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1. An exclusively breast fed 2 year old boy is brought to the emergency center with pain in his right leg after a fall. Physical examination reveals a small child with a 3 cm anterior fontanelle, a flattened occiput, a prominent forehead, significant dental caries, bumpy ribs, and bowed extremities....

1. An exclusively breast fed 2 year old boy is brought to the emergency center with pain in his right leg after a fall. Physical examination reveals a small child with a 3 cm anterior fontanelle, a flattened occiput, a prominent forehead, significant dental caries, bumpy ribs, and bowed extremities. Radiographs reveal a greenstick fracture at the site of pain, along with fraying at the distal ends of the femur. Choose the one most appropriate vitamin or trace element replacement therapy to treat the described condition. a. Vitamin A b. Vitamin B6 c. Vitamin C d. Vitamin D e. Vitamin E 2. A mother wishes to breast feed her newborn infant, but is worried about medical conditions that would prohibit her from doing so. You counsel her that of her listed conditions, which of the following is a contraindication to breast feeding? Select one: a. HIV infection b. Upper respiratory tract infection c. Mastitis d. Cracked and bleeding nipples 3. The most common cause of neonatal seizures is: a. Vascular events b. Intracranial infections c. Brain malformations d. Hypoxic ischemic encephalopathy e. Metabolic disturbances 4. At 43 weeks gestation, a long, thin infant is delivered. The infant is apneic, limp, pale, and covered with ‘’pea soup’’ amniotic fluid. Which of the following is the best first step in the resuscitation of this infant at delivery? a. Intubation and suction of the trachea; provision of oxygen b. Artificial ventilation with bag and mask c. Chest compressions d. Administration of 100% oxygen by mask e. Catheterization of the umbilical vein 5. A nursing mother asks if her 3 month old baby requires water supplementation select one: a. Need additional water 200ml/day b. No additional H2O needed c. Need additional water 10ml/kg day 6. The mainstay of the initial treatment of this disease is aggressive therapy with normal saline, often in conjunction with a loop diuretic to further increase calcium excretion. What disease are we talking about? a. Distal renal tubular acidosis b. Dent disease c. Hypervitaminosis D d. Autosomal recessive hypophosphatemic rickets 7. A 10 month old child can do all the following except: a. Follows one step command without gesture b. Says mama or dada c. Points to objects d. Speaks first real word e. Inhibition to ‘’no’’ 8. A newborn infant at 5 min after birth have blue extremities, a heart rate of 85, no response to stimulation, flexion, with irregular respiration. What is her Apgar score? Select one: a. 4 b. 5 c. 3 d. 6 9. Which of the following clinical features listed below is not helpful in cases of bilateral DDH a. Wide perineum b. Bilateral positive klisic test c. Hyperlordosis d. Asymmetry of the thigh folds 10. The imaginary line between the anterior superior iliac spine and the greater trochanter is: a. Shenton line b. Perkins line c. Klisic line d. Hilgenreiner line 11. The 15 years girl, 45 Kg, applied to the emergence department with complaints of swelling of the face and lips, shortness of breath, a feeling of suffocation, dry hoarseness that developed after eating a peanut-containing sandwich. The patient was treated appropriately, the pathological symptoms resolved. How long should patient stay in hospital after treatment of anaphylactic shock? Select one: a. At least 1 hr b. At least 6 hr c. At least 2 hr d. At least 4 hr 12. The child walks alone, crawls upstairs, makes tower of 3 cubes, makes a line with crayon, follows simple commands, responds to his/her name, hugs parents. How old is he/she? a. 15 MO b. 18 MO c. 24 MO d. 28 MO 13. What is the diagnostic modality of choice for DDH (developmental dysplasia of the hip) after four to six months of age? Select one: a. MRI b. Xray c. CT d. US 14. Choose correct answer: if someone only has one X chromosome and no Y chromosomes in their somatic cells, they: a. Are metafemales b. Have Turner syndrome c. Have Klinefelter syndrome 15. A term neonate is examined following a protracted breech delivery. A complete neurologic examination reveals an asymmetrical Moro reflex, with the left arm being nonreactive. The left arm falls limply and close to the side of the body when drawn upward, and the resting position is adducted and internally rotated with pronation of the forearm. The neonate’s finger show a normal response to the hand grasp reflex bilaterally. Vital signs are within normal limits, and no other abnormalities are noted on examination. Which of the following is the most likely diagnosis? Select one: a. Pseudobulbar palsy b. Erb palsy c. Spinal cord injury d. Klumpke paralysis e. Bell palsy 16. A single umbilical artery in a newborn infant increases the risk of which of the following? a. Meningomyelocele b. Occult renal anomaly c. Omphalocele d. Gastroschisis e. Omphalitis 17. A newborn has a flat, salmon-colored lesion on the glabella, which becomes darker red when he cries. What is your diagnosis? Select one:  a. Neurocutaneous phakomatosis b. Nevus sebaceous (of Jadassohn) c. Salmon patch, stork bite or angel kiss d. Mongolian spots 18. Of the following, the findings that are most suggestive of DiGeorge syndrome are: a. Cataract and a congenital heart defect b. Hypotonia and a congenital heart defect c. Cutis aplasia and a congenital heart defect d. Hypocalcemia and a congenital heart defect e. Mongoloid slant to the eyes and a congenital heart defect 19. Common skin and soft tissue manifestation of neonatal bacterial infection are: a. Facial cellulitis b. Scalp abscess c. Fasciitis d. Breast abscess e. Omphalitis 20. Jaundice associated with breast-feeding occurs in a newborn select one: a. At 2nd 3rd day of life b. 3 days later c. At birth d. After the 7th day of life 21. The earliest clinical feature of ataxia-telangiectasis is: a. Ataxia b. Jaundice c. Inability to walk d. Ocular telangiectasis e. Cutaneous telangiectasis 22. Which of the following is the main risk factor for the development of severe jaundice? a. Gestational age 37-38 wk b. Jaundice observed before discharge c. Blood group incompatibility with positive direct antiglobulin test d. Macrosomic infant of a diabetic mother e. Maternal age >25 year 23. Risk factors for development severe hyperbilirubinemia in infants are all the following except: a. Jaundice observed in the first 24 hr b. Cephalohematoma or significant bruising c. Exclusive breast-feeding d. Discharge from hospital after 72 hr 24. This is an infant reflex, that develops between 28-32 weeks of gestation and disappears between 3-6 months of age. It is a response to a sudden loss of support and involves three distinct components: spreading out the arms, pulling the arms in and crying. What is this reflex called? Select one: a. Asymmetric tonic neck reflex b. Palmar grasp reflex c. Rooting reflex d. Moro reflex e. Thumb-finger grasp 25. All the following are features of ataxia-telangiectasis except: a. Hepatic manifestation b. High incidence of malignancy c. Chronic sinopulmonary disease d. Oculocutaneous telangiectasis e. Variable humoral and cellular immunodeficiency 26. When baby’s head turned to one side, the arm on that side stretches out and the opposite arm bends up at the elbow. What is this reflex called? Select one: a. Asymmetric tonic neck reflex b. Rooting reflex c. Moro reflex d. Thumb-finger grasp e. Palmar grasp reflex 27. A term neonate is delivered via normal spontaneous delivery without complication. Her mother is HBsAg-positive. Your recommendation is: select one: a. Using goat’s milk instead of breast milk b. Supplement her breast-feeding with a multivitamin c. No delay in initiation of breast-feeding is required d. Discontinue breast-feeding and initiate soy formula feedings 28. In addition to infection and bleeding, the most common cause of death in Wiskott-Aldrich syndrome is: a. Renal failure b. Graft versus host disease c. Protracted bloody diarrhea d. EBV-associated malignancies e. Development of autoimmune disease 29. In the case of X-linked dominant inheritance: a. Males are more frequently affected, affected males often present in each generation b. Both parents of an affected person are carriers; not typically seen in every generation  c. Females are more frequently affected because all daughters and no sons of an affected man will be affected d. Each affected person usually has an affected parent; occurs in every generation 30. Vaccines are defined as whole parts of microorganisms administered to prevent an infectious disease. Which of the following is a live attenuated vaccine? a. Hepatitis A b. Hepatitis B c. Pneumococcal d. Varicella e. Diphtheria 31. A 38 week, 7lb2oz baby boy is born by spontaneous vaginal delivery. At 10 mins he has a heart rate over 100, has a strong cough and cry, is pink all over and continues to move all limbs equally. What is his Apgar score at 10 minutes? Select one: a. 7 b. 6 c. 9 d. 10 e. 8 32. A newborn infant at 1 min after birth have blue extremities, a heart rate of 77, no response to stimulation, is limp with irregular respiration. What is her Apgar score? Select one: a. 5 b. 6 c. 3 d. 7 33. A 17-month-old girl presented with history of recurrent attacks of pneumonia since the age 8 months. You suspect predominant B-cell defect. Of the following the best simple initial screening test is: a. IgA measurement b. IgG measurement c. IgM measurement d. IgE measurement e. Isohemagglutinin titer 34. Which of the following diseases is characterized by an incubation period from 12 hr to 7 days? a. Rubella b. Inf. Mononucleosis c. Chickenpox d. Scarlet fever 35. The preferable antibiotic in neonate with pertussis is:  a. Azithromycin