ARDS as presenting symptom in an infant with CD40L deficiency (Hyper-IgM syndrome Type 1).

We report on a 4 month old male infant with respiratory syncytial virus (RSV) infection leading to acute respiratory distress syndrome (ARDS). A diagnostic algorithm including extended infectiological and immunological work-up revealed absence of CD40-ligand. ARDS was treated successfully with a complex respiratory therapy plus intravenous immunoglobulin substitution. Molecular analysis detected mutations in the CD40L gene (Hyper-IgM syndrome Type 1). The case underlines the importance of an extended diagnostic work-up in an uncommonly severe course of respiratory infection in early infancy.

Morphometric examination of native lungs in human lung allograft recipients.

The aim of the study was to estimate the degree of lung damage in patients with alpha(1)-antitrypsin (alpha1AT) deficiency, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (CF) at the time of lung transplantation. Using unbiased stereological methods, lung-, bronchial- and vessel-volume, capillary length, and alveolar surface area and densities were estimated in recipient lungs from 21 consecutive patients with pre-transplant diagnoses including COPD (n=7), alpha1AT deficiency (n=6) and CF (n=8). Six unused adult donor lungs served as controls. Information relating to patient demography and pre-transplant lung function was obtained by retrospective chart review. Disease groups differed significantly with respect to demographics and pre-transplant lung function. Total lung volume was similar in all groups. Bronchial volume was significantly larger in CF patients compared to the control group (p<0.0001) and to the other two diagnostic groups: alpha1AT deficiency (p=0.0001) and COPD (p<0.0001). Alveolar surface density and capillary length density were significantly lower in patients with alpha1AT deficiency and COPD compared to controls (p<0.0001, respectively) and to patients with CF (p<0.0002, respectively). There were no correlations between clinical lung function and morphometric measurements. We conclude that unbiased microscopic stereological morphometry is an evolving science with the potential to elucidate pulmonary disease pathogenesis.

Lung morphometry by unbiased methods in emphysema: bronchial and blood vessel volume, alveolar surface area and capillary length.

We have estimated lung volume, bronchial volume, vessel volume, alveolar surface area and capillary length in patients who died of lung failure due to emphysema and chronic obstructive pulmonary disease (COPD) and in patients with no clinical signs of respiratory disease. Unbiased morphometric methods were applied to the right lung. The patients with emphysema had equal lung volumes and bronchial and vessel volumes compared to the control group. The alveolar surface area and surface density were significantly decreased to about 67%, of control values. The capillary length and length density were significantly decreased to about 68% of control values. The proportional decreases in alveolar surface area and capillary length suggest that compensatory capillary proliferation has not occurred. These unbiased morphometric studies of emphysema have yielded results in end-stage emphysema that are comparable to those previously reported using biased methods. However, the unbiased methods may provide new insights when applied to earlier stages of the disease.

Extraorbital giant cell angiofibroma.

A number of intriguing fibrovascular mesenchymal proliferations with benign or low grade malignant potential have recently been described. Giant cell angiofibroma was introduced as an entity by Dei Tos et al. in 1995 and initially considered to be a lesion of the orbit. We describe an extraorbital example, indicating that giant cell angiofibroma is not confined to the orbit. Immunologically, giant cell angiofibroma is positive for CD 34, bcl-2 and vimentin, and negative for epithelial and muscle markers, and S-100. The tumor shares several morphological and immunological properties with giant cell fibroblastoma and solitary fibrous tumor, yet it features a histology sufficiently characteristic to allow its categorization as a separate entity. The recommended treatment is complete but conservative excision. Metastases have not been reported.

Macrophage and lymphocyte chimerism in bronchoalveolar lavage cells from human lung allograft recipients.

