Fabry disease caused by the GLA p.Phe113Leu (p.F113L) variant: Natural history in males.

BACKGROUND, AIMS AND METHODS: The α-galactosidase gene (GLA) c.337T>C/p.Phe113Leu variant was originally described in patients with late-onset cardiac forms of Fabry disease (FD), who had residual α-galactosidase activity. It has since emerged as the most commonly reported GLA variant in Portuguese subjects diagnosed with FD but is also prevalent in the Italian population, where two boys carrying the GLA Leu113 allele were identified in a large-scale newborn screening program, the variant allele segregating in both cases with the same surrounding haplotype. To further delineate the genotype-phenotype correlations of this GLA variant, we have reviewed the natural history and clinical phenotypes of 11 symptomatic Portuguese males, from 10 unrelated families originating from several different areas in mainland Portugal and Madeira Island, who were diagnosed with FD associated with the GLA Leu113 allele in a diversity of clinical and screening settings. Nine of the patients were the probands of their respective families. To test whether the GLA Leu113 allele inherited by the 10 Portuguese and the two Italian families resulted from independent mutational events, we have additionally performed a haplotype analysis with 5 highly polymorphic, closely linked microsatellite markers surrounding the GLA gene. RESULTS AND CONCLUSIONS: Hemizygosity for the GLA Leu113 variant allele is associated with a late-onset form of FD, invariably presenting with severe cardiac involvement. Clinically relevant cerebrovascular and kidney involvement may also occur in some patients but the pathogenic relationship between the incomplete α-galactosidase deficiency and the risks of stroke and of chronic kidney disease is not straightforward. The observation that the Leu113 allele segregated within the same GLA microsatellite haplotype in both the Portuguese and Italian families suggests its inheritance from a common ancestor.

Perdas econômicas causadas por lesões nodulares características de reação vacinal contra febre aftosa em bovinos abatidos em Mato Grosso do Sul, Brasil / Econômic losses caused by carcterisitc nodular injuries of vaccine reaction against foot-and-mouth disease in cattle slaughtered in Mato Grosso do Sul, Brazil

Para avaliar a incidência e estimar as perdas econômicas decorrentes de lesões nodulares características de reação vacinal contra febre aftosa foi acompanhado o abate de 72.000 bovinos no Mato Grosso do Sul, de maio a junho de 2018. O estudo foi realizado com base na avaliação macroscópica das lesões, que resultaram em uma incidência/mês média de 99,0% no rebanho abatido. O peso médio de descarte devido ao abscesso foi de 1,56kg/carcaça e a perda estimada foi de 673.920kg/ano ou 44.928 arrobas/ano. O preço médio de venda da carne foi de R$ 11,65/kg, sendo estimada perda de R$ 7.851.168,00/ano (US$ 2.116.117,79/ano). A elevada incidência de lesões nodulares características de reação à vacina contra febre aftosa nos bovinos abatidos no Mato Grosso do Sul representa um problema sanitário e econômico relevante na cadeia da carne.

Effect of probiotics on giardiasis. Where are we?

ABSTRACT Giardiasis, an intestinal infection caused by Giardia lamblia, was recently included in the 'Neglected Diseases Initiative' by the World Health Organization. Symptomatic patients can develop mild diarrhea up to a severe malabsorption syndrome, and children may show significantly impaired cognitive and physical development. Currently, nitroimidazoles are the main class of antibiotic used to treat giardiasis. Despite the efficacy of these drugs, adverse effects and reported resistance have increased, encouraging studies to identify and develop therapeutic alternatives. In this context, probiotics may represent an attractive option. Probiotics are defined as live microorganisms that, when administered in suitable amounts, confer a health benefit to the host. The use of probiotics in the treatment of parasitosis has been suggested because of its beneficial effects, such as a reduced time of gastrointestinal symptoms and parasite load in animal models and humans. Probiotics have been studied as an alternative treatment for giardiasis. In this review, we evaluated probiotic effectiveness in the treatment and prevention of this disease. Based on the studies examined herein, we conclude that probiotics have a beneficial effect on the modulation of giardiasis.

