General Measures: General treatment is principally supportive. During the edema phase the child is often limited to quiet activities, but activity is not restricted during remission. Children can be remarkably active; there is no evidence that restriction affects the ultimate outcome. Acute and intercurrent infections are treated with appropriate antibiotics, and providers make efforts to minimize the risk of infection.

Diet: The child in remission maintains a regular diet. However, salt is restricted during periods of massive edema and while on corticosteroid therapy; no salt is added at the table, and foods with very high salt content are excluded. The child tolerates this diet for a time, but it should be adjusted to the child’s appetite and must not interfere with nutrient intake. Although a low-sodium diet will not remove edema, its rate of increase may be reduced. Water is seldom restricted. A diet generous in protein is logical, but there is no evidence that it is beneficial or alters the outcome of the disease. The presence of azotemia and renal failure is a contraindication for high-protein intake.

Corticosteroid Therapy: The response of most affected children to corticosteroids has established these drugs as prime therapeutic agents in the management of nephrotic syndrome (Kim, Bellew, Silverstein, et al, 2005). Corticosteroid therapy begins as soon as the diagnosis has been determined and is administered orally in a dosage of 60 mg/m²/day in evenly divided doses. Prednisone, the safest and least expensive drug, is the steroid of choice. The drug is continued daily for 6 weeks, then reduced to 40 mg/m² on alternate days for 6 more weeks. Studies suggest that the duration of steroid treatment for the initial episode should be at least 3 months (Hodson, Knight, Willis, et al, 2004).

The course of the disease is fairly predictable. There is little change during the first few days of therapy. In most patients diuresis occurs as the urinary protein excretion diminishes within 7 to 21 days after the initiation of steroid therapy. Other clinical manifestations stabilize or return to normal shortly thereafter. Almost 95% of patients between 1 and 10 years of age who have satisfactory laboratory measurements of C3 complement and a renal clearance of immunoglobulin G, as well as no hypertension, hematuria, or renal insufficiency, will have complete resolution of proteinuria with therapy. If the child has not responded to therapy within 28 days of daily steroid administration, the likelihood of subsequent response diminishes rapidly.

Children with MCNS are often described according to their response to corticosteroid therapy (Box 30-4). Children with MCNS typically relapse one to three times per year. Steroid-dependent children tend to have frequent relapses over many years and receive large amounts of steroids, which results in cushingoid features and may cause growth retardation. They also require supportive treatment (diuretics, diet). Steroid-resistant children are thought to have a high risk of developing chronic renal failure (CRF) (Kim, Bellew, Silverstein, et al, 2005).

Immunosuppressant Therapy: It is possible to reduce the relapse rate and induce long-term remission in children with frequent relapsing or steroid-resistant nephrotic syndrome with administration of an oral alkylating agent, usually cyclophosphamide (Cytoxan) or chlorambucil. Prolonged courses of cyclosporin and levamisole reduce the risk of relapse in children compared with corticosteroids alone (Durkan, Hodson, Willis, et al, 2005). The drugs share many characteristics, and the response to oral alkylating agents appears to depend on dose, duration of therapy, age, and duration of the disease.

The nurse must consider significant side effects of cyclophosphamide and discuss them with parents of children for whom this drug is contemplated. Anticipate leukopenia, and remember that cyclophosphamide may cause azoospermia with potential sterility in males treated for more than 2 to 3 months and may cause variable effects on gonadal function in females.

Diuretics: One characteristic of the edema of nephrotic syndrome is its usual lack of responsiveness to diuretic agents. However, loop diuretics, usually furosemide in combination with metolazone, are sometimes useful in cases in which edema interferes with respiration or there is hypotension, hyponatremia, or skin breakdown. In addition, plasma expanders such as salt-poor human albumin may be administered to severely edematous children requiring prompt control; however, they must be administered frequently because the glomeruli are readily permeable to albumin in the acute stage.

Prognosis: The prognosis for ultimate recovery in most cases is good. MCNS is a self-limiting disease, and in children who respond to steroid therapy the tendency to relapse decreases with time. With early detection and prompt implementation of therapy to eradicate proteinuria, progressive basement membrane damage is minimized so that renal function is usually normal or near normal when the tendency for relapses is past. It is estimated that approximately 80% of affected children have this favorable prognosis, although half the children have relapses even after 5 years, and 20% after 10 years (Bagga and Mantan, 2005).

Family Support and Home Care: Most children are treated at home during relapses. Teach parents to detect signs of relapse and to notify the health care provider if they occur. Home care is preferred unless the edema and proteinuria are severe. Instruct parents in urine testing for albumin, administration of medications, and general care. Urine is usually tested daily while the child is receiving medicine for nephrotic syndrome or if the child has an illness, and twice a week during remission. Salt is restricted to no additional salt during relapse and steroid therapy, but a regular diet is suitable for the child in remission. Instruct parents regarding avoiding contact with infected playmates, but the child is permitted to attend school. It is important for parents of children on corticosteroid therapy to be aware of the common side effects of steroid therapy (e.g., rounding of the face, increased appetite, behavior changes, abdominal distention, and hirsutism) and to distinguish some of these from the edema formation of the disease. Reassure parents that the symptoms will disappear gradually after discontinuation of the drug. The child should receive close medical or nursing observation to detect unusual but more serious side effects (see Critical Thinking Exercise).

The prolonged course of the relapsing form of nephrotic syndrome is taxing to both the child and the family. In the worst cases of frequent remissions and exacerbations with periodic disruption of family life by hospitalization, a severe strain is placed on the child and the family, both psychologically and financially. Parents of children with frequent relapses poorly responsive to medications need reassurance regarding this characteristic of the disease so they do not become discouraged. At the same time, impress on them the importance of long-term care to gain their cooperation. A satisfactory response is more likely when relapses are detected and therapy is instituted early, and remissions are prolonged when instructions are carried out faithfully. For example, one child had an exacerbation when his mother reduced the dosage of his drug because it was so expensive.

Social isolation is a problem for these children. Isolation is related to frequent hospitalization or confinement during relapse, the risk of infection that may precipitate an exacerbation, lack of energy, and the child’s reluctance to face friends at home or school because of the changes in appearance resulting from the disease or the medication. Both parents and child need someone to listen to their complaints, to assist them in coping with both short- and long-term problems associated with the disease, and to find solutions to their problems. Continuous support of the child and family is one of the major nursing considerations.

