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PubMed1998

Genetic determinants of Pseudomonas aeruginosa colonization in cystic fibrosis patients in Canada.

The present study was aimed at analyzing whether the rate of colonization and the age at colonization with Pseudomonas aeruginosa was genetically determined in cystic fibrosis (CF) patients. These two variables were calculated among 127 CF patients whose genotypes were known and who were monitored at the Clinique de Fibrose Kystique in Saguenay Lac-Saint-Jean. No statistically significant differences were found in the rate or the age at colonization when the patients were grouped by genotype; however, this result could be due to the small number of patients in each genotype group. The rate of colonization was significantly lower among CF patients carrying the A455E mutation, a "mild" allele with respect to exocrine pancreatic function, than among those carrying either the deltaF508 or the 621 + IG- > T mutation, both of which are "severe" alleles. The results confirm previous reports that the rate of colonization with Pseudomonas aeruginosa is, at least in part, genetically determined.

Adolescent↗
PubMed1988

Problems of fibrin adhesion of the nerves.

Although the fibrin adhesion enjoys increasing success in many areas of surgery, it has not, however, become fully established in nerve anastomosis. It was in this area particularly that significant advantages were expected. As the fibrin clot dissolved prematurely, however, and dehiscences ensued, antifibrinolytic substances had to be added to the adhesive. Fibroses occurred frequently as a result, which to date encumber nerve adhesive. We examined various factors of the adhesive system for their fibrosis-inducing effect. We were able to establish that no additional fibrosis-promoting effect emanated from aprotinin and the fibrin clot as a supply barrier, whereas thrombin, Factor XIII and fibronectin possess a fibrosis-promoting effect.

Animals↗
PubMed1984

Candidiasis-induced esophageal strictures.

Candidiasis of the esophagus progressing to hard fibrosed strictures of the esophagus in 2 patients is reported. Both patients had deficient immunologic systems and received extended courses of broad-spectrum antibiotic therapy for control of sepsis. The strictures were progressive despite adequate antifungal therapy and several attempts at dilatations and necessitated visceral esophageal substitution as definitive surgical therapy.

Adult↗
PubMed1984

Inflammatory pleuropulmonary fibrosis in essential fatty acid deficient rats and the lack of response to methysergide.

In view of the association between essential fatty acid (EFA) deficiency and human cystic fibrosis, we have investigated the possible occurrence of pulmonary disease in rats fed an EFA deficient (EFAD) diet for 40 weeks. In contrast to a few slight spontaneous lesions consisting of pleural membrane hyperplasia, which were found in the lungs of control rats, a much greater incidence of fibrotic lesions was observed in the lungs of EFAD rats. These pleuropulmonary fibroses extended from the hyperplastic pleural membrane into the alveoli and were characterized by collagen deposition and marked macrophage infiltration to the extent that, in some cases, the alveolar septa were completely obstructed by inflammatory exudate. These findings lend indirect support to the contention that EFA deficiency plays a role in the aetiology of cystic fibrosis, at least with regard to pulmonary lesions. Administration of methysergide (10 mg/kg/day, p.o.) for a total of 11 weeks, did not alter the incidence of fibrosis in the lungs of EFAD rats, despite the finding that a man who had developed pleuropulmonary fibrosis as a result of chronic methysergide treatment exhibited a relative serum EFA deficiency. While a relative EFA deficiency may be a predisposing factor for the induction of fibrosis by chronic methysergide treatment, our data are not sufficient to make a decision upon this hypothesis.

Adult↗
PubMed1994

Hepatic cysts and hyperechogenicities: perinatal assessment and unifying theory on their origin.

The authors report their experience with the perinatal diagnosis of six cases of hepatic cyst and six of liver hyperchogenicities. The in utero diagnosis of hepatic cysts was difficult when the cysts were large or subhepatic. A precise diagnosis was sometimes achieved only after surgery. Small intraparenchymal cysts tended to regress spontaneously. Liver hyperechogenicities were diffuse or localized. They were unrelated to liver infection, tumors, meconium peritonitis or biliary tract anomaly. Both entities could be related to vascular disruption phenomenon (VDP) in which structural anomalies result from damage to normal development of embryonic or fetal vessels with anomalies of perfusion to the developing organs. The consequences of VDP to the liver are variable depending on the time and duration of the phenomenon. Localized infarct or ischemia can fibrose or calcify leading to segmental hyperechogenicities. Necrosis with tissue reabsorption can be the origin of some (sub)hepatic cysts. When such anomalies are detected there should be a careful study of the placentation, and a search for potential associated anomalies should be undertaken.

