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Biomedical subjects

J Kuś

Publications and source records attributed to J Kuś.

At least 37 records · Page 2Linked to original sources

Kabuki (Niikawa-Kuroki) syndrome associated with immunodeficiency.

We report a case of a 19-year-old male with the cardinal features of the Kabuki syndrome (KS) and, in addition, with severe immunodeficiency. Finding immune deficiency in a KS patient, prompted us to determine whether this association was related to a deletion within the DiGeorge chromosomal region. Fluorescence in situ hybridization (FISH) with the Oncor probe N25(D22S75) revealed no deletion of 22q11.2 in the patient.

Abnormalities, Multiple↗

[Diagnostic value of IgG antibody levels against 38 kDa mycobacterial antigen].

Tuberculosis diagnosis bases on clinical and radiological symptoms and identification of mycobacteria. Accuracy of both methods is limited. Therefore reliable serological test would have considerable advantage. The present study was aimed at evaluating IgG-mediated immune response against specific mycobacterial antigens 38 kDa in group of 200 patients and control subjects. Our material consisted of 104 tuberculosis patients, 25 with sarcoidosis, 24 with lung cancer, 13 with bacterial or fungal pulmonary infection, 8 with mycobacterial infections other than tuberculosis and 26 healthy persons. We used commercially available ELISA based kits (Pathozyme TB-complex). Specificity of 100% and sensitivity of 49% was achieved. Sensitivity increased to 59% in chronic cases and to 52% in culture positive cases. Sensitivity decreased to only 14% in group of new culture negative cases. Measurement of IgG serum level against 38 kDa can be helpful in tuberculosis diagnosis. As the test lacks falsely positive results it indicates its high positive predictive value.

Adult↗

[Ischemic heart disease as a factor for hindering diagnosis of idiopathic pulmonary fibrosis].

UNLABELLED: The aim of the study was to find factors that may influence diagnostic process of idiopathic pulmonary fibrosis (IPF). Clinical presentation, lung function data and radiological changes were assessed in 57 patients with diagnosis of IPF. There were 25 females and 32 males in the study group; mean age was 61.4 +/- 10.9 years. Mean duration of symptoms in the whole group was 18.4 +/- 16.0 months. The most common symptoms were dyspnea on exertion (96.5% of cases) and cough (84.2% of cases). 56 out of 57 patients (98.2% of cases) had crackles on ausculation of the lungs. In one third of the patients clubbing of the fingers was found. Mean FVC% of predicted was 77.2 +/- 19.8%, and mean DLCO% of predicted was 61.6 +/- 16.8%. The most common associated disease was ischemic heart disease, found in 43.9% of patients. Duration of symptoms in patients with ischemic heart disease was 25.7 +/- 18.8 months and was as twice long as in the rest of the patients, 12.8 +/- 10.5 months (p < 0.005). CONCLUSIONS: 1: Associated ischemic heart disease may cause delay in establishing the diagnosis of IPF; 2. Crackles on ausculation are characteristic for IPF and together with disseminated changes on chest radiograms indicate correct diagnosis in patients with associated ischemic heart disease.

Aged↗

[Evaluation of diagnostic and therapeutic management in patients with bacteriologically negative pulmonary tuberculosis].

The study aimed at assessing the frequency and type of errors in the diagnosis and management of newly registered bacteriologically negative cases of pulmonary tuberculosis. Random sample of 560 out of 7272 such patients registered in 1993 was subject of detailed analysis of all available medical documentation. The analysis was performed by an independent team of three specialists: pulmonologist, radiologist and epidemiologist. The results of the analysis indicate insufficient utilization of modern available diagnostic methods. Apart from clinical assessment the main basis for diagnosis of tuberculosis was A-P radiography. Tomography examination was performed in 35.5% of patient only, bronchoscopic in 19.8% and cytologic examination of sputum in 15.9%. Bacteriological examinations during the first phase of diagnosis, usually in hospitals, were satisfactory. Error in diagnosis was found in 63 patients, i.e. 11.3% of cases. The treatment in patients considered as correctly diagnosed, was in the intensive phase correct, but in the continuation phase--too long.

