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Voice prosthesis-assisted speech rehabilitation after total laryngectomy has proven to be successful in the majority of patients and exceeds the results of traditional oesophageal speech. Nevertheless 10-30% failure rates are still reported. In part this is due to prosthesis-related problems, in particular ingrowth of Candida species in the valve-bearing parts of the devices. A new indwelling, low-resistance and valveless voice prosthesis is described; the Nijdam voice prosthesis. The device has an average device lifetime of 19 weeks which appears to be superior to other indwelling voice prostheses. Replacement indications mainly comprise leakage or increased airflow resistance. Most frequent local complications are granulation tissue and hypertrophic scar tissue formation. These occur in 12% of the patients and can easily be treated. Replacement is a simple outpatient procedure. The prosthesis is interchangeable with other types of indwelling voice prosthesis.

Objective: Salivary glands of patients with Sjögren's syndrome (SS) have been shown to be a site of anti-SS-B/La antibody production. The present study investigated differences in the localization of the SS-B/La antigen in labial salivary gland (LSG) tissue between SS and non-SS patients, which may explain the local antigen-driven anti-SS-B/La response., Methods: Distribution of SS-B/La was studied immunohistologically in the LSG biopsy samples of 9 SS patients, 10 non-SS patients, and in normal tissues obtained at autopsy within 2 hours after death, using a mouse monoclonal antibody directed to SS-B/La. In 3 SS and 3 non-SS patients, LSGs were also studied with affinity-purified biotinylated human antibodies directed against SS-B/La., Results: In the non-SS patients, SS-B/La was primarily observed in the nucleoli of acinic cells of the LSGs. Patients with either primary SS or secondary SS showed an accumulation of SS-B/La in the nucleoplasm of acinic cells. In the SS patients, SS-B/La was also detected in the cytoplasm as a diffuse or perinuclear staining. Sometimes, SS-B/La was found along the membrane of acinic cells as well. This aberrant nuclear and cytoplasmic distribution of SS-B/La in SS patients correlated well with abnormalities in the composition of the plasma cell population in the LSGs, but not with a lymphocytic focus score > 1., Conclusion: The accumulation and redistribution of SS-B/La in the LSGs may play an important role in the local antigen-driven anti-SS-B/La response in SS, and can also be used to improve the diagnostic possibilities of the LSG biopsy.

The Groningen, Nijdam and Provox voice prostheses (VP) are all low-pressure, indwelling voice prostheses. Although there are differences concerning the valve mechanism, they have a similar design and are therefore interchangeable. In a prospective study, 845 consecutive replacements were evaluated in 158 patients. Average device lifetime differed significantly from 13 weeks for the Provox VP and 15.8 weeks for the Groningen VP, to 19 weeks for the Nijdam device. Leakage through or around a voice prosthesis was the main replacement indication. Leakage occurred significantly more often with the Provox VP (80.2%) than with the Groningen VP (58.8%) or the Nijdam VP (55%). Increased airflow resistance as a replacement indication occurred significantly more often with the Groningen VP (45.4%) and the Nijdam VP (45.9%) than with the Provox VP (22.7%). Complications during the replacement procedure were rare and usually mild. Replacement was usually an easy outpatient procedure; general anaesthesia was only necessary in 3.0% of 845 replacements, without any significant difference between the three devices. Granulation tissue and hypertrophic scar tissue formation were the most frequent local complications. They occurred in less than 10% of our patients but were significantly more common in patients with a Nijdam VP.

Prosthesis-assisted tracheo-esophageal speech has proven its value in post-laryngectomy voice rehabilitation, although manual occlusion of the tracheostoma during speech is necessary. In contrast a tracheostoma valve enables hands-free speech. We have now had experience with 30 patients using the Blom-Singer tracheostoma valve for more than 6 months and have found that most patients prefer prosthesis-assisted speech with the tracheostoma valve. Measurement of several speech parameters with digital and valve occlusion of the tracheostoma did not show any significant differences between the two speaking conditions. Problems included maintenance of an airtight seal, outward forcing of the valve diaphragm during forced expiration and subjective increased airflow resistance.

Identifying mutations that cause specific osteochondrodysplasias will provide novel insights into the function of genes that are essential for skeletal morphogenesis. We report here that an autosomal dominant form of Stickler syndrome, characterized by mild spondyloepiphyseal dysplasia, osteoarthritis, and sensorineural hearing loss, but no eye involvement, is caused by a splice donor site mutation resulting in "in-frame" exon skipping within the COL11A2 gene, encoding the alpha 2(XI) chain of the quantitatively minor fibrillar collagen XI. We also show that an autosomal recessive disorder with similar, but more severe, characteristics is linked to the COL11A2 locus and is caused by a glycine to arginine substitution in alpha 2(XI) collagen. The results suggest that mutations in collagen XI genes are associated with a spectrum of abnormalities in human skeletal development and support the conclusion of others, based on studies of murine chondrodysplasia, that collagen XI is essential for skeletal morphogenesis.

