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3. Nephropathic cystinosis in children: An overlooked disease

Author

Neveen Soliman, El-Baroudy, R., Rizk, A., Bazaraa, H., and Younan, A.

Subjects
Male, Administration, Topical, Cysteamine, Cystinosis, Administration, Oral, lcsh:Medicine, Diagnostic Techniques, Ophthalmological, Severity of Illness Index, Corneal Diseases, Medication Adherence, Cornea, Predictive Value of Tests, Diagnosis, Humans, Child, Corneal cystine crystal score, lcsh:R, Infant, Cysteamine eye drops, Fanconi Syndrome, Treatment Outcome, Child, Preschool, Disease Progression, Kidney Failure, Chronic, Female, Ophthalmic Solutions
Abstract

Nephropathic cystinosis is rare genetic disease characterized by defective lysosomal cystine transport and increased lysosomal cystine. Corneal Cystine Crystal Scoring (CCCS) for diagnosis of nephropathic cystinosis was studied in all suspected children with renal Fanconi syn-drome and siblings of diagnosed cases over a two year period. In addition to oral cysteamine, cys-teamine eye drops were provided to all diagnosed patients and CCCS was followed up on a quarterly basis. Of 33 screened cases, 14 had corneal cystine crystals. Crystals were absent in two cystinotic patients under the age of 20 months. The mean age at diagnosis was 52.7 months and five patients had ERSD. After six months of treatment, the mean CCCS did not increase from the initial value of 1.81; associated with a decrease of 0.5 in two cases and a similar increase in two others. Scores decreased in two other patients after 12 months. Compliance was generally inadequate due to the high frequency of administration and the need for multi-drug regimen. CCCS is a simple and reasonably sensitive method for diagnosis of nephropathic cystinosis above two years of age. To-pical treatment with cysteamine eye drops prevents progression of deposits and may decrease it with adequate compliance. Further follow up is still recommended to monitor long term effects of both systemic and topical cysteamine therapy.

Published
2009

4. Lack of association of CTLA-4 +49 A/G polymorphism with predisposition to type 1 diabetes in a cohort of Egyptian families

Author

Kamel, AM, Mira, MF, Mossallam, GI, Ebid, GTA, Radwan, ER, Aly Eldin, NH, Mamdouh, M, Amin, M, Badawy, N, Bazaraa, H, Ibrahim, A, and Salah, N

Abstract

Background: Type 1 diabetes is one of the most common chronic childhood illnesses. Interplay between genetic susceptibility and environmental factors is thought to provide the fundamental element for the disease. Apart from the Major Histocompatibility locus which is the main contributor to risk susceptibility, more than 40 loci are recognized. One among these is the CTLA-4, however data from the literature are controversial. The aim of our study was to investigate the role of CTLA4 49 A/G as a risk susceptibility factor for the development of type 1 diabetes in a cohort of Egyptian families.Subjects and methods: This is a case control study including 88 Egyptian families with one or more index cases (

Published
2014

5. Paediatric nephrology II

Author

Musial, K., primary, Zwolinska, D., additional, Pruthi, R., additional, Sinha, M., additional, Casula, A., additional, Lewis, M., additional, Tse, Y., additional, Maxwell, H., additional, O'Brien, C., additional, Inward, C., additional, Sharaf, E., additional, Fadel, F., additional, Bazaraa, H., additional, Hegazy, R., additional, Essam, R., additional, Manickavasagar, B., additional, Shroff, R., additional, McArdle, A., additional, Ledermann, S., additional, Shaw, V., additional, Van't Hoff, W., additional, Paudyal, B., additional, Prado, G., additional, Schoeneman, M., additional, Nepal, M. K., additional, Feygina, V., additional, Bansilal, V., additional, Tawadrous, H., additional, Mongia, A. K., additional, Melk, A., additional, Kracht, D., additional, Doyon, A., additional, Zeller, R., additional, Litwin, M., additional, Duzowa, A., additional, Sozeri, B., additional, Bayzit, A., additional, Caliskan, S., additional, Querfeld, U., additional, Wuhl, E., additional, Schaefer, F., additional, Schmidt, B., additional, Canpolat, N., additional, Kara Acar, M., additional, Pehlivan, S., additional, Tasdemir, M., additional, Sever, L., additional, Nusken, E., additional, Taylan, C., additional, von Gersdorff, G., additional, Schaller, M., additional, Barth, C., additional, Dotsch, J., additional, Roomizadeh, P., additional, Gheissari, A., additional, Abedini, A., additional, Garzotto, F., additional, Zanella, M., additional, Kim, J., additional, Cena, R., additional, Neri, M., additional, Nalesso, F., additional, Brendolan, A., additional, Ronco, C., additional, Celkan, T., additional, Lacinel, S., additional, Keser, A., additional, Taner Elmas, A., additional, Tabel, Y., additional, Ipek, S., additional, Karadag, A., additional, Elmas, O., additional, Ozyalin, F., additional, Hoxha (Qosja), A., additional, Gjyzari, A., additional, Tushe, E., additional, Said, R. M., additional, Abdel Fattah, M. A., additional, Soliman, D. A., additional, Mahmoud, S. Y., additional, Hattori, M., additional, Uemura, O., additional, Hataya, H., additional, Ito, S., additional, Hisano, M., additional, Ohta, T., additional, Fujinaga, S., additional, Kise, T., additional, Goto, Y., additional, Matsunaga, A., additional, Hashimoto, T., additional, Tsutsumi, Y., additional, Ito, N., additional, Akizawa, T., additional, Maher, S., additional, Cho, B.-S., additional, Choi, Y.-M., additional, Suh, J.-S., additional, Farid, F., additional, El-Hakim, I., additional, Salman, M., additional, Rajnochova Bloudickova, S., additional, Viklicky, O., additional, Seeman, T., additional, Yuksel, S., additional, Caglar, M., additional, Becerir, T., additional, Tepeli, E., additional, Calli Demirkan, N., additional, Yalcin, N., additional, Ergin, A., additional, Hladik, M., additional, Sigutova, R., additional, Vsiansky, F., additional, Safarcik, K., additional, Svagera, Z., additional, Abd El Monem Soliman, N., additional, Bazaraa, H. M., additional, Nabhan, M. M., additional, Badr, A. M., additional, Abd El Latif Shahin, M., additional, Skrzypczyk, P., additional, Panczyk-Tomaszewska, M., additional, Roszkowska-Blaim, M., additional, Wawer, Z., additional, Bienias, B., additional, Zajaczkowska, M., additional, Szczepaniak, M., additional, Pawlak-Bratkowska, M., additional, Tkaczyk, M., additional, Kilis-Pstrusinska, K., additional, Jakubowska, A., additional, Prikhodina, L., additional, Ryzhkova, O., additional, Poltavets, N., additional, and Polyakov, V., additional

