Necrotizing enterocolitis (NEC) is a severe intestinal condition primarily affecting preterm neonates. It has a high mortality rate, particularly in infants with a birthweight of below 1,500 g or for those requiring surgical intervention. The European Reference Network for Inherited and Congenital Anomalies (ERNICA) has developed a clinical practice guideline to aid clinical decision-making pertaining to the surgical treatment and management of NEC in preterm neonates. This guideline was developed in accordance with the Guidelines 2.0 checklist and GRADE methodology. A multidisciplinary group of Europe's top experts collaborated with patient representatives to develop this guideline. After selecting critical points in care for which recommendations are required, a systematic review of the literature and critical appraisal of the evidence was performed. The Evidence to Decision framework was used as a guide to structure the consensus meetings and draft the recommendations. The panel developed seven recommendations and three good practice statements on the following topics: indications for surgery, peritoneal drainage, surgical technique, management of extensive NEC, enteral feeding, and neurodevelopmental outcomes in premature neonates with NEC. The certainty of evidence was graded as (very) low for most recommendations. However, the panel weighed up the benefits and harms in light of all relevant arguments and expert opinion. This guideline provides recommendations on caring for premature neonates with NEC. These recommendations can assist clinicians in their care decisions and can inform families on treatment options and relevant considerations. This guideline will be revised every 5 years to ensure it remains up to date.

• MeSH
• Enteral Nutrition MeSH
• Clinical Decision-Making MeSH
• Humans MeSH
• Evidence-Based Medicine MeSH
• Enterocolitis, Necrotizing * surgery   diagnosis   MeSH
• Infant, Premature MeSH
• Infant, Newborn MeSH
• Check Tag
• Humans MeSH
• Infant, Newborn MeSH
• Publication type
• Journal Article MeSH
• Practice Guideline MeSH
• Systematic Review MeSH
• Geographicals
• Europe MeSH

The aim of the present study was to examine gender and age-specific effects on subjective daytime sleepiness (as measured by the Epworth Sleepiness Scale), body weight and eating behaviour in patients with central disorders of hypersomnolence. Based on the European Narcolepsy Network database, we compared 1035 patients with narcolepsy type I and 505 patients with other central disorders of hypersomnolence ("narcoleptic borderland"), including narcolepsy type II (N = 308) and idiopathic hypersomnia (N = 174), using logistic regression and general linear models. In the entire study population, the Epworth Sleepiness Scale was higher in women (N = 735, mean age = 30 years, mean Epworth Sleepiness Scale = 16.6 ± SD 3.9) than in men (N = 805, mean age = 32 years, mean Epworth Sleepiness Scale = 15.8 ± SD 4.4). In women with narcolepsy type I (N = 475), both Epworth Sleepiness Scale and body mass index increased in parallel with age. In women of the narcoleptic borderland (N = 260), the Epworth Sleepiness Scale markedly peaked in their early 30s, while body mass index only started to rise at that age. This rise in body mass index following the Epworth Sleepiness Scale peak cannot be explained by sleepiness-induced uncontrolled eating, as self-reported uncontrolled eating was negatively associated with the Epworth Sleepiness Scale in this group. We propose that the narcoleptic borderland harbours a unique cluster of women in their fertile years with an unexplored aetiology requiring further investigation towards tailored interventions.

• MeSH
• Adult MeSH
• Body Mass Index * MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Narcolepsy * physiopathology   MeSH
• Disorders of Excessive Somnolence * physiopathology   epidemiology   MeSH
• Sex Factors MeSH
• Feeding Behavior MeSH
• Body Weight MeSH
• Age Factors MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

This article provides an up-to-date review of the management of chronic pancreatitis, highlighting advancements in medical therapy, nutritional support, endoscopic and surgical approaches, and emerging treatments. Nutritional management accentuates addressing malabsorption and nutrient deficiencies. Advances in endoscopy and parenchyma-sparing surgical techniques have opened new avenues for improved patient outcomes, with total pancreatectomy and islet autotransplantation offering the only definitive solution for selected patients. Additionally, emerging therapies, including anti-inflammatory and immune-modulating agents, show promise for future treatment options. Emphasizing a multidisciplinary approach, this review aims to equip health care professionals with a comprehensive overview of current management strategies and future directions.

