未经证实:比尔贫血点,紫癜,和荨麻疹样爆发(BASCULE)综合征是一种漏报的儿科血管疾病,来自肢端综合征组。在儿童中,这些包括阵发性肢端综合征(雷诺现象和冻疮样病变),永久性肢端综合征(肢端发色),和短暂性肢端综合征,它们的病原体与病毒感染有关,爱泼斯坦-巴尔病毒,and,最近,SARS-CoV-2。
UNASSIGNED:我们报告了一例与体位性心动过速综合征(POTS)相关的BASCULE综合征,并对描述儿童BASCULE综合征的病例报告进行了叙述性回顾。此外,我们介绍了在法国医学界进行的前瞻性实践调查的结果.
UNASSIGNED:一名14岁男孩报告下肢有瘙痒性红氰状病变,这种情况发生在他站立的时候,当他躺下时完全解决了。他报告了虚弱和抽筋。他提出了与POTS相关的典型BASCULE综合征,并通过倾斜测试证实。身体检查和血管检查均在正常范围内。我们确定了12例病例报告,描述了自2016年以来的21例儿科病例。大多数患者是12至19岁的青少年或新生儿。此外,文献中有20%的病例出现POTS或直立不耐受。我们对95名法国医生的调查证实,在一般儿科实践中,BASCULE综合征是一种未被诊断和未被认可的疾病。至少在法国。在这些医生中,65%的人已经遇到过类似症状的患者,但只有30%的人宣称他们了解巴苏尔综合征.
UNASSIGNED:对临床表现的认识不足导致患者咨询急诊室,进行了多次不必要的调查。因此,我们建议BASCULE综合征的诊断基于临床观察,不需要实验室测试,避免不必要的医疗费用。我们建议医生在怀疑POTS时进行倾斜测试。
UNASSIGNED: Bier anemic spots, cyanosis, and urticaria-like eruption (BASCULE) syndrome is an underreported pediatric vascular disorder from the group of acrosyndromes. In children, these include paroxysmal acrosyndromes (Raynaud\'s phenomenon and chilblain-like lesions), permanent acrosyndromes (acrocyanosis), and transient acrosyndromes, in which their pathogeneses are associated with virus infections, Epstein-Barr virus, and, more recently, SARS-CoV-2, respectively.
UNASSIGNED: We reported a
case of BASCULE syndrome associated with postural orthostatic tachycardia syndrome (POTS) and provided a narrative review of
case reports describing the BASCULE syndrome in children. Moreover, we presented the results of a prospective practice survey that we performed in the French medical community.
UNASSIGNED: A 14-years-old boy reported pruritic erythrocyanic lesions on the lower limbs, which occurred whenever he was in a standing position and fully resolved when he laid down. He reported asthenia and cramps. He presented a typical BASCULE syndrome associated with POTS confirmed by a tilt-test. Physical and vascular examinations were within the normal range. We identified 12
case reports, describing 21 pediatric cases since 2016. Most patients were adolescents between 12 and 19 years of age or were newborns. Furthermore, 20% of cases in the literature have presented POTS or orthostatic intolerance. Our survey among 95 French physicians confirmed that BASCULE syndrome is an underdiagnosed and under recognized disease in the general pediatric practice, at least in France. Among these physicians, 65% had already encountered patients with similar symptoms, but only 30% declared that they had knowledge of the BASCULE syndrome.
UNASSIGNED: The under-recognition of the clinical manifestations leads the patients to consult emergency rooms, with multiple unnecessary investigations performed. Therefore, we suggest that the diagnosis of BASCULE syndrome is based on clinical observations, without the need for laboratory tests, to avoid unnecessary health costs. We suggest physicians to perform a tilt-test when POTS is suspected.