of interstitial pneumonitis and lymphocyte meningitis. No viral cytopathic effects in pulmonary tissues were found, and toxicological studies were reported as negative. Conclusions: There is no evidence of a causal relationship between fatal events and vaccine administration.