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. 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. 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. 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.
In general aneurysms of the pulmonary arteries are less frequent than intracranial, aortic or other vascular locations. Infectious causes include bacteria such as Staphylococcus sp and Streptococcus sp, mycobacteria, Treponema pallidium (syphilis) and rarely fungi. We report a 7 year old female with two right-sided parahilar pseudo-aneurysm of fungal origin with a prior history of ventricular septal defect. Pulmonary mycotic pseudo-aneurysms are very rare and require a high suspicion to diagnose. If a patient is still symptomatic for fever and cough for a long time, and consolidation on x-ray is not improving on antibiotics, contrast-enhanced computed tomography is indicated. It can be suspected that the "friable mass attached to ventricular septal defect patch" was a source of fungeal emboli to pulmonary arteries thus giving weight to the infective endocarditis etiology. A prior history of ventricular septal defect repair could favour fungal endocarditis.
Dyspnea is a non-specific symptom that requires fast diagnostics, accurate diagnosis and proper treatment. The most common causes of dyspnea include exacerbation of chronic obstructive pulmonary disease (COPD) and chronic heart failure (CHF). Distinction between these two medical conditions seems to be critical in diagnostics of emergencies. At the same time, basic diagnostic tools available in emergency room, such as classic radiography (X-ray) of the chest, electrocardiography (ECG) or b-type natriuretic peptide test, are sometimes ambiguous. Therefore looking for additional diagnostic tool seems to be justified and necessary. Transthoracic lung ultrasound assessment is a simple and easily accessible examination, enabling the early and explicit diagnostics of pulmonary oedema and its distinction from other, non-cardiac causes of dyspnea. This review outlines the current knowledge on the subject of transthoracic lung ultrasound (TLUS), particularly in respect of its clinical usefulness in distinction of causes of dyspnea exacerbation.
Chronic obstructive pulmonary disease (COPD) affects approximately 10% of adults older than 40 years and is an important causes of disability and death in elderly subjects. A large proportion of COPD patients suffer from cardiovascular comorbidities. Thromboembolic events contribute considerably to morbidity and mortality in these subjects. This review summarizes the current evidence regarding the association of COPD with increased thromboembolic risk. We discuss multiple mechanisms potentially linking these conditions and available pharmacological interventions reducing the risk of thrombotic arterial and venous events with special attention paid to new oral anticoagulants.
The prevalence of obstructive sleep apnea-hypopnea syndrome (OSAHS) ranges from 4 to 7% in men and from 2 to 5% in women. Its deleterious consequences such as traffic accidents, cardiovascular complications increasing morbidity and mortality, make it a major health problem. Apart from obesity (a major risk factor for OSAHS), hypothyroid patients are prone to reveal this phenotype. Although hypothyroidism seems an acknowledged risk factor for OSAHS, some authors report the lack of clinically relevant association. The argument partly depends on the increased prevalence of hypothyroidism in OSAHS patients, but the epidemiological data is limited and somehow inconsistent; even less is known about sub-clinical hypothyroidism in OSAHS patients. Even if frequency of overt and sub-clinical hypothyroidism in OSAHS patients is comparable to the general population, screening for it seems beneficial, as hormone replacement therapy may improve sleep disordered breathing. Unfortunately, this favorable outcome was found only in a few studies with limited number of patients with hypothyroidism. Yet, despite the lack of international guidelines and no large multicentre studies on the topic available, we think that TSH screening might prove beneficial in vast majority of OSAHS patients.
Sarcoidosis is an inflammatory disease of unknown etiology. Most commonly it results in the formation of non-caseating granulomas in intrathoracic lymph nodes and lung parenchyma, but the clinical course and picture may be complicated by extrapulmonary involvement and many non-respiratory signs and symptoms which are directly related to the disease. In addition, sarcoidosis patients may suffer from a plethora of symptoms of uncertain or unknown origin. Fatigue is one of these symptoms, and according to some authors it is reported by the majority of patients with active sarcoidosis, but also by a smaller proportion of patients with inactive sarcoidosis, or even with complete clinical and radiological remission. Therefore the term fatigue syndrome is frequently used to name this clinical problem. The definition of fatigue syndrome in sarcoidosis is imprecise and the syndrome is usually recognized by use of validated questionnaires. In this review the uptodate knowledge in this field was presented and different challenges connected with this syndrome were described.
