Single gene ciliopathy characterized by dilated distal convoluted tubules & collecting ducts
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. ePub, 2016
Dell KM et al: Kidney disease progression in autosomal recessive polycystic kidney disease. J Pediatr. 171:196-201.e1, 2016
Park E et al: Hepatorenal fibrocystic diseases in children. Pediatr Nephrol. 31(1):113-9, 2016
Büscher R et al: Combined liver and kidney transplantation and kidney after liver transplantation in children: Indication, postoperative outcome, and long-term results. Pediatr Transplant. 19(8):858-65, 2015
Cramer MT et al: Cystic kidney disease: a primer. Adv Chronic Kidney Dis. 22(4):297-305, 2015
Obusez EC et al: Autosomal recessive polycystic kidney disease with Caroli syndrome. J Urol. 193(2):679-80, 2015
Guay-Woodford LM: Autosomal recessive polycystic kidney disease: the prototype of the hepato-renal fibrocystic diseases. J Pediatr Genet. 3(2):89-101, 2014
Guay-Woodford LM et al: Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 165(3):611-7, 2014
Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
Bergmann C et al: Clinical consequences of PKHD1 mutations in 164 patients with autosomal-recessive polycystic kidney disease (ARPKD). Kidney Int. 67(3):829-48, 2005
Traubici J et al: High-resolution renal sonography in children with autosomal recessive polycystic kidney disease. AJR Am J Roentgenol. 184(5):1630-3, 2005
Zerres K et al: New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene. Clin Genet. 66(1):53-7, 2004
Guay-Woodford LM et al: Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 111(5 Pt 1):1072-80, 2003
Stein-Wexler R et al: Sonography of macrocysts in infantile polycystic kidney disease. J Ultrasound Med. 22(1):105-7, 2003
Avni FE et al: Hereditary polycystic kidney diseases in children: changing sonographic patterns through childhood. Pediatr Radiol. 32(3):169-74, 2002
Single gene ciliopathy characterized by dilated distal convoluted tubules & collecting ducts
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. ePub, 2016
Dell KM et al: Kidney disease progression in autosomal recessive polycystic kidney disease. J Pediatr. 171:196-201.e1, 2016
Park E et al: Hepatorenal fibrocystic diseases in children. Pediatr Nephrol. 31(1):113-9, 2016
Büscher R et al: Combined liver and kidney transplantation and kidney after liver transplantation in children: Indication, postoperative outcome, and long-term results. Pediatr Transplant. 19(8):858-65, 2015
Cramer MT et al: Cystic kidney disease: a primer. Adv Chronic Kidney Dis. 22(4):297-305, 2015
Obusez EC et al: Autosomal recessive polycystic kidney disease with Caroli syndrome. J Urol. 193(2):679-80, 2015
Guay-Woodford LM: Autosomal recessive polycystic kidney disease: the prototype of the hepato-renal fibrocystic diseases. J Pediatr Genet. 3(2):89-101, 2014
Guay-Woodford LM et al: Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 165(3):611-7, 2014
Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
Bergmann C et al: Clinical consequences of PKHD1 mutations in 164 patients with autosomal-recessive polycystic kidney disease (ARPKD). Kidney Int. 67(3):829-48, 2005
Traubici J et al: High-resolution renal sonography in children with autosomal recessive polycystic kidney disease. AJR Am J Roentgenol. 184(5):1630-3, 2005
Zerres K et al: New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene. Clin Genet. 66(1):53-7, 2004
Guay-Woodford LM et al: Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 111(5 Pt 1):1072-80, 2003
Stein-Wexler R et al: Sonography of macrocysts in infantile polycystic kidney disease. J Ultrasound Med. 22(1):105-7, 2003
Avni FE et al: Hereditary polycystic kidney diseases in children: changing sonographic patterns through childhood. Pediatr Radiol. 32(3):169-74, 2002
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