PPS COMPILED SAMPLEX [PART 1 OF 5] - 652 items total with Rationale Flashcards
(150 cards)
1
Q
“Patient with recurrent pneumonia, abscesses, osteomyelitis
a. Chediak Higashi Syndrome
b. Chronic Granulomatous Disease
c. Leucocyte Adhesion Deficiency
d. None of the above”
A
B
“Chediak Higashi - defective granulation of neutrophils; hemophagocytic lymphohistiocytosis
Chronic Granulomatous Disease - unable to kill catalase-positive microorganism; recurrent pneumonia, lymphadenitis, hepatic subcutaneous, or other abscesses, osteomyelitis at multiple sites, FHx of recurrent infections
Leucocyte adhesion Deficiency - delayed separation of umbilical cord, omphalitis
Nelson 21st Ch156 Disorders of phagocyte function pg1133”
2
Q
“Adverse drug reactions in children than in adults
a. Aspirin - Reye syndrome
b. Cefaclor - serum sickness
c. Valproic acid - hepatotoxic in infants
d. All of the above”
A
D
3
Q
“B complement defect can be detected by measuring:
a. lgG
b. lgM
c. lgA
d. Complement”
A
C
“Nelson 20th, ch 122 eval of immunodeficiency pg 999-1000
A simple screening test for B cell defects is the measurement of serum IgA. If the IgA level is normal, selective IgA deficiency, which is the most common B cell defect, is excluded, as are most of the permanent types of hypogammaglobulinemia, as IgA is usually very low or absent in those conditions. Ig IgA is low, IgG and IgM should be measured.
Nelson 21st p1097
One useful test for B-cell function is to determine the presence and titer of isohemagglutinins, or natural antibodies to type A and B red blood cell polysaccharide antigens. This test measures predominantly IgM antibodies. Isohemagglutinins may be absent normally in the first 2 yr of life and are always absent if the patient is blood type AB.
Nelson 21st p1097
One useful test for B-cell function is to determine the presence and titer of isohemagglutinins, or natural antibodies to type A and B red blood cell polysaccharide antigens. This test measures predominantly IgM antibodies. Isohemagglutinins may be absent normally in the first 2 yr of life and are always absent if the patient is blood type AB.”
4
Q
"A patient eats nuts. Developed anaphylactic shock. What will you give? A. Diphenhydramine B. Cetririzine C. Epinephrine IM D. None of the above"
A
C
“Nelson 21st Ch 174 Anaphylaxis pg 1231
Epinephrine is the most important medication and there should be no delay in its administration. Epinephrine should be given by the IM route to the lateral thigh (1:1000 dilution, 0.01 mg/kg; max 0.5mg). For children >12yo, many recommend the 0.5mg IM dose. The IM dose can be repeated at intervals of 5-15 min if symptoms persist or worsen. If there is no response to multiple doses of epinephrine, IV epinephrine using the 1:10,000 dilution may be needed.”
5
Q
“10 yr old with asthma born to a then 16yr old mother, underweight. Grandmother has asthma. What is the predictor for asthma morbidity & mortality
a. Underweight
b. Age of mother
c. Age of patient
d. Family history of asthma”
A
B
“Nelson 21st p1206. Table 169.16. Risk factors for asthma morbidity and mortality
BIOLOGIC
Previous severe asthma exacerbation (intensive care unit admission, intubation for asthma)
Sudden asphyxia episodes (respiratory failure, arrest)
Two or more hospitalizations for asthma in past year
Three or more emergency department visits for asthma in past year
Increasing and large diurnal variation in peak flows
Use of >2 canisters of short-acting β-agonists per month
Poor response to systemic corticosteroid therapy
Male gender
Low birthweight
Nonwhite (especially black) ethnicity
Sensitivity to Alternaria
ENVIRONMENTAL Allergen exposure Environmental tobacco smoke exposure Air pollution exposure Urban environment
ECONOMIC AND PSYCHOSOCIAL Poverty Crowding Mother <20 yr old Mother with less than high school education Inadequate medical care: Inaccessible Unaffordable No regular medical care (only emergency) Lack of written Asthma Action Plan No care sought for chronic asthma symptoms Delay in care of asthma exacerbations Inadequate hospital care for asthma exacerbation Psychopathology in the parent or child Poor perception of asthma symptoms or severity Alcohol or substance abuse"
6
Q
“Discharge criteria for asthma
a. 02 saturation >92%
b. 40% PEF
c. both of the above
d. none of the above”
A
A
“Nelson 21st p1208.
The patient may be discharged home if there is sustained improvement in symptoms, normal physical findings, PEF >70% of predicted or personal best, and oxygen saturation >92% while the patient is breathing room air for 4 hr. Discharge medications include administration of an inhaled β-agonist up to every 3-4 hr plus a 3-7 day course of an OCS. Optimizing controller therapy before discharge is also recommended.”
7
Q
“A patient presented with nasal congestion, sneezing. PE showed edematous boggy and bluish mucus membranes. Associated with postnasal drip, conjunctival suffusion. This could be due to what?
a. Allergic Rhinitis
b. Bronchial asthma
c. Bronchiolitis
d. None of the above “
A
A
“Nelson 21st p1180
Typical complaints include intermittent nasal congestion, itching, sneezing, clear rhinorrhea, and conjunctival irritation…Conjunctival edema, itching, tearing, and hyperemia are frequent findings. A nasal exam performed with a source of light and a speculum may reveal clear nasal secretions; edematous, boggy, and bluish mucus membranes with little or no erythema; and swollen turbinates that may block the nasal airway.”
8
Q
“Asthma therapy stepped down after:
a. 2 months
b. 3 months
c. 4 months
d. 5 months “
A
B
”
Nelson 21st p1200
Asthma therapy can be stepped down after good asthma control has been achieved and maintained for at least 3 mo.”
9
Q
“A patient with bronchial asthma came in respiratory distress. Can talk in phrases. Classification?
a. Mild
b. Moderate
c. Severe
d. Critical”
A
B
“Nelson 21st p1190. Table 169.4”
10
Q
"A patient with asthma attacks during cold season. What will you give? A. Cetirizine B. Montelukast C. SABA D. prednisone"
A
C
“Nelson 21st p1199. Table 169.11”
11
Q
"Treatment for all ages with persistent bronchial asthma A. Daily ICS B. SABA C. Daily ICS + LABA D. Montelukast"
A
A
“Nelson 21st p1199. Table 169.11.
Nelson 21st p1198
The preferred treatment for all patients with persistent asthma is ICS therapy, as monotherapy or in combination with adjunctive therapy. The type(s) and amount(s) of daily controller medications to be used are determined by the asthma severity and control rating. “
12
Q
"lpratropium Br acts on which receptor? A. M1 B. M2 C. M3 D. B2"
A
B
“NCBI Muscarinic receptor antagonists, from folklore to pharmacology; finding drugs that actually work in asthma and COPD
doi: 10.1111/j.1476-5381.2010.01190.x
Blocking M2 receptors with muscarinic antagonists including atropine and ipratropium or using selective M2 receptor antagonists such as gallamine, significantly potentiates vagally induced bronchoconstriction”
13
Q
“True of allergic rhinitis except
A. Associated with at least two fold increase in risk for asthma
B. Risk increases in children introduced to foods or formula early in infancy
C. Increased likelihood in rural and underdeveloped regions
D. All of the above are true”
A
C
“Nelson 21st p1179
Childhood AR is associated with a 3 fold increase in risk for astham at an older age
*no mention of association between AR and introduction to food/formula; no mention of regions where AR is more prevalent”
14
Q
"What is the most common inciting agent of outdoor anaphylaxis in children? A. Insect bites B. Exposure to pollens C. Food allergy D. Intake of medicines"
A
C
“Nelson 21st p1228
The most common causes of anaphylaxis in children are different for hospital and community settings. Anaphylaxis occurring in the hospital results primarily from allergic reactions to medications and latex. Food allergy is the most common cause of anaphylaxis occurring outside the hospital.”
15
Q
"Which of the following signs/ symptoms is a major feature of atopic dermatitis in infants? A. Chronic scaling of the scalp B. Post-auricular fissures C. Hyper-linear palms D. Facial lichenification"
A
A
“Nelson 21st p1210. Table 170.1 Clinical features of atopic dermatitis
MAJOR FEATURES
- Pruritus
- Facial and extensor eczema in infants and children
- Flexural eczema in adolescents
- Chronic or relapsing dermatitis
- Personal or family history of atopic disease
ASSOCIATED FEATURES
- Xerosis
- Cutaneous infections (Staphylococcus aureus, group A streptococcus, herpes simplex, coxsackievirus, vaccinia, molluscum, warts)
- Nonspecific dermatitis of the hands or feet
- Ichthyosis, palmar hyperlinearity, keratosis pilaris
- Nipple eczema
- White dermatographism and delayed blanch response
- Anterior subcapsular cataracts, keratoconus
- Elevated serum IgE levels
- Positive results of immediate-type allergy skin tests
- Early age at onset
- Dennie lines (Dennie-Morgan infraorbital folds)
- Facial erythema or pallor
- Course influenced by environmental and/or emotional factors”
16
Q
"Case of immunodeficiency with no tonsils and lymph nodes that are palpable. Had multiple infections. Males are more affected than females. what is the cause? A. X- linked agammaglobulinemia B. X-linked lymphoproliferative C. Complement deficiency D. Chronic mucocutaneous candidiasis"
A
A
17
Q
“What immunoglobulin is tested for B cell deficiency?
a. lgM
b. lgG
c. lgA
d. lgE”
A
C
”"”Nelson 20th, ch 122 eval of immunodeficiency pg 999-1000
A simple screening test for B cell defects is the measurement of serum IgA. If the IgA level is normal, selective IgA deficiency, which is the most common B cell defect, is excluded, as are most of the permanent types of hypogammaglobulinemia, as IgA is usually very low or absent in those conditions. Ig IgA is low, IgG and IgM should be measured.
Nelson 21st p1097
One useful test for B-cell function is to determine the presence and titer of isohemagglutinins, or natural antibodies to type A and B red blood cell polysaccharide antigens. This test measures predominantly IgM antibodies. Isohemagglutinins may be absent normally in the first 2 yr of life and are always absent if the patient is blood type AB.
Nelson 21st p1097
One useful test for B-cell function is to determine the presence and titer of isohemagglutinins, or natural antibodies to type A and B red blood cell polysaccharide antigens. This test measures predominantly IgM antibodies. Isohemagglutinins may be absent normally in the first 2 yr of life and are always absent if the patient is blood type AB.”””
18
Q
“What is not a major criteria for the diagnosis of atopic dermatitis?
a. Pruritus
b. Family history of allergic diseases
c. Rashes found at the extensor surfaces
d. Xerosis”
A
D
”"”Nelson 21st p1210. Table 170.1 Clinical features of atopic dermatitis
MAJOR FEATURES
- Pruritus
- Facial and extensor eczema in infants and children
- Flexural eczema in adolescents
- Chronic or relapsing dermatitis
- Personal or family history of atopic disease
ASSOCIATED FEATURES
- Xerosis
- Cutaneous infections (Staphylococcus aureus, group A streptococcus, herpes simplex, coxsackievirus, vaccinia, molluscum, warts)
- Nonspecific dermatitis of the hands or feet
- Ichthyosis, palmar hyperlinearity, keratosis pilaris
- Nipple eczema
- White dermatographism and delayed blanch response
- Anterior subcapsular cataracts, keratoconus
- Elevated serum IgE levels
- Positive results of immediate-type allergy skin tests
- Early age at onset
- Dennie lines (Dennie-Morgan infraorbital folds)
- Facial erythema or pallor
- Course influenced by environmental and/or emotional factors”””
19
Q
“What is true regarding asthma?
a. Chronic inflammatory and airway hyperresponsiveness
b. Chronic immunologic response
c. Use of bronchodilator to decrease hyperresponsiveness
d. All of the above are true”
A
D
20
Q
“Which has no role in the prevention of asthma?
a. Blockage of the histamine pathway
b. Blockage of Phospholipase A
c. Blockage of leukotrienes
d. None”
A
B
B - MOA of nifedipine
21
Q
“Child puts tongue to palate and scratches it. What is this manifestation of allergic rhinitis?
a. Allergic salute
b. Allergic shiners
c. Nasal crease
d. Allergic cluck”
A
D
“Nelson 21st p1180
Children with AR often perform the allergic salute, an upward rubbing of the nose with an open palm or extended index finger. This maneuver relieves itching and briefly unblocks the nasal airway. It also gives rise to the nasal crease, a horizontal skin fold over the bridge of the nose.
