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Biomedical subjects

A Beham

Publications and source records attributed to A Beham.

At least 19 recordsLinked to original sources
PubMed2002

[Recurrent palmoplantar hidradenitis in childhood].

We describe five cases of recurrent palmoplantar hidradenitis in children. The pathogenesis of this highly characteristic disease presenting with painful erythematous papules and nodules involving palms of the hands, soles of the feet, or both is not clear at the moment. One possible mechanism is a temporal relationship between exposure to dampness and cold, which also applied in four of these children. The self-limited disease tends to reappear. After thoroughly informing the children and their parents on avoiding provoking factors, all children experienced freedom from the disease during a 12-month follow-up.

Child↗
PubMed2001

[Confluent and reticulate papillomatosis. Successful therapy with azithromycin].

Confluent and reticulated papillomatosis is a fairly rare dermatosis of still unknown origin affecting mostly female young adults. The lesions are mainly localized in the midline of the trunk. Systemic treatment is the treatment of choice because the disease is resistant to topical therapy and recurrences are often seen. In recent publications retinoids and minocycline have been reported as the favourite therapeutic approaches. We successfully treated a 19-year-old-girl with azithromycin resulting in a complete healing of all skin lesions. The patient has been free of disease for five months. Based on our own case and data in literature, azithromycin is an effective, reasonable and safe therapeutic alternative.

Administration, Oral↗
PubMed2001

Laryngeal manifestation of gout: a case report of a subglottic gout tophus.

A subglottic tophaceous deposition of urate crystals is a rare finding. We report on a case of a male Caucasian who had a moderate dysphonia without any further laryngeal symptoms. The laryngoscopy revealed a hemispheric lesion on the left subglottic region. An excision biopsy was performed, and the histopathological examination of the dissected specimen showed a tophus. Diagnostic and therapeutic strategies are discussed.

Arytenoid Cartilage↗
PubMed2001

Quantification of mucosal leucocyte endothelial cell interaction by in vivo fluorescence microscopy in experimental colitis in mice.

Leucocyte recruitment to sites of intestinal inflammation is a crucial, multi-step process that leads ultimately to the accumulation of cells in the inflamed tissue. We established a new in vivo model system of experimental colitis to quantify leucocyte-endothelial cell interaction and leucocyte extravasation in the inflamed mucosa of the colon. Furthermore, we investigated the pathophysiological role of ICAM-1 in the intestinal microcirculation in vivo. Using the model of dextran sodium sulphate (DSS)-induced acute and chronic colitis in mice, in vivo microscopy was performed in the colonic submucosal postcapillary venules and the submucosal collecting venules in normal or inflamed murine colonic segments. ICAM-1 expression was blocked by an anti-ICAM-1 monoclonal antibody or by suppressing NF-kappaB activation by gliotoxin. Significant increases in leucocyte adhesiveness (51-fold in postcapillary venules, 30-fold in collecting venules, P < 0.01) and extravasation (6.5-fold) could be demonstrated as early as day 2 of DSS-application in acute colitis (P < 0.01). This was paralleled by increases in both the histological damage scores and myeloperoxidase activities. In chronic dextran sodium sulphate-induced colitis significant increases in leucocyte-endothelium interactions and leucocyte extravasation were observed. Blocking ICAM-1 expression with a monoclonal antibody or gliotoxin, leucocyte sticking and extravasation were significantly down-regulated in vivo compared to controls (> 70%; P < 0.01). This new model system offers the possibility to specifically assess the role of adhesion molecules in the colonic mucosa in vivo as well as to investigate and quantify the effectiveness of experimental therapeutic approaches in acute or chronic intestinal inflammation.

Acute Disease↗
PubMed2001

Prognostic impact of molecular analyses in adenoid cystic carcinomas of the salivary gland.

