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Walk the Even Hospital Database by book and chapter — the raw source passages that ground Ask, DDx, and the rest.
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©2013 UpToDate ® Print Email Biochemical findings in four patients with ALAD porphyria Patient German B German H Swedish Belgian Sex Male Male Male Male Age of onset 15 15 At birth 63 Clinical course Moderate Moderate Severe Mild Tests Test results in percent, Upper limit of normal = 100 Plasma ALA - - - 1155 PBG - - - Normal Coproporphyrin 300 167 - - Protoporphyrin 127 327 - - Urine ALA 2449 2373 8150 794 PBG 238 200 447 Normal Uroporphyrin 361 224 - 888 Coproporphyrin 5380 4698 - 2987 Total porphyrin - - 7247 - Stool Coproporphyrin Normal Normal 284 285 Protoporphyrin Normal Normal Normal 300 Erythrocyte Protoporphyrin, Zn PP 3192 3164 663 408 Erythrocyte ALAD activity 0 1 1 1 Enzymes Activation by dithiothreitol None None None None Activation by Zn+ None None None None PBGD activity Normal Normal Normal Normal -: Not determined. Data reproduced with permission from: Sassa S. ALAD porphyria. In: Seminars in Liver Disease, Berk PD (Ed), Thieme, New York 1998. p.95.