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

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

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
• ateroskleróza prevence a kontrola   MeSH
• dietoterapie klasifikace   MeSH
• dyslipidemie * diagnóza   etiologie   farmakoterapie   krev   MeSH
• farmakoterapie klasifikace   MeSH
• lipidy krev   MeSH
• poruchy metabolismu lipidů diagnóza   farmakoterapie   klasifikace   MeSH
• Publikační typ
• přehledy MeSH

Health effects of vegan diets among children and adolescents are a controversial public health topic. Thus, the aim of the present systematic review is to evaluate a broad range of health outcomes among vegan children and adolescents aged 0 to 18 years. 18 studies met the inclusion criteria (17 cross-sectional, 1 RCT). Meta-analyses showed lower protein, calcium, vitamin B2, saturated fatty acid, and cholesterol intakes, and lower ferritin, HDL and LDL levels as well as height in vegan compared to omnivorous children/adolescents. Higher intakes of carbohydrates, polyunsaturated fatty acids, fiber, folate, vitamins C and E, magnesium, iron, and potassium were observed in vegans. Blood levels of vitamin B12 were higher among vegan children due to supplement use. Single study results suggested further differences between vegan and non-vegan children, such as lower bone mineral content or urinary iodine among vegan children. Risk of Bias was rated as high or very high in 7 out of 18 studies. The certainty of evidence for the meta-analyses was low (n = 2) or very low (n = 46). Overall, the available evidence points to both risks and benefits associated with a vegan diet among children, although more and better designed studies are needed.

• MeSH
• dieta veganská * MeSH
• dítě MeSH
• fyziologie výživy dětí MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• nutriční stav MeSH
• potravní doplňky MeSH
• předškolní dítě MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH
• metaanalýza MeSH
• přehledy MeSH
• systematický přehled MeSH

Background: Intermittent fasting may be an effective tool for weight loss, but it is still unclear from previous studies to date whether it is as effective as a continuous energy restriction in terms of reducing adipose tissue and whether it leads to unwanted muscle loss. Objectives: The aim of this study was to compare the effect of intermittent fasting (IF) with continuous energy restriction (CER) on the body weight and body composition and to assess the effect of intermittent fasting also in isolation from the energy restriction. Methods: After completion of a three-week dietary intervention, differences in the weight loss and differences in the body composition were compared between three groups. The first group consumed 75% of their calculated energy intake requirements in a six-hour time window. The second group consumed 75% of their calculated energy intake requirements without a time window and the third group consumed 100% of their calculated energy intake requirements in a six-hour time window. The changes in the weight and body composition were assessed by BIA. Results: Of the 95 randomized participants, 75 completed the intervention phase of the study. The highest mean weight loss was achieved by the IF with ER (energy restriction) group (2.3 ± 1.4 kg), followed by the CER group (2.2 ± 1.1 kg); the difference between the groups did not reach statistical significance. The lowest mean weight loss was observed in the IF without ER group (1.1 ± 1.2 kg), the difference reaching statistical significance compared to the IF with ER (p=0.003) and CER (p=0.012) groups. The highest mean adipose tissue loss was observed in the CER group (1.5 ± 1.2 kg) followed by the IF with ER group (1.3 ± 1.1 kg), with no statistically significant differences between the groups. A mean adipose tissue loss was found in the IF without ER group (0.9 ± 1.1 kg) with no statistically significant differences compared to the IF with ER and CER groups. The highest mean fat-free mass loss was found in the IF with ER group (1.1 ± 1.0 kg), followed by the CER group (0.65 ± 0.91 kg) with no statistically significant differences. The IF without ER group showed the lowest mean fat-free mass loss (0.2 ± 1.3 kg), which reached statistical significance compared to the IF with ER group (p=0.027). Conclusion: The results showed a comparable effect in the weight loss and body fat reduction regardless of the timing of the food intake. The diet quality, together with the energy intake, appeared to be one of the most important factors influencing the body composition.

