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Thursday, March 29, 2012

CELIAC DISEASE (CD):


Also known as Gluten- induced enteropathy (GSE ) and celiac sprue, is a disease of the small intestine  characterized by abnormal mucosa and permanent intolerance to gluten.CD is second  only to cystic fibrosis as a case of malabsorption in children
Frequency
·         It varies from 1 in 300 to 1 in 4000 , and appears to be declining , possibly in relation to environmental factors
·         It is seen more commonly in Europe than in America  and is rarely reported in Asians and Africans
·         The exact cause of CD is unknown , but that appears to be an inherited predisposition with an influence by environmental factors
·         seen between 1-5yrs of age
Etiology
·         Gluten was identified as causative agent in 1953
·         Gluten is found in wheat, rye, barley & oats. Alpha glandin fraction is responsible factor having strong association with HLA a8, DRW3 histocompatibility antigen
·         Environmental trigger appears to be necessary
·         an inherited disease. Celiac disease effects those with a genetic predisposition.
Pathogenesis
Factors suggesting immunological basis-
·          Antibodies to alpha glandin demonstrated in serum and intestinal secretions
·         Serum IgA are high & IgM are low
·         Favorable response to immunosuppressive agents

Clinical manifestations
The first evidence of the disease may be FTT and iarrhea
·         Impaired fat absorption
®    Steatorrhea (excessively large , pale, oily, frothy stools)
®    Exceedingly fowl smelling stools
·         Impaired absorption of the nutrients
®    Malnutrition
®    Muscle wasing(especially prominent in legs and buttocks)
®    Aneamia
®    Anorexia
®    Abdominal distension
·         Behavioural changes
®    Irritability
®    Fretfulness
®    Uncooperativeness
®    Apathy
·         Celiac crisis
®    Acute, severe episodes of profuse watery diarrhea and vomiting
®    May be precipitated by
¾    Infections(especially GIT)
¾    Prolonged fluid and electrolyte depletion
¾    Emotional disturbances
Diagnosis:
¾    Clinical picture
¾    H/o improvement with exclusion of wheat  from diet and recurrence of diarrhea on reintroduction of wheat , rye, barley, or oats in the diet
¾    Increased excretion of fats
¾    Serum xylose levels less than 20 mg / 100ml 2 hours after administration of 0.5 gm of xylose per kg of body weight
¾    Jejuna biopsy shows characteristic villous atropy
¾    Evidence of secondary lactose deficiency
Complication
·         osteoporosis
·         lymphoma of the small intestine
·         infertility
·         autoimmune liver disease
Treatment
Ø  Exclusion of all cereals containing gluten(wheat, rye, barley, oats)
Ø  Gradual introduction of foods in acute cases with simple sugar, banana, skimmed milk rice& lentil gruel(khichri)
Ø  Milk and lactose containing diets withheld for first few weeks as secondary lactose deficiency is common
Ø  Celiac crisis to be treated energetically with rehydration therapy
Ø  Corticosteroids can be used in celiac crisis and in patients who are symptomatic in spite of gluten withdrawal for 1 week
Ø  Iron and vitamine suppliments to counteract aneamia and vitamine deficiencies
Ø  Prolonged i/v alimentation required in very severe chronic cases before they start tolerating oral feeds
Ø  Iron and folate suppliments
Ø  Vitamine A and D in water soluble forms
Ø  In children with chronic diarrhea secondary to bile acid malabsorption, the use of cholestyramine (Questran) to bind bile acids may help to reduce the duration and severity of the diarrhea.

