Chapter 49 - Genitourinary.docx

UNIT 12 Health Conditions of Children Genitourinary Conditions Mandy Rickard Originating US chapter by Marilyn J. Hockenberry http://evolve.elsevier.com/Canada/Perry/maternal OBJECTIVE S On completion of this chapter the reader will be able to: Demonstrate an understanding of diagnostic procedu...

UNIT 12 Health Conditions of Children Genitourinary Conditions Mandy Rickard Originating US chapter by Marilyn J. Hockenberry http://evolve.elsevier.com/Canada/Perry/maternal OBJECTIVE S On completion of this chapter the reader will be able to: Demonstrate an understanding of diagnostic procedures related to genitourinary disease. Describe the various factors that contribute to urinary tract infections in infants and children. Discuss the preoperative preparation and postoperative care of the child and parents when the child has a structural defect of the genitourinary tract. Demonstrate an understanding of the causes and mechanisms of edema formation in nephrotic syndrome. Outline a nursing care plan for a child with nephrotic syndrome. Compare the child with minimal-change nephrotic syndrome and the child with acute glomerulonephritis in terms of clinical manifestations and nursing care. Contrast the causes, complications, and management of acute and chronic renal failure. Demonstrate an understanding of the types of renal dialysis. Recognize signs of kidney transplant rejection. URINARY SYSTEM STRUCTURE AND FUNCTION The urinary system is an excretory system that consists of the kidneys, which produce urine; the ureters, which transport urine to the bladder; the urinary bladder which stores urine; and the urethra, which dis- charges urine from the bladder. Kidney Structure and Function The major purpose of the kidneys is to balance the body fluids, which include water and electrolytes. The kidneys must adapt to external and internal factors in order to maintain this equilibrium. These factors include diet, hydration variables, and extrarenal losses of water and sol- utes. Glomerular filtration produces urine, and tubular reabsorption creates equilibrium. Reabsorption occurs when substances are moved from the tubular lumen to the blood. Secretion is the movement of sub- stances back from the blood to the lumen. Kidneys remove metabolic wastes from the blood and excrete the wastes in urine. In addition, the kidneys help to regulate the production of red blood cells (RBCs) by converting plasma globulin into erythropoietin and stimulating erythropoiesis production in the bone marrow. A lack of erythropoietin decreases the number of RBCs and can lead to anemia when significant kidney disease is present. The kidneys also aid in cal- cium absorption and in maintaining blood volume, composition, and pH. The enzyme renin is secreted by the kidneys in response to a low- ered blood volume, lowered blood pressure, or an increase in the secre- tion of catecholamines. Renin stimulates the production of angiotensin, which creates constriction of the arterioles. This in turn leads to an increase in blood pressure and stimulates the production of aldosterone by the adrenal cortex. The functional unit of the kidney is the nephron. The nephron con- tains a renal corpuscle and a renal tubule. Kidney and nephron structures are illustrated in Figure 49.1. The corpuscle consists of the glomerulus and a Bowman’s capsule. The glomerular capillary receives blood from the afferent arteriole and passes it to the efferent arteriole. The efferent arte- riole gives rise to the peritubular capillary system and loops of Henle and then into collecting ducts. Nephrons function to remove wastes, such as urea from the blood, and to regulate water and electrolyte concentrations. Urine is formed when water and dissolved material are forced out of the glomerular capillaries. Filtration pressure is the force that moves materials out of the glomerulus and into the Bowman’s capsule via hydrostatic pres- sure inside glomerular capillaries. The glomerular filtration rate (GFR) is the amount of fluid filtered from the capillaries into the Bowman’s capsule of the kidneys. This filtration is affected by the osmotic pressure of the plasma, hydrostatic pressure in Bowman’s capsule, and the diameter of the efferent and afferent arterioles. The kidneys produce about 125 mL of glomerular fluid per minute, and most of this is reabsorbed. 1311 Interlobular Capsule (fibrous) arteries Renal column Cortex Renal sinus Minor calyces Hilum Major Glomerular filtration and absorption are relatively low in the infant and do not reach adult levels until the ages of 1 or 2 years. This may be related to the glomerular epithelia cells being more shaped like a cube, a high afferent arteriole resistance, and incomplete formation of the tubules and smaller glomeruli. As a result, the newborn is at risk of being unable to excrete excess water and solutes quickly or efficiently. Substances are selectively reabsorbed by the tubules; glucose and amino acids are reabsorbed by active transport, water is reabsorbed by osmo- sis, and proteins are reabsorbed by pinocytosis and returned to the blood. Excess of these substances may be excreted in the urine. Sodium is mostly reabsorbed before urine is excreted and is concentrated in the Renal pelvis Renal papilla of pyramid Ureter A Medulla calyces Medullary pyramid Renal corpuscle Renal tubule renal medulla. Antidiuretic hormone (ADH), also known as vaso- pressin, from the posterior pituitary gland causes the permeability of the distal tubule and collecting ducts to increase and promotes the reab- sorption of water. Electrolytes are moved by active transport and diffusion. There is a limit to the concentration gradient against which sodium can be trans- ported out; if there is a larger than normal amount remaining in the tubules, water stays with the sodium. GENITOURINARY DYSFUNCTION Clinical Manifestations As in most disorders of childhood, the incidence and type of kidney or urinary tract dysfunction change with the child’s age and maturation. In addition, the presenting health concerns and the significance of these concerns vary with age. For example, urinary tract infections have a greater significance in infancy than at an older age. In newborns, renal abnormalities may be associated with a number of other malforma- tions, for example, obvious neural tube defects to the subtle abnormal shape or position of the outer ear. Failure to thrive in children may be a sign of impaired renal function. While many of the clinical manifestations of renal disease are com- mon to a variety of childhood disorders, their presence is an indication to obtain further information from the child’s history, family history, and laboratory studies as part of a complete physical examination. Sus- pected renal disease can be further evaluated by means of radiographic studies and renal biopsy (Table 49.1). Laboratory Tests. Both urine and blood studies, including routine urinalysis, contribute vital information for the detection of renal con- ditions. Specific urine and blood tests provide additional information. Because nurses are usually the persons who collect the specimens for examination and who often perform many of the screening tests, they should be familiar with the tests, their functions, and the factors that can alter or distort the results of the tests. The major urine and blood tests are outlined in Tables 49.2 and 49.3. Nursing Care. Nursing responsibilities in assessment of genito- urinary disorders or diseases begin with observation of the child for any manifestations that might indicate dysfunction. Many conditions have specific characteristics that distinguish them from other disorders. These are discussed throughout the chapter. The nurse is generally responsible for preparing infants, children, and parents for tests and for collecting urine and (sometimes) blood specimens for observation and laboratory analysis (see Collection of Specimens, Chapter 44). An important nursing responsibility is to maintain careful intake and output and blood pressure measurements Fig. 49.1 A: Kidney structure. B: Components of the nephron. (From Patton, K. T., & Thibodeau, G. A. [2010]. Anatomy and physiology [7th ed.]