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1.
Nutrients ; 13(8)2021 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-34445041

RESUMO

The benefits of breastfeeding (BF) include risk reduction of later overweight and obesity. We aimed to analyse the association between breastfeeding practices and overweight/obesity among preschool children participating in the ToyBox study. Data from children in the six countries, participating in the ToyBox-study (Belgium, Bulgaria, Germany, Greece, Poland, and Spain) 7554 children/families and their age is 3.5-5.5 years, 51.9% were boys collected cross-sectionally in 2012. The questionnaires included parents' self-reported data on their weight, height, socio-demographic status, and infant feeding practices. Measurements of preschool children's weight and height were done by trained researchers using standard protocols and equipment. The ever breastfeeding rate in the total sample was 85.0% (n = 5777). Only 6.3% (n = 428) of the children from the general sample were exclusively breastfed (EBF) for the duration of the first six months. EBF for four to six months was significantly (p < 0.001) less likely among mothers with formal education < 12 years (adjusted Odds Ratio (OR) = 0.61; 95% Confidence interval (CI) 0.44-0.85), smoking throughout pregnancy (adjusted OR = 0.39; 95% CI 0.24-0.62), overweight before pregnancy (adjusted OR = 0.67; 95%CI 0.47-0.95) and ≤25 years old. The median duration of any breastfeeding was five months. The prevalence of exclusive formula feeding during the first five months in the general sample was about 12% (n = 830). The prevalence of overweight and obesity at preschool age was 8.0% (n = 542) and 2.8% (n = 190), respectively. The study did not identify any significant association between breastfeeding practices and obesity in childhood when adjusted for relevant confounding factors (p > 0.05). It is likely that sociodemographic and lifestyle factors associated with breastfeeding practices may have an impact on childhood obesity. The identified lower than desirable rates and duration of breastfeeding practices should prompt enhanced efforts for effective promotion, protection, and support of breastfeeding across Europe, and in particular in regions with low BF rates.


Assuntos
Aleitamento Materno , Obesidade Pediátrica/epidemiologia , Fatores Etários , Índice de Massa Corporal , Pré-Escolar , Estudos Transversais , Europa (Continente)/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Estilo de Vida , Masculino , Obesidade Pediátrica/diagnóstico , Obesidade Pediátrica/prevenção & controle , Prevalência , Fatores de Proteção , Medição de Risco , Fatores de Risco , Determinantes Sociais da Saúde , Fatores Socioeconômicos , Fatores de Tempo
2.
Lancet Gastroenterol Hepatol ; 6(10): 864-873, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34364544

RESUMO

The term non-alcoholic fatty liver disease (NAFLD), and its definition, have limitations for both adults and children. The definition is most problematic for children, for whom alcohol consumption is usually not a concern. This problematic definition has prompted a consensus to rename and redefine adult NAFLD associated with metabolic dysregulation to metabolic (dysfunction)-associated fatty liver disease (MAFLD). Similarities, distinctions, and differences exist in the causes, natural history, and prognosis of fatty liver diseases in children compared with adults. In this Viewpoint we, an international panel, propose an overarching framework for paediatric fatty liver diseases and an age-appropriate MAFLD definition based on sex and age percentiles. The framework recognises the possibility of other coexisting systemic fatty liver diseases in children. The new MAFLD diagnostic criteria provide paediatricians with a conceptual scaffold for disease diagnosis, risk stratification, and improved clinical and multidisciplinary care, and they align with a definition that is valid across the lifespan.


Assuntos
Síndrome Metabólica/complicações , Síndrome Metabólica/fisiopatologia , Hepatopatia Gordurosa não Alcoólica/diagnóstico , Hepatopatia Gordurosa não Alcoólica/etiologia , Adolescente , Adulto , Criança , Consenso , Progressão da Doença , Feminino , Humanos , Resistência à Insulina/fisiologia , Comunicação Interdisciplinar , Masculino , Síndrome Metabólica/epidemiologia , Hepatopatia Gordurosa não Alcoólica/epidemiologia , Hepatopatia Gordurosa não Alcoólica/patologia , Obesidade/complicações , Obesidade/epidemiologia , Prevalência , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Fatores de Risco , Índice de Gravidade de Doença
3.
Metab Brain Dis ; 36(7): 2169-2172, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34427841

RESUMO

Biallelic pathogenic variants in the neuroblastoma amplified sequence (NBAS) gene were firstly (2015) identified as a cause of fever-triggered recurrent acute liver failure (RALF). Since then, some patients with NBAS deficiency presenting with neurologic features, including a motor delay, intellectual disability, muscular hypotonia and a mild brain atrophy, have been reported. Here, we describe a case of pediatric patient diagnosed with NBAS deficiency due to a homozygous c.2809C > G, p.(Pro937Ala) variant presenting with RALF with severe hyperammonemia, acquired microcephaly and progressive brain atrophy. Not reported in the literature findings include severe hyperammonemia during ALF episode, and neurologic features in the form of acquired progressive microcephaly with brain atrophy. The latter raises the hypothesis about a primary neurologic phenotype in NBAS deficiency.

