DukeHealth.org: Your Child's Health

What Is Neuroblastoma?

Fri, 27 Apr 2012 11:38:46 -0400

While uncommon, cancers do occur in children and with a diagnosis there comes, of course, many questions and concerns.

Michael Armstrong, MD, PhD, an expert in treatment of pediatric cancers, explains what neuroblastoma -- one type of pediatric cancer -- is and how it is treated.

-- Dennis Clements MD, PhD, MPH

Michael Armstrong, MD, PhDNeuroblastoma is the third most common cancer in childhood, behind leukemia (blood cell tumor) and brain tumors. It affects 650 to 750 children in the United States each year. Nearly all cases occur before the age of 10 with a peak incidence at age two. Additionally, neuroblastoma is the most common tumor in infancy.

Neuroblastoma is a tumor of the sympathetic nervous system, which is responsible for “fight or flight” responses. It is composed of very primitive cells called neuroblasts similar to those found in the developing nervous system of a baby in utero.

The most common location for neuroblastoma to occur is in the nerve tissue of the adrenal glands, small organs that sit on top of the kidneys.

Neuroblastoma tumors can also occur anywhere along either side of the spinal column in the neck, chest, or abdomen. Neuroblastoma commonly migrates to lymph nodes, bones, bone marrow, liver, and skin.

Symptoms of Neuroblastoma

The most common symptoms found in children with neuroblastoma tumors are caused by the tumor pressing against important structures or from its invasion of the bones or bone marrow. Possible symptoms include:

An enlarged or bulging abdomen
Lump in the neck or chest
Shortness of breath
Bone pain or limping
Weakness or paralysis (inability to move a body part)
Bulging eyes
Dark circles around the eyes (“Raccoon eyes”)
Painless, bluish lumps under the skin in infants
Jerky, involuntary muscle movements
Uncontrolled eye movements
Feeling tired
Easy bruising or bleeding

Diagnosis

If your pediatrician suspects a possible neuroblastoma tumor, the following tests may be performed under the direction of a pediatric oncologist:

Physical exam
Blood tests to assess bone marrow, liver, and kidney function
X-ray, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI) to look for possible tumor
MIBG scan -- MIBG is a compound that is taken up specifically by neuroblastoma cells and can be detected by a special scanner. Used to find presence of neuroblastoma tumors throughout the body
Bone scan -- a specialized test to detect presence of tumor in the bones
Bone marrow biopsy to assess for presence of neuroblastoma cells in the bone marrow
A urine test to detect presence of homovanillic acid (HVA) and vanilyl mandelic acid (VMA), two compounds commonly secreted by neuroblastoma tumors
Tumor biopsy. If a tumor is found on imaging, a pediatric surgeon will obtain a piece of the tumor. This is important to identify the specific type of tumor as well as determine important biologic features of the tumor which may direct therapy

Treatment of Neuroblastoma

Several treatments of neuroblastoma are available including chemotherapy, surgery, radiation therapy, and autologus bone marrow transplant (using one’s own bone marrow stem cells).

The treatment regimen is determined by the child’s “risk” group. Risk is determined by factors including the child’s age at diagnosis, how much the tumor has spread (stage), and certain characteristics of the tumor cells.

In infants, the tumor often does not require treatment and will regress on its own over time. Other children require much more therapy in order to achieve a remission.

A new innovation in neuroblastoma is directing the child’s own immune system to fight the neuroblastoma. A recent publication reveals that use of an anti-neuroblastoma antibody, called ch14.18, after autologus bone marrow transplant leads to a 20 percent increase in disease-free survival at two years after diagnosis – increasing from 46 percent to 66 percent.

Prognosis

Children with low- or intermediate-risk neuroblastoma have a great prognosis with 80 to 90 percent surviving five years after diagnosis. Children with high-risk neuroblastoma do not have as good an outlook, with close to two-thirds succumbing to their disease.

High-risk neuroblastoma is an area of intense research to improve outcomes for this group. The promising results of antibody therapy are encouraging and hopefully further strides will be made in the upcoming years with the development of new drugs and treatments.

If you are concerned that your child may have a tumor like neuroblastoma, please contact your child’s pediatrician for further evaluation.

-- Michael Armstrong, MD, PhD, specializes in treatment of neuroblastoma, pediatric solid tumors, and leukemia for Duke Children's.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

The Value of Saving Umbilical Cord Blood

Wed, 14 Mar 2012 10:54:49 -0400

Today, patients frequently ask me whether they should store blood from their baby for possible future use. Thirty years ago I was never asked this question.

Jessica M. Sun, MD, a pediatric blood and marrow transplant specialist at Duke Children's, explains why you might want to save your child's umbilical cord blood.

-- Dennis Clements MD, PhD, MPH

What is umbilical cord blood?

Umbilical cord blood is a baby’s blood left in the placenta after the baby is born and the umbilical cord is cut.

Historically, umbilical cord blood was discarded with the placenta as medical waste. Over the past few decades, cord blood has been shown to contain stem cells and early precursor cells that can be used for life-saving stem cell transplantation for children and adults in need of a stem cell transplant.

Cord blood is more tolerant of a new host and can be used without full matching, providing increased access to transplantation for patients who cannot find a matched donor.

How is umbilical cord blood used in medicine?

Hematopoietic stem cell transplantation can be an effective therapy for pediatric and adult patients with certain cancers, immune deficiencies, bone marrow failure syndromes, and some genetic diseases including inborn errors of metabolism and hemoglobinopathies.

Traditionally, stem cells used for transplantation were obtained from bone marrow or blood. More recently, cord blood has become an alternative source of stem cells for transplantation.

A major limitation to stem cell transplantation therapy is the ability to find a suitable donor. Only 20 to 25 percent of patients in need of a transplant have relative who is a “match” and can serve as their donor. Of those without a related donor, only 10 to 50 percent of patients (depending on their race and ethnicity) will find a matched unrelated bone marrow donor through the National Marrow Donor Program and other donor registries.

Cord blood transplantation does not require as strict matching as bone marrow, so many patients who cannot find a matched bone marrow donor can find a suitable cord blood donor. It is estimated that more than 4,000 cord blood transplants are being performed each year around the world.

Cord blood is also being studied as a source of stem cells for other purposes, including regenerative therapies for tissues damaged by injury or disease. However, these applications remain unproven and are currently the subject of ongoing research.

How is umbilical cord blood collected and stored?

Umbilical cord blood can be collected without risk to the mother or infant donor. Cord blood can be collected from the placenta, either during the third stage of labor or within 10 to 15 minutes after delivery of the placenta, by sterilely puncturing one of the umbilical veins with a needle and allowing the cord blood to drain into a sterile bag containing an anticoagulant to prevent clotting.

After collection from the placenta, some of the red blood cells are usually removed and the volume of the cord blood collection is reduced.

For long-term storage, cells undergo specialized freezing procedures and are stored in special freezers under liquid nitrogen. Maximal storage time, or expiration date, is unknown, but cells are likely to remain usable for decades. Cord blood units from public banks have been successfully transplanted after 18 years in storage.

What are the options for cord blood storage?

There are two main types of cord blood banks, public and private.

In general, public banks are nonprofit entities supported by federal or private funding. After the mother consents, public banks collect cord blood from healthy full-term pregnancies at no cost to the donor’s family.

In giving consent, the infant’s mother acknowledges that the donation is voluntary and gives up all rights to the cord blood for the public good. The mother also agrees to allow her medical records and the baby’s newborn records to be reviewed, gives a detailed family medical history, and allows a sample of her own blood to be taken for infectious disease testing.

Units passing screening tests designed to eliminate risks of transmitting genetic or infectious diseases are typed, placed in the search registry, and are available to any suitable patient in need of transplantation. Units that do not meet criteria for public banking may be discarded or used for research purposes.

Private cord blood banks are generally for-profit companies that store “directed donations” intended for future use by the child or a family member. Using a kit provided by the bank, the cord blood is collected by the physician, midwife, or nurse delivering the baby and shipped back to the company’s banking facility. The parents of the infant are charged an initial fee for collection and processing of the cord blood and then an annual fee for storage.

Varying degrees of testing is performed on the units, and minimal standards are used to determine whether a unit is eligible for processing and banking. The majority of private collections are undertaken as an investment in the unknown potential for cord blood to be used to treat serious illnesses in the future.

Most obstetricians and pediatricians feel that routine cord blood storage in healthy babies is unnecessary. In this regard, it is important to note that a child’s own cord blood would not be used for transplantation of a child with leukemia or other cancers, in part due to concern for contamination with cancerous cells, and it would not be used to treat a genetic condition because the cord blood would contain the same genetic problem.

