Exam 1 Review

Question 1

Sickle cell crisis prophylaxis

Answer 1

PCN - from 2 mo. to 2 years - 125 BID; 2 - 5 years - 250 BID (erythromycin if allergic)

Vaccines: PCV 13 on strict, regular schedule - 2, 4, 6, & 12 months; add PCV 23 at 2 years and the second 1-3 years later (3-5yo). If the first dose of PCV 23 is delayed until after 10 yo, they should be given 5 years apart

Goal: no invasive pneumococcal process.

Question 2

2-4 years old. Hx of sickle cell. (Pleuritic chest pain), fever, wheezing, dry cough, rales on auscultation, and pulmonary infiltrates on chest X-ray.

Answer 2

Acute Chest Syndrome

Question 3

Acute Chest Syndrome prevention and management

Answer 3

P: after their third pain crisis of any severity in 12 months, offer hydroxyurea therapy to children and adolescents starting as early as 9 months of age
M: emergent transportation to ED on fluids

Question 4

Implications for asplenic patients

Answer 4

Greater risk for many infections, mostly S. pneumoniae
Of great importance for caregivers to know/learn the early signs of infection (fever > 100.4, fatigue, malaise), and to report them immediately

Question 5

CF colonization: significant culprits

Answer 5

P. aeurginosa - most common
S. aureus - also common
B. sepacia - more severe, greater respiratory involvement

Question 6

Role of the DNP in CF therapy is focused on -1- and -2-

Answer 6

1. Nutrition & growth

2. medication regimen maintenance

Question 7

Rationale for the following in CF patients:

1. Pancrease
2. Lipase
3. A, D, E, & K
4. dornase alfa

Answer 7

1 - 2. Pancreatic insufficiency is a hallmark of CF, meaning they underproduce these enzymes, so replacement is necessary to mitigate malabsorption of essential fatty acids

3. Malabsorption of fats also means malabsorption of fat-soluble vitamins like these, so replacement is necessary for adequate nutrition
4. Nebulized recombinant human rhDNase used to thin the mucus alongside airway clearance therapy (ACT) to decrease obstruction

Question 8

Cl- sweat test result interpretation

Answer 8

30-60: borderline; needs referral to CF clinic for further diagnostics
>60: diagnostic for CF

Question 9

Newborn not screened for CF, CF can be identified through…

Answer 9

…meconium ileus and frequent infections

Question 10

Postnatally, -2- or -1- is present in 11.9% of infants younger than 1 year with -3-; (2) it results from thick gastrointestinal secretions that become adherent to the intestinal mucosa, leading to bowel obstruction.-1- is often accompanied by abdominal distention and dilated loops of bowel on imaging, and a reported 30% of cases of -1- are complicated by intestinal perforation and peritonitis. Sequale include FTT and poor growth.

Answer 10

1. Meconium ileus
2. delayed meconium passage
3. CF

Question 11

Frequent infections associated with CF ages birth to 10 years include -1-
Age 10-20 years also includes -1- with intermittent -2- infections as well

Answer 11

1. S. aureus

2. P. aeruginosa

Question 12

Liver issues in CF patients aged birth to 10 years includes -1-
From 10 to 20 years, -2- is the chief hepatic concern
Beyond 20 years of age, -3- can be identified as a common hepatic pathology

Answer 12

1. abnormal liver function tests
2. cirrhosis
3. portal HTN

Question 13

• Weight gain in healthy child increases slowly over days/weeks
• Parent observation “clothes don’t fit”
• Pallor, fatigue
• Puffiness of face/eyes – Subsides during day
• Decreased UOP
• Urine frothy or foamy
• Generalized Edema develops ** this is what brings them in
UA: proteinuria
CMP: hypoalbuminemia

Answer 13

Indicative of Nephrotic Sx

Question 14

Nephrotic syndrome exacerbation/acute phase management

Answer 14

Salt restriction and steroids (responsive to steroids –> good prognosis, will be well managed going forward; resistant –> renal biopsy –> more intensive therapy/nephrology referral)

