OBY and PEDS EXAM 3

Hemophilia- Bleeding disorder

Group of bleeding disorders due to coagulation factor deficits hemophilia A ( factor VIII) “classic”, 80% of cases= x-linked recessive and 1/3 of new cases are new mutations= hemophilia B (factor IX), also x-linked recessive and known as “Christmas diesease”

Von Willebrand Disease

Issue with platelets + von Willebrand factor
affects platelet sticking + clotting

Hemophilia- Development Considerations

Infant- circumcision and Toddler- prone to injuries

Hemophilia- Assessment

excessive bleeding, joint pain and stiffness, impaired mobility, easy bruising/hematomas with minor injuries, activity intolerance, active bleeding: gums, epistaxis, hematuria, tarry stools

Hemophilia Complications:

airway obstruction: bleeding into neck, chest, mouth, Intracranial hemorrhage: headache, slurred speech, decreased LOC

Hemophilia expected lab findings

prolonged PTT, factor-specific assays, platelets and PT are normal, and genetic testing to identify family carriers

Hemophilia- Nursing considerations

Avoid IM injections, give SQ if possible, no rectal temps, avoid skin punctures and if needed maintain surgical aseptic technique, venipuncture preferred over finger/heal stick, monitor urine and stool for blood, acetaminophen not aspirin, RICE ( Rest, Ice, Compression, Elevate), PT/OT for ROM exercises to maintain mobility

Hemophilia- Medical Management

DDAVP (desmopressin): synthetic vasopressin; IV; increase factor VIII ( Hemophilia A only), give prior to dental /surgical procedures

IV Factor Transfusions:
Proven effective in reducing complications in children, outpatient/home therapy

Corticosteroids: watch for infection, avoid NSAIDS (potential inhibition platelet function)
EACA, amicar: inhibit clot destruction (antifibrinolytic)
Birth control: menorrhagia managment
Future: gene therapy

Hemophilia- Client Education

Close supervision and safe environment, medical identification bracelet, low contact sports only ( bowling, fishing, swimming, golf), dental procedures in controlled environment; minimize flossing; soft brush, shave with electric razor only, pressure for 15 min + ice for superficial bleeding, significant bleeding=seek care for factor replacement

Thalassemia

Inherited blood disorder of hemoglobin synthesis, autosomal recessive with varying expressivity. Mediterranean ( Greek and Italian), Asian, Middle Easter descent

Thalassemia minor

asymptomatic silent carrier

Thalassemia trait

mild microcytic anemia

Thalassemia intermedia

moderate to severe anemia + splenomegaly

Thalassemia major (Cooley anemia)

severe anemia requiring transfusions to survive

Beta Thalassemia Manifestations

Bone malformations (maxillary, frontal lobe), osteoporosis and osteopenia, splenomegaly and hepatomegaly, cardiomyopathy, short statues, yellow or bronze skin pigmentation

Beta Thalassemia Treatments:

Transfusion therapy (severe anemia) - CVC placement, chelation therapy ( free iron buildup), bone marrow transplantation, monitor effects on growth and development, splenectomy, growth hormone replacement

Idiopathic Thrombocytopenic Purpura (ITP)

an acquired hemorrhagic disorder characterized by: thrombocytopenia ( excessive destruction of platelet), purpura (discoloration caused by petechiae beneath the skin

It involves the evolution of antibodies against multiple platelet antigens, leading to reduced platelet survival and impaired platelet production

ITP Forms

Acute self-limiting, often follows upper respiratory tract or other infections (childhood diseases) a

Chronic: (greater than 12 months duration)

ITP Treatments

Steroids

Intravenous Gamma Globulin (IV infusion thought to bind with Fc receptors on platelet antibodies to produce more)
Anti D antibody: plasm-derived immunoglobulin that causes a transient hemolytic anemia, age 1 and 19 yrs old; Rh(D)-positive blood type, no suspicion of SLE, no active infection, no previous reaction to plasma products, no splenectomy, no active bleeding, platelet count 20,000 or above
- If Anti D given, pre-medicate with acetaminophen and monitor the patient for one hour after administration, with IV access readily available

PEDS Genitourinary system: Renal and Urethral Structure

Larger kidneys in relation to abdomen, urethral opening close to rectum, shorter urethras

PEDS Genitourinary system: Renal and urinary function

All nephrons present at birth but immature, reduced glomerular filtration rate, unable to concentrate urine, less urine output per day but more voids, lower blood urea, nitrogen and creatinine levels

PEDS Genitourinary system: Reproductive organ structure and function

Immature structure and function until puberty (typically during adolescence)

Normal Characteristics of Urine

Color range, clear, in newborns, production is 1 to 2 mL/kg/hr, in children production is about 1ml/ kg/hr

