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1. Nephronophthisis-Related Ciliopathies

Author

Stokman, M.F., Lilien, M., and Knoers, N.

Subjects
Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11]
Abstract

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Published
2023

2. Environmental and parental risk factors for congenital solitary functioning kidney — a case–control study.

Author

Groen in 't Woud, Sander, Roeleveld, Nel, van Rooij, Iris A. L. M., Feitz, Wout F. J., Schreuder, Michiel F., van der Zanden, Loes F. M., van Wijk, J. A. E., Westland, R., Renkema, K. Y., Lilien, M. R., Keijzer-Veen, M. G., Kloosterman, F. J., Steffens, M. G., Gracchi, V., Zegers, B., Jira, P. E., van der Deure, H., van Rooij, R. W. G., Wijnands - van den Berg, E., and Breukels, M.

Subjects
LIFESTYLES, OBESITY, CONFIDENCE intervals, KIDNEY failure, CASE-control method, DIABETES, KIDNEY abnormalities, QUESTIONNAIRES, DESCRIPTIVE statistics, RESEARCH funding, FOLIC acid, FERTILIZATION in vitro, ODDS ratio, SMOKING, ENVIRONMENTAL exposure, PARENTS, DISEASE risk factors, DISEASE complications
Abstract

Background: The etiology of congenital solitary functioning kidney (CSFK) is largely unknown but likely includes various risk factors. We performed a case–control study to compare exposure to environmental and parental risk factors during embryonic kidney development between children with CSFK and healthy controls. Methods: We included 434 children with CSFK and 1302 healthy controls from the AGORA data- and biobank matched on year of birth. Exposure to potential risk factors was investigated using parental questionnaire data. Crude and adjusted odds ratios (aORs) with 95% confidence intervals (CIs) were estimated for each potential risk factor. Multiple imputation was used to deal with missing values. Confounders for each potential risk factor were selected using directed acyclic graphs. Results: Maternal stress was newly identified as a risk factor for CSFK (aOR 2.1, 95% CI 1.2–3.5). Known associations with conception using in vitro fertilization/intracytoplasmic sperm injection (aOR 1.8, 95% CI 1.0–3.2), maternal infections during pregnancy (aOR 2.5, 95% CI 1.4–4.7), smoking during pregnancy (aOR 1.4, 95% CI 1.0–2.0), and parental CAKUT (aOR 6.6, 95% CI 2.9–15.1) were confirmed, but previous associations with diabetes and obesity could not be replicated. Folic acid supplement use and younger maternal age seemed to reduce the risk of CSFK (aORs 0.7, 95% CI 0.5–1.0, and 0.8, 95% CI 0.6–1.0, respectively). Conclusions: Environmental and parental risk factors are likely to be involved in the development of CSFK and future studies should combine genetic, environmental, and gene-environment interaction analyses. Women wanting to become pregnant should consider optimizing their health and lifestyle. [ABSTRACT FROM AUTHOR]

Published
2023
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4. GeNepher: building a data- and biobank for (suspected) hereditary renal disease

Author

Claus, L. R., Boulogne, F., Lilien, M. R., Rookmaaker, M. B., van der Zwaag, A., Nguyen, T. Q., Verhaar, M. C., Knoers, N. V. A. M., Deelen, P., Franke, L., de Borst, M. H., van Eerde, A. M., Stem Cell Aging Leukemia and Lymphoma (SALL), Groningen Institute for Gastro Intestinal Genetics and Immunology (3GI), Groningen Institute for Organ Transplantation (GIOT), and Groningen Kidney Center (GKC)

Published
2020

14. Treatment and long-term outcome in primary distal renal tubular acidosis

Author

Lopez-Garcia SC, Emma F, Walsh SB, Fila M, Hooman N, Zaniew M, Bertholet-Thomas A, Colussi G, Burgmaier K, Levtchenko E, Sharma J, Singhal J, Soliman NA, Ariceta G, Basu B, Murer L, Tasic V, Tsygin A, Decramer S, Gil-Peña H, Koster-Kamphuis L, La Scola C, Gellermann J, Konrad M, Lilien M, Francisco T, Tramma D, Trnka P, Yüksel S, Caruso MR, Chromek M, Ekinci Z, Gambaro G, Kari JA, König J, Taroni F, Thumfart J, Trepiccione F, Winding L, Wühl E, Ağbaş A, Belkevich A, Vargas-Poussou R, and Blanchard A

Subjects
Acidosis, Renal Tubular/complications/genetics/*therapy, Adolescent, Adult, Aged, Bicarbonates/blood, Calcium/urine, Child, Child, Preschool, Cohort Studies, DNA Mutational Analysis, Deafness/complications/genetics/therapy, Female, Genetic Association Studies, Glomerular Filtration Rate, Hearing Loss, Sensorineural/complications/genetics/*therapy, Humans, Infant, Infant, Newborn, Male, Middle Aged, Mutation, Nephrocalcinosis/complications/genetics/therapy, Rare Diseases/complications, Vacuolar P
Abstract

BACKGROUND: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. METHODS: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. RESULTS: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate. CONCLUSION: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.

Published
2019

18. Abstract P-286

Author

Raymakers-Janssen, P., primary, Tibboel, D., additional, Cransberg, K., additional, Kneyber, M., additional, van den Heuvel-Eibrink, M., additional, Lilien, M., additional, and Wösten-van Asperen, R., additional

Published
2018
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19. KOUNCIL: Kidney-Oriented Understanding of Correcting Ciliopathies

Author

Stokman, M, primary, Oud, M, additional, Reeuwijk, J Van, additional, Lilien, M, additional, De Kar, N Van, additional, Nijman, I, additional, Gilissen, C, additional, Kroes, HY, additional, Bongers, E, additional, Geijsen, N, additional, Kamsteeg, E, additional, Cuppen, E, additional, Roepman, R, additional, Giles, R, additional, Renkema, K, additional, Arts, H, additional, and Knoers, N, additional

Published
2015
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20. SP001TARGETED SEQUENCING OF 399 RENAL GENES RECLASSIFIES PRIMARY DISEASE DIAGNOSES IN YOUNG ESRD PATIENTS