While on therapy UA will be unclear/unreliable, continue until proteinuria is gone and/or blood albumin levels return to healthy range

Question 15

Periorbital edema ( worse in morning)
Loss of appetite
↓ UOP
Dark colored urine ( coke cola, or tea)
Antecedent -1- infection

Answer 15

Post-strep glomerulonephritis (PSGN)

1. streptococcal

Question 16

• Clinical findings – diagnostic studies
– UA with microscopic: red blood cell casts; mild proteinuria, hematuria
– Serum C3 or C4 (low); total protein, albumin (mild hypoalbuminemia)
– CBC (mild to moderate anemia), ESR, ASO (elevated)
– Serum electrolytes (normal to high), BUN/creatinine (elevated in acute phase only), cholesterol (normal to high)

Answer 16

Findings suggestive of PSGN, especially HEMATURIA (as in all nephritic pathologies)

• streptozyme test, DNA titer may also be positive for streptococcal infectious agent, but unlikely as PSGN generally develops after the infection has resolved
• if tests/cultures are positive, then treat with antibiotics, but do not administer antibiotics without a known pathogen

Question 17

Type 1 renal tubular acidosis involves failure of the -1- tubules to -2- causing -3- as well as acidosis
Type 2 RTA involves failure of the -4- tubules to -5- also causing -3- as well as acidosis

Answer 17

1. distal
2. excrete hydrogen
3. hypokalemia & hyperchloremia
4. proximal
5. reabsorb HCO3-

Question 18

The standard treatment for both major types of RTA is…

Answer 18

…potassium citrate

Question 19

Polyuria
Polydipsia
Headaches
Malaise
Poor growth evident after 12 months
1. Urine pH > 5.5
2. Urine pH = 5.5

Answer 19

RTA

1. Type 1
2. Type 2

Question 20

X-linked oxidant retention –> hemolytic disease

Causes jaundice, tea-colored urine, back/flank pain, and anemic syndromes

Answer 20

G6PD deficiency

Question 21

Triggers of G6PD deficiency-related hemolytic episodes

Answer 21

Food: soy, fava, red wine
Drugs: antimalarials, sulfa drugs, ibuprofen & aspirin

Question 22

G6PD deficiency treatment (primary, secondary, tertiary)

Answer 22

Newborn screening
Trigger avoidance
Blood transfusion if hemolytic episode is severe

Question 23

Albuterol treatment frequency for an active exacerbation

Answer 23

Rescue inhaler every 20 minutes x 3. Then ER/ED

Question 24

Three parts of asthma pathology

Answer 24

Constriciton
Inflammation
Congestion/occlusion

Question 25

Steps of asthma therapy

Answer 25

Intermittent: SABA
Mild persistent: ICS w/ SABA rescue
Moderate persistant: increase ICS dose, consider adding LABA

Need to increase therapy, but inhalants are not enough: PO leukotriene inhibitor (montelukast)

Failure of above - refer to pulmonology

Question 26

In a 0-4 year old:
Sudden shortness of breath
Cyanosis
Systolic ejection click heard in the pulmonic area

Answer 26

Tetralogy of Fallot (Tet)

Question 27

Defects present in Tet

Answer 27

1. Impaired pulmonary artery outflow (IPAO) –> 2. R ventricular hypertrophy
2. Ventricular Septal Defect (VSD) + IPAO –> 4. Aortic override

Question 28

Major Consideration for Functional Asplenia in infants and toddlers [Congenital Heart Defect (CHD), not SCD]

Answer 28

Prophylactic antibiotics: Daily PCN from 2 mo to 2 years; PCN/Amox from 2-5 years
Allergy: erythro, clinda, Bactrim instead

Question 29

The most common CHD
Poor feeding/poor weight gain in infants
Diaphoresis
Whole-systolic murmur

Answer 29

VSD
Size of septal defect determines volume of mumur/bruit/thrill
May remain after VSD has been repaired

