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Biomedical subjects

G Hermann

Publications and source records attributed to G Hermann.

At least 55 records · Page 3Linked to original sources

Regression of skeletal changes in type 1 Gaucher disease with enzyme replacement therapy.

A 9-year-old patient presented with an acute onset of pain of the right femur showing destructive changes with periosteal elevation mimicking osteomyelitis. Technetium-99m sulfur colloid (99mTc SC) marrow scan revealed lack of uptake in the right femoral head and the entire shaft of the right femur. Eight months following enzyme replacement therapy, radiography showed almost complete reconstitution of the femoral shaft, and 99mTc SC marrow scan uptake returned to normal.

Bone Marrow↗

Fibrocartilaginous dysplasia.

We present the case of a 53-year-old patient with fibrocartilagenous dysplasia. The area involved was in the proximal femur and presented a lytic lesion with extensive calcifications which radiologically mimicked chondrosarcoma. The radiopathological pattern of this rare entity is discussed in detail and the literature reviewed.

Biopsy↗

Early postoperative flow rates after internal thoracic artery grafting for the left coronary artery system.

OBJECTIVE: The low perioperative flow rates of internal thoracic artery (ITA) conduits have been regarded as a limitation of their use in critical coronary situations with a high myocardial blood demand. To clarify whether these restrictions are justified, early postoperative flow rates were determined. METHODS: Following bilateral ITA grafting, 48 of 106 patients (April 1993-September 1994) underwent recatheterization. Subsequent to control angiography between days 8 and 12, 20 of these patients were studied by intravascular Doppler techniques applied for ITA grafts supplying the left anterior descending artery (LAD) and branches of the circumflex system (CX) (n = 20). Doppler spectral analysis allowed for determination of the average peak velocity and diastolic-systolic velocity ratio. Vascular diameters were assessed by simultaneously performed quantitative angiography and mean flow rates were calculated. All parameters were recorded at rest and following selective stimulation with nitroglycerin (0.2 mg) and papaverine (12.5 mg) to evaluate the graft flow capacity. RESULTS: Baseline values of average peak velocity at rest were 24.6 +/- 11.5 cm/s for ITA-LAD conduits and 21.9 +/- 6.8 cm/s for ITA-CX pedicles. Following dilative stimulation with papaverine, a significant increase in average peak velocities were obtained for both locations (ITA-LAD: 47.3 +/- 17.1 cm/s, ITA-CX: 42.3 +/- 11.8 cm/s). The application of nitroglycerin had a similar effect (ITA-LAD: 42.6 +/- 15.3 cm/s, ITA-CX: 40.3 +/- 10.7 cm/s). The vascular diameters of ITA conduits remained unchanged on nitroglycerin stimulation, whereas papaverine effected significant dilatation in both locations. Flow rates at rest were not significantly different (ITA-LAD: 51.0 +/- 34.2 ml/min, ITA-CX: 44.7 +/- 16.4 ml/min) and maximal flow increase was observed following papaverine stimulation of the LAD conduits (116.1 +/- 90.6 ml/min). Dilative stimulation effected an increase in diastolic-systolic velocity ratios from average values at rest in a range between 34% and 41.7% for both groups and substances. CONCLUSIONS: The basic blood flow in functioning ITA grafts appears to be similar in conduits supplying the LAD and marginal branches. Flow rates between 50 and 60 ml/min at rest should meet myocardial demands, even in the LAD position. Increased flow rates were predominantly based on higher flow velocities with an increased diastolic flow proportion. Enlargement of the graft diameter may exert additional effects, at least following papaverine stimulation at a particular concentration.

Blood Flow Velocity↗

Effect of Graves' intrathyroidal lymphocytes. Effect of Graves' disease intrathyroidal lymphocytes (ITL) on xenotransplants of human thyroid tissue in athymic nude mice.

It has been suggested that ITL are of importance in the pathogenesis of human autoimmune thyroid disease. Aim of our study was to investigate function and morphology of xenotransplanted human thyroid tissue in nude mice following systemic application of lymphocyte preparations from patients with Graves' disease (GD) and non-toxic nodular goiter (NTG). ITL obtained from 13 patients with GD and peripheral blood lymphocytes (PBL) from 12 patients with NTG were injected into nude mice bearing 8 weeks old xenografts of normal human thyroid tissue. ITL- and PBL-subsets were analyzed by flow cytometer (FACScan) before engraftment. Two days after injection the thyroid transplants were examined histologically (HE) as well as immunohistologically by staining with: CD3, CD31, CD45R, HLA class II, ICAM-1, VCAM-1, IgG, IgM and Ki67. Flow cytometry showed a significant higher number of the lymphocyte-subsets CD3, DR+ and CD4 in GD-ITL as compared to the subsets in NTG-ITL. After injection of GD-ITL to the nude mice also a significant higher number of CD3+ human lymphocytes was found in the transplants. GD-lymphocytes stimulated thyroid tissue function significantly more pronounced than NTG-lymphocytes (histomorphological evaluation). In addition, a significant increase of HLA-class II, ICAM-1-, VCAM-1-, CD45-expression and number of Ig presenting plasma cells were observed only after injection of GD-ITL. Our data demonstrate, for the first time in vivo (nude mouse model), that GD-lymphocytes of both peripheral and intrathyroidal origin selectively migrate into human thyroid transplants ("homing"). They survive there for at least two days and induce significant functional as well as histological changes, like expression of gene products and IgG synthesis. These results suggest an important role of ITL in the pathogenetic mechanisms and clinical course of GD.

