137 results on '"Durmaz, Özlem"'
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2. Kroni̇k Karaci̇ğer Hastalığı Olan Çocuğun İzlemi̇ Ve Toplumda Bakımı
- Author
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Önal, Zerrin, primary and Durmaz, Özlem, additional
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- 2024
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3. The role of partial splenic artery embolization in the management of refractory esophageal variceal bleeding due to portal vein thrombosis.
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Dinçer, Büşra Tetik, Urgancı, Nafiye, Bayrak, Aylin Hasanefendioğlu, Durmaz, Özlem, and Özden, İlgin
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LEUKOCYTE count ,SPLENIC artery ,MEDICAL sciences ,UMBILICAL veins ,ESOPHAGEAL varices - Abstract
Background: Gastro-esophageal variceal hemorrhage (GEVH) is one of the major causes of life-threatening gastrointestinal bleeding in children. Medical, endoscopic, angiographic, and surgical interventions can be utilized in treatment. In this case report, we describe partial splenic artery embolization for refractory GEVH due to portal vein thrombosis. Case presentation: A 3-year-old male patient was admitted with abdominal distension. He had been followed up for hepatosplenomegaly for 2 years. The patient's body weight was 15.5 kg (50-75th percentile, 0.69 SDS) and height was 96 cm (50-75th percentile, 0.27 SDS). The general condition was moderate, and the skin appeared pale. The liver was palpable 2 cm, and the spleen was palpable 6 cm below the costal margin. Other system examinations were normal. Laboratory findings included hemoglobin (Hgb) of 7.1 g/dL, hematocrit (Hct) of 24%, white blood cell count of 9800/mm3, platelets of 67000/mm3, and INR of 1.3. Abdominal CT angiography demonstrated a hypodense thrombus at the portal confluence, almost completely occluding the lumen. Endoscopy revealed esophageal varices at the 2 and 7 o'clock positions in the distal esophagus, which became more prominent with insufflation and had red spots on them. Hyperemia was observed in the corpus and antrum of the stomach. Findings were consistent with stage 3 esophageal varices and increased vascularity in the duodenum due to portal vein thrombosis. Medical treatment with a proton pump inhibitor and beta-blocker was initiated. The patient underwent endoscopic band ligation (EBL) three times over 9 months. Despite EBL, the patient presented with GEVH three times during a 1.5-year follow-up. Due to newly developed multiple varices observed on control endoscopy a decision was made to perform splenic artery embolization. Interventional radiology performed selective lower splenic pole embolization. Six months later, the patient underwent another selective embolization. The patient has been followed up for 3 years without GEVH. Conclusions: In this case, splenic artery embolization was observed to be an effective, repeatable, and safe treatment method for patients with hypersplenism caused by portal hypertension and refractory esophageal variceal bleeding. [ABSTRACT FROM AUTHOR]
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- 2025
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4. Complementary Roles of Cadaveric and Living Donor Liver Transplantation in Acute Liver Failure
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Özden, İlgin, Yavru, Hacer Aysen, Durmaz, Özlem, Orhun, Günseli, Salmaslıoğlu, Artür, Güllüoğlu, Mine, Alper, Aydın, İbiş, Cem, Serin, Kürşat Rahmi, Önal, Zerrin, Özcan, Perihan Ergin, Poyanlı, Arzu, Hançerli, Selda, Çağatay, Atahan, Cantez, Serdar, and Kaymakoğlu, Sabahattin
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- 2021
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5. The Consequences of HLA Screening in the Prevention of Graft‐Versus‐Host Disease in Living Donor Liver Transplantation.
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Ercan, Leman Damla, Durmaz, Özlem, Kaymakoğlu, Sabahattin, Önal, Zerrin, Büyükbabani, Nesimi, Güllüoğlu, Mine, Alper, Aydın, İbiş, Cem, Cantez, Serdar, Yavru, Hacer Ayşen, Oğuz, Fatma Savran, and Özden, İlgin
- Subjects
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LIVER transplantation , *IMMUNE recognition , *MEDICAL screening , *LIVER diseases , *MYCOPHENOLIC acid - Abstract
Aims: To study the effects of routine HLA screening and the policy of avoiding donor‐dominant one‐way HLA match to prevent graft‐versus‐host disease (GVHD) after living donor liver transplantation (LDLT). Patients and Methods: The records of potential living liver donors and recipients who attended our center between 2007 and 2018 were reviewed retrospectively. Results: Of the 149 patients who underwent LDLT and survived longer than 3 months, two developed GVHD despite our strict policy. The first patient presented with grade II GVHD limited to the skin. She was treated successfully by briefly discontinuing immunosuppression and switching to everolimus. In the second case, the policy had been relaxed due to the availability of a single donor for ABO‐incompatible transplantation without any intervention to decrease anti‐A antibody levels (special case: A2 to O). Nevertheless, the patient presented with grade I GVHD limited to skin and was treated successfully by adding oral methylprednisolone to tacrolimus and mycophenolate mofetil. To the best of our information, this is the second reported case who recovered from GVHD after LDLT from a donor, homozygous at HLA A, B and DR and a recipient, heterozygous for all. Sixteen potential donors (1.2% of all candidates) of 14 recipients were disqualified solely on the basis of the HLA results; five of these patients died due to unavailability of another donor. Conclusion: The results support the policy of avoiding HLA combinations that preclude immune recognition of graft lymphocytes as foreign to decrease the risk of GVHD after LDLT. [ABSTRACT FROM AUTHOR]
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- 2024
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6. Efficacy and safety of odevixibat in patients with Alagille syndrome (ASSERT): a phase 3, double-blind, randomised, placebo-controlled trial
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Ovchinsky, Nadia, primary, Aumar, Madeleine, additional, Baker, Alastair, additional, Baumann, Ulrich, additional, Bufler, Philip, additional, Cananzi, Mara, additional, Czubkowski, Piotr, additional, Durmaz, Özlem, additional, Fischer, Ryan, additional, Indolfi, Giuseppe, additional, Karnsakul, Wikrom W, additional, Lacaille, Florence, additional, Lee, Way S, additional, Maggiore, Giuseppe, additional, Rosenthal, Philip, additional, Ruiz, Mathias, additional, Sokal, Etienne, additional, Sturm, Ekkehard, additional, van der Woerd, Wendy, additional, Verkade, Henkjan J, additional, Wehrman, Andrew, additional, Clemson, Christine, additional, Yu, Qifeng, additional, Ni, Quanhong, additional, Ruvido, Jessica, additional, Manganaro, Susan, additional, and Mattsson, Jan P, additional
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- 2024
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7. Efficacy and safety of odevixibat in patients with Alagille syndrome (ASSERT): a phase 3, double-blind, randomised, placebo-controlled trial
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MDL patientenzorg, Ovchinsky, Nadia, Aumar, Madeleine, Baker, Alastair, Baumann, Ulrich, Bufler, Philip, Cananzi, Mara, Czubkowski, Piotr, Durmaz, Özlem, Fischer, Ryan, Indolfi, Giuseppe, Karnsakul, Wikrom W., Lacaille, Florence, Lee, Way S., Maggiore, Giuseppe, Rosenthal, Philip, Ruiz, Mathias, Sokal, Etienne, Sturm, Ekkehard, van der Woerd, Wendy, Verkade, Henkjan J., Wehrman, Andrew, Clemson, Christine, Yu, Qifeng, Ni, Quanhong, Ruvido, Jessica, Manganaro, Susan, Mattsson, Jan P., MDL patientenzorg, Ovchinsky, Nadia, Aumar, Madeleine, Baker, Alastair, Baumann, Ulrich, Bufler, Philip, Cananzi, Mara, Czubkowski, Piotr, Durmaz, Özlem, Fischer, Ryan, Indolfi, Giuseppe, Karnsakul, Wikrom W., Lacaille, Florence, Lee, Way S., Maggiore, Giuseppe, Rosenthal, Philip, Ruiz, Mathias, Sokal, Etienne, Sturm, Ekkehard, van der Woerd, Wendy, Verkade, Henkjan J., Wehrman, Andrew, Clemson, Christine, Yu, Qifeng, Ni, Quanhong, Ruvido, Jessica, Manganaro, Susan, and Mattsson, Jan P.
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- 2024
8. Long-term outcomes of six patients after partial internal biliary diversion for progressive familial intrahepatic cholestasis
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Erginel, Basak, Soysal, Feryal Gun, Durmaz, Ozlem, Celik, Alaattin, and Salman, Tansu
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- 2018
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9. P1 Analysis of long-term treatment effects of odevixibat on clinical outcomes in children with progressive familial intrahepatic cholestasis in odevixibat clinical studies vs external controls from the NAPPED database
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Hansen, Bettina, primary, Valcheva, Velichka, additional, Yu, Qifeng, additional, Wessel, Daan BE van, additional, Thompson, Richard J, additional, Gonzales, Emmanuel, additional, Jankowska, Irena, additional, Sokal, Etienne, additional, Grammatikopoulos, Tassos, additional, Kadaristiana, Agustina, additional, Jacquemin, Emmanuel, additional, Spraul, Anne, additional, Lipiński, Patryk, additional, Czubkowski, Piotr, additional, Rock, Nathalie, additional, Shagrani, Mohammad, additional, Kumar, Kishwer, additional, Alkuraya, Fowzan, additional, Sateesh, Meddirevula, additional, Broering, Dieter, additional, Nicastro, Emanuele, additional, Kelly, Deirdre A, additional, Nebbia, Gabriella, additional, Arnell, Henrik, additional, Fischler, Björn, additional, Serranti, Daniele, additional, Arikan, Cigdem, additional, Polat, Esra, additional, Debray, Dominique, additional, Lacaille, Florence, additional, Goncalves, Cristina, additional, Hierro, Loreto, additional, Bartolo, Gema Muñoz, additional, Mozer-Glassberg, Yael, additional, Azaz, Amer, additional, Brecelj, Jernej, additional, Dezsőfi, Antal, additional, Calvo, Pier Luigi, additional, Grabhorn, Enke, additional, Sturm, Ekkehard, additional, Woerd, Wendy L van der, additional, Kamath, Binita M, additional, Wang, Jian-She, additional, Li, Liting, additional, Durmaz, Özlem, additional, Onal, Zerrin, additional, Felzen, Antonia, additional, Nomden, Mark, additional, Sonajalg, Jaak, additional, Stein, Philip, additional, Ni, Quanhong, additional, Clemson, Christine, additional, Mattsson, Jan P, additional, and Verkade, Henkjan J, additional
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- 2023
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10. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency
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UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Felzen, Antonia, van Wessel, Daan B.E., Gonzales, Emmanuel, Thompson, Richard J., Jankowska, Irena, Shneider, Benjamin L., Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad, Broering, Dieter, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, Jan B.F., Serranti, Daniele, Arikan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Goncalves, Cristina, Hierro, Loreto, Muñoz Bartolo, Gema, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Hartleif, Steffen, van der Woerd, Wendy J., Kamath, Binita M., Wang, Jian-She, Li, Liting, Durmaz, Özlem, Kerkar, Nanda, Jørgensen, Marianne Hørby, Fischer, Ryan, Jimenez-Rivera, Carolina, Alam, Seema, Cananzi, Mara, Laverdure, Noemie, Ferreira, Cristina Targa, Guerrero, Felipe Ordoñez, Wang, Heng, Sency, Valerie, Kim, Kyung Mo, Chen, Huey-Ling, de Carvalho, Elisa, Fabre, Alexandre, Bernabeu, Jesus Quintero, Zellos, Aglaia, Alonso, Estella M., Sokol, Ronald J., Suchy, Frederick J., Loomes, Kathleen M., McKiernan, Patrick J., Rosenthal, Philip, Turmelle, Yumirle, Horslen, Simon, Schwarz, Kathleen, Bezerra, Jorge A., Wang, Kasper, Hansen, Bettina E., Verkade, Henkjan J., UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Felzen, Antonia, van Wessel, Daan B.E., Gonzales, Emmanuel, Thompson, Richard J., Jankowska, Irena, Shneider, Benjamin L., Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad, Broering, Dieter, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, Jan B.F., Serranti, Daniele, Arikan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Goncalves, Cristina, Hierro, Loreto, Muñoz Bartolo, Gema, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Hartleif, Steffen, van der Woerd, Wendy J., Kamath, Binita M., Wang, Jian-She, Li, Liting, Durmaz, Özlem, Kerkar, Nanda, Jørgensen, Marianne Hørby, Fischer, Ryan, Jimenez-Rivera, Carolina, Alam, Seema, Cananzi, Mara, Laverdure, Noemie, Ferreira, Cristina Targa, Guerrero, Felipe Ordoñez, Wang, Heng, Sency, Valerie, Kim, Kyung Mo, Chen, Huey-Ling, de Carvalho, Elisa, Fabre, Alexandre, Bernabeu, Jesus Quintero, Zellos, Aglaia, Alonso, Estella M., Sokol, Ronald J., Suchy, Frederick J., Loomes, Kathleen M., McKiernan, Patrick J., Rosenthal, Philip, Turmelle, Yumirle, Horslen, Simon, Schwarz, Kathleen, Bezerra, Jorge A., Wang, Kasper, Hansen, Bettina E., and Verkade, Henkjan J.
