ALL THINGS NICU

Harlequin Color Change

2011-06-27T01:55:00.000-07:00

Babies' blood vessels start off highly reactive and unstable. A mild change in temperature or position or mood can cause swift changes in the diameters of the blood vessels, with resultant color changes of the overlying skin. The most extraordinary example of this is the harlequin effect.

A sharp line from the forehead to the pubis divides the body into 2 vertical halves. One side turns dark red, the other quite pale. The overall effect is reminiscent of the bold patches of color on a harlequin costume.

This rare but dramatic event only occurs in the immediate newborn period. It usually begins when the baby is positioned on her side. The upper half of the body becomes pale, and the lower half deep red. Changing her position can reverse the pattern. If she moves a lot, the muscle activity will erase the color changes (rather like shaking an Etch-a-sketch toy).

The harlequin color change is most common in low birthweight infants, but can occur in any child. Babies who experience this once will often take on the harlequin pattern multiple times.

Still, the condition is as temporary as being a newborn. The harlequin color change is considered harmless and not associated with any permanent problem.

My Thoughts on.... "Will my baby know he/she is loved, even if I can't be there?"

2011-01-21T16:03:00.000-08:00

This is a question most NICU parents ask themselves, but may not actually ask their nurse.... And here's my thought:

I truly believe that babies know the spirit of the people around them. We've got more than 120 nurses in our unit alone, and although there are 120 different personalities with 120 different levels of interpersonal skills, one fact remains: We love our babies. The Good Lord knows moms and dads can't be with them all the time in the hospital; and I wholeheartedly believe that He takes care of that little minor detail. For even the least of His creatures of the field He provides for..... and I fully believe that babies know they are in the presence of a loving soul (be it their nurses, the doctors, or even the security guards that "ooh and ahh" over how cute the babies are during their nightly rounds....) it doesn't matter what loving spirit they are in the presence of, I believe they can feel it. Someone (much more powerful than us) will make sure our babies feel love and acceptance.... even when their parents are unable to be at their bedside.

Matthew 10:29-31 (New International Version)
29 Are not two sparrows sold for a penny? Yet not one of them will fall to the ground apart from the will of your Father. 30 And even the very hairs of your head are all numbered. 31 So don't be afraid; you are worth more than many sparrows.

Tetralogy of Fallot

2011-01-21T10:07:00.000-08:00

Tetralogy of Fallot / TOF is a cardiac anomaly that refers to a combination of four related heart defects that commonly occur together. The four defects include:

1. Pulmonary stenosis (narrowing of the pulmonary valve and outflow tract or area below the valve, that creates an obstruction (blockage) of blood flow from the right ventricle to the pulmonary artery
2. Ventricular septal defect / VSD
3. Overriding aorta (the aortic valve is enlarged and appears to arise from both the left and right ventricles instead of the left ventricle as occurs in normal hearts)
4. Right ventricular hypertrophy (thickening of the muscular walls of the right ventricle, which occurs because the right ventricle is pumping at high pressure)

A small percentage of children with tetralogy of Fallot may also have additional ventricular septal defects, an atrial septal defect / ASD or abnormalities in the branching pattern of their coronary arteries. Some patients with tetralogy of Fallot have complete obstruction to flow from the right ventricle, or pulmonary atresia. Tetralogy of Fallot may be associated with chromosomal abnormalities, such as 22q11 deletion syndrome.

The pulmonary stenosis and right ventricular outflow tract obstruction seen with tetralogy of Fallot usually limits blood flow to the lungs. When blood flow to the lungs is restricted, the combination of the ventricular septal defect and overriding aorta allows oxygen-poor blood ("blue") returning to the right atrium and right ventricle to be pumped out the aorta to the body.

This "shunting" of oxygen-poor blood from the right ventricle to the body results in a reduction in the arterial oxygen saturation so that babies appear cyanotic, or blue. The cyanosis occurs because oxygen-poor blood is darker and has a blue color, so that the lips and skin appear blue.

The extent of cyanosis is dependent on the amount of narrowing of the pulmonary valve and right ventricular outflow tract. A narrower outflow tract from the right ventricle is more restrictive to blood flow to the lungs, which in turn lowers the arterial oxygen level since more oxygen-poor blood is shunted from the right ventricle to the aorta.

Signs and Symptoms of Tetralogy of Fallot:
Tetralogy of Fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. Babies with tetralogy of Fallot usually have a patent ductus arteriosus at birth that provides additional blood flow to the lungs, so severe cyanosis is rare early after birth.

As the ductus arteriosus closes, which it typically will in the first days of life, cyanosis can develop or become more severe.

The degree of cyanosis is proportional to lung blood flow and thus depends upon the degree of narrowing of the outflow tract to the pulmonary arteries.

Rapid breathing in response to low oxygen levels and reduced pulmonary blood flow can occur. The heart murmur, which is commonly loud and harsh, is often absent in the first few days of life.

The arterial oxygen saturation of babies with tetralogy of Fallot can suddenly drop markedly. This phenomenon, called a "tetralogy spell," usually results from a sudden increased constriction of the outflow tract to the lungs so that pulmonary blood flow is further restricted. The lips and skin of babies who have a sudden decrease in arterial oxygen level will appear acutely more blue.

Children having a tetralogy spell will initially become extremely irritable in response to the critically low oxygen levels, and they may become sleepy or unresponsive if the severe cyanosis persists.

A tetralogy spell can sometimes be treated by comforting the infant and flexing the knees forward and upward. Most often, however, immediate medical attention is necessary.

Diagnosis of Tetralogy of Fallot:
When a newborn baby with significant cyanosis is first seen, they are often placed in supplemental oxygen. The increased oxygen improves the child's oxygen levels in cases of lung disease, but breathing extra oxygen will have little effect on the oxygen levels of a child with tetralogy of Fallot.

Failure to respond to this "hyperoxia test" is often the first clue to suspect a cyanotic cardiac defect. Infants with tetralogy of Fallot can have normal oxygen levels if the pulmonary stenosis is mild (referred to as "pink" tetralogy of Fallot). In these children, the first clue to suggest a cardiac defect is detection of a loud murmur when the infant is examined.

Once congenital heart disease is suspected, echocardiography can rapidly and accurately demonstrate the four related defects characteristic of tetralogy of Fallot.

Cardiac catheterization is occasionally required to evaluate the size and distribution of the pulmonary arteries and to clarify the branching patterns of the coronary arteries. Catheterization can also demonstrate whether patients have pulmonary blood flow supplied by an abnormal blood vessel from the aorta (aortopulmonary collateral).

Treatment for Tetralogy of Fallot:
Once tetralogy of Fallot is diagnosed, the immediate management focuses on determining whether the child's oxygen levels are in a safe range.

If oxygen levels are critically low soon after birth, a prostaglandin infusion is usually initiated to keep the ductus arteriosus open which will provide additional pulmonary blood flow and increase the child's oxygen level.

These infants will usually require surgical intervention in the neonatal period. Infants with normal oxygen levels or only mild cyanosis are usually able to go home in the first week of life.

