Introduction
介绍

1.1 Overview of Autosomal Dominant Polycystic Kidney Disease (ADPKD)
1.1 常染色体显性遗传性多囊肾病 （ADPKD） 概述

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a common dominant genetic disorder and the fourth leading cause of kidney failure, with 5% of kidney failure cases attributed to ADPKD progression. Global incidence rates vary significantly, ranging from 1 in 1,000 to 1 in 400. In the UK, the incidence is approximately 3.9 per 10,000, meaning that 1 in every 2,500 people has ADPKD, making it a relatively prevalent rare kidney disease.
常染色体显性遗传性多囊肾病 （ADPKD） 是一种常见的显性遗传病，也是肾衰竭的第四大原因，5% 的肾衰竭病例归因于 ADPKD 进展。全球发病率差异很大，从 1/1,000 到 1/400 不等。在英国，发病率约为每 10,000 人 3.9 人，这意味着每 2,500 人中就有 1 人患有 ADPKD，使其成为一种相对普遍的罕见肾脏疾病。

Etiology
病因学

ADPKD is caused by genetic mutations, primarily in the PKD1 and PKD2 genes, which account for approximately 60% and 15% of cases, respectively. There are other unknown gene mutations that contribute to ADPKD. The type of gene mutation can influence the severity of ADPKD. Patients with PKD1 mutations tend to have more severe symptoms, develop more cysts, and have larger kidney volumes, leading to a faster progression of the disease compared to those with PKD2 mutations.
ADPKD 是由基因突变引起的，主要发生在 PKD1 和 PKD2 基因中，分别约占病例的 60% 和 15%。还有其他未知的基因突变会导致 ADPKD。基因突变的类型会影响 ADPKD 的严重程度。与 PKD2 突变患者相比，PKD1 突变的患者往往症状更严重，出现更多的囊肿，肾脏体积更大，导致疾病进展更快。

Due to the hereditary nature of Autosomal Dominant Polycystic Kidney Disease (ADPKD), approximately 85% of patients have a family history and often carry similar genetic mutations as their relatives. Studies indicate that whether ADPKD patients progress to end-stage renal disease (ESRD), as well as the age at which they reach this stage, tends to be similar among family members. However, influenced by modifier genes, environmental factors, and demographic factors such as age, the severity of the disease may still vary significantly even among ADPKD patients within the same family.
由于常染色体显性遗传性多囊肾病 （ADPKD） 的遗传性，大约 85% 的患者有家族史，并且通常携带与其亲属相似的基因突变。研究表明，ADPKD 患者是否进展为终末期肾病 （ESRD），以及他们达到这一阶段的年龄，在家庭成员之间往往相似。然而，受修饰基因、环境因素和人口统计学因素（如年龄）的影响，即使在同一家庭内的 ADPKD 患者中，疾病的严重程度也可能仍有很大差异。

Pathology
病理学

In ADPKD patients, multiple cysts form on the epithelium of renal tubules in both kidneys. The number and size of these cysts increase over time, with varying growth rates among individual cysts. The expanding cysts enlarge the kidneys and gradually compress the renal tubules, blood vessels, and lymphatic vessels, causing displacement and obstruction. As the renal structure becomes increasingly compressed, cells within the kidney gradually undergo apoptosis, atrophy, and fibrosis, leading to the destruction of kidney function. Eventually, the kidneys lose their normal vasculature, fibrotic bands form between cysts, glomerulosclerosis occurs, and the renal tubules atrophy.
在 ADPKD 患者中，双肾肾小管上皮上形成多个囊肿。这些囊肿的数量和大小随着时间的推移而增加，单个囊肿的生长速度不同。扩大的囊肿使肾脏增大，并逐渐压迫肾小管、血管和淋巴管，导致移位和阻塞。随着肾脏结构的日益压缩，肾脏内的细胞逐渐凋亡、萎缩和纤维化，导致肾功能破坏。最终，肾脏失去正常的脉管系统，囊肿之间形成纤维化带，发生肾小球硬化，肾小管萎缩。

Renal cysts can begin to form while the fetus is in the womb and develop throughout life. Before the age of 30, ADPKD patients typically lose functioning nephron units, but kidney function remains normal. However, after losing about 60% of nephron units, kidney function begins to decline, leading to a gradual decline over several decades and potentially progressing to end-stage renal disease (ESRD).
肾囊肿可以在胎儿在子宫内时开始形成，并在一生中发展。在 30 岁之前，ADPKD 患者通常会失去功能性肾单位，但肾功能保持正常。然而，在失去约 60% 的肾单位后，肾功能开始下降，导致几十年内逐渐下降，并可能发展为终末期肾病 （ESRD）。

Diagnosis
诊断

ADPKD can be diagnosed through imaging or genetic testing. Common diagnostic methods include ultrasound, MRI, and CT, with MRI and CT providing greater specificity than ultrasound. However, ultrasound is the preferred method due to its status as the gold standard, ease of access, and lower cost, as recommended by KDIGO and other organizations. Although genetic testing is an option, unknown gene mutations may result in missed diagnoses, reducing its sensitivity. Furthermore, despite declining costs, genetic testing remains more expensive than imaging, limiting its broader use. In patients with a family history of ADPKD, the presence of enlarged bilateral kidneys with multiple cysts confirms the diagnosis.
ADPKD 可以通过影像学或基因检测来诊断。常见的诊断方法包括超声、MRI 和 CT，其中 MRI 和 CT 的特异性高于超声。然而，正如 KDIGO 和其他组织所推荐的那样，超声是首选方法，因为它是黄金标准、易于访问和成本较低。虽然基因检测是一种选择，但未知基因突变可能会导致漏诊，从而降低其敏感性。此外，尽管成本下降，但基因检测仍然比成像更昂贵，限制了其更广泛的应用。在有 ADPKD 家族史的患者中，双侧肾脏肿大伴多个囊肿可确诊。

Disease Severity
疾病严重程度

The progression of ADPKD varies significantly among patients. Some may reach diagnostic criteria only at the age of 60-70 without progressing to ESRD, while others can be diagnosed as early as in utero. Generally, the onset and diagnosis of ADPKD occur in adulthood, with multiple cysts typically appearing around the age of 50. half of the patients develop ESRD around the age of 60, with the likelihood increasing with age.
ADPKD 的进展因患者而异。有些人可能在 60-70 岁时才达到诊断标准，而不会发展为 ESRD，而另一些人可以在子宫内早点诊断出来。一般来说，ADPKD 的发病和诊断发生在成年期，多个囊肿通常在 50 岁左右出现。一半的患者在 60 岁左右发展为 ESRD，可能性随着年龄的增长而增加。

Disease Progression Stages
疾病进展阶段

The progression of ADPKD can be classified based on chronic kidney disease (CKD) staging criteria, determined by the degree of kidney function impairment. Kidney function impairment is quantified using glomerular filtration rate (GFR) into five stages: G1-G5 (with G3 further divided into G3a and G3b).
ADPKD 的进展可以根据慢性肾脏病 （CKD） 分期标准进行分类，该分期标准由肾功能损害的程度决定。使用肾小球滤过率 （GFR） 将肾功能损害量化为五个阶段： G1-G5 （G3 进一步分为 G3a 和 G3b）。

In CKD stage 1, GFR ≥ 90 ml/min/1.73 m², indicating normal renal function but with evidence of kidney damage (such as proteinuria, hematuria, or imaging abnormalities).
在 CKD 1 期，GFR ≥ 90 ml/min/1.73 m²，表明肾功能正常，但有肾损伤的证据（如蛋白尿、血尿或影像学异常）。

In stage 2, GFR is 60-89 ml/min/1.73 m².
在第 2 阶段，GFR 为 60-89 ml/min/1.73 m²。

Stage 3 is subdivided into G3a (GFR 45-59 ml/min/1.73 m²) and G3b (GFR 30-44 ml/min/1.73 m²).
第 3 阶段细分为 G3a （GFR 45-59 ml/min/1.73 m²） 和 G3b （GFR 30-44 ml/min/1.73 m²）。

In stage 4, GFR declines to 15-29 ml/min/1.73 m².
在第 4 阶段，GFR 下降到 15-29 ml/min/1.73 m²。

In the G5 stage, the GFR falls below 15 mL/min, indicating the progression to ESRD.
在 G5 阶段，GFR 降至 15 mL/min 以下，表明进展为 ESRD。

The rate of eGFR decline was used as the primary endpoint in the Phase 3 trials of Tolvaptan, serving as a key indicator for assessing the effectiveness of disease intervention. Other methods to assess disease progression include blood pressure and proteinuria since severe kidney damage can lead to hypertension and proteinuria. While eGFR is used to assess kidney function, it is generally not employed to evaluate early intervention effects, as the development of kidney cysts typically does not affect eGFR until later stages.
在 Tolvaptan 的 3 期试验中，eGFR 下降率被用作主要终点，作为评估疾病干预有效性的关键指标。其他评估疾病进展的方法包括血压和蛋白尿，因为严重的肾损伤可导致高血压和蛋白尿。虽然 eGFR 用于评估肾功能，但通常不用于评估早期干预效果，因为肾囊肿的发展通常直到晚期才影响 eGFR。

Treatment
治疗

Currently, there are very few drugs available to treat ADPKD. Tolvaptan is the first drug approved for ADPKD treatment in multiple countries, but its use is limited to though its use is limited to patients with rapidly progressing disease. Most ADPKD patients rely on early diagnosis and management to slow disease progression and address related complications. When the disease progresses to ESRD, dialysis or kidney transplantation becomes the final treatment option.
目前，可用于治疗 ADPKD 的药物很少。托伐普坦是第一个在多个国家被批准用于治疗 ADPKD 的药物，但其使用仅限于疾病快速进展的患者。大多数 ADPKD 患者依靠早期诊断和管理来减缓疾病进展并解决相关并发症。当疾病发展为 ESRD 时，透析或肾移植成为最终的治疗选择。

1.2 Health-Related Quality of Life (HRQOL)
1.2 健康相关生活质量 （HRQOL）

Quality of life (QoL) is a measure of an individual's overall satisfaction and well-being across various aspects of life. In the management of ADPKD, QoL, as a factor closely related to chronic progressive diseases, should be taken into consideration. Health-related quality of life (HRQoL) is a key dimension of QoL, focusing specifically on assessing the impact of health issues, particularly the limitations imposed by long-term diseases on physical and psychological well-being. As a critical indicator of health and well-being, HRQoL plays a significant role in policy-making and resource allocation.
生活质量 （QoL） 是衡量个人在生活各个方面的整体满意度和幸福感的指标。在 ADPKD 的管理中，应考虑 QoL 作为与慢性进展性疾病密切相关的因素。与健康相关的生活质量 （HRQoL） 是 QoL 的一个关键维度，特别关注评估健康问题的影响，特别是长期疾病对身心健康的限制。作为健康和福祉的关键指标，HRQoL 在政策制定和资源分配中发挥着重要作用。

1.3 Factors Affecting HRQOL in ADPKD Patients
1.3 影响 ADPKD 患者 HRQOL 的因素

The HRQOL of ADPKD patients is influenced by its complications. In the early stages of ADPKD:
ADPKD 患者的 HRQOL 受其并发症的影响。在 ADPKD 的早期阶段：

In the early stages of ADPKD, impaired kidney function leads to a reduced ability to concentrate urine, resulting in polyuria and polydipsia, which may negatively impact the patient's urinary-related quality of life. As the cysts enlarge, they can cause obstruction of the urinary tract, leading to complications such as urinary tract infections, cyst infections, urinary stones, and gross hematuria, further contributing to pain.
在 ADPKD 的早期阶段，肾功能受损会导致尿液浓缩能力下降，导致多尿和烦渴，这可能会对患者的泌尿相关生活质量产生负面影响。随着囊肿扩大，它们会导致尿路梗阻，导致尿路感染、囊肿感染、尿路结石和肉眼血尿等并发症，进一步加剧疼痛。

During the progressive decline of kidney function in ADPKD patients, renal functions such as filtering blood to produce urine, regulating blood pressure, maintaining electrolyte and acid-base balance, controlling erythropoiesis, and supporting bone health are critical for overall health. As kidney function deteriorates, the risk of consequences of the disease process such as anemia, secondary hyperparathyroidism, metabolic bone disease, and malnutrition significantly increases. The decline in kidney function in ADPKD patients is measured in stages using the common chronic kidney disease (CKD) grading system. Throughout the progression of CKD, factors that commonly impact quality of life include psychological issues like anxiety and depression, along with physical symptoms such as pain, fatigue, and limited mobility, which may also occur during the decline in renal function in ADPKD patients.
在 ADPKD 患者肾功能进行性下降期间，过滤血液产生尿液、调节血压、维持电解质和酸碱平衡、控制红细胞生成和支持骨骼健康等肾功能对整体健康至关重要。随着肾功能恶化，贫血、继发性甲状旁腺功能亢进症、代谢性骨病和营养不良等疾病过程后果的风险显着增加。ADPKD 患者肾功能的下降是使用常见的慢性肾脏病 （CKD） 分级系统分阶段测量的。在 CKD 的整个进展过程中，通常影响生活质量的因素包括焦虑和抑郁等心理问题，以及疼痛、疲劳和行动不便等身体症状，这些症状也可能发生在 ADPKD 患者肾功能下降期间。

End-stage renal disease (ESRD): Treatment options for ESRD include dialysis (hemodialysis or peritoneal dialysis), kidney transplantation, and conservative care. Most ADPKD patients undergo hemodialysis rather than peritoneal dialysis due to insufficient abdominal space for fluid. However, dialysis can cause severe treatment-related symptoms, which significantly impair health-related quality of life.
终末期肾病 （ESRD）：ESRD 的治疗选择包括透析（血液透析或腹膜透析）、肾移植和保守治疗。由于腹部空间不足，大多数 ADPKD 患者接受血液透析而不是腹膜透析。然而，透析会导致严重的治疗相关症状，从而严重损害与健康相关的生活质量。

