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Biomedical subjects

H Hamm

Publications and source records attributed to H Hamm.

At least 91 records · Page 5Linked to original sources

Five-year experience with combined operative and radiotherapeutic treatment of recurrent gynecologic tumors infiltrating the pelvic wall.

BACKGROUND: Whereas 25 to 50% of selected patients with gynecologic tumors who relapse centrally in an irradiated pelvis can be salvaged by exenteration, postirradiation recurrence infiltrating the pelvic side wall generally has been fatal. We have designed the combined operative and radiotherapeutic treatment (CORT) procedure for the treatment of postirradiation recurrence infiltrating the pelvic wall and developed several new techniques for its realization. The aim of the surgery is as follows: (1) total resection of the tumor with only a microscopic margin (R1) at the pelvic wall, preserving the bony pelvis and the neurovascular support of the leg; (2) modulation of the therapeutic index for a second high-dose irradiation of the pelvic wall by transferring autologous tissue from the abdomen or the thigh, and (3) reconstruction of pelvic organ functions lost due to tumor resection. The tumor bed is irradiated postoperatively with brachytherapy through transcutaneous guide tubes implanted at the pelvic wall. METHODS: Between April 1989 and December 1994, we treated 48 patients with postirradiation recurrent or persistent gynecologic malignancies infiltrating the pelvic wall with CORT and followed them prospectively with the following endpoints: tumor control, survival, complications, and quality of life. RESULTS: At a median follow-up of 33 months (range, 3-71 months), the 5-year survival probability calculated with the Kaplan-Meier method was 44%. The overall local control rate was 68%, and 85% in the last 25 patients in the series. The censored severe complication rate at 5 years was 33%. No patient died as a consequence of the treatment. Quality of life was self-assessed with a validated questionnaire by 15 patients without evidence of disease, and was rated with a total of 74% of the maximum score points. Age of the patient, state of resection at the pelvic wall (R1 vs. R2), and recurrent tumor size independently influenced tumor progression after CORT in this series. CONCLUSIONS: CORT appears to be a feasible, innovative treatment with long term survival potential and acceptable quality of life for selected patients with postirradiation gynecologic tumor recurrence infiltrating the pelvic wall. R1 resection of the tumor at the pelvic wall is mandatory; however, the reconstruction options within the pelvis are limited.

Adult↗

[Microcystic adnexal carcinoma of the skin. An underestimated tumor].

Microcystic adnexal carcinoma is a rare semimalignant cutaneous neoplasm characterised by slow but locally aggressive growth. Its histogenesis and nomenclature are still a subject of controversy. Under-recognition and the tumor's bland cytologic features lead to frequent misdiagnoses. In addition, subclinical tumour invasion explains the high incidence of local recurrence. Thus, the tumour makes great demands on the planning of its surgical removal. We report the case of an 81-year-old woman with a microcystic adnexal carcinoma of the lower lip, which was treated by micrographic surgery. On the basis of this case, differential diagnoses, histogenesis and micrographic surgery as the treatment of choice are discussed.

Aged↗

[Giant cell fibroblastoma. A rare soft tissue tumor in childhood].

We report on a 9-year-old girl with a giant cell fibroblastoma of the right anterior chest wall. This rare soft tissue tumour occurs predominantly in the first two decades of life. The typical clinical presentation is a solitary, usually 2-6 cm large, non-tender mass of blue-greyish colour which is mostly located on the back, anterior chest wall, thigh or groin. The histology shows a loose infiltrate of predominantly bland spindle cells in dermis and subcutis. Characteristic elements of the tumour are large angiectoid branching spaces lacking any endo- or epithelium, and relatively small multinucleated cells (floret cells). The recurrence rate is high if the tumour is not excised with adequate safety margins. Metastases are not reported. It is of pre-eminent importance to differentiate this rare benign tumor from sarcomas, in order to avoid an inappropriately aggressive therapy.

Biomarkers, Tumor↗

[White genital piedra].