Malformações congênitas e abortos induzidos experimentalmente pela ingestão de Poincianella pyramidalis (Tul. ) L. P. Queiroz (catingueira) em ovelhas / Congenital malformations and abortions induced experimentally by the ingestion of Poincianella pyramidalis (Tul. ) L. P. Queiroz (catingueira) in sheep

Poincianella pyramidalis (catingueira) é uma planta tóxica associada a abortos, perdas embrionárias e malformações em caprinos e animais de laboratório. Surtos e casos esporádicos de intoxicação espontânea em ovinos vem sendo relatados nos últimos cinco anos no agreste de Pernambuco. Devido à ocorrência destes casos espontâneos e a escassez de pesquisas na espécie ovina, o objetivo desse trabalho foi realizar um estudo experimental para avaliação dos efeitos teratogênicos de P. pyramidalis em ovelhas. No experimento foram utilizadas 16 ovelhas e três carneiros, sem raça definida (SRD). As fêmeas foram divididas em quatro grupos, com quatro animais por grupo e mantidas em baias individuais, foram submetidas a sincronização do estro e acasalamento por monta natural, após 30 dias foi realizado o diagnóstico para confirmação da gestação. Os grupos foram denominados como: grupo 1 (G1), grupo 2 (G2), grupo 3 (G3) e grupo 4 (G4). No G1 (controle) as ovelhas foram alimentadas com 1% de suplementação concentrada (SC) baseado no peso vivo (PV) e 2% de volumoso a base de silagem de milho (SM), com uma ingestão diária de 3% de matéria seca (MS) com base no PV. No G2 a alimentação era constituída de 1% de SC com base no PV e 2% de volumoso. Sendo que destes 2% de volumoso, 50% era feno de P. pyramidalis e os outros 50% de SM. No G3 a alimentação era constituída de 1% de SC com base no PV e 2% de volumoso. Sendo que destes 2% de volumoso, 80% foi de feno de P. pyramidalis e os outros 20% de SM. No G4 as ovelhas foram alimentadas com 1% de SC com base no PV e o volumoso constituído de 100% de feno de P. pyramidalis. Todos os grupos receberam SC à base de farelo de milho, farelo de algodão, sal mineral especifico para ovinos e água ad libitum. Mensalmente os animais foram submetidos à ultrassonografia para detecção de perdas embrionárias ou fetais. Foram realizados exames sorológicos para Neospora caninum e Toxoplasma gondii antes da introdução dos animais no experimento. Os fetos com malformações foram avaliados no setor de Patologia da UFRPE / Unidade Acadêmica de Garanhuns (UAG) para descrição das lesões. No G2, ocorreram cinco casos de malformações de oito cordeiros nascidos. No G3, ocorreram quatro casos de malformação, sendo um decorrente de aborto. No G4, uma ovelha abortou e as outras três pariram quatro fetos malformados. As principais malformações encontradas no trabalho foram artrogripose, palatosquise, queilosquise, hipoplasia da mandíbula, aplasia do osso incisivo, malformações de olho e língua, polidactilia e monodactilia. Nos grupos de maior percentual de ingestão da planta foram observadas maiores quantidades de malformações e chamou a atenção a ocorrência de retenção de placenta e a alta frequência de mortalidade perinatal nos cordeiros que apresentaram algum tipo de malformação. Demonstrou-se com esse trabalho que a P. pyramidalis é uma importante causa de malformação congênita, aborto e mortalidade perinatal em ovinos, sendo de extrema importância que se mantenha as ovelhas prenhes fora do alcance desta planta durante todo o período gestacional.(AU)

Poincianella pyramidalis (catingueira) is a toxic plant associated with abortions, embryonic losses and malformations in goats and laboratory animals. Outbreaks and sporadic cases of spontaneous intoxication in sheep have been reported in the last five years in the agreste of Pernambuco. Due to the occurrence of these spontaneous cases and the lack of researches in sheep, an experimental study was carried out to evaluate the teratogenic effects of P. pyramidalis on sheep. In the experiment, 16 female and three male sheeps, mixed breed, were used. Females were divided in four groups, with four animals per group and maintained in individual stalls, underwent estrus synchronization and mating by natural mating and after 30 days the diagnosis was made to confirm gestation. Groups were named group 1 (G1), group 2 (G2), group 3 (G3), and group 4 (G4). In G1 (control) the ewes were fed with 1% of concentrate supplementation (CS) based on live weight (LW) and 2% of maize silage (MS) with a daily intake of 3% dry matter (DM) based on LW. In G2 feeding was constituted of 1% of CS based on LW and 2% of forage, including 2% of roughage, 50% was hay of P. pyramidalis and the other 50% of DM. In G3 feed was constituted of 1% of CS based on LW and 2% of roughage. Among these 2% of roughage, 80% was hay of P. pyramidalis and the other 20% of DM. In G4 the sheep were fed with 1% CS based on the LW and the roughage consisting of 100% P. pyramidalis hay. All groups received CS with corn meal, cottonseed meal, mineral salt specific for sheep and water ad libitum. Animals were monthly submitted to ultrasonography to detect embryonic or fetal losses. Serological tests were performed for Neospora caninum and Toxoplasma gondii before introduction of the animals in the experiment. Fetuses with malformations were evaluated in the Laboratory of Animal Anatomy and Pathology of the Academic Unit of Garanhuns (UAG), UFRPE, to describe the lesions. In G2, there were five cases of malformations of eight born lambs. In G3, there were four cases of malformation, one due to abortion. In G4, one sheep aborted and the other three gave birth to four malformed fetuses. Main malformations found in the study were arthrogryposis, palatoschisis, chylosquisis, hypoplasia of the mandible, aplasia of the incisive bone, malformations of the eye and tongue, polidactyly and monodactyly. In the groups with higher doses, larger numbers of malformations were observed, and the occurrence of placental retention and the high frequency of perinatal mortality in lambs presenting some type of malformation were noted. It has been demonstrated that P. pyramidalis is an important cause of congenital malformation, abortion and perinatal mortality in sheep, therefore keeping pregnant sheep away from this plant throughout the gestation period is utmost important.(AU)

