Kidney and its Collecting System

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• Kidney and its Collecting System
• Lecture By
• Dr. Amitabha Basu. MD

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KIDNEYWHAT WE WILL STUDY IN THIS LECTURE

• Few ANOMALIES
• NON-NEOPLASTIC
• NEOPLASTIC

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ANOMALIES of kidney

1. Horseshoe" kidney
2. Agenesis
3. Ectopic kidney

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• Here is a "horseshoe" kidney. This is a
  congenital anomaly that most often occurs in
  association with other anomalies or syndromes .

• Complications
• Stone formation,
• Infection,

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Agenesis

• Bilateral-
• Presentation
• Oligohydramnios
• Potter facies Flat nose, low set ear and
  recessed chin.
• Pulmonary hypoplasia
• Unilateral
• Compensatory hyperplasia ( particularly of
  glomeruli) of other kidney.

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Note

• Any kidney disease if present in a baby in the
  uterus that fetus will have pulmonary
  hypoplasia.
• Severity of the disease depends on the severity
  of the kidney lesion.

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Ectopic kidney

• Most common pelvis
• Small but normal function
• Present as a mass in pelvis.

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Sequence by which we will proceed

1. TERMS and definitions (Important)
2. Cystic diseases
3. Interstitial diseases
4. Hypertensive disease
5. Kidney stone
6. Urate nephropathy.
7. Childhood Hemolytic Disease

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TerminologyYou must Know

1. Azotemia and Uremia
2. Acute Renal Failure
3. Chronic Renal failure

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AZOTEMIAIts a Biochemical Change due to a
diseased Kidney

• ELEVATION OF BLOOD UREA NITROGEN (BUN)
• Elevated creatinine
• Decreased GFR( GLOMERULAR FILTRATION RATE)

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Uremia

• A patient with Azotemia with profound clinical
  signs and symptoms. (failure of renal excretory
  function along with , metabolic and endocrine
  abnormality)
• Clinical Asterixis ( flapping tremor)

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Fluid and Electrolytes Hyperkalemia Metabolic acidosis
Calcium Phosphate and Bone Hypocalcemia, Secondary hyperparathyroidism
Hematologic Anemia, Bleeding diathesis
Cardiopulmonary Uremic pericarditis
Gastrointestinal Nausea and vomiting, gastritis, colitis
Neuromuscular Peripheral neuropathy, Encephalopathy
Dermatologic Sallow color, Pruritus
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Terms
Oliguria Urine output lt 500/400 ml/24 hours Cause nephritic syndromes, Acute renal failure.
Anuria Urine output NIL Cause Stone, ARF (some times), tumors.
Polyuria Urine output gt 3000ml/24 hours ( at least 2.5 ml) Cause Diabetes insipidus, DM
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Acute Renal Failure

1. Acute Loss of Renal Function
2. Oliguria less urine (lt 400ml/day)
3. Or, Anuria No urine
4. Recent onset azotemia

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Cause of acute renal failure (ARF)

1. Adult
2. Acute Tubule necrosis
3. Crescentic Glomerulonephritis
4. Papillary necrosis
5. Children
6. Childhood Hemolytic-Uremic Syndrome

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Chronic Renal failure

• Prolonged signs and symptoms of Uremia.
  Increasing BUN, creatinine.
• END RESULT OF ALL CHRONIC RENAL DISEASES.
• Leads to uremia.

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End-stage renal disease (ESRD)

• Definition.
• End-stage of various kidney disease.
• When GFR lt 5 of normal
• Survival only by dialysis or transplant

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Cystic Diseases of Kidney

• Adult polycystic Kidney Diseases
• Childhood polycystic Kidney Diseases
• Hemodialysis associate cyst

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Autosomal-dominant (adult) polycystic kidney
disease (ADPKD)

• Genetic
• Mutation of PKD1 ( ch 16) and PKD 2 gene and
  loss of Polycystin 1 and Polycystin 2 (stromal
  material) respectively.
• Result abnormal stroma and cell-to-cell adhesion
  ? tubular dilatation (cyst formation) and
  vascular dilatation.

