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Questions and Answers
What is the main purpose of a renogram in the context of hydronephrosis?
What is the main purpose of a renogram in the context of hydronephrosis?
Which statement about medical therapy for UPJ obstruction is correct?
Which statement about medical therapy for UPJ obstruction is correct?
What is the gold standard surgical procedure for UPJ obstruction?
What is the gold standard surgical procedure for UPJ obstruction?
Which of the following is NOT one of the accepted criteria for surgical intervention in infants and children?
Which of the following is NOT one of the accepted criteria for surgical intervention in infants and children?
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What is the success rate of endopyelotomy compared to other surgical options?
What is the success rate of endopyelotomy compared to other surgical options?
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What is the main issue associated with renal agenesis?
What is the main issue associated with renal agenesis?
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Which condition is characterized by the absence of both kidneys?
Which condition is characterized by the absence of both kidneys?
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At what gestational age does nephrogenesis typically cease?
At what gestational age does nephrogenesis typically cease?
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What is a common outcome of unilateral renal agenesis?
What is a common outcome of unilateral renal agenesis?
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What is the condition referred to as Potter’s syndrome associated with?
What is the condition referred to as Potter’s syndrome associated with?
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In cases of renal hypoplasia, which of the following statements is correct?
In cases of renal hypoplasia, which of the following statements is correct?
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What are potential associated anomalies with bilateral renal agenesis indicated by oligohydramnios?
What are potential associated anomalies with bilateral renal agenesis indicated by oligohydramnios?
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What is the term for a kidney with partially developed or atrophic renal cortex?
What is the term for a kidney with partially developed or atrophic renal cortex?
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What condition is characterized by a rudimentary kidney?
What condition is characterized by a rudimentary kidney?
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Which type of kidney abnormality is associated with the presence of large non-communicating cysts and absence of renal cortex?
Which type of kidney abnormality is associated with the presence of large non-communicating cysts and absence of renal cortex?
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What is the primary method for diagnosing Multicystic Dysplastic Kidney (MCDK)?
What is the primary method for diagnosing Multicystic Dysplastic Kidney (MCDK)?
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Which of the following complications is NOT associated with Multicystic Dysplastic Kidney (MCDK)?
Which of the following complications is NOT associated with Multicystic Dysplastic Kidney (MCDK)?
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Which structural abnormality involves the failure of the kidney to ascend during embryogenesis?
Which structural abnormality involves the failure of the kidney to ascend during embryogenesis?
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What percentage of patients with horseshoe kidney experience vesicoureteral reflux (VUR) as a complication?
What percentage of patients with horseshoe kidney experience vesicoureteral reflux (VUR) as a complication?
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What is the implication of a kidney being described as 'larger than normal' in Multicystic Dysplastic Kidney (MCDK)?
What is the implication of a kidney being described as 'larger than normal' in Multicystic Dysplastic Kidney (MCDK)?
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What is the relationship between associated anomalies and ectopic kidney?
What is the relationship between associated anomalies and ectopic kidney?
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What is the most common side affected by ureteropelvic junction (UPJ) obstruction?
What is the most common side affected by ureteropelvic junction (UPJ) obstruction?
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Which imaging technique is primarily used to detect vesicoureteral reflux (VUR) and posterior urethral valves (PUV)?
Which imaging technique is primarily used to detect vesicoureteral reflux (VUR) and posterior urethral valves (PUV)?
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What is a common clinical presentation of UPJ obstruction in adults?
What is a common clinical presentation of UPJ obstruction in adults?
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Which statement about the epidemiology of UPJ obstruction is correct?
Which statement about the epidemiology of UPJ obstruction is correct?
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Which condition can lead to urinary obstruction at the ureteropelvic junction?
Which condition can lead to urinary obstruction at the ureteropelvic junction?
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What is a potential complication of persistent hydronephrosis due to UPJ obstruction?
What is a potential complication of persistent hydronephrosis due to UPJ obstruction?
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In neonates with hydronephrosis, which symptom is typically present?
In neonates with hydronephrosis, which symptom is typically present?
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What characteristic finding is often seen in a Doppler ultrasound of an obstructed kidney?
What characteristic finding is often seen in a Doppler ultrasound of an obstructed kidney?
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What describes a duplex kidney?
What describes a duplex kidney?
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Which condition is characterized by a kidney crossing the midline?
Which condition is characterized by a kidney crossing the midline?
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What is often a cause of hydronephrosis?
What is often a cause of hydronephrosis?
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What are the primary features of hydronephrosis detected via antenatal ultrasound?
What are the primary features of hydronephrosis detected via antenatal ultrasound?
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In a complete duplex kidney, where does the upper pole ureter typically drain?