b. Erythromycin c. Clarithromycin d. Trimethoprim-sulfamethoxazole e. Amoxycillin 36. Which of the following vaccines is given via IM route? a. BCG b. MMR c. Polio 37. Which of the following diseases is not characterized by X-link inherited? a. Brutton agammaglobulinemia b. IPEX syndrome c. Wiskoltt Aldrich syndrome d. DiGeorge syndrome 38. After institution of treatment of anaphylaxis, the patient should be monitored in the emergency room for at least a. 1 hour b. 4 hours c. 6 hours d. 12 hours e. 24 hours 39. In case of food-associated anaphylaxis, the parents are advised that children must not take exercise after ingestion of the triggering food for a. 2-3 hours b. 4-6 hours c. 8-42 hours d. 13-18 hours e. 19-24 hours 40. A newborn has hydrocephalus, chorioretinitis, intracranial calcifications and anemia. Select the most likely diagnosis a. Congenital cytomegalovirus b. Congenital syphilis c. Congenital herpes simplex virus d. Congenital rubella e. Congenital toxoplasmosis 41. Unpredictable drug reactions include a. Dose dependent b. Drug toxicity c. Drug interactions d. Adverse effects e. Allergic reaction 42. Perioral rash, pigmentation of skin creases and neuritis is seen in deficiency of: a. Zinc b. Copper c. Magnesium d. Biotin (vitamin B7) 43. Frozen breast milk can be used up to: a. 48 hr b. 72 hr c. 4 mo d. 6 mo e. 1 yr 44. Cranial CT is a valuable diagnostic tool in the evaluation of the following conditions except: a. Skull fractures b. Intracranial hemorrhages c. Acute infarcts d. Hydrocephalus e. Impending herniation 45. Vitamin D deficiency is caused by decreased absorption from the gastrointestinal tract because of: select one: a. All of them are correct b. Malabsorption c. Celiac disease d. Gastrectomy e. Inflammatory bowel disease 46. The father of a 1 week old infant comes to the office in a panic. He has just noticed on his child a right anterior shoulder mass that seems tender. The father is an osteosarcoma survivor and fears the child has the same malignancy. In reviewing the baby’s discharge papers, you note the child was a term, appropriate for gestational age vaginal delivery with a birth weight of 3200g (7lb, 1oz). Apgar scores were 9 at 1 and 5 minutes. Your examination is significant for a large firm mass on the right clavicle; the rest of the examination is normal. Management of this problem should include which of the following? a. Magnetic resonance imaging of the right shoulder b. Reassurance and supportive care c. A biopsy of the mass for culture and cytology d. Referral to an orthopedic surgeon e. Skin biopsy to test for osteogenesis imperfecta 47. A 4-year-old whose diet consists mostly of cheese puffs and cola begins to have problems walking at night, complaining that he cannot see well. In addition, his skin has become dry and scaly, and he has complained of headache for a month. Choose the one most appropriate vitamin or trace element replacement therapy to treat the described condition. Select one: a. Vitamin A b. Vitamin D c. Vitamin E d. Vitamin B6 e. Vitamin C 48. In the case of hip dislocation, the imaginary klisic line point: a. Toward the umbilicus b. Above the umbilicus c. Below the umbilicus 49. A 10-year-old patient calls his parents from summer camp to state that he has had fever, muscular pain (especially in the neck), headache, and malaise. He describes the area from the back of his mandible toward the mastoid space as being full and tender and that his earlobe on the affected side appears to be sticking upward and outward. Drinking sour liquids causes much pain in the affected area. When his father calls your office, you remind him that he had refused immunizations for his child on religious grounds. Which of the following preventable diseases has his child acquired? a. Mumps b. Varicella c. Rubella d. Measles e. Diphtheria 50. A term neonate is delivered via normal spontaneous delivery without complication. Within the first 2 hours of life, he become tachypneic, with occasional apneas and two seizures episodes. The infant is large for gestational age and his weight is in the 95th percentile for his age. There are no evident dysmorphic features and the rest of the physical examination is unremarkable. Serum studies demonstrate a blood glucose level of 30mg/dl. Which of the following conditions is most likely present in the mother? a. Diabetes mellitus b. Graves disease c. Hepatic cirrhosis d. Rheumatoid arthritis e. Seizure disorder f. Systemic lupus erythematous (SLE) 51. The most reliable sign of a dislocated hip after the 2nd month of life is: a. Limited hip abduction b. Apparent shortening of the thigh c. Proximal location of the greater trochanter d. Asymmetry of the gluteal or thigh folds e. Positive ortolani test 52. The boy calls you 10 days after you saw him in your office and diagnosed him with mononucleosis. He now has a fever of 39.5, cannot swallow, and feels that he cannot open his mouth easily. You see him hours later in the office and you note that his voice is quite muffled. On examination his temperature is 38.4 (he took acetaminophen 2 hours prior). He cannot open his mouth wide, but on minimal opening you note white exudates covering both tonsils, marked tonsillar enlargement, right greater than left and a visible buble at the superior aspect of the right tonsil, uvula is deviated opposite to pathologic site. What is his diagnosis? a. Secondary strep pharyngitis b. Peritonsilar abscess c. Retropharyngeal abscess d. Mucoplasma pneumoniae cellulitis 53. Choose correct answer: for infants after four to six months of age is recommended to obtain: a. Xray b. CT c. MRI d. US 54. Anterior fontanelle closes at the age of: a. 1 year b. 2 years c. 3 years d. 4 years 55. The hallmark of refeeding syndrome is the development of severe select one: a. Hypercalcemia b. Hyperphosphatemia c. Hypophosphatemia d. Hyponatremia e. Hypernatremia 56. An absolute contraindication to breast-feeding is select one: a. Active pulmonary tuberculosis b. Hepatitis C infection of the mother c. Herpes simplex lesion of the lips d. Maternal high grade fever e. Alcohol intake 57. Which is the primary tooth that erupts into the oral cavity select one: a. The primary upper central incisors b. The lower primary central incisors c. The primary incisors d. The primary lower incisors 58. How many teeth does a three year old child have? Select one: a. 20 b. 28 c. 16 d. 24 59. Which of the following is fine motor skill? Select one: a. Sits without support b. Brings hands together in midline c. Transfers object hand to hand d. Rolls back to stomach 60. The composition of breast milk per 100 ml: a. 67 calories, 3.5g protein, 3.5g fat and 4.5g carbohydrate b. 67 calories, 1.2g protein, 4g fat and 4.5g carbohydrate c. 67 calories, 1.2g protein, 4g fat and 7g carbohydrate d. 100 calories, 3.5g protein, 4g fat and 7g carbohydrate e. 100 calories, 3.5g protein, 3.5g fat and 4.5g carbohydrate 61. A characteristic symptom of this disease is excessive sleeping, a large tongue, puffy face, larger- than-normal fontanelles on the head, prolonged jaundice after birth, weak cry. Select one: a. Galactosemia b. Maple syrup urine disease c. Congenital hypothyroidism d. Phenylketonuria 62. The cataract, congenital heart disease, nerve deafness are characteristic feature for: select one: a. Congenital rubella syndrome b. Congenital varicella syndrome c. Toxoplasmosis d. HIV 63. A 12-year-old boy comes into your office complaining of sore throat of 1 week’s duration. It is getting worse by the day, and this morning he felt as if he could not swallow his breakfast, both because of the pain on swallowing and because it felt as if something was blocking it down there. He has been feeling hot and cold but has not measured his temperature. He is feeling fatigued, but the rest of his review of system is negative. He has had multiple episodes of tonsillitis this year. On examination, he is cooperative but appears quit tired. His temperature is 38.6. the other vital signs are normal. His conjunctivae are clear, tympanic membrane are normal, and oropharynx is quite erythematous, with enlarged erythematous tonsils covered by diffuse white exudates bilaterally. No petechiae are seen. His lymphatic exam reveals anterior and posterior cervical lymphadenopathy, all 1 to 2.5 cm in diameter, mobile, and moderately tender. His lungs are clear. The abdominal examination reveals a palpable spleen tip 4 cm under the rib cage. There is no rash. What is his most likely diagnosis? Select one: a. Viral upper respiratory tract infection b. Mononucleosis c. Mumps 64. One of the listed signs are not characteristic feature for rubella select one: a. Incubation period of 14-21 days b. Suboccipital lymph nodes enlargement c. Forchheimer spots d. Koplik spots 65. 