BACKGROUND: There is a lack of information on the current healthcare systems for children with kidney diseases across Europe. The aim of this study was to explore the different national approaches to the organization and delivery of pediatric nephrology services within Europe. METHODS: In 2020, the European society for Paediatric Nephrology (ESPN) conducted a cross-sectional survey to identify the existing pediatric nephrology healthcare systems in 48 European countries covering a population of more than 200 million children. RESULTS: The reported three most important priorities in the care of children with kidney diseases were better training of staff, more incentives for physicians to reduce staff shortages, and more hospital beds. Positive achievements in the field of pediatric nephrology included the establishment of new specialized pediatric nephrology centers, facilities for pediatric dialysis and transplant units in 18, 16, and 12 countries, respectively. The most common problems included no access to any type of dialysis (12), inadequate transplant programs for all ages of children (12), lack of well-trained physicians and dialysis nurses (12), inadequate reimbursement of hospitals for expensive therapies (10), and lack of multidisciplinary care by psychologists, dieticians, physiotherapists, social workers and vocational counsellors (6). Twenty-five of 48 countries (52%) expected to have a shortage of pediatric nephrologists in the year 2025, 63% of clinical nurses and 56% of dialysis nurses. All three groups of health care professionals were expected to be lacking in 38% of countries. Prenatal assessment and postnatal management of renal malformations by a multidisciplinary team including obstetricians, geneticists, pediatricians, and pediatric surgeons was available in one third of countries. CONCLUSIONS: Our study shows that there are still very marked differences in pediatric health care systems across the European countries and highlights the need need for appropriate services for children with kidney disease in all European countries.
- Publikační typ
- časopisecké články MeSH
Arteriální hypertenze je nejdůležitější modifikovatelný rizikový faktor kardiovaskulární morbidity a mortality. Léčbu arteriální hypertenze u dítěte/adolescenta je třeba zahájit co nejdříve po stanovení diagnózy a posouzení rizikových faktorů pro hypertenzí mediovaného orgánového poškození (HMOD). Léčba zahrnuje jak vhodná nefarmakologická (také režimová opatření nebo změnu životního stylu), tak farmakologická opatření. Změny životního stylu u dítěte a adolescenta se skládají z kombinace intervencí (dieta k redukci nadváhy/obezity a omezení příjmu soli, pohyb, behaviorální terapie aj.). Nefarmakologická opatření v případě primární hypertenze mohou vést k dostatečné redukci TK bez nutnosti antihypertenzní medikace, u hypertenze sekundární pak jsou součástí komplexní terapie zahrnující i podání farmak.
Arterial hypertension is the most important modifiable risk factor for cardiovascular morbidity and mortality. Appropriate therapy of the disease should be initiated in children and adolescents as early as possible; mostly at the time of the diagnosis confirmation and the evaluation of the risk factors for hypertension-mediated organ damage (HMOD). Therapy includes non-pharmacological and pharmacological interventions. Nonpharmacological therapy consists from combined lifestyle interventions (diet, physical activity, body weight changes and behavioral therapy components). The complex of lifestyle interventions can lead to effective reduction in blood pressure without pharmacotherapy in the case of primary hypertension, and is important part of therapeutic approach in the case of secondary hypertension in children and adolescents, where the medication is necessary.
Trombotická mikroangiopatie je patologická léze spuštěná poškozením nebo dysfunkcí endotelu, která se vyskytuje v širokém spektru onemocnění. Jejími charakteristickými znaky jsou mikroangiopatická hemolytická anémie, trombocytopenie a ischémie orgánů, zejména ledvin, na podkladě mikrotrombotizace zejména v kapilárním řečišti. V dětském věku se vyskytuje jak v primární, tak sekundární, tj. získané formě – nejčastěji při infekci shiga-toxin produkující Escherichia coli. Porozumění epidemiologickým souvislostem, podmiňujícím příčinám, laboratorním a klinickým nálezům a diferenciální diagnóze je nezbytné pro včasnou a cílenou terapii těchto život ohrožující stavů.
Thrombotic microangiopathy (TMA) is a pathological lesion triggered by endothelial injury or dysfunction. TMA occurs in a wide range of diseases and is characterized by microangiopathic haemolytic anaemia, thrombocytopenia and ischemic organ injury due to thrombosis in the microvascular capillary bed. In the paediatric population, TMA occurs in both as a primary, i.e. congenital form, and a secondary, acquired form – especially linked to E. coli infection. Understanding the epidemiology, pathophysiology, laboratory and clinical signs and differential diagnosis is crucial for a timely specific therapeutic approach to this life-threatening disease.
- MeSH
- diferenciální diagnóza MeSH
- dítě MeSH
- hemolyticko-uremický syndrom diagnóza patologie terapie MeSH
- lidé MeSH
- prognóza MeSH
- protein ADAMTS13 metabolismus MeSH
- trombotická trombocytopenická purpura diagnóza patologie terapie MeSH
- trombotické mikroangiopatie * diagnóza komplikace patologie terapie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- přehledy MeSH
Ačkoliv je zvyšující se výskyt hypertenze v dětské populaci ve světě v poslední době často probírané téma, nemáme k dispozici recentní data z české populace, která by nám zodpověděla zdánlivě jednoduchou otázku "Kolik českých dětí má vysoký krevní tlak?". Pro určení odhadu prevalence hypertenze můžeme vycházet z mnoha jiných recentních dat, ať už ze studií provedených v USA, mnoha evropských zemích či nejnovější metaanalýzy. Jediná dodnes publikovaná studie provedená v ČR vycházela z dat, která již brzy dosáhnou stáří dvaceti let a mohou se tedy již značně lišit od reality dneška. Role PLDD v diagnostice hypertenze a následné sérii základních vyšetření je stále nezastupitelná, a to tím více, čím rychleji přibývá dětí s touto diagnózou. V přehledu uvádíme tato základní vyšetření, která by měla být provedena u každého dětského pacienta před odesláním do specializované ambulance.