Published
2013
Full Text
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6. ABSTRACT 829

Author

Rady, H., primary, Hafez, M., additional, Bazaraa, H., additional, and Aly, Y., additional

Published
2014
Full Text
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7. Poster Session 3

Author

Fabbri, G. M. T., primary, Baldasseroni, S., additional, Panuccio, D., additional, Zoni Berisso, M., additional, Scherillo, M., additional, Lucci, D., additional, Di Pasquale, G., additional, Mathieu, G., additional, Burazor, I., additional, Burazor, M., additional, Perisic, Z., additional, Atanaskovic, V., additional, Erakovic, V., additional, Stojkovic, A., additional, Vogtmann, T., additional, Schoebel, C., additional, Sogorski, S., additional, Sebert, M., additional, Schaarschmidt, J., additional, Fietze, I., additional, Baumann, G., additional, Penzel, T., additional, Mornos, C., additional, Ionac, A., additional, Cozma, D., additional, Dragulescu, D., additional, Mornos, A., additional, Petrescu, L., additional, Pescariu, L., additional, Brembilla-Perrot, B., additional, Khachab, H., additional, Lamberti, F., additional, Bellini, C., additional, Remoli, R., additional, Cogliandro, T., additional, Nardo, R., additional, Bellusci, F., additional, Mazzuca, V., additional, Gaspardone, A., additional, Aguinaga Arrascue, L. E., additional, Bravo, A., additional, Garcia Freire, P., additional, Gallardo, P., additional, Hasbani, E., additional, Quintana, R., additional, Dantur, J., additional, Inoue, K., additional, Ueoka, A., additional, Tsubakimoto, Y., additional, Sakatani, T., additional, Matsuo, A., additional, Fujita, H., additional, Kitamura, M., additional, Wegrzynowska, M., additional, Konduracka, E., additional, Pietrucha, A. Z., additional, Mroczek-Czernecka, D., additional, Paradowski, A., additional, Bzukala, I., additional, Nessler, J., additional, Igawa, O., additional, Adachi, M., additional, Atarashi, H., additional, Kusama, Y., additional, Kodani, E., additional, Okazaki, R., additional, Nakagomi, A., additional, Endoh, Y., additional, Baez-Escudero, J. L., additional, Dave, A. S., additional, Sasaridis, C. M., additional, Valderrabano, M., additional, Tilz, R., additional, Bai, R., additional, Di Biase, L., additional, Gallinghouse, G. J., additional, Gibson, D., additional, Pisapia, A., additional, Wazni, O., additional, Natale, A., additional, Arujuna, A., additional, Karim, R., additional, Rinaldi, A., additional, Cooklin, M., additional, Rhode, K., additional, Razavi, R., additional, O'neill, M., additional, Gill, J., additional, Kusa, S., additional, Komatsu, Y., additional, Kakita, K., additional, Takayama, K., additional, Taniguchi, H., additional, Otomo, K., additional, Iesaka, Y., additional, Ammar, S., additional, Reents, T., additional, Fichtner, S., additional, Wu, J., additional, Zhu, P., additional, Kolb, C., additional, Hessling, G., additional, Deisenhofer, I., additional, Gilbert, G., additional, Mohanty, P., additional, Cunningham, J., additional, Metz, T., additional, Horton, R., additional, Tao, S., additional, Yamauchi, Y., additional, Okada, H., additional, Maeda, S., additional, Obayashi, T., additional, Isobe, M., additional, Chan, J., additional, Johar, S., additional, Wong, T., additional, Markides, V., additional, Hussain, W., additional, Konstantinidou, M., additional, Wissner, E., additional, Fuernkranz, A., additional, Yoshiga, Y., additional, Metzner, A., additional, Kuck, K.- H., additional, Ouyang, F., additional, Kettering, K., additional, Gramley, F., additional, Mollnau, H., additional, Weiss, C., additional, Bardeleben, S., additional, Biasco, L., additional, Scaglione, M., additional, Caponi, D., additional, Di Donna, P., additional, Sergi, D., additional, Cerrato, N., additional, Blandino, A., additional, Gaita, F., additional, Fiala, M., additional, Wichterle, D., additional, Sknouril, L., additional, Bulkova, V., additional, Chovancik, J., additional, Nevralova, R., additional, Pindor, J., additional, Januska, J., additional, Choi, J. I., additional, Ban, J. E., additional, Yasutsugu, N., additional, Park, J. S., additional, Jung, J. S., additional, Lim, H. E., additional, Park, S. W., additional, Kim, Y. H., additional, Kuhne, M., additional, Reichlin, T., additional, Ammann, P., additional, Schaer, B., additional, Osswald, S., additional, Sticherling, C., additional, Ohe, M., additional, Goya, M., additional, Hiroshima, K., additional, Hayashi, K., additional, Makihara, Y., additional, Nagashima, M., additional, Fukunaga, M., additional, An, Y., additional, Dorwarth, U., additional, Schmidt, M., additional, Wankerl, M., additional, Krieg, J., additional, Straube, F., additional, Hoffmann, E., additional, Kathan, S., additional, Defaye, P., additional, Mbaye, A., additional, Cassagneau, R., additional, Gagniere, V., additional, Jacon, P., additional, Pokushalov, E., additional, Romanov, A., additional, Artemenko, S., additional, Shabanov, V., additional, Elesin, D., additional, Stenin, I., additional, Turov, A., additional, Losik, D., additional, Kondo, K., additional, Miake, J., additional, Yano, A., additional, Ogura, K., additional, Kato, M., additional, Shigemasa, C., additional, Sekiguchi, Y., additional, Tada, H., additional, Yoshida, K., additional, Naruse, Y., additional, Yamasaki, H., additional, Igarashi, M., additional, Machino, T., additional, Aonuma, K., additional, Chen, S., additional, Liu, S., additional, Chen, G., additional, Meng, W., additional, Zhang, F., additional, Yan, Y., additional, Sciarra, L., additional, Dottori, S., additional, Lanzillo, C., additional, De Ruvo, E., additional, De Luca, L., additional, Minati, M., additional, Lioy, E., additional, Calo', L., additional, Lin, J., additional, Nie, Z., additional, Zhu, M., additional, Wang, X., additional, Zhao, J., additional, Hu, W., additional, Tao, H., additional, Ge, J., additional, Johansson, B., additional, Houltz, B., additional, Edvardsson, N., additional, Schersten, H., additional, Karlsson, T., additional, Wandt, B., additional, Berglin, E., additional, Hoyt, R. H., additional, Jenson, B. P., additional, Trines, S. A. I. P., additional, Braun, J., additional, Tjon Joek