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1. GIMAP5 deficiency reveals a mammalian ceramide-driven longevity assurance pathway

Author

Park, Ann Y., Leney-Greene, Michael, Lynberg, Matthew, Gabrielski, Justin Q., Xu, Xijin, Schwarz, Benjamin, Zheng, Lixin, Balasubramaniyam, Arasu, Ham, Hyoungjun, Chao, Brittany, Zhang, Yu, Matthews, Helen F., Cui, Jing, Yao, Yikun, Kubo, Satoshi, Chanchu, Jean Michel, Morawski, Aaron R., Cook, Sarah A., Jiang, Ping, Ravell, Juan C., Cheng, Yan H., George, Alex, Faruqi, Aiman, Pagalilauan, Alison M., Bergerson, Jenna R. E., Ganesan, Sundar, Chauvin, Samuel D., Aluri, Jahnavi, Edwards-Hicks, Joy, Bohrnsen, Eric, Tippett, Caroline, Omar, Habib, Xu, Leilei, Butcher, Geoffrey W., Pascall, John, Karakoc-Aydiner, Elif, Kiykim, Ayca, Maecker, Holden, Tezcan, İlhan, Esenboga, Saliha, Heredia, Raul Jimenez, Akata, Deniz, Tekin, Saban, Kara, Altan, Kuloglu, Zarife, Unal, Emel, Kendirli, Tanıl, Dogu, Figen, Karabiber, Esra, Atkinson, T. Prescott, Cochet, Claude, Filhol, Odile, Bosio, Catherine M., Davis, Mark M., Lifton, Richard P., Pearce, Erika L., Daumke, Oliver, Aytekin, Caner, Şahin, Gülseren Evirgen, Aksu, Aysel Ünlüsoy, Uzel, Gulbu, Koneti Rao, V., Sari, Sinan, Dalgıç, Buket, Boztug, Kaan, Cagdas, Deniz, Haskologlu, Sule, Ikinciogullari, Aydan, Schwefel, David, Vilarinho, Silvia, Baris, Safa, Ozen, Ahmet, Su, Helen C., and Lenardo, Michael J.

Published
2024
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2. Author Correction: GIMAP5 deficiency reveals a mammalian ceramide-driven longevity assurance pathway

Author

Park, Ann Y., Leney-Greene, Michael, Lynberg, Matthew, Gabrielski, Justin Q., Xu, Xijin, Schwarz, Benjamin, Zheng, Lixin, Balasubramaniyam, Arasu, Ham, Hyoungjun, Chao, Brittany, Zhang, Yu, Matthews, Helen F., Cui, Jing, Yao, Yikun, Kubo, Satoshi, Chanchu, Jean Michel, Morawski, Aaron R., Cook, Sarah A., Jiang, Ping, Ravell, Juan C., Cheng, Yan H., George, Alex, Faruqi, Aiman, Pagalilauan, Alison M., Bergerson, Jenna R. E., Ganesan, Sundar, Chauvin, Samuel D., Aluri, Jahnavi, Edwards-Hicks, Joy, Bohrnsen, Eric, Tippett, Caroline, Omar, Habib, Xu, Leilei, Butcher, Geoffrey W., Pascall, John, Karakoc-Aydiner, Elif, Kiykim, Ayca, Maecker, Holden, Tezcan, İlhan, Esenboga, Saliha, Heredia, Raul Jimenez, Akata, Deniz, Tekin, Saban, Kara, Altan, Kuloglu, Zarife, Unal, Emel, Kendirli, Tanıl, Dogu, Figen, Karabiber, Esra, Atkinson, T. Prescott, Cochet, Claude, Filhol, Odile, Bosio, Catherine M., Davis, Mark M., Lifton, Richard P., Pearce, Erika L., Daumke, Oliver, Aytekin, Caner, Şahin, Gülseren Evirgen, Aksu, Aysel Ünlüsoy, Uzel, Gulbu, Koneti Rao, V., Sari, Sinan, Dalgıç, Buket, Boztug, Kaan, Cagdas, Deniz, Haskologlu, Sule, Ikinciogullari, Aydan, Schwefel, David, Vilarinho, Silvia, Baris, Safa, Ozen, Ahmet, Su, Helen C., and Lenardo, Michael J.

