Protenuria

Answer 1

proteinuria is nephrotoxic.

Question 2

persistent

Answer 2

Repeated tests, perhaps up to 3 times over a period of several weeks, should be performed to verify that a single instance of proteinuria is, in fact, persistent.
First-voided morning specimens should be used to establish if proteinuria is persistent.

Question 3

Dipstick

Answer 3

trace : < 20 mg/dl 1+ : 30 mg/dl 2+ : 100 mg/dl 3+ : 300 mg/dl 4+ : > 2000 mg/dl

Dipstick primarily detect albuminuria, less
sensitive for other forms of proteinuria (low molecular wt. proteins, Bence Jone protein,
gamma globulins)
False –ve : Dilute urine SG<1.005 low mwt. proteinuria
False +ve :
Highly concentrated urine Gross hematuria, pyuria, bacteruria Contamination with antiseptic agents (Chlorheridine) Urine pH >7.0 Alkali urin
Phenazopyridine therapy

Question 4

dipstick is positive for protein

Answer 4

A dipstick is positive for protein if : ≥ 1+ with SG ≤ 1.015 ≥ 2+ with SG > 1.015
 U prot./Ucr ratio (mg/mg) First morning voided urine
Child < 2years - <0.5 (N) ≥ 2 years <0.2mg/mg (N)
Nephrotic range proteinuria - >2
Times 24 hr. urine collection Child : <150 mg/day ≤ 4 mg/m2/hr (N) 4 – 40 mg/m2/hr Abnormal >40 mg/m2/hr Nephrotic range >50mg/kg/day

Question 5

Transient Proteinuria

Answer 5

Transient Proteinuria
Tem. > 38.3C (101 F) Exercise, dehydration Cold exposure, heart failure Seizures, stress
Usually doesn’t exceed 2+ on dipstick No evaluation or therapy is needed

Question 6

Orthostatic (Postural) proteinuria

Answer 6

Most common cause of persistent proteinuria in school
aged children and adolescents
Cause : Unknown
60% of child. with persistent proteinuria usually
asymptomatic (N) or minimally ↑ amounts of protein in the supine position
In the uptight posit. : 10-fold : 1g/day No hematuria, HTN, hypoalbuminemia, edema or renal
dysfunction
 Evaluation:
Urine analysis, dipstick for 3 consecutive days Ur prot./Cr ratio

Question 7

Fixed Proteinuria

Answer 7

Fixed Proteinuria
Signifcant proteinuria on a frst morning urine
sample on 3 consecutive days (>+1 on
dipstick, or >0.2 prot/Cr ratio) indicate renal
dis. (Glomerular/Tubular)
Glomerular proteinuria
Renal dis. : ↑ glomerular capillary wall
permeability
Range
: <1gm - >30 gm/day
 Selective: loss of plasma proteins of M. wt.
up to - albumin
 Non-selective: Los of albumin and larger M.
wt. protein e.g. IgG

Question 8

Glomerular proteinuria

Answer 8

Glomerular proteinuria
Causes : Isolated proteinuria FSGS, MPGN, MGN, diabetic nephropathy, SC nephropathy Proteinuria as a feature : PSGN, IgA
nephropathy, HSPN, LN, alport syndrome RENAL BIOPSY – Often needed

Question 9

Tubular proteinuria

Electrophoresis of the urine

Answer 9

Tubular proteinuria
Low grade fxed proteinuria Prot./Cr ratio <1.0 Low molecular wt. proteins : Usually reabsorbed by prox. tubules Usually symptomatic unlike glomerular one
Electrophoresis of the urine:
diferentiate between tubular and
glomerular
 Tubular: Little or no albumin
 Glomerular : albumin - major
 Causes :             Cystinosis, Wilson dis., Lowe synd., ATN,    renal dysplasia, galactosemia ,polycystic kid. dis.,  Refux nephropathy

Question 10

Nephrotic syndrom

Answer 10

Nephrotic syndrome
Nephrotic syndrome (NS) is the most common glomerulopathy encountered in pediatric practice.
The nephrotic syndrome is caused by renal diseases that increase the permeability across the glomerular fltration barrier.
Primarily a Pediatric disorder 15 times more common in child. >adults.

Question 11

Idiopathic nephrotic syndrome (INS)

Answer 11

Idiopathic nephrotic syndrome (INS) in children is a clinical manifestation of various histopathologic abnormalities of the kidney tuft, including minimal change nephrotic syndrome (MCNS), difuse mesangial hypercellularity(DMH) and focal segmental glomerulosclerosis (FSGS).
MCNS accounts for 89%, DMH for 2%, and FSGS for 9% of INS cases.
87% of children with INS respond to initial corticosteroid therapy .
60% of steroid-sensitive patients relapse, one-third of them relapse frequently or become steroid-dependent .

Steroid-resistant nephrotic children initially diagnosed as MCNS are at the 60% risk of progression to FSGS.

Question 12

Steroid-resistant nephrotic syndrome (SRNS)

Answer 12

Steroid-resistant nephrotic syndrome (SRNS) is the most common glomerular disease in children that progresses to end-stage renal failure .
After a follow-up of 10 years, end-stage renal failure develops in 30–40% of children with SRNS .

