[Clinical case presented to the experts].
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Diagnosis, Differential↗
Biomedical subjects
D Stoll
Publications and source records attributed to D Stoll.
Acute asphyxia caused by Gerhardt's syndrome associated with asthma.
Vocal cord dysfunction (VCD) has been frequently discussed as confounding the diagnosis of asthma, and it is recommended to examine vocal cord function in cases of difficult asthma. However, there is also strong evidence that asthma may be associated with VCD, sometimes masquerading as exercise-induced asthma. Gerhardt's syndrome frequently induces severe acute respiratory distress requiring ventilatory support, and this condition might be mistaken for a severe acute asthma attack. Here, a case of bilateral paralysis of the vocal cords, which was associated with asthma, is described.
Acute Disease↗
Epidemiological and clinical aspects of nasal polyposis in France; the ORLI group experience.
Nasal polyposis (NP) is a common condition in patients consulting ENT practitioners in France. A multicenter prospective study was performed to evaluate symptoms, demography, environmental factors, personal and family history and associated conditions like asthma, and food or drugs sensitivity (FDS) in patients suffering from NP. In each investigation center assessments were performed at the moment of the initial consultation by the same investigator, then updated with complementary exploration results required by the protocol. The chi 2 test and the Fisher test were used for statistical analysis. In this study 224 patients were included. Males were predominant at 63%. Asthma was found in 45% of cases without relevant sex difference. However, FDS, positive in 31% of the patients, was statistically higher in females than in males (42.9% vs. 24.4%). Severe and major symptoms were more frequently found in the female population. Environment and habitat factors did not appear to be relevant. High rates of NP (52.66%) and asthma (43.58%) were found in the family history. Hereditary factors were suggested and lead us to further study the genetic factors potentially involved in this pathology.
Asthma↗
[Surgical methods of removal of T4 nasopharyngeal malignancies. A preliminary report on 18 cases].
OBJECTIVES: To describe the technique and evaluate postoperative sequelae and results of transfacial surgical removal of T4N0 nasopharyngeal malignant neoplasms. MATERIAL AND METHODS: Eighteen cases of surgically-treated nasopharyngeal cancers, managed between january 1993 and october 2000, were retrospectively studied. Three patients were non-responders to neoadjuvant chemotherapy, 6 suffered local recurrence following standard treatment and 7 were operated first either because of a huge and osteolytic tumor or because of an uncommon histolopathology. RESULTS: Preoperative Vth nerve neuralgia (8 cases) was alleviated (4 cases) or cured (3 cases). At the time of diagnosis, ophthalmoplegia was observed in 3 cases. It disappeared postoperatively in 2 cases and after following radiation therapy in one. Median follow-up of the series was of 31.8 mths. Four patients were died of disease; one of them from distant metastases 4 years post-surgery. One patient is alive with distant metastases. The 13 last (72.2%) are alive without disease with a 35.6 mths median follow-up (6-77 mths). DISCUSSION: This short and heterogeneous series cannot lead to any evidence-based conclusion. But mid-term free-of-disease survival of 4 of the 5 patients suffering local recurrence and of patients presenting with so-called non-radiation-sensitive tumors is to be considered. In comparison with the high risk of neurological sequels following reirradiation, absence of specific toxicity of surgery observed in this series is to be underlined. CONCLUSION: In selected cases surgical treatment does not demonstrate any toxicity and is able to offer mid-term local control of the disease. Its role is to be evaluated further before inclusion in the therapeutic algorithm of these very bad prognosis-associated tumors.
Adult↗
[Parathyroid cervical hematoma: a case report].
Haemorrhagic cysts of the parathyroid gland are rare. Our case report concerns a presentation with a constrictive cervical haematoma, confirmed by ultrasound-guided aspiration. The haematoma, rapidly recurred, indicating exploration of the neck and haemostasis. Histological examination of excised tissue showed the haemorrhagic nature of the cyst.
Adult↗
Zirconia stationary phases for extreme separations.
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Adsorption↗
Enzymatic synthesis of carbon-fluorine bonds.
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Azides↗
[Ethmoid adenocarcinomas: retrospective study of 76 patients].
