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PROTEINURIA/NEPHROTIC SYNDROME LEARNING OBJECTIVES: ∙Define proteinuria . ∙Classify proteinuria . ∙Define nephrotic syndrome.

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PROTEINURIA / NEPHROTIC SYNDROME. Prof. Jameela Kari Dr. Khald Khorshied . PROTEINURIA/NEPHROTIC SYNDROME LEARNING OBJECTIVES: ∙Define proteinuria . ∙Classify proteinuria . ∙Define nephrotic syndrome. ∙Recognize the clinical presentation. ∙Discuss the Causes.

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PROTEINURIA / NEPHROTIC SYNDROME

Prof. Jameela Kari

Dr. Khald Khorshied .

PROTEINURIA/NEPHROTIC SYNDROME

LEARNING OBJECTIVES:

∙Define proteinuria.

∙Classify proteinuria.

∙Define nephrotic syndrome.

∙Recognize the clinical presentation.

∙Discuss the Causes.

∙Discuss the investigation.

∙ List the complications.

PROTEINURIA /NEPHROTIC SYNDROME

CONTENTS :

Definition of proteinuria.

Methods of urine collection of urine analysis, 24 hours collection of urine and urine albumin/creatinine ratio.

Steroid Sensitive Nephrotic Syndrome (SSNS) {Investigation, treatment and complications}.

Proteinuria

*Normal range  100mg/m2/day (150mg/day), <4 mg/ m2/hour

*Proteinuria = >4mg/m2/hour

*Nephrotic range  > 1 gm/ m2/day

>40mg / m2/hour

Spot urine for albumin/creatinine ratio (mg:mg)

Normal = <0.2 (0.5 if <2yr) g/g

Nephrotic => 2 g/g

Dipstick test:

negative, trace

1+ (closest to 30 mg/dL)

2+ (closest to 100 mg/dL)

3+ (closest to 300 mg/dL)

4+ (greater than 2,000 mg/dL)

Conditions Particularly Associated with Proteinuria

NON-PATHOLOGIC PROTEINURIA

PATHOLOGIC PROTEINURIA

NON-PATHOLOGIC PROTEINURIA

Excessive protein excretion, not as result of a disease state

Generally less than 1,000 mg/24 hr (1.00 g/24 hr) and is never associated with edema.

Postural (Orthostatic)Proteinuria: Children with this disorder excrete normal or slightly increased amounts of protein in the supine position. In the upright position, the amount of protein in the urine may increase 10-fold or more

Febrile Proteinuria: does not exceed +2 on the dipstick

Exercise Proteinuria: does not exceed +2 on the dipstick

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PATHOLOGIC PROTEINURIA

  • Tubular Proteinuria:
    • Injury to the proximal tubules results in diminished re-absorptive capacity and the loss of these low molecular weight proteins in the urine; rarely exceeds 1 g/24 hr; not associated with edema.
    • May be associated with other defects of proximal tubular function, such as glucosuria, phosphaturia, bicarbonate wasting, and aminoaciduria (FANCONI)

Glomerular Proteinuria

  • Increased permeability of the glomerular capillary wall
  • Selective (loss of plasma proteins of low molecular weight protein including albumin), primarily in minimal-change nephrosis, or nonselective (loss of albumin and of larger molecular weight proteins such as IgG)
  • Nephrotic range (40mg/m2/hour), non-nephrotic range (4mg/kg/hour)

PERSISTENT ASYMPTOMATIC PROTEINURIA

  • Persists for 3 mo
  • The amount less than 2 g/24 hr; it is never associated with edema.
  • Causes: postural proteinuria, membranous and membrano-proliferative glomerulonephritis, pyelonephritis, hereditary nephritis, developmental anomalies, and "benign" proteinuria.
  • Investigation: urine culture; measurement of creatinine clearance, 24-hr protein excretion, serum albumin, C3 complement levels, and renal ultrasound

Indications for Renal Biopsy

Persistent asymptomatic proteinuria in excess of 1,000 mg/24 hr (1 g/24 hr) or the development of hematuria, hypertension, or diminished renal function.