6-month-old infant has been exclusively fed a commercially available infant formula. Upon introduction of fruit juices, however, the child develops jaundice, hepatomegaly, vomiting, lethargy, irritability, and seizures. Tests for urine-reducing substances are positive. Which of the following is likely to explain this child’s condition? a. Tyrosinemia b. Galactosemia c. Hereditary fructose intolerance d. Alpha 1 antitrypsin deficiency e. Glucose-6-phosphatase deficiency 66. The eczema and musty odor are a characteristic feature of: select one: a. Galactosemia b. Congenital hypothyroidism c. Maple syrup urine disease d. Phenylketonuria 67. The major clinical significance of this disease is transplacental infection and fetal damage, suboccipital, postauricular cervical lymph nodes are most prominent, the duration of the rash is generally 3 days, and usually resolves without desquamation. What disease are you suspect? Select one: a. Mumps b. Chickenpox c. Rubella d. Scarlet fever 68. The disease begins acutely with: - fever of 38.3 or higher, often with chills - very sore and red throat, sometimes with white or yellowish patches - difficulty swallowing - enlarged lymph nodes in the neck that are tender to the touch - red rash. The rash looks like a sunburn and feels like sandpaper. It typically begins on the face or neck and spreads to the trunk, arms and legs. If pressure is applied to the reddened skin, it will turn pale. - red lines. The folds of skin around the groin, armpits, elbows, knees and neck usually become a deeper red than the surrounding rash. - flushed face. The face may appear flushed with a pale ring around the mouth. - strawberry tongue. The tongue generally looks red and bumpy, and it’s often covered with a white coating early in the disease. What disease do you suspect? Select one: a. Scarlet fever b. Rubella c. Chickenpox d. Measles 69. Which is a minor risk factor for developing severe hyperbilirubinemia? Select one: a. Previous sibling with jaundice b. Exclusive breast-feeding, particularly if nursing is not going well and weight loss is excessive c. Jaundice observed in the first 24 hr d. Gestation age 35-36 wk e. Cephalohematoma 70. The girl is 7 years old. The anamnesis shows that she was taking antibiotics and antiinflammatory drugs due to monoarthritis. The child has a fever, signs of a flu-like symptoms for the last five days, bullous and erosive lesions involve oral, ocular, and genital mucosae and vast areas of the skin with detachments more than 30% of the body surface area. What disease do you suspect? Select one: a. Steven-Johnson syndrome and toxic epidermal necrolysis b. Toxic epidermal necrolysis c. Steven-Johnson syndrome 71. Breast milk stored at room temperature stored for: a. 4 hrs b. 8 hrs c. 12 hrs d. 24 hrs 72. Which of the following diseases is characterized by X-link inherited? a. Cartilage hair hypoplasia  b. Chediak-Higashi syndrome c. Duncan disease d. Ataxia-telangiactasis 73. A mentally retarded 14-year-old boy has a long face, large ears, micropenis, and large testes. Chromosome analysis is likely to demonstrate which of the following? Select one: a. Trisomy 18 b. Fragile X syndrome c. Trisomy 13 d. Williams syndrome e. Trisomy 21 74. A newborn infant is noted to have microcephaly with sloping forehead, cutis aplasia on the scalp, microphthalmia, and cleft lip and palate. His echocardiogram demonstrates a complex heart lesion including atrial septal defect (ASD), ventricular septal defect (VSD), and dextrocardia. What disease do you suspect? Select one: a. Down syndrome b. Cri Du Chat syndrome c. Trisomy 13 d. Angelman syndrome 75. You have an 11-day-old term infant in your office for a well-child visit. The mother notes that she received a letter that day from the state’s department of health reporting that her child’s newborn screen had come back abnormal, indicating possible galactosemia. Which of the following is the most appropriate management at this point? Select one: a. Refer to endocrinology for evaluation b. Supplement her breast-feeding with a multivitamin c. Discontinue oral feeds and begin total parenteral malnutrition d. Ultrasound of pancreas e. Discontinue breast-feeding and initiate soy formula feedings 76. A newborn infant at birth is noted to have acrocyanosis, a heart rate of 140, grimaces to stimulation and is active with a lusty cry. What is her Apgar score? a. 9 b. 8 c. 6 d. 7 77. A 38 week, 3.4 Kg baby boy is born by spontaneous vaginal delivery. At one minute he has a heart rate over 100, is crying with grimace and is moving all slightly blue limbs equally. What is his Apgar score at 1 minute? Select one:  a. 8 b. 9 c. 7 d. 10 e. 6 78. What are the assessment categories of the Apgar scoring system that assists in newborn resuscitation management? Select one: a. Appearance, poop, grunt, aspastic, heart rate b. Afebrile, pulse, grunt, aspartic, respirations c. Activity, poop, grin, appearance, heart rate d. Appearance, pulse, grimace, activity, respirations e. Activity, posture, good color, afebrile, heart rate 79. A newborn infant at 5 min after birth have blue extremities, a heart rate of 85, no response to stimulation, flexion, with absent of repiration. What is her Apgar score? Select one: a. 6 b. 3 c. 4 d. 5 e. 7 80. How long does infection with EBV last? a. 1 month b. 6 months c. 1 year d. 2 years e. Lifelong 81. A 38 week, 7lb2oz baby boy is born by spontaneous vaginal delivery. At 5 mins he has a heart rate over 100, is crying with grimace, is pink all over and continues to move all limbs equally. What is his Apgar score at 5 minutes? Select one: a. 7 b. 6 c. 10 d. 9 e. 8 82. You have been recently named as the medical director of the normal newborn nursery in your community hospital and have been asked to write standardized admission orders for all pediatricians to follow. Which of the following vaccines will you include on these orders? a. Hepatitis A vaccine b. Hepatitis B vaccine  c. Combination diphtheria, tetanus, and acellular pertussis vaccine d. Inactivated polio virus e. Haemophilus influenza B vaccine 83. Under which age does the monospote test to detect heterophile antibody lose its sensitivity? a. 1 year b. 2 years c. 4 years d. 8 years e. 10 years 84. Which fraction of bilirubin crosses the renal glomerular barrier and is detected in the urine? a. Conjugated bilirubin b. Unconjugated bilirubin c. Both of them 85. A 36 week, 5lb7oz baby girl is born by cesarian section. At 5 minutes she has irregular respirations with coughing, peripheral cyanosis, some flexion, and her heart rate is over 100. What is her 5 minute Apgar score? Select one: a. 6 b. 8 c. 7 d. 10 e. 9 86. A 9-month-old boy presented with recurrent attacks of diarrhea, thrush, and failure to thrive shortly after birth. You suspect immune deficiency. Of the following, the most common cause of this condition is: a. Hyper-IgE syndrome b. Defect in phagocytic cells c. Deficiencies in T-cell function d. Defect in antibody production e. Defect in complement proteins 87. A newborn infant has a blue-gray pigmented lesion on the sacral area. It is clearly demarcated and does not fade into the surrounding skin. What is the most likely diagnosis? Select one: a. Salmon patch, stork bite or angel kiss b. Nevus sebaceous (of Jadassohn) c. Mongolian spots d. Port wine stains 88. In the case of autosomal dominant inheritance: a. Males are more frequently affected, affected males often present in each generation b. Both parents of an affected person are carriers; not typically seen in every generation  c. Females are more frequently affected because all daughters and no sons of an affected man will be affected d. Each affected person usually has an affected parent; occurs in every generation 89. Which fraction of serum bilirubin can passage across the blood-brain barrier? a. Free or unbound bilirubin b. Conjugated bilirubin c. Fraction d. Unconjugated bilirubin 90. Excess accumulation which bilirubin fraction in the body causes intense itching of the skin? a. Free or unbound bilirubin b. Conjugated bilirubin c. Fraction d. Unconjugated bilirubin 91. All of the following statements are true, except: a. Kernicterus occurred only in infants with a bilirubin level >20mg/dl b. Kernicterus is a neurologic syndrome resulting from the deposition of conjugated bilirubin in the basal ganglia and brainstem nuclei c. Hyperbilirubinemia may lead to encephalopathy at any time during the neonatal period d. In the 2 phase of disease hypertonia of extensor muscles, opisthotonos, retrocollis occue 92. Which of the following is false regarding phototherapy? a. Dark skin does not reduce the efficacy of phototherapy b. Phototherapy and, if it is unsuccessful, exchange transfusion remain the primary treatment modalities used to keep the maximal total serum bilirubin below pathologic levels c. The development of bronze Man syndrome is an indication of immediate cessation of phototherapy d. If the serum bilirubin level exceeds 20mg, phototherapy should be continued for more than 49-72 hours 93. Regarding breast milk jaundice, the following are true except: a. It develops in an estimated 2% of breast-fed term infants b. Maximal unconjugated bilirubin concentrations as high as 10-30mg/dl reached during the 2nd-3rd week c. Jaundice may persist for 3-10 weeks d. Phototherapy may be of benefit e. Kernicterus never occur 94. A 4-year-old boy born to immigrant parents comes to your clinic. He presents with the following symptoms: sensorineural hearing loss, supernuclear ophthalmoplegia, enamel hypoplasia, and choreoathetosis. The most likely cause of his symptoms is which of the following? a. Stoke b. Congenital infection with cytomegalovirus c. Congenital infection with rubella d. Kernicterus  e. Lissencephaly 95. The following factors are associated with decreased risk of significant jaundice except: a. Gestational age 41 week b. Male gender c. Exclusive bottlefeeding d. Black race e. Discharge from hospital after 72 hr 96. Jaundice, consisting of either indirect or direct bilirubin that is present at birth or appears within the 1st 24 hours of life may be due to all the following except: a. Erythroblastosis fetalis b. Criggler Najjar syndrome c. Concealed hemorrhage d. Sepsis e. Congenital infections 97. A search to determine the cause of jaundice should be made in all the following conditions except: a. If it appears in the first 24-36 hr of life b. If serum bilirubin is rising at a rate faster than 5mg/dl/24 hr c. If serum bilirubin is >12 mg/dl in a fullterm infant d. If jaundice persists after 10-14 days of life e. If direct bilirubin fraction is > mg/dl at any time 98. The therapeutic effect of phototherapy depends on the following factors except: a. Distance between the light and the infant b. Skin color c. Surface area of exposed skin d. Rate of hemolysis e. In vivo metabolism and excretion of bilirubin 99. Early ultrasonographic signs of hydrops include: a. Double-bowel wall sign (bowel edema) b. Lehydramnios c. Ascites d. Pleural effusions e. Scalp edema 