Etiology

Despite decades of research, the etiology of IBD is not completely understood, and there is no known cure. There is evidence to indicate a multifactorial etiology. Research is focused on theories of defective immunoregulation of the inflammatory response to bacteria or viruses in the GI tract in individuals with a genetic predisposition (Silbermintz and Markowitz, 2006). In CD the chronic immune process is characterized by a T helper 1 cytokine profile, whereas in UC the response is more humoral and mediated by T helper 2 cells (Silbermintz and Markowitz, 2006).

Development of IBD has a genetic influence. Several IBD susceptibility genes have now been identified through family and twin studies (Sauer and Kugathasan, 2010). Family-based genetic studies have linked chromosome 6 in UC and the NOD2 gene in CD (Sauer and Kugathasan, 2010).

Pathophysiology

The inflammation found with UC is limited to the colon and rectum, with the distal colon and rectum the most severely affected. Inflammation affects the mucosa and submucosa and involves continuous segments along the length of the bowel with varying degrees of ulceration, bleeding, and edema. Thickening of the bowel wall and fibrosis are unusual, but longstanding disease can result in shortening of the colon and strictures. Extraintestinal manifestations are less common in UC than in CD. Toxic megacolon is the most dangerous form of severe colitis.

The chronic inflammatory process of CD involves any part of the GI tract from the mouth to the anus but most often affects the terminal ileum. The disease involves all layers of the bowel wall (transmural) in a discontinuous fashion, meaning that between areas of intact mucosa, there are areas of affected mucosa (skip lesions). The inflammation may result in ulcerations; fibrosis; adhesions; stiffening of the bowel wall; stricture formation; and fistulas to other loops of bowel, bladder, vagina, or skin.

Clinical Signs and Symptoms

Children with UC may experience mild, moderate, or severe symptoms, depending on the extent of mucosal inflammation and systemic symptoms. Most include bloody diarrhea or occult fecal blood, abdominal pain, and varying degrees of systemic manifestations and growth abnormalities (Beattie, Croft, Fell, et al, 2006; Leichtner and Higuchi, 2004). One of the earliest signs of UC may be growth failure with decreased linear growth velocity (Beattie, Croft, Fell, et al, 2006). Growth failure is most likely a result of chronic poor dietary intake caused by anorexia related to GI symptoms. UC often manifests with the insidious onset of diarrhea, possibly with hematochezia, and usually without fever or weight loss. The course of the disease may remain mild with intermittent exacerbations. Some children and adolescents are seen with grossly bloody diarrhea, cramps, urgency with defecation, mild anemia, fever, anorexia, weight loss, and moderate signs of systemic illness. Severe UC is characterized by frequent bloody stools, abdominal pain, significant anemia, fever, and weight loss. Extraintestinal manifestations are less common in UC than in CD and may precede colitis. The erythrocyte sedimentation rate (ESR) may be elevated, indicating a systemic response to an inflammatory process. Enlarged lymph nodes (lymphadenopathy), arthritis, and the skin lesions of erythema nodosum may be present.

Common presenting manifestations of CD include diarrhea, abdominal pain with cramps, fever, and weight loss. Mild GI symptoms, poor growth, and extraintestinal manifestations may be present for several years before overt GI symptoms are present. Both malabsorption and anorexia are factors that contribute to the growth problems that are prevalent in CD. The effects of UC and CD are listed in Fig. 33-4.

Children with CD have multiple risk factors for impaired bone accrual, including poor growth, delayed maturation, malnutrition, decreased activity, chronic inflammation, and steroid therapy (Dubner, Shults, Baldassano, et al, 2009). Growth delay persists in many children with CD following diagnosis, despite improved disease activity (Pfefferkorn, Burke, Griffiths, et al, 2009).

The disease process can also involve the colon, causing diarrhea, cramps, and urgency with defecation. Signs of colitis, such as gross rectal bleeding or stool with occult blood, are similar to those seen in UC. Perianal disease, including skin tags, abscesses, fissures, and fistulas, is a feature of CD. Extraintestinal manifestations include erythema nodosum, pyoderma gangrenosum, arthralgia and arthritis, uveitis and episcleritis, sclerosing cholangitis, autoimmune hepatitis, nephrolithiasis, and pneumonitis (Silbermintz and Markowitz, 2006). Table 33-2 provides a comparison of UC and CD.

Diagnostic Evaluation

The diagnosis of UC and CD comes from the history, physical examination, laboratory evaluation, and other diagnostic procedures. Laboratory tests include a CBC to evaluate anemia and an ESR or CRP to assess the systemic reaction to the inflammatory process. Levels of total protein, albumin, iron, zinc, magnesium, vitamin B₁₂, and fat-soluble vitamins may be low in children with CD. Stools are examined for blood, leukocytes, and infectious organisms. A serologic panel is often used in combination with clinical findings to diagnose IBD and to differentiate between CD and UC. Observational studies on the utility of blood tests to detect perinuclear antineutrophilic cytoplasmic antibodies (pANCA) and anti-Saccharomyces cerevisiae antibodies (ASCA) showed that the combination is specific, but not sensitive for diagnosing ulcerative colitis (Reese, Constantinides, Simillis, et al, 2006).

In patients with CD, an upper GI series with small bowel follow-through assists in assessing the existence, location, and extent of disease. Upper endoscopy and colonoscopy with biopsies are an integral part of diagnosing IBD (Langan, Gotsch, Krafczyk, et al, 2007). Endoscopy allows direct visualization of the surface of the GI tract so that the extent of inflammation and narrowing can be evaluated. CT and ultrasound also may be used to identify bowel wall inflammation, intraabdominal abscesses, and fistulas. CD lesions may pierce the walls of the small intestine and colon, creating tracts called fistulas between the intestine and adjacent structures such as the bladder, anus, vagina, or skin.

Therapeutic Management

The natural history of the disease continues to be unpredictable and characterized by recurrent flare-ups that can severely impair patients’ physical and social functioning (Vernier-Massouille, Balde, Salleron, et al, 2008). The goals of therapy are to (1) control the inflammatory process to reduce or eliminate the symptoms, (2) obtain long-term remission, (3) promote normal growth and development, and (4) allow as normal a lifestyle as possible. Treatment is individualized and managed according to the type and the severity of the disease, its location, and the response to therapy.

Nursing Care Management

The nursing considerations in the management of IBD extend beyond the immediate period of hospitalization. These interventions involve continued guidance of families in terms of (1) managing diet; (2) coping with factors that increase stress and emotional lability; (3) adjusting to a disease of remissions and exacerbations; and (4) when indicated, preparing the child and parents for the possibility of diversionary bowel surgery.

Because nutritional support is an essential part of therapy, encouraging the anorexic child to consume sufficient quantities of food is often a challenge. Successful interventions include involving the child in meal planning; encouraging small, frequent meals or snacks rather than three large meals a day; serving meals around medication schedules when diarrhea, mouth pain, and intestinal spasm are controlled; and preparing high-protein, high-calorie foods such as eggnog, milkshakes, cream soups, puddings, or custard (if lactose is tolerated). (See Feeding the Sick Child, Chapter 27.) Using bran or a high-fiber diet for active IBD is questionable. Bran, even in small amounts, has been shown to worsen the patient’s condition. Occasionally the occurrence of aphthous stomatitis further complicates adherence to dietary management. Mouth care before eating and the selection of bland foods help relieve the discomfort of mouth sores.

When NG feedings or TPN is indicated, nurses play an important role in explaining the purpose and the expected outcomes of this therapy. The nurse should acknowledge the anxieties of the child and family members and give them adequate time to demonstrate the skills necessary to continue the therapy at home if needed (see Critical Thinking Exercise).

The importance of continued drug therapy despite remission of symptoms must be stressed to the child and family members. Failure to adhere to the pharmacologic regimen can result in exacerbation of the disease. (See Compliance, Chapter 27.) Unfortunately, exacerbation of IBD can occur even if the child and family are compliant with the treatment regimen; this is difficult for the child and family to cope with.

