Ann Clin Case Rep | Volume 8, Issue 1 | Case Report | Open Access

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 

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.

Citation:

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..