[Primary ciliary dyskinesia--importance of early diagnosis]. / Primárna dyskinéza riasiniek--význam vcasnej diagnózy.

BACKGROUND: Primary ciliary dyskinesia (PCD) is an inherited disease characterized by specific ultrastructural defects of cilia and sperms. The impairment of mucociliary clearance (MCC) results in chronic respiratory infections and subsequently in bronchiectasis. MAIN PURPOSE: The evaluate rational decisions in early diagnosis of PCD. METHODS: Samples of nasal mucosa or tissue of tonsilla pharyngea after adenotomy were studied by transmission electron microscopy (TEM) in 47 patients aged 1-15 years, suffering from recurrent or chronic respiratory infections. RESULTS: Congenital ultrastructural ciliary defects specific for PCD--the lack of dynein arms, radial spokes defects and microtubular transposition--were observed in 13 patients. TEM investigation is an expansive, time consuming method not available in routine practice. Therefore we have evaluated a diagnostic procedure which uses available examination methods focused on the diagnoses of PCD. TEM of respiratory cilia is indicated in patients with situs viscerum solitus if chronic respiratory disease develops and after more frequent causes--asthma, cystic fibrosis, congenital anomalies of respiratory system and immunodeficiency had been excluded. CONCLUSIONS: The correct and early diagnosis is important for effective therapy in order to improve MCC. This approach can prevent the development of bronchiectasis during childhood.

[Hemodynamics, lymphatic circulation and ultrastructure of the lungs during hemodilution with blood substitutes]. / Hemodynamické pomery, lymfatická cirkulácia a ultrastruktúra pl'úc pocas substitucnej hemodilúcie.

The effect of substitution hemodilution on transcapillary fluid exchange in the lung was studied in experiments on 30 dogs. After administration of Ringer's solution and of oncotic solutions (3.4% and 10% Rheodextran), the lymph flow through the right lymphatic trunk was measured and the protein content in the lymph was determined. The obtained results were compared with electron micrographs of the lung. Ringer's solution was found to escape rapidly from the vascular bed. A 3.9-fold increase in lymph flow was recorded and edematous changes were observed in the interstitial space. Administration of 10% Rheodextran induced an 8.3-fold increase of lymph flow and enhanced also the protein transport in the lymph. The permeability of pulmonary endothelial vascular walls was deranged. Isooncotic 3.4% Rheodextran provided adequate transcapillary exchange of fluids in the lungs and proved to be the suitable solution for substitution of blood losses. In the ultrastructure of the lungs no pathological changes were observed. (Fig. 8, Ref. 33).