10/12/2017
AIIMS PEDIATRICS 2014
1. ASYMETRICAL Moro's reflex is seen in
a. Erb palsy
b. Sternomastoid tumor
c. Hypoxic ischemic encephalopathy
d. Poliomyelitis
Ans: Option A i.e ERB’s palsy ( Page 142 OP ghai. Eight edition. )
Moros reflex is best elicited by sudden dropping of the baby’s head in relation to trunk; the response consists of opening of the hands and extension and abduction of the upper extremities, followed by anterior flexion( embracing) of upper extremities with audible cry. The hand opening is present by 28 wks, extension and abduction by 32 wks an anterior flexion by 37 wks. Moros reflex disappears by 3 – 6 months in normal infants. The most common cause of depressed or absent Moros is a disturbance of the CNS system. An asymmetrical Moros is indicative of root plexus injury.
Moros reflex may be unilateral if there is fracture of clavicle or shoulder dislocation of that particular side.
It is important to know the onset and disappearance of various primitive and postural reflex
Reference Illingworth 10th – the abnormal child page 72 - 86
Reflex Fully developed Duration
Palmar grasp 32 weeks 2-3 months
Rooting 36 weeks Less prominent after 1 month
Moro 37 weeks 5-6 months
Tonic neck (asmy) 1 month 6-7 months
Parachute 10-11 months Remains throughout life
Symmetric tonic neck 6- 8 months months 8-12 months
Crossed extensor 36 weeks 1-2 months
2. A man with skin lesions over face, mentally deteriorating and intracranial calcification. His wife and daughter are normal, His 6 yr old has the same condition?
1)NF-1
2)NF-2
3)Xeroderma Pigmentosum
4)autosomal dominant inheritance
Ans: Option D i.e autosomal dominant inheritance. ( option OP ghai 8th edition page 586).
The important clue in this option is the presence of mental detioration in the index case and inheritance pattern of father to son transmission. Mental detioration is not seen in Neurofibramatosis 1 or NF2. Xero derma pigmentosum has X linked recessive inheritance hence no father to son transmission.
In tuberous sclerosis the index case will have adenoma sebaceum over face, intracranial calcification due to intracranial tuberous which may get calcified and mental detioration. U may think struge weber syndrome which has facial port wine stain and intracranial calcification, but there is no mental retardation in Struge weber syndrome and it has sporadic inheritance.
Comparision of neurocutaneous syndromes
Features Tuberous sclerosis Neurofibromatosis Sturge weber Ataxia telengectasia
Inheritance Autosomal dominant Autosomal dominant Sporadic Autosomal recessive
Age of onset Infancy to adulthood 8 – 15 yrs Infancy 2 yrs – 8yrs
Skin findings ash leaf macules , shagreen patch and adenoma sebaceum Six or more Café au lait macules 5 mm before puberty and 15 mm after puberty. Two or more neurofibromas or one plexiform neurofibroma. Freckling in axillary or inguinal regions Facial nevus flammens or port wine stain, unilateral in distribution of first branch of trigeminal nerve Cutaneous telengectasia seen by 2yrs – 7 yrs over ear lobule, eye lids and extensor surface of elbow
Eye findings Retinal hamartoma and retinal achromic patches Two or more Lisch nodules over iris, optic nerve glioma. Posterior subscapular lenticular opacity in NF 2 Unilateral glaucoma Ocular telengectasia over bulbar conjunctiva
CNS findings Cortical tubers and subependymal giant cell astrocytoma UBO or unindentified bright objects, these are small hamartomas seen in thalamus, basal ganglia and cerebellum. Meningioma or schwanomma, acoustic neuroma in NF 2 Unilateral cortical and sub cortical calcification Cerebellar atrophy
Other system Heart, kidney, lungs, bone and teeth Sphenoid bone, long bone pseudoarthosis None Immune deficieny and lympho reticular malignancy
Mental retardation Yes in a proportion No No No
Epilepsy Myoclonic epilepsy in infancy and multifocal epilepsy No Refractory focal epilepsy
No. may have movement disorder like dystonia and choreoathetosis
Other neurocutaneous syndromes seen in children include Incontinenta pigmenti, hypomelanosis of Ito and PHACE ( posterior fossa anomaly, hemangioma over face, arterial anomaly, cardiac anomaly and eye anomaly)