Tjien, A., additional, Zeppenfeld, K., additional, Tavilla, G., additional, Klautz, R. J. M., additional, Schalij, M. J., additional, Krausova, R., additional, Cihak, R., additional, Peichl, P., additional, Kautzner, J., additional, Pirk, J., additional, Skalsky, I., additional, Maly, J., additional, Imai, K., additional, Sueda, T., additional, Orihashi, K., additional, Picarra, B. C., additional, Santos, A. R., additional, Dionisio, P., additional, Semedo, P., additional, Matos, R., additional, Leitao, M., additional, Banha, M., additional, Trinca, M., additional, Elder, D. H. J., additional, George, J., additional, Jain, R., additional, Lang, C. C., additional, Choy, A. M., additional, Konert, M., additional, Loescher, S., additional, Hartmann, A., additional, Aversa, E., additional, Chirife, R., additional, Sztyglic, E., additional, Mazzetti, H., additional, Mascheroni, O., additional, Tentori, M. C., additional, Pop, R. M., additional, Margulescu, A. D., additional, Dulgheru, R., additional, Enescu, O., additional, Siliste, C., additional, Vinereanu, D., additional, Menezes Junior, A., additional, Castro Carneiro, A. R., additional, De Oliveira, B. L., additional, Shah, A. N., additional, Kantharia, B., additional, De Lucia, R., additional, Soldati, E., additional, Segreti, L., additional, Di Cori, A., additional, Zucchelli, G., additional, Viani, S., additional, Paperini, L., additional, Bongiorni, M. G., additional, Kutarski, A., additional, Czajkowski, M., additional, Pietura, R., additional, Malecka, B., additional, Heintze, J., additional, Eckardt, L., additional, Bauer, A., additional, Meine, M., additional, Van Erven, L., additional, Bloch Thomsen, P. E., additional, Lopez Chicharro, M. P., additional, Merhi, O., additional, Soga, Y., additional, Andou, K., additional, Nobuyoshi, M., additional, Gonzalez-Mansilla, A., additional, Martin-Asenjo, R., additional, Unzue, L., additional, Torres, J., additional, Garralda, E., additional, Coma, R. R., additional, Rodriguez Garcia, J. E., additional, Yaegashi, T., additional, Furusho, H., additional, Kato, T., additional, Chikata, A., additional, Takashima, S., additional, Usui, S., additional, Takamura, M., additional, Kaneko, S., additional, Chudzik, M., additional, Mitkowski, P., additional, Przybylski, A., additional, Lewek, J., additional, Smukowski, T., additional, Maciag, A., additional, Castrejon Castrejon, S., additional, Perez-Silva, A., additional, Estrada, A., additional, Doiny, D., additional, Ortega, M., additional, Lopez-Sendon, J. L., additional, Merino, J. L., additional, O'mahony, C., additional, Coats, C., additional, Cardona, M., additional, Garcia, A., additional, Calcagnino, M., additional, Lachmann, R., additional, Hughes, D., additional, Elliott, P. M., additional, Conti, S., additional, Pruiti, G. P., additional, Puzzangara, E., additional, Romano, S. A., additional, Di Grazia, A., additional, Ussia, G. P., additional, Tamburino, C., additional, Calvi, V., additional, Radinovic, A., additional, Sala, S., additional, Latib, A., additional, Mussardo, M., additional, Sora, S., additional, Paglino, G., additional, Gullace, M., additional, Colombo, A., additional, Ohlow, M.- A. G., additional, Lauer, B., additional, Wagner, A., additional, Schreiber, M., additional, Buchter, B., additional, Farah, A., additional, Fuhrmann, J. T., additional, Geller, J. C., additional, Nascimento Cardoso, R. M., additional, Batista Sa, L. A., additional, Campos Filho, L. F. C., additional, Rodrigues, S. V., additional, Dutra, M. V. F., additional, Borges, T. R. S. A., additional, Portilho, D. R., additional, Deering, T., additional, Bernardes, A., additional, Veiga, A., additional, Gartenlaub, O., additional, Goncalves, A., additional, Jimenez, A., additional, Rousseauplasse, A., additional, Deharo, J. C., additional, Striekwold, H., additional, Gosselin, G., additional, Sitbon, H., additional, Martins, V., additional, Molon, G., additional, Ayala-Paredes, F., additional, Sancho-Tello, M. J., additional, Fazal, I. A., additional, Brady, S., additional, Cronin, J., additional, Mcnally, S., additional, Tynan, M., additional, Plummer, C. J., additional, Mccomb, J. M., additional, Val-Mejias, J. E., additional, Oliveira, R. M., additional, Costa, R., additional, Martinelli Filho, M., additional, Silva, K. R., additional, Menezes, L. M., additional, Tamaki, W. T., additional, Mathias, W., additional, Stolf, N. A. G., additional, Misawa, T., additional, Ohta, I., additional, Shishido, T., additional, Miyasita, T., additional, Miyamoto, T., additional, Nitobe, J., additional, Watanabe, T., additional, Kubota, I., additional, Thibault, B., additional, Ducharme, A., additional, Simpson, C., additional, Stuglin, C., additional, Gagne, C. E., additional, Williams, R., additional, Mcnicoll, S., additional, Silvetti, M. S., additional, Drago, F., additional, Penela, D., additional, Bijnens, B., additional, Doltra, A., additional, Silva, E., additional, Berruezo, A., additional, Mont, L., additional, Sitges, M., additional, Mcintosh, R., additional, Baumann, O., additional, Raju, P., additional, Gurunathan, S., additional, Furniss, S., additional, Patel, N., additional, Sulke, N., additional, Lloyd, G., additional, Mor, M., additional, Dror, S., additional, Tsadok, Y., additional, Bachner-Hinenzon, N., additional, Katz, A., additional, Liel-Cohen, N., additional, Etzion, Y., additional, Mlynarski, R., additional, Mlynarska, A., additional, Wilczek, J., additional, Sosnowski, M., additional, Sinha, A. M., additional, Sinha, D., additional, Noelker, G., additional, Brachmann, J., additional, Weidemann, F., additional, Ertl, G., additional, Jones, M., additional, Searle, N., additional, Cocker, M., additional, Ilsley, E., additional, Foley, P., additional, Khiani, R., additional, Nelson, K. E., additional, Turley, A. J., additional, Owens, W. A., additional, James, S. A., additional, Linker, N. J., additional, Velagic, V., additional, Cikes, M., additional, Pezo Nikolic, B., additional, Puljevic, D., additional, Separovic-Hanzevacki, J., additional, Lovric-Bencic, M., additional, Biocina, B., additional, Milicic, D., additional, Kawata, H., additional, Chen, L., additional, Phan, H., additional, Anand, K., additional, Feld, G., additional, Birgesdotter-Green, U., additional, Fernandez Lozano, I., additional, Mitroi, C., additional, Toquero Ramos, J., additional, Castro Urda, V., additional, Monivas Palomero, V., additional, Corona Figueroa, A., additional, Hernandez Reina, L., additional, Alonso Pulpon, L., additional, Gate-Martinet, A., additional, Da Costa, A., additional, Rouffiange, P., additional, Cerisier, A., additional, Bisch, L., additional, Romeyer-Bouchard, C., additional, Isaaz, K., additional, Morales, M.