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1. Vascular access for chronic hemodialysis in children: arteriovenous fistula or central venous catheter?

Author

Merouani, Aicha, Lallier, Michel, Paquet, Julie, Gagnon, Johanne, and Lapeyraque, Anne Laure

Subjects
Kidneys -- Transplantation, Fistula, Arteriovenous -- Research, Chronic kidney failure -- Care and treatment -- Demographic aspects -- Research, Hemodialysis -- Methods -- Demographic aspects -- Research, Central venous catheters -- Research, Health
Abstract

Background The choice of vascular access (VA) for hemodialysis (HD) in end-stage renal disease (ESRD) is arteriovenous fistula (AVF) or central venous catheter (CVC). Whereas clinical practice guidelines suggest AVF to preserve the vascular bed, pediatric nephrologists tend to favor CVC for shorter-term dialysis. Our objective was to determine whether pediatric priority allocation policies for deceased-donor kidneys affect VA planning. Methods Pediatric priority for deceased-donor kidneys was instituted in Quebec in 2004. We retrospectively compared clinical practice on AVF, CVC, wait time on transplant list, HD duration in pre-policy (group A) and post-policy (group B) from 1997-2011. Results We identified 78 patients with a median age of 14.7 years (range, 0.7-20.5 years) and weight of 46 kg (12.5-95 kg); AVF decreased from 76% in group A to 41%ingroupB (p = 0.002). Waittimes on transplant list were significantly reduced: median 413.5 days (range, 21,910 days) in group A vs. 89 days (range, 18-692 days) in group B (p = 0.003). Time on HD for deceased-donor recipients was shorter: 705 (range, 51-1,965 days) group A vs. 349.5 days (range, 158-1,060 days) group B (p = 0.01). Conclusions This is the first study to document VA changes related to pediatric priority allocation policy. Our fistula-first center saw a shift toward CVC-first. Keywords Arteriovenous fistula * End-stage renal disease * Kidney transplantation * Pediatric chronic hemodialysis * Pediatric priority for deceased-donor kidneys * Vascular access, Introduction Transplantation is the renal replacement therapy of choice for children with end-stage renal disease (ESRD) [1]. The most commonly used modality while awaiting a kidney transplant is hemodialysis, rather [...]

Published
2014
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2. Molecular Absorbent Recirculating System therapy (MARS®) in pediatric acute liver failure: a single center experience

Author

Bourgoin, Pierre, Merouani, Aicha, Phan, Veronique, Litalien, Catherine, Lallier, Michel, Alvarez, Fernando, and Jouvet, Philippe

Subjects
Liver failure -- Diagnosis -- Care and treatment -- Research, Absorption (Physiology) -- Physiological aspects -- Research, Health
Abstract

Background Supportive care as a bridge to transplant or recovery remains challenging in children suffering from acute liver failure (ALF). We report our experience in children using the Molecular Absorbent Recirculating System (MARS®). Methods Retrospective data from children receiving therapy using MARS® from October 2009 to October 2012 were included in this single-center retrospective study. Patient characteristics, clinical presentation and complications of ALF, clinical and biological data before and after each MARS® session, technical modalities and adverse events were recorded. Results A total of six children underwent 17 MARS® sessions during the study period. Two adolescents were treated with the adult filter MARSFLUX® and four infants were treated with the MiniMARS® filter. The mean PEdiatric Logistic Dysfunction (PELOD) score at admission was 19 (range 11-33). All patients were mechanically ventilated, and four had acute kidney injury. The neurological course improved in one case, judged as stable in two cases and worsened in one case; data were unavailable in two cases. Mean serum ammonia levels decreased significantly following treatment with MARS® from an initial 89 ± 29 to 58 ± 35 mcmol/L (p = 0.02). No other significant biological improvement was observed. Hemodynamic status improved/remained unchanged in the adolescent group, but in the infants four of the seven sessions were poorly tolerated and two sessions were aborted. Three patients died, two were successfully transplanted and one recovered without transplantation. Conclusion In our experience, treatment with MARS® is associated with encouraging results in adolescents, but it needs modification for very sick infants to improve tolerance. Keywords: Children * Acute liver failure * Molecular Absorbant Recycling System (MARS®) * Clinical experience, Introduction Acute liver failure (ALF) in children is defined as a multisystem disorder that includes severe impairment of liver function [1]. In contrast with the adult definition, in which encephalopathy [...]

