Rajalingam V, Chao S M, Tan C L, Tan I K
Department of Paediatrics, Singapore General Hospital, Singapore.
Ann Acad Med Singap. 1996 Jul;25(4):587-9.
Severe sustained hypertension occurs in only 0.1% of the paediatric population and only about 2% of these patients will have an underlying endocrine cause. Phaeochromocytoma as a catecholamine-secreting tumour causing severe hypertension is exceedingly rare in children. A high index of suspicion and an awareness of the clinical spectrum are therefore necessary to make the diagnosis. Phaeochromocytomas can have protean manifestations which may be mistaken for a variety of clinical conditions. We highlight the problems encountered in making the diagnosis in an 11-year-old Chinese girl who presented with sustained hypertension, heart failure and transient renal impairment with two normal 24-hour urinary vanillyl mandelic acid (VMA) results before a third produced the diagnosis. We emphasize that total reliance on a single biochemical urinary screening is not acceptable. The measurement of urinary catecholamines or their metabolites increases the sensitivity of diagnosis. We recommend that in situations where biochemical screening is doubtful, appropriate imaging should be undertaken to exclude the diagnosis.
严重持续性高血压仅发生于0.1%的儿科人群，其中仅有约2%的患者存在潜在的内分泌病因。嗜铬细胞瘤作为一种分泌儿茶酚胺的肿瘤，导致严重高血压，在儿童中极为罕见。因此，高度的怀疑指数和对临床谱的认识对于做出诊断是必要的。嗜铬细胞瘤可有多种表现，可能被误诊为各种临床疾病。我们强调了在一名11岁中国女孩的诊断过程中遇到的问题，该女孩表现为持续性高血压、心力衰竭和短暂性肾功能损害，在第三次检测前两次24小时尿香草扁桃酸（VMA）结果均正常，第三次检测才确诊。我们强调完全依赖单一的生化尿液筛查是不可接受的。测定尿儿茶酚胺或其代谢产物可提高诊断的敏感性。我们建议，在生化筛查结果存疑的情况下，应进行适当的影像学检查以排除诊断。