Biopsy↗

[Analysis of diagnostic errors and recommendations of diagnostic procedures in bacteriologically negative pulmonary tuberculosis].

The paper presents a detailed analysis of errors in the diagnosis of bacteriologically negative pulmonary tuberculosis in a random sample of 560 patients out of all such patients registered in 1993. The false diagnosis was found in 63 patients i.e. in 11.3% of the sample. Among the 63 patients with false diagnosis of tuberculosis there were 15 cases with lung cancer, 15 cases with pneumonia and/or pleuritis, 15 cases with disease of circulatory system with abnormal radiological of the lungs, 8 cases with old post-tuberculosis changes in the lung considered as relapses, 4 cases of bronchiolitis obliterans with organizing pneumonia, 3 cases of sarcoidosis and 1 case of aspergillosis in a post-tb cavity. The most serious errors were those related to failure in diagnosing (or of too late diagnosis) of lung cancer. These failures comprised 2.7% of patients in the sample. The chance to detect a lung cancer in the sample was 2900 cases in 100,000 men and 2200 cases in 100,000 women. This chance was much higher than in general population--29x for men and 100x for women. The main source of errors was false interpretation of radiological examinations and neglect to utilise other diagnostic procedures like bronchoscopy, cytology or tomography examinations. As an outcome of the analysis authors present their recommendations for diagnostic procedures in the diagnosis of bacteriologically negative pulmonary tuberculosis.

Adult↗

[Liver abscess in the course of ulcerative colitis].

The case of 19 year old female patient treated because of the ulcerative colitis. The abscess of the liver has appeared in this patient. This kind of complication has been never described in medical literature.

Adult↗

[Pattern of breathing and obstruction pressure in patients with airway obstruction after inhalation of bronchodilators].

The neuromuscular drive is increased in patients with an airway obstruction. The aim of the study was to estimate an influence of beta-agonist on breathing pattern and mouth occlusion pressure (P0.1) in patients with reversible and nonreversible airway obstruction. Ventilatory function tests, pattern of breathing analysis, mouth occlusion pressure (P0.1) and inspiratory impedance (P0.1/Vt/Ti) were measured in 23 obstructive patients and 20 healthy subjects. In all patients these measurements were repeated 20 minutes after bronchodilator inhalation (0.2 mg fenoterol). During quiet room-air breathing in patients we observed increased Vt, Vt/Ti comparing with healthy persons. The time of inspiration (Ti) and total time (Ttot) were shortened in comparison to our control group. P0.1 and inspiratory impedance were significantly increased (P0.1 3.6 +/- 1.6 vs 1.6 +/- 0.3 cm H2O, p < 0.01 and P0.1/Vt/Ti 6.6 +/- 2.3 vs 3.8 +/- 1.0 cm H2O/L/s). Measurements performed after bronchodilator inhalation revealed decrease of P0.1 and P0.1/Vt/ Ti in patients responsive to beta-agonist (delta FEVI > 15%). In unresponsive patients (delta FEVI < or = 15%) such decrease in neuromuscular respiratory drive was not observed. We conclude that diminishing of increased neuromuscular respiratory drive in patients with reversible obstruction is a consequence of airway resistance decreasing. It seems to be an additional, advantageous for a patient, effect of bronchodilator inhalation.

Administration, Inhalation↗

[Pulmonary reaction after furazidin (Furagin). Case report].

For the first time in Poland we present the case of pulmonary reaction to furazidin which is by chemical structure closely related to nitrofurantoin. 63 years old woman presented generalized symptoms of acute hypersensitivity reaction induced by furazidin as well as features of chronic pulmonary fibrosis. After few months of treatment with this drug patients complained of weight loss, dyspnea on effort, non-productive cough, chills and fever. Radiological and functional evaluation of respiratory system confirmed features of lung fibrosis. Drug provocation test was positive. In vitro furazidin in low concentrations stimulated proliferation of patient's lymphocytes. After cessation of treatment we have observed rapid improvement of clinical, radiological, biochemical and functional parameters.

