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Biomedical subjects

J A Werner

Publications and source records attributed to J A Werner.

At least 19 recordsLinked to original sources

Contact endoscopy of the nose in patients with Rendu-Osler-Weber syndrome.

OBJECTIVE: Teleangiectases are the source of hemorrhage in many HHT patients. Most frequent site of bleeding is the nose and more than 90% of all individuals with HHT suffer from recurrent epistaxis. Despite all efforts, treatment of epistaxis in HHT continues to be a problem for many otorhinolaryngologists, who can alleviate recurrent nosebleeds by Septodermoplasty or laser therapy, but rarely can stop nasal hemorrhages permanently. Recurrence is almost inevitable, but the mechanisms of recurrence are not fully understood. METHODS: Prior to routine Nd:YAG laser therapy of nasal telangiectases the nasal mucosa of 17 patients with HHT according to the clinical diagnostic criteria of the HHT Foundation International was examined with a 0 degrees contact rhinoscope in areas with clinically visible telangiectases as well as in clinically normal mucosa. The digitally recorded images were compared to findings of a group of five healthy volunteers and the findings of five patients with polypoid sinusitis. RESULTS: Visualization of subepithelial vessels was feasible in all individuals of the study group as well as the control groups. Dilated vascular loops and tortuous vessels could be found in the study groups as well as in the control group, but the overall density of telangiectatic vessels was on an average higher in the HHT group. The process of vessel dilatation and tortuous configuration seemed to progress with age. CONCLUSION: Contact endoscopy allows the investigation of the angioarchitecture of capillaries of the nasal mucosa in vivo. This observation may be of significance for studies of nasal diseases, which are accompanied by epistaxis. With this regard it seems to be of special interest for studies of HHT.

Adolescent↗

A historical review of head and neck cancer in celebrities.

INTRODUCTION: The illnesses of celebrity patients always receive more attention from the general public than those of ordinary patients. With regard to cancer, this fact has helped to spread information about the four major malignancies: breast cancer, prostatic cancer, lung cancer and colorectal cancer. Head and neck cancer, on the other hand, is still not well recognised by the lay public, although the risk factors are similar to those of lung cancer. It was the objective of this analysis to identify cases of celebrity patients, the description of which could help to increase awareness of head and neck cancer, its symptoms and risk factors. METHODS: The Internet and medical literature databases were searched for celebrity patients who had suffered from head and neck cancer. RESULTS: The search revealed numerous famous head and neck cancer patients. However, only seven cases were documented well in the medical literature. Among the identified persons were one emperor, two United States presidents, a legendary composer, a world-renowned medical doctor, an outstanding athlete and an extraordinary entertainer. In spite of their exclusive position in society, these patients did not have a better prognosis compared with ordinary patients of their time. Only two of the group experienced long term survival and only one was cured. None of these influential figures used their influence to fund research or to promote knowledge about their respective diseases. CONCLUSION: The identified cases could help increase public awareness of head and neck cancer. Similar to activities in other oncologic fields, current celebrity head and neck cancer patients should be encouraged to discuss their diseases openly, which could have a positive effect on public health.

Famous Persons↗

Evidence of lymphangiogenesis in Warthin's tumor of the parotid gland.

The details of the pathogenesis of cystadenolymphoma (Warthin's tumor) of the parotid gland are still unclear. Neovascularization is considered to be a pivotal factor for solid tumor progression and biological behavior of the tumor. Using double-labeling immunohistochemistry for LYVE-1 and CD34 (specific markers for lymphatic and vascular endothelial cells, respectively) this study analyzes lymphatic vessel density (LVD) and blood vessel density (BVD) in 10 Warthin's tumors and 10 pleomorphic adenomas of the parotid gland as well as in 5 normal parotid glands and 5 normal parotid lymph nodes. There was no significant difference in the intratumoral LVD and BVD among pleomorphic adenoma and normal parotid gland tissue. In contrast, the intratumoral LVD and BVD were significantly higher in Warthin's tumor than pleomorphic adenoma, normal parotid gland and parotid lymph node (P<0.0001 versus P<0.004). The increase in lymphatic vessels in Warthin's tumor suggests that epithelial tumor cells might promote lymphangiogenesis in this kind of lesions.

Adenolymphoma↗

Telemetric monitoring of tracheal pressure after tracheal occlusion for treatment of severe congenital diaphragmatic hernia.

