obsolete_porphyria: ['A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.']
| Name | E4_PORBILIR |
|---|---|
| Long name | Disorders of porphyrin and bilirubin metabolism |
| Hospital Discharge registry | ICD-10: E80 |
| Cause of Death registry | ICD-10: E80 |
| Include | E4_HERERYPOR, E4_PORCUTTAR, E4_PORPHYNAS, E4_CATAPEROXDEF, E4_GILBERT, E4_CRIGLERNAJJAR, E4_PORBILIRNAS |
| Level in the ICD-hierarchy | 3 |
| First defined in version | DF2 |
| Latin name | Perturbationes metabolismi porphyrini et bilirubini |
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | 142 | 52 | 90 |
| Unadjusted prevalence (%) | 0.07 | 0.04 | 0.10 |
| Mean age at first event (years) | 44.60 | 45.96 | 43.82 |
| Case fatality at 5-years (%) | 4.23 | 0.00 | 6.67 |
| All | Female | Male | |
|---|---|---|---|
| Median nb. of events per indiv. | 1.0 | 1.0 | 1.0 |
| Recurrence at 6 months (%) | 26.76 | 25.00 | 27.78 |
before Disorders of porphyrin and bilirubin metabolism
after Disorders of porphyrin and bilirubin metabolism
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