Quality of life in patients with cystic fibrosis depending on the severity of the disease and method of its treatment

Introduction: Quality of life is a measure not only of disease severity and its functional impact (physical, psychological, and social functioning) but also the efficacy of novel therapies. The aim of this study was to analyze the quality of life in cystic fibrosis patients depending on the severity of the disease and methods of its treatment.Material/Methods: The study included groups of cystic fibrosis patients: 1) after lung transplantation, 2) requiring chronic oxygen therapy, and 3) in stable clinical status. Forty-five men and women older than 18 years were enrolled. The participants were examined with the Polish version of the Cystic Fibrosis Quality of Life Questionnaire (CFQoL) adapted by Dębska & Mazurek.Results: Patients from analyzed groups differed significantly in terms of their quality of life in most of the subscales included in CFQoL, but not in Future Concerns and Interpersonal Relationships.Discussion: Although lung transplantation markedly improves the quality of life of patients with cystic fibrosis, they still experience problems with social functioning and future concerns

Evaluation of anxiety and depression incidence in adolescents with cystic fibrosis or malignant diseases

Background. Malignant diseases and cystic fibrosis are responsible for longlasting states of frustration and suffering, which particularly affect adolescents. Considering potentially dangerous effects of anxiety and depressive episodes, prompt and reliable diagnosis and therapy become especially important for both quality of life and its preservation. Problems with the evaluation of incidence of anxiety and widely understood depression, and contradictory results found in literature, encouraged researchers to attempt the assessment of incidence of chosen negative emotions in adolescents. Material and methods. The study group comprised of adolescents and young adults, aged from 14 to 21, hospitalised because of malignant diseases (n = 78; group I) and cystic fibrosis (n = 53; group II). The incidence of anxiety and depression was evaluated with the Hospital Anxiety and Depression Scale (HADS). Results. Anxiety was observed in 25% of CF patients and 14% of cancer patients whereas depression was diagnosed in 10% of group I patients and 6% of group II respondents. The study groups showed no difference in the level of anxiety and depression (p ≥0.05). No statistically significant correlations between patients’ groups, divided in regard to the level of anxiety and depression, were found (Ch2 = 2.4, p = 0.3 vs. Ch2 = 1.04, p = 0.59). The multivariate analysis (ANOVA) also confirmed that anxiety and depression incidence did not depend on diagnosis (R Rao = 1.5, p = 0.23). Both groups showed strong positive relation between the level of anxiety and depression (r = 0.66, p = 0.001). Conclusions. The level of anxiety and depression was observed in a small proportion of respondents. Adolescents suffering from CF and malignant diseases showed no difference in regard to the anxiety and depression levels. However, it could be possible that patients hid disturbing symptoms (anxiety and depression). Therefore, special care must be delivered to those whose level of depression and anxiety are close to the upper norm limit.Background. Malignant diseases and cystic fibrosis are responsible for longlasting states of frustration and suffering, which particularly affect adolescents. Considering potentially dangerous effects of anxiety and depressive episodes, prompt and reliable diagnosis and therapy become especially important for both quality of life and its preservation. Problems with the evaluation of incidence of anxiety and widely understood depression, and contradictory results found in literature, encouraged researchers to attempt the assessment of incidence of chosen negative emotions in adolescents. Material and methods. The study group comprised of adolescents and young adults, aged from 14 to 21, hospitalised because of malignant diseases (n = 78; group I) and cystic fibrosis (n = 53; group II). The incidence of anxiety and depression was evaluated with the Hospital Anxiety and Depression Scale (HADS). Results. Anxiety was observed in 25% of CF patients and 14% of cancer patients whereas depression was diagnosed in 10% of group I patients and 6% of group II respondents. The study groups showed no difference in the level of anxiety and depression (p ≥0.05). No statistically significant correlations between patients’ groups, divided in regard to the level of anxiety and depression, were found (Ch2 = 2.4, p = 0.3 vs. Ch2 = 1.04, p = 0.59). The multivariate analysis (ANOVA) also confirmed that anxiety and depression incidence did not depend on diagnosis (R Rao = 1.5, p = 0.23). Both groups showed strong positive relation between the level of anxiety and depression (r = 0.66, p = 0.001). Conclusions. The level of anxiety and depression was observed in a small proportion of respondents. Adolescents suffering from CF and malignant diseases showed no difference in regard to the anxiety and depression levels. However, it could be possible that patients hid disturbing symptoms (anxiety and depression). Therefore, special care must be delivered to those whose level of depression and anxiety are close to the upper norm limit