Question 30

Diminished or absent pulses in the lower extremities

Difference of 25+ between brachial and femoral SBP

Answer 30

Coarctation of the Aorta

Question 31

Characterized by mixed-oxygen blood

Commonly found alongside atrioventricular septal defect in T21

Answer 31

Congenital heart failure

Question 32

Similar symptoms to VSD, but milder
Physiologic in utero, typically resolves within the first two days of life
Considered pathologic if not resolved by week 3

Answer 32

Patent Ductus Arteriosis (PDA)

Question 33

Systolic ejection murmur
Widely split S2
Exercise intolerance in older children

Answer 33

Atrial septal defect (ASD)

Question 34

Jaundice
Enlarged skull bones
Hepatosplenomegaly
Poor growth
Hemochromatosis

Answer 34

Beta thalassemia Major
or
Alpha thalassemia (mod/sev)

Question 35

Treatment of thalassemias

Answer 35

Transfusions
Chelation therapy to reduce iron retention from transfusions
Splenectomy if hemochromatosis presents in spleen

Question 36

Bruises/bleeds easily
Decreased factor 8 or 9
1. prolonged PTT
2. PTT unaffected
Patient should not -3-

Answer 36

1. Hemophilia A
2. Von Willebrand’s disease
3. Play contact sports

Question 37

Patient under 13 with three BP readings which average to a value

1. > 95th percentile for their age/BMI
2. > 90th percentile for their age/BMI
3. > 95th percentile + 12 mmHg for their age/BMI

Answer 37

1. Stage 1 Hypertension
2. “Elevated Blood Pressure” previously “prehypertension)
3. Stage 2 Hyptertension

Question 38

HTN vs PHTN

Answer 38

Activity intolerance vs pronation intolerance
L-sided vs R-sided heart failure
Obesity is a risk factor for each, but for metabolic disease vs respiratory restriction respectively

Question 39

A. Restricted caloric intake relative to energy requirements, leading to significantly low body weight for age, sex, projected growth, and physical health

B. Intense fear of gaining weight or behaviors that consistently interfere with weight gain, despite being at a significantly low weight

C. Altered perception of one’s body weight or shape, excessive influence of body weight or shape on self-value, or persistent lack of acknowledgment of the seriousness of one’s low body weight

Subtypes: -1- type (weight loss is achieved primarily through dieting, fasting, and/or excessive exercise. In the previous 3 mo, there have been no repeated episodes of excessive eating followed by self-induced vomiting or misuse of laxatives, diuretics, or enemas, etc.); -2- type (in the previous 3 mo, there have been repeated episodes of excessive eating followed by self-induced vomiting or misuse of laxatives, diuretics, or enemas, etc.)

-3-: all of the above criteria are met yet the individual’s weight is within or above the normal range despite
significant weight loss

-4-: recurrent self-induced vomiting; misuse of laxatives, diuretics, or other medications, etc., in the absence of excessive eating with the intent to influence weight or body shape

Answer 39

Anorexia Nervosa (AN)

1. restricting
2. binge-eating/purging
3. Atypical AN
4. Purging disorder

Question 40

Repeated episodes of both of the following: within a distinct period of time (e.g., 2 h), eating an amount of food that is clearly larger than what most individuals would eat during a similar period of time under similar circumstances AND a sense that one cannot limit or control their overeating during the episode

Repeated use of inappropriate compensatory behaviors for the prevention of weight gain, such as self-induced vomiting; misuse of laxatives, diuretics, or other medications; fasting; or excessive exercise

On average, the episodes and compensatory behaviors both occur at least once a week for 3 mo

Self-value is overly influenced by body shape and weight

The episodes and compensatory behaviors do not occur exclusively during periods of caloric restriction or excessive exercise

-1-: All of the above criteria are met, but, on average, the episodes and compensatory behaviors occur less than once a week and/or for <3 mo

Answer 40

Bulemia Nervosa (BN)
1. BN (of low frequency and/or limited duration)

Question 41

Recurrent episodes of both of the following: within
a distinct period of time (eg, 2 h), eating an amount of food that is clearly larger than what most individuals would eat during a similar period of time under similar circumstances AND a sense that one cannot limit or control their overeating during the episode