. Mosby.) in children with genitourinary dysfunction and those who might be at risk for developing renal complications (e.g., children in shock, post- operative patients). For example, any significant degree of renal disease can diminish the GFR, a measure of the amount of plasma from which a TABLE 49.1 Radiological and Other Tests of Urinary System Function Comment and Nursing Test Procedure Purpose Responsibilities Urine culture and Collection of sterile specimen Determines presence of pathogens and the Send specimen to laboratory immediately sensitivity medications to which they are sensitive after collection. Use catheterization, midstream urine (MSU), or suprapubic aspiration specimen. Renal and bladder ultrasound Transmission of ultrasonic waves through renal parenchyma, along ureteral course, and over bladder Allows visualization of renal parenchyma and renal pelvis and any dilatation/ hydronephrosis without exposure to external beam radiation or radioactive isotopes Visualization of dilated ureters and bladder wall are also possible. Doppler ultrasound can be used to evaluate renal vascular flow. Noninvasive procedure Testicular (scrotal) ultrasound Transmission of ultrasonic waves through scrotal contents and testis Allows visualization of scrotal contents, including testis Testicular ultrasound is used to identify masses, and Doppler-enhanced ultrasound is used to differentiate hyperemia of epididymo-orchitis from ischemia or torsion. Noninvasive procedure Should not be routinely used for diagnosis of cryptorchidism (undescended testes) Scout film Flat plate roentgenogram of abdomen and pelvis for kidney, ureters, bladder (KUB) Detects and establishes renal outlines, presence of calculi, or opaque foreign bodies in bladder Prepare as for routine X-ray film. Voiding cystourethrography Contrast medium injected into bladder through urethral catheter until bladder is full; films taken before, during, and after voiding Visualizes bladder outline and urethra. Determines presence of vesicoureteral reflux (retrograde flow of urine into ureters/ kidneys), and shows complications of bladder emptying such as bladder outlet obstruction Prepare child for catheterization. Radionuclide (nuclear) cystogram Radionuclide-containing fluid injected through urethral catheter until bladder is full; images generated before, during, and after voiding Alternative to voiding cystourethrography in children with allergy to intravesical contrast material Allows evaluation of reflux, although visualization of anatomical details is relatively poor Prepare child for catheterization. Reassure patient and parents that allergic response to contrast materials is avoided by use of radionuclide. Radioisotope imaging studies (renal scans) Contrast medium injected intravenously; computer analysis to measure uptake or washout (excretion) for analysis of organ function DTPA radioisotope used to measure glomerular filtration rate; estimate of differential renal function and renal washout to determine presence and location of upper urinary tract obstruction DMSA radioisotope is used to visualize renal scars and differential renal function; it does not visualize ureters and bladder. MAG3 radioisotope combines features of DTPA (evaluation of upper urinary tract obstruction) with features of DMSA radioisotope (differential renal function and drainage time for assessment of obstruction). Insert or assist with insertion of intravenous infusion. Monitor intravenous infusion. Urethral catheterization may accompany DTPA radioisotope scan; prepare child for catheterization when indicated. Intravenous pyelography (IVP) (intravenous urogram; excretory urogram) Intravenous injection of a contrast medium Medium secreted and concentrated by tubules X-ray films made 5, 10, and 15 min after injection; delayed films (30, 60 min, etc.) are obtained if obstruction suspected Defines urinary tract Provides information about integrity of kidneys, ureters, and bladder Retroperitoneal masses visualized when they shift position of ureters Preparation for test: Infants <2 yr of age—Give no solid food, omit one breastfeeding/bottle on morning of examination; perform studies early to avoid withholding of fluids. Children 2–14 yr of age—Give cathartic evening before examination, nothing orally after midnight, enema (soapsuds) morning of examination. Rare test in pediatrics and not commonly requested Continued TABLE 49.1 Radiological and Other Tests of Urinary System Function—cont’d Test Computed tomography (CT) Procedure Narrow-beam X-rays and computer analysis providing precise reconstruction of area Purpose Visualizes vertical or horizontal cross-section of kidney Especially valuable to distinguish tumours and cysts Comment and Nursing Responsibilities Noncontrast scan is noninvasive. Contrast-enhanced CT scan preparation is similar to that for IVP. Cystoscopy Direct visualization of bladder and lower urinary tract through small scope inserted via urethra Investigation of bladder and lower tract lesions; visualizes ureteral openings, bladder wall, trigone, and urethra NPO orders per protocol, typically no solid food after midnight, liquids until 4 to 6 hr before procedure. Carry out preoperative preparations; cystoscopy is done under anaesthesia in children. Retrograde pyelography Contrast medium injected through ureteral catheter Visualizes pelvic calyces, ureters, and bladder Usually done in conjunction with a surgical procedure to delineate anatomy prior to surgery. Give cathartic if ordered. Give preoperative medication if ordered. Observe for reaction to contrast medium. Monitor vital signs after procedure. Renal angiography Contrast medium injected directly into Visualizes renal vascular system, especially for Prepare child for insertion of a spinal needle renal artery via catheter placed in renal arterial stenosis or perfusion catheter in renal pelvis femoral artery (or umbilical artery in (anaesthetic often required). newborn) and advanced to renal artery Renal biopsy Removal of kidney tissue by open or percutaneous technique for study by light, electron, or immunofluorescent microscopy Yields histological and microscopic information about glomeruli and tubules; helps distinguish between types of nephritic syndromes and identification of renal masses Distinguishes other renal disorders Usually done under anaesthesia in image guided therapy department. Urodynamics Set of tests designed to measure bladder filling, storage, and evacuation functions Uroflowmetry—Test to determine efficiency of urination Cystometrogram—Graphic comparison