Nursing Care: Mother & Child at Risk - PDF

BACHELOR OF SCIENCE IN NURSING: CARE OF MOTHER AND CHILD AT RISK OR WITH PROBLEMS (ACUTE AND CHRONIC) COURSE MODULE COURSE UNIT WEEK 3 12 14...

BACHELOR OF SCIENCE IN NURSING: CARE OF MOTHER AND CHILD AT RISK OR WITH PROBLEMS (ACUTE AND CHRONIC) COURSE MODULE COURSE UNIT WEEK 3 12 14 Alterations in Nutrition and Gastrointestinal, Metabolism & Endocrine Read course and unit objectives Read study guide prior to class attendance Read required learning resources; refer to unit terminologies for jargons Proactively participate in classroom discussions Participate in weekly discussion board (Canvas) Answer and submit course unit tasks At the end of this unit, the students are expected to: Cognitive: 1. Identify the different signs and symptoms associated with GIT problems 2. Discuss the different GIT problem seen in pediatric clients 3. Describe the characteristics of infants that affect their ability to adapt to fluid loss or gain  4. Discuss the pathophysiologic processes associated with specific gastrointestinal disorders 5. Identify the nursing interventions necessary to provide education needed to care for a child with gastrointestinal disorders. 6. Describe the common diagnostic tests used in the diagnosis and treatment of gastrointestinal disorders. 7. Identify and describe the different Metabolic and Endocrine Disorders. 8. Summarize signs and symptoms that may indicate a disorder of the endocrine system. 9. Differentiate among the various categories of diabetes mellitus. 10. Discuss the management and nursing care of the child with diabetes mellitus in the acute care setting Affective: Listen attentively during class discussions Demonstrate tact and respect when challenging other people’s opinions and ideas Accept comments and reactions of classmates on one’s opinions openly and graciously. Develop heightened interest in studying Nursing Informatics Psychomotor: 1. Participate actively during class discussions and group activities 2. Express opinion and thoughts in front of the class Hockenberry, M. and Wilson, D. (2015). Wong’s Nursing Care of Infants and Children 1st Philippine Edition, The Child with Gastrointestinal Dysfunction (Volume 2, pp. 1051-1121). Mosby. PROBLEMS IN NUTRITION AND GASTROINTESTINAL The gastrointestinal (GI) system involves a long body tract and numerous organs. Because it is so long and diverse, a multitude of possible disorders can occur along it, including both congenital disorders and acquired illnesses. Because the GI system is responsible for taking in and processing nutrients for all parts of the body, any problem with the system can quickly affect other body systems and, if not adequately treated, can affect overall health, growth, and development. CLEFT LIP/ PALATE Cleft lip and cleft palate are two distinct facial defects that can occur singly or in combination. Cleft lip with or without cleft palate occurs in 1 out of every 750 to 1000 live births. The incidence is higher in Asian (1 in 500) than in White children (1 in 750). The defect is less common in African Americans, with an incidence of 1 of every 2000 live births. Cleft lip and palate occur together in approximately 45% of cases, while cleft palate occurs alone approximately 35% of the time, and cleft lip occurs alone approximately 20% of the time. Cleft lip with or without cleft palate results when the maxillary processes fail to fuse with the elevations on the frontal prominence during the sixth week of gestation. Normally, union of the upper lip is complete by the seventh week. Fusion of the secondary palate occurs between 5 and 12 weeks of gestation. Failure of the tongue to move downward at the correct time prevents the palatine processes from fusing. The intrauterine development of the hard and soft palates is completed in the first trimester. It is during this time that other major organ systems develop. Approximately 30% of children with cleft and/or palate will have another congenital anomaly. There is an increased incidence in families with a prior history of cleft lip or palate. The cause is believed to be multifactorial, involving a combination of environmental and genetic influences. Etiologic factors include smoking during pregnancy, maternal use of alcohol, and use of medications such as anticonvulsants and steroids during pregnancy. A cleft that involves the lip is readily apparent at birth. It may be a simple dimple in the vermilion border of the lip or a complete separation extending to the floor of the nose. The defect may be unilateral or bilateral and may occur alone or in combination with a cleft palate defect. Varying degrees of nasal deformity may also be present. Cleft palate defects are less obvious when they occur without a cleft lip and may not be detected at birth. Clefts of the hard palate form a continuous opening between the mouth and nasal cavity and may be unilateral or bilateral, involving just the soft palate or both the soft and hard palates. Cleft lip and palate are usually diagnosed at birth or during the newborn assessment, but may be diagnosed in utero. Successful imaging of the face via transabdominal ultrasound can be performed as early as 13 to 14 weeks’ gestation. Use of three-dimensional ultrasound or magnetic resonance imaging, if available, allows for a clearer picture of the defect and enhances the ability to detect isolated cleft palate prenatally. After the child is born, cleft lip and cleft palate are diagnosed by characteristic physical findings. The upper lip, alveolar arches, nostrils, and primary and secondary palates should be inspected and palpated. Management is directed toward closure of the cleft(s), prevention of complications, and facilitation of normal growth and development in the child. Cleft lip repair typically occurs at most centers between 2 and 3 months of age. Most physicians adhere to the “rule of tens”: the infant must be 10 weeks old, weigh 10 pounds, and have a hemoglobin of 10. The two most common procedures for repair of CL are the Tennison-Randall triangular flap (Z-plasty) and the Millard rotational advancement technique. The difference between these two is that the Tennison-Randall procedure crosses the philtral line and the Millard procedure advances a triangle of tissue in the upper third of the lip and does not cross the midline. Surgeons often use a combination of these two techniques to address individual differences. Cleft palate repair typically occurs between 6 and 12 months. There is concern that early CP repair interferes with skeletal growth of the midface, but postponing palate closure beyond the child’s first words may result in increased speech disorders. The most common techniques to repair CP include the Veau-Wardill-Kilner V-Y pushback procedure and the Furlow double-opposing Z-plasty. Approximately 20% to 30% of children with repaired CP will need a secondary surgery to improve velopharyngeal closure for speech. Secondary procedures may include palatal lengthening, pharyngeal flap, sphincter pharyngoplasty, or posterior pharyngeal wall augmentation.  Children with CL may require multiple surgeries to achieve optimal aesthetic outcomes but are not at risk for increased speech problems. Although some children with CP and CL/P do not require speech therapy, many have some degree of speech impairment that requires speech therapy at some point throughout childhood. Articulation errors result from a history of velopharyngeal dysfunction, incorrect articulatory placement, improper tooth alignment, and varying degrees of hearing loss. Improper drainage of the middle ear as a result of inefficient function of the Eustachian tube relating to the history of CP contributes to recurrent otitis media, which leads to conductive hearing loss in many children with CP; many children with clefts will have pressure-equalization tubes placed. Extensive orthodontics and prosthodontics may be needed to correct malposition of the teeth and maxillary arches. Academic achievement, social adjustment, and behavior should be monitored, particularly in children with syndromic cleft conditions. Feeding the infant with a cleft presents a challenge to nurses and parents. Growth failure in infants with CL/P or CP has been attributed to preoperative feeding difficulties. After surgical repair, most infants who have isolated CL, CP, or CL/P with no associated syndromes gain weight or achieve adequate weight and height for age. Post-operative care for infants include: Assess vital signs frequently and maintain the infant’s airway. Measure intake and output. Prevent aspiration through proper positioning during and after feeding. Prevent the infant from rubbing the suture line on the bedding by positioning the infant in a supine position. Maintain soft elbow immobilizers. Maintain the suture line or Steri-Strips placed over the incision. Place antibiotic body ointment on the incision site as ordered. Medicate the infant as prescribed to control pain and to minimize crying and stress on the suture