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[Periodic fever syndromes].

Periodic fever syndromes comprise a group of disorders characterized by attacks of seemingly unprovoked inflammation. The genetic causes of five hereditary autoinflammatory syndromes have been identified in the last few years: familial Mediterranean fever, the cryopyrinopathies [Muckle-Wells, chronic infantile neurological, cutaneous, articular syndrome (CINCA) and familial autoinflammatory syndromes], TNF-receptor associated periodic syndrome, cyclic neutropenia syndrome and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. The study of periodic fever syndromes has progressed from clinical characterization to genetic analysis and to the definition of the functional defects linking genes or domains to apoptotic proteins and signal transduction pathways. This new research opens the way for more specific treatment options with a further improvement in prognosis and outcome.

Adolescent↗

Tuberculoid tenosynovitis and carpal tunnel syndrome caused by Mycobacterium szulgai.

Mycobacterium szulgai, a scotochromogenic mycobacterium, is a newly recognized pathogen of man and has been reported to cause pulmonary infections, olecranon bursitis and cervical adenitis. We isolated M. szulfai from granulomatous tissue removed at surgery from a young florist with the carpal tunnel syndrome. The organism was susceptible to ethambutol and rifampin but resistant to isoniazid. Cure was achieved by debridement and chemotherapy with ethambutol and rifampin. Neither the source in our patient nor the natural habitat of M. szulgai is known. Because it resembles M. gordonae and M. flavescens, common scotochromogenic mycobacteria in tapwater, care must be taken to avoid dismissing M. szulgai as a contaminant when it is isolated from tissue.

Adult↗

Flashlamp-excited dye laser therapy of idiopathic vulvodynia is safe and efficacious.

OBJECTIVE: The management of chronic vulvovaginal pain, not explicable on specific histologic grounds, presents a major problem in referral centers for lower genital tract diseases. STUDY DESIGN: This article reports on a two-step protocol in a sample of 175 medical nonresponders, drawn from a 2-year cohort of 725 women with vulvovaginal pain. The first maneuver was the use of a flashlamp-excited dye laser to selectively photocoagulate symptomatic subepithelial blood vessels in 168 women; the second was the microsurgical removal of chronically painful Bartholin's glands in 52 women not responsive or not suited to flashlamp-excited dye laser photothermolysis. RESULTS: Dye laser response rates were independent of whether patients manifested macroscopic foci of painful erythema ("vestibular adenitis") or just colposcopically apparent hyperemia-ectasia of the individual blood vessels ("pruritic papillomatosis") (56% vs 45% after a single surgical procedure; 76% vs 65% after serial retreatment; p not significant). Conversely, response rates were much lower among women in whom pressure on the Bartholin's glands produced sharp, lancinating pain (15% vs 66% after a single surgical procedure; 22% vs 93% after serial retreatment; p < 0.001). Forty-two (85%) of 50 patients with flashlamp-excited dye laser failure had deep pain; however, the impasse to progress was broken by gland removal. Final response rates were 92.5% (complete response 62%; partial response 30%) in the "surface-only" group and 80.3% in the "surface-plus-deep" group (chi 2 = 14.9; p < 0.001). The major complication was acute bacterial cellulitis, occurring in the first postoperative week. Modification of the treatment protocol to include topical antibiotics with an occlusive dressing reduced the cellulitis rate from 17.2% to 2.5%. In four women (1.8%) Koebner-like exophytic condylomas also developed within 1 month of flashlamp-excited dye laser surgery. CONCLUSION: The availability of a safe, efficacious, and relatively noninvasive treatment should reduce the need for resective surgery in most patients with idiopathic vulvodynia.

Adolescent↗

Yersinia pseudotuberculosis affecting the appendix.

Yersinia pseudotuberculosis is an uncommon cause of abdominal pain. It has a much lower incidence than Yersinia enterocolitica, and most reports have emanated from Europe or North America. This report is about a patient with Yersiniosis affecting the appendix alone, in contrast to the usual picture of mesenteric adenitis or septicemia associated with this organism.

