Acute porphyria in an intensive care unit

被引:0
作者
de Mora Almazan, Maria Calvo [2 ]
Acuna, Mariano [3 ]
Garrido-Astray, Concepcion [1 ,4 ]
Arcos Pulido, Beatriz [2 ]
Gomez-Abecia, Sara [1 ]
Chicot Llano, Marta [2 ]
Gonzalez Parra, Emilio [3 ]
Gracia Iguacel, Carolina [3 ]
Alonso Alonso, Pablo Pedro [2 ]
Egido, Jesus [3 ]
Enriquez de Salamanca, Rafael [1 ]
机构
[1] Univ Complutense, Unidad Porfirias, Hosp Doce de Octubre, Madrid 28041, Spain
[2] Univ Autonoma Madrid, Unidad Cuidados Intensivos, Hosp La Princesa, E-28049 Madrid, Spain
[3] Fdn Jimenez Diaz, Dept Nefrol, E-28040 Madrid, Spain
[4] Univ Europea Madrid, Dept Ciencias Morfol & Biomed, Madrid, Spain
来源
EMERGENCIAS | 2012年 / 24卷 / 06期
关键词
Acute porphyria; Seizure; Intermittent acute porphyria; Coproporphyria; Intensive care; INTERMITTENT; NEUROPATHY; IDENTIFICATION; DIAGNOSIS; SEIZURES; HMBS;
D O I
暂无
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
Porphyrias are rare hereditary metabolic disorders caused by the inactivity of certain enzymes that participate in heme synthesis. We report 3 cases in which porphyria debuted with acute episodes in young patients. As is often the case, diagnosis was delayed, and intensive care was required for severe encephalopathy. Symptoms improved rapidly after hemin therapy was started, but peripheral polyneuropathy persisted for several months in 1 patient. We report the first case of a porphyria-related seizure triggered by use of the morning-after pill (levonorgestrel). [Emergencias 2012;24:454-458]
引用
收藏
页码:454 / 458
页数:5
相关论文
共 20 条
[1]   Recommendations for the diagnosis and treatment of the acute porphyrias [J].
Anderson, KE ;
Bloomer, JR ;
Bonkovsky, HL ;
Kushner, JP ;
Pierach, CA ;
Pimstone, NR ;
Desnick, RJ .
ANNALS OF INTERNAL MEDICINE, 2005, 142 (06) :439-450
[2]   Acute intermittent porphyria in women:: clinical expression, use and experience of exogenous sex hormones.: A population-based study in northern Sweden [J].
Andersson, C ;
Innala, E ;
Bäckström, T .
JOURNAL OF INTERNAL MEDICINE, 2003, 254 (02) :176-183
[3]  
Badminton MN, 2002, INT J CLIN PRACT, V56, P272
[4]   Epidemiology and clinical characteristics of seizures in patients with acute intermittent porphyria [J].
Bylesjo, I ;
Forsgren, L ;
Lithner, F ;
Boman, K .
EPILEPSIA, 1996, 37 (03) :230-235
[5]  
Rodríguez CC, 2010, EMERGENCIAS, V22, P40
[6]   The little imitator - Porphyria: A neuropsychiatric disorder [J].
Crimlisk, HL .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1997, 62 (04) :319-328
[7]   Identification of acute porphyria: evaluation of a commercial screening test for urinary porphobilinogen [J].
Deacon, AC ;
Peters, TJ .
ANNALS OF CLINICAL BIOCHEMISTRY, 1998, 35 :726-732
[8]   Liver Transplantation From Donors With Acute Intermittent Porphyria [J].
Dowman, Joanna K. ;
Gunson, Bridget K. ;
Bramhall, Simon ;
Newsome, Philip N. ;
Badminton, Mike N. .
ANNALS OF INTERNAL MEDICINE, 2011, 154 (08) :571-572
[9]  
Enriquez de Salamanca R, 2010, ENDOCRINOLOGIA, P1139
[10]   Mechanism of action of emergency contraception [J].
Gemzell-Danielsson, Kristina .
CONTRACEPTION, 2010, 82 (05) :404-409