今日の臨床サポート

水腎症

著者: 和田耕一郎 島根大学医学部 泌尿器科学講座

監修: 中川昌之 公益財団法人 慈愛会 今村総合病院 泌尿器科顧問

著者校正/監修レビュー済:2022/07/06
参考ガイドライン:
  1. 日本小児泌尿器科学会:小児先天性水腎症(腎盂尿管移行部通過障害)診療手引き 2016
患者向け説明資料

概要・推奨   

  1. 急性と慢性の水腎症では症状が異なり、急性の場合には腰背部・側腹部痛を伴うことが多いが、腫瘍や下部尿路閉塞に伴って緩徐に生じる水腎症は無症状なこともある。
  1. 水腎症のうち、腎機能低下、高カリウム血症、感染を伴う場合は致命的となりうるので、診断と治療には特に急を要する。
  1. 腰背部痛や側腹部痛では水腎症を念頭に置き、まず超音波検査を行い、尿路閉塞の位置と原因を同定するには単純CTを撮影する。
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
和田耕一郎 : 未申告[2022年]
監修:中川昌之 : 特に申告事項無し[2022年]

改訂のポイント:
  1. 水腎症に特化したガイドラインはなく、新生児や小児は日本小児泌尿器科学会の診療手引きを盛り込んだ。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 水腎症は、泌尿器科医から救急外来を含めた内科・外科系医師が遭遇する可能性がある一般的な病態である。
  1. 腎で産生された尿が、機能的あるいは解剖学的な尿路通過障害により停滞し、腎盂・腎杯が拡張した状態を指す。
 
水腎症の腹部超音波所見

A:右腎のcentral echo complexの解離が明らかで水腎症と診断される。
B:同一症例の超音波ドプラ法による画像で、拡張した腎杯周囲の実質には血流が保たれている。

出典

img1:  椎名浩昭先生ご提供
 
 
 
  1. 腎盂内圧の上昇は尿細管機能、糸球体濾過能、腎血流量に影響するが、急性水腎症では腎実質の解剖学的障害はない。慢性水腎症では、腎乳頭の圧迫、腎杯周囲の腎実質の菲薄化、腎杯間の隔壁の癒着が進行性に生じ、腎皮質の萎縮と腎機能低下を認める。
  1. 急性で短期間の水腎症では腎機能障害は可逆的であるが、慢性水腎症では不可逆的な尿細管萎縮とネフロン損失に至る。
  1. 水腎症の病因と症状は、先天性(新生児および子ども)と後天性(成人)では大きく異なる。
  1. 新生児の腎盂拡張は一過性のことが多いが、尿路閉塞や膀胱尿管逆流がその原因であることがある。
  1. 後天性水腎症の場合、若年層では結石が水腎症の一般的な原因である。
  1. 妊娠や婦人科腫瘍に関連して20~60歳までは女性に多い。男性では、60歳以降の罹患率の上昇は前立腺疾患との関連が示唆される。
問診・診察のポイント  
  1. 背部痛や側腹部痛では水腎症を念頭に置き、疼痛の原因と尿路閉塞の関連性を検討する。

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文献 

E D Vaughan, F R Bühler, J H Laragh
Normal renin secretion in hypertensive patients with primarily unilateral chronic hydronephrosis.
J Urol. 1974 Aug;112(2):153-6.
Abstract/Text
PMID 4843320
Michiel F Schreuder, Rik Westland, Joanna A E van Wijk
Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney.
Nephrol Dial Transplant. 2009 Jun;24(6):1810-8. doi: 10.1093/ndt/gfn777. Epub 2009 Jan 26.
Abstract/Text BACKGROUND: Many papers are published on cohorts with unilateral multicystic dysplastic kidney (MCDK) patients, but show variable results as to the incidence of associated urinary tract abnormalities. The objective of this study was to describe the status of the urinary tract, including contralateral hypertrophy and malformations, in patients with unilateral MCDK based on a meta-analysis of the literature, taking into account the timing of diagnosis (pre- versus postnatal) as a possible source of bias.
METHODS: A systematic review of the scientific literature in English was conducted using PubMed and Embase. A meta-analysis was performed with the studies that were identified using our reproducible search.
RESULTS: Based on analysis of the data in 19 populations, the overall incidence of unilateral MCDK is 1 in 4300 with an increasing trend over the years. A total of 67 cohorts with over 3500 patients with unilateral MCDK were included in the meta-analysis. Fifty-nine percent of patients were male and the MCDKs were significantly more often found on the left side (53.1%). Associated anomalies in the solitary functioning kidney were found in 1 in 3 patients, mainly vesicoureteric reflux (VUR, in 19.7%). In patients with VUR, 40% have severe contralateral VUR, defined as grade III-V. Contralateral hypertrophy, present in 77% of patients after a follow-up of at least 10 years, showed a trend to be less pronounced in patients with VUR. Timing of the diagnosis of MCDK did not essentially influence the results.
CONCLUSIONS: These aggregate results provide insight into the incidence, demographic data and associated anomalies in patients with unilateral MCDK. One in three patients with unilateral MCDK show anomalies in the contralateral, solitary functioning kidney. However, studies into the long-term consequences of these anomalies are scarce.