of bladder pressure as a function of volume Voiding pressure study—Comparison of detrusor contraction pressure, sphincter electromyelogram, and urinary flow Determine characteristic of voiding dysfunction Used to identify type (cause) of incontinence or urinary retention Usually reserved for assessment of bladder function in children with neurogenic bladder or congenital bladder outlet obstruction Prepare child for urinary catheterization. The bladder will be filled with saline solution and filling pressures will be recorded; the child may experience fullness, coolness from the saline fluid, and urine leakage during the study. To measure sphincter EMG, it may be necessary to insert needles. DMSA, Dimercaptosuccinic acid; DTPA, diethylenetriamine pentaacetic acid; EMG, electromyography; MAG3, mercaptoacetyltriglycine; NPO, nothing by mouth. TABLE 49.2 Urine Tests of Renal Function Test Normal Range Deviations Significance of Deviations Physical Tests Volume Age-related Polyuria Osmotic factors (urinary glucose level in diabetes mellitus) Infants—<1 mL/kg/hr Oliguria Retention caused by obstructive disease Inadequate bladder emptying caused by neurogenic bladder or obstructive disorder Children—0.5 mL/kg/hr Anuria Obstruction of urinary tract; acute renal failure Specific gravity With normal fluid intake— 1.016–1.022 Newborn—1.001–1.020 High Dehydration Presence of protein or glucose Presence of radiopaque contrast medium after radiological examinations Others— 1.001–1.030 Low Excessive fluid intake Distal tubular dysfunction Insufficient antidiuretic hormone Diuresis Chronic glomerular disease Continued TABLE 49.2 Urine Tests of Renal Function—cont’d Test Osmolality Normal Range Newborn— 274–305 mmol/kg H2O Thereafter— 282–300 mmol/kg H2O Deviations High or low Significance of Deviations Same as for specific gravity More sensitive index than specific gravity Appearance Clear pale yellow to deep gold Cloudy Contains sediment Cloudy reddish pink to reddish brown Blood from trauma or disease Myoglobin after severe muscle destruction Light Dilute Dark Concentrated Red Trauma Chemical Tests pH Newborn—5–7 Thereafter— 4.8–7.8 Average—6 Weak acid or neutral If associated with metabolic acidosis, suggests tubular acidosis If associated with metabolic alkalosis, suggests potassium deficiency Urinary infection Alkaline Metabolic alkalosis Protein level Absent Present Abnormal glomerular permeability (e.g., glomerular disease, changes in blood pressure) Most kidney disease Glucose level Absent Present Diabetes mellitus Infusion of concentrated glucose-containing fluids Glomerulonephritis Impaired tubular reabsorption Ketone levels Absent Present Conditions of acute metabolic demand (stress) Diabetic ketoacidosis Leukocyte esterase Absent Present Can identify both lysed and intact white blood cells via enzyme detection Nitrites Absent Present Most species of bacteria convert nitrates to nitrites in the urine Microscopic Tests White blood cell <1 or2 count >5 polymorphonuclear leukocytes/ field Urinary tract inflammatory process Lymphocytes Allograft rejection Malignancy Red blood cell count <1 or2 4–6/field in centrifuged specimen Trauma Stones Glomerular injury Infection Neoplasms Presence of bacteria Absent to a few >100 000 organisms/mL in centrifuged specimen Urinary tract infection Presence of casts Occasional Granular casts Tubular or glomerular disorders Cellular casts White blood cell Red blood cell Degenerative process in advanced renal disease Pyelonephritis Glomerulonephritis Hyaline casts Proteinuria; usually transient given substance is totally cleared in 1 minute. A number of substances can be used, but the most useful clinical estimation of glomerular fil- tration is the clearance of creatinine, an end product of protein metab- olism in muscle and a substance that is freely filtered by the glomerulus and secreted by renal tubular cells. The nurse’s responsibility in con- ducting this test is the collection of urine, usually a 12- or 24-hour specimen. GENITOURINARY TRACT DISORDERS AND DEFECTS Urinary Tract Infection Urinary tract infections (UTIs) occur in 1% of boys and 1 to 3% of girls. During the first year of life, UTIs are more common in males, partic- ularly in uncircumcised boys (Sorokan et al., 2015/2021). Circumcision status should be assessed in male infants with unexplained fever. TABLE 49.3 Blood Tests of Renal Function Normal Significance of Test Range Deviations Deviations Urea Newborn—2.9– Elevated Renal disease—acute or 10.0 mmol/L chronic (the higher the urea, Infant, child— the more severe the 2.0–7.1 mmol/L disease) Increased protein catabolism Dehydration Hemorrhage High protein intake Corticosteroid therapy Uric acid Child—120–360 mcmol/L Increased Severe renal disease Creatinine Infant—10–56 mcmol/L Child—<53 mcmol/L Adolescent— <98 mcmol/L Increased Severe renal impairment UTI may involve the urethra and bladder (lower urinary tract) or the kidneys (upper urinary tract). True UTIs involving the upper uri- nary tract are usually associated with fever, whereas lower urinary tract infections consist of lower urinary tract symptoms such as dysuria, uri- nary frequency, and urgency. The Canadian Paediatric Society (CPS) recommends suprapubic aspiration or urethral catheterization to diag- nose UTIs in young infants (see Chapter 44). Urine obtained by bag is frequently inaccurate because of contamination and should be avoided for UTI diagnosis (Robinson et al., 2014/2020). Because it is often impossible to localize the infection, the broad designation UTI is applied to the presence of significant numbers of microorganisms any- where within the urinary tract, except the distal third of the urethra, which is usually colonized with bacteria. Various terms used to describe urinary tract disorders include the following: Bacteriuria—Presence of bacteria in the urine Asymptomatic bacteriuria—Significant bacteriuria (usually defined as more than 100 000 colony-forming units [CFU]) with no evidence of clinical infection. May occur in children predisposed to colonization. Symptomatic bacteriuria—Bacteriuria accompanied by physical signs of UTI (dysuria, suprapubic discomfort, hematuria, fever) Recurrent UTI—Repeated episode of bacteriuria or symptomatic UTI Persistent UTI—Persistence of bacteriuria despite antibiotic treatment Febrile UTI—Bacteriuria accompanied by fever and other physical signs of UTI; presence of a fever typically implies a pyelonephritis Diagnosis of UTI requires obtaining a sterile specimen (in and out catheter, suprapubic aspiration in infants and in children who have not achieved toilet independence; midstream urine (MSU) in older children), a positive urinalysis (leukocyte esterase nitrites), positive routine and microscopy (+ white blood cell [WBC], + bacteria), and a positive urine culture (>50 000 CFU of a single organism) (Robinson et al., 2014/2020). Cystitis—Inflammation of the bladder; relatively uncommon in children Urethritis—Inflammation of the urethra Pyelonephritis—Inflammation of the upper urinary tract and kidneys Urosepsis—Febrile UTI coexisting with systemic signs of bacterial ill- ness; blood culture reveals presence of urinary pathogen Etiology. A variety of organisms can be responsible for UTI. Escher- ichia coli (80% of cases) and other Gram-negative enteric organisms are the most common uropathogens; these organisms are usually found