The gene variants that cause ADPKD cyst formation may also lead to abnormal cyst development in other parts of the body. Common extrarenal cyst locations include the liver, arachnoid membrane, spleen, pancreas, and seminal vesicles. Liver cysts are the most common extrarenal manifestation of ADPKD, occurring in over 95% of ADPKD patients aged over 70. Although this complication usually does not affect liver function, it can cause pain and reduce physical activity. Additionally, the increased liver volume may lead to restrictive lung disease, causing shortness of breath, and early satiety may result in malnutrition.
导致 ADPKD 囊肿形成的基因变异也可能导致身体其他部位的异常囊肿发育。常见的肾外囊肿部位包括肝脏、蛛网膜、脾脏、胰腺和精囊。肝囊肿是 ADPKD 最常见的肾外表现，发生在 95% 以上的 70 岁以上的 ADPKD 患者中。虽然这种并发症通常不会影响肝功能，但它会引起疼痛并减少身体活动。此外，肝脏体积增加可能导致限制性肺病，导致呼吸急促，早饱可能导致营养不良。

Abnormal gene expression can also affect blood vessels, significantly increasing the risk of intracranial aneurysms in ADPKD patients. Intracranial saccular aneurysms, the most common and severe type, occur in 5-10% of ADPKD patients. However, in those with a family history of the condition, the prevalence rises to 25%. These aneurysms carry a high risk of rupture, potentially leading to fatal brain hemorrhage, severe neurological damage, and a lasting psychological burden on the patient.
异常的基因表达也会影响血管，显着增加 ADPKD 患者患颅内动脉瘤的风险。颅内囊状动脉瘤是最常见和最严重的类型，发生在 5-10% 的 ADPKD 患者中。然而，在有该病家族史的人中，患病率上升到 25%。这些动脉瘤具有很高的破裂风险，可能导致致命的脑出血、严重的神经损伤，并给患者带来持久的心理负担。

Other Factors Affecting HRQOL
影响 HRQOL 的其他因素

Studies on the HRQOL of ADPKD patients show that the kidneys and complications significantly impair physical health, but have a relatively minor impact on mental health. One factor affecting mental health is chronic pain, which often leads to anxiety and depression. Depression is relatively common among ADPKD patients, affecting about 60% of them, severely impacting their quality of life.
对 ADPKD 患者 HRQOL 的研究表明，肾脏和并发症会显着损害身体健康，但对心理健康的影响相对较小。影响心理健康的一个因素是慢性疼痛，这通常会导致焦虑和抑郁。抑郁症在 ADPKD 患者中相对常见，影响了大约 60% 的患者，严重影响了他们的生活质量。

Pain
疼痛

ADPKD patients often exhibit pain symptoms, with 60% of patients under 40 years old experiencing pain. Compared to general pain, ADPKD-related pain is often accompanied by overall fatigue, limited physical activity, and reduced sleep quality, as well as significant psychological distress. Pain tends to be recurrent and severe, with unpredictable patterns, ranging from sudden acute pain to persistent chronic dull pain. Pain primarily occurs in the lower back (71%), abdomen (61%), head (49%), and chest (30%), but the specific source of the pain is often unclear. This specific pain significantly impacts the HRQOL of ADPKD patients, with about 30% of patients requiring regular pain medication to alleviate symptoms. However, medical professionals often regard this pain as general pain, without giving it sufficient attention. Patients commonly express a desire for more information and medical support on pain management to reduce its negative impact on their quality of life
ADPKD 患者经常表现出疼痛症状，60% 的 40 岁以下患者感到疼痛。与一般疼痛相比，ADPKD 相关疼痛通常伴有整体疲劳、体力活动受限、睡眠质量下降以及严重的心理困扰。疼痛往往是反复发作的和严重的，具有不可预测的模式，从突然的急性疼痛到持续的慢性钝痛。疼痛主要发生在下背部 （71%）、腹部 （61%）、头部 （49%） 和胸部 （30%），但疼痛的具体来源往往不清楚。这种特定的疼痛会显着影响 ADPKD 患者的 HRQOL，大约 30% 的患者需要定期服用止痛药来缓解症状。然而，医疗专业人员经常将这种疼痛视为一般疼痛，而没有给予足够的重视。患者通常表示希望获得更多关于疼痛管理的信息和医疗支持，以减少其对他们生活质量的负面影响.

Cognitive Factors
认知因素

Patients with ADPKD often experience anxiety and depression due to the uncertainty surrounding disease progression, making it difficult for them to plan for the future. This uncertainty exacerbates their physical symptoms. Moreover, the tendency of healthcare providers to downplay early symptoms and the lack of adequate information provided to patients further intensify their uncertainty about the future. As a genetic disorder, ADPKD frequently leads to feelings of guilt and resentment regarding the transmission of the disease. Even family members who are not affected by the condition may experience a sense of genetic guilt despite a negative diagnosis.
由于疾病进展的不确定性，ADPKD 患者经常会感到焦虑和抑郁，使他们难以规划未来。这种不确定性加剧了他们的身体症状。此外，医疗保健提供者倾向于淡化早期症状以及缺乏向患者提供的足够信息，进一步加剧了他们对未来的不确定性。作为一种遗传性疾病，ADPKD 经常导致对疾病传播的内疚感和怨恨。即使诊断为阴性，但即使是不受这种疾病影响的家庭成员也可能会产生遗传内疚感。

1.4 Measurement
1.4 测量

Quality of life (QoL) measurement scales are widely used and are generally divided into generic scales and scales specific to certain populations. Compared to generic scales, QoL scales tailored for ADPKD patients are more comprehensive and exhibit higher sensitivity.
生活质量 （QoL） 测量量表应用广泛，通常分为通用量表和特定人群的量表。与通用量表相比，为 ADPKD 患者量身定制的 QoL 量表更全面，灵敏度更高。

Generic Scales:
通用秤：

The SF-36 consists of 36 questions designed to measure a patient's quality of life across eight dimensions. Each dimension is scored on a scale of 0 to 100, with higher scores indicating better health status in that dimension. A value set is used to transform these dimension scores into two composite scores: the Physical Component Summary (PCS) and the Mental Component Summary (MCS). The eight dimensions include Physical Functioning, Social Functioning, Role-Physical, Role-Emotional, Vitality, Mental Health, Bodily Pain, and General Health. The Physical Component Summary (PCS) and Mental Component Summary (MCS) scores are standardized with 50 as the average for the general population. Scores above 50 indicate better health, while scores below 50 suggest worse health.
SF-36 由 36 个问题组成，旨在从 8 个维度衡量患者的生活质量。每个维度的评分范围为 0 到 100，分数越高表示该维度的健康状况越好。值集用于将这些维度分数转换为两个综合分数：物理成分摘要 （PCS） 和心理成分摘要 （MCS）。这八个维度包括身体机能、社会功能、角色-身体、角色-情感、活力、心理健康、身体疼痛和一般健康。身体成分总结 （PCS） 和心理成分总结 （MCS） 分数标准化，以 50 作为一般人群的平均分。分数高于 50 表示健康状况较好，而低于 50 分表示健康状况较差。

The SF-12v2 is a simplified version of the SF-36, using 12 questions to generate the same two composite scores: PCS and MCS. The scoring method follows the same approach, using a value set to calculate these summary scores.
SF-12v2 是 SF-36 的简化版本，使用 12 个问题生成相同的两个综合分数：PCS 和 MCS。评分方法遵循相同的方法，使用值集来计算这些汇总分数。

The EQ-5D consists of five dimensions: Mobility, Self-care, Usual Activities, Anxiety/Depression. A value set is used to calculate the EQ-5D index from the scores across these dimensions, with the index typically ranging from 0 (equivalent to death) to 1 (perfect health).
EQ-5D 由五个维度组成：行动能力、自我保健、日常活动、焦虑/抑郁。一个值集用于根据这些维度的分数计算 EQ-5D 指数，指数通常从 0（相当于死亡）到 1（完美健康）不等。

Kidney Disease-Specific Scales:
肾脏疾病特异性量表：

The Kidney Disease-Specific Scales in the KDQOL-SF 1.3 expand upon the SF-36 by exploring the specific impact of kidney disease on patients. It includes the following 20 dimensions. Higher scores indicate better health status in these specific domains.
KDQOL-SF 1.3 中的肾脏疾病特异性量表通过探索肾脏疾病对患者的具体影响，在 SF-36 的基础上进行了扩展。它包括以下 20 个维度。分数越高表示这些特定领域的健康状况越好。

ADPKD-Specific Scales:
ADPKD 特定量表：

The ADPKD-IS is used to assess the burden of ADPKD on QoL in three dimensions: physical, emotional, and fatigue. It uses a 1-5 Likert scale to measure the severity of the burden, with higher scores indicating a greater impact of the disease on health-related quality of life.
ADPKD-IS 用于从三个维度评估 ADPKD 对 QoL 的负担：身体、情绪和疲劳。它使用 1-5 李克特量表来衡量负担的严重程度，分数越高表示疾病对健康相关生活质量的影响越大。

The ADPKD-UID is used to measure urinary-related QoL in ADPKD patients across three dimensions: urinary frequency, urgency, and nocturia over the course of a week. It also uses a 1-5 Likert scale, with higher scores indicating a greater impact of the disease on urinary-related QoL
ADPKD-UID 用于测量 ADPKD 患者在一周内的尿频、尿急和夜尿三个维度的尿液相关 QoL。它还使用 1-5 李克特量表，分数越高表示疾病对泌尿相关 QoL 的影响越大

1.5 The Need for Research
1.5 研究的必要性

Studies have shown that healthcare professionals often overlook the impact of ADPKD (Autosomal Dominant Polycystic Kidney Disease) on patients' health-related quality of life (HRQoL), especially in the early stages of the disease. Healthcare providers typically do not prioritize HRQoL issues until the disease has progressed to advanced stages, nearing kidney failure. Although physicians recognize that symptoms may emerge early in the disease, they often underestimate their severity, leading to inadequate early intervention and insufficient informational support. Some patients report that ADPKD significantly affects their quality of life even in the early stages. However, when seeking treatment at this stage, they often feel frustrated and discouraged. This is largely due to healthcare providers' failure to offer effective symptom management and their limited provision of information about the disease, resulting in patients' lack of sufficient knowledge to manage their condition.
研究表明，医疗保健专业人员经常忽视 ADPKD（常染色体显性遗传性多囊肾病）对患者健康相关生活质量 （HRQoL） 的影响，尤其是在疾病的早期阶段。医疗保健提供者通常不会优先考虑 HRQoL 问题，直到疾病发展到晚期，接近肾衰竭。尽管医生认识到症状可能在疾病早期出现，但他们往往低估了症状的严重性，导致早期干预不足和信息支持不足。一些患者报告说，即使在早期阶段，ADPKD 也会显着影响他们的生活质量。然而，在这个阶段寻求治疗时，他们经常感到沮丧和气馁。这主要是由于医疗保健提供者未能提供有效的症状管理以及他们提供有关疾病的信息有限，导致患者缺乏足够的知识来管理他们的病情。

The 2014 Brussels PKD Declaration pointed out that ADPKD has a profound physical and psychological impact on patients and their families, emphasizing the importance of addressing patients' HRQoL needs to improve care quality. While improving HRQoL can help clinicians provide more patient-centered and personalized care, current ADPKD research mainly focuses on physiological indicators such as blood pressure, kidney size, and function. In contrast, there is relatively little research on the quality of life of ADPKD patients, leading to insufficient attention and understanding in this important area.
2014 年布鲁塞尔 PKD 宣言指出，ADPKD 对患者及其家人具有深远的身心影响，强调了满足患者 HRQoL 需求对提高护理质量的重要性。虽然改善 HRQoL 可以帮助临床医生提供更以患者为中心的个性化护理，但目前的 ADPKD 研究主要集中在血压、肾脏大小和功能等生理指标上。相比之下，对 ADPKD 患者生活质量的研究相对较少，导致对这一重要领域的关注和理解不足。

1.6 AIM
1.6 目标

This study will focus on the HRQoL of ADPKD patients at different stages of CKD, aiming to better understand the changes in HRQoL across the disease stages, thereby providing useful insights for developing targeted care recommendations.
本研究将重点关注 CKD 不同阶段 ADPKD 患者的 HRQoL，旨在更好地了解 HRQoL 在不同疾病阶段的变化，从而为制定有针对性的护理建议提供有用的见解。

Method
方法

2.1 Theoretical Methods
2.1 理论方法

The systematic review combined both review and synthesis to organise the obtained data. The study design follows the PRISMA guidelines
系统综述结合了综述和综合来组织获得的数据。研究设计遵循 PRISMA 指南.

