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Congenital Solitary Kidney from Birth to Adulthood
Pierluigi Marzuillo1, Stefano Guarino1, Anna Di Sessa1
1Department of Woman, Child and of General and Specialized Surgery, Università degli Studi della Campania "Luigi Vanvitelli", Naples, Italy.
Insights
Congenital solitary functioning kidney patients show better outcomes than previously thought. Congenital anomalies of the kidney and urinary tract (CAKUT) are the primary risk factor for kidney injury in these patients.
Area of Science:
- Pediatric Nephrology
- Urology
- Clinical Medicine
Background:
- Congenital solitary functioning kidney (CSFK) is a condition present from birth.
- Long-term outcomes for CSFK patients are not well-established.
Purpose of the Study:
- To assess the long-term course of patients with CSFK diagnosed prenatally and followed into adulthood.
- To identify risk factors associated with kidney injury in CSFK patients.
Main Methods:
- Retrospective study of 56 patients with CSFK, diagnosed prenatally and followed from infancy to adulthood (≥18 years).
- Kidney injury defined by reduced eGFR, hypertension, or proteinuria.
- Modifiable risk factors (serum uric acid, smoking, obesity) and CAKUT were assessed.
Main Results:
- 26.8% of patients experienced mild kidney injury.
- Congenital anomalies of the kidney and urinary tract (CAKUT) were the sole significant risk factor for kidney injury (HR 4.9).
- Patients without CAKUT or modifiable risk factors had a 100% survival free from kidney injury at 30 years.
Conclusions:
- Adult outcomes for CSFK patients appear favorable.
- Early diagnosis, lifestyle promotion, and monitoring are crucial for improving CSFK patient prognosis.
- CAKUT is a key predictor of kidney injury in CSFK.
Purpose:
To evaluate the course of prenatally diagnosed and early-enrolled congenital solitary functioning kidney patients followed until adulthood and to identify risk factors for kidney injury.
Materials And Methods:
Among all congenital solitary functioning kidney patients followed (1993-2018), we recalled 56 patients with prenatal diagnosis and congenital solitary functioning kidney confirmation at 1-3 months of life reaching at least 18 years of age. Serum uric acid, heavy smoking (≥25 cigarettes/day) and overweight/obesity were clustered as modifiable risk factors. Kidney injury was defined by estimated glomerular filtration rate <90 ml/minute/1.73 m2 and/or 24-hour ambulatory blood pressure monitoring confirmed hypertension and/or proteinuria. Modifiable risk factors and congenital anomalies of the kidney and urinary tract (CAKUT) of congenital solitary functioning kidney were evaluated as risk factors for kidney injury.
Results:
The mean followup period was 21.1 years (range 18-33 years). Mild kidney injury was found in 15 out of 56 patients (26.8%). The mean age at proteinuria, reduced estimated glomerular filtration rate and hypertension onset was 19.7 years (1.2 SDS), 20.7 years (2.7 SDS), and 22 years (5.6 SDS), respectively. Patients with CAKUT of congenital solitary functioning kidney and with both CAKUT of congenital solitary functioning kidney and modifiable risk factors presented survival free from kidney injury of 0% at 22.2 and 24.2 years of age, respectively. Patients with modifiable risk factors presented 42.4% of survival at 30 years. Patients without CAKUT of congenital solitary functioning kidney nor modifiable risk factors presented 100% of survival at 30 years of age (p=0.002). The presence of CAKUT of congenital solitary functioning kidney was the only significant risk factor (HR 4.9; 95% CI 1.8-14.2; p=0.003).
Conclusions:
The outcomes of congenital solitary functioning kidney in early adulthood appear better than previously reported. Prompt diagnosis of congenital solitary functioning kidney, healthy lifestyle promotion and monitoring of serum uric acid may improve the prognosis of congenital solitary functioning kidney patients.
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