UNIT III: METABOLIC DISORDERS REVIEW OF ANATOMY AND PHYSIOLOGY I.

GASTROINTESTINAL SYSTEM Functions: Digestion Absorption Elimination Four Divisions: 1. UPPER - oral cavity - esophagus - stomach 2. MIDDLE Small Intestine - duodenum - jejunum - ileum 3. LOWER Large Intestine - cecum, colon, rectum 4. ACCESSORY ORGANS - salivary glands - liver and gall bladder - pancreas MOUTH contains the lips, cheeks, palate, tongue, teeth, salivary glands, muscles, and maxillary bones forms the entryway of GIT for food ESOPHAGUS a collapsible muscular tube about 10 inches long carries food from the pharynx to the stomach STOMACH comprised of the cardia, fundus, body, and pylorus serves as the food storage during early stage of digestion Two Sphincters: lower esophageal (cardiac) sphincter pyloric sphincter

Jejunum - approximately about 8 ft long Ileum - approximately about 12 ft long - terminal part of small intestine LARGE INTESTINE 5 feet long, 6-7 cm diameter absorbs water and eliminates wastes Three Divisions: 1. CECUM - blind pouch that projects down at the junction of ileum and colon Ileocecal valve - prevents the reflux of feces from the cecum to small intestine APPENDIX - wormlike structure - arise from the cecum, approximately 2.5 cm

2. COLON 3 subdivisions: a. Ascending colon b. Transverse colon c. Descending colon d. Sigmoid colon 3. RECTUM ACCESSORY DIGESTIVE ORGANS Pancreas Liver and Gall Bladder Salivary glands LIVER largest gland in the body, weighing 3 to 4 lbs. contains Kupffer cells, which remove bacteria in the portal venous blood removes excess glucose and amino acids from the portal blood synthesizes glucose, amino acids, and fats aids in the digestion of fats, carbohydrates, and proteins stores and filters blood (200-400 mL) stores vitamins A, D, and B and iron secretes bile to emulsify fats (5001000 mL of bile a day) HEPATIC DUCTS deliver bile to the gallbladder via the cystic duct deliver bile to the duodenum via the common bile duct the common duct opens into the duodenum, with the pancreatic duct at the Ampulla of Vater GALLBLADDER stores and concentrates bile

SMALL INTESTINE major digestive enzyme portion of final digestion and food absorption Three Parts/Subdivision: Duodenum - contains the openings of the bile and pancreatic ducts - approximately 22 cm

contracts to force bile into the duodenum during the digestion of fats cystic duct joins the hepatic duct to form the common bile duct Sphincter of Oddi guards the entrance into the duodenum presence of fatty materials in the duodenum stimulates the liberation of cholecystokinin, which causes contraction of the gallbladder and relaxation of the sphincter of Oddi

variable extension from the uvula and soft and hard palate

PANCREAS Two Divisions: 1. EXOCRINE GLAND secretes sodium bicarbonate pancreatic juices contain enzymes for digesting carbohydrates, fats, proteins 2. ENDOCRINE GLAND Islets of Langerhans secrete insulin Insulin is secreted into the bloodstream and is important for carbohydrate metabolism Secretes glucagons to raise blood glucose levels Secretes somatostatin to exert a hypoglycemic effect DISTURBANCES IN INGESTION

Problems associated with cleft lip and cleft palate Eating problems: food and liquids can pass from the mouth back through the nose Ear infections/hearing loss: prone to fluid build-up in the middle ear causing hearing loss Speech problems: nasal sound, speech difficult to understand Dental problems: missing, extra, malformed, or displaced teeth Interventions 1. Assess the ability to suck, swallow, handle normal secretions, and breathe without distress. 2. Assess fluid and calorie intake daily and monitor weight. 3. Modify feeding techniques; plan to use specialized feeding techniques, and special nipples. 4. Hold the child in upright position and direct the formula to the side and back of the mouth to prevent aspiration. 5. Feed small amounts gradually and burp frequently. 6. Position on side after eating 7. Encourage breast feeding if appropriate. 8. Encourage the parents to describe their feelings related to the deformity. 9. Teach the parents the *ESSR method of feeding to allow the child to finish swallowing what has been placed in the mouth. *ESSR (ENLARGE the nipple, STIMULATE the suck reflex, SWALLOW, REST) 10. Teach the parents the *ESSR method of feeding to allow the child to finish swallowing what has been placed in the mouth. 11. Keep suction equipment and bulb syringe at bedside. 12. Have babys ear checked anytime he or she has a cold or URT infection. 13. Talk normally to baby (NO baby talk); talk often. Surgical Management Cheiloplasty repair of cleft lip; performed during the first weeks of life (2-3 months).

