Description

Acute respiratory distress syndrome (ARDS) is a complex respiratory illness characterized by respiratory distress and hypoxemia that occur within 72 hours of a serious injury or surgery in a person with previously normal lungs. ARDS may occur as a result of clinical conditions and injuries such as sepsis, trauma, viral pneumonia, fat emboli, drug overdose, reperfusion injury after lung transplantation, smoke inhalation, and near-drowning.

Pathophysiology

The hallmark of ARDS is increased permeability of the alveolarcapillary membrane that results in pulmonary edema. During the acute phase of ARDS, the alveolocapillary membrane is damaged, with an increasing pulmonary capillary permeability and resulting interstitial edema. When fibrosis occurs, the child may demonstrate respiratory distress and the need for mechanical ventilation. The lungs become stiff as a result of surfactant inactivation, gas diffusion is impaired, and eventually bronchiolar mucosal swelling and congestive atelectasis occur. The net effect is decreased functional residual capacity, pulmonary hypertension, and increased intrapulmonary right-to-left shunting of pulmonary blood flow. Surfactant secretion is reduced, and the atelectasis and fluid-filled alveoli provide an excellent medium for bacterial growth. Hypoxemia is expressed as the partial pressure of oxygen (PaO₂) to fraction of inspired oxygen (FiO₂) ratio, or P/F ratio; the P/F ratio for ARDS is 200.

Clinical Signs and Symptoms

The child with ARDS may first demonstrate only symptoms caused by an injury or infection, but, as the condition deteriorates, hyperventilation, tachypnea, increasing respiratory effort, cyanosis, and decreasing oxygen saturation occur. At times the developing hypoxemia is not responsive to oxygen administration.

Diagnostic Evaluation

The criteria for diagnosis of ARDS in children are an acute antecedent illness or injury, acute respiratory distress or failure, no evidence of prior cardiopulmonary disease, and diffuse bilateral infiltrates evidenced on chest radiography.

Treatment

Treatment involves supportive measures, such as:

Nursing Care Management

Patient and Family Teaching

Description

Congenital adrenal hyperplasia (CAH) is a family of disorders caused by decreased enzyme activity required for cortisol production in the adrenal cortex. The most common defect is 21-hydroxylase deficiency, which constitutes more than 90% of all cases of CAH.

Pathophysiology

Interference in the biosynthesis of cortisol during fetal life results in an increased production of ACTH, which stimulates hyperplasia of the adrenal gland. Depending on the enzymatic defect, increased quantities of cortisol precursors and androgens are secreted. There are six major types of biochemical defects. The most common is partial or complete 21-hydroxylase deficiency. With partial deficiency, enough aldosterone is produced to preserve sodium, and adequate cortisol is produced to prevent signs of adrenocortical insufficiency.

Clinical Signs and Symptoms

Diagnostic Evaluation

Clinical diagnosis is initially based on congenital abnormalities that lead to difficulty in assigning gender to the newborn and on signs and symptoms of adrenal insufficiency or hypertension.

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Allergic rhinitis affects many children and is associated with numerous airway disorders, including asthma, OME, and chronic sinusitis. Seasonal allergic rhinitis (or hay fever) usually follows a spring-fall pattern and is caused by tree, grass, and weed pollens. Seasonal allergic rhinitis usually does not develop until the individual has been sensitized by two or more pollen seasons. Peak incidence for allergic rhinitis is in the adolescent and postadolescent age groups, but younger children are also affected.

Pathophysiology

Allergic rhinitis requires two conditions: a familial predisposition to develop allergy and exposure of a sensitized person to the allergen. Inhalants in the form of microscopic airborne particles (e.g., pollens, mold, animal danders, and environmental dusts) enter the upper respiratory tract with inhalation and bind to submucosal mast cells in the respiratory tract epithelium. In the allergic child, symptoms are mediated by immunoglobulin E (IgE), which is produced by the child’s B lymphocytes. The IgE molecules on the cell surfaces trigger the rapid release of mast cell mediators (e.g., histamine, prostaglandins, and leukotrienes), as well as cell interactive compounds called cytokines. Histamine, a potent vasodilator, acts directly on local receptors to produce vasodilation, mucosal edema, and increased production of mucus. The cytokines summon cells to the area and are responsible for the slower late-phase allergic reaction of inflammation and destruction of the mucosal surface that progresses to chronic nasal obstruction. Repeated exposure of these sensitized membranes to specific aeroallergens results in clinical allergic disease.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Recognition of symptoms and referral for further evaluation

Preparation for administration of skin (allergen) and immunotherapy

To distinguish allergies from colds, be aware that:

Allergies occur repeatedly and are often seasonal.

Allergies are seldom accompanied by fever.

Allergies often involve itching in the eyes and nose.

Allergies usually trigger constant and consistent bouts of sneezing.

Allergies are often accompanied by ear and eye problems.

