Paediatrics Marrow Pg 151-160 (Genitourinary System )
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Questions and Answers

What is a common complication associated with obstructive uropathy?

  • Decreased kidney size
  • Increased risk of liver disease
  • Ureteral obstruction (correct)
  • Hypertrophy of the bladder

    • PUJ obstruction is usually bilateral in congenital cases.

    False

    What condition might indicate a need for Anderson Hynes Pyeloplasty?

    Abnormal renal function

    A significant symptom of PUJ obstruction in older children is ___________ crisis, characterized by severe abdominal pain.

    <p>Dietl's</p> Signup and view all the answers

    Match the following investigative procedures with their descriptions:

    <p>DTPA scan = Assesses and manages PUJ obstruction; kidney retains tracer Intravenous urography = Contrast is retained in the kidney to visualize obstruction</p> Signup and view all the answers

    What is the recommended dosage of oral Prednisolone for 3 weeks in patients with cardiac problems?

    <p>60 mg/kg/day</p> Signup and view all the answers

    Patients with established Rheumatic Heart Disease should receive prophylaxis until they reach the age of 40.

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

    How long should patients without carditis receive prophylaxis?

    <p>5 years or till age 18, whichever is longer.</p> Signup and view all the answers

    Aspirin should be administered at a dosage of ______ mg/kg/day in 4 divided doses for 10 weeks.

    <p>90-120</p> Signup and view all the answers

    Match the scenarios with their appropriate duration of prophylaxis:

    <p>No carditis = 5 years or till age 18 Carditis (without RHD) = 10 years or till age 25 Established RHD/undergone surgery = Till age 40 or life long</p> Signup and view all the answers

    What is the most common severe cause of obstructive uropathy in male children?

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

    Cloacal Extrophy is a condition observed only in female children.

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

    What is the primary clinical feature of PUv that indicates urinary obstruction?

    <p>Poor urine stream</p> Signup and view all the answers

    The condition where a membrane valve causes obstruction is known as __________.

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

    Match the following conditions with their descriptions:

    <p>PUv = Membrane valve causing obstruction in males Cloacal Extrophy = Defect in the development of the bladder and lower abdominal wall Omphalocele = Abdominal organs protrude through the abdominal wall Ileal Extrophy = Defect in the terminal part of the ileum</p> Signup and view all the answers

    What is a hallmark feature of Nephritic Syndrome?

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

    Increased levels of fibrinogen are associated with a decreased risk of infarctions.

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

    What types of bacterial infections are patients with proteinuria at an increased risk for?

    <p>Encapsulated bacterial infections like Streptococcus pneumoniae and Haemophilus influenzae Type B.</p> Signup and view all the answers

    Post-streptococcal glomerulonephritis can occur ___ to ___ days after an initial Group A Streptococcal infection.

    <p>7, 10</p> Signup and view all the answers

    Match the following clinical features with their associated syndrome:

    <p>Hematuria = Nephritic Syndrome Increased risk of infections = Nephrotic Syndrome Localized edema = Nephritic Syndrome Increased anticoagulant proteins = Nephrotic Syndrome</p> Signup and view all the answers

    What genetic defect is associated with Alport's syndrome?

    <p>COL4A5 gene mutation</p> Signup and view all the answers

    Hematuria is not a clinical feature of Alport's syndrome.

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

    What is the primary management strategy for patients with hereditary nephritis?

    <p>Regular follow up and supportive treatment</p> Signup and view all the answers

    In chronic kidney disease, _______ inhibitors are used to reduce the progression of the disease.

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

    Match the following features with their associated conditions:

    <p>Anterior lenticonus = Alport's syndrome IgA deposits = Mesangial proliferation Chronic nephropathy = ACE inhibitors High-frequency sensorineural hearing loss = Alport's syndrome</p> Signup and view all the answers

    What can result from Vesicoureteric reflux (VUR) in normal kidneys?

    <p>Renal hypertrophy and damage</p> Signup and view all the answers

    Cysts in the kidneys usually increase in size as a child ages.

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

    What gene defect is associated with Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

    <p>PKD1 gene</p> Signup and view all the answers

    The defining characteristic of a horseshoe kidney is the fusion of the lower ______ of the kidney.