Signs on physical examination include abnormalities of facial development, dental malocclusion, the allergic gape (continuous open-mouth breathing), chapped lips, allergic shiners (dark circles under the eyes), and the transverse nasal crease.”
22
Q
“10-month-old underweight infant presents with pearly string node, whitish plaques on the pharynx and buccal mucosa without previous antibiotic treatment. She was given nystatin for 4 days and provided temporarily relief and noted recurrence of the plaques after. What else will you ask in the history with regards to the cause of the diagnosis?
a. Hygiene
b. T cell defect
c. B cell defect
d. Complement defect”
A
B
“Nelson 21st p1112
Defects in cellular immunity, historically referred to T-cell defects, comprise a large number of distinct immune deficiencies. The manifestations usually include prolonged viral infections, opportunistic fungal or mycobacterial infections, and a predisposition to autoimmunity.”
23
Q
“Test for T-cell deficiency
a. Candida test
b. ANC
c. ESR
d. CRP”
A
A
24
Q
“Murmur with thrill
a. 1/6
b. 2/6
c. 3/6
d. 4/6”
A
D
“Nelson 21st p2353
The intensity of systolic murmurs is graded from I to VI:
I, barely audible;
II, medium intensity;
III, loud but no thrill;
IV, loud with a thrill;
V, very loud but still requiring positioning of the stethoscope at least partly on the chest; and
VI, so loud that the murmur can be heard with the stethoscope off the chest.
In patients who have undergone prior heart surgery, a murmur of grade IV or greater may be heard in the absence of a thrill.”
25
"PMI will move to 5th LICS at
a. 1 year old
b. 2 years old
c. 3 years old
d. 4 vears old"
D
26
"Case of rheumatic fever with the ff: fever, polyarthritis, elevated esr, normal cardiac borders, no murmurs, prolonged P-R interval. Aso positive. Which is true?
A. Something about corticosteroids din and the arthritis na mali
B. Corticosteroids are not necessary because there is no carditis
C. Fulfilled Jones criteria of 1 major and at least 3 minor
D. The difference between RHO and ARF is ECH findings"
B
```
"Nelson 21st p1448
Antiinflammatory agents (e.g., salicylates, corticosteroids) should be withheld if arthralgia or atypical arthritis is the only clinical manifestation of presumed acute RF.
```
Nelson 21st p1449
Patients with carditis and more than minimal cardiomegaly and/or congestive heart failure should receive corticosteroids. The usual dose of prednisone is 2 mg/kg/day in 4 divided doses for 2-3 wk, followed by half the dose for 2-3 wk and then tapering of the dose by 5 mg/24 hr every 2-3 days. When prednisone is being tapered, aspirin should be started at 50 mg/kg/day in 4 divided doses for 6 wk to prevent rebound of inflammation."
27
```
"Female athlete with sudden LOC. What part of history will you be most worried of? Event happened after:
A. Quarrel with boyfriend
B. Stress after difficult exam
C. Playing with volleyball
D. None of the above"
```
C
"Nelson 21st p2448
Approximately 65% of sudden deaths are a result of heart-related problems in patients with either normal or congenitally (corrected, palliated, or unoperated) abnormal hearts. Competitive high school sports (basketball, football) are high-risk environmental factors. Common identifiable causes of death in competitive athletes includes hypertrophic cardiomyopathy, with or without obstruction to left ventricular outflow, other cardiomyopathies, and anomalous coronary arteries; most are sudden unexplained deaths"
28
```
"Most prognosticating diagnostics in Kawasaki
A. 2D Echo
B. ESR
C. CRP
D. ECG"
```
A
"Nelson 21st p1316
The vast majority of patients with KD return to normal health; timely treatment reduces the risk of coronary aneurysms to <5%. Acute KD recurs in 1–3% of cases. The prognosis for patients with CCA depends on the severity of coronary disease; therefore, recommendations for follow-up and management are stratified according to coronary artery status."
29
"Preterm infant, seen at the local health center. Noted with continuous murmur, bounding pulses. What is the most probable lesion?
a) VSD
b) ASD
c) PDA
d) TOF"
C
Nelsons 21st C2382 A small PDA is associated with normal peripheral pulses, and a large PDA results in bounding peripheral arterial pulses and a wide pulse pressure, caused by runoff of blood into the pulmonary artery during diastole. The classic continuous murmur is described as “machinery-like” in quality.
30
"Initial management of hypercyanotic spell includes the following, except:
a) knee chest position
b) oxygen support
c) sedation
d) intubation"
D
"Nelsons 21st p2397
Depending on the frequency and severity of hypercyanotic attacks, 1 or more of the following procedures should be instituted in sequence: (1) placement of the infant on the abdomen in the knee-chest position while making certain that the infant’s clothing is not constrictive, (2) administration of oxygen (although increasing inspired oxygen will not reverse cyanosis caused by intracardiac shunting), and (3) injection of morphine subcutaneously in a dose not in excess of 0.2 mg/kg. Calming and holding the infant in a knee-chest position may abort progression of an early spell. Premature attempts to obtain blood samples may cause further agitation and may be counterproductive."
31
```
"2 hour old neonate, who was born vigorous and without complications, developed progressive cyanosis. Chest PE is normal. What is the most probable lesion?
a. ASD
b. VSD
c. TOF
D. TGA"
```
D
"VSD (Nelsons 21st p2379)
Characteristically, a loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and it is frequently accompanied by a thrill.
ASD (Nelsons 21st p2375)
In most patients with an ASD, the characteristic finding is that the second heart sound (S2) is widely split and fixed in its splitting during all phases of respiration.
TOF (Nelsons 21st p2397)
The systolic murmur is usually loud and harsh; it may be transmitted widely, especially to the lungs, but is most intense at the left sternal border. The murmur is generally ejection in quality at the upper sternal border, but it may sound more holosystolic toward the lower sternal border. It may be preceded by a click. The murmur is caused by turbulence through the RVOT. It tends to become louder, longer, and harsher as the severity of pulmonary stenosis increases from mild to moderate; however, it can actually become less prominent with severe obstruction, especially during a hypercyanotic spell, because of shunting of blood away from the RV outflow through the aortic valve.
TGA (Nelsons p2407-2408)
Physical findings, other than cyanosis, may be remarkably nonspecific. The precordial impulse may be normal, or a parasternal heave may be present. The second heart sound (S2) is usually single and loud, although it may be split. Murmurs may be absent, or a soft systolic ejection murmur may be noted at the mid-left sternal border."
32
"What is the length of prophylaxis for rheumatic fever with carditis? Patient has mild mitral regurgitation.
a) 5 years after last episode
b) 10 years after last episode
c) 18 years after last episode
d) for life"
D
"Nelson 21st p1450. Table 210.5. Duration of prophylaxis for people who have had acute rheumatic fever: AHA recommendations
Rheumatic fever without carditis - 5 yr or until 21 yr of age, whichever is longer
Rheumatic fever with carditis but without residual heart disease (no valvular disease) - 10 yr or until 21 yr of age, whichever is longer
Rheumatic fever with carditis and residual heart disease (persistent valvular disease) - 10 yr or until 40 yr of age, whichever is longer, stometimes lifelong prophylaxis "
33
```
"The 1st heart sound is best heard at the:
a. Right sternal border
b Left sternal
C. Midclavicular
d. Apex"
```
D
"The first heart sound is best heard at the apex, whereas the second heart sound should be evaluated at the upper left and right sternal borders. S1 is caused by closure of the AV valves. S2 is caused by closure of the semilunar valves.
Nelson 21st Ch 449, p 2352"
34
"Infant with blue lower extremities and pink right upper extremity?
a. Shunt through the PDA with COA
b. VSD
c. TOF
d. TAPVR"
A
35
"Decreased and muffled heart sounds, unusually quiet precordium, dyspneic patient. Diagnosis?
a. Pericardial effusion
b. Heart failure
c. Myocarditis
d. Endocarditis"
A
36
```
"A neonate presented with fever and cyanosis on the 3rd DOL. On PE, cyanotic, tachycardic, cardiomegaly by CXR
A. Sepsis
B. TOF
C. Viral myocarditis
D. cardiac fibroelastosis"
```
C
37
"A Patient came in with 3/6 holosystolic murmur but otherwise asymptomatic. CXR: no cardiomegaly
a. ASD
b. VSD
c. TOF
d. TGA"
B
"VSD (Nelsons 21st p2379)
Characteristically, a loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and it is frequently accompanied by a thrill.
ASD (Nelsons 21st p2375)
In most patients with an ASD, the characteristic finding is that the second heart sound (S2) is widely split and fixed in its splitting during all phases of respiration.
TOF (Nelsons 21st p2397)
The systolic murmur is usually loud and harsh; it may be transmitted widely, especially to the lungs, but is most intense at the left sternal border. The murmur is generally ejection in quality at the upper sternal border, but it may sound more holosystolic toward the lower sternal border. It may be preceded by a click. The murmur is caused by turbulence through the RVOT. It tends to become louder, longer, and harsher as the severity of pulmonary stenosis increases from mild to moderate; however, it can actually become less prominent with severe obstruction, especially during a hypercyanotic spell, because of shunting of blood away from the RV outflow through the aortic valve.
TGA (Nelsons p2407-2408)
Physical findings, other than cyanosis, may be remarkably nonspecific. The precordial impulse may be normal, or a parasternal heave may be present. The second heart sound (S2) is usually single and loud, although it may be split. Murmurs may be absent, or a soft systolic ejection murmur may be noted at the mid-left sternal border."
38
```
"A 3-month old male was brought to the OPD because of skin lesions. Two weeks PTC, diffuse scaling & crusting were noted on the scalp. His mother applied virgin coconut oil twice daily but no improvement was noted. Three days PTC, greasy, scaly & erythematous papules appeared on the face, neck, axillae & diaper areas. The lesions did not seem to be pruritic because he continued to have good suck & slept well. What is the most probable diagnosis?
A. Psoriasis
B. Atopic dermatitis
C. Candidosis
D. Seborrheic dermatitis"
```
D
"Nelson 21st p3495
Seborrheic dermatitis is a chronic inflammatory disease most common in infancy and adolescence that parallels the distribution, size, and activity of the sebaceous glands.
The disorder may begin in the 1st month of life, and typically resolves by 1 yr. Diffuse or focal scaling and crusting of the scalp, sometimes called cradle cap, may be the initial, and at times, the only manifestation. A greasy, scaly, erythematous papular dermatitis, which is usually nonpruritic in infants, may involve the face, neck, retroauricular areas, axillae, umbilicus, and diaper area. "
39
"An 11-year old female was seen at the OPD because of multiple comedones on the central area of her face. Which of the following interventions has proven benefit ?
A. Avoid all forms of nuts & oily foods.
B. Repetitive cleansing of the face with preparations containing hexachlorophene
C. Apply topical retinoids for 6 -8 weeks
D. Engage in physical activities to improve circulation"
C
"Nelson 21st p3577
With the exception of isotretinoin therapy, no evidence shows that early treatment alters the course of acne
Little evidence shows that the ingestion of particular foods can trigger acne flares
No evidence shows that preparations containing alcohol or hexachlorophene decrease acne, because surface bacteria are not involved in the pathogenesis.
All topical preparations must be used for 6-8 wk before their effectiveness can be assessed. Retinoids may be used alone for mild acne, but combination therapy is frequently more effective. A popular and effective combination is use of benzoyl peroxide gel in the morning and a retinoid at night"
40
"Lab finding consistent with Cushing's disease
a. High urinary cortisol
b. Hypoglycemia
c. Hypotension
d. None of the above"
A
"Nelson 21st p2984 Cushing syndrome
Urinary excretion of free cortisol is increased. This is best measured in a 24 hour urine sample and is expressed as a ratio of micrograms of cortisol excreted per gram of creatinine.
Hypertension and hyperglycemia usually occur; hyperglycemia may progress to frank diabetes."
41
"To determine bone age maturation, which is not examined on xray?
a. Hand
b. Wrist
c. Knee
d. Ankle"
D
"AAP Vol 140 Issue 6 (December 2017)
Bone age is an interpretation of skeletal maturity, typically based on radiographs from the left hand and wrist or knee..."