line. After cleft palate surgery, avoid the use of metal utensils or straws, which may disrupt the surgical site. Throughout the child’s development, an important goal is the development of a healthy personality and self-esteem. Many communities have CP parents’ groups that offer help and support to families. Agencies that provide services and information for children with CL/P and their families. HIRSCHSPRUNG DISEASE Hirschsprung disease, also known as congenital aganglionic megacolon, is a congenital anomaly in which inadequate motility causes mechanical obstruction of the intestine. The disease occurs in approximately 1 in 5000 live births, and is more common in males than females. Hirschsprung disease can occur as a single anomaly or in combination with other anomalies such as congenital heart defects, Down syndrome, and urinary tract anomalies. Hirschsprung disease is the congenital absence of ganglion cells (nerve cells) in the wall of a variable segment of rectum and colon. The absence of autonomic parasympathetic ganglion cells in the colon prevents peristalsis at that portion of the intestine, resulting in the accumulation of intestinal contents and abdominal distention. In most cases, the area lacking ganglion cells is limited to the rectosigmoid region of the colon. Clinical manifestations of Hirschsprung disease vary depending on the child’s age at onset. Symptoms in newborns generally include abdominal distention, feeding intolerance, bilious vomiting, and failure to pass meconium within the first 24 to 48 hours after birth. Enterocolitis (inflammation of the intestines) is a complication of Hirschsprung disease that can be fatal if not recognized and treated early. Symptoms of enterocolitis include fever, foul smelling and/or bloody diarrhea (frequent, watery stools), abdominal pain, and vomiting. The older infant or child may have a history of failure to gain weight, malnutrition, and chronic severe constipation (difficult and infrequent defecation with passage of hard, dry stool). Diagnosis is made on the basis of the history, bowel patterns, radiographic contrast studies, and rectal biopsy for presence or absence of ganglion cells. The rectum is small in size on palpation and does not contain stool. Abdominal radiographs generally show a distended bowel with dilated bowel loops throughout the abdomen. Water-soluble contrast studies reveal a transition zone between the normal and aganglionic bowel. The primary repair of Hirschsprung disease is to remove the aganglionic portion of the bowel using a pull-through procedure. A primary repair may not be possible in the presence of extensive dilated proximal bowel, enterocolitis, or bowel perforation. In that case, a temporary colostomy is created and is closed when the definitive surgery takes place. nger, 2013). The return of normal bowel function depends on the amount of bowel involved. Some fecal incontinence and constipation may persist following surgery. Enterocolitis is a serious complication that can occur before or after surgery, resulting in ischemia and ulceration of the bowel wall. Treatment for enterocolitis associated with Hirschsprung disease includes rectal irrigations and antibiotics. Nursing care includes monitoring for infection, managing pain, maintaining hydration, measuring abdominal circumference to detect any distention, and providing support to the child and family. INTUSSUSCEPTION Intussusception is the most common cause of intestinal obstruction in children between the ages of 3 months and 3 years. Intussusception is more common in boys than in girls and is more common in children with cystic fibrosis. Although specific intestinal lesions occur in a small percentage of the children, generally the cause is not known. More than 90% of intussusceptions do not have a pathologic lead point, such as a polyp, lymphoma, or Meckel diverticulum. The idiopathic cases may be caused by hypertrophy of intestinal lymphoid tissue secondary to viral infection. Intussusception occurs when one segment of the bowel telescopes into another 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. The most common site is the ileocecal valve (ileocolic), where the ileum invaginates into the cecum and then further into the colon. 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. Frequently, subjective findings lead to the diagnosis (abdominal pain, abdominal mass, bloody stools), which can be confirmed by ultrasonography. Spontaneous reduction occurs in up to 10% of patients. Conservative treatment consists of radiologist-guided pneumo-enema (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. Intravenous 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. Laparoscopic surgical repair is commonly performed. ACUTE APPENDICITIS Appendicitis is an inflammation of the vermiform appendix, the small sac near the end of the cecum, and is the most common cause of emergency surgery in children. The condition occurs most often in children and adolescents ages 10 to 19 years. While the overall rate of perforated appendix is 20% to 35%, the rate in children less than 3 years of age is 80% to 100% as compared to 10% to 20% in children 10 to 17 years of age (Minkes & Alder, 2014). Appendicitis almost always results from an obstruction in the appendiceal lumen. It can be caused by a fecalith (hard fecal mass), parasitic infestations, stenosis, hyperplasia of lymphoid tissue, or a tumor. Continued secretion of mucus following acute obstruction of the lumen increases pressure, causing ischemia, cellular death, and ulceration. The appendix may perforate or rupture, resulting in fecal and bacterial contamination of the peritoneum. Peritonitis spreads quickly and if untreated can result in small bowel obstruction, electrolyte imbalances, septicemia, and hypovolemic shock. At onset, symptoms include periumbilical cramps, abdominal tenderness, anorexia, nausea, and fever. As the inflammation progresses, pain in the right lower abdomen becomes constant. Pain is often most intense at the McBurney point, halfway between the anterior superior iliac crest and the umbilicus. Symptoms progress to include guarding, rigidity, nausea, vomiting, onset of pain before vomiting, anorexia, and rebound tenderness following palpation over the right lower quadrant. As appendicitis progresses, the child remains motionless, usually in a side-lying position with knees flexed. Sudden relief of pain usually means that the appendix has perforated. Diagnosis of appendicitis in young children can be difficult because their pain may be less localized and their symptoms more diffuse than in the older child. Continuing evaluations over several hours are often needed to establish the diagnosis. The presence of an elevated white blood cell count (above 10,000/mm3 ), increased neutrophil ratio, and an elevated C-reactive protein combined with the symptoms supports a diagnosis of appendicitis. A white blood cell count greater than 15,000/mm3 in a patient with appendicitis is a strong indicator that the appendix has perforated. Abdominal ultrasound is preferred as the initial screening tool in the diagnosis of appendicitis; however, CT scans are more sensitive and may be used, especially when the appendix cannot be seen well on ultrasound or the results are inconclusive. Treatment of uncomplicated appendicitis involves immediate surgical removal (appendectomy), generally through a laparoscopic appendectomy. Preoperatively, the child is kept NPO. Intravenous fluids and electrolytes, and antibiotics are administered. Postoperatively, the child has an abdominal incision with a dressing covering the incision. Antibiotics may be administered. The child is generally discharged within 24 to 36 hours as long as they have adequate oral intake, are afebrile, and receive effective pain relief with oral pain medication. With perforated appendix, laparoscopic appendectomy is generally performed. Postoperatively, the child with a perforated appendix may have a nasogastric tube to decompress the abdomen and will remain NPO until signs of bowel function return. Bowel function is best indicated by the passage of flatus or stool. The child will also have a peripheral or temporary central line for administration of intravenous fluids and medications. After surgery for a perforated appendix, the child will receive antibiotics for several days. Morphine is generally given for pain. ESOPHAGEAL ATRESIA (EA)/ TRACHEOESOPHAGEAL FISTULA (TEF) Congenital esophageal atresia and tracheoesophageal fistula are rare malformations that represent a failure of the esophagus to develop as a continuous passage and a failure of the trachea and esophagus to separate into distinct structures. These defects may occur as separate entities or in combination, and without early diagnosis and treatment, they pose a serious threat to the infant’s well-being. The defect affects occurs in approximately 1 in 4500 neonates. At least 90% of those affected also have a tracheoesophageal fistula. The cause of EA/ TEF is unknown. In esophageal