Pediatrics: AIIMS NOV 2013

Q-1. Neonatal sepsis is caused by all except:
a) Staphylococcus aureus
b) E. coli
c) Group B streptococci
d) Acinetobactor

Answer: Acinetobactor
Explanation:
Early onset of sepsis (< 5 days):
Early onset of sepsis appears most commonly on day 1 of the life.
Respiratory distress due to pneumonia is the most common presenting sign.
Early onset of sepsis is most often caused by group B beta-hemolytic streptococci, E. coli, Haemophilus influenzae, and Listeria monocytogenes.
Late onset of sepsis (> 5 days):
Late onset of sepsis presents in more subtle manner with poor feeding, lethargy, hypotonia, temperature instability and apnea.
Late onset of sepsis is more often associated with meningitis or other localized infections.
Late onset of sepsis is caused by coagulase negative Staphylococci, Staph aureus, Enterococcus, Pseudomonas and other gram negative organisms.

Q-2. An 8 year old boy complains of muscle weakness. On examination his calves are bulky and show muscle tightening. His serum creatine kinase levels are increasing with age. Which of the following is the most likely diagnosis?
a) Hereditary sensori-motor neuropathy
b) Myelin deficiency
c) Dystrophin deficiency
d) Congenital myopathy

Answer: Dystrophin deficiency
Explanation:
Duchenne muscular dystrophy:
Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy.
Mutations in the human dystrophin gene at locus Xp21 cause Duchenne muscular dystrophy.
Dystrophin is responsible for connecting the cytoskeleton of each muscle fiber to the underlying basal lamina (extracellular matrix) through a protein complex containing many subunits.
Dystrophin is distributed not only in skeletal muscle but also in smooth and cardiac muscles and in the brain.
Duchenne muscular dystrophy is characterized by pseudo-hypertrophy of the calf muscle
and elevated levels of serum creatine kinase.
Important point:
After calf, tongue and then forearm muscles are the most common muscle to hypertrophy.

Q-3. A 2 days old premature neonate develops GTCS what is the investigation done to diagnose the pathology
a) Trans-cranial ultrasound
b) CT head
c) MRI brain
d) X-ray

Answer: Trans-cranial ultrasound
Explanation:
Investigations that should be considered in all neonates with seizures include blood sugar, hematocrit, bilirubin, serum electrolytes, arterial blood gas, anion gap, cerebrospinal fluid (CSF) examination, cranial ultrasound (US) and electroencephalography (EEG).
Neuro-sonography should be done in all infants with seizures.
Neuro-sonography is an excellent tool for detection of intra-ventricular and parenchymal hemorrhage but is unable to detect SAH and sub-dural hemorrhage.
A CT and MRI scan should be done in all infants where an etiology is not available after the first line of investigations.

Q-4. A pediatrician in a district hospital specialized neonatal care unit calls an ophthalmologist for consultation for which of the following?
a) A newborn with respiratory distress
b) A baby born at 28 weeks of gestation
c) Newborn with jaundice
d) A newborn with birth weight 2300 grams

Answer: A baby born at 28 weeks of gestation
Explanation:
Retinopathy of prematurity (ROP) is a disorder of the developing retina of low birth weight preterm infants that potentially leads to blindness in a small but significant percentage of those infants.
In almost all term infants, the retina and retinal vasculature is fully developed, and ROP cannot occur; however, in preterm infants, the development of the retina, which proceeds from the optic nerve head anteriorly during the course of gestation, is incomplete.
Retinal examinations in preterm infants should be performed by an ophthalmologist who has sufficient knowledge and experience to identify accurately the location and sequential retinal changes of ROP.
Important point:
Approximate 65 % of infants with birth weight of < 1250 gm will develop some degree of ROP.

Q-5. An Afro-American kid of 6 years of age presented with abdominal pain, chronic hemolysis, elevated serum bilirubin and abnormal RBC shape on peripheral smear. Most likely disorder responsible for this condition
a) Tri-nucleotide repeats
b) Point mutation
c) Antibodies against RBC membrane
d) Genetic imprinting

Answer: Point mutation
Explanation:
Sickle cell anemia:
Sickle-cell anemia is caused by a point mutation in the β-globin chain of hemoglobin, causing the hydrophilic amino acid glutamic acid to be replaced with the hydrophobic amino acid valine at the sixth position.
Autosomal recessive disorder and caused by homo-zygosity for sickle gene.
Clinical findings:
Hemolytic anemia
Tissue ischemia
Organ dys-function due to vaso-occlusion
Splenomegaly (Acute splenic sequestration- Characterized by sudden enlargement of the spleen with pooling of red cells, acute exacerbation of anemia and severe cases, shock and death)
Dactylitis or hand and foot syndrome
Recurrent episode of Musculo-skeletal or abdominal pain
Important points:
Neonatal screening: Hemoglobin electrophoresis
A high prevalence of sickle hemoglobin is found in person of central African origin.

