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Short Bowel Syndrome

Posted by on Monday, 4 October, 2010

What is Short Bowel Syndrome?

Short bowel syndrome (SBS) occurs after congenital (before birth) or postnatal loss of at least half of the small intestine, with or without loss of a large part of the intense carmine. The loss of the bowel may be due to disease or surgical removal. Short Bowel Syndrome (SBS) produces malabsorption, a condition where the body can not break and / or absorb sugars, proteins or fats.

short bowel syndrome

The extent of the problems associated with SBS depends mostly articles from the small intestine are affected. The small intestine normal height ranges between 10 and 28 meters and is divided into three sections.

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS).

Short bowel syndrome is the result of the alteration of intestinal digestion and absorption that occurs following extensive bowel resection. It represents a complex disorder that affects normal intestinal physiology with nutritional, metabolic, and infectious consequences.

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS).
The small intestine of the newborn is about 250 cm in length. In adulthood, small intestine, grows to about 750 cm. Consequently, infants and toddlers have a favorable long-term prognosis compared with an adult in terms of growth potential of the intestine after intestinal resection. Intestinal adaptation can take weeks or months to achieve, in the meantime, children who underwent intestinal resection require nutritional support through a variety of therapies, including parenteral nutrition. The duodenum and jejunum are responsible for the absorption of most dietary components with the exception of vitamin B-12 and bile acids.

Jejunum is characterized by long and wild large absorbent surface. Tight junctions are relatively large, so that the epithelium of larger molecules to pass more, and the free circulation and quick water and electrolytes. Comparison of the ileum is less wild, and the absorption capacity of surfaces unless the jejunum. In addition, tight connections are more stringent, reducing the flow of water and electrolytes in the vascular space into the intestinal lumen and, consequently, the ileum is a more efficient absorption into the water. Although nutrients are not as well absorbed in the small intestine, which has specific receptors on the site, the absorption of bile acid and vitamin B12. In addition, many hormones that affect the gastrointestinal tract, intestine, including enteroglucagon and peptide YY, produced in the ileum. 

Sites of nutrient absorption:

  • Duodenum – Iron
  • Jejunum – Carbohydrates, proteins, fat, vitamins
  • Ileum – Bile acids, vitamin B-12

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of short bowel syndrome. Similarly, studies in animals have shown that intestinal transit time is more likely to improve (ie, increase) in patients with proximal small-bowel resection as opposed to patients with distal small-bowel resection.

Since the jejunum can not develop site-specific carriers for the transport of vitamin B12 and bile salts, and therefore poorly absorbs permanently in patients after ileal resection. In addition, the loss of enteroglucagon and peptide YY can not be stressed in the regulation of bowel motility.

The leading causes of death among infants with short bowel syndrome who are treated with parenteral nutrition include central line sepsis and liver failure with prolonged use of parenteral nutrition.

The history of patients with short bowel syndrome (SBS) is usually a child born with a congenital anomaly, such as omphalocele, gastroschisis, intestinal atresia or, requiring small bowel resection. In addition, premature infants with necrotizing enterocolitis and require extensive bowel resection, with or without loss of the ileocecal junction also contribute to this patient population.

Other patients have a history of malrotation and volvulus, ischemic bowel, which had to undergo a bowel resection. Congenital short bowel syndrome is a rare cause of short bowel syndrome.

Children with short bowel syndrome may have various medical problems, according to the degree of intestinal resection and the level of medical complexity. The history should consider all potential ramifications of clinical case management of short bowel syndrome, including:

Parenteral nutrition

  • The degree of home nutrition support necessary in the management of a child on total parenteral nutrition (TPN) is noteworthy.
  • Guidelines for the safe use of parenteral nutrition have been established by the American Society for Parenteral and Enteral Nutrition.
  • Patients may present with issues entirely separate from the medical problems related to short bowel syndrome, including problems associated with intravenous access, infection, and signs and clinical symptoms associated with TPN-related liver disease.

Enteral Feeding

  • Quickly initiate enteral feeding for all children with short bowel syndrome.
  • Once again, patients can present a full history of medical problems associated with short bowel syndrome, including questions gastrostomy tube or nasogastric tube. For example, gastrostomy tubes may fall accidentally. In these patients, the immediate replacement of these tubes is important to maintain patency of the tube entry site.
  • Although complications are rare, be aware of the potential of gastric ulcer migration, and tube gastrostomy, intestinal obstruction, which may be associated with bilious vomiting and the risk of pancreatitis.