Patient and Family Teaching

Description

Anemia caused by an inadequate supply of dietary iron is the most prevalent nutritional disorder in the United States and the most common mineral disturbance. Children 12 to 36 months of age are at risk for anemia as a result of cow’s milk being a major staple of many infants’ diet. Preterm infants are especially at risk because of their reduced fetal iron supply. Adolescents are also at risk because of their rapid growth rate combined with poor eating habits.

Pathophysiology

Iron-deficiency anemia can be caused by any number of factors that decrease the supply of iron, impair its absorption, increase the body’s need for iron, or affect the synthesis of Hgb.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

A primary nursing objective is to prevent nutritional anemia through family education. Because breast milk is a poor iron source after 5 months of lactation, reinforce the importance of administering iron supplementation in the exclusively breast-fed infant by 6 months of age.

In the formula-fed infant, discuss with parents the importance of using iron-fortified formula and the introduction of solid foods at the appropriate age during the first year of life. Cereals are one of the first semisolid foods to be introduced into the infant’s diet at approximately 6 months of age.

Diet education of teenagers is especially difficult, especially because teenage girls are particularly prone to following weight-reduction diets. Emphasizing the effect of anemia on appearance (pallor) and energy level (difficulty maintaining popular activities) may be useful.

Discuss side-effects of iron therapy:

An adequate dosage of oral iron turns the stools a tarry green color.

Vomiting or diarrhea can occur with iron therapy. If the parents report these symptoms, the iron can be given with meals and the dosage reduced and then gradually increased until tolerated.

Liquid preparations of iron may temporarily stain the teeth. If possible, the medication should be taken through a straw or given through a syringe or medicine dropper placed toward the back of the mouth. Brushing the teeth after administration of the drug lessens the discoloration.

Description

Anorexia nervosa (AN) is an eating disorder characterized by a refusal to maintain a minimally normal body weight and by severe weight loss in the absence of obvious physical causes.

Pathophysiology

The disorder appears to be caused by a combination of genetic, neurochemical, psychodevelopmental, and sociocultural factors. The dominant aspects of AN are a relentless pursuit of thinness and a fear of fatness, usually preceded by a period of mood disturbances and behavior changes.

Clinical Signs and Symptoms

Diagnostic Evaluation

Diagnosis is made on the basis of criteria established by the American Psychiatric Association (see Box 17-16 in text book).

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Narrowing or stricture of the aortic valve, causing resistance to blood flow from the left ventricle, decreased cardiac output, left ventricular hypertrophy, and pulmonary vascular congestion. The prominent anatomic consequence of aortic stenosis (AS) is the hypertrophy of the left ventricular wall, which eventually leads to increased end-diastolic pressure resulting in pulmonary venous and pulmonary arterial hypertension. Left ventricular hypertrophy also interferes with coronary artery perfusion and may result in myocardial infarction or scarring of the papillary muscles of the left ventricle, which causes mitral insufficiency. Valvular stenosis, the most common type, is usually caused by malformed cusps that result in a bicuspid rather than tricuspid valve or fusion of the cusps. Subvalvular stenosis is a stricture caused by a fibrous ring below a normal valve; supravalvular stenosis occurs infrequently. Valvular AS is a serious defect for the following reasons: (1) the obstruction tends to be progressive; (2) sudden episodes of myocardial ischemia, or low cardiac output, can result in sudden death; and (3) surgical repair rarely results in a normal valve. This is one of the rare instances in which strenuous physical activity may be curtailed because of the cardiac condition.

Pathophysiology

A stricture in the aortic outflow tract causes resistance to ejection of blood from the left ventricle. The extra workload on the left ventricle causes hypertrophy. If left ventricular failure develops, left atrial pressure will increase; this causes increased pressure in the pulmonary veins, which results in pulmonary vascular congestion (pulmonary edema).

Clinical Signs and Symptoms

Newborns with critical AS demonstrate signs of decreased cardiac output with faint pulses, hypotension, tachycardia, and poor feeding (see Box 3-1, Common Signs and Symptoms of Congestive Heart Failure). Children show signs of exercise intolerance, chest pain, and dizziness when standing for a long period. There is a characteristic murmur that is low-pitched, harsh, and rasping, heard loudest at the base in the second intercostal space. Patients are at risk for bacterial endocarditis, coronary insufficiency, and ventricular dysfunction.

Diagnostic Evaluation

See Table 3-1, Procedures for Cardiac Diagnosis; and Table 3-2, Current Interventional Cardiac Catheterization Procedures in Children.

TABLE 3-1 Procedures for Cardiac Diagnosis

TABLE 3-2 Current Interventional Cardiac Catheterization Procedures in Children

Valvular Aortic Stenosis

Subvalvular Aortic Stenosis

Assist in Measures to Assess and Improve Cardiac Function

Monitor Cardiac Function

Vital signs monitoring

Respiratory assessment

Cardiac assessment

Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function)

Heart sounds

Dysrhythmias

Keep accurate record of intake and output.

Weigh child or infant on same scale at same time of day.

Manage Common Side Effects of Cardiac Defects

Provide Support for the Child and Family

Prepare the Child and Family for Diagnostic and Operative Procedures

Educate the Child and Family

Assess child’s and family’s level of knowledge regarding aortic stenosis.