• MeSH
• Pancreatitis, Chronic * therapy   MeSH
• Humans MeSH
• Nutritional Support * methods   MeSH
• Pancreatectomy * methods   MeSH
• Islets of Langerhans Transplantation methods   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH

Úvod: Plicní sekvestrace je vzácná vrozená anomálie dolních dýchacích cest, která je většinou diagnostikována už v dětském věku. Jde o plicní parenchym, který má anomální nebo chybějící komunikaci s tracheobronchiálním stromem. Cévní zásobení není napojeno na plicní oběh, ale pochází ze systémového oběhu. Intralobární plicní sekvestrací se rozumí plicní sekvestr, který má společnou pleuru s normálním parenchymem. Naopak extralobární sekvestr má svou vlastní viscerální pleuru, která nesouvisí s normální plící. Extralobární sekvestrace na rozdíl od intralobární většinou nemá infekční projevy, protože chybí napojení na tracheobronchiální strom. Naopak se může projevit hypoxií, kardiálním selháním či vzácně torzí sekvestru. Častěji však jde o náhodný asymptomatický nález. Vedoucí úlohu v diagnostice má v současnosti CT, které umožňuje zobrazení přívodných cév. Metodou léčby je chirurgická resekce, hlavně jako prevence krvácení či maligního zvratu tkáně. Resekce také poskytne materiál pro bioptické vyšetření. Kazuistika: 68letá pacientka s nefropatií byla v režii nefrologa indikována k ultrasonografii břicha, kde byl náhodně zjištěn tumor v retroperitoneu vlevo. Bylo doplněno CT vyšetření a poté byla indikována k exstirpaci tumoru pro podezření na neurogenní tumor, avšak histologicky šlo o extralobární plicní sekvestraci. Další průběh pak nebyl nijak komplikován. Propuštěna do domácí péče byla 5. pooperační den. Závěr: Kazuistikou chceme prezentovat velmi raritní diagnózu, která je o to vzácnější, že byla nalezena u dospělé pacientky.

Introduction: Pulmonary sequestration is a rare congenital anomaly of the lower respiratory tract, usually diagnosed in childhood. It involves lung parenchyma that has abnormal or absent communication with the tracheobronchial tree. The blood supply is not connected to the pulmonary circulation but comes from the systemic circulation. Intralobar pulmonary sequestration refers to pulmonary sequestration that shares a common pleura with normal parenchyma. In contrast, extralobar sequestration has its own visceral pleura that is not continuous with the normal lung. Extralobar pulmonary sequestration, unlike intralobar pulmonary sequestration, typically does not present with infectious manifestations due to the absence of a connection to the tracheobronchial tree. Instead, it may manifest as hypoxia, cardiac failure, or rarely, torsion of the sequestrum. However, it is more often an incidental asymptomatic finding. Computed tomography currently plays a leading role in diagnosis, enabling visualization of the feeding vessels. Surgical resection is the mainstay of the treatment, primarily to prevent hemorrhage or malignant transformation of the tissue. Resection also provides material for biopsy examination. Case report: A 68-year-old patient with nephropathy was referred by her nephrologist for abdominal ultrasound, which incidentally detected a tumor in the left retroperitoneum. A CT scan was performed, and the patient was then referred for tumor excision due to suspicion of a neurogenic tumor. Postoperatively, she was monitored in the intensive care unit with oxygen therapy for hypoxemia. The further course was uncomplicated. She was discharged home on postoperative day 5. Conclusion: We present a case of this very rare diagnosis, which is even rarer in an adult patient.

