Gastrointestinal Alterations

1
Gastrointestinal Alterations

• NUR 264
• Pediatrics
• Angela J. Jackson, RN, MSN

2
Developmental Differences

• GI system of the newborn very ineffective because
  of immaturity
• Sucking and swallowing automatic reflexes until
  nerves and muscles develop by 6 weeks of age
• Newborn stomach capacity 10 -20ml, expanding to
  200ml by one month of age and to adult capacity
  of 2000-3000ml by late adolescence
• Peristalsis is greater in the infant, emptying
  time of the stomach 2-3hrs in the newborn
  increases to 3-6hrs by one to two months of age

3
Developmental Differences

• Infants metabolic rate is faster than an adults.
  Infants require 100calories per kg, adults
  require 30-40 calories per kg
• Regurgitation common in infants (relaxed cardiac
  sphincter tone)
• Length of small intestine is proportionally
  greater in an infant, six times the body length
• Infants secretes more fluids and electrolytes
  into the intestine than the adult
• Large intestines of the infant are
  proportionately shorter than an adults,
  resulting in less epithelial lining available for
  absorption of water

4
Developmental Differences

• Liver function is immature at birth. Toxic
  substances are inefficiently detoxified and
  medications are inefficiently processed.
• Infants are deficient in several digestive
  enzymes, including amylase (carbohydrate
  digestion), lipase (fat absorption), trypsin
  (protein digestion)
• Infants intestines are more permeable to
  proteins, allowing passage of protein into the
  bloodstream, increasing susceptibility to food
  protein allergens

5
Cleft Lip and Cleft Palate
6
Cleft Lip and Cleft Palate

• Most common craniofacial malformation
• Cleft lip Simple notch in vermillion border of
  lip to complete opening extending to floor of the
  nose. May be unilateral or bilateral
• Cleft palate Fissure or small opening to
  complete opening between mouth and nasal cavity.
  May be isolated or occur with cleft lip

7
Cleft Lip and Cleft Palate Etiology

• Mulitfactorial Genetic increased incidence in
  families
• Environmental factors Drugs (anticonvulsants),
  alcohol, smoking, maternal illness, infection,
  folic acid deficiency, parental age

8
Cleft Lip and Cleft Palate Etiology

• Cleft lip with or without palate more common in
  males
• Cleft palate more common in females

9
Cleft Lip Complications

• Cleft Lip and Cleft Palate
• Facial disfigurement may cause shock, guilt and
  grief for the parents and may block parental
  bonding with the child
• Cleft Lip
• Abnormal development of external nose, nasal
  cartilages, nasal septum, usually nose is
  flattened
• Dental anomalies missing, malpositioned teeth on
  side of abnormality

10
Cleft Palate Complications

• Cleft Palate
• Infants have feeding problems because they are
  unable to develop suction due to opening between
  mouth and nasal cavity.
• Increases the risk of aspiration the increased
  open space in the mouth causes some formula to
  exit through the nose
• Increases the risk of upper respiratory
  infection and otitis media

11
Cleft Lip and Cleft Palate Diagnosis

• Prenatal by ultrasound (may be seen by 13 to 16
  weeks gestation)
• Apparent on assessment at birth
• Unable to generate negative pressure in oral
  cavity to suck and may impair ability to swallow
  resulting in feeding difficulties

12
Cleft Lip and Cleft Palate Therapeutic Management

• Multidisciplinary care plastic surgeon,
  neurosurgeon, orthodontist, otolaryngologist,
  speech pathologist, pediatrician
• Surgical closure of Cleft Lip 3 months or 12
  pounds
• Palate prosthesis may be used prior to surgery
  for a cleft palate. Cleft palate surgery is done
  before child develops faulty speech patterns
  (approximately 12 - 18 months)

13
Cleft Lip and Cleft Palate Therapeutic Management

• Prevention of complications infection, speech
  difficulties, malocclusion problems, hearing
  problems
• Facilitation of parent-child bonding, normal
  growth and development, speech and language
  development

