Horseshoe nephropathy in children - The disease is rare and should be understood

Horseshoe kidney is a kidney birth defect that is not well known. The cause of this defect is still not clearly explained, however, horseshoe shaped kidneys can cause many health effects of sufferers, especially children.

So what is horseshoe-shaped kidney disease and how these defects can affect the health of children, invite you to learn with aFamilyToday Health in this article!

What is horseshoe kidney disease?

Horseshoe nephrology is a birth defect caused by abnormalities in the rotation and movement of the kidneys as the fetus grows in the womb . The person with this defect may not experience any effects, but it can also cause serious health problems.

 

You can imagine the kidney shape of patients with this disease exactly as their name suggests. The two kidneys are joined together by a "fake kidney waist" to form a horseshoe-like shape.

However, not only is the kidneys abnormal in shape and structure, but their location is not the same as that of nature. Instead of being located in the upper abdomen, below the rib cage, and on either side of the spine, the horseshoe kidney is usually located lower in the pelvis. This is not the only birth defect related to the position and structure of the kidney, another common condition that is lost in the kidney.

Before understanding why horseshoe-shaped kidney condition and its effects on health, let's explore the normal process of kidney formation.

The process of kidney formation in normal people

From the embryonic stage to the time humans become a complete individual, our kidneys need to go through 3 developmental processes to become complete and fully functional:

Pronephros (Pronephros)

Central kidney (Mesonephros)

Post-Renal (Metanephros)

A set of primordial cells and structures that fuse together to form a fully functioning kidney. The post-renal stage will be completed when the fetus is about 6 weeks old, including 2 structures: "posterior mediastinum" and "ureter's germ / bud". These structures eventually form the kidneys and ureters.

Few people know that before entering the post-renal stage, the kidneys are in the pelvis, not in the upper abdomen as the position of the "mature" kidney. As the embryo develops, the kidney's position gradually changes, and they move from the pelvis to a position below the chest and on either side of the spine. Not only moving upwards, the kidneys also rotate inward so that the kidney sinks toward the spine. This is done before your  baby is 8 weeks old .

After having an overview of the kidney formation process, you can imagine: If any disruption occurs during the rotation and movement of the kidneys to the final position, not only the kidneys will lie empty. In the right position, you can also merge into a block instead of splitting into two separate left and right kidneys.

The process of forming horseshoe kidneys

Horseshoe nephropathy in children - The disease is rare and should be understood

 

 

Horseshoe-shaped kidney is often referred to as "abnormal fusion". When abnormal fusion occurs, one kidney attaches to the other. This is due to a disruption in the normal movement of both kidneys.

Rarely, a movement abnormality affects only one kidney, resulting in both kidneys being present on one side of the spine. This condition is called a crossed fused ectopic kidney. The above abnormalities usually occur between 7 and 9 weeks of pregnancy.

In common horseshoe-shaped kidney disease, the lower extremities of the two kidneys join together and create a typical horseshoe shape. The tubes that carry urine from the kidneys, also called ureters, persist and drain urine to each side separately. The part that connects the two kidneys is called the "fake kidney waistline".

This waistline may or may not lie symmetrically on the spine. If they lie on one side, they are called asymmetrical horseshoes. Renal functional tissues may or may not be involved in making the false kidney waistline, so there are cases where the two kidneys are joined together by a strip of nonfunctioning fibrous tissue.

Horseshoe-shaped kidney disease is uncommon, incidence is about 1/500 and is more common in boys than girls. Most children do not experience many serious health effects from the disease.

However, about a third of children with this deformity have problems with the heart, blood vessels, nervous system, reproductive system, urinary system, digestive system and bone. There is no cure for horseshoe kidney disease, but a doctor can help manage its symptoms.

Symptoms of horseshoe kidney disease

Most horseshoe-shaped kidneys do not cause any symptoms. In fact, this pathology is often discovered randomly based on the results of imaging tests taken for another reason. However, when the disease does show symptoms, it is usually related to the flow of urine produced by the abnormal position and orientation of the kidneys.

