Department of Cardiology, The First College of Clinical Medical Science, China Three Gorges University and Yichang Central People's Hospital, Yichang, China.
Department of Cardiology, Institute of Cardiovascular Diseases, China Three Gorges University, Yichang, China.
Medicine (Baltimore). 2024 Aug 2;103(31):e39128. doi: 10.1097/MD.0000000000039128.
Turner syndrome is characterized by complete or partial loss of the second sex chromosome. In patients with Turner syndrome, hypertension is well described. However, the literature regarding malignant hypertension is scarce. Therefore, an accurate and timely diagnosis and treatment are important.
A 13-year-old female with Turner syndrome presented to the emergency department with malignant hypertension, headache, spraying vomiting, convulsion, and loss of consciousness. Considering her medical history, symptoms, and auxiliary examination, secondary hypertension (primary reninism) was suspected, but without any occupying or hyperplasia in renal and adrenal.
A type of secondary hypertension, primary reninism.
The patient was immediately transferred to the pediatric intensive care unit. Subsequently, she was given nifedipine 0.35 mg/kg and captopril 0.35mg/kg to reduce blood pressure (BP), mannitol and furosemide to reduce cranial pressure, and phenobarbital and midazolam to terminate restlessness successively. Three hours later, the BP was consistently higher than 170/120 mm Hg, sodium nitroprusside was pumped intravenously, then, giving oral drug transition. Finally, she was given Valsartan-Amlodipine Tablets (I) (80 mg valsartan and 5 mg amlodipine per day) and bisoprolol (2.5 mg per day).
For 2.5 years of follow-up, the BP reduced to 110-130/60-85 mm Hg, heart rate ranged between 65 and 80 bpm, and she could go to school without any headache, convulsion, and syncope.
The clinical phenotype of Turner syndrome is complex and varied, affecting multiple systems and organs. Turner syndrome with malignant hypertension is rare, so we should systematically evaluate secondary hypertension, target-organ damage, and accompanied by standard management when Turner syndrome presents with hypertension.
特纳综合征的特征是完全或部分丢失第二条性染色体。特纳综合征患者中高血压描述较多,但恶性高血压文献较少。因此,准确及时的诊断和治疗非常重要。
一位 13 岁的特纳综合征女性患者因恶性高血压、头痛、喷射性呕吐、抽搐和意识丧失到急诊科就诊。考虑到她的病史、症状和辅助检查,怀疑为继发性高血压(原发性肾素增多症),但肾脏和肾上腺无占位或增生。
一种继发性高血压,原发性肾素增多症。
患者立即被转至儿科重症监护病房。随后,给予硝苯地平 0.35mg/kg 和卡托普利 0.35mg/kg 以降低血压,甘露醇和呋塞米降低颅内压,苯巴比妥和咪达唑仑依次终止躁动。3 小时后,血压持续高于 170/120mmHg,静脉泵入硝普钠,然后过渡到口服药物。最后,给予缬沙坦氨氯地平片(I)(每天 80mg 缬沙坦和 5mg 氨氯地平)和比索洛尔(每天 2.5mg)。
随访 2.5 年,血压降至 110-130/60-85mmHg,心率在 65-80bpm 之间,头痛、抽搐和晕厥消失,可上学。
特纳综合征的临床表型复杂多样,影响多个系统和器官。伴有恶性高血压的特纳综合征少见,因此当特纳综合征出现高血压时,应系统评估继发性高血压、靶器官损害,并进行标准管理。