Assess child’s and family’s understanding of what they have heard about aortic stenosis.

Assess child’s and family’s understanding of the surgery and/or cardiac catheterization.

Teach the child and/or family at least four characteristics of congestive heart failure that may occur because of the aortic stenosis such as:

Fatigue after exertion

Fast heart rate

Fast breathing, shortness of breath, dyspnea

Retractions

Nasal flaring

Cool extremities

Diaphoresis

Decreased urinary output

Puffiness (edema)

Fussiness

Decreased appetite

Educate child and family about care such as medication preparation and administration.

Assess child’s and family’s access to appropriate pharmacy for any special preparation medications.

If the child is on anticoagulation therapy, educate the child and family about:

Anticoagulation therapy

Dietary restrictions

Laboratory testing required

The need for close medical follow-up

Signs and symptoms of complications from blood clots and bleeding

Educate child and family about when to notify their physician of any clinical changes.

Provide appropriate educational resources and review materials with them.

Clarify information presented by the health care team including appointment for follow-up visit.

Discuss SBE (subacute bacterial endocarditis) prophylaxis with the pediatric cardiologist.

Description

Aplastic anemia (AA) refers to a condition in which the formed elements of the blood are simultaneously depressed. The peripheral blood smear demonstrates pancytopenia or the triad of profound anemia, leukopenia, and thrombocytopenia. Aplastic anemia can be primary (congenital, or present at birth) or secondary (acquired). The best-known congenital disorder of which aplastic anemia is an outstanding feature is Fanconi syndrome, a rare hereditary disorder characterized by pancytopenia, hypoplasia of the bone marrow, and patchy brown discoloration of the skin resulting from the deposit of melanin and associated with multiple congenital anomalies of the musculoskeletal and genitourinary systems. The syndrome appears to be inherited as an autosomal recessive trait with varying penetrance; therefore, affected siblings may demonstrate several different combinations of defects.

Pathophysiology

Most of the cases of AA are considered idiopathic where no cause is found. Several etiologic factors contribute to the development of acquired hypoplastic anemia:

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Therapy is directed at restoring function to the marrow and involves two main approaches:

Immunosuppressive therapy to remove the presumed immunologic functions that prolong aplasia. Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG) is the principal drug treatment used for aplastic anemia. Cyclosporine may also be used. ATG and cyclosporine suppress T cell–dependent autoimmune responses but do not cause bone marrow suppression.

Replacement of the bone marrow through transplantation. Bone marrow transplantation is the treatment of choice for severe aplastic anemia when a suitable donor exists. Hematopoietic stem cell transplantation should be considered early in the course of the disease if a compatible donor can be found.

Colony-stimulating factor (CSF), and granulocyte-macrophage colony-stimulating factor (GM-CSF) given parenterally, may be used to enhance bone marrow production.

Androgens also may be used with ATG to stimulate erythropoiesis if the AA is nonresponsive to initial therapies.

Nursing Care Management

Patient and Family Teaching

Description

Appendicitis is an inflammation of the vermiform appendix (blind sac at the end of the cecum); appendicitis is the most common cause of emergency abdominal surgery in childhood.

Pathophysiology

Appendicitis may be caused by obstruction of the lumen of the appendix by hardened fecal material (fecalith); swollen lymphoid tissue, occurring after a viral infection; or a parasite such as Enterobius vermicularis or pinworms, that can obstruct the appendiceal lumen.

With acute obstruction, the outflow of mucus secretion is blocked and pressure builds within the lumen, resulting in compression of blood vessels. The resulting ischemia is followed by ulceration of the epithelial lining and bacterial invasion. Subsequent necrosis causes perforation or rupture with fecal and bacterial contamination of the peritoneal cavity. The resulting inflammation spreads rapidly throughout the abdomen (peritonitis), especially in young children, who are unable to localize infection.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Juvenile idiopathic arthritis (JIA) is a chronic autoimmune inflammatory disease causing inflammation of joints and other tissue with an unknown cause. JIA starts before age 16 with peak onset between 1 and 3 years of age.

Pathophysiology

A popular causal theory is an infectious or environmental agent, which triggers an abnormal inflammatory response in a genetically predisposed child, resulting in chronic arthritis, but there is no substantiating evidence. The disease process is characterized by chronic inflammation of the synovium with joint effusion and eventual erosion, destruction, and fibrosis of the articular cartilage. Adhesions between joint surfaces and ankylosis of joints occur if the inflammatory process persists.

Clinical Signs and Symptoms

Diagnostic Evaluation

JIA is a diagnosis of exclusion; there are no definitive tests. Classifications are based on the clinical criteria of age of onset before 16 years, arthritis in one or more joints for 6 weeks or longer, and exclusion of other etiologies. Additional diagnostic tests may include:

Treatment

There is no cure for JIA. The major goals of therapy are to:

Nursing Care Management

Patient and Family Teaching

Description

Asthma is a chronic inflammatory disorder of the airways characterized by recurrent episodes of wheezing, breathlessness, chest tightness, and cough.

