Postoperative metabolic decompensation in maple syrup urine disease is completely prevented by insulin

Proton magnetic resonance spectroscopy r

Proton magnetic resonance spectroscopy reflects metabolic decompensation in maple syrup urine disease

✍ W. Heindel; H. Kugel; U. Wendel; B. Roth; G. Benz-Bohm 📂 Article 📅 1995 🏛 Springer-Verlag 🌐 English ⚖ 426 KB

Analytical approach in diagnosis of inhe

Analytical approach in diagnosis of inherited metabolic diseases: Maple syrup urine disease (MSUD) - simultaneous analysis of metabolites in urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry (enantio-MDGC-MS)

✍ Podebrad, Frank ;Heil, Martin ;Leib, Saandra ;Geier, Bernhard ;Beck, Thomas ;Mos 📂 Article 📅 1997 🏛 John Wiley and Sons 🌐 English ⚖ 573 KB

## Abstract __M__aple Syrup __U__rine disease (USUD) is an autosomal recessive inherited metabolic disorder of branched‐chain amino acid (L‐valin, L‐leucine, and L‐isoleucine metabolism named after the characteristic smell of affected patients urine. MSUD is caused by a deficiency of the branched‐c