4.
Am J Case Rep ; 22: e932374, 2021 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-34283821

RESUMO

BACKGROUND Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). The role and timing of LTx in PFIC-1 remains debated. We present 2 case reports of male siblings with PFIC-1 who benefited from different treatments. CASE REPORT Both siblings harbored a homozygous truncating mutation in ATP8B1 characteristic for PFIC-1 and both underwent PEBD after unsuccessful UDCA treatment at the age of 7 and 5 months, respectively. The older brother, after initial improvement of symptoms, developed severe pruritus, cholestasis, and diarrhea 9 months after PEBD and underwent LTx at the age of 16 months. Chronic diarrhea and abnormal transaminases activity appeared soon after transplantation. A liver biopsy was performed 3 months after LTx and showed severe macrovesicular steatosis (95%). Sixteen months after LTx, total biliary diversion was performed, with rapid relief from diarrhea and significant regression of graft steatosis by <30%. In his brother we observed persistent severe pruritus and cholestasis after PEBD, but we decided to postpone LTx due to lack of a living related donor and risk of graft steatosis. Eight months after PEBD, bilirubin and bile acids significantly decreased and pruritus disappeared completely. Currently, in 5-year follow-up, liver function is stable and he has no pruritus. CONCLUSIONS The good effect of PEBD may be delayed in PFIC-1, even in severe mutation; thus, the decision to perform LTx should be made cautiously. Total biliary diversion is an efficient procedure in case of persistent symptoms after LTx and can reverse graft steatosis in children with PFIC-1.


Assuntos
Colestase Intra-Hepática , Colestase , Transplante de Fígado , Adenosina Trifosfatases , Criança , Colestase Intra-Hepática/genética , Colestase Intra-Hepática/cirurgia , Humanos , Lactente , Masculino , Mutação , Irmãos , Resultado do Tratamento
5.
Clin Res Hepatol Gastroenterol ; 45(6): 101753, 2021 Jul 24.
Artigo em Inglês | MEDLINE | ID: mdl-34311135

RESUMO

BACKGROUND: The role of microbiota in biliary atresia (BA) remains unclear. The aim of our study was to assess efficacy and safety of LGG treatment in children with BA after HPE with special focus on bacterial cholangitis (BCH) and quantitative assessment of the gut microbiota composition and metabolism. METHODS: We performed double-blind placebo controlled trial with patients randomized into treatment group who received LGG (n = 14) and placebo (n = 16). The gut microbiota and short-chain fatty acids (SCFA) were assessed at baseline and after 6 months of treatment. Clinical and laboratory parameters including episodes of bacterial cholangitis (BCH) were collected during the study period and after 2-year follow-up. Additionally, stool composition of BA patients was compared with healthy age-matched control group. RESULTS: There were lower concentration of SCFA in children with BA compared to control group and significant increase in the number of Enterococcus bacteria. After 6 months of treatment, neither laboratory parameters nor gut microbiota composition differed between LGG group and placebo. PP analysis results were similar to ITT analysis, no significant differences between study and control group. Overall, there were 11 (36%) patients who developed at least one episode of bacterial cholangitis; 3 (21%) in the LGG group compared to 8 (50%) placebo group (p = 0.14). Bacterial cultures were positive in 22% of cases and recurrence after the first episode was observed in 27% of patients. The level of total bilirubin decreased below 2 mg/dl after 6 months of the study in 6 (42.8%) patients in the LGG group and in 8 (50%) patients in the placebo group (p = 0.73). During 2-year follow-up 6 out of 14 patients (42.8%) in the LGG group and 11 out of 16 placebo patients (68.7%) underwent liver transplantation (p = 0.27). CONCLUSIONS: Patients with BA present with specific microbiota profiles and decreased SCFA what gives opportunities to implement novel therapeutic options based on modulation of  microbiota. Whether LGG is an effective therapy needs to be studied in a larger group with similar outcome parameters.