Etiology

The exact cause of PUD is unknown, although infectious, genetic, and environmental factors are important. There is an increased familial incidence, and the disease is increased in persons with blood group O.

There is a significant relationship between the bacterium Helicobacter pylori and ulcers. H. pylori is a microaerophilic, gram-negative, slow-growing, spiral-shaped, and flagellated bacterium known to colonize the gastric mucosa in about half of the population of the world (Sung, Kuipers, and El-Serag, 2009). H. pylori synthesizes the enzyme urease, which hydrolyses urea to form ammonia and carbon dioxide. Ammonia then absorbs acid to form ammonium, thus raising the gastric pH. H. pylori may cause ulcers by weakening the gastric mucosal barrier and allowing acid to damage the mucosa. It is believed that it is acquired via the fecal-oral route, and this hypothesis is supported by finding viable H. pylori in feces.

In addition to ulcerogenic drugs, both alcohol and smoking contribute to ulcer formation. There is no conclusive evidence to implicate particular foods, such as caffeine-containing beverages or spicy foods, but polyunsaturated fats and fiber may play a role in ulcer formation. Psychologic factors may play a role in the development of PUD, and stressful life events, dependency, passiveness, and hostility have all been implicated as contributing factors.

Pathophysiology

Most likely, the pathologic condition is due to an imbalance between the destructive (cytotoxic) factors and defensive (cytoprotective) factors in the GI tract. The toxic mechanisms include acid, pepsin, medications such as aspirin and nonsteroidal antiinflammatory drugs (NSAIDs), bile acids, and infection with H. pylori. The defensive factors include the mucus layer, local bicarbonate secretion, epithelial cell renewal, and mucosal blood flow. Prostaglandins play a role in mucosal defense because they stimulate both mucus and alkali secretion. The primary mechanism that prevents the development of peptic ulcer is the secretion of mucus by the epithelial and mucus glands throughout the stomach. The thick mucus layer acts to diffuse acid from the lumen to the gastric mucosal surface, thus protecting the gastric epithelium. The stomach and the duodenum produce bicarbonate, decreasing acidity on the epithelial cells and thereby minimizing the effects of the low pH (Chelimsky and Czinn, 2001). When abnormalities in the protective barrier exist, the mucosa is vulnerable to damage by acid and pepsin. Exogenous factors, such as aspirin and NSAIDs, cause gastric ulcers by inhibition of prostaglandin synthesis.

Zollinger-Ellison syndrome may occur in children who have multiple, large, or recurrent ulcers. This syndrome is characterized by hypersecretion of gastric acid, intractable ulcer disease, and intestinal malabsorption caused by a gastrin-secreting tumor of the pancreas. The pathogenesis, manifestations, and complications of PUD are outlined in Fig. 33-5.

Clinical Manifestations

The clinical manifestations of PUD vary according to the child’s age and the ulcer’s location. Common clinical manifestations include chronic abdominal pain, especially when the stomach is empty, such as during the night or early morning; recurrent vomiting; hematemesis; melena; chronic anemia; and abdominal tenderness (Box 33-9).

Diagnostic Evaluation

Diagnosis is based on the history of symptoms, physical examination, and diagnostic testing. The focus is on symptoms such as epigastric abdominal pain, nocturnal pain, oral regurgitation, heartburn, weight loss, hematemesis, and melena. History should include questions relating to the use of potentially causative substances such as NSAIDS, corticosteroids, alcohol, and tobacco. Laboratory studies may include a CBC to detect anemia, stool analysis for occult blood, liver function tests (LFTs), sedimentation rate, or CRP to evaluate IBD; amylase and lipase to evaluate pancreatitis; and gastric acid measurements to identify hypersecretion. A lactose breath test may be performed to detect lactose intolerance.

Radiographic studies such as an upper GI series may be performed to evaluate obstruction or malrotation. An upper endoscopy is the most reliable procedure to diagnose PUD. A biopsy can determine the presence of H. pylori. A blood test can also identify the presence of the antigen to this organism. The ¹³C urea breath test measures bacterial colonization in the gastric mucosa. This test is used to screen for H. pylori in adults and children. Polyclonal and monoclonal stool antigen tests are an accurate, noninvasive method both for the initial diagnosis of H. pylori and for the confirmation of its eradication after treatment (Gisbert, de la Morena, and Abraira, 2006).

Diagnosis is based on the history (pattern of pain) and physical examination. Frequently a history of epigastric and periumbilical pain accompanies PUD. However, children often find it difficult to describe the location of their pain and frequently indicate the location by moving their hand in a circular movement all around the stomach area. Asking the child to take one finger and point to the area where it hurts the most often helps to identify the location of the pain. Pain may also be elicited during the examination with palpation. Routine laboratory studies to diagnose PUD include a CBC with differential, ESR, blood chemistry studies, urinalysis, and stool analysis to identify anemia or inflammation and to rule out infection. A ¹³C urea breath test is often performed to determine the presence of antibodies to H. pylori. An upper GI series is rarely helpful in identifying ulcers in children; fiberoptic endoscopy is the most reliable way to detect PUD in children. Direct visualization of the gastric and duodenal mucosa with biopsy to determine the presence of H. pylori is the most commonly used and effective way to arrive at the diagnosis.

Therapeutic Management

The major goals of therapy for children with PUD are to relieve discomfort, promote healing, prevent complications, and prevent recurrence. Management is primarily medical and consists of administration of medications to treat the infection and to reduce or neutralize gastric acid secretion. Antacids are beneficial medications to neutralize gastric acid. Histamine (H₂) receptor antagonists (antisecretory drugs) act to suppress gastric acid production. Cimetidine, ranitidine, and famotidine are examples of these medications. These medications have few side effects.

PPIs, such as omeprazole and lansoprazole, act to inhibit the hydrogen ion pump in the parietal cells, thus blocking the production of acid. Although these drugs have not been well studied in children, thy are utilized in clinical practice to treat ulcers, GER, esophagitis, and gastritis. They appear to be well tolerated and to have infrequent side effects (e.g., headache, diarrhea, nausea, vomiting).

Mucosal protective agents, such as sucralfate and bismuth-containing preparations, may be prescribed for PUD. Sucralfate is an aluminum-containing agent that forms a barrier over ulcerated mucosa to protect against acid and pepsin. Sucralfate is available in both pill and liquid forms. Because sucralfate blocks the absorption of other medications, it should be given separately from other medications.

Bismuth compounds are sometimes prescribed for the relief of ulcers, but they are used less frequently than PPIs. Although these compounds inhibit the growth of microorganisms, the mechanism of their activity is poorly understood. In combination with antibiotics, bismuth is effective against H. pylori. Although concern has been expressed about the use of bismuth salts in children because of potential side effects, none of these side effects has been reported when these compounds have been used in the treatment of H. pylori infection.

Triple-drug therapy is the standard first-line treatment regimen for H. pylori (O’Connor, Gisbert, and O’Morain, 2009). Combination therapy has demonstrated 90% effectiveness in eradication of H. pylori when compared with antibiotic monotherapy. Examples of drug combinations used in triple therapy are (1) bismuth, clarithromycin, and metronidazole; (2) lansoprazole, amoxicillin, and clarithromycin; and (3) metronidazole, clarithromycin, and omeprazole. The benefits on the use of probiotics as an adjunct to treatment remain unclear, with conflicting literature on their effect on eradication and minimizing side effects (O’Connor, Gisbert, and O’Morain, 2009).

Common side effects of medications include diarrhea, nausea, and vomiting. In addition to medications, the child with PUD should have a nutritious diet and avoid caffeine. Warn adolescents about gastric irritation associated with alcohol use and smoking.

Children with an acute ulcer who have developed complications, such as massive hemorrhage, require emergency care. The administration of IV fluids, blood, or plasma depends on the amount of blood loss. Replacement with whole blood or packed cells may be necessary for significant loss.

Surgical intervention may be required for complications such as hemorrhage, perforation, or gastric outlet obstruction. Ligation of the source of bleeding or closure of a perforation is performed. A vagotomy and pyloroplasty may be indicated in children with recurring ulcers despite aggressive medical treatment.