Published
2024
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6. SCYL1 variants cause a syndrome with lowγ-glutamyl-transferase cholestasis, acute liver failure, and neurodegeneration (CALFAN)

Author

Lenz, Dominic, McClean, Patricia, Kansu, Aydan, Bonnen, Penelope E., Ranucci, Giusy, Thiel, Christian, Straub, Beate K., Harting, Inga, Alhaddad, Bader, Dimitrov, Bianca, Kotzaeridou, Urania, Wenning, Daniel, Iorio, Raffaele, Himes, Ryan W., Kuloğlu, Zarife, Blakely, Emma L., Taylor, Robert W., Meitinger, Thomas, Kölker, Stefan, Prokisch, Holger, Hoffmann, Georg F., Haack, Tobias B., and Staufner, Christian

Published
2018
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7. Intestinal Failure and Aberrant Lipid Metabolism in Patients With DGAT1 Deficiency

Author

van Rijn, Jorik M., Ardy, Rico Chandra, Kuloğlu, Zarife, Härter, Bettina, van Haaften-Visser, Désirée Y., van der Doef, Hubert P.J., van Hoesel, Marliek, Kansu, Aydan, van Vugt, Anke H.M., Thian, Marini, Kokke, Freddy T.M., Krolo, Ana, Başaran, Meryem Keçeli, Kaya, Neslihan Gurcan, Aksu, Aysel Ünlüsoy, Dalgıç, Buket, Ozcay, Figen, Baris, Zeren, Kain, Renate, Stigter, Edwin C.A., Lichtenbelt, Klaske D., Massink, Maarten P.G., Duran, Karen J., Verheij, Joke B.G.M, Lugtenberg, Dorien, Nikkels, Peter G.J., Brouwer, Henricus G.F., Verkade, Henkjan J., Scheenstra, René, Spee, Bart, Nieuwenhuis, Edward E.S., Coffer, Paul J., Janecke, Andreas R., van Haaften, Gijs, Houwen, Roderick H.J., Müller, Thomas, Middendorp, Sabine, and Boztug, Kaan

Published
2018
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17. Early period intensive care follow-up after liver transplantation in children: a single center experience.

Author

Botan, Edin, Gün, Emrah, Güneş, Setenay Akyüzlüer, Gurbanov, Anar, Özen, Hasan, Kuloglu, Zarife, Kırsaçlıoğlu, Ceyda, Kırımker, Elvan Onur, Selvi, Özlem Can, Çiftçi, Ergin, Fitöz, Suat, Koloğlu, Meltem, Kansu, Aydan, Balcı, Deniz, and Kendirli, Tanıl

Abstract

Background. Liver transplantation (LT) is a well-established, life-saving treatment for children with irreversible acute and chronic liver failure (LF). We aimed to evaluate the factors associated with morbidity and mortality in the early period of LT in children by reviewing our pediatric intensive care unit (PICU) experience. Methods. We reviewed children's medical records followed in the PICU after LT between May 2015-August 2021, including demographic parameters, indications for LT, operative variables, respiratory and circulatory support requirements, LT-related complications and survival. Results. During this period, 40 pediatric patients who underwent LT were evaluated. LT was performed in 35 (87.5%) cases of chronic liver disease and 5 (12.5%) cases of acute liver failure. Twenty-four patients had chronic liver failure due to cholestatic liver disease. The patients' Pediatric Risk of Mortality (PRISM) III score was 18.82±SD (2-58) at PICU admission. 1-year survival was 87.5%, and overall survival was 85%. Younger age, low body weight, preoperative pediatric end-stage liver disease (PELD), and model for end-stage liver disease (MELD) values of 20 and higher were important risk factors for unfavorable outcomes after living donor liver transplantation (LDLT). These risk factors are both associated with technically more challenging vascular and bile duct reconstruction and higher complication rates, and increased mortality during the early period after LT. Conclusions. The early period of optimum PICU management in pediatric LT recipients is crucial for successful outcomes, which is also related to the patients' characteristics, disease severity scores, and surgical procedures. [ABSTRACT FROM AUTHOR]

Published
2023
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25. Transaldolase haploinsufficiency in subjects with acetaminophen‐induced liver failure

