Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-6bf8c574d5-vmclg Total loading time: 0 Render date: 2025-03-01T07:00:00.266Z Has data issue: false hasContentIssue false

Chapter 41 - Fibrocystic Liver Disease in Children

from Section V - Other Considerations and Issues in Pediatric Hepatology

Published online by Cambridge University Press:  19 January 2021

By
Ronen Arnon ,
Jaime Chu  and
Frederick J. Suchy
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
Get access

Summary

Fibrocystic liver disease refers to a heterogeneous group of disorders with shared, but also distinct, pathophysiologic and clinical features. Cystic dilatation of intrahepatic bile duct structures and variable degrees of portal fibrosis are the hallmarks of fibrocystic liver disease. In many instances, there are morphologic abnormalities in the kidneys that parallel those of the liver. It has been recognized for centuries that hepatic and renal cysts are seen in the same individuals [1], although it has not always been accepted that they are manifestations of the same diseases. The older literature contains confusing descriptive classifications of fibrocystic diseases, with imprecise and overlapping definitions. Even now, attempts at describing clinical and radiographic features, prognosis, natural history, and treatment are somewhat hampered by reliance on these descriptive reports. However, much of the molecular basis for these disorders has been elucidated, and clinical diagnoses are being modified using more exact genetic criteria. The current consensus is that genetic determinants of differentiation and development of renal tubules and biliary structures result in a broad spectrum of congenital abnormalities grouped under the heading of fibrocystic liver and kidney disease.

Type
Chapter
Information
Liver Disease in Children , pp. 749 - 768
Publisher: Cambridge University Press
Print publication year: 2021

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Bristowe, C. Cystic disease of the liver associated with a similar disease of the kidneys. Trans Pathol Soc Lond 1856;7:229–34.Google Scholar
Jorgensen, MJ. The ductal plate malformation. Acta Pathol Microbiol Scand Suppl 1977(257):1–87.Google Scholar
Desmet, VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme “ductal plate malformation.” Hepatology 1992;16(4):1069–83.CrossRefGoogle ScholarPubMed
Rogers, TN, Woodley, H, Ramsden, W, Wyatt, JI, Stringer, MD. Solitary liver cysts in children: not always so simple. J Pediatr Surg 2007;42(2):333–9.Google Scholar
Kerr, DN, Harrison, CV, Sherlock, S, Walker, RM. Congenital hepatic fibrosis. Q J Med 1961;30:91–117.Google Scholar
Ward, CJ, Hogan, MC, Rossetti, S, Walker, D, Sneddon, T, Wang, X, et al. The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet 2002;30(3):259–69.Google Scholar
Bergmann, C, Senderek, J, Windelen, E, Kupper, F, Middeldorf, I, Schneider, F, et al. Clinical consequences of PKHD1 mutations in 164 patients with autosomal-recessive polycystic kidney disease (ARPKD). Kidney Int 2005;67(3):829–48.Google Scholar
Rossetti, S, Torra, R, Coto, E, Consugar, M, Kubly, V, Malaga, S, et al. A complete mutation screen of PKHD1 in autosomal-recessive polycystic kidney disease (ARPKD) pedigrees. Kidney Int 2003;64(2):391–403.Google Scholar
O’Brien, K, Font-Montgomery, E, Lukose, L, Bryant, J, Piwnica-Worms, K, Edwards, H, et al. Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr 2012;54(1):83–9.Google Scholar
Gunay-Aygun, M, Gahl, WA, Heller, T. (1993). Congenital hepatic fibrosis overview. In: Adam, MP, Ardinger, HH, Pagon, RA, Wallace, SE, Bean, LJH, Stephens, K, et al., (Eds.), GeneReviews((R)). Seattle, WA: University of Washington.Google Scholar
Srinath, A, Shneider, BL. Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. J Pediatr Gastroenterol Nutr 2012;54(5):580–7.Google Scholar
