References of "Thiry, Albert"
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See detailConcomitant nodal involvement by Langerhans Cell Histiocytosis and Hodgkin Lymphoma
Geurten, Claire ULg; THIRY, Albert ULg; Jamblin, Paul et al

Poster (2015, March 12)

Introduction : Langerhans cell histiocytosis is defined as a clonal neoplastic proliferation of myeloid dendritic cells that upon activation migrate from the mucosal to lymph nodes. Definitive diagnosis ... [more ▼]

Introduction : Langerhans cell histiocytosis is defined as a clonal neoplastic proliferation of myeloid dendritic cells that upon activation migrate from the mucosal to lymph nodes. Definitive diagnosis is made by anatomo-pathological and immunohistochemical analysis. Langerhans cell histiocytosis is rarely, yet not exceptionally, found coexisting with other malignant neoplasms, suggesting it might arise in reaction to the cytokinic secretion of malignant cells. Case : We report the case of a 10-year-old female presenting with an isolated laterocervical lymphadenopathy and a mild general condition alteration tracing back to two months earlier. Nodal biopsy was performed and revealed concomitant involvement by Langerhans cell histiocytosis and Hodgkin lymphoma. Treatment of lymphoma led to the disappearance of the whole symptomatology. Discussion : Literature beholds reports of 30 cases of the simultaneous occurence of Hodgkin lymphoma with Langerhans cell histiocytosis, which is more than fortuitous regarding the low incidence of both diseases. A common etiology could explain such an association, but it might also be possible that background inflammatory cells of Hodgkin lymphoma stimulate the proliferation of Langerhans cells, making it a reactive process when occurring simultaneously with other neoplasms. Clinicians should thus be aware of the possibility of this association and carefully exclude any other life-threatening malignant proliferation when confronted to apparently isolated Langerhans cell histiocytosis. [less ▲]

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See detailCharacteriation of GPR101 expression across different species
Trivellin, G; Bjelobaba, I; Daly, Adrian ULg et al

in Abstract book - ENDO 2015 (2015, March)

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See detailA novel mutation of the luteinizing hormone/choionic gonadotrophin receptor gene leading to Leydig cell hypoplasia type I
Potorac, Iulia ULg; Rivero-Müller, A; Pintiaux, Axelle ULg et al

in Symposium "Perspectives in Endocrinology" - 5ème édition (2015, February 07)

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See detailA novel inactivating mutation of the LH/chorionic gonadotrophin receptor with impaired membrane trafficking leading to Leydig cell hypoplasia type 1.
Rivero-Muller, Adolfo; Potorac, Iulia ULg; Pintiaux, Axelle ULg et al

in European journal of endocrinology / European Federation of Endocrine Societies (2015), 172(6), 27-36

OBJECTIVE: The LH/chorionic gonadotrophin receptor (LHCGR) is a G protein-coupled receptor (GPCR) that plays a central role in male sexual differentiation, regulation of ovarian follicular maturation ... [more ▼]

OBJECTIVE: The LH/chorionic gonadotrophin receptor (LHCGR) is a G protein-coupled receptor (GPCR) that plays a central role in male sexual differentiation, regulation of ovarian follicular maturation, ovulation and maintenance of corpus luteum and pregnancy, as well as maintenance of testicular testosterone production. Mutations in the LHCGR gene are very rare. The aim of this work was to study the clinical and molecular characteristics of a rare familial LHCGR mutation. METHODS: Five affected members of a family, including a phenotypically female, but genotypically male (46,XY), patient with Leydig cell hypoplasia type 1 and four genotypically female siblings with reproductive abnormalities, were studied genetically. Cell trafficking studies as well as signalling studies of mutated receptor were performed. RESULTS: The five affected patients were all homozygous for a novel mutation in the LHCGR gene, a deletion of guanine in position 1850 (1850delG). This resulted in a frameshift affecting most of the C-terminal intracellular domain. In vitro studies demonstrated that the 1850delG receptor was completely incapable of transit to the cell membrane, becoming trapped within the endoplasmic reticulum. This could not be rescued by small-molecule agonist treatment or stimulated intracellularly by co-expression of a yoked human chorionic gonadotrophin. CONCLUSIONS: This novel LHCGR mutation leads to complete inactivation of the LHCGR receptor due to trafficking and signalling abnormalities, which improves our understanding of the impact of the affected structural domain on receptor trafficking and function. [less ▲]