• MeSH
• Respiratory System Abnormalities surgery   diagnostic imaging   MeSH
• Bronchopulmonary Sequestration * surgery   diagnostic imaging   MeSH
• Humans MeSH
• Retroperitoneal Neoplasms * surgery   diagnostic imaging   MeSH
• Aged MeSH
• Congenital Abnormalities MeSH
• Rare Diseases MeSH
• Check Tag
• Humans MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Case Reports MeSH
• Research Support, Non-U.S. Gov't MeSH

Pierre Robinova sekvence (PRS) je vrozená vada, která zahrnuje mikrognatii, glosoptózu a u části pacientů rovněž rozštěp patra různého rozsahu. To vede k obstrukci horních dýchacích cest a problémům s krmením. PRS vyžaduje komplexní přístup, který kombinuje multidisciplinární péči a využívá jak chirurgické, tak neinvazivní metody. V posledních letech se jako jedna z hlavních neinvazivních možností léčby prosazuje Tübingenská palatinální destička (TPP), která stabilizuje polohu jazyka a pomáhá eliminovat obstrukci dýchacích cest. Současně uvolněním a předsunutím jazyka také stimuluje růst dolní čelisti a výrazně zlepšuje krmení novorozenců, čímž snižuje potřebu chirurgických zákroků, jako je např. tracheostomie. Tato metoda je nyní dostupná na specializovaných pracovištích v ČR, včetně Pediatrické kliniky 2. LF UK a FN Motol ve spolupráci se Stomatologickou klinikou 3. LF UK a FN KV Praha a s Rozštěpovým centrem Praha FN KV a Centrem pro léčbu rozštěpů obličeje v Brně. Významnou roli v péči o pacienty s PRS hraje multidisciplinární tým složený z pediatrů, ORL specialistů, plastických a maxilofaciálních chirurgů, ortodontistů a logopedů, který zajišťuje optimalizaci léčby a prevenci komplikací. Tento článek se zaměřuje na dostupné možnosti léčby PRS v České republice a zdůrazňuje přínosy TPP i nové technologie, jako je 3D tisk, které umožňují přesně přizpůsobené pomůcky pro efektivní léčbu.

Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and, in some patients, cleft palate of varying extent. This leads to upper airway obstruction and feeding difficulties. PRS requires a comprehensive approach that combines multidisciplinary care with both surgical and non-invasive methods. In recent years, the Tübingen Palatal Plate (TPP) has emerged as one of the primary non-invasive treatment options. TPP stabilizes the tongue position, helping to eliminate airway obstruction. Additionally, by relieving and advancing the tongue, it stimulates mandibular growth and significantly improves feeding in newborns, reducing the need for surgical interventions such as tracheostomy. This method is now available at specialized centers in the Czech Republic, including the Pediatric Department of the 2nd Faculty of Medicine, Charles University and University Hospital Motol, in collaboration with the Dental Department of the 3rd Faculty of Medicine, Charles University and FN KV Prague, and with Prague Cleft Center at FN KV, and the Facial Cleft Treatment Center in Brno. A multidisciplinary team plays a vital role in the care of PRS patients, comprising pediatricians, ENT specialists, plastic and maxillofacial surgeons, orthodontists, and speech therapists. This team ensures treatment optimization and prevents complications. This article focuses on the available treatment options for PRS in the Czech Republic, highlighting the benefits of TPP and new technologies, such as 3D printing, which allow for precisely tailored devices to enhance treatment efficacy.

• MeSH
• Glossoptosis therapy   MeSH
• Humans MeSH
• Airway Obstruction MeSH
• Orthodontic Appliances MeSH
• Pierre Robin Syndrome * diagnosis   etiology   therapy   MeSH
• Cleft Palate therapy   MeSH
• Patient Care Team MeSH
• Check Tag
• Humans MeSH

V souvislosti s narůstající prevalencí nadváhy a obezity stoupají počty těchto pacientů i na jednotkách intenzivní péče – aktuální statistiky uvádějí 20–30 % přijímaných pacientů v pásmu obezity a 25 % s nadváhou. Nutriční cíle a strategie u pacientů s obezitou jsou jednou z největších výzev moderní nutriční péče. Zahrnují komplexní diagnostiku malnutrice a správné stanovení energetického příjmu i příjmu bílkovin, pro které je možné využít vzorců doporučených Evropskou společností pro parenterální a enterální výživu (ESPEN) nebo Americkou společností pro parenterální a enterální výživu (ASPEN). Výzkum se zaměřuje na možnosti imunonutrice a ovlivnění mikrobioty.

