Domestic cat embryos generated without zona pellucida are capable of developing in vitro but exhibit abnormal gene expression and a decreased implantation rate.

The removal of the zona pellucida has been used to improve the in vitro development of domestic cat embryos generated by IVF and SCNT. However, the in vivo development of domestic cat embryos generated without the zona pellucida has not been evaluated. The objective of this study was to evaluate the effects of zona pellucida removal on the in vitro and in vivo development of domestic cat embryos generated by IVF. For this purpose, two experimental groups were created: 1) domestic cat embryos cultured in vitro (Zona-intact group, ZI) and 2) domestic cat embryos cultured in vitro without the zona pellucida (Zona-free group, ZF). Domestic cat embryos were generated by IVF and cultured in vitro for 8 days. In the ZF group, the zona pellucida was removed after IVF, and embryos were cultured using the well of the well system (WOW). Cleavage, morula and blastocyst rates were evaluated in both groups. The diameter and total cell number of blastocysts were assessed. Relative expression of pluripotency (OCT4, SOX2 and NANOG), differentiation (CDX2 and GATA6) and apoptotic markers (BAX and BCL2) was evaluated in blastocysts. Finally, to evaluate in vivo development, embryos at days 5, 6 and 7 of development were transferred into recipient domestic cats, and ultrasonography was performed to evaluate implantation. No differences were observed in the cleavage, morula or blastocyst rates between embryos from the ZI and ZF groups. The diameter (mean ± SD) of blastocysts from the ZF group was greater (253.4 ± 83.3 µm) than that from the ZI group (210.5 ± 78.5 µm). No differences were observed in the relative expression of OCT4, CDX2 or GATA6. However, the relative expression of SOX2 and NANOG was significantly reduced in ZF blastocysts compared to ZI blastocysts. Furthermore, the relative expression of BAX was higher in ZF blastocysts than in ZI blastocysts. Finally, four pregnancies were confirmed after the transfer of ZI embryos (n = 110). However, no pregnancies were observed after the transfer of ZF embryos at the morula or blastocyst stage (n = 56). In conclusion, domestic cat embryos cultured without the zona pellucida were able to develop in vitro until the blastocyst stage. However, the removal of the zona pellucida negatively affected the gene expression of pluripotency and apoptosis markers, and ZF embryos were unable to implant. This might indicate that the removal of the zona pellucida is detrimental for the implantation and in vivo development of domestic cat embryos.

Clinical Practice Guidelines for diagnosis of amyloidosis: Part 1/3 Year 2020 / [Guía de Práctica Clínica para el diagnóstico de la amiloidosis: Parte 1/3. Año 2020]

Method: Use the PICO format to generate a series of questions, focusing on the specificity and sensitivity of the amyloidosis diagnostic test. PubMed searches were conducted in English and Spanish from July to August 2019. The level of evidence and recommendation are based on the GRADE system (http://www.gradeworkinggroup.org/index.htm). The recommendations are graded according to their direction (for or against) and strength (strong and weak). Finally, it is recommended to use GLIA tools to evaluate the obstacles and facilitators in implementation. Suggested explanation: A strong suggestion indicates a high degree of trust in support or opposition to the intervention. When defining a strong recommendation, this guide uses the "recommended" language. The weaker recommendations indicate that the outcome of the intervention (favorable or unfavorable) is doubtful. In this case, if a weak recommendation is defined, the "recommendation" language is used. How to use these guidelines: Recommendations must be explained within the scope of special care in validated diagnostic studies conducted by specially trained doctors. It is not assumed to change the coexistence conditions of the disease process. Presumably, the attending physician has a high degree of suspicion of amyloidosis. It assumes that diagnostic research is conducted by well-trained doctors using a validated standardized method. This guide is intended for health care professionals and those involved in health care policies to help ensure that the necessary agreements have been reached to provide appropriate care. Recommendations: For patients with suspected amyloidosis, it is recommended; Confirmation in the tissue by biopsy and Congo red staining with the characteristic green birefringence under polarized light is recommended.Confirmation by electron microscopy of the biopsy tissue is recommended. Protein typing by mass spectrometry is recommended. Protein typing by optical and / or electronic immunomicroscopy is recommended, as long as there are reliable antibodies. Measurement of serum free light chains is recommended for evaluation of a monoclonal plasma cell proliferative disorder. Serum and urinary immunofixation is recommended for evaluation of a monoclonal plasma cell proliferative disorder. Measurement of serum free light chains, plus serum and urinary immunofixation is recommended for the evaluation of a monoclonal plasma cell proliferative disorder. For patients suspected of having amyloidosis, it is suggested; Demonstration of a monoclonal plasma cell proliferative disorder by demonstration of clonal plasma cells by the most sensitive technique available in the bone marrow for the diagnosis of AL-type amyloidosis. Confirmation of ATTRv amyloidosis by DNA sequencing of the 4-exon amyloidogenic TTR gene in patients with suspected ATTRv amyloidosis.

