Neonatal period
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- MeSH
- anemie diagnóza MeSH
- hematokrit metody MeSH
- nemoci novorozenců MeSH
- novorozenec MeSH
- polycytemie diagnóza MeSH
- Check Tag
- novorozenec MeSH
Za 16 rokov bolo na pracovisku autorov operovaných 46 detí s vrodenou malformáciou bránice. Z tohto súboru bolo 12 detí (26 %), u ktorých sa prvé príznaky VBH objavili až po novorodeneckom období. Najčastejšie to boli opakované respiračné infekty u 9 detí, gastrointestinálne ťažkosti zaznamenali v 4 prípadoch. Deti boli operované elektívne: u 8 pacientov išlo o ľavostrannú Bochdalekovu herniu (Ix s vakom), 3 deti mali hiátovú herniu a Morgagniho hernia bola u 1 dieťaťa. Pooperačné nezaznamenali žiadnu včasnú ani neskorú komplikáciu. V diskusii autori rozoberajú problematiku VBH s neskorou manifestáciou, čo je problém hlavne diagnostický. Preto na základe vlastných skúseností v zhode s literárnymi údajmi doporučujú dôkladné cielené vyšetrenie detí s chronickými nešpecifickými respiračnými alebo gastrointestinálnymi ťažkosťami.
In the course of 16 years at the authors department 46 children with congenital malformation of the diaphragm were operated. This group comprised 12 children (26 %) where the first symptoms developed only after the neonatal period. Most frequently the symptoms were repeated respiratory infections (in 9 children),and gastrointestinal complaints in 4 children. The children were operated electively: in 8 patients a left-sided Bochdalek hernia (Ix with a sac) was involved, 3 children had a hiatus hernia and one child a Morgagni hernia. After surgery no early or late complications were recorded. In the discussion the authors deal with inborn diaphragmatic hernia with a late manifestation which is mainly a diagnostic problem. Therefore, based on their own experience and consistent with data in the literature, the authors recommend careful aimed examination of children with chronic non-specific respiratory or gastrointestinal complaints.
- MeSH
- brániční hernie diagnóza chirurgie MeSH
- diagnostické techniky a postupy MeSH
- diagnostické zobrazování MeSH
- dítě MeSH
- elektivní chirurgické výkony metody statistika a číselné údaje MeSH
- kojenec MeSH
- lidé MeSH
- novorozenec MeSH
- předoperační péče MeSH
- předškolní dítě MeSH
- vrozená brániční kýla MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- novorozenec MeSH
- předškolní dítě MeSH
IMPORTANCE: Red blood cell (RBC) transfusions are frequently administered to preterm infants born before 32 weeks of gestation in the neonatal intensive care unit (NICU). Two randomized clinical trials (Effects of Transfusion Thresholds on Neurocognitive Outcomes of Extremely Low-Birth-Weight Infants [ETTNO] and Transfusion of Prematures [TOP]) found that liberal RBC transfusion thresholds are nonsuperior to restrictive thresholds, but the extent to which these results have been integrated into clinical practice since publication in 2020 is unknown. OBJECTIVE: To describe neonatal RBC transfusion practice in Europe. DESIGN, SETTING, AND PARTICIPANTS: This international prospective observational cohort study collected data between September 1, 2022, and August 31, 2023, with a 6-week observation period per center, from 64 NICUs in 22 European countries. Participants included 1143 preterm infants born before 32 weeks of gestation. EXPOSURE: Admission to the NICU. MAIN OUTCOMES AND MEASURES: Study outcome measures included RBC transfusion prevalence rates, cumulative incidence, indications, pretransfusion hemoglobin (Hb) levels, volumes, and transfusion rates, Hb increment, and adverse effects of RBC transfusion. RESULTS: A total of 1143 preterm infants were included (641 male [56.1%]; median gestational age at birth, 28 weeks plus 2 days [IQR, 26 weeks plus 2 days to 30 weeks plus 2 days]; median birth weight, 1030 [IQR, 780-1350] g), of whom 396 received 1 or more RBC transfusions, totaling 903 transfusions. Overall RBC transfusion prevalence rate during postnatal days 1 to 28 was 3.4 transfusion days per 100 admission days, with considerable variation across countries, only partly explained by patient mix. By day 28, 36.5% (95% CI, 31.6%-41.5%) of infants had received at least 1 transfusion. Most transfusions were given based on a defined Hb threshold (748 [82.8%]). Hemoglobin levels before transfusions indicated for threshold were below the restrictive thresholds set by ETTNO in 324 of 729 transfusions (44.4%) and TOP in 265 of 729 (36.4%). Conversely, they were between restrictive and liberal thresholds in 352 (48.3%) and 409 (56.1%) transfusions, respectively, and above liberal thresholds in 53 (7.3%) and 55 (7.5%) transfusions, respectively. Most transfusions given based on threshold had volumes of 15 mL/kg (470 of 738 [63.7%]) and were administered over 3 hours (400 of 738 [54.2%]), but there was substantial variation in dose and duration. CONCLUSIONS AND RELEVANCE: In this cohort study of very preterm infants, most transfusions indicated for threshold were given for pretransfusion Hb levels above restrictive transfusion thresholds evaluated in recent trials. These results underline the need to optimize practices and for implementation research to support uptake of evidence.
- MeSH
- jednotky intenzivní péče o novorozence * statistika a číselné údaje MeSH
- lidé MeSH
- novorozenec nedonošený MeSH
- novorozenec MeSH
- prospektivní studie MeSH
- transfuze erytrocytů * statistika a číselné údaje metody MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- Geografické názvy
- Evropa MeSH
Kongenitální adrenální hyperplazie (CAH) je autozomálně recesivně dědičná porucha tvorby steroidních hormonů v kůře nadledvin. Předpokládaná incidence v naší populaci je 1:10 000 až 1:15 000. Důsledkem porušené tvorby steroidních hormonů je nadledvinová nedostatečnost, která se může projevit již v novorozeneckém věku a ohrozit život dítěte. Avšak i v pozdějším věku dětí způsobuje CAH řadu vážných klinických problémů. Včasný záchyt pomocí novorozeneckého screeningu může přispět k záchraně nejtíživěji postižených dětí a ke zlepšení kvality života většiny ostatních. Novorozenecký screening je metodicky založen na stanovení hladiny 17a-hydroxyprogesteronu v suché kapce krve na filtračním papírku. Ve vyspělých zemích je screening CAH nejčastěji zaváděným screeningem po screeningu kongenitální hypotyreózy a fenylketonurie. I v České republice by měla být jeho efektivita ověřena pro event. celoplošné provádění.
Congenital adrenal hyperplasia (CAH) is an autosomal recessive inherited disorder of adrenal steroidogenesis. The expected incidence in the Czech population is 1:10 000 to 1:15 000. It results in adrenal insufficiency, which can be life threatening already in the newborn period. Numerous serious clinical problems can arise also in later childhood. An early recognition of CAH by the neonatal screening may save the life of the most severely affected patients and substantially improve the quality of life of the remaining. The neonatal screening for CAH is based on the estimation of the 17a-hydroxyprogesterone level in the dried blood spot. In developed countries, the screening for CAH is currently the most widespread addition to screening programs for phenylketonuria and congenital hypothyroidism. The assessment of its efficacy in conditions of the Czech Republic is essential.
- MeSH
- dítě MeSH
- kongenitální adrenální hyperplazie diagnóza genetika terapie MeSH
- lidé MeSH
- novorozenec MeSH
- novorozenecký screening MeSH
- steroid-21-hydroxylasa enzymologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- novorozenec MeSH
- Geografické názvy
- Česká republika MeSH