Nursing Care Management

The primary nursing goal is to promote healing of the ulcer through compliance with the medication regimen. If an analgesic-antipyretic is needed, acetaminophen, not aspirin or NSAIDs, is used. Critically ill neonates, infants, and children in intensive care units should receive H₂ blockers to prevent stress ulcers.

For nonhospitalized children with chronic illnesses, consider the role stress plays. In children, many ulcers occur secondary to other conditions, and the nurse should be aware of family and environmental conditions that may aggravate or precipitate ulcers. Children may benefit from psychologic counseling and from learning how to cope constructively with stress.

Pathophysiology

The circular muscle of the pylorus thickens as a result of hypertrophy. This produces severe narrowing of the pyloric canal between the stomach and the duodenum. Consequently, the lumen at this point is partially obstructed. Over time the size of the opening is reduced, and the partial obstruction may progress to complete obstruction. The hypertrophied pylorus may be palpable as an olivelike mass in the upper abdomen (Fig. 33-6).

Pyloric stenosis is not a congenital disorder. Evidence suggests that local innervation may be involved in the pathogenesis. In most cases this is an isolated lesion; however, it may be associated with intestinal malrotation, esophageal and duodenal atresia, and anorectal anomalies.

Clinical Manifestations

Infants with HPS have nonbilious vomiting in the early stages (Box 33-11). The vomiting may be projectile and progressive, becoming brown in later stages if gastritis develops. Vomiting usually begins at 3 weeks of age but can start as early as 1 week and as late as 5 months. Initially the infant is hungry and irritable, but prolonged vomiting may lead to dehydration, weight loss, and failure to thrive. Gastric peristalsis may be visible on examination, and the olive-shaped mass in the epigastrium just to the right of the umbilicus may be palpated (see Fig. 33-6, A).

Diagnostic Evaluation

The diagnosis of HPS is often made after the history and physical examination. The olivelike mass is most easily palpated when the stomach is empty, the infant is quiet, and the abdominal muscles are relaxed. If the diagnosis is inconclusive from the history and physical examination, ultrasonography will demonstrate an elongated mass surrounding a long pyloric canal. If ultrasonography does not demonstrate a hypertrophied pylorus, upper GI radiography should be done to rule out other causes of vomiting.

Laboratory findings reflect the metabolic alterations created by severe depletion of both water and electrolytes from extensive and prolonged vomiting. There are decreased serum levels of both sodium and potassium, although these may be masked by the hemoconcentration from extracellular fluid depletion. Of greater diagnostic value are a decrease in serum chloride levels and increases in pH and bicarbonate (carbon dioxide content), indicative of metabolic alkalosis. The blood urea nitrogen will be elevated as evidence of dehydration.

Therapeutic Management

Surgical relief of the pyloric obstruction by pyloromyotomy is the standard therapy for this disorder. Preoperatively the infant must be rehydrated and metabolic alkalosis corrected with parenteral fluid and electrolyte administration. Replacement fluid therapy usually delays surgery for 24 to 48 hours. The stomach is decompressed with an NG tube. In infants with no evidence of fluid and electrolyte imbalance, surgery is performed without delay.

The surgical procedure is often performed by laparoscope and consists of a longitudinal incision through the circular muscle fibers of the pylorus down to, but not including, the submucosa (pyloromyotomy, sometimes called Fredet-Ramstedt procedure) (see Fig. 33-6, B). The procedure has a high success rate. The use of a small incision for the laparoscope may result in a shorter surgical time, more rapid postoperative feeding, and shorter hospital stay (van der Bilt, Kramer, van der Zee, et al, 2004).

Feedings are usually begun 4 to 6 hours postoperatively, beginning with small, frequent feedings of glucose, water, or electrolyte solution. If clear fluids are retained, about 24 hours after surgery formula is started in the same small increments. The amount and the interval between feedings are gradually increased until a full feeding schedule is reinstated, which usually takes about 48 hours.

Nursing Care Management

Nursing care involves primarily observation for clinical features that help establish the diagnosis, careful regulation of fluid therapy, and reestablishment of normal feeding patterns. Nurses must be alert to signs of HPS in infants and refer them for medical evaluation. Consider the possibility of HPS in the very young infant who appears alert but fails to gain weight and has a history of vomiting after meals. Base assessment on observation of eating behaviors, evidence of characteristic clinical manifestations, hydration, and nutritional status.

Preoperatively the emphasis is on restoring hydration and electrolyte balance. The infant is kept NPO and given IV fluids of glucose and electrolytes based on serum electrolyte values—usually sodium chloride solution with added potassium (when there is adequate urinary output). Careful monitoring of the IV fluids and strict monitoring of intake, output, and urine specific gravity are important. Record accurate description of any vomiting and the number and character of stools.

Observations include assessment of vital signs, particularly those that indicate fluid or electrolyte imbalances. These infants are especially prone to metabolic alkalosis from loss of hydrogen ions and depletion of potassium, sodium, and chloride, all of which are contained in gastric secretions. Assess the skin and mucous membranes for alterations in hydration status; daily weights provide added clues to water gain or loss. (See Chapter 28 for manifestations of fluid and electrolyte disturbances.)

When stomach decompression and gastric lavage are part of preoperative management, the nurse is responsible for ensuring that the NG tube is patent and functioning properly and that the type and amount of NG drainage is recorded. General hygienic care, with particular attention to the skin and mouth in dehydrated infants, is important. Protection from infection is essential because infants with impaired nutritional status are more susceptible to infection than normal newborns.

Encourage parents to visit and become involved in the child’s care. Most parents need support and reassurance that the condition is caused by a structural problem and is not a reflection of their parenting skills and capacities.

Pathophysiology

Intussusception occurs when a proximal segment of the bowel telescopes into a more distal segment, pulling the mesentery with it. The mesentery is compressed and angled, resulting in lymphatic and venous obstruction. As the edema from the obstruction increases, pressure within the area of intussusception increases. When the pressure equals the arterial pressure, arterial blood flow stops, resulting in ischemia and the pouring of mucus into the intestine. Venous engorgement also leads to leaking of blood and mucus into the intestinal lumen, forming the classic currant jelly–like stools (Wyllie, 2007). The most common site is the ileocecal valve (ileocolic), where the ileum invaginates into the cecum and then further into the colon (Fig. 33-7). Other forms include ileoileal (one part of the ileum invaginates into another section of the ileum) and colocolic (one part of the colon invaginates into another area of the colon) intussusceptions, usually in the area of the hepatic or splenic flexure or at some point along the transverse colon.

Pathophysiology Review

Clinical Manifestations

Intussusception usually manifests with the sudden onset of crampy abdominal pain, inconsolable crying, and a drawing up of the knees to the chest in an otherwise healthy child (Box 33-12). Between episodes the child appears normal. As the obstruction progresses, bilious vomiting may occur and lethargy increases. The classic triad of intussusception symptoms (abdominal pain, abdominal mass, bloody stools) is present in only 29% to 33% of children (Huppertz, Soriano-Gaabarro, Grimprel, et al, 2006). With atypical cases, lethargy may be the primary symptom. If the distal bowel remains distended, necrosis and perforation are possible.

Diagnostic Evaluation

Frequently the diagnosis can be made on subjective findings alone. However, definitive diagnosis is based on a barium enema, which clearly demonstrates the obstruction to the flow of barium. Initially an abdominal radiograph is obtained to detect intraperitoneal air from a bowel perforation, which would contraindicate a barium enema. A rectal examination reveals mucus, blood, and occasionally a low intussusception itself.

Therapeutic Management

Conservative treatment consists of radiologist-guided pneumoenema (air enema) with or without water-soluble contrast or ultrasound-guided hydrostatic (saline) enema, the advantage of the latter being that no ionizing radiation is needed (Huppertz, Soriano-Gaabarro, Grimprel, et al, 2006). Recurrence of intussusception after conservative treatment is rare. Herwig, Brenkert, and Losek (2009) found hospitalized children needed minimal interventions after undergoing enema-reduced intussusception.

