The role of CYP1A1/2 in cholesterol ester accumulation provides a new perspective for the treatment of hypercholesterolemia

Cholesterol is an important precursor of many endogenous molecules. Disruption of cholesterol homeostasis can cause many pathological changes, leading to liver and cardiovascular diseases. CYP1A is widely involved in cholesterol metabolic network, but its exact function has not been fully elucidated. Here, we aim to explore how CYP1A regulates cholesterol homeostasis. Our data showed that CYP1A1/2 knockout (KO) rats presented cholesterol deposition in blood and liver. The serum levels of low-density lipoprotein cholesterol, high-density lipoprotein cholesterol and total cholesterol were significantly increased in KO rats. Further studies found that the lipogenesis pathway (LXRα-SREBP1-SCD1) of KO rats was activated, and the key protein of cholesterol ester hydrolysis (CES1) was inhibited. Importantly, lansoprazole can significantly alleviate rat hepatic lipid deposition in hypercholesterolemia models by inducing CYP1A. Our findings reveal the role of CYP1A as a potential regulator of cholesterol homeostasis and provide a new perspective for the treatment of hypercholesterolemia.

Anatomopathological findings of Testudines necropsied in the Distrito Federal, Brazil / Achados anatomopatológicos de Testudines necropsiados no Distrito Federal, Brasil

In order to determine the main anatomopathological findings of Testudines necropsied in the Distrito Federal, all necropsy records performed at the "Laboratório de Patologia Veterinária" of the "Universidade de Brasília" (LPV-UnB) on Testudines during the period from January 2008 to July 2020 were reviewed. The 72 cases reviewed were grouped and classified according to species, sex, origin, season of occurrence, and diagnosis. In 69.44% of the cases the species was informed in the necropsy protocols, which included Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) and Podocnemis expansa (2%). In 30.55% of the cases this parameter was not informed and were classified only as Testudines. In 41.66% of the cases the sex was informed, being female 22.22%, male 19.44%, and 58.33% were not informed. Of these animals 79.16% were from environmental agencies and 20.84% from zoos and/or guardians. In 70.83% of the animals analyzed they were directly related to the autumn and winter seasons, with June being the most frequent month (29.17%). The conclusive diagnosis was possible in 68.05% of the cases. The category of disorders caused by injurious agents (48.97%) was the most prevalent, followed by inflammatory disorders (32.65%) and nutritional and metabolic disorders (28.57%). The main diagnoses were carapace and/or plastron fracture with 30.61%, hepatic steatosis (20.40%) and pneumonia (10.22%). Most cases of carapace or plastron fracture and hepatic steatosis occurred in animals from environmental agencies.

Com o objetivo de determinar os principais achados anatomopatológicos de Testudines necropsiados no Distrito Federal, foram revisadas todas as fichas de necropsia realizadas no Laboratório de Patologia Veterinária da Universidade de Brasília (LPV-UnB) em Testudines, durante o período de janeiro de 2008 a julho de 2020. Os 72 casos revisados foram agrupados e classificados quanto à espécie, sexo, procedência, estação do ano de ocorrência e diagnóstico. Em 69,44% dos casos havia a espécie informada nos protocolos de necropsia, que incluíam Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) e Podocnemis expansa (2%). Em 30,55% dos casos não tiveram esse parâmetro informado e foram classificados apenas como Testudines. Em 41,66% casos foi informado o sexo, sendo fêmea 22,22%, macho 19,44% e não informados 58,33%. Destes animais 79,16% eram de órgão ambiental e 20,84% de zoológicos e ou tutores. Em 70,83% dos animais analisados tiveram direta relação com as estações de outono e inverno, sendo o mês de junho o mais frequente (29,17%). O diagnóstico conclusivo foi possível em 68,05% dos casos. A categoria de distúrbios causados por agentes lesivos (48,97%) foi a mais prevalente, seguido por distúrbios inflamatórios (32,65%) e dos distúrbios nutricionais e metabólicos (28,57%). Os principais diagnósticos foram fratura de carapaça e ou plastrão com 30,61%, esteatose hepática (20,40%) e pneumonia (10,22%). A maior parte dos casos de fratura de carapaça ou plastrão e de esteatose hepática ocorreram em animais provenientes de órgão ambiental.

