PULMONARY ALVEOLAR MICROLITHIASIS. DISCREPANCIES BETWEEN RADIOLOGICAL FINDINGS AND CLINICAL PATTERN- CASE STUDY.

Pulmonary alveolar microlithiasis is a rare genetic disorder, inherited autosomally recessively, which is characterized by intra-alveolar deposition of microliths built mostly of calcium salts and phosphorus. This case study describing management of patient with pulmonary alveolar microlithiasis. A 49-year-old woman, diagnosed with pulmonary microlithiasis in 1979 was admitted to Pneumology Department due to increased dyspnea. On admission there were no clinical signs of active infection. The chest computer tomography scan confirmed the presence of advanced microlithiasis. Pulmonary function test revealed mild restriction with moderate diffusion impairment, due to severe hypoxemia present on 6-minute walking test patient was sent for specific assessment to local lung transplant team in Zabrze for consideration for lung transplantation. According to International Society for Heart & Lung Transplantation guidelines the patient was observed in 6 months intervals to reveal whether further disease progression will be observed. Clinical condition of our patient does not correlate with radiological scans, severe respiratory symptoms and cardiological complications. Computer tomography scan should not be the only indication for lung transplant.

Short-term CPAP treatment induces a mild increase in inflammatory cells in patients with sleep apnoea syndrome.

BACKGROUND: Nasal CPAP has been proven to be an efficient method of treating SAS patients without facial dysmorphism. However, it still remains a matter of debate why it is not universally well tolerated. The AIM OF THE STUDY was to evaluate the influence of initial CPAP treatment on nasal function in SAS patients. PATIENTS AND METHODS: Forty-two patients were consecutively included in a prospective clinical study and divided into the three following groups: 1) SAS subjects (26 patients qualifying for CPAP treatment), 2) First control group (C1) (9 patients with mild or moderate SAS, not willing to be treated with CPAP, AHI > 5 [n/h]), 3) Second control group (C2) (7 healthy subjects, AHI < or = 5). Nasal patency was measured by active anterior rhinomanometry (AAR) at recruitment and after a three-day CPAP treatment. After each AAR nasal lavage was obtained from both nostrils. Total inflammatory cell count (TCC) in each nasal lavage was then calculated in a Neubauer's chamber. RESULTS: Initial CPAP treatment caused a statistically significant rise of TCC in nasal lavage of SAS patients, when compared with initial values [n*10(5)/ml] (pre: 1.30, post: 1.92, p = 0.009). No significant differences (p > 0.05) were found both in initial TCC and nasal patency values among the three studied groups. CONCLUSIONS: SAS subjects present an unchanged nasal patency when compared to control subjects. Initial CPAP therapy might be responsible for evoking local nasal inflammation.

[The relationship between cigarette smoking and some types of idiopathic interstitial pneumonias]. / Zwiazek palenia papierosów z niektórymi postaciami samoistnych sródmiazszowych zapalen pluc.

Desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) and idiopathic pulmonary fibrosis (IPF) are the interstitial lung diseases of unknown etiology. Among patients with these disorders there is a prevalence of current or ex-smokers: over 90% in RB-ILD, 60-90% in DIP and 41-83% in IPF. Thus, cigarette smoking is to be a risk factor of a great significance. In clinical practice a correct differential diagnosis between RB-ILD and DIP on one hand and IPF on the other one is very important. Both bronchoalveolar lavage fluid and lung biopsy specimen taken from RB-ILD and DIP patients contain increased proportion of dusty brown alveolar macrophages. On CT scans the extent of fibrosis and inflammation areas is rather low. Contrary to IPF the prognosis of RB-ILD and DIP is generally good. Most patients improve clinically and radiologically with smoking cessation and corticosteroids. Further attempts towards new facts regarding the relationship between cigarette smoking and the pathogenesis and clinical course of different types of interstitial lung diseases are still needed.

[The influence of body mass index on the patients with sleep apnea syndrome]. / Wplyw wskaznika masy ciala na obraz kliniczny chorych na zespól bezdechu we snie.

UNLABELLED: Obesity is a characteristic feature of patients with sleep apnea syndrome (SAS). The aim of the study was to show how obesity exerts an influence on the polisomnography results and other clinical feature in untreated SAS patients. MATERIAL AND METHODS: On the basis of SAS questionnaire SAS was suspected in 770 patients. The preliminary investigation was MESAM 4, and revealed 110 patients with desaturation during sleep. In all of them polisomnografic examination confirmed diagnosis. The other performed investigations: spirometry and blood pressure during the day and polisomnografic examination. RESULTS: the analysis of the obtained results show the dependence of BMI on the polysomnographic study results, spirometry and blood pressure value. CONCLUSIONS: We observed that obesity is favorable to the development of: obstructive SAS, hypoventilation and desaturation during sleep, hypertension by SAS patients, SAS provoked disorders of sleep structure by all of the SAS patients.

[The phenotypical review of patients with sleep apnea syndrome]. / Ocena fenotypowa chorych z rozpoznanym zespolem bezdechu we snie.

UNLABELLED: Sleep apnea syndrome (SAS) is common with obesity. This may be a risk factor for the evolution of hypertension, diabetes mellitus and other metabolic disorders. The aim of the study was to estimate, how is the frequency of adiposity, diabetes mellitus and hyperlipidemia by the SAS patients. Material and methods. On the basis of questionnaire SAS was suspected in 770 patients. The preliminary investigation was MESAM 4, and revealed 110 patients with desaturation during sleep. In all of them polisomnografic examination confirmed diagnosis. Glucose and lipides values and also description of: gender, age, and body mass index (BMI) was analysed. CONCLUSIONS: The analysis of the obtained results show the dependence of apnea-hypopnea index (AHI) and BMI, glucose and lipids values. The most frequent features in SAS patients is obesity, diabetes mellitus was the second one, hyperlipidemia was seldom observed.

[Primary cardiac malignant fibrous histiocytoma in 47-year-old woman]. / Pierwotny pleomorficzny miesak fibrohistiocytarny serca u 47-letniej kobiety.

A case of pleomorphic malignant fibrous histiocytoma of the right atrium and right ventricle is presented. Primary cardiac malignant fibrous histiocytoma (MFH) is extremely rare and its etiology and clinical course are not precisely known. We treated a 47-year-old female patient with persistent fever and cough, shortness of breath and anasarca. The patient died after eight months with symptoms of cardiac shock. Autopsy revealed a big soft yellow tumor in the right heart. Microscopically, atypical fibroblast-like cells, pleomorphic and multinucleated giant tumor cells with eosinophilic cytoplasm were observed within the myxoid and fibrous stroma. Immunohistochemically, tumor cells were positive for alfa-1-antitrypsin and partially positive for CD68 and vimentin. According to histopathologic and immunohistochemical features we diagnosed pleomorphic MFH.