atresia, the foregut fails to lengthen, separate, and fuse into two parallel tubes (the esophagus and trachea) during fetal development. Instead the esophagus may end in a blind pouch or develop as a pouch connected to the trachea by a fistula. Esophageal atresia is often associated with a maternal history of polyhydramnios. Symptoms in the newborn include excessive salivation and drooling, often accompanied by three classic signs for this defect: cyanosis, choking, and coughing. Sneezing may also be noted. During feeding, fluid returns through the infant’s nose and mouth. Aspiration places the infant at risk for pneumonia. Depending on the type of defect, the infant’s abdomen may be distended because of air trapping. Esophageal atresia is suspected based on the presence of polyhydramnios and a small or absent fetal gastric bubble noted on prenatal ultrasound. After the child is born, a radiopaque catheter is inserted into the hypopharynx and advanced until it encounters an obstruction. Chest radiographs are taken to ascertain esophageal patency or the presence and level of a blind pouch. Sometimes fistulas are not patent, which makes them more difficult to diagnose. The presence of gas in the stomach or small bowel is indicative of a coexisting TEF. Primary repair is preferred when possible and involves connecting the two ends of the esophagus and ligating the fistula if present. If primary repair of the esophageal atresia is not possible in the neonatal period, a gastrostomy tube is placed for feedings and the fistula is ligated. Esophageal atresia is a surgical emergency. Preoperatively the infant requires close observation and intervention to maintain a patent airway. Specific interventions include: Have suction readily available to remove any secretions that accumulate in the nasopharyngeal  airway. Place the infant with the head of the bed slightly elevated to minimize aspiration of secretions into the trachea. Use continuous or low intermittent suction to remove secretions from the blind pouch. Withhold oral fluids, and provide maintenance intravenous fluids. Constantly monitor the infant’s vital signs and overall condition. After surgery administer intravenous fluids and antibiotics. Monitor strict intake and output. Total parenteral nutrition may be needed until gastrostomy or oral feedings are tolerated. Monitoring and assessment of feeding tolerance are ongoing. Feedings are introduced slowly and in small amounts. PYLORIC STENOSIS Hypertrophic pyloric stenosis (HPS) occurs when the circumferential muscle of the pyloric sphincter becomes thickened, resulting in elongation and narrowing of the pyloric channel. This produces an outlet obstruction and compensatory dilation, hypertrophy, and hyperperistalsis of the stomach. This condition usually develops in the first 2 to 5 weeks of life, causing projectile nonbilious vomiting, dehydration, metabolic alkalosis, and growth failure. The precise etiology is unknown. The reported incidence is 1 to 3 per 1000 live births with a male-to-female ratio of 4 to 6 :1. There is a genetic predisposition, and siblings and offspring of affected persons are at increased risk of developing HPS. It is more common in full-term than in preterm infants and is seen less frequently in African-American and Asian infants than in white infants. The circular muscle of the pylorus thickens as a result of hypertrophy (increased size) and hyperplasia (increased mass). This produces severe narrowing of the pyloric canal between the stomach and the duodenum, causing partial obstruction of the lumen. Over time, inflammation and edema further reduce the size of the opening, resulting in complete obstruction. The hypertrophied pylorus may be palpable as an olivelike mass in the upper abdomen. Pyloric stenosis is not a congenital disorder. Substantial evidence supports decreased expression of neuronal nitric oxide synthase in the nerve fibers of the pyloric circular muscle in infants with HPS. Symptoms usually become evident 2 to 8 weeks after birth, although onset may vary (Taylor et al., 2013). Initially, the infant appears well or regurgitates slightly after feedings. The parents may describe the infant as a “good eater” who vomits occasionally. As the obstruction progresses, the vomiting becomes projectile. In projectile vomiting, the contents of the stomach may be ejected up to 3 feet from the infant. The vomitus is nonbilious and may become blood tinged because of repeated irritation to the esophagus. The infant generally appears hungry, especially after emesis; irritable; fails to gain weight; and has fewer and smaller stools. The child may present with dehydration and metabolic alkalosis depending on how long the child has been vomiting. On physical examination, peristaltic waves may be observed across the abdomen as the stomach attempts to move contents past the narrowed pyloric canal. An olive-sized mass in the right upper quadrant may be evident. An abdominal ultrasound to determine the diameter and length of the pyloric muscle is the preferred method performed to confirm the diagnosis. Blood tests will determine if the child is dehydrated or has an electrolyte or acid–base imbalance. Infants with pyloric stenosis are at risk for hypochloremia, hypokalemia, and metabolic alkalosis; however, recent studies show that normal laboratory values are found most often. This could be attributed to earlier diagnosis and the increased use of ultrasound to confirm the diagnosis. Surgery is performed as soon as possible after the infant’s fluid and electrolyte balance is restored. Laparoscopic pyloromyotomy is the preferred surgical method to correct pyloric stenosis. The prognosis is good. The infant is usually taking fluids within a few hours following surgery and discharged on full- strength formula within 24 hours after surgery. Nursing care management includes meeting the infant’s fluid and electrolyte needs, minimizing weight loss, promoting rest and comfort, preventing infection, and providing supportive care for parents. IMPERFORATED ANUS Imperforate anus includes several forms of malformation without an obvious opening. Frequently a fistula leads from the distal rectum to the perineum or genitourinary system. The fistula may be evidenced when mecomium is evacuated through the vaginal opening, the perineum below the vagina, the male urethra, or the perineum under the scrotum. The presence of meconium on the perineum does not indicate anal patency. A fistula may not be apparent at birth, but as peristalsis increases, meconium is forced through the fistula into the urethra or onto the newborn’s perineum. The anus and rectum originate from an embryologic structure called the cloaca. Lateral growth of the cloaca forms the urorectal septum that separates the rectum dorsally from the urinary tract ventrally. The rectum and urinary tract separate completely by the seventh week of gestation. Anomalies that occur reflect the stage of development of these processes. Rectal atresia and stenosis occur when the anal opening appears normal, there is a midline intergluteal groove, and usually no fistula exists between the rectum and urinary tract. Rectal atresia is a complete obstruction (inability to pass stool) and requires immediate surgical intervention. Rectal stenosis may not become apparent until later in infancy when the infant has a history of difficult stooling, abdominal distention, and ribbonlike stools. The diagnosis of an anorectal malformation is based on the physical finding of an absent anal opening. Other symptoms may include abdominal distention, vomiting, absence of meconium passage, or presence of meconium in the urine. Additional physical findings with an anorectal malformation are a flat perineum and the absence of a midline intergluteal groove. The appearance of the perineum alone does not accurately predict the extent of the defect and associated anomalies. The primary management of anorectal malformations is surgical. After the defect has been identified, take steps to rule out associated life-threatening defects, which need immediate surgical intervention. Provided no immediate life-threatening problems exist, the newborn is stabilized and kept NPO for further evaluation. IV fluids are provided to maintain glucose and fluid and electrolyte balance. The current recommendation is that surgery be delayed at least 24 hours to properly evaluate for the presence of a fistula and possibly other anomalies. The surgical treatment of anorectal malformations varies according to the defect but usually involves one or possibly a combination of several of the following procedures: anoplasty, colostomy, posterior sagittal anorectoplasty (PSARP) or other pull-through with colostomy, and colostomy (take-down) closure. The first nursing responsibility is assisting in identification of anorectal malformations. A newborn who does not pass stool within 24 hours after birth or has meconium that appears at a location other than the anal opening requires further assessment. Preoperative