Anti-Infective Agents, Urinary↗

[Reasons for chronic sputum culture positive tuberculosis--evaluation based on personal experience].

It is commonly believed that chronic sputum positivity in treated pulmonary tuberculosis is caused by the patients' noncompliance. We have undertaken retrospective analysis of the records of 59 patients to verify reasons of treatment failure. The group consisted of 12 women and 47 men aged between 22 to 87 (median: 47) who were sputum culture positive from 1 to 12 years (x = 3.4). According to the retrospective analysis 44 patients showed more than one reason for treatment failure. In 36 cases improper therapy was responsible for treatment failure involving insufficient number of drugs in 28 cases, untimely termination of treatment in 14 cases, inadequate drugs in 11 cases, poor control of treatment in 5 cases and insufficient doses of drugs in 1 case. The patients' noncompliance was the main reason in 33 cases, allergy and adverse reaction to drugs were the reasons of failure of the therapy in 21 cases. 18 patients (30.5%) showed primary drug resistance. The patients' records analysis revealed that the drug resistance rate was related to the duration of treatment. At the beginning every patient was resistant to 2 drugs on average while at the end of the treatment the number increased to 4. In spite of this only 31 patients (52.5%) received other than first line drugs.

Adult↗

[Treatment of patients with chronic pulmonary tuberculosis with expectoration--evaluation of effectiveness].

48 patients with chronic pulmonary tuberculosis (10 women and 38 men) aged between 22 and 87 years were treated. They were sputum culture positive from 1 to 12 years (x = 3.6). In chronics all patients but one were resistant to one or more drugs (average to 4 drugs). Control group consisted of 29 patients with recently diagnosed pulmonary tuberculosis sensitive to all drugs. Treatment of chronics based on: selection of drugs according to susceptibility test, using of 4-6 antituberculous drugs, treatment for not less than one year after sputum culture conversion. Control group was treated with standard therapy consisted of 3 or 4 drugs. Out of 48 chronics 13 patients dropped out due to: drug toxicity in 5 cases, noncompliance in 5 cases and death in 3 cases (in 2nd and 5th months of treatment). Of remaining 35 chronics sputum conversion was achieved in 30 cases (85.7%) average after 9 weeks. In control group 3 patients died during 2 weeks of treatment and remaining patients became sputum culture negative after average 6 weeks. Difference is significant (p = 0.006).

Adult↗

[Treatment of patients for chronic pulmonary tuberculosis with expectoration--selection and tolerance to drugs].

In retrospective analysis of 48 patients with chronic pulmonary tuberculosis we concluded that in spite of increasing drug resistance to first line drugs only 52% chronics received other than first line antituberculous drugs. Our treatment was based on the selection of drugs according to susceptibility tests. 4-6 drugs were used at the beginning of treatment. 13 patient were excluded of the group. Of remaining 35 chronics sputum conversion was achieved in 30 cases (85.7%). Our successful treatment of patients with chronic tuberculosis was based on the following principles: treatment with other than first line antituberculous drugs and treatment with the combination of 5 or 6 drugs selected on susceptibility tests in the initial phase continuing with 2-3 drugs for 12-18 months after sputum conversion. Ofloxacin improves the efficacy of regimens used for treatment of chronic tuberculosis.

Antitubercular Agents↗

[Reasons for chronic expectoration in patients with pulmonary tuberculosis registered in the Central Tuberculosis Registry].