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See detailIntensity of prolactinoma on T2-weighted magnetic resonance imaging: towards another gender difference.
KREUTZ, Julie ULg; VROONEN, Laurent ULg; Cattin, Françoise et al

in Neuroradiology (2015)

INTRODUCTION: Clinical presentations of prolactinomas are quite different between genders. In comparison with women's prolactinoma, those in men showed predominance of large tumors with high prolactin ... [more ▼]

INTRODUCTION: Clinical presentations of prolactinomas are quite different between genders. In comparison with women's prolactinoma, those in men showed predominance of large tumors with high prolactin (PRL) levels. This preponderance could be attributed to a greater proliferative potential of the tumors. Differences in magnetic resonance imaging (MRI) signal at diagnosis have not been yet clearly evaluated. METHODS: We conduct a retrospective study comparing MRI signal intensity (SI) on T2-weighted images (T2-WI) between 41 men and 41 women to investigate whether or not men prolactinoma present specific features. RESULTS: In addition to the size of the adenoma and PRL levels (P < 0001), prolactinomas in men also exhibit differences from those in women in signal on T2-WI on MRI (P < 0001). Women's prolactinomas are mostly of high SI on T2-WI while men's prolactinomas exhibit a more heterogeneous pattern of SI on T2-WI. Prolactinomas presenting with low SI on T2-WI are almost exclusively encountered in men. CONCLUSIONS: Presence of T2-WI hypointensities in pituitary adenoma can be predictive of a different subtype of prolactinoma almost encountered in men and possibly translate the presence of spherical amyloid deposits, in agreement with the literature. [less ▲]

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See detailA novel mutation of the luteinizing hormone/choionic gonadotrophin receptor gene leading to Leydig cell hypoplasia type I
Potorac, Iulia ULg; Rivero-Müller, Adolfo; Pintiaux, Axelle ULg et al

in Abstract book - 24th Meeting of the Belgian Endocrine Society (2014, October 18)

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See detailMcCune-Albright Syndrome: A Detailed Pathological And Genetic Analysis of Disease Effects in an Adult Patient.
Vasilev, Vladimir; Daly, Adrian ULg; THIRY, Albert ULg et al

in The Journal of clinical endocrinology and metabolism (2014)

Context: McCune Albright syndrome (MAS) is a clinical association of endocrine and non-endocrine anomalies caused by post-zygotic mutation of the GNAS1 gene, leading to somatic activation of the ... [more ▼]

Context: McCune Albright syndrome (MAS) is a clinical association of endocrine and non-endocrine anomalies caused by post-zygotic mutation of the GNAS1 gene, leading to somatic activation of the stimulatory alpha subunit of G protein (Gsalpha). Important advances have been made recently in describing pathological characteristics of many MAS-affected tissues, particularly pituitary, testicular and adrenal disease. Other rarer disease related features are emerging. Objective: To study pathological and genetic findings of MAS on a tissue-by-tissue basis in classically and non classically affected tissues. Design: A comprehensive autopsy and genetic analysis Setting: Tertiary referral University Hospital Patients: Adult male patient with MAS and severe disease burden including gigantism Intervention(s): Clinical, hormonal and radiographic studies; gross and microscopic pathology analyses, conventional PCR and droplet digital PCR analyses of affected and non affected tissues Main Outcome Measure: Pathological findings, presence of GNAS1 mutations Results: The patient was diagnosed with MAS syndrome at six years of age based on the association of cafe-au-lait spots and radiological signs of polyostotic fibrous dysplasia. Gigantism developed and hyperprolactinemia, hypogonadotropic hypogonadism and hyperparathyroidism were diagnosed throughout adult period. The patient died at the age of 39 from pulmonary embolism. A detailed study revealed mosaiscism for the p.R201C GNAS mutation distributed across many endocrine and non-endocrine tissues. These genetically implicated tissues included rare or previously undescribed disease associations including primary hyperparathyroidism, and hyperplasia of the thymus and endocrine pancreas. Conclusions: This comprehensive pathological study of a single patient highlights the complex clinical profile of MAS and illustrates important advances in understanding the characteristics of somatic GNAS1 related pathology across a wide range of affected organs. [less ▲]

