Kim Hyunsuk, Park Hayne Cho, Ryu Hyunjin, Kim Kiwon, Kim Hyo Sang, Oh Kook-Hwan, Yu Su Jong, Chung Jin Wook, Cho Jeong Yeon, Kim Seung Hyup, Cheong Hae Il, Lee Kyubeck, Park Jong Hoon, Pei York, Hwang Young-Hwan, Ahn Curie
Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
Department of Internal Medicine, Armed Forces Capital Hospital, Seongnam-si, Gyeonggi-do, Korea.
PLoS One. 2015 Dec 7;10(12):e0144526. doi: 10.1371/journal.pone.0144526. eCollection 2015.
Mass effect from polycystic kidney and liver enlargement can result in significant clinical complications and symptoms in autosomal dominant polycystic kidney disease (ADPKD). In this single-center study, we examined the correlation of height-adjusted total liver volume (htTLV) and total kidney volume (htTKV) by CT imaging with hepatic complications (n = 461) and abdominal symptoms (n = 253) in patients with ADPKD. "Mass-effect" complications were assessed by review of medical records and abdominal symptoms, by a standardized research questionnaire. Overall, 91.8% of patients had 4 or more liver cysts on CT scans. Polycystic liver disease (PLD) was classified as none or mild (htTLV < 1,600 mL/m); moderate (1,600 ≤ htTLV <3,200 mL/m); and severe (htTLV ≥ 3,200 mL/m). The prevalence of moderate and severe PLD in our patient cohort was 11.7% (n = 54/461) and 4.8% (n = 22/461), respectively, with a female predominance in both the moderate (61.1%) and severe (95.5%) PLD groups. Pressure-related complications such as leg edema (20.4%), ascites (16.6%), and hernia (3.6%) were common, and patients with moderate to severe PLD exhibited a 6-fold increased risk (compared to no or mild PLD) for these complications in multivariate analysis. Similarly, abdominal symptoms including back pain (58.8%), flank pain (53.1%), abdominal fullness (46.5%), and dyspnea/chest-discomfort (44.3%) were very common, and patients with moderate to severe PLD exhibited a 5-fold increased risk for these symptoms. Moderate to severe PLD is a common and clinically important problem in ~16% of patients with ADPKD who may benefit from referral to specialized centers for further management.
多囊肾和肝脏肿大引起的占位效应可导致常染色体显性多囊肾病(ADPKD)出现严重的临床并发症和症状。在这项单中心研究中,我们通过CT成像检查了身高校正后的肝脏总体积(htTLV)和肾脏总体积(htTKV)与ADPKD患者肝脏并发症(n = 461)和腹部症状(n = 253)之间的相关性。通过查阅病历和腹部症状,采用标准化研究问卷对“占位效应”并发症进行评估。总体而言,91.8%的患者CT扫描显示有4个或更多肝囊肿。多囊肝病(PLD)分为无或轻度(htTLV < 1600 mL/m);中度(1600≤htTLV < 3200 mL/m);重度(htTLV≥3200 mL/m)。在我们的患者队列中,中度和重度PLD的患病率分别为11.7%(n = 54/461)和4.8%(n = 22/461),中度(61.1%)和重度(95.5%)PLD组均以女性为主。腿部水肿(20.4%)、腹水(16.6%)和疝气(3.6%)等与压力相关的并发症很常见,在多变量分析中,中度至重度PLD患者出现这些并发症的风险增加了6倍(与无或轻度PLD相比)。同样,背部疼痛(58.8%)、胁腹疼痛(53.1%)、腹部胀满(46.5%)和呼吸困难/胸部不适(44.3%)等腹部症状也很常见,中度至重度PLD患者出现这些症状的风险增加了5倍。中度至重度PLD在约16%的ADPKD患者中是一个常见且具有临床重要性的问题,这些患者可能受益于转诊至专科中心进行进一步治疗。