BMS Study Smart Pediatric Review Dr. Jinan Darwish MBBCh , LRCPSI, BAO ( NUI) CABP 18th May, 2013

Cleft lip& palate ( OSCE) Represents failure of the palatal shelves to approximate or fuse. incidence of cleft palate is ≈1/2,500 births Possible causes include maternal drug exposure, a syndrome-malformation complex, or genetic factors (autosomal dominant) The timing of surgical correction should be individualized. Usually closure of the palate is usually done before 1 yr of age Recurrent otitis media and hearing loss, malposition of the teeth, speech defects,velopharyngeal incompetence.

Anemia ( MCQs/ OSCE) A- Aplastic Hb , Plt, neutrophils Normal MCV Low/absent retics B-Hemolytic Hb MCV (retics) LDH Unconjugated bilirubin haptoglobin C-Iron deficiency Hb ferriten Iron TIBC HCT MCV

Cushing’s syndrome: Common iatrogenic problem in paeds ex Cushing’s syndrome: Common iatrogenic problem in paeds ex. Steroids in Asthma

congenital hypothyroidism: ( OSCE)

Congenital Hypothyroidism Investigations: Universal neonatal screening Blood Wrist & hand xray Radio-active technetium scan Echocardiogram Management: Thyroxine Replacement Regular Follow up Complications: Excess thyroxine –advanced bone age Severe mental neurological delay

Cystic Fibrosis (MCQs) Autosomal recessive inheritence Defective CFTR Neonates 15% meconium ileus presentation Sweat test ( gold standard) Complications: Resp (cor pulmonale, pneumthorax) GI (Cirrhosis, portal HTN, DIOS) Endocrine ( DM, infertility 99% men & subfertility in females) Psychological/Behavioural

Cystic Fibrosis CF( gene CFTR mutation F508 sweat chloride Chloride concentration > 60mEq/L Cystic Fibrosis CF( gene CFTR mutation F508

Developmental Dysplasia of the hip (MCQs) Associations: FHx ( first degree relative , 20%); first born ( smaller uterus), oligohydramnios, breech dlivery, LSCS Examination : OrtolAni: Abduct&Anterior ; Relocate a dislocated hip Barlow : Adduct & posterior; Dislocates unstable hip.

Developmental Dysplasia of the hip Diagnosis: USS hip: High false + rate in infants <6 weeks Xray hip: >4 months ( 2 views: adduction & abduction) Management: Supportive: Pavlik harness abduction splints until 5-6 months.USS monitoring Surgical correction : Late diagnosis ( > 6 months)

Diabetes mellitus 1 ( MCQs) RBS > 11.0 mmol/L Fasting > 7.0 mmol/L HbA1c >7.5% Urinalysis: Ketonuria, glycosuria ( Stable product of non-enzymatic irreversible glycosylation of β-chain of HB by plasma glucose) DKA

Down Sydrome Trisomy 21 (OSCE)

Febrile Seizures Rule of 6 : peak 18 months Usually arise from infection/inflammation outside the CNS in an otherwise neurologically normal child. Seizures arising from fever due to meningitis/encephalitis are not included in the definition. Simple: Isolated,brief,generalized clonic/tonic seizure. Complex : > 15 mins, focal features,repeat seizure within same illness or incomplete recovery from seizure < 1 hour. Febrile status epilepticus: > 30mins ( up to 5% present s status epilepticus) Differential Diagnosis: CNS infection, epilepsy ( 1-2% will develop it)

G6PD deficiency ( MCQ)

Fluids ( MCQ)

Pearl of wisdom

Henoch-Schönlein purpura (MCQ/OSCE)

Hydrocele (OSCE) -Small hydroceles in infancy are benign and spontaneously resolve by 9-12 months of age. -Large hydroceles rarely resolve and may cause vascular compromise and testicular atrophy; these should be resected. -A communicating hydrocele (which changes in size) indicates a completely patent processus vaginalis and has the potential for hernia formation. This variety should also be repaired

Hydrocephalus ( OSCE) Obstructive Communicating CSF production Mx: Shunt with a one-way valve from from ventricle to peritoneum or right atrium. Complications: Shunt: obstruction,infection (staph.epidermidis, over drainage subdural haemorrhage. Long-term : Global developmental delay, impaired memory & vision, precocious puberty.

