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31 Cards in this Set

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HIRSCHPRUNG's DISEASE (5 questions)
* newborn: failure to pass meconium
*older child: constipation, FTT, rectum could be empty.

s/s: distension, fever, bloody diarrhea, PENCIL THIN STOOLS.

Lack of innervation to colon.

More common in males. Congenital.

Best confirmation by biopsy.

RX: removal of bowel section. Reanastomosis: around 2-4 months or later (will have colostomy until). ABX, NGT for decompression. Monitor abdominal girth.

TEACHING: (newborn) if no passage of meconium before discharge, inform parents to notify if no stool for 48h or abdominal distension.
(older): bowel irrigation with NS.
(postsurgical): fever, ostomy care.
INTUSSECPTION (5 questions)
Prolapse of one portion of the colon into another. Most frequent cause of intestinal obstruction in infancy. More common in boys (3mo-6yo).

Most common site is ileocecal valve. Walls rub together causing inflammation, edema and decreased blood flow. Can lead to necrosis, perforation, hemorrhage and peritonitis.

ONSET IS ABRUPT. Previously healthy child with sudden onset of abd pain and fecal emesis. Pain may come and go.

STOLL BECOMES RED-CURRANT-JELLYLIKE.

PALPABLE MASS URQ.

DX via US, xray.

**barium from DX may resolve issue and move it back into place** if not then surgery.

Strict I&O, abd girth.

Postop: NGT patency, electrolytes. Bowel sounds.

BEGIN clr liquids AFTER bowel sounds return. Progress at half strength to full feedings.

Can be discharged AFTER a return to normal eating. Parents report fever or decrease in appetite.

**PASSAGE OF NORMAL STOOL MAY INDICATE RESOLUTION **
IMPEROFRATE ANUS (2 questions)
DX at birth. May appear as only a dimple.

Stool in urine may indicate fistula. May be part of VACTERL syndrome (congenital abnormalities that include anorectal malformations as well as vertebral, CHD, TEF, radial limb defects).

Stenosed anal openings can be managed with dilation.

AN IMPERFORATE ANAL MEMBRANE IS EXCISED SURGICALLY THEN FOLLOWED BY DAILY MANUAL DILATIONS.

** temporary colostomy is performed to rest the bowel after reconstruction - generally closed in a few months**

May never be able to be bowel retrained.

RN care: IVF and NGT for decompression.
(post op) NO RECTAL TEMPS AFTER SURGERY. Advance diet from clear to breast milk slowly. Colostomy care.

Teaching: monitor for infection.
APPENDICITIS (1 question - patho)
Inflammation of veriform appendix. Most often in adolescent boys. Most common cause for emergency abdominal surgery in peds.

Incidents of ruptured appendix much higher in kids <4 due to inability to verbalize symptoms such as pain and nausea.

Caused by fecalith, parasite, stenosis, hyperplasia of lymph tissue or tumor.

**Pain will be half way between anterior superior illiac crest and umbilicus: MCBURNEYS POINT **

Need differential DX with older females (menstrual, ectopic, etc).

Symptoms: guarding, rigidity, rebound tenderness. vomiting, constipation or diarrhea. Child will remain motionless in a side lying position with knees raised.

RUPTURE: fever with sudden relief of pain, progressing to distension, rapid breathing, pallor, chills and irritability.

LABS: increased WBC (>15K) and elevated C-RP. CT is most reliable.
CELIAC DISEASE (1 question: AX)
GLUTEN sensitive enteropathy is a chronic malabsorption syndrome. Fairly common (1 in 133 people). High incidence in kids with Down syndrome.

early stages: excretion of high amounts of fat (steatorrhea). Greasy and foul smelling, frothy and excessive. Leads to decreased absorption of A,D, E, K and B12 (fat soluble vitamins).

s/s: chronic diarrhea, vomiting, irritability, malabsorption, abd pain, FTT. Can lead to protein deficiency (small extremities, big belly). Calcium problems (teeth and bones).

DX: fecal fat content. AGA antibodies elevated.

Symptoms improve with dietary changes. Villi return to normal in 6 months. Growth will improve steadily (normal in one year). May require vitamin supplements.

Life-long diet modifications.
UTI (5 questions)
Cystitis: lower (bladder and urethra)
Pyelonephritis: upper (ureters, renal pelvis, renal parenchyma).

Second most common infection in children. More common in females.

Many first UTIs are caused by E.coli.

