Biomedical subjects

C Godfraind

Publications and source records attributed to C Godfraind.

At least 19 recordsLinked to original sources

PubMed2006

Could the pale, soft, and exudative condition be explained by distinctive histological characteristics?

Pork quality depends on various genetic and environmental factors. Despite the improvement of slaughter conditions, the PSE type is still one of the main concerns in this field. This study was conducted on nonstressed animals to evaluate the tissue characteristics of some muscles usually involved during stress compared with a reference muscle, the M. triceps brachii, which is actually not subject to stress-caused damages. Samples of M. triceps brachii, M. longissimus dorsi, M. biceps femoris, and M. semimembranosus were taken from pigs exhibiting 1 of the 3 HAL genotypes (NN, Nn, or nn) and 2 of the 3 RN genotypes (rn+rn+ or rn+RN-). Histoenzymology and immunohistochemistry were used to compare the fiber typing and capillary network in these muscles within these different stress susceptibility genotypes. In comparison with the reference muscle, M. triceps brachii, the combination of a high value of the number of type IIb fibers and a low vascular network showed a primary effect on muscles usually involved during stress. This led to the definition of a PSE index. A dramatic increase (P < 0.001) in this PSE index was systematically found in muscles usually involved in the PSE-type condition. These results show that distinctive histological characteristics were associated with the vulnerability of some muscles independently of the genotypes. Moreover, this study highlights the distinctive histological features of each genotype and is likely to suggest some interactions between them.

Animals↗

PubMed2006

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium.

From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In 85 patients a 'definite' diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained 'probable'. We further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72 patients different final diagnoses were made, Alzheimer's disease being the most frequent (N = 20). The demographic parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly increased age-specific incidence (> 6/10(6)/year) of sCJD patients between 70 and 90 years old in the period 2002-2004 compared to 1998-2001 and retrospectively obtained data (1990-1997, p < 0.01). We undertook a detailed clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased vigilance for the diagnosis. In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although these patients are both clinically and neurobiochemically similar to the general sCJD phenotype.

Age Distribution↗

PubMed2006

Atopic myelitis: a clinical, biological, radiological and histopathological diagnosis.

We describe a young patient with an unusual intramedullary lesion filled with eosinophils. The 21-year-old man developed chronic myelitis without optic neuritis or signs of systemic or infectious disease. A spinal biopsy was conducted because of the progressive extension and pseudo-tumoural aspect of the lesion. Histopathological analysis of the biopsy specimen revealed a severe inflammatory process with macrophages and numerous eosinophils. The eosinophil count in the blood and cerebrospinal fluid (CSF) were normal. Clinical, laboratory and radiological data did not correspond with the usual causes of eosinophilic myelitis. Abnormal mite antigen-specific IgE levels and features similar to Japanese cases of atopic myelitis suggested an allergic origin. Despite normal total IgE levels, this case may be the second case of atopic myelitis reported in a Caucasian patient. Striking differences with the first reported case are the absence of a typical history of atopy and normal total IgE levels. This case highlights that atopic myelitis should be considered in myelopathy occurring in Caucasian patients even in the absence of hyperIgEaemia.

Adult↗

PubMed2005

Metastasis of a small cell carcinoma from undetected origin to the pituitary gland--the otolaryngologist's view.

Tumour metastasis to the anterior pituitary-hypothalamic area is rare. We present a patient who had severe headache, bitemporal quadrant hemianopsia and an expanding mass in the sella turcica as revealed by MRI. Partial resection via a transsphenoidal approach was performed and postoperative radiation therapy was initiated. Immunohistochemical investigation identified the tumour as a metastatic small cell carcinoma whose primary site remained undetected for more than 12 months despite repeated oncological evaluations. We reviewed the literature on metastatic disease of the pituitary gland.

Aged↗

PubMed2003

CDKN2A, CDKN2B and p14ARF are frequently and differentially methylated in ependymal tumours.

Ependymal tumours are histologically and clinically varied lesions. Numerical abnormalities of chromosome 9 are frequently associated with these tumours. Nevertheless, the three important tumour suppressor genes located in this chromosome, CDKN2A, CDKN2B and p14 ARF, have not been reported to be commonly altered in them. We studied promoter methylation of these genes, an important mechanism associated with gene silencing in a series of 152 ependymal tumours of WHO grades I to III. Methylation status of the CDKN2A, CDKN2B and p14 ARF promoters was assessed by methylation-specific polymerase chain reaction and the genetic results were correlated to clinicopathological features. We observed promoter methylation for CDKN2A in 21% (26/123) of tumours, for CDKN2B in 32% (23/71) and p14 ARF in 21% (23/108). For all three genes, posterior fossa ependymomas were less frequently methylated in paediatric patients than in adults. For CDKN2B, extracranial tumours were more frequently methylated than intracranial ones. For CDKN2B and p14 ARF, methylation was more frequent in low-grade tumours; the reverse was observed for CDKN2A. CDKN2A, CDKN2B and p14 ARF promoters were methylated in 21-32% of the tumours. Frequencies of methylation varied according to clinicopathological features. This suggests a role for these genes in ependymoma tumorigenesis.