IV fluids, NG decompression, and antibiotic therapy may be used before hydrostatic reduction is attempted. If these procedures are not successful, the child may require surgical intervention. Surgery involves manually reducing the invagination and, when indicated, resecting any nonviable intestine.

Nursing Care Management

The nurse can help establish a diagnosis by listening to the parent’s description of the child’s physical and behavioral symptoms. It is not unusual for parents to state that they thought something was seriously wrong before others shared their concerns. The description of the child’s severe colicky abdominal pain combined with vomiting is a significant sign of intussusception.

As soon as a possible diagnosis of intussusception is made, the nurse prepares the parents for the immediate need for hospitalization, the nonsurgical technique of hydrostatic reduction, and the possibility of surgery. It is important to explain the basic defect of intussusception. The nurse can easily demonstrate this by creating a model of the defect. Use the example of a telescoping rod, or push the end of a finger on a rubber glove back into itself. Then demonstrate the principle of reduction by hydrostatic pressure by filling the glove with water, which pushes the “finger” into a fully extended position.

Physical care of the child does not differ from that for any child undergoing abdominal surgery. Even though nonsurgical intervention may be successful, the usual preoperative procedures, such as maintenance of NPO status, routine laboratory testing (CBC and urinalysis), signed parental consent, and preanesthetic sedation, are performed. For the child with signs of electrolyte imbalance, hemorrhage, or peritonitis, additional preparation, such as replacement fluids, whole blood or plasma, and NG suctioning, may be needed. Before surgery the nurse monitors all stools.

Postprocedural care includes observations of vital signs, blood pressure, intact sutures and dressing, and the return of bowel sounds. After spontaneous or hydrostatic reduction, the nurse observes for passage of water-soluble contrast material (if used) and the stool patterns, since the intussusception may recur. Children may be admitted to the hospital or monitored on an outpatient basis. A recurrence of intussusception is treated with the conservative reduction techniques described above, but a laparotomy is considered for multiple recurrences.

Because hospitalization may be the child’s first separation from the parents, it is important to preserve the parent-child relationship by encouraging rooming-in or extended visiting. It may be the parents’ first experience with hospitalization, necessitating their preparation for procedures such as IV therapy, frequent vital sign and blood pressure monitoring, dressings, and NPO. Because of the rapidity of the onset, diagnosis, and treatment, parents may feel stunned or numb. They may ask few questions, or they may constantly make inquiries, sometimes the same ones several times. Because of the circumstances surrounding this condition, be accepting and understanding of the parents’ reactions.

Diagnostic Evaluation

It is imperative that malrotation and volvulus be diagnosed promptly and surgical treatment instituted quickly. An upper GI series is the definitive procedure to diagnose this condition.

Therapeutic Management

Surgery is indicated to remove the affected area. Because of the extensive nature of some lesions, short-bowel syndrome (SBS) is a postoperative complication.

Nursing Care Management

Preoperatively the nursing care is the same as that provided to an infant or child with intestinal obstruction. Postoperatively the nursing care is similar to that provided to the infant or child who has undergone abdominal surgery.

Pathophysiology

Celiac disease is characterized by villous atrophy in the small bowel in response to the protein gluten (Maki and Lohi, 2004). Gluten is found in wheat, barley, rye, and oat grains. When individuals are unable to digest the gliadin component of gluten, an accumulation of a toxic substance that is damaging to the mucosal cells occurs. Damage to the mucosa of the small intestine leads to villous atrophy, hyperplasia of the crypts, and infiltration of the epithelial cells with lymphocytes. Villous atrophy leads to malabsorption due to the reduced absorptive surface area (see Fig. 33-1 and the discussion of absorption on p. 1296).

Clinical Manifestations

Symptoms of celiac disease appear when solid foods such as beans and pasta are introduced in the child’s diet between the ages of 1 and 5 years (Box 33-13). There is usually an interval of several months between the introduction of gluten into the diet and the onset of symptoms. Intestinal symptoms are common in children diagnosed within the first 2 years of life. Other symptoms include failure to thrive, chronic diarrhea, abdominal distention, muscle wasting, anorexia, and irritability. (See Critical Thinking Exercise on Inflammatory Bowel Disease.)

Diagnostic Evaluation

The diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of mucosal inflammation, crypt hyperplasia, and villous atrophy (Dieterich, Esslinger, and Schuppan, 2003).

Therapeutic Management

Treatment of celiac disease consists primarily of dietary management. Although a gluten-free diet is prescribed, it is difficult to remove every source of this protein. Some patients are able to tolerate restricted amounts of gluten. Because gluten occurs mainly in the grains of wheat and rye, but also in smaller quantities in barley and oats, these four foods are eliminated. Corn, rice, and millet are substitute grain foods.

Children with untreated celiac disease may have lactose intolerance, especially if their mucosal lesions are extensive. Lactose intolerance usually improves as the mucosa heals with gluten withdrawal. Specific nutritional deficiencies, such as iron, folic acid, and fat-soluble vitamin deficiencies, are treated with appropriate supplements.

Nursing Care Management

The main nursing consideration is helping the child adhere to the dietary regimen. Considerable time is involved in explaining the disease process to the child and parents, the specific role of gluten in aggravating the disorder, and those foods that must be restricted. It is difficult to maintain a diet indefinitely when the child has no symptoms and temporary transgressions result in no difficulties. However, the majority of individuals who relax their diet will experience a relapse of their disease and possibly exhibit growth retardation, anemia, or osteomalacia. There is also the risk of developing malignant lymphoma of the small intestine or other GI malignancies.

Although the chief source of gluten is cereal and baked goods, grains are frequently added to processed foods as thickeners or fillers. To compound the difficulty, gluten is added to many foods as hydrolyzed vegetable protein, which is derived from cereal grains. The nurse must advise parents of the necessity of reading all label ingredients carefully to avoid hidden sources of gluten.

Many of children’s favorite foods contain gluten, including bread, cake, cookies, crackers, donuts, pies, spaghetti, pizza, prepared soups, some processed ice cream, many types of chocolate candy, milk preparations such as malts, hot dogs, luncheon meats, meat gravy, and some prepared hamburgers. Many of these products can be eliminated from the infant’s or young child’s diet fairly easily, but monitoring the diet of the school-age child or adolescent is more difficult. Luncheon preparation away from home is particularly difficult, since bread, luncheon meats, and instant soups are not allowed. For families on restricted food budgets, the diet adds an additional financial burden because many inexpensive or convenient foods cannot be used.

In addition to restricting gluten, other dietary alterations may be necessary. For example, in some children who have more severe mucosal damage, the digestion of disaccharides is impaired, especially in relation to lactose. Therefore these children often need a temporarily lactose-free diet, which necessitates eliminating all milk products. In general, dietary management includes a diet high in calories and proteins, with simple carbohydrates such as fruits and vegetables, but low in fats. Because the bowel is inflamed as a result of the pathologic processes in absorption, the child must avoid high-fiber foods, such as nuts, raisins, raw vegetables, and raw fruits with skin, until inflammation has subsided.

It is important to stress long-range complications and to remind parents of the child’s physical status before dietary treatment and the dramatic improvement after treatment. The nurse can be instrumental in allowing the child to express concerns and frustration while focusing on ways in which the child can still feel normal. Encourage the child and parents to find new recipes using suitable ingredients, such as Mexican or Chinese dishes that use corn or rice. Consult a nutritionist to provide children and their families with detailed dietary instructions and education.*

Several resources are available to assist children and parents in all aspects of coping with celiac disease. The Celiac Sprue Association/United States of America† provides support and guidance to families and supplies educational materials concerning a gluten-free diet, food sources, recipes, and travel information.

Therapeutic Management

The goals of therapy for infants and children with SBS include (1) preserve as much length of bowel as possible during surgery; (2) maintain optimum nutritional status, growth, and development while intestinal adaptation occurs; (3) stimulate intestinal adaptation with enteral feeding; and (4) minimize complications related to the disease process and therapy (Goday, 2009).