Urinary stasis increases risk. Incomplete emptying (school age children).

Vesicouretal reflux: backflow from bladder to ureters during voiding.

Risk of kidney damage:
*Uti in less than 1 year old.
*delayed DX and RX for upper UTI
* anatomic obstruction or nerve interruption
*recurrent upper UTIs

S/S: (young)FUO, FTT, poor feeding, V/D, strong smelling urine and irritability.

Any child under 2 with FUO should be worked up for UTI.

Toddlers and older have classic signs:
* Lower: frequency, dysuria, urgency, enuresis, strong smell, cloudy, hematuria, abd pain.
* Upper: high fever, chills, abd pain, flank pain, CVA tenderness, persistent vomiting, dehydration.

UA/culture needed. Can dipstick (leukocyte esterase test IDs WBC and pyuria - nitrate dip detects gram negative bacteria).

>100K fcu is positive confirmation (clean catch - 50K for sterile cath). Collection bags are not sterile and cant be confirmed.

Specimens must be delivered within one hour or refrigerated.

Assess child for "toxic appearance (very ill). Collect early morning urine (more concentrated).

RX: ABX, promote rehydration, assess renal function. Encourage frequent voiding. I&O. Daily weights. In children, expect 1mL/kg/hr output.

Toilet training may regress. Cotton rather than nylon underwear.
Hypo/Epi spadius
Congenital. Abnormal location of urethra in males.

Hypo: 1 in 125 males.
Epispadius: 1 in 40K males

Can be mild (just off center), or severe (in the scrotum). Often occurs with chordee (bowing of penis).

Epispadius is a mild expression of exstrophy (where the bladder is outside the body).

Should not be circumsized, skin can be used to surgery. In 1st year of life, often outpatient. May receive anticholinergics to relieve bladder spasms.

Post op: simple dressing with a stent. May have blood but urine should progress to no visible blood vithin a few hours. Encourage fluids. Strict I&O. No urine for 1 hour may indicate obstruction.

Double diaper to protect stent. Limit activity (2 weeks). No holding on hip! Complete ABX.

Call DR if: swollen/discolored penis, redness/swelling/drainage, excessive bright red bleeding, urine from anywhere but catheter, fever not resolved with tylenol.
NEPHROTIC SYNDROME (9 questions)
not a specific disease, a clinical state. Characteristics:
edema, MASSIVE proteinuria, hypoalbuminemia, hypoproteinemia, hyperlipidemia, and altered immunity.

Results from a disease that affects only the kidneys such as glomerulonephritis.
90% of kids with NS have a primary disease called minimal change nephrotic syndrome (MCNS). Generally between 2-7 years old. More common in males. Immune connection is strongly suspected. Often preceded by respiratory infection (2-3d).
Increased glomerular permeability lets negatively charged molecules (albumin) to pass and be excreted. Results in edema - fluid remains in interstitial spaces. Immunoglobulins are also lost, reducing immunity. Liver responds by increasing synthesis of lipoprotein. Can lead to acute renal failure.

s/s: edema develops over several weeks (periorbital, snug clothes). HTN, irritable, anorexia, hematuria, oliguria, malaise. URINE MAY BE FROTHY OR FOAMY. Respiratory distress. Massive weight gain related to loss of albumin and sodium intake.

Malnourishment: plae, shiny skin, brittle hair, increased risk of thrombosis.

DX: UA, serum albumin, NA, BUN, cholesterol, electrolytes.
albumin <25g/L and urine protein of >40mg/m2/h are diagnostic.

Relapse testing: dipstick urine +2 for 3 consecutive days.

RX: Corticosteroids used to treat proteinuria (fall in 2-3 weeks). Will take for 6 more weeks, then 6 weeks of e/o day. 90% have full remission.

70% relapse. Often associated with resp infection or live virus immunizations (withhold immunizations for 6 months after completing corticosteroid therapy).

Also may get antiHTN, diuretics, ABX. Immunosuppressives in extreme cases of repeated relapse. IV albumin. No salt diet should be used when on corticosteroids.

Weigh daily. strict I&O. Abdominal girth. Test urine q-shift. Assess for skin breakdown. Watch for s/e of steroids (moon face, increased appetite, hair growth, distension,mood swings, nausea and hyperglycemia).