Cysts↗
PubMed1996

Cholesterol crystal embolization to liver, gallbladder, and pancreas.

We retrospectively studied the clinical features of all 44 patients (35 men, 9 women, mean age 74.5 years) registered with a diagnosis of hepatic, biliary, and/or pancreatic cholesterol crystal embolization (CCE) in the Dutch National Pathology Information System (DNPIS) from 1973 through 1994. Liver CCE was found in 12 (11 autopsies and 1 biopsy), gallbladder CCE in 2 (resection specimens), pancreas CCE in 19 (18 autopsies and 1 biopsy), and both liver and pancreas CCE in 11 (all autopsies) patients. Five patients presented with focal liver cell necrosis, 1 with acalculous necrotizing cholecystitis, 1 with chronic cholecystitis, 10 with necrotizing pancreatitis, and 1 with chronic fibrosating pancreatitis. Four patients died of CCE-induced pancreatitis. Nineteen patients died as a consequence of other CCE sites. These were reported in 37 patients. All patients had a history of atherosclerotic vascular disease. In half the patients a possibly CCE provoking factor (vascular surgery and/or cannulation, anticoagulant treatment) was present. We conclude that liver cell necrosis, cholecystitis, and pancreatitis may be caused by CCE, particularly in elderly male patients with a history of atherosclerosis.

Aged↗
PubMed1981

Cerebral haemorrhage in Moyamoya disease at autopsy.

Nineteen fatal cases of occlusion of the circle of Willis, so-called Moyamoya disease, were examined clinicopathologically. Fresh and massive cerebral haemorrhage was confirmed in 14 and cerebral infarcts of 4 of 19 patients. Among these 14 patients, massive haemorrhage was found in the basal ganglia, thalamus and hypothalamus of 9, and in the thalamus, cerebral peduncle and midbrain of 5. Pathologically, fibrosing stenoses or occlusions involved the circle of Willis and its major branches in all cases. In 13 of 17 patients numerous collateral channels, muscular in type, paralleled the circle, bypassing the occluded natural passages, Rupture of dilated small muscular collateral arteries was demonstrated in fresh and old haemorrhagic lesions in 3 of the 14 patients. Saccular aneurysm of cerebral arteries in the subarachnoid spaces was present in two of the 19. No rupture involved the perforating arteries in the subarachnoid space. These findings strongly suggest that in patients with Moyamoya disease rupture of overgrown perforating arteries as collaterals in brain may be main cause of single or repeated cerebral haemorrhage. Stenoses or occlusions of these perforators are presumably an important factor in the occurrence of cerebral infarcts.

Adolescent↗
PubMed1993

Rheumatoid arthritis as a cause of cardiac compression. Favourable long-term outcome of pericardiectomy.

In order to clarify the significance of rheumatoid arthritis (RA) as a cause of cardiac compression, we scrutinized pericardiectomy files of 47 patients over a ten-year period at two university hospitals in Finland. Five patients with RA were found. All the patients with RA were men with seropositive disease and subcutaneous rheumatoid nodules. Two of the patients had pulmonary fibrosis, one had cutaneous vasculitis and three had had rheumatoid pleurisy. There was a mean delay of 10 months from the first cardiac symptom to the diagnosis of cardiac compression, the most common misdiagnosis being primarily a liver disease. On the basis of clinical and operative data, four out of the five patients had constrictive pericarditis and one had an effusive-constrictive form of the disease. The histopathological findings in all cases were consistent with chronic fibrosing pericarditis. A follow-up of seven to seventeen years of four patients has not revealed any signs of recurrent pericardial disease. Our results demonstrate that RA is an important aetiological factor for cardiac compression. The long-term outcome of this manifestation seems to be good after pericardiectomy.

Adult↗
PubMed1991

Validated method for automatic detection of lung sound crackles.

Crackling lung sounds are associated with many pulmonary diseases. Their occurrence reflects the quality and the severity of the disease. An automatic method for crackle detection is developed, based on analysing the spectral stationarity of the lung sound. The method is validated by studying the crackles of 20 adult patients; 10 with fibrosing alveolitis (FA) and 10 with bronchiectasis (BE). The number of crackles detected by the automatic method in inspiratory cycles is compared to the number of crackles counted from time-expanded waveforms by two expert observers. The total number of inspiratory cycles studied is 117 and that of crackles 1064. The method has a sensitivity of 89 per cent and a positive predictivity of 88 per cent for patients with FA, and 80 per cent and 83 per cent respectively, for patients with BE. The linear correlation coefficients between the numbers of crackles counted by the automatic method and by the observers is 0.86 (p less than 0.001) for the patients with FA and 0.93 (p less than 0.001) for the patients with BE. The values refer to whole inspiratory cycles. The new automatic method seems reliable enough for clinical and scientific purposes. It enables a rapid and objective analysis of large materials with crackling lung sounds.

