Renal Disease Prt.2 Flashcards
•Each nephron processes ~_____ of filtrate daily with____% reabsorption
180L
99%
•Tubular system performs (3)
reabsorption, secretion, and concentration
•The____ is the functional unit of the kidney with distinct segments
nephron
Key Tubular Segments and Functions
Proximal tubule
LOH
Distal tubule
Collecting duct
Proximal Tubule
1. Reabsorbs 65-80% of filtered (3)
2. Handles virtually all (2) reabsorption
3. Reabsorbs 80-90% of (1)
sodium, chloride, water
glucose and amino acid
bicarbonate
- Establishes concentration gradient
- Impermeable to water in ascending limb
Loop of Henle
•Fine-tunes electrolyte balance
• Regulated by hormones (PTH, aldosterone)
Distal Tubule
• Water reabsorption (ADH-controlled)
• Final acid-base regulation
• Potassium secretion
Collecting Duct
Pathophysiological Patterns
•_______: Failure to reabsorb filtered substances
•_______: Failure to secrete substances
•_______: Disrupted water handling
Wasting Syndromes
Retention Syndromes
Concentration/Dilution Defects
Clinical Manifestations
• Polyuria/polydipsia
•Electrolyte abnormalities
•Acid-base disturbances
•Growth issues in children
• Nephrolithiasis/nephrocalcinosis
Tubular Disorders
Tubular disorders
Diagnostic Approach (4)
• Urinalysis (pH, specific gravity, glucose, protein)
•Serum electrolytes and acid-base status
•Specialized functional testing
• Imaging and genetic studies when indicated
Proximal Tubular Disorders (5)
Fanconi Syndrome
Renal Glucosuria
Cystinuria
RTA T2
Hartnup Disease
Loop of Henle Disorders (2)
Bartter Syndrome
Medullary Sponge Kidney
Distal Tubular Disorders (2)
RTA T1
Gitelman Syndrome
Collecting Duct Disorders (2)
Nephrogenic DI
RTA T4
Proximal Tubular Disorders
•Pathophysiology: Generalized proximal tubular dysfunction
Fanconi Syndrome
Proximal Tubular Disorders
•Clinical Features: Polyuria, polydipsia, dehydration, growth retardation
• Laboratory Findings:
• Glucosuria with normal blood glucose
• Aminoaciduria
• Phosphaturia and hypophosphatemia
• Bicarbonaturia and metabolic acidosis
• Low-molecular-weight proteinuria
Fanconi Syndrome
Proximal Tubular Disorders
•Pathophysiology: Isolated defect in glucose reabsorption
Renal Glucosuria
Proximal Tubular Disorders
•Clinical Features: Often asymptomatic
• Laboratory Findings:
• Glucosuria with normal blood glucose
•No other tubular abnormalities
Renal Glucosuria
Proximal Tubular Disorders
•Pathophysiology: Defective transport of dibasic amino acids
Cystinuria
Proximal Tubular Disorders
•Clinical Features: Recurrent kidney stones
Cystinuria
Proximal Tubular Disorders
Cystinuria
• Laboratory Findings:
Increased urinary excretion of (4)
•Characteristic____ crystals in urine
•Positive______test
cystine, ernithine, lysine, and arginine
hexagonal
cyanide-nitroprusside test
Proximal Tubular Disorders
•Pathophysiology: Defective bicarbonate reabsorption
Renal Tubular Acidosis Type 2 (Proximal RTA)
Proximal Tubular Disorders
•Clinical Features: Growth failure, rickets, osteomalacia
• Laboratory Findings:
•Hyperchloremic metabolic acidosis
• Urine pH < 5.5 during acidosis
•Bicarbonaturia
• Hypokalemia
Renal Tubular Acidosis Type 2 (Proximal RTA)
Proximal Tubular Disorders
•Pathophysiology: Defective neutral amino acid transport
Hartnup Disease
Proximal Tubular Disorders
•Clinical Features: Pellagra-like rash, cerebellar ataxia
• Laboratory Findings:
• Increased urinary excretion of neutral amino acids
•Normal serum amino acid levels
Hartnup Disease
Hartnup Disease
•Clinical Features:
Pellagra-like rash
cerebellar ataxia
RTA T2
•Clinical Features:
Growth failure, rickets, osteomalacia
Cystinuria
•Clinical Features:
Recurrent kidney stones
Fanconi syndrome
•Clinical Features:
Polyuria, polydipsia, dehydration, growth retardation
Loop of Henle Disorders
• Pathophysiology: Defect in sodium-potassium-chloride cotransporter
Bartter Syndrome
Loop of Henle Disorders
•Clinical Features: Growth retardation, muscle weakness, polyuria
• Laboratory Findings:
• Hypokalemic metabolic alkalosis
•Hypochloremia
•Normal blood pressure
•Elevated renin and aldosterone
• Hypercalciuria
Bartter Syndrome
Loop of Henle Disorders
• Pathophysiology: Cystic dilation of collecting ducts in renal pyramids
Medullary Sponge Kidney
Loop of Henle Disorders
•Clinical Features: Nephrolithiasis, hematuria, UTIs
• Laboratory Findings:
•Nephrocalcinosis
• Hypercalciuria
•Microscopic hematuria
•Sterile pyuria
Medullary Sponge Kidney
Bartter syndrome
•Clinical Features:
Growth retardation, muscle weakness, polyuria
Medullary sponge kidney
•Clinical Features:
Nephrolithiasis, hematuria, UTIs
Distal Tubular Disorders
•Clinical Features: Growth failure, rickets, nephrocalcinosis
• Laboratory Findings:
•Hyperchloremic metabolic acidosis
• Urine pH > 5.5 despite acidosis
•Hypercalciuria
•Hypokalemia
•Nephrocalcinosis
Renal Tubular Acidosis Type 1 (Distal RTA)
Distal Tubular Disorders
•Pathophysiology: Inability to secrete hydrogen ions
Renal Tubular Acidosis Type 1 (Distal RTA)
Distal Tubular Disorders
•Clinical Features: Muscle weakness, fatigue, tetany
• Laboratory Findings:
• Hypokalemic metabolic alkalosis
•Hypomagnesemia
•Hypocalciuria
•Normal blood pressure
Gitelman Syndrome
Distal Tubular Disorders
•Pathophysiology: Defect in thiazide-sensitive sodium-chloride cotransporter
Gitelman Syndrome
Collecting Duct Disorders
•Clinical Features: Polyuria, polydipsia, dehydration
• Laboratory Findings:
•Dilute urine (specific gravity < 1.005)
• Hypernatremia
•No response to desmopressin
•Normal or elevated ADH levels
Nephrogenic Diabetes Insipidus
Collecting Duct Disorders
•Pathophysiology: Resistance to antidiuretic hormone (ADH)
Nephrogenic Diabetes Insipidus
Collecting Duct Disorders
•Clinical Features: Muscle weakness, cardiac arrhythmias
• Laboratory Findings:
•Hyperkalemia
•Mild hyperchloremic metabolic acidosis
•Urine pH < 5.5
•Low or normal aldosterone levels
•Associated with diabetic nephropathy or interstitial nephritis
Renal Tubular Acidosis Type 4 (Hyperkalemic RTA)
Collecting Duct Disorders
• Pathophysiology: Aldosterone deficiency or resistance
Renal Tubular Acidosis Type 4 (Hyperkalemic RTA)