The aim of the retrospective population-based study was to identify reasons for failure of sputum culture negativity from the 12th month on since the start of chemotherapy of pulmonary tuberculosis (PTB). Medical records of 97 patients with PTB (85 men and 12 women) remaining sputum culture positive for over 12 months, reported to the Central TB Registry (1988-1990), were reviewed. The mean age was 46.7 years. There were 39.2% blue collar workers, 18.6% peasants, 21.6% unemployed, 14.4% pensioners. Only 4.1% constituted persons with education above elementary level. 44 patients abused alcohol, 13 of them had severe alcohol related health problems. Initial susceptibility tests were done in 79 subjects, showing resistance to at least one drug in 15. In 21 subjects the new resistance occurred during observation. The mean period until conversion of sputum culture was 23.5 months. Sputum culture conversion was finally achieved in 63 (64.9%) subjects. The reasons for delayed sputum culture conversion were identified in 86 persons. The main reason for long duration of positive sputum culture in 75 (87.2%) subjects was poor compliance. In 7 (8.1%) cases evident treatment error was relevant. Serious adverse reactions to drugs were responsible in 4 (4.6%).

Adult↗

[Common variable immunodeficiency--analysis of the clinical aspects and results of substitution treatment].

The clinical picture and the effects of treatment with intravenous immunoglobulins of 14 adult patients (4 women, 10 men) with common variable immunodeficiency are presented. In most patients the disease began in the second decade of life (17.4 years on average), and the diagnosis was delayed by 7.5 years. Mean observation time was 38.5 months. Recurrent sinopulmonary infections were the most significant clinical feature and were observed in all patients, although chronic diarrhea and viral hepatitis, predominant of B type, were also frequently seen (both in 57% of patients). The diagnosis was made according to following criteria: frequent bacterial infections of the respiratory tract, serum IgG levels reduced below 300 mg/dl, (mean, 60.7 mg/dl), normal numbers of circulating B lymphocytes and exclusion of the secondary type hypogammaglobulinaemia. After 6 months of treatment with fresh plasma 20 ml/kg or intravenous immunoglobulins 200-400 mg/kg, mean serum IgG levels rose to 654 mg/dl. Treatment was clinically efficacious and markedly reduced the number of infections of the respiratory tract. The delay of the diagnosis, resulted in high rate of irreversible damage of respiratory tract observed in our group of patients.

Adolescent↗

[Cystic fibrosis in adults--clinical aspects].

Nineteen patients with cystic fibrosis were seen in the I Department of Tuberculosis and Lung Diseases during 3.5 years. There were 12 (63%) female, and 7 male, aged from 16 to 35 years (mean 23.2). Most patients were diagnosed in childhood, but 4 were diagnosed in their early adulthood. The diagnosis was confirmed by positive chloride sweat test in all cases. Molecular DNA analyses were performed in 16 cases. In 9 (56%) cases two mutations in the CFTR gene were identified. In 5 cases one mutation was identified. All patients had bronchiectases confirmed by CT. Spirometry showed lung function impairment with predominantly obstructive pattern. Mean VC was 2.57l, mean FEVI was 1.66l. In 7 (37%) cases FEVI was lower then 30% of predictive value. Hypoxemia was found in 11 (58%) cases and hypercapnia in 3 (16%) cases. Sputum cultures were positive for mucoid P. aeruginosa in 12 (63%) cases, for Staph. aureus in 16 (84%) cases. Persistent colonisation with nontuberculous mycobacteria was found in 2 (10.5%) cases. Aspergillus fumigatus was identified in sputum cultures in 2 subjects who had also positive precipitation test. Diabetes mellitus was diagnosed in 2 cases. Meconium ileus equivalent was seen in 1 case. Pneumothorax was seen in 1 case. One patient died in the endstage of the illness.

Adolescent↗

[Prevalence of diagnosing extrinsic allergic alveolitis in Poland in the years 1990-1994].

Poland is the country, where almost 40% of population inhabit rural areas. So EAA may be an important health problem. The aim of the study was to assess incidence of EAA in different districts in Poland. We asked hospitals to send us lists of patients with diagnosis of EAA established in the period of 1990-1994. In some hospitals we collected information by ourselves. This way, we have got data concerning patients with EAA. The highest prevalence of EAA 1.5-7.0/100,000 population was observed in eastern and south-western regions of Poland. Eastern Poland is rural area with small not specialised farms and possible high exposure to EAA antigens. In south-western Poland (Silesia) keeping pigeons is a very popular hobby, which may explain high incidence of EAA.

