Pediatrics Final (new content)

Normal ureter and bladder anatomy

With contraction of the bladder during urination, the uretero-vesicular junction (UVJ) closes, ensuring a uni-directional flow of urine from the ureters into the bladder

Vesicoureteral reflux

the retrograde passage of urine at the uterovesicular junction (UVI) into the ureters. Urine refluxes from the bladder into the ureters when the bladder contracts due to incompetent UVJ

Diagnosis of VCUR

A history of urinary tract infections causing febrile episodes raises suspicion for VCUR, a voiding cystourethrogram will show reflux and hydronephrosis

voiding cystourethrogram

the urethra is catheterized and the bladder is filled with contrast material, images are taken of the bladder full and with voiding, if refluxing from the bladder is occurring the contrast will be seen in the ureter and possibly the kidney as shown in the VCUG image

signs of VCUG with reflux

Kidney- filled with contrast, ureter that is engorged from refluxing urine, bladder- filled with contrast

Manifestations of VCUR

reflux and stasis of urine, hydronephrosis, more severe urinary tract infections, scarring of the kidney tissue, kidney injury (complication- UTI)

Signs of a UTI in infants and young children

Fever, V/D, abdominal/flank pain, dysuria, abdominal distention, lethargy, irritability, apnea in newborns

hydronephrosis

stretching/swelling of the renal pelvis due to chronic reflux and obstruction of urine flow which can lead to kidney tissue damage, sometimes discovered during fetal ultrasound

Grades of Hydronephrosis

1 is the least severe, and 5 is the most severe

medical treatment of VCUR

- prophylactic, low dose antibiotic (usually PCN or sulfa medication) to prevent urinary tract infection until child grows out of the anatomical defect

- Frequent voiding to avoid a full bladder that will aggrevate refluxing

- increased fluid intake to encourage frequent voiding and reduce risk of infection

- urine culture is obtained if there are signs and symptoms of infection

parent education for VCUR

- S/S of UTI, report suspicion to PCP

- rationale for low-dose prophylactic antibiotics

- education of required testing

- female children: front-to-back cleansing after toiletings, most common infecting organism in UTI is E. Coli\

- encourage fluid intake and frequent voiding

- uncircumcised males: routine hygiene to reduce risk of UTI

- Follow up: urine analysis/culture, VCUG

procedural treatment for VCUR: Surgical

ureteral re-implantation, performed if medical rx fails, open or laparoscopic approach used, ureters are removed from bladder and re-implanted at an appropriate angle and depth through the bladder wall to prevent reflux

procedural treatment for VCUR: STING

subureteral trans-urethral injection, endoscopic injection, trans-urethral injection of a bulking agent performed into the submucosa of the UVJ, bulking agent enhances competence of junction closure during bladder contraction, performed via cystoscopy as shown in the picture, child with be NPO prior to this procedure in preparation for sedation or anesthesia

Hypospadias

congenital anomaly where the urethral opening is on the ventral surface of the penis, genetic predisposition

Hypospadias associated risks

mis-directed urine flow, infertility due to abnormal semen flow, erectile dysfunction, abnormal appearance of penis

Characteristic appearance of Hypospadias

small prepuce, chordee, severity variable, urethral opening can occur at any location along ventral surface, even close to the scrotom, bifid scrotom

Hypospadias: small prepuce

small or no foreskin noted

Hypospadias: chordee

a curvature of the penis may be present

Hypospadias: degree of severity variable

degree of curvature and distance of urethral opening from penis tip along the ventral surface can vary

Bifid scrotum

a cleft or split in the midline of the scrotum, appearing as two separate scrotal halves

treatment for Hypospadias

no circumcision is done, surgical correction: performed at 6 to 12 months of age before toliet training initiated

Post-op care for ureteral re-implantation and hypospadias repair

routine pain management, foley/stent care- ensure tube patency and monitor output hourly, administer antibiotic until foley/stent removal to prevent infection, administer oxybutynin

oxybutynin

anti-spasmodic of smooth muscle (bladder), be aware of extended release versus immediate release dosage forms for this medication

Similarities between Hypospadias and VCUR

congenital abnormalities, variable degrees of severity, post op care includes pain management, foley care, and anti-spasmodic therapy

differences between Hypospadias and VCUR

VCUR can be treated without surgery for mild forms, VCUR can lead to infection and organ damage, VCUR requires imaging to diagnose, timing of surgery for hypospadias is typically earlier in life

Acute Glomerulonephritis (AGN)

Immuno-pathogenic condition usually following an infection, Group A beta-hemolytic streptococcal infection (acute poststreptococcal glomerulonephritis- APSGN), secondary causes can include: SLE and sickle cell disease, occurs suddenly about 10-21 days following infection, typically seen in the school-aged child, greater incidence in males, usually lasts 2-4 weeks

glomerofiltration rate (GFR)

in normal GFR physiology, blood is filtered as it flows through Bowman's capsule. Urine is created from the filtration that occurs across the glomerular membrane.