Hypospadias ( OSCE) With / without chordee Most are distal Usually isolated If assoc with undesc. testis: ambiguous genitalia workup. Surgical correction between 6-12 months

Infantile Glaucoma (OSCE) Triad: tearing, Photophobia, blepharospasm Signs: Large corneas, injection, edema. Primary: a)Congenital b)Syndromes: Sturge Weber, NF-1,Marfan, Stickler, HCR, OCR(Lowe) C)Ocular abnormalities: Secondary: Infection(TORCH)/cataracts surgery/trauma/tumor Treatment primarily surgical

Inflammatory Bowel Disease (MCQ)

Intussusception (MCQ) - The most common cause of intestinal obstruction between 3 mo and 6 yr of age. -80% of the cases occur before 24 mo - The male: female ratio is 4:1. - if left untreated, most will lead to intestinal infarction, perforation, peritonitis, and death. - Air, hydrostatic (saline), and, less often, water-soluble contrast enemas have replaced barium examinations - Air reduction is associated with fewer complications and lower radiation exposure than traditional contrast hydrostatic techniques.

Kawasaki Disease (MCQ/ OSCE) KAWASAKI DISEASE DIAGNOSTIC CRITERIA Kawasaki is also called Muco Cutaneous Lymph Node Syndrome or MCLNS Diagnostic criteria are  Fever for > 5 days + at least 4 of the following 5 criteria MCLNS M = Mucous membrane changes: injected pharynx, strwberry tongue, injected dry cracked lips C = Conjunctivitis: non purulent and bilateral (D/D w scarlet fever) L = Limb changes: edema, erythema and periungueal desquamation N = Nodes enlargement in the Neck: usulally > 1.5 cm in diameter S = Scarlattiniform rash 

Labial adhesions (MCQ/ OSCE) -Common disorder in prepubertal females. Peak incidence around the age of 13-23 months -The disorder is usually asymptomatic - May be associated with postvoid dribbling, also called vaginal voiding and occasionally UTIs -Treatment of labial adhesions is typically conservative. If left untreated, labial adhesions usually spontaneously resolve (80% within 1 year) - If treatment is necessary based on symptoms or parental request, estrogen cream is indicated twice daily for 2-4 weeks .Once the labia separate, apply emollient 3-5 times a day for several months to allow complete healing and prevention of recurrence.

Measles (MCQ/ OSCE) Measles: complications "MEASLES COMP" (complications): Myocarditis Encephalitis Appendicitis Subacute sclerosing panencephalitis Laryngitis Early death Sh!ts (diarrhoea) Corneal ulcer Otis media Mesenteric lymphadenitis Pneumonia and related (bronchiolitis-bronchitis-croup) 7 Cs of Measles? Cough Coryza Conjunctivitis Concurrent fever and rash Coplik (koplik  spot Cephalocaudal spreading rash Cervical lymphadenopathy

Measles Measles is caused by the paramyxovirus, and is highly contagious.  The incubation period is on average 10 days (7-18 days), and patients are usually considered infectious for several days before and after the onset of the rash when the viral load is highest. There is a prodromal illness which consists of the 3 C's (cough, coryza and conjunctivitis). Koplik spots (seen in the buccal mucosa) are pathognomonic for measles and are usually seen several days after onset of the ilness. Described as being like "grains of sand" Rash starts 3-4 days after onset of symptoms. Starts behind the ears and forehead, spreading over the whole body from head to foot. The rash is dark red macules and papules which become confluent and blotchy. Rash usually lasts for 5-10 days and classically desquamates as it resolves. Patients should be isolated for at least 5 days after appearance of rash. Any contacts if not immune should be vaccinated within 72 hours. Anti-measles IgM is generally detectable 3 days after onset of rash. Anti-measles IgG has a delayed onset before becoming positive - usually peaks at 14 days after onset of rash. Other diagnostic tests include NPA for measles IF and first pass urine for measles PCR.

