Medicine Marrow Pg No 705-714 (Nephrology)
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Questions and Answers

Which of the following is a feature of chronic kidney disease (CKD)?

  • Increase in the number of functional nephrons
  • Increased interstitial fibrosis (correct)
  • Reduction in tubular atrophy
  • Decreased glomerulosclerosis

    • Intra-uterine growth retardation is associated with a decreased risk of CKD.

    False

    What is the significance of the PAX-2 gene in kidney development?

    It is important for the formation of the epithelium of the ureteric bud.

    CKD can be influenced by maternal factors such as age of conception and __________.

    <p>pre-eclampsia/hypertension</p> Signup and view all the answers

    Match the following features with their related conditions:

    <p>Wilms tumor = WAGR mutation Aniridia = WAGR mutation Genitourinary abnormalities = WAGR mutation Mental retardation = WAGR mutation</p> Signup and view all the answers

    Which of the following structures is derived from the primitive urogenital sinus?

    <p>Spongy urethra</p> Signup and view all the answers

    The anal canal above the pectinate line is derived from proctoderm.

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

    What is the primary origin of the kidneys during embryonic development?

    <p>Intermediate mesoderm</p> Signup and view all the answers

    The __________ urethra is part of the definitive urogenital sinus in females.

    <p>part of urethra</p> Signup and view all the answers

    Match the following parts of the urethra with their respective sexes:

    <p>Prostatic urethra = Males Spongy urethra = Males Part of urethra = Females Membranous urethra = Males</p> Signup and view all the answers

    What significant event happens by the end of the 8th week during kidney development?

    <p>Formation of urine</p> Signup and view all the answers

    No new nephrons can be formed or replaced after birth.

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

    What is the result of decreased birth weight on nephron function?

    <p>Decreased number of functional nephrons</p> Signup and view all the answers

    By 32 to 36 weeks of gestation, _____ of nephrons are formed.

    <p>60-70%</p> Signup and view all the answers

    Match the following events in kidney development with their corresponding timings:

    <p>Epithelial-mesenchymal interaction = 6 - 8th week First fully formed nephron = End of 8th week Completion of nephrogenesis = 9 - 10 weeks Fixed number of nephrons = At birth</p> Signup and view all the answers

    Which of the following is not a symptom of uncomplicated cystitis?

    <p>Flank pain</p> Signup and view all the answers

    Nitrofurantoin is the first-line treatment for uncomplicated urinary tract infections.

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

    What investigation is performed to rule out anatomical abnormalities in a urinary tract infection?

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

    Amikacin is associated with the side effect of __________.

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

    Match the following treatments with their respective indications:

    <p>Nitrofurantoin = Uncomplicated cystitis Amikacin = Acute pyelonephritis Cotrimoxazole = Uncomplicated cystitis Cefoperazone sulbactam = Acute pyelonephritis</p> Signup and view all the answers

    What is the primary approach used in laparoscopic retroperitoneal adrenalectomy?

    <p>Posterior approach</p> Signup and view all the answers

    The medulla receives a high blood supply and is seldom prone to hypoxia.

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

    What are the major and minor calyces typically numbered to?

    <p>Major: 3-4, Minor: 9-12</p> Signup and view all the answers

    The tip of the renal pyramid is known as the _____ which is a vital structure.

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

    Match the kidney structure with its description:

    <p>Renal pyramid = Protrudes into minor calyx Calyx = Collects urine from the renal pyramids Renal columns of Bertini = Cortical tissues between pyramids Papilla = Most vulnerable part of medulla</p> Signup and view all the answers

    What is the estimated occurrence of unilateral renal agenesis in live births?

    <p>1 in 10,000</p> Signup and view all the answers

    Bilateral renal agenesis is also referred to as Potter's Syndrome.

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

    What mutation is commonly associated with unilateral renal agenesis?

    <p>PAX-2 mutation</p> Signup and view all the answers

    The most common cause of death after birth due to renal agenesis is _____ syndrome.

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

    Match the following findings to their associated type of renal agenesis:

    <p>Pulmonary hyperplasia = Bilateral Renal Agenesis Absent Kidney = Bilateral Renal Agenesis PAX-2 mutation = Unilateral Renal Agenesis Optic nerve coloboma = Unilateral Renal Agenesis</p> Signup and view all the answers

    What structures does the intermediate mesoderm give rise to?

