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Biomedical subjects

D Blockmans

Publications and source records attributed to D Blockmans.

At least 19 recordsLinked to original sources
PubMed2001

Clinical value of [(18)F]fluoro-deoxyglucose positron emission tomography for patients with fever of unknown origin.

We describe the diagnostic contribution of [(18)F]fluoro-deoxyglucose (FDG) positron emission tomography (PET) scan in 58 consecutive cases of fever of unknown origin (FUO) and compare this new approach with gallium scintigraphy. This investigation was performed from March 1996 through October 1998 at Gasthuisberg University Hospital in Leuven, Belgium. A final diagnosis was established for 38 patients (64%). Forty-six FDG-PET scans (79%) were abnormal; 24 of these abnormal scans (41% of the total number of scans) were considered helpful in diagnosis, and 22 (38% of the total number) were considered noncontributory to the diagnosis. In a subgroup of 40 patients (69%), both FDG-PET and gallium scintigraphy were performed. FDG-PET scan and gallium scintigraphy were normal in 23% and 33% of these cases, respectively; helpful in diagnosis in 35% and 25%, respectively; and noncontributory in 42% each. All foci of abnormal gallium accumulation were also detected by use of an FDG-PET scan. We conclude that FDG-PET is a valuable second-step technique in patients with FUO because it yielded diagnostic information in 41% of the patients in whom the probability of a definite diagnosis was only 64%. FDG-PET scan compares favorably with gallium scintigraphy for this indication. Because of the quick results (within hours instead of days), FDG-PET scan may replace gallium scintigraphy as a radiopharmaceutical for the evaluation of patients with FUO.

Citrates↗
PubMed2000

Periaortitis and aortic dissection due to Wegener's granulomatosis.

We describe here a patient with abdominal periaortitis and intramural dissection as early manifestations of Wegener's granulomatosis (WG). Surgical biopsies taken from the retroperitoneal inflammatory tissue surrounding the aorta showed granulomatous vasculitis. The patient had antiproteinase-3 antibodies and suffered from nasal, pulmonary, nervous and renal WG involvement. Although being a vasculitis of medium size and small vessels, WG should be included in the systemic vasculitides which can give rise to (peri)aortic inflammation.

Adult↗
PubMed2000

Positron emission tomography scan in the diagnosis and follow-up of aortitis of the thoracic aorta.

We report a case of a 62-year-old female patient with an inflammatory reaction of the thoracic aortic wall. The diagnosis was made by computed tomographic (CT) scan, magnetic resonance imaging (MRI) and positron emission tomographic scan with 18-fluorodeoxyglucose (FDG-PET). The patient was treated with corticosteroids. The inflammatory parameters as well as FDG-uptake on PET scan returned to normal. Due to its aspecific presentation, the diagnosis of aortitis is often hard to establish. With this case the possible role of FDG-PET scan as a valuable tool in the diagnosis and monitoring of this inflammatory aortic disorder was demonstrated.

Aorta, Thoracic↗
PubMed2000

Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement.

Here we present an unusual case of a 53-year old patient presenting AL-kappa amyloidosis with diffuse-type amyloidosis of lungs, lymph nodes and pleura. The underlying pathology was a B-cell immunoglobulin-secreting non-Hodgkin lymphoma, as proven by the presence of a monoclonal B-cell population in the bone marrow. Diffuse parenchymal infiltration of the lungs is extremely rare in non-systemic amyloidosis, with only 4 previous cases having been reported in the English literature.

Amyloidosis↗
PubMed1999

Giant cell arteritis can be associated with T4-lymphocytic alveolitis.

We describe three patients with histologically proven giant cell arteritis who presented with respiratory complaints. In one patient, dry cough and dyspnoea dominated the clinical picture. In the other two patients, a diagnosis of giant cell arteritis was readily suspected by the presence of typical complaints, although both patients spontaneously mentioned a persistent cough and dyspnoea, respectively. Radiographs of the chest were normal. Lung function tests, including a carbon monoxide (CO)-diffusion capacity measurement, were always normal. Broncho-alveolar lavage fluid examination showed a normal cell count but an increased number of lymphocytes (16-61%) with a predominance of T4-lymphocytes (65.5-84.5%). We conclude that respiratory complaints and T4-lymphocytic alveolitis can be associated with giant cell arteritis.

Aged↗
PubMed1999

New arguments for a vasculitic nature of polymyalgia rheumatica using positron emission tomography.