- A., additional, Bianchini, E., additional, Startari, U., additional, Faita, F., additional, Bombardini, T., additional, Gemignani, V., additional, Piacenti, M., additional, Adhya, S., additional, Kamdar, R. H., additional, Millar, L. M., additional, Burchardt, C., additional, Murgatroyd, F. D., additional, Klug, D., additional, Kouakam, C., additional, Guedon-Moreau, L., additional, Marquie, C., additional, Benard, S., additional, Kacet, S., additional, Cortez-Dias, N., additional, Carrilho-Ferreira, P., additional, Silva, D., additional, Goncalves, S., additional, Valente, M., additional, Marques, P., additional, Carpinteiro, L., additional, Sousa, J., additional, Keida, T., additional, Nishikido, T., additional, Fujita, M., additional, Chinen, T., additional, Kikuchi, T., additional, Nakamura, K., additional, Ohira, H., additional, Takami, M., additional, Anjo, D., additional, Meireles, A., additional, Gomes, C., additional, Roque, C., additional, Pinheiro Vieira, A., additional, Lagarto, V., additional, Reis, H., additional, Torres, S., additional, Ortega, D. F., additional, Barja, L. D., additional, Montes, J. P., additional, Logarzo, E., additional, Bonomini, P., additional, Mangani, N., additional, Paladino, C., additional, Chwyczko, T., additional, Smolis-Bak, E., additional, Sterlinski, M., additional, Pytkowski, M., additional, Firek, B., additional, Jankowska, A., additional, Szwed, H., additional, Nakajima, I., additional, Noda, T., additional, Okamura, H., additional, Satomi, K., additional, Aiba, T., additional, Shimizu, W., additional, Aihara, N., additional, Kamakura, S., additional, Brzozowski, W., additional, Tomaszewski, A., additional, Wysokinski, A., additional, Bertoldi, E. G., additional, Rohde, L. E., additional, Zimerman, L. I., additional, Pimentel, M., additional, Polanczyk, C. A., additional, Boriani, G., additional, Lunati, M., additional, Gasparini, M., additional, Landolina, M., additional, Lonardi, G., additional, Pecora, D., additional, Santini, M., additional, Valsecchi, S., additional, Rubinstein, B. J., additional, Wang, D. Y., additional, Cabreriza, S. E., additional, Richmond, M. E., additional, Rusanov, A., additional, Quinn, T. A., additional, Cheng, B., additional, Spotnitz, H. M., additional, Kristiansen, H. M., additional, Vollan, G., additional, Hovstad, T., additional, Keilegavlen, H., additional, Faerestrand, S., additional, Brigesdotter-Green, U., additional, Nawar, A. M. R., additional, Ragab, D. A. L. I. A., additional, Eluhsseiny, R. A. N. I. A., additional, Abdelaziz, A. H. M. E. D., additional, Nof, E., additional, Abu Shama, R., additional, Buber, J., additional, Kuperstein, R., additional, Feinberg, M. S., additional, Barlev, D., additional, Eldar, M., additional, Glikson, M., additional, Badran, H., additional, Samir, R., additional, Tawfik, M., additional, Amin, M., additional, Eldamnhoury, H., additional, Khaled, S., additional, Tolosana, J. M., additional, Martin, A. M., additional, Hernandez-Madrid, A., additional, Macias, A., additional, Fernandez-Lozano, I., additional, Osca, J., additional, Quesada, A., additional, Padeletti, L., additional, Botto, G. L., additional, De Santo, T., additional, Szwed, A., additional, Martinez, J. G., additional, Degand, B., additional, Villani, G. Q., additional, Leclercq, C., additional, Ritter, P., additional, Watanabe, I., additional, Nagashima, K., additional, Okumura, Y., additional, Kofune, M., additional, Ohkubo, K., additional, Nakai, T., additional, Hirayama, A., additional, Mikhaylov, E., additional, Vander, M., additional, Lebedev, D., additional, Zarse, M., additional, Suleimann, H., additional, Bogossian, H., additional, Stegelmeyer, J., additional, Ninios, I., additional, Karosienne, Z., additional, Kloppe, A., additional, Lemke, B., additional, John, S., additional, Gaspar, T., additional, Rolf, S., additional, Sommer, P., additional, Hindricks, G., additional, Piorkowski, C., additional, Fernandez-Armenta, J., additional, Mont, L. L., additional, Zeljko, H., additional, Andreu, D., additional, Herzcku, C., additional, Boussy, T., additional, Brugada, J., additional, Obayahi, T., additional, Hegrenes, J., additional, Lim, E., additional, Mediratta, V., additional, Bautista, R., additional, Teplitsky, L., additional, Van Huls Van Taxis, C. F. B., additional, Wijnmaalen, A. P., additional, Gawrysiak, M., additional, Schuijf, J. D., additional, Bax, J. J., additional, Huo, Y., additional, Richter, S., additional, Arya, A., additional, Bollmann, A., additional, Akca, F., additional, Bauernfeind, T., additional, Schwagten, B., additional, De Groot, N. M. S., additional, Jordaens, L., additional, Szili-Torok, T., additional, Miller, S., additional, Kastner, G., additional, Maury, P., additional, Della Bella, P., additional, Delacretaz, E., additional, Sacher, F., additional, Maccabelli, G., additional, Brenner, R., additional, Rollin, A., additional, Jais, P., additional, Vergara, P., additional, Trevisi, N., additional, Ricco, A., additional, Petracca, F., additional, Bisceglia, C., additional, Baratto, F., additional, Salguero Bodes, R., additional, Fontenla Cerezuela, A., additional, De Riva Silva, M., additional, Lopez Gil, M., additional, Mejia Martinez, E., additional, Jurado Roman, A., additional, Montero Alvarez, M., additional, Arribas Ynsaurriaga, F., additional, Baszko, A., additional, Krzyzanowski, K., additional, Bobkowski, W., additional, Surmacz, R., additional, Zinka, E., additional, Siwinska, A., additional, Szyszka, A., additional, Perez Silva, A., additional, Estrada Mucci, A., additional, Ortega Molina, M., additional, Lopez Sendon, J. L., additional, Merino Llorens, J. L., additional, Kaitani, K., additional, Hanazawa, K., additional, Izumi, C., additional, Nakagawa, Y., additional, Yamanaka, I., additional, Hirahara, T., additional, Sugawara, Y., additional, Suga, C., additional, Ako, J., additional, Momomura, S., additional, Galizio, N., additional, Gonzalez, J., additional, Robles, F., additional, Palazzo, A., additional, Favaloro, L., additional, Diez, M., additional, Guevara, E., additional, Fernandez, A., additional, Greenberg, S., additional, Epstein, A., additional, Goldman, D. S., additional, Sangli, C., additional, Keeney, J. A., additional, Lee, K., additional, Piers, S. R. D., additional, Van