- Abstract
Background & aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001). Conclusions: Individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment. Impact and implications: This manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mutation. Until
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- 2023
11. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency
- Author
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Felzen, Antonia, van Wessel, Daan B.E., Gonzales, Emmanuel, Thompson, Richard J., Jankowska, Irena, Shneider, Benjamin L., Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad, Broering, Dieter, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, Jan B.F., Serranti, Daniele, Arikan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Goncalves, Cristina, Hierro, Loreto, Muñoz Bartolo, Gema, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Hartleif, Steffen, van der Woerd, Wendy J., Kamath, Binita M., Wang, Jian She, Li, Liting, Durmaz, Özlem, Kerkar, Nanda, Jørgensen, Marianne Hørby, Fischer, Ryan, Jimenez-Rivera, Carolina, Alam, Seema, Cananzi, Mara, Laverdure, Noemie, Hansen, Bettina E., Felzen, Antonia, van Wessel, Daan B.E., Gonzales, Emmanuel, Thompson, Richard J., Jankowska, Irena, Shneider, Benjamin L., Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad, Broering, Dieter, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, Jan B.F., Serranti, Daniele, Arikan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Goncalves, Cristina, Hierro, Loreto, Muñoz Bartolo, Gema, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Hartleif, Steffen, van der Woerd, Wendy J., Kamath, Binita M., Wang, Jian She, Li, Liting, Durmaz, Özlem, Kerkar, Nanda, Jørgensen, Marianne Hørby, Fischer, Ryan, Jimenez-Rivera, Carolina, Alam, Seema, Cananzi, Mara, Laverdure, Noemie, and Hansen, Bettina E.
- Abstract
Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001). Conclusions: Individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment. Impact and implications: This manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mu
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- 2023
12. Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis
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MDL patientenzorg, Thompson, Richard J., Artan, Reha, Baumann, Ulrich, Calvo, Pier Luigi, Czubkowski, Piotr, Dalgic, Buket, D'Antiga, Lorenzo, Di Giorgio, Angelo, Durmaz, Özlem, Gonzalès, Emmanuel, Grammatikopoulos, Tassos, Gupte, Girish, Hardikar, Winita, Houwen, Roderick H.J., Kamath, Binita M., Karpen, Saul J., Lacaille, Florence, Lachaux, Alain, Lainka, Elke, Loomes, Kathleen M., Mack, Cara L., Mattsson, Jan P., McKiernan, Patrick, Ni, Quanhong, Özen, Hasan, Rajwal, Sanjay R., Roquelaure, Bertrand, Shteyer, Eyal, Sokal, Etienne, Sokol, Ronald J., Soufi, Nisreen, Sturm, Ekkehard, Tessier, Mary Elizabeth, van der Woerd, Wendy L., Verkade, Henkjan J., Vittorio, Jennifer M., Wallefors, Terese, Warholic, Natalie, Yu, Qifeng, Horn, Patrick, Kjems, Lise, MDL patientenzorg, Thompson, Richard J., Artan, Reha, Baumann, Ulrich, Calvo, Pier Luigi, Czubkowski, Piotr, Dalgic, Buket, D'Antiga, Lorenzo, Di Giorgio, Angelo, Durmaz, Özlem, Gonzalès, Emmanuel, Grammatikopoulos, Tassos, Gupte, Girish, Hardikar, Winita, Houwen, Roderick H.J., Kamath, Binita M., Karpen, Saul J., Lacaille, Florence, Lachaux, Alain, Lainka, Elke, Loomes, Kathleen M., Mack, Cara L., Mattsson, Jan P., McKiernan, Patrick, Ni, Quanhong, Özen, Hasan, Rajwal, Sanjay R., Roquelaure, Bertrand, Shteyer, Eyal, Sokal, Etienne, Sokol, Ronald J., Soufi, Nisreen, Sturm, Ekkehard, Tessier, Mary Elizabeth, van der Woerd, Wendy L., Verkade, Henkjan J., Vittorio, Jennifer M., Wallefors, Terese, Warholic, Natalie, Yu, Qifeng, Horn, Patrick, and Kjems, Lise
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- 2023
13. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency
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Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), Felzen, Antonia; van Wessel, Daan B.E.; Gonzales, Emmanuel; Thompson, Richard J.; Jankowska, Irena; Shneider, Benjamin L.; Sokal, Etienne; Grammatikopoulos, Tassos; Kadaristiana, Agustina; Jacquemin, Emmanuel; Spraul, Anne; Lipi?ski, Patryk; Czubkowski, Piotr; Rock, Nathalie; Shagrani, Mohammad; Broering, Dieter; Nicastro, Emanuele; Kelly, Deirdre; Nebbia, Gabriella; Arnell, Henrik; Fischler, Björn; Hulscher, Jan B.F.; Serranti, Daniele; Polat, Esra; Debray, Dominique; Lacaille, Florence; Goncalves, Cristina; Hierro, Loreto; Muñoz Bartolo, Gema; Mozer-Glassberg, Yael; Azaz, Amer; Brecelj, Jernej; Dezs?fi, Antal; Calvo, Pier Luigi; Grabhorn, Enke; Hartleif, Steffen; van der Woerd, Wendy J.; Kamath, Binita M.; Wang, Jian-She; Li, Liting; Durmaz, Özlem; Kerkar, Nanda; Jørgensen, Marianne Hørby; Fischer, Ryan; Jimenez-Rivera, Carolina; Alam, Seema; Cananzi, Mara; Laverdure, Noemie; Ferreira, Cristina Targa; Guerrero, Felipe Ordoñez; Wang, Heng; Sency, Valerie; Kim, Kyung Mo; Chen, Huey-Ling; de Carvalho, Elisa; Fabre, Alexandre; Bernabeu, Jesus Quintero; Zellos, Aglaia; Alonso, Estella M.; Sokol, Ronald J.; Suchy, Frederick J.; Loomes, Kathleen M.; McKiernan, Patrick J.; Rosenthal, Philip; Turmelle, Yumirle; Horslen, Simon; Schwarz, Kathleen; Bezerra, Jorge A.; Wang, Kasper; Hansen, Bettina E.; Verkade, Henkjan J., Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM), School of Medicine, Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), Felzen, Antonia; van Wessel, Daan B.E.; Gonzales, Emmanuel; Thompson, Richard J.; Jankowska, Irena; Shneider, Benjamin L.; Sokal, Etienne; Grammatikopoulos, Tassos; Kadaristiana, Agustina; Jacquemin, Emmanuel; Spraul, Anne; Lipi?ski, Patryk; Czubkowski, Piotr; Rock, Nathalie; Shagrani, Mohammad; Broering, Dieter; Nicastro, Emanuele; Kelly, Deirdre; Nebbia, Gabriella; Arnell, Henrik; Fischler, Björn; Hulscher, Jan B.F.; Serranti, Daniele; Polat, Esra; Debray, Dominique; Lacaille, Florence; Goncalves, Cristina; Hierro, Loreto; Muñoz Bartolo, Gema; Mozer-Glassberg, Yael; Azaz, Amer; Brecelj, Jernej; Dezs?fi, Antal; Calvo, Pier Luigi; Grabhorn, Enke; Hartleif, Steffen; van der Woerd, Wendy J.; Kamath, Binita M.; Wang, Jian-She; Li, Liting; Durmaz, Özlem; Kerkar, Nanda; Jørgensen, Marianne Hørby; Fischer, Ryan; Jimenez-Rivera, Carolina; Alam, Seema; Cananzi, Mara; Laverdure, Noemie; Ferreira, Cristina Targa; Guerrero, Felipe Ordoñez; Wang, Heng; Sency, Valerie; Kim, Kyung Mo; Chen, Huey-Ling; de Carvalho, Elisa; Fabre, Alexandre; Bernabeu, Jesus Quintero; Zellos, Aglaia; Alonso, Estella M.; Sokol, Ronald J.; Suchy, Frederick J.; Loomes, Kathleen M.; McKiernan, Patrick J.; Rosenthal, Philip; Turmelle, Yumirle; Horslen, Simon; Schwarz, Kathleen; Bezerra, Jorge A.; Wang, Kasper; Hansen, Bettina E.; Verkade, Henkjan J., Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM), and School of Medicine
- Abstract
Background & Aims: bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: from the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: the groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001). Conclusions: individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment. Impact and implications: this manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mutation. Until, 1. MD/PhD scholarship from the University of Groningen, Groningen, The Netherlands 2. ESPGHAN Networking Grant 2019 3. ChiLDReN and CTSA National Institutes of Health grants: Ann & Robert H. Lurie Children's Hospital, Chicago: U01DK062436; University of Colorado, Denver: U01DK62453, UL1 TR002535; Baylor college of Medicine, Houston: U01DK103149; Children's Hospital of Philadelphia, Philadelphia: U01DK062481, UL1TR000003; Children's Hospital of Pittsburgh, Pittsburgh: U01DK062466; University of California, San Francisco U01DK062500; University of California, San Francisco CTSI grant UL1TR001872; Riley Hospital for Children, Indianapolis: U01DK084536; Seattle Children’s Hospital, Seattle: DK084575; Children’s Hospital Los Angeles, California: U01DK084538. 4. Unrestrictive research grant from Albireo 5. Unrestrictive research grant from Mirum Pharmaceuticals. 6. C&W de Boer Stichting research grant.
