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PubMed1979

Conduction system in two cases of sudden death two years after the Mustard procedure.

This report describes a serial section examination of the conduction system in two children who died suddenly 2 years following the Mustard procedure for complete transposition. The first child manifested sinus rhythm alternating with junctional rhythm in the last year of life. The second child, 2 months before death, had first-degree atrioventricular (AV) block which progressed to second-degree block with 2:1 conduction alternating with a junctional rhythm with AV dissociation. Examination of the conduction system in both cases revealed the approaches to the sinoatrial (SA) and the AV nodes to be markedly fibrosed. In addition, in Case 1 the SA node was interrupted by sutures and in Case 2 the SA node was considerably fibrosed. The arrhythmias produced and the probable cause of sudden death in both cases may be related to surgical injury to the approaches to the SA and AV nodes. This study and the review of the literature emphasize the necessity of maintaining the integrity of the SA node, the approaches to the SA and AV nodes, and the superior preferential pathway while performing the Mustard procedure for complete transposition.

Atrioventricular Node↗
PubMed1995

[Intercellular interactions in morphogenesis of initial lesions and sclerosis in interstitial lung diseases].

A total of 117 patients with pulmonary interstitial diseases (PID) were examined. The functional activity of alveolar macrophages was assayed in the lavage fluid and in lung tissue biopsy specimens from the generation of active oxygen forms, the secretion of tumor necrosis factor, fibronectin, expression of c-fos- and c-sis-oncoprotein. The stereotypic value for various PID was the development of alveolitis running in 2 stages: 1) early one, including exudative inflammation and 2) late one, involving sclerotic changes up to the formation of the honeycomb lung. This results in the block of the blood-air barrier and progression of respiratory failure and hypoxia in patients. The morphogenesis of fibrosing alveolitis is formed of alveolar septal damages caused by etiological agents of various nature, which is frequently unclear, by active forms of oxygen, lipid peroxidation products, proteases, tumor necrosis factor, which are produced by activated alveolar macrophages and polymorphonuclear leukocytes. The alveolar macrophage that secretes growth factors, c-fos- and c-sis-oncoproteins plays the key role in the progression of sclerotic changes. Lung cancer may develop at the end of fibrosing alveolitis at the stage of the honeycomb lung.

Adolescent↗
PubMed1995

[Lung transplantation].

Human lung transplantation was successfully performed in the early eighties and is now an option for patients with endstage lung disease, which is associated with poor survival. Most frequent indications for lung transplantation are emphysema, cystic fibrosis, fibrosing alveolitis, primary pulmonary hypertension and Eisenmenger's syndrome. Single lung transplantation (SLT) is most often performed in emphysema, fibrosing alveolitis and other diseases which are not associated with chronic infection of the lung. Double lung transplantation was recently replaced by the technique of sequential single lung or bilateral lung transplantation (BLT). Cardiopulmonary bypass can often be avoided and problems of the airway anastomosis are less frequent using BLT. Main indications for this procedure are cystic fibrosis, bronchiectasis and primary pulmonary hypertension (PPH). In PPH often only SLT is performed. Cor pulmonale is reversible following SLT or BLT even if the heart is not replaced. Combined heart-lung transplantation (HLT) is reserved for some cases of Eisenmenger's syndrome and few centers still prefer HLT in patients with cystic fibrosis. Patients are usually accepted for transplantation when they are considered to have life expectancy of 12 to 24 months. Quality of life and physical working capacity are severely decreased and patients suffer dyspnea NYHA grade III or IV. Most of the patients are hypoxic and need continuous oxygen therapy. Hypercapnia is also a negative predictive factor for survival without transplantation. In PPH cardiac index of less than 2 litres/m2 is associated with poor outcome. Not only absolute values for FEV1 and pO2 have to be considered in finding the best moment for assessment for transplantation but the clinical course of the disease during previous months and years also has to be taken into account. Contraindications to transplantation include acute infection, concomitant diseases of other organs, bronchial carcinoma and psychiatric disorders if noncompliance is likely. To achieve good results after lung transplantation, proper donor and recipient selection, experienced surgery and careful postoperative management are essential. Complications must be diagnosed early to provide effective treatment. Most complications occur within the first months after surgery. Early complications include primary organ failure, pleural bleeding, problems at the site of the airway anastomosis, infection and acute rejection. Acute rejection is common but can be treated successfully if diagnosed early.(ABSTRACT TRUNCATED AT 400 WORDS)

Comorbidity↗
PubMed1994

Crotalaria juncea intoxication in horses.

