MCQs In Pediatric Infectious Diseases -Part IV

A 10-year-old girl has had a rash for 4 days without other symptoms. She is taking no

medications. Physical examination reveals erythematous cheeks (Item Q1A) and a lacy,

reticulated erythema involving the extremities .

Of the following, the MOST likely diagnosis is

A. erythema infectiosum

B. phototoxic reaction

C. polymorphous light eruption

D. scarlet fever

E. systemic lupus erythematosus

MCQs In Pediatric Nephrology -Part IV

Q 1:

An 18-month-old girl is brought to the hospital with a history of 6 days of bloody diarrhea. She has been drinking well but has not been wetting her diaper. She has been irritable. On physical examination, she has periorbital edema. She appears pale and is tachycardic. Her CBC shows a hemoglobin of 6 g/dL and a

platelet count of 100,000/mm3. Her blood urea nitrogen (BUN) is 50 mg/dL and creatinine is 5.5 mg/dL. Her urinalysis shows gross hematuria. Which of the following is the most likely causative organism for her clinical problem?

(A) E. coli 0157:H7

(B) group A Streptococci

(C) group B Streptococci (GBS)

(D) S. aureus

(E) the cause of this illness is not known

MCQs In Neonatology - Part IV

Q 1:

A5-week-old bottle-fed boy presents with persistent and worsening projectile vomiting, poor weight gain, and hypochloremic metabolic alkalosis. Of the following diagnostic modalities, which would most likely reveal the diagnosis?

(A) ultrasound of abdomen

(B) barium enema

(C) evaluation of stool for ova and parasites

(D) testing well water for presence of nitrites

(E) serum thyroxine

Answer:

(A)

The case presented is classic of pyloric stenosis. This results from hypertrophy and hyperplasia of smooth muscle in the stomach, causing a narrowed, even, obstructed outlet.

Persistent projectile vomiting causes ongoing losses of calories and electrolytes, resulting in growth failure and hypochloremic metabolic alkalosis. Hyponatremia and hypokalemia may also be associated. Often, the diagnosis can be made by physical examination alone. However, if an olive-shaped mass is not palpated, an
abdominal ultrasound may confirm the diagnosis.

MCQs In Neonatal Hematology

Q 1:

Aweek-old infant presents blood in his stools. He was born at home, with the father assisting in the delivery; no physician or midwife was present. He has been breast-fed and has been nursing well. On examination, you also note some blood in his nose. He is not jaundiced; a rectal examination and guaic test of the stool

confirms that blood is present. His examination is otherwise normal. He is on no medications.

Which of the following is the most likely diagnosis?

(A) child abuse

(B) vitamin K deficiency

(C) breast milk allergy

(D) sepsis

(E) liver disease

Answer:

(B)

Neonates are routinely given intramuscular vitamin K at the time of birth. This is done to prevent the transient deficiency of vitamin- K-dependent factors, which occurs because of absence of bacterial intestinal flora which synthesize vitamin K. Hemorrhagic disease in the newborn because of vitamin K deficiency may
result in GI, nasal, subgaleal, and intracranial bleeding, or bleeding after circumcision. The prothrombin time (PT), partial thromboplastin time (PTT), and bleeding time are prolonged.

These all correct after administration of vitamin K. Child abuse should always be considered with unusual bleeding, but the history reveals the etiology in this case. Babies are more likely to be allergic to formula than breast milk; however, it occurs rarely and may present with bloody stools. It does not, however, cause
epistaxis.

 Neonatal sepsis may result in disseminated intravascular coagulation and bleeding; the infant is usually ill appearing, with associated acidosis or shock. Liver disease may cause factor deficiencies and should be excluded if there is no response to vitamin K.

MCQs In Pediatric Hematology

Q 1 -2 : The 2 questions are related

Q1:

A15-month-old African American male, who is otherwise healthy, is found to have a hemoglobin level

of 8 g/dL on routine screening. The mean corpuscular volume (MCV) is decreased. His lead screen is within normal limits. You obtain a diet history, which reveals that he drinks about 30–40 oz of whole cow’s milk a day. He eats no meat and some fruits and vegetables.  Which of the following is the most likely cause?

(A) sickle cell anemia

(B) thalassemia major

(C) lead poisoning

(D) iron-deficiency anemia

(E) anemia of chronic disease

Q 2:

The most effective next step in management would be to obtain which of the following?

(A) iron studies—serum iron, total iron binding capacity, ferritin

(B) reticulocyte count

(C) hemoglobin electrophoresis

(D) a repeat hemoglobin in 1 month after treatment with folic acid

(E) a repeat hemoglobin in 1 month after treatment with iron

Answer :

Q 1:

(D)

 Iron deficiency is the most common cause of microcytic anemia. In children it is often related to excessive consumption of cow’s milk, which is low in iron content, and inadequate consumption of iron-rich foods. Allergy to cow’s milk may also cause occult GI blood losses.

 In thalassemia major, there is usually physical evidence of chronic anemia with signs of bone marrow expansion (frontal bossing) and severe anemia often requiring transfusions.

Lead poisoning may cause microcytic anemias; it may also be associated with iron-deficiency anemia, which enhances lead absorption and, therefore, should always be excluded. Anemia of chronic disease (renal disease) may be microcytic or normocytic and should be excluded by history and examination.

Q 2:

 (E)

If iron deficiency is strongly suspected, it is reasonable to treat empirically with 3–6 mg/kg/day of elemental iron. An increase in hemoglobin of 1 g/dL within 2–4 weeks confirms the diagnosis.

If laboratory confirmation is necessary because the child is at low risk for iron deficiency, confirmatory iron studies may be obtained. The serum iron is low, the total iron binding capacity high, and the ferritin is low

 A reticulocyte count is helpful in hemolytic anemias where it is elevated.

Bone marrow aspirate in iron deficiency is necessary if bone marrow infiltration is suspected (leukemia), but is overinvasive in this situation. Hemoglobin electrophoresis may be done if thalassemia or sickle cell anemia is likely

MCQs Pediatric Otolaryngology

Q1:

Otitis media occurring during the first 4  weeks of life deserves special consideration, because the bacteria responsible for infections during this time may be different from those that affect older infants and children. Which of the following organisms is the most likely to cause otitis media in these infants?

(A) Chlamydia trachomatis

(B) E. coli

(C) Neisseria gonorrhoeae

(D) Treponema pallidum

(E) Toxoplasma gondii

Answer:

(B)

C. trachomatis is considered an unusual cause of otitis media at any age. N. gonorrhoeae causes conjunctivitis in the newborn. Syphilis and toxoplasmosis cause congenital infections. E. coli is one of the neonatal pathogens that also causes otitis media in neonates. The symptoms of otitis media in newborns are often similar to those of sepsis; they are subtle and nonspecific and may include poor feeding, lethargy,
vomiting, or diarrhea. Once the diagnosis is established, the initial therapy should be similar to that for neonatal sepsis, such as parenteral ampicillin and cefotaxime. Under ideal circumstances, the results of cultures obtained by tympanocentesis may then allow further treatment with a more specific antibiotic of low
toxicity. Older infants may respond well to oral therapy but require frequent observation.

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Q 2:

A 6-month-old infant is diagnosed with her first episode of otitis media. She does not have any allergies to medications. Which of the following medications would be the recommended initial therapy for this infant?

(A) amoxicillin
(B) amoxicillin-clavulanic acid
(C) cephalexin
(D) ceftriaxone
(E) erythromycin

Answer

(A) H. influenzae, S. pneumoniae, and Moraxella catarrhalis are the most common bacterial pathogens in otitis media of children. Amoxicillin is still the initial drug to use in uncomplicated otitis media because of its good coverage, except for beta-lactamase-positive organisms, and its excellent safety profile. The other drugs (except for erythromycin) are acceptable second-line medications.


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Q 3:

A 4-year-old previously healthy but unimmunized boy presents with sudden onset of high fever, inspiratory stridor, and refusal to drink. Of the following causes of inspiratory stridor, which best fits this clinical scenario?

