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Article: Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis.

Boglou, Panagiotis / Papanas, Nikolaos / Oikonomou, Anastasia / Bakali, Stamatia / Steiropoulos, Paschalis

2016 Volume 2016, Page(s) 8708251

Abstract: Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect ... ...

Abstract	Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.
Language	English
Publishing date	2016-08-15
Publishing country	United States
Document type	Journal Article
ZDB-ID	2502642-2
ISSN	1687-9635 ; 1687-9627
ISSN (online)	1687-9635
ISSN	1687-9627
DOI	10.1155/2016/8708251
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Necrotizing sarcoid granulomatosis: A distinctive form of pulmonary granulomatous disease.

Karpathiou, Georgia / Batistatou, Anna / Boglou, Panagiotis / Stefanou, Dimitrios / Froudarakis, Marios E

The clinical respiratory journal

2017 Volume 12, Issue 4, Page(s) 1313–1319

Abstract: Objectives: To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognised by pulmonologists and pathologists.: Data source: PubMed was searched for the term necrotising or necrotizing ... ...

Abstract	Objectives: To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognised by pulmonologists and pathologists. Data source: PubMed was searched for the term necrotising or necrotizing sarcoid granulomatosis. Study selection: All cases reported in the English literature were included. Results: NGS is presented at all ages (range 8-68 years) with a median age of 42 years old. It shows female (62%) and Caucasian (80%) predominance. The most frequent symptoms are cough, fever, dyspnoea and chest pain. Extra-pulmonary involvement is found in one third of the cases, with ocular being the most common (12.5%). At imaging, multiple nodules (64.75%) or a solitary mass (20.49%) are found accompanied by mediastinal lymphadenopathy at one third of the cases. It can be clinically mistaken for malignancy as it is tumour-like, increases rapidly in size and it is hyperfixating in PET-SCAN. Histologically, NGS is defined by large areas of necrosis, well-formed granulomas and vascularitis. Conclusion: NGS is a disease often confounded clinically with malignancy or with sarcoidosis even histologically when all criteria are not strictly applied. This review provides NGS' characteristics and discusses its differential diagnosis form sarcoidosis, Wegener granulomatosis and tuberculosis.
MeSH term(s)	Biopsy ; Diagnosis, Differential ; Granulomatosis with Polyangiitis/diagnosis ; Humans ; Lung/pathology ; Necrosis/diagnosis ; Sarcoidosis, Pulmonary/diagnosis
Language	English
Publishing date	2017-08-13
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2442214-9
ISSN	1752-699X ; 1752-6981
ISSN (online)	1752-699X
ISSN	1752-6981
DOI	10.1111/crj.12673
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Article ; Online: Hypoglycaemia due to interaction of glimepiride with isoniazid in a patient with type 2 diabetes mellitus.

Boglou, Panagiotis / Steiropoulos, Paschalis / Papanas, Nikolaos / Bouros, Demosthenes

BMJ case reports

2013 Volume 2013

Abstract: Hypoglycaemia is a well-recognised untoward effect of sulfonylureas. We report a case of severe hypoglycaemia after isoniazid initiation in a type 2 diabetic patient. An oral glucose tolerance test revealed high serum insulin and C peptide, suggesting ... ...

Abstract	Hypoglycaemia is a well-recognised untoward effect of sulfonylureas. We report a case of severe hypoglycaemia after isoniazid initiation in a type 2 diabetic patient. An oral glucose tolerance test revealed high serum insulin and C peptide, suggesting hyperinsulinaemia, and it was used to ascertain the relationship between insulin, glucose and C peptide levels. Insulin and C peptide elevation was attributed to the interaction between the two drugs. As a cytochrome inhibitor, isoniazid increased serum glimepiride concentration, resulting in hyperinsulinaemia. The diagnosis of occult insulinoma or nesidioblastosis was ruled out by CT and MRI, as we could not perform more sensitive, still invasive, diagnostic procedures. After isoniazid withdrawal, hypoglycaemia regressed and glimepiride was reinitiated. In conclusion, this case illustrates the need of caution when prescribing isoniazid in patients with type 2 diabetes mellitus receiving glimepiride to avoid hypoglycaemia.
MeSH term(s)	Aged ; Antitubercular Agents/adverse effects ; Diabetes Mellitus, Type 2/complications ; Diabetes Mellitus, Type 2/drug therapy ; Drug Interactions ; Female ; Humans ; Hypoglycemia/chemically induced ; Hypoglycemic Agents/adverse effects ; Isoniazid/adverse effects ; Latent Tuberculosis/complications ; Latent Tuberculosis/drug therapy ; Sulfonylurea Compounds/adverse effects ; Withholding Treatment
Chemical Substances	Antitubercular Agents ; Hypoglycemic Agents ; Sulfonylurea Compounds ; glimepiride (6KY687524K) ; Isoniazid (V83O1VOZ8L)
Language	English
Publishing date	2013-04-16
Publishing country	England
Document type	Case Reports ; Journal Article
ISSN	1757-790X
ISSN (online)	1757-790X
DOI	10.1136/bcr-2012-008528
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Article ; Online: Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

