scholarly journals Renal Transplantation from a Deceased Donor with Polycystic Kidney Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Marina M. Tabbara ◽  
Giselle Guerra ◽  
Gaetano Ciancio

Renal transplantation is the gold standard treatment for patients with end-stage renal disease (ESRD) as it demonstrates improved long-term survival compared to patients who remain on renal replacement therapy. The widening gap between the demand and supply of organs warrants the expansion of donor criteria for renal transplantation. Kidneys with multiple cysts are often rejected for transplantation. Here, we present our recent experience of a 72-year-old patient with ESRD due to a biopsy-proven diabetic nephropathy who received a deceased donor kidney with adult polycystic kidney disease (APKD). At 31-month posttransplant, he had a serum creatinine of 1.6 mg/dL. Deceased donors affected by APKD should be considered an acceptable option for successful renal transplantation in select recipients, as well as an alternative kidney source to increase the donor pool.


2019 ◽  
Vol 8 (2) ◽  
pp. 122-126
Author(s):  
Sarah Mian ◽  
Yogesh Acharya ◽  
Ranjan Dahal

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited renal disorder that impacts approximately 12 million worldwide. It is characterized by bilateral kidney enlargement and cystic growth. Hypertension (HTN) is a focal point in the management of ADPKD and is linked to a faster progression to end stage renal disease. Current novel therapies have proven to reduce the progression of renal damage. The ideal goal is to minimize risk through preventative studies and pharmacology to further increase life expectancy and quality. The purpose of this article is to highlight the importance of blood pressure management in ADPKD and review current literature to determine the most effective preventative pharmacotherapy.



1999 ◽  
pp. 22
Author(s):  
Cathy K. Naughton ◽  
Abdelhamid M. Elbahnasy ◽  
Matthew D. Dunn ◽  
Ralph V. Clayman ◽  
Arieh L. Shalhav ◽  
...  


2020 ◽  
pp. 106689692094289
Author(s):  
Kritika Krishnamurthy ◽  
Ruben Delgado ◽  
Jyotsna Kochiyil ◽  
Ana Maria Medina

Genetically driven tissue destruction followed by remodeling in adult polycystic kidney disease (APKD) raises the possibility of malignant transformation. Renal cell carcinoma (RCC) associated with APKD has been frequently reported in the literature; however, only a few cases of nonepithelial neoplasms arising in APKD have been described so far. Histiocytic sarcoma (HS) is a lymphohematopoietic malignant neoplasm that accounts for less than 1% of hematologic malignancies. In this article, we describe a case of primary HS occurring in a 61-year-old man with end-stage renal disease secondary to APKD. This is the first reported case of primary HS in the setting of APKD. The aberrant h-caldesmon expression seen in this case is another novel finding that has previously not been described. This case highlights the importance of morphology in guiding diagnostic workup and reiterates the necessity of maintaining a high index of suspicion for neoplastic entities in APKD.







2021 ◽  
Vol 22 (12) ◽  
pp. 6523
Author(s):  
Adrian Cordido ◽  
Marta Vizoso-Gonzalez ◽  
Miguel A. Garcia-Gonzalez

Autosomal recessive polycystic kidney disease (ARPKD) is a rare disorder and one of the most severe forms of polycystic kidney disease, leading to end-stage renal disease (ESRD) in childhood. PKHD1 is the gene that is responsible for the vast majority of ARPKD. However, some cases have been related to a new gene that was recently identified (DZIP1L gene), as well as several ciliary genes that can mimic a ARPKD-like phenotypic spectrum. In addition, a number of molecular pathways involved in the ARPKD pathogenesis and progression were elucidated using cellular and animal models. However, the function of the ARPKD proteins and the molecular mechanism of the disease currently remain incompletely understood. Here, we review the clinics, treatment, genetics, and molecular basis of ARPKD, highlighting the most recent findings in the field.



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