Answer to question

Answer 

a. A detailed developmental history should be taken.You  want to ask about family history although this is more difficult in this case since he was adopted.

b. Benign Rolandic epilepsy of childhood or simple partial seizures.

c. Electroencephalogram (EEG).

In this case, the EEG showed left-sided centrotemporal spikes. This appearance is typical for benign Rolandic epilepsy of childhood and can also be bilateral.

A sleep EEG often intensif|es the spike activity. Further investigations like neuro-imaging are not indicated

because of the diagnostic nature of the EEG and the absence of any neurological abnormalities on clinical

examination.

d. The best treatment is not to treat! The condition is

benign and because the seizures usually occur during

sleep or on waking, it is well tolerated. If the seizures

are interfering with schooling or quality of life, carbamazepine or valproate may be used.The age of onset

is between 3 and 13 years.

e. Prognosis is excellent. Seizures commonly stop at the

age of 12 years and the EEG normalizes several years

later. There is no increased risk of seizure development

in later life. This is a genetic disorder transmitted

as an autosomal dominant trait. Forty percent of close relatives have a history of febrile convulsions

or epilepsy.

ANSWER TO QUESTION # 05

Question#05

After iron therapy is initiated, how early can a response be detected?

ANSWER: 

2 to 5 days: Increase in reticulocyte count

7 to 10 days: Increase in hemoglobin level

For patients with mild iron-deficiency anemia, the hemoglobin level should be checked after several weeks of therapy. For patients with more severe anemia, it may be useful to check the hemoglobin and reticulocyte levels after several days to make certain that the hemoglobin has not declined to dangerous levels and that the reticulocyte response is beginning.

ANSWER TO QUESTION # 03

What is the Mentzer index?

MCV/RBC. This is one of the formulas used to distinguish the hypochromic, microcytic anemias of the thalassemia trait from iron deficiency. As a general rule, iron deficiency causes alterations in RBCs that tend to be variable, whereas thalassemia generally results in more uniformly smaller cells. In patients with the beta-thalassemia trait, the Mentzer index is usually <13; in patients with iron deficiency, it is usually >13.

ANSWER TO QUESTION # 02

Question#02

How is the RDW useful for distinguishing causes of microcytic anemia?

ANSWER:

The red blood cell distribution width (RDW) is a quantification of anisocytosis (variation in red-cell size). It is derived from the RBC size histogram that is measured by automated cell counters, and it is reported as a percentage. In children, normal values range from about 11.5% to 14.5% but can vary among instruments. Statistically, it is the coefficient of variation of red-cell volume distribution. When elevated in a patient with microcytosis, it suggests that iron deficiency is a more likely cause of anemia than the thalassemia trait. Children with the thalassemia trait tend to have values that overlap with normal RDW values. The combination of an RDW above the normal range with a free erythrocyte protoporphyrin level of >35 μg/dL is more sensitive and specific for iron-deficiency anemia.

Answer to Question # 01

Question#01

As iron becomes depleted from the body, what is the progression at which laboratory tests change?

ANSWER: Look at the figure

The left end of the line for each test indicates the point at which the result deviates from its baseline. As shown in Figure, in general, the depletion of marrow, liver, and spleen reserves (as represented by ferritin) occurs first. This is followed by a decrease in transport iron (as represented by transferrin saturation) and finally a fall in hemoglobin and MCV. The figure illustrates that the absence of anemia does not exclude the possibility of iron deficiency and that iron depletion is relatively advanced before anemia develops. Tests of soluble transferrin receptor have become of interest in patients with iron-deficiency anemia because the elevated levels are very sensitive indicators.

ANSWERS OF 'TO MATCH'

ANSWERS OF 'TO MATCH'

1. I. X-linked hypophosphataemic rickets

In this condition, a mutation in the PEX gene on the X chromosome

causes an isolated defect in phosphate reabsorption. This low rate of tubular reabsorption of phosphate results in hypophosphataemia, and therefore rickets develops. Typical radiological changes in rickets include cupping and fraying of the metaphyseal regions. Other features include delayed dentition. The normal 2 5-hydroxy vitamin D and P T H levels, together with a low phosphate level, provide the clue to the diagnosis.

2. F. Vitamin D deficiency rickets

This child has vitamin D deficiency leading to rickets. The low 25-hydroxy vitamin D level provides the clue to the diagnosis. This child requires a careful detailed history of diet and sun exposure. Rickets typically causes genu varum (bowed legs) in children under 2 years of age, and genu valgum (knock-knees) in children over 2 years of age.