Gunay-Aygun, M, Font-Montgomery, E, Lukose, L, Tuchman Gerstein, M, Piwnica-Worms, K, Choyke, P, et al. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology 2013;144(1):112–21 e2.Google Scholar
Summerfield, JA, Nagafuchi, Y, Sherlock, S, Cadafalch, J, Scheuer, PJ. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986;2(2):141–56.Google Scholar
Guay-Woodford, LM, Bissler, JJ, Braun, MC, Bockenhauer, D, Cadnapaphornchai, MA, Dell, KM, et al. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr 2014;165(3):611–17.Google Scholar
Akhan, O, Karaosmanoglu, AD, Ergen, B. Imaging findings in congenital hepatic fibrosis. Eur J Radiol 2007;61(1):18–24.Google Scholar
Kummer, S, Sagir, A, Pandey, S, Feldkotter, M, Habbig, S, Korber, F, et al. Liver fibrosis in recessive multicystic kidney diseases: transient elastography for early detection. Pediatr Nephrol 2011;26(5):725–31.CrossRefGoogle ScholarPubMed
Wicher, D, Jankowska, I, Lipinski, P, Szymanska-Rozek, P, Kmiotek, J, Janczyk, W, et al. Transient elastography for detection of liver fibrosis in children with autosomal recessive polycystic kidney disease. Front Pediatr 2018;6:422.Google Scholar
Hartung, EA, Wen, J, Poznick, L, Furth, SL, Darge, K. Ultrasound elastography to quantify liver disease severity in autosomal recessive polycystic kidney disease. J Pediatr 2019;209:107–15.Google Scholar
Munoz-Garrido, P, Marin, JJ, Perugorria, MJ, Urribarri, AD, Erice, O, Saez, E, et al. Ursodeoxycholic acid inhibits hepatic cystogenesis in experimental models of polycystic liver disease. J Hepatol 2015;63(4):952–61.Google Scholar
Shneider, BL, de Ville de Goyet, J, Leung, DH, Srivastava, A, Ling, SC, Duche, M, et al. Primary prophylaxis of variceal bleeding in children and the role of MesoRex bypass: summary of the Baveno VI Pediatric Satellite Symposium. Hepatology 2016;63(4):1368–80.Google Scholar
Shneider, BL, Magid, MS. Liver disease in autosomal recessive polycystic kidney disease. Pediatric Transplantation 2005;9(5):634–9.Google Scholar
Guay-Woodford, LM, Desmond, RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics 2003;111(5 Pt 1):1072–80.Google Scholar
Garcia-Tsao, G, Sanyal, AJ, Grace, ND, Carey, W. Practice Guidelines Committee of the American Association for the Study of Liver Disease, Practice Parameters Committee of the American College of Gastroenterology. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology 2007;46(3):922–38.Google Scholar
Kinugasa, H, Nouso, K, Kobayashi, Y, Yasunaka, T, Onishi, H, Nakamura, S, et al. Hepatocellular carcinoma occurring in hepatobiliary fibropolycystic disease. Hepatol Res 2011;41(3):277–81.CrossRefGoogle ScholarPubMed
Onuchic, LF, Furu, L, Nagasawa, Y, Hou, X, Eggermann, T, Ren, Z, et al. PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats. Am J Hum Genet 2002;70(5):1305–17.Google Scholar
Lu, H, Galeano, MCR, Ott, E, Kaeslin, G, Kausalya, PJ, Kramer, C, et al. Mutations in DZIP1 L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease. Nat Genet 2017;49(7):1025–34.Google Scholar
Caroli, J, Couinaud, C, Soupault, R, Porcher, P, Eteve, J. A new disease, undoubtedly congenital, of the bile ducts: unilobar cystic dilation of the hepatic ducts. Sem Hop 1958;34(8/2):496–502.Google Scholar
Jordon, D, Harpaz, N, Thung, SN. Caroli’s disease and adult polycystic kidney disease: a rarely recognized association. Liver 1989;9(1):30–5.Google Scholar
Rawat, D, Kelly, DA, Milford, DV, Sharif, K, Lloyd, C, McKiernan, PJ. Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis. J Pediatr Gastroenterol Nutr 2013;57(2):161–6.Google Scholar
Ramond, MJ, Huguet, C, Danan, G, Rueff, B, Benhamou, JP. Partial hepatectomy in the treatment of Caroli’s disease. Report of a case and review of the literature. Dig Dis Sci 1984;29(4):367–70.Google ScholarPubMed
