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COH – PEDIATRIC SYNOPSIS OF GENITO URINARY DISORDERS

GENITO URINARY DISORDERS

Vesicoureteral Reflux (VUR)

Definition:

Vesicoureteral reflux (VUR) is a retrograde flow of urine from the bladder into the ureters and sometimes into the kidneys, leading to recurrent urinary tract infections (UTIs) and kidney damage.

Types:

  1. Primary VUR – Congenital incompetence of vesicoureteral junction
  2. Secondary VUR – Due to bladder dysfunction (e.g., neurogenic bladder, posterior urethral valves)

Signs & Symptoms:

  • Recurrent febrile UTIs
  • Dysuria (painful urination)
  • Abdominal or flank pain
  • Enuresis (bedwetting) in older children
  • Hypertension (chronic cases due to kidney damage)

Complications:

  • Recurrent pyelonephritis
  • Renal scarring → Chronic kidney disease (CKD)
  • Hypertension

Diagnosis:

  • Voiding Cystourethrogram (VCUG) – Gold standard
  • Renal ultrasound – Detects hydronephrosis
  • DMSA (Dimercaptosuccinic Acid) Scan – Detects renal scarring

Management:

  • Antibiotic prophylaxis (for mild VUR)
  • Surgical correction (Ureteral reimplantation for severe cases)
  • Behavioral therapy (Timed voiding, Double voiding)

Posterior Urethral Valves (PUV)

Definition:

Posterior urethral valves are congenital obstructive membranes in the male posterior urethra, leading to bladder outlet obstruction.

Signs & Symptoms:

  • Antenatal hydronephrosis (diagnosed on fetal ultrasound)
  • Weak urinary stream
  • Urinary retention or difficulty voiding
  • Recurrent UTIs
  • Failure to thrive

Complications:

  • Bladder dysfunction (neurogenic bladder)
  • Hydronephrosis → Chronic kidney disease (CKD)

Diagnosis:

  • VCUG – Shows dilated posterior urethra (keyhole sign)
  • Renal ultrasound – Shows hydronephrosis

Management:

  • Initial catheterization (to relieve obstruction)
  • Endoscopic ablation of the valves
  • Vesicostomy (in severe cases for bladder drainage)

Wilms Tumor (Nephroblastoma)

Definition:

Wilms tumor is a malignant renal tumor of childhood, originating from metanephric blastema.

Associated Syndromes:

  • WAGR Syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, Retardation)
  • Beckwith-Wiedemann Syndrome (Macrosomia, Organomegaly, Hemihypertrophy)

Signs & Symptoms:

  • Asymptomatic abdominal mass (most common presentation)
  • Hematuria (blood in urine)
  • Hypertension (due to renin secretion by tumor)
  • Abdominal pain

Complications:

  • Metastasis (most commonly to lungs and liver)
  • Hypertension and renal dysfunction

Diagnosis:

  • Abdominal ultrasound – Initial imaging
  • CT/MRI abdomen – Confirms tumor extent
  • Biopsy (not always needed due to risk of tumor seeding)

Management:

  • Nephrectomy (surgical removal of the kidney)
  • Chemotherapy (Vincristine, Actinomycin D, Doxorubicin for advanced cases)
  • Radiation therapy (for stage III or IV cases)

Nephrotic Syndrome

Definition:

Nephrotic syndrome is a glomerular disorder characterized by massive proteinuria (>3.5 g/day), hypoalbuminemia, edema, and hyperlipidemia.

Types:

  1. Minimal Change Disease (Most common in children)
  2. Focal Segmental Glomerulosclerosis (FSGS)
  3. Membranous Nephropathy

Signs & Symptoms:

  • Generalized edema (periorbital, pedal, and ascites)
  • Frothy urine (due to protein loss)
  • Hypercoagulability (due to loss of antithrombin III)
  • Infections (due to loss of immunoglobulins)

Complications:

  • Thrombosis (Renal vein thrombosis, DVT, PE)
  • Infections (Peritonitis, Sepsis)
  • Acute kidney injury (AKI)

Diagnosis:

  • Urine dipstick – Heavy proteinuria
  • Serum albumin <2.5 g/dL
  • Lipid profile – Hyperlipidemia
  • Renal biopsy (if atypical presentation)

Management:

  • Corticosteroids (Prednisolone 2 mg/kg/day for Minimal Change Disease)
  • Diuretics (Furosemide for severe edema)
  • ACE inhibitors (to reduce proteinuria)

Hemolytic Uremic Syndrome (HUS)

Definition:

Hemolytic Uremic Syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, commonly caused by Shiga toxin-producing E. coli (STEC) O157:H7 infection.