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- 2023
14. Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis
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Thompson, Richard J., primary, Artan, Reha, additional, Baumann, Ulrich, additional, Calvo, Pier Luigi, additional, Czubkowski, Piotr, additional, Dalgic, Buket, additional, D’Antiga, Lorenzo, additional, Di Giorgio, Angelo, additional, Durmaz, Özlem, additional, Gonzalès, Emmanuel, additional, Grammatikopoulos, Tassos, additional, Gupte, Girish, additional, Hardikar, Winita, additional, Houwen, Roderick H.J., additional, Kamath, Binita M., additional, Karpen, Saul J., additional, Lacaille, Florence, additional, Lachaux, Alain, additional, Lainka, Elke, additional, Loomes, Kathleen M., additional, Mack, Cara L., additional, Mattsson, Jan P., additional, McKiernan, Patrick, additional, Ni, Quanhong, additional, Özen, Hasan, additional, Rajwal, Sanjay R., additional, Roquelaure, Bertrand, additional, Shteyer, Eyal, additional, Sokal, Etienne, additional, Sokol, Ronald J., additional, Soufi, Nisreen, additional, Sturm, Ekkehard, additional, Tessier, Mary Elizabeth, additional, van der Woerd, Wendy L., additional, Verkade, Henkjan J., additional, Vittorio, Jennifer M., additional, Wallefors, Terese, additional, Warholic, Natalie, additional, Yu, Qifeng, additional, Horn, Patrick, additional, and Kjems, Lise, additional
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- 2023
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15. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency
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Felzen, Antonia, primary, van Wessel, Daan B.E., additional, Gonzales, Emmanuel, additional, Thompson, Richard J., additional, Jankowska, Irena, additional, Shneider, Benjamin L., additional, Sokal, Etienne, additional, Grammatikopoulos, Tassos, additional, Kadaristiana, Agustina, additional, Jacquemin, Emmanuel, additional, Spraul, Anne, additional, Lipiński, Patryk, additional, Czubkowski, Piotr, additional, Rock, Nathalie, additional, Shagrani, Mohammad, additional, Broering, Dieter, additional, Nicastro, Emanuele, additional, Kelly, Deirdre, additional, Nebbia, Gabriella, additional, Arnell, Henrik, additional, Fischler, Björn, additional, Hulscher, Jan B.F., additional, Serranti, Daniele, additional, Arikan, Cigdem, additional, Polat, Esra, additional, Debray, Dominique, additional, Lacaille, Florence, additional, Goncalves, Cristina, additional, Hierro, Loreto, additional, Muñoz Bartolo, Gema, additional, Mozer-Glassberg, Yael, additional, Azaz, Amer, additional, Brecelj, Jernej, additional, Dezsőfi, Antal, additional, Calvo, Pier Luigi, additional, Grabhorn, Enke, additional, Hartleif, Steffen, additional, van der Woerd, Wendy J., additional, Kamath, Binita M., additional, Wang, Jian-She, additional, Li, Liting, additional, Durmaz, Özlem, additional, Kerkar, Nanda, additional, Jørgensen, Marianne Hørby, additional, Fischer, Ryan, additional, Jimenez-Rivera, Carolina, additional, Alam, Seema, additional, Cananzi, Mara, additional, Laverdure, Noemie, additional, Ferreira, Cristina Targa, additional, Guerrero, Felipe Ordoñez, additional, Wang, Heng, additional, Sency, Valerie, additional, Kim, Kyung Mo, additional, Chen, Huey-Ling, additional, de Carvalho, Elisa, additional, Fabre, Alexandre, additional, Bernabeu, Jesus Quintero, additional, Zellos, Aglaia, additional, Alonso, Estella M., additional, Sokol, Ronald J., additional, Suchy, Frederick J., additional, Loomes, Kathleen M., additional, McKiernan, Patrick J., additional, Rosenthal, Philip, additional, Turmelle, Yumirle, additional, Horslen, Simon, additional, Schwarz, Kathleen, additional, Bezerra, Jorge A., additional, Wang, Kasper, additional, Hansen, Bettina E., additional, and Verkade, Henkjan J., additional
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- 2023
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16. JAG1 MUTATION SPECTRUM IN CASES WITH ALAGILLE SYNDROME FROM TURKIYE.
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ASLANGER, Ayça Dilruba, YILDIRIM, Behiye Tuğçe, KALAYCI, Tuğba, ŞENTÜRK, Leyli, AVCI, Şahin, ALTUNOĞLU, Umut, GÜLEÇ, Çağrı, KARAMAN, Volkan, DOĞAN, Güzide, ÖNAL, Zerrin, DURMAZ, Özlem, KARAMAN, Birsen, and UYGUNER, Zehra Oya
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GENETIC variation ,MEDICAL genetics ,COMPARATIVE genomic hybridization ,FLUORESCENCE in situ hybridization ,NUCLEOTIDE sequencing - Abstract
Copyright of Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi Dergisi is the property of Istanbul Tip Fakultesi Dergisi and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2023
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17. Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With İnflammatory Bowel Disease: A Multicenter Study
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Kalaycı, Ayhan Gazi, Yüksekkaya, Hasan, Baran, Maşallah, Kuloğlu, Zarife, Ozgenc, Funda, Balamtekin, Necati, Study Group, Turkish Ibd, Aydoğan, Ayşen, Urgancı, Nafiye, Artan, Reha, Ugraş, Meltem, Arslan, Nur, Tutar, Engin, Tümgör, Gökhan, Özkan, Tanju, Ünal, Fatih, Öztürk, Yeşim, Üstündağ, Gonca, Sarı, Sinan, Erkan, Tülay, Önal, Zerrin, Çaltepe, Gönül, Akçam, Mustafa, Arslan, DURAN, Baysoy, Gökhan, Çakır, Murat, Dalgıç, Buket, Doğan, Yaşar, Durmaz, Özlem, Ecevıt, Çiğdem, Eren, Makbule, Gökçe, Selim, Gülerman, Fulya, Yaman, Aytaç, Bekem Soylu, Özlem, Gürakan, Figen, Hızlı, Samil, Işık, Ishak, Özen, Hasan, Özbay Hoşnut, Ferdağ, Kutluk, Günsel, Kasırga, Erhun, Karabiber, Hamza, Kutlu, Tufan, and Kansu, Aydan
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medicine.medical_specialty ,Adolescent ,MEFV ,Familial Mediterranean fever ,Colonoscopy ,Disease ,medicine.disease_cause ,Inflammatory bowel disease ,Gastroenterology ,Severity ,Association ,Crohn Disease ,familial Mediterranean fever ,inflammatory bowel disease ,Internal medicine ,medicine ,Humans ,Modifier ,Child ,Ulcerative-Colitis ,Children ,mutation analysis ,Mutation ,Disorders ,medicine.diagnostic_test ,business.industry ,Turkish Children ,Frequency ,Inflammatory Bowel Diseases ,medicine.disease ,Childhood ,Ulcerative colitis ,digestive system diseases ,Mutation testing ,Colitis, Ulcerative ,Original Article ,Activity Index ,business ,Mefv Gene-Mutations - Abstract
BACKGROUND: The aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases has been reported previously. METHODS: Children with IBD (334 ulcerative colitis (UC), 224 Crohn’s disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. RESULTS: A total of 597 children (mean age: 10.8 ± 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/heterozygous) in patients with UC (P < .05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P = .031, P = .045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P = .007). Conclusion: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not have a high impact on inflammatory response and clinical outcome of the disease.
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- 2021
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18. Attenuated live vaccine in children with solid organ transplantation on everolimus therapy: Report of two cases
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Keskindemirci, Gonca, primary, Önal, Zerrin, additional, Özbörü Aşkan, Öykü, additional, Yücel, Esra, additional, Varol, Selin, additional, Hançerli Törün, Selda, additional, Gökçay, Gülbin, additional, and Durmaz, Özlem, additional
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- 2022
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19. Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial
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Thompson, Richard J, primary, Arnell, Henrik, additional, Artan, Reha, additional, Baumann, Ulrich, additional, Calvo, Pier Luigi, additional, Czubkowski, Piotr, additional, Dalgic, Buket, additional, D'Antiga, Lorenzo, additional, Durmaz, Özlem, additional, Fischler, Björn, additional, Gonzalès, Emmanuel, additional, Grammatikopoulos, Tassos, additional, Gupte, Girish, additional, Hardikar, Winita, additional, Houwen, Roderick H J, additional, Kamath, Binita M, additional, Karpen, Saul J, additional, Kjems, Lise, additional, Lacaille, Florence, additional, Lachaux, Alain, additional, Lainka, Elke, additional, Mack, Cara L, additional, Mattsson, Jan P, additional, McKiernan, Patrick, additional, Özen, Hasan, additional, Rajwal, Sanjay R, additional, Roquelaure, Bertrand, additional, Shagrani, Mohammad, additional, Shteyer, Eyal, additional, Soufi, Nisreen, additional, Sturm, Ekkehard, additional, Tessier, Mary Elizabeth, additional, Verkade, Henkjan J, additional, and Horn, Patrick, additional
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- 2022
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20. Hepatitis C infection in childhood
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Durmaz, Ozlem
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- 2012
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21. Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial
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MDL patientenzorg, Thompson, Richard J, Arnell, Henrik, Artan, Reha, Baumann, Ulrich, Calvo, Pier Luigi, Czubkowski, Piotr, Dalgic, Buket, D'Antiga, Lorenzo, Durmaz, Özlem, Fischler, Björn, Gonzalès, Emmanuel, Grammatikopoulos, Tassos, Gupte, Girish, Hardikar, Winita, Houwen, Roderick H J, Kamath, Binita M, Karpen, Saul J, Kjems, Lise, Lacaille, Florence, Lachaux, Alain, Lainka, Elke, Mack, Cara L, Mattsson, Jan P, McKiernan, Patrick, Özen, Hasan, Rajwal, Sanjay R, Roquelaure, Bertrand, Shagrani, Mohammad, Shteyer, Eyal, Soufi, Nisreen, Sturm, Ekkehard, Tessier, Mary Elizabeth, Verkade, Henkjan J, Horn, Patrick, MDL patientenzorg, Thompson, Richard J, Arnell, Henrik, Artan, Reha, Baumann, Ulrich, Calvo, Pier Luigi, Czubkowski, Piotr, Dalgic, Buket, D'Antiga, Lorenzo, Durmaz, Özlem, Fischler, Björn, Gonzalès, Emmanuel, Grammatikopoulos, Tassos, Gupte, Girish, Hardikar, Winita, Houwen, Roderick H J, Kamath, Binita M, Karpen, Saul J, Kjems, Lise, Lacaille, Florence, Lachaux, Alain, Lainka, Elke, Mack, Cara L, Mattsson, Jan P, McKiernan, Patrick, Özen, Hasan, Rajwal, Sanjay R, Roquelaure, Bertrand, Shagrani, Mohammad, Shteyer, Eyal, Soufi, Nisreen, Sturm, Ekkehard, Tessier, Mary Elizabeth, Verkade, Henkjan J, and Horn, Patrick
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- 2022
22. EP1232: ODEVIXIBAT EFFECTS ON CHOLESTASIS-RELATED PARAMETERS: ANALYSIS OF POOLED DATA FROM THE PEDFIC 1 AND PEDFIC 2 STUDIES IN CHILDREN WITH PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS
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Thompson, Richard J., primary, Artan, Reha, additional, Baumann, Ulrich, additional, Czubkowski, Piotr, additional, Dalgic, Buket, additional, Durmaz, Özlem, additional, Gonzalès, Emmanuel, additional, Grammatikopoulos, Tassos, additional, Gupte, Girish, additional, Horn, Patrick, additional, Lachaux, Alain, additional, Mckiernan, Patrick, additional, Özen, Hasan, additional, Rajwal, Sanjay R., additional, Roquelaure, Bertrand, additional, Sturm, Ekkehard, additional, Verkade, Henkjan J., additional, Yu, Qifeng, additional, and Kjems, Lise, additional
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- 2022
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23. Nutritional characteristic of children with inflammatory bowel disease in the nationwide inflammatory bowel disease registry from the Mediterranean region