PMID 19171687
Kirsten Y Renkema, Paul J Winyard, Ilya N Skovorodkin, Elena Levtchenko, An Hindryckx, Cécile Jeanpierre, Stefanie Weber, Rémi Salomon, Corinne Antignac, Seppo Vainio, Andreas Schedl, Franz Schaefer, Nine V A M Knoers, Ernie M H F Bongers, EUCAKUT consortium
Novel perspectives for investigating congenital anomalies of the kidney and urinary tract (CAKUT).
Nephrol Dial Transplant. 2011 Dec;26(12):3843-51. doi: 10.1093/ndt/gfr655.
Abstract/Text Congenital anomalies of the kidney and urinary tract (CAKUT) are the commonest cause of chronic kidney disease in children. Structural anomalies within the CAKUT spectrum include renal agenesis, kidney hypo-/dysplasia, multicystic kidney dysplasia, duplex collecting system, posterior urethral valves and ureter abnormalities. While most CAKUT cases are sporadic, familial clustering of CAKUT is common, emphasizing a strong genetic contribution to CAKUT origin. Animal experiments demonstrate that alterations in genes crucial for kidney development can cause experimental CAKUT, while expression studies implicate mislocalization and/or aberrant levels of the encoded proteins in human CAKUT. Further insight into the pathogenesis of CAKUT will improve strategies for early diagnosis, follow-up and treatment. Here, we outline a collaborative approach to identify and characterize novel factors underlying human CAKUT. This European consortium will share the largest collection of CAKUT patients available worldwide and undertake multidisciplinary research into molecular and genetic pathogenesis, with extension into translational studies to improve long-term patient outcomes.

PMID 22121240
A Taylor, S Clark, T Ball
Comparison of Tc-99m MAG3 and Tc-99m DTPA scintigraphy in neonates.
Clin Nucl Med. 1994 Jul;19(7):575-80.
Abstract/Text Tc-99m MAG3 scans were obtained in two neonates in whom previous Tc-99m DTPA scans had been interpreted as showing possible obstruction. In both patients, Tc-99m MAG3 provided superior diagnostic images and allowed obstruction to be excluded. The favorable dosimetry of MAG3, coupled with the fact that its clearance is two to three times higher than that of DTPA, makes MAG3 an excellent radiopharmaceutical for pediatric patients, particularly in those patients with impaired renal function and possible obstruction.

PMID 7924094
Abstract/Text OBJECTIVE: To present an explanation for impaired drainage on diuretic renography in infants with a prenatal diagnosis of unilateral renal pelvic dilatation (RPD) who are stable both in terms of renal function and dilatation on follow-up investigations, despite undergoing no surgical intervention, and who undergo diuretic renography postnatally; this should provide some insight into whether impaired drainage is a sign of obstruction in these asymptomatic infants.
MATERIALS AND METHODS: Using a combination of published studies on the technique of diuretic renography and an analysis of such studies, a theoretical model of the infant kidney was developed to assess the possibility of prolonged drainage.
RESULTS: The results from the model showed prolonged drainage in many different situations, thus offering an explanation of expected impaired drainage even if there was no obstruction.
CONCLUSION: Understanding the pathophysiology of prenatally diagnosed RPD allows different interpretations of the diuretic renogram and may affect the treatment of these children.

PMID 11298056
I Gordon, H K Dhillon, H Gatanash, A M Peters
Antenatal diagnosis of pelvic hydronephrosis: assessment of renal function and drainage as a guide to management.
J Nucl Med. 1991 Sep;32(9):1649-54.
Abstract/Text The optimal management of children with antenatally diagnosed unilateral hydronephrotic kidneys is unclear. We have monitored renal function and drainage using 99mTc-DTPA renography in 77 children with such kidneys presenting at 1-14 mo of age with the aims of throwing light on the natural history of the condition and evaluating the response of the kidney to pyeloplasty. Since management was independent of the drainage criteria on renography, the opportunity also was presented to evaluate the efficacy of diuretic renography for diagnosing obstruction in these children. Follow-up ranged between 1 and 6 yr, during which renography was performed between 2 and 6 times in each patient. Thirty-nine patients were treated conservatively, while 30 patients underwent pyeloplasty at varying times after presentation. No overall significant change in function was observed either in the nonoperated children or as a result of surgery. This also applied to sub-groups of children with poor drainage. Function improved significantly, however, in a sub-group of nonoperated children who presented with impaired function in the hydronephrotic kidney. Surgery, although having no overall effect on function, significantly improved drainage. As a means of identifying obstruction, 99mTc-DTPA diuretic renography was unreliable, erroneously indicating obstruction in 24 nonoperated children in whom function of the hydronephrotic kidney never became abnormal and who could not, therefore, have been considered to have obstruction. This retrospective study underlines the importance of performing a randomized prospective study on the value of pyeloplasty in this group of children.