14
Cleft Lip and Cleft Palate Nursing Management

• Newborn assessment assess ability to suck,
  swallow and feed
• Assess parents reaction to infant
• Teach feeding pre and post op Feed in an upright
  position, use soft, elongated nipple, frequent
  burping, prevent aspiration, adequate nutrition,
  breastfeeding
• Monitor weight gain
• Postop care Logan bow, restraints, pain
  medication, cleansing wound site and mouth,
  positioning (side or supine for cleft lip, side
  or prone for cleft palate)
• Discharge teaching and Home care planning
  feeding, suture care, restraints, pain management
  and follow-up

15
Esophageal Atresia (EA) and Tracheoesophageal
Fistula (TEF)
16
EA TEF

• Failure of esophagus to develop as a continuous
  passage to the stomach and failure of the
  esophagus and trachea to separate into distinct
  structures

17
EA TEF Clinical Manifestations

• Frothy saliva in mouth
• Drooling, coughing and choking
• Sudden coughing, gagging at feedings. Feeding
  comes out of the nose and mouth, becomes cyanotic
  and stops breathing as feeding is aspirated
• When the trachea joins the stomach, the stomach
  becomes distended with air

18
EA TEF Diagnosis

• Polyhydramnios should suggest the possibility of
  a high gastrointestinal obstruction
• Inability to identify fetal stomach bubble on a
  prenatal ultrasound
• Inability to pass a nasogastric tube into the
  stomach
• Radiographic studies curling of NG tube in upper
  esophagus, air in the stomach

19
EA TEF Therapeutic Management

• Maintain a patent airway
• Prevent pneumonia
• Gastric or blind pouch decompression
• Surgical repair as soon as possible
• Preferably, surgical repair is done in one stage,
  but may have to be done in two stages, based on
  infants condition
• 1st stage Close TEF and insert gastrostomy tube
• 2nd stage Connect ends of esophagus

20
EA TEF Nursing Considerations

• Preoperative care
• Maintain patent airway have suction available
• Thermoregulation
• Insert NG tube for gastric decompression
• Keep NPO
• Establish IV access to maintain hydration

21
EA TEF Nursing Considerations

• Postoperative care
• Maintain patent airway
• Prevent trauma to the anastomosis
• Elevate gastrostomy tube and keep open to allow
  gas to escape and gastric secretions to flow into
  the small intestine
• Provide emotional support to family, encourage
  bonding
• Teach parents gastrostomy care, signs of
  infection, signs of esophageal stricture

22
Gastroschisis and Omphalocele
23
Gastroschisis

• Extrusion of the large and small intestine
  through a defect in the abdominal wall to the
  right of the umbilical cord. The bowel has no
  covering membrane, and is exposed to the amniotic
  fluid

24
Omphalocele

• Herniation of abdominal contents through the
  umbilical cord. The bowel is covered by a
  protective sac, and the size of the defect is
  variable, ranging from 1cm to all of the
  abdominal contents

25
Gastroschisis and Omphalocele Diagnosis

• Maternal serum alpha-fetoprotein (elevated)
• Prenatal Ultrasound

26
Gastroschisis and Omphalocele Therapeutic
Management

• Cover exposed contents loosely with sterile
  saline-soaked pads and plastic drape to prevent
  water loss, drying and temperature instability.
  Gastroschisis be careful not to wrap around
  bowel can cause pressure necrosis as exposed
  bowel expands
• IV fluids
• Antibiotics
• Surgical closure primary or staged repair