Appendix↗

Factor XIIIa-containing cells and fibrosis in oral and maxillofacial lesions: an immunohistochemical study.

The distribution of subunit A of blood coagulation factor XIII (FXIIIa) was investigated by the avidin-biotin-peroxidase complex (ABC) method in various oral and maxillofacial tissues. These tissues were from normal tongue, gingiva, lip, and submandibular gland, and from Dilantin gingival hyperplasia (one case), pyogenic granuloma (three cases), peripheral fibroma (four cases), squamous cell carcinoma (seven cases), chronic sclerosing submandibular adenitis (two cases), and fibrous dysplasia of the mandibular bone (one case). The distribution of collagenous components was examined in the same tissues by means of the Sirius red F3BA method. By means of the ABC method, FXIIIa was detected in the cytoplasm of certain connective tissue cells in each of the tissues examined. These FXIIIa-containing cells were sparse in the normal tissues but evidently abundant in the fibrous connective tissue of inflammatory and neoplastic lesions. In the present study, the close relationship between the distribution of FXIIIa-containing cells and that of collagenous components is demonstrated. The role that FXIIIa-containing cells play in the process of fibrosis is discussed.

Collagen↗

Subchronic oral toxicity in guinea pigs of soot from a polychlorinated biphenyl-containing transformer fire.

We have previously described the acute po toxicity in guinea pigs of soot from a transformer fire at the State Office Building in Binghamton, New York. The soot was determined to contain polychlorinated biphenyls, biphenylenes, dibenzodioxins, and dibenzofurans. The present study evaluates soot toxicity in guinea pigs receiving 0, 0.2, 1.9, 9.3, or 46.3 ppm soot in the feed for 90 days or 231.5 ppm for 32 days. At 231.5 ppm, body weight loss, thymic atrophy, bone marrow depletion, skeletal muscle and gastrointestinal tract epithelial degeneration, and fatty infiltration of hepatocytes were observed. Mortality had reached 35% by Day 32 (when survivors were killed), with total soot consumption of approximately 400 mg/kg. At 46.3 or 9.3 ppm soot, a reduced rate of body weight gain was observed, and at 46.3 ppm, the mortality by Day 90 was 30%. Relative (to body) thymus weights were decreased in both groups, while relative spleen weights were increased at 46.3 ppm soot only. Salivary gland interlobular duct squamous metaplasia and focal lacrimal gland adenitis were detected histopathologically, while bone marrow depletion was noted only in females at the higher dose. Diminished serum alanine aminotransferase (ALT) activity in both sexes and decreased serum sodium levels in male and potassium levels in female animals were detected at both dose levels. decreased gamma-glutamyl transferase activity and red blood cell count and elevated serum creatinine and triglycerides were observed only in animals fed 46.3 ppm soot. At 1.9 ppm soot, salivary gland duct metaplasia was observed in both sexes, along with decreased relative thymus weights, ALT activity, and serum sodium levels in male animals only. No effects attributable to soot exposure were noted in animals receiving 0.2 ppm soot for 90 days. Total average soot consumption for male and female animals in the 0.2, 1.9, 9.3, and 46.3 ppm dosage groups was 1.2, 12, 55, and 275 mg/kg, respectively. Although many of the observed effects were typical of acute exposure of guinea pigs to the Binghamton soot or to polychlorinated aromatic hydrocarbons in general, salivary gland duct metaplasia has not been previously reported. Toxic effects of this subchronic exposure were observed at lower total doses than with acute exposure, although variations in absorption due to the effects of different vehicles (aqueous in the acute study versus the feed in this study) could account for some or all of this difference.

Administration, Oral↗

Infections with Mycobacterium malmoense in England and Wales.

Mycobacterium malmoense has been isolated from 11 patients in England and Wales. It was thought to be the cause of pulmonary disease in 9 of them and cervical adenitis in 2. The cultural and biochemical characters of the species are described and its position in relation to the avium-intracellulare complex and the non-chromogenicum-terrae-triviale complex determined by matching coefficients using 88 characters. Brief details are given of the 11 cases.

