Q. Imp fatty acid prsnt in brst milk which is imp for growth-1. dha2. palmitic acid 3. linoleic acid 4. linolenic acid

Discussion- linolenic acid .... linolenic is most imp....though dha (Docosa Hexanoic Acid ) prsnt only in brst milk but linolenic most imp.. from linolenic ..dha can be derived ......

MK-I P=330 Q-238

Age Agent

< 2 mth Gp B & D steptococciG- enteric bacilliListeria monocytogenes

2mth - 12 yrs S.pneumoniaeN. meningitidisH. influenzae type b

Early onset sepsis in INDIA E.ColiEarly onset sepsis in whole world Gp B streptococciLate onset/nosocomial Klebsiella

** Neonatal sepsis.... As in meningitis, Grp B str. (Str. agalactiae) is mc cause of neonatal sepsis worldwide.... In India, E. coli is m.c cause....

Most common cause of Nosocomial Neontal sepsis in India is.... Klebsiella...... M.C cause of Nosocomial neonatal sepsis worldwide is E. coli.... (See Q-134, AA-I P-305)

* Dear doc. There are 2 M.c. sequelaes of neonatal sepsis.... Neonatal meningitis and pneumonia. These are often taken as a spectrum in a neonate. That's why the Microbiology of sepsis and meningitis is same.

** Prematurity/LBW and Diabetes in mother are M.c causes of neonatal hypoglycemia. Former shd be the M.c cause overall.

** M. c cause of pneumonia in pediatric age group is Str. pneumoniae.

** ETEC(bacterial) and Rotavirus(viral) are Mc cause of diarrhea in children in India.

** Mc cause of meningits in infants (3mth - 2 yrs.) .... Meningococcus(worldwide),, H. influenza (India).........

*** For VAP(Ventilator associated pneumonia): - There are 2 types: - a) Early-onset VAP - Occurs in first 4 days of hospital stay. Microbiology of this is basically patients own flora. M.C organism is Streptococcus pneumonia.

b) Late-onset VAP: - Occurs at 5 or more days of hospital stay. M.c agent here is Pseudomonas. MRSA is 2nd M.C..

** Mc cause of neontal seizures is hypoxic-ischemic encephalopathy

** M.c cause of Infant mortality, neontal mortality and early neonatal mortality is Low birth weight.

** "Perinatal deaths are associated with intrauterine growth restriction (IUGR); conditions that predispose the fetus to asphyxia, such as placental insufficiency; severe congenital malformations; and overwhelming early-onset neonatal infections" - Nelson's 18th Ed.

• Children 2 yrs of age and older, at high risk for acquiring systemic pneumococcal infections, or with increased risk of serious disease if they become infected, should be vaccinated for pneumococcal vaccine. The high risk category includes sickle cell disease, functional asplenia, *nephrotic syndrome, or CRF, immunosupression and infection wiht the HIV and CSF leaks.

Q. (GT-88, Q 146) Starting Mid-may & lasting through June, a 6 yr old boy c/o Itchy eyes, watery nose, sneezing. SAFEST & MOST PRACTICAL approach to t/t:(1) Antihistaminics---------------------------------ans(2) Avoidance of the offending allergen(3) Desensitization therapy(4) Topical steroidsDiscussion- Well the question clearly asks us "SAFEST & MOST PRACTICAL" method. Which here clearly is ANTIHISTAMINCS. I completely agree with all of you that Best t/t would be (2)Avoidance of offending allergen, BUT, SAFEST & MOST PRACTICAL - Antihistaminics.

• ALTE(Apparent Life Threatening Event): Episode of apnoea associated with marked change in color and muscle tone such that an observer typically believes that the infant will die without vigourous stimulation or resuscitation

• Werdnig-Hoffman disease(also known as "Severe infantile spinal muscular atrophy", or "spinal muscular atrophy type I") is an autosomal recessive neuromuscular disease. It is the most severe form of spinal muscular atrophy, which is one of a number of neuromuscular diseases classified as a type of muscular dystroph. Infants assume a flaccid ''frog-leg'' posture. Werdnig-Hoffman affects the lower motor neurons only. Most die by 2yrs of age.◦ Symptoms-It is evident before birth or within the first few months of life. There may be a

reduction in fetal movement in the final months of pregnancy. Symptoms include floppiness of the limbs and trunk, feeble movements of the arms and legs, swallowing and feeding difficulties, and impaired breathing. Affected children never sit or stand unassisted and will require respiratory support to survive before the age of 2. Other symptoms include:▪ Fasciculations * of the tongue▪ Marked Hypotonia in Proximal, Distal muscles, Intercostals & bulbar muscles (Patient lies

in a Frog-Leg position,i.e. hips adducted & knee flexed)▪ Flaccid Quadriplegia▪ Difficulty breathing▪ Poor feeding- need gastrostomy▪ Weak cry▪ Areflexia in the extremities▪ Normal Intelligence

• The most common cause of blood in stool of a newborn is swallowed maternal blood. The apt test

distinguishes adult hemoglobin from from fetal Hb. If adult Hb is detected blood is swallowed maternal blood.

• PEM results in anergy(impaired cellular immunity) and a drop in the helper T-cell count result in problems with a cutaneous response to subcutaneous injection of common antigens). Also all phases of phagocytic function are impaired. TLC is decreased. In the negative nitrogen balance that occurs, endogenous amino acids derive from the breakdown of muscle.

