Index cases of intracranial aneurysms in autosomal dominant polycystic kidney disease: longitudinal experience from a single renal transplantation centre DOI Creative Commons
Joel Ern Zher Chan,

Kate S. Olakkengil,

Shantanu Bhattacharjya

и другие.

ANZ Journal of Surgery, Год журнала: 2025, Номер unknown

Опубликована: Апрель 22, 2025

Abstract Background The prevalence of intracranial aneurysms (ICAs) is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than the general population. This extrarenal manifestation carries significant mortality and morbidity risks. Our study aims to estimate of, characterize ICA(s) among ADPKD cohort South Australia's statewide transplantation centre. Methods A retrospective review prospectively collected data was conducted for all who underwent nephrectomy/ies between 1 January 1995 31 December 2021. Incidences neuroimaging radiologically diagnosed ICAs, presence risk factors including family history are compared those an alternate primary nephrological diagnosis (control). Descriptive statistics a narrative cases presented. Results Seven had ICAs (14.3% patients, 23.3% neuroimaging). Six did not meet current criteria ICA screening, five no known ICA. Three suffered aneurysmal rupture (mean age 36). Two these involved smaller ICAs. Cases demonstrated detectable vascular changes on early neuroimaging, hypoplastic anatomical variants, growth, de novo formation, association other abnormalities. Conclusion Early detection pre‐aneurysmal should be prioritized ADPKD. Although limited by small cohort, our findings consistent previous literature suggesting ruptures occur earlier aneurysms. Further investigation required into how behave. Nonetheless, it vital that centres coordinate multidisciplinary, patient‐centred approach surveillance, management patients.

Язык: Английский

KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary DOI Creative Commons
Vicente E. Torres, Curie Ahn, Thijs R.M. Barten

и другие.

Kidney International, Год журнала: 2025, Номер 107(2), С. 234 - 254

Опубликована: Янв. 21, 2025

Язык: Английский

Процитировано

5
Autosomal Dominant Polycystic Kidney Disease DOI
Fouad T. Chebib, Christian Hanna, Peter C. Harris

и другие.

JAMA, Год журнала: 2025, Номер unknown

Опубликована: Март 24, 2025

Importance Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive development of cysts and the most common inherited disorder worldwide. ADPKD accounts for 5% to 10% failure in US Europe, its prevalence 9.3 per 10 000 individuals. Observations typically diagnosed individuals aged 27 42 years primarily caused pathogenic variants PKD1 (78%) or PKD2 (15%) genes. Most persons with have an affected parent, but de novo suggested 25% families. More than 90% patients older 35 hepatic cysts, which may cause abdominal discomfort occasionally require medical surgical intervention. Hypertension affects 70% 80% ADPKD, approximately 9% 14% develop intracranial aneurysms, a rupture rate 0.57 1000 patient-years. Approximately 50% replacement therapy 62 age. The severity can be quantified using Mayo Imaging Classification (MIC), stratifies based on total volume adjusted height age ranges from 1A 1E. Patients MIC 1C 1E larger kidneys because more rapid growth (6%-10% year) compared those 1B (1%-5% earlier progression therapy, occurs at mean 58.4 1C, 52.5 1D, 43.4 Optimal management includes systolic blood pressure lower 120 mm Hg patients, 110/75 who estimated glomerular filtration (eGFR) greater 60 mL/min/1.73 m 2 are younger 50 years, dietary sodium restriction (<2000 mg/d), weight management, adequate hydration (>2.5 L daily). vasopressin type receptor antagonist tolvaptan reduces annual eGFR decline 0.98 1.27 indicated 3 year slow delay onset failure. Conclusion genetic worldwide cysts. hypertension liver aneurysms. First-line treatment control, hydration. Tolvaptan high risk

Язык: Английский

Процитировано

1
Long-term tolvaptan therapy in a case of very early-onset polycystic kidney disease DOI

Vaiva Joneliūnaitė,

Astrid Godron-Dubrasquet, Lise Allard

и другие.

Pediatric Nephrology, Год журнала: 2025, Номер unknown

Опубликована: Март 1, 2025

Язык: Английский

Процитировано

0
Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidney disease in high- and middle-income countries. DOI
Charlotte Gimpel, Steffen Fieuws, Jonas Hofstetter

и другие.

Kidney International, Год журнала: 2025, Номер unknown

Опубликована: Март 1, 2025

Язык: Английский

Процитировано

0
Index cases of intracranial aneurysms in autosomal dominant polycystic kidney disease: longitudinal experience from a single renal transplantation centre DOI Creative Commons
Joel Ern Zher Chan,

Kate S. Olakkengil,

Shantanu Bhattacharjya

и другие.

ANZ Journal of Surgery, Год журнала: 2025, Номер unknown

Опубликована: Апрель 22, 2025

Abstract Background The prevalence of intracranial aneurysms (ICAs) is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than the general population. This extrarenal manifestation carries significant mortality and morbidity risks. Our study aims to estimate of, characterize ICA(s) among ADPKD cohort South Australia's statewide transplantation centre. Methods A retrospective review prospectively collected data was conducted for all who underwent nephrectomy/ies between 1 January 1995 31 December 2021. Incidences neuroimaging radiologically diagnosed ICAs, presence risk factors including family history are compared those an alternate primary nephrological diagnosis (control). Descriptive statistics a narrative cases presented. Results Seven had ICAs (14.3% patients, 23.3% neuroimaging). Six did not meet current criteria ICA screening, five no known ICA. Three suffered aneurysmal rupture (mean age 36). Two these involved smaller ICAs. Cases demonstrated detectable vascular changes on early neuroimaging, hypoplastic anatomical variants, growth, de novo formation, association other abnormalities. Conclusion Early detection pre‐aneurysmal should be prioritized ADPKD. Although limited by small cohort, our findings consistent previous literature suggesting ruptures occur earlier aneurysms. Further investigation required into how behave. Nonetheless, it vital that centres coordinate multidisciplinary, patient‐centred approach surveillance, management patients.

Язык: Английский

Процитировано

0