Patho of AGN

immune complexes deposit in basement membrane of glomeruli, glomerular edema and trauma result from the immune complex deposition, from edema and trauma- decreased glomerular filtration rate and hematuria occurs, decreased GFR leads to excessive accumulation of water and sodium, expanded plasma and interstitial fluid (hypovolemia) occurs from the retention of water and sodium

Outward effects of AGN

tea-colored urine and periorbital edema

Manifestations of AGN

decreased UOP, elevated specific gravity from concentrated urine (over 1.015), tea-colored urine from hematuria, peri-orbital and peripheral edema, anorexia and vomitting (due to retention of wastes from decreased GFR), irritability and lethargy from edema, hypertension ad headache from hypervolemia, proteinuria and hematuria from glomeruli trauma, serum lab values (elevated BUN and Cr, ASO titer is evidence of recent strep infection)

Medical care for AGN

antibiotic if active infection present, diuretic therapy for hypervolemia: furosemide, anti-hypertensive agent, low Na+ diet and restricted fluid intake, possible K restriction (oligouria)

Nursing care for AGN

monitor fluid balance (strict I and O, daily weight, VS with BP Q4h), monitor BUN and Cr results-potential complication of renal failure, monitor urine for blood/protein and track BP as outcome measures for disease improvement, implement seizure precautions; potential complication of hypertensive encephalopathy, monitor breath sounds- potential risk for pulmonary edema/effusion

Nephrotic syndrome

an increase in permeability of the glomeruli to plasma proteins caused by an auto-immune response

Idiopathic Nephrotic syndrome

most common type of nephrotic syndrome, typically effects children 2-6 years of age, illness course can be weeks to months/relapses

Secondary Nephrotic syndrome

Caused by a condition that damages the glomeruli such as AGN

congenital Nephrotic syndrome

hereditary disorder characterized by protein loss, edema and intravascular volume contraction

edema caused by protein loss in urine because albumin is a key factor in holding onto water in the blood

3 types of pressure in the bowman's capsule

colloid osmotic pressure, hydrostatic blood pressure, hydrostatic capsule pressure. The pushing and pulling enforces homeostasis of the blood volume as it passes through the Bowman's capsule

colloid osmotic pressure (COP)

created by plasma proteins which PULLS fluid INTO the vascular space

hydrostatic blood pressure

created by blood pressure which PUSHES fluid OUT OF the vascular space

hydrostatic capsule pressure

created by Bowman's Capsule that PUSHES against the blood hydrostatic pressure

Patho of Nephrotic Syndrome

Loss of serum proteins into the urine leading to reduced colloidal osmotic pressure of the intravascular space (PULL) resulting in leakage of fluid into body tissue and intravascularly hypovolemic, decreased renal blood flow leading to: release of renin resulting in vasoconstriction and increased secretion of ADH and aldosterone leading to Na+ and fluid rentention

manifestations of Nephrotic Syndrome due to loss of serum protein

hypo-albuminemia (norm is 3.5-5.5), significant proteinuria causing frothy urine, hyperlipidemia from low serum albumin

manifestations of Nephrotic Syndrome due to loss of COP and intravascular fluid leak into tissue

Anasarca- significant generalized edema, fatigue and poor appetite from severe edema, increased specific gravity (over 1.015), weight gain

manifestations of Nephrotic Syndrome due to decreased intravascular volume and hypovolemia

decreased UOP, low or normal BP, elevated BUN from dehydration, elevated Cr

effects of Nephrotic Syndrome

anasarca- severe tissue edema from hypoalbuminemia, frothy urine from high urine protein level

Nephrotic Syndrome and immunosupressive treatment primary

teroid therapy: extended course of weeks to months required, early responders have best prognosis for recovery

- side effects: growth suppression, weight gain, hyperglycemia, immunosuppression

Nephrotic Syndrome and immunosupressive treatment secondary

Cyclophosphamide-immunosuppressant agent/chemo, cyclosporine- DMARD, immunosuppressive agent, used in non-responders

Supportive care for Nephrotic Syndrome

albumin infusion and diuretic

- albumin pulls fluid from the tissue into the vascular space followed by diuresis with the diuretic

- diuretics such as: furosemide, spironolactone, metolazone

Fluid and salt restriction

short term complications of Nephrotic Syndrome

- immunosuppression from medications

- infections due to immunosuppression and interstitial fluid accumulation: (peritonitis, pneumonia, cellulitis)

- thromboembolism from combination of hypovolemia, reduced mobility, and hyperlipidemia

