Der Internist
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Pain and breathlessness are common symptoms in advanced disease. Pain should be treated with a combination of non-opioids and opioids. Coanalgesics play an important role in the treatment of neuropathic pain. ⋯ Opioids are the drugs of choice for breathlessness. The efficacy of benzodiazepines could not be confirmed, they should only be used as second line therapy. Also, oxygen should only be given regularly after an individual test.
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Porphyrias are metabolic disorders of the heme biosynthesis. Clinically, they can be differentiated into acute and non-acute porphyrias. The symptomatic phase of acute hepatic porphyrias is characterized by overproduction of neurotoxic porphyrin precursors and porphyrins. ⋯ The location of the deficient enzyme within the heme biosynthesic pathway determines the pattern of the accumulated porphyrins. Besides light protection, there are different therapies depending on the type of non-acute porphyria. Ultimately, liver transplantation may be considered in therapy-resistant cases of acute hepatic porphyrias and bone marrow transplantation in severe cases of erythropoietic porphyrias.
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A 29-year old woman was admitted with recurrent hemoptysis. Due to the combination of hemoptysis, alveolar infiltrates and anemia a diffuse alveolar hemorrhage syndrome was suspected. After exclusion of underlying diseases and due to the typical histology we confirmed the diagnosis of an idiopathic pulmonary hemosiderosis. In consequence of an acute progression of the disease with pulmonary hemorrhage and severe anemia an empiric therapy with corticosteroids and azathioprine was initiated, resulting in a distinct recovery.