Q- A 7-yr old boy with leukaemia in remission develops a fever, vesicular rash, and cough. Tzank test is positive, and a CXR shows a small left lower lobe infiltrate. The boys mother is uncertain whether he has had chickenpox. What is the most appropriate initial course of action-

1. Administer herpes zoster immune-globulin2. Administer antibiotic based on gram stain from bronchoscopic washing3. Administer intravenous acyclovir and ceftriaxone------ans4. Admit the patient to hospitals and observe closely

Explanations-Susceptible immuno-compromised children(eg. those with leukaemia) should receive VZ immune globulin with-in 48 hrs of exposure for maximal effectiveness and be closely observed. Those who develop signs of disseminated varicella(as in this case) or herpes zoster should be treated with acyclovir or vidarabine. Because there is evidence of possible bacterial pneumonia, antibiotic therapy also should be provided for this patient

Q- A 2-yr old girl presents with poor weight gain and slow growth. She has episodes of vomiting and dehydration. Evaluation reveals hyperchloremic metabolic acidosis, normal creatinine and a persistently alkaline urine. Therapy indicated is-

1. High-caloric nutritional supplements2. Institution of anti esophageal reflux precautions3. Alkalinizing agents sufficient to normalise the serum CO2----------ans4. Pancreatic enzymes for probable cystic fibrosis

Explanation- The child probably has RTA, and bicarbonate therapy should restore good clinical health and normal growth. If the acidosis is corrected the extra caloric supplementation will probably not be required.

Q. Most common cause of infant mortality-a) respiratory diseases b) diarrhoeal diseases c) infectious diseases ----------ansd) none of the above

** M.c causes of Infant mortality in India are.. Low birth weight, Respiratory INFECTIONS, Diarrheal diseases (mostly infectious), congenital malformation, Cord INFECTION..... in that order.... Now have a close look at options and the list of causes. ARI(acute resp. INFECTIONS) and diarrhea(mostly INFECTIOUS causes in Indian Infants) forms most common INFECTIOUS causes of infant mortaility in India.

If i had been examiner at JIPMER , i'd had added 4th option as "Low birth weight"..... After LBW, Infectious causes are most common....

*** HSP and IgA nephropathy have same clinical features, except that HSP also have component of Vasculitis. Simply saying: - Ig A nephropathy + Vasculitis = HSP.

** Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by prominent tissue deposition of IgA-containing immune complexes, especially in the skin and kidney. It has a prominent cutaneous involvement similarly seen in mixed cryoglobulinemia and hypersensitivity vasculitis: palpable petechiae or purpura is a major finding in these disorders. The pathogenesis is similar to that of IgA nephropathy, with similar histologic findings in the kidney.

• GYNECOMASTIA: ◦ Genetic Gender disorder (Klinefelter)◦ Young boy (pubertal)*◦ Neonate*◦ Estrogen ◦ Cirrhosis/ Cimetidine/ Ca Channel blockers ◦ Old age* ◦ Marijuana ◦ Alcoholism ◦ Spironolactone ◦ Tumors (Testicular & adrenal) ◦ Isoniazid/ Inhibition of testosterone ◦ Antineoplastics (Alkylating Agents)/ Antifungal(ketoconazole)

* Asterisk indicates physiologic cause.

Necrotising enterocolitis- o Common in pre-term infants exposed to stress in first week of life. Common in infants with

Asphyxia, RDS, Fetal distress, Hyperbilirubinemia, Exchange transfusion and Formula feedso C/F- Abdominal distension, Apnea, Bradycardia, temperature instability, cyanosis and lethargyo Breast milk is protectiveo Pneumatosis intestinalis seen. In late stages perforation and pneumoperitonium occurs.

Fanconi anemia FA is characterized by short stature, skeletal anomalies, increased incidence of solid tumors and leukemias, bone marrow failure (aplastic anemia), and cellular sensitivity to DNA cross-linking agents such as mitomycin C .

Fanconi syndrome disease of the proximal tubuleof the kidney in which glucose, amino acids , uric acid, phosphate and bicarbonate is passed into the urine, instead of being reabsorbed.

Noonan syndrome- Autosomal dominant disorder in both sexes characterised by webbed neck, short stature, congenital heart disease, cubitus valgus and other congenital defects despite normal karyotype and normal gonads

Astrocytomas are most common type of glial tumors. Astrocytomas are common in the cerebellar hemisphere. o Cerebellar astrocytomas- There is ataxia and incoordination more on the side of lesion. Nystagmus

is observed on lateral gaze of the child to the affected side. Areflexia and hypotonia are present. The head is tilted to the side of lesion to relieve the increased intracranial pressure caused by herniation of tumor or cerebellar tonsils through forman magnum. Complete surgical excison is often feasible.

o Low grade astrocytomas are more common in children than adults. Pilocytic astrocytoma named for its characteristic spindle-shaped cells, is the most common childhood brain tumor. It frequently occurs in cerebellum. Complete surgical excison usually produces long term disease free survival.

o Over 2/3 of brain tumors of children are infratentorial containing medulloblastomas and astrocytomas of cerebellum; brain stem glioma and ependymomas account for the rest. Most of these tumors occur near the midline obstructing CSF circulation and causing hydrocephalous early in the disease.

Hysparrythmia- is a very chaotic and disorganised brain wave behaviour with no recognisable pattern. Seen in infant diagnosed with infantile spasms(salaam seizure)..Ghai=P-511

Q- A 9 month old child presented with h/o cough, respiratory distress and stridor which decreases on lying down. What is the most likely diagnosis-

1. Laryngo-tracheobronchitis2. Retropharyngeal abscess----ans(GT-77)3. Acute epiglottitis4. Foreign body

EXPLANATION- Retropharyngeal abscess presents with cough, drooling of saliva, respiratory abscess, stridor and a bulge in the lateral side of the neck. The stridor decreases on lying down owing to the presence of abscess behind the pharyngeal wall which gives way for air to enter or escape in lying down position.