Twenty horses died 30 d after being fed a diet containing 40% of tritured Crotalaria juncea seeds. Before death, they had staggering, dyspnea and fever. At necropsy the most evident lesions were areas of lung parenchyma consolidation and enlarged and congested livers. Histopathological examination revealed diffuse fibrosing alveolitis with hyaline membranes, suggesting a blood-borne insult, and passive congestion in the liver with compression of the hepatocyte trabecules. To confirm the diagnosis, guinea pigs were given 60% of a commercial diet + 40% tritured C juncea seeds. After 4 mo of feeding the animals died with dyspnea. Their lungs had diffuse fibrosing alveolitis with discrete formation of hyaline membranes and the livers were congested. Reproduction of the lesions implicated the plant and supported the diagnosis of C juncea intoxication in the horses.

Animals↗
PubMed1994

Development of fibrosis after lung irradiation in relation to inflammation and lung function in a mouse strain prone to fibrosis.

The development of lung fibrosis after single-dose thoracic irradiation was studied histologically in C57L/J male mice. Lung function was monitored using uptake of carbon monoxide. During the latent period (prior to 15 weeks postirradiation) mice were chosen at random, while during the early phase (15-22 weeks) mice were sacrificed when they developed a functional deficit of at least 50%. Excess mice with a deficit of 50% in the early phase were followed into the late phase and sacrificed at 31 or 40 weeks. Two scoring methods were used to quantify lung damage. Fibrotic lesions and foci of inflammation were counted for the latent period, and the proportion of nonfunctional acini was determined for the early and late phases. After a dose 1 Gy less than the LD50/180, small regions of mild inflammatory infiltration appeared at 6 weeks, and small, focal fibrotic lesions containing numerous macrophages appeared at 8 weeks postirradiation. The number of fibrotic lesions increased steadily during the latent period in a manner that is consistent with conversion of inflammatory lesions to foci of fibrosis. Mice sacrificed upon developing a 50% functional deficit during the early phase had approximately equal proportions of lung affected by fibrosis and inflammation. Those mice which developed a similar respiratory deficit in the early phase and were followed into the late phase usually showed little change in lung function. However, when sacrificed at 31 weeks they had twice as much fibrosis and very little inflammation, suggesting that the inflammatory lesions had become fibrosed. The average number of macrophages per unit area of fibrosis declined during the latent period and changed little during the early and late phases. Lymphocytes and mast cells were also quantified in fibrosed regions.

Animals↗
PubMed1993

Pathogenesis of lipodermatosclerosis of venous disease: the lesson learned from eosinophilic fasciitis.

The histological features of lipodermatosclerosis and eosinophilic fasciitis and its variants were compared in a prospective study of outpatients attending the vascular clinic and inpatients in the Department of Medicine of a regional university hospital. Main outcome measures examined were swelling and induration of the subcutaneous layers with a stocking distribution in the calves. The inflammatory and fibrosing alterations involving the panniculus adiposus, superficial fascia and perimysium were essentially indistinguishable histologically in patients with lipodermatosclerosis or eosinophilic fasciitis and its variants. The intensity of the subcutaneous induration was related to the underlying nosological entity and the duration of the process. Infectious cellulitis was found to aggravate the clinical symptoms of lipodermatosclerosis. Since the stereotypical inflammatory and fibrosing processes in lipodermatosclerosis and eosinophilic fasciitis and its variants are similar irrespective of the initiating factors, it is suggested that there is a common final pathway in the pathogenesis of both disorders.

