巨型肾血管肌脂肪瘤和肺淋巴管瘤病：十多年后的随访报告

IF 0.5 Q4 UROLOGY & NEPHROLOGY

Saudi Journal of Kidney Diseases and Transplantation Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI:10.4103/sjkdt.sjkdt_324_21

Kiran Nasir, Aasim Ahmad

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引用次数: 0

摘要

肾血管肌脂肪瘤（AML）和肺淋巴管瘤病（LAM）是结节性硬化症（TS）的两种主要表现形式，TS是一种常染色体显性多系统疾病。mTOR抑制剂在患有肾AML、LAM和脐下巨细胞星形细胞瘤的TS患者中显示出良好的疗效。本病例报告是我们在 2010 年报告的一名巨型 AML 和 LAM 患者的随访报告，该患者因左侧巨型 AML 大出血而需要进行紧急肾切除术。

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Giant Renal Angiomyolipomas and Pulmonary Lymphangioleiomyomatosis: Follow-up Report after More than a Decade.

Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis and kidney transplant. mTOR inhibitors showed promising results in TS patients with renal AMLs, LAM, and subependymal giant cell astrocytomas. This case report is a follow-up of a patient we reported in 2010 with giant AMLs and LAM who required an emergency nephrectomy for massive hemorrhage from giant left-sided AMLs.

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来源期刊

Saudi Journal of Kidney Diseases and Transplantation UROLOGY & NEPHROLOGY-

CiteScore

1.00

自引率

0.00%

发文量

审稿时长

53 weeks

期刊介绍： Saudi Journal of Kidney Diseases and Transplantation (SJKDT, ISSN 1319-2442) is the official publication of the Saudi Center for Organ Transplantation, Riyadh, Saudi Arabia. It is published six times a year. SJKDT publishes peer-reviewed original research work and review papers related to kidney diseases, urinary tract, renal replacement therapies, and transplantation. The journal publishes original papers and reviews on cell therapy and islet transplantation, clinical transplantation, experimental transplantation, immunobiology and genomics and xenotransplantation related to the kidney. The journal also publishes short communications, case studies, letters to the editors, an annotated bibliography and a column on news and views.