care includes diagnostic evaluation, GI decompression, bowel preparation, and IV fluids. For the newborn with a perineal fistula, an anoplasty is performed, which involves moving the fistula opening to the center of the sphincter and enlarging the rectal opening. Postoperative nursing care after anoplasty is primarily directed toward healing the surgical site without other complications. A program of anal dilations is usually initiated when the child returns for the 2-week check-up. Feedings are started soon after surgical repair, and breastfeeding is encouraged because it causes less constipation. CELIAC DISEASE (Malabsorption Syndrome) Celiac disease is a sensitivity or abnormal immunologic response to protein, particularly the gluten factor of protein found in grains—wheat, rye, oats, and barley. When children with the disorder ingest gluten, changes occur in their intestinal mucosa or villi that prevent the absorption of foods, especially fat, across the intestinal villi into the bloodstream. Celiac disease, also known as gluten-induced enteropathy, gluten sensitive enteropathy, and celiac sprue, is a permanent intestinal intolerance to dietary wheat gliadin and related proteins that produces mucosal lesions in genetically susceptible individuals. As a result, children develop steatorrhea (bulky, foul-smelling, fatty stools); deficiency of fat-soluble vitamins A, D, K, and E (the vitamins are not absorbed because the fat is not absorbed); malnutrition; and a distended abdomen from the fat, bulky stools. Because vitamin D is one of the fat-soluble vitamins, rickets or loss of calcium from bones may occur. Hypoprothrombinemia may occur from loss of vitamin K. In addition, children may have hypochromic anemia (iron-deficiency anemia) and hypoalbuminemia from poor protein absorption. Celiac disease is characterized by villous atrophy in the small bowel in response to the protein gluten. 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 caused by the reduced absorptive surface area. Genetic predisposition is an essential factor in the development of celiac disease. Membrane receptors involved in preferential antigen presentation to CD4+ T cells play a crucial role in the immune response characteristic of celiac disease. Genes located on the HLA region of chromosome 6, namely HLA-DQ2 or HLA-DQ8, are found in almost 100% of those affected with celiac disease (Murdock and Johnston, 2005). When the inflammatory reaction is activated by gluten, CD4+ T cells produce cytokines, which are likely to contribute to the intestinal damage. The damage consists of infiltration of the lamina propria, crypt hyperplasia, and villous atrophy and flattening. With sufficient villous atrophy, malabsorption occurs. Typically, children are seen with impaired growth, chronic diarrhea, abdominal distention, muscle wasting with hypotonia, poor appetite, and lack of energy. The clinical manifestations are usually insidious and chronic. The first evidence may be growth failure and diarrhea. Less typical presentation has been observed in children ages 5 to 7 years who have abdominal pain; nausea; vomiting; bloating; constipation; or extraintestinal manifestations, including iron deficiency anemia, short stature, pubertal delay, dental enamel defects, alopecia, and abnormal LFT results. Older children have been found to have osteoporosis. Untreated celiac disease can evolve into celiac crisis, characterized by abdominal distention, explosive watery diarrhea, and dehydration with electrolyte imbalance, leading to hypotensive shock and lethargy. The diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of villous atrophy with hyperplasia of the crypts and abnormal surface epithelium while the patient is eating adequate amounts of gluten and a full clinical remission after gluten is withdrawn. Within 1 or 2 days of instituting the diet, most children with celiac disease demonstrate a favorable response, including weight gain and improved appetite. Within a few weeks, there is resolution of the diarrhea and steatorrhea. Commercially available serologic tests for celiac disease include antigliadin antibodies of both the immunoglobulin A and G classes (IgA and IgG); antiendomysium IgA; and antitissue transglutaminase IgA (anti-TG2) and IgG antibodies for screening first-degree relatives of known celiac disease patients and those with known celiac disease– associated disorders such as type 1 diabetes, thyroiditis, arthritis, primary biliary cirrhosis, Down syndrome, Turner syndrome, Williams syndrome, and osteopenia or osteoporosis. False-positive results are likely when only one serologic test is used because patients with these disorders can also test positive for these antibodies. Use of more than one test increases diagnostic accuracy. Ruling out total IgA deficiency is necessary to minimize false-negative results Treatment is to continue the gluten-free diet for life because there is some suggestion that these children are more prone to GI carcinoma later in life if they do not continue the diet into adulthood. In addition to this, children need to have water-soluble forms of vitamins A and D administered. Both iron and folate may be necessary as well to correct any anemia present. The main nursing consideration is helping the child adhere to the dietary regimen. This requires a wheat-, barley-, and rye-free diet; oats may be safe for most patients but contamination with other gluten products may occur in harvesting; therefore, caution should be exercised with oats. Children who have silent celiac disease, without clinical manifestations, should also adhere to a strict gluten-free diet. Considerable time is involved in explaining the disease process to the child and parents, the specific role of gluten in aggravating the disorder, and the 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, most individuals who relax their diet will experience a relapse of their disease and possibly exhibit growth restriction, 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 an infant’s or young child’s diet easily but monitoring the diet of a school-age child or adolescent is more difficult. Luncheon preparation away from home is particularly difficult because 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 and 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 because 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 registered dietitian to provide children and their families with detailed dietary instructions and education. PROBLEMS IN METABOLISM AND ENDOCRINE GROWTH HORMONE DEFICIENCY If production of human growth hormone (GH, or somatotropin) is deficient, children cannot grow to full size. As a result, children remain in proportion but well below the average on a standard growth chart. Deficient production of GH may result from a nonmalignant cystic tumor of embryonic origin that places pressure on the pituitary gland or from increased intracranial pressure because of trauma. In most children with hypopituitarism, the cause of the defect is unknown. It may have a genetic origin. HYPOPITUITARISM (DWARFISM) Hypopituitarism is diminished or deficient secretion of pituitary hormones. The consequences of the condition depend on the degree of dysfunction and can lead to gonadotropin deficiency with absence or regression of secondary sex characteristics; growth hormone (GH) deficiency, in which children display retarded somatic growth; thyroid-stimulating hormone (TSH) deficiency, which produces hypothyroidism; and corticotropin deficiency, which results in manifestations of adrenal hypofunction. Congenital Hypopituitarism can be seen in newborn infants, often because of birth trauma. Symptoms of hypoglycemia and seizure activity often manifest within the first 24 hours after birth. Idiopathic Hypopituitarism, or idiopathic pituitary growth failure, is usually related to GH deficiency, which inhibits somatic growth in all cells of the body. Growth failure is defined as an absolute height of less than −2 standard deviation (SD) for age or a linear growth velocity consistently less than −1 SD for age. When this occurs without the presence of hypothyroidism, systemic disease, or malnutrition, then an abnormality of the GH–insulin-like growth factor (IGF-I) axis should be considered. Not all children with short stature have GH deficiency. In most instances, the cause is either familial short stature or constitutional growth delay. Familial short stature refers to otherwise healthy children who have ancestors with adult height in the lower percentiles. Constitutional growth delay refers to individuals (usually boys) with delayed linear growth, generally beginning as a toddler, and skeletal and sexual maturation that is behind that of age mates. Typically, these children will reach normal adult height. Often there is a history of a similar pattern of growth in one of the child’s parents or other family members. The untreated child will proceed through normal changes as expected based on bone age. Although treatment with GH is not