Published
2014
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18. Contributors

Author

Albright, Robert C., Jr, Amerling, Richard, Angeli, Paolo, Angelotti, Maria Lucia, Antonelli, Massimo, Antoniotti, Riccardo, Arulkumaran, Nishkantha, Asfar, Pierre, Ash, Stephen R., Aucella, Filippo, Aucella, Francesco, Ave, Samuele, Bagshaw, Sean M., Balaraman, Vasanthi, Baldwin, Ian, Bargman, Joanne M., Barletta, Gina-Marie, Barletta, Jeffrey F., Barnela, Shriganesh R., Bayır, Hülya, Beaulieu, Monica, Bellasi, Antonio, Bellomo, Rinaldo, Beloncle, François, Bhansali, Arjun, Bihorac, Azra, Billings, Frederic T., IV, Birk, Horst-Walter, Bonilla-Reséndiz, Luis Ignacio, Bouchard, Josée, Bourke, Edmund, Braitberg, George, Brendolan, Alessandra, Brocca, Alessandra, Brophy, Patrick D., Bucala, Richard, Bunchman, Timothy E., Burdmann, Emmanuel A., Busse, Laurence W., Caires, Renato Antunes, Caironi, Pietro, Camilla, Roberta, Campos, Israel, Canaud, Bernard, Cantaluppi, Vincenzo, Martinez, Maria P., Capasso, Giovambattista, Carcillo, Joseph A., Carlesso, Eleonora, Dip., Casino, Francesco G., Castellano, Giuseppe, Catania, Matteo, Cawcutt, Kelly A., Cerda, Jorge, Charen, Elliot, Chawla, Lakhmir S., Chiaramonte, Stefano, Chua, Horng-Ruey, Cianciaruso, Bruno, Ciceri, Paola, Cieslak, Jacek, Clark, William R., Claure-Del Granado, Rolando, Clementi, Anna, Co, Ivan N., Coelho, Fernanda Oliveira, Conte, Ferruccio, Corey, Howard E., Cosmai, Laura, Costalonga, Elerson Carlos, Costamagna, Andrea, Costanzo, Maria Rosa, Cozzolino, Mario, Cramer, Carl H., II, Cravedi, Paolo, Crepaldi, Carlo, Creteur, Jacques, Crew, R. John, da Costa e Silva, Verônica Torres, Davenport, Andrew, Davies, Andrew R., D'Costa, Rohit, Dean, Dawson F., Debiais, Charlotte, de Cal, Massimo, Dedhia, Paras, de Grooth, Harm-Jan, Dell'Aquila, Roberto, Dellepiane, Sergio, Dellinger, Richard Phillip, Del Vecchio, Lucia, Depner, Thomas A., De Rosa, Silvia, Deutschman, Clifford S., Devarajan, Prasad, Dewitte, A., Di Iorio, Biagio R., Di Lullo, Luca, Di Micco, Lucia, Di Nardo, Matteo, Ding, Xiaoqiang, D'Ippoliti, Fiorella, Di Somma, Salvatore, Doi, Kent, Dries, David J., Druml, Wilfred, Duke, Graeme, Durand, Francois, Eadon, Michael T., Eckstein, Devin, Egi, Moritoki, Eiam-Ong, Somchai, Elbers, Paul W.G., Elli, Francesca, Elliott, Steve, Emlet, David R., Endre, Zoltan, Evans, Roger G., Fanelli, Vito, Fattahi, Fatemeh, Federspiel, Christine Kinggaard, Ferrada, Marcela A., Ferrari, Fiorenza, Fiaccadori, Enrico, Fiorentino, Marco, Fisher, Caleb, Flessner, Michael F., Formica, Marco, Forni, Lui G., Francoz, Claire, French, Craig, Fuhrman, Dana Y., Fumagalli, Giordano, Galbusera, Miriam, Gallieni, Maurizio, Gammill, Hilary S., Gao, Dayong, Garzotto, Francesco, Gatta, Giuseppe, Genga, Kelly R., Genovesi, Simonetta, Genyk, Yuri S., Geradin, Christel, Gesualdo, Loreto, Giavarina, Davide, Giuliani, Anna, Glezerman, Ilya G., Goldstein, Stuart L., Golper, Thomas A., Gómez, Hernando, Granata, Antonio, Grandaliano, Giuseppe, Grasselli, Giacomo, Groeneveld, A.B. Johan, Guerci, Philippe, Gunnerson, Kyle J., Harbord, Nikolas, Harshman, Lyndsay A., Hennessy, Anthony J., Hill, Graham L., Hobson, Charles, Hohenstein, Bernd, Honoré, Patrick M., Horwitz, Edward, Hosseinian, Leila, Hoste, Eric A.J., House, Andrew A., Humes, H. David, Husain-Syed, Faeq, Ince, Can, Ing, Todd S., Jacobs, Rita, Jaswal, Dharmvir, Jeyabalan, Arun, Joannes-Boyau, Olivier, Joannidis, Michael, Joyce, Emily, Kane-Gill, Sandra L., Kaplan, Lewis J., Kashani, Kianoush, Katz, Nevin, Kellum, John A., Khanna, Ramesh, Kim-Campbell, Nahmah, King, Joshua D., Kirwan, Christopher J., Kiss, Joseph E., Klein, David, Kotanko, Peter, Krediet, Raymond T., Kuhlmann, Martin K., Kuiper, Jan Willem, Lachance, Philippe, Lameire, Norbert, Langer, Thomas, Lankadeva, Yugeesh