(A) epiglottitis
(B) vascular ring
(C) croup
(D) foreign body aspiration
(E) laryngeal tumor


Answer:

(A)

Croup and epiglottitis have similar presentations but need to be distinguished immediately. Croup usually results from a viral infection of the larynx and epiglottitis from a bacterial (H. influenzae type B) infection of the epiglottis. Children with epiglottitis tend to be toxic in appearance. Croup involves the airway,
and epiglottitis involves the airway and the digestive tract. Children with croup usually will swallow and drink. Children with epiglottitis most often will refuse to drink and may even drool as a result of their refusal to swallow saliva. Patients with foreign bodies in their upper airways do not typically have fever.
Patients with vascular rings and laryngeal tumors have more gradual onset of symptoms.


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Q 4:

A 10-year-old boy is brought in with a chief complaint of multiple colds. On further questioning, you elicit a history of chronic, clear nasal discharge with no seasonal variation. Other symptoms include sneezing, itching of the nose and eyes, as well as tearing and occasional eye redness. Some relief is obtained with
an over-the-counter cold medicine containing antihistamine and a decongestant. His history suggests which of the following?

(A) nasal foreign body
(B) immunologic deficiency
(C) rhinitis medicamentosa
(D) chronic sinusitis
(E) allergic rhinitis

Anser


(E)

The symptoms are suggestive of perennial allergic rhinitis. Causative agents are usually those to which the child is exposed year round, such as house dust, mold spores, or pet danders.

Seasonal allergic rhinitis is attributable to sensitization to pollens of trees, grasses, and weeds. Nasal foreign bodies usually result in a foul smelling, unilateral purulent, and occasionally blood tinged, discharge. Recurrent
infections may rarely be attributable to immunologic deficiencies. Recurrent pneumonias are the most common complaint.

Rhinitis medicamentosa occurs secondary to excessive use of vasoconstrictor nose drops or sprays,
resulting in rebound nasal obstruction.

Sinusitis is suggested by a bilateral purulent nasal discharge, often accompanied by fever,
cough, headache, and sometimes sinus tenderness.

MSQs In bone diseases

Q1:

A 1-year-old Saudi infant is in for well-child care. He is primarily breast-fed. His parents do not give him much solid food because he has no teeth. He receives no medications or supplements. His parents are concerned about his bowed legs. On examination, you note some other bony abnormalities including frontal bossing, enlargement of the costochondral junctions, a protuberant sternum (pigeon chest), and severe bowing of the legs. You obtain x-rays to confirm your clinical diagnosis and also note a healing fracture of the

left femur. Which of the following is the most likely diagnosis?

(A) osteogenesis imperfecta

(B) scurvy

(C) congenital syphilis

(D) rickets

(E) chondrodystrophy

Answer

(D)

 Babies who are exclusively breast-fed for prolonged periods of time are at risk for developing rickets. Dark-skinned infants are at high risk, especially during winter months when they receive inadequate sunlight. Supplementation with vitamin D is recommended in children who are at high risk, as well as pregnant and lactating mothers. Clinical features include craniotabes, a thinning of the outer table of the skull.

This may also occur in osteogenesis imperfecta. Enlargement of the costochondral junctions (rachitic rosary) may be seen in rickets, scurvy, and chondrodystrophy. Other features may include delayed primary teeth, enamel defects, and caries. There may be thickening of the wrists and ankles; bending of the femur, tibia, and
fibula result in bowlegs or knock-knees.

Greenstick fractures of long bones may occur without symptoms.

 Diagnosis is based on history of inadequate vitamin D intake and clinical features. Diagnosis may be confirmed by x-rays and chemistry; serum calcium is low or normal, serum phosphorus is low, serum
alkaline phosphatase is elevated, and serum 25-hydroxycholecalciferol is decreased. Breast milk contains adequate vitamin C as long as the mother is not deficient.

MCQs In Pediatric Urology

Q1 :

A14-year-old boy presents with sudden onset of pain and swelling of his right testicle. There was no history of trauma, he is not sexually active, and denies any history of penile discharge. On examination, the scrotum is swollen and tender. The cremasteric reflex is absent. A testicular flow scan shows a “cold spot” or

absent flow to the affected side. Which of the following is the most likely cause?

(A) inguinal hernia

(B) hydrocele

(C) epididymitis

(D) testicular torsion

(E) torsion of the appendix testis

Answer :

(D) 

Testicular torsion is the most common cause of testicular pain in boys 12 years and older and is uncommon in those under 10 years. It may be sometimes related to trauma or injury but may occur spontaneously. If not diagnosed early, loss of blood flow to the testicle may result in permanent loss of testicularfunction. 

Torsion of the testicular appendix usually occurs between the ages of 2 and 11 years. The testicular appendix is a vestigial stalk at the upper pole of the testis. Torsion results in pain and swelling of the scrotum, but

the onset of pain is more gradual. There is a 3- to 5-mm indurated, tender mass at the upper pole of the testis. It may sometimes be visible as

a “blue-dot.” Testicular scan may be helpful when this cannot be clinically differentiated from testicular torsion. A hydrocele is a painless collection of fluid in the tunica vaginalis.

Transillumination confirms that the mass is filled with fluid. Hydroceles are present in 1–2% of male newborns and usually resolve by age 1 year. In older boys, a communicating hydrocele may be associated with an inguinal hernia. Inguinal hernias usually appear as a bulge in the inguinal area extending into the scrotum. Hernias are painless, and are more noticeable during crying or straining. They are painful only when strangulated or incarcerated. Epididymitis is an acute inflammation of the epididymis, and is more common in sexually active adolescents. Urinalysis showspyuria and the etiology may be gonococcus or chlamydia but is often undetermined.

MCQs In Pediatric Rheumatology

Q 1:

A17-year-old girl comes to the clinic with several weeks of joint pain and rash. The joint pain is most prominent in the hands. She states that the pain is most severe in the morning and tends to improve over the day. She has noted some swelling of her fingers. She has also had a rash on her face that becomes more prominent when she is outdoors. She states that sunlight tends to bother her eyes. On further questioning, she states that she has not felt well for several months. She has had intermittent fever, has been more tired than usual, and has lost weight although she has not been restricting her diet. On physical examination, she looks tired. She has lost 5 lbs since her last visit 1 year ago. She has an erythematous rash on her cheeks. She has several shallow ulcers in her mouth. She has fusiform swelling of her fingers and pain with movement of her fingers. Which of the following is the most likely diagnosis?

(A) systemic lupus erythematosus (SLE)

(B) dermatomyositis

(C) juvenile rheumatoid arthritis

(D) rheumatic fever

(E) Lyme disease

Answer

(A)

 SLE is an autoimmune disorder that affects multiple organs. The diagnosis is based on the presence of four or more major criteria. These include malar rash, oral ulcers, arthritis, and photosensitivity. This patient has all of these symptoms, as well as the systemic symptoms often seen at presentation. The disorder is predominately a disease of women and, in the pediatric population, is a disease of adolescence.

Chronic renal disease is an important and common cause of morbidity and mortality among patients with SLE.

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Q 2:

A 3-year-old boy has had fever for 4 days. On physical examination he has bilateral cervical lymphadenopathy, injected pharynx, and dry cracked lips. A throat swab is done and the rapid strep test is negative. The child is sent home and advised to follow-up if symptoms worsen. The child is brought back 2 days later with all previous findings including a maculopapular rash, swollen hands, and conjunctivitis.
Which of the following is the most likely diagnosis?

(A) Scarlett fever
(B) Kawasaki disease
(C) toxic shock syndrome
(D) infectious mononucleosis
(E) erythema infectiosum

B

Criteria require fever of at least 5 days’ duration for a clinical diagnosis of Kawasaki disease.

 According to these criteria, patients also must have at least four of five other findings, including bilateral conjunctival infection, one or more changes of the oral mucous membranes (e.g., pharyngeal erythema; dry, fissured, and erythematous lips; and strawberry tongue), one or more changes of the extremities (e.g.,
erythema, edema, and desquamation), rash, and cervical lymphadenopathy. Kawasaki disease occurs most commonly during the first 2 years of life. Thrombocytosis, rather than thrombocytopenia, is an almost invariable feature late in the course of illness. The most common serious complication of Kawasaki disease is coronary artery aneurysm formation which can result in thrombosis, aneurysmal rupture, or other cardiac effects. 

MCQs In Syndromes

Q 1 -2 :

A 12-year-old boy comes to the clinic for a sports physical. He is new to your practice. He comes with

his foster mother, who states that he was recently placed in her care because of his mother’s problems with drug abuse. Although a complete medical history is not available, she knows that he has not

received regular care. He does not have any chronic medical problems. She also knows that his father

died of heart disease when he was 35. On physical examination, the boy’s height is greater than the

95th percentile. His arm span exceeds his height. 

