BACKGROUND: Nutrition plays a vital role in the outcome of critically ill children, particularly those with AKI. Currently, there are no established guidelines for children with AKI treated with continuous RRT (CRRT). A thorough understanding of the metabolic changes and nutritional challenges in AKI and CRRT is required. Our objective was to create clinical practice points for nutritional assessment and management in critically ill children with AKI receiving CRRT. METHODS: PubMed, MEDLINE, Cochrane, and Embase databases were searched for articles related to the topic. Expertise of the authors and a consensus of the workgroup were additional sources of data in the article. Available articles on nutrition therapy in pediatric patients receiving CRRT through January 2023. RESULTS: On the basis of the literature review, the current evidence base was examined by a panel of experts in pediatric nephrology and nutrition. The panel used the literature review as well as their expertise to formulate clinical practice points. The modified Delphi method was used to identify and refine clinical practice points. CONCLUSIONS: Forty-four clinical practice points are provided on nutrition assessment, determining energy needs, and nutrient intake in children with AKI and on CRRT on the basis of the existing literature and expert opinions of a multidisciplinary panel.
- MeSH
- akutní poškození ledvin * terapie MeSH
- dítě MeSH
- konsensus MeSH
- kontinuální metody náhrady funkce ledvin * MeSH
- kritický stav terapie MeSH
- lidé MeSH
- nutriční stav MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND: Pediatric kidney transplantations are rarely performed, and there is limited knowledge about the diversity in current clinical practices across Europe. This study aims to explore the utility of clinical snapshot studies in identifying these disparities, establishing a foundation for future snapshot studies and standardization efforts. METHODS: A pilot clinical snapshot study was conducted, with invitations extended to all 109 pediatric kidney transplant centres in Europe. Each participating centre provided pre-, peri-, and postoperative data concerning their most recent thirty transplantations. The primary outcomes encompassed the evaluation of disparities in donor-recipient selection, surgical techniques, post-operative drainage procedures, and immunosuppressive therapy protocols. Secondary outcomes involved the analysis of rejection rates, incidence of infections, and graft survival. RESULTS: The study involved 439 patients from fifteen centres (14%) in twelve countries, with varying transplant volumes (range 1-29 transplantations per year) and follow-up periods. Significant differences were found among centres in terms of donor types, cold and warm ischemia time, pre-emptive transplant rates, and kidney transplant drainage methods. The rate of living donors varied between 3% and 90% and the median duration of cold ischemia ranged was 770 min after deceased donation and 147 min after living donation. Basiliximab was the dominant induction therapy, yet steroid withdrawal varied widely. Infection, rejection, and graft survival rates also varied significantly between centres. CONCLUSION: This study revealed substantial variation in clinical practices among European centres performing pediatric kidney transplantations. These findings could serve as a stimulus for international dialogue and collaboration.
- Publikační typ
- časopisecké články MeSH
V článku je zpracována problematika empirické perorální antibiotické terapie nejčastějších akutních komunitních bakteriální infekcí u dětí, a sice tonzilitidy, otitis media, sinusitidy, pneumonie, cystitidy, pyelonefritidy, erysipelu, flegmóny, impetiga a erythema migrans. Kromě antibiotik první volby jsou uvedeny i alternativy při výpadku antibiotik nebo při alergii. Součástí doporučení jsou také magistraliter receptury vybraných antibiotik. V článku je dále věnována pozornost klasifikaci antibiotik AWaRe, která rozděluje dostupná antibiotika do tří skupin podle rizika indukce rezistence bakterií.
This article addresses the issue of empirical oral antibiotic therapy for the most common acute community-acquired bacterial infections in children, specifically tonsillitis, otitis media, sinusitis, pneumonia, cystitis, pyelonephritis, erysipelas, cellulitis, impetigo, and erythema migrans. In addition to first-choice antibiotics, alternatives are provided in cases of antibiotic shortages or allergies. The recommendations also include magistral (compounded, pharmacist-prepared) formulas for selected antibiotics. The article further focuses on the AWaRe classification of antibiotics, which divides available antibiotics into three groups according to the risk of inducing bacterial resistance.
