Peds - Exam 2

CoA clinical manifestations

1. *BP is higher in the arms than in the legs* 2. *Bounding pulses in arms with weak or absent pulses in legs* 3. Cool lower extremities 4. CHF in infants with *potential for severe acidosis and hypotension* 5. Older children may experience *dizziness, HA, fainting, and epistaxis r/t HTN* 6. Patients are *at risk for HTN, ruptured aorta, aortic aneurysm, and stroke*

AV canal clinical manifestations

1. *CHF is most severe with complete AV canal* 2. Mild cyanosis that increases with crying 3. Patients are at high risk for developing pulmonary vascular obstructive disease

3 types of nephrotic syndrome

1. Primary: idiopathic nephrosis, childhood nephrosis, or minimal change nephrotic syndrome (MCNS) - 80% of cases of nephrotic syndrome result from MCNS 2. Secondary: occurs after glomerular damage 3. Congenital: autosomal recessive gene causes the hereditary form of nephrotic syndrome

Nephrotic syndrome labs

1. *3-4+ proteinuria; 3 ½ g* 2. Serum protein reduced 3. *Serum albumin < 2.5* 4. Plasma lipids and cholesterol increased 5. BUN and specific gravity increased 6. Hgb and hct are usually normal or elevated 7. Platelet count is high (500,000-1,000,000) as a result of hemoconcentration 8. Sodium and calcium decreased

Enuresis treatments

*Successful treatment is defined as a specified period of dry nights, varying from 7-28 nights* 1. Conditioning therapy involves training the child to awaken to urinate after a stimulus is given, especially with a urine alarm - When moisture is detected, the buzzer alarms and awakens the child 2. Retention control training: child drinks fluids while awake then delays urination to stretch the bladder to accommodate larger volumes of urine 3. Kegel/pelvic muscle exercises may be helpful in children with daytime enuresis 4. In the waking schedule treatment, the child is awakened during night at intervals to void 5. *Drug therapy is effective but considered a LAST resort* - DDAVP, antidiuretics, anticholinergics, antispasmodics, and tricyclic antidepressants

Erikson's stage of development for toddlers

*Autonomy vs. shame and doubt* - Independence is paramount for toddlers, who are attempting to do everything for themselves - Separation anxiety continues to occur when parents leave toddlers - Infants begin to discover that their behavior is their own and has an effect on others - Although they are aware of their will and control over others, they are confronted with the conflict of exerting autonomy and relinquishing dependence on others - Exerting their will has negative consequences whereas retaining dependent, submissive behavior is generally rewarded with affection and approval - They feel shame for feeling the urge to revolt against others will and fear that they will exceed their own capacity for manipulating the environment - *Children express social modalities of letting go on play activities such as casting or throwing objects, taking objects out of drawers, boxes. Holding on tighter when someone says "no do not touch"* - Development of ego (reason, common sense) vs superego (conscience; aware of ability to achieve and fail → shame/doubt)

Tetralogy of Fallot (TOF)

- *4 defects: VSD, pulmonic stenosis, overriding aorta, and RV hypertrophy* - Because the VSD is usually large, pressure may be equal in both ventricles - Shunt direction depends on the difference between PVR & SVR - If PVR is higher → right-to-left shunt - If SVR is higher → left-to-right shunt - *Pulmonic stenosis → decreased blood flow to the lungs → decreased amount of oxygenated blood that returns to the left side of the heart* - Depending on the position of the aorta, blood from both ventricles may be distributed systemically *(oxygenated + unoxygenated blood delivered to body)*

Ventricular Septal Defect (VSD)

- *Abnormal opening between the ventricles* - VSDs are commonly associated with other defects - Many VSDs (20-60%) *close spontaneously during the first year of life* in children with small to moderate defects - *Blood flows from the higher pressure LV → RV → pulmonary artery → increased flow to the lungs* - Increased pressure in the RV causes *RV hypertrophy* - *RA may also enlarge* as a result of incomplete RV emptying

Truncus arteriosus interventions

- *Early repair is performed in the first month of life - involves closing the VSD and excising the pulmonary arteries from the aorta and attaching them to the RV by homograft* (segments of cadaver aorta and pulmonary artery) - Homografts are preferred to synthetic conduits; they are more flexible and less prone to obstruction - *Post op complications include persistent HF, bleeding, pulmonary HTN, dysrhythmias, and residual VSD.* - Because conduits are not living tissue, they will grow with the child and may also become narrowed with calcifications → *1 or more conduit replacements will be needed in childhood* - Mortality > 10%; future surgeries required to replace conduits

Coarctation of the Aorta (CoA)

- *Narrowing of the aorta* which results in *increased pressure proximal to the defect* (head and upper extremities) and *decreased pressure distal to the obstruction* (body and lower extremities)

Pulmonary Stenosis

- *Narrowing of the pulmonary artery* - Resistance causes *RV hypertrophy and decreased pulmonary blood flow* - *RV failure → increased RA pressure → foramen ovale reopens → unoxygenated blood shunted into LA → systemic cyanosis* - If stenosis is severe, HF occurs, and systemic venous engorgement is noted - An associated defect such as *PDA partially compensates for the obstruction by shunting blood from the aorta → pulmonary artery → lungs*

Aortic Stenosis

- *Narrowing or stricture of the aortic valve causing resistance to blood flow in the LV* - The extra workload on the LV causes *LV hypertrophy*

Transposition of the Great Arteries (TGA)

- *Pulmonary artery is connected to the LV and the aorta is connected to the RV, with no communication between the systemic and pulmonary circulations* - *Patent foramen ovale (most common) or PDA must be present* to permit blood to enter the systemic or pulmonary circulation *for mixing of saturated and desaturated blood* - Another associated defect may be a VSD, which increases the risk of HF

VUR repair & post-op care

- *There is a high rate of spontaneous resolution over time in those less than 1 year of age and lower grades of VUR* 1. Surgical management of VUR corrects the anatomy at the insertion of the refluxing ureter into the bladder - Consists of open surgical correction with reimplantation or endoscopic correction 2. Surgical intervention is *indicated in patients who are unlikely to resolve their reflux and are at risk for renal scarring* (those with grade 5 renal reflux with scarring, grade 5 reflux over 6 years of age, and children who fail medical therapy) 3. Endoscopic correction is a *minimally invasive* alternative; *a bulking agent is injected during cystoscopy to change the angle of the ureter* - No incision; outpatient procedure 4. *Renal ultrasound is performed 1 month post-op to check for renal obstruction* - Resolution rate following open surgery is 98%; resolution following endoscopic injection is 50-92%, so a *VCUG may only be recommended following endoscopic injection*

