Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Oct 2009
ReviewThe clinical approach to lung disease in patients with cystic fibrosis.
There is strong evidence that early, aggressive therapy of lung disease leads to improved quality and quantity of life for patients with cystic fibrosis (CF). The treatment of pulmonary disease associated with CF is multifactorial, encompassing prophylaxis, aggressive treatment of infection, use of antiinflammatory agents, and treatment of severe complications. ⋯ Clinicians caring for patients with CF should maximize current therapies with the goal of preserving lung function until the time a more definitive curative or controller medication is developed. Empowering patients in the process of providing their own care is a key to achieving this goal.
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Symptoms of anxiety and depression have important consequences for patients with chronic disease, including lower rates of adherence and increased morbidity. This review discusses the prevalence of anxiety and depression in patients with cystic fibrosis (CF) across the life span and highlights current understanding of how these symptoms affect adherence, health outcomes, and health-related quality of life (HRQOL). Anxiety and depression appear to be more common in patients with CF than in the general population. ⋯ Depression, specifically, leads to lower adherence to recommended therapies. Given the prevalence and impact of anxiety and depression in CF, it seems appropriate to recommend annual screening using a validated approach, with referral for either pharmacotherapy or cognitive behavioral therapy of those with apparent symptoms. A large-scale epidemiological study of anxiety and depression in CF is under way and should provide better prevalence estimates and more information on the effects of these psychological symptoms.
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Tremendous progress has been made in understanding the genetics of hereditable pulmonary arterial hypertension (HPAH) since its description in the 1950s. Germline mutations in the gene coding bone morphogenetic receptor type 2 ( BMPR2) are detectable in the majority of cases of HPAH, and in a small proportion of cases of idiopathic pulmonary arterial hypertension (IPAH). ⋯ The variable clinical expression makes genetic counseling complex because the majority of carriers of a BMPR2 mutation will not be diagnosed with the disease. This issue will become increasingly important, as clinical testing for BMPR2 mutations is now available for the evaluation of patients and family members with HPAH and IPAH.
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Semin Respir Crit Care Med · Aug 2009
ReviewPulmonary hypertension in chronic obstructive pulmonary disease and interstitial lung diseases.
Pulmonary hypertension (PH) is a common complication of chronic respiratory diseases and particularly of chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILD). Owing to its frequency COPD is by far the most common cause of PH. It is generally a mild to moderate PH, pulmonary artery mean pressure (PAP) usually ranging between 20 and 25 mm Hg, but PH may worsen during exercise, sleep, and particularly during exacerbations of the disease. ⋯ In ILD the presence of PH predicts a poor prognosis. The treatment of PH relies on long-term oxygen therapy. "New" vasodilator drugs have rarely been used in COPD and ILD patients exhibiting severe PH. In advanced ILD the presence of PH is a supplemental argument for considering lung transplantation.
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Semin Respir Crit Care Med · Aug 2009
ReviewIdiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and differences.
Pulmonary veno-occlusive disease (PVOD) is a rare disorder and can be misdiagnosed as idiopathic pulmonary arterial hypertension (iPAH). PVOD and iPAH often share a similar clinical presentation, genetic background, and hemodynamic profile. PVOD accounts for 5 to 10% of cases initially considered as iPAH. ⋯ Therefore, a noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests, and bronchoalveolar lavage may be helpful to detect PVOD. In contrast with iPAH, PVOD is characterized by an even poorer prognosis and the possibility of developing severe pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice, but cautious use of specific PAH therapy can be helpful in select patients while awaiting this intervention.