Métodos: Se generó un listado de preguntas con el formato PICO centradas en la especificidad y sensibilidad de las pruebas diagnósticas en amiloidosis. Se realizó la búsqueda en PubMed durante julio-agosto del 2019, en inglés y español. Los niveles de evidencia y los grados de recomendación se basan en el sistema GRADE (http://www.gradeworkinggroup.org/index.htm). Las recomendaciones se graduaron según su dirección (a favor o en contra) y según fuerza (fuertes y débiles). Las recomendaciones finales fueron evaluadas con la herramienta GLIA para barreras y facilitadores en la implementación de éstas. Interpretación de recomendaciones: Las recomendaciones fuertes indican alta confianza, ya sea a favor o en contra, de una intervención. En esta guía se utiliza el lenguaje "se recomienda" cuando se define una recomendación fuerte. Las recomendaciones débiles indican que los resultados para una intervención, favorable o desfavorable, son dudosos. En este caso, se utiliza el lenguaje "se sugiere", cuando se define una recomendación débil. Como utilizar estas pautas: Las recomendaciones deben ser interpretadas en el contexto de la atención especializada, con estudios diagnósticos validados y realizados por médicos entrenados. No asume condiciones coexistentes que modificaran el curso de la enfermedad. Se asume que el médico tratante tiene alto nivel de sospecha de amiloidosis. Asume que los estudios diagnósticos son realizados por médicos entrenados con métodos validados y estandarizados. Esta guía es relevante para los profesionales de la salud y los involucrados en las políticas sanitarias, para ayudar a asegurar que existan los acuerdos necesarios para brindar la atención adecuada. Recomendaciones En pacientes con sospecha de amiloidosis se recomienda: La confirmación en el tejido mediante biopsia y tinción con rojo Congo con la característica birrefringencia verde bajo luz polarizada. La confirmación mediante microscopía electrónica en el tejido de biopsia. La tipificación de la proteína mediante espectrometría de masa. La tipificación de la proteína mediante inmunomicroscopía óptica y/o electrónica, en la medida que haya anticuerpos confiables. La medición de las cadenas livianas libres séricas para evaluación de un trastorno proliferativo de células plasmáticas monoclonales. La Inmunofijación sérica y urinaria para la evaluación de un trastorno proliferativo de células plasmáticas monoclonales. La medición de las cadenas livianas libres sérica, más la Inmunofijación sérica y urinaria para la evaluación de un trastorno proliferativo de células plasmáticas monoclonales. En pacientes con sospecha de amiloidosis se sugiere: Demostración de un trastorno proliferativo de células plasmáticas monoclonales mediante la demostración de plasmocitos clonales por la técnica más sensible disponible en la médula ósea para el diagnóstico de amiloidosis de tipo AL. La confirmación de amiloidosis ATTRv mediante secuenciación de ADN del gen TTR amiloidogénico de los 4 exones en pacientes con sospecha de amiloidosis por ATTRv.

The effect of body dissatisfaction on disordered eating: The mediating role of self-esteem and negative affect in male and female adolescents.

This study aimed to determine whether self-esteem and negative affect sequentially mediate the relationship between body dissatisfaction and disordered eating. A total of 806 adolescents (61.8% females) completed the Drive for Thinness, Bulimia, and Body Dissatisfaction subscales of the Eating Disorder Inventory-2, the Anxiety and Depression subscales of the General Health Questionnaire-28, and the Negative Self-beliefs subscale of the Eating Disorder Belief Questionnaire. Mediational analyses showed that body dissatisfaction had both direct and indirect effects through self-esteem and negative affect on disordered eating. It was also observed that negative self-esteem mediated-completely in boys and partially in girls-the relationship between body dissatisfaction and negative affect.

[AA amyloidosis. A single institution cohort study]. / Amiloidosis AA. Estudio de cohorte en una institución.

