About hie syndrome

What is hie syndrome?

Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare primary immunodeficiency disorder. Symptoms often become apparent at birth or early during infancy or childhood. The disorder is characterized by the triad of highly elevated levels of IgE in serum, recurring abscesses of the skin, and recurrent pneumonia AR-HIES is inherited as an autosomal recessive trait and the first symptoms include the development of a dry, red, flaky skin rash (eczema).

The clinical triad of AR-HIES is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; see this term), but other features such as persistent cutaneous viral infections and neurological symptoms are unique to the AR-HIES form. For years, researchers considered them different expressions of the same disorder, but now researchers consider them similar, yet distinct disorders.

The first case of hyper IgE syndrome was described in the medical literature in 1966. The physicians termed the disorder Job syndrome after the biblical character of Job who was covered in boils and sores over his entire body.

What are the symptoms for hie syndrome?

When the brain experiences a reduction in oxygen or blood flow, hypoxic-ischemic encephalopathy (HIE), a type of brain dysfunction (brain injury), takes place. HIE can happen prior to birth, during labor and delivery, or the following delivery. How much Brain damage occurs depends on how long the brain is deprived of oxygen and blood flow.

HIE syndrome in children could not have any lasting effects. HIE may have a modest to moderate impact on some people while having a more profound handicap on others. HIE-related brain injury can result in developmental delay, cognitive impairment, cerebral palsy, or epilepsy.

Symptoms

The degree and scope of the brain damage, as well as the regions of the brain that were affected, frequently influence the symptoms of HIE Syndrome. Some symptoms of children who have HIE syndrome are listed below.

1. Be unresponsive to sights or sounds and sloppy. As opposed to a healthy baby, some infants with HIE are extremely tense and respond to stimulation more strongly.

2. Have Abnormal movements or seizures.

3. Have trouble eating since their throat and mouth muscles are weak.

4. Show indications of organ malfunction, particularly in the blood, kidneys, liver, heart, and lungs.

Symptoms
Have a weak cry,Decreased alertness and activity,Lack of typical reflexes,Abnormal (not typical) movements or seizures,Low or high muscle tone,Breathing problems
Conditions
Uterine rupture and tear are two conditions that might lead to HIE syndrome in your child,Although HIE primarily affects the brain, reduced blood flow or oxygen levels can also have negative effects on the lungs, liver, heart, intestines, and kidneys,It may cause long-term incapacity and, regrettably, in rare circumstances
Drugs
Levetiracetam,Phenytoin,Lidocaine,Benzodiazepines,Phenobarbital

What are the causes for hie syndrome?

HIE syndrome is a type of brain dysfunction that develops when there is a prolonged lack of oxygen or blood supply to the brain. Hypoxic refers to a lack of oxygen, ischemia to a lack of blood flow, and encephalopathy to a disease of the brain. HIE syndrome can appear at any time, including during pregnancy, labor, delivery, and the postpartum period.

When the blood or oxygen flow to the brain has been interrupted, the remainder of the body may have been "starved" of oxygen. As a result, damage to the intestines, kidneys, liver, heart, and other organs is possible. These organs typically function correctly once more. The brain may not fully recover if it has been injured. Usually, the length of time the brain was oxygen-depleted determines the severity of the damage. Some children's health may be slightly or moderately impacted by HIE syndrome, whilst other children may experience far more severe and pervasive problems, such as cognitive impairment, cerebral palsy (motor impairment), epilepsy, or developmental delay.

Causes

1. HIE is brought on by an abrupt reduction in the supply of blood and oxygen to the developing brain during labor or pregnancy.
2. The growing brain of the infant experiences a rapid deterioration and eventual death of its cells due to a lack of oxygen and blood.
3. HIE is typically brought on by obstetric problems during labor and delivery, but it can also be brought on by prenatal complications or occurrences.

Symptoms
Have a weak cry,Decreased alertness and activity,Lack of typical reflexes,Abnormal (not typical) movements or seizures,Low or high muscle tone,Breathing problems
Conditions
Uterine rupture and tear are two conditions that might lead to HIE syndrome in your child,Although HIE primarily affects the brain, reduced blood flow or oxygen levels can also have negative effects on the lungs, liver, heart, intestines, and kidneys,It may cause long-term incapacity and, regrettably, in rare circumstances
Drugs
Levetiracetam,Phenytoin,Lidocaine,Benzodiazepines,Phenobarbital

What are the treatments for hie syndrome?

HIE syndrome is a type of brain dysfunction that develops when there is a prolonged lack of oxygen or blood supply to the brain. Hypoxic refers to a lack of oxygen, ischemia to a lack of blood flow, and encephalopathy to a disease of the brain. HIE syndrome can appear at any time, including during pregnancy, labor, delivery, and the postpartum period.

HIE syndrome can have minor or moderate impacts on some children's health while leaving others with considerably more severe and long-lasting disabilities, such as developmental delay, cerebral palsy (motor impairment), epilepsy, or cognitive impairment.