The tracheoesophageal puncture technique with insertion of a valved prosthesis for speech rehabilitation after total laryngectomy is a reliable and simple procedure for successful vocal rehabilitation. The available voice prosthesis can be separated into indwelling and non-indwelling types. The details of the indwelling long-term prostheses (low-pressure Groningen Prosthesis, Nijdam, prosthesis, Provox prosthesis) are discussed. These prostheses need limited patient care. Replacement of the indwelling prosthesis is a simple out-patient procedure, although it is best performed by an ENT-specialist. The local complication rate is low and generally not significant. Hand-free speech is now possible by placement of a tracheostoma valve. Current experiences are encouraging for continued use.

Background: One of the main features of systemic sclerosis (SSc) is vascular damage, the mechanism of which is not understood. The aim of this study was to investigate if complement (C) regulatory molecules, membrane cofactor protein (MCP), decay-accelerating factor (DAF), and CD59, which normally protect endothelial cells from damage by autologous C, are absent or down-regulated in vascular endothelium of patients with SSc. A deficiency or persistent down-regulation of the above molecules is likely to render vascular endothelium of these patients susceptible to damage by physiologically or pathologically activated C. From this point of view, expression of MCP, DAF, and CD59 was tested on endothelium of skin of normal subjects and patients with diffuse and limited forms of SSc., Experimental Design: Punch biopsies of normal skin (N = 11) and lesional and non-lesional skin of patients with diffuse (N = 5) and limited (N = 5) forms of SSc were obtained and serial sections prepared. Immunoperoxidase staining of these sections was carried out using four monoclonal antibodies (MoAbs) directed to different epitopes of DAF, four to different epitopes of MCP, one to CD59 and one to von Willebrand factor. von Willebrand factor served as a marker of endothelial cells. Besides normal skin, lesional skin of systemic lupus erythematosus and several inflammatory and proliferative diseases, described below, served as controls., Results: The endothelium of normal skin strongly expressed all the three proteins. However, the endothelium of lesional and nonlesional skin of all the 10 patients with diffuse or limited forms of the disease tested, expressed either decreased or undetectable amounts of MCP and DAF. CD59 expression was normal in some patients and lower than normal in others. Aberrant expression of MCP, DAF, or CD59 was not found in other control inflammatory, connective tissue and proliferative diseases studied., Conclusions: In view of the common function of MCP and DAF to protect self cells from autologous C, their decrease or virtual absence from the endothelium of patients with SSc strongly suggests that this deficiency may contribute to vascular damage, resulting in intima proliferation and, finally, fibrosis.

Primary antiphospholipid syndrome or PAPS is characterised by antiphospholipid antibodies and arterial and/or venous thromboses. Numerous other clinical features have been shown to be related to this syndrome. Chorea is a well known but rare phenomenon in systemic lupus erythematosus; it has been shown to be strongly related to the presence of antiphospholipid antibodies. We describe two patients with chorea that appeared to be caused by the PAPS.

In a group of 45 patients with Sjögren's syndrome (SS) and 80 controls the high specificity (95%) and sensitivity (100%) of a recently proposed bivariate quantitative immunohistologic (QIH) criterion for SS, based on percentages of IgA and IgG-containing plasma cells in labial salivary gland (LSG) tissue, was confirmed. The best univariate QIH criterion for discrimination between LSG biopsies of SS patients and controls appeared to be based on the percentage of IgA containing plasma cells, and had a specificity of 99% and a sensitivity of 96%. A criterion based only on the percentages of IgM-containing plasma cells, proposed in another recent study, resulted in a high number (31%) of false negatives. Interobserver reproducibility of QIH diagnoses was excellent. Moreover it was demonstrated that accuracy, precision and the interobserver reproducibility of plasma cell counting depends on the choice of tissue fixation and immunohistologic staining procedure. The combination of formol sublimate fixation and peroxidase anti-peroxidase procedure appeared to be the best combination for QIH examination. Furthermore, in 2 SS patients systemic monoclonal IgM/kappa gammopathy was preceded by high predominance of IgM and kappa containing plasma cells in the plasma cellular infiltrate of the LSG tissue.

In this paper an immunodominant epitope of Topoisomerase I is described. An epitope expression sublibrary was constructed from Topoisomerase I cDNA. The subclones were screened with an antiserum from a patient with systemic sclerosis (SSc). The positive clones defined one immunodominant B cell epitope (epitope III), which was located at the carboxyterminal part of the protein. The epitope, 52 amino acids in length, neither contains the p30gag sequence nor the suggested active site Tyr-723, both presumed antibody recognition sites. More than 70% of our anti-TopoI sera recognize this epitope III, indicating that it is a major recognition site of the anti-TopoI autoantibodies in SSc sera. DNA relaxation experiments show that all sera that recognize epitope III and most sera with antibodies to other epitopes inhibit Topoisomerase I activity.

