Hematuria in Children Flashcards
Hematuria in Children
Presence of red blood cells (RBC) in urine (>10 RBC per mm3 of freshly voided, unspun urine or >5 RBC per high power field (HPF) of 10 ml of fresh urine, centrifuged at 2,000 rpm and resuspended in 0.5 ml). Microscopic hematuria (microhematuria) refers to the presence of RBC without urine discoloration, detected by microscopy or chemical (dipstick) analysis. Threshold for a positive readout is a hemoglobin concentration of approximately 0.6 mg/l. Microscopic hematuria is much more frequent than gross hematuria.
Hematuria in Children
While malignancies of the urogenital tract are an important differential diagnosis in adults, they are rare in children (e.g., Wilms tumor).
Hematuria in Children
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Hematuria in Children
Def. : ≥ 5 RBCs/µl of urine DD/Red urine without RBCs
Hemoglobin and Myoglobin
Drugs : Chloroquine, desferoxamine, ibuprofen,
iron sorbitol, metronidazole, nitrofurantoin, rifampicin, salicylate, sulfasalazine
Dyes (Vegetables, fruits) : Beets, Blackberries, food coloring
Metabolites :
Homogentisic acid, Melanin methemoglobin, Porphyrin, Tyrosinosis, Urates
Causes of hematuria
Causes of hematuria
Glomerular :
Isolated renal dis. : IgAN, PSGN
Multisystem dis. : SLE; HSP, HUS Extra-glomerular :
Upper urinary tract : Pyelonephritis, ATN, Vascular
malformation (Hemangioma), Hypercalciuria,
hydronephrosis, trauma
Lower urinary tract : Cystitis, urolithiasis, heavy
exercise
Macroscopic hematuria
Macroscopic hematuria can be the presenting sign of parenchymal kidney disease (e.g., membranoproliferative glomerulonephritis, Alport syndrome, IgA nephropathy, polycystic kidney disease), yet >40 % of children presenting gross hematuria have no identifiable cause.
Fifteen to 20 % of patients with painless micro- or macrohematuria have hypercalciuria.
History
A history of passage of clots in urine suggests an
extraglomerular cause of hematuria.
➢ A history of fever, abdominal pain, dysuria, frequency, and
recent enuresis in older children may point to a urinary tract infection as the cause of hematuria.
➢ A history of recent trauma to the abdomen may be
indicative of hydronephrosis.
history of early-morning periorbital puffiness, weight gain,
oliguria, the presence of dark-colored urine, and the presence of edema or hypertension suggests a glomerular cause.
➢ Hematuria due to glomerular causes is painless.
➢ A history of a recent throat or skin infection may suggest
postinfectious glomerulonephritis.
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A history of joint pains, skin rashes, and prolonged fever in adolescents suggests a collagen vascular disorder.
The presence of anemia cannot be accounted for by hematuria alone, and, in a patient with hematuria and pallor, other conditions such as systemic lupus erythematosus and bleeding diathesis should be considered.
Skin rashes and arthritis can occur in Henoch-Schönlein purpura and systemic lupus erythematosus.
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Information regarding exercise, menstruation, recent bladder catheterization, intake of certain drugs or toxic substances, or passage of a calculus may also assist in the differential diagnoses.
Because certain diseases that present with hematuria are inherited or familial, asking for a family history that is suggestive of Alport syndrome, collagen vascular diseases, urolithiasis, or polycystic kidney disease is important.
Tea or cola-coloured urine, facial/body edema, HTN, Oliguria Hx of recent URTI, Skin or GI infections Hx of skin rash, joint involvements Frequency, dysuria, fever Flank mass Headache, visual changes, epistaxis, heart failure Child abuse F. Hx Physical exam.