From the retrospective study of 76 adenocarcinomas of the ethmoid sinuses, results were expressed in two terms: the morbidity related to surgery and the oncologic outcome. This case study extends between 1975 and 2000. It includes 71 men and 5 women, with an average of 61 years. 81% of them works in wood dust, with a mean duration of exposure of 26 years. The diagnosis of adenocarcinoma is realized in the three months after the first signs, essentially rhinologicals, next neurologicals and ophthalmologicals. The majority of tumors were classified as T3N0M0, i.e. 57.89%. The treatment of this tumors is surgical: 34.2% surgical only and 59.2% with radiotherapy. The transfacial approach (paralateronasal and degloving) and the combined surgery are respectively performed in 36 patients and 35 patients. 23.2% patients had a local recurrence, and 10% developed cervical nodes and systemic metastasis. Survival rate based on the Kaplan-Meier actuarial method is 82% at 3 years, 80% at 5 years, 72% at 10 years. The prognosis of ethmoidal cancer is strictly correlated to local control. Local recurrence is statistically more likely in patients with involvement of the dura, brain and sphenoid sinus. With the analysis of the carcinologics results, we discuss the therapeutics indications of the adenocarcinomas and a new classification. Taking in account the involvement of the dura, sphenoid and orbit. We did not find any statistical differences between T3 patients treated by combined approach (n = 13) or by transfacial approach (n = 15).
Actuarial Analysis↗
[Glomangioma or "glomus tumor" of the nasal cavity: apropos of a new case and review of the literature].
Glomangioma, or "glomic tumour" is a benign soft tissue tumour appearing most commonly at the distal extremities, in the nailbed and subcutaneous tissue. This is a vascular tumour, rare for the dermatologist, and exceptional in an ENT site. We describe here what is, as far as we can tell from the literature, the eighteenth case. The problem is the diagnosis of a vascular tumour, arising usually from the septal mucosa, which has to be distinguished histopathologically from a haemangiopericytoma. Radiological investigation will define the extent of the tumour and guide the treatment plan, which is purely surgical and consists of wide excision to avoid the possibility of a local recurrence.
Adult↗
Mapping of genes encoding glycoside hydrolases on the chromosome of Cellulomonas fimi.
Cellulomonas fimi genomic DNA was digested with HpaI, MunI, HindIII, and NsiI, producing fragments ranging in size from 20 to 1400 kbp that were resolved by pulsed field gel electrophoresis. Genetic and physical linkages were determined by Southern blotting and were used to construct a genome map. Cellulomonas fimi has a single circular chromosome of approx. 4000 kbp. Except for two closely linked genes, cbh6A and cel5A, the genes known to encode glycoside hydrolases are scattered widely on the chromosome.
Actinomycetales↗
[French multicenter prospective epidemiologic study (ORLI Group) of allergic and lung diseases associated with nasal polyposis].
224 patients presenting with nasal polyposis (NP) were included in a french prospective multicenter study. NP was evaluated by nasal endoscopy and computed tomography. Allergic status was documented using skin prick-tests and/or specific IgE. Pneumologic assessment included spirometry with carbamyl-choline hyper-reactivity test or beta 2 mimetic broncho-dilation test. Minimal follow up period was 1 year. 45% of the whole population were considered as asthmatic. Asthma onset occurred before and after the NP onset in respectively 45.7%, 22.3% and 32% of cases; these two conditions started simultaneously in 32% of patients. Skin prick-tests and/or specific IgE were positive in 32.5% of cases. In most of the cases (80%), patients were polysensitized to house dust mite and/or pollens and/or animal danders and/or fungi. 31% of the population had idiosyncrasy, caused by drugs in general and especially aspirin in 44% of cases. The global population could be divided in two groups according to the occurrence of previous polypectomy or not. The group "polypectomy" and the group "no polypectomy" were similar regarding the frequency, the age of onset, the course and the severity of associated asthma. Familial history (parents, children, brothers and sisters) was of great interest: 58.7% of the patients had one (or more) relative suffering from NP, 43.6% of the patients had one (or more) relative suffering from asthma and 12.2% of the patients had one (or more) relative suffering from idiosyncrasy. These results support a genetic etiology for NP.