Currently, directed donation of umbilical cord blood for another family member is recommended when a first-degree relative has a high risk pediatric cancer that can be treated with transplantation therapy, a hemoglobinopathy or other transfusion-dependent blood disorder, a congenital immune deficiency, or an inborn error of metabolism.

How can I donate my child’s umbilical cord blood?

It is always a good idea to discuss options for cord blood banking with your obstetric provider or pediatrician.

To privately store your baby’s cord blood for possible future use by the child or a family member, you may contact one of the many private cord blood banks to arrange collection, shipment, and payment. Additional information about cord blood banking, including a list of private banks, can be found through the Parent’s Guide to Cord Blood Foundation.

To donate your baby’s cord blood for public use, first check whether the hospital at which you plan to deliver works with a cord blood bank to collect cord blood for public donation.

In North Carolina, public collections are available at Duke, UNC, Durham Regional, Womack Army Medical Center, Women’s Hospital of Greensboro, and Rex Hospitals.

If your hospital does not participate in public cord blood banking, there are a few public cord blood banks, including the Carolinas Cord Blood Bank at Duke, that offer a free kit program so that public donations may be collected at other hospitals. Interested parents should contact the bank (919-668-2071) at least six weeks before the baby’s due date to learn more about the program.

Currently, public donations are limited to mothers who have a healthy pregnancy, are 18 years or older, and are pregnant with a single baby. More information about public cord blood donation is available through the National Marrow Donor Program.

-- Jessica M. Sun, MD, is a pediatric hematology / oncology specialist in the Duke Department of Pediatrics.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

Dangers in the Home

Thu, 23 Feb 2012 08:06:23 -0500

Although a lot of pediatrics is dedicated to caring for sick children, most children are healthy when they come in for a routine check-up. It is a great opportunity to explore ways to keep our children safe.

Sara Robert, MD, a pediatrician with Duke Children's Primary Care, points out some common dangers in the home and ways to keep children safe.

-- Dennis Clements MD, PhD, MPH

Sara Robert, MDInjuries are the leading cause of morbidity and mortality in children less than 19 years of age. According to the Centers for Disease Control (CDC), more than 12,000 children zero to 19 years of age die each year in the U.S. from unintentional injury.

Although the leading causes of injury death differ by age group, these preventive tips about how to keep our children healthy and safe in and about our home can be useful for everyone.

Anoxic/Threats to Breathing

Suffocation is the leading cause of injury death for children less than one year. In the 1990s, deaths related to sudden infant death syndrome (SIDS) decreased dramatically as parents were educated to place their sleeping infant only on their back, rather than on their side or stomach.

Choking items around the home can include food, toys, and household items. Items in reach of a child should be large enough that they cannot fit inside an empty roll of toilet paper. Additionally, old plastic shopping bags or balloons should not be in reach of children, as these can cause suffocation.

Safe eating practices include sitting at a table or in a highchair while eating, cutting food into small bites, and steaming to soften to the appropriate texture. Common choking culprits include grapes, hot dogs, popcorn, hard candies, and foods that clump (such as cheese or peanut butter).

If a child chokes, know these steps for clearing the obstruction:

Heimlich maneuver for children (health.harvard.edu)
Heimlich maneuver for infants (health.harvard.edu)

Although it sounds great to save money and borrow a crib or likewise use an old family heirloom, there are many specifications that you will need to check to make sure the crib is safe, including newer recommendations against drop-side cribs, as well as older recommendations that the distance between slats is no more than 2-3/8 of an inch apart.

Mini-blinds should not be in reach of children. Pay close attention to the drawstrings, which should be tied out of the reach of children.

Drowning is the leading cause of death from ages one to four years. Any water source, including toilets, baths, jacuzzis, and pools should be closely supervised while children are around.

Falls

Although most parents at some point turn to find their child standing precariously on a couch, or climbing on the table, there are many steps that we can take to avoid falls.

It can take months before some babies begin to roll from back to front. But it is always safest to directly supervise a baby placed on a diaper changing table, couch, or bed top, not even leaving them for a second to grab a spare bag of wipes. A safe alternative would be to change the child on the floor.

Stairs are also a notoriously dangerous place that seem to magically draw the toddler who is just learning to climb. Sturdy safety gates are recommended, to be installed securely sometime well before your infant becomes mobile, usually by eight or nine months as most children crawl before or around this time.

Walkers are no longer recommended due to concerns the mobile infant might fall down stairs. Instead we now often recommend stationary exersaucers as a safer alternative.

Always leave car seats, bumbo, and bouncy chairs on the floor rather than perched on higher surfaces.

Lastly, falls from windows can be very dangerous. Make sure windows are securely fastened, and never assume a screen is a barrier to a child’s fall.

Ingestions/Poisonings

Once children become mobile, they can (and love to) get into anything. How many times have you turned around to find an entire toilet paper roll unraveled on the bathroom floor? That is why we recommend spending some time looking around the house to assure ourselves all dangerous items are safely stored.

This includes cleaning products, paints, medications, or any other choking hazards. It’s a good idea to lock cabinet doors with childproof locks, keep medications in childproof containers, and store these kinds of products up high.

Burns/Fire

Even on a little baby we see burns, often from splattering of a cup of hot coffee or tea. We recommend that hot water heaters are set at less than 120 degrees, as the rate of burn is much slower below this temperature.

Never leave electric appliances anywhere a child can reach them, especially not near the bath where they could tumble into the water. Plug all electric outlets with store bought devices. Matches should be kept out of reach of children, and children should also be taught never to touch them.

When cooking on the stove top, make sure pan handles point toward the back of the stove and therefore cannot be grabbed by little hands. Likewise, make certain no toddling kids are around when you open the door to an oven, as it only takes a second to get burns all over an explorative hand.

Remember to check that your smoke alarms are working, changing batteries at the appropriate times. Carbon monoxide (CO) detectors are also recommended to avoid CO intoxication.

Avoid feeding children food or drinks that may be at scalding temperatures -- in particular, we recommend not heating contents of bottles in the microwave as they can unevenly distribute heat and even overheat the food. Always test the temperature of food before feeding your child.

Guns

It has been estimated that anywhere between 30 to 50 percent of homes in the U.S. contain a gun. Chances are great that your child will be visiting family or friends in a house where a gun is stored. Safe gun storage methods include storing the gun and ammunition separately and locked up, such as in a safe.

Educate your child about what a gun is and if they should see one: never to touch it --instead, leave the area immediately and go tell an adult.

This is particularly important as guns are often very visible in television, movies, and video games, often glamorizing them rather than making them appear dangerous. Talk to your child about this!

Lastly, according to the New England Journal of Medicine (Suicide in the Home in Relation to Gun Ownership, A Kellerman, N Engl J Med 1992; 327:467-472), readily available guns are associated with an increased risk of suicide in the home. If you know about a family member or friend who is feeling depressed, guns should absolutely be removed from the home.

-- Sara Robert, MD, is a pediatrician with Duke Children's Primary Care.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

Hepatitis in Children

Thu, 22 Dec 2011 13:20:37 -0500

Hepatitis is a general term that simply means inflammation of the liver. There are many different causes of hepatitis in children.

Megan Butler, MD, an expert in pediatric hepatology at Duke, explains what hepatitis is and how it is treated.

-- Dennis Clements MD, PhD, MPH

Megan Butler, MDHepatitis may be sudden onset (acute) or chronic (long standing). There is a wide range of clinical finding with hepatitis depending on the severity of the inflammation.

Hepatitis may be mild and self-limiting and resolve with no treatment, or it may become chronic and lead to liver failure requiring liver transplant.

If the cause of the hepatitis can be treated quickly, the liver is likely to recover fully.

Causes of Hepatitis

Hepatitis has numerous causes. The following is a short list of possible causes of hepatitis and some examples of each:

Infections: Viral, hepatitis A, hepatitis B, hepatitis C, fungal
Toxin/medication: Tylenol overdose, poisonous mushrooms, alcohol
Autoimmune: Immune cells of the body mistaking the liver cells as foreign and attacking the healthy liver
Genetic/metabolic: Cystic fibrosis, Wilson’s disease (copper buildup in liver), hemochromatosis, alpha-1-antitrypsin deficiency
NAFLD: Fatty liver usually associated with obesity and metabolic syndrome

Hepatitis Symptoms

The symptoms of hepatitis are similar regardless of the cause of inflammation.

