Peds 4

Non-Hodgkins

4 types: Burkitt's lymphoblastic large B cell anaplastic large cell

hypoplastic left heart syndrome

95% mortality rate if not treated 58% survival if surgery at 5yo

B Rheumatic fever

A 15-year-old patient will be undergoing a mitral valve replacement for severe mitral stenosis. When reviewing the patient's chart, which of these infectious diseases would likely be present in this patient's history? Please choose from one of the following options. A Meningitis B Rheumatic fever C Varicella (chicken pox) D Haemophilus influenzae

B Exertional dyspnea

A 18-year-old patient with a congenital atrial septal defect has lived with the defect without significant problems until now. Which of the following is an indication that the patient is becoming hemodynamically symptomatic? Please choose from one of the following options. A Bronchoconstriction and wheezing B Exertional dyspnea C Intermittent claudication D Blood pressure 140/90

A Potassium 3.2mEq/L3.2

A 5-year-old child with a congenital heart defect is to receive digoxin PO. Prior to administration, the healthcare provider checks the most recent laboratory report. Which laboratory value should be of most concern to the healthcare provider? Please choose from one of the following options. A Potassium 3.2mEq/L3.2 B Digoxin level 0.8 ng/mL - 0.8 C Hemoglobin 10g/dL10 D Creatinine 0.4mg/dL0.4

A Carbon dioxide

A client with anemia may be tired due to a tissue deficiency of which of the following substances? A Carbon dioxide B Factor VIII C Oxygen D T-cell antibodies

C Fluid overload

A clinic nurse instructs the mother of a child with sickle cell disease about the precipitating factors related to pain crisis. Which of the following, if identified by the mother as a precipitating factor, indicates the need for further instructions? A Infection B Trauma C Fluid overload D Stress

D Hemophilia A results from a deficiency of factor VIII

A pediatric nurse health educator provides a teaching session to the nursing staff regarding hemophilia. Which of the following information regarding this disorder would the nurse plan to include in the discussion? A Hemophilia is a Y linked hereditary disorder B Males inherit hemophilia from their fathers C Females inherit hemophilia from their mothers D Hemophilia A results from a deficiency of factor VIII

C Drinks coffee or tea with meals

A vegetarian client was referred to a dietitian for nutritional counseling for anemia. Which client outcome indicates that the client does not understand nutritional counseling? The client: A Adds dried fruit to cereal and baked goods B Cooks tomato-based foods in iron pots C Drinks coffee or tea with meals D Adds vitamin C to all meals

A Failure to thrive

An infant is being prepared for surgical repair of a ventricular septal defect (VSD). Which of the following problems will be prevented by closing the defect? Please choose from one of the following options. A Failure to thrive B Ventricular dysrhythmias C Heart block D Respiratory alkalosis

A Pallor and fatigue C Bronzing of the skin E Pathologic bone fractures

An infant with a diagnosis of beta-thalassemia major is admitted to the pediatric unit because of failure to thrive. For which of the following clinical manifestations of this disease should the healthcare provider assess? Select all that apply. A Pallor and fatigue B Bleeding tendencies C Bronzing of the skin D Leukopenia E Pathologic bone fractures

D Tachycardia

During an assessment of a patient experiencing acute hemorrhage and anemia, the healthcare provider would most likely expect to find: Please choose from one of the following options. A Hypotension B Jaundice C Nausea D Tachycardia

unknown

ITP cause?

Joints carditis nodules erythema marginatum sydenham's chorea

Jones criteria for rheumatic fever?

D Congestive heart failure and hypoxemia

The healthcare provider is caring for a child with congenital heart disease. When planning care, monitoring for which of the following complications will be included in the plan of care? Please choose from one of the following options. A Bradycardia and hepatomegaly B Pulmonary hypotension and cyanosis C Increased pulmonary compliance and cyanosis D Congestive heart failure and hypoxemia

D Upright in an infant seat

The healthcare provider is caring for an infant with a diagnosis of a congenital heart defect. The baby's pulse is 158 and the respiratory rate is 74. Which of the following is the best position for the baby to be placed? Please choose from one of the following options. A Side-lying with a blanket roll at the back B Supine with the legs slightly elevated C Prone position with the head elevated D Upright in an infant seat

B "Your baby needs this drug because it interferes with substances that keep the PDA open."

