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PubMed2005

Detection of pericardial inflammation with late-enhancement cardiac magnetic resonance imaging: initial results.

OBJECTIVE: To examine the value of late-enhancement cardiac magnetic resonance imaging (MRI) for detection of pericardial inflammation. MATERIALS AND METHODS: Late-enhancement cardiac MRI was performed in 16 patients with clinical suspicion of pericardial disease. Pericardial effusion, pericardial thickening and pericardial enhancement were assessed. MRI findings were compared with those of definitive pericardial histology (n=14) or microbiology (n=2). A control group of 12 patients with no clinical evidence of pericardial disease were also imaged with the same MRI protocol. RESULTS: Sensitivity and specificity for late-enhancement MRI detection of pericardial inflammation was of 100%. There was MRI late enhancement of the pericardial layers in all five patients with histological/microbiological evidence of inflammatory pericarditis. MRI demonstrated no pericardial thickening and no MRI late enhancement with or without a pericardial effusion in any of the five patients with histological evidence of a normal pericardium. MRI detected pericardial thickening in the absence of both pericardial effusion and late enhancement in all six patients with histological evidence of chronic fibrosing pericarditis. The 12 control subjects showed no evidence of pericardial MRI late enhancement. CONCLUSIONS: These findings demonstrate that MRI late enhancement can be used to visualize pericardial inflammation in patients with clinical suspicion of pericardial disease.

Adult↗

PubMed2005

Down's syndrome and esophageal achalasia: a rare but important clinical entity.

We report a case of esophageal achalasia (EA) in Downa9s syndrome. A six-year-old girl with DS was referred from another hospital for further management of dysphagia and growth retardation (weight 16.3 kg, height 105.4 cm: both below -1.5 SD). Dysphagia commenced when she was one year old and gastroesophageal reflux (GER) was diagnosed when she was four. Routine investigations suggested EA or esophageal stenosis secondary to GER. While dissecting the esophago-gastric junction in preparation for a Heller-Dor esophagocardiomyotomy (HD-ECM), the crus of the diaphragm was noted to be narrowed, severely fibrosed and attached to the lower esophagus which was covered by dense scar tissue suggestive of an old esophageal perforation secondary to GER esophagitis. A Nissen fundoplication was performed, but dysphagia persisted postoperatively, and a narrowing 2 cm above the fundoplication wrap with proximal dilatation was found on repeated barium studies. At re-laparotomy, the fundoplication was unwrapped and HD-ECM performed. Eight months postoperatively, she is well with no recurrence. EA must be considered in any DS patient presenting with dysphagia.

Cardia↗

PubMed2006

Free functional gracilis muscle transfer for reconstruction of massive ectopic calcification following a lower extremity fracture: a case report and overview of the literature.

We present a patient with massive posttraumatic ectopic calcification in the lower extremity. The patient complained of an increasing tender soft tissue mass with signs of acute inflammation and foot dorsiflexion weakness following a tibial fracture for 34 years. A large radiopaque mass was resected and reconstructed using a free functional gracilis muscle transfer, resulting in recovery of stable soft tissue and foot dorsiflexion. The resected material showed highly fibrosed soft tissue with extensive dystrophic calcifications. There were no sign of recurrence at 18-month follow-up.

Female↗

PubMed1997

Downregulation of decorin expression in dermal fibroblasts by interleukin-4.

Decorin, a chondroitin/dermatan sulphate proteoglycan, plays an important role in the assembly of extracellular matrix components. In this study, we investigated the influence of interleukin-4 (IL-4) on the expression of decorin and sulphated glycosamino-glycans (GAGs) in cultures of human skin fibroblasts. IL-4 inhibited the expression of decorin mRNA and its core protein synthesis. IL-4 stimulated dermatan sulphate but decreased chondroitin sulphate synthesis without altering the total amount of sulphated GAGs. IL-4 had no effect on the initiation activity of the dermatan sulphate and chondroitin sulphate chains, as evaluated by the effect of exogenous beta-xyloside that acts as an artificial chain initiator for dermatan sulphate and chondroitin sulphate biosynthesis. Type I collagen mRNA levels and protein synthesis were increased by this IL-4. Therefore, the effects of IL-4 on decorin and GAGs as well as type I collagen may have important implications for wound healing and for the treatment of several fibrosing diseases.

Adult↗

PubMed2002

Tumefactive fibroinflammatory lesion of the head and neck treated with steroids: a case report.