Initially your child may have non-specific flu-like symptoms including fever, fatigue, muscle aches, vomiting, diarrhea and rash. Abdominal pain, yellowing of the skin and eyes (jaundice), and dark urine may occur. A doctor may notice enlargement of the liver on exam.

As hepatitis becomes chronic, the liver may actually become smaller as inflammation is replaced by scarring (fibrosis) of the liver. Extensive scarring of the liver can lead to cirrhosis.

The liver may be unable to produce the proteins needed for normal body functions. This can lead to swelling of the abdomen with fluid (ascites), fluid accumulation of the legs, enlargement of the spleen, or easy bleeding and bruising.

Varices may develop. These are enlarged veins in the esophagus, stomach, intestine, and other organs that may produce life-threatening bleeding. Severe hepatitis may lead to problems with other organ systems such as lung, kidney, and central nervous system as well.

Diagnosis of Hepatitis

It is important to seek medical attention if you notice yellowing of your child’s eyes or skin. Your doctor will likely obtain laboratory testing to identify the extent of liver involvement as well as measures of the function of the liver.

They will also send tests to determine the cause of the hepatitis. They may obtain radiology studies of the liver (ultrasound, MRI, etc.).

At times, a biopsy of the liver is most informative. This is a procedure where a piece of liver tissue is obtained to be evaluated under a microscope.

Hepatitis Treatment

Treatment for hepatitis involves largely supportive therapy. Medications may be used to correct any abnormalities associated with liver dysfunction. Your child may be admitted to the hospital for observation or treatment.

Severe cases of hepatitis can cause significant dysfunction and be life threatening. These patients are critically ill and require careful monitoring in the intensive care unit. They may be placed on a ventilator to support breathing. They may require dialysis to support kidney function.

Medications and other techniques may be needed to maintain neurologic status. Significant bleeding is a risk with severe hepatitis and may require a procedure or surgery to stabilize.

If the underlying cause of hepatitis can be found, therapy may be direct toward that as well. In severe cases, liver transplant may be an option.

Prognosis

Prognosis for hepatitis is extremely variable. Some cases are transient mild elevation of liver enzymes that resolve with no intervention. These patients will typically have no further episodes of hepatitis and no future liver problems.

Other cases of hepatitis can lead to chronic hepatitis. These patients need to be followed by a pediatric gastroenterologist or pediatric hepatologist (liver doctor) and their liver function followed closely. They may need medications to maintain liver function, but otherwise can be fairly healthy and active. They may be at risk for liver cancers later in life. Severe cases may lead to liver failure.

It is important to see your doctor immediately if you think your child has any symptoms of hepatitis. Severe cases of hepatitis need to be evaluated by a pediatric liver transplant center as soon as possible.

-- Megan W. Butler, MD, is an assistant professor in Duke Pediatrics' Division of Gastroentorology, Hepatology, and Nutrition.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

New Applications of Interventional Cardiology in Children

Tue, 29 Nov 2011 14:27:44 -0500

Not very long ago children with congenital heart disease had few treatment options. Thankfully, that is no longer the case.

Duke pediatric cardiologist Gregory A. Fleming, MD, MSCI, explains exciting research in interventional cardiology and how that translates to treatments for children.

-- Dennis Clements MD, PhD, MPH

Gregory A. Fleming, MD, MSCIA diagnostic cardiac catheterization is a procedure in which small, flexible catheters are inserted through veins or arteries into the heart to measure oxygen levels and pressures in chambers of the heart as well as inject a special dye into the heart to visualize the structures of the heart under x-ray (fluoroscopy).

The purpose of a diagnostic catheterization is to obtain information to help guide further treatment.

What Is Interventional Cardiology?

Interventional cardiology refers to interventions performed by a cardiologist during a cardiac catheterization procedure to fix or stabilize certain problems within the heart or within blood vessels of the heart.

Interventions are commonly performed on adults with blockage of the coronary arteries (the blood vessels that supply the heart muscle with oxygen) to prevent heart attacks. These interventions usually consist of dilating the coronary arteries with special balloon catheters and sometimes placing a metal stent into the narrowed portion of the coronary artery.

Interventions in children are usually different than those performed in adults. In children, many different types of interventions are performed to fix or stabilize malformations or birth defects of the heart (Congenital Heart Defects) that can occur during development of the heart.

Numerous devices have been specially engineered to close abnormal blood vessel connections and abnormal holes between chambers of the heart. These devices are released into the heart through the catheters that are inserted into the veins and arteries.

Some common interventions in children that have been performed for many years are:

Creation of a hole between the two upper chambers of the heart (balloon atrial septostomy) in some “blue babies” to allow them to survive until a corrective surgery can be performed
Closure (embolization) of a patent ductus arteriosus (PDA), an abnormal connection between the artery going to the body (aorta) and the artery going to the lungs (pulmonary artery), or other abnormal blood vessels with a small metal plug or a metal coil
Closure of an atrial septal defect (ASD), an abnormal opening between the two upper chambers of the heart, with a device inserted through a special catheter
Opening up narrowed or abnormal heart valves (valvuloplasty) with special balloon catheters in order to relieve obstruction
Opening up narrowed blood vessels (angioplasty) with special balloon catheters with or without metal stents

Newer Applications of Interventional Cardiology in Children

Exciting research is ongoing in the field of interventional pediatric cardiology. Some of this research has resulted more recently in the following:

New types of interventions that can be performed in the catheterization lab and therefore prevent the need for open heart surgery
Hybrid procedures: Interventions that can be performed by interventional cardiologists working with pediatric heart surgeons to make the procedure better and safer
Fetal interventions: Interventions performed before delivery of fetuses that are diagnosed during pregnancy with life threatening heart disease. These procedures can help the heart develop with an improved chance of survival after delivery
Perfecting the types of devices currently being used to close holes and abnormal vessels

Some examples of newer applications of interventional cardiology in children are:

Replacing heart valves (pulmonary valve replacement) using valves that are sewn into metal stents and inserted in the heart using special balloon catheters
Using devices to close holes between the lower pumping chambers of the heart (ventricular septal defects) that are delivered using special catheters
Hybrid procedures:

Hybrid Norwood procedure: newer procedure used for babies born with underdevelopment of the left side of the heart (hypoplastic left heart syndrome)
Closing ventricular septal defects (VSD) with a device inserted by a cardiologist through a small puncture of the heart made by a cardiac surgeon without the need for a heart lung bypass machine
Fetal interventions:

Opening up narrowed valves (valvuloplasty) during pregnancy in order to allow improved development and function of the other chambers of the heart before delivery
Creating holes in the heart (septostomy) to allow relief of elevated pressures in the heart that would cause significant problems after delivery

Those are a few of the many new opportunities that exist for children with heart disease. It is an exciting time for research in this area, and we expect that more advances will come to fruition in the coming years.

-- Gregory A Fleming, MD, MSCI, is a pediatric cardiologist in the Duke Department of Pediatrics.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

Stabilizing the Critically Ill Child

Wed, 05 Oct 2011 09:43:48 -0400

No one willingly wants to be admitted to the hospital -- and certainly not in an emergency. Unfortunately, sometimes some of my young patients do need to be admitted, and it is comforting for both the children and parents to know what to will happen once they are there.

Kyle Rehder, MD, a pediatric intensivist, explains what you can expect if your child is admitted to the hospital with a critical illness.

-- Dennis Clements MD, PhD, MPH

Kyle Rehder, MDIt is usually clear what a pediatric subspecialist does: a cardiologist takes care of children’s hearts; a neurologist takes care of the brain.

But what about a pediatric intensivist? In short, a pediatric intensivist, or critical care physician, specializes in the stabilization and care of extremely ill children.

Early Interventions

When a critically ill child presents for medical attention, time is truly of the essence.

Whether the child is sick from injury, infection, cancer, heart disease, surgery, or some other crisis, lost minutes can mean the difference between life and death.

Rapid interventions are often required to ensure the child gets blood flow and oxygen to vital organs and injured tissues. Attention must then be paid to reversing the underlying disease process that led to critical illness.

Rigorous care and monitoring continues after the initial stabilization, until the child is stable enough to be moved out of the intensive care unit (ICU).

Advanced Technologies

Children with critical illness often require various types of support for their failing organs. Fortunately, pediatric intensivists also have sophisticated tools at their disposal, including different types of mechanical ventilators, medicines to keep the heart pumping and maintain blood pressure, and in extreme cases, heart-lung bypass machines.

When one system fails, state-of-the-art technology may often be used to support the patient during their recovery.

