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CASE REPORT Table of Contents  
Ahead of print publication
Acute fatty liver of pregnancy


 Department of Obstetrics and Gynaecology, Dubai Hospital, Al Baraha, Dubai

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  Abstract 


Acute fatty liver of pregnancy (AFLP) is a rare and serious disorder occurring in the third trimester of pregnancy. It can cause both maternal and fetal morbidity and mortality. Its presentation can be confusing, and differential diagnosis of other liver pathology needs to be considered. Prompt and aggressive management is required to save maternal life. We present a case of AFLP at a tertiary care hospital in Dubai.

Keywords: Clinical presentation, fatty liver, multidisciplinary team, pregnancy


How to cite this URL:
AlSayari SY, ElSayed A, Tambawala ZY, Al Ani K. Acute fatty liver of pregnancy. Hamdan Med J [Epub ahead of print] [cited 2019 Sep 17]. Available from: http://www.hamdanjournal.org/preprintarticle.asp?id=240727





  Introduction Top


Acute fatty liver of pregnancy (AFLP) is an uncommon life-threatening obstetric emergency. Its pathognomic presentation includes malaise, nausea, vomiting, epigastric pain and jaundice in the third trimester of pregnancy.[1]

Due to high maternal and perinatal mortality associated with AFLP, early diagnosis, prompt delivery and supportive care are essential to save maternal life. We report a typical case of AFLP and discuss the management and complications of this rare obstetric disorder.


  Case Report Top


A 26-year-old previously healthy woman, Gravida 2 Para 1, presented to the emergency room (ER) of Dubai Hospital at 34+4 weeks' gestation with a 3-day history of malaise, nausea, vomiting, fever, abdominal pain and decreased foetal movements. Her pregnancy had been uncomplicated until presentation.

On admission, the patient looked unwell, jaundiced with peripheral cyanosis. She was haemodynamically stable with blood pressure of 122/76 mmHg and pulse of 93 bpm. Her obstetric examination revealed 34-week-size uterus with cephalic presentation. Vaginal examination revealed 2–3 cm dilated cervix with intact membranes.

Obstetric ultrasound in ER showed a single foetus with no foetal heart, and intrauterine death was confirmed. The patient was admitted to labour suite, and laboratory and imaging investigations were requested.

Her laboratory investigations results were as follows: haemoglobin (Hb) 16.3 g/dL, white blood cell count 33,000/μL with neutrophilia 26,100/μL, platelets 192,000/μL, urea 44 mg/dL, creatinine 3.24 mg/dL, serum Na + 133 mmol/L and serum K + 4.9 mmol/L. Urine showed 1 + proteinuria and no pus cells or bacteria.

Liver function tests (LFTs) showed total bilirubin of 12.1 mg/dL with indirect bilirubin of 2.9 mg/dL and direct bilirubin of 9.2 mg/dL, serum glutamic pyruvic transaminase (SGPT) of 156 U/L, serum glutamic oxaloacetic transaminase (SGOT) of 268U/L, alkaline phosphatase of 474 U/L, blood albumin of 2.7 g/dL, globulin of 2.0 g/dL, amylase of 15 U/L, lipase of 19.30 U/L, lactate dehydrogenase of 2094 U/L and ammonia of 96 umol/L.

Malarial parasite was not seen. Hepatitis A immunoglobulin (Ig) G and IgM were negative. Hepatitis B surface antigen and hepatitis C antibodies were negative. Blood glucose was 50 mg/dl.

Activated partial thromboplastin time (APTT) was 62.6 s, fibrinogen was 106.8 mg/dL, international normalised ratio (INR) was 2.2, prothrombin time (PT) was 21.9 s, D-dimer was 4.4, C-reactive protein was 33.0 mg/L, procalcitonin was 3.05 ng/mL, lactic acid was 3.5 mmol/L on arterial blood gas analysis, and TORCH screening for toxoplasma, Epstein–Barr virus, Cytomegalovirus, rubella, Venereal Disease Research Laboratory and Parvo virus was negative. Antimitochondrial antibodies were negative and antinuclear factor was negative. Blood culture was obtained which later reported no growth.

Abdominal ultrasound scan (USS) showed liver which is normal in size and texture, with no intrahepatic biliary duct dilatation or vascular congestion. Common bile duct and portal vein were normal in calibre. The gallbladder was mildly thick walled; there was no evidence of cholelithiasis. Pancreas, spleen and both kidneys were normal in size and texture. Based on the clinical presentation and laboratory results, the diagnosis of AFLP was made.

Her management involved multidisciplinary team care of obstetricians, physicians, surgeons, gastroenterologists and nephrologists. The surgical team ruled out any surgical cause for her jaundice.

The patient was started initially on intravenous (IV) fluid infusion and once the laboratory results confirmed hypoglycaemia of 50 mg/dl, she was given 25 ml of 50% dextrose each hour as needed to keep blood glucose levels above 80 mg/dl. The patient was attended by the medical and gastroenterology teams who started her on IV antibiotics and advised close monitoring on liver function, platelets, coagulation profile and repeat USS of the abdomen.

It was decided to expedite her delivery; therefore, the patient was started on oxytocin for labour augmentation and had a vaginal delivery 2 h later of a stillbirth baby girl weighing 2 kg. The placenta was sent for histopathology, foetal swabs were taken and fluorescence in situ hybridisation karyotype was sent.

The patient was shifted to the Intensive Care Unit (ICU) for close monitoring. Due to her deranged liver and coagulation parameters, she received fresh frozen plasma (FFP) and cryoprecipitate infusion.