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

    Match the following congenital anomalies with their key characteristics:

    <p>Autosomal Recessive Polycystic Kidney Disease = Bilateral condition with multiple cysts Horseshoe Kidney = Fusion of lower poles of kidneys Vesicoureteric reflux = Leads to renal hypertrophy Periportal fibrosis = Hepatic manifestation of ARPKD</p> Signup and view all the answers

    What characterizes massive proteinuria in nephrotic syndrome?

    <p>Excretion &gt; 40 mg/m²/hr</p> Signup and view all the answers

    Hypoalbuminemia in nephrotic syndrome results from decreased oncotic pressure.

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

    Which condition is associated with unilateral renal agenesis in females?

    <p>MRKH syndrome</p> Signup and view all the answers

    Name one condition commonly seen in nephrotic syndrome.

    <p>Minimal change disease</p> Signup and view all the answers

    Bilateral renal agenesis leads to normal amniotic fluid volume during the antenatal period.

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

    The primary corticosteroid used in the management of nephrotic syndrome is __________.

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

    Match the following nephrotic syndrome features with their descriptions:

    <p>Massive proteinuria = Excretion &gt; 40 mg/m²/hr Hypoalbuminemia = Serum albumin &lt; 3 g/dl Anasarca = Generalized edema Hyperlipidemia = Increased lipid production by liver</p> Signup and view all the answers

    What is the most common type of cystic dysplasia in congenital anomalies of the kidney?

    <p>multicystic dysplastic kidney (MCDK)</p> Signup and view all the answers

    The primary consequence of oligohydramnios in bilateral renal agenesis is __________ syndrome.

    <p>Potter's</p> Signup and view all the answers

    Match the following facial features to their descriptions in conditions affecting renal development.

    <p>Prominent epicanthal folds = A distinctive facial feature often seen in congenital renal conditions Low nasal bridge = Commonly associated with various dysplastic kidney syndromes Receding chin = Frequently observed in babies with kidney malformations Depressed nasal bridge = Can indicate severe developmental disturbances in renal health</p> Signup and view all the answers

    What is the most common age group affected by nephrotic and nephritic syndrome?

    <p>5 - 15 years</p> Signup and view all the answers

    Hypertensive encephalopathy is a complication associated with nephrotic and nephritic syndrome.

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

    What is the primary role of Renal biopsy in nephrotic and nephritic syndrome?

    <p>To rule out other causes when low C3 persists beyond 12 weeks.</p> Signup and view all the answers

    The commonest cause of chronic and recurrent nephritis is __________.

    <p>IgA nephropathy</p> Signup and view all the answers

    Match the following clinical features with their corresponding details:

    <p>Hematuria = Presence of blood in urine Oliguria = Reduced urine output Edema = Swelling due to fluid retention Hypertension = Elevated blood pressure</p> Signup and view all the answers

    What is the recommended steroid dosage for frequent relapses during treatment for nephrotic syndrome?

    <p>2 mg/kg/day for daily use</p> Signup and view all the answers

    Steroid-resistant nephrotic syndrome most commonly occurs within the first 3 months of life.

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

    What is the role of the gene NPHS1 in congenital nephrotic syndrome?

    <p>It codes for Nephrin and is inherited in an autosomal recessive manner.</p> Signup and view all the answers

    The gene WT1 is associated with an increased risk of _____ in addition to ambiguous genitalia in males.

    <p>Wilms tumor</p> Signup and view all the answers

    Match the following treatments with their description:

    <p>Levamisole = Immunomodulatory drug Cyclosporin = Calcineurin inhibitor Mycophenolate = Immunomodulatory drug Tacrolimus = Calcineurin inhibitor</p> Signup and view all the answers

    Study Notes

    Anti-inflammatory Medications

    • Steroids: Preferred for cardiac problems. Oral Prednisolone (60mg/kg/day) for 3 weeks, then taper over 9 weeks.
    • Aspirin: 90-120 mg/kg/day, divided into 4 doses, for 10 weeks. Taper over the following 2 weeks.

    Duration of Prophylaxis

    • No Carditis: Prophylaxis for 5 years or until age 18, whichever is longer.
    • Carditis (without Rheumatic Heart Disease): Prophylaxis for 10 years or until age 25, whichever is longer.
    • Established Rheumatic Heart Disease/Undergone Surgery: Prophylaxis until age 40 (ideally lifelong).

