Clinical Characteristics of Rapid Progression in Asia-Pacific Patients With ADPKD

Oh, Yun Kyu; Ryu, Hyunjin; Ahn, Curie; Park, Hayne C.; Ma, Yiyi; Xu, Dechao; Ecder, Tevfik; Kao, Tze-Wah; Huang, Jenq‐Wen; Rangan, Gopala K.

doi:10.1016/j.ekir.2023.06.018

Cited by 2 publications

(3 citation statements)

References 31 publications

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“…In patients with ADPKD, eGFR is normal due to nephron compensation, until it begins to decrease during disease progression [4]. Treatment decisions in patients with a high HtTKV who are considered for tolvaptan induction should take into account the decline in renal function after nephron compensation is no longer effective [20,23,24].…”

Section: Discussionmentioning

confidence: 99%

“…For patients with a baseline ∆eGFR/year of ≥0 (∆eGFR unchanged or improving), it is likely that tolvaptan treatment was initiated during this 'nephron compensation period' and that patients entered a period of renal decline during the 6 years of treatment, resulting in a pre-and post-∆eGFR/year comparison that may have given the impression of no treatment effect [20,23,24]. Observing the change in ∆eGFR/year alone suggests that these patients were started on tolvaptan too early.…”

Section: Discussionmentioning

confidence: 99%

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Long-Term Effects of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease: Predictors of Treatment Response and Safety over 6 Years of Continuous Therapy

Yamazaki,

Kawano,

Miyoshi

et al. 2024

IJMS

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Tolvaptan, an oral vasopressin V2 receptor antagonist, reduces renal volume expansion and loss of renal function in patients with autosomal dominant polycystic kidney disease (ADPKD). Data for predictive factors indicating patients more likely to benefit from long-term tolvaptan are lacking. Data were retrospectively collected from 55 patients on tolvaptan for 6 years. Changes in renal function, progression of renal dysfunction (estimated glomerular filtration rate [eGFR], 1-year change in eGFR [ΔeGFR/year]), and renal volume (total kidney volume [TKV], percentage 1-year change in TKV [ΔTKV%/year]) were evaluated at 3-years pre-tolvaptan, at baseline, and at 6 years. In 76.4% of patients, ΔeGFR/year improved at 6 years. The average 6-year ΔeGFR/year (range) minus baseline ΔeGFR/year: 3.024 (−8.77–20.58 mL/min/1.73 m2). The increase in TKV was reduced for the first 3 years. A higher BMI was associated with less of an improvement in ΔeGFR (p = 0.027), and family history was associated with more of an improvement in ΔeGFR (p = 0.044). Hypernatremia was generally mild; 3 patients had moderate-to-severe hyponatremia due to prolonged, excessive water intake in response to water diuresis—a side effect of tolvaptan. Family history of ADPKD and baseline BMI were contributing factors for ΔeGFR/year improvement on tolvaptan. Hyponatremia should be monitored with long-term tolvaptan administration.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Long-Term Effects of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease: Predictors of Treatment Response and Safety over 6 Years of Continuous Therapy

Yamazaki,

Kawano,

Miyoshi

et al. 2024

IJMS

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“…Since the development of the Mayo clinic imaging classification, there is increasing interest in characterizing patients with ADPKD in Asia. 13 There is also increased focus on ADPKD research in China, and a specific clinical practice guideline for ADPKD in China has in fact been recently updated. 10 , 14 Similar to the study by Chen et al , 10 our study aims to provide a detailed analysis of the clinical, biochemical, and imaging features of a large prospective cohort of patients with ADPKD in Asia.…”

Section: Discussionmentioning

confidence: 99%

Clinical Characteristics and Kidney Outcomes in Chinese Patients with Autosomal Dominant Polycystic Kidney Disease

Fung,

Szeto,

Chow

et al. 2024

Kidney360

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Background The management of autosomal dominant polycystic kidney disease (ADPKD) remains challenging with variable and uncertain genotype-phenotype correlations. The Mayo clinic imaging classification allows a more accurate risk stratification but is limited by the atypical imaging patterns. We aim to assess the clinical characteristics and the morphology of the cystic kidneys in a cohort of Chinese patients with ADPKD. Method Ninety-eight patients with ADPKD were recruited prospectively from August 2019 to December 2020 in Prince of Wales Hospital, Hong Kong. They were subsequently followed up every 6 months for a minimum of2 years. We reviewed the clinical characteristics and MRI imaging patterns at baseline and the kidney outcome at the end of the follow-up. Atypical imaging patterns included unilateral; segmental; asymmetric; lopsided and bilateral atrophy as defined by the Mayo Imaging Classification. Result Mean age was 51.5 ± 14.3 years old and the mean eGFR 68.7 ± 27.5 ml/min per 1.73 m2. The ninety-eight patients included 36 males:62 females. Seventy-six patients (77.6%) had a family history. Seventeen of the 98 (17.3%) patients had atypical imaging patterns. Compared to typical cases, atypical cases were older at the time of diagnosis (49.5 ± 16.0 vs 33.0 ± 13.0 years, p<0.001), at the time of starting antihypertensive medications (52.4 ± 14.8 vs 39.7 ± 11.0 years, p=0.001) and less likely to have a positive family history (58.8% vs 81.5%, p=0.042). Patients with atypical patterns showed a lower eGFR decline as compared to those with the typical pattern (-0.86 ± 4.34 vs -3.44 ± 4.07 ml/min per 1.73m2/year, p=0.022). Conclusion In this cohort of Chinese patients with ADPKD, an atypical imaging pattern was observed in 17% of the cases, associated with later presentation and a milder disease course. Future genotyping studies will help to define the genetic architecture and the basis for the phenotypic spectrum in Chinese ADPKD patients.

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Clinical Characteristics of Rapid Progression in Asia-Pacific Patients With ADPKD

Cited by 2 publications

References 31 publications

Long-Term Effects of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease: Predictors of Treatment Response and Safety over 6 Years of Continuous Therapy

Long-Term Effects of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease: Predictors of Treatment Response and Safety over 6 Years of Continuous Therapy

Clinical Characteristics and Kidney Outcomes in Chinese Patients with Autosomal Dominant Polycystic Kidney Disease

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