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Questions and Answers
Which of the following is a feature of chronic kidney disease (CKD)?
Which of the following is a feature of chronic kidney disease (CKD)?
Intra-uterine growth retardation is associated with a decreased risk of CKD.
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?
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 __________.
CKD can be influenced by maternal factors such as age of conception and __________.
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Match the following features with their related conditions:
Match the following features with their related conditions:
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Which of the following structures is derived from the primitive urogenital sinus?
Which of the following structures is derived from the primitive urogenital sinus?
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The anal canal above the pectinate line is derived from proctoderm.
The anal canal above the pectinate line is derived from proctoderm.
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What is the primary origin of the kidneys during embryonic development?
What is the primary origin of the kidneys during embryonic development?
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The __________ urethra is part of the definitive urogenital sinus in females.
The __________ urethra is part of the definitive urogenital sinus in females.
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Match the following parts of the urethra with their respective sexes:
Match the following parts of the urethra with their respective sexes:
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What significant event happens by the end of the 8th week during kidney development?
What significant event happens by the end of the 8th week during kidney development?
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No new nephrons can be formed or replaced after birth.
No new nephrons can be formed or replaced after birth.
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What is the result of decreased birth weight on nephron function?
What is the result of decreased birth weight on nephron function?
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By 32 to 36 weeks of gestation, _____ of nephrons are formed.
By 32 to 36 weeks of gestation, _____ of nephrons are formed.
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Match the following events in kidney development with their corresponding timings:
Match the following events in kidney development with their corresponding timings:
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Which of the following is not a symptom of uncomplicated cystitis?
Which of the following is not a symptom of uncomplicated cystitis?
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Nitrofurantoin is the first-line treatment for uncomplicated urinary tract infections.
Nitrofurantoin is the first-line treatment for uncomplicated urinary tract infections.
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What investigation is performed to rule out anatomical abnormalities in a urinary tract infection?
What investigation is performed to rule out anatomical abnormalities in a urinary tract infection?
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Amikacin is associated with the side effect of __________.
Amikacin is associated with the side effect of __________.
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Match the following treatments with their respective indications:
Match the following treatments with their respective indications:
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What is the primary approach used in laparoscopic retroperitoneal adrenalectomy?
What is the primary approach used in laparoscopic retroperitoneal adrenalectomy?
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The medulla receives a high blood supply and is seldom prone to hypoxia.
The medulla receives a high blood supply and is seldom prone to hypoxia.
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What are the major and minor calyces typically numbered to?
What are the major and minor calyces typically numbered to?
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The tip of the renal pyramid is known as the _____ which is a vital structure.
The tip of the renal pyramid is known as the _____ which is a vital structure.
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Match the kidney structure with its description:
Match the kidney structure with its description:
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What is the estimated occurrence of unilateral renal agenesis in live births?
What is the estimated occurrence of unilateral renal agenesis in live births?
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Bilateral renal agenesis is also referred to as Potter's Syndrome.
Bilateral renal agenesis is also referred to as Potter's Syndrome.
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What mutation is commonly associated with unilateral renal agenesis?
What mutation is commonly associated with unilateral renal agenesis?
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The most common cause of death after birth due to renal agenesis is _____ syndrome.
The most common cause of death after birth due to renal agenesis is _____ syndrome.
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Match the following findings to their associated type of renal agenesis:
Match the following findings to their associated type of renal agenesis:
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What structures does the intermediate mesoderm give rise to?
What structures does the intermediate mesoderm give rise to?
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The medial swelling of the urogenital ridge develops into the nephrogenic cord.
The medial swelling of the urogenital ridge develops into the nephrogenic cord.
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Which of the following is a potential cause of Papillary Necrosis?
Which of the following is a potential cause of Papillary Necrosis?
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Papillary Necrosis has a good prognosis.
Papillary Necrosis has a good prognosis.
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Where is the urogenital ridge located in the embryo?
Where is the urogenital ridge located in the embryo?
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The pronephros forms on day ___ of embryonic development.
The pronephros forms on day ___ of embryonic development.
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Name one systemic condition that can lead to Papillary Necrosis.
Name one systemic condition that can lead to Papillary Necrosis.
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Match the following events to their corresponding timeline:
Match the following events to their corresponding timeline:
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Papillary Necrosis can result in __________ hypodensities indicating possible inflammation.
Papillary Necrosis can result in __________ hypodensities indicating possible inflammation.
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Which of the following is a remnant of the mesonephric duct?
Which of the following is a remnant of the mesonephric duct?
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Match the causes of Papillary Necrosis with their descriptions:
Match the causes of Papillary Necrosis with their descriptions:
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The nephrogenic cord is part of the genital ridge.
The nephrogenic cord is part of the genital ridge.
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What develops from the lateral swelling of the urogenital ridge?
What develops from the lateral swelling of the urogenital ridge?
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Which artery is typically longer, the right renal artery or the left renal artery?
Which artery is typically longer, the right renal artery or the left renal artery?
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The left kidney is positioned lower than the right kidney due to the size of the liver.
The left kidney is positioned lower than the right kidney due to the size of the liver.
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What is the primary venous drainage for both the right and left renal veins?
What is the primary venous drainage for both the right and left renal veins?
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The left renal vein crosses the ______ before draining into the IVC.
The left renal vein crosses the ______ before draining into the IVC.
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Match the following blood supply components with their corresponding descriptions:
Match the following blood supply components with their corresponding descriptions:
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Which of the following conditions is associated with early onset chronic kidney disease (CKD) by the age of 3-5 years?
Which of the following conditions is associated with early onset chronic kidney disease (CKD) by the age of 3-5 years?
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Wilms tumour is a common feature associated with Beckwith-Wiedemann syndrome.
Wilms tumour is a common feature associated with Beckwith-Wiedemann syndrome.
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What is the primary renal failure age associated with Frasier's syndrome?
What is the primary renal failure age associated with Frasier's syndrome?
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In Russell Silver syndrome, one of the characteristic features is __________ facies.
In Russell Silver syndrome, one of the characteristic features is __________ facies.
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Match the following syndromes with their associated features:
Match the following syndromes with their associated features:
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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
-
Ultrasound (USG)
- Rule out obstruction, emphysematous pyelonephritis, and pelvicalyceal anatomical abnormalities.
- If USG is normal, proceed to diagnosis of acute pyelonephritis.
-
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)
-
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
- Lateral branches of the abdominal aorta:
-
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.
