Q-1. A preterm infant with poor respiration at birth starts throwing seizures at 10 hours after birth. Anti-epileptic of choice shall be-
a) Levetiracetam
b) Phenytoin
c) Phenobarbitone
d) Lorazepam
Answer: Phenobarbitone
Explanation:
Phenobarbitone: The drug of choice for neonatal seizures
Important point:
Neonatal seizure may not be effectively controlled with anti-epileptic drugs unless their underlying cause is treated.
Q-2. Which of the following is a true statement about trisomy 13?
a) Bilateral microphthalmia
b) Neurofibroma
c) Rocker bottom feet
d) Dermoid cyst
Answer: Bilateral microphthalmia
Explanation:
Trisomy 13:
In many affected infants and children, such abnormalities may include developmental delays, profound mental retardation, unusually small eyes (microphthalmia), an abnormal groove in the upper lip (cleft lip), incomplete closure of the roof of the mouth (cleft palate), un-descended testes (cryptorchidism) in affected males, and extra (supernumerary) fingers and toes (polydactyly).
Additional malformations of the head and facial (craniofacial) area may also be present, such as a relatively small head (microcephaly) with a sloping forehead; a broad, flat nose; widely set eyes (ocular hypertelorism); vertical skin folds covering the eyes; inner corners (epicanthal folds); scalp defects; and malformed, low-set ears.
Affected infants may also have incomplete development of certain regions of the brain (e.g., the forebrain); kidney (renal) malformations; and structural heart (cardiac) defects at birth (congenital).
Q-3. A child with hypoglycemia is not able to utilize glucose from glycogenolysis or gluconeogenesis which of the following enzyme is deficient in the child
a) Fructo-kinase
b) Glucokinase
c) Glucose 6- phosphatase
d) Trans-ketolase
Answer: Glucose 6- phosphatase
Explanation:
Glycogen storage disease type I is a group of rare inherited diseases resulting from a defect in the glucose-6-phosphatase system that is required for the hydrolysis of glucose-6-phosphate into glucose and inorganic phosphate.
The main diagnostic criteria are:
Hepatomegaly
Fast-induced hypoglycemia
Hyper-lactacidemia
Hyperlipidemia
Important point:
Glucose-6-phosphatase (G6Pase), an enzyme found mainly in the liver and the kidneys, plays the important role of providing glucose during starvation through glycogenolysis or gluconeogenesis.
Q-4. A neurosurgeon dropped his kid to the school then there he saw a child with uncontrollable laughing and precocious puberty. When he again went to the school in parents teachers meeting he talked to the father of that boy and advised him to get an MRI done and the diagnosis was confirmed. What is the most probable diagnosis?
a) Hypothalamic hamartoma
b) Pineal Germinoma
c) Pituitary adenoma
d) Cranio-pharyngioma
Answer: Hypothalamic hamartoma
Explanation:
Hypothalamic hamartomas are rare, tumor-like malformations that occur during fetal development and are present at birth.
They are non-progressive lesions and do not expand, spread or metastasize to other locations.
Clinical features of hypo-thalamic hamartoma:
Gelastic (laughing) seizures
Central precocious puberty
Developmental delay
Cognitive deterioration
Psychiatric symptoms such as rage behaviors
Important point:
MRI is the imaging modality of choice for diagnosis of hamartoma.
Q-5. Which of the following is not a sign of PDA in a preterm baby?
a) Apnea
b) Tachycardia
c) Necrotizing entero-colitis
d) Narrow pulse pressure
Answer: Narrow pulse pressure
Explanation:
Clinical findings of typical PDA (Patent Ductus Arteriosus):
Bounding pulse
Widened pulse pressure
Narrowly spilt S2
Paradoxically splitting of S2 (Rare)
Important point:
Paradoxically splitting of S2 is caused by the volume overload of the LV and prolonged ejection of blood from this chamber.