Adipose Tissue↗
PubMed1993

[Pathogenesis of development of lung cancer in idiopathic interstitial pneumonia--growth factors in bronchoalveolar lavage fluid].

It has been generally accepted that lung fibrosis as in idiopathic interstitial pneumonia (IIP) is frequently associated with the development of lung cancer. This observation implies that the mechanism involved in carcinogenesis and/or enhanced proliferation of cancer cells is common to the fibrosing process. However, there are few studies reported on the pathogenesis of associated lung cancer except for several studies assessed from the point of view of surgical pathology. This study was undertaken to learn whether BAL fluid, which reflects the local milieu of the fibrosing process, enhances the proliferation of human lung cancer cell line Lu-99, Lu-65 and rat lung fibroblasts as assessed by 3H-thymidine incorporation. BAL fluid was obtained from patients with IIP (n = 8) and normal volunteers (n = 8). BAL fluids from patients with IIP enhanced the mean incorporation of 3H-thymidine of Lu-99 up to 3.6 times (p < 0.01) compared to that of normal volunteers. Furthermore, the mean incorporation of Lu-65 was increased up to 1.8 times (p < 0.05) by BAL fluids from patients with IIP. In contrast, BAL fluids from patients caused no significant increase of the mean incorporation of rat lung fibroblasts as compared to normal BAL fluids. The enhancing activities on the growth of cancer cell line Lu-99, Lu-65 were eluted in several fractions by high performance liquid chromatography using Superose 12. These observations indicate that the BAL fluid of IIP patients contains factors enhancing the growth of cancer cells.

Aged↗
PubMed1993

Adenocarcinoma of the lung in association with chronic sarcoidosis.

Adenocarcinoma of the lung occurred three years after the diagnosis of pulmonary and cutaneous sarcoidosis. The combination of pulmonary sarcoidosis and carcinoma, which may present diagnostic difficulties is rarely reported. As with other fibrosing lung diseases, however, chronic fibrosing sarcoidosis may predispose to adenocarcinoma.

Adenocarcinoma↗
PubMed1995

Bronchoalveolar lavage in radiologically detected diffuse lung disease. Diagnostic value of total and differential cell count in a series of 130 patients.

During a 7-year period bronchoalveolar lavage (BAL) was performed as a routine diagnostic procedure at fiberoptic bronchoscopy in 172 consecutive patients with diffuse pulmonary lesions. In 42 patients, BAL was technically insufficient or data were incomplete. These patients were excluded. The remaining 130 patients consisted of 78 men and 52 women with a median age of 43 years (range 19-79); 59 were smokers. They were divided into 6 groups: I. sarcoidosis (n = 77); II. cryptogenic fibrosing alveolitis (n = 16); III. secondary fibrosing alveolitis (n = 7); IV. malignancy (n = 7); V. allergic alveolitis (n = 6); VI. miscellaneous (n = 17). Group VI was not included in the statistical evaluation, which involved only the 113 patients in groups I-V. BAL was performed in a segment of the right middle lobe with 150-200 ml isotonic saline. The return fluid (BALF) was filtered through two layers of cotton gauze, and total and differential cell counts were assessed. Median BALF return volume was 67% (range 35-90). Eighty percent of the procedures were performed by the same operator. Total cell count displayed no significant difference amongst the five diagnostic groups (p = 0.06). Differential cell count displayed differences amongst the groups respecting macrophages (p = 0.002), lymphocytes (p = 0.0004), neutrophils (p = 0.0001) and eosinophils (p = 0.04). Patients with sarcoidosis had a higher percentage of lymphocytes, patients with secondary fibrosis a higher percentage of neutrophils, and patients with cryptogenic fibrosis a higher percentage of eosinophils than the other groups. Malignant cells were observed in BALF in 14.3% of patients with malignant lesions. Among the patients with sarcoidosis, 75% had a lymphocyte-dominated BALF (> 10%) compared with 31% of the patients with cryptogenic fibrosis, 14% of the patients with secondary fibrosis, and 43% of the patients with malignancy. Dominance of neutrophils (> 10%) and/or eosinophils (> 5%) in BALF was observed in cryptogenic and secondary fibrosis. In most patients, BAL cannot provide a definite diagnosis, but may support the clinical suspicion of a specific diagnosis. In clinical practice, BAL seems to be of limited value in the diagnostic evaluation of radiologically detected diffuse, non-infectious pulmonary lesions.