Anatomopathological findings of Testudines necropsied in the Distrito Federal, Brazil

ABSTRACT: In order to determine the main anatomopathological findings of Testudines necropsied in the Distrito Federal, all necropsy records performed at the Laboratório de Patologia Veterinária of the Universidade de Brasília (LPV-UnB) on Testudines during the period from January 2008 to July 2020 were reviewed. The 72 cases reviewed were grouped and classified according to species, sex, origin, season of occurrence, and diagnosis. In 69.44% of the cases the species was informed in the necropsy protocols, which included Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) and Podocnemis expansa (2%). In 30.55% of the cases this parameter was not informed and were classified only as Testudines. In 41.66% of the cases the sex was informed, being female 22.22%, male 19.44%, and 58.33% were not informed. Of these animals 79.16% were from environmental agencies and 20.84% from zoos and/or guardians. In 70.83% of the animals analyzed they were directly related to the autumn and winter seasons, with June being the most frequent month (29.17%). The conclusive diagnosis was possible in 68.05% of the cases. The category of disorders caused by injurious agents (48.97%) was the most prevalent, followed by inflammatory disorders (32.65%) and nutritional and metabolic disorders (28.57%). The main diagnoses were carapace and/or plastron fracture with 30.61%, hepatic steatosis (20.40%) and pneumonia (10.22%). Most cases of carapace or plastron fracture and hepatic steatosis occurred in animals from environmental agencies.

RESUMO: Com o objetivo de determinar os principais achados anatomopatológicos de Testudines necropsiados no Distrito Federal, foram revisadas todas as fichas de necropsia realizadas no Laboratório de Patologia Veterinária da Universidade de Brasília (LPV-UnB) em Testudines, durante o período de janeiro de 2008 a julho de 2020. Os 72 casos revisados foram agrupados e classificados quanto à espécie, sexo, procedência, estação do ano de ocorrência e diagnóstico. Em 69,44% dos casos havia a espécie informada nos protocolos de necropsia, que incluíam Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) e Podocnemis expansa (2%). Em 30,55% dos casos não tiveram esse parâmetro informado e foram classificados apenas como Testudines. Em 41,66% casos foi informado o sexo, sendo fêmea 22,22%, macho 19,44% e não informados 58,33%. Destes animais 79,16% eram de órgão ambiental e 20,84% de zoológicos e ou tutores. Em 70,83% dos animais analisados tiveram direta relação com as estações de outono e inverno, sendo o mês de junho o mais frequente (29,17%). O diagnóstico conclusivo foi possível em 68,05% dos casos. A categoria de distúrbios causados por agentes lesivos (48,97%) foi a mais prevalente, seguido por distúrbios inflamatórios (32,65%) e dos distúrbios nutricionais e metabólicos (28,57%). Os principais diagnósticos foram fratura de carapaça e ou plastrão com 30,61%, esteatose hepática (20,40%) e pneumonia (10,22%). A maior parte dos casos de fratura de carapaça ou plastrão e de esteatose hepática ocorreram em animais provenientes de órgão ambiental.

Anatomopathological findings of Testudines necropsied in the Distrito Federal, Brazil / Achados anatomopatológicos de Testudines necropsiados no Distrito Federal, Brasil

In order to determine the main anatomopathological findings of Testudines necropsied in the Distrito Federal, all necropsy records performed at the "Laboratório de Patologia Veterinária" of the "Universidade de Brasília" (LPV-UnB) on Testudines during the period from January 2008 to July 2020 were reviewed. The 72 cases reviewed were grouped and classified according to species, sex, origin, season of occurrence, and diagnosis. In 69.44% of the cases the species was informed in the necropsy protocols, which included Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) and Podocnemis expansa (2%). In 30.55% of the cases this parameter was not informed and were classified only as Testudines. In 41.66% of the cases the sex was informed, being female 22.22%, male 19.44%, and 58.33% were not informed. Of these animals 79.16% were from environmental agencies and 20.84% from zoos and/or guardians. In 70.83% of the animals analyzed they were directly related to the autumn and winter seasons, with June being the most frequent month (29.17%). The conclusive diagnosis was possible in 68.05% of the cases. The category of disorders caused by injurious agents (48.97%) was the most prevalent, followed by inflammatory disorders (32.65%) and nutritional and metabolic disorders (28.57%). The main diagnoses were carapace and/or plastron fracture with 30.61%, hepatic steatosis (20.40%) and pneumonia (10.22%). Most cases of carapace or plastron fracture and hepatic steatosis occurred in animals from environmental agencies.(AU)