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MORPHOLOGY GROSS

1. Bilateral or Unilateral May weight up to 4 kg.
2. Composed solely of cysts of varying sizes.( 3-4cm
   in Diameter) no intervening parenchyma.
3. Cyst may contain serous / hemorrhagic fluid

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ADULT POLYCYSTIC KIDNEY(APKD)CLINICAL FEATURES

1. PRODUCE SYMPTOMS usually ON AND AFTER 4th DECADE.
2. Flank pain, heavy dragging sensation
3. Intermittent gross Hematuria.
4. Hypertension /Urinary infections (75)
5. Saccular aneurism in Circle of Willis.
6. 1/3rd cases Asymptomatic Liver Cyst found.
7. Colonic Diverticulum.

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ADULT POLYCYSTIC KIDNEY(APKD)Prognosis

1. Progresses slowly (many years with azotemia )
2. Endstage Renal failure occur at about age 50.
3. Uremia or Hypertensive complications ( read
   hypertensive complications).

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• Childhood Polycystic Kidney

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Childhood Polycystic Kidney

• Autosomal Recessive
• Progress to renal failure
• Gross Bilateral and sponge like appearance

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Micro Cysts are elongated and radially. Present
in cortex and medullaThese cysts are dilated
distal tubules and collecting ducts.
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Clinical features

1. Clinical Features are present at birth
2. Young adult die due to renal failure
3. Hepatic fibrosis lead to early cirrhosis
4. If the disease is present in a fetus Pulmonary
   hypoplasia may be presents.

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ACQUIRED (DIALYSIS-ASSOCIATED) CYSTIC DISEASE
Due to repeated dialysis in chronic renal
failure. Cysts at the corticomedullary junction
and in the medulla.
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• ACQUIRED (DIALYSIS-ASSOCIATED) CYSTIC DISEASE
• Complications
• May deposit Amyloid (Aß2 micro- globulin) in
  the kidney.
• And high chance Renal cell carcinoma.

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My kidney stone What a beauty!

• Can occur at any level in urinary collecting
  system.
• Symptomatic Urolithiasis is more common in Males

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Renal stone urolithiasis

• 1. Calcium oxalate (or phosphate) 75
• 2. Magnesium ammonium phosphate (struvite, or
  "triple phosphate")
• 10-15
• 3. Uric acid 6.
• 4. Cystene stone.

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Calcium stone
Increase in the Urine Concentration (super
saturation) of stones constituents( calcium)
Calcium Oxalate and Phosphate Idiopathic Hypercalciuria (normal serum calcium) Hypercalciuria and hypercalcemia ( primary hyperparathyroidism / others) Ethylene glycol toxicity with acidosis No known metabolic abnormality (15-20)
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Hypercalcemia

• Non neoplastic cause
• Sarcoidosis ( due to ? 1,25-dihydroxyvitamin D )
• Pagets disease
• Primary hyperparathyroidism ( due to ? PTH)
• Neoplastic cause ( due to ? PTH like peptides)
• Renal cell carcinoma
• Squamous cell carcinoma
• Breast carcinoma
• Metastatic tumor in bone (High alkaline
  phosphatase is the marker).
• Multiple Myeloma.

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Calcium oxalate Crystal in Urine and kidney
Positive crystal in polarized light
Envelop
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Struvite stones

• Cause Infection" by Proteus Vulgaris that
  split urea to ammonia, favor their formation.
• Present in alkaline urine
• Urine coffin lid crystal found in urine.
• Morphology stag horn calculi.

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A large "staghorn" calculus

• Create a cast of renal pelvis and calyceal
  system.
• Associated with pyelonephritis and atrophy of
  cortex of kidney.

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Uric acid stones

• Cause
• Hyperuricemia (gout)
• Leukemia undergoing chemotherapy ( massive cell
  damage)
• Urine Needle like crystal in acid urine.
• X ray cannot be seen

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Clinical

1. Their passage is marked by intense abdominal or
   back or flank pain known as renal or ureteral
   "colic.
2. Hematuria
3. Bacterial Infection (pyelonephritis)
4. Hydronephrosis, hydroureter.

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• Hydronephrosis and its cause

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What is Hydronephrosis ?

• Def Dilation of renal Pelvis and calyces,
• associated with atrophy of the parenchyma.

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Pathogenesis

• 1. Initially the renal Pelvis and calyces are
  dilated
• 2. Result in the atrophy of the Cortex and
  infection (pyelonephritis).