    <p>Kidneys and reproductive organs</p> Signup and view all the answers

    The medial swelling of the urogenital ridge develops into the nephrogenic cord.

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

    Which of the following is a potential cause of Papillary Necrosis?

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

    Papillary Necrosis has a good prognosis.

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

    Where is the urogenital ridge located in the embryo?

    <p>Cervical to sacral region</p> Signup and view all the answers

    The pronephros forms on day ___ of embryonic development.

    <p>22</p> Signup and view all the answers

    Name one systemic condition that can lead to Papillary Necrosis.

    <p>Diabetes mellitus</p> Signup and view all the answers

    Match the following events to their corresponding timeline:

    <p>Day 22 = Formation of the pronephros End of 4th week = Formation of mesenchyme from nephrogenic cord 5th week = No role in humans</p> Signup and view all the answers

    Papillary Necrosis can result in __________ hypodensities indicating possible inflammation.

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

    Which of the following is a remnant of the mesonephric duct?

    <p>Hydatid of Morgagni</p> Signup and view all the answers

    Match the causes of Papillary Necrosis with their descriptions:

    <p>Complicated UTI = Common in diabetic patients Analgesic nephropathy = Caused by overuse of pain relief medications Snake bite = Can lead to acute kidney injury Sickle cell anemia = Associated with vascular occlusion in kidneys</p> Signup and view all the answers

    The nephrogenic cord is part of the genital ridge.

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

    What develops from the lateral swelling of the urogenital ridge?

    <p>Nephrogenic cord</p> Signup and view all the answers

    Which artery is typically longer, the right renal artery or the left renal artery?

    <p>Left renal artery</p> Signup and view all the answers

    The left kidney is positioned lower than the right kidney due to the size of the liver.

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

    What is the primary venous drainage for both the right and left renal veins?

    <p>Inferior vena cava (IVC)</p> Signup and view all the answers

    The left renal vein crosses the ______ before draining into the IVC.

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

    Match the following blood supply components with their corresponding descriptions:

    <p>Inferior phrenic artery = 1st arterial branch from aorta Middle suprarenal artery = 2nd arterial branch from aorta Renal artery = 3rd arterial branch from aorta Gonadal artery = 4th arterial branch from aorta</p> Signup and view all the answers

    Which of the following conditions is associated with early onset chronic kidney disease (CKD) by the age of 3-5 years?

    <p>Diffuse mesangial sclerosis</p> Signup and view all the answers

    Wilms tumour is a common feature associated with Beckwith-Wiedemann syndrome.

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

    What is the primary renal failure age associated with Frasier's syndrome?

    <p>by 15-20 years</p> Signup and view all the answers

    In Russell Silver syndrome, one of the characteristic features is __________ facies.

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

    Match the following syndromes with their associated features:

    <p>Denys Drash syndrome = Wilms tumour Frasier's syndrome = Gonadal dysgenesis Beckwith-Wiedemann syndrome = Macroglossia Russell Silver syndrome = Triangular facies</p> Signup and view all the answers

    Study Notes

    CKD Features

    • Decrease in number of functional nephrons
    • Percentage of interstitial fibrosis, tubular atrophy and glomerulosclerosis
    • Maternal factors can influence the development of CKD in offspring
      • Age of conception
      • Pre-eclampsia/hypertension
      • Placental insufficiency
    • Intra-uterine growth retardation (IUGR) can lead to increased risk of CKD, hypertension, hyperuricemia, diabetes, and metabolic syndrome

    WAGR Mutation

    • A mutation in the WT1 gene can lead to WAGR syndrome.
    • WAGR syndrome manifests as a combination of four conditions:
      • Wilms tumor
      • Aniridia
      • Genitourinary abnormalities
      • Mental retardation

    Embryology of the Kidney

    • Intermediate mesoderm forms the urogenital system, the somites, muscles, and myotomes.
    • Lateral plate mesoderm differentiates into parietal and visceral layers.
    • Parietal lateral plate mesoderm forms the outer layer of pericardium, pleura, and peritoneum.
    • Visceral lateral plate mesoderm forms the inner layer of pericardium, pleura, and peritoneum.