BACKGROUND: Adenoid cystic carcinoma (ACC) of the salivary gland is a generally slow-growing but highly malignant neoplasm with a remarkable capacity for recurrence. Prognosis is greatly influenced by the histological subtype (tubular, cribriform or solid), presence of tumour at the margins, anatomic size, and lymph node metastases. However, none of these parameters has proven to be an unequivocal predictor of disease activity. Therefore, the current study was undertaken to investigate the prognostic value of molecular markers. PATIENTS AND METHODS: Samples from 22 patients, including 4 patients with recurrent disease, were included in the study. By means of immunohistochemistry, the staining pattern of p53, bcl-2, P-glycoprotein, glutathione S-transferase, and topoisomerase as well as sequence analyses of p53 were performed. These molecules were chosen because of their proven association with poor prognosis and therapy resistance in other malignancies. RESULTS: Homozygous p53 mutations were found in all of the 4 recurrent tumors. The other proteins were detected in some tumors, but showed no correlation with histological subtype or recurrence of tumor. CONCLUSION: The results of the current study emphasize the prognostic value of a p53 alteration as an independent prognostic marker. Further, it could be demonstrated for the first time that proteins known for their association with radio- and chemotherapy resistance can be overexpressed in some ACCs suggesting that those molecules could influence the outcome of new therapeutical approaches.

Biomarkers, Tumor↗
PubMed2000

Radiotherapy as adjunct to surgery for malignant carotid body paragangliomas presenting with lymph node metastases.

PURPOSE: Three cases of malignant carotid body paragangliomas with nodal metastases are reported. PATIENTS AND METHODS: Between 1985 and 1994, 3 female patients (51 to 65 years of age) were referred for postoperative radiotherapy after complete (2) or incomplete (1) surgical excision of a malignant carotid paraganglioma (Shamblin III). Preoperative angiographic embolization of the tumor-supplying arteries was performed in all cases. In 2 patients resection of the internal carotid artery and reconstruction by saphenous vein graft was necessary. Continuous course radiotherapy of the tumor bed (50 to 56 Gy/2 Gy) and regional lymph nodes (50 Gy) using photon beams was delivered in 2 patients. The third patient having had incomplete resection cancelled radiotherapy after 4 Gy. RESULTS: Within an observation time of 110 and 119 months no evidence of recurrence was obtained in both patients irradiated postoperatively. The third patient died of progressive disease. Twelve months after the withdrawn irradiation she presented with a tumor progression into the brain and an ulcerated mass on the right side of the neck and was irradiated consecutively for palliation (Figures 1a to 1f). In none of the patients severe acute or late radiation-induced complications were observed. CONCLUSION: In patients with malignant paraganglioma moderate dose postoperative radiotherapy of the tumor bed and regional lymph nodes is well tolerated. It seems to be effective to prolong local control after surgery, to eradicate microscopic lymphatic disease and eventually to postpone further spreading.

Aged↗
PubMed2000

[Dubowitz syndrome and atopic eczema. Case report of monozygotic twins].

Autosomal recessive inheritance, intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, psychomotoric retardation, and often uncharacterized eczematous skin lesions distinguish the rare Dubowitz syndrome. Here a pair of monozygotic twins with Dubowitz syndrome and clear-cut atopic eczema is presented.

Child↗
PubMed2000

[Cutaneous angiosarcoma].

BACKGROUND AND OBJECTIVES: Angiosarcomas of the skin arise almost exclusively in the following clinical settings: 1. the face and scalp, usually in elderly individuals, 2. lymphedematous regions (lymphedema-associated angiosarcomas), and 3. skin that has been previously irradiated (post-radiation angiosarcomas). Clinical and histopathologic diagnosis of angiosarcoma is difficult often resulting in great delay that obviates against early and possibly successful treatment of these very aggressive neoplasms. Diagnostic problems are described, and prognostic factors as well as the effect of different forms of treatment on the outcome are discussed. PATIENTS/METHODS: Retrospective study of 11 patients with cutaneous angiosarcomas. Clinical presentation, histopathology, therapy and survival time are analysed. RESULTS: Only 1 of 11 cases cutaneous angiosarcoma was clinically identified. Survival time was 1-24 months. Three patients who received radical surgery have not developed metastases and are still alive. CONCLUSIONS: Clinical and histopathologic diagnosis of cutaneous angiosarcomas is often very difficult. Prognosis is very bad; radical surgery seems to be the best therapeutical option.