    Pathogenesis of Congenital Kidney and Urinary Tract Anomalies

    • 20% to 40% of cases may develop Vesicoureteric reflux (VUR) leading to renal hypertrophy and damage.
    • Cysts can compress the normal kidney structures causing dysfunction. Usually, the left kidney is larger than the right.
    • Cysts can reduce in size spontaneously within 5 to 7 years of age.

    Autosomal Recessive Polycystic Kidney Disease (ARPKD)

    • A bilateral condition affecting both kidneys.
    • Characterized by the presence of multiple cysts.
    • Caused by a defect in the PKD1 gene on chromosome 6p.
    • May include hepatic manifestations such as liver cirrhosis (periportal fibrosis).
    • Common symptoms include bilateral abdominal mass localized to the loin area.

    Diagnosis of Autosomal Recessive Polycystic Kidney Disease

    • Gene testing for the PKHDI gene.
    • Ultrasound (USG): Hyperechoic bright kidneys and a loss of cortico-medullary differentiation.

    Treatment of Autosomal Recessive Polycystic Kidney Disease

    • A combined renal and hepatic transplantation might be required in severe cases.

    Horseshoe Kidney

    • Characterized by fusion of the lower poles of the kidney.
    • A potential genetic link to Turner's syndrome exists (approximately 30%).

    Nephrotic Syndrome

    • Disease can be in remission or relapse (recurrence of proteinuria).
    • Daily Steroids: 2 mg/kg/day.
    • Alternate-day Steroids: 1.5 mg/kg/day for 4 weeks.
    • Relapse during treatment: Relapse within 14 days after treatment.
    • Frequent relapses: 2 or more relapses within 6 months or 3 or more relapses within 1 year.
    • Steroid dependence: Treatment options are available to prevent steroid dependence.

    Steroid-Resistant Nephrotic Syndrome

    • Usually occurs after three months and often involves significant glomerular lesions or congenital nephrotic syndrome.
    • Investigations include renal biopsy, glomerular filtration rate (GFR) estimation, and genetic testing.

    Common Mutations in Congenital Nephrotic Syndrome

    • NPHS1 (Finnish Type): Autosomal recessive inheritance, codes for Nephrin.
    • NPHS2: Autosomal recessive inheritance, codes for Podocin.
    • WT1: Autosomal dominant inheritance, associated with an increased risk of Wilms tumor and ambiguous genitalia in males.

    Management of Steroid-Resistant Nephrotic Syndrome

    • Avoid steroids.
    • Immunomodulatory drugs:
      • Levamisole
      • Mycophenolate
    • Calcineurin inhibitors (immunomodulatory):
      • Cyclosporin
      • Tacrolimus

    Obstructive Uropathy

    • Complications:
      • Ureteral obstruction
      • Hydronephrosis
      • Urinary tract infection (UTI)
      • Stone
    • Increased risk of Wilms' tumor (4-fold).

    Pelviureteric Junction (PUJ) Obstruction

    • Congenital: Usually unilateral (left side more common than right).
    • Antenatal Period: Increased kidney size (hydronephrosis).
    • Postnatal Period:
      • Abdominal mass
      • Stone
      • Urinary tract infection (UTI)
    • Older Child: Dietl's crisis (intermittent episodes of severe abdominal pain).

    Investigations for PUJ Obstruction

    • DTPA scan: Helps in assessing and managing PUJ obstruction. The kidney retains tracer.
    • Intravenous urography: Contrast is retained in the kidney.

    Management of PUJ Obstruction

    • Normal renal function: Follow-up (spontaneous resolution).
    • Abnormal renal function: Anderson Hynes Pyeloplasty (relieving obstruction).

    Posterior Urethral Valve (PUV)

    • Only seen in male children.
    • Most common severe cause of obstructive uropathy.

    Pathophysiology of Posterior Urethral Valve

    • A membrane valve causes obstruction.
    • Located in the prostatic urethra (lower part), just distal to the verumontanum.

    Clinical Features of Posterior Urethral Valve

    • Poor urine stream.
    • Abdominal mass.
    • Urinary tract infection (UTI).
    • Hydronephrosis.