White piedra is a rare hair infection of man and certain animals, caused by the widespread Trichosporon fungi. The infection is characterized clinically by whitish, soft and easily removable coating of the hair shafts; however, the shafts may be destroyed by the pathogens invading at localized points. While all body hair may be affected, the pubic hair is most frequently affected. This paper is the first report genital white piedra in a 34-year-old Central European man. In all, 22 cases have been described world-wide in young men ranging in age from 20 to 40 years. Only 2 men, aged 42 and 53 years, do not fall in this range. Recently, a major African study disclosed this disease in women for the first time. In moderate latitudes, however, this infection appears to occur almost exclusively in young men.

Adult↗

Involvement of the adherens junction-actin filament system in acantholytic dyskeratosis of Hailey-Hailey disease. A histological, ultrastructural, and histochemical study of lesional and non-lesional skin.

Hailey-Hailey disease is a blistering genodermatosis that shows acantholytic dyskeratosis throughout the epidermis. The aim of our study was to investigate the involvement of adherens structures and cytofilaments in this particular type of acantholysis. Both lesional and non-lesional skin from 18 patients was studied histologically and ultrastructurally. Additionally, the samples were stained for desmosomes, adherens junctions, keratin filaments, actin filaments, and actin-associated proteins, and finally investigated with an electron and a confocal laser scanning microscope (CLSM), respectively. Acantholytic dyskeratosis was not only confined to lesions, but was also focally detectable in clinically unaffected skin. Despite disruption and internalization of the desmosomes, keratinocytes remained linked together by well-preserved adherens junctions. Staining for actin filaments with fluorochrome-labeled phalloidin showed a remarkable formation of actin stress fibers in these keratinocytes. Thus, incomplete acantholysis, as demonstrable in both lesional and non-lesional skin of Hailey-Hailey patients, may be due to a cohesive function of the adherens junction-actin system succeeding the dissolution of desmosomes. Most remarkably, none of the adnexal epithelia expressed the intrinsic defect of cell adhesion. This finding offers an explanation for the successful treatment of Hailey-Hailey disease by dermabrasion, which after complete removal of the involved epidermis results in reepithelialization from skin appendages.

Acantholysis↗

Topical immunotherapy in alopecia areata: anamnestic and clinical criteria of prognostic significance.

BACKGROUND: Topical immunotherapy of alopecia areata (AA) is an effective but time-consuming treatment with unknown long-term risks. OBJECTIVE: The purpose of this study was to identify criteria which allow a selection of patients with a good prognosis for topical immunotherapy with diphencyprone. METHODS: The anamnestic and clinical data of 50 successfully and 55 unsuccessfully treated patients were compared by the Mann-Whitney test. RESULTS: Five factors were found to be of prognostic significance: type of AA (p < or = 0.001), presence of nail changes (p < or = 0.001), duration of AA before treatment (p < or = 0.005), age at onset (p < or = 0.01) and association with atopic eczema (p < or = 0.02). CONCLUSION: A selection of AA patients who are likely to respond to topical immunotherapy is possible on the basis of anamnestic and clinical data.

Administration, Topical↗

Detection of soluble adhesion molecules in pleural effusions.

PURPOSE: Multiple immune functions such as activation of T cells and monocytes or cytolysis of tumor cells are mediated via the adhesion receptor/ligand pairs CD2/LFA-3 and LFA-1/ICAM-1. Because soluble forms of LFA-3 (sLFA-3) and ICAM-1 (sICAM-1) can interfere with these functions, we asked whether increased levels of sLFA-3 and/or sICAM-1 can be found in malignant or inflammatory effusions compared with transudates. METHODS: sLFA-3 and sICAM-1 levels were measured by enzyme-linked immunoassays in pleural effusions from 70 patients (6 transudates, 10 inflammatory, 47 malignant, and 7 other effusions). Twenty pleural fluid samples were tested in parallel for the complete sLFA-3 molecule or sLFA-3-domain 1 only. RESULTS: Increased levels of sICAM-1 were found in all types of exudates compared with transudates. Highest levels of sICAM-1 were measured in malignant exudates, particularly in effusions caused by mesotheliomas, non-small lung cancers, and gynecologic malignancies. This was also true for sLFA-3. However, sLFA-3 levels were not increased in inflammatory effusions. sLFA-3 levels correlated significantly with protein, cholesterol, lactate dehydrogenase, and sICAM-1 levels. Comparison of sLFA-3-domain 1 and the complete sLFA-3 molecule revealed identical sLFA-3 levels, suggesting the absence of nonfunctional split products. CONCLUSIONS: Elevated levels of the complete sLFA-3 molecule were found in malignant pleural effusions, while sICAM-1 level was elevated in both inflammatory and malignant effusions. Secretion of sICAM-1 and sLFA-3 by tumor cells might block T-cell-mediated immune functions such as tumor cytotoxicity. Alternatively, secretion of soluble adhesion molecules might reflect the generalized inflammation within the pleural space.