Adolescent↗

PubMed2003

Tumour necrosis and microvascular proliferation are associated with 9p deletion and CDKN2A alterations in 1p/19q-deleted oligodendrogliomas.

A subset of oligodendrogliomas and oligoastrocytomas has been associated with 1p/19q deletion. Subsequently, this genetic alteration was linked to chemosensitivity and classic histology of oligodendrogliomas. Tumoural progression includes deletions of 9p, 10q and alterations of CDKN2A. However, these (epi)genetic changes have not been associated with specific histological features. In a series of 45 gliomas including oligodendrogliomas, oligoastrocytomas and astrocytomas, deletions of chromosomal regions implied in these tumours (1p, 9p, 10, 17p13, 19q and 22) were looked for by microsatellite analysis. Tumours that were deleted for 1p and 19q were selected. Subsequently, presence of deletions in the other studied regions, (epi)genetic changes in p14ARF, CDKN2A and CDKN2B, as well as histological features, were associated to these tumours. 1p/19q deletion was observed in 22 tumours. Twenty-one of them presented regions of classic histology of oligodendroglioma. A deletion of 9p was found in eight of them, always in association with tumour necrosis and/or microvascular proliferation. In addition, (epi)genetic alterations of CDKN2A were observed in 71% of these tumours. Presence of regions of classic histology of oligodendroglioma in a tumour sample is predictive of 1p/19q deletions. Necrosis and/or microvascular proliferation are signs of an additional 9p deletion. Finally, as CDKN2A (epi)genetic alterations were found in 71% of the 1p/19q/9p-deleted oligodendrogliomas, CDKN2A may have a role in oligodendroglioma-associated microvascular proliferation.

Central Nervous System Neoplasms↗

PubMed2001

Complex karyotype and absence of mutation in the c-kit receptor in aggressive mastocytosis presenting with pelvic osteolysis, eosinophilia and brain damage.

Aggressive mastocytosis is a form of systemic mast cell disease (SMCD) characterized by organ infiltration, bone lesions. eosinophilia and lymphadenopathies. Here we report a patient with unusual clinical features, namely osteolysis without other bone lesions commonly found in SMCD, major eosinophilia and cerebral infarction. The mast cells exhibited a classical immunophenotype (CD2+, CD9+, CD13+, CD25+, CD35+, CD45c+ and CD117+). Cytogenetic investigation showed novel complex aberrations, and clonal evolution was correlated with clinical progression. The screening for recurrent point mutations affecting the c-kit gene was negative. Mainly, the ASP816VAL substitution was not detected in our patient. Treatment with steroids and interferon was only temporarily effective.

Aged↗

PubMed2000

Spinal neurenteric cyst presenting in infancy with chronic fever and acute myelopathy.

The authors describe the clinical, radiologic, and pathologic features of a neonatal spinal neurenteric cyst (NC) presenting with long-lasting fever and acute myelopathy, and compare this observation with other infants reported in the literature. This observation shows that NC must be considered in the differential diagnosis of acute myelopathy with persistent fever in infancy. Fever is attributed to degenerative changes in the NC, triggering inflammatory cell infiltration and tumor necrosis factor alpha secretion.

Consanguinity↗

PubMed2000

Retrospective study of Creutzfeldt-Jakob disease in Belgium: neuropathological findings.

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that affects about 1 in 10(6) inhabitants in most countries. Recently, a new variant of CJD has been linked to the epidemic of bovine spongiform encephalopathy. Therefore, vigilance concerning the disease's incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Belgian autopsies, we found the characteristic triad of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while the cerebellum was mostly spared. Immunohistochemistry was performed using hydrated autoclave pretreatment and several monoclonal antibodies directed against the prion protein. We identified prion-immunoreactive patterns and locations reflecting the important heterogeneity, independently of the antibody that was used. Granular prion immunoreactivity was observed in astrocytes. We studied the regional intensity of the prion immunostaining and determined that the frontal cortex with 95% positive immunoreactivity was best suited for a biopsy. We studied the disease duration in sporadic CJD patients who showed neuropathological lesions of other neurodegenerative disorders (such as Alzheimer's disease). The study shapes the framework in which a prospective neuropathological registry will be able to function.