in the anal and perineal region. Other organisms associated with UTI include Proteus, Pseudomonas, Klebsiella, Staphylococcus aureus, Haemophilus, and coagulase-negative Staphylococcus organ- isms. Most uropathogens originate in the gastrointestinal (GI) tract, migrate to the periurethral area, and ascend to the bladder. Several factors contribute to the development of UTI in childhood including anatomical, physical, and chemical conditions or properties of the child’s urinary tract. Anatomical and physical factors. The structure of the lower uri- nary tract is believed to account for the increased incidence of bacte- riuria in females. The short urethra, which measures about 2 cm in young girls and 4 cm in mature women, provides a ready pathway for invasion of organisms. In addition, the closure of the urethra at the end of micturition may return contaminated bacteria to the blad- der. The longer male urethra (as long as 20 cm in an adult) and the antibacterial properties of prostatic secretions inhibit the entry and growth of pathogens. However, when considering newborns and infants, risk stratification for development of UTI falls into three cat- egories: females, circumcised males and uncircumcised males, with the latter being at an increased risk of bacterial contamination of the bladder for the first several months of life. This is particularly true in the presence of urinary tract anomalies such as hydronephrosis, hydroureter, or vesicoureteral reflux (VUR). Under normal conditions the act of completely and repeatedly emp- tying the bladder (i.e., every 2 hours in children with toilet indepen- dence) flushes away any organisms before they have an opportunity to multiply and lead to symptoms. However, holding or infrequent voiding patterns or high postvoid residuals (PVRs) allow bacteria from the urethra to rapidly become established in the rich medium. Elevated PVR urines more commonly occur in girls and may be caused by dys- functional voiding (miscommunication between bladder and pelvic floor muscles), constipation, or urinary tract pathologies that affect the functioning of the bladder. Altered urine and bladder chemistry. Several mechanical and chemical characteristics of the urine and bladder mucosa help maintain urinary sterility. An increased fluid intake promotes flushing of the normal bladder and lowers the concentration of organisms in the infected bladder by encouraging more frequent voiding. Diuresis also seems to enhance the antibacterial properties of the renal medulla. Most pathogens favour an alkaline medium. Normally, urine is slightly acidic, with a median pH of 6.0. A urine pH of about 5 hampers but does not eliminate bacterial multiplication. Much has been reported about the use of cranberry products to increase urine acidity in an effort to prevent UTI. Studies done in adults offer limited evidence for the value of cranberry products in promoting urinary tract health. A Cochrane Review concluded that the benefit of cranberry juice was too small to recommend its use for prevention of UTIs (Jepson et al., 2012). Diagnostic Evaluation. The clinical manifestations of UTI depend on the child’s age (Box 49.1). Diagnosis of UTI is confirmed by detec- tion of bacteriuria in urine culture combined with positive urinalysis, microscopy, and symptom development. But urine collection is often difficult, especially in infants and very small children. Unless the spec- imen is a first-morning sample, a recent high fluid intake may indicate BOX 49.1 Clinical Manifestations of Urinary Tract Infections Birth to 1 Month Fever Poor feeding Vomiting Failure to gain weight Rapid respiration (acidosis) Respiratory distress Spontaneous pneumothorax or pneumomediastinum Weak urine stream Jaundice Seizures Dehydration Other anomalies or stigmata Enlarged kidneys or bladder to 24 Months Poor feeding Vomiting Failure to gain weight Excessive thirst Foul-smelling urine Pallor Fever Seizures (with or without fever) Dehydration Enlarged kidneys or bladder to 14 Years Poor appetite Vomiting Growth failure Excessive thirst Painful urination Swelling of face Seizures Pallor Fatigue Blood in urine Abdominal or back pain Edema Hypertension Tetany a falsely low organism count. Thus, children should not be encouraged to drink large volumes of water in an attempt to obtain a specimen quickly. NURSING ALERT A child who exhibits the following should be evaluated for UTI: Fever with no obvious source Dysuria Strong-smelling urine in association with other symptoms Frequency or urgency The most accurate estimates of bacterial content are obtained from suprapubic aspiration (in children younger than 2 years of age) or properly performed in and out bladder catheterization (as long as the first few millilitres are excluded from collection). Tests to detect bacteriuria are being used with increasing frequency in screening for UTI. The dipstick tests for leukocyte esterase and nitrites are quick and inexpensive methods for detecting infection before obtaining final culture results. In the absence of a positive uri- nalysis, routine culture and sensitivity testing is not routinely advised because of the risk of false positive results and unnecessary treatment with antibiotics. Once infection has been confirmed, other tests such as ultrasono- graphy, voiding cystourethrogram (VCUG), intravenous (IV) pyelo- gram, and dimercaptosuccinic acid (DMSA) scan may be performed after the infection subsides, to identify anatomical abnormalities and existing kidney damage from recurrent infection. Localization of the infection site may involve more specific tests, including percutaneous kidney taps and bladder washout procedures; however, these tests are exceedingly rare in children. Therapeutic Management. The objectives of treatment of children with UTI are to (1) eliminate current infection, (2) identify contributing factors to reduce the risk of recurrence, (3) prevent systemic spread of the infection, and (4) preserve renal function. Antibiotic therapy should be initiated on the basis of identification of the pathogen, the child’s history of antibiotic use, and the location of the infection. Several anti- microbial medications are available for treating UTI, but all of them can occasionally be ineffective because of resistance of organisms. Common anti-infective medications used for UTI include the penicillins, sulfon- amide (including trimethoprim and sulfamethoxazole in combination), the cephalosporins, and nitrofurantoin. The CPS has recommended significant changes in managing UTIs, given new research findings in children over 2 months of age until 24 months of age with an acute UTI and no underlying urinary tract issues or risks for a neurogenic bladder (Robinson et al., 2014/2020). The recommendations include antibiotic treatment for 7 to 10 days for a febrile UTI. Oral antibiotics may be prescribed initially if the child is not seriously ill. Children who are over 2 years of age should have a renal/bladder ultrasound with first febrile UTI to rule out any signifi- cant renal abnormalities. A voiding cystourethrogram is not required for children with a first UTI unless the renal/bladder ultrasound is abnormal and suggestive of renal anomalies or obstructive uropathy. It is important to carefully separate UTIs in children less than 24 months of age and those in older children, as the management of infection, the