Porth's Essentials of Pathophysiology, 4e

he kidneys are subject to many of the same disease processes that affect other organs, including genetic and developmental defects, infections, immunologic disorders, and neoplasms. Although many disorders of renal function originate in the kidneys, others develop secondary to diseases such as systemic lupus erythema- tosus and diabetes mellitus. Some of these diseases are progressive, eventually leading to chronic kidney disease and the need for dialysis or transplantation. As a major cause of work loss, physicians’ visits, and hospitaliza- tions, kidney diseases remain among the most costly ill- nesses throughout the industrial world. The content in this chapter focuses on congenital and hereditary disorders of the kidney, disorders of glomer- ular function, tubulointerstitial disorders, obstructive disorders, and malignant neoplasms. Acute renal injury and failure and chronic kidney disease are discussed in Chapter 26, and disorders that predominantly affect the lower urinary tract and bladder are discussed in Chapter 27. The kidneys begin to develop early in the fifth week of gestation and start to function approximately four weeks later. 1 Urine formation begins at about the ninth week of gestation, with the rate of urine production increasing throughout gestation to reach a volume of about 50 mL/hour at term. 2 The urine that is produced is excreted into the amniotic cavity and is the main con- stituent of amniotic fluid. In pregnancies that involve infants with nonfunctional kidneys or obstruction of urine outflow from the kidneys, the amount of amniotic fluid is small—a condition called oligohydramnios . 2,3 Disorders of Kidney Development About 10% of all people are born with potentially sig- nificant malformations of the urinary system. 4 These disorders sometimes result from hereditary influences, but most often are the result of an acquired defect that arises during embryonic development. Disorders of Renal Function 25 C h a p t e r Congenital and Hereditary Disorders of the Kidney T

Congenital and Hereditary Disorders of the Kidney Disorders of Kidney Development Agenesis, Hypoplasia, and Dysplasia Alterations in Kidney Position and Form Cystic Diseases of the Kidney Autosomal Dominant Polycystic Kidney Disease Autosomal Recessive Polycystic Kidney Disease Nephronophthisis and Medullary Cystic Kidney Disease Simple and Acquired Renal Cysts Disorders of Glomerular Function Etiology and Pathogenesis of Glomerular Injury Types of Glomerular Disease Acute Nephritic Syndrome Rapidly Progressive Glomerulonephritis Nephrotic Syndrome IgA Nephropathy Hereditary Nephritis (Alport Syndrome) Chronic Glomerulonephritis Glomerular Lesions Associated with Systemic Disease Tubular and Interstitial Disorders Tubulointerstitial Nephritis Pyelonephritis Acute Pyelonephritis Chronic Pyelonephritis and Reflux Nephropathy Drug-Related Nephropathies Obstructive Disorders Mechanisms of Renal Damage

Hydronephrosis Clinical Features

Kidney Stones

Types of Stones Clinical Features Malignant Tumors of the Kidney Wilms Tumor Renal Cell Carcinoma

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