Nursing Care Management

The most important components of nursing care are administration and monitoring of nutritional therapy. During PN therapy, take care to minimize the risk of complications related to the central venous access device (i.e., catheter infections, occlusions, dislodgment, or accidental removal). Care of the enteral feeding tubes and monitoring of enteral feeding tolerance are also important nursing responsibilities (see Critical Thinking Exercise).

When hospitalization is prolonged, the child’s developmental and emotional needs must be met. This often requires special planning to promote normal family adjustment and adaptation of the hospital routines. Chapter 26 discusses care of the hospitalized child.

Many infants with SBS have an intestinal ostomy performed at the time of the initial bowel resection. Routine ostomy care is another important nursing responsibility. Since infants and children with SBS have chronic diarrhea, perineal skin irritation is often a problem after ostomy closure. Frequent diaper changes, gentle perineal cleansing, and protective skin ointments help prevent skin breakdown. (See Diaper Dermatitis, Chapter 13.)

When hospitalization is prolonged, the child’s developmental and emotional needs must be part of the care plan. This often requires special efforts to promote normal family adaptation to hospital routines. It may be months to years before the child no longer requires specialized nutritional support. Family members require psychosocial support and education to cope successfully with SBS.

Etiology

The esophagus is a common site of upper GI bleeding. Esophagitis caused by GER may lead to chronic and often occult blood loss. Esophageal varices secondary to portal hypertension may cause massive bleeding. Peptic inflammation (gastritis and duodenitis) or ulceration is the most common cause of upper GI bleeding in children. Hemorrhagic gastritis may occur in the newborn infant after a difficult delivery or asphyxia. In this circumstance gastric perforation is a serious complication that requires emergent treatment. Less common causes of upper GI bleeding include bleeding disorders, vascular malformations, GI duplications, Mallory-Weiss syndrome (an esophageal tear caused by protracted vomiting), and hematobilia (bleeding into biliary passages).

In lower GI bleeding, small amounts of bright red blood in the stool of a healthy child may be due to an anal fissure. Colonic polyps are another cause of passage of bright red blood per rectum in toddlers and older children. Bleeding associated with diarrhea may indicate a serious problem. Enteric infections remain the leading cause, but the nurse should consider necrotizing enterocolitis, hemolytic uremic syndrome, IBD, and food allergy. Other causes are intussusception with the passage of blood per rectum (see p. 1323) or Meckel diverticulum with the painless passage of currant jelly–like stools (see p. 1312).

Pathophysiology

The GI tract has an extensive surface area and a rich vascular supply. Bleeding can occur anywhere along the GI tract from a vein, artery, or vascular malformation. In an otherwise healthy newborn infant, hemophilia or inherited coagulation-factor deficits are rarely accompanied by bleeding unless other conditions are superimposed (Gilger, 2004). Children with liver disease may also have deficient coagulation factors because of poor synthesis and malabsorption of vitamin K, which is a risk factor for GI bleeding.

Portal hypertension may lead to GI bleeding because the formation of portosystemic shunts can result in dilated venous channels in vulnerable locations such as the esophagus and stomach. These dilated venous channels (varices) may bleed, causing severe GI hemorrhage.

Diagnostic Evaluation

The diagnosis of GI bleeding is often made on the basis of the history and physical examination. Hematemesis is the vomiting of bright red blood or denatured blood that looks like coffee grounds, usually representing an upper GI source of bleeding. Hematochezia is the passage of bright red blood per rectum, indicating lower GI bleeding. This blood may precede or follow a bowel movement or be mixed with or coat the stool. Bright red blood that coats the stool may be due to a hard bowel movement, hemorrhoids, or anal fissures. Blood mixed with stool indicates a bleeding source proximal to the rectum. Blood passed alone after a bowel movement is most likely due to bleeding in the perianal or rectal area, possibly caused by a polyp. Blood with mucus in the stool indicates an inflammatory or infectious condition, and currant jelly–like stools indicate vascular compromise, such as intussusception. Melena is the passage of black, tarry stools that contain denatured (digested) blood and suggests an upper GI source of bleeding. Occasionally, bright red blood may be passed per rectum from an upper GI source of bleeding when the bleeding is massive. It is important to test emesis or stool for occult blood to differentiate true bleeding from the ingestion of food containing food coloring. In older children, false-positive stool tests for occult blood can also occur with the ingestion of red meats and iron preparations.

Critical Thinking Exercise—Hematemesis

Laboratory studies are determined on the basis of the history and physical examination. In many instances, a CBC with platelet quantification, prothrombin, partial thromboplastin, and coagulation studies will be done. Children who have acute illness, fever, and joint pain in addition to GI bleeding need an ESR and stool studies with culture to evaluate for enteric pathogens, ova and parasites, and Clostridium difficile. When IBD is suspected, a metabolic panel to determine total protein and albumin may be added to a CBC, ESR, and LFTs. The child with massive painless rectal bleeding may require a nuclear medicine scan to rule out Meckel diverticulum. If there is evidence of portal hypertension or chronic liver disease, LFTs, liver imaging studies, and a liver biopsy may be necessary. A barium enema is performed if intussusception is suspected.

Imaging studies help differentiate among several suspected diagnoses. CT of the sinuses helps localize bleeding that is coming from the nasopharynx or sinuses. A chest radiograph may distinguish hemoptysis related to cystic fibrosis, bronchiectasis, or other chronic lung conditions from hematemesis. Angiography can be used to identify the source of bleeding and to allow embolization or vasopressin infusion for treatment. Endoscopy is the diagnostic method chosen when the source of bleeding is thought to be secondary to gastritis, esophagitis, PUD, colitis, or polyps. Endoscopic examinations also permit visualization of the intestinal mucosa and collection of biopsy specimens and cultures.

Therapeutic Management

Treatment of GI bleeding in children depends on its severity and cause. The first step in management of acute GI bleeding is to assess the magnitude of blood loss and restore the child’s hemodynamic stability. Severe bleeding necessitates hospitalization. IV fluids (normal saline or lactated Ringer solution) are administered rapidly. Oxygen therapy is indicated if the bleeding is severe. Transfusion of blood products may be required if the blood loss is significant, and any existing coagulopathy should be corrected.

Upper GI mucosal lesions are usually treated with H₂ receptor antagonists (cimetidine, ranitidine, or famotidine) or PPIs (omeprazole or lansoprazole) and antacids to reduce acidity and promote mucosal healing. Variceal hemorrhage can be treated with peripheral vasopressin infusion and endoscopic sclerotherapy to hasten tissue fibrosis. Balloon tamponade to place pressure on the bleeding area may be performed as a temporary measure until endoscopic sclerotherapy can be done.

Therapy for lower GI bleeding is directed toward the primary underlying condition. The treatment may include medical or surgical management. Surgery may be required if the bleeding is severe despite aggressive medical intervention.

Nursing Care Management

The infant or child with acute and severe GI bleeding requires emergency care. Initial management includes assessment of the magnitude of bleeding and hemodynamic status and assistance with resuscitation efforts (see Critical Thinking Exercise).

Administer oxygen, and make certain suction equipment is available. An IV catheter should be inserted and preparation made for the administration of IV fluids, usually normal saline or lactated Ringer solution. Draw blood for laboratory analysis, including hemoglobin, hematocrit, blood urea nitrogen, creatinine, coagulation studies, and type and crossmatch. The nurse should be prepared to insert an NG tube to help locate the site of bleeding and to lavage the stomach with normal saline at room temperature if upper GI bleeding is suspected. Avoid taking rectal temperatures to prevent further irritation or damage to the rectal mucosa of a child suspected of having rectal bleeding or fissures. After the child is stabilized, ongoing monitoring in an intensive care setting may be indicated.

In cases of mild or chronic bleeding, there is more time for a thorough history and diagnostic evaluation, often in an outpatient setting. Important nursing responsibilities include assisting with the history and physical examination, diagnostic procedures, and education regarding the therapeutic plan.

