OSCE in Pediatrics Vivek Jain, RG Holla, Manish Mittal
Chapter Notes

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Connective Tissue1

  • Q1. This 8-month-old male infant had deformities of the limbs since birth due to fractures of multiple bones.
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    1. Identify the condition.
    2. How is the condition inherited?
    3. What is the biochemical defect?
    4. What are the medical treatment options available for this condition?
    5. What is the triad of this disease?2
  • Q2. This 3-year-old male child presented with pain in abdomen for past 7 days (colicky), swelling over dorsum of hands and feet and pain in both ankles with swelling of right knee. Two days ago, the child developed a rash over the lower limbs.
    1. What is your most probable diagnosis?
    2. Which antimicrobial agent is used in the treatment of this condition?
    3. What is the specific medical treatment for this condition?
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  • Q3. A 2 ½-year-old boy is brought with history of high grade, continuous fever, without chills and rigors, since one week. The parents deny history of diarrhea, coryza, vomiting or headache. Clinically, the child is conscious, irritable, febrile, normotensive, with no neck stiffness. There is conjunctival congestion, crusting of the skin around the lips and two discrete left sided cervical lymph nodes measuring 2 cm in size. Systemic examination is normal. The photograph of the child is shown.
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    1. What is your diagnosis?
    2. Name three laboratory abnormalities found on blood tests in this condition.
    3. What is the most common cause of death in above-mentioned patient?
    4. What drug (drug of choice) you would like to give to this patient?
    5. Meningitis can be one of complications (state true/false).
  • Q4. Male child aged 4 years is brought with history of painful swelling of both wrists, elbows, first metacarpophalangeal joints of both hands since 5 months, with low grade fever. There is no history of weight loss, rash, bleeding from any site or progressive pallor. Clinically, other than the swelling, stiffness and limitation of movement of the affected joints, there are no other significant findings.
    1. What is the most probable diagnosis?
    2. Name two investigations you would perform.
    3. Name three indications of the use of steroid in this disease.
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  • Q5. 2½-year-old boy was brought with history of sudden onset of limp followed by refusal to walk. The child was afebrile and there was no history of trauma. The child had been treated for an episode of loose stools with blood and mucus about two weeks back. Clinically, the child was afebrile, normotensive with no rash, lymphadenopathy, petechiae or pallor. The left hip was painful and movements were restricted. Other joints were normal. On investigations, other than a mildly elevated ESR, all investigations were within normal limits.4
    1. What is the most probable diagnosis and what is the most likely responsible agent?
    2. Name the HLA positivity which is associated with this disease.
    3. What is the outlook for this disease?
    4. Name four viruses which are associated with arthritis.
  • Q6. A 14-year-old girl has been brought with history of high grade fever since three weeks with progressive breathlessness and swelling of feet and abdomen. The fever is not associated with chills or rigors, dysuria, coryza, jaundice or alteration in bowel habits. The child had a generalized seizure lasting for 2 minutes today. Clinically, the child is drowsy but arousable is pale, has oral ulcers, arthritis of both knees and a left sided pleural effusion. There is no jaundice or neck stiffness. The cardiac examination reveals distant heart sounds. The liver is palpable 4 cm below the costal margin and free fluid is present. A 2D echo confirms a pericardial effusion.
    1. What is the most probable diagnosis?
    2. What investigation would confirm the diagnosis?
    3. What hematological parameters do you expect to find?
    4. Name two drugs that can lead to this syndrome.
  • Q7. Five-year-old girl presented with history of insidious onset of weakness and fatigue on walking. Parents report that the child is particularly reluctant to climb stairs and is unable to lift her hands to comb her hair. The child has also developed a reddish rash around the eyes, over the dorsum of the hands and on the knees and elbows. There is history of joint pains of all the joints and the child has progressively restricted her physical activity. There is history of a upper respiratory illness a month back. Clinically, the child is afebrile, with no pallor, jaundice, petechiae. There is a papular non-erythematous rash over the face and limbs. There is generalized arthralgia, with no evidence of arthritis. Neurological examination shows proximal muscle weakness.
    1. What is the most likely diagnosis in this patient?
    2. How would you investigate to reach a diagnosis?
    3. What is the treatment for isolated cutaneous lesions?
    4. Bed rest should be given to the child (State true or false).
  • Q8. An infant presents with history of excessive irritability and fever since 1 week. He has been under treatment for conjunctivitis for the past 3 days with ciprofloxacin eye drops. The child is febrile (temperature 102 °F, HR—150/min RR-38/min BP—100/60 mmHg). He has a fine rash over his upper chest and his throat and oral mucosa is congested. His liver is 3 cm and is tender to palpate.
    1. What is the most likely diagnosis?
    2. What drugs would you use for therapy? Mention doses.
    3. Name one finding on USG abdomen.
    4. Name 4 common differentials for this condition.5
  • Q9. A 26-days-old neonate is brought by the mother with a non-pruritic rash over the face and body noted since three weeks. The child has a heart rate of 60/min but is hemodynamically stable.
    1. What disease would you look for in the mother?
    2. What antibody is responsible for the disease?
    3. Name two hematological findings one would expect in this infant.
    4. What is the long term outlook for the dermatological manifestations?
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  • Q10.
    1. What is the dose of methotrexate in juvenile rheumatoid arthritis?
    2. Name two important side effects of methotrexate.
    3. Which micronutrient supplementation is necessary while giving methotrexate on a long-term basis?
    4. Name one skin condition where methotrexate is used.
    5. What is the mode of action?
  • Q11. A 12-year-old female had a 2 year history of chronic sinusitis. She had one episode of hemoptysis and respiratory distress during the past 2 years. Urine analysis reveals hematuria.
