Person: GÜLLÜLÜ, MUSTAFA
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GÜLLÜLÜ
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MUSTAFA
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Publication Screening for fabry disease in patients who underwent renal biopsy and identification of a novel mutation(Aves, 2021-04-01) Oruç, Ayşegül; Yıldız, Abdulmecit; Akgür, Suat; Aydın, Mehmet Fethullah; Ersoy, Alparslan; Yavuz, Mahmut; Dilek, Kamil; Güllülü, Mustafa; ORUÇ, AYŞEGÜL; YILDIZ, ABDULMECİT; AKGÜR, SUAT; Aydın, Mehmet Fethullah; ERSOY, ALPARSLAN; YAVUZ, MAHMUT; DİLEK, KAMİL; GÜLLÜLÜ, MUSTAFA; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Nefroloji Anabilim Dalı.; 0000-0002-0342-9692; 0000-0002-5665-7402; AAJ-8220-2020; AAH-4002-2021; HIG-9032-2022; EJA-1761-2022; CPX-5894-2022; EHM-7377-2022; EUF-5229-2022; JGS-9425-2023Background: The X-linked Fabry disease (FD) with lysosomal storage of globotriaosylceramide (Gb3) due to alpha-galactosidase deficiency contributes to nephropathy consisting of proteinuria and renal failure eventually. Early initiation of the enzyme replacement therapy promises favorable renal outcomes. With the importance of early diagnosis, we screened FD among proteinuric patients in whom biopsy findings revealed Fabry nephropathy.Methods: Patients with light microscopic biopsy findings of vacuolated cells, focal and/or segmental glomerular sclerosis, tubular atrophy, and interstitial fibrosis were not associated with particular etiology, the presence of acro-paresthesia, angiokeratomas, and cornea verticillata, stroke history younger than 50 years, family history of renal failure with no cardiovascular risk factors were screened. Fifty-three of 308 consecutive adult patients (45.34 +/- 15.23 years old, 60.1% male) who underwent renal biopsy because of proteinuria were enrolled in the study. Screening for FD was performed by assessing alpha-Gal A activity in dried blood spots (DBS) for males and by genetic testing for females.Results: Fifty-three patients (39.94 +/- 11.97 years, 69.8% male) who underwent renal biopsy were screened. Laboratory findings revealed mean serum creatinine of 1.44 +/- 1.06 mg/dL, mean estimated glomerular filtration rate of 78.31 +/- 39.89 mL/min/1.73 m(2), and mean proteinuria of 4.32 +/- 3 g/day, whereas the females genetic screening was negative. Two of 37 males had low enzyme activity (<0.1 micmol/L/h) and confirmed FD by genetic analysis in whom one had a novel mutation of GLA gene (c.(1047G>A) p.(Trp349*)).Conclusion: It is worth noting that FD screening in patients with proteinuria, in whom vacuolated cells, mesangial expansion, glomerulosclerosis, interstitial fibrosis, and tubular atrophy of unknown etiology, are present in the renal biopsy either with or without a family history of kidney disease.Publication Trends of primary glomerular disease in Turkey from 2009 to 2017: A regional registry report from tsn-gold working group(Oxford Univ, 2020-06-01) Gül, Cuma Bülent; Küçük, Mehmet; Öztürk, Savaş; Demir, Erol; Eren, Necmi; Süönü, Abdullah; Seyahi, Nurhan; Dede, Fatih; Derici, Ülver; Koç, Yener; Şahin, Garip; Oymak, Oktay; Şahin, Gulizar Manga; Tatar, Erhan; Dursun, Belda; Dheir, Hamad; Apaydın, Suheyla; Süleymanlar, Gültekin; Ulu, Memnune Sena; Altunören, Orçun; Kutlay, Sim; Meşe, Meral; Şahin, İdris; Üstündağ, Sedat; Türkmen, Kultiğin; Yılmaz, Mehmet