Wednesday, August 26, 2009

35 - Juvenile Myoclonic Epilepsy ( Janz syndrome )

Here are some important points about Juvenile Myoclonic Epilepsy :

1. Juvenile Myoclonic Epilepsy is otherwise called Janz syndrome .

2. It usually begins between the ages of 12 and 16 year .

3. It accounts for approximately 5 % of all the epilepsies .

4. Patients note frequent myoclonic jerks on awakening , making hair combing and tooth-brushing difficult .

5. As the myoclonus tends to abate later in the morning, most patients do not seek medical advice at this stage and some deny the episodes .

6. A few years later, early morning generalized tonic-clonic seizures develop in association with the myoclonus.

7. The EEG shows a 4-6/sec irregular spike and wave pattern, which is enhanced by photic stimulation .

8. The neurologic examination is normal.

9. Majority of the patients respond dramatically to valproate, which is required lifelong .

10. Discontinuance of the drug causes a high rate of recurrence of seizures .

MCQs in Pediatrics 2nd edition
MCQs in Pediatrics ; Over 600 Questions with Explanatory Notes

34 - PGI Chandigarh december 2007 mcqs part 2

8q: True statements about juvenile myoclonic epilepsy ?
a. DOC is sodium valproate
b. mental retardation
c. seizure can develop
d. neurological examination is abnormal
e. life long treatment needed

Click here to read a short notes on Juvenile Myoclonic Epilepsy .

9q: A child presented in the casualty with fever , unconscious and papilloedema . what is the next step/s to be done ?
a. intubation
b. oxygenation
c. CT scan
d. lumbar puncture


10q: A child with jaundice is being given food intravenously . which of the following can occur out of it ?
a. infection
b. malnutrition
c. diarrhea
d. hyperglycemia
e. metabolic acidosis


11q: Most common cardiac tumor in childhood ?
a. rhabdomyoma
b. lymphoma
c. atrial myxoma
d. sarcoma
e. fibroma


12q: loss of pulmonary surfactant in premature infant ?
a. pulmonary edema
b. collapse of alveoli
c. elastic recoil of lungs
d. all the above


13q: unconjugated hyperbilirubinemia is seen in ?
a. physiological jaundice
b. breast milk jaundice
c. gilbert syndrome
d. biliary atresia
e. rotor syndrome

33 - PGI Chandigarh december 2007 mcqs part 1

1q: What is meant by a " suppositious child " ?
a. second born out of a twin pregnancy
b. child born out of wed lock
c. child reared by a woman and she claims the child
d. heterozygous mother and father
e. homozygous mother and father


2q: An adolescent child complains of night pains in the knee . It could be due to ?
a. juvenile rheumatoid arthritis
b. idiopathic growth pain
c. osteosarcoma
d. paget's disease
e. osteomyelitis


3q: Infants of diabetic mother manifests ?
a. hyperglycemia
b. hypoglycemia
c. hypocalcemia
d. increased fetal defects
e. hyperbilirubinemia


4q: 7 day old baby presented in the emergency department with unconsciousness, blue in appearance with 85 % in oxygen saturation . the diagnosis ?
a. tetralogy of fallot
b. TGA
c. TAPVC
d. PDA


5q: Oral glucose tolerance test in children is done with ?
a. 1.5 gm/kg glucose
b. 1.75 gm/kg glucose
c. 2 gm/kg glucose
d. 2.5 gm/kg glucose
e. 75 gms as an adult


6q: A new born of 7 days old presented with meningitis . most common cause ?
a. E.coli
b. streptococcal pneumonia
c. N.meningitides
d. streptococcal agalactiae
e. H.influenzae


7q: Still born child is defined by ?
a. greater than 20 weeks
b. greater than 24 weeks
c. greater than 28 weeks
d. greater than 32 weeks
e. greater than 1300 grams of weight

32 - Idiopathic Thrombocytopenic Purpura ( ITP ) Mcqs part 2

11q: In adolescents with new onset ITP , an antinuclear antibody test should be done to evaluate for ?
a. HIV
b. SLE
c. Kawasaki disease
d. Evan’s syndrome


12q: What is the test to be done in an ITP patient with unexplained anemia to rule out Evan’s syndrome?
a. ELISA
b. Coomb’s test
c. Blood smear
d. Bone marrow aspiration


13q: Differential diagnosis of ITP are ?
a. Exposure to medication that induces drug-dependent antibodies
b. Splenic sequestration due to previously unappreciated portal hypertension
c. Early aplastic processes such as fanconi anemia
d. Amegakaryocytic thrombocytopenia
e. Thrombocytopenia-absent radius ( TAR ) syndrome
f. All the above


14q: What is the disease that should be considered in young males found to have low platelet counts , particularly if there is a history of eczema and recurrent infection ?
a. SLE
b. HIV
c. Wiskott-aldrich syndrome
d. Hemolytic uremic syndrome


