PDF(Pubmed)
Abstract：
BACKGROUND: 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.
METHODS: 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.
METHODS: A type of secondary hypertension, primary reninism.
METHODS: 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).
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
METHODS: A type of secondary hypertension, primary reninism.
METHODS: 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).
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
背景：特纳综合征的特征是第二性染色体完全或部分丢失。在特纳综合征患者中，高血压是很好的描述。然而,关于恶性高血压的文献很少.因此,准确及时的诊断和治疗很重要。
方法：一名13岁女性患者，因恶性高血压就诊于急诊科，头痛,喷吐,抽搐,和失去知觉。考虑到她的病史,症状,和辅助检查，怀疑继发性高血压（原发性肾素增多症），但肾脏和肾上腺无任何占位或增生。
方法：A型继发性高血压，初级肾素主义。
方法：患者立即转移到儿科重症监护病房。随后，给予硝苯地平0.35mg/kg和卡托普利0.35mg/kg以降低血压（BP），甘露醇和呋塞米降低颅压，苯巴比妥和咪达唑仑相继终止躁动。三个小时后,BP始终高于170/120mmHg，硝普钠静脉注射，然后,给予口服药物过渡。最后,患者接受缬沙坦-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).
结果：经过2.5年的随访，BP降至110-130/60-85mmHg，心率在65和80bpm之间，她可以毫无头痛地上学,抽搐,和晕厥。
结论：特纳综合征的临床表型复杂多变，影响多个系统和器官。Turner综合征合并恶性高血压少见,所以我们应该系统地评估继发性高血压，靶器官损伤,并伴随着标准的管理，当特纳综合征出现高血压。
方法：一名13岁女性患者，因恶性高血压就诊于急诊科，头痛,喷吐,抽搐,和失去知觉。考虑到她的病史,症状,和辅助检查，怀疑继发性高血压（原发性肾素增多症），但肾脏和肾上腺无任何占位或增生。
方法：A型继发性高血压，初级肾素主义。
方法：患者立即转移到儿科重症监护病房。随后，给予硝苯地平0.35mg/kg和卡托普利0.35mg/kg以降低血压（BP），甘露醇和呋塞米降低颅压，苯巴比妥和咪达唑仑相继终止躁动。三个小时后,BP始终高于170/120mmHg，硝普钠静脉注射，然后,给予口服药物过渡。最后,患者接受缬沙坦-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).
结果：经过2.5年的随访，BP降至110-130/60-85mmHg，心率在65和80bpm之间，她可以毫无头痛地上学,抽搐,和晕厥。
结论：特纳综合征的临床表型复杂多变，影响多个系统和器官。Turner综合征合并恶性高血压少见,所以我们应该系统地评估继发性高血压，靶器官损伤,并伴随着标准的管理，当特纳综合征出现高血压。