Adult↗
PubMed1996

The lungs in rheumatoid arthritis--a clinical, radiographic and pulmonary function study.

OBJECTIVE: To determine the prevalence and spectrum of pulmonary abnormalities in patients with rheumatoid arthritis (RA) in a developing country. DESIGN: This was a prospective hospital-based survey of a randomly selected group of patients with RA who were seen in a rheumatic diseases unit. SETTING: Groote Schuur Hospital and Princess Alice Orthopaedic Hospital, Cape Town. PATIENTS: A group of 104 patients with RA were randomly selected from a total of 330 patients with RA who were seen in the rheumatic diseases unit. All the patients were interviewed and a clinical assessment, chest radiographs and pulmonary function tests were performed. RESULTS: Fifty-six patients (53.8%) had evidence of one or more current or previous pulmonary diseases: rheumatoid nodule in 1 (1%), bronchiectasis in 2 (1.9%), fibrosing alveolitis in 5 (4.8%), pneumonia in 5 (4.8%), asthma in 9 (8.7%), pleural disease in 17 (16.3%) and tuberculosis in 25 (24%). Excluding patients who were smokers or ex-smokers or who had coexistent pulmonary disease, there were 20 patients (19.2%) who had pulmonary abnormalities that could be attributed to RA: rheumatoid nodule in 1 (1%), fibrosing alveolitis in 5 (4.8%) (1 of whom also had pleural disease), pleural disease alone in 8 (7.7%), diffusion defect in 5 (4.8%) and airways obstruction in 1 (1%). CONCLUSION: This study provides clinical and lung function criteria that allow a clinically useful stratification of abnormalities in relation to a spectrum of common causes of pulmonary dysfunction that need to be distinguished from pulmonary abnormalities caused by RA. Pulmonary abnormalities are common and about 20% of RA patients may have an abnormality related to RA.

Aged↗
PubMed1996

Direct assessment of gastrointestinal inflammation and mucosal immunity in children with cystic fibrosis.

Fibrosing colonopathy is a recently described complication of cystic fibrosis, of unknown aetiology but possibly related to treatment with high-dose pancreatic enzyme supplements. We have used a whole gut perfusion technique to study subclinical gut inflammation in cystic fibrosis patients; concentrations of haemoglobin, IgG, albumin, alpha-1-antitrypsin, granulocyte elastase, IL1 beta, and IL8 were measured in whole gut lavage fluid: 23 tests were performed in 17 children with cystic fibrosis (20 elective tests, three lavages to treat distal intestinal obstruction syndrome (DIOS)). None has had fibrosing or haemorrhagic colitis. There were 12 tests in control children with constipation or precolonoscopy. Moderately abnormal results were obtained for many of the parameters studied, in specimens from all the cystic fibrosis children; however there were no significant differences between tests on high-dose and low-dose enzyme supplements of the same brand in the five children who had duplicate tests performed electively. The lavage fluid specimens from two cystic fibrosis children were strikingly abnormal in all tests apart from haemoglobin and alpha-1-antitrypsin. These were two of the three children with DIOS, and were also the only cases in the series taking Nutrizym 22. These data suggest that the majority of cystic fibrosis children, including those on high-dose enzyme supplements, do not have clinically significant colitis, but that there is subclinical mucosal inflammation in a minority (two of 17 in this series), for which DIOS and/or Nutrizym 22 treatment may be risk factors. Alternatively, inflammation and dysmotility in the proximal colon may be directly produced by a drug or other agent, producing a clinical syndrome indistinguishable from DIOS. Tests for indices of inflammation in gut lavage fluid offer a new approach to the detection and measurement of iatrogenic intestinal and colonic injury.

