Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt

Article Article scientifique Article Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt eng 2023-12-28 2024-02 author Chabbey Isaline Cécile Coline Department of Pediatrics, Gynecology and Obstetrics, Swiss Pediatric Liver Center, Division of Pediatric Surgery University of Geneva Geneva Switzerland. 834120 author Cudalbu Cristina CIBM Center for Biomedical Imaging Vaud Switzerland.,Animal Imaging and Technology Ecole Polytechnique Fédérale de Lausanne Lausanne Switzerland. author Barras Eugénie Diagnostic Department, Pediatric Radiology Unit, Radiology Division Geneva University Hospitals Geneva Switzerland. 670766 author Hanquinet Sylviane Diagnostic Department, Pediatric Radiology Unit, Radiology Division Geneva University Hospitals Geneva Switzerland. 133458 Décanat et administration de la faculté de médecine (ADMED) author Maréchal Bénédicte Advanced Clinical Imaging Technology Siemens Healthineers International AG Lausanne Switzerland.,Department of Radiology, Lausanne University Hospital (CHUV) University of Lausanne Vaud Switzerland.,LTSS, Ecole Polytechnique Fédérale de Lausanne (EPFL) Lausanne Switzerland. author Rougemont-Pidoux Anne-Laure Division of Clinical Pathology, Swiss Pediatric Liver Center Geneva University Hospitals Geneva Switzerland. 29140 Département de pathologie et immunologie (PATIM) author Wacker Bou Puigdefabregas Julie Caroline Department of Pediatrics, Gynecology and Obstetrics, Pediatric Cardiology Unit University of Geneva, Geneva, Switzerland Geneva Switzerland. 540977 author Zangas-Gheri Florence Pediatric Neurology Unit Geneva University Hospitals Geneva Switzerland. author Mclin Valérie Anne Department of Pediatrics, Gynecology and Obstetrics, Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit University of Geneva Geneva Switzerland. 799651 Département de Pédiatrie, Gynécologie et Obstétrique (PETRI) 35-42 10.1002/jpr3.12035 38545268 2691-171X PMC10964341 https://onlinelibrary.wiley.com/doi/10.1002/jpr3.12035 Background: Chronic hepatic encephalopathy (CHE) has been reported both in patients with congenital porto-systemic shunts (CPSS) and chronic liver disease. CHE is difficult to recognize in children as there is no clear definition and its manifestations are highly variable. CHE is associated with variations in brain volumes and metabolites that have already been demonstrated using 1.5-3T MRI systems. However, the in-depth study of brain metabolism requires the high spectral resolution of high magnetic fields. Objectives and methods: We analyzed the neurometabolic profile, brain volumes and T1relaxation times of a child with a CPSS using high field proton magnetic resonance spectroscopy (1H MRS, 7T) combined with MRI and compared it to an age-matched control group. We also evaluated the impact of shunt closure on neurocognitive symptoms using adapted neuropsychological tests. Results: 7T MRS revealed a significant increase in glutamine compared to controls, a decrease in brain osmolytes, and a slight elevation in NAA concentrations. 7T MRI scans showed morphological abnormalities but no changes in the signal intensity of the globus pallidus. Neurocognitive testing revealed attention deficit disorder, language difficulties, and mild intellectual disability. Most of these areas improved after shunt closure. Conclusions: In this paediatric case of type B HE with normal fasting ammonia, neurometabolic profile was compatible with what has been previously shown in chronic liver disease, while also demonstrating an isolated glutamine peak. In addition, neurocognitive function partially improved after shunt closure, arguing strongly for shunt closure in both presymptomatic and symptomatic patients. Chronic liver disease Congenital porto‐systemic shunts Hepatic encephalopathy Proton magnetic resonance spectroscopy Dewey 616.0757 Dewey 618 Swiss National Science Foundation Translational Non-Invasive Metabolic Studies towards Novel Treatments of Chronic Hepatic Encephalopathy in Developing Brain, from 3D Organotypic Brain Cell Cultures to the In vivo Rat and Human Brain 173222 CH Projects Swiss National Science Foundation Enhanced MR Spectroscopic mapping of brain regional changes in type C hepatic encephalopathy of juvenile rats to develop novel combinatorial treatments 201218 CH Projects 58 Département de pédiatrie, gynécologie et obstétrique 223 62 Département de radiologie et informatique médicale 227 false 179 L'hypertension pulmonaire (pédiatrie) 228 JPGN reports 5 1 8b05fd2e-33b9-474b-a539-e63dc5eccec9 Article (Published version) Public Article_scientifique_from_europePmc.pdf 1157132 application/pdf CC BY-NC-ND-4.0 c6498f0c79dcecfd56cfbb6328b0e1f162e5158b67d52f3f2880148a870f5629 ea97eedd-50c8-4c55-817e-43f3c0701e56 Appendix Public jpr312035-sup-0001-supplementary_ma.docx 727365 application/vnd.openxmlformats-officedocument.wordprocessingml.document 6c22703dc1b8122cc897802733f6cf93a17d265e995e4a29ce4a640e1934f907 openAccess Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique Faculté de médecine / Section de médecine clinique / Département de radiologie et informatique médicale

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Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt	JPGN reports			2024	84	144

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Barras, Eugénie