R., Laurin, Louis-Philippe, Lazzeri, Elena, Leblanc, Martine, Lefebvre, Joannie, Lentini, Paolo, Leray-Moragués, Hélène, Levin, Adeera, Lew, Susie Q., Liapis, Helen, Liu, Kathleen D., Livigni, Sergio, Locatelli, Francesco, Lorenzin, Anna, Lu, Jian-Da, Lu, Renhua, Lysak, Nicholas, Macedo, Etienne, Madan, Niti, Madore, François, Maerz, Linda L., Maiden, Matthew J., Malhotra, Rakesh, Marengo, Marita, Mariano, Filippo, Marik, Paul E., Marini, John J., Marino, Rossella, Marshall, Mark R., Mårtensson, Johan, Matsuura, Ryo, May, Clive N., Mazzone, Patrizio, McCauley, Jerry, McCullough, Peter A., McMahon, Blaithin A., Mehta, Ravindra L., Mele, Caterina, Menon, Madhav, Meola, Mario, Mérouani, Aicha, Meuwly, Jean-Yves, Milla, Paola, Misra, Madhukar, Misra, Paraish S., Mizock, Barry A., Modi, Jwalant R., Moeckel, Gilbert, Molitoris, Bruce A., Morabito, Santo, Mucelli, Roberto Pozzi, Murray, Patrick T., Murugan, Raghavan, Nadim, Mitra K., Nair, Devika, Nalesso, Federico, Neri, Mauro, Nguyen, Trung C., Ni, Zhaohui, Noris, Marina, Novick, Tessa, O'Horo, John C., Okusa, Mark Douglas, Opal, Steven M., Opdam, Helen Ingrid, Ostermann, Marlies, Ottaviano, Emerenziana, Oudemans-van Straaten, Heleen M., Overgaard-Steensen, Christian, Padalino, Massimo A., Panichi, Vincenzo, Parameswaran, Priyanka, Patel, Samir S., Payen, Didier, Pea, Federico, Peacock, W. Frank, Peart, Sandrica Young, Peerapornratana, Sadudee, Pelosi, Paolo, Peng, Zhi-Yong, Perico, Norberto, Peruzzi, Licia, Pesce, Francesco, Pesenti, Antonio, Petrucci, Ilaria, Pham, Phuong-Chi, Pham, Phuong-Thu, Phoon, Richard K.S., Piano, Salvatore, Pinsky, Michael R., Piquilloud, Lise, Pistolesi, Valentina, Plank, Lindsay D., Plötz, Frans B., Podestá, Manuel Alfredo, Porta, Camillo, Pozzato, Marco, Prencipe, Michele, Prowle, John R., Puthucheary, Zudin A., Qu, Lirong, Rachoin, Jean-Sebastien, Radhakrishnan, Jai, Ranieri, V. Marco, Ratanarat, Ranistha, Remuzzi, Giuseppe, Resnick, Shelby, Rewa, Oleksa G., Ricci, Zaccaria, Ridel, Christophe, Rifai, Kinan, Ring, Troels, Rizo-Topete, Lilia M., Roessler, Eric, Romagnani, Paola, Romagnoli, Stefano, Ronco, Claudio, Ronco, Federico, Rosner, Mitchell H., Rossetti, Emanuele, Russell, James A., Saab, Georges, Sabatino, Alice, Saboo, Sonali S., Samoni, Sara, Sappington, Penny Lynn, Sartori, Marco, Savige, Judy, Schena, Francesco Paolo, Schneider, Antoine Guillaume, Schraverus, Pieter, Schulte, Wibke, Segoloni, Giuseppe, Semler, Matthew W., Sharma, Aashish, Shaw, Andrew, Sheth, Naitik, Shukla, Ashutosh, Siddall, Eric C., Sievers, Theodore M., Siew, Edward D., Singbartl, Kai, Singer, Mervyn, Singh, Pooja, Smith, Loren E., Soni, Sachin S., Soto, Mara Serrano, Spapen, Herbert D., Srisawat, Nattachai, Srivastava, Ajay, Stellin, Giovanni, Symons, Jordan M., Szamosfalvi, Balazs, Tai, Kian Bun, Takalkar, Unmesh V., Teitelbaum, Isaac, Tetta, Ciro, Thakar, Charuhas V., Tonon, Marta, Trepiccione, Francesco, Triulzi, Darrell, Tushar, Chopra, Uchino, Shigehiko, Valika, Ali, Van Biesen, Wim, Vandenberghe, Wim, Vanholder, Raymond, Vanmassenhove, Jill, Verbine, Anton, Vergano, Marco, Villa, Gianluca, Villeneuve, Pierre-Marc, Vincent, Jean-Louis, Vinsonneau, Christophe, Virzì, Grazia Maria, Visconti, Federico, Visvanathan, Ravindran, Van Vong, Li, Walmrath, Hans-Dieter, Ward, Peter A., Weir, Matthew A., Wen, Xiaoyan, Wendon, Julia, Winchester, James Frank, Wong, Adrian, Woodhouse, Elke L., Xue, Jun, Yadav, Anju, Yerram, Preethi, Yessayan, Lenar, Yeun, Jane Y., Yu, Alex W., Zaccaria, Marta, Zacchia, Miriam, Zachariah, Teena P., Zamperetti, Nereo, Zampieri, Fernando G., Zanco, Pierluigi, Zanella, Alberto, Zanoli, Luca, Zappitelli, Michael, Zaragoza, Jose J., Zarbock, Alexander, Zaroccolo, Marta, Zhang, Han, and Zimmer, Andrea