Q 1: 

Which of the following is the most likely cause of his tall stature?

(A) Ehlers-Danlos syndrome

(B) Kleinfelter syndrome

(C) Marfan syndrome

(D) Noonan syndrome

(E) Williams syndrome

Q2:

 As you continue your physical examination, you remember that congenital heart disease is common in this particular syndrome. Which of the following is the most likely congenital heart defect in patients with this syndrome?

(A) supravalvular aortic stenosis

(B) AV canal defects

(C) coarctation of the aorta

(D) pulmonary valvular stenosis

(E) mitral valve prolapse


Q 1 :

(C) Marfan syndrome is a genetic disorder of connective tissue. It is transmitted in an autosomal
dominant manner. Patients have tall stature and skeletal disproportion, where the
arm span exceeds the height. Other important clinical features include subluxation of the
ocular lens which occurs in 50–80% of patients.

Progressive dilatation of the aortic root and ascending aorta can lead to dissection or rupture.

Q 2:

 (E) Marfan syndrome is associated with mitral valve prolapse and aortic root dilatation.

MCQs In Pediatric Oncology

Q1 :

Amother brings in her 3-year-old girl because  she felt a smooth mass on the left side of her belly when she was giving her a bath. Which of the following is the most likely diagnosis?

(A) Wilms tumor

(B) neuroblastoma

(C) acute lymphoblastic leukemia

(D) Hodgkin’s disease

(E) hepatoblastoma

Answer

(A)

 Wilms tumor is a malignant embryonal neoplasm of the kidney. It is the second most common solid tumor of childhood. Girls are affected more frequently than boys (2:1). The incidence of Wilms tumor peaks at 1–3 years of age.

 The classic presentation is a painless abdominal mass that is usually hard, smooth,and unilateral. Hematuria occurs in 12–25% of children with Wilms tumor, and hypertension has been reported in up to 60% of patients.

Aniridia or hemihypertrophy may be observed in patients with Wilms tumor.


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MCQs In Puberty

Q 1:

A 14-year-old boy complains of breast enlargement on the left side. He denies pain, discharge,

or any drug use. He is on no medications and is otherwise healthy. On physical examination, his both genitalia and pubic hair growth. Initial management should include which of the following?\

(A) magnetic resonance imaging of the head

(B) urine drug screen for marijuana

(C) chromosome analysis

(D) reassurance that this is a normal condition

(E) ultrasound imaging of the abdomen and testes

Answers :

(D) Gynecomastia is the enlargement of male breast tissue and occurs in approximately onethird of adolescent males during early- to midpuberty.

It usually resolves spontaneously and requires no further evaluation beyond a careful history and physical examination. Features include: breast tissue <4 cm in diameter and resembling female breast budding, and pubertal development between Tanner stage II and IV.

Pubertal development signs precede gynecomastia by at least 6 months. It may be more
noticeable in obese boys. A drug and medication history should be obtained; these include estrogens, androgens, human chorionic gonadotropin (hCG), cardiovascular drugs (reserpine, methyldopa, digitalis),
cytotoxic agents (busulfan, vincristine), antituberculosis drugs (INH), psychoactive drugs (tricyclic antidepressants, diazepam), ketoconazole, spironolactone, cimetidine, and phenytoin. Illegal drugs include marijuana, heroin, methadone, amphetamines, as well as alcohol.

If there is evidence of precocious puberty, hypogonadism or macrogynecomastia (breast tissue >5 cm diameter), laboratory testing should be done including dehydroepiandrosterone sulfate (DHAS), FSH, and LH, hCG, estradiol, and testosterone. Thyroid-stimulating hormone (TSH) may be obtained to rule out
hyperthyroidism. Boys with Klinefelter syndrome have hypogonadism (testes <3 cm in diameter), delayed pubertal development, and gynecomastia. Laboratory tests reveal increased FSH and LH, and decreased testosterone; the diagnosis is confirmed by chromosome
analysis.

If DHAS, hCG, or estradiol levels are increased, an MRI of the head to exclude a CNS tumor and ultrasound of abdomen and testes to rule out an adrenal, liver, or testicular tumor should be considered.

MCQs In Pediatric Emergencies

Q 1:

A 2-month-old infant is brought to the emergency department with irritability and lethargy.  The parents state that he was well until he rolled off the couch on to the floor yesterday. On examination, he is inconsolable and afebrile. The fontanels are full and tense. He has a generalized tonic-clonic seizure. Which of the following is the most important initial diagnostic study to order?

(A) serum calcium, phosphorus, and magnesium levels

(B) analysis of cerebrospinal fluid (CSF)

(C) cranial computed tomography (CT) scan

(D) serum ammonia level

(E) serum acetaminophen level

Answer :

(C)

Though infection must be considered as an etiology, acute trauma is more likely in this scenario.
This case represents the classic picture of the shaken baby syndrome which produces intracranial trauma without obvious external findings. This infant is critically ill and lacks preceding illness or constitutional symptoms.

The tense fontanels reflect increased intracranial pressure. Acranial CT scan may show diffuse edema or a localized lesion, such as a subdural hemorrhage. Metabolic causes of seizures do not cause increased intracranial pressure. Acetaminophen toxicity does not cause CNS symptoms.


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Q 2:

A5-year-old pedestrian is hit by a car in a mall parking lot and he is brought to the emergency department. There was loss of consciousness for less than 1 minute. On evaluation, the child has no neurologic deficits and a CT scan of the head reveals no intracranial abnormalities and no obvious skull fractures. The parents want to know what possible long-term problems there might be. You remember that problems after head trauma may include the development of seizures and that the risk of developing posttraumatic epilepsy is increased by which of the following?

(A) a brief loss of consciousness
(B) an acute intracranial hemorrhage
(C) retrograde amnesia
(D) posttraumatic vomiting
(E) a small linear skull fracture

Answer

(B)

Late posttraumatic epilepsy is diagnosed when a seizure occurs for the first time more than 1 week after a head injury. Factors that correlate with an increased risk of developing posttraumatic epilepsy include presence of a depressed skull fracture, acute intracranial hemorrhage, cerebral contusion, or unconsciousness lasting more than 24 hours. Because the risk of a subsequent seizure is approximately
75%, acute and chronic treatment with anticonvulsants is indicated. Loss of consciousness,
retrograde amnesia, and vomiting are relatively common immediate consequences of head trauma. They are usually transient and are not highly correlated with a risk of subsequent posttraumatic seizures.

MCQs In Pediatric Ophthalmology

Q 1:

A 5-year-old child was hit in the right eye by a toy. He is rubbing at his eye, which is watering

profusely. There is a small abrasion at the corner of the eye. He is mildly photophobic, but

his pupils are equal, symmetric, and reactive to light and accommodation. His vision is normal.

Which of the following is the most appropriate next step in the management of this patient?

(A) Perform a fluorescein dye stain of the cornea to determine if there is a corneal abrasion.

(B) Refer him immediately to an ophthalmologist.

(C) Irrigate the eye with sterile normal saline.

(D) Discharge him to home with antibiotic eye ointment.

(E) Apply a patch to the eye and follow-up in a week.

Answer:

(A)

Superficial corneal injuries expose underlying layers causing pain, photophobia, tearing, and decreased vision. Irrigation is recommended only if a foreign body is suspected.

Abrasions are detected by instilling fluorescein dye and inspecting the cornea using blue-filtered light. Treatment consists of frequent applications of topical antibiotic ointment until the epithelium is healed. The use of a patch does not accelerate healing, and if improperly applied, may abrade the cornea. Referral to an
ophthalmologist should be considered if there are significant changes in vision, or signs of
deeper or more penetrating injury which often result in papillary abnormalities.

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Q 2:


A 5-year-old febrile child presents with swelling of the right eyelid. Proptosis and limitation
of ocular movements is noted. Which of the following is the most likely diagnosis?

(A) retinoblastoma
(B) orbital cellulitis
(C) periorbital cellulitis
(D) neuroblastoma
(E) hyphema

answer:


(B)

 Orbital (also referred to as postseptal) cellulitis is a medical emergency. It is a bacterial infection of the orbit. It must be distinguished from periorbital (also referred to as preseptal) cellulitis by the presence of proptosis or limitations of extraocular movements.