prevention of future infections, and the diagnostic workup for UTI is different between these groups. UTI in older children who develop a UTI after developing toilet independence can be usually managed with conservative measures and aggressive management of bladder and bowel dysfunction. Even if structural abnormalities are detected (such as VUR), most cases will not require any invasive man- agement of these issues (Lorenzo et al., 2019). In infants, if anatomical defects such as VUR or bladder neck obstruction are present, surgical correction of these abnormalities may be necessary to prevent recurrent infection. Ongoing follow-up is an important component of medical management for monitoring and prevention of recurrence of infections. Vesicoureteral reflux. Vesicoureteral reflux (VUR) refers to the abnormal retrograde flow of urine from the bladder, through the ure- ters to the kidneys. During filling, the bladder is a low-pressure reser- voir; however, once full, the mechanism of the bladder contracting causes it to become high pressure. With VUR, the ureters are not tun- nelled into the bladder mucosa in a standard fashion, which allows this high-pressure contraction to force urine back through the ureters and into the kidneys. When the bladder is colonized with bacteria, this may predispose children to UTIs. Primary reflux results from congenitally abnormal insertion of ureters into the bladder; secondary reflux occurs as a result of chronically high bladder pressures, persistent PVRs, or voiding dysfunction. VUR itself does not cause UTIs. In the presence of reflux, infected urine (bacteria) from the bladder has access to the kidney, resulting in kidney infection (pyelonephritis). These children are usually very symptomatic with high fever, vomiting, and chills. Reflux, when asso- ciated with UTI, is the most common cause of renal scarring in chil- dren. Renal scarring may occur with the first episode of febrile UTI; however, one infection does not necessarily equate to one scar (RIVUR Trial Investigators, 2014). VUR is managed conservatively with daily low-dose antibiotic therapy in infants, typically with trimeth- oprim 2 mg/kg/day, usually until toilet independence has been achieved. Urine should only be checked if the child is symptomatic for UTI as routine urine cultures risk growing contaminants and over- treatment with antibiotics. Routine VCUGs are not commonly employed because of the invasiveness of the test, and most cases of VUR are not clinically significant. Some evidence suggests that children may outgrow VUR over time. Current management of VUR is conservative with monitoring and prophylaxis. A small subset of children with VUR may require surgical management in the instance of breakthrough UTIs (i.e., infection while on prophylaxis) or UTIs after cessation of prophy- laxis. Surgical management consists of a minimally invasive endo- scopic injection of a bulking agent at the ureterovesical junction, which prevents reflux from occurring. Overall cure rates relate to the degree of reflux and range from 72 to 96% (Chen et al., 2010). An alternative surgical procedure is a ureteric reimplantation, which can be done open or laparoscopically and involves tunnelling the ureters into the bladder. It is considered the definitive procedure for correction of reflux. However, given the invasiveness of this pro- cedure and the potential for complications it is reserved for a very small subset of patients with VUR. Prognosis. With prompt and adequate treatment at the time of diag- nosis, the long-term prognosis for UTI is usually excellent. The hazard of progressive renal injury is greatest when infection occurs in young children (especially those younger than 2 years of age) and is associated with congenital renal malformations or reflux. Therefore, early diagno- sis of children at risk is particularly important. Nursing Care. Nurses should instruct parents to consider the possi- bility of UTI should their child have a fever with no obvious source, especially in children with urinary tract abnormalities that may predis- pose them to infections. This is more important in infants as fever may be the only sign of a UTI. In general, any infant less than 3 months of age should be assessed in an emergency department where screening for UTI will occur as a part of standardized workup. Fussiness, crying, poor feeding, and vomiting could also be presenting signs of UTI in infants. Older children, particularly once they have achieved toilet inde- pendence, may experience more traditional signs of UTI, such as dys- uria, frequency, urgency, and fever. When infection is suspected, collecting an appropriate specimen is essential. It is the nurse’s responsibility to take every precaution to obtain acceptable clean-voided specimens so that the use of other, more invasive collecting procedures can be avoided except when absolutely indicated. Given the unreliability of a specimen obtained via a urine collection bag, suprapubic aspiration of urine or sterile catheterization should be done in the infant or young child who has fever. Frequently, additional tests are performed to detect anatomical defects. Children need to be prepared for these tests as appropriate for their age. This includes an explanation of the procedure, its purpose, and what the child will experience (see Preparation for Diagnostic and Therapeutic Procedures, Chapter 44). Sometimes a simple description of the urinary system is helpful. For children younger than 3 to 4 years of age, the procedure can be explained on a doll. For those who are older, a simple drawing of the bladder, urethra, ureters, and kidneys makes the procedure more understandable. Handling actual equipment, when feasible, can be helpful in allaying anxiety in children of all ages. Anticipatory instruction on distraction techniques such as deep breathing, storytelling, and imagery may help the child relax during the actual procedures. If surgery is indicated, the child’s understanding of the procedure can help decrease their fear and anxiety concerning more extensive medical-surgical intervention. Because antibacterial medications are indicated in treating UTI, the nurse needs to advise parents on proper dosage and administration. When antibiotics such as nitrofurantoin are used for prolonged therapy to maintain urine sterility, parents need an explanation of the medica- tion’s continued necessity when no signs of infection are present. For all children an adequate or increased fluid intake should be encouraged. One adverse effect that can occur with antibiotic treatment is antibiotic-associated diarrhea (AAD). The CPS defines AAD as three or more loose stools per day for 2 or more days occurring for up to 2 weeks after the initiation of antibiotics; it occurs in about 30% of patients. Parents should be made aware that probiotics (specifically Lactobacillus rhamnosus GG and Saccharomyces boulardii) may decrease the incidence of AAD (Marchand & Canadian Paediatric Society, Nutrition and Gastroenterology Committee, 2012/2019). Prevention. Prevention is the most important goal in both primary and recurrent infection, and most preventive measures are simple hygienic habits that should be a routine part of daily care (see Guidelines box: Prevention of Urinary Tract Infection and Clinical Reasoning Case Study: Urinary Tract Infection and Constipation). Sexually