42
"Hormone that has no role in bone maturation in adolescents?
a. Thyroid hormone
b. GH
c. Adrenal
d. Estrogen"
C
"Labo ng choice ng adrenal hindi ko gets kung anong adrenal
Nelson 21st p2876
The biologic effects of growth hormone include increases in linear growth, bone thickness, soft tissue growth, protein synthesis, fatty acid release from adipose tissue, insulin resistance, and blood glucose
Nelson 21st p2912
Thyroid hormones increase oxygen consumption, stimulate protein synthesis, influence growth and differentiation, and affect carbohydrate, lipid, and vitamin metabolism.
Nelson 21st p2957
Glucocorticoids have multiple effects on carbohydrate, lipid, and protein metabolism. They also regulate immune, circulatory, and renal function. They influence growth, development, bone metabolism, and CNS activity.
In stress situations, glucocorticoid secretion can increase up to 10-fold. This increase is believed to enhance survival through inmproved cardiac contractility, cardiac output, sensitivity to the pressor effect of catecholamines and other pressor hormones, work capacity of the skeletal muscles, and capacity to mobilize energy stores.
Nelson 21st p2958
The most significant effect on long-term glucocorticoid excess on calcium and bone metabolism is osteoporosis. Glucocorticoids inhibit osteoblastic activity by decreasing the number and activity of osteoblasts. Glucocorticoids also decrease osteoclastic activity but to a lesser extent, leading to low bone turnover and overall negative balance. The tendency of glucocorticoids to lower serum calcium and phosphate causes secondary hypoparathyroidism. These actions decrease bone accretion and results in a net loss of bone material.
Nelson 21st p2993
Maturation and closure of the epiphyses is estrogen-dependent, as demonstrated by a very tall 28 year old, completely masculinized male with continued growth as a result of incomplete closure of epiphyses, who had complete estrogen insensitivity caused by an estrogen-receptor defect.
"
43
"2-week-old neonate with vomiting, Na 124, K 6, Cl 100, HC03 15
a. Congenital adrenal hyperplasia
b. Congenital hypothyroidism
c. ACTH insensitivity syndrome
d. None of the above"
A
"Nelson 21st p2973 Congenital adrenal hyperplasia
The signs and symptoms of of cortisol and aldosterone deficiency, and the pathophysiology underlying them, are essentially those described in Chapter 593. These include progressive weight loss, anorexia, vomiting, dehydration, weakness, hypotension, hypoglycemia, hyponatremia, and hyperkalemia. These problems typically first develop in affected infants at approximately 10-14 days of age. Without treatment, shock, cardiac arrythmias, and death may occur within days or weeks.
Nelson 21st p2917 Congenital hypothyroidism
Because symptom are usually, not present at birth, the clinical depends on neonatal screening tests for the diagnosis of congenital hypothyroidism. The anterior and posterior fontanels are open widely, and the presence of this sign at birth may be a clue to early recognition of contenital hypothyroidism. Prolonged jaundice may be present due to delayed maturation of hepatic glucoronide conjugation. Affected infants cry little, sleep much, have poor appetitets, and are generally sluggish.
Feeding difficulties, especially sluggishness, lack of interest, and choking spells during nursing, can be present during the first month of life. Respiratory difficulties, partly causes by macroglossia, include apneic episodes, noisy respiration, and nasal obstruction. There may be constipation unresponsive to treatment. The abdomen is large, and umbilical hernia may be present. The temperature may be subnormal (<35C) and the skin may be cold and mottled, particularly on the extremities. Edema of the genitals and extremities may be present. The pulse is slow and heart murmurs, cardiomegaly, and asymtomatic pericardial effusion are common. Macrocytic anemia is often present. Because symptoms appear gradually and may be nonspecific, the clinical diagnosis of neonatal hypothyroidism is often delayed.
"
44
```
"Case of DKA given insulin. What is the most common adverse event to anticipate?
A. Hyponatremia, dilutional
B. Hypoglycemia
C. Hypertension
D. Bleeding"
```
B
"Nelson 21st p3030
Insulin must be given to promote movement of glucose into cells, to subdue hepatic glucose production, and to halt the movement of fatty acids from the periphery to the liver. An initial insulin bolus does not speed recovery and may increase the risk of hypokalemia and hypoglycemia. Therefore, insulin infusion is typically begun without an insulin bolus at a rate of 0.1 units/kg/hr. This approximates maximal insulin output in normal subjects during an oral glucose tolerance test."
45
"Anterior pituitary does not secrete this hormone
a) Antidiuretic hormone
b) Prolactin
c) growth hormone
d) TSH"
A
"Nelson 21st p2876
Five cell types in the anterior pituitary produce 6 peptide hormones:
1. Somatotrope - growth hormone (GH)
2. Lactotropes - prolactin (PRL)
3. Thyrotropes - thyroid stimulating hormone (TSH)
4. Corticotropes - proopiomelanocortin (precursor of adrenocorticotropic hormone, ACTH)
5. Gonadotropes - luteinizing hormone (LH) and follicle stimulating hormone (FSH)
```
Nelson 21st p2880
Argenine vasopresin (antidiuretic hormone, ADH) and oxytocin are the 2 hormones produced by neurosecretion in the hypothalamic nuclei and released from the posterior pituitary. "
```
46
"A child was diagnosed with hyperthyroidism. What drug will you start?
a. Levothyroxine
b. PTU
c. Methimazole
d. Propranolol"
C
"Nelson 21st p2932
Methimazole is the first line ATD for children with Graves disease and functions by blocking the organification of iodide necessary to synthesize thyroid hormone. Methimazole has a long serum half-life (6-8 hr) that allows once- or twice-daily dosing.
Propylthiouracil is a similar ATD that is effective in hyperthyroidism, but its use is not recommended in children due to its potential to cause liver failure."
47
"Most common cause of congenital hypothyroidism
a. Autoimmune thyroiditis
b. Iodine deficiency
c. Thyroid dysgenesis
d. Iodine excess"
C
"Nelson 21st p2194
Thyroid dysgenesis is the most common cause of permanent congenital hypothyroidism, accounting for 80-85% of cases. "
48
"HbA1C reflects blood glucose from
a. 1-2 months
b. 2-3 months
c. 3-4 months
d. 4-5 months"
B
"Nelson 21st p3036
Because a blood sample at any given time contains a mixture of red blood cells of varying ages, exposed for varying times to varying blood glucose concentrations, an HbA1c measurement reflects the average blood glucose concentration from the preceding 2-3 mo."
49
"A 13- year-old female without breastbud and menarche is concerned. Which will support delayed puberty?
A. No menarche by 16 years old
B. No breast development by 13 years old
C. Both of the above
D. None of the above"
B
"Nelson 21st p2907
Delayed puberty is a failure of development of any pubertal feature by 13 yr of age in females and 14 yr of age in males
Delay or absence of puberty is caused by
1. Constitutional delay: a variant of normal
2. Hypogonadotropic hypogonadism: low gonadotropin levels as a result of a defect of the hypothalamus/pituitary gland
3. Hypergonadotropic hypogonadism: high gonadotropic levels as a result of a lack of negative feedback because of a gonadal problem. Females may have isolated absence of adrenarche with normal breast development """
50
```
"A patient with generalized obesity, short stature, with fracture of the lower extremities.
A. Addison's disease
B. Cushing syndrome
C. Congenital hypothyroidism
D. None of the above"
```
B
"Nelson 21st p2984 Cushing syndrome
The face is rounded, with prominent cheeks and a flushed appearance (moon facies). Generalized obesity is common in younger children. In children with adrenal tumors, signs of abnormal masculinization occur frequently; accordingly there may be hirsutism on the face and trunk, pubic hair, acne, deepening of the voice, and enlargement of the clitoris in girls. Growth is impaired, with length falling below the 3rd percentile, except when significatn virilization produces normal or even accelerated growth. Hypertension is common and may occasionally lead to heart failure. An increased susceptibility to infection may also lead to sepsis.
In older children, in addition to obesity, short stature is a common presenting feature. Gradual onset of obesity and deceleration or cessation of growth may be the only early manifestations. Older children most ofen have severe obesityof the face and trunk compared to the extremities. Purplish striae on the hips, abdomen, and thighs are common. Pubertal development may be delayed, or amenorrhea may occur in girls pas menarche. Weakness, headache, and emotional lability may be prominent. Hypertension and hyperglycemia may occur; hyperglycemia may progress to frank diabetes. Osteoporosis is common and may cause pathologic fractures. "
51
```
"Adolescent female engaged in ballet still with amenorrhea, eating problem, and low bone density.
A. Anorexia nervosa
B. Bulimia nervosa
C. Female athlete triad
D. None of the above"
```
C
"Nelson p3706
In females with eating disorders and those who exercise to the point of excessive weight loss with amenorrhea or oligomenorrhea, exercise can be detrimental to bone mineral acquisition, resulting in reduced bone mineral content, or osteopenia.
Specifically, bone mineralization is negatively affected by amenorrhea (absence of menstruation for ≥3 consecutive months). This may be influenced by abnormal eating patterns, or disordered eating. When occurring together, disordered eating, amenorrhea, and osteoporosis
form the female athlete triad."
52
"Thiocyanite from Kamote causes thyroid enlargement due to
a. Deiodinase deficiency
b. Competitive binding with iodine
c. Destruction of thyroid follicles
d. None of the above"
B
"Nelson 21st p2927 Table 583.1. Goitrogens and their mechanism
Cassava, lima beans, sweet potato - contain cyanogenic glucosides that are metabolized to thiocyanates that compete with iodine for uptake by the thyroid "
53
```
"Child with anterior neck mass treated with thyroid meds but no response to treatment and has normal thyroid function
tests. what to do next?
A. Observe
B. Repeat thyroid function tests
C. FNAB
D. Increase medication dose"
```
C
54
"10-year-old female came to the OPD. PE shows purplish striae at abdomen, hips and thigh. Hypertension is also noted. What is your consideration?
a. Cushing Syndrome
b. Pheochromocytoma
c. Metabolic syndrome
d. None of the above"
A
"Nelson 21st p2984 Cushing syndrome
The face is rounded, with prominent cheeks and a flushed appearance (moon facies). Generalized obesity is common in younger children. In children with adrenal tumors, signs of abnormal masculinization occur frequently; accordingly there may be hirsutism on the face and trunk, pubic hair, acne, deepening of the voice, and enlargement of the clitoris in girls. Growth is impaired, with length falling below the 3rd percentile, except when significatn virilization produces normal or even accelerated growth. Hypertension is common and may occasionally lead to heart failure. An increased susceptibility to infection may also lead to sepsis.
In older children, in addition to obesity, short stature is a common presenting feature. Gradual onset of obesity and deceleration or cessation of growth may be the only early manifestations. Older children most ofen have severe obesityof the face and trunk compared to the extremities. Purplish striae on the hips, abdomen, and thighs are common. Pubertal development may be delayed, or amenorrhea may occur in girls pas menarche. Weakness, headache, and emotional lability may be prominent. Hypertension and hyperglycemia may occur; hyperglycemia may progress to frank diabetes. Osteoporosis is common and may cause pathologic fractures.
Nelson 21st p2986 Phaeochromocytoma
Phaeochromocytomas detected by surveillance of patients who are known carriers of mutations in tumor-suppression genes may be asymptomatic. Otherwise, patients are detected owing to hypertension, which results from excessive secretion of metanephrines, epinephrine, and norepinephrine. Between attacks of hypertension, the patient may be free of symptoms. During attacks, the patient complains of headache, palpitations, abdominal pain and dizziness; pallor, vomiting, and sweating also occur. Seizures and other manifestations of hypertensive encephalopathy may occur.
Nelsons 21st p351
The metabolic syndrome consists of central obesity. hypertension, glucose intolerance, and hyperlipidemia "
55
"Which of the following is compatible with brain death?
a. Trauma victim, no brainstem function, Temp 33C
b. Hypoxic baby, isoelectric on EEG
c. Drowning victim, no brainstem function
d. All of the above"
C
"Nelson 21st p563-565
Brain death is determined by clinical assessment. Although ancillary tests such as EEG and cerebral blood flow studies are sometimes used to assist in making the diagnosis, repeated clinical examinations is the standard for diagnosis.