    The Cloaca

    • The hindgut distal to the allantois forms the cloaca.
    • The primitive urogenital sinus develops into the definitive urogenital sinus, which gives rise to the prostate, bladder, and urethra.
    • In males, the prostatic urethra extends up to the opening of the ejaculatory duct.
    • In females, the prostatic urethra contributes to the urethra.
    • The pelvic part of the urethra forms in both males and females.
    • The phallic part of the urethra forms the spongy urethra in males and the vestibule in females.
    • The primitive rectum gives rise to the rectum and anal canal up to the pectinate line.

    Urinary Tract Infection (UTI)

    • Uncomplicated UTI is characterized by cystitis in young, healthy, sexually active females.
    • Uncomplicated UTI symptoms include urgency, frequency, dysuria, and the absence of fever and flank pain.
    • Treatment for uncomplicated UTI:
      • Nitrofurantoin 100 mg BD x 3-5 days (Drug of choice)
      • Cotrimoxazole 200 mg BD x 3-5 days
      • Fosfomycin 3 g sachet
    • Complicated UTI encompasses all other causes, requiring further investigation.

    Investigation Protocol for UTI

    1. Ultrasound (USG)
      • Rule out obstruction, emphysematous pyelonephritis, and pelvicalyceal anatomical abnormalities.
      • If USG is normal, proceed to diagnosis of acute pyelonephritis.
    2. Treatment for acute pyelonephritis:
      • Amikacin (Note: potential nephrotoxicity)
      • Beta-lactam inhibitors:
        • Cefoperazone sulbactam 1.5g IV BD
        • Piperacillin + tazobactam 4.5g IV QID (4.5g IV BD in renal failure)
    3. Contrast-enhanced computed tomography (CECT) abdomen
      • Use low volume, iso-osmolar contrast

    Developmental Timeline of Kidneys

    • 6-8th week: Epithelial-mesenchymal interaction/transition involving the epithelium of the ureteric bud and the mesenchyme of the metanephric bud. WT1 gene (a transcription factor) plays a crucial role.
    • End of 8th week: Formation of urine through filtration and the development of the first fully formed nephron.
    • 9-10 weeks: Ascent of the kidney with rotation and completion of nephrogenesis.
    • 32-36 weeks: Formation of 60-70% of nephrons in the third trimester. There is a fixed number of nephrons after this point.
    • At birth: No new nephrons are formed or replaced after birth. Increased urinary concentration ability reaches adult levels by 1.5 years of age.

    Pathogenesis of CKD

    • Nephron number is a crucial factor in CKD development.
    • Low birth weight often associates with decreased functional nephrons, leading to increased single nephron glomerular filtration rate (GFR).
    • Increased single nephron GFR can result in glomerular hyperfiltration, intraglomerular hypertension, and subsequent hyperfiltration injury.
    • Hyperfiltration injury stretches the capillary pore, increasing its diameter and leading to proteinuria (albuminemia).
    • Proteinuria triggers the release of TGF-β, which contributes to interstitial inflammation, tubular injury, and the progression of CKD.
    • Fibrosis of tubules and interstitium are eventual consequences of this process.

    Applied Anatomy of Kidneys

    • Laparoscopic retroperitoneal adrenalectomy is performed using a posterior approach.
    • Nephrectomy employs an anterior approach, with the patient in a supine position.

    Parts of the Kidney

    • Calyx:
      • Major calyx: 3-4 in number.
      • Minor calyx: 9-12 in number.
    • Renal pyramid:
      • Base: Located at the corticomedullary junction.
      • Apex: Protrudes into the minor calyx.
      • Tip (Papilla): A vital structure that is prone to detachment and excretion in urine, leading to papillary necrosis.
    • Renal columns of Bertini: Cortical tissues extending between the base of the pyramids.
    • Medulla:
      • Receives a lesser blood supply, making it susceptible to hypoxia.
      • The papilla is the most vulnerable part, prone to detachment and papillary necrosis.

    Intermediate Mesoderm

    • The intermediate mesoderm gives rise to the urogenital system, encompassing the kidneys and reproductive organs.

    Urogenital Ridge

    • The urogenital ridge is located in the cervical to sacral region of the embryo, adjacent to the Wolffian duct.
    • The urogenital ridge comprises two swellings:
      • Medial swelling: Develops into the genital ridge, forming either ovaries or testes.
      • Lateral swelling: Develops into the nephrogenic cord, the excretory part of the kidney.