Nutrition

  • Supervise all children in enteral and parenteral long-term specific nutritional problems.
  • Patients may present with various symptoms related to specific nutritional deficiencies, including vitamins specific (or mineral) deficiencies and associated signs, symptoms and electrolyte disturbances and possible complications.

Medical and surgical histories: get a detailed account of past medical and surgical patient.

  • Pathology that leads to surgical resection
  • The extent and location of intestinal resection, the presence or absence of ileocecal valve.
  • Medical complications
  • RPT dependence
  • Enteral nutrition
  • enteral access
  • Type nutritional formula used
  • Dietary Supplements
  • Drugs
  • Allergies

History of complications associated with short bowel syndrome

  • Malabsorptive diarrhea
  • Dehydration
  • Vomiting
  • Bloating
  • Gastroesophageal reflux
  • Failure to thrive
  • Drug toxicities

Persistent Pulmonary Hypertension

Posted by on Wednesday, 28 April, 2010

Persistent pulmonary hypertension (PPHN) is the presence of blood flow through the lungs and heart seen in fetuses. While growing inside the womb, the mother did the breathing for him/her. Oxygenated blood went from the mother’s lungs, through her body , across the placenta, and into the baby. Therefore, the baby did not need to use it’s lungs. However, after the bay is born it cries, and takes breaths, the pressure in the pulmonary (lung) vessels should decrease; therefore blood can travel to the lungs to get oxygen. when a baby has PPHN, the pressure in the pulmonary vessels does not decrease causing blood to shunt the wrong way (away from the lungs), as the baby was still inside the womb. Therefore, the baby receives unoxygenated blood to their body.

persistent pulmonary hypertension

This condition is usually seen in near term, term, or post term infants. Symptoms are usually seen with in the first twelve hours of life. Several factors that cause PPHN, are stressful birthing process, swallowing the meconuim (the first few stools which are thick, sticky and pasty) by gasping for a breath in the birthing canal. The meconium can get into the baby’s lungs and cause the baby to have difficult breathing after birth. Other causes of PPHN may be cold stress, low blood sugar, low calcium, thickening of the blood, or infection.

Symptoms that are first noticed will be that the baby may begin to breathe faster, known as (tachypnea) and the baby’s chest may suck in (retractions) as if their having a difficult time breathing. The baby will probably need to be placed on a breathing machine(ventilator). A nurse will need to give some medication(sedation) to help the baby rest. You may see a bluish discoloration of the skin (cyanosis) because of decreased oxygen, resulting in the baby’s blood pressure being low. The main goal is to get oxygenated blood to the rest of the baby’s body. This is done by using a ventilator and medications. The baby may also need to be on a special medication to increase their blood pressure (vasopressor). The baby will have several lines and intravenous access to measure and monitor their condition. PPHN is a very Serious Condition. Some babies show long-term developmental problems, therefore, the baby’s development should be closely evaluated.


Respiratory Syncytial Virus

Posted by on Tuesday, 20 April, 2010

RSV stands for Respiratory Syncytial Virus, the most frequent cause of serious respiratory tract infections in infants and children under 4 years of age. This is such a common virus that RSV has infected virtually all children by the age of three. In most young children, it results in a mild respiratory infection that is not distinguishable from a common cold. RSV occurs throughout the year and is most prevalent in winter months.

respiratory-syncytial-virus

RSV causes nasal stuffiness and discharge, cough and sometimes ear infections. It is usually self-limiting and does not require hospitalization or specific treatment, even in the majority of those who also have lower respiratory tract involvement. These children may have a low-grade fever for several days, respiratory symptoms that may last for 1 to 2 weeks, and a cough that sometimes persists beyond 2 weeks.

Sometimes an infant or a young child who is experience his or her first RSV infection may develop a severe infection in the lower respiratory tract that is best managed in the hospital. Approximately 90,000 children are hospitalized with these infections each year. Most commonly, the ones requiring hospitalization are newborns and infants and those with complicating or underlying conditions, such congenital heart, lung disease or prematurity.