Meningitis (OSCE)

Neonatal Jaundice (MCQ)

Pneumonia (OSCE/ MCQ)

Pneumothorax (OSCE) What is this five week old baby’s chest xray showing? Answer: spontaneous right-sided tension pneumothorax Name objects labeled by arrows? Answer: Blue- ECG lead Red – Endotracheal tube (ETT) Green- Nasogastric tube (NGT) How would you treat? Answer: Insert right intercostral drain (ICD)  

Pyloric Stenosis (MCQ) Multifactorial inheritence 30% first-born males M:F 4:1 7% FHx (parental) 12% Associated Nephrotic Syndrome Progressive non-bilous vomiting within 30 mins of feed; may become projectile. Hypochloraemic Hypokalemic Alkalosis USS abdomen: Pyloric muscle diameter > 3-4 mm & pyloric channel > 18mm in length. Rx: Ramstedt pyloromyotomy

Rickets ( MCQ/ OSCE)

Retinoblastoma (OSCE) Leukocoria Absent red reflex Should not be missed

Scabies (OSCE) Sarcoptes scabei Mechanism of spread: prolonged direct human contact( >20mins)- holding hands/playing contact games.Fomite (towels,underclothing,toilet seats) Rx: Permethrin 5% dermal cream applied to all areas below the neck overnight. Malathion 0.5% (Allergic to Permethrin) Mittens < 2 years prevents excoriation & secondary infection.

SCD mode of inheritence; premarital & new born screening, how to read HPLC

Talipes Equinovarus (Club foot) Features: CAVE (cavus, adductus, varus, equinus). 1) Plantar flexion of foot at ankle 2) Inversion of heel 3) Medial Deviation of forefoot. Categories: 1) Idiopathic 2) Neurogenic 3) Syndromic(Larsen sn, Arthrog) Always check for assoc. anomalies, specially spine. Early treatment(first day): Casting , rapid correction Late treatment: Surgical

Tetralogy of Fallot (MCQ) Most common cyanotic disease. components PROVe Pulmonary Hypertension Right Ventricular Hypertrophy Overriding Aorta VSD

Thalassemia( OSCE)

Transposition of Great Arteries Most common cyanotic disease in first 24 hours

Turner Syndrome (MCQ/ OSCE) Turner Syndrome Patients look like CLOWNS (only to remember its features): C - Cardiac anomalies (most common - coarctation of aorta) L - Lymphoedema, low thyroid O - Ovaries under developed (streak ovaries), primary Amenorrhea W - Webbed neck N - Nipples widely placed S - Short stature, Sensoneural hearing loss, Short 4th metacarpal

Undescended Testis (MCQ/ OSCE) 4.5% of boys have undesc. Testis at birth 0.8% by age 6 months Will not descend after 4 months of age Histopathologic changes demonstratable by 6-12 months of age. Surgical correction at 6months of age(no later than 9-15months)- success rate of 98% LHRH, HCG: Not effective

Varicella ( chicken pox) ( MCQ/ OSCE)

MCQs

1. A patient with acute asthma is most likely to have decreased Forced expiratory volume in 1 second Residual volume Functional residual capacity Total lung capacity Tidal volume

2. A 14-month old infant has had a clear nasal discharge and a mild cough for about two weeks. Despite therapy with Amoxicillin, his condition has not improved. The cough has worsened and the infant has been vomiting at each feeding. The leukocyte count is 20,000/mm3 with 80% lymphocytes, 15% neutrophils and 5% band forms. X-ray study of the chest shows bilateral perihilar infiltrates with shaggy heart border. The most likely diagnosis is Acute bronchial asthma Acute lympocytic leukemia with pulmonary infiltration Influenza virus infection Pertusis Hemophilus influenzae pneumonia

3.Regarding steroid inhaler therapy for a 4-year old child with asthma, you should tell the mother It is most effective with acute exacerbation It should be administered every two hours when the child is having an acute episode of asthma The primary value is in the prevention of episodes of asthma It will not be of value in the management of the child’s exercise-induced episodes of asthma The most common side effect is the development of oral candidiasis.

Of the following , the most likely diagnosis is 4. A 2-1/2 year old child has had cough for two weeks after having been hospitalized one month ago for pneumonia in the right lower lobe. X-ray study of the chest reveals persistence of pneumonia in the right lower lobe. Of the following , the most likely diagnosis is Staphylococcal pneumonia Foreign body aspiration Cystic Fibrosis Pneumonia due to Mycoplasma pneumoniae Pneumonia due to respiratory synctial virus

5. School phobias (school refusals) in grade-school children most commonly result from Fear of a strict teacher Concern about failing school Fear of separation from parent Desire to avoid unfriendly classmates Inability to do school work