CLEFT LIP/PALATE A congenital anomaly Result of failure of soft tissue or bony structure to fuse during embryonic development Defects involve abnormal openings in the lip or palate that may occur unilaterally or bilaterally Causes Genetic (chromosome abnormalities) Environmental factors (pesticide exposure) exposure to radiation Rubella virus Teratogenic factors (eg. alcohol, anti-seizure/anticonvulsant medications, steroids) Assessment Cleft lip ranges from a slight notch to a complete separation from floor of the nose Cleft palate include nasal distortion midline or bilateral cleft

Rule of Ten: 10 wks old, 10 lbs, 10 gm hgb NOTE! Closure of cleft lip precedes that of the palate. Uranoplasty or Palatoplasty repair of cleft palate done between 12 and 18 months cleft palate is closed before the child develops faulty speech habits ESOPHAGEAL ATRESIA Blind pouch at each end , widely separated and with no communication to the trachea. The esophagus terminates before it reaches the stomach. TRACHEOESOPHAGEAL FISTULA The proximal esophageal segment terminates in a blind pouch, and the distal segment is connected to trachea. A fistula is present that form an unnatural connection with trachea. Different Types Predisposing Factors Associated with: premature infants low birth weight main etiology is unknown * Failure of the embryonic esophagus and trachea to develop and separate correctly Assessment INITIAL SIGNS Frothy saliva in the mouth and nose; drooling Regurgitation and poor feeding Coughing and choking during feedings LATE SIGNS Cyanosis after feeding Apnea Abdominal distention CARDINAL SIGN Excessive amount of frothy saliva in the mouth Diagnosis Inability to pass a small gauge (Fr. 5) OGT via mouth into the stomach Coiled OGT/NGT on plain films suggest non- passage of the tube due to an atretic esophagus Barium-enhanced x-ray examination may reveal a dilated esophageal pouch Surgical Management

Thoracotomy with division and ligation of tracheoesophageal fistula Thoracotomy and end to end anastomosis Gastrostomy

NOTE: Treatment is done ASAP! Baby may be fed intravenously until surgery is performed. Mucus and saliva continuously removed via a catheter. Surgery within the first 24 hours of life if with no other abnormalities of the heart and or lungs.

HIATAL HERNIA Also known as Diaphragmatic Hernia A portion of the stomach herniates through the diaphragm and into the thorax Causes Congenital weakness of the muscles of the diaphragm Aggravated by factors that increase abdominal pressure such as: - pregnancy - tumors - ascites - heavy lifting - obesity TWO TYPES 1. Sliding Hiatal Hernia upper segment of the stomach including the esophageal-stomach junction slides through the opening in the diaphragm in relation to position changes Caused by a muscle weakness in the esophageal hiatus 2. Paraesophageal/Rolling Hiatal Hernia upper segment of the stomach moves into the chest; the esophageal-stomach junction remains in normal position. caused by anatomic defect in the hiatus Assessment Heartburn/pyrosis Regurgitation & vomiting Dysphagia Feeling of fullness Management MEDICATIONS Antacids Antiemetics

Histamine receptor antagonist Gastric acid secretion inhibitors AVOID: anticholinergics, Ca channel blockers, diazepam (lowers LES pressure)