Tricuspid atresia

- *Tricuspid valve fails to develop* → no communication from the RA to RV - *Patent foramen ovale (or other atrial septal opening) is required* to permit blood flow into the LA - *Blood flows from the RA → ASD or a patent foramen ovale → LA → LV → VSD → RV → lungs* - Condition is often associated with pulmonic stenosis and TGA - Mixing of unoxygenated and oxygenated blood in the left side of the heart → systemic desaturation and pulmonary obstruction → decreased pulmonary blood flow → *mostly unoxygenated blood delivered to the body*

Atrioventricular Canal Defect (AV Canal)

- ASD, VSD, and clefts of the mitral and tricuspid valves creates a large central AV valve that *allows blood to flow between all 4 chambers of the heart* - *Most common cardiac defect in children with down syndrome* - PVR decreases after birth → left-to-right shunting occurs → pulmonary blood flow increases - Resultant pulmonary vascular engorgement predisposes the child to development of HF

Furosemide (lasix) nursing interventions

- Drug of choice in severe HF - Causes excretion of chloride and potassium 1. Begin to record output as soon as drug is given 2. Observe for dehydration caused by profound diuresis 3. Observe for side effects: *N/V, diarrhea, ototoxicity, hypokalemia, dermatitis, postural hypotension* - Signs of hypokalemia: muscle weakness, hypotension, dysrhythmias, tachy or bradycardia, irritability, drowsiness 4. *Encourage consumption of foods high in potassium* (bananas, oranges, whole grains, legumes, leafy veggies) *and/or give potassium supplements* 5. Monitor chloride and acid-base balance with long-term therapy 6. *Observe for signs of digoxin toxicity caused by hypokalemia*

Truncus arteriosus

- Failure of normal separation and division of the embryonic bulbar trunk into the pulmonary artery and the aorta → *results in development of a single vessel that overrides the ventricles* - The single vessel empties both ventricles and provides pulmonary, systemic, and coronary circulation - *Blood mixes in the common great artery → leads to desaturation and hypoxemia* - *Blood ejected from the heart flows to the lower-pressure pulmonary arteries → pulmonary blood flow is increased and systemic blood flow is reduced* - VSD is usually present

Patent Ductus Arteriosus (PDA)

- Failure of the fetal ductus arteriosus (artery connecting the aorta and pulmonary artery) to close within the first weeks of life - Typically closes with infant's first breath; PDA is a *common complication of severe respiratory disease in preterm infant* - *Blood shunts from the higher-pressure aorta → lower-pressure pulmonary artery* - *Aorta delivers blood to the body and additional blood to the pulmonary artery → recirculated through the lungs → returned to the left heart* - Results in increased workload on the left side of the heart, *increased pulmonary vascular congestion* and resistance, and potentially *increased RV pressure & hypertrophy*

Pediatric heart sounds

- S1 is synchronous with carotid pulse - S1 is louder at the apex (mitral and tricuspid) - S2 is louder at the base (pulmonic and aortic) - *Pulses should normally be stronger in legs than arms* - If blood pressure is taken in all four extremities then *LEG blood pressures should be higher than ARM blood pressures*

Ambiguous genitalia

- Sex undetermined by visualization - Chromosome analysis is essential - Ultrasound determines presence or absence of ovaries - *Careful testing and evaluation are necessary to aid in gender assignment to avoid lifelong problems for the child; parents participate in the decision* - Explaining the disorder to parents so they can explain it to others is the most therapeutic approach while the parents await the gender assignment of their child

Piaget's theory of cognitive development

- The sensorimotor stage transitions to the preoperational stage - The concept of *object permanence is fully developed* - Toddlers *have and demonstrate memories* of events that relate to them - *Domestic mimicry (playing house) is evident* - Preoperational thought *does not allow for toddlers to understand other viewpoints*, but it does allow toddlers to symbolize objects and people in order to imitate previously seen activities

Spironolactone (Aldactone) nursing interventions

- Weak diuretic - Has *potassium-sparing effect*, frequently used with thiazides, furosemide - Poorly absorbed from GI tract - *Takes several days to achieve maximum actions* 1. Observe for side effects: *skin rash, drowsiness, ataxia, hyperkalemia* - Signs of hyperkalemia: muscle weakness, twitching, bradycardia, v. fib, oliguria, apnea 2. *Do NOT administer potassium supplements* 3. When diuretics are given, record I&O and monitor body weight at the same time each day to evaluate the drug's effectiveness 4. Give diuretics early in the day to children who are toilet trained to avoid the need to urinate at night

TOF interventions

1. *Complete repair: performed in the 1st year of life* for increasing cyanosis and hypercyanotic spells 2. *Palliative shunt: in infants who cannot undergo complete repair*, a procedure to increase pulmonary blood flow and increase oxygen saturation may be performed - Preferred procedure is a *modified Blalock-Taussig shunt* operation, which provides *blood flow to the pulmonary arteries from the left or right subclavian artery via a graft* - *No radial pulse with this repair* - Mortality < 3%

ASD interventions

1. *Digoxin and diuretics (lasix, aldactone) for CHF* 2. Surgical patch closure for moderate to large defects 3. Smaller defects may be repaired during cardiac catheterization (Amplatzer Septal Occluder most commonly used) - Excellent prognosis; elective closure < 1% mortality

Toilet training teaching for parents

1. *Do not begin during time of change or stress* 2. *If child protests, stop and try again in several weeks* 3. Parents should begin readiness phase of toilet training by teaching the child about how the body functions 4. Important considerations are the selection of the child's clothing and the potty chair or use of the toilet - Potty chair allows children a feeling of security - If potty chair isn't available, having the child sit facing the toilet provides added support 5. *Practice sessions should be limited to 5-8 min and parents should stay with the child* 6. Children should be praised for cooperative behavior and successful evacuation 7. Frequent reminders and trips to the toilet are necessary to avoid daytime accidents

Aortic stenosis interventions

1. *First-line procedure: balloon dilation in cath lab* 2. Palliative: valvotomy - *25% of patients require additional surgery within 10 years for recurrent stenosis* - Aortic valvotomy in older children has < 5% mortality but can die of sudden death 3. Aortic valve replacement when stenosis is severe

TGA interventions

1. *IV prostaglandin E may be administered to keep the PDA open to temporarily increase blood mixing, provide an oxygen saturation of 75%, and to maintain CO* 2. During cardiac catheterization or under echo guidance, a *balloon atrial septostomy* may also be performed to increase mixing by opening the atrial septum 3. Surgical: *an arterial switch procedure is the procedure of choice performed in the first few weeks of life* - Involves *anastomosis of the pulmonary artery to the proximal aorta* - Coronary arteries are switched to create a new aorta - Reimplantation of the coronary arteries is critical to the infant's survival, and they must be reattached without kinking to provide the heart with its supply of oxygen - Mortality < 2%

