112 GI Conditions

Child with a Gastrointestinal Disorder

Chapter 34

Development of GI Abnormalities

  • A newborn has a stomach capacity of approximately 15-20 ml.

  • By the end of the 2nd week, the stomach capacity expands to 90 ml.

  • Health History:

    • Food habits

    • Elimination habits

    • Known food allergies

    • Use of vitamins or supplements

    • Cultural practices

    • Problems

    • Past medical history

  • Physical Assessment:

    • Height and weight

    • Elimination habits

    • Hydration status

    • Inspect abdomen

    • Palpate observing for rebound tenderness

Vomiting

(Emesis)- forceful expulsion or emptying of stomach contents

  • Reasons:

    • GI disorders: pyloric sphincter blockage, reflux, severe gastroenteritis, overdistention.

    • Non-GI disorders: Increased ICP, food intolerance or allergy, ingestion of toxic substance, or chemotherapy.

  • Assessment:

    • Intractable – when vomiting occurs even with use of antiemetics. Consider pyloric stenosis, tumors, obstruction, foreign bodies, and ingestion of poisonous materials.

  • Interventions:

    • Prevent aspiration, Place them upright

    • Place on NPO

    • Fluid imbalance management

    • Nursing considerations: monitor for dehydration, aspiration, sore throat, and weight loss. Oral hygiene should be performed after each vomiting episode.

Gastroesophageal Reflux (GER or GERD)

GER= Gastroesophageal reflux & GERD= Gastroesophageal reflux disease

  • Assessment:

    • Behaviors in relation to eating/feeding patterns

    • Onset in relation to child’s age

    • pH of the stomach contents

    • Coexisting weight loss or failure to thrive (FTT)

    • Severity of discomfort in chest area

  • Procedures:

  • Interventions:

    • Place in upright position for feedings

    • Provide smaller, frequent meals, feed slowly, assess for discomfort

    • Burp frequently

    • Administer GERD meds 30 minutes before meals

    • In infants, work with nutritionist to determine feasibility of thickening formula

  • Nissen’s Fundoplication Surgery:

    • Nursing Considerations: Identify GERD early, differentiate pain from more serious problems such as heart pain, provide pharmacological and non-pharmacological interventions such as positioning after eating and sleep with head of bed (HOB) elevated.

Diarrhea

  • Assessment:

    • Assess frequency and characteristics of stools

    • Measure the overall stool loss & duration of symptoms

    • Check color and consistency of stool

    • Weigh daily

    • Relationship of diarrhea with feeds

    • Check skin intergrity around anus

    • Assess for presence of frank or occult blood in stools

    • Causative factors such as stress, inflammatory bowel disease, food sensitivities or intolerances, allergies, and medications

    • Potential for dehydration

  • Interventions:

    • NPO status

    • Oral rehydration

    • Avoid fruit juices or any fluids with concentrated sugars

    • Follow BRAT diet (bananas, rice, applesauce, toast)

    • Administer antidiarrheals, antiprotozoals, or antibiotics as ordered

    • Cleanse and maintain hygiene

  • Nursing Considerations: Enteric precautions should be used until etiology is identified (C. difficile is highly contagious). Handwashing is crucial, no hand sanitizer.

Constipation

  • Acute: associated with diet low in liquids, which decreases fluid in GI tract and produces hard, drier stools.

  • Treatment: Push fluids and provide a diet rich in fruits and veggies.

  • Chronic: may be related to low fluid intake, decreased activity levels, or poor nutrition.

  • Health Promotion: Establish a toileting routine, consider suppositories or enemas, increase daily fiber.

  • Assessment:

    • Note frequency of passing Hard, dry and infrequent stools.

    • Sample of stool to perform guaiac test for presence of frank or occult blood.

    • Measure abdominal girth and document daily.

    • Assess perirectal area for rashes, swelling, bleeding, fissures, and pain during stooling.

