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A Case of Hyperammonemia Not Attributable to Liver Disease and Treated With IV Ammonia Scavengers.
Cureus
November 2024
Department of Internal Medicine, Wrexham Maelor Hospital, Wrexham, GBR.
Hyperammonemia is a serious metabolic condition marked by elevated ammonia levels in the blood, leading to neurological damage and systemic complications if untreated. While often associated with liver dysfunction, inborn metabolic errors such as fatty acid oxidation defects, pyruvate metabolism disorders, urea cycle disorders (UCDs), urea splitting bacterial infections, hemato-oncological disorders, and portosystemic shunts are less commonly recognized but significant causes, particularly outside neonatal populations. These metabolic errors, due to partial enzyme deficiencies, may present later in life with atypical symptoms.
View Article and Find Full Text PDFValproate-induced hyperammonemic encephalopathy in neurosurgical patients: Our experience and systematic literature review.
Neurosurg Rev
November 2024
Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India.
Article Synopsis
- Sodium valproate is commonly used to treat seizures, chronic pain, and bipolar disorder, but it can cause severe side effects like valproate-induced hyperammonemic encephalopathy (VHE), which is life-threatening.
- A study reviewed four neurosurgical patients who developed VHE after using valproate for seizure prevention, revealing a 50% mortality rate and indicating that prompt diagnosis and treatment are crucial.
- Treatment involved discontinuing valproate and, in some cases, dialysis; half of the patients experienced clinical improvement after normalization of ammonia levels.
Correlation of ammonia and blood laboratory parameters with hepatic encephalopathy: A systematic review and meta-analysis.
PLoS One
September 2024
Neuroscience Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Background And Objectives: Emerging research suggests that hyperammonemia may enhance the probability of hepatic encephalopathy (HE), a condition associated with elevated levels of circulating ammonia in patients with cirrhosis. However, some studies indicate that blood ammonia levels may not consistently correlate with the severity of HE, highlighting the complex pathophysiology of this condition.
Methods: A systematic review and meta-analysis through PubMed, Scopus, Embase, Web of Science, and Virtual Health Library were conducted to address this complexity, analyzing and comparing published data on various laboratory parameters, including circulating ammonia, blood creatinine, albumin, sodium, and inflammation markers in cirrhotic patients, both with and without HE.
Sodium Valproate-Induced Hyperammonemia: A Case Series in a Tertiary Care Hospital.
Cureus
July 2024
Pharmacology, Christian Medical College and Hospital, Ludhiana, IND.
Background: Sodium valproate (VPA) is an extensively used anti-convulsant, which is an effective drug for treatment of epilepsy in adults and children, as well as for conditions like migraine, bipolar disorder, mania, and trigeminal neuralgia. Sedation, vertigo, ataxia, dose-dependent tremors, headaches, and gastrointestinal side effects are the most often reported adverse effects associated with VPA. A potential life-threatening event reported with VPA is hyperammonemia (HA), which is defined as an increase in serum level of ammonia.
View Article and Find Full Text PDFClinical features and CPS1 variants in Chinese patients with carbamoyl phosphate synthetase 1 deficiency.
BMC Pediatr
August 2024
Children's Medical Center, Peking University First Hospital, Beijing, 102600, China.
Background: Carbamoyl phosphate synthetase 1 (CPS1) deficiency (OMIM 237300), an autosomal recessive rare and severe urea cycle disorder, is associated with hyperammonemia and high mortality.
Methods: Herein we present 12 genetic variants identified in seven clinically well-characterized Chinese patients with CPS1 deficiency who were admitted to the Children's Medical Center of Peking University First Hospital from September 2014 to August 2023.
Results: Seven patients (two male and five female patients including two sisters) experienced symptoms onset between 2 days and 13 years of age, and they were diagnosed with CPS1 deficiency between 2 months and 20 years.
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