Emin; Kazancıoğlu, Rümeyza Turan; Uzun, Özcan; Candan, Ferhan; Aydın, Zeki; Oygar, Deren; Aktaş, Nimet; Erdem, Yunus; Paydaş, Saime; Taymez, Dilek Güven; Can, Başak; Kiykim, Ahmet; Koç, Leyla; Sezer, Siren; Duranay, Murat; Bardak, Simge; Altıntepe, Lüftullah; Kaya, Burcu; Azak, Alper; Ecder, Sabahat Alışır; Cavdar, Caner; Selçuk, Nedim Yılmaz; Güllülü, Mustafa; GÜLLÜLÜ, MUSTAFA; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Nefroloji Anabilim Dalı.; CTG-8811-2022Publication Characteristics of primary glomerular diseases patients with hematuria in Turkey: the data from TSN-GOLD Working Group(Springer, 2020-10-27) Şumnu, Abdullah; Türkmen, Kültigin; Cebeci, Egemen; Türkmen, Aydın; Eren, Necmi; Seyahi, Nurhan; Oruç, Ayşegül; Dede, Fatih; Derici, Ülver; Baştürk, Taner; Şahin, Garip; Sipahioğlu, Murat; Şahin, Gülizar Manga; Tatar, Erhan; Dursun, Belda; Sipahi, Savaş; Yılmaz, Mürvet; Süleymanlar, Gültekin; Ulu, Sena; Güngör, Özkan; Kutlay, Sim; Bahçebaşı, Zerrin Bicik; Şahin, İdris; Kurultak, İlhan; Sevinç, Can; Yılmaz, Zülfikar; Kazancıoğlu, Rümeyza Turan; Çavdar, Caner; Candan, Ferhan; Aydın, Zeki; Oygar, Deren; Gül, Bülent; Altun, Bülent; Paydaş, Saime; Uzun, Sami; İstemihan, Zülal; Ergül, Metin; Dinçer, Mevlüt Tamer; Güllulü, Mustafa; Pişkinpaşa, Serhan; Akçay, Ömer Faruk; Ünsal, Abdulkadir; Koyuncu, Sümeyra; Gök, Mahmut; Öztürk, Savaş; ORUÇ, AYŞEGÜL; GÜLLÜLÜ, MUSTAFA; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Nefroloji Anabilim Dalı.; 0000-0002-0342-9692; AAH-4002-2021; CTG-8811-2022Purpose Hematuria is one of the most common laboratory findings in nephrology practice. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular disease (PGD) patients with hematuria in our country. Methods Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. Demographic characteristics, laboratory and biopsy findings were also recorded. Results Data of 3394 PGD patients were included in the study. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Patients with hematuria had statistically higher systolic blood pressure, serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria. However, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-h proteinuria, serum total, HDL and LDL cholesterol, and C3 levels when compared with patients without hematuria. Hematuria was present 609 of 1733 patients (35.8%) among the patients presenting with nephrotic syndrome, while it was presented in 1090 of 1661 (64.2%) patients in non-nephrotics (p < 0.001). Conclusion This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.Publication Is the severity of glomerular igg staining in patient with iga nephropathy useful for predicting poor renal prognosis? The data from Tsn-gold working group(Oxford University Press, 2020-06-01) Turgutalp, Kenan; Cebeci, Egemen; Türkmen, Aydin; Derici, Ülver; Seyahi, Nurhan; Eren, Necmi; Dede, Fatih; Güllülü, Mustafa; Baştürk, Taner; Şahin, Gülizar Manga; Yılmaz, Mürvet; Sipahi, Savaş; Şahin, Garip; Ulu, Memnune Sena; Tatar, Erhan; Gündoğdu, Ali; Kazancıoğlu, Rümeyza Turan; Sevinç, Can; Güngör, Özkan; Şahin, İdris; Kutlay, Sim; Kurultak, İlhan; Aydın, Zeki; Altun, Bülent; Dursun, Belda; Yılmaz, Zülfikar; Uzun, Özcan; Süleymanlar, Gültekin; Candan, Ferhan; Sezer, Siren; Tanburoğlu, Derya Başak; Bahçebaşı, Zerrin Bicik; Taymez, Dilek