VUR nursing care management

1. *Prevent pyelonephritis to reduce scarring with low dose, long-term antibiotics (Bactrim) and cultures every 2-3 months* - Antibiotics result in sterile urine; reflux of sterile urine does not cause renal damage 2. Encourage compliance in children receiving medical therapy 3. Educate on the importance of hygiene and a frequent voiding schedule 4. Parents need to know that *breakthrough infections can occur despite continuous antibiotic prophylaxis*; need to be aware of UTI symptoms and seek medical attention promptly 5. Assess for high fevers, vomiting, chills 6. *Siblings are at risk for VUR; nurses should encourage parents to be aware of this and have their children screened*

Toddler play

1. *Solitary play evolves into parallel play, in which toddlers observe other children and then may engage in activities nearby* 2. Parents can foster proper motor skills by allowing child to be active; *toddlers should not be confined to playpens* 3. Toys that have small parts should be kept out of reach 4. Age-appropriate toys should be provided. Play activities include: - Filling and emptying containers - Playing with blocks - Looking at books - Playing with toys that can be pushed and pulled - Tossing balls

Wilms tumor treatment & nursing care

1. *Surgery is performed within 24-48 hours of admission* - *The tumor (kept intact to prevent spread of cancer cells), affected kidney, and adjacent adrenal gland are removed* 2. Prognosis for localized tumor (stage I-II): 90% success 3. *Chemotherapy is indicated for all stages; radiation for stages III or IV* - Most effective chemo agents are actinomycin D and vincristine 4. *May reoccur in the lung later in life* but rare if caught early 5. *DO NOT PALPATE ABDOMEN* - To reinforce need for caution, it may be necessary to post a sign on the bed - *Careful bathing and handling* are also important in preventing trauma to the tumor site 6. Monitor BP pre-op; HTN from excess renin production is a possibility 7. *At risk for bowel obstruction* from post-op adhesion formation - *Monitor GI activity: bowel sounds, distention, and vomiting* 8. Frequently assess BP and for signs of infection in the post-op period, esp. during chemo 9. Family and child support - Because the parents usually discover the mass, take into consideration their feelings regarding the diagnosis - Some parents are grateful for their detection of the tumor; others feel guilty for not finding it sooner - Post-op period is difficult for parents. From surgery, the stage and pathology of the tumor are determined - Assess parents understanding of the diagnosis

CoA interventions

1. *Surgical repair is the treatment of choice* - Repair is by resection of the coarcted portion with anastomosis of the aorta or enlargement of the constricted section using a graft - Because this defect is outside the heart and pericardium, *cardiopulmonary bypass is not required* - Residual permanent HTN after repair seems to be r/t age and time of repair - *Elective surgery for COA is advised within the first 2 years of life* 2. Nonsurgical: *balloon angioplasty is a primary intervention for COA in older infants and children* - In adolescents, stents may be placed in the aorta to maintain patency - Mortality is < 5% in children with isolated coarc

Enuresis teaching

1. *Teach parents and child to schedule toileting and to limit fluids (especially caffeine) after 6 pm* - Child may have sips of water before bed but no large drinks 2. Change soiled pajamas and bed linens to prevent breakdown 3. Reassure parents that bed-wetting is not necessarily a manifestation of emotional disturbance and does not represent willful misbehavior 4. *Parents need to understand that punishment such as scolding, shaming, and threatening is contraindicated* because of their negative emotional impact and limited success in reducing the behavior 5. Encourage parents to be patient and understand and to communicate love and support to the child 6. *Positive reinforcements for nights without enuresis* (ex: sticker chart) 7. Communication with children is directed toward eliminating the emotional impact of the problem; relieving feelings of shame, guilt, and the burden of parental disapproval

HUS signs & symptoms

1. Anorexia, lethargy, irritability 2. *Pallor with bruising, purpura, or rectal bleeding* 3. Anuria (absence of urine) and HTN in severely affected patients 4. Excessive accumulation of water and retention of sodium results in *circulatory congestion and edema* 5. *Seizures and stupor suggest CNS involvement* 6. *Signs of acute HF may be present* 7. Mild cases demonstrate anemia, thrombocytopenia, and azotemia (elevated BUN and creatinine); UOP may be reduced or increased - Complications: chronic renal failure, HTN, CNS disorders

Non-cyanotic heart defects with obstruction of blood flow

1. Aortic Stenosis 2. Coarctation of the Aorta (CoA) 3. Pulmonary Stenosis

Safety concerns for parents with a toddler

1. Aspiration - Avoid small objects (grapes, coins, candy) that can become lodged in the throat - Check clothing for safety hazards (loose buttons) - Balloons should be kept away from toddlers 2. Bodily harm - Keep sharp objects out of reach - Keep firearms in locked boxes or cabinets - Toddlers should not be left unattended with any animals present - Toddlers should be taught stranger safety 3. Burns - Check bath water temperature - Turn down thermostats on hot water heaters - Have working smoke detectors in the home - Pot handles should be turned toward the back of the stove - Cover electrical outlets - Toddler should wear sunscreen when outside 4. Drowning - Toddlers should not be left unattended in bathtubs - Toilet lids should be kept closed - Toddlers should be closely supervised when near pools or any other body of water - Toddlers should be taught to swim 5. Falls - Keep doors and windows locked - Crib mattresses should be kept in the lowest position with the rails all the way up - Safety gates should be used across stairs - *Children >35 in should switch from crib to bed* 6. Motor-vehicle injuries - *Rear-facing car seat until age 2, then transition to forward-facing car seat* - *Remain in car seats until age 5, then transition to a booster seat* 7. Poisoning - Avoid lead paint exposure - Place safety locks on cabinets that contain cleaners and other chemicals - Keep poison control center number near phone - Medications should be kept in childproof containers, away from the reach of toddlers - A working carbon monoxide detector should be placed in the home 8. Suffocation - Avoid plastic bags - Crib mattresses should fit tightly. - *Crib slats should be no farther apart than 6 cm (2.4 in).* - Pillows should be kept out of cribs - Drawstrings should be removed from jackets and other clothing

