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Transcript of Organ Pathology Seminar / FAQ Kidney & Urinary Tract Pathology Jaroslava Dušková Inst. Pathol.,1st...
![Page 1: Organ Pathology Seminar / FAQ Kidney & Urinary Tract Pathology Jaroslava Dušková Inst. Pathol.,1st Med. Faculty, Charles Univ. Prague.](https://reader031.fdocuments.net/reader031/viewer/2022032803/56649e1b5503460f94b09fd5/html5/thumbnails/1.jpg)
Organ Pathology
Seminar / FAQ
Kidney & Urinary Tract Pathology
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
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Diseases of the kidney
and urinary tract
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Classification?
Most frequent/important ones ?
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Disease Nosologic Unit Definition Incidence , age/sex prevalence (if any) Etiology Possible clinical manifestation Pathogenesis
– macroscopy– microscopy– ultrastructure or other dg. tools – other (nonmorphological) dg. tools
Complications Healing & prognosis
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Non-neoplastic– inflammation
acute/chronic specific/non-specific superficial/intersticial
– pseudotumours Neoplastic
–benign–borderline–malignant
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Classification
inborn – agenesis, hypoplasia, dysplasia, cystoses
acquired– noneoplastic
inflamm. + lithiasis pseudotumours
– NEOPLASMS benign MALIGNANT
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Morphology features of “benign” and malignant hypertension in kidney
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Morphology features of “benign” and malignant hypertension in kidney
„Benign“ hypertension : atherosclerosis, fibroelastosis, hyalinized arterioles multiple scars – granular surface
Malignant hypertension (diast. pressure over 130mmHg) -autoimmune- IB against renin –angiotensin components. Fibrinoid necroses of arterioles, kidney edema & hemorrhages, necroses of kidney cortex. Death from brain haemorrhage or heart failure if not treated aggresively
RQ 99, 100
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Vasculitides Affecting the Kidney Systemic AB against BM – Goodpasture – pulmorenal
syndrome Vasculitides with immune complexes – Henoch-
Schönlein, SLE, ANCA –positive
– Wegener´s granulomatosis– microscopic polyangiitis– Churg- Strauss syndrome with eosinophilia and
astmaKidney morphology : necrotizing pauciimmune
GN with crescents
RQ 101
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Glomerulonephritis/Glomerulopathy
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Glomerulonephritis/Glomerulopathy primary (inborn, acquired) – secondary
(following or accompanying another disease like SLE, hepatitis C etc.)
Manifesting clinically as:– proteinuria-nefrotic syndrome– hematuria prevalent– combined (h+p)-uria– GN in vasculitides– GN in SLE– chronic GN
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Glomerulonephritis
special group of inflamm. glomerular diseases caused by:– depositon of immune complexes– antibodies against glomerular
components– antibodies against glomerular depositions– ANCA – anti-neutrophil-cytoplasm-
antibodies
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Glomerulonephritis – clinic– nephritic syndrome
hypertension hematuria mild proteinuria oedema
– nephrotic syndrome massive proteinuria hypalbuminemia oedema hypercholesterolemia
Terminal stage of kidney failure UREMIA
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Glomerulonephritis - morphologyMacroscopy: early: mild enlargement, petechiae, late: end stage kidneyMicroscopy: glomerular changes & scaring
Diagnosis: clinical symptoms, punction biopsy immunohistochemistry and electron microscopy
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Glomerulopathies manifesting with acute nephritic syndrome
Acute postinfectious (strepto and others, SLE…)
Kidney morphology : GN with increased cellularity (mesangial and endocapillary)
Membranoproliferative – secondary to hepatitis B, C, HIV, SLE, neoplasms….
RQ 105
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Glomerulopathies with hematuria
Clinic: hematuria - diff. dg!!!. Progression to failure
IgA nephropathy– Kidney morphology : mesaingioproliferative
GN with IgA deposits Henoch Schönlein Alport syndrome – mutation of colagen IV gene
RQ 105
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Rapidly Progressive GN Clinic: hematuria, proteinuria, loss of kidney function Kidney morphology : GN with crescents Etiology - variable:
– ANCA –positive Wegener´s granulomatosis microscopic polyangiitis Churg- Strauss syndrome with eosinophilia and astma
– anti GBM GN
– Vasculitides with immune complexes – Henoch-Schönlein, SLE,
– idiopatic…(= unknown)
RQ 103
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Glomerulopathies with nephrotic syndrome
Clinic: proteinuria Minimal change disease (in children ) – fusion
of pedicels, response to steroid th. NSAID related GN in adults Focal Segmental GlomeruloSclerosis: heroin,
secondary to gl.-loss nephropathy Amyloid Diabetic GN
RQ 104
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SLE and its Kidney manifestations Multisystem autoimmune disease with rash,
artralgiae, oral ulcers, RENAL DISORDERS… Libman –Sacks endocarditis, CNS dmage…..