Author

van Eerde, Albertien M., primary, van der Zwaag, A., additional, de Borst, M. H., additional, Peters, E. J., additional, Renkema, K. Y., additional, Elferink, M., additional, van Zon, P. H.A., additional, Lilien, M. R., additional, van Haaften, G. W., additional, Giles, R. H., additional, Navis, G. J., additional, and Knoers, N.V. A.M., additional

Published
2015
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22. Children of non-Western origin with end-stage renal disease in the Netherlands, Belgium and a part of Germany have impaired health-related quality of life compared with Western children

Author

Schoenmaker, N. J., primary, Haverman, L., additional, Tromp, W. F., additional, van der Lee, J. H., additional, Offringa, M., additional, Adams, B., additional, Bouts, A. H. M., additional, Collard, L., additional, Cransberg, K., additional, van Dyck, M., additional, Godefroid, N., additional, van Hoeck, K., additional, Koster-Kamphuis, L., additional, Lilien, M. R., additional, Raes, A., additional, Taylan, C., additional, Grootenhuis, M. A., additional, and Groothoff, J. W., additional

Published
2013
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23. Bone mineral density, bone metabolism and body composition of children with chronic renal failure, with and without growth hormone treatment

Author

Boot, A. M., Nauta, J., de Jong, M. C., Groothoff, J. W., Lilien, M. R., van Wijk, J. A., Kist-van Holthe, J. E., Hokken-Koelega, A. C., Pols, H. A., de Muinck Keizer-Schrama, S. M., Pediatrics, Internal Medicine, and Other departments

Subjects
musculoskeletal diseases, Aangeboren stoornissen in magnesiumtransport. Genetica en Pathophysiologie, Heriditary disorders of magnesiumtransport. Genetic localisation and pathophysiology
Abstract

OBJECTIVE: Osteopenia has been reported in adult patients with chronic renal failure (CRF). Only a few studies have been performed in children. The objective of this study was to evaluate bone mineral density (BMD), bone turnover, body composition in children with CRF and to study the effect of GH on these variables. DESIGN: Two groups were identified: patients with growth retardation who received GH (GH-group) and patients most of whom were not growth retarded who did not receive GH (no-GH-group). After an observation period of 6 months, the patients in the GH-group started GH treatment. Patients were studied every 6 months during 18 months. PATIENTS: Thirty-six prepubertal patients (27 boys and 9 girls), mean age 7.9 years, with CRF participated in the study. The GH-group consisted of 17 patients of whom 14 completed one year treatment. The no-GH-group consisted of 19 patients, of whom 16 were followed for 6 months, 14 for 12 months and 13 for 18 months. MEASUREMENTS: Lumbar spine BMD, total body BMD and body composition were assessed by dual energy X-ray absorptiometry, compared to age-and sex-matched reference values of the same population and expressed as standard deviation scores (SDS). BMD of appendicular bone was measured by quantitative microdensitometry (QMD). Blood samples were obtained to assess bone metabolism and growth factors. RESULTS: Baseline mean lumbar spine and total body BMD SDS of all patients were not significantly different from normal. Mean lumbar spine and total body BMD SDS did not change significantly in the GH-group during GH treatment. The change of QMD at the midshaft during the first 6 months of GH treatment was significantly smaller than during the observation period (P

Published
1998

24. Renal development / Cystic diseases

Author

Yosypiv, I., primary, Song, R., additional, Preston, G., additional, Van Eerde, A. M., additional, Van Binsbergen, E., additional, Konijnenberg, Y., additional, Maiburg, M. C., additional, Lichtenbelt, K., additional, Nikkels, P. G. J., additional, Vd Smagt, J., additional, Renkema, K. Y., additional, Giltay, J. C., additional, De Jong, T. P. V. M., additional, Lilien, M. R., additional, Knoers, N. V. A. M., additional, Gueydan, C., additional, Serena, G., additional, Stephan, G., additional, Koesters, R., additional, Zeineb, B., additional, Laure, D., additional, Catherine, A., additional, Marie-Therese, B., additional, Gauguier, D., additional, Lelongt, B., additional, Moon, S. H., additional, Park, H. C., additional, Lee, H.-Y., additional, Hwang, J. H., additional, Jeong, J. C., additional, Park, J.-Y., additional, Lee, S. W., additional, Hwang, Y.-H., additional, Kang, K. W., additional, Ahn, C., additional, Gattone, V., additional, Carr, A., additional, Crosler-Roberts, R., additional, Wang, X., additional, Liu, Y., additional, Shen, J., additional, Wuthrich, R., additional, Serra, A., additional, Mei, C., additional, Tuta, L., additional, Botea, F., additional, Guigonis, V., additional, Rodier, N., additional, Bahans, C., additional, Decramer, S., additional, Bertholet-Thomas, A., additional, Heidet, L., additional, Eckart, P., additional, Lavocat, M.-P., additional, Vrillon, I., additional, Cloarec, S., additional, Lahoche, A., additional, Bessenay, L., additional, Louillet, F., additional, Roussey, G., additional, Rousset-Riviere, C., additional, Dunand, O., additional, Baudouin, V., additional, Nobili, F., additional, Pietrement, C., additional, De Parscau, L., additional, Gajdos, V., additional, Morin, D., additional, Laffargue, F., additional, Llanas, B., additional, Palcoux, J.-B., additional, Delrue, M.-A., additional, Dizier, E., additional, Taupiac, E., additional, Laroche, C., additional, Lacombe, B., additional, Bourthoumieu, S., additional, El-Meanawy, A., additional, Rufanova, V., additional, and Stelloh, C., additional

Published
2012
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25. Genetic diseases