Pathophysiology

Inflammation contributes to heightened airway reactivity in asthma. The mechanisms contributing to airway inflammation are multiple and involve a number of different pathways. Another important component of asthma is bronchospasm and obstruction. The mechanisms responsible for the obstructive symptoms in asthma include: (1) inflammation and edema of the mucous membranes, (2) accumulation of tenacious secretions from mucous glands, and (3) spasm of the smooth muscle of the bronchi and bronchioles, which decreases the caliber of the bronchioles. Bronchial constriction is a normal reaction to foreign stimuli, but in the child with asthma it is abnormally severe, producing impaired respiratory function. The smooth muscle arranged in spiral bundles around the airway causes narrowing and shortening of the airway, which significantly increases airway resistance to airflow. Because the bronchi normally dilate and elongate during inspiration and contract and shorten on expiration, the respiratory difficulty is more pronounced during the expiratory phase of respiration.

Increased resistance in the airway causes forced expiration through the narrowed lumen. The volume of air trapped in the lungs increases as airways are functionally closed at a point between the alveoli and the lobar bronchi. This trapping of gas forces the individual to breathe at higher and higher lung volumes. Consequently, the person with asthma fights to inspire sufficient air. This expenditure of effort for breathing causes fatigue, decreased respiratory effectiveness, and increased oxygen consumption. The inspiration occurring at higher lung volumes hyperinflates the alveoli and reduces the effectiveness of the cough. As the severity of obstruction increases, there is a reduced alveolar ventilation with carbon dioxide retention, hypoxemia, respiratory acidosis, and, eventually, respiratory failure.

Clinical Signs and Symptoms

Diagnostic Evaluation

Asthma is classified into four categories based on the symptom indicators of disease severity. These categories are:

Additional diagnostic measures may include:

Treatment

The overall goals of asthma management are to:

These may be accomplished with the following:

Nursing Care Management

Patient and Family Teaching

Description

Eczema or eczematous inflammation of the skin refers to a descriptive category of dermatologic diseases and not to a specific etiology. AD is a type of pruritic eczema that usually begins during infancy and is associated with allergy with a hereditary tendency (atopy). Three forms of AD are recognized: infantile (infantile eczema), childhood, and preadolescent and adolescent. The majority of children with infantile AD have a family history of eczema, asthma, food allergies, or allergic rhinitis, which strongly supports a genetic predisposition; many children with AD will develop asthma.

Pathophysiology

The cause of AD is unknown but may be related to an immune reaction with abnormal function of the skin, including alterations in perspiration, peripheral vascular function, and heat intolerance. Patients with AD have dry skin and evidence of increased transepidermal water loss; a defect in the ceramide cells, which help retain water and provide a barrier function; and increased colonization of the skin with Staphylococcus aureus. House dust mites, certain foods, mold, and animal hair may play a role in the etiology of AD. Many children with AD have elevated toxic-specific IgE levels. IgE food sensitization has been found to be a major risk factor for infantile AD.

Clinical Signs and Symptoms

Appearance of Lesions

Infantile form

Erythema

Vesicles

Papules

Weeping

Oozing

Crusting

Scaling

Often symmetric

Childhood form

Symmetric involvement

Clusters of small erythematous or flesh-colored papules or minimally scaling patches

Dry; may be hyperpigmented

Lichenification (thickened skin with accentuation of creases)

Keratosis pilaris (follicular hyperkeratosis) common

Adolescent and Adult Form

Same as childhood manifestations

Dry, thick lesions (lichenified plaques) common

Confluent papules

Other Manifestations

Intense itching

Unaffected skin dry and rough

African-American children likely to exhibit more papular or follicular lesions than Caucasian children

May exhibit one or more of the following:

Lymphadenopathy, especially near affected sites

Increased palmar creases (many cases)

Atopic pleats (extra line or groove of lower eyelid)

Tendency toward cold hands

Pityriasis alba (small, poorly defined areas of hypopigmentation)

Facial pallor (especially around nose, mouth, and ears)

Bluish discoloration beneath eyes (“allergic shiners”)

Increased susceptibility to unusual cutaneous infections (especially viral)

Diagnostic Evaluation

Diagnosis is based on a combination of history and morphologic findings (see Clinical Signs and Symptoms on the previous page).

Treatment

The major goals of management are to (1) hydrate the skin, (2) relieve pruritus, (3) reduce flare-ups or inflammation, and (4) prevent and control infection.

Nursing Care Management

Patient and Family Teaching

Description

Abnormal opening between the atria, allowing blood from the higher-pressure left atrium to flow into the lower-pressure right atrium. There are three types of atrial septal defect (ASD):

Pathophysiology

Because left atrial pressure slightly exceeds right atrial pressure, blood flows from the left to the right atrium, causing an increased flow of oxygenated blood into the right side of the heart. Despite the low pressure difference, a high rate of flow can still occur because of low pulmonary vascular resistance and the greater distensibility of the right atrium, which further reduces flow resistance. This volume is well tolerated by the right ventricle because it is delivered under much lower pressure than with a ventricular septal defect. Although there is right atrial and ventricular enlargement, cardiac failure is unusual in an uncomplicated ASD. Pulmonary vascular changes usually occur only after several decades if the defect is left unrepaired.