Surgery Fundoplication : the gastric fundus of the stomach is wrapped around the lower end of the esophagus and stitched in place, reinforcing the closing function of the lower esophageal sphincter Nursing Interventions 1. Relieve pain 2. Modify diet: a. High CHON diet to enhance LES pressure b. SFF (4-6) and minimize the amount of liquid c. Eat slowly and chew food properly d. Avoid: fatty foods, cola, coffee, tea, chocolate, alcohol (may cause decrease LES pressure) e. Upright position before and after eating (1-2 hrs) f. Do not eat at least 3 hours before bedtime to prevent nightmare reflux g. No evening snacks h. Reduce weight 3. Promote lifestyle changes a. Elevate head of the bed 6-12 in. for sleep b. Avoid factors that increase intraabdominal pressure c. Avoid smoking (causes rapid and significant drop in LES pressure) Complications Ulceration Hemorrhage Regurgitation and aspiration of stomach of contents Strangulation Incarceration of the stomach in the chest with possible necrosis , peritonitis and mediastinitis PYLORIC STENOSIS Hypertrophy of the pyloric sphincter causing stenosis and obstruction Develops in the first few weeks of life 5:1000 live births, common in Caucasians More common in males (4:1) 2-5x more common in first born

Etiology: Unknown Signs and Symptoms Apparent between 2nd-6th wk of life Vomiting (from mild regurgitation to forceful & projectile usually after feeding) Vomitus may contain gastric contents, mucus, blood tinged but does not contain bile Hunger & irritability Failure to thrive visible gastric peristalsis (form left to right across the epigastrium during or immediately following feeding) palpable olive-shaped mass to the right of the epigastric area signs of dehydration; electrolyte imbalances Diagnosis History and physical exam Abdominal USD UGIS: reveals narrowed diameter of pylorus Serum electrolytes: decreased Na, K & Cl ABG: metabolic alkalosis Nursing Interventions 1. Monitor VS, I&O, weight. 2. Monitor for signs of dehydration and electrolyte imbalances. 3. Prepare the child and parents for pyloromyotomy. Surgery Pyloromyotomy: incision into the muscle of the pylorus; may be done by laparoscopy (a.k.a. Fredet-Ramstedt operation)

DISTURBANCES IN ABSORPTION CELIAC DISEASE It is an immunological (allergic) reaction within the inner lining of the small intestine to proteins (gluten). Prevalence Common in European countries, particularly in Ireland, Italy, Sweden, and Austria.

Symptoms Etiology Idiopathic in 90% of pediatric cases Three Etiologies 1. Intraluminal Mass is pulled forward by peristalsis and brings attached bowel wall with it 2. Intramural Abnormality of bowel wall causes it not to contract properly, allowing a kink which serves as a lead point 3. Extraluminal Factor (e.g. inflamed appendix) causes area of abnormal peristalsis, allowing a kink which serves as a lead point Epidemiology Majority are in children Peak incidence 5-9 months of age Approximately 10% occur in adults Seen in all age groups Equal in males and females Signs and Symptoms severe colicky abdominal pain piercing cry and child draws knees to abdomen vomiting of bile-stained fecal material or gastric contents currant jelly-like stools (with blood and mucus) tender and distended abdomen palpable sausage-shaped mass in the RLQ hypoactive or hyperactive bowel sounds Management Barium enema: to reduce the telescoping by hydrostatic pressure (Defer if signs perforation and shock occur) Antibiotic, IV fluids NGT to decompress GI Surgery Colonic: surgical resection Enteroenteric: may require resection or manual reduction during surgery, may be treated with enema reduction, or may require no intervention Nursing Interventions Routine pre- and post-op care for abdominal surgery Monitor f/e and treat for imbalances Monitor bowel sounds Watch for signs of peritonitis and refer

Cause and Risk Factors Cause: unknown Risk Factors: It tends to be more common in people who have: Type 1 diabetes Autoimmune thyroid disease Down syndrome Microscopic colitis Complications Malnutrition Loss of calcium and bone density Cancer Lactose intolerance Neurologic deficits Tests and Diagnosis Blood Tests Collecting a sample of small intestine for testing Swallowing a camera pill that collects pictures of the small intestine Treatment: There is no cure except for change in diet (GLUTEN FREE) Drugs Summary C= E = Education about the disease L= I = Identification of nutritional deficiencies A = Access to advocacy groups C= INFLAMMATORY DISORDERS INTUSSUSCEPTION Definition telescoping of the bowel into itself (usually at the lower valve) common about 6 months of age more common in boys than girls associated with cystic fibrosis and celiac disease