Com o objetivo de determinar os principais achados anatomopatológicos de Testudines necropsiados no Distrito Federal, foram revisadas todas as fichas de necropsia realizadas no Laboratório de Patologia Veterinária da Universidade de Brasília (LPV-UnB) em Testudines, durante o período de janeiro de 2008 a julho de 2020. Os 72 casos revisados foram agrupados e classificados quanto à espécie, sexo, procedência, estação do ano de ocorrência e diagnóstico. Em 69,44% dos casos havia a espécie informada nos protocolos de necropsia, que incluíam Phrynops geoffroanus (38%), Trachemys dorbigni (36%), Chelonoidis carbonaria (14%), Chelonoidis denticulata (10%) e Podocnemis expansa (2%). Em 30,55% dos casos não tiveram esse parâmetro informado e foram classificados apenas como Testudines. Em 41,66% casos foi informado o sexo, sendo fêmea 22,22%, macho 19,44% e não informados 58,33%. Destes animais 79,16% eram de órgão ambiental e 20,84% de zoológicos e ou tutores. Em 70,83% dos animais analisados tiveram direta relação com as estações de outono e inverno, sendo o mês de junho o mais frequente (29,17%). O diagnóstico conclusivo foi possível em 68,05% dos casos. A categoria de distúrbios causados por agentes lesivos (48,97%) foi a mais prevalente, seguido por distúrbios inflamatórios (32,65%) e dos distúrbios nutricionais e metabólicos (28,57%). Os principais diagnósticos foram fratura de carapaça e ou plastrão com 30,61%, esteatose hepática (20,40%) e pneumonia (10,22%). A maior parte dos casos de fratura de carapaça ou plastrão e de esteatose hepática ocorreram em animais provenientes de órgão ambiental.(AU)

L-carnitine supplementation descreases hepatic triglyceride accumulation in Holstein cows during the transition period / La suplementación con L-carnitina disminuye la acumulación de triglicéridos hepáticos en vacas Holstein durante el periodo de transición / Suplementação com L-carnitina diminui o acúmulo de triglicerídeos hepáticos em vacas Holandesas durante o período de transição

Abstract Background: The increased use of body reserves observed during peripartum leads to higher needs of L-carnitine by cows, which is restrictive under the production conditions of Colombian high tropics. Objective: To evaluate the lipotropic potential of L-carnitine in Holstein dairy cows during the transition period to lactation. Methods: Twenty-one Holstein cows were fed 0, 100, or 200 g/d L-carnitine fumarate from d 260 of gestation to d 20 postpartum. Hepatic triacylglycerides concentration, total carnitine, free carnitine, acylcarnitine, and serum levels of non-esterified fatty acids (NEFA), β-hydroxybutyrate (β-HB) and urea were determined by spectrophotometry. Repeated measures analysis was used to determine the effects of dose, measurement period, and their interactions. Results: Hepatic triglycerides and the different forms of carnitine showed no difference between sampling periods (p>0.05). Hepatic triglycerides concentration was low and decreased in response to 200 g/d L-carnitine fumarate supplementation (p<0.05). This decrease in hepatic triglycerides could be due to increased fatty acid oxidation. L-carnitine supplementation significantly increased (p<0.05) blood urea concentration, possibly through stimulation of the urea cycle, as previously described in other species. Conclusion: Supplementation with L-carnitine decreased the hepatic concentration of triglycerides, possibly due to increased liver oxidation of fatty acids.

Resumen Antecedentes: El incremento en la utilización de reservas corporales durante el periparto en vacas exige una alta disponibilidad de L-carnitina, la cual puede ser limitante bajo las condiciones propias de producción del trópico alto colombiano. Objetivo: Evaluar el potencial lipotrópico de la L-carnitina en vacas lecheras Holstein durante el periodo de transición a la lactancia. Métodos: Se suministraron dosis de 0, 100 y 200 g/d de fumarato de L-carnitina a 21 vacas Holstein a partir del d 260 de gestación y hasta el d 20 postparto. Se determinaron las concentraciones hepáticas de triacilglicéridos, carnitina total, carnitina libre y acil carnitina, y las concentraciones plasmáticas de ácidos grasos no esterificados (NEFA), β-hidroxibutirato (β-HB) y urea. Un análisis de medidas repetidas fue usado para determinar los efectos de la dosis, el periodo de medición, y sus interacciones. Resultados: Las concentraciones hepáticas de triglicéridos y de las diferentes formas de carnitina no difirieron significativamente (p>0,05) entre periodos de muestreo. La concentración hepática de triglicéridos fue baja, y mostró una disminución significativa (p<0,05) en respuesta a la suplementación con 200 g/d de fumarato de L-carnitina. La disminución en los triglicéridos hepáticos podría deberse a un aumento en la oxidación de ácidos grasos. La suplementación con L-carnitina aumentó significativamente (p<0,05) la concentración de urea en sangre, posiblemente a través de un mecanismo de estimulación del ciclo de la urea, descrito previamente en otras especies. Conclusión: La suplementación con L-carnitina disminuyó la concentración hepatica de trigliceridos, debido posiblemente a un aumento en la oxidación de ácidos grasos en hígado.

