Paediatrics Marrow Pg 161-170 (Genitourinary System )
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Paediatrics Marrow Pg 161-170 (Genitourinary System )

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Questions and Answers

What is the recommended GFR level to initiate Kidney Replacement Therapy (KRT)?

  • 20 ml/min/1.73 m²
  • 15 ml/min/1.73 m²
  • 10 ml/min/1.73 m²
  • 12 ml/min/1.73 m² (correct)

    • Hemodialysis is typically done at home for patients.

    False

    What is the advantage of peritoneal dialysis compared to hemodialysis?

    Better hypertension control.

    The definitive treatment for kidney failure after finding a donor is __________.

    <p>renal transplantation</p> Signup and view all the answers

    Match the type of dialysis with its key characteristics:

    <p>Peritoneal dialysis = Home set-up, approx 12 hr/day, better HTN control Hemodialysis = Hospital set-up, approx 3 times/week for 3-4 hrs each</p> Signup and view all the answers

    What is a primary characteristic of Vesico-Ureteric Reflux (VUR)?

    <p>Reflux of urine from the bladder into the ureter</p> Signup and view all the answers

    Vesico-Ureteric Reflux (VUR) is commonly caused by infections in children.

    <p>False</p> Signup and view all the answers

    What is the primary diagnostic tool used to confirm Vesico-Ureteric Reflux (VUR)?

    <p>micturating cysto urethrogram (MCV)</p> Signup and view all the answers

    What is the minimum absolute increase in serum creatinine that indicates Acute Kidney Injury (AKI)?

    <p>0.3 mg/dl</p> Signup and view all the answers

    Decreased urine output is defined as less than 0.5 ml/kg/hr for over 6 hours in AKI.

    <p>True</p> Signup and view all the answers

    Vesico-Ureteric Reflux may lead to __________ of the kidneys due to recurrent urinary tract infections.

    <p>scarring</p> Signup and view all the answers

    What are the two main characteristics used to define acute kidney injury?

    <p>Increased serum creatinine and decreased urine output.</p> Signup and view all the answers

    Match the following features of VUR with their correct descriptions:

    <p>Autosomal dominant = Screen siblings for VUR CKD = Chronic Kidney Disease from bilateral VUR Hypertension = Possible complication of bilateral VUR Reflux nephropathy = Renal damage due to reflux</p> Signup and view all the answers

    An absolute serum creatinine level of _____ mg/dl or greater indicates a significant decline in kidney function.

    <p>4</p> Signup and view all the answers

    Match the following stages of AKI with their corresponding serum creatinine increases:

    <p>Stage 1 = ↑ of ≥ 0.3 mg/dl over 48 hrs or 1.5 to 2 fold from baseline Stage 2 = 2 to 3 fold increase from baseline Stage 3 = Absolute serum creatinine ≥ 4 mg/dl or patient undergoing dialysis</p> Signup and view all the answers

    What is the mode of inheritance of Liddle Syndrome?

    <p>Autosomal dominant</p> Signup and view all the answers

    Liddle Syndrome is caused by a loss of function mutation in the SCNN1 gene.

    <p>False</p> Signup and view all the answers

    What is the result of ENAC overactivity in Liddle Syndrome?

    <p>Increased sodium reabsorption and subsequent hypertension.</p> Signup and view all the answers

    In Liddle Syndrome, the primary electrolyte imbalance seen is __________.

    <p>hypokalemia</p> Signup and view all the answers

    Match the following features with their related conditions:

    <p>Metabolic alkalosis = Liddle Syndrome Hypokalemic metabolic alkalosis = Bartter Syndrome Metabolic acidosis = Gitelman Syndrome Hypermagnesemia = Not specifically associated with any</p> Signup and view all the answers

    Which of the following is a consequence of ENAC overexpression in Liddle Syndrome?

    <p>Hypertension</p> Signup and view all the answers

    Inhibition of aldosterone secretion is a feature of Liddle Syndrome.

    <p>True</p> Signup and view all the answers

    Name one type of evaluation associated with Liddle Syndrome.

    <p>Hypokalemic metabolic alkalosis.</p> Signup and view all the answers

    What is the primary mode of inheritance of Gitelman syndrome?

    <p>Autosomal Recessive</p> Signup and view all the answers

    Gitelman syndrome is characterized by hyperkalemia.

    <p>False</p> Signup and view all the answers

    Which region of the nephron is primarily affected in Gitelman syndrome?

    <p>Distal Convoluted Tubule (DCT)</p> Signup and view all the answers

    Gitelman syndrome results in excretion of __________ and __________ in urine, leading to metabolic alkalosis.