The 3 components for determining brain death are demonstration of coexisting irreversible coma with a known cause, absence of brainstem reflexes, and apnea.
Before determiniation of brain death can be made, it is of utmost importance that the cause of the coma be determined to rule out a reversible condition.
Contributing factors that can interfere with the neurologic examination must be corrected:
- Temp >35C
- SBP or MAP in acceptable range
- Sedative/analgesic drug effect excluded as a contributing factor
- Metabolic intoxication excluded as a contributing factor
- Neuromuscular blockade excluded as a contributing factor "
56
"Which is a poor prognostic factor for drowning
a. Arrived at ER GCS 10
b. Submersion for more than 5 minutes
c. No return of cardiac activity for 25 minutes
d. HR 0 on arrival at ER"
C
"Nelson 21st p612
Poor outcome is highly likely in patients with deep coma, apnea, absence of pupillary responses, and hyperglycemia in the ED, with submersion durations >10min and with failure of response to CPR given for 25min"
57
"Case of a 2 month old infant with diarrhea, sunken eyeballs, oliguria, present with distress. What is the cause of tachypnea?
a. Metabolic acidosis
b. Metabolic alkalosis
c. Respiratory alkalosis
d. Respiratory acidosis"
A
58
"Child with diarrhea. Sunken eyeballs. What is the ABG?
a. Metabolic acidosis
b. Metabolic alkalosis
c. Respiratory alkalosis
d. Respiratory acidosis"
A
59
"What fluids will you use for resuscitation?
a. 20 ml/kg 0.9 NSS
b. 10 ml/kg 0.9 NSS
c. 10 ml/kg DSLR
d. 20 ml/kg D5NSS"
A
60
"In drowning, which causes apnea
a. Water in pharynx
b. Myocardial hypoxia
c. Muscle spasm
d. Hypoxic ischemic injury of CNS"
D
"Nelson 21st p609
After experimental submersion, a conscious animal initially panics, trying to surface. During this stage, small amounts of water enter the hypopharynx, triggering laryngospasm. There is a progressive decrease in arterial blood oxyhemoglobin saturation (SaO2), and the animal soon loses consciousness from hypoxia. Profound hypoxia and medullary depression lead to terminal apnea. At the same time, the cardiovascular response leads to progressively decreasing cardiac output and oxygen delivery to other organs"
61
"Hydration for burns is similar to hydration for the ff EXCEPT:
a. Dengue
b. Shock
c. Anaphylaxis
d. Diarrhea"
C
62
"Dengue shock is best resuscitated with
a. LR
b. D5LR
c. D5NSS
d. DlOW"
A
"Nelsons 21st p1764
Rapid intravenous replacement of fluids and electrolytes can frequently sustain patients until spontaneous recovery occurs. Normal saline is more effective that the more expensive Ringer lactated saline in treating shock. When the pulse pressure is <=10 mmHg or when elevation of the hematocrit persists after the replacement of fluids, plasma or colloid preparations are indicated. Oral rehydration of children who are being monitored is useful. "
63
"1 old child was brought to ER for cyanosis. Revived by mothers by blowing on his face. Work up done and you noted posterior rib fracture at the 9th & 10th ribs. What will you do?
a. Discard fractures
b. Report to CPU
c. Refer to Ortho for comanagement of fractures
d. None of the above"
B
"Nelsons 21st p101
Fractures that strongly suggest abuse include classic metaphyseal lesions, posterior rib fractures, and fractures of the scapula, sternum, and spinous processes, especially in young children. These fracture all require more force that would be expected from a minor fall or routine handling and activities of a child.
In abused infants, rub, metaphyseal, and skull fractures are most common. Femoral and humeral fractures in nonambulatory infants are also very worrisome for abuse. With increasing mobility and running, toddlers can fall with enough rotational force to cause a spiral femoral fracture. Multiple fractures in various stages of healing are suggestive of abuse.
Clavicular, femoral, supracondylar, humeral, and distal extremity fractures in children older than 2 yr are most likely noninflicted unless they are multiple or accompanied by other signs of abuse. "
64
"3 month old with profuse tearing on right eye. What will you do?
a. Culture
b. Cold compress
c. Criggler massage
d. Topical antibiotic"
C
65
```
"Patient lost consciousness after drinking from a metal polish container. Etiology?
A. Lead
B. Mercury
C. Cyanide
D. Arsenic"
```
C
66
```
"How to manage jellyfish sting
A. Seawater
B. Cold compress
C. Hydrogen peroxide
D. Urinate"
```
A
Uptodate: If vinegar is not available, the rescuer should remove visible tentacles by hand and then rinse the sting site with seawater. Fresh water promotes nematocyst activation and should not be used for rinsing the sting site. Because pressure during manual tentacle removal can promote nematocyst discharge and seawater rinsing may distribute intact nematocysts to previously unaffected areas, it is important to apply vinegar first, if at all possible [33].
67
```
"Earliest derangement in any form of shock?
A. Hypoglycemia
B. Hypocalcemia
C. Hyperkalemia
D. Hyponatremia"
```
A
68
"Case of a sudden paleness and no pulse at the PER. What to do first?
A. Attempt IV access
B. Give rescue breaths
C. Give CPR
D. Refer to your senior resident or consultant"
C
69
```
"Effective CPR is characterized by:
A. Allowing full chest recoil
B. 30:3 for single rescuer. 15:3 for double rescuer
C. 160 compressions per minute
D. Hyperventilation to RR 50-60"
```
A
"""High quality CPR:
1. Chest compression fraction >80%
2. Compression rate of 100-120bpm (30:2 for single rescuer, 15:2 for double resucer)
3. Compression depth of at least 50 mm (2 inches) in adults and at least 1/3 the AP dimension of the chest in infants and children.
4. No excessive ventilation
(https://cpr.heart.org/en/resuscitation-science/high-quality-cpr)"""
70
```
"Case of severe sepsis and pneumonia. What is deranged?
A. Metabolic acidosis
B. Metabolic and respiratory acidosis
C. Respiratory acidosis
D. Metabolic alkalosis"
```
B
71
"Scooter or skateboard fracture case. What is the most common bone involved?
a) Distal radius
b) Proximal radius
c) Distal ulna
d) Proximal ulna"
A
"""Nelson 21st p3666
Fractures of the wrist and forearm are very common fractures in children, accounting for nearly half of all fractures seen in the skeletally immature.
The most common mechanism of injury is a fall on the outstretched hand. Eighty percent of forearm fractures involve the distal radius and ulna, 15% involve the middle third, and the rest are rare fractures of the proximal third of the radius or ulnar shaft.
Orthobullets
Distal Radius Fractures are the most common site of pediatric forearm fractures and generally occur as a result of a fall on an outstretched hand with the wrist extended"""
72
"Overdose on metoclopropramide developed tardive dyskinesia symptoms. What is the antidote?
a) Diphenhydramine
b) Prochlorperazine
c) Paracetamol
d) Ephedrine"
A
73
"Child with fracture, complication?
a. Fat embolus
b. Soft tissue infection
c. DVT
d. Avascular necrosis"
A
74
"An infant came in with head trauma due to fall. Skull xray was normal however on x ray there was a finding of fractured tibia and ribs. What is your impression?
a. Accident
b. Child abuse
c. Osteogenesis imperfecta
d. None of the above"
B
"""Nelsons 21st p101
Fractures that strongly suggest abuse include classic metaphyseal lesions, posterior rib fractures, and fractures of the scapula, sternum, and spinous processes, especially in young children. These fracture all require more force that would be expected from a minor fall or routine handling and activities of a child.
In abused infants, rub, metaphyseal, and skull fractures are most common. Femoral and humeral fractures in nonambulatory infants are also very worrisome for abuse. With increasing mobility and running, toddlers can fall with enough rotational force to cause a spiral femoral fracture. Multiple fractures in various stages of healing are suggestive of abuse.
Clavicular, femoral, supracondylar, humeral, and distal extremity fractures in children older than 2 yr are most likely noninflicted unless they are multiple or accompanied by other signs of abuse. """
75
"When will you do compressions?
a. < 60 bpm with good perfusion
b. < 60 bpm with poor perfusion
c. < 60 bpm with normal GCS
d. Respiratory insufficiency"
B
"Nelson 21st p538
A heart rate <60 bpm with poor perfusion is an indication to begin chest compression. "
76
"What will you do if you witnessed a victim collapse?
a. Activate AED
b. Activate emergency response system
c. Recheck AED settings
d. Assess if you need to do CP"
B
"
Nelsons 21st p530
A child found unresponsive from an unwitnessed collapse should be approached with a gentle touch and the verbal question ""Are you OK?"" If there is no response, the caregiven should immediately shout for help and send someone to activate the emergency response system and locate an automated external defibrillator (AED).
If the caregiver witnesses the sudden collapse of a child, the caregiver should have a higher suspicion for a sudden cariac event. In this case, rapid deployment of an AED is crucial. Any interruptions in care of the child to activate EMS and located the nearest AED should be very brief. "
77
"Teenager with periumbilical pain, after a day PE (+) RLQ pain. Diagnosis?
a. Torsion
b. Appendicitis
c. UTI
d. Acute pancreatitis"
B
"Nelson 21st p2049
Abdominal pain is consistently the primary symptom in acute appendicitis, benginning shortly after the onset of illness...thus, the classic description of periumbilical mid-abdominal pain migrating to the RLQ. "
78
"What is the initial manifestation of lead intoxication?
a. GI: abdominal pain and constipation
b. CNS: headache and neurological deficits
c. Nephro: hematuria and hypertension
d. Respi : respiratory distress"
A
"
Nelsons 21st p3799
Gastrointestinal symptoms of lead poisoning include anorexia, abdominal pain, vomiting, and constipation, often occuring and recurring over a period of weeks. Children with BLL >20 ug/dl are affected.
CNS symptoms are related to worsening cerebral edema and increased ICP. Headaches, change in mentation, lethargy, papilledema, seizures, and coma leading to death are rarely seen at levels lower than 100 ug/dl.
Other organs may also be affected by lead toxicity, but symptoms usually are not apparent in children. At high levels (>100 ug/dl), renal tubular dysfunction is observed. "
79
```
"Child came in with decreased urine output for 24 hours, CRT> 2 seconds, dry lips with no tears, sunken eyeballs. What
will you do?
a. Give ORS 50 -100 ml/kg
b. Restrict nutrition
c. Bolus of 20 ml/kg of PNSS
d. Hook to 10LPM via FM"
```
C
"Severe dehydration, resuscitate via IV boluses of 20ml/kg pNSS
Nelsons 21st p429 Table 70.1. Clinical evaluation of dehydration
1. Mild dehydration (<5% in an infant, <3% in an older child) - normal or increased pulse, decreased urine output, thirsty, normal physical findings
2. Moderate dehydration (5-10% in an infant, 3-6% in an older child) - tachycardia, little or no urine output, irritable/lethargic, sunken eyes and fontanel, decreased tears, dry mucus membranes, mild delay in elasticicty (skin turgor), delayed capillary refill (>1.5 sec), cool and pale
3. Severe dehydration (>10% in an infant, >6% in an older child) - peripheral pulses either rapid and weak or absent, decreased BP, no urine output, very sunken eyes and fontanel, no tears, parched mucuous membranes, delayed elasticity (poor skin turgor), very delayed capillary refil (>3 sec), cool and mottled, depressed consciousness"
80
"When will you brace a 14 y/o child with scoliosis?
a. 25 - 30 degrees
b. 10-20 degrees
c. 20- 25 degrees
d. More than 30 degrees"
D
"Nelson 21st p3636
Braces are offered for treatment of skeletally immature patients with curves >30 degrees at the first visit, or in patients who are being followed and have developed progression of their curvature beyond 25 degrees. Bracing is ineffective in curvatures >45 degrees, as these patients have already reached the threshold for surgical intervention."
81
"Define hyperkyphosis.
a. > 50 degrees
b. 40-50 degrees
c. 30-40 degrees
d. 20-30 degrees"
A
"Nelson 21st p3640
The normal thoracic spine has 20-50 degrees of kyphosis as measured from T3 to T12 using the Cobb method on a standing lateral radiograph of the spine. A thoracic kyphosis in excess of the normal range of values is termed hyperkyphosis. These patients may present with cosmetic concerns, back pain, or both."