See question is different from AIPGE one MK-I P=329• Laryngotracheobronchitis-

◦ M/C cause-->parainfluenzae◦ Rx: Dexamethasone

• Epiglottitis-◦ M/C -->

▪ India-->H. Influenzae b▪ Overall-->Str. pyogenes(d/t vaccinations)

Enuresis-o Diagnosis is made when urine is voided twice a week for at least 3 consecutive monthso Marked familial patterno Prevalence at 5 yrs is 7% for males and 3% for females

Cyanotic heart disease: All Congenital heart diseases starting with Letter "T" are cyanotic, e.g., Tetralogy of Fallot (TOF), Transposition of great vessels (TGA), Tricuspid atresia, Truncus arteriosus and TAPVC.

- Cyanotic heart disease suggest Right to Left shunt, indicating higher pressure in Right ventricle compared to Left. - All forms of cyanotic congenital heart disease are associated with elevated pressures in the right ventricle. Therefore, the ECG shows right axis deviation and right ventricular hypertrophy. EXCEPT "Tricuspid Atresia" - i.e., abnormal opening of Tricuspid valve.....Tricuspid atresia has a hypoplastic right ventricle and, therefore, the ECG shows left axis deviation and left ventricular hypertrophy.

2. Naloxone is not used during resuscitation of a newborn whose mother is on1.Methadone-----ans2.Phencyclidine3.Amphetamine4.CocaineExplanation- Methadone(opioid)high t-1/2 opioid dependence of neonateopioid withdrawal syndromehyper secretionairway blockFrom nelson Naloxone is contraindicated in infants born to mothers with opiate addiction because it precipitates acute neonatal withdrawal with severe seizures (i.e. Not given to mother)

3. Meconium aspiration is carried out 3 times in a non-vigorous baby, still no respiration. The next step will be 1.Chest compression2.O2 inhalation3.Bag and mask intubation4.Tickling the sole of the foot----ansExplanation-MAS(P=139/140)Baby resuscitationTABC

1. Temp(Make dry) MSL--> Vigorous or non-vigorous (Colour* of the baby is not the criteria)a. Vigorous -

a. Good respirationb. Good muscle tonec. HR >100

d. Non-Vigorous - absence of any one of above three points2. Airways3. Tactile4. Bag-mask(30 sec) if HR <60*5. CC(30 sec)If not successful6. Adr. (IV best route)

4.Intraosseous route can be used up to1.<6 year2.<12 year3.<10 year4.Any age-----ansExplanation-Children-Upper tibia mediallyAdult-Lower tibia just below Medial Meleoli

5.95% of newborns pass at urine within-1.24 hr-----ans2.36 hr3.48 hr4.12 hrExplanation- Meconium

• 24 hr(92-94 %)• 48 hr(98-99 %)

6.All reflexes are present at birth except-1.MORO2.ATNR3.STNR----ans4.Crossed extensorExplanation-(P-145-for all reflexes)STNR(Symmetric Tonic Neck Reflex)-

Flexion of neck flexion of handsExtension of neck extension of hands

If it persists there is no creeping(By 6 months child bears weight on extended elbows)

7.Persistent Moro’s reflex at 12 weeks indicates-1.Normal child-----ans2.Brain damage3.Irritable child4.Hungry childExplanation-MOROVestibular reflex3 mth(12 wks) MORO absent in 90%If persistent at 5-7 mthsabnormal

8.Best treatment for Erythema Toxicum is-

1.Erythromycin2.Ampiciliin3.Clotrimazole Cream4.No treatment------ansExplanation-(P=146)Never appears on day one. Always appears on day 2disappears by 7 days.Shows *eosinophilic infiltrations.

9.Heat loss in LBW baby at birth is maximum by-1.Conduction2.Convection3.Radiation4.Evaporation------ansExplanation- Drying at birth prevents heat losses d/t evaporation. After that radiation is the most important cause of heat loss.

• Convection causes maximum loss in incubator.

10.Breast milk can be stored in freezer for1.1 month------ans2.24 hrs3.10 hrs4.3 monthsExplanation-

Room temperature8-10 hrs

Refrigerator 24 hr

Freezer 1 mth(3 mth given in ghai is incorrect)

11.An infant is born at term and weighs 1600gms. The infant’s weight, length , and head circumference are less than fifth percentile. Which of the following is NOT a cause of the growth retardation1. Congenital infection2. Preeclampsia-------ans3. Cell toxins4. Chromosomal defectsExplanation-

Symmetric IUGR TORCHChromosomal abnormalitiesDysmorphic syndromeDrug/Radiation exposure in 1st trimester

Asymmetric IUGR Drug/Radiation exposure in late 3rd trimester

39.Which of the following is true regarding cretinism-1.Short limbs compared to trunk------ans2.Proportionate shortening3.Short trunk and short stature4.Short limbs and long statureDiscussion-Upper segment(1.7) : (1.0)Lower segmentGH deficiency-->Proportionate short stature

12. Neonatal sepsis is associated with all except1.Increased CRP2.Increased haptoglobulins3.Increased ESR4.Gastric aspirate > 5 PMN

5.All of the above are present in neonatal sepsis------ansExplanation-(P=161)Six screening test for NNS

1. CRP2. Haptoglobin3. ESR4. Gastric aspirate >55. TLC (high or low)6. Band cell ratio or percentage(miniature neutrophils)

i. Ratio > 0.2ii.Percentage > 30%

M/C cause E. Coli

13. A term infant has not passed meconium for 48 hrs. He presents with distension of abdomen and emesis since one day. Next most appropriate investigation would be-1.Genetic testing for cystic fibrosis2.Anorectal Manometry3.Lower bowel contrast enema------ans4.OesophagoscopyExplanation-

• Firstanorectal anomaliesthenmeningomyelocoele(coz leads to paralytic ileus)contrast enema(structural malposition of duct)meconium plug can be foundthen sweat chloride for CF

• Manometry or biopsyrule out Hirschprung

14. Vomiting on the first day of birth is caused by 1.Pyloric stenosis2.Oesophageal atresia3.Aerophagy4.Amniotic gastritis----ansExplanation-If regurgitation (effortless unlike vomiting) is in place of vomiting AerophagyAmniotic gastritis do Stomach wash

