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Lungenmanifestation
- 1. Lungenmanifestation Lungenveränderungen im Krankheitsverlauf bei Mukoviszidose-Patienten
- 2. CF- Organbefall Leberzirrhose Fehlfunktion der Bauchspeicheldrüse Verdauungsstörungen Mangelernährung Gallensteine Männer meist unfruchtbar Diagnose: Schweißtest häufig wiederkehrende Lungeninfekte
- 4. Anatomie und Physiologie der Lunge
- 5. Die Lunge - Lage im Brustkorb
- 6. Anatomie des Respirationstraktes Netter Atlas Images
- 9. Die Atemwege Kleine ( periphere ) Atemwege Kleine Bronchien und Bronchioli (< 2 mm Durchmesser ) Große Atemwege Proximale Bronchien and kleine Bronchien (> 2 mm Durchmesser)
- 10. Die Atemwege – ein verzweigtes Röhrensystem
- 12. Alveoli Funktionseinheit der Lunge Netter Atlas Images
- 13. Pulmonar-Arterien & -Venen Netter Atlas Images
- 14. Intrapulmonaler Blutfluss Netter Atlas Images
- 15. Struktur der Bronchiolen und Alveolen Alveolar sac Netter Atlas Images
- 16. Bronchien Proximale intrapulmonale Atemwege Bronchiolen Distale intrapulmonale Verzweigung Respiratorische Bronchiolen Letzte Verzweigung im Brochialbaum Netter Atlas Images
- 17. Gasaustausch in der Lunge Gasaustausch zwischen Alveolen und Kapillargefäßen Sauerstoff Kohlendioxid Alveolenwand Gefäßwand
- 19. Spirometrie
- 21. Atemmanöver
- 24. Lungenfunktionstest: SPIROMETRIE Die Spirometrie misst das ein- und ausgeatmete Luft-Volumen als Funktion der Zeit während mehrerer Atemmanöver. . FEV1 FVC FEV1/FVC ratio Beurteilung: Die Spirometrie misst die Luftmenge, die eine Person atmet, um Krankheiten zu diagnostizieren oder deren Fortschreiten zu dokumentieren.
- 28. Veränderungen der Bronchien bei CF
- 34. Lungenfunktionsverlust bei CF-Patienten - FEV1
- 43. Welche Behandlungs-Möglichkeiten gibt es?
- 48. Relevante Keime bei der CF Cephalosporin, Aminopenicillin + ß-Lactamase-Inhibitoren, Makrolide H. influenzae Mittel der Wahl Häufigster Erreger Burkholderia cepacia Meropenem+Fosfomycin Staphylococcus aureus Ciprofloxacin + Colistin oder Tobramycin (inhalativ) Pseudomonas aeruginosa Mukoviszidose
- 53. Prävalenz Pseudomonas aeruginosa Besiedlung (2004) Source: Zentrum für Qualität und Management im Gesundheitswesen, 2005 Nachweis von Pseudomonas Mukoviszidose
- 54. Die Spätfolgen
Notas del editor
- Phenotypic expression of CF disease is extremely heterogenous. There is considerable age-related variability, and the severity of disease in specific organs varies considerably within and between patients with CF. In some affected organs, phenotypic variability is tightly linked to genotype. In others, modifier genes and extrinsic factors (environmental, therapeutic and iatrogenic) clearly influence disease heterogeneity.
- Phenotypic expression of CF disease is extremely heterogenous. There is considerable age-related variability, and the severity of disease in specific organs varies considerably within and between patients with CF. In some affected organs, phenotypic variability is tightly linked to genotype. In others, modifier genes and extrinsic factors (environmental, therapeutic and iatrogenic) clearly influence disease heterogeneity.