27
Gastroschisis and Omphalocele Surgical Closure

• Primary closure All of the exposed bowel is
  placed inside the abdominal cavity and the
  abdomen is closed during a single procedure
• Staged repair When the abdominal cavity is not
  large enough to contain all of the exposed bowel,
  the abdominal wall is stretched and a silo is
  created. The edges of the abdominal wall are
  sutured to plastic sheeting that is then
  suspended with mild tension to the top of the
  incubator. The silo and the suture lines are
  covered with providone-iodine to prevent
  infection. The silo is gently compressed on a
  daily basis until all of the exposed bowel is
  enclosed in the abdominal cavity. This process
  takes several days (7-10) to complete. Once all
  of the exposed bowel is contained within the
  abdominal cavity, the silo is removed and the
  abdominal wall is closed

28
Gastroschisis and OmphalocelePotential
Complications

• Postoperative complications respiratory distress
  due to increased abdominal pressure, infection,
  intestinal obstruction, vena cava compression
  resulting in decreased blood flow to extremities,
  evisceration, intestinal volvulus

29
Gastroschisis and Omphalocele Nursing
Considerations

• Preoperatively careful handling and positioning,
  prevent infection, thermoregulation, assess
  respiratory status, fluid replacement
• Postoperatively mechanical ventilation, IV
  fluids, TPN, antibiotics, monitor for signs of
  complications, assess bowel function
• Family support, discharge teaching parenteral
  nutrition, oral stimulation, signs of bowel
  obstruction, follow-up care

30
Biliary Atresia

• Absence of bile duct or obstruction of bile duct
  prevents flow of bile from the liver to the
  gallbladder and small intestine
• Bile accumulates in the liver and bile plugs form
• Inflammation, edema and irreversible liver injury
  occur, resulting in fibrosis of the liver
• Causes excretion of bilirubin and bile salts in
  the urine

31
Biliary Atresia Clinical Manifestations

• Jaundice, developing between 2 weeks and 2 months
  of age
• Tea colored urine due to bilirubin and bile salt
  excretion
• Light colored stools due to absence of bile
  pigments
• Hepatomegaly
• Abdominal distention
• Bruising due to prolonged bleeding time
• Intense itching
• Failure-to-thrive, malnutrition as fat and
  fat-soluble vitamins can not be absorbed

32
Biliary Atresia Diagnosis and Treatment

• Diagnosed by ultrasound, liver biopsy and
  increased bilirubin levels
• Fatal without treatment
• Surgery hepatic portoenterostomy (Kasai
  procedure, formation of a substitute duct) to
  correct obstruction and allow for drainage of
  bile from liver into intestines
• Surgery is not a cure end stage liver disease
  may result in need for liver transplant

33
Biliary Atresia Nursing Considerations

• Monitor nutrition fat soluble vitamin
  supplements, TPN, Intralipids, daily weights
• Monitor IO, stool patterns
• Good skin care
• Discharge teaching (nutrition, skin care, support
  groups, signs of rejection)

34
Diaphragmatic Hernia

• Herniation of the abdominal contents through a
  defect in the diaphragm into the chest cavity and
  usually develops on the left side
• Intestines and abdominal structures enter the
  thoracic cavity, compressing the lung

35
Diaphragmatic Hernia Clinical Manifestations

• Severe respiratory distress
• Cyanosis, tachypnea, retractions
• Decreased or absent breath sounds on the side of
  the defect
• Barrel-shaped chest
• Heart sounds shifted to the right
• Bowel sounds heard over the chest
• Scaphoid abdomen

36
Diaphragmatic Hernia Diagnosis

• Prenatal ultrasound
• Chest x-ray

37
Diaphragmatic Hernia Treatment

• Respiratory support O2, intubation, No Bag/Mask
  ventilation, pulse oximeter
• Blood gases to monitor acid-base status
• Jet ventilator or High frequency ventilation for
  severe respiratory distress
• ECMO (extra corporeal membrane oxygenation)

38
Diaphragmatic Hernia Treatment

• Position head and thorax higher than abdomen,
  place on affected side
• IV fluids
• NG tube to decompress stomach/intestines
• Medications
• Opoids (Fentanyl) or paralyzing agents
  (pancuronium) if agitated
• Isotropic (Dopamine) for blood pressure support
  and improve cardiac output
• Surfactant to improve oxygenation
• Sodium bicarbonate to prevent acidosis