A child who develops signs of more stressful breathing, deeper and more frequent coughing, and who generally acts sicker by appearing tired, less playful, and less interested in food may have developed a more serious RSV infection an need to be hospitalized. In the great majority of cases RSV infection is self-limiting and requires no specific therapy. For the more serious cases, that are hospitalized, the doctor may order an antiviral treatment that is administered in a mist form This will depend on the severity of the illness, any associated diseases or conditions, and several other factors.

Children and adults of all ages can become infected. The infection in older children and may be very mild, usually causing cold- like symptoms. A person becomes infected by coming in close contact with another infected person or by the secretions from an infected person. An infant usually acquires the infection from close contact with an older family member who may have only a mild, cold-like symptoms. As noted earlier, RSV occurs throughout the year, but because it occurs in a wide-scale, sudden outbreaks, and is so prevalent in the winter months, it is not feasible or advisable to attempt to prevent the normal child’s exposure to RSV infection. When a family member is infected, extra precautions may be taken by washing of hands often, and preventing spreading of infectious secretions on tissues and other objects.

Although a child can get a second RSV infection, it’s very unlikely that the symptoms will be much milder than the first time. Most children recover completely and will handle their next respiratory infection with no more than the average child. A few children, however, appear to be more susceptible to subsequent respiratory problems. Susceptibility may relate, however, to some other underlying medical condition or allergy found in medically fragile children.


Failure to Thrive

Posted by on Saturday, 17 April, 2010

In the first few years of life is a time when most children begin to gain weight and grow much more rapidly than later on in childhood. Sometimes, however, children and infants don’t meet expected standards of growth. Although most of these children follow growth patterns that vary from the norm standards, others are considered to have “failure to thrive.” This is a general diagnosis, with many possible causes. Common to all cases however, is the failure to gain sufficient weight as expected, which is often accompanied by poor height growth. Diagnosing and treating a child who fails to thrive focuses on identifying any underlying problem. From there, doctors and the family work together to get the child back into a healthy growth pattern.

neonate

Although it’s been recognized for more than a century, failure to thrive lacks a precise definition, in part because it describes a condition rather than a specific disease. Children who fail to thrive don’t receive or are unable to take in, retain, or utilize the calories needed to gain weight and grow as expected.

Most diagnoses of failure to thrive are made in infants and toddlers in the first few years of life, a crucial period for physical and mental development. After birth, a child’s brain grows as much in the first year as it will grow during the rest of the child’s life. poor nutrition during this period can have permanent effects on a child’s mental development. Typically the average term baby will double his or her birth weight by 4-months old and triples it at the 1-year mark, children with failure to thrive often don’t meet those milestones. Sometimes, a child who starts out “plump” and who shows signs of growing well can begin to fall off in weight plan. After a while, linear (height) growth may slow as well.

If the conditions progresses, the undernourished child may:

  • Become disinterested in his or her surroundings
  • Tend to avoid eye contact
  • Become irritable
  • Don’t reach developmental milestones such as sitting up, crawling, walking and talking at the usual age.

Failure to thrive can result from a wide variety of underlying causes, to find out more about failure to thrive, I recommend purchasing my training manual, where I have a in depth chapter on the causes and treatment for this medical condition, along with many other interesting topics regarding medically fragile children.


Shaken Baby Syndrome

Posted by on Thursday, 15 April, 2010

Shaken baby syndrome: Is an extreme form of child abuse caused by the violent shaken of an infant or child.

Shaken baby syndrome can occur in as little as five seconds of shaking. Injuries usually occur in children younger than 2 years of age but have be seen in children up to the age of 5. When a child is shaken their brains can bounce back and forth inside their skulls causing bruising to the brain, also known as a cerebral contusion, this causes swelling, pressure and bleeding inside the brain. If the larger blood vessels on the outer region of the brain tear, this can cause additional swelling, bleeding and pressure sometimes causing permanent brain damage and even death. Other areas of the body can also be affected such as the neck, eyes, and spine.

In some cases, it’s an angry parent or caregiver that shakes the baby to punish or quiet them. Such shaking usually takes place when the baby is crying inconsolably and the frustrated caregiver finally loses control. Research has shown that a crying baby is the number one trigger leading caregivers to shaking an infant. Statistics show an estimated at 1,200 to 1,400 children annually are injured or killed by shaking in the U.S. This is one of the most unrealized forms of child abuse, as many times the caregiver didn’t intend on harming the baby. It is estimated that over 300 babies a year will die from being shaken in this country. This means that roughly 20% of the victims die and the other remaining 80% suffer permanent disability.

