早发性常染色体多囊肾病患者甲状腺结节的发病率。

Incidence of thyroid nodules in early stage autosomal polycystic kidney disease.

机构信息

Department of Endocrinology and Internal Medicine, Medical University of Gdansk, Gdansk, Poland.

Department of Nephrology, Transplantology and Internal Diseases, Faculty of Medicine, Medical University of Gdansk, Gdansk, Poland.

出版信息

BMC Nephrol. 2022 Mar 3;23(1):85. doi: 10.1186/s12882-022-02714-w.

DOI:10.1186/s12882-022-02714-w

PMID:35241008

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8892779/

Abstract

BACKGROUND

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Defect in cilia-mediated signaling activity is a crucial factor leading to cyst formation. Hence, ADPKD is regarded as a systemic disorder with multiple extrarenal complications, including cysts in other organs, for instance, the liver, pancreas, spleen, or ovaries. Interestingly, loss-of-function of primary cilia has been recently found to contribute to a malignant transformation from degenerated thyroid follicles. However, the increased incidence of thyroid nodules in ADPKD patients has not yet been fully confirmed.

OBJECTIVES

To determine the incidence of thyroid lesions in patients with ADPKD in comparison to previous population studies. Moreover, we aimed to investigate if the pace of the disease progression is associated with a higher prevalence of thyroid lesions.

MATERIAL AND METHODS

In 49 early-stage ADPKD patients recruited from our center, we performed ultrasonography of the thyroid glands, and laboratory evaluation of thyroids function. We compared the results with population studies.

RESULTS

Twenty-three individuals had solid, cystic-solid, or cystic lesions revealed in the ultrasonography and 2 patients had a positive past medical history for thyroidectomy due to nodular goiter. In 10 patients out of the 23, only minor cysts with no clinical significance were found and 13 out of the 23 patients had solid or cystic-solid lesions, which occurred to be benign based on three years of follow-up or the biopsy of the nodule.

CONCLUSIONS

We found no increased incidence of thyroid gland lesions in early ADPKD patients in comparison to previous population studies. Plausibly, mechanisms other than defective cilia signaling are involved in the risk for focal thyroid lesions formation. Moreover, the rate of progression of kidney function decline seems to be not accompanied by the higher incidence of thyroid pathology.

摘要

背景

常染色体显性多囊肾病（ADPKD）是最常见的遗传性肾脏疾病。纤毛介导的信号活性缺陷是导致囊肿形成的关键因素。因此，ADPKD被认为是一种全身性疾病，伴有多种肾脏外并发症，包括其他器官的囊肿，例如肝、胰腺、脾或卵巢。有趣的是，最近发现原发性纤毛丧失功能有助于退化的甲状腺滤泡发生恶性转化。然而，ADPKD 患者甲状腺结节的发病率增加尚未得到充分证实。