3. G. Primary hypoparathyroidism

Low parathyroid hormone levels result in hyperphosphataemia and

hypocalcaemia. The seizures are likely to be secondary to hypocalcaemia. In this case the hypoparathyroidism is likely to be due to Di George syndrome (congenital abnormality of the third and fourth brachial arches leading to hypoparathyroidism, absent thymus, dysmorphic features and congenital cardiac disease).

4. L. Proximal renal tubular acidosis

Proximal (type 2) renal tubular acidosis is caused when there is reduced proximal tubular reabsorption of bicarbonate. Norm ally 85% of the filtered bicarbonate is reabsorbed in the proximal tubule. The distal tubule cannot fully compensate for this load, so considerable amounts of bicarbonate are lost in the urine, and the serum bicarbonate level falls, resulting in a metabolic acidosis. The urine can still be acidified because distal hydrogen ion secretion is normal. Thus the acidic urinary pH is the clue that this is proximal renal tubular acidosis. Patients may present with faltering growth or vomiting.

5. C. Distal renal tubular acidosis

Distal (type 1) renal tubular acidosis is caused when the distal nephron cannot secrete hydrogen ions, thus preventing the reabsorption of the final 15% of bicarbonate. This causes a metabolic acidosis. However, the urinary pH cannot be reduced below about 5.5-6.0, even in the presence of severe systemic acidosis. If there is systemic metabolic acidosis, the body would usually try to compensate by producing acidic urine. In this case the urine cannot be acidified, which provides the clue to the diagnosis

of distal renal tubular acidosis. Patients may present with urinary stones or nephrocalcinosis.

Answer to MCQS of the WEEK

Answer to MCQS of the WEEK

The child in the question likely has Kasabach-Merritt phenomenon, which is seen with large vascular anomalies (ie, Kaposiform hemangioendothelioma and tufted angioma). Platelet and RBC sequestration within the vascular tumor causes peripheral thrombocytopenia, coagulopathy, and microangiopathic hemolytic anemia. Treatment options include corticosteroids, α- interferon, and vincristine. Surgery frequently results in excessive bleeding.

Nevus simplex is a common minor vascular malformation seen on the glabella, eyelids, and nape of the neck in newborns; such lesions on the face are also called “angel or stork kiss,” while lesions on the back of the neck are called “stork bite.” 

Nevus flammeus, or port wine stain, is a large sharply demarcated pink to purple vascular malformation that can occur anywhere. Port wine stain involving the VI distribution of the trigeminal nerve should raise the suspicion of Sturge-Weber syndrome. 

PHACE(S) syndrome includes posterior fossa malformations, large facial hemangiomas, arterial abnormalities, coarctation of the aorta, eye abnormalities, and sternal defects. 

Infantile fibrosarcoma is a malignant congenital tumor that can be easily mistaken for an infantile hemangioma; a high index of suspicion is required to investigate further if a “hemangioma” is not resolving as expected.

Correct answer is 'a'

Pediatric Cases- Answer

Pediatric Cases


Answer: 

1. C. 

2. A

3. D

Congenital diaphragmatic hernia (CDH) occurs in 1 of every 2000-3000 live births and accounts for 8% of all major congenital anomalies. It occurs more frequently on the left side and can be difficult to differentiate from a congenital cystic adenomatoid malformation. Mortality after live birth is generally reported to range from 40 to 62%. Factors leading to an adverse outcome include associated anomalies, prenatal diagnosis, prematurity, low birth weight and early pneumothorax. 

The outcome in patients with a late presentation of CDH (approx 10% present after the newborn period) is very good, with low or no mortality. 

The main risks associated with CDH affecting morbidity and mortality is of pulmonary hypertension and pulmonary hypoplasia. Treatment is aimed at mechanical ventilation avoiding high peak inspiratory pressures. 

High frequency ventilation is sometimes used to avoid this and in extreme circumstances ECMO is required. Fetal surgery has not as yet been shown to improve survival. A delayed surgical approach that enables preoperative stabilization is important in reducing morbidity and mortality.

Answers: Immunodeficiency

The answers are 103-c, 104-f, 105-b, 106-g, 107-e.