Panagiotis Boglou / Nikolaos Papanas / Anastasia Oikonomou / Stamatia Bakali / Paschalis Steiropoulos

Case Reports in Medicine, Vol

2016 Volume 2016

Abstract	Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.
Keywords	Medicine ; R
Subject code	610
Language	English
Publishing date	2016-01-01T00:00:00Z
Publisher	Hindawi Limited
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: Idiopathic pulmonary hemosiderosis in adults: a case report and review of the literature.

Tzouvelekis, Argyris / Ntolios, Paschalis / Oikonomou, Anastasia / Koutsopoulos, Anastasios / Sivridis, Efthimios / Zacharis, George / Kaltsas, Kostantinos / Boglou, Panagiotis / Mikroulis, Dimitrios / Bouros, Demosthenes

Case reports in medicine

2012 Volume 2012, Page(s) 267857

Abstract: Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon ... ...

Abstract	Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon exclusion of all other possible causes of DAH in combination with specific pathologic findings revealing bland alveolar haemorrhage with absence of vasculitis and/or accumulation of immune complexes within lung parenchyma. Here we describe a rare case of idiopathic pulmonary hemosiderosis in an otherwise healthy 27-year-old Greek male patient with relapsing episodes of fever accompanied by general fatigue and discomfort. He was at this time point a light smoker and had been hospitalised once in the past for similar symptoms. His iron deficiency anemia coupled with chest high-resolution computed tomography and bronchoalveolar lavage revealed findings compatible with diffuse alveolar hemorrhage. After excluding all other sources of bleeding through extensive gastrointestinal workup and thorough immunologic profile, video-assisted thoracic lung biopsy was performed and the diagnosis of Idiopathic Pulmonary Hemosiderosis was established. Patient was treated with high doses of oral corticosteroids, leading to clinical response. We highlight the need for vigilance by the respiratory physician for the presence of DAH, a challenging, acute condition requiring early recognition along with identification of the underlying syndrome and appropriate treatment to achieve optimal results.
Language	English
Publishing date	2012-07-18
Publishing country	United States
Document type	Journal Article
ZDB-ID	2502642-2
ISSN	1687-9635 ; 1687-9627
ISSN (online)	1687-9635
ISSN	1687-9627
DOI	10.1155/2012/267857
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Article ; Online: Idiopathic Pulmonary Hemosiderosis in Adults

Argyris Tzouvelekis / Paschalis Ntolios / Anastasia Oikonomou / Anastasios Koutsopoulos / Efthimios Sivridis / George Zacharis / Kostantinos Kaltsas / Panagiotis Boglou / Dimitrios Mikroulis / Demosthenes Bouros

Case Reports in Medicine, Vol

A Case Report and Review of the Literature

2012 Volume 2012

Abstract	Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon exclusion of all other possible causes of DAH in combination with specific pathologic findings revealing bland alveolar haemorrhage with absence of vasculitis and/or accumulation of immune complexes within lung parenchyma. Here we describe a rare case of idiopathic pulmonary hemosiderosis in an otherwise healthy 27-year-old Greek male patient with relapsing episodes of fever accompanied by general fatigue and discomfort. He was at this time point a light smoker and had been hospitalised once in the past for similar symptoms. His iron deficiency anemia coupled with chest high-resolution computed tomography and bronchoalveolar lavage revealed findings compatible with diffuse alveolar hemorrhage. After excluding all other sources of bleeding through extensive gastrointestinal workup and thorough immunologic profile, video-assisted thoracic lung biopsy was performed and the diagnosis of Idiopathic Pulmonary Hemosiderosis was established. Patient was treated with high doses of oral corticosteroids, leading to clinical response. We highlight the need for vigilance by the respiratory physician for the presence of DAH, a challenging, acute condition requiring early recognition along with identification of the underlying syndrome and appropriate treatment to achieve optimal results.
Keywords	Medicine ; R
Subject code	610
Language	English
Publishing date	2012-01-01T00:00:00Z
Publisher	Hindawi Limited
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: Increased expression of epidermal growth factor receptor (EGF-R) in patients with different forms of lung fibrosis.