Nursing care
Nursing assessment
  • steatorrhea
  • Chronic diarrhea or constipation or both
  • Anorexia
  • Generalized malnutrition and failure to thrive
  • Coagulaton difficulty
  • Irritability
  • Anemia
  • Clubbed fingers
  • Dental enamel defect
  • Vomiting rash
  • Electrolyte study for ca, protein(hypocalcemia, hypoalbuminemia)
  • Blood study for HB, BT, CT(hypothrombinemia)
  • Stool specimen for high fat
Nursing diagnosis
  1. Imbalanced nutrition less than body requirement related to poor absorption of the nutrients
  2. Chronic pain – abdomen related to the disease condition
  3. Fluid electrolyte imbalance related to underlying pathology
  4. High risk for complication – anemia, bleeding related reduced thrombin level
  5. Alteration in comfort related to the disease condition
  6. Anxiety related to the unexpected outcome of the disease
  7. Altered parental coping related to the need for long term care
Nursing interventions
This can be divided as :
  1. Assisting with the diagnosis
  2. Assisting parents in their adjustment to the diagnosis
  3. Providing nutritious diet
  4. Correcting nutritional deficiencies
  5. Prevention and care during celiac crisis
  6. Educating the child and parents during longterm follow up care
In genrral;
  • Eliminate all gluten from the food
  • Give the child corn and rice product , soy and potato flour, breast milk or soy – based formula, and fresh fruits
  • Replace vitamins and calories ; give small frequent meals
  • Monitor for staetorrhoea its disappearance is a good indication that the child’s ability to absorb nutrients is improving