The bulk of immunodeficiencies can be ruled out with little cost. The NBT or other respiratory burst

assay will help identify phagocytic-cell defects such as chronic granulomatous disease (resulting in the liver abscess in the child in question). A newer test for CGD is the dihydrorhodamine (DHR) flow cytometry study.

Wiskott-Aldrich syndrome must be considered in a patient with severe eczema and unusual infections, and is a strong possibility with this history if the platelet count is low (but is unlikely if the platelet count is normal). Other findings include eosinophilia and elevated IgE.

B-cell defects are likely to result in low immunoglobulin A, G, and M levels and result in multiple infections such as that described in the 3-year-old with otitis media and sinusitis.

An intradermal skin test using Candida albicans will result in no response in the patient with T-cell deficiencies, such as in the question of the dysmorphic child who possibly has DiGeorge syndrome. Asplenia results in Howell-Jolly bodies and also an increased risk for encapsulated organisms such as pneumococcus or meningococcus; a CBC with a peripheral smear can rule out this disease. Should any of these tests prove to be positive, more extensive, invasive, and expensive testing can be undertaken.

ANSWERS of MCQS dated 23/3/2020

MCQ#01
c
Indomethacin is contraindicated in patients with bleeding disorder, NEC, oliguria (less than 1 mL/kg/hr), elevated creatinine level (more than 1.8 mg/dL), and thrombocytopenia (less than 50,000/mm3).


MCQ#02
c


MCQ#03
a



MCQ#04
b
HIV DNA PCR.


MCQ#05
b

ANSWERS to PEDIATRIC GASTROENTEROLOGY MCQS Pulished on 13/9/2019

MCQ#1

Correct Answer: B

EXPLANATION:

The typical patient with CVS presents with recurrent, stereotypic episodes of frequent emesis every 2 to 4 weeks that begin suddenly and last 24 to 48 hours. There is a return to periods of baseline health between episodes. The vomiting cannot be attributed to another disorder. A thorough history and physical examination is important, as specific conditions and symptoms suggest that the diagnosis is not consistent with CVS. These conditions are (1) presentation at less than 2 years of age; (2) bilious vomiting, abdominal tenderness, and/or severe abdominal pain; (3) attacks associated with intercurrent illness, fasting, and/or a high-protein meal; (4) abnormalities on neurologic examination; or (5) progressively worsening episodes or a conversion to a continuous or chronic pattern.

The patient in scenario A had nonbilious emesis that occurs after a period of fasting or a high-protein meal. These symptoms suggest a partial urea cycle enzyme deficiency, which can occur after such situations. The patient in scenario

C has bilious emesis and abdominal pain. Although CVS can present with similar symptoms, it is important to rule out a surgical condition, such as volvulus or malrotation, as well as pancreatitis. In addition, this child does not have a 3-week cycle with a return to baseline. The patient in scenario D has neurologic symptoms, which is concerning for increased intracranial pressure or a metabolic disorder. The patient in scenario E has symptoms consistent with pyloric stenosis.

MCQ#2

Correct Answer: B

EXPLANATION:

A child who presents with acute vomiting should first be evaluated for the degree of dehydration; if severe (5%-10%) dehydration is present, immediate intravenous access and fluids should be provided. However, this child is not severely dehydrated. In addition, there is a high suspicion for CVS. If CVS is suspected in a child without other “alarm” symptoms such as severe dehydration, shock, abnormal neurologic examination, or severe abdominal pain, it is appropriate to check labs prior to initiation of intravenous fluids. Serum electrolytes can help assess for the possibility of a metabolic, renal, or endocrine disorder. They can also demonstrate abnormalities such as hypoglycemia or elevated BUN/creatinine that would assist in deciding on appropriate fluid resuscitation.

Abdominal radiograph may be useful in patients with suspected intestinal obstruction; head CT may be useful in patients with focal neurologic findings on examination suggesting increased intracranial pressure as a cause for vomiting. Intravenous proton pump inhibitors are unlikely to be helpful in the immediate treatment of acute recurrent nonbloody, nonbilious vomiting.