3. A newborn figure with xray given which is true??
a. Iniencephaly
b. anencephaly
c. schizencephaly
d. cranioraschisis
Ans: option A. i.e Iniencephaly ( reference page no 137 Swaiman Pediatric neurology). The image had shown fetus with visceral anomaly hence answer is option A
In anencephaly, the absence of the brain and calvaria can be total or partial. Acrania is defined as congenital partial or total absence of the skull.
Craniorachischisis is characterized by anencephaly, accompanied by a contiguous bony defect of the spine and exposure of neural tissue. Craniorachischisis is typically, an extremely thin and flattened spinal cord, accompanied by foreshortening of the neuraxis and is thought to result from impaired convergent extension during embryogenesis.
In iniencephaly, dysraphism in the occipital region is accompanied by severe retroflexion of the neck and trunk, with three cardinal features: deficiency of the occipital bone, cervicothoracic spinal retroflexion, and rachischisis. Iniencephaly differs from anencephaly in that the cranial cavity is present and skin covers the head and retroflexed region. Severe retroflexion of the neck present on fetal ultrasound examination may suggest the diagnosis. A majority of the patients also have visceral and other severe CNSmalformations.
Another related condition ismeroanencephaly. Meroanencephaly is a rare form of anencephaly characterized by malformed cranial bones and a median cranial defect, through which protrudes the area cerebrovasculosa. In encephalocele, the brain and meninges herniate through a defect in the calvaria
4. A child has albinism. What needs to be evaluated next?
a. ENT
b. ECG
c. ECHO
d. Eye
Ans: Option D i.e Evaluation of the eyes. ( reference page no 535 Nelson pediatrics)
Albinism is due to bio defects in the synthesis of melanin. Types include Oculocutaneous albinism, Ocular albinism and localized albinism. Melanin is also present in cochlea. In waardenberg syndrome which is atype of localized albinism there is sensory neural deafness along with white fore lock of hair.
Eye findings include – translucent iris, misrouting of optic fibres at the chiasm hence absence of stereoscopic vision, decreased visual acuity and strabismus.
5. Olympian brow & raghades
a. Cong syphilis
b. Hyper IgE
c. CMV infection
d. Gauchers disease
Ans: option A. i.e congenital syphilis. ( reference Harrison 17 edition p 1042, 1043)
• Transmission of T. pallidum across the placenta occur at any stage of pregnancy.
• Congential syphilis have their onset usually after fourth month of gestation.
• Adequate treatment of mother before < 16 wks prevents fetal damage
• Maternal syphilis can cause - fetal loss, still birth, prematurity and nonfatal congential syphilis
• Congenital syphilis – has early and late manifestations.
• Residual stigmata of late syphilis is seen in later child hood after > 2 yrs of age
Early congenital syphilis Late congenital syphilis Residual stigmata
Usually at 1m – 4 m age Usually after 2 yrs age Late child hood and adult
Earliest sign – persistent rhinitis or snuffles Interstial keratitis Hutchinson teeth – centrally notched peg shaped upper incisor widely placed
Bone changes – osteochondritis, periosteitis and osteitis leads to pseudo paralysis due to pain Eight nerve deafness Mulberry molars
Mucocutaneous lesions Recurrent arthropathy Frontal bossing with saddle nose, known as Olympian brow
Hepato splenomegaly , jaundice and lymphadenopathy Clutton joints – bilateral knee effusion Rhagades – linear scars at angle of mouth and nose
Anemia, thrombocytopenia and leucocytosis Gummatous periostitis Saber shin – anterior tibial bowing
6. Hypoglycemia not seen in-?
a. appropriate gestational age
b. Small for gestational age
c. Large for gestational age
d. Post dated babies
Ans: Option A. Appropriate for gestational age. ( reference OP ghai page 179). Hyoplycemia is seen in children with,
a. Inadequate substrate – Preterm, SGA, LBW
b. Relative hyperinsulinemia - IDM, LGA and Rh iso immunization
c. Sickness - Birth asphyxia, hypothermia and sepsis
Screeing for these high risk babies are done at 2 hr, 6 hr , 12 hr, 24 hr, 48 hr and 72 hour after birth.