There is limited epidemiological information on AA amyloidosis in Argentina, so the objective of this study was to describe the epidemiological characteristics of this disease in a tertiary hospital in our country. We designed a prospective clinical cohort of all consecutive patients with AA amyloidosis confirmed by immunohistochemistry in tissue from the Institutional Registry of Amyloidosis of the Hospital Italiano de Buenos Aires, in the period 04/01/2012- 12/31/2017. Of the 121 patients in the registry, 18 were included with AA for the analysis. Of the total included, 50% (9) were female, with a median age of 53.5 (interquartile range, RII 46-61) years. The 88.9% (16) of cohort presented renal compromise, all had proteinuria, and 6 required dialysis. Six had amyloid infiltration of the digestive system. The latency between the onset of the underlying disease and the diagnosis of AA had a median of 27 (RII 8-35) years. The underlying disease was of inflammatory origin in 6 cases. In 50% (9) of the patients the cause of AA amyloidosis was unknown. In the remaining 50%, these causes resemble those observed in developed countries. Furthermore, our results highlight the importance of their differential diagnosis to identify the most appropriate treatment or follow-up according to the situation presented by each patient.

La amiloidosis AA causa principalmente disfunción renal, lo que lleva a un elevado riesgo de mortalidad a mediano plazo. Existe escasa información epidemiológica sobre la amiloidosis AA en Argentina, por lo que el objetivo de este trabajo fue describir las características epidemiológicas de esta enfermedad en un hospital de tercer nivel en nuestro país. Se realizó una cohorte prospectiva de todos los pacientes consecutivos con evidencia de amiloidosis AA, por inmunohistoquímica de tejidos, incluidos en el Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, desde el 01/04/2012 hasta el 31/12/2017. De los 121 pacientes del registro, se incluyeron 18 con AA para el análisis. Del total incluido, 50% (9) eran mujeres, con una mediana de edad de 53.5 (rango intercuartil, RII 46-61) años. El 88.9% (16) presentó compromiso renal, todos tuvieron proteinuria, y 6 requirieron diálisis. Seis tuvieron infiltración amiloide del aparato digestivo. La latencia entre la aparición de la enfermedad subyacente y el diagnóstico de AA tuvo una mediana de 27 (RII 8-35) años. La enfermedad subyacente fue de origen inflamatorio en 6 casos. En el 50% (9) de los enfermos la causa de amiloidosis AA fue desconocida. En el restante 50% esas causas se asemejan a las de países desarrollados. A su vez, nuestros resultados resaltan la importancia de su diagnóstico diferencial para identificar el tratamiento o seguimiento más adecuado según el cuadro que presente cada paciente.

Amiloidosis AA: Estudio de cohorte en una institución / AA amyloidosis. A single institution cohort study

La amiloidosis AA causa principalmente disfunción renal, lo que lleva a un elevado riesgo de mortalidad a mediano plazo. Existe escasa información epidemiológica sobre la amiloidosis AA en Argentina, por lo que el objetivo de este trabajo fue describir las características epidemiológicas de esta enfermedad en un hospital de tercer nivel en nuestro país. Se realizó una cohorte prospectiva de todos los pacientes consecutivos con evidencia de amiloidosis AA, por inmunohistoquímica de tejidos, incluidos en el Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, desde el 01/04/2012 hasta el 31/12/2017. De los 121 pacientes del registro, se incluyeron 18 con AA para el análisis. Del total incluido, 50% (9) eran mujeres, con una mediana de edad de 53.5 (rango intercuartil, RII 46-61) años. El 88.9% (16) presentó compromiso renal, todos tuvieron proteinuria, y 6 requirieron diálisis. Seis tuvieron infiltración amiloide del aparato digestivo. La latencia entre la aparición de la enfermedad subyacente y el diagnóstico de AA tuvo una mediana de 27 (RII 8-35) años. La enfermedad subyacente fue de origen inflamatorio en 6 casos. En el 50% (9) de los enfermos la causa de amiloidosis AA fue desconocida. En el restante 50% esas causas se asemejan a las de países desarrollados. A su vez, nuestros resultados resaltan la importancia de su diagnóstico diferencial para identificar el tratamiento o seguimiento más adecuado según el cuadro que presente cada paciente.

There is limited epidemiological information on AA amyloidosis in Argentina, so the objective of this study was to describe the epidemiological characteristics of this disease in a tertiary hospital in our country. We designed a prospective clinical cohort of all consecutive patients with AA amyloidosis confirmed by immunohistochemistry in tissue from the Institutional Registry of Amyloidosis of the Hospital Italiano de Buenos Aires, in the period 04/01/2012- 12/31/2017. Of the 121 patients in the registry, 18 were included with AA for the analysis. Of the total included, 50% (9) were female, with a median age of 53.5 (interquartile range, RII 46-61) years. The 88.9% (16) of cohort presented renal compromise, all had proteinuria, and 6 required dialysis. Six had amyloid infiltration of the digestive system. The latency between the onset of the underlying disease and the diagnosis of AA had a median of 27 (RII 8-35) years. The underlying disease was of inflammatory origin in 6 cases. In 50% (9) of the patients the cause of AA amyloidosis was unknown. In the remaining 50%, these causes resemble those observed in developed countries. Furthermore, our results highlight the importance of their differential diagnosis to identify the most appropriate treatment or follow-up according to the situation presented by each patient.