Antinuclear and other antibodies 6 classes of kidney involvement (minim,
mesangial, focal, diffuse, membranous, advanced) – difference in therapy and prognosis
RQ 106
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Diseases of Kidney Tubules and Intersticium
Acute tubular necrosis ATN ( drugs - atb, toxins – heavy metals, - shock kidney– oliguria <400ml - anuria < 100ml - subsequently polyuria
>3000ml in 24 hours
Tubulointersticial nefritis TIN (bacteria, viruses, metabolic disorders)
Drug induced – NSAID, analgetic – phenacetin
Light chain deposition disease
RQ 98
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Ascendent kidney infections
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Ascendent kidney infections
tubulointerstitial nephritis – pyelonephritis mostly G- bacteries risk increased in
– diabetics, – pregnant, – lithiasis
complications: – urosepsis– amyloid
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Pathology of gonorrhoea
Man urethritis gonorrhoica
anterior urethritis gonorrhoica
posterior – strictura partis membranaceaa urethrae
balanitis erosiva (esp. in phimosis)
prostatitis abscedens vesiculitis seminalis
purulenta hydrocele acuta
Woman endocervicitis colpitis ulcerosa endometritis salpingitis purulenta pelvic inflamm. tumour
Newborn
vulvovaginitis
conjunctivitis
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Systemic Effects of Uremia – 1/2 skeletal – osteitis fibrosa
– demineralization and fibrosis – osteomalacia (rickets), pain, spontaneous fractures
cardiopulmonary & blood– hypertension, normochromic and normocytic
anemia, edema, pericarditis – friction rub GIT
– anorexia, nausea, mouth ulcers, stomatitis, peptic ulcers, colitis, bleeding, hiccups
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Systemic Effects of Uremia – 2/2 NEUROLOGIC – ENCEPHALOPATHY
– fatigue, loss of attention, problém solving difficulties, motor coordination, twitching, stupor , coma
Integumentary & immunologic– abnormal pigmentation and pruritus– increased risk of infection and neoplasms
Reproductive– menorrhagia, amenorrhea, – infertility, loss of libido – both sexes affected
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Kidney Transplantation and Rejection Morphology
Rejection – result of MAJOR HC and minor HC glycoproteins and peptides
T and B lymhocytes activity Acute cellular: (Banff criteria)
– mild – intersticium and tubules damage– severe – arterial damage
Acute humoral (early and late – years): Donor Specific Antibodies
Chronic: intersticial fibrosis , vascular damageGraft damage of non- rejection type: infection – viruses
–CMV, drugs, preceding diseases – diabetes….
RQ 107
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Kidney Neoplasms ?
classification principles clinical manifestation most frequent representatives complications
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Kidney Neoplasms
benign - mixed mesench., adenoma, papiloma
MALIGNANT – ADENOCARCINOMA,
UROTHELIAL CARCINOMA
Complications : anemia, polyglobulia, METASTASES,
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Kidney Tumours - mesenchymal
Angiomyolipoma - mixed mesenchymal tumour ICD-O M 8860/0
sporadic or associated with tuberous sclerosis (TSC 1,2 genes – 9q34)
occasionally large & multiple, bulging
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Papillary (Chromophillic) Ca M 8260/3 10% In dialysed more frequent; can be multifocal and
bilateral X-ray hypovascular Histology – papillary/ tubulopapillary
type 1 – cubic cellstype 2 - cylindric cells (worse prognosis)
Genetics – trisomy or tetrasomy 7 and 17
in men often Y chromosome missing mutation of c-met oncogen
Prognosis : G, pT dependentslightly better than in conventional ca
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Chromophobe Carcinoma M 8317/3 5%
Macro - brown color Mikro - solid, cytoplasms clear or
eosinophillic positive in Hale´s colloidal iron staining raisin-like cell nuclei
Elmi microvesicles in cytoplasm
Genetics missing chromosomes -1, 2, 10, 13, 6, 21, 17
Prognosis: G, pT dependent
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Oncocytoma M8290/0
Kidney cortex may be multicentric and bilateral Macro – tan with a central stellate scar Micro - eosinophillic granular cytoplasm
bizarre nuclei Elmi – mitochondria filling up the cytoplasm Biological behaviour benign
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Nephroblastoma (Wilms´tumour)M 8960/3 syn. - embryonal adenosarcoma Children - preschool age Macro: gray-white large retroperitoneal
mass palpable through abdominal wall Micro: undifferentiated renal blastema,
tubular and glomeruloid formations may be present
Prognosis: curable (stage!) – resection and chemotherapy
Follow up: - nephrogenic rests
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Nephroblastoma (Wilms´tumour)M 8960/3
Genetics WAGR syndrome – Wilms-Aniridia-Genital Anomaly-RetardationDenys – Drash syndrome DDS - gonadal dysgenesis and renal abnormalitiesinactivation of the WT 1 geneBeckwith –Wiedeman syndrome BWS - organomegaly ( tongue, kidney, liver, hemihypertrophy)loss of silencing of maternal IGF 2 gene
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Kidney Carcinoma – report
type grade (Fuhrmann) stage prognostic factors (MIB1, p53…)
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Urothelial Neoplasms ?
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Urothelial Neoplasms
papiloma (rare) urothelial papilocarcinoma
incidence growing
Symptoms– hematuria
microscopic – anemia macroscopic
– obstruction – ureteri and pelvis
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Urothelial Carcinoma – report
type grade resection completeness stage prognostic factors (MIB1, p53…)
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Urothelial Carcinoma - complications
local recurrence progression metastases