Digoxin nursing interventions

1. Assess HR before administering digoxin - *In infants, withhold if HR is <80* - *In young children, withhold if HR <60* 2. Observe for signs of toxicity: *bradycardia, dysrhythmias, anorexia, nausea, vomiting, neurologic and visual disturbances (halo vision)* - Vomiting associated with digoxin toxicity is often unrelated to feedings, and infants show a decrease in oral intake - If toxicity is suspected, check the digoxin drug level - Therapeutic levels range from 0.8-2 mcg/L - *Withhold if toxicity occurs* 3. Closely monitor serum potassium level while taking digoxin - *A decrease in potassium enhances the effects of digoxin, increasing the risk of toxicity* - *Increased potassium levels diminish digoxin's effect* 4. Give digoxin at regular 12h intervals - *If a dose is missed, do not give an extra dose or increase the dose*; stay on the same medication schedule 5. If the child vomits, do not give a second dose

Wilms tumor clinical manifestations

1. Associated with: - Aniridia (no iris) - Hemihypertrophy (1 side of body larger) - Beckwith-Wiedemann syndrome (overgrowth syndrome) 2. Metastasis rare 3. Diagnosis with radiographic studies, abdominal ultrasound/CT, hematologic and biochemical studies, and urinalysis

TOF clinical manifestations

1. Children assume a *squatting position* to improve oxygenation - Relieves chronic hypoxia, especially during exercise or play - *Reduces the venous return from the legs (which is desaturated) and increases SVR → more blood diverted to the pulmonary artery* 2. Infant may have acute episodes of hypercyanosis and hypoxia *(tet spells)* - Tet spells occur when the infant's *oxygen requirements exceed the blood supply, usually during crying or after feeding* - Results from an *obstruction to pulmonary blood flow* and communication between ventricles - The mechanism of tet spells is thought to include spasm of the infundibular septum, which worsens the right-to-left shunt *(delivers unoxygenated blood to body)* - At risk for *emboli, seizures, LOC, and sudden death* following a tet spell 3. *Hypoxia causes acidosis* which further increases vascular resistance and decreases pulmonary blood flow 4. *Polycythemia* - *Body attempts to improve tissue oxygenation by producing additional RBCs* and thereby increases the oxygen-carrying capacity of the blood. 5. Tet spell interventions: - Place infant in *knee-chest position* - Employ a *calm, comforting* approach - Administer *100% blow-by oxygen* - Give *IV or SC morphine* to reduce infundibular spasms

Pulmonary stenosis clinical manifestations

1. Cyanosis with severe narrowing 2. Systolic ejection murmur 3. *RV hypertrophy* 4. Exercise intolerance

VUR signs & symptoms

1. Cystitis (bladder infection) → pyelonephritis (kidney infection) - *Reflux with infection is the most common cause of pyelonephritis in children* - VUR is a *risk for renal scarring* because it allows bacteria to ascend from the bladder to the kidney and cause pyelonephritis 2. When bladder pressure is high enough, refluxing urine can fill the ureter and renal pelvis - Urine in ureter can drain back down into bladder and remain in bladder until the next void 3. *High fever, vomiting, chills* - VUR increases the chance of febrile UTI but does NOT cause it

VUR diagnosis

1. Cystoscopy - *Direct visualization of bladder and lower urinary tract through small scope inserted via urethra* - Investigation of bladder and lower tract lesions; visualizes urethral opening, bladder wall, and urethra - *NPO after midnight* 2. Voiding cystourethrography (VCUG) - *Contrast medium injected into bladder through urethral catheter until bladder is full; films taken before, during, and after voiding* - Visualizes bladder outline and urethra, reveals reflux of urine into ureters, and shows abnormalities of bladder emptying - Nursing responsibility: *prepare child for catheterization*

UTI signs & symptoms

1. Decreased appetite 2. Increased frequency of urination 3. Incontinence in a toilet-trained child 4. Thirst 5. Crying with dysuria 6. Fever 7. Fatigue 8. Hematuria 9. Complaints of abdominal or flank pain

Fetal circulation

1. Ductus arteriosus: shunt of blood from pulmonary artery to descending aorta, bypassing the lungs 2. Foramen ovale: valve allowing blood flow directly from right to left atrium 3. Ductus venosus: connection of umbilical vein to inferior vena cava, bypassing the liver

Toddler nutrition

1. During the period from 12-18 months of age the growth rate slows, decreasing the child's nutritional needs - *Toddlers manifest this decreased caloric need with a decreased appetite (physiologic anorexia)* - As long as they are growing normally (as illustrated by the growth chart) physiologic anorexia is NOT a concern 2. *Milk intake should average between 2-3 cups/day (16-24 ounces)* - *Switch from whole to low-fat (2%) at 2 years of age* 3. General guide: *½ - 1/3 of adult portion sizes or 1 tablespoon of solid food per year of age at each meal* 4. *Limit fruit juice to 4-6 ounces/day*

Causes of UTIs in children

1. E. coli 2. Structure of the lower urinary tract (shorter urethra) accounts for increased incidence of bacteriuria in females 3. Prevalence of UTI is higher in uncircumcised males less than 3 months (20%) 4. *Urinary stasis is the most important factor influencing occurrence of UTI* - Ordinarily urine is sterile, but the body's temp provides an excellent culture medium - Emptying the bladder flushes away organisms; however, *urine that remains in the bladder allows organisms to multiply and invade surrounding tissue* 5. *Incomplete emptying may result from reflux, anatomic abnormalities (newborns with low-set ears or ear tags), or extrinsic ureteral or bladder compression* (constipation) - Neurogenic bladder (decreased sensation) in children with CP or spina bifida - The hazard of *progressive renal injury is greatest when infection occurs in young children (especially < 2 years of age) and is associated with congenital renal malformations and reflux*

UTI nursing management

1. Encourage good toilet habits 2. Encourage dietary intake of fluid and fiber to help avoid constipation or stool and fluid retention 3. Teach parents S/S of UTI (dysuria) 4. UA performed at well-child visits once child is toilet trained 5. Prep family and child developmentally for tests

Nephrotic syndrome treatment

1. Goals of therapy are to reduce excretion of urinary protein, reduce fluid retention in tissue, prevent infection, and minimize therapy complications 2. Low salt diet 3. Fluid restriction in severe cases 4. Diuretics - Furosemide, alone or in combination, is useful is cases in which edema interferes with respiration or ambulation or there is HTN or significant edema in the scrotum or labia 5. *25% albumin* - *Albumin is treated like a blood product* 6. *Corticosteroids* - Prednisone is the steroid of choice - *Children who require frequent courses of steroid therapy are highly susceptible to complications of steroids, such as growth retardation, behavior changes, increased appetite, HTN, obesity, GI bleeding, infections, and hyperglycemia* - Children with steroid-resistant nephrotic syndrome are started on immunosuppressant therapy

