Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
Department of Pediatrics, McMaster University, Hamilton, Ontario, Canada.
Am J Med Genet A. 2021 Mar;185(3):766-773. doi: 10.1002/ajmg.a.62034. Epub 2020 Dec 24.
Reports of patients with concomitant diagnoses of two inherited genetic disorders, sometimes referred to as "double trouble," have appeared intermittently in the medical literature. We report eight additional cases with dual diagnoses of two genetic conditions. All cases had a phenotype atypical for their primary diagnosis, leading to the search for a second genetic diagnosis. These cases highlight the importance of the history, physical examination and continued work-up if the phenotype of the patient falls drastically outside what has been reported with their primary diagnosis. Some of the diagnoses of the patients presented here (e.g., Myotonic Dystrophy Type 1, fascioscapulohumeral muscular dystrophy) would not have been identified by genetic testing done on a next generation sequencing backbone (e.g., panel or exome sequencing). When the clinical picture is atypical or more severe than expected the possibility of a dual diagnosis (double trouble) should be considered. Identification of a second genetic condition can impact management and genetic counseling.
患者同时患有两种遗传性疾病的报告,有时被称为“双重麻烦”,在医学文献中时有出现。我们报告了另外 8 例同时患有两种遗传疾病的病例。所有病例的表型均不符合其主要诊断的典型特征,导致寻找第二种遗传诊断。这些病例强调了如果患者的表型与主要诊断的报告明显不同,那么需要进行病史、体格检查和进一步检查的重要性。这里介绍的一些患者的诊断(例如,强直性肌营养不良 1 型、肩肱型肌营养不良症)如果仅通过下一代测序(例如,panel 或外显子测序)进行基因检测,可能无法识别。当临床表现不典型或比预期更严重时,应考虑双重诊断(双重麻烦)的可能性。确定第二种遗传疾病可以影响管理和遗传咨询。