HERNIA


Introduction:-
A hernia is a protrusion of a portion of an organ  or organs through an abnormal opening.
Incidence
      About 5 out of 100 children have inguinal hernias (more boys than girls).
      Some may not have symptoms until adulthood.
Causes, and risk factors
      Usually, there is no obvious cause of a hernia, although they are sometimes associated with heavy lifting.
      Hernias can be seen in infants and children. This can happen when the lining around the abdominal organs does not close properly before birth.
      If you have any of the following, you are more likely to develop a hernia:
- Family history of hernias
  - Cystic fibrosis
  - Undescended testicles
  - Extra weight
  - Chronic cough
  - Chronic constipation from straining to have bowel movements
  - Enlarged prostate from straining to urinate
Types
      Diaphragmatic
      Hiatal
§  sliding
      Epigastric hernia
      Abdominal
§  Umblical
§  Omphalocele
§  gastroschisis
      Intraabdominal
      Inguinal hernia
§  Direct
§  Indirect
A diaphragmatic hernia
§  Is a birth defect, which is an abnormality that occurs before birth as a fetus is forming in the mother's uterus.
§  An opening is present in the diaphragm (the muscle that separates the chest cavity from the abdominal cavity).
§  With this type of birth defect, some of the organs that are normally found in the abdomen move up into the chest cavity through this abnormal opening.
types of diaphragmatic hernia:
Bochdalek hernia
  A Bochdalek hernia involves an opening on the left side of the diaphragm. The stomach and intestines usually move up into the chest cavity.
Morgagni hernia
  A Morgagni hernia involves an opening on the right side of the diaphragm. The liver and intestines usually move up into the chest cavity.
Incidence
®    Bochdalek hernia:
§  makes up about 90 percent of all cases.
§  occurs in one out of every 2,200 to 5,000 live births.
®    Morgagni hernia makes up two percent of all cases
®    Parents who have had one child with an isolated diaphragmatic hernia   have  2 percent or two in 100.
®    Morgagni hernia is more common in girls than boys, whereas Bochdalek hernia is slightly more common in boys than girls.
®    Babies with the Bochdalek type of diaphragmatic hernia are more likely to have another birth defect.Almost 20 percent have a congenital heart defect.
®    Between 5 to 16 percent have a chromosomal abnormality.
Causes of  diaphragmatic hernia
®    As a fetus is growing in its mother's uterus before birth, different organ systems are developing and maturing.
®    The diaphragm forms between the 7th and 10th week of pregnancy.
®    The esophagus (the tube that leads from the throat to the stomach), the stomach, and the intestines are also developing at this time.
®    In a Bochdalek hernia, the diaphragm may not develop properly, or the intestine may become trapped in the chest cavity as the diaphragm is forming.
®    In a Morgagni hernia, the tendon that should develop in the middle of the diaphragm does not develop properly.
®    In both cases, normal development of the diaphragm and the digestive tract does not occur.
®    Diaphragmatic hernia is a multifactorial condition, which means that "many factors," both genetic and environmental, are involved.
Symptoms of a diaphragmatic hernia
The symptoms of a Bochdalek diaphragmatic hernia are often observable soon after the baby is born they are .
§  difficulty breathing
§  fast breathing
§  fast heart rate
§  cyanosis (blue color of the skin)
§  abnormal chest development, with one side being larger than the other
§  abdomen that appears caved in (concave)
§  A baby born with a Morgagni hernia may or may not show any symptoms.
Diagnosis
§  physical examination.
§  A chest x-ray to look at the abnormalities of the lungs, diaphragm, and intestine.
§  A blood test known as an arterial blood gas is often performed to evaluate the baby's breathing ability.
§  blood test for chromosomes (to determine if there is a genetic problem)
§  ultrasound of the heart (echocardiogram)
Treatment for a diaphragmatic hernia:
Specific treatment will be determined by your baby's physician based on the following:
¾    when the problem is diagnosed (during pregnancy or after birth)
¾    your baby's overall health and medical history
¾    the severity of the problem
¾    your baby's tolerance for specific medications, procedures, or therapies
¾    your opinion or preference
Definitive Treatment may include:
Neonatal intensive care
¾    Babies with diaphragmatic hernia are often unable to breathe effectively on their own because their lungs are underdeveloped.
¾    Most babies will need mechanical ventilator to help their breathing.
¾    Correct acidosis
¾    GI decompression
ECMO
¾    Some infants may need to be placed on a temporary heart/lung bypass machine called ECMO if they have severe problems.
¾    ECMO may be used temporarily while a baby's condition stabilizes and improves.
Surgery
¾    When the baby's condition has improved, the diaphragmatic hernia will be repaired with an operation.
¾    The stomach, intestine, and other abdominal organs are moved from the chest cavity back to the abdominal cavity. The hole in the diaphragm is repaired.
¾    Many babies will need to remain in the NICU for a while after surgery. Although the abdominal organs are now in the right place, the lungs still remain underdeveloped. The baby will usually need to have breathing support for a period of time after the operation. Once the baby no longer needs help from a breathing machine (ventilator), he/she may still need oxygen and medications to help with breathing for weeks, months, or years.
Nursing care  :  preop
¾    Reduce stimulation- envt/ care stimulation
¾    Promt recognition , resuscitation and stabilisation
¾    Maintain suction , o2 & IVF
¾    Positioning – head up
¾    medications
Nursing care  :  postop
¾    Carryout routine post op care and observation
¾    Relieve pain and discomfort
¾    Support family because it is a critical illness
Complications
¾    Many babies will have chronic lung disease and may require oxygen or medications to help their breathing for weeks, months, or years.
¾    Many babies will have gastroesophageal reflux
¾    Some babies will have difficulty growing. This is known as failure to thrive
¾    Delayed milestone
¾    Prone for hearing loss
Hiatal hernia
Sliding: protrusion of an abdominal structure usually stomach  through esophagial hiatus
Symptoms
¾    Dysphagia
¾    FTT
¾    Vomiting‘
¾    Neck contortions
¾    Frequent unexplined respiratory problem
¾    Bleeding
¾    Usually associated with GER
¾    May cause gastric volvulus and obstruction
Dignosis
¾    Fluroscopy
Management
¾    Management of GERD symptoms
¾    Positioning
¾    Pharmacological measures
¾    Dietary management
¾    Surgical management hen complications are related to GERD  despite medical management
Nursing care
¾    Be alert to significant signs and carryout routine post op. care
Epigastric
Epigastric  hernia
Introduction
A midline protrusion may be due to the failure of the rectus abdominis muscles to join inutero.This occurs in the midline on the linea alba in the midline between the umblicus and lower end of sternum.
Feature
¾    Severe pain
¾    When running a finger down the midline of the abdomen while the child is standing , if hernia persist a small protrusion may be felt between the fingers of the linea alba
Tratment
Surgical correction
Abdominal
Umbilical Hernia
Weakness in the abdominal wall around umbilicus ; incomplete closure of abdominal wall , allowing the abdominal contents to protrude through opening
Incidence
High in premature and african American infants
Symptoms
¾    Noted by inspection and palpation of the abdomen
¾    Usually closes spontaneously by 1-2 yrs of age