    Timeline of Embryonic Kidney Development

    • Day 22 (4th week): Formation of the pronephros, which degenerates rapidly.
    • End of 4th week: Formation of the mesonephros, which has no functional role in humans.
    • 5th week: Formation of the mesenchyme of the metanephric blastema from the nephrogenic cord and the epithelium of the ureteric bud from the mesonephric duct.

    Remnants of the Mesonephric System

    • Appendix of epididymis: Remnant of the mesonephric duct.
    • Appendix of testis: Remnant of the mesonephric duct.
    • Hydatid of Morgagni: Remnant of the mesonephric duct.

    Unilateral Renal Agenesis

    • More common in females (F > M).
    • Occurs in approximately 1 in 10,000 live births.
    • Associated with PAX-2 mutation.
    • Associated with:
      • Optic nerve coloboma
      • Dextrocardia
      • Hypertension (due to decreased nephron number)
      • Flat foot

    Bilateral Renal Agenesis (Potter's Syndrome)

    • Most common cause of death immediately after birth.
    • Associated with RET gene/GDNF protein (Glial Derived Neurotrophic Factor) mutation.
    • Findings:
      • Pulmonary hyperplasia
      • Absent kidneys and renal arteries
      • Disc-shaped adrenal glands

    Gross Anatomy of the Kidney

    • Levels:
      • S₁ - S₄ (Fetal) - Ascend with rotation.
      • L₃: Hilum
    • Left kidney: Higher than the right kidney (due to the presence of the liver on the right side).

    Blood Supply

    • Arterial supply:
      • Lateral branches of the abdominal aorta:
        • 1st: Inferior phrenic artery
        • 2nd: Middle suprarenal artery
        • 3rd: Renal artery
          • Right renal artery: Longer.
        • 4th: Gonadal artery
    • Venous drainage:
      • Right and left renal veins drain into the inferior vena cava (IVC).
      • Left renal vein:
        • Longer
        • Crosses the midline
        • Tributaries: Ureteral vein, gondial vein, adrenal vein.
        • Susceptible to compression by a superior mesenteric artery aneurysm.

    Kidney Transplantation

    • TC + DTPA (technetium-99m-labeled dimercaptosuccinic acid) is used to assess single kidney GFR, and the kidney with higher GFR is retained in the donor.
    • Anastomosis:
      • Donor ureter is connected to the recipient bladder.
      • Donor renal artery is connected to either the internal iliac artery, external iliac artery, or aorta.
      • Donor renal vein is connected to the external iliac vein.
    • Donor kidney is typically located in the right iliac fossa.
    • Recipient kidney remains in its original location.

    Papillary Necrosis

    • Causes:
      • Complicated UTI in diabetic patients.
      • Analgesic nephropathy (Phenacetin + aspirin + caffeine -> CKD).
      • Snake bite.
      • Sickle cell anemia.
    • Prognosis: Poor.
    • Key Findings:
      • Slightly enlarged kidney.
      • Thickened infundibulum and ureter.
      • Patchy hypodensities (possible inflammation).
      • Nephrocalcinosis.

    Denys-Drash Syndrome

    • Features:
      • Wilms tumor.
      • Disorders of sexual development (DSD): Male pseudohermaphroditism.
      • Diffuse mesangial sclerosis leading to early-onset CKD (within 3-5 years).

    Frasier's Syndrome

    • Gonads:
      • Gonadoblastoma.
      • Gonadal dysgenesis.
      • External genitalia are normal.
    • Biopsy: Focal segmental glomerulosclerosis.
    • Renal failure: Usually develops by 15-20 years of age.

    Role of WT1 Gene in Kidney Development

    • The WT1 gene does not play a role in epithelial-mesenchymal interaction.

    Beckwith-Wiedemann Syndrome

    • Caused by a genomic imprinting anomaly.
    • Features:
      • Macroglossia.
      • Hemihypertrophy of limbs.
      • Omphalocele.
      • Wilms tumor.
      • Microcephaly/ear lobe crease.
      • Hypoglycemia.
      • Medullary sponge kidney (in adults).

    Russel-Silver Syndrome

    • Features:
      • Triangular facies.
      • Mental retardation.

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    Description

    This quiz explores the key features of chronic kidney disease (CKD) and the genetic implications of WAGR syndrome. It covers important aspects such as the impact of maternal factors on CKD development and the embryological origins of the kidneys. Test your understanding of these critical topics in nephrology and genetics.

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