Biomarkers and Imaging Findings of Anderson-Fabry Disease-What We Know Now.

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder, caused by deficiency or absence of the alpha-galactosidase A activity, with a consequent glycosphingolipid accumulation. Biomarkers and imaging findings may be useful for diagnosis, identification of an organ involvement, therapy monitoring and prognosis. The aim of this article is to review the current available literature on biomarkers and imaging findings of AFD patients. An extensive bibliographic review from PubMed, Medline and Clinical Key databases was performed by a group of experts from nephrology, neurology, genetics, cardiology and internal medicine, aiming for consensus. Lyso-GB3 is a valuable biomarker to establish the diagnosis. Proteinuria and creatinine are the most valuable to detect renal damage. Troponin I and high-sensitivity assays for cardiac troponin T can identify patients with cardiac lesions, but new techniques of cardiac imaging are essential to detect incipient damage. Specific cerebrovascular imaging findings are present in AFD patients. Techniques as metabolomics and proteomics have been developed in order to find an AFD fingerprint. Lyso-GB3 is important for evaluating the pathogenic mutations and monitoring the response to treatment. Many biomarkers can detect renal, cardiac and cerebrovascular involvement, but none of these have proved to be important to monitoring the response to treatment. Imaging features are preferred in order to find cardiac and cerebrovascular compromise in AFD patients.

Toluene exposure enhances acute and chronic formalin-induced nociception in rats: Participation of 5-HT3 receptors.

The purpose of this study was to evaluate the effect of acute toluene exposure on formalin (0.5% and 1%)-induced acute and long-lasting nociceptive hypersensitivity in rats. In addition, we sought to investigate the role of peripheral 5-HT3 receptors in the pronociceptive effect of toluene. Toluene exposure (6000ppm) for 30min enhanced 0.5% or 1% formalin-induced acute nociception and long-lasting secondary allodynia and hyperalgesia. In contrast, exposition to toluene for 30min in rats previously injected (six days before) with 1% formalin did not affect long-lasting hypersensitivy. Local peripheral pre-treatment with alosetron (5-HT3 receptor antagonist, 10-100 nmol) reduced the pronociceptive effect of toluene in acute nociception and long-lasting secondary allodynia and hyperalgesia. Alosetron (100nmol) was also able to reduce the nociceptive effects of 1% formalin in absence of toluene. Moreover, local peripheral injection of m-CPBG (5-HT3 receptor agonist, 300 nmol) enhanced 0.5% formalin-induced acute and long-lasting nociception in air- and toluene-exposed rats. Alosetron (10nmol) blocked the pronociceptive effects of m-CPBG (300nmol) on 0.5% formalin-induced acute and long-lasting hypersensitivity in rats exposed to toluene. Alosetron (at 10nmol) did not modify formalin-induced nociceptive behaviors. Finally, local peripheral pre-treatment with methiothepin (non-selective 5-HT receptor antagonist, 1.5nmol), did not affect the pronociceptive effect of toluene on 1% formalin-induced acute and long-lasting hypersensitivity. Our data demonstrate that acute exposure to toluene has pronociceptive effects in formalin-induced acute nociception and long-lasting hypersensitivity. Our data suggest that this pronociceptive effect depend on activation of peripheral 5-HT3, but not methiothepin-sensitive 5-HT, receptors.

Structure-activity relationship for the anticonvulsant effects of organic solvents.