When orbital cellulitis is suspected, cultures of blood and CSF should be obtained, appropriate antibiotics should be administered intravenously, an ophthalmologist should be consulted, and CT films should be obtained to delineate the extent of the infectious process.Both retinoblastoma and battered child syndrome
may present with lid edema.

Typically, these children are afebrile and nontoxic in appearance. Hyphema is hemorrhage into the anterior chamber of the eye and is caused by trauma. Twenty percent of patients with neuroblastoma present with eye symptoms from metastasis. Proptosis is one of the possible presentations and can be of relatively acute onset.

In general, other systemic symptoms are present and have developed more gradually.


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Q 3:

Among the conditions that cause edema of the eyelids is orbital cellulitis. This is a serious infection that must be recognized early and treated aggressively if complications are to be avoided. Which of the following features is useful in differentiating orbital cellulitis from periorbital (preseptal) cellulitis?

(A) proptosis
(B) elevated WBC count
(C) fever
(D) lid swelling
(E) conjunctival inflammation


Answer:

(A)

Proptosis and limitation of extraocular motility distinguish orbital cellulitis from periorbital cellulitis. Fever, lid swelling, redness of the eye, and leukocytosis generally are present in either condition. Orbital cellulitis (infection within the orbit) may follow directly from a wound near the orbit or may result from bacteremia,
but the most common source involves extension from the paranasal sinuses. The organisms most frequently implicated as pathogens are H. influenzae, S. aureus, group A beta-hemolytic Streptococci, and S. pneumoniae. The risk of complication is great, with extension resulting in cavernous sinus thrombosis,
meningitis, or brain abscess. Prompt hospitalization and parenteral antibiotic therapy are indicated. 

MCQs In General Pediatrics

Q 1:

A young mother claims that her 4-week-old child sleeps best on his stomach. You tell her

that the safest sleep position for infants is which of the following?

(A) on the back

(B) on the stomach

(C) on the side

(D) on the back with the head elevated by a pillow

(E) in the parents’ bed

(A)

Prone sleeping is a major risk factor for sudden infant death syndrome (SIDS). Since the 1992 American Academy of Pediatrics (AAP) recommendation that infants be placed to sleep on their backs, the frequency of prone sleeping has decreased from 70 to 20%, and the SIDS rate has decreased by >40%. Side sleeping has a slightly higher SIDS risk than supine but is still safer than the prone position. Other risk
factors include maternal smoking, soft bedding, overheating, younger maternal age, prematurity,
low birth weight, and male gender.

Q 2:

A 2-year-old girl has severe dental caries of the upper and lower incisors. Her teeth are brushed twice daily with a small amount of fluoride-containing toothpaste. What is the feeding practice most likely to result in this
pattern of dental caries?

(A) drinking juice from a cup at snack time
(B) drinking juice from a bottle at snack time
(C) drinking milk from a bottle at meal time
(D) prolonged breast-feeding beyond the first year
(E) drinking a bottle of juice in bed

Answer :

(E)

Organic acids produced by bacterial fermentation lower the pH of dental plaque causing demineralization and caries of the adjacent tooth. Nursing bottle caries is a pattern of caries involving the upper and lower incisors. It occurs because of prolonged contact of the tooth to a sugar-containing liquid (juice or milk). This
is more likely to occur with overnight exposure or with use of a bottle. Asimilar pattern of caries
may rarely occur with breast-fed babies who feed through the night. Cup feeding or drinks
given during mealtimes are less likely to cause prolonged contact to the teeth.

MCQs In Viral Hepatitis

Q 1:

A baby is born to a mother who is positive for hepatitis B surface antigen (HBsAg). Your plan

is to do which of the following?

(A) Give the infant a hepatitis B immunization.

(B) Give the infant hepatitis B immune globulin (HBIG).

(C) Give the infant a hepatitis B immunization and HBIG.

(D) Obtain liver function tests and hepatitis serology of the infant.

(E) Give the HBIG only if the child is positive for HBsAg.

C

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Q 2:

You receive a call from the parents of a 1 year old who is due for his well-child visit next week. They have just received a letter from their daycare center that an employee has hepatitis A. Which of the following is the best treatment plan?

(A) Give hepatitis A immune globulin and hepatitis A vaccine.
(B) Treat with hepatitis A immune globulin.
(C) Obtain hepatitis A serology and give hepatitis A vaccine.
(D) Give hepatitis A vaccine.
(E) No treatment is needed.

(A)

 Children, especially those in daycare, commonly are infected with the hepatitis A virus. Unlike adults, children most often are asymptomatic. Frequently, outbreaks of hepatitis A in a daycare center are not recognized until a daycare worker or parent of an attendee becomes ill. Immunization against hepatitis A
virus is now routinely recommended because of this. After exposure both immune globulin
and vaccine should be given to the unvaccinated individual.

MCQs In Pediatric Neurology For Medical Students.

Q1:

You suspect the diagnosis of Werdnig-Hoffman disease in an infant with severe hypotonia.

Which other finding will support this diagnosis?

(A) normal deep tendon reflexes

(B) seizures

(C) fasciculations of the tongue

(D) recurrent fevers

(E) atrophy of the optic nerve

MCQs In Pediatric Hematology - For Medical Students

A 2-year-old African-American child presents with painful swelling of the hands and feet.

Laboratory evaluation reveals hemoglobin of 9 g/dL with white blood cell count of 11,500

and platelet count of 250,000. Which additional laboratory test will support your diagnosis?

(A) skeletal survey

(B) VDRL testing

(C) bone marrow aspiration

(D) hemoglobin electrophoresis

(E) serum calcium measurement

Answer :

(D) The child described has the classic handfoot syndrome seen in infants and toddlers with sickle cell disease. Dactylitis, presumably secondary to infarction of the small bones, causes painful swelling of the hands and feet. Hemoglobin electrophoresis would show presence of high levels of HbS. 

In children younger than 5 years, the small bones of the hands and feet are frequently affected, and in contrast to most episodes of bone pain in older children, physical findings are common. 

This painful dactylitis (“hand-foot syndrome”) is typically the first clinical manifestation of SCD. A young child cries with pain; refuses to bear weight; and has puffy, tender, and warm feet or hands, or both. The child may appear acutely ill, be febrile, and have an impressive leukocytosis. At the onset of soft tissue swelling, bony changes are not generally apparent on radiographs. 

After 1 to 2 weeks, subperiosteal new bone, irregular areas of radiolucency, cortical thinning, or complete destruction of bone can be seen. All the bone changes are usually reversible but may persist for as long

as 8 months. A rare complication, permanent shortening of the digits after hand-foot crisis, has been

reported. Dactylitis before 1 year of age is a strong predictor of overall severity (stroke, death, high pain rate, or recurrent ACS) by 10 years of age, although recent single-institution evidence suggests that dactylitis is not a strong predictor of subsequent pain or ACS.

MCQs In Pediatric Infectious Diseases

Q 1:

A 2-year-old child was recently adopted from India. She appears to be healthy, and there are no abnormal symptoms. Her weight and height are at 25th percentile for age. Her examination is normal. On screening, you find a positive TB skin test using purified protein derivative (PPD) with 20 mm induration. She has a history of receiving a BCG vaccination at birth. Your management plan is to do which of the following?

(A) Obtain a chest x-ray and treat only if this is abnormal.

(B) Obtain a chest x-ray and initiate prophylactic treatment with isoniazid (INH).

(C) Repeat the test in 3–6 months.

(D) Attribute the positive PPD to the BCG vaccination and do serial yearly x-rays.

(E) Obtain sputum cultures.

Answer :

(B)

Generally, the interpretation of tuberculin skin test (TST) is the same regardless of BCG status.

 Induration >5 mm is considered positive in children in close contact with known or suspected cases of tuberculosis disease or children suspected to have tuberculosis disease.

Induration >10 mm is considered positive in children at greater risk of disseminated disease (age <4 years; other medical conditions such as lymphoma, diabetes, chronic renal failure, or malnutrition) or children at greater risk of exposure to tuberculosis disease (born in, or parents born in high-prevalence regions, travel to these regions, exposure to adults at high risk, such as HIV infected, homeless, or drug abusers).

Induration >15 mm is positive in children >4 years without any risk factors.

 Radiographic evaluation of all children with positive TST is recommended. Latent tuberculosis infection is
defined as an infection in a person with a positive TST, no physical findings of the disease, and a chest radiograph that is either normal or reveals only granulomas or calcifications in the
lungs or regional lymph nodes.