- MeSH
- antibakteriální látky * farmakologie klasifikace terapeutické užití MeSH
- antibiotická rezistence MeSH
- bakteriální infekce * epidemiologie farmakoterapie klasifikace MeSH
- bakteriální pneumonie farmakoterapie MeSH
- dítě MeSH
- impetigo etiologie farmakoterapie klasifikace MeSH
- lidé MeSH
- otitis media farmakoterapie klasifikace MeSH
- příprava léků klasifikace metody MeSH
- sinusitida farmakoterapie MeSH
- směrnice pro lékařskou praxi jako téma MeSH
- tonzilitida farmakoterapie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- přehledy MeSH
INTRODUCTION: Data on age-related differences in rejection rates, infectious episodes, and tacrolimus exposure in pediatric kidney transplant recipients (pKTRs) on a tacrolimus-based immunosuppressive regimen are scarce. METHODS: We performed a large-scale analysis of 802 pKTRs from the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) registry from 40 centers in 14 countries. The inclusion criteria were a tacrolimus-based immunosuppressive regimen and at least 2 years of follow-up. The patient population was divided into 3 age groups (infants and young children aged <6 years, school-aged children 6-12 years, and adolescents aged >12 years) to assess age-related differences in outcome. RESULTS: Median follow-up was 48 months (interquartile range [IQR], 36-72). Within the first 2 years posttransplant, infants, and young children had a significantly higher incidence of infections (80.6% vs. 55.0% in adolescents, P < 0.001) and a significantly higher number of cumulative hospital days (median 13 days vs. 7 days in adolescents, P < 0.001). Adolescents had a significantly higher rate of biopsy-proven acute rejection episodes in the first-year posttransplant (21.7%) than infants and young children (12.6%, P = 0.007). Infants and young children had significantly lower tacrolimus trough levels, lower tacrolimus concentration-to-dose (C/D) ratios as an approximation for higher tacrolimus clearance, and higher tacrolimus interpatient variability (TacIPV) (all P < 0.01) than adolescents. CONCLUSION: This is the largest study to date in European pKTRs on a tacrolimus-based immunosuppressive regimen, and it shows important age-related differences in rejection rates, infection episodes, as well as tacrolimus exposure and clearance. This data suggests that immunosuppressive therapy in pKTRs should be tailored and personalized according to the age-specific risk profiles of this heterogeneous patient population. The data may serve as a benchmark for future studies with novel immunosuppressive drugs.
- Publikační typ
- časopisecké články MeSH
This article provides a comprehensive overview of electrolyte and water homeostasis in pediatric patients, focusing on some of the common serum electrolyte abnormalities encountered in clinical practice. Understanding pathophysiology, taking a detailed history, performing comprehensive physical examinations, and ordering basic laboratory investigations are essential for the timely proper management of these conditions. We will discuss the pathophysiology, clinical manifestations, diagnostic approaches, and treatment strategies for each electrolyte disorder. This article aims to enhance the clinical approach to pediatric patients with electrolyte imbalance-related emergencies, ultimately improving patient outcomes.Trial registration This manuscript does not include a clinical trial; instead, it provides an updated review of literature.
- MeSH
- acidóza diagnóza krev terapie MeSH
- dítě MeSH
- elektrolyty krev MeSH
- hyperkalcemie terapie krev diagnóza etiologie MeSH
- hyperkalemie terapie diagnóza krev etiologie MeSH
- hypernatremie terapie diagnóza etiologie patofyziologie MeSH
- hypokalcemie diagnóza etiologie terapie MeSH
- hypokalemie terapie diagnóza krev etiologie MeSH
- hyponatremie terapie etiologie diagnóza MeSH
- lidé MeSH
- náhlé příhody * MeSH
- poruchy acidobazické rovnováhy diagnóza terapie patofyziologie MeSH
- vodní a elektrolytová nerovnováha * terapie MeSH
- vodní a elektrolytová rovnováha fyziologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
Schimke immuno-osseous dysplasia is a rare multisystemic disorder caused by biallelic loss of function of the SMARCAL1 gene that plays a pivotal role in replication fork stabilization and thus DNA repair. Individuals affected from this disease suffer from disproportionate growth failure, steroid resistant nephrotic syndrome leading to renal failure and primary immunodeficiency mediated by T cell lymphopenia. With infectious complications being the leading cause of death in this disease, researching the nature of the immunodeficiency is crucial, particularly as the state is exacerbated by loss of antibodies due to nephrotic syndrome or immunosuppressive treatment. Building on previous findings that identified the loss of IL-7 receptor expression as a possible cause of the immunodeficiency and increased sensitivity to radiation-induced damage, we have employed spectral cytometry and multiplex RNA-sequencing to assess the phenotype and function of T cells ex-vivo and to study changes induced by in-vitro UV irradiation and reaction of cells to the presence of IL-7. Our findings highlight the mature phenotype of T cells with proinflammatory Th1 skew and signs of exhaustion and lack of response to IL-7. UV light irradiation caused a severe increase in the apoptosis of T cells, however the expression of the genes related to immune response and regulation remained surprisingly similar to healthy cells. Due to the disease's rarity, more studies will be necessary for complete understanding of this unique immunodeficiency.