Adult↗

The scleroatrophic syndrome of Huriez: a cancer-prone genodermatosis.

We report a 24-year-old woman, her 6-year-old son and her 17-month-old daughter, who all suffer from a rare congenital genodermatosis first delineated by Huriez et al. in the 1960s. The clinical features of this autosomal dominant condition include scleroatrophy of the hands and feet, nail hypoplasia, mild palmoplantar keratoderma and hypohidrosis. Histological changes are non-specific, but immunohistological and ultrastructural examination in our index patient revealed an almost complete absence of epidermal Langerhans cells in the affected skin. This new finding may be linked to the cancer proneness of the scleroatrophic skin. In this family, the grandmother had died at the age of 37 years from metastatic squamous cell carcinoma which had arisen on the thenar eminence.

Adult↗

Scalp necrosis in temporal (giant cell) arteritis:implications for the dermatologic surgeon.

Temporal arteritis, a variant of giant cell arteritis, is a systemic granulomatous vasculitis of large and medium-sized arteries. Usually the clinical features are dominated by ophthalmological and neurological complications. In rare instances, ischaemic necrosis, especially of the scalp, may lead patients to the dermatologist. We report a 76-year-old woman presenting with a unilateral scalp necrosis, accompanied by a dramatic ipsilateral impairment of vision. Immediately after duplex-sonography of the extracranial vessels and after initiation of corticosteroid therapy, the diagnosis of temporal arteritis was confirmed by temporal artery biopsy. One month later, because of insufficient secondary healing of the ulcer, the defect was covered by a mesh graft. The taking of the graft was delayed due to immunosuppressive therapy, but was complete. The patient unfortunately died as a result of complications related to surgical removal of an aspergilloma in the sphenoid cavity secondary to immunosuppressive therapy. We discuss the technique of artery biopsy and the possibility of surgical management of scalp necrosis in temporal arteritis.

Aged↗

Seasonal onset of bronchiolitis obliterans syndrome in lung transplant recipients.

BACKGROUND: Bronchiolitis obliterans syndrome is the major complication in long-term survival of patients with lung transplants. Bronchiolitis obliterans syndrome is thought to represent a form of chronic allograft rejection and is associated with obstructive airways disease. Viral infections or other exogenous factors may trigger this condition. METHODS: Because respiratory viral infections show seasonal clustering we studied seasonal onset of bronchiolitis obliterans syndrome in 157 lung and heart-lung transplant recipients. Individual baseline values of forced expiratory volume in 1 second were evaluated according to the International Society for Heart and Lung Transplantation criteria. For bronchiolitis obliterans syndrome classification, values of forced expiratory volume in 1 second were determined by the average of two measurements made at least 1 month apart. Onset of bronchiolitis obliterans syndrome was defined as the date of the initial pulmonary function test showing a persistent decline of forced expiratory volume in 1 second. Other factors causing obstructive airways disease were excluded. RESULTS: Forty-nine patients (31%) showed development of bronchiolitis obliterans syndrome (n = 10 stage I, n = 13 stage II, n = 26 stage III) with onset of bronchiolitis obliterans syndrome 507 +/- 372 days (mean +/- standard deviation) after transplantation. Baseline value of forced expiratory volume in 1 second was reached at 270 +/- 231 days. Between January and March of each year onset of bronchiolitis obliterans syndrome developed in 23 patients (47%). In the second (April to June) and third (July to September) quarters a persistent decline of pulmonary function test results developed in 13 (27%) and 12 (24%) patients, respectively, whereas only 1 patient (2%) showed deterioration between October and December (p < 0.001). CONCLUSIONS: Seasonal clustering of onset of bronchiolitis obliterans syndrome might thus indicate underlying unknown infectious triggers.