Author

Inazu, T., primary, Kawahara, T., additional, Endou, H., additional, Anzai, N., additional, Sebesta, I., additional, Stiburkova, B., additional, Ichida, K., additional, Hosoyamada, M., additional, Testa, A., additional, Leonardis, D., additional, Catalano, F., additional, Pisano, A., additional, Mafrica, A., additional, Spoto, B., additional, Sanguedolce, M. C., additional, Parlongo, R. M., additional, Tripepi, G., additional, Postorino, M., additional, Enia, G., additional, Zoccali, C., additional, Mallamaci, F., additional, Working Group*, M., additional, Luque de Pablos, A., additional, Garcia-Nieto, V., additional, Lopez-Menchero, J. C., additional, Ramos-Trujillo, E., additional, Gonzalez-Acosta, H., additional, Claverie-Martin, F., additional, Arsali, M., additional, Demosthenous, P., additional, Papazachariou, L., additional, Athanasiou, Y., additional, Voskarides, K., additional, Deltas, C., additional, Pierides, A., additional, Lee, S., additional, Jeong, K. H., additional, Ihm, C., additional, Lee, T. W., additional, Lee, S. H., additional, Moon, J. Y., additional, Wi, J. G., additional, Lee, H. J., additional, Kim, E. Y., additional, Rogacev, K., additional, Friedrich, A., additional, Hummel, B., additional, Berg, J., additional, Zawada, A., additional, Fliser, D., additional, Geisel, J., additional, Heine, G. H., additional, Brabcova, I., additional, Dusilova-Sulkova, S., additional, Krejcik, Z., additional, Stranecky, V., additional, Lipar, K., additional, Marada, T., additional, Stepankova, J., additional, Viklicky, O., additional, Buraczynska, M., additional, Zukowski, P., additional, Zaluska, W., additional, Kuczmaszewska, A., additional, Ksiazek, A., additional, Gaggl, M., additional, Weidner, S., additional, Hofer, M., additional, Kleinert, J., additional, Fauler, G., additional, Wallner, M., additional, Kotanko, P., additional, Sunder-Plassmann, G., additional, Paschke, E., additional, Heguilen, R., additional, Albarracin, L., additional, Politei, J., additional, Liste, A. A., additional, Bernasconi, A., additional, Kusano, E., additional, Russo, R., additional, Pisani, A., additional, Messalli, G., additional, Imbriaco, M., additional, Prikhodina, L., additional, Ryzhkova, O., additional, Polyakov, V., additional, Lipkowska, K., additional, Ostalska-Nowicka, D., additional, Smiech, M., additional, Jaroniec, M., additional, Zaorska, K., additional, Szaflarski, W., additional, Nowicki, M., additional, Zachwieja, J., additional, D'arrigo, G., additional, Moskowitz, J., additional, Piret, S., additional, Tashman, A., additional, Velez, E., additional, Lhotta, K., additional, Thakker, R., additional, Cox, J., additional, Kingswood, J., additional, Mbundi, J., additional, Attard, G., additional, Patel, U., additional, Saggar, A., additional, Elmslie, F., additional, Doyle, T., additional, Jansen, A., additional, Jozwiak, S., additional, Belousova, E., additional, Frost, M., additional, Kuperman, R., additional, Bebin, M., additional, Korf, B., additional, Flamini, R., additional, Kohrman, M., additional, Sparagana, S., additional, Wu, J., additional, Ford, J., additional, Shah, G., additional, Franz, D., additional, Zonnenberg, B., additional, Cheung, W., additional, Urva, S., additional, Wang, J., additional, Kingswood, C., additional, Budde, K., additional, Kofman, T., additional, Narjoz, C., additional, Raimbourg, Q., additional, Roland, M., additional, Loriot, M.-A., additional, Karras, A., additional, Hill, G. S., additional, Jacquot, C., additional, Nochy, D., additional, Thervet, E., additional, Jagodzinski, P., additional, Mostowska, M., additional, Oko, A., additional, Nicolaou, N., additional, Kevelam, S., additional, Lilien, M., additional, Oosterveld, M., additional, Goldschmeding, R., additional, Van Eerde, A., additional, Pfundt, R., additional, Sonnenberg, A., additional, Ter Hal, P., additional, Knoers, N., additional, Renkema, K., additional, Storm, T., additional, Nielsen, R., additional, Christensen, E., additional, Frykholm, C., additional, Tranebjaerg, L., additional, Birn, H., additional, Verroust, P., additional, Neveus, T., additional, Sundelin, B., additional, Hertz, J. M., additional, Holmstrom, G., additional, Ericson, K., additional, Fabris, A., additional, Cremasco, D., additional, Zambon, A., additional, Muraro, E., additional, Alessi, M., additional, D'angelo, A., additional, Anglani, F., additional, Del Prete, D., additional, Alkmim Teixeira, A., additional, Quinto, B. M., additional, Jose Rodrigues, C., additional, Beltrame Ribeiro, A., additional, Batista, M., additional, Kerti, A., additional, Csohany, R., additional, Szabo, A., additional, Arkossy, O., additional, Sallai, P., additional, Moriniere, V., additional, Vega-Warner, V., additional, Lakatos, O., additional, Szabo, T., additional, Reusz, G., additional, Tory, K., additional, Addis, M., additional, Tosetto, E., additional, Meloni, C., additional, Ceol, M., additional, Cristofaro, R., additional, Melis, M. A., additional, Vercelloni, P., additional, Marra, G., additional, Kaniuka, S., additional, Nagel, M., additional, Wolyniec, W., additional, Obolonczyk, L., additional, Swiatkowska-Stodulska, R., additional, Sworczak, K., additional, Rutkowski, B., additional, Chen, C., additional, Jiang, L., additional, Chen, L., additional, Fang, L., additional, Mozes M., M., additional, Boosi, M., additional, Rosivall, L., additional, Kokeny, G., additional, Diana, R., additional, Gross, O., additional, Johanna, T., additional, Rainer, G., additional, Ayse, C., additional, Henrik, H., additional, Gerhard-Anton, M., additional, Nabil, M., additional, Intissar, E., additional, Belge, H., additional, Bloch, J., additional, Dahan, K., additional, Pirson, Y., additional, Vanhille, P., additional, and Demoulin, N., additional

Published
2012
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26. Paediatric nephrology