82
"Most common etiologic agent in osteomyelitis?
a. Streptococcus epidermidis
b. Salmonella typhi
c. Staphylococcus aureus
d. Kingella kingae"
C
"Nelson 21st p3670
Bacteria are the most common pathogens in acute skeletal infections. Staphylococcus aureus is the most common infecting organism in osteomyelitis among all age groups, including newborns."
83
"Child with history of trauma to the right leg. Days after noted to be swelling, erythematous with pain over the site. Diagnosis?
a. Cellulitis
b. Pyomyositis
c. Myositis
d. Osteomyelitis"
D
"Nelson 21st p3671
Older infants and children are more likely to have pain, fever, and localizing signs such as edema, erythema, and warmth. With involvement of the lower extremities, limp, or refusal to walk is seen in approximately half of patients.
Focal tenderness over a long bone can be an important finding. Local swelling and redness with osteomyelitis suggests spread of infection beyond the metaphysis and into the subperiosteal space, representing a secondary soft tissue inflammatory response"
84
"Indication for intubation except?
a. Normal 02 sat
b. CO2 retention
c. Inability to maintain airway
d. Inadequate oxygenation"
A
"Nelsons 21st p536-537
A child generally requires intubation when at least one of these conditions exists:
1. The child is unable to maintain airway patency or protect the airway against aspiration (as occurs in settings of neurologic compromise)
2. The child is failing to maintain adequate oxygenation
3. The child is failing to control blood carbon dioxide levels and maintain safe acid-base balance
4. Sedation and/or paralysis is required for procedure
5. Care providers anticipate a deteriorating course that will eventually lead to any of the first 4 conditions
There are few absolute contraindications to tracheal intubation, but experts generally agree that in settings of known complete airway obstruction, endotracheal intubation should be avoided, and emergency cricothyrodotomy performed instead. Another important consideration is to ensure that caregivers provide appropriate cervical spine protection during the intubation procedure when neck or spinal cord injury is suspected. "
85
"Leading cause of injury/ death in children?
a. Fall
b. Drowning
c. Vehicular accident
d. Head Injury"
C
"Leading causes of pediatric death (US, 2016)
1. Motor vehicle crashes
2. Firearm-related injuries
3. Malignant neoplasms
4. Suffocation
Leading causes of pediatric death, Philippines (DOH, 2010)
Infant
1. Bacterial sepsis
2. Pneumonia
3. Respiratory distress of newborn
Age 1-4
1. Pneumonia
2. Gastroenteritis
3. Congenital anomalies
Age 5-9
1. Pneumonia
2. Dengue
3. Drowning
Age 10-14
1. Pneumonia
2. Drowning
3. Diseases of nervous system"
86
```
"A boy incurred fracture of the right arm from skateboarding. What is the most probable involved structure?
A. proximal ulna
B. proximal radius
C. distal ulna
D. distal radius"
```
D
"Nelson 21st p3666
Fractures of the wrist and forearm are very common fractures in children, accounting for nearly half of all fractures seen in the skeletally immature.
The most common mechanism of injury is a fall on the outstretched hand. Eighty percent of forearm fractures involve the distal radius and ulna, 15% involve the middle third, and the rest are rare fractures of the proximal third of the radius or ulnar shaft.
Orthobullets
Distal Radius Fractures are the most common site of pediatric forearm fractures and generally occur as a result of a fall on an outstretched hand with the wrist extended"
87
```
"In sports clearance, when should a pre-participation physical evaluation be done?
A. 6 months before start of school
B. 3 to 4 weeks before start of school
C. 1 year before start of school
D. None of the above"
```
B
"Nelson 21st p3679
At a minimum, a focused annual interim evaluation should be done on an otherwise healthy young athlete. The PSE is optimally performed 3-6 weeks before the start of practice. "
88
```
"Angle in scoliosis that rarely progress after skeletal maturity
A. <30 degrees
B. 45 degrees
C. 50 degrees
D. 60 degrees"
```
A
"Nelson 21st p3636
A curve with an RVAD <20 degrees will resolve in about 80% of cases, while one with an RVAD >20 degrees will progress in over 80% of cases. Curves that resolve typically do so before 2 yr of age."
89
```
"Angle at which a patient with scoliosis will develop restrictive lung disease?
A. >30 degrees
B. >40 degrees
C. >60 degrees
D. >80 degrees"
```
D
"Nelson 21st p3636
A study of curve morphology and pulmonary function found that thoracic curves >70 degrees were associated with below normal pulmonary function. However, patients with curve magnitudes <50 degrees may also have some degree of pulmonary impairment, suggesting that magnitude alone cannot fully predict pulmonary function. Factors such as thoracic kyphosis, curve stiffness, location of curve apex, and degree of vertebral rotation may also impact pulmonary function. Surgical correction is correlated with improved total lung capacity in patients with severe restrictive pulmonary function preoperatively."
90
```
"Patient developed fever and diffuse erythema associated with conjunctivitis, mouth and oral ulcers that preceded skin lesions. With denudation of skin with gentle pressure. On history, with previous intake of Amoxicillin and Cotrimoxazole.
A. Toxic epidermal necrolysis
B. SJS
C. Epidermolysis Bullosa
D. Erythema multiforme"
```
A
"Nelson 21st p3482-3484
Drugs, particularly sulfonamides, NSAIDS, antibiotics, and anticonvulsants, are the most common precipitants of SJS and TEN.
SJS is defined as affected BSA <10%
SJS-TEN overlap syndrome is affected BSA 10-30%
TEN is affected BSA >10%
The skin lesions are accompanied by involvement of 2 or more mucosal surfaces, namely the eyes, oral cavity, upper airway or esophagus, GI tract, and anogenital mucosa
TEN is defined by:
1. Widespread blister formation and morbilliform or confluent erythema, associated with skin tenderness
2. Absence of target lesions
3. Sudden onset and generalization withing 24-48hr
4. Histologic findings of full-thickness epidermal necrolysis and a minimal-to-absent dermal infiltrate "
91
```
"Patient developed sudden onset of rashes described as doughnut -shaped with erythematous outer border, inner pale ring and dusky purple to necrotic center
A. Erythema annulare
B. Erythema multiforme
C. Erythema marginatum
D. Erythema toxicum"
```
B
"Erythema annulare centrifugum (NCBI) - benign lesion
The initial lesions of EAC begin as asymptomatic, annular or polycyclic lesions that grow slowly (2 to 3 mm per day), rarely reaching more than 10 cm in diameter, then develop central clearing. An individual lesion can enlarge to greater than 6 cm in diameter over a period of 1 to 2 weeks. Characteristically, there is a trailing scale at the inner border of the annular erythema
```
Erythema multiforme (Nelson 21st p3481) - vesiculobullous disorder
The diagnosis of erythema multiforme is established by finding the classic lesion: doughnut-shaped, target-like (iris or bull’s-eye) papules with an erythematous outer border, an inner pale ring, and a dusky purple to necrotic center (which sometimes blisters and erodes)
```
```
Erythema marginatum (Nelson 21st p1447) - rheumatic fever
Erythema marginatum is a rare (approximately 1% of patients with acute RF) but characteristic rash of acute RF. It consists of erythematous, serpiginous, macular lesions with pale centers that are not pruritic.
```
```
Erythema toxicum (Nelson 21st p869) - neonatal lesion
In many neonates, small, white papules on an erythematous base develop 1-3 days after birth. This benign rash, erythema toxicum, persists for as long as 1 wk, contains eosinophils, and is usually distributed on the face, trunk, and extremities"
```
92
"5 year old noted to be limping 3 months prior to consult
a. Developmental hip dysplasia
b. Legg-Calve-Perthes disease
c. Osteochondritis dissecans
d. Osgood-Schlatter disease"
B
93
```
"A 17-year old female had difficulty of breathing with severe dehydration & drowsiness. An ABG showed acute metabolic acidosis. The review of systems showed polyphagia, polydipsia, polyuria & pruritus in the vaginal area with a whitish, non-foul smelling & non-bloody discharge. What is the appropriate initial management?
A. Sodium bicarbonate
B. Insulin therapy
C. Fluid replacement
D. Correction of electrolytes"
```
C
"""Severe dehydration, resuscitate via IV boluses of 20ml/kg pNSS
Nelsons 21st p429 Table 70.1. Clinical evaluation of dehydration
1. Mild dehydration (<5% in an infant, <3% in an older child) - normal or increased pulse, decreased urine output, thirsty, normal physical findings
2. Moderate dehydration (5-10% in an infant, 3-6% in an older child) - tachycardia, little or no urine output, irritable/lethargic, sunken eyes and fontanel, decreased tears, dry mucus membranes, mild delay in elasticicty (skin turgor), delayed capillary refill (>1.5 sec), cool and pale
3. Severe dehydration (>10% in an infant, >6% in an older child) - peripheral pulses either rapid and weak or absent, decreased BP, no urine output, very sunken eyes and fontanel, no tears, parched mucuous membranes, delayed elasticity (poor skin turgor), very delayed capillary refil (>3 sec), cool and mottled, depressed consciousness"""
94
```
"Which of the following sites is appropriate for intraosseous infusion?
A. Anterior proximal tibia
B. Iliac crest
C. Posterior superior iliac spine
D. Distal femur"
```
A
"Nelson 21st p543
If venous access is not available within approximately 1 min in a child with cardiopulmonary arrest, an IO needle should be placed in the anterior proximal tibia (with care taken to avoid traversing the epiphyseal plate). "
95
```
"The clinical presentation of a patient in shock does NOT include:
A. Decreased urine output
B. Bradycardia
C. Hypotension
D. Respiratory distress"
```
C
"Nelson 21st p537
The definition of shock does not include hypotension, and it is important for care providers to understand that shock does not begin when blood pressure drops; it merely worsens and becomes more difficult (refractory) to treat once blood pressure is abnormal."
96
"Father refuses to consent to a procedure due to cultural belief. What will you do?
a. Legal matters
b. Best interest of the child
c. Still give transfusion
d. Honor religious views"
A
"A
Nelson 21st P48
Differences in religious beliefs or ethic-based cultural norms may lead to conflict between patients, families, and medical caregivers over the approach to medical care. Pediatricians need to remain sensitive to and maintain an attitude of respect for these differences, yet recognize that an independent obligation exists to provide effective medical treatment to the child. An adult with decision-making capacity is recognized as having the right to refuse treatment on religious or cultural grounds, but children who have not yet developed this capacity are considered a vulnerable population who has a right to treatment. In situations that threaten the life of the child or that may result in substantial harm, legal intervention should be sought if reasonable efforts toward collaborative decision-making are ineffective. If a child’s life is imminently threatened, medical intervention is ethically justified despite parental objections."
97
"Patient with subglottic stenosis and needs respiratory support. Patient is healthy. Parents lack funds. What will you do?
a. Send patient home.
b. Refer to PCSO
c. Stay in the hospital, wean to CPAP, await surgery.
d. None of the above"
C
98
"Who has the least power to give consent?
a. Parents
b. Eldest sibling 21 years old
c. Guardian who is unrelated
d. Grandmother"
C
99
"For participation of child in nontherapeutic research. Which is true
a. Children may be exposed to a limited degree of risk with IRB approval, parental permission, and assent if child is capable
b. Children are not allowed to be exposed to nontherapeutic research
c. Children are allowed to be subjects of nontherapeutic research if with ethics approval
d. None of the above"
A
"Nelson 21st p49
The more widely held opinion is that children may be exposed to a limited degree of risk with IRB approval, parental permission, and assent if the child is capable. The federal regulations allow healthy children to participate in minimal-risk research regardless of the potential benefit to the child"
100
Patient with hypermobile joints,
Ehler Danlos syndrome
101
"Most forgotten role by pedia?
a. Anticipatory care regarding child abuse
b. Pregnant mom should visit paediatrician
c. Infant care of feeding
d. None of the above"
B
102
"Which is most ethical?
A. Pediatrician informing parents of the need to refer to a sub specialist
B. Pediatrician charging exuberant amount for a difficult case
C. Pediatrician not disclosing full prognosis to parents to protect them from emotional stress
D. All of the above are ethical"
A
103
"When will you talk to a parent/guardian re withholding/ withdrawal of life support?
A. Vegetative state of SSPE
B. When going home
C. DIC in meningococcemia
D. Shock, multifactorial from AGE with severe dehydration and sepsis"
A
"Nelson 21st p45
Decisions to use, limit, or withrdraw life sustaining medical treatment should be made after careful consideration of all pertinent factors recognizable by both family and medical staff, including medical likelihood of particular outcomes, burdens on the patient and family, religious and cultural decision-making frameworks, and input by the patient when possible.