Several organic solvents have anticonvulsant or convulsant actions depending on the dose and exposure time. To study if there is a structure-activity relationship for organic solvents as anticonvulsant agents we subjected independent groups of mice to a single 30-min exposure session to inhale n-hexane, cyclohexane, benzene (8000ppm each), toluene (500-6000ppm), m-xylene (1000-6000ppm), ethylbenzene (500-4000ppm) or propylbenzene (500-4000ppm). Immediately after, animals were injected i.p. with 90mg/kg pentylenetetrazol (PTZ) and re-exposed to the same solvent for another 30min. During this time, the occurrence of seizures and death was recorded. n-Hexane and cyclohexane had no anticonvulsant effect. Benzene and alkylbenzenes delayed the onset of PTZ-induced seizures. In addition, all four alkylbenzenes decreased the number of animals that seized. Propylbenzene and ethylbenzene were equally effective, but more potent than toluene and m-xylene to block PTZ actions. In the second part of the study we exposed independent groups of mice to 8000ppm n-hexane, cyclohexane (solvents without effect in the PTZ experiment), 8000ppm benzene or 6000ppm toluene, m-xylene, ethylbenzene or propylbenzene following the same experimental protocol (i.e. 30-min exposure, injection, 30-min re-exposure), but using 120mg/kg NMDA as the convulsant agent. All aromatic compounds prevented NMDA lethal effects, but only benzene and toluene decreased the percentage of animals that seized. Taken together, our data suggest that the benzene ring alone or substituted with alkyl groups is necessary for the anticonvulsant effect of acute solvent exposure against seizures and/or death produced by PTZ or NMDA.

Anderson-Fabry Disease: A Rare Disease That Mimics Common Cardiac, Neurological, Renal, and Other Disorders: Approach for the Differential Diagnosis and Follow-Up

Abstract Anderson-Fabry disease (AFD) is a rare inherited X-linked disease, caused by mutations of the gene encoding the α-galactosidase A enzyme, that leads to a deficiency or absence of its activity with consequent accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in the lysosomes of several cells types in the organism, mainly the endothelial, nervous system, cardiac, and renal cells. Its heterogeneous and nonspecific presentation, similar to other common pathologies, delays the diagnosis and leads to incorrect therapy. In the presence of attenuated phenotypes with predominant involvement of an organ, it is even harder to identify patients with AFD. It is highly important to be aware of this diagnosis, since enzyme replacement therapy is currently available. This review aims to approach the clinical manifestations of AFD and the phenotypes related to the differential diagnosis for each manifestation and the frequency of follow-up recommended.

Colangiocarcinoma em cão com metástase em omento, linfonodos regionais e pâncreas ­ Relato de caso / Cholangiocarcinoma in dog with metastatic omentum, regional lymph nodes and pancreas - Case report / Colangiocarcinoma en perro con metástasis en epiplón, ganglios linfáticos regionales y páncreas ­ Relato de caso

Os colangiocarcinomas são neoplasmas malignos originados de ductos biliares ou da vesícula biliar que apresentam como principal diagnóstico diferencial os hepatocarcinomas com padrão acinar. Relata-se aqui um caso de colangiocarcinoma de ductos biliares em um cão com metástase em omento, linfonodos regionais e pâncreas, diagnosticado no Laboratório de Patologia Veterinária da UFPR ­ Setor Palotina.

Cholangiocarcinomas are malign neoplasms originated from bile ducts or from the gallbladder that present hepatocellular carcinomas with acinar pattern as the main differential diagnostic. This work has the objective of describing a case of cholangiocarcinoma of the bile ducts in a dog with metastasis in omentum, regional lymph nodes and pancreas, diagnosed at the Veterinary Pathology Laboratory of UFPR ­ Setor Palotina.

Los colangiocarcinomas son neoplasmas malignos originados de vías biliares o de la vesícula biliar que presentan como principal diagnóstico diferencial los hepatocarcinomas con estándar acinar. Se describe un caso de colangiocarcinoma de vías biliares en un perro con metástasis en el epiplón, nódulos linfáticos regionales y páncreas, diagnosticado en el Laboratorio de Patología Veterinaria de la UFPR ­ Sector de Palotina.