Rees, J. B., additional, Thijssen, J., additional, Borleffs, C. J. W., additional, Van Der Velde, E. T., additional, Leclercq, C. H., additional, Hero, M., additional, Mizobuchi, M., additional, Enjoji, Y., additional, Yazaki, Y., additional, Shibata, K., additional, Funatsu, A., additional, Kobayashi, T., additional, Nakamura, S., additional, Amit, G., additional, Pertzov, B., additional, Zahger, D., additional, Medesani, L., additional, Rana, R., additional, Albano, F., additional, Fraguas, H., additional, Pedersen, S. S., additional, Hoogwegt, M. T., additional, Theuns, D. A. M. J., additional, Van Den Broek, K. C., additional, Tekle, F. B., additional, Habibovic, M., additional, Alings, M., additional, Van Der Voort, P., additional, Denollet, J., additional, Vrazic, H., additional, Jilek, C., additional, Lesevic, H., additional, Tzeis, S., additional, Semmler, V., additional, Gold, M. R., additional, Burke, M. C., additional, Bardy, G. H., additional, Varma, N., additional, Pavri, B., additional, Stambler, B., additional, Michalski, J., additional, Investigators, T. R. U. S. T., additional, Safak, E., additional, Schmitz, D., additional, Konorza, T., additional, Wende, C., additional, Schirdewan, A., additional, Neuzner, J., additional, Simmers, T., additional, Erglis, A., additional, Gradaus, R., additional, Goetzke, J., additional, Coutrot, L., additional, Goehl, K., additional, Bazan Gelizo, V., additional, Grau, N., additional, Valles, E., additional, Felez, M., additional, Sanjuas, C., additional, Bruguera, J., additional, Marti-Almor, J., additional, Chu, S. Y., additional, Li, P. W., additional, Ding, W. H., additional, Schukro, C., additional, Leitner, L., additional, Siebermair, J., additional, Stix, G., additional, Pezawas, T., additional, Kastner, J., additional, Wolzt, M., additional, Schmidinger, H., additional, Behar, N. A. T. H. A. L. I. E., additional, Kervio, G., additional, Petit, B., additional, Maison-Balnche, P., additional, Bodi, S., additional, Mabo, P., additional, Foley, P. W. X., additional, Mutch, E., additional, Brashaw-Smith, J., additional, Ball, L., additional, Leyva, F., additional, Kim, D. H., additional, Lee, M. J., additional, Lee, W. S., additional, Park, S. D., additional, Shin, S. H., additional, Woo, S. I., additional, Kwan, J., additional, Park, K. S., additional, Munetsugu, Y., additional, Tanno, K., additional, Kikuchi, M., additional, Ito, H., additional, Miyoshi, F., additional, Kawamura, M., additional, Kobayashi, Y., additional, Man, S., additional, Algra, A. M., additional, Schreurs, C. A., additional, Van Der Wall, E. E., additional, Cannegieter, S. C., additional, Swenne, C. A., additional, Iitsuka, K., additional, Kondo, T., additional, Goebbert, K., additional, Karossiene, Z., additional, Goldman, D., additional, Kallen, B., additional, Kerpi, E., additional, Sardo, J., additional, Arsenos, P., additional, Gatzoulis, K., additional, Manis, G., additional, Dilaveris, P., additional, Tsiachris, D., additional, Mytas, D., additional, Asimakopoulos, S., additional, Stefanadis, C., additional, Sideris, S., additional, Kartsagoulis, E., additional, Barbosa, O., additional, Marocolo Junior, M., additional, Silva Cortes, R., additional, Moraes Brandolis, R. A., additional, Oliveira, L. F., additional, Pertili Rodrigues De Resende, L. A., additional, Vieira Da Silva, M. A., additional, Dias Da Silva, V. J., additional, Hegazy, R. A., additional, Sharaf, I. A., additional, Fadel, F., additional, Bazaraa, H., additional, Esam, R., additional, Deshko, M. S., additional, Snezhitsky, V. A., additional, Stempen, T. P., additional, Kuroki, K., additional, Igawa, M., additional, Kuga, K., additional, Ferreira Santos, L., additional, Dionisio, T., additional, Nunes, L., additional, Machado, J., additional, Castedo, S., additional, Henriques, C., additional, Matos, A., additional, Oliveira Santos, J., additional, Kraaier, K., additional, Olimulder, M. A. G. M., additional, Galjee, M. A., additional, Van Dessel, P. F. H. M., additional, Van Der Palen, J., additional, Wilde, A. A. M., additional, Scholten, M. F., additional, Chouchou, F., additional, Poupard, L., additional, Philippe, C., additional, Court-Fortune, I., additional, Barthelemy, J.- C., additional, Roche, F., additional, Dolgoshey, T. S., additional, Madekina, G. A., additional, Sugiura, S., additional, Fujii, E., additional, Senga, M., additional, Dohi, K., additional, Sugiura, E., additional, Nakamura, M., additional, Ito, M., additional, Eitel, C., additional, Mendell, J., additional, Lasseter, K., additional, Shi, M., additional, Urban, L., additional, Hatala, R., additional, Hlivak, P., additional, De Melis, M., additional, Garutti, C., additional, Corbucci, G., additional, Mlcochova, H., additional, Maxian, R., additional, Arbelo, E., additional, Dogac, A., additional, Luepkes, C., additional, Ploessnig, M., additional, Chronaki, C., additional, Hinterbuchner, L., additional, Guillen, A., additional, Bun, S. S., additional, Latcu, D. G., additional, Franceschi, F., additional, Prevot, S., additional, Koutbi, L., additional, Ricard, P., additional, Saoudi, N., additional, Nazari, N., additional, Alizadeh, A., additional, Sayah, S., additional, Hekmat, M., additional, Assadian, M., additional, Ahmadzadeh, A., additional, Wnuk, M., additional, Jedrzejczyk-Spaho, J., additional, Kruszelnicka, O., additional, Piwowarska, W., additional, Fedorowski, A., additional, Burri, P., additional, Juul-Moller, S., additional, Melander, O., additional, Mitro, P., additional, Murin, P., additional, Kirsch, P., additional, Habalova, V., additional, Slaba, E., additional, Matyasova, E., additional, Barlow, M. A., additional, Blake, R. J., additional, Rostoff, P., additional, Wojewodka Zak, E., additional, Froidevaux, L., additional, Sarasin, F. P., additional, Louis-Simonet, M., additional, Hugli, O., additional, Yersin, B., additional, Schlaepfer, J., additional, Mischler, C., additional, Pruvot, E., additional, Occhetta, E., additional, Frascarelli, F., additional, Burali, A., additional, and Dovellini, E., additional