Animals↗

Epithelioid cells in myoid hamartoma of the breast: a potential diagnostic pitfall for core biopsies.

OBJECTIVE: We report six cases of myoid hamartoma of the breast, a rare benign lesion in which the characteristic smooth muscle cells may have epithelioid histology. We emphasize the importance of radiographic correlation and immunohistochemical studies to diagnosis, particularly on stereotactic core biopsies, to avoid potential confusion with infiltrating lobular carcinoma. DESIGN: Case studies. Prospective and retrospective analysis of six cases, including stereotactic biopsy of two. SETTING: Academic medical center-based pathology practice. PATIENTS: Six postmenopausal women, aged 50 to 59 years, with palpable or nonpalpable mammographically evident breast masses. RESULTS: All the lesions were radiographically well circumscribed, most showing heterogeneous radiodensity. Histologically variable amounts of glandular, fibrous, and adipose tissue were admixed with smooth muscle cells, which occasionally had prominent epithelioid features. All the lesions' myoid cells stained for smooth muscle markers as well as steroid receptor proteins. Stereotactic core biopsy was diagnostic in one case, making excision unnecessary. CONCLUSIONS: With proper radiographic correlation and immunohistochemical confirmation, myoid hamartoma can be confidently diagnosed even on the limited tissue samples yielded by stereotactic core biopsy.

Basement Membrane↗

Resolution of a proximal humeral defect in type-1 Gaucher disease by enzyme replacement therapy.

Although rarely they are a normal variant in children, significant defects in the medial aspect of the proximal humeral metaphysis occur in patients with Gaucher disease due to cortical infiltration and erosion of the periosteum by Gaucher cells. Such changes may lead to pathological fractures in Gaucher patients. These crescentic erosions resolved in a 19-year-old patient with type-1 Gaucher disease following enzyme replacement therapy.

Adult↗

Stress-induced glucocorticoid response modulates mononuclear cell trafficking during an experimental influenza viral infection.

The migration, distribution, and localization of lymphoid cells throughout the body is critical to the efficiency and development of the immune response. This study examined the role of endogenous glucocorticoids in mononuclear cell (MNC) trafficking during the development of an immune response to infection by influenza A/PR8 virus. Accumulation of MNC in the draining lymph nodes and at the site of virus replication (lungs) was studied in infected mice, and infected mice subjected to a stressor (physical restraint). The glucocorticoid antagonist, RU486, was used to block the activity of endogenous corticosterone during development of the immune response. PR8-infected mice demonstrated an elevation in circulating corticosterone regardless of whether they were treated with RU486 or a placebo. Thus, some 'afferent' signal associated with the infection, and/or the immune response to infection, activated the hypothalamic-pituitary-adrenal axis (HPA) and was not subject to negative feedback regulation. The initial accumulation of MNC in the draining lymph nodes and lungs during infection, however, was independent of the glucocorticoid response. Our previous studies demonstrated that virally infected animals subjected to physical restraint had highly elevated plasma corticosterone levels, suppressed lymphadenopathy, and reduced accumulation of MNC in the lungs. In the present study, RU486 treatment restored cellularity to the draining lymph nodes and enhanced accumulation of MNC in lungs of stressed, A/PR8 virus-infected mice.

Animals↗

Determinants of success with spot localization biopsy of the breast.