Coordination of Care

Teamwork is essential for the critically ill child. The combined efforts of physicians, nurses, respiratory therapists, and pharmacists are required to act quickly to stabilize children during this precarious time.

Each team member has a specific role to fill, and all ICU providers receive training in structured communication and teamwork to ensure seamless cooperation, particularly in emergencies.

Thoughtful coordination of care with other specialists is also often necessary for treatment of children in the pediatric intensive care units.

Patient safety is a constant concern and priority in the intensive care unit. Not only are critically ill children more susceptible to injury and infection due to their fragile state, but the number and complexity of medical interventions required for children in the intensive care unit also places them at increased risk.

Multidisciplinary teams within the ICUs tirelessly work to minimize these risks whenever possible.

Family-Centered Approach to Care

Despite the need for immediate interventions and the sometimes hectic nature of the ICU, family-centered care is an important priority. There will rarely be a more stressful time in a parent’s life than during the critical illness of one of their children.

It is important for the medical team to partner with families in determining care plans. Families are included in daily rounds and encouraged to be present at the bedside. A Family Advisory Committee, containing parents of former ICU parents, also helps guide unit policies to foster family-centered care.

ICU care extends beyond the walls of the unit, as the critical care team responds to pediatric medical emergencies throughout the inpatient hospital and outpatient clinics, through the Code Blue and Rapid Response Teams.

Children are also commonly admitted directly from other hospitals and emergency rooms, providing support by phone to help stabilize children and transport them safely to the ICU. When you need help, the ICU team is always just a call away.

It is always rewarding to see children recover from life-threatening illness. Children tend to get critically ill much faster than adults, but have amazing resilience and ability to mend. With early intervention and the proper support, even the sickest of children can make it home to lead full lives.

-- Kyle Rehder, MD, is a pediatric intensivist with Duke Department of Pediatric's Division of Critical Care Medicine.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

Bronchiolitis in Children

Thu, 11 Aug 2011 13:16:09 -0400

Whenever winter looms on the calendar, many parents begin to worry that if their infants get colds, they will have trouble breathing. How can we tell when a common cold has progressed to something that needs more attention?

Kathleen Bartlett, MD, a pediatric hospitalist at Duke, explains what bronchiolitis is and when to be concerned enough to see a doctor.

-- Dennis Clements MD, PhD, MPH

What is bronchiolitis?

Kathleen W. Bartlett, MDBronchiolitis is an infection of the small airways in the lungs called “bronchioles.” The bronchioles are tiny tubes that carry air in and out of the lungs. In bronchiolitis these tubes becomes swollen, irritated, and blocked with mucous, making it difficult for the air to move smoothly in and out of the lungs.

Bronchiolitis occurs in children under two years of age. Young children have smaller airways than older children and adults, making them more susceptible to the breathing difficulties that occur with this infection.

Bronchiolitis is most commonly caused by respiratory syncytial virus (RSV), but can also be caused by a host of other common respiratory viruses. Because these viruses tend to circulate in the winter months, bronchiolitis typically occurs from November through March in North America. There is no evidence of a bacterial cause for bronchiolitis.

Bronchiolitis can be diagnosed on the basis of symptoms and physical exam findings, although nasal secretions may be tested for some of the specific viral causes. Blood tests and chest x-rays typically are not helpful in diagnosing bronchiolitis.

What are the symptoms of bronchiolitis?

Bronchiolitis usually starts out with common cold symptoms including:

Low-grade fevers
Congested or runny nose
Mild cough
Decreased appetite

After a few days symptoms may progress to include:

Faster breathing
Wheezing
Worsening cough including coughing spells
Decreased feeding

Usually children start to get better four or five days into the illness, but the cough can last up to two-to-three weeks.

When should children with bronchiolitis see a doctor?

Most children with bronchiolitis do not need medical attention, but certain children are at risk for a more complicated course. These include children with a history of prematurity or heart disease, children who are less than 6 months old, children who attend daycare or have older siblings, and children who are exposed to cigarette smoke.

A child with any of the following symptoms should see a doctor:

Rapid breathing
Grunting with each breath
Flaring of the nostrils with each breath
Using the muscles between the ribs or at the base of the neck to breathe
Decreased wet diapers or poor feeding

Rarely children may have pauses in their breathing or turn blue with bronchiolitis. If this happens, you should call 911 immediately.

How is bronchiolitis treated?

Because bronchiolitis is caused by viruses, there are no medications that can cure the infection. Antibiotics and steroids are not helpful in most cases of bronchiolitis.

The limited treatment available is aimed at supporting the child through the illness and relieving symptoms. It is reasonable to try an inhaled asthma medication called albuterol to see if it can relax the airways and make breathing easier. For most children with bronchiolitis, albuterol does not help.

Frequent suctioning of the nose with saline drops and a bulb syringe is also recommended. Since infants prefer to breathe through their noses, this simple treatment may make a child more comfortable and improve feeding tremendously.

Children with bronchiolitis are at risk for dehydration, so parents should encourage fluid intake even if the appetite is decreased.

Although most children with bronchiolitis are managed at home, approximately 2 percent of young children in the U.S. are hospitalized with bronchiolitis each year. Reasons for admission usually include one or more of the following:

Low oxygen saturation (amount of oxygen measured in the blood stream)
Difficulty breathing
Poor feeding causing dehydration
Pauses in the breathing

Even in the hospital, care is aimed at supporting the child as her own immune system fights off the viral infection. Typically children in the hospital receive oxygen until their saturations are back to normal, intravenous fluids until their feeding improves, and monitoring until their breathing improves. Suctioning is also a mainstay of hospital care.

In addition, hospital physicians may try nebulized hypertonic saline, a concentrated salt water solution that is given as an inhaled treatment to attempt to break up secretions in the lungs. None of these treatments cure the infection; they simply support the child through the illness. Hospitalization usually lasts one-to-three days, but in some severe cases may last for weeks.

Can bronchiolitis be prevented?

Twenty percent of infants in the U.S. are diagnosed with bronchiolitis, and many more likely have a milder, unrecognized version of the disease. However, the following may reduce a child’s chances of getting bronchiolitis:

Good hand washing
Avoiding exposure to other sick children or adults
Avoiding cigarette smoke exposure
Breastfeeding

Select children who are at very high risk of severe bronchiolitis may be given monthly injections of a medication called palivizumab (Synagis). This injection is a temporary antibody against RSV. It does not provide lasting immunity the way vaccines do. Because palivizumab is expensive and only partially effective in preventing bronchiolitis, it is reserved for premature infants and those with congenital heart disease.

-- Kathleen W. Bartlett, MD, is a pediatric hospitalist at Duke.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

What to Expect When a Child Is Admitted to the Hospital

Thu, 28 Jul 2011 09:58:07 -0400

Perhaps nothing in life is as stressful as having a child who is sick or injured. Every year, there are approximately 14,500 visits to the pediatric emergency department (ED) at Duke, and of these, over 1,700 require admission or overnight observation.

Donald T. Ellis II, MD, of Duke Pediatrics' Division of Hospital and Emergency Medicine, wrote this article in the hope that, by removing a portion of the unknown component, the hospitalization will be more comfortable for both you and your child.

-- Dennis Clements MD, PhD, MPH

Donald T. Ellis II, MDWhen a child is admitted to the hospital through the emergency department, it is quite common for a parent or other caregiver to feel overwhelmed. They have most likely told the story or “history” to the triage nurses, the ED nurses, the resident physician (and sometimes a student as well), the attending, or supervising, doctor, and occasionally one or more consulting physicians.

If needed, the child may have had blood drawn, an intravenous line (IV) placed, x-rays or other imaging studies performed, and medications given.

Often, these events require several hours for completion. When it seems as if there is nothing more to do than be transported upstairs, there is actually a great deal to accomplish to ensure a smooth transition.

Starting the Process

After the physicians determine that a child needs to be hospitalized, a request will be submitted to bed control, the hospital personnel overseeing inpatient beds. This assures that there is not only a bed space, but also available staffing to care for the patient upstairs.

Concurrently, the admitting team is notified, and although the majority of pediatric patients are admitted to general pediatrics, there are hundreds of patients every year who are admitted to a surgical or medical specialty service (for example, neurosurgery, critical care, or cardiology).

For patients under the care of the general pediatric team, the ED physicians will contact the inpatient physicians to discuss the case. By having a direct conversation, they can communicate not only the history, physical exam findings, and the results of any diagnostic tests, but they can also ensure that the inpatient team has a good understanding of what the other diagnostic possibilities are at that time.