The episodes include 3 or more of the following: eating much more quickly than normal, eating until
uncomfortably full, eating large amounts of food when not feeling hungry, eating alone because of embarrassment at how much one is eating, and feeling guilty, disgusted, or depressed afterward

Marked anguish is experienced regarding these episodes

On average, the episodes occur at least once a week for 3 mo

The episodes are not associated with the use of inappropriate compensatory behavior and does not occur only in the context of caloric restriction or excessive exercise

-1-: All of the above criteria are met, but, on average, the episodes occur less than once a week and/or for <3 mo

Answer 41

Binge Eating Disorder (BED)

1. BED (of low frequency and/or limited duration)

Question 42

A disrupted eating pattern (eg, seeming lack of interest in eating or food; restraint based on the sensory qualities of food; concern about unpleasant consequences of eating) as evidenced by persistent failure to meet appropriate nutritional and/or energy needs associated with 1 (or more) of the following: significant weight loss or, in children, failure to achieve expected growth and/or weight gain, marked nutritional deficiency, reliance on enteral feeding
or oral nutritional supplements, significant interference with psychosocial functioning

The disturbance cannot be better explained by lack of available food or by an associated culturally sanctioned practice

The eating disturbance cannot be attributed to a coexisting medical condition nor better explained by another mental disorder. If the eating disturbance occurs in the context of another condition or disorder, the severity of the eating disturbance exceeds that routinely associated with the condition or disorder

Answer 42

Avoidant/Restrictive Food Intake Disorder (ARFID)

Question 43

A patient with a diagnosis of major depression who has attempted suicide says to the nurse, “I should have died! I’ve always been a failure. Nothing ever goes right for me.” Which response demonstrates therapeutic communication?

A. “You have everything to live for.”
B. “Why do you see yourself as a failure?”
C. “Feeling like this is all part of being depressed.”
D. “You’ve been feeling like a failure for a while?”

Answer 43

Correct Answer: D. “You’ve been feeling like a failure for a while?”

Responding to the feelings expressed by a patient is an effective therapeutic communication technique. The correct option is an example of the use of restating. It’s frequently useful for nurses to summarize what patients have said after the fact. This demonstrates to patients that the nurse was listening and allows the nurse to document conversations. Ending a summary with a phrase like “Does that sound correct?” gives patients explicit permission to make corrections if they’re necessary.

Option A: Some people confuse empathizing with sympathizing. To establish a good nurse-patient relationship, the nurse should use empathy, not sympathy. Sympathy is defined as the feelings of concern or compassion one shows for another. By sympathizing, the nurse projects his or her own concerns to the client, thus, inhibiting the client’s expression of feelings.
Option B: This option blocks communication because it minimizes the patient’s experience and does not facilitate exploration of the patient’s expressed feelings. In addition, the use of the word “why” is nontherapeutic.
Option C: Internal validation is a non-therapeutic communication technique. This refers to making an assumption about the meaning of someone else’s behavior that is not validated by the other person (jumping into conclusion).

Question 44

When the community health nurse visits a patient at home, the patient states, “I haven’t slept the last couple of nights.” Which response by the nurse illustrates a therapeutic communication response to this patient?

A. “I see.”
B. “Really?”
C. “You’re having difficulty sleeping?”
D. “Sometimes, I have trouble sleeping too.”

Answer 44

Correct Answer: C. “You’re having difficulty sleeping?”

The correct option uses the therapeutic communication technique of restatement. Although restatement is a technique that has a prompting component to it, it repeats the patient’s major theme, which assists the nurse in obtaining a more specific perception of the problem from the patient.

Option A: An essential factor to build a therapeutic nurse-client relationship is showing genuine interest to the client. For the nurse to do this, he or she should be open, honest, and display congruent behavior. Congruence only occurs when the nurse’s words match with her actions.
Option B: Stay away from nontherapeutic habits such as asking irrelevant personal questions, stating personal opinions, or showing disapproval. Ask open-ended questions, such as, “Tell me about your difficulties,” to encourage the patient to take the lead in the discussion, and prompt him by suggesting he tell you more.
Option D: This option is not a therapeutic response since it does not encourage the patient to expand on the problem. Offering personal experiences moves the focus away from the patient and onto the nurse.