Adult↗
Identification of Glu-519 as the catalytic nucleophile in beta-mannosidase 2A from Cellulomonas fimi.
Incubation of the beta-mannosidase Man2A from Cellulomonas fimi with 2-deoxy-2-fluoro-beta-D-mannosyl fluoride (2FMan beta F) resulted in time-dependent inactivation of the enzyme (inactivation rate constant k(i)=0.57 min(-1), dissociation constant for the inactivator K(i)=0.41 mM) through the accumulation of a covalent 2-deoxy-2-fluoro-alpha-D-mannosyl-beta-mannosidase 2A (2FMan-Man2A) enzyme intermediate, as observed by electrospray ionization mass spectrometry. The stoichiometry of inactivation was 1:1. Removal of excess inactivator and regeneration of active enzyme by transglycosylation of the covalently attached inhibitor to gentiobiose [Glc beta(1-6)Glc] demonstrated that the covalent intermediate was catalytically competent. Comparison by MS of the peptic digests of 2FMan-Man2A with peptic digests of native Man2A revealed a peptide of m/z 1520 that was unique to 2FMan-Man2A, and one of m/z 1036.5 that was unique to a Man2A peptide. Their sequences, determined by collision-induced fragmentation, were CSEFGFQGPPTW and FGFQGPPTW, corresponding to residues 517-528 and 520-528 of Man2A respectively. The difference in mass of 483.5 between the two peptides equals the sum of the masses of the tripeptide CSE plus that of 2-fluoromannose. It was concluded that in 2FMan-Man2A, the 2-fluoromannose esterified to Glu-519 blocks hydrolysis of the Glu-519-Phe-520 peptide bond, and that Glu-519 is the catalytic nucleophile in this enzyme. This residue is conserved in all members of family 2 of the glycosyl hydrolases. This represents the first ever labelling and identification of an active-site nucleophile in a beta-mannosidase.
Actinomycetales↗
Mannanase Man26A from Cellulomonas fimi has a mannan-binding module.
A modular mannanase (Man26A) from the bacterium Cellulomonas fimi contains a mannan-binding module (Man26Abm) that binds to soluble but not to insoluble mannans. Man26Abm does not bind to cellulose, chitin or xylan. The K(d) for binding of Man26Abm to locust bean gum (LBG) is approximately 0.2 microM. Man26A is the first mannanase reported to contain a mannan-binding module.
Amino Acid Sequence↗
Multispecific CD4+ T cell response to a single 12-mer epitope of the immunodominant heat-shock protein 60 of Yersinia enterocolitica in Yersinia-triggered reactive arthritis: overlap with the B27-restricted CD8 epitope, functional properties, and epitope presentation by multiple DR alleles.
Yersinia heat-shock protein 60 (Ye-hsp60) has recently been found to be a dominant CD4 and CD8 T cell Ag in Yersinia-triggered reactive arthritis. The nature of this response with respect to the epitopes recognized and functional characteristics of the T cells is largely unknown. CD4+ T cell clones specific for Ye-hsp60 were raised from synovial fluid mononuclear cells from a patient with Yersinia-triggered reactive arthritis. and their specificity was determined using three recombinant Ye-hsp60 fragments, overlapping 18-mer synthetic peptides as well as truncated peptides. Functional characteristics were assessed by cytokine secretion analysis in culture supernatants after specific antigenic stimulation. Amino acid positions relevant for T cell activation were detected by single alanine substitutions within the epitopes. Fragment II comprising amino acid sequence 182-371 was recognized by the majority of clones. All these clones were specific for peptide 319-342. Th1 clones and IL-10-secreting clones occurred in parallel, sometimes with the same fine specificity. The 12-mer core epitope 322-333 is a degenerate MHC binder and is presented to some T cell clones in a "promiscuous" manner. This epitope is almost identical with a B27-restricted CTL epitope of Ye-hsp60. Cross-reactivity of Ye-hsp60-specific T cell clones with self-hsp60 was not observed. In conclusion, an interesting Ye-hsp60 T cell epitope has been identified and characterized. It remains to be determined whether this epitope is also relevant in other reactive arthritis patients.
Adolescent↗
A microarray enzyme-linked immunosorbent assay for autoimmune diagnostics.