Occasionally, the admitting team asks for additional testing before the patient is transported, and the ED staff tries to accommodate these requests as much as possible.

In the Hospital

Once a bed space is assigned for the child, the ED nurse will call “report” to the nursing staff on the pediatric ward. This allows the entire health care team the opportunity to become familiar with your child’s case.

Taking report, however, does not take the place of hearing the information firsthand. Therefore, staff will likely want the opportunity to review the history with you after your child’s arrival on the inpatient ward. By this series of checks and double-checks, the entire health care team can familiarize itself with the details that make your child’s condition unique.

When all of the preparations are complete, a staff member will escort you and your child from the emergency department. At times, it is necessary to be accompanied by several personnel and monitoring equipment. Usually, you will use a special elevator reserved for patients and staff to either the fifth or seventh floor of the hospital.

The staff will go with you to your assigned room. Although all pediatric rooms with the exceptions of the intensive care and step-down units are designed as single-patient quarters, in most cases, you are welcome to stay in the pediatric ward with your child as long as he or she is hospitalized. In fact, we would love it if you would! Parents and guardians can provide significant comfort to children, regardless of age.

While nothing will ever negate all of the emotional and physical stress of having a child in the hospital, our goal is for every child to receive world-class medical care. Hopefully, this description of the process of being admitted from the emergency department to the pediatric ward decreases the potential anxiety of the unknown.

Clay Bordley, MD, MPH, and Kelly Anderson, RN, assisted with this article.

-- Donald T. Ellis II, MD, is a hospital and emergency medicine specialist with the Duke Department of Pediatrics.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

Wheezing Infants

Thu, 30 Jun 2011 12:44:40 -0400

Almost 50 percent of infants will have an episode of wheezing in the first year of life. A wheezing baby can lead to many questions and concerns for parents and for pediatricians.

Katharine Kevill, MD, who specializes in pediatric pulmonology, explains the causes, symptoms, and risk factors for infant wheezing and educates parents about when to take their wheezing child to the pediatrician.

-- Dennis Clements MD, PhD, MPH

What is a wheeze?

Katharine Kevill, MDThe term wheezing may mean different things to different people.

The Merriam-Webster Dictionary defines the verb wheeze as "to breathe with difficulty usually with a whistling sound." Physicians often have a more specific definition such as "a musical and continuous sound that originates from oscillations in narrowed airways."

Air moving through a narrowed airway can make a whistling sound, in the same way that the wind whistles as it moves through a tunnel.

Sometimes, wheezes are only heard with the stethoscope, but other times they are heard with the naked ear.

The wheezing sound can originate at any points from the upper to the lower airways. The upper airways start at the pharynx and extend down to the level of the larynx (sometimes called the voicebox).

The lower airways start with the largest airway, the trachea. The airways become smaller as they branch out into bronchi and then bronchioles. Finally, air reaches the alveoli, or air sacs.

The tone of the wheeze depends upon where the airway is narrowed. Babies with a lot of upper airway congestion may have coarse, noisy breathing.

Wheezes that come from multiple places in the lower airway may have a musical tone, with several different pitches (polyphonic).

Usually wheezing occurs when the baby exhales, but it can also occur when he or she inhales (inspiration). The noise made due to airway obstruction during inspiration is called stridor.

Why are infants more susceptible to wheezing than older children?

Obstruction to airflow depends upon the width of the airway. Babies have very narrow airways, so even a small decrease in the width can cause a large obstruction to airflow.

Furthermore, babies have very compliant chest walls relative to older children. When babies breathe out forcefully, this can cause the chest wall to move inward and place pressure on the airways, which can cause the airways to narrow.

What are the causes for wheezing in infants?

Many wheezing episodes in babies are caused by bronchiolitis, or inflammation of the small airways, called bronchioles. Usually, bronchiolitis is caused by viral respiratory infections.

Other problems may cause babies to have noisy breathing, even when they don’t have a cold. Gastroesophageal reflux (food going from the stomach back up the esophagus) or swallowing problems can cause babies to have a lot of nasal congestion and noisy breathing.

Babies may also be born with variations in the structure (or anatomy) of their respiratory tract that can cause wheezing. One common anatomic variation is tracheomalacia, where the cartilage that supports the trachea is not yet firm enough. In this case, the trachea narrows as the baby breathes out, causing an airflow obstruction.

Accidental aspiration of an object into the airway can also lead to airway obstruction and wheezing. Other possible causes for wheezing in infancy include diseases such as cystic fibrosis.

If a baby wheezes, will he or she develop asthma?

Although almost 50 percent of infants have an episode of wheezing in the first year of life, most of them do not go on to develop asthma.

For children who have had at least three episodes of wheezing within 12 months, some risk factors have been associated with an increased likelihood of developing asthma. Three major risk factors for developing asthma include:

A parent with asthma
Eczema in the child
Allergy in the child to any inhaled allergen (such as dust, mold, etc.)

When should a parent take a wheezing baby to the pediatrician?

Certainly any concerns that parents have about their infant’s breathing should be discussed with the pediatrician.

If the infant is having a lot of trouble breathing, then it may be necessary to bring the baby to the pediatrician or the emergency room right away.

Signs of respiratory distress or trouble breathing may include:

Severe coughing
Blue color of the face or lips
Crying but not making much noise
Flaring out at the nostrils
Sucking in at the base or the top or the ribs
Breathing really fast

In most cases, infants who wheeze do very well. Usually infants with bronchiolitis stop wheezing after the viral infection runs its course. Other problems such as reflux or swallowing problems typically improve with age.

Most infants with wheezing are treated by their general pediatricians and require few, if any, studies. In some cases, pediatricians may refer wheezing infants to a physician that specializes in respiratory issues such as a pediatric pulmonologist or an otolaryngologist for further evaluation.

-- Katharine Kevill, MD, is a pediatric pulmonologist with Duke Department of Pediatric's Division of Pulmonary and Sleep Medicine.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

Iron Deficiency Anemia in Children

Wed, 01 Jun 2011 10:05:53 -0400

Iron-deficiency anemia is a preventable illness that affects many infants and toddlers in the United States.

Jennifer Rothman, MD, pediatric hematologist and associate director of the Duke Pediatric Sickle Cell Clinic, explains the importance of screening for iron deficiency anemia, symptoms of the disease, and ways to prevent this illness in children.

--Dennis Clements, MD, PhD

What is iron deficiency?

Jennifer Rothman, MD Iron deficiency is the most common nutritional deficiency in the world. The World Health Organization (WHO) estimates that nearly one third of the world’s population may have low red blood cell numbers due to insufficient iron.

In the United States, iron deficiency remains common with 9 percent of toddlers between the ages of 12-36 months having inadequate iron stored in their bodies.

What is iron deficiency anemia?

Anemia is when a person has a significantly lower-than-average red blood cell number or a decrease in the protein called hemoglobin (oxygen carrier) for his or her age and gender.

In toddlers, that is defined by the WHO as lower than 11 gm/dL for both boys and girls. In the U.S., 5 percent of toddlers between the ages of 12-36 months have anemia, and iron deficiency is the cause of anemia in 40 percent of those children.

Why do we need iron?

Iron is important for many processes in our body including carrying oxygen to all of our organs. Most of the iron in our body is found in hemoglobin, a protein in our red blood cells. Iron is also stored in our liver, bone marrow, and spleen in a storage form called ferritin.

Iron is necessary to make red blood cells, as well as for normal growth and development. Iron deficiency in infancy may be associated with developmental delays or behavioral problems.

What causes iron deficiency?

A developing fetus gets all of its iron from its mother during pregnancy. Most of the iron stores are given to the fetus in the third trimester.

A full-term infant born from a healthy mother will have enough iron to support growth and development until four to six months of age. After that, iron needs to be absorbed through the diet in the form of breast milk, iron-fortified formula, solid foods, or vitamin supplements.

Iron deficiency is most often caused by not enough iron absorbed through the diet. Sometimes people lose a lot of iron from the body in the form of blood loss. A good example of that is very heavy menstrual bleeding or bloody diarrhea from inflammatory bowel disease.

What are the symptoms of iron deficiency?

Symptoms of iron deficiency include:

Pale skin, lips, or hands
Fatigue or sleepiness
Not wanting to eat
Fast heart rate
Pica -- eating non food items like ice, paper, dirt, or couch cushions

Who is at risk for iron deficiency?

Infants, toddlers, teenagers, pregnant women, and, primarily, menstruating females are at risk for iron deficiency.