2.2 Study Protocol and Registration
2.2 研究方案和注册

This study has been registered in PROSPERO, The detailed content of the registered protocol is presented in Appendix 1.
这项研究已在 PROSPERO 中注册，注册方案的详细内容见附录 1。

2.3 Information Sources and Search Strategy
2.3 信息来源和检索策略

The following electronic databases were searched: MEDLINE via Ovid (last search date: 2024.08.01), Embase via Ovid (last search date:2024.08.01 ), PsycINFO via Ovid (last search date:2024.08.01), CINAHL via Embase (last search date:2024.08.01) and Web of Science (last search date:2024.08.01)
检索了以下电子数据库：MEDLINE via Ovid （最后检索日期：2024.08.01）， Embase via Ovid （最后检索日期：2024.08.01） ， PsycINFO via Ovid （最后检索日期：2024.08.01）， CINAHL via Embase （最后检索日期：2024.08.01） 和 Web of Science （最后检索日期：2024.08.01）

XX and colleagues explored the optimal combination of databases to retrieve the most comprehensive and relevant literature through actual searches. Their study demonstrated that the combination of Embase, MEDLINE, Web of Science, and Google Scholar captures the broadest and most relevant range of literature, minimizing unnecessary time spent on literature searches. Therefore, this study will utilize this combination to gather relevant literature. Additionally, PsycINFO, which primarily includes psychological literature related to the mental health of ADPKD patients, and CINAHL, which focuses on nursing and related health literature, will also be used due to their strong relevance to the topic of this review.
XX 及其同事探索了数据库的最佳组合，以通过实际搜索检索最全面和最相关的文献。他们的研究表明，Embase、MEDLINE、Web of Science 和 Google Scholar 的组合可以捕获最广泛和最相关的文献，最大限度地减少花在文献搜索上的不必要时间。因此，本研究将利用这种组合来收集相关文献。此外，由于与本综述主题密切相关，主要包括与 ADPKD 患者心理健康相关的心理学文献的 PsycINFO 和专注于护理和相关健康文献的 CINAHL，也将被使用。

In developing the literature search strategy, we divided the keywords into two main themes: ADPKD and HRQOL. Each theme encompasses several related synonyms. The search terms were combined into strings, with truncation symbols used to ensure the results cover different spelling variants of the search terms. Spacing symbols were also employed to capture various combinations of keywords, increasing the flexibility of the search. To broaden the search scope, database-specific suffixes like “mp” were appended. The strings were connected using Boolean logic symbols (e.g., AND, OR, NOT).
在制定文献检索策略时，我们将关键词分为两个主要主题：ADPKD 和 HRQOL。每个主题都包含几个相关的同义词。搜索词被组合成字符串，并使用截断符号来确保结果涵盖搜索词的不同拼写变体。此外，还采用了间距符号来捕获各种关键字组合，从而提高了搜索的灵活性。为了扩大搜索范围，附加了特定于数据库的后缀，如“mp”。字符串使用布尔逻辑符号（例如 AND、OR、NOT）连接。

To obtain the latest research data and ensure the results are more manageable for a student project, the study limits the time range to the past ten years, specifically between 2014 and 2024. Due to translation resource limitations, the language scope for literature screening was limited to English. Additionally, the study subjects were restricted to human-only studies to increase the relevance of the search results. To enhance the reliability of the conclusions, only peer-reviewed literature was included in this study.
为了获得最新的研究数据并确保结果对学生项目更易于管理，该研究将时间范围限制在过去十年，特别是 2014 年至 2024 年之间。由于翻译资源的限制，文献筛选的语言范围仅限于英语。此外，研究对象仅限于仅限人类的研究，以提高搜索结果的相关性。为了提高结论的可靠性，本研究仅纳入了同行评议的文献。

To improve the focus of the search and reduce the inclusion of irrelevant literature, the search will be limited to the first 30 results, as Google Scholar ranks studies by relevance, ensuring that the most relevant studies appear at the top. Additionally, limiting the number of studies makes the results more manageable for analysis.
为了提高搜索的重点并减少不相关文献的包含，搜索将仅限于前 30 个结果，因为 Google Scholar 会按相关性对研究进行排名，确保最相关的研究出现在顶部。此外，限制研究的数量使结果更易于分析。

2.4 Eligibility Criteria
2.4 资格标准

Justification of eligibility criteria:
资格标准的理由：

a) Population
一）人口

Since ADPKD can affect individuals at any age, this study will not impose any age restrictions on the inclusion criteria, allowing for comprehensive data collection from both pediatric and adult patients. In stage G5 of CKD in ADPKD patients, kidney function is severely compromised, often necessitating dialysis or kidney transplantation. Therefore, this study will include patients undergoing dialysis or who have received a kidney transplant to capture data from all stages of disease progression, including the most advanced stages.
由于 ADPKD 可以影响任何年龄的个体，因此本研究不会对纳入标准施加任何年龄限制，从而允许从儿科和成人患者那里收集全面的数据。在 ADPKD 患者的 CKD G5 期，肾功能严重受损，通常需要透析或肾移植。因此，本研究将包括接受透析或接受肾移植的患者，以捕获疾病进展所有阶段的数据，包括最晚期阶段。

As a hereditary disease, ADPKD’s genetic transmission may impact not only the physical health but also the psychological well-being of the patient's relatives, including children who might inherit the condition. Additionally, the chronic nature of ADPKD means that caregiving responsibilities can significantly affect the quality of life of relatives, especially those who provide daily care. This study will include first-degree relatives, who have a higher likelihood of being affected genetically, and cohabiting relatives, who are often the primary caregivers for ADPKD patients.
作为一种遗传性疾病，ADPKD 的基因传递不仅会影响患者亲属的身体健康，还会影响患者亲属的心理健康，包括可能遗传该疾病的孩子。此外，ADPKD 的慢性性质意味着照顾责任会显着影响亲属的生活质量，尤其是那些提供日常护理的亲属。这项研究将包括一级亲属，他们受到遗传影响的可能性更高，以及同居亲属，他们通常是 ADPKD 患者的主要照顾者。

b) Stages of CKD
b） 慢性肾病的分期

To understand the quality of life of ADPKD patients at different stages of chronic kidney disease (CKD), this study will include studies that clearly define the stages of CKD, as well as those involving renal/kidney replacement therapies such as dialysis or kidney transplantation. For studies that do not clearly state the CKD stage, information provided in the text (e.g., estimated glomerular filtration rate [eGFR]) will be used to calculate and determine the CKD stage. Studies that include only specific CKD stages, even if they cover only one stage, as well as those focusing on renal/kidney replacement therapies, will meet the inclusion criteria and be included in the search results. Conversely, studies that do not define CKD stages or renal replacement therapies will be excluded.
为了了解慢性肾脏病 （CKD） 不同阶段的 ADPKD 患者的生活质量，本研究将包括明确定义 CKD 阶段的研究，以及涉及肾/肾替代疗法（如透析或肾移植）的研究。对于未明确说明 CKD 分期的研究，将使用文本中提供的信息（例如，估计肾小球滤过率 [eGFR]）来计算和确定 CKD 分期。仅包括特定 CKD 分期的研究，即使它们只涵盖一个阶段，以及那些专注于肾/肾替代疗法的研究，将符合纳入标准并被纳入检索结果。相反，未定义 CKD 分期或肾脏替代疗法的研究将被排除在外。

c) HRQoL
c） HRQoL

HRQoL data is primarily collected through patient self-reports. The scales used to assess HRQoL are divided into three categories: generic scales, kidney-specific scales, and ADPKD-specific scales. Since ADPKD-specific scales were introduced later, and given the variety of HRQoL measurement tools, it is expected that multiple assessment scales will be used in the included studies. Therefore, this study will include literature that uses at least one quantitative measurement tool to assess HRQoL to ensure the diversity of assessment tools.
HRQoL 数据主要通过患者自我报告收集。用于评估 HRQoL 的量表分为三类：通用量表、肾脏特异性量表和 ADPKD 特异性量表。由于 ADPKD 特异性量表是后来引入的，并且考虑到 HRQoL 测量工具的多样性，预计纳入的研究将使用多种评估量表。因此，本研究将包括使用至少一种定量测量工具来评估 HRQoL 的文献，以确保评估工具的多样性。

d)Study Types
d） 研究类型

The study will include RCTs that report baseline data or placebo group data on health-related quality of life (HRQoL). Observational studies will also be included to understand the current state and progression of HRQoL in ADPKD patients. Systematic reviews related to the research topic will also be included to extract original studies from them.
该研究将包括报告健康相关生活质量 （HRQoL） 基线数据或安慰剂组数据的 RCT。还将包括观察性研究，以了解 ADPKD 患者 HRQoL 的现状和进展。还将包括与研究主题相关的系统评价，以从中提取原始研究。

Due to the lack of complete quantitative data, which hinders data extraction and analysis, or the potential methodological bias arising from the absence of rigorous peer review, the following types of studies will be excluded to ensure the quality and scientific rigor of the included literature: editorials, commentaries, non-peer-reviewed articles, conference abstracts, and protocols. Furthermore, as this study focuses on quantitative data analysis, qualitative studies and case reports will also be excluded.
由于缺乏完整的定量数据，阻碍了数据的提取和分析，或者由于缺乏严格的同行评审而产生潜在的方法学偏差，以下类型的研究将被排除，以确保纳入文献的质量和科学严谨性：社论、评论、非同行评审文章、会议摘要和协议。此外，由于本研究侧重于定量数据分析，定性研究和病例报告也将被排除在外。

2.5 Study Selection Method
2.5 研究选择方法

Literature retrieved from various databases will be imported into EndNote for deduplication, and subsequently into Rayyan for title and abstract screening based on the inclusion and exclusion criteria established by the research team. If eligibility remains unclear, the literature will be retained for the full-text screening phase. During full-text screening, each document will be assessed against the criteria, and reasons for exclusion will be documented.
从各种数据库中检索到的文献将被导入 EndNote 进行重复数据删除，随后导入 Rayyan 根据研究团队制定的纳入和排除标准进行标题和摘要筛选。如果资格仍不清楚，文献将被保留在全文筛选阶段。在全文筛选期间，将根据标准评估每份文件，并记录排除原因。

As this is a student project, all steps will be conducted independently by the researcher.
由于这是一个学生项目，所有步骤都将由研究人员独立进行。

2.6 QA Tools
2.6 QA 工具

To account for the diversity of included study types and minimize differences in the final results, the JBI Critical Appraisal Tools will be used for quality assessment. These tools are similarly designed, ensuring uniformity in evaluation across different study types and providing a comprehensive, standardized framework that guarantees consistency and comparability in the assessment results. The JBI tools are highly reliable, globally recognized, scientifically robust, and also concise and user-friendly, making them practical for reviewers with limited research experience. The detailed QA content of the JBI Critical Appraisal Tools will be provided in the attached document.
为了说明纳入研究类型的多样性并最大限度地减少最终结果的差异，JBI 关键评估工具将用于质量评估。这些工具的设计相似，可确保不同研究类型评估的一致性，并提供一个全面的标准化框架，以保证评估结果的一致性和可比性。JBI 工具高度可靠、全球认可、科学稳健、简洁且用户友好，对于研究经验有限的审稿人来说非常实用。附件中将提供 JBI 关键评估工具的详细 QA 内容。

2.7 Data extraction
2.7 数据提取

A data extraction form was developed based on the data items recommended in the Cochrane Handbook, and adjusted according to the research topic to ensure that the collected data meets the research needs. Before formally starting data extraction, a pre-test was conducted using a study that had passed the literature screening to confirm the form's feasibility and make necessary adjustments. The extracted data from the pre-test paper will serve as a template to guide subsequent data extraction work.
根据 Cochrane 手册中推荐的数据项开发数据提取表，并根据研究主题进行调整，以确保收集的数据满足研究需求。在正式开始数据提取之前，使用通过文献筛选的研究进行了预测试，以确认表格的可行性并进行必要的调整。从预试卷中提取的数据将作为模板，指导后续的数据提取工作。

To increase the accuracy of the obtained data, it is typically recommended that at least two trained researchers independently extract data from the included literature, with regular meetings to check for consistency in the extraction content and discuss any issues encountered during extraction to improve data accuracy. If there is a discrepancy between the two reviewers' data extraction results, a third-party reviewer is usually brought in for evaluation. However, since this is a student project, all of the above steps will be independently completed by a single researcher.
为了提高所获数据的准确性，通常建议至少两名训练有素的研究人员独立地从纳入的文献中提取数据，并定期召开会议以检查提取内容的一致性并讨论提取过程中遇到的任何问题，以提高数据准确性。如果两个审阅者的数据提取结果之间存在差异，通常会引入第三方审阅者进行评估。但是，由于这是一个学生项目，上述所有步骤都将由一名研究人员独立完成。

Data Items:
数据项：

(1) Study Information: Title, author, publication year, country;
（1） 研究信息：标题、作者、出版年份、国家;

(2) Methods: Study design, study objectives, inclusion criteria, exclusion criteria;
（2） 方法：研究设计、研究目标、纳入标准、排除标准;

(3) Characteristics of Participants/Groups: Sample size, average age, proportion of females, proportion of patients with family history,incidence of comorbidities
（3） 参与者/组特征：样本量、平均年龄、女性比例、有家族史的患者比例、合并症发生率

(4) Study Results and Conclusions: Measurement tools, main findings on HRQoL for patients and relatives at different CKD stages;
（4） 研究结果和结论： 测量工具，不同 CKD 阶段患者和亲属的 HRQoL 的主要发现;

2.8 Data synthesis
2.8 数据合成

Meta-analysis would be a more robust approach for presenting results if heterogeneity in outcomes could be controlled. However, in this review, due to the variety of health-related quality of life measurement tools, the varying quality of assessments, and the expected small number of included studies, conducting a meta-analysis is not feasible. Therefore, a narrative synthesis will be employed to describe the results.The key information of each study was summarised in text form and compiled into tables.
如果可以控制结局的异质性，Meta 分析将是一种更可靠的结果呈现方法。然而，在本综述中，由于与健康相关的生活质量测量工具多种多样，评估质量参差不齐，以及预期的纳入研究数量少，进行meta分析是不可行的。因此，将采用叙述性综合来描述结果。每项研究的关键信息以文本形式总结并汇编成表格。

2.9 Ethics
2.9 道德规范

This systematic review is considered to be low-risk as it only involves secondary data collection so did not require ethical approval. The relevant risk assessment form was signed by the student and the supervisor, appendix X
本系统综述被认为是低风险的，因为它只涉及二级数据收集，因此不需要伦理批准。相关风险评估表由学生和导师签署，附录 X.

