Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

Baris Turkbey; Iclal Ocak; Kailash Daryanani; Esperanza Font-Montgomery; Linda Lukose; Joy Bryant; Maya Tuchman; Parvathi Mohan; Theo Heller; William A. Gahl; Peter L. Choyke; Meral Gunay-Aygun

doi:10.1007/s00247-008-1064-x

Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

Baris Turkbey, Iclal Ocak, Kailash Daryanani, Esperanza Font-Montgomery, Linda Lukose, Joy Bryant, Maya Tuchman, Parvathi Mohan, Theo Heller, William A. Gahl, Peter L. Choyke, Meral Gunay-Aygun

Research output: Contribution to journal › Review article › peer-review

74 Scopus citations

Abstract

ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.

Original language	English (US)
Pages (from-to)	100-111
Number of pages	12
Journal	Pediatric radiology
Volume	39
Issue number	2
DOIs	https://doi.org/10.1007/s00247-008-1064-x
State	Published - Feb 2009
Externally published	Yes

Keywords

Autosomal recessive polycystic kidney disease
Children
Congenital hepatic fibrosis
MR cholangiography
US

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Radiology Nuclear Medicine and imaging

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10.1007/s00247-008-1064-x

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title = "Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)",

abstract = "ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.",

keywords = "Autosomal recessive polycystic kidney disease, Children, Congenital hepatic fibrosis, MR cholangiography, US",

author = "Baris Turkbey and Iclal Ocak and Kailash Daryanani and Esperanza Font-Montgomery and Linda Lukose and Joy Bryant and Maya Tuchman and Parvathi Mohan and Theo Heller and Gahl, {William A.} and Choyke, {Peter L.} and Meral Gunay-Aygun",

note = "Funding Information: Acknowledgements We thank the ARPKD/CHF Alliance and all patients and their families who generously participated in this investigation. Supported by the intramural research programs of the National Human Genome Research Institute, National Cancer Institute, National Institute of Diabetes and Digestive and Kidney Diseases, and the National Institutes of Health Clinical Center.",

year = "2009",

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doi = "10.1007/s00247-008-1064-x",

language = "English (US)",

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T1 - Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

AU - Turkbey, Baris

AU - Ocak, Iclal

AU - Daryanani, Kailash

AU - Font-Montgomery, Esperanza

AU - Lukose, Linda

AU - Bryant, Joy

AU - Tuchman, Maya

AU - Mohan, Parvathi

AU - Heller, Theo

AU - Gahl, William A.

AU - Choyke, Peter L.

AU - Gunay-Aygun, Meral

N1 - Funding Information: Acknowledgements We thank the ARPKD/CHF Alliance and all patients and their families who generously participated in this investigation. Supported by the intramural research programs of the National Human Genome Research Institute, National Cancer Institute, National Institute of Diabetes and Digestive and Kidney Diseases, and the National Institutes of Health Clinical Center.

PY - 2009/2

Y1 - 2009/2

N2 - ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.

AB - ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.

KW - Autosomal recessive polycystic kidney disease

KW - Children

KW - Congenital hepatic fibrosis

KW - MR cholangiography

KW - US

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M3 - Review article

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VL - 39

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JO - Pediatric Radiology

JF - Pediatric Radiology

SN - 0301-0449

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ER -

Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

Abstract

Keywords

ASJC Scopus subject areas

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