INTRODUCTION: Prenatal tracheal occlusion using endoscopic techniques obstructs the normal egress of lung fluid during pulmonary development and stimulates lung growth in cases of congenital diaphragmatic hernia (CDH). Although FETO might be an effective strategy for treatment of CDH, the mechanism especially due to the supposed increasing transpulmonary pressure is unknown. OBJECTIVE: The purpose of this study was to monitor the pressure below the attached balloon in the fetal lamb telemetrically. METHODS: Four time-dated pregnant Merino ewes underwent fetal and maternal surgery. A special prepared silicone catheter was placed below the epiglottis by laryngoscopy on day 110 or 140 of gestation. The tracheal pressure below the fixed catheter could be monitored telemetrically using the Data Sciences TA11-PA-C40 pressure device. Hundred and twenty measurement points were recorded over a period of 2 min. RESULTS: A maximum of lung pressure rate was found immediately after implantation (23.7 +/- 4.6 mm Hg). During the first hour, the pressure decreased to an average value of 16.9 mmHg. About 70 h after the block, this value decreased to a minimum level of 8.3 +/- 0.4 mmHg. CONCLUSION: Decreasing pressure variation might indicate that lung growth has stopped and that the ideal point of time to remove the balloon is achieved. Increasing pressure has to be related to the morphometric analysis of the lung's structural development and maturation, comparing the efficacy of FETO in preventing or reversing pulmonary hypoplasia. Further investigation of continuous telemetric monitoring of tracheal pressure in the fetal lamb is required.

Animals↗

Early supraglottic cancer: how extensive must surgical resection be, if used alone?

OBJECTIVES: Two centre based evaluations of oncologic results of endoscopic resection of supraglottic cancer without post-operative irradiation. PATIENTS AND METHODS: Twenty-six patients with clinical T1 (n=5) or T2 (n=21) primary squamous cell carcinomas of the supraglottic larynx and with N0 (n=24) or N1 (n=2) neck disease were treated by endoscopic supraglottic laryngectomy coupled with neck dissection(s). Endoscopic resection was standardized whereas neck dissections (NDs) varied from classical modified radical ND to selective ND of levels I to IV. RESULTS: Pathologically, three T2 patients were upstaged to T3, four N0 patients to N1 and one N2 patient down-staged to N1. Within an average of 42 months, there were no local failures and only one regional failure. CONCLUSIONS: Endoscopic resection of T1 and T2 supraglottic cancer without post-operative irradiation achieved good oncological results. No patients with lateralized primary cancers were found to have contralateral cancer on pathological evaluation from bilateral dissections.

Carcinoma, Squamous Cell↗

Meta-analysis of the prognostic significance of perinodal spread in head and neck squamous cell carcinomas (HNSCC) patients.

To assess the risk factor of capsular rupture for individual prognosis and potential therapeutic decision making, the present meta-analysis elaborated the prognostic significance of perinodal spread in a large group of patients suffering from head and neck squamous cell carcinomas (HNSCC). A review of the published literature was conducted, and fixed and random effects models were applied for estimation of the summarised odds ratio and 95% confidence intervals, including a test for homogeneity of the odds ratios. Study methodology allowed the enrollment of only nine studies of 115 published papers. Excluded studies lacked regarding primary tumour location, number and location of lymph node metastases, values on five-year survival, or adequate follow-up data. A summarised odds ratio of 2.7 leads to the conclusion that perinodal spread negatively impacts the five-year survival. The lower confidence limit of more than 2 also supports the concept that perinodal spread significantly reduces (doubled risk) the five-year-survival. These results support the conclusion that perinodal spread is a significant adverse risk factor for survival in patients with HNSCC.

Carcinoma, Squamous Cell↗

[Recurrent nosebleeds in patients with hereditary hemorrhagic telangiectasia].

BACKGROUND AND OBJECTIVE: Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal dominant vascular disease, which can cause multiple symptoms, the cardinal one being chronic recurrent nosebleeds. The aim of this study was to determine, whether these chronic recurrent episodes are felt by the patients to be merely isolated episodes or affect their quality of life (QoL). Also assessed was the extent to which QoL is more impaired in patients with HHT than in those with other chronic diseases. PATIENTS AND METHODS: This prospective cross-sectional study was based on a German questionnaire, "Profile of Quality of Life of Chronically Ill Patients"(PLC) together with a newly formulated questionnaire dealing with HHT-specific symptoms. Comparisons were made with German normative reference data, as well as with data from randomly selected patients with various chronic diseases. RESULTS: 90% of patients with HHT considered their recurrent nosebleeds to be the most disturbing symptom, interfering with hobbies and leisure time in 63% of them. Those with HHT had worse results in 5 of 6 PLC scales than those with other chronic diseases such as cardiomyopathy or rheumatic disease. 73% of the patients stated that they would appreciate new ways of treating these nosebleeds. CONCLUSION: There is a great need for optimizing the treatment of chronic recurrent nosebleeds. Lasting reduction of such bleeds by new therapeutic methods seems unlikely to happen in the near future. For this reason multimodal treatment options, including psychological and relaxation techniques, should be considered. Such an approach may reduce the stress on patients (and subsequent nosebleeds) and result in a direct improvement of their quality of life.