Injury to the brain is most likely to happen when a baby is shaken and then the baby’s head hits something even hitting a soft object, such as a pillow or mattress is enough to injure newborns and small infants. Newborn infant brains are much softer and their neck muscles and ligaments are weak. Since their heads are large and heavy in proportion to their bodies. The result of being shaken is a type of whiplash, similarly occurring in some auto accidents. It is important to note that shaken baby syndrome doesn’t occur from playful swinging or gentle bouncing. It rarely occurs from accidents such as small falls, those injuries often cause smaller head injuries.

Signs & Symptoms that you should look for if you suspect Shaken baby Syndrome
  • Decreased alertness
  • Seizures (convulsions)
  • Vomiting
  • Extreme irritability
  • Sleepiness, lethargy
  • Loss of vision
  • Loss of consciousness
  • Pale or blueish skin tone
  • Even lack of appetite

In some cases their may not be any visible signs of trauma such as bleeding, bruising or swelling and may difficult to diagnose in a routine office visit. where broken ribs or bone fractures are easily spotted on x-rays.

If your child suffers a head injury and begins vomiting first turn the child’s head to one side to prevent aspiration of the vomit and if you suspect any spinal injury carefully roll the child’s entire body over on it’s side so they don’t aspirate and seek medical attention as soon as possible.

Do not ignore the signs if you suspect child abuse in your home or in the home of someone you know, report it to the local police or county social services

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What Defines a child that is Medically Fragile?

Posted by on Tuesday, 13 April, 2010

“Medically Fragile or Special Health Care Needs Dependent”

To be defined as a special needs child, children must have special health care needs meaning the child must have a the need for specialized in-home health care based upon one or more of the following criteria:

  • Enteral Feeding Tube (nutrition fed through a tube placed in the nose, the stomach, or the small intestine.)
  • (TPN Dendent) Total Parenteral Feeding (supplies all the nutritional needs of the body by bypassing the digestive system, dripping nutrient solution directly into a vein.)
  • Cardiorespiratory Monitoring
  • Intravenous Therapy
  • Ventilator dependent
  • Oxygen Support
  • Urinary Catherization
  • Renal Dialysis
  • Ministrations imposed by tracheotomy, colostomy, Ileostomey, or other medical, surgical procedures.

Also, any condition that can rapidly deteriorate resulting in permanent injury or death. These children are commonly referred to as “medically fragile.” Typically, medically fragile children are infants under 3 years of age who are prone to hospitalization.

The following are a few examples of conditions that often, but certainly not always, qualify a child as medically fragile.

  • Prenatal exposure to drugs and/or alcohol
  • Congenital or hereditary defects such as spina bifida, sickle cell, anemia, cystic fibrosis, netherton syndrome, heart , lung defects.
  • Burns
  • Cerebral Palsy
  • Muscular Dystrophy
  • Epilepsy
  • BPD – Bronchopulmonary dysplasia
  • Failure to thrive
  • HIV positive and symptomatic
  • Permaturity

With the on going advances in medicine, the quality of life and longevity has vastly improved for medically fragile children. Many medically fragile children are able to be cared for in “specialized licensed foster care homes” with mandated individualized care plans outlining the child’s medical care needs as opposed to having to stay in hospital ward. This home setting has proven to to facilitate improvements in the child’s condition and well being.


Hip Dysplasia

Posted by on Sunday, 11 April, 2010

Developmental dysplasia of the hip (DDH) is a deformity of the hip that can occur before, during, or weeks after birth.
At periodic checkups, a doctor will examine the baby’s hips to rule out DDH, which can cause hip dislocation and/or an abnormal walk. It’s important to recognize DDH early, so a child can receive timely treatment and avoid orthopedic problems later in life.

What Is DDH?

The hip is a ball-and-socket joint. In a normal-functioning hip, the rounded top of the thighbone, or femoral head, rests comfortably in the acetabulum (the cup-like hipbone socket). In mild cases of DDH, the femoral head moves back and forth within the socket, causing a child to have an unstable hip. In more serious cases, the head becomes dislocated, moving completely out of the socket, but sometimes can be put back in with pressure. In the most severe cases, the femoral head may not even reach the socket where it should be held in place. Hip dislocations are relatively uncommon, affecting just 1 in 1,000 live births. However, some degree of instability of the hip is seen in as many as 1 in 3 newborns. Girls are more likely to develop dislocations of the hip.