The parents or caregivers of a child with GI bleeding may be extremely anxious and panic stricken. They need reassurance that most instances of bleeding are self-limiting and can be treated successfully. In life-threatening situations, special emotional support is required. Keep the family informed about the source, cause, and treatment of the bleeding.

Etiology

The majority (90%) of cases of viral hepatitis are caused by six viruses:

In earlier resources the HCV, HDV, HEV, and HGV infections were characterized as non-A, non-B; most of these non-A, non-B infections were caused by HCV (Castiglia, 1996, 2001). In addition, CMV, EBV, and herpes simplex virus may occasionally cause hepatitis. The clinical symptoms of these viruses are similar. Epidemiologic features and serologic testing are used to differentiate the causes. Table 33-3 compares the features of HAV, HBV, and HCV.

Hepatitis A incidence in the United States has declined 92%, from 12.0 cases per 100,000 population in 1995 to 1.0 case per 100,000 population in 2007, the lowest rate ever recorded. Declines were greatest among children and in those states where routine vaccination of children was recommended beginning in 1999 (Daniels, Grytdal, Wasley, et al, 2009). The virus is spread directly or indirectly by the fecal-oral route by either ingestion of contaminated foods, direct exposure to infected fecal material, or close contact with an infected person. The virus is particularly prevalent in developing countries with poor living conditions, inadequate sanitation, crowding, and poor personal hygiene practices. The spread of HAV has been associated with improper food handling and high-risk areas such as households with infected persons, residential centers for the disabled, and daycare centers. The average incubation period is about 4 weeks, with a range of 15 to 50 days. Fecal shedding of the virus can occur for 2 to 3 weeks before and for a week after the onset of jaundice. During this time, although the individual is asymptomatic, the virus is most likely to be transmitted. Infants with HAV infection are likely to be asymptomatic (anicteric hepatitis). Children often have diarrhea, and their symptoms are frequently attributed to gastroenteritis. Only 1 in 12 young children develops jaundice. Most adults develop clinical signs with icteric hepatitis. The prognosis of HAV infection is usually good, and complications are rare.

Hepatitis B can be an acute or chronic infection, ranging from an asymptomatic, limited infection to fatal, fulminant (rapid and severe) hepatitis (Yuen and Lai, 2001). There are no environmental or animal reservoirs for HBV. Humans are the main source of infections. HBV may be transmitted parenterally, percutaneously, or transmucosally. Hepatitis B surface antigen (HBsAg) has been found in all body fluids, including feces, bile, breast milk, sweat, tears, vaginal secretions, and urine, but only blood, semen, and saliva have been found to contain infectious HBV particles. HBV infection from human bites has been documented, but transmission from feces has not. Hepatitis B has been acquired after blood transfusion, but the likelihood of this has been reduced through blood product–screening procedures. Adults whose occupations are associated with considerable exposure to blood or blood products, such as health care workers, are at an increased risk of contracting HBV.

Most HBV infection in children is acquired perinatally. Transmission from mother to infant during the perinatal period (i.e., blood exposure during delivery) results in chronic infection in 70% to 90% of infants if the mother is positive for HBsAg and HBeAg (Tran, 2009; American Academy of Pediatrics, 2009). Perinatal infection occurs during the birthing process when the infant comes in contact with maternal body fluids, most likely blood. It is still not known if the virus enters the infant via mucosal membranes, intestinal tract, or skin abrasions. HBsAg has been detected in breast milk, but it is not clear whether HBV infection is transmitted through ingested breast milk or from swallowed maternal blood from injured nipples (Tran, 2009). Infants and children who are not infected during the perinatal period remain at high risk for acquiring person-to-person transmission from their mother during the first 5 years of life.

HBV infection occurs in children and adolescents in specific high-risk groups: (1) individuals with hemophilia or other disorders who have received multiple transfusions, (2) children and adolescents involved in IV drug abuse, (3) institutionalized children, (4) preschool children in endemic areas, and (5) individuals engaged in heterosexual activity or sexual activity with homosexual males. The incubation period for HBV infection ranges from 45 to 160 days with an average of 120 days (American Academy of Pediatrics, 2009). HBV infection can cause a carrier state and lead to chronic hepatitis with eventual cirrhosis or hepatocellular carcinoma in adulthood.

Hepatitis C (HCV) is transmitted parenterally through exposure to blood and blood products from HCV-infected persons (Bonkovsky and Mehata, 2001). Recent improvements in donor screening and inactivation procedures for blood products such as the factor concentrates used for hemophilia patients have significantly reduced the risk of transmission through blood products. The mechanism of nonparenteral or nonpercutaneous transmission of HCV is uncertain. Sexual transmission among monogamous couples and among family contacts is uncommon. Maternal coinfection with HIV has been associated with increased risk of perinatal transmission of HCV and may depend on the HCV genotype and the serum titer of maternal HCV-RNA. All persons with HCV antibody or HCV-RNA in their blood are considered to be infectious (American Academy of Pediatrics, 2009).

The clinical course is variable. The incubation period for HCV ranges from 14 to 180 days, with an average of 45 days. The natural history of the disease in children is not well defined. Some children may be asymptomatic, but hepatitis C can become a chronic condition and can cause cirrhosis and hepatocellular carcinoma. About 60% to 70% of individuals infected with HCV develop chronic disease. Infection with HCV is the leading reason for liver transplantation in the United States (America Academy of Pediatrics, 2009).

Hepatitis D occurs in children already infected with HBV. HDV is a defective RNA virus that requires the helper function of HBV. The incubation period is from 2 to 8 weeks. Both acute and chronic forms of hepatitis D tend to be more severe than hepatitis B and can lead to cirrhosis. HDV infection occurs mostly in drug abusers, individuals with hemophilia, and persons immigrating from endemic areas.

Hepatitis E is enterically transmitted non-A, non-B hepatitis. Transmission may occur through the fecal-oral route or from contaminated water. The incubation period is 2 to 9 weeks. This illness is uncommon in children, does not cause chronic liver disease, is not a chronic condition, and has no carrier state. However, it can be a devastating disease among pregnant women, with an unusually high case-fatality rate.

HGV is blood borne but can also be transmitted by organ transplantation. High-risk groups include transfusion recipients, IV drug users, and individuals infected with HCV. Individuals with the virus are often asymptomatic, and most infections are chronic. The incubation period is unknown.

Pathophysiology

Pathologic changes occur primarily in the parenchymal cells of the liver and result in variable degrees of swelling; infiltration of liver cells by mononuclear cells; and subsequent degeneration, necrosis, and fibrosis. Structural changes within the hepatocyte account for altered liver functions, such as impaired bile excretion, elevated transaminase levels, and decreased albumin synthesis. The disorder may be self-limiting, with regeneration of liver cells without scarring, leading to a complete recovery. However, some forms of hepatitis do not result in complete return of liver function. These include fulminant hepatitis, which is characterized by a severe, acute course with massive destruction of the liver tissue causing liver failure and high mortality within 1 to 2 weeks, and subacute or chronic active hepatitis, which is characterized by progressive liver destruction, uncertain regeneration, scarring, and potential cirrhosis.

The progression of liver disease is characterized pathologically by four stages: (1) stage one is characterized by mononuclear inflammatory cells surrounding small bile ducts, (2) in stage two there is proliferation of small bile ductules, (3) stage three is characterized by fibrosis or scarring, and (4) stage four is cirrhosis.

Clinical Manifestations

The clinical manifestations and course of uncomplicated acute viral hepatitis are similar for most of the hepatitis viruses. Usually the prodromal, or anicteric, phase (absence of jaundice) lasts 5 to 7 days. Anorexia, malaise, lethargy, and easy fatigability are the most common symptoms. Fever may be present, especially in adolescents. Nausea, vomiting, and epigastric or right upper quadrant abdominal pain or tenderness may occur. Arthralgia and skin rashes may occur and are more likely in children with hepatitis B than those with hepatitis A. The transaminases, rather than the bilirubin, will often be elevated in acute hepatitis, and hepatomegaly may be present. Some mild cases of acute viral hepatitis do not cause symptoms or can be mistaken for influenza.