100. The blood smear of infant with hemolytic disease of the newborn (erythroblastosis fetalis) typically shows the following except: a. Polychromasia b. Increased reticulocyte count c. A marked increase in nucleated RBCs d. Thrombocytopenia e. Low white blood cell count 101. One of the following is false in hemolytic disease of the newborn caused by blood group A and B incompatibility: a. ABO incompatibility occurs in 20-25% of pregnancies b. A1 is more antigenic than A2 c. Natural antibodies against A and B factors are usually IgM antibodies that do not cross the placenta d. Isoimmune hemolytic disease may be found in first-born infants irrespective of infant blood group e. Most cases are mild 102. One of the following is false in hemolytic disease of newborn caused by Rh incompatibility a. Infant is Rh positive b. Never occur in first born infant c. Maternal antibody titers can help predict the severity of fetal disease d. Severe anemia is frequent 103. One of the following is false in plethora in the newborn infant (polycytemia): a. Polycytemia is defined as central Hct of 65% or higher b. Peripheral (heelstick) Hct and Coulter counter results values are higher than central values c. The incidence of neonatal polycytemia is 8% in small for gestational age (SGA) infants d. Many affected infants are asymptomatic e. Neonatal RBCs have decreased deformability and filterability, which predispose to stasis in the microcirculation 104. Regarding hemorrhagic disease of the newborn, all the following are true except: a. Age of onset is usually in 2-7 days old infants b. More frequent in breast-fed than in formula-fed infants c. Oral vitamin K is as effective as intramuscular route in the prevention and treatment of the disease d. Prothrombin time (PT) and partial thromboplastin time (PTT) are prolonged 105. Erythroblastosis fetalis is characterized by all of the following excpet: a. Elevated amniotic fluid bilirubin levels b. Fetal anasarca c. Decreased umbilical cord nucleated red blood cells d. Fetal hepatosplenomegaly 106. The differential diagnosis of a neonate with hemolytic anemia includes all of the following except: a. Pyruvate kinase deficiency b. ABO incompatibilty c. Criggler Najjar syndrome d. Hereditary spherocytosis 107. A 1-day-old infant who was born by a difficult forceps delivery is alert and active. She does not move her left arm spontaneously or during a Moro reflex. Rather, she prefers to maintain it internally rotated by her side with the forearm extended and pronated. The rest of her physical examination is normal. This clinical scenario most likely indicates which of the following? a. Fracture of the left clavicle b. Fracture of the left humerus c. Left-sided Erb-Duchenne paralysis d. Left-sided Klumpke paralysis e. Spinal injury with left hemiparesis 108. Your older sister, her husband, their 2-day-old infant, and their pet Chihuahua arrive at your door. The parents of the child are concerned because the pediatrician noted the child was yellow and ordered some studies. They produce a wad of papers for you to review. Both the mother and baby have O positive blood. The baby’s direct serum bilirubin is 0.2 mg/dl, with a total serum bilirubin of 11.8 mg/dl. Urine bilirubin is positive. The infant’s white blood cell count is 13,000/ul with a differential of 50% polymorphonuclear cells, 45% lymphocytes, and 5% monocytes. The hemoglobin is 17 g/dl, and the platelet count is 278,000/ul. Reticulocyte count is 1.5%. the peripheral smear does not show fragments or abnormal cell shapes. which of the following is the most likely explanation for this infant’s skin color? a. Rh or ABO hemolytic disease b. Physiologic jaundice c. Sepsis d. Congenital spherocytic anemia e. Biliary atresia 1. The inheritance pattern of Congenital nephrotic syndrome is/are: a. Autosomal recessive b. X-linked recessive c. Autosomal dominant d. Sporadic 2. True/False: A renal biopsy is necessary to confirm the diagnosis of primary idiopathic nephrotic syndrome. False 3. A 5-year-old male, his parents and two siblings have visited a water slide park. Two days later, the patient developed diarrhea, which turned bloody. The bloody diarrhea was associated with crampy abdominal pain and vomiting. When the vomiting started, the patient was taken to a local emergency department where he was given intravenous fluids and prescribed loperamide for diarrhea. One day later, the patient returned to the emergency department because he looked pale, becoming somnolent, and had not urinated for 24 hours. The initial laboratory investigations revealed a hemoglobin of 8g/l, leukocyte count 17,500ul, and a platelet count of 19000/ul, the peripheral blood smear showed 12% schistocytes. The BUN was 35mg/dl and the serum creatinine was 2,5mg/dl. The serum sodium was 141mEq/l, potassium 5,6mEq/l, chloride 105mEq/l and total bicarbonate 18mmol/l. the serum calcium was 8,2mg/dl, inorganic phosphorus 7,0mg/dl and glucose 310 mg/dl. During the next 24 hours the patient’s urinary output decreased to 55ml/24hr, with an increase in the serumcreatinine to 3.8mg/dl. Because the patient remained severely oliguric and uremic with symptoms of nausea and vomiting, peritoneal dialysis was began. After 3 weeks of peritoneal dialysis, the urine output rose to 200ml/24hr.and platelet count increased to 95,000/ul. In the present case, which treatment has been shown to be most efficacious? a. Supportive treatment b. Plasma infusion c. Intravenous immune globulin d. Tissue-type plasminogen activator e. Oral Shiga toxin-binding agent 4. What is the likely etiology of D+ HUS? a. E. coli O157:H7 b. Shigella sonnei c. Entamoeba histolytica d. haemophilus influenza 5. Early treatment of diarrhea caused by E. coli 0157:H7 with antibiotics: a. Decreases the likelihood that the patient will develop hemolytic-uremic syndrome (HUS). b. Increases the likelihood that the patient will develop HUS c. Exacerbates diarrheal symptoms 6. Which of the following statements is wrong? a. The maxillary sinuses are not pneumatized until 4 yr of age. b. The sphenoidal sinuses are present by 5 yr of age c. The frontal sinuses are completely pneumatized by7-8 yr. 7. If the patient presents with persistent symptoms of a respiratory infection, including nasal discharge and cough, within 10-14 days without improvement, findings on radiographic studies (sinus plain film, CT scans) indicate opacification, mucosal, with which diseases should a differential diagnosis be made? a. Viral upper respiratory tract infection, b. Allergic rhinitis, c. nonallergic rhinitis d. Nasal foreign body. e. with all of them 8.Which antibiotic is used to treat frontal sinusitis? a. amoxicillin b. ampicillin-sulbactam c. trimethoprim-sulfamethoxazole d. Ceftriaxone 9. The 2 major complications of untreated GABHS infection are: a. post-streptococcal glomerulonephritis b. acute rheumatic fever c. post-streptococcal endocarditis d. post-streptococcal arthritis e. a and b f. a and c g. B and c h. c and d 10. Which of the following is superfluous? Potential indications for Tonsillectomy and Adenoidectomy include: a. 7 or more throat infections treated with antibiotics in the preceding yr, b. 5 or more throat infections treated in each of the preceding 2 yr, c. 3 or more throat infections treated with antibiotics in each of the preceding 3 yr. d. adenotonsillar hypertrophy, e. failure to thrive. f. None of the answers are superfluous 11. Which disease is characterized by the steeple sign on the radiograph? a. Croup b. Acute epiglottitis c. Acute infectious laryngitis d. Bacterial tracheitis 12. Which disease is characterized by the thumb sign on the radiograph? a. Croup b. Acute epiglottitis c. Acute infectious laryngitis d. Bacterial tracheitis 13. Which of the following statements is wrong? a. viral pneumonia occurs between the ages of 2 and 3 yr, decreasing slowly thereafter. b. The most common causes of viral infection are RSV , Parainfluenza virus, Adenovirus c. On chest radiograph viral pneumonia is usually characterized by hyperinflation with bilateral interstitial infiltrates and peribronchial cuffing. d. The definitive diagnosis of a viral infection rests on the isolation of a virus or detection of the viral genome or antigen in respiratory tract secretions. e. The patients with known viral infection may have coexisting bacterial pathogens, It is therefore advisable to start antibiotic therapy immediately. 14. More than 60 mEq/L of chloride in sweat is diagnostic of CF when 1 or more other criteria are present. The procedure requires care and accuracy. A false negative test result is associated with all of the following except one: a. Cystic fibrosis transmembrane conductance regulator (CFTR) mutations with preserved sweat duct function b. hypernatremia c. Edema d. Insufficient sweat quantity e. Dilution 15. More than 60 mEq/L of chloride in sweat is diagnostic of CF when 1 or more other criteria are present. The procedure requires care and accuracy. A false-positive test result is associated with all of the following except one: a. Hypothyroidism b. Nephrogenic diabetes insipidus c. Down syndrome d. Klinefelter syndrome e. Familial cholestasis syndrome 16. What statement is wrong? a. For most pediatric patients with non-CF related bronchiectasis suggested systemic antibiotics to treat acute exacerbations and routine chest physiotherapy. b. For patients with persistent Pseudomonas aeruginosa infection, chronic treatment with inhaled aminoglicosides are suggested. c. For children with non-CF-related bronchiectasis treating with Dnase is suggested. d. Chronic treatment with oral azithromycin may be considered for children older then six years of age who have frequent exacerbations or progressive decline in pulmonary function. 