Tzouvelekis, Argyris / Ntolios, Paschalis / Karameris, Andreas / Vilaras, George / Boglou, Panagiotis / Koulelidis, Andreas / Archontogeorgis, Kostas / Kaltsas, Konstantinos / Zacharis, George / Sarikloglou, Evangelia / Steiropoulos, Paschalis / Mikroulis, Dimitrios / Koutsopoulos, Anastasios / Froudarakis, Marios / Bouros, Demosthenes

BioMed research international

2013 Volume 2013, Page(s) 654354

Abstract: Introduction: Emerging evidence supports the role of epidermal growth factor-receptor (EGFR) in fibrogenesis. The aim of our study was to investigate the expression profiles of EGFR in three forms of IIPs, including idiopathic pulmonary fibrosis (IPF), ... ...

Abstract	Introduction: Emerging evidence supports the role of epidermal growth factor-receptor (EGFR) in fibrogenesis. The aim of our study was to investigate the expression profiles of EGFR in three forms of IIPs, including idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), and nonspecific interstitial pneumonia (NSIP). Patients and methods: Twenty newly diagnosed patients with IPF, 15 with COP, and 15 with NSIP (cellular, n = 4 and fibrotic, n = 11) were investigated. Fifteen paraffin blocks obtained from the normal part of lungs removed for benign lesions were used as controls. Immunohistochemistry was carried out using specific monoclonal antibody. Results were verified by qRT-PCR. Results: A significant EGFR upregulation, both in protein and mRNA level, was observed in IPF, COP, and fibrotic NSIP samples compared to controls. EGFR was primarily localized in the hyperplastic alveolar epithelium surrounding areas of fibrosis in IPF, COP, and fibrotic NSIP samples, as assessed by double immunohistochemistry analysis with surfactant protein-A. EGFR mRNA levels were positively associated with indicators of lung fibrosis (type 1 collagen mRNA levels) and negatively correlated with functional prognostic parameters. Conclusions: We conclude that EGFR is upregulated in the hyperplastic alveolar epithelium in all three fibrotic forms of IIPs indicating a potential role during abnormal reepithelization.
MeSH term(s)	Adult ; Aged ; Aged, 80 and over ; Cryptogenic Organizing Pneumonia/diagnosis ; Cryptogenic Organizing Pneumonia/metabolism ; Cryptogenic Organizing Pneumonia/pathology ; ErbB Receptors/biosynthesis ; ErbB Receptors/metabolism ; Female ; Humans ; Idiopathic Pulmonary Fibrosis/diagnosis ; Idiopathic Pulmonary Fibrosis/metabolism ; Idiopathic Pulmonary Fibrosis/pathology ; Immunohistochemistry ; Lung/pathology ; Male ; Middle Aged ; Prognosis ; Pulmonary Alveoli/metabolism ; Pulmonary Alveoli/pathology ; Respiratory Mucosa/metabolism ; Respiratory Mucosa/pathology ; Transcriptome
Chemical Substances	ErbB Receptors (EC 2.7.10.1)
Language	English
Publishing date	2013-06-09
Publishing country	United States
Document type	Journal Article
ZDB-ID	2698540-8
ISSN	2314-6141 ; 2314-6133
ISSN (online)	2314-6141
ISSN	2314-6133
DOI	10.1155/2013/654354
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Article ; Online: Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema.

Tzouvelekis, Argyris / Zacharis, George / Oikonomou, Anastasia / Mikroulis, Dimitrios / Margaritopoulos, George / Koutsopoulos, Anastasios / Antoniadis, Antonis / Koulelidis, Andreas / Steiropoulos, Paschalis / Boglou, Panagiotis / Bakali, Matina / Froudarakis, Marios / Bouros, Demosthenes

BMC pulmonary medicine

2013 Volume 13, Page(s) 31

Abstract: Background: Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The aim of our study was to investigate the incidence of autoimmune markers ... ...

