Interdependence between degree of porphyrin excess and disease severity in congenital erythropoietic porphyria (Gunther's disease)

Archives of Dermatological Research

Volumn 289, Issue 5, 1997, Pages 272-276

  Freesemann, A G ^a   Bhutani, L K ^b   Jacob, K ^c   Doss, M O ^a  

^a PHILIPPS UNIVERSITY MARBURG   (Germany)

^b ALL INDIA INSTITUTE OF MEDICAL SCIENCES   (India)

^c LUDWIG MAXIMILIANS UNIVERSITY   (Germany)

Author keywords

Congenital erythropoietic porphyria; Disease expression; Porphobilinogen deaminase; Porphyrins and isomers; Uroporphyrinogen III synthase

Indexed keywords

COPROPORPHYRIN; PORPHOBILINOGEN DEAMINASE; PORPHYRIN; PROTOPORPHYRIN; UROPORPHYRINOGEN III SYNTHASE;

ARTICLE; AUTOSOMAL RECESSIVE INHERITANCE; BILE; BLOOD ANALYSIS; CHILD; CLINICAL ARTICLE; CONGENITAL ERYTHROPOIETIC PORPHYRIA; DISEASE SEVERITY; ENZYME ACTIVITY; ENZYME DEFICIENCY; FECES ANALYSIS; FEMALE; HEMOLYSATE; HEMOLYTIC ANEMIA; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HIGH PERFORMANCE THIN LAYER CHROMATOGRAPHY; HUMAN; HUMAN CELL; INFANT; LIVER METABOLISM; MALE; METABOLIC DISORDER; PHOTOSENSITIVITY DISORDER; PORPHYRIN METABOLISM; PRIORITY JOURNAL; SPECTROFLUOROMETRY; SPECTROPHOTOMETRY; SYMPTOM; URINALYSIS;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; COPROPORPHYRINS; ERYTHROCYTES; FECES; FEMALE; HEMOLYSIS; HUMANS; HYDROXYMETHYLBILANE SYNTHASE; INDIA; MALE; PHOTOSENSITIVITY DISORDERS; PORPHYRIA, ERYTHROPOIETIC; PORPHYRINS; UROPORPHYRINOGEN III SYNTHETASE;

EID: 0030758552     PISSN: 03403696     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004030050192     Document Type: Article

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