    <p>Na+</p> Signup and view all the answers

    Match the following consequences of Gitelman syndrome with their descriptions:

    <p>Hypokalemia = Low potassium levels in the blood Hypomagnesemia = Low magnesium levels in the blood Hypochloremic = Low chloride levels in the blood Metabolic alkalosis = Increased blood pH due to bicarbonate retention</p> Signup and view all the answers

    What is the typical pH level of urine?

    <p>Acidic</p> Signup and view all the answers

    Urine is typically neutral in pH.

    <p>False</p> Signup and view all the answers

    What is the primary characteristic of urine concerning its pH level?

    <p>Acidic</p> Signup and view all the answers

    The pH of urine is generally considered to be __________.

    <p>acidic</p> Signup and view all the answers

    Match the following urine properties with their characteristics:

    <p>Acidic = Prevents bacterial growth Basic = Promotes UTIs Neutral = Rarely occurs in human urine Variable = Depends on diet and hydration</p> Signup and view all the answers

    Which of the following is a clinical presentation of inherited tubular disorders?

    <p>Polyuria</p> Signup and view all the answers

    Electrolyte imbalances are not associated with inherited tubular disorders.

    <p>False</p> Signup and view all the answers

    What is the primary defect seen in Fanconi syndrome?

    <p>Type II Renal Tubular Acidosis (RTA)</p> Signup and view all the answers

    The reabsorption of bicarbonate, glucose, and amino acids primarily occurs in the ________.

    <p>proximal convoluted tubule (PCT)</p> Signup and view all the answers

    Match the inherited tubular disorders with their characteristics:

    <p>Fanconi syndrome = Type II Renal Tubular Acidosis Bartter syndrome = Electrolyte imbalances with hypokalemia Gitelman syndrome = Electrolyte imbalances with magnesium wasting Liddle syndrome = Hypertension due to increased sodium reabsorption</p> Signup and view all the answers

    What is the most common presentation of D+ HUS?

    <p>Diarrhea</p> Signup and view all the answers

    The Coombs' test is typically positive in cases of D+ HUS.

    <p>False</p> Signup and view all the answers

    What are schistocytes, and where are they typically found?

    <p>Lysed red blood cells found in the peripheral smear.</p> Signup and view all the answers

    The duration of symptoms in D+ HUS typically lasts for __________ days.

    <p>5-10</p> Signup and view all the answers

    Match the following stages of Chronic Kidney Disease (CKD) with their corresponding GFR values:

    <p>Stage 1 = GFR &gt; 90 Stage 2 = GFR 60-89 Stage 3 = GFR 30-59 Stage 4 = GFR 15-30 Stage 5 = GFR &lt; 15</p> Signup and view all the answers

    Which of the following is a common cause of obstructive uropathy?

    <p>Stones</p> Signup and view all the answers

    Reflex nephropathy causes scars in the kidneys.

    <p>True</p> Signup and view all the answers

    What is the recommended management for short stature in patients with renal complications?

    <p>Recombinant growth hormone (rGH)</p> Signup and view all the answers

    The primary management for anemia in patients with GFR less than 30 ml/min/1.73 m² is __________.

    <p>Darbopoetin</p> Signup and view all the answers

    Match the following management strategies with their corresponding complications:

    <p>Anemia = Darbopoetin injection Mineral bone disease = Calcitriol supplementation Short stature = Recombinant growth hormone Hypertension = Lifestyle management</p> Signup and view all the answers

    Which of the following is a pre-renal cause of Acute Kidney Injury (AKI)?

    <p>Dehydration</p> Signup and view all the answers

    Hemolytic Uremic Syndrome (HUS) can be classified into two types: D+ and D-.

    <p>True</p> Signup and view all the answers

    What is the primary goal in the management of pre-renal AKI?

    <p>Restore circulating blood volume</p> Signup and view all the answers

    In the case of hyperkalemia, _________ can be used to shift potassium intracellularly.

    <p>insulin</p> Signup and view all the answers

    Match the complications of AKI with their management:

    <p>Anemia = Packed Red Blood Cell transfusion Metabolic acidosis = IV sodium bicarbonate Hyperkalemia = Dialysis if refractory Hyponatremia = Fluid management</p> Signup and view all the answers

    Study Notes

    Kidney Replacement Therapy (KRT)

    • For patients with End Stage Kidney Disease (ESKD).
    • Starts when Glomerular Filtration Rate (GFR) is below 12 ml/min/1.73 m².
    • Two main types:
      • Dialysis:
        • Peritoneal dialysis: Done at home, involves fluid exchange in the peritoneal cavity.
        • Hemodialysis: Done in hospital, involves filtering blood through a machine.
      • Renal transplantation: The preferred treatment, involves receiving a kidney transplant.