Clinical Signs and Symptoms

Patients are typically asymptomatic, although they may demonstrate mild exercise intolerance or frequent upper respiratory infections. Development of congestive heart failure is rare in the pediatric population with ASD but becomes more prevalent in adults (see Box 3-1). The physical exam is mostly unremarkable. There may be a systolic ejection murmur through the pulmonary valve, but the most significant finding upon auscultation is a fixed splitting of the second heart sound. Patients are at risk for atrial dysrhythmias (probably caused by atrial enlargement and stretching of conduction fibers) and pulmonary vascular obstructive disease and emboli formation later in life from chronically increased pulmonary blood flow.

Diagnostic Evaluation

Nursing Care Management

Assist in Measures to Assess and Improve Cardiac Function

Monitor Cardiac Function

Vital signs monitoring

Respiratory assessment

Cardiac assessment

Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function)

Heart sounds

Dysrhythmias

Keep accurate record of intake and output.

Weigh child or infant on same scale at same time of day.

Monitor for signs and symptoms of postpericardiotomy syndrome following surgery:

Fever

Malaise

Nausea

Vomiting

Abdominal pain

Friction rub may be auscultated.

Administer Medications

Review child’s history related to cardiac management.

Administer medications on schedule.

Assess effectiveness of medications and any side effects noted.

Be aware of drug-drug and drug-food interactions.

Follow guidelines for medication administration.

Manage Common Side Effects of Cardiac Defects

Prevent fatigue during feeding; offer small frequent feedings to infant’s or child’s tolerance.

Organize nursing care to allow child/infant uninterrupted rest.

Promote activities that do not cause fatigue.

Provide Support for the Child and Family

Communicate with the child and family in an appropriate style.

Provide the child and family with honest answers.

Provide emotional support.

Offer age-appropriate interventions.

Provide information on available resources for support for the child and family.

Patient and Family Teaching

Educate the Child and Family

Assess child’s and family’s level of knowledge regarding the ASD.

Assess child’s and family’s understanding of what they have heard about ASD.

Assess child’s and family’s understanding of the surgery and/or cardiac catheterization.

Teach the child and/or family at least four characteristics of congestive heart failure that may occur because of the ASD such as:

Fatigue after exertion

Fast heart rate

Fast breathing, shortness of breath, dyspnea

Retractions

Nasal flaring

Cool extremities

Diaphoresis

Decreased urinary output

Puffiness (edema)

Fussiness

Decreased appetite

Educate child and family about care such as medication preparation and administration.

Assess child’s and family’s access to appropriate pharmacy for any special preparation medications.

Teach the child and family to notify their physician of any clinical changes.

Assess and record results and family’s participation in care.

Provide appropriate educational resources and review materials with them.

Clarify information presented by the health care team including appointment for follow-up visit.

Discuss SBE (subacute bacterial endocarditis) prophylaxis with pediatric cardiologist.

Description

Attention deficit hyperactivity disorder (ADHD) refers to developmentally inappropriate degrees of inattention, impulsiveness, and hyperactivity. To be diagnosed as ADHD, the symptoms must have been present before age 7 years and must be present in at least two settings.

Pathophysiology

While the cause of ADHD is unknown, there is some support for a genetic origin. Psychologic adversity and perinatal brain insults are also being considered.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Autism spectrum disorders (ASD) are complex neurodevelopmental disorders of brain function accompanied by intellectual and social behavioral deficits.

Pathophysiology

ASD is now recognized as a genetic disorder of prenatal and postnatal brain development. Immune and environmental factors (e.g., viral infections) may interact with the genetic susceptibility to increase the development of ASD.

Clinical Signs and Symptoms

Children with ASD demonstrate several peculiar and often seemingly bizarre characteristics, primarily in social interactions, communication, and behavior.

Inability to maintain eye contact with another person

Avoidance of body contact, and language delay at a very early age

Limited functional play; may interact with toys in an unusual or odd manner

ASD children may have significant gastrointestinal symptoms. Constipation is a common symptom and can be associated with acquired megarectum in children with ASD.

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Biliary atresia (BA) is a destructive, idiopathic, inflammatory process that leads to fibrosis and obliteration of the biliary tree. Untreated biliary atresia results in progressive cirrhosis and death in most children by 2 years of age.

