gAChR antibodies in children and adolescents with acquired autoimmune dysautonomia in Japan.
gAChR antibodies in children and adolescents with acquired autoimmune dysautonomia in Japan.
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DOI：
10.1002/acn3.51317
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发表时间：
2021-04
影响因子：
5.3
通讯作者：
Nakane S
中科院分区：
文献类型：
--
作者：
Yamakawa M;Watari M;Torii KI;Kuki I;Miharu M;Kawazu M;Mukaino A;Higuchi O;Maeda Y;Ikeda T;Takamatsu K;Tawara N;Nakahara K;Matsuo H;Ueda M;Takahashi T;Nakane S
获得性自主神经功能障碍患者可能存在神经节细胞烟碱型乙酰胆碱受体(GAChR)特异性抗体。然而，儿童和青少年获得性自主神经功能障碍(AAD)的临床特征尚不清楚。本研究旨在探讨儿童获得性自主神经功能障碍的临床特点。本研究回顾了2012年1月至2019年4月间转诊至本实验室进行抗体检测的一系列血清gAChR抗体阳性的AAD患者。本研究包括2 0 0例有≥临床特征的患者(2 0年，2 0例；2 0年，175例)。通过对儿童和成人患者的比较，我们发现AAD的先天感染和起病时的自主神经症状以及胃肠道症状在儿童中更常见。我们确认有4名儿童(20.0%)符合体位直立性心动过速综合征(POTS)的诊断标准。拥有罐子的儿童数量明显高于成年人(P=0.002)。此外，上消化道功能障碍在儿童中比在成人中更普遍(P=0.042)。特别是，60.0%的儿童和21.1%的成人会出现恶心和呕吐(P<；0.001)。小儿麻痹性肠梗阻发生率(20.0%)明显高于成人(6.3%)(P=0.030)。至于自主神经以外的表现，脑病在儿童(15.0%)比成人(1.1%)更常见(P<；0.001)。儿童AAD患者有其自身的临床特点，这些特点可能是儿童和青少年所特有的。
Patients with acquired autonomic dysfunction may have antibodies specific to the ganglionic nicotinic acetylcholine receptor (gAChR). However, the clinical features of children and adolescents with acquired autonomic dysfunction (AAD) remain unclear. This study aimed to determine the clinical features of pediatric patients with acquired autonomic dysfunction. This study retrospectively examined a series of patients of AAD with serum gAChR antibodies who were referred to our laboratory for antibody testing between January 2012 and April 2019. The study included 200 patients (<20 years, 20 cases; ≥20 years, 175 cases) with clinical features of AAD. Upon comparing pediatric and adult patients, we found that antecedent infection and autonomic symptoms at onset with gastrointestinal symptoms occurred more frequently in children with AAD. We confirmed that four children (20.0%) met the diagnostic criteria for postural orthostatic tachycardia syndrome (POTS). A significantly higher number of children than adults had POTS (P = 0.002). In addition, upper GI dysfunction was more prevalent in children than in adults (P = 0.042). In particular, nausea and vomiting occurred in 60.0% of children with AAD and in 21.1% of adults (P < 0.001). The frequency of paralytic ileus was significantly higher in children with AAD (20.0%) relative to adults (6.3%) (P = 0.030). Regarding extra‐autonomic manifestations, encephalopathy was more frequent in children (15.0%) than in adults (1.1%) (P < 0.001). Pediatric AAD patients have their own clinical characteristics, and these features may be unique to children and adolescents.
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影响因子：
4.4
作者：
Blitshteyn, Svetlana;Brinth, Louise;Martinez-Lavin, Manuel
通讯作者：
Martinez-Lavin, Manuel
影响因子：
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作者：
Dhamija, Radhika;Tan, K. Meng;Lennon, Vanda A.
通讯作者：
Lennon, Vanda A.
影响因子：
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作者：
Maeda Y;Migita K;Higuchi O;Mukaino A;Furukawa H;Komori A;Nakamura M;Hashimoto S;Nagaoka S;Abiru S;Yatsuhashi H;Matsuo H;Kawakami A;Yasunami M;Nakane S
通讯作者：
Nakane S
影响因子：
2.7
作者：
Baker, S. K.;Morillo, C.;Vernino, S.
通讯作者：
Vernino, S.
影响因子：
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作者：
Gibbons, Christopher H.;Centi, Justin;Freeman, Roy
通讯作者：
Freeman, Roy