α-Ketoisocaproic Acid Disrupts Mitochondrial Bioenergetics in the Brain of Neonate Rats: Molecular Modeling Studies of α-ketoglutarate Dehydrogenase Subunits Inhibition.

Brain accumulation of the branched-chain α-keto acids α-ketoisocaproic acid (KIC), α-keto-β-methylvaleric acid (KMV), and α-ketoisovaleric acid (KIV) occurs in maple syrup urine disease (MSUD), an inherited intoxicating metabolic disorder caused by defects of the branched-chain α-keto acid dehydrogenase complex. Patients commonly suffer life-threatening acute encephalopathy in the newborn period and develop chronic neurological sequelae of still undefined pathogenesis. Therefore, this work investigated the in vitro influence of pathological concentrations of KIC (5 mM), KMV (1 mM), and KIV (1 mM) on mitochondrial bioenergetics in the cerebral cortex of neonate (one-day-old) rats.

Diabetic Striatopathy: An Extremely Rare Form of Diabetes Mellitus End-Organ Lesions.

Diabetes mellitus is one of the most frequent endocrinopathies in the medical routine, appearing across different specialties. Although neurological involvement in the form of peripheral neuropathy is the most recurrent form acknowledged by physicians, the spectrum of neurological involvement can be more diverse. Here, we present a case of diabetic striatopathy, a rare neurological manifestation of diabetes mellitus with poor metabolic control, in a patient whose epidemiological group was not classically.

Can you Diagnose Immune Thrombocytopenia in Myeloma? A Case of Thrombocytopenia in a Patient with Multiple Myeloma.

Unlabelled: Multiple myeloma (MM) is the second most common haematological malignancy, the diagnosis of which has doubled in recent years. Immune thrombocytopenia consists of isolated thrombocytopenia secondary to antibody mediated peripheral platelet destruction. In most cases, there is no identifiable cause (primary immune thrombocytopenia).