Author

Oaks, Zachary, primary, Jimah, John, additional, Grossman, Craig C., additional, Beckford, Miguel, additional, Kelly, Ryan, additional, Banerjee, Sanjay, additional, Niland, Brian, additional, Miklossy, Gabriella, additional, Kuloglu, Zarife, additional, Kansu, Aydan, additional, Lee, William, additional, Szonyi, Laszlo, additional, Banki, Katalin, additional, and Perl, Andras, additional

Published
2020
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27. Allogeneic hematopoietic stem cell and liver transplantation in a young girl with dedicator of cytokinesis 8 protein deficiency

Author

Kuloglu, Zarife, primary, Balcı, Deniz, additional, Haskoloğlu, Zehra Şule, additional, Kendirli, Tanıl, additional, Bingöl‐Koloğlu, Meltem, additional, Tuna‐Kırsaçlıoğlu, Ceyda, additional, Bal, Sevgi, additional, Selbuz, Suna, additional, Kırımker, Onur, additional, Savaş, Berna, additional, Altuntaş, Cansu, additional, Güner, Şükrü Nail, additional, Can, Özlem Selvi, additional, Karayalçın, Kaan, additional, Doğu, Figen, additional, Kansu Tanca, Aydan, additional, and İkincioğulları, Aydan, additional

Published
2019
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29. Treatment of Childhood Functional Constipation: Comparison of Senna, Trimebutine and Lactulose.

Author

DEMIR, Arzu Meltem, KULOGLU, Zarife, and KANSU, Aydan

Subjects
*LACTULOSE, *COGNITIVE consistency
Abstract

Objective: Data for the drugs used in maintenance therapy of functional constipation is limited in childhood. We aimed to evaluate the efficacy of senna, trimebutine, and lactulose in children with functional constipation (FC) or functional fecal incontinence (FFI). Material and Methods: Patients (2-18 years old) diagnosed as FC or FFI according to Rome III criteria who were treated with lactulose (n=36), senna (n=29), and trimebutine (n=33)were included. Patients completed a 0-5 Likert scale questionnaire about their symptoms. Stool consistency was evaluated by the Bristol Stool Chart. Compliance and tolerance to treatment and side effects were recorded. Clinical improvement and pharmacoeconomic evaluation were performed. Results: Clinical improvement in the senna group was significantly higher (p=0.036). Senna showed the highest improvement in stool consistency (p<0.001) and decreased fecal incontinence (p=0.039). Abdominal distention, abdominal pain, nausea/vomiting, and anal fissure were improved by trimebutine (p<0.05). Both senna and trimebutine decreased painful defecation and stool withholding. Lactulose improved anorexia and anal fissure. Senna was the most cost-effective drug. Conclusion: Treatment options for constipation should be tailored according to the predominant symptom. Lactulose may be recommended as the initial treatment for children with anorexia or anal fissure, trimebutine for abdominal distention, abdominal pain, nausea/vomiting, or anal fissure, respectively. Senna may be prescribed for the cost-effectiveness with high clinical improvement and less unpleasant taste. [ABSTRACT FROM AUTHOR]

Published
2020
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30. Intestinal Failure and Aberrant Lipid Metabolism in Patients With DGAT1 Deficiency

Author

Rijn, Jorik M. van, Ardy, Rico Chandra, Kuloglu, Zarife, Haerter, Bettina, Haaften-Visser, Desiree Y. van, Doef, Hubert P.J. van der, Lugtenberg, D., Middendorp, S., Boztug, Kaan, Rijn, Jorik M. van, Ardy, Rico Chandra, Kuloglu, Zarife, Haerter, Bettina, Haaften-Visser, Desiree Y. van, Doef, Hubert P.J. van der, Lugtenberg, D., Middendorp, S., and Boztug, Kaan

Abstract

Contains fulltext : 194412.pdf (Publisher’s version ) (Open Access)

Published
2018

31. Proteinuria in a Crohn's disease patient: Answers

Author

Kurt-Sukur, Eda Didem, Özçakar, Z. Birsin, Yilmaz, Songül, Özdel, Semanur, Kuloglu, Zarife, Ekim, Mesiha, Kansu, Aydan, and Yalçinkaya, Fatos