17. Which of the followings is the correct answer? the most common chronic symptoms of asthma are: a. Intermittent dry coughing and expiratory wheezing b. Intermittent dry coughing and both expiratory and inspiratory wheezing. c. Intermittent dry coughing and inspiratory wheezing 18. A 9-year-old girl comes in to see you with fever. She had a runny nose several days ago, which has now changed to thick yellow discharge. Three days ago she developed a fever of 39,4c., which has remained until today. She also has a mild headache and a nighttime cough, but no other symptoms. What is her diagnosis? a. Allergic rhinitis b. Rhinovirus c. Influenza d. Sinusitis e. Immotile cilia syndrome. 19. Characteristic features of this disease are: “Allergic Salute”, dental malocclusion, “allergic gape” or continuous open-mouth breathing, chapped lips, “allergic shiners” (dark circles under the eyes), and the transverse nasal crease. conjunctival edema, itching, tearing and hyperemia are also frequent findings. Inhalant allergens are the main cause of this disease. What disease do you suspect? a. Allergic rhinitis b. Sinusitis c. Asthma 20. 2-year-old male presents to your clinic for evaluation of respiratory difficulties. His mom reports that he was in his usual State of health until yesterday when he began having shortness of breath, cough, and an occasional wheeze. Mom gave him two puffs of his brother’s albuterol inhaler, which initially provided some relief. Mom denies preceding symptoms, such as rhinorrhea, fever, or other URI symptoms. Mom also denies smokers in the home, pets, or carpets, but notes that the symptoms did start after playing next door with his 4-year-old neighbour. The patient has wheezed once before when he was 4 months old and diagnosed with RSV. On physical exam, the patient is in moderate respiratory distress. His oxygen saturation is 92% on room air, with a respiratory rate of 44. Other vital signs are normal. His lung exam is notable for diffuse expiratory wheezing with variably decreased breath sounds at the right base, The rest of his exam normal. A. What is the most likely cause of this patient’s respiratory distress? a. Viral infection b. Foreign body aspiration c. Asthma d. Bacterial pneumonia e. Anaphylaxis B. Your initial step in treatment in your office is: a. Supplemental oxygen b. Oral steroids c. Inhaled bronchodilator d. Subcutaneous epinephrine 21. All of the following are true regarding asthma education except: a. Asthma action plans are warranted and efficacious. b. Peak flow monitoring and/or symptom monitoring are not reliable methods of self- management.  c. Asthma action plans must be reviewed and revised by the physician. d. All action plans should include contacts for urgent care e. Skill sets to be taught include inhaler use, self-monitoring and environmental control. 22. Which of the following is not known to exacerbate asthma symptoms? a. weather changes b. Mite exposure c. Beta-agonist drugs d. Aspirin e. Smoke 23. Which of the following contribute to or exacerbate asthma or allergies? a. Maternal tobacco smoking b. Indoor pets c. Dust mist d. B and C e. All of the above 24. The underlying pathology of asthma is characterized by inflammation of the airway. This inflammation is mediated by many factors, one of which is major basic protein. What is the source of major basic protein? a. Mast cells b. IgE receptors c. Neutrophils d. IL-2 e. Eosinophils 25. How many typical upper respiratory tract infections per year does a healthy child have? a. 2 to 3 b. 3 to 4 c. 4 to 5 d. 5 to 6 e. More than 6 26. This is the most common life-limiting autosomal recessive genetic trait among white persons. The characteristic features of this disease are acute or persistent respiratory symptoms, Failure to thrive, malnutrition, abnormal stools, meconium ileus, intestinal obstruction, family history, rectal prolapse, nasal polyps, sinus disease, Hepatobiliary disease. What disease do you suspect? a. Cystic fibrosis b. Asthma c. Duncan diseasse (X-linked lymphoproliferative disease) d. DiGorge syndrome (thymus hypoplasia) 27. Bronchiectasis is a complication of the following immunodeficiency diseases except one: a. Severe combined immunodeficiency b. Selective IgA immunodeficiency  c. X-linked agammaglobulinemia d. Duncan diseasse (X-linked lymphoproliferative disease) e. DiGorge syndrome (thymus hypoplasia) 28. Which antibiotic is the first line medication for treatment of bacterial community-acquired pneumonia? a. For mildly ill children who do not require hospitalization, amoxicillin is recommended. In communities with a high percentage of penicillin-resistant pneumococci, high doses of amoxicillin (80-90 mg/kg/24 hr) should be prescribed. b. The empiric treatment of suspected bacterial pneumonia in a hospitalized child iniciated with Parenteral cefotaxime or ceftriaxone. c. If clinical features suggest staphylococcal pneumonia (pneumatoceles, empyema), initial antimicrobial therapy should also include vancomycin or clindamycin. 29. Which of the following statements is right? Factors suggesting need for hospitalization of children with pneumonia are: a. Age < 6 mo b. Sickle cell anemia with acute chest syndrome c. Toxic appearance d. Requirement for supplemental oxygen e. Social factors (e.g., inability of caregivers to administer medications at home or follow up appropriately) f. All answers are right. 30. Autoantibodies that are specific for SLE include: a. Antidouble stranded DNA (anti-dsDNA) and antiribonuclear protein (anti RNP) b. Anti-ds DNA and anti-Smith c. Anti-SS-A and anti-Smith d. Anti-histone antibodies and anti-dsDNA e. Anti-SS-A and antiRNP 31. Complications which may occur in SLE include all except: a. Platelet counts less than 5000/mm3. b. Psychosis, seizures, or cognitive dysfunction c. interstitial pneumonitis, interstitial fibrosis or pleuritic. d. Progressive, erosive arthritis in the majority of patients who have joint swelling. e. Pericarditis, myocarditis, or valvular dysfanction. 32. The following statement(s) is/are true about the outcomes in childhood SLE: a. Infection is a major cause of mortality in children with SLE. b. Cardiovascular disease, including myocardial infarction and stroke, is a major long-term cause of morbidity and mortality. c. Most children are able to discontinue all medications after 5 years of treatment. d. A and B e. All of the above. 33. Which statement is correct regarding drug-induced lupus?  a. All patients require treatment with corticosteroids to control symptoms. b. Renal involvement is common. c. Anti-dsDNA is usually positive. d. Minocycline is one of several drugs known to cause drug-induced lupus. e. Complement levels are usually depressed in drug-induced lupus. 34. All of the following are helpful in diagnosing juvenile dermatomyositis (JDM) except: a. Presence of Gottron’s papules and a heliotrope rash over the eyelids. b. Presence of ANA and an elevated sedimentation rate. c. Elevated serum muscle enzymes d. Proximal muscle weakness e. Muscle biopsy showing inflammatory cell infiltrate, and perifascicular atrophy and necrosis. 35. All are true about the complications and outcome of JDM except: a. Calcinosis may cause limitation in joint range and cosmetic problems. b. More than two-thirds of patients have good to excellent functional outcomes c. Mortality is usually secondary to respiratory failure or gastrointestinal vasculitis. d. Dysphagia and reflux are secondary to lower esophageal sphincter dysfunction. e. In sever cases, dysphonia may be present. 36. A 5-year-old child presents to the clinic with a history of fever, rash, a swollen knee and ankle, shortness of breath and fatigue. His past medical history is significant for the fact that he had a strep throat infection 3 weeks prior to this visit but did not take the amoxicillin that was prescribed for him. On physical exam, he has a temperature of 38,9C. He has a systolic regurgitant murmur at the apex and a gallop. He has hepatomegaly and good peripheral pulses. What is the most likely diagnosis in this child? a. Acute rheumatoid fever b. Infective endocarditis c. Kawasaki disease d. Sickle cell disease 37. Which heart valve is the most commonly affected with acute rheumatic fever? a. Mitral valve b. Aortic valve c. Tricuspid valve d. pulmonary valve. 38. A 2-year-old child presents with a 10-day history of fever, a heart murmur, bilateral nonexudative conjunctivitis, swollen and erythematous lips and strawberry tongue with erythematous rash on the face, trunks and extremities. The most likely diagnosis in this patient is: a. Kawasaki disease b. Measles c. Viral upper respiratory tract infection d. Group A beta hemolytic streptococcal pharyngitis 39. Which of the following statements is true regarding coronary artery involvement with this Kawasaki disease?  a. There is a 50% incidence of coronary artery aneurysms if untreated. b. The peak incidence for coronary artery aneurysms is 6 to 12 months following the onset of fever. c. Patients with giant coronary artery aneurysms greater than 8 mm in diameter are at highest risk for late stenosis and myocardial infection. d. Coronary artery rupture is the most common cause of mortality within the first 7 days of the onset of fever. 