active females should be advised to urinate as soon as pos- sible after they have intercourse, to flush out bacteria introduced during the activity. Prevention of Urinary Tract Infection CLINICAL REASONING CASE STUDY Urinary Tract Infection and Constipation Obstructive Uropathy Structural or functional abnormalities of the urinary system that obstruct the normal flow of urine can lead to dilatation of the urinary tract (kidneys or ureters). This dilatation is referred to as hydro- nephrosis, which is not an illness or disease but a finding on ultrasound. This is detected prenatally in one in five pregnancies and is self-limiting Ureteropelvic valve Ureteropelvic stricture Fibrous band Stenosis Ureteral orifice Urethral sphincter muscle in urogenital diaphragm Polycystic kidney Hydronephrosis Dysplasia-agenesis of ureter Posterior vesicoureteral valve Posterior vesicourethral valve Urethral stenosis in up to 85% of children (Rickard et al., 2017). In some children, the dilatation may be due to an underlying pathology such as obstruction, which may require a surgical intervention. Obstruction may be congenital or acquired, unilateral or bilateral, complete or incomplete, with acute or chronic manifestations. The obstruction can occur at any level of the upper or lower urinary tract (Figure 49.2). Partial obstruction may not be symptomatic, and changes caused may be partially or completely reversible if there is early interven- tion. Boys are affected more frequently than girls, and malformations are more common in children with other congenital abnormalities (e.g., prune belly syndrome, chromosomal anomalies, anorectal malforma- tions, defects of the pinna of the ear). If the obstruction is not diagnosed or left untreated, damage to distal nephrons in chronic uropathy alters the ability to concentrate urine, contributing to increased urine flow and metabolic acidosis occurring from the decreased excretion of acid secondary to the impaired ability of the distal nephron to secrete hydrogen ions. In addition, static urine serves as a medium for bacterial growth; therefore, UTIs may be more likely in children with urinary tract dilatation, which eventually may further increase the extent of renal damage. Most cases of urinary tract obstruction in children born in devel- oped countries are detected prenatally, and any surgical correction required is carried out before deterioration of renal function. Typically, surgical procedures to relieve obstruction are performed (such as pyeloplasty or ureterovesicostomy, or ureterostomy) instead of using external drainage devices as these may make future surgical correction more difficult. Medical complications of acute or chronic renal failure (chronic kidney disease [CKD]) or infection are managed as described later for those disorders. Nursing Care. Nursing goals in urinary tract obstruction include assistance in its identification, assisting with diagnostic procedures, and caring for children with complications. Preparing parents and chil- dren for diagnostic procedures is a major nursing responsibility, as is Fig. 49.2 Major sites of urinary tract obstruction. preparation for surgical procedures (see Preparation for Diagnostic and Therapeutic Procedures, Chapter 44). Parents and children need emotional support and counselling dur- ing the lengthy management of these disorders. Children may be dis- charged with urinary drainage systems that require nursing education of the parents and older child regarding home care as well as recogni- tion of complications, such as obstruction of urine flow or infection. Drainage tubes should be observed for obstruction resulting from sed- iment, small blood clots, or kinking. If indicated, instructions on site care and drainage tube irrigation need to be provided, including obser- vation for signs of infection or dislodgement. Those with progressive renal deterioration may face the prospect of dialysis or transplantation, as well as the emotions that accompany these procedures. External Defects Defects of the external genitourinary tract are serious conditions, pri- marily because some require multiple operations, interprofessional care, and lifelong follow-up, which may affect the psychological well- being of the child. The major anomalies of the lower genitourinary tract, their description, and their management are summarized in Table 49.4. Phimosis. Phimosis refers to tight foreskin that precludes retraction over the glans. Phimosis can be classified as physiological or patho- logical. Physiological phimosis is a normal finding in infants and young boys and usually resolves as the child grows and may persist until ado- lescence. Ballooning with voiding commonly occurs in boys with phi- mosis and does not require treatment. Pathological phimosis occurs TABLE 49.4 Anomalies of the Genitourinary Tract Anomaly Inguinal hernia—Protrusion of abdominal contents through inguinal canal into scrotum Therapeutic Management Detected as painless inguinal swelling of variable size Surgical closure of inguinal defect Hydrocele—Fluid in scrotum that fluctuates in size Surgical repair indicated if spontaneous resolution does not occur by 24 mo of age Phimosis—Tight, nonretractile foreskin; Physiological—Normal with healthy supple foreskin Pathological occurs as a result of scarring from forceful retraction, recurrent infection (balanitis/balanoposthitis) or a skin condition called balanitis xerotica obliterans (BXO) Physiological—Gentle stretching with or without the application of topical steroid ointment Pathological—Circumcision Hypospadias—Urethral opening located on the ventral aspect of penis Objectives of surgical correction: Enable child to void in standing position and direct stream voluntarily in usual manner Appearance of a circumcised penis Sexually functional Chordee—Ventral curvature of penis, often associated with hypospadias Surgical repair Epispadias—Meatal opening located on dorsal surface of penis Surgical correction, usually including penile and urethral lengthening and bladder neck reconstruction (if necessary) Cryptorchidism—Failure of one or both testes to descend normally through inguinal canal Detected by inability to palpate testes within scrotum Surgical—Orchiopexy Objectives of therapy: Optimize testicular function Prevent damage to undescended testicle Decrease incidence of malignant tumour formation that, while rare, has been reported to be increased with untreated undescended testes, particularly those that are intra-abdominal Avoid trauma and torsion Close inguinal canal Exstrophy of bladder—Eversion of posterior bladder through anterior bladder wall and lower abdominal wall; associated with open pubic arch (a severe defect) Potential objectives of surgical correction: Preserve renal function Attain urinary control Perform adequate reconstructive repair Improve sexual function (especially in males) secondary to a chronic inflammatory process, forceful retraction result- ing in scarring, or recurrent episodes of infections. Pathological phimo- sis has a characteristic appearance of white scar tissue around the preputial opening and will not improve on its own. A circumcision is required for management of pathological phimosis. Balanitis is an inflammation or infection of the glans penis. Balano- posthitis is infection of phimotic foreskin, which is usually managed with topical treatments and, rarely, oral