6.
Sci Rep ; 11(1): 15261, 2021 07 27.
Artigo em Inglês | MEDLINE | ID: mdl-34315985

RESUMO

Autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC) are two very closely related autoimmune liver diseases with overlapping clinical features and similar management strategies. The purpose of this study was to assess the utility of quantitative imaging markers to distinguish ASC from AIH in paediatrics. 66 participants (N = 52 AIH, N = 14 ASC) aged 14.4 ± 3.3 years scheduled to undergo routine biopsy and baseline serum liver biochemistry testing were invited to undergo MRI (non-contrast abdominal MRI and 3D fast spin-echo MRCP). Multiparametric MRI was used to measure fibro-inflammation with corrected T1 (cT1), while the biliary tree was modelled   using quantitative MRCP (MRCP +). Mann-Whitney U tests were performed to compare liver function tests with imaging markers between patient groups (ASC vs AIH). Receiver operating characteristic curves and stepwise logistic regressions were used to identify the best combination of markers to discriminate between ASC and AIH. Correlations between liver function tests and imaging markers were performed using Spearman's rank correlation. cT1 was significantly correlated with liver function tests (range 0.33 ≤ R ≤ 56, p < 0.05), as well as with fibrosis, lobular and portal inflammation (range 0.31 ≤ R ≤ 42, p < 0.05). 19 MRCP + metrics correlated significantly with liver function tests (range 0.29 ≤ R ≤ 0.43, p < 0.05). GGT and MRCP + metrics were significantly higher in ASC compared to those with AIH. The best multivariable model for distinguishing ASC from AIH included total number of ducts and the sum of relative severity of both strictures and dilatations AUC: 0.91 (95% CI 0.78-1). Quantitative MRCP metrics are a good discriminator of ASC from AIH.

7.
Rocz Panstw Zakl Hig ; 72(2): 209-220, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34114781

RESUMO

During the COVID-19 pandemic, care for an adequate diet, well adapted to the body's needs and the current level of physical activity, becomes of particular importance. Many dietary compounds participate in the functioning of the immune system, while vitamins D, C, A (including beta-carotene), E, B6, B12, folic acid, zinc, copper, selenium, iron, amino acids, n-3 and n-6 polyunsaturated fatty acids and intestinal microbiota are crucial in various types of defence processes. There has been no evidence that consumed food and its compounds, including those with pro-/prebiotic properties, play a significant role in preventing SARS-CoV-2 infection or alleviating its course. However, in terms of the nutritional value of food and the prevention of dysbiosis, recommending a varied diet with a high proportion of plant-based foods and an adequate amount of animal-based foods has a sound scientific basis. Malnutrition, underweight and obesity are considered independent and prognostic risk factors of severe SARS-CoV-2 infection, which reduce a patient's chances of survival. Therefore, ensuring good nutritional status, including healthy body weight, is a reasonable approach in the prevention of viral infection SARS-CoV-2 or alleviating its course. The document is accompanied by two catalogues of practical nutritional recommendations during the COVID-19 pandemic, addressed to the general population and children.


Assuntos
Dieta Saudável/estatística & dados numéricos , Promoção da Saúde/normas , Estado Nutricional , Recomendações Nutricionais , Sociedades Médicas/normas , Academias e Institutos/normas , Adulto , COVID-19 , Criança , Suplementos Nutricionais/estatística & dados numéricos , Humanos , Fenômenos Fisiológicos da Nutrição , Valor Nutritivo , Polônia , Saúde Pública , Oligoelementos/uso terapêutico
8.
J Pediatr Gastroenterol Nutr ; 73(4): 455-462, 2021 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-33908738

RESUMO

OBJECTIVES: Wilson disease (WD) is a copper metabolism disorder with toxic copper accumulation in the liver leading to liver steatosis or fibrosis. In vitro studies suggest that fatty acid-binding protein 1 (L-FABP) and lipid droplet-associated protein 5 (PLIN5) may have an impact on both processes, but knowledge about these potential biomarkers is insufficient in the case of WD. Thus, the aim of this study was to determine L-FABP and PLIN5 levels in sera of WD patients in relation to liver steatosis/fibrosis. METHODS: The final study involved 74 WD children in whom liver steatosis (WD1 subgroup, n = 28) and fibrosis (WD2 subgroup, n = 13) were assessed with the use of transient elastography. Control groups included WD children without steatosis and fibrosis (WD0 subgroup, n = 33) and healthy children (n = 75). L-FABP and PLIN5 measurements were performed in sera with the use of the immunoenzymatic method. RESULTS: L-FABP was significantly higher in the WD2 subgroup, and the correlation between L-FABP concentration and liver fibrosis was confirmed statistically by regression analysis (P = 0.04) with Pearson's coefficient r = 0.24. L-FABP was significantly correlated with alanine aminotransferase (r = 0.42) and aspartate aminotransferase (r = 0.37) activity. PLIN5 concentration was similar in all groups and was not related to steatosis and fibrosis. CONCLUSIONS: Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.