    1. What is the most likely diagnosis?
    2. What lab test can help in diagnosing this patient.
    3. What is the most important differential diagnosis for this patient?
  • Q12. Answer the following questions in relation to juvenile rheumatoid arthritis.
    1. Name the monoclonal antibody to TNF alpha, used in refractory cases of juvenile rheumatoid arthritis.6
    2. What is the drug of choice for long-term treatment of juvenile rheumatoid arthritis?
    3. What is the minimum number of joints involved to be classified as polyarticular arthritis?
    4. MAS in context of JIA refers to minimal access surgery (State true/false).
  • Q13. This male child, aged 11 years was admitted with history of easy fatigability and jaundice and high colored urine of three months duration. Clinically, the child was ambulant, afebrile and normotensive. There was icterus, but no pallor, lymphadenopathy, cutaneous bleeding or signs of fulminant or chronic liver disease. The liver and spleen were palpable as shown in the figure. Finding on eye examination is shown in the figure. Other systems were normal. The relevant lab results showed serum bilirubin of 2.5 mg/dL, direct positive, SGOT 243 U/L, SGPT 676 U/L, serum alkaline phosphatase 465 U/L. Other investigations including hematology, biochemistry, coagulation profile and serology for hepatitis were normal. The child was born of a consanguineous marriage and had an elder sibling who had jaundice, was unable to walk properly and was improving on medications.
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    1. What is the most probable diagnosis?
    2. What is the finding in the eye?
    3. How will you proceed to confirm the diagnosis? Name three confirmatory tests.
    4. How will you treat the child?
    5. What dietary advice would you give for the child?7
  • A 1:
    1. Osteogenesis imperfecta.
    2. Autosomal dominant.
    3. Structural/quantitative defect in type I collagen formation.
      1. Growth hormone—improves bone histology.
      2. Bisphosphonates (iv pamidronate or oral olpadronate)—decreases bone resorption by osteoclasts.
    4. Fragile bones, blue sclera and early deafness.
  • A 2:
    1. Henöch Schönlein purpura.
    2. Dapsone.
    3. Steroids.
  • A 3:
    1. Kawasaki disease.
    2. Thrombocytosis, raised ESR, elevated CRP, leucocytosis with neutrophilia, hypoalbuminemia.
    3. Coronary artery aneurysm rupture.
    4. Intravenous immunoglobulin, aspirin.
    5. True.
  • A 4:
    1. Juvenile inflammatory arthritis/juvenile rheumatoid arthritis (polyarticular type).
    2. Rheumatoid factor, antinuclear factor, ESR, CRP.
    3. During serious disease flare, bridging medication while changing to another line of treatment, intraarticular use for persistent limited joint disease, ocular control of uveitis.
  • A 5:
    1. Reactive arthritis due to Shigella.
    2. HLA B27.
    3. Good.
    4. Chikungunya, Parvovirus B19, Hepatitis B, Adenovirus, CMV, EBV, Varicella, Mumps, Enteroviruses (Echo, Coxsackie).
  • A 6:
    1. SLE.
    2. Anti ds-DNA, Anti-Smith antibody.
    3. Hemolytic anemia with reticulocytosis, leucopenia < 4000/cu mm, lymphopenia < 1500/cumm, thrombocytopenia < 100000/cu mm.
    4. Phenytoin, sulfonamides, procainamide, hydralazine.8
  • A 7:
    1. Dermatomyositis.
    2. Increased serum creatinine phosphokinase levels, SGOT and LDH levels and MRI of thigh (focal inflammatory myopathy) and muscle biopsy (perifascicular atrophy).
    3. Hydroxy chloroquine (maximum up to 5 mg/kg/day) with low dose steroids (1 mg/kg/day).
    4. False.
  • A 8:
    1. Kawasaki disease.
    2. IVIG 2 gm/kg slowly over 10–12 hours.
      Aspirin (initial 80–100 mg/kg/day q 6 hourly until 14th day of illness and then 3–5 mg/kg/day till 6–8 weeks after onset of illness).
    3. Hydrops gallbladder.
      1. Measles.
      2. Scarlet fever.
      3. Juvenile rheumatoid arthritis.
      4. Drug reaction/toxic shock syndrome.
  • A 9:
    1. SLE.
    2. Anti Ro SSA, anti Ro SSB.
    3. Neutropenia, thrombocytopenia.
    4. 25% of lesions scar. Remaining resolve in 3–4 months.
  • A 10:
    1. 5 to 15 mg/m2/week.
    2. Hepatotoxicity and megaloblastic anemia.
    3. Folic acid.
    4. Psoriasis.
    5. Antimetabolite. Inhibits DNA and purine synthesis.
  • A 11:
    1. Wagener granulomatosis.
    2. ANCA and anti-PR3 (Proteinase 3—normally PR-3 is restricted to neutrophils granules but in this disease they are on neutrophil surface.
    3. Churg-Strauss syndrome (Vasculitis—asthma, circulating eosinophilia and eosinophils on skin biopsy. There is no destructive upper airway disease).
  • A 12:
    1. Infleximab.
    2. Methotrexate.
    3. 5 or more.
    4. False: macrophage activation syndrome.9
  • A 13:
    1. Wilson disease.
    2. Sunflower cataract.
    3. Serum ceruloplasmin, serum copper, urinary copper excretion, hepatic copper content.
    4. Restrict copper to less than 1 mg/day, D-penicillamine 20 mg/kg/day, zinc 25 mg three times a day.
    5. Avoid copper containing foods: avoid storing or cooking in copper or brass vessels, liver, shellfish, nuts, chocolates.