[Presentations of transthyretin associated familial amyloid polyneuropathy in Argentina]. / Presentaciones de polineuropatía amiloidótica familiar asociada a transtiretina en la Argentina.

Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) is a hereditary disease with variable geographical distribution. The aim of this study was to present our experience with TTR-FAP patients. We retrospectively analyzed nine cases belonging to different families. Diagnostic criteria were based on the combination of compatible clinical picture, histopathological findings and genetic confirmation. Five cases showed the classic presentation and other 4 the late onset variant. The p.Val30Met mutation in the TTR gene was found in 6 cases and p.Ala36Pro, p.Thr60Ala and p.Tyr114Cys in the remaining 3, respectively. The median age of symptom onset was 35 years (26-60 range). Mean time to diagnosis was 4.2 ± 1.5 years. Our patient series showed the heterogeneity in clinical presentation of TTR-FAP in a non-endemic region of South America.

Presentaciones de polineuropatía amiloidótica familiar asociada a transtiretina en la Argentina / Presentations of transthyretin associated familial amyloid polyneuropathy in Argentina

La polineuropatía amiloidótica familiar asociada a transtiretina (PAF-TTR) es una enfermedad hereditaria con distribución geográfica variable. El objetivo de este trabajo fue presentar nuestra experiencia con pacientes con PAF-TTR. Se evaluaron retrospectivamente 9 casos pertenecientes a diferentes familias. Los criterios diagnósticos utilizados se basaron en la combinación de un cuadro clínico compatible, hallazgos histopatológicos y confirmación genética. Cinco casos mostraron la presentación clásica y 4 la variante de inicio tardío. La mutación p.Val30Met en el gen TTR fue hallada en 6 casos y p.Ala36Pro, p.Thr60Ala y p.Tyr114Cys en los 3 los restantes, respectivamente. La edad media de inicio fue 35 años (rango 26-60). El tiempo medio al diagnóstico fue de 4.2 ± 1.5 años. Siete pacientes recibieron diagnóstico erróneo inicial, 3 de la variante clásica y 4 de la tardía. Nuestra serie de pacientes mostró marcada heterogeneidad en la presentación clínica del grupo de PAF-TTR de inicio tardío, en una región no endémica de Sudamérica.

Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) is a hereditary disease with variable geographical distribution. The aim of this study was to present our experience with TTR-FAP patients. We retrospectively analyzed nine cases belonging to different families. Diagnostic criteria were based on the combination of compatible clinical picture, histopathological findings and genetic confirmation. Five cases showed the classic presentation and other 4 the late onset variant. The p.Val30Met mutation in the TTR gene was found in 6 cases and p.Ala36Pro, p.Thr60Ala and p.Tyr114Cys in the remaining 3, respectively. The median age of symptom onset was 35 years (26-60 range). Mean time to diagnosis was 4.2 ± 1.5 years. Our patient series showed the heterogeneity in clinical presentation of TTR-FAP in a non-endemic region of South America.

Normal-weight and overweight female adolescents with and without extreme weight-control behaviours: Emotional distress and body image concerns.

The objective of this study was to analyse emotional distress and concerns related to body image in 712 normal-weight and overweight adolescent girls. A total of 12.3 per cent of the normal-weight girls and 25 per cent of the overweight girls showed extreme weight-control behaviours. In normal-weight adolescents, their engagement in extreme weight-control behaviours was associated with high levels of somatic symptoms, a drive for thinness and control over eating. In overweight girls, high levels of drive for thinness and anxiety were associated with extreme weight-control behaviours. Finally, the implications for preventive and therapeutic programmes are discussed.

Risky eating behaviors and beliefs among adolescent girls.

This study investigated the prevalence of weight control and binge eating behaviors in a sample of 767 adolescent girls aged 16-20 years, and the differences between adolescents with and without altered eating behaviors regarding anthropometric and body image variables and beliefs associated with eating disorders. Adolescents who engaged in unhealthy strategies were found to be at a higher risk of eating disorders, since these behaviors were accompanied by higher levels of drive for thinness and body dissatisfaction, as well as by beliefs associated with the importance of weight and body shape as a means of personal and social acceptance.