Result
结果

3.1 Ethics 

A total of 2501 articles were retrieved based on the search strategy (see Figure X). After removing 293 duplicates, 2,208 articles were included. A preliminary screening based on the titles and abstracts identified 51 studies as relevant to the research topic. After excluding 2 studies without full-text availability, 49 studies were selected for full-text review. Additionally, 4 new studies were identified through other sources, including reference lists and citation tracking. After excluding 2 studies without full text, 2 studies proceeded to full-text review. A total of 44 articles were excluded for not meeting the inclusion criteria. The reasons for exclusion included: studies involving populations of PLD (polycystic liver disease) patients; studies where patient stratification is based on age or kidney volume, rather than chronic kidney disease (CKD)-related criteria; studies where the classification of patients by estimated glomerular filtration rate (eGFR) does not align with standard CKD stages; studies where outcome measures were not assessed using standardized scales, or lacked specific measurements of health-related quality of life; and studies categorized as design protocols or other forms of research not meeting the requirements of a systematic review. (the reasons for exclusion at the full-text screening stage are presented in the appendix) 

Ultimately, a total of 7 articles were included in the systematic review. 

3.2 Study Characteristics 

The publication years of the 7 included studies range from 2015 to 2024. Among the included studies, four were cross-sectional studies within observational research, while the remaining three were longitudinal studies. Two of the longitudinal studies only included baseline data and can thus be regarded as cross-sectional studies（1，7）. Although one study was a longitudinal design, some participants underwent multiple HRQoL assessments; however, the primary design of the study was based on cross-sectional data for comparison and analysis（6）. The analysis focused on the impact of different CKD stages and pain on HRQoL, rather than changes over time, and as such, it can also be considered a cross-sectional study. 

Four of the seven studies were conducted in a single country, specifically the UK (n=139), the USA (n=1086；n=1043), and Japan (n=188). The other three studies were conducted across multiple countries, with two studies carried out within Europe, and one study spanning multiple countries across Europe, North America, Asia, and Oceania. 

3.3. Sample Characteristics 

Description of study population 

All studies required the inclusion of ADPKD patients. Most studies specified that patients should be 18 or 20 years old and above, with only two studies having no age requirement (1, 5). One study explicitly required patients to have hypertension (5). 

When filtering study data, CKD stages were classified according to specific CKD grading criteria or converted CKD stages. The results showed that out of the seven studies, four provided complete usable data. Of these, only two studies (1, 6) comprehensively included quality of life data for ADPKD patients across CKD stages 1–5. One study (7) collected quality of life data from dialysis patients, and another (3) collected data from patients in CKD stages 1–3. The remaining three studies had patient eGFR classifications that did not conform to standard CKD classification criteria, so only partial data could be included. The usable data were as follows: quality of life data for patients in CKD stage 3 (2), CKD stage 3a (5), and dialysis and post-kidney transplant stages (4). 

These seven studies included a total of 6,573 participants, with the number of participants in each study ranging from 139 to 3,409. Three studies had over 1,000 participants (1, 6, 5), two of which, with sample sizes of 1,086 and 3,409, included ADPKD patients across all CKD stages. The remaining studies had fewer than 500 participants. With the exception of one study that did not mention the recruitment method, four studies primarily used hospital-based recruitment. Two studies recruited participants through patient registries, with sample sizes of 1,086 and 139 participants. The study with 1,086 participants collected data covering all CKD stages, while in the study with 139 participants, only data from CKD stage 3 were usable. 

A total of three studies mentioned participant recruitment: one study reported no significant differences between those who participated and those who did not, while the other two studies reported recruitment rates of 91% (4) and 84% (6), indicating a high overall recruitment rate and representativeness of the sample. Some patients were excluded from the data analysis due to difficulty in contact or incomplete data (2).
共有三项研究提到了受试者招募：一项研究报告了参与者和未参与者之间没有显著差异，而另外两项研究报告的招募率分别为 91% （4） 和 84% （6），表明总体招募率和样本的代表性很高。由于难以联系或数据不完整，一些患者被排除在数据分析之外 （2）。

The average age of the included patients ranged from 43 to 64 years. Three studies provided age breakdowns by CKD stage (4, 5, 7), with the average age of CKD stage 3a patients being 47 years, kidney transplant patients 59 years, and dialysis patients 56.7 and 64 years, respectively. The gender distribution in most studies was around 50%, showing no significant gender differences, except for one study where 71% of participants were female (1).
纳入患者的平均年龄为 43 至 64 岁。三项研究提供了按 CKD 分期 （4、5、7） 划分的年龄细分，其中 CKD 3a 期患者的平均年龄分别为 47 岁、肾移植患者 59 岁和透析患者 56.7 岁和 64 岁。大多数研究的性别分布约为 50%，没有显示显着的性别差异，除了一项研究 71% 的参与者是女性 （1）。

Two studies reported the genotype distribution of ADPKD patients, with Study 1 showing a PKD1 genotype proportion of 47.4% and PKD2 at approximately 8.7%, while Study 3 reported a PKD1 proportion of 65.8% and PKD2 at 17.6% (1, 3).
两项研究报告了 ADPKD 患者的基因型分布，研究 1 显示 PKD1 基因型比例为 47.4%，PKD2 约为 8.7%，而研究 3 报告的 PKD1 比例为 65.8%，PKD2 比例为 17.6% （1， 3）。

Additionally, three studies reported the proportion of ADPKD patients with a recorded family history of the disease, ranging from 69.4% to 86% (2, 5, 6).
此外，3 项研究报告了有该疾病家族史的 ADPKD 患者的比例，范围为 69.4% 至 86% （2， 5， 6）。

Comorbidities
合并症

Five studies reported on the comorbidities of patients (2, 3, 4, 6, 7). Study 2 indicated that 92.1% of patients in CKD stage 3 had comorbidities (eGFR > 60: 70.8% vs. eGFR 30–60: 92.1% vs. eGFR < 30: 94.4%).
5项研究报告了患者的合并症（2、3、4、6、7）。研究 2 表明，92.1% 的 CKD 3 期患者患有合并症（eGFR > 60：70.8% vs. eGFR 30-60：92.1% vs. eGFR < 30：94.4%）。

Hypertension was the most common comorbidity across all CKD stages (2, 3, 4, 6, 7). Study (2) reported that the prevalence of hypertension in CKD stage 3 patients was 78.9%. Study (3) showed a prevalence of 58.7% in CKD stages 1-3. In dialysis and kidney transplant patients, the prevalence was 84% and 60%, respectively (4). Additionally, study (6) reported that the prevalence of hypertension ranged from 67.6% to 89.1%. Due to the inclusion criteria, all patients in study (5) had hypertension.
高血压是所有 CKD 分期 （2 、 3 、 4 、 6 、 7 分期） 中最常见的合并症。 研究 （2） 报告称，CKD 3 期患者的高血压患病率为 78.9%。研究 （3） 显示 CKD 1-3 期的患病率为 58.7%。在透析和肾移植患者中，患病率分别为 84% 和 60% （4）。此外，研究 （6） 报告称，高血压的患病率从 67.6% 到 89.1% 不等。根据纳入标准，研究 （5） 中的所有患者都患有高血压。

Study 6 reported that, in addition to hypertension, common comorbidities in the first three stages of CKD included hematuria, upper urinary tract infections, and proteinuria, each with a frequency exceeding 20%. In CKD stage 4, the frequency of anemia increased to 29%, and in CKD stage 5, it rose to 53.5% (6). Study 4 showed that the frequency of anemia in dialysis patients was significantly higher than in kidney transplant patients (89% vs. 11%). Additionally, the frequency of hyperthyroidism in dialysis patients was higher than in kidney transplant patients (79% vs. 19%). Study 7 reported that the frequencies of cerebrovascular and cardiovascular diseases among dialysis patients were 19.1% and 12.2%, respectively. Study 2 also reported that the frequency of depression among patients was 22%, although it did not specify the CKD stage.
研究 6 报告称，除高血压外，CKD 前 3 阶段的常见合并症包括血尿、上尿路感染和蛋白尿，每一种的频率都超过 20%。在 CKD 4 期，贫血频率增加到 29%，在 CKD 5 期，贫血率上升到 53.5% （6）。研究 4 显示，透析患者贫血的频率明显高于肾移植患者（89% 对 11%）。此外，透析患者甲状腺功能亢进症的频率高于肾移植患者 （79% vs. 19%）。研究 7 报告称，透析患者脑血管和心血管疾病的频率分别为 19.1% 和 12.2%。研究 2 还报告了患者抑郁的频率为 22%，尽管它没有具体说明 CKD 分期。

3.4. Measurement of Quality of Life
3.4. 生活质量的测量

The tools used to assess the quality of life (QoL) in ADPKD patients were categorized into general QoL scales, kidney disease-specific scales, and ADPKD-specific scales. Most studies employed multiple QoL measurement tools. The most frequently used general QoL tool was the SF-36 Health Survey, which was utilized in three studies. The SF-12v2, a simplified version of the SF-36, and the kidney disease-specific KDQOL-SF 1.3, which is an extension of the SF-36, were used in six of the seven studies, each employing at least one of these tools. Other quality of life measurement tools included the generic scale EQ-5D, the ADPKD-specific scale ADPKD-IS, and the ADPKD Urinary Impact Scale.
用于评估 ADPKD 患者生活质量 （QoL） 的工具分为一般 QoL 量表、肾脏疾病特异性量表和 ADPKD 特异性量表。大多数研究采用了多种 QoL 测量工具。最常用的通用 QoL 工具是 SF-36 健康调查，用于 3 项研究。SF-12v2 是 SF-36 的简化版本，肾脏疾病特异性 KDQOL-SF 1.3 是 SF-36 的扩展，在七项研究中被使用，每项研究都至少使用了其中一种工具。其他生活质量测量工具包括通用量表 EQ-5D、ADPKD 特异性量表 ADPKD-IS 和 ADPKD 尿液影响量表。

3.5 Results of Primary Quality of Life Outcomes
3.5 主要生活质量结局的结果

Quality of Life of Patients in Different Stages of CKD
CKD 不同阶段患者的生活质量

Six studies have used the SF-36-based scale to assess the HRQoL in ADPKD patients. The scale divides the HRQoL score into two components: Physical Component Summary and Mental Component Summary.
六项研究使用基于 SF-36 的量表来评估 ADPKD 患者的 HRQoL。该量表将 HRQoL 分数分为两个部分：身体成分总结和心理成分总结。

For the physical component, HRQoL scores showed a decreasing trend as CKD stages advanced. The most significant decline in physical HRQoL was observed at CKD stage 5, with the lowest scores seen in ADPKD patients undergoing dialysis. After kidney transplantation, physical HRQoL scores improved.
对于物理成分，HRQoL 评分随着 CKD 分期的进展呈下降趋势。在 CKD 第 5 期观察到体格 HRQoL 下降最显着，接受透析的 ADPKD 患者得分最低。肾移植后，体格 HRQoL 评分有所改善。

For the mental component, the variation in HRQoL across CKD stages was less pronounced compared to the physical aspect. Nevertheless, ADPKD patients on dialysis still had the lowest mental HRQoL scores, while those who underwent kidney transplantation experienced notable improvements in mental health scores.
对于心理成分，与身体方面相比，CKD 阶段 HRQoL 的变化不那么明显。尽管如此，接受透析的 ADPKD 患者的心理 HRQoL 评分仍然最低，而接受肾移植的患者的心理健康评分有显着改善。

(Based on SF36) Physical Component Summary:
（基于 SF36）物理组件摘要：

(Based on SF36) Mental Component Summary:
（基于 SF36）心理成分总结：

Changes in the eight SF-36 dimensions across CKD stages:
CKD 阶段的 8 个 SF-36 维度的变化：

Four studies assessed the eight specific dimensions of the SF-36.
四项研究评估了 SF-36 的 8 个具体维度。
ADPKD patients in CKD stages 1, 2, and 3 had relatively high scores in most dimensions, but their scores were lower in the General Health and Vitality dimensions.
CKD 1 、 2 和 3 期的 ADPKD 患者在大多数维度上的得分相对较高，但他们在一般健康和活力维度上的得分较低。
Across all dimensions, ADPKD patients undergoing dialysis had significantly lower scores compared to other CKD groups, particularly in the Physical Functioning and General Health dimensions.
在所有维度上，与其他 CKD 组相比，接受透析的 ADPKD 患者的评分显着降低，尤其是在身体功能和一般健康维度。

EQ-5D
EQ-5D 系列
One study examined the EQ-5D index for patients with ADPKD across CKD stages 1 to 5 (based on the U.S. general population) (6), revealing a decreasing trend in EQ-5D index scores as CKD progressed, with scores dropping from 0.92 in CKD1 to 0.83 in CKD5. Another study (4) assessed the EQ-5D index in dialysis and kidney transplant people with ADPKD (based on the general populations of the UK, Denmark, and Switzerland) (4). The EQ-5D index for dialysis patients ranged from 0.68 to 0.80, while kidney transplant patients had index scores between 0.80 and 0.85. Across the five dimensions of the EQ-5D, the percentage of dialysis patients reporting issues was higher than for kidney transplant patients, with four dimensions showing nearly double the percentage of issues compared to transplant patients (Mobility: 48% vs. 22%; Self-care: 18% vs. 8%; Usual activities: 62% vs. 32%; Anxiety/Depression: 41% vs. 22%).
一项研究检查了 CKD 1 至 5 期 ADPKD 患者的 EQ-5D 指数（基于美国普通人群）[6]，显示随着 CKD 的进展，EQ-5D 指数评分呈下降趋势，评分从 CKD1 的 0.92 下降到 CKD5 的 0.83。另一项研究 （4） 评估了透析和肾移植 ADPKD 患者的 EQ-5D 指数（基于英国、丹麦和瑞士的一般人群）（4）。透析患者的 EQ-5D 指数范围为 0.68 至 0.80，而肾移植患者的指数评分在 0.80 至 0.85 之间。在 EQ-5D 的五个维度中，透析患者报告问题的百分比高于肾移植患者，四个维度显示问题百分比几乎是移植患者的两倍（活动能力：48% 对 22%;自我保健：18% 对 8%;日常活动：62% 对 32%;焦虑/抑郁：41% 对 22%）。