Normal urinalysis

pH: 5 to 9
Specific gravity: 1.001 to 1.035
Protein: less than 20 mg/dL
Urobilinogen: Up to 1 mg/dL
NOT normal: glucose, ketones, hemoglobin, WBC, RBCs, casts, nitrites

Genitourinary Assessment: Urine and voiding characteristics

Color, clarity, odor, amount, and frequency

Pain or discomfort: pain during urination or in flank and genital areas

Genitalia: urethral location, presence of testes, discharge or drainage, and presence of lesions or redness

Edema: Location, severity, and pitting or nonpitting

Diagnostic Studies: CT, cystoscopy, renal biopsy, blood urea nitrogen, creatinine, and culture

Urine output:

-Infants: 1-3 mL/Kg/h
-children: 0.1-1 mL/kg/h

Nursing Interventions for Genitourinary Disorders

Maintain adequate hydration
Maintain normal fluid and electrolyte balance
Meet nutritional requirements
Prevent Infection
Promote bladder emptying
Promote Pain relief and comfort

Promote incisional healing and prevent skin breakdown
Provide emotional and psychosocial support

Administer and manage medications

Etiology and Pathophysiology of UTIs (1)

Physiologic and mechanical defense mechanisms that maintain sterility: emptying the bladder, normal antibacterial properties of the urine and tract, ureterovesical junction competence, peristaltic activity

Etiology and Pathophysiology of UTIs (2)

Contributing factor is urologic instrumentation: allows bacteria present in the opening of the urethra to enter the urethra or bladder

Sexual intercourse promotes: “milking” of bacteria from the perineum and vagina and may cause minor urethral trauma

Pediatric Clinical Manifestations of UTIs

Symptoms vary depending on age, urinary frequency, fever in some cases, odiferous urine, blood or blood-tinged urine, even with significatn bateria, a pediatric patient may have no symptoms or nonspecific symptoms such as fatigue or anorexia

Enuresis

Incontinence of urine past the age of toilet training, diurnal versus nocturnal, primary or secondary, assess for asymptomatic UTI

Multifaceted approach for treatment: fluid restriction, bladder exercise, timed voiding, enuresis alarms, antidiuretic hormones, tricyclic antidepressants, anticholinergics

Inguinal Hernia

Intra-abdominal structures protrude through defects in abdominal wall
- Incarcerated hernias require immediate medical attention to prevent bowel ischemia
-present with palpable, round, smooth, and nontender mass
-Size of bulge increase when infant is crying or straining
-Elective, outpatient surgical correction unless incarceration
-hernia incarceration requires requires emergent surgery to prevent ischemia

Acute Pyelonephritis

Kidney infection; severe type of UTI with systemic symptoms

Key symptom of pyelonephritis

Costovertebral angle (CVA) tenderness

Systemic symptoms of pyelonephritis

Fever, chills, nausea, vomiting, anorexia

Urinary symptoms of pyelonephritis

Frequency, urgency, nocturia, dysuria

Pain associated with pyelonephritis

Low back pain and suprapubic pain

Urine findings in pyelonephritis

Hematuria, foul-smelling urine, bacteriuria, pyuria

Important clinical note (Acute pyelonephritis)

Symptoms may improve, but infection (bacteriuria/pyuria) can persist

Diagnostic Studies of Acute pyelonephritis

Urinalysis, WBC casts, CBC, Imaging studies (IVP or CT), ultrasound

Collaborative Care of Acute Pyelonephritis

Hospitalization, parenteral antibiotics
- relapses treated with 6-week course of antibiotics
-reinfections treated as individual episodes or managed with long-term therapy
- prophylaxis may be used for recurrent cases

Phimosis

Foreskin of the penis cannot be retracted ( normal in young infants)
- Normal during the newborn period; pathological after the toddler stage
- Paraphimosis if prepuce cannot be pulled over glans when foreskin retracted
- venous stasis causing edema and severe pain
- presents with irritation, bleeding, erythema, pain, dysuria, and discharge
-treat with topical steroid creams twice daily for 2 to 8 weeks

-Paraphimosis: is a medical emergency; reduction required, and circumcision can treat the condition

Cryptorchidism

Undescended testes: one or both testes fail to descend through inguinal canal into scrotum
-mechanical, hormonal enzymatic, or chromosomal
- asymptomatic but absence of testicles in scrotal sac

-conduct physical exam in warm environment with a calm child

-treat with orchiopexy (inguinal incision and mobilization of testis and spermatic cord) b/w 6 and 15 m of age
-Infertility and increased risk for cancer if untreated

Cryptorchidism ( interdisciplinary care)

