Kidney function in adults born with unilateral renal agenesis or nephrectomized in childhood

Wikstad, I.; Celsi, Gianni; Larsson, Lars; Herin, Peter; Aperia, Anita

doi:10.1007/bf00862585

Cited by 113 publications

(58 citation statements)

References 22 publications

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“…Finally, 6% of children already had CKD stage 3 or higher during follow-up (congenital 4% versus acquired 9%; P=0.05). Other studies that have included solitary functioning kidney patients with a normal renal ultrasound at diagnosis (suggesting absence of ipsilateral CAKUT) report a relatively mild renal outcome (34,40,42,44). Although the exact proportion of individuals with CKD needs to be determined, the increasing number of studies illustrates that a solitary functioning kidney should not be assumed to be harmless.…”

Section: Clinical Outcomementioning

confidence: 99%

Clinical Implications of the Solitary Functioning Kidney

Westland

Schreuder

Goudoever

et al. 2014

Clinical Journal of the American Society of Nephrology

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Congenital anomalies of the kidney and urinary tract are the major cause of ESRD in childhood. Children with a solitary functioning kidney form an important subgroup of congenital anomalies of the kidney and urinary tract patients, and a significant fraction of these children is at risk for progression to CKD. However, challenges remain in distinguishing patients with a high risk for disease progression from those patients without a high risk of disease progression. Although it is hypothesized that glomerular hyperfiltration in the lowered number of nephrons underlies the impaired renal prognosis in the solitary functioning kidney, the high proportion of ipsilateral congenital anomalies of the kidney and urinary tract in these patients may further influence clinical outcome. Pathogenic genetic and environmental factors in renal development have increasingly been identified and may play a crucial role in establishing a correct diagnosis and prognosis for these patients. With fetal ultrasound now enabling prenatal identification of individuals with a solitary functioning kidney, an early evaluation of risk factors for renal injury would allow for differentiation between patients with and without an increased risk for CKD. This review describes the underlying causes and consequences of the solitary functioning kidney from childhood together with its clinical implications. Finally, guidelines for follow-up of solitary functioning kidney patients are recommended.

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Section: Clinical Outcomementioning

confidence: 99%

Clinical Implications of the Solitary Functioning Kidney

Westland

Schreuder

Goudoever

et al. 2014

Clinical Journal of the American Society of Nephrology

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“…This clinical observation has led to a proposal that remnant glomeruli of maturing kidneys are uniquely susceptible to the development of focal glomerular sclerosis. Unequivocal evidence that the young are indeed predisposed to glomerular damage after nephron loss has recently been obtained from the test in humans of renal function after nephrectomy in childhood versus unilateral renal agenesis by Wikstad et al (25), and histologic demonstration in experimental animals by Celsi et al (26) and O'Donnell et al (27). In contrast to the very good prognosis with normal renal function after loss of a kidney from trauma or transplant donation in the adult, the 15 patients with unilateral renal agenesis studied by Wikstad et al (25) showed a decline in inulin clearance rates, and nearly half developed microalbuminuria.…”

mentioning

confidence: 99%

“…Unequivocal evidence that the young are indeed predisposed to glomerular damage after nephron loss has recently been obtained from the test in humans of renal function after nephrectomy in childhood versus unilateral renal agenesis by Wikstad et al (25), and histologic demonstration in experimental animals by Celsi et al (26) and O'Donnell et al (27). In contrast to the very good prognosis with normal renal function after loss of a kidney from trauma or transplant donation in the adult, the 15 patients with unilateral renal agenesis studied by Wikstad et al (25) showed a decline in inulin clearance rates, and nearly half developed microalbuminuria. In light of the above-discussed mechanisms previously tested in a variety of experimental settings, this predisposition of young glomeruli may be attributed to exaggerated hemodynamic abnormality and/or glomerular hypertrophy that follow nephron loss.…”

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confidence: 99%

Mechanism of the Unique Susceptibility of Deep Cortical Glomeruli of Maturing Kidneys to Severe Focal Glomerular Sclerosis

et al. 1990

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ABSTRACT. The alterations in single glomerular hemodynamics, glomerular size, and development of glomerular sclerosis after subtotal nephrectomy were assessed in -32-d-old young and >3-mo-old adult Munich-Wistar rats. In 6 wk, young rats developed more pronounced glomerular sclerosis and more marked elevation in blood urea nitrogen. The deep (versus superficial) cortical region of young rats was characterized by having a greater number of glomeruli with advanced sclerosis. Single nephron glomerular filtration rate of superficial glomeruli of the young increased to a much greater extent than whole kidney glomerular filtration rate, whereas there were comparable post-subtotal nephrectomy increases in whole kidney glomerular filtration rate in these two age groups, indicating that the deep glomeruli were exposed to a lesser hemodynamic load than were the superficial. Since the remnant nephrons of young and adult rats achieved equally high glomerular pressures and comparably large glomerular size shortly after subtotal nephrectomy, the unique susceptibility of young glomeruli to sclerosis is attributed to the intrinsic property of these glomeruli, rather than the abnormal hemodynamics or stimuli promoting hypertrophy and mesangial matrix accumulation imposed upon the glomeruli. (Pediatr Res 28: 270-276,1990) Abbreviations BUN, blood urea nitrogen BW, body weight GFR, glomerular filtration rate PAmean, mean planar area of glomeruli Pee, glomerular capillary pressure SBP, systolic blood pressure SNGFR, single nephron glomerular filtration rate sNPX, subtotal nephrectomy In view of the marked functional and structural internephron heterogeneity seen in chronically diseased kidneys in both humans and animals ( 1 -4), various initial immune or nonimmune insults appear to cause loss of a selected population of functioning nephrons. Extensive nephron population loss leads to functional