15. Earliest signs of NEC is1.increased gastric aspirates2.Abdominal distension-----ans3.Bleeding per rectum4.PneumoperitoneumANSWER- B(P=164)Risk Factor- PrematurityMetabolic triad-

1) Low pH2) Low Na3) Low platelet count

Treatmentnon-specificPrevention-Antenatal steroids-Also prevents:

IVH HMD, Bronchopulmonary dysplasia PDA NEC

• Antenatal steroids does not protect againstROP(Retinopathy of prematurity)• Slowly induce feed

16.Transient tachypnea of new born (TTNB) is commonly seen in which of the following situations

1.Full term requiring caesarians section2.Term with precipitate labor3.Term with prolonged labor4.All-----ansExplanations-Risk factors-

• Precipitate labour, Prolonged labour• Male sex, Macrosomia, Maternal sedation or excess i.v. Fluid• CS, Cord milking, Cord clamping delay

In TTNB there is fluid overload in lungs-->new born is asymptomatic except for the tachypnoea-->fluid is cleared by the lymphatics within 24 hrs

X-Ray-Neonate at age 6 hours. Overaeration and streaky, bilateral, pulmonary interstitial opacities and prominent perihilar interstitial markings are seen along with mild cardiomegaly

18.Nile blue sulphate test in amniotic fluid is a test of1.Lung maturity2.Skin maturity----ans3.Liver4.KidneyExplanations- (Best is foetal lung maturity-->RxPG)Amniotic fluid aspiration-->make smear-->then stain-->orange blue(acanthocytes)-->it was used to assess lung maturity

Saturated phosphadylcholinesterase-->best test for lung maturity

19.A newborn has Apgar score of 3 at one minute.On examination cyanosed, apex beat shifted to right side and scaphoid abdomen.He is resuscitated with Bag & tube ventilation.But no improvement, next step will be 1.Orogastric suction2.X-ray chest3.End tidal CO2-------ans4.SurgeryExplanation-Diaphragmatic hernia-->decompress the stomach(oro-gastric intubation)-->no improvement after intubation-->confirm the position of the tube-->End tidal CO2

20.Most effective mechanism of intensive phototherapy is1.Photoxidation2.Photoisomerisation

3.Structural isomerisation----ans4.NoneExplanation- Lumirubin formation(irreversible so important metabolism)Phototherapy source-->distance as close as the babyS/E-

• Diarhhoea

• BB Syndrome-->occurs in conjugated hyperbilirubinemia• Thrombocytopenia/Hypocalcemia

21.Double Volume (160ml/kg) Exchange transfusion replaces______% neonatal blood volume1.87%------ans2.67%3.97%4.100%Explanation- O -ve RBCs suspended in AB plasma-->best blood==> next best is O -ve` whole blood

22. 24 yr old primipara develops toxemia in her last trimester and treated with MgSO4.At 38 weeks ,she delievered 2.1 kg baby,kept in ICU at 18 hrs of age.Had a hematorit of 79%,platelet-1.7 lacs,glucose-48mg%, Mg-2.8 meq/lit and Ca -8.7 mg/dl.At 12 hr baby had a seizure.Most likely cause will be1.Polycythemia------ans2.Hypoglycemia3.Hypocalcemia4.HypermagnesemiaExlanation-Hematocrit > 68% is polycythemiaPolycythemia-->M/C cause-->IUGR(i. e. Chronic fetal hypoxia)Rx: Phlebotomy(replace extra blood with normal saline)

Hypoglycemia

• <40 is symptomatic hypoglycemia(Threshhold of hypoglycemic seizures) • WHO-

▪ NN hypoglycemia <47▪ After Neonatal period <54

Calcium < 7.5 leads to seizure

25. M.C congenital abnormality in infant of diabetic mother is1.CVS------ans2.Neural tube defects3.Caudal regression syndrome4.Lazy colonExplanation-Maximum Risk increased is of NTD(20 times more in IDM)For CVS it is 18 times increased-->still it is M/C-->Assymetric septal hypertrophy-->asymptomatic disappears after birth to 2 months. Second M/C CVS anomaly VSD then TGA

Organ spared by insulin--> Kidney + Adrenals

26.I.V glucose is given in all except1.IDM2.H/o Hyperglycemia3.H/o unconsciousness----ans4.NeonateExplanation- Following are C/I in unconscious pt.-->

• Vit. B-1• Naloxone• Glucose

27.Intergrated Management of childhood & neonatal illness includes except1.ARI2.Malaria3.Diarrhea4.TB-----ansExplanation-Read IMNCI

0-2 mth Bacterial infection, Jaundice, Diarrhoea,Dysentry

2-5 yr Pneumonia, Measles, Malaria, Malnutrition,Otitis Media, Anemia, Diarrhoea, Dysentry

32.In PEM all are increased except1.Insulin----ans2.Corisol3.Growth Hormone4.ProlactinExplantion-Stress hormone high-->GH,Prolactin,Cortisol

33.Ht.Velocity in females peaks at SMR stage-1.22.3----ans3.44.5Explanation-In males Stage 4Max. Weight velocity-->stage 4 for both males and females

Onset of ADHD < 7 yrs

Onset of Autism < 3yr

35.False regarding breath holding spells is1.It must be immediately attended to prevent hypoxia and onset of seizure-----ans2.Parents are advised to ignore and not o reinforce these attacks3.It does not contribute to increased risk of seizure disorder4.It is fairly common in the first two years of lifeExplanation-Types-

1.2.

There are four types of breath holding spells.1. The most common is termed simple breath holding spell, in which the manifestation is the holding of breath

in end expiration. The usual precipitating event is a frustration or injury leading to the temporary cessation of breathing in end expiration. There is no major alteration of circulation or oxygenation and the recovery is spontaneous.