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Doğan, Yaşar, Yücel, Aylin, Yaman, Aytaç, Üstündağ, Gonca Handan, Ünal, Fatih, Korkut Uğraş, Meltem, Tümgör, Gökhan, Tosun, Mahya Sultan, Bekem Soylu, Özlem, KULOĞLU, ZARİFE, Çetin, Funda, Urgancı, Nafiye, Sayar, Ersin, Öztürk, Yeşim, Özkan, Tanju, Özen, Hasan, Önal, Zerrin, SARI, SİNAN, Yüksekkaya, Hasan, Çaltepe, Gönül, Kutluk, Günsel, DUMLUPINAR, EBRU, AKÇAM, Mustafa, Arslan, Duran, Arslan, Nur, Artan, Reha, Uncuoğlu Aydoğan, Ayşen, Balamtekin, Necati, Kasırga, Erhun, Karabiber, Hamza, Kansu, Aydan, Kalaycı, Ayhan Gazi, Baran, Maşallah, Baysoy, Gökhan, Çakır, Murat, Çeltik, Coşkun, Işık, İshak, Özbay Hoşnut, Ferda, Hızlı, Şamil, Gürakan, Figen, Gulerman, Fulya, Gökçe, Selim, Erdemir, Gülin, Eren, Makbule, Ecevit, Çiğdem, Durmaz, Özlem, and Dalgıç, Buket
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Nutrition and Dietetics ,Gender-Differences ,Validation ,Medicine (miscellaneous) ,Colitis Activity Index ,Body-Mass Index ,Growth ,Ulcerative-Colitis ,Patterns ,Pediatric Crohns-Disease - Abstract
BACKGROUND/OBJECTIVES: We analyzed the nationwide pediatric inflammatory bowel disease (PIBD) registry (1998-2016), to evaluate the nutritional status at the time of diagnosis. SUBJECTS/METHODS: Nine types of nutritional status by the combination of weight-for-length (2 years) and length/height-for-age with three categories (2 SD) were described. Malnutrition was defined by WHO criteria. Univariate and multivariate regression analysis was used to identify risk factors for malnutrition. RESULTS: In total, 824 IBD patients (498 Ulcerative colitis (UC); 289 Crohn's Disease (CD); 37 Indeterminate Colitis (IC); 412 male; the median age 12.5 years) were eligible. The prevalence of eutrophy, wasting/thinness, stunting, overweight, tall stature, concurrent wasting/thinness and stunting, tall stature with overweight, tall stature with wasting/thinness, and short stature with overweight were 67.4%, 14.9%, 6.6%, 3.1%, 3.2%, 3.3%, 1.1%, 0.4%, and 0.1%, respectively. The prevalence of malnutrition was 32.7%, indicating a higher prevalence in CD (p < 0.001). Incidence of overweight was less common in the CD than UC and IC (p < 0.001). Multivariate analysis revealed that age of onset (>10 years), prepubertal stage, severe disease activity, perianal involvement, and high C reactive protein level were independently associated with malnutrition in pediatric IBD. CONCLUSION: We showed the frequency of nutritional impairment in PIBD. The percentage of overweight subjects was lower than the other studies. The age of onset, disease activity, CRP level, perianal involvement, and pubertal stage were associated with a higher risk for developing malnutrition. Our results also confirmed that CD patients are particularly vulnerable to nutritional impairment., Society of Turkish Pediatric Gastroenterology, Hepatology, and Nutrition, This research was funded by The Society of Turkish Pediatric Gastroenterology, Hepatology, and Nutrition.
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- 2022
24. Fecal Calprotectin Levels in the Babies with Infantile Colic
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Karabayır, Nalan, primary, Özden, Tülin, additional, Durmaz, Özlem, additional, and Gökçay, Gülbin, additional
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- 2021
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25. Çocukluk Yaş Grubunda Karaciğer Nakli Öncesi ve Sonrası Zayıflatılmış Canlı Aşı Uygulamaları-Türkiye’den Tek Merkez Deneyimi
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KESKİNDEMİRCİ, Gonca, ÖNAL, Zerrin, ÖZBAY, Yeşfa Şebnem, ÖZEK, Esra, HANÇERLİ TÖRÜN, Selda, DURMAZ, Özlem, and GÖKÇAY, Gülbin
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child,liver transplantation,attenuated live vaccination ,Health Care Sciences and Services ,Sağlık Bilimleri ve Hizmetleri ,Çocuk,karaciğer nakli,canlı aşılama - Abstract
The aim of this study was to evaluate the attenuated live vaccine administration before and after liver transplantation. This study was carried out at the Child Health Surveillance Unit of the Social Pediatrics Department. Besides healthy children, children with special needs are being followed-up at the Unit. Each child has a personal health record in the unit. The health records of patients who had received attenuated live vaccine before liver transplantation under 1 year of age or after liver transplantation were evaluated. Demographic characteristics, data showing immunologic status before vaccination, vaccine related reactions and serologic responses were obtained from health records of each child. Retrospective files of 5 patients who were in follow-up were examined. Four patients at least 2 years after the liver transplantation had attenuated live vaccination (measles-mumps-rubella vaccine and / or chickenpox vaccine). No adverse reaction was noted after the immunization among these 4 children. A 6-month-15-day-old infant who was a candidate for liver transplantation had been vaccinated with measles-mumps-rubella and chickenpox vaccine and it was determined that the serological response after vaccination was not complete in the baby who did not have any adverse reactions after vaccination. Our findings demonstrated that despite the use of immusuppressive therapy, it is safe to apply attenuated live vaccines after the transplantation if appropriate conditions were provided. The timing of the rapid effective immunization before transplantation under one year of age needs to be further investigated, Karaciğer nakli öncesi ve sonrası çocuklarda canlı aşı uygulamalarının değerlendirilmesidir. Çalışmamız Sosyal Pediatri Bilim Dalı Çocuk Sağlığı İzlem Birimi'nde yapılmıştır. Birimde sağlıklı çocukların yanı sıra özel ihtiyaçları olan çocuklar da takip edilmektedir. Birimde takipli her çocuğun sağlık kaydı bulunmaktadır. 1 yaşın altında karaciğer nakli öncesinde veya karaciğer nakli sonrasında zayıflatılmış canlı aşı almış hastaların sağlık kayıtları değerlendirildi. Her çocuğun sağlık kayıtlarından demografik özellikler, aşılama öncesi immünolojik durumu gösteren veriler, aşı ile ilgili reaksiyonlar ve serolojik yanıtlar kaydedildi. Takipte olan 5 hastanın geriye dönük dosya incelemendi. Bunlardan dördüne nakilden en erken 2 yıl sonra olmak üzere zayıflatılmış canşı aşı (kızamık-kızamıkçık-kabakulak ve/veya suçiçeği aşıları) uygulanmıştır. Bu dört hastanın hiçbirinde aşı sonrası bir sorun gelişmemiştir. Nakil öncesi zayıflatılmış canlı aşı uygulanan 6 ay 15 günlük bebekte kızamık- kızamıkçık-kabakulak ve suçiçeği aşıları uygulanmıştır. Aşı sonrası herhangi bir istenmeyen reaksiyon gözlenmeyen bebekte aşı sonrası serolojik yanıtın tam olmadığı belirlenmiştir. Bulgularımız nakil sonrası immunsüpresif kullanmakta olan çocuklarda gerekli değerlendirmeler sonrasında zayıflatılmış canlı aşı uygulanmasının güvenli olduğunu ortaya koymaktadır. Diğer yandan karaciğer nakli planlanan, acil aşılanması gereken 1 yaş altındaki hastalarda KKK ve suçiçeği aşı uygulamaları sonrası yeterli yanıt oluşması konusunda ayrıntılı değerlendirme gerekmektedir.
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- 2020
26. Oral Abstracts
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MDL patientenzorg, Gonzales, Emmanuel, Thompson, Richard J, Kamath, Binita M., Lacaille, Florence, Lainka, Elke, Shteyer, Eyal, D'Antiga, Lorenzo, Houwen, RHJ, Verkade, Henkjan J., Durmaz, Özlem, Grammatikopoulos, Tassos, Loomes, Kathleen M., Karpen, Saul J., Mack, Cara Lynn, Ni, Quinhong, Kjems, Lise, Horn, Patrick, MDL patientenzorg, Gonzales, Emmanuel, Thompson, Richard J, Kamath, Binita M., Lacaille, Florence, Lainka, Elke, Shteyer, Eyal, D'Antiga, Lorenzo, Houwen, RHJ, Verkade, Henkjan J., Durmaz, Özlem, Grammatikopoulos, Tassos, Loomes, Kathleen M., Karpen, Saul J., Mack, Cara Lynn, Ni, Quinhong, Kjems, Lise, and Horn, Patrick
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- 2021
27. THU-289 - Analysis of long-term treatment effects of odevixibat on clinical outcomes in children with progressive familial intrahepatic cholestasis in odevixibat clinical studies vs external controls from the NAPPED database
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Hansen, Bettina, Valcheva, Velichka, Yu, Qifeng, van Wessel, Daan, Thompson, Richard, Gonzalès, Emmanuel, Jankowska, Irena, Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad, Kumar, Kishwer, Alkuraya, Fowzan, Sateesh, Meddirevula, Broering, Dieter Clemens, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Serranti, Daniele, Arıkan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Gonçalves, Cristina, Llanillo, Loreto Hierro, Bartolo, Gema Muñoz, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Sturm, Ekkehard, van der Woerd, Wendy L., Kamath, Binita M., Wang, Jian-She, Liting, Li, Durmaz, Ozlem, Önal, Zerrin, Felzen, Antonia, Nomden, Mark, Sonajalg, Jaak, Stein, Philip, Ni, Quanhong, Clemson, Christine, Mattsson, Jan, and Verkade, Henkjan J.
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- 2023
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28. THU-284 - Efficacy and safety outcomes with odevixibat treatment: Pooled data from the phase 3 ASSERT and ASSERT-EXT studies in patients with Alagille syndrome
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Ovchinsky, Nadia, Aumar, Madeleine, Baker, Alastair, Baumann, Ulrich, Bufler, Philip, Cananzi, Mara, Durmaz, Ozlem, Fischer, Ryan, Indolfi, Giuseppe, Karnsakul, Wikrom, Lacaille, Florence, Lee, Way Seah, Maggiore, Giuseppe, Rosenthal, Philip, Ruiz, Mathias, Sokal, Etienne, Sturm, Ekkehard, van der Woerd, Wendy L., Verkade, Henkjan J., Wehrman, Andrew, Clemson, Christine, Yu, Qifeng, Ni, Quanhong, Ruvido, Jessica, Manganaro, Susan, Mattsson, Jan, and Czubkowski, Piotr
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- 2023
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29. TOP-057 - Long-term efficacy and safety of odevixibat in patients with progressive familial intrahepatic cholestasis: results with 96 weeks or more of treatment
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Thompson, Richard, Durmaz, Ozlem, Grammatikopoulos, Tassos, Di Giorgio, Angelo, Ni, Quanhong, Stein, Philip, Clemson, Christine, and Sturm, Ekkehard
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- 2023
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30. Çocukluk Yaş Grubunda Karaciğer Nakli Öncesi ve Sonrası Zayıflatılmış Canlı Aşı Uygulamaları-Türkiye’den Tek Merkez Deneyimi
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KESKİNDEMİRCİ, Gonca, primary, ÖNAL, Zerrin, additional, ÖZBAY, Yeşfa Şebnem, additional, ÖZEK, Esra, additional, HANÇERLİ TÖRÜN, Selda, additional, DURMAZ, Özlem, additional, and GÖKÇAY, Gülbin, additional
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- 2021
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31. Human herpes virus type 8-associated Kaposi sarcoma in a pediatric liver transplant recipient
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Çeltik, Coşkun, Ünüvar, Ayşegül, Aydoğan, Ayşen, Gökçe, Selim, Öztürk, Gülyüz, Güllüoğlu, Mine, Ylmaz, Gülden, Türkoğlu, Salih, Anak, Sema, Sökücü, Semra, and Durmaz, Özlem
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- 2011
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32. Faecal calprotectin levels during the first year of life in healthy children
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Günaydın Şahin, Burcu Sena, primary, Keskindemirci, Gonca, additional, Özden, Tülin Ayşe, additional, Durmaz, Özlem, additional, and Gökçay, Gülbin, additional
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- 2020
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33. Attenuated live vaccine in children with solid organ transplantation on everolimus therapy: Report of two cases.