Author

Shi, H., primary, Wen, J., additional, LI, Z., additional, Elsayed, M., additional, Kamal, K., additional, Shi, H., additional, El Shal, A., additional, Youssef, D., additional, Caubet, C., additional, Lacroix, C., additional, Benjamin, B., additional, Bandin, F., additional, Bascands, J.-L., additional, Monsarrat, B., additional, Decramer, S., additional, Schanstra, J., additional, Laetitia, D.-B., additional, Ulinski, T., additional, Aoun, B., additional, Ozdemir, K., additional, Dincel, N., additional, Sozeri, B., additional, Mir, S., additional, Berdeli, A., additional, Akyigit, F., additional, Mizerska-Wasiak, M., additional, Panczyk-Tomaszewska, M., additional, Szymanik-Grzelak, H., additional, Roszkowska-Blaim, M., additional, Jamin, A., additional, Dehoux, L., additional, Monteiro, R. C., additional, Deschenes, G., additional, Bouts, A., additional, Davin, J.-C., additional, Dorresteijn, E., additional, Schreuder, M., additional, Lilien, M., additional, Oosterveld, M., additional, Kramer, S., additional, Gruppen, M., additional, Pintos-Morell, G., additional, Ramaswami, U., additional, Parini, R., additional, Rohrbach, M., additional, Kalkum, G., additional, Beck, M., additional, Carter, M., additional, Antwi, S., additional, Callegari, J., additional, Kotanko, P., additional, Levin, N. W., additional, Rumjon, A., additional, Macdougall, I. C., additional, Turner, C., additional, Booth, C. J., additional, Goldsmith, D., additional, Sinha, M. D., additional, Camilla, R., additional, Loiacono, E., additional, Donadio, M. E., additional, Conrieri, M., additional, Bianciotto, M., additional, Bosetti, F. M., additional, Peruzzi, L., additional, Conti, G., additional, Bitto, A., additional, Amore, A., additional, Coppo, R., additional, Maldyk, J., additional, Chou, H.-H., additional, Chiou, Y.-Y., additional, Bochniewska, V., additional, Jobs, K., additional, Jung, A., additional, Fallahzadeh Abarghooei, M. H., additional, Zare, J., additional, Sedighi Goorabi, V., additional, Derakhshan, A., additional, Basiratnia, M., additional, Fallahzadeh Abarghooei, M. A., additional, Hosseini Al-Hashemi, G., additional, Fallahzadeh Abarghooei, F., additional, Kluska-Jozwiak, A., additional, Soltysiak, J., additional, Lipkowska, K., additional, Silska, M., additional, Fichna, P., additional, Skowronska, B., additional, Stankiewicz, W., additional, Ostalska-Nowicka, D., additional, Zachwieja, J., additional, Girisgen, L., additional, Sonmez, F., additional, Yenisey, C., additional, Kis, E., additional, Cseprekal, O., additional, Kerti, A., additional, Szabo, A., additional, Salvi, P., additional, Benetos, A., additional, Tulassay, T., additional, Reusz, G., additional, Makulska, I., additional, Szczepanska, M., additional, Drozdz, D., additional, Zwolnska, D., additional, Tolstova, E., additional, Anis, L., additional, Alber, B., additional, Edouard, B., additional, Gerard, C., additional, Seni, K., additional, Dunia Julienne Hadiza, T., additional, Christian, S., additional, Benoit, T., additional, Francois, B., additional, Adama, L., additional, Rosenberg, A., additional, Munro, J., additional, Murray, K., additional, Wainstein, B., additional, Ziegler, J., additional, Singh-Grewal, D., additional, Boros, C., additional, Adib, N., additional, Elliot, E., additional, Fahy, R., additional, Mackie, F., additional, Kainer, G., additional, Polak-Jonkisz, D., additional, Zwolinska, D., additional, Laszki-Szczachor, K., additional, Janocha, A., additional, Rusiecki, L., additional, Sobieszczanska, M., additional, Garzotto, F., additional, Ricci, Z., additional, Clementi, A., additional, Cena, R., additional, Kim, J. C., additional, Zanella, M., additional, Ronco, C., additional, Purzyc, L., additional, Peco-Antic, A., additional, Kotur-Stevuljevic, J., additional, Paripovic, D., additional, Scekic, G., additional, Milosevski-Lomic, G., additional, Bogicevic, D., additional, Spasojevic-Dimitrijeva, B., additional, Hassan, R., additional, El-Husseini, A., additional, Sobh, M., additional, Ghoneim, M., additional, Harambat, J., additional, Bonthuis, M., additional, Van Stralen, K. J., additional, Ariceta, G., additional, Battelino, N., additional, Jahnukainen, T., additional, Sandes, A. R., additional, Combe, C., additional, Jager, K. J., additional, Verrina, E., additional, Schaefer, F., additional, Espindola, R., additional, Bacchetta, J., additional, Cochat, P., additional, Stefanis, C., additional, Leroy, S., additional, Fernandez-Lopez, A., additional, Nikfar, R., additional, Romanello, C., additional, Bouissou, F., additional, Gervaix, A., additional, Gurgoze, M., additional, Bressan, S., additional, Smolkin, V., additional, Tuerlinkx, D., additional, Stefanidis, C., additional, Vaos, G., additional, Leblond, P., additional, Gungor, F., additional, Gendrel, D., additional, Chalumeau, M., additional, Rawlins, D., additional, Simpson, J. M., additional, Arnaud, G., additional, Anne, M., additional, Stephanie, T., additional, Flavio, B., additional, Veronique, F. B., additional, Stephane, D., additional, Mumford, L., additional, Marks, S., additional, Ahmad, N., additional, Maxwell, H., additional, Tizard, J., additional, Vidal, E., additional, Amigoni, A., additional, Varagnolo, M., additional, Benetti, E., additional, Ghirardo, G., additional, Brugnolaro, V., additional, Murer, L., additional, Christine, G., additional, Degi, A., additional, Szabo, A. J., additional, Reusz, G. S., additional, Vidoni, A., additional, Ramondo, G., additional, and Miotto, D., additional

Published
2012
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27. Fewer pre-emptive renal transplantations and more rejections in immigrant children compared to native Dutch and Belgian children

Author

Tromp, W. F., primary, Cransberg, K., additional, van der Lee, J. H., additional, Bouts, A. H., additional, Collard, L., additional, Van Damme-Lombaerts, R., additional, Godefroid, N., additional, Van Hoeck, K. J., additional, Koster-Kamphuis, L., additional, Lilien, M. R., additional, Raes, A., additional, Ranguelov, N., additional, and Groothoff, J. W., additional