Adult↗

[Adenoid cystic carcinoma secondary to heterotopic salivary gland tissue of the cervical lymph nodes].

BACKGROUND: The presence of heterotopic salivary gland tissue in intra-, periparotideal and cervical lymph nodes is not an uncommen finding. In some rare cases the salivary gland inclusions in lymph nodes may undergo neoplastic transformation. PATIENT AND METHODS: An isolated and indolent tumor was detected in level III in the right cervical area in a 60 year-old male patient. The histological assessment of the extirpated node seemed to indicate lymph node metastasis of an adenoid cystic carcinoma. The radiological and endoscopic findings did not reveal a primary tumor. RESULTS: Due to the fact of missing of a primary tumor, the diagnosis of an adenoid cystic carcinoma in heterotopic salivary gland tissue of the cervical lymph node was established after long-term observation. CONCLUSION: After exclusion of an occult carcinoma of the salivary glands, the possibility of malignant transformation of heterotopic intranodal salivary gland inclusions should be considered in patients suffering from salivary gland carcinoma in the area of the cervical lymph nodes.

Carcinoma, Adenoid Cystic↗

Clinical features of cystadenolymphoma (Warthin's tumor) of the parotid gland: a retrospective comparative study of 96 cases.

The details of the etiopathogenesis of cystadenolymphoma (Warthin's tumor) are still unclear. To explore the possible risk factors for the development of this tumor, medical records of 81 patients with 96 Warthin's tumors of the parotid glands were compared retrospectively with those of 91 patients with pleomorphic adenoma. The medical history and clinical tumor characteristics of all patients were similar. There were no significant differences between these two patient groups with respect to concomitant diseases, regular medications, and preoperative laboratory findings. However, a significant male predominance of patients with Warthin's tumor could be noted (P<0.05). The male to female ratio was 3.3:1 in patients with Warthin's tumor. Multifocal Warthin's tumor were detected in five cases (6.2%), and 10 patients (12.3%) had bilateral lesions. The odds ratio for the incidence of Warthin's tumor among current smokers compared with never smokers was 8.3 (P<0.0001). Compared with never smokers, clearly higher odds of Warthin's tumor was observed in heavy smokers (more than 30 pack-years) (odds ratio=24.1, P<0.0001) than patients who smoked less than 30 pack-years (odds ratio=4.9, P<0.0001).

Adenolymphoma↗

Clinical patterns of metastasis.

In human solid cancer, lymph node status is the most important indicator for clinical outcome. Recent developments in the sentinel lymph node concept and technology have resulted in a more precise way of examining micrometastasis in the sentinel lymph node and the role of lymphovascular system in the facilitation of cancer metastasis. Different patterns of metastasis are described with respect to different types of solid cancer. Expect perhaps for papillary carcinoma and sarcoma, the overwhelming evidence is that solid cancer progresses in an orderly progression from the primary site to the regional lymph node or the sentinel lymph node in the majority of cases with subsequent dissemination to the systemic sites. The basic mechanisms of cancer metastasis through the lymphovascular system form the basis of rational therapy against cancer. Beyond the clinical patterns of metastasis, it is imperative to understand the biology of metastasis and to characterize patterns of metastasis perhaps due to heterogeneous clones based on their molecular signatures.

Animals↗

[State of the art of classification, diagnostics and therapy for cervicofacial hemangiomas and vascular malformations].

The successful treatment of vascular anomalies depends on profound knowledge of the biologic behavior of vascular lesions and their correct classification. On the base of the clinical course Mulliken and Glowacki developed a biologic classification that was accepted as official classification by the ISSVA (International Society for the Study of Vascular Anomalies). Based on an extended literature research, this manuscript will give an overview of different internationally accepted treatment concepts. Even if a wait-and-see strategy can be recommended in many cases of uneventful hemangiomas in infants the proliferative growth of such lesions requires an adequate treatment indication. Vascular malformations that persist lifelong require treatment in the majority of the cases, especially when clinical symptoms occur. Based on individual parameters such as the diameter, location or growth behavior, different therapeutic options as cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgical intervention and/or embolisation can be performed successfully. None of those treatment concepts, however, represents the only treatment method of choice.

Adrenal Cortex Hormones↗

Mutation analysis in hereditary haemorrhagic telangiectasia in Germany reveals 11 novel ENG and 12 novel ACVRL1/ALK1 mutations.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal-dominant disease characterized by recurrent epistaxis, mucocutaneous telangiectasias and visceral arteriovenous malformations. Mutations in endoglin (ENG) and activin A receptor type II-like kinase 1 (ACVRL1 or ALK1) have been found in patients with HHT. We have screened a total of 51 unselected German index cases with the suspected diagnosis of HHT. We identified 30 different mutations in 32 cases (62.7%) by direct sequencing. Among these mutations, 11 of 13 ENG mutations and 12 of 17 ACVRL1 mutations were not previously reported in the literature. Two of the ACVRL1 mutations were each shared by two families. An analysis of the genotype-phenotype correlation is consistent with a more common frequency of pulmonary arteriovenous malformations in patients with ENG mutations than in patients with ACVRL1 mutations in our collective.

