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161 Possible Causes for Acute Metabolic Decompensation

  • Adrenal Insufficiency

    In the case of non-compliance with therapeutic regimens or if left untreated, an acute metabolic decompensation may result in fatal adrenal crisis.[symptoma.com] In order to prevent so-called adrenal crisis, which are life-threatening events resulting from an acute metabolic decompensation, missing hormones have to be supplemented.[symptoma.com] Laboratory analyses of blood samples typically reveal hyponatremia, hypochloremia, hyperkalemia, and metabolic acidosis.[symptoma.com]

  • Short Chain Acyl CoA Dehydrogenase Deficiency

    This letter should be updated and include a detailed explanation of the management of acute metabolic decompensation, emphasizing the importance of intravenous glucose regardless[dovepress.com] Biochemical abnormalities such as hypoglycemia were documented in 42%–96% of patients with a severe metabolic decompensation, while analysis of ketone bodies was only reported[dovepress.com] […] for cases identified clinically, usually presenting with severe hypoglycemia during the first 2 years of life. 6 Prior to NBS, the most common clinical presentation was acute[dovepress.com]

  • Disorder of Carbohydrate Metabolism

    Treatment The treatment focuses on the general chronic condition and the acute metabolic decompensation that is common with the disorder.[lecturio.com] In acute metabolic decompensation states, glucose infusions are needed as rapidly as possible, followed by insulin injections to promote anabolism.[lecturio.com] In the acute setting, the intake of branched-chain amino acids is stopped until their levels in circulation normalize.[lecturio.com]

  • Biotinidase Deficiency

    However, none of the affected children had acute metabolic decompensation.[ncbi.nlm.nih.gov] Previous studies have shown that the administration of biotin to affected children can be a lifesaving procedure that can reverse acute symptoms and prevent irreversible neurologic[ncbi.nlm.nih.gov]

  • Carnitine Deficiency

    Systemic primary carnitine deficiency (CDSP, MIM 212140) is a disorder of fatty acid oxidation manifesting in acute metabolic decompensation or in progressive cardiomyopathy[ncbi.nlm.nih.gov] metabolic decompensation, or later in life with skeletal and cardiac myopathy or sudden death from arrhythmia [ 12 ].[karger.com] […] accumulation results in impaired fatty acid oxidation, and if carnitine supplements are not initiated, patients with primary carnitine deficiency can present with early acute[karger.com]

  • Barth Syndrome

    Donati MA, Malvagia S, Paquini E, Morrone A, La Marca G, et al. (2006) Barth syndrome presenting with acute metabolic decompensation in the neonatal period.[enlivenarchive.org] Acute metabolic decompensation and sudden death in Barth syndrome: report of a family and a literature review. Eur J Pediatr. 2008; 167: 941?944.[rarediseasesjournal.com] Acute metabolic decompensation and sudden death in Barth syndrome: report of a family and a literature review. Eur. J.[frontiersin.org]

  • Carnitine Transporter Deficiency

    Patients present in infancy with hypoketotic hypoglycaemia and acute metabolic decompensation, or later in life with skeletal myopathy or cardiomyopathy.[medical-dictionary.thefreedictionary.com] metabolic decompensation, or later in life with skeletal and cardiac myopathy or sudden death from arrhythmia [ 12 ].[karger.com] […] accumulation results in impaired fatty acid oxidation, and if carnitine supplements are not initiated, patients with primary carnitine deficiency can present with early acute[karger.com]

  • Ornithine Transcarbamylase Deficiency

    […] could be adapted to explore acute decompensation in other types of inborn errors of metabolism.[ncbi.nlm.nih.gov] Although he had been recently treated for mild hyperammonemia, there was no evidence of acute metabolic decompensation prior to presentation, and plasma ammonia and amino[ncbi.nlm.nih.gov] Acute extrapyramidal syndrome in mild ornithine transcarbamylase deficiency: metabolic stroke involving the caudate and putamen without metabolic decompensation Keegan, Catherine[deepblue.lib.umich.edu]

  • Acute Disease

    Precipitating factors Acute metabolic decompensation in a patient with MSUD is almost always precipitated by a stressor, such as infection, injury, surgery, hormonal changes[newenglandconsortium.org] Parents of children with diagnosed metabolic disorders know the signs of decompensation in THEIR children.[newenglandconsortium.org] […] crisis), and intermittent (asymptomatic but at risk for metabolic crises during acute illness, usually infection).[newenglandconsortium.org]

  • Reye Syndrome

    metabolic decompensation.[ncbi.nlm.nih.gov] […] deficiency illustrates that electron microscopy may help to differentiate this disorder from Reye syndrome even if a liver biopsy is performed in a patient who recovered from an acute[ncbi.nlm.nih.gov]

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