The healthcare provider is preparing to administer indomethacin to an infant with a persistent patent ductus arteriosus (PDA). The mother of the baby asks why the medication is being given to her baby. What is the healthcare provider's best response? Please choose from one of the following options. A "Your baby needs help clearing the extra fluid from the lungs." B "Your baby needs this drug because it interferes with substances that keep the PDA open." C "This drug will help your baby's heart contract with a stronger force." D "This drug is a non-steroidal anti-inflammatory drug, so it will help control your baby's pain." What other medication is sometimes used?

D "The newborn has a high concentration of fetal hemoglobin in the blood for some time after birth."

The mothers asks the nurse why her child's hemoglobin was normal at birth but now the child has S hemoglobin. Which of the following responses by the nurse is most appropriate? A "The placenta bars passage of the hemoglobin S from the mother to the fetus." B "The red bone marrow does not begin to produce hemoglobin S until right after birth." C "Antibodies transmitted from you to the fetus provide the newborn with temporary immunity." D "The newborn has a high concentration of fetal hemoglobin in the blood for some time after birth."

C Obstruction to circulation

The nurse explains to the parents of a 1-year-old child admitted to the hospital in a sickle cell crisis that the local tissue damage the child has on admission is caused by which of the following? A Autoimmune reaction complicated by hypoxia B Lack of oxygen in the red blood cells C Obstruction to circulation D Elevated serum bilirubin concentration.

A "Tell me about your feelings right now."

What is the most appropriate nursing response to a terminal client who is fearful of dying? A "Tell me about your feelings right now." B "When the doctor arrives, everything will be fine." C "This is a bad situation, but you'll feel better soon." D "Please be assured we're doing everything we can to make you feel better."

C Potassium

What supplemental medication is most frequently ordered in conjuction with furosemide (Lasix)? A Chloride B Digoxin C Potassium D Sodium

C Eccymosis formation around a joint

Which of the following assessments in a child with hemophilia would lead the nurse to suspect early hemarthrosis? A Child's reluctance to move a body part B Cool, pale, clammy extremity C Eccymosis formation around a joint D Instability of a long bone in passive movement

D Inotropic agents

Which of the following classes of medications maximizes cardiac performance in clients with heat failure by increasing ventricular contractility? A Beta-adrenergic blockers B Calcium channel blockers C Diuretics D Inotropic agents

C Hemophilia A

Which of the following disorders results from a deficiency of factor VIII? A Sickle cell disease B Christmas disease C Hemophilia A D Hemophilia B

A Apical pulse 90-110

Which of the following parameters should be checked before administering digoxin? A Apical pulse B Blood pressure C Radial pulse D Respiratory rate What rate?

A None

Which of the following symptoms is expected with hemoglobin of 10 g/dl? A None B Pallor C Palpitations D Shortness of breath

A Crackles

Which of the following symptoms is most commonly associated with left-sided heart failure? A Crackles B Arrhythmias C Hepatic engorgement D Hypotension

C Pain related to tissue anoxia

Which of the following would the nurse identify as the priority nursing diagnosis during a toddler's vaso-occlusive sickle cell crisis? A Ineffective coping related to the presence of a life-threatening disease B Decreased cardiac output related to abnormal hemoglobin formation C Pain related to tissue anoxia D Excess fluid volume related to infection

B Heart failure

With which of the following disorders is jugular vein distention most prominent? A Abdominal aortic aneurysm B Heart failure C Myocardial infarction D Pneumothorax

iron

administration: give exact amount - more is not better liquid may stain teeth-use straw, dropper or syringe avoid fluoride, antacids, coffee, milk, tea, dairy, bread within 1h - interferes w absorption

pedal pulses (mark it) pulse ox

baseline info needed for heart cath?

transposition of the great arteries

blue baby that does NOT respond to O2?