Lendoire, JC, Raffin, G, Grondona, J, Bracco, R, Russi, R, Ardiles, V, et al. Caroli’s disease: report of surgical options and long-term outcome of patients treated in Argentina. Multicenter study. J Gastrointest Surg 2011;15(10):1814–19.Google Scholar
Kim, RD, Book, L, Haafiz, A, Schwartz, JJ, Sorensen, JB, Gonzalez-Peralta, RP. Liver transplantation in a 7-month-old girl with Caroli’s disease. J Pediatr Surg 2011;46(8):1638–41.Google Scholar
Millwala, F, Segev, DL, Thuluvath, PJ. Caroli’s disease and outcomes after liver transplantation. Liver Transpl 2008;14(1):11–17.Google Scholar
Harris, PC, Torres, VE. Polycystic kidney disease. Annu Rev Med 2009;60:321–37.CrossRefGoogle ScholarPubMed
Cornec-Le Gall, E, Torres, VE, Harris, PC. Genetic complexity of autosomal dominant polycystic kidney and liver diseases. J Am Soc Nephrol 2018;29(1):13–23.Google Scholar
Porath, B, Gainullin, VG, Cornec-Le Gall, E, Dillinger, EK, Heyer, CM, Hopp, K, et al. Mutations in GANAB, encoding the glucosidase II alpha subunit, cause autosomal-dominant polycystic kidney and liver disease. Am J Hum Genet 2016;98(6):1193–207.CrossRefGoogle ScholarPubMed
Dell, KM. The spectrum of polycystic kidney disease in children. Adv Chronic Kidney Dis 2011;18(5):339–47.Google Scholar
Gascue, C, Katsanis, N, Badano, JL. Cystic diseases of the kidney: ciliary dysfunction and cystogenic mechanisms. Pediatr Nephrol 2011;26(8):1181–95.Google Scholar
Wilson, PD. Polycystic kidney disease: new understanding in the pathogenesis. Int J Biochem Cell Biol 2004;36(10):1868–73.CrossRefGoogle ScholarPubMed
Lee-Law, PY, van de Laarschot, LFM, Banales, JM, Drenth, JPH. Genetics of polycystic liver diseases. Curr Opin Gastroenterol 2019;35(2):65–72.Google Scholar
Gimpel, C, Bergmann, C, Bockenhauer, D, Breysem, L, Cadnapaphornchai, MA, Cetiner, M, et al. International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol 2019;15(11):713–26.Google Scholar
Bae, KT, Zhu, F, Chapman, AB, Torres, VE, Grantham, JJ, Guay-Woodford, LM, et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol 2006;1(1):64–9.CrossRefGoogle Scholar
Gunay-Aygun, M. Liver and kidney disease in ciliopathies. Am J Med Genet C Semin Med Genet 2009;151 C(4):296–306.Google Scholar
Torres, VE, Harris, PC, Pirson, Y. Autosomal dominant polycystic kidney disease. Lancet 2007;369(9569):1287–301.Google Scholar
Janssens, P, Weydert, C, De Rechter, S, Wissing, KM, Liebau, MC, Mekahli, D. Expanding the role of vasopressin antagonism in polycystic kidney diseases: from adults to children? Pediatr Nephrol 2018;33(3):395–408.Google Scholar
Chebib, FT, Perrone, RD, Chapman, AB, Dahl, NK, Harris, PC, Mrug, M, et al. A practical guide for treatment of rapidly progressive ADPKD with tolvaptan. J Am Soc Nephrol 2018;29(10):2458–70.Google Scholar
Desmet, VJ. Ludwig symposium on biliary disorders–part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 1998;73(1):80–9.Google Scholar
Burns, CD, Kuhns, JG, Wieman, TJ. Cholangiocarcinoma in association with multiple biliary microhamartomas. Arch Pathol Lab Med 1990;114(12):1287–9.Google Scholar
Xu, AM, Xian, ZH, Zhang, SH, Chen, XF. Intrahepatic cholangiocarcinoma arising in multiple bile duct hamartomas: report of two cases and review of the literature. Eur J Gastroenterol Hepatol 2009;21(5):580–4.Google Scholar
Nagano, Y, Matsuo, K, Gorai, K, Sugimori, K, Kunisaki, C, Ike, H, et al. Bile duct hamartomas (von Mayenburg complexes) mimicking liver metastases from bile duct cancer: MRC findings. World J Gastroenterol 2006;12(8):1321–3.Google Scholar
Temmerman, F, Missiaen, L, Bammens, B, Laleman, W, Cassiman, D, Verslype, C, et al. Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther 2011;34(7):702–13.Google Scholar
van Keimpema, L, Nevens, F, Vanslembrouck, R, van Oijen, MG, Hoffmann, AL, Dekker, HM, et al. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology 2009;137(5):1661–8 e1–2.CrossRefGoogle ScholarPubMed
