syndromology in nephrology
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Syndromology in nephrology. Martina Peiskerová 1.LF UK Praha Klinika nefrologie 9/2007. Haematuria Proteinuria Leucocyturia Polyuria, oliguria, anuria Nephrotic syndrome Nephritic syndrome Acute glomerulonephritis Rapidly progressive glomerulonephritis Pulmonary-renal syndromes. - PowerPoint PPT PresentationTRANSCRIPT
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Syndromology in nephrology
Martina Peiskerová 1.LF UK Praha
Klinika nefrologie 9/2007
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Syndromology in nephrology - outline
• Haematuria• Proteinuria• Leucocyturia
• Polyuria, oliguria, anuria
• Nephrotic syndrome • Nephritic syndrome
• Acute glomerulonephritis• Rapidly progressive
glomerulonephritis • Pulmonary-renal syndromes
• Chronic glomerulonephritis ??
• Acute renal failure• Chronic kidney disease• (Chronic renal failure)• Uraemia
• Tubular syndromes
• Hypertension• Pain • Obstruction
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Haematuria• Definition > 2 red cells / hpf , • Hamburger’s sediment (3 hours) > 2000/min.
• microscopic x macroscopic • persitent x transient (exercise, menstruation, trauma,
infection)• glomerular x non-glomerular x uncertain origin (exercise,
over-anticoagulation, factitious)
• Source: kidney x urinary tract– Renal glomerular haematuria (IgA GN, thin basement memrane
disease, Alport, other GN) – Renal non-glomerular haematuria (tumours, cysts, calculs,
pyelonephritis, papillary necrosis, renal vein thrombosis) – Urinary tract bleeding (cystitis, prostate, tumours, stricture,
Schistosoma haematobium)
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Clinical importance of haematuria• Cause dependent• The most frequent causes: - inflammation or infection of the prostate or urinary bladder
– urinary calculi– malignant neoplasms– glomerular disorders
• Risk of malignity: age >40, smoking, NSA, pelvic irradiation, CFA treatment)
• Glomerular disorder more likely if:– proteinuria > 0.5 g/24h– dysmorphic erythrocytes present and red blood cells casts on
phase-contrast microscopy– ↑BP
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Diagnosis of haematuria - history and physical examination
• Pyuria or dysuria urinary tract infection• Respiratory tract infection postinfectious GN,
IgA nephropathy• Family history polycystic kidney disease,
hereditary nephritides• Low back pain ureteral obstruction• Physical exercise, injury post-exercise/post-
traumatic hematuria• Micturition disorders in older men prostatic
obstruction • History of bleeding from multiple sources
coagulation disorder
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Phase-contrast microscopy
• A-dysmorphic erythrocytes
• B-isomorphic erythrocytes
• C-acanthocytes• (spur / spiny /
star cells)• D- neutrophils• E-lymphocytes• F-eosinophils
(arrow),
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Diagnosis + Treatment of haematuria
• Urinalysis• Urine microscopy (sediment, phase-contrast)• PSA
• Imaging (US, IVU,CT, angiography)• Cystoscopy• Urine cytology
• Renal biopsy (in glomerular hematuria)
• Early diagnosis is essential• Treatment of the causing disorder
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Proteinuria
• benign (<1g/day, age < 30, fever, cold, exercise, CCF, seizures, postural), vs. pathological
• importance of abnormal proteinuria:
marker of intrinsic renal disease, prognostic factor for progression of renal insufficiency, risk factor for CV mortality, treatment target in CKD
• normally < 150 mg/day (albumine < 30 mg/ day)• microalbuminuria 30-300 mg/day
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Proteinuria 2Pathophysiology • glomerular (mostly albumin), • tubular (beta2microglobulin), • overflow (light chains in myeloma),• secretory (tumour, inflammation)
Quantity• Mild < 1,0 g/day• Significant 1,0 – 3,5 g/day (probably glomerular)• Nephrotic range > 3,5 g/day (probably glomerular)
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Leucocyturia
• neutrophiles – infection, GN, TIN• sterile pyuria (treated UTI, Chlamydia, calculi,
prostatitis, bladder tumor, papillary necrosis, TIN, TB)
• lymphocytes – TIN
Active urinary sediment• red blood cells, proteinuria, white blood cells,
and "casts" of cells
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Urinary sediment abnormalities„Mixed urinary findings“
• isolated haematuria or haematuria + mild proteinuria (<1g/day) … good prognosis
• isolated proteinuria (<3,5g/day) .. worse prognosis
• nephrotic proteinuria + haematuria … the worst prognosis
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Nephrotic syndrome= clinical complex consisting of:
• Proteinuria of >3.5g / 1.73m2 / 24 hours
• Hypoalbuminaemia
• Oedema
• Hyperlipidaemia
• Lipiduria
• Hypercoagulability
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Patophysiology of the nephrotic syndrome. Primary insult- increased glomerular permeability, causing plasma protein leakage into urine. Hypoalbuminemia is the cause of the main clinical features.
