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At least 217 records · Page 12Linked to original sources
PubMed1995

Cystic fibrosis-associated colitis and fibrosing colonopathy.

OBJECTIVE: To describe our experience with cystic fibrosis (CF)-associated colitis and fibrosing colonopathy, and to assess treatment strategies. STUDY DESIGN: We reviewed hospital charts and autopsy reports of all University of Minnesota patients with CF between 1975 and August 1994. We identified six patients with colonopathy and compared them with a cohort of 79 patients with CF in the same age range and seen during the same period. RESULTS: All patients with colonopathy had bloody diarrhea; five of the six had abdominal pain. Stool frequency and related symptoms distinguished the patients with colonopathy from the cohort population. All took a higher median dose of pancreatic enzymes than the cohort population during the 3 months preceding the onset of symptoms (p < 0.002). For all six patients, barium studies revealed loss of haustration, and shortening and diffuse narrowing of the colonic lumen with relative rectal sparing. The distal ileal mucosa was irregular in four patients. A histopathologic study reveal fibrosis of the submucosa or lamina propria, and focal acute cryptitis in all six patients. Other features included ascites (2/6) and nodular regenerative hyperplasia of the liver (1/6). One patient continues to have symptoms, three had subtotal colectomy, and the condition of two improved after a regimen including a low-fat diet, withholding of pancreatic enzymes, and supplemental parenteral nutrition was initiated. CONCLUSIONS: Fibrosing colonopathy represents a newly recognized gastrointestinal complication of cystic fibrosis. Affected persons have taken larger doses of pancreatic enzymes than similar patients with cystic fibrosis, and have bloody diarrhea. We developed a medical protocol that may avoid surgical resection of the colon in some of these patients.

Biopsy↗
PubMed2000

Risk of cryptogenic fibrosing alveolitis in metal workers.

We report increased proportional mortality from cryptogenic fibrosing alveolitis in the workforce of a major UK engineering company. Measures of metal exposure from unbiased historical occupational records showed that among employees who have worked with metal, the risk of death from or with cryptogenic fibrosing alveolitis increased in relation to the duration of metal-working.

Aged↗
PubMed1986

Histological abnormalities in muscle from patients with certain types of fibrositis.

In muscle biopsy specimens from fibrositis patients and healthy subjects no differences in electrical charges on the contractile proteins were detected with a microelectrode technique. However, microscopical examination of fibrositis muscle showed muscle fibres connected by a network of reticular or elastic fibres which are absent in normal muscle and which may be the cause of the disorder.

Adult↗
PubMed1999

High-resolution computed tomography in the evaluation of fibrosing alveolitis.

High-resolution computed tomography (HRCT) is now widely used in the investigation of patients with suspected or known diffuse lung disease. This article reviews some of the technical aspects of HRCT and the pathologic considerations that should be appreciated in the context of diagnosing fibrosing alveolitis. The precise quantitation of disease extent and characterization of disease pattern on HRCT has been used to provide new insights about the prognosis and pathophysiology of fibrosing lung disease.

Diagnosis, Differential↗
PubMed1993

Pregnancy-associated ectopic decidua (deciduosis) of the greater omentum. An analysis of 60 biopsies with cases of fibrosing deciduosis and leiomyomatosis peritonealis disseminata.

Ectopic decidua (deciduosis) of the omentum was investigated in biopsies taken during cesarean sections and in tubal pregnancies in 60 cases. An ectopic decidua of various abdominal organs was present in 48 further cases. All omentum biopsies showed decidual reactions:focal deciduosis in 97% and diffuse deciduosis in 3%. With increasing duration of pregnancy, there was vacuolar degeneration and fragmentation of the decidua cells as a manifestation of regressive changes. Co-expression of antibodies to vimentin, desmin and actin can be detected immunohistochemically. Ectopic decidua is a physiological phenomenon of pregnancy and arises from a progesterone-induced metaplasia of the pluripotential cells of the "subcelomic mesenchyma". The involution of the decidua takes place in the fourth to sixth week post partum. A fibrosing deciduosis of the omentum with development of a collagenous connective tissue at its surface is described as metaplasia of the hormone-dependent "subcelomic" mesenchyma. Immunohistochemically, a second case with leiomyomatosis peritonealis disseminata from the same investigation material shows the transformation of desmin- and actin-positive decidual cells into myofibroblasts and leiomyocytes in the form of a direct metaplasia. As pregnancy-associated mesenchymal metaplasias, ectopic decidua, fibrosing deciduosis and leiomyomatosis peritonealis disseminata depend on hormone and may regress post partum.

Adolescent↗
PubMed1997

Late reopening of fibrosed capsular bags to reposition decentered intraocular lenses.

The infrequent postoperative complication of intraocular lens decentration in the presence of an intact fibrosed capsule has traditionally been treated with lens explantation and exchange for a sulcus-fixated lens. Many of these patients can be managed by reopening the fibrosed capsular bag and repositioning the lens within the bag. These cases can be performed quickly using topical anesthesia regardless of the time since the primary cataract procedure.

