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Öğe Abdominal sarcoidosis: cross-sectional imaging findings(Aves, 2015) Gezer, Naciye Sinem; Basara, Isil; Altay, Canan; Harman, Mustafa; Rocher, Laurence; Karabulut, Nevzat; Secil, MustafaSarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%-70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis.Öğe Abdominal sarcoidosis: cross-sectional imaging findings(Aves, 2015) Gezer, Naciye Sinem; Basara, Isil; Altay, Canan; Harman, Mustafa; Rocher, Laurence; Karabulut, Nevzat; Secil, MustafaSarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%-70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis.Öğe Abdominal sarcoidosis: cross-sectional imaging findings(Aves, 2015) Gezer, Naciye Sinem; Basara, Isil; Altay, Canan; Harman, Mustafa; Rocher, Laurence; Karabulut, Nevzat; Secil, MustafaSarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%-70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis.Öğe Clinical presentations, metabolic abnormalities and end-organ complications in patients with familial partial lipodystrophy(W B Saunders Co-Elsevier Inc, 2017) Akinci, Baris; Onay, Huseyin; Demir, Tevfik; Savas-Erdeve, Senay; Gen, Ramazan; Simsir, Ilgin Yildirim; Keskin, Fatma Ela; Erturk, Mehmet Sercan; Uzum, Ayse Kubat; Yaylali, Guzin Fidan; Ozdemir, Nilufer Kutbay; Atik, Tahir; Ozen, Samim; Yurekli, Banu Sarer; Apaydin, Tugce; Altay, Canan; Akinci, Gulcin; Demir, Leyla; Comlekci, Abdurrahman; Secil, Mustafa; Oral, Elif AriogluObjective. Familial partial lipodystrophy (FPLD) is a rare genetic disorder characterized by partial lack of subcutaneous fat. Methods. This multicenter prospective observational study included data from 56 subjects with FPLD (18 independent Turkish families). Thirty healthy controls were enrolled for comparison. Results. Pathogenic variants of the LMNA gene were determined in nine families. Of those, typical exon 8 codon 482 pathogenic variants were identified in four families. Analysis of the LMNA gene also revealed exon 1 codon 47, exon 5 codon 306, exon 6 codon 349, exon 9 codon 528, and exon 11 codon 582 pathogenic variants. Analysis of the PPARG gene revealed exon 3 p.Y151C pathogenic variant in two families and exon 7 p.H477L pathogenic variant in one family. A non-pathogenic exon 5 p.R215Qvariant of the LMNB2 gene was detected in another family. Five other families harbored no mutation in any of the genes sequenced. MRI studies showed slightly different fat distribution patterns among subjects with different point mutations, though it was strikingly different in subjects with LMNA p.R349W pathogenic variant. Subjects with pathogenic variants of the PPARG gene were associated with less prominent fat loss and relatively higher levels of leptin compared to those with pathogenic variants in the LMNA gene. Various metabolic abnormalities associated with insulin resistance were detected in all subjects. End-organ complications were observed. Conclusion. We have identified various pathogenic variants scattered throughout the LMNA and PPARG genes in Turkish patients with FPLD. Phenotypic heterogeneity is remarkable in patients with LMNA pathogenic variants related to the site of missense mutations. FPLD, caused by pathogenic variants either in LMNA or PPARG is associated with metabolic abnormalities associated with insulin resistance that lead to increased morbidity. (C) 2017 Elsevier Inc. All rights reserved.Öğe Determining residual adipose tissue characteristics with MRI in patients with various subtypes of lipodystrophy(Aves, 2017) Altay, Canan; Secil, Mustafa; Demir, Tevfik; Atik, Tahir; Akinci, Gulcin; Kutbay, Nilufer Ozdemir; Temeloglu, Ela Keskin; Simsir, Ilgin Yildirim; Ozisik, Secil; Demir, Leyla; Eren, Erdal; Tuna, Emine Burcin; Aytac, Hasibe; Onay, Huseyin; Akinci, BarisPURPOSE We aimed to investigate residual adipose tissue with whole-body magnetic resonance imaging to differentiate between subtypes of lipodystrophy. METHODS A total of 32 patients 12 with congenital generalized lipodystrophy [CGL], 1 with acquired generalized lipodystrophy [AGL], 12 with familial partial lipodystrophy [FPLD], and 7 with acquired