Neuroendokrynna hiperplazja wieku niemowlęcego — opis przypadku

Neuroendocrine cell hyperplasia of infancy is a rare form of children’s interstitial lung disease recognised usually in infancy and in children younger than two years old. The typical clinical scenario, such as chest retractions, tachypnoea, hypoxaemia, crackles, characteristic changes in high-resolution computed tomography and histological examination of the lung parenchyma, is the cornerstone for diagnosis. In the article, the authors describe clinical manifestation of neuroendocrine cell hyperplasia and a present case of an infant with this rare interstitial lung disease.Neuroendokrynna hiperplazja wieku niemowlęcego jest chorobą śródmiąższową płuc rozpoznawaną przede wszystkim u niemowląt i dzieci do drugiego roku życia. Typowe objawy, takie jak: wciąganie międzyżebrzy, tachypnoe i hipoksemia, trzeszczenia, charakterystyczne zmiany w tomografii komputerowej o wysokiej rozdzielczości oraz w badaniu histopatologicznym miąższu płucnego pozwalają na postawienie rozpoznania. Autorzy w artykule opisują obraz kliniczny tej choroby oraz prezentują przypadek niemowlęcia z tą rzadką chorobą śródmiąższową

Public spirometry campaign in chronic obstructive pulmonary disease screening – hope or hype?

INTRODUCTION: Underdiagnosis of COPD seems to be a relevant clinical and social problem. We hypothesized that active public spirometry campaign may help identify subjects with airflow limitation consistent with COPD. The aim of the study was (1) to evaluate the willingness of random smokers to undergo public spirometry, (2) to assess the ability to obtain an acceptable quality spirometry during a public campaign, and (3) to assess the relationships between the presence and severity of respiratory symptoms and readiness to undergo spirometry. MATERIAL AND METHODS: Pedestrians aged > 40 years and a smoking history > 10 pack-years were recruited by medical students to fill a questionnaire and perform spirometry. Those with obstructive or borderline ventilatory insuffciency were invited and encouraged to undergo stationary spirometry in a pulmonary outpatient department. RESULTS: Nine hundred and five subjects meeting the inclusion criteria were invited to the study. Only 178 subjects agreed to complete the questionnaire and undergo spirometry. Airway obstruction and borderline spirometry result (classified as possible airway obstruction) were found in 22 and 37 subjects, respectively. Of these, only 15 patients attended follow-up visit to verify public spirometry results. Extrapolation of the limited data showed the incidence of newly diagnosed airway obstruction as 10.7%. CONCLUSION: Public spirometry campaign does not seem to be an effective way of COPD screening. Smokers are reluctant to undergo complimentary spirometry even in the presence of pronounced respiratory symptoms. Our observations may be helpful in elaborating future screening programs for COPD.

Significance of congestive heart failure as a cause of pleural effusion: Pilot data from a large multidisciplinary teaching hospital

Background: Epidemiological data on the causes of pleural effusion (PE) are scarce. Data on the local prevalence of various causes of PE may play a crucial role in the management strategy of patients with PE. The aim of the study was to investigate the causes of PE and to assess 30-day mortality rate in unselected adult patients treated in a large, multidisciplinary hospital. Methods: Retrospective analysis of medical records, including chest radiographs, of 2835 consecutive patients admitted to the hospital was performed. Radiograhic signs of PE were found in 195 of 1936 patients in whom chest radigraphs were available. These patients formed the study group. Results: The leading causes of PE were as follows: congestive heart failure (CHF; 37.4%), pneumonia (19.5%), malignancy (15.4%), liver cirrhosis (4.2%) and pulmonary embolism. The cause of PE in 6.7% patients was not established. There was a significant predominance of small volume PE as compared to a moderate or large volume PEs (153, 28 and 14 patients, respectively). Almost 80% of patients with CHF presented with small volume PE, while almost 50% of patients with malignant PE demonstrated moderate or large volume PE. Thirty-day mortality rate ranged from 0% for tuberculous pleurisy to 40% for malignant PE (MPE). Conclusions: Pleural effusion was found in 10.1% of patients treated in a large multidisciplinary hospital. CHF was the leading cause of PE. Although 30-day mortality in patients with CHF was rela­tively high, it was lower than that in parapneumonic PE and MPE

Influence of bronchoscopy on nitric oxide in exhaled air (FENO)