Adult↗
PubMed2000

[Coloproctological diseases of the pelvic floor].

PURPOSE: The aim of this review is to describe the most frequent and important coloproctologic pelvic floor disorders. Relevant diagnostic procedures of the pelvic floor will be presented. MATERIAL AND METHODS: A host of diagnoses and symptoms such as the descending perineum syndrome, rectocele, cystocele, enterocele, uterine and vaginal descensus, anal and rectal prolapse, outlet obstruction, anismus, inertia recti and intussusception are included under the heading "pelvic floor disorders". Although symptoms are often varied, problems concerning urinary and/or faecal continence commonly lead to primary consultation of a physician. RESULTS: Quite often, apparently divergent symptoms such as constipation and incontinence are simultaneously mentioned. A clear gender disposition is observed with female patients inflicted nine-fold in comparison to male patients. The primary consultant may belong to a variety of specialities such as urology, proctology, gynaecology or dermatology, depending upon the predominant symptom. A feeling of trust is essential for the treatment of a disorder involving highly intimate regions of the body. DISCUSSION: An exact medical history and standardized proctologic evaluation consisting of inspection, palpation, rectoscopy and proctoscopy may be augmented by investigations such as anorectal manometry or endosonography. Conventional defecography has been replaced more and more by dynamic MRI of the pelvic floor in specialized institutions, enabling additional gynaecologic and urologic investigations avoiding ionizing radiation.

Colonic Diseases↗
PubMed2000

Optimizing the therapeutic approach in tinea capitis of childhood with itraconazole.

BACKGROUND: Tinea capitis is the most common dermatophytosis of childhood with increasing incidence. Whereas griseofulvin is considered by many as the mainstay of treatment, newer oral antifungal agents, including fluconazole, itraconazole and terbinafine have demonstrated higher efficacy, resulting in shorter treatment durations. OBJECTIVES: We aimed to determine the optimum regimen for the treatment of childhood tinea capitis with itraconazole. METHODS: A mycological culture outcome-dependent combination of a 28-day continuous and facultative additional 14-day courses with itraconazole was used in 42 children (20 girls; 22 boys) aged 12-140 months (mean 66) with tinea capitis due to Microsporum canis (n = 26) and Trichophyton violaceum (n = 16). The drug was given orally according to the patients' body weight (50 mg daily for < 20 kg; 100 mg daily for > or = 20 kg) over 4 weeks. Direct microscopy and fungal culture as a parameter for efficacy were repeated 2 weeks after termination of treatment. Assessment of efficacy was based on the evaluation of results from light microscopy and culture at 8 weeks after initiation of treatment, and in the case of a further positive mycological culture at 14 and 20 weeks, respectively. A positive fungal culture at these times resulted in an additional course for 2 weeks with the initially chosen itraconazole dosage. RESULTS: In 34 of 42 patients a single 4-week course of itraconazole resulted in a complete mycological cure of lesions as demonstrated by light microscopy and mycological culture. Four of 42 patients had to be treated by a second itraconazole course for 2 weeks, and four children received a third course of itraconazole for 2 weeks until all lesions showed negative direct microscopy and mycological culture. No abnormal haematological or biochemical results occurred. Apart from transient, completely reversible indigestion in two children, no side-effects were observed. CONCLUSIONS: A culture-based 28-day continuous therapeutic regimen plus facultative cultural outcome-dependent additional 14-day courses of a body weight-adapted dosage of itraconazole in tinea capitis due to M. canis and T. violaceum is discussed; this offers the advantage of an effective therapy with complete negative direct microscopy as well as negative cultural results, within a shorter active treatment period (cf. previous studies with continuous administration of itraconazole).