Pathophysiology

The exact cause of BA is unknown. BA has two distinct forms, postnatal and fetal/embryonic, and different pathogenic mechanisms are suggested for each. Postnatal BA is probably the result of infection or an immune-mediated mechanism. An abnormal direct bilirubin has been designated as ≥1.0 mg/dL if the total bilirubin is ≤5 mg/dL or a value of direct bilirubin that represents more than 20% of the total bilirubin if it is ≥5 mg/dL. Direct hyperbilirubinemia first appears after the resolution of physiologic jaundice of the newborn. Histology demonstrates bile duct remnants and a progressive inflammatory process. In the fetal embryonic form of BA, there is a congenital absence of biliary ductal patency and an absence of bile duct remnants; many infants have associated congenital anomalies. Varying degrees of cholestasis occur, resulting in retention of irritants and toxins. Cholestasis is the accumulation of compounds that cannot be excreted because of occlusion or obstruction of the biliary tree. Injury to the liver occurs as the result of the inflammation caused by the cholestasis.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Infant botulism is an acute flaccid paralysis caused by the preformed toxin produced by the anaerobic bacillus Clostridium botulinum.

Pathophysiology

Infant botulism occurs primarily in infants less than 6 months of age when spores or vegetative cells of C. botulinum are ingested with the subsequent release of the toxin from organisms colonizing the gastrointestinal tract. The symmetric descending paralysis associated with botulism occurs as a result of blockade of the neurotransmitter at the autonomic neuromuscular and voluntary motor junctions. C. botulinum, types A and B, are the most common causative strains of infant botulism. Common sources of botulism spores are soil and honey. Nonhuman milk substances are also believed to be the source of infant botulism spores.

Clinical Signs and Symptoms

Diagnostic Evaluation

Diagnosis is made on the basis of the clinical history, physical examination, and laboratory detection of the organism in the patient’s stool, and less commonly in the blood. EMG may be helpful in establishing the diagnosis; however, results may be normal early in the course of the illness.

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Bronchiolitis is an acute viral infection with maximum effect at the bronchiolar level. The infection occurs primarily in winter and spring, and it is the most frequent cause of hospitalization in children less than 1 year old. Although most cases of bronchiolitis are caused by RSV, adenoviruses, parainfluenza viruses are also implicated; recently, human metapneumovirus has also been associated with bronchiolitis in children.

Pathophysiology

The causative virus affects the epithelial cells of the respiratory tract. The ciliated cells swell, protrude into the lumen, and lose their cilia. RSV produces a fusion of the infected cell membrane with cell membranes of adjacent epithelial cells, thus forming a giant cell with multiple nuclei. At the cellular level, this fusion results in multinucleated masses of protoplasm, or syncytia.

The bronchiolar mucosa swells, and lumina are subsequently filled with mucus and exudate. The walls of the bronchi and bronchioles are infiltrated with inflammatory cells, and peribronchiolar interstitial pneumonitis is usually present. Because luminal epithelial cells are shed into the bronchioles when they die, the lumina are frequently obstructed, particularly on expiration. The varying degrees of obstruction produced in small air passages lead to hyperinflation, obstructive emphysema resulting from partial obstruction, and patchy areas of atelectasis. Dilation of bronchial passages on inspiration allows sufficient space for intake of air, but narrowing of the passages on expiration prevents air from leaving the lungs. Thus air is trapped distal to the obstruction and causes progressive overinflation (emphysema).

Clinical Signs and Symptoms

Diagnostic Evaluation

Rapid immunofluorescent antibody–direct fluorescent antibody staining (DFA), or enzyme-linked immunosorbent assay (ELISA) techniques for RSV antigen detection, which are performed on nasal secretions

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Inflammation of the large airways: trachea and bronchi often in association with an upper respiratory infection

Pathophysiology

Viral agents and Mycoplasma pneumoniae are often the primary cause of the disease. The causative agents are inhaled into the upper airway and cause a local inflammatory response in the upper airway, producing the signs and symptoms.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

β-Thalassemia is the most common of the thalassemias and occurs in four forms: two heterozygous forms: thalassemia minor, an asymptomatic silent carrier, and thalassemia trait, which produces a mild microcytic anemia; thalassemia intermedia, which is manifested as splenomegaly and moderate to severe anemia; and a homozygous form, thalassemia major (also known as Cooley anemia), which results in a severe anemia that would lead to cardiac failure and death in early childhood without transfusion support.

Pathophysiology

Normal postnatal Hgb is composed of 2 α- and 2 β-polypeptide chains. In β-thalassemia there is a partial or complete deficiency in the synthesis of the β-chain of the Hgb molecule. Consequently, there is a compensatory increase in the synthesis of α-chains, and γ-chain production remains activated, resulting in defective Hgb formation. This unbalanced polypeptide unit is very unstable; when it disintegrates, it damages RBCs, causing severe anemia. To compensate for the hemolytic process, an overabundance of erythrocytes is formed unless the bone marrow is suppressed by transfusion therapy. Excess iron from hemolysis of supplemental RBCs in transfusions and from the rapid destruction of defective cells is stored in various organs).

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Bulimia is an eating disorder characterized by repeated episodes of binge eating followed by inappropriate compensatory behaviors, such as self-induced vomiting; misuse of laxatives, diuretics, or other medications; fasting; or excessive exercise. The binge behavior consists of secretive, frenzied consumption of large amounts of high-calorie (or “forbidden”) foods during a brief time (usually less than 2 hours). The binge is counteracted by a variety of weight control methods (purging), including self-induced vomiting, diuretic and laxative abuse, and rigorous exercise. These binge-purge cycles are followed by self-deprecating thoughts, a depressed mood, and an awareness that the eating pattern is abnormal.