2. The second type are the Cyanotic breath-holding spells. Cyanotic-->most common in males-->ignore in safe position. They are usually precipitated by anger or frustration although they may occur after a painful experience. The child cries and has forced expiration sometimes leading to cyanosis (blue in color), loss of muscle tone, and loss of consciousness. The majority of children will regain consciousness. There is no "post ictal" phase (as is seen with seizures),

no incontinence, and the child is fine in between spells. EEGs are normal in these children. There is no relationship to the subsequent development of seizures or cerebral injury as a consequence of breath holding spells.

3. In the third type, known as Pallid breath-holding spells*, the most common stimulus is a painful event. Pallid-->painful stimulus-->most dangerous-->may lead to severe bradycardia-->Atropine. The child turns pale (as opposed to blue) and loses consciousness with little if any crying. The EEG is also normal, and again there is no post ictal phase, nor incontinence. The child is usually alert within a minute or so. There may be some relationship with adulthood syncope in children with this type of spell.

4. A fourth type, known as Complicated breath-holding spells, may simply be a more severe form of the two most common types. This type generally begins as either a cyanotic or pallid spell that then is associated with seizure like activity. An EEG taken while the child is not having a spell is still generally normal.

36.Best parameter for assessing adolescent obesity is1.Weight for age percentile chart2.Skin fold thickness measurement------ans3.Kanawati index4.Body mass indexDiscussion-Kanawati index-->for PEMBMI has errors-->coz muscle-men will have muscle weight without much fat

Overweight BMI exceeds 85th percentile for that age and sex

Obesity BMI exceeds 95th percentile for that age and sex/Skin fold thickness above 85% also suggests obesity

37.A 2 mo child of 46, XY karyotype presents with ambiguous genitalia, BP 116/86 mmHg.The most likely enzyme deficiency is1.21-hydroxylase2.17-hydroxylase-----ans3.11-hydroxylase4.3-beta hydroxysteroid dehydrogenaseDiscussion:See 116/86 is hypertensive for neonate (Newborn BP- 80/60)

38.A 2 wk old breast feed baby presents with frequent vomiting and FTT ,There are features of moderate dehydration, Blood sodium is 122 m eq/l and potassium is 6.1 m Eq/L B.urea=44,S.creatinine=0.6. The most likely diagnosis is-1.Gitelan syndrome2.Bratter syndrome3.21 hydroxylase deficiency------ans4.Ac.Tubular NecrosisDiscussion-Males present in 2nd week of age-->adrenal crisis-->Polyuria, Dehydration, Shock,Acidosis,Hypoglycemia(ck)Female presents with ambiguous genitalia/virilismIf K+ is <5 then-->4

ATN presents with acute renal failure (ARF) and is one of the most common causes of ARF. The presence of "muddy brown casts" of epithelial cells found in the urine during urinalysis is pathognomonic for ATN.

40.In Familial Short stature relationship of chronical age (CA), bone age (BA) and height age (HA) is1.CA=BA>HA-----ans2.CA>BA=HA3.CA<BA=HA

4.CA>BA>HADiscussion- ghai-50Constitutional-->B(delay in growth spurt;bone age), growth velocity is normal

41.Most common site and time for taking blood sample or neonatal hypothyroidism-1.Cord blood at time of birth---ans2.Heel pad at birth3.Heel pad at 3 days4.Cord blood 2 hrsDiscussion- Correction in new ghai-->take from heel(duration not specified)Why not 1st 3 days-->TSH(stress hormone)-->delivery (stress)-->TSH increased-->high on day one-->so taken at the time of birth-->then repeated on day 4Why cord blood-->compliance is more

42.Common to acute and chronic malnutrition is1.wt. for age-----ans2.wt. for height3.ht. for age4.BMI

43.Consider the following1.Growth retardation2.Skin changes3.Mental apathy4.Hair changesWhich of these is/are the constant clinical feature(s) of kwashiorkor-a.1 onlyb.1 and 3-------ansc.2,3, and 4d.1,2,3,4Discussion-

1. Psychomotor slowing2. GR3. Oedema

44. A 5 year boy has precocious puberty bp 130/80.Estimation of which of the following will help in diagnosis?1.17-Hydroxyprogesterone2.DOCA3.Aldosterone4.11-Deoxycortisol--------ansDiscussion-

Delayed puberty Males-->Constitutional

Females-->Turner's syndrome

47. A child with alopecia, psoriatic dermatitis in the genitals and mouth, and hypogonadism is suffering from-1.Copper deficiency2.Iron deficiency3.Zinc deficiency-------ans4.Magnesium deficiencyDiscussion-Biochemical marker of zinc-->Thymulin(secreted from thymus)Zinc recommended in all diarrhoea after 2 mths of age for 10 days

48.In a child with absent thumb, radial deviation of wrist, bowling of forearm, which of the following investigation will not be useful?1.Bone marrow examination2.Platelet count3.Karyotyping4.2D ECHO------------ans(supported by AA-I 77; ROAMS+)Discussion- Echo also useful--> http://emedicine.medscape.com/article/960401-diagnosisFanconi anemia--> thumb malformation

49.Content of Citrate in ORS is____ millimoles/L1.202.153.10----ans4.30Discussion- (P=272,109); ROAMS-725Bicarbonate-->30(since 1 millimole of citrate-->3 millimole of bicarbonate)

50.A 4kg infant with severe dehydration(10%) needs ____amount of IV fluid in the first 24 hrs1.1200ml2.800ml-------ans3.1000ml4.600mlDiscussion-Upto 10 yrs of age- IV fluid required= Maintainence+Dehydration

Maintainence + Dehydration

(100) x (wt. in kg) + (% of dehydration) x (weight in grams)

(100 x 4) + (10/100 x 4000) = 800

51.Most common cause of rectal bleeding in infancy is-1.polyp2.intussusception-----ans3.diverticulum4.anal fissureDiscussions-In infancy-

1. Intusseptions(occurs during weaning period)2. Fissure

For >1 yr-1. Fissure2. M eckel's diverticulum(Massive bleeding)

Polyps not seen in children.