 Children with latent tuberculosis infection should receive prophylaxis, usually 9 months of INH. Those with
symptoms, signs, and/or radiographic manifestations are said to have tuberculosis disease.

There is no benefit to repeating the test in 3–6 months, and it will delay treatment. Sputum cultures are difficult to obtain in younger children.

 Gastric aspirate specimens obtained with a nasogastric tube are preferred. Culture material should be obtained in children with evidence of the disease in order to obtain information on drug susceptibility and resistance patterns.


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Q 2:

A parent brings in a 5-year-old boy being treated for acute lymphocytic leukemia (ALL). He states a friend who is staying with them at their home has just come down with chicken pox. Your patient has not had chicken pox or received immunization with varicella vaccine. What is the appropriate treatment?

(A) acyclovir given IV
(B) varicella vaccine
(C) varicella immune globulin (VZIG)
(D) varicella vaccine and VZIG
(E) acyclovir given IV for 7 days, varicella vaccine, and VZIG

Answer :


(E)

Children with chicken pox may be infectious for 1 or 2 days before the appearance of the rash.
Once skin lesions have crusted, the patient is no longer infectious. Susceptible individuals can
contract chicken pox from patients with zoster.

In the cases of both chicken pox and zoster, transmission is thought to occur by the respiratory
route rather than by direct contact. The virus can travel long distances in the air and remain viable.

Transmission from one hospital patient to other susceptible hospitalized patients has been reported to occur through air vents. VZIG should be given within 3 or 4 days of exposure to varicella-susceptible individuals who are immunocompromised.

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Q 3

A 3-month-old infant is brought to your office in the winter with a history of 1 day of vomiting, followed by 3 days of diarrhea. She has had six to eight stools per day, which are loose and foul smelling. On examination, she looks well. Which of the following viruses is the most likely cause of her illness?

(A) adenovirus
(B) enterovirus
(C) human herpesvirus, type 6
(D) respiratory syncytial virus
(E) rotavirus

Answer :

(E)

 Because many childhood viral illnesses have seasonal presentations, the etiologic agent may be suspected on the basis of clinical and seasonal presentation. Yearly winter outbreaks of bronchiolitis and pneumonia are associated with respiratory syncytial virus. Summer outbreaks of gastroenteritis are associated with
enterovirus, while winter outbreaks are associated with rotavirus. Although adenovirus can cause diarrhea, it more commonly causes respiratory symptoms. Human herpes virus type 6
is the etiologic agent in roseola infantum.

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Q 4:

A 10-year-old boy comes to your office in the winter with a sore throat he has had for 2 days. In addition, he has had fever, headache, and abdominal pain. He does not have any allergies to medications. On examination, he has a temperature of 38.6°C, an erythematous pharynx, and tender cervical adenopathy. Arapid screening test for group Astreptococcus is performed and is positive. Which of the following would
be the most appropriate antimicrobial agent?

(A) erythromycin
(B) penicillin
(C) trimethoprim-sulfamethoxazole
(D) azithromycin
(E) cefaclor

Answer:


(B)

Penicillin remains the drug of choice for treatment of streptococcal pharyngitis. Amoxicillin,
macrolides, and cephalosporins are acceptable alternatives.


Q 5:

 The same child returns to your office the next day. He has taken the medication you prescribed. He is feeling a little better. His fever has resolved, but he has developed a rash. His examination is unchanged, except that he is afebrile and has a fine, papular rash over his body, which is accentuated in his axilla and groin. Which of the following is the most likely cause of his rash?

(A) allergic reaction to the antibiotic
(B) rash from the antibiotic seen in patients with mononucleosis
(C) scarlet fever
(D) serum sickness
(E) viral exanthem typical of enterovirus

(C)

Scarlet fever is caused by toxins made by group A Streptococci. It is usually seen in patients with strep throat. The rash is papular and described as sandpaper like. Sometimes it is easier to feel it than to see it. An allergic rash would be urticarial. More than 80% of patients with EBV infection develop a maculopapular
rash if given amoxicillin.

 This patient’s clinical course is not typical for EBV which presents more gradually, and patients often have posterior cervical adenopathy and splenomegaly.

Patients with serum sickness often have urticarial rashes, sometimes progressing to angioedema. They may also have arthritis, myalgias, and lymphadenopathy. The rash in enteroviral infections is typically macular


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Q 6:

A 4-year-old child presents to your office in July with a history of a low-grade fever (38.1°C) and “sores” in his mouth for 2 days. He has been refusing to eat but has been drinking an adequate amount of liquids. On examination, he is afebrile and seems well hydrated. He has ulcers on his tongue and posterior pharynx,
which are 4 mm in diameter. You also note a few vesicles on his hands and feet, which are 3–4 mm in size and mildly tender. Which of the following is the most likely diagnosis?

(A) herpes simplex virus (HSV)
(B) coxsackie virus
(C) aphthous ulcers
(D) Behçet syndrome
(E) traumatic ulcers

Answer


(B)

Coxsackie A16 is the major cause of hand, foot, and mouth disease. This is a summer enteroviral illness presenting with classic lesions of the hand, feet, and mouth. Herpetic gingivostomatitis is the most common cause of stomatitis in children aged 1–3 years.

There is often a high fever, fetor oris, refusal to eat, and irritability. The lesions are initially vesicular, and soon form ulcers ranging from 2 to 10 mm in diameter. The tongue, cheek, and gums are usually involved, and there may be submaxillary lymphadenitis.

Aphthous ulcerations (canker sores) are painful ulcerations, which present as erythematous, indurated papules that erode to form circumscribed necrotic ulcers with gray fibrinous exudates and erythematous
halo. They are 2–10 mm in diameter, heal spontaneously, and often recur. Behçet syndrome is a multisystem disorder characterized by recurrent oral and genital ulceration, iritis or uveitis, as well as other cutaneous, arthritic, neurologic, vascular, and gastrointestinal (GI) manifestations. It is rare in children. Traumatic
oral ulcers may be seen in chronic cheek biters but do not involve extremities.

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Q 7:

A10-year-old boy comes to the office with fever and chills for 5 days and myalgia. He has recently returned from a 2-week vacation to New England with his family. On physical examination he has mild splenomegaly. Which of the following is the most likely cause of his symptoms?

(A) Kawasaki disease
(B) pneumococcus
(C) babesiosis
(D) leptospirosis
(E) psittacosis

Answer :

(C)

Kawasaki disease is an acute vasculitis of unknown etiology. Humans contract brucellosis by direct contact with infected animals or by drinking unpasteurized milk. Babesiosis is transmitted by ticks. Leptospirosis is
obtained from exposure to the urine of infected animals. Psittacosis is obtained from exposure to bird feces.

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Q 8:


A 6-year-old girl has a low-grade fever, headache, and nasal congestion. She has a flushed face and has developed a lacy reticular rash on the trunk and extensor surface of her arms and legs. Palms and soles are spared. Her mother has been ill with a low-grade fever and some joint stiffness and pain. Which of the following is the most likely diagnosis?

(A) rubella
(B) measles
(C) scarlet fever
(D) roseola infantum
(E) erythema infectiosum (fifth disease)

(E)

Erythema infectiosum is a common childhood viral exanthem caused by parvovirus B19.

It was the fifth in a classification system of childhood exanthems; the others were rubella, measles, scarlet fever, atypical scarlet fever, and roseola infantum. The rash classically presents early with flushed cheeks or a “slapped cheek” appearance. It is followed by development of a macular erythematous rash on trunk and
extremities, which then shows central clearing, developing a lacy, reticulated appearance.

 The infection is often not clinically apparent. Adult and older adolescents, especially females, may
develop arthropathy. The symptoms are usually self-limited. Parvovirus B19 is clinically significant
in people with hemolytic anemias because it may induce a transient aplastic crisis.

Immunocompromised individuals are also at risk for chronic infections accompanied by anemia, neutropenia, and thrombocytopenia. It may also induce fetal demise in case of primary infection of pregnant women.

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Q 9:

A 3-year-old boy was bitten while teasing a neighborhood cat. On examination, there are two puncture wounds on the right hand and some superficial scratch marks. There is erythema, warmth, and induration around the puncture sites. Which of the following organisms most likely caused the infection?

