PDF(Pubmed)
Abstract：
UNASSIGNED: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease and the 4th leading cause of renal replacement therapy in the world. ADPKD is a systemic disorder as cysts may develop in several organs. Liver cysts are the most common extrarenal manifestations and are often incidentally detected. Even though cysts do not influence liver function, they can grow to a very great size and can significantly enlarge liver volume, causing structural distortion of the biliary tree and patient discomfort due to the mass effect. Nephrectomy is frequently considered in preparation for renal transplantation in patients with remarkable kidneys\' enlargement. There are currently no globally recognized clinical guidelines for nephrectomy. Although cysts do not normally affect liver function in ADPKD, after nephrectomy cases of liver fibrosis and Budd-Chiari have been reported. These are uncommon disorders due to the obstruction of the blood flow in the hepatic venous causing spleen and liver volume enlargement, portal hypertension, and hepatic cirrhosis.
UNASSIGNED: We present a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy in 47-year-old ADPKD patient.
UNASSIGNED: This finding underscores the critical significance of meticulously examining the anatomical relationship between polycystic kidneys and the liver before performing nephrectomy. Additionally, it highlights the importance of assessing liver involvement and associated complications. By integrating liver assessment into the criteria, we can significantly enhance patient care and improve the overall management of ADPKD before kidney transplantation.
UNASSIGNED: We present a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy in 47-year-old ADPKD patient.
UNASSIGNED: This finding underscores the critical significance of meticulously examining the anatomical relationship between polycystic kidneys and the liver before performing nephrectomy. Additionally, it highlights the importance of assessing liver involvement and associated complications. By integrating liver assessment into the criteria, we can significantly enhance patient care and improve the overall management of ADPKD before kidney transplantation.
摘要：
■常染色体显性遗传性多囊肾病（ADPKD）是最常见的遗传性肾脏疾病，也是世界上第四大肾脏替代治疗的主要原因。ADPKD是一种全身性疾病，因为囊肿可能在几个器官中发展。肝囊肿是最常见的肾外表现，常被偶然发现。即使囊肿不影响肝功能，它们可以长到非常大的尺寸，并且可以显着扩大肝脏体积，由于质量效应导致胆道树结构变形和患者不适。在肾脏明显增大的患者的肾移植准备中，经常考虑进行肾切除术。目前尚无全球公认的肾切除术临床指南。尽管囊肿通常不会影响ADPKD的肝功能，肾切除术后肝纤维化和Budd-Chiari的病例已有报道。这些是罕见的疾病，由于肝静脉血流阻塞导致脾脏和肝脏体积增大，门静脉高压症,和肝硬化。
■我们介绍了一例肝纤维化伴脾肿大和严重全血细胞减少的病例，这是47岁的ADPKD患者双侧肾切除术后的迟发性并发症。
■这一发现强调了在进行肾切除术之前仔细检查多囊肾和肝脏之间的解剖关系的重要意义。此外,它强调了评估肝脏受累和相关并发症的重要性.通过将肝脏评估纳入标准，我们可以显着加强患者护理，改善肾移植前ADPKD的整体管理。
■我们介绍了一例肝纤维化伴脾肿大和严重全血细胞减少的病例，这是47岁的ADPKD患者双侧肾切除术后的迟发性并发症。
■这一发现强调了在进行肾切除术之前仔细检查多囊肾和肝脏之间的解剖关系的重要意义。此外,它强调了评估肝脏受累和相关并发症的重要性.通过将肝脏评估纳入标准，我们可以显着加强患者护理，改善肾移植前ADPKD的整体管理。