Resumo Antecedentes: O aumento do uso de reservas corporais durante o periparto requer alta disponibilidade de L-carnitina, que pode ser limitante nas condições de produção no trópico alto colombiano. Objetivo: Avaliar o potencial lipotrópico da L-carnitina em vacas leiteiras Holstein durante o período de transição para a lactação. Métodos: Quantidades de 0, 100 e 200 g/d de fumarato de L-carnitina foram administradas a 21 vacas holandesas durante o d 260 da gestação e até ao d 20 pós-parto. Foram determinadas as concentrações hepáticas de triacilglicerídeos, carnitina total, carnitina livre e acil carnitina e as concentrações plasmáticas de ácidos graxos não esterificados (NEFA), β-hidroxibutirato (β-HB) e ureia. Uma análise das medidas repetidas foi usada para determinar os efeitos da dose, período de medição e suas interações. Resultados: As concentrações hepáticas de triglicerídeos e as diferentes formas de carnitina não diferiram significativamente entre os períodos de amostragem (p>0,05). A concentração hepática de triglicerídeos foi baixa e mostrou diminuição significativa (p<0,05) em resposta à suplementação com 200 g/d de fumarato de L-carnitina. A diminuição dos triglicerídeos hepáticos pode ser devido a um aumento na oxidação de ácidos graxos. A suplementação com L-carnitina aumentou significativamente (p<0,05) a concentração de ureia no sangue, possivelmente através de um mecanismo de estimulação do ciclo da ureia previamente descrito em outras espécies. Conclusão: A suplementação com L-carnitina diminuiu a concentração hepática de triglicerídeos, possivelmente devido ao aumento da oxidação de ácidos graxos no fígado.

Efecto del nivel de complementación con metionina-colina en vacas Holstein durante el periodo de transición sobre las concentraciones hepáticas de triglicéridos, colina y carnitina / Effect of the level of complementation with methionine-coline in Holstein cows during the transitional period on the hepatic concentrations of triglycerides, choline and carnitine

RESUMEN Con el objetivo de evaluar el potencial lipotropics de metionina-colina en vacas lecheras durante el periodo de transición, dosis de 20 g/d de una Fuente Comercial Protegida de la Degradación Ruminal (FCPDR) de metionina y sus combinaciones con una FCPDR de colina en dosis de 60 y 120 g/d se suministraron a vacas entre el día 260 de gestación y el día 20 posparto. Estas pastoreaban kikuyo (Cenchrus clamdestinus) y recibieron un complemento alimenticio. En los días 270 de gestación y 10 y 20 posparto se cuantificaron las concentraciones plasmáticas de ácidos grasos no esterificados (AGNES), β-Hidroxibutirato (BHB) y triglicéridos (TG), así como las concentraciones hepáticas de triacilglicéridos, de colina, carnitina total, carnitina libre y acil carnitina. Durante el preparto se presentaron los valores significativamente más altos (p < 0.05) de TG plasmáticos y los significativamente más bajos (p < 0.05) de AGNES y BHB. Las concentraciones hepáticas de TG, de colina y de las diferentes formas de carnitina no difirieron significativamente entre periodos de muestreo y no se vieron afectadas por la suplementación con metionina-colina. Por su parte, las concentraciones plasmáticas de BHB aumentaron significativamente (p < 0.05) con la suplementación conjunta de metionina y colina, lo que sugiere un aumento en la oxidación de ácidos grasos. Se concluye que con concentraciones bajas de AGNES la metionina y colina no son nutrientes limitantes para la exportación de TG desde el hígado a la sangre. Bajo esta condición no se observó lipidosis hepática y como consecuencia la suplementación con metionina-colina no ocasionó efectos significativos sobre las concentraciones hepáticas de TG.

ABSTRACT In order to evaluate the lipotropic potential of methionine-coline in dairy cows during the transition period, the effect of supplementation with 20 g / d of a Ruminal Degradation Protected Commercial Source (FCPDR) of methionine was evaluated, and their combinations with a choline FCPDR in doses of 60 and 120 g / day. Cows grazed kikuyo (Cenchrus clandestinus) and received a nutritional supplement between day 260 of gestation and day 20 postpartum. On day 270 of gestation and on days 10 and 20 postpartum blood samples and liver biopsy were taken, in which were quantified: Non-esterified Fatty Acids (NEFAS), β-Hydroxybutyrate (BHB) and Triglycerides; And hepatic concentrations of triacylglycerides, choline, total carnitine, free carnitine and acyl carnitine. The highest values (p < 0.05) of plasma triglycerides and the significantly lower values (p < 0.05) of AGNES and BHB were presented during the prepartum. Hepatic concentrations of triglycerides, choline and different forms of carnitine did not differ significantly between sampling periods, and were unaffected by methionine-choline supplementation. Plasma concentrations of BHB increased significantly (p < 0.05) with co-supplementation with methionine and choline, suggesting an increase in fatty acid oxidation. At relatively low concentrations of AGNES, methionine and choline are not limiting nutrients for the exportation of triglycerides from the liver to the blood, therefore under this condition no hepatic lipidosis was observed and consequently methionine-choline supplementation did not cause significant effects on hepatic triglyceride concentrations.