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Keskindemirci, Gonca, Önal, Zerrin, Özbörü Aşkan, Öykü, Yücel, Esra, Varol, Selin, Hançerli Törün, Selda, Gökçay, Gülbin, and Durmaz, Özlem
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RUBELLA ,VACCINATION of children ,TRANSPLANTATION of organs, tissues, etc. ,EVEROLIMUS ,BLOOD products ,AUTOIMMUNE hemolytic anemia - Abstract
Keywords: children; everolimus; solid organ transplantation EN children everolimus solid organ transplantation 1 3 3 02/13/23 20230301 NES 230301 Abbreviations IgG immunoglobulin G MMR measles-mumps-rubella SOT solid organ transplantation INTRODUCTION In childhood, some vaccines may not be administered before solid organ transplantation (SOT) due to the age at the time of planned surgery, clinical status, and the lack of sufficient time between transplantation and vaccination.[1] Although there was indecisiveness about the attenuated live vaccine administration to children after SOT, current studies and consensus recommendations reported that it can be administered in selected cases.[[2]] Here, attenuated live vaccine administration is described in two cases who had to use everolimus instead of tacrolimus after SOT. It was noticed that the varicella vaccine had not been administered, and she tested negative for varicella IgG. [Extracted from the article]
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- 2023
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34. Investigation of impaired carbohydrate metabolism in pediatric liver transplant recipients
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Gökçe, Selim, Durmaz, Özlem, Çeltik, Coşkun, Aydoğan, Ayşen, Baş, Firdevs, Türkoğlu, Ümit, Özden, İlgin, and Sökücü, Semra
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- 2009
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35. Reliability Of Different Endoscopic Classification Systems In Predicting Pediatric Reflux Esophagitis
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SÖKÜCÜ", EMİROĞLU, Halil Haldun, and DURMAZ, Özlem
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Health Care Sciences and Services ,Sağlık Bilimleri ve Hizmetleri ,Gastroesophageal reflux disease - Abstract
Aim: Retrospective in nature, this study was aimed at evaluating the reliability of four endoscopic classification systems in predicting histological reflux esophagitis in children undergoing esophagogastroduedenoscopy. Materials and Methods: This retrospective study included 213 children (112 male, 101 female, average age 8.4 ± 4.8 years, median age 9 years, range 2 months–18 years) who underwent diagnostic esophagogastroduodenoscopy between January 2002 and December 2004 and evaluated for the presence of reflux esophagitis. Data for age and gender, and detailed endoscopic and histopathological reports were retrieved from medical records. Los Angeles, Savary-Miller, Hetzel-Dent, and Tytgat endoscopic classification systems were used in the evaluation of patients with erosive distal esophagitis. The histological findings were classified according to Knuff & Leape. When reflux-related esophageal damage was identified as a result of the histological examination of endoscopic biopsy samples collected from distal esophagus, the patients were diagnosed with reflux esophagitis. The Statistical Package for the Social Sciences for Windows Release 12.0 (SPSS, Chicago, IL, USA) was used to analyse the statistical data.Results: On the histological examination of esophageal mucosal biopsy specimens of 213 patients, 71 (33.3%) patients had normal (grade 0), 75 (35.2%) patients with only histologic changes of reflux (grade 1) without esophagitis and 67 (31.5%) patients were reflux esophagitis (grade 2–5) were detected. There were 49 (23%) patients with mild esophagitis (grade 2), 6 (3%) patients with moderate esophagitis (grade 3) and 12 (6%) patients with severe esophagitis 2 (1%) patients with grade 4 and 10 (5%) patients with grade 5) in 67 patients with reflux esophagitis.On the endoscopical examination of esophageal mucosal appearances of 213 patients, 36 (16.9%) patients, 36 (16.9%) patients, 100 (46.9%) patients and 90 (42.3%) patients were diagnosed with esophagitis according to the Los Angeles, Savary-Miller, Hetzel-Dent and Tytgat endoscopic classification systems, respectively. When the four different endoscopic classification systems evaluated in terms of score correlation with the histological diagnosis, the most linear relationship was found between LA endoscopic classification and Knuff & Leape histological classification (r = 0.544, p
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- 2020
36. ESKİ TÜRKÇE VE ÇAĞDAŞ KIPÇAK BAŞKURT, TATAR, KAZAK, KIRGIZ TÜRKÇELERİNDE ʽBİZʼ ZAMİRİ
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DURMAZ, Özlem
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Dil,Zamir,Türkçenin dönemleri ,Social ,Language,Turkish periods,Pronoun ,Sosyal - Abstract
‘Bizʼ is used as the first person pronoun in the northwestern (Kipchak) group Tatar, Bashkir, Kazakh, Kyrgyz dialects. This pronoun has been used since the Old Turkish Era and continues to be used in dialects. In this study, the form of the 1st person pronoun which is one of the criteria that makes language the mother tongue and which can be followed since Old Turkish is evaluated in Tatar, Bashkir, Kazakh, Kyrgyz dialects and their usage in these periods. Literature review was done on the subject. The data collected from the scanned studies were examined one by one and the ‘we’ pronoun used in Tatar, Bashkir, Kazakh, Kyrgyz dialects of the Old Turkish and Contemporary Kipchak groups was shown with examples. As a result, the pronoun iz we an used in the Old Turkic and Kipchak group Tatar, Bashkir, Kazakh, Kyrgyz dialects is presented with examples and it is tried to fill the gap in this area., ʽBizʼKuzeybatı (Kıpçak) grubu Tatar, Başkurt, Kazak, Kırgız lehçelerinde çokluk 1.şahıszamiri olarak kullanılmaktadır. Bu zamir Eski Türkçe devrinden günümüze kadarkullanılmış ve lehçelerde de kullanılmaya devam etmektedir. Bu çalışmada, diliana dil yapan ölçütlerden olan ve Eski Türkçeden beri takip edilebilen çokluk1.şahıs zamirinin Çağdaş Kıpçak grubu Tatar, Başkurt, Kazak, Kırgız lehçelerindekişekli ve bu dönemlerdeki kullanımı değerlendirilmiştir. Konuile ilgili literatür taraması yapılmıştır. Taranan çalışmalardan toplananveriler tek tek incelenerek Eski Türkçe ve Çağdaş Kıpçak grubu Tatar, Başkurt,Kazak, Kırgız lehçelerinde kullanılan ‘biz’ zamiri örneklerle gösterilmiştir. Eski Türkçede ve Kıpçakgrubu Tatar, Başkurt, Kazak, Kırgız lehçelerinde kullanılan ʽbiz’ zamiriörneklerle ortaya konmuş ve bu alandaki boşluk doldurulmaya çalışılmıştır.
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- 2019
37. Farklı branşlarda ve cinsiyetteki elit sporcuların imgeleme biçimlerinin incelenmesi / Özlem Engeloğlu Durmaz ; Danışman Osman İmamoğlu
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Durmaz, Özlem Engeloğlu, İmamoğlu, Osman, and OMÜ, Sosyal Bilimler Enstitüsü, Spor Yöneticiliği Anabilim Dalı
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Spor ,İmgelem ,Elit sporcularda imgeleme ,TEZ YÜK LİS D963f 2019 - Abstract
Tez (yüksek lisans) -- Ondokuz Mayıs Üniversitesi, 2019 Libra Kayıt No: 127428 …
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- 2019
38. Farklı branşlarda ve cinsiyetteki elit sporcuların imgeleme biçimlerinin incelenmesi
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Engeloğlu Durmaz, Özlem, İmamoğlu, Osman, and Beden Eğitimi ve Spor Ana Bilim Dalı
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Spor ,Sportsmen ,Sport branches ,Sex ,Imagery ,Sports - Abstract
Amaç: Farklı branşlarda ve cinsiyetteki elit sporcuların imgeleme biçimlerinin incelenmesidir.Materyal ve Metot: Elit düzeyde spor yapan katılımcılara araştırmacı tarafından hazırlanan 4 maddelik kişisel bilgi formu ve Hall ve ark. (1998) tarafından geliştirilen, Kızıldağ ve Tiryaki (2000) tarafından Türkçe' ye uyarlanan sporda imgelenme envanteri kullanılmıştır. Verilerin analizinde SPSS 21 paket programı kullanılmıştır. Verilere bağımsız t testi, ANOVA ve Pearson korelasyon analizleri yapılmıştır. Tüm istatistiki işlemler p 0.05) in the analysis of the remaining 303 surveys independent t test, ANOVA and Pearson correlation analysis were used to determine the relationship between them. All statistical procedures were evaluated according to p
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- 2019
39. Fecal Calprotectin Levels in the Babies with Infantile Colic.
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Karabayır, Nalan, Özden, Tülin A., Durmaz, Özlem, and Gökçay, Gülbin
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INFANTILE colic ,CALPROTECTIN ,GENDER ,INFANTS ,BIRTH weight - Abstract
Copyright of Journal of the Child / Çocuk Dergisi is the property of Journal of Child and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2021
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40. Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With İnflammatory Bowel Disease: A Multicenter Study.