Aged↗

Mycobacterium malmoense in the north-east of England.

We report a 6 year experience (1979-1984) of isolating Mycobacterium malmoense in the north-east of England. Eleven subjects were involved of whom 10 had active infection--9 pulmonary, one cervical adenitis. The 11 new isolates represent 0.7% of all new mycobacterial isolates during this period and 10% of new non-tuberculous isolates. In all but 2 cases there was pre-existing pulmonary disease and/or a recognised predisposing factor to mycobacterial infection. The organisms were generally insensitive to isoniazid but sensitive to both rifampicin and ethionamide. The results of chemotherapy are described.

Adult↗

Nodal and extranodal lymphoproliferative disorders in Sjogren's syndrome: a clinical and immunopathologic study.

Sjogren's syndrome (SS) is frequently associated with both reactive and neoplastic lymphoproliferative disease. Over a 12-year period beginning in 1970, 21 of 138 patients with SS followed at two tertiary university medical centers had biopsies taken of enlarged lymph nodes (18) or extranodal lymphoid infiltrates (8). Many had immunologic studies performed on fresh tissue and all had paraffin-embedded tissue available for histochemical and immunoperoxidase studies. Eight of our patients had malignant lymphomas which were chiefly B cell neoplasms including two lymphoplasmacytic lymphomas and two follicular center cell lymphomas. The remaining 13 patients had either reactive adenitis (usually with follicular hyperplasia) or atypical lymphoid hyperplasia which failed to meet both histopathologic and immunopathologic criteria for malignancy. None of the nine patients with reactive hyperplasia has yet progressed to lymphoma, while one of four patients with atypical lymphoid hyperplasia progressed to overt lymphoma. Clinical features such as age, duration of disease, extent of lymphadenopathy, splenomegaly, or parotid swelling failed to identify those subsets of patients with lymphadenopathy at increased risk for lymphoma. Recognition of lymphoma in two patients was greatly facilitated by tissue immunologic studies demonstrating focal areas of monotypic B cell proliferation. In one patient in whom the histopathologic diagnosis was immunoblastic sarcoma of B cells, tumor cells were L26-negative and strongly UCHL1-positive suggesting T cell differentiation. In three patients with relatively homogeneous extranodal lymphoid infiltrates, B cell polyclonality on tissue immunoperoxidase studies, and the absence of cytologic atypia, precluded a diagnosis of malignant lymphoma; none of these three patients has progressed to overt lymphoma. Our results indicate that (1) patients with SS develop a variety of B cell lymphomas and other lymphoproliferative disorders, and (2) the nature of the lymphoproliferative disorder is best determined by multiparameter analysis including immunologic phenotyping.

Adult↗

The stability and immunogenicity of a dispersed-grown freeze-dried Pasteur BCG vaccine.

The level of antituberculous immunity seems to be related to the number of memory T cells induced. This may vary as a function of the multiplication and persistence of BCG in host tissues. The most important requirements for a BCG vaccine are, therefore, the immunogenicity of the strain, the high proportion of live to dead bacilli, and adequate dispersion and low levels of soluble antigens. The surface-grown Pasteur BCG vaccine contains a very high proportion of bacilli killed by ball-milling and freeze-drying. It also contains clumps and soluble antigens, all factors influencing cell-mediated immune processes and viability control. Therefore, several batches of vaccine were prepared on an industrial scale using one of the most immunogenic strains (French 1173 P2) and grown as dispersed bacilli by a modified cell type culture method. This method provided fully viable, well-dispersed vaccines which have a viability and heat stability superior to that of the classical surface-grown BCG. The immunogenicity was checked by multiplication and persistence in mouse organs and the skin reactivity and tuberculin hypersensitivity in guinea-pigs showed results comparable to those obtained with classical vaccine. Small-scale tests in children showed superior immunogenicity of the dispersed as opposed to the classical vaccine and there was no suppurative adenitis.