Causes:

  • Infectious (Most common) – E. coli O157:H7, Shigella dysenteriae
  • Non-infectious – Complement disorders, Drug-induced

Signs & Symptoms:

  • Bloody diarrhea (preceding symptom in STEC-HUS)
  • Oliguria (low urine output), Hematuria
  • Pallor (due to hemolytic anemia)
  • Petechiae and bruising (due to thrombocytopenia)
  • Hypertension (due to kidney dysfunction)

Complications:

  • Acute kidney injury (AKI) → Dialysis requirement
  • Seizures (due to CNS involvement)
  • Hypertension and chronic kidney disease (CKD)

Diagnosis:

  • Peripheral smear – Schistocytes (fragmented RBCs)
  • Serum creatinine – Elevated (kidney injury)
  • Stool culture – Detects E. coli O157:H7

Management:

  • Supportive therapy (IV fluids, Electrolyte management)
  • Avoid antibiotics (May worsen Shiga toxin release)
  • Dialysis (if severe AKI)
  • Plasma exchange (if atypical HUS due to complement disorder)

Key Points for Competitive Exams

✔️ Most common cause of nephrotic syndrome in children? – Minimal Change Disease
✔️ Most common abdominal tumor in children? – Wilms Tumor
✔️ Gold standard test for VUR? – Voiding Cystourethrogram (VCUG)
✔️ Most common cause of congenital urinary obstruction in males? – Posterior Urethral Valves (PUV)
✔️ Triad of Hemolytic Uremic Syndrome (HUS)? – Hemolytic anemia, Thrombocytopenia, AKI
✔️ Keyhole sign on VCUG indicates? – Posterior Urethral Valves
✔️ Gold standard test for Wilms tumor? – CT Abdomen
✔️ Most common glomerular disease causing chronic kidney disease? – Focal Segmental Glomerulosclerosis (FSGS)

Nephrotic Syndrome

Definition:

Nephrotic syndrome is a glomerular disorder characterized by massive proteinuria (>3.5 g/day), hypoalbuminemia, edema, and hyperlipidemia.

Types:

  1. Minimal Change Disease (Most common in children, steroid-responsive)
  2. Focal Segmental Glomerulosclerosis (FSGS, steroid-resistant)
  3. Membranous Nephropathy (Common in adults, autoimmune-related)

Causes:

  • Primary (Idiopathic) Nephrotic Syndrome (Minimal Change Disease)
  • Secondary Nephrotic Syndrome
    • Infections: Hepatitis B/C, HIV, Malaria
    • Autoimmune diseases: Systemic Lupus Erythematosus (SLE)
    • Drugs: NSAIDs, Penicillamine

Signs & Symptoms:

  • Generalized edema (Periorbital in the morning, pedal and ascites later)
  • Frothy urine (due to protein loss)
  • Hypercoagulability (risk of thrombosis, renal vein thrombosis)
  • Hypoalbuminemia (causes decreased oncotic pressure and edema)

Complications:

  • Infections (peritonitis, sepsis, pneumonia) due to loss of immunoglobulins
  • Thrombosis (DVT, pulmonary embolism) due to loss of antithrombin III
  • Hypovolemic shock (in severe albumin loss cases)

Diagnosis:

  • Urine dipstick – Heavy proteinuria (3+ or 4+)
  • Serum albumin <2.5 g/dL
  • Lipid profile – Hyperlipidemia (high cholesterol, triglycerides)
  • Renal biopsy (if steroid-resistant or atypical presentation)

Management:

  • Corticosteroids (Prednisolone 2 mg/kg/day for 4-6 weeks)
  • Diuretics (Furosemide for severe edema, cautiously used)
  • ACE inhibitors (to reduce proteinuria)
  • Low salt, high-protein diet

Glomerulonephritis

Definition:

Glomerulonephritis is inflammation of the glomeruli, leading to hematuria, proteinuria, hypertension, and renal impairment.