OBJECTIVE: To study the possible contribution of fluorodeoxyglucose (FDG)-positron emission tomography (PET) in the diagnosis of giant cell arteritis and polymyalgia rheumatica. METHODS: A consecutive case series consisting of five patients with polymyalgia rheumatica, six patients with temporal arteritis and 23 age-matched patients with other inflammatory conditions were evaluated with FDG-PET. Studies were performed before therapy with steroids was started. RESULTS: A total of 4/6 patients with giant cell arteritis and 4/5 patients with polymyalgia had increased FDG uptake in their thoracic vessels, compared to 1/23 controls (P < 0.001). Increased vascular FDG uptake in the upper legs was seen in 8/11 patients with giant cell arteritis or polymyalgia compared to 8/23 control patients (P < 0.05), and in the lower legs in 6/11 patients compared to 6/23 controls (P = not significant). CONCLUSIONS: FDG-PET scan is the first non-invasive technique which may indicate large-vessel vasculitis and which can show its extension throughout the body. It strongly suggests that polymyalgia rheumatica is a form of vasculitis.

Aged↗
PubMed1998

[Inflammation phenomena in vasculitis: from immune complexes to less immune forms].

Immune complexes are involved in the pathogenesis of Henoch-Schönlein purpura, essential mixed cryoglobulinemia, hepatitis B-associated periarteritis nodosa and hypersensitivity vasculitis (related to infection or medications). ANCA's probably play a pathogenic role in Wegener's Granulomatosis, microscopic polyangiitis and renal-limited vasculitis. Pathologic responses of T-lymfocytes and granuloma formation have been demonstrated in temporal arteritis, Takayasu arteritis, Wegener's Granulomatosis, Chürg-Strauss syndrome and Kawasaki syndrome. The eventual pathogenic role of AECA's is less clear, with the possible exception of rheumatoid arthritis and systemic lupus erythematosus. These four pathogenic factors are not mutually exclusive: several mechanisms may play in one disorder, as has been demonstrated for Wegener's Granulomatosis. From these new insights in the pathogenesis of vasculitis, new therapies with better efficacy and fewer side effects may arise.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1998

[Functional mechanisms of intravenous immunoglobulins].

Polyclonal immunoglobulins for intravenous use are increasingly used in clinical practice. The mechanisms involved in the treatment of patients with immunodeficiency diseases are not yet clear. In this article we review the most important of these mechanisms and we illustrate the use of immunoglobulins in different diseases. We discuss the interactions with the Fc-receptors, the interactions with the complement system, the influence on superantigens, idiotype-anti-idiotype interactions and the influence on B and T cell receptors. A better understanding of these mechanisms can lead to a better use of these expensive preparations.

Antibodies, Anti-Idiotypic↗
PubMed1998

Clinical spectrum associated with positive ANCA titres in 94 consecutive patients: is there a relation with PR-3 negative c-ANCA and hypergammaglobulinaemia?

OBJECTIVE: To calculate the positive predictive value (ppv) of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) and anti-proteinase 3 (PR 3) antibodies for Wegener's granulomatosis (WG) and to evaluate their association with other diseases. METHODS: The clinical files of all 94 patients who had a positive c- or perinuclear (p)-ANCA test, or both, in the laboratory of the University Hospital, Leuven between April 1995 and March 1996 and who attended the Internal Medicine Department of the hospital were retrospectively studied. RESULTS: Of the 94 patients with ANCAs (fluorescence titre > or = 1/40), 57 were c-ANCA positive and 45 p-ANCA positive (eight were simultaneously c- and p-ANCA positive). Of the 57 c-ANCA positive patients, 23 had WG. The ppv for WG thus was 40%. This value did not increase by defining a higher threshold for a positive ANCA. There was not a good relation between ANCA titres and disease activity in the WG patients, nor was there a relation between anti-PR 3 antibody levels and WG disease activity. The ppv of anti-PR 3 antibodies for WG however was very high (85%). There was a positive correlation between the level of (hyper) gammaglobulinaemia and c-ANCA titres in those patients with final diagnoses not known to be associated with c-ANCA. Forty five patients had positive p-ANCAs. The largest group were those with inflammatory bowel disease (n = 20, of whom the majority had colitis ulcerosa or primary sclerosing cholangitis, or both); the great majority of these patients had no anti-myeloperoxidase antibodies. Vasculitis was present in eight patients, of whom two had WG (both were also c-ANCA positive). CONCLUSION: There is a low ppv of c-ANCAs for WG, caused by a high percentage of PR 3 negative, positive c-ANCA determinations, possibly related to hypergammaglobulinaemia. Anti-PR 3 antibodies have a high ppv for WG. However, neither c-ANCA titre, nor the level of anti-PR 3 antibodies correlated with the activity of the disease.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1997

Skewed X-chromosome inactivation in female carriers of dyskeratosis congenita.

In this study, we report on a family with X-linked dyskeratosis congenita (DC). Linkage analysis with markers in the factor VIII gene at Xq28 yielded a LOD score of 2 at a recombination of 0. Clinical manifestations of DC, such as skin lesions following the Blaschko lines, were present in two obligate carrier females. Highly skewed X inactivation was observed in white blood cells, cultured skin fibroblasts, and buccal mucosa from female carriers of DC in this family. This suggests a critical role for the DC gene in bone marrow-cell and fibroblast-cell proliferation.

