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EVALUATION & MANAGEMENT OF PROTEINURIA AND NEPHROTIC SYNDROME IN CHILDREN BY: PROF. ABDULLAH AL SALLOUM Consultant Paediatric Nephrologist Paediatric Department. Proteinuria. Associated with progressive renal disease Involved in the mechanism of renal injury. - PowerPoint PPT Presentation
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EVALUATION & MANAGEMENT OF PROTEINURIA AND NEPHROTIC
SYNDROME IN CHILDREN
BY:
PROF. ABDULLAH AL SALLOUM Consultant Paediatric Nephrologist
Paediatric Department
ProteinuriaProteinuria
Associated with progressive renal disease
Involved in the mechanism of renal injury
Clinical Testing for ProteinuriaClinical Testing for Proteinuria
Urinary dipstickScreening test Color reaction between urinary albumin and
tetrabromphenol blueTrace 15 mg/dl 1 + 30 mg/dl 2 + 100 mg/dl 3 + 300 mg/dl 4 + 2000 mg/dl
Urinary dipstickUrinary dipstick
False-negative Diluted urine
False-positiveAlkaline urine (PH>8.0)Concentrated urine (sp.gravity>1:025)Antiseptic contamination(Chlorhexidine, benzalkonium chloride)After intravenouse radiograph contrast
Alternative Office MethodAlternative Office Method
Sulfosalicylic acide lead to precipitation of proteins including LMW proteins
Quantitative estimate of proteinuria Quantitative estimate of proteinuria 24-hour urine collections Urinary protein/creatinine (pr/cr) ratio
Spot urine specimen First morning specimenNormal values
<0.2 mg protein/mg creatinine in children > 2 years <0.5 mg protein/1 mg creatinine in children 6-24 months old
Protein Handling by the Kidneys in Protein Handling by the Kidneys in Normal ChildrenNormal Children
Normal rate of protein excretion <4mg/m2/hr<100mg/m2/day – 50% Tamm-Horsfall protein
– 30% Albumin
– 20% other protein
• Restricted filtration of largeProteins (albumin & Immunoglubulin)
Proximal tabules reabsorb most of LMW protein (insulin, B2 microglobulin)
Protein Handling in Renal Protein Handling in Renal DisordersDisorders
Excess urinary protein losses 1. Increase permeability of the glomeruli
(glomerular)2. Decrease reabsorption of LMW
proteins by the renal tubules (tubular)
Types of proteinuriaTypes of proteinuria 1. Transient
Fever Stress Dehydration Exercise
2. Orthostatic proteinuria Excess urine protein in upright position but normal during
recumbency School age <1gm/m2/day
3. Persistent proteinuria: Proteinuria of ≥1 + by dipstick in
multiple occasions
Association Between Proteinuria and Association Between Proteinuria and Progressive Renal DamageProgressive Renal Damage
Persistent proteinuria should be viewed as a marker of renal disease and also as a cause of progressive renal injury.
Association Between Proteinuria and Association Between Proteinuria and Cardiovascular DiseaseCardiovascular Disease
Severe persistent proteinuria is a long-term risk factor for atherosclerosis in children
1. Metabolic disturbances associated with proteinuria (hypercholeseterolemia, hypertriglyceridemia and hypercougalability2. Hypertension 3. Renal insufficiency 4. Steroid therapy
Evaluating Children with ProteinuriaEvaluating Children with Proteinuria
[A] First stage Complete history and physical examination (BP) Complete urinanalysisUrindipstick before going to bed and after arise Blood level of Albumin, creatinine, cholesterol,
electrolyte
[B] Second stage Renal ultrasonography Measurement of serum C3, C4, complementAntinuclear antibody Serology for hepatitis B, C, ± HIV
1. Dietary recommendations Chronic renal insufficiency Dietary protein restriction Nephrotic syndrome: avoid an excess of dietary
protein because it may: a. Worsen proteinuria
b. Will not result in a higher serum albumin
c. Recommendation: give recommended daily allowance of protein for age
Nonspecific Treatment Options for Nonspecific Treatment Options for Persistent ProteinuriaPersistent Proteinuria
2. 2. Blood pressure control/inhibition of Blood pressure control/inhibition of angiotensin effectsangiotensin effects
ACE: and angiotensin II receptors blockers
Reduce BP Reduce urinary protein excretion Decrease the risk of renal fibrosis
ACE: are contraindicated during pregnancy
Approach to Proteinuria in Adolescents Approach to Proteinuria in Adolescents with insulin-dependent DMwith insulin-dependent DM
Good glycemic control is the first goal in preventing renal injury
The first sign of renal injury in IDDM is microalbuminuria
Microalbuminuria 20-200 microgram/min/1.73m230-300 mg albumin/g creatinine
Overt proteinuria Albuminuria>200 microgram/minute/1.73m2
Evaluation and Treatment of Evaluation and Treatment of Patients with NSPatients with NS Definition
Heavy proteinuria, hypoalbuminemia Hypercholestremia and edema
Prevalence 2-3 cases per 100,000 children The majority will have steroid responsive
MCNS
Pretreatment Renal Biopsy in NSPretreatment Renal Biopsy in NS
Infantile NS Adolescence Persistent hematuria Hypertension Depressed serum complement Reduced renal function
Clinical Problems Associated with Clinical Problems Associated with Children NSChildren NS
[A] Edema
Gravity dependent Periorbital in the early morning hours then
generalized Severe edema present as ascites,
pleural effusions, scrotal or vulvar edema, skin breakdown.