Adolescent↗
PubMed1977

[Coronary atherosclerosis, coronary thrombosis and myocardial infarction in autopsy cases. VII. Communication: Complications in myocardial infarctions (author's transl)].

2393 cases of myocardial infarctions autopsied during the period from 1.1. 1951 until 31. 12. 1969 in our Institute were scored for aneurysm and rupture of the cardiac wall as well as for partietal thrombosis and endocardial fibrosis, and analysed concerning frequency and distribution of sex. Aneurysm of the ventricle walls was present in 23.94 per cent of myocardial infarctions. Most of the aneurysms were found in the posterior wall of the left ventricle. Callous infarctions showed more aneurysms than fresh myocardial lesions. A rupture was present in 31.82 per cent of acute aneurysms and in 4.53 per cent of old aneurysms. Parietal thromboses we found in 13.92 per cent of all infarctions. In 14.21 per cent of all myocardial infarctions endocardial fibroses were present. Parietal thromboses and endocardial fibroses were significantly most frequent in infarctions of the right ventricle.

Acute Disease↗
PubMed1996

[Studies of serum markers in patients with interstitial pneumonia/pulmonary fibrosis complicated with collagen diseases: clinical evaluation of CYFRA21-1].

The serum concentration of several markers in patients with collagen disease was studied to evaluate the useful indices for the diagnosis of interstitial pneumonia/pulmonary fibroses (pneumonitis). Procollagen N-terminal type III peptides, type IV collagen and monoamine oxidase were measured as the fibrosing markers. Squamous cell related antigen (SCC) and soluble cytokeratin 19 fragments (CYFRA21-1) were measured as the tumor markers. Hyaluronic acid, ESR and CRP were measured as the inflammation markers. The 119 patients with collagen disease (71 patients with RA, 16 with SSc, 9 with SLE, 8 with PM/DM, 6 with MCTI) and 9 with other collagen diseases) who have been followed in our hospital were studied. Of 119 patients, 23 patients (RA14, SSc7, PM/DM2) were complicated with pneumonitis. The results were as follows. 1. All the serum markers except CYFRA21-1 had no significantly difference between with and without pneumonitis. The serum CYFRA21-1 level in the pneumonitis group was higher than that of the non pneumonitis group (1.38 vs 0.66 ng/ml, P < 0.001). 2. The cut-off value was set at 2.0 ng/ml, corresponding to 26.1% sensitivity and 97.9% specificity for pneumonitis complicated with collagen disease. 3. The CYFRA21-1 level in early stage of pneumonitis group (from onset to measurement < 1 year) was significantly higher than that of late stage group (from onset to measurement > 1 year). And there are 75.0% sensitivity in early stage of pneumonitis group. 4. Case study was suggested that CYFRA21-1 had a potential as a diagnostic and monitoring marker for pneumonitis.

Adult↗
PubMed1998

Cutaneous graft-versus-host disease.

Graft-versus-host disease (GvHD) includes inflammatory and/or fibrosing manifestations that may arise at various times after transplantation of any organ containing lymphoid cells. For the dermatologist, the importance of GvHD is real and current. Indeed, because it has become easier to perform bone marrow transplantation (EMT), the indications of BMT have become broader, making follow-up of patients receiving grafts a widespread practice. Nonetheless, GvHD remains a frequent complication of BMT and its principal target organ is the skin. Furthermore, recent innovations, such as grafting of umbilical cord blood and the mobilization of peripheral blood progenitor cells, will be the source of new questions concerning the development of GvHD under these conditions. Finally, because of its analogies with other spontaneous idiopathic skin diseases, GvHD constitutes a model that may lead to a better understanding of the pathophysiological features of these diseases. In this review, the cutaneous aspects of GvHD are emphasized.

Acute Disease↗
PubMed2000

Recurrent basal cell carcinoma after incomplete resection.