Adult↗

[Bronchiolitis obliterans organizing pneumonia].

Idiopathic BOOP is a clinicopathological entity defined histopathologically by the presence of granulation tissue in bronchioles, alveolar ducts and alveoli. 2 men and 6 women (mean age 47.2 years, range 22-66) with idiopathic BOOP have been observed, the mean period of observation being 31.1 months. In 7 cases the disease started with fever, cough and in 6 with progressive dyspnea. The interval between the onset of symptoms and the beginning of treatment ranged from 2 to 10 months. One woman was asymptomatic. The initial findings were: tachypnoea (6), crackles (7), decreased VC (6), reduced diffusing capacity (6), raised erythrocyte sedimentation rate (7). In 6 cases chest radiographs showed multiple alveolar patchy opacities of various size predominantly in the middle and lower lung zones. HRCT demonstrates peripheral distribution of changes. In one case diffuse reticulonodular infiltrates and in one case solitary tumor-like opacity with cavitation were observed. Seven subjects were given prednisone. Three subjects recovered completely, in 1 clinical and radiological improvement was observed. In 3 cases relapse occurred. The woman with the solitary tumor-like opacity was cured by surgical excision of the lesion.

Adult↗

[Dyspnea, respiratory mechanics and gas exchange in patients with idiopathic pulmonary fibrosis].

The sensation of dyspnea on exertion is usually the first clinical symptom in patients with IPF. The aim of the study was to assess breathlessness in patients with IPF at the time of diagnosis. The study group included 9 patients (5 women and 4 men), aged 61.4 +/- 10.5 (mean +/- SD) yrs, (range 38-71 yrs). FVC was 65.8 +/- 8.3% pred., FEVI 70.0 +/- 9.0% pred., TLC 65.1 +/- 10.5% pred. 6 min walking test was performed, during which pts reported their breathlessness according to Borg scale. All patients but one reported breathlessness during exercise. Patients were divided into two groups according to the level of dyspnea at the end of the walking test. Patients with dyspnea equal or less than moderate (= < level 3 Borg scale) had significantly higher lung volumes FVC (2.57 +/- 0.44 vs 1.79 +/- 0.5 l, p < 0.05), FEVI (2.23 +/- 0.29 vs 1.56 +/- 0.36 l, p < 0.02), TLC (4.26 +/- 0.26 vs 3.13 +/- 0.69 l, p < 0.02), DLCO (4.49 +/- 0.52 vs 3.43 +/- 0.23 mmol/min/kPa, p < 0.02), and smaller alveolar-arterial oxygen tension difference at rest (31.8 +/- 5.6 vs 43.8 +/- 2.8 mmHg, p < 0.01) as compared to the patients with more severe dyspnea (> level 3 Borg scale). All patients desaturated during the test, but there was no correlation between the degree of desaturation and dyspnea level at the end of the test. 4 patients from the first group (dyspnea level = < 3 Borg scale) desaturated from 6.3% to 19.4% from baseline value. So patients with IPF may experience severe desaturation during normal daily activity not being aware of it. Dyspnea on exertion in these patients seems to depend on both disturbed lung mechanics and gas exchange. Simple 6-minutes walking test proved to be useful in assessing severity of the disease in patients with IPF.

Adult↗

[Use of methotrexate for treatment of corticosteroid-dependent asthma].

We evaluated the effects of methotrexate treatment in 20 subjects (10 men, 10 women) suffering from corticosteroid-dependent asthma. All patients had been treated with 15 mg of prednisone or more per day for at least 3 years before including in the study. Methotrexate (15 mg once weekly) had been given for 9.7 months on average. Oral steroid intake was reduced by 50% in 8 patients and it was possible to discontinue oral corticosteroids in further 6 patients without deterioration of spirometric parameters at the end of the study. Side effects of methotrexate therapy were observed in 40% of patients, and required the discontinuation of treatment in 6 subjects.

Administration, Oral↗