39
Diaphragmatic Hernia Treatment

• Surgical repair
• Fetal surgery
• Post-birth surgery return abdominal contents to
  abdomen and close defect in diaphragm

40
Diaphragmatic Hernia Nursing Considerations

• Preoperative care stabilization
• Postoperative care
• Position on affected side to facilitate expansion
  of unaffected lung
• Maintain respiratory support
• IV fluids, fluid and electrolyte balance
• Stress reduction
• Prevent infection
• Pain management
• Chest tube care
• Family support, promote bonding
• Discharge teaching (wound care, feedings,
  prevention of infection)

41
Anorectal Malformations

• Congenital anomalies of the anus and rectum
  caused by abnormal development
• Imperforate anus any abnormality without an
  obvious opening may have a fistula from rectum
  to perineum or GI tract

42
Anorectal Malformation Clinical Manifestations

• No anal opening
• Failure to pass meconium within 24 hours after
  birth
• Passing meconium through the urethra or vagina

43
Anorectal Malformations Diagnosis

• External physical exam
• X-rays with contrast
• Abdominal ultrasound

44
Anorectal Malformations Treatment

• Anal stenosis manual dilations
• Imperforate anal membrane excise membrane and
  manual dilations
• Perineal fistula Anoplasty and dilations
• High or intermediate malformations Colostomy,
  repair defects between 6-12 months

45
Anorectal malformations Nursing Considerations

• Complete assessment at birth
• Rectal temperature
• Asses for passage of meconium stool
• Preoperative care assist with diagnostic
  evaluation, IV fluids, gastrointestinal
  decompression

46
Anorectal Malformations Nursing Considerations

• Postoperative care
• perineal care, protective ointments and dressings
  to protect skin
• Positioning side-lying with hips elevated or
  supine with legs elevated to prevent pressure on
  perineal sutures
• Colostomy care
• NG tube for abdominal decompression
• IV fluids ( feedings begin when peristalsis
  returns)
• Family support
• Discharge teaching perineal and wound care,
  prevention of constipation, colostomy care,
  delayed toilet training, manual dilations,
  observe for complications, follow-up care

47
Pyloric Stenosis

• An increasing hyperplasia and hypertrophy of the
  circular muscle at the pylorus, narrows the
  pyloric canal. Most commonly seen in male
  infants between ages 1 and 6 months
• Hypertrophy eventually obstructs the pylorus
  opening. Obstruction results in inability of
  food to pass out of the stomach, causing vomiting

48
Pyloric Stenosis Clinical Manifestations

• Vomiting beginning at 3-6 weeks of age. Infant
  begins to regurgitate small amounts of formula
  immediately after feeding, which progresses to
  projectile vomiting
• Vomiting can occur during feedings, after
  feedings or several hours later
• Infant will be hungry after vomiting and will
  accept a second feeding
• Failure to gain weight
• Sign and symptoms of dehydration
• Palpable olive-shaped mass in right upper
  quadrant
• Visible peristaltic waves

49
Pyloric Stenosis Diagnosis

• History and physical
• UGI
• Ultrasound

50
Pyloric Stenosis Treatment

• Correct electrolyte imbalances and dehydration
• Pyloromyotomy split the muscle to make the
  opening larger

51
Pyloric Stenosis Nursing Considerations

• Record emesis frequency, characteristics,
  relationship to feedings
• IO
• Daily weights
• Pre-op care correct electrolytes, hydration,
  insert NG tube, family teaching

52
Pyloric Stenosis Nursing Considerations

• Post-op care
• IV fluids
• Begin feedings as ordered
• Place on right side with head elevated after
  feeding
• Monitor IO
• Monitor labs
• Wound care observe for drainage, redness, change
  dressings as ordered
• Pain management
• Discharge teaching Wound care, S/S of infection,
  feeding