40. Name the two drugs most commonly used for acute management of Kawasaki disease: a. Aspirin and IVIG b. Penicillin and IVIG c. Steroids and aspirin d. Steroids and penicillin 41. A 4-year-old girl presents with erythematous maculopapular lesions on the buttocks that extend to the lower extremities and trunk. Initially the lesions blanched on pressure but later became purpuric. As her lower extremities became swollen and painful. her most important complaint was severe abdominal pain. On examination the temperature was 37,5C, the patient was in moderate distress because of abdominal pain, which was diffuse and intermittent. Examination of the skin over the trunk and buttocks revealed many 0,5 to 1 cm purpuric lesions and mildly swollen ankles and knees. The urinalysis revealed protein/creatinine ratio 1,8. protein 3+, specific gravity 1,018, ph 5, the BUN was 31 mg/dl. Which disease do you suspect? a. Kawasaki disease b. Henoch-Schonlein purpura c. Acute rheumatoid fever d. None of the above 42. A 5-year-old boy was brought to the emergency room for evaluation of a new rash. The patient awoke this morning covered in “red dots”, according to his mother. He had a “cold” approximately 2 weeks previously, but otherwise has been well. He takes no medications and has had no sick contacts. There is no history of toxin ingestions. The family history is non-contributory. On physical exam, the child is well-appearing and afebrile. He has diffuse petechiae covering his face, neck, chest, back, stomach, arms and legs. He also has large bruises on his forearms and upper and lower legs. He has no palpable lymphadenopathy or hepatosplenomegaly. A complete blood count done in the emergency room is within normal limits except for a platelet count of 8,000/ul. The most likely diagnosis for this child is: a. Acute lymphoblastic leukemia b. ITP (Idiopathic thrombocytopenic purpura) c. Von Willebrand disease d. Thrombocytopenia with radii (TAR) syndrome 43. Which of the following is the least common complication of ITP (Idiopathic thrombocytopenic purpura)? a. Intracranial hemorrhage  b. Epistaxis c. Intra-articular hemorrhage d. Melena 44. Which of the following is the best initial treatment for patients with ITP? a. Intravenous immune globulin b. Ibuprofen c. Splenectomy d. Platelet transfusion 45. Which of the following is not a feature of Wiskott-Aldrich syndrome? a. Eczematous rash b. Thrombocytopenia c. Anemia d. Recurrent infections 46. What is the inheritance pattern for factor VIII deficiency? a. Autosomal recessive b. Autosomal dominant c. X-linked d. Mitochondrial 47. Which of the following is the least likely to occur in patients with vWD (von Willebrand disease)? a. Gingival hemorrhage b. Menorrhagia c. Prolonged epistaxis d. Hemarthrosis 48. Which of the following is the best treatment for a patient with sever bleeding and type I von Willebrand disease?? a. Intranasal ddAVP (desmopressin) b. Fresh frozen plasma c. Recombinant factor VIII d. Platelet transfusion 49. All the following are true about GI involvement in HSP except: a. GI hemorrhage is common and may be occult or gross b. GI symptoms can present before the rash c. A normal barium enema rules out intussusception in HSP d. Complications include intussusception, bowel infarction and perforation. e. Ultrasonography may show edema of bowel wall and may identify an intussusception. 50. True/False: Abnormal vital signs within the first 30-60 minutes of life are always pathologic and indicate an unhealthy newborn. a. True b. False 51. Postmature newborn 3000gr, with red-purple skin, born by cesarean section, the mother is sick with diabetes. the child has irritability, lethargy, tachypnea.in laboratory tests it is noted hyperbilirubinemia, hypoglycemia, thrombocytopenia, Hct more then 65%. What disease do you suspect? a. Hemorrhagic disease of the newborn b. Plethora c. Hemolytic disease of the newborn caused by RH incompatibility d. Hemolytic disease of the newborn caused by blood group A and B incompatibility. 52. what answer are wrong? Clinical features of rickets are: a. Failure to thrive b. Muscle weakness c. Valgus or varus deformities. d. Mental retardation 53. What is the most common cause of anaphylaxis occurring outside the hospital? a. Food allergy b. Latex allergy c. Inhalant allergy 54. This disorder is characterized by progressive difficulty with coordinating movements, beginning in early childhood, usually before age 5. oculocutaneous are visible small linear red blood vessels. Characteristic feature of this disease also are: chronic sinopulmonary disease, a high incidence of malignancy, and variable humoral and cellular immunodeficiency. The most frequent humoral immunologic abnormality is the selective absence of IgA, which occurs in 50-80% of these patients. Hypercatabolism of IgA also occurs. IgE concentrations are usually low, and the IgM may be of the low molecular weight variety. What disease do you suspect? a. Ataxia-teleangiectasia b. hyper-IgE syndrome c. DiGeorge syndrome d. Selective IgA deficiency 55. Atopic dermatitis, eczema, pemphigus, urticaria, toxic epidermal necrolysis are associated with: a. thrombocytosis b. lymphocytosis c. Anemia d. Eosinophilia 56. Children suffering from this disease have light skin and silvery hair and frequently complain of solar sensitivity and photophobia that is associated with rotary nystagmus. Also typical prolonged bleeding times with normal platelet counts, resulting from impaired platelet aggregation associated with a deficiency of the dense granules containing adenosine diphosphate and serotonin. The most life-threatening complication of is the development of an accelerated phase characterized by pancytopenia, high fever, and lymph histiocytic infiltration of liver, spleen, and lymph nodes. What disease are you thinking about?  a. DiGeorge syndrome b. Selective IgA deficiency c. CHÉDIAK-HIGASHI SYNDROME d. X-LINKED AGAMMAGLOBULINEMIA 57. Friends are considering adopting a “special needs” child from another country. The family has few details, but the information they have received so far suggests the 4-year-old child has had surgery for an endo-cardial cushion defect, is short for his age, and had a history of what sounds like surgically repaired duodenal atresia at birth. You are suspicious this child may have which of the following syndromes? a. Kleinfelter b. Waardenberg c. Marfan d. Down e. Turner 58. An 11-year-old is the tallest child in his sixth grade class. He is thin, has a high arched palate, mild scoliosis, and joint hyperextensibility. He is an honor student, and enjoys basketball but must use prescription sports goggles so he can see the ball on the court. He is likely to have which of the following conditions? a. Cerebral gigantism (Sotos syndrome) b. Homocystinuria c. XXY (Klinefelter syndrome) d. Marfan syndrome e. XYY 59. 16 years old girl with short stature, webbed neck, puffiness, non-functioning ovaries, high blood pressure. What is her probable diagnosis? a. Down syndrome b. Marfan syndrome, c. Patau syndrome d. Turner syndrome e. Klinefelter syndrome 60. A 14-year-old girl awakens with a mild sore throat, low-grade fever, and a diffuse maculopapular rash. During the next 24 hours, she develops tender swelling of her wrists and redness of her eyes. In addition, her physician notes mild tenderness and marked swelling of her posterior cervical and occipital lymph nodes. Four days after the onset of her illness, the rash has vanished. Which of the following is the most likely diagnosis? a. Rubella b. measles c. Roseola d. Erythema infectiosum e. Erythema multiforme 61. Which of the following is a first-line treatment for anaphylaxis?  a. Supplemental oxygen b. Antihistamine c. Corticosteroid d. Epinephrne e. Nebulizerd albuterol 62. A characteristic symptom of which congenital disease is a sweet odor of urine, sweat, earwax, ataxia, lethargy, vomiting, poor feeding, irritability, seizures? a. galactosemia b. phenylketonuria c. maple syrup urine disease d. Congenital Hypothyroidism 63. A 1-month-old baby with cataract was admitted in the hospital with 5 days duration of vomiting, jaundice, hypoglycemia. Physical exam reveals hepatosplenomegaly and poor weight gain. Which screening tests would most likely be abnormal? What is his probable diagnosis? a. galactosemia b. phenylketonuria c. maple syrup urine disease d. Congenital Hypothyroidism 64. A 1-week-old newborn presents with vomiting of 3 days duration, lethargy, dystonia, irritability, poor feeding. Physical exam reveals a masty odor of the urine. a. galactosemia b. phenylketonuria c. maple syrup urine disease d. Congenital Hypothyroidism 65. Which of the following statements is true? In neonates and pediatric patients, SIRS manifests as: a. temperature instability, b. respiratory dysfunction (altered gas exchange, hypoxemia, acute respiratory distress syndrome) c. cardiac dysfunction (tachycardia, delayed capillary refi ll, hypotension), d. perfusion abnormalities (oliguria, metabolic acidosis) e. A and d f. All of above 66. The most reasonable indication for treating mononucleosis with steroids is: a. Severe splenomegaly b. Severe pain from sore throat c. Severe airway obstruction d. Hemolitic anemia e. Myocarditis 67. Abnormal cells that do not contain a multiple of haploid number of chromosomes are termed:  a. aneuploid cells b. Monosomies c. Triploid cells d. Polyploid cells 68. Choose correct answer: A chromosomal abnormality that causes a woman to be unusually short in stature (average 4'7"), to have a webbed neck, and to generally lack feminine secondary sexual characteristics is: a. Triple-X syndrome b. Turner syndrome c. XXY syndrome 69. A 10-year-old has complained for 1 month of intermittent epigastric pain that awakens him from sleep. He notes that eating food sometimes helps. He reports black stools during the prior week, and also admits that he has occasionally vomited frank blood. What disease do you suspect? a. Peptic ulcer disease b. Acute pancreatitis c. Gastroesophageal reflux d. Pyloric stenosis 70. A 6 week –old boy comes to your office with several days of vomiting and irritability. He vomits everything he eats. The vomiting does not have any blood in it and is not associated with diarrhea or fever. The vomiting is described as being light in color but not green. The baby is the product of a full-term gestation with no complications. On physical examination this is a well nourished child: the weight is 4,4kg, the HR is 180, and the RR is 38. his anterior fontanel is slightly sunken and there are dry mucous membranes. The abdomen is nondistended with normal bowel sounds. The remainder of examination is unremarkable. This child’s diagnosis most likely is: a. Gastroesophageal reflux b. Malrotation c. Pyloric stenosis d. Viral gastroenteritis 71. The diagnostic test to confirm diagnosis of pyloric stenosis is: a. pH probe b. Upper GI series c. Ultrasound of the abdomen d. The diagnosis should be made on clinical grounds and no further testing is necessary. 