MCQ#3

Correct Answer: A

EXPLANATION:

This patient has chronic recurrent vomiting, with at least 3 episodes of mild but frequent emesis over a 3-month period. Among the most frequent causes of vomiting in the schoolage child and adolescent are gastro-esophageal reflux disease, which may occur after meals and is not typically associated with weight loss. In a child with chronic vomiting without alarm symptoms or other concerning examination findings, the most likely diagnosis is an acid-peptic disorder such as gastroesophageal reflux disease or gastritis that should be initially treated with empiric 2- to 4-week trial of H 2 -receptor antagonist or proton pump inhibitor for acid suppression. If there is no symptomatic improvement after a time limited trial of these medications, it may then be appropriate to proceed with laboratory testing, imaging, or consultation with pediatric gastroenterology. Odansetron may be useful for symptomatic improvement in the setting of acute, rather than chronic, vomiting.

MCQ#4

Correct Answer: E

EXPLANATION:

This patient most likely has chronic nonspecific diarrhea of childhood, also called toddler’s diarrhea. She is well grown, has no other symptoms other than diarrhea, and has significant intake of apple juice daily, which has a high fructose-to-glucose ratio and is often implicated in toddler’s diarrhea. Toddler’s diarrhea is thought to be caused by mild carbohydrate malabsorption and hypermotility; a reasonable first step in treatment of suspected toddler’s diarrhea is elimination of juice from the diet with reevaluation.

Determination of stool electrolytes and osmolality can be helpful in distinguishing between osmotic and secretory diarrhea. Stool pH and reducing substances may help in screening for malabsorptive causes of diarrhea. Stool cultures may help identify infectious causes of chronic diarrhea such as protozoal or bacterial infection. However, in an otherwise healthy, well-grown child who has a history consistent with toddler’s diarrhea, empiric elimination of juice from the diet is a more reasonable first step in management rather than pursuing laboratory evaluation for less likely causes. Although lactose intolerance/lactase deficiency may be another cause of chronic diarrhea in childhood, excessive juice intake is more likely and should be pursued as a cause prior to trying a lactose-free diet.

MCQ#5

Correct Answer: D

EXPLANATION:

It is important to distinguish between the common problem of functional constipation and less common organic causes of constipation, including Hirschsprung disease. A diagnosis of Hirschsprung disease is suggested by small-caliber soft stool, episodes of explosive soft stool preceded by a lack of stool passage and abdominal distension, and history of delayed meconium passage in the first few days of life. If Hirschsprung disease is suspected, suction rectal biopsy should be considered as the next step in evaluation.

Children with functional constipation may have fecal incontinence (encopresis) with soiling of the undergarments due to seepage of liquid stool around impacted rectal fecal mass. Urinary tract infections may also occur due to partial

urethral obstruction by pressure from fecal mass in the colon or by ascending infection from soiled undergarments. Many children with functional constipation have a history of large caliber stools and stool retentive posturing in an attempt to avoid uncomfortable passage of large and/or hard stools.

THANK FOR YOUR ANSWERS

ANSWERS of MCQs PUBLISHED on 12/9/2019 with the title 'PEDIATRIC MCQS: Orthopedic'

ANSWERS of MCQs PUBLISHED on 12/9/2019 with the title 'PEDIATRIC MCQS: Orthopedic'  

MCQ#01

Answer C

Metatarsus adductus is one of the most common causes of an intoeing gait and is typically diagnosed in infancy. It is fairly easily distinguished from clubfoot due to flexibility of the foot, as opposed to clubfoot, which cause a rigid deviation of the foot. DDH is found in 10% of patients with metatarsus adductus and diligence must be paid to evaluating for this abnormality. While clavicular fracture can be found in infancy and has an association with DDH, there is no known association with metatarsus adductus. Pes planus, or flat foot, is ubiquitous in infants, as arch development has not yet occurred. Myelomeningocele, while much rarer in the era of folic acid use prior to pregnancy, is still diagnosed peripartum, but is not related to the presence of metatarsus adductus.

MCQ#02

Answer B

Femoral ante-version is the most common cause of an in-toeing gait in children over the age of 4. It is due to excessive internal rotation at the level of the hip, causing the entire leg to be inwardly deviated. In internal tibial torsion, the internal deviation is at the level of the tibia and therefore the patella are noted to be straight on examination, as opposed to femoral anteversion, where they are internally rotated. Metatarsus adductus is a disorder of infants, causing inward deviation of the forefoot. Slipped capital femoral epiphysis is a cause of an out-toeing gait due to medial displacement of the cap of the femur. While fractures can cause a limp, they do not specifically lead to an intoeing gait.