The most common symptoms are:

A urinary tract infection (UTI) can cause fever, unpleasant odor, or urinary urgency, frequent urination, pain or other problems while urinating.

Kidney stones  lead to throbbing pain in the back, side / side or lower abdomen, nausea or vomiting, cloudy or bloody urine, chills, and fever.

Kidney retention , which occurs when something blocks the urinary tract and causes urine to build up, causing the kidneys to swell. Symptoms include: feeling heavy, underweight, urinating less, urinary tract infection.

Children with this disease are at risk for other health problems such as:

Wilms' tumor , a kidney tumor that can occur in young children

Polycystic kidney disease , which causes cysts that are not cancerous

Kidney cancer

Hydrocephalus , or hydrocephalus in the brain

Spina bifida , when a part of the spinal cord experiences a "fault" in its formation

The cause of horseshoe kidneys

There is currently no firm basis to explain the cause of this defect. Experts believe that horseshoe kidneys are at risk when a child has certain genetic disorders, especially:

Turner syndrome , a condition that occurs in girls, causes height problems and leads to shorter-than-normal ovarian conditions.

Edwards Syndrome , also known as Trisomy 18, causes slow growth in the womb, low birth weight, and a few other serious medical problems.

Methods of diagnosis and treatment of horseshoe kidney disease

Horseshoe nephropathy in children - The disease is rare and should be understood

 

 

Usually, doctors do not diagnose horseshoe-shaped kidney conditions before a baby is born. Symptoms are often similar to some other health problems, so in order to get an accurate diagnosis, you need to take your child to see a doctor.

After asking about symptoms and health history, the doctor will conduct a physical examination and also recommend that you have your child undergo other tests such as:

Blood test to evaluate the ability of the kidneys to function.

Urine test, which helps check for signs of infection.

Kidney ultrasound, a test that uses ultrasound waves to create an image of an organ in the body. The imaging results will help the doctor see abnormal signs such as kidney stones, cysts or tumors.

An intravenous kidney scan (IVP) or an X-ray of the bladder-urethra while urinating (VCUG) uses X-rays to evaluate the activity of the urinary system in the body.

The truth is that scientists have yet to find a cure for this rare disease. Once they find that the kidneys fuse together into a horseshoe, doctors usually stay the same and do not interfere with the condition. However, you may need to treat certain problems caused by horseshoe-shaped kidneys. The most common case is the use of antibiotics during an infection or surgery to remove kidney stones from the body.

In the absence of symptoms, there is no need for in-depth treatment.

Complications of horseshoe kidney disease

Most complications stem from the symptoms and signs of horseshoe kidney, often related to a blockage of the urinary tract.

People with this disease are at risk for a type of kidney tumor called "Wilms tumor" but the cause is unknown. There has been research lasting nearly 30 years showed that 41 patients with Wilms tumor have horseshoe kidney disease.

People with horseshoe-shaped kidney disease are often vulnerable to closed- stomach injuries . For example, if you wear a seat belt while traveling by car, the rope will run across your abdomen including horseshoe kidneys on your spine. When the impact occurs, the kidneys are more susceptible to damage because the normal kidney position is higher and the two kidneys that are not connected are less at risk of injury.

Keep in mind that horseshoe kidney is a fairly rare kidney structural and position abnormality. Most patients show no symptoms and often find the disease by accident through imaging tests. However, you should also be aware of the warning signs associated with obstructive urine flow, kidney stones or urinary tract infection.

If you notice symptoms of suspected horseshoe kidney disease, you should see your doctor to be examined and diagnosed promptly. Certain treatments (including surgery) to relieve urinary obstruction may be used. Most patients are carefully monitored and do not require further evaluation or treatment. In addition, you should be aware of the possible risks associated with horseshoe-shaped kidney disease when experiencing closed stomach injuries.

 

 


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