Journal Basic Info
- Impact Factor: 1.809**
- H-Index: 6
- ISSN: 2474-1655
- DOI: 10.25107/2474-1655
Major Scope
- Genetics
- Biochemistry and Biostatistics
- Allergy & Immunology
- Otolaryngology
- Microbiology
- Cardio-Thoracic Surgery
- Hematology
- Endocrinology
Abstract
Citation: Ann Clin Case Rep. 2023;8(1):2397.DOI: 10.25107/2474-1655.2397
Diagnosis of Neurovisceral Crisis of Acute Intermittent Porphyria in the Puerperium
Llorente Ruiz B1*, Luján Varas J1, Rascón Risco M2 and Molina Montero R1
1Department of Intensive Care Medicine, Príncipe de Asturias University Hospital, Spain
2Department of Radiology, Príncipe de Asturias University Hospital, Spain
*Correspondance to: Llorente Ruiz B
PDF Full Text Case Report | Open Access
Abstract:
Acute porphyria’s are rare inherited disorders due to deficiencies of heme synthesis enzymes. The clinical presentation is heterogeneous depending on the enzyme affected. In the case of acute intermittent porphyria, a deficiency of porphobilinogen deaminase is established. The diagnosis is biochemical by the determination of accumulated precursors. Treatment is based on adequate metabolic input and supply of human hemin. Its association with pregnancy is rare. The patterns of symptoms in the pregnant woman varies considerably between individual patients. The time of maximum risk seems to be in the initial period of pregnancy and in the puerperium where changes in the balance of steroid hormones are more likely to occur.
Keywords:
Cite the Article:
Llorente Ruiz B, Luján Varas J, Rascón Risco M, Molina Montero R. Diagnosis of Neurovisceral Crisis of Acute Intermittent Porphyria in the Puerperium. Ann Clin Case Rep. 2023; 8: 2397..