Complete repair is usually done electively when the children are about six months of age, as long as the oxygen levels remain adequate. Progressive or sudden decreases in oxygen saturation may prompt earlier corrective repair.

Surgical correction of the defect is always necessary. Occasionally, patients will require a surgical palliative procedure prior to the final correction.

Corrective repair of tetralogy of Fallot involves closure of the ventricular septal defect with a synthetic Dacron patch so that the blood can flow normally from the left ventricle to the aorta.

The narrowing of the pulmonary valve and right ventricular outflow tract is then augmented (enlarged) by a combination of cutting away (resecting) obstructive muscle tissue in the right ventricle and by enlarging the outflow pathway with a patch.

In some babies, however, the coronary arteries will branch across the right ventricular outflow tract where the patch would normally be placed. In these babies an incision in this area to place the patch would damage the coronary artery so this cannot safely be done.

When this occurs, a hole is made in the front surface of the right ventricle (avoiding the coronary artery) and a conduit (tube) is sewn from the right ventricle to the bifurcation of the pulmonary arteries to provide unobstructed blood flow from the right ventricle to the lungs.

Treatment for Tetralogy of Fallot: Results
Survival of children with tetralogy of Fallot has improved dramatically over recent decades. In the absence of confounding risk factors, more than 95 percent of infants with tetralogy of Fallot successfully undergo surgery in the first year of life.

Surgical repair is more difficult when the pulmonary arteries are critically small or when the lung blood flow is supplied predominantly by aortopulmonary collaterals.

Most babies are fairly sick in the first few days after surgery, since the right ventricle is "stiff" from the previous hypertrophy (thickness) and because an incision is made into the muscle of the ventricle, making the muscle temporarily weaker.

This right ventricular dysfunction usually improves significantly in the days following surgery. Patients may also have rhythm problems after surgery.

An abnormally fast rhythm (called junctional tachycardia) may occur and may require treatment with medication or the use of a temporary pacemaker. This abnormal rhythm is usually temporary and the rhythm generally will return to normal as the right ventricle recovers.

Patients are also at risk for slow heart rates after surgery due to heart block. Heart block may be caused by injury to or inflammation of the conduction system in the heart. In many patients, the conduction improves and normal rhythm returns. Rarely, a permanent pacemaker may be necessary.

Since a normal circulation is produced by the tetralogy of Fallot repair procedure, long-term cardiac function is usually excellent.

However, the repair does usually leave the child with a leaky (insufficient) pulmonary valve. In this situation, after the right ventricle pumps blood out to the pulmonary arteries, some of the blood will flow back into the right ventricle. This creates extra volume in the right ventricle forcing it to work harder and become dilated.

In a small percentage of children, this pulmonary insufficiency can lead to diminished function of the right ventricle. Symptoms of fatigue, especially with exercise, may develop. In these cases, replacement of the pulmonary valve is often recommended.

Patients who have had repair of tetralogy of Fallot can also redevelop a narrowing at the outflow area or in the branch (left or right) pulmonary arteries, which will cause the right ventricle to pump at abnormally high pressures.

If these problems occur, surgical intervention to further widen the outflow tract or pulmonary arteries may be necessary. Narrowing the pulmonary arteries can sometimes be treated without surgery, with balloon dilation of the vessels during cardiac catheterization.

EXCELLENT GRAPHIC DESCRIPTION OF TOF FOUND HERE!

Full Article found Here

MECP2 Gene Duplication

2010-09-22T19:09:00.000-07:00

The following information was obtained from here.

The syndrome was first discovered in 2005.

The MECP2 Duplication Syndrome is usually caused by duplication of DNA on the Xq28 region of the chromosome. Most reported duplications are sub-microscopic (cannot be seen with a microscope by standard chromosome analysis) and span 0.3 to 4 megabases of DNA in size. Many cases of “functional disomy” of the Xq28 region (meaning an extra copy of the Xq28 region that occurs somewhere other than directly at Xq28) due to chromosome Xq-Yq translocation, chromosome Xq-Xp rearrangements, and chromosome X-autosomal chromosome translocations have also been reported. Many of these cases were reported before the name “MECP2 Duplication Syndrome” was assigned.

MECP2 Duplication Syndrome is most commonly inherited in an X-linked manner. Most affected males have inherited the MeCP2 duplication from a carrier mother, however, spontaneous (also known as de novo) duplications have been reported. If the mother has a MECP2 duplication, the chance of transmitting it in each pregnancy is 50%. In the case of de novo duplications, the possibility exists that the mother can have mosaicism and therefore only carry the duplicated X chromosome in her ova or egg cells (or only in some of these cells). Because ova or germ-line mosaicism cannot be ruled out in de novo cases, the risk to subsequent pregnancies in de novo cases is approximated to be about 5%. Because the duplication affects the X chromosome, MECP2duplication syndrome occurs in all males who have the duplication. In females who have symptoms, it is thought that the X chromosome with the duplicated allele is active in a number of cells (one copy of the X chromosome is turned off in every somatic cell in females, a normal process called X chromosome inactivation).

When MECP2 Duplication Syndrome results from a duplication that is present on the Y chromosome, or one of the autosomes (chromosomes 1-22), then it is important to assess if either parent is a carrier. To date, no cases of men transmitting the duplication have been reported. This is because, as far as we know, all boys/men who have the duplication have MECP2 Duplication Syndrome. Therefore, in the majority of boys who have the duplication syndrome due to the Xq28 duplication being present on the Y chromosome, the duplication event likely occurred spontaneously when the sperm developed in the father. Just like in females, however, men can have germ-line mosaicism, and so the risk to subsequent pregnancies in de novo cases is estimated to be 5%. If the Xq28 duplication is carried on one of the autosomes, then the duplication may be de novo, carried by the mother, or be a result of germ-line mosaicism.

The Xq28 region contains several genes, and one of these is MECP2(methyl-CpG binding protein 2). The beginning and end of the duplicated region (breakpoints) vary among different individuals, but the finding that MECP2 is the only duplicated gene in all patients with a significant role in the nervous system supports its important role in causing MECP2 Duplication Syndrome. Furthermore, genetically engineered mice that have twice the normal levels of MeCP2 protein develop the features of the duplication syndrome. These studies pinpoint increased levels of MeCP2 (rather than other proteins) as the culprit of this syndrome. This is why the syndrome is now called “MECP2Duplication Syndrome.”

It is important to note however, that some boys have larger duplications that include many other genes. The full extent of phenotypes due to duplication of other genes is not completely understood. We do know, however, that boys who also have duplication of the Filamin A (FLNA) gene are at risk for intestinal pseudo-obstruction and perhaps other phenotypes that have been associated with other types of mutations inFLNA. Therefore, it is helpful for all boys with MECP2 Duplication Syndrome to have a study to map the extent and gene content of their duplication. When detailed studies are performed, some boys are found to have triplication of Xq28 which appears to result in a syndrome that is more severe, especially when the MECP2 gene is included in the triplicated region. Finally, some cases of duplication of Xq28 actually have breakpoints (ends) that are located within the MECP2 gene. In these cases, it may be that disruption of one copy of the MECP2 gene, rather than duplication, causes the phenotype.

