Von Willebrand Disease in Children

Von Willebrand Disease in Children
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All parents handle their child’s bruises and bloody noses from time to time. For the majority of kids, a plaster and a considerate hug are all it takes to get them back in the game.

However a small number of kids have frequent nosebleeds, unexplained bruising, or extended bleeding after a tooth is pulled. Such extreme or extended bleeding could be a sign of a genetic disorder known as von Willebrand disease.

For many kids with this condition, the symptoms are so moderate that they never ever know they have it. Those with a more severe kind of the disease, however, need correct diagnosis and a treatment plan to help them lead normal, active lives.

von Willebrand disease is the most typical bleeding condition seen in children and it affects roughly 1% of the population. Due to the fact that the bleeding symptoms in von Willebrand disease are generally moderate, the diagnosis is frequently delayed. Prompt diagnosis and management can assist to avoid possibly deadly bleeding occasions and unnecessary exposure to blood products. In this review, the different types of von Willebrand disease are detailed, the problems in diagnosis are talked about and the healing technique to this typical condition is explained.

About von Willebrand Disease

Von Willebrand disease, or vWD, is an acquired condition that affects the blood’s capability to thicken properly. It takes its name from Dr. Erik von Willebrand, who first described the condition in 1926. As a group, bleeding conditions (including hemophilia) are uncommon. Von Willebrand disease is the most common inherited bleeding condition, affecting about 1% of the population.

Normally, bleeding takes place when a blood vessel is cut or torn. The blood’s ability to clot (to plug the hole in the blood vessel and stop the flow of blood) is a complex procedure including platelets and proteins called clotting elements. Von Willebrand factor is involved in the early stages of blood clotting, and also carries the important clotting protein element VIII. (Factor VIII is the protein that is missing out on or faulty when somebody has hemophilia.).

People with vWD have bleeding issues since the levels or functioning of these blood parts needed for clotting are irregular.

Types of von Willebrand Disease

As with numerous conditions, there are numerous kinds of vWD. Unlike hemophilia, which impacts just kids, vWD impacts kids and women similarly.

  • In Type 1, the level of von Willebrand factor in the blood is lowered; the level of factor VIII also may be minimized. This is the most common and mildest type of the disease– the symptoms may be so minor that the person isn’t really ever identified. Individuals with Type I vWD generally do not bleed spontaneously however can have considerable bleeding with trauma, surgery, or when they have a tooth pulled.
  • In Type 2, the level of von Willebrand factor in the blood is normal, but does not work properly. Within this type are several subtypes:.
    • Type 2A: the foundation that make up the factor (called multimers) are smaller than normal or break down too quickly.
    • Type 2B: the element adheres to the platelets too well, resulting in clumping of the platelets, which can cause a low platelet number.

Other type 2 subtypes exist, however are much rarer.

  • Type 3 patients have very low or absent von Willebrand factor and element VIII. Symptoms are severe and might consist of bleeding into joints and muscles.
  • Pseudo, or platelet-type, von Willebrand disease resembles Type 2B, however the problem is in the platelets rather of in the element.

Causes of vWD

Like hemophilia, vWD is a congenital disease that is generally passed from parent to child (extremely hardly ever, it can be acquired after birth). The child of a man or a female with vWD has a 50% possibility of getting the gene.

In types 1 and 2, a child can inherit the gene for the disease from one parent just. In type 3, the child generally needs to acquire the gene from both parents. In those cases, the child will likely have severe symptoms, even if the parents don’t have any symptoms at all. Likewise, a child can acquire the gene and reveal no symptoms, however as a carrier can pass the gene on to any offspring.

Von Willebrand Disease in Children

Symptoms and signs

The symptoms of von Willebrand disease can consist of:

  • bruising that’s unusual in place or frequency
  • unusual menstrual bleeding
  • bleeding in the mucous membranes, such as the gums, nose, and lining of the gastrointestinal system
  • extreme or prolonged bleeding after a tooth is pulled or tonsils are removed or lengthened oozing from cuts
  • extreme or prolonged bleeding after circumcision

Diagnosis and Treatment

Since symptoms can be mild, vWD can be hard to detect and typically goes undiscovered.

Blood tests used to detect vWD consist of:

  • von Willebrand element antigen test, which measures the quantity of von Willebrand aspect
  • von Willebrand activity test (also called ristocetin cofactor or RCF activity test), which measures how well the von Willebrand element works
  • factor VIII activity test (also called factor VIII coagulant assay), which determines the level of aspect VIII and its capability to work
  • von Willebrand multimers test, which assists to classify the kind of von Willebrand disease
  • platelet function tests, which determine how well the platelets work

Tests might need to be duplicated due to the fact that the levels they spot may fluctuate in time. Likewise, the doctor will take a household case history to see if other loved ones have a bleeding disorder.

The most common treatment for vWD is desmopressin, a medication that causes a temporary increase in the von Willebrand aspect and aspect VIII levels. It can be given intravenously (into a vein) or intranasally (into the nose), but may be ineffective in treating Type 2A or Type 2B.

Individuals with Type 3 (and some with Type 2A and 2B) will need treatment with Humate-P, an intravenous medication stemmed from human plasma that contains aspect VIII and von Willebrand aspect. Those with Type 1 likewise might get Humate-P in certain circumstances, such as significant injury or significant surgery, although they normally can be treated with desmopressin.

Medication to hinder the breakdown of blood clots (such as aminocaproic acid) likewise might be used.

Precautions

Kids with vWD ought to avoid unnecessary trauma, including contact sports. If bleeding does take place, use pressure to the area. During nosebleeds, pinch the soft part of the nose and have the child lean somewhat forward to keep the blood from streaming down the throat.

Since of the possibility of excessive bleeding, male infants who are at risk for vWD (those with a family history of the disease) must not be circumcised without a doctor’s OK.

Ladies with vWD who have started their periods might wish to take additional pads or a modification of clothes in case of mishaps. Heavy menstrual bleeding often can be controlled with birth control pills.

Kids with vWD ought to not take aspirin and other non-steroidal anti-inflammatory drugs (such as ibuprofen) for pain or fever. These drugs disrupt platelet function and can increase the risk of bleeding. It is safe to take acetaminophen, which doesn’t impact platelet function.

Call your doctor right away if your child experiences any excessive or unexplained bleeding.

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