Biomedical subjects

M Pfaltz

Publications and source records attributed to M Pfaltz.

32 records · Page 2Linked to original sources

PubMed1992

[Primary pulmonary hypertension and mesangioproliferative glomerulonephritis in HIV infection].

Four years after an HIV infection and without any preceding illness characteristic of AIDS, a 24-year-old woman developed dyspnoea on exertion and peripheral oedema. She had for several years been an intravenous drug addict and contracted hepatitis A and B. There were no symptoms of the HIV infection. Clinical, radiological and echocardiographic examination demonstrated right ventricular failure caused by pulmonary hypertension not due to pulmonary embolism or another known aetiology. The patient died suddenly 9 months after the diagnosis from heart failure. Autopsy established primary pulmonary hypertension with pathognomonic plexogenic pulmonary arterial disease which had led to cor pulmonale with overload myocarditis. Although there had been no clinical signs of renal failure, there was histological evidence of mesangioproliferative glomerulonephritis and non-destructive interstitial nephritis. This case demonstrates that, in addition to the typical AIDS-associated diseases, other rarer syndromes may, in uncertain ways but connected with the HIV infection, decide the prognosis of such patients.

Adult↗

PubMed1991

Cylindroma overexpresses collagen VII, the major anchoring fibril protein.

The cell clusters of the human adnexal tumor, cylindroma, are surrounded by an unusual, thick, unorganized basement membrane-like zone in vivo. Ultrastructural analysis of this electron-dense zone revealed recesses that contained numerous anchoring fibrils. Indirect immunofluorescence staining with antibodies to collagen VII, the major structural component of the anchoring fibrils, showed strong fluorescence of this zone. Collagen VII could be isolated from tumor tissue by limited pepsin digestion or by extraction with chaotropic agents. Cylindroma cells were cultured from the tumor and passaged several times. The cells exhibited a strong expression of collagen VII in vitro, when assayed with immunofluorescence staining or immunoblotting of culture extracts. These results suggest that cylindroma can be used as an abundant source to produce collagen VII in vitro to study the biosynthesis and regulation of collagen VII and the formation of the anchoring fibrils, as other culture systems have failed to produce ample amounts of this anchoring fibril protein.

Aged↗

PubMed1991

CO2 laser therapy for steatocystoma multiplex.

The case of a 22-year-old Philippine woman with a nonhereditary, classical syndrome of steatocystoma multiplex with histological and ultrastructural findings is reported. The good results of CO2 laser therapy are shown and the literature is reviewed.

Adult↗

PubMed1990

Epidermolysis bullosa dystrophica inversa in a child.

A 4-year-old child with dystrophic epidermolysis bullosa inversa is described. Clinical features were blistering of the skin, erosions, scarring and milia formation. The areas involved included the trunk, with preference for the axillary and inguinal folds, the neck and sacral area, and proximal extremities. Notably, the hands and feet were completely spared, with only mild nail dystrophy. Ultrastructural analysis revealed dermolytic blistering and absent or rudimentary anchoring fibrils. Collagen VII, the main structural protein of these fibrils, was present in the skin, as shown by indirect immunofluorescence. These findings suggest that a mutation that prevents appropriate supramolecular aggregation of collagen VII into anchoring fibrils may underlie this subtype of dystrophic epidermolysis bullosa in some patients.

Autoantibodies↗

PubMed1990

Banded structures in multiple familial trichoepithelioma.

A case of multiple familial trichoepithelioma was studied by electron microscopy. Tumour cells showed a dilated, rough endoplasmic reticulum (rER), in which banded, electron-dense structures measuring approximately 50 nm in width were found. Some of these banded structures were arranged parallel to each other with an interval of 250 nm.

Aged↗

PubMed1989

Malignant haemangioendothelioma of the thyroid, immunohistochemical evidence of heterogeneity.