9.
Nutrients ; 13(4)2021 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-33916419

RESUMO

Complementary feeding (CF) should start between 4-6 months of age to ensure infants' growth but is also linked to childhood obesity. This study aimed to investigate the association of the timing of CF, breastfeeding and overweight in preschool children. Infant-feeding practices were self-reported in 2012 via a validated questionnaire by >7500 parents from six European countries participating in the ToyBox-study. The proportion of children who received breast milk and CF at 4-6 months was 51.2%. There was a positive association between timing of solid food (SF) introduction and duration of breastfeeding, as well as socioeconomic status and a negative association with smoking throughout pregnancy (p < 0.005). No significant risk to become overweight was observed among preschoolers who were introduced to SF at 1-3 months of age compared to those introduced at 4-6 months regardless of the type of milk feeding. Similarly, no significant association was observed between the early introduction of SF and risk for overweight in preschoolers who were breastfed for ≥4 months or were formula-fed. The study did not identify any significant association between the timing of introducing SF and obesity in childhood. It is likely that other factors than timing of SF introduction may have impact on childhood obesity.


Assuntos
Alimentação Artificial/estatística & dados numéricos , Aleitamento Materno/estatística & dados numéricos , Sobrepeso/epidemiologia , Obesidade Pediátrica/epidemiologia , Peso ao Nascer , Pré-Escolar , Europa (Continente)/epidemiologia , Feminino , Humanos , Masculino , Exposição Materna/estatística & dados numéricos , Sobrepeso/etiologia , Sobrepeso/prevenção & controle , Obesidade Pediátrica/etiologia , Obesidade Pediátrica/prevenção & controle , Fatores de Risco , Autorrelato/estatística & dados numéricos , Fumar/epidemiologia , Classe Social , Fatores de Tempo
10.
Pediatr Neonatol ; 62(3): 278-283, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33663989

RESUMO

BACKGROUND: Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and their attachment to proteins and lipids. Histologically, liver steatosis, fibrosis and cirrhosis have been reported in CDG. The aim of the study was to characterize the histopathological and ultrastructural liver changes in CDG patients hospitalized in our Institute, and to find the most characteristic features, as articles concerning the liver microscopic features in CDG are sparse. METHODS: Out of 32 CDG patients diagnosed and followed-up in our Institute, the liver biopsy was performed in 4 of them, including 2 with MPI-CDG, 1 with SRD5A3-CDG, and 1 with PGM1-CDG, as a part of diagnostic process. In one patient, diagnosed post mortem with PMM2-CDG, the histopathological study comprised liver autopsy samples. RESULTS: The most common histopathological liver finding was the presence of steatosis (4/5) of varying severity, the mixed macro- and microvesicular type as well as the foamy degeneration of hepatocytes. In two patients, liver steatosis was associated with fibrosis, stage 4 (cirrhosis) and 2 according to Batts and Ludwig classification, respectively. In two patients, besides steatosis, mild inflammatory infiltrates composed of lymphoid cells in portal tracts were observed. No correlation between the patient's age and histopathological features was observed. CONCLUSIONS: The histopathological changes in the liver of CDG patients are miscellaneous; thus, based on the microscopic examination only, we can not identify (even suspect) the exact CDG. The most common histopathologic finding in our cohort of CDG patients was the presence of liver steatosis (of various severity) and foamy degeneration of hepatocytes.