53
Intussusception

• Invagination or telescoping of one portion of
  intestine into another portion of intestine, most
  common site is the ileocecal valve

54
Intussusception Clinical Manifestations

• Acute, colicky abdominal pain screaming and
  drawing knees to chest
• Non-bilious vomiting in early stage, becomes bile
  stained
• Blood and mucus in the stool (currant jelly
  stools)
• Sausage-shaped mass in right upper quadrant
• Abdomen is tender, distended
• Lethargy, fever, weak thready pulse, shallow
  respirations

55
Intussusception Diagnosis

• Barium or air contrast enema x-rays
• Abdominal ultrasound

56
Intussusception Treatment

• Non-surgical hydrostatic reduction with barium,
  water-soluble contrast or air enema
• Surgical intervention if there is evidence of
  intestinal perforation, peritonitis or shock, or
  if enema is unsuccessful

57
Intussusception Nursing Considerations

• Obtain history of symptoms from caregivers
• Explain procedures to caregivers
• Pre-intervention care correct electrolyte
  imbalance hemorrhage with fluid replacement,
  administer antibiotics, NG tube, monitor IO
• Post-intervention care observe for passage of
  contrast material
• Support family and encourage family to stay with
  pt
• Discharge teaching SS of recurrence

58
Hirschsprungs Disease

• Motility disorder of the bowel caused by the
  absence of parasympathetic ganglion cells in the
  large intestine

59
Hirschsprungs Disease Clinical Manifestations

• Infant failure to pass meconium within 24-48
  hours of birth, abdominal distention, bile
  stained vomiting, refusal to eat, intestinal
  obstruction
• Older infant and child chronic constipation,
  abdominal distention, explosive passage of stool,
  inadequate weight gain, ribbon-like or pellet
  shaped stool, foul-smelling stool, vomiting,
  palpable fecal mass

60
Hirschsprung's Disease Diagnosis

• Rectal exam absence of stool in rectum with
  tight anal sphincter
• Barium enema
• Rectal biopsy for absence of ganglion cells

61
Hirschsprungs Disease Treatment

• First stage surgery colostomy to allow for
  means of defecation and to rest bowel
• Second stage surgery aganglionic section is
  removed and normal bowel is anastomosed to rectum
  and the colostomy is closed. Performed when child
  is between 6-15 months of age and weighs 18-20
  pounds
• Newer surgery is to do second stage surgery
  without first stage surgery
• Laparoscopic Surgery pull affected bowel
  through anal opening to remove

62
Hirschsprungs Disease Nursing Considerations

• Pre-op management IVs for fluid and electrolyte
  balance, saline enemas to cleanse bowel, NPO, NG
  tube, antibiotics and antibiotic enemas
• Post-op management NG tube management until
  peristalsis returns, monitor abdominal distention
  and incisions, colostomy care
• Family teaching colostomy care, incision care,
  complications such as enterocolitis, leaks or
  strictures at anastomosis site (abdominal
  distention, irritability, diarrhea, vomiting,
  constipation)

63
Inflammatory Bowel Disease (IBD) Crohns
Disease and Ulcerative Colitis

• Chronic disorders that cause inflammation or
  ulceration in the small and large intestine
• Crohns disease may involve the entire
  gastrointestinal tract and all layers of the
  bowel wall, with an asymmetric distribution of
  lesions (disease-free skip areas)
• Ulcerative colitis involves inflammation of the
  mucosa and submucosa of the colon and rectum,
  with a symmetric, continuous distribution of
  lesions

64
IBD Clinical Manifestations

• Diarrhea
• Abdominal pain
• Rectal bleeding
• Weight loss
• Growth retardation
• Perianal disease (painful defecation, bright red
  rectal bleeding, skin tags, hemorrhoids,
  fistulas, and abscesses)
• Fistulas

