Abrantes Catarina, Brigas Daniela, Casimiro Hugo Jorge, Madeira Margarida
Department of Nephrology, Setúbal Hospital Center, Setúbal, Portugal.
Department of Internal Medicine, Setúbal Hospital Center, Setúbal, Portugal.
BMJ Case Rep. 2018 Apr 5;2018:bcr-2017-224108. doi: 10.1136/bcr-2017-224108.
A 58-year-old male patient was admitted at the São Bernardos's Hospital (Setúbal, Portugal) with generalised muscle spasms, dyspnoea, laryngospasm and bronchospasm in the context of severe hypocalcaemia. Despite efforts to correct serum calcium, it remained below average, leading to question the true cause of hypocalcaemia. Low parathyroid hormone and 25-hydroxyvitamin D, along with facial anomalies, palate defect and cognitive impairment with concomitant psychiatric disorder led to a suspicion of a DiGeorge/velocardiofacial/22q11.2 deletion syndrome (DS), which was confirmed through genetic testing. The 22q11.2 DS has a wide phenotypic expression and there are growing reports of diagnosis being made in adulthood. This case report highlights the importance of understanding the cause of refractory hypocalcaemia and alerts medical community to carefully access these patients, for this metabolic disorder may only present in later stages of life.
一名58岁男性患者因严重低钙血症导致全身肌肉痉挛、呼吸困难、喉痉挛和支气管痉挛,被收治于圣贝尔纳多医院(葡萄牙塞图巴尔)。尽管努力纠正血清钙水平,但仍低于正常水平,这使得人们对低钙血症的真正原因产生疑问。甲状旁腺激素和25-羟维生素D水平较低,同时伴有面部畸形、腭裂以及认知障碍和精神疾病,这引发了对DiGeorge/心脏面综合征/22q11.2缺失综合征(DS)的怀疑,基因检测证实了这一诊断。22q11.2 DS具有广泛的表型表达,成年期诊断的报告也越来越多。本病例报告强调了了解难治性低钙血症病因的重要性,并提醒医学界要仔细评估这些患者,因为这种代谢紊乱可能仅在生命后期出现。