52. A 2-month baby presents with history of jaundice, tumeric colored urine and pale stools since birth, Examination reveals liver span of 10cms, firm in consistency and spleen of 3cms,The most specific investigation for establishing “the diagnosis would be-1.Liver function tests2.Peroperative cholangiogram----ans3.HIDA scan4.Liver biopsyDiscussion-if option B is not given-->then biopsy-->

• Giant cells on biopsy-->hepatitis

• lakes of bile channels-->biliary atresiaBiliary atresia should be operated before 60 days...HIDA scan -->rules out diagnosis of biliary atresia-->does not diagnose it

53.A 7 year girl from Bihar presented with 3 episodes of massive hematemesis and malena.There is no history of jaundice. On examination she had a large spleen ,non palpable liver and mild ascites. Portal vein was not visualized on ultrasonography Liver function tests were normal and endoscopy revealed esophageal varices. Most likely diagnosis1.Kala-azar with portal hypertension2.Portal hypertension of unknown etiology3.Chronic liver disease with portal hypertension4.Non-cirrhotic portal hypertension—ans (i.e extrahepatic portal HT M/C cause in Childhood PHT)

54.Statement not true about Wilson’s disease is-1.Wing beating tremors2.Hepatomegaly is earliest manifestation3.Kayser Fleischer ring4.Manifesting 1st fear of life------ansDiscussion-

• Screening: Serum Ceruloplasmin• Most specific: Liver biopsy• Liver signs don't occur in < 4 yr• CNS sign's don't occur in < 8yr

CNS-->basal ganglia-->behaviour abormality,psychiatric problems(Sensory system never affected)KF-->disappears after treatment; may be absent in young patients with liver disease but are always present in patients with neurologic symptoms.

55.Anatomic closure of ductus arteriosus occurs-1.Immediately after birth2.2-3 week of age----ans3.10-15 hr of age4.3 months of ageDiscussion-

Ductus arteriosus 2-3 wks(func closure 72 hrs)

Foramen Ovale 3 mths of age

Umbilical vessels and ductus venosus..... 2 -3 mnths

56. A newborn female child, weight 3.5 kg, delievered by uncomplicated delievery ,developed respiratory distress immediately after birth.On chest x-ray ground glass appearance was seen.Baby put on mechanical ventilation and was given surfactant but condition of baby deteriorates and increasing hypoxemia was present.A full term female sibling died within a week with the same complaints .ECHO is normal.Usual cultures are negative.Your diagnosis is-1.Total anomalous pulmonary vein connection2.Meconium aspiration syndrome3.Neonatal pulmonary alveolar proteinosis---ans4.Disseminated HSV infectionDiscussion-all features of HMD+previous sibling deathterm babies affectedheriditory defect-->Surfactant composition is abnormal-->protein B m/c

57.A 29 day old child presents with features of CHF,LHV and short systolic murmur.Which of the following is the most likely cause?

1.TOF2.VSD-----ans3.TGA4.Rheumatic feverDiscussion-L-->R shunt-

• ASD• VSD• PDA

All present around 4 wks-->why-->Coz initially pul.pressure high-->increase in RV pressure• So no shunting-->no CHF(pressure of RV=LV)• At 4 wks-->physiological decrease in pul. pressure-->shunting occurs-->CHF• VSD(M/C type perimembranous)-->LVH first-->then pul pressure increases-->pul HT-->RVH

59.A child with VSD developed Eisenmenger syndrome.What is the corret sequence?1.L to R shunt,RHV,Pulmonary hypertension ,R to L shunt2.L to R shunt,Pulmonary hypertension ,RVH,R to L shunt-----ans3.L to R shunt,RHV,R to L shunt, Pulmonary hypertension4.Pulmonary hypertension ,L to R shunt,RHV,R to L shunt

60.TOF consists of all except-1.PS-----ans2.RVH3.Overridding of aorta4.Large VSDDiscussion- PS may occur but by definition it is Rt. Ventricular pul. Outflow obstruction(i.e. infundibular stenosis)

61.Potts shunt is-1. Rt subclavian artery ti rt pulmonary artery2. Descending aorta to left pulmonary artery------ans3. Left subclavian to left pulmonary artery4. Ascending aorta to right pulmonary artery

62.A 5 day,full term male baby was severly cyanosed and later ballon atrial septostomy was done which showed improvement in oxygenation. Most likely Dx is1.Tetralogy of Fallot2.Transposition of great vessels-----ans3.TAPVC4.Tricuspid atresiaDiscussion-TGA-->two different blood cycles occur independently in the great vessels-

• Oxygenated blood cycle• Venous blood cycle

The child can-not survive if there is no mixing-->give PGE to keep ductus artriosus patent-->then do atrial septal rupture-->ruskin baloon atrial septotomy-->patient improves(Jerney arterial switch sugery-->best Sx)

63.A neonate presents with recurrent abdominal pain,restlessness,irritability and diaphoresis on feeding.Cardiac auscultation reveals a nonspecific murmur .He is believed to be at risk for MI. Most likely diagnosis is1.ASD2.VSD3.TOF4.Abnormal origin of the coronary artery---ansDiscussion- ALCAPA(Abnormal Coronary Artery from Pulmonary Artery)Starts from pulmonary artery-->leads to dilated cardiomyopathy-->CHF may occur

Only surgically correctible cause of DCM

64. GFR in children approaches adult values(when corrected for surface area) by-1.1yr2.2yr------ans3.3yr4.4yrDiscussion-ans is from RxPG with referencediluting capacity acquird at-->birthconcentrating capacity-->2nd yr(exact age 1.5 yr)