Subjects
Crohn's disease -- Research -- Risk factors -- Development and progression -- Care and treatment, Proteinuria -- Research -- Risk factors -- Development and progression -- Complications and side effects, Gastrointestinal system -- Research, Health
Abstract

Author(s): Eda Didem Kurt-Sukur[sup.2] , Z. Birsin Özçakar[sup.2] , Songül Yilmaz[sup.2] , Semanur Özdel[sup.2] , Zarife Kuloglu[sup.3] , Mesiha Ekim[sup.2] , Aydan Kansu[sup.3] , Fatos Yalçinkaya[sup.1] Author Affiliations: (1) Division [...]

Published
2015
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33. Proteinuria in a Crohn's disease patient: Questions

Author

Kurt-Sukur, Eda Didem, Özçakar, Z. Birsin, Yilmaz, Songül, Özdel, Semanur, Kuloglu, Zarife, Ekim, Mesiha, Kansu, Aydan, and Yalçinkaya, Fatos

Subjects
Crohn's disease -- Case studies -- Patient outcomes -- Care and treatment -- Diagnosis -- Complications and side effects, Health
Abstract

Author(s): Eda Didem Kurt-Sukur[sup.1] , Z. Birsin Özçakar[sup.1] , Songül Yilmaz[sup.1] , Semanur Özdel[sup.1] , Zarife Kuloglu[sup.2] , Mesiha Ekim[sup.1] , Aydan Kansu[sup.2] , Fatos Yalçinkaya[sup.1] Author Affiliations: (1) Division [...]

Published
2015
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40. Two Cases of Unexplained Bleeding: Munchausen Syndrome by Proxy

Author

Kurt, Funda, primary, Baskan, Sevgi, additional, Bezirci, Ozdecan, additional, Canturk, Gurol, additional, Kuloglu, Zarife, additional, Orhon, Filiz Simsek, additional, Oguz, Sinan, additional, Suskan, Emine, additional, Tekin, Deniz, additional, Ulukol, Betul, additional, and Yalcinkaya, Fatos, additional

Published
2012
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45. Mesalamine Intolerance in Three Children with Crohn's Disease.

Author

Tuna Kirsaclioglu, Ceyda, Kuloglu, Zarife, Ustundag, Gonca, Kansu, aydan, İnce, Erdal, Ensari, arzu, and Girgin, Nurten

Subjects
*MESALAMINE, *INFLAMMATION, *CROHN'S disease, *BIOMARKERS, *JUVENILE diseases, *PATIENTS, *THERAPEUTICS, *ADRENOCORTICAL hormones, *C-reactive protein, *INFLAMMATORY mediators, *NONSTEROIDAL anti-inflammatory agents
Abstract

Objective: To present the mesalamine-induced acute exacerbation of symptoms and inflammatory markers in children with Crohn's disease (CD).Clinical Presentation and Intervention: Three children who presented with CD had acute exacerbation of colitis symptoms or elevated inflammatory markers when mesalamine was added to treatment while tapering/ceasing steroid treatment. While on steroid treatment, the patients maintained clinical and laboratory remission, but with the initiation of mesalamine treatment, they had abdominal pain and bloody mucoid diarrhoea and/or elevation of white blood cell count, C-reactive protein level and erythrocyte sedimentation rate. Bacterial pathogens were excluded from the urine, throat and blood cultures, parasites with stool examination, viral pathogens with serology. Within 3-7 days after the mesalamine treatment had been stopped, the patients showed improvement of colitis symptoms and normalisation of white blood cell count, C-reactive protein level and erythrocyte sedimentation rate.Conclusion: In this study mesalamine mimicked CD relapse in children with CD while tapering or after stopping steroid treatment. Awareness of this side effect of mesalamine could prevent a misdiagnosis of steroid dependency. [ABSTRACT FROM AUTHOR]

Published
2016
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48. Liver Histology of Children With Chronic Hepatitis Treated With Interferon-a Alone or in Combination With Lamivudine

Author

Kuloglu, Zarife, Krsaçloglu, Ceyda Tuna, Kansu, Aydan, Erden, Esra, and Girgin, Nurten