usually indicated, its use has become controversial, especially in relation to parental and child requests for treatment to accelerate growth. Children with hypopituitarism generally grow normally during the first year and then follow a slowed growth curve that is below the third percentile. Skeletal proportions and weight are normal for the age, but these children may appear younger than their chronologic age. Dentition is delayed, and teeth may be overcrowded and malposition because of the undeveloped jaw. Sexual development is usually delayed but is otherwise normal unless the gonadotropin hormones are deficient. Growth may extend into the third or fourth decade of life, but permanent height is usually diminished if the disorder is left untreated. Symptoms such as headache and vision changes may indicate the presence of a tumor. Only a small number of children with delayed growth or short stature have hypopituitary dwarfism. Usually, the cause is constitutional delay. Diagnostic evaluation is aimed at isolating organic causes, which, in addition to GH deficiency, may include hypothyroidism, over-secretion of cortisol, gonadal aplasia, chronic illness, nutritional inadequacy, Russell-Silver dwarfism, or hypochondroplasia. A complete diagnostic evaluation should include a family history, a history of the child’s growth patterns and previous health status, physical examination, psychosocial evaluation, radiographic surveys, and endocrine studies. Accurate measurement of height (using a calibrated stadiometer) and weight and comparison with standard growth charts are essential. Multiple height measures reflect a more accurate assessment of abnormal growth patterns. Parental height and familial patterns of growth are important clues to diagnosis. A skeletal survey in children younger than 3 years of age and radiographic examination of the hand–wrist for centers of ossification (bone age) in older children are important in evaluating growth. Definitive diagnosis is based on absent or subnormal reserves of pituitary GH. Because GH levels are variable in children, GH stimulation testing is usually required for diagnosis. Initial assessment of the serum IGF-I and IGF binding protein 3 (IGFBP3) indicates a need for further evaluation of GH dysfunction if levels are less than −1 SD below the mean for age. It is recommended that GH stimulation tests be reserved for children with low serum IGF-I and IGFBP3 levels and poor growth who do not have other causes for short stature. GH stimulation testing involves the use of pharmacologic agents such as levodopa, clonidine, arginine, insulin, propranolol, or glucagon to provoke the release of GH. Children with poor linear growth, delayed bone age, and abnormal GH stimulation tests are considered GH deficient. Treatment of GH deficiency caused by organic lesions is directed toward correction of the underlying disease process (e.g., surgical removal or irradiation of a tumor). The definitive treatment of GH deficiency is replacement of GH, which is successful in 80% of affected children. Biosynthetic GH is administered subcutaneously on a daily basis. Growth velocity increases in the first year and then declines in subsequent years. Final height is likely to remain less than normal (Bryant, Baxter, Cave, and others, 2007), and early diagnosis and intervention are essential. The principal nursing consideration is identifying children with growth problems. Even though most growth problems are not a result of organic causes, any delay in normal growth and sexual development poses special emotional adjustments for these children. The nurse may be a key person in helping to establish a diagnosis. Preparation of the child and family for diagnostic testing is especially important if a number of tests are being performed, and the child requires particular attention during provocative testing. Blood samples are usually taken every 30 minutes for a 3-hour period. Children also have difficulty overcoming hypoglycemia generated by tests with insulin, so they must be observed carefully for signs of hypoglycemia, but those receiving glucagon are at risk of nausea and vomiting. Clonidine may cause hypotension, requiring administration of intravenous (IV) fluids. Children undergoing hormone replacement require additional support. The nurse should provide education for patient self-management during the school-age years. Nursing functions include family education concerning medication preparation and storage, injection sites, injection technique, and syringe disposal. PITUITARY HYPERFUNCTION (GIGANTISM) Excess GH before closure of the epiphyseal shafts results in proportional overgrowth of the long bones until the individual reaches a height of 2.4 m (8 ft) or more. Vertical growth is accompanied by rapid and increased development of muscles and viscera. Weight is increased but is usually in proportion to height. Proportional enlargement of head circumference also occurs and may result in delayed closure of the fontanels in young children. Children with a pituitary-secreting tumor may also demonstrate signs of increasing intracranial pressure, especially headache. If over-secretion of GH occurs after epiphyseal closure, growth is in the transverse direction, producing a condition known as acromegaly. Typical facial features include overgrowth of the head, lips, nose, tongue, jaw, and paranasal and mastoid sinuses; separation and malocclusion of the teeth in the enlarged jaw; disproportion of the face to the cerebral division of the skull; increased facial hair; thickened, deeply creased skin; and an increased tendency toward hyperglycemia and diabetes mellitus (DM). Acromegaly can develop slowly, leading to delays in diagnosis and treatment. Diagnosis is based on a history of excessive growth during childhood and evidence of increased levels of GH. Radiographic studies may reveal a tumor in an enlarged sella turcica, normal bone age, enlargement of bones (e.g., the paranasal sinuses), and evidence of joint changes. Endocrine studies to confirm excess of other hormones, specifically thyroid, cortisol, and sex hormones, should also be included in the differential diagnosis. If a lesion is present, surgery is performed to remove the tumor when feasible. Other therapies aimed at destroying pituitary tissue include external irradiation and radioactive implants. New pharmacologic agents have evolved and may be used in combination with other therapies. Depending on the extent of surgical extirpation and degree of pituitary insufficiency, hormone replacement with thyroid extract, cortisone, and sex hormones may be necessary.  The primary nursing consideration is early identification of children with excessive growth rates. Although medical management is unable to reduce growth already attained, further growth can be retarded. The earlier the treatment, the more control there is in predetermining a normal adult height. Nurses should also observe for signs of a tumor, especially headache, and evidence of concurrent hormonal excesses, particularly the gonadotropins, which cause sexual precocity. Children with excessive growth rates require as much emotional support as those with short stature. SYNDROME OF INAPPROPRIATE ANTIDIURETIC HORMONE (SIADH) Syndrome of inappropriate antidiuretic hormone (SIADH) results from an excessive amount of serum ADH. It is seen in children with central nervous system infections, brain tumors, and brain trauma; in children with pulmonary disorders such as pneumonia, asthma, or cystic fibrosis; and in children receiving positive-pressure ventilation. Some medications, including diuretics and chemotherapy, have been associated with SIADH. Failure of normal feedback mechanisms from the hypothalamus, pituitary gland, and kidney results in excessive secretion of ADH, leading to water reabsorption despite the presence of low serum osmolality. ADH secretion causes increased permeability of the distal renal tubules and collecting ducts and resulting in water reabsorption. Elevated ADH also causes suppression of the renin-angiotensin mechanism and sodium excretion. The outcome is water intoxication (an abnormal proportion of water to sodium in the extracellular fluid) and hyponatremia. Signs of SIADH are related to water intoxication and hyponatremia, and include elevated blood pressure, distended jugular veins, crackles in lung fields, weight gain without edema, fluid and electrolyte imbalance, and concentrated urine with decreased urine output. As serum sodium levels continue to fall, lethargy, confusion, headache, altered level of consciousness, seizures, and coma occur because of cerebral edema. Laboratory findings include a high urine osmolality, low serum osmolality, low serum sodium, high urine sodium, and decreased blood urea nitrogen. Fluids are restricted to prevent further dilution of the blood. Medications include diuretics, demeclocycline to block action of ADH at the renal collecting tubules, and hypertonic saline IV fluids. Nursing care focuses on preventing