antibiotics. See Table 49.4 for more information. Nursing care. Proper hygiene of the phimotic foreskin in infants and young boys consists of external cleansing during routine bathing. The foreskin should not be forcibly retracted, because it may create scarring that can prevent future retraction. Furthermore, retraction of the tight foreskin can result in paraphimosis, a condition in which the retracted foreskin cannot be replaced in its normal position over the glans. This causes edema and venous congestion created by con- striction by the tight band of foreskin—a urological emergency that requires immediate evaluation. Hydrocele. A hydrocele is the presence of peritoneal fluid in the scrotum between the parietal and visceral layers of the tunica vaginalis and is the most common cause of painless scrotal swelling in children and adolescents. Hydroceles may be communicating or noncommunicating. A communicating hydrocele usually develops when the processus vaginalis does not close during development, allow- ing for communication with the peritoneum. Noncommunicating hydroceles have no connection to the peritoneum with fluid coming from the mesothelial lining of the tunica vaginalis. Hydroceles are com- mon in newborns and often resolve spontaneously, usually by 24 months of age. In older children, noncommunicating hydroceles may be idiopathic or a reactive hydrocele that occurs secondary to scro- tal issues such as trauma. Communicating hydroceles may change in size during the day or with straining, whereas noncommunicating hydroceles are not reduc- ible and do not change size with crying or straining. Surgical repair is indicated for communicating hydroceles persisting past 2 year of age, because there is a risk for development of incarcerated inguinal hernia. Idiopathic hydroceles are repaired if symptomatic, and reactive hydro- celes usually resolve with treatment of the underlying cause, such as epididymitis. Nursing care. Surgical correction is an outpatient procedure. Par- ents should be advised that there may be temporary swelling and discoloration of the scrotum that resolves spontaneously. The child should avoid using straddle toys for 2 to 4 weeks, and strenuous activ- ities in older boys may need to be avoided for 1 month. If a dressing is used, it is removed in 2 to 3 days, and typically the child can bathe in 3 days. Cryptorchidism (Undescended Testes). Cryptorchidism is failure of one or both testes to descend normally through the inguinal canal into the scrotum. The incidence of cryptorchidism is common in pre- mature babies, multiple births, and other genetic conditions. The testes spontaneously descend by 6 months of age in most cases. Absence of testes within the scrotum can be a result of undescended (cryptorchid) testes, retractile testes, or anorchism (absence of testes). Anorchism is the complete absence of a testis. Anorchism is suspected whenever one or both testes cannot be palpated in the patient with apparent cryptorchidism. In some cases, bilateral anorchism is associated with disorders of sex development with genotypic and phenotypic abnormalities, specifically congenital adrenal hyperplasia (CAH). Retractile testes can be found at any level within the path of testic- ular descent, but they are most commonly identified in the groin. For- tunately, they are not truly cryptorchid. Instead, they are introverted to an inguinal or abdominal position because of an overactive cremasteric reflex. The cremasteric reflex, observed as withdrawal of the testis above the scrotum and into the inguinal canal in response to various stimuli, including exposure to cool temperatures, is active during infancy and peaks around 4 to 5 years of age. Unlike the cryptorchid testis, the retractile testis can be gently moved into the scrotum without residual tension and does not require treatment. Retractile testes can become ascending testes and require annual monitoring. Therapeutic management. Orchiopexy, or surgical repositioning of the testis, is performed on palpable testes. Exploratory surgery may be required if the testis is not palpable. The goal of surgery is to place and fix viable undescended testes to a normal scrotal position or to remove nonviable testicular remnants. Scrotal positioning reduces the risk for torsion and trauma and permits easier examination of the testis, because there is an increased risk for testicular cancer despite treatment of undescended testes. In the routine surgical procedure for unde- scended testes, the testes are brought down into the scrotum and secured in that position without tension or torsion. A simple orchio- pexy for a palpable testis can usually be performed as an outpatient pro- cedure. If exploratory surgery is needed to determine if a testis is present, an examination under anaesthesia is the initial step. Depend- ing on findings, a diagnostic laparoscopic procedure or an open ingui- nal approach may be performed. If an intra-abdominal testis is identified, a definitive procedure follows, which may be open or lapa- roscopic. Approximately 10% of boys with nonpalpable testes are found to have an absent testicle at the time of surgery. Nursing care. Postoperative nursing care is directed toward pre- venting infection and instructing parents in home care of the child, including pain control. Observation of the wound for complications and activity restrictions are discussed. The child should avoid vigorous activities and use of straddle toys for 2 to 4 weeks postoperatively. Gen- eral care is similar to that described for hydrocele repair. Parents may be concerned about the child’s future fertility. Recent studies show some decreased fertility in bilateral cryptorchism, but in patients with unilateral cryptorchism the fertility rate approximates that found in the general population. The risk for testicular cancer is a concern, which is decreased if surgery is done before puberty, but all boys with cryptorchidism should be taught testicular self-examination at puberty to facilitate early detection (Kolon et al., 2014). Surgical treatment is indicated as soon as possible after 6 months of age and should be completed by 2 years of age because spontaneous descent rarely occurs after 6 months of age, and treatment by 1 to 2 years of age is associated with improved fertility and testicular growth. Hypospadias and Epispadias. Hypospadias is a common congen- ital defect of the penis that occurs in approximately 1 in 250 males (Elder, 2020b). There are three characteristics typically associated with hypospadias: meatal position that can open below the glans penis or anywhere along the ventral surface of the penis, the scrotum, or the per- ineum; ventral skin deficiency with a hooded dorsal foreskin; and ven- tral curvature/chordee. Mild cases of hypospadias (Figure 49.3) may be repaired for cos- metic reasons and usually involve a single surgical procedure. More severe forms of hypospadias (e.g., proximal or severe chordee) may require a staged repair; surgical repair is recommended in order to min- imize functional disturbances in the future. The surgical goals of hypo- spadias repair are a normally positioned urethral meatus, a straight penis when erect, the ability to void standing up, and healthy sexual function. Infants born with hypospadias should not be circumcised, as the procedure is much more complex than a standard newborn