Signs and symptoms in Gaucher Disease: priority nursing diagnoses / Señales y síntomas de la Enfermedad de Gaucher: diagnósticos prioritarios de enfermería / Sinais e sintomas na Doença de Gaucher: diagnósticos de enfermagem prioritários

ABSTRACT Objective: Identify the signs and symptoms of patients with Gaucher Disease, inferring possible priority nursing diagnoses. Method: Cross-sectional study, developed in a specialized laboratory, between 2013 and 2015. The sample (n = 91) comprised the records of patients with genetic diagnosis for Gaucher Disease. The study respected research norms. Results: Prevalence of female sex (57.1%), age at diagnosis between 0 and 10 years, and origin from the Southeast Region of Brazil were prevalent. Hematologic changes, bone pain, hepatomegaly, splenomegaly, and fatigue were the most recurrent signs and symptoms. The inferred diagnoses for the studied population were: Risk for bleeding; Fatigue; Chronic pain and Acute pain; Impaired physical mobility; Imbalanced nutrition: less than body requirements; and Risk for Developmental Delay. Conclusion: The establishment of Priority Nursing Diagnoses based on signs and symptoms makes it possible to achieve expected outcomes for each individual in the care context.

RESUMEN Objetivo: Identificar las señales y los síntomas de pacientes con Enfermedad de Gaucher, infiriendo los posibles diagnósticos prioritarios de enfermería. Método: Estudio transversal, desarrollado entre 2013 y 2015 en un laboratorio especializado. La muestra (n=91) estaba constituida por los registros de pacientes con diagnóstico genético de la Enfermedad de Gaucher. El estudio respetó las normas de la investigación. Resultados: Prevaleció el sexo femenino (57,1%), con franja de edad entre 0 y 10 años y procedencia de la Región Sureste de Brasil. Las alteraciones hematológicas, el dolor óseo, la hepatomegalia, la esplenomegalia y el cansancio fueron las señales y los síntomas más recurrentes. Los diagnósticos inferidos de la población estudiada fueron: Riesgo de sangrado; Fatiga; Dolor crónico y Dolor agudo; Movilidad física perjudicada; Nutrición desequilibrada: menos que las necesidades corporales; y Riesgo de Desarrollo atrasado. Conclusión: El establecimiento de los diagnósticos prioritarios de enfermería, a partir de las señales y de los síntomas, permite obtener los resultados esperados para cada individuo en el contexto del cuidado.

RESUMO Objetivo: Identificar os sinais e sintomas de pacientes com Doença de Gaucher, inferindo os possíveis diagnósticos de enfermagem prioritários. Método: Estudo transversal, desenvolvido em laboratório especializado, entre 2013 e 2015. A amostra (n=91) foi constituída dos registros de pacientes com diagnóstico genético para Doença de Gaucher. O estudo respeitou normas de pesquisa. Resultados: Foram prevalentes o sexo feminino (57,1%), faixa etária ao diagnóstico entre 0 e 10 anos e proveniência da Região Sudeste do Brasil. Alterações hematológicas, dor óssea, hepatomegalia, esplenomegalia, cansaço foram os sinais e sintomas mais recorrentes. Os diagnósticos inferidos para a população estudada foram: Risco de sangramento; Fadiga; Dor crônica e Dor aguda; Mobilidade física prejudicada; Nutrição desequilibrada: menos do que as necessidades corporais; e Risco de Desenvolvimento atrasado. Conclusão: O estabelecimento dos Diagnósticos de Enfermagem prioritários a partir dos sinais e sintomas possibilita alcançar resultados esperados a cada indivíduo no contexto do cuidado.

Research and development of primary bilateral lipedema / 中华整形外科杂志

Lipedema is secondary to local fat deposition, a disease characterized by the symmetric thickening of lower limbs, mostly occurs in women, especially in adolescence and pregnancy. In its early stage, it could be easily confused with lymphedema. Extensive literature review on primary fat edema in recent years, as well as a summary of the clinical symptoms and signs and diagnosis and treatment of lipedema were conducted, so as to provide a useful reference for clinicians.