MCQ#03

Answer C

Babies are born with the maximum amount of varus deformity of the knees. This angular deformity of the knee should improve over time and typically resolves by age 2. Patients whose genu varum persists after this age should be evaluated for other, less typical, cause of varus deformity, including rickets, skeletal dysplasias, or other metabolic bone diseases.

MCQ#04

Answer B

Genu valgus is normal between the ages of 3 and 8 and maximal between the ages of 4 and 6. As long as this patient’s valgus improves over time, there is no further intervention required and the parent can be reassured. If the patient has persistent valgus deformity into adolescence, then a workup for previous fracture or metabolic bone disease may be indicated. Neither orthotics nor physical therapy has been shown to be effective in expediting the resolution of genu valgus.

MCQ#05

Answer C

Developmental dysplasia of the hip is defined as an abnormal relationship between the femur and the acetabulum. Intrauterine conditions that place a strain on the hip and leg, such as breech presentation or oligohydramnios, predispose the fetus to DDH. Torticollis, or wryneck, can also be caused by this type of mechanism and children born with torticollis have a 14% to 20% incidence of DDH.

Patients with metatarsus adductus also have an increased risk of DDH due to the same mechanism; however, this mechanism is not associated with other causes of intoeing, such as tibial torsion or femoral ante-version. Teratologic hip

dislocations can be due to conditions such as arthrogryposis. DDH is not specifically associated with congenital scoliosis, which is due to mal-development of vertebral bodies, or branchial cleft disorders.

ANSWERS of PEDIATRIC RHEUMATOLOGY MCQS Pulished on 10/9/2019

Answer 1. D

Any child with fever and an inflamed, swollen joint should be considered to have septic arthritis until proven otherwise. It is imperative to obtain synovial fluid for gram stain and culture. In general, for this situation, a synovial fluid culture is most likely to achieve the highest rate of identification of the organism, although this rate is still not 100%. Obtaining blood cultures may increase the yield of identifying the organism, but in the absence of osteomyelitis, bone culture will not be useful. While MRI or ultrasound can document effusion and inflammation and help to determine if there is concomitant osteomyelitis, neither test will yield identification of the actual organism.

Answer 2. C

Patients with Kawasaki disease can develop arthritis as part of their disease. The arthritis, which affects approximately 1/3 of patients with KD, usually occurs in the acute phase of the

illness and resolves within 3 weeks to 3 months. The presence of arthritis is not considered a treatment failure and therefore repeat dosing of IVIg is not required. Intermittent NSAID therapy may help joint pain, but chronic treatment is not necessary, with either NSAIDs or aspirin. Intra-articular corticosteroid injections can be helpful in patients with chronic arthritis, but are not indicated in this context.

Answer 3. B

This patient has a history of recurrent oral ulceration and at least 1 episode of genital ulceration. She has a rash and arthritis on exam. All of these findings are consistent with a diagnosis of Behcet Disease (BD). The diagnosis requires recurrent oral ulceration (see Figure 203-2 ) and at least 2 of the following:

recurrent genital ulceration, eye disease, skin lesions, and a positive pathergy test. This patient does not fulfill criteria, as she has had only one episode of genital ulceration. A positive pathergy test would provide sufficient evidence to make a diagnosis of BD. The pustular rash is nonspecific and biopsy would not be definitive. Patients with BD can have CNS involvement, including encephalitis or aseptic meningitis, but the MRI/MRA findings would be nonspecific. A C-reactive protein can be elevated in any systemic inflammation and is not helpful for making a specific diagnosis. This patient, although she has arthritis, is unlikely to have rheumatoid arthritis given her presentation. A positive RF would not be helpful in this case, given the low pretest probability for RA.

Answer 4. A

Treatment of SLE is a balance of benefits of medications against the known toxicities of the drugs used. While prednisone is the mainstay of treatment for significant organ involvement, the side effects of prolonged use, including risk of avascular necrosis, osteoporosis, and growth delay, are significant. Use of prednisone should be minimized, particularly in patients

with mild disease, and weaned as fast as is safe for the patient. Steroid-sparing medications, such as cyclophosphamide and azathioprine, have their own toxicities, including bone marrow suppression and risk of malignancy, and are reserved for serious organ involvement. Low-dose aspirin has been used in patients with anti-phospholipid antibodies to decrease clotting risk, but its actual benefit is uncertain.

Hydroxychloroquine, on the other hand, has been shown to decrease the number of lupus flares and increase the time between flares. It also lowers serum lipid levels, a benefit in patients with renal disease and in chronic corticosteroid use.