Special risk factors include:

Preterm infants born before the third trimester (before 37 weeks gestation)
Infants born to mothers with diabetes or severe anemia
Vegan or vegetarians without a source of iron-rich foods
Exclusive breast feeding beyond four to six months (not receiving iron-fortified solid foods in addition to breast milk)
- At four to six months, an infant has outgrown his or her stored iron, and, while breast milk does have iron, it is not enough to keep up with an infant’s rapid growth.
Early introduction of cow’s milk (before 12 months) or excessive cow’s milk intake (more than 24 ounces per day)
- Cow’s milk is low in iron and can actually prevent iron from being absorbed from the diet. In addition, some children develop small amounts of bleeding from their intestines when they have too much cow’s milk.
Continued use of bottle feeding after 12 months of age
Blood loss through heavy menses or bloody diarrhea
Children with special health care needs

How do you screen for iron deficiency anemia?

Due to how common anemia is in childhood and the potential impact of anemia on growth and development, the American Academy of Pediatrics recommends screening for iron deficiency anemia at 12 months of age by checking a hemoglobin level as well as getting a good history for iron deficiency risk factors.

If the hemoglobin is low, then it is helpful to get a full blood count to look at the size and shape of the blood cells. Iron deficiency anemia is associated with small red blood cells, or cells that have a low MCV.

Another helpful test includes a serum ferritin which measures the amount of iron stored in the body. The serum ferritin may be falsely high if your child has recently been sick.

Screening for iron deficiency is recommended at any age for a child who has symptoms or significant risk factors.

How do you treat and monitor iron deficiency anemia?

If anemia is identified in a child with risk factors for iron deficiency, then it is reasonable to start iron replacement without sending a serum ferritin.

Iron replacement consists of an iron vitamin, either liquid or pill, at a dose of 6 mg/kg/day of elemental iron. Taking the iron supplement with a vitamin C-fortified liquid, such as orange juice, will help the iron be better absorbed. Milk intake should be limited and an iron-rich diet should be encouraged.

If iron deficiency is the cause of the anemia, then an increase in the hemoglobin by 1 gm/dL after four to six weeks is expected. Iron supplementation should be continued for at least six weeks after normalization of the hemoglobin in order to refill the child’s iron stores.

A repeat blood count should be performed three to six months after the iron supplement is stopped to make sure the child is maintaining his or her iron stores. If there is not an improvement in hemoglobin while taking iron supplementation, then further investigation is recommended.

Sometimes the lack of improvement is because the iron is not being taken as prescribed or the child’s diet has not changed. Other times, the cause of the anemia may not be iron deficiency.

How do you prevent iron deficiency?

Preterm infants who did not receive many red blood cell transfusions during the newborn period, should receive an iron-containing preterm infant formula or breast milk with an iron-containing vitamin.

Full-term infants can get iron from iron-fortified formula or breast milk. Breast-fed infants should start a vitamin with iron (1mg/kg/day) at four months of age until iron-containing solid foods, like iron-fortified rice cereal, are introduced.

Infants should be weaned to a cup around 12 months and should not start cow’s milk until older than 12 months. Cow’s milk should be limited to no more than 24 ounces per day (eight to 12 ounces will be sufficient and is much less likely to cause anemia).

Toddler diets should include good sources of iron included red meat, beans, green vegetables, and iron-fortified cereals. Parents raising their children as vegetarians should take special care to identify iron-rich foods.

-- Jennifer A. Rothman, MD, is the associate director of the Duke Pediatric Sickle Cell Clinic.

-- Dennis Clements, MD, PhD, is the chief of primary care pediatrics at Duke Children's Hospital.

When Is Puberty Too Early?

Wed, 13 Apr 2011 10:54:20 -0400

In the past 10 years, parents increasingly ask me whether their child’s maturing to puberty is occurring at too early an age. Often, girls in fourth grade seem to be maturing, and their parents are not ready for it.

Deanna Adkins, MD, a pediatric endocrinologist, discusses the normal progression of puberty and explains warning signs that indicate puberty is happening too early.

-- Dennis Clements MD, PhD, MPH

Deanna Adkins, MDAs any parent who has been to his child’s classroom or sporting activity knows, kids come in all sizes.

These size differences get pronounced when puberty arrives in some of the children and not others.

One of the more noticeable things that occur is that girls shoot up past the boys around 11 or 12 years of age. Then the boys catch up and pass them by around 14 or so.

This is the usual pattern, but many children follow their own pattern that can be very different from this.

Early and late bloomers are considered different from the normal pattern of puberty, but these patterns occur frequently in adolescents and should be considered a variant of normal. Both tend to run in the family with both boys and girls going into puberty a little later or earlier than their peers.

When should a parent worry?

What Is Early or Precocious Puberty?

As previously mentioned, the first signs of puberty are expected at very different times in boys and girls. There has been much study and discussion in the medical community about the early onset of puberty in and when it should be evaluated.

The focus has traditionally been on girls, but recently there has been some evidence and discussion surrounding this in boys as well.

Early Puberty in Girls

For girls, puberty is generally considered to be too early if it begins at age seven or eight. African-American and Hispanic girls tend to start puberty slightly earlier than Caucasian girls.

The average age of pubertal onset in girls is 10-and-a-half years old, but it ranges from seven to 13 years old. The average age of menarche is 12-and-a-half to 13 years of age. The whole process of puberty should take three to four years.

Rapidly progressing puberty -- start to finish in less than two years -- can be a concern as well because it can be due to an endocrine disorder.

The first sign of puberty in girls is most often breast development. Other signs include:

Body odor
Pubic hair
Acne
Growth spurt
Menses (rarely)

Early Puberty in Boys

For boys, puberty is generally considered too early before the age of nine years. In boys, onset of puberty is from nine to 14 years, but on average starts at 11-and-a-half to 12 years old.

The whole process of puberty should take three to four years. Rapidly progressing puberty can also be a concern in males.

The first sign of puberty in boys is usually testicular growth. Other signs include:

Body odor
Pubic hair
Penile growth
Acne
Axillary hair
Facial hair
Growth spurt
Deepening of the voice

If Your Child Shows Signs of Early Puberty

If any of these signs occur in girls before age seven or eight or in boys before age nine, an evaluation should be considered.

Often, the first step is an x-ray of the hand and wrist called a bone age test to see if these hormones have affected the growth and bone maturity of the child already. Further evaluation can include blood tests, ultrasound, or MRI.

Causes of Early Puberty

In almost 90 percent of the cases of early puberty in girls, there is no known cause. In the other 10 percent, possible causes could be an abnormal brain structure or tumor, ovarian cysts (including McCune Albright syndrome), deficient thyroid function (hypothyroidism), head trauma, radiation, adrenal hyperplasia or tumor, or exposure to environmental hormones or hormone-like chemicals.

In boys, the onset of early puberty is more likely to be caused by an underlying disease.

Diseases that cause early puberty include structural abnormalities of the brain; radiation; tumors of the brain, testis, liver, and adrenal gland; inherited disorders such as adrenal hyperplasia or testotoxicosis; exposure to hormones or hormone-like compounds in the environment, or hypothyroidism.

Possible other causes of early puberty that are currently being studied include environmental chemicals and obesity.

Risk Factors for Developing Early Puberty

Risk factors often linked to early puberty in children include:

Being a female
Obesity
African-Americans and Hispanics
Exposure to hormones from medications or environment
Head injury including radiation and surgery
Other medical conditions such as congenital adrenal hyperplasia, McCune Albright syndrome, neurofibromatosis, and hypothyroidism

Treatments for Early Puberty

The first step is to treat the cause of early puberty. This may include surgery to remove the tumor or medications to replace the thyroid.

To stop or slow down the puberty, medication is often used to regulate testosterone or estrogen production; these medications are known as LHRH analogs or antagonists. They are available in several forms that include a monthly injection, quarterly injection, subcutaneous implant, and, rarely, a nasal spray.

For certain forms of early puberty, other medications are used. These block estrogen production from male hormones (androgens) or block the estrogen receptor itself.

One of the complications of early puberty is early closing of the growth plates that leads to short stature. If there is evidence that this is occurring, growth hormone can be added to the treatments above to optimize final adult height.

In addition to the physical changes in early puberty, there are also psychological concerns that may need to be addressed.

This may require a referral to a counselor as puberty can be a difficult transition at the normal age of onset and can be very difficult in a young child who may have more difficulty understanding the changes going on in their bodies. These children are at risk for low self-esteem, depression, and substance abuse.