Q-6. Which of the following is not given in the treatment of cyanotic spells in a patient of TOF?
a) Phenylephrine
b) Propanolol
c) Calcium chloride
d) Sodium bicarbonate

Answer: Calcium chloride
Explanation:
Treatment of cyanotic spells in a patient of TOF:
Oxygen
NS or RL
Subcutaneous morphine
Sodium bicarbonate
Phenylephrine and propranolol
Important point:
Dehydration precipitates spell in ToF

Q-7. A newborn has frothing of mouth. Cyanosis is present on day one. The most probable diagnosis is?
a) Lung hypoplasia
b) Lung cyst
c) Diaphragmatic hernia
d) Esophageal atresia

Answer: Esophageal atresia
Explanation:
Esophageal atresia:
Most of affected infants, the fistula is between the distal esophagus and the airway (Tracheo-esophageal fistula).
Poly-hydramnios is common.
Infants present in the first hours of life with copious secretion, choking, cyanosis and respiratory distress.
Diagnosis is can be confirmed with chest radiography after careful placement of a naso-gastric tube.

Q-8. A 6 weeks old baby presents with cough and cold for the last three days. Respiratory rate is 48/min. Patient is febrile. There are no chest retractions but wheezing is present. Which of the following statement is not true?
a) Antibiotics are not required
b) Child is suffering from pneumonia
c) Treat only wheezing
d) Treat only fever

Answer: Child is suffering from pneumonia
Explanation:
Old WHO classification of pneumonia
No pneumonia: Cough and cold
Pneumonia: Fast breathing
Severe pneumonia: Chest indrawing
Very severe pneumonia: Any general danger sign
Revised WHO classification and treatment of childhood pneumonia:
The new classification is therefore simplified to include only two categories of pneumonia; “pneumonia” with fast breathing and/or chest indrawing, which requires home therapy with oral amoxicillin, and “severe pneumonia”, pneumonia with any general danger sign, which requires referral and injectable therapy.
Important point:
WHO respiratory rate thresholds for identifying children with pneumonia:
Children younger than 2 months: Greater than or equal to 60 breaths/ min
Children aged 2-11 months: Greater than or equal to 50 breaths/ min
Children aged 12-59 months: Greater than or equal to 40 breaths/ min

Q-9. Efficacy of phototherapy is not affected by
a) Skin pigmentation
b) Type of light used
c) Spectral irradiation by incident light
d) Initial concentration of bilirubin

Answer: Skin pigmentation
Explanation:
Factors affecting the dose and efficacy of phototherapy:
Spectrum of light emitted
Spectral irradiation by incident light
Spectral power or average spectral irradiance across surface
Initial concentration of bilirubin
Important points:
Blue green spectrum is most effective.
Failure of phototherapy suggests hemolysis or cholestasis.

Q-10. To establish the diagnosis of H-type trachea- esophageal fistula, which if the following is required?
a) Chest X-ray
b) Tracheo- bronchoscopy
c) CT scan
d) Esophagoscopy

Answer: Tracheo- bronchoscopy
Explanation:
H-type trachea- esophageal fistula:
Diagnosis is confirmed with esophagogram or video‐esophagogram.
If there is a doubt, bronchoscopy and simultaneous administration of methylene blue should be the next diagnostic step.

Q-11. A 3 days baby is admitted with intra-ventricular hemorrhage. Baby develops abdominal distention. The X-ray abdomen showed pneumatosis portalis. Stage the necrotizing entero-colitis
a) 1B
b) 2a
c) 2b
d) 3a

Answer: 2b
Explanation:
Necrotizing enterocolitis staging: Radiological Finding
Stage-I: Intestinal dilation
Stage IIa: Pneumatosis intestinalis
Stage IIb: Portal venous gas (Pneumatosis portalis)
Stage-IIIa: Ascites
Stage-IIIb: Pneumo-peritoneum or signs of perforation

Q-12. A 4 years old girl presented with abdominal lump. Bone scan is needed in
a) Wilm’s tumor
b) Neuroblastoma
c) Rhabdomyosarcoma
d) PNET

Answer: Neuroblastoma
Explanation:
Neuroblastoma:
Neuroblastoma arises from neural crest tissue of the sympathetic ganglia or adrenal medulla.
It is composed of small, fairly uniform cells with little cytoplasm and hyper-chromatic nuclei that may form rosette patterns.
Neuroblastoma is most common solid tumor outside the CNS.
The most common sites of metastases are, bone, lymph nodes, liver, and sub-cutaneous tissue.
Bone scan are obtained for detecting bone and bone marrow metastasis.
Important points:
“Small, round blue cell” malignancies in childhood: Neuroblastoma, Ewing sarcoma, Rhabdomyosarcoma, Lymphoma and Peripheral neuro-ectodermal tumor