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  1. Article ; Online: Fluid management in children with volume depletion.

    Zieg, Jakub / Narla, Deepti / Gonsorcikova, Lucie / Raina, Rupesh

    Pediatric nephrology (Berlin, Germany)

    2023  Volume 39, Issue 2, Page(s) 423–434

    Abstract: 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 ...

    Abstract 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 term(s) Child ; Humans ; Dehydration/diagnosis ; Dehydration/etiology ; Dehydration/therapy ; Fluid Therapy/adverse effects ; Water-Electrolyte Balance ; Physical Examination
    Language English
    Publishing date 2023-07-14
    Publishing country Germany
    Document type Review ; Journal Article
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-023-06080-z
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Practical recommendations for the management of children after kidney and liver transplantation.

    Zieg, Jakub / Bauer, David / Krejčová, Vlasta / Gonsorčíková, Lucie

    Casopis lekaru ceskych

    2023  Volume 161, Issue 7-8, Page(s) 296–302

    Abstract: The number of pediatric solid organ transplantations is growing. This therapy leads often to better quality of life but also brings some specific complications. Our review summarizes practical recommendations for long-time care of the children after ... ...

    Title translation Praktická doporučení pro péči o dětské pacienty po transplantaci ledviny a jater.
    Abstract The number of pediatric solid organ transplantations is growing. This therapy leads often to better quality of life but also brings some specific complications. Our review summarizes practical recommendations for long-time care of the children after kidney and liver transplantation. The knowledge of the issues related to transplantation is essential for the first contact physicians, whose cooperation with transplant centre contributes highly to adequate management of these children.
    MeSH term(s) Child ; Humans ; Liver Transplantation ; Quality of Life ; Kidney ; Organ Transplantation ; Physicians
    Language English
    Publishing date 2023-02-06
    Publishing country Czech Republic
    Document type Review ; Journal Article
    ZDB-ID 413441-2
    ISSN 1805-4420 ; 0008-7335
    ISSN (online) 1805-4420
    ISSN 0008-7335
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  3. Article: The role of endoscopy in non-oncologic gastrointestinal disorders in pediatric patients.

    Gonsorčíková, Lucie / Netvalová, Silvie / Vyhnánek, Radim / Bauer, David / Fabián, Ondřej

    Ceskoslovenska patologie

    2022  Volume 58, Issue 2, Page(s) 100–106

    Abstract: Gastrointestinal (GIT) diseases represent an important part of pediatric health disorders. The recent years have brought not only significant improvement of digestive endoscopy technologies and a new equipment suitable for pediatric age but also progress ...

    Title translation Endoskopická diagnostika nejčastějších nenádorových onemocnění gastrointestinálního traktu v pediatrii.
    Abstract Gastrointestinal (GIT) diseases represent an important part of pediatric health disorders. The recent years have brought not only significant improvement of digestive endoscopy technologies and a new equipment suitable for pediatric age but also progress in management of diagnostic approach and treatment of the pediatric GIT diseases. In contrast to adult patients, endoscopic examination in pediatrics is in most cases performed for diagnostic, not therapeutical purposes. The histological assessment of biopsy specimens taken during endoscopy therefore forms an integral part of the endoscopic examination and in most cases the diagnosis cannot be concluded without their evaluation. In particular, the clinical gastroenterologist expects from the pathologist a description that will help confirm or contradict the diagnosis considered after the macroscopic examination. In this review, we would like to highlight the most common endoscopic findings of the gastrointestinal tract in pediatric population and the role of histology in determining the correct diagnosis.
    MeSH term(s) Adult ; Child ; Endoscopy, Gastrointestinal ; Gastrointestinal Diseases/diagnosis ; Gastrointestinal Diseases/pathology ; Humans
    Language English
    Publishing date 2022-07-26
    Publishing country Czech Republic
    Document type Journal Article ; Review
    ZDB-ID 138273-1
    ISSN 1210-7875 ; 0009-0611 ; 0371-1854
    ISSN 1210-7875 ; 0009-0611 ; 0371-1854
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  4. Article ; Online: Current views on the diagnosis and management of hypokalaemia in children.

    Zieg, Jakub / Gonsorcikova, Lucie / Landau, Daniel

    Acta paediatrica (Oslo, Norway : 1992)

    2016  Volume 105, Issue 7, Page(s) 762–772

    Abstract: Unlabelled: Hypokalaemia is a common electrolyte disorder in children, caused by decreased potassium intake, increased gastrointestinal and urinary losses or transcellular shift. Patients with severe hypokalaemia may suffer from symptoms such as life- ... ...