65
IBD Diagnosis

• History and physical exam
• Endoscopy
• Colon x-rays barium enema, upper GI series, CT
  scan for bowel thickening
• Labs CBC (anemia), elevated WBC sed rate,
  hypoproteinemia, fluid and electrolyte
  imbalances, stool for blood, leukocytes, and
  culture
• Mucosal biopsy

66
IBD Treatment

• Control inflammatory process
• Corticosteroids,
• Sulfasalazine (acts directly on the bowel mucosa
  to reduce inflammation)
• Antibiotics, Flagyl (helpful for the perianal
  conditions associated with CD)
• Immunosuppressive medications

67
IBD Treatment

• Promote growth and development
• High-protein, high calorie foods
• Vitamin, iron, folic acid supplements
• Enteral feedings
• TPN
• Surgical treatment
• Total colectomy
• Subtotal colectomy with ileostomy
• Intestinal resections for obstructions

68
IBD Complications

• Malnutrition
• Growth failure
• Intestinal strictures
• GI bleeding
• Toxic megacolon (acute dilation of colon
  secondary to severe inflammation of bowel mucosa.
  S/S spiking fever, acute abdominal pain,
  distention colon can thin out and shred leading
  to hemorrhage, peritonitis, and death. If colon
  perforates, emergency surgery with total
  colectomy and ileostomy)

69
IBD Nursing Considerations

• Nutrition
• Several small meals/day
• Supplements (Ensure)
• Decrease bran and fiber foods
• Decrease dairy products
• Bland foods, avoid hot, spicy foods
• Medications
• Continue medications while in remission
• Monitor side effects
• Teach ostomy care and coping skills
• Give support and referrals
• Interventions to raise self-esteem

70
Necrotizing Enterocolitis (NEC)

• Necrosis of the mucosa of the small and large
  intestine, usually distal ileum and proximal
  colon
• Life-threatening condition of preterm infants
• Risk factors intestinal ischemia, bacterial
  colonization of bowel, presence of hypertrophic
  solutions (formula) in intestines, perinatal
  asphyxia, respiratory distress syndrome, exchange
  transfusions, umbilical artery catheters

71
NEC Clinical Manifestations

• Abdominal tenderness and distention
• Redness of abdominal wall
• Bloody stools
• Decreased bowel sounds
• Increased gastric residuals
• Bilious vomiting

72
NEC Clinical Deterioration

• Apnea and bradycardia
• Lethargy
• Temperature instability
• Decreased urine output
• Increased abdominal distention
• Shock (cool, mottled skin, pallor, decreased
  intensity of peripheral pulses)
• Hypotension
• Acidosis
• Sepsis

73
NEC Diagnosis

• Clinical findings
• Abdominal x-rays dilated bowel loops,
  pneumatosis intestinalis

74
NEC Treatment

• NPO
• NG tube for continuous gastric drainage and
  decompression
• O2 and ventilation if indicated
• IV fluids for parenteral nutrition, electrolytes,
  antibiotics
• Monitor labs CBC, blood gases, electrolytes
• Surgery if no improvement resection of necrotic
  bowel
• Postop complications intestinal obstruction due
  to strictures, short bowel syndrome

75
NEC Nursing Considerations

• Early identification of clinical manifestations
• Measure abdominal girth
• Gastric residual prior to feedings
• Presence of bowel sounds
• Hemoccult all stools
• Accurate IO
• Frequent VS
• Observe response when feedings begin again
• Family teaching answer question and explain
  procedures, care of ostomy, feedings, observation
  of complications

76
Short Bowel Syndrome

• Disorder characterized by inadequate surface are
  of the small intestine and usually occurs after
  surgical resection of the intestine in cases of
  necrotizing enterocolitis, volvus, or Crohns
  disease.
• May not be a permanent disorder because the
  intestine can grow and adapt

77
Short Bowel Syndrome Clinical Manifestations

• Malabsorption
• Malnutrition
• Diarrhea
• Electrolyte imbalances
• Vitamin and mineral deficiencies
• Skin irritation and breakdown on the buttocks and
  perineum
• Bacterial overgrowth in the remaining small
  intestine