VSD interventions

1. HF is common; treat if it develops 2. Palliative: pulmonary artery banding (*placement of a band around the main pulmonary artery to decrease pulmonary blood flow*) may be done in infants with multiple muscular VSDs or complex anatomy 3. *Complete repair (procedure of choice)*: small defects are repaired with sutures. Large defects require sewing a knitted Dacron patch over the opening - Cardiopulmonary bypass is used for both procedures - The approach for the repair is generally through the RA and tricuspid valve - Post-op complications include residual VSD and conduction disturbances 4. Nonsurgical: *cath closure of defects carries more risk than with ASDs* - Good prognosis. Risks depend on the location of the defect, the number of defects, and the presence of other associated cardiac defects - Single defects are associated with low mortality (< 2%), multiple defects can carry a higher risk

AGN complications

1. Hypertensive encephalopathy - Hyperperfusion of the brain and cerebral edema leads to HA, dizziness, abdominal discomfort, vomiting - *May progress to loss of vision, disorientation, and seizures* 2. Acute cardiac decompensation as a result of hypervolemia 3. Acute renal failure (ARF) - uncommon

Preoperational stage of toddlerhood (2-4 yr)

1. Increased use of language as mental symbolization - Refers to self as pronoun, begins to use verbs in past tense, uses many future-oriented words (tomorrow, next day) but has poor concept of passage of time - *Uses 2 or 3 word phrases* 2. Egocentrism still present; possessive of own toys, *uses word "mine"* 3. *Follows directions using words such as up, behind, under, in back of*

AV canal interventions

1. Palliative: *pulmonary artery banding is occasionally done in small infants with severe symptoms* 2. *Complete repair in infancy is most common* - consists of patch closure of the septal defects and reconstruction of the AV tissue 3. Post-op complications include *heart block, CHF, mitral regurgitation, dysrhythmias, and pulmonary HTN* - Operative mortality < 5%

UTI prevention

1. Perineal hygiene - wipe from front to back 2. Avoid tight clothing or diapers; wear cotton panties rather than nylon 3. Encourage toilet posture to relax the pelvic floor: knees separated and feet supported for girls 4. Avoid "holding" urine; encourage child to void frequently 5. Take time to relax and empty bladder completely with each void 6. Encourage generous fluid intake - WATER - Cranberry juice prevents attachment of e. coli to tract walls - Avoid caffeinated beverages (irritating to bladder) 7. Increase dietary fiber and fluid 8. Use stool softener as needed 9. Encourage physical activity 10. Avoid bubble baths - If child does take a bath, wash hair LAST to avoid getting soap in the water 11. Advise adolescent girls (sexually active) to void immediately after intercourse to flush out bacteria introduced during sex

Aortic stenosis clinical manifestations

1. LV hypertrophy → LV failure → increased pressure in LA → increased pressure in pulmonary veins → *pulmonary vascular congestion* 2. Newborns with critical aortic stenosis have *signs of decreased CO* with faint pulses, hypotension, tachycardia, and poor feeding 2. Children show signs of *exercise intolerance, chest pain, and dizziness when standing for a long period* 3. Patients are at *risk for IE, coronary insufficiency, and ventricular dysfunction*

ASD clinical manifestations

1. May be asymptomatic 2. *Loud, harsh systolic murmur heard in pulmonic area* 3. *May develop CHF (esp. RHF) from increased pulmonary blood flow* 4. *At risk for atrial dysrhythmias* (possibly caused by RA enlargement and stretching of conduction fibers) 5. Increased flow of oxygenated blood to the right side of the heart → *decreased flow of oxygenated blood to the body*

PDA clinical manifestations

1. May be asymptomatic or show signs of HF 2. *Loud, continuous "washing machine" murmur heard in systole over the pulmonic area (2nd left intercostal space)* 3. Wide pulse pressure and bounding pulses - Decreased DBP

Nephrotic syndrome education

1. Most children are treated at home during relapses. *Home care is preferred (because of susceptibility to infection) unless the edema and proteinuria are severe* 2. Teach parents to detect signs of relapse and notify HCP if they occur 3. Nurses should instruct parents in urine testing for albumin, administration of medications, and general care 4. *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* 5. *Salt is restricted to no additional salt during relapse and steroid therapy*, but a regular diet is suitable for a child in remission 6. Instruct parents on avoiding contact with infectious playmates 7. It is important for parents of children on corticosteroid therapy to *be aware of the common side effects of steroid therapy (rounding of the face, increased appetite, behavior changes, abdominal distention, hirsutism)* and to distinguish some of these from the edema formation of the disease - *Reassure parents that the symptoms will disappear gradually after d/c the drug* 8. Social isolation is common; provide support

TGA clinical manifestations

1. Murmur 2. *Severe cyanosis hours to days after birth (increases as PDA closes)* 3. Cardiomegaly and heart failure

Truncus arteriosus clinical manifestations

1. Murmur 2. Moderate to severe CHF 3. Cyanosis 4. Poor growth 5. Activity intolerance

Enuresis etiology & psychological effects

1. No clear etiology has been determined; it may be r/t longer duration of sleep in infancy, a positive family history, and a slower rate of physical development in children up to 3 years of age 2. *Children with enuresis often have other developmental disorders, learning problems, or behavior difficulties*, such as increased motor activity and aggression - Children with ADHD may be so involved with play that they are unaware of a full bladder or forget to empty the bladder 3. *Enuresis can cause serious psychologic problems r/t the impact on the child's social life* (not being able to attend overnight camps, school field trips, or sleep overs) 4. Adolescents have described themselves as being anxious or tense, having difficulty sleeping, and having bad dreams;*many delay or avoid getting treatment, believing they will eventually grow out of it 5. *Children may have significant stress or anxiety in the home environment if parental response is harsh* 6. Enuresis may be r/t attempts to toilet train before child is ready 7. Functional or structural defect may result in enuresis

AGN treatment & nursing interventions

1. No specific treatment is available for AGN. Management consists of supportive measures; early recognition and treatment of complications 2. *Children with normal BP and UOP can be treated at home* 3. *Those with substantial edema, HTN, gross hematuria, or significant oliguria are often hospitalized to prevent complications such as seizures* - *Implement seizure precautions*: suction at bedside, side-lying position, padded side-rails, nothing in mouth 4. *Daily weight is the most useful means to assess fluid balance* 5. *Sodium restriction* for children with edema and HTN - BP measurements q.4-6h - Lasix for edema and fluid overload (if ARF is not present) - HTN treated with diuretics or other antihypertensives 6. *Antibiotics for those with persistent strep* 7. *Fluid restriction, if prescribed* - Amount permitted should be evenly divided throughout waking hours and served in small cups to give illusion of bigger serving 8. Plan activities with *frequent rest periods* to avoid fatigue - *Strenuous activity is restricted until no proteinuria or hematuria* 9. *Follow-up care weekly, followed by monthly visits for evaluation and UA*