65. Macrocephaly with development delay with increase NAA may be seen with-1.Canavan’s disease-----ans2.Metachromatic leukodystrophy3.Adrenoleukodystrophy4.Alexander’s diseaseDiscussion- See AA-I P-78

• Leucodystrophy(White matter degenerated dystrophy-->grey matter is also affected but white more)

◦ Adrenoleukodystrophy :X-linked disease that affects young males, usually begining at 7 or 8 yrs old age. Characterised by demyelination specially posterior of the CNS, adrenal insufficiency, mental deterioration, aphasia, apraxia and dysarthria. Appx. 2/3 of pts. have loss of vision

◦ Cnanavan's disease -deficiency of aspartiocyclase-->NIAA increased-->dont read more

◦ Metachromatic leucodystro phy - a lysosomal storage disease which is commonly listed in the family of leucodystrophies. MLD is directly caused by a deficiency of the enzyme arylsulfatase. Without this enzyme, sulfatides build up in many tissues of the body, eventually destroying the myelin sheath of the nervous system.presents with weakness and decreased relexes and symmetric CNS demyelination, high urine metachromatic granules.

Q. Extensive involvement of deep white matter with hyperintense thalamic lesion on non-contrast CT scan of the brain is seen in:

a. Alexander’s disease

b. Krabbe’s ds. ----------answer.............

c. Canavan’s ds

d. Metachromatic leucodystrophy

It is also known as leukodystrophies. Some people are also called it Galactosylceramidase Deficiency Disease

66. Treatment of choice for primary grade –V B/L VUR in a 6-mo boy is:1.Antibiotic prophylaxis-----ans2.Ureteric reimplantation3.Cystoscopy f/b sub ureteric injection of Teflon4.Bilateral ureterostomyDiscussion- If 16 mth-->ans B. VUR-->new born females more common

67.Most specific finding in acute pyelonephritis is-1.Bacteria on gram stain-----ans2.WBC casts3.High grade fever4.x-ray KUBDiscussion-both 1 and 2 will be there but 1 more important since cast is not specific for UTI

68. Most common gene defect in idiopathic steroid resistance nephritic syndrome is1. NPHS 12. NPHS 2-----ans3. Actinin 4. None

Discussion- NPHS 2-->ARACTN-->alpha actinin 4 protein-->AD steroid resistant nephrotic syndrome(rare)

69. Which of the following is TRUE about post-streptococcal glomerulonephritis?1.50% of pharyngitis leads to glomerulonephritis2.Common during the summer season-------ans3.Early treatment of pharyngitis may decrease the incidence of glomerulonephritis4.Recurs commonlyDiscussion-

1. Only 2-3 % pharyngitis2. Skin infection is more common during this period3. PSGN no change in incidence even if phryngitis is treated4. HUS-->incidence rises(if treated)

70. An infant with history of diarrhea 5 days has urea 200mg% and creatinine 5 mg%.The platelet count is 90000. Fragmented RBC’s are found in the peripheral smear.Probable Dx is1.HUS-----ans2.DIC3.Microangiopathic hemolytic anemia4.HSPDiscussion- HUS-->5-10 days after diarrhoea or desentry....age <5 yrs..... IgA NEPHROPATHY vs POSTSTREPTOCOCCAL GN-

o BOTH ARE COMMON IN CHILDREN o BOTH MAY PRESENT AFTER URTI o BOTH PRESENTS WITH GROSS HAEMATURIA o BUT, WHILE IgA NEPHROPATHY PRESENTS WITHIN THREE DAYS AFTER URTI,

PRESENTATION OF PSGN IS USUALLY DELAYED TO MORE THAN TEN DAYS.o PSGN-->1-3 wks after URTI-->EHEC O157:7-->but in INDIA-->Shigella

IgA nephropathy M/C Chr. Glomerular d/e in the world

M/C Cause of recurrent hematuria

Causes of recurrent hematuria-1. Iga nephropathy2. Alport's Syndrome3. Benign familial hematuria(thin BM)4. Idiopathic hypercalcemia-->give thiazide(no restriction on calcium intake)

71. Most common cause of vasculitis in children-1.Acute rheumatic fever2.HSP3.PAN4.Kawasaki’s disease------ansDiscussion-M/C vasculitis-->KawasakiM/C leucocytoclastic vasculitis-->HSP

73.Which one of the following is the most common cause of abdominal mass in neonates?1.Neuroblastomas2.Wilm’s tumor3.Hydronephrosis-----ans4.Multicystic dysplastic kidneysDiscussion- From nelson:

1. Hydronephrosis2. Multicystic dysplastic kidney3. Neuroblastoma

At 1 yr-->Neuroblastoma

74. A 2 year girl presents with poor growth. She has episodes of vomiting and dehydration. Evaluation reveals hyperchloremic metabolic acidosis,normal creatinine, and persistently alkaline urine Rx of choice is-1.High-caloric nutritional supplements2.Antiesophageal reflux medications3.Alkalinizing agents normalize the serum CO2-----ans4.Pancreatic enzymes for cystic fibrosis

Causes of normal serum anion gap causes-1. Diarrhoea2. RTA*3. Uterosigmoidoscopy

75. Proximal RTA has all except-1.Hypokalemia2.Bicarbonate wasting3.Nephrocalcinosis-------ans4.Hyperchloremia

76. A 9 mo child is clinically diagnosed as barter syndrome. Most likely finding on renal biopsy is-1.Hyperplasia of juxtaglomerular apparatus------ans2.Interstitial nephritis3.Distal tubular dilatation4.Distal tubular necrosis

77. M.C. etiology of Renal Artery stenosis in children is-1.fibrointimal hyperplasia------ans2.takayasu arteritis3.fibromedial hyperplasia4.PANDiscussion-Takayasu--> Fibromuscular dysplasia, m/c cause of RAS in India