Treatment

There are numerous Hie syndrome treatments available to help treat children who have Hie syndrome. Some treatments are maintained below.

1. Brain Cooling: Infants born with HIE require three days of chilling as part of immediate treatment. According to research, brain damage may be reduced if the brain is kept a few degrees below body temperature shortly after birth.

2. For three days, your infant will be placed on a cooling blanket. Then, after receiving medications to make them more comfortable while they sleep, they are watched closely for any indications of discomfort.

3. Other therapies include sustaining kidney and liver function, supporting the heart and blood pressure, and using mechanical ventilation (a breathing tube) if the infant is unable to breathe on their own.

Symptoms
Have a weak cry,Decreased alertness and activity,Lack of typical reflexes,Abnormal (not typical) movements or seizures,Low or high muscle tone,Breathing problems
Conditions
Uterine rupture and tear are two conditions that might lead to HIE syndrome in your child,Although HIE primarily affects the brain, reduced blood flow or oxygen levels can also have negative effects on the lungs, liver, heart, intestines, and kidneys,It may cause long-term incapacity and, regrettably, in rare circumstances
Drugs
Levetiracetam,Phenytoin,Lidocaine,Benzodiazepines,Phenobarbital

What are the risk factors for hie syndrome?

HIE syndrome is brain injury brought on by oxygen deprivation that happens during the perinatal period, the weeks before birth, or during labor and delivery.

The rest of the body may have been "deprived" of oxygen if the blood or oxygen supply to the brain has been cut off. The heart, liver, kidneys, and colon could all suffer harm as a result. Usually, these organs operate normally again. However, if the brain has been hurt, it might not entirely heal. The extent of the damage is typically based on how long the brain went without oxygen. HIE syndrome can have minor or moderate impacts on some children's health while leaving others with considerably more severe and long-lasting disabilities, such as developmental delay, cerebral palsy (motor impairment), epilepsy, or cognitive impairment.

Risk factors

The biggest risk factor for HIE is asphyxia. The occurrence and severity of hypoxic-ischemic encephalopathy depend on the degree and duration of oxygen deprivation. Asphyxia can result from a variety of factors, including but not restricted to:

1. Acute maternal hypotension

2. Blood containing less oxygen due to poorly functioning lungs

3. Cardiac complications

4. Injury from cephalopelvic disproportion

5. Injury from umbilical cord complications

6. Impaired blood flow to the brain during birth

7. Interruption in breathing or poor oxygen supply

Symptoms
Have a weak cry,Decreased alertness and activity,Lack of typical reflexes,Abnormal (not typical) movements or seizures,Low or high muscle tone,Breathing problems
Conditions
Uterine rupture and tear are two conditions that might lead to HIE syndrome in your child,Although HIE primarily affects the brain, reduced blood flow or oxygen levels can also have negative effects on the lungs, liver, heart, intestines, and kidneys,It may cause long-term incapacity and, regrettably, in rare circumstances
Drugs
Levetiracetam,Phenytoin,Lidocaine,Benzodiazepines,Phenobarbital

Is there a cure/medications for hie syndrome?

Hypoxia describes a decrease in the oxygen delivered to organs, including the brain. Ischemia describes a lack of blood flow to the organs. The word "encephalopathy" is used to denote any type of widespread brain malfunction. A shortage of oxygen and blood flow to the brain can result in brain dysfunction known as hypoxic-ischemic encephalopathy (or HIE). HIE syndrome is occasionally also referred to as birth asphyxia, but this term only applies to extremely specific subsets of newborns with brain damage. Full-term babies who have hypoxic-ischemic encephalopathy (HIE) are at considerable risk for death or severe disability by the time they turn two.

Unfortunately, there is no cure for HIE. Additionally, the severity of the illness can vary, which means that the available treatments can be very different. Fortunately, there are numerous therapies and treatments that can help children with HIE function better.

Treatment

1. Infants born with HIE require three days of chilling as part of immediate treatment. According to research, brain damage may be reduced if the brain is kept a few degrees below body temperature shortly after birth.

2. Some other treatments include supporting the heart and maintaining healthy blood pressure, sustaining kidney and liver function, and mechanical ventilation (breathing tube) if the baby is unable to breathe independently.

Symptoms
Have a weak cry,Decreased alertness and activity,Lack of typical reflexes,Abnormal (not typical) movements or seizures,Low or high muscle tone,Breathing problems
Conditions
Uterine rupture and tear are two conditions that might lead to HIE syndrome in your child,Although HIE primarily affects the brain, reduced blood flow or oxygen levels can also have negative effects on the lungs, liver, heart, intestines, and kidneys,It may cause long-term incapacity and, regrettably, in rare circumstances
Drugs
Levetiracetam,Phenytoin,Lidocaine,Benzodiazepines,Phenobarbital

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