    • Question child and caregiver about reluctance to use toilet.

  • Interventions:

    • Modifying diet to increase fiber and hydration, to requiring daily stool softeners if needed.

    • Nursing considerations: if other measures do not work, may need prescribed Laxatives. If chronic constipation, may need to refer child for further work-up.

Cleft Lip and Cleft Palate

  • Cleft Lip: Occurs during fetal development in approximately the 7th gestational week.

    • Failure of tissues to fuse completely.

    • May be partial or complete and unilateral or bilateral.

    • More common in males.

    • Serious complication includes risk of aspiration during feedings or wet burps due to the incomplete closure of the mouth.

    • Surgical intervention takes place when the infant is at least 10 weeks old and weighs 10 lbs.

  • Cleft Palate: Occurs during the 9th & 10th week.

    • Incidence is higher in girls vs boys.

    • The severity of the incomplete fusion of the bone and tissue of the upper jaw and palate will determine the long-term care.

    • Aspiration is common and the child is at higher risk for more frequent respiratory infections and otitis media.

    • Surgery will not take place until the infant is older than 10 months and weaned from the bottle.

  • Assessment:

    • Observe for respiratory distress during feedings

    • Check for ability to produce complete and quality swallow during feeding

    • Check for abdominal distension during feedings.

  • Interventions:

    • Preoperative: Smaller, frequent feedings slowly in an upright position, burp frequently, improve nutrition use cleft lip nipple with palate flap, medicine droppers, syringe feedings, manual compression on the nipple. Gavage feedings if not tolerating oral feeds.

    • Sterile Water after feed to prevent accumulation in open palate area.

    • Emotional support and education to family.

    • Postoperative: Maintain airway free of secretions and monitor for edema, assess for distress, cyanosis, tachycardia, restlessness, monitor for postoperative complications – no oral temps! Treat pain promptly, prevent trauma to suture line:

    • Nursing Considerations: Focus on prevention of suture line injury.

    • ESSR: Eat, Swallow, Suck, Rest.

Esophageal Atresia (EA)

  • Abnormal Termination of the esophagus, alone or in association with other congenital defects.

  • Presence of esophageal blind pouch at proximal end of esophagus prevents passage of breast milk, formula, or any fluids.

    • Food doesn’t enter stomach and newborn is at risk for aspiration from the pouch, as well as dehydration and starvation

  • Assessment:

    • Presence of excessive oral secretions, coughing, choking & respiratory distress, intermittent Cyanosis associated with fluid intake or excessive secretions.

  • Interventions:

    • Maintain NPO status until PEG tube in place for feeds.

    • Suction frequently to prevent aspiration and respiratory distress.

    • Prepare child for surgery to repair the defect.

  • Nursing considerations:

    • First feed should be very small amount to assess for defect. If tolerates small amounts, then further feedings or breastfeeding can be initiated.

Tracheoesophageal Fistula (TEF)

    -Abnormal opening between trachea and esophagus

  • Assessment:

    • Coughing, choking, and intermittent Cyanosis caused by food passing through fistula into the trachea.

    • Abdominal distension from air entering the stomach from the fistula.

    • Pain or discomfort from gastric acids refluxing across the fistula.

  • Interventions:

    • NPO status until diagnostics and definitive diagnosis reached.

    • Maintain patent airway. Have suction available at all times.

    • Prepare for surgery.

  • Nursing considerations: Educate about the surgical procedure, gastrostomy tube feedings needed until the child is well-healed.

Pyloric Stenosis

  • Muscles around pyloric valve grows, narrowing the valve (stenosis) leading to blocked gastric emptying.

  • Identified by classic episodes of projectile vomiting and an olive-shaped mass during palpation.

  • Etiology: Unknown. Male gender is affected five times more often and white children are at greatest risk.

  • Pyloromyotomy: small incision in abdomen to split the hypertrophied tissue.

  • Assessment:

    • Progressively worsening projectile vomiting.