78
Short Bowel Syndrome Treatment

• TPN via a central line
• Enteral feedings via an NG or gastrostomy tube to
  stimulate growth of the small intestine

79
Short Bowel Syndrome Nursing Considerations

• Provide good skin care
• Monitor IO
• Administer TPN and tube feedings
• Monitor for signs and symptoms of infection
• Monitor lab values liver function tests, renal
  function tests, liver enzymes, calcium, magnesium
  and phosphorus levels

80
Inborn Errors of Metabolism Phenylketonuria (PKU)

• PKU Disease of protein metabolism, characterized
  by the absence of the hepatic enzyme needed to
  metabolize the amino acid phenylalanine
• Inherited as an autosomal recessive trait
• Detected in 110,000 25,000 births
• Highest incidence in Caucasian children

81
PKU Clinical Manifestations

• Failure to thrive
• Frequent vomiting
• Irritability
• Hyperactivity
• Unpredictable, erratic behavior
• Bizarre behavior patterns in older children
  (screaming episodes, head banging, arm biting,
  disorientation, catatonia)
• Seizures

82
PKU Diagnosis

• Guthrie blood test performed on fresh heel blood,
  not cord blood
• Test is most reliable if blood sample is taken
  after the infant has ingested a source of protein
• Initial specimens that are taken within the first
  24 hours of life should be repeated by the second
  week of life
• After 3 months of treatment, a natural protein
  challenge test should be performed to confirm the
  diagnosis of PKU

83
PKU Treatment

• Low-phenylalanine diet
• Diet is calculated to maintain serum
  phenylalanine levels between 2 and 8mg/dl
• Significant brain damage occurs when levels are
  greater than 10-15mg/dl
• Growth retardation occurs at levels less than
  2mg/dl
• Daily amounts are individualized for each child
  and require frequent changes based on appetite,
  growth and development and blood levels

84
PKU Treatment

• This target is an easy way to visualize the foods
  allowed on the diet for PKU. Phenyl-Free is the
  center of the target diet. As the foods get
  further away from the bull's-eye, they are higher
  in phenylalanine. The foods outside the target
  are not included in the low-phenylalanine meal
  plan.

85
PKU Nursing Considerations

• Assessment and treatment of symptoms
• Teaching family dietary requirements
  low-phenylalanine foods, eliminated or restricted
  protein foods, no aspartame
• Assist school-age children and adolescents to
  cope with peer pressure, and to monitor daily
  allowance of phenylalanine
• Family support

86
Inborn Errors of Metabolism Galactosemia

• Error in carbohydrate metabolism
• Autosomal-recessive disorder
• Affects approximately 150,000 births
• Death occurs during the first month of life if
  untreated

87
Galactosemia Clinical Manifestations

• Jaundice
• Vomiting
• Weight loss
• Hepatosplenomegaly
• Diarrhea
• Drowsiness
• Lethargy
• Hypotonia
• Sepsis

88
Galactosemia Diagnosis

• Guthrie blood test
• History
• Physical exam
• Galactosuria
• Increased levels of galactose in the blood
• Decreased levels of enzyme

89
Galactosemia Treatment

• Eliminate all milk and lactose-containing foods,
  including breast milk

90
Galactosemia Nursing Considerations

• Same as for PKU except for lactose dietary
  restrictions
• Many drugs contain lactose as a filler and must
  be avoided
• Diet is easier to maintain because many more
  foods are allowed

91
Protein and Energy MalnutritionKwashiorkor and
Marasmus

• Kwashiorkor Deficiency of protein with low or
  adequate supply of calories from carbohydrates