cir- cumcision. Also, the foreskin is used during the urethral reconstruc- tion, which may take place once the child is older than 6 months of age. Any infant born with a suspected hypospadias should be referred to a pediatric urologist. Epispadias results from failure of urethral canalization. About 55% of the affected infants are boys who have a widened pubic symphysis and a broad, spadelike penis with the urethra opening on its dorsal sur- face. Girls have a wide urethra and a bifid clitoris. Severity ranges from mild anomaly to a severe form associated with exstrophy of the bladder. Surgical correction is usually necessary. Affected male infants should not be circumcised. Exstrophy of the Bladder. Bladder exstrophy (Figure 49.4) is rare, occurring only in about 1 in 35 000 to 40 000 live births (Elder, 2020a). It often occurs in conjunction with epispadias and is more com- mon in males than in females. It results from abnormal development of the bladder, abdominal wall, and symphysis pubis that causes the blad- der, urethra, and ureteral orifices to all be exposed due to the abdominal wall defect. The bladder is visible in the suprapubic area as a red mass with numerous folds, with urine draining from it onto the infant’s skin. Immediately after birth, the exposed bladder is covered with a ster- ile, nonadherent dressing to protect it until closure can be performed. Fig. 49.3 Hypospadias. (Courtesy H. Gil Rushton, MD, Children’s National Medical Center.) Fig. 49.4 Exstrophy of bladder. (Courtesy H. Gil Rushton, MD, Children’s National Medical Center.) Historically, early multidisciplinary surgery was performed within the newborn period, usually within the first several days of life. It has now become more common to wait until infants are several months old before attempting the repair. Disorders of Sex Development. A disorder of sex development (DSD) in the newborn (Figure 49.5) often is discovered by the nurse during a physical assessment. Erroneous or abnormal sexual differen- tiation may be a genetic defect, such as congenital adrenal hypoplasia, which can be life-threatening because it involves deficiency of all adre- nocortical hormones. Other possible causes of DSD include chromo- somal abnormalities, defective sex hormone synthesis in males, and the placental transfer of masculinizing agents to female fetuses. Gender assignment should be based on data gathered from the following Fig. 49.5 Disorder of sex development (i.e., structure may be enlarged clitoral hood and clitoris or micropenis and bifid scrotum). (Courtesy Edward S. Tank, MD, Division of Urology, Oregon Health Sciences University.) sources: family history, including the ingestion of steroids during preg- nancy and relatives who had DSD or who died during the newborn period; physical examination; chromosomal analysis (results are avail- able in 2 or 3 days); endoscopy, ultrasonography and radiographic con- trast studies; biochemical tests, such as analysis of urinary steroid excretion, which helps detect several of the adrenocortical syndromes; and, in some instances, laparotomy or gonad biopsy. The infant and child with DSD pose very complex management questions, including sex assignment and potential genital surgery. Traditional approaches are being questioned and continue to evolve. Infants should be referred to an interprofessional team consisting of urology, endocrinology, gen- eral surgery, psychiatry, and social work as soon as possible to ensure timely and accurate diagnosis. Therapeutic intervention, including any counselling and surgery, should be started as soon as possible. Any child born with DSD should not receive gender assignment until a proper assessment has been done. Gender assignment is a complex topic and should be based on age at presentation, potential for mature sexual function, potential fertility, and the long-term psychological and emotional impact on the child and family. Parents need much support as they learn to address this challenging situation. Psychological Issues Related to Genital Surgery. Surgery involving sexual organs can be particularly disruptive to children, espe- cially preschoolers and school-age children. Some of the difficulties of hospitalization, separation, and anxiety can be eased by hospital prac- tices that are sensitive to the child’s needs (see Chapter 43). A child’s body image is largely derived as a result of feedback from the primary caregivers, and parental anxiety regarding an acceptable physical appearance and adequate future sexual competency is readily communicated to an affected child. Children with birth defects are at risk for developing a distorted body image that reflects the caregiver’s subtly communicated evaluation of their bodies. After extensive review of the emotional, cognitive, and body image issues that may occur in children undergoing surgical recon- struction of a genital abnormality, if surgery is indicated, it is recom- mended that surgery be accomplished between the ages of 6 and 15 months, to minimize the psychological effects of surgery and anaesthesia. Nursing care. Preparing children and their families for diagnostic and surgical procedures (see Preparation for Diagnostic and Therapeu- tic Procedures, Chapter 44) and for home care is a major nursing func- tion. Most postoperative care involves care of the surgical site. Tub baths are discouraged for 1 week after simple surgeries. The surgical site needs to be kept clean and otherwise protected from infection and should be inspected for signs of infection. Dressings, if any, should be inspected regularly. More complex surgeries require additional care and observation (e.g., catheter care for urethral reconstruction and care of urinary diversion stomas and collection devices). Some older children’s activities, such as pushing, lifting, playing with straddle toys or in sandboxes, swimming, and engaging in rough activities, may be restricted after some types of surgical repairs. Precise restrictions depend on the specific type of surgery. Activities of infants and toddlers are not limited. In most cases the results of surgery are satisfactory. However, in some of the more severe anomalies, such as exstrophy and those that require stomas, additional emotional interventions may be needed. A major concern of parents and children is related to surgery affecting the genitalia directly. Concerns about penis size, appearance of the gen- italia, potential ability to procreate, and rejection by peers are potential fears that require psychological adjustment, particularly during adolescence. GLOMERULAR DISEASE Nephrotic Syndrome Nephrotic syndrome is a clinical state that includes massive proteinuria, hypoalbuminemia, hyperlipidemia, and edema. The disorder can occur as (1) a primary disease known as idiopathic nephrosis, childhood nephrosis, or minimal-change nephrotic syndrome (MCNS); (2) a sec- ondary disorder that occurs as a clinical manifestation after or in asso- ciation with glomerular damage that has a known or presumed cause; or (3) a congenital form inherited as an autosomal recessive disorder. The disorder is characterized by increased glomerular permeability to plasma protein, which results in massive urinary protein loss. The glo- merulus is responsible for the initial step in the formation of urine, and the filtration rate depends on an intact glomerular membrane. This dis- cussion