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“…Bununla birlikte, tek böbrekli hastalarda karşı böbreğin sağlam olduğu durumda kan basıncı ve renal fonksiyonlarının kontrol grubu ile benzer olduğunu gösteren çalışmalar da mevcuttur (20,21) . Bu çalışmada her iki grupta renal fonksiyon bozukluğu saptanmadı.…”

Section: Discussionunclassified

Clinical characteristics and follow-up results of unilateral renal agenesis and hypoplasia of children

Evrengül¹,

Ertan²,

Serdaroğlu³

et al. 2016

BUCH

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ÖZAmaç: Az nefron sayısına sahip çocuklarda, yaşamlarının ilerleyen dönemlerinde hipertansiyon, proteinüri ve kronik böbrek yetmezliği gelişme riski yüksektir. Bu renal hasar, glomerüler hiperfiltrasyondam kaynaklanmaktadır. Bu çalışmanın amacı, tek taraflı agenezik ve hipoplazik böbreği olan çocukların klinik özelliklerinin ve izlem sonuçlarının değerlendirilmesidir. Yöntem: Celal Bayar Üniversitesi Tıp Fakültesi ve Dr. Behçet Uz Çocuk Hastanesinde takip edilen, tek taraflı renal agenezisi ve hipoplazisi olan hastaların klinik, radyolojik ve nükleer tıp inceleme bulguları değerlendirildi. Bulgular: Bu çalışma ortalama yaşı 8,9±4 yıl olan 30 (%60) erkek ve 20 (%40) kız hastadan oluşmaktaydı. Yirmi beş hasta renal agenezi, 25 hasta renal hipoplazi tanısı aldı. Laboratuvar incelemesinde ortalama serum kreatinin düzeyi 0,45±0,22 mg/dl ve tahmin edilen glomerüler filtrasyon hızı (eGFR) 144±30,3 ml/dk. idi. Yirmi dokuz (%58) hastada hiperfiltrasyon saptandı. Proteinüri ve hipertansiyonu olan 1 (%4) hasta vardı. On beş (%30) hastada ürolojik anamoli mevcuttu. Sonuç: Bu çalışmada, tek taraflı renal agenezisi ve hipoplazisi olan hastaların serum kreatinin düzeyi normal bulundu. Ancak bu hastaların 29 (%58) hiperfiltrasyon vardı. Hiperfiltasyonu olan çocukların çoğunda yaşamlarının ilerleyen döneminde renal hasar geliştiği için bu çocuk-ların doğumdan itibararen klinik takiplerinin yapılması gerekmektedir.Anahtar kelimeler: Konjenital böbrek hastalıkları, renal agenezi, renal hipoplazi ABSTRACT Objective: Children with an inadequate number of nephrons have an increased risk of developing hypertension, proteinuria and chronic kidney disease in later life. This renal injury is caused by glomerular hyperfiltration. The aim of this study is to evaluate clinical characteristics and follow-up results of the children with unilateral renal agenesis and hypoplasia. Methods: We evaluated the clinical, radiological, and nuclear medicine findings of the children with unilateral agenetic or hypoplastic kidneys who were followed at Celal Bayar University School of Medicine and Dr. Behcet Uz Children's Hospital between 2005 and 2012. Results: The study consisted of 30 (60%) males and 20 (40%) females with a mean age of 8.9±4 years. A total of 25 patients were with renal agenesis and 25 with renal hypoplasia were diagnosed. On laboratory evaluation, mean serum creatinine level was 0.45±0.22 mg/ dl and the mean estimated glomerular filtration rate (eGFR) was 144±30.3 ml/min. 29 patient (58%) had hyperfiltration according to an eGFR. Proteinuria and hypertension were noted in 1 (4%) patient. Urological anomalies were found in 15 patients (30%). Conclusion: Our study has determined that children with unilateral renal agenesis and hypoplasia had normal serum creatinine values. Howewer 29 patients (58%) had hyperfiltration according to eGFR. Because many children with hiperfiltration develop renal injury in later life, we emphasize the need for clinical follow-up in these patients starting at birth.

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Kidney function in adults born with unilateral renal agenesis or nephrectomized in childhood

Cited by 113 publications

References 22 publications

Clinical Implications of the Solitary Functioning Kidney

Clinical Implications of the Solitary Functioning Kidney

Mechanism of the Unique Susceptibility of Deep Cortical Glomeruli of Maturing Kidneys to Severe Focal Glomerular Sclerosis

Clinical characteristics and follow-up results of unilateral renal agenesis and hypoplasia of children

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