…… AGA babies who are breast feeding adequately do not require screening for hypoglycemia.
Even though post dated babies usually do not have hypoglycemia, the best answer for this question would be option A. i.e Appropriate for gestation age babies
7. Enzyme replacement therapy is for
a. Gauchers ds
b. Nieman pick disease
c. Tay sachs disease
d. Krabbes disease
Ans: Option A . i.e Gaucher disease ( reference OP Ghai 8 edition – 662 )
First storage disorder for which ERT was started . Other disease which have ERT include,
8. Child presenting with 6 days fever. . cervical lymphadenopathy, rash, strawberry tongue
a. kawasaki disease
b. Scarlet fever
c. Measles
d. ITP
Ans: Option A . i.e Kawasaki disease. ( reference OP ghai page no – 631)
Kawasaki disease is an acute febrile mucocutaneous lymph node syndrome affecting infants and young children. More than 80 % cases are seen in children < 5 yrs age. Up to 15 – 25 % of untreated children will have acquired coronary artery vasculitis in the form of aneurysm and may have sudden cardiac death.
The diagnostic criteria for Kawasaki include,
Fever for 5 days plus any 4 of the 5 conditions
1. Bilateral non purulent conjunctival injection
2. Changes in mucosa of oropharynx – strawberry tongue and fissured lips
3. Changes in peripheral extremities – edema and erythema of hands, periungual desquamation
4. Polymorphous rash – never vesicular
5. Unilateral cervical lymphadenopathy > 1. 5 cm
Features Kawasaki Scarlet fever Measles
Age of onset Less than 5 yrs 2 yr – 8 yrs Children of any age
Fever Persistent Variable Before rash for 3 – 4 days. Also coryza and cough present
Eyes Bilateral non purulent conjunctival injection
Normal Bilateral conjunctivitis
Rash Polymorphous rash – never vesicular Papular erythrdoderma, Pastia lines and circum oral pallor. Maculopapular begins on face and spreads downwards over 1 – 2 days.
Oropharynx strawberry tongue and fissured lips Strawberry tongue and pharyngitis Koplik spots on day 2 or day 3 opposite lower molar.
Peripheral extremities edema and erythema of hands, periungual desquamation Flaky desquamation None
Cervical Lymphnodes Unilateral, non purulent, non painful Bilateral painful May have Bilateral painful lymphadenitis
9. Child has decreased ca+, increased phosphate, decreased urinary excretion of ca+&phosphorous, and LD alkaline phosphatase increased. the dx is
1 . Glomerular rickets
2. Nutritional rickets
3. Renal tubular rickets
4. Hypophosphatemic rickets
Answers: Option A. i.e Glomerular rickets ( reference – OP Ghai page114 and 495 )
The first step in evaluation of rickets is to look at serum phosphate. Except for CKD with renal osteodystrophy in all other rickets the serum phosphate is low.
In all cases of rickets serum paratharmone is high due to secondary or tertiary hyperparathyroidism except for hypophosphatemic rickets where serum PTH is normal and serum calcium is normal.