Characteristics of MECP2 duplication in affected boys:
*Hypotonia
*As a result of hypotonia, motor development including sitting, crawling, and walking is severely delayed or impaired
*Mental retardation (in 100%)
*Recurrent respiratory infections (in 75%)
*Epilepsy (in 50%)
*Constipation and/or reflux
*Limited or absent speech
*Autistic behaviors
*Ataxia
*Progressive spasticity (usually noticed in the legs more than the arms)
*Stereotyped movements of hands
*Teeth grinding
*Developmental regression occurs in some boys
Characteristics of FLNA duplication:
*Intestinal pseudo-obstruction
*Perhaps other problems

PLAYING FOR PREEMIES SEPTEMBER 24

2010-08-20T07:57:00.000-07:00

On Sept. 24th at the Clements/Ardmore football game, we are having "Playing For Preemies" Night. Ardmore High School Athletic Department is teaming up The Melissa George Neonatal Fund and The Huntsville Hospital Foundation for this event. We have some special things going on that week and that night. All proceeds go to the Neonatal Intensive Care Unit at Huntsville Hospital.

1st Event: We are going to pre-sell game t-shirts in the weeks leading up. (Grey shirt with Melissa George and game logo).

2nd Event: Four businesses to sponsor one quarter of the game for $250. Those 4 sponsors would get their logo on the back of the game t-shirts.

3rd: Amy George will be our special guest for the pep rally that day. High School Pep Rally will start at 2:15. All three administrators are going to take a pie in the face from 3 lucky students. Students can buy a chance to "Pie the Principal" for $1 during the week.

4th: Mr. Johns, Principal at Cedar Hill Elementary is taking a pie in the face too. Students can buy a chance to "Pie the Principal" for $1 during the week. We are doing a mini pep rally at Cedar Hill at 8:30 that morning to draw the winner.

5th: Dr. Casey Lewis, Principal at Johnson Elementary is taking a pie in the face too. Students can buy a chance to "Pie the Principal" for $1 during the week. We are doing a mini pep rally at Johnson at 9:30 that morning to draw the winner as well.

6th: Huntsville Hospital Foundation is setting up an informational and donation booth at the game that night. The NICU nurses will serve as honorary captains for the coin toss.

Special Invitation: You are all invited to participate in any and or all of these events. The NICU is very special to me and my wife as our 5 month old daughter spent the first 52 days of her life there. We are so grateful for the nurses and doctors that made our stay as comforting as it could be in such a stressful time. We are excited about the opportunity to give back to such a great group of people..... Rusty Bates, Assistant Principal/Athletic Director, Ardmore High School

Polydactyly or Extra Digits

2010-08-15T01:40:00.000-07:00

Polydactyly literally means "extra digits." There may be an extra thumb, small finger, or, less commonly, an extra digit in the central part of the hand. Polydactyly is one of the most common congenital hand anomalies.
Does polydactyly cause my baby any pain?
No, typically there is no pain associated with polydactyly.
What are the different types of polydactyly?
Radial, or pre-axial polydactyly means that there is an extra thumb; there are several different types of radial polydactyly. Ulnar, or post-axial polydactyly means that there is an extra small finger; there may be a well-formed extra small finger, or just a poorly-formed extra digit attached by a thin stalk of soft tissue. Central polydactyly means that the extra digit is in the central part of the hand, between the thumb and small finger.
Who gets polydactyly?
Polydactyly can occur in any newborn infant. Most types of radial polydactyly are not inherited. Postaxial polydactyly with a small, poorly-formed extra digit is ten times more common in African-Americans than in Caucasians and is inherited as an autosomal dominant trait (that is, there is a 50% chance of polydactyly in the children of an affected individual). However, postaxial polydactyly with a well-formed extra digit is equally common in all ethnicities. Central polydactyly is inherited as an autosomal dominant condition with variable expression, meaning that it may be more or less severe from one generation to the next.
What causes polydactyly?
When the hands and feet are developing in the womb, they start out as flat "paddles" that then normally separate into five digits. Polydactyly occurs when this separation process is excessive, and an extra "segment" is created. This may be caused by a genetic abnormality or by environmental influences.
What are the main issues related to polydactyly?
The primary issue in most types of polydactyly is function of the hand and digits; appearance of the hand is also an issue, but is secondary to function.
Are there other problems that occur commonly with polydactyly?
Certain rare types of preaxial polydactyly are associated with other problems, such as blood disorders, heart abnormalities, or craniofacial abnormalities. Postaxial polydactyly in which the extra digit is well-formed is associated with polydactyly of the feet, also.
What is the treatment for babies with polydactyly?
Polydactyly is treated surgically. In preaxial polydactyly, a single thumb must be reconstructed from the two duplicated, or split, thumbs. This procedure involves reconstructing the skin and soft tissues, the tendons, joints, and ligaments to create a single thumb. In postaxial polydactyly, when the extra digit is attached only by a narrow stalk of soft tissue, this may be removed either with a minor operation or, if the stalk is narrow enough, by ligating the stalk in the nursery. When the extra digit is well-formed, the surgery is more involved and may involve reconstruction of soft tissues, tendons, joints, and ligaments as in preaxial polydactyly. Finally, central polydactyly requires a complex surgical procedure to reconstruct the hand. Again, the soft tissues, tendons, ligaments, and joints must be reconstructed. In some of these cases, more than one operation is required.

CONGENITAL CHYLOTHORAX

2010-07-16T20:54:00.000-07:00

Congenital Chylothorax

Congenital Chylothorax (CC) is the accumulation of lymphatic fluid in the pleural space around the lungs. CC is usually a result of blockage of the thoracic duct during pregnancy. Infants with CC receiving regular formula feedings have persistent accumulations of fluid around the lungs because the production of lymphatic fluid is increased tenfold following regular formula feedings.

TREATMENT
Mechanical Ventilation to help the infant's lungs expand fully.
Thoracenteses which is removing the fluid from around the lungs.
Inserting a chest tube for drainage may be needed in some cases with respiratory distress.
Special Formulas (such as Enfaport) that consists of low-fat high-protein milk supplemented with MCT oil is used if the infant is able to take nutrition by mouth, or
IV nutrition combined with drainage of the space around the lungs is utilized.

Hopefully, with conservative treatment, the accumulation of fluid around the lungs resolves on its own, if not, surgical interventions will be considered.

SURGICAL CONSIDERATIONS
Surgical interventions might be considered if the chylothorax does not resolve or becomes worse. Surgical interventions are not risk free and not always successful, especially when associated with a malformed thoracic duct or a thoracic duct that does not function properly, or if infection is involved.

Kawasaki Disease

2010-05-08T20:46:00.000-07:00

Kawasaki disease (KD), also known as Kawasaki syndrome, is a serious illness characterized by inflammation of blood vessels throughout the body that primarily affects young children and infants. Kawasaki disease is the leading cause of acquired heart disease in children. Although about 80 percent of patients are under five years of age, older children and teenagers can also get KD, but this is uncommon. KD is more common in boys than girls, and the majority of cases are diagnosed in the winter and early spring. It is not contagious.