BACKGROUND: The accurate diagnosis of nonpalpable lesions of the breast by spot localization biopsy depends upon numerous variables. STUDY DESIGN: Multivariate analysis was used to identify variables influencing the success of spot localization biopsy of 757 lesions (27.3 percent of which were malignant) in 738 patients. RESULTS: Ninety percent of the lesions were removed with the first excision as documented by specimen radiography, but 2.3 percent (18 lesions) were not evident on specimen radiography with up to four excisional attempts. Eight (44 percent) of these 18 were in fact removed because they could not be seen on follow-up mammograms. Lesions with calcifications only were significantly more difficult to remove than were masses (87 compared with 96 percent, respectively, p < 0.001). The use of both hooked wire and methylene blue dye was significantly less successful than wire or dye alone (84 compared with 93 and 91 percent, respectively, p < 0.001). A second attempt was successful in 71 percent (52 lesions) of the remaining 75 percent lesions and additional attempts at excision were often associated with success. Success was highly variable among surgeons but could not be related to experience. CONCLUSIONS: Calcifications are more difficult to remove than masses, and combining hooked wire with dye does not improve the success rate. Additional excisional attempts are worthwhile if the lesion is not seen on the first radiograph of the specimen.

Adult↗

Giant cell tumour of the seventh cervical vertebra.

A 43-year-old man presented with bubbly expansion and rarefaction of the body of the seventh cervical vertebra. The lesion involved the right side of the vertebral arch and had a soft-tissue component extending mainly anterior to the vertebra. Histopathologic examination of the specimen obtained by open biopsy revealed a giant cell tumour. Giant cell tumours of the spine, excluding the sacrum, are rare. Radiographically, they may be confused with metastatic carcinoma, plasmacytoma, lymphoma, chordoma and even benign lesions, particularly aneurysmal bone cyst and brown tumour of hyperparathyroidism. However, giant cell tumour occurs mainly in younger patients, involves the vertebral body selectively in most cases and has a bubbly appearance associated with rarefaction and expansion of the vertebral body, characteristics that may be helpful in the diagnosis.

Adult↗

Interval changes in nonpalpable breast lesions as an indication of malignancy.

OBJECTIVE: To determine whether the patterns of interval changes in nonpalpable breast lesions can be used to determine malignancy. PATIENTS AND METHODS: The authors reviewed the records for 128 nonpalpable breast lesions detected by mammography between March 1990 and May 1992 for which previous imaging studies were available. Changes in size, density and contour of the 59 masses and changes in size, number, configuration and pattern for the 69 cases involving microcalcifications were determined from comparison of the current mammograms with the earlier imaging studies, obtained 6 to 42 months previously. These findings were correlated with the pathological diagnosis based on needle localization and excisional biopsy. RESULTS: Twelve (55%) of the 22 new masses and 15 (48%) of the 31 masses that changed were malignant, but none of the 6 masses that did not change were malignant (chi 2 test, p < 0.01). All of the 31 masses that changed increased in size (and of these, 15 [48%] were malignant). In addition, 14 (45%) of the masses that changed exhibited increased density (and of these 9 [64%] were malignant), and 11 (35%) became irregular (and all of these were malignant). Eight (47%) of the 17 cases of new calcifications, 18 (43%) of the 42 cases of calcifications that changed and none of the 10 cases of unchanged calcifications were malignant (chi 2 test, p < 0.01). Twelve (44%) of the 27 calcifications that changed in area and 14 (40%) of the 35 that changed in number were malignant. Fifteen (60%) of the 25 calcifications that changed from scattered to grouped and 8 (53%) of the 15 that changed from punctate to linear were malignant. CONCLUSION: A change in the density or contour of a mass is a fairly definite sign of malignancy. The authors conclude that interval observation is a good method of detecting malignancy in breast masses but is less reliable for detecting malignancy in calcifications.

Biopsy↗

Case report 864. Elliptical, multicentric periosteal osteoid osteoma.

An extremely rare case of multicentric periosteal osteoid osteoma in a child was presented. The lesions were found to be embedded in a narrow elliptical strip, suggesting the possibility that lesional tissue was stretched in a longitudinal fashion as a result of the rapid skeletal growth. In young patients with stretched periosteal reaction who complain of pain, multicentric osteoid osteoma should be suspected. These nidi can be obscured by the exuberant periosteal reaction. Accurate evaluation of the radiographic findings is very important.

Child, Preschool↗

Extraosseous extension of Gaucher cell deposits mimicking malignancy.

Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients.

Adult↗

MRI of malignant fibrous histiocytoma of soft tissue: analysis of 13 cases with pathologic correlation.

We reviewed the magnetic resonance (MR) appearances of 13 malignant fibrous histiocytomas (MFH) of soft tissue and correlated each with the respective lesion's histopathology. The MR images were evaluated for signal intensity on T1- and T2-weighted spin echo sequences, homogeneity of the lesion, presence of internal low signal septations, and margin definition. Histologic subtypes of MFH included storiform-pleomorphic, giant cell, myxoid, and inflammatory. We could not establish a correlation between MR appearance and histopathology. Instead, our series exhibited general features suggestive of malignant soft tissue neoplasms, namely poor margin definition, internal low signal septation, and heterogeneous high signal intensity on T2-weighted images.

Adult↗