Nitric oxide has been extensively studied as a noninvasive marker of airway inflammation,especiallyin asthma. Assuming, bronchoscopy can produced not only systemic but also local inflammatory response we hypothesized that bronchofiberoscopy can be responsible for an increase in nitric oxide synthesis with resulting increase in fractional concentration of exhaled nitric oxide (FENO). Seventeen subjects (10 M, 7 F), at mean age of 53.8±14.1 yrs undergoing diagnostic bronchoscopy participated in the study. The indications for bronchoscopy were as follows: lung cancer (n=5; 29%), interstitial lung diseases (n=3; 18%), slowly resolving pneumonia (n=3; 18%), hemoptysis (n=3; 18%), differential diagnosis of asthma/dyspnea (n=3; 18%). During bronchoscopy bronchial washing (n=7) and bronchoalveolar lavage (BAL) (n=10) has been performed. FENO has been analyzed on-line with chemiluminescence analyzer (NIOX, Aerocrine, Sweden) according to American Thoracic Society guidelines, before and at 1, 2, 3 and 24 hours after bronchoscopy. Mean FENO before bronchoscopy was 19.7±4.5 ppb (mean ± SEM), post - bronchoscopy a decrease with a nadir at second hour (12.1±1.5 ppb,

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

Abnormalities in lung volumes and airflow in children with newly diagnosed connective tissue disease

Introduction: Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course. Material and methods: There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months. Results: FEV1, FEV1/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs 9 (11%). 24-months observation didn’t reveal progression in lung function impairment. Conclusions: Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.    

Holter ECG monitoring during research bronchofiberoscopy in patients with asthma

Bronchoscopy is a very useful tool in asthma research studies. The study was undertaken to evaluate the effect of bronchoscopy, BAL and bronchial biopsies on heart rate and arrhytmias in patients with asthma. Twenty patients (12 M, 8 F, mean age 39,6±16,3 yrs) with asthma (mean FEV1 81±19.5% pred.; mean FEV1%VC 69±12.3%) participated in the study. Holter ECG monitoring was performed twice: before (1 or 2 days) and on the day of bronchoscopy. Heart rate and cardiac arrhythmias were compared to prebronchoscopy recording at four separate time intervals: during bronchoscopy, first postbronchoscopic hour,second postbronchoscopic hour and total 24 hours. There were no significant differences between mean heart rate at the time of bronchoscopy (88.5±14.1min-1 vs. 83.7±11.9 min-1), first and second postbronchoscopic hour(80.9±15.8min-1 vs. 85.7±13.7 min-1 and 82.6±13.6 min-1 vs. 80.6±11.6 min-1) as well as total 24 hours (76.1±11.2 min-1 vs. 75.9±9.4 min-1) as compared to prebronchoscopic recordings. Max. heart rate during bronchoscopy was higher as compared to the corresponding time of prebronchoscopic recording (134.5±11.5 min-1 vs. 122.5±19.6 min-1,

The interpretation of carbon monoxide diffusing capacity test depending of hemoglobin concentration

The carbon monoxide diffusion capacity (DLCO) is among others dependent of the hemoglobin value. The result of DLCO test in patients with anemia change when we adjust DLCO for hemoglobin (Hb) concentration. The aim of the study was to estimate if the differences between result of DLCO and DLCO/VA before and after adjust the Hb value can change the interpretation of the test in the group with normal and low value of Hb. The study group consist of 25 patients with normal level of Hb (group A) and 21 ones with anemia (group B). All studied have been done spirometry, bodypletyzmografy and DLCO test. All tests were made on the SensorMedics. The DLCO test was made in the single breath diffusing capacity program Results. The values of the Hb in the group A were above 13g/dl for female and 14g/dl for man. In the group B the Hb value were less then 10g/dl. In the group A the middle Hb concentration was 14,49 &plusmn; 1,36g/dl. DLCO and DLCO/VA before and after Hb value adjusted were 91,4 &plusmn; 17,98 vs. 90,7 &plusmn; 17,58 % i 101,5 &plusmn; 19,46 vs. 100,7 &plusmn; 18,65% (p > 0,05) In the group B the middle Hb concentration was 8,77 &plusmn; 0,97g/dl. DLCO and DLCO/VA before and after Hb value adjusted were: 57,05 &plusmn; 17,55 vs.72,19 &plusmn; 25,27% i 67,57 &plusmn; 11,18 vs. 84,66 &plusmn; 14,62% (p < 0,05) Conclusions: 1.The were non statistically important change in the DLCO test results after consideration on Hb level in the studied group without anemia, so in the patients with normal level of Hb the DLCO test result doesn't change the interpretation of the test after the consideration on Hb concentration 2. In patients with anemia we shout adjust the Hb value to the DLCO test because the results with out this can completely change the interpretation of the test and clinical diagnosis