Anesthesia, Local↗
PubMed1990

Combined cryptogenic fibrosing alveolitis and emphysema: the value of high resolution computed tomography in assessment.

Simple tests of lung function may be misleading in the assessment of patients with interstitial lung disease. Eight patients are described with cryptogenic fibrosing alveolitis (histologically proven in four) with severe breathlessness and low gas transfer (median DLCO 32.4%, range 9.2 to 35.3%, % predicted) in whom lung volumes were preserved [median VC 98.7, range 67.5-131.1%; median TLC 92.5, range 88.1 to 121.2, (% predicted)], and in whom there was no evidence of airflow obstruction [median FEV1/FVC 84.6, range 68-116 (% predicted)]. All were current or ex-heavy smokers. Thoracic high resolution computed tomography revealed upper zone emphysema, the extent of which was not appreciated using conventional chest radiography. The atypical physiological and radiological features can be explained by coincidental cryptogenic fibrosing alveolitis and emphysema and high resolution computed tomography was valuable in the assessment of these patients.

Aged↗
PubMed2002

Juvenile idiopathic fibrosing pancreatitis.

Chronic pancreatitis is a rare differential diagnosis of obstructive jaundice and/or recurrent abdominal pain in childhood and adolescence. The hereditary calcifying and the noncalcifying obstructive form are the two major forms of juvenile chronic pancreatitis. Other causes include cystic fibrosis, hyperparathyroidism, hyperlipoproteinemia and ascariasis. Even less common is the so called idiopathic or fibrosing pancreatitis. Since the first description by Comfort in 1946 only 41 further cases of juvenile idiopathic fibrosing pancreatitis have been published. An association with gene mutations (PRSS1, SPINK1, CTFR-5T genotype) is suspected. We report the cases of a 17-year-old male patient who presented with painless obstructive jaundice and a 16-year-old female patient who presented with abdominal pain and obstructive jaundice. Both patients underwent surgical treatment with duodenum-preserving pancreatic head resection. The relevant literature with special regard to modern pancreatic surgery is reviewed to give an overview about this rare but surgically treatable pediatric condition, which merits the attention of pediatricians and gastroenterologists in cases of children and adolescents suffering from unexplained abdominal pain.

Abdominal Pain↗
PubMed1981

Different distributions of kinetically apparent molecular varieties of alpha 2-macroglobulin in health and in patients with cryptogenic fibrosing alveolitis.

1. Proteinase inhibitors have been studied in whole serum by using a kinetic method that avoids potentially damaging protein separation procedures. 2. The alpha 2-macroglobulin of an individual can be allocated unambiguously into one of seven categories according to the binding of trypsin to inhibitor in two kinetically apparent binding modes (beta- and alpha-modes). 3. The distribution of alpha 2-macroglobulin beta:alpha ratios in a healthy adult population is defined, and shown to be independent of sex and age. 4. The distribution of beta:alpha ratios in a group of patients with cryptogenic fibrosing alveolitis was found to be significantly different (P less than 0.005) from the normal distribution. 5. Changes in the beta:alpha ratio were noted in five of six patients with cryptogenic fibrosing alveolitis after treatment, but on no occasion when two healthy subjects were assessed a total of nine times. 6. The molecular interpretation and the possible importance of altered proteinase inhibition in inflammation and fibrosis are discussed.

Adult↗
PubMed2000

Recent advances in the aetiology of cryptogenic fibrosing alveolitis.

Crytogenic fibrosing alveolitis is the commonest intersititial lung disease but, until recently, very little has been known about its aetiology. The histopathologist usually sees this disease at transbronchial biopsy or at autopsy. This article reviews the current knowledge of the aetiology of cryptogenic fibrosing alveolitis looking at possible infective, occupational, drug-related, smoking-associated, genetic and dietary factors. Knowledge of the possible roles of these factors in the disease process informs histopathologists when they are reporting these biopsies and enables them to make a larger contribution to defining the pathogenetic mechanisms.

Feeding Behavior↗
PubMed2002

Frontal fibrosing alopecia associated with cutaneous lichen planus in a premenopausal woman.

A 45-year-old premenopausal woman presented with an 18-month history of a band-like area of fibrosing alopecia affecting the frontoparietal scalp. She also had marked thinning of the eyebrows. The histopathology was consistent with frontal fibrosing alopecia (FFA). Several months later she developed multiple pruritic papules on the wrists and feet. The clinical presentation and histopathology were consistent with cutaneous lichen planus. Although FFA has been reported to occur with mucosal lichen planus this is the first reported case of FFA associated with cutaneous lichen planus. This provides further evidence that FFA is a variant of lichen planopilaris.

Alopecia↗
PubMed2002

Increased circulating levels of soluble Fas ligand are correlated with disease activity in patients with fibrosing lung diseases.