osteogenic sarcoma

bone tumor occurring in the growth metaphysis or end of long bones trauma brings malignancy to attention of HCP malignant primitive bone-forming cells that spreads to other areas and bones

sickle cell pain

can last several hours to several weeks crisis: deep pain, lumbosacral, bone marrow ischemia, spine, knee, shoulder, elbow, femur hand-foot syndrome acute chest syndrome priaprism

hemosiderosis

can occur/result from hemorrhage within an organ iron liberated from extravascular RBCs is deposited within that organ

soft tissue sarcoma/rhabdomyosarcoma

cause unknown metastasis common (lungs, bone, bone marrow)

thalassemia

cause: autosomal recessive disorder

epitaxis

causes include: trauma injury nose picking URI allergic rhinitis

bacterial endocarditis

causes include: strep staph

pulmonary edema pulmonary fibrosis ARDS

chronic heart failure with rales is indicative of?

retinoblastoma

clinical manifestations: absence of red reflex strabismus blindness painful eyes

Neuroblastoma

clinical manifestations: depends on location and extent most common primary site is abdomen w involvement of adrenal gland or paraspinal ganglion palpable abdominal mass that crosses midline abdominal pain, fullness edema in lower extremities

soft tissue sarcoma/rhabdomyosarcoma

clinical manifestations: depends on site orbitis nasopharyngeal extremities retroperitoneal sinuses middle ear peritoneum most common symptom is a hard, tender mass

ALL

clinical manifestations: fever bone pain pallor anemia fatigue bruising bleeding

AML

clinical manifestations: fever bone pain pallor anemia fatigue bruising bleeding as well as - at risk for DIC, gingival hypertrophy, and chloromas

Non-Hodgkins

clinical manifestations: mediastinal mass pleural effusion lymphadenopathy abdominal pain CNS symptoms petechiae bruising bleeding

Ewing's sarcoma

clinical manifestations: pain and swelling systemic sx: weight loss fever increased ESR

osteogenic sarcoma

clinical manifestations: pain at site of tumor swelling, mass limp

Hodgkins

clinical manifestations: prolonged symptoms painless, enlarged lymph nodes (cervical, supraclavicular) malaise anorexia night sweats fever

n/v low grade fever loss of pulse in cathed extremity transient dysrhythmias acute hemorrhage

complications of heart cath?

Kawasaki disease

complications: coronary artery dilation or aneurysm formation - most common in subacute phase, ECG, anticoagulation meds

ALL AML

diagnose by: bone marrow aspirate CBC lumbar puncture

retinoblastoma

diagnosis: CT MRI BMA spinal tap

Ewing's sarcoma

diagnosis: biopsy of bone lesion CT MRI PET

Hodgkins

diagnosis: biopsy of node bone marrow biopsy labs (CBS, liver function, ESR, ferritin, copper) xray CT

neuroblastoma

diagnosis: biopsy of tissue or mass BMA xray CT MRI labs (CBC, LFT, CMP, ferritin, LDH) urine for VMA and HVA

Non-Hodgkins

diagnosis: bone marrow biopsy biopsy of mass or node lumbar puncture CT/PET labs (CBC, LFT, CMP, uric acid, UA)

osteogenic sarcoma

diagnosis: cray CT MRI PET labs (CBC, CMP, LDH, alkaline phosphatase) biopsy

soft tissue sarcoma/rhabdomyosarcoma

diagnosis: tumor biopsy CT MRI PET bone marrow biopsy lumbar puncture labs (CBC, UA, CMP, LFT)

epitaxis

education includes: staying calm do not lay down lean forward & apply pressure for 10+ minutes pet.jelly to prevent crusting evaluate if 30+ min of bleeding

hemosiderosis

excessive accumulation of iron deposits in the tissues

prophylactic antibiotics

future precautions for rheumatic fever?

indomethacin

given IV used to treat PDA in the neonate and premature infant to remain patency

Ewing's sarcoma

highly malignant arises from primitive undifferentiated cells present in any bone often occur in older children and young adults (rare before 5yo)

HSCT

indicated for AML during 1st remission and ALL after 2nd remission indicated for non-hodgkins lymphoma & certain solid tumors