Therapeutic  management
§  No treatment for small cases
§  Operative repair if persist  to age 4-6 yrs or if defects is>1.5-2cm by age 2
Nursing care
§  Discourage use of home remadies ( eg belly bands, coins etc )reassure parents
Omphalocele
Omphalocele
Protrusion of intraabdominal viscera into the base of the umbilical cord, sac is covered with peritoneum without skin
Gastroschisis
Gastroschisis
Protrusion of the intra abdominal containts through the defect in the abdominal wall lateral to umbilical ring , there is never a peritoneal sac
Intraabdominal hernia
Intraabdominal
Is caused by a loop of bowel slipping out of its normal position and becoming caught in a defective area of the mysentery or becoming compressed between the bands of the peritoneum
Intestinal obstruction can occur
Inguinal hernia
Incidence:
§  3% in full term infants.
§  Hernias are more common in premature males.
Indirect  inguinal hernia
§  the protruding loop of bowel passes obliquely through the abdominal wall,while in
a direct inguinal hernia
§  the herniating bowel pushes directly forward through the abdominal wall, taking a shorter course to the outside.
Diagnosis
§  Palpable mass along the inguinal canal.
§  Placing a finger tip on the scrotum and extending the finger into the inguinal canal will demonstrate a patent tunnel and one may feel bowel within the canal.
Treatment:
§  Inguinal hernias do not spontaneously resolve so surgery is required. If bowel becomes entrapped within the inguinal canal an incarcerated hernia can occur. This is a surgical emergency because the entrapped bowel can become ischemic.
LAPAROSCOPIC HERNIA REPAIR
§  During laparoscopic surgery, we make a small ½ inch cut in the skin at the belly button.
§  Then a cannula (thin tube) is introduced in between the muscle fibres without cutting any of the muscle.
§  Through the cannula, the laparoscope is inserted into the patient's body.
§  It is equipped with a tiny camera and light source that allows it to send images through a fibre-optic cord to a television monitor.
§  The television monitor shows a high resolution magnified image. Watching the monitor, the surgeon can perform the procedure.
§  Herniorrhaphy
§  Herniotomy
Nursing care  preoperative care
§  Infant should be fed until few hours before the surgery
§  Preanesthetic medicines as ordered
Post operative care
§  Shift from the surgical facility when the effects of GA over and the child able to tolerate oral feeds
§  Keep the incision clean and dry
§  Sponge bath
§  Change soiled linen
Conclusion :
    Hernia which is the protrusion of organ or organs through the abnormal opening ,varies in its severity based on its location and the organs involved and the age of the child.
BIBLIOGRAPHY:
1.      Wong D.L etal . Essentials Of Paediatric Nursing. 6th edition. Missouri: Mosby;2001
2.      Marlow D.R. Redding B. Textbook of Paediatric nursing. 1st edition.Singapore: Harwourt Brace & company; 1998
3.      Dr.Chaudari KC. Indian Journa of Paediatrics. Nov22 2007
4.      Parthasarathy IAP textbook of Paediatrics. 2nd edition. jaypee: NewDelhi; 2002
Judith A.S Straight A’s in Paediatric Nursing. 2nd edition.Wolters Kluwer : Newyork;2008