BACKGROUND: Chimerism is suggested to predict a more favourable prognosis in solid organ transplantation. MATERIAL AND METHOD: Forty-eight bronchoalveolar lavages from 10 patients (5 females and 5 males) who had received sex-mismatched donor lungs were monitored for varying periods of time, of up to 2 years, at regular intervals (median 3.0 (0.5-24) months). To investigate the chimerism in macrophages and lymphocytes in bronchoalveolar lavage cells a cloned 2.12 kilobase large biotinylated Y-chromosome-specific DNA-probe was used for in situ hybridization. RESULTS: Donor macrophages disappeared in seven patients within the first 6 months after surgery (median 3.0 (1-6) months). But 15% donor macrophages could be detected in one patient 1 year and 10% in 2 patients two years after surgery. Donor lymphocytes disappeared in all patients within 3 months (median 1 (0.5-3) months). There was no correlation between periods or severity of acute rejection and percentage of donor macrophages and donor lymphocytes in bronchoalveolar lavage. None of the patients developed obliterative bronchiolitis. CONCLUSION: Macrophage chimerism in lung may exist for several years. Whilst our results do not elucidate the role of local macrophage chimerism, they do not currently support the view that chimerism prevents rejection.

Human lung volume, alveolar surface area, and capillary length.

We compare the effectiveness of morphometric methods for estimating lung parameters. Various stereological methods are applied on human lungs and described in detail. The lung volume was estimated by Cavalieri's principle and by fluid displacement. Both methods are reliable, but Cavalieri's principle is superior when systematic sections are needed or when volumes of parts of the lung are wanted. Point counting demonstrated that 87.5% of the lung is parenchyma, 5.4% is vessel volume, and 7.1% is bronchia volume. Alveolar surface was estimated on vertical and isotropic uniform random tissue (IUR) sections. The capillary length and length density was estimated on projected images of vertical slices (Gokhale method) and on IUR sections. Only minute differences were found whether IUR sections or vertical sections were used. Of the total variation, approximately 2% was due to the stereological variation and approximately 98% was due to the biological variation on IUR sections and vertical sections. Estimates for volumes, surfaces, and lengths coming from model-based and design-based methods gave similar results for human lungs. In our hands, the design-based methods were easier to use and required less time. However, only the design-based methods offer the guarantee of an unbiased estimate.

Quantitative analysis of ultrasound B-mode images of carotid atherosclerotic plaque: correlation with visual classification and histological examination.

This paper presents a quantitative comparison of three types of information available for 52 patients scheduled for carotid endarterectomy: subjective classification of the ultrasound images obtained during scanning before operation, first-and second-order statistical features extracted from regions of the plaque in still ultrasound images from three orthogonal scan planes and finally a histological analysis of the surgically removed plaque. The quantitative comparison was made with the linear model and with separation of the available data into training and test sets. The comparison of subjective classification with features from still ultrasound images revealed an overall agreement of 60% for classification of echogenicity and 70% for classification of structure. Comparison of the histologically determined relative volume of soft materials with features from the still images revealed a correlation coefficient of r = -0.42(p = 0.002). for mean echogenicity of the plaque region. The best performing feature was of second order and denoted Contrast (r = -0.5). Though significant, the latter correlation is probably not strong enough to be useful for clinical prediction of relative volume of soft materials for individual patients. Reasons for this is discussed in the paper, together with suggestions for improvements.

Parachordoma of the sacrococcygeal region--a neuroepithelial tumor.

The parachordoma is a seldom, benign tumor of uncertain histogenesis. A case in a 25-year-old man is presented. The parachordoma in the present study reacted with antibodies to GFA, S-100, NSE and vimentin, but not with antibodies to EMA, keratin and NF. Differential diagnoses are the chordoma, the extraskeletal myxoid chondrosarcoma and the subcutaneous sacrococcygeal myxopapillary ependymoma. The immunohistochemical reactions of these tumors were compared and we found that the parachordoma had an immunologically different staining pattern. The parachordoma is thus immunologically different from the chordoma, the extraskeletal myxoid chondrosarcoma and the subcutaneous sacrococcygeal myxopapillary ependymoma. We conclude that the parachordoma is an entity of its own. The immunohistochemical reactions indicate that the parachordoma is a neuroepithelial tumor with glial differentiation.