Proximal Tubular Acidosis (Type II)

Impaired bicarbonate reabsorption in the proximal tubule causes proximal tubular acidosis. It may occur as an isolated defect (primary); however, more often it appears in association with other proximal tubular disorders (secondary). As a result of a depressed renal threshold, bicarbonate reabsorption in the proximal tubule is incomplete, causing the plasma concentration of bicarbonate to stabilize at a lower level than normal. This results in a hyperchloremic metabolic acidosis. There is no impairment of distal tubular integrity or, in most cases, of the distal acidifying mechanism.

A more complex abnormality in the proximal tubules is Fanconi syndrome, in which transport mechanisms are damaged by the accumulation of toxic metabolites or the tubular epithelium is damaged by heavy metals such as lead, cadmium, or platinum. Fanconi syndrome can be part of a number of hereditary diseases, be acquired, or be idiopathic (with a cause that is not identifiable). The major clinical manifestation and presenting symptom of Fanconi syndrome is growth failure. Tachypnea from hyperchloremic metabolic acidosis is also evident. Dehydration, vomiting, episodic fever, nephrolithiasis secondary to hypercalciuria, muscle weakness or paralysis as a result of hypokalemia, and episodes of severe life-threatening acidemia (sometimes triggered by a concurrent infection) may also be seen. Complications are rare. The disorder appears to be transient and resolves spontaneously in time.

Distal Tubular Acidosis (Type I)

Distal tubular acidosis is caused by the kidney’s inability to establish a normal pH gradient between tubular cells and tubular contents. Its most characteristic feature is the inability to produce a urinary pH below 6.0 despite the presence of severe metabolic acidosis (Watanabe, 2005).

Distal renal tubular acidosis may occur as a primary, isolated defect or in association with other diseases or disorders. Most secondary causes are rare. The primary disorder is usually considered to be a hereditary defect with a variable degree of expression and a greater penetrance in females. After the age of 2 years the child usually has growth failure, often with a history of vomiting, polyuria, dehydration, anorexia, and failure to thrive. Evidence of bone demineralization may be present, along with the occasional formation of urinary calculi (urolithiasis) in older children.

The inability to secrete hydrogen ions causes an accumulation of the ions in the body, which soon depletes the available hydrogen buffer and produces a sustained acidosis. Acidosis slows normal somatic growth, and demineralization of bone occurs as bone salts are mobilized to buffer the excessive hydrogen ions. Increased serum levels of both calcium and phosphorus contribute to the development of stones within the renal system. Both sodium and potassium are secreted in larger amounts. Serum potassium levels are depleted as the distal tubules excrete large amounts of potassium ions in an attempt to conserve sodium because hydrogen ions are unable to participate in the exchange. Hyponatremia stimulates increased aldosterone secretion, which further aggravates the hypokalemia. With the depletion of bicarbonate ions, more chloride is reabsorbed in the proximal tubule to create a hyperchloremia.

Therapeutic Management

Treatment of both proximal and distal disorders consists of the administration of sufficient bicarbonate or citrate to balance metabolically produced hydrogen ions; to maintain the plasma bicarbonate level within normal range; and to correct associated electrolyte disorders, especially hypokalemia. Proximal disorders require large volumes of bicarbonate to compensate for urinary losses; in distal disorders the alkali required to maintain a normal plasma concentration is low. Most authorities favor a mixture of sodium and potassium bicarbonate (or citrate) to prevent deficiencies of either cation. The citrate solutions (Bicitra, Polycitra, or Shohl solution) are usually more easily tolerated than bicarbonate solutions. Shohl solution is effective but has the disadvantage of requiring preparation by a pharmacist.

Nursing Care Management

Nursing goals include recognizing the possibility of renal tubular acidosis in children who fail to thrive or who display other symptoms suggestive of the disorders and referring these children for medical evaluation. Helping parents understand the importance of adhering to the medication plan as a long-term goal is essential. (See Compliance and Administration of Medication, Chapter 27.) Children who must continue the medication indefinitely need to learn the importance of taking the medications as soon as they are old enough to assume responsibility for their own care.

Clinical Manifestations and Diagnostic Evaluation

NDI is manifested in the newborn period by vomiting, unexplained fever, failure to thrive, and severe recurrent dehydration with hypernatremia. The passage of copious amounts of dilute urine, which produces severe dehydration and hypoelectrolytemia, is a serious threat to life during this period and may be responsible for the high incidence of cognitive impairment and motor retardation found in affected persons. Growth retardation is probably related to diminished food intake and poor general health because of uncontrolled polydipsia. Diagnosis is suspected on the basis of the patient and family history and confirmed by a urine osmolality value consistently below that of plasma. Lack of response to vasopressin administration rules out other causes.

Therapeutic Management

Therapy involves provision of adequate volumes of water to compensate for urinary losses and minimize urine output through diet and medication (Dell and Avner, 2007). As a result of an insatiable thirst, most of the child’s time is spent drinking and voiding, with little time for activity and stimulation. These children may go to great lengths to satisfy their thirst. A low-sodium, low-solute diet and the use of chlorothiazide or ethacrynic acid diuretics to increase the reabsorption of sodium and water in the proximal tubule help to reduce the amount of tubular fluid delivered to the distal tubules and to diminish the volume of water excreted. Urinary output may be reduced when nonsteroidal antiinflammatory drugs (NSAIDs) are administered in conjunction with chlorothiazide. Supplemental potassium may be required to prevent hypokalemia as a result of thiazide therapy. Normal growth and a normal life span are possible if the disease is recognized early and treatment is instituted and maintained.

Nursing Care Management

Nursing goals for children with NDI and their families are to recognize signs of the disorder early and assist them in coping with the long-term inconvenience of the continual thirst and elimination problems. Families need to learn to administer medications and help with diet planning for those on sodium restriction and needing supplemental potassium. The problem of ensuring adequate hydration is lifelong, and families need to adapt to away-from-home fluid needs and avoid activities that contribute to dehydration when fluids may not be available. Genetic counseling is recommended.

Etiology

In the majority of cases of HUS no causative agents have been identified, although recent theories implicate genetic factors, prostacyclin deficiency, neuraminidase and agglutination, endotoxins (especially Shigella endotoxin), antithrombin III deficiency, deficiency of antioxidants, and reduced platelet aggregation. The appearance of the disease has been associated with Rickettsia organisms; viruses such as coxsackievirus, echovirus, and adenovirus; E. coli; pneumococci; Shigella organisms; and Salmonella organisms and may represent an unusual response to these infections. HUS caused by enteric infection of the E. coli O157:H7 serotype is the most prevalent pathogen in the United States and Europe, with about 70,000 cases and 60 deaths occurring annually (Bell, Griffin, Lozano, et al, 1997; Caprioli, Peng, and Remuzzi, 2005). Occurrences have been traced to undercooked meat, especially ground beef; unpasteurized apple juice; alfalfa sprouts; and public pools.