Kawasaki disease

interventions include: monitor vitals & cardiac status I&O daily weights assess for heart failure IV fluids to prevent dehydration clear liquids, soft foods IV gamma globulin aspirin as needed comfort measures (oral hygiene, cool cloths, lotion, lip balm, calm environment, cluster care)

electrolytes (potassium, magnesium, calcium)

labs to follow for digoxin?

radiation

local ionization directly to the tumor minimal effects to surrounding tissue methods include: external beam, implanted seeds, 3D conformal, intraoperative, proton beam

rheumatic fever

major s/sx include: carditis polyarthritis sydenham's chorea erythema marginatum subcutaneous nodules

Hodgkins

malignancy in lymphoid system that originates in cervical nodes and spreads to other nodes peak incidence is 25-29 in developed nations, adolescents in underdeveloped

<11

mild anemia

rheumatic fever

minor s/sx include: arthralgia increased ESR increased CRP prolonged PR on ECG

<7

moderate anemia

AML

more common in hispanics and african american populations than caucasians?

sequestration of sickle cell

mortality rate 50% excessive pooling of blood in the liver and spleen fatal b/c spleen can hold 1/5 of body's blood supply repeated calls for spleen removal teach parents to palpate spleen

ALL

most common cancer in children?

Neuroblastoma

most common extracranial solid tumor in children usually seen in early childhood present in tissue of brain, adrenal medulla, pelvis, mediastinum, & paraspinal ganglion 40% diagnosed under 1yo 95% less than 10yo

Non-Hodgkins

no single focal origin (rarely located) rapid onset widespread onset more common in children less than 10yo very aggressive survival rate 90%

11-13

normal hemoglobin levels?

tetralogy of fallot

nursing actions include: knee-chest positioning to shunt blood to pulmonary artery faster supplement O2 surgery at 3-12mo

thalassemia

nursing care: blood transfusions observe for reactions, follow protocol support services, emotional support refer to appropriate agencies

decreased cardiac output

nursing plan includes: I&O limit fluids and sodium monitor fluid intake note chest pain auscultate heart sounds cardiac monitoring check labs review EKG CXR admin O2 PRN fowlers/high-fowlers positioning monitor BP, pulse, & cond. before giving meds monitor bowel function

AML

poorer prognosis: ALL or AML?

temp/color/pulse of extremity vitals Q15min I&O observe for bleeding O2 compared to baseline leg straight in bed for 4-8h advance diet as tolerated pressure dressing removed in 24h no sand bags

postop info after heart cath?

prior ECG, ECHO, CBC, platelets NPO height weight allergies

preop info before heart cath?

good hygiene dental health avoid tattoos, piercings contact MD if open sores or cuts do not heal preventative antibiotics

prevention of bacterial endocarditis?

ITP

prognosis: 75-80% recover in 6mo some develop chronic adolescents more likely to develop chronic/persistent type

right

s/sx include: fatigue ascites increased venous pressure enlarged liver & spleen distended jugular veins anorexia GI distress weight gain dependent edema possibly secondary to chronic pulmonary problems right or left sided HF?

pulmonary stenosis

s/sx include: mild/moderate can be asymptomatic severe: dyspnea with exertion, fatigue, cyanosis murmur SOB

anemia

s/sx include: pallor tachycardia headache fatigue SOB muscle weakness irritability lack of interest in play anorexia murmurs low grade fever yellowish skin grayish look hair falls out brittle nails spoon shaped nails spleen/liver enlargement cool skin decreased peripheral pulses *can be normal weight, overweight, underweight* if unresolved can lead to physical & developmental delays

left

s/sx include: restlessness confusion orthopnea tachycardia exertional dyspnea fatigue cyanosis paroxysmal nocturnal dyspnea pulmonary congestion: cough, crackles, tachypnea, blood tinged sputum left or right sided HF?

tetralogy of fallot

s/sx include: systolic murmur profoundly cyanotic or not result of decrease in pulmonary blood flow tet spells cyanosis at birth, progressive cyanosis

sickle cell

s/sx include: asymptomatic in first 6mo pallor irritability fatigue SOB jaundice *pain*in any organ or joint

digoxin

s/sx toxicity include: loss of appetite n/v diarrhea abd pain visual changes decreased heart rate