Published
2019
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19. Draining Fluids through a Peritoneal Catheter in Newborns after Cardiac Surgery Helps to Control Fluid Balance

Author

Ruano Cea, Elisa, Jouvet, Philippe, Vobecky, Suzanne, and Merouani, Aicha

Subjects
Article Subject
Abstract

Dialysis can be used in severe cases, but may not be well tolerated. In such patients, peritoneal drainage could be an alternative option for fluid removal. We report the case of a newborn with a truncus arteriosus who developed postoperatively a complicated clinical course with right ventricular dysfunction, prerenal condition as well as fluid overload despite diuretic therapy. Dialysis was indicated for fluid removal. Peritoneal dialysis was started using a surgically placed Tenckhoff catheter and stopped due to inefficacy and leaks and no other modalities of dialysis were used. However, the catheter was left in place over a period of two months for fluid drainage and removed because of unexplained fever. In order to determine the effect of peritoneal drainage, we selected a period of one week before and one week after the removal of the drain to compare daily clinical data, urine electrolytes and renal function and found a positive effect on fluid balance control. We conclude that the fluid removal by continuous peritoneal drainage is a simple and safe alternative that can be used to control fluid balance in infants after cardiac surgery.

Published
2010
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22. Systemic and renal hemodynamic changes in the luteal phase of the menstrual cycle mimic early pregnancy.

Author

CHAPMAN, ARLENE B., ZAMUDIO, STACY, WOODMANSEE, WHITNEY, MEROUANI, AICHA, OSORIO, FRITZ, JOHNSON, ANN, MOORE, LORNA G., DAHMS, THOMAS, COFFIN, CAROLYN, ABRAHAM, WILLIAM T., and SCHRIER, ROBERT W.

Abstract

Blood pressure decreases during early pregnancy in association with a decrease in peripheral vascular resistance and increases in renal plasma flow and glomerular filtration rate. These early changes suggest a potential association with corpora lutea function. To determine whether peripheral vasodilation occurs following ovulation, we studied 16 healthy women in the midfollicular and midluteal phases of the menstrual cycle. A significant decrease in mean arterial pressure in the midluteal phase of the cycle (midfollicular of 81.7 ± 2.0 vs. midluteal of 75.4 ± 2.3 mmHg, P < 0.005) was found in association with a decrease in systemic vascular resistance and an increase in cardiac output. Renal plasma flow and glomerular filtration rate increased. Plasma renin activity and aldosterone concentration increased significantly in the luteal phase accompanied by a decrease in atrial natriuretic peptide concentration. Serum sodium, chloride, and bicarbonate concentrations and osmolarity also declined significantly in the midluteal phase of the menstrual cycle. Urinary adenosine 3',5'-cyclic monophosphate (cAMP) excretion increased in the luteal compared with the follicular phase, whereas no changes in urinary cGMP or NO2/NO3 excretion were found. Thus peripheral vasodilation occurs in the luteal phase of the normal menstrual cycle in association with an increase in renal plasma flow and filtration. Activation of the renin-angiotensin-aldosterone axis is found in the luteal phase of the menstrual cycle. These changes are accompanied by an increase in urinary cAMP excretion indicating potential vasodilating mediators responsible for the observed hemodynamic changes. [ABSTRACT FROM AUTHOR]

Published
1997
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25. Contributors