Preventing Early Puberty

While genetic factors play a role in the early onset of puberty, parents can help delay the environmental causes of early puberty. Preventive measures include:

Encourage your child to maintain a healthy weight.
Avoid exposure to exogenous hormones like estrogen, testosterone, DHEA, androstenedione that may be found in creams/gels, hair treatments, medications, and nutritional supplements.

-- Deanna Adkins, MD, is a pediatric endocrinologist with Duke Department of Pediatric's Division of Endocrinology.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

The Genetics of Autism: What We Know That You Should Know

Fri, 25 Feb 2011 16:01:56 -0500

Parents frequently ask me about what causes autism. I explain that little is known about the causes, but geneticists are beginning to understand some of the reasons kids develop autism.

Dr. Yong-hiu Jiang, who specializes in pediatric medical genetics, answers frequently asked questions parents often have about the link between genetics and autism.

-- Dennis Clements MD, PhD, MPH

What is autism?

Yong-hui JiangAutism is the name given to a group of developmental brain disorders that are characterized by poor social interaction and communication and by restricted or repetitive behavior. These signs appear before a child is three years old.

What causes autism?

For many children with autism, we do not know the cause. However, for some children, autism is caused by a change in their genetic information (DNA).

Genetic changes causing autism can occur in a single gene or can involve missing or extra pieces of chromosomes that include many genes (microdeletions and microduplications).

Some people with autism have other symptoms, such as specific physical differences or learning problems, which are part of a genetic syndrome.

Examples of genetic syndromes that include autism as a symptom include:

Fragile X syndrome
Rett syndrome
Angelman syndrome
Tuberous sclerosis

In rare cases, autism can also be part of clinical presentation of a metabolic disorder (a genetic condition in which the body cannot break down or make a specific substance).

Why do scientists believe that genetics is an important cause of autism?

Findings from twin and family studies led scientists to believe that genetic differences are an important cause of autism. However, non-genetic factors may also have a role.

Unless a known genetic change has been found in a child, doctors cannot be sure exactly how autism is inherited in the family. It may be caused by inheriting a combination of multiple genes from both parents or by a new genetic change that is only present in the child’s DNA.

Why has my pediatrician referred my child with autism to the genetics clinic?

Although we still have a lot more to learn, scientists have already learned a great deal by studying the genetics of autism over the last decade. This knowledge is helping doctors to find the cause of autism in some children.

A diagnosis of a change in a specific gene or a microdeletion or duplication can help to tailor medical, developmental, or school interventions and to answer the questions many parents have about the chances that other children in the family could also have autism.

Therefore, pediatricians now routinely recommend a clinical genetics evaluation after a child is diagnosed with autism.

What can I expect when I visit the autism genetics clinic?

During the clinic visit, a clinical genetics doctor or genetic counselor will ask questions about your child's medical and developmental history and collect a detailed family history.

If your child has had previous medical evaluations or blood tests, it is helpful to bring those records with you. The doctor will also perform a detailed physical examination.

At the end the visit, the doctor will discuss whether your child may have a known genetic syndrome and whether any genetic tests might be helpful in finding the cause of your child's condition. If you have any questions about the evaluation, testing, or your child’s health, you can ask the doctor or genetic counselor during the visit.

What types of genetic tests are requested for children with autism?

Clinical genetics doctors usually decide which types of genetic tests to order after evaluating your child.

The samples needed are usually blood and urine. Other more specific tests may also be ordered at the end of visit.

The following blood tests are often requested:

Chromosome microarray
Fragile X syndrome DNA test
Rett syndrome MeCP2 mutation test
Plasma amino acid analysis
Creatine/guanidinoacetate determination
Acylcarnitine profile

The following urine tests are often requested:

Urine organic acid analysis
Creatine/guanidinoacetate determination
Purine profile

What is a chromosome microarray?

Chromosome microarray (CMA) is a new method of analyzing chromosomes for a large number of genetic disorders. With a single test, CMA analyzes all chromosomes simultaneously for extra or missing pieces of genetic material.

CMA has much higher sensitivity for finding these types of chromosome differences than the older chromosome test called a karyotype. However, it is not able to find all genetic changes in a person's DNA.

Why is the CMA test used to look for genetic causes of autism?

Since CMA was developed, many large research studies have shown that it finds chromosome differences thought to cause autism in about 5 to 10 percent of children with the condition.

CMA also can detect many other common genetic disorders that cause intellectual disabilities.

What are the benefits of knowing the genetic defect in my child with autism?

In most cases, knowing the genetic cause for autism in a child will not lead to a cure. However, knowing the genetic change may help you better understand your child's condition and know what to expect in the future.

Your child's physician may be able to recommend specific preventive care measures and guide routine health monitoring for your child.

What's new in autism genetic research at Duke and nationwide?

Research on autism genetics is moving at a fast pace. Due to the development of cutting edge techniques, human genetics researchers are now able to analyze the entire genetic blueprint (genome) of a person.

This technology can be used to find out more about genetic causes of autism.

Neuroscientists are looking for differences in how the brain develops and works in people with autism. Such research studies are ongoing at Duke and other research institutes nationwide. If you are interested in participating in these studies, please contact Dr. Yong-hui Jiang, MD, PhD.

-- Yong-hui Jiang, MD, PhD, is a pediatric medical geneticist at Duke Children's Hospital.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

Cavities in Children

Tue, 01 Feb 2011 00:00:00 -0500

Frequently when I see a two-year-old for a well child visit, I exclaim, “Oh my –- your child has cavities between all his baby molars!” The parents wonder, “How can that be?”

If your child eats a lot of sticky foods, she may be at risk for cavities between the teeth. Dr. Martha Ann Keels, a pediatric dentist at Duke Children’s, explains why these foods often cause cavities in children and how to prevent them.

-- Dennis Clements MD, PhD, MPH

Have you ever used a toothpick or your fingernail to remove food from between your teeth? Many children do not develop the sensation of something being caught between their teeth until they are grooming to go out on a date. That can lead to problems with cavities between teeth, especially if the child frequently eats sticky foods.

Unfortunately, many parents start their children’s day with a gooey breakfast bar and gummy vitamins. Both items are very cariogenic (cavity causing) and sticky. These foods collect on top of and between the molars (the teeth in the back of the mouth that they chew with).

The Beginning of Tooth Decay

Figure one (courtesy of Dr. Ted Croll) shows the molars of a young child with dried fruit snacks caught between the teeth and on the top surface of the molars. If the dried fruit snacks are left in contact with the enamel, the process of tooth decay begins.

Figure 1

The sticky food serves as a carbohydrate source for cariogenic bacteria, which in turn process the sugars into lactic acid via glycolysis. It is the lactic acid that breaks down the enamel of the teeth, resulting in cavities.

It can be a very upsetting dental visit when parents of a four-year old hears from the dentist that their child has multiple cavities between the primary (baby) molars.

Around age four, the back primary molars drift in closer proximity and touch each other therefore requiring the dentist to take bitewing radiographs to visualize between these teeth.

Prior to age four, the molars should have space between them allowing the dentist to easily visualize all sides of the teeth. Humans do the majority of their chewing with their molars. If a child is chewing more sticky sugary foods or candies, the risk of developing cavities between the molars increases.

In figure 2, you can see a left bitewing radiograph of a four year old demonstrating four cavities (dark radiolucent areas) in between her four primary (molars). The right side of her mouth had the same disease process. She had eight cavities in total between all of her baby molars. The white areas on the top of her molars indicate she has had previous fillings in the grooves on the chewing surfaces of her teeth.

Figure 2

Many people do not realize the risk of having foods caught between their teeth, which can result in several interproximal (in between the teeth) cavities. (Cavities require local or general anesthesia and fillings to repair, which will prevent a future significant infection in the jaw).

Dentists cannot detect this type of tooth decay without radiographs. Tooth brushing alone cannot prevent these types of cavities.

Avoiding Cavities

To remove food particles from between teeth, one must floss. American Academy of Pediatric Dentists (AAPD) recommends that parents should start flossing their child’s teeth when any two teeth are touching and continue to do so until approximately age eight when the child has sufficient dexterity to assume the responsibility of self-flossing.

In providing healthy lifestyle suggestions for parents, it is helpful to use the AAP prevention message of “ 5-2-1-0” -- eating five fresh fruits and vegetables everyday, two hours or less of screen time, one hour or more of physical activity, and zero sugared drinks.

However, a health care provider must be diligent in asking if the caregiver is feeding “fresh” fruit, as many parents are substituting processed fruit, such as dried fruit snacks, fruit roll-ups, and fruit-by-the-yard for fresh fruit as the fruit choice for their child. These types of non-fresh fruit snacks create a high caries (the process that causes cavities) risk.