In renal tubular acidosis type II there is phosphaturia, glucosuria and aminoaciduria( Fanconi syndrome) hence there is low serum phosphate
10. A baby with suspected neonatal enterocolitis has portal vein gas on x ray. Appropriate stage is ?
a. Stage III.
b. Stage II a
c. Stage II b
d. Stage IV
Ans: Option C ( reference OP ghai page no 139 7 th edition and cloherty seventh edition page no – 343)
Modified bell staging for NEC
Stage Classification Systemic features Abdominal features Radiological features Treatment
IA Suspect NEC Temperature instability, apnea, bradycardia, lethargy Mild abd distension Mild intestinal dilation NPO / Antibiotic
I B Suspect NEC Same as I A Fresh blood per re**um Same As I A NPO / Antibiotic
II A Proven NEC Same as I A + absent bowel sounds.
+ or- abdominal tenderness Intestinal dilation and Pneumatosis intestinalis NPO / Antibiotic for 7- 10 days
II B Proven NEC Same as I A + mild metabolic acidosis + mild thrombocytopenia ++ defntive abdominal tenderness, abdominal cellulitis and right lower quadrant mass ++Portal veins gas NPO / Antibiotic for 10 – 14 days
IIIA Advanced NEC, severely ill, bowel intact IIB + respiratory acidosis, DIC, Shock, neutropenia, severe apnea ++ features of peritonitis, marked abdominal distension and marked tenderness +++ Ascites NPO / Antibiotic for 14 days with resuscitation , ventilation and inotropic support
IIIB Advanced NEC, severely ill, bowel perforated Same as III A Same As III A +++ Pneumoperitoneum Same as IIIA along with surgery
11. urine sample of newborn with hepatosplenomegaly stained with geimsa stain
1)hiv
2)cmv
3)rubella
4) toxoplasmosis
Ans Option D ( page no 626 cloherty manual of neonatal care and Wallachs intepretaion of diagnostic tests page no 1032).
Viruses like rubella and CMV can only be isolated from urine via viral culture using special media. Only protozoa like the trophozoite of toxoplasmosis can be stained and visualized using giemsa stain.
12. A girl with graying of hair , same manifestation in school , few others in village , not in neighbouring village , or siblings in other residing states does not have graying of hair?
a. environmental
b. nutritional
c. genetic
d. Infection
Ans: Option A. ( ref Emery essentials of medical genetics page 86)
The graying of hair of the index case is also present in few other people in her village. Nutritional causes usually affect a particular age group either children or adults not both. Hence environmental cause is more likely.
13. Management of medulloblastoma in 1.5yo child. what is not advised
a. open surgery with excision of the lesion
b. CT
c. RT
d. VP shunt for the obstructive hydrocephalus
Ans: Option B ( reference page no 1360 Swaiman pediatric neurology)
In non-disseminated patients, who comprise 70–80 percentof all patients with medulloblastoma, multiple studies have demonstrated that the extent of surgical resection is prognostic of outcome, with those patients undergoing more complete or near-total resections having better outcome than patients whose tumors were subtotally resected . This has been shown both in infants and in older children, but has not been clearly demonstrated in children with disseminated disease.
For infants and young children with medulloblastoma, treatment remains quite problematic. Due to concerns over the detrimental long-term effects of craniospinal radiation therapy, chemotherapeutic approaches have been used to delay, if not completely obviate, the need for radiotherapy . With a variety of different regimens, including mechlorethamine, nitrogen mustard, procarbazine, and prednisone (MOPP), and cisplatin, cyclophosphamide, vincristine, and etoposide , approximately 20 percent of infants can be treated with chemotherapy alone .
14. Exudative retinitis in one eye in a 7 year boy cause?
a. COATS disease
b. Retinopathy of prematurity
c. Familial exudative vitreo retinopathy
d. Diabetic retinopathy
Ans: Option A. COATS disease. ( reference page 2603 Nelson pediatrics)
For a 7yr old with unilateral exudative retinopathy the cause is COATS disease.
Coats disease - affects boys, first decade, unkown cause. Unilateral Telengectasia of retinal vessles with leakage to form sub retinal and intra retinal exudates. Condition is non familial. It is associated or some times seen in children with Fascio scapular humeral dystrophy. Treatment – photocoagulation or cryotherapy
Familial exudative vitreoretinopathy: Bilateral, autosomal dominant with incomplete penetrance. Avascular of peripheral retina is significant finding.