78.In what respect Marfan’s syndrome differs from homocystinuria-1.Arachnodactyly2.Mental defects-------ans3.Hyperextensible joints4.PectusDiscussion-Marfans-->fibrillin-->but in brain no colaagen is foundHomocystinuria-->thrombotic-->since stimulates factor XII

79.True about dietry Rx of galactosemia is-1.Must be continued life long-------ans2.Ensures normal intellectual development if started in the first week of life

3.Can be relaxed after puberty4.Involves delayed weaningDiscussion- (Ghai= 612)2-->maternal intake during pregnancy also affects brainGalactosemia Causes-

• Galactokinase• Uridyl transferase(M/C)• Epimerase

Galactose---(galactokinase--deficeiency causes cataract)--->Gal-1-phosphate----(U.P.---Liver and CNS also affected)--->Galactosemia

E.Coli sepsis is very very common in galactosemia-->Ck E.Coli survives on lactose(Ananthanarayan-272)

80.A child has recurrent hypoglycemia attacks & hepatomegaly.He is most likely suffering from1.Neonatal hepatitis2.Neonatal diabetes3.von Gierke disease------ans4.GalactosemiaDiscussion-No glycogen breaks into glucoseSpleen is not enlarged(no connection with glucose)

83. M.C. etiology of pneumonia at 3 mo-3yr is1.Streptococcus pneumoniae-----ans2.Hemophilus influenzae3.Mycoplasma pneumoniae4.Pneumocystis cariniiDiscussion-

< 3mths E.Coli

3mth-3 yr India-->H. influenzae

World-->Strep. pneumoniae(d/t vaccinations)

84. A child presents with history of respiratory infections.His sweat chlorides levels are 36 and 41 mEq/L on two different occasions.Which other test would you do to exclude the diagnosis of cystic fibrosis?1.Repeat sweat chloride measurements2.Nasal septum potential difference----ans3.Fat in stool for next 72 hours4.DNA analysis for delta F-508 mutationDiscussion- Infection: M/C Peudomonas aeruginosa, 2nd most common--> S. AureusClinicla features-

• Hallmark of cystic fibrosis are salty tasting skin,poor growth and poor weight gain despite a normal food intake

• Accumulation of thick, sticky mucus, frequent chest infections and coughing or shortness of breath

• +ve newborn screening test (trypsinogen* level high in blood)

• Sibling CCF

Laboratory features-

• Abnoramal sweat chloride-->at least 2 times

• Abnormal NSPD

• Mutations

86. Life threatening (Red flag) features of acute severe asthma in children include-1.Altered sensorium2.Pulsus paradoxus3.Audible wheeze in inspiration and expiration4.Oxygen saturation 92-95%Which of these statements is/are correct?a.1b.2,4c.1,2------------------ansd.1,2,3,4

87.Best Px of asthma in 1 year old chld is-1.inhaled b2 agonists2.inhaled steroids----ans(steroids are best but generally it is not provided in options) KDT-2273.Ketotifen4.LT antagonists

89.Neural tube defects in utrero occur during-1.3-4weeks-----ans2.7-8 weeks3.12-15 weeks4.5-6 monthsDiscussion- folic acid-->0.4 mg*If first baby is suffered-->then risk 4 times more

90. Pseudotumor cerebri (benign intracranial hypertension) is seen in all except-1.Hypervitaminosis A2.Tetracycline therapy3.Vitamin A deficiency4.Hyperbilirubinemia------ansDiscussion- Pseudotumor cerebri-Rx- LP for diagnosis usually suffices for symptoms-->if not controlled then-->Steroids(it's listed in cause also)

Treat before 4 wks to prevent blindness d/t optic atrophy

91. True statement about infantile spasms is-1.Onset usually between 2-4 years age2.There is a sudden flexion of trunk, neck and limbs followed by gradual relaxation----ans3.Usually results from metabolic abnormalities4.EEG shows a normal recordDiscussion-(P-511)EEG-->Hyps-arrythmia pattern-->totally irregularRx-->ACTH

92. True statement about febrile seizures is-1.Neurological deficit may be present in interictal period2.No need for anticonvulsant therapy----------------ans3.Leads to subsequent intellectual impairment4.Seizures are usually focal

Discussion-(P=509)Anticonvulsant thrapy-->long term thrapy(see p -402 KDT 6/e)Seizure type here-->generalisedRx-->Paracetamol+Diazepam

93. Most sensitive and specific investigation to diagnosis HIV infection in newborn is1.RNA PCR-------------ans2.P24 antigen assay3.Western blot4.G 124 antigen assayDiscussion-All below have equal specificity < 18 mths-

• RT PCR• RNA PCR• DNA PCR

94. Most common complication of mumps in is-1.Orchitis------------------ans2.Aseptic meningitis3.Myocarditis4.PancreatitisDiscussion-

• For children it's-->2• For measles in adults-->m/c complication Pneumonia; and for children Otitis Media

95. Diptheritic cardiomyopathy occurs most commonly in________ week of infection-1.4th2.3rd3.2nd------ans4.1stDiscussion-1st wk--> Bull's neck2nd wk--> cardiomyopathy, palatal palsy4th wk-->neuropathy(m/c)5th wk--> loss of accomodation

96. A child had fever and coryza for last 3 days f/b maculopapular erythematous rash which lasted for 48 hours leaving behind pigment .Dx is1.Measles2.Fifth disease3.Roseola infantum-----ans4.TyphoidDiscussion-Roseola infantum- Ulcers at uvuloplatoglossal junction (Nagayama* spots) are seenErythema infectiosum-->Parvo-virus B-19-->slapped cheek appearence-->Arthritis m/c complicationSixth disease-->HHV 6-->measles like rash-->fever absent at disappearence of rash-->duration of rash(1-3 days)

97. In a 2 