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See detailReceptor expression in craniopharyngiomas causing tumor growth in pregnancy: case report and review of the literature
VROONEN, Laurent ULg; TOME, Monica; THIRY, Albert ULg et al

in Abstract book : 57èmes Journées Internationales d'Endocrinologie Clinique - Henri-Pierre Klotz (2014, June)

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See detailReceptor expression in craniopharyngiomas causing tumor growth in pregnancy : case report and review of the literature
VROONEN, Laurent ULg; Tome Garcia, M; THIRY, Albert ULg et al

in Abstract book - Symposium "Perspectives in Endocrinology" Congresses Highlights 2013:ECE Copenhagen, ENDO SF, SFE Paris (2014, February)

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See detailA clinically novel AIP mutation in a patient with a very large, apparently sporadic somatotrope adenoma.
Salvatori, Roberto; Daly, Adrian ULg; Quinones-Hinojosa, Alfredo et al

in Endocrinology, diabetes & metabolism case reports (2014), 2014

Heterozygous germline inactivating mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene lead to pituitary adenomas that most frequently present in the setting of familial isolated ... [more ▼]

Heterozygous germline inactivating mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene lead to pituitary adenomas that most frequently present in the setting of familial isolated pituitary adenoma syndrome, usually as somatotropinomas and prolactinomas. More recently, they have been found in a significant percentage of young patients presenting with pituitary macroadenoma without any apparent family history. We describe the case of a 19-year-old man who presented with a gigantic somatotropinoma. His family history was negative. His peripheral DNA showed a heterozygous AIP mutation (p.I13N), while tumor tissue only had the mutated allele, showing loss of heterozygosity (LOH) and suggesting that the mutation caused the disease. LEARNING POINTS: AIP mutations may be observed in sporadic somatotrope adenomas occurring in young patients.LOH is a strong indicator that an AIP variant is disease causing.Somatotrope adenomas in carriers of AIP mutations are generally larger and more difficult to cure. [less ▲]

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See detailReceptor expression in craniopharyngiomas causing tumor growth in pregnancy : case report and review of the literature
Tome, M; VROONEN, Laurent ULg; THIRY, Albert ULg et al

in Annales d'Endocrinologie (2013, October), 74

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See detailReceptor expression in craniopharyngiomas causing tumor growth in pregnancy : case report and review of the literature
Tome, Monica; VROONEN, Laurent ULg; THIRY, Albert ULg et al

in Endocrine Abstracts - 15th European Congress of Endocrinology (2013, May)

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See detailCharacterization of spontaneous bone marrow recovery after sublethal total body irradiation: importance of the osteoblastic/adipocytic balance.
Poncin, Géraldine ULg; Beaulieu, Aurore ULg; Humblet, Chantal ULg et al

in PLoS ONE (2012), 7(2), 30818

Many studies have already examined the hematopoietic recovery after irradiation but paid with very little attention to the bone marrow microenvironment. Nonetheless previous studies in a murine model of ... [more ▼]

Many studies have already examined the hematopoietic recovery after irradiation but paid with very little attention to the bone marrow microenvironment. Nonetheless previous studies in a murine model of reversible radio-induced bone marrow aplasia have shown a significant increase in alkaline phosphatase activity (ALP) prior to hematopoietic regeneration. This increase in ALP activity was not due to cell proliferation but could be attributed to modifications of the properties of mesenchymal stem cells (MSC). We thus undertook a study to assess the kinetics of the evolution of MSC correlated to their hematopoietic supportive capacities in mice treated with sub lethal total body irradiation. In our study, colony-forming units - fibroblasts (CFU-Fs) assay showed a significant MSC rate increase in irradiated bone marrows. CFU-Fs colonies still possessed differentiation capacities of MSC but colonies from mice sacrificed 3 days after irradiation displayed high rates of ALP activity and a transient increase in osteoblastic markers expression while ppargamma and neuropilin-1 decreased. Hematopoietic supportive capacities of CFU-Fs were also modified: as compared to controls, irradiated CFU-Fs significantly increased the proliferation rate of hematopoietic precursors and accelerated the differentiation toward the granulocytic lineage. Our data provide the first evidence of the key role exerted by the balance between osteoblasts and adipocytes in spontaneous bone marrow regeneration. First, (pre)osteoblast differentiation from MSC stimulated hematopoietic precursor's proliferation and granulopoietic regeneration. Then, in a second time (pre)osteoblasts progressively disappeared in favour of adipocytic cells which down regulated the proliferation and granulocytic differentiation and then contributed to a return to pre-irradiation conditions. [less ▲]