partial lipodystrophy [APL]) were included. RESULTS Despite generalized loss of metabolically active adipose tissue, patients with CGL1 caused by AGPAT2 mutations had a significant amount of residual adipose tissue in the scalp, earlobes, retro-orbital region, and palms and soles. No residual adipose tissue was noted particularly in the head and neck, palms and soles in CGL2 caused by BSCL2 mutations. CGL4 caused by mutations in the PTRF gene was characterized with well-preserved retro-orbital and bone marrow fat in the absence of any visible residual adipose tissue in other areas. No residual adipose tissue was observed in AGL. Despite loss of subcutaneous fat, periarticular adipose tissue was preserved in the lower limbs of patients with FPLD. Retro-orbital adipose tissue was surprisingly preserved in APL, although they lacked head and neck fat. CONCLUSION Lipodystrophies are a heterogeneous group of disorders characterized by generalized or partial loss of adipose tissue, which can be congenital or acquired. Our results suggest that residual adipose tissue characteristics can help distinguish different subtypes of lipodystrophy.Öğe Discrimination of oncocytoma and chromophobe renal cell carcinoma using MRI(Aves, 2019) Akin, Isil Basara; Altay, Canan; Guler, Ezgi; Camlidag, Ilkay; Harman, Mustafa; Danaci, Murat; Tuna, Burcin; Yorukoglu, Kutsal; Secil, MustafaPURPOSE We aimed to evaluate magnetic resonance imaging (MRI) features, including signal intensities, enhancement patterns and T2 signal intensity ratios to differentiate oncocytoma from chromophobe renal cell carcinoma (RCC). METHODS This retrospective study included 17 patients with oncocytoma and 33 patients with chromophobe RCC who underwent dynamic MRI. Two radiologists independently reviewed images blinded to pathology. Morphologic characteristics, T1 and T2 signal intensities were reviewed. T2 signal intensities, wash-in, wash-out values, T2 signal intensity ratios were calculated. Sensitivity and specificity analyses were performed. RESULTS Mean ages of patients with oncocytoma and chromophobe RCC were 61.0 +/- 11.6 and 58.5 +/- 14.0 years, respectively. Mean tumor size was 60.6 +/- 47.3 mm for oncocytoma, 61.7 +/- 45.9 mm for chromophobe RCC. Qualitative imaging findings in conventional MRI have no distinctive feature in discrimination of two tumors. Regarding signal intensity ratios, oncocytomas were higher than chromophobe RCCs. Renal oncocytomas showed higher signal intensity ratios and wash-in values than chromophobe RCCs in all phases. Fast spin-echo T2 signal intensities were higher in oncocytomas than chromophobe RCCs. CONCLUSION Signal intensity ratios, fast spin-echo T2 signal intensities and wash-in values constitute diagnostic parameters for discriminating between oncoytomas and chromophobes. In the excretory phase of dynamic enhanced images, oncocytomas have higher signal intensity ratio than chromophobe RCC and high wash-in values strongly imply a diagnosis of renal oncocytoma.Öğe How to perform an excellent radiology board examination: a web-based checklist(Springer, 2021) Dicle, Oguz; Ozan, Sema; Sahin, Hatice; Secil, MustafaBackgroundBoard exams are now considered as means of quality procedures that aim to keep the professional knowledge and skills of the physicians at the highest level. in addition, for an assessment to be scientifically valid, it has to be done within defined standards. Although there are different sources in this field, there is a need for a resource that details the steps required for the examinations to be performed perfectly, brings descriptions of the reasons for the procedure and associates the steps with assessment standards. Experts with national and international experience both in radiology and medical education contributed to the preparation of this checklist.ResultsThe guide includes 174 elements to consider before, after the exam order and examination. From the perspective of assessment standards, it has been observed that the steps to be considered before the exam have a greater impact on the validity and reliability of the exam. The standard in which the questions are most associated was validity with 117 (67.24%) questions.ConclusionsWe think that our guide, which will be accessible in the web environment, will be useful to the teams with a development goal or just start the exam, the candidates who will take the exam and the examiners.