    Vesico-Ureteric Reflux (VUR)

    • Backflow of urine from the bladder to the ureters.
    • Usually present at birth.
    • Can cause scarring of the kidneys and lead to CKD.
    • Diagnosed using micturating cysto urethrogram (MCU), also known as voiding cysto urethrogram (VCUG).

    Acute Kidney Injury (AKI)

    • Sudden decrease in kidney function.
    • Key features:
      • Increase in serum creatinine levels, either absolute or relative.
      • Decreased urine output.
      • Staged based on severity of creatinine elevation and urine output.

    Inherited Tubular Disorders (ITDs)

    • Group of disorders affecting the kidneys' ability to reabsorb water, electrolytes, and nutrients.
    • Common symptoms:
      • Polyuria (excess urine production)
      • Electrolyte imbalances
      • Failure to thrive
    • Some common ITDs:
      • Fanconi syndrome
      • Bartter syndrome
      • Gitelman syndrome
      • Liddle syndrome

    Fanconi Syndrome

    • Generalized proximal tubule dysfunction.
    • Key features:
      • Impaired bicarbonate reabsorption leading to metabolic acidosis with normal anion gap.
      • Glycosuria (glucose in urine)
      • Aminoaciduria (amino acids in urine)
      • Phosphaturia (phosphate in urine)

    D+ HUS

    • Hemolytic Uremic Syndrome associated with diarrhea.
    • Caused by Shiga-toxin producing bacteria like Shigella dysenteriae or Enterohemorrhagic E.coli (EHEC).
    • Key features:
      • Most common age group: under 4 years old.
      • Usually presents with diarrhea.
      • Symptoms: pallor, weakness, oliguria (reduced urine output).
    • Often self-resolving, but supportive care and antibiotics are needed. Dialysis may be required in severe cases.

    Chronic Kidney Disease (CKD)

    • Progressive decline in kidney function over time.
    • Staged based on GFR and other factors.
    • Management focuses on slowing the progression of CKD and treating complications.

    Genitourinary System

    • The system responsible for the production, storage, and elimination of urine.
    • Includes kidneys, ureters, bladder, and urethra.

    Etiology of Chronic Kidney Disease (CKD)

    • Variety of causes:
      • Active space (structural):
        • Obstructive uropathy: blockage in the urinary tract.
        • Reflex nephropathy: damage caused by vesicoureteric reflux.
      • Chronic glomerulonephritis: inflammation of the filtering units of the kidneys.
      • Congenital anomalies: birth defects in the urinary system.

    Management of Chronic Kidney Disease (CKD)

    • Focuses on addressing the cause and managing complications:
      • Surgical correction: for structural anomalies.
      • Medical management for irreversible damage.
      • Nutritional guidance: No protein restrictions, but emphasis on polyunsaturated fatty acids (PUFAs) and complex carbohydrates to promote growth.

    Complications of Chronic Kidney Disease (CKD)

    • Common complications and their management:
      • Anemia:
        • Cause: reduced erythropoietin production.
        • Management: Darbopoetin (recombinant erythropoietin).
      • Mineral Bone Disease:
        • Cause: imbalance of calcium, phosphorus, and Vitamin D.
        • Management: Calcitriol supplementation, calcium supplementation, phosphate binders (calcium-based or non-calcium based).
      • Short stature:
        • Management: Recombinant growth hormone (rGH).
      • Hypertension (HTN):
        • Management: Lifestyle modifications, ACE inhibitors, ARBs.

    Acute Kidney Injury (AKI)

    • Etiology of AKI:
      • Pre-renal causes (most common): Reduced blood flow to the kidneys.
      • Renal causes: Direct damage to the kidney tissues.
      • Post-renal causes: Blockage in the urinary tract.

    Management of AKI

    • Focuses on identifying the cause and providing appropriate treatment:
      • Pre-renal AKI: Fluid resuscitation
      • Renal AKI: Fluid management and preventing further kidney injury.

    Complications of AKI

    • Include:
      • Anemia: Managed with PRBC transfusion if needed.
      • Metabolic acidosis: Managed with IV sodium bicarbonate.
      • Hyponatremia: May require fluid management.
      • Hyperkalemia: Managed with fluid management, K+ binding resins, or dialysis in refractory cases.

    Hemolytic Uremic Syndrome (HUS)

    • Two main types:
      • D+ HUS: Associated with diarrhea, caused by Shiga-toxin producing bacteria.
      • D- HUS: Not associated with diarrhea, caused by a defect in the alternate complement pathway.

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    Test your knowledge on crucial kidney health topics such as Kidney Replacement Therapy, Vesico-Ureteric Reflux, and Acute Kidney Injury. This quiz covers definitions, diagnostics, and treatment options that are essential for understanding these conditions. Perfect for healthcare students and professionals alike.

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