    Investigations for Posterior Urethral Valve

    • Voiding cystourethrogram (m/c):
      • Dilated kidneys
      • Dilated ureters
      • Dilated bladder
      • Dilated posterior urethra
      • Presence of the valve (PUV)

    Management of Posterior Urethral Valve

    • Primary: Catheter to relieve obstruction.
    • Definitive: Endoscopic (cystoscopic) fulguration of the valve.

    Cloacal Extrophy, Omphalocele, and Ileal Extrophy

    • These are various conditions that are commonly discussed in conjunction with genitourinary anomalies.
    • Images are provided to visually depict these conditions but formatting and labeling are not well-suited for a summary.

    Complications of Nephrotic Syndrome

    • Proteinuria:
      • Decreased immunoglobulins/complement proteins leading to impaired opsonization.
      • Increased risk of encapsulated bacterial infections like Streptococcus pneumoniae and Haemophilus influenzae Type B.
      • Increased risk of spontaneous bacterial peritonitis (SBP), cellulitis, and pneumonia due to impaired opsonization.
    • Hypercoagulability:
      • Increased anticoagulant proteins (Protein C/Protein S).
      • Increased production of fibrinogen in the liver.
      • Increased risk of infarctions and associated manifestations (e.g., stroke).

    Nephritic syndrome Glomerulonephritis

    • Clinical Features:
      • Hematuria (hallmark): Cola-colored urine. Urine microscopy findings include >5 RBC/hpf, dysmorphic RBCs, and RBC casts.
      • Oliguria.
      • Localized edema: For example, periorbital edema (puffiness of the eyes).
      • Hypertension (HTN), often mild proteinuria.
    • Common Diseases:
      • Post-streptococcal glomerulonephritis
      • IgA nephropathy
      • Hereditary nephritis

    Post-Streptococcal Glomerulonephritis (PSGN)

    • Post-infectious phenomenon occurring 7-10 days after initial infection.
    • Pathogenesis: Group A β hemolytic streptococci.
    • Initial Infection: Pharyngitis or pyoderma.
    • Lag Period: 1-2 weeks for pharyngitis (M strain), 3-6 weeks for pyoderma (M strain).
    • Immune Complex Formation: Immune complexes are formed.
    • Kidney Deposition: Immune complexes are deposited in the kidney, causing nephritis.

    Investigations for Post-Streptococcal Glomerulonephritis

    • C3 levels: Normal.
    • ASO titre: No increase.
    • Renal biopsy: Mesangial proliferation and IgA deposits.

    Management of Post-Streptococcal Glomerulonephritis

    • Chronic nephropathy (10-20% of cases): Requires constant follow-up.
    • Chronic kidney disease.
    • ACE inhibitors/ARB: Reduce renal progression of disease and risk of CKD.
    • Fish oil (Omega 3 PUFA): Reduces macrophage entry into the mesangium, preventing initiating factors for IgA deposition.

    Hereditary Nephritis (Alport's Syndrome)

    • X-linked dominant inheritance.
    • Defect in the COL4A5 gene (α chain of type IV collagen).

    Clinical Features of Hereditary Nephritis

    • High-frequency sensorineural hearing loss.
    • Hematuria.
    • Triad:
      • Ocular findings:
        • Anterior lenticonus (diagnostic)
        • Macular flecks
        • Corneal erosions

    Investigations for Hereditary Nephritis

    • Gene testing for COL4A5 gene mutation.
    • Renal biopsy.

    Management of Hereditary Nephritis

    • Regular follow-up (many develop end-stage kidney disease by the second or third decade).
    • Supportive treatment.
    • ACE inhibitors to reduce renal progression.

    Renal Agenesis

    • Unilateral Renal Agenesis:
      • Normal contralateral kidney undergoes hypertrophy, resulting in normal overall renal function.
      • Asymptomatic.
      • Incidental finding on USG.
      • Reassurance and avoidance of contact sports (trauma to the unaffected kidney) is recommended.
      • Associated with MRKH (Mayer-Rokitansky-Kuster-Hauser) syndrome in females.
      • Pelvis USG is used to rule out uterine maldevelopment, vaginal aplasia, and confirm normal ovaries.
    • Bilateral Renal Agenesis:
      • Both kidneys are not developed: Severe presentation.
      • Decreased urine output leading to decreased amniotic fluid volume (oligohydramnios) during the antenatal period.
      • Oligohydramnios leads to fetal compression and Potter's syndrome.