Öğe Magnetic resonance spectroscopy to assess hepatic steatosis in patients with lipodystrophy(Aves, 2020) Altay, Canan; Secil, Mustafa; Adiyaman, Suleyman Cem; Saydam, Basak Ozgen; Demir, Tevfik; Akinci, Gulcin; Akinci, BarisBackground/Aims: Lipodystrophy is a rare metabolic disorder characterized by a near-total or partial lack of subcutaneous adipose tissue and is associated with insulin resistance. We aimed to evaluate the efficacy of magnetic resonance spectroscopy (MRS) imaging to explore the fat content of the liver in patients with lipodystrophy and to determine the relationship between liver fat accumulation and clinical presentations of lipodystrophy. Materials and Methods: Between July 2014 and February 2016, 34 patients with lipodystrophy were assessed by MRS for the quantification of hepatic steatosis. All patients had metabolic abnormalities associated with insulin resistance. Metabolic parameters and the MRS findings were analyzed to identify potential correlations between liver fat content and disease severity. Results: the MRS fat ratios (MRS-FRs) were markedly higher, indicating severe hepatic steatosis in lipodystrophy. Patients with generalized and partial lipodystrophy had comparable levels of MRS-FRs, although patients with generalized lipodystrophy were significantly younger. Patients with genetic lipodystrophy had elevated MRS-FRs compared with those with acquired lipodystrophy (p=0.042). the MRS-FR was positively correlated with liver enzyme alanine aminotransferase (p=0.028) and serum adiponectin (p=0.043). Conclusion: Our data suggest that MRS might be an effective, non-invasive imaging method to quantify hepatic fat content in patients with lipodystrophy. Further studies are needed to validate the technique and threshold values, which would allow accurate comparison of data acquired by different machines and centers.Öğe Natural History of Congenital Generalized Lipodystrophy: A Nationwide Study From Turkey(Oxford Univ Press Inc, 2016) Akinci, Bans; Onay, Huseyin; Demir, Tevfik; Ozen, Samim; Kayserili, Hulya; Akinci, Gulcin; Nur, Banu; Tuysuz, Beyhan; Ozbek, Mehmet Nun; Gungor, Adem; Simsir, Ilgin Yildirim; Altay, Canan; Demir, Leyla; Simsek, Enver; Atmaca, Murat; Topaloglu, Haluk; Bilen, Habib; Atmaca, Hulusi; Atik, Tahir; Cavdar, Umit; Altunoglu, Umut; Aslanger, Ayca; Mihci, Ercan; Secil, Mustafa; Saygili, Fusun; Comlekci, Abdurrahman; Garg, AbhimanyuContext: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by near-total lack of body fat. Objective: We aimed to study natural history and disease burden of various subtypes of CGL. Design: We attempted to ascertain nearly all patients with CGL in Turkey. Setting: This was a nationwide study. Patients or Other Participants: Participants included 33 patients (22 families) with CGL and 30 healthy controls. Main Outcome Measure(s): We wanted to ascertain genotypes by sequencing of the known genes. Whole-body magnetic resonance imaging was used to investigate the extent of fat loss. Metabolic abnormalities and end-organ complications were measured on prospective follow-up. Results: Analysis of the AGPAT2 gene revealed four previously reported and four novel mutations (CGL1; c.144C>A, c.667_705delinsCTGCG, c.268delC, and c.316 + 1G > T). Analysis of the BSCL2 gene revealed four different homozygous and one compound heterozygous possible disease-causing mutations (CGL2), including four novel mutations (c.280C > T, c. 631delG, c. 62A > T, and c. 465-468delGACT). Two homozygous PTRF mutations (c.481-482insGTGA and c. 259C > T) were identified (CGL4). Patients with CGL1 had preservation of adipose tissue in the palms, soles, scalp, and orbital region, and had relatively lower serum adiponectin levels as compared to CGL2 patients. CGL4 patients had myopathy and other distinct clinical features. All patients developed various metabolic abnormalities associated with insulin resistance. Hepatic involvement was more severe in CGL2. End-organ complications were observed at young ages. Two patients died at age 62 years from cardiovascular events. Conclusions: CGL patients from Turkey had both previously reported and novel mutations of the AGPAT2, BSCL2, and PTRF genes. Our study highlights the early onset of severe metabolic abnormalities and increased risk of end-organ complications in patients with CGL.