11.
Orphanet J Rare Dis ; 16(1): 17, 2021 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407696

RESUMO

BACKGROUND: Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and the attachment of glycans to proteins and lipids. Our study aimed to describe the clinical, biochemical, and molecular findings of CDG patients, and to present the long-term follow-up. MATERIAL AND METHODS: A single-center study (1995-2019 years) of patients with congenital disorders of N-glycosylation and combined N- and O-hypoglycosylation was performed. RESULTS: Among 32 patients included into the study, there were 12 PMM2-CDG, 3 ALG13-CDG, 3 ALG1-CDG, 1 ALG3-CDG, 3 MPI-CDG, 1 PGM1-CDG, 4 SRD5A3-CDG, 1 DPAGT1-CDG, 3 ATP6AP1-CDG, 1 ATP6V0A2-CDG. The phenotypic and genotypic spectrum during long-term (in some cases over 20 years) observation was characterised and several measurements of serum Tf isoforms taken. Statistical analysis revealed strong negative correlation between asialo-Tf and tetrasialo-Tf, as well as between disialo-Tf and tetrasialo-Tf. Within CDG type I, no difference in % Tf isoforms was revealed between PMM2-CDG and non-PMM2-CDG patients. However, these two groups differed significantly in such diagnostic features as: cerebellar ataxia, failure to thrive, hypothyroidism, pericardial effusion, cardiomyopathy, inverted nipples, prolonged INR. The effect of treatment with mannose in 2 patients with MPI-CDG was assessed and we found that % of asialo-Tf, monosialo-Tf, and disialo-Tf was significantly lowered, whereas tetrasialo-Tf and pentasialo-Tf rose, coming closer or falling into the reference range. CONCLUSIONS: The novel finding was an abnormal Tf IEF pattern in two ALG13-CDG patients and normal in one ALG1-CDG patient. Clinical manifestation of presented CDG patients was similar to that reported in the literature. Mannose supplementation in MPI-CDG patients, as well as galactose supplementation in PGM1-CDG patient, improved patients' clinical picture and Tf isoform profiles.


Assuntos
Defeitos Congênitos da Glicosilação , ATPases Vacuolares Próton-Translocadoras , Defeitos Congênitos da Glicosilação/genética , Seguimentos , Genótipo , Glicosilação , Humanos , Manosiltransferases , Fenótipo
12.
J Pediatr Gastroenterol Nutr ; 72(1): 108-114, 2021 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-32925554

RESUMO

OBJECTIVES: Autoimmune hepatitis (AIH) is a progressive liver disease managed with corticosteroids and immunosuppression and monitored using a combination of liver biochemistry and histology. However, liver biopsy is invasive with risk of pain and bleeding. The aim of the present study was to investigate the utility of noninvasive imaging with multiparametric magnetic resonance imaging (MRI) (mpMRI) to provide clinically useful information on the presence and extent of hepatic inflammation, potentially guiding immunosuppression. METHODS: Eighty-one participants (aged 6-18), 21 healthy and 60 AIH patients, underwent multiparametric MRI to measure fibro-inflammation with iron-corrected T1 (cT1) at the Children's Memorial Health Institute in Warsaw alongside other clinical blood tests and liver biopsy at recruitment and after an average of 16-month follow-up (range 9-22 months). Correlation analyses were used to investigate the associations between cT1 with blood serum markers and histological scores. RESULTS: At recruitment, patients with AIH had a higher cT1 value than healthy controls (P < 0.01). cT1 correlated significantly with key histopathological features of disease. Treatment naïve AIH patients showed evidence of inflammation and heterogeneity across the liver compared to healthy controls.At follow-up, cT1 showed utility in monitoring disease regression as most patients showed significantly reduced fibro-inflammation with treatment (P < 0.0001) over the observational period. Six patients had histological fibrosis and clear fibro-inflammation on MR despite biochemical remission (normalized aspartate aminotransferase (AST), alanine aminotransferase (ALT), and immunoglobulin G [IgG]). CONCLUSIONS: Multiparametric MRI can measure disease burden in pediatric AIH and can show changes over time in response to therapy. Active disease can be seen even in biochemical remission in children.


Assuntos
Hepatite Autoimune , Imageamento por Ressonância Magnética Multiparamétrica , Alanina Transaminase , Aspartato Aminotransferases , Criança , Hepatite Autoimune/diagnóstico por imagem , Humanos
13.
Clin Res Hepatol Gastroenterol ; 45(1): 101408, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32278775