Tumefactive fibroinflammatory lesion is an idiopathic fibrosclerosing disorder of the head and neck region that clinically simulates a malignant process, but is histologically benign. This lesion is believed to be part of a broader fibrosclerotic syndrome that includes idiopathic mediastinal and retroperitoneal fibrosis, sclerosing cholangitis and Riedel's thyroiditis. The tumefactive fibroinflammatory lesion differs from other fibrosing conditions encountered in the head and neck region: fibromatoses, nodular fasciitis and fibrosarcomas. Although there is no optimum treatment, steroid therapy is suggested as the first line of management. Our patient was treated with corticosteroids and had a favourable response, supporting this approach as the initial treatment.

Aged↗

PubMedDate not supplied

Matrix metalloproteinase-1 activation via plasmin generated on alveolar epithelial cell surfaces.

Plasmin is a potent protease related to tissue repair/remodeling not by fibrinolysis alone but also by activation of cytokines such as transforming growth factor and hepatocyte growth factor and by activation of matrix metalloproteases. We examined whether matrix matalloproteinase-1 was activated via plasminogen activation on surfaces of cultured alveolar epithelial cells (A-549). Cells were cultured overnight with plasminogen, pro-matrix metalloproteinase-1, and type I collagen as a substrate. Sodium dodecil sulfate-polyacrylamide gel electrophoresis was used to detect type I collagen degradation in culture supernatant. Collagen degradation corresponded to cell surface plasmin generation. No such finding was seen in the absence of cells or plasminogen. Alveolar epithelial plasminogen activation is important in matrix metalloproteinase-1 activation and thus presumably in tissue remodeling in pulmonary fibrosing pulmonary diseases such as idiopathic pulmonary fibrosis.

Cells, Cultured↗

PubMed2002

Different expression of endothelin in the bronchoalveolar lavage in patients with pulmonary diseases.

Endothelin (ET) is a broncho- and vasoconstrictive cytokine, but it also possesses proinflammatory and mitogenic activity. It is suggested to be involved in the pathogenesis of fibrotic lung diseases. We analyzed the concentration of ET 1 in the bronchoalveolar lavage (BAL) fluid in 95 patients with different lung diseases, among them 41 patients with interstitial lung diseases (13 fibrosing alveolitis in systemic sclerosis (FASS), 9 idiopathic pulmonary fibrosis (IFP), 8 sarcoidosis (S), 6 occupational lung disease (OLD), 5 other alveolitidies A), 27 patients with pneumonia, and 8 patients with chronic obstructive pulmonary disease (COPD). A heterogeneous group of 19 patients served as controls. The median ET concentration was 3.3 pg/ml. Significantly higher concentration was found in patients with FASS (5.8 pg/ml), IPF (5.0 pg/ml), and S (5.1 pg/ml) compared with OLD (2.8 pg/ml), A (1.9 pg/ml), COPD (1.5 pg/ml), and the control group (2.5 pg/ml). In pneumonia, the elevated ET concentration (4.1 pg/ml) was accompanied by a high alveolocapillary leakage. When normalized to BAL albumin concentration, only FASS presented with significantly elevated ET/albumin in the BAL compared with the control group (134.5 vs. 56.l pg/mg, p < 0.05). There were no correlations between ET and BAL differential cell count or pulmonary function tests. In current smokers, ET in BALF was significantly higher compared with non- or ex-smokers (3.9 vs. 2.0 pg/ml, p < 0.01), but not so the ET/albumin ratio (65.0 vs. 62.5 pg/mg). In summary, ET in the BAL is differentially expressed in distinct inflammatory and interstitial lung disease. Consistently high concentrations are found in FASS and elevated ET concentration could be discussed in IPF, sarcoidosis, and pneumonia. ET concentration in BAL is influenced by current smoking habits.