26 cases of malignant haemangioendothelioma (MHE) of the thyroid gland were investigated immunohistochemically with the endothelial marker UEA-1 lectin and the panepithelial marker Lu-5. The results were compared with the results of staining for factor VIII-related antigen in the same cases observed in a previous study of Pfaltz et al. The 26 cases were classified on light microscopic grounds without reference to the immunohistochemical results as classical MHE (15 cases) and borderline cases intermediate between MHE and undifferentiated carcinoma (11 cases). 7 of the 15 classical MHE revealed one or both of the vascular markers, but did not express the epithelial marker. One case showed no staining and another reacted only with Lu-5. Vascular and epithelial markers were found in 6 cases of the 15 classical MHE and in 2 of the 11 borderline cases. These findings indicate that MHE of the thyroid may represent a heterogeneous group of lesions. Tumours positive only for endothelial markers strongly support the hypothesis that MHE is of endothelial origin, whereas tumours which reacted only to the epithelial marker may be undifferentiated carcinomas. Cases with both epithelial and endothelial features on immunohistochemical investigation may represent either tumours in which the malignant cells are in transition from epithelial to mesenchymal differentiation as suggested by Eckert et al. or are tumours of malignant endothelial cells with epithelial differentiation particularly of their cytoskeleton.

Aged↗

PubMed1989

Type VII collagen is a component of cylindroma basement membrane zone.

Cylindroma basement membranes (BMs) were studied by electron microscopy and immunofluorescence with antibodies to Types IV and VII collagen in order to correlate the ultrastructure with the biochemical characteristics. Type IV collagen served as a marker for lamina densa structures and Type VII collagen for the anchoring fibrils. Ultrastructurally, the cylindroma BMs were composed of a wide band of BM-like material in which numerous anchoring fibrils were embedded. The BMs surrounding the tumor cell clusters and nodules of BM-like material entrapped within the tumor cell islands stained positively for both Types IV and VII collagen. We conclude, that anchoring fibrils are a major component of cylindroma BM zone. A trichoepithelioma investigated as control showed a BM similar to the one found at the dermoepidermal junction, i.e. a BM distinctly different from the one of cylindroma. The trichoepithelioma BMs and nodules of BM-like material included within the tumor also stained positively for both Type IV and VII collagen.

Adult↗

PubMed1989

[Follow-up and ultrastructure of plaque-type blue nevus].

A 46-year-old patient suffering from a plaque-type blue nevus of the right neck and ear region is reported. The clinical behavior over 28 years and the histology and ultrastructure support the benign nature of this extremely rare variant of common blue nevus. Periodic observation is therefore recommended, and excision should only be performed in cases causing cosmetic problems.

Cell Transformation, Neoplastic↗

PubMed1987

Immunohistochemistry in the diagnosis of malignant mesothelioma.

The histological diagnosis of malignant mesothelioma of the pleura, especially the distinction from peripheral adenocarcinoma of the lung, may be difficult. The immunohistochemical reports previously published on this subject show diverging results mainly because a variety of antibodies and staining techniques have been used by the different authors. To obtain comparable and reproducible results standard techniques and commercialized antibodies should be applied in routine pathology. In order to investigate the value of immunohistochemistry for the separation of the two entities formalin fixed and paraffin embedded blocks of 47 mesotheliomas and 22 adenocarcinomas were investigated with the PAP technique and commercially available antibodies to carcino-embryonic antigen (CEA), keratin, vimentin, epithelial membrane antigen (EMA), pregnancy specific antigen (SP1), S-100 protein and monoclonal antibody lu-5 (mAB lu-5). CEA positivity was found in all 22 adenocarcinomas examined, but only 2/47 (4%) of all mesotheliomas showed a positive result. SP1 was positive in 13/22 (59%) of the adenocarcinomas, whereas only 3/47 (6%) mesotheliomas were positive for this marker. No significant difference in the rate of positive cases in the adenocarcinoma and mesothelioma group could be found with the other above mentioned antigens. The results of our study indicate that especially CEA, but also SP1 are valuable markers in the diagnosis of malignant mesothelioma.

Adenocarcinoma↗

PubMed1986

Abnormal basement membrane structures in autoimmune thyroid disease.