In a complete duplex kidney, where does the upper pole ureter typically drain?
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What grading system is used to assess the severity of hydronephrosis?
What grading system is used to assess the severity of hydronephrosis?
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What typically characterizes incomplete duplex kidneys?
What typically characterizes incomplete duplex kidneys?
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What is a critical aspect when assessing hydronephrosis in a prenatal ultrasound?
What is a critical aspect when assessing hydronephrosis in a prenatal ultrasound?
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Study Notes
Congenital Anomalies of Kidney
- Congenital kidney anomalies are structural abnormalities present at birth.
- Kidney development occurs in three phases: pronephros, mesonephros, and metanephros.
- Kidney development begins around the 4th week and finishes around the 36th week of intrauterine life.
Renal Agenesis (Absent Kidney)
- Renal agenesis is a condition where one or both kidneys are absent.
- It's caused by failure of the ureteral bud to communicate with the metanephric blastema.
- This condition is relatively rare (1 in 500 to 1 in 3200 live births).
- Unilateral agenesis sometimes goes undiagnosed until later in life.
- Bilateral agenesis usually leads to fatal outcomes soon after birth due to respiratory issues.
Renal Hypoplasia
- Renal hypoplasia involves a smaller-than-normal kidney size.
- It's not dysplastic, meaning the structure is typical.
- It has a reduced number of calyces, nephrons, and glomeruli.
- Unilateral hypoplasia is frequently an incidental finding, often associated with other urinary tract issues or hypertension.
- Bilateral hypoplasia can be compatible with life, with chronic kidney disease as a possible outcome.
- Segmental hypoplasia presents as a kidney with only partial development or atrophied renal tissue.
Renal Dysplasia
- Renal dysplasia involves structural abnormalities.
- It can affect parts or all of the kidney.
- Manifestations are cysts and cartilage-like formations within the kidney.
- Polycystic kidney disease (PKD) is a hereditary form most commonly presenting as bilateral disease.
- Autosomal-dominant PKD (ADPKD): affects mainly adults.
- Autosomal-recessive PKD (ARPKD): usually found in children.
- Multicystic dysplastic kidney (MCDK) is among the most common congenital anomalies in children.
- MCDK usually involves a non-functioning kidney replaced by many cysts.
Abnormalities in Shape and Position
- Ectopic kidney: A kidney that doesn't ascend to its normal position during development can be in the pelvis, thorax, or other abnormal areas.
- Horseshoe kidney: The lower poles of the kidneys fuse across the midline, leading to a "horseshoe" shape. The isthmus is at the level of L4-L5.
- Crossed fused ectopia: One kidney crosses the midline to the opposite side of the body and is fused to the normal kidney's lower pole.
Abnormalities of the Collecting System
- Duplex kidney: Characterized by two collecting systems within one kidney, and two ureters. Complete or incomplete.
- Hydronephrosis: Dilated renal pelvis/collecting system. Possible causes include UPJO. It is more common in the left kidney., It can be detected during fetal ultrasounds (antenatal) and can be assessed further by further diagnostics to pinpoint the cause.
Diagnostic Tests
- Ultrasound (US).
- Voiding Cystourethrogram (VCUG).
- Diuretic Renography.
- Doppler sonography.
Medical and Surgical Treatments
- Conservative management for cysts under 5cm.
- Nephrectomy for cysts over 5cm or significant complications, or if no involution is seen by age 2.
- Pyeloplasty: surgical correction of ureteropelvic junction obstruction.
- Laparoscopic
- Open
- RALP.
- Endopyelotomy: minimally invasive procedure using a balloon to open the obstructed area.
Etiology
- Intrinsic obstruction: narrowing of the ureters due to scar tissue.
- Extrinsic obstruction: compression of the ureters from nearby structures like vessels.
Epidemiology
- Congenital anomalies of the kidneys are relatively common, occurring in about 1 in 100 pregnancies.
- Left kidney more commonly affected than right
- Male to female ratio varies from 3-4:1
Clinical Presentation
- Neonates: primarily present with hydronephrosis
- Older children: include urinary tract infections (UTIs), flank pain, flank masses, and hematuria.
- Adults: include back and flank pain, UTIs, pyelonephritis, and hypertension.
Ureteropelvic Junction Obstruction (UPJ)
- A common cause of hydronephrosis.
- Most commonly detected in left kidney during antenatal ultrasound
- A frequent cause of hydronephrosis.
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Description
This quiz explores congenital anomalies of the kidney, including renal agenesis and renal hypoplasia. You'll learn about the developmental phases of the kidneys and the implications of these structural abnormalities. Test your knowledge on the causes, prevalence, and outcomes of these conditions.