Published
2012
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28. Important differences in management policies for children with end-stage renal disease in the Netherlands and Belgium--report from the RICH-Q study

Author

Tromp, W. F., primary, Schoenmaker, N. J., additional, van der Lee, J. H., additional, Adams, B., additional, Bouts, A. H. M., additional, Collard, L., additional, Cransberg, K., additional, Van Damme-Lombaerts, R., additional, Godefroid, N., additional, van Hoeck, K., additional, Koster-Kamphuis, L., additional, Lilien, M. R., additional, Raes, A., additional, Offringa, M., additional, and Groothoff, J. W., additional

Published
2011
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29. Lessons learned from efforts to improve the quality of care in children with end-stage renal disease in the Netherlands and Belgium

Author

Tromp, W. F., primary, van der Lee, J. H., additional, Offringa, M., additional, Bouts, A. H. M., additional, Collard, L., additional, Cransberg, K., additional, Van Damme-Lombaerts, R., additional, Godefroid, N., additional, Van Hoeck, K., additional, Koster-Kamphuis, L., additional, Lilien, M. R., additional, Raes, A., additional, and Groothoff, J. W., additional

Published
2011
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36. Calcinosis in juvenile dermatomyositis: a possible role for the vitamin K-dependent protein matrix Gla protein.

Author

Summeren, M. J. H. van, Spliet, W. G. M., Royen-Kerkhof, A. van, Vermeer, C., Lilien, M., Kuis, W., and Schurgers, L. J.

Subjects
DERMATOMYOSITIS, CALCIFICATION, PROTEINS, BIOPSY, CUTANEOUS manifestations of general diseases, MYOSITIS
Abstract

Objectives. The aims of the present study were to investigate whether the calcification inhibitor matrix Gla protein (MGP) is expressed in muscle biopsies of patients with juvenile dermatomyositis (JDM), and whether different forms of MGP are differentially expressed in JDM patients with and without subcutaneous calcifications. [ABSTRACT FROM PUBLISHER]

Published
2008
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37. Observation of Fluorescence Excitation Spectra of tert-Pentoxy and 3-Pentoxy Radicals

Author

Wang, C., Deng, W., Shemesh, L. G., Lilien, M. D., Katz, D. R., and Dibble, T. S.

Abstract

Fluorescence excitation spectra of tert-pentoxy [CH3CH2C(O·)(CH3)2] and 3-pentoxy radicals were observed for the first time. The radicals were produced by laser photolysis of the corresponding pentyl nitrites at 355 nm and studied in the wavelength range 345−400 nm. For tert-pentoxy, 12 vibronic bands were labeled in three progressions. One dominant progression corresponds to the C−O stretch mode with initial vibrational interval 551 ± 10 cm-1. Two other unknown mode progressions have vibrational intervals of 587 ± 10 and 631 ± 10 cm-1. The transition origin was tentatively assigned at 25 491 ± 10 cm-1 (392.3 ± 0.1 nm). For 3-pentoxy, 15 vibronic bands were labeled in three progressions. The vibrational intervals in each progression are of consistent anharmonic tendency. On the basis of our assignments of the C−O stretch progression, the transition origin T0, the C−O stretching vibration frequency νC-O, and the anharmonic constant χC-O are derived to be 26 437 ± 10, 578 ± 6, and −17 ± 2 cm-1, respectively. The initial vibrational intervals of two other unknown modes are 596 ± 10 and 590 ± 10 cm-1. The fluorescence lifetime of 3-pentoxy was determined to be about 150 ns. The short extent of the laser-induced fluorescence (LIF) excitation spectra of many C2−C5 alkoxy radicals is tentatively explained as arising from the opening of a predissociation channel at relatively low excitation energies. Results of efforts to obtain LIF excitation spectra for 10 additional large alkoxy radicals are reported.

Published
2000

38. Laser-Induced Fluorescence Excitation Spectra of tert-Butoxy and 2-Butoxy Radicals

Author

Wang, C., Shemesh, L. G., Deng, W., Lilien, M. D., and Dibble, T. S.

Abstract

Laser-induced fluorescence (LIF) excitation spectra of t-C4H9O (tert-butoxy) and 2-C4H9O (2-butoxy) radicals were investigated in the wavelength range 335−400 nm. The radicals were formed by laser photolysis of the corresponding butyl nitrites at 355 nm. For tert-butoxy, 16 vibronic bands in two progressions were labeled. The dominant progression corresponds to C−O stretching mode with ν‘C-O = 521 ± 10 cm-1. The transition origin was tentatively assigned at 25 866 cm-1 (386.6 nm). Numerous bands remain unassigned. The LIF excitation spectrum of 2-butoxy, consisting of 15 vibronic bands in four progressions, was observed for the first time. A C−O stretching frequency ν‘C-O = 567 ± 10 cm-1 was obtained from the dominant progression. The transition origin was tentatively assigned at 26 185 cm-1 (381.9 nm). Three other progressions are evident, which have different vibrational band intervals:  617 ± 10, 590 ± 10, and 552 ± 10 cm-1. Zero-pressure fluorescence lifetimes for numerous vibronic bands of tert-butoxy and 2-butoxy were determined to be about 150 and 85 ns, respectively. These spectra can be used as a convenient spectroscopic tool for kinetic studies of butoxy radicals and should provide a starting point for investigations of their excited states structure and dynamics.