BACKGROUND: Because the probability of basal cell carcinoma (BCC) recurrence was thought to be 30% to 50%, surgical tradition became not to perform additional resection when the margin was positive. OBJECTIVE: To determine whether there is an association among age or sex of the patient, anatomic location, histologic type, or reconstructive procedures and the signs and symptoms of the recurrence, interval between incomplete resection and Mohs micrographic surgery (MMS), or extent of MMS resection. DESIGN: During 20 years, all patients with incompletely excised BCC of the head referred for MMS were sequentially prospectively accrued into the cohort. SETTING: An outpatient MMS practice. PATIENTS: Nine hundred ninety-four patients. MAIN OUTCOME MEASURES: Interval to tumor recurrence, interval to MMS, and extent of MMS as determined by mean surface area resected, depth of resection, and number of tumor nests. RESULTS: The interval to signs or symptoms of recurrence and to MMS from incomplete resection was greater for men, patients older than 65 years, those having a tumor on the nose or cheek, those with aggressive or fibrosing BCC, and those who underwent flap reconstruction (P =.001). The extent of MMS resection was greater for those with flap and split-thickness skin graft repairs. The number of tumor nests identified by MMS was significantly greater in those treated with split-thickness skin graft and flap (P =.001). CONCLUSION: Because it is more difficult to control recurrent BCC, treating tumor remaining at the margin of resection in the immediate postoperative period could result in less extensive surgery.

Age Factors↗
PubMed2005

Three cases of large retinal capillary hemangiomas treated with verteporfin and photodynamic therapy.

OBJECTIVE: To investigate the efficacy of verteporfin and photodynamic therapy in the treatment of large retinal capillary hemangiomas. METHODS: Case reports of 3 patients with large retinal capillary hemangiomas treated with photodynamic therapy using verteporfin. Standard verteporfin dosages (6 mg/m(2) of body surface area) were given. Both standard and modified photodynamic protocols were followed. Modified protocols included shorter verteporfin infusion times and longer light exposure times. RESULTS: Pretreatment best-corrected Snellen visual acuity of the 3 affected eyes were 20/100, 20/50, and 2/200, respectively. All cases had associated exudative retinal detachments involving the macula. Cases 1 and 2 were classic endophytic retinal capillary hemangiomas. Case 3 was a reactive retinal capillary hemangioma. Case 1 had 2 photodynamic therapy treatments, and after 8 months, visual acuity improved to 20/40. Two years after initiating photodynamic therapy, the visual acuity was 20/30 and there was no reperfusion of the hemangioma. Case 2 had 3 photodynamic therapy treatments. The hemangioma was fibrotic, and 20 months after initiating photodynamic therapy visual acuity improved to 20/30. Case 3 had 1 treatment, 11 weeks later and visual acuity improved to 20/400. Four months after treatment, visual acuity returned to counting fingers because of tractional elevation of the macula as the capillary hemangioma fibrosed. Vitrectomy surgery was performed, and choroidal and retinal neovascularization was discovered. Three months after vitrectomy visual acuity was 20/400. In cases 1 and 2, the capillary hemangioma ultimately regressed, and the exudative detachment resolved. CONCLUSIONS: Verteporfin and photodynamic therapy were effective in achieving closure of large retinal capillary hemangiomas. In all cases, the hemangioma underwent fibrosis with consequent macular puckering due to retinal traction. In all cases, the visual acuity improved.

Adolescent↗
PubMed1991

Orbital and eyelid involvement with Erdheim-Chester disease. A report of two cases.

Erdheim-Chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with Erdheim-Chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.

Adult↗
PubMed1984

Experimental and clinical reconstructive surgery of the larynx and trachea.

Laboratory study was undertaken to define the healing patterns of intramural bone and cartilage grafts in the canine trachea. These studies indicate that with solid intramural grafting, both displacement and resorption prevent predictably successful graft incorporation. With graft failure, intraluminal stenting maintained separation of the cricotracheal incisional margins so that strap musculature adhered and subsequently fibrosed to provide a rigid and expanded anterior wall. The concept of direct strap-muscle intramural grafting for tracheal expansion was applied in a clinical setting using eight patients having extensive laryngotracheal stenosis. Successful reconstruction occurred in seven of the eight patients.

Animals↗