Comparison of two tobramycin nebuliser solutions: Pharmacokinetic, efficacy and safety profiles of T100 and TNS

AbstractBackgroundTobramycin inhalation is an accepted treatment of chronic pseudomonal infection in cystic fibrosis (CF) patients. Twice daily inhalation is efficacious, but time-consuming.MethodsIn this randomized, open-label, multicentre, two-period, crossover study, 58 patients with CF and chronic Pseudomonas aeruginosa (PA) infection received two tobramycin nebuliser solutions: T100/eFlow or TNS/PARI LC PLUS. The primary objective was to demonstrate the equivalence of both treatments with respect to pharmacokinetics (area under the concentration–time curve and maximum concentration in plasma). Secondary endpoints were tobramycin sputum pharmacokinetics, reduction in PA colony forming units, improvement of lung function, incidence of adverse drug reactions and reduction of inhalation times.ResultsTobramycin plasma AUC and Cmax were lower after administration of T100 than after TNS. The study failed to demonstrate systemic bioequivalence of the two treatments. After T100 administration, tobramycin sputum AUC and Cmax achieved higher values than after TNS. Changes in efficacy parameters from baseline were similar. Safety profiles were not different or unexpected. Inhalation time per inhalation was shorter during treatment with T100.ConclusionThe lower systemic drug burden and the higher local drug deposition together with a comparable efficacy/safety profile and a shorter inhalation time render T100/eFlow an attractive treatment option for CF patients.(www.controlled-trials.com/ISRCTN85410458)

Problems of patients with cystic fibrosis during transition to adulthood

The proper care of cystic fibrosis patients extends over their lifetime. More than half of the children with the disease die before adulthood. An important element in the patient’s care is a time of transition from a paediatric to the care of an internist and the patient’s acceptance of this necessity. Transition from paediatric care to an internist should be adequately prepared. It is not only a question of transfer of medical records, but also careful preparation of patients for such transition. The patients expect not only continuity of care but also the introduction to the management with the disease. The creation of a base for specialist hospital treatment for exacerbation of the disease at the adulthood is an important element in the care of these patients. The problem has been solved in the children group, but is still waiting for solution in adults with cystic fibrosis. It has been proven that care in the centres carried out by a specialized team ensures longer life and better quality of life of these patients. The paper is an overview of these two important elements of care of adults with cystic fibrosis

Distribution of antibodies to selected antigens of Pseudomonas aeruginosa in children and young adults with cystic fibrosis