Airway resistance is the ratio of driving pressure to the rate of the airflow in the airways. The most frequent methods used to measure airway resistance are whole-body plethysmography, the interrupter technique and the forced oscillation technique. All these methods allow to measure resistance during respiration at the level close to tidal volume, they do not require forced breathing manoeuvres or deep breathing during measurement. The most popular method for measuring airway resistance is whole-body plethysmography. The results of plethysmography include among others the following parameters: airway resistance (Raw), airway conductance (Gaw), specific airway resistance (sRaw) and specific airway conductance (sGaw). The interrupter technique is based on the assumption that at the moment of airway occlusion, air pressure in the mouth is equal to the alveolar pressure . In the forced oscillation technique (FOT), airway resistance is calculated basing on the changes in pressure and flow caused by air vibration. The methods for measurement of airway resistance that are described in the present paper seem to be a useful alternative to the most common lung function test - spirometry. The target group in which these methods may be widely used are particularly the patients who are unable to perform spirometry.
The risk of Mycobacterium tuberculosis complex (MTBC) infection is correlated with the concentration of infectious particles and exposure time. In closed populations, healthy people staying in very frequent, close and prolonged contact with a smear-positive person, become infected and represent another link in the chain of transmission of the disease. Therefore, in the fight against tuberculosis, an important element is quick identification of the patient and potentially infected people from his environment. In epidemiological investigation of tuberculosis (TB), family members are brought under special control as they are particularly exposed to transmission of infectious diseases. The study included 150 patients with bacteriologically confirmed tuberculosis who were members of 59 families. In the years 2003-2013 this population represented all TB cases detected in Poland in a family environment.Three PCR-based genotyping methods: spoligotyping, IS6110-Mtb1-Mtb2 PCR and MIRU-VNTR typing were used. Of 150 patients, 138 could be assigned to intra-household transmission on the basis of identical DNA fingerprints upon a combined typing approach. For 12 patients in 6 households, the M. tuberculosis isolates were clearly distinct in individual analysis - IS6110-Mtb1-Mtb2 PCR, spoligotyping or MIRU-VNTR typing or in three genotyping methods, suggesting that these patients were infected by the sources in the community. The analysis confirmed the transmission of tuberculosis among members of 53 families. In the remaining 6 families the source of infection were people outside the households. In all families with young children, strains isolated from them have identical DNA patterns as strains obtained from their adult caregivers. To confirm the transmission of TB in the study population of patients, epidemiological analysis required the addition of a genotyping methods characterised by high discriminatory power.
Asthma is the most prevalent chronic disease in adults. It affects their quality of life. Studies confirm that depression and anxiety occurs in asthma patients. The study involved 96 patients with asthma divided into two groups: patients with controlled (n = 33) and uncontrolled asthma (n = 63). The analysis of asthma control was performed on the basis of the ACT (Asthma Control Test) results. The study used SF-36 (Short Form 36) questionnaire and HADS (Hospital and Depression Scale) Scale. An analysis of the correlations between QoL (Quality of Life) and the level of depression revealed a decrease in QoL scores in MCS (Mental Component Score) domain in the group with controlled asthma (71.8 - patients without depression, 53.4 - patients with probable depression, and 51.4 - patients with depression; p = 0.032). A similar analysis of the correlations between QoL and the level of anxiety in this group of patients proved no correlations in PCS (Physical Component Score) and MCS domains. In the group of patients with uncontrolled asthma, anxiety and depression correlated negatively with the QoL in PCS and MCS domains. Anxiety and depression are found in asthma patients, with higher severity observed in patients with uncontrolled asthma. Female gender, the level of asthma control, asthma severity, smoking, as well as diagnoses of anxiety and depression are predictors of a significantly lower QoL in asthma. Anxiety and depression are found in asthma patients, with higher severity observed in patients with uncontrolled asthma. Female gender, the level of asthma control, asthma severity, smoking, as well as diagnoses of anxiety and depression are predictors of a significantly lower quality of life in asthma.
