Brain and spine malformations and neurodevelopmental disorders in a cohort of children with CAKUT.
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 th...
| Publicado en: | Pediatric Nephrology Vol. 39; no. 7; pp. 2115 - 2130 |
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| Autores principales: | , , , , , , , , , , , |
| Formato: | diagnostic images research tables/charts Journal Article |
| Publicado: |
Springer Nature
Jul2024
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=177623344&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 177623344 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Jul2024 vid: 39 iid: 7 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 177623344 175539330 177623344 177623344 10.1007/s00467-024-06289-6 177623344 ppf: 2115 ppct: 15 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Brain and spine malformations and neurodevelopmental disorders in a cohort of children with CAKUT. aug: au: Boeri, Silvia Bodria, Monica Ammendola, Rosa Maria Giacomini, Thea Tortora, Domenico Nobili, Lino Malacarne, Michela Rossi, Andrea Verrina, Enrico Piaggio, Giorgio Mancardi, Maria Margherita Severino, Mariasavina affil: https://ror.org/0107c5v14 Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health Department of Neuroscience (DINOGMI), University of Genoa, Genoa, Italy sug: subj: Nervous System Abnormalities Risk Factors Brain Abnormalities Mental Disorders Diagnosed in Childhood Risk Factors Urogenital Abnormalities Complications Kidney Abnormalities Urinary Tract Abnormalities Risk Assessment Human Male Female Record Review Child Descriptive Statistics Chi Square Test Fisher's Exact Test Malformations of Cortical Development Risk Factors Arnold-Chiari Malformation Risk Factors Heart Defects, Congenital Risk Factors Neural Tube Defects Risk Factors Caudal Regression Syndrome Risk Factors Developmental Disabilities Risk Factors Intellectual Disability Risk Factors Neurologic Examination Seizures Risk Factors Behavioral and Mental Disorders Risk Factors Magnetic Resonance Imaging Tertiary Health Care Funding Source Child: 6-12 years Male Female ab: 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. pubtype: Academic Journal doctype: diagnostic images research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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