- Pulmonary ventilation is by definition the movement of air in to and out to the lungs. This air flows defines various lung volumes and lung capacities, which are defined in the next slides Ventilation volumes include “dead space ventilation” (air NOT involved in gas exchange) and “alveolar ventilation” (air involved in gas exchange= respiration)
- Anterior view of ribcage and lungs The respiratory system is responsible for gaseous exchange between the circulatory system and the outside world. In particular, the upper airways are the nasal cavity, the pharynx and the larynx, whereas the lowers are the trachea, the primary bronchi and the bronchial ramification. With regard to the lungs, their apexes are just above the clavicle, while the lower borders follow costal cartilage 6 on the anterior side, cross rib 6 at the mid-clavicular line and are found at vertebral body 10 on the posterior side. The lungs are divided first into right and left, the left being smaller to accommodate the heart. In humans, the left lung has two major lobes , an upper and a lower lobe, separated by an oblique fissure, and the lingula (a small remnant next to the apex of the heart) ; the right lung has an upper, middle and a lower lobe, separated by an oblique and a transverse fissure. The lungs are covered by a thin membrane called pleura, which is a two-layered structure: the parietal pleura lines the walls of the chest cage and covers the upper surface of the diaphragm, and the pulmonary pleura, or visceral layer, tightly covers the surface of the lungs. There is normally a slight amount of watery fluid within the pleural cavity that lubricates the pleural surfaces and allows the lungs to slide freely over the inner surface of the thoracic wall during breathing.
- Looking at a normal frontal chest radiograph, lungs can be easily recognize, because of their dark appearance, due to the presence of air inside them. Evident are also heart, trachea and bronchial bifurcation.
- Trachea: The trachea is protected ventrally by ‘C’-shaped cartilage (in blue) that can be palpated under the skin in the neck. Dorsally, there is a band of smooth muscle (in brown) that links the two horns of the cartilage. Longitudinal mucosal ridges are present on the posterior wall of the trachea, and correspond to thick longitudinal bundles of elastin in the subepithelial lamina propria. This elastin contributes to elastic recoil on expiration, and the elastin fibers could link up with those in the airways, including alveolar ducts and walls. Bronchi: The right main bronchus is larger than the left, carrying 55% of each breath. The two main bronchi divide into five lobar bronchi, and these then progressively divide to form the 19 bronchopulmonary segments. Bronchi have interlocking spirals of smooth muscle bands, prominent submucosal mucus glands of mixed seromucinous type (yellow) and patches of cartilage (blue). Approximately eight generations of bronchi may be present, and the central conducting airways of internal diameter > 2 mm are the major site of resistance to airflow in the normal lung. Membranous (conducting) bronchiole: The transition from bronchus to bronchiole takes place in airways of about 1 mm diameter. There are around seven generations of membranous bronchioles (MB). The last order of MB is confusingly named the terminal bronchiole (TB), despite them leading into further generations of respiratory bronchioles. The membranous bronchiole has a continuous layer of smooth muscle (brown), but lacks submucosal glands and cartilage in the wall. Respiratory bronchiole and alveoli: Respiratory bronchioles (RB) have alveoli directly attaching to their wall, so are capable of gas transfer, and have thin bands of smooth muscle (brown) spiralling around their wall. There are generally about three generations of respiratory bronchioles, and four generations of alveolar ducts (AD), eventually terminating in the alveolar sacs (AS). References 1. T. Hansel and P. J. Barnes. An atlas of chronic obstructive pulmonary disease COPD. 2004
- The trachea is the principal tube that carries air to and from the lungs. It is about 11.4 cm long and about 2 cm in diameter in the adult. It extends from the larynx to the bronchial tubes and is situated in front of the esophagus. It is nearly but not quite cylindrical, being flattened posteriorly. The trachea consists of a supporting layer of connective and muscular tissue in which are embedded from 16 to 20 U-shaped rings of hard cartilage that encircle the front of the tube.
- The alveoli are the final branches of the respiratory tree and act as the primary gas exchange units of the lung. The inner walls of the alveoli are covered with a lipid material known as surfactant. This surfactant helps to stabilize the alveoli, preventing their collapse. Absence of surfactant would lead to alveolar walls stick together and not allow for complete expansion.
- Respiration, and specifically the so called “ external respiration ”, is the process that allows taking oxygen from the air and returning carbon dioxide to it: the lungs are designed to accomplish this major physiologic role. Incoming air is distributed through all the branches of the bronchiole tree, which are engineered such that the gas flows progressively in the terminal respiratory units (alveoli). The specific gaseous exchange effectively takes place in the alveoli, where the different concentration gradients for oxygen and carbon dioxide, between alveolar air and pulmonary capillary blood, drive the exchange flow. The effectiveness of these gas movements needs a tight structural and functional partnership between alveoli and capillary vessels which is highlights by the rich vasculature of the lungs, shown in the picture.
- The gas-blood barrier between the alveolar space and the pulmonary capillaries is extremely thin, allowing for rapid gas exchange. To reach the blood, oxygen must diffuse through the alveolar epithelium, a thin interstitial space, and the capillary endothelium; CO 2 follows the reverse course to reach the alveoli.