Published
2011
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9. Nephropathic cystinosis in children: An overlooked disease

Author

Soliman Neveen, El-Baroudy Ramzi, Rizk Akmal, Bazaraa Hafez, and Younan Amal

Subjects
Corneal cystine crystal score, Cysteamine eye drops, Cystinosis, Diagnosis, Medicine
Abstract

Nephropathic cystinosis is rare genetic disease characterized by defective lysosomal cystine transport and increased lysosomal cystine. Corneal Cystine Crystal Scoring (CCCS) for diagnosis of nephropathic cystinosis was studied in all suspected children with renal Fanconi syn-drome and siblings of diagnosed cases over a two year period. In addition to oral cysteamine, cys-teamine eye drops were provided to all diagnosed patients and CCCS was followed up on a quarterly basis. Of 33 screened cases, 14 had corneal cystine crystals. Crystals were absent in two cystinotic patients under the age of 20 months. The mean age at diagnosis was 52.7 months and five patients had ERSD. After six months of treatment, the mean CCCS did not increase from the initial value of 1.81; associated with a decrease of 0.5 in two cases and a similar increase in two others. Scores decreased in two other patients after 12 months. Compliance was generally inadequate due to the high frequency of administration and the need for multi-drug regimen. CCCS is a simple and reasonably sensitive method for diagnosis of nephropathic cystinosis above two years of age. To-pical treatment with cysteamine eye drops prevents progression of deposits and may decrease it with adequate compliance. Further follow up is still recommended to monitor long term effects of both systemic and topical cysteamine therapy.

Published
2009

12. Genetic polymorphism of ACE and the angiotensin II type1 receptor genes in children with chronic kidney disease

Author

Elshamaa Manal F, Sabry Samar M, Bazaraa Hafez M, Koura Hala M, Elghoroury Eman A, Kantoush Nagwa A, Thabet Eman H, and Abd-El Haleem Dalia A

Subjects
angiotensin-converting enzyme, angiotensin II type one receptor, DNA polymorphisms, end-stage renal disease, Children, Therapeutics. Pharmacology, RM1-950
Abstract

Abstract Aim and Methods We investigated the association between polymorphisms of the angiotensin converting enzyme-1 (ACE-1) and angiotensin II type one receptor (AT1RA1166C) genes and the causation of renal disease in 76 advanced chronic kidney disease (CKD) pediatric patients undergoing maintenance hemodialysis (MHD) or conservative treatment (CT). Serum ACE activity and creatine kinase-MB fraction (CK-MB) were measured in all groups. Left ventricular mass index (LVMI) was calculated according to echocardiographic measurements. Seventy healthy controls were also genotyped. Results The differences of D allele and DI genotype of ACE were found significant between MHD group and the controls (p = 0.0001). ACE-activity and LVMI were higher in MHD, while CK-MB was higher in CT patients than in all other groups. The combined genotype DD v/s ID+II comparison validated that DD genotype was a high risk genotype for hypertension .~89% of the DD CKD patients were found hypertensive in comparison to ~ 61% of patients of non DD genotype(p = 0.02). The MHD group showed an increased frequency of the C allele and CC genotype of the AT1RA1166C polymorphism (P = 0.0001). On multiple linear regression analysis, C-allele was independently associated with hypertension (P = 0.04). Conclusion ACE DD and AT1R A/C genotypes implicated possible roles in the hypertensive state and in renal damage among children with ESRD. This result might be useful in planning therapeutic strategies for individual patients.

Published
2011
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13. Assessment of Tissue Perfusion Using the Peripheral Perfusion Index and Lactate Clearance in Shock in Pediatric Patients.

Author

Bazaraa H, Roby S, Salah E, and Algebaly H

Subjects
Child, Preschool, Female, Humans, Infant, Male, Predictive Value of Tests, Prospective Studies, Shock diagnosis, Lactic Acid metabolism, Perfusion Index, Shock metabolism, Shock mortality, Skin blood supply
Abstract

Background: Pediatric shock has a high mortality rate because many of the early clinical signs are subtle and have poor sensitivity and specificity. Pediatric shock was categorized either: compensated with normal blood pressure, poor skin perfusion (CRT >2 s, mottled, cool peripheries, peripheral cyanosis), weak peripheral pulse, age specific tachycardia, tachypnoea, and oliguria or decompensated with hypotension (SBP < 70 + (2× age in years) mm Hg and decreased mental status. The perfusion index is a non-invasive method for assessing peripheral perfusion and may be a useful marker for identifying shock early in pediatric patients., Objective: This prospective cohort study (November 2019 to August 2020) evaluated whether the perfusion index, lactate, and/or lactate clearance could predict mortality among pediatric shock patients., Methods: Fifty children (68% male) with shock underwent assessments at presentation to the emergency room to evaluate their heart rate, blood pressure, capillary refill time, central venous pressure, perfusion index, cardiac index, systemic vascular resistance, central venous oxygen saturation, and lactate clearance., Results: The perfusion index range was 0.03 to 2.2 and ≤0.18 as the cut-off for mortality prediction providing 74% sensitivity and 78% specificity. The serum lactate concentration range was 0 to 16 mmol/L and >5.7 mmol/L as the cut-off for mortality prediction provided 70% sensitivity and 96% specificity at presentation to the emergency room. The lactate clearance range was 3% to 75% and >10% as the cut-off for survival prediction after resuscitation and at 6 h later., Conclusion: Perfusion index (PI), lactate, and lactate clearance provided comparable sensitivity and specificity for predicting outcomes among pediatric patients with shock Therefore, we suggest that the PI is an inexpensive, rapid, and non-invasive tool that can be used to predict illness severity and mortality in busy pediatric intensive care units and emergency departments. This tool may guide better patient triage and an earlier diagnosis of shock in this setting., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by the Shock Society.)

Published
2021
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14. Prognostic value of baseline carotid blood flow in critically ill children with septic shock.