The concept of futility has been used to support unilateral forgoing of LSMT by holding that clinicians should not provide futile (or useless) interventions. If medical futility is defined narroly as the impossibility of achieving a desired physiologic outcome, forgoing a particular intervention is ethically justified. "
104
"What is not an example of informed consent?
A. Parents who were explained thoroughly of the case
B. Consent given freely
C. Paternalistic attitude of physicians
D. None of the above"
C
105
"Pregnant female undergoing peritoneal dialysis, informed of possible outcome/risks decided to push through with her pregnancy. What does this represent?
a. Autonomy
b. Beneficence
c. Nonmaleficence
d. Full disclosure"
A
106
"Not true of DNAR?
a. Must be discussed with parents and be part of the comprehensive plan
b. Is not irrevocable
c. Doctors decision is more important than the parents
d. All of the above are true"
C
"Nelson 21st p46
An advance directive is a mechanism that allows patients and/or appropriate surrogates to designate the desired medical interventions under applicable circumstances. Discussion and clarification of resuscitation status should be included in advance care planning. Decisions regarding resuscitation status in the out-of-hospital setting can be an important component of providing comprehensive care. "
107
```
"A research study on antihypertensive. A 13 year-old male agreed to be included in a study and started to experience unwanted symptoms. The principle that suggests that the voice of the patient must be heard is called
A. Assent
B. Dissent
C. Autonomy
D. Beneficence"
```
A
"Nelson 21st p45
For older children, the concept of assent suggests that the voice of the patient must be heard. "
108
"Most common form of child abuse
a. Violence
b. Physical abuse
c. Negligence
d. Sexual abuse"
C
"Nelson 21st p99
Of the 683,000 children with substantiated reports (9.2 per 1,000 children), 78.3% experienced neglect (including 1.9% medical neglect), 17.2% physical abuse, 8.4% sexual abuse, and 6.2% psychological maltreatment."
109
```
"Metabolite affected in nerve conduction
A. Hyponatremia
B. Hypokalemia
C. Hypocalcemia
D. Hypomagnesemia"
```
D
"Nelson 21st p404
Magnesium is a necessary cofactor for hundreds of enzymes. It is important for membrane stabilization and nerve conduction. Adenosine triphosphate and guanosine triphosphate need associated magnesium when they are used by ATPases, cyclases, and kinases"
110
"Which electrolyte is responsible for membrane stabilization and action potential?
a. Sodium
b. Potassium
c. Phosphorous
d. Calcium"
D
"Calcium stabilizes the cell membrane of heart cells, preventing arrythmias
(Nelson 21st p400)"
111
```
"Breastmilk compared with formula milk?
A. Higher osmolarity
B. Higher protein
C. Lower osmolarity
D. None of the above"
```
B
"Without the addition of a thickener, the mean osmolality was similar for raw human milk (281 ±2.23mOsm/kg), pasteurized human milk (285 ± 1.28mOsm/kg), and an infant formula (287 ±15.43mOsm/kg) after 1 hour at 37± 2°C
Breastmilk has MORE components compared to formula milk, EXCEPT for iron, vitamin D, vitamin K"
112
```
"Preterm nutrition should be:
A. Higher in protein
B. Lower in protein
C. Same as term
D. Lowered in calories during the 3rd week"
```
A
"Nelson 21st p904
In the absence of intravenous amino acids, extremely premature infants lose 1-2% of body protein stores per day. IV amino acids and dextrose should be started immediately after birth"
113
```
"Case of a child with dermatitis on the extremities and periorificial area and growth retardation. Deficient nutrient?
A. Biotin
B. Niacin
C. Zinc
D. Cobalamin"
```
C
"Nelson 21st p3597 Acrodermatitis enterohepatica
Acrodermatitis enteropathica is a rare autosomal recessive disorder caused by an inability to absorb sufficient zinc from the diet
The cutaneous eruption consists of vesiculobullous, eczematous, dry, scaly, or psoriasiform skin lesions symmetrically distributed in the perioral, acral, and perineal areas and on the cheeks, knees, and elbows. The hair often has a peculiar, reddish tint, and alopecia of some degree is characteristic. Ocular manifestations include photophobia, conjunctivitis, blepharitis, and corneal dystrophy detectable by slit-lamp examination. Associated manifestations include chronic diarrhea, stomatitis, glossitis, paronychia, nail dystrophy, growth retardation, irritability, delayed wound healing, intercurrent bacterial infections, and superinfection with Candida albicans."
114
```
"Which one has a defect on amino acid metabolism
A. MSUD
B. G6PD
C. Galactosemia
D. Congenital adrenal hyperplasia"
```
A
"MSUD - alpha ketoacid dehydrogenase deficiency, affects metabolism of leucine, isoleucine, valine
G6PD - glucose 6 phosphate deficiency, causes hemolytic anemia through the lack of protective mechanisms against oxidation
Galactosemia - inability to metabolize galactose (carbohydrate metabolism)
Congenital adrenal hyperplasia - no effect on metabolism of carbohydrates, proteins"
115
"Which electrolyte abnormality can cause fatal arrhythmias?
a. Hypernatremia
b. Hyperkalemia
c. Hypermagnesemia
d. Hypercalcemia"
B
"Nelson 21st p400
The most important effect of hyperkalemia result from the role of K in membrane polarization. The cardiac conduction system is usually the dominant concern. Changes in the ECG begin with peaking of the T waves. This is followed by ST segment depression, increase PR interval, flattening of the P wave, and widening of the QRS complex. However, the correlation between K level and ECG changes is poor. This process can eventually progress to ventricular fibrillation. Asystole may occur. "
116
"What is the first sign of hypermagnesemia?
a. Hyporeflexia
b. Apnea
c. Bradycardia
d. Decreased muscle tone"
A
"Hypermagnesemia inhibits acetlycholine release in the neuromuscular junction, producing hypotonia, hyporeflexia, and weakness. Paralysis occurs at high concentrations
(Nelson 21st p407)"
117
```
"Nipple pain is among the most common complaints of breastfeeding mothers. This is most commonly due to:
A. Mastitis
B. Inadequate milk supply
C. Engorgement
D. Poor infant positioning"
```
D
"Nelson 21st p322
Nipple pain is one of the most common complaints of breastfeeding mothers in the immediate postpartum period. Poor infant positioning and improper latch are the most common reasons for nipple pain beyond the mild discomfort felt early in breastfeeding. "
118
"First-time mother asked for practical tips in weaning. Which of the following recommendations is CORRECT?
A. The infant should learn to drink from a cup at around 12 months of age.
B. Phytate intake should be increased to enhance mineral absorption.
C. Give at least 30 ounces per day of cow milk.
D. Give no more than 2 ounces per day of unsweetened fruit juices"
A
"Nelson 21st p326 Table 56.7. Important principles for weaning
- Begin at 6 mo of age.
- At the proper age, encourage a cup rather than a bottle.
- Introduce 1 new food at a time.
- Energy density should exceed that of breast milk.
- Iron-containing foods (meat, iron-supplemented cereals) are required.
- Zinc intake should be encouraged with foods such as meat, dairy products, wheat, and rice.
- Phytate intake should be low to enhance mineral absorption.
- Breast milk should continue to 12 mo of age; formula or cow’s milk is then substituted.
- Give no more than 24 oz/day of cow’s milk.
- Fluids other than breast milk, formula, and water should be discouraged.
- Give no more than 4-6 oz/day of 100% fruit juice; no sugarsweetened beverages."
119
"Why should bedtime feeding bottles be discouraged?
A. It increases the risk for aspiration & GERD.
B. The practice is associated with dental carries.
C. Absorption is decreased at night.
D. Bedtime feeding is directly related to obesity."
B
"Nelson 21st p326
Bottle weaning should begin around 12-15 mo, and bedtime bottles should be discouraged because of the association with dental carries. Unless being used at mealtime, the sippy cup should only contain water to prevent caries. Sugar-sweetened beverages and 100% fruit juice should also be discouraged from being used in bottles in all infants at all times."
120
```
"A 17-year old male is 5 feet tall & weighs 50 kg. Based on his BMI, he is considered:
A. Underweight
B. Normal
C. Overweight
D. Obese"
```
B
"5 feet = 152.4cm = 1.52m
BMI = 21.64
Patient is 17 years old, so we can use adult criteria. BMI is normal
Nelson 21st p346
Adults with a BMI >=30 meet the criterion for obesity, and those with a BMI 25--30 fall in the overweight range.
Obesity and overweight are defined using BMI percentiles for children >=2 yr old and weight/length percentiles for infants <2 yr old. The criterion for obesity is BMI >=95th percentile and for overweight si BMI between 85th and 95th percentiles.
"
121
```
"A 4-year old female was seen at the OPD with the following features: skin-&-bone appearance, old man fades, prominent rib cage & shoulders, loose skin on the upper arms & thighs, & loose buttocks with absent muscle mass. What is the most probable diagnosis?
A. Marasmus
B. Kwashiorkor
C. Marasmus-kwashiorkor
D. Severe acute malnutrition"
```
A
"Nelson 21st p336
Severe acute malnutrition is defined as severe wasting and/or bilateral edema. Other terms are marasmus (severe wasting), kwashiorkor (characterized by edema), and marasmic-kwashiorkor (severe wasting and edema).
Severe wasting is most visible on the thighs, buttocks, and upper arms, as well as over the ribs and scapulae, where loss of fat and skeletal muscle is greatest. Wasting is preceded by failure to gain weight and then by weight loss. The skin loses turgor and becomes loose as subcutaneous tissues are broken down to provide energy. The face may retain a relatively normal appearance, but eventually becomes wasted and wizened. The eyes may be sunken from loss of retroorbital fat, and lacrimal and salivary glands may atrophy, leading to lack of tears and a dry mouth. Weakened abdominal muscles and gas from bacterial overgrowth of the upper gut may lead to a distended abdomen. Severely wasted children are often fretful and irritable."
122
```
"What is the most characteristic manifestation of niacin deficiency?
A. Dementia
B. Dermatitis
C. Diarrhea
D. Dehydration"
```
B
"Nelson 21st p368-369
After a long period of deficiency, the classic triad of dermatitis, diarrhea, and dementia appears. Dermatitis, the most characteristic manifestation of pellagra, can develop suddenly or insidiously and may be initiated by irritants, including intense sunlight."
123
```
"What is the most common cause of death among children with severe, uncorrected thiamine deficiency?
A. Wernicke encephalopathy
B. Cardiac failure
C. Megaloblastic anemia
D. Pseudotumor cerebri"
```
B
"Nelson 21st p365-366
Death from thiamine deficiency usually is secondary to cardiac involvement. The initial signs are cyanosis and dyspnea, but tachycardia, enlargement of the liver, loss of consciousness, and convulsions can develop rapidly."
124
```
"A 2-year old female was seen at the ER due to cramping periumbilical pain, nausea & vomiting. In the past month, he had repeated episodes of painless, bright red rectal bleeding & brick-colored stools. What is the most probable diagnosis?
A. Volvulus
B. Malrotation
C. Intussusception
D. Meckel diverticulum"
```
D
"Nelson 21st p1954-1955
The majority of symptomatic Meckel diverticula are lined by an ectopic mucosa, including an acid-secreting mucosa that causes intermittent painless rectal bleeding by ulceration of the adjacent normal ileal mucosa.
Less often, a Meckel diverticulum is associated with partial or complete bowel obstruction. The most common mechanism of obstruction occurs when the diverticulum acts as the lead point of an intussusception. The mean age of onset of obstruction is younger than that for patients presenting with bleeding."
125
"A 11-month old male had diarrhea for the past 2 days. At the ER, he drank eagerly, was restless & irritab le. He weighed 7 kg, had sunken eyeballs, dry lips, clear breath sounds & flat abdomen. The skin pinch goes back slowly. What is the most appropriate initial management?