Adolescent↗
PubMed2000

CK7 expression in carcinomas of the Waldeyer's ring area.

Primary carcinomas of the Waldeyer's ring area are typically nonkeratinizing squamous cell carcinomas (SCC). Their cervical lymph node metastases are not uncommonly cystic and filled with necrotic tumor cells. Some cysts, however, contain clear fluid. During the investigation of SCC producing "fluid-filled" cystic metastases, we evaluated hematoxylin and eosin (H&E) sections of 90 primary SCC for their site of origin. We analyzed the cytokeratin (CK) profile of primary and metastatic carcinoma with special focus on the expression of CK7, a putative marker for ductal differentiation. CK7 was expressed in submucosal minor salivary gland acini and ducts, but not in the squamous surface epithelium of the Waldeyer's ring. CK7 was expressed in 11 primary SCC (8 base of tongue/3 palatine tonsil). The CK7-positive SCC were deep-seated, arose from large excretory ducts of submucosal minor salivary glands, and showed only insignificant surface involvement. They were characterized by a solid infiltrative growth pattern of basaloid cells with focal ductal differentiation. Salivary ducts adjacent to the carcinoma showed extensive intraductal hyperplasia and metaplasia. All CK7-positive carcinomas produced CK7-positive cystic nodal metastases, most of which contained paucicellular fluid. No solid CK7-positive nodal metastases were identified. In summary, a subset of carcinomas occurring in the Waldeyer's ring area appear to arise from large excretory ducts of submucosal minor salivary glands with only limited surface involvement, express CK7, and produce CK7-positive cystic "fluid-filled" nodal metastases. The histomorphology and immunophenotype suggest that these carcinomas represent basaloid SCC arising from excretory ducts of the submucosal minor salivary glands.

Biomarkers, Tumor↗
PubMed2000

Nasopharyngeal angiofibroma: true neoplasm or vascular malformation?

Nasopharyngeal angiofibromas (NA) are rare tumor-like lesions characterized by architecturally irregular vessels set in a fibrous stroma. The unique morphology, the strong predilection for male adolescents, and the uncertainty about its etiology contributes to significant confusion regarding the classification of NA, which still has not been solved today. Based on immunohistochemical and electron microscopic examinations, we demonstrate in detail the various unusual vascular architectural features of NA. They represent discontinuous vascular basal laminae, focal lack of pericytes, and pronounced irregularity of the smooth muscle layers. In thick smooth muscle layers and pads, the orientation of muscle cells is frequently disturbed, and the individual cells differ in size and shape. Occasionally, the muscle layers disperse peripherally into individual cells, creating the impression of vessel-independent smooth muscle cells within the stroma. The summation of all morphological irregularities demonstrated in this paper allows the conclusion that NA represent vascular malformations.

Actins↗
PubMed2000

Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis.

We present the MR imaging findings in a 43-year-old male patient with bilateral idiopathic sclerosing inflammation of the orbit. Bilateral enhancing retrobulbar masses, with concentric compression of the retrobulbar segment of the left optic nerve, were seen. MR imaging proved to be the only means to distinguish between the different intraorbital structures and to determine the exact site of optic nerve compression. To our knowledge, this is the first documented case of MR imaging findings of this entity.

Adult↗
PubMed2000

Nasopharyngeal angiofibroma: an APC-gene-associated tumor?

Nasopharyngeal angiofibromas are extremely rare, locally invasive tumors of unknown cause exclusively occurring in male adolescents. Recently, 6 cases have been reported in patients with familial adenomatous polyposis coli (Gardners syndrome). Mutations or allelic loss of the adenomatous polyposis coli (APC) gene have therefore been implied in the pathogenesis of nasopharyngeal angiofibroma. The authors analyzed 11 cases of nasopharyngeal angiofibromas from 9 male patients for mutations in the mutation cluster region and allelic loss of the APC gene. Six were primary tumors; 2 first recurrences; and 1, primary tumor with 2 recurrences. Direct sequence analysis was performed on several overlapping polymerase chain reaction (PCR) products. Detection of allelic loss was performed by restriction length polymorphism analysis at a polymorphic locus. No mutation was detected in 11 tumors of 9 different patients. None of the 9 informative (heterozygous) cases carried an allelic loss. We conclude that alterations of the APC gene do not play a major role in the development of nasopharyngeal angiofibroma. The coincidence of nasopharyngeal angiofibromas and adenomatous polyposis coli in some families implies the possibility that another gene in this region might be responsible for the development of nasopharyngeal angiofibromas. HUM PATHOL 31:1411:1413.