Adult↗

PubMed2004

Erdheim-Chester disease: a case report.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare histiocytosis, histologically characterized by xanthogranulomatous inflammation. It may affect the bones, heart, lung, liver, kidneys, retroperitoneum, brain, and, rarely, the orbit. In the majority of patients, orbital infiltration is bilateral. CASE: A 61-year-old man presented with bilateral exophthalmos and progressive loss of visual function caused by chorioretinal folds and papillary swelling due to retrobulbar pseudotumor. The computed tomogram of the abdomen showed retroperitoneal thickening, leading to the interpretation of multifocal fibrosclerosis. This was further substantiated by an orbital biopsy revealing chronic fibrosing inflammation. Retrobulbar radiotherapy (20 Gy) and long-term systemic corticosteroid treatment followed. Although the retroperitoneal involvement decreased, no significant effect on orbital involvement was achieved. A second review of the orbital biopsy revealed foamy cell infiltration and the presence of a sclerotic process. Immunohistochemical examination demonstrated positive CD 68 stains, whereas S-100 and CD 1a were negative, thus confirming ECD. The histologic finding was comparable to a biopsy of the retroperitoneum. Endonasal decompression was performed but visual acuity (VA) decreased to 20/250 in the right eye and on finger counting in the left eye. The patient continues to be under therapy with prednisolone 20 mg/day and methotrexate 25 mg/week. CONCLUSIONS: The clinical orbital manifestation of ECD occurs in two different forms: one presenting as a mild impairment of visual function, while the second, clinical form, observed in our patient, is characterized by a progressive loss of VA despite therapeutic efforts such as immunosuppressive systemic therapy, radiation, and surgery. The described case illustrates that clinical findings in multifocal fibrosclerosis overlap with those observed in ECD.

Erdheim-Chester Disease↗

PubMed2001

Malignant mesothelioma--German mesothelioma register 1987-1999.

OBJECTIVES: The study group comprised a collective of 1,605 patients with malignant mesotheliomas and with lung tissue available for lung dust analyses. METHOD: Clinical features, occupational histories, expositions and individual data were evaluated, and the asbestos bodies concentrations (asbestos bodies/cm3 lung tissue or g wet tissue) were determined. RESULTS: Mesotheliomas developed mainly in men (94.5%). Of the cases, 96.4% were of pleural origin and only 3.3% were peritoneal mesotheliomas. The biphasic subtype predominated (61.3%), followed by the epithelioid type (29.3%). The sarcomatoid subtype was rarely developed (9.4%). Mean age at first diagnosis was 60.4 years. The mean survival time from time of symptom onset was 13.5 months. Patients with epithelioid subtypes had a longer survival time (16.9 months) than those with biphasic (13.1 months) and sarcomatoid subtypes (5.5 months). Of the patients, 73% presented pleural effusions as initial symptoms of the disease. An increased asbestos burden was identified by light microscopy in 84.8% of the patients. There was no association between histological subtypes and the asbestos burden of the lungs. Patients with peritoneal mesotheliomas had distinctly higher asbestos burdens in the lungs than patients with pleural mesotheliomas. There exists no association between lung asbestos burdens and survival times. The mean latency period was 37.8 years. A trend: higher asbestos burden of the lung/shorter latency periods was suggested. About 70% of the patients had a history of occupational exposure to asbestos dust. Most patients worked in the building trade, the locksmith and machine building industries and in the steel and blast-furnace industries. Of the patients, 25.6% had asbestos-associated lung fibroses, in 40.7% of the cases pleural plaques were identified. CONCLUSIONS: The most important causal factor for development of mesotheliomas is still asbestos, primarily amphibole asbestos. The recurring occurrence of mesotheliomas in younger people without known asbestos exposure needs the urgent investigation of other inducing factors for mesotheliomas.

Adult↗

PubMed2002

Diffuse mesenterial sclerosis: a characteristic feature of chronic small-bowel allograft rejection.

Chronic rejection is the major cause of late intestinal allograft dysfunction. The aim of this study was to analyze in detail the histopathological features of chronic rejection in the ACI-to-Lewis rat model of intestinal transplantation. Chronic rejection was achieved in orthotopic small-bowel allografts (ACI-Lewis) by limited immunosuppression with cyclosporin A (CyA). Isogeneic transplants (ACI-ACI) as well as native bowels (ACI) with and without immunosuppression served as controls. Bowels were removed together with the mesenteries 90 days postoperatively and analyzed using sections stained with hematoxylin and eosin as well as Masson's trichrome. The slides were coded, randomized and analyzed by grading of histological abnormalities. The most striking alterations of the allografts were noticed in the mesenteries exhibiting an extensive infiltration by mononuclear cells accompanied by a progressive diffuse fibrosis with shrinking of the mesenteries. These changes were most pronounced in the perivascular areas of the mesenteric arteriae and venae rectae. Three of five allografts showed vasculitis with myointimal proliferation of the arteriae rectae. Focally, there was spill-over of the inflammatory cells onto the intestinal muscularis propria. The mucosa of the allografts showed mild blunting, lymphocytic infiltration of the crypt epithelium and increased crypt cell apoptoses. The submucosa was unaffected, and there were no detectable abnormalities of the enteric ganglion cells. The present data support the view that chronic rejection of intestinal allografts is characterized by a diffuse sclerosing mesenteritis which may significantly contribute to late graft dysfunction. The present model may be useful to study the pathomechanisms of this inflammatory fibrosing process.