Animals↗

[Benign inoculation lymphoreticulosis or cat-scratch disease].

Discovered in 1950-51 by two great parisian medical teams, inoculation lymphoreticulosis, also called cat's scratch disease, is locoregional adenitis whose aetiology is imperfectly determined. Following, often bu not always, cat's scratch, it may evolve to suppuration. Pus is always aseptic. Attributed to Bedsonia's family micro-organism, no cultivation was possible up to date. Evolution is as a rule favourable, but disease is poorly accessible to tetracycline family's antibiotics. Diagnosis is possible by intradermoreaction. The antigen used is tyndallised pus of homologous infection. The role of the cat, still healthy, has not yet been explained.

Animals↗

Mycobacterial infection is an important infective complication in British Asian dialysis patients.

To define the extent and nature of mycobacterial infection in patients on an adult dialysis unit whose catchment population contains a large proportion of non-Caucasian subjects, a retrospective survey of all new patients accepted onto our maintenance dialysis programme between January 1987 and December 1989 was carried out. Twenty-six Asian, 13 Afro-Caribbean, two Oriental and 170 Caucasian patients were accepted onto the dialysis programme in the three-year recruitment period. Eight of the 26 Asian patients, but none of the others, had developed mycobacterial infection by the end of December 1990. One patient had a cerebral tuberculoma with miliary mottling on chest X-ray, one pulmonary tuberculosis, one tuberculous adenitis and 5 tuberculous peritonitis (four due to Mycobacterium tuberculosis and one Mycobacterium kansasii). All the patients had been living in the UK for an average of 15 (range 6-24) years, with no known recent exposure to tuberculosis. Five patients are now alive and well, one developed malabsorption following M. kansasii peritonitis, but two with tuberculous peritonitis died before treatment could be instituted. Mycobacterial infections were associated with a high level of mortality and morbidity. No Asian patient developed mycobacterial infection during post-transplant immunosuppressive therapy in the study period, probably because of the routine anti-tuberculous chemoprophylaxis employed in this group of patients. The diagnosis of mycobacterial infection should be suspected when an Asian dialysis patient develops a pyrexia of unknown origin. It is likely, though not proven, that anti-tuberculous chemoprophylaxis might reduce this high incidence of tuberculous infection in Asian dialysis patients.

Adult↗

Comparative trial of administration of half (0.05 mg) and quarter (0.025 mg) dose of intradermal Pasteur BCG on 291 infants from birth to 1 year in French Guyana.

The study concerns 291 newborns and infants aged 0 to 1 year placed randomly in two groups which respectively received 0.025 and 0.05 mg Pasteur BCG by intradermal injection, between 1 December 1988 and 28 February 1989. This random test aimed to determine if the administration of one quarter dose (0.025 mg) of intradermal BCG conferred immunity comparable to that of one half (0.05 mg) while diminishing the risk of complications, in particular suppurative adenopathy, in infants of ages 0-1 year. Statistical analysis of the results showed that the 0.025 mg dose of intradermal BCG entails an immunoresponse as satisfactory as that entailed by the 0.05 mg dose, while the rate of suppurative adenitis is significantly higher in the group that received the 0.05 mg dose. It is therefore well justified to recommend a dose of 0.025 mg of intradermal Pasteur BCG for infants aged 1 year and less.

BCG Vaccine↗

Pulsed field gel electrophoresis of representatives of Mycobacterium tuberculosis and Mycobacterium bovis BCG strains.

Using field inversion gel electrophoresis (FIGE), different Mycobacterium tuberculosis strains, such as phage prototypes, exhibit different DNA restriction patterns which are easy to compare. Virulent and avirulent variants of M. tuberculosis H37, as well as daughter strains of M. bovis BCG, display characteristic DNA profiles. BCG strains isolated from suppurative adenitis following vaccination of French patients showed patterns identical to the BCG Pasteur strain used for vaccination. These results demonstrate that FIGE of DNA restriction fragments generated by DraI represents a suitable technique for the analysis of mycobacteria at a genomic level. The DraI profiles allow the differentiation and precise identification of the BCG Pasteur, Glaxo, Russian and Japanese strains.