[Suspected tumor in undiagnosed bronchial foreign body]. / Tumormistanke ved ikkeerkendt bronkialt fremmedlegeme.

A case of long term retention of a bronchial foreign body in a 62-year old female patient is presented, which mimicked lung carcinoma. Despite a negative history of choking, the diagnosis airway foreign body should be kept in mind.

[Pulmonary coccidioidomycosis]. / Pulmonal coccidioidomycosis.

Coccidioidomycosis is an acute, generally mild, respiratory disease that rarely becomes chronic or disseminated and fatal. The aetiologic agent is Coccidioides immitis, a dimorphic fungus growing in soil. The fungus is endemic in hot semi-arid areas in the USA and South America. Human beings and animals become infected by inhaling dust with arthroconidia. The fungus gives rise to either a purulent or a granulomatous inflammation or both. Clinically and radiologically, it can be confused with neoplasms and tuberculosis. The disease may be fatal in immuno-deprived persons who are also at risk of reactivation of a latent infection. Coccidioidomycosis can be detected serologically. The standard treatment of severe illness is Amphotericin B. The authors present the first histologically and serologically verified case of pulmonary coccidioidomycosis in Denmark. An increased number of coccidioidomycosis can be expected with increased travelling and an increased number of immuno-deprived persons.

Influenza A-associated rhabdomyolysis with acute renal failure.

Rhabdomyolysis induced acute renal failure as a rare complication of influenza A infection has been mainly described in adults. Consideration of this potentially life-threatening complication in pediatric patients presenting with influenza is important as clinical symptoms may be unspecific and early diagnosis leading to prompt treatment is essential to decrease associated morbidity and mortality. We report a 9 year old girl who developed severe rhabdomyolysis with myoglobinuric renal failure associated with influenza A virus infection. Receiving supportive therapy including intensive care management the patient recovered renal function completely.

Appendicitis and Enterobius vermicularis.

A histologic examination was made of all appendices delivered to the Dept. of Pathology, Sundby Hospital, Copenhagen, in 1980-87, to ascertain the incidence of Enterobius vermicularis (pinworm) and the possible relation to acute appendicitis. Altogether 2267 appendices were examined, and E. vermicularis was identified in 4.1%. The highest incidence occurred in the 6- to 15-year age group. Infestation was more frequent in female than in male appendices, with the ratio of 1 male to 1.8 females, except in the 11- to 15-year age group, in which the ratio was 1. There was a highly significant difference in the incidence of E. vermicularis in normal appendices and in inflamed appendices, which may indicate that 1) the presence of E. vermicularis in the appendix can give the symptoms of acute appendicitis, or 2) E. vermicularis leaves or does not enter an inflamed appendix.

Granuloma annulare localized to the shaft of the penis.

A case of granuloma annulare localized to the shaft of the penis is reported. The differential diagnoses are discussed. Penile granuloma annulare is a rare disorder and it is concluded that biopsies of penile lesions are recommended to verify the correct diagnosis.

Epididymal metastasis from prostatic carcinoma.

Epididymal metastasis from prostatic carcinoma is rare. A case mimicking cystadenocarcinoma of the epididymis is presented and the literature is reviewed. Investigation for prostate-specific antigen should always be done in carcinoma of the epididymis, and when subcapsular orchidectomy is performed the epididymis should be carefully examined.

Echo-lucency of computerized ultrasound images of carotid atherosclerotic plaques are associated with increased levels of triglyceride-rich lipoproteins as well as increased plaque lipid content.