In connection with the increasing prevalence of overweight and obesity, the number of these patients in intensive care units is increasing – current statistics indicate 20–30% of patients admitted in the obesity zone and 25% in the overweight zone. Nutritional goals and strategies for patients with obesity are one of the biggest challenges of modern nutritional care. They include a comprehensive diagnosis of malnutrition and the correct determination of energy and protein intake, for which it is possible to use formulas recommended by the European Society for Parenteral and Enteral Nutrition (ESPEN) or the American Society for Parenteral and Enteral Nutrition (ASPEN). The research focuses on the possibilities of immunonutrition and influencing the microbiota.

• MeSH
• Energy Metabolism MeSH
• Humans MeSH
• Nutritional Status MeSH
• Nutrition Therapy * MeSH
• Obesity * diet therapy   MeSH
• Critical Care MeSH
• Check Tag
• Humans MeSH

• MeSH
• Child MeSH
• Humans MeSH
• Motor Skills MeSH
• Perception MeSH
• Postural Balance MeSH
• Eating * physiology   MeSH
• Primary Health Care MeSH
• Sense Organs MeSH
• Feeding Behavior * physiology   MeSH
• Check Tag
• Child MeSH
• Humans MeSH

Na základě pověření vlády a ministerstva zdravotnictví zpracoval Státní zdravotní ústav analýzu dodržování Mezinárodního kodexu marketingu náhrad mateřského mléka v ČR. Kodex byl přijat v roce 1981 a jeho cílem je omezit nevhodný marketing a chránit kojení. Tento kodex reguluje reklamu a propagaci výrobků, určených k výživě i krmení kojenců a malých dětí. Pro monitoring implementace kodexu v ČR byla vytvořena síť NetCode. V rámci sítě byl vyvinut soubor metodik (protokolů), které průběh a pravidla monitoringu definují.

The National Institute of Public Health was assigned by the Government and the Ministry of Health to conduct an analysis of the compliance with the International Code of Marketing of Breast-milk Substitutes in the Czech Republic. The Code was adopted in 1981, and it aims to reduce inappropriate marketing and protect breastfeeding. This code regulates the advertising and promotion of products intended for the nutrition and feeding of infants and young children. The NetCode was created to monitor the implementation of the Code in the Czech Republic, and a set of methodologies (protocols) was developed to define the monitoring process and rules.

• MeSH
• Data Analysis MeSH
• Breast Feeding MeSH
• Humans MeSH
• Marketing * MeSH
• Infant Formula * legislation & jurisprudence   MeSH
• World Health Organization MeSH
• Nutrition Policy MeSH
• Check Tag
• Humans MeSH
• Geographicals
• Czech Republic MeSH

The circadian clock in choroid plexus (ChP) controls processes involved in its physiological functions, but the signals that synchronize the clock have been sparsely studied. We found that the ChP clock in the fourthventricle (4V) is more robust than that in the lateral ventricle (LV) and investigated whether both clocks use information about mealtime as a signal to synchronize with the current activity state. Exposure of mPer2Luc mice to a 10-day reverse restricted feeding (rRF) protocol, in which food was provided for 6 h during daytime, advanced the phase of the ChP clock in 4V and LV, as evidenced by shifted (1) PER2-driven bioluminescence rhythms of ChP explants ex vivo and (2) daily profiles in clock gene expression in both ChP tissues in vivo. In contrast, clocks in other brain regions (DMH, ARC, LHb) of the same mice did not shift. The 4V ChP responded more strongly than the LV ChP to rRF by modulating the expression of genes to ensure a decrease in resistance to cerebrospinal fluid drainage and increase the secretory capacity of ChP cells. Mechanistically, rRF affects the ChP clock through food-induced increases in insulin, glucose and temperature levels, as in vitro all three signals significantly shifted the clocks in both ChP tissues, similar to rRF. The effect of glucose was partially blocked by OSMI-1, suggesting involvement of O-linked N-acetylglucosamine posttranslational modification. We identified mechanisms that can signal to the brain the time of feeding and the associated activity state via resetting of the ChP clock.