(A) Pasturella multicoda
(B) Bartonella henselae
(C) Eikenella corrodens
(D) Peptostreptococcus species
(E) alpha Streptococci


Answer:

(A)

P. multicoda and S. aureus are organisms commonly associated with cat bites. The cat’s sharp teeth and claws predispose the victim to puncture wounds. Wound infections are more common in cat bites than dog bites. E. corrodens,

Peptostreptococcus species, and alpha Streptococci are more common with human bites. B. henselae
causes cat-scratch disease, which presents with subacute lymphadenitis.

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Q 10:

A 7-year-old boy presents with a rash. His mother states that he was well until 3 days ago when he developed fever and malaise. The next day, the rash started as papules on the trunk, which rapidly changed to vesicles. The lesions have spread all over the body. On physical examination, he has no fever and seems well. You note numerous vesicles all over the body, some of which have crusted over. Which of the following is the most likely diagnosis?

(A) chicken pox
(B) Kawasaki disease
(C) measles
(D) rubella
(E) staphylococcal scalded skin syndrome


Answer

(A)

This is a typical presentation of chickenpox. A prodrome of fever and malaise is followed by the rapid eruption of papules that turn to vesicles and crust over. The rash in measles, rubella, and Kawasaki disease are macular or maculopapular. In staphylococcal scalded skin syndrome, a diffuse, tender erythroderma
develops.

Clinical Cases - Endocrinology

Q1:

A 13-year-old girl presented at clinic having been diagnosed as having hypothyroidism by her family

doctor who had confirmed the diagnosis with TFT’s. She also had a 2-year history of a limp in her left leg. On examination, she was short and obese with a goitre and other signs of hypothyroidism. She had

limitation of movement of her left hip and a limp.

Questions

1 What is the most likely diagnosis?

2 What investigations should be done?

3 What is the treatment?

Answers :

1 Slipped upper femoral epiphysis and Hashimoto’s disease.

2 Frontal and lateral hip X-rays (a frontal X-ray alone may not demonstrate the slipped epiphysis)
and thyroid autoantibodies.

3 In spite of the long history, urgent referral to an orthopaedic surgeon and urgent surgery are
necessary. An acute or chronic slip of the epiphysis may cause avascular necrosis of the femoral head.
Prophylactic pinning of the other femoral head is advocated by some surgeons. T4 treatment should
also be started.

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Case 2

A 13-year-old boy was referred to the regional endocrine clinic for consideration of growth hormone (GH) treatment. He also had delayed puberty and intermittent headaches. On examination, his height was > –4.0 SD with evidence of growth failure for at least 4 years. His weight was –1.0 SD and he was entirely prepubertal. A recent GH stimulation test at the referring hospital showed a maximum response to a diethylstilbestrol primed clonidine test of 5 mU/L. He was said to have had normal TFT’s 2 years previously with a FT4 = 9.2 pmol/L (9–24) and a TSH of 1.2 mU/L (0.4–4.0).

Questions

1 Are these TFT’s normal?
2 What is the likely overall diagnosis?
3 Is there a problem in interpreting his clonidine test?

MCQs In Pediatric Pulmonology

Q1 :

You are caring for an 8-month-old infant in the pediatric intensive care unit. She has been

hospitalized for 1 week with respiratory syncytial virus bronchiolitis. In discussions with the

mother, she reports that the child has very frequent, large, foul-smelling stools. Physical

examination of the child reveals a temperature of 37.0°C, heart rate of 140 beats/min, respiratory

rate of 35 breaths/min on the ventilator, and blood pressure of 80/40 mm Hg. Her oxygen

saturation is 90%, and her most recent arterial blood gas shows a pH of 7.25, Paco2 of 70 mm

Hg, and Pao2 of 70 mm Hg. Her weight is 7 kg, and she appears malnourished, with decreased

muscle development. No obvious congenital abnormalities are apparent. Her chest appears

hyperinflated, she has no heart murmur, her pulses are equal and strong, and her abdomen is

protuberant with normal bowel sounds. Computed tomography scan of her chest was obtained

earlier today to evaluate enlarging cystic-appearing lesions on her chest radiograph.

Of the following, the MOST likely underlying diagnosis in addition to bronchiolitis is

A. Clostridium difficile infection

B. congenital lobar emphysema

C. cystic adenomatoid malformation

D. cystic fibrosis

E. pulmonary sequestration

Pediatrics For Medical Students - Neonatology

You are called to evaluate a 4-day-old infant in the nursery for worsening jaundice. The patient is the first child of a 32-year-old mother, who had an uncomplicated full-term pregnancy and normal spontaneous vaginal delivery. The mother is otherwise healthy but has a sister who had her gallbladder removed as a child. The infant has been formula fed and has been eating and stooling normally. The total bilirubin level is 19.2 mg/dL, with a direct fraction of 0.3 mg/dL. On physical examination, the infant is jaundiced but

otherwise well, with no other significant physical findings. SELECT THE ONE BEST ANSWER

1. The differential diagnosis of the unconjugated hyperbilirubinemia in this child includes which of the following?

(A) biliary atresia

(B) alpha1-antitrypsin deficiency

(C) ABO blood type incompatibility

(D) Caroli syndrome

(E) Sickle cell anemia

Answer

C

 Neonatal jaundice occurs in approximately twothirds of infants and is defined by bilirubin levels higher than 5 mg/dL. Bilirubin is generated as one of the products of the breakdown of hemoglobin and the conjugation of bilirubin to bilirubin glucuronide occurs in the liver.

 Neonatal unconjugated hyperbilirubinemia is therefore the result of either increased bilirubin production or decreased conjugation.

Neonatal hemolytic anemias, with increased bilirubin production, can result in severely abnormal unconjugated hyperbilirubinemia levels in neonates.

Blood group mismatches because of ABO or Rh mismatches are common causes of neonatal hemolytic
anemia and unconjugated hyperbilirubinemia.

Other causes of unconjugated hyperbilirubinemia include hemolytic anemias because of red blood cell membrane or enzyme defects and increased red blood cell turnover associated with polycythemia, internal hemorrhages such as cephalohematomas or intraventricular hemorrhages. Decreased bilirubin conjugation as
a result of Crigler-Najjar or Gilbert syndromes also result in unconjugated hyperbilirubinemia. Biliary
atresia, α1-antitrypsin deficiency, and Caroli syndrome are all causes of neonatal conjugated hyperbilirubinemia

2. The laboratory evaluation of this infant’s hyperbilirubinemia should include all of the following except

(A) complete blood count with smear

(B) indirect and direct Coombs test

(C) prothrombin time (PT)/partial thromboplastin time (PTT)

(D) maternal blood type

(E) reticulocyte count

Answer

C

Evaluation for suspected neonatal hemolytic anemias should include evaluation of the complete blood
count along with indirect and direct Coombs tests and maternal and neonatal blood types. In the absence of
excessive bleeding or bruising, there is no indication for coagulation studies. The complete blood count will
reveal the degree of anemia, if any, and the peripheral smear will demonstrate the morphologic features of
the red blood cells that could suggest underlying etiologies.

The Coombs tests will evaluate whether or not there are antibodies that could be contributing to
autoimmune or alloimmune hemolysis. Neonatal and maternal blood types are needed to evaluate the possible presence of ABO and Rh incompatibility.


3. Which of the following combinations of parents’ blood types place an infant at highest risk for
hemolytic anemia?

(A) maternal Rh negative, paternal Rh negative, first child
(B) maternal Rh negative, paternal Rh positive,second child
(C) maternal type O, paternal type O, first child
(D) maternal type AB, paternal type B, second child
(E) maternal type AB, paternal type A, first child

Answer

(B)

 Mothers with Rh negative blood type develop antibodies against the Rh antigen after exposure during
pregnancy to an Rh-positive fetus or after transfusion with Rh-positive blood. In the presence of an
Rh-positive fetus, the mother’s antibodies can cross the placenta and destroy fetal red blood cells, resulting in neonatal hemolytic anemia. Rh hemolytic disease can be prevented with high titer Rho(D) immune globulin treatment for Rh-negative mothers who have been exposed to Rh-positive infants.