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See detailCancer du sein: importance de la nomenclature anatomo-pathologique
BLETARD, Noëlla ULg; DETREMBLEUR, Nancy ULg; SCAGNOL, Irène ULg et al

in Revue Médicale de Liège (2011), 66(5-6), 261-4

The breast pathology includes a large array of entities for which macroscopic and microscopic analysis remains fundamental. Tissue and cell morphology allows in most cases the distinction between benign ... [more ▼]

The breast pathology includes a large array of entities for which macroscopic and microscopic analysis remains fundamental. Tissue and cell morphology allows in most cases the distinction between benign or malignant tumours and therefore provides the clinicians with essential information for the therapeutic strategy. In the Pathology laboratory, immunohistochemistry and molecular biology have improved the specificity of the diagnosis and have introduced new prognostic and predictive markers for tumour management. The last edition of the WHO classification, released in 2003, distinguishes 21 varieties of invasive carcinoma and 2 categories of intraepithelial neoplasia based on the morphology and immunohistochemical profile. Other diseases can affect the breast, although much less frequently, such as Paget’s disease of the nipple, phyllode tumours, sarcomas, lymphomas... These diseases will not be reviewed here. [less ▲]

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See detailEtude génétique et anatomopathologique du syndrome de McCune-Albright chez l'adulte
Beckers, Albert ULg; Burlacu, Maria Cristina ULg; Thiry, Albert ULg et al

in Annales d'Endocrinologie (2010, September), 71(5), 345

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See detailAnatomo-pathological and genetic studies of adult McCune-Albright syndrome
Burlacu, Maria Cristina ULg; Thiry, Albert ULg; Livadariu, Elena ULg et al

in European Neuroendocrine Association - Liège, 22-25 septembre 2010 (2010, September)

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See detailEtude génétique et anatomopathologique du syndrome de McCune-Albright chez l'adulte
Beckers, Albert ULg; Burlacu, M.; Thiry, Albert ULg et al

in 27ème Congrès de la Société Française d'Endocrinologie - Deauville, 29 septembre - 2 octobre 2010 (2010, September)

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See detailTesticular effects of isolated luteinizing hormone deficiency and reversal by long-term human chorionic gonadotropin treatment.
Valdes Socin, Hernan Gonzalo ULg; Salvi, Roberto; Thiry, Albert ULg et al

in Journal of Clinical Endocrinology and Metabolism (2009), 94(1), 3-4

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See detailAngiosarcome sur lymphoedeme chronique: un cas de syndrome de Stewart-Treves.
Gonne, E.; Collignon, Joëlle ULg; Kurth, William ULg et al

in Revue Médicale de Liège (2009), 64(7-8), 409-13

The Stewart-Treves Syndrome is defined as an angiosarcoma (very aggressive malignant tumor originating from endothelial cells) appearing in a specific clinical setting. This tumor develops in patients ... [more ▼]

The Stewart-Treves Syndrome is defined as an angiosarcoma (very aggressive malignant tumor originating from endothelial cells) appearing in a specific clinical setting. This tumor develops in patients suffering from chronic lymphedema of the upper limb following mastectomy and axillary lymph node dissection for breast cancer. The diagnosis relies on medical history, clinical examination and a histological assesment (biopsy or resection). This syndrome represents a rare clinical entity. Unfortunately, the prognosis is poor. A large surgical resection is the treatment of choice if the patient is a candidate for a surgical resection with a curative intent Radiotherapy is sometimes used as a palliative local treatment. Chemotherapy is only used in more advanced cases, not curable by surgery alone. [less ▲]

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See detailAnatomo-pathological and genetic studies of adult McCune-Albright syndrome
Burlacu, M. C.; Thiry, Albert ULg; Livadariu, E. et al

in 18th Meeting of the Belgian Endocrine and Metabolic societies : Bruxelles, 25 octobre 2008 (2008, October)

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