    • Olive-shaped mass in the upper right quadrant of abdomen.

    • Clinical signs of dehydration – decreased urine output, cry, decreased tears, poor skin turgor, sunken fontanelle.

  • Interventions:

    • Preoperative: NPO, position on right side to prevent aspiration.

    • Postoperative: NGT patency, strict intake and output, correct electrolyte imbalances, introduce clear liquids slowly.

  • Nursing considerations: May present with FTT (failure to thrive) and moderate to severe dehydration. Assess for distress and report any changes immediately.

Hirschsprung’s Disease

  • Also known as congenital aganglionic megacolon.

    • Characterized by absence of neuro tissue called ganglia cells in the lower GI tract.

    • Most commonly affects the rectosigmoid region of the colon and is four times more common in males.

  • Assessment:

    • Usually diagnosed in early infancy but may not be severe enough to present until early toddlerhood.

    • History of infrequent stools, explosive stools, or stools that are thin and ribbon-like.

  • Interventions:

    • Surgical removal of the segment of bowel that doesn’t have the nerves and is causing lack of peristalsis.

    • Diagnostics include tissue biopsy to identify the lack of nerves in the distal colon.

  • Nursing Considerations:

    • Preoperative and Postoperative care focused on nutritional status and elimination patterns.

Intussusception

  • Characterized by Invagination or Telescoping of one bowel segment into the other.

  • Assessment:

    • Acute abdominal pain in the child without previous GI symptoms.

    • Red blood-tinged stools referred to as red currant jelly stool.

    • Abdominal distention and tenderness.

    • Possible passage of bile-stained vomitus.

  • Interventions:

    • Need rapid intervention to prevent tissue inflammation, injury, hypoxia, and tissue death.

    • Barium enema to reduce telescoped bowel.

    • Surgery if enema is not successful.

  • Nursing considerations: Educate families that this condition can recur and immediate care must be sought to prevent complications.

Appendicitis

  • Average age of onset is 10 years old.

  • Assessment:

    • Progressive RLQ pain with nausea/vomiting, chills, and fever.

    • Elevated WBC count.

    • Positive ultrasound indicating enlarged or distended appendix.

    • Sudden stop of abdominal pain must be reported immediately, as it may indicate rupture.

  • Interventions:

    • Preoperative: prepare child and family for surgical removal, keep NPO, maintain semi-fowler’s position for comfort.

    • Postoperative: monitor for pain management, bleeding, and infection; may require NG tube and drain if appendix ruptured.

Inflammatory Bowel Syndromes

  • Ulcerative Colitis:

    • Primarily affects the colon, with continuous lesions involving superficial mucosa. Presents a “lead pipe” appearance on imaging.

    • Assessment: Copious, frequent bloody stools (3-20/day), significant weight loss, anemia, electrolyte imbalances, increased ESR, tachycardia, fever, pallor, fatigue, and “extraintestinal symptoms” such as joint tenderness, arthritis, and skin rashes.

    • Diagnostics: Hematest of stools, colonoscopy, barium enema, and biopsies.

    • Interventions: vary depending on severity

    • Pharmacological: antidiarrheals, anti-inflammatories, and analgesics.

    • Diet modifications to help control diarrhea.

    • Severe cases may require surgery to remove diseased bowel segments.

  • Crohn’s Disease:

    • Ulcerations that can affect mucosa from mouth to anus, presenting as “skip lesions.”

    • Assessment: Bloody diarrhea, cramping abdominal pain, weight loss leading to growth retardation, abscess formation, and perianal fissures or fistulas. Extraintestinal symptoms include finger clubbing, arthritis, amenorrhea, and delayed sexual development.

    • Interventions: Similar pharmacological approach to UC; nutritional deficiencies should be corrected. Surgery may be required in childhood for approximately 50% of cases.