Pulmonary aspergillosis is a condition caused by the fungi Aspergillus. The form of disease depends on the immunological condition of the host organism and other concomitant illnesses that influence the pulmonary tissue. Asthmatic patients, in particular with the severe form of disease, who require the use of systemic glucocorticoids, are predisposed to develop allergic bronchopulmonary aspergillosis. Development of aspergilloma in the lung is preceded by the formation of pathological cavity in the course of another illness. The study reports a case of a severe asthma patient who developed aspergilloma in atypical localisation, without the presence of predisposing anatomical changes and illnesses.
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Primary pulmonary angiosarcoma (PPA) is a rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by aggressive course and poor prognosis. The early diagnosis is difficult due to diverse clinical and radiological manifestations. We present a case report of 70 year-old man, active cigarette-smoker, with a 2-month history of non-massive hemoptysis. The thorax CT revealed several solid pulmonary nodules surrounded by areas of ground glass opacity. As bronchoscopy failed to deliver adequate tissue samples, video assisted thoracic surgery (VATS) with pleura and lung biopsy was necessary. Histopathological findings were consistent with pulmonary angiosarcoma. Since no extrapulmonary lesions were demonstrated, the final diagnosis of primary pulmonary angiosarcoma was made. The patient died three months after the onset of symptoms. Our case report highlights that differential diagnosis in patients with hemoptysis and pulmonary nodules should include primary pulmonary sarcoma.
The authors present the review of factors influencing epidemiological situation of tuberculosis in Poland and in the world. The groups of increased risk of tuberculosis, and clinical conditions predisposing to activation of latent tuberculosis infection (LTBI) such as HIV, uremia, diabetes mellitus, transplantation of organs, treatment with glucocorticosteroids and with antibodies to TNF and to its receptors, were presented. The higher prevalence and worse prognosis of tuberculosis in elderly people was emphasised. The methods of LTBI recognition, according to recent recommendations, with special consideration to patients in immunosupression, were shown. Methods of treatment to prevent LTBI activation, according to WHO experts, were also presented. All data were discussed in relation to the actual epidemiological situation of tuberculosis in Poland.
Laryngopharyngeal reflux (LPR) is a complex of symptoms caused by the backflow of gastric contents into the larynx, pharynx, nasopharynx, sinuses and even to the middle ear space.The symptomatology of LPR includes: chronic cough, hoarseness, throat clearing, laryngitis,"globus pharyngeus", swallowing disturbances, postnasal drip, "fetor ex ore".In the article, the authors present two boys with chronic cough, in one of them the asthma was suspected and antiasthmatic treatment was administrated; in our patients according to the 24-hour pharyngeal pH-metry LPR was diagnosed. The aim of this study was to emphasise that pediatricians should be able to recognise symptoms of LPR. The appropriate diagnosis and treatment leads the symptoms to subside.
The study aimed to evaluate the association between food consumption pattern and asthma in Indian population. 125 asthma and corresponding age and sex matched healthy controls were recruited for the purpose of a study. A self- reported food-frequency questionnaire (NNR-Dietary Pattern in Asthma Questionnaire) comprising of 80 food and drink items, belonging to 15 groups, was developed based on routine dietary habits and ISAAC phase two and phase three questionnaires. There was no significant difference of gender, height, weight, BMI and socioeconomic status between asthma and control groups (p > 0.05). The consumption of fast food, salted snacks, fried snacks; nuts and dry fruits were significantly higher in asthmatics (p < 0.05). Similarly, there was a tendency to higher consumption of fats and oil, sugar and carbonated drink in asthmatics (p > 0.05). On the contrary, consumption of cereals, milk and milk products, non-vegetarian food, fruits and fruit juice tends to be higher in healthy controls, though neither of them could reach a statistically significant (p > 0.05). Consumption of fast food, salted snacks, fried snacks, fats and oils nuts, dry fruits, carbonated drinks may be associated with asthma in India. Hence, it is imperative to reduce consumption and increase awareness of influence of fast food on asthma through public health policies.