Pulmonary stenosis interventions

1. Nonsurgical: *balloon angioplasty* 2. Surgical: in infants, Brock procedure (closed valvotomy). In children, pulmonary valvotomy with cardiopulmonary bypass - *Need for surgical treatment is rare with widespread use of balloon angioplasty* - Mortality is less than 1%

Tricuspid atresia clinical manifestations

1. Older children have signs of *chronic hypoxemia with clubbing* 2. Failure to thrive and growth retardation 3. Heart failure 4. *Severe cyanosis within hours of birth (increasing as PDA closes; PDA keeps fetus with tricuspid atresia alive in utero)* 5. Tachycardia 6. Dyspnea

PDA interventions

1. Prostaglandin production keeps the ductus arteriosus open in utero; therefore, *administration of ibuprofen or indomethacin (prostaglandin inhibitor) is successful in closing a PDA* in some preemies and newborns - Indomethacin inhibits platelet function and affects renal function in neonates - *Requires close monitoring for bleeding and assessment of renal function* (adequate UOP, BUN and creatinine levels) 2. Nonsurgical treatment: *coils to occlude the PDA* are placed in the cath lab - Premature or small infants (with small femoral arteries) and patients with large or unusual PDAs may require surgery 3. Surgical ligation of the patent vessel is performed via left thoracotomy - Excellent prognosis; elective closure < 1% mortality

Nephrotic syndrome nursing care

1. Strict measurement of I&O is essential - Weigh diapers, examine urine for albumin, daily weight, measure abdominal girth 2. Assess edema - Assess for increased or decreased swelling around eyes and dependent areas, degree of pitting, and color and texture of skin 3. Monitor vitals for signs of complications such as shock and infection 4. *Elevating edematous extremities may be helpful to shift fluid, but diuresis with medications and salt and water restriction to remove edema fluid are best therapies* 5. *Because these children are vulnerable to URI, protect them from infected individuals* 6. Loss of appetite creates a problem; combined efforts with parents, dietitians, etc. is necessary to formulate an adequate and attractive diet 7. As edema subsides, children are allowed increased fluids 8. *Irritability and mood swings accompanying the disease process and steroid therapy are not unusual*

HUS treatment

1. Supportive measures initially focus on managing renal failure: fluid replacement, treatment of HTN, and *correction of acidosis and electrolytes* - *Administer tums (calcium gluconate) to replace calcium* - *Kayexalate for hyperkalemia* - Administer albumin hydroxide (binds with phosphorus) 2. *Hemodialysis or peritoneal dialysis is instituted in any child who has been anuric for 24 hours* or who demonstrates oliguria with uremia or HTN and seizures 3. *FFP and plasmapheresis, blood transfusion with fresh PRBCs* for severe anemia 4. Fluid restriction 5. *High calorie, high carb, low protein, low sodium, low potassium diet* - Prompt treatment = 95% recovery - 10-50% have residual renal impairment

Sensorimotor stages of toddlerhood

1. Tertiary circular reactions (13-18 mo): - Experimentation to achieve previously unattainable goals using all sensory cues and physical skills - Increased concept of object permanence; can find hidden objects, but *only in first location and realizes that "out of sight" is not out of reach* - Differentiation of oneself from objects - Early traces of memory - Awareness of spatial, causal, and temporal relationships - Able to enter into an action at any point without reproducing entire sequence - *Ventures away from parents for longer periods and gains comfort from parents voice even if parent is not visible* - Nests objects - *Gestures "up" and "down"* 2. Invention of new means through mental combinations (19-24 mo) - Searches for objects through *several hiding places* - Will *infer cause by associating 2 or more experiences* (ex: candy missing, sister smiling) - *Imitates* words, sounds of animals, and adult behavior - *Follows directions and understands requests* - Has some sense of time; *waits in response to "just a minute"; may use word "now"* - *Refers to self by name* - Egocentrism in thought and behavior - *Uses words up, down, come, and go with meaning*

Cyanotic heart defects with decreased pulmonary blood flow

1. Tetralogy of Fallot (TOF) 2. Tricuspid Atresia

Anticipatory guidance for toddler nutrition

1. Toddlers are generally *picky eaters* who will repeatedly request their favorite foods 2. Exposure to a new food may need to occur 8-15 times before toddlers develops an acceptance of it 3. *If there is a family history of allergy, then cows' milk, chocolate, citrus fruits, egg whites, seafood, and nut butters may be gradually introduced while monitoring for reactions* 4. Prefer finger foods because of increasing autonomy 5. *Regular meal times and nutritious snacks best meet nutrient needs* - Snacks or desserts that are high in sugar, fat, or sodium should be avoided 6. Foods should be *cut into small, bite-size pieces* to make them easier to swallow and to prevent choking - *Foods that are potential choking hazards (nuts, grapes, hot dogs, peanut butter, raw carrots, tough meats, popcorn) should be avoided.* 7. Adult supervision should always be provided during snack and mealtimes. 8. *No drinking or eating during play activities or while lying down* 9. Parents should follow dietary recommendations outlined by the United States Department of Agriculture - Encourages a variety of fruits, vegetables, whole grains, and low-fat and nonfat dairy products in addition to fish, beans, and lean meat

Cyanotic heart defects with mixed blood flow

1. Transposition of the Great Arteries (TGA) or Vessels (TGV) 2. Truncus Arteriosus 3. Hypoplastic Left Heart Syndrome

UTI diagnosis

1. UA indicates pyuria (WBCs in urine), bacteriuria, and possibly hematuria - *WBCs, RBCs, hgb, leukocyte esterase (enzyme present in WBCs, indicating pyuria), nitrites (indicating bacteriuria)* 2. Diagnosis is made on the basis of *pyuria and the presence of at least 1 bacterium on gram stain*; detection of bacteria in urine culture confirms diagnosis - key to distinguishing true UTI from asymptomatic bacteriuria is the presence of pyuria 3. Other labs: CMP, BUN, creatinine, specific gravity 4. Contamination of a specimen by organisms from sources other than urine is the most common cause of false-positive results 5. *Do NOT encourage children to drink large volumes of water in an attempt to obtain a specimen; high fluid intake may dilute urine* and indicate a falsely low organism count 6. AAP guidelines for evaluating febrile infants recommends obtaining a urine specimen for culture and UA BEFORE antibiotics are administered 7. Most accurate tests of bacterial content are suprapubic aspiration (for children < 2 years) and bladder catheterization (as long as first few mL are excluded) 8. Urine specimen must be fresh: < 1 hr after voiding with storage at room temp or < 4 hrs after voiding with refrigeration 9. *Ultrasound, VCUG, and intravenous pyelography (IVP) may be performed after the infection subsides to identify anatomic abnormalities contributing to the development of infection and existing kidney changes from recurrent infection*