A better choice for a snack is fresh fruit (sliced apples or banana) or vegetables (celery sticks, carrots). For the occasional sweet treat, one that melts, such as a scoop of ice cream, a Hershey Kiss, or M&Ms is a better choice as this type of treat does not adhere to the enamel. If it melts readily on your hand, it will easily melt off of the teeth.

Pediatricians and their staffs can help encourage healthy eating habits and good oral hygiene rituals including flossing between any two teeth that touch. They can also encourage avoiding sticky snacks and candies and limiting the frequency of even the “better” sugary treats. “All things in moderation” applies to treats as well.

-- Martha Ann Keels, DDS, PhD, is a pediatric dentist with Duke Department of Pediatrics's Division of Pediatric Dentistry.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

Diarrhea in Children

Mon, 22 Nov 2010 16:06:46 -0500

When should parents worry about their child's frequent stools? When parents ask me about this, I always respond by asking the parents what their stool patterns are like -- since not everyone has the same idea about what is normal.

Once convinced it is an unusual pattern, we discuss what causes frequent stools and when to worry.

Dr. Leon Reinstein of Duke Children's helps us to understand what we should consider when it comes to diarrhea in children.

-- Dennis Clements MD, PhD, MPH

Leon J. Reinstein, MD

What is diarrhea?

Diarrhea is an increase in the number of stools (bowel movements) per day and an increase in the looseness of stools. Mild diarrhea may vary from one to three stools a day; severe diarrhea can consist of up to 20 stools a day.

In general, diarrhea is a common problem that may last only a few days and disappear on it’s own. Usually related to a viral or bacterial gastroenteritis, an infection causing diarrhea, abdominal cramping, vomiting and fever.

Diarrhea may be:

Acute (lasting less than two weeks): usually related to bacterial or viral infections, it may also be a reaction to medications such as antibiotics
Chronic (lasting longer than two weeks): related to conditions such as an irritable bowel syndrome, parasites or an inflammatory bowel disease such as Crohn’s disease, ulcerative colitis or Celiac disease

How did my child get diarrhea?

By ingesting contaminated food or water
By touching the stool of an infected person
When touching an object contaminated with the stool of an infected person, and then ingesting the germs

What are the symptoms of diarrhea?

Your child may experience one or many of the symptoms associated with diarrhea:

Cramping
Abdominal pain
Bloating/distended abdomen
Nausea
Urgent need to used the restroom Fever
Bloody stools

These symptoms may be a sign of other conditions. Always consult your child's physician.

When do I worry about diarrhea?

You should always call your child’s physician if your child is less than six months of age or has other symptoms such as:

Abdominal pain
Blood in the stool
Frequent vomiting
Unable to eat or drink
High fever
Diarrhea longer than two weeks
Any signs of dehydration such as:

o Dry mouth

o Weight loss

o Extreme thirst

o Frequent and persistent diarrhea

o Urinates less frequently

o Sunken eyes or depressed “soft spot”

o No tears when crying

o Difficulty staying awake

What are treatments for diarrhea?

Treatment will be determined by your child’s physician. Most causes of diarrhea will not need treatment. It will depend on the age of your child and the cause of diarrhea. Treatment involves replacing lost fluids and is geared to prevent complication of dehydration.

Administration of anti- diarrhea medication is strongly discouraged. Antibiotics are used only in specific infections.

It is important to maintain oral hydration and a regular diet as much as possible. Avoid sodas or sports drinks. Give Pedialyte or Gatorade as alternatives.

Infant and children at times may require an admission to the hospital if unable to maintain adequate oral intake.

How do I protect my family?

Diarrhea is easily spread to others in the family. Take the following precautions:

Everyone in the family should wash their hands with soap and water after using the bathroom, handling diapers, or preparing foods
Change diapers away from where food is being prepared or eaten
Keep dirty diapers in a covered container away from children or pets
Clean the bathroom thoroughly every day
If your child is in day care, inquire about their policy on caring for sick children

Always remember to wash your hands.

-- Leon J. Reinstein, MD, is a pediatric gastroenterologist with Duke Children's.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.

Cardiac Catheterization for Children

Fri, 29 Oct 2010 08:42:30 -0400

Occasionally children are born with complex birth defects of the heart or may aquire heart/lung disease later in life. When this is the case, a cardiac catheterization may be required to diagnose or fix the problem.

Dr. Kevin Hill, a pediatric cardiologist with Duke Children's, tells us more about this procedure and what to expect.

-- Dennis Clements MD, PhD, MPH

What Is a Cardiac Catheterization?

Kevin D. Hill, MD, MSA cardiac catheterization is a procedure where a thin, flexible tube called a catheter is inserted into a vein or artery and guided into the heart. The catheter can then be used to:

Obtain blood samples and measure oxygen levels in the chambers of the heart and in the lungs
Obtain pressure measurements from the heart chambers and in the lungs
Inject a special dye known as contrast that can then be followed with a video x-ray (fluoroscopy) as it is pumped through the heart and lungs

Thus a catheterization provides valuable information about the structure and function of the heart, lungs, and associated blood vessels.

Cardiac catheterizations are often performed in adults, most commonly to evaluate for blockage of the coronary arteries -- the vessels that supply blood to the heart muscle. While children occasionally require evaluation of their coronary arteries, most pediatric catheterizations are performed to evaluate and treat birth defects of the heart.

Catheterization equipment is specifically designed to accommodate the smaller size of children’s hearts and blood vessels.

Before the development of echocardiography (ultrasound of the heart), catheterization was the primary tool used for the diagnosis of heart defects in children.

While most heart lesions can now be diagnosed with an echocardiogram, catheterization is still sometimes needed to diagnose very complex heart defects. Catheterization is also important as part of the pre-operative assessment of many heart defects.

More recently catheterization has evolved to include a variety of interventional procedures including:

Closure of holes in the heart including atrial septal defects and ventricular septal defects
Creation of holes to improve oxygen saturations and sometimes to relieve elevated pressures (septostomy)
Dilation of narrowed blood vessels (angioplasty)
Opening of narrowed or occluded valves (valvuloplasty)
Placement of stents to open up narrowed vessels
Occlusion of unnecessary and detrimental blood vessels (embolization)
Obtain small specimens of heart tissue for microscopic analysis (biopsy)

Recent technology has even allowed for the replacement of heart valves in the catheterization laboratory without any surgery.

How Is a Catheterization Performed?

Typically a catheterization is performed using the femoral artery and vein. These are vessels located in the leg that can be accessed from the groin region.

Other vessels that are sometimes used include the internal jugular vein located in the neck, the subclavian artery and vein located along the shoulder, and occasionally the hepatic (liver) vessels that can be accessed from the right side of the abdomen just below the ribs.

Almost all catheterizations in children are performed with sedation. Sometimes general anesthesia is used and the infant, child or adolescent is so sedated that a breathing machine (ventilator) is required to help breath during the procedure.

Sometimes less sedation is required and the child is asleep but still capable of breathing without the support of a ventilator.

Once the infant, child, or adolescent is asleep, a needle is used to access the blood vessels and a thin tube called a sheath is inserted into the vessel.

The sheath allows catheters to be passed into the vessel but prevents bleeding. Once the sheath is in place, the catheter can be navigated up into the heart and lungs.

This portion of the procedure is not painful. Catheterization does not require any surgical incision.

How Long Does It Take to Recover from a Cardiac Catheterization?

At the end of the procedure the catheters and sheaths will be removed from the body.

A nurse or physician will hold pressure over the catheterization site for about 10 minutes. This allows a clot to form and prevents any bleeding. After the clot is formed, a special dressing will be applied to the site. The dressing is designed to keep some pressure over the puncture site to prevent any further bleeding.

After four to six hours, the dressing will be removed and a smaller bandage will be left in place. Most children have recovered from the procedure within several hours. Sometimes children can be discharged home on the day of the procedure and sometimes an overnight hospital stay is required.

Occasionally, for more complicated catheterization procedures, a longer hospital stay is needed.

The catheterization site is usually fully healed within a few days. To be safe, we recommend avoiding bathing or soaking in water for three days and avoid heavy lifting (greater than 10 pounds) for 10 days.

There may be additional precautions depending on the complexity of the procedure and interventions performed.

-- Kevin D. Hill, MD, MS, is a pediatric cardiologist based at Duke University Medical Center. He also sees patients at Duke Children's Cardiology of Fayetteville.

-- Dennis Clements, MD, PhD, MPH, is the chief of primary care pediatrics at Duke Children's Hospital.