Developmental abnormalities and inflammations of the kidney and urinary tract

1
Developmental abnormalities and inflammations of
the kidney and urinary tract
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Cystic diseases

• Cystic renal dysplasia
• Polycystic kidney
• Adult (AD)
• Infantile (AR)
• Medullary cystic
• Dialysis associated cystic
• Simplex cysts
• Renalis cystic disease associated with inherited
  diseases
• Glomerulocystic diseases
• Extraparenchymal renal cysts

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Polycystic kidney (ADPKD)

• Frequency 1 400-1000
• CRF 5-10
• AD
• PKD1 16p13.3 (85) RF 40 y. 5, 50 y 35, 60
  y. 70, 70y 95
• polycystin-1
• PKD2 4q21 (15) RF 0, 5, 15, 45
• polycystin-2

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Scenario of the development of the ADPKD

• Mutation
• Cell-cell, cell-matrix relation abnormality
• Altered epithelial proliferation, and.
  differentiation
• Abnorm. ECM Epith. proliferation Fluid secretion
• Vascular injury CYST Interstitial
  inflammation fibrosis

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Polycystic kidney clincal signs

• Pain
• Haematuria
• Progressive renal insufficiency
• Proteinuria (not more than 2 g / day)
• Polyuria
• Hypertension

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Polycystic kidney other accompaniing congenital
anomalies

• Hepatic cysts
• Berry anerysms
• Mitralis prolapse other valvular anomalities
• Th. Dialysis, renal transplatation
• oka
• 40 coronary / hypertensive heart disease
• 25 infection
• 15 aneurysm rupture, hypertensive
  intracerebral haemorrhage
• 20 other causes

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Renal dysplasia(Cystic renal dysplasia)

• Nomenclature !!!!
• Sporadic
• Unilateral, ( might be bi)
• Metanephric differentiation abnormality
• Combined with lower urinary tract anomalies
• ( Vesicoureteral junction obstruction, ureter
  agenesia)
• Clin. Palpable abdominal mass

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Clinical manifestation of renal disorders

• Acute nephritis macroscopic haematuria, mild
  proteinuria, mild hypertension
• Nephrosis severe proteinuria gt 3.5g/day
• hypoalbuminaemia, oedema, hyperlipidemia,
  lipiduria
• Acute renal insufficiency abrupt oligo/anuria
• Chronic renal insuff/failure

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Clinical manifestation of renal disorders

• Renal tubular defects
• polyuria, electrolit disorders (Metabolic
  acidosis)
• Urine infections pain, fever, etc
• Nephrolithiasis colica, haematuria
• Obstruction pain, infection, stone formation

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Clinical manifestations of renal disorders

• Chronic renal insuff
• Decreased renal reserve GFR lt50
• Se creatinin, BUN norm. Free of symptoms
• Renal insufficiency GFR 20-50 Se creatinin,
• BUN anaemia, hypertension, sign polyuria,
• Renal failure GFR lt20-25, oedema,
  hypocalcaemia, acidosis, neurological, GI,
  cardiovascular sequales
• End-stage GFR lt 5

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General signs of renal insufficiency

• Fluid /electrolite
• Dehydration
• Oedema
• Hyperkalaemia
• Metabolic acidosis

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General signs of renal insufficiency

• Calcium, phosphate
• Hyperphosphataemy
• Hypocalcaemy
• Secunder hypoparathyreosis
• Renal osteodystrophy
• Haematological
• Anaemia
• Diathesis

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General signs of renal insufficiency

• Cardiovascular
• Hypertenison
• Congestive cardiac insuff
• Pulmonary oedema
• Serous membranes
• Uremic pericarditis,
• pleuritis,
• peritonitis

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General signs of renal insufficiency

• GI
• Vomit
• Hemorrhage
• Oesophagitis, gastritis, colitis
• Neuromuscular
• Myopathy, Neuropathy, Encephalopathy
• Dermatological
• Yellow skin, itching, dermatitis

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Causes of Tubulointerstitial Nephritis

• Infection
• Acut bacterial pyelonephritis
• Chronic pyelonephritis (reflux nephropathy)
• Other infections ( viral, parasitic)
• Toxins
• Drugs
• Acute hypersensitive nephritis
• Analgesic nephropathy
• Heavy metals
• Lead, Cadmium
• Metabolic diseases
• Urate nephropathy
• Hypercalcaemic nephropathy
• Hypokalaemic nephropathy
• Oxalate nephropathy

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Tubulointerstitial nephritis

• Physical factors
• Chronic obstruction
• Radiation nephropathy
• Neoplasms
• Myeloma multiplex
• Immunological diseases
• Transplant rejection
• Sjögren sy
• Sarcoidosis
• Vascular disorders
• Other
• Balkan nephropathy
• Nephronophthisis medullary cystic disease
• Idiopathic interstitial nephritis

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Pyelonephritis

• Infection
• Ascending
• E. Coli
• Klebsiella
• Proteus
• Enterobacter
• Streptococcus faecalis
• Haematogenous
• Staphylococcus
• E.Coli

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Renal stones

• Calcium oxalat, phosphat 70
• MG, ammonium phosphat 15-20
• struvit
• Urate 5-10
• Cystin 1-2