Most testes spontaneously descend, surgical procedure, orchiopexy, if testicles do not descend into the scrotal sac by 6 to 15 months of age
-Hormone therapy- human chorionic gondadotropin
-slightly higher risk of testicular cancer if untreated

-In the teen or adult the testicle would be removed

Inguinal Hernia

Intra-abdominal structures protrude through defects in abdominal wall

-incarcerated hernias require immediate medical attention to prevent bowel ischemia

-Present with palpable, round smooth, and nontender mass

-size of bulge increase when infant is crying or straining

-elective, outpatient surgical correction

Hydrocele

fluid accumulation in the scrotal sac, typically noncommunicating and self-limiting; resolves by 1 year of age
-transillumination of the scrotum to confirm fluid collection

- In persists beyond 12 to 18 months likely noncommunicating and require surgical repair

Hypospadias and Epispadias

urethral meatus is not at the end of the penis, hypospadias on ventral side, epispadias on dorsal side, can occur at any point on the shaft, observed soon after birth when urine stream originates form alternative location on penis

• assess for chordee (fibrous band causing downward curve)
  -avoid circumcision because foreskin is used in repair, surgical correction b/w ages 6 to 12 months, double-diapering postoperatively

Hypospadias

Urethra on ventral (underside)

Epispadias

Urethra on dorsal (top)

Normal vs abnormal urethral meatus location

Normal = tip of penis; defect = opening not at tip

Possible location of urethral opening defect

Can occur anywhere along penile shaft

How hypospadias/epispadias is identified

Abnormal urine stream direction noted after birth

Chordee (associated complication)

Fibrous band → downward penile curvature

Circumcision in hypospadias/epispadias

Avoid—foreskin needed for surgical repair

Treatment for hypospadias/epispadias

Surgical correction to reposition urethra

Timing of surgical repair

Usually 6–12 months of age

hypospadias/epispadias medical management

circumcision not recommended, foreskin may be needed for reconstructive surgery, release of tight chordee, placement of urethra opening at head of penis, surgery recommended at around six to nine months of age, long term outcomes (leaking at the site, and body image)

Testicular torsion

Testicular torsion is a medical emergency that requires immediate intervention, failure to surgically repair the defect can lead to necrosis and testicular loss. Hypospadias and epispadias require surgical intervention, but it is not emergent. Phimosis does not typically require surgical intervention.

Vesicoureteral Reflux

• Retrograde flow of urine from bladder unto ureters and renal pelvis and calyces during voiding

• may occur in one or both ureters

• increased risk for UTI and pyelonephritis

• Grades I to V based on degree of backflow

• Asymptomatic without UTIs

• Voiding cystourethrogram to diagnose

• prophylactic antibiotics at bedtime if low grades

• surgical corrections if high grades

Therapeutic Managment of VUR

• prevent bacteria from reaching the kidneys

• low does antibiotic therapy

• urine culture every 2 to 3 months or any time a child has a fever

• VCUG to assess the status

• Prognosis: most children outgrow VUR, but for severe forms surgical intervention may be needed

Nephrotic Syndrome

• Kidney damage and too much protein in the urine

• congenital, primary or secondary

• increased glomerular basement membrane permeability

• almost exclusively albumin lost in urine

• primary manifest in three ways: minimal change, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis

• profound generalized edema developing several weeks

• reduce edema, decrease proteinuria, manage symptoms, improve nutrition, and prevent infection

Nephrotic Syndrom Characteristics

most common presentation of glomerular injury in children
- characteristics: massive proteinuria (loss of 15 grams/days), hypoalbuminemia, hyperlipidemia, edema

Chidren with Nephrotic Syndrome

periorbital edema, swelling of face, swelling of abdomen and legs

Nephrotic Syndrom (MCNS) pathophysiology

glomerular membrane, normally is impermeable to large proteins, becomes permeable to proteins, especially albumin, albumin is lost in urine=hyperalbuminuria, serum albumin is decreased= hypoalbuminemia, fluid shifts form the plasma to the interstitial spaces: hypovolemia, ascites

Diagnosis of MCNS

suspected based on clinical manifestations

• generalized edema (develops gradually or rapidly)

• proteinuria 2+ on dipstick testing

• hypoalbuminemia

• hypercholesterolemia ( in the absence of hematuria and hypertension)

• low serum protein and sodium

may require renal biopsy to distinguish from other types of nephrotic syndrome

Management of Nephrotic syndrom

Supportive Care
Diet

• low to moderate protein

• sodium restrictions when large amounts of edema are present

Steroids (first line of therapy)

• dose is 2 mg/kg divided into twic-a-day doses

• prednisone is the drug of choice (least expensive and safest)

Immunosuppressant therapy ( cyclophosphamide [cytoxan])