ROP - disease of the developing retinal vasculature usually seen in preterm babies less than 34 weeks. Clinical manifestation range from transient peripheral retinal changes to progressive vasoproliferation, scarring and retinal detachment.
Diabetic retinopathy – can be proliferative or non proliferative. It is rare in prepubertal children and prevalence increase after 15 yrs age
15. ROP when screening for 28WOG born with treatment in NICU and sepsis after 2 weeks
a. 2 weeks
b. 4 weeks
c. 6 weeks
d. 8 weeks
Ans : Option A. ( reference page no 841 cloherty manual of neonatology. And AIIMS neonatology protocol page no 174.)
The timing of the ROP is related to the post natal age of the baby. More preterm the baby more chance of immature retinal vessels and hence ROP. The current recommendation is to screen all babies less than 30 weeks gestation and less than 1500 gms weight.
The first screen of preterm babies born > 26 wks should be carried out 31 weeks or 4 weeks later whichever is later. For example if the baby is born at 30 wks gestation screening is done at 34 wks. ( not 31 wks). So for this baby born at 28 wks, screening should be done at 32 weeks of gestation. Since the baby had already had sepsis for 2 weeks, this means the baby was 30 weeks gestation at discharge and hence he should be screened at 32 weeks i.e 2 weeks later. Hence answer is option A
16. staging of HIE - with lethargy, hypotonia after 12hrs of birth with resucitation and 2 episodes of seizures
a. stage 2
b. Stage 1
c. Stage 3
d. No HIE
Ans: Option A i.e stage 2 ( reference op ghai 8th edition page 167 and cloherty neonatology page 716)
The question demands knowledge of staging of birth asphyxia. Two different staging are commonly used of which the simpler one is Levene staging given in Ghai pediatrics. Other standard staging is sarnat and sarnat stages given in cloherty manual
Feature Mild HIE Moderate HIE Severe HIE
Consciousness Irritability Lethargy Comatose
Tone Hypotonia Hypotonia Severe hypotonia
Seizures No Yes Prolonged
Sucking/ respiration Poor suck Unable to suck Unable to sustain respiration
Staging system of HIE –sarnat and sarnat staging
Stage I – Hyper alert + strong moro + mydriasis
Stage II – Lethargic + moro weak + miosis + seizures
Stage III – Stuporous, coma + absent moro + unequal pupils, poor light reflex + decerebrate posturing
17. A 6 weeks old boy with fever and cough for 3 days. On ex rhonchi present. RR – 48. No chest indrawing. false statement- child has pneumonia
a. Treat for wheeze
b. Antibiotics should not be given
c. Child has pneumonia
d. Fever should be treated
Ans: Option C . i.e Child has pneumonia. ( reference OP Ghai – 8th edition page no
For a 7 weeks old infant RR of 48 is not fast breathing also there is no chest indrawing hence there is no pneumonia according to ARTI classification. Most of community acquired pneumonia in infants 0m – 12 months are viral pneumonia which require no antibiotics but only symptomatic treatment for wheeze.
Acute respiratory tract infection control program
Signs/ symptoms Classification Therapy
Cough or cold with no fast breathing or chest indrawing No pneumonia Home remedy
Respiratory rate
a. < 2 months - > 60/ min
b. 2m – 12 m -- > 50 min Pneumonia Oral cotrimoxazole or Amoxy
c. 12m – 60 m -- > 40 / min
Chest indrawing present Severe pneumonia IV/ IM pencillin
Cyanosis, inability to feed, severe chest indrawing Very Severe pneumonia IV pencillin + Gentamycin
Parameters to classify as very severe disease:
1. Not able to drink
2. Convulsions, abnormally sleepy or difficult to awake
3. Stridor in calm child
4. Severe malnutrition
A child with fast breathing and no chest indrawing would be classified as PNEUMONIA.
Child without chest indrawing or fast breathing – NO PNEUMONIA.