Pathophysiology

The etiology and pathophysiology of the disorder is unclear. It appears to be caused by a combination of genetic, neurochemical, psychodevelopmental, and sociocultural factors. Patients with eating disorders commonly have psychiatric problems, including affective disorder, anxiety disorder, obsessive-compulsive disorder, and personality disorder. In addition, many experts associate the development of an eating disorder with family characteristics such as an adolescent perception of high parental expectations for achievement and appearance, difficulty managing conflict and poor communication styles, enmeshment and occasionally estrangement between family members, devaluation of the mother or the maternal role, and marital tension. Bulimia is observed more commonly in older adolescent girls and young women; males with bulimia are less common. BN patients may be of average or slightly above average weight.

Clinical Signs and Symptoms

The following may be present and suggest the need for hospitalization (i.e., not all patients with bulimia will manifest the following):

Diagnostic Evaluation

The diagnosis is based on the American Psychiatric Association diagnostic criteria listed in Box 17-7 of the textbook. The diagnosis is confirmed by at least two binge-eating episodes per week for the preceding 3 months.

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Celiac disease, also known as gluten-induced enteropathy, gluten-sensitive enteropathy, and celiac sprue, is an immune-mediated enteropathy of the proximal small intestine triggered by inappropriate immune response to ingested gluten and gluten-related proteins found in wheat, rye, and barley.

Pathophysiology

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 DQ8, are found in almost 100% of those affected with celiac disease. Once 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.

Clinical Signs and Symptoms

Diagnostic Evaluation

Diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of mucosal inflammation, crypt hyperplasia, and villous atrophy.

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Cerebral palsy (CP) is defined as a group of permanent motor syndromes that occur as a result of disorders of early brain development. The condition involves motor disorders; disturbances of sensation, perception, communication, cognition, and behavior; secondary musculoskeletal problems; and epilepsy. The primary disturbance includes abnormal muscle tone and coordination. The majority of children with CP, however, do not have significant cognitive dysfunction.

Pathophysiology

The exact mechanism that causes the neurologic brain lesions of CP is often not identified. Some children with CP have congenital malformations of the brain; others may have evidence of vascular occlusion, atrophy, loss of neurons, and degeneration. Hypoxic infarction or hemorrhage adjacent to the lateral ventricles is often identified as a cause of CP. Ataxic CP may occur in relation to cerebral hypoplasia and, in some cases, severe hypoglycemia. Additional antenatal factors that contribute to CP include maternal chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation, maternal sepsis, elevated maternal temperature prior to delivery, and urinary tract infection; however, not all infants born to mothers with these clinical signs will develop CP. Preterm birth, low birthweight (less than 1000 grams), and the occurrence of intracerebral hemorrhage and periventricular leukomalacia are associated with an increased incidence of CP.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Early recognition and promotion of optimal development to enable affected children to attain normalization and their potential within the limits of their existing health problems

Five broad aims of therapy

Establish locomotion, communication, and self-help.

Gain optimal appearance and integration of motor functions.

Correct associated defects as effectively as possible.

Provide educational opportunities adapted to the individual child’s needs and capabilities.

Promote socialization experiences with other affected and unaffected children.

Mobilization devices; AFOs (ankle foot othroses); wheelchairs, special walkers

Orthopedic surgery to correct contracture or spastic deformities

Medications to decrease spasticity: Dantrolene, Baclofen, dilantin; Botulinum toxin A (Botox)

AED medications: carbamazepime and valproic acid

Dental hygiene

Technical aides such as voice synthesizers

Early intervention education

Speech therapy and physical therapy

Nutrition: may require special feeding techniques for oral motor skill; enteral feeding in severe cases.

Nursing Care Management

Patient and Family Teaching

Description

Cirrhosis occurs at the end stage of many chronic liver diseases, including biliary atresia and chronic hepatitis.

Pathophysiology

Cirrhosis can also result from infectious, autoimmune, or toxic factors and from chronic diseases such as hemophilia and cystic fibrosis. A cirrhotic liver is irreversibly damaged.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Nutritional assessments are important to promote growth and development.

Manage side effects such as pruritis, ascites, peritonitis, and GI bleeding.

Treat pruritis with medications; keep nails trimmed to prevent infection.

Manage acites: The amount of salt in the diet may be restricted to 2 grams per day and fluid to 1.2 liters per day. In most patients with cirrhosis, however, salt and fluid restriction is not enough, and diuretics have to be added.

Monitor lab studies closely for hematologic abnormalities and coagulation disorders.

Observe for signs of peritonitis: fever is a concern.

Patient and Family Teaching

Description

Clefts of the lip (CL) and palate (CP) are facial malformations that occur during embryonic development and are the most common congenital deformity of the head and neck. They may appear separately or, more often, together. Cleft lip and palate (CL/P) is more common than CP alone.