Q-6. A 7 years old girl with falling grades and complaints by teacher that she is inattentive in class to her parents and has bad school performance. On hyper-ventilation, her symptoms increased and showed the following EEG findings. Diagnosis is (graph is given)
a) Myoclonic epilepsy
b) Myoclonus
c) Absence seizure
d) Juvenile myoclonic epilepsy
Answer: Absence seizure
Explanation:
Absence or petit mal seizure:
Age of onset- 3-15 years
Clinical manifestations:
Lapse of consciousness or vacant stares lasting about 10 seconds
Automatism of face and hand
Clonic activity
Diagnosis:
Hyperventilation provoke attacks
EEG findings: Bilaterally synchronous, high voltage spikes and waves
Q-7. Which of the following in the natural course of disease has no reversal of the shunt?
a) ASD
b) VSD
c) TOF
d) PDA
Answer: TOF
Explanation:
Heart diseases have reversal of the shunt:
ASD
VSD
PDA
Q-8. Correct about respiratory distress syndrome is
a) Seen after 6 hours of birth
b) Ante natal steroid
c) Term pregnancy
d) Air bronchogram seen on X-ray chest
Answer: Air bronchogram seen on X-ray chest
Explanation:
Respiratory distress syndrome, also known as hyaline membrane disease, occurs almost exclusively in premature infants immediate or soon after birth.
The incidence and severity of respiratory distress syndrome are related inversely to the gestational age of the newborn infant.
Chest radiographs in a premature infant with respiratory distress syndrome: Poor lung expansion, air bronchogram, and reticular granular appearance
Antenatal corticosteroid and surfactant therapy reduces the risk of respiratory distress syndrome and neonatal death.
Q-9. A pre-term 32 weeks new-born baby with respiratory rate of 86/min. with presence of grunting. On examination there was on nasal flaring, chest behind in movement than abdomen, minimal intercostals retraction and no xiphi-sternal retraction. The Silverman scoring for the neonate shall be
a) 3
b) 4
c) 5
d) 6
Answer: 4
Explanation:
Q-10. A child presented to the casualty with seizures. On examination an oval hypo- pigmented macules were noted on the trunk, along with sub-normal IQ. Probable diagnosis of the child is
a) Neurofibromatosis
b) Sturge weber
c) Tuberous sclerosis
d) Incontinenta pigmenti
Answer: Tuberous sclerosis
Explanation:
Tuberous sclerosis is a dominantly inherited disease. All most all patients have deletion on chromosome 9 (TSC1) or 16 (TSC2 gene).
The gene products hemartin and tuberin have tumor suppressing effect.
Clinical findings:
Dermatological features:
Hypio-melanotic macules (Oval or ash leaf)
Facial angio-fibroma
Ungual fibromas
Shagreen patches
Neurological features:
Seizures (Most common presenting feature)
Mental retardation:
Renal lesions:
Renal cysts or angio-myo-lipoma
Cardio-pulmonary involvement:
Cystic lung disease
Rhabdomyoma of heart
Diagnosis:
Chest radiograph- Lung honeycombing
CT scan- Pathognomic sub-ependymal nodular calcification
MRI- Hypo-myelinated lesions
Q-11. A child presents with seborrheic dermatitis, sinusitis and chronically draining ears. On examination child has failure to thrive with hepato-splenomegaly and exophthalmos. Probable diagnosis is
a) Histiocytosis-X
b) Wegner’s granulomatosis
c) Chronic granulomatous disease
d) Chediak-Higashi syndrome
Answer: Histiocytosis-X
Explanation:
Clinical findings of Langerhans cell histiocytosis or histiocytosis X:
Bony lesions
Fever
Weight loss
Otitis media
Exophthalmos
Diabetes insipidus
Clinical findings of disseminated Langerhans cell histiocytosis or histiocytosis X:
Seborrheic skin rashes
Lymphadenopathy
Hepato-splenomegaly
Hematological abnormalities
Q-12. In a child, CSF examination is not used in diagnosis of
a) ALL
b) Hodgkin’s lymphoma
c) Non-Hodgkin’s lymphoma
d) AML
Answer: Hodgkin’s lymphoma
Explanation:
CSF examination is required to detect CNS dissemination.
CSF spread in ALL and Non Hodgkin’s lymphoma is well known.
Important point:
Central nervous system (CNS) involvement by Hodgkin lymphoma (HL) is rare.
Q-13. The developmental age of child who knows her full name and gender, can eat without spilling and can dress herself without supervision is
a) 2 years
b) 3 years
c) 4 years
d) 5 years
Answer: 4 years
Explanation:
Milestone and child’s age:
Eat without spilling– 18 months
Knows her full name and gender– 3 years
Dress with supervision– 3 years
Dress without supervision- 4 years
Q-14. Most common organism causing neonatal sepsis
a) Staphylococcus aureus
b) E. coli
c) Haemophilus influenzae
d) Klebsiella
Answer: Klebsiella
Explanation:
Most common organism causing neonatal sepsis: Klebsiella > Staphylococcus aureus > Pseudomonas