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Brain and spine malformations and neurodevelopmental disorders in a cohort of children with CAKUT.
- Source :
-
Pediatric Nephrology . Jul2024, Vol. 39 Issue 7, p2115-2129. 15p. - Publication Year :
- 2024
-
Abstract
- Background: Congenital anomalies of the kidney and urinary tract (CAKUT) represent 20–30% of all birth defects and are often associated with extra-renal malformations. We investigated the frequency of brain/spine malformations and neurological features in children with CAKUT. Methods: We reviewed the clinico-radiological and genetic data of 199 out of 1,165 children with CAKUT evaluated from 2006 to 2023 (99 males, mean age at MRI 6.4 years) who underwent brain and/or spine MRI. Patients were grouped according to the type of CAKUT (CAKUT-K involving the kidney and CAKUT-H involving the inferior urinary tract). Group comparisons were performed using χ2 and Fisher exact tests. Results: Brain/spine malformations were observed in 101/199 subjects (50.7%), 8.6% (101/1165) of our CAKUT population, including midbrain-hindbrain anomalies (40/158, 25.3%), commissural malformations (36/158, 22.7%), malformation of cortical development (23/158, 14.5%), Chiari I anomaly (12/199, 6%), cranio-cervical junction malformations (12/199, 6%), vertebral defects (46/94, 48.9%), caudal regression syndrome (29/94, 30.8%), and other spinal dysraphisms (13/94, 13.8%). Brain/spine malformations were more frequent in the CAKUT-K group (62.4%, p < 0.001). Sixty-two subjects (62/199, 31.2%) had developmental delay/intellectual disability. Neurological examination was abnormal in 40/199 (20.1%). Seizures and/or electroencephalographic anomalies were reported in 28/199 (14%) and behavior problems in 19/199 subjects (9%). Developmental delay/intellectual disability was more frequent in kidney dysplasia (65.2%) and agenesis (40.7%) (p = 0.001). Conclusions: We report a relative high frequency of brain/spine malformations and neurodevelopmental disorders in children with CAKUT who underwent MRI examinations in a tertiary referral center, widening the spectrum of anomalies associated with this condition. [ABSTRACT FROM AUTHOR]
- Subjects :
- *BRAIN abnormalities
*URINARY organ abnormalities
*KIDNEY abnormalities
*RISK assessment
*CONGENITAL heart disease
*NEUROLOGIC examination
*CHILD psychopathology
*CEREBRAL cortex abnormalities
*RESEARCH funding
*FISHER exact test
*NEURAL development
*DESCRIPTIVE statistics
*CHI-squared test
*MAGNETIC resonance imaging
*TERTIARY care
*ARNOLD-Chiari deformity
*CAUDAL regression syndrome
*DEVELOPMENTAL disabilities
*INTELLECTUAL disabilities
*MEDICAL records
*ACQUISITION of data
*SEIZURES (Medicine)
*NERVOUS system abnormalities
*NEURAL tube defects
*DISEASE risk factors
*DISEASE complications
GENITOURINARY organ abnormalities
Subjects
Details
- Language :
- English
- ISSN :
- 0931041X
- Volume :
- 39
- Issue :
- 7
- Database :
- Academic Search Index
- Journal :
- Pediatric Nephrology
- Publication Type :
- Academic Journal
- Accession number :
- 177623344
- Full Text :
- https://doi.org/10.1007/s00467-024-06289-6