Author

Mamdouh F, Bazaraa H, Baz A, and Algebaly HF

Abstract

Background and Aim: Hemodynamic monitoring and cardiac output (CO) assessment in the ICU have been trending toward less invasive methods. Carotid blood flow (CBF) was suggested as a candidate for CO assessment. The present study aimed to test the value of carotid artery ultrasound analysis in prediction of mortality in pediatric patients with septic shock., Methodology/principal Finding: Forty children with septic shock were included in the study. Upon admission, patients were subjected to careful history taking and thorough clinical examination. The consciousness level was assessed by the Glasgow Coma Scale (GCS). Laboratory assessment included complete blood count, C-reactive protein, arterial blood gases, serum electrolytes, and liver and kidney function tests. Electrical cardiometry was used to evaluate hemodynamic parameters. Patients were also subjected to transthoracic 2-D echocardiography. CBF was evaluated using GE Vivid S5 ultrasound device through dedicated software. At the end of study, 14 patients (35.0%) died. It was found that survivors had significantly higher CBF when compared non-survivors [median (IQR): 166.0 (150.0-187.3) versus 141.0 (112.8-174.3), p = 0.033]. In addition, it was noted that survivors had longer ICU stay when compared with non-survivors [16.5 (9.8-31.5) versus 6.5 (3.0-19.5) days, p = 0.005]. ROC curve analysis showed that CBF could significantly distinguish survivors from non-survivors [AUC (95% CI): 0.3 (0.11-0.48), p = 0.035] (Fig 2). Univariate logistic regression analysis identified type of shock [OR (95% CI): 28.1 (4.9-162.4), p<0.001], CI [OR (95% CI): 0.6 (0.43-0.84), p = 0.003] and CBF [OR (95% CI): 0.98 (0.96-0.99), p = 0.031]. However, in multivariate analysis, only type of shock significantly predicted mortality., Conclusions: CBF assessment may be a useful prognostic marker in children with septic shock., Competing Interests: The authors have declared that no competing interests exist.

Published
2021
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15. Does renal transplant in children with LUTD improve their bladder function?

Author

Aboulela W, Fawzy AM, Abdelmawla MA, Salah DM, Eldin MS, Mohamed Anwar AZ, El Ghoneimy M, Shouman AM, Shoukry AI, Bazaraa H, Tawfiek ER, Fadel F, Badawy H, Morsi HA, and Ghoneima W

Subjects
Adolescent, Adult, Allografts, Child, Child, Preschool, Creatinine blood, Female, Graft Survival, Humans, Kidney Failure, Chronic complications, Living Donors, Male, Pediatrics, Proportional Hazards Models, Renal Dialysis, Retrospective Studies, Treatment Outcome, Urodynamics, Urologic Diseases complications, Kidney Failure, Chronic surgery, Kidney Transplantation methods, Urinary Bladder physiopathology, Urologic Diseases physiopathology
Abstract

Much is still unknown about LUT function after receiving renal graft. Graft function was the main focus of different studies discussing the same issue. However, these studies ignored the effects of the graft on lower tract function and more demand for bladder cycling and growth of the child. Therefore, we aimed at evaluating the LUT function after RT into patients with LUTD. We enrolled a retrospective cohort of 83 live renal transplant children with LUTD. The 44 patients in Group (A) had a defunctionalized bladder, and the 39 patients in Group (B) had underlying LUT pathology. All patients had clinical and urodynamic evaluation of LUT functions at least 1 year after RT. We found that the improvement in patients with impaired bladder compliance was 73% in Group (A) and 60% in Group (B), with no statistically significant difference between the study groups. In Group (B), there was statistically significant worsening of MFP (8.4%) and mean PVR (79.9%) after RT. In Group (A), mild but stable significant improvement of all clinical and urodynamic parameters was observed. Serum creatinine was significantly worse in patients with pathological LUTD compared with those with defunctionalized bladder but without significant effect on graft survival. All LUT variables seemed to have no adverse effect on graft survival except for use of CIC and augmented bladder. Incident UTI independent of LUT variables accounted for 20% of graft creatinine change., (© 2020 Wiley Periodicals LLC.)

Published
2020
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16. Combined liver-kidney transplantation for primary hyperoxaluria type I in children: Single Center Experience.

Author

Kotb MA, Hamza AF, Abd El Kader H, El Monayeri M, Mosallam DS, Ali N, Basanti CWS, Bazaraa H, Abdelrahman H, Nabhan MM, Abd El Baky H, El Sorogy STM, Kamel IEM, Ismail H, Ramadan Y, Abd El Rahman SM, and Soliman NA

Subjects
Child, Child, Preschool, Female, Follow-Up Studies, Graft Survival, Humans, Hyperoxaluria, Primary mortality, Male, Retrospective Studies, Survival Rate, Treatment Outcome, Hyperoxaluria, Primary surgery, Kidney Transplantation methods, Liver Transplantation methods
Abstract

Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to surgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwent simultaneous CLKT, only two survived and are well, one with insignificant complications, and other suffered from abdominal Burkitt lymphoma managed by excision and resection anastomosis, four cycles of rituximab, cyclophosphamide, vincristine, and prednisone. The other two died, one due to uncontrollable bleeding within 36 hours of procedure, while the other died awaiting renal transplant after loss of renal graft to recurrent renal oxalosis 6 months post-transplant. PH1 with ESKD is a rare disease; simultaneous CLKT offers good quality of life for afflicted children. Graft shortage and renal graft loss to oxalosis challenge the outcome., (© 2018 Wiley Periodicals, Inc.)

Published
2019
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17. Risk factors for urological complications following living donor renal transplantation in children.

Author

ElSheemy MS, Ghoneima W, Aboulela W, Daw K, Shouman AM, Shoukry AI, Soaida S, Salah DM, Bazaraa H, Fadel FI, Hussein AA, Habib E, Saad IR, El Ghoneimy M, Morsi HA, Lotfi MA, and Badawy H

Subjects
Adolescent, Adult, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Logistic Models, Male, Middle Aged, Postoperative Complications epidemiology, Retrospective Studies, Risk Factors, Urologic Diseases epidemiology, Kidney Transplantation methods, Living Donors, Postoperative Complications etiology, Urologic Diseases etiology
Abstract

The aim of this study was to detect possible risk factors for UC and UTI following pediatric renal Tx and effect of these complications on outcome. One hundred and eight children who underwent living donor Tx between 2009 and 2015 were retrospectively included. Extraperitoneal approach was used with stented tunneled extravesical procedure. Mean recipient age was 9.89 ± 3.46 years while mean weight was 25.22 ± 10.43 kg. Seventy-three (67.6%) recipients were boys while 92 (85.2%) were related to donors. Urological causes of ESRD were present in 33 (30.6%) recipients (14 [13%] posterior urethral valve, 16 [14.8%] VUR, and 3 [2.8%] neurogenic bladder). Augmentation ileocystoplasty was performed in 9 (8.3%) patients. Mean follow-up was 39.3 ± 17.33 months. UC were detected in 10 (9.3%) children (leakage 4 [3.7%], obstruction 3 [2.8%], and VUR 3 [2.8%]) while UTIs were reported in 40 (37%) children. After logistic regression analysis, UC were significantly higher in children with cystoplasty (44.4% vs 6.1%; P = .001). UTIs were significantly higher in girls (51.4% vs 30.1%; P = .001) and in children with urological causes of ESRD (51.5% vs 30.7%; P = .049). UC and UTI were not significantly associated with increased graft loss or mortality. UC were significantly higher in children with cystoplasty while UTIs were significantly higher in girls and children with urological causes of ESRD. Presence of UC did not affect the rate of graft loss or mortality due to its early detection and proper management., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Published
2018
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18. Outcomes of living donor renal transplantation in children with lower urinary tract dysfunction: a comparative retrospective study.