Diuretics

Family Issues with MCNS

Chronic condition with relapses

• developmental milestones

• social isolation: lack of energy, immunosuppression, protection, change in appearance due to edema affects and self-image

Nursing Interventions for MCNS

Aseptic technique during cath, avoid unnecessary caths and early removal of indwelling catheters

Prevent nosocomial infections: wash hands before and after contact, and wear gloves for care of the urine

Nursing Inteventions for MCNS

Routine and thorough perineal care for all hospitalized patients

• avoid incontinent episodes by answering the call light and offering the bedpan at frequent intervals

Nursing Interventions for MCNS (cont’d)

Ensure adequate fluid intake (patient with urinary problems may think drinking fluids will be more uncomfortable)
- dilute urine, making the bladder less irritable

• flushes out bacteria before they can colonize

• avoid caffeine, alcohol, citrus juices, chocolate, and highly spiced foods

Potenital bladder irritants

Nursing Interventions for MCNS

Discharge- to- home instructions
- follow-up urine culture

• recurrent symptoms typically occur 1 to 2 week after therapy

• encourage adequate fluids even after infection

• low-dose, long-term antibiotics to prevent relapses or reinfections

• explain the rationale to enhance compliance

Acute Poststreptococcal Glomerulonephritis

Inflammation and cellular proliferation of the glomeruli not caused by direct infection of the kidneys

• follows exposure to group A beta-hemolytic streptococcus

• Impaired capillary perfusion and reduced glomerular filtration

• Typically affects children from 4 to 12 yrs old

• Latency period 1 to 2 weeks after pharyngitis until symptoms develop

• Presents with gross hematuria, edema, and HTN

• therapeutic interventions to maintain blood pressure and fluid volume status

Acute Glomerulonephritis (AGN)

Types: most are postinfectious ( pnemococcal, streptococcal, or viral), may be a primary event

or may be a manifestation of a systemic disorder: systemic lupus erythematosus (SLE), sickle cell disease, other

Acute Glomerulonephritis (AGN)

Symptoms

Generalized edema due to decreased glomerular filtration: especially in periorbital area, facial edema is more prominent in the morning, spreads during the day to involve the lower extremities and abdomen

Hypertension: due to increased extracellular fluid, oliguria (severely reduced volume)

Acute Glomerulonephritis (AGN)

Symptoms (cont’d)

Hematuria: bleeding in the upper urinary tract, resulting in smoky brown urine ( resembling tea or cola)

Proteinuria: increased amount of protein signifies increased severity of renal disease

Nursing Management of Acute Post-Streptococcal Glomerulonephritis (APSGN)

Monitor for acute hypertension: monitor the blood pressure every 4 to 6 hours

manage edema: daily weight, accurate I&O, daily abdominal girth

Nutrition: low sodium, low to moderate protein

Susceptibility to infection (give penicillin if +strep) bed res is not necessary

Hemolytic Uremic Syndrome

Thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and acute renal failure

• main cause of acute kidney injury

• usually preceded by diarrhea

• mainly caused by shiga toxins and verotoxins

• most common in preschool and school-aged children

• presents with subtle onset with vomiting, abdominal pain, anorexia, and ascites

• microangiopathic hemolytic anemia , thrombocytopenia, and acute kidney injury

• symptomatic management

Hemolytic-Uremic Syndrome

Pathophysiology: enteric infection of E.coli o157:H7
- undercooked meat, unpasteurized fruit or juice, alfalfa sprouts and other greens, lettuce, salami
Contaminated drinking or swimming water

Pathophysiology of HUS

Injury to glomelular arterioloes which become swollen and occluded due to fibrin and clots. RBC try to pass through and become damaged along with platelets. Damaged red blood cells clog the filtering system of the kidneys leading to renal failure

HUS symptoms

Spleen removes RBCs and platelets resulting in hemolytic anemia, thrombocytopenia
- renal failure: lab tests

• decreased hct, hgb

• decreased platelets

• increased BUN and creatinine

Symptoms of HUS

vomiting, irritability ,lethargy
- marked pallor

• bruising, petechiae, jaundice, bloody diarrhea

• oliguria, anuria
  
  CNS: seizures, stupor or coma

• Sometimes signs of acute renal failure
  set up seizure precautions

HUS therapeutic managment

peritoneal or hemodialysis in any child that is anuric for 24 hrs
- or any child who is oliguric with uremia or HTN and seizures

• electrolyte and fluid retention

• sometimes FFP or blood transfusions to treat anemia, SOB, anemia

• platelet transfusion

• 95% recovery rate

• some long term complications: HTN, chronic renal failure, CNS injury

Wilms Tumor

Etiology
Diagnostic evaluation

Therapeutic management: surgical removal, chemotherapy and /or irradiation
Nursing considerations= do not palpate abdomen ( put sign over bed)