    • Nursing considerations: Promote fluid and food intake, monitor for complications such as perforation, sepsis, hemorrhage, and obstruction.

Celiac Disease

  • Definition: Gluten intolerance.

  • Assessment:

    • Flare-ups or celiac crises can be associated with events such as gluten ingestion, infections, prolonged fasting, or exposure to anticholinergic drugs.

    • Steatorrhea – greasy, bulky, and very malodorous stools that appear frothy and full of fat.

    • Presents with organic failure to thrive (FTT) – weight loss or inadequate gain, muscle wasting, and anemia. Anorexia and abdominal pain are also common.

    • Lab studies include IgG and IgA antibodies and a 72 hr fecal fat study, with biopsy of the jejunum showing villous atrophy.

  • Interventions:

    • Educate on the strict gluten-free diet.

    • Correct electrolyte disturbances

    • Restore fluids.

    • Nursing considerations: Reinforce dietary education, provide written instructions for the child and family.

Necrotizing Enterocolitis (NEC)

  • Occurs primarily in premature infants or sick full-term infants.

  • Varying degrees of dead or necrotic tissue in the transmural or mucosal segments of the intestines.

  • Assessment:

    • Monitor for three stages of enterocolitis.

      •   Stage 1: Marked by fluctuating temps, hypoglycemia, abdominal distention, heme-positive stools, poor perfusion, lethargy, and recurrent apnea/bradycardia

      • Stage 2: Marked by the items in stage 1 plus severe distention, grossly bloody stools, extreme abdominal tenderness, and absent bowel sounds

      • Stage 3: Marked by the evidence of the beginning of septic shock, including the deterioration of VS, metabolic acidosis, severe edema of the abdominal wall, and disseminated intravascular coagultaion (DIC)

  • Interventions:

    • Provide IV antibiotics, vascular support, and IV feedings.

    • Surgical ostomy may be required to remove dead tissue and allow bowel to rest and heal.

  • Nursing Considerations:

    • Recognized as a life-threatening disease with long healing time; parents need emotional support.

Enteral Feeding

  • Reasons to receive: Congenital intestinal function disorders, oral malformations, conditions that cause dysphagia, failure to thrive, neurological disorders that disrupt adequate calorie consumption.

  • Daily weight, hydration, adequate stooling, and tolerance of feedings will determine if the procedure is successful.

  • Means of administering:

    • Nasogastric tube

    • Gastrostomy tube

    • PEG tube (percutaneous endoscopic gastrostomy tube)

    • “Button” feeding tube.

Child with a Genitourinary Condition

Chapter 35

The Development of the Genitourinary System

  • Begins during the 11th & 12th week of fetal development.

  • By the 13th week: kidneys are producing urine.

  • Newborn: produces larger quantities of urine with lower specific gravity.

  • Newborn: kidneys are immature.

Renal Function

  • Functions of the kidneys:

    • Eliminate liquid waste products.

    • Produce erythropoietin.

    • Produce renin.

    • Regulate fluids and electrolytes.

    • Regulate acid/base balance.

  • Reasons why infants are at risk for dehydration:

    • Higher extracellular fluid percentage.

    • Higher turnover rate.

Fluid Maintenance Requirements

  • Water Intoxication:

    • Intake of large amounts of free water.

    • Associated with severe mental illness, low cognitive function, extreme thirst.

  • Urine Output:

    • Weight of diapers is measured. One gram of urine = 1 ml of urine.

    • Newborns produce approximately 10 ml/hour.

    • Infants produce approximately 5-10 ml/hour.

    • Toddler and preschool children produce approximately 15-20 ml/hour.

    • School-age children produce approximately 10-25 ml/hour.

    • Adolescents produce approximately 40-80 ml/hr.

Dehydration

  • Assessments:

    • Intake and output.

    • Specific gravity and urine color.

    • Skin turgor.

    • Mucous membranes.

    • Fontanels and eye sockets.