Approach to proteinuria

  • History and examination
  • Confirm persistent proteinuria
  • 24 hours for protein
  • Fractional urine collection (orthostatic test)
  • U/Es, creatinine clearance (GFR),serum protein and albumin
  • Strept. Serology, C3, ANA
  • US and further imaging
  • ? Renal biopsy

Nephrotic syndrome

  • Definition
  • Types:
    • Idiopathic 90%
      • Minimal change (85%)
      • Mesangial proliferation (5%) and membraneous nephropathy.
      • Focal segmental glumerulosclerosis (10%)
    • Secondary 10%

Minimal Change NS

  • More common in boys
  • 2-10years old
  • 2-3/100,000 (UK)
  • oedema "pitting"
  • Weight gain, ascites and/or pleural effusions
  • Declining urine output

Pathophysiology

 glomerular permeability to protein

Edema

 lipids

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Diagnosis

Urine analysis….protein > 2+

24 hours protein excretion

Urine albumin/creatinine (>2 g/g)

Serum Albumin < 25 gm/dl

Increased cholesterol and triglycerides

Other investigations: complements (C3 &C4), U&E, lipids, HepBSAg, ANA & ASO titer.

Nephritis

  • Frank haematuria
  • Hypertension
  • Low complement
  • Non-nephrotic range proteinuria

Nephrotic

  • None or microscopic haematuria (20%)
  • Normal BP
  • Normal complement
  • Nephrotic range proteinuria
  • Complications
  • Hypovalaemia
  • Infection: peritonitis (pneumococci)
  • Thrombosis
  • Management
  • Edema no added salt diet, IV albumin and diuretics if severe (physical discomfort)
  • Hypovolaemia IV albumin
  • STEROID: Prednisolone 60mg/m2/day (Max. 60 mg) or 2mg/kg/day(divided doses) for 6 weeks followed by 40mg/m2 (max. 40 mg)on alternate days for 6weeks. Then taper the dose by 10 mg/m2 on alternate days every 8 days.
  • Excellent prognosis  90% response to steroid
  • If no response after 4 weeks  biopsy
  • If frequent relapsers (2 or more in 6 months) or steroid dependent…consider:
      • Levamisole
      • Cyclophosphamide
      • Cyclosporin /Tacrolimus
  • If steroid resistant  biopsy and referral to pediatric nephrology
urinary tract infections uti
URINARY TRACT INFECTIONS (UTI).

LEARNING OBJECTIVES:

  • Define UTI.
  • List the risk factors.
  • List the clinical presentation
  • Recognize how to investigate radiologically.
  • Outline the radiological investigations required.
  • Discuss the management.

CONTENTS:

  • UTI (Presentation at different ages, investigation and treatment).
  • Congenital renal tract abnormalities (V-U-Reflux).

…………………………………………………………….

  • Incidence: 0.1% of newborn infants
  • Clinical manifestations are vague and nonspecific (failure to thrive, weight loss, poor feeding, jaundice, diarrhea, and fever)
  • 75% are caused by Escherichia coli, but other enterobacteria and gram-positive cocci are not uncommon.
  • Diagnosis is confirmed by a positive bladder urine culture obtained either by suprapubic aspiration, catheterization or clean catch
  • 25-30% associated with V-U reflux
  • US kidneys, DMSA scan, micturating cystogram
  • TREATMENT. Parenteral antibiotics, usually including an aminoglycoside and ampicillin or a cephalosporin
  • Prophylactic antibiotics until V-U reflux is excluded
  • Older children: girls 6-8%, boys 2% circumcised (0.2%)
  • more specific symptoms (dysuria, abdominal pain, haematuria)
  • Diagnosis: urine culture and colony count: midstream (clean-catch), catheterization, or suprapubic puncture
  • Treatment: antibiotics (Trimethoprium), aminoglycoside and ampicillin or a cephalosporin,
  • 25-30% associated with V-U reflux
  • Reflux will be resolved by 5 years of age in most of the children
  • Recurrent infection leads to renal scars which is permanent  reflux nephropathy (hypertension and impaired renal function)
  • Every child with proved UTI (2nd) needs investigations to exclude V-U reflux and scars.
  • If there is V-U reflux.. Prophylaxis is indicated
  • All ages  US kidneys
  • < 1 year  MCUG to exclude reflux
  • < 5 years  DMSA scan to exclude scars
renal agenesis potter s sequence

Congenital anomalies of renal tract

PUV

Renal agenesis (Potter’s sequence)

Renal dysplasia

Horseshoe kidny

PUJ obstruction

Prune Belly Syndrome

Polycystic kidneys

Ureteric duplication

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