Outcomes of a Cohort of Prenatally Diagnosed and Early Enrolled Patients with Congenital Solitary Functioning Kidney

Marzuillo, Pierluigi; Guarino, Stefano; Grandone, Anna; Somma, Allegra Di; Vecchia, Nicoletta Della; Esposito, Tiziana; Macchini, Giulia; Marotta, Rosaria; Apicella, Andrea; Diplomatico, Mario; Rambaldi, Pier Francesco; Perrone, Laura; Giudice, Emanuele Miraglia del; Manna, Angela La; Polito, Cesare

doi:10.1016/j.juro.2017.05.076

Cited by 39 publications

(43 citation statements)

References 18 publications

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“…Since a decrease in kidney function is expected later in the course of hyperfiltration injury, serum creatinine measurement can be performed less frequently than screening for hypertension and albuminuria. Indeed, cSFK cohorts published to date showed that an isolated decrease in estimated GFR (eGFR) occurred in only 0.3–8% of their population and may especially occur during puberty [6] , [7] , [52] , [53] , [54] . Therefore, it seems reasonable to monitor eGFR once every 5 yr until the onset of puberty and every 2 yr thereafter.…”

Section: Evidence Synthesismentioning

confidence: 99%

“…Living with a cSFK predisposes to hypertension, proteinuria, and kidney function loss [4] , [5] , [6] . The magnitude of the risk of living with a cSFK is still a topic of debate, with kidney injury rates ranging from 6% to 60% at age 15 and limited studies in adulthood [6] , [7] . In addition, large differences exist in the management of this condition.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations

Woud

Westland

Feitz

et al. 2021

European Urology Open Science

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Context A congenital solitary functioning kidney (cSFK) is a common developmental defect that predisposes to hypertension and chronic kidney disease (CKD) as a consequence of hyperfiltration. Every urologist takes care of patients with a cSFK, since some will need lifelong urological care or will come with clinical problems or questions to an adult urologist later in life. Objective We aim to provide clear recommendations for the initial clinical management and follow-up of children with a cSFK. Evidence acquisition PubMed and EMBASE were searched to identify relevant publications, which were combined with guidelines on related topics and expert opinion. Evidence synthesis Initially, cSFK diagnosis should be confirmed and risk factors for kidney injury should be identified using ultrasound. Although more research into early predictors of kidney injury is needed, additional congenital anomalies of the kidney or urinary tract and absence of compensatory kidney hypertrophy have repeatedly been associated with a worse prognosis. The role of voiding cystourethrography and antibiotic prophylaxis remains controversial, and is complicated by the exclusion of children with a cSFK from studies. A yearly follow-up for signs of kidney injury is recommended for children with a cSFK. As masked hypertension is prevalent, annual ambulatory blood pressure measurement should be considered. During puberty, an increasing incidence of kidney injury is seen, indicating that long-term follow-up is necessary. If signs of kidney injury are present, angiotensin converting enzyme inhibitors are the first-line drugs of choice. Conclusions This overview points to the urological and medical clinical aspects and long-term care guidance for children with a cSFK, who are at risk of hypertension and CKD. Monitoring for signs of kidney injury is therefore recommended throughout life. Large, prospective studies with long-term follow-up of clearly defined cohorts are still needed to facilitate more risk-based and individualized clinical management. Patient summary Many children are born with only one functioning kidney, which could lead to kidney injury later in life. Therefore, a kidney ultrasound is made soon after birth, and other investigations may be needed as well. Urologists taking care of patients with a solitary functioning kidney should realize the long-term clinical aspects, which might need medical management.

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Section: Evidence Synthesismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations

Woud

Westland

Feitz

et al. 2021

European Urology Open Science

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“…In other studies the prognosis for children with a congenital SFK is suggested to be more favorable. Indeed, in a recent retrospective study of ∼300 children prenatally diagnosed with a congenital SFK, only 3.9% had signs of kidney injury by a median age of 7 years ( Marzuillo et al, 2017 ). Similarly, La Scola et al (2016) observed that in a cohort of 146 children with a congenital SFK, only 5% of patients had hypertension at a mean age of 12 years and only 4% presented with proteinuria at a mean age of 6 years.…”

