Adverse Drug Reaction Classification System

ADR Ontology
ADR Term Porphyria acute
ADR ID BADD_A03511
ADR Hierarchy
03      Congenital, familial and genetic disorders
03.08      Metabolic and nutritional disorders congenital
03.08.01      Inborn errors of porphyrin metabolism
03.08.01.002      Porphyria acute
09      Hepatobiliary disorders
09.01      Hepatic and hepatobiliary disorders
09.01.10      Hepatic metabolic disorders
09.01.10.001      Porphyria acute
14      Metabolism and nutrition disorders
14.14      Inborn errors of metabolism
14.14.01      Inborn errors of porphyrin metabolism
14.14.01.002      Porphyria acute
17      Nervous system disorders
17.19      Congenital and peripartum neurological conditions
17.19.01      Congenital and hereditary central nervous system disorders NEC
17.19.01.001      Porphyria acute
19      Psychiatric disorders
19.07      Psychiatric disorders NEC
19.07.03      Mental disorders due to a general medical condition NEC
19.07.03.003      Porphyria acute
Description An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine. [MeSH]
MedDRA Code 10036182
MeSH ID D017118; D017092; D046349; D046350
ADR Severity Grade (FAERS) Not Available
ADR Severity Grade (CTCAE) Not Available
Synonym
Acute intermittent porphyria | Erythropoietic porphyria | Hereditary coproporphyria | Porphyria acute | Variegate porphyria | ALA dehydratase deficient porphyria | Delta-aminolevulinic acid dehydratase deficient porphyria | ALAD porphyria | Acute hepatic porphyria | Porphyria, Acute Intermittent | Acute Intermittent Porphyria | Acute Intermittent Porphyrias | Intermittent Porphyria, Acute | Intermittent Porphyrias, Acute | Porphyrias, Acute Intermittent | Acute Porphyria | Acute Porphyrias | Porphyria, Acute | Porphyrias, Acute | Hydroxymethylbilane Synthase Deficiency | Deficiencies, Hydroxymethylbilane Synthase | Deficiency, Hydroxymethylbilane Synthase | Hydroxymethylbilane Synthase Deficiencies | Synthase Deficiencies, Hydroxymethylbilane | Synthase Deficiency, Hydroxymethylbilane | Uroporphyrinogen Synthase Deficiency | Deficiencies, Uroporphyrinogen Synthase | Deficiency, Uroporphyrinogen Synthase | Synthase Deficiencies, Uroporphyrinogen | Synthase Deficiency, Uroporphyrinogen | Uroporphyrinogen Synthase Deficiencies | Porphyria, Swedish Type | Porphyrias, Swedish Type | Swedish Type Porphyria | Swedish Type Porphyrias | Type Porphyria, Swedish | Type Porphyrias, Swedish | UPS Deficiency | Deficiencies, UPS | Deficiency, UPS | UPS Deficiencies | PBGD Deficiency | Deficiencies, PBGD | Deficiency, PBGD | PBGD Deficiencies | Porphobilinogen Deaminase Deficiency | Deaminase Deficiencies, Porphobilinogen | Deaminase Deficiency, Porphobilinogen | Deficiencies, Porphobilinogen Deaminase | Deficiency, Porphobilinogen Deaminase | Porphobilinogen Deaminase Deficiencies
Drugs Leading to the ADR
Drug IDDrug NameADR Frequency (FAERS)ADR Severity Grade (FAERS)
BADD_D00356Carbamazepine--
BADD_D00696Divalproex sodium--
BADD_D01338Mafenide acetate--
BADD_D01366Medroxyprogesterone acetate--
BADD_D02329Valproic acid--
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