Tönjes Anke, Quinkler Marcus, Knappe Ulrich, Störmann Sylvère, Schöfl Christof, Schopohl Jochen, Meyhöfer Sebastian M
Dtsch Med Wochenschr. 2023 Mar;148(7):380-385. doi: 10.1055/a-1847-2553. Epub 2023 Mar 20.
Acromegaly is a rare disease in which chronic growth hormone overproduction (usually from an anterior pituitary adenoma) leads to various systemic complications. The management of acromegaly and the comorbidities of the disease is complex and requires a multidisciplinary approach. Early diagnosis is extremely important, as then the chances of a complete cure are significantly higher. The operation is the therapy of first choice and should be performed at a specialized center with an experienced neurosurgeon. With good patient information and guidance, the drug therapy of acromegaly patients in specialized practices and clinics can usually lead to biochemical control and thereby normalization of mortality risk. As with numerous rare diseases, care in specialized centers and recording and evaluation in registry studies can contribute to better patient care and the optimization of therapy and diagnostic guidelines. We assume that with the help of the German Acromegaly Registry, which currently includes more than 2500 patients with acromegaly, we will be able to present a realistic picture of the care situation in Germany in the coming years.
肢端肥大症是一种罕见疾病，慢性生长激素分泌过多（通常源于垂体前叶腺瘤）会导致各种全身并发症。肢端肥大症及其合并症的管理很复杂，需要多学科方法。早期诊断极为重要，因为此时完全治愈的几率会显著更高。手术是首选治疗方法，应由经验丰富的神经外科医生在专业中心进行。通过良好的患者信息告知和指导，在专业诊所对肢端肥大症患者进行药物治疗通常可实现生化控制，从而使死亡风险正常化。与众多罕见疾病一样，在专业中心进行护理以及在登记研究中进行记录和评估有助于更好地照顾患者，并优化治疗和诊断指南。我们认为，借助目前已纳入2500多名肢端肥大症患者的德国肢端肥大症登记处，我们将能够在未来几年呈现德国护理情况的真实图景。
