Risk assessment of severe congenital anomalies of the kidney and urinary tract (CAKUT): A birth cohort

Chryso P. Katsoufis, Marissa J. DeFreitas, Juan C. Infante, Miguel Castellan, Teresa Cano, Daniela Safina Vaccaro, Wacharee Seeherunvong, Jayanthi J. Chandar, Carolyn L. Abitbol

Research output: Contribution to journalArticle

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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 noninvasive 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 preemptive transplantation.

Original languageEnglish (US)
Article number182
JournalFrontiers in Pediatrics
Volume7
Issue numberMAY
DOIs
StatePublished - Jan 1 2019

Fingerprint

Parturition
Chronic Kidney Failure
Cystatin C
Kidney
Dialysis
Creatinine
Nutritional Support
Kidney Diseases
Serum
ROC Curve
Kidney Transplantation
Growth Hormone
Area Under Curve
Minerals
Renal Dialysis
Early Diagnosis
Transplantation
Urine
Safety
Bone and Bones

Keywords

  • Biomarkers of early CKD
  • Cystatin C
  • Nadir creatinine
  • Neonatal CAKUT
  • Peak creatinine

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Risk assessment of severe congenital anomalies of the kidney and urinary tract (CAKUT) : A birth cohort. / Katsoufis, Chryso P.; DeFreitas, Marissa J.; Infante, Juan C.; Castellan, Miguel; Cano, Teresa; Vaccaro, Daniela Safina; Seeherunvong, Wacharee; Chandar, Jayanthi J.; Abitbol, Carolyn L.

In: Frontiers in Pediatrics, Vol. 7, No. MAY, 182, 01.01.2019.

Research output: Contribution to journalArticle

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