The disease is named after Tomisaku Kawasaki, a Japanese pediatrician who first described the illness in the medical literature in 1967. Although it is more prevalent among children of Asian and Pacific Island descent, KD affects people of all racial and ethnic groups. It is estimated that more than 4,200 children are diagnosed with Kawasaki Disease in the U.S. each year. The cause of KD is unknown, although an agent, like a virus, is suspected. There is no currently accepted scientific evidence that KD is caused by carpet cleaning or chemical exposure.

How is it treated?
Treatment for Kawasaki disease starts in the hospital. It may include:

Immunoglobulin (IVIG) medicine. This is given through a vein (intravenous, or IV) to reduce inflammation of the blood vessels.
Aspirin to help pain and fever and to lower the risk of blood clots.
Aspirin therapy is often continued at home. Because of the risk of Reye syndrome, do not give aspirin to your child without talking to your doctor. If your child is exposed to or develops chickenpox or flu (influenza) while taking aspirin, talk with your doctor right away.

How serious is Kawasaki disease?
Most children with Kawasaki disease get better and have no long-term problems. Treatment is important because it shortens the illness and reduces the chances of problems.

Some children who are not treated will have damage to the coronary arteries. An artery may get too large and form an aneurysm. Or the arteries may narrow or develop blood clots. A child who has damaged coronary arteries may be more likely to have a heart attack as a young adult.

Kawasaki Disease is characterized by an inflammation of the blood vessels throughout the body. There is no specific test for KD; doctors make a clinical diagnosis based on a collection of symptoms and physical findings. Early symptoms of KD include:
Fever that lasts for five or more days
Rash, often worse in the groin area
Red bloodshot eyes, without drainage or crusting
Bright red, swollen, cracked lips, “strawberry” tongue, which appears with shiny bright red spots after the top coating sloughs off
Swollen hands and feet and redness of the palms and soles of the feet
Swollen lymph nodes in the neck
Understandably, children with these symptoms are extremely uncomfortable and irritable. Any parent whose child has persistent fever and any of these symptoms should take him or her to the doctor immediately.

Without treatment, about 25% of children develop heart disease involving the coronary arteries. Timely diagnosis and treatment (which usually includes intravenous gamma globulin) is highly effective in preventing coronary complications. Doctors continue to study the long-term outcome of children who do not appear to have coronary involvement. Other kinds of longer-term consequences (e.g., non-coronary) are extremely rare. There is no evidence that links KD with autism or a seizure disorder. A very small number of KD children might have a seizure in the early acute stage of KD when there are very high fevers, but there is no on-going or long term seizure prone condition.

Newborn's Conversation with God

2009-11-30T14:30:00.000-08:00

A Newborn's Conversation with God

A baby asked God, "They tell me you are sending me to earth tomorrow, but how am I going to live there being so small and helpless?"

God said, "Your angel will be waiting for you and will take care of you."

The child further inquired, "But tell me, here in heaven I don't have to do anything but sing and smile to be happy.."

God said, "Your angel will sing for you and will also smile for you. And you will feel your angel's love and be very happy."

Again the small child asked, "And how am I going to be able to understand when people talk to me if I don't know the language?"

God said, "Your angel will tell you the most beautiful and sweet words you will ever hear, and with much patience and care, your angel will teach you how to speak."

"And what am I going to do when I want to talk to you?"

God said, "Your angel will place your hands together and will teach you how to pray."

"Who will protect me?"

God said, "Your angel will defend you even if it means risking its life."

"But I will always be sad because I will not see you anymore."

God said, "Your angel will always talk to you about Me and will teach you the way to come back to Me, even though I will always be next to you."

At that moment there was much peace in Heaven, but voices from Earth could be heard and the child hurriedly asked, "God, if I am to leave now, please tell me my angel's name."

God said, You will simply call her, " Mom ."

Temperature, Weight, and Length Conversions

2009-05-19T22:39:00.000-07:00

Temperature Conversion Chart

Weight Conversion Chart

Wolff-Parkinson-White syndrome

2009-04-29T21:51:00.001-07:00

What is Wolff-Parkinson-White syndrome?

Wolff-Parkinson-White syndrome is a condition characterized by abnormal electrical pathways in the heart that cause a disruption of the heart's normal rhythm (arrhythmia).

The heartbeat is controlled by electrical signals that move through the heart in a highly coordinated way. A specialized cluster of cells called the atrioventricular node conducts electrical impulses from the heart's upper chambers (the atria) to the lower chambers (the ventricles). Impulses move through the atrioventricular node during each heartbeat, stimulating the ventricles to contract slightly later than the atria.

People with Wolff-Parkinson-White syndrome are born with an extra connection in the heart, called an accessory pathway, that allows electrical signals to bypass the atrioventricular node and move from the atria to the ventricles faster than usual. The accessory pathway may also transmit electrical impulses abnormally from the ventricles back to the atria. This extra connection can disrupt the coordinated movement of electrical signals through the heart, leading to an abnormally fast heartbeat (tachycardia) and other arrhythmias. Resulting symptoms include dizziness, a sensation of fluttering or pounding in the chest (palpitations), shortness of breath, and fainting (syncope). In rare cases, arrhythmias associated with Wolff-Parkinson-White syndrome can lead to cardiac arrest and sudden death. The most common arrhythmia associated with Wolff-Parkinson-White syndrome is called paroxysmal supraventricular tachycardia.

Complications of Wolff-Parkinson-White syndrome can occur at any age, although some individuals born with an accessory pathway in the heart never experience any health problems associated with the condition.

Wolff-Parkinson-White syndrome often occurs with other structural abnormalities of the heart or underlying heart disease. The most common heart defect associated with the condition is Ebstein anomaly, which affects the valve that allows blood to flow from the right atrium to the right ventricle (the tricuspid valve). Additionally, Wolff-Parkinson-White syndrome can be a component of several other genetic syndromes, including hypokalemic periodic paralysis (a condition that causes episodes of extreme muscle weakness), Pompe disease (a disorder characterized by the storage of excess glycogen), and tuberous sclerosis (a condition that results in the growth of noncancerous tumors in many parts of the body).

How common is Wolff-Parkinson-White syndrome?

Wolff-Parkinson-White syndrome affects 1 to 3 in 1,000 people worldwide. Only a small fraction of these cases appear to run in families.

Wolff-Parkinson-White syndrome is a common cause of an arrhythmia known as paroxysmal supraventricular tachycardia. Wolff-Parkinson-White syndrome is the most frequent cause of this abnormal heart rhythm in the Chinese population, where it is responsible for more than 70 percent of cases.

What genes are related to Wolff-Parkinson-White syndrome?

Mutations in the PRKAG2 gene cause Wolff-Parkinson-White syndrome.

A small percentage of all cases of Wolff-Parkinson-White syndrome are caused by mutations in the PRKAG2 gene. Some people with these mutations also have features of hypertrophic cardiomyopathy, a form of heart disease that enlarges and weakens the heart (cardiac) muscle. The PRKAG2 gene provides instructions for making a protein that is part of an enzyme called AMP-activated protein kinase (AMPK). This enzyme helps sense and respond to energy demands within cells. It is likely involved in the development of the heart before birth, although its role in this process is unknown.