The disease usually follows an acute gastrointestinal or upper respiratory tract infection and tends to occur in scattered outbreaks in small geographic areas. HUS is clinically and pathologically similar to thrombocytopenic purpura, except for the hypertension associated with HUS.

Pathophysiology

The primary site of injury appears to be the endothelial lining of the small glomerular arterioles, but other organs and tissues may be involved (e.g., the liver, brain, heart, pancreatic islet cells, and muscles). The endothelium becomes swollen and occluded with the deposition of platelets and fibrin clots (intravascular coagulation). Red blood cells are damaged as they move through the partially occluded blood vessels. The spleen removes these fragmented red blood cells, causing acute hemolytic anemia. Fibrinolytic action on the precipitated fibrin causes these fibrin-split products to appear in the serum and urine. The characteristic thrombocytopenia is produced by the platelet aggregation within damaged blood vessels or the damage and removal of platelets.

Clinical Manifestations

The disease occurs after a prodromal period during which there is an episode of diarrhea and vomiting. Less often the preceding illness is an upper respiratory tract infection or, occasionally, varicella, measles, or a UTI.

The hemolytic process persists for several days to 2 weeks. During this time the child is anorexic, irritable, and lethargic. There is marked and rapid onset of pallor accompanied by hemorrhagic manifestations such as bruising, purpura, or rectal bleeding. Severely affected patients are anuric and often hypertensive. Seizures and stupor suggest central nervous system involvement, and there may be signs of acute heart failure. Mild cases demonstrate anemia, thrombocytopenia, and azotemia; urinary output may be reduced or increased.

Diagnostic Evaluation

The triad of anemia, thrombocytopenia, and renal failure is sufficient for diagnosis. Proteinuria, hematuria, and urinary casts are evidence of renal involvement; BUN and serum creatinine levels are elevated. A low hemoglobin and hematocrit and a high reticulocyte count confirm the hemolytic nature of the anemia.

Therapeutic Management

In general, providers direct treatment toward control of the complications and hematologic manifestations of renal failure (Siegler and Oakes, 2005). The initial supportive measures for most children are those used in managing renal failure: fluid replacement (calculated with great care), treatment of hypertension, and correction of acidosis and electrolyte disorders (Siegler and Oakes, 2005). The most consistently effective treatment is early hemodialysis, PD, or continuous hemofiltration, which is instituted in any child who has been anuric for 24 hours or who demonstrates oliguria with uremia or hypertension and seizures. Blood transfusions with fresh, washed packed cells are administered for severe anemia but are used with caution to prevent circulatory overload from added volume.

Once vomiting and diarrhea have resolved, the child is restarted on enteral nutrition. Sometimes parenteral nutrition is required for children with severe, persistent colitis and for those in whom tissue catabolism is marked. There is no substantial evidence that heparin, corticosteroids, or fibrinolytic agents are beneficial, and in some instances they may aggravate the condition. The usefulness of plasma infusion for treatment of HUS is currently being studied; it may be useful in selected cases.

Nursing Care Management

Nursing care is the same as that provided in ARF and, for children with continued impairment, includes management of chronic disease. Because of the sudden and life-threatening nature of the disorder in a previously well child, parents are often ill prepared for the impact of hospitalization and treatment. Therefore support and understanding are especially important aspects of care.

Therapeutic Management

Severe injury requires close observation in the hospital intensive care unit and blood replacement if there is severe internal or external bleeding. In most cases bleeding subsides spontaneously. Surgical exploration is indicated if there are multiple injuries, extravasation of blood around the kidneys, or disruption of the major vessels or the collecting system. Children with less severe injuries, such as contusions only, are placed on bed rest. They should remain on bed rest for 3 days after cessation of gross bleeding, since the substance released from injured renal tissue (urinary urokinase) has strongly fibrinolytic properties that may precipitate serious bleeding. The prognosis depends on the nature and extent of the injury.

Nursing Care Management

Nursing management is directed toward recognizing and assisting in the diagnosis of renal injury. Care of both the child and the family is primarily supportive. The nurse implements all the concepts related to emergency hospitalization and care. (See Chapter 26.) Postsurgical care, if indicated, is the same as for any other surgical patient. Recommendations for children with a solitary kidney participating in sports are varied. Each child should be individually assessed and advised on the need for protective equipment before engaging in contact, collision, or limited contact activities (Rice and Council on Sports Medicine and Fitness, 2008).

Etiology

ARF can develop as a result of a large number of related or unrelated clinical conditions: poor renal perfusion; acute renal injury; or the final expression of chronic, irreversible renal disease. The most common cause in children is transient renal failure resulting from dehydration or other causes of poor perfusion that respond to restoration of fluid volume. Causes of ARF are usually classified as prerenal, intrinsic renal, and postrenal. Severe or longstanding prerenal or postrenal causes can produce severe secondary renal damage.

Pathophysiology

ARF is usually reversible, but the deviations of physiologic function can be extreme, and mortality in the pediatric age-group is still high. There is severe reduction in the glomerular filtration rate, an elevated BUN level, and decreased tubular reabsorption of sodium from the proximal tubule. Consequently, there is increased concentration of sodium in the distal tubule, which causes stimulation of the renin mechanism. The local action of angiotensin causes vasoconstriction of the afferent arteriole, which further reduces glomerular filtration and prevents urinary losses of sodium. There is a significant reduction in renal blood flow.

The pathologic conditions that produce ARF caused by glomerulonephritis, HUS, and other renal disorders are discussed in relation to those disease processes. The necrotic processes within the nephron can be cortical, tubular, or both.

Clinical Manifestations

In many instances of ARF the infant or child is already critically ill with the precipitating disorder, and the explanation for development of oliguria may or may not be readily apparent. The underlying illness often overshadows the renal failure and often assumes the priority of care (e.g., the patient who is in shock from endotoxemia, the infant who is severely dehydrated from gastroenteritis, or a child who is subject to seizures as a result of hypertensive encephalopathy associated with AGN).

The prime manifestation of ARF is oliguria, generally a urinary output of less than 1 ml/kg/hr. Anuria (no urinary output in 24 hours) is uncommon except in obstructive disorders. Other symptoms related to ARF include edema, drowsiness, circulatory congestion, and cardiac arrhythmia from hyperkalemia. Seizures may be caused by hyponatremia or hypocalcemia and tachypnea from metabolic acidosis. With continued oliguria, biochemical abnormalities can develop rapidly, and circulatory and central nervous system manifestations appear.