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Urgancı, Nafiye, Ozgenc, Funda, Kuloğlu, Zarife, Yüksekkaya, Hasan, Sarı, Sinan, Erkan, Tülay, Önal, Zerrin, Çaltepe, Gönül, Akçam, Mustafa, Arslan, Duran, Arslan, Nur, Artan, Reha, Aydoğan, Ayşen, Balamtekin, Necati, Baran, Maşallah, Baysoy, Gökhan, Çakır, Murat, Dalgıç, Buket, Doğan, Yaşar, and Durmaz, Özlem
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- 2021
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41. Mekansal deneyim ve temsilinin çokluduyumsallık bağlamında değerlendirilmesi
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Durmaz, Özlem, Arı, İffet Hülya, and Mimarlık Ana Bilim Dalı
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Architecture ,Mimarlık - Abstract
Bireyin fiziksel çevredeki yaşamı; kaçınılmaz olarak bedenine içkin duyumsama pratikleri ile deneyimlediği mekana ilişkin, çevresel bilgi edinme ve anlamlandırma süreçlerini kapsar. Mekanın bütüncül algısı ve kavranışı; duyumsama yoluyla tetiklenen bilişsel süreçler ile geliştirilen zihinsel yaratılar üzerinden irdelenir. Bireyin zihinsel yaratıları üzerinden mekanla kurduğu ilişkiyi açığa çıkaran temsil, çalışmanın odağına alınırken; mekanın fiziksel özelliklerinin aktarımını sağlayan bir araç olmaktan öte, mekansal deneyimin değerlendirildigi ve bu değerlendirme yolu ile yeni mekansal kavrayışları oluşturmaya imkan tanıyan bir ortam olarak ele alınır. Bireyin mekanla olan ilişkisinde, bedensel deneyimin görme duyusu baskınlığında geliştirilmesi, deneyime ait bilişsel süreçlerin değerlendirilmesine imkan sağlayan temsil kavramının da görme duyusuna hitap edecek biçimde üretilmesine sebep olur. Mimari tasarım pratiğinde mekanın niceliksel bilgisini aktarma ithiyacı, temsil ortamında yine ağırlıklı olarak görsel üretimleri beraberinde getirirken; mekansal deneyimin çokluduyumsal niteliğinin yok sayılmasına yol açar. Temsilin neyi, niçin, nasıl temsil ettiği sorularına yönelik araştırmada öne çıkan mimari tasarım pratiği ve uygulama sürecinde benimsenen kartezyen anlayış; mekanı, ancak kartezyen koordinat sistemi üzerinde tanımlanabilen uzunluk, genişlik, derinlik gibi soyut-matematiksel sistemlerin kurgusuna dönüştürür. Bireyin mekanla olan ilişkisinin temsil biçimleri üzerinden açığa çıktığı düşünülürse; mimarlık disiplini çerçevesinde geleneksel temsil biçimlerinin matematiksel sembol ve ifadelerle oluşturulmasını sağlayan -ve bu nedenle de ağırlıklı olarak görsel olarak hizmet veren- kartezyen mekan yaklaşımının araştırılması ve sorgulanması gerekmektedir. Çünkü rasyonel ve matematiksel ifadelerle bireyi mekandan ayrıştıran kartezyen anlayış; bireyin mekanla olan dinamik, ilişkisel ve deneyimsel bütünlüğü ile ters düşmektedir. Mekan; matematiksel bilgi üzerinden ilerleyen Kartezyen anlayış yerine, deneyime ait fiziksel ve bilişsel süreçlerin bilgisini irdeleyen fenomenolojik yaklaşım ile kavranılabilir. Tez kapsamında, bireyin mekanla olan deneyimsel ilişkisi ve bireyde tetiklenen bilişsel süreçler araştırılırken; bu ilişki üzerinden üretilen temsillerde mekanın niceliğinden öte, deneyimsel niteliği vurgulanır. Mekanın yapısal özelliklerinin temsili, mekansal deneyimi önceleyemeyeceği gerekçesiyle eleştirilmektedir. Kartezyen düşüncenin de temeli olan 'nicelik olarak ölçülebilme' durumuyla birlikte; -görsel birer nesneye dönüşen- geleneksel mimari temsil biçimlerinin, birey ve mekan arasında gerçekleşen çok boyutlu deneyimi ve kavrayışı aktarma ve değerlendirme sürecinde yeterli bir sistem oluşturmadığı düşüncesine savunulmaktadır. Bireyin çokluduyumsal olarak deneyimlediği mekan yerine, mekansal deneyimin kendisinin ifadesini ön plana çıkarmak amaçlanmaktadır.Bireyin mekan ile olan etkileşimine ait tüm katmanlar önce deşifre edilip ardından temsil biçimlerinin bu bağlamda değerlendirilmesiyle; deneyimsel mekanın kavranışına, bilgisine ve üretimine katkı sağlamak amacıyla deneyimi oluşturan duyumları vurgulayan alternatif bir temsil modeli tartışmaya açılmaktadır. Çalışma kapsamında değerlendirilen kavramlar ve öngörüler doğrultusunda tez 5 başlık altında incelenmiştir. Tezin 1. bölümünde; bireyin dahil olduğu fiziksel çevreye ilişkin bilgi edinme ve anlamlandırma süreçlerinde etkin kavramlar ve ilişkiler ağı üzerinden; kavramlara ilişkin giriş niteliğindeki yorum ve öngörülerin sunulmasıyla, tezin amacı, kapsamı ve araştırma yöntemi açığa çıkarılmaktadır.2. Bölümde; birey-mekan etkileşiminin temsil üzerinden değerlendirilmesi sürecinde etkin biçimde açığa çıkan; birey ve mekan, deneyim ve duyumsama, temsil ve gözmerkezcilik kavramlarına ilişkin tanım ve yorumlara yer verilmektedir. Birey ve mekan kavramlarının birbirinden ayrıştırılamaz biçimde; fiziksel ve zihinsel deneyim kurgusuyla bütünleştiğinin altı çizilirken, birey ve mekan arasındaki fiziksel ve bilişsel süreçlerde görmeyi merkeze alan davranış biçimi eleştirilmektedir. İlgili kavramların değerlendirilmesiyle temsil kavramı, birey ile mekan arasındaki düşünme ve değerlendirme ortamı olarak kavramsallaştırılarak bireyin mekan ile kurduğu başka bir çeşit ilişki türü olarak yorumlanmaktadır.Bireyin fiziksel çevredeki yaşamının, kaçınılmaz olarak çevresel bilgi edinme ve anlamlandırma süreçlerini kapsadığı gerekçesiyle, bilişsel süreçler ve temsil kavramının detaylandırılması 3. Bölümde yapılmaktadır. Bireyin mekansal deneyimi ile paralel biçimde ilerleyen bilişsel aktiviteleri kapsamında algılama, kavrama, bellekte depolama, düşünme ve tepki oluşturma süreçleri gerçekleşir. Bireyin mekana ilişkin bütüncül algısı; bedensel duyumsama ile başlayan nörofizyolojik süreçlerin oluşturduğu zihinsel (soyut) mekan yaratısı biçimindeki çıktıları üzerinden kavrayışla açıklanmaya çalışılmaktadır. Bu süreçler ile elde edilen zihinsel yaratı, soyut temsil kavramı ile ifade edilirken; soyut bağlamdaki yaratının çeşitli araç ve tekniklerle maddesel bağlama kazandırılması ise temsilin somut hali şeklinde ifade edilmektedir. Temsilin bireyin bedensel deneyiminden temellenip, zihinsel süreçteki işleyişi ve ardından tekrar somut biçimde duyumsanır ürüne dönüşümü irdelenmesi; mimarlık disiplinine ait temsil üretme süreci üzerinden gerçekleştirilmektedir. Mimari tasarımda temsilin `ne amaçla` kullanıldığı sorgulaması; tasarım ve uygulama süreçlerinin işlevsel aşamalandırılması üzerinden değerlendirilir. Temsilin `neyi?`, `nasıl?` temsil ettiği sorusu ile; ağırlıklı olarak görme duyusundan temellenen ve görme duyusuna hitap eden geleneksel mimari temsil anlayışının 2 ve 3-boyutlu araçları ile sanal ortamdaki araçları deşifre edilmektedir. Bireyin mekanla ilişkisini temsil üzerinden geliştirmeye yönelik imkan tanıyan temsilin; mekanın 'ne' olduğu yerine 'nasıl deneyimlendiği'ni temsil etmesi gerekliliği vurgulanır. Somut biçimdeki temsil ürünü ister 2 veya 3-boyutlu ister sanal temsil teknikleriyle geliştirilmiş olsun, tekrar deneyimlenmeye müsait yapısı gereği bireyin mekanla kurduğu ilişkinin zihinsel boyutunda katmanlaşmaya imkan tanıdığı vurgulanır.4. Bölümde; mekanın çokluduyumsal biçimde deneyimlendiği kavrayışında gerçekleşen çalışmalara ilişkin bilgi ve temsil biçimlerine yer verilir. Mekanın çokluduyumsal yapısı hakkında yapılan kentsel ölçekteki duyu yürüyüşü içerikli çalışmaların aktarımını sağlayan temsil biçimleri ile tasarımı çokluduyumsal biçimde gerçekleştirilen mekanların deneyimlerinin aktarımında kullanılan temsil biçimleri; çokluduyumsal deneyim kapsamında sorgulanmaktadır. Temsil biçimlerine yönelik araştırmanın sonunda, mekansal deneyime ait bellek ve kavrayışın irdeleneceği temsil; bir düşünce mekanı olarak, bireyin mekansal deneyimini değerlendirip hakkında bilişsel süreçlerini geliştirmesine olanak sağlayacağı bir ortam olarak ele alınır. Bu doğrultuda temsil araç ve tekniklerini Kartezyen anlayış ve gözmerkezci yapıdan özgürleştirmek fikri ile çokluduyumsal temsil kavramı geliştirilir. Geleneksel temsil tekniklerinin gözmerkezcilliğine yönelik eleştiri ve araştırmalardan yola çıkan, tümevarımsal analiz yöntemi ile geliştirilen çokluduyumsal temsil kavramı, üretim sürecinin nasıl ilerlemesi gerektiği konusunda detaylandırılarak genişletilir. Bu doğrultuda deneyimin ve deneyimin temsilinin öznel sürecine vurgu yapacak biçimde Gayrettepe Metro sergi alanında yer alan `Karanlıkta Diyalog Deneyimsel Sergi` tarafımca deneyimlenip, deneyime ait çokluduyumsal temsil üretimi gerçekleştirilir. Temsil üretim sürecine ilişkin detayların aktarılmasıyla, mekansal deneyimin çokluduyumsallığı ve temsilin bilişsel süreçleri etkileyen kurgusu vurgulanır. 5. bölüm olan sonuç ve tartışma bölümünde ise; tezde ele alınan tüm kavramlar ile `Karanlıkta Diyalog` deneyimine ait temsilin çokluduyumsal biçimde üretme sürecine ait mekansal kavrayış ve bellek etkileri irdelenir. Çokluduyumsal biçimde temsili gerçekleştirilen mekansal deneyimin, hem diğer bireyler tarafından çokluduyumsanmasına izin veren yapısı ile yeni algı ve kavrayışlara yön vermesi; hem de temsil üreten bireyin, temsilini ürettiği gerçeklikle arasındaki bağın katmanlaşması ile geliştirilen belleğin kendi üzerinde bükülüp derinleşmesine imkan sağlayacağı düşüncesi vurgulanır. The individual's life comprises the processes of elaboration and information of space where experienced through bodily senses. Holistic perception and comprehension of space are examined by mental creations that are developed in cognitive processes which are triggered by sensorial. Focusing on the representation that reveals the relation between individual's mental creation and space, it is treated as a thinking medium that enables to -new- spatial comprehensions rather than a tool that transfers architectural tectonics. As space is experienced in visual dominance, the representation that enables for evaluating experiential triggered cognitive processes causes to production in the visual format that appeals to the sense of sight. In architectural design practice, the requirement of transferring the quantitative information of space is produced with visual dominance as well as caused to disregarding multisensorial experience of space.While investigating how, what, why representation does represent, in architectural design practice that treats Cartesian understanding which transformed the space to abstract-mathematical systems is highlighted. When it is thought that the representation methods reveal the relation between individual and space; cartesian understanding that serves visual dominance because it enables the production of representation with mathematical symbols in the framework of architectural design must be questioned. As Cartesian understanding conflicts with the dynamic, relational and experiential integrity of individual with space; space can be comprehended with the phenomenological approach that examines the information of cognitive and physical processes of experience rather than Cartesian understanding that separates the individual from space by rational and mathematical notions. Within the scope of this thesis, while researching the relation and triggered cognitive process between individual and space; it is emphasized the experientiality of space rather than quantitative on produced representations. Due to not being anticipating the spatial experience, representation of architectural tectonics of space is criticized. Measurable as a quantitative approach which is the root of Cartesian understanding presents architectural representation methods as visual objects. It is argued that it does not an adequate system in process of evaluating and transferring multisensorial experience and comprehension which is related with individual and space. So, it is aimed to highlight the representation of spatial experience instead of space where is experienced multisensorial.An alternative representation model that emphasizes the experiential senses to contribute the production, information and comprehension of space is presented through deciphering representation methods that includes all layer about the relation between individual and space.The study mainly indicates that spatial experience consists of the whole of all fragments, gathered through motion, sensation and cognition. Within this framework, the thesis is examined in five main chapters. In the first chapter, purpose, scope and research method of the thesis are illustrated. Also, the content and relational network, that effected processes of information about the physical environment are explained. The second chapter includes definitions and interpretations about notions such as individual and space, experience, representation and ocularcentrism that are emerged during the evaluation of the relation between individual and space through the representation. Highlighting the unity of individual and space through bodily experiences and mental creations, ocularcentrism that becomes focus behavior of physical and mental experience processes is criticized. By elaborating related notions, representation conceptualized as thinking and evaluating medium between individual and space, so it can be read some kind of relation type between individual and space.Knowledge of representation and cognitive processes that related to bodily and spatial experiences is elaborated in chapter three. This chapter based on questions which are: `What the effective practices in understanding process his/her existence in the physical environment, and how it works?