Abstract	Background: Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The aim of our study was to investigate the incidence of autoimmune markers in patients with CPFE. Methods: In this multicenter study we retrospectively evaluated records from patients with CPFE (n=40) and IPF (n=60) without emphysema. Baseline demographic characteristics, high-resolution computed tomography (HRCT), spirometry, histopathological, treatment, serum immunologic and survival data were investigated. B cell presence was estimated with CD20 immunostaining in representative lung biopsy samples from CPFE patients and control subjects. Results: A statistically significant increased number of CPFE patients with elevated serum ANA with or without positive p-ANCA titers compared to patients with IPF without emphysema was observed. Patients with CPFE and positive autoimmune markers exhibited improved survival compared to patients with a negative autoimmune profile. A massive infiltration of clusters of CD20+ B cells forming lymphoid follicles within the fibrotic lung in CPFE patients with positive serum immunologic profile compared to patients with negative profile, was noted and positively correlated with improved survival. Conclusions: A significant proportion of patients with CPFE may present with underlying auto-immune disorders that may reside insidiously and be associated with favorable prognosis. Early identification of these patients using a panel of auto-antibodies may lead to more targeted and effective therapeutic applications.
MeSH term(s)	Adult ; Aged ; Antibodies, Anti-Idiotypic/blood ; Antibodies, Antineutrophil Cytoplasmic/blood ; Antigens, CD20/metabolism ; Autoimmunity/physiology ; Biomarkers/blood ; Biopsy ; Case-Control Studies ; Comorbidity ; Emphysema/epidemiology ; Emphysema/immunology ; Emphysema/mortality ; Female ; Humans ; Kaplan-Meier Estimate ; Lung/metabolism ; Lung/pathology ; Male ; Middle Aged ; Pulmonary Fibrosis/epidemiology ; Pulmonary Fibrosis/immunology ; Pulmonary Fibrosis/mortality ; Retrospective Studies
Chemical Substances	Antibodies, Anti-Idiotypic ; Antibodies, Antineutrophil Cytoplasmic ; Antigens, CD20 ; Biomarkers
Language	English
Publishing date	2013-05-22
Publishing country	England
Document type	Journal Article ; Multicenter Study
ZDB-ID	2059871-3
ISSN	1471-2466 ; 1471-2466
ISSN (online)	1471-2466
ISSN	1471-2466
DOI	10.1186/1471-2466-13-31
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Article ; Online: Fat embolism due to bilateral femoral fracture: a case report.

Porpodis, Konstantinos / Karanikas, Michael / Zarogoulidis, Paul / Konoglou, Maria / Domvri, Kalliopi / Mitrakas, Alexandros / Boglou, Panagiotis / Bakali, Stamatia / Iordanidis, Alkis / Zervas, Vasilis / Courcoutsakis, Nikolaos / Katsikogiannis, Nikolaos / Zarogoulidis, Konstantinos

International journal of general medicine

2012 Volume 5, Page(s) 59–63

Abstract: Fat embolism syndrome is usually associated with surgery for large bone fractures. Symptoms usually occur within 36 hours of hospitalization after traumatic injury. We present a case with fat embolism syndrome due to femur fracture. Prompt supportive ... ...

Abstract	Fat embolism syndrome is usually associated with surgery for large bone fractures. Symptoms usually occur within 36 hours of hospitalization after traumatic injury. We present a case with fat embolism syndrome due to femur fracture. Prompt supportive treatment of the patient's respiratory system and additional pharmaceutical treatment provided the positive clinical outcome. There is no specific therapy for fat embolism syndrome; prevention, early diagnosis, and adequate symptomatic treatment are very important. Most of the studies in the last 20 years have shown that the incidence of fat embolism syndrome is reduced by early stabilization of the fractures and the risk is even further decreased with surgical correction rather than conservative management.
Language	English
Publishing date	2012-01-16
Publishing country	New Zealand
Document type	Case Reports
ZDB-ID	2452220-X
ISSN	1178-7074 ; 1178-7074
ISSN (online)	1178-7074
ISSN	1178-7074
DOI	10.2147/IJGM.S28455
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Article ; Online: Williams-Campbell syndrome: a case report.

Konoglou, Maria / Porpodis, Konstantinos / Zarogoulidis, Paul / Loridas, Nikolaos / Katsikogiannis, Nikolaos / Mitrakas, Alexandros / Zervas, Vasilis / Kontakiotis, Theodoros / Papakosta, Despoina / Boglou, Panagiotis / Bakali, Stamatia / Courcoutsakis, Nikolaos / Zarogoulidis, Konstantinos

International journal of general medicine

2012 Volume 5, Page(s) 41–44

Abstract: Introduction: Williams-Campbell syndrome, also known as bronchomalacia, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis. There have been few reports about ... ...

Abstract	Introduction: Williams-Campbell syndrome, also known as bronchomalacia, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis. There have been few reports about patients affected by saccular bronchiectasis, paracicatricial emphysema, and diminished cartilage. These are all characteristic of Williams-Campbell syndrome. Case presentation: This report presents a 57-year-old woman with progressive dyspnea, cough, sputum production, and fever. The clinical and laboratory examination revealed that the patient had a respiratory infection due to bronchiectasis caused by Williams-Campbell syndrome, which was undiagnosed in the patient until then. Conclusion: Although a rare syndrome, when patients' signs and symptoms include recurrent respiratory infections, bronchiectasis, productive cough, and dyspnea, Williams-Campbell syndrome should be included in the differential diagnosis.
Language	English
Publishing date	2012-01-11
Publishing country	New Zealand
Document type	Case Reports
ZDB-ID	2452220-X
ISSN	1178-7074 ; 1178-7074
ISSN (online)	1178-7074
ISSN	1178-7074
DOI	10.2147/IJGM.S28447
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