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112 Possible Causes for Nonketotic Hypoglycemia

  • Lethal Neonatal Carnitine Palmitoyl Transferase II Deficiency

    He presented on the first day of life with nonketotic hypoglycemia, seizures, hepatomegaly, cardiomegaly with biventricular hypertrophy, and ventricular arrhythmias.[genome.jp] He came to medical attention during an episode of somnolence attributable to mild nonketotic hypoglycemia (glucose, 2 mmol/L).[clinchem.aaccjnls.org] DiscussionIn addition to dysmorphia, the clinical features of neonatal CPT IIdeciency include nonketotic hypoglycemia, metabolic acidosis,seizures, arrhythmias, nephromegaly[docslide.net]

  • Glutaric Aciduria

    hypoglycemia, heart disease, acidosis, vomiting, nausea, weakness, lethargy, and enlargement of their liver.[disabled-world.com] […] flavoprotein deficiency Multiple FAD dehydrogenase deficiency Multiple acyl-CoA dehydrogenase deficiency Symptoms of Glutaric Acidemia Type II People with GA2 can experience nonketotic[disabled-world.com]

  • Carnitine Deficiency

    The presence of failure to thrive, recurrent infections, hypotonia, encephalopathy, cardiomyopathy, or nonketotic hypoglycemia requires investigation of carnitine status.[jamanetwork.com] Primary carnitine deficiency is an autosomal recessive disorder caused by defective carnitine transport and manifests as nonketotic hypoglycemia or skeletal or heart myopathy[ncbi.nlm.nih.gov] Patients may present as infants with nonketotic hypoglycemia, hypotonia, Reye syndrome or sudden infant death or later in life with cardiomyopathy (characteristically dilated[genedx.com]

  • Carnitine Transporter Deficiency

    Patients may present as infants with nonketotic hypoglycemia, hypotonia, Reye syndrome or sudden infant death or later in life with cardiomyopathy (characteristically dilated[genedx.com] Affected children present in infancy with nonketotic hypoglycemia, Reye syndrome or sudden infant death ( 1 , 2 ).[pnas.org]

  • Acute Fatty Liver of Pregnancy

    The nonketotic hypoglycemia can imitate Reye’s syndrome (a rare but serious condition in infant and children that causes swelling in the brain and liver) or defects in the[preeclampsia.org] hypoglycemia, hepatic failure, and death associated with fatty acid oxidation defects in newborns.[emedicine.medscape.com] Some babies are at risk of developing life threatening nonketotic hypoglycemia, an inherited condition in which the body is unable to breakdown and process some of the building[preeclampsia.org]

  • Disorder of Ornithine Metabolism

    Fatty oxidation defect- will also have nonketotic hypoglycemia Transient hyperammonemia of newborn- clinically, it is typically pre-term infant with substantial respiratory[pedclerk.bsd.uchicago.edu] […] metabolism disorder- will also present with lactic aciduria and anion gap Organic acidemia- may inhibit a urea cycle enzyme but will present with metabolic acidosis/ketotic hypoglycemia[pedclerk.bsd.uchicago.edu]

  • Medium Chain Acyl CoA Dehydrogenase Deficiency

    Hypoglycemia and Carnitine Deficiency due to MCAD Deficiency Disorder Subdivisions None General Discussion Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is a rare[metrohealth.net] […] to defect in Beta-Oxidation of Fatty Acids Carnitine Deficiency Secondary to MCAD Deficiency Dicarboxylicaciduria due to MCADH Deficiency MCAD Deficiency ACADM Deficiency Nonketotic[metrohealth.net]

  • Glutaric Aciduria Type 2

    hypoglycemia, heart disease, acidosis, vomiting, nausea, weakness, lethargy, and enlargement of their liver.[disabled-world.com] The neonatal-onset forms are usually fatal and are characterized by severe nonketotic hypoglycemia, metabolic acidosis, multisystem involvement, and excretion of large amounts[mitodb.com] Multiple acyl-CoA dehydrogenation deficiency (MADD) in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy.[link.springer.com]

  • Beckwith-Wiedemann Syndrome

    TABLE 1 Hypoglycemic Screening Performed at Birth Showed Nonketotic Hypoglycemia Consistent With HH At 1 year of age, the glucose homeostasis was reassessed after the medications[pediatrics.aappublications.org]

  • Left Ventricular Noncompaction

    hypoglycemia, seizure and metabolic acidosis.[ncbi.nlm.nih.gov] […] authors report case of an 8-mo-old infant with HMG-CoA lyase deficiency, who presented with macrocephaly, left ventricular noncompaction, recurrent pulmonary infections, nonketotic[ncbi.nlm.nih.gov]

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