Efectividad de la Medicina Natural y Tradicional en los servicios de urgencias / Effectiveness of Natural and Traditional Medicine in emergency departments

Fundamento: el uso de los medios naturales, como métodos terapéuticos en el tratamiento de las enfermedades dolorosas constituye en la actualidad un reto para las ciencias médicas. Objetivo: evaluar la efectividad de la aplicación de la Medicina Natural y Tradicional como método terapéutico en el servicio de urgencias del Hospital General Docente Mártires de Mayarí. Métodos: se realizó un estudio observacional, descriptivo, prospectivo, en pacientes con enfermedades dolorosas en la consulta de Medicina Natural y Tradicional, ubicada en el servicio de urgencias del Hospital General Docente Mártires de Mayarí del referido municipio, de octubre 2013 a marzo del 2014. El universo estuvo constituido por 180 pacientes con afecciones dolorosas que acudieron al servicio y que les fue indicado la aplicación de las diferentes modalidades de la terapéutica alternativa. La muestra no probabilística quedó constituida por 150 pacientes, que cumplieron con los criterios de inclusión establecidos para esta investigación, a los que se les aplicó tratamiento natural. Resultados: el grupo etáreo entre 50 y 60 años constituyó el de mayor incidencia, predominó el sexo masculino, el mayor porciento de pacientes pertenecen al nivel cultural obrero, solo el 20 % de la muestra tuvieron accesibilidad anterior a la terapéutica. La bursitis del hombro fue la enfermedad de mayor incidencia en tanto la moxibustión constituyó la técnica más utilizada. La mayoría de los pacientes tratados tuvieron evolución satisfactoria, su situación inicial se modificó con una única sesión de tratamiento y se recomendó la continuidad terapéutica en su área de salud. Conclusiones: la Medicina Natural y Tradicional es un método cómodo y barato que no requiere grandes recursos para su aplicación, no se reportan complicaciones durante la aplicación del tratamiento y se reduce el consumo de medicamentos al mínimo por parte de los mismos.

Background: the use of natural means, like therapeutic methods in the treatment of painful diseases, is a challenge for medical sciences nowadays. Objective: to asses the effectiveness of the application of Natural and Traditional Medicine as a therapeutic method in the emergency department of Mártires de Mayarí General Teaching Hospital, Mayarí, Holguín. Methods: an observational, descriptive, prospective study was conducted in patients with painful diseases who were seen at the Natural and Traditional Medicine consultation of the emergency department of Mártires de Mayarí General Teaching Hospital, Mayarí, Holguín from October 2013 to March 2014. The universe was composed of 180 patients with painful conditions who underwent the application of different treatments of alternative therapeutics. The non-probabilistic sample was composed of 150 patients who matched the inclusion criteria established for the research. These patients underwent natural treatment. Results: the age group between 50 and 60 years predominated as well as male sex. The greatest percent of patients belonged to the working class. Only the 20 % of the sample had had previous accessibility to the therapeutics. Shoulder bursitis was the disease with the greatest incidence, while moxibustion was the most used technique. Most patients had a satisfactory progress and got better with only one treatment session. It was recommended to continue the treatment in the health area of each patient. Conclusions: Natural and Traditional Medicine is a convenient and cheap method that does not require great resources for its application. Besides, no complications are reported during the treatments and the consumption of medicaments is minimized.

Efectividad de la Medicina Natural y Tradicional en los servicios de urgencias / Effectiveness of Natural and Traditional Medicine in emergency departments

Fundamento: el uso de los medios naturales, como métodos terapéuticos en el tratamiento de las enfermedades dolorosas constituye en la actualidad un reto para las ciencias médicas.Objetivo: evaluar la efectividad de la aplicación de la Medicina Natural y Tradicional como método terapéutico en el servicio de urgencias del Hospital General Docente Mártires de Mayarí.Métodos: se realizó un estudio observacional, descriptivo, prospectivo, en pacientes con enfermedades dolorosas en la consulta de Medicina Natural y Tradicional, ubicada en el servicio de urgencias del Hospital General Docente Mártires de Mayarí del referido municipio, de octubre 2013 a marzo del 2014. El universo estuvo constituido por 180 pacientes con afecciones dolorosas que acudieron al servicio y que les fue indicado la aplicación de las diferentes modalidades de la terapéutica alternativa. La muestra no probabilística quedó constituida por 150 pacientes, que cumplieron con los criterios de inclusión establecidos para esta investigación, a los que se les aplicó tratamiento natural. Resultados: el grupo etáreo entre 50 y 60 años constituyó el de mayor incidencia, predominó el sexo masculino, el mayor porciento de pacientes pertenecen al nivel cultural obrero, solo el 20 porciento de la muestra tuvieron accesibilidad anterior a la terapéutica. La bursitis del hombro fue la enfermedad de mayor incidencia en tanto la moxibustión constituyó la técnica más utilizada. La mayoría de los pacientes tratados tuvieron evolución satisfactoria, su situación inicial se modificó con una única sesión de tratamiento y se recomendó la continuidad terapéutica en su área de salud.Conclusiones: la Medicina Natural y Tradicional es un método cómodo y barato que no requiere grandes recursos para su aplicación, no se reportan complicaciones durante la aplicación del tratamiento y se reduce el consumo de medicamentos al mínimo por parte de los mismos (AU)

