The Kidney

dr. I Made Naris Pujawan, [Link], [Link]

Clinical Manifestations of Renal Diseases
• Nephritic syndrome
• Rapidly progressive glomerulonephritis
• The nephrotic syndrome
• Asymptomatic hematuria or proteinuria
• Acute renal failure
• Chronic renal failure
• Renal tubular defects
• Urinary tract infection
• Nephrolithiasis (renal stones)
• Nephrolithiasis (renal stones)
Glomerular Diseases
• Glomerular diseases constitute some of the major problems in nephrology
• Glomeruli may be injured by a variety of factors and in the course of
several systemic diseases
• Systemic immunological diseases such as systemic lupus erythematosus
(SLE), vascular disorders such as hypertension, metabolic diseases such as
diabetes mellitus, and some hereditary conditions such as Fabry disease
often affect the glomerulus.
• These are termed secondary glomerular diseases to differentiate them
from disorders in which the kidney is the only or predominant organ
involved
• The latter constitute the various types of primary glomerulonephritis or,
because some do not have a cellular inflammatory component,
glomerulopathy
• However, both the clinical manifestations and glomerular histologic
changes in primary and secondary forms can be similar
• Although much is not known about etiologic agents and triggering
events, it is clear that immune mechanisms underlie most forms of
primary glomerulopathy and many of the secondary glomerular
disorders
• Glomerulonephritis can be readily induced experimentally by antigen-
antibody reactions
• Furthermore, glomerular deposits of immunoglobulins, often with
components of complement, are found in the majority of individuals
with glomerulonephritis
• Cell-mediated immune reactions also may play a role, usually in
concert with antibody-mediated events
Tubular and Interstitial Diseases
• Most forms of tubular injury involve the interstitium as well;
therefore, diseases affecting these two components are discussed
together
• Under this heading we consider two major groups of processes:
• (1) ischemic or toxic tubular injury, leading to acute kidney injury
(AKI) and acute renal failure, and
• (2) inflammatory reactions of the tubules and interstitium
(tubulointerstitial nephritis)
Vascular Diseases
• Benign nephrosclerosis is the term used for the renal pathology associated
with sclerosis of renal arterioles and small arteries
• Two processes participate in the arterial lesions:
• Medial and intimal thickening, as a response to hemodynamic changes, aging,
genetic defects, or some combination of these
• Hyaline deposition in arterioles, caused partly by extravasation of plasma proteins
through injured endothelium and partly by increased deposition of basement
membrane matrix
• On histologic examination there is narrowing of the lumens of arterioles
and small arteries, caused by thickening and hyalinization of the walls
(hyaline arteriolosclerosis)
• Malignant nephrosclerosis is the form of renal disease associated with the
malignant or accelerated phase of hypertension
Congenital Anomalies
• Congenital Anomalies
• Agenesis of the Kidney
• Hypoplasia
• Ectopic Kidneys
• Horseshoe Kidneys

MULTICYSTIC RENAL DYSPLASIA

Cystic Diseases of the Kidney
Urinary Tract Obstruction (Obstructive Uropathy)
• Obstruction increases susceptibility to infection and to stone formation, and
unrelieved obstruction almost always leads to permanent renal atrophy, termed
hydronephrosis or obstructive uropathy
• Obstruction may be sudden or insidious, partial or complete, unilateral or
bilateral
• it may occur at any level of the urinary tract from the urethra to the renal pelvis
• It can be caused by lesions that are intrinsic to the urinary tract or extrinsic
lesions that compress the ureter
• Hydronephrosis is the term used to describe dilation of the renal pelvis and
calyces associated with progressive atrophy of the kidney due to obstruction to
the outflow of urine
• Obstruction also triggers an interstitial inflammatory reaction, leading eventually
to interstitial fibrosis, by mechanisms similar to those discussed earlier
Morphology
• The kidney may be slightly to massively enlarged,
depending on the degree and the duration of the
obstruction
• In chronic cases the picture is one of cortical tubular
atrophy with marked diffuse interstitial fibrosis
• Progressive blunting of the apices of the pyramids
occurs, and these eventually become cupped.
• In far-advanced cases the kidney may become
transformed into a thin-walled cystic structure
having a diameter of up to 15 to 20 cm
Urolithiasis (Renal Calculi, Stones)
• Stones may form at any level in the urinary tract, but most arise in the kidney
• There are four main types of calculi
• calcium stones (about 70%),
• triple stones or struvite stones, composed of magnesium ammonium
phosphate
• uric acid stones
• cystine
• Increased urinary concentration of the stones' constituents, such that it exceeds
their solubility (supersaturation
• A low urine volume in some metabolically normal patients may also favor
supersaturation
Morphology
• Stones are unilateral in about 80% of patients
• The favored sites for their formation are within the
renal calyces and pelves and in the bladder
• If formed in the renal pelvis they tend to remain
small, having an average diameter of 2 to 3 mm
• These may have smooth contours or may take the
form of an irregular, jagged mass of spicules
• Often many stones are found within one kidney
• On occasion, progressive accretion of salts leads to
the development of branching structures known as
staghorn calculi, which create a cast of the pelvic
and calyceal system.
Tumors of the Kidney
• BENIGN TUMORS
• Renal Papillary Adenoma
• Angiomyolipoma
• Oncocytoma
• MALIGNANT TUMORS
• Renal Cell Carcinoma (Adenocarcinoma of the Kidney)
• Urothelial Carcinomas of the Renal Pelvis
• Wilms tumor
Renal Cell Carcinoma
• Account for 85% of renal cancers in adults.
• There are approximately 30,000 new cases per year and 12,000
deaths from the disease
• The tumors occur most often in older individuals, usually in the sixth
and seventh decades of life, and show a 2 : 1 male preponderance
• Because of their gross yellow color and the resemblance of the
tumor cells to clear cells of the adrenal cortex, they were at one time
called hypernephroma
• It is now clear that all these tumors arise from tubular epithelium and
are therefore renal adenocarcinomas
• Tobacco is the most significant risk factor
• Most renal cancer is sporadic, but unusual forms of autosomal
dominant familial cancers occur, usually in younger individuals
• Classification of renal cell carcinoma: histology, cytogenetics, and
genetics
• The major types of tumor are as follows
• Clear cell carcinoma
• Papillary carcinoma
• Chromophobe renal carcinoma
• Collecting duct (Bellini duct) carcinoma
• The three classic diagnostic features of renal cell carcinoma
are costovertebral pain, palpable mass, and hematuria, but
these are seen in only 10% of cases
• The most reliable of the three is hematuria
• Renal cell carcinomas produce a number of paraneoplastic
syndromes
• this tumor is its tendency to metastasize widely before giving
rise to any local symptoms or signs