Researchers are uncertain how PRKAG2 mutations lead to the development of Wolff-Parkinson-White syndrome and related heart abnormalities. Research suggests that these mutations alter the activity of AMP-activated protein kinase in the heart, although it is unclear whether the genetic changes overactivate the enzyme or reduce its activity. Studies indicate that changes in AMP-activated protein kinase activity allow a complex sugar called glycogen to build up abnormally within cardiac muscle cells. Other studies have found that altered AMP-activated protein kinase activity is related to changes in the regulation of certain ion channels in the heart. These channels, which transport positively charged atoms (ions) into and out of cardiac muscle cells, play critical roles in maintaining the heart's normal rhythm.

In most cases, the cause of Wolff-Parkinson-White syndrome is unknown.

How do people inherit Wolff-Parkinson-White syndrome?

Most cases of Wolff-Parkinson-White syndrome occur in people with no apparent family history of the condition. These cases are described as sporadic and are not inherited.

Familial Wolff-Parkinson-White syndrome accounts for only a small percentage of all cases of this condition. The familial form of the disorder typically has an autosomal dominant pattern of inheritance, which means one copy of the altered gene in each cell is sufficient to cause the condition. In most cases, a person with familial Wolff-Parkinson-White syndrome has inherited the condition from an affected parent.

Oral Candidiasis - Thrush

2009-04-08T15:04:00.000-07:00

Candida albicans accounts for about 90% of human yeast infections.

Epidemiololgy

Candida albicans is a common inhabitant in normal adult gastrointestinal tracts and vaginas. During pregnancy, the colonization rate may be 33%.
10% of normal infants are colonized in the GI and respiratory tract at 5 days of age and this rate increases in preterm infants. The skin is usually colonized at 2 weeks of age.
The neonate acquires the yeast from the mother at the time of delivery.
Thrush usually appears about 1 week of age and diaper dermatitis at 3-4 months.
Thrush is a disease of infants, and in older children it is associated with the use of antibiotics.
Recurrent or persistent thrush should raise the suspicion of a possible underlying immunodeficiency syndrome.

Clinical presentation

White curdish like lesions on the buccal mucosa, tongue, palate, and gingiva. The lesions are difficult to scrape off and this differentiates it from milk. After scraping, there is an erythematous base and some bleeding.
Symptoms- usually asymptomatic and no interference with eating. Rarely, may develop esophagitis in an immunocompetent hosts and feeding may be decreased.
Esophagitis due to Candida infection may present with chest pain

Treatment

Untreated, will resolve in 3-8 weeks but in most instances antifungal agents are used. Mild cases may be watched without treatment.
Nystatin suspension. Various reports have reported cure rates between 50-80% on standard 100,000 units per dose 4 times a day. May have to double the dose or apply it directly to the lesions with a cotton swab. Nystatin does not adhere well to the lesions and is swallowed rapidly and this interferes with its efficacy.
In nursing mothers, the breast may be a reservoir for the yeast so that the application of a topical antifungal between feeds to the breast may help eradicate the infection.
Gentian violet has been shown to be effective but is messy to use.
Miconazole
Always check for diaper dermatitis because often associated with oral monilia infection

Hearing Screening

2009-03-31T21:23:00.000-07:00

Early Newborn Hearing Screening Leads to Early Detection and Intervention for Hearing Loss

Posted 03/25/2009

Marilyn W. Edmunds, PhD, CRNP; Laurie E. Scudder, MS, NP

Biernath K, Holstrum WJ, Eichwald J
J Midwifery Womens Health. 2009;54:18-26

Article Summary

A variety of conditions can cause loss of hearing in a newborn infant. More than half of profound congenital hearing loss is believed to be genetic in origin. For every child with a profound hearing loss, there are an estimated 1 or 2 children with lesser but clinically significant bilateral or unilateral loss. Genetic deafness is usually caused either by the possession of a single gene (dominant deafness) or 2 genes (recessive deafness). Genetic causes of hearing loss can be categorized as syndromic (having symptoms in addition to hearing loss, such as blindness or heart defects) or nonsyndromic (having no additional symptoms). The majority of genetic cases of hearing loss are nonsyndromic, autosomal recessive. The major environmental (nongenetic) cause of hearing loss is congenital cytomegalovirus (CMV) infection, a condition that occurs in 1 in 150 live births. The risk for permanent disability is highest when a woman becomes infected with CMV during the first trimester of pregnancy. Infection control practices to prevent exposure to CMV must be followed by all women who are pregnant or trying to become pregnant.

Universal newborn hearing screening is becoming the standard of care in the United States. The US Preventive Services Task Force (USPSTF) reported in July 2008 that there is scientific evidence to recommend newborn hearing screening for all infants, updating their statement of 2001.^[1] Universal newborn hearing screening is the first step in the national Early Hearing Detection and Intervention (EHDI) program.

The Joint Committee on Infant Hearing Loss 2007: Principles of EHDI state^[2]:

All infants should have access to hearing screening using a physiologic measure before 1 month of age.
All infants who do not pass the initial hearing screen and the subsequent rescreening should have appropriate audiologic and medical evaluations, to confirm the presence of hearing loss, before 3 months of age.
All infants with confirmed permanent hearing loss should receive intervention services before 6 months of age. A simplified, single point of entry into an intervention system appropriate to children with hearing loss is optimal.
The EHDI system should be family-centered with infant and family rights and privacy guaranteed through informed choice, shared decision making, and parental consent. Families should have access to information about all intervention and treatment options and counseling with regard to hearing loss.
The child and family should have immediate access to high-quality technology, including hearing aids, cochlear implants, and other assistive devices when appropriate.
All infants and children should be monitored for hearing loss in the medical home. Continued assessment of communication development should be provided by appropriate providers to all children with or without risk indicators for hearing loss.
Appropriate interdisciplinary intervention programs for deaf and hard-of-hearing infants and their families should be provided by professionals who are knowledgeable about childhood hearing loss. Intervention programs should recognize and build on strengths, informed choices, traditions, and cultural beliefs of the families.
Information systems should be designed to interface with electronic health records and should be used to measure outcomes and report the effectiveness of EHDI services at the community, state, and federal levels.

Viewpoint

The goal of newborn hearing screening is to identify hearing problems early so that infants will develop the best communication and language skills possible. Midwives and other obstetric providers should educate women in regard to CMV infection, its possible effects on the newborn, and infection control practices to reduce the risk of acquiring CMV during pregnancy. This information is particularly important for women who are planning to become pregnant.

FULL ARTICLE HERE

PREEMIE PARTY 2009

2009-03-28T22:10:00.000-07:00

CCHS: Congenital Central Hypoventilation Syndrome or Ondine Curse

2009-03-16T20:04:00.000-07:00

Congenital central hypoventilation syndrome (CCHS) (formerly known as 'The Ondine Curse' after the mythical story - see below) is a disorder of the automatic control of breathing. In CCHS, the body "forgets" to breathe, especially during sleep. Researchers have identified a particular gene defect (PHOX2B) associated with congenital central hypoventilation syndrome, but how the mutation causes the syndrome is not yet known. It is also not known how frequently CCHS occurs, but it is estimated to be a rare disorder.

