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<p> </p> <table border="0" width="57%"><tbody><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/001.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_001.jpg" alt="mcith_001.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 1 </strong> </span>An approach to inherited metabolic disorders with chronic encephalopathy.[3] GM2: GM2 gangliosidosis, GM1: GM1 gangliosidosis, NCL: neuronal ceroid lipofuscinosis, MELAS: mitochondrial encephalopathy lactic acidosis syndrome, X-ALD: X-adrenoleukodystrophy, MLD: metachromatic leukodystrophy, MPS: mucopolysaccharidosis, MSD: multiple sulfatase deficiency.<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/002.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_002.jpg" alt="mcith_002.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 2 </strong> </span>Biochemical evaluation of hypoglycaemia Pl: Plasma, HFI: Hereditary fructose intolerance, FAO: Fatty acid oxidation, GSD: Glycogen storage disorder, FBP: Fructose-1,6-bisphosphatase deficiency, GH: Growth hormone, IGF-1: insulin-like growth factor-1<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/003.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_003.jpg" alt="mcith_003.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 3 </strong> <strong></strong></span><strong>Evaluation of metabolic acidosis</strong><span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/004.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_004.jpg" alt="mcith_004.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 4 </strong> </span>Evaluation of metabolic acidosis with increased anion gap L/P: Lactate/pyruvate ratio, N: Normal, «: Decreased, ª: Increased, GSD I: glycogen storage disorder type I, FBP: fructose-1, 6-bisphosphatase deficiency, PEPCK: phosphoenolpyruvate carboxykinase deficiency, PDH: Pyruvate dehydrogense deficiency, PC: pyruvate carboxylase deficiency<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/005.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_005.jpg" alt="mcith_005.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 5 </strong> </span>An approach to diagnosis of hyperammonemia in older children OA: organic acidurias, FAO: fatty acid oxidation defects, PC: pyruvate carboxylase deficiency, PDH: pyruvate dehydrogenase deficiency, ASA: argininosuccinic acid, AS: argininosuccinic aciduria, NAGS: N-acetylglutamate synthetase deficiency, CPS I: carbamoyl phosphate synthetase I deficiency, OTC: ornithine transcarbamoylase deficiency, HHH: hyperornithinemia hyperammonemia homocitrullinuria syndrome, LPI: lysinuric protein intolerance<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/006.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_006.jpg" alt="mcith_006.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 6 </strong> </span>MRI (FLAIR) in a 20 months old girl with phenylketonuria. Note the periventricular hyperintensities<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/007.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_007.jpg" alt="mcith_007.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 7 </strong> </span>MRI (T2W ) in a 14 days- old baby with classic maple syrup urine disease. Note the diffuse symmetrical white matter hyper intensity of white matter. In addition, note involvement of globus pallidi and thalami<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/008.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_008.jpg" alt="mcith_008.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 8 </strong> </span>MRI (T2 W) in a 15 months old baby with glutaric aciduria type 1. Note the widened sylvian fissures, bilateral symmetrical signal changes in the basal ganglia. and widened subarachnoid spaces<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr><tr style="border-top:1pt dotted #C0C0C0;border-bottom:1pt dotted #C0C0C0;padding-bottom:.1in;padding-top:.1in;"><td valign="top" style="padding-top:.1in;padding-bottom:.1in;"> <a href="http://www.biology-online.org/js/tiny_mce/plugins/imagemanager/files/boa002/009.jpg"><img src="/js/tiny_mce/plugins/imagemanager/files/boa002/mcith/mcith_001.jpg" alt="mcith_001.jpg"></a> </td> <td valign="top" style="padding-left:.1in;padding-top:.1in;padding-bottom:.1in;"><span style="font-family:Arial;"><strong> Figure 9 </strong> </span>MRI (T2W) in a 12- year old boy with methylmalonic acidemia. Note the bilateral symmetrical hyperintensities involving the medial globus pallidi<span style="font-family:Arial;"></span><p> (Click image to enlarge) </p> </td> </tr></tbody></table><p> </p>
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