Congenital disorders of the kidney are conditions that are present at birth and can significantly affect kidney function and overall health. These disorders can vary widely in their manifestations and severity. Here’s a detailed summary of the main types of congenital kidney disorders based on recent resources:

1. Polycystic Kidney Disease (PKD): There are two primary types of PKD:

   • Autosomal Dominant Polycystic Kidney Disease: This form commonly develops later in life and leads to the formation of fluid-filled cysts in the kidneys.
   • Autosomal Recessive Polycystic Kidney Disease: This variant is typically evident at birth and can cause more severe complications, including liver and lung issues.

2. Renal Agenesis: This condition occurs when one or both kidneys do not develop at all. If only one kidney is missing (unilateral renal agenesis), individuals may live normally with the functional kidney. However, if both kidneys are absent (bilateral renal agenesis), it is often life-threatening.

3. Renal Hypoplasia: This refers to underdeveloped kidneys, which can be smaller in size and may not function properly, potentially leading to renal insufficiency.

4. Horseshoe Kidney: This anomaly occurs when both kidneys are fused together at the lower end, forming a horseshoe shape. This condition can lead to complications, such as obstructions or infections.

5. Hydronephrosis: This is characterized by the swelling of a kidney due to a buildup of urine, usually caused by a blockage that prevents urine from draining properly to the bladder.

6. Vesicoureteral Reflux (VUR): This condition allows urine to flow backward from the bladder into the kidneys, increasing the risk of kidney infections.

7. Congenital Anomalies Associated with Syndromes: There are several syndromes involving congenital kidney anomalies such as:

   • Alport Syndrome: Characterized by malformation of blood vessels in the kidneys.
   • Fraser Syndrome and Townes-Brocks Syndrome, among others, which may include various renal abnormalities alongside other physical symptoms.

8. Other Notable Disorders: Conditions like renal coloboma syndrome and 17q12 deletion syndrome can also lead to kidney anomalies, as well as more generalized syndromic presentations that affect kidney development.

For a more comprehensive understanding and details regarding these conditions, you can explore the following resources:

• Congenital Diseases of the Kidney - Riley Children's Health
• Congenital Anomalies of the Kidneys and Urinary Tract - CHOP
• Hereditary and Congenital Kidney Diseases - UC Davis Health
• Congenital Kidney Abnormalities - Children's Health Nephrology
• Congenital Anomalies of Kidney and Urinary Tract - MedlinePlus
• Clinical Review of Congenital Anomalies - PMC
• Norton Children's Services - Congenital Kidney Anomalies

Each of these resources provides additional insights into how these disorders develop and impact health outcomes.

Sources

Congenital Diseases of the Kidney - Riley Children's HealthCongenital Anomalies of the Kidneys and Urinary TractHereditary and Congenital Kidney Diseases - UC Davis HealthCongenital kidney abnormalities – Children's Health NephrologyCongenital anomalies of kidney and urinary tract - MedlinePlusCongenital Anomalies of the Kidney and Urinary Tract: A Clinical ...Congenital anomalies of the kidneys and urinary tract

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