72. An 8-year-old is accidentally hit in the abdomen by a baseball bat. After several minutes of discomfort, he seems to be fine. Over the ensuing 24 hours, however, he develops a fever, abdominal pain radiating to the back, and persistent vomiting. On examination, the child appears quite uncomfortable. The abdomen is tender, with decreased bowel sounds throughout, but especially painful in the midepigastric region with guarding. What disease do you suspect? a. Acute pancreatitis  b. Hemorrhage in the spleen c. Gastro-duodenitis d. Not all above 73. Which of the following tests is most likely to confirm the diagnosis of acute pancreatitis? a. Serum lipase levels b. CBC with differential and platelets c. Serum total and direct bilirubin levels d. Abdominal radiograph e. Electrolyte panel 74. Which of the following is wrong? a. For children with suspected H. pylori infection, an initial upper endoscopy is recommended to evaluate and confirm H. pylori disease. b. H. pyloric is a gram-negative, S-shaped rod that produces urease, oxidase. c. C-urea breath tests and stool antigen tests are noninvasive method of detecting H. pylori infection. d. Immunoglobulin G (IgG) antibody detection may be helpful for assess the success of antimicrobial eradication therapy. 75. Which of the following is false regarding “stress”ulceration? a. Stress ulceration usually occurs within 24 hr of onset of a critical illness in which physiologic stress is present. b. Preterm and term infants in the neonatal intensive care unit can also develop gastric mucosal lesions and can present with upper GI bleeding or perforated ulcers. c. Prophylactic measures to prevent stress ulcers in children consists triple therapy with antibiotics, bismuth salts and PPI. 76. Which of the following disease is bacterial genesis? a. Rubella b. Measles c. Scarlet fever d. Inf. Mononucleosis 77. An infant can regard his parent’s face, follow to the midline, lift his head from the examining table, smile spontaneously, and respond to a bell. He does not yet regard his own hand, follow past the midline, nor lift his head to a 45° angle off the examining table. Which of the following is the most likely age of the infant? a. 1 month b. 3 months c. 6 months d. 9 months e. 12 months 78. A child is brought to your clinic for a routine examination. She can put on a T-shirt but requires a bit of help dressing otherwise. She can copy a circle well but has difficulty in copying a square. Her speech is understandable and she knows four colors. She balances proudly on each foot for 2 seconds but is unable to hold the stance for 5 seconds. Which of the following is the most likely age of this child? a. 1 year b. 2 years c. 3 years d. 4 years e. 5 years 79. The mother of a 3-day-old infant brings her child to your office for an early follow-up visit. The mom notes that the child has been eating well, has had no temperature instability, and stools and urinates well. She notes that over the previous 3 days the child has had a progressive “rash” on the face as pictured here. Which of the following is the most likely diagnosis? a. Herpes b. Neonatal acne c. Milia d. Seborrheic dermatitis e. Eczema 80. A child can walk well holding on to furniture but is slightly wobbly when walking alone. She uses a neat pincer grasp to pick up a pellet, and she can release a cube into a cup after it has been demonstrated to her. She tries to build a tower of two cubes with variable success. She is most likely at which of the following age? a. 2 months b. 4 months c. 6 months d. 9 months e. 1 year 81. A newborn’s mother complains that her infant seems to have very small white dots all over his nose. The dots do not wipe off with bathing, but they are also not erythematous. For each of the descriptions listed below, select the most likely diagnosis a. Sebaceous nevus b. Salmon patch c. Neonatal acne d. Pustular melanosis e. Erythema toxicum f. Seborrheic dermatitis g. Milia 82. In infants younger than three months, it is most important to evaluate a. The stability of the hips using the Ortolani maneuver b. The Barlow maneuver c. The Galeazzi test (also called the Allis or Perkins test) d. The Klisic test e. Decreased abduction 83. Is any statement wrong listed below? In infants after three months of age it is most important to evaluate a. The stability of the hips using the Ortolani and Barlow maneuver b. The Galeazzi test (also called the Allis or Perkins test) c. The Klisic test d. Decreased abduction 84. A 15-year-old vegetarian being treated for tuberculosis develops peripheral neuropathy. choose the one most appropriate vitamin or trace element replacement therapy to treat the described condition. a. Vitamin A b. Vitamin B6 c. Vitamin C d. Vitamin D e. Vitamin E f. Iron g. Vitamin K h. Folate i. Niacin 85. A haploid cell (n) has number of chromosomes: a. 23 b. 24 c. 45 d. 46 86. Choose correct answer: Which of the following statements is true of sex chromosome abnormalities in humans? a. They usually have mild effects and rarely are fatal b. Most are not gender specific c. They cannot be diagnosed before birth 87. Choose correct answer: Male sex chromosome abnormalities can be due to abnormal numbers of ______________ chromosome. a. The X b. The Y c. Either the X or the Y 88. Choose correct answer: A chromosomal abnormality that causes a man to have asexual to feminine body contours with large breasts; small penis, testes, and prostate gland; relatively little body hair; and sterility is: a. Kleinfelter syndrome b. XXY syndrome c. Richard Speck Syndrome 89. Choose correct answer: The likelihood of a child being born with a major genetic defect, such as mental retardation, can often be detected by sampling: a. the mother's uterus cells b. cells from the embryo or fetus c. the father's blood cells 90. Choose correct answer: Which of the following can be detected now by examining a karyotype? a. over 3,000 genetic defects b. an unborn child's gender or sex c. both of the above 91. Choose correct answer: Which syndrome is also known as Trisomy 18? a. Down syndrome b. Edwards syndrome c. Patau syndrome d. Kleinfelter syndrome 92. Choose correct answer: Which syndrome is also known as Trisomy 21? a. Down syndrome b. Edwards syndrome c. Patau syndrome d. Kleinfelter syndrome e. Turner syndrome 93. Choose correct answer: Which of the following chromosome abnormalities is also known as Turner syndrome? a. Trisomy 21 b. 47,XXY c. Trisomy 13 d. 45,X0 94. Choose correct answer: Which chromosomal abnormality is known as Patau syndrome? a. Trisomy 13 b. Trisomy 18 c. Trisomy 21 d. 47, XXX e. 47,XXY 95. Choose correct answer: 47, XXY is also known as __________________. a. Down syndrome b. Edwards syndrome  c. Patau syndrome d. Kleinfelter syndrome e. Turner syndrome 96. The newborn was delivered after prolonged labor with vacuum. There is marker lesion on head an oedematous swelling. It is diffuse type pits occur when pressure is given with finger. It is generally a benign condition, and it usually resolves within a few days and requires no treatment. What lesion is most likely to be found on head? a. Caput succedaneum b. Subgaleal hemorrhage c. Cephalohematoma 97. Newborn has diminished movement on the affected side of face, partial closing of the eye when crying, the mouth is drawn over to the unaffected side. Which injury did you suspect? Bell's palsy 98. Disappearance of what reflex allows infants begin to examine objects in the midline and manipulate them with both hands? a. Moro reflex b. Asymmetric tonic neck reflex c. Palmar grasp reflex d. Rooting reflex 99. About how many months a kid if he can - transfers objects from one hand to the other - pulls self up to sit and sits erect with supports - rolls over from tummy to back 6 months 100. A 15-year-old healthy boy, who is vaccinated according to the schedule, had his spleen removed two weeks ago due to injury. Which of the following vaccines would you recommend? Choose the correct answer: a. because he was vaccinated according to the recommended immunization Schedule for persons aged 0-18 year. he does not need any vaccination else, except single vaccination against meningococcal disease. b. because he was vaccinated according to the recommended immunization Schedule for persons aged 0-18 year. he does not need any vaccination else except vaccination against season flu. c. within two weeks after the operation, the boy should be vaccinated against Haemophilus influenza, Pneumococcal and meningococcal diseases. because after operation he is at hight risk for exposure to these diseases. 101. What statement is right? a. Serum antibodies may be detected as soon as 7-10 days after injection of antigen. b. Early antibodies are usually of the IgM class that can fix complement.  c. IgM antibodies tend to decline as IgG antibodies increase. d. The IgG antibodies tend to peak approximately 1 mo after vaccination and with most vaccines persist for some time after a primary vaccine course. e. all statements is right 102. Studies of undervaccinated children have documented opportunities that were missed because this condition was used as an invalid contraindication. what state are they talking about? a. Sever acute illness without fever b. Moderate acute illness without fever c. Mild illness without fever d. Preterm infants 103. what answer is wrong? a. Most children walk with a mature gait and run steadily before the end of their 3th yr. b. Language development occurs most rapidly between 2 and 5 yr of age. c. Bed wetting is normal up to age 5 yr in girls and age 9 yr in boys. 104. What of the following Statements is wrong? a. Approximately 50% of patients with AD experience symptoms in the 1st year of life. b. AD involves the face, scalp, extensor surfaces of the extremities, the diaper area. c. Acute AD skin lesions are intensely pruritic with erythematous papules. d. There are no specific laboratory tests to diagnose AD. 105. a 18mo boy with chronic diarrhea, Inadequate food supplies, has severe weight loss, wasting, sunken eyes, wrinkled skin, abdomen distention. what disease are we talking about? a. marasmus b. Noma c. Kwashiorcor 106. A patient with mononucleosis and splenomegaly should be advised to avoid contact sports for at least how long? a. 1 to 2 weeks b. 3 weeks to 6 weeks c. 1 year d. life e. None of the above-no recommendation to avoid contact sports. 