A heterogeneous emphysema is one of the most severe forms of chronic obstructive pulmonary disease (COPD). In some cases, besides the standard pharmacotherapy, a new treatment option of emphysema can be used - bronchoscopic lung volume reduction (BLVR) with the use of intrabronchial valves. To examine the health-related quality of life (HRQoL) of patients with severe emphysema after intrabronchial valve (IBV) implantation for the treatment of one lung. From 2011 to 2013 a single centre prospective observational study was performed. The study assessed the effect of the therapeutic BLVR intervention, measured by St. George Respiratory Questionnaire (SGRQ). A statistical analysis by use of Wilcoxon test for dependent variables was performed. Twenty patients were enrolled to the study (mean age 63 ± 10 years), all ex-smokers with tobacco exposure 38 ± 11.3 packyears. After 3 months of IBV treatment the average SGRQ score improved significantly in total (-12.8; p < 0.001) and in domains and the differences were for: "symptoms" (-8.5; p < 0.001), "activity" (-13.9; p < 0.001) and "influence on life"(-13.5; p < 0.002). The presented study revealed a significant improvement of the quality in the life measured by SGRQ after IBV treatment for heterogeneous emphysema. For the first time our study showed the significant improvement of all three domains of SGRQ after IBV treatment.
Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients.
Multi-drug resistant tuberculosis (MDR TB) is a significant health problem in some parts of the world. Three major cytokines involved in TB immunopathogenesis include IL-2, IL-4 and IL-10. The susceptibility to MDR TB may be genetically determined. The aim of the study was to assess the association of IL-2, IL-4, IL-10 gene polymorphisms with multi-drug resistant tuberculosis (MDR TB) in Ukrainian population. We observed 140 patients suffering from infiltrative pulmonary tuberculosis (PT) and 30 apparently healthy subjects. The patients were assigned to two groups whether they suffer or do not suffer from pulmonary MDR TB. Interleukin gene (IL) polymorphisms, particularly T330G polymorphism in the IL-2 gene, C589T polymorphism in the IL-4 gene and G1082A polymorphism in the IL-10 gene were studied through polymerase chain reaction. Circulating levels of IL-2, IL-4 and IL-10 in venous blood were estimated using ELISA. Prior to treatment, patients with PT showed significant increase of IL-2 levels and decrease of IL-4 and IL-10 levels compared to apparently healthy subjects. Circulating IL-4 and IL-10 levels were significantly decreased whilst serum IL-2 level was significantly increased in patients with MDR TB compared to non-MDR TB. Low IL-4 and IL-10 secretion and considerable IL-2 alterations were shown to be significantly associated with mutations of homozygous and heterozygous genotypes affecting C589T polymorphism in the IL-4 gene, G1082A polymorphism in the IL-10 gene and T330G polymorphism in the IL-2 gene in patients with PT. Heterozygous genotype and mutations homozygous genotypes gene in polymorphisms determining specified cytokines' production is a PT risk factor and may lead to disease progression into chronic phase. Heterozygous genotype of aforementioned cytokine genetic polymorphisms was significantly the most frequent in patients with MDR TB.
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Pulmonary hypertension (PHTN) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance, which eventually leads to right ventricular failure and death. At present there is no cure for pulmonary arterial hypertension (PAH); however over the past decade targeted pharmaceutical options have become available for the treatment of PAH. Prior to evaluation for therapeutic options a definitive diagnosis of pulmonary arterial hypertension must be made via comprehensive physical exam and definitive diagnostic testing. Screening test of choice remains echocardiography and gold standard for definitive diagnosis is right heart catheterization. Once the establishment of a diagnosis of PAH is made therapeutic options may be a possibility based on a diagnostic algorithm and disease severity of the PAH patient. There are different classes of medications available with different mechanisms of actions which net a vasodilatory effect and improve exercise tolerance, quality of life as well and survival.