Question 13

Nephrotic syndrome

Answer 13

90% : Idiopathic
 Minimal change (85%)
 Mesangial proliferative. (5%)
 FSGS (10%)
10% - Secondary to systemic or glomerular dis.
Membranous
Membranoproliferative GN

Pathophysiology
↑ permeability of glomerular
capillary
Loss of –ve charged glycoproteins
Edema : ↓ oncotic pressure
Hyper lipidemia
↑ hepatic protein synthesis ↓ lipid catabolism

Question 14

Idiopathic Nephrotic Syndrome

2-6 y

Answer 14

Idiopathic Nephrotic Syndrome
90% of children Pathophysiology
MCNs : 85% pf cases
>95% respond to steroid therapy
Mesangial proliferation :
5% 50% respond to steroid therapy
FSGS : 10%
20% respond to steroid therapy

Idiopathic nephrotic syndrome is the most
common form of childhood nephrotic syndrome, representing more than 90 percent of cases between 1 and 10 years of age and 50 percent after 10 years of age

Idiopathic Nephrotic Syndrome
Age younger than six years of age Absence of hypertension Absence of hematuria by Addis count Normal complement levels Normal renal function
Idiopathic Nephrotic Syndrome
CF : M > F 2:1 2 – 6 years
Edema, Anorexia, Irritability, Abd. pain,diarrhea
HTN, gross hematuria= uncommon

Question 15

Idiopathic Nephrotic Syndrome

DD/marked edema:

Answer 15

Idiopathic Nephrotic Syndrome

DD/marked edema: Protein-losing enteropathy Hepatic failure, CHF Acute or chronic GN, Protein malnutrition

Question 16

Diagnosis of NS

Answer 16

Diagnosis
Urine analysis:
3+ or 4+ proteinuria Microscopic hematuria : 20%
Protein/Cr ratio >2 24 Uprot.: 3.5 g/day – Adults >40 mg/m2/hr – Child.
Serum creatinine Serum albumin Serum cholesterol, TG C3, C4 Renal biopsy :

normal complement levels, negative antinuclear antibody (ANA) fndings, negative viral screens
(ie, HIV, hepatitis B and C).
no family history of kidney disease may be considered for steroid treatment prior to kidney biopsy.

Question 17

Treatment of nephrotic syndrome

Answer 17

Treatment
Treatment of edema
Prednisone (First episode)
Treatment of nephrotic syndrome
A  trial  of  corticosteroids  is  the  frst  step  in treatment  of  idiopathic  nephrotic  syndrome (INS)  in  which  kidney  biopsy  is  not  initially indicated.

Question 18

Kidney biopsy

Answer 18

Kidney biopsy
younger than 1 year or older than 8 years
recurrent gross hematuria
relevant family history of kidney disease
symptoms of systemic disease, positive viral screens

Question 19

Corticosteroid (steroid) treatment (MCNS, INS that does not require initial biopsy)

Answer 19

Corticosteroid (steroid) treatment (MCNS, INS that does not require initial biopsy)
Induction therapy
Exclude active infection or other
contraindications prior to steroid therapy
oral prednisone or prednisolone at 60 mg/m2/d
(2 mg/kg/d), with a maximum of 60 mg, daily for
6 weeks
Gipson DS, Massengill SF, Yao et al.
Pediatrics.Aug2009;124(2):747- 57.
Maintenance therapy
oral prednisone or prednisolone at 40 mg/m2 (or 1.5 mg/kg), with a maximum of 40 mg, given as a single dose on alternate days for 6 weeks

Question 20

Diet in NS

Answer 20

Diet
A sodium restricted diet . Thereafter, a normal diet can be followed. During severe edema, careful and modest fuid restriction
Protein restriction is not indicated, except in cases of acute or chronic kidney failure when severe azotemia is present and, even then, protein restriction should be done carefully as to avoid impaired somatic growth.

Question 21

Outcome NS

Answer 21

Outcome
Relapse  is  defned  as  the  recurrence  of  oedema  and  proteinuria with urinary dipstick proteinuria of ≥2+.
Frequent  relapses  (FR)  were  defned  as  the  recurrence  of proteinuria at least twice within 6 months or at least four times within the last 12 months.
Steroid  dependent  (SD)  when  they  relapsed  while  still  on  a weaning  course  of  prednisolone,  or  within  14  days  of discontinuing steroids.
Steroid resistance (SR)denotes a lack of remission after 4 weeks of corticosteroid therapy

Question 22

Congenital NS

Infantile nephrotic syndrome:

Answer 22

Congenital NS
presentation of nephrotic syndrome during the irst 3 months of life (often present before or at birth). Finnish type Cong. Infection HIV, HBV Difuse mesangial sclerosis
Denys- Drash syndrome
Infantile nephrotic syndrome:
presentation of nephrotic syndrome between 3 and 12 months of age.

Question 23

ADHD

Answer 23

ADHD • Symptoms must be in two settings: Home and

school for more than 6 months • 6 out of 9 symptoms • Present before age 12