Although the presence of renal cysts has been reported to be associated with aortic aneurysm or dissection by imaging studies, an autopsy study has not been performed. Therefore, in our institute, recent consecutive adult autopsy cases (n=108, 64 males and 44 females) were reviewed. The circumferences and atherosclerosis ratios of both thoracic and abdominal aorta were individually measured and graded. The number of renal cysts was scored and graded. Age of subjects along with histories of smoking, hypertension, and diabetes mellitus were confirmed. Multiple linear regression analyses demonstrated that severity of atherosclerosis and the number of renal cysts were correlated with thoracic aortic circumference, while only the number of renal cysts was correlated with abdominal aortic circumference (p<0.05), which was more predominant in female subjects (p<0.05). Microscopically, significantly more dilated renal tubules (by Student's t-test, p<0.05) along with decreased stainability of basement membrane by Periodic acid-Schiff staining and immunostaining of type IV collagen were noted in background renal tissues in cases with numerous renal cysts than in age- and sex-matched controls without renal cysts (n=10 vs. 10). The present study suggests that a syndrome that affects both aorta and renal tubules may exist.
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http://dx.doi.org/10.1016/j.prp.2013.04.012 | DOI Listing |
J Pak Med Assoc
January 2025
Department of Urology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
Objective: To assess the incidence of post-transplant lymphocele, identify risk factors, and analyse the effectiveness and outcomes of different management approaches.
Methods: The prospective, descriptive study was conducted at the Department of Renal Transplant, Sindh Institute of Urology and Transplantation, Karachi, from February 25, 2021, to December 25, 2022, and comprised of 300 transplant. Ultrasound was performed in all patients presenting with symptomatology attributed to lymphocele.
Eur Clin Respir J
January 2025
Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.
Background: Birt-Hogg-Dubé syndrome (BHD), a rare genetic disease characterized by multiple pulmonary cysts, can lead to spontaneous pneumothorax, cutaneous hamartomas, renal cysts, and renal cell cancer. The overall aim of this study was to assess clinical characteristics of patients with BHD-emphasizing on trends in pulmonary function patterns.
Methods: By use of data from electronic patient journals, we conducted a retrospective cohort study on clinical characteristics and pulmonary function tests (PFT) from patients with BHD, who were clinically followed-up in a Danish tertiary referral center for rare and interstitial lung diseases.
Intern Med
January 2025
Department of Nephrology, The University of Osaka Graduate School of Medicine, Japan.
We encountered a family with hereditary renal failure, renal medullary cysts, pancreatic hypoplasia, hypomagnesemia, liver enzyme abnormalities, and diabetes mellitus (DM). We identified a novel heterozygous variant of HNF1B (NM_000458.4:c.
View Article and Find Full Text PDFExp Clin Transplant
December 2024
>From the Department of Nephrology, Toho University Faculty of Medicine, Tokyo, Japan; and the Department of Urology, Ryukyu University, Okinawa, Japan.
Objectives: This study aimed to determine interventions and identify risk factors for lymphocele formation following kidney transplant.
Materials And Methods: We conducted a retrospective review of 296 adult kidney transplants from 2010 to 2022 to investigate postoperative lymphocele formation. We divided patients into lymphocele cases and nonlymphocele cases.
Genetic modifiers are believed to play an important role in the onset and severity of polycystic kidney disease (PKD), but identifying these modifiers has been challenging due to the lack of effective methodologies. In this study, we investigated zebrafish mutants of , a newly identified ADPKD gene, and observed phenotypes similar to those seen in mammalian models, including kidney cysts and bone defects. Using efficient microhomology-mediated end joining (MMEJ)-based genome editing technology, we demonstrated that CRISPRants recapitulate mutant phenotypes while bypassing the early lethality of the mutants to allow for renal cyst analysis in adult fish.
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