Published
1999

39. Occult blood trial in feces of patients of the 'Ernesto Guevara' hospital

Author

Jenny de la Caridad Hernández Betancourt, María Elena Camejo Nieves, Mailín Rodríguez Alonso, Lilién Morales Rodríguez, and María Rodríguez Rosales

Subjects
colonic neoplasms, hemorrhage, occult blood, Medicine, Medicine (General), R5-920
Abstract

Background: colorectal cancer screening by occult blood test is an opportunity for the early diagnosis and prompt treatment of one of the primary causes of death because of malignant tumors in Cuba and in the world.Objective: to determine the results of the application of occult blood test screening in feces samples processed in the ultra-micro-analytic laboratory of Dr. Ernesto Guevara de la Serna Teaching General Hospital in Las Tunas province during 2015.Methods: the information about the patients processed in the trial: age, origin, gender, cause of indication, symptoms and personal history were taken from the tests registration in the ultra-micro-analytic laboratory.Results: a month average of 39 was found in 271 samples studied and positivity in 18,8 %. Abdominal pain, bleeding or changes in bowel habits resulted in a positivity that always exceeded 20 %. Only the 5,2 % of the tests were consistent with the investigation and none of those cases was positive. 69,9 % of the individuals studied came from the outpatient consultation and there were only 8 hospitalized patients. Several deficiencies were detected in the trial requests, related to the completion of the clinical data.Conclusions: there has not been made good use of occult blood test in feces in Las Tunas province.

Published
2016

42. Focal segmental glomerulosclerosis in a patient homozygous for a CD2AP mutation.

Author

Löwik, M. M., Groenen, P. J. T. A., Pronk, I., Lilien, M. R., Goldschmeding, R., Dijkman, H. B., Levtchenko, E. N., Monnens, L. A., and van den Heuvel, L. P.

Subjects
*KIDNEY diseases, *PATIENTS, *GENETIC mutation, *ACTIN, *DIAGNOSIS, *GENES, *CYTOSKELETON
Abstract

Focal segmental glomerulosclerosis (FSGS) is a histologic diagnosis in several kidney diseases characterized by proteinuria and a severe decrease in kidney function. Mutations in several genes were found in patients with primary FSGS, one of which is a CD2-associated protein CD2AP (originally referred to as CMS). This gene encodes an adaptor protein that plays a role in endocytosis, cell motility, and cell survival. Mice deficient in Cd2ap (the mouse homolog) die due to kidney failure, while heterozygous mice develop lesions similar to those of FSGS patients. In the kidney, CD2AP regulates the actin cytoskeleton. The only previously described patient with CD2AP mutation had a severely truncated protein. In this study, we describe a patient with a novel mutation resulting in a premature stop codon yielding a protein truncated by only 4%. This shortened CD2AP protein displays a significantly decreased F-actin binding efficiency in vitro with no expression of the mutated allele in the patient's lymphocytes. Heterozygous expression of the CD2AP mutation in both parents did not lead to any kidney pathology, as both have normal glomerular filtration rates and no proteinuria.Kidney International (2007) 72, 1198–1203; doi:10.1038/sj.ki.5002469; published online 22 August 2007 [ABSTRACT FROM AUTHOR]

Published
2007
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43. Clinical practice recommendations for kidney involvement in tuberous sclerosis complex: a consensus statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes & Kidney Working Group.

Author

Mekahli D, Müller RU, Marlais M, Wlodkowski T, Haeberle S, de Argumedo ML, Bergmann C, Breysem L, Fladrowski C, Henske EP, Janssens P, Jouret F, Kingswood JC, Lattouf JB, Lilien M, Maleux G, Rozenberg M, Siemer S, Devuyst O, Schaefer F, Kwiatkowski DJ, Rouvière O, and Bissler J

Subjects
Humans, Consensus, Angiomyolipoma genetics, Angiomyolipoma etiology, Practice Guidelines as Topic, Tuberous Sclerosis genetics, Tuberous Sclerosis therapy, Tuberous Sclerosis complications
Abstract

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the presence of proliferative lesions throughout the body. Management of TSC is challenging because patients have a multifaceted systemic illness with prominent neurological and developmental impact as well as potentially severe kidney, heart and lung phenotypes; however, every organ system can be involved. Adequate care for patients with TSC requires a coordinated effort involving a multidisciplinary team of clinicians and support staff. This clinical practice recommendation was developed by nephrologists, urologists, paediatric radiologists, interventional radiologists, geneticists, pathologists, and patient and family group representatives, with a focus on TSC-associated kidney manifestations. Careful monitoring of kidney function and assessment of kidney structural lesions by imaging enable early interventions that can preserve kidney function through targeted approaches. Here, we summarize the current evidence and present recommendations for the multidisciplinary management of kidney involvement in TSC., (© 2024. Springer Nature Limited.)

Published
2024
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44. Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.

Author

Kohl S, Avni FE, Boor P, Capone V, Clapp WL, De Palma D, Harris T, Heidet L, Hilger AC, Liapis H, Lilien M, Manzoni G, Montini G, Negrisolo S, Pierrat MJ, Raes A, Reutter H, Schreuder MF, Weber S, Winyard PJD, Woolf AS, Schaefer F, and Liebau MC

Subjects
Child, Humans, Kidney pathology, Kidney Diseases pathology, Urogenital Abnormalities, Renal Insufficiency pathology
Abstract

Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term 'kidney dysplasia' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field., (© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)

Published
2022
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45. Refining Kidney Survival in 383 Genetically Characterized Patients With Nephronophthisis.

Author

König JC, Karsay R, Gerß J, Schlingmann KP, Dahmer-Heath M, Telgmann AK, Kollmann S, Ariceta G, Gillion V, Bockenhauer D, Bertholet-Thomas A, Mastrangelo A, Boyer O, Lilien M, Decramer S, Schanstra JP, Pohl M, Schild R, Weber S, Hoefele J, Drube J, Cetiner M, Hansen M, Thumfart J, Tönshoff B, Habbig S, Liebau MC, Bald M, Bergmann C, Pennekamp P, and Konrad M

