For finals pt.3(start Hemophilia )

• Hemophilia

  -Linked recessive trait

  -Males inherit hemophilia from their mothers and females inherit the carrier status from their fathers
• • Hemophilia A - deficiency of Factor VIII
  • Hemophilia B - deficiency of factor IX
• Give assessment of hemophilia

  Prolonged bleeding after minor injury

  • At birth after cutting cord
  • Following circumcision
  • Following IM immunization
• Management of Hemophilia

  • Prepare to administer Factor VIII concentrate / cryoprecipitate
  • Thaw slowly; gently rotate bottle

  Check notes
• Imperforated anus


  Failure of the 2 sections of the bowel to meet or if the membrane between the surfaces does not dissolve during the 7th week of intrauterine life
• PATHOPHYSIOLOGY

  • Interference with anorectal structure development at varying stages leads to various anomalies
  • Continued communication between the urogenital tract and rectal portions of the cloacal plate causes rectourethral fistulas or rectovestibular fistulas
• Assessment of imperforated anus

  • No anal formation
  • Inability to insert rectal thermometer
  • A membrane filled with black meconium is seen protruding from the anus
  • No stool is passed leading to abdominal distention
• LABORATORY AND DIAGNOSTIC OF IMPERFORATED ANUS

  • PE
  • Lower abdominal X-ray
  • Abdominal and pelvic U/Z
  • IV pyelogram and voiding cystourethrogram
• MEDICAL MANAGEMENTImperforated Anus

  surgical correction unless fistula is present

  Possible colostomy with multi-staged surgical repair
• Nursing Management of Imperforated Anus

  Post-op, NGT is inserted for decompression

  - Check bowel sounds

  - Small oral feedings are resumed when bowel sounds are present

  - Maintain the suture line clean by irrigating it with normal saline

  - Take temperature through the axilla

  - Position side-lying with the legs flexed or in prone position to keep the hips elevated to reduce edema on pressure on the surgical site

  - Provide colostomy care, if present

  - A new colostomy stoma - red and edematous
• INTUSSUSCEPTION


  -INVAGINATION / TELESCOPING of the portion of the intestine into another - ileocecal valve
• most common cause of obstruction of intussusception is in the first 2 years of life
• Risk factors of Intussusception

  • cystic fibrosis, celiac disease and gastroenteritis
• PATHOPHYSIOLOGY of intussusception

  • Inflammation and ischemia
  • eventual tissue necrosis, perforation, and peritonitis
• Assessment of intussusception

  • Paroxysmal, severe abdominal pain, which causes them to draw up their legs and cry
  • olive size bulge under right rib cage
  • vomiting
  • projectile; bile-stained fecal emesis
  • peristaltic waves during and after feeding
  • failure to thrive
  • dehydration
  • blood in stool after 12 hours and “currant jelly” in appearance
  • distended abdomen
• LABORATORY AND DIAGNOSTIC OF INTUSSUSCEPTION

  • abdominal X-ray
  • barium enema
  • reveal affected area of intestine
  • UGIS
  • narrowing of diameter of pylorus
  • decreased serum Na, K, Cl - increased Hct - metabolic alkalosis
• Treatment INTUSSUSCEPTION


  : hydrostatic reduction by barium enema
• Surgery Intussusception


  performed if reduction by barium edema is not successful or tissue necrosis is present
• Medications of intussusception

  antibiotics to treat peritonitis

  analgesics to manage postoperative pain
• NURSING MANAGEMENT:

  • Monitor for signs of perforation and shock and report immediately
  • Antibiotics, IV fluids, and decompression via nasogastric tube may be prescribed
  • Monitor for passage of normal, brown stool, which indicates that the intussusception has reduced itself
  • Monitor for the return of normal bowel sounds, for the passage of barium and the characteristics of stool Administer clear liquids and advance the diet gradually as prescribed
  • Provide post-operative care if surgery is indicated
• Pyloric stenosis


  classified as a congenital defect is characterized by hypertrophy of the circular muscle fibers of the pylorus

  the average affected infant does not show symptoms until about the third week of life
• Pyloric stenosis


  the infant often eats well and gains weight and then starts vomiting occasionally after meal

  • Alkalosis
  • Constipation and urine is scanty
• Pyloric stenosis


  Gastric peristaltic waves passing from left to right across the abdomen seen during or after feedings
• Diagnosis of pyloric stenosis

  Ultrasonographic or radiographic

  barium swallow show an abnormal retention of barium in the stomach and increased peristalsis
• Treatment of pyloric


  pyloromyotomy (also known as a Fredet-Ramstedt operation)
• Spina bifida


  collective term for all spinal cord disorders
• DIAGNOSTIC OF SPINA BIFIDA

  • maternal serum assay or amniocentesis of alpha-
  • fetoprotein levels (15wk)Sonogram
• Types of defects in spina bifida

  • Anencephaly
  • Microcrocephaly
  • Meningocele
  • MYELOMENINGOCELE
  • Encephalocele
• ANENCEPHALY


  absence of the cerebral hemispheres

  On visual inspection at birth, the disorder is obvious
• MICROCROCEPHALY

  • a defect in brain development associated
• In microcrocephaly, there is maternal phenylketonuria or an intrauterine infection such as rubella.

  
  

  It may also result from severe malnutrition or anoxia in early infancy
• MENINGOCELE


  If the meninges covering the spinal cord herniated through unformed vertebrae, a meningocele occurs

  The protrusion may be covered by a layer of skin or only the clear dura mater
• MYELOMENINGOCELE


  the spinal cord and the meninges protrude through the vertebrae defect the same as with a menigocele. The difference is that the spinal cord often ends at the point of the defect, so motor and sensory function is absent beyond this point
• In myelomeningocele, the child will have flaccidity and lack of sensation of the lower extremities and loss of bowel and bladder control. The infant’s legs are lax; urine and stools continually dribble because of lack of sphincter control.
• Myelomeningocele accompanies talipes (clubfoot) defect
• Types of clubfoots

  1. Varus
  2. Valgus
  3. Equinus
  4. Calcaneus
• ENCEPHALOCELE


  a cranial meningocele or myelomeningocele.

  occurs most often in the occipital area of the skull but may occur as a nasal or nasopharyngeal defect
• Asssesment of encephalocele

  • All types of neural defects are readily visible at birth
  • during intrauterine life by sonography, fetoscopy, amniocentesis or analysis of AFP in maternal serum
  • An infant without sphincter control voids continually, this pattern is the same for defecating
• THERAPEUTIC MANAGEMENT

  surgery is done as soon after birth as possible usually within 24 hours
• NURSING MANAGEMENT

  • Never allow the sac to dry; cracked, ruptured sac can lead to quick decompression of the CSF