Successful liver transplantation in a child with Caroli’s disease

Meier, Carolin; Deutscher, J; Müller, Susanna; Haluany, Karin; Fangmann, J.; Siekmeyer, Werner; Richter, Thomas; Kieß, Wieland

doi:10.1111/j.1399-3046.2007.00879.x

Cited by 10 publications

(8 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The indication for transplant was recurrent cholangitis in only 1 patient in this cohort. In the patients with isolated liver transplants, posttransplant renal function was documented in 5 patients (24–26). The 3 patients with ARPKD described by Arikan et al (24) had improved renal function 18 months after liver transplantation.…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

View full text Add to dashboard Cite

Objectives The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD). Methods A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation. Results Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0–38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33–75 years). There was a relative paucity of data on combined liver-kidney transplantation. Conclusions Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.

show abstract

Section: Resultsmentioning

confidence: 99%

“…The patient with iCHF studied by Dehghani (25) had normal renal function 7 months after liver transplant. Renal function acutely declined after liver transplantation in the patient described by Meier et al (26), and was believed to be secondary to acyclovir used for the treatment of varicella zoster virus infection.…”

Section: Resultsmentioning

confidence: 99%

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

View full text Add to dashboard Cite

show abstract

“…Data show that liver transplantation for CD or CS is a secure procedure with an encouraging long-term outcome. Results are comparable to those who undergo OLT for other etiologies of chronic liver disease [11, 14, 28, 29]. …”

Section: Discussionmentioning

confidence: 56%

Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases

et al. 2011

View full text Add to dashboard Cite

Caroli's disease (CD) is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts. Patients with bilobar or progressive disease may require orthotopic liver transplantation (OLT). In the MELD era, living donor liver transplantation (LDLT) raised as the ultimate treatment option for these patients, once their MELD score is usually low. Herein, we describe 2 cases of patients (a 2-year-old girl and a 19-year-old teenager) that successfully underwent LDLT as a treatment for diffuse CD. The good postoperative courses of the two cases indicate that LDLT is a feasible option in the treatment of this disorder, even in complicated or early age patients.

show abstract

“…Until now, the youngest pediatric recipient described in a case report was an 8-year-old girl who initially presented at 4.5 years of age. The patient received a whole organ graft and remained well during the 57 months of follow-up [13] (Table 1).…”

Section: Discussionmentioning

confidence: 95%