    Renal Dysplasia

    • Abnormal differentiation.
    • Most common: Cystic dysplasia, especially multicystic dysplastic kidney (MCDK) caused by abnormal metanephric differentiation.
    • The most common cystic renal malformation.

    Features Associated with Renal Dysplasia

    • Facial Features (Image): Prominent epicanthal folds, low nasal bridge, and receding chin.
    • Body Features (Image): Depressed nasal bridge, receding chin, pulmonary hypoplasia (chest compression, fatal if severe), and limb deformities like CTEV (Congenital Talipes Equinovarus) in some cases.

    Nephrotic Syndrome

    • Features:

      • Massive proteinuria (hallmark): Excretion > 40 mg/m²/hr or > 1 g/m²/day. Spot dipstick: 3+ or 4+. Protein:creatinine (P:C) ratio > 0.2.
      • Hypoalbuminemia (decreased oncotic pressure): Serum albumin < 3 g/dl.
      • Anasarca (generalized edema).
      • Hyperlipidemia (increased production of lipoprotein and lipids by the liver).

    • Pathology:

      • Minimal change disease (MCD): Most common.
      • Significant glomerular lesions:
        • Focal segmental glomerulosclerosis (FSGS)
        • Mesangial proliferative glomerulonephropathy
        • C3 glomerulopathy.

    Management of Nephrotic Syndrome

    • Diet: High protein diet with no added salt.
    • Steroids:
      • Prednisolone 2 mg/kg/day daily (up to 60mg) for 6 weeks.
      • 1.5 mg/kg/d on alternate days (up to 40mg) for 6 weeks.
    • Diuretics (for significant edema):
      • Furosemide.

    Response to Nephrotic Syndrome Treatment

    • Protein in urine < 4 mg/m³/hr or spot dipstick: Nil/trace.
    • P:C ratio < 0.2: Confirms minimal change disease (MCD) without a biopsy.
    • If no response: Add Spironolactone.

    Clinical Features of Nephritic Syndrome

    • Incidence: Most common age group: 5-15 years (school-going children). Male > Female.
    • Clinical presentation: Abrupt onset of hematuria, oliguria, edema, and HTN. No preceding history of infection is required for diagnosis. Oliguria severity determines disease severity.
    • Complications:
      • Hypertensive encephalopathy: Seizures.
      • Left ventricular failure.

    Investigations for Nephritic Syndrome

    • Antibody titre:
      • Anti-Streptolysin O (ASO)
      • Anti-DNAase B (additional in skin infection)
    • Serum C3:
      • Decreased in immune complex-mediated disorders.
      • Usually normalizes within 8-12 weeks.
    • Renal biopsy: Not performed routinely.
      • Indications:
        • Persistent low C3 > 12 weeks (to rule out other causes).
        • Systemic illness: High fever, rash, joint pain.
        • No resolution > 10 days.
      • Findings:
        • Electron microscopy: Sub-epithelial immune complex deposits (humps).
        • Immunofluorescence: IgG + C3 deposition in capillary walls and mesangium (starry sky appearance).

    Management of Nephritic Syndrome

    • Self-limiting condition.
    • Supportive management for hypertension:
      • Amlodipine
      • Nifedipine
    • No prophylactic role of antibiotics (unlike rheumatic fever).

    IgA Nephropathy (Berger's Disease)

    • Most common cause of chronic and recurrent nephritis (associated with chronic kidney disease).

    Pathogenesis of IgA Nephropathy

    • Any viral/bacterial upper respiratory tract infection (URTI): IgA deposits in the mesangium and capillary walls.

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    Description

    This quiz covers essential information regarding anti-inflammatory medications like steroids and aspirin, particularly their usage in cardiac problems. It also explores the duration of prophylaxis based on the presence of carditis and critical insights into the pathogenesis of congenital kidney and urinary tract anomalies, including conditions such as Autosomal Recessive Polycystic Kidney Disease.

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