A. ORS (volume per volume) after each loose bowel movement
B. 400-600 ml ORS to be given in the first 4 hours
C. 30 ml ORS per kg to be given in 1 hour
D. 70 ml ORS per kg to be given in 4 hours"
B
"""Moderate dehydration. Treat with IMCI plan B
Choice A is plan A
Choice B is plan B (although recommended is to give 450-600ml ORS)
Choice C and D is plan C
Nelsons 21st p429 Table 70.1. Clinical evaluation of dehydration
1. Mild dehydration (<5% in an infant, <3% in an older child) - normal or increased pulse, decreased urine output, thirsty, normal physical findings
2. Moderate dehydration (5-10% in an infant, 3-6% in an older child) - tachycardia, little or no urine output, irritable/lethargic, sunken eyes and fontanel, decreased tears, dry mucus membranes, mild delay in elasticicty (skin turgor), delayed capillary refill (>1.5 sec), cool and pale
3. Severe dehydration (>10% in an infant, >6% in an older child) - peripheral pulses either rapid and weak or absent, decreased BP, no urine output, very sunken eyes and fontanel, no tears, parched mucuous membranes, delayed elasticity (poor skin turgor), very delayed capillary refil (>3 sec), cool and mottled, depressed consciousness
IMCI 2014 Chart Booklet p19 Plan B: Treat some dehydration with ORS
In the clinic, give recommended amount of ORS over a 4 hour period
```
Determine the amount of ORS to give during the first 4hrs
Weight <6kg, up to 4mos: 200-450ml
Weight <10kg, 4-12mos: 450-800ml
Weight <12kg, 12mos-2yrs: 800-960ml
Weight 12-19kg, 2-5yrs: 960-1000ml """
```
126
Patient with blue sclerae, fractures
Osteogenesis imperfecta
"Nelson 21st p3736-3737
Osteogenesis imperfecta, the most common genetic cause of osteoporosis, is a generalized disorder of connective tissue.
The collagen structural mutations in OI cause the bones to be globally abnormal. The bone matrix contains abnormal type I collagen fibrils and relatively increased levels of types III and V collagen. Bone cells contribute to OI pathology, with abnormal osteoblast differentiation and increased numbers of active bone resorbing osteoclasts.
Classic OI was described with the triad of fragile bones, blue sclerae, and early deafness. "
127
"Care for down syndrome
a. Down syndrome curve should be used to evaluate weight & height
b. Eye exam at 4mos and before school entry due to risk of cataracts
c. Refrain from contact sports because 30% risk of hip dysplasia
d. Refer to cardio because 50% present with cardiac abnormalities"
B
"Nelson 21st p662 Table 98.8. Health supervision for children with Down syndrome
Congenital heart disease
- screened at birth and at young adulthood for acquired valve disease
- 50% risk of congenital heart disease
Strabismus, cataracts, nystagmus
- screened at birth or by 6 mo, check vision annually
- cataracts occur in 15%, refractive errors in 50%
Hearing impairment or losss
- screened at birth or by 3 mo with ABR, check hearing q6mo until 3yr, annually thereafter
- risk for congenital hearing loss 50-70%, risk for serous otitis media
Constipation
- screened at birth
- increased risk for Hirschsprung disease
Celiac disease
- screened at 2 yr or if with symptoms
- screen with IgA and tissue transglutaminase antibodies
Hematologic disease
- screened at birth and in adolescence or if with symptoms
- increased risk for neonatal polycythemia, (18%), leukemoid reaction, leukemia
Hypothyroidism
- screened at birth, repeat at 6-12mo then annually
- congenital (1%) and acquired (5%)
Growth and development
- screened at each visit, use Dosn syndrome growth curves
- discuss school placement options, proper diet to avoid obesity
Obstructive sleep apnea
- screened at 1 yr and each visit
- monitor for snoring, restless sleep
Atlantoaxial sublixation or instability (incidence 10-30%)
- screened at each visit by history and PE
- radiographs at 3-5 yr or when planning to participate in contact sports
- radiographs indicated wherever neurologic symptoms are present even if transient (neck pain, torticollis, gait disturbance, weakness)
- screen for high risk sports e.g. diving, swimming, contact sports
Gynecologic care
- adolescent girls; menstruation and contraception issues
Recurrent infection
- screened when present; check IgG and IgA leevels
Psychiatric, behavioral disorders
- screened at each visit
- depression, anxiety, OCD, schizophrenia (10-17%), autism (5-10%), early onset Alzheimer "
128
"What enzyme is involved in Pompes disease?
a. Galactose-1-Phosphate Uridyltransferase
b. Glucose-6 Phosphatase
c. Lactate Dehydrogenase
d. Acid Alpha Glucosidase"
D
"Nelson 21st p779 Table 105.1. Features of the disorders of carbohydrate metabolism
Ia - von Gierke - glucose-6-phosphatase deficiency
II - Pompe - acid alpha glucosidase deficiency
IIIa - Cori/Forbes - liver and muscle debrancher deficiency
IV - Andersen - branching enzyme deficiency
V - McArdle - myophosphorylase deficiency
VI - Hers - liver phosphorylase deficiency
Galactosemia with transferase deficiency - galactose-1-phosphate uridyltransferase deficiency
Lactate dehydrogenase deficiency - lactate dehydrogenase deficiency "
129
"Which of the following is not true regarding Turner's syndrome?
a. 45 XO results in pregnancy loss
b. Webbed neck, edema of hands
c. Maternal age is a risk factor"
C
"Nelson 21st p669
Turner syndrome is a condition characterized by complete or partial monosomy of the X chromosome and defined by a combination of phenotypic features. Half the patients with Turner syndrome have a 45, X chromosome complement. The other half exhibit moasicism and varied structural abnormalities of the X or Y chromosome. Maternal age is not a predisposing factor for children with 45, X.
Clinical findings in the newborns can include small size for gestational age, webbing of the neck, protruding ears, and lymphedema of the hands and feet. Congenital heart defects (40%) and structural renal anomalies (60%) are common. The most common heart defects are bicuspid aortic valves, coarctation of the aorta, aortic stenosis, and mitral valve prolapse. "
130
Most important diagnostics for genetic defects.
DNA sequencing
131
"In diagnosing metabolic disorders, which is false?
A. They are usually normal at birth and present later in life
B. The earlier the symptoms, the poorer the prognosis
C. Will respond to some form of therapy, hence need to be diagnosed early
D. Are mostly autosomal dominant."
D
Most metabolic disorders are autosomal recessive
132
"Female w/ short stature and webbed neck. She has associated coarctation of the aorta.
a) Turner syndrome
b) Noonan syndrome
c) Klinefelter syndrome
d) Nelson syndrome"
A
"Nelson 21st p669
Turner syndrome is a condition characterized by complete or partial monosomy of the X chromosome and defined by a combination of phenotypic features. Half the patients with Turner syndrome have a 45, X chromosome complement. The other half exhibit moasicism and varied structural abnormalities of the X or Y chromosome. Maternal age is not a predisposing factor for children with 45, X.
Clinical findings in the newborns can include small size for gestational age, webbing of the neck, protruding ears, and lymphedema of the hands and feet. Congenital heart defects (40%) and structural renal anomalies (60%) are common. The most common heart defects are bicuspid aortic valves, coarctation of the aorta, aortic stenosis, and mitral valve prolapse.
In contrast to Turner syndrome, Noonan syndrome affects both sexes and has a different pattern of congenital heart disease, typcically involving right sided lesions.
Klinefelter syndrome (47, XXY) is not associated with specific heart defects. Persons with Klinefelter syndrome are phenotypically male; this syndrome is the most common cause of hypogonadism and infertility in males and is the most common sex chromosome aneuploidy in humans. "
133
"Newborn child was noted to have prolonged jaundice, hypotonia, macroglossia. Diagnosis?
a. Congenital adrenal hyperplasia
b. G6PD
c. Congenital hypothyroidism
d. Galactosemia"
C
```
"Nelson p2917 Congenital hypothyroidism
Prolonged jaundice (indirect hyperbilirubinemia) may be present due to delayed maturation of hepatic glucuronide conjugation. Affected infants cry little, sleep much, have poor appetites, and are generally sluggish. Feeding difficulties, especially sluggishness, lack of interest, somnolence, and choking spells during nursing, may be present during the 1st mo of life. Respiratory difficulties, partly caused by macroglossia, include apneic episodes, noisy respirations, and nasal obstruction."
```
134
"Newborn child with facies but with high pitched cat like cry. Diagnosis?
a. Cri-du chat
b. Down syndrome
c. Edwards syndrome
d. Turner syndrome"
A
"Medline Plus
Cri-du-chat (cat's cry) syndrome, also known as 5p- (5p minus) syndrome, is a chromosomal condition that results when a piece of chromosome 5 is missing. Infants with this condition often have a high-pitched cry that sounds like that of a cat. The disorder is characterized by intellectual disability and delayed development, small head size (microcephaly), low birth weight, and weak muscle tone (hypotonia) in infancy. Affected individuals also have distinctive facial features, including widely set eyes (hypertelorism), low-set ears, a small jaw, and a rounded face. Some children with cri-du-chat syndrome are born with a heart defect."
135
"Different chromosomal make up in an individual with tissue that contains>/= 2 different cell lines derived from a single zygote and the result of mitotic non dysjunction?
a. Translation
b. Genetic mosaicism
c. Transcription
d. None of the above"
B
"Nelsons 21st p672
Mosaicism describes an individual or tissue that contains ≥2 different cell lines typically derived from a single zygote and the result of mitotic nondisjunction"
136
"Type of gene inheritance wherein the mother is a carrier, males are predominantly affected, may produce daughters that have 25% chance of becoming a carrier?
a. X- linked recessive
b. Y linked recessive
c. Autosomal dominant
d. Autosomal recessive"
A
"Nelsons 21st p645
In X-liked disorders, males are more commonly affected than females. Female carriers of these disorders are generally unaffected, or if affected, they are affected more mildly than males. In each pregnancy, female carriers have a 25% chance of having an affected son, a 25% chance of having a carrier daughter, and a 50% chance of having a child that does not inherit the mutated X-linked gene. Since affected males pass their X chromosome to all their daughters and their Y chromosome to all their sons, they have a 50% chance of having an unaffected son that does not carry the disease gene and a 50% chance of having a daughter who is a carrier."
137
```
"What features are common with Trisomy 13 and 18?
A. Rockerbottom feet
B. Overlapping digits
C. Liver/renal anomalies
D. Congenital heart disease"
```
D
"Trisomy 13, Patau syndrome
- Cleft lip often midline; flexed fingers with postaxial polydactyly; ocular hypotelorism, bulbous nose; low-set, malformed ears; microcephaly; cerebral malformation, especially holoprosencephaly; microphthalmia, cardiac malformations; scalp defects; hypoplastic or absent ribs; visceral and genital anomalies
- Early lethality in most cases, with a median survival of 12 days; ~80% die by 1 year; 10-year survival ~13%.
- Survivors have significant neurodevelopmental delay.
Trisomy 18, Edwards syndrome
- Low birthweight, closed fists with index finger overlapping the 3rd digit and the 5th digit overlapping the 4th, narrow hips with limited abduction, short sternum, rocker-bottom feet, microcephaly, prominent occiput, micrognathia, cardiac and renal malformations, intellectual disability
- ~88% of children die in the 1st year; 10-year survival ~10%.
- Survivors have significant neurodevelopmental delay."
138
"Case of Down syndrome. Prognosis in adulthood.
A. Increased spontaneous abortion in first trimester
B. Sterile
C. Aggressive and self-injurious behavior
D. Increased risk for solid tumors"
B
"Nelson 21st p661
The life expectancy of children with Down syndrome is reduced and is approximately 50-55 yr. Retrospective studies have shown premature aging and an increased risk of Alzheimer disease in adults with Down syndrome. Persons with Down syndrome have fewer than expected deaths caused by solid tumors and ischemic heart disease.
Leukemias accounted for 69% of all cancers in people with Down syndrome and 97% of all cancers in children with Down syndrome. There was decreased risk of solid tumors in all age groups with Down syndrome, including neuroblastomas and nephroblastomas in children and epithelial tumors in adults.
Most adults with Down syndrome are able to perform activities of daily living. However most have difficulty with complex financial, legal, or medical decisions, and a guardian may be appointed.
Nelson 21st p660
Common behavioral difficulties that occur in children with Down syndrome include inattentiveness, stubbornness, and a need for routine and sameness. Aggression and self-injurious behavior are less common in this population than other children with similar degrees of intellectual disability from other etiologies.
