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PEDIATRIC RENAL DISEASES
Agnes Alarilla-Alba, MD FPPS,FPSN, FPNSP
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Pediatric Renal Disease Objectives:
To present the common pediatric renal diseases To give the common clinical manifestaions, physical findings, pathognomonic laboratory findings and management principles To emphasize the differences and similarities of common adult and pediatric illnesses.
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Syndromes in Nephrology
Acute Nephritic Syndrome Nephrotic Syndrome Isolated Urinary Abnormalities Acute Renal Injury Chronic Renal Injury Urinary Tract Infection Renal Tubular Defects Hypertension Urinary Tract Obstruction Urolithiasis
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CASE 1 Give me pertinent thoughts about the case:
A 16 year old male , joined the Alpha Beta Gamma Frat. He underwent hazing and was admitted to the fraternity . Sister saw his brother black and blue and later at night urinated red urine. The sister panicked and rushed him to the hospital?
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RED URINE What causes the red urine is there blood or none
Dipstick test done: + Urinalysis revealed (-) RBC
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Manifestations HIGHLY colored urine: one of the most alarming manifestions of renal disease
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Definition Urine microscopic examination findings of
1. red blood cells (RBC) > 6/ul in a fresh uncentrifuged midstream cleancatch specimen RBC > 3/hpf in centrifuged sediment from 10 ml of freshly voided midstream urine
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Hematuria If highly suspicious:May do DIPSTICK test However take note of false positive results which is seen in myoglobinuria and hemoglobinuria TO CONFIRM microscopic analysis of the urine is warranted
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Diagnostic test if there is blood in urine
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HEMATURIA (dipstick) Fever Strenuous exercise Intake of ascorbic acid
FALSE POSITIVE FALSE NEGATIVE Fever Strenuous exercise Intake of ascorbic acid Use of formalin
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Diffrentials if + dipstick
Hematuria Myoglobinuriaviral myositis, crush injury, DIC, toxins and prolonged seizure Creatinine kinase is five times elevated Hemoglobinuria hemolysis
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* Check intake of medications
Manifestations RED URINE * Check intake of medications Spurious: medications and food Rifampicin Nitrofurantoin Furazolidone Metronidazole Sulfa Phenazopyridine Pyridium Methyldopa
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Manifestations RED URINE Beets Berries Urates Serratia Marcesens Alkaptonuria Bilirubin
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Differential diagnosis of Hematuria
Dark brown Red /PINK Bile pigments Methemoglobinemia Alanine Laxative Drugs(resorcinnol, thymol) Alkaptunuria Free hemoglobin Myoblobin Porphyrins Urates in high concentration Foods Drugs 9benzene, chloroquine desfuroximme)
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Screening : Urinalysis
At age of 5 and Once at the second decade of life
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CASE 2 A six year old child complained of flank pain. He urinated that pm and noted to have bloody urine. The pain persisted and It was so unbearable that he was rushed to the ER. Urinalysis revealed: Ph of 6 Specific gravity of 1.025 Dipstick: + heme Wbc: 3/hpf RBC: tntc + calcium oxalate cystals
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HEMATURIA Presence of 5 or more RBC per high power field in the urine
Look for morphology because it will give you a clue on the source of the RBC
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Causes of Hematuria glomerulus/collecti ng duct and interstitium
UPPER Urinary Tract Lower Urinary tract: glomerulus/collecti ng duct and interstitium brown colored RBC casts dysmorphic RBC proteinuria pelvocalyceal system down wards reddish or pink urine passage of blood clots eumorphic red blood cells
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Hematuria Renal Stones FEVER Strenuous exercise
Non Glomerular Glomerular Renal Stones FEVER Strenuous exercise Mechanical Trauma ( masturbation) Menstruation Foreign Bodies UTI( cystitis, urethritis,) coagulopathie Without systemic involvement With systemic involvement
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Glomerular Hematuria Isolated recurrent gross hematuria
Thin Basement Membrane Disease Ig A nephropathy Alport Syndrome Glomerulonephritis associated with infection AGN Other Chronic Infection
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Glomerular Hematuria Associated with systemic manifestations
HUS HSP SLE Glomerular Illnenesses AGN MPGN Membranous
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Cola /brown urine/rbc cast/ nephritic syndrome
WORK UP for HEMATURIA Cola /brown urine/rbc cast/ nephritic syndrome YES GLOMERULAR CBC Electrolytes BUN/Crea Serum protein Cholesterol Aso/ana 24 hr protein NO Non glomeular U c/s Sickle cell Ultrasound of KUB Crystallucia hyrdronephrosis
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Isolated Glomerular disease
Ig A disease Alport Disease Thin Basement Membrane disease
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Ig A NEPHROPATHY Most common chronic cause of hematuria
Deposition of Ig A in the messangial area M>F Gross hematuria noted 1-2 days after URTI,loin pain C3 normal, Ig A elevated only to 15% of patients so not of use