Similarly, mothers with type O blood have antibodies against antigens for blood types A and B that can
react to and destroy fetal red blood cells with these blood type antigens. However, a fetus that also possesses type O blood will not be susceptible to hemolysis from these antibodies. A mother with
type AB blood has no antibodies to blood group antigens, and therefore the fetus is not exposed to
any anti-red blood cell antigen antibodies. Despite the presence of these antibodies, only 33% of infants
with ABO “mismatch” will have a positive direct Coombs test, and of those, only 20% will develop
jaundice from excessive hemolysis

4. The differential diagnosis of a neonate with hemolytic anemia includes all of the following except

(A) pyruvate kinase deficiency
(B) ABO incompatibility
(C) Crigler-Najjar syndrome
(D) hereditary spherocytosis
(E) Rh disease

answer

(C)

Crigler-Najjar syndrome is caused by the absence of glucuronyl transferase and results in severe indirect
hyperbilirubinemia. Pyruvate kinase and G6PD are both red blood cell enzymes that, when deficient, can
result in neonatal hemolytic anemia. Hereditary spherocytosis, as well as other syndromes with red blood
cell structural abnormalities, such as hereditary elliptocytosis and paroxysmal nocturnal hemoglobinuria,
can also result in neonatal hemolytic anemia.

 A maternal-fetal blood type mismatch such as ABO incompatibility results in alloimmune hemolytic
anemia, with the mother’s antibodies reacting to and destroying the neonatal red blood cells.


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Q 2:

Anewborn has delayed passage of meconium stools and barium enema radiograph shows dilated proximal colon and small obstructed distal colon. What should be the next diagnostic test?

(A) abdominal CT-scan
(B) stool studies
(C) rectal suction biopsies
(D) sweat chloride testing
(E) chromosome analysis

Answer :

(C)

 Congenital megacolon (Hirschsprung disease) is the result of congenital absence of ganglion cells in a segment of large bowel. Absentor deficient peristalsis in the affected segment results in functional obstruction. This causes constipation and distention of bowel proximal to the aganglionic area. It is this chronically
distended bowel that has led to the name megacolon. Severe cases present as neonatal intestinal obstruction.

Practically all normal, full-term born neonates pass meconium in the first 48 hours of life. Hirschprung disease should be considered in any full-term infant with delayed passage of stool. Rectal manometry
and rectal suction biosy are the easiest and most reliable indicators of Hirschprung disease.

MCQs in Pediatric Neurology Part 111

A 7-month-old male died following a progressive neurological illness over 6 weeks, with somnolence,

blindness, deafness, and generalized limb spasticity. Autopsy showed bilateral symmetric necrotic lesions of the thalamus, pons, inferior olive, and spinal cord. The most likely diagnosis is :

(A) Leber hereditary optic neuropathy

(B) mitochondrial neurogastrointestinal encephalopathy

(C) Leigh syndrome

(D) Alpers disease

(E) myoclonic epilepsy with ragged-red fibers

MCQs In Electrolyte Disorders

Q1 

You are evaluating a 2-week-old breastfed infant who is 15% below his birthweight and has been

lethargic and fed poorly over the past 4 days. You administer a normal saline fluid bolus.

Laboratory results include:

· Blood glucose of 126.0 mg/dl (7.0 mmol/L)

· Serum sodium of 170.0 mEq/L (170.0 mmol/L)

· Serum potassium of 5.0 mEq/L (5.0 mmol/L)

· Blood urea nitrogen of 31.0 mg/dL (11.1 mmol/L)

· Serum creatinine of 2.9 mg/dL (256.4 mcmol/L)

Of the following, the MOST appropriate initial fluid for correction is a solution containing 5%

dextrose and\

A. NaCl (%): 0.20

KCl (mEq/L): 40

Duration of Infusion(hr): 12 to 24

B. NaCl (%): 0.45

KCl (mEq/L): 0

Duration of Infusion(hr): 48 to 72

C. NaCl (%): 0.45

KCl (mEq/L): 40

Duration of Infusion(hr): 12 to 24

D. NaCl (%): 0.9

KCl (mEq/L): 0

Duration of Infusion(hr): 12 to 24

E. NaCl (%): 0.9

KCl (mEq/L): 40

Duration of Infusion(hr): 48 to 72

Answer:

B

 Hypernatremia results from excessive sodium administration (incorrectly mixed formula, sodium bicarbonate, hypertonic saline) or a deficit of water in relation to sodium. Such a deficit may occur from decreased water intake (inadequate breastfeeding, fluid restriction, lack of access to fluids), fluid losses (nephrogenic and central diabetes insipidus, increased insensible losses), or fluid losses in greater proportion than sodium losses (diarrhea, vomiting, diuretic use, burns).

Treatment of hypernatremia is directed at correction of both the serum sodium
concentration and the circulatory volume. Initially, some children may require isotonic fluid
boluses to restore circulation. It is important to note that children who have hypernatremia often
appear less dehydrated than they actually are due to preservation of the extracellular volume.
The following equation can be used to estimate the free water deficit:
Water deficit (mL)= 4 mL x ideal body weight (kg) x desired change in serum sodium
concentration

Hypernatremia, especially if chronic, should be corrected slowly, with a desired goal of
decreasing the serum sodium by 0.5 mEq/L per hour to avoid cerebral edema. Severe
hypernatremia (serum sodium >170.0 mEq/L [170.0 mmol/L]), as described for the child in the
vignette, should be corrected over 48 to 72 hours. Fluid administration generally consists of 1/4
to 1/2 normal saline solutions. Symptoms of overcorrection, such as changes in mental status or
onset of seizures, suggest the development of cerebral edema and should be treated with
hypertonic saline and slowing of the sodium correction. In general, potassium administration
should be withheld in cases of severe hypernatremic dehydration until adequate urine output is
assured

;

MCQs In Metabolic Diseases

Q1:

The pregnant mother of a child in your practice recently learned that her grandmother had a

child who died of "probable metabolic disease" at 2 days of age. She does not know details, and

medical records on that child no longer are available. The mother asks if her pregnancy can be

tested to see if the fetus could be affected with the same disorder.

Of the following, the MOST accurate statement regarding metabolic disease in the prenatal

setting is that

A. fetuses affected with metabolic diseases are unlikely to come to term

B. knowing the parents’ ethnic backgrounds aids in determining which tests should be offered

C. level 2 ultrasonography during the second trimester is likely to be helpful in detecting metabolic

disease

D. poor fetal growth is common in metabolic diseases

E. prenatal metabolic screening panels are widely available

Answer

B

The vignette highlights the importance of accessing as much information as possible
regarding family members who die from metabolic causes. Even if a diagnosis cannot be made
in a particular instance, medical records may be helpful in offering the expectant couple
guidance or in making a diagnosis posthumously. Taking a careful family history, with attention
to familial ethnic, religious, and geographic origins, may bring to light conditions that are likely to
run in families. For example, because of the high carrier rate for some metabolic diseases,
parents of Ashkenazi Jewish descent, whose ancestors originated from Eastern Europe, are at
increased risk for having children affected by Tay-Sachs disease  and people of
French-Canadian heritage are at increased risk for having children affected by tyrosinemia.

Cases

A 3 year old male child born of non consanguineous marriage presented with bilateral cataracts since 10 months. Birth history and milestones were normal. There was no jaundice, failure to thrive or dysmorphic features. On examination, apart from bilateral white reflex, other systems were normal.

Question :

How to approach such a case_?

Answer 

Causes of cataracts in a child are varied and include intrauterine infections, genetic disorders, metabolic disorders, hypoparathyroidism and even prematurity. This child has no prematurity, dysmorphic features or delayed milestones. 

Thus, most likely cause of cataract in this child would be metabolic disorder. Common metabolic disorders leading to white cataract are:

 • Hypoparathyroidism 

• Galactosemia 

• Lowe’s syndrome 

• Diabetes mellitus

 In this child, since there is no jaundice or hepatomegaly, galactosemia type 1 and 3 seem unlikely. 

Lowe’s syndrome is associated with RTA and mental retardation. 

Diabetes mellitus would have additional features of polyuria, polydipsia. Thus in this child, one must rule out Hypoparathyroidism. 

The calcium, phosphorus, alkaline phosphatase in this child was normal. Galactosemia type 2 is a possibility and one must do the galactokinase enzyme levels.

 Galactosemia workup for galactokinase deficiency was positive.

MCQs In Muscular diseases

Q1 :

A 6-year-old boy has had difficulty walking and lower leg pain for 2 days. Five days ago, he had

fever and cough that had lasted for 3 days. On physical examination, the child has no fever, and

vital signs are normal, as are cranial nerves, speech, and language. Muscle bulk, tone, and

reflexes are normal and symmetric, but his lower legs are painful to palpation. Serum creatine

kinase is 2,000 U/L, and urine is negative for myoglobin.

