No abstract

Pulmonary rehabilitation can reduce the functional depletion caused by the thoracic surgical operation. Identification of more predictive factors of severe complications can help making preoperative risk stratification more precisely.

Background: Functional condition is crucial for operability of patients with lung cancer and/or chronic respiratory diseases. The aim of the study was to measure changes of functional and quality of life parameters in terms of the effectiveness of perioperative pulmonary rehabilitation (PR). Conclusions: Improvements in exercise capacity and quality of life were seen following PR both before and after thoracic surgery.

A 25-year-old woman with Hallopeau-Siemens recessive dystrophic epidermolysis bullosa had generalized blistering, scarring and milia since birth. In the course of the disease, acral pseudosyndactyly developed, and the patient suffered from corneal erosions, oesophageal strictures, malabsorption, recurrent severe pneumonias and nephrotic syndrome. In addition, she had severe anaemia, sideropaenia, hypocalcaemia, heavy proteinuria and hypoalbuminaemia. A rapidly growing skin squamous cell carcinoma developed on the neck that spread to axillary and cervical lymph nodes. Recurrent hypocalcaemic tetanic convulsions and dyspnoea and a pneumonia refractory to antibiotics led to the premature demise of the patient. Autopsy revealed extensive amyloidosis of the renal, hepatic and splenic tissues. AA type amyloid deposits were detected in the renal glomeruli and in the lung, explaining the patient's unusually severe pulmonary infections. In essence, the patient had severe recessive dystrophic epidermolysis bullosa, complicated by squamous cell carcinoma, recurrent pneumonias and nephrotic syndrome due to secondary amyloidosis of the kidney and lung. The possibility of secondary pulmonary amyloidosis should be considered in severe dystrophic epidermolysis bullosa patients with recurrent pulmonary infections.

Risk stratification is supported by laboratory parameters, lung function, oxygen uptake and comorbidities. Pulmonary rehabilitation can improve functionality and quality of life. Orv Hetil. 2017; 158(50): 1989-1997.