Background: the use of natural means, like therapeutic methods in the treatment of painful diseases, is a challenge for medical sciences nowadays.Objective: to asses the effectiveness of the application of Natural and Traditional Medicine as a therapeutic method in the emergency department of Mártires de Mayarí General Teaching Hospital, Mayarí, Holguín.Methods: an observational, descriptive, prospective study was conducted in patients with painful diseases who were seen at the Natural and Traditional Medicine consultation of the emergency department of Mártires de Mayarí General Teaching Hospital, Mayarí, Holguín from October 2013 to March 2014. The universe was composed of 180 patients with painful conditions who underwent the application of different treatments of alternative therapeutics. The non-probabilistic sample was composed of 150 patients who matched the inclusion criteria established for the research. These patients underwent natural treatment.Results: the age group between 50 and 60 years predominated as well as male sex. The greatest percent of patients belonged to the working class. Only the 20 percent of the sample had had previous accessibility to the therapeutics. Shoulder bursitis was the disease with the greatest incidence, while moxibustion was the most used technique. Most patients had a satisfactory progress and got better with only one treatment session. It was recommended to continue the treatment in the health area of each patient.Conclusions: Natural and Traditional Medicine is a convenient and cheap method that does not require great resources for its application. Besides, no complications are reported during the treatments and the consumption of medicaments is minimized (AU)

Knowledge and Practices of Pediatricians Regarding Functional Constipation in the State of Minas Gerais, Brazil.

OBJECTIVES: The aim of the study was to analyze the knowledge, approaches, and practices of pediatricians participating in a regional pediatric conference regarding functional constipation (FC) to identify knowledge gaps. METHODS: Cross-sectional regional study based on a semistructured questionnaire related to the Rome III criteria and the participant profiles. RESULTS: Of 400 questionnaires distributed, 264 (66.0%) were completed. Most pediatricians (76.7%) did not recognize the Rome III criteria, but the majority did recognize the following risk factors for FC: low-fiber diet (97.8%), family history (86.6%), onset of toilet training (76.4%), and weaning (62.4%). Foods considered high in fiber were oats (95.7%), leafy vegetables (95.3%), and wheat bran (93.2%). Digital rectal examination was not performed by 57.7% of nongastroenterologists. Complementary examinations were ordered in 27.5% and 72.5% of patients by general pediatricians and pediatric gastroenterologists, respectively, although the sample of gastroenterologists was not representative. The most prescribed drugs by nongastroenterologists were mineral oil (72.6%), magnesium hydroxide (52.1%), lactulose (41.0%), and polyethylene glycol (25.2%). Pediatric gastroenterologists prescribed magnesium hydroxide (91.7%), polyethylene glycol (91.7%), and mineral oil (58.3%). Most pediatricians (70.0%) considered the average treatment duration to be less than 1 year, although gastroenterologists considered it to be more than 1 year (54.6%). CONCLUSIONS: The knowledge of pediatricians regarding FC is inadequate. Constipation may not receive the public health attention it deserves within pediatric residency programs. The authors suggest that better education regarding FC should be included in medical school and residency program curricula to improve patient care.

Combination of diacerhein and antiepileptic drugs after local peripheral, and oral administration in the rat formalin test.

Preclinical Research The present study was designed to evaluate the possible antinociceptive interaction between diacerhein and some antiepileptic drugs (carbamazepine, topiramate and gabapentin) on formalin-induced nociception. Diacerhein, each of the antiepileptics or a fixed dose-ratio combination of these drugs was assessed after local peripheral and oral administration in rats. lsobolographic analyses were used to define the interaction between drugs. Diacerhein, antiepileptic drugs (carbamazepine, topiramate and gabapentin) or their combinations yielded a dose-dependent antinociceptive effect when administered by both routes. Theoretical ED30 values for the combination estimated from the isobolograms were obtained as follows: diacerhein-carbamazepine (85.99 ± 7.07 µg/paw; 56.53 ± 4.56 mg/kg po), diacerhein-topiramate (197.97 ± 22.90 µg/paw; 13.06 ± 2.44 mg/kg po) and diacerhein-gabapentin (96.87 ± 17.73 µg/paw; 17.90 ± 4.70 mg/kg p.o.) for the local peripheral and oral administration routes, respectively. These values were significantly higher than the experimentally obtained ED30 values: diacerhein-carbamazepine (49.33 ± 3.37 µg/paw; 35.49 ± 7.91 mg/kg po), diacerhein-topiramate (133.00 ± 39.10 µg/paw; 8.87 ± 1.46 mg/kg po) and diacerhein-gabapentin (70.98 ± 14.73 µg/paw; 10.95 ± 3.23 mg/kg po). The combinations produced their antinociceptive effects without motor impairment in the rotarod test indicating synergistic interactions with a good side effect profile.