Monitor for signs of perforation and shock (fever, heart rate, changes in level of consciousness, or blood pressure, and respiratory distress)

HIRSCHSPRUNGS DISEASE Definition A congenital absence of ganglion cells in the rectum and upward in the colon Also known as congenital aganglionosis or aganglionic megacolon Causes absence of autonomic parasympathetic ganglion cells usually 2-25 cm proximally from the anus results in mechanical obstruction from the inadequate motility in the distal segment Signs and Symptoms failure in passing meconium abdominal distension loose stool: ribbon-like stool nausea, anorexia and lethargy bile stained or fecal vomiting Weight loss or failure to grow Diagnostic Exam Rectal Biopsy - confirm presence or absence of ganglion cells Management drug therapy: stool softener isotonic enemas diet therapy Surgery palliative: loop or double bowel colostomy corrective: abdominoperineal pullthrough bowel containing ganglia is pulled down and anastomosed to the rectum Nursing Interventions Administer enemas as ordered do not use tap water or soap suds enema in infants because of danger of water intoxication use volume appropriate to weight of child Infants: 150-200 ml Children: 250-500 ml

Do not treat loose stools; child is constipated Administer TPN Provide low residue diet Client teaching and discharge planning concerning colostomy care and decrease residue

Hernia Protrusion of a tissue, structure, or part of an organ through the muscle tissue or the membrane by which it is normally contained. Characteristics Hernias can be classified according to their anatomical location. Inguinal Hernia A. Indirect Congenital in origin Herniation thru the spermatic cord then to the inguinal canal Common in male children and young adult Common in right side B. Direct Weakness of the muscle of the abdominal wall Common on elderly men than women

Ventral Hernia Incisional hernia Occurs at the site of previous surgical incision that healed inadequately Risk Factors Infection, inadequate nutrition, distension, obesity Femoral Hernia Hernia occurs thru the femoral ring More common in female High incidence of strangulation and incarceration Umbilical Hernia Due to increased abdominal pressure More common in female Usually occurs in obese andmultiparous women Causes Predisposing Factors: Heavy lifting Family history of hernias Undescended testicles Extra weight Chronic cough Chronic constipation Enlarged prostate

Stretching of muscles during pregnancy Scars from previous surgery

Signs and Symptoms Pain at the site Visible or palpable lump Vague symptoms resulting from pressure on an organ which has become "stuck" sometimes leading to organ dysfunction Medical Management Mechanical reduction Use of truss held by a belt to reduce hernia Surgical Management Uncomplicated hernias are principally repaired by pushing back, or "reducing", the herniated tissue, and then mending the weakness in muscle tissue (herniorrhaphy). When to Contact a Medical Professional If hernia cannot be pushed back into the abdomen using gentle pressure If with nausea, vomiting, or fever Hernia becomes red, purple, dark, or discolored Groin pain, swelling, or a bulge Umbilical hernia fails to heal on its own by the time your child is 5 years old DIVERTICULOSIS and DIVERTICULITIS Diverticulosis: an outpouching of the intestinal mucosa Non-inflamed diverticula Can occur in any part of the intestine but most common in the sigmoid colon Divericulitis: an inflammation of one or more diverticula that results a diverticula to perforate Undigested foods block the diverticula-invasion by bacteria Incidence Common above 45 years old Obese Etiology and Risk Factors Low fiber diet Muscle weakness of the bowel Signs and Symptoms LLQ pain which increases with coughing, straining, or lifting Diarrhea, constipation Elevated temperature

Nausea and vomiting Abdominal distension and tenderness Palpable tender rectal mass Blood in the stool in 15% of cases Change in bowel pattern, and character Fever, abdominal pain and urinary manifestation

Medical Management Diet therapy if asymptomatic High fever diet, bulk laxative NPO NGT feeding IV fluids, antibiotics Report to the physician Surgical Management Indication: hemorrhage, obstruction, abscess and perforation Ligation and removal of the sac Resection of the bowel involved Temporary colostomy Nursing Interventions Maintain on NPO status Administer IV or TPN Restrict clients activities Monitor bowel sounds, abdominal tenderness, peritonitis, hemorrhage and perforation Monitor stools (color, consistency, +/- blood) Low residue, high protein diet Avoid gas forming foods and milk products Avoid smoking Administer antimicrobial agents, corticosteroids

IMPERFORATE ANUS Description A defect that occurs during the 5th to 7th wee k of fetal development Incidence Affects 1 in 5,000 babies and is slightly more common in males.