Nobiletin attenuates palmic acid-induced lipidosis in hepatocytes and regulatory mechanism of lncLSTR / 中国病理生理杂志

AIM:To observe the effects of nobiletin on palmic acid (PA)-induced lipidosis in hepatocytes and to discuss the regulatory mechanism of lncLSTR. METHODS:AML12 cells were cultured in vitro. The control group, PA group (0.2 mmol/L) and protection group (exposure to nobiletin at 1 mg/L, 5 mg/L, 15 mg/L or 50 mg/L for 2 h, fol-lowed by treatment with 0.2 mmol/L PA) were established according to the experimental requirements. The lipid accumula-tion was morphologically observed by Oil red O staining in the cells. The qPCR was applied to detect mRNA expression, and the protein expression was determined by and Western blot. RESULTS:PA treatment (0.2 mmol/L) induced lipidosis, while 50 mg/L nobiletin pretreatment suppressed the lipidosis. Compared with control group, the mRNA expression of Apoc2 in PA group was significantly down-regulated (P<0.05), but increased by nobiletin pretreatment compared with PA group (P<0.05). The protein expression of Apoc2 in PA group was significantly down-regulated (P<0.05), but increased by nobiletin pretreatment (P<0.05). The expression of lncLSTR in PA group was significantly increased (P<0.05) and that was inhibited by nobiletin pretreatment (P<0.05). A negative correlation between Apoc2 protein and lncLSTR expression in PA group (R2=0.717 9, P<0.01) was observed. A negative correlation between Apoc2 protein and lncLSTR expression in protection group (R2=0.525 3, P<0.05) was also found. CONCLUSION:Nobiletin has anti-lipidosis effect on hepa-tocytes. The mechanism is partially related to the inhibition of up-regulation of lncLSTR, thus down-regulating Apoc2 expres-sion.

Diagnostic laparoscopy in a leopard cat (Prionailurus bengalensis) with intercostal abdominal hernia and hepatic lipidosis

Intercostal abdominal hernia in the 11th intercostal space was identified in a leopard cat. Although mild leukopenia was found in laboratory examinations, no remarkable abnormality was revealed in medical imaging. To investigate abdominal organs, diagnostic laparoscopy was performed after hernia repair. In laparoscopic view, closure of the herniation site and a lesion with whitish discoloration in the liver (left medial lobe) were observed. Subsequently, laparoscopic liver biopsy was performed against the affected hepatic tissue. Histologically, the sample was diagnosed as mild hepatic lipidosis. Laparoscopy is considered useful for abdominal visceral examination and liver biopsy in a leopard cat patient.

Extraction, quantification and distribution of the major lipid fractions in small liver biopsies of cows in transition period / Extracción, cuantificación y distribución de las principales fracciones lipídicas en pequeñas biopsias de hígado de vacas en el periodo de transición / Extracão, quantificação e distribuição das principais frações lipídicas em pequenas biópsias de fígado de vacas em período de transição

There are difficulties in understanding changes in the distribution of the major lipid fractions in the liver of dairy cows due to the need for invasive techniques for sampling and relatively large quantities of sample needed for analysis. The objective of this research was to integrate a method for extracting total lipid (TL) with the enzymatic determination of its fractions in small biopsies. Liver biopsies from 60 Holstein cows in the transition period (between day 270 of gestation and 20 postpartum) underwent TL extraction using Hexane-Isopropanol mixture. TL obtained was dissolved in hexane, several aliquots of different volumes were used for the enzymatic determination of: triglycerides (TG), phospholipids (PL), total cholesterol (TC) and free fatty acids (FFA). The amount of liver sample used was 49.6 mg ± 18.45mg (Mean ± Standar Deviation). The average was 7.20% TL. The average coefficient of variation (CV) in TL extraction was 7.62%. TL. TG, PL, TC and FFA represented 52.5%; 18.30%; 19.30% and 0.41% respectively of the TL. CV for enzimatic determinations of TG, PL, TC and FFA were: 5.2%; 9.9%; 6.1% and 4.0% respectively, indicating that this method is suitable for determination of lipid fractions in small liver samples. High correlation coefficient was observed between TL and TG (0.96); while for TL and PL was -0.42; and for TL and TC -0.62; It is indicating that the increase in hepatic lipid occurred mainly in the TG fraction and consequently showed a relative decrease in the fractions PL and TC.

Existen dificultades para conocer los cambios en la distribución de las principales fracciones lipídicas en el hígado de las vacas lecheras debido a la necesidad de técnicas invasivas para su muestreo y de cantidades relativamente grandes de muestra para su análisis. El objetivo de esta investigación fue integrar un método para la extracción de lípidos totales (LT) con la determinación enzimática de sus diferentes fracciones en pequeñas biopsias. Biopsias hepáticas provenientes de 60 vacas Holstein en el periodo de transición (entre el día 270 de gestación y 20 posparto) fueron sometidas a la extracción de LT utilizando una mezcla Hexano-Isopropanol. Los LT obtenidos fueron disueltos en hexano, varias alícuotas de diferentes volúmenes fueron utilizados para la determinación enzimática de: triglicéridos (TG), fosfolípidos (FL), Colesterol total (CT) y Ácidos Grasos Libres (AGL). La cantidad de muestra hepática utilizada fue 49.6 mg ±18.45mg (Media ± Desviación estándar). El promedio de LT fue 7.20%. El coeficiente promedio de variación (CV) en la extracción de LT fue 7,62%. Los TG, FL, CT y AGL representaron el: 52,5%; 18,30%; 19,30% y 0.41% de los LT respectivamente. Los CV para las determinaciones enzimáticas de TG, FL, CT y AGL fueron: 5,2%; 9,9%; 6,1% y 4,0% respectivamente, indicando que la determinación de las principales fracciones lipídicas en pequeñas biopsias de hígado se puede realizar de manera confiable a partir de una única muestra. Se observó un coeficiente de correlación alto entre: LT y TG (0,96); mientras que para LT y FL fue de -0,42; y para LT y CT de -0,62; lo que indicó que el aumento en los lípidos hepáticos ocurrió principalmente en la fracción TG, y en consecuencia presentó una disminución relativa en la fracciones FL y CT.