Six hours post-delivery, the patient had significant vaginal bleeding in the ICU. She was transferred back to the operation theatre (OT), and the findings showed vaginal laceration and atonic uterus. The lacerations were sutured and an intrauterine Bakri balloon was inserted. The patient had correction of her coagulopathy by transfusion of packed red blood cell, platelets, cryoprecipitate and FFP. The balloon was removed after 24 h with minimal bleeding.

Twenty-four hours post-balloon removal, she had further vaginal bleeding which was explored in the OT and a vaginal pack was inserted. The following day, the patient started to have active bleeding again, and her coagulation profile was slightly deranged with platelet count of 52,000 μ/L, INR of 1.8, PT of 17.4 s and APTT of 48.7 s, so she was re-examined in the OT and haemostasis was achieved. Later, her LFTs and coagulation profile showed total bilirubin of 4.6 mg/dL, SGPT of 22 U/L, SGOT of 44 U/L, total protein of 5.0 g/dL, albumin of 3.3 g/dL, globulin of 1.7 g/dL, alkaline phosphatase of 87 U/L, Hb of 8.4 g/dL, platelet of 56,000/μL, INR of 1.3 with PT 14.3 s and APTT 39.3 s. Her bilirubin normalised to 1.8 mg/dL after 9 days of delivery.

She was discharged home on day 10 post-delivery in a stable condition.


  Discussion Top


AFLP is a sudden severe illness occurring almost exclusively in the third trimester, in which microvesicular fatty infiltration may result in encephalopathy and hepatic failure.[2]

AFLP is a diagnosis of exclusion. Although severe preeclampsia is more common than AFLP and other liver disorders related to pregnancy, our patient's presentation and laboratory test results were in favour of an AFLP diagnosis.

AFLP was first described by Sheehan in 1940[3] as an ‘acute yellow atrophy of the liver.’ It is characterised by microvesicular fatty infiltration of hepatocytes without any inflammation or necrosis. It has an incidence of approximately 1 in 10,000–15,000 pregnancies.[4] AFLP is unique to pregnancy and tends to occur more commonly in nulliparous women.[5]

The aetiology of the disease is unknown. It is a life-threatening obstetrical emergency that can lead to high maternal and perinatal morbidity and mortality. Due to the high level of clinical suspicion and early diagnosis and the implementation of active multidisciplinary treatment including early termination of pregnancy, there has been a significant reduction in both maternal and foetal mortality rates.[1]

Women who develop AFLP are more likely to have a heterozygous long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency. LCHAD is found on the mitochondrial membrane and is involved in the beta oxidation of long-chain fatty acids. This is a recessive gene mutation. Women who are carriers of LCHAD mutation have an increased risk of recurrence of AFLP in 20%–70% of pregnancies.[6]

Six or more of the following features are used to diagnose AFLP in the absence of other explanation: vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, elevated bilirubin >14 μmol/L, hypoglycaemia <4 mmol/L, elevated urate >340 μmol/L, leucocytosis 11 × 109 L, ascites or bright liver on ultrasound, elevated tramsaminases, elevated ammonia >47 μmol/L, renal impairment creatinine >150 μmol/L, coagulopathy (PT >14 s or APTT >34 s) or microvesicular steatosis on liver biopsy.[7]

Our patient had vomiting, abdominal pain, elevated bilirubin and transaminases and elevated creatinine and coagulopathy with no proteinuria at presentation.

The diagnosis of AFLP remains challenging since there is no specific non-invasive diagnostic test to identify it. Ultrasound and computed tomography scans of the liver have been used, but the specificity and sensitivity of these studies are insufficient to make a diagnosis. Although liver biopsy is the gold standard for confirming the diagnosis, it is rarely used in clinical practice.[2]

Induction of labour with an attempt for vaginal delivery within 24 h is a reasonable approach. Due to the associated coagulopathy, epidural or spinal analgesia is contraindicated.[2]

Caution should be exercised to avoid vaginal trauma and lacerations during vaginal delivery. In the case of caesarean delivery, the patient should receive general anaesthesia. Prophylactic antibiotics are recommended.

Recovery is usually complete with no signs of chronic liver disease. Sepsis and haemorrhagic complications remain the most life-threatening.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ko H, Yoshida EM. Acute fatty liver of pregnancy. Can J Gastroenterol 2006;20:25-30.  Back to cited text no. 1
    
2.
Powrie RO, Greene MF, Camann W, editors. de Swiet's Medical Disorders in Obstetric Practice. 5th ed. London, UK: Blackwell Publishing Ltd.; 2010.  Back to cited text no. 2
    
3.
Sheehan HL. The pathology of acute yellow atrophy and delayed chloroform poisoning. J Obstet Gynaecol Br Emp 1940;47:49-62.  Back to cited text no. 3
    
4.
Wattson WJ, Seeds JW. Acute fatty liver of pregnancy. Obstet Gynecol Surv 1990;45:5891.  Back to cited text no. 4
    
5.
Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol 2007;109:956-66.  Back to cited text no. 5
    
6.
Ibdah JA. Acute fatty liver of pregnancy: An update on pathogenesis and clinical implications. World J Gastroenterol 2006;12:7397-404.  Back to cited text no. 6
    
7.
Ch'ng CL, Morgan M, Hainsworth I, Kingham JG. Prospective study of liver dysfunction in pregnancy in Southwest Wales. Gut 2002;51:876-80.  Back to cited text no. 7
    

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Correspondence Address:
Saba Yehya AlSayari,
Department of Obstetrics and Gynaecology, Dubai Hospital, Al Baraha
Dubai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2227-2437.240727





 

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