• MeSH
• antropometrie MeSH
• dospělí MeSH
• energetický příjem * fyziologie   MeSH
• hmotnostní úbytek * fyziologie   MeSH
• index tělesné hmotnosti MeSH
• kalorická restrikce * metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• obezita dietoterapie   patofyziologie   MeSH
• omezení příjmu potravy * fyziologie   MeSH
• přerušované hladovění MeSH
• složení těla * fyziologie   MeSH
• tělesná hmotnost fyziologie   MeSH
• tuková tkáň 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
• randomizované kontrolované studie MeSH

BACKGROUND AND AIMS: Prolonged fasting, which leads to the mobilization of fat from adipose tissue, can result in the development of hepatosteatosis. However, it is not yet known whether the accumulation of fat in the liver after fasting can be affected by concurrent obesity. Therefore, this study aimed to assess how excessive adiposity influences changes in liver fat content induced by fasting and subsequent refeeding. METHODS AND RESULTS: Ten lean women and eleven women with obesity (age: 36.4 ± 7.9 and 34.5 ± 7.9 years, BMI: 21.4 ± 1.7 and 34.5 ± 4.8 kg/m2) underwent a 60-h fasting period followed by 2 days of isocaloric high-carbohydrate refeeding. Magnetic resonance spectroscopy (MRS) examinations of liver were conducted at baseline, after 48 h of fasting, and at the end of refeeding period. Hepatic fat content (HFC) increased in lean women after fasting, whereas no statistically significant change in HFC was observed in women with obesity. Additionally, fasting led to significant reductions in liver volume in both groups, likely attributable to glycogen depletion, with subsequent restoration upon refeeding. Notably, changes in hepatic fat volume (HFV) rather than HFC inversely correlated with baseline liver fat content and HOMA-IR. CONCLUSION: We demonstrated that prolonged fasting results in accumulation of fat in the liver in lean subjects only and that this accumulation is inversely related to baseline fat content and insulin resistance. Moreover, the study underscored the importance of evaluating hepatic fat volume rather than hepatic fat content in studies that involve considerable changes in hepatic lean volume.

• MeSH
• adipozita * MeSH
• časové faktory MeSH
• dietní sacharidy aplikace a dávkování   MeSH
• dospělí MeSH
• játra * metabolismus   diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• obezita * patofyziologie   terapie   metabolismus   MeSH
• omezení příjmu potravy * MeSH
• sacharidová dieta škodlivé účinky   MeSH
• studie případů a kontrol MeSH
• ztučnělá játra diagnostické zobrazování   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH

• MeSH
• dekubity MeSH
• dietoterapie * metody   MeSH
• hodnocení stavu výživy MeSH
• hojení ran * MeSH
• nutriční stav MeSH
• podvýživa MeSH
• senioři nad 80 let MeSH
• Check Tag
• senioři nad 80 let MeSH
• Publikační typ
• kazuistiky MeSH

Exokrinní pankreatická nedostatečnost (EPI – exocrine pancreatic insufficiency) je charakterizována nedostatečnou sekrecí pankreatických trávicích enzymů. Dle mechanistické teorie není nedostatkem pankreatických enzymů v tenkém střevě zajištěno trávení potravy, což je spojeno především s nedostatkem esenciálních mastných kyselin a liposolubilních vitaminů a ve svém důsledku vede k neschopnosti zajistit nutriční a metabolické potřeby organizmu. V diagnostice je standardem stanovení fekální elastázy. Toto stanovení je jednoduché, limitací je malá senzitivita stanovit možné změny pankreatické funkce již v tzv. iniciálních stadiích nemoci. Alternativou vyšetření fekální elastázy je použití dechových testů s využitím mixu triglyceridů označených radioaktivně na uhlíku C13. Test je sice neinvazivní, ale časově náročný a obtížněji dostupný. Klinickými symptomy EPI jsou především takové příznaky, které souvisí s mikrobiálním trávením a následnou malabsorbcí/maldigescí mikro- i makronutrientů. Kromě subjektivního pocitu nadýmání, borborygmů nebo osmotického průjmu jsou častým nálezem nízké hladiny liposolubilních vitaminů a některých stopových prvků. Do obrazu EPI patří i osteoporóza nebo sarkopenie. V terapii EPI je zásadním přístupem dietoterapie a substituce pankreatickými enzymy. Zásadou je podání odpovídající dávky především pankreatické lipázy: 40 000–50 000 jednotek k hlavním jídlům, s aplikací během jídla. Menší jídla (svačiny) jsou substituovány poloviční dávkou. Optimální galenickou formou jsou kapsle s ochranným obalem proti inaktivaci enzymů žaludeční kyselinou solnou před jejich vstupem do duodena. Galenickou formou jsou kapsle s obsahem enzymů v mikročásticích o velikosti 1,0–2,0 mm, které se z kapsle uvolní při vstupu do duodena. Jedná se o tzv. řízenou synchronizaci liberalizace enzymů, které obsahuje kapsle. EPI je stavem v populaci poddiagnostikovaným a podléčeným. Kontrola pacientů tak musí zahrnout kromě zhodnocení celkového klinického stavu i sledování změn, které mohou manifestovat malabsorpci. Nutriční stav je doporučeno sledovat alespoň jednou ročně, a to v pravidelných intervalech.