<3

severe anemia

radiation

side effects: depends on site anemia thrombocytopenia neutropenia oral mucosa esophagitis skin reaction pneumonitis headache n/v somnolence syndrome (sleep 20h a day) growth delay

chemo

side effects: hematopoetic GI hepatic renal integument reproductive

sickle cell

teaching on triggers includes: hypoxemia increased hydrogen ions increased plasma osmolarity decreased plasma volume decreased temp.

iron

teaching: keep locked away to prevent overdose avoid side effects by taking with or after meals stools are tarry, green color constipation may ocur

thalassemia

transfusions: blood transfusions q 2-3 weeks stem cell transplant cord blood transplant

bedrest antibiotics prophylactic antibiotics increased does aspirin/antacids teaching

treatment for rheumatic fever?

anemia

treatment includes: treat the cause caregiver education breastfeeding until 4-6 mo them 1mg/kg/day iron by 6mo, 1 feeding per day vitamin C rich fruit/day after 6mo, pureed meats child 1-5yo no more than 24oz cow, goat, or soy milk per day consumption of iron rich foods cook acidic foods in iron pan give exact amount of iron

cooley's anemia

treatment/management: no cure-will die if not tx by 5-6yo normalize hct & hgb to relieve anemia can add 2 decades to life blood transfusions q2-3 weeks (can lead to iron overload) splenectomy eliminates the site for hemolysis so it decreases the need for transfusions chelating agents for reducing/reversing effects of iron overload desferal or exjade stem cell transplant/cord blood transplant

hemosiderosis

treatment: O2 supplementation blood transfusion respiratory therapy (excessive secretions) vent for resp. failure

Non-Hodgkins

treatment: aggressive multi-agent chemo risk for tumor lysis syndrome HSCT radiation not indicated

Hodgkins

treatment: chemo radiation steroids preservation of fertility

AML

treatment: chemo IV chemo meds methotrexate HSCT

retinoblastoma

treatment: depends on unilateral/bilateral, quality of vision, metastasis surgery radiation laser cryotherapy chemo HSCT radiation

hypoplastic left heart syndrome

treatment: formerly inoperable, comfort measures until death when PDA closed now 3 options: no tx, heart transplant, or palliative surgery-O2-prostaglandins

Hemophilia B

treatment: plasma desmopressin acetate will not work

ITP

treatment: supportive care self limiting restrict activity prednisone IV immune globulin anti-D antibody splenectomy if persists 1+ years only acetaminophen for pain

neuroblastoma

treatment: surgery chemo radiation biotherapy HSCT

Ewing's sarcoma

treatment: surgery radiation chemo

osteogenic sarcoma

treatment: surgery (limb sparing) chemo radiation

soft tissue sarcoma/rhabdomyosarcoma

treatment: surgical resection radiation chemo

ALL

treatment: chemo prednisone tumor lysis syndrome hematopoetic stem cell transplant 80% survival rate

Hemophilia A

treatment: recombinant factor amount depends on degree may give prophylactically 3-4x a week give immediately after injury desmopressin acetate prior to dental/surgical procedures

ALL

trisomy 21 (down syndrome) children are at an increased risk for this type of cancer?

retinoblastoma

tumor of the eye found in infants & young children genetic component can metastasize to brain, lymph nodes, bone, organs 96% 5 year survival rate

A B C

types of hemophilia?

ALL & AML

types of leukemias?

Hodgkins & Non-Hodgkins

types of lymphomas?

osteogenic ewings soft tissue rhabdomyosarcoma

types of sarcomas?

prostaglandins

utilized to maintain the patency of the ductus arteriosus until surgical ligation

increased BP in arms weak/absent femoral pulses

vital signs differences for coarctation of the aorta?

Hemophilia A (beta thalassemia)

what is Cooley's anemia?

depends on size of hole school-age before surgical repair

when can we repair an ASD septal defect?

2-12 mo

when can we repair an VSD septal defect?