Answer 5. E

Systemic sclerosis is very rare in children, with less than 5% of patients developing their disease before the age of 16 years. In children, the most common presenting feature is Raynaud phenomenon, followed by proximal skin induration that develops later. The figure below shows classic Raynaud phenomenon in a 4-year-old girl. Note the ischemic changes (blanching phase) of the second and fourth fingers and the cyanosis (bluish phase) of the fifth finger. Internal organ involvement can be significant, but is not typically an early finding. Dysfunction of the esophagus is the most common gastrointestinal manifestation, seen symptomatically as dysphagia and reflux. Pulmonary involvement can include interstitial fibrosis, pleuritis, or pulmonary hypertension, which can lead to right-sided heart failure.

CORRECT ANSWERS OF PEDIATRIC MCQS PUBLISHED ON 9/9/2019

MCQ#01 

Correct Answer is D

Explanation:

The majority of urticaria and angioedema are mast cell mediated conditions resulting in the release of histamine, leukotrienes, and other mast cell mediators into the superficial dermis (urticaria) and deep dermis (angioedema). In children, common viral or bacterial infections account for 80% of acute urticaria. Treatment of acute urticaria and angioedema should focus on identifying and discontinuing any underlying triggering process and symptom suppression until resolution of the acute episode.

Symptom suppression can often be achieved with administration of H1 antihistamine receptor antagonists. It is most efficient to maximize the H1-receptor antagonist therapy prior to adding additional medications. The use of first generation H1-receptor antagonists, such as diphenhydramine, is limited by their sedating and anticholinergic side effects.

Cetirizine, a second-generation H1-receptor antagonist, is preferable for controlling urticarial symptoms that persist for more than a few days.

H2-receptor antagonists (ranitidine) and leukotriene inhibitors (montelukast) are useful adjunctive medications.

Short courses of oral steroids (prednisone) can be used to control severe urticaria that is refractory to high dose antihistamines, but should not be prior to a trial of antihistamine therapy.

MCQ#02

Correct Answer is C

Explanation:

Food allergies have a strong genetic component. Siblings of a peanut allergic child are 6 times more likely to develop a peanut allergy. 64% of monozygotic twins share a peanut allergy compared to 3% of dizygotic twins.

MCQ#03

Correct Answer is D

Explanation:

Food allergies can be grouped into 2 general categories: IgE mediated and non-IgE-mediated. IgE-mediated reactions are typically of rapid onset with clinical symptoms usually developing within minutes to a few hours of ingestion of the offending food. These reactions are due to release of mast cell granules and present with the typical characteristics of type I hypersensitivity reactions (flushing, urticaria, and/or angioedema).

The diagnostic workup for a suspected IgE-medicated food allergy includes a skin prick test and/or measurement of serum food-specific IgE antibodies. Both of these tests have sensitivity estimated to be >85%. Specificity, however, is <40%, since both tests measure allergic sensitization and may not correlate with clinical allergy. Clinical allergy can only be assessed by an oral food challenge. For some common food allergens, studies have suggested cut-off values for serum food-specific IgE antibodies that can predict the likelihood of developing systemic allergic reactions in a particular patient.

Intradermal skin testing requires a small amount of the allergen solution is injected into the skin. An intradermal skin test may be done when a substance does not cause a reaction in the skin prick test, but is still a suspected allergen for a given patient. The intradermal test is more sensitive than the skin prick test, but also has more false-positive results. It is not used

for the diagnosis of IgE-mediated food allergy, but rather for the evaluation of seasonal and perennial AR.

The atopy patch test is used to elicit cellular mediated hypersensitivity reactions in sensitized subjects. Patient skin is exposed to the allergen usually for 48 hours and reactions are usually interpreted at 72 hours. Patch testing is used for the assessment of contact allergen sensitization, but is only recently being evaluated for cellular-mediated food hypersensitivity disorders. While patch testing has shown promise for the diagnosis of non-IgE mediated food allergy, there are currently no standardized reagents, application methods, or guidelines for interpretation.

MCQ#04

Correct Answer is A

Explanation:

Current management of food allergies relies on the careful elimination of the offending food from the diet, including instruction on reading of labels and often requires education of the parents by a dietician. Institution of therapeutic measures to stall the development of severe reactions in the case of an accidental food exposure is also necessary. All patients with a history of a systemic reaction to food should be prescribed injectable epinephrine and instructed on its use. The drug should be employed quickly in the case of an impending anaphylactic reaction. Daily use, however, is not indicated.