Animals↗

PubMed2006

Differing distributions of CXCR3- and CCR4-positive cells among types of interstitial pneumonia associated with collagen vascular diseases.

Interstitial pneumonia (IP) is an important complication in collagen vascular diseases (CVDs). We examined the distribution of helper T cell subsets in lung biopsies of cases of IP associated with CVD (CVD-IP). The tissues from 27 CVD-IP patients with rheumatoid arthritis (RA), 8 with polymyositis or dermatomyositis (PM/DM), and 8 with systemic sclerosis (SSc) were compared with those from 10 patients with idiopathic pulmonary fibrosis (IPF) in our previous study. The expressions of CXCR3 and CCR4 (chemokine receptors associated in vitro with Th1 and Th2 cells, respectively) in the mononuclear infiltrate were analyzed immunohistochemically. The positive cells were semiquantified in fibrosing areas of the CVD-IP and IPF cases. The number of CXCR3-positive cells was significantly greater in RA-IP than in PM/DM-IP, SSc-IP, or IPF, whereas there were fewer CCR4-positive cells in RA-IP, PM/DM-IP, and SSc-IP than in IPF. The CXCR3-/CCR4-positive cells ratio was significantly higher in RA-IP and PM/DM-IP (but not in SSc-IP) than in IPF. These results support previous reports of the dominance of Th2 cells in some SSc-IP and IPF cases. However, Th1-type immune responses may predominate in RA-IP and PM/DM-IP. Our findings suggest that the pathogenesis of CVD-IPs differs with the helper T cell subset.

Adult↗

PubMed2001

Constrictive bronchiolitis obliterans. Characterisation of fibrogenesis and lysyl oxidase expression patterns.

The process leading to irreversible fibrotic constriction of the bronchioles was studied in two cases of bronchiolitis obliterans (BO) after bone marrow transplantation. Because lysyl oxidase (LOX) is the main collagen cross-linking enzyme that might account for irreversible fibrosis, its expression was studied together with expression of extracellular matrix (ECM) proteins. Characteristic types of lesions could be distinguished on the basis of histological and immunohistological criteria. An inflammatory stage was characterised by infiltration restricted to the bronchioles by lymphocytes and dendritic cells. A fibro-inflammatory stage was characterised by the coexistence of a persistent immune cellular lesion pattern with further focal modelling of a sub-epithelial neo-synthesised connective matrix. LOX expression was observed at the tips of intra-luminal fibrotic protrusions, together with tenascin and cellular fibronectin. A fibrotic stage was characterised by dense ECM deposits spreading throughout the peri-bronchiolar connective tissue, resulting in bronchiole obliteration and final disappearance. In contrast to reversible cases of fibrosis, persistence of long-term LOX expression reflecting continuing fibrosing activity might account for the irreversible status of BO. Our two cases illustrated that, at inflammatory and fibro-inflammatory stages, BO may be stabilised by immunosuppressive treatment, while the persistence of LOX expression in the fibrotic stage might correspond to a disease that becomes irreversible and fatal.

Adult↗

PubMed2003

Transient exocrine pancreatic insufficiency as a possible complication of an enterovirus infection.

UNLABELLED: Exocrine pancreatic insufficiency is an exceptional problem in children, mostly associated with diseases like cystic fibrosis, Shwachman-Diamond syndrome or chronic pancreatitis, as is the case in idiopathic fibrosing pancreatitis. Many viral infections are known to cause acute pancreatitis. Most of them, however, are transient with no remaining damage. The differential diagnosis of persisting diarrhoea after gastrointestinal infection does not routinely include a search for exocrine pancreatic insufficiency. CONCLUSION: This is the first description of a child with a transient but severe exocrine pancreatic insufficiency probably induced by an ordinary enterovirus infection

Acute Disease↗

PubMed1997

Genotype-phenotype correlation in cystic fibrosis patients compound heterozygous for the A455E mutation.