KDQOL-SF 1.3
KDQOL-SF 1.3 系列
Two studies used the KDQOL-SF 1.3 to assess HRQoL in patients with ADPKD at CKD stages 1 to 3 and stage 3 (2, 3). In addition to SF-36 data, this instrument measures kidney function-related HRQoL, with scores ranging from 0 to 100, where higher scores indicate better quality of life. Across all dimensions, HRQoL scores decreased as CKD progressed.
两项研究使用 KDQOL-SF 1.3 评估 CKD 1 至 3 期和 3 期 ADPKD 患者的 HRQoL （2， 3）。除了 SF-36 数据外，该仪器还测量与肾功能相关的 HRQoL，分数范围为 0 到 100，其中分数越高表示生活质量越好。在所有维度中，HRQoL 评分都随着 CKD 的进展而下降。

KDQOL-SF 1.3

ADPKD-IS
Two studies utilized the ADPKD-IS scale (1, 6) to measure HRQoL in patients with CKD stages 1 to 5. Study 1 focused on the impact of sleep disturbances on the fatigue dimension, finding that CKD stage 5 patients experienced significantly more frequent and severe sleep disruptions than patients at other stages (88% of patients experienced disturbances, with 39% reporting that dull kidney pain completely disrupted their sleep). Study 6 provided assessments of the physical, emotional, and fatigue burdens of patients across CKD stages. Scores generally increased with CKD progression, though scores for CKD2 were lower than for CKD1 in all dimensions. The ranges for the physical, emotional, and fatigue dimensions were 1.44–2.33, 1.66–2.27, and 1.71–2.56, respectively.
两项研究使用 ADPKD-IS 量表 （1， 6） 来测量 CKD 1 至 5 期患者的 HRQoL。研究 1 侧重于睡眠障碍对疲劳维度的影响，发现 CKD 5 期患者比其他阶段的患者经历更频繁和更严重的睡眠中断（88% 的患者经历过障碍，其中 39% 的患者报告说钝痛完全扰乱了他们的睡眠）。研究 6 评估了 CKD 各个阶段患者的身体、情感和疲劳负担。评分通常随着 CKD 的进展而增加，尽管 CKD2 的评分在所有维度上都低于 CKD1。身体、情绪和疲劳维度的范围分别为 1.44-2.33、1.66-2.27 和 1.71-2.56。

ADPKD-UIS
Study 6 measured urinary symptoms across CKD stages, including frequency, urgency, and nocturia. Scores for these dimensions generally increased with CKD progression, though the scores for CKD2 were lower than those of other stages.
研究 6 测量了 CKD 各个阶段的泌尿系统症状，包括尿频、尿急和夜尿。这些维度的评分通常随着 CKD 的进展而增加，尽管 CKD2 的评分低于其他阶段的评分。

3.6 Results of Secondary Quality of Life Outcomes
3.6 次要生活质量结果的结果

The health-related quality of life of relatives of ADPKD patients was not addressed in the included literature.
纳入的文献未涉及 ADPKD 患者亲属的健康相关生活质量。

3.7 Subgroup Analyses
3.7 子组分析

Comorbidities
合并症

As expected, comorbidities in ADPKD patients are negatively associated with HRQoL. Study 2 found a significant negative correlation between depression and both PCS (r = -0.62, P < 0.001) and MCS (r = -0.81, P < 0.001). Additionally, the presence of known intracranial aneurysms and hepatic cysts was positively correlated with the "Kidney Disease Burden" dimension, explaining 9% of the variance in patients' burden scores.
正如预期的那样，ADPKD 患者的合并症与 HRQoL 呈负相关。研究 2 发现抑郁症与 PCS （r = -0.62，P < 0.001） 和 MCS （r = -0.81，P < 0.001） 之间存在显著的负相关。此外，已知颅内动脉瘤和肝囊肿的存在与 “肾脏疾病负担” 维度呈正相关，解释了患者负担评分 9% 的差异。

Pain
疼痛

Three studies examined the relationship between pain and HRQoL (Studies 1, 3, and 5). Study 1 reported a significant positive correlation between the ADPKD-PDS (pain severity) and ADPKD-IS (HRQoL) scales, indicating that higher pain severity was associated with stronger patient responses to pain (P < 0.0001).
3 项研究检查了疼痛与 HRQoL 之间的关系（研究 1、3 和 5）。研究 1 报告了 ADPKD-PDS（疼痛严重程度）和 ADPKD-IS （HRQoL） 量表之间存在显着的正相关，表明较高的疼痛严重程度与更强的患者对疼痛的反应相关 （P < 0.0001）。

The intensity of three ADPKD-specific types of pain—dull pain, sharp pain, and discomfort—showed statistically significant increases as CKD progressed (P-values: 0.0259, 0.0047, and 0.0002, respectively). However, the effect of CKD stage on pain intensity was small, as indicated by low eta-squared values from one-way ANOVA: 0.015 for dull pain, 0.021 for sharp pain, and 0.030 for discomfort.
随着 CKD 的进展，三种 ADPKD 特异性疼痛类型（钝痛、锐痛和不适）的强度显示出统计学上的显着增加（P 值：分别为 0.0259、0.0047 和 0.0002）。然而，CKD 分期对疼痛强度的影响很小，如单因素方差分析的低 eta 平方值所示：钝痛为 0.015，尖锐疼痛为 0.021，不适为 0.030。

Study 3 stratified patients based on back pain and found significant differences in 14 of 20 KDQoL-SF dimensions (P < 0.01), with the greatest difference in "Physical Activity Limitations" (absolute mean difference >10). However, the study did not assess the relationship between pain and CKD stage, potentially introducing bias from confounding factors.
研究 3 根据背痛对患者进行分层，发现 20 个 KDQoL-SF 维度中的 14 个存在显著差异 （P < 0.01），其中 “身体活动受限” 差异最大 （绝对平均差异 >10）。然而，该研究没有评估疼痛与 CKD 分期之间的关系，这可能会引入混杂因素的偏倚。

Study 5 reported no significant correlation between pain intensity and eGFR, the measure used to classify CKD stages, though this was only mentioned descriptively without supporting data. Additionally, the study noted that approximately 20% of patients experienced abdominal discomfort.
研究 5 报告了疼痛强度与 eGFR（用于对 CKD 分期进行分类的测量方法）之间没有显著相关性，尽管这只是描述性提及，没有支持数据。此外，该研究指出，大约 20% 的患者感到腹部不适。

Gender
性

Three studies investigated the relationship between gender and HRQoL in ADPKD patients (Studies 2, 3, and 5). Study 2 identified gender as an independent factor influencing the "Kidney Disease Impact" dimension of HRQoL, explaining 18% of the variance. Female gender was also an independent risk factor for poorer mental health (MCS, B = −18.305, P < 0.001). Study 3 found a significant association between gender and the pain and sleep dimensions of the KDQoL-SF, with women reporting lower HRQoL scores for both pain and sleep (Bonferroni-corrected p-values: pain = 0.040; sleep = 0.020).
3 项研究调查了 ADPKD 患者性别与 HRQoL 之间的关系（研究 2、3 和 5）。研究 2 将性别确定为影响 HRQoL 的“肾脏疾病影响”维度的独立因素，解释了 18% 的方差。女性也是心理健康状况较差的独立危险因素 （MCS， B = −18.305， P < 0.001）。研究 3 发现性别与 KDQoL-SF 的疼痛和睡眠维度之间存在显著关联，女性报告的疼痛和睡眠 HRQoL 评分较低（Bonferroni 校正的 p 值：疼痛 = 0.040;睡眠 = 0.020）。

Study 5 stratified HRQoL scores for CKD stage 3a patients by gender, showing that women scored lower in the bodily pain dimension than men (82.98 vs. 73.93) and experienced higher radicular pain (median [IQR]: men 3.0 vs. women 4.0). Women also reported more severe abdominal distension symptoms, such as abdominal enlargement and reduced food intake, which were more common in those with lower eGFR levels (P < 0.05).
研究 5 按性别对 CKD 3a 期患者进行了分层 HRQoL 评分，显示女性在身体疼痛维度上的得分低于男性（82.98 对 73.93），神经根疼痛更高（中位数 [IQR]：男性 3.0 对女性 4.0）。女性还报告了更严重的腹胀症状，如腹部增大和食物摄入减少，这些症状在 eGFR 水平较低的人群中更常见 （P < 0.05）。

Economic status
经济状况

Economic status may be a significant factor affecting the quality of life in ADPKD patients. Study 1 found that patients unable to access appropriate care due to insurance issues experienced a significant decrease in quality of life (OR = 0.46, 95% CI: 0.21–0.99, P = 0.0418). Study 6 reported that the number of medical visits increased with CKD progression (CKD1: 1.7 vs. CKD5: 4.5), while the employment rate decreased as CKD advanced (CKD1: 72.9% vs. CKD5: 48.5%), further highlighting the financial burden patients face as CKD progresses.
经济状况可能是影响 ADPKD 患者生活质量的重要因素。研究 1 发现，由于保险问题而无法获得适当护理的患者生活质量显着下降 （OR = 0.46,95% CI： 0.21–0.99，P = 0.0418）。研究 6 报告称，随着 CKD 的进展，就诊次数增加（CKD1：1.7 vs. CKD5：4.5），而就业率随着 CKD 的进展而下降（CKD1：72.9% vs. CKD5：48.5%），进一步凸显了随着 CKD 进展患者面临的经济负担。

3.8 Quality Assessment
3.8 质量评估

All studies clearly defined objective inclusion and exclusion criteria, and provided detailed descriptions of the study settings, timeframes, and characteristics of the study populations, ensuring comparability among participants and helping to reduce selection bias. Most studies identified potential confounding factors. However, four studies did not control for confounders, and three studies did not employ appropriate statistical methods. Among the three longitudinal studies included in the quality analysis（1，6，7）, none controlled for confounding factors, and they only performed descriptive statistics without further analytical statistical methods, resulting in a higher risk of bias. Additionally, one other study did not control for confounding factors（4）, also presenting a potential risk of confounding bias.
所有研究都明确定义了客观的纳入和排除标准，并提供了研究设置、时间框架和研究人群特征的详细描述，确保参与者之间的可比性并有助于减少选择偏倚。大多数研究确定了潜在的混杂因素。然而，4项研究没有控制混杂因素，3项研究没有采用适当的统计方法。在质量分析中包含的三项纵向研究 （1,6,7） 中，没有一项控制混杂因素，它们只进行描述性统计，没有进一步的分析统计方法，导致更高的偏倚风险。此外，另一项研究没有控制混杂因素 （4），也存在混杂偏倚的潜在风险。

Table N: Quality Assessment of Included Studies Using the JBI Critical Appraisal Tool
表 N：使用 JBI Critical Appraisal 工具对纳入的研究进行质量评估

Legend to the Table N:
表 N 的图例：

Were the criteria for inclusion in the sample clearly defined?
样本的纳入标准是否明确定义？

Were the study subjects and the setting described in detail?
是否详细描述了研究对象和环境？

Was the exposure measured in a valid and reliable way?
是否以有效和可靠的方式测量了暴露？

Were objective, standard criteria used for measurement of the condition?
是否使用客观、标准的标准来测量病情？

Were confounding factors identified?
是否确定了混杂因素？

Were strategies to deal with confounding factors stated?
是否说明了处理混杂因素的策略？

Were the outcomes measured in a valid and reliable way?
结局是否以有效和可靠的方式测量？

Was appropriate statistical analysis used?
是否使用了适当的统计分析？

Discussion
讨论

4.1 Summary of Main Findings
4.1 主要调查结果总结

After excluding studies with a high risk of bias, Studies 2, 3, 4, and 5 were included in the sensitivity analysis. The conclusions were consistent with the overall findings: as CKD stage progresses, the physical health-related quality of life (HRQOL) of ADPKD patients declines, with dialysis patients reporting the lowest quality of life across all stages, while HRQOL improves significantly for kidney transplant patients. There was little difference in the psychological dimension of HRQOL among patients at different CKD stages; however, compared to dialysis patients, kidney transplant patients experienced an improvement in psychological HRQOL.
在排除具有高偏倚风险的研究后，研究 2、3、4 和 5 被纳入敏感性分析。结论与总体结果一致： 随着 CKD 分期的进展，ADPKD 患者的身体健康相关生活质量 （HRQOL） 下降，透析患者在所有阶段的生活质量最低，而肾移植患者的 HRQOL 显着改善。 不同 CKD 分期患者 HRQOL 的心理维度差异不大;然而，与透析患者相比，肾移植患者的心理 HRQOL 有所改善。