107. In what cases we don’t treat newborns with IV acyclovir? a. Infants with community-acquired varicella who experience severe varicella, especially those who have a complication such as pneumonia, hepatitis, or encephalitis. b. All premature infants born 30wk gestation, whose mother demonstrate varicella in the period from 5 days prior to delivery to 2 days afterward. d. All Infants born >30wk gestation, whose mother demonstrates varicella >5 days prior to delivery. 108. The simultaneous presence of lesions in various stages of evolution is characteristic of this disease. This lesions often appear first on the scalp, face or trunk. Clouding and umbilication of the lesions begin in 24-48hr. While the initial lesions are crusting, new crops form on the trunk and then the extremities. What disease do you suspect? a. Rubella b. Chickenpox c. Measles d. Scarlet fever 109. Which of these disease prevention measures routinely administered to all newborns. a. Vitamin K prophylaxis, b. antibiotic eye prophylaxis, c. hepatitis B immunization, d. All of above 110. List three early disease detection measures routinely administered to all newborns. Answer: Newborn blood and metabolic disease screening, hearing screening, physical examination. 111. True/False: Breast milk is associated with a decrease in the incidence of several common infections. Answer: True 112. true/false: breastfeeding lowers risk of asthma, eczema, obesity, sudden infant death syndrome. Answer: True 113. True/False: Circumcision should be routinely recommended based on medical advantages. Answer: False 114. True/False: Normal stools from breast fed infants appear to be loose, yellow and seedy. Answer: True 115. Which of the following statements is wrong? a. IM injections should be given with the needle at a 90º angle to the skin and the skin should be punched, not stretched. b. Deep SC injections should be given with the needle at a 45º angle to the skin and the skin should be bunched, not stretched. c. It is not necessary to aspirate the syringe after the needle is introduced into the muscle. 116. This is the classic position of breastfeeding. It involves mom sitting upright, with her baby positioned on his side, his head and neck lying along mom forearm and his body against mom’s stomach, in a tummy-to-mummy position. What is this position called? a. Cradle hold  b. Cross-cradle hold c. Rugby ball hold 117. What is an appropriate quantity of formula for an infant during the first 6 months of life ? 95-115 kcal/kg/day 118. True/false: Signs of good breastfeeding include the following: audible rhythmic swallowing during nursing, breasts feeling less full after each feeding session, at least 1-2 wet diapers per day for the first 2 days of life, 4-6 wet diapers every 24 hours after the 3rd day of life, and at least 3-4 bowel movements every 24 hours. Lack of persistent pain during breastfeeding sessions and absence of sore nipples are also signs of appropriate breastfeeding. Answer: True 119. Which of the following is wrong? a. Right vaccines must not be used after their expiry date. b. Vaccines should not be given intravenously. c. If more than one injection is to be given in the same limb, they should be administered at least 2.5cm apart. d. All of above 120. True/false: Indicators for inadequate breastfeeding include: less than 6 urinations per day and 3-4 stools per day by day 5-7 of life, decreased activity level, difficulty arousing, weight loss of greater than 15% of birth weight within the first week of life. Answer: true 121. All of the following are true regarding BCG vaccination except: a. During an intra-dermal injection, considerable resistance is felt and a raised, blanched bleb showing the tips of the hair follicles is a sign that the injection has been correctly administered. b. A bleb of 7mm in diameter is approximately equivalent to 0.1ml and is a useful indication of the volume that has been injected. c. If no resistance is felt, the needle should be removed and reinserted before more vaccine is given. d. Vaccines not given by the IM route include Bacillus Calmette-Guérin (BCG) vaccine, which is given by intravenous injection. 122. Which of the following vaccines is given orally? a. Polio vaccine b. BCG c. MMR 123. Normal inspiratory to expiratory (I:E) ratio normally is 1:2. If it's taking longer to breath out (expiration time), that indicates: a. upper airway obstruction b. lower airway obstruction 124. For correctly measure blood pressure, it is need to choose the appropriate size of cuff. if use too big size of cuff, the measured pressure could be: a. falsly low b. Falsly elevated. 125. If a patient’s blood pressure is 90 mmhg/50mmHg, his MAP is: Answer :(63,3mmHg) 126. If a patient’s blood pressure is 80 mmHg/45mmHg, his MAP is: Answer: (56,6mmHg) 127. If a patient’s blood pressure is 100 mmHg/70mmHg, his MAP is: Answer: (80mmHg) 1. the likelihood of a child being born with a major genetic defect, such as mental retardation, can be detected by sampling: a. The mother’s uterus cells b. Cells from the embryo or fetus c. The father’s blood cell 2. a 10-year-old child with physical features of tall stature, a long thin face, scoliosis, pectus excavatum with a family history of sudden death at a young age presents for a preparcipation sports physical. What is the likely diagnosis in this patient? a. Marfan syndrome b. Turner syndrome c. Down syndrome d. Noonan syndrome 3. which of the following is true regarding Noonan syndrome? a. Noonan affects only males b. Noonan syndrome is the result of sex chromosome abnormality c. The clinical features of Noonan syndrome have significant overlap with turner syndrome d. Noonan syndrome is not genetic condition e. None of the above are true 4. standard treatment of growth failure in Turner syndrome includes: a. Leg-lengthening procedures b. Maximizing caloric intake c. Estrogen replacement d. Recombinant growth hormone e. None of the following 5. which of the following medical problems are not associated with Turner syndrome? a. Coarctation of the aorta b. Agenesis of the corpus callosum c. Horseshoe kidney d. Premature gonadal failure e. Hypothyroidism 6. a newborm was evaluated in the nursery. Pregnancy was complicated by a prenatal ultrasound that showed coarctation of the aorta. This female infant was delivered at term by normal spontaneous vaginal delivery. On physical examination the baby is noted to have lymphedema of the feet and hands, webbed neck, and shield chest. The most likely diagnosis to explain the feature is: a. Down syndrome b. Kleinfelter syndrome c. Turner syndrome d. Marfan syndrome 7. all of the following are features of trisomy 18 except: a. Small palpebral fissures  b. Clenched hands c. Hypoplastic nails d. Protruding tongue, large cheeks e. Cardiac defect 8. which of the following are features of trisomy 13? a. Microphthalmia, cleft lip and palate, polydactyly, holoprosencephaly b. Up-slanting palpebral fissures, epicanthal folds, clinodactyly c. Hypotonia, rocker bottom feet, clenched hand, low set malformed ears d. Obesity, hypogenitalism, almond shaped eyes, small hands and feet e. Supravalvular aortic stenosis, hypercalcemia, short stature 9. the most common cause of trisomy 21 is: a. Maternal non-disjunction at meiosis with increasing risk associated with advanced maternal age b. Non-disjunction associated with advanced paternal age c. Mosaicism d. Sporadic events unrelated to maternal age e. Environmental exposures 10. Which of the following investigation does not need to be considered in a newborn suspected of having Down syndrome? a. Echocardiogram b. Thyroid testing c. Complete blood count d. Renal ultrasound e. None of these is necessary 11. All of the following are associated with medical problems in children with down syndrome except: a. Hearing loss b. Polycystic kidneys c. Eye problems d. Atlantoaxial instability e. Sleep apnea 12. Which of the following is not a common feature of Down syndrome in the newborn infant? a. Small low set ears with over folded upper helix b. Transverse palmar (simian) crease c. Increased gap between toes 1 and 2 d. Rocker bottom feet e. Excess nuchal skin fold 13. Indications for genetic counseling are: a. Maternal age >35 yr b. Maternal age >29 yr c. Paternal age >50 yr  d. Paternal age >35 yr 14. In a child with failure to thrive, the indications for hospitalization include severe malnutrition or failure of outpatient management. The period after which the child needs hospitalization, if he has not been responded to outpatient management, is about: a. 1-2 wk b. 4 wk c. 2-3 mo d. 4 mo e. 5-6 mo 15. Which of the following statements is false? a. Birth weight triples during the 1st year and quadruples by 2yr of age b. Birth length increases during the 1st year of life and doubles by 4 year of age c. The period starting after 6 months until 15 months is characterized by the acquisition of self- feeding skills because the infant can grasp finger foods, learn to use a spoon, and eat soft foods d. Guidelines for vitamin D supplementation recommend a daily vit D intake of 800 IU/day for children, and adolescents who are ingesting