6. You are discussing with a resident who has just evaluated a 4-year-old who has classic varicella lesions and a history that is consistent with this diagnosis. He put small pox as D/D. Of the following, the MOST accurate statement about the tow pathologies is that lesions of both varicella and smallpox follow a 7- to 10-day course from eruption to resolution lesions of both varicella and smallpox frequently produce deep, pitted scars varicella lesions are transient vesicles; smallpox lesions are persistent pustules until resolution of the illness varicella lesions are concentrated on the face; smallpox lesions are concentrated over bony Prominences varicella lesions appear in stages or crops; smallpox lesions are uniformly in the same stage of development

7. A 13 year old female presents with hypertension and increasing weight. Which of the following features would be most suggestive of Cushing’s syndrome rather than simple obesity? Moon face. Acanthosis Nigricans Abdominal striae. Proximal myopathy Buffalo Hump

8. A 24 month old boy presents with high fever of 6 days duration, tender neck, red eyes and cracked lips. In the second week he developed maculo-papular erythematous rash and blanching. What is the most likely diagnosis Kawasaki disease Stevens-Johnson syndrome Acute toxoplasmosis Meningococcal sepsis Measles.

Which on of the following is a common adverse effect of this 9. A 12 year old female underwent a renal transplant. She was concerned about the effects of long-term Cyclosporin treatment. Which on of the following is a common adverse effect of this Alopecia. Paraesthesiae. Hepatotoxicity Nephrotoxicity. Bone marrow depression

10. A 7 years old male presented with a strongly positive Mantoux test 10. A 7 years old male presented with a strongly positive Mantoux test. Which one of the following statements regarding his immune reaction is correct? The reaction typically develops within 24 hours It is a cell mediated immune response The area of induration will be less than 10 mm in diameter The response is mediated by B lymphocytes If a skin biopsy were taken, immunohistochemistry would show immune complex deposition.

11. A 5-year old girl is brought to your office by her mother who reports that the child is one of four children in her nursery school class who have similar rashes. Two days ago the patient had a low grade fever with the temperature reaching 38 C . The cheeks became red. She denied having sore throat. Today she has a generalized, discrete maculopapular rash. The most likely diagnosis is Rubella Measles Scarlet fever Erythema infectiosum Pityriasis rosea

Of the following agents, you should first administer 12. Stridor, tachypnea and wheezing develop suddenly in a 10-year old girl who has received an injection of contrast medium for intravenous pyelography. Large urticarial lesions appear over the face , chest and extremities. Of the following agents, you should first administer Diphenhydramine Epinephrine Theophylline Corticosteroids Antibiotics

The infant most likely has 13. A 4-week old girl was found shortly after birth to have a cardiac murmur. Her color is now good and weight gain has been satisfactory. Pulse rate is 160/min and respiratory rate is 70/min. Her mother has noted that the infant sweats despite normal ambient temperature and that she sleeps poorly. The infant most likely has Pneumonia Sepsis Paroxysmal Atrial tachycardia A left to right shunt circulation A Septal defect that has begun to close

Advise mother that breast feeding is contraindicated. 14. A woman who is positive for hepatitis B surface antigen (HbsAg) but negative for hepatitis Be antigen (HbeAg), delivers at term. After careful bathing, the BEST management for this woman’s infant would be to: Administer gamma globulin intramuscularly immediately and at 1 month of age. Administer HBIG and HB vaccine immediately and HB vaccine again at 1 month and 6 months of age. Administer hepatitis B immunoglobulin (HBIG) if cord blood is positive for HBSAg. Administer hepatitis B (HB) vaccine immediately and at 1 month and 6 months of age Advise mother that breast feeding is contraindicated.

15. The parents of an 18-month old boy bring the infant to you as a new patient. They state that he has been constipated since early infancy. He has been managed with fair success with glycerin suppositories and laxatives. Physical examination discloses no distention of the abdomen. A copious amount of fecal material is palpated in the large bowel. The rectal ampulla is empty. The management of choice would be to Continue the present treatment regimen Continue the present treatment regimen but add more bulk to the diet Prescribe mineral oil Order a barium enema examination Discontinue all medications and observe the patient