OBJECTIVE: The Fas-Fas ligand (FasL) pathway is one of the important apoptosis-signalling molecule systems. We previously determined that this pathway may be involved in the pathogenesis of fibrosing lung diseases. In the present study, we evaluated the clinical significance of the levels of soluble forms of Fas (sFas) and FasL (sFasL) in serum from patients with fibrosing lung diseases. METHODOLOGY: We measured sFas, sFasL, KL-6 (a measure of alveolar type II cell damage), surfactant protein D (SP-D), and surfactant protein A (SP-A) levels in serum from 35 patients with idiopathic pulmonary fibrosis (IPF), 17 patients with interstitial pneumonia associated with collagen vascular diseases (CVD-IP), and 13 normal healthy controls using enzyme-linked immunosorbent assays (ELISA). RESULTS: The serum levels of sFasL were significantly increased in patients with active IPF and CVD-IP, compared with those with inactive disease and controls. There was no significant difference in sFasL levels between patients with inactive disease and controls. Serum sFasL levels were significantly correlated with lactate dehydrogenase and KL-6 levels in IPF. The decrease in sFasL levels following corticosteroid therapy was not correlated with the clinical course of IPF. There was no significant difference in serum sFas levels between IPF or CVD-IP patients and controls. CONCLUSIONS: Although further studies need to be performed on a large number of patients with histologically proven IPF or CVD-IP, it would seem that serum sFasL levels may reflect the activity of IPF and CVD-IP.

Antigens↗
PubMedDate not supplied

Nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease.

We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. The most common patient complaints are pruritus and dysesthesia. The extremities are predominantly involved with sparing of the torso and face. Dysfunction of internal organs has not been described, distinguishing it from other fibrosing conditions such as scleroderma. The skin biopsy is characterized by haphazardly arranged dermal collagen spindle cells in the reticular dermis. Extensive mucin deposits are interposed between collagen bundles and there are an increased number of fibroblast-type cells. In contrast to scleroderma, inflammatory cells are generally absent. Corticosteroid therapy can be tried, but in our patient was of no benefit.

Fibrosis↗
PubMed1996

Mediastinal compression syndromes due to idiopathic fibrosing mediastinitis--report of three cases and review of the literature.

Fibrosing mediastinitis is the most often observed benign cause of mediastinal compression syndromes, particularly the vena cava superior syndrome. We report 3 cases with such compression syndromes (2 x superior vena cava syndrome, 1 x symptomatic tracheal obstruction) due to fibrosing mediastinitis in which tumor resection led to a relief of symptoms. The operative procedures performed in our cases and in general as well as conservative treatment modalities reported for this rare disease are discussed with respect to our own experience and that of the recent publications in the European and American literature.

Adult↗
PubMed2003

Clinical and pathologic features of nephrogenic fibrosing dermopathy: a report of two cases.

Nephrogenic fibrosing dermopathy is a rare, recently described fibrotic skin condition that primarily affects patients with a history of renal disease. We describe 2 patients on hemodialysis with the characteristic clinical and pathologic features. Nephrogenic fibrosing dermopathy should be distinguished from other fibrotic disorders, such as scleromyxedema, systemic sclerosis, and eosinophilic fasciitis

Adult↗
PubMed1998

Simultaneous involvement of thyroid by Riedel's [correction of Reidel's] disease and fibrosing Hashimoto's thyroiditis: a case report.

We report an unusual thyroid lesion showing histologic features of both Riedel's [corrected] disease and fibrosing Hashimoto's thyroiditis in a 57-year-old white female. The clinical presentation was hypothyroidism associated with a solitary firm to hard cold nodule replacing the entire right lobe of thyroid gland. Pathological examination demonstrated extensive replacement of the thyroid parenchyma with dense keloidal fibrosis, intermixed well-developed lymphoid follicles, scattered lymphocytes, and plasma cells. The fibrotic process extended into the perithyroidal soft tissues and skeletal muscle with complete obliteration of the thyroid capsule. These findings were consistent with Riedel's [corrected] disease. However, the immunohistochemical stains for B and T markers and immunoglobulin light chains showed an immunoprofile consistent with Hashimoto's thyroiditis. This combination of Riedel's [corrected] disease and fibrosing Hashimoto's thyroiditis is rare and coincidental, as both represent two distinct clinicopathological entities.

B-Lymphocytes↗
PubMed1990

Fibrosing alveolitis due to sulphasalazine in a patient with rheumatoid arthritis.

Rheumatoid arthritis is being increasingly treated with sulphasalazine. We report the first case of a patient being treated for rheumatoid arthritis who developed severe dyspnoea with chest radiograph shadowing, reversible on discontinuation of sulphasalazine and subsequent steroid therapy. An histological diagnosis of fibrosing alveolitis was made. Thirteen cases of similar reactions to sulphasalazine, all in patients treated for inflammatory bowel disease, can be found in the literature. We identify two types of pulmonary reaction, an eosinophilic pneumonitis and a fibrosing alveolitis. Adequate histological investigation is needed to differentiate between the two and management may be different. The diagnosis of pulmonary reactions are important because they can be easily confused with complications of the underlying disease but the prognosis is much better.

Arthritis, Rheumatoid↗