Nelson 21st p659
Most males with Down syndrome are sterile, but some females have been able to reproduce, with a 50% chance of having trisomy 21 pregnancies. "
139
```
"Case of Turner syndrome. Which PE finding will support diagnosis?
A. Murmur
B. Redundant nuchal skin folds
C. Breast development
D. None of the above"
```
B
"Nelsons 21st p669 Table 98.16. Signs associated with Turner syndrome
```
Short stature
Congenital lymphedema
Horseshoe kidneys
Patella dislocation
Increased carrying angle of elbow (cubitus valgus)
Madelung deformity (chondrodysplasia of distal radial epiphysis)
Congenital hip dislocation
Scoliosis
Widespread nipples
Shield chest
Redundant nuchal skin (in utero cystic hygroma)
Low posterior hairline
Coarctation of aorta
Bicuspid aortic valve
Cardiac conduction abnormalities
Hypoplastic left heart syndrome and other left-sided heart
abnormalities
Gonadal dysgenesis (infertility, primary amenorrhea)
Gonadoblastoma (increased risk if Y chromosome material is
present)
Learning disabilities (nonverbal perceptual motor and visuospatial
skills) (in 70%)
Developmental delay (in 10%)
Social awkwardness
Hypothyroidism (acquired in 15–30%)
Type 2 diabetes mellitus (insulin resistance)
Strabismus
Cataracts
Red-green color blindness (as in males)
Recurrent otitis media
Sensorineural hearing loss
Inflammatory bowel disease
Celiac disease (increased incidence)"
```
140
"Not true of Hirschprung disease
a. Common in preterm
b. Presents as bilious emesis or aspirates
c. Most dangerous complication is intestinal perforation
d. All are true"
A
"Nelson 21st p1961
Hirschsprung disease, or congenital aganglionic megacolon, is a developmental disorder (neurocristopathy) of the enteric nervous system, characterized by the absence of ganglion cells in the submucosal and myenteric plexus. It is the most common cause of lower intestinal obstruction in neonates, with an overall incidence of 1 in 5,000 live births. The male:female ratio for Hirschsprung disease is 4 : 1 for shortsegment disease and approximately 2 : 1 with total colonic aganglionosis. Prematurity is uncommon. Hirschsprung disease is usually diagnosed in the neonatal period secondary to a distended abdomen, failure to pass meconium, and/or bilious emesis or aspirates with feeding intolerance."
141
"True of signs and symptoms of PUD
a. In school age children and adolescents presents as epigastric pain & nausea
b. In infants and younger children presents as dyspepsia & abdominal fullness
c. Pain is sharp or burning
d. Epigastric pain is alleviated by food intake"
A
"Nelson 21st p1969-1970
The presenting symptoms of peptic ulcer disease vary with the age of the patient. Hematemesis or melena is reported in up to half of the patients with peptic ulcer disease. School-age children and adolescents more commonly present with epigastric pain and nausea, similar to the presentation generally seen in adults. Dyspepsia, epigastric abdominal pain, and fullness are also seen in older children. Infants and younger children usually present with feeding difficulty, vomiting, crying episodes, hematemesis, or melena. In the neonatal period, gastric perforation can be the initial presentation.
The classic symptom of peptic ulceration, epigastric pain alleviated by the ingestion of food, is present only in a minority of children. Many pediatric patients present with poorly localized abdominal pain, which may be periumbilical.
The pain is often described as dull or aching, rather than sharp or burning, as in adults. It can last from minutes to hours; patients have frequent exacerbations and remissions lasting from weeks to months. Nocturnal pain waking the child is common in older children. A history of typical ulcer pain with prompt relief after taking antacids is found in <33% of children."
142
"Infant with non bilious vomiting, hungry after vomiting. Noted mass to the right of umbilicus. What will you do?
A. Observe for presence of blood in stool
B. Clear the patient as high risk and proceed with surgery
C. Clear the patient as moderate risk and proceed with surgery
D. None of the above"
B
"Nelson 21st p1947-1948
Nonbilious vomiting is the initial symptom of pyloric stenosis. The vomiting may not be projectile initially, but is usually progressive, occuring immediately after feeding.
The diagnosis has traditionally been established by palpating the pyloric mass. The mass is firm, movable, approximately 2cm in length, olive-shaped, hard, best palpated from the left side, and located above and to the right of the umbilicus in the mid-epigastrium beneath the liver edge.
The surgical procedure of choice is pyloromyotomy. The surgical treatment of pyloric stenosis is curative, with an operative mortality of 0-0.5%"
143
"3 month old with bilious vomiting, non-distended abdomen, peristaltic movement can be noted . X ray showed double bowel gas with short segment of small intestine
a. Duodenal obstruction
b. Volvulus
c. Meckel diverticulum
d. Hirschsprung disease"
A
"Nelson 21st p1951
The hallmark of duodenal obstruction is bilious vomiting without abdominal distensions, which is often noted at the first day of life. Peristaltic waves may be visualized early in the disease process.
The diagnosis is suggested by the presence of a double bubble sign on a plain abdominal radiograph. The appearance is caused by a distended and gas filled stomach and proximal duodenum, which are invariably connected. Contrast studies are generally not necessary and may be associated with aspiration. "
144
"At what age is encopresis normal?
a. 3 years old
b. 4 years old
c. 5 years old
d. 6 years old"
A
Nelson 21st p 1959 - In functional constipation, daytime encopresis is common. Encopresis is defined as voluntary or involuntary passage of feces into appropriate places at least once a month for 3 consecutive months once a chronologic or developmental age of 4 yr have been reached.
145
```
"Gold standard to diagnose Hirschprung disease
A. Rectal biopsy
B. Manometry
C. Abdominal plain Xray
D. Barium enema"
```
A
"Nelson 21st p1963
Rectal suction biopsy is the gold standard for diagnosing Hirschsprung disease. The biopsy material should contain an adequate amount of submucosa to evaluate for the presence of ganglion cells. To avoid obtaining biopsies in the normal area of hypoganglionosis, which ranges from 3 to 17mm in length, the suction biopsy should be obtained no closer than 2cm above the dentate line.
Anorectal manometry evaluates the internal anal sphincter while a balloon is distended in the rectum. In patients with Hirschsprung disease, the internal anal sphincter fails to relax in response to rectal distension, and there is absence of the rectoanal inhibitor reflex.
An unprepared constrast enema is most likely to aid in the diagnosis of children older than 1mo of age because the proximal ganglionic segment might not be significatnly dilated in the first few wk of life.
Classic findings are based on the presence of an abrupt narrow transition zone between the normal dilated proximal colon and a smaller caliber obstructed distal aganglionic segment. "
146
"What is most commonly used to diagnosed GERD, but cannot distinguish mild or non-acidic reflux?
a) Upper GI series
b) Esophagogram
c) 24 hour pH monitoring
d) Manometry"
A
"Nelson 21st p1936-1937
For most typical GERD presentations, particularly in older children, a thorough history and physical examination suffice initially to reach the diagnosis.
Contrast (usually barium) radiographic study of the esophagus and upper GI tract is performed in children with vomiting and dysphagia to evaluate for achalasia, esophageal strictures, hiatal hernia, and gastric outlet or intestinal obstruction. It has poor sensitivity and specificity in the diagnosis of GERD as a result of its limited duration and inability to diferentiate physiologic GER from GERD. Furthhermore, contrast radiography neither accurately assesses mucosal inflamation nor correlates with the severity of GERD.
Extended esophageal pH monitoring of the distal esophagus provides a quantitative and sensitive documentation of acidic reflux episodes, the most important tpe of reflux episodes for pathologic reflux.
Endoscopy allows diagnosis of erosive esophagitis and complications such as strictures or Barrett esophagus.
Esophageal manometry is not useful in demonstrating gastroesophageal reflux but may be of use to evaluate transient LES relaxations and pressures.
The multichannel intraluminal impedance is a cumbersome test, but with potential applications both for diagnosing GERD and understanding esophageal functions. It is an important tool in those with respiratory symptoms, particularly for the determination of nonacid reflix.
Laryngotracheobronchoscopy evaluates for visible airway signs that are associated with extraesophageal GERD, such as posterior laryngeal inflammation and vocal cord nodules; it can permid diagnosis of silent aspiration.
Empirical antireflux therpy, using a time-limited trial of high dose PPI is a cost-effective strategy for diagnosis in adults, although not formally evaluated in older children. "
147
"Infant that is well in between episodes of pain, on abdominal exam noted to have a sausage shaped mass?
a. Pyloric stenosis
b. Intussusception
c. Meckels diverticulum
d. Abscess"
B
"Nelsons 21st p1947 Pyloric stenosis
The diagnosis has traditionally been established by palpating the pyloric mass. The mass is firm, movable, approximately 2cm in length, olive-shaped, hard, best palpated from the left side, and located above and to the right of the umbilicus in the mid-epigastrium beneath the liver edge.
Nelsons 21st p1966 Intussussception
The classic triad of pain, a palpable sausage-shaped abdominal mass, and bloody or currant-jelly stool is seen in <30% of patients with intussussception. The combination of paroxysmal pain, vomiting, and a palpable abdominal mass has a positive predictive value of >90%; the presence of rectal bleeding increases this to approximately 100%
Palpation of the abdomen usually reveals a slightly tender sausage-shaped mass, slightly ill-defined, which might increase in size and firmness during a paroxysm of pain and is most often in the right upper abbdomen, with its long axis cephalocaudal. If it is felt in the epigastrisum, the long axis is transverse. Approximately 30% of patients do not have a palpable mass.
Nelson 21st p1954-1955 Meckels diverticulum
The majority of symptomatic Meckel diverticula are lined by an ectomic mucosa, including an acid-secreting mucosa that causes intermittent painless rectal bleeding by ulceration of the adjacent normal ileal mucosa.
Less often, a Meckel diverticulum is associated with partial or complete bowel obstruction. The most common mechanism of obstruction occcurs when the diverticulum acts as the lead point of an intussussception. "
148
"What is the imaging of choice in a patient with painless rectal bleeding?
a. MAG28-diuretic
b. Technitium pertecnitate 99
c. Barium swallow
d. Ultrasound"
B
"Nelson 21st p1954-1955
Meckel diverticulum is the most common congenital GI anomaly, occuring in 2-3% ooof all infants.
Rule of 2s
- Meckel diverticulum are found in approximately 2% of the general population
- usually 2 feet proximal to the ileocecal valve
- approximately 2 inches in length
- can contain 2 types of ectopic tissue (pancreatic and gastric)
- present before 2 yrs old
- 2x more common in females
The most sensitive study is a Meckel radionuclide scan, which is performed after IV infusion of technetium-99m pertechnetate."
149
"6 year old child has history of encopresis during the day, moves bowel 1-2 times per day, has a habit of holding in stools. Diagnosis?
a. Stress induced encopresis
b. Functional constipation
c. Hirschsprung disease
d. Normal for age"
B
"Nelson 21st p1959. Table 358.5 Rome IV diagnostic criteria for defecatory disorders in children and adults
Functional constipation (developmental age >=4yr) - must include >=2 of the following >=1x/wk for >=1 mo with insufficient criteria to diagnose irritable bowel syndrome
- <=2 defecations in the toilet per week
- >=1 episode of fecal incontinence per week
- history of retentive posturinig or excessive volitional stool retention
- history of painful or hard bowel movements
- presence of a large fecal mass in the rectum
- history of large diameter stools that can obstruct the toilet
- after appropriate evaluation, symptoms cannot be fully explained by another medical condition
Nelson 21st p1959-1960
Encopresis is ddefined as voluntary or involuntary passage of feces into inappropriate places at least once a month for 3 consecutive months once a chronologic or developmental age of 4yr has been reached. Encopresis is not diagnosed when the behavior is exclusively the result of direct effects of a substance (e.g. laxatives) or a general medical condition (except through a mechanism involving constipation).
Nelson 21st p1962
Hirschsprung disease in older patients must be distinguished from other causes of abdominal distension and chronic constipation. The history often reveals constipation starting in infancy that has responded poorly to medical management. Failure to thrive is not uncommon. Fecal incontinence, fecal urgency, and stool-withdrawing behaviors are usually not common. Significant abdominal distension is unusual in non-Hirschsprung related constipation, as is emesis.
"
150
"Patient noted to have meconium ileus,removed by gastrograffin. Long term and close follow up is done due to?
a. Functinal constipation
b. Congenital aganglionic colon
c. Both of the above
d. None of the above"
B