Gevers, TJ, Drenth, JP. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol 2013;10(2):101–8.Google Scholar
Kabbej, M, Sauvanet, A, Chauveau, D, Farges, O, Belghiti, J. Laparoscopic fenestration in polycystic liver disease. Br J Surg 1996;83(12):1697–701.Google Scholar
Hoevenaren, IA, Wester, R, Schrier, RW, McFann, K, Doctor, RB, Drenth, JP, et al. Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease. Liver Int 2008;28(2):264–70.Google Scholar
Drenth, JP, Chrispijn, M, Nagorney, DM, Kamath, PS, Torres, VE. Medical and surgical treatment options for polycystic liver disease. Hepatology 2010;52(6):2223–30.CrossRefGoogle ScholarPubMed
Doshi, SD, Bittermann, T, Schiano, TD, Goldberg, DS. Waitlisted candidates with polycystic liver disease are more likely to be transplanted than those with chronic liver failure. Transplantation 2017;101(8):1838–44.Google Scholar
Waanders, E, te Morsche, RH, de Man, RA, Jansen, JB, Drenth, JP. Extensive mutational analysis of PRKCSH and SEC63 broadens the spectrum of polycystic liver disease. Hum Mutat 2006;27(8):830.Google Scholar
Drenth, JP, Tahvanainen, E, te Morsche, RH, Tahvanainen, P, Kaariainen, H, Hockerstedt, K, et al. Abnormal hepatocystin caused by truncating PRKCSH mutations leads to autosomal dominant polycystic liver disease. Hepatology 2004;39(4):924–31.Google Scholar
Jaeken, J, Matthijs, G, Saudubray, JM, Dionisi-Vici, C, Bertini, E, de Lonlay, P, et al. Phosphomannose isomerase deficiency: a carbohydrate-deficient glycoprotein syndrome with hepatic-intestinal presentation. Am J Hum Genet 1998;62(6):1535–9.Google Scholar
Janssen, MJ, Waanders, E, Te Morsche, RH, Xing, R, Dijkman, HB, Woudenberg, J, et al. Secondary, somatic mutations might promote cyst formation in patients with autosomal dominant polycystic liver disease. Gastroenterology 2011;141(6):2056–63 e2.Google Scholar
Lu, W, Fan, X, Basora, N, Babakhanlou, H, Law, T, Rifai, N, et al. Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotes. Nat Genet 1999;21(2):160–1.CrossRefGoogle ScholarPubMed
Wills, ES, Roepman, R, Drenth, JP. Polycystic liver disease: ductal plate malformation and the primary cilium. Trends Mol Med 2014;20(5):261–70.Google Scholar
Carone, FA, Nakamura, S, Schumacher, BS, Punyarit, P, Bauer, KD. Cyst-derived cells do not exhibit accelerated growth or features of transformed cells in vitro. Kidney Int 1989;35(6):1351–7.Google Scholar
Calvet, JP. Polycystic kidney disease: primary extracellular matrix abnormality or defective cellular differentiation? Kidney Int 1993;43(1):101–8.Google Scholar
Nauta, J, Sweeney, WE, Rutledge, JC, Avner, ED. Biliary epithelial cells from mice with congenital polycystic kidney disease are hyperresponsive to epidermal growth factor. Pediatr Res 1995;37(6):755–63.Google Scholar
Santos-Laso, A, Izquierdo-Sanchez, L, Lee-Law, PY, Perugorria, MJ, Marzioni, M, Marin, JJ, et al. New advances in polycystic liver diseases. Semin Liver Dis 2017;37(1):45–55.CrossRefGoogle ScholarPubMed
Masyuk, AI, Masyuk, TV, Splinter, PL, Huang, BQ, Stroope, AJ, LaRusso, NF. Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular Ca2+ and cAMP signaling. Gastroenterology 2006;131(3):911–20.Google Scholar
Nauli, SM, Alenghat, FJ, Luo, Y, Williams, E, Vassilev, P, Li, X, et al. Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet 2003;33(2):129–37.Google Scholar
Chebib, FT, Sussman, CR, Wang, X, Harris, PC, Torres, VE. Vasopressin and disruption of calcium signalling in polycystic kidney disease. Nat Rev Nephrol 2015;11(8):451–64.Google Scholar
Graf, A, Meng, F, Hargrove, L, Kennedy, L, Han, Y, Francis, T, et al. Knockout of histidine decarboxylase decreases bile duct ligation-induced biliary hyperplasia via downregulation of the histidine decarboxylase/VEGF axis through PKA-ERK1/2 signaling. Am J Physiol Gastrointest Liver Physiol 2014;307(8):G813–23.CrossRefGoogle Scholar