4.2 Limitations and Strengths of the Study
4.2 研究的局限性和优势

Strengths of this study:
本研究的优势：

Research on the health-related quality of life (HRQoL) of patients with autosomal dominant polycystic kidney disease (ADPKD) remains scarce. In this systematic review, no prior English-language review was identified that examines the impact of ADPKD progression on patient HRQoL across different stages of chronic kidney disease (CKD). Therefore, this study provides valuable insights into the HRQoL of ADPKD patients at varying CKD stages.
关于常染色体显性遗传性多囊肾病 （ADPKD） 患者健康相关生活质量 （HRQoL） 的研究仍然很少。在本系统评价中，未发现先前的英文评价来检查 ADPKD 进展对慢性肾脏病 （CKD） 不同阶段患者 HRQoL 的影响。因此，本研究为不同 CKD 阶段的 ADPKD 患者的 HRQoL 提供了有价值的见解。

By categorizing ADPKD patients according to CKD stages, the study offers a detailed understanding of how disease progression affects HRQoL, highlighting the changes in patients’ HRQoL at different stages. These findings can assist healthcare professionals in better understanding the disease’s impact, delivering personalized care recommendations, and improving patient-centered disease management strategies.
通过根据 CKD 分期对 ADPKD 患者进行分类，该研究详细了解了疾病进展如何影响 HRQoL，突出了患者在不同阶段的 HRQoL 变化。这些发现可以帮助医疗保健专业人员更好地了解疾病的影响，提供个性化的护理建议，并改进以患者为中心的疾病管理策略。

Moreover, the study design also included an exploration of the HRQoL of first-degree relatives and cohabiting family members of ADPKD patients. Given that ADPKD is a hereditary disorder, the study aimed to assess the potential dynamic impact of the disease’s progression on relatives who may or may not have the genetic condition. Although the final literature did not cover this aspect, this research perspective provides new directions for future quantitative studies.
此外，研究设计还包括对 ADPKD 患者一级亲属和同居家庭成员的 HRQoL 的探索。鉴于 ADPKD 是一种遗传性疾病，该研究旨在评估疾病进展对可能患有或可能没有遗传病的亲属的潜在动态影响。虽然最终的文献没有涵盖这方面，但这一研究视角为未来的定量研究提供了新的方向。

Limitations of this study:
本研究的局限性：

Limited number of included studies: Only seven studies were included in this review, with a wide variation in the number of participants across these studies. This limitation affects the representativeness of the sample, and the small number of included studies may not fully capture the changes in HRQoL in ADPKD patients.
纳入的研究数量有限： 本综述仅纳入了 7 项研究，这些研究的受试者人数差异很大。这种局限性影响了样本的代表性，并且纳入的研究数量少可能无法完全捕捉 ADPKD 患者 HRQoL 的变化。

Single-researcher approach reduces objectivity: As this is a student thesis, literature screening, data extraction, and quality assessment were conducted independently by a single researcher. According to best practices, these steps should be performed independently by at least two trained reviewers. Conducting these steps by a single researcher may have led to omissions or biases in data extraction, thereby reducing the objectivity of the study.
单一研究人员方法降低了客观性：由于这是一篇学生论文，文献筛选、数据提取和质量评估由单一研究人员独立进行。根据最佳实践，这些步骤应由至少两名训练有素的审阅者独立执行。由单个研究人员执行这些步骤可能会导致数据提取中的遗漏或偏差，从而降低研究的客观性。

Insufficient use of disease-specific instruments: The studies predominantly used generic HRQoL instruments, with ADPKD-specific tools being underutilized. This may limit the ability to identify ADPKD-specific impacts on patient quality of life.
疾病特异性工具的使用不足：这些研究主要使用通用的 HRQoL 工具，而 ADPKD 特异性工具未得到充分利用。这可能会限制确定 ADPKD 对患者生活质量的特异性影响的能力。

High heterogeneity: a. Differences in study design: This review included both cross-sectional and longitudinal studies. Although only baseline data from longitudinal studies were used, the differences in study design still hinder comparability, particularly as further statistical analysis of the baseline data from longitudinal studies was lacking. b. Differences in CKD stages among participants: The included studies covered different CKD stages. Some studies included patients across all CKD stages, while others focused on specific stages (e.g., dialysis). The differences in patient distribution across CKD stages reduce the comprehensiveness of comparisons regarding HRQoL across different CKD stages. c. Variations in HRQoL measurement tools: There was considerable variation in the HRQoL instruments used across the included studies, with different assessment dimensions and scoring criteria, making it difficult to compare the results. Additionally, all HRQoL data were self-reported by patients, which may introduce recall bias, compromising the accuracy of the data.
高异质性： a.研究设计的差异： 本综述包括横断面和纵向研究。尽管仅使用了来自纵向研究的基线数据，但研究设计的差异仍然阻碍了可比性，特别是在缺乏对纵向研究基线数据的进一步统计分析的情况下。b.受试者之间 CKD 分期的差异： 纳入的研究涵盖了不同的 CKD 阶段。一些研究纳入了所有 CKD 阶段的患者，而另一些研究则侧重于特定阶段（例如透析）。不同 CKD 分期患者分布的差异降低了不同 CKD 分期 HRQoL 比较的全面性。c. HRQoL 测量工具的差异：纳入研究中使用的 HRQoL 工具存在相当大的差异，具有不同的评估维度和评分标准，因此难以比较结果。此外，所有 HRQoL 数据均由患者自行报告，这可能会引入召回偏倚，从而影响数据的准确性。

Limitations in data analysis: Due to the high heterogeneity of the studies, a meta-analysis could not be conducted. Instead, a narrative synthesis was performed, which may reduce the robustness of the findings.
数据分析的局限性： 由于研究的高度异质性，无法进行荟萃分析。相反，进行了叙述性综合，这可能会降低研究结果的稳健性。

4.3 Comparison with Other Studies
4.3 与其他研究的比较

HRQOL in the Physical Domain:
物理域中的 HRQOL：

The findings of this review show that patients’ physical health-related quality of life declines with the progression of CKD stages, with dialysis patients experiencing the lowest HRQOL among ADPKD patients at various stages. Research by XX et al. indicates that, even in the early stages of CKD, patients with ADPKD experience symptoms such as pain, generalized weakness and fatigue, and shortness of breath, which limit their physical activity. A study by XX et al. on adolescent ADPKD patients also showed that, despite being in the early stages of the disease, some patients experienced pain and urinary problems, reducing their health-related quality of life. Thus, pain may occur frequently even in the early stages of CKD, possibly because in the early stages of CKD, although kidney function has not yet declined, kidney cysts have already formed and caused organic lesions.
本综述的结果表明，随着 CKD 分期的进展，患者与身体健康相关的生活质量下降，透析患者的 HRQOL 在各个阶段的 ADPKD 患者中最低。XX 等人的研究表明，即使在 CKD 的早期阶段，ADPKD 患者也会出现疼痛、全身虚弱和疲劳以及呼吸急促等症状，这些症状限制了他们的身体活动。XX 等人对青少年 ADPKD 患者进行的一项研究还表明，尽管处于疾病的早期阶段，但一些患者经历了疼痛和泌尿问题，从而降低了他们与健康相关的生活质量。因此，即使在 CKD 的早期阶段，疼痛也可能频繁发生，这可能是因为在 CKD 的早期阶段，虽然肾功能尚未下降，但肾囊肿已经形成并引起器质性病变。

Research by XX et al. shows that in the late stages of CKD, ADPKD severely limits patients’ normal physical activities. Patients in CKD stages 4–5 and post-kidney transplantation reduced their physical activity by approximately 30% due to disease-related limitations on physical activity, while physical activity in dialysis patients sharply declined to below 50%. The decline in the quality of life in late-stage CKD patients may be due to the increasing number of complications as CKD progresses, which reduces health-related quality of life. The health-related quality of life (HRQOL) of dialysis patients is markedly lower than that of patients in other stages of the disease, potentially due to the significant comorbidities associated with dialysis, which severely compromise their quality of life. The study by XX et al. points out that end-stage renal disease patients typically experience multiple complications, with these symptoms often interconnected in specific patterns, forming symptom clusters. For instance, fatigue, sleep disturbances, shortness of breath, and dizziness are closely related and influence one another, further diminishing the patients' quality of life.
XX 等人的研究表明，在 CKD 的晚期，ADPKD 严重限制了患者的正常身体活动。由于与疾病相关的身体活动限制，CKD 4-5 期和肾移植后患者的体力活动减少了约 30%，而透析患者的体力活动急剧下降至 50% 以下。晚期 CKD 患者生活质量的下降可能是由于随着 CKD 的进展，并发症数量增加，这降低了与健康相关的生活质量。透析患者的健康相关生活质量 （HRQOL） 明显低于疾病其他阶段的患者，这可能是由于与透析相关的严重合并症，这严重损害了他们的生活质量。XX 等人的研究指出，终末期肾病患者通常会经历多种并发症，这些症状通常以特定模式相互关联，形成症状群。例如，疲劳、睡眠障碍、呼吸急促和头晕密切相关并相互影响，进一步降低患者的生活质量。

Health-related Quality of Life in the Psychological Domain:
心理领域与健康相关的生活质量：

At the diagnosis stage, the qualitative study by XX et al. points out that the diagnosis of ADPKD has a negative impact on patients' mental health, often leaving them feeling anxious, fearful, and lost. On the one hand, due to the hereditary nature of the disease, most patients have a family history, and the experiences of affected relatives deepen their negative outlook on the disease. On the other hand, although some patients have no obvious symptoms, the lack of effective treatment measures and the sense of losing control over the disease make them feel powerless, leading to feelings of depression and frustration.
在诊断阶段，XX 等人的定性研究指出，ADPKD 的诊断对患者的心理健康有负面影响，往往会让他们感到焦虑、恐惧和迷茫。一方面，由于疾病的遗传性，大多数患者都有家族史，受影响的亲属的经历加深了他们对疾病的负面看法。另一方面，虽然部分患者没有明显的症状，但缺乏有效的治疗措施和对疾病失控的感觉使他们感到无能为力，导致抑郁和沮丧的感觉。

In the early stages of the disease, research by XX et al. shows that due to the invisible nature of symptoms, patients' fatigue, depression, and other states are often not perceived by those around them, leaving them feeling isolated and misunderstood. Research by XX et al. indicates that some adolescents with early-stage CKD worry about the progression of the disease and feel lonely and depressed due to the limitations imposed by the disease on social and physical activities.
在疾病的早期阶段，XX 等人的研究表明，由于症状的无形性，患者的疲劳、抑郁和其他状态往往不被周围的人察觉到，让他们感到孤立和被误解。XX 等人的研究表明，一些患有早期 CKD 的青少年担心疾病的进展，并由于疾病对社交和身体活动的限制而感到孤独和沮丧。

Patients' anxiety worsens as the disease progresses. Research by XX et al. indicates that ADPKD patients typically undergo regular kidney function tests, and any decline in kidney function triggers increased anxiety. The study by XX et al. shows that anxiety levels significantly rise in the late stages of CKD, which reduces patients' health-related quality of life. Research by XX et al. and XX et al. further indicates that negative emotions outweigh positive ones in ADPKD patients, with the most common being anxiety, fear, and concern about disease progression. Patients not only worry about potential complications but also fear the progression of the disease to end-stage renal disease. Moreover, uncertainty regarding the prognosis of end-stage renal disease, coupled with the persistence of physical symptoms, further intensifies their anxiety.
随着疾病的进展，患者的焦虑会恶化。XX 等人的研究表明，ADPKD 患者通常会定期接受肾功能检查，肾功能的任何下降都会引发焦虑增加。XX 等人的研究表明，在 CKD 晚期，焦虑水平会显著上升，这会降低患者与健康相关的生活质量。XX 等人和 XX 等人的研究进一步表明，ADPKD 患者的负面情绪大于积极情绪，最常见的是焦虑、恐惧和对疾病进展的担忧。患者不仅担心潜在的并发症，还担心疾病发展为终末期肾病。此外，终末期肾病预后的不确定性，加上身体症状的持续存在，进一步加剧了他们的焦虑。

Concerns about passing the disease on to their children are also a significant source of negative emotions. Patients often perceive carrying and transmitting ADPKD to their offspring as their personal responsibility, leading to profound feelings of guilt over their children's potential illness and a sense of shame for carrying the disease-causing gene. Since ADPKD is a progressive disease, those who were undiagnosed at the time of having children experience even stronger feelings of guilt regarding their offspring's risk of developing the condition.
担心将疾病传染给孩子也是负面情绪的重要来源。患者通常认为携带 ADPKD 并将其传播给后代是他们的个人责任，导致对孩子的潜在疾病产生深深的内疚感，并为携带致病基因而感到羞耻。 由于 ADPKD 是一种进行性疾病，那些在生孩子时未被诊断出来的人对他们的后代患上这种疾病的风险感到更强烈的内疚感。

Chronic pain is another reason for the deterioration of patients’ psychological well-being. Particularly during dialysis, treatment exacerbates pain symptoms, and the close association between pain and anxiety and depression may further lower the psychological HRQOL of patients.
慢性疼痛是患者心理健康恶化的另一个原因。特别是在透析期间，治疗会加剧疼痛症状，疼痛与焦虑和抑郁之间的密切关联可能会进一步降低患者的心理 HRQOL。

As a chronic disease, cognitive and behavioral factors in ADPKD patients have a significant impact on their health-related quality of life (HRQOL). Research shows that patients with more positive expectations about the future of their disease generally exhibit a higher quality of life. However, negative emotions such as anxiety and depression can burden patients psychologically, potentially affecting their positive outlook on the future and subsequently lowering their HRQOL.
作为一种慢性疾病，ADPKD 患者的认知和行为因素对其健康相关生活质量 （HRQOL） 有重大影响。研究表明，对疾病的未来有更积极期望的患者通常表现出更高的生活质量。然而，焦虑和抑郁等负面情绪会给患者带来心理负担，可能会影响他们对未来的积极展望，从而降低他们的 HRQOL。

