617
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.
Congenital and Hereditary
Disorders of the Kidney
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.
T
Disorders of
Renal Function
25
C h a p t e r
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
