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桥本甲状腺炎伴脑桥中央髓鞘溶解症青少年中出现的多自身免疫及肾小管酸中毒的不常见表现。

Unusual Presentation of Polyautoimmunity and Renal Tubular Acidosis in an Adolescent With Hashimoto's Thyroiditis and Central Pontine Myelinolysis.

机构信息

Department of Pediatrics I, Neonatology, Pediatric Intensive Care, Pediatric Neurology, University Hospital Essen, University Duisburg-Essen, Duisburg, Germany.

Department of Pediatrics II, Pediatric Nephrology, University Hospital Essen, University Duisburg-Essen, Duisburg, Germany.

出版信息

Front Endocrinol (Lausanne). 2020 Oct 14;11:548877. doi: 10.3389/fendo.2020.548877. eCollection 2020.

Abstract

Hashimoto's thyroiditis is frequently associated with other autoimmune diseases and may include renal involvement. A 17-year-old female with previously diagnosed Hashimoto's thyroiditis and vitiligo was admitted to a pediatric intensive care unit with hypokalemic paralysis and acidosis, after having suffered from recurrent muscular weakness for approximately one year. A few days later she developed central pontine myelinolysis. After initial stabilization she was also diagnosed with distal renal tubular acidosis (dRTA) and tubular proteinuria which can occur in Sjögren's syndrome. Extended screening for autoimmune diseases additionally revealed celiac disease. Treatment with Prednisone and substitution of potassium quickly lead to the resolution of proteinuria and dRTA, but unilateral paralysis of the sixth nerve as a result of central pontine myelinolysis was irreversible. This is the rare case of polyautoimmunity including autoimmune thyroiditis, Sjögren's syndrome, vitiligo and celiac disease in an adolescent with few disease-specific symptoms. The diagnoses were made via a complicating nephritis causing dRTA and proteinuria. Delay in diagnosis lead to permanent neurological damage. This case highlights the need for pediatricians to be aware of rare accompanying diseases and their complications in "common" pediatric autoimmune diseases like Hashimoto's thyroiditis and celiac disease.

摘要

桥本甲状腺炎常与其他自身免疫性疾病相关,可能包括肾脏受累。一名 17 岁女性,既往诊断为桥本甲状腺炎和白癜风,在经历约 1 年的反复肌肉无力后,因低钾性瘫痪和酸中毒入住儿科重症监护病房。几天后,她出现了脑桥中央髓鞘溶解症。在初始稳定后,她还被诊断为远端肾小管酸中毒(dRTA)和肾小管蛋白尿,这可能发生在干燥综合征中。对自身免疫性疾病的进一步筛查还发现了乳糜泻。泼尼松治疗和钾替代迅速导致蛋白尿和 dRTA 得到解决,但由于脑桥中央髓鞘溶解症导致的第六对脑神经单侧瘫痪是不可逆的。这是一例罕见的多自身免疫性疾病,包括青少年自身免疫性甲状腺炎、干燥综合征、白癜风和乳糜泻,其疾病特异性症状较少。这些诊断是通过导致 dRTA 和蛋白尿的并发肾炎做出的。诊断延迟导致了永久性的神经损伤。这个病例强调了儿科医生需要意识到“常见”儿科自身免疫性疾病(如桥本甲状腺炎和乳糜泻)中罕见的伴随疾病及其并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb4f/7591671/6650b76a3bbc/fendo-11-548877-g0001.jpg

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