Author

Abaterusso, Cataldo, Ahern, Stéphane P., Aisa, Maria Cristina, Albright, Robert C., Jr., Alfaro, Vicente, Al-Khafaji, Ali, Allo, Jean-Christophe, Amerling, Richard, Amore, Alessandro, Anderson, Robert J., Andreucci, Michele, Andreucci, Vittorio E., Andrikos, Emilios, Arroyo, Vicente, Arthur, John M., Ash, Stephen R., Assanelli, Emilio, Aucella, Filippo, Bagshaw, Sean M., Balafa, Olga, Balbi, André Luís, Baldwin, Ian, Ballestri, Marco, Bargman, Joanne M., Barletta, Gina-Marie, Barletta, Jeffrey F., Barozzi, Libero, Barsoum, Rashad S., Beaulieu, Monica, Beer-Stolz, Donna, Bellomo, Rinaldo, Bernardo, Jose, Bertolotto, Michele, Bestoso, John T., Betro, Gerard A., Bhattacharya, Mallar, Bihl, Geoffrey R., Blot, Stijn I., Boer, Willem, Boim, Mirian A., Bonello, Monica, Bonventre, Joseph V., Booth, A.D., Bourke, Edmund, Braitberg, George, Brancaccio, Diego, Brendolan, Alessandra, Brezzi, Brigida, Brodie, James C., Brophy, Patrick D., Brown, Ryan, Bucala, Richard, Buckmaster, Jonathan, Budisavljevic, Milos N., Bunchman, Timothy E., Burdmann, Emmanuel A., Bussolati, Benedetta, Butkus, Matthew A., Buzzelli, Daniela, Calzavacca, Paolo, Camussi, Giovanni, Canaud, Bernard, Cano, Noël J.M., Cantaluppi, Vincenzo, Capasso, Giovambattista, Cappelli, Gianni, Carlesso, Eleonora, Casino, Francesco G., Castillo, Leticia, Cerutti, Roberta, Chawla, Lakhmir S., Chionh, Chang Yin, Chiu, Alexander, Chow, May T., Chugh, Kirpal S., Cianciaruso, Bruno, Cignarelli, Mauro, Claessens, Yann-Erick, Clark, John A., Clark, William R., Cohen, David J., Cohen, Scott D., Constable, Peter, Coppo, Rosanna, Corey, Howard E., Cozzolino, Mario, Craig, Maureen, Cramer, Carl H., II, Crandall, Mark, Cravedi, Paolo, Crepaldi, Carlo, Crew, R. John, Cronin, Donald F., Cruz, Dinna N., Canton, Antonio Dal, Dan, Maurizio, D'Angelo, Angela, Davenport, Andrew, Davies, Andrew R., Dear, James W., Gasperi, Andrea De, Dell'Aquila, Roberto, Rocca, Giorgio Della, Prete, Dorella Del, Delude, Russell L., Depner, Thomas, Derchi, Lorenzo E., Devarajan, Prasad, Waele, Jan J. De, Dhainaut, Jean-François, Diaz-Buxo, José A., Micco, Lucia Di, Divino-Filho, José Carolina, Doernberg, Sarah, Doig, Gordon S., Dries, David J., Drudi, Francesco Maria, Druml, Wilfred, Duke, Graeme, Durward, Andrew, Egi, Moritoki, Esposito, Ciro, Evenepoel, Pieter, Faga, Teresa, Fan, Sheung Tat, Feinfeld, Donald A., Féraille, Eric, Fernández, Javier, Finfer, Simon, Fink, Mitchell P., Finkel, Kevin W., Flessner, Michael F., Formica, Marco, Forni, Lui G., Fortenberry, James D., French, Craig, Fumagalli, Roberto, Furlanut, Mario, Fute, Micheline Djouguela, Gabriel, Daniela Ponce, Galassi, Andrea, Galbusera, Miriam, Galli, Francesco, Galli, Giovanni, Gallieni, Maurizio, Gallo, Giampiero, Gambaro, Giovanni, Gammill, Hilary S., Gangemi, Ezio Nicola, Gao, Dayong, Garwood, Susan, Garzotto, Francesco, Gatti, Antonietta M., Gattinoni, Luciano, Geibel, John P., George, Stephen, Gesualdo, Loreto, Gibney, R.T. Noel, Gipson, Debbie S., Glezerman, Ilya G., Glorieux, Griet, Goldstein, Stuart L., Golper, Thomas A., Gowrishankar, Manjula, Grandi, Fabio, Gregoretti, Cesare, Grinnell, Brian W., Groeneveld, A.B. Johan, Gruber, Steven J., Guadagni, Gualtiero, Gunnerson, Kyle J., Gupta, Akanksha, Gura, Victor, Guzzo, Isabella, Hackbarth, Richard, Halperin, Mitchell L., Harbord, Nikolas, Haymann, Jean-Philippe, Heffner, Alan C., Hennessy, Anthony J., Heyman, Samuel N., Hill, Graham L., Hilton, Philip J., Himmelfarb, Jonathan, Hirasawa, Hiroyuki, Hoenich, Nicholas A., Holdsworth, Stephen R., Holley, Anthony, Honoré, Patrick M., Hoste, Eric A.J., House, Andrew A., Huang, David T., Huang, Zhongping, Hubmayr, Rolf D., Hughes, Alun D., Humes, H. David, Ikizler, T. Alp, Imberti, Barbara, Ing, Todd S., Jaber, Bertrand L., Janvier, Gérard, Jeyabalan, Arundhathi, Jha, Vivekanand, Joannes-Boyau, Olivier, Joannidis, Michael, Jones, Daryl A., Jörres, Achim, Kamel, Kamel S., Kamp, Ryan C., Kannan, Neeta, Kaplan, Lewis J., Karacan, Özgür, Karajala-Subramanyam, Vijay, Kaushal, Gur P., Kellum, John A., Kemper, Markus J., Khan, Asjad, Khanna, Ramesh, Kher, Vijay, Kimmel, Paul L., Kindgen-Milles, Detlef, Kitching, A. Richard, Kjellstrand, Carl M., Kohn, Orly F., Kooienga, Laura A., Kooman, Jeroen P., Kotanko, Peter, Krediet, Raymond T., Kroon, A.A., Kuang, Dingwei, Kuhlmann, Martin K., Kuiper, Jan