What Causes It?

The causes of DDH aren’t completely understood, but experts think that many factors are involved. The cramping of the fetus inside the uterus which is more likely to happen in first pregnancies when the uterus is tight, or in pregnancies where there is a decrease of amniotic fluid (liquid in the womb) can increase the likelihood of DDH. Other factors include abnormal positioning of the fetus inside the womb, such as being in the breech position (buttocks face the birth canal), especially when the knees extend out with the feet near the head (called “frank breech”). Having other conditions develop as a result of positioning, like metatarsus adductus (an inward curving of the foot), increases the odds of a child developing DDH. DDH also may be caused by the infant’s response to the mother’s hormones that relax the ligaments for labor and delivery, causing the baby’s hip to soften and stretch during labor. In 20% of cases, family history is a factor, and if it is, future children should be checked by ultrasound at 6 weeks of age.

Signs and Symptoms

DDH usually affects only one side of the body, most often the right side, and pain is rare.
Infants often don’t show signs that they have DDH, and there may be no signs at all. Still, doctors look for these indicators:

  • At birth, an inability to move the thigh outward at the hip as far as possible
  • An audible “click” during routine post-natal checkups
  • Different leg lengths
  • Asymmetry in the fat folds of the thigh around the groin or buttocks
  • After 3 months, asymmetry in the motion of the hip and obvious shortening of the affected leg
  • In older kids, an exaggeration in the spinal curvature that may develop to compensate for the abnormally developed hip
  • Limping in older children

Diagnosis

A doctor can determine whether a hip is dislocated or likely to become dislocated by gently pushing and pulling on the child’s thighbones, and determining whether they are loose in their sockets. In one commonly used diagnostic test, a child lies on a flat surface and his or her thighs are spread out in order to gauge the hips’ range of motion. A second test brings the knees together and attempts to push the femoral head rearward, out of the socket. It is during these tests that the doctor will hear a “click,” which may indicate a dislocation. These maneuvers are done at routine checkups until the baby is walking normally. Sometimes a doctor will recommend that a child undergo an X-ray or ultrasound to get a better view of a dislocated hip. Ultrasounds are recommended for babies under 4 months of age, while X-rays are performed on older kids. Prior to 4 months of age, hip tissue has not yet hardened (or ossified) from flexible cartilage to bone, and therefore does not show up on X-ray images that capture only bony anatomy and not cartilage.

Treatment

Treatment for DDH depends on the age of the child and the severity of the condition. Mild cases may correct themselves in the first few weeks of life. If an unstable hip is seen in newborns, the hip may be held in position with a Pavlik harness. This device keeps the femoral head in its socket by holding the knee up toward the child’s head. A shoulder harness attaches to foot stirrups to keep the leg elevated. The goal is to tighten the ligaments in the area and stimulate normal forming of the hip socket. Treatment with the Pavlik harness lasts about 6 to 12 weeks, and continues until the hip is stable and ultrasound exams are normal. After a child reaches 6 months of age the Pavlik harness will be ineffective. Older kids will need to undergo one of the following treatments:

  • Closed reduction. The bone is manually put back into place after the child is put under anesthesia. This treatment is usually preferred in children younger than 18 months.
  • Open reduction. The hip is realigned and the thighbone is placed back into the hip socket through surgery. During the procedure, tight muscles and tissues surrounding the hip joint are loosened and then later tightened up once the hip is back in place. This is the procedure of choice for kids older than 18 months.

After reaching age 2 or 3, a child might need surgery on the pelvis to deepen the hip socket (if it’s too shallow) or to shorten the thighbone or realign it. Following surgery, the child is put in a hip spica (body cast). About 1 in 20 babies with DDH needs more than the Pavlik harness to correct the condition.

DDH can’t be prevented, but if it’s recognized early and treated appropriately, most children will develop normally and have no related problems. DDH does not cause pain initially, but if left untreated it can result in significant impairment of function. Kids with untreated DDH will have legs of uneven lengths in adulthood, which can lead to a limp or waddling gait, back and hip pain, and overall decreased agility.