Symptoms
Infants born with CCHS may have symptoms ranging from mild to severe, including:

Poor breathing or complete lack of spontaneous breathing, especially during sleep
Abnormal pupils (70% of individuals)
Feeding difficulty due to acid reflux and decreased intestinal motility
An intestinal disorder called Hirschsprung disease (20% of individuals), in which part of the large intestine has no nerves.

Diagnosis
In order to be diagnosed with congenital central hypoventilation syndrome, an individual must have the following:

Persistent evidence of poor breathing during sleep
Symptoms begin during the first year of life
There are no other respiratory or muscular conditions that could account for the breathing difficulty
There is no evidence of heart disease.

Some infants with CCHS may be mistakenly thought to have heart defects, or to have another type of breathing problem. Unfortunately, some infants with unrecognized CCHS may die and be thought to have had SIDS (Sudden Infant Death Syndrome).

Treatment
If an infant is suspected to have congenital central hypoventilation syndrome, a sleep study is performed to determine how severe the breathing difficulty is. Other special tests of respiratory function can be done as well. Complete cardiac and neurological examinations are done to rule out any other type of disorder. Early diagnosis and treatment are important to prevent serious complications caused by periods of low or no oxygen.

Treatment focuses on providing breathing support, usually through the use of a respirator (ventilator). Some children with CCHS will need a ventilator 24 hours per day; others may only need breathing help when sleeping. In some individuals, a surgical implant in the diaphragm muscle can allow electrical stimulation of the muscle to control breathing.

Children with CCHS are able to lead active lives. They do, however, need close supervision while swimming or playing in pools, since their bodies may "forget" to breathe while underwater. CCHS is a lifelong condition, but with proper treatment it does not alter life expectancy.

This condition's name is a reference to the myth of "Ondine," a water nymph who had an unfaithful mortal lover. He swore to her that his "every waking breath would be a testimony of [his] love", and upon witnessing his adultery, she cursed that if he should fall asleep, he would forget to breathe. Eventually, he fell asleep from sheer exhaustion, and his breathing stopped.

Meckel's Diverticulum

2009-03-07T08:00:00.000-08:00

A Meckel’s diverticulum is a small pouch of tissue on the intestine (bowel). It forms when the baby is still growing in the womb. A Meckel’s diverticulum may bleed. It may also become infected. In either case, it must be removed.

What Are the Symptoms of Meckel’s Diverticulum?
Many people with a Meckel’s diverticulum never have symptoms. When a problem does occur, it’s often around age 2. The most common signs of a problem include:

Blood in stool
Anemia (a health problem due to blood loss).
Signs of infection (fever, chills, or pain or tenderness in the abdomen)

How Is Meckel’s Diverticulum Diagnosed?
Most Meckel’s aren’t found unless they cause symptoms. If a Meckel’s is suspected, tests that may be done include:

Blood tests: These check for signs of bleeding or infection.
Stool sample: This may be taken to check for blood.
Meckel’s scan: A special dye is injected into the child’s bloodstream through an IV (intravenous) line. This dye may make the Meckel’s tissue show up on a scan.
Ultrasound: This test uses sound waves to make images. In some cases, a Meckel’s can be seen on an ultrasound image.
Other tests: Imaging tests such as an x-ray or CT scan may be done to rule out other problems.

How Is a Meckel’s Diverticulum Treated?
If the child has no symptoms, treatment might not be needed. But if the Meckel’s diverticulum is causing symptoms, it will likely be removed with surgery.

What Are the Long-Term Concerns?
Unless it causes symptoms, a Meckel’s usually isn’t a problem. Once the diverticulum is removed, most children have no further symptoms.

NRP Algorithm

2009-02-02T22:11:00.000-08:00

FOUND HERE.

Keppra Use in Neonatal Seizures

2009-02-02T22:06:00.000-08:00

Neonatal seizures are a common problem in the NICU and are associated with severe morbidity and mortality. A seizure is a symptom of a neurological dysfunction and not a disease itself. Seizures result from excessive simultaneous electrical discharge or depolarization of neurons. Most neonatal seizures are sub clinical with no visible signs. Causes include ischemia, hypoxia, hypoglycemia, hypo or hypernatremia, among other metabolic disorders; structural causes from IVH or trauma; intracerebral meningitis; drug withdrawal; and genetics. The main prediction of outcome of seizure is the underlying etiology. Those with hypoxic ischemic encephalopathy (HIE) or major brain malformations do worse than those with transient hypoglycemia or small subarachnoid hemorrhage. There is debate as to whether neonatal seizures are harmful, however, animal studies suggest they are indeed harmful. According to these studies a strong increase in damage to the neonatal (rat) brain associated with seizures was noted when the brain had been exposed to HI, and seizures accelerated anoxia induced death in rat hippocampus. In a human infant study, 68 infants with risk for seizures were monitored. Their baseline etiologies, Apgars, blood gasses, etc., were all similar, but their baseline EEG’s were not. The group that went on to seize had more abnormality of the background EEG. The occurrence of electrographic seizure activity (ESz) was correlated with micronencephaly, severe cerebral palsy and failure to thrive. Also those with the greatest number of ESz were more likely to have these severe outcomes. Data suggests an association between the amount of ESz and subsequent mortality and morbidity in at-risk infants in general and infants with perinatal asphyxia. Therefore, seizure detection is extremely important. But, currently detection rates for clinically apparent seizures are 52% inaccurate. 50 to 90% of all neonatal seizures are sub clinical with absolutely no clinical signs detected by experts.

Treatment: Phenobarbital and phenytoin are still the drugs of choice but are only about 45% effective at eliminating ESz individually and 57% effective when used together. Side effects include hypotension, apnea, and other common AED side effects. Recent rat data suggest that Phenobarbital, Phenytoin and benzodiazepines may themselves accelerate apoptotic (cell death) neurodegeneration in the developing brain. New drug treatments in the pipeline include Topamax, Levetiracetam, Lidocaine, Bumatenide (+Phenobarbital). Of these, a recently released IV formulation of Levetiracetam (Keppra) is a good candidate for treatment of neonatal seizures. It has been determined to be safe, have no drug interactions, is neuroprotective, and does not cause neuronal apoptosis in animals.

Hypoglycemia

2009-01-12T20:38:00.001-08:00

What is hypoglycemia?

Hypoglycemia is a condition in which the amount of blood glucose (sugar) in the blood is lower than normal.

What causes hypoglycemia?

Hypoglycemia may be caused by conditions that:

lower the amount of glucose in the bloodstream
prevent or lessen storage of glucose
use up glycogen stores (sugar stored in the liver)
inhibit the use of glucose by the body

Many different conditions are associated with hypoglycemia, including the following:

inadequate maternal nutrition in pregnancy
excess insulin produced in a baby of a diabetic mother
severe hemolytic disease of the newborn (incompatibility of blood types of mother and baby)
birth defects and congenital metabolic diseases
birth asphyxia
cold stress (conditions that are too cold)
liver disease

Who is affected by hypoglycemia?

