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印度常染色体显性多囊肾病中的特大肾脏

Exceptionally Large Kidneys in Autosomal Dominant Polycystic Disease in India.

作者信息

Shiza Saher T, Guttikonda Jyothsna

机构信息

Internal Medicine, Deccan College of Medical Sciences, Hyderabad, IND.

Nephrology, Star Hospitals, Hyderabad, IND.

出版信息

Cureus. 2021 Mar 15;13(3):e13905. doi: 10.7759/cureus.13905.

DOI:10.7759/cureus.13905
PMID:33880261
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8046683/
Abstract

We report a case of exceptionally large kidneys in autosomal dominant polycystic kidney disease (ADPKD) in India. A 43-year-old male with a family history of ADPKD presented with abdominal pain, intermittent fever, and a sense of bilateral fullness in both flanks. On examination, he had bilaterally enlarged kidneys extending towards iliac fossae. The serum metabolic panel revealed elevated serum creatinine and blood urea nitrogen. Ultrasound abdomen and pelvis showed enlarged kidneys with parenchyma replaced by multiple varying-sized cysts and few cysts in both the kidneys leading to hemorrhagic transformation. CT abdomen showed bulky bilateral kidneys with multiple non-communicating cysts, with few cysts showing the hemorrhagic and calcific transformation. The right kidney measured 30.3 x 15 cm, weighing 9 lb, was resected. The left kidney measured 37.0 x 14.0 cm and was resected three months later. The specimen weighed 19.8 lb. Histopathological examination showed a gross specimen with a bossellated surface composed of sub-capsular multiple cysts of varying sizes. Both the enlarged kidneys were resected due to cyst hemorrhage and infection. The patient is currently on hemodialysis until he receives a renal graft.

摘要

我们报告了一例印度常染色体显性多囊肾病(ADPKD)患者出现异常巨大肾脏的病例。一名有ADPKD家族史的43岁男性,出现腹痛、间歇性发热以及双侧胁腹部胀满感。检查发现,他双侧肾脏增大并延伸至髂窝。血清代谢指标显示血清肌酐和血尿素氮升高。腹部及盆腔超声检查显示肾脏增大,实质被多个大小不一的囊肿取代,双侧肾脏有少数囊肿发生出血性转变。腹部CT显示双侧肾脏肿大,有多个不连通的囊肿,少数囊肿呈现出血性和钙化性转变。右肾大小为30.3×15厘米,重9磅,被切除。左肾大小为37.0×14.0厘米,三个月后被切除。标本重19.8磅。组织病理学检查显示大体标本表面呈乳头状,由包膜下多个大小不一的囊肿组成。由于囊肿出血和感染,双侧增大的肾脏均被切除。该患者目前正在接受血液透析,直至接受肾移植。

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本文引用的文献

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Cerebral Aneurysms in Autosomal Dominant Polycystic Kidney Disease: A Comparison of Management Approaches.常染色体显性多囊肾病相关的颅内动脉瘤:管理方法的比较。
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A case of unilateral nephrectomy performed for autosomal dominant polycystic kidney disease with marked unilateral enlargement.一例因常染色体显性多囊肾病伴显著单侧增大而行单侧肾切除术的病例。
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