Cystic fibrosis (CF) has a high incidence in the French-Canadian population of Saguenay Lac-Saint-Jean (Quebec). The A455E mutation accounts for 8.3% of the CF chromosomes. This mutation was shown to be associated with a milder lung disease in the Dutch population. Twenty two CF patients distributed in 17 families and compound heterozygotes for the A455E mutation have been followed at the Clinique de Fibrose Kystique de Chicoutimi. Fourteen patients also carried the delta F508 mutation while the remaining eight patients had the 621 + 1G-->T mutation. Each patient was matched by sex and age to a patient homozygous for the delta F508 mutation. The pairs were analyzed for several clinical and laboratory variables. The A455E compound heterozygotes were diagnosed at a later age (P = 0.003) and had chloride concentrations at the sweat test lower than those homozygous for the delta F508 mutation (P = 0.007). More patients were pancreatic sufficient (P = 0.004). They had a higher Shwachman score (P = 0.001) and better pulmonary function tests (P < 0.02). CF patients compound heterozygous for the A455E mutation have a milder pancreatic and lung disease than the delta F508 homozygotes. Therefore, the A455E should be associated with a better prognosis.

Adolescent↗

PubMed2006

Unexpected pathology during laparoscopic bariatric surgery.

BACKGROUND: The popularity of bariatric surgery has increased in recent years with the escalating incidence of morbid obesity in our society. The improvement in minimally invasive technology and the increased number of laparoscopic bariatric procedures being performed have resulted in the discovery of unexpected pathology not suspected preoperatively. The authors hypothesized that the occurrence of unexpected pathology is not associated with immediate adverse outcomes during laparoscopic bariatric procedures. METHODS: From December 2002 to June 2004, 398 patients underwent laparoscopic bariatric surgery for morbid obesity. A retrospective chart review was performed to determine the incidence of unexpected findings and their effect on patient results. RESULTS: Nine unexpected pathologic lesions were found in eight patients (2%). The findings included lesions on the small bowel (n = 3), stomach (n = 4), and liver (n = 2). In all cases except one (for which a biopsy was performed), the abnormalities were found and removed laparoscopically. The final pathology showed gastric leiomyomas (n = 2), gastric gastrointestinal stromal cell tumors (n = 2), ectopic pancreatic tissue (n = 2), arteriovenous malformation (n = 1), biliary adenoma (n = 1), and fibrosed hemangioma (n = 1). The planned bariatric procedures were completed for all the patients without incident. No complications occurred postoperatively, and all were discharged in 1 to 3 days (mean, 2 days). CONCLUSIONS: Unexpected findings occur with relative frequency during laparoscopic bariatric procedures. Biopsy or removal of these lesions usually does not increase complications nor preclude continuation of the planned bariatric procedure.

Adult↗

PubMed2001

Aortic and mitral valve replacement in a patient with acute febrile neutrophilic dermatosis (Sweet's syndrome): report of a case.

A 29-year-old woman was admitted to our hospital with severe orthopnea, fever, and acute dermatosis. She had a 5-year history of episodic acute neutrophilic dermatosis and peripheral leukocytosis following a high fever, which were symptoms consistent with a diagnosis of Sweet's syndrome. Echocardiography revealed remarkable dysfunction of the left ventricle due to severe aortic regurgitation, which had not been present at a previous admission when mild mitral regurgitation was detected. The aortic and mitral valves were replaced with prosthetic valves on an emergency basis. The leaflets of the aortic valve were very thin and appeared fragile. The anterior leaflet of the mitral valve showed severe prolapse due to the torn chordae and hypoplasia of the posterior strut chordae. Her postoperative course was uneventful. Microscopic examination revealed fibrosal degeneration and the infiltration of lymphocytes and macrophages into both heart valves. This may be the first case report of valvulitis and Sweet's syndrome occurring simultaneously.

Adult↗

PubMed1998

Prevention of local scar formation after operative discectomy for lumbar disc herniation.

This was a prospective study. The study evaluated the use of Preclude Spinal Membrane to inhibit peridural fibrosis and reduce fibroses-related problems after first-time lumbar discectomy. Peridural scarring causes tethering of dura and nerve roots. Following discectomy Preclude Spinal Membrane was applied to patients of first group (10 patients). The second group (10 patients) was operated on without Preclude Spinal Membrane. Outcome was evaluated with MRI, 3 and 6 month after operation for all patients. No peridural or epidural scar tissue could be found in patients with Preclude Spinal Membrane, in the control group who were operated on without the preclude Spinal Membrane scar tissue of varying degree with complete enclosure of the nerve roots and dura was found.

Adult↗

PubMed2004

P-ANCA cranial pachymeningitis: a case report.

Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment.

Antibodies, Antineutrophil Cytoplasmic↗