Hepatorenal tyrosinaemia (HT1) is an autosomal recessive disorder of tyrosine degradation resulting in hepatic and renal dysfunction, neurological sequelae may occur in some patients. The use of nitisinone (NTBC) has revolutionised treatment and outcome of this disorder. NTBC has to be combined with a low protein diet. While NTBC modulates the disease course in HT1 patients, several issues are open. Optimal dosage, doses per day, therapeutic range of NTBC concentration, mode of protein restriction and biomarkers are not well defined. HCC and neurocognitive deficits are long-term sequelae. Early diagnosis and treatment are essential to minimise the risk for these complications. Clinical guidance for management of HT1-patients is required. Randomised clinical studies are difficult in the presence of therapeutic options. We discussed these issues in a consensus group of 10 paediatricians, 1 adult hepatologist, 1 geneticist, 2 dieticians, 2 newborn screening specialists with experience in HT1, 1 psychologist and 2 representatives of a patient group from the German-speaking countries (DACH). Recommendations were based on scientific literature and expert opinion, also taking into account recent experience with newborn screening. There was strong consensus that newborn screening using succinylacetone (SA) and early treatment are essential for a good outcome. The dose of NTBC should be as low as possible without losing metabolic control. This has to be accompanied by a low protein diet, in some patients a simplified diet without calculation of protein intake. Specific education and psychosocial support are recommended. Indications for liver transplantation were defined. Monitoring shall include clinical findings, levels of SA, tyrosine, phenylalanine and NTBC in (dried) blood.
- MeSH
- cyklohexanony * terapeutické užití MeSH
- heptanoáty MeSH
- konsensus MeSH
- lidé MeSH
- nitrobenzoany * terapeutické užití MeSH
- nízkoproteinová dieta MeSH
- novorozenec MeSH
- novorozenecký screening * metody MeSH
- transplantace jater MeSH
- tyrosinemie * diagnóza terapie MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Německo MeSH
- MeSH
- kazuistiky jako téma MeSH
- kritický stav ošetřování MeSH
- lidé MeSH
- metabolismus MeSH
- novorozenec MeSH
- novorozenecká sepse * diagnóza etiologie komplikace MeSH
- parenterální výživa * metody MeSH
- Staphylococcus epidermidis patogenita MeSH
- živiny aplikace a dávkování klasifikace MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- Publikační typ
- přehledy MeSH
- Klíčová slova
- gastrická sonda,
- MeSH
- enterální výživa klasifikace metody MeSH
- gastrointestinální intubace * klasifikace metody ošetřování MeSH
- lidé MeSH
- metody výživy klasifikace mortalita MeSH
- novorozenec MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- Publikační typ
- přehledy MeSH
BACKGROUND: Exclusive enteral nutrition (EEN) is an effective treatment for active Crohn's disease (CD). This study explored the immunostimulatory potential of a cell-free fecal filtrate and related this with changes in the fecal microbiota and metabolites in children with active CD undertaking treatment with EEN. METHODS: Production of tumor necrosis factor α (TNFα) from peripheral blood mononuclear cells was measured following their stimulation with cell-free fecal slurries from children with CD, before, during, and at completion of EEN. The metabolomic profile of the feces used was quantified using proton nuclear magnetic resonance and their microbiota composition with 16S ribosomal RNA sequencing. RESULTS: Following treatment with EEN, 8 (72%) of 11 patients demonstrated a reduction in fecal calprotectin (FC) >50% and were subsequently labeled FC responders. In this subgroup, TNFα production from peripheral blood mononuclear cells was reduced during EEN (P = .008) and reached levels like healthy control subjects. In parallel to these changes, the fecal concentrations of acetate, butyrate, propionate, choline, and uracil significantly decreased in FC responders, and p-cresol significantly increased. At EEN completion, TNFα production from peripheral blood mononuclear cells was positively correlated with butyrate (rho = 0.70; P = .016). Microbiota structure (β diversity) was influenced by EEN treatment, and a total of 28 microbial taxa changed significantly in fecal calprotectin responders. At EEN completion, TNFα production positively correlated with the abundance of fiber fermenters from Lachnospiraceae_UCG-004 and Faecalibacterium prausnitzii and negatively with Hungatella and Eisenbergiella tayi. CONCLUSIONS: This study offers proof-of concept data to suggest that the efficacy of EEN may result from modulation of diet-dependent microbes and their products that cause inflammation in patients with CD.
- MeSH
- Crohnova nemoc * terapie mikrobiologie imunologie MeSH
- dítě MeSH
- enterální výživa * metody MeSH
- feces * mikrobiologie chemie MeSH
- leukocytární L1-antigenní komplex * analýza MeSH
- leukocyty mononukleární imunologie metabolismus MeSH
- lidé MeSH
- mladiství MeSH
- střevní mikroflóra * MeSH
- TNF-alfa * metabolismus MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Úvod: Akutní poškození ledvin (AKI – acute kidney injury) se vyskytuje u kriticky nemocných pacientů s výrazným dopadem na jejich morbiditu a mortalitu. Náhlé snížení renálních funkcí ovlivňuje činnost a funkci jiných orgánů, mezi jinými také střevo navozením střevní dysmikrobie, jež recipročně zhoršuje průběh AKI a může také vést k rozvoji chronické nefropatie. Cíl: Patofyziologie AKI, sepse a střevní dysmikrobie v kritickém stavu pacienta s dopadem na vývoj nemoci a jiné orgánové systémy s možnostmi jejího pozitivního ovlivnění nutričními opatřeními. Závěr: Kriticky nemocní pacienti zejména v septickém stavu komplikovaném AKI jsou náchylní ke komplikacím typu střevní dysmikrobie, sekundární sepse a imunodeficience. Léčba základního onemocnění a jeho komplikací spolu s nutriční podporou napomáhá ke zlepšení klinického stavu této skupiny nemocných.
