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

BACKGROUND: Primary, secondary and tertiary healthcare services in Europe create complex networks covering pediatric subspecialties, sociology, economics and politics. Two surveys of the European Society for Paediatric Nephrology (ESPN) in 1998 and 2017 revealed substantial disparities of kidney care among European countries. The purpose of the third ESPN survey is to further identify national differences in the conceptualization and organization of European pediatric kidney health care pathways during and outside normal working hours. METHODS: In 2020, a questionnaire was sent to one leading pediatric nephrologist from 48 of 53 European countries as defined by the World Health Organization. In order to exemplify care pathways in pediatric primary care nephrology, urinary tract infection (UTI) was chosen. Steroid sensitive nephrotic syndrome (SSNS) was chosen for pediatric rare disease nephrology and acute kidney injury (AKI) was analyzed for pediatric emergency nephrology. RESULTS: The care pathways for European children and young people with urinary tract infections were variable and differed during standard working hours and also during night-time and weekends. During daytime, UTI care pathways included six different types of care givers. There was a shift from primary care services outside standard working hours to general outpatient polyclinic and hospital services. Children with SNSS were followed up by pediatric nephrologists in hospitals in 69% of countries. Patients presenting with community acquired AKI were admitted during regular working hours to secondary or tertiary care hospitals. During nights and weekends, an immediate shift to University Children's Hospitals was observed where treatment was started by intensive care pediatricians and pediatric nephrologists. CONCLUSION: Gaps and fragmentation of pediatric health services may lead to the risk of delayed or inadequate referral of European children with kidney disease to pediatric nephrologists. The diversity of patient pathways outside of normal working hours was identified as one of the major weaknesses in the service chain.

• Publikační typ
• časopisecké články MeSH

• Publikační typ
• abstrakt z konference MeSH

BACKGROUND: Impaired kidney concentration capacity is present in half of the patients with autosomal dominant polycystic kidney disease (ADPKD). The kidney concentrating capacity was further impaired within the animal model of autosomal recessive polycystic kidney disease (ARPKD). To date, only one small study has investigated it in children having ARPKD. Therefore, we aimed to study the kidney concentrating ability in a larger cohort of children with ARPKD. METHODS: Eighteen children (median age 8.5 years, range 1.3-16.8) were retrospectively investigated. A standardized kidney concentrating capacity test was performed after the application of a nasal drop of desmopressin (urine osmolality > 900 mOsmol/kg). The glomerular filtration rate was estimated using the Schwartz formula (eGFR) and blood pressure (BP) was measured as office BP. RESULTS: Kidney concentrating capacity was decreased (urine osmolality < 900 mOsmol/kg) in 100% of children with ARPKD. The median urine osmolality after desmopressin application was 389 (range 235-601) mOsmol/kg. Sixteen patients (89%) were defined as hypertensive based on their actual BP level or their use of antihypertensive drugs. The maximum amounts of urinary concentration correlated significantly with eGFR (r = 0.72, p < 0.0001) and hypertensive scores (r = 0.50, p < 0.05), but not with kidney size. Twelve patients (67%) were defined as having CKD stages 2-4. The median concentrating capacity was significantly lower in children within this group, when compared to children with CKD stage 1 possessing a normal eGFR (544 mOsmol/kg, range 413-600 mOsmol/kg vs. 327 mOsmol/kg, range 235-417 mOsmol/l, p < 0.001). CONCLUSIONS: Impaired kidney concentrating capacity is present in most children with ARPKD and is associated with decreased eGFR and hypertension. A higher resolution version of the Graphical abstract is available as Supplementary information.

• MeSH
• chronická renální insuficience * komplikace   MeSH
• desmopresin MeSH
• dítě MeSH
• hodnoty glomerulární filtrace MeSH
• hypertenze * MeSH
• ledviny MeSH
• lidé MeSH
• polycystické ledviny autozomálně dominantní * MeSH
• polycystické ledviny autozomálně recesivní * komplikace   MeSH
• retrospektivní studie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

Accurate blood pressure (BP) measurement and its correct interpretation are essential factors influencing proper diagnosis and hypertension therapy. There are 5 methods of BP measurement. The office/casual/clinic blood pressure measurement has a central role as the most used method. This measurement is significantly influenced by its users and also by patient's response. Therefore, most of hypertension guidelines in adults but also in pediatrics have recommended out-of-office BP methods, i.e. home blood pressure measurement or ambulatory blood pressure monitoring. Automated office blood pressure measurement (AOBP) with the patient alone in a room is new method of BP measurement recommended for BP measurement in adult population. The aim of the paper is AOBP overview about its use in adults and children age.

• Klíčová slova
• automatické klinické měření krevního tlaku,
• MeSH
• ambulantní monitorování krevního tlaku metody   trendy   MeSH
• dítě MeSH
• hypertenze diagnostické zobrazování   prevence a kontrola   MeSH
• krevní tlak fyziologie   MeSH
• lidé MeSH
• měření krevního tlaku * klasifikace   metody   trendy   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• práce podpořená grantem MeSH
• přehledy MeSH

Souhrn Arteriální hypertenze je jedním z nejdůležitějších, hlavních rizikových faktorů kardiovaskulární morbidity a mortality u dospělých a kardiovaskulárních a renálních onemocnění v dětském věku. Tato doporučení pro diagnostiku a léčbu hypertenze u dětí vycházejí ze současných doporučení Evropské společnosti pro hypertenzi.

• MeSH
• anamnéza MeSH
• antihypertenziva aplikace a dávkování   klasifikace   MeSH
• dítě MeSH
• hypertenze * diagnóza   klasifikace   terapie   MeSH
• krevní tlak MeSH
• lidé MeSH
• měření krevního tlaku metody   MeSH
• mladiství MeSH
• statistika jako téma MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• práce podpořená grantem MeSH
• přehledy MeSH