1、Developmental abnormalities and inflammations of the kidney and urinary tract,Cystic diseases,Cystic renal dysplasia Polycystic kidney- Adult (AD)- Infantile (AR) Medullary cystic Dialysis associated cystic Simplex cysts Renal cystic disease associated with inherited diseases Glomerulocystic disease
2、s Extraparenchymal renal cysts,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, 45polycystin-2,Scenario of the development of the ADPKD,MutationCell-cell, cell-matrix relation
3、 abnormalityAltered epithelial proliferation, and. differentiationAbnorm. ECM Epith. proliferation Fluid secretionVascular injury CYST Interstitial inflammation fibrosis,Polycystic kidney clinical signs,Pain Haematuria Progressive renal insufficiency Proteinuria (not more than 2 g / day) Polyuria Hy
4、pertension,Polycystic kidney other accompaniing congenital anomalies,Hepatic cysts Berry anerysms Mitral prolapse other valvular anomalitiesTh.: Dialysis, renal transplatation cause: 40 % : coronary / hypertensive heart disease 25 % : infection 15 % : aneurysm rupture, hypertensive intracerebral hae
5、morrhage 20% : other causes,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,Clinical m
6、anifestation of renal disorders,Acute nephritis: macroscopic haematuria, mild proteinuria, mild hypertension Nephrosis: severe proteinuria 3.5g/dayhypoalbuminaemia, oedema, hyperlipidemia, lipiduria Acute renal insufficiency: abrupt oligo/anuria Chronic renal insuff/failure:,Clinical manifestation o
7、f renal disorders,Renal tubular defects: polyuria, electrolit disorders (Metabolic acidosis)Urine infections : pain, fever, etcNephrolithiasis: colica, haematuriaObstruction: pain, infection, stone formation,Clinical manifestations of renal disorders,Chronic renal insuff: Decreased renal reserve: GF
8、R =50 % Se creatinin, BUN norm. Free of symptomsRenal insufficiency: GFR 20-50 % Se creatinin,BUN anaemia, hypertension, sign: polyuria, Renal failure: GFR: 20-25%, oedema, hypocalcaemia, acidosis, neurological, GI, cardiovascular sequalesEnd-stage: GFR: 5%,General signs of renal insufficiency,Fluid
9、 /electroliteDehydrationOedemaHyperkalaemiaMetabolic acidosis,Calcium, phosphateHyperphosphataemyHypocalcaemySecunder hypoparathyreosisRenal osteodystrophy HaematologicalAnaemiaDiathesis,General signs of renal insufficiency,General signs of renal insufficiency,CardiovascularHypertenisonCongestive ca
10、rdiac insuffPulmonary oedema Serous membranesUremic pericarditis, pleuritis,peritonitis,General signs of renal insufficiency,GIVomitHemorrhageOesophagitis, gastritis, colitisNeuromuscularMyopathy, Neuropathy, EncephalopathyDermatologicalYellow skin, itching, dermatitis,Causes of Tubulointerstitial N
11、ephritis,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
12、nephropathy Oxalate nephropathy,Tubulointerstitial nephritis,Physical factors Chronic obstruction Radiation nephropathy Neoplasms Myeloma multiplex Immunological diseases Transplant rejection Sjgren sy Sarcoidosis Vascular disorders Other Balkan nephropathy Nephronophthisis medullary cystic disease
13、Idiopathic” interstitial nephritis,Pyelonephritis,InfectionAscendingE. ColiKlebsiellaProteusEnterobacterStreptococcus faecalisHaematogenousStaphylococcusE.Coli,Renal stones,Calcium oxalat, phosphat 70% MG, ammonium phosphat 15-20%struvit Urate 5-10 % Cystin 1-2 %,Factors leaidng to stone formation:,
14、High ionic - concentration Low ph, Decreased renal flow, Bacteria,Deficiency in inhibitors of crystal formation:Pyrophosphate Diphosphonate GAG Osteopontin Nephrocalcin,Diseases of the urinary bladder,Symptoms of diseases of the urinary bladder,Pain, dysuriaUrinary frequencyHaematuriaCystoscopy: red
15、 spots on the mucosa, protruding flat or papillary tumor, rigid bladder wall,Diagnostic possibilitiesLaboratory tests + cytologyCulturegraphyCystoscopyTUR,BenignPapilloma Inverted papillomaMesenchymal,Malignant UCC- (TCC) - papillary - CIS Adenocc SQCC Sarcoma Lymphoma,Metastatic Infiltration”,Tumor
16、s of the bladder,Malignant UCC papillary CIS,Tumors of the bladder,Bladder cancer - etiology,Smoking Arylamine, 2-naphthylamine Schistosomiasis Analgetics Cyclophosphamide Radiation injury Chr. abnormalities 9p,9q deletion (superfitial papillary, rarely flat lesions) 9 monosomy, 17p deletion (p53) 1
17、3q, 11p,14q deletion,Papillary tumors- Nomenclature, STAGE,Papilloma PapillomaGrade I PUNLMPLow GradeGrade IIGrade III High Grade,WHO WHO/ISUP,CIS,Gr. III flat” tumorDifferent from papillary TCCPrognosis is worth,Bladder tumors prognosis, therapy,Papilloma, PUNLMP Ta, T1 : 98 % min. 10 yLGSurgicalMultifocal laesions: local chemotherapy, CIS, HG TCC /T1 BCG instillationHG TCC / T2 HG TCC /T1 BCG refractory cystectomy,