PMID 1880563
John R Leyendecker, Craig E Barnes, Ronald J Zagoria
MR urography: techniques and clinical applications.
Radiographics. 2008 Jan-Feb;28(1):23-46; discussion 46-7. doi: 10.1148/rg.281075077.
Abstract/Text Magnetic resonance (MR) urography comprises an evolving group of techniques with the potential for allowing optimal noninvasive evaluation of many abnormalities of the urinary tract. MR urography is clinically useful in the evaluation of suspected urinary tract obstruction, hematuria, and congenital anomalies, as well as surgically altered anatomy, and can be particularly beneficial in pediatric or pregnant patients or when ionizing radiation is to be avoided. The most common MR urographic techniques for displaying the urinary tract can be divided into two categories: static-fluid MR urography and excretory MR urography. Static-fluid MR urography makes use of heavily T2-weighted sequences to image the urinary tract as a static collection of fluid, can be repeated sequentially (cine MR urography) to better demonstrate the ureters in their entirety and to confirm the presence of fixed stenoses, and is most successful in patients with dilated or obstructed collecting systems. Excretory MR urography is performed during the excretory phase of enhancement after the intravenous administration of gadolinium-based contrast material; thus, the patient must have sufficient renal function to allow the excretion and even distribution of the contrast material. Diuretic administration is an important adjunct to excretory MR urography, which can better demonstrate nondilated systems. Static-fluid and excretory MR urography can be combined with conventional MR imaging for comprehensive evaluation of the urinary tract. The successful interpretation of MR urographic examinations requires familiarity with the many pitfalls and artifacts that can be encountered with these techniques.

PMID 18203929
Rose de Bruyn, Stephen D Marks
Postnatal investigation of fetal renal disease.
Semin Fetal Neonatal Med. 2008 Jun;13(3):133-41. doi: 10.1016/j.siny.2007.10.008. Epub 2008 Jan 14.
Abstract/Text Following the introduction of routine prenatal scanning, and more recently detailed anomaly scans, antenatal ultrasound (US) diagnosis of fetal renal tract abnormalities has become well established. Prenatal detection has resulted in a group of asymptomatic infants becoming patients in the last two decades. These infants are referred for paediatric (general, urological and nephrological) consultations, radiological imaging and monitoring. The urgency with which to undertake imaging depends on the suspected antenatal diagnosis and clinical scenario, with bilateral hydronephrosis, posterior urethral valves and complicated duplex systems taking a high priority. Treatment is mainly preventive and relies on close follow-up and timely intervention. US remains the main imaging modality of choice postnatally; together with nuclear medicine, it gives a powerful combination of both anatomy and function. However, magnetic resonance urography may have increasing importance in becoming the investigation of choice of the future for more complex cases.

PMID 18249044
J Damien Grattan-Smith, Stephen B Little, Richard A Jones
MR urography evaluation of obstructive uropathy.
Pediatr Radiol. 2008 Jan;38 Suppl 1:S49-69. doi: 10.1007/s00247-007-0667-y. Epub 2007 Dec 11.
Abstract/Text Hydronephrosis and the evaluation of obstructive uropathy are the most common indications for MR urography in our practice. Typically our patients fall into one of two groups: infants with antenatal hydronephrosis and older children who present with abdominal pain, hematuria or urinary tract infection. Obstruction in children is usually chronic and partial. Intermittent episodes of increased pressure occur when the urine production exceeds the capacity for drainage. MR urography uses a fluid and diuretic challenge to assess the hydronephrotic kidney. High-quality anatomic images provide a morphologic assessment of the hydronephrotic system. Although it is relatively straightforward to determine if a system is not obstructed on the basis of the renal transit time (RTT), no single parameter is adequate to fully characterize obstruction. By evaluating the changes in signal intensity in the renal parenchyma following contrast administration, the hydronephrotic systems are classified as compensated or decompensated. Delayed RTT and the presence of urine-contrast levels indicate stasis. Calyceal transit time and the difference between the volumetric and the Patlak differential renal function (vDRF-pDRF) are measures of the physiologic changes within the kidney. Additionally, MR urography provides prognostic information by assessing the quality of the renal parenchyma and identifying uropathy preoperatively. MR urography combines both anatomic and functional information in a single test and is capable of providing a comprehensive evaluation of obstructive uropathy that could ultimately help select those patients most likely to benefit from surgical intervention.

PMID 18071689
Carmelo Mamì, Antonina Paolata, Antonella Palmara, Teresa Marrone, Luca F Berte, Lucia Marseglia, Francesco Arena, Rosa Manganaro
Outcome and management of isolated moderate renal pelvis dilatation detected at postnatal screening.
Pediatr Nephrol. 2009 Oct;24(10):2005-8. doi: 10.1007/s00467-009-1229-3. Epub 2009 Jul 7.
Abstract/Text The aim of this study was to evaluate the incidence and outcome of isolated moderate renal pelvis dilatation (RPD) [anterior-posterior diameter (APD) 10-15 mm] in an unselected population of 2-month-old infants prospectively followed for up to 12-14 months of life. Isolated moderate renal pelvis dilatation was detected in 282 of the 11,801 (2.4%), infants screened; 240 infants with normal renal ultrasound were enrolled as the control group. Resolution of RPD was considered when an APD
PMID 19582482

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