[Alpha-1 antitrypsin deficiency. The experience of Pulido Valente Hospital with augmentation therapy]. / Défice de alfa-1 antitripsina. A experiência do Hospital de Pulido Valente com a terapêutica de reposição.

Alpha-1 antitrypsin (AAT) is synthesised in the liver and has half-life of 4-5 days. AAT has antiprotease activity, with particular affinity for neutrophil elastase. Its deficiency leads to a lack of effective lung protection against activated neutrophil enzymes. Deficiency of AAT is a genetic disorder that occurs as a result of the inheritance of two protease inhibitor deficient alleles. Of the deficient alleles, Pi*Z is the most common, and the homozygous form Pi*ZZ results in the lowest serum levels, usually below 50 mg/ dl. The "protective threshold" is 80 mg/dl. Smoking increases the risk of emphysema. The current goal of augmentation therapy is to raise the plasma levels, above protective threshold and slow disease progression. The authors present the experience of the Day Care Hospital of the Pulido Valente Hospital with five male patients presenting emphysema due to AAT deficiency, receiving weekly intravenous treatment with Prolastin. We performed a clinical, respiratory functional and radiological evaluation between 2003 and 2007. The results point to a slower progression of the disease, with clinical and radiological stability and a reduced rate of FEV1 decline. Augmentation therapy is an expensive treatment and its use is lacking supportive evidence of efficacy by randomized controlled clinical trials. Evidence that it confers benefits is based on observational studies. Our experience is positive, showing clinical, radiological and functional benefits. The literature available points to a decrease in mortality, but we could not affirm so in our small population.

[Oncologic pain]. / Dor oncológica.

Pain can be defined by several ways, but is usually describes as an unpleasant sensorial or emotional experience related to real or potential tissue damage, or described in terms of such damage. The cancer patient may experience pain related to the cancer itself, its treatment or not related at all with the oncologic disease. It has an extreme importance to the patient, as it is interpreted as a worsening of the prognosis or near death. Therefore it is extremely important a correct approach and treatment of cancer pain. Pain can be treated by pharmacologic, non-pharmacologic means and by more invasive procedures. The options for pharmacologic treatment are various, since nonopioid, opioid analgesics and co-analgesics. The authors present a review of the pharmacological treatment of cancer pain and alert to the importance of the recognition of pain as an illness and the possibility to be relieved.