Adult↗
PubMed1996

Predictive value of nailfold capillaroscopy in the diagnosis of connective tissue diseases.

We revised the clinical files of 326 patients who underwent nailfold capillaroscopy. These patients could be subdivided into 4 groups: I: patients with clinical suspicion of connective tissue disease, II: patients with isolated Raynaud's phenomenon, III: patients with existing connective tissue disease, IV: patients with acrocyanosis, chronic pernio or related disorders. The presence of megacapillaries was noted. The sensitivity of their presence for the various categories of connective tissue disease was as follows: systemic sclerosis (n = 11): 100%, CREST (n = 15): 73%, MCTD (n = 9): 56%, dermatomyositis (n = 7): 86%. Nineteen patients with megacapillaries had no final diagnosis of connective tissue disease (specificity 93.3%). The positive predictive value of the presence of megacapillaries for a scleroderma spectrum disorder (SSD) was 63.5% and the negative predictive value of a normal capillaroscopy 96.7%. We conclude that nailfold capillaroscopy can be advised to rule out SSD's.

Angioscopy↗
PubMed1995

Platelet activation.

This review article describes the different receptors, second-messengers and mechanisms involved in platelet activation. Several platelet agonists have well-defined receptors at the platelet membrane of which a number are single polypeptides with 7 hydrophobic transmembrane domains. These receptors are connected, via GTP regulatory proteins, with cytoplasmic second-messenger-generating enzymes. Phospholipase C and adenylate cyclase are the two best-known enzymes, generating inositol triphosphate (IP3) and diacyl glycerol from phosphatidylinositol biphosphate and cyclic AMP from ATP respectively. The intraplatelet free calcium level, which is critical for the activation status of the platelet, is increased by IP3 and is lowered in the presence of rising cyclic AMP concentrations. Shape-change occurs with small increases in intraplatelet calcium, while aggregation and secretion of granules take place at higher calcium, levels. The role of myosin and actin filaments and of transmembrane glycoproteins is further discussed.

Animals↗
PubMed1995

Linear IgA dermatosis: a new cause of fever of unknown origin.

We describe a 60-year-old patient who presented with prolonged fever, weight loss, adenopathies and malaise. Two months later, an aphthous stomatitis and pharyngitis developed, together with ulcerations bilaterally in the groin. Two separate skin biopsies revealed the diagnosis of linear IgA dermatosis. This entity, which is well known to dermatologists but not to internists, should be added to the extensive list of disorders than can provoke the syndrome of fever of unknown origin.

Autoimmune Diseases↗
PubMed1995

Platelet ultrastructural and functional studies in myelodysplasia.

We studied the platelets of 8 patients with myelodysplasia aged 49-77 years, using both ultrastructural and functional techniques. Five of the 8 patients were classified as having refractory anaemia, and 3 as refractory anaemia with excess of blasts (RAEB). Electron microscopically, the myelodysplastic patients had in addition to normal ones, platelets containing significantly less alpha-granules. In part of these hypogranular platelets, the dense tubular system was abundant, but in contrast with normal platelets, it was not dispersed between the other organelles, nor did it form a membrane complex with the open cannalicular system. From a functional point of view, collagen-induced shape change was the most frequently disturbed parameter: there was a total loss of collagen-induced shape change in 5 patients. In 2 patients, there was a complete lack of response to collagen in platelet-rich plasma (both shape change and aggregation); in one of them, there was also a total loss of adenosine triphosphate secretion in response to all inducers tested. After 4 years of follow-up, 5 patients had died, of whom 3 were RAEB patients. An initial complete absence of collagen-induced shape change was found in these 5 patients, while in the 3 patients who were still alive at the end of the follow-up period, collagen-induced shape change was normal in 2 and slightly diminished in one.

Aged↗
PubMed1994

Kaposi's sarcoma presenting as generalized lymphedema.

We present a HIV-1 seropositive patient with generalized lymphedema, due to a rare lymphangiomatous variant of Kaposi's sarcoma, successfully treated with interferon-alpha. The clinical presentation and treatment possibilities of Kaposi's sarcoma are reviewed.

Blotting, Western↗
PubMed1993

Nailfold capillaroscopy in connective tissue disorders and in Raynaud's phenomenon.

This article focuses on the value and the limitations of nailfold capillaroscopy in the differential diagnosis of certain connective tissue diseases and in the early recognition of secondary Raynaud's phenomenon. The finding of a "scleroderma-pattern" (apical enlargement and drop-out of capillaries) points almost certainly to an underlying progressive systemic sclerosis, mixed connective tissue disease or dermatomyositis.

Arthritis, Rheumatoid↗