15q: All are true regarding the treatment of ITP except ?
a. There is no data showing that treatment affects either short- or long-term clinical outcome of ITP
b. Compared with untreated control subjects , treatment appears to be capable of inducing a more rapid rise in platelet count to the theoretically safe level of greater than 2 lakh/ cubic mm
c. Platelet transfusion in ITP is usually contraindicated unless life threatening bleeding is present
d. There is enough data to show that early therapy prevents intracranial hemorrhage


16q: What is the best treatment for a case of ITP with minimal, mild or moderate symptoms ?
a. No therapy other than education and counselling of the family and patient
b. Intravenous immunoglobulin ( IVIG ) at a dose of 0.8-1.0 g/kg/day for 1-2 days
c. Intravenous anti-D therapy at a dose of 50-75 micrograms/kg
d. Prednisone at a dose of 1-4 mg/kg/24 hr


17q: What is the dose of IVIG given to a patient of ITP that induces a rapid rise in platelet count ( usually greater than 2 lakh/cubic mm ) in 95 % of patients with 48 hrs ?
a. 0.8-1.0 g/kg/day for 1-2 days
b. 1.0-1.5 g/kg/ day for 1-2 days
c. 0.8-1.0 g/kg/day for 4 days
d. 1.0-1.5 g/kg/day for 4 days


18q: The role of splenectomy in ITP should be reserved for which of the following circumstances ?
a. The older child ( greater than or equal to 4 years of age ) with severe ITP that has lasted greater than 1 year ( chronic ITP ) and whose symptoms are not easily controlled with therapy
b. When life threatening hemorrhage ( intracranial hemorrhage ) complicated acute ITP, if the platelet count cannot be corrected rapidly with transfusion of platelets and administration of IVIG and corticosteroids
c. Both of the above
d. none of the above


19q: In ITP , which is the primary site of both antiplatelet antibody synthesis and platelet destruction ?
a. Liver
b. Spleen
c. Gall bladder
d. pancreas


20q: All of the following are true about chronic ITP except ?
a. Approximately 50 % of patients who present with acute ITP have persistent thrombocytopenia for greater than 6 months and are said to have chronic ITP
b. Splenectomy is successful in inducing complete remission in 64-88 % of children with chronic ITP
c. Before splenectomy the child should receive pneumococcal and meningococcal vaccines
d. After splenectomy the child should receive pencillin prophylaxis

31 - Idiopathic Thrombocytopenic Purpura ( ITP ) Mcqs part 1

1q: What is the most common cause of acute onset of thrombocytopenia in an otherwise well child ?
a. SLE
b. Wiskott-Aldrich syndrome
c. HIV
d. ( autoimmune )ITP


2q: All of the following statements are true about Idiopathic Thrombocytopenic Purpura (ITP) except ?
a. A recent history of viral illness is described in 50-65% of cases of childhood ITP
b. Autoantibodies directed against the platelet surface develops
c. EBV related ITP is usually of short duration and HIV associated ITP is usually chronic
d. Splenomegaly is frequently seen


3q: Which of the following is a classical presentation of ITP ?
a. Previously healthy 1-4 year old child who has sudden onset of bleeding from gums and mucous membranes
b. Previously healthy 1-4 year old child who has sudden onset of generalized petechiae and purpura
c. Previously healthy 1-4 year old child presents with splenomegaly
d. Previously healthy 1-4 year old child presents with lymphadenopathy


4q: What is the normal platelet count ?
a. 150 – 450 X 10 power 9 / Litre
b. 150 – 450 X 10 power 3 / cubic mm
c. Both a and b are true
d. None of them


5q: Thrombocytopenia refers to a reduction in platelet count to ?
a. Less than 1.5 lakh/cubic mm
b. Less than 2 lakh / cubic mm
c. Less than 1 lakh / cubic mm
d. Less than 2.5 lakh / cubic mm


6q: In 70-80 % of children who present with acute ITP , spontaneous resolution occurs within ?
a. 3 months
b. 6 months
c. 9 months
d. 1 year


7q: All of the following are true about ITP except ?
a. Therapy does not appear to affect the natural history of the illness
b. Fewer than 1 % of patients have intracranial haemorrhage
c. 80 % of children who present with acute ITP go on to have chronic ITP
d. Severe thrombocytopenia ( platelet count less than 20 x 10 power 9 / litre ) is common


8q: Autoimmune hemolytic anemia and thrombocytopenia is otherwise called ?
a. Tourette’s syndrome
b. Evans syndrome
c. Capgras syndrome
d. None


9q: All are true about acute ITP except ?
a. The hemoglobin value is normal or may be decreased if there have been profuse nosebleeds or menorrhagia
b. WBC count is decreased
c. Differential count is normal
d. Bone marrow examination shows normal granulocytic and erythrocytic series with characteristically normal or increased numbers of megakaryocytes
e. Platelet antibody testing is seldom useful in acute ITP


10q: What are the indications for bone marrow aspiration in ITP ?
a. An abnormal WBC count
b. An abnormal differential count
c. Unexplained anemia
d. Findings suggestive of bone marrow disease on history and physical examination
e. All the above



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