    Abstract Unlabelled: Hypokalaemia is a common electrolyte disorder in children, caused by decreased potassium intake, increased gastrointestinal and urinary losses or transcellular shift. Patients with severe hypokalaemia may suffer from symptoms such as life-threatening cardiac arrhythmias. The aim of our study was to review the aetiology of hypokalaemia, suggest a diagnostic algorithm and discuss the management of patients with various aetiologies of hypokalaemia.
    Conclusion: Understanding the pathophysiology of hypokalaemic states, along with a detailed medical history, physical examination and specific laboratory tests are required for proper diagnosis and appropriate treatment.
    MeSH term(s) Adrenal Cortex Diseases/complications ; Child ; Disease Management ; Genetic Diseases, Inborn/complications ; Humans ; Hypokalemia/diagnosis ; Hypokalemia/etiology ; Hypokalemia/therapy
    Language English
    Publishing date 2016-07
    Publishing country Norway
    Document type Journal Article ; Review
    ZDB-ID 203487-6
    ISSN 1651-2227 ; 0365-1436 ; 0803-5253
    ISSN (online) 1651-2227
    ISSN 0365-1436 ; 0803-5253
    DOI 10.1111/apa.13398
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  5. Article ; Online: The Accuracy of a Home-performed Faecal Calprotectin Test in Paediatric Patients With Inflammatory Bowel Disease.

    Lerchova, Tereza / Hradsky, Ondrej / Copova, Ivana / Potuznikova, Kristyna / Gonsorcikova, Lucie / Bronsky, Jiri

    Journal of pediatric gastroenterology and nutrition

    2019  Volume 69, Issue 1, Page(s) 75–81

    Abstract: Objectives: Owing to the invasiveness of endoscopy, the use of biomarkers, especially faecal calprotectin (FC), has become standard for remission assessment. This study aimed to compare the accuracy for detection of endoscopic activity using recently ... ...

    Abstract Objectives: Owing to the invasiveness of endoscopy, the use of biomarkers, especially faecal calprotectin (FC), has become standard for remission assessment. This study aimed to compare the accuracy for detection of endoscopic activity using recently developed FC home test using smartphone application (FC-IBDoc) against standard enzyme-linked immunosorbent assay (ELISA).
    Methods: In all, 102 consecutive observations (89 participants) were included in prospective observational study. FC-IBDoc was performed parallelly with FC-ELISA in paediatric patients with inflammatory bowel disease indicated for endoscopy. Both tests were performed by trained staff. Mucosal healing was defined using Simple Endoscopic Score for Crohn disease (CD) ≤2 in patients with CD (n = 44), ulcerative colitis (UC) Endoscopic Index of Severity ≤4 in patients with UC (n = 27) and Rutgeerts score i0 and i1 without colon involvement in patients with CD after ileocaecal resection (n = 19).
    Results: Out of 102 endoscopic findings 23 were assessed as mucosal healing. We found an association of the mucosal healing scores of the entire group both with FC-ELISA (P = 0.002) and FC-IBDoc (P = 0.001). The area under the receiver operating characteristic curve for FC-ELISA was 0.883 (95% confidence interval 0.807-0.960), with optimal cut-off at 136.5 μg/g. The area under the receiver operating characteristic curve for FC-IBDoc was 0.792 (95% confidence interval 0.688-0.895) with optimal cut-off at 48 μg/g. The FC-ELISA was more accurate than FC-IBDoc when tested by a Delong test (P = 0.023).
    Conclusions: Standard FC-ELISA for FC evaluation is more reliable predictor of mucosal healing than the FC-IBDoc in paediatric patients with inflammatory bowel disease. The cut-off values for both tests were incongruous.
    MeSH term(s) Adolescent ; Area Under Curve ; Biomarkers/analysis ; Child ; Colitis, Ulcerative/diagnostic imaging ; Colitis, Ulcerative/metabolism ; Crohn Disease/diagnostic imaging ; Crohn Disease/metabolism ; Endoscopy, Gastrointestinal ; Enzyme-Linked Immunosorbent Assay ; Feces/chemistry ; Female ; Humans ; Intestinal Mucosa/diagnostic imaging ; Intestinal Mucosa/physiopathology ; Leukocyte L1 Antigen Complex/analysis ; Male ; Mobile Applications ; Prospective Studies ; ROC Curve ; Reagent Kits, Diagnostic/standards ; Reproducibility of Results ; Self Care ; Severity of Illness Index ; Smartphone ; Wound Healing
    Chemical Substances Biomarkers ; Leukocyte L1 Antigen Complex ; Reagent Kits, Diagnostic
    Language English
    Publishing date 2019-03-21
    Publishing country United States
    Document type Comparative Study ; Journal Article ; Observational Study ; Research Support, Non-U.S. Gov't
    ZDB-ID 603201-1
    ISSN 1536-4801 ; 0277-2116
    ISSN (online) 1536-4801
    ISSN 0277-2116
    DOI 10.1097/MPG.0000000000002331
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  6. Article ; Online: Sustainability of biologic treatment in paediatric patients with Crohn's disease: population-based registry analysis.