Há dificuldades para compreender mudanças na distribuição das principais frações lipídicas no fígado de vacas leiteiras, devido à necessidade de técnicas invasivas para a amostragem e quantidades relativamente grandes de amostra necessárias para análise. O objetivo desta pesquisa foi o de integrar um método para extrair lipídios totais (LT) com a determinação enzimática de suas frações em pequenas biopsias de fígado. As biópsias do fígado de 60 vacas da raça Holandesa no período de transição (entre o dia 270 de gestação e 20 pós-parto) foram submetidos a extração de LT usando mistura hexano-isopropanol. LT obtido foi dissolvido em hexano, foram usadas várias aliquotas de volumes diferentes para a determinação enzimática de: triglicéridos (TG), fosfolipidos (FL), colesterol total (CT) e os ácidos gordos livres (AGL). A quantidade de amostra utilizada fígado foi de 49,6 mg ±18.45mg (Média ± desvio padrão). A média dé % de LT foi de 7,20. O coeficiente médio de variação (CV) na extração de LT foi 7,62%. TG, FL, CT e AGL representou 52,5%; 18,30%; 19,30% e 0,41%, respectivamente, da LT. CV para determinação enzimática de TG, FL, CT e AGL foram: 5,2%; 9,9%; 6,1% e 4,0%, respectivamente, indicando que este método é adequado para a determinação das fracções de lipídios em pequenas amostras de fígado. Foram observados altos coeficientes de correlação entre LT e TG (0,96); ao passo que para LT e FL foi -0,42; e para LT e CT foi -0,62; indicando que o aumento no lipídio hepático ocorre principalmente na fracção de TG e, consequentemente se apresentou uma diminuição relativa nas fracções FL e CT

A case report on cerebrotendinous xanthomatosis.

Cerebrotendinous xanthomatosis (CTX) is a rare hereditary neuro-metabolic disease in which deposition of cholesterol and cholestanol occurs in various tissues including CNS. It is characterized by juvenile cataract, tendon xanthomas and progressive neurological defects. It is one of a group of neurologic disorder collectively referred to as leukodystrophy, which predominantly affects the CNS white matter. We are presenting a patient with cerebrotendinous xanthomatosis, who is now 36 years old, and shows the natural course of disease in an untreated patient. He presented with xanthomas on Achilles tendon, elbow and knees and showed cerebellar and pyramidal signs. He had recurrent seizures and was mentally subnormal.

Association of adult weight gain and nonalcoholic fatty liver in a cross-sectional study in Wan Song Community, China

Our objective was to examine associations of adult weight gain and nonalcoholic fatty liver disease (NAFLD). Cross-sectional interview data from 844 residents in Wan Song Community from October 2009 to April 2010 were analyzed in multivariate logistic regression models to examine odds ratios (OR) and 95% confidence intervals (CI) between NAFLD and weight change from age 20. Questionnaires, physical examinations, laboratory examinations, and ultrasonographic examination of the liver were carried out. Maximum rate of weight gain, body mass index, waist circumference, waist-to-hip ratio, systolic blood pressure, diastolic blood pressure, fasting blood glucose, cholesterol, triglycerides, uric acid, and alanine transaminase were higher in the NAFLD group than in the control group. HDL-C in the NAFLD group was lower than in the control group. As weight gain increased (measured as the difference between current weight and weight at age 20 years), the OR of NAFLD increased in multivariate models. NAFLD OR rose with increasing weight gain as follows: OR (95%CI) for NAFLD associated with weight gain of 20+ kg compared to stable weight (change <5 kg) was 4.23 (2.49-7.09). Significantly increased NAFLD OR were observed even for weight gains of 5-9.9 kg. For the “age 20 to highest lifetime weight” metric, the OR of NAFLD also increased as weight gain increased. For the “age 20 to highest lifetime weight” metric and the “age 20 to current weight” metric, insulin resistance index (HOMA-IR) increased as weight gain increased (P<0.001). In a stepwise multivariate regression analysis, significant association was observed between adult weight gain and NAFLD (OR=1.027, 95%CI=1.002-1.055, P=0.025). We conclude that adult weight gain is strongly associated with NAFLD.