Adolescent↗
PubMed1999

CD44 expression in sinonasal melanomas: is loss of isoform expression associated with advanced tumour stage?

The expression of the adhesion molecule CD44 was examined in 14 primary sinonasal melanomas (SMs), aggressive neoplasms with short survival times, as CD44 overexpression has been linked to poor survival in human cancers. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded sections with CD44 isoform-specific monoclonal antibodies to the CD44 standard (s) and variant isoforms (v) v5 and v6. CD44s, v5, and v6 were strongly expressed in a membranous pattern in SM in situ, early invasive SM, and in uninvolved respiratory/squamous epithelium. In invasive SM, membranous CD44s expression was identified in a large proportion of melanoma cells. Membranous staining of CD44v5 and v6 was lost in invasive SM, independently of the histological subtype. Diffuse cytoplasmic staining was observed focally in invasive SM and loss of cytoplasmic expression of CD44v6 and v5 was associated with advanced tumour stage in the linear-by-linear association test (p = 0.042 and 0.066, respectively). CD44s may not be important for malignant transformation, as it is expressed in both benign and malignant melanocytes. Loss of membranous CD44 isoform expression in widely invasive SM suggests that loss of cellular adhesion facilitates matrix and vascular infiltration and dissemination of sinonasal melanoma cells.

Adult↗
PubMed1999

Life-threatening tracheal metastasis complicating ovarian cancer--a case report.

BACKGROUND: Tracheal metastasis is a rare manifestation of recurrent ovarian cancer. CASE: We describe tracheal metastasis causing increasing respiratory distress in a patient with progressive stage IIIc undifferentiated serous-papillary cancer involving the peritoneum and pleura and the retroperitoneal, diaphragmatic, parahilar, mediastinal, pretracheal, paratracheal, and supraclavicular lymph nodes. The situation necessitated rapid endoscopic laser ablation. CONCLUSION: Malignant tracheal obstruction should be considered in the differential diagnosis of patients with advanced ovarian cancer and respiratory distress.

Adult↗
PubMed1999

Cystic lymph node metastases of squamous cell carcinoma of Waldeyer's ring origin.

We analysed in a retrospective study the frequency of cystic lymph node (LN) metastases in neck dissection specimens of 123 patients with primary squamous cell carcinoma (SCC) arising in the palatine tonsils (62 M/14 F), the base of the tongue (38 M/5 F) and the nasopharynx (2 M/2 F). Eighty-two per cent of patients had metastases (64 tonsillar SCC, 33 base of tongue SCC and all four nasopharynx SCC) in 368 LN of a total 2298 sampled LN. Thirty-nine per cent of patients had exclusively solid metastases and 37% of patients had exclusively cystic metastases. A total of 62 patients had some signs of cyst formation in one or more metastatically affected LN (27 with only histological evidence of cyst formation with luminal diameters < 5 mm, 35 with clinically detectable cyst with luminal diameter > 5 mm). Cystic metastases were more common in patients with SCC of the base of the tongue (P = 0.005), while solitary clinically evident cystic metastasis with lumina > 5 mm were found exclusively in tonsillar carcinoma (P = 0.024). In comparison with solid metastases, cyst formation was associated with N-categories (N2b and N3, P = 0.005) in SCC of the base of the tongue origin. No such association was observed for tonsillar SCC (P = 0.65). The primary mechanism of cyst formation was cystic degeneration.

Branchioma↗