Clinical profile of autosomal dominant polycystic liver disease

@article{Qian2003ClinicalPO,
  title={Clinical profile of autosomal dominant polycystic liver disease},
  author={Qi Qian and Airong Li and Bernard F. King and Patrick S Kamath and Donna Lager and John C. Huston and Clarence Shub and Sonia Davila and Stefan Somlo and Vicente E. Torres},
  journal={Hepatology},
  year={2003},
  volume={37}
}
Most reports on the natural history, manifestations, and treatment of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The purpose of this study was to develop a clinical profile of isolated autosomal dominant polycystic liver disease (ADPLD) using nonaffected family members as controls. The study included 146 probands, known affected relatives, and first‐degree relatives of affected individuals… 
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Investigation of polycystic liver disease in patients with autosomal dominant polycystic kidney disease using magnetic resonance imaging
TLDR
The incidence of polycystic liver disease in ADPKD patients was found to be high and the presence of PLD was not associated with the clinical findings of AD PKD, and it may be beneficial to perform liver imaging at the time of diagnosis and in their routine follow-up.
New Advances in Evaluation and Management of Patients with Polycystic Liver Disease
TLDR
In the symptomatic APLD patient, surgical therapy remains the mainstay of therapy and includes cyst aspiration and sclerosis, fenestration with and without hepatic resection and orthotopic liver transplantation, to include outcome measurements and complication rates.
Boy with autosomal recessive polycystic kidney and autosomal dominant polycystic liver disease
TLDR
The patient with AR PKD and PCLD has so far demonstrated a benign clinical outcome, consistent with the great phenotypic variability of ARPKD and, apart from the liver cyst, asymptomatic manifestation of PCLD in childhood.
Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases.
TLDR
The current knowledge of the pathophysiology, clinical course, diagnosis and treatment strategies of polycystic liver diseases are outlined.
Progress on Autosomal Dominant Polycystic Kidney Disease
TLDR
Current clinical trials investigating multiple therapeutic targets bring hope for treatments that may impede the progression of ADPKD.
Diagnosis and screening of autosomal dominant polycystic kidney disease.
TLDR
Both DNA linkage or gene-based direct sequencing are now available for the diagnosis of ADPKD, especially in subjects with equivocal imaging results, subjects with a negative or indeterminate family history, or in younger at-risk individuals being evaluated as potential living-related kidney donors.
Polycystic liver disease: a clinical review.
  • N. Chandok
  • Medicine, Biology
    Annals of hepatology
  • 2012
Clinical Correlates of Mass Effect in Autosomal Dominant Polycystic Kidney Disease
TLDR
Moderate to severe PLD is a common and clinically important problem in ~16% of patients with ADPKD who may benefit from referral to specialized centers for further management.
Management of polycystic liver disease
The adult forms of polycystic liver disease are characterized by autosomal dominant inheritance and numerous hepatic cysts, with or without renal involvement. Mutations in two distinct genes…
The Liver and Polycystic Kidney Disease
TLDR
The pathogenic sequence and genetic profile of PKD-associated liver cyst formation and progression is under extensive investigation, and therapeutic strategies to prevent and retard renal and liver cysts growth should be available in the near future.
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References

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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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