    Hradsky, Ondrej / Copova, Ivana / Durilova, Marianna / Kazeka, Denis / Lerchova, Tereza / Mitrova, Katarina / Schwarz, Jan / Vetrovcova, Romana / El-Lababidi, Nabil / Karaskova, Eva / Veghova-Velganova, Maria / Sulakova, Astrid / Gonsorčíková, Lucie / Veverkova, Marketa / Zeniskova, Ivana / Zimen, Martin / Bortlik, Martin / Bronsky, Jiri

    Pediatric research

    2023  

    Abstract: Background: We aimed to evaluate the predictors of sustainability of biologic drugs for paediatric patients with Crohn's disease (CD).: Methods: The Czech National Prospective Registry of Biologic and Targeted Therapy of Inflammatory Bowel Disease ( ... ...

    Abstract Background: We aimed to evaluate the predictors of sustainability of biologic drugs for paediatric patients with Crohn's disease (CD).
    Methods: The Czech National Prospective Registry of Biologic and Targeted Therapy of Inflammatory Bowel Disease (CREdIT) was used to identify the biologic treatment courses in paediatric patients with CD. Mixed-effects Cox models and propensity score analyses were employed to evaluate predictors of treatment sustainability.
    Results: Among the 558 observations of 473 patients, 264 were treated with adalimumab (47%), 240 with infliximab (43%), 41 with ustekinumab (7%), and 13 with vedolizumab (2%). Multivariable analysis revealed higher discontinuation risk with infliximab compared to adalimumab (HR = 0.600, 95%CI 0.389-0.926), both overall and in first-line treatment (HR = 0.302, 95%CI 0.103-0.890). Infliximab versus adalimumab was associated with shorter time to escalation (HR = 0.094, 95%CI 0.043-0.203). Propensity-score analysis demonstrated lower sustainability of infliximab (HR = 0.563, 95%CI 1.159-2.725). The time since diagnosis to treatment initiation (HR = 0.852, 95%CI 0.781-0.926) was the most important predictor. Baseline immunosuppressive therapy prolonged sustainability with infliximab (HR = 2.899, 95%CI 1.311-6.410).
    Conclusions: Given the results suggesting shorter sustainability, the need for earlier intensification and thus higher drug exposure, and the greater need for immunosuppression with infliximab than with adalimumab, the choice of these drugs cannot be considered completely equitable.
    Impact: Our study identified predictors of sustainability of biologic treatment in paediatric patients with Crohn's disease, including adalimumab (versus infliximab), early initiation of biologic treatment, and normalised baseline haemoglobin levels. Infliximab treatment was associated with earlier intensification, higher drug exposure, and a greater need for immunosuppression. Parents and patients should be fully informed of the disadvantages of intravenous infliximab versus adalimumab during the decision-making process. This study emphasises the importance of not delaying the initiation of biologic therapy in paediatric patients with Crohn's disease.
    Language English
    Publishing date 2023-11-27
    Publishing country United States
    Document type Journal Article
    ZDB-ID 4411-8
    ISSN 1530-0447 ; 0031-3998
    ISSN (online) 1530-0447
    ISSN 0031-3998
    DOI 10.1038/s41390-023-02913-7
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  7. Article ; Online: Is It Useful to Monitor Thiopurine Metabolites in Pediatric Patients with Crohn's Disease on Combination Therapy? A Multicenter Prospective Observational Study.

    Pospisilova, Kristyna / Siroka, Jitka / Karaskova, Eva / Hradsky, Ondrej / Lerchova, Tereza / Zarubova, Kristyna / Copova, Ivana / Gonsorcikova, Lucie / Velganova-Veghova, Maria / Francova, Irena / Urbanek, Lubor / Geryk, Milos / Mihal, Vladimir / Bronsky, Jiri

    Paediatric drugs

    2021  Volume 23, Issue 2, Page(s) 183–194

    Abstract: Background: The additional value of azathioprine concomitant treatment on infliximab pharmacokinetics in children is not well described yet.: Aims: In the present study, we aimed to describe the relationship between thiopurine metabolite levels, ... ...