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MORPHOLOGY

• Bilateral Hydronephrosis
• Obstruction in the urethra (urethral
  valve-child),
• Pregnancy,
• Benign Hyperplasia of Prostate.

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Unilateral Hydronephrosis

• Obstruction from
• Within Lumen of ureter Stone, necrotic Papilla.
• From the wall of the Lumen transitional cell
  carcinoma
• Outside the ureter Enlarged lymph node, Tumor

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Unilateral Hydronephrosis note blunted calyces
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Clinical Features

• Unilateral- unfortunately remain Silent, so it
• may cause major renal damage.
• Recurrent urinary tract infection.

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• DISEASE AFFECTING TUBULES AND INTERSTITIUM
• Pyelonephritis
• Acute
• Chronic
• Acute tubular Necrosis

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Pyelonephritis

• TYPES
• ACUTE AND CHRONIC

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ACUTE PYELONEPHRITISETIOLOGY PATHOGENESIS

• Gram Negative Rods ( from urine)
• E. coli, Proteus, Klebsiella, Enterobacter and
  Pseudomonas.
• Others
• Staphylococcus Aureus (from blood).

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Route of spread of infection

• Ascending Infections reach the kidney either by
  ascending up the urinary tract common in female.
• Hematgenous spread with sepsis or infective
  Endocarditis, abscess
• (Staphylococcus Aureus).

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Ascending Infection Pathogenesis

• Colonization of bacteria take place in the distal
  urethra (introitus of female).
• Then they are carried up to wards the bladder
  with urine,
• Infected urine enter ureter by vesicoureteral
  Reflux.
• And reach kidney

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Less angle of ureter
Normal angle of ureter
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Predisposing Factors for Pyelonephritis

• Obstruction, short urethra.
• Patient Age
• adult female gt male
• Diabetes mellitus hyperglycemia
• BPH, Uterine prolapse.
• Hospital procedure Urethral catheterization,
  cystoscopy, urethral trauma.

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Morphology of pyelonephritis

1. Abscess (Neutrophils in the interstitium) patchy
2. Papillary necrosis is common in Diabetes
   (involve apical 2/3 rd of renal papilla)

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Papillary necrosis is common in Diabetes(
involve apical 2/3 rd of papilla)
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Clinical courses Acute Pyelonephritis

• lt 1 year presence of urethral valve
• Upto 40 years FgtM
• After 40 years MF
• Sudden temperature,chill,malise, pain in
  costovertebral angle,
• Dysuria, increased frequency,ungency

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Urinary Findings Acute Pyelonephritis

• Leucocytes (mainly neutrophil)
• Bacteriuria ( urine culture ve)
• WBC cell cast.

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• Chronic Pyelonephritis

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Etiology

• Repeated lower urinary tract infections for
  years.
• Obstruction
• Chronic reflux

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PathogenesisTwo Forms

• 1. Chronic obstructives Pyelonephritis
• Obstruction predispose to infection.
• 2. Chronic Reflux associated Pyelonephritis.
• Common form of chronic Pyelonephritic Scarring.

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Key word Broad Scars, irregular shape of both
kidney.
Chronic reflux associated Pyelonephritis
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Microscopy

• Colloid Cast in tubules
• (Thyroidization)
• The large collection of chronic inflammatory
  cells
• Intersitial fibrosis

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Clinical Finding chronic Pyelonephritis

• Produce polyuria and Nocturia
• (usually in a bilateral involvement).
• Important cause of Chronic renal Failure.

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Other interstitial disease

• Drug-induced interstitial nephritis
• Analgesic Nephropathy
• Urate Nephropathy

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Drug-induced interstitial nephritis

• Etiology Synthetic penicillin, rifampicin,
  thiazides, phenylbutazone.
• Clinical
• Onset 15 days (avg.) after exposure of the drug
• Fever, rash, eosinophilia and renal abnormality.
• Withdrawal of the drug is followed by recovery.

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Kidney change

• Micro eosinophilc infiltrate
• Urine eosinophil
• Mild protenuria

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Analgesic Nephropathy

• Etiology only after consuming large amount of
  medicine
• Like Phenacetin, acetaminophen, aspirin,
  Caffeine, Codeine.
• Morphology papillary necrosis and intestinal
  nephritis

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Analgesic Nephropathy

• Clinical Features
• CRF, Hypertension, anemia, massive protenuria.
• Complications
• Transitional Cell Carcinoma who survives renal
  failure.