Aged↗
PubMedDate not supplied

The spectrum of colonic involvement in pancreatitis.

Colonic involvement as a result of pancreatitis is an uncommon but interesting complication of the disease. Among the significant manifestations we have seen are: 1) adynamic ileus of the transverse colon, 2) fibrosing, stenosing pericolitis localized to the splenic flexure area, and 3) colonic hemorrhage secondary to necrosis and fistula formation. Ordinarily, conservative treatment will suffice, but in complicated cases, individualized surgical treatment becomes necessary.

Adult↗
PubMed1990

[Modifying regeneration success of glued nerve anastomosis by local corticoid administration].

Although the fibrin adhesion is being used in many areas of surgery with increasing success, it has, however, not become fully established in nerve anastomosis. At the beginning fibrin clots dissolved prematurely and dehiscences ensued. Antifibrinolytic substances had to be added to the adhesive to stabilize the clot. As a result fibroses occurred frequently, which up to now encumber nerve adhesive. The conventional suture technique is still not convincing also. Because reports showed a beneficial influence on conventional nerve sutures, the object of this study was a comparison between conventional suture technique and nerve anastomosis by fibrin adhesives with and without additive of corticosteroids. Electrophysiological methods were used, because regeneration is clinically determinating. The results do not recommend the use of local corticosteroids in nerve anastomoses fixed by fibrin adhesive.

Anastomosis, Surgical↗
PubMed1999

[CT densitometry for the grading of subcutaneous fibrosis after the photon-neutron therapy of malignant salivary gland tumors].

BACKGROUND: To evaluate alternative treatment regimen e.g. neutron therapy determination of treatment efficacy as well as side effects is important. Sensitivity of computed tomography (CT) in detecting changes of connective tissue after neutron therapy was examined. PATIENTS AND METHODS: In the course of their follow-up period (median 45 months) 12 patients with malignant salivary gland tumors who had postoperatively received neutron (1/12) or photon/neutron therapy (11/12) were examined by means of CT densitometry on 3 representative scans in the area of radiotherapy. In 3 ROI (regions of interest) in subcutaneous fatty tissue the density at the irradiated and the non-irradiated side was determined according to Hounsfield units (HU) and the average density was calculated. The average density of both sides was compared and correlated with the clinical grade of fibrosis according to LENT SOMA. RESULTS: All CT measurements (216 ROI, 18 ROI per patient) showed higher density levels on the irradiated side than on the non-irradiated side. The average density on the irradiated side was -57.7 +/- 4.7 HU and on the non-irradiated side -69.4 +/- 5.8 HU (p = 0.002). In 3/12 patients a clinical fibrosis was not seen; however, the relative density measured on the irradiated and non-irradiated side deviated by up to 8%. This could have been caused by minimal changes not being noticed by either patient and examiner. In patients with determined fibrosis Grade 1 (8/12) the relative density deviation was 4 to 39%. In 1/12 patients with determined fibrosis Grade 2 the relative density deviation was 50% (Figures 1a and 1b). Fibrosis Grade 3 and 4 did not occur (Table 1). CONCLUSIONS: Fibrosis is correlated with an increasing value of HU of the tissue density in CT. With the described method it is possible to graduate radiation induced subcutaneous fibrosis in correlation to the clinical fibrosis grade according to LENT SOMA. In the patients we examined subcutaneous fibroses after photon/neutron therapy were moderate. Especially in characterising subclinical or slight changes of connective tissue after radiotherapy computed tomography is of value.

Absorptiometry, Photon↗
PubMed2000

Cell-wall-deficient forms of Staphylococcus aureus as lung pathogens: an ultrastructural study.

The interaction between stable protoplast L forms of Staphylococcus aureus and the alveolar wall of infected rats was observed in the course of experimental pulmonary infection (days 3, 7, 14, and 30 p.i.). The L forms were successfully cultivated from bronchoalveolar lavage samples taken throughout the tested interval. The ultrastructural results demonstrated the ability of the L forms to invade the alveolar wall where they established, grew and reproduced mainly in the interstitium. The infection caused lung lesions: granulomas, focal fibroses and destruction of type I alveolar epithelial cells.