Of the following, the MOST likely diagnosis is

A. dermatomyositis

B. Duchenne muscular dystrophy

C. Guillain-Barré syndrome

D. metabolic myopathy

E. viral myositis

Answer

E


A gait disturbance, such as described for the boy in the vignette, can result from a variety of
potentially serious disease processes and, therefore, requires urgent evaluation. The muscle
pain and otherwise normal neurologic findings prompted measurement of serum creatine kinase,
which can help to localize the problem rapidly. The prodrome of an upper respiratory tract illness
and rapid onset of symptoms is suggestive of viral myositis.

Dermatomyositis is a more indolent, chronic process and should not present acutely with
muscle pain. Moreover, dermatomyositis is characterized by specific skin findings such as the
heliotrope rash over the eyelids and Gottron papules .

 Duchenne muscular dystrophy presents with more chronic weakness. Although Guillain-Barré syndrome
can present with pain and weakness, the preserved reflexes, focal pain over the leg muscles, and elevated creatine kinase value are not consistent with that diagnosis. Metabolic myopathies due to mitochondrial dysfunction can present with acute pain, weakness, tender muscles, and rhabdomyolysis. However, the prevalence is much lower than viral myositis. Influenza A and B and enteroviruses may cause viral myositis.

MCQs In Pediatric Cardiology Part II

Q 1:

You are evaluating a 12-year-old boy in the emergency department who presents with fever,

chills, malaise, and blood in his urine. On physical examination, he appears comfortable and alert

and has a temperature of 102.7°F (39.3°C), a blood pressure of 110/40 mm Hg, no rashes, and

clear breath sounds. He has a diastolic murmur heard best in the sitting position (Item Q133).

You elicit no abdominal or flank tenderness.

Of the following, the BEST next step in the management of this patient is

A. administration of broad-spectrum antibiotics

B. blood cultures

C. renal ultrasonography

D. transesophageal echocardiography

E. urine culture

Answer

B

The patient described in the vignette has history and physical examination findings that are
highly suggestive of infective endocarditis. These include symptoms of chills and malaise; a
history of fever; and the findings of hematuria, a new murmur, and fever. Typically, the diagnosis
is confirmed by isolation of the offending organism from blood cultures. Blood cultures from three
to five sites should be obtained prior to initiation of antibiotic therapy. Because the bacterial
shedding is constant, the practitioner should not wait until the patient is febrile to obtain blood
cultures. Viridans streptococci (eg, S bovis, S mitis) as well as Staphylococcus aureus are the
most common bacterial pathogens causing endocarditis in children. However, clinicians must be
concerned about organisms such as Enterococcus, coagulase-negative Staphylococcus, fungi,
and a group of bacteria referred to as the HACEK organisms (Haemophilus sp, Actinobacillus
actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella kingae).

The HACEK organisms are gram-negative oral and pharyngeal flora that are fastidious and slow growing,
often requiring growth factors and carbon dioxide to be isolated in cultures.

Treatment of endocarditis depends on the isolated organism. In general, long-term antibiotic
treatment (4 to 6 weeks) is undertaken in an effort to eradicate completely the bacteria that have
been sequestered in a nonvascular vegetation. Surgery is reserved for patients who develop
severe congestive heart failure from severe valve regurgitation or deterioration.

The boy in the vignette requires intravenous antibiotic treatment, but blood cultures should
be obtained before therapy is begun. He also should undergo echocardiography, which may be
performed from the transesophageal approach to improve the sensitivity, but similar to renal
ultrasonography, such a study is performed after blood cultures have been obtained. The
absence of vegetation at the time of echocardiography does not rule out a diagnosis of infective
endocarditis. Patients who have infective endocarditis may exhibit hematuria from the deposition
of immune complexes resulting in glomerulonephritis. Although fever and hematuria may be
associated with urinary tract infection, the presence of a diastolic murmur and absence of
urinary symptoms make such a diagnosis unlikely.

------

Q2:

 A 5-year-old full term male infant was severely cyanotic at birth. Prostaglandin E was administered
initially and later balloon atrial septostomy was done which showed improvement in oxygenation. The most
likely diagnosis of this infant is:

a. Tetralogy of Fallot
b. Transposition of great vessels
c. Truncus arteriosis
d. Tricuspid atresia
e. PDA


Answer

B

Q 3:

Which one of the following CHD has cyanosis without cardiomegaly and/or CCF?

a. TGV
b. TOF
c. Congenital MR
d. Congenital PS


Answer

B

Q 4:

One-year-old child with PDA; which is true:

a. Symptoms are similar to aortopulmonary window
b. Chances of spontaneous closure is high
c. Indomethacin may help in closure
d. Endocarditis is rare

Answer

A

Q5:

An 8-yr-old male child is admitted with a diagnosis of rheumatic fever with arthritis, carditis and CCF, with
reference to this case, consider the following as initial lines of management:

a. Eradication of remnant streptococcal infection
b. Administration of an anti-inflammatory drug
c. Institution of decongestive therapy
d. Institution of graded and gradually increasing exercise

Answer

C

Q6:

 A young boy had developed CCF, found to have membraneous VSD. He showed spontaneous improvement. This is most likely due to:

a. Perimembraneous closure of VSD
b. Development of AR
c. Pulmonary vascular changes
d. Infective endocarditis

Answer

C


---------------------------

Q 7

A full-term newborn develops cyanosis a few hours after birth. Oxygen administration does
not improve color or oxygen saturations. Which of the following is the most likely diagnosis?

(A) atrial septal defect
(B) ventricular septal defect
(C) patent ductus arteriosus
(D) aortic stenosis
(E) pulmonary stenosis

MCQs In Pediatric Toxicology

Q1 : 

A resident in continuity clinic approaches you to review the laboratory values obtained at a

patient’s 12-month health supervision visit. The fingerstick hemoglobin measurement was 10.5

g/dL (105.0 g/L), and the lead concentration was 11.0 mcg/dL (0.53 mcmol/L).

Of the following, the next BEST step for this boy is to

A. admit him to the hospital for chelation therapy

B. call child protective services to move him to a shelter

C. call the health department to arrange for an environmental investigation

D. measure the venous lead concentration

E. refer him for formal developmental evaluation and neuropsychometric testing

Answer:

D

The American Academy of Pediatrics policy statement recommends the use of venous
samples for initial screening whenever possible. If capillary testing is performed and the lead
concentration is greater than 10.0 mcg/dL (0.5 mcmol/L), the lead concentration must be
confirmed by a venous sample because microlead sampling is more likely to yield false-positive
results due to contamination from environmental lead.

Lead ingestion may cause a microcytic anemia by interfering with iron absorption and
utilization in heme production and can inhibit enzymes required for heme synthesis directly.
Children who have lead poisoning may have pica either as a cause or symptom of lead
poisoning. In these children, iron supplementation should be initiated until the presence or
absence of iron deficiency is determined.

Most asymptomatic children who have mildly elevated blood lead concentrations are not
candidates for chelation therapy with the currently available drugs because the toxicity of these
drugs outweighs the potential benefit of treatment, and chelation is unlikely to increase lead
excretion significantly. Chelation therapy should be considered, however, if lead concentrations
are higher than 44.0 mcg/dL (2.12 mcmol/L). The role of chelation is not clearly defined for
children whose blood lead concentrations range from 20.0 to 45.0 mcg/dL (0.97 to 2.17
mcmol/L). In this range, the clinician may choose to pursue further environmental screening,
attempt to eradicate lead from the child's environment, and measure blood lead concentrations
monthly. If the concentration remains in this range, despite successful eradication of the lead
source, the physician should institute behavior modification, nutritional sufficiency, or chelation
treatment.
Succimer is the drug of choice for children whose blood lead concentrations are 45.0 to
100.0 mcg/dL (2.17 to 4.8 mcmol/L). At values higher than 69.0 mcg/dL (3.3 mcmol/L), a second
drug, CaNa2EDTA, is added.

Blood lead concentrations fall precipitously after completion of chelation, but rebound within
weeks, even if there is no further exposure to lead, due to release of lead from bone stores. In
general, the concentrations do not return to the high values seen before chelation, and a second
course of chelation rarely is indicated.