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Eva Pazkov
Department of Pediatrics, University
Hospital Hradec Krlov
Charles University in Prague,
Medical Faculty Hradec Krlov
Lecture topics
Hemolytic-uremic syndrome
Hematuria
Akute and chronic glomerulonephritis
Nephrotic syndrome
Overview of glomerular diseases classification
Urinary tract infections
Congenital abnormalities
Vesico-ureteral reflux
Urinary tract obstructions
Hemolytic-uremic syndrome
Most frequent etiology of pediatric ARF
Etiology: multifactorial, preceded by infections with GIT
strains of E-coli
Hemolytic-uremic syndrome
Lab signs: low hemoglobin, slightly elevated
bilirubin, schistocytes in blood smear,
thrombocytopenia, negative direct Coombs test,
proteinuria, hematuria, casts, hyperazotemia
Therapy: renal failure treatment, peritonal
dialysis in children 3 months to 3 years (60 80
%), hemodialysis in preschool children,
transfusion in extreme anemia, antiaggregation ?
(dipyridamole), antihypertensive drugs
Prognosis: better in infants, worse in older
children
Hematuria
Differential diagnosis: discolored urine with
blood or other compounds
Urine colors:
Dark yellow:
concentrated urine
bile pigments
Red:
blood
myoglobin
porphyrins
beetroot, blueberries
urates
blood
hemogentis acid
Glomerulonephritis (GN)
Immunologicly mediated inflammation of
glomeruli
GN immunokomplex IK noted in
glomerular membrane induction of
pathologic changes
GN based on antibodies against basal
membrane
Glomerulonephritis (GN)
Acute
Rapidly progressive
Chronic
Classification according to renal biopsy
Rapidly progressive GN
RPGN fast, irrevesible glomerular damage
Oliguria or anuria, ARI lasting weeks, months, rarely in children
1. RPGN with anti-basal membrane antibodies (Goodpasture syndrome)
2. RPGN with granular deposits (IK type)
3. RPGN with ANCA antibodies
Clinical picture:
Therapy:
Prognosis: grim
Chronic glomerulonephritis
Lasting years, progressive
Histologic finding classification
Urinary syndromes hematuria and proteinuria
Inflammatory changes: diffuse, focal, segmental
Proliferative endocapillary
extracapillary (worse)
Non-proliferative
Morphologic division
Mesangioproliferative GN
1. No clinical manifestation + mild
hematuria + mild proteinuria
Therapy: symptomatic
2. With nephrotic syndrome: (edema +
proteinuria + hypertension)
Membranoprolipherative GN
Mesangial enlargement + thickening of basal
membrane
Localisation of deposites:
1. subendothelial
2. intramembranous
3. subepithelial
male : female = 2 : 1
episodes of gross hematuria and microscopic HU in relation to infections
renal functions normal, proteinuria small (< 1g / 24 h)
C3 in serum normal
Alport syndrome
X - linked
Most frequent hereditary nephritis, progressive
Variability in clinical manifestation, histologic character
Histology: mesangial proliferation, thickening of capillary
wall, progression of glomerular sclerosis, interstitial
inflammation, fibrosis
Clinical signs: asymptomatic microscopic hematurie (or
episodes of macroscopic hematuria)
+ proteinuria biopsy
+ vision, hearing impairment
Therapy: specific doesnt exist (RI in 2./3. decenium)
Membranous GN
Rare in children as a reason for hematuria x most frequent reason of
nephrotic syndrome in adults
1.
2.
primary
secondary (conjoined with hepatitis B)
Histology: diffuse thickening of GBM, deposits of IgG + C3 on the
epithelial side of the membrane
Immunocomplex disease
Clinical signs:
Renal biopsy
Prognosis: mostly spont. regression, sometimes persistant proteinuria
Therapy: restriction on salt + diuretics, or intermittent prednisone
Other GN
Focal segmental glomerulosclerosis
nonselective proteinuria
severe course
in 10 years only 50% non-dialysed
corticoids
Nephrotic syndrome
Incidence 2:100 000
Functional a structural disorder of glomerular filter
Types of NS:
1.
2.
3.
Congenital
Primary idiopathic
Secondary
Laboratory findings:
GNF
systemic inflammation (SLE, HS purpura, diabetes mellitus,
amyloidosis)
vascular disorders (thrombosis of renal vein, constrictive
pericarditis)
infections malaria, syphilis
toxins and alergens
tumor (hemoblastosis)
Ortostatic proteinuria
13 15 years
Vertically proteinuri
Horisontally no proteinuria
Etiology ?
High children with prominent lumbar scoliosis
blood: FW, KO and diff., biochemistry urea, kreatinin, uric acid, Na,
K,Cl, CaP, cholesterole, ALT, AST, total protein, albumin,
electroforesis of proteins
indicated (suspicion of CGN): ASLO, CIK, C3,C4, quant. Ig, ANF,
ANCA, anti ds DNA, HBsAg
urinanalysis
Hamburger sediment 1-2x per week
Quantitative proteinuria in 24 h and selectivity 2x week
KBU 3x
creatinine clearence
concentration test with Adiuretin after stable state reached
Abdominal US (ascites !) and urinary tract
Nephrotic syndrome
1.