The most common cause of gross hematuria in chidlren : UTI
The most common cause of gross hematuria in chidlren : UTI Recurrent episodes of gross hematuria : IgAN, Alport syndrome Thin glomerular basement membrane dis. Hypercalciuria or urolithiasis
Lab., Radiologic evaluation : Asymptomatic patient
Persistent asymptomatic isolated microscopic hematuria :
Persistent asymptomatic isolated microscopic hematuria : Urine C/s, Urine Ca/Cr Renal/bladder U/S Urine analysis of all first degree relatives S. creatinine, electrolytes CBC, peripheral blood smear ANA, Anti-DNA Coagulation studies VCUG
Hematuria
Referral to Ped. Nephrologist :
Hematuria
Referral to Ped. Nephrologist :
Patient with Nephritis Patient with hypertension, Renal insufficiency Patient with Urolithiasis / Nephrocalcinosis F.Hx of renal dis. Patient in persistent asympt. Hematuria >1 year
Renal biopsy
ACUTE NEPHRITIC SYNDROME
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ACUTE NEPHRITIC SYNDROME
a clinical syndrome defined by the association of hematuria, proteinuria, and often arterial hypertension and renal
failure.
due to glomerular injury with glomerular inflammation.
Glomerular injury
Glomerular injury
Immunologic. inherited (presumably biochemical). coagulation disorders.
Immunologic injury is the most common cause and results in glomerulonephritis
MC cause مهم جداً
Mc of gross hematuria = UTI
MC of glumerular gross hematutria IgAn
2nd PSGN
PSGN
Clinical feature
Age : 5-12 years uncommon before 3 yr. OA Acute nephritic syndrome 1-2 wks after antecedent streptocc.
Pharyngitis or 3-6 wks.
after Streptococal Pyoderma
Edema : 2º to salt & water retention NS in 10-20% of Pt. Nonspecific sympt.: fever, malaise, …etc Acute phase generally resolves within 6-8 wks.
PSGN dig
Diagnosis :
Urine analysis CBC, Urea, Cr., Electrolytes, C3, C4 Evidence of streptococcal inf.
(ASO titre)
Anti-DNase B level (Skin Inf.) Streptozyme test Renal biopsy
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Considerations for renal biopsy
In PSGN
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the development of acute renal failure nephrotic syndrome, the absence of evidence for streptococcal infection, the absence of hypocomplementemia, the persistence of marked hematuria or proteinuria or both, diminished renal function, or a low C3 level for more than 3 mo after onset
PSGN
Complications
Prevention
Treatment
Prognosis
Complications
Hypertensive encephalopathy : 10% CHF, hyperkalemia, ↑ PO4, ↓Ca Acidosis, Seizures, Uremia
Prevention : Early systemic antibiotic therapy of streptococcal throat and skin infections will not eliminate the risk of glomerulonephritis. Family members of patients with acute glomerulonephritis should be cultured for group A beta-hemolytic streptococci and treated if culture-positive
Treatment : Symptomatic
Prognosis : Complete recovery in >95%
Recurrence : Extremely rare
IgA nephropathy
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IgA nephropathy
IgA nephropathy is the most common glomerulonephritis worldwide.
Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus.
There are other diseases associated with glomerular IgA deposits, the most common being IgA vasculitis (formerly known as Henoch–Schönlein purpura [HSP]),
IgAN is typically diagnosed during teenage years and young adulthood. Natural disease progression leads to end-stage renal disease within 20 years of diagnosis in 30–35 % of patients (range 20–50 %).
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Mainfestations
The commonest mode of presentation is macroscopic hematuria following an upper respiratory tract
infection.
Signs and symptoms
IgA nephropathy
The classic presentation (in 40–50% of the cases) is episodic hematuria,
which usually starts within a day or two of a non-specific upper respiratory tract infection(hence synpharyngitic), as opposed to post-streptococcal glomerulonephritis,
Renal function usually remains normal, though rarely, acute kidney failure may occur
Recurrent macroscopic hematuria with or without proteinuria is the hallmark of childhood IgA nephropathy and is the most common presenting symptom.