• MeSH
• Circadian Clocks * physiology   genetics   MeSH
• Period Circadian Proteins metabolism   genetics   MeSH
• Circadian Rhythm physiology   MeSH
• Mice, Inbred C57BL MeSH
• Mice, Transgenic MeSH
• Mice MeSH
• Choroid Plexus * metabolism   physiology   MeSH
• Gene Expression Regulation MeSH
• Feeding Behavior * physiology   MeSH
• Lateral Ventricles metabolism   physiology   MeSH
• Animals MeSH
• Check Tag
• Male MeSH
• Mice MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH

Střevní mikrobiom je právem označovaný jako neviditelný orgán, který významně ovlivňuje lidský organismus po celý jeho život. Procesů, na kterých se podílí, je mnoho. Kromě dobře známé role v oblasti trávení dále ovlivňuje maturaci imunitního systému, metabolické programování a propojuje i poměrně vzdálené orgány (např. osa střevo–mozek). Vývoj střevního mikrobiomu začíná záhy po narození a působící faktory můžeme rozdělit na modifikovatelné (výživa, antibiotická léčba, prostředí) a neovlivnitelné (gestační stáří, způsob porodu). Alterace střevního mikrobiomu těmito faktory je spojována jak s krátkodobou, tak dlouhodobou morbiditou. Cílem současného výzkumu je porozumět nejen vývoji samotnému, ale i všem činitelům, které mohou do tohoto přirozeného procesu zasahovat. Poznání a pochopení komplexity střevního mikrobiomu nám umožní činit taková medicínská rozhodnutí, která budou mít pro novorozence značné benefity. Tento přehledový článek přináší vhled do problematiky vzniku a vývoje střevního mikrobiomu a s tím spojené možné klinické aspekty u donošeného i nedonošeného novorozence. Součástí našeho sdělení jsou i doporučení, která vycházejí ze současného poznání, k ovlivnění negativních následků alterace střevního mikrobiomu v tomto křehkém období.

Gut microbiome is regarded as an invisible organ influencing the human organism throughout the entire lifespan. Microbiome determines various physiological processes including immune system maturation, metabolic programming and furthermore, facilitates connections even between relatively distant organs (e.g. gut-brain axis). Its development begins shortly after birth and is modified by various aspects. We can categorize them into modifiable (antibiotic treatment, diet) and non-modifiable factors (gestational age, delivery mode). Intestinal alteration caused by these influencing factors might contribute to short-term and long-term morbidity. Our objective is to comprehend the microbiome development itself and its modifying factors. Understanding the microbiome complexity could help us make such medical decisions to outweigh negative sequelae of an early gut alteration. This review presents topics concerning microbiome origin and its development along with potential clinical aspects in term and preterm newborn. We also include recommendations for parents and health care professionals regarding possible attitudes, based on current scientific knowledge, to diminish early gut microbiome alterations.

• MeSH
• Anti-Bacterial Agents adverse effects   MeSH
• Immune System microbiology   growth & development   MeSH
• Breast Feeding MeSH
• Humans MeSH
• Infant, Premature immunology   MeSH
• Infant, Newborn MeSH
• Brain-Gut Axis physiology   immunology   MeSH
• Risk Factors MeSH
• Gastrointestinal Microbiome * physiology   drug effects   MeSH
• Check Tag
• Humans MeSH
• Infant, Newborn MeSH
• Publication type
• Review MeSH