    Abstract Background: The additional value of azathioprine concomitant treatment on infliximab pharmacokinetics in children is not well described yet.
    Aims: In the present study, we aimed to describe the relationship between thiopurine metabolite levels, infliximab trough levels, anti-IFX antibody formation, and clinical and laboratory markers of disease activity in pediatric patients with Crohn's disease, and to assess non-adherence.
    Methods: Data were collected prospectively during repeated visits from pediatric patients followed for Crohn's disease in two Czech pediatric inflammatory bowel disease centers between January 2016 and June 2017. Thiopurine metabolites (6-thioguanine and 6-methylmercaptopurine) were measured by high-performance liquid chromatography. Infliximab trough levels and anti-IFX antibody serum levels were measured routinely by ELISA. The risk of loss of response to infliximab therapy was also assessed.
    Results: A significant association between infliximab serum levels and 6-thioguanine erythrocyte levels was observed when tested as categorical variables (63 patients, 321 observations). To predict infliximab levels > 5 µg/mL, we propose a 6-thioguanine cutoff of 278 pmol/8 × 10
    Conclusion: Thiopurine metabolite monitoring in pediatric patients with Crohn's disease is useful when optimizing combination therapy. Pediatric patients with undetectable 6-thioguanine levels are more likely to lose response to infliximab therapy. When targeting optimal infliximab levels, the 6-thioguanine cutoff levels in children appear to be higher than in adults.
    MeSH term(s) Adolescent ; Azathioprine/therapeutic use ; Biomarkers ; Child ; Crohn Disease/drug therapy ; Drug Therapy, Combination ; Female ; Humans ; Immunologic Factors/therapeutic use ; Infliximab/therapeutic use ; Longitudinal Studies ; Male ; Mercaptopurine/analogs & derivatives ; Mercaptopurine/analysis ; Prospective Studies
    Chemical Substances Biomarkers ; Immunologic Factors ; 6-methylthiopurine (6V404DV25O) ; Infliximab (B72HH48FLU) ; Mercaptopurine (E7WED276I5) ; Azathioprine (MRK240IY2L)
    Language English
    Publishing date 2021-03-11
    Publishing country Switzerland
    Document type Journal Article ; Multicenter Study ; Observational Study
    ZDB-ID 1492748-2
    ISSN 1179-2019 ; 1174-5878
    ISSN (online) 1179-2019
    ISSN 1174-5878
    DOI 10.1007/s40272-021-00439-1
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  8. Article ; Online: Correction to: Fecal calprotectin is not a clinically useful marker for the prediction of the early nonresponse to exclusive enteral nutrition in pediatric patients with Crohn disease.

    Copova, Ivana / Hradsky, Ondrej / Zarubova, Kristyna / Gonsorcikova, Lucie / Potuznikova, Kristyna / Lerchova, Tereza / Nevoral, Jiri / Bronsky, Jiri

    European journal of pediatrics

    2018  Volume 177, Issue 11, Page(s) 1695

    Abstract: This article was originally published with all author names incorrectly listed. All author names have now been transposed and appear correctly above. The original article was corrected. ...

    Abstract This article was originally published with all author names incorrectly listed. All author names have now been transposed and appear correctly above. The original article was corrected.
    Language English
    Publishing date 2018-10-02
    Publishing country Germany
    Document type Journal Article ; Published Erratum
    ZDB-ID 194196-3
    ISSN 1432-1076 ; 0340-6199 ; 0943-9676
    ISSN (online) 1432-1076
    ISSN 0340-6199 ; 0943-9676
    DOI 10.1007/s00431-018-3260-5
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  9. Article ; Online: Fecal calprotectin is not a clinically useful marker for the prediction of the early nonresponse to exclusive enteral nutrition in pediatric patients with Crohn disease.

    Copova, Ivana / Hradsky, Ondrej / Zarubova, Kristyna / Gonsorcikova, Lucie / Potuznikova, Kristyna / Lerchova, Tereza / Nevoral, Jiri / Bronsky, Jiri

    European journal of pediatrics

    2018  Volume 177, Issue 11, Page(s) 1685–1693

    Abstract: Exclusive enteral nutrition (EEN) has been recommended as the first-line therapy in children with active Crohn disease (CD). The primary aim of our study was to determine whether it is possible to use the difference between basal fecal calprotectin (F- ... ...