Doença renal policística em bezerro / Polycystic kidney disease in a calf

This report describes a case of polycystic kidney disease in a male, mixed breed bovine aged one year. On clinical examination revealed a poor nutritional status, pallid mucous membranes, moderated dehydration (8 to 10%), ulcers on the ventral portion of the tongue, multifocal areas of hypotrichosis covered with scabs throughout the entire body and permanent sternolateral decubitus. The blood exam revealed aregenerative anemia and leukocytosis (neutrophilia) with deviation to the left. The necropsy revealed enlarged, pallid kidneys with a finely irregular natural surface. The histological analysis revealed the nearly complete replacement of the renal cortex by numerous small cysts, distension of the Bowman space, tubular regeneration, fibrosis, edema and mild interstitial lymphoplasmacytic inflammatory infiltrate. Keywords: diseases of cattle, kidney disease, lesions uremia, nephropathy

Waist-to-height ratio: an accurate anthropometric index of abdominal adiposity and a predictor of high HOMA-IR values in nondialyzed chronic kidney disease patients.

OBJECTIVE: Chronic kidney disease (CKD) is associated with metabolic disorders, including insulin resistance (IR), mainly when associated with obesity and characterized by high abdominal adiposity (AbAd). Anthropometric measures are recommended for assessing AbAd in clinical settings, but their accuracies need to be evaluated. The aim of this study was to evaluate the precision of different anthropometric measures of AbAd in patients with CKD. We also sought to determine the AbAd association with high homeostasis model assessment index of insulin resistance (HOMA-IR) values and the cutoff point for AbAd index to predict high HOMA-IR values. METHODS: A subset of clinically stable nondialyzed patients with CKD followed at a multidisciplinary outpatient clinic was enrolled in this cross-sectional study. The accuracy of the following anthropometric indices: waist circumference, waist-to-hip ratio, conicity index and waist-to-height ratio (WheiR) to assess AbAd, was evaluated using trunk fat, by dual x-ray absorptiometry (DXA), as a reference method. HOMA-IR was estimated to stratify patients in high and low HOMA-IR groups. The total area under the receiver-operating characteristic curves (AUC-ROC; sensitivity/specificity) was calculated: AbAd with high HOMA-IR values (95% confidence interval [CI]). RESULTS: We studied 134 patients (55% males; 54% overweight/obese, body mass index ≥ 25 kg/m(2), age 64.9 ± 12.5 y, estimated glomerular filtration rate 29.0 ± 12.7 mL/min). Among studied AbAd indices, WheiR was the only one to show correlation with DXA trunk fat after adjusting for confounders (P < 0.0001). Thus, WheiR was used to evaluate the association between AbAd with HOMA-IR values (r = 0.47; P < 0.0001). The cutoff point for WheiR as a predictor for high HOMA-IR values was 0.55 (AUC-ROC = 0.69 ± 0.05; 95% CI, 0.60-0.77; sensitivity/specificity, 68.9/61.9). CONCLUSIONS: WheiR is recommended as an effective and precise anthropometric index to assess AbAd and to predict high HOMA-IR values in nondialyzed patients with CKD.

Chronic functional constipation in children: adherence and factors associated with drug treatment.

OBJECTIVE: The aim of the present study was to evaluate the treatment adherence of children with chronic functional constipation. METHODS: The present study is a prospective and longitudinal study realized at a pediatric gastroenterology clinic of a Brazilian University Hospital, between August 2009 and October 2011. Rome III criteria and the Bristol Stool Scale were used to define constipation and to characterize feces, respectively. Drug treatment was prescribed for patients according to the protocols previously standardized in the clinic. Specific questionnaires, containing questions related to 1 dependent variable and independent variables were completed in the first and sixth months of the treatment. Independent variables related to the patients, their caregivers, the disease itself, and the therapeutic plan were analyzed and compared with the dependent variable (adherence to the treatment). Adherence was considered when the patient returned with >75% of the prescribed medicine containers empty. RESULTS: Fifty children participated in both the first and sixth months of treatment. The mean age of the sample was 77.6 ± 43.8 months and the mean age of the onset of symptoms was 18.8 ± 27.9 months. The adherence rate was 38% in the first month and 30% in the sixth month. Patients who were treated with polyethylene glycol had greater adherence than patients who were prescribed other laxatives, with statistical significance in the second moment of the study (P = 0.19 and P = 0.04, respectively). CONCLUSIONS: The study showed low adherence rates to drug treatment of constipation in children. It is necessary to seek new strategies to increase treatment adherence, while avoiding complications and reducing costs.