BACKGROUND: Echo-lucency of carotid atherosclerotic plaques on computerized ultrasound B-mode images has been associated with a high incidence of brain infarcts as evaluated on CT scans. We tested the hypotheses that triglyceride-rich lipoproteins in the fasting and postprandial state predict carotid plaque echo-lucency and that echo-lucency predicts a high plaque lipid content. METHODS AND RESULTS: The study included 137 patients with neurological symptoms and > or = 50% stenosis of the relevant carotid artery. High-resolution B-mode ultrasound images of carotid plaques were computer processed to yield a measure of echogenicity (gray-scale level). Lipoproteins were measured before and hourly for 4 hours after a standardized fatty meal. A subgroup of 58 patients underwent endarterectomy. On linear regression analysis, echo-lucency (low gray-scale level) was associated with elevated levels of fasting and postprandial plasma triglycerides (P=.0002 and P=.002), IDL cholesterol (P=.0009 and P=.006), and VLDL/chylomicron remnant cholesterol (P=.0003 and P=.0004) and triglycerides (P=.0003 and P=.003), the area under the plasma triglyceride curve 0 to 4 hours after a fatty meal (P=.001), and body mass index (P=.0001). On ANCOVA, body mass index, fasting IDL cholesterol, and fasting plasma triglycerides were independent predictors of echo-lucency. Echo-lucency was associated with increased relative plaque lipid content (P=.02). CONCLUSIONS: Increased plasma levels of triglyceride-rich lipoproteins predict echo-lucency of carotid plaques, which is associated with increased plaque lipid content. Because echo-lucency has been associated with a high incidence of brain infarcts on CT scans, triglyceride-rich lipoproteins may predict a plaque type particularly vulnerable to rupture.

Increased acute phase reactants are associated with levels of lipoproteins and increased carotid plaque volume.

OBJECTIVES: to test the hypotheses that increased acute phase reactants predict elevated levels of lipoproteins and increased plaque volume as well as echolucency of carotid plaques. MATERIALS AND METHODS: the study included 258 patients with >or =50% carotid artery stenosis. Acute phase reactants (orosomucoid, C-reactive protein (CRP)) were measured in the fasting state, and ultracentrifugated lipoproteins before and after a standardised fat load test. Echogenicity of carotid plaques was detected using high-resolution B-mode ultrasound and computer-assisted image processing. A subgroup of 81 patients underwent carotid endarterectomy. RESULTS: on linear regression analysis orosomucoid levels were positively associated with fasting and postprandial levels of all triglyceride-rich lipoproteins, and negatively associated with HDL cholesterol (p -values <0.0001); results for CRP were less pronounced. Orosomucoid and CRP both predicted the presence of an increased carotid plaque volume on univariate analysis (p =0.01 and p =0.02). Finally, orosomucoid was negatively associated with echolucency of carotid plaques ( p =0.05). CONCLUSIONS: elevated levels of acute phase reactants are strongly associated with elevated levels of triglyceride-rich lipoproteins, increased plaque volume, and borderline significantly associated with echolucency of carotid plaques. Elevated acute phase reactants possibly predict severity of atherosclerosis, and presence of lipid-rich, rupture-prone plaques.

Lipid-rich carotid artery plaques appear echolucent on ultrasound B-mode images and may be associated with intraplaque haemorrhage.

OBJECTIVE: To relate the histological composition of carotid artery plaques with morphology as evaluated by B-mode ultrasound. DESIGN: Prospective study. MATERIAL AND METHODS: Seventy-eight symptomatic patients underwent carotid endarterectomy after preoperative ultrasound Duplex scanning evaluating plaque morphology. Morphometric analysis of the removed specimen was performed in order to quantify content of lipid, haemorrhage, calcification and fibrous tissue. RESULTS: Echolucent plaques contained more lipid (p = 0.01) and less calcification (p = 0.01) and fibrous tissue (p = 0.03) than echo-rich plaques. Intraplaque haemorrhage was directly related to lipid content (p = 0.004) and inversely related to amount of fibrous tissue in the plaque (p = 0.02). CONCLUSION: The intensity of the reflected B-mode ultrasound signal appears related to the histological composition of the plaque. The association between intraplaque haemorrhage and a high lipid content may support the theory of the lipid-rich plaque being more prone to rupture.