    • Skin color (pale), capillary refill (delayed), and blood pressure (low).

    • Body temperature, pulse, and respiratory rate.

    • Lethargy, weak cry, and poor muscle tone.

Dehydration (cont.)

  • Interventions:

    • Oral fluid replacement.

    • IV fluid boluses.

  • Nursing considerations:

    • Strict intake and output monitoring.

    • Weigh diapers regularly.

    • Mouth care is essential.

Urinary Tract Infections (UTIs)

  • One of the most common childhood infections.

  • Bladder= Cystitis

  • Urethra= Urethritis

  • Kidney= Pyelonephritis

  • Caused by urine refluxing back into the ureters during or after urination, incomplete bladder emptying, or inadequate hygiene of the perineum after stools.

  • Assessments:

    • Frequency, urgency, pain, odor and clarity of urine, fever, dehydration, hematuria, lethargy, and poor feeding, abdominal, pelvic, or flank pain, the number of UTIs in the past.

  • Interventions:

    • Encourage hydration.

    • Administer antibiotics (amoxicillin, ampicillin, cephalexin, gentamicin, sulfa).

  • Nursing considerations:

    • Prevention: Avoid irritating products, strong soaps, bath salts, and ensure to wipe front to back.

    • Health Promotion: Preventing UTIs and STIs in sexually active teens.

Enuresis

  • Nocturnal incontinence: involuntary urination at night after the child has been toilet trained. Most common form.

  • Diurnal incontinence: daytime incontinence and mostly associated with a condition called unstable bladder.

  • Assessments:

    • Medical exam and health history.

    • May be related to: unstable bladder, holding onto urine or infrequent daytime voiding, cystitis, neurogenic bladder, sphincter abnormality, trauma, and overflow incontinence.

  • Interventions:

    • Children less than 5 years: behavioral interventions, bedwetting alarms.

    • Children older: bladder training, and possibly medication.

    • There is a direct link between adenoidal hypertrophy and enuresis due to enlarged adenoids.

  • Nursing considerations: Educate parents about intervention strategies and expectations.

Nephrotic Syndrome/Nephrosis

  • Characterized by several symptoms associated with the development of pores along the filtration membrane of the kidney and loss of serum proteins.

  • Most cases are considered idiopathic. (meaning no cause is found)

  • Cardinal symptoms:

    • Severe proteinuria.

    • Severe hypoproteinemia: low levels of protein in blood leading to generalized edema.

    • Hyperlipidemia: elevated cholesterol, triglycerides, and total lipids.

  • Assessments: Fatigue, anorexia, edema, weight gain, golden-yellow foamy urine.

  • Interventions:

    • Diuretics, corticosteroids, IV albumin, low-salt, high-protein diet.

  • Nursing considerations: Provide emotional support and education about prognosis and treatment.

Acute Glomerulonephritis (AGN)

  • Most often follows a severe strep infection.

  • Assessments:

    • Tea colored urine, low urine output, fatigue, hypertension, edema, sore throat, rash on buttocks and legs, joint pain, headache, increased breathing effort, seizures.

  • Diagnostics: throat, blood, urine cultures, UA, chest x-ray, renal ultrasound, and renal biopsy.

  • Interventions:

    • Administer Diuretics, correct electrolyte imbalances, treat strep with antibiotics, treat hypertension, may require dialysis for short-term management of build-up of waste products in the blood.

  • Nursing considerations: Rapidly identify any history of infection and report to the healthcare provider.

Hemolytic Uremic Syndrome (HUS)

  • Rare and potentially lethal form of kidney failure in children who have developed a gastrointestinal infection such as E. coli.

  • More common in young children and closely associated with consumption of inadequately cooked meat, unpasteurized dairy products, or Juices, and exposure to contaminated swimming pools, water parks, and daycare facilities and fast-food restaurants.

  • Commonly seen in children ages 6 months – 5 years.