[B] [B] Electrolyte disturbances in NSElectrolyte disturbances in NS
1. Hyponatremia
↑antidiuretic hormone H2O→ > Na retention
Total body Na > normal
2. Pseudohyponatremia Result from high lipid level
Dependent on lab methodology
3. PseudohypocalcemiaNormal level of free ionized ca
Low level of protein-bound ca
[C][C] InfectionsInfections
1.Varicella
Varicella antibody should be obtained Varicella – zoster immunoglobulin within 72 hours
of exposure Steroid should be tapered to 1 mg/kg/day Acyclorir or valacylovir if varicella does develop
2.2. Other infectionOther infection
Cellulitis 1 peritonitis
The organisms usually Pneumococcus E-coli
Immunization in N.S.Immunization in N.S.
Live viral vaccines should not be given if patient on high dose of steroids
Pneumococcal vaccine is recommended to all NS (off steroids)
Varicella vaccine (varivax) in 2 doses regimen is safe and efficacious
Antibodies to vaccines may fall during relapses (still contravesial)
[D] [D] HyperlipidemiaHyperlipidemia
Transient and severe hypercholesterolemia during relapses
Persist in treatment-resistent NS Atherosclerosis in young NS Dietary modification : limited benefit Cholestyramine is approved in NS
Approaches to treatment of NSApproaches to treatment of NS
[A] Prednisone/prednisolone Mainstay of treatment of NS
Typical protocol:
2 mg/kg/day (60mg/m2/day) (4+4 wks treatment) 4 wks daily steroid 4 wks every other day Recently: 6+6 weeks induce a higher rate of long
remissions than the standard (4+4)
Treatment of Relapses of NSTreatment of Relapses of NS
60-80% of patients will relapsePrednisolone 2mg/kg/day until the patient is
free of proteinuria for 3 days then 4-6 wks of every other day treatment.
Side effects of GlucocorticoidsSide effects of Glucocorticoids
(Must be discussed with the family) Cushingoid habitus Ravenous appetite Behavioral and psychological changes (mood liability) Gastric irritation (including ulcer) Fluid retention Hypertension Steroid-induced bone disease (avascular necrosis, bone demineralization) Decreased immune function Growth retardation Nigh sweats Cataracts Pseudotumor cerebri Steroid-related diabetes
[B] [B] IV Pulse SteroidsIV Pulse Steroids
May give success in steroid-resistant NS High dose IV methylprednisolone
30 mg/kg (max Igm)To be given every other day for 6 doses To continue in tapering regiment for period
up to 18 months.Side Effects
Hypertension Arrhythmias
[C] [C] Cytotoxix DrugsCytotoxix Drugs 1. Cyclophosphamide
Over 12 weeks Total cumulative dose 170 mg/kg Side Effects Bone marrow suppressions
Oligospermia, azoospermia and ovarium fibrosis
(If given close to puberty) Hemorrhagic cystitis Risk of malignancy
2. Chlorambucil May cause seizure
[D] [D] Cyclosporin ACyclosporin ASteroid dependent or resistant NS To be given after renal biopsy Relapses high after withdrawal Side Effects
Hypertension Nephrotoxicity Hyperkalemia Hypomagnesemia Hypertrichosis Gingival hyperplasia
[E] [E] LevamisoleLevamisole
Weak steroid sparing drug Long term use Side Effects
Neutropenia Rash Gastrointestinal disturbances Seizures
Other Practical Aspects of the Other Practical Aspects of the Management of NSManagement of NS
Fluid intake should be limited to double of insensible water loss in severely edematous NS
Combined diuretics and IV albumin can be given in severe edema
Diuretics should not be given in mild edema ACE: should not be given in the initial course of
prednisolone because of the risk of hypotension and thrombosis in the diuretic phase
ACE: can be given to steroid-resistant NS Schooling, activities, diet should be individualized