Der Internist
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In November 2017, the latest American guidelines for the management of arterial hypertension were published. With these guidelines lowering the threshold for hypertension to 130/80 mm Hg, the latest European guidelines were expected with excitement. ⋯ The latest European guidelines for the management of hypertension include several changes. One of the most important aspects is that-in contrast to the American guidelines-the threshold for diagnosis remains at 140/90 mm Hg, whereas treatment target range has been lowered by roughly 10 mm Hg and single pill fixed dose combinations are recommended.
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Within the last two decades the therapeutic options for newly diagnosed multiple myeloma have changed dramatically. The implementation of high-dose chemotherapy with melphalan and subsequent autologous blood stem cell transplantation initially led to prolonged survival in younger, fit patients. Furthermore, recent data suggest that patients with high-risk disease seem to benefit most from tandem transplantation approaches. ⋯ For patients where high-dose therapy and stem cell transplantation are not feasible, bortezomib and lenalidomide are available as backbone agents of various combination regimens. Recently, the anti-CD38 antibody daratumumab has been approved in combination with bortezomib, melphalan and prednisone as primary treatment for newly diagnosed patients. An allogeneic stem cell transplantation can be considered for younger patients without relevant comorbidities and with high-risk disease or early relapse after autologous blood stem cell transplantation but should only be performed within controlled clinical trials and in specialized centers.
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Mucoviscidosis or cystic fibrosis (CF) is one of the most frequent monogenetic diseases in middle Europe. It is inherited in an autosomal recessive manner. A defect in the cystic fibrosis transmembrane conductance regulator (CFTR) channel reduces chloride ion transport to the cell membrane, which leads to malfunctions in all exocrine glands. ⋯ Therefore, the further development of symptomatic treatment becomes of great importance due to the complications that have already occurred before the implementation of the CFTR modulators. The implementation of modulators in early childhood can attenuate or prevent early irreversible complications. Therefore, in this article special emphasis is placed on new developments in symptomatic treatment and on new treatment options.
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Porphyrias are caused by enzyme defects of heme biosynthesis. According to their clinical presentation and to each affected pathway, they are categorized into acute and non-acute as well as hepatic and erythropoietic porphyrias. Acute hepatic porphyrias, e.g. acute intermittent porphyria (AIP), porphyria variegata (VP), hereditary coproporphyria (HCP) and 5‑aminolevulinic acid dehydratase-deficient porphyria (ALADP) are characterized by accumulation of the porphyrin precursors 5‑aminolevulinic acid (ALA) and porphobilinogen (PBG) that correlate with severe abdominal, psychiatric, neurological or cardiovascular symptoms. ⋯ Patients with PCT benefit from iron depletion, low-dose chloroquine treatment and/or hepatitis C virus elimination. Afamelanotide is associated with better sunlight tolerance in patients with EPP and XLP. Moreover, innovative therapies that highly selectively address dysregulated steps of the heme biosynthetic pathway are currently under clinical trial.
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Abstract