The most likely diagnosis is 16. A 2-month old boy is hospitalized because of cough and rapid respirations. The infant has been well since birth except for a mild mucoid conjunctivitis. Length and weigh are at the 15th percentile. An x-ray study of the chest reveals hyperinflation and bilateral interstitial pneumonia. His temperature is 38.0C and his respiratory rate is 56/min. Leukocyte count is 10,000/mm3 with 60% Neutrophil, 5% band forms, 35% lymphocytes, and 10% eosinophils. The serum IgM concentration is markedly increased. The most likely diagnosis is Pertussis Pneumonia due to Chlamydia Trachomatis Pneumonia due to Myoplasma pneumoniae Pneumonia due to Hemophillus Influenzae Pneumonia due to Staphylococcus Aureus

17. An infant who has not yet passed meconium begins vomiting when he is 36 hours old. His abdomen has doughy distension. The rectum is empty on examination, no meconium was seen on the examiner’s finger. An x-ray study of the abdomen shows distended intestinal loops, but fluid levels are not present. The abdomen has a granular, ground glass appearance with flecks of calcium scattered throughout. The most likely diagnosis is Aganglionic megacolon (Hirschsprung’s disease) Intestinal Atresia Meconium plug syndrome Ruptured appendix Cystic fibrosis, Meconium ileus and peritonitis

18. In infants with serious dehydration, the clinical signs of tachycardia , poor turgor and cool distal extremities result primarily from metabolic acidosis Starvation Hypovolemia Cortisol deficit Oliguria

19. A 12 years old girl presented with acute Guillain-Barre syndrome and has developed worsening proximal muscle weakness. Which one of the following tests should be used to monitor her respiratory function? Arterial blood gas. Vital capacity. FEV1/FVC ratio. Peak expiratory flow rate. Chest expansion

20. A child with chronic renal failure has persistent acidosis with an increased anion gap. Increased serum concentration of which of the following substances would likely contribute most to this finding? Lactic acid Protein Urea Inorganic acids Keto acids

The most likely diagnosis is 21. Hyperpnea, lethargy and fever have developed in a 2 year old girl with rheumatoid arthritis. She is not dehydrated or disoriented. There is no nuchal rigidity on physical examination. The lungs are clear. Findings on x-ray study of the chest are normal. Blood oozes from a venipuncture site. Urinalysis reveals reducing substance but no ketones. An aunt has diabetes mellitus. The most likely diagnosis is Diabetic Ketoacidosis Reactive airways disease Gastroenteritis with acidosis Salicylate intoxication Oranophosphate ingestion

B. hospitalization with intravenous fluids and gut rest for 24 hours 22- A 2-year-old boy presents with a 3-day history of diarrhea and vomiting. He has been able to tolerate small amounts of fluids. He is moderately dehydrated, with dry mucous membranes and a heart rate of 145 beats/min. Of the following, the BEST management for this patient's fluid status is A. hospitalization with intravenous fluids and a restrictive bland diet B. hospitalization with intravenous fluids and gut rest for 24 hours C. oral rehydration therapy at home followed by a diet of fruits, vegetables, and meats D. oral rehydration therapy at home followed by a clear liquid diet for 24 hours E. oral rehydration therapy at home followed by a restrictive bland diet

23. The parents of a child who has Down syndrome and a 47,XX+21 karyotype come to you for counseling about future pregnancies. Of the following, their risk for giving birth to another child who has trisomy is CLOSEST to no greater than the general population at risk 1% added to the mother's age-related risk 5% added to the mother's age-related risk 10% added to the mother's age-related risk 25% added to the mother's age-related risk

24. An 8-year old boy is brought to your office because he has fever, bilateral knee pain, and a limp. The patient is a black child with generalized lymphadenopathy, an enlarge spleen and a swollen, tender knee. Laboratory studies reveal a hemoglobin concentration of 5 g/dL and a leukocyte count of 2500 mm3 with 80% lymphocytes. Which of the following studies would be most helpful in confirming the diagnosis? Erythrocyte sedimentation rate Heterophil titer Coagulation profile Examination of a bone marrow aspirate X-ray study of the knee

25. A diagnosis of Kawasaki Syndrome has been made in a 6-year old girl. Four days after hospitalization, abdominal pain and distention develop. These developments are most likely due to: Hydrops of the gallbladder Enterocolitis Intussusception Volvulus Hepatic Haematoma

1. A patient with acute asthma is most likely to have decreased Forced expiratory volume in 1 second Residual volume Functional residual capacity Total lung capacity Tidal volume