Milder food allergic reactions, those involving the skin or the gastrointestinal system exclusively, can be treated with oral antihistamines. Oral antihistamines should be used for

symptoms only and daily use is not indicated.

The indication of glucocorticoids for the treatment of acute food allergic reactions is controversial. There is no consistent evidence that glucocorticoids prevent the development of late-phase reactions and are not routinely indicated. 

The use of oral immunotherapy for desensitization to food allergens is currently an area of investigation. The safety and efficacy of this approach has not been established in infants

and children.

MCQ#05

Correct Answer is E

Explanation:

Drug reactions can be classified into immunologic and nonimmunologic etiologies. The majority (75–80%) of adverse drug reactions are predictable, nonimmunologic effects, including: reactions due to overdose, toxicity, pharmacologic side effects, indirect side effects, and drug-drug interactions. The remaining 20–25% of adverse drug events are due to unpredictable effects, which may or may not be immune-mediated.Immune-mediated reactions constituting true drug hypersensitivity account for 5–10% of all drug reactions.

Drug hypersensitivity reactions commonly manifest with dermatologic symptoms. Typically an erythematous, maculopapular rash develops within 2 weeks of drug initiation, originating on the trunk with eventual spread to the limbs.

Pruritis and low-grade fever may accompany the drug eruption. Nephrotoxicity and ototoxicity are known dose-dependent side of effects of vancomycin and gentamicin respectively. Nausea and vomiting is a common adverse drug reaction to certain antibiotics, but nausea and vomiting do not represent an immunologic drug reaction.

Infectious mononucleosis is most commonly secondary to infection with the Epstein–Barr virus. A rash is a relatively common adverse effect of amoxicillin treatment in patients with acute Epstein–Barr virus. It is a non-immunologically mediated reaction. Amoxicillin is not indicated for the treatment of viral infections including the Epstein–Barr virus.

ANSWERS OF MCQS PUBLISHED ON 3/9/2019

ANSWERS

MCQ#01

B

IgA deficiency is the most common primary immunodeficiency, occurring in approximately 1 in 600 people. While most people with selective IgA deficiency are asymptomatic, some develop recurrent infections at mucosal barriers, such as the respiratory and gastrointestinal tracts.

Some patients with IgA deficiency develop anti-IgA antibodies and are at risk for anaphylaxis when they receive any blood product containing IgA, the most common of which is pooled intravenous immunoglobulin.

MCQ#02

C

Given the chronic recurrent respiratory tract infections in an otherwise healthy child with arthritis, this patient is likely to have IgA deficiency. The relationship of autoimmune disease and IgA deficiency has been well described, particularly with juvenile idiopathic arthritis and celiac disease. There is no treatment for IgA deficiency and treatment of the arthritis will not improve IgA production. Given the destructive nature of untreated JIA, the medication cannot be stopped. More aggressive treatment for arthritis, such as with methotrexate, steroids, or biologic agents will lead to further immunosuppression and the patient will need to be monitored for infection and treated aggressively when present.

MCQ#03

A

Disorders of the alternative complement pathway and terminal complement deficiencies have all been associated with increased risk of pyogenic infections, particularly Neisseria. Terminal complement component deficiencies, such as C5

deficiency lead to infection by affecting the ability to make a membrane attack complex and thereby lyse the bacteria. Disorders of the classical pathway can lead to infection, but are more commonly associated with autoimmune disease, particularly systemic lupus erythematosus.

MCQ#04

A

The genetic defect in AT, the ATM gene, leads to impairment of DNA repair. Patients with AT are thus highly sensitive to ionizing radiation, and are at significantly increased risk for developing malignancies, particularly of lymphoid origin. Exposure of patients to ionizing radiation or radiomimetic agents should be strictly limited, including standard radiography.

MCQ#05

D

Disorders of B-cell development result in maturation arrest of B cells and lack of mature B cells in the peripheral circulation. This condition results in agammaglobulinemia. The affected child depends on maternal immunoglobulin for humoral immune system protection; however, once maternal immunoglobulin decays (typically by 6 months of age), the child has no protection against infections with encapsulated bacteria or viruses, such as enterovirus, leading to chronic recurrent infection.