Relationship among blood indicators of lipomobilization and hepatic function during early lactation in high-yielding dairy cows

Blood indicators are used as a tool to diagnose metabolic disorders. The present work was conducted to study the relationships among blood indicators of lipomobilization and hepatic function in high-yielding dairy cows. Two groups of Holstein cows were studied: 27 early lactation cows and 14 mid lactation cows from four different herds with similar husbandry characteristics in Galicia, Spain. Blood samples were obtained to measure beta-hydroxybutyrate (BHB), non-esterified fatty acids (NEFA), triglycerides (TG), and the activity of aspartate transaminase (AST) and gamma-glutamyl transferase. Cows in early lactation had higher levels of BHB and NEFA than mid lactation cows. High lipomobilization (NEFA > 400 micromol/L) was detected in 67% and 7% of early lactation and mid lactation cows, respectively, while subclinical ketosis (BHB > 1.2 mmol/L) was detected in 41% and 28% of the early lactation and lactation cows, respectively. TG concentrations were low in all cows suffering subclinical ketosis and in 61% of the cows with high lipomobilization. During early lactation, 30% of cows suffered hepatic lipidosis as detected by levels of AST. Compromised hepatic function was observed in early lactation cows as shown by lower concentrations of glucose, total protein, and urea.

Estudo da doença de Gaucher em Santa Catarina / Study of Gaucher disease in Santa Catarina

A doença de Gaucher (DG) foi a primeira doença de armazenamento lisossomal descrita e a mais encontrada. Caracteriza-se pela deficiência hereditária da atividade da enzima lisossomal glucocerebrosidase, que bloqueia o metabolismo do glicocerebrosídeo. A proposta deste trabalho foi estudar as características clínicas, laboratoriais e radiológicas, as principais mutações encontradas, relacionando-as com as formas clínicas e avaliar a resposta à terapia de reposição enzimática (TRE) nos pacientes com DG em Santa Catarina. Foram estudados dez pacientes com DG no Hospital Universitário, no período entre 1998 e 2003, após confirmação diagnóstica da doença pela dosagem da enzima beta-glicosidase em leucócitos. Pesquisa das mutações foi realizada em amostras de sangue e de mucosa oral. A média de idade ao diagnóstico foi de 19,6 anos. A DG tipo 1 foi diagnosticada em 80 por cento dos casos, e a tipo 2 em 20 por cento. Quatro pacientes tiveram história familiar de DG. Hepatoesplenomegalia foi a manifestação clínica mais comum. Anemia e trombocitopenia ocorreram em 100 por cento dos casos. Dores ósseas foram relatadas por 75 por cento dos pacientes. Os alelos mutantes encontrados foram N370S e L444P. Houve elevação dos níveis de hemoglobina em todos os pacientes com DG tipo 1. Concluímos que a DG tipo 1 é a forma clínica mais comum. Anemia, trombocitopenia, hepatoesplenomegalia e osteopenia são as características mais freqüentes dos pacientes com DG. O alelo N370S é o mais freqüente, estando relacionado com o tipo 1. O alelo L444P em homozigose sugere letalidade precoce. A TRE é segura e efetiva para a DG tipo 1.

Gaucher Disease (GD) was the first described and is the most common lysosomal deposit disease. It is characterized byahereditary deficiency of glucocerebrosidase lysosomal enzyme activity which blocks the metabolism of glucocerebrosideo. The aim of this work was to study the clinical, laboratorial and radiological characteristics, the main mutations correlating them with the clinical form of the disease and evaluating the response to enzymatic replacement therapy in patients with GD in Santa Catarina. Ten GD patients were studied at a University Hospital between 1998 and 2003. The disease was diagnosed by measurement of the beta-glucosidase enzyme in leukocytes. Investigation of mutations used samples of blood and oral mucus. The average age at diagnosis was 19.6 years. Type 1 GD was diagnosis in 80 percent of the cases and type 2 in 20 percent. Four patients had a family history of GD. Hepatosplenomegaly was the most common clinical manifestation. Anemia and thrombocitopenia occurred in all cases. Bone pain was reported by 75 percent of the patients. The mutanted alleles identified were N370S and L444P. The hemoglobina levels were elevated in all patients with type 1 GD. In conclusion, type 1 GD is the most common clinical form and anemia, thrombocitopenia, hepatosplenomegaly and osteopenia were the most common characteristics of GD patients. The N370S allele is the most common mutation and is related with type 1 GD 1. Homozygosity of the L444P allele suggests early death. Enzyme replacement therapy is safe and efficacious in type 1 GD.