Thyroidectomy specimens of 8 patients with focal thyroiditis, 7 with diffuse thyroiditis, 13 with Graves' disease and 5 with nodular goiter were investigated by electron microscopy for changes in the follicular basement membrane. Five different types of abnormal basement membrane structures (ABMS), analogous to the ones described in the glomerular basement membrane, were found. Type 1 ABMS were present in all diagnostic groups and seemed to be nonspecific. Type 2 ABMS, thought to represent immune complex deposits, were frequently present in patients with focal or diffuse thyroiditis and in patients with Graves' disease, but could not be demonstrated in thyroidectomy specimens of patients with nodular goiter. The occurrence of type 2 ABMS in both focal and diffuse thyroiditis suggests a common etiology of these two morphologic entities. ABMS types 3, 4, and 5 could not be detected in nodular goiter but were seen in various amounts in all other diagnostic groups. The origin of these structures remains to be explained.

Adult↗

PubMed1986

Budd-Chiari syndrome in sarcoidosis.

A 34-year-old previously healthy woman presented with bilateral interstitial lung changes and thrombotic occlusions of the hepatic veins. A transcaval wedge resection of the liver and a hepato-caval anastomosis were performed. This operation reestablished the impaired intrahepatic venous flow and relieved the portal hypertension and the associated symptoms. Histopathological examination of both liver and lung tissues revealed noncaseating granulomas without microorganisms, confirming the suspected diagnosis of sarcoidosis. The hepatic veins were demonstrated to be narrowed by sarcoid granulomas forming the mechanical basis for venous stasis and extensive thrombotic occlusions. To our knowledge only one other case of a Budd-Chiari syndrome occurring in a patient with sarcoidosis has been reported.

Adult↗

PubMed1984

[Glomerular changes in interstitial kidney diseases].

49 kidney specimens of patients with primary interstitial kidney disease (hydronephrosis in 12, tubulo-interstitial nephritis in 13, analgesic nephropathy in 10, adult polycystic renal disease in 9, oxalosis in 4, and renal dysplasia in 1) were examined for glomerular lesions. Light microscopic investigation showed severe focally and segmentally accentuated glomerular changes in 13 cases (27%). Lesions resembling focal sclerosis were found in 11 cases and focal-segmental mesangial proliferation in 2 cases. The results reveal that glomerular lesions occur frequently in primary interstitial kidney disease and can be found in all of the above-mentioned diagnostic entities.

Adolescent↗

PubMed1983

Mixed medullary and follicular carcinoma of the thyroid.

We report a case of medullary carcinoma of the thyroid which on light microscopy showed not only the well known arrangement of cells in sheets and nests but also unequivocal follicular structures. These follicular structures are present both in the primary tumor and in lymph node metastases. Immunohistochemical investigations revealed that the cells lining the follicles produce thyroglobulin, whereas the remaining tumor tissue is positive for calcitonin and carcinoembrionic antigen. This case represents a medullary carcinoma of the thyroid with an atypical pattern consisting of both thyroglobulin and calcitonin producing cells.

Adenocarcinoma↗

PubMed1983

Malignant hemangioendothelioma of the thyroid and factor VIII-related antigen.

Thirty-six malignant hemangioendotheliomas of the thyroid were examined immunohistochemically using antibody probes to factor VIII-related antigen in order to reevaluate the histogenesis of this neoplasia. The 36 cases were reclassified according to their light microscopic features without prior knowledge of the immunohistochemical results. Three different tumor types were discerned: Group I: classical hemangioendotheliomas (20 cases); Group II: borderline cases between malignant hemangioendotheliomas and anaplastic carcinomas (14 cases) and Group III: anaplastic carcinomas with hemangio-endotheliomatous features (2 cases). Factor VIII-related antigen could be demonstrated in 12 (60%) tumors of group I, 3 (21%) tumors of group II and in neither tumor of group III. Five control cases with the typical histological picture of anaplastic carcinoma of the thyroid were negative for factor VIII-related antigen. The results of our study suggest that at least part of the tumors termed as malignant hemangioendotheliomas are in fact derived from endothelial cells.

Aged↗