Additionally, patients' ability to cope with and manage stress, as well as their level of self-efficacy, play crucial roles in their HRQOL. Negative emotions increase feelings of helplessness towards the disease and undermine patients' self-efficacy. The study by XX et al. shows that emotional distress, such as anxiety and depression, can reduce patients' adherence to disease management.
此外，患者应对和管理压力的能力以及他们的自我效能水平在他们的 HRQOL 中起着至关重要的作用。负面情绪会增加对疾病的无助感，并破坏患者的自我效能感。XX 等人的研究表明，焦虑和抑郁等情绪困扰会降低患者对疾病管理的依从性。

The studies included in this review primarily used the SF-36 scale to measure patients' HRQOL, with the Mental Health dimension assessing anxiety and depression. However, the scale does not provide a separate, detailed assessment of anxiety, which may result in less sensitive measurements of patients' anxiety levels. Furthermore, the SF-36 does not evaluate patients' feelings of guilt or self-efficacy, which could lead to an underestimation of the patients' psychological condition in the study results.
本综述纳入的研究主要使用 SF-36 量表来测量患者的 HRQOL，心理健康维度评估焦虑和抑郁。然而，该量表没有提供单独的、详细的焦虑评估，这可能导致对患者焦虑水平的测量不太敏感。此外，SF-36 不评估患者的内疚感或自我效能感，这可能导致研究结果中低估了患者的心理状况。

Impact of ADPKD on Patients’ HRQOL:
ADPKD 对患者 HRQOL 的影响：

A qualitative study by XX et al. shows that compared to death, ADPKD patients are more concerned about disease-related outcomes that impose a significant burden on their quality of life. This may be because death is an inevitable outcome. The ultimate progression of the disease—end-stage renal disease requiring dialysis or kidney transplantation—is considered the most significant factor affecting quality of life. Pain symptoms and complications such as cyst infections and bleeding have a substantial impact on patients' quality of life, being considered as important as death. Other disease outcomes that are seen as greatly limiting quality of life include fatigue, liver cysts, and financial status.
XX 等人的一项定性研究表明，与死亡相比，ADPKD 患者更关心对他们的生活质量造成重大负担的疾病相关结果。这可能是因为死亡是不可避免的结果。疾病的最终进展——需要透析或肾移植的终末期肾病——被认为是影响生活质量的最重要因素。疼痛症状和并发症（如囊肿感染和出血）对患者的生活质量有重大影响，被认为与死亡一样重要。其他被认为极大地限制生活质量的疾病结局包括疲劳、肝囊肿和财务状况。

The Relationship Between Pain and HRQOL:
疼痛和 HRQOL 之间的关系：

This review’s findings suggest that higher levels of pain are associated with poorer HRQOL in ADPKD patients; however, the correlation between pain intensity and chronic kidney disease (CKD) progression remains inconsistent. Even though some studies have found a statistically significant association between pain intensity and CKD progression, the effect of CKD stage on pain intensity is relatively weak. One possible explanation is that, compared to the CKD stage, the size and location of cysts have a more pronounced effect on pain intensity, potentially overshadowing the influence of CKD stage on pain perception.
本综述的结果表明，ADPKD 患者较高水平的疼痛与较差的 HRQOL 相关;然而，疼痛强度与慢性肾脏病 （CKD） 进展之间的相关性仍然不一致。尽管一些研究发现疼痛强度与 CKD 进展之间存在统计学上的显着关联，但 CKD 分期对疼痛强度的影响相对较弱。一种可能的解释是，与 CKD 分期相比，囊肿的大小和位置对疼痛强度的影响更明显，可能掩盖了 CKD 分期对疼痛感知的影响。

The Relationship Between Complications and HRQOL:
并发症与 HRQOL 之间的关系：

This review demonstrates that depression, known cerebral aneurysms, and hepatic cysts, which are common complications, reduce the HRQOL of patients with ADPKD. Hepatic cysts are a common early complication in ADPKD patients, causing physical discomfort such as abdominal distension, dyspnea, nausea, and vomiting. The review also indicates that some ADPKD patients experience abdominal distension.
本综述表明，抑郁症、已知的脑动脉瘤和肝囊肿是常见的并发症，可降低 ADPKD 患者的 HRQOL。肝囊肿是 ADPKD 患者常见的早期并发症，可引起腹胀、呼吸困难、恶心和呕吐等身体不适。该评论还表明，一些 ADPKD 患者会出现腹胀。

As CKD progresses, the number and severity of complications in patients increase, with hypertension being a common complication. Compared to patients with essential hypertension, ADPKD patients are more prone to target organ damage due to hypertension, which leads to progressive diseases such as cardiovascular conditions and further diminishes health-related quality of life. Additionally, depression is highly prevalent among ADPKD patients, and its incidence increases with disease progression. Depression also contributes to sleep disorders, significantly impairing health-related quality of life. A study by XX and colleagues further indicated that ADPKD patients have a higher risk of developing both benign and malignant tumors compared to patients with ordinary kidney diseases, and this risk persists even after kidney transplantation.
随着 CKD 的进展，患者并发症的数量和严重程度增加，高血压是一种常见的并发症。与原发性高血压患者相比，ADPKD 患者更容易因高血压而发生靶器官损伤，从而导致心血管疾病等进行性疾病，并进一步降低与健康相关的生活质量。此外，抑郁症在 ADPKD 患者中非常普遍，其发病率随着疾病进展而增加。抑郁症还会导致睡眠障碍，严重损害与健康相关的生活质量。XX 及其同事的一项研究进一步表明，与普通肾病患者相比，ADPKD 患者患良性和恶性肿瘤的风险更高，并且即使在肾移植后这种风险仍然存在。

The Relationship Between Gender, and HRQOL:
性别与 HRQOL 之间的关系：

This review found significant gender differences in the health-related quality of life (HRQOL) of ADPKD patients, with women experiencing more severe physical pain than men. Symptoms of abdominal distension caused by hepatic cysts were also more prevalent in women with poorer kidney function. Similarly, the study by XX et al. indicated that female patients experienced more severe pain and greater issues with hepatic cysts, alongside higher levels of anxiety and depression compared to men. One possible explanation is that women tend to have larger total liver and kidney mass, and the impact of menstrual symptoms may exacerbate pain severity. This heightened pain, in turn, intensifies feelings of anxiety and depression. Furthermore, since hepatic cysts are associated with estrogen, women are more likely to suffer from abdominal distension due to cysts, contributing to greater abdominal discomfort, which further diminishes their HRQOL.
本综述发现 ADPKD 患者的健康相关生活质量 （HRQOL） 存在显著的性别差异，女性比男性经历更严重的身体疼痛。肝囊肿引起的腹胀症状在肾功能较差的女性中也更为普遍。同样，XX 等人的研究表明，与男性相比，女性患者经历了更严重的疼痛和更大的肝囊肿问题，同时焦虑和抑郁程度更高。一种可能的解释是女性的肝脏和肾脏总质量往往较大，月经症状的影响可能会加剧疼痛的严重程度。这种加剧的痛苦反过来又加剧了焦虑和抑郁的感觉。此外，由于肝囊肿与雌激素有关，女性更容易因囊肿而出现腹胀，导致更大的腹部不适，从而进一步降低她们的 HRQOL。

HRQOL of Relatives of ADPKD Patients:
ADPKD 患者亲属的 HRQOL：

Although this study did not directly provide quantitative information on the HRQOL of relatives of ADPKD patients, existing literature has explored this issue. A qualitative study by XX et al. shows that first-degree relatives of ADPKD patients (e.g., parents or spouses) often serve as the primary caregivers.
虽然这项研究没有直接提供有关 ADPKD 患者亲属 HRQOL 的定量信息，但现有文献已经探讨了这个问题。XX 等人的一项定性研究表明，ADPKD 患者的一级亲属（例如，父母或配偶）通常是主要照顾者。

Whether the caregiver also has ADPKD is a significant factor affecting their HRQoL. ADPKD-affected caregivers are less likely to report that their sleep, daily activities, and work are affected, possibly because they have already adapted to their own disease-related circumstances. However, these relatives report that their quality of life is more impacted during household tasks compared to non-ADPKD caregivers, likely due to the worsening of their own symptoms.
护理人员是否也患有 ADPKD 是影响其 HRQoL 的重要因素。受 ADPKD 影响的照护者不太可能报告他们的睡眠、日常活动和工作受到影响，这可能是因为他们已经适应了自己的疾病相关情况。然而，这些亲属报告说，与非 ADPKD 护理人员相比，他们的生活质量在家务中受到的影响更大，这可能是由于他们自己的症状恶化。

Overall, caregivers’ physical HRQOL is mainly affected by fatigue, with sleep deprivation being the primary cause. Caregivers attribute sleep disturbances to worries about the progression of the ADPKD patient’s condition, as well as frequent nocturnal urination and snoring (possibly caused by kidney pressure) in patients. Psychologically, many caregivers feel anxious and guilty due to concerns about the patient's disease progression and the risk of inheritance to their children. Some caregivers also experience anger and frustration due to the incurable nature of the disease.
总体而言，护理人员的身体 HRQOL 主要受疲劳影响，睡眠剥夺是主要原因。护理人员将睡眠障碍归因于对 ADPKD 患者病情进展的担忧，以及患者频繁的夜间排尿和打鼾（可能是由肾压引起的）。在心理上，许多照顾者由于担心患者的疾病进展和遗传给孩子的风险而感到焦虑和内疚。由于这种疾病的不治之症，一些护理人员也会感到愤怒和沮丧。

Other considerations:
其他注意事项：

Health insurance coverage is an important factor influencing patients' health-related quality of life (HRQoL), likely due to the significant financial burden ADPKD places on patients.
健康保险覆盖率是影响患者健康相关生活质量 （HRQoL） 的重要因素，这可能是由于 ADPKD 给患者带来了巨大的经济负担。

The study by XX et al. shows that medical costs for ADPKD patients are higher at all stages of CKD compared to patients with other kidney diseases, with the cost difference becoming significantly larger from CKD stage 3 onward, peaking during the dialysis stage. The research by XX et al. indicates that the total costs for ADPKD patients in CKD stages 4-5 are approximately twice those of patients in CKD stages 1-3. The economic burden from dialysis is the highest among all CKD stages, with XX et al. reporting that dialysis costs exceed those of CKD stage 1 by more than 30 times. ADPKD patients with hypertension, as well as those at high risk for rapid disease progression, face even higher healthcare expenditures.
XX 等人的研究表明，与其他肾脏疾病患者相比，ADPKD 患者在 CKD 各个阶段的医疗费用都更高，从 CKD 3 期开始，成本差异明显变大，在透析阶段达到顶峰。XX 等人的研究表明，CKD 4-5 期 ADPKD 患者的总费用约为 CKD 1-3 期患者的两倍。透析的经济负担在所有 CKD 阶段中最高，XX 等人报告说，透析成本是 CKD 1 阶段的 30 倍以上。患有高血压的 ADPKD 患者以及疾病快速进展的高风险患者面临着更高的医疗保健支出。

4.4 Policy and Clinical Implications
4.4 政策和临床意义

The limited number and heterogeneity of studies included in this review restrict the ability to provide clear, evidence-based recommendations for ADPKD-related policy development. However, this review consolidates important evidence on the relationship between pain, gender, comorbidities, and HRQoL in ADPKD patients. Additionally, it highlights the health-related quality of life (HRQoL) of relatives who may also be affected by ADPKD, emphasizing the unique burden that hereditary diseases impose. Unlike other common chronic progressive diseases, ADPKD poses not only physical and psychological challenges to patients but also genetic-related burdens and caregiving responsibilities to family members who may themselves be at risk for the disease.
本综述中纳入的研究数量有限且异质性限制了为 ADPKD 相关政策制定提供明确、循证建议的能力。然而，本综述巩固了 ADPKD 患者疼痛、性别、合并症和 HRQoL 之间关系的重要证据。此外，它还强调了也可能受 ADPKD 影响的亲属的健康相关生活质量 （HRQoL），强调了遗传性疾病带来的独特负担。与其他常见的慢性进行性疾病不同，ADPKD 不仅对患者构成身体和心理挑战，还给本身可能面临疾病风险的家庭成员带来与遗传相关的负担和照顾责任。

The findings of this review offer valuable insights into the physical and psychological burden faced by ADPKD patients and their families. Furthermore, the review underscores the gaps in current research on the HRQoL of ADPKD patients, particularly regarding the unique challenges posed by the hereditary nature of the disease. These insights should inform both clinical practice and future research to better address the comprehensive needs of ADPKD patients and their families, guiding the development of more targeted interventions and support systems.
本综述的结果为 ADPKD 患者及其家人面临的生理和心理负担提供了有价值的见解。此外，该综述强调了当前对 ADPKD 患者 HRQoL 的研究存在差距，特别是在疾病遗传性带来的独特挑战方面。这些见解应为临床实践和未来研究提供信息，以更好地满足 ADPKD 患者及其家人的综合需求，指导开发更有针对性的干预措施和支持系统。