Diagnostic Evaluation

When a previously well child develops ARF without obvious cause, a careful history is obtained to reveal symptoms that may be related to glomerulonephritis; obstructive uropathy; or exposure to nephrotoxic chemicals, such as ingestion of heavy metals or inhalation of carbon tetrachloride or other organic solvents or drugs (e.g., methicillin, sulfonamides, NSAIDs, neomycin, polymyxin, and kanamycin). Laboratory data reflect the kidney dysfunction—hyperkalemia, hyponatremia, metabolic acidosis, hypocalcemia, anemia, or azotemia (Table 30-7).

Therapeutic Management

The most effective management of ARF is prevention. The development of ARF is a known risk in certain situations. This should be anticipated and recognized, and adequate therapy should be implemented (e.g., fluid therapy for children with hypovolemia in conditions such as dehydration, burns, and hemorrhage). Nephrotoxic drugs should be used with caution or avoided in children with renal disease, and all personnel should be knowledgeable about precautions related to their administration. For example, a generous fluid intake is needed for children receiving antimetabolite drugs and after radiotherapy.

The treatment of ARF is directed toward (1) treatment of the underlying cause, (2) management of the complications of renal failure, and (3) provision of supportive therapy within the constraints imposed by the renal failure. Treatment of poor perfusion resulting from dehydration consists of volume restoration as described in the treatment of dehydration. (See Chapter 28.) If oliguria persists after restoration of fluid volume or if the renal failure is caused by intrinsic renal damage, the physiologic and biochemical abnormalities that have resulted from kidney dysfunction must be corrected or controlled. Central venous pressure monitoring is usually implemented.

Initially a Foley catheter is inserted to rule out urine retention, to collect available urine for electrolytes and analysis, and to monitor the results of diuretic administration. The catheter may or may not be removed. Some clinicians believe that it serves little purpose during the oliguric phase and predisposes the patient to bladder infection prefer collection bags for measuring urinary output. Others maintain a catheter for hourly urine measurements.

Nursing Care Management

Nursing care of the infant or child with ARF involves addressing the underlying cause plus carefully observing and managing the renal status. The major goal is reestablishment of renal function (with emphasis on providing an adequate caloric intake to minimize reduction of protein stores); prevention of complications; and monitoring of fluid balance, laboratory data, and physical manifestations. The probability of dialysis or continuous hemofiltration is high, and the nurse must anticipate the availability of the necessary equipment. Because the child requires intensive observation and often specialized equipment, the usual disposition is admission to an intensive care unit where equipment and trained personnel are available.

Nursing Care Plan—The Child with Acute Renal Dysfunction

The major nursing tasks in the care of the infant or child with ARF are monitoring and assessing fluid and electrolyte balance. Limiting fluid intake requires ingenuity on the part of caregivers to cope with the child who is thirsty. One strategy involves rationing the daily intake with small amounts of fluid served in containers that give the impression of larger volumes. Older children who understand the rationale of fluid limits can help determine how their daily ration should be distributed.

Meeting nutritional needs is sometimes a problem because the child may be nauseated and because getting the child to eat concentrated foods without fluids may be difficult. When nourishment is provided by the IV route, careful monitoring is essential to prevent fluid overload. This can become a major challenge in the face of nutritional requirements and administration of IV medications. The IV drugs being used may be nephrotoxic, which can require a specified volume of solution for delivery. In some instances blood products must also be delivered. Preventing fluid overload while delivering medications and calories requires concerted collaboration. In addition, nursing measures such as maintaining an optimum thermal environment, reducing any elevation of body temperature, and reducing restlessness and anxiety are used to decrease the rate of tissue catabolism.

The nurse must be continually alert for behavior changes that indicate the onset of complications. Infection from reduced resistance, anemia, and general morbidity is a constant threat. Fluid overload and electrolyte disturbances can precipitate cardiovascular complications such as hypertension and cardiac failure. Fluid and electrolyte imbalances, acidosis, and accumulation of nitrogenous waste products can produce neurologic involvement manifested by coma, seizures, or alterations in sensorium.

Although children with ARF are usually quite ill and voluntarily diminish their activity, infants may become restless and irritable, and children are often anxious and frightened. Frequent, painful, and stress-producing treatments and tests must be performed. A supportive, empathetic nurse can provide comfort and stability in a threatening and unnatural environment.

Etiology

A variety of diseases and disorders can result in CRF. The most common causes of CRF before age 5 years are congenital renal and urinary tract malformations (particularly renal hypoplasia and dysplasia and obstructive uropathy) and VUR. Glomerular and hereditary renal disease predominate in children 5 to 15 years of age. The glomerular diseases that most commonly lead to CRF are chronic pyelonephritis, CGN, and glomerulonephropathy associated with systemic diseases such as anaphylactoid purpura and lupus erythematosus. Hereditary nephritis, congenital nephrotic syndrome, Alport syndrome, polycystic kidney, and several other hereditary disorders result in renal failure in childhood. Renal vascular disorders such as HUS, vascular thrombosis, or cortical necrosis are less common causes.

Pathophysiology

Early in the course of progressive nephron destruction, the child remains asymptomatic with only minimum biochemical abnormalities. Unless its presence is detected in the process of routine assessment, signs and symptoms that indicate advanced renal damage often emerge only late in the course of the disease. Midway in the disease process, as increasing numbers of nephrons are totally destroyed and most others are damaged to varying degrees, the few that remain intact are hypertrophied but functional. These few normal nephrons are able to make sufficient adjustments to stresses to maintain reasonable degrees of fluid and electrolyte balance. Definitive biochemical examination at this time reveals restricted tolerance to excesses or restrictions. As the disease progresses to the end stage because of severe reduction in the number of functioning nephrons, the kidneys are no longer able to maintain fluid and electrolyte balance, and the features of the uremic syndrome appear.

The following sections briefly summarize the pathophysiology of specific biochemical abnormalities.

Clinical Manifestations

The first evidence of difficulty is usually loss of normal energy and increased fatigue on exertion. For example, the child may prefer quiet, passive activities rather than participation in more active games and outdoor play. The child is usually somewhat pale, but the change is often so subtle that it may not be evident to parents or others. Blood pressure is sometimes elevated. Growth is affected early in the development of CRF, and falling behind on the growth chart is often the first measurable sign.