Güven; Oygar, Deren; Akçalı, Esra; İstemihan, Zulal; Akçay, Ömer Faruk; Dinçer, Mevlüt Tamer; Ergül, Metin; Yenigün, Ezgi; Türkmen, Kültigin; Öztürk, Savaş; GÜLLÜLÜ, MUSTAFA; Bursa Uludağ Üniversitesi/Tıp Fakültesi.; CTG-8811-2022Publication The relationship between glomerular igg staining and poor prognostic findings in patients with iga nephropathy: The data from tsn-gold working group(BMC, 2021-10-28) Turgutalp, Kenan; Cebeci, Egemen; Turkmen, Aydin; Derici, Ulver; Seyahi, Nurhan; Eren, Necmi; Dede, Fatih; Basturk, Taner; Sahin, Gulizar Manga; Yilmaz, Murvet; Sipahi, Savas; Sahin, Garip; Ulu, Sena; Tatar, Erhan; Gundogdu, Ali; Kazancioglu, Rumeyza Turan; Sevinc, Can; Gungor, Ozkan; Sahin, Idris; Kutlay, Sim; Kurultak, Ilhan; Aydin, Zeki; Altun, Bulent; Dursun, Belda; Yilmaz, Zulfikar; Uzun, Ozcan; Suleymanlar, Gultekin; Candan, Ferhan; Sezer, Siren; Tanburoglu, Derya Basak; Bahcebasi, Zerrin Bicik; Taymez, Dilek; Akcali, Esra; Oygar, Deren; Istemihan, Zulal; Bardak, Simge; Akcay, Omer Faruk; Dincer, Mevlut Tamer; Dervisoglu, Erkan; Yenigun, Ezgi; Turkmen, Kultigin; Ozturk, Savas; Güllülü, Mustafa; GÜLLÜLÜ, MUSTAFA; Bursa Uludağ Üniversitesi/Tıp Fakültesi.; 0000-0003-0245-1844; 0000-0001-7427-618X; 0000-0002-2708-3470; 0000-0002-0409-2710; 0000-0001-6829-121X; 0000-0002-5068-4231; 0000-0002-9594-4121; 0000-0002-4069-9181; 0000-0002-8683-3737; 0000-0001-5607-1375; 0000-0002-6648-6053; 0000-0002-4017-0640; 0000-0002-6851-4871; 0000-0002-0961-3810; P-9618-2015; HKV-4703-2023; GRX-9954-2022; V-1616-2019; AAA-8435-2020; AFR-4244-2022; JYQ-2550-2024; S-7661-2016; AAS-4390-2020; Q-6320-2019; R-1388-2018Background Galactose-deficient IgA1 (Gd-IgA1) has an increased tendency to form immunocomplexes with IgG in the serum, contributing to IgAN pathogenesis by accumulating in the glomerular mesangium. Several studies showed that glomerular IgG deposition in IgAN is an important cause of mesangial proliferation and glomerular damage. This study aims to determine the association of the positivity of IgG and the intensity of IgG staining with a poor renal prognosis. Methods A total of 943 IgAN patients were included in the study. Glomerular IgG staining negative and positive patients were compared using Oxford classification scores, histopathological evaluations, proteinuria, eGFR, albumin, blood pressures. IgG positive patients were classified as (+), (++), (+++) based on their staining intensity, and the association with the prognostic criteria was also evaluated. Results 81% (n = 764) of the patients were detected as IgG negative, while 19% (n = 179) were positive. Age, gender, body mass index, blood pressure, proteinuria, eGFR, uric acid values were similar in IgG positive and negative patients who underwent biopsy (p > 0.05). Intensity of glomerular IgG positivity was not found to be associated with diastolic and systolic blood pressure, urea, uric acid, age, eGFR, albumin, proteinuria (p > 0.05 for all, r = - 0.084, r = - 0.102, r = - 0.006, r = 0.062, r = 0.014, r = - 0.044, r = - 0.061, r = - 0.066, r = 0.150, respectively). There was no difference for histopathological findings between IgG (+), IgG (++), IgG (+++) groups (for all, p > 0.05). Conclusion Glomerular IgG negativity and positivity detected by routine IFM in IgAN patients is not associated with poor renal prognostic risk factors.