RESUMO

BACKGROUND: Deoxyguanosine kinase (DGUOK) deficiency is one of the causes of the hepatocerebral form of mitochondrial depletion syndrome (MDS). It is characterized by an early onset of liver failure with concomitant neurological deterioration. In the current literature, there are only few reports regarding long-term observation of children with DGUOK deficiency. Liver transplantation (LTx) is controversial due to extrahepatic involvement and unpredictable outcome. METHODS: Five patients (2 boys) from 4 different families with hepatocerebral MDS associated with DGUOK mutations diagnosed with liver failure were treated in our hospital between 2010-2019. RESULTS: In all children clinical symptoms developed within the first days of live and hypoglycemia (hypoketotic), conjugated hyperbilirubinemia (cholestasis), severe lactic acidosis, and coagulopathy were observed. Two neonates had low birth-weight for gestational age and failed to thrive. Mild neurological involvement as hypotonia was observed in all children. Three children died at the age of 2, 6 months and 6,5 months of age, respectively, due to end-stage liver failure. In one case, LTx was not considered, in two patients (sisters) parents did not agree to this procedure. LTx was subsequently performed in two patients at the age of 6 and 7 months, respectively, one from deceased, and one from living related donor, in both before the final confirmation of DGUOK mutations. One boy died 2 months after LTx due to post-LTx procedure-related complications; one is still alive with 3years of follow-up, with good liver function and mild neurological disturbances. The diagnosis of DGUOK deficiency was confirmed by biallelic DGUOK mutations detection. Equally, patients were compound heterozygotes (three cases) and homozygotes (two cases). Three known molecular variants, including regulatory substitutions (c.1A>G, c.3G>A) and in-frame insertion (c.813_814insTTT) were identified. CONCLUSIONS: Prognosis in patients with DGUOK deficiency is generally poor. Based on a review of the literature and our experience liver transplantation in selected patients with DGUOK mutation does not appear to be contraindicated, especially in those without or with minimal neurologic abnormalities.

14.
Artigo em Alemão | MEDLINE | ID: mdl-33276394

RESUMO

A male dog with benign prostatic hyperplasia and several small intraprostatic cysts was treated with a GnRH-agonist implant containing 4,7 mg deslorelin (Suprelorin®). Within 2 weeks after the implantation, the prior urethral bleeding worsened. A large intraprostatic cyst was detected sonographically. The patient was subsequently treated with osaterone acetate (0.4 mg/kg p. o. once a day for 7 days) and enrofloxacin (5 mg/kg p. o. once a day for 21 days). The clinical symptoms receded within 10 days. Within one month, the cyst regressed completely. The mechanisms of cyst enlargement are discussed.


Assuntos
Cistos , Doenças do Cão , Implantes de Medicamento/efeitos adversos , Hormônio Liberador de Gonadotropina/agonistas , Hiperplasia Prostática , Animais , Cistos/induzido quimicamente , Cistos/tratamento farmacológico , Cistos/veterinária , Doenças do Cão/induzido quimicamente , Doenças do Cão/tratamento farmacológico , Doenças do Cão/patologia , Cães , Implantes de Medicamento/uso terapêutico , Masculino , Próstata/patologia , Doenças Prostáticas/induzido quimicamente , Doenças Prostáticas/tratamento farmacológico , Doenças Prostáticas/patologia , Doenças Prostáticas/veterinária , Hiperplasia Prostática/complicações , Hiperplasia Prostática/tratamento farmacológico , Hiperplasia Prostática/veterinária , Pamoato de Triptorrelina/administração & dosagem , Pamoato de Triptorrelina/efeitos adversos , Pamoato de Triptorrelina/análogos & derivados , Pamoato de Triptorrelina/uso terapêutico
15.
Artigo em Inglês | MEDLINE | ID: mdl-33036371

RESUMO

This study aimed at (1) studying the effect of the standardized ToyBox intervention on European preschoolers' snacking behavior, and (2) studying whether a higher process evaluation score from teachers and parents/caregivers was associated with a more positive result for preschoolers' snack intake. A sample of 4970 preschoolers (51.4% boys, 4.74 ± 0.44 years) from six European countries provided information on snack intake with the use of a Food Frequency Questionnaire. To investigate the effect of the intervention, multilevel repeated measures analyses were executed for the total sample and the six country-specific samples. Furthermore, questionnaires to measure process evaluation were used to compute a total process evaluation score for teachers and parents/caregivers. No significant intervention effects on preschoolers' snack intake were found (all p > 0.003). In general, no different effects of the intervention on snack intake were found according to kindergarten teachers' and parents'/caregivers' process evaluation scores. The lack of effects could be due to limited intervention duration and dose. To induce larger effects on preschoolers' snack intake, a less standardized intervention which is more tailored to the local needs might be needed.