In young children most of the prodromal symptoms disappear with the onset of jaundice, or the icteric phase. Many children with acute viral hepatitis, however, never develop jaundice. If jaundice occurs, it is often accompanied by dark urine and pale stools. Pruritus may accompany jaundice and can be bothersome for children.

Children with chronic active hepatitis may be asymptomatic but more commonly have nonspecific symptoms of malaise, fatigue, lethargy, weight loss, or vague abdominal pain. Hepatomegaly may be present, and the transaminases are often very high, with mild to severe hyperbilirubinemia.

Fulminant hepatitis is due primarily to HBV or HCV. Many children with fulminant hepatitis develop characteristic clinical symptoms and rapidly develop manifestations of liver failure, including encephalopathy, coagulation defects, ascites, deepening jaundice, and increasing white blood cell count. Changes in mental status or personality indicate impending liver failure. Although children with acute hepatitis may have hepatomegaly, a rapid decrease in the size of the liver (indicating loss of tissue due to necrosis) is a serious sign of fulminant hepatitis. Complications of fulminant hepatitis include GI bleeding, sepsis, renal failure, and disseminated coagulopathy.

Diagnostic Evaluation

Diagnosis is based on the history; physical examination; and serologic markers for hepatitis A, B, and C. No LFT is specific for hepatitis, but serum aspartate and serum aminotransferase levels are markedly elevated. Serum bilirubin levels peak 5 to 10 days after clinical jaundice appears. Histologic evidence from liver biopsy may be required to establish the diagnosis and to assess the severity of the liver disease. Serologic markers indicate the antibodies or antigens formed in response to the specific virus and confirm the diagnosis. Serum immunologic tests are not available to detect HAV antigen, but there are two HAV antibody tests: anti-HAV immunoglobulin G (IgG) and immunoglobulin M (IgM). Anti-HAV antibodies are present at the onset of the disease and persist for life. A positive anti-HAV antibody test indicates the following: acute infection, immunity from past infection, passive antibody acquisition (e.g., from transfusion, serum immunoglobulin infusion), or immunization. To diagnose an acute or recent HAV infection, a positive anti-HAV IgM test that is present with the onset of the disease and that persists for only 2 or 3 days is required.

Diagnosis of hepatitis B is confirmed by the detection of various hepatitis virus antigens and the antibodies that are produced in response to the infection. These antibodies and antigens and their significance include:

Tests are available for detection of all the HBV antigens and antibodies except HBcAg. HBsAg is detectable during acute infection. Presence of HBsAg indicates that the individual has been infected with the hepatitis virus. If the infection is self-limiting, HBsAg disappears in most patients before serum anti-HBs can be detected (termed the window phase of infection). IgM anti-HBc is highly specific in establishing the diagnosis of acute infection, as well as during the window phase in older children and adults. However, IgM anti-HBc usually is not present in perinatal HBV infection (American Academy of Pediatrics, 2009). Neonatal infection is most likely to occur in infants born to mothers who are HbeAg positive. In contrast, hepatitis B is much less likely to occur in infants whose mothers are HbsAg positive but HbeAg negative and who have antibodies to HBeAg (Chang, 2004).

Clinical improvement is usually associated with a decrease in or disappearance of these antigens, followed by the appearance of their antibodies. For example, anti-HBc of the IgM class often occurs early in the disease, followed by a rise in anti-HBc of the IgG class. Because the antibodies persist indefinitely, they are used to identify the carrier state (individuals with HBV who have no clinical disease but are able to transmit the organism). Persons with chronic HBV infection have circulating HBsAg and anti-HBc, and on rare occasions anti-HBsAg is present. Both anti-HBs and anti-HBc are detected in persons with resolved infection, but anti-HBs alone is present in individuals who have been immunized with the HBV vaccine.

HCV-RNA is the earliest serologic marker for HCV. HCV-RNA can be detected during the incubation period before symptoms of HCV disease are expressed. A positive HCV-RNA indicates active infection, and persistence of HCV-RNA indicates chronic infection. A negative test correlates with resolution of the disease. HCV-RNA is also used to determine patient response to antiviral therapy for HCV.

The history of all patients should include questions to seek evidence of (1) contact with a person known to have hepatitis, especially a family member; (2) unsafe sanitation practices, such as contaminated drinking water; (3) ingestion of certain foods, such as clams or oysters (especially from polluted water); (4) multiple blood transfusions; (5) ingestion of hepatotoxic drugs, such as salicylates, sulfonamides, antineoplastic agents, acetaminophen, and anticonvulsants; and (6) parenteral administration of illicit drugs or sexual contact with a person who uses these drugs.

Therapeutic Management

Treatment options for viral hepatitis are limited. The goals of management include early detection, support and monitoring of the disease, recognition of chronic liver disease, and prevention of spread of the disease. No specific effective therapy for either acute or chronic hepatitis B or hepatitis C exists. Special high-protein, high-carbohydrate, low-fat diets are generally not of value. The use of corticosteroids alone or with immunosuppressive drugs is not advocated in the treatment of chronic viral hepatitis. However, steroids have been used to treat chronic autoimmune hepatitis. Hospitalization is required in the event of coagulopathy or fulminant hepatitis. Human interferon-α has been used in the treatment of chronic hepatitis B and C in adults and is being used to treat these infections in children. Therapy for hepatitis depends on the severity of inflammation and the cause of the disorder.

A number of antiviral medications are being used currently to treat HBV and HCV (Degertekin and Lok, 2009). Telbivudine is more potent than lamivudine but is associated with a high rate of antiviral resistance compared with entecavir or tenofovir. Combined therapy with lamivudine and adefovir reduces the rate of antiviral resistance compared with lamivudine monotherapy. Individualizing dose and duration of pegylated interferon and ribavirin according to on-treatment virologic response may improve sustained virologic response rates. Several specifically targeted antiviral therapies, notably protease and polymerase inhibitors, are promising but must be used in combination with pegylated interferon and ribavirin. These agents have multiple side effects, and patients require regular monitoring and support. Many products are under current investigation in clinical trials, largely with adult patients.

Nursing Care Management

Nursing objectives depend largely on the severity of the hepatitis, the medical treatment, and factors influencing the control and transmission of the disease. Because children with mild viral hepatitis are frequently cared for at home, it is often the nurse’s responsibility to explain any medical therapies and infection control measures. When further assistance is needed for parents to comply with instructions, a public health nursing referral is necessary.

Encourage a well-balanced diet and a schedule of rest and activity adjusted to the child’s condition. Because the child with HAV is not infectious within a week after the onset of jaundice, the child may feel well enough to resume school shortly thereafter. Caution parents about administering any medication to the child, since normal doses of many drugs may become dangerous because of the liver’s inability to detoxify and excrete them.

Standard Precautions are followed when children are hospitalized. However, these children are not usually isolated in a separate room unless they are fecally incontinent or their toys and other personal items are likely to become contaminated with feces. Discourage children from sharing their toys. (See Infection Control, Chapter 27.)

Hand washing is the single most effective measure in prevention and control of hepatitis in any setting. (For a discussion of preventive measures in the daycare center, see Chapter 15; see also Infection Control, Chapter 27.) Parents and children need an explanation of the usual ways in which HAV (fecal-oral route) and HBV (parenteral route) are spread. Parents should also be aware of the recommendation for universal vaccination against HBV for newborns and adolescents. (See Chapter 12.)

In young people with HBV infection who have a known or suspected history of illicit drug use, the nurse has the responsibility of helping them realize the associated dangers of drug abuse, stressing the parenteral mode of transmission of hepatitis, and encouraging them to seek counseling through a drug program.

Pathophysiology

Cirrhosis occurs as a result of hepatocyte injury with necrosis, fibrosis, regeneration, and eventual degeneration. The diminished parenchymal cell mass causes regeneration of tissue with nodular areas of proliferating hepatocytes that stretch the surrounding connective tissue. Hepatocytes respond to injury with deposition of collagen that forms fibrous connective tissue. This scar tissue and nodular areas of regeneration impair the intrahepatic blood flow. Ongoing necrosis and self-perpetuation of this pathologic process are the result of cirrhosis.