Activin Receptors, Type II↗

Value of digital volume tomography in patients with conductive hearing loss.

Digital volume tomography (DVT) is an extension of panoramic tomography. With this diagnostic technique, characterized by high resolution, a narrow section width (0.125 mm) and three-dimensional display, small pathological processes can be well visualized. Twenty-five patients with the history of a progressive hearing loss were examined with DVT (Accu-I-tomo, Morita, Japan). The results were compared with pre- and intraoperative findings to evaluate the diagnostic value of DVT in cases of erosion of the ossicular chain. With high resolution and artifact-free demonstration of the middle ear and the ossicular chain, it was possible to define its continuity preoperatively by DVT in all 25 cases. An intact ossicular chain was found by DVT in 13 cases and was later confirmed by surgery. The predicted erosion of the ossicles was verified in 12 patients, and a tympanoplasty type III was performed. Digital volume tomography is an excellent technique to examine the middle ear cleft and inner ear, and expands the application of diagnostic possibilities in the lateral skull base. Therefore, improvement in preoperative diagnosis is achieved along with more accurate planning of the surgical procedure. Digital volume tomography delivers a small radiation dose with a high resolution and a low purchase price for the equipment.

Adult↗

The status of neck node metastases in breast cancer--loco-regional or distant?

Metastases to cervical lymph nodes do not exclusively derive from malignancies of the head and neck area. In the literature the region where distant metastases of breast carcinomas to the neck occur is exclusively named "supraclavicular". The system established by head and neck surgeons regarding neck node topography allows interdisciplinary management of patients with cervical lymph node metastases from breast cancer. Twelve patients suffering from breast cancer who presented with cervical masses have been examined. Most lymph node metastases were found in the posterior triangle of the neck and at the caudo-jugular level, but some metastases were even found in the upper jugular levels. The results presented show that neck node metastases of breast cancer are located superiorly to the supraclavicular region in more than 50% of the cases. According to the AJCC Staging System for Breast Cancer metastases located in the supraclavicular fossa are assessed as loco-regional metastases (N3c). Lymph node metastases situated above the supraclavicular region are not mentioned, but should be considered as distant metastases. This important question remains unanswered and deserves clarification in the current classification of the AJCC Staging System for Breast Cancer.

Adult↗

Tissue that has lost its track: Warthin's tumour.

The origin of cystadenolymphoma (Warthin's tumour) of the parotid gland is unknown. According to the most favoured hypothesis, Warthin's tumour results from the proliferation of salivary gland ductal cells that were entrapped in parotid lymph nodes during embryonal life. If this is correct, and the almost total lack of recurrence and malignant transformation of this lesion lends credence to this view, Warthin's tumour is correctly classified in the group of tumour-like lesions and should not be considered to be a monomorphic adenoma.

Adenolymphoma↗

[Anesthesiological management of the EXIT procedure. Case report and literature review].

The ex-utero intra-partum (EXIT) procedure enables the surgeon to perform invasive procedures in an infant during cesarean section before clamping the umbilical cord. Specific anesthesiological concepts are necessary for ensuring sufficient umbilical perfusion. We report the case of a 33-year-old female undergoing cesarean section in the 36th week of pregnancy because of a large fetal cervical tumor. The EXIT procedure was performed in order to secure the infant's airway during delivery. The anesthesiological management and interdisciplinary tasks are discussed in the literature review.

Adult↗

Sialadenosis of the major salivary glands in a patient with central diabetes insipidus--implications of aquaporin water channels in the pathomechanism of sialadenosis.

Sialadenosis, also referred to as sialosis, is a disease of unknown aetiology. It regularly manifests itself as a massive swelling in both parotid regions involving the major salivary glands, preferably the parotid glands and is characterized by lack of any detectable, underlying pathologies. In this case report we describe a 24-year-old white female patient with diabetes insipidus who developed sialadenosis of the major salivary glands during a period of enhanced water requirement, which the patient tried to compensate for by more frequent nasal ADH application. Since ADH acts on aquaporins (AQPs) in the kidney, we were interested if AQP expression in the patients salivary glands was affected. Surprisingly, compared to normal control tissues we observed an extensively high signal for AQP5, which is the dominant AQP found in salivary acinar cells. Interestingly, previous studies on AQP5 knock out mice found AQP5 to be required for cell volume regulation. We therefore suggest that aquaporin water channels and antidiuretic hormone together with a disturbance in the body's water household are potential key-factors in the pathophysiological events leading to the development of the disease entity called sialadenosis.

Adult↗