    Abstract Exclusive enteral nutrition (EEN) has been recommended as the first-line therapy in children with active Crohn disease (CD). The primary aim of our study was to determine whether it is possible to use the difference between basal fecal calprotectin (F-CPT) and the value at week 2 of EEN to predict clinical response at week 6. We prospectively collected stool samples for F-CPT analysis and clinical and laboratory parameters during EEN from 38 pediatric patients (28 boys, median age 12.8 years) with newly diagnosed active luminal CD. The difference between F-CPT concentrations before EEN and at week 2 did not predict clinical non-response at week 6 (OR 0.9996 95% CI 0.9989-1.0002, p = 0.18); however, it predicted patients who did not achieve clinical remission at week 6 (OR 0.9993, 95% CI 00.9985-0.9998, p = 0.006) with sensitivity of 58%, and specificity of 92% for cut-off of F-CPT increase by 486 μg/g.Conclusions: An early decrease in F-CPT levels in children with newly diagnosed active luminal CD did not predict clinical response at week 6 of EEN induction therapy, and clinical remission was predicted with low accuracy. Therefore, F-CPT cannot be used as a predictor to select the patients in whom EEN should be terminated. What is Known: • The fecal calprotectin (F-CPT) is an important marker of intestinal inflammation. • Approximately 25% of pediatric patients with Crohn disease (CD) do not achieve clinical remission, and there is still no sufficient predictor of response to exclusive enteral nutrition (EEN) treatment. What is New: • The difference between the F-CPT concentrations before EEN treatment and at week 2 did not predict clinical response to treatment at week 6, even if it predicted clinical remission, however, with low accuracy. F-CPT is not a suitable predictor to select the patients for discontinuing of EEN induction therapy.
    MeSH term(s) Adolescent ; Biomarkers/analysis ; Child ; Crohn Disease/metabolism ; Crohn Disease/therapy ; Enteral Nutrition/adverse effects ; Feces/chemistry ; Female ; Humans ; Leukocyte L1 Antigen Complex/analysis ; Male ; Prospective Studies ; Sensitivity and Specificity ; Treatment Outcome
    Chemical Substances Biomarkers ; Leukocyte L1 Antigen Complex
    Language English
    Publishing date 2018-08-20
    Publishing country Germany
    Document type Journal Article ; Observational Study
    ZDB-ID 194196-3
    ISSN 1432-1076 ; 0340-6199 ; 0943-9676
    ISSN (online) 1432-1076
    ISSN 0340-6199 ; 0943-9676
    DOI 10.1007/s00431-018-3228-5
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  10. Article ; Online: Familial mild hyperglycemia associated with a novel ABCC8-V84I mutation within three generations.

    Gonsorcikova, Lucie / Vaxillaire, Martine / Pruhova, Stepanka / Dechaume, Aurélie / Dusatkova, Petra / Cinek, Ondrej / Pedersen, Oluf / Froguel, Philippe / Hansen, Torben / Lebl, Jan

    Pediatric diabetes

    2011  Volume 12, Issue 3 Pt 2, Page(s) 266–269

    Abstract: We present a unique case of a 19-year-old man with a positive family history of persistent mild hyperglycemia and a novel V84I mutation in ABCC8. The proband was initially detected to have fasting hyperglycemia (ranging 6.1-6.4 mmol/L) at the age of 12 ... ...

    Abstract We present a unique case of a 19-year-old man with a positive family history of persistent mild hyperglycemia and a novel V84I mutation in ABCC8. The proband was initially detected to have fasting hyperglycemia (ranging 6.1-6.4 mmol/L) at the age of 12 years. Increased fasting blood glucose was also subsequently detected in five additional family members (in his twin brother, sister, mother, maternal aunt, and grandfather). The grandfather has been known to have mild diabetes since 30 years and has never been treated. After having excluded a causative mutation in five maturity-onset diabetes of the young genes (MODY1-4 and 6), we identified a novel ABCC8 V84I mutation, which segregated with autosomal dominant transmission of mild hyperglycemia within three generations. This mutation that is located in a conserved area of transmembrane domain TMD0 seems to be a rare cause of clinical phenotype resembling glucokinase-deficient diabetes.
    MeSH term(s) ATP-Binding Cassette Transporters/genetics ; Adolescent ; Adult ; Child ; Genes, Dominant ; Humans ; Hyperglycemia/genetics ; Male ; Middle Aged ; Potassium Channels, Inwardly Rectifying/genetics ; Receptors, Drug/genetics ; Sulfonylurea Receptors ; Young Adult
    Chemical Substances ATP-Binding Cassette Transporters ; Potassium Channels, Inwardly Rectifying ; Receptors, Drug ; Sulfonylurea Receptors
    Language English
    Publishing date 2011-01-09
    Publishing country Denmark
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1502504-4
    ISSN 1399-5448 ; 1745-1426 ; 1399-543X
    ISSN (online) 1399-5448
    ISSN 1745-1426 ; 1399-543X
    DOI 10.1111/j.1399-5448.2010.00719.x
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