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Table of Contents:
Preface
Polycystic Kidney Disease: Growth of Cysts and Recognition of Phenotypes 1
Molecular Genetics of ADPKD 8
Pathogenesis of Renal Cysts in Dominantly Inherited Polycystic Kidney Disease 16
ADPKD: Practical Implications of Population Genetics 20
The Rate of Progression of Renal Failure in ADPKD 28
The Patient with ADPKD on Maintenance Hemodialysis 33
Ethical Issues and Genetic Counselling 39
Polycystic Liver Disease 44
ADPKD-Associated Intracranial Aneurysm: New Insights and Unanswered Questions 53
Hypertension in ADPKD 59
Is Progression of Renal Involvement Similar in Twins with ADPKD? A Multicentric European Study 65
Clinical Data of a Cooperative Italian Study of ADPKD 72
Clinical Applications of Genetic Linkage Analysis for the Molecular Diagnostics of ADPKD, Using DNA Markers Linked to the PKD1 and PKD2 Genes 88
Weak Evidence for Allelic Association in the Cypriot PKD1 Population 93
Interfamilial and Intrafamilial Variability of Clinical Expression in ADPKD 97
Molecular Genetic Analysis of ADPKD in Irish Families 102
Urinary Epidermal Growth Factor Concentration in Patients Affected by ADPKD 105
Lipid Peroxidation and Activity of Superoxide Dismutase in ADPKD 109
Tubulointerstitial Changes in the Han:SPRD Rat Model for ADPKD 113
Plasma Concentration of Endothelin and Arterial Pressure in Patients with ADPKD 118
Regulation by TGF-[beta] and bFGF of ECM Expression by PKD Cells 'In Culture' 122
Polycystic Liver Disease: Immunohistochemical Characterization of Cyst Epithelia and Extracellular Matrix 127
Effects of Reduced Protein Intake in Rats with Congenital Polycystic Kidney without Renal Failure 134
Enrolling ADPKD Patients for Renal Transplantation: Clinical and Ethical Issues 137
Clinical Features and Outcome of ADPKD Patients after Renal Transplantation 141
Renal Transplantation in Polycystic Kidney Disease 144
Is Nephrectomy Indicated in Uremic Patients with ADPKD Waiting for Transplant? 150
Daily Hemofiltration for an ADPKD Liver Failure Patient 154
Cardiac Valvular Abnormalities in ADPKD. Preliminary Results from the Italian Multicentric Study
Budd-Chiari Syndrome following Pretransplantation Nephrectomy in an ADPKD Patient with Liver Cysts 163
Magnetic Resonance Angiography and Intracranial Aneurysms in Polycystic Kidney Disease. A Preliminary Study 167
Least Squares Regression Analysis of Arterial Pressure Estimators Contributing to Early Renal Failure in ADPKD 171
Polycystic Kidney Disease Patients on Renal Replacement Therapy: Data from the Catalan Renal Registry 177
Vessel Malformation in a Patient with ADPKD: Megadolichobasilar Artery, Abdominal Aortic and Iliac Aneurysms 182
Presymptomatic Diagnosis of ADPKD in Childhood: Ultrasonographic, Computed Tomography and Gene Linkage Analysis Studies 185
Subject Index 189
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