AGN labs

1. UA shows *hematuria, proteinuria, and increased specific gravity* - *Discoloration of urine reflects RBC and hgb content* 2. Negative urine cultures 3. Normal electrolyte and carbon dioxide levels 4. Impaired GFR → elevated BUN and creatinine 5. *ASO indicates previous strep infection and remains elevated for approximately 10 days* after the initial infection 6. Serum complement level (C3) is initially decreased but increases with improvement of the disease

HUS interventions

1. Verify blood product matches prescription 2. Verify the patient and product identification with another nurse 3. Remain with the patient during the first 5 minutes and obtain vital signs 4. *Obtain vital signs again in 15 min → 30 min → hourly while infusing* 5. *If transfusion reaction occurs (initially tachypnea followed by spike in temp), STOP the transfusion* and send blood back to the blood bank 6. Nursing care is focused on support of family because of the sudden and life-threatening nature of the disorder in a previously well child 7. Teach parents to cook ground beef until no pink color is seen and to use a meat thermometer 8. Discourage parents from giving children unpasteurized apple juice and unwashed raw vegetables 9. Discourage use of antimotility drugs for diarrhea

Nephrotic syndrome signs & symptoms

1. Weight gain 2. Periorbital edema apparent in the morning but subsides during the day, when swelling of the abdomen, genitalia, and lower extremities is more prominent 3. Ascites - *Edema of intestinal mucosa may cause diarrhea, loss of appetite, and poor intestinal absorption* 4. Elevated BP 5. Child may be irritable and more easily fatigued or lethargic 6. *Urine is frothy (from albumin) and decreased in volume* - *NO BLOOD IN URINE UNLIKE AGN* - Urine appears darkly opalescent 7. Extreme pallor 8. May experience skin breakdown from severe edema 9. *Child is more susceptible to infection*

Atrial Septal Defect (ASD)

Abnormal opening between the atria, allowing *blood from the higher-pressure LA to flow into the lower-pressure RA* → increased flow of oxygenated blood into the right side of the heart

Immune-complex disease or reaction that occurs as a by-product of streptococcal infection:

Acute glomerulonephritis (AGN) - Manifestation of a systemic disorder - Common features include oliguria, edema, HTN, circulatory congestion, *hematuria and proteinuria 3-4+* - Most cases are postinfectious and have been associated with strep and viral infections; acute poststreptococcal glomerulonephritis occurs most commonly (APSGN) - *Peak age 6-7 yrs old; uncommon in children younger than 2 yrs* - Occurs in boys 2:1

________________ is a severe defect characterized by externalization of the bladder

Bladder exstrophy - Results from failure of the abdominal wall and underlying structures, including the ventral wall of the bladder, to fuse in utero - Surgical repair is required; *the bladder is closed during the neonatal period, preferably within the first 1-2 days of life* - *Covered with clear plastic wrap or a thin film dressing without adhesive until closure is performed* - Petroleum jelly is avoided because it tends to damage the bladder mucosa - After repair, the child's bladder is allowed to increase capacity. Several surgical procedures may be necessary to create a urethral sphincter mechanism to aid in urination

________________ is the ventral curvature of the penis

Chordee - Results from the replacement of normal skin with a fibrous band of tissue and usually accompanies more severe forms of hypospadias - *May leave the infants gender in doubt when combined with hypospadias because the perineal position of the meatus may be mistaken for a female urethra*

________________ is failure of one or both testes to descend normally through the inguinal canal into the scrotum

Cryptochidism - A *nonpalpable testis* is typically observed by the parent or during routine physical exam - *Important to differentiate true undescended testis from the more common retractile testis*; retractile testis will be observed in the scrotum when the child is being bathed in warm water - Retractile testis can be pushed back into the scrotum, but truly undescended ones cannot - Truly undescended testes require surgical treatment - *The longer the testis is exposed to higher body heat, the greater the likelihood of damage. To preserve fertility, surgery should be done at an early age*

In a child with congenital heart disease, it is important for the nurse to assess what?

Daily weights - Growth is slowed in children with CHD - The metabolic rate of these infants is greater because of poor cardiac function and increased heart and respiratory rates - *Caloric needs are greater than those of the average infant because of their increased metabolic rate, yet fatigue limits their ability to take in adequate calories* - The diet should include *increased protein and increased fat* to facilitate the child's intake of sufficient calories - *May also supplement by increasing caloric density of formula*

The goals of treatment of children with UTI are to:

Eliminate the acute infection, prevent complications, and reduce the likelihood of renal damage 1. Antibiotic therapy depends on lab C&S tests - *Bactrim: SEs include urticaria, photosensitivity; teach importance of sunscreen use while taking* - Penicillins, cephalosporins, nitrofurantoin 2. *Children with suspected pyelonephritis and fever are admitted to the hospital and given IV antibiotics for 48 hours*

_______________ is defined as intentional or involuntary passage of urine in children who are beyond the age when voluntary bladder control should normally have been acquired

Enuresis (bed-wetting) - Medical evaluation is recommended when enuresis occurs at least *2 times a week for a minimum of 3 consecutive months in a child > 5 years* - Urinary incontinence must not be related to diuretics or medical conditions such as diabetes, spina bifida, or seizure disorder - *More common in boys; nocturnal bed-wetting usually ceases between 6-8 years without intervention* - 1% of bed-wetters do not improve until 17 or older - Enuresis may serve as a trigger for child abuse

_______________ is the urethral opening on the dorsal surface of the penis

Epispadias - Circumcision will not be performed - Surgically repaired within first 6-12 months of life

Toddler development - fine & gross motor skills

Gross motor skills 1-2 years: - Runs fairly well - Up and down steps, two feet on each 3 years: - Jumping with both feet Fine motor skills 1-2 years: - Builds a tower of 3-4 blocks - Turns pages of book one at a time - Turns doorknob - Unscrews lids 2-3 years: - Good hand-finger coordination - Makes two strokes for a cross and draws circles

________________ is an acute renal disease characterized by a triad of manifestations: anemia, thrombocytopenia, and renal failure.