2. A 6-year old boy suffers major abdominal trauma in a motor vehicle accident. One hour later in the emergency department, blood pressure is 65/40 mmHg. Hemoglobin concentration is 6.0 g/dL. Arterial blood gas and pH analysis while the patient is breathing room air shows pH 7.47, PO2 45 mmHg and PCO2 30 mmHg, FiO2 is 0.21. An x-ray study of the chest reveals diffuse bilateral pulmonary infiltrates. Which of the following will provide the greatest improvement in oxygen transport? Vasopressors Intubation and ventilation with positive end-expiratory pressure (PEEP) Administration of 100% oxygen Blood transfusion Diuretics

The most likely diagnosis is Acute bronchial asthma 3. A 14-month old infant has had a clear nasal discharge and a mild cough for about two weeks. Despite therapy with Amoxicillin, his condition has not improved. The cough has worsened and the infant has been vomiting at each feeding. The leukocyte count is 20,000/mm3 with 80% lymphocytes, 15% neutrophils and 5% band forms. X-ray study of the chest shows bilateral perihilar infiltrates with shaggy heart border. The most likely diagnosis is Acute bronchial asthma Acute lympocytic leukemia with pulmonary infiltration Influenza virus infection Pertusis Hemophilus influenzae pneumonia

4.Regarding steroid inhaler therapy for a 4-year old child with asthma, you should tell the mother It is most effective with acute exacerbation It should be administered every two hours when the child is having an acute episode of asthma The primary value is in the prevention of episodes of asthma It will not be of value in the management of the child’s exercise-induced episodes of asthma The most common side effect is the development of oral candidiasis.

5. A 2-1/2 year old child has had cough for two weeks after having been hospitalized one month ago for pneumonia in the right lower lobe. X-ray study of the chest reveals persistence of pneumonia in the right lower lobe. Of the following , the most likely diagnosis is Staphylococcal pneumonia Foreign body aspiration Cystic Fibrosis Pneumonia due to Mycoplasma pneumoniae Pneumonia due to respiratory synctial virus

6. School phobias (school refusals) in grade-school children most commonly result from Fear of a strict teacher Concern about failing school Fear of separation from parent Desire to avoid unfriendly classmates Inability to do school work

7. A 6-year old boy suffers major abdominal trauma in a motor vehicle accident. One hour later in the emergency department, blood pressure is 65/40 mmHg. Hemoglobin concentration is 6.0 g/dL. Arterial blood gas and pH analysis while the patient is breathing room air shows pH 7.47, PO2 45 mmHg and PCO2 30 mmHg, FiO2 is 0.21. An x-ray study of the chest reveals diffuse bilateral pulmonary infiltrates. Which of the following will provide the greatest improvement in oxygen transport? Vasopressors Intubation and ventilation with positive end-expiratory pressure (PEEP) Administration of 100% oxygen Blood transfusion Diuretics

8. You are discussing with a resident who has just evaluated a 4-year-old who has classic varicella lesions and a history that is consistent with this diagnosis. He put small pox as D/D. Of the following, the MOST accurate statement about the tow pathologies is that lesions of both varicella and smallpox follow a 7- to 10-day course from eruption to resolution lesions of both varicella and smallpox frequently produce deep, pitted scars varicella lesions are transient vesicles; smallpox lesions are persistent pustules until resolution of the illness varicella lesions are concentrated on the face; smallpox lesions are concentrated over bony Prominences varicella lesions appear in stages or crops; smallpox lesions are uniformly in the same stage of development

9. A 16 years old female presents with hypertension and increasing weight. Which of the following features would be most suggestive of cushing’s syndrome rather than simple obesity? Moon face. Acanthosis Nigricans Abdominal striae. Proximal myopathy Buffalo Hump

10. A 24 month old boy presents with high fever of 6 days duration, tender neck, red eyes and cracked lips. In the second week he developed maculopapular erythematous rash and blanching. What is the most likely diagnosis Kawasaki disease Stevens-Johnson syndrome Acute toxoplasmosis Meningococcal sepsis Measles.

Which on of the following is a common adverse effect of this 11. A 15 years old female underwent a renal transplant. She was concerned about the effects of long-term Cyclosporin treatment. Which on of the following is a common adverse effect of this Alopecia. Paraesthesiae. Hepatotoxicity Nephrotoxicity. Bone marrow depression

12. A 7 years old male presented with a strongly positive Mantoux test 12. A 7 years old male presented with a strongly positive Mantoux test. Which one of the following statements regarding his immune reaction is correct? The reaction typically develops within 24 hours It is a cell mediated immune response The area of induration will be less than 10 mm in diameter The response is mediated by B lymphocytes If a skin biopsy were taken, immunohistochemistry would show immune complex deposition.