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ALPORT Syndrome Due to mutation on type 4 collagen
85% due to mutation to c\COLA45 gene encoding for the alpha of type 4 collagen Hearing deficits and ocular abnormalities ( anterior lenticonus)
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Alport Syndrome PROGNOSIS
Progressive disease in % at 15 to 2o years after onset Poor prognostic factors: increased proteinuria, persistent hypertension, decreased renal function
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Alport Syndrome PROGNOSIS Primary treatment is BP control,
Fish oil is for progressive renal disease Tonsillectomy known to decrease incidence of hematuria Renal transplantation: recurrence in 20-30%
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Thin Glomerular Basement Membrane disease
Persistent hematuria and the thinning of the GBM on EM Mutations in COL4A3 and COL4A4
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Acute nephritic syndrome
Hematuria Edema Hypertension Oliguria Azotemia
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Acute Post Streptococcal Glomerulonephritis(APSGN)
Most common type of AGN Most common form of Post Infectious AGN Classic example of Post Infectious AGN
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Acute Poststreptococcal GlomAlonephritis
Sudden onset (previously well) Edema Hematuria Hypertension Oliguria 1-4 weeks following a proven or highly presumed streptococcal infection
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Clinical signs & symptoms
PATHOPHYSIOLOGY Antigen-IgG-C3 Complex Infiltration of inflammatory cells & proliferation of glomerular cells & matrix expansion basement membrane permeability glomerular filtration surface GFR Na & water retention Clinical signs & symptoms
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Case 3 A 12 year old, female child complained of leg swelling. Upon history, she was complaining of oral ulcers. Initially she thought it was because of the braces. Later she complained of blurring of vision. Had several refractions but it was deteriorating. She had several headaches thus Consult.
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Case 3 PE: Noted malar rash and skin hyperpigmentations
Joint swelling , grade II edema, Labs: Urinalysis : ++Protein, RBC TNTC Creatine: Normal C3 low, CBC : anemia
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SYSTEMIC LUPUS ERYTHEMATOSUS
11 criteria and must fulfill 4 out of the eleven criteria to dignose Mediated by immune complexes – involving t and b CLASS is 5 but newest is 6 Class IV: is the most common yet severe form of nephropathy diffuse proliferattive GN
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Systemic Lupus Erythematosus
Adolescent female Renal disease in % Severity of renal involvement gives the prognosis ANA screening, DsDNA more specific,low C3 TX: prednisone, cyclophosphamide and rituximab
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HENOCH SCHOENLEIN PURPURA
SMALL VESSEL vasculitis characterized by: abdominal pain, arthritis and purpura Like Ig A nephropathy In children less than5: ureteritis( loin pain and renal colic ureteral stenosis Renal manif: 12 weeks
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RAPIDLY PROGRESSIVE GN
Crescentric GN Fast deterioration to renal failure May be associated with immune com0lex or not Pulse steroid plus cyclophosphamid
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GOODPASTURE SYNDROM Pulmonary hemorrhage and GN Hemoptysis is common
Progression to ESRD Cyclophosphamide and plasmapharesis
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HEMOLYTIC UREMIC SYNDROME
Most common cause of acute renal failure in the world Microangiopathic hemolytic anemia, uremia and thrombocytopenia E coli 0157:H7 80% diarrhea associated
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HEMOLYTIC UREMIC SYNDROME
<4 years Pallor , irritability, weakness, Supportive therapy, early dialysis, full medical
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MEMBRANOUS GN Most common nephrotic syndrome in adults
Has secondary causes like SLE, HEP B, sarcoidosis,ITP, nueroblastoma, Minimal proliferative changes
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Syndromes in Nephrology
Acute Nephritic Syndrome Nephrotic Syndrome Isolated Urinary Abnormalities Acute Renal Failure Chronic Renal Failure Urinary Tract Infection Renal Tubular Defects Hypertension Urinary Tract Obstruction Urolithiasis
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Proteinuria Definition Among Children
Rate of excretion of protein varies with age and size. Corrected for body surface area
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Clinical feature Periorbital Facial Edema Abdominal Scrotal
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24-hour Urine Protein Excretion in Children of Different Ages
Age Protein Protein Protein Concentration Excretion Excretion (mg/L) (mg/24 hr) (mg/24/m2 BSA) 91 2-4 years 49 71 85 years years 83 63
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24-hour Urine Protein Excretion in Children of Different Ages
Age Protein Protein Protein Concentration Excretion Excretion (mg/L) (mg/24 hr) (mg/24/m2 BSA) Premature (5-30 days) 29 182 32 145 Full Term 2-12 months 38 109
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Proteinuria Definition Among Children Mainly tubular proteinuria
Protein excretion is highest among newborns Mainly tubular proteinuria Immaturity of their renal function
