Description
Renal cystic diseases include a large number of sporadic and genetically determined congenital, developmental, or acquired conditions. A cyst is a cavity lined by epithelium and filled with fluid. Cysts derive primarily from tubules (from Bowman's capsule to the collecting duct). Cystic kidneys of different etiologies may appear morphologically similar, whereas the same etiologic entity may cause a wide spectrum of renal abnormalities. Simple renal cysts are acquired and are not associated with any disease. In contrast, polycystic kidney diseases can be complicated by renal failure or tumor development. Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially lethal single-gene disorder. Its prevalence at birth is between 1:400 and 1:1,000. It may progress to end stage renal disease by age 60 with 4.4% of patients requiring renal replacement therapy (dialysis or transplant) have ADPKD. The book is a comprehensive guide to diagnosis and management of the condition with special reference and experience around the same in India. The authors have put together the most relevant facts about the disease for an easy comprehension and understanding of the same by practitioners and students across the specialty.
Chapter
Genetics of Autosomal Dominant Polycystic Kidney Disease
Two-Hit Suppressor Model Of Cystogenesis
Preimplantation Genetic Diagnosis (PGD)
Pathology, Pathogenesis and Clinical Presentation of Autosomal Dominant Polycystic Kidney Disease
Proliferation of Epithelial Cells
Role of Growth Factor Receptor Proteins
Role of cAMP in Cyst Epithelium Proliferation
Role of Cytokines in Cyst Proliferation
Role of PC-1 and PC-2 Proteins in Cyst Proliferation
Abnormal Secretory Process in Cyst Development and Expansion
Role of Cell Polarity in Abnormal Fluid Secretion and Cyst Expansion
Disturbed Organization of Extracellular Matrix
Other Factors Influencing Cystogenesis
Role of Genes and Their Proteins in ADPKD
Role of Apoptosis in Cystogenesis
Role of Primary Cilia in Cystogenesis
Hematuria and Cyst Hemorrhage
Urinary Tract Infection and Cyst Infection
Extrarenal Manifestations
Intracranial Aneurysm (ICA)
Other Associated Conditions
The Diagnosis of Autosomal Dominant Polycystic Kidney Disease
Genetic Analysis Of Autosomal Dominant Polycystic Kidney Disease
Imaging Studies In The Diagnosis Of Autosomal Dominant Polycystic Kidney Disease
Complications of Autosomal Dominant Polycystic Kidney Disease
Non-response to antibiotic Therapy
Indications for Intervention
screening Recommendations
Other Vascular abnormalities
Medical Management of Autosomal Dominant Polycystic Kidney Disease
Molecular Pathogenesis And Targeted Therapeutic Options
Increased Apoptosis in Polycystic Kidney Disease
Cilia and Cell Cycle Disruption
Treatment of Hypertension
Vasopressin V2-Receptor Antagonists
Surgical Management of Autosomal Dominant Polycystic Kidney Disease Patients
Nephrolithiasis in Autosomal Dominant Polycystic Kidney Disease Patients
Renal Cell Carcinoma in Autosomal Dominant Polycystic Kidney Disease Patients
Prognosis of Autosomal Dominant Polycystic Kidney Disease
Prognosis Of Autosomal Dominant Polycystic Kidney Disease
Renal Replacement in Autosomal Dominant Polycystic Kidney Disease Patients
Renal Transplantation in Autosomal Dominant Polycystic Kidney Disease Patients
In addition to routine pretransplant evaluation, do adpkd patients require specific evaluation?
Should potential renal complications require nephrectomy prior to transplantation?
When Transplantation From A Living Relative Is Considered, The Existence Of Adpkd In The Donor Should Be Formally Ruled Ou ...
Is There Any Immunosuppression Protocol That May Have A Favorable Course In Adpkd Patients?
Is there any increased incidence of post-transplant complication in adpkd patients?
As compared to nonadpkd patients, is there an increase in the rate of graft and patient survival in adpkd?
Other Books in This Series
Polycystic Kidney Disease - ECAB
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