Although the rising prevalence of hypertension among children in the whole world is lately often discussed, recent data from the Czech population are not available to answer seemingly an easy question "How many Czech children are hypertensive?". To determine estimate of the prevalence of hypertension we may base on the many other recent data which include studies performed in the USA, many European countries or the newest meta-analysis. The only published study performed in the Czech Republic until today is based on the data which will be soon twenty years old and thus can quite differ from the reality of today. The role of the general pediatrician in the diagnostics of hypertension and subsequent set of the basic examinations is still crucial even more as number of children with such diagnosis is rapidly increasing. In the review we state these basic examinations which should be performed in every child patient before referring to the specialized ambulance.
- MeSH
- časná diagnóza MeSH
- dítě MeSH
- hypertenze * diagnóza epidemiologie prevence a kontrola MeSH
- klinická studie jako téma MeSH
- krevní tlak MeSH
- lidé MeSH
- měření krevního tlaku metody MeSH
- mladiství MeSH
- prevalence MeSH
- směrnice pro lékařskou praxi jako téma MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- Publikační typ
- přehledy MeSH
BACKGROUND: Pediatric blood pressure (BP) assessment and management is increasingly important. Uncontrolled systolic and combined hypertension leads to hypertension-mediated organ damage. The impact of isolated diastolic hypertension is less clearly understood. METHODS: We analyzed the prevalence of ambulatory isolated diastolic hypertension (IDH) in primary (PH) and secondary (SH) hypertension, and associations with BMI Z-score (BMIz) and left ventricular mass index adjusted to the 95th percentile (aLVMI) in a large, multicenter cohort of hypertensive children. Hypertensive children were divided and analyzed in three ambulatory hypertension subgroups: 24-h, daytime, and nighttime. Specifically, we sought to determine the prevalence of ambulatory 24-h, daytime, or nighttime IDH. RESULTS: Prevalence of IDH varied based on ambulatory phenotypes, ranging from 6 to 12%, and was highest in children with SH. Children with IDH tended to be more likely female and, in some cases, were leaner than those with isolated systolic hypertension (ISH). Despite previous pediatric studies suggesting no strong association between diastolic blood pressure and left ventricular hypertrophy (LVH), we observed that children with IDH were equally likely to have LVH and had comparable aLVMI to those with ISH and combined systolic-diastolic hypertension. CONCLUSIONS: In summary, ambulatory IDH appears to be a unique phenotype with a female sex, and younger age predilection, but equal risk for LVH in children with either PH or SH.
- MeSH
- ambulantní monitorování krevního tlaku * MeSH
- diastola MeSH
- dítě MeSH
- hypertenze * epidemiologie diagnóza etiologie MeSH
- hypertrofie levé komory srdeční * epidemiologie etiologie diagnóza MeSH
- krevní tlak * MeSH
- lidé MeSH
- mladiství MeSH
- předškolní dítě MeSH
- prevalence MeSH
- rizikové faktory MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, which is mainly caused by pathogenic variants in two particular genes: PKD1 and PKD2. ADPKD caused by variants in other genes (GANAB or IFT140) is very rare. CASE REPORT: In a 6-year-old girl examined for abdominal pain, a cystic mass in the upper part of the right kidney was detected during an abdominal ultrasound. She was referred to pediatric oncology and urology for suspicion of a tumorous mass and the condition was assessed as a cystic nephroma. A heminephrectomy was then performed on the upper cystic part of the right kidney. The histological examination was inconclusive; therefore, genetic testing was recommended. Kidney and liver cysts were detected sonographically in the mother, but DNA analysis of the PKD1 and PKD2 genes did not reveal any pathogenic variant; the cause of the pathological formation in the kidneys remained unclear. Nine years later, next-generation sequencing of a panel of genes for kidney disease was performed and a heterozygous deletion was found on chromosome 16; this included exon 13 of the IFT140 gene. The same deletion was found in the patient's mother. Currently, the patient is 14 years old and has mild sonographic findings, normal glomerular filtration, mild proteinuria, and hypertension. CONCLUSION: Pathogenic variants of the IFT140 gene very rarely cause ADPKD; however, they should be considered in all children with autosomal dominant forms of PKD and asymmetric/atypical cystic kidney involvement or negative findings of PKD1 and PKD2.
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- MeSH
- ambulantní monitorování krevního tlaku * MeSH
- antihypertenziva terapeutické užití MeSH
- dítě MeSH
- hypertenze etiologie farmakoterapie diagnóza MeSH
- hypertrofie levé komory srdeční * etiologie patofyziologie MeSH
- krevní tlak * účinky léků MeSH
- lidé MeSH
- mladiství MeSH
- transplantace ledvin * škodlivé účinky MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- dopisy MeSH