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Excretion Rate Normal Abnormal 4 mg/m2/hr or <100 mg/m2/day
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Excretion Rate Nephrotic Range Proteinuria Adult Normal
> 40 mg/m2 hr Adult Normal <150mg/day
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Mechanisms of Proteinuria
Passage of macromolecules inversely proportional to size Ionic Charges Low molecular weight proteins are freely filtered through the glomerulus
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Proteinuria Why Low Excretion ? Glomeruli restrict filtration of large
serum proteins such as albumin and immunoglobulins
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Proteinuria Why Low Excretion ? Proximal tubule reabsorb most of the
LMW proteins such as Insulin or B microglobulin
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Differences of Pediatric vs Adult Nephrotics
It is MINIMAL CHANGE NEPHROTIC syndrome unless proven other wise If if occurs with in the age range of 3 to 11 may still have good prognosis 80% chance of recovery If steroid sensitive better prognosis If young female adolescents watch out SLE
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Algorithm Describing the Management of Nephrotic/Syndrome in Children/Adolescent
Patient with Clinical and Laboratory features with MCNS Treat with Prednisone 60mg/m2/day (max 80 mg/day) Good Response to therapy with infrequent or no relapse. Continue Prednisone as needed Good Initial Response to but patient has frequent relapses or delayed resistence to steroid No Response to Therapy
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Algorithm Describing the Management of Nephrotic/Syndrome in Children/Adolescent
Good Response to therapy with infrequent or no relapse. Continue Prednisone as needed Good Initial Response to but patient has frequent relapses or delayed resistence to steroid No Response to Therapy Consider Renal biopsy and Define appropriate therapy based on the biopsy Treatment options: pulse IV steroids cyclosporin a levanisolone in addition to prednisolone and cytotoxic agents For Renal Biopsy or begin 2nd line of treatment without biopsy Option#1 Option#2 Give 8-12 wks trial with cytotoxic agent ( cyclo, chlorambucil) No response or persistent relapse of NS
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Syndromes in Nephrology
Acute Nephritic Syndrome Nephrotic Syndrome Isolated Urinary Abnormalities Acute Kidney injury Chronic kidney Injury Uri/inary Tract Infection Renal Tubular Defects Hypertension Urinary Tract Obstruction Urolithiasis
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OLIGURIA Defined is <1 mL/kg/hr (infants <10kg) <0.5 mL/kg/hr (children) <500mL/day (adults) Kher, Makker. Pediatric Nephrology. 1995 A presenting manifestation of ARI however urine output is a poor indicator of renal function. Benfield. Pediatric Nephrology. 2004
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Acute Kidney Injury Defined as a rapid decline in the GFR, resulting in impairment of the excretion of nitrogenous waste product and loss of water and electrolytes regulation as well as the acid base balance Abrupt increase in serum creatinine or abrupt decrease in urine outpur
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Acute Kidney Injury OLIGURIC NON OLIGURIC Acute kidney injury with a urine volume of < 400 ml/m2/day Acute kidney injury where the urine volume is normal
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pRIFLE CRITERIA
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Approach to Oliguria/Azotemia
Suspect Acute Renal INJURY Clinical History Physical Examination Biochemical Profile Urinalysis Urinary indices Obtain Pre-Renal Renal Post-Renal
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INCIDENCE In a six-year review, the PNSP reported that in admissions, 14% (*) were due to renal failure for various causes. *
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CATEGORIES OF ACUTE RENAL FAILURE
AVNER, HARMON, NIAUDET. Pediatric Nephrology. 5th ed
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Kidney function is dependent on
adequacy of blood supply to the kidney (Prerenal) integrity of renal parenchyma (Renal) patency of urinary tract (Postrenal)
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Causes of Renal Failure
PRE-RENAL Decrease Plasma Volume Decrease Cardiac Output Reno-vascular Obstruction Urinary Sodium < 10
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Causes of Renal Failure
POST-RENAL Bladder Outflow Obstruction Urethral Obstruction
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Causes of Renal Failure
Glomerular and Small Vessel Disease HUS Interstitial Nephritides Tubular Lesions Urinary Sodium > 50
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RENAL FAILUREs Sudden onset Higher chance of reversibility No stunting
Acute Renal Failure Chronic RENAL FAILURE Sudden onset Higher chance of reversibility No stunting Slightly anemic Normal sized or enlarged kidneys by ultrasound Heart may not be enlarged Insidous onset Lesser chance of reversibility Severe anemia but with signs of compensation Hypertension Stunted Small kidneys by ultrasound Enlarged heard LV hyperthropy
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Growth Charts: Invaluable Tools
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Syndromes in Nephrology
Acute Nephritic Syndrome Nephrotic Syndrome Isolated Urinary Abnormalities Acute Renal Failure Chronic Renal Failure Urinary Tract Infection Hypertension Urinary Tract Obstruction Urolithiasis
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