Failure of hepatocellular function and portal hypertension occur and often lead to complications, including ascites, severe cholestasis, encephalopathy (hepatic coma), and GI bleeding.

Clinical Manifestations

Clinical manifestations of cirrhosis include jaundice, poor growth, anorexia, muscle weakness, and lethargy. Ascites, edema, GI bleeding, anemia, and abdominal pain may be present in children with impaired intrahepatic blood flow. Pulmonary function may be impaired because of pressure against the diaphragm due to hepatosplenomegaly and ascites. Dyspnea and cyanosis may occur, especially on exertion. Intrapulmonary arteriovenous shunts may develop, which can also cause hypoxemia. Spider angiomas and prominent blood vessels on the upper torso are often present.

Diagnostic Evaluation

The diagnosis of cirrhosis is based on (1) the history, especially in regard to prior liver disease, such as hepatitis; (2) physical examination, particularly hepatosplenomegaly or a sudden decrease in liver size; (3) laboratory evaluation, especially LFTs, such as bilirubin and aminotransferases, ammonia, albumin, cholesterol, and prothrombin time; and (4) liver biopsy for characteristic changes. Doppler ultrasonography of the liver and spleen is useful to confirm ascites, to evaluate the blood flow through the liver and spleen, and to determine the patency and size of the portal vein if liver transplantation is considered.

Therapeutic Management

Unfortunately, there is no successful treatment to arrest the progression of cirrhosis. The goals of management include monitoring liver function and managing specific complications such as esophageal varices and malnutrition. Assessment of the child’s degree of liver dysfunction is important so that the child can be evaluated for transplantation at the appropriate time.

Liver transplantation has improved the prognosis substantially for many children with cirrhosis. The combination of new immunosuppressive medications and new surgical techniques has resulted in 90% 1-year survival rates in many large hospital centers. The policy governing the allocation of livers for transplantation by the United Network for Organ Sharing allows patients with acute fulminant liver failure, plus those with failed liver grafts and the sickest pediatric patients, to be placed at the top of the network’s transplantation lists (Ott, 1997). Although this change has benefited many pediatric patients, the shortage of available donors for children continues to dictate transplantation decisions, and many children continue to die while waiting for a suitable donor. (See Biliary Atresia, Chapter 11.)

Nutritional support is an important therapy for children with cirrhosis and malnutrition. Supplements of fat-soluble vitamins are often required, and mineral supplements may be indicated. In some instances aggressive nutritional support in the form of continuous tube feedings or PN may be necessary.

Esophageal and gastric varices are a life-threatening complication of portal hypertension. Acute hemorrhage is managed with IV fluids, blood products, vasopressin, and gastric lavage. Balloon tamponade with a Sengstaken-Blakemore tube may be indicated. Endoscopic sclerotherapy and endoscopic banding ligation are also effective therapies for esophageal and gastric varices.

Ascites can be managed by sodium restriction and diuretics. Severe ascites with respiratory compromise can be managed with administration of albumin or by paracentesis.

Although the full mechanism of hepatic encephalopathy is unknown, failure of the damaged liver to remove endogenous toxins, such as ammonia, plays a role. Treatment is directed at limiting the ammonia formation and absorption that occur in the bowel, especially with the drugs neomycin and lactulose. Because ammonia is formed in the bowel by the action of bacteria on ingested protein, neomycin reduces the number of intestinal bacteria so less ammonia is produced. The fermentation of lactulose by colonic bacteria produces short-chain fatty acids, which lower the colonic pH, thereby inhibiting bacterial metabolism. This decreases the formation of ammonia from bacterial metabolism of protein.

Nursing Care Management

Several factors influence nursing care of the child with cirrhosis, including the cause of the cirrhosis, the severity of complications, and the prognosis. The prognosis is often poor unless successful liver transplantation occurs. Therefore nursing care of this child is similar to that for any child with a life-threatening illness. (See Chapter 23.) Hospitalization is required when complications such as hemorrhage, severe malnutrition, or hepatic failure occur. Nursing assessments are directed at monitoring the child’s condition, and interventions are aimed at treatment of specific complications. If liver transplantation is an option, the family needs support and assistance to cope (see Family-Centered Care box).

1. Yes, there is sufficient evidence to arrive at some conclusions for an initial plan of management.

2. a. Constipation in infancy can be caused by medical conditions such as Hirschsprung disease, hypothyroidism, or strictures, or it can be simple functional constipation.

3. Initially, the nurse practitioner can tell Harry’s mother that functional constipation may occur with changes in the diet (e.g., the change from breast-feeding 6 weeks ago to bottle-feeding of cow’s milk–based formula). The nurse practitioner can recommend that Harry’s mother slowly introduce cereal and prune juice into Harry’s diet. Cereal and one or two offerings of fruit juice each day may help to prevent further constipation. Often, simple measures such as the introduction of solid foods or other dietary modifications help to remedy functional constipation.

4. The initial data seem to point to the conclusion that Harry has functional constipation. However, the one episode of diarrhea and the two episodes of passage of ribbonlike stools do not usually occur with functional constipation.

1. Yes, there are sufficient data to arrive at some conclusions about what to include in Susan’s discharge planning.

2. a. The goals of nutritional support for a patient with CD include (1) correction of nutrient deficits and replacement of ongoing losses, (2) provision of adequate energy and protein for healing, and (3) provision of adequate nutrients to support normal growth.

3. The most immediate priority for discharge is to teach Susan and her family how to insert the NG tube, how to administer the feedings, how to obtain the supplies needed for the tube feedings at home, and how to observe for any untoward effects of the NG feedings. As Susan’s discharge nurse, you should have Susan and another family member insert the NG tube, demonstrate how to check the placement of the NG tube, and show how to start and stop the feedings while Susan is in the hospital. You also need to arrange for the appropriate vendors to deliver the feeding tube supplies and feeding pump to Susan’s home before discharge. While doing all this teaching, you should also be alert to any questions or anxieties that Susan or her family members may express.

4. Yes. Susan is to receive nighttime NG tube infusions at home, and her family has expressed a desire to perform this procedure at home. Therefore this discharge teaching is needed and required.

1. Yes. The best response is answer 2. Dehydration and electrolyte disturbances are common with diarrhea. Important initial nursing interventions include assessment of the child’s hydration status, including vital signs, weight, urine specific gravity, and intake and output.

2. a. SBS is a malabsorptive disorder that occurs as a result of decreased mucosal surface area, usually because of extensive resection of the small intestine.

3. Prevention of dehydration and electrolyte disturbances are priorities for this child. Temperature management also is important.

4. Children with chronic malabsorption may have severe diarrhea and are particularly susceptible to dehydration and electrolyte imbalance with an acute episode of illness such as infectious gastroenteritis.

1. Yes. The best response is 4. Because this infant has acute severe gastrointestinal (GI) bleeding, immediate nursing actions include an assessment of hemodynamic status for possible shock (option 2). The infant should not be left unattended, and the nurse should immediately call for assistance because further vomiting and potential aspiration may occur.

2. a. Hematemesis in an infant can indicate GI bleeding and can quickly lead to shock. Immediate assessment of the child’s hemodynamic state should be completed.

3. Treatment of GI bleeding in children depends on its severity and cause. The first step in management of acute GI bleeding is to restore the child’s hemodynamic stability and assess the magnitude of blood loss.

4. Severe bleeding necessitates immediate attention. Intravenous fluids (normal saline or lactated Ringer solution) are administered rapidly. Oxygen therapy is indicated if the bleeding is severe. Transfusion of blood products may be required if the blood loss is significant, and any existing coagulopathy should be corrected. The nurse should also anticipate that an NG tube will be inserted to lavage the stomach and monitor for further bleeding. A correct conclusion is that blood will need to be drawn for laboratory studies, including hemoglobin, hematocrit, platelet count, white blood cell count, and a type and crossmatch for potential transfusion.