- Skin breakdown from anasarca, reduced mobility, poor nutrition

- potential relapse of nephrotic syndrome

long term complications of Nephrotic Syndrome

renal failure and renal insufficiency

Nursing implications of Nephrotic Syndrome

- monitor fluid balance: strict I's and O's and daily weight

- monitor urine protein, BUN, or Cr for signs of improvement from therapy

- monitor VS and BP for signs of hypovolemia or infection

- monitor abnormal girth for improvement in edema

- monitor respiratory status for pulmonary complications

- monitor skin integrity and implement preventative skin care

- promote infection control measures due to immunosuppressive therapy

- promote nutrition with salt and fluid restriction

- encourage ambulation to prevent thromboembolism and/or SCDs

- educate family on all of the above

renal biopsy: care before biopsy

- NPO prior for 4-6 hours if patient is sedated for procedure

- check coagulation studies prior for bleeding risk

renal biopsy: care after biopsy

renal tissue is highly vascular; therefore care focuses on prevenetion of and monitoring for bleeding:

- monitor VS, UOP, and hematocrit immediately after procedure

- bedrest x 24 hours

- no heavy lifting for 5-7 days

- lay supine on back roll to keep pressure on needle aspiration site immediately after procedure

Types of GU dysfunction

- Motility disorders: GERD, Hirchsprung

- Obstructive disorders: plyoric stenosis, intussception

- Inflammatory disorders: appendicitis

GERD

Inadequate muscle tone of the sphincter located at the entrance of the stomach, resulting in a reflux of stomach contents

Manifestations of GERD

Upper respiratory manifestations, hoarseness, irritability, cyanotic spells (b/c of upper resp effects), vomiting, crying with feeding, Failure to thrive

Is GERD normal in peds?

some reflux is normal, especially in infants 4-12 months, there more prone to reflux b/c sphincter is not mature

Children at risk for GERD

- children with a neurological condition (high or low muscle tone)

- obese children

- preterm infants

- children with a chronic lung disease- increased abdominal pressure from coughing and hyperinflation

- children with tracheoesophageal fistula- poor GI motility

- any condition that creates increased pressure on the abdomen

how to diagnose GERD

pH probe or barium swallow x-ray

pH probe

gold standard test, 24 hours monitoring for reflux, inserted via nares into lower esophagus, sensors along the probe register level/duration of acid reflux, involved and expensive but the most reliable

Barium Swallow x-ray

shows GI anatomy, child drinks barium contrast material followed by x-ray, reflux of barium can be seen, opague- seen on radiography, can be done outpatient

Potential complications of GERD

- Esophagitis from acid: chronic blood loss, food refusal

- Baretts esophagus: inflammation and scarring, cancer risk

- Chronic infections from high level reflux: aspiration pneumonia, sinusitis, otitis media

- Failure to Thrive: repeated emesis, food refusal, developmental delay

- Acute Life Threatening Event: aspiration, death

Non-medical treatment for GERD

- Anti-reflux positioning

- Thicken feeding: 1 tsp to 1 tbsp rice cereal

- avoid bedtime feeding

- avoid caffeine

- Position prone with head elevated 30 min after feeding- infants must be monitored if sleeping

- decrease volume of feedings and increase frequency

- avoid tight clothing

- avoid rigorous play after feedings

Naso-jejunal tube feeding and GERD

feeding into the jejunum prevents reflux since the stomach is bypassed, can't do a large volume, small volume but continous

GERD medications

- H2-receptor antagonists (reduce gastric acid): Ranitidine, cimetidine

- Proton pump inhibitors (reduce gastric acid secretion and increase LES tone, and heal): omeprazole, lansoprazole, pantoprazole (IV only)

- Motility agent (move food through GI tract): metoclopramide

- Sucralfate is given to coat the esophagus if esophagitis is present

Surgical intervention for GERD

Nissen fundoplication with or without G tube placement (fundus wrapped tightly around lower espohagus to prevent reflux)

complications of Nissen Fundoplication

Dumping syndrome, loosening of the surgical wrap may lead to GERD reoccurence, Gas-bloat syndrome

Dumping syndrome

Gastric contents empty too quickly into duodenum leading to discomfort, tachycardia, and diaphoresis. Giving feedings more slowly may help.