Relationship between airway basement membrane thickness and lung function tests in patients with asthma

Introduction: Airway remodeling is a characteristic feature of asthma. It is believed that airway remodeling affects lung function and bronchial hyper-responsiveness. Therefore, the relationship between remodeling and lung function is still a matter of extensive research. However, the results of many studies are inconsistent. The aim of the study was to assess the relationship between lung function parameters and basement membrane (BM) thickness in patients with asthma. Material and methods: Twenty asthma patients were chosen for the study (ten male, ten female, mean age 37 &#177; 15 yrs). Ten were newly diagnosed, steroid-naive patients and the other ten were patients known to have asthma who had not been treated with steroids for at least three months. The study group was selected based on the results of: clinical assessment, allergic skin-prick tests, lung function testing and bronchial challenge with methacholine. Nine (45%) patients had chronic mild, nine (45%) had moderate and two (10%) had intermittent asthma. Mean FEV1% pred. was 83 &#177; 18, mean FEV1%VC 69 &#177; 9, mean FVC% pred. 101 &#177; 14. All patients underwent research fiberoptic bronchoscopy with BAL and bronchial mucosal biopsies. Light-microscopic measurements of BM thickness were performed in hematoxylin-eosin stained slides of bronchial wall specimens with semi-automatic software analysis MultiScan Base 08.98. Results: Mean BM thickness was 12.8 &#177; 2.8 &#956;m (range: 8.5&#8211;20.7 &#956;m). No significant correlations between BM thickness and FEV1% pred., FEV1%VC, FVC% pred., RV% pred., TLC% pred., Raw (pre- and post-bronchodilator) and PC20 were observed. Conclusions: In our group of asthma patients, mean BM was significantly thickened. No relationship between BM thickness and lung function tests, including hyper-responsiveness, was found.Wstęp: W przebiegu astmy dochodzi do remodelingu oskrzeli, na który składa się między innymi pogrubienie błony podstawnej. Istnieją opinie, że przebudowa może wpływać na czynność układu oddechowego, w tym na nadreaktywność oskrzeli. Wyniki dotychczasowych badań nie są jednoznaczne. Badanie miało na celu wyjaśnienie, czy istnieje zależność pomiędzy grubością błony podstawnej i wybranymi wynikami badań czynnościowych, w tym testu nadreaktywności oskrzeli, u chorych na astmę. Materiał i metody: Do badania zakwalifikowano 20 chorych na astmę (M/K 10/10, średni wiek 37 &#177; 15 lat), w tym 10 chorych ze świeżo rozpoznaną astmą i 10 chorych z dawno postawionym rozpoznaniem, którzy z różnych przyczyn nie przyjmowali glikokortykosteroidów w okresie 3 miesięcy poprzedzających badanie. U wszystkich chorych przeprowadzono wywiad i badanie fizykalne, RTG klatki piersiowej, badanie spirometryczne z próbą rozkurczową oraz pletyzmografię, test nadreaktywności oskrzeli z metacholiną, punktowe testy skórne i oznaczenie całkowitego IgE w surowicy. U 9 chorych (45%) stwierdzono astmę przewlekłą lekką, u 9 (45%) umiarkowaną, a u 2 (10%) astmę epizodyczną. Średnia wartość natężonej pierwszosekundowej objętości wydechowej wyniosła 83 &#177; 18% w. n., natomiast średni wskaźnik FEV1%VC - 69 &#177; 9. U wszystkich chorych wykonywano bronchofiberoskopię z pobraniem wycinków z oskrzeli płatowych i/lub segmentowych. Pomiarów grubości błony podstawnej dokonywało dwóch badających wspólnie, na poprzecznych przekrojach skrawków barwionych hematoksyliną i eozyną oglądanych pod mikroskopem świetlnym, przy użyciu programu komputerowego MultiScan Base 08.98. Wyniki: Grubość błony podstawnej wyniosła 8,5-20,7 &#956;m (średnia 12,8 &#177; 2,8 &#956;m). Nie stwierdzono istotnych statystycznie związków pomiędzy grubością błony podstawnej a wartościami FEV1% w. n., TLC % w. n., RV % w. n., Raw, PC20. Wnioski: W badanej grupie chorych na astmę stwierdzono pogrubienie błony podstawnej. Nie stwierdzono natomiast związku pomiędzy grubością błony podstawnej a ocenianymi wykładnikami czynności układu oddechowego