` and `What/how/why does representation represent?` and then chapter developed with accordingly. For this, information and evaluation are given about cognitive processes and representation methods in architectural design. With intangible processes that are based on spatial experience developed in brain, people gains spatial perception, comprehension and memory and then, these determine the behavior of individual. The holistic spatial perception of individual is explained with comprehension through the mental (intangible) creation that created by the sensory triggered neurophysiological processes. That's why the experience-based thinking and information of the individual is conceptualized as a (mental) intangible representation. And producing with tangible tools is conceptualizes as a (physical) tangible representation which is examined during the thesis.Producing process of representation that is transferred by individual the multisensorial experience and experience-based holistic perception of space through the representation tools and methods in architectural design are examined. Questioning about `why does representation represent?` examined through staging in processes of architectural design and practice. With the question that: `What/how/why does representation represent?` the representations that based on vision dominance and address to sense of sight are deciphered by 2-dimensonal and 3-dimensional tools of traditional representation. Due to representation provides to improve the spatial relation of individual, the necessity of representation that should represent the `how space get experienced?` instead of `what is space?` is emphasized. The product of representation whether tangible or virtual is re-experiential being, hence it emphasized that is enabled to folding the mental creation of relation with space.The fourth chapter of the thesis includes spatial examples that are experienced multisensoriality. Researches that are executed about multisensorial experience in space scale are questioned through used representation methods. These examples were selected due to emphasizing multisensory experience that occurs at various spatial scales. It is anticipated that the tools and methods of representation used in these studies are different from traditional representation because of the comprehension way of spatial experience.At the end of the examination of examples, representation of the memory and comprehension of spatial experience is considered as an environment in which an individual can evaluate the spatial experience and develop his cognitive processes. In this context, multisensorial representation that can be set the representation methods free from Cartesian understanding and ocularcentrism is presented. The concept of multisensorial representation, which is derived from criticism and research on the centrality of traditional representation techniques and developed by inductive analysis, is extended by detailing how the production process should proceed. So, in order to emphasize the subjectivity of experience and its representation, `Dialogue in the Dark Experiential Exhibition` which presents in the exhibition area of Gayrettepe Metro Station is experienced by myself. Also, multisensorial representation of this experience is produced. By transferring the details of the subjective multisensorial representation production process; multisensoriality of spatial experience and effects on the cognitive processes of representation are emphasized. In the last chapter, all concepts in the thesis and individual's spatial comprehension and memory are examined through the effects of multisensorial experience and multisensorial representation process of `Dialogue in the Dark.` Production of multisensorial representation of spatial experience allows to gaining new comprehensions by other individuals who experienced it. Also, it allows to folding and deepening the memory of space by the process of production. 145
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- 2017
42. FRI276 - The phenotype of compound heterozygous BSEP deficiency patients is determined by the combined residual function of the two ABCB11 mutations: results from the NAPPED consortium
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Felzen, Antonia, van Wessel, Daan, Thompson, Richard, Gonzales, Emmanuel, Jankowska, Irena, Sokal, Etienne, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jacquemin, Emmanuel, Spraul, Anne, Lipiński, Patryk, Czubkowski, Piotr, Rock, Nathalie, Shagrani, Mohammad Ali, Broering, Dieter Clemens, Algoufi, Talal, Mazhar, Nejat, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, JBF, Serranti, Daniele, Arıkan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Gonçalves, Cristina, Hierro, Loreto, Bartolo, Gema Muñoz, Glassberg, Yael Mozer, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Sturm, Ekkehard, van der Woerd, Wendy, Kamath, Binita M., Wang, Jian-She, Liting, Li, Durmaz, Ozlem, Kerkar, Nanda, Jørgensen, Marianne Hørby, Fischer, Ryan, Jimenez-Rivera, Carolina, Alam, Seema, Cananzi, Mara, Ruiz, Mathias, Targa, Cristina, Ferrero, Felipe Ordoñez, Wang, Heng, Kim, Kyungmo, Chen, Huey-Ling, Carvalho, Elisa, Hansen, Bettina, and Verkade, Henkjan
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- 2020
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43. Profile of Anemia in pediatric liver transplant recipients
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Sütçü, Murat, primary, Cantez, Mustafa Serdar, additional, Gerenli, Nelgin, additional, and Durmaz, Özlem, additional
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- 2018
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44. An evaluation of impact of urban transformation on the fundamental rights in the context the Turkish legal system
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Durmaz, Özlem, Tokuzlu, Lami Bertan, and Hukuk
- Abstract
258 pages
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- 2016
45. Kentsel dönüşümün Türkiye'de ki hukuk düzeninde temel haklara etkisi üzerine bir değerlendirme
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Durmaz, Özlem, Tokuzlu, Lami Bertan, and Hukuk Ana Bilim Dalı
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Hukuk ,Turkish Law ,Property right ,Fundamental rights ,Gentrification ,Law ,Urban transformation ,Law order - Abstract
Birinci bölümde kent, kentleşme ve kentlileşme kavramlarının değişik tanımları yapılarak açıklanmaya çalışılmıştır. Ayrıca kentsel dönüşümün çeşitli tanımları yapılarak bu kavram hakkında genel bilgi verilmiştir. Dünyada ve Türkiye' de dönüşümün tarihi gelişimi üzerinde durularak, kentsel dönüşümün amacı, kentsel dönüşüm modellerinin neler olduğu ve kentsel dönüşümün hangi yöntemler izlenerek yapılabildiği anlatılmış, kentsel dönüşüm projelerinin toplum üzerindeki etkileri açıklanmaya çalışılmıştır. İkinci bölümünde; kentsel dönüşüm projelerinin temel haklar denince ilk akla gelen; mülkiyet hakkı ve diğer temel haklarla olan ilişkisi değerlendirilmiştir. İlk olarak temel hakların başında gelen mülkiyet hakkı daha sonra diğer temel haklardan olan barınma, konut ve yerleşmenin korunması hakkı ve en son olarak da kültürel ve tarihsel yaşamın korunması hakkı ile olan ilişkisi değerlendirilmiştir. İlk önce, temel hakların yabancı Anayasa'lar da ki ve uluslar arası alanlardaki düzenleniş biçimleri değerlendirilmiştir. Çünkü ülkelerde ve Türkiye'de ki temel hakların; düzenleniş ve kentsel dönüşümlerden etkilenmeleri farklılık gösterebilmektedir. Daha sonra her temel hakkın ülkemizde düzenleniş biçimlerine kısaca değinilmiş, Avrupa İnsan Hakları Mahkemesi kararlarından bazılarına yer verilmiş ve son başlıkta da kentsel dönüşüm projelerine, bazı dünya ülkelerinden örnekler verilerek yapılış biçimleri değerlendirilmeye çalışılmıştır. Üçüncü bölümde Türkiye'de mevcut yasal düzenlemeler çerçevesinde kentsel dönüşümün alt yapısı anlatılmaya çalışılmıştır. Tarihi alanların dahi göz ardı edilerek, ülkemizde her geçen gün bir yenisi çıkarılan veya var olan yasalara ek maddeler getirilerek değiştirilen kanunlar eliyle kentsel dönüşüm projelerinin nasıl desteklendiği üzerinde durulmuştur. Çıkarılan deprem yasası adı ile de bilinen 6306 sayılı Afet Riski Altındaki Alanların Dönüştürülmesi Hakkında Kanun ' un neler getirdiği, kişileri nasıl etkilediği ve bu yasanın kendisinin bir afet yasası olduğu, var olan maddeler ışığında açıklanmaya çalışılmıştır. T.C. Başbakanlık Toplu Konut İdaresi Başkanlığı (`TOKİ`) adlı kurumun bu dönüşümleri nasıl yönlendirdiği üzerinde durularak ülkemizde yapılan kentsel dönüşüm çalışmalarına örnekler verilerek ana hatlarıyla ortaya konmuş bu dönüşümlerin nasıl yapıldığı değerlendirilmiş ve var olan gelişmeler açıklanmaya çalışılmıştır. Ayrıca kentsel dönüşüm çalışmalarının verdiği zararlarına karşı ne gibi yollar izlenebileceğine değinilmiştir. Dördüncü bölümde sonuç olarak; Türkiye'de yapılan kentsel dönüşüm projelerinin bir oldu bitti sonucu yapıldığı, dönüşümlerin yapılabilmesi için değişik yasalar çıkartıldığı, kişilerin- toplumun ihtiyaçları göz önünde bulundurulmadan, kurum ve kuruluşlardan öneri almadan yapıldığı, rant odaklı çalışmalar olduğu ve temel haklara zararlar verdiği tespitleri ortaya konulmuştur. Bu gibi çalışmaların kentsel dönüşüm de başarı getirmesi bir yana daha çok zararlar vereceği, toplumlar tarafından desteklenmeyeceği ve sadece görünüşte var olan (kişileri afetlerden, depremlerden koruması) amacına hizmet etmeyeceği sonucuna varılmıştır.Son olarak, ekler bölümü ile iki yerel mahkeme kararı konularak tez sonlandırılmıştır.1) Kentsel Dönüşüm 2) Soylulaştırma 3) Mülkiyet Hakkı 4) Temel Haklar 5) Kentsel Dönüşüm Projeleri Under first chapter, the concepts of city, urbanisation, being urbanised are defined and explained. Moreover, different definitions of and general information regarding Urban Transformation are elaborated. The chapter further provides the historical development of the urban transformation in the world and Turkey, and explains the impetus of the Urban Transformation; elaborates different models of transformation and identifies the possible different methods; also it has been attempted to explain the impact of Urban Transformation projects on society.Second chapter evaluates the social implications of the transformation projects and their relationship with the right of property and other fundamental rights. Firstly, the relationship with the right of property, which is a primary fundamental right; then, the