Introduction: Eradication of Pseudomonas aeruginosa (P.a.) in patients with cystic fibrosis (CF) is possible if it is initiated in the early course of infection. Therefore, the detection of P.a. as early as possible is an important goal of care. Regular determination of antibodies to P.a. antigens in serum may be useful in patients who have not yet been infected or were infected intermittently. The aim of the present study was to assess the concentrations of antibodies to selected antigens of P. aeruginosa in the serum of children with CF and with known status of P.a. infection.Material and methods: The study was performed in 111 CF patients (27 not infected with P. aeruginosa, 29 with intermittent infection and 55 with chronic infection). The concentrations of IgG antibodies to the alkaline protease (AP), elastase (ELA) and exotoxin A (Exo-A) were measured. The increased concentration of antibodies was defined as exceeding 500 units (according to the manufacturer). The results of antibodies assessment were analysed according to previous infection status and the results of present culture.Results: At the time of the study, P.a. was cultured from sputum of 57 patients: 9 out of 29 (31%) with intermittent infection, and 48 out of 55 (87%) with chronic infection. Increased concentrations of antibodies to one or more P.a. antigens were found in 60 patients, and to all three types of antigens in 30 patients. Increased serum antibody concentration was found significantly more often in the patients with chronic P.a. infection compared to those with intermittent infection (82% vs. 35%, p = 0.0001). In the patients with chronic P.a. infection (especially with mucoid type), serum antibody concentrations were significantly higher than in other patients. Higher concentrations of antibodies were also found in the patients with positive result of P.a. culture at the time of the study, compared to those with negative culture. In 19% of patients not infected with P.a., increased serum antibodies to at least one P.a. antigen were found. The clinical significance of such findings is unclear and needs further investigation.Conclusions: In the present study, the increased serum concentrations of IgG antibodies to P. aeruginosa antigens (AP, ELA and Exo-A) were found most often in the patients with chronic P.a. infection and in those in whom P.a. (especially mucoid type) was cultured at the time of the study. The clinical significance of the elevated antipseudomonal antibodies level in 19% of the patients never infected with P.a. is unclear and needs further investigation.Wstęp: Eradykacja Pseudomonas aeruginosa (P.a.) u pacjentów z mukowiscydozą jest możliwa, jeśli zostanie rozpoczęta na wczesnym etapie zakażenia. Z tego powodu wykrycie P.a. stanowi ważny element opieki nad chorymi. Oznaczanie przeciwciał dla antygenów P.a. w surowicy może być użyteczne u pacjentów, którzy dotychczas nie byli zakażeni Pseudomonas aeruginosa lub byli zakażeni w sposób przerywany. Celem badania była ocena stężenia przeciwciał dla wybranych antygenów Pseudomonas aeruginosa w surowicy chorych na mukowiscydozę (CF, cystic fibrosis), ze znaną historią zakażenia.Materiał i metody: Badania wykonano u 111 pacjentów (27 dotychczas nie zakażonych, 29 z okresowym zakażeniem i 55 przewlekle zakażonych), u których oznaczano stężenia przeciwciał IgG dla alkalicznej proteazy (AP), elastazy (ELA) i egzotoksyny A (Exo-A) metodą immunoenzymatyczną. Za podwyższone stężenie przeciwciał uznawano wartość powyżej 500 jednostek producenta. Wyniki zestawiono z aktualnym badaniem mikrobiologicznym plwociny oraz z wywiadem dotyczącym zakażenia P.a.Wyniki: Aktualny wzrost P.a. z plwociny stwierdzono u 57 chorych: 9 spośród 29 (31%) z okresowym zakażeniem i 48 spośród 55 (87%) z zakażeniem przewlekłym. U 60 chorych stwierdzono podwyższone stężenie przeciwciał dla co najmniej jednego antygenu P.a., u 30 – dla wszystkich badanych antygenów. Odsetek chorych z podwyższonym surowiczym stężeniem przeciwciał był istotnie wyższy wśród chorych przewlekle zakażonych w porównaniu z zakażonymi okresowo (82% v. 35%, p = 0,0001). U chorych przewlekle zakażonych P.a. (zwłaszcza szczepem śluzowym) stężenia przeciwciał były wyższe niż w pozostałych grupach. U chorych aktualnie zakażonych P.a. stwierdzono istotnie wyższe stężenie przeciwciał antypseudomonalnych w porównaniu z chorymi, u których aktualnie nie stwierdzano wzrostu P.a. U 19% badanych z dotychczas i aktualnie ujemnymi wynikami mikrobiologicznego badania w kierunku obecności P.a uzyskano dodatnie wyniki oznaczenia przeciwciał dla co najmniej 1 antygenu tej bakterii. Zjawisko to ma niejasne znaczenie kliniczne i wymaga dalszej obserwacji.Wnioski: W badanej grupie chorych na mukowiscydozę odpowiedź immunologiczna na zakażenie P.a. zależała w większości przypadków od czasu i intensywności zakażenia. Stężenie przeciwciał było również związane z aktualną obecnością szczepu P.a. w hodowli – najwyższe stężenie przeciwciał obserwowano wśród aktualnie zakażonych typem śluzowym P.a.. Znaczenie kliniczne przeciwciał przeciwko P.a. u 19% chorych dotychczas nie zakażonych tym patogenem jest niejasne i wymaga dalszych badań

Acute subglottic laryngitis. Etiology, epidemiology, pathogenesis and clinical picture

In about 3% of children, viral infections of the airways that develop in early childhood lead to narrowing of the laryngeal lumen in the subglottic region resulting in symptoms such as hoarseness, a barking cough, stridor, and dyspnea. These infections may eventually cause respiratory failure. The disease is often called acute subglottic laryngitis (ASL). Terms such as pseudocroup, croup syndrome, acute obstructive laryngitis and spasmodic croup are used interchangeably when referencing this disease. Although the differential diagnosis should include other rare diseases such as epiglottitis, diphtheria, fibrinous laryngitis and  bacterial tracheobronchitis, the diagnosis of ASL should always be made on the basis of clinical criteria

Czy na podstawie badania cytologicznego błony śluzowej nosa można przewidzieć, u kogo wystąpi marsz alergiczny?