DNA, Bacterial↗

Hematometra presenting as acute appendicitis: a case report.

A case of intrauterine blood passing into the abdominal cavity and resulting in a clinical picture similar to acute appendicitis is presented. To our knowledge, hematometra presenting in this manner has not been reported in the medical literature. Some disease processes which more commonly mimic acute appendicitis include nonspecific mesenteric adenitis, gynecologic disorders, diverticulitis, and urinary tract infection. Unusual diseases presenting in this manner include splenic torsion, infarcted omentum, ileocecal tuberculosis, and duodenal hematoma.

Abdomen, Acute↗

[Cat scratch disease in immunocompetent children].

First cause of chronic benign lymphadenopathy, cat scratch disease occurs mainly in children and young adults. The nature of its bacterial agents is not definitely established, but Atlanta's CDC actually considers Afipia felis and Rochalimaea hensaela as the agents of the disease. It usually presents as chronic adenitis but there are severe forms of the disease with systemic manifestation. In such forms, new techniques such as serology and molecular biology are particularly useful for the diagnosis. The prognosis is always excellent and an antibiotic treatment is only recommended for systemic forms to reduce the duration of the disease.

Cat-Scratch Disease↗

[Tuberculous dacryocystitis].

BACKGROUND: Acute infection and inflammation of the nasolacrimal sac may complicate congenital obstruction of the nasolacrimal duct. Acute dacryocystitis is uncommon later and tuberculosis is exceptionally responsible for it. CASE REPORT: A 4 1/2 year-old boy was admitted because he suffered from acute left dacryocystitis with fever and cervical adenitis. Involvement of both lacrymal gland and lymph node persisted despite antibiotic and corticosteroid therapy. Needle biopsy of the lymph node showed presence of Mycobacterium tuberculosis and excision revealed caseating granulomas. The tuberculin skin test was positive while pulmonary tuberculosis was discovered in the patient's father. The patient was successfully given izoniazid, rifampin for 9 months and pyrazinamide for 2. Drainage of the sac area was necessary after 1 month of treatment followed by dacryocystorhinostomy. CONCLUSION: This rare case of tuberculous dacryocystitis permits to delineate the difficulties of ophthalmologic therapy.

Acute Disease↗

Clinical features of HIV seropositive and HIV seronegative patients with tuberculous lymphadenitis in Dar es Salaam.

SETTING: The medical wards of a referral hospital in Dar es Salaam, Tanzania. OBJECTIVE: To investigate the impact of HIV infection on clinical features in tuberculous lymphadenitis. DESIGN: A prospective clinical study of HIV seropositive and HIV seronegative patients with lymphadenopathy. RESULTS: Of 128 patients with peripheral lymphadenopathy, 24 had no tuberculosis (TB) and in 10 patients TB was found only in other organs. The remaining 94 patients, of whom 76% were HIV seropositive, formed our study population. TB lymphadenitis was considered proven in 89 and probable in 5 patients. Disseminated TB (both TB adenitis and TB in other organs) was diagnosed more often in HIV seropositive than in HIV seronegative patients (52% versus 26%, P < 0.03). 59% of the 71 HIV-infected patients compared to only 4% of the 23 patients without HIV infection were over 30 years of age (P < 0.02). The following clinical features were significantly associated with HIV infection: dyspnoea, respiratory rate > 20/min, low motility score (bedridden), neurological abnormalities, hepatomegaly, splenomegaly, lymph node size < 2.5 cm, negative PPD skin test, lymphopenia (< 1000/cm3) and presence of pleural fluid. CONCLUSION: Co-infection with HIV influences several clinical and laboratory features in patients with tuberculous lymphadenitis.

Adult↗