Author

Saad IR, Habib E, ElSheemy MS, Abdel-Hakim M, Sheba M, Mosleh A, Salah DM, Bazaraa H, Fadel FI, Morsi HA, and Badawy H

Subjects
Adolescent, Child, Child, Preschool, Female, Humans, Kidney Failure, Chronic etiology, Living Donors, Male, Retrospective Studies, Urethral Diseases complications, Urinary Bladder Diseases complications, Kidney Failure, Chronic surgery, Kidney Transplantation
Abstract

Objectives: To compare outcomes of renal transplantation (RTx) in children with end-stage renal disease (ESRD) resulting from lower urinary tract dysfunction (LUTD) vs other causes., Patients and Methods: A database of children (<18 years old) who underwent RTx between May 2008 and April 2012 was reviewed. Patients were divided into those with LUTD (group A, n = 29) and those with other causes of ESRD (group B, n = 74). RTx was performed after achieving low intravesical pressure (<30 cmH2 O) with adequate bladder capacity and drainage. The groups were compared using Student's t-test, Mann-Whitney, chi-squared or exact tests. Graft survival rates (GSRs) were evaluated using Kaplan-Meier curves and the log-rank test., Results: The mean ± sd (range) age of the study cohort was 5.05 ± 12.4 (2.2-18) years. Causes of LUTD were posterior urethral valve (PUV; 41.4%), vesico-ureteric reflux (VUR; 37.9%), neurogenic bladder (10.3%), prune belly syndrome (3.4%), obstructive megaureter (3.4%) and urethral stricture disease (3.4%). There was no significant difference in age, dialysis duration or donor type. In group A, 25 of the 29 patients (86.2%) underwent ≥1 surgery to optimize the urinary tract for allograft. Pretransplant nephrectomy was performed in 15 of the 29 patients (51.7%), PUV ablation in nine patients (31%) and ileocystoplasty in four patients (13.7%). The mean ± sd follow-up was 4.52 ± 1.55 and 4.07 ± 1.27 years in groups A and B, respectively. There was no significant difference in creatinine and eGFR between the groups at different points of follow-up. The GSRs at the end of the study were 93.1 and 91.1% in groups A and B, respectively (P = 1.00). According to Kaplan-Meier survival curves, there was no significant difference in the GSR between the groups using the log-rank test (P = 0.503). No graft was lost as a result of urological complications. In group B, one child died from septicaemia. The rate of urinary tract infections was 24 and 12% in groups A and B, respectively, but was not significant. No significant difference was found between the groups with regard to the incidence of post-transplantation hydronephrosis. Of the 22 patients who had hydronephrosis after transplantation, three were complicated by UTI. Injection of bulking agents was required in two patients for treatment of grade 3 VUR. In the third patient, augmentation cystoplasty was needed., Conclusion: Acceptable graft function, survival and UTI rates can be achieved in children with ESRD attributable to LUTD. Thorough assessment and optimization of LUT, together with close follow-up, are key for successful RTx., (© 2015 The Authors BJU International © 2015 BJU International Published by John Wiley & Sons Ltd.)

Published
2016
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19. Metabolic encephalopathy in Egyptian children.

Author

Hindawy A, Gouda A, El-Ayyadi A, Megahed H, and Bazaraa H

Subjects
Child, Preschool, Egypt, Female, Humans, Infant, Male, Brain Diseases, Metabolic diagnosis, Fatty Acids metabolism, Lipid Metabolism, Inborn Errors diagnosis
Abstract

Fatty Acid Oxidation disorders represent an expanding group of inborn errors of metabolism. Clinical manifestations include episodic encephalopathy, hypoketotic hypoglycemia, Reye like episodes, hepatic, muscular, cardiac affection and sudden death. Analysis of urinary organic acids and plasma fatty acids of 44 clinically suspected patients by Gas Chromatography Mass spectrometry revealed 4 cases of Medium chain acyl-CoA dehydrogenase deficiency (MCADD), 3 cases of Very long chain acyl-CoA dehydrogenase deficiency, 9 cases of multiple defects of acyl-CoA dehydrogenation in addition to 3 patients with other metabolic disorders. Timely detection of these disorders including screening for MCADD can have a favorable impact on the outcome of these patients (Tab. 11, Fig. 3, Ref. 24) Full Text (Free, PDF).

Published
2007

20. Electrophoretic mobility shift assay identifies vitamin D binding protein (Gc-globulin) in human, rat, and mouse sera.

Author

Tang WX, Bazaraa HM, Magiera H, Cooke NE, and Haddad JG

Subjects
Actins isolation & purification, Animals, Evaluation Studies as Topic, Humans, Mice, Mice, Inbred C57BL, Rabbits, Rats, Rats, Sprague-Dawley, Staining and Labeling, Vitamin D-Binding Protein blood, Vitamin D-Binding Protein isolation & purification, Electrophoresis, Polyacrylamide Gel methods, Vitamin D-Binding Protein analysis
Abstract

Serum vitamin D binding protein (DBP, also known as Gc-globulin) is a multifunctional protein capable of binding both vitamin D metabolites and actin. DBP can be visualized when analyzed by polyacrylamide gel electrophoresis followed by staining. Confirmation of its identity had previously required immunoprecipitation with specific anti-DBP antisera or occupancy of the protein with radioactive vitamin D sterols. We present studies showing that preincubation of G-actin with mammalian sera produced a discernible DBP protein band shift on native gel electrophoresis. Addition of DNaseI, a 33-kDa intracellular protein with an avid actin-binding site, to the incubations resulted in a supershift of DBP-actin complexes to an even more cathodal region of the gels. Following incubations with human, rat, and murine sera the same actin shift occurred as did the actin plus DNaseI supershift. The migrations of each complex were correlated with purified DBP migrations under identical conditions. It was confirmed that the supershifted bands contained DBP by Western blotting and detection of DBP by binding of 25-OH[3H]D3. After intravenous G-actin injections into living mice, a serum DBP-actin complex could be detected on native gels as the uncomplexed DBP band decreased in intensity. This simple, direct-staining technique appears to be suitable for identifying DBP/Gc phenotypes in human populations as well as for semiquantitatively monitoring the plasma actin-scavenger system in vivo in animal models or in human diseases.

Published
1996
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