Long-term Outcome After Cholecystocolostomy For Progressive Familial Intrahepatic Cholestasis

Part one:The effect of defecation frequency after the cholecysto colonic Roux-Y anastomosis on the prognosis of patients with progressive familial intrahepatic cholestasisObjective:To investigate the relationship between defecation frequency and long-term outcomes of cholecystocolostomy for patients with progressive familial intrahepatic cholestasis.Methods:We retrospectively analyzed the characteristics of patients with PFIC who successfully underwent cholecystocolostomy for bile diversions in our institution from Auguest 2003 to January 2017.And comparison the relationship between the defecation frequency and the prognosis of the patients.Results:The mean age of the 41 patients was 40 months,The median age was 17 months,22 patients were male,19 patients were female.The follow-up periods was 3 to 173 months.The prognosis of 30 patients with 2-4 times daily defecation frequency was better,only 1 case was reoperated after surgery.2 children who had more than 5 defecation frequency every day after surgery were died of intractable diarrhea.6 of 9 cases with no more than 1 times daily defecation frequency were experienced poor postoperative effect,incluing 1 case died,2 cases of liver transplantation,3 cases recurrenced of symptoms and 2 of them experienced reoperation.Conclusions:For patients with progressive familial intrahepatic cholestasis,the defecation frequency is closely related to the prognosis after cholecystocolostomy,and is an important indicator of long-term prognosis.Part two:Long-term outcome after cholecystocolostomy for progressive familial intrahepatic cholestasisObjective:To investigate the long-term outcomes after cholecystocolostomy for patients with progressive familiar intrahepatic cholestasis in our institution.Methods:We retrospectively analyzed the characteristics of 34 patients with PFIC who underwent cholecystocolostomy for bile diversions in our institution.Outcomes included changes in serum bile acids,bilirubin,pruritus,liver enzymes,and survival with native liver were assessed in a regular follow-up.Results:There are 34 patients underwent cholecystocolostomy for bile diversions.Partial liver function parameters significantly decreased postoperatively.serum total bilirubin(0 = 163.54±106.02,36 mos.= 23.38± 17.66?mol/L)and bile acid(0 ?325.83±153.09,36 mos.= 48.36± 79.71 ? mol/L)decreased after cholecystocolostomy in PFIC patients(P<0.001).All patients experienced decreased severity of pruritus(88.2%vs.12.9%,P<0.001)and a greater freedom from growth retardation after cholecystocolostomy(height 0 =-3.35,36 mos.=-1.03,P<0.001;weight 0 =-3.67,36 mos.=-1.65,P<0.001).Defecation frequency increased in PFIC patients after cholecystocolostomy(P = 0.002).29 patients with native liver survival have not yet required liver transplantation after cholecystocolostomy.The median follow-up period was 81 months(3-171 months).2 patients undertwent reoperation(60,83 months post cholecystocolostomy);2 patients undertwent orthotopic liver transplantation postoperative(20,39 months post cholecystocolostomy);3 patients died at home due to gastroenteritis-associated dehydration and cholangitis-induced septic shock before transplantation.4 patients suffered from severe constipation,all associated with high serum bile acids(>200 umol/L)and pruritus.There were 23 patients suffered increased frequency of defecation,but these patients all have good outcomes and no complication associated with fat malabsorption occurred,the blood bile acids in these 23 patients were normal or near normal.And they all developed completely or near-completely free from jaundice and pruritus.Conclusions:Cholecystocolostomy is a safe and effective method for PFIC patients in decreasing TBA level and alleviating jaundice and pruritus.However,the defecation after cholecystocolostomy may be vital for the prognosis of PFIC patients,increased frequency of defecation is benefical to alleviate the clinical symptoms.This is the first long-term large scale analysis of cholecystocolostomy approaches to PFIC.Approaches single and well tolerated,and generally result in improvement of pruritus and cholestasis.