No abstract available.
Porphyria, Acute Intermittent* ; Pregnancy*

No abstract available.
Porphyria, Acute Intermittent* ; Pregnancy*

No abstract available.
Leg* ; Porphyria, Acute Intermittent* ; Porphyrias

The Porphyria are a group of inherited and acquired disorders characterized by partial defects in the heme biosynthetic pathway. Among the hepatic forms, acute intermittent porphyria(AIP) is the most severe and common type in western hemisphere. Though its association with pregnancy is rare, it presents the obstetrician with challenging problems in diagnosis and management and it is probable that pregnancy had some deleterious effect in acute porphyria. The authors present a cae of AIP in pregnancy with a review of literature.
Biosynthetic Pathways ; Diagnosis ; Heme ; Porphyria, Acute Intermittent* ; Porphyrias ; Pregnancy*

Adolescent ; Aminolevulinic Acid ; pharmacology ; Female ; Humans ; Porphyria, Acute Intermittent ; physiopathology

We report a case of acute intermittent porphyria presenting with variable symptoms and signs such as hypertension, polyneuropathy, syndrome of inappropriate secretion of antidiuretic hormone and cerebral infarction. A 47 year-old female patient entered hospital with abdominal pain followed by generalized seizure. She was diagnosed to have acute intermittent porphyria in consequence of Watson-Schwartz test and δ-ALA in 24 hours urine. She was managed with conservative treatment but died of respiratory failure.
Abdominal Pain ; Cerebral Infarction ; Female ; Humans ; Hypertension ; Polyneuropathies ; Porphyria, Acute Intermittent* ; Respiratory Insufficiency ; Seizures

Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Humans ; Porphyria, Acute Intermittent ; diagnosis ; therapy

Endocrinology ; methods ; Humans ; Male ; Middle Aged ; Porphyria, Acute Intermittent ; diagnosis ; genetics

Abdominal Pain ; etiology ; Child, Preschool ; Humans ; Male ; Porphyria, Acute Intermittent ; complications ; diagnosis ; therapy ; Prognosis ; Treatment Outcome

Acute intermittent porphyria (AIP) has complicated clinical manifestations and is often accompanied by hypertension.AIP may cause hypertension through adrenergic effect,heme deficiency,inflammation,inappropriate secretion of antidiuretic hormone,toxicity of delta-aminolevulinic acid(ALA,aporphyrin precursor),and elevated serum glucose level.The prevention and treatment strategies for AIP accompanied with hypertension mainly include the controlling of porphyria attacks,application of antihypertensive drugs,lifestyle intervention,and management of latent AIP patients.
Humans ; Porphyria, Acute Intermittent ; Blood Glucose ; Hypertension/etiology* ; Inflammation ; Life Style