Assuntos
Comportamentos Relacionados com a Saúde , Promoção da Saúde/métodos , Obesidade Pediátrica/prevenção & controle , Lanches , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Masculino , Instituições Acadêmicas , Inquéritos e Questionários
16.
J Pediatr Gastroenterol Nutr ; 71(5): 647-654, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33093372

RESUMO

OBJECTIVES: Chronic cholestatic liver diseases are often associated with disturbed lipid metabolism, which may potentially increase cardiovascular (CV) risk but the evidence is scarce. The aim of the study was to assess factors associated with increased CV risk in children with Alagille syndrome (AGS) and biliary atresia (BA). METHODS: We investigated 17 patients with AGS, ages 11.0 years (8.4-13.4) and 19 with BA, ages 13.5 years (10.4-15.1) in whom we performed thorough biochemical assessment including lipid profiles and oxidative stress biomarkers, blood pressure (BP)-systolic, diastolic and mean, carotid intima-media thickness (cIMT), and pulse wave velocity (PWV). RESULTS: There were abnormal lipid profiles in 82% of children with AGS and 52.6% with BA. In AGS group, we observed significantly higher levels of TC, LDL C, APO B, lower glutathione concentration and glutathione peroxidase activity and lower blood pressure, lower cIMT (P = 0.02), cIMT-SDS (P = 0.04), and PWV (P = 0.04). We, however, observed elevated blood pressure in 2/19 patients with BA and none-with AGS (BA vs AGS: P = 0.12), whereas cIMT-SDS was increased only in 2/17 patients with AGS and in 6/19 with BA (P = 0.24), and abnormal PWV-SDS values were detected in 3/17 of AGS and 8/19 of BA patients (P = 0.15). Neither presence of dyslipidemia nor Lp-X correlated with vascular parameters. CONCLUSIONS: Children with BA and AGS may present with increased cardiovascular risk factors but vascular parameters are not directly related to lipid abnormalities. cIMT and BP should be considered for clinical practice in these cholestatic disorders so as to determine individuals with potential CV risk.


Assuntos
Doenças Cardiovasculares , Hepatopatias , Adolescente , Doenças Cardiovasculares/etiologia , Espessura Intima-Media Carotídea , Criança , Fatores de Risco de Doenças Cardíacas , Humanos , Análise de Onda de Pulso , Medição de Risco , Fatores de Risco
17.
Mediators Inflamm ; 2020: 8327945, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32963496

RESUMO

Gene expression profiles of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) were evaluated in peripheral blood leukocytes of children with nonalcoholic fatty liver disease (NAFLD). Gene expression patterns were correlated with their plasma protein counterparts, systemic parameters of liver injury, and selected markers of inflammation. The MMP-2, MMP-9, MMP-12, MMP-14, TIMP-1, TIMP-2, TGF-ß, and IL-6 transcripts levels were tested by the real-time PCR. Plasma concentrations of MMP-9, TIMP-1, MMP-9/TIMP-1 ratio, MMP-2/TIMP-2 ratio, sCD14, leptin, resistin, IL-1 beta, and IL-6 and serum markers of liver injury were estimated by ELISA. The MMP-9, TIMP-2 expression levels, plasma amounts of MMP-9, TIMP-1, and the MMP-9/TIMP-1 ratio were increased in children with NAFLD. Concentrations of AST, ALT, GGT, and leptin were elevated in serum patients with NAFLD, while concentration of other inflammatory or liver injury markers was unchanged. The MMP-2 and MMP-9 levels correlated with serum liver injury parameters (ALT and GGT concentrations, respectively); there were no other correlations between MMP/TIMP gene expression profiles, their plasma counterparts, and serum inflammatory markers. Association of MMP-2 and MMP-9 expression with serum liver injury parameters (ALT, GGT) may suggest leukocyte engagement in the early stages of NAFLD development which possibly precedes subsequent systemic inflammatory responses.

18.
Artigo em Inglês | MEDLINE | ID: mdl-32740536

RESUMO

OBJECTIVES: Chronic cholestatic liver diseases are often associated with disturbed lipid metabolism, which may potentially increase cardiovascular (CV) risk, but the evidence is scarce. The aim of the study was to assess factors associated with increased CV risk in children with Alagille syndrome (AGS) and biliary atresia (BA). METHODS: We investigated 17 patients with AGS aged 11.0y (8.4-13.4) and 19 with BA aged 13.5y (10.4-15.1) in whom we performed thorough biochemical assessment including lipid profiles and oxidative stress biomarkers, blood pressure (BP)- systolic, diastolic and mean, carotid intima-media-thickness (cIMT) and pulse wave velocity (PWV). RESULTS: There were abnormal lipid profiles in 82% of children with AGS and 52.6% with BA. In AGS group we observed significantly higher levels of TC, LDL C, APO B, lower glutathione concentration and glutathione peroxidase activity and lower blood pressure, lower cIMT (p = 0.02), cIMT-SDS (p = 0.04) and PWV (p = 0.04). However, we observed elevated blood pressure in 2/19 patients with BA and none-with AGS (BA vs AGS: p = 0.12), while cIMT-SDS was increased only in 2/17 patients with AGS and in 6/19 with BA (p = 0.24) and abnormal PWV-SDS values were detected in 3/17 of AGS and 8/19 of BA patients (p = 0.15). Neither presence of dyslipidemia nor Lp-X correlated with vascular parameters. CONCLUSIONS: Children with BA and AGS may present with increased cardiovascular risk factors but vascular parameters are not directly related to lipid abnormalities. cIMT and BP should be considered for clinical practice in these cholestatic disorders so as to determine individuals with potential CV risk.