Animals↗
PubMed1994

Riedel's thyroiditis--case report and literature review.

A fifty two year old female underwent attempted thyroidectomy for a progressively enlarging cervical swelling. At operation the thyroid gland was extensively fibrosed and not resectable, frozen section showed fibrous replacement. The patient was given a short course of postoperative corticosteroid therapy and was maintained on thyroid replacement therapy. Thirty months later the patient remains asymptomatic.

Combined Modality Therapy↗
PubMed1994

Riedel's thyroiditis associated with high titers of antimicrosomal and antithyroglobulin antibodies and hypothyroidism.

Riedel's thyroiditis is a rare, chronic inflammatory disease of the thyroid gland. The aggressive fibrosis with extension beyond the thyroid into adjacent tissues contrasts with the diffuse, but intracapsular fibrosis of Hashimoto's thyroiditis. Most current studies refute the possibility of progression from a highly fibrosing form of Hashimoto's thyroiditis to a Riedel's thyroiditis based on the distinct clinical and laboratory data, although an unknown immunological basis is suggested for both diseases. The authors describe a patient with Riedel's thyroiditis, probably associated with Hashimoto's thyroiditis, sent to surgery because her cytological examination suggested thyroid malignancy. This patient had clinical and laboratory features of hypothyroidism and very high titers of antimicrosomal and antithyroglobulin antibodies, which decreased after surgery. Pathology studies disclosed Riedel's thyroiditis with intense lymphocytic infiltration suggestive of Hashimoto's thyroiditis. Quantitative immunohistochemical studies were not able to distinguish between both diseases.

Adult↗
PubMed1999

Expression of C-C chemokines in bronchoalveolar lavage cells from patients with granulomatous lung diseases.

To determine the role of C-C chemokines in the pathogenesis of granulomatous lung diseases, we studied the mRNA levels of C-C chemokines, regulated on activation normal T expressed and secreted (RANTES), macrophage inflammatory protein (MIP)-1alpha, MIP-1beta, and monocyte chemoattractant protein (MCP)-1 in bronchoalveolar lavage (BAL) cells obtained from patients with sarcoidosis (n = 17), hypersensitivity pneumonitis (HP) (n = 4), and cryptogenic fibrosing alveolitis (CFA) (n = 10) using the reverse transcription-polymerase chain reaction (RT-PCR) technique. The mRNA levels of RANTES, MIP-1alpha, and MIP-1beta in BAL cells were significantly correlated with the lavaged lymphocyte proportion, and a significant inverse correlation was observed between the mRNA level of MIP-1beta and the CD4/CD8 ratio of lavaged lymphocytes. Among the three diseases, the mRNA levels of RANTES and MIP-1alpha were significantly higher in the patients with sarcoidosis or HP compared with those in the patients with CFA. The level of MIP-1beta mRNA was significantly higher in the HP patients compared with that in the patients with sarcoidosis or CFA. No significant differences were observed in the level of MCP-1 mRNA among the three diseases. Thus, RANTES and MIP-1alpha were suggested to be important in the pathogenesis of granulomatous inflammation in sarcoidosis and HP. MIP-1beta might play an important role in the pathogenesis of HP, mediating the recruitment of lymphocytes specific to HP.

Adult↗
PubMed2005

[The Jo-1 Syndrome--immunological findings and clinical manifestations].

El The Jo-1 syndrome is an autoimmune disease which is characterized by the presence of autoantibodies against the Jo-1 antigen. The designation Jo-1 is derived from the name of the first patient (John P.) who was tested positive for this antibody. This patient suffered from polymyositis and fibrosing alveolitis. The Jo-1 antigen was identified as histidyl-transfer-RNA synthetase present in the cytosol. The Jo-1 syndrome is a member of a family of autoimmune diseases, called anti-synthetase syndromes. These syndromes are characterized by autoantibodies directed against aminoacyl-transfer-RNA synthetases. The etiology of the Jo-1 syndrome is unknown. The most frequent clinical manifestation is myositis, which may present as polymyositis or dermatomyositis. In addition to muscle involvement, interstitial lung disease is frequently found and critical for the prognosis. Furthermore, symptoms of other autoimmune disorders such as polyarthritis may occur. Similar to polymyositis and dermatomyositis, the Jo-1 syndrome may present as myositis overlap syndrome. In these cases, antibodies against U1-RNP are detected. The Jo-1 syndrome responds to treatment with corticosteroids and, if necessary, azathioprine, methotrexate or cyclophosphamide. The clinical manifestations of the Jo-1 syndrome are illustrated by two clinical cases.

Aged↗