Question 45

A patient experiencing disturbed thought processes believes that his food is has been poisoned. Which communication technique should the nurse use to encourage the patient to eat?

A. Using open-ended questions and silence
B. Sharing personal preference regarding food choices
C. Documenting reasons why the patient does not want to eat
D. Offering opinions about the necessity of adequate nutrition

Answer 45

Correct Answer: A. Using open-ended questions and silence.

Open-ended questions and silence are strategies used to encourage patients to discuss their problems. Sharing personal food preferences is not a patient-centered intervention. One of the most important skills of a nurse is developing the ability to establish a therapeutic relationship with clients. For interventions to be successful with clients in a psychiatric facility and in all nursing specialties it is crucial to build a therapeutic relationship.

Option B: Focusing on one’s self is a non-therapeutic communication technique. This refers to responding in a way that focuses attention on the nurse instead of the client. An essential factor to build a therapeutic nurse-client relationship is showing genuine interest to the client. For the nurse to do this, he or she should be open, honest, and display congruent behavior. Congruence only occurs when the nurse’s words match with her actions.
Option C: Focusing on the negative should be done less than giving options for the patient. Encourage the patient to consider the pros and cons of possible options. In dealing with clients their interest should be the nurse’s greatest concern. Thus, empathizing with them is the best technique as it acknowledges the feelings of the client and at the same time, it allows a client to talk and express his or her emotions.
Option D: The remaining option is not helpful to the patient because they do not encourage the patient to express feelings. The nurse should not offer opinions and should encourage the patient to identify the reasons for the behavior.

Question 46

A patient admitted to a mental health unit for treatment of psychotic behavior spends hours at the locked exit door shouting. “Let me out. There’s nothing wrong with me. I don’t belong here.” What defense mechanism is the patient implementing?

A. Denial
B. Projection
C. Regression
D. Rationalization

Answer 46

Correct Answer: A. Denial.

Denial is a refusal to admit to a painful reality, which was treated as if it does not exist. It involves blocking external events from awareness. If some situation is just too much to handle, the person refuses to experience it. This is a primitive and dangerous defense – no one disregards reality and gets away with it for long! It can operate by itself or, more commonly, in combination with other, more subtle mechanisms that support it.

Option B: In projection, a person unconsciously rejects emotionally unacceptable features and attributes them to other persons, objects, or situations. Projection is a psychological defense mechanism proposed by Anna Freud in which an individual attributes unwanted thoughts, feelings, and motives onto another person.
Option C: Regression allows the patient to return to an earlier, more comforting, although less mature, way of behaving. This is a movement back in psychological time when one is faced with stress. Regression functions as a form of retreat, enabling a person to psychologically go back in time to a period when the person felt safer.
Option D: Rationalization is justifying illogical or unreasonable ideas, actions, or feelings by developing acceptable explanations that satisfy the teller and the listener. Rationalization is a defense mechanism proposed by Anna Freud involving a cognitive distortion of “the facts” to make an event or an impulse less threatening.

Question 47

Enlarged aortic diameter + ectopia lentis (dislocated lens)

Enlarged aortic diameter + FBN1 mutation

Enlarged aortic diameter + systemic feature score >7 (wrist and/or hand sign, pectoris carinatum, hindfoot deformity, etc)

OR

Ectopia lentis + FBN1 mutation known to cause aortic
disease

Answer 47

Diagnostic criteria for Marfan Syndrome without a family history.
With a family history, all that’s required is one of the following:
Ectopia lentis
Systemic feature score >7
Aortic diameter with a Z-score of >2 (above 20 years of
age) or >3 (below 20 years of age)

Question 48

A common, clinically and genetically heterogeneous condition characterized by distinctive facial features, short stature, chest deformity, congenital heart disease [Pulmonary Valve Stenosis (PVS), isolated ASDs, and partial atrioventricular canal defects are the most common], and other comorbidities.

Answer 48

Noonan Syndrome