Abstract

To evaluate histological changes with interferon monotherapy or interferon plus lamivudine combination therapy in children with hepatitis B e antigen (HBeAg)–positive chronic hepatitis B. 31 children aged 2–13 years were randomly treated with interferon (IFN) (group 1, n = 16) or IFN plus simultaneously started lamivudine (group 2, n = 15). IFN-a2a was given 9 MU/m23 times per week for 6 months in each group; lamivudine was given 4 mg · kg-1· day-1for 24 months. Liver biopsy specimens were evaluated according to the Knodell score before therapy and after 24 months of therapy. Histological response was defined as a decrease in the histological activity index (HAI) score by at least 2 points. Efficacy of therapy was evaluated at 24 months of therapy in all children. Alanine aminotransferase normalization, HbeAg, and hepatitis B virus DNA clearance were not different. Complete response and histological response were 37.5%/62.5% and 40%/46.7% in groups 1 and 2, respectively (P= NS). At baseline and at 24 months of therapy, total HAI and components of HAI were not different in the 2 groups. In comparison with baseline, a significant decrease in scores of periportal ± bridging necrosis was observed in group 1 (P= 0.01); periportal ± bridging necrosis, intralobular degeneration, focal necrosis, and necroinflammation scores significantly decreased in group 2 (P= 0.04 and P= 0.02) at 24 months of therapy. The addition of lamivudine to IFN-a did not increase the effectiveness of the treatment in terms of complete and histological responses. Both therapies seemed to be effective in the regression of periportal ± bridging necrosis. In addition, combination therapy was also effective in the regression of intralobular degeneration, focal necrosis, and necroinflammatory activity index.

Published
2007
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49. Comparison of Two Different Regimens of Combined Interferon-a2A and Lamivudine Therapy in Children with Chronic Hepatitis B Infection

Author

Kansu, Aydan, Doganci, Tümay, Akman, Sezin A, Artan, Reha, Kuyucu, Necdet, Kalayci, Ayhan Gazi, Dikici, Bünyamin, Dalgiç, Buket, Selimoglu, Ayse, Kasirga, Erhun, Özkan, Tanju B, Kuloglu, Zarife, Aydogdu, Sema, Bosnak, Mehmet, Ertekin, Vildan, Tanir, Gönül, Haspolat, Kenan, Girgin, Nurten, and Yagci, Rasit Vural

Abstract

Aim To evaluate the efficacy of two regimens of combined interferon-a2a (IFN-a2a) and lamivudine (3TC) therapy in childhood chronic hepatitis B.Methods A total of 177 patients received IFN-a2a, 9 million units (MU)/m2for 6 months. In group I (112 patients, 8.7±3.5 years), 3TC (4 mg/kg/day, max 100 mg) was started simultaneously with IFN-a2a, in group II (65 patients, 9.6±3.8 years) 3TC was started 2 months prior to IFN-a2a. 3TC was continued for 6 months after antiHBe seroconversion or stopped at 24 months in non-responders.Results Baseline alanine aminotransferase (ALT) was 134.2 ±34.1 and 147.0 ±45.3; histological activity index (HAI) was 7.4 ±2.7 and 7.1 ±2.3; and HBV DNA levels were above 2,000 pg/ml in 76% and 66% of patients in groups I and II, respectively (P>0.005). Complete response was 55.3% and 27.6% in groups I and II, respectively (P<0.01). AntiHBe seroconversion was higher and earlier, and HBV DNA clearance was earlier in group I (P<0.05). HBsAg clearance was 12.5% and 4.6% and antiHBs seroconversion was 9.8% and 6.2% in groups I and II, respectively (P>0.05). Breakthrough occurred in 17.9% and 24.6%; breakthrough times were 15.9 ±4.6 and 14.1 ±5.1 months; and relapse rates were 6.8% and none in groups I and II, respectively (P>0.05, P>0.05, P>0.05). Responders had higher HAI (HAI>6) and higher pre-treatment ALT than non-responders.Conclusion Simultaneous 3TC+IFN-a2a yields a higher response and earlier antiHBe seroconversion and viral clearance than consecutive combined therapy. Relapse rate is low. Predictors of response are high basal ALT and high HAI scores. 3TC can be administered for 24 months without any side effect and breakthrough rate is comparable with previous studies.

Published
2006
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