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Metabolic albumin turnover in healthy subjects vs. subjects with nephrotic syndrome.
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The “underfill” mechanism of edema formation. In this theory, hypovolemia (caused by hypoalbuminemia and decreased oncotic plasma pressure) is the main cause of renal Na+ a H20 retention.
??
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The “overfill” mechanism of edema formation. In this theory, abnormal renal Na+ and H20 retention is the main cause of Starling forces alteration at local tissue level.
??
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(Possible) consequences of proteinuria and lipid spectrum abnormalities.
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Diagram showing pathogenetic factors leading to hypercoagulability, tromboembolism and renal vein thrombosis.
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Causes of nephrotic syndrome
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Treatment of nephrotic syndrome• Symptomatic
– NaCl, H20 restriction
– diuretic therapy
– ultrafiltration
– nephrectomy
• Specific (depending on the causative disease)– immunosuppressive
therapy
– in amyloidosis, treatment of the causative process
• Treatment and prevention of complications• thromboembolism
• lipid metabolism disturbances
• immunoglobulin deficiency
• Ineffective: high protein diets, albumin supplementation.
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Nephritic syndrome
Glomerular inflammatory changes leading to• ↓ GFR • moderate proteinuria • oedema• hypertension • haematuria (red cell casts).
Typical example: Poststreptococcal glomerulonephritis in children
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Differences between nephrotic and nephritic syndromes
Typical features Nephrotic syndrome Nephritic syndrome
onset slow acute
swelling ++++ ++
arterial blood pressure normal increased
central venous pressure normal/low increased
proteinuria ++++ ++
hematuria present/not present +++
red cell casts not present present
glomerular filtration normal normal/low
serum albumin low normal/slightly decreased
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Histology (light microscopy) of acute poststreptococcal GN (marked invasion of polymorphonuclear cells)
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Histology of acute poststreptococcal GN (subepithelial hump-like deposits (strait arrows), subendothelial (arched arrows) and mesangial deposits). Endocapillary hypercellularity caused by neutrophil infiltration, endothelial and mesangial proliferation.
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Immunological findings in poststreptococcal GN
1. The serial estimation of complement - • Early in the acute phase, the levels of hemolytic
complement activity (CH50 and C3) reduced. • Within 8 weeks return to normal
2. Serial ASO titer measurements - twofold or greater rise in titer are highly indicative of a recent infection.
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Continuous alterations of structural changes caused by glomerular inflammation (upper part), clinical syndromes (middle part) and specific nosologic units (lower part).
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Rapidly progressive GN (RPGN)• Severe glomerular disorder → ↓ glomerular filtration in
days or weeks. • Clinical features: acute uremic or nephritic syndrome with
renal insufficiency rapidly → renal failure• Histology: negative IF (pauci-immune), crescentic GN
(crescent = half-moon-shaped lesion in Bowman’s space composed of proliferating parietal epithelial cells and infiltrating monocytes). Crescentic GN: >70% glomeruli are involved.
• Typical diseases : WG, GP and SLE.• + Extrarenal symptoms: pulmonary, skin, ORL, CNS..
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Large cellular crescent filling the Bowman’s space and compressing the glomerular tuft in WG.
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Acute renal failure 1• due to rapid ↓ GFR (hours, days)• retention of urea, creatinine, disorders in electrolytes, acid-base,
fluid homeostasis• oliguric x non-oliguric• anuria < 100 ml/day, oliguria < 400 ml/day, polyuria > 3l/day
• RIFLE classification Risk.. Injury…Failure.. Loss…End-stage)• Acute kidney injury classification : 1. s-creat to 1,5-2x baseline / oliguria > 6 hours 2. s-creat to 2-3x baseline / oliguria > 12 hours 3. s-creat above 3x baseline / anuria
* the highest risk – pulmonary edema, hyperkalemia
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Acute renal failure 2 - causes• Prerenal (from ↓ BP → ↓ GFR, or arterial stenosis or
NSA, ACEI)• Intrinsic - ATN (ischemic – e.g.myoglobinuria, myeloma casts, nephrotoxic – radiocontrast, drugs – gentamicin, vancocin, cisplatin) - vascular - acute GN - acute TIN• Postrenal (obstructive)
• Patients at risk of developping ARF: ↑age, DM, pre-existing renal disease, surgery, volume depletion, cardiac disease, cirrhosis, drugs – NSA, ACEI, ARB), myeloma