Exocrine pancreatic insufficiency (EPI) is characterized by insufficient secretion of pancreatic digestive enzymes. According to the mechanistic theory, the lack of pancreatic enzymes in the small intestine does not ensure the digestion of food, which is mainly associated with the lack of essential fatty acids and liposoluble vitamins and, as a result, leads to the inability to ensure the nutritional and metabolic needs of the organism. In diagnostics, the standard is determination of fecal elastase. This determination is simple, the limitation is the low sensitivity to determine possible changes in pancreatic function already in the so-called initial stages of the dis ease. An alternative to fecal elastase testing is the use of breath tests using a mixture of triglycerides, radioactively labeled with carbon C13. Although the test is non-invasive, it is time-consuming and more difficult to access. The clinical symptoms of EPI are mainly those related to microbial digestion and subsequent malabsorption/maldigestion of micro- and macronutrients. In addition to the subjective feeling of bloating, borborygmy or osmotic diarrhea, low levels of liposoluble vitamins and some trace elements are frequent findings. Osteoporosis or sarcopenia belong to the picture of EPI. In EPI therapy, diet therapy and pancreatic enzyme replacement are essential approaches. The principle is to administer an adequate dose, especially of pancreatic lipase: 40,000–50,000 units with main meals, with application during meals. Smaller meals (snacks) are substituted with half the dose. The optimal galenic form is capsules with a protective cover, against the inactivation of enzymes by gastric acid, before they enter the duodenum. The galenic form is capsules containing enzymes in microparticles, 1.0–2.0 mm in size, which are released from the capsule upon entering the duodenum. This is the so-called controlled synchronization of the liberalization of the enzymes contained in the capsule. EPI is an underdiagnosed and undertreated condition in the population. The control of patients must therefore include, in addition to the evaluation of the overall clinical condition, the monitoring of changes that may manifest malabsorption. It is recommended to monitor the nutritional status at least once a year, at regular intervals.

• MeSH
• dietoterapie metody   MeSH
• enzymoterapie metody   MeSH
• exokrinní pankreatická insuficience * diagnóza   terapie   MeSH
• lidé MeSH
• malabsorpční syndromy etiologie   MeSH
• osteoporóza etiologie   MeSH
• pankreatická elastasa analýza   MeSH
• pankreatin terapeutické užití   MeSH
• Check Tag
• lidé MeSH

AIMS: We investigated whether a short period of tightly controlled low-carbohydrate diet (LCD) leads to higher time in range without increasing the associated risks in children and young people with diabetes (CYPwD). METHODS: Thirty-five (CYPwD) were recruited into this randomized controlled cross-over study (20 female; 20 CSII; age 14.5 ± 2.9 years; HbA1c 48.9 ± 9.4 mmol/mol). The interventions were five and five weeks of ready-made food box deliveries of isocaloric diets in random order: either LCD (94.5 ± 4.7 g/day) or recommended carbohydrate diet (RCD) (191 ± 19.2 g/day). The outcomes were continuous glucose monitoring parameters, anthropometric, laboratory and quality of life (QoL) data. RESULTS: Time in range was significantly higher in the LCD than in the RCD period (77.1 % vs. 73.8 %, P=0.008). Times in hyperglycemia and average glycaemia were significantly lower in the LCD. There was no difference between the diets in time in hypoglycemia or glycemic variability. The subjects' body weight and BMI were significantly lower during the LCD. There was no significant difference in the LDL-cholesterol levels. No significant differences were observed in the self-assessed QoL. CONCLUSIONS: Short-term LCD led to an improvement of glycemic parameters without increasing time in hypoglycemia, disturbing the lipid profile or negatively affecting the quality of life of CYPwD.

• MeSH
• diabetes mellitus 1. typu * dietoterapie   krev   MeSH
• dieta s omezením sacharidů * metody   MeSH
• dítě MeSH
• glykovaný hemoglobin metabolismus   analýza   MeSH
• klinické křížové studie * MeSH
• krevní glukóza metabolismus   analýza   MeSH
• kvalita života MeSH
• lidé MeSH
• mladiství MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• randomizované kontrolované studie MeSH