19.
Front Pediatr ; 8: 414, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32793533

RESUMO

Objective: To evaluate the clinical utility of panel-based NGS in the diagnostic approach of monogenic cholestatic liver diseases. Study design: Patients with diagnosis of chronic cholestatic liver disease of an unknown etiology underwent NGS of targeted genes panel. Group 1 included five patients (prospectively recruited) hospitalized from January to December 2017 while group 2 included seventeen patients (retrospectively recruited) hospitalized from 2010 to 2017 presenting with low-GGT PFIC phenotype (group 2a, 11 patients) or indeterminant cholestatic liver cirrhosis (group 2b, 6 patients). Results: Among 22 patients enrolled into the study, 21 various pathogenic variants (including 11 novel) in 5 different genes (including ABCB11, ABCB4, TJP2, DGUOK, CYP27A1) were identified. The molecular confirmation was obtained in 15 out of 22 patients (68%). In group 1, two out of five patients presented with low-GGT cholestasis, and were diagnosed with BSEP deficiency. Out of three patients presenting with high-GGT cholestasis, one patient was diagnosed with PFIC-3, and the remaining two were not molecularly diagnosed. In group 2a, seven out of eleven patients, were diagnosed with BSEP deficiency and two with TJP-2 deficiency. In group 2b, three out of six patients were molecularly diagnosed; one with PFIC-3, one with CYP27A1 deficiency, and one with DGUOK deficiency. Conclusions: Panel-based NGS appears to be a very useful tool in diagnosis of monogenic cholestatic liver disorders in cases when extrahepatic causes have been primarily excluded. NGS presented the highest diagnosis rate to identify the molecular background of cholestatic liver diseases presenting with a low-GGT PFIC phenotype.

20.
Res Vet Sci ; 132: 17-27, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32474261

RESUMO

Teriflunomide (TER) is an immunomodulatory agent. Although the first reports on the use of TER in dogs have already appeared, immune mechanisms underlying the immunomodulatory effect of TER do not seem to have been fully elucidated yet. There were two aspects of this study. First, further insight into the mode of action of TER was gained by investigating its effect on the expression of IL-2 receptor α-chain (CD25) and Forkhead box P3 (Foxp3) by CD4+ and CD8+ T cells and apoptosis of these cells. Second, in view in the earlier lack of data on the effect of TER on T cells in dogs, the results of this study filled in this gap. TER at a concentration which can be achieved in vivo prevented or reduced the activation-induced CD25 expression on CD4+ and CD8+ T cells, respectively. Taking into consideration the role of CD25 in T cell proliferation, this effect may constitute an additional mechanism responsible for the antiproliferative effect of the drug. Under stimulation conditions, TER induced Foxp3 expression in Foxp3-negative CD4+ and CD8+ T cells, while down-regulating it under unstimulated conditions. These results suggest that TER may generate iTreg cells, but this process requires cell activation. TER was not found to affect on the absolute count and apoptosis of CD4+ and CD8+ T cells. The results suggest that the impairment of CD25 expression during T cell activation and generation of iTreg cells may constitute additional mechanisms, besides the principal one, underlying the immunomodulatory effect of TER.


Assuntos
Apoptose , Crotonatos/farmacologia , Cães/imunologia , Fatores de Transcrição Forkhead/metabolismo , Fatores Imunológicos/farmacologia , Subunidade alfa de Receptor de Interleucina-2/metabolismo , Linfócitos T Reguladores/imunologia , Toluidinas/farmacologia , Animais , Linfócitos T CD4-Positivos/imunologia , Linfócitos T CD4-Positivos/metabolismo , Linfócitos T CD8-Positivos/imunologia , Linfócitos T CD8-Positivos/metabolismo , Linfócitos T Reguladores/metabolismo
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