Hemolytic uremic syndrome (HUS) - Primarily in infants *6 months to 5 years old* - *Associated with e. coli* (undercooked ground beef), unpasteurized milk or fruit juice (apple), sprouts, lettuce, salami, or drinking/swimming in sewage-contaminated water - *Occurs after gastroenteritis or URI and persists for several days to 2 weeks*

________________ is the presence of fluid in the scrotum

Hydrocele - Unlike a hernia, hydrocele *may not be reducible and may not be produced by a sudden increase in intraabdominal pressure* (such as straining) - Scrotum appears to be *larger after an active day and smaller in the morning* - Can predispose the child to herniation; therefore, *surgical repair is indicated if spontaneous resolution does not take place by 1 year of age* - Advise parents that there is often temporary swelling and discoloration of the scrotum that resolves spontaneously

________________ is a condition in which the urethral opening is located on the ventral (underside) surface of the penis

Hypospadias - Circumcision will not be performed - *Preferred time for surgical repair is 6-12 months before child has developed body image*

Non-cyanotic heart defects with increased pulmonary blood flow

Increased pulmonary blood flow causes a left-to-right shunt 1. Patent Ductus Arteriosus (PDA) 2. Atrial Septal Defect (ASD) 3. Ventricular Septal Defect (VSD) 4. Atrioventricular Canal Defect (AV Canal)

_________________ is a protrusion of peritoneum through the abdominal wall in the inguinal canal

Inguinal hernia - Usually asymptomatic unless abdominal contents are forced into the patent sac - Often appears as *painless inguinal swelling that varies in size* - May first be noticed when the infant is crying or straining to stool - *Disappears during rest or is reducible by gentle compression* - Occasionally becomes *incarcerated (irreducible)* or *strangulated (loss of blood supply)* that can progress to strangulation and necrotic bowel if untreated - Treatment is prompt, elective surgical repair in the healthy child - Managed on an outpatient basis

Nephrotic syndrome pathophysiology

May be a metabolic, biochemical, physiochemical, or immune-mediated disturbance in the basement membrane of the glomeruli Basement membrane (normally impermeable) becomes increasingly permeable to albumin and other large proteins → Albumin (fluid volume expander) leaks through the membrane and is lost in urine, reducing the serum albumin level (hypoalbuminemia) → Fluid accumulates in interstitial spaces (edema) and body cavities (ascites) → Shift of fluid (hypovolemia) stimulates renin-angiotensin system and secretion of antidiuretic hormone → BP increases; sodium and water reabsorbed to increase intravascular volume

________________ is the narrowing or stenosis of the preputial opening of the foreskin

Phimosis - Prevents retraction of the foreskin - Normal finding in infants and young boys and usually disappears as the child grows - *Occasionally the narrowing obstructs the flow of urine, resulting in a dribbling stream or even ballooning of the foreskin with accumulated urine during voiding* - Proper hygiene consists of external cleansing during routine bathing - *Foreskin should NOT be forcibly retracted since it may create scarring*

According to Erikson, several characteristics, especially _______________ and _______________, are typical of toddlers in their quest for autonomy

Negativism and ritualism Negativism: - Give negative responses to requests - "No" or "me do" - Express strong emotions with rapid mood swings and frequently disrupt the environment - *If scolded for doing something wrong, they can have a temper tantrum and almost instantaneously pull at the parent's legs to be picked up and comforted* Ritualism: - The need to maintain sameness and reliability, provides a sense of comfort - Toddlers can venture out with security when they know that familiar people, places, and routines exist - *Change (such as hospitalization) represents a threat* - *Without comfortable rituals, they have little opportunity to exert autonomy, resulting in dependency and regression*

________________ is defined as massive proteinuria, hypoalbuminemia, hyperlipidemia and edema.

Nephrotic syndrome - *Most common glomerular injury in children 2-7 years old* - The disorder is a clinical manifestation of a large number of distinct glomerular disorders in which increased glomerular permeability to plasma protein *results in massive urinary protein loss*

AGN signs & symptoms

Onset of nephritis *appears after a latent period (10 days).* Because the child appears well during this time, parents may not recognize the association 1. Periorbital, gonadal, abdominal, or lower extremity edema - Edema is more prominent in the face in the morning but spreads during the day 2. Loss of appetite 3. *Decreased UOP* 4. *Cola- or tea-colored urine* 5. Pale, irritable, lethargic 6. Older children may complain of HA, abdominal discomfort, or dysuria 7. HTN 8. *Patient may have seizures from hypertensive encephalopathy, pulmonary and circulatory congestion, or hematuria*

Toilet training readiness

Physical readiness: - *Voluntary control of the anal and urethral sphincters is achieved after the child is walking, between 18-24 months* - Ability to stay dry for 2 hours; decreased number of wet diapers; waking dry from a nap - Regular bowel movements *(bowel training occurs before bladder training)* - Gross motor skills of sitting, walking, and squatting - Fine motor skills to remove clothing Mental readiness: - *Recognition of urge* - Verbal or nonverbal communication skills to indicate when wet or has urge - Cognitive skills to imitate appropriate behavior and follow directions Physiologic readiness: - *Expressing willingness to please parent* - Able to sit on toilet for 5-8 min without fussing or getting off - Curiosity about adults' toilet habits - Impatience with diapers; desire to be changed immediately Parental readiness: - Recognition of child's level of readiness - Willingness to invest time - *Absence of family stress or change* such as divorce, moving, new sibling, or vacation

HUS pathophysiology

Primary site of injury is the endothelial lining of the small glomerular arterioles → Endothelium becomes swollen and occluded with the deposition of platelets and fibrin clots → RBCs are damaged as they move through partially occluded blood vessels → *Spleen removes fragmented RBCs, causing acute hemolytic anemia* → *Thrombocytopenia is produced by the platelet aggregation within damaged blood vessels or the damage and removal of platelets*

1. For the neonate whose pulmonary blood flow depends on the PDA, a *continuous infusion of prostaglandin E is started* until surgical intervention can be arranged - *Do NOT administer oxygen to a cyanotic neonate; oxygen closes the PDA needed for survival* 2. Palliative treatment is the *placement of a shunt* (pulmonary-to-systemic artery anastomosis) to increase blood flow to the lungs → *bidirectional Glenn shunt at 4-9 months → Modified Fontan* - Modified Fontan procedure directs systemic venous return to the lungs through surgical connections between the RA and the pulmonary artery - This procedure *separates oxygenated and unoxygenated blood inside the heart and eliminates excess volume load on the ventricle* but does NOT restore anatomy - Mortality < 5% - *Long-term problems: pericardial effusions, dysrhythmias, developmental delay*

Tricuspid atresia interventions

_______________________ refers to the retrograde flow of urine from the bladder into the ureter and kidneys

Vesicoureteral reflux (VUR)

________________, or nephroblastoma, is the most common malignant renal and intraabdominal tumor of childhood

Wilms tumor - *Peak incidence at 3 years of age* - 3 times more common in caucasians than African American and Asian - *Slightly favors the left kidney*; 10% both kidneys - May be associated with several congenital malformation syndromes, but there is *no method of identifying gene carriers* at this time