Other manifestations may appear as the disease progresses. The child does not eat as well (especially breakfast), shows less interest in normal activities such as schoolwork or play, and has a decreased or increased urinary output and a compensatory intake of fluid. For example, a child who has achieved bladder control may wet the bed at night. Pallor becomes more evident as the skin develops a characteristic sallow, muddy appearance as a result of anemia and deposition of urochrome pigment in the skin. The child may complain of headache, muscle cramps, and nausea. Other signs and symptoms include weight loss, facial puffiness, malaise, bone or joint pain, growth retardation, dryness or itching of the skin, bruised skin, and sometimes sensory or motor loss. Amenorrhea is common in adolescent girls.

Therapy is generally initiated before the appearance of the uremic syndrome, although on some occasions the symptoms may be observed. Manifestations of untreated uremia reflect the progressive nature of the homeostatic disturbances and general toxicity. Gastrointestinal symptoms include loss of appetite, nausea, and vomiting. Bleeding tendencies are apparent in bruises, bloody diarrheal stools, stomatitis, and bleeding from the lips and mouth. There is intractable itching, probably related to hyperparathyroidism. Deposits of urea crystals may appear on the skin as uremic frost but are seldom seen because of the availability of dialysis and transplantation (Springhouse, 2008). There may be an unpleasant uremic odor to the breath. Respirations become deeper as a result of metabolic acidosis, and circulatory overload is manifested by hypertension, congestive heart failure, and pulmonary edema. Progressive confusion, dulling of the sensorium, and ultimately coma are signs of neurologic involvement. Other signs may include tremors, muscular twitching, and seizures.

Diagnostic Evaluation

The diagnosis of CRF is usually suspected on the basis of any of a number of clinical manifestations, a history of prior renal disease, or biochemical findings. The onset is usually gradual, and the initial signs and symptoms are vague and nonspecific. Laboratory and other diagnostic tools and tests are of value in assessing the extent of renal damage, biochemical disturbances, and related physical dysfunction. Often they can help establish the nature of the underlying disease and differentiate between other disease processes and the pathologic consequences of renal dysfunction.

Therapeutic Management

The multiple complications of CRF are managed according to medical protocols such as the National Kidney Foundation Kidney Disease Outcomes Quality Initiative evidence-based clinical practice guidelines (www.kidney.org/professionals/KDOQI). The goals of management are to promote effective renal function, maintain body fluid and electrolyte balance within acceptable limits, treat systemic complications, and promote as active and normal a life as possible for the child for as long as possible. This becomes increasingly difficult as the disease progresses toward its inevitable end. Therapeutic measures designed to relieve one manifestation may negatively affect another. For example, antihypertensive agents may further impair renal function.

Nursing Care Management

The child with CRF has a life maintained by drugs and artificial means, and the multiple stresses placed on these children and their families are often overwhelming. The unrelenting course of the disease process is one of progressive deterioration. There is no means by which to prevent the irreversible progress of renal insufficiency, nor is there any known cure. As the affected child progresses from renal insufficiency to uremia and then to dialysis and transplantation with intense therapy, the need for supportive nursing care is also intensified. Team effort is more important than ever and involves coordination of personnel from medicine, nursing, social services, child life, physical and occupational therapy, dietetics, and psychologic or psychiatric specialties (see Nursing Care Plan).

Progressive disease places a number of stresses on the child and family. There is a continuing need for repeated examinations that often entail painful procedures, side effects, and frequent hospitalizations. Diet therapy can become progressively more restrictive and intense, and parents may need help in learning to select appropriate foods, read labels carefully for sodium and potassium content, and modify meals to accommodate the child’s special needs. The child is required to take a variety of medications. Compliance is difficult when long-term therapies are involved. Ever present in all aspects of the treatment regimen is the agonizing realization that, without treatment, death is inevitable.

ESRD presents the same nonspecific stresses on child and family as any other chronic or life-threatening illness. (See Chapters 22 and 23.) The reactions and adaptation of the child and family depend on the child’s age and developmental stage, the family’s cultural and socioeconomic background, the quality of the interpersonal relationships of family members, and the communication patterns within the family. In general, the problems observed and the emotional responses to the stress of the illness are influenced less by the nature of the illness than by the family relationships and personalities (see Family-Centered Care box).

One of the first and most noticeable changes is the alteration in physical appearance—fluctuations in weight, anemia, and failure to grow. Children must adjust to the fact that they will always be different from their peers in some ways. They will be shorter, often more tired, and unable to participate in all the activities that are attractive to young people. Children who have had diversion procedures, dialysis, and other surgeries or who urinate into a bag need to learn positive coping strategies for the alterations in their body image and for the questions and potential teasing of peers. It is not unusual for children with chronic conditions to exhibit behavioral regression. This is particularly so for children with renal failure because their appearance is often of a child much younger than their chronologic age.

School is often difficult for these children. Frequent absences for illnesses, evaluations, or treatments disrupt the educational process and socialization. Teachers and school systems are not always sympathetic to the rights and needs of a child with a chronic illness (e.g., the right to equal education and the need for flexibility and special help at times), which places an additional burden on the parents.

In some families illness and stressful experiences act as a unifying force; in other families stress aggravates preexisting problems and contributes to family disharmony. The relentless nature of the disease and its therapies not only places physical and emotional stresses on the family but is also a chronic drain on the family finances. Insurance rarely covers the full cost of the multiple hospitalizations and outpatient expenses. The federal Medicare program, for which most children qualify, funds ESRD care. However, there are many hidden costs, such as transportation to special treatment centers, meals, and sometimes lodging away from home. Some private foundations, churches, and community groups provide temporary assistance, and nurses should become familiar with those in the area of their practice that can offer financial and educational services to these families. For example, the National Kidney Foundation* and numerous other agencies provide services and information for families, including pamphlets and descriptive literature. Particularly useful are booklets written for children with renal disease.

Certain specific stresses related to ESRD and its treatment are predictable. When it first becomes apparent that kidney failure is inevitable, both the child and the parents experience depression and anxiety. Acceptance is particularly difficult if renal failure progresses rapidly after the diagnosis. Children, and especially parents, usually express denial and disbelief. Denial can also develop when progression to ESRD has been prolonged and both the child and the parents come to believe it will never occur.

Once the renal failure is established and the symptoms become progressively more distressing, parents usually perceive the initiation of hemodialysis or kidney transplantation as a positive experience. After the initial concerns of implementing the treatment, the child begins to feel better, and parental anxiety is relieved for a time.