Introduction: Acute kidney injury (AKI) occurs in critically ill patients with significant impact on their morbidity and mortality. The sudden reduction in renal function affects the function of other organs, comprising the intestine, inducing intestinal dysmicrobia, which reciprocally worsens the course of AKI and may also lead to the development of chronic nephropathy. Purpose: Pathophysiology of AKI, sepsis and intestinal dysmicrobia in a critical patient with impact on the development of the disease and other organ systems with possibilities of its positive influence by nutritional measures. Conclusion: Critically ill patients especially in septic status complicated by AKI are prone to complications such as intestinal dysmicrobia, secondary sepsis and immunodeficiency. Treatment of the underlying disease and its complications along with nutritional support helps to improve the clinical outcome of this group of patients.
- MeSH
- akutní poškození ledvin * komplikace patofyziologie MeSH
- dysbióza * etiologie mikrobiologie patologie MeSH
- lidé MeSH
- nutriční podpora MeSH
- péče o pacienty v kritickém stavu MeSH
- podvýživa etiologie terapie MeSH
- sepse patologie MeSH
- septický šok patologie MeSH
- uremické toxiny škodlivé účinky MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- práce podpořená grantem MeSH
- přehledy MeSH
Very low carbohydrate high fat (VLCHF) diet and high-intensity interval training (HIIT) are widely utilized for weight reduction and cardiorespiratory fitness improvement, respectively. To assess the acceptability of these approaches, it is essential to examine mental health-related indicators. This secondary analysis of a randomized controlled trial investigated the isolated and synergistic effects of VLCHF and HIIT on mental health-related indicators in individuals with excessive weight or obesity. Sixty-eight participants (age = 42 ± 10.2; 20-60 years; BMI = 29.8 ± 3.7) were analysed across four groups: HIIT (n = 15, 4 males, 11 females), VLCHF (n = 19, 4 males, 15 females), VLCHF + HIIT (n = 19, 4 males, 15 females), and control (n = 15, 4 males, 11 females). The 12-week intervention, involved VLCHF diet or HIIT sessions, depending on group affiliation and completing online questionnaires via Qualtrics software before and after the intervention. The questionnaires included the 12-item Short Form Survey (SF-12) for mental (MHS) and physical health scores (PHS), the Satisfaction with Life Scale (SWLS), and the Perceived Stress Scale (PSS). Using the Kruskal-Wallis test, we found no significant differences in mental health-related indicators between groups after 12 weeks, except for SWLS (p = 0.031; ES = 0.133; medium), which improved significantly in the VLCHF + HIIT group compared to the HIIT group. Our findings indicate that HIIT and VLCHF, alone or combined, do not significantly affect mental health-related indicators.
- MeSH
- dieta s omezením sacharidů * metody MeSH
- dieta s vysokým obsahem tuků * škodlivé účinky MeSH
- dospělí MeSH
- duševní zdraví * MeSH
- hmotnostní úbytek MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- obezita * MeSH
- randomizované kontrolované studie jako téma MeSH
- vysoce intenzivní intervalový trénink * metody MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
OBJECTIVE: To evaluate whether implementing a nutritional care bundle is associated with growth and morbidity in very preterm (VPT) infants. STUDY DESIGN: This study compared 87 VPT infants (<32 gestational weeks) born 2018 (Before group) with 75 infants born 2020 (After group), treated at a single center in the Czech Republic. A nutritional care bundle was implemented during 2019. RESULTS: Median gestational age (weeks) was 30.0 [IQR 27.6-31.1] for the Before group and 29.9 [IQR 27.9-30.6] for the After group. During postnatal days 1-14, parenteral fluid intake was significantly lower in the After group compared to the Before group and conversely for enteral fluid intake. Infants in the After group achieved full enteral feeds by postnatal day 14 (72.9 % vs. Before group 51.9 %). Weight z-scores decreased significantly less from birth to 36 weeks postmenstrual age in the After group (-0.8 [IQR -1.3 to -0.5]) compared to the Before group (-1.5 [IQR -2.0 to -1.2]). Head circumference z-scores decreased significantly less in the After group (-0.8±0.9) than the Before group (-1.6±1.1). Decreased rate of patent ductus arteriosus (PDA) requiring treatment was observed in the After group (P < 0.001). CONCLUSIONS: Implementation of a nutritional care bundle in VPT infants was associated with improved postnatal growth and may reduce treatment-requiring PDA.
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Příručka, která se zaměřuje na atopickou dermatitidu u kojenců a předškolních dětí. Určeno široké veřejnosti, zejména rodičům.
- MeSH
- alergeny MeSH
- atopická dermatitida * MeSH
- dietoterapie MeSH
- kojenec MeSH
- péče o dítě MeSH
- potravinová alergie MeSH
- předškolní dítě MeSH
- Check Tag
- kojenec MeSH
- předškolní dítě MeSH
- Publikační typ
- populární práce MeSH
- příručky MeSH
- Konspekt
- Pediatrie
- NLK Obory
- dermatovenerologie
- pediatrie
- zdravotní výchova
- MeSH
- antropometrie metody MeSH
- hodnocení stavu výživy MeSH
- kwashiorkor diagnóza etiologie MeSH
- nutriční podpora klasifikace metody MeSH
- podvýživa * diagnóza etiologie klasifikace terapie MeSH
- poruchy výživy diagnóza klasifikace MeSH
- protein-energetická malnutrice diagnóza etiologie MeSH
- Publikační typ
- přehledy MeSH