injury, monitoring fluid balance, administering medications, and managing nutritional intake. Monitor intake and output, serum sodium, urine osmolality, and specific gravity. Educate the parents about the child’s fluid restrictions and the hidden sources of water and fluids in foods to help avoid excessive fluid intake. HYPOTHYROIDISM (CRETINISM) Hypothyroidism is a disorder in which levels of active thyroid hormones are decreased. It may be congenital or acquired. Congenital hypothyroidism occurs in approximately 1 in 3000 to 1 in 4000 live births worldwide. It is twice as common in females as it is in males. Thyroid hormones are important for growth and development and for metabolizing nutrients and energy. When these hormones are not available to stimulate other hormones or specific target cells, growth is delayed, and intellectual disability develops. Congenital hypothyroidism is usually caused by a spontaneous gene mutation, an autosomal recessive genetic transmission of an enzyme deficiency, hypoplasia or aplasia of the thyroid gland, failure of the CNS–thyroid feedback mechanism to develop, or iodine deficiency. Intellectual disability is irreversible if the disorder is not treated. Acquired hypothyroidism can be idiopathic or result from autoimmune thyroiditis (Hashimoto thyroiditis), late-onset thyroid dysfunction, isolated thyroid-stimulating hormone (TSH) deficiency caused by pituitary or hypothalamic dysfunction, or exposure to drugs or substances such as lithium that interfere with thyroid hormone synthesis. In the case of Hashimoto thyroiditis, the thyroid is infiltrated by lymphocytes that cause an autoimmune reaction and an enlarged thyroid. A genetic predisposition to autoimmune thyroiditis and an autosomal dominant inheritance of thyroid antibodies has been identified. Infants with congenital hypothyroidism have few clinical signs of the disorder in the first weeks of life. Symptoms may include jaundice, thick tongue, hypotonia, umbilical hernia, hoarse cry, dry skin, constipation, and large fontanelles. Children with acquired hypothyroidism have many of the same signs as adults: decreased appetite; dry, cool skin; thinning hair or hair loss; depressed deep tendon reflexes; bradycardia; constipation; sensitivity to cold temperatures; abnormal menses; and a goiter (a nontender enlarged thyroid gland). Manifestations unique to children include change in past normal growth patterns with a weight increase, decreased height velocity, delayed bone and dental age, hypotonia with poor muscle tone, and delayed or precocious puberty. Congenital hypothyroidism is usually detected during newborn screening. A decreased T4, normal T3, and elevated thyroid-stimulating hormone level indicate hypothyroidism. An elevated TSH level indicates that the disease originated in the thyroid, not the pituitary. Levothyroxine is the drug of choice for newborns with congenital hypothyroidism. The recommended starting dose is 10 to 15 mcg/kg per day. The dose is increased gradually as the child grows to ensure a euthyroid (thyroid hormones in appropriate balance) state. A pediatric endocrinologist monitors treatment. To ensure an adequate growth rate and prevent intellectual disability, the hormone must be taken throughout life. Periodic evaluation of T4 and TSH serum levels, bone age, and growth parameters is necessary to assess for signs of excess or inadequate thyroid hormone. Antithyroid antibodies are measured in children with a goiter and suspected Hashimoto thyroiditis because increased titers of antithyroglobulin and anti-microsomal antibodies are often found. Children with congenital hypothyroidism that are diagnosed before 3 months of age have the best prognosis for optimal mental development. Children with acquired hypothyroidism usually have normal growth following a period of catch-up growth. Many adolescents with Hashimoto thyroiditis have a spontaneous remission. Nursing care focuses on teaching the parents and child about the disorder and its treatment and monitoring the child’s growth rate. Explain how to administer thyroid hormone (e.g., tablets can be crushed and mixed in a small amount of formula or applesauce if the child gets all of the medication). Advise parents that the child may experience temporary sleep disturbances or behavioral changes in response to therapy. Teach the parents how to assess for an increased pulse rate, which could indicate the presence of too much thyroid hormone, and advise them to report problems such as fatigue, which could indicate an improper drug dose that needs to be adjusted. HYPERTHYROIDISM Hyperthyroidism occurs when thyroid hormone levels are increased, resulting in excessive levels of circulating thyroid hormones. Graves disease is the most common cause of hyperthyroidism in children, occurring more often in females and in children ages 11 to 15 years. Graves disease is an autoimmune disorder. Immunoglobulins produced by the B lymphocytes stimulate over secretion of thyroid hormones, resulting in the clinical symptoms. It has a high familial incidence. Other less common causes of hyperthyroidism result from thyroiditis and thyroid hormone– producing tumors, including thyroid adenomas and carcinomas, and pituitary adenomas. Congenital hyperthyroidism can occur in infants of mothers with Graves disease as a result of transplacental transfer of immunoglobulins. This condition generally resolves by 6 to 12 weeks of age but can last longer. Signs and symptoms are caused by hyperactivity of the sympathetic nervous system and may include an enlarged, nontender thyroid gland (goiter), prominent or bulging eyes (exophthalmos), eyelid lag, tachycardia, nervousness, increased appetite with weight loss, emotional lability, moodiness, heat intolerance, hypertension, hyperactivity, irregular menses, insomnia, tremor, and muscle weakness. The thyroid gland may be slightly enlarged or grow to 3 to 4 times its normal size; feel warm, soft, and fleshy; and have an auditory bruit on auscultation. Onset is subtle, and the condition often goes unrecognized for 1 to 2 years. Children with Graves disease usually have difficulty concentrating, behavioral problems, and declining performance in school. They become easily frustrated in the classroom and overheated and fatigued during physical education class. Children with this disorder find it difficult to relax or sleep. These symptoms usually prompt parents to seek medical treatment for the child. The most serious complication of hyperthyroidism is severe thyrotoxicosis, also called thyroid crisis or thyroid storm. It is a life-threatening emergency resulting from extreme hyperthyroidism, in which elevated circulating levels of TH result in a hypermetabolic state. Symptoms include muscle weakness, diaphoresis, tachycardia, tremor, palpitations, diarrhea, irritability, nervousness, and anxiety. Diagnostic studies include laboratory evaluation of serum TSH, T3, and T4 levels and a thyroid scan. T3 and T4 levels are markedly elevated, whereas the TSH level is decreased. Serum studies are also performed to detect thyroid autoantibodies anti-TG (antithyroglobulin) and anti-TPO (antiperoxidase), usually present in Graves disease and Hashimoto thyroiditis. A thyroid scan is performed to identify nodules or to confirm the high uptake of radioactive iodine associated with Graves disease. The goal of clinical therapy is to inhibit excessive secretion of thyroid hormones. Treatment may include medication therapy, radiation therapy, or surgery. Medication therapy is most often the initial treatment, but compliance is often a problem because of side effects. Methimazole (Tapazole) and propylthiouracil (PTU) are antithyroid drugs that are used in children with hyperthyroidism. Because of the concern for severe liver disease with the administration of PTU, current recommendations are that children with hyperthyroidism receive methimazole instead. Symptoms usually improve within weeks of starting treatment. Adjunct therapy with beta-adrenergic blocking agents such as propranolol or atenolol may be administered to relieve symptoms of tremors, tachycardia, lid lag, and excessive sweating. Less than 30% of children achieve remission after a 2-year course of treatment with medication. Radiation therapy or thyroidectomy are other options if medication therapy is not effective. Thyroidectomy (removal of most of the thyroid) provides an immediate cure and avoids radiation and possible long-term complications of radioactive iodine. Removal of the thyroid gland results in hypothyroidism. Complications of thyroidectomy include hemorrhage, hypocalcemia, and damage to the laryngeal nerve paresis.  