4.5 Future Research Directions
4.5 未来的研究方向

1. This systematic review indicates a limited number of studies addressing health-related quality of life (HRQoL) in patients with autosomal dominant polycystic kidney disease (ADPKD). Most studies do not strictly differentiate between the stages of chronic kidney disease (CKD), instead employing a broad classification into early (CKD stages 1 and 2), middle (CKD stage 3), and late stages (CKD stages 4 and 5). Furthermore, while there are qualitative studies on the quality of life of patients' relatives, no quantitative research has been identified that specifically assesses their HRQoL.
1. 本系统评价表明，针对常染色体显性遗传性多囊肾病 （ADPKD） 患者健康相关生活质量 （HRQoL） 的研究数量有限。大多数研究并未严格区分慢性肾脏病 （CKD） 的阶段，而是将广泛分类为早期（CKD 1 期和 2 期）、中期（CKD 3 期）和晚期（CKD 4 期和 5 期）。此外，虽然有关于患者亲属生活质量的定性研究，但尚未确定专门评估其 HRQoL 的定量研究。

a. Future studies should focus on more detailed analyses of HRQoL stratified by CKD stage to better capture the changes in patient quality of life as the disease progresses.
一个。未来的研究应侧重于对按 CKD 分期分层的 HRQoL 进行更详细的分析，以更好地捕捉随着疾病进展患者生活质量的变化。

b. Additionally, future research should consider conducting quantitative studies to assess the HRQoL of relatives of ADPKD patients, with particular attention to the impact of different CKD stages on the relatives' quality of life. This would provide a foundation for optimizing caregiving strategies.
b.此外，未来的研究应考虑进行定量研究以评估 ADPKD 患者亲属的 HRQoL，特别关注不同 CKD 阶段对亲属生活质量的影响。这将为优化护理策略提供基础。

2. Pain-related factors: The conclusions regarding the association between pain intensity and CKD stages in this review are inconsistent. However, given the significant impact of pain on the HRQoL of ADPKD patients, future research should focus on identifying the factors influencing pain. This will enable the development of targeted pain management strategies aimed at improving patients' quality of life.
2. 疼痛相关因素： 本综述中关于疼痛强度与 CKD 分期之间关联的结论不一致。然而，鉴于疼痛对 ADPKD 患者 HRQoL 的显着影响，未来的研究应侧重于确定影响疼痛的因素。这将有助于制定有针对性的疼痛管理策略，旨在提高患者的生活质量。

3. Longitudinal HRQoL research: Most HRQoL studies on ADPKD patients are cross-sectional, and existing longitudinal studies are short-term, primarily focusing on cross-sectional comparisons. There is a lack of research examining long-term changes in HRQoL as the disease progresses. Future studies should extend their duration to investigate these long-term changes and their impact.
3.纵向 HRQoL 研究： 大多数关于 ADPKD 患者的 HRQoL 研究是横断面的，现有的纵向研究是短期的，主要集中在横断面比较。缺乏研究来检查随着疾病进展而 HRQoL 的长期变化。未来的研究应该延长它们的持续时间，以调查这些长期变化及其影响。

No conflicts of interest were declared in this study.
本研究未宣布利益冲突。

Reference：
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Appendix：
附录：

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Medline via Ovid:
Medline 通过 Ovid：

1. (ADPKD or "Autosomal Dominant Polycystic Kidney" or (Dominant and (PKD or ((Renal or Kidney) adj1 (cyst* or failure* or multicystic or cystic or Polycystic))))).mp.
1. （ADPKD 或“常染色体显性遗传多囊肾”或（显性和 （PKD 或（（肾脏或肾脏）adj1（囊肿* 或衰竭* 或多囊或囊性或多囊))))).mp.

("life quality" or "quality of life" or HRQOL or QOL or "mental health" or ((health or function* or mental or symptom*) adj1 (status or assess*)) or "self report*" or "patient* report*" or "PROMs" OR "SF-36" OR "WHOQOL-BREF" OR "15D" OR "EQ-5D" OR "EQ5D" OR "KDQOL-SF" OR "ADPKD-IS" OR "ADPKD-impact scale" OR "PKD-9 questionnaire" OR "SF12" OR "SF-6D" OR "HUI" OR "VAS" OR "EuroQol" OR "EuroQual").mp.
（“生活质量”或“生活质量”或HRQOL或QOL或“心理健康”或（（健康或功能*或精神或症状*）adj1（状态或评估*））或“自我报告*”或“患者*报告*”或“舞会”或“SF-36”或“WHOQOL-BREF”或“15D”或“EQ-5D”或“EQ5D”或“KDQOL-SF”或“ADPKD-IS”或“ADPKD影响量表”或“PKD-9问卷”或“SF12”或“SF-6D”或“HUI”或“VAS”或“EuroQol”或“EuroQual”）.mp。

3 1 and 2
3 1 和 2

4 3 not ("Qualitative Studies" or "Case Reports" or "Editorials" or "Commentaries" or "Conference Abstracts" or "Protocols").ti.
4 3 不是（“定性研究”或“病例报告”或“社论”或“评论”或“会议摘要”或“协议”）.ti。

5 limit 4 to (english language and yr="2014 -Current" and (adaptive clinical trial or classical article or clinical study or clinical trial, all or clinical trial, phase i or clinical trial, phase ii or clinical trial, phase iii or clinical trial, phase iv or clinical trial or comparative study or controlled clinical trial or "corrected and republished article" or evaluation study or journal article or meta analysis or multicenter study or observational study or pragmatic clinical trial or randomized controlled trial or "systematic review") and "humans only (removes records about animals)")
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1.(ADPKD or "Autosomal Dominant Polycystic Kidney" or (Dominant and (PKD or ((Renal or Kidney) adj1 (cyst* or multicystic or cystic or Polycystic))))).mp.
1.（ADPKD 或“常染色体显性遗传性多囊肾”或（显性和 （PKD 或（（肾脏或肾脏）adj1（囊肿* 或多囊性或囊性或多囊性))))).mp. 

2 ("life quality" or "quality of life" or HRQOL or QOL or "mental health" or ((health or function* or mental or symptom*) adj1 (status or assess*)) or "self report*" or "patient* report*" or "PROMs" OR "SF-36" OR "WHOQOL-BREF" OR "15D" OR "EQ-5D" OR "EQ5D" OR "KDQOL-SF" OR "ADPKD-IS" OR "ADPKD-impact scale" OR "PKD-9 questionnaire" OR "SF12" OR "SF-6D" OR "HUI" OR "VAS" OR "EuroQol" OR "EuroQual").mp.
2（“生活质量”或“生活质量”或 HRQOL 或 QOL 或“心理健康”或（（健康或功能*或精神或症状*）adj1（状态或评估*））或“自我报告*”或“患者*报告*”或“舞会”或“SF-36”或“WHOQOL-BREF”或“15D”或“EQ-5D”或“EQ5D”或“KDQOL-SF”或“ADPKD-IS”或“ADPKD-影响量表”或“PKD-9 问卷”或“SF12”或“SF-6D”或“hui”或“VAS”或“EuroQol”或“EuroQual”）.mp。

3. 1 and 2
3. 1 和 2 

4. 3 not ("Qualitative Studies" or "Case Reports" or "Editorials" or "Commentaries" or "Conference Abstracts" or "Protocols").ti.
4. 3 不是（“定性研究”或“病例报告”或“社论”或“评论”或“会议摘要”或“协议”）.ti。 

5. limit 4 to (all journals and human and english language and "0110 peer-reviewed journal" and yr="2014 -Current")
5. 限制 4 至 （所有期刊和人类和英语语言以及“0110 同行评审期刊” 和 yr=“2014 -Current”）

Embase via Ovid:
Embase 通过 Ovid：

1 (ADPKD or "Autosomal Dominant Polycystic Kidney" or (Dominant and (PKD or ((Renal or Kidney) adj1 (cyst* or multicystic or cystic or Polycystic))))).mp.
1 （ADPKD 或“常染色体显性遗传性多囊肾”或（显性和 （PKD 或（（肾脏或肾脏）adj1 （囊肿* 或多囊或囊性或多囊))))).mp.

2 ("life quality" or "quality of life" or HRQOL or QOL or "mental health" or ((health or function* or mental or symptom*) adj1 (status or assess*)) or "self report*" or "patient* report*" or "PROMs" OR "SF-36" OR "WHOQOL-BREF" OR "15D" OR "EQ-5D" OR "EQ5D" OR "KDQOL-SF" OR "ADPKD-IS" OR "ADPKD-impact scale" OR "PKD-9 questionnaire" OR "SF12" OR "SF-6D" OR "HUI" OR "VAS" OR "EuroQol" OR "EuroQual").mp.
2（“生活质量”或“生活质量”或 HRQOL 或 QOL 或“心理健康”或（（健康或功能*或精神或症状*）adj1（状态或评估*））或“自我报告*”或“患者*报告*”或“舞会”或“SF-36”或“WHOQOL-BREF”或“15D”或“EQ-5D”或“EQ5D”或“KDQOL-SF”或“ADPKD-IS”或“ADPKD-影响量表”或“PKD-9 问卷”或“SF12”或“SF-6D”或“hui”或“VAS”或“EuroQol”或“EuroQual”）.mp。

3 1 and 2
3 1 和 2 

4 3 not ("Qualitative Studies" or "Case Reports" or "Editorials" or "Commentaries" or "Conference Abstracts" or "Protocols").ti.
4 3 不是（“定性研究”或“病例报告”或“社论”或“评论”或“会议摘要”或“协议”）.ti。 

5 limit 4 to (human and english language and "remove medline records" and (article or article in press) and yr="2014 -Current")
5 将 4 限制为 （Human and English language and “remove medline records” and （article or article in press） and yr=“2014 -Current”）

CINAHL via EBSCO:
CINAHL 通过 EBSCO：

( ADPKD OR “Autosomal Dominant Polycystic Kidney” OR (Dominant AND (PKD OR ((Renal OR Kidney) N1 (cyst* OR multicystic OR cystic OR Polycystic)))) )
（ ADPKD OR “常染色体显性遗传性多囊肾” OR （显性遗传 AND（PKD OR （（肾脏 OR 肾脏） N1 （囊肿* OR 多囊性 OR 囊性 OR 多囊性）））） ）

AND
和

("life quality" OR "quality of life" OR HRQOL OR QOL OR "mental health" OR ((health OR function* OR mental OR symptom*) N1 (status OR assess*)) OR "self report*" OR "patient* report*" OR "PROMs" OR "SF-36" OR "WHOQOL-BREF" OR "15D" OR "EQ-5D" OR "EQ5D" OR "KDQOL-SF" OR "ADPKD-IS" OR "ADPKD-impact scale" OR "PKD-9 questionnaire" OR "SF12" OR "SF-6D" OR "HUI" OR "VAS" OR "EuroQol" OR "EuroQual")
（“生活质量”或“生活质量”或HRQOL或QOL或“心理健康”或（（健康或功能*或精神或症状*）N1（状态或评估*））或“自我报告*”或“患者*报告*”或“舞会”或“SF-36”或“WHOQOL-BREF”或“15D”或“EQ-5D”或“EQ5D”或“KDQOL-SF”或“ADPKD-IS”或“ADPKD影响量表”或“PKD-9问卷”或“SF12”或“SF-6D”或“hui”或“VAS”或“EuroQol”或“EuroQual”）

NOT
不

TI ( ("Qualitative Studies" OR "Case Reports" OR "Editorials" OR "Commentaries" OR "Conference Abstracts" OR "Protocols") )
TI （ （ （“定性研究” OR “病例报告” OR “社论” OR “评论” OR “会议摘要” OR “协议”） ）

Limiters - Publication Date: 20140101-20241231; English Language; Peer Reviewed; Exclude Pre-CINAHL; Exclude MEDLINE records; Human
限制器 - 发布日期：20140101-20241231;英语;同行评审;排除 Pre-CINAHL;排除 MEDLINE 记录;人

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web of science:
Web of Science：

TS=(ADPKD OR "Autosomal Dominant Polycystic Kidney" OR (Dominant AND (PKD OR ((Renal OR Kidney) NEAR/1 (cyst* OR multicyclic OR cystic OR Polycystic))))) AND TS=("life quality" OR "quality of life" OR HRQOL OR QOL OR "mental health" OR ((health OR function* OR mental OR symptom*) NEAR/1 (status OR assess*)) OR "self report*" OR "patient* report*" OR "PROMs" OR "SF-36" OR "WHOQOL-BREF" OR "15D" OR "EQ-5D" OR "EQ5D" OR "KDQOL-SF" OR "ADPKD-IS" OR "ADPKD-impact scale" OR "PKD-9 questionnaire" OR "SF12" OR "SF-6D" OR "HUI" OR "VAS" OR "EuroQol" OR "EuroQual")
TS=（ADPKD OR “常染色体显性遗传性多囊肾” OR （显性遗传 AND（PKD OR （（肾脏或肾脏） NEAR/1 （囊肿* 或多环 OR 囊性 OR 多囊性))))) AND TS=（“生活质量” 或 “生活质量” OR HRQOL 或 QOL 或“心理健康” 或 （（健康或功能* 或精神 OR 症状*） NEAR/1 （状态或评估*）） 或“自我报告*” 或“患者* 报告*” 或“舞会” 或 “SF-36” 或“WHOQOL-BREF” 或“15D” 或 “EQ-5D” 或 “EQ5D” 或 “KDQOL-SF” 或 “ADPKD-IS” 或 ”ADPKD 影响量表“或”PKD-9 问卷“或”SF12“或”SF-6D“或”hui“或”VAS“或”EuroQol“或”EuroQual”）

AND LA=(English) AND PY=(2014-2024) and Preprint Citation Index (Exclude – Database) and MEDLINE® (Exclude – Database) and Article or Review Article or Early Access (Document Types) and Book or Meeting or Editorial Material (Exclude – Document Types)
AND LA=（英文） AND PY=（2014-2024） 和预印本引文索引 （Exclude – 数据库） 和 MEDLINE® （Exclude – 数据库） 和文章或评论文章或早期访问（文献类型）和书籍或会议或编辑材料（Exclude – 文献类型）

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Search "ADPKD HRQoL" and retrieve the first 30 hits.
搜索 “ADPKD HRQoL” 并检索前 30 个结果。