  • Assessments: vomiting, severe abdominal pain, watery or bloody diarrhea. Will appear pale, fatigued, irritable, and dehydrated. Small bruises visible and can have hypertension, edema, and edema leading to dialysis.

  • Interventions: PICU stay is often required; there is no cure, focus on treating symptoms.

  • Nursing considerations: Provide emotional support and answer caregiver questions.

Congenital Genitourinary Anomalies

  • The most common is Vesicoureteral Reflux (VUR) – urine flows back from the bladder into the ureters and often up to the kidneys during or after urination.

  • Exstrophy of the bladder: lower portion of abdominal wall and anterior bladder wall are absent.

  • Hypospadias: abnormal opening in the glans penis for elimination of urine.

  • Epispadias: meatus is located on the dorsum of the penis.

  • Neurogenic bladder: nerves between bladder and brain are damaged, resulting in the inability to regulate urine elimination.

  • Associated issues: urinary tract infections, kidney damage from recurrent reflux.

  • Nursing considerations: Surgical interventions should take place before psychosocial development is affected.

Reproductive Disorders

  • Genetic or congenital reproductive system disorders:

    • Intersex conditions - atypical physical sex characteristics.

    • Turner syndrome: loss of part or all of X chromosomes.

    • Klinefelter syndrome: presence of an extra X chromosome, producing a child who is male with delayed secondary sex development.

  • Acquired reproductive system disorders:

    • Foreign bodies in the vagina.

    • Pediatric vulvovaginitis.

    • Labial adhesions: scarring from surgical procedures, complications of female circumcisions, and scarring from chemical or thermal burns.

Sexually Transmitted Diseases (STIs)

  • Most common STIs found in childhood include:

    • Chlamydia

    • Gonorrhea

    • Genital warts

    • Genital herpes

  • Assessments: dysuria, lesions, odors, discharges, and discomfort.

    • Health history, sexual history, use of birth control should be evaluated.

  • Interventions: Administer antibiotics or antivirals as necessary.

  • Nursing considerations: Education on good perineal hygiene, condom use, and voiding before and after sex is pivotal.

Child with a Metabolic Condition

Chapter 32

Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

  • Overproduction and excretion of ADH from the posterior pituitary results in the kidneys absorbing more water, leading to decreased urine output and increased fluid retention.

  • Risks: shaken baby syndrome, head trauma, brain tumor, brain surgery, or infections of the brain.

  • Assessments: weakness, confusion, anorexia, fluid overload symptoms, decreased urine output.

  • Monitor urine specific gravity to assess fluid balance.

  • Interventions: fluid restriction, IV Lasix, and IV solution (isotonic or hypotonic).

  • Nursing considerations: distinguish SIADH from Diabetes Insipidus.

Deficient Anterior Pituitary Hormone: Pituitary Dwarfism

  • Occurs early in utero development when the fetus’ pituitary secretes too little growth hormone (GH).

  • Growth is typically less than 2 inches annually until preschool age, when growth is significantly delayed.

  • Assessments: Child appears younger than age; intelligence is typically normal. Lab evaluation for growth hormone deficiency is crucial.

  • Interventions: Human growth hormone injections are key to treatment.

  • Nursing considerations: Good oral evaluation because of jaw growth retardation; education and emotional support needed for parents and child to help cope with condition.

Hypersecretion of Anterior Pituitary Hormone: Gigantism (Acromegaly)

  • High linear bone growth results from excessive secretion of GH and insulin-like growth factor (IGF-1).

  • Assessments: mild to moderate obesity, visual changes, soft-tissue hypertrophy, peripheral neuropathies such as carpal tunnel, osteoarthritis, headaches, oily skin, and acne.

  • Interventions:

    • Lab studies to assess growth hormone levels.

    • MRI to assess the pituitary gland for tumors.

    • X-rays to assess severity of skeletal malformation.

  • Nursing considerations: psychological support to deal with altered body image is essential.