13. A 5-year old girl is brought to your office by her mother who reports that the child is one of four children in her nursery school class who have similar rashes. Two days ago the patient had a low grade fever with the temperature reaching 38 C . The cheeks became red. She denied having sore throat. Today she has a generalized, discrete maculopapular rash.The most likely diagnosis is Rubella Measles Scarlet fever Erythema infectiosum Pityriasis rosea

Of the following agents, you should first administer 14. Stridor, tachypnea and wheezing develop suddenly in a 10-year old girl who has received an injection of contrast medium for intravenous pyelography. Large urticarial lesions appear over the face , chest and extremities. Of the following agents, you should first administer Diphenhydramine Epinephrine Theophylline Corticosteroids Antibiotics

The infant most likely has 15. A 4-week old girl was found shortly after birth to have a cardiac murmur. Her color is now good and weight gain has been satisfactory. Pulse rate is 160/min and respiratory rate is 70/min. Her mother has noted that the infant sweats despite normal ambient temperature and that she sleeps poorly. The infant most likely has Pneumonia Sepsis Paroxysmal Atrial tachycardia A left to right shunt circulation A Septal defect that has begun to close

Advise mother that breast feeding is contraindicated. 16. A woman who is positive for hepatitis B surface antigen (HbsAg) but negative for hepatitis Be antigen (HbeAg), delivers at term. After careful bathing, the BEST management for this woman’s infant would be to: Administer gamma globulin intramuscularly immediately and at 1 month of age. Administer HBIG and HB vaccine immediately and HB vaccine again at 1 month and 6 months of age. Administer hepatitis B immunoglobulin (HBIG) if cord blood is positive for HBSAg. Administer hepatitis B (HB) vaccine immediately and at 1 month and 6 months of age Advise mother that breast feeding is contraindicated.

17. The parents of an 18-month old boy bring the infant to you as a new patient. They state that he has been constipated since early infancy. He has been managed with fair success with glycerin suppositories and laxatives. Physical examination discloses no distention of the abdomen. A copious amount of fecal material is palpated in the large bowel. The rectal ampulla is empty. The management of choice would be to Continue the present treatment regimen Continue the present treatment regimen but add more bulk to the diet Prescribe mineral oil Order a barium enema examination Discontinue all medications and observe the patient

The most likely diagnosis is 18. A 2-month old boy is hospitalized because of cough and rapid respirations. The infant has been well since birth except for a mild mucoid conjunctivitis. Length and weigh are at the 15th percentile. An x-ray study of the chest reveals hyperinflation and bilateral interstitial pneumonia. His temperature is 38.0C and his respiratory rate is 56/min. Leukocyte count is 10,000/mm3 with 60% Neutrophil, 5% band forms, 35% lymphocytes, and 10% eosinophils. The serum IgM concentration is markedly increased. The most likely diagnosis is Pertussis Pneumonia due to Chlamydia Trachomatis Pneumonia due to Myoplasma pneumoniae Pneumonia due to Hemophillus Influenzae Pneumonia due to Staphylococcus Aureus

19. In infants with serious dehydration, the clinical signs of tachycardia , poor turgor and cool distal extremities result primarily from metabolic acidosis Starvation Hypovolemia Cortisol deficit Oliguria

20. An infant who has not yet passed meconium begins vomiting when he is 36 hours old. His abdomen has doughy distension. The rectum is empty on examination, no meconium was seen on the examiner’s finger. An x-ray study of the abdomen shows distended intestinal loops, but fluid levels are not present. The abdomen has a granular, ground glass appearance with flecks of calcium scattered throughout. The most likely diagnosis is Aganglionic megacolon (Hirschsprung’s disease) Intestinal Atresia Meconium plug syndrome Ruptured appendix Cystic fibrosis, Meconium ileus and peritonitis

21. A 12 years old girl presented with acute Guillain-Barre syndrome and has developed worsening proximal muscle weakness. Which one of the following tests should be used to monitor her respiratory function? Arterial blood gas. Vital capacity. FEV1/FVC ratio. Peak expiratory flow rate. Chest expansion