TYPES: Low imperforate anus where the rectum may be in the normal location but is too tight to allow the infant to have a bowel movement Intermediate imperforate anus where the opening is too close to the urethra

High imperforate anus where the rectal opening is completely absent

Diagnostic Test Physical examination of the anus Abdominal X-rays: provide a general overview of the anatomical location of the imperforate anus in a crosstable lateral view Signs and Symptoms Failure to pass stool within 1st 24 hours Abdominal distention Presence of stool in the urine Nausea and vomiting Surgical Management Low imperforate anus is repaired with anoplasty. This is an operative procedure done to move the fistula opening back to the anal sphincter. Colostomy: usually done within first day of life. A colostomy allows the infant to have normal bowel movements and relieves the bowel obstruction DISTURBANCES IN ELIMINATION APPENDICITIS Description Characterized by inflammation of the appendix. Mild cases may resolve without treatment; most require removal of the inflamed appendix. Untreated, mortality is high, mainly due to peritonitis and shock. Causes Foreign bodies Trauma Intestinal worms Lymphadenitis Obstructing fecalith Manifestations Fours Signs 1. ROVSINGS Pressure over the descending colon causes pain in the right lower quadrant of the abdomen 2. PSOAS Occasionally, an inflamed appendix lies on the psoas muscle and patient will lie with the right hip flexed for pain relief. Elicited by passively extending the thigh of a patient lying on the side with knees extended, or

asking the patient to actively flex thigh at the hip. + abdominal pain = + positive psoas sign *a positive psoas sign on the right may suggest appendicitis* 3. OBTURATOR If an inflamed appendix is in contact with the obturator internus, spasm of the muscle can be demonstrated by flexing and internally rotating the hip causing pain in the hypogastrium. 4. BLUMBERG Rebound tenderness Deep palpation of the viscera over the suspected inflamed appendix followed by sudden release of the pressure causes the severe pain on the site Diagnostics Doppler ultrasound CT scan CBC leukocytosis Surgery PERITONITIS Description Inflammation of the peritoneum, the serous membrane which lines part of the abdominal cavity and viscera. May be localized or generalized, and may result from infection Signs and Symptoms The main manifestations of peritonitis: (called PERITONISM) 1. Acute abdominal pain 2. Abdominal tenderness 3. Abdominal guarding *which are exacerbated by moving the peritoneum* Causes and Types 1. Infected peritonitis: associated with presence of pathogens Perforation of part of the GIT most common causes: PUD, gastric cancer, appendicitis, Crohns disease, ingestion of a sharp objects (fish bone, toothpick) Disruption of the peritoneum - letting micro-organisms into the peritoneal cavity - trauma, surgical wound - continuous peritoneal dialysis - intra-peritoneal chemotherapy

Spontaneous bacterial peritonitis (SBP) - is a peculiar form of peritonitis occurring in the absence of an obvious source of contamination. Systemic infections

2. Non-infected peritonitis - leakage of sterile body fluids into the peritoneum. blood (e.g. Endometriosis, blunt abdl trauma) gastric juice (e.g. peptic ulcer, gastric CA) bile (e.g. liver biopsy) urine (pelvic trauma) 3. Primary peritonitis - is usually caused by liver disease. 4. Secondary peritonitis - is caused by other conditions that allow bacteria, enzymes, or bile into the peritoneum from a hole or tear in GI or biliary tracts Diagnostics Blood tests to see if bacteria present in the blood Samples of fluid from the abdomen identify the bacteria causing the infection CT scan identifies fluid in the abdomen, or an infected organ X-rays detect air in the abdomen, which indicates that an organ may be torn or perforated Treatment General supportive measures IVF, rehydration, electrolyte replacement IV Antibiotics, broad-spectrum antibiotics Surgery: (exploratory laparotomy) is needed to perform a full exploration and lavage of the peritoneum