Labial salivary gland histopathology has long been considered to be the most disease-specific single test for diagnosis of Sjögren's syndrome. However, the diagnostic yield of widely used grading systems of focal lymphocytic sialoadenitis is rather low. Determination of the percentages IgA-, IgG-, IgM-, kappa-, and lambda-containing plasma cells in minor salivary glands obtained from a lower lip biopsy has recently been shown to be extremely valuable in Sjögren's syndrome, not only for diagnosis, but also for prognosis with regard to the development of systemic monoclonal lymphoproliferative disease. This technique greatly improves the value of labial salivary gland biopsy in Sjögren's syndrome.

A retrospective analysis was performed to evaluate with the efficacy of elective supraomohyoid neck dissection (SOND) with frozen section (FS) analysis in 57 newly diagnosed patients (62 SONDs) with squamous cell carcinoma of the oral cavity. The protocol included sampling of both the most suspect and largest node in the jugulodigastric region (if present) and the most distal jugulo-omohyoid lymph node (if present). These nodes were then studied with FS histological examination. In the absence of evident nodes for FS analysis during surgery, histological examination uncovered occult metastatic disease in 3 of 11 SOND specimens. Among the remaining patients FS analysis revealed occult metastatic disease in 10 of the 51 samples (19.6%). In these latter cases surgery was continued using standard or modified radical neck dissection en bloc with the primary tumor. In 1 specimen only a single metastasis was found outside the original extent of the SOND. Among 41 FS analysis reports stating the absence of metastatic disease, histological examination of the SOND specimens demonstrated occult nodal disease in 7 (17%). All of the cervical metastases appeared in the ipsilateral side of the neck. False FS reports did not occur. In the histologically proven absence of metastatic disease in the SOND specimens, disease recurrence in the neck occurred only in 3 cases (7%), all in the presence of local failure: once in the previous SOND area, once in the ipsilateral supraclavicular region and once on the contralateral side.(ABSTRACT TRUNCATED AT 250 WORDS)

A retrospective analysis was performed to evaluate the efficacy of elective supraomohyoid neck dissection in 57 newly diagnosed patients with squamous cell carcinoma of the oral cavity. The protocol included sampling of both the most suspicious and the largest node in the jugulodigastric region (if present) and the most distal jugulo-omohyoid lymph node (if present) for frozen section examination. In 10 cases, frozen section biopsy revealed metastatic disease, and surgery was continued using standard or modified radical neck dissection en bloc with the primary tumor. In another 10 cases, histologic examination of the supraomohyoid neck dissection specimens revealed occult nodal disease at other sites. In the histologically proven absence of metastatic disease in the supraomohyoid neck dissection specimens, disease recurrence in the neck occurred in only three cases (7%), all in the presence of local failure. The results of our analysis support the conclusion that elective supraomohyoid neck dissection with frozen section biopsy appears to be a valid staging procedure and a valuable approach to the management of the clinically node-negative neck in squamous cell carcinoma of the oral cavity.

Recently, a mutant fibronectin gene was identified in skin fibroblasts obtained from sclerotic lesions of 7 Japanese patients with systemic sclerosis (SSc). Two point mutations were found adjacent to the cell-attachment tetrapeptide DNA sequence in exon 7 of the fibronectin gene. In the present study, we investigated whether these point mutations are present in the fibronectin gene of Dutch patients with SSc. We were unable to demonstrate the point mutations in the Dutch SSc patients studied.

Three patients are presented to illustrate the diagnostic and prognostic value of quantitative immunohistological examination of labial salivary gland biopsy in Sjögren's syndrome. In an obvious case of primary Sjögren's syndrome and in a case of rheumatoid arthritis without clinical and serological evidence of secondary Sjögren's syndrome, quantitative immunohistological examination of the labial salivary gland biopsy gave more appropriate information than the widely accepted lymphocytic focus score criterion for Sjögren's syndrome. In the third case quantitative immunohistological examination disclosed monoclonal B-cell expansion in the labial salivary gland biopsy six months before evidence of systemic monoclonal gammopathy appeared.

Arterio-venous shunting has been demonstrated in the limbs of ten subjects with traumatic quadriplegia, as shown by increased venous oxygen concentration and a Doppler sonogram pattern indicative of continuous forward flow. This phenomenon is similar to that seen in the diabetic neuropathic foot, suggesting that autonomic dysfunction is a major factor in the development of foot ulceration in the quadriplegic and in the diabetic.