Babies who are more likely to develop hypoglycemia include:

Babies born to diabetic mothers may develop hypoglycemia after delivery when the source of glucose (the mother's blood) is gone and the baby's insulin production metabolizes the existing glucose.
Small for gestational age or growth-restricted babies may have too few glycogen stores.
Premature babies, especially those with low birthweights, who often have limited glycogen stores (sugar stored in the liver) or an immature liver function.

Why is hypoglycemia a concern?

The brain depends on blood glucose as its main source of fuel. Too little glucose can impair the brain's ability to function. Severe or prolonged hypoglycemia may result in seizures and serious brain injury.

What are the symptoms of hypoglycemia?

Symptoms of hypoglycemia may not be obvious in newborn babies. The following are the most common symptoms of hypoglycemia. However, each baby may experience symptoms differently. Symptoms may include:

jitteriness
cyanosis (blue coloring)
apnea (stopping breathing)
hypothermia (low body temperature)
poor body tone
poor feeding
lethargy
seizures

The symptoms of hypoglycemia may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis.

How is hypoglycemia diagnosed?

A simple blood test for blood glucose levels can diagnose hypoglycemia. Blood may be drawn from a heel stick, with a needle from the baby's arm, or through an umbilical catheter (a tube placed in the baby's umbilical cord). Generally, a baby with low blood glucose levels will need treatment.

Treatment for hypoglycemia:

Specific treatment for hypoglycemia will be determined by your baby's physician based on:

your baby's gestational age, overall health, and medical history
extent of the disease
your baby's tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
your opinion or preference

Treatment includes giving the baby a rapid-acting source of glucose. This may be as simple as giving a glucose/water mixture or formula as an early feeding. Or, the baby may need glucose given intravenously. The baby's blood glucose levels are closely monitored after treatment to see if the hypoglycemia occurs again.

Prevention of hypoglycemia:

There may not be any way to prevent hypoglycemia, only to watch carefully for the symptoms and treat as soon as possible. Mothers with diabetes with blood glucose levels in tight control can help minimize the amount of glucose that goes to the fetus.

Persistent pulmonary hypertension (PPHN)

2009-01-12T20:36:00.000-08:00

What is persistent pulmonary hypertension (PPHN)?

Persistent pulmonary hypertension (PPHN) is also known as persistent fetal circulation. In this condition, a newborn baby's circulation changes back to the circulation of a fetus, where much of the blood flow bypasses the lungs.

Who is affected by persistent pulmonary hypertension?

About one in every 500 to 700 babies develops PPHN. It occurs most often in full-term or post-term babies after a difficult birth, or birth asphyxia (a condition that results from too little oxygen).

What causes persistent pulmonary hypertension?

Click Image to Enlarge

During pregnancy, the fetal lungs are not used to exchange oxygen and carbon dioxide, so the lungs need less blood supply. The fetal circulation sends most of the blood supply away from the lungs through special connections in the heart and the large blood vessels. When a baby begins to breathe air at birth, this fetal circulation changes dramatically. The change in pressure in the lungs helps close the fetal connections and redirect the blood flow. Now blood is pumped to the lungs to help with the exchange of oxygen and carbon dioxide.

When a baby has lowered oxygen levels or difficulty breathing at birth, these changes may not occur and the baby's circulation returns back to the fetal system with blood directed away from the lungs. The lung pressure stays high. That is why this condition is called persistent pulmonary hypertension.

Why is persistent pulmonary hypertension a concern?

When blood is shunted away from the baby's lungs, it is difficult for the lungs to do the work of exchanging oxygen and carbon dioxide. Even breathing air with 100 percent oxygen, babies with PPHN have low blood oxygen levels. This can be serious, as all of the body's organs are dependent on oxygen-rich blood being pumped to them and may become damaged from lack of oxygen.

What are the symptoms of persistent pulmonary hypertension?

The following are the most common symptoms of persistent pulmonary hypertension. However, each baby may experience symptoms differently. Symptoms may include:

baby appears ill at delivery or in first hours after birth
cyanosis (blue coloring)
rapid breathing
rapid heart rate
low blood oxygen levels while receiving 100 percent oxygen

The symptoms of PPHN may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis.

How is persistent pulmonary hypertension diagnosed?

In addition to a complete medical and delivery history and physical examination at birth, diagnostic procedures for PPHN may include:

x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
blood tests
oxygen levels (when checked at different places on the baby's body, levels can be compared to see if the blood is being circulated to the lungs)

Treatment for persistent pulmonary hypertension:

Specific treatment for persistent pulmonary hypertension will be determined by your baby's physician based on:

your baby's gestational age, overall health, and medical history
extent of the condition
your baby's tolerance for specific medications, procedures, or therapies
expectations for the course of the condition
your opinion or preference

Treatment may include:

supplemental oxygen (giving 100 percent oxygen by a mask or plastic hood)
placing an endotracheal tube into the baby's windpipe (ET tube)
mechanical breathing machine (to do the work of breathing for the baby)
medications (to completely relax the baby's muscles and reflexes so that he/she will better respond to the mechanical ventilator)
inhalation of nitric oxide (to help dilate the blood vessels in the lungs)
extracorporeal membrane oxygenation (ECMO) - a special technique that may be used when a baby does not respond to maximum medical care. With ECMO, blood from the baby's veins is pumped through an artificial lung where oxygen is added and carbon dioxide is removed. The blood is then returned back to the baby. ECMO is only used in specialized NICUs.

Treatment of PPHN is aimed at increasing the oxygen to the rest of the body systems. Long-term health problems may be related to damage from lowered oxygen in the body.

Periventricular leukomalacia (PVL)

2009-01-12T20:35:00.000-08:00

What is periventricular leukomalacia (PVL)?

Periventricular leukomalacia (PVL) is damage and softening of the white matter, the inner part of the brain that transmits information between the nerve cells and the spinal cord as well as from one part of the brain to another.

"periventricular" means around or near the ventricles, the spaces in the brain containing the cerebrospinal fluid
"leuko" means white
"malacia" means softening

Why is periventricular leukomalacia a concern?

With PVL, the area of damaged brain tissue can affect the nerve cells that control motor movements. As the baby grows, the damaged nerve cells cause the muscles to become spastic, or tight, and resistant to movement. Babies with PVL have a higher risk of developing cerebral palsy (a group of disorders that prevent the child from controlling their muscles normally), and may have intellectual or learning difficulties.

PVL may occur alone or in addition to intraventricular hemorrhage (bleeding inside the brain).

What causes periventricular leukomalacia?

It is not clear why PVL occurs. This area of the brain is very susceptible to injury, especially in premature babies, whose brain tissues are fragile. PVL may happen when the brain receives too little oxygen. However, it is not clear when the trigger for PVL occurs - before, during, or after birth. Most babies who develop PVL are premature, especially those born before 30 weeks gestation. Other factors that may be associated with PVL include early rupture of membranes (amniotic sac) and infection inside the uterus.