Nursing care focuses on teaching the child and parents about the disorder and its treatment, promoting rest, providing emotional support, and, if the child needs surgery, providing preoperative and postoperative teaching and care. Promote increased caloric intake by providing five or six moderate meals per day. Encourage the child and family to express their feelings and concerns about the disorder. Pointing out even slight improvements in the child’s condition increases adherence with therapy. CUSHING SYNDROME Cushing syndrome, also called adrenocortical hyperfunction, is characterized by a group of symptoms resulting from excess blood levels of glucocorticoids (especially cortisol). The most common cause of Cushing syndrome is the prolonged administration of glucocorticoid hormones. Cushing disease is a type of Cushing syndrome and is caused by a pituitary tumor. During infancy most cases of endogenous Cushing disease are caused by a functioning adrenocortical tumor. The most common cause of endogenous Cushing syndrome in children older than 7 years of age is Cushing disease in which a pituitary tumor (adenoma) secretes excessive ACTH. This leads to bilateral adrenal hyperplasia. The remainder of this discussion will focus on the child with Cushing syndrome caused by a pituitary tumor (Cushing disease). Obesity is common in children with Cushing syndrome. Excessive weight gain is followed by slowed linear growth. The child develops the characteristic cushingoid features, which include a rounded (moon) face with prominent cheeks. Additional manifestations include hirsutism, acne, deepening of the voice, and hypertension. Older children may also experience delayed puberty, irregular menstrual periods, headaches, weakness, pathologic fractures, emotional problems, and hyperglycemia. Diagnosis is based on characteristic physical findings and laboratory values, including increased 24- hour urinary levels of free cortisol and elevated nighttime salivary cortisol level. The child will also have an abnormal glucose tolerance test. The adrenal-suppression test using an 11 p.m. dose of dexamethasone reveals that adrenal cortisol output is not suppressed overnight as would occur normally in children. Computed tomography (CT) and magnetic resonance imaging (MRI) are used to detect the specific location of tumors in the adrenal and pituitary glands. Surgical removal of the pituitary adenoma is the current treatment of choice when this is the cause of Cushing syndrome. Irradiation of the pituitary is performed when surgical removal of the adenoma does not substantially reduce cortisol levels. Bilateral removal of the adrenal glands may be necessary in some cases to stop the excessive secretion of cortisol. Lifelong hormone replacement is required when both adrenal glands are removed. Nursing assessment includes monitoring the child’s vital signs, fluid status, nutritional status, and weight. Additional assessment includes monitoring muscle strength and endurance. Teach the child and family about the disorder and its treatment. For children undergoing surgery, provide preoperative and postoperative teaching and care. DIABETES MELLITUS Diabetes mellitus, the most common metabolic disease in children, is a disorder of hyperglycemia resulting from defects in insulin secretion, insulin action, or both, leading to abnormalities in carbohydrate, protein, and fat metabolism. There are two main types of diabetes. Most children have immune-mediated type 1 diabetes, formerly called insulin-dependent diabetes mellitus or juvenile diabetes. Type 1 Diabetes Type 1 diabetes results from destruction of pancreatic islet beta cells, which fail to secrete insulin. The body becomes dependent on exogenous sources of insulin. Type 1 diabetes is a multifactorial disease caused by autoimmune destruction of insulin-producing pancreatic beta cells in individuals who are genetically predisposed. Type 1 diabetes has familial tendencies but does not show any specific pattern of inheritance. The number of autoantibodies helps predict the risk of developing type 1 diabetes. For children with one antibody, the risk is only 10% to 15%, while those with three or more antibodies have a risk of 55% to 90%. The child inherits a susceptibility to the disease rather than the disease itself. It is believed that an event such as a virus or other environmental factors trigger the inflammatory process, resulting in development of islet cell serum antibodies. These antibodies can be detected in the blood months to years before the onset of beta cell destruction. Insulin helps transport glucose into the cells so that the body can use it as an energy source. It also prevents the outflow of glucose from the liver to the general circulation. Environmental factors such as enteroviruses or toxins are believed to lead to an autoimmune destruction of the beta cells in the islets of Langerhans. Antigens are generated, leading to production of antibodies that indicate ongoing destruction of the islet cells. Chronic immune-mediated destruction of the beta cells continues over a period of time. Symptoms of type 1 diabetes are evident when approximately 90% of the beta cells have been destroyed. As the secretion of insulin decreases, the blood glucose level rises and the glucose level inside the cells decreases. When the renal threshold for glucose (180 mg/dL) is exceeded, glycosuria (abnormal amount of glucose in the urine) occurs as a result of osmotic diuresis. Fluids follow the highly osmotic glucose and water is excreted in large volumes of urine (polyuria). When glucose is unavailable to the cells for metabolism, free fatty acids provide an alternate source of energy. The liver metabolizes fatty acids at an increased rate, producing acetyl coenzyme A (CoA). The by-products of acetyl CoA metabolism (ketone bodies) accumulate in the body, resulting in a state of metabolic acidosis, or ketoacidosis.  Type 2 Diabetes Type 2 diabetes usually arises because of insulin resistance in which the body fails to use insulin properly combined with relative (rather than absolute) insulin deficiency. People with type 2 can range from predominantly insulin resistant with relative insulin deficiency to predominantly deficient in insulin secretion with some insulin resistance. It typically occurs in those who are older than 45 years of age, are overweight and sedentary, and have a family history of diabetes. The symptomatology of diabetes is more readily recognizable in children than in adults, so it is surprising that the diagnosis may sometimes be missed or delayed. Diabetes is a great imitator; influenza, gastroenteritis, and appendicitis are the conditions most often diagnosed when it turns out that the disease is really diabetes. CAUSE CLINICAL MANIFESTATIONS CLINICAL THERAPY TYPE 1 DM Polyuria, polydipsia Blood glucose monitoring Immune mediated, insulin May have polyphagia Insulin deficiency due to pancreatic Weight loss Dietary management, balancing beta-cell destruction Ketoacidosis may be present at carbohydrate intake to insulin diagnosis, at continued risk for Exercise ketoacidosis Short duration of symptoms Initial period of decreased insulin requirement, then need insulin for survival. TYPE 2 DM Obese, little or no weight loss, or may Diet with decreased calories and low- Insulin resistance with have significant weight loss fat foods relative insulin secretory Acanthosis nigricans Decrease sedentary activity time or defect Long duration of symptoms increase routine physical activity Polyuria, polydipsia, may be mild or Blood glucose monitoring absent Oral medication (metformin) to Glycosuria with or without ketonuria improve insulin sensitivity Ketoacidosis may be present May need insulin Lipid disorders Hypertension Androgen-mediated problems such as acne, hirsutism, menstrual disturbances, polycystic ovary disease Excessive weight gain and fatigue due to insulin resistance Insulin is needed to support the metabolism of carbohydrates, fats, and proteins, primarily by facilitating the entry of these substances into the cells. Insulin is needed for the entry of glucose into the muscle and fat cells, prevention of mobilization of fats from fat cells, and storage of glucose as glycogen in the cells of liver and muscle. Insulin is not needed for the entry of glucose into nerve cells or vascular tissue. The chemical composition and molecular structure of insulin are such that it fits into receptor sites on the cell membrane. Here it initiates a sequence of poorly defined chemical reactions that alter the cell membrane to facilitate the entry of glucose into the cell and stimulate enzymatic systems outside the cell that metabolize the glucose for energy production. With a deficiency of insulin, glucose is unable to enter the cells, and its concentration in the bloodstream increases. The increased concentration of glucose (hyperglycemia) produces an osmotic gradient that causes the movement of body fluid from the intracellular space to the interstitial space and then to the extracellular space and into the glomerular filtrate to “dilute” the hyperosmolar filtrate. Normally, the renal tubular capacity to transport glucose is adequate to reabsorb all the glucose in the glomerular filtrate. When the glucose concentration in the glomerular filtrate exceeds the renal threshold (6180 mg/dl), glucose spills into the urine (glycosuria) along with