Teaching: prevent infection (steroids may mask symptoms). Protect skin - keep clean and dry, turn frequently, use cotton to separate skin on skin contact. Emotional support (allow choices when possible). Weight weekly to monitor for changes (possible relapse). Bone density testing if on steroids longterm.
LEUKEMIA ( 8 questions)
Most common malignacy in peds under 14 (peak at 2-3). Cancer of blood forming organs. Proliferation of WBCs. ALL and ANLL.

ALL: 25% of all ped CA and 78% of leukemias. More common in whites and boys. Effects B and T lymphocytes.

ANLL: 17% of leukemias./ More common in under 2 years old. Affects myeloid cells (platelets, RBCs and granulocytes (neut/baso/eosin).

Leukemia occurs when stem cells in the bone marrow produce immature WBCs that dont function normally. Proliferate rapidly. Fills bone marrow. Spiil intot he circulatory system. Leaves body vulnerable to infection.

Fills bone marrow - replaces stem cells and can lead to anemia and abnormal bleeding.

s/s: fever, pallor, overt blleding, lethargy, malaise, anorexia, joint pain. Petechiae, frank bleeding and joint pain are cardinal signs of bone marrow failure. Hepatosplenomegaly and lymphadenopathy are common. If it has entered CNS they will have HA, vomitting, papilledema (eyes) - nerve pressure.

DX: blood counts, bone marrow aspiration. 25% lyphoblasts is definitive.

RX: radiation and chemotherapy (see slide).

Most important factor in a good prognosis: initial leukocyte count. Higher is worse. Under 12months have a poor prognosis.
10% relapse within one year.

Teaching: avoid infection. Mouth care. Attention to renal function when receiving cyclophosamide. Will receive fluids at 1.5x normal before and after RX.
RADIATION
Induction: prednisone, vincristine, L-asparaginase, daunorubicin
consolidation: L-asparaginase, doxorubicin
Delayed intensification: vincristine, ARA-C, cyclophosamide
Maintenance: methotrexate, 6-mercaptopurine, 6-thioguanine

Maintenance may go on 2-3 years (decreases infection resistance).

RX for ANLL is similar but uses some different drugs.

Maximum cell death occurs during induction.
CHD (general)
defect of heart, great vessels or persistence of a fetal structure at birth. 1%. Most develop during 1st 8 weeks of gestation. Combined genetic and environmental factors.

ex: exposure to phenytoin, warfarin, alcohol/ maternal rubella / IDM, phenylketonuria / increased maternal age / maternal genetics / chromosomal abnormalities (marfan, turner, down syndrome).

1st indication is usually a murmur. 1/3 of infants born with CHD develop life-threatening symptoms within the first few days.
CARDIAC CATHETERIZATION
NPO for several hours. Asked to void and given PO sedative.
Monitor: VS, H&H, cap refill. Skin and neuro assessment for post op comparison.

Post op: monitor for arrythmias, bleeding, hematoma, thrombus, infection. Check cath site for bleeding and neuro/perfusion of LEs q15 for 1st hour, then q30 for one hour. CHECK UNDER BUTTOCKS TO MAKE SURE BLOOD HAS NOT RUN. Monitor I&O (contrast causes diuresis).

May take child on tour of cathlab.

Direct pressure for 15min after. Then pressure dressing for 6 hours - keep leg straight. Do not elevate HOB or flex hips. Limit activity fr 24 hours. Clear fluids then progress.
CHD that increase Pulmonary Blood flow

PDA
PATENT DUCTUS ARTERIOSIS:

Allow blood to flow between left and right side of heart. Blood is shunted to right side due to higher pressures form left side. Increased mount of blood sent to lungs. Pulmonary vascular resistance is increased to try to reduce blood flow as wells as pulmonary HTN. RVH develops to overcome increasing Pulmonary VR and deliver blood to the lungs.

s/s: increased HR, RR and metabolic rate. Diaphoresis occurs with feeding. May be unable to take in enough calories. Poor weight gain. If CHF develops: dyspnea, tachypnea, intercostal retractions, periorbital edema. Full bounding pulses. Widened pulse pressure. Thrill in pulmonic area.
High risk for frequent respiratory infections, pneumonia, infective endocarditis.
RX: surgical ligation. IV indomethacin stimulates closure.

10% of all CHD. Nearly all preterm infants under 27 weeks.
ATRIAL SEPTAL DEFECT
increased pulmonary blood flow
Hole between left and right atria, such as when the foramen ovale fails to close.

s/s: May be asymptomatic. May not know until preschool. May develop CHF, tire easily. Por growth with large ASD. Soft ejection murmur.