Procedure

Hemodialysis requires special dialysis equipment—the hemodialyzer, or so-called artificial kidney (Figs. 30-4 and 30-5). Hemodialyzers are available in two forms: parallel flow (plate) and hollow fiber. Hollow fiber dialyzers are preferable for children because their blood compartment is rigid and available in relatively small volumes. Pediatric dialysis can be safely carried out when the total dialysis circuit volume does not exceed 10% of the child’s estimated blood volume.

Hemodialysis also requires one of three means of blood access: grafts, fistulas, or external access devices. An arteriovenous fistula is an access in which a vein and artery are connected surgically. The preferred site is the radial artery and a forearm vein. The creation of a subcutaneous (internal) arteriovenous fistula by anastomosing a segment of the radial artery and brachiocephalic vein produces dilation and thickening of the superficial vessels of the forearm to provide easy access for repeated venipuncture. Fewer complications and less restriction of activity are observed with the use of a fistula. If vessels are inadequate for an autogenous fistula, a synthetic graft may be placed in the arm or thigh with either a loop or straight configuration. Both the graft and the fistula require needle insertion at each dialysis. For short-term external vascular access, percutaneous catheters are inserted in the femoral or internal jugular veins, even in very small children. Avoid subclavian access because of the potential complication of stenosis. For long-term external vascular access, cuffed, dual-lumen (single-lumen for infants) catheters can be surgically placed similarly to other central venous access devices. They are ready to be used immediately, and do not require needles.

Various hemodialysis schedules are used, but most centers recommend dialysis three times a week for 3 to 5 hours, depending on the child’s size. The length of a hemodialysis treatment, the blood flow rate, and dialyzer characteristics contribute to adequacy of treatment. Current target levels for adequacy are a urea reduction ratio of 65% or higher or a Kt/V (Clearance × Time/Volume) of 1.4 or higher. For a complete description of the highly specialized process of hemodialysis, see the numerous references available on this topic.

Dietary limitations are necessary in chronic dialysis to avoid biochemical complications. Fluid and sodium are restricted to prevent fluid overload and its associated symptoms of hypertension, cerebral manifestations, and congestive heart failure. Potassium is restricted to prevent complications related to hyperkalemia; phosphorus restriction helps prevent parathyroid hyperactivity and its attendant risk of abnormal calcification in soft tissues. Adequate protein intake is necessary to maximize growth potential. Fluid limitations are determined by residual urinary output and the need to limit intradialytic weight gain.

Seizures during or after hemodialysis are now uncommon. With the current practice of hemodialysis, cerebral edema caused by alterations in osmolality in the brain when the BUN level is lowered rapidly (associated with dialysis disequilibrium syndrome) is rare.

Home Hemodialysis

With appropriate cannulization and proper training and education of both the child and the parents, hemodialysis can be performed at home. Time spent in transportation is eliminated, the environment is more pleasant and secure, and the child is able to assume a more active role in the treatment program. Home hemodialysis is especially advantageous for children waiting for a transplant who live a great distance from the dialysis center or for children who have had one or more kidney transplant failures.

Home hemodialysis units are available to some children, and the preparation and management are similar to those required for hemodialysis in the hospital (Geary, Piva, Tyrrell, et al, 2005). The patient is equipped with a dialysis unit that is used with the vascular access established for outpatient dialysis. Parents of children on home hemodialysis must know how to operate the equipment, connect the unit to the vascular access, and assess the child’s status. Home hemodialysis is more prevalent in sparsely populated regions of the country.

Nursing Care Management

Initiating a hemodialysis regimen is a traumatic and anxiety-provoking experience for most children. After surgery for implantation of the graft, fistula, or long-term external access device, the initial experience with the hemodialysis machine and its implication can be frightening. They need reassurance about the preparations for dialysis and the conduct of the treatment. They are anxious about repeated venipunctures (with implanted shunts and for blood chemistries) and about the sight of their blood leaving their body and entering the machine (see Atraumatic Care box). The child’s physiologic response to the treatment (e.g., nausea and vomiting, cramps, or seizures) can also cause anxiety. These are usually individual responses related to the child’s overall well-being and degree of compliance with the total medical regimen. Once the initial fear of the machine has been resolved, the nurse can help children develop strategies for dealing with restricted activity and movement for the duration of each treatment (see Fig. 30-5).

With their increased need for independence and their urge for rebellion, adolescents may not adapt as well. They resent dependency on a machine, parents, professional staff, and an unrelenting therapy program. Depression, hostility, or both are common in adolescents undergoing hemodialysis. The adverse consequences of the disease include the need for diet restrictions, limitations in physical activity (resulting from lack of energy, frequent illnesses, and specific restrictions related to access), and the sense of being different from other children. Withdrawal from peers and social isolation are the rule, and noncompliance with the therapeutic regimen is not uncommon.

Body changes related to the disease process, such as growth retardation, skin color changes, and lack of sexual maturation, are stress provoking. Dietary restrictions are particularly burdensome for both children and parents. Children feel deprived when unable to eat foods previously enjoyed and unrestricted for other family members. Consequently, failure to cooperate is not uncommon. Diet restrictions are interpreted as punishment; because they may not be able to fully understand the purpose of the restrictions, some will sneak forbidden food items at every opportunity. Allowing children, especially adolescents, maximum participation in and responsibility for their own treatment program is helpful. The extent of adherence and adjustment depends on the personalities of the involved persons, the quality of their relationships, and their coping mechanisms.

After weeks, months, or years of hemodialysis, the parents and the child feel anxiety associated with the prognosis and continued pressures of the treatment. The relentless need for treatment interferes with family plans and activities, including school. Graft and fistula problems are a common source of aggravation. Most families and children on hemodialysis look to kidney transplantation as a desirable alternative to long-term treatment.

Procedure

In acute situations PD catheter insertion may be accomplished at the bedside; catheters for long-term use are placed surgically in the operating room with the patient under anesthesia. A catheter is inserted through the anterior abdominal wall, and the catheter cuff is sutured into place. Chronic PD catheters are tunneled through a subcutaneous tract before exiting the skin in a manner similar to implantation of central venous access devices. At the time of dialysis a commercially prepared dialysis solution is allowed to flow by gravity through the catheter and into the peritoneal cavity, where it remains while equilibrium between plasma and dialysis fluid takes place. Approximately 30 to 50 ml/kg, or 1100 ml/m², of dialysis solution is instilled at each cycle. The fluid is then allowed to flow by gravity drainage into a receptacle, and fresh dialysis solution is again instilled.

