A CASE REPORT OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Charg-Strauss syndrome or allergic granulomatous angiitis, is defined as a systemic vasculitis identified by the presence of allergic rhinitis and/or asthma, correlated with the presence of marked eosinophilia in the peripheral blood, eosinophilic infiltration of various organs with extensive areas of necrosis, eosinophilic, giant cell vasculitis of vessels of small and medium caliber, as well as perivascular and interstitial necrotizing granulomas. The frequency is 10 - 14 cases per million in the adult population. The average time interval from the onset of the disease to establishing the diagnosis is 49.7 (±6.1) months. Knowledge of the diagnostic criteria and features of the course of EGPA is necessary for early diagnosis and timely initiation of protocolic treatment. This article presents a clinical case of atypical EGPA without existing asthma and with atypical immunological changes.

Acute steroid myopathy: a highly overlooked entity.

BACKGROUND: Myopathy in patients being treated with corticosteroids is known primarily among chronically treated patients or in critically ill and mechanically ventilated patients receiving corticosteroids, often in high doses. AIM: To highlight the entity of acute, early onset corticosteroid-treatment-associated myopathy and its characteristics. DESIGN AND METHODS: Reporting our experience with four patients and reviewing all published reports of myopathy developing ≤14 days of initiating corticosteroid-treatment. RESULTS: Acute corticosteroid myopathy (ASM) exists, though the syndrome appears to be rare. It is characterized by unpredictability and heterogeneity, sometimes developing within 1-3 days, after a single dose, which may not be high and administered by varied routes. Proximal limb muscle weakness is the most common form, but distal limb, bulbar and respiratory muscles may be involved. Steroid cessation often leads to improvement/resolution, but irreversibility may occur. CONCLUSIONS: A high index of suspicion for the possibility of ASM is necessary to ensure drug discontinuation and recovery. This is particularly true since the entity is not widely recognized and its symptoms are often erroneously interpreted as due to the patient's underlying disease.

[CORRECTION OF BILE FLOW CHARACTERISTICS IN PATIENTS WITH NON-ALCOHOLIC FATTY LIVER DISEASE IN COMBINATION WITH HYPERURICEMIA].

Basis on study through integrated comparative assessment of clinical, biochemical survey data revealed that in patients with impaired metabolism of uric acid in a greater percentage of common biliary sludge, a violation of the rheological properties of bile, a violation of cholate-cholesterol ratio index, which indicates an increased risk of bile stones. The study found that despite the high levels of uric acid there is a violation of the spectrum of bile acids, cholic and deoxycholic growth acid reduction taurocholic acid. Thus, application of ursodeoxycholic acid, rosuvastatin and allopurinol in these study patients with NAFLD dosages in combination with hyperuricemia improves the clinical symptoms and normalization of biochemical parameters and normalizes the spectrum of biliary acids.

Large outbreak of Legionnaires' disease and Pontiac fever at a military base.

We investigated a mixed outbreak of Legionnaires' disease (LD) and Pontiac fever (PF) at a military base to identify the outbreak's environmental source as well as known legionellosis risk factors. Base workers with possible legionellosis were interviewed and, if consenting, underwent testing for legionellosis. A retrospective cohort study collected information on occupants of the buildings closest to the outbreak source. We identified 29 confirmed and probable LD and 38 PF cases. All cases were exposed to airborne pathogens from a cooling tower. Occupants of the building closest to the cooling tower were 6·9 [95% confidence interval (CI) 2·2-22·0] and 5·5 (95% CI 2·1-14·5) times more likely to develop LD and PF, respectively, than occupants of the next closest building. Thorough preventive measures and aggressive responses to outbreaks, including searching for PF cases in mixed legionellosis outbreaks, are essential for legionellosis control.

Eight years of Legionnaires' disease transmission in travellers to a condominium complex in Las Vegas, Nevada.

Travel is a risk factor for Legionnaires' disease. In 2008, two cases were reported in condominium guests where we investigated a 2001 outbreak. We reinvestigated to identify additional cases and determine whether ongoing transmission resulted from persistent colonization of potable water. Exposures were assessed by matched case-control analyses (2001) and case-series interviews (2008). We sampled potable water and other water sources. Isolates were compared using sequence-based typing. From 2001 to 2008, 35 cases were identified. Confirmed cases reported after the cluster in 2001-2002 were initially considered sporadic, but retrospective case-finding identified five additional cases. Cases were more likely than controls to stay in tower 2 of the condominium [matched odds ratio (mOR) 6·1, 95% confidence interval (CI) 1·6-22·9]; transmission was associated with showering duration (mOR 23·0, 95% CI 1·4-384). We characterized a clinical isolate as sequence type 35 (ST35) and detected ST35 in samples of tower 2's potable water in 2001, 2002, and 2008. This prolonged outbreak illustrates the importance of striving for permanent Legionella eradication from potable water.

[Distribution of main EEG rhythms in patients with initial neurosensory hearing disorders of vascular genesis].

Forty patients with neurosensory hearing disorders of vascular genesis were examined and divided into two groups. Patients with tone hearing disorders in conventional (0.125-8 kHz) and extended (9-16 kHz) frequency ranges entered group 1, those with such disorders in the extended range--group 2. Patients of group 1 had more pronounced cardiovascular dysfunction. Control group consisted of 15 persons with normal hearing. Electroencephalography (EEG) has shown that initial neurosensory hearing disorders were accompanied with redistribution of the main EEG rhythms especially in group 1. Compared to controls, these patients demonstrated significantly (p<0.05) reduced representation of alpha-rhythm while that of beta-activity rose showing involvement of cerebral cortical structures. Adequate preventive measures in patients with initial neurosensory hearing disorders of vascular genesis are able to protect such patients against development of neurosensory hypoacusis of vascular genesis.