relationship with housing right, which is another fundamental right, and finally the relationship with cultural and the protection of historical heritage is evaluated. First of all, the way of regulation of fundamental rights under foreign constitutions and international regulations is examined, which is followed by examination of the way of regulation in Turkey. Because of the fundamental rights in countries affected by urban renewal and Turkey may vary. Given some of the European Court of human Rights decision and Examples were given and explained for the aim of understanding how world countries has been conducted Urban Transformation.Under third chapter, the back ground of urban transformation has been explained through the existing laws. Then focused on how the urban transformation projects are supported in Turkey through frequently promulgated laws or frequent amendments to the existing laws which disregard even the historical areas. Also the chapter examines the Act regarding the Transformation of the Areas under the Disaster Risk, which is also recalled as the Earthquake Act, what sort of rules it lays down, how affect the citizens and it is attempted to illustrate that the act itself is a disaster act which is elaborated within the light of the provisions that it lays down. Evaluated how the institution recalled TOKI orients those transformations and urban transformation projects' examples in Turkey were given and explained and assessed in detail. It has also been mentioned as to what roads could be menitored against the damage caused by the urban renewel work. Under the fourth chapter, as a conclusion, it is laid down that the urban transformation projects conducted in Turkey have been implemented as fait accompli; in order to implement the project different regulations have been enacted; the projects have not considered the people's and society's needs or have been implemented without receiving prior consultation from the relevant bodies or institutions; instead, they have been targeted for obtaining unjust revenues. It is concluded that these kind of conducted projects do not provide any success in terms of urban transformation; rather, they lead to significant losses; they are not supported by the society and they do not serve to the main impetus of providing protection against disasters (earthquakes). It has been reached to the conclusion that when the examples conducted globally are taken into consideration, success rate is considerably low. This thesis also incorporated the decisions rendered by the European Court of Human Rights with regard to the right of property and under the annexes section, various regulations which are in force and various regulations which are projected to be implemented in Turkey are also included. Finally, the thesis put two district court decisions with appendices is terminated.1) Urban Transformation2) Gentrification3) The Right of Property4) Fundamental Rights5) Urban Transformation Projects 319
- Published
- 2016
46. FRI-436-Congenital porto-systemic shunts in children: Preliminary results from the IRCPSS
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McLin, Valérie, Franchi-Abella, Stéphanie, Debray, Dominique, Korff, Simona, Casotti, Valeria, Colledan, Michele, D’Antiga, Lorenzo, Goyet, Jean De Ville De, Durmaz, Ozlem, Lurz, Ebehard, Hierro, Loreto, Kanavaki, Ino, Lipsich, José, Lopez-Santamaria, Maria, Maria, Magnusson, Mozer-Glassberg, Yael, Ponce, Md, Pop, Tudor, Prieto, Cr, Rock, Nathalie, Stephenne, Xavier, Varma, Sharat, Wildhaber, Barbara, Guerin, Florent, and Gonzales, Emmanuel
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- 2019
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47. PS-195-Predicting long-term outcome after surgical biliary diversion in Bsep-deficiency patients: Results from the NAPPED consortium
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Wessel, Daan van, Thompson, Richard, Grammatikopoulos, Tassos, Kadaristiana, Agustina, Jankowska, Irena, Lipiński, Patryk, Czubkowski, Piotr, Gonzales, Emmanuel, Jacquemin, Emmanuel, Spraul, Anne, Sokal, Etienne, Shagrani, Mohammad Ali, Broering, Dieter Clemens, Algoufi, Talal, Mazhar, Nejat, Nicastro, Emanuele, Kelly, Deirdre, Nebbia, Gabriella, Arnell, Henrik, Fischler, Björn, Hulscher, JBF, Serranti, Daniele, Arıkan, Cigdem, Polat, Esra, Debray, Dominique, Lacaille, Florence, Gonçalves, Cristina, Hierro, Loreto, Bartolo, Gema Muñoz, Mozer-Glassberg, Yael, Azaz, Amer, Brecelj, Jernej, Dezsőfi, Antal, Calvo, Pier Luigi, Grabhorn, Enke, Sturm, Ekkehard, Woerd, Wendy van der, Kamath, Binita M., Wang, Jian-She, Liting, Li, Durmaz, Ozlem, Hansen, Bettina, and Verkade, Henkjan
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- 2019
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48. Atipik Çölyak Hastalığı ve Eşlik Eden Otoimmün Hepatit
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GÖKÇE, Selim, DURMAZ, Özlem Süoğlu, ÇELTİK, Coşkun, AYDOĞAN, Ayşen, GÜNGÖR, Mine Güllüoğlu, and SÖKÜCÜ, Semra
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Çölyak hastalığı,glutensiz diyet,otoimmün hepatit,çocuklar ,Celiac disease ,gluten-free diet ,autoimmune hepatitis ,children - Abstract
Celiac disease (CD) was diagnosed in a 10-year-old boy who was presented with refractory iron deficiency anemia (IDA), hypertransaminasemia and short stature. Anemia resolved within one month after the institution of gluten-free diet. We performed liver biopsy because of hypergamaglobulinemia and high titers of smooth muscle antibody. On the basis of histologic findings together with clinical and laboratory findings, type 1 autoimmune hepatitis (AIH) was diagnosed. We aimed to report a CD case displaying more than one type of atypical pictures. Concomitant AIH and unusual rapid response of hepatitis to conventional treatment are also emphasized. Turkish Başlık: Atipik Çölyak Hastalığı ve Eşlik Eden Otoimmün Hepatit Anahtar Kelimeler: Çölyak hastalığı; glutensiz diyet; otoimmün hepatit; çocuklar Burada tedaviye dirençli demir eksikliği, transaminaz yüksekliği ve boy kısalığı ile başvuran, çölyak serolojisi pozitif saptanan ve ince bağırsak biyopsisi ile çölyak hastalığı tanısı alan 10 yaşında olgu sunuldu. Anemi sadece glutensiz diyet ile bir ay içerisinde düzeldi. Başvuru anında hipergamaglobülinemi ve anti-düz kas antikor pozitifliği nedeniyle yapılan karaciğer biyopsisinde portal lenfoplazmositer infiltrasyon saptandı. Histolojik, klinik ve laboratuar bulgular ile otoimmün hepatit tanısı aldı ve immünsüpresif tedavi başlandı. Olgu, atipik prezentasyonun yanında eşlik eden otoimmün hepatitin glutensiz diyet altında immunsüpresif tedaviye alışılagelmişin dışında hızlı yanıt vermesi nedeniyle sunuma değer bulunmuştur., Burada tedaviye dirençli demir eksikliği, transaminaz yüksekliği ve boy kısalığı ile başvuran, çölyak serolojisi pozitif saptanan ve ince bağırsak biyopsisi ile çölyak hastalığı tanısı alan 10 yaşında olgu sunuldu. Anemi sadece glutensiz diyet ile bir ay içerisinde düzeldi. Başvuru anında hipergamaglobülinemi ve anti-düz kas antikor pozitifliği nedeniyle yapılan karaciğer biyopsisinde portal lenfoplazmositer infiltrasyon saptandı. Histolojik, klinik ve laboratuar bulgular ile otoimmün hepatit tanısı aldı ve immünsüpresif tedavi başlandı. Olgu, atipik prezentasyonun yanında eşlik eden otoimmün hepatitin glutensiz diyet altında immunsüpresif tedaviye alışılagelmişin dışında hızlı yanıt vermesi nedeniyle sunuma değer bulunmuştur.
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- 2014
49. Demographic findings of 104 patients with percutaneous endoscopic gastrostomy
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CANTEZ, Mustafa Serdar, CANTEZ, Serdar, GERENLİ, Nelgin, ERTEKİN, Vildan, and DURMAZ, Özlem
- Subjects
Child,demographic findings,percutaneous endoscopic gastrostomy ,Health Care Sciences and Services ,Çocuk,demografik bulgular,perkütan endoskopik gastrostomi ,Sağlık Bilimleri ve Hizmetleri - Abstract
Amaç: Çocukluk döneminde perkütan endoskopik gastrostomi işlemi yapılan olguların demografik bulgularını değerlendirmek.Gereç ve Yöntem: Çalışmaya 2005 2012 arasında endoskopi birimimizde perkütan endoskopik gastrostomi işlemi uygulanan olgular alınmıştır Olguların demografik bulguları hasta dosyalarından elde edilmiş ve geriye dönük olarak incelenmiştir.Bulgular: Çalışma grubumuzda yedi sene içinde 104 olguya 46 kız 44 269 perkütan endoskopik gastrostomi ve perkütan endoskopik gastrostomi ilişkili işlem gerçekleştirilmişti Ortalama izlem süresi 36 5 aydı 3 84 ay Perkütan endoskopik gastrostomi işlemi uygulanmış olguların çoğunluğunu nörolojik ve metabolik hastalıkları olan olgular oluşturmaktaydı sırasıyla 45 ve 40 Perkütan endoskopik gastrostomi işlemi uygulanmış olgularda ortanca yaş 5 9 2 ay 16 yaş ortalama tartı 17 7 kg 3 47 kg idi Olguların işlem öncesi tartı Z skoru ortanca değeri 1 98 olarak hesaplandı Kırk beş olgunun işlem sonrası ortalama on sekizinci ayda ortanca Z skor değeri 1 21 olarak bulundu p=0 0007 Önemli komplikasyon üç olguda 2 8 hafif komplikasyon tüm işlemler arasında 10 sıklıkta saptanmıştı İşleme bağlı cilt komplikasyonu sıklığı 12 5 n=13 idi En sık görülen cilt komplikasyonu yerel stoma enfeksiyonuydu 7 13 Çalışma grubumuzdaki hastalarda sistemik enfeksiyon gözlenmemiştir.Çıkarımlar: Bir çok ülkeden bildirilen olgu serilerinden farklı olarak ülkemizde perkütan endoskopi gastrostomi gereksinimi olan çocuklar arasında metabolik hastalığı bulunanların sıklığı dikkati çekmektedir Komplikasyonların görülmesine karşılık perkütan endoskopik gastrostomi işlemi çocukluk çağında beslenmenin sağlanmasında güvenilir bir y öntemdir., Aim: The aim of this study was to evaluate demographic findings of 104 children who had undergone percutaneous endoscopic gastrostomy insertion procedure Material and Method: Children who had undergone PEG insertion procedure between 2005 and 2012 were included in the study Demographic data were collected retrospectively from the clinic charts Results: A hundred and four patients 44 girls 46 with 269 percutaneous endoscopic gastrostomy and percutaneous gastrostomy related procedures in the last 7 years were enrolled in this study The mean follow up time was 36 5 months 3 84 months Patients with neurological disabilities and metabolic diseases constituted the majority 45 and 40 respectively The median age of patients with percutaneous endoscopic gastrostomy procedure was 5 9 years 2 months 16 years and the mean weight was 17 7 kg 3 47 kg The median weight Z score before PEG procedure was 1 98 The follow up weight Z scores were available for 45 patients The follow up z score was 1 21 after a mean period of 18 months following the PEG procedure p= 0 0007 Major complications were observed in 3 patients 2 8 Minor complications were observed in 10 of all procedures Skin complications were observed in 13 patients 12 5 Local infections in the stoma site were the most common skin complications 7 13 Systemic infections were not observed Conclusions: Children with metabolic diseases constituted a major subgroup in our cohort This finding is an important difference of our cohort compared to other European pediatric series in which primary neurological disabilities were the main indication for PEG Despite complications PEG procedure seems to be safe for supporting children with nutritional problems Turk Arch Ped 2013; 48: 210 4
- Published
- 2014
50. Çocuk Karaciğer Nakil Alıcılarında Anemi Profili.
- Author
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SÜTÇÜ, Murat, CANTEZ, Mustafa Serdar, GERENLI, Nelgin, and DURMAZ, Özlem
- Subjects
PEDIATRIC surgery ,METABOLIC disorders ,BILIARY atresia ,ANEMIA ,LIVER transplantation ,HEPATOLOGY - Abstract
Copyright of Journal of the Child / Çocuk Dergisi is the property of Journal of Child and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2018
- Full Text
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