Abstract

Introduction: Nephronophthisis (NPH) comprises a group of rare disorders accounting for up to 10% of end-stage kidney disease (ESKD) in children. Prediction of kidney prognosis poses a major challenge. We assessed differences in kidney survival, impact of variant type, and the association of clinical characteristics with declining kidney function., Methods: Data was obtained from 3 independent sources, namely the network for early onset cystic kidney diseases clinical registry ( n = 105), an online survey sent out to the European Reference Network for Rare Kidney Diseases ( n = 60), and a literature search ( n = 218)., Results: A total of 383 individuals were available for analysis: 116 NPHP1 , 101 NPHP3 , 81 NPHP4 and 85 NPHP11/TMEM67 patients. Kidney survival differed between the 4 cohorts with a highly variable median age at onset of ESKD as follows: NPHP3, 4.0 years (interquartile range 0.3-12.0); NPHP1 , 13.5 years (interquartile range 10.5-16.5); NPHP4, 16.0 years (interquartile range 11.0-25.0); and NPHP11/TMEM67, 19.0 years (interquartile range 8.7-28.0). Kidney survival was significantly associated with the underlying variant type for NPHP1 , NPHP3 , and NPHP4 . Multivariate analysis for the NPHP1 cohort revealed growth retardation (hazard ratio 3.5) and angiotensin-converting enzyme inhibitor (ACEI) treatment (hazard ratio 2.8) as 2 independent factors associated with an earlier onset of ESKD, whereas arterial hypertension was linked to an accelerated glomerular filtration rate (GFR) decline., Conclusion: The presented data will enable clinicians to better estimate kidney prognosis of distinct patients with NPH and thereby allow personalized counseling., (© 2022 International Society of Nephrology. Published by Elsevier Inc.)

Published
2022
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46. Ten-year trends in epidemiology and outcomes of pediatric kidney replacement therapy in Europe: data from the ESPN/ERA-EDTA Registry.

Author

Bonthuis M, Vidal E, Bjerre A, Aydoğ Ö, Baiko S, Garneata L, Guzzo I, Heaf JG, Jahnukainen T, Lilien M, Mallett T, Mirescu G, Mochanova EA, Nüsken E, Rascher K, Roussinov D, Szczepanska M, Tsimaratos M, Varvara A, Verrina E, Veselinović B, Jager KJ, and Harambat J

Subjects
Child, Edetic Acid, Europe epidemiology, Humans, Kidney Failure, Chronic epidemiology, Kidney Failure, Chronic therapy, Registries, Renal Replacement Therapy
Abstract

Background: For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We examined trends in incidence and prevalence of KRT and patient survival., Methods: We included all children aged <15 years starting KRT 2007-2016 in 22 European countries participating in the ESPN/ERA-EDTA Registry since 2007. General population statistics were derived from Eurostat. Incidence and prevalence were expressed per million age-related population (pmarp) and time trends studied with JoinPoint regression. We analyzed survival trends using Cox regression., Results: Incidence of children commencing KRT <15 years remained stable over the study period, varying between 5.5 and 6.6 pmarp. Incidence by treatment modality was unchanged over time: 2.0 for hemodialysis (HD) and peritoneal dialysis (PD) and 1.0 for transplantation. Prevalence increased in all age categories and overall rose 2% annually from 26.4 pmarp in 2007 to 32.1 pmarp in 2016. Kidney transplantation prevalence increased 5.1% annually 2007-2009, followed by 1.5% increase/year until 2016. Prevalence of PD steadily increased 1.4% per year over the entire period, and HD prevalence started increasing 6.1% per year from 2011 onwards. Five-year unadjusted patient survival on KRT was around 94% and similar for those initiating KRT 2007-2009 or 2010-2012 (adjusted HR: 0.98, 95% CI:0.71-1.35)., Conclusions: We found a stable incidence and increasing prevalence of European children on KRT 2007-2016. Five-year patient survival was good and was unchanged over time. These data can inform patients and healthcare providers and aid health policy makers on future resource planning of pediatric KRT in Europe.

Published
2021
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47. Beyond nephronophthisis: Retinal dystrophy in the absence of kidney dysfunction in childhood expands the clinical spectrum of CEP83 deficiency.

Author

Veldman BCF, Kuper WFE, Lilien M, Schuurs-Hoeijmakers JHM, Marcelis C, Phan M, Hettinga Y, Talsma HE, van Hasselt PM, and Haijes HA

Subjects
Child, Child, Preschool, Cilia, Ciliopathies diagnostic imaging, Ciliopathies pathology, Female, Genetic Predisposition to Disease, Humans, Kidney diagnostic imaging, Kidney Diseases diagnostic imaging, Kidney Diseases genetics, Kidney Diseases pathology, Male, Microtubule-Associated Proteins deficiency, Retina diagnostic imaging, Retinitis Pigmentosa diagnostic imaging, Retinitis Pigmentosa pathology, Ciliopathies genetics, Microtubule-Associated Proteins genetics, Retina pathology, Retinitis Pigmentosa genetics
Abstract

The CEP83 protein is an essential part in the first steps of ciliogenesis, causing a ciliopathy if deficient. As a core component of the distal appendages of the centriole, CEP83 is located in almost all cell types and is involved in the primary cilium assembly. Previously reported CEP83 deficient patients all presented with nephronophthisis and kidney dysfunction. Despite retinal degeneration being a common feature in ciliopathies, only one patient also had retinitis. Here, we present two unrelated patients, who both presented with retinitis pigmentosa, without nephronophthisis or any form of kidney dysfunction. Both patients harbor bi-allelic variants in CEP83. This report expands the current clinical spectrum of CEP83 deficiency. For timely diagnosis of CEP83 deficiency, we advocate that CEP83 should be included in gene panels for inherited retinal diseases., (© 2021 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)

Published
2021
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48. Effect of therapeutic hypothermia on renal and myocardial function in asphyxiated (near) term neonates: A systematic review and meta-analysis.

Author

van Wincoop M, de Bijl-Marcus K, Lilien M, van den Hoogen A, and Groenendaal F

Subjects
Animals, Humans, Infant, Newborn, Asphyxia physiopathology, Asphyxia Neonatorum physiopathology, Cardiomyopathies physiopathology, Hypothermia, Induced adverse effects, Kidney physiopathology, Myocardium pathology
Abstract