- This illustration shows the clustered configuration of the terminal bronchioles, as they connect to the terminal alveolar sacs. The semi-spherical sacs connect between each other via pores of Kohn. The alveolar airspace allows intimate contact between the unoxygenated blood, delivered by the pulmonary artery, passing through the enriched capillary network to exchange oxygen, exchanging carbon dioxide into the airspace and the hemoglobin, then absorbing oxygen across the membrane. This results in nearly 100% oxygen-saturated blood in the pulmonary vein. The terminal bronchioles are noted to be wrapped by some elastic fibres, as well as smooth muscle bands, which are capable of reacting to sympathetic neural control. In the section of the alveolar sacs are shown the openings of the alveolar ducts into the semi-spherical protrusions, which allow intimate contact of unoxygenated blood, delivered by the pulmonary arteries, with capillary surface areas, to allow gas exchange. The air ventilating the sacs contains up to 20% oxygen. After gaseous exchange with pulmonary arterial blood the saturated pulmonary venous blood can be 100% saturated with an oxygen tension of 100 mm of Hg. Carbon dioxide traverses the membrane in reverse (bloodstream to air sac) and results in a 5% partial pressure of carbon dioxide in the expired gas.
- The trachea ramifies into the proximal intrapulmonary airways ( bronchi ), which are characterised by the presence of cartilage in their walls, mucous glands beneath the basement membrane and columnar epithelium. The distal subdivisions are the membranous bronchioles (MB) which differ from the bronchi in having no cartilage or mucous glands and being lined by cubical rather than columnar epithelium. The last order of MB is confusingly named the terminal bronchiole (TB), which have a lumen surrounded by a continuous layer of smooth muscle and an internal diameter in the range 0.30-1 mm. The total number of terminal bronchioles in the two lungs is of the order of 25x10 3 . Respiratory bronchioles (RB) have alveoli directly attaching to their wall, so are capable of gas transfer, and have thin bands of smooth muscle spiralling around their wall.
- In summary, when a breath is taken, air passes in through the nostrils, through the nasal passages, into the pharynx, through the larynx, down the trachea, into one of the main bronchi, then into smaller bronchial tubules, through even smaller bronchioles, and into alveolus. It is here that the exchange of oxygen and carbon dioxide between the air and the blood in the lungs occurs. O2 diffuses from the alveoli into the capillaries, and from there into the red blood cells. The opposite process occurs with Carbon dioxide which diffuses from the red blood cells through the capillary walls, into the alveoli and leaves the alveoli, exhaled through the nose and mouth.
- Self explanatory slide
- The most common pulmonary function testing is spirometry. Spirometry measures the volume of air inspired or expired as a function of time A spirometry tracing is obtained by having a person inhale to total lung capacity and exhaling as hard and completely as possible “ Forced expiration parameters” commonly assessed by spirometry are: FEV 1 (forced expiratory volume in one second) FVC (forced vital capacity) FEV 1 /FVC ratio, expressed in % These parameters will be discussed in the next slides
- .
- Epithelzellen sind mit Flüssigkeitsfilm (ASL = airway surface liquid) unterschiedlicher Viskosität benetzt PLL (= periciliar liquid layer) periziliäre Sol-Phase mit Wasseranteil von 90% hochvisköse Gel-Phase (Mucus) mit Muzinen, Proteinen, Glycanen, Lipiden - klebrig zum Entfernen von Fremdstoffen in der Sol-Phase schlagen die Flimmerepithelzellen annährend synchron, Mucus schwimmt wie Korken im Wasser auf der Sol-Phase und transportiert angeklebte Fremdstoffe heraus
- MCT = mukoziliärer Transport
- Als Fibrose wird eine Kollagen faservermehrung in menschlichen Geweben und Organen bezeichnet. Kollagenfasern sind eine spezielle Form des Bindegewebes.
- Als Fibrose wird eine Kollagen faservermehrung in menschlichen Geweben und Organen bezeichnet. Kollagenfasern sind eine spezielle Form des Bindegewebes.
- intermittierend – immer wiederkehrende in mehr oder weniger größeren Abständen chronische Infektion = mehr als 6 Monate Erregernachweis Nachweis von spezifischen Antikörpern
- Consumer Behaviour Common traits Who are they? Why would they use your product? What features of your product appeals to them? When is your product used by them? Where is your product used by them? How will you inform them about your product? Group your customers iin segments, the following part of the plan will stick on these segments!