2.
3.
4.
5.
6.
Relapse treatment:
Prednison 2 mg/kg/d (max. 80 mg/d) in 3 daily doses till negative
proteinuria, = 6x consecutive negative urinanalysis and then 2
mg/kg/every 2nd day 6 weeks
regimen modalities and supportive care the same
Indications for renal biopsy in nephrotic syndrome:
newborns and infants high probability of congenital NS
children older than 8 y in first attack of NS probability of CGN
corticoresistant NS (= no response to treatment with CS in 8 weeks)
NS with frequent relapses (2 and more relapses in 6 months and
relapsing within 2 months after corticotherapy finished)
corticodependent NS (relapse during alternate CS dosing or within 2
weeks after corticotherapy finished)
patients with significant signs of chronic nephritis hypertension,
azotemia, hypocomplementemia, macroscopic hematuria
Lupus nephritis
Nephritis in Henoch-Schnlein purpura (anafylactoid purpura)
Berger nephritis (IgA nephropathy)
Goodpasture syndrome
Glomerular impairment in systemic infections
Septicemia
Infectious endocarditis
Shunt nephritis (in arteriovenous shunt)
Syphilis
Parasite nephropathy
Nephropathy in malaria
Periarteritis nodosa
Wegener granulomatosis
Thrombotic microangiopathy (hemolytic-uremic
syndrome and thrombotic thrombocytopenic purpura)
Glomerular thrombosis (intravascular coagulation)
Benign nephrosclerosis
Malignant nephrosclerosis
Scleroderma (systemic sclerosis)
Diabetic glomerulosclerosis
Amyloidosis
Nephropathy in dysproteinemia
Nephropathy in liver disease
Nephropathy in sickle cell anemia
Nephropathy in congenital cyanotic heart disease and pulmonary hypertension
Hereditary nephropathy
Alport syndrome
Benign recurrent hematuria
Thin basal membrane syndrome
Nail-patella syndrome (osteonychodysplasia)
Congenital nephrotic syndrome (finnish type)
Infantile nephrotic syndrome (french type)
Diffuse mesangial sclerosis)
Fabry disease etc.
Symptomatic
with renal parenchyma impairment pyelonephritis
infections of lower urinary tract cystitis, urethritis
approx. 10-20% UTI not possible to specify from
history, clinical signs and basic lab investigation
Asymptomatic bacteriuria
bacteriuria not connected with clinical signs
Acute
Chronic or recurrent
Diagnosis of UTI:
clinical signs
lab findings
acute pyelonephritis
newborns: sepsis, lethargy, irritability, convulsions, failure to thrive,
fever, protracted icterus
infants: fever (neednt be), vomiting, diarrhea, failure to thrive
toddlers: fever, abdominal pain, vomiting, diarrhea
children: fever > 38,5C, side or back pain
chronic pyelonephritis
may have latent course
fatigue, subfebrilia of unknown origin, intermittent back and side
pain
late sign polyuria, headache (hypertension)
in 10% of children with acute PN scars in renal parenchyma, may lead to hypertension or
CRI
Risk factors for renal scarring:
urinary tract obstruction
VUR of higher grade
low age
delayed treatment
number of pyelonefritis attacks
unusual bacteriuria
US
MCG
urethra calibration in girl with relapses
in boy any age and in girls below 1 year of age after 1st UTI
in girls older than 1 year after 3rd UTI
Relapsing UTI urodynamic investigation
age
localisation of UTI
anomaly of urinary tract
Acute pyelonephritis
ATB i.v. 5-10 days, followed with ATB p.o. till total 10-14 days (normalisation of lab. parameters)
ATB p.o. according to sensitivity of agens cotrimoxazol, cephalosporine, amoxycilin
in older children without major alteration treatment can be initiated p.o. :
ceph
alosporines
es of 2. or
cephalosporin
or 3. generation
generation (cefuroxime
(cefuroxime-axetil Zinnat)
amoxycillin clavulanate
adequate treatment = urine sterile within 24 h
fever lowered within 48 72 h
pyuria vanishes within 3 4 days
CRP < 20 mg/l in 4-5 days
normalisation of FW within 2-3 weeks
if no treatment effect within 2-3 days:
res
resista
istance of etiol. agens
obstruction
obstruction of urinary tract
supportive care: ibuprophen lowers the inflammation size ; hydration, catheterisation in cong.
kidney and urinary tract anomalies
Resolution
Congenital abnormalities
of urinary tract
1. Bilateral renal agenesis 1 : 4000 births
Congenital abnormalities
of urinary tract
5.
a)
b)
UTI, in girls under 1 year in 57%, over 1 year in 36%, in boys under 1 year and
between 2-16 years in 30%
DMSA scan proof of renal scarring
MCG, US
Diagnosis:
Treatment: