Albumin liver dialysis as pregnancy-saving procedure in cholestatic liver disease and intractable pruritus

Abstract

Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating System (MARS) has been reported to reverse severe cholestasis-linked pruritus. Here, we report the first use of MARS during a spontaneous pregnancy and its successful outcome in a patient with PFIC3 and intractable pruritus. Albumin dialysis could be considered as a pregnancy-saving procedure in pregnant women with severe cholestasis and refractory pruritus.

Treatment of resistant pruritus from cholestasis with albumin dialysis: Combined analysis of patients from three centers

“…In patients who do not respond to medical therapies, different invasive procedures, such as hemodialysis [11], charcoal hemoperfusion [12], and plasmapheresis [13][14][15] have been used with positive, although uncertain results, due to the small number of patients evaluated. More recently, the therapeutic effects of various methods of extracorporeal albumin dialysis [16][17][18][19][20][21][22][23][24][25][26][27][28], mainly the molecular adsorbent recirculating system (MARS), have been published [16][17][18][19][20][21][22][23][24][25][26][27]. However, from these case reports or short series (the highest has included seven patients) [21], it is difficult to ascertain the actual efficacy of the procedure in patients with cholestatic pruritus.…”

Biliary transporter gene mutations in severe intrahepatic cholestasis of pregnancy: Diagnostic and management implications

“…Plasmapheresis or Molecular Adsorbent Recycling System provide other treatment options, with the aim of reducing circulating cholestatogenic factors …”

“…Plasmapheresis or Molecular Adsorbent Recycling System provide other treatment options, with the aim of reducing circulating cholestatogenic factors. 38,39 Both Case 1 and Case 3 had flare ups in the immediate postpartum period after stopping or downgrading therapy. Because of her sister's experience, Case 2 remained on rifampicin after delivery, which appeared to control the cholestasis initially, but she eventually required NBD 6 weeks later.…”

Therapeutic plasma exchange as a novel treatment for severe intrahepatic cholestasis of pregnancy: Case series and mechanism of action

“…TPE has been used for pruritus associated with primary biliary cholangitis, with improvement in the majority of the 37 reported cases, 2 of whom were pregnant, 19 and for a patient with pregnancy-related pruritus secondary to progressive familial intrahepatic cholestasis (PFIC) with moderate success, as the patient subsequently required molecular adsorbent recirculating system (MARS) therapy. 20 Four case reports of TPE use for ICP were identified, which report varying success. [21][22][23][24] Thus, it presents a novel therapeutic option for women with severe ICP, with the aims of improving their debilitating pruritus and reducing serum bile acid concentrations (and therefore adverse perinatal outcomes).…”

“…Its use in pregnancy has been limited to conditions typically associated with immunoglobulin‐related disease, dramatically elevated lipids, and severely deranged coagulation. TPE has been used for pruritus associated with primary biliary cholangitis, with improvement in the majority of the 37 reported cases, 2 of whom were pregnant, and for a patient with pregnancy‐related pruritus secondary to progressive familial intrahepatic cholestasis (PFIC) with moderate success, as the patient subsequently required molecular adsorbent recirculating system (MARS) therapy . Four case reports of TPE use for ICP were identified, which report varying success .…”