Acute Fatty Liver of Pregnancy: Letter To Editor

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Letter to Editor

Acute Fatty Liver of hours and oozing was still present. Repacking was done.
Liver enzymes kept on increasing (maximum serum

Pregnancy bilirubin 15.5 mg%, SGOT 3450 IU/L, SGPT 2600 IU/L
on postpartum day 3) and clinically she was in grade 1
to grade 2 encephalopathy. Viral markers for hepatitis
A, B, C and E were negative. Following hemodialysis for
Dear Editor, the fourth continuous day, her vitals were found to be
Acute fatty liver of pregnancy (AFLP) is a rare, stabilized. Vaginal pack was removed after 48 hours of
potentially life‑threatening, pregnancy‑related disease repacking and she was extubated on fourth postpartum
that affects 1 in 7000 to 16,000 pregnancies. [1]. The day. LFT though decreasing continued to be deranged.
condition occurs more commonly in primigravidas, Urine output was 450 ‑ 500ml/24 h. Hemodialysis was
multiple pregnancy, and pregnancies carrying a male done every alternate day till 16th postpartum day. During
fetus.[2] At presentation, one should keep in mind other this period she developed ascites and bilateral pleural
pregnancy‑related liver disease that mimic AFLP such effusion owing to hypoproteinemia for which she was
as HELLP syndrome (hemolysis, elevated liver enzymes, treated with albumin. Alternate day USG carried out and
and low platelet count). Bleeding and disseminated showed improvement in fatty changes, disappearance of
intravascular coagulation (DIC) are one of most common ascites and pleural effusion. Liver enzymes normalized
complications. on 19th postpartum day. Urine output started increasing
and 25th postpartum day she was discharged.
We report that a 21‑year‑old primigravida
( B M I –   2 0 . 4   k g /  m 2 ) w i t h t w i n p r e g n a n c y Acute fatty liver almost always manifests late in pregnancy
(diamniotic‑dichorionic), admitted with labor pains at with a mean gestational age of 37.5 weeks;[3] some do not
35 weeks and 3 days period of gestation with blood become clinically evident until delivery.[4] Persistent
pressure of 150/96 mm Hg and urine albumin of 1+. nausea and vomiting are major symptoms with half of
Her biochemical and hematological parameters were affected women might have hypertension, proteinuria
within normal limits. She delivered 2 live female babies and edema, alone or in combination. According to
by vaginal delivery with episiotomy weighing 2.25 kg the Swansea criteria,[5] six or more of the following
and 1.9 kg at 20:25 hrs and 20:28 hrs, respectively. features are used to diagnose AFLP in the absence
Post‑delivery at 02:00 hrs, vulval hematoma was drained of other explanations: vomiting; abdominal pain;
in OT. Postoperatively, she was found to have pallor polydipsia/polyuria; encephalopathy; elevated bilirubin
and minimal vaginal oozing. She was given 2 PRBC >14 μmol/L (0.8 mg%); hypoglycemia <4 mmol/L
and 10 mg vitamin K. Four hours later, blood clots were (72 mg%); elevated urate >340 μmol/L (5.7 mg%);
found in vagina (approx 800 ml). Exploration under GA leukocytosis >11 × 10 9 /L; ascites or bright liver
was done. Tear was excluded. There was active bleeding on ultrasound; elevated transaminases; elevated
from the uterus, oozing from the episiotomy site. Vaginal ammonia >47 μmol/L (27.5 mg%); renal impairment
packing, inj. PGF2 alpha 02 doses at 20 min apart, 4 creatinine >150 μmol/L (1.7mg%); coagulopathy
units of PRBC and 8 FFP were given in the OT, and she (PT >14 sec or APTT >34 sec), or microvesicular steatosis
was shifted to ICU. Hematological investigations were on liver biopsy. Using these criteria, we anticipated
suggestive of consumptive coagulopathy with deranged the diagnosis of AFLP. The patient had elevated
liver enzymes and coagulation parameters [SGOT: 756 bilirubin, elevated transaminases, elevated creatinine,
IU/L, SGPT: 356 IU/L, LDH: 2754 IU/L, INR: 3.19, encephalopathy, leukocytosis, fatty liver grade 2‑3 and
platelets 130,000/mm3, PT: 32 sec (13), PTTK: 39 sec (27), coagulopathy. A patient with severe preeclampsia/
TLC 19,000/mm3]. She was given injectable antibiotics, HELLP will usually present with proteinuria.
FFP 2 units 6 hourly and cryoprecipitates 8 units. She was
put on ventilator at 15:00 hrs in P‑SIMV mode, PEEP 5, Systemic complications of AFLP are due to fulminant
and respiratory rate 15/min. Investigations 6 hours later hepatic failure and include encephalopathy, acute renal
showed increasing liver enzymes, INR 2.94, platelets failure, infection, pancreatitis, gastrointestinal hemorrhage,
48,000/mm3, Hb 6.5 gm% with low urine output. In coagulopathy, and at least mild hypoglycemia. Symptoms
view of ARF and risk of fluid overload, dialysis was may rapidly progress from restlessness, confusion,
started along with frusemide infusion. She continued on and disorientation to asterixis, seizures, psychosis,
blood products. USG abdomen revealed hepatomegaly and ultimately coma.[6] Other systemic effects include
with fatty changes grade 2‑3, medical renal disease, respiratory failure, sometimes requiring assisted
and minimal ascites. Vaginal packing removed after 24 ventilation, ascites,[4] and gastrointestinal bleeding