What are the symptoms of periventricular leukomalacia?

PVL may not be apparent until later months. Each baby may experience symptoms differently. The most common symptom of PVL is spastic diplegia, tight, contracted muscles, especially in the legs. Symptoms of PVL may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis.

How is periventricular leukomalacia diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for PVL may include:

cranial ultrasound, a painless test that uses sound waves to view the baby's brain through the fontanelles, the soft openings between the skull bones. With PVL, the ultrasound shows cysts or hollow places in the brain tissue.
magnetic resonance imaging (MRI). This test uses a combination of a large magnet, radio frequencies, and a computer to produce detailed images of internal structures. MRI may show some of the early changes in the brain tissue that occur with PVL.

Treatment for periventricular leukomalacia:

There are no treatments for PVL. Management of the problems that can result from PVL will be determined by your baby's physician based on:

your baby's gestational age, overall health, and medical history
extent of the condition
your baby's tolerance for specific medications, procedures, or therapies
expectations for the course of the condition
your opinion or preference

Babies at risk for PVL may need special care after discharge from the hospital. Follow-up may include physical therapy, occupational therapy, and speech therapy.

Group B streptococcus (GBS)

2009-01-12T20:31:00.001-08:00

What is group B streptococcus?

Group B streptococcus (GBS) is a bacterium that can be found in the digestive tract, urinary tract, and genital area of adults. Although GBS infection usually does not cause problems in healthy women before pregnancy, it can cause serious illness for the mother and baby during pregnancy and after delivery.

Why is group B streptococcus a concern?

One out of every four or five pregnant women carries GBS in her rectum or vagina. In the pregnant mother, GBS infection may cause chorioamnionitis (a severe infection of the placental tissues) and postpartum (after birth) infection. Urinary tract infections caused by GBS can lead to preterm labor and birth.

Newborns can contract GBS during pregnancy, or from the mother's genital tract during labor and delivery. GBS is the most common cause of life-threatening infections in newborns, including pneumonia and meningitis. About one out of every 100 to 200 babies whose mothers carry GBS develop symptoms of GBS disease. Nearly 75 percent of the cases of GBS disease among newborns occur in the first week of life, called early-onset disease. Premature babies are more susceptible to GBS infection than full-term babies.

Although it is very rare, GBS infection may also develop in babies one week to several months after birth, called late-onset disease. Meningitis is more common with late-onset GBS disease. Only about half of late-onset GBS disease among newborns comes from a mother who is a GBS carrier. The source of infection for others with late-onset GBS disease is unknown.

How is group B streptococcus diagnosed?

GBS can be cultured from the mother's vagina or rectum with a swab during a pelvic examination. GBS can also be cultured from a mother's urine. Cultures are usually done between 35 and 37 weeks of pregnancy and may take a few days to complete. Cultures collected earlier in pregnancy do not accurately predict whether a mother will have GBS at delivery.

In infants, GBS may be cultured from samples of sterile body fluids, such as blood or spinal fluid. Most newborns with GBS infection have symptoms in the first few hours after birth. Symptoms are related to the body systems that are affected by the infection and may include breathing problems, changes in blood pressure, or neurologic problems such as seizures.

Treatment for group B streptococcus:

Treatment of GBS depends on when GBS infection is diagnosed - during pregnancy or after delivery. Specific treatment for GBS will be determined by your physician based on:

the pregnancy and birth history, overall health, and medical history
extent of the disease
your baby's tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
your opinion or preference

If the mother has a positive GBS culture during pregnancy, the Centers for Disease Control and Prevention (CDC) and the American College of Obstetricians and Gynecologists (ACOG) recommend treatment with intravenous (IV) antibiotics during labor to reduce the risk of transmission of the infection to the baby. Treatment may also be needed for women with certain risk factors, including the following:

fever during labor
rupture of membranes (bag of waters) for 18 hours or longer
labor or rupture of membranes before 37 weeks gestation
history of GBS infection in a previous baby

Newborns who become ill with GBS infection may require care in the newborn intensive care unit (NICU). They are usually treated with intravenous (IV) antibiotics. Other treatments and specialized care may be needed depending on the severity of the infection and whether the infection causes serious problems such as meningitis or pneumonia.

Prevention of group B streptococcus:

It is important to understand that, in spite of testing and treatment, some babies still develop GBS disease. Research is ongoing to develop vaccines to prevent GBS disease. In the future, women who are vaccinated against GBS may make antibodies that cross the placenta and protect the baby during birth and early infancy.

Fetal Circulation Diagram

2009-01-12T20:25:00.001-08:00

Growth Calculator

2009-01-12T20:21:00.000-08:00

Congenital Muscular Torticollis

2009-01-12T20:16:00.001-08:00

What is congenital muscular torticollis?

Congenital muscular torticollis (CMT) is a shortened neck muscle that causes the neck to twist. Congenital means that the condition is present at birth. Torticollis means "twisted neck." Most frequently CMT involves the muscle that goes diagonally across the neck from the collarbone (clavicle) to the head just behind the ear. This muscle is called the sternocleidomastoid muscle.

CMT causes the back of the head to turn toward and the face to turn away from the affected side. Your child will not want to move his or her face towards the affected side because of pain.

What is the cause?

The cause of torticollis is not completely understood. One theory is that there is stretching of the muscle during the delivery of the head. Severe stretching leads to bleeding into the muscle. If enough bleeding and swelling occurs, pressure builds up and damages the muscle. Then, scar tissue replaces some of the muscle. Other theories are that it is caused by crowding inside the uterus, a blood vessel problem, or a primary muscle problem. Whichever theory is correct, the baby keeps the neck turned in one direction.

How is it diagnosed?

CMT is diagnosed after an exam by your health care provider.

What is the treatment?

Many times children with CMT get better on their own. You can help your child improve by putting your child in situations where she has to stretch the affected muscle on her own. For example, lay the child in the crib so that if she wants to see what is going on in the room, she will have to turn her chin towards the shoulder of the affected side. This will stretch the affected muscle and help cure the torticollis naturally.

Treatment of CMT involves passive stretching exercises. This means that you help your baby move her neck. Exercises to improve CMT are described below. Do these exercises in sets of 10 repetitions, three times each day.

Side bend: Place one hand on the shoulder of the affected side of the neck (the side with the tight cord-like feeling in the muscle) and the other hand on the top of the child's head. Gently move the neck so that the child is looking straight ahead. With the child looking straight forward, gently bend the neck so the ear opposite the affected side goes toward or touches the shoulder on that side. Gentle downward pressure can also be applied to the shoulder on the affected side.
Head turn: With the hand on the shoulder of the affected side and the other hand on the top of the child's head, gently move the neck so that the child is looking straight ahead. Now gently turn the child's head so that the chin moves toward the shoulder of the injured side. (The opposite direction of the way the child prefers to turn the head.) Gentle downward pressure can also be applied to the shoulder on the affected side.

Your baby will probably be referred to a physical therapist to help determine how the treatment is going. If the condition is still present after the age of 1 year, your child may need surgery to restore movement of the muscle.