Gas-bloat syndrome

inability to burp or vomit d/t the tight surgical wrap, if the patient has a G-tube then venting the tube can provide release of gas or allow regurgitation of the stomach contents

care for enteral tubes

elevate HOB for feedings, provide non-nutritive sucking for infants with pacifier, use equipment specifically for enteral feeding (IV tubing/pumps are not used for feeding b/c that can lead to serious errors), verify tube placement prior to use: each tube is different in placement verification, use clean technique: wear clean gloves

NGT

nose to stomach, inserted by nurse, placement check by x-ray or air auscultation or aspiration of stomach contents, feeding by gravity or pump and continous or bolus

NJT

nose to jejunum, inserted by nurse, placement checked by x ray or length of tubing exposed, feeding technique pump only continous only

GT

abdomen surface to stomach, inserted by surgeon, placement checked by tube appearing intact or aspiration of stomach contents, feeding technique through gravity or pump and continous or bolus

JT

abdomen surface to jejunum, surgeon, inserted by surgeon, placement checked by tube appears intact, feeding by pump only and continous only

Hirchsprung disease patho

lack of ganglion cells in a section of the bowel leads to un-opposed sympathetic stimulation leads to constricted bowel in aganglionic section of bowel causing blockage of stool proximal to aganglionic bowel

Hirchsprung disease

congenital agonglionic megacolon, lack of ganglion cells of the intestinal tract, typically involves the rectum and distal colon, greater risk in males, increased risk in Trisomy 21

Hirchsprung manifestations

abdominal distention, vomiting- may be bilious, constipation, meconium ileus (thick and sticky, blocks intestine), ribbon-like stool, foul-smelling stool, dilated proximal colon on x-ray

Diagnostic testing for Hirchsprung

- Rectal biopsy: tissue specimen taken to evaluate presence or absence of ganglion cells

- Barium enema: used to evaluate location of the "transition zone"- the area between normal bowel and aganglionic bowel

treatment for Hirchsprung

surgery with the goal of removing the aganglionic section of the intestinal tract to allow normal stooling

types of surgery for Hirchsprung

1. pull through procedure- aganglionic section is resected and the normal intestine is pulled through and anastomosed to the rectum

2. two step procedure: the aganglionic section is resected and a diverting ostomy is created, re-anastomosis to the rectum is performed at a later date after inflammation resolves

Preop care for Hirchsprung surgery

NPO, serial abdominal circumference to monitor for enterocolitis, TPN and lipids for nutrition, cleanse bowel- saline enemas, IV antibiotics- prevent postop infection, neomycin enema- reduce intestinal flora to prevent postop infection

postop care for Hirchsprung surgery

TPN, IV antibiotics, NGT to low intermittent suction, for pull thorugh frequent diaper changes and protective skin barrier d/t loose stool as well as rectal dilations to prevent rectal strictures at the anastomomosis site, ostomy care for 2 step procedure

Complications of Hirchsprung

Enterocolitis, stool incontinence (leakage of stool can occur after surgery), Short bowel syndrome, anastomatic strictures

enterocolitis

acute bowel inflammation that can lead to perforation, peritonitis, and shock. Manifestations include fever, abd distention, and tenderness, explosive diarrhea. Can occur before or after surgery

Short bowel syndrome

decreased intestinal surface area due to significant removal of diseased bowel. Malabsorption of nutrients, diarrhea, bacterial overgrowth, and dysmotility of the intestines can occur. Can occur after surgery

Anastomotic strictures

can occur after surgery, rectal dilation may be required

Pyloric stenosis

pyloric sphincter hypertrophies causing a narrow outlet from the stomach, develops shortly after birth, risk factors: male, first born child, and caucasian

Pyloric stenosis manifestations

- Palpable olive shaped mass over abdomen

- non-bilious and projectile vomiting

- Failure to thrive

- dehydration and electrolyte imbalance

- metabolic alkalosis from repeated emesis ( serum pH over 7.45)

dehydration and electrolyte imbalance s/s

irritability, weakness, lethargy, sunken fontanel and lack of tears, poor urine output, dry mucus membranes

Diagnostic testing for pyloric stenosis

ultrasound shows thickened pylorus

pyloric stenosis preop care

monitor electrolytes and venous pH, correct dehydration, electrolyte imbalance, and metabolic alkalosis with IV fluids, insert NG tube to low intermittent suction.

pyloric stenosis operative procedure

laparoscopic pyloromyotomy, incision through the pylorus muscle

pyloric stenosis post op care

control incision pain, monitor vitals, monitor Is and Os, small feedings, incision monitoring and care

intussusception

Telescoping of the bowel and mesentery leads to the following series of events: (lymphatic and venous obstruction, edema and ischemia of bowel wall, bleeding and increased mucous in the bowel), unknown cause, increased incidence in young children over 5 years of age, male incidence greater then females

intussusception manifestations

currant jelly stool d/t intestinal bleeding and mucous production, heme and stool, intermittent and crampy abdominal pain, bilious vomitting, sausage-shaped abdominal mass

intussusception diagnostics

ultrasound

intussusception complications

bowel perforation and peritonitis

bowel perforation conditions of risk

Appendicitis, intussception, enterocolitis, abdominal trauma, GI ulcer

bowel perforation manifestations

fever, abdominal pain and distention, nausea and emesis, elevated WBC, signs of sepsis

bowel perforation interventions

NPO with IVF, NGT to LIS, pain control, monitor GI distention/ VS/ bowel sounds/ lab results, antibiotics