Banales, JM, Masyuk, TV, Gradilone, SA, Masyuk, AI, Medina, JF, LaRusso, NF. The cAMP effectors Epac and protein kinase a (PKA) are involved in the hepatic cystogenesis of an animal model of autosomal recessive polycystic kidney disease (ARPKD). Hepatology 2009;49(1):160–74.CrossRefGoogle ScholarPubMed
Spirli, C, Okolicsanyi, S, Fiorotto, R, Fabris, L, Cadamuro, M, Lecchi, S, et al. ERK1/2-dependent vascular endothelial growth factor signaling sustains cyst growth in polycystin-2 defective mice. Gastroenterology 2010;138(1):360–71 e7.Google Scholar
Spirli, C, Okolicsanyi, S, Fiorotto, R, Fabris, L, Cadamuro, M, Lecchi, S, et al. Mammalian target of rapamycin regulates vascular endothelial growth factor-dependent liver cyst growth in polycystin-2-defective mice. Hepatology 2010;51(5):1778–88.Google Scholar
Alvaro, D, Onori, P, Alpini, G, Franchitto, A, Jefferson, DM, Torrice, A, et al. Morphological and functional features of hepatic cyst epithelium in autosomal dominant polycystic kidney disease. Am J Pathol 2008;172(2):321–32.Google Scholar
Masyuk, TV, Masyuk, AI, LaRusso, NF. Therapeutic targets in polycystic liver disease. Curr Drug Targets 2017;18(8):950–7.Google Scholar
Sanzen, T, Harada, K, Yasoshima, M, Kawamura, Y, Ishibashi, M, Nakanuma, Y. Polycystic kidney rat is a novel animal model of Caroli’s disease associated with congenital hepatic fibrosis. Am J Pathol 2001;158(5):1605–12.Google Scholar
Goto, M, Hoxha, N, Osman, R, Wen, J, Wells, RG, Dell, KM. Renin-angiotensin system activation in congenital hepatic fibrosis in the PCK rat model of autosomal recessive polycystic kidney disease. J Pediatr Gastroenterol Nutr 2010;50(6):639–44.CrossRefGoogle ScholarPubMed
Moyer, JH, Lee-Tischler, MJ, Kwon, HY, Schrick, JJ, Avner, ED, Sweeney, WE, et al. Candidate gene associated with a mutation causing recessive polycystic kidney disease in mice. Science 1994;264(5163):1329–33.Google Scholar
Guay-Woodford, LM, Green, WJ, Lindsey, JR, Beier, DR. Germline and somatic loss of function of the mouse cpk gene causes biliary ductal pathology that is genetically modulated. Hum Mol Genet 2000;9(5):769–78.Google Scholar
Raynaud, P, Tate, J, Callens, C, Cordi, S, Vandersmissen, P, Carpentier, R, et al. A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Hepatology 2011;53(6):1959–66.Google Scholar
Yoder, BK, Richards, WG, Sommardahl, C, Sweeney, WE, Michaud, EJ, Wilkinson, JE, et al. Differential rescue of the renal and hepatic disease in an autosomal recessive polycystic kidney disease mouse mutant. A new model to study the liver lesion. Am J Pathol 1997;150(6):2231–41.Google Scholar
Isfort, RJ, Cody, DB, Doersen, CJ, Richards, WG, Yoder, BK, Wilkinson, JE, et al. The tetratricopeptide repeat containing Tg737 gene is a liver neoplasia tumor suppressor gene. Oncogene 1997;15(15):1797–803.Google Scholar
El-Youssef, M, Mu, Y, Huang, L, Stellmach, V, Crawford, SE. Increased expression of transforming growth factor-beta1 and thrombospondin-1 in congenital hepatic fibrosis: possible role of the hepatic stellate cell. J Pediatr Gastroenterol Nutr 1999;28(4):386–92.Google Scholar
Locatelli, L, Cadamuro, M, Spirli, C, Fiorotto, R, Lecchi, S, Morell, CM, et al. Macrophage recruitment by fibrocystin-defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis. Hepatology 2016;63(3):965–82.Google Scholar
Lee, SO, Masyuk, T, Splinter, P, Banales, JM, Masyuk, A, Stroope, A, et al. MicroRNA15a modulates expression of the cell-cycle regulator Cdc25A and affects hepatic cystogenesis in a rat model of polycystic kidney disease. J Clin Invest 2008;118(11):3714–24.Google Scholar
Hand, NJ, Master, ZR, Eauclaire, SF, Weinblatt, DE, Matthews, RP, Friedman, JR. The microRNA-30 family is required for vertebrate hepatobiliary development. Gastroenterology 2009;136(3):1081–90.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×