Willem, Lam, Man-Fai, Lamacchia, Olga, Lameire, Norbert, Langenberg, Christoph, Lauri, Gianfranco, Leblanc, Martine, Ledebo, Ingrid, Lentini, Paolo, Leonard, Edward F., Letteri, Jeffrey J., Leunissen, Karel M., Leverve, Xavier M., Levin, Adeera, Leypoldt, John K., Liangos, Orfeas, Licari, Elisa, Lieberthal, Wilfred, Linden, Peter K., Lipman, Jeffrey, Liu, Kathleen D., Liu, Shiguang, Livigni, Sergio, Lo, Wai-Kei, Lugano, Manuela, Lui, Sing-Leung, Lupo, Antonio, Luyckx, Valerie A., Macias, William L., Madden, Nicholas, Madore, François, Majors, Daniel S., Mancini, Elena, Mangione, Filippo, Mankad, Sunil, Manzini, Pier Paolo, Marangella, Martino, Marenzi, Giancarlo, Mariano, Filippo, Marik, Paul E., Marini, John J., Marquis, François, Marshall, John C., Marshall, Mark R., Mathew, Roy, Matsuda, Kenichi, Matthay, Michael A., Maxvold, Norma J., May, Clive N., McCauley, Jerry, McCunn, Maureen, McKenna, Joseph, Mehta, Ravindra L., Mele, Caterina, Merouani, Aicha, Mesnard, Laurent, Messa, Piergiorgio, Metnitz, Philipp G.H., Misra, Madhukar, Mitzner, Steffen R., Mizock, Barry A., Mokhlesi, Babak, Molitoris, Bruce A., Morelli, Andrea, Morgan, Thomas John, Morigi, Marina, Mount, Peter, Mucelli, Roberto Pozzi, Mueller, Bruce A., Murray, Patrick, Murugan, Raghavan, Nakamura, Masataka, Nalesso, Federico, Nester, Carla M., Nissenson, Allen, Nolph, Karl, Ocampo, Catalina, Oda, Shigeto, Okusa, Mark D., Opal, Steven M., Opdam, Helen, Osswald, Hartmut, Straaten, Heleen M. Oudemans–van, Padalino, Massimo A., Paden, Matthew L., Paganini, Emil P., Palevsky, Paul M., Panat, Mani John, Paolini, Francesco, Parikh, Dipen, Patroniti, Nicolò, Pavlica, Pietro, de La Garanderie, Didier Payen, Pea, Federico, Peng, Zhiyong, Perazella, Mark A., Perego, Angelo F., Pereira, Estela Regina, Pérez, Evans R. Fernández, Perico, Norberto, Pertica, Nicoletta, Pertosa, Giovanni, Peruzzi, Licia, Petras, Dimitris, Pham, Phuong-Chi, Pham, Phuong-Thu, Phoon, Richard K.S., Picca, Stefano, Piccinni, Pasquale, Pinsk, Maury N., Pinsky, Michael R., Piroddi, Marta, Plamondon, Isabelle, Plank, Lindsay D., Plötz, Frans B., Poghosyan, Lusine, Polanco, Natalia, Polanco, Patricio M., Polaschegg, Hans Dietrich, Ponikvar, Rafael, Porecca, Silvia, Portilla, Didier, Powell, T. Brian, Quigley, Raymond, Rabb, Hamid, Ragaller, Maximilian, Rampino, Teresa, Ranpuria, Reena, Rastogi, Anjay, Ratanarat, Ranistha, Raza, Naem, Reade, Michael C., Reeves, John H., Reiter, Karl, Remuzzi, Giuseppe, Ricci, Zaccaria, Ricksten, Sven-Erik, Ridel, Christophe, Rifai, Kinan, Ring, Troels, Riopel, Julie, Rocha, Eduardo, Roessler, Eric, Rona, Roberto, Ronco, Claudio, Rondeau, Eric, Rosen, Seymour, Rosenberger, Christian, Rowan, Shane, Roy, Thomas, Saab, Georges, Sadahiro, Tomohito, Sala, Carla, Sala, Chiara, Salama, Alan D., Salmon, Adrian, Salvatori, Gabriela, Sam, Ramin, Sandoval, Ruben M., Santoro, Antonio, Saotome, Takao, Sappington, Penny L., Sarma, J. Vidya, Savige, Judy, Schena, Francesco Paolo, Schepers, Eva, Schetz, Miet, Schmidt, Gregory A., Schor, Nestor, Schusterschitz, Nicola, Segoloni, Giuseppe, Shah, Nirva, Shah, Shamik H., Shah, Sudhir V., Sharfuddin, Asif A., Shaw, Andrew, Shiga, Hidetoshi, Shoji, Hisataka, Shukla, Ashutosh, Simpson, Fiona, Singbartl, Kai, Singer, Mervyn, Solez, Kim, Sowinski, Kevin M., Stafford-Smith, Mark, Stange, Jan, Stefanelli, Luca, Stein, Deborah M., Stella, Maurizio, Stellin, Giovanni, Story, David A., Subramanian, Sanjay, Swärd, Kristina, Symons, Jordan M., Tai, Kian Bun, Tattersall, James, Tedeschi, Luisa, Teitelbaum, Isaac, Teixeira, Vicente P. Castro, Tetta, Ciro, Thakar, Charuhas V., Theilen, Hermann, Thomas, Karl W., Tolwani, Ashita, Trepiccione, Francesco, Triolo, Giorgio, Tsang, Jennifer L.Y., Tutal, Emre, Uchino, Shigehiko, Unruh, Mark, Vail, G. Matthew, Valentino, Massimo, Vallon, Volker, Van Biesen, Wim, van der Sande, Frank M., Vandijck, Dominique M., Vanholder, Raymond, Varghese, Sanju A., Venkataraman, Ramesh, Venkatesh, Bala, Verbine, Anton, Vincent, Jean-Louis, Vinsonneau, Christophe, Visvanathan, Ravindran, Voinescu, Alexandra, Walters, Scott, Wan, Li, Ward, Peter A., Ward, Richard A., Warrillow, Stephen, Webb, Steve, Whitlow, Kenneth Scott, Wieslander, Anders, Wilkinson, Alan H., Wille, Keith, Winchester, James Frank, Wu, Christine, Yassin, James, Yeun, Jane Y., Yip, Terence Pok-Siu, Yu, Alex W., Zacchia, Miriam, Zaida, Najam, Zamperetti, Nereo, Zappitelli, Michael, and Zarbock, Alexander