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Letter to Editor

from gastric ulceration and Mallory‑Weiss syndrome.[7] References


Hepatorenal syndrome eventually develops and leads to
oliguria and acute tubular necrosis.[6] Ultrasound and CT 1. Reyes H, Sandoval L, Wainstein A, Ribalta J, Donoso S,
Smok G, et al. Acute fatty liver of pregnancy: A clinical study
scans of the liver have been used for diagnosis, but the
of 12 episodes in 11 patients. Gut 1994;35:101‑6.
specificity and sensitivity of these studies are insufficient 2. Fesenmeier MF, Coppage KH, Lambers DS, Barton JR, Sibai
to make a diagnosis and the likelihood of false negative BM. Acute fatty liver of pregnancy in 3 tertiary care centres.
results is high.[4] Liver biopsy is the gold standard test, Am J Obstet Gynaecol 2005;192:1416‑9.
but it is invasive and requires a patient with normal 3. Castro MA, Goodwin TM, Shaw KJ, Ouzounian JG,
coagulation status.[3] McGehee WG. Disseminated intravascular coagulation and
antithrombin III depression in acute fatty liver of pregnancy.
Am J Obstet Gynecol 1996a;174:211‑6.
Supportive care of patients with AFLP should include
4. Bacq Y. Liver diseases unique to pregnancy: A 2010 update.
careful monitoring for evidence of progressive hepatic
Clin Res Hepatol Gastroenterol 2011;35:182‑93.
failure, hypoglycemia, and coagulopathy. This should
5. Joshi D, James A, Quaglia A, Westbrook RH, Heneghan MA.
occur in an intensive care setting and in consultation with Liver disease in pregnancy, Lancet 2010;375:594‑605.
physicians well‑versed in the care of critically ill patients. 6. Castro MA, Fassett MJ, Reynolds TB, Shaw KJ, Goodwin
Spontaneous resolution usually follows delivery. Maternal TM. Reversible peripartum liver failure: A new perspective
deaths are caused by sepsis, hemorrhage, aspiration, on the diagnosis, treatment, and cause of acute fatty liver
renal failure, pancreatitis and gastrointestinal bleeding.[8] of pregnancy, based on 28 consecutive cases. Am J Obstet
Although maternal mortality rates in the past approached Gynecol 1999;181:38995.
75 percent but Sibai (2007) cites an average mortality rate 7. Ibdah JA. Acute fatty liver of pregnancy: An update on
pathogenesis and clinical implications. World J Gastroenterol
of 7 percent with 70 percent preterm delivery rate and 2006;12:7397 ‑404.
perinatal mortality rate of approximately 15 percent, 8. Martin JN Jr, Briery CM, Rose CH, Owem MT, Bifill JA,
which in the past was nearly 90 percent.[9] Early diagnosis, Files JC. Postpartum plasma exchange as adjunctive therapy
prompt therapy, adequate supportive care and a for severe acute fatty liver of pregnancy. J Clin Apher
multidisciplinary approach are the key to a good outcome. 2008;23:138‑43.
9. Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol
2007;199:956‑66.
Acknowledgement
Col PJ Kumar (Nephrologist), Col GS Narula (Critical Care), Lt
Col Shalin Trivedi, Maj Ashish Vadhera (Anesthesist).
Access this article online

Madhusudan Dey, Kumar Reema Quick Response Code:


Website:
www.najms.org

Department of Obstetrics and Gynecology,


Armed Forces Medical Services, Military Hospital, DOI:
Jalandhar, Punjab, India. 10.4103/1947-2714.103339
E-mail: [email protected]

612 North American Journal of Medical Sciences | November 2012 | Volume 4 | Issue 11 |

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