[Pregnancy and labor problems in patients with acute porphyria].
OBJECTIVE
To evaluate the impact of developing pregnancy on porphyrin metabolism in reproductive-aged women with acute porphyria (AP).


METHODS
The prospective clinical data of 33 pregnancies were analyzed in 28 patients with the established diagnosis of AP. The latter was verified by the quantitative analysis of 24-hour urinary porphyrin excretion and the diminished activity of the pathognomonic enzyme.


RESULTS
Each case was analyzed in detail according to different criteria. Poor prognostic factors for pregnancy are identified in AP. The used curation policy for pregnant patients is described. The pregnant women with occurring AP episodes are subdivided into clinical groups requiring different curation approaches. The scheme for the used working protocol is given.


CONCLUSIONS
The accumulated experience with curating the patients with AP will be able to avoid the existing prohibitory practice, providing a way to develop a new quality of life in the patients' families.
Ia S
IaS
Pustovoĭt
I V
IV
Karpova
R G
RG
Shmakov
S A
SA
Makhinia
D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007744:Obstetric Labor Complications / Q000453:epidemiology / Q000209:etiology*; D017118:Porphyria, Acute Intermittent / Q000453:epidemiology / Q000652:urine*; D011166:Porphyrins / Q000652:urine*; D011247:Pregnancy; D011248:Pregnancy Complications; D011256:Pregnancy Outcome; D011446:Prospective Studies; D011788:Quality of Life; D012307:Risk Factors; D012426:Russia / Q000453:epidemiology; D055815:Young Adult
D011166:Porphyrins