Cystic Fibrosis (CF)

Case report:

A 18 year old patient complains of a significant increase in its purulent expectoration with increased cough, fever to 39 � C, fatigue, anorexia and increased shortness of breath. The patient is known in practice for many years as a cystic fibrosis patient and is always an outpatient basis and have been hospitalized for severe recurrent infections of his bronchial system. The physical examination of the very slim among obese patients results in a significant cyanosis, through the lungs, a mixture of wet and dry increased bronchial sounds, increased respiratory rate by 28 / min and at the capillary blood gas analysis hypoxia with PaO2 of 61 torr.

Pathogenesis:

In 1989, the gene was identified on the long arm of chromosome 7, which is coding for the CFTR protein (CFTR is a localized apical cell membrane in the chloride channel). The defect induced by this, greatly increased viscosity of Bronchialsekretes causes a decreased clearance mucociliary the results of which increasingly manifest infections with bacteria and fungi. These infections cause by the release of bacterial toxins and enzymes, oxygen radicals by granulocytes, through a direct or indirect Cilia damage and increasing the viscosity of Bronchialsekretes for grabs for recurrent bacterial bronchial exacerbations. Particularly problematic for the prognosis of cystic fibrosis patients is infection with Pseudomonas aeruginosa, since deteriorated from that point on, the prognosis for patients is clear.

Diagnosis:

The determination of complete blood count in the patient showed an increase in the total white to 15,000 / uL, 85% granulocytes and rod nuclei in the differential blood count 7 granulocytes, continue the C-reactive protein was significantly increased, BSG increased in the first hour at 45 mm and the eosinophilic cationic protein also moderately increased. Although P. aeruginosa and S. aureus are the most common pathogen in cystic fibrosis, many other species so that a bacteriological analysis of sputum as possible occur (Stenotrophomonas maltophilia, Haemophilus influenzae and parainfluenzae, Burkholderia cepacia, Candida albicans, Aspergillus fumigatus), with Quantitative determination is necessary.

Therapy:

The basis of the treatment of cystic fibrosis is the physical therapy, continue the inhalation treatment, the promotion of body weight by a high-caloric-enriched fat-soluble vitamins and Pankreasfermenten food and an anti-inflammatory nonspecific therapy with ibuprofen (Aktren et al). With significantly increased sputum production with increased viscosity is today inhaled with DNase (Pulmozyme), whereby the viscosity of bronchial secretion is reduced. Antibiotic can be treated prophylactically, continuously or intermittently also. In the present exacerbation of the 18 year-old patient (Cipro) treatment is started at a dose of 750 mg twice daily for detection of Pseudomonas aeruginosa in spite of the low body weight of 50 kg with normal renal function with a high-dose ciprofloxacin. Additionally, it should with tobramycin (GERNEBCIN) -Inhalationen be started daily at a dose of 80 mg three or four times. If under this treatment on an outpatient basis no adequate therapy success is achieved, a stationary combination treatment according to the sensitivity results with a Pseudomonas Betalaktamantibiotikum effective in combination with an aminoglycoside antibiotic must take place.