Öğe Partial lipodystrophy of the limbs in a diabetes clinic setting(Elsevier Sci Ltd, 2016) Demir, Tevfik; Akinci, Baris; Demir, Leyla; Altay, Canan; Atik, Tahir; Cavdar, Umit; Secil, Mustafa; Comlekci, AbdurrahmanObjective: Partial lipodystrophy of the limbs (PLL) is a newly described form of lipodystrophy that is characterized by symmetrical distal lipoatrophy of the limbs and insulin resistant diabetes. Research design and methods: In this study, we prospectively screened our patients with type 2 diabetes for the presence of PLL phenotype. Metabolic parameters of PLL patients were compared to those with type 2 diabetes who applied to our diabetes clinic during the same period of time. Results: Between Sep 2013 and Mar 2015, 2020 patients with type 2 diabetes were evaluated for the presence of PLL. PLL was confirmed in 16 patients. The prevalence of PLL was calculated as 0.79% in our diabetes clinic. The most common phenotypic presentations were loss of subcutaneous fat in the forearms, calves and thighs, and loss of fat in forearms and calves. Patients with PLL had poor metabolic control and marked insulin resistance compared to subjects with type 2 diabetes. Diabetes had been diagnosed at a younger age in patients with PLL. Patients with PLL also had more atherogenic lipid profiles. Conclusions: Our data suggests that PLL is a relatively common form of lipodystrophy in diabetes clinics, which is associated with poor metabolic control and marked insulin resistance. The recognition of PLL in patients with type 2 diabetes can help better clinical management by alerting the physician to these associated co-morbidities. (C) 2015 Primary Care Diabetes Europe. Published by Elsevier Ltd. All rights reserved.Öğe A subset of patients with acquired partial lipodystrophy developing severe metabolic abnormalities(Taylor & Francis Inc, 2019) Saydam, Basak Ozgen; Sonmez, Melda; Simsir, Ilgin Yildirim; Erturk, Mehmet Sercan; Kulaksizoglu, Mustafa; Arkan, Tugba; Hekimsoy, Zeliha; Cavdar, Umit; Akinci, Gulcin; Demir, Tevfik; Altay, Canan Tuncer; Mihci, Ercan; Secil, Mustafa; Akinci, BarisPurpose/Aim of the study: Acquired partial lipodystrophy (APL) is a rare disease characterized by selective loss of adipose tissue. In this study, we aimed to present a subset of patients with APL, who developed severe metabolic abnormalities, from our national lipodystrophy registry. Materials and Methods: Severe metabolic abnormalities were defined as: poorly controlled diabetes (HbA1c above 7% despite treatment with insulin more than 1 unit/kg/day combined with oral antidiabetics), severe hypertriglyceridemia (triglycerides above 500 mg/dL despite treatment with lipid-lowering drugs), episodes of acute pancreatitis, or severe hepatic involvement (biopsy-proven non-alcoholic steatohepatitis (NASH)). Results: Among 140 patients with all forms of lipodystrophy (28 with APL), we identified 6 APL patients with severe metabolic abnormalities. The geometric mean for age was 37 years (range: 27-50 years; 4 females and 2 males). Five patients had poorly controlled diabetes despite treatment with high-dose insulin combined with oral antidiabetics. Severe hypertriglyceridemia developed in five patients, of those three experienced episodes of acute pancreatitis. Although all six patients had hepatic steatosis at various levels on imaging studies, NASH was proven in two patients on liver biopsy. Our data suggested that APL patients with severe metabolic abnormalities had a more advanced fat loss and longer disease duration. Conclusions: We suggest that these patients represent a potential subgroup of APL who may benefit from metreleptin or investigational therapies as standard treatment strategies fail to achieve a good metabolic control.