DX: echo reveals dilated RV.

RX: Some sponataeneously close. Surgery may be used. May use an occluder (umbrella).

10% of CHDs.
VENTRICULAR SEPTAL DEFECT
increased pulmonary blood flow
From LV to the pulmonary artery.

s/s: Only 15% are large enough to cause CHF. Systolic murmur.

DX: may see LVH/RVH.

RX: most small VSD close spontaneously in 1st 6 months. Treatment is conservative when CHF not present. Surgical patching if growth is poor. Can have tachyarrythmias.

*Most common CHD 40%
TETRAOLOGY OF FALLOT
decreased pulmonary blood flow (cyanotic)
4 defects: pulmonic stenosis, RVH, VSD and ovveriding aorta. Some also have open foramen ovale.

about 10% of CHD.

RIGHT to LEFT shunting.

Cyanosis that does not respond as expected to O2 is a classic sign.

Become cyanotic as DA closes.
s/s of chronic hypoxemia include: fatigue, clubbing, exertional dyspnea, delayed development. Diaphoresis and stopping during feeds. Poor weight gain. Polycythemia. Metabolic acidosis.

Hypercyanotic episodes can occur suddenly. Toddlers will often squat (instinctively) to relieve dyspnea - reduces cardiac output by dereasing venous return. Usually occurs between 2mon and 2 years of age.

TET speels: increased rate and depth of respiration, increased HR, cyanosis, pallor, poor tissue perfusion, diaphoresis, irrirability and crying, seizures and LOC.

DX: xray - boot shaped heart. RVH. VSD. Elevated H&H, increased clotting time.

RX: place infant in knee-chest during hypercyanotic episodes. Calming. O2. MS and propranolol IV. Monitor for metabolic acidosis.

Total repair bfore 6 months. May have palliative shubt placed first.

LIFELONG endocarditis prophylaxis is required!
PULMONIC STENOSIS
decreased pulmonary blood flow (cyanotic)
Narrowing in the area of the pulmonic valve. Increases preload. Results in RVH.

Second most common CHD (12%)

s/s: may be asymptomatic. DOE and fatigue. CHF (rare). HF in severe cases. Loud murmur and split s2/thrill.

DX: Cxry will show enlarged pulmonary artery. RVH. Increased RV pressure (cath).

RX: dilation by balloon valvuloplasty for simple cases. Surgical resection. Do not typically increase in severity.

LIFELONG endocarditis prophylaxis is required!
PULMONARY or TRICUSPID ATRESIA
decreased pulmonary blood flow (cyanotic)
no communication between RV and pulmonary artery. Only way blood flows to the lungs is through a patent DA.

s/s: cyanotic at birth. Tachypnea, CHF, pulmonary edema, hepatomegaly, acidosis, clubbing, ploycythemia, growth delays. Continuous murmur. RVH.

RX: prostoglandin E1 given IMMEDIATELY to maintain PDA. Also dig and diuretics. Surgery.
TGA - TRANSPOSITION OF THE GREAT ARTERIES
mixed defects
Occurs in 5%

RV goes to the aorta and LV goes to the pulmonary artery.

LIFE THREATENING at birth. Survival depends on a PDA and open foramen ovale.

s/s: cyanosis at birth, does nto respond to O2. Progresses to acidosis. CHF may develop immediately. Tachypnea (>60) without retractions or other signs. Loud S2. May have no murmur. Infants require a long time to feed.

RX: Prostoglandin E1 to maintain PDA. Surgery at 1 week. Survival without surgery is ipossible.
AORTIC STENOSIS
obstructing defect
anatomic stenosis that decreases CO and increases pressure load on LV. 3-6%.

s/s: Often asymptomatic. Can lead to pulmonary edema and CHF. Child may have cooler feet than hands, leg cramps, stronger pulses in UE than LE. Decreased blood to GI can lead to necrotizing enterocolitis.

RX: newborns with severe stenosis require PGE1 for PDA until the valve is dialted. Is usually progressive as valve calcifies. Valve replacement may be required. Lifelong endocarditis prophylaxis.
COARCTATION OF THE AORTA
obstructing defect
Narrowing or restriction of descending aorta, often near DA. 5% -

s/s: Child may have cooler feet than hands, leg cramps, stronger pulses in UE than LE (brachial and radial pulses bounding). Leg pain after exercise. Decreased blood to GI can lead to necrotizing enterocolitis.