In order to quantify autoantibodies in the sera of patients with autoimmune disease, we have created a microarray-based immunoassay that allows the simultaneous analysis of 18 known autoantigens. The microarrays contain serial dilutions of the various antigens, thereby allowing accurate determination of autoantibody titer using minimal amounts of serum. The assay is very sensitive and highly specific: as little as 40 fg of a known protein standard can be detected with little or no cross-reactivity to nonspecific proteins. The signal intensities observed from serial dilutions of immobilized antigen correlate well with serial dilutions of autoimmune sera. Miniaturized and highly parallelized immunoassays like these will reduce costs by decreasing reagent consumption and improve efficiency by greatly increasing the number of assays that can be performed with a single serum sample. This system will significantly facilitate and accelerate the diagnostics of autoimmune diseases and can be adapted easily to any other kind of immunoassay.
Antibodies, Antinuclear↗
Highly potent inhibitors of human cathepsin L identified by screening combinatorial pentapeptide amide collections.
By screening a combinatorial pentapeptide amide collection in an inhibition assay, we systematically evaluated the potential of 19 proteinogenic amino acids and seven nonproteinogenic amino acids to serve as building blocks for inhibitors of human cathepsin L. Particularly efficient were aromatic, bulky, hydrophobic amino-acid residues, especially leucine, and positively charged residues, especially arginine. Building blocks for potential inhibitory peptides were combined by random selection from their activity pattern. This random approach for the design of inhibitors was introduced to compensate for the inaccuracy induced by shifted docking of combinatorial compound collections at the active center of cathepsin L. Thereby, we obtained structurally defined pentapeptide amides which inhibited human cathepsin L at nanomolar concentrations. Among the most potent novel inhibitors, one peptide, RKLLW-NH2, shares the amphiphilic character of the nonamer fragment VMNGLQNRK of the autoinhibitory, substrate-like, but reverse-binding prosegment of human cathepsin L which blocks the active center of the enzyme. Obviously, RKLLW-NH2 carries the functions that are important for enzyme-peptide interaction in a condensed form. This hypothesis was confirmed by structure-activity studies using truncated and modified pentapeptides.
Amides↗
[Nasal comfort and Cottle septoplasty. Prospective acoustic rhinometry study apropos of 102 cases].
Between june 1997 and september 1998, we have prospectively studied a cohort of 102 patients who were referred to ENT consultation. All patients suffered a septal deviation and a syndrome that we call "morphological". All were operated on following the Cottle's septoplasty technique. At pre- and postoperative consultation, patients were asked to answer a questionnaire and a functional evaluation of the nasal air flow was assessed by acoustic rhinometry. We have described a nasal comfort grading that we compare with the objective results as assessed by acoustic rhinometry. Our study did not permit us to find any correlation between nasal comfort and minimal cross sectional area (MCA). But evaluation of the MCA was disturbed by several methodological artifacts. A statistical correlation between the nasal comfort grading and nasal volume in the side of the septal deviation was found. The acoustic rhinometry evaluation elicited two essential observations: the first is the increase of nasal fossae volume of 30% (and, at least, 26% of the MCA). The other is a relative decrease of 45% of nasal mucosa congestion. This study shows that the nasal septum has an essential function in the pathogenesis of the so-called "morphological" functional syndrome. The morphological correction obtained by the Cottle's septoplasty acts upon the nasal vasomotricity in decreasing nasal mucosa congestion and avoid a surgical reduction of the inferior turbinate.
Adolescent↗
[Olfactory esthesioneuromas].
Olfactory neuroblastomas are rare malignant tumours of the nose arising from the olfactory epithelium. In a retrospective series of 18 cases and a review of the literature, the authors establish the main features of this tumour. They emphasise the difficulties of histological examination, often requiring immuno-cyt-chemistery and electron microscopy. They point out the value of a new classification, and confirm treatments being radio-surgical, with use of the combined rhino-neurosurgical approach when there is extension to the cribriform plate. They report a 5-year survival of 91.5%, with 87.5% at 10 years. The high incidence of lymph gland metastases (16.6%) is greater than that of local recurrence (11%) especially in the more advanced cases. The question of prophylactic treatment of the neck nodes is one to be considered.
Adolescent↗