In PD each pass or cycle is characterized by inflow time, dwell time, and drain time. The length of each portion of the cycle is part of the dialysis prescription. The dwell time varies according to the goals of the treatment (i.e., removal of water, solute, electrolyte, or all of these) (Schroder, 2005). The procedure is usually continued until renal function is restored, waste products are reduced, or (in prolonged need) the patient is switched to a form of chronic PD—continuous ambulatory peritoneal dialysis (CAPD) or continuous cycling peritoneal dialysis (CCPD). An acute PD catheter may remain in place for several weeks provided that aseptic technique is adhered to by all who enter the system.

Home Dialysis

The development of satisfactory methods for CAPD and its alternative, CCPD, has provided additional means for managing ESRD at home. In both methods commercially available sterile dialysis solution is instilled into the peritoneal cavity through the surgically implanted indwelling catheter. The warmed solution is allowed to enter the peritoneal cavity by gravity and remains a variable length of time according to the procedure used. Dialysis solution is infused and dialysate drained through a single catheter.

In CAPD the dialysis solution is instilled and the line is clamped off and worn attached to the abdomen or thigh or even placed in a pocket. Manufacturers offer a variety of disconnect devices (e.g., Y set), all of which minimize the connectivity and amount of tubing the patient carries between exchanges. The solution remains in the peritoneum for 4 to 6 hours. The dialysate is then drained via gravity into a bag. Another warmed bag is infused, and the process is repeated so that there is fluid in the abdomen continuously. The procedure is performed a minimum of three times during the day and once at night. For an active child CAPD has proved to be a satisfactory alternative to hemodialysis.

CCPD is a modification of CAPD and intermittent PD. The dialysis exchange is usually performed at night using a PD machine that warms the dialysis fluid and automates the cycles of inflow of dialysis fluid and outflow of dialysate. As with CAPD, the CCPD system is opened during the day, but only twice as opposed to multiple times. Nighttime dialysis allows the child more freedom during the day and relieves parents of the need to perform multiple exchanges.

The care and management of the procedure are the responsibility of the parents of young children. Older children and adolescents are able to carry out the procedure themselves, thus providing them with some control and less dependency. This is especially important for adolescents.

Nursing Care Management

The availability of home dialysis has offered a greater degree of freedom for persons undergoing long-term dialysis. It eliminates the need for a residence convenient to a dialysis unit and for frequent trips to the unit, except for monthly evaluations. The nurse is responsible for teaching the family. Education focuses on (1) the disease, its implications, and the therapeutic plan; (2) the possible psychologic effects of the disease and the treatment; and (3) the technical aspects of the procedure.

The family must learn how to take vital signs before and after the dialysis and how to interpret the significance of blood pressure and temperature variations. They need to know how to vary the composition of the dialysis solution to compensate for variations in the vital signs and to maintain an accurate record of all aspects of the treatment.

Parents of the young child using CAPD need to learn how to exchange bags and manage the procedure at home. Even newborn infants are able to benefit from PD. Older children can learn to take responsibility for their own treatments as much as possible. Encourage the family to ask questions throughout the preparation time, including those that clarify anatomy and physiology, mechanical functioning, and side effects of the disease and the treatment. The PD schedule is outlined to meet the individual needs of the patient and family. Most schedules are arranged for uninterrupted sleep at night and coordination of the dialysis with school and other activities. The nurse discusses diet, medication, and activity and explores feelings about the entire therapeutic program with the child and family.

Infection is the greatest hazard of PD; therefore instruct the family to contact the appropriate persons at the earliest evidence of peritonitis. In most instances peritonitis can be controlled with antibiotics. Unfortunately, there is a high incidence of peritonitis, and repeated infections may necessitate replacement of the catheter or its removal and abandonment of the peritoneum as an access route.

The importance of emotional and material support cannot be overemphasized. The National Kidney Foundation, mentioned previously, provides a number of services and information for families of children with renal disease. A relatively new organization, the American Association of Kidney Patients,* has been organized to promote the interest and welfare of kidney patients. It provides education and support for patients and public education regarding all areas of kidney disease.

Procedure

The kidney graft is placed in the extraperitoneal space, usually the anterior iliac fossa; the renal artery is anastomosed to the internal iliac or hypogastric artery; the renal vein is anastomosed to the hypogastric vein; and the ureter is implanted into the bladder or anastomosed to the recipient’s ureter. Small children receiving a large donor kidney may require placement within the abdomen with vessel anastomoses to the aorta and inferior vena cava. Unless there is medical contraindication, the recipient’s failed kidneys are left in place. Severe hypertension, neoplasm, large and continuous protein losses, and persistent severe VUR are the usual reasons for nephrectomy.

The primary goal in transplantation is the long-term survival of the grafted tissue. The means by which this is attempted include (1) securing tissues that are antigenically similar to that of the recipient and (2) suppressing the recipient’s immune mechanism.

Selection of Donor Tissue

The source of a donor kidney is either a live person or a deceased donor. The closer the genetic relationship between the donor and recipient, the better the possibility of long-term survival. The only truly compatible tissue match is that between identical twin siblings. The next best possible match is a sibling, then a parent or grandparent. In some states the use of siblings is impossible until the possible donor is of age to give consent for removal of a kidney. Unrelated donors are least likely to be compatible. Careful immunologic studies are carried out to determine the donor whose kidney is least likely to be rejected by the recipient.

Suppression of the Immune Response

After the best possible tissue match is obtained for a transplant, the survival time can be significantly lengthened by suppressing the recipient’s immune response. The immunosuppressant therapy of choice in kidney transplantation is corticosteroids (prednisone) in conjunction with cyclosporine and azathioprine. Other therapies include antilymphocyte globulin or monoclonal antibodies, administered intravenously for 14 days either for induction or rescue from rejection.

The administration of these drugs is not without hazard. The major problem encountered with nonspecific immunosuppression is that it not only suppresses the immune response to the grafted tissue but also suppresses the body’s capacity to respond to other antigenic stimuli. Consequently, the child is vulnerable to overwhelming infections.

Prednisone is an immunosuppressant and antiinflammatory agent that acts to stabilize cell walls, reduce migration of white blood cells into the inflamed area, and inhibit deposition of fibrin and collagen. It also depresses T cells, B cells, and phagocytes. A number of complications from corticosteroid therapy are cause for concern. Interference with linear growth has led many centers to use alternate-day administration in an effort to improve growth rates and to decrease other long-term side effects such as cataracts, fluid and sodium retention, hypertension, gastric ulcer, and obesity. Researchers are studying steroid free and early steroid withdrawal treatment protocols with the goal of minimizing side effects without compromising graft survival (Miller and Brennan, 2009).