RX: balloon dilation. Surgical resection.
CHF rx,med,path
CO is inadequate to support body. Most common cause in infants is blood volume overload due to CHD. 90% of time occurs between age 6-12 months.

Organ perfusion is inadequate. Kidneys respond by activating renin-angiotensin system to retain salt and water. Increased PVR, HR. Increases to pulmonary congestion and systemic edema.

s/s: slow onset, look for infant tiring during feeding.

dx: based on manifestations of tachycardia, resp distress, and crackles.

RX: diuretics. Monitor K+ levels (when not choosing spironolactone). Inotropics and ACEi can be given to reduce the heart's workload. Dig is given to improve contractility and increase CO. Initial dose is high (digitilization) and then followed by lower maintenance doses. Also BBs.

signs of Dig toxicity: (early) arrythmias. Early sings in adults are not common in peds. Therapeutic range is 0.8-2.0, over 2 is toxic. Take levels 6-8 hours after dosing. Can be affected by loss of intestinal therapy (ABX therapy). Monitor closely.

Abdominal measurements (ascites), weight diapers. Turn frequently and provide skin care.
LATEX ALLERGY
50% of Spina Bifida pts and 34% of patients with three or more surgeries have it.

Caused by an IgE mediated response that develops after repeated exposure. Have a high incidence of allergy to kiwi, bananas and avocado.
PSAGN
Group A beta-hemolytic strep infection of pharynx or skin. History of pharyngitis or tonsillitis 2-3 weeks before symptoms. 50% of kids self resolve.

Complications: renal failure, hypertensive encephalopathy (szr), pulmonary edema, heart failure.

AX: periorbital edema (mornings), anorexia, oliguria, hematuria, pallor, lethargy, HTN, proteinuria, azotemia (high nitrogen)increased BUN and creatinine, increased ASO titer.

in older child: HA, flank pain and dysuria.
Differences between PSAGN and NS
PSAGN: acute (infection) NS: slower onset
P: proteinuria NS: proteinuria and hyperlipidemia
P: hematuria NS: frothy urine
P: once NS: relapses
JRA
chronic autoimmune inflammatory disease.

More oftne in girls.

ages 2-5 and 9-12.

May be a few joints or systemic.

RX: drug therapy, and maybe surgery. NSAIDs used to reduce inflammation. May be treated with MTX if they do not respond. Should get eye exams q6 months. Warm compresses. At risk for altered growth related to closure of epiphyseal plates, contractures and steroid cuased growth inhibition.
PERTUSSIS
Most common <6 months. Can affect those with waning immunity then be spread to kids that have not been immunized.

Droplet and direct contact with discharge.

Incubation 7-10 days.

Can be communicated 7d after exposure (end of incubation). Can still expose others for 5-7 days after ABX.
IMPETIGO
Highly contagious skin disease of staph or strep. Most common around the face, mouth, hands, neck and extremities.
Most common bacterial skin infection in kids. Needs a portal (abrasion, insect bite, etc). May develop several lesions through self inoculation. Vesicle or pustule that progresses to exudative or golden honey crusted stage.

DX: visual inspection and a gram stain/culture.

RX: removal of crusts and topical ABX. Bullous impetigo needs systemic abx. Infectious for 48 hours after abx.
PINWORMS
Transmission from inhaled or carried eggs from hand to mouth. Hatch in upper intestine in 15-28 days. Migrate to cecum. Females migrate and lay eggs outside anus. Causes itching, irritability, restlessness, short attention span. Can migrate to the vagina. Best time to check is 3-4am with scotch tape.
LYME DISEASE
highest risk of infection in the summer. Children 5-8. Most common vector-born illness in the US. 1-55d incubation - a rash in 48 hours is not attributable to Lyme disease. Not contagious.

Localized: malaise, fatigue, stiff neck, mild fever. Growing rash that looks like a bruise.
If untreated can have more rashes, HA, fatigue, nerve palsies and meningitis.
Late: CNS changes.
5TH DISEASE
Highest in children between 5-14. Respiratory and blood transmission. 6-21d incubation.

Flu-like illness for 2-3d. then symptom free for 1-7d. Then fiery rash on face "slapped face" look. Circumoral pallor. Then lacelike rash on trunk. Proximal to distal but not on palms or soles. Caused by parvovirus.