Types:

  1. Acute Post-Streptococcal Glomerulonephritis (PSGN) (Most common in children)
  2. IgA Nephropathy (Berger’s Disease)
  3. Rapidly Progressive Glomerulonephritis (RPGN, seen in Goodpasture’s Syndrome, Wegener’s Granulomatosis)

Causes:

  • Infections: Group A Beta-hemolytic Streptococcus (PSGN), Viral infections (Hepatitis B/C, HIV)
  • Autoimmune disorders: Lupus Nephritis (SLE), Goodpasture Syndrome
  • Drugs: NSAIDs, Penicillamine

Signs & Symptoms:

  • Hematuria (“Cola-colored” or “tea-colored” urine)
  • Hypertension (due to fluid retention)
  • Oliguria (reduced urine output)
  • Edema (facial, periorbital, and peripheral)

Complications:

  • Acute kidney injury (AKI)
  • Hypertensive encephalopathy
  • Chronic kidney disease (CKD) in progressive cases

Diagnosis:

  • Urinalysis – Hematuria, RBC casts, proteinuria
  • ASO (Anti-streptolysin O) titer – Indicates recent Streptococcal infection (for PSGN)
  • Renal biopsy – In severe or atypical cases

Management:

  • Supportive treatment (Fluid and salt restriction)
  • Antihypertensives (Nifedipine, Labetalol)
  • Diuretics (for volume overload)
  • Antibiotics (Penicillin for PSGN, if infection is still active)

Renal Failure

Definition:

Renal failure is the inability of the kidneys to filter waste, maintain electrolyte balance, and regulate fluid volume, leading to accumulation of toxins and fluid overload.

Types:

  1. Acute Kidney Injury (AKI) – Sudden loss of kidney function
  2. Chronic Kidney Disease (CKD) – Progressive, irreversible kidney damage

Causes of AKI:

  • Prerenal (Decreased blood flow to kidneys) – Dehydration, Hypovolemia, Shock
  • Intrinsic Renal (Direct kidney damage) – Glomerulonephritis, HUS, Nephrotoxins (NSAIDs, Aminoglycosides)
  • Postrenal (Obstruction to urine flow) – Posterior urethral valves, Stones, Tumors

Causes of CKD:

  • Congenital anomalies (PUV, Renal Dysplasia, Reflux Nephropathy)
  • Glomerular diseases (FSGS, IgA Nephropathy)
  • Metabolic disorders (Diabetes, Hypertension)

Signs & Symptoms:

  • Oliguria or Anuria (Low urine output)
  • Fluid overload (Edema, Hypertension, Pulmonary edema)
  • Uremic symptoms (Fatigue, Nausea, Anemia, Pericarditis, Encephalopathy)

Complications:

  • Hyperkalemia → Cardiac arrhythmias
  • Metabolic acidosis
  • Growth retardation (in CKD)
  • End-stage renal disease (ESRD) requiring dialysis

Diagnosis:

  • Serum Creatinine & BUN – Elevated in renal failure
  • Urinalysis – Proteinuria, hematuria, casts
  • Renal ultrasound – To assess kidney structure

Management of AKI:

  • Correct underlying cause (Fluid resuscitation in prerenal AKI, Avoid nephrotoxins)
  • Diuretics (If fluid overload is present)
  • Dialysis (if life-threatening complications occur)

Management of CKD:

  • Dietary modification (Low potassium, Low phosphorus, High calcium diet)
  • Erythropoietin therapy (For anemia)
  • Calcium carbonate (For hyperphosphatemia)
  • Renal transplantation (Definitive treatment for ESRD)

Key Points for Competitive Exams

✔️ Most common cause of nephrotic syndrome in children? – Minimal Change Disease
✔️ Most common cause of glomerulonephritis in children? – Post-Streptococcal Glomerulonephritis (PSGN)
✔️ Gold standard test for nephrotic syndrome? – 24-hour urinary protein (>3.5 g/day)
✔️ Best initial test for acute kidney injury (AKI)? – Serum creatinine and BUN
✔️ Triad of Hemolytic Uremic Syndrome (HUS)? – Hemolytic anemia, Thrombocytopenia, Acute kidney injury
✔️ Gold standard test for CKD progression? – Glomerular Filtration Rate (GFR)
✔️ Main treatment for steroid-resistant nephrotic syndrome? – Immunosuppressants (Cyclophosphamide, Tacrolimus)
✔️ Most common cause of end-stage renal disease (ESRD) in children? – Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)

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