an osmotic diversion of water (polyuria), a cardinal sign of diabetes. The urinary fluid losses cause the excessive thirst (polydipsia) observed in diabetes. This water “washout” results in a depletion of other essential chemicals, especially potassium. Protein is also wasted during insulin deficiency. Because glucose is unable to enter the cells, protein is broken down and converted to glucose by the liver (glucogenesis); this glucose then contributes to the hyperglycemia. These mechanisms are similar to those seen in starvation when substrate (glucose) is absent. The body is actually in a state of starvation during insulin deficiency. Without the use of carbohydrates for energy, fat and protein stores are depleted as the body attempts to meet its energy needs. The hunger mechanism is triggered, but increased food intake (polyphagia) enhances the problem by further elevating blood glucose. Long-term complications of diabetes involve both the microvasculature and the macrovasculature. The principal microvascular complications are nephropathy, retinopathy, and neuropathy. Microvascular disease develops during the first 30 years of diabetes, beginning in the first 10 to 15 years after puberty, with renal involvement evidenced by proteinuria and clinically apparent retinopathy. Macrovascular disease develops after 25 years of diabetes and creates the predominant problems in patients with type 2 DM. The process appears to be one of glycosylation, wherein proteins from the blood become deposited in the walls of small vessels (e.g., glomeruli), where they become trapped by “sticky” glucose compounds (glycosyl radicals). The buildup of these substances over time causes narrowing of the vessels, with subsequent interference with microcirculation to the affected areas. With poor diabetic control, vascular changes can appear as early as 2 1 2 to 3 years after diagnosis; however, with good to excellent control, changes can be postponed for 20 or more years. Intensive insulin therapy appears to delay the onset and slow the progression of retinopathy, nephropathy, and neuropathy. Hypertension and atherosclerotic cardiovascular disease are also major causes of morbidity and mortality in patients with DM. Other complications have been observed in children with type 1 DM. Hyperglycemia appears to influence thyroid function, and altered function is frequently observed at the time of diagnosis and in poorly controlled diabetes. Limited mobility of small joints of the hand occurs in 30% of 7- to 18-year-old children with type 1 DM and appears to be related to changes in the skin and soft tissues surrounding the joint because of glycosylation. Three groups of children who should be considered as candidates for diabetes are (1) children who have glycosuria, polyuria, and a history of weight loss or failure to gain despite a voracious appetite; (2) those with transient or persistent glycosuria; and (3) those who display manifestations of metabolic acidosis, with or without stupor or coma. In every case, diabetes must be considered if there is glycosuria, with or without ketonuria, and unexplained hyperglycemia. Glycosuria by itself is not diagnostic of diabetes. Other sugars, such as galactose, can produce a positive result with certain test strips, and a mild degree of glycosuria can be caused by other conditions, such as infection, trauma, emotional or physical stress, hyperalimentation, and some renal or endocrine diseases. An 8-hour fasting blood glucose level of 126 mg/dl or more, a random blood glucose value of 200 mg/dl or more accompanied by classic signs of diabetes, or an oral glucose tolerance test (OGTT) finding of 200 mg/dl or more in the 2- hour sample is almost certain to indicate diabetes. Postprandial blood glucose determinations and the traditional OGTTs have yielded low detection rates in children and are not usually necessary for establishing a diagnosis. Serum insulin levels may be normal or moderately elevated at the onset of diabetes; delayed insulin response to glucose indicates impaired glucose tolerance. The management of the child with type 1 DM consists of a multidisciplinary approach involving the family; the child (when appropriate); and professionals, including a pediatric endocrinologist, diabetes nurse educator, nutritionist, and exercise physiologist. The definitive treatment is replacement of insulin that the child is unable to produce. However, insulin needs are also affected by emotions, nutritional intake, activity, and other life events such as illnesses and puberty. The complexity of the disease and its management requires that the child and family incorporate diabetes needs into their lifestyle. Medical and nutritional guidance are primary, but management also includes continuing diabetes education, family guidance, and emotional support. CAUSE CLINICAL MANIFESTATIONS CLINICAL THERAPY HYPOGLYCEMIA Insulin dose too high for food Rapid onset If conscious, give 15g of eaten Irritability, nervousness, tremors, shaky carbohydrate. Wait 15 minutes and Insulin injection into muscle feeling, difficulty concentrating or recheck blood glucose level. Give Too much exercise for insulin speaking, behavior change, confusion, another 15g of carbohydrate if 70 dose repeating something over and over mg/dL or below. Recheck the blood Too long between meals/ Unconsciousness, seizure, shallow glucose level in 15 minutes. snacks breathing, tachycardia Too few carbohydrates eaten Pallor, sweating If unconscious, give Illness, stress Moist mucous membranes, hunger glucagon by injection. Headache, dizziness, blurred vision, double vision, photophobia Numb lips or mouth HYPERGLYCEMIA Insulin dose too low for food Gradual onset Give additional insulin at usual eaten Lethargy, sleepiness, slowed injection time. Illness or injury, stress responses, or confusion Too many carbohydrates Deep, rapid breathing Give correction scale insulin doses eaten Flushed skin, dry skin for specific blood glucose levels Meals/ snacks too close Dry mucous membranes, thirst, hunger, when ill or injured. together dehydration Insulin injected just under skin Weakness, fatigue Give extra injections if or injected into hypertrophied Headache, abdominal pain, nausea, hyperglycemia and moderate to areas vomiting large ketones. Decreased activity Blurred vision Shock Increase fluids. Nursing care focuses on teaching the child and parents about the disease and its management, planning dietary intake, providing emotional support, and planning strategies for daily management in the community. DIABETIC KETOACIDOSIS (DKA) When insulin is absent or insulin sensitivity is altered, glucose is unavailable for cellular metabolism, and the body chooses alternate sources of energy, principally fat. Consequently, fats break down into fatty acids, and glycerol in the fat cells is converted by the liver to ketone bodies (β-hydroxybutyric acid, acetoacetic acid, acetone). Any excess is eliminated in the urine (ketonuria) or the lungs (acetone breath). The ketone bodies in the blood (ketonemia) are strong acids that lower serum pH, producing ketoacidosis. Ketones are organic acids that readily produce excessive quantities of free hydrogen ions, causing a fall in plasma pH. Then chemical buffers in the plasma, principally bicarbonate, combine with the hydrogen ions to form carbonic acid, which readily dissociates into water and carbon dioxide. The respiratory system attempts to eliminate the excess carbon dioxide by increased depth and rate (Kussmaul respirations, or the hyperventilation characteristic of metabolic acidosis). The ketones are buffered by sodium and potassium in the plasma. The kidneys attempt to compensate for the increased pH by increasing tubular secretion of hydrogen and ammonium ions in exchange for fixed base, thus depleting the base buffer concentration. With cellular death, potassium is released from the cells (intracellular fluid) into the bloodstream (extracellular fluid) and excreted by the kidneys, where the loss is accelerated by osmotic diuresis. The total body potassium is then decreased even though the serum potassium level may be elevated as a result of the decreased fluid volume in which it circulates. Alteration in serum and tissue potassium can lead to cardiac arrest. If these conditions are not reversed by insulin therapy in combination with correction of the fluid deficiency and electrolyte imbalance, progressive deterioration occurs, with dehydration, electrolyte imbalance, acidosis, coma, and death. Diabetic ketoacidosis (DKA) should be diagnosed promptly in a seriously ill patient and therapy instituted in an intensive care unit. Ketoacidosis must be differentiated from other causes of acidosis or coma, including hypoglycemia, uremia, gastroenteritis with metabolic acidosis, salicylate intoxication encephalitis, and other intracranial lesions. DKA is a state of relative insulin insufficiency and may include the presence of hyperglycemia (blood glucose level ≥200 mg/ dl), ketonemia (strongly positive), acidosis (pH