Indonesian Journal of Clinical Pathology and Medical Laboratory
ISSN 0854-4263
Vol. 18 / No. 3 / Published : 2012-01
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Original Article :
Acute fatty liver of pregnancy
Author :
- Meiti Muljanti*1
- Leonita Anniwati*2
- Juli Soemarsono*3
- Bag PK FK-UNAIR/RSU Dr. Soetomo Surabaya
- Bag PK FK-UNAIR/RSU Dr. Soetomo Surabaya
- Bag PK FK-UNAIR/RSU Dr. Soetomo Surabaya
Abstract :
Acute fatty liver of pregnancy (AFLP) is an uncommon but potentially fatal complication that occurs on the third trimester gestation or early postpartum period. AFLP is characterized by microvesicular fatty infiltration in hepatocytes. The cause of AFLP is unknown, possibly is caused by deficiency of mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) which result in the increased accumulation of medium and long-chain fatty acids. LCHAD deficiency is an autosomal recessive disorder and often occur in mothers with heterozygous genetic abnormality having a homozygous foetus resulting in excess of toxic metabolites. Clinical manifestations of AFLP are not specific; headache, fatique, nausea and vomiting. AFLP is characterized by profound liver failure, renal failure, intestinal haemorrhage, disseminated intravascular coagulation (DIC) and pancreatitis. Laboratory tests tend to show elevation of bilirubin, hyperuricaemia, significant hypoglycaemia, hypocholesterolemia, hypofibrinogenemia and coagulation disturbance. CT scan and ultrasound show fatty liver but cannot support to establish the diagnosis of AFLP, liver biopsy is the gold standard for its diagnosis. Prenatal and genetic examination is needed by heterozygote women and their foetus. Early diagnosis, prompt delivery, correction of hypoglycemia, coagulopathy and prevention of liver failure complication are the key to survive. Severe case of AFLP should be reffered to Liver Unit Department because it is possible that the patient require liver transplantation. Perlemakan hati akut di kehamilan (acute fatty liver of pregnancy = AFLP) merupakan kasus yang jarang terjadi, tetapi dapat mengalami komplikasi yang mematikan. Hal tersebut dapat terjadi pada trimester ketiga kehamilan atau awal pasca persalinan. AFLP ditandai oleh penyusupan (infiltrasi) lemak mikrovesikuler sel hati (hepatosit) tanpa peradangan atau kematian jaringan (nekrosis). Penyebab penyakit AFLP secara tepat masih belum diketahui, diduga kekurangan mitochondrial trifunctional protein (MTP) dan long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) mengakibatkan penumpukan asam lemak rantai sedang dan rantai panjang di hati. Kekurangan LCHAD merupakan kelainan otosom yang muncul (resesif) dan sering terjadi pada ibu memiliki kelainan gen heterozigot dengan janin homozigot, sehingga mengakibatkan kelebihan metabolit toksik. Manifestasi klinis AFLP tidak khas seperti : nyeri kepala, lemah, mual dan muntah. Di AFLP yang berat dapat terjadi gagal hati berat, gagal ginjal akut, perdarahan usus, disseminated intravascular coagulation (DIC), pankreatitis dan kadar glukosa darah rendah (hipoglikemia). Hasil laboratorik terjadi hiperbilirubinemia, hiperurisemia, hipoglikemia, hipokolesterolemia, hipofibrinogenemia dan gangguan koagulasi. Pemeriksaan CT scan dan ultrasonografi menunjukkan perlemakan hati tetapi tidak dapat membantu menetapkan diagnosis AFLP, sebagai baku emas adalah biopsi hati. Untuk perempuan heterozigot dan janinnya perlu uji genetik sebelum lahir. Kunci untuk bertahan hidup adalah diagnosis dini, persalinan segera, pengobatan segera hipoglikemia dan koagulopati, dan pencegahan komplikasi gagal hati. Kasus AFLP yang sangat parah harus dirujuk ke Unit Spesialis Hati karena kemungkinan penderita memerlukan pencangkokan hati.
Keyword :
References :
Ko HH, Yoshida E,(2006) Review: Acute fatty liver of pregnancy USA : Can J Gastroenter
Ibdah JA,(2006) Acute fatty liver of pregnancy: An update on pathogenesis and clinical implications USA : World J Gastroenterol
Lau HH, Chen YY, et al,(2010) Acute fatty liver of pregnancy in a Taiwanese tertiary care center : A retrospective review Taiwan : Taiwan J Obstet Gynecol
Castro MA, Fasset MJ, et al,(1999) Reversible peripartum liver failure : A new perspective on the diagnosis, treatment, and cause of acute fatty liver of pregnancy based on 28 consecutive cases USA : Am J Obstet Gynecol
Spence D,(2010) Case Report: Anesthetic Management of Acute Fatty Liver of Pregnancy in the Postpartum Period USA : AANA Journal
Archive Article
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Volume : 18 / No. : 3 / Pub. : 2012-01 |
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