Section: Clinical Outcomesmentioning

confidence: 99%

Physiology and Pathophysiology of Compensatory Adaptations of a Solitary Functioning Kidney

et al. 2020

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Children born with a solitary functioning kidney (SFK) have an increased risk of hypertension and kidney disease from early in adulthood. In response to a reduction in kidney mass, the remaining kidney undergoes compensatory kidney growth. This is associated with both an increase in size of the kidney tubules and the glomeruli and an increase in single nephron glomerular filtration rate (SNGFR). The compensatory hypertrophy and increase in filtration at the level of the individual nephron results in normalization of total glomerular filtration rate (GFR). However, over time these same compensatory mechanisms may contribute to kidney injury and hypertension. Indeed, approximately 50% of children born with a SFK develop hypertension by the age of 18 and 20–40% require dialysis by the age of 30. The mechanisms that result in kidney injury are only partly understood, and early biomarkers that distinguish those at an elevated risk of kidney injury are needed. This review will outline the compensatory adaptations to a SFK, and outline how these adaptations may contribute to kidney injury and hypertension later in life. These will be based largely on the mechanisms we have identified from our studies in an ovine model of SFK, that implicate the renal nitric oxide system, the renin angiotensin system and the renal nerves to kidney disease and hypertension associated with SFK. This discussion will also evaluate current, and speculate on next generation, prognostic factors that may predict those children at a higher risk of future kidney disease and hypertension.

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“…Over half of pregnancies involving severe CAKUTs have adverse birth outcomes, such as stillbirth or even spontaneous abortion [6]. Among live births, CAKUTs are closely associated with impaired renal function, particularly chronic kidney and kidney-related diseases in young adults [7–10]. For example, approximately 39.8% of end-stage renal disease cases in patients younger than 20 years old in Japan are owing to CAKUTs [9].…”

Section: Introductionmentioning

confidence: 99%

Prevalence, types, and malformations in congenital anomalies of the kidney and urinary tract in newborns: a retrospective hospital-based study

Chen

et al. 2019

Ital J Pediatr

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Background Congenital anomalies of the kidney and urinary tract (CAKUTs) are some of the most common birth defects affecting newborns. CAKUTs often have poor birth outcomes owing to the limited experience of physicians in developing countries regarding antenatal and postnatal diagnosis. We aimed to estimate the epidemiology of CAKUTs using data from a hospital-based registry in Zhejiang Province, China. Methods We included a total of 2790 newborns with CAKUTs, identified among 1,748,038 births during 2010–2016. The prevalence and type of CAKUTs, maternal and neonatal characteristics, and associated malformations were analyzed. Results The average prevalence of CAKUTs born to mothers overall and mothers aged ≥35 years were both around 1.60 per 1000 births (95% confidence interval (CI), 1.54–1.66; 95% CI, 1.44–1.83, respectively) during the study period. The prevalence of CAKUTs changed over time among all women and women of advanced maternal age, although no significant trends were observed. CAKUTs were more likely to occur in male than female newborns (odds ratio (OR) 1.28, 95% CI 1.18–1.38), in multiple births than singletons (OR 1.53, 95% CI 1.21–1.92) and in urban areas than rural areas (OR 1.27, 95% CI 1.18–1.37). The overall prenatal detection rate of CAKUTs was 73.87%. The average gestational age at antenatal diagnosis was 26.57 ± 8.70 weeks. A total 22.69% CAKUTs had associated malformations. Congenital heart defects were the most common anomalies, accounting for 8.89% of the whole population. The main proportion in subgroups was hydronephrosis, representing 31.79% of registered CAKUTs. Conclusions There was a nearly twofold increase in the prevalence of CAKUTs from 2010 to 2016 in Zhejiang Province. CAKUTs are strongly associated with male sex, multiple births, urban areas, and other nonurinary congenital malformations.

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Outcomes of a Cohort of Prenatally Diagnosed and Early Enrolled Patients with Congenital Solitary Functioning Kidney

Cited by 39 publications

References 18 publications

Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations

Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations

Physiology and Pathophysiology of Compensatory Adaptations of a Solitary Functioning Kidney

Prevalence, types, and malformations in congenital anomalies of the kidney and urinary tract in newborns: a retrospective hospital-based study

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