92
Kwashiorkor

• Growing cells are damaged due to deficient
  protein for tissue growth and cell repair
• Clinical manifestations include
• Scaly, dry skin
• Loss of hair
• Night blindness
• Weight loss in conjunction with generalized edema
  (hypoalbuminemia)
• Hypokalemia
• Hypernatremia
• Muscle atrophy
• Diarrhea

93
Marasmus

• General malnutrition of both calories and protein
• Common in underdeveloped countries during times
  of drought
• Failure to thrive

94
Marasmus

• Gradual wasting and atrophy of body tissues,
  especially subcutaneous fat
• Clinical manifestations include
• Children appear to be very old, with flabby,
  wrinkled skin
• Severely emaciated appearance
• Small head with decreased brain growth
• No skin sores
• No edema
• Infection-prone
• Minimal body metabolism thermoregulation
  problems
• Apathetic, fretful withdrawn, lethargic

95
Kwashiorkor and Marasmus Treatment

• Diet with quality proteins, carbohydrates,
  vitamins, minerals introduced slowly
• Rehydrate with fluids to replace electrolytes
• Medications antibiotics, anti-diarrheals

96
Kwashiorkor and Marasmus Nursing Considerations

• Provide essential physiological needs
• Food
• Rest
• Protection from infection
• Hygiene
• Developmental stimulation
• Provide education about proper nutrition

97
Fluid and Electrolytes Developmental Differences

• Water constitutes approximately 80 of body
  weight of infants, 65 of body weight in
  children, and 50 of body weight in adults
• Infants have more extracellular fluid than
  intracellular fluid
• Infants and children lt2 years of age lose a
  greater proportion of fluids per day
• Infants have greater BSA to weight ratio and
  higher metabolism, making them more prone to
  fluid depletion and increased water lose through
  the skin

98
Fluid and Electrolytes Developmental Differences

• Infants and children have greater GI surface area
• Infants and children take in and excrete a
  greater volume of water daily
• Infants and children lt2 have immature kidney
  function and are inefficient in concentrating or
  diluting urine, conserving or excreting Na, and
  acidifying urine. They are more likely to become
  dehydrated with concentrated formula or
  overhydrated with dilute formula or excessive
  water
• Fluid disturbances in infants and children are
  usually caused by dehydration, water intoxication
  and edema

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Dehydration

• A critical condition that results from an
  extracellular fluid loss
• Children are more susceptible to dehydration than
  adults because a larger portion of a childs body
  fluid is located in extracellular spaces
• Dehydration that is not corrected will lead to
  hypovolemic shock and death

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Dehydration

• Hypotonic occurs when there is a sodium loss
  that is greater than the water loss
• Isotonic dehydration occurs when the sodium and
  water loss are equal. Most common type of
  dehydration in children
• Hypertonic occurs when the loss of water is
  greater than the loss of sodium

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Dehydration Clinical Manifestations

• Weight loss
• Rapid, thready pulse
• Hypotension
• Decreased peripheral circulation
• Decreased urinary output
• Dry mucous membranes
• Absence of tears
• Sunken fontanel

• Dehydration is classified as
• Mild (lt5 weight loss)
• Moderate (5-10 weight loss)
• Severe (gt10 weight loss)

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Dehydration Diagnosis

• Based on history, physical exam
• Measurement of acute weight loss
• Hypotonic and isotonic dehydration hemoglobin,
  hematocrit, glucose, BUN, creatine and protein
  elevated. Urine is highly concentrated, dark
  amber in color, has strong odor

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Dehydration Treatment

• Oral rehydration is the preferred route, using a
  solution that contains glucose, sodium, potassium
  and bicarbonate.
• If child has persistent vomiting, severe
  dehydration, or is unable to drink for other
  reasons, rehydration is achieved through
  administration of IV fluids, based on the type of
  dehydration

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Dehydration Nursing Considerations

• Physical assessment for signs and symptoms of
  dehydration
• Daily weights
• Measure IO
• Administer IV fluids
• Teach caregiver the signs of dehydration
• Teach parents about oral rehydration fluids

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Any Questions?