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001-es BibID:
BIBFORM106202
035-os BibID:
(WOS)000906505300001 (Scopus)85145501296 (Pubmed)36605210
Első szerző:
Abolhassani, Hassan
Cím:
Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021 / Hassan Abolhassani, Tadej Avcin, Nerin Bahceciler, Dmitry Balashov, Zsuzsanna Bata, Mihaela Bataneant, Mikhail Belevtsev, Ewa Bernatowska, Judit Bidlo, Péter Blazsó, Bertrand Boisson, Mikhail Bolkov, Anastasia Bondarenko, Oksana Boyarchuk, Anna Bundschu, Jean-Laurent Casanova, Liudmyla Chernishova, Peter Ciznar, Ildikó Csürke, Melinda Erdős, Henriette Farkas, Daria S. Fomina, Nermeen Galal, Vera Goda, Sukru Nail Guner, Péter Hauser, Natalya I. Ilyina, Teona Iremadze, Sevan Iritsyan, Vlora Ismaili-Jaha, Milos Jesenak, Jadranka Kelecic, Sevgi Keles, Gerhard Kindle, Irina V. Kondratenko, Larysa Kostyuchenko, Elena Kovzel, Gergely Kriván, Georgina Kuli-Lito, Gábor Kumánovics, Natalja Kurjane, Elena A. Latysheva, Tatiana V. Latysheva, István Lázár, Gasper Markelj, Maja Markovic, László Maródi, Vafa Mammadova, Márta Medvecz, Noémi Miltner, Kristina Mironska, Fred Modell, Vicki Modell, Bernadett Mosdósi, Anna A. Mukhina, Marianna Murdjeva, Györgyi Műzes, Umida Nabieva, Gulnara Nasrullayeva, Elissaveta Naumova, Kálmán Nagy, Beáta Onozó, Bubusaira Orozbekova, Malgorzata Pac, Karaman Pagava, Alexander N. Pampura, Srdjan Pasic, Mery Petrosyan, Gordana Petrovic, Lidija Pocek, Andrei P. Prodeus, Ismail Reisli, Krista Ress, Nima Rezaei, Yulia A. Rodina, Alexander G. Rumyantsev, Svetlana Sciuca, Anna Sediva, Margit Serban, Svetlana Sharapova, Anna Shcherbina, Brigitta Sitkaustiene, Irina Snimshchikova, Shqipe Spahiu-Konjusha, Miklós Szolnoky, Gabriella Szűcs, Natasa Toplak, Beáta Tóth, Galina Tsyvkina, Irina Tuzankina, Elena Vlasova, Alla Volokha
Dátum:
2022
ISSN:
1664-3224
Megjegyzések:
The J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI. In this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients' data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively.We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).
Tárgyszavak:
Orvostudományok
Klinikai orvostudományok
idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:
Frontiers in Immunology. - 13 : 13 (2022), p. 1-14. -
További szerzők:
Avcin, Tadej
Bahceciler, Nerin
Balashov, Dmitry
Bata Zsuzsanna
Bataneant, Michaela
Belevtsev, Michael
Bernatowska, Ewa
Bidló Judit
Blazsó Péter
Boisson, Bertrand
Bolkov, Mikhail
Bondarenko, Anastasia
Boyarchuk, Oksana
Bundschu, Anna
Casanova, Jean-Laurent
Chernishova, Liudmyla
Ciznar, Peter
Csürke Ildikó
Erdős Melinda (1975-) (infektológus, gyermekimmunológus)
Farkas Henriette
Fomina, Daria S.
Galal, Nermeen
Goda Vera
Guner, Sukru Nail
Hauser Péter
Ilyina, Natalya I.
Iremadze, Teona
Iritsyan, Sevan
Ismaili-Jaha, Vlora
Jesenak, Milos
Kelecic, Jadranka
Keles, Sevgi
Kindle, Gerhard
Kondratenko, Irina V.
Kostyuchenko, Larysa
Kovzel, Elena
Kriván Gergely
Kuli-Lito, Georgina
Kumánovics Gábor
Kurjane, Natalja
Latysheva, Elena A.
Latysheva, Tatiana V.
Lázár István (1986-) (geográfus)
Markelj, Gasper
Markovic, Maja
Maródi László (1949-) (gyermekgyógyász infektológus, immunológus)
Mammadova, Vafa
Medvecz Márta
Miltner Noémi (1990-) (molekuláris biológus)
Mironska, Kristina
Modell, Fred
Modell, Vicki
Mosdósi Bernadett
Mukhina, Anna A.
Murdjeva, Marianna
Műzes Györgyi
Nabieva, Umida
Nasrullayeva, Gulnara
Naumova, Elissaveta
Nagy Kálmán
Ónozó Beáta
Orozbekova, Bubusaira
Pac, Malgorzata
Pagava, Karaman
Pampura, Alexander N.
Pasic, Srdjan
Petrosyan, Mery
Petrovic, Gordana
Pocek, Lidija
Prodeus, Andrei P.
Reisli, Ismail
Ress, Krista
Rezaei, Nima
Rodina, Yulia A.
Rumyantsev, Alexander G.
Sciuca, Svetlana
Sediva, Anna
Serban, Margit
Sharapova, Svetlana
Shcherbina, Anna
Sitkaustiene, Brigita
Snimshchikova, Irina
Spahiu-Konjusha, Shqipe
Szolnoky Miklós
Szűcs Gabriella (1963-) (belgyógyász, allergológus és klinikai immunológus, reumatológus)
Toplak, Nataŝa
Lajszné Tóth Beáta (1978-) (molekuláris biológus)
Tsyvkina, Galina
Tuzankina, Irina
Vlasova, Elena
Volokha, Alla
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