22. A child with chronic renal failure has persistent acidosis with an increased anion gap. Increased serum concentration of which of the following substances would likely contribute most to this finding? Lactic acid Protein Urea Inorganic acids Keto acids

The most likely diagnosis is 23. Hyperpnea, lethargy and fever have developed in a 2 year old girl with rheumatoid arthritis. She is not dehydrated or disoriented. There is no nuchal rigidity on physical examination. The lungs are clear. Findings on x-ray study of the chest are normal. Blood oozes from a venipuncture site. Urinalysis reveals reducing substance but no ketones. An aunt has diabetes mellitus. The most likely diagnosis is Diabetic Ketoacidosis Reactive airways disease Gastroenteritis with acidosis Salicylate intoxication Oranophosphate ingestion

B. hospitalization with intravenous fluids and gut rest for 24 hours 24- A 2-year-old boy presents with a 3-day history of diarrhea and vomiting. He has been able to tolerate small amounts of fluids. He is moderately dehydrated, with dry mucous membranes and a heart rate of 145 beats/min. Of the following, the BEST management for this patient's fluid status is A. hospitalization with intravenous fluids and a restrictive bland diet B. hospitalization with intravenous fluids and gut rest for 24 hours C. oral rehydration therapy at home followed by a diet of fruits, vegetables, and meats D. oral rehydration therapy at home followed by a clear liquid diet for 24 hours E. oral rehydration therapy at home followed by a restrictive bland diet

25. The parents of a child who has Down syndrome and a 47,XX+21 karyotype come to you for counseling about future pregnancies. Of the following, their risk for giving birth to another child who has trisomy is CLOSEST to no greater than the general population at risk 1% added to the mother's age-related risk 5% added to the mother's age-related risk 10% added to the mother's age-related risk 25% added to the mother's age-related risk

26. An 8-year old boy is brought to your office because he has fever, bilateral knee pain, and a limp. The patient is a black child with generalized lymphadenopathy, an enlarge spleen and a swollen, tender knee. Laboratory studies reveal a hemoglobin concentration of 5 g/dL and a leukocyte count of 2500 mm3 with 80% lymphocytes. Which of the following studies would be most helpful in confirming the diagnosis? Erythrocyte sedimentation rate Heterophil titer Coagulation profile Examination of a bone marrow aspirate X-ray study of the knee

27. A diagnosis of Kawasaki Syndrome has been made in a 6-year old girl. Four days after hospitalization, abdominal pain and distention develop. These developments are most likely due to; Hydrops of the gallbladder Enterocolitis Intussusception Volvulus Hepatic Haematoma

OSCE Blood: CBC, LFT, TORCH,HPLC, Radiology: CXR, KUB ECG: Hyperkalemia, VT, SVT,WPW,VF Pictures: Syndromes Growth charts: short stature, FTT, obesity Equipment

Station 1 1- Name this condition 2-Who does it affect more males or females? 3-List the components of the triad 4-Which cardiac anomalies are associated with it?

Station 2 1- What is the diagnosis? 2-What are the causes? 3-How would you proceed in investigating it?

Prune Belly Syndrome: -AKA Eagle-Barrett Syndrome -Three main problems: Poor development of the abdominal muscles,(cryptorchidism) and urinary tract problems -The causes of prune belly syndrome are unknown -The condition affects mostly boys (95%). -The amniotic fluid volume may be normal or decreased in neonates with prune belly syndrome. -The presence of oligohydramnios may account for some of the accompanying findings of the extremities -Poor abdominal wall muscles : poor coughing and constipation -Mortality rate 20% -Patients with prune belly syndrome also have an increased incidence of TOF and ventriculoseptal defects.

Congenital Cataracts: -Definition: Opacification of the lens. Congenital cataracts usually are diagnosed at birth. -Patients require a full metabolic, infectious, systemic, and genetic workup. -Common causes are hypoglycemia, trisomy (eg, Down, Edward, and Patau syndromes), myotonic dystrophy, TORCH, and prematurity. -Workup: For unilateral cataracts: TORCH titers and Venereal Disease Research Laboratory (VDRL) test. -For bilateral cataracts: CBC, BUN, TORCH titers, VDRL, urine for reducing substances, red cell galactokinase, urine for amino acids, calcium, and phosphorus. -Additional Tests: CT scan of brain. Hearing test

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