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Chronic kidney disease → Renal insufficiency → Renal failure
* exocrine dysfunction (ions – K, Na, P, H.., fluid, and other catabolites – uremic toxins retention)
• endocrine dysfunction (erythropoietin, 1,25 vitamin D metabolism, renin-angiotensin system)
→ laboratory: GF < 1,0 ml/s, hyperkalemia, hypocalcemia, hyperphosphatemia, metabolic acidosis, anemia
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Stages of kidney disease NKF/ KDOQI
1. Asymptomatic urinary abnormalities: GFR > 90 ml/min (> 1,5 ml/s)
2 Mild CRF: GFR 60-89 ml/min (1-1,5 ml/s)
3 Moderate CRF: GFR 30-59 ml/min (0,5-1 ml/s)
4 Severe CRF: GFR 15-29% (0,25-0,5 ml/s)
5 Approaching ESRD: GFR < 15 ml/min (< 0,25 ml/s)
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Uremic syndrome - clinical features 1
• Gastrointestinal – Anorexia, nausea, vomiting
• Neurological– Central: uremic encefalopathy (daytime
drowsiness, disorientation, myoclonus, coma)– Peripheral: uremic polyneuropathy (restless legs
syndrome)• Respiratory
– pulmonary edema
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Uremic syndrome - clinical features 2
• Cardiac– uremic pericarditis
• Dermatological– pruritus
• Hematological– fatigue due to anemia
• Endocrinological– secondary hyperparathyreoidism (bone pain),
dysmenorrhea
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Uremia
* in 3 different clinical situations → different clinical
features– acute renal failure – exocrine dysfunction, no time
for endocrine dysfunction development– chronic renal failure – endocrine and exocrine renal
dysfunction (fluid excretion usually preserved until late stages)
– dialysis treated CRF –caused by insufficient dialysis treatment and/or insufficient substitution of the decreased renal endocrine production (EPO, vitamin D, etc.).
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Treatment of uremia• Conservative:
- diet: Na, K, PO3 and protein restriction
- control of hypertension
- NaHC03 treatment to reduce metabolic acidosis
- anemia management (erythropoietin)
- secondary hyperparathyroidism management (vitamin D, phosphate binders)
• Renal replacement therapy: hemodialysis, peritoneal dialysis, renal transplantation
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Pulmonary-renal syndromes• Acute kidney disease (ARF or RPGN) + Pulmonary
haemmorhage• Features: cough, anaemia, dyspnoea, haemoptysis,
hypoxaemia, alveolar shadowing on CXR (df.dg. pulmonary oedema) + features of systemic disease: skin rush, sinusitis, artritis, fever, fatigue
• Main causes: ANCA vasculitis, antiGBM nephritis, SLE, Henoch-Schonlein purpura
• Other causes: pulmonary oedema, infection (pneumonia – Pneumocystis, viruses..), hantavirus, pulmonary emboli, acute respiratory distress syndrome
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Hypertension
• Primary hypertension – kidney is victim –
- vascular nephrosclerosis..
• Secondary hypertension – kidney is vilain
- glomerular and vascular diseases
• Control of hypertension is crucial in slowing progression of kidney disease → aim BP 120/75 mm Hg
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Tubular syndromesTubular dysfunction may occur in any renal injury
Tubular syndromes in the context of normal GFR:• Generalised – Fanconi syndrome : multiple tubular defects caus in variable degree → phosphaturia → rickets, osteomalacia, osteoporosis → aminoaciduria – no clinical sequelae → glycosuria – rarely hypoglycemia → defective bicarbonate reabsorption – renal tubular acidosis → Na loss → rarely ↓BP or metabolic alcalosis → K loss → hypokalaemia → muscle weakness, constipation, arrhytmias → proteinuria – LMW, no clinical sequelae → polyuria – dehydration → hypercalciuria → rarely nephrolitihiasis/calcinosis
• Isolated – genetic mechanisms involved - glycosuria - to distinguish from DM - aminoaciduria - e.g. cystinuria → recurrent cystin stone formation (AR inheritance) - phosphaturia – e.g. vitamin D resistant rickets (XR inheritance)
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PainAn agressive and destructive renal disease may be
painless !!
Loin pain - constant dull ache, may irradiate to abdomen, genitalia• cause: distension of the renal capsule• differential: nerve root irritation (T10-12) Ureteric colic - sudden onset, extremely sever, pale, distressed patient• localisation: loin, iliac fossa, genitalia, upper thigh• cause: passage of the stone, blood clot or necrotic papillae
Suprapubic pain • causes: over-distension of the bladder, cystitis, bladder cancer
Bladder irritability - dysuria, frequency, urgency• causes: over-distension of the bladder, cystitis
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Bladder outflow obstructionSymptoms• Obstructive – voiding: hesitancy, impaired force of stream,
incomplete emptying• Storage – filling : frequency, dysuria, urgency
Causes• Structural – prostatic hyperplasia, carcinoma, urethral
stricture
• Functional – bladder neck dyssynergia, DM, multiple sclerosis, spinal corde lesions, drugs - antidepressants