Adult↗

[Life threatening angioedema caused by acquired C1 inhibitor deficiency associated with paraproteinemia and livedo racemosa].

A 61-year-old patient with life-threatening angioneurotic oedema was found to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enzymatic activity), C2, C4 and CH50, which are characteristic for the hereditary form of angioneurotic oedema, markedly lowered C1q was found, which is typical for the acquired form. There were no antibodies against C1-INH. Repeated thorough examination disclosed no neoplasm, though the presence of neoplasm has often been reported to be associated with the acquired C1-INH deficiency. However, the patient showed persistent paraproteinaemia and paraproteinuria and developed livedo reticularis. Treatment with danazol resulted in a rise of the complement fraction levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.

Angioedema↗

Naproxen-induced pseudoporphyria: appearance of new skin lesions after discontinuation of treatment.

Non-steroidal antiinflammatory drugs (NSAIDs) are routinely used in the therapy of chronic inflammatory joint diseases in childhood. Recently the NSAID naproxen was recognized to induce pseudoporphyria. This rare photodermatitis is characterized by skin fragility and vesiculation, resulting in shallow scarring. We report 4 children with juvenile rheumatoid arthritis who developed naproxen-induced pseudoporphyria. All children had received naproxen for more than 5 months when pseudoporphyria occurred. A disorder of porphyrin metabolism was excluded by analysis of the urine, serum and erythrocytes. Previous reports on naproxen-induced pseudoporphyria described a rapid disappearance of blisters after discontinuation of treatment. However, in our patients, new lesions appeared for up to 5 weeks after discontinuation of the therapy and skin fragility was apparent for up to 6 months after cessation of treatment. Since naproxen is a widely used drug in the treatment of children with juvenile rheumatoid arthritis parents of fair-skinned children should be alerted to the possibility of this rare adverse effect.

Arthritis, Juvenile↗

Sclerosis of the skin in the GEMSS syndrome. An overproduction of normal collagen.

BACKGROUND: We describe a recently observed set of autosomal dominant GEMSS (glaucoma, lens ectopia, microspherophakia, stiffness of the joints, and shortness) syndrome in a 47-year-old woman and her 23-year-old son. In addition, sclerosis of the skin, from which both patients suffered, is investigated in detail. OBSERVATIONS: The histologic examination of skin biopsy specimens obtained from the upper aspects of the backs of both patients revealed a markedly thickened dermis. Immunohistochemical examination of the dermal collagen bundles showed a collagen pattern similar to systemic sclerosis and normal control skin. In situ hybridization showed a markedly enhanced gene expression of transforming growth factor beta 1. CONCLUSION: The sclerotic skin changes in GEMSS syndrome are the result of an abnormally increased production of normal collagen that might be attributable to the enhanced in situ production of transforming growth factor beta 1.

Abnormalities, Multiple↗

Elevated surfactant protein A in bronchoalveolar lavage fluids from sarcoidosis and hypersensitivity pneumonitis patients.

Interstitial lung diseases often are accompanied by histopathologic evidence of alveolar type 2 cell alterations. In the alveolar milieu, the surfactant-specific protein A (SP-A) is a secretory product of alveolar type 2 cells. Therefore, we measured SP-A levels in bronchoalveolar lavage (BAL) fluids from patients with untreated sarcoidosis (n = 35) and hypersensitivity pneumonitis (HP [n = 10]) and compared the results with those from 21 healthy control subjects. In sarcoidosis patients, SP-A was markedly higher than in control subjects with a mean of 8.0 micrograms/ml of recovered BAL fluid +/- 0.7 SEM (p < 0.0001 compared with control subjects). In HP, SP-A values were comparable with those in sarcoidosis with a mean of 9.0 micrograms/ml +/- 1.7 SEM. Mean SP-A in the control group was 4.0 micrograms/ml +/- 0.3 SEM. These results suggest that SP-A secretion is stimulated in sarcoidosis and HP. Further studies seem justified to investigate the role of the surfactant system in interstitial lung diseases as well as the potential clinical usefulness of SP-A measurements in BAL.

Adolescent↗