- MeSH
- apoptóza genetika MeSH
- arterioskleróza genetika etiologie imunologie MeSH
- dítě MeSH
- DNA-helikasy genetika MeSH
- lidé MeSH
- metabolické nemoci kostí etiologie genetika MeSH
- nefrotický syndrom etiologie genetika MeSH
- oprava DNA * genetika MeSH
- osteochondrodysplazie * genetika imunologie MeSH
- plicní embolie genetika etiologie MeSH
- poruchy růstu genetika etiologie MeSH
- primární imunodeficience * genetika diagnóza imunologie MeSH
- syndromy imunologické nedostatečnosti genetika imunologie MeSH
- T-lymfocyty imunologie MeSH
- ultrafialové záření škodlivé účinky MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Volume depletion is a common condition and a frequent cause of hospitalization in children. Proper assessment of the patient includes a detailed history and a thorough physical examination. Biochemical tests may be useful in selected cases. Understanding the pathophysiology of fluid balance is necessary for appropriate management. A clinical dehydration scale assessing more physical findings may help to determine dehydration severity. Most dehydrated children can be treated orally; however, intravenous therapy may be indicated in patients with severe volume depletion, in those who have failed oral therapy, or in children with altered consciousness or significant metabolic abnormalities. Proper management consists of restoring circulatory volume and electrolyte balance. In this paper, we review clinical aspects, diagnosis, and management of children with volume depletion.
- MeSH
- dehydratace * diagnóza etiologie terapie MeSH
- dítě MeSH
- fyzikální vyšetření MeSH
- lidé MeSH
- tekutinová terapie * škodlivé účinky MeSH
- vodní a elektrolytová rovnováha MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
There is no specific treatment for proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID), a disease that is very rare in the pediatric population. We report the case of a 15-year-old boy who presented with mildly reduced kidney function and nephrotic syndrome. Kidney biopsy revealed PGNMID with monoclonal deposits of IgG3 with kappa light chain restriction. Flow cytometry showed a significant CD38 plasma cell population in the peripheral blood in the absence of other signs of hematological malignancy. The patient was treated with a 6-month course of daratumumab, a monoclonal antibody targeting CD38. There was a significant reduction in proteinuria and normalization of kidney function. Based on positive experience with adults, daratumumab should also be studied in children with PGNMID.
- MeSH
- antigeny CD38 imunologie analýza MeSH
- biopsie MeSH
- imunoglobulin G * krev MeSH
- ledviny patologie imunologie účinky léků MeSH
- lidé MeSH
- membranoproliferativní glomerulonefritida * farmakoterapie imunologie patologie MeSH
- mladiství MeSH
- monoklonální protilátky * terapeutické užití MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
INTRODUCTION: Hepatocyte nuclear factor 1-beta (HNF1B) gene variants or the chromosome 17q12 deletion (17q12del) represent the most common monogenic cause of developmental kidney disease. Although neurodevelopmental disorders have been associated with the 17q12del, specific genotype-phenotype associations with respect to kidney function evolution have not yet been fully defined. Here, we aimed to determine whether 17q12del or specific HNF1B variants were associated with kidney survival in a large patient population with HNF1B disease. METHODS: This was a retrospective observational study involving 521 patients with HNF1B disease from 14 countries using the European Reference Network for rare kidney diseases with detailed information on the HNF1B genotype (HNF1B variants or the 17q12del). Median follow-up time was 11 years with 6 visits per patient. The primary end point was progression to chronic kidney disease (CKD) stage 3 (estimated glomerular filtration rate [eGFR] < 60 ml/min per 1.73 m2). Secondary end points were the development of hypomagnesemia or extrarenal disorders, including hyperuricemia and hyperglycemia. RESULTS: Progression toward CKD stage 3 was significantly delayed in patients with the 17q12del compared to patients with HNF1B variants (hazard ratio [HR]: 0.29, 95% confidence interval [CI]: 0.19-0.44, P < 0.001). Progression toward CKD stage 3 was also significantly delayed when HNF1B variants involved the HNF1B Pit-1, Oct-1, and Unc-86 homeodomain (POUh) DNA-binding and transactivation domains rather than the POU-specific domain (POUs) DNA-binding domain (HR: 0.15 [95% CI: 0.06-0.37), P < 0.001 and HR: 0.25 (95% CI: 0.11-0.57), P = 0.001, respectively). Finally, the 17q12del was positively associated with hypomagnesemia and negatively associated with hyperuricemia, but not with hyperglycemia. CONCLUSION: Patients with the 17q12del display a significantly better kidney survival than patients with other HNF1B variants; and for the latter, variants in the POUs DNA-binding domain lead to the poorest kidney survival. These are clinically relevant HNF1B kidney genotype-phenotype correlations that inform genetic counseling.
- Publikační typ
- časopisecké články MeSH