Published
2009
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26. Draining Fluids through a Peritoneal Catheter in Newborns after Cardiac Surgery Helps to Control Fluid Balance.

Author

Cea, Elisa Ruano, Jouvet, Philippe, Vobecky, Suzanne, and Merouani, Aicha

Subjects
PERITONEAL access, CARDIAC surgery, NEONATAL surgery, REGULATION of body fluids, CATHETERS, TRUNCUS arteriosus
Abstract

Fluid overload, a common complication following cardiac surgery in infants is often difficult to manage. Dialysis can be used in severe cases, but may not be well tolerated. In such patients, peritoneal drainage could be an alternative option for fluid removal. We report the case of a newborn with a truncus arteriosus who developed postoperatively a complicated clinical course with right ventricular dysfunction, prerenal condition as well as fluid overload despite diuretic therapy. Dialysis was indicated for fluid removal. Peritoneal dialysis was started using a surgically placed Tenckhoff catheter and stopped due to inefficacy and leaks and no other modalities of dialysis were used. However, the catheter was left in place over a period of two months for fluid drainage and removed because of unexplained fever. In order to determine the effect of peritoneal drainage, we selected a period of one week before and one week after the removal of the drain to compare daily clinical data, urine electrolytes and renal function and found a positive effect on fluid balance control. We conclude that the fluid removal by continuous peritoneal drainage is a simple and safe alternative that can be used to control fluid balance in infants after cardiac surgery. [ABSTRACT FROM AUTHOR]

Published
2010
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27. Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec

Author

Larochelle, Jean, Alvarez, Fernando, Bussières, Jean-François, Chevalier, Isabelle, Dallaire, Louis, Dubois, Josée, Faucher, Frédéric, Fenyves, Daphna, Goodyer, Paul, Grenier, André, Holme, Elisabeth, Laframboise, Rachel, Lambert, Marie, Lindstedt, Sven, Maranda, Bruno, Melançon, Serge, Merouani, Aicha, Mitchell, John, Parizeault, Guy, and Pelletier, Luc

Subjects
*HEPATORENAL syndrome, *TYROSINEMIA, *HYDROLASES, *PERIPHERAL neuropathy, *METABOLITES, *LONGITUDINAL method
Abstract

Abstract: Background: Hepatorenal tyrosinemia (HT1, fumarylacetoacetate hydrolase deficiency, MIM 276700) can cause severe hepatic, renal and peripheral nerve damage. In Québec, HT1 is frequent and neonatal HT1 screening is practiced. Nitisinone (NTBC, Orfadin ®) inhibits tyrosine degradation prior to the formation of toxic metabolites like succinylacetone and has been offered to HT1 patients in Québec since 1994. Methods: We recorded the clinical course of 78 Québec HT1 patients born between 1984 and 2004. There were three groups: those who never received nitisinone (28 patients), those who were first treated after 1month of age (26 patients) and those treated before 1month (24 patients). Retrospective chart review was performed for events before 1994, when nitisinone treatment began, and prospective data collection thereafter. Findings: No hospitalizations for acute complications of HT1 occurred during 5731months of nitisinone treatment, versus 184 during 1312months without treatment (p<0.001). Liver transplantation was performed in 20 non-nitisinone-treated patients (71%) at a median age of 26months, versus 7 late-treated patients (26%, p<0.001), and no early-treated patient (p<0.001). No early-treated patient has developed detectable liver disease after more than 5years. Ten deaths occurred in non-nitisinone treated patients versus two in treated patients (p<0.01). Both of the latter deaths were from complications of transplantation unrelated to HT1. One probable nitisinone-related event occurred, transient corneal crystals with photophobia. Interpretation: Nitisinone treatment abolishes the acute complications of HT1. Some patients with established liver disease before nitisinone treatment eventually require hepatic transplantation. Patients who receive nitisinone treatment before 1month had no detectable liver disease after more than 5years. [Copyright &y& Elsevier]

Published
2012
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28. The Québec NTBC Study.

Author

Alvarez F, Atkinson S, Bouchard M, Brunel-Guitton C, Buhas D, Bussières JF, Dubois J, Fenyves D, Goodyer P, Gosselin M, Halac U, Labbé P, Laframboise R, Maranda B, Melançon S, Merouani A, Mitchell GA, Mitchell J, Parizeault G, Pelletier L, Phan V, and Turcotte JF

Subjects
Heptanoates metabolism, Humans, Infant, Newborn, Liver Diseases drug therapy, Liver Diseases etiology, Liver Diseases metabolism, Liver Transplantation methods, Neonatal Screening methods, Quebec, Tyrosinemias complications, Tyrosinemias metabolism, Cyclohexanones therapeutic use, Enzyme Inhibitors therapeutic use, Nitrobenzoates therapeutic use, Tyrosinemias drug therapy
Abstract

In this chapter we describe the current Quebec NTBC Study protocol. Quebec's unique characteristics have influenced the development of the protocol, including a high prevalence of hepatorenal tyrosinemia (HT1), universal newborn screening for HT1, availability of treatment with nitisinone (NTBC) and special diet, a large territory, where HT1 treatment is coordinated by a small number of centers. Screened newborns are seen within 3 weeks of birth. Patients with liver dysfunction (prolonged prothrombin time and/or international normalized ratio (INR) provide sensitive, rapidly available indicators) are treated by NTBC and special diet. The specific diagnosis is confirmed by diagnostic testing for succinylacetone (SA) in plasma and urine samples obtained before treatment. After an initial period of frequent surveillance, stable patients are followed every 3 months by assay of plasma amino acids and NTBC and plasma and urine SA. Abdominal ultrasound is done every 6 months. Patients have an annual visit to the coordinating center that includes multidisciplinary evaluations in metabolic genetics, hepatology, imaging (for abdominal ultrasound and magnetic resonance imaging) and other specialties as necessary. If hepatocellular carcinoma is suspected by imaging and/or because of progressive elevation of alphafetoprotein, liver transplantation is discussed. To date, no patient in whom treatment was started before 1 month of age has developed hepatocellular carcinoma, after surveillance for up to 20 years in some. This patient group is the largest in the world that has been treated rapidly following newborn screening. The protocol continues to evolve to adapt to the challenges of long term surveillance.

Published
2017
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