Captopril-induced jaundice: report of 2 cases and a review of 13 additional reports in the literature.

Two elderly patients, treated with captopril for left ventricular dysfunction and diabetes, developed severe cholestatic jaundice for which no alternative explanation could be found. The jaundice resolved completely after discontinuation of the drug. A review of the literature identifies a highly similar and distinctive clinical pattern in another 13 cases reported and suggests that hepatotoxicity is a well-established yet rare adverse effect of captopril. However, the very common use of this drug, together with the severity of the liver injury it may cause, calls for a high index of suspicion and increased awareness of this phenomenon.

Nitrofurantoin-induced immune-mediated lung and liver disease.

A 60-year-old woman with multiple sclerosis and recurrent urinary tract infections (UTI) was evaluated for the recent onset of a dry cough, dyspnea on exertion, and jaundice. Investigation demonstrated interstitial lung disease with bilateral infiltrates and unilateral effusion, as well as a severe chronic active hepatitis with marked fibrosis. Other notable features were positive antinuclear antibodies and anti-smooth-muscle antibodies and the absence of any possible cause except for nitrofurantoin treatment (Macrodantin, 100 mg/day), which the patient had been taking for the previous 3 years as a prophylactic measure against UTI. The patient died of pneumococcal septicemia less than 30 days after presentation. Pulmonary or hepatic injury caused by nitrofurantoin treatment is rare; their combined occurrence is hardly ever described. Combined drug-induced pulmonary and hepatic toxicity is reviewed and should be considered early in the differential diagnosis to allow reversibility and avoid serious outcomes.

[A comparative analysis of the neurophysiological mechanisms of hearing disorders of different origins by the indices of the background and evoked bioelectrical activity of the brain]. / Sravnitelyni analiz neirofiziologicheskikh mekhanizmov narusheniia slukha razlichnogo geneza po pokazateliam fonovoi i vyzvannoi bioélektricheskoi aktivnosti golovnogo mozga.

The hearing function was studied as was the bioelectrical activity of the brain in workers exposed to noise in their occupations and in those persons coming into contact with ionizing radiation with normal and disordered hearing. The studies made showed that those persons under exposure to ionizing radiation exhibited more pronounced disturbances in the brain stem structures of the acoustic analyzer than those exposed to noise even if they were early in the course of the ear disorder. We consider it expedient to undertake studies on bioelectrical activity of the brain to gain further insights into the mechanisms of sensorineural hearing loss.

Interleukin-6 functions in autoimmune encephalomyelitis: a study in gene-targeted mice.

The encephalitogenic peptide pMOG 35-55 from the myelin oligodendrocyte glycoprotein was used to induce experimental autoimmune encephalomyelitis (EAE) in H-2b mice with the interleukin-6 (IL-6) gene intact or disrupted. The IL-6+/+ mice developed a chronic form of EAE ascending paralysis, whereas the IL-6-/- mice were resistant to the disease. Injections of recombinant IL-6 following pMOG immunization induced severe disease in the IL-6-/- mice. Histological examination of brain and spinal cord sections showed that the perivascular infiltration of inflammatory cells evident in IL-6+/+ mice was absent in the IL-6-/- animals and could be restored by exogenous IL-6 administration. Anti-MOG antibody levels were much lower in the IL-6-/- mice, but were not restored to high levels by IL-6 injections which elicited the development of pMOG 35-55-induced EAE. T lymphocytes reactive to the pMOG antigen were recovered from lymph nodes of both types of mice and Tcell lines could be established from both. Adoptive transfer of Tcell lines from IL-6+/+ mice induced EAE in the mice with the intact IL-6 gene but less in the IL-6-deficient mice, indicating that the resistant phenotype cannot be explained solely by lack of encephalitogenic Tcells. The absence of cell infiltrates in the brain and spinal cords of IL-6-/- mice upon adoptive transfer of the pathogenic Tcells from IL-6+/+ mice is consistent with a function of IL-6 in the local perivascular inflammatory process.

Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association?

A 63-year-old woman with a 1-year history of abdominal pain and intrahepatic cholestasis developed anorexia, weight loss, lassitude and diarrhoea. Studies led to a diagnosis of primary intestinal T-cell lymphoma involving especially the proximal small intestine and infiltrating the mesenteric lymph nodes, bone marrow and skin. An associated severe hypoalbuminaemia (1.3 g dL-1) was most probably the result of protein-losing enteropathy. Liver biopsy demonstrated concentric fibrosis of the bile ducts ('onion skin' lesions, with an inflammatory cell infiltrate and lymphoid aggregates) and was considered almost pathognomonic of primary sclerosing cholangitis. Sudden death due to pulmonary embolism occurred and a limited autopsy confirmed the diagnosis. Other associated diseases such as coeliac disease or inflammatory bowel disease were not found. This first report of the simultaneous occurrence of two rare diseases - primary sclerosing cholangitis and intestinal T-cell lymphoma - may indicate an intriguing association, possibly mediated by the effect of cytokines released by the infiltrating T-cells into the portal circulation.

[The action of thyroliberin on the myoelectric activity of the stomach in the skate and the cod]. / Deistvie tiroliberina na mioélektricheskuiu aktivnost' zheludka skata i treski.

The influence of exogenous thyrotropin-releasing hormone on the myoelectrical activity of stomach was investigated in skate and cod. The i.v. or intracerebral application of the peptide (5-8-10 micrograms/kg) led to an increase in the amplitude of the stomach cardiac and pyloric potentials, and to a change in their rate. The effect lasted longer following intracerebral administration of the peptide and was abolished by vagotomy. Irrespective of the technique of the administration, the peptide effect on the stomach myoelectrical activity was abolished by atropine and not affected by phentolamine or propranolol.