Background: Therapeutic hypothermia (TH) is a well-established neuroprotective therapy applied in (near) term asphyxiated infants. However, little is known regarding the effects of TH on renal and/or myocardial function., Objectives: To describe the short- and long-term effects of TH on renal and myocardial function in asphyxiated (near) term neonates., Methods: An electronic search strategy incorporating MeSH terms and keywords was performed in October 2019 and updated in June 2020 using PubMed and Cochrane databases. Inclusion criteria consisted of a RCT or observational cohort design, intervention with TH in a setting of perinatal asphyxia and available long-term results on renal and myocardial function. We performed a meta-analysis and heterogeneity and sensitivity analyses using a random effects model. Subgroup analysis was performed on the method of cooling., Results: Of the 107 studies identified on renal function, 9 were included. None of the studies investigated the effects of TH on long-term renal function after perinatal asphyxia. The nine included studies described the effect of TH on the incidence of acute kidney injury (AKI) after perinatal asphyxia. Meta-analysis showed a significant difference between the incidence of AKI in neonates treated with TH compared to the control group (RR = 0.81; 95% CI 0.67-0.98; p = 0.03). No studies were found investigating the long-term effects of TH on myocardial function after neonatal asphyxia. Possible short-term beneficial effects were presented in 4 out of 5 identified studies, as observed by significant reductions in cardiac biomarkers and less findings of myocardial dysfunction on ECG and cardiac ultrasound., Conclusions: TH in asphyxiated neonates reduces the incidence of AKI, an important risk factor for chronic kidney damage, and thus is potentially renoprotective. No studies were found on the long-term effects of TH on myocardial function. Short-term outcome studies suggest a cardioprotective effect., Competing Interests: The authors have declared that no competing interests exist.

Published
2021
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49. Early Acute Kidney Injury in Preterm and Term Neonates: Incidence, Outcome, and Associated Clinical Features.

Author

Gallo D, de Bijl-Marcus KA, Alderliesten T, Lilien M, and Groenendaal F

Subjects
Creatinine, Female, Humans, Incidence, Infant, Newborn, Infant, Premature, Pregnancy, Retrospective Studies, Acute Kidney Injury diagnosis, Acute Kidney Injury epidemiology, Acute Kidney Injury etiology, Asphyxia Neonatorum
Abstract

Background: Critically ill neonates are at high risk of kidney injury, mainly in the first days of life. Acute kidney injury (AKI) may be underdiagnosed due to lack of a uniform definition. In addition, long-term renal follow-up is limited., Objective: To describe incidence, etiology, and outcome of neonates developing AKI within the first week after birth in a cohort of NICU-admitted neonates between 2008 and 2018. Renal function at discharge in infants with early AKI was assessed., Methods and Subjects: AKI was defined as an absolute serum Cr (sCr) value above 1.5 mg/dL (132 μmol/L) after the first 24 h or as stage 2-3 of the NIDDK neonatal definition. Clinical data and outcomes were collected from medical records and retrospectively analyzed., Results: From January 2008 to December 2018, a total of 9,376 infants were admitted to the NICU of Wilhelmina Children's Hospital/UMC Utrecht, of whom 139 were diagnosed with AKI during the first week after birth. In 72 term infants, the most common etiology was perinatal asphyxia (72.2%), followed by congenital kidney and urinary tract malformations (CAKUT) (8.3%), congenital heart disease (6.9%), and sepsis (2.8%). Associated conditions in 67 preterm infants were medical treatment of a hemodynamic significant PDA (27.2%), -CAKUT (21%), and birth asphyxia (19.4%). Among preterm neonates and neonates with perinatal asphyxia, AKI was mainly diagnosed by the sCr >1.5 mg/dL criterion. Renal function at discharge improved in 76 neonates with AKI associated with acquired conditions. Neonates with stage 3 AKI showed increased sCr values at discharge. Half of these were caused by congenital kidney malformations and evolved into chronic kidney disease (CKD) later in life. Neurodevelopmental outcome (NDO) at 2 years was favorable in 93% of surviving neonates with detailed follow-up., Conclusion: During the first week after birth, AKI was seen in 1.5% of infants admitted to a level III NICU. Renal function at discharge had improved in most neonates with acquired AKI but not in infants diagnosed with stage 3 AKI. Long-term renal function needs further exploration, whereas NDO appears to be good., (© 2021 The Author(s) Published by S. Karger AG, Basel.)

Published
2021
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50. Citrate versus heparin anticoagulation in continuous renal replacement therapy in small children.

Author

Raymakers-Janssen PAMA, Lilien M, van Kessel IA, Veldhoen ES, Wösten-van Asperen RM, and van Gestel JPJ

Subjects
Anticoagulants pharmacology, Blood Coagulation drug effects, Child, Preschool, Citric Acid pharmacology, Female, Heparin pharmacology, Humans, Infant, Intensive Care Units statistics & numerical data, Male, Prospective Studies, Renal Replacement Therapy methods, Renal Replacement Therapy statistics & numerical data, Time Factors, Treatment Outcome, Water-Electrolyte Balance, Acute Kidney Injury therapy, Anticoagulants therapeutic use, Citric Acid therapeutic use, Critical Illness therapy, Heparin therapeutic use, Renal Replacement Therapy adverse effects
Abstract

Background: Citrate is preferred over heparin as an anticoagulant in adult continuous renal replacement therapy (CRRT). However, its potential adverse effects and data on use in CRRT in infants and toddlers is limited. We conducted a prospective study on using citrate in CRRT in critically ill small children., Methods: Children who underwent CRRT with the smallest filter in our PICU between November 2011 and November 2016 were included. Both heparin and citrate were applied according to a strict protocol. Our primary outcome was circuit survival time. Secondary outcomes were alkalosis, citrate toxicity, and number of red blood cell transfusions., Results: Heparin was used in six patients (121 circuits, total CRRT time 3723 h). Citrate was used in 14 patients (105 circuits, total CRRT time 4530 h). Median circuit survival time with heparin was 21 h (IQR 14.5-27.5) compared to 45.2 h (IQR 37.5-52.8) with citrate (p < 0.001). Actual administered effluent dose compared to prescribed dose was 85% (IQR 69-98%) with heparin compared to 92% (IQR 88-98%) with citrate (p = 0.31). No patient treated with citrate developed citrate toxicity. No other differences in electrolytes were found between the two CRRT regimes. In the heparin group, a median of 6.5 units of red blood cells (IQR 1.5-23.8) were given during CRRT, compared to three in the citrate group (IQR 2.0-5.0, p = 0.12)., Conclusions: Use of regional citrate significantly prolongs circuit survival time and thereby should increase CRRT efficiency when compared to heparin. In addition, citrate appears safe for CRRT in critically ill small children.

Published
2017
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