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A smaller proportion (20-30%), usually the older population, have microscopic hematuria and proteinuria (less than 2 gram/day).
Very rarely (5% each), the presenting history is:
Nephrotic syndrome Acute kidney failure Chronic kidney failure
Outcome
IgA nephropathy
Outcome
Serum complement levels are normal. The course in the majority of patients is benign, including spontaneous
resolution. Yet, up to 20% of paediatric patients progress eventually
to CKD stage 5, although the rate of progression is usually very slow.
Henoch-Schönlein purpura (HSP)
➢ The peak incidence is approximately age 4-5 years. ➢ A slight male preponderance is observed
➢ Its onset is preceded by an upper respiratory infection in at least 30% of
patients.
➢ Purpuric palpable rash is seen predominantly on the posterior aspects of the
body and lower extremities.
➢ Abdominal pain, joint pain, and swelling are often present.
s usually reveals microscopic hematuria and
sometimes proteinuria
➢ Hematuria is likely related to deposition of IgA immune
complexes and associated inflammatory processes in the glomerular mesangium.
➢ complement 3 (C3) and antinuclear antibodies (ANA) levels may be needed to rule out systemic lupus
Hemolytic uremic syndrome (HUS
Hemolytic uremic syndrome (HUS)
➢ The hemolytic-uremic syndrome is a common cause of
acute renal failure in children.
➢ a thrombocytopenic microangiopathic hemolytic anemia
and renal failure after a preceding bout (1-15 d) of often bloody gastroenteritis.
➢ Gross hematuria may be observed in some cases, but the
more usual finding is microscopic hematuria.
Hemolytic uremic syndrome (HUS) is defined clinically by the triad of microangiopathic hemolytic anemia, thrombocytopenia and renal involvement/acute kidney injury (AKI).
The term “typical” (or “classical”) HUS is reserved for HUS induced by Shiga toxin-producing bacteria (STPB, predominantly Shiga toxin-producing Escherichia coli (STEC); STEC HUS). “Atypical” initially referred to all non- STEC HUS, but is now commonly applied to HUS related to the dysregulation of the alternative pathway (AP) of complement (aHUS).
HUS
HUS
Hemolytic-uremic syndrome is generally classified into the more common diarrhea-associated hemolytic-uremic syndrome (D+ HUS) and the atypical hemolytic-uremic syndrome (D- HUS).
D+ HUS is caused by shiga toxin produced by Escherichia coli O157:H7.
It is rare in blacks and has a female preponderance
The disease mostly occurs during the summer and the autumn seasons.
HUS
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HUS
Hemolytic-uremic syndrome is generally classified into the more common diarrhea-associated hemolytic-uremic syndrome (D+ HUS) and the atypical hemolytic-uremic syndrome (D- HUS).
D+ HUS is caused by shiga toxin produced by Escherichia coli O157:H7.
It is rare in blacks and has a female preponderance
The disease mostly occurs during the summer and the autumn seasons.
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HUS
Although ingestion of undercooked contaminated ground beef products is the most common source of infection, cases have been reported after the ingestion of raw milk, fruits, and vegetables in contact with manure.
Infection has also been reported after swimming in pools or lakes and after exposure to the pathogen in nursing homes and day care centers.
The disease process begins when Shiga toxin is absorbed through damaged colonic mucosa and binds to glycophospholipid globotriaosyl ceramide (Gb3) receptors in vascular endothelial cells.
Although involvement of the GI tract, kidney, and the hematologic system are commonly observed, involvement of other organs such as the liver, pancreas, gall bladder, lungs, and the CNS have been frequently reported.
Treatment is mainly supportive.
Meticulous care of electrolyte abnormalities, hypertension, and, if necessary, dialysis has resulted in a dramatic decrease in mortality rates (from 40% in the decades when it was first described to the 5-10% seen today).