Amyloid beta-Peptides↗

PubMed2000

Orbital metastasis as primary manifestation of thyroid carcinoma.

A 59-year-old woman with unknown primary tumor developed progressive painless left upper eyelid swelling and exophthalmos. Computed tomography (CT) showed a well-circumscribed left orbital mass producing bone lysis. Immunohistologic staining of the incisional biopsy specimen was positive for thyroglobulin, suggesting an orbital metastasis from thyroid carcinoma. At this time, thyroglobulin was high (1400 ng/dL). Total thyroidectomy with lymph node dissection disclosed a follicular carcinoma with microscopic foci of papillary variant follicular carcinoma. Two months after radioiodine treatment, the CT showed a regression of the orbital tumor mass with concomitant decrease in thyroglobulin (428 ng/dL). Although orbital metastases of thyroid carcinoma are uncommon, thyroid carcinoma has to be considered as a potential primary tumor in a patient with an orbital metastasis.

Carcinoma, Papillary, Follicular↗

PubMed2000

Transcription factor hepatocyte nuclear factor 6 regulates pancreatic endocrine cell differentiation and controls expression of the proendocrine gene ngn3.

Hepatocyte nuclear factor 6 (HNF-6) is the prototype of a new class of cut homeodomain transcription factors. During mouse development, HNF-6 is expressed in the epithelial cells that are precursors of the exocrine and endocrine pancreatic cells. We have investigated the role of HNF-6 in pancreas differentiation by inactivating its gene in the mouse. In hnf6(-/-) embryos, the exocrine pancreas appeared to be normal but endocrine cell differentiation was impaired. The expression of neurogenin 3 (Ngn-3), a transcription factor that is essential for determination of endocrine cell precursors, was almost abolished. Consistent with this, we demonstrated that HNF-6 binds to and stimulates the ngn3 gene promoter. At birth, only a few endocrine cells were found and the islets of Langerhans were missing. Later, the number of endocrine cells increased and islets appeared. However, the architecture of the islets was perturbed, and their beta cells were deficient in glucose transporter 2 expression. Adult hnf6(-/-) mice were diabetic. Taken together, our data demonstrate that HNF-6 controls pancreatic endocrine differentiation at the precursor stage and identify HNF-6 as the first positive regulator of the proendocrine gene ngn3 in the pancreas. They also suggest that HNF-6 is a candidate gene for diabetes mellitus in humans.

Animals↗

PubMed2000

Cervical epidural pseudotumor and multifocal fibrosclerosis. Case report and review of the literature.

The authors present the case of a 45-year-old man suffering from progressive quadriplegia due to an expansive C3-T2 epidural mass. Neuropathological examination demonstrated pseudotumor tissue. The patient had had an orbital pseudotumor 5 years before admission, and other systemic manifestations of an idiopathic inflammatory disease were discovered. This case is extremely rare. Nine cases of multifocal fibrosclerosis with central nervous system involvement are described in the literature. To the authors' knowledge, this is the first description of a cervical epidural pseudotumor. Modern imaging has made the diagnosis of such an entity possible, and it is important for the neurosurgeon to consider this syndrome because the combination of surgery and systemic medical therapy can ensure a long-term survival with good quality of life.

Anti-Inflammatory Agents↗

PubMed1999

A retrospective study of Creutzfeldt-Jakob disease in Belgium.

Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob d1551isease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.

Adult↗

PubMed1999

Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome.

Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). The majority of the tumors occurred in infants and young children and involved the hypothalamic/chiasmatic region. The tumors were histologically similar to PA, but they were more monomorphous and more myxoid. Rosenthal fibers were not seen and only 1 of 18 tumors had eosinophilic granular bodies. At the end of the follow-up period, 6 patients were dead and 12 were alive with evidence of disease. Progression free survival (PFS) at 1 year was 38.7%. In comparison, we identified a control group of 13 classical PAs in the same age range and location as the study group. In this group, PFS at 1 year was 69.2%, which was significantly better than that for pilomyxoid tumors (p = 0.04). There was no CSF dissemination or death due to tumor progression among patients with classical PA. Eight of these patients are alive with recurrent disease, and 4 have no evidence of disease. While the monomorphous pilomyxoid tumors have some resemblance to classical PA, our results suggest that the former is a more aggressive variant or a separate entity that needs to be recognized for prognostic purposes.

Astrocytoma↗

PubMed1998

Intraepithelial infiltration by mast cells with both connective tissue-type and mucosal-type characteristics in gut, trachea, and kidneys of IL-9 transgenic mice.

IL-9 transgenic mice were analyzed for the presence of mast cells in different tissues. In these mice, increased mast cell infiltration was found in the gastric and intestinal epithelium as well as in the upper airways and kidney epithelium, but not in other organs, such as skin. IL-9 transgenic mast cells do not show signs of massive degranulation such as that found in IL-4 transgenic mice and are not involved in spontaneous pathologic changes. Gastric mast cells showed a phenotype related to connective-type mast cells, since they were stained by safranin, and strong expression of mouse mast cell protease-4 and -5 was found in this organ. However, they also expressed proteases related to the mucosal cell type, such as mouse mast cell protease-1 and -2. In vitro, although IL-9 by itself did not induce mast cell development from bone marrow progenitors, it strongly synergized with stem cell factor for the growth and differentiation of mast cells expressing the same protease pattern as that observed in IL-9 transgenic mice. Since constitutive stem cell factor expression was observed in vivo, and anti-c-Kit Abs inhibited IL-9 transgenic mastocytosis in the gut, this synergistic combination of factors is likely to be responsible for the mastocytosis observed in IL-9 transgenic mice. Taken together, these data demonstrate that IL-9 induces the in vivo amplification of a nonclassical mast cell subset with a mucosal localization but expressing proteases characteristic of both connective tissue-type and mucosal mast cells.

Animals↗

PubMed1998

Role of mouse hepatitis virus-A59 receptor Bgp1a expression in virus-induced pathogenesis.

Expression of Bgp1a, a glycoprotein that serves as receptor for mouse hepatitis virus-A59 has been analyzed in various mouse tissues and correlated with the pathogenicity that this virus induces in the corresponding organs. Expression of Bgp1a was observed in many cells of epithelial origin, including hepatocytes and endothelial cells. It was also shown on macrophages and B lymphocytes. Bgp1a localization may easily explain infection and lysis of some cell types like hepatocytes. In contrast, other cell types that express the viral receptor are not infected after in vivo inoculation with mouse hepatitis virus-A59, which may be due to inaccessibility of the receptor to the virus during mouse infection, or to resistance to this virus in some cell types. This may account for the ability of the blood-brain barrier to prevent mouse hepatitis virus-A59 spreading into the central nervous system. In other organs, the virus may induce pathogenesis indirectly, resulting in the destruction of cells that do not express Bgp1a, like thymic lymphocytes, or else impair cell functions such as cytokine and immunoglobulin production by macrophages and B lymphocytes, respectively.

Animals↗

PubMed1998

Morphological analysis of mouse hepatitis virus A59-induced pathology with regard to viral receptor expression.

Mouse hepatitis virus, strain A59 (MHV-A59), is a coronavirus that triggers in susceptible mice a wide variety of pathologies, including hepatitis, thymus involution, B lymphocyte polyclonal activation and, after intra-cerebral inoculation, transient demyelination. One receptor that mediates entry of the virus into target cells has been identified: it is a glycoprotein of the carcinoembryonic antigen family, called Bgp1a. The availability of antibodies recognizing this molecule permits the analysis of its cellular expression and of the relationship between receptor expression and pathology induced by the virus. Bgp1a is found on epithelial and endothelial cells as well as on B lymphocytes and macrophages. In the liver, Bgp1a expression correlates well with infection of hepatocytes and endothelial cells, leading to the development of hepatitis. However, other cells expressing this molecule, such as central nervous system endothelial cells, are not infected by the virus. This observation may explain how the blood-brain barrier prevents dissemination of MHV-A59 from the general circulation into the brain. Thymic atrophy results from apoptosis of immature double-positive T lymphocytes which might be caused indirectly by infection of a small proportion of thymus epithelial cells that express Bgp1a rather than by infection of T cells that do not express the receptor. Finally, polyclonal activation of B lymphocytes, leading to increased secretion of antibodies of the IgG2a isotype, involves a cascade of events, including cytokine secretion, that may result from the interaction of MHV-A59 with B cells and macrophages that express Bgp1a. Therefore, after viral infection, cellular expression of Bgp1a may have different results: cell lysis; alteration of cellular functions that may lead to indirect death of other cell types, or resistance to infection.

Animals↗

PubMed1998

Pre- and peroperative misdiagnosed sellar tumour.

We describe the case of a 76-year old woman complaining of headaches and progressive sight loss. Cerebral CT scan and MRI disclosed a large sellar tumour. Endocrine evaluation showed no evidence of pituitary hypersecretion, but a slight hyperprolactinemia related to stalk compression. A non-functioning pituitary adenoma was suspected. The pathological diagnosis was however a meningioma. In the literature, about 5% of sellar tumours are not pituitary adenomas and this is an alternate diagnosis to consider in the presence of non-secreting macroadenomas.

Adenoma↗