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Chronic urate nephropathy

• Cause
• Cellular necrosis of tumor cells with
  chemotherapy
• Gout ( painful great toe increased uric acid) .

Morphology Tophi in interstitium with a foreign
body reaction. ( chalky white material on trans
section)
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• Acute Tubular Necrosis
• (ATN)
• Definition
• Types (morphology)
• Pathophysiology of renal failure
• Urine findings
• Clinical

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ACUTE TUBULAR NECROSIS

1. Definition Necrosis of tubular epithelium
2. Most common cause of Acute renal Failure in
   adult.
3. Reversible renal lesion( if taken care of
   immediately).

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Two patterns (ETIOLOGY)

• 1. Ischemic ATN due to Shock, mismatched blood
  transfusion, myoglobinuria ( damage of skeletal
  muscle).
• 2. Nephrotoxic ATN due to poisons, mercury,
  gentamicin, radiographic contrast agent etc.
  

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Distribution of ATN (very important) recall the
causes
PATCHY
DIFFUSE
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Necrotic epithelial cell in ATN note there is no
inflammatory cells
Features of reversible cell injury due to
hypoxia. Cell swelling, clumping
chromatin Biochemical feature of cell High
Na High water Increased lactic acid
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Urinalysis

• Protein (hyaline) cast or tubular epithelial
  (cellular) cast seen in the tubules

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ATN Natural history of disease.
Featured by Anuria /oliguria and rapid rise in blood urea nitrogen. Featured by Anuria /oliguria and rapid rise in blood urea nitrogen.
Initiation phase slight decline in urine output with a rise in BUN
Maintenance phase Oliguria rising BUN, hyperkalemia, metabolic acidosis, uremia
Recovery phase Due to Regeneration of tubular epithelium occur increase in urine volume (gt 3 L/day). Hypokalemia, infection.
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• DISEASE INVOLVING THE BLOOD VESSELS

Benign Nephrosclerosis Malignant nephrosclerosis
Thrombotic Microangiopathies
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Benign Nephrosclerosis

• Associated with long-standing hypertension,
• Patients are usually old.
• This infrequently cause Uremia and death
• A mild proteinuria is present.
• GFR mildly diminished.

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Benign nephrosclerosis. (Morphology-
Gross)Symmetrically atrophic kidney (weighing
110 to 130 g)Characteristic finely granular
appearance as seen here.
Granular Surface
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Identify the microscopic picture below
Hyaline arteriolosclerosis
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• Malignant Nephrosclerosis
• Its a Medical Emergency

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Malignant nephrosclerosis Diastolic BPgt120mmHg
flea-bitten appearances on gross
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Malignant Hypertension more terms about the
blood vessels change

• Necrotizing Arteriolitis
• Hyperplasic arteriosclerosis
• Fibrinoid necrosis

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Malignant nephrosclerosis Clinical features

• Papilledema, encephalopathy, cardiovascular
  abnormality, and
• Renal failure
• Nausea, vomiting
• Marked proteinuria
• Hematuria.
• Visual impairment

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Thrombotic Microangiopathies

• Childhood Hemolytic Disease
• Thrombotic Thrombocytopenic Purpura (adult)
• Clinical Presentation
• 1. Hemolytic anemia ( jaundice, increased
  reticulocytes, schistocytes)
• 2.Thrombocytopenia
• 3. Acute renal failure

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Childhood Hemolytic Disease

• Etiology E. coli infection - Shiga-like toxin.
• Pathogenesis
• Shiga toxin ? injury to endothelium ? increased
  endothelin and tissue factor and loss of
  endothelial Nitric Oxide ? vasoconstriction and
  fibrin thrombosis in glomeruli ? acute renal
  failure.

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Fibrin thrombi in microcirculation/ glomeruli
Increased fibrin degradation product, prolonged
aPTT, PT , clotting time and bleeding time.
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Clinical

• A bloody diarrhea is followed in a few days by
  renal failure.
• Jaundice
• Azotemia/uremia
• Prognosis rapidly fatal, but recover in a few
  weeks with supportive dialysis.

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Do not eat infected Hamburger !!!
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• THANK YOU