[Simultaneous occurrence of hereditary angioneurotic edema and Crohn disease]. / Hereditaer angioneuroticus oedema és Crohn-betegség együttes elöfordulása.

Hereditary C1 esterase inhibitor deficiency is often associated with immunpathologic disorders. The authors present a case of the rare coincidence of hereditary angioedema (HAE) and Crohn's disease. The history of the patient is analysed along with the familial occurrence of the disease. Characteristic abdominal manifestations of C1 esterase inhibitor deficiency are compared to the clinical signs of Crohn's disease. Differential diagnostic pitfalls are described along with efficatious therapeutic options.

[Efficacy of short-term danazol treatment in hereditary angioneurotic edema patients undergoing maxillofacial and dental surgical procedures]. / Rövid távú danazolkezelés hatékonyságának vizsgálata herediter angioneuroticus oedemás betegek maxillofacialis és fogsebészeti beavatkozása során.

The authors evaluated the efficacy of short-term danazol prophylaxis in 12 hereditary angioedema patients undergoing maxillofacial or dental procedures. Hereditary angioedema is an autosomal dominant disorder resulting from the deficiency of the C1 esterase inhibitor. Characteristic clinical manifestations include the formation of subcutaneous and submucosal edema. The latter can lead to fatal upper airway obstruction. Edematous attacks can be precipitated by tissue injury from operative procedures, dental surgery in particular. In patients undergoing surgical and diagnostic procedures performed in the cephalic and cervical regions, such attacks can be prevented by appropriate drug therapy. Short-term danazol prophylaxis was effective in all 12 patients with a history of edematous complications that had occurred after dental procedures; none of them developed postoperative edema. The serum levels of complement components determined pre- and postoperatively as well as at 6- and 12-hour intervals also demonstrated the efficacy of prophylactic therapy.

Bioplast fibrin implants in nasoseptal perforation.

This study includes 9 cases of nasoseptal perforation following submucous septectomy. Sheets from Bioplast fibrin, an absorbable biomaterial, were implanted to prevent thepersistence of perforations. Postoperative mucosal growth on both implant surfaces ensured closure in 6 of the 9 cases. The method has the advantage of simplicity and it is commendable in the case of smaller defects when the mucosa is not markedly atrophic.

[Equilibrium troubles with cervical syndrome and their relation with otorhinolaryngology (author's transl)]. / Gleichgewichtsstörungen bei zervikalen Syndromen und ihre Beziehungen zur HNO-Heilkunde

The equilibrium troubles occuring in connection with cervical locomotoric diseases and their otorhino-laryngologic relations have been studied in the ORL Department of National Institute of Rheumatism and Medical Hydrology. It has been found that: a) there is no connection between the seriousness of roentgenologic deformations and equilibrium troubles; b) the damage of the periphery is not characteristic; c) the complaints and symptoms are explained by the hypoxia of vestibular kern-areas as well as by damages of receptors in cervical muscles, bands and joints; d) hypotension and other central vascular damages form an important factor in the development of equilibrium troubles.

Immunoglobulin levels of tonsillar cells.

Immunoglobulin G, A and M contents of homogenized tonsillar lymphocytes were measured. Higher Ig values were found in adults than in children. Inflammatory processes increased the immunoglobulin levels of tonsillar lymphocytes. The changes in tonsillar IgG and IgA levels suggest the importance of local immunoglobulin production in the defence mechanism of tonsils. Two isolated IgA deficiencies were detected among the 85 patients.

The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures.

PURPOSE: This study evaluated the efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial or dental procedures. PATIENTS AND METHODS: Twelve patients with a history of edema after dental procedures were administered danazol (600 mg/d) 4 days preoperatively and 4 days postoperatively. The serum levels of complement components were determined preoperatively and postoperatively as well as at 6, 12 and 24 hours in six patients. RESULTS: None of the 12 patients developed angioneurotic edema. The serum levels of the complement components were decreased immediately after surgery and returned to normal within 24 hours. CONCLUSION: The short-term prophylactic use of danazol in patients with hereditary angioedema undergoing oral surgery is an effective preventive measure.

Angioedema due to acquired C1-esterase inhibitor deficiency in a patient with Helicobacter pylori infection.

The first component of the classical pathway of the complement system, C1 is regulated by a serum protein, the C1-esterase inhibitor (C1-INH). Deficiency of this protein leads to the release of vasoactive mediators (C2 kinin and bradykinin) that increase vascular permeability and can induce edema formation in subcutaneous and submucosal tissues. The genetic variant of C1-INH deficiency is inherited as an autosomal dominant trait and causes hereditary angioneurotic edema. The acquired form of C1-INH deficiency is characterized by similar manifestations and can occur in association with lymphoproliferative diseases, malignancy, immune disorders, and infections. The authors present a case of acquired C1-INH deficiency in a patient with Helicobacter pylori infection. Complete eradication of this pathogen was followed by the resolution of symptoms and normalization of serum complement levels. It seems therefore probable that in this patient, acquired C1-INH deficiency was related to Helicobacter pylori infection. To our best knowledge, no similar observations have been published so far. Specific immune reactions are important contributing factors in H. pylori. Excessive consumption of complement by antibodies directed against H. pylori is a potential cause of C1-INH deficiency observed in our case.

Clinical immunological study of T lymphocytes and EA rosette forming cells in tonsils.

Tonsillar E and EA rosette forming lymphocyte subpopulations were studied in 120 tonsillectomized patients. T cell ratios was usually lower in the tonsils than in the blood. EA binding cells were studied with indicator systems of human or rabbit antibody sensitized red cells, respectively. Poorly sensitized human RBCs (EArabbit) bind much better to tonsillar cells than to blood lymphocytes and so this system proved to be specially suitable to study tonsillar EA binding cells. Increase in E and EA rabbit rosette forming cell frequencies were found with the age of patients. Decrease in percentage of T cells and EA rabbit rosette binding cells were found with high frequency of acut tonsillitis and with clinical sysmptoms of chronic local inflammation. The variation of these lymphocyte subpopulations with the local tonsillar inflammatory processes suggest a considerable clinical immunological role of local T cells and of this portion of Fc receptor postive lymphocytes.

Coincidence of hereditary angioedema (HAE) with Crohn's disease.

A patient with two diseases, based presumably on different immunopathological mechanisms, hereditary angioedema (HAE) and Crohn's disease, was followed for 8 years. For more than three years of this observation period, detailed laboratory data were also available and could be analyzed. Both diseases had severe courses requiring chronic treatment with danazol and sulfasalazine, respectively. During exacerbation of Crohn's disease, the levels of C4 was found to be significantly lower than during the periods free of symptoms of both diseases. This drop was probably due to an impaired C1-inhibitor activity. HAE attacks and acute exacerbation of Crohn's disease never occurred simultaneously. This finding may be a mere chance but may also indicate that the different immunopathological processes underlying HAE and Crohn's disease influence each other.