Wstęp: Naturalna ewolucja choroby atopowej połączona ze zmianą lokalizacji narządowej bywa określana pojęciem "marszu alergicznego". Celem pracy była analiza przydatności cytologii błony śluzowej nosa jako badania ułatwiającego przewidywanie wystąpienia chorób atopowych oraz objawów klinicznych poprzedzających marsz alergiczny. Materiał i metody: Badaniem retrospektywnym objęto 146 dzieci (60 dziewczynek i 86 chłopców), wyłonionych z grupy 1620 dzieci, po uwzględnieniu kryteriów kwalifikacji (wiek do 4 lat w momencie zgłoszenia, objawy sugerujące chorobę alergiczną, cytologia błony śluzowej nosa na początku obserwacji, obserwacja trwająca co najmniej 4 lata). Wyniki: Średni wiek na początku obserwacji wyniósł 2 lata i 3 miesiące (SD 10 m-cy). Po 4 latach rozpoznano ostatecznie alergiczny nieżyt nosa (ANN) u 85 dzieci (58,2%), zespół atopowego wyprysku/zapalenia skóry (ZAZS) u 51 (34,9%), astmę oskrzelową (AO) u 48 (32,9%), natomiast choroby niealergiczne u 36 (22,5%). U wszystkich dzieci, u których rozpoznano AO, współistniał ANN. Statystycznie istotne okazały się różnice pomiędzy grupami rozpoznań w odsetku eozynofilów (p < 0,001), neutrofilów (p < 0,001) i limfocytów (p = 0,028). W badaniu cytologicznym błony śluzowej nosa u dzieci, u których rozwinął się ANN (izolowany lub z chorobami współistniejącymi), średni odsetek eozynofilów na początku obserwacji był znamiennie wyższy w porównaniu z dziećmi z izolowanym ZAZS (18% v. 3%; p = 0,004) lub chorobami niealergicznymi (18% v. 4%; p < 0,001). Obecność co najmniej 8% eozynofilów w cytologii błony śluzowej nosa była najbardziej pomocna w wykrywaniu dzieci, u których w trakcie obserwacji rozwinął się ANN (czułość 80%, swoistość 95%). Wnioski: U dzieci w wieku poniżej 4 lat eozynofilia w cytologii błony śluzowej nosa, wynosząca co najmniej 8%, wiązała się z wysokim ryzykiem rozwoju ANN. Marsz alergiczny dotyczył dzieci, u których na początku 4-letniej obserwacji występował ZAZS lub/i objawy alergii pokarmowej. Podwyższona eozynofilia błony śluzowej nosa u małych dzieci może być traktowana jako wskaźnik ryzyka marszu alergicznego. Pneumonol. Alergol. Pol. 2010; 78, 4: 263-270Introduction: The term allergic march has been used to describe natural evolution of the atopic disease in children, accompanied by the change in organ manifestation with time. The aim of the study was to analyze the role of the cellular components of the nasal cytology as a tool for prediction of atopic diseases and clinical symptoms preceding allergic march. Material and methods: In a retrospective manner out of a group of 1620 children, 146 symptomatic children (60 girls and 86 boys) meeting inclusion criteria (age below 4 years at first visit, symptoms suggesting allergy, nasal cytology performed at the beginning of observation, observation of at least 4 years) were included in analysis. Results: Mean age of children at time of enrollment was 27 months (SD 10 months). After 4 years allergic rhinitis (AR) was diagnosed in 85 children (58.2%), atopic eczema/dermatitis syndrome (AEDS) in 51 (34.9%) and asthma in 48 (32.9%). Nonallergic etiology was identified in 36 patients (22.5%). All patients with asthma suffered from AR. Significant differences between groups were found in number of eosinophils (p < 0.001), neutrophils (p < 0.001), and lymphocytes (p = 0.028) in cytological examination of nasal mucosa. In children with AR (alone or combined with other comorbidities) nasal eosinophilia was higher than in children with AEDS (18% v. 3%; p = 0.004) or non-allergic disease (18% v. 4%; p < 0.001). Nasal eosinophilia of at least 8% was predictive for development of AR (sensitivity 80%, specificity 95%). Conclusions: In children below 4 years nasal eosinophilia &#8805; 8% was predictive for AR development. Allergic march was observed in children with AEDS or/and gastrointestinal allergy symptoms present at the beginning of observation. Nasal eosinophilia in small children might be predictive for the risk of allergic march. Pneumonol. Alergol. Pol. 2010; 78, 4: 263-27

Time trends in diagnostic testing for primary ciliary dyskinesia in Europe

status: publishe