{"title":"妊娠期急性间歇性卟啉症1例报告及文献复习。","authors":"Uma Pandey, V K Dixit","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>The porphyrias are a group of rare metabolic disorders, each arising from a predominantly hereditary catalytic dysfunction of one of the eight enzymes in the porphyrin-haem biosynthetic pathway. Acute intermittent porphyria is the commonest type of porphyria worldwide. The disease is more common in women than men. It is therefore important for the obstetricians to know about this entity as many women present with abdominal pain during pregnancy. This case shows that if acute intermittent porphyria is properly treated in a mother there is normal maternal and foetal outcome. A case of acute intermittent porphyria known before pregnancy has been reported in a patient who had three spontaneous abortions in the past, she was delivered by elective caesarean section. The pathogenesis of the disease, its' symptoms, diagnosis, therapeutic approach both during and out of pregnancy have been also discussed.</p>","PeriodicalId":17244,"journal":{"name":"Journal of the Indian Medical Association","volume":"111 12","pages":"850-1"},"PeriodicalIF":0.0000,"publicationDate":"2013-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Acute intermittent porphyria in pregnancy: a case report and review of literature.\",\"authors\":\"Uma Pandey, V K Dixit\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>The porphyrias are a group of rare metabolic disorders, each arising from a predominantly hereditary catalytic dysfunction of one of the eight enzymes in the porphyrin-haem biosynthetic pathway. Acute intermittent porphyria is the commonest type of porphyria worldwide. The disease is more common in women than men. It is therefore important for the obstetricians to know about this entity as many women present with abdominal pain during pregnancy. This case shows that if acute intermittent porphyria is properly treated in a mother there is normal maternal and foetal outcome. A case of acute intermittent porphyria known before pregnancy has been reported in a patient who had three spontaneous abortions in the past, she was delivered by elective caesarean section. The pathogenesis of the disease, its' symptoms, diagnosis, therapeutic approach both during and out of pregnancy have been also discussed.</p>\",\"PeriodicalId\":17244,\"journal\":{\"name\":\"Journal of the Indian Medical Association\",\"volume\":\"111 12\",\"pages\":\"850-1\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2013-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of the Indian Medical Association\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Indian Medical Association","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Acute intermittent porphyria in pregnancy: a case report and review of literature.
The porphyrias are a group of rare metabolic disorders, each arising from a predominantly hereditary catalytic dysfunction of one of the eight enzymes in the porphyrin-haem biosynthetic pathway. Acute intermittent porphyria is the commonest type of porphyria worldwide. The disease is more common in women than men. It is therefore important for the obstetricians to know about this entity as many women present with abdominal pain during pregnancy. This case shows that if acute intermittent porphyria is properly treated in a mother there is normal maternal and foetal outcome. A case of acute intermittent porphyria known before pregnancy has been reported in a patient who had three spontaneous abortions in the past, she was delivered by elective caesarean section. The pathogenesis of the disease, its' symptoms, diagnosis, therapeutic approach both during and out of pregnancy have been also discussed.
期刊介绍:
The Journal of the Indian Medical association, popularly known as JIMA, an indexed (in index medicus) monthly journal, has the largest circulation (over 1.75 lakh Copies per month) of all the indexed and other medical journals of India and abroad. This journal is also available in microfilm through Bell & Howels, USA. The founder leaders of this prestigious journal include Late Sir Nilratan Sircar, Dr Bidhan Chandra Roy, Dr Kumud Sankar Ray and other scholars and doyens of the medical profession. It started in the pre-independence era (1930) with only 122 doctors.