Cyclosporine is a powerful immunosuppressant that acts to decrease the production of T cells. The side effects of this drug are hypertension, which may appear within several days of transplant; hirsutism; and nephrotoxicity. Serum blood levels determine maintenance doses of cyclosporine. After the initial IV therapy immediately after the transplant, the drug is administered orally. When the liquid form is used, each center has specific instructions for administering cyclosporine. The concerns are that the complete dose will be taken and absorption of the medication will be maximized.

Azathioprine, another immunosuppressant, interferes with cellular protein synthesis. The problem related to the toxic effect of azathioprine is mainly neutropenia, which is usually managed by reduced dosage. (See Chapter 36 for a discussion of immunosuppressive therapy and related nursing care.)

Rejection

Rejection of a transplanted kidney is the most common cause of transplant failure. Rejection can be one of three types: hyperacute, acute, or chronic. Hyperacute rejection is irreversible, develops immediately or within a few hours after revascularization, and is related to circulating antibodies preformed in the recipient against the donor tissue antigens.

Acute rejection usually occurs between the first few days and months after transplantation but may occur years later, especially if the patient becomes poorly compliant with immunosuppressant medications. Both biochemical and clinical abnormalities are evidence of rejection. The most common finding is fever, which is usually accompanied by swelling and tenderness over the graft, hypertension, and diminished urinary output. A severe reaction may cause oliguria. Increases in serum BUN and creatinine levels are laboratory evidence of decreased transplant function. Most acute rejection episodes respond to IV administration of methylprednisolone sodium succinate (Solu-Medrol), antilymphocyte globulin, or monoclonal antibodies.

Slow, gradual deterioration of renal function that typically begins 6 months or more after transplantation characterizes chronic rejection. Elevated serum creatinine, proteinuria, or hematuria are all signs of rejection. In addition, the rejection may have symptomatology indistinguishable from that of the original kidney disease. No present therapy can halt the process, which inevitably leads to loss of the implanted kidney.

Prognosis

The overall graft survival rate for kidneys at 1, 3, and 5 years is 96%, 91%, and 86% from living donors and 94%, 81%, and 80% from deceased donors, according to the annual report of the North American Pediatric Renal Trials and Collaborative Studies (2008). Predictors of graft survival for children include age at transplantation, pretransplantation dialysis, early rejection, and race. Malignancies, infection, and hypertension are the most life-threatening problems after transplantation (Groothoff, 2005). Long-term graft survival is not guaranteed, and many children require a second or third transplant. Successful kidney transplantation does improve rehabilitation of children with CRF, both educationally and psychologically.

Nursing Care Management

The possibility of kidney transplantation often comes as a hope for relief from the rigors of dialysis or the restriction of a conservative management regimen. Most children and families respond well to a kidney transplant. Children with successful kidney transplants are usually able to resume life activities similar to those of their unaffected peers. The rehabilitation of children with kidney transplants is influenced primarily by their pattern of functioning before becoming ill. It is important to remember that transplantation is a treatment that has a far less negative impact on the child’s normal life activities than dialysis. However, stresses remain for the child and family in relation to the uncertainty of the future, the child’s health and well-being, social isolation, and financial burdens.

A variety of serious emotional and psychologic conflicts may arise as a consequence of donor selection, including ambivalence of donors faced with surgery and relinquishing a kidney, feelings of guilt if one should prove to be unacceptable as a donor, and the emotional impact of having a live relative–donated kidney rejected by the recipient. This especially can result in guilt feelings when a parent is the donor.

The child recipient responds in various ways to a kidney transplant. The concept of having a foreign body, especially a deceased donor kidney, inside their own body is sometimes disturbing to children. They often speculate about the age, sex, personality, and physical characteristics of the donor. They may fear that the kidney will wear out if it came from an older person. Some children are distressed to find that their donor kidney came from a person of the opposite sex. Corticosteroid therapy, necessary in kidney transplants, creates undesirable side effects (e.g., growth failure, obesity, characteristics of Cushing syndrome [see Fig. 38-5], acne, and hirsutism) that are often a source of emotional and social problems for older children. Gum hyperplasia, brittle fingernails, and hair breaking can also occur.

Working with children and their families during the various stages of renal failure, dialysis, and transplantation is a difficult and challenging experience. Nurses must become familiar with the family; assess family strengths, weaknesses, and coping mechanisms; and be prepared to provide intensive support and guidance during the prolonged experience. The child and family need help accepting what is happening to them. They also need help in following the nurse’s anticipatory guidance regarding predictable stresses and in dealing constructively with the physical, emotional, and financial burdens that are an ongoing part of this prolonged disability.

• The main function of the kidney is to maintain the composition and volume of body fluids in equilibrium. Common inflammatory disorders of the genitourinary tract include UTI, nephrotic syndrome, and AGN.

• Management of UTIs is directed at eliminating infection, detecting and correcting functional or anatomic abnormalities, preventing recurrences, and preserving renal function.

• VUR is the retrograde flow of bladder urine into the ureters.

• Common features of AGN are oliguria, edema, hypertension, circulatory congestion, hematuria, and proteinuria.

• Therapeutic management of AGN involves maintenance of fluid balance and treatment of hypertension.

• Nephrotic syndrome is characterized by increased glomerular permeability to protein.

• Management of nephrotic syndrome is aimed at reducing excretion of protein, reducing or preventing fluid retention by tissues, and preventing infection and other complications. These are accomplished through dietary control, use of diuretics, corticosteroid therapy, and immunosuppressant therapy.

• Primary functions of the renal tubules are acidification of urine; potassium secretion; and selective and differential reabsorption of sodium, chloride, water, and other substances.

• The most common renal tubular disorders are renal tubular acidosis and NDI.

• Management of HUS is aimed at control of hematologic manifestations and complications of renal failure.

• In ARF management is directed at determining treatment of the underlying cause, managing the complications of renal failure, and providing supportive therapy.

• Abnormalities in CRF are waste product retention, water and sodium retention, hyperkalemia, acidosis, calcium and phosphorus disturbance, anemia, hypertension, and growth disturbances.

• When the child needs home dialysis, the nurse educates the family about the disease, its implications, the therapeutic plan, possible psychologic effects of the disease, and the treatment and technical aspects of the procedure.

• The major concerns in kidney transplantation are tissue matching, prevention of rejection, and psychologic concerns involving self-image as related to possible body changes resulting from the effects of corticosteroid therapy.

Urinary Tract Infection and Constipation

Nephrotic Syndrome