Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease

Hogan, Marie C.; Simmons, Kathryn; Ullman, Lawrence; Gondal, Maryam; Dahl, Neera K.

doi:10.34067/kid.0000000000000296

Cited by 6 publications

(4 citation statements)

References 96 publications

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“…Secondary end points included kidney pain and pain interference with quality of life. 22 Serum copeptin levels were measured as a marker of vasopressin activity because copeptin and vasopressin are secreted in equimolar amounts. 23 eGFR, spot, and 24-hour Uosm were also measured ( Supplemental Table 1 ).…”

Section: Methodsmentioning

confidence: 99%

Feasibility of Water Therapy for Slowing Autosomal Dominant Polycystic Kidney Disease Progression

Dev,

Zhu,

Barash

et al. 2024

Kidney360

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Background In animal models of ADPKD, high water intake (HWI) decreases vasopressin secretion and slows disease progression, but the efficacy of HWI in human ADPKD is uncertain. Methods This exploratory, prospective, crossover study of ADPKD subjects (n=7) evaluated the hypothesis that HWI slows the rate of increase in height-adjusted total kidney volume (ht-TKV; a biomarker for ADPKD progression) and reduces pain. Subjects at high risk of ADPKD progression (i.e., Mayo Imaging Classifications 1C/1D) were evaluated during 6 months of usual water intake (UWI), followed by 12 months of HWI calculated to reduce urine osmolality (Uosm) to < 285 mOsm/kg. Measurements of Uosm, serum copeptin (secreted in equimolar amounts with vasopressin), MRI measurements of htTKV, and pain survey responses were compared between HWI and UWI. Results During HWI, mean 24-hour Uosm decreased compared to UWI (428 [398-432] mOsm/kg vs. 209 [190-223] mOsm/kg; p=0.01), indicating adherence to the protocol. Decreases during HWI also occurred in levels of serum copeptin (5.8±2.0 pmol/L to 4.2±1.6 pmol/L; p=0.03), annualized rate of increase in ht-TKV (6.8% [5.9 – 8.5] to 4.4% [3.0 – 5.0]; p<0.02), pain occurrence and pain interference during sleep (p<0.01). HWI was well tolerated. Conclusions HWI in patients at risk of rapid progression of ADPKD slowed the rate of ht-TKV growth and reduced pain. This suggests that suppressing vasopressin levels by HWI provides an effective non-pharmacologic treatment of ADPKD.

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Section: Methodsmentioning

confidence: 99%

Feasibility of Water Therapy for Slowing Autosomal Dominant Polycystic Kidney Disease Progression

Dev,

Zhu,

Barash

et al. 2024

Kidney360

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“…Colonic diverticula, which are small pouches that develop in the colon, as well as in the abdominal wall and abdominal hernias, have been documented to occur more frequently in patients with ADPKD [ 96 ].…”

Section: Extrarenal Cystic Manifestationsmentioning

confidence: 99%

Autosomal Dominant Polycystic Kidney Disease: Extrarenal Involvement

Righini,

Mancini,

Busutti

et al. 2024

IJMS

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Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder, but kidneys are not the only organs involved in this systemic disorder. Individuals with the condition may display additional manifestations beyond the renal system, involving the liver, pancreas, and brain in the context of cystic manifestations, while involving the vascular system, gastrointestinal tract, bones, and cardiac valves in the context of non-cystic manifestations. Despite kidney involvement remaining the main feature of the disease, thanks to longer survival, early diagnosis, and better management of kidney-related problems, a new wave of complications must be faced by clinicians who treated patients with ADPKD. Involvement of the liver represents the most prevalent extrarenal manifestation and has growing importance in the symptom burden and quality of life. Vascular abnormalities are a key factor for patients’ life expectancy and there is still debate whether to screen or not to screen all patients. Arterial hypertension is often the earliest onset symptom among ADPKD patients, leading to frequent cardiovascular complications. Although cardiac valvular abnormalities are a frequent complication, they rarely lead to relevant problems in the clinical history of polycystic patients. One of the newest relevant aspects concerns bone disorders that can exert a considerable influence on the clinical course of these patients. This review aims to provide the “state of the art” among the extrarenal manifestation of ADPKD.

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“…A partial hepatectomy and liver transplantation are options when there is no dominant cyst suitable for aspiration, but the enlarged liver impairs the quality of life. Thus, a comprehensive radiological report that includes the size and location of the largest cyst, spatial cyst distribution, complex cyst presence, and an assessment of the liver parenchymal reserve becomes instrumental in guiding therapeutic decisions [11,42,43].…”

Section: Liver Volume and Liver Cyst Fractionmentioning

confidence: 99%

“…ADPKD has numerous extrarenal manifestations that contribute valuable prognostic data and aid in disease monitoring [11,12]. These include cardiovascular complications, fluid accumulation, and mass effects due to enlargement of the kidney(s) and liver, all detectable on magnetic resonance imaging (MRI) [13].…”

Section: Introductionmentioning

confidence: 99%

A Primer for Utilizing Deep Learning and Abdominal MRI Imaging Features to Monitor Autosomal Dominant Polycystic Kidney Disease Progression

Zhu,

He,

Blumenfeld

et al. 2024

Biomedicines

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Abdominal imaging of autosomal dominant polycystic kidney disease (ADPKD) has historically focused on detecting complications such as cyst rupture, cyst infection, obstructing renal calculi, and pyelonephritis; discriminating complex cysts from renal cell carcinoma; and identifying sources of abdominal pain. Many imaging features of ADPKD are incompletely evaluated or not deemed to be clinically significant, and because of this, treatment options are limited. However, total kidney volume (TKV) measurement has become important for assessing the risk of disease progression (i.e., Mayo Imaging Classification) and predicting tolvaptan treatment’s efficacy. Deep learning for segmenting the kidneys has improved these measurements’ speed, accuracy, and reproducibility. Deep learning models can also segment other organs and tissues, extracting additional biomarkers to characterize the extent to which extrarenal manifestations complicate ADPKD. In this concept paper, we demonstrate how deep learning may be applied to measure the TKV and how it can be extended to measure additional features of this disease.

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Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease

Cited by 6 publications

References 96 publications

Feasibility of Water Therapy for Slowing Autosomal Dominant Polycystic Kidney Disease Progression

Feasibility of Water Therapy for Slowing Autosomal Dominant Polycystic Kidney Disease Progression

Autosomal Dominant Polycystic Kidney Disease: Extrarenal Involvement

A Primer for Utilizing Deep Learning and Abdominal MRI Imaging Features to Monitor Autosomal Dominant Polycystic Kidney Disease Progression

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