Multicystic Dysplastic Kidney in Children: Causes and Treatment Options - Pediatrics

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Pediatric Multicystic Dysplastic Kidney


Hello, my son is currently 13 months old and had diarrhea a month ago.
An ultrasound now shows multiple renal cysts.
What type of condition could cause multiple renal cysts in such a young child? At what age would this start to affect his kidney function? Does kidney failure impact sexual function? What treatments are necessary?

Zeng Mama, 30~39 year old female. Ask Date: 2011/03/17

Dr. Pei Rensheng reply Pediatrics


Dear Mother Tseng,
Hello.
Polycystic kidney disease (PKD), also known as cystic kidney disease, is a hereditary kidney disorder characterized by the growth of numerous cysts in the kidneys, leading to an increase in kidney size and a decline in kidney function.
Polycystic kidney disease is one of the most common genetic disorders.
Statistics indicate that approximately 1 in 10,000 people are affected by this condition.
In the early stages, the renal cysts in polycystic kidney disease are small and may not present any clinical symptoms, making diagnosis challenging.
However, various imaging techniques such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can be utilized for evaluation.
If there is a family history of polycystic kidney disease, the diagnosis can be established based on the patient's age, clinical manifestations, and family history.
Polycystic kidney disease is often associated with extrarenal cysts, particularly in the liver, but can also occur in other organs such as the pancreas, ovaries, gastrointestinal tract, and major blood vessels.
Renal cystic disease can also present in a rare form known as childhood polycystic kidney disease, which is associated with a high mortality rate.
Approximately 30% of affected newborns may die from respiratory failure within the first four weeks of life, and over 50% of patients may die from end-stage renal disease (ESRD) before the age of ten.
With advancements in medical treatment, early intervention can improve survival rates, with an 82% survival rate by age ten and a 67-79% survival rate by age fifteen.
Wishing you health and happiness!
Sincerely,
Dr.
Ren-Sheng Pei
Director, Pediatrics Department
Taoyuan Hospital, Department of Health

Reply Date: 2011/03/17

More Info


Multicystic Dysplastic Kidney (MCDK) is a congenital condition characterized by the presence of multiple cysts in one kidney, which can lead to the underdevelopment of that kidney. This condition is typically diagnosed in infants or young children, often during routine ultrasounds performed for other reasons, such as abdominal pain or urinary issues.

Causes of Multicystic Dysplastic Kidney
MCDK is primarily caused by abnormal development of the kidney during fetal growth. The exact reasons for this abnormality are not fully understood, but it is believed to be related to genetic factors and environmental influences during pregnancy. In many cases, MCDK occurs sporadically, meaning it arises without a clear hereditary pattern. However, there are instances where it can be associated with other congenital anomalies or syndromes.


Impact on Kidney Function
In children with MCDK, the affected kidney is usually non-functional or has significantly reduced function. The other kidney typically compensates for the loss of function, and many children with MCDK can lead normal lives without significant health issues. However, the degree to which kidney function is affected can vary. Regular monitoring through ultrasound and kidney function tests is essential to assess the health of the remaining kidney and to ensure it is functioning adequately.

As for the age at which kidney function might be impacted, it can vary significantly. Some children may show signs of renal impairment early on, while others may not experience any issues until later in childhood or even adolescence. It is crucial to maintain regular follow-ups with a pediatric nephrologist to monitor kidney function and overall health.


Effects of Kidney Failure on Sexual Function
Kidney failure can have various systemic effects on the body, including hormonal imbalances that may impact sexual function. However, in the context of a child with MCDK, it is unlikely that kidney failure would directly affect sexual function at such a young age. Most concerns regarding sexual function arise in older adolescents or adults with chronic kidney disease or end-stage renal disease.


Treatment Options
The treatment for MCDK primarily focuses on monitoring and managing any complications that may arise. Here are some key points regarding treatment:
1. Monitoring: Regular follow-up appointments with a pediatric nephrologist are essential. This includes routine ultrasounds to assess the size and function of the remaining kidney and blood tests to monitor kidney function.

2. Management of Complications: If the child develops high blood pressure or urinary tract infections, these conditions will need to be managed appropriately.
3. Surgical Intervention: In some cases, if the cystic kidney becomes symptomatic (for example, causing pain or recurrent infections), surgical removal of the affected kidney may be considered. However, this is not common and is typically reserved for specific situations.

4. Nutritional Support: Ensuring that the child has a balanced diet and adequate hydration is important for overall kidney health.

5. Education and Support: Educating the family about the condition and providing psychological support can help in coping with the diagnosis and any potential challenges.

In conclusion, while MCDK can pose challenges, many children with this condition can lead healthy lives with appropriate monitoring and management. It is essential to maintain open communication with healthcare providers to ensure the best outcomes for your child. If you have further concerns or questions, do not hesitate to reach out to your child's healthcare team for personalized advice and support.

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