Hypothyroidism

  • Risk: diet or drinking water deficient of iodine.

  • Assessments: enlarged thyroid, short stature, hypotonia. In acquired hypothyroidism, assess for goiter. Other symptoms include slow growth, weight gain, decreased activity, constipation, tiredness, and delayed mental processing.

  • Diagnostics: Thyroxine (T4) screening; if decreased, then TSH is drawn.

  • Interventions: Thyroxine and thyroid hormone replacement are critical.

  • Nursing considerations: Education about the importance of medication compliance, regular check-ups, and lab draws.

Hyperthyroidism

  • Grave's Disease: autoimmune destruction of the thyroid gland. Often in adolescent females, but may occur in younger children if family history prevails.

  • Signs: increased production of T4, enlargement of the thyroid gland, ocular changes such as protrusion, staring, and eyelid lag.

  • Assessments: tremor, muscle weakness, nervousness, irritability, fatigue, weight loss, diarrhea, excessive perspiration, exophthalmos.

  • Diagnostics: T3 and T4 screening is essential.

  • Interventions: Medications to limit thyroid hormone secretion.

  • Nursing considerations: Educate family that the child may experience school problems and that medication therapy is likely lifelong. Monitor weight and provide high-protein, high-calorie meals.

Hyperfunction of the Adrenal Gland: Cushing’s Syndrome

  • Occurs when there is an overproduction and a hypersecretion of corticosteroid hormones, leading to prolonged exposure to steroids.

  • Often associated with tumors in adrenal gland or with excessive or prolonged use of corticosteroids.

  • Assessments: central obesity, decreased glucose tolerance, poor wound healing, muscle weakness and atrophy, easy bruising, osteoporosis, acne, hypertension, mood disorders, hirsutism.

  • Interventions: taper corticosteroids, or surgical intervention to remove tumor if present.

  • Nursing considerations: Monitor for withdrawal symptoms, educate that mood swings may last weeks to months after treatment is discontinued.

Type 1 Diabetes Mellitus

  • Previously called: Insulin-dependent diabetes.

  • Often first presents with diabetic ketoacidosis (DKA).

  • Assessments:

    • Polyphagia: excessive hunger.

    • Polydipsia: excessive thirst.

    • Polyuria: excessive urination.

  • Interventions: glycemic control through insulin therapy, education on carbohydrate counting, monitoring HbA1c levels, and patient education on disease management.

Type 2 Diabetes Mellitus

  • Non-insulin-dependent diabetes.

  • Typically seen in obese and older children.

  • Assessments: hyperglycemia, darkened and thickened pigmentation around the base of the neck, hypertension, sleep apnea, hyperlipidemia.

  • Interventions: lifestyle changes such as exercise, dietary adjustments for weight loss, oral hypoglycemic medications, and insulin if not controlled.

  • Nursing considerations: education aimed at preventing progression to type 1 diabetes and preventing hyperglycemia.

Inborn Errors of Metabolism (IEM)

  • Phenylketonuria (PKU): inability to metabolize phenylalanine. Commonly noted with blonde hair and blue eyes.

    • Assessments: musty-smelling urine, poor weight gain, delayed growth.

    • Interventions: special formula, avoid food containing phenylalanine such as eggs, flour, fish, legumes, nuts, bread, cheese, poultry, and meats.

  • Galactosemia: deficiency of the galactose enzyme.

    • Assessments: feeding difficulties, lethargy, poor weight gain, jaundice, and bleeding.

    • Interventions: Use soy-based, lactose-free, or meat-based formula such as Nutramigen.

  • Maple Syrup Urine Disease (MSUD): deficiency of decarboxylase enzyme which breaks down branched-chain amino acids.

    • Assessments: maple syrup odor to urine, vomiting, poor feeding, lethargy, seizures, developmental delay, and may lead to coma if untreated.

    • Interventions: restrict branched-chain amino acids in high-protein food.