Adiposity and cardiovascular disease risk factors in renal transplant recipients: Are there differences between sexes?

OBJECTIVE: The aim of this study was to evaluate high body adiposity and cardiovascular disease (CVD) risk factors prevalence, in renal transplant recipients (RTR), comparing men with women. METHODS: In this retrospective cross-sectional study, 102 patients (55 men) who were 49 ± 1.2 y and 114.3 ± 9 mo post-transplant (post-tx) were evaluated. Pretransplant (pre-tx) period data and weight gain during the first year post-tx were obtained from patient charts and post-tx data were collected during a routine visit at nephrology clinic. Body mass index (BMI) ≥ 25 kg/m(2) defined overweight and BMI ≥ 30 kg/m(2) defined obesity. RESULTS: Pre-tx overweight prevalence was low and similar between men and women (26%), whereas only women showed obesity (11%). Post-tx body weight increased significantly in the entire group, leading to an increase in overweight (to 38% in men and 51% in women) and obesity (to 11% in men and 23% in women) prevalence. Post-tx comparisons between men and women showed that women had higher (women versus men; P < 0.05) BMI values (26.7 ± 0.8 versus 24.7 ± 0.5 kg/m(2)), weight gain during first year post-tx (9.2 ± 1.1 versus 5.5 ± 1 kg), abdominal obesity (57% versus 23%) and diabetes (34% versus 16%) prevalence. The associations between adiposity and CVD risk factors showed that pre-tx overweight increased the risk for diabetes in post-tx; pos-tx high BMI and abdominal obesity increased the risk for metabolic syndrome; abdominal obesity increased the risk for dyslipidemia in women. CONCLUSIONS: High body adiposity prevalence was high after transplantation and increased the risk for metabolic syndrome, an important CVD risk factor. Women showed higher total body adiposity values, abdominal obesity, and diabetes prevalence than men. Abdominal obesity increased the risk for dyslipidemia in women.

Adesão ao tratamento de doenças crônicas em pediatria: uma revisão crítica da literatura / Adherence to treatment of chronic diseases in pediatrics: a critical literature review

O objetivo do estudo é apresentar revisão da literatura sobre os fatores relacionados à adesão e à não adesão a medicamentos prescritos para pacientes portadores de doenças crônicas prevalentes na faixa etária pediátrica. Foi realizada revisão crítica da literatura, tendo sido selecionados artigos referentes à não adesão, publicados nas bases de dados Medline e Lilacs, no período de 1999 a 2011. A porcentagem de adesão a diversos tipos de tratamento é variável, sendo encontrados dados na literatura, em relação a doenças crônicas, de 4,6 a 100% de adesão, podendo ser atribuída à dificuldade de padronização da definição de critérios utilizados para a avaliação, bem como a diversidade das amostras dos estudos. Vale destacar que a resposta a um tratamento vai além da sua eficácia clínica, o que impõe o conceito de não adesão. Adesão ao tratamento medicamentoso em doenças crônicas é tema complexo. Os profissionais de saúde devem estar atentos aos pacientes que não estão respondendo ao tratamento, reduzindo, desta forma, sequelas e comorbidades, assim como custos adicionais em saúde...

The aims of this study was to present a review of the literature on the factors related to adherence and no adherence to medications prescribed to patients with chronic diseases prevalent in the pediatric population. We performed a critical review of the literature, having been selected articles from non-compliance, published in Medline and Lilacs in the period 1999 to2011. The percentages of adherence to various types of treatment is variable, and find data in the literature in relation to chronic diseases from 4.6 to 100% compliance, which can be attributed to the difficulty in standardizing the definition of criteria used for evaluation, and the diversity of the study samples. Note that the response to treatment goes beyond the clinical efficacy, which imposes the concept of non-compliance. Adherence in chronic diseases is a complex issue. Health professionals should be alert to patients who are not responding totreatment, thus reducing sequel and comorbidities, as well as additional costs in health...