Abstract
Recent advances in the early diagnosis of fetal CAKUT with an increase in fetal surgical interventions have led to a growing number of neonatal survivors born with severe renal dysfunction. This, in turn, has required the development of multi-disciplinary treatment paradigms in the individualized management of these infants with advanced stage kidney disease from birth. Early multi-modal management includes neonatal surgical interventions directed toward establishing adequate urine flow, respiratory support with the assessment of pulmonary hypoplasia, and establishing metabolic control to avoid the need for dialysis intervention. The development of specialized imaging to assess for residual renal mass with non-invasive 3-dimensional techniques are rapidly evolving. The use of non-radioactive imaging offers improved safety and allows for early prognostic-based planning including anticipatory guidance for progression to end stage renal disease (ESRD). The trajectory of kidney function during the neonatal period as determined by peak and nadir serum creatinine (SCr) and cystatin C (CysC) during the first months of life provides a guide toward individualized prospective management. This is a single center experience based on a birth cohort of 42 subjects followed prospectively from birth for an average of 6.1 ± 2.8 years at the University of Miami/Holtz Children's Hospital during the past decade. There was an 8:1 male: female ratio. The birth cohort was divided into 3 subgroups according to CKD Stages at the current age: CKD 1–2 (Group 1) (eGFR ≥ 60 ml/min/1.73 m2) (N = 15), CKD stage 3–5 (Group 2) (eGFR ≤ 59 ml/min/1.73 m2) (N = 12), and ESRD—Dialysis and/or Transplantation (Group 3) (N = 15). A neonatal CysC >3.0 mg/L predicted progression to ESRD while a nadir SCr >0.6 mg/dL predicted progression to CKD 3–5 with the highest specificity and sensitivity by ROC-AUC analysis (P < 0.0001). Medical management was directed toward nutritional support with novel formula designs, early introduction of growth hormone and strict control of mineral bone disorder. One of the central aspects of the management was to avoid dialysis for as long as feasible with a primary goal toward pre-emptive transplantation.
Highlights
Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause of end stage renal disease (ESRD) in children accounting for 50–70% of those who begin renal replacement therapy (RRT) worldwide [1,2,3,4]
From a total of 52 subjects born during the study period with a diagnosis of severe CAKUT, 42 met inclusion criteria and had adequate neonatal data to predict progression of chronic kidney disease (CKD) over a median of 5.5 years
All have functioning grafts at a current age of 6.8 ± 2.4 years. In this single center experience of a large birth cohort with severe CAKUT, we provide neonatal predictors of early progression of CKD as well as management paradigms that can promote longevity and growth
Summary
Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause of end stage renal disease (ESRD) in children accounting for 50–70% of those who begin renal replacement therapy (RRT) worldwide [1,2,3,4]. Most challenging is the early management of these infants with bilateral and severe CAKUT who have significant renal compromise at birth [3,4,5]. The natural history of severe neonatal CAKUT remains incompletely understood with respect to the demographic and biological “predictors” of progression to chronic kidney disease (CKD). Consistent with the developmental and physiologic renal accommodations to the extra-uterine environment, there is a natural improvement in glomerular filtration rate (GFR) from birth throughout the first 2 years of life [4,5,6,7,8]. The management of infants with severe CAKUT must consider and optimize this renal development in hopes of preserving nephron reserve to extend the longevity of individual renal function
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