Pathophysiology

Cleft deformities represent a defect in cell migration that results in a failure of the maxillary and premaxillary processes to come together between the 3rd and 12th week of embryonic development. Although often appearing together, CL and CP are distinct malformations embryologically, occurring at different times during the developmental process. Merging of the upper lip at the midline is completed between the 7th and 11th weeks of gestation. Fusion of the secondary palate (hard and soft palate) takes place later, between the 7th and 12th weeks of gestation. In the process of migrating to a horizontal position, they are separated by the tongue for a short time. If there is delay in this movement, or if the tongue fails to descend soon enough, the remainder of development proceeds but the palate never fuses. CL and CP may be caused by exposure to teratogens such as alcohol, anticonvulsants, steroids, and retinoids, but there is little evidence to link isolated clefts to any single teratogenic agent with the exception of phenytoin. Use of phenytoin during pregnancy is associated with a tenfold increase in the incidence of CL. The incidence of CL among mothers who smoke during pregnancy is twice as great as the incidence in mothers who do not smoke during pregnancy.

Clinical Signs and Symptoms

Diagnostic Evaluation

Treatment

Nursing Care Management

Patient and Family Teaching

Description

Localized narrowing near the insertion of the ductus arteriosus, which results in increased blood pressure proximal to the defect (head and upper extremities) and decreased blood pressure distal to the obstruction (body and lower extremities).

Pathophysiology

The effect of a narrowing within the aorta is increased pressure proximal to the defect (upper extremities) and decreased pressure distal to it (lower extremities).

Clinical Signs and Symptoms

There may be high blood pressure and bounding pulses in the arms, weak or absent femoral pulses, and cool lower extremities with lower blood pressure. There are signs of congestive heart failure (CHF) in infants (see Box 3-1). In infants with critical coarctation, the hemodynamic condition may deteriorate rapidly with severe acidosis and hypotension. In this situation, mechanical ventilation and inotropic support are often necessary before surgery. Older children may experience dizziness, headaches, fainting, and epistaxis resulting from hypertension. Patients are at risk for hypertension, ruptured aorta, aortic aneurysm, stroke, and bacterial endocarditis.

Diagnostic Evaluation

See Table 3-1.

Treatment

Nursing Care Management

Assist in Measures to Assess and Improve Cardiac Function

Monitor Cardiac Function

Vital signs monitoring

Respiratory assessment

Cardiac assessment

Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function)

Heart sounds

Four-extremity blood pressures (If the subclavian artery is used in the repair, it is not possible to measure an accurate blood pressure in the left arm.)

Keep accurate record of intake and output.

Weigh child or infant on same scale at same time of day.

Monitor for signs and symptoms of postcoarctectomy syndrome.

Abdominal pain and/or distension

Ascites

Fever

Increased white blood cell count

Vomiting

Monitor abdominal girth.

Monitor for blood in gastric contents or stool.

Monitor neurologic function.

Administer Medications

Review child’s history related to cardiac management.

Administer medications on schedule.

Assess effectiveness of medications and any side effects noted.

Be aware of drug-drug and drug-food interactions.

Follow guidelines for medication administration.

Manage Common Side Effects of Cardiac Defects

Prevent fatigue during feeding; offer small frequent feedings to infant’s or child’s tolerance.

Organize nursing care to allow child/infant uninterrupted rest.

Promote activities that do not cause fatigue.

Provide Support for the Child and Family

Communicate with the child and family in an appropriate style.

Provide the child and family with honest answers.

Provide emotional support.

Offer age-appropriate interventions.

Provide information on available resources for support for the child and family.

Patient and Family Teaching

Prepare the Child and Family for Diagnostic and Operative Procedures

Provide explanation of diagnostic tests and why each test is performed because many of them are invasive procedures.

Prepare the child prior to the invasive procedure. Remember discussion is dependent upon the child’s age and development.

Use simple words or pictures to describe procedures and answer all questions the child and family may have.

Postoperative wound care teaching as needed

Post-cardiac catheterization wound care teaching as needed

Assess and record results of teaching and family’s participation in care.

Educate the Child and Family

Assess child’s and family’s level of knowledge regarding the COA.

Assess child’s and family’s understanding of what they have heard about COA.

Assess child’s and family’s understanding of the surgery and/or cardiac catheterization.

Teach the child and/or family at least four characteristics of congestive heart failure that may occur because of the COA such as:

Fatigue after exertion

Fast heart rate

Fast breathing, shortness of breath, dyspnea

Retractions

Nasal flaring

Cool extremities

Diaphoresis

Decreased urinary output

Puffiness (edema)

Fussiness

Decreased appetite

Educate child and family about care such as medication preparation and administration.

Assess child’s and family’s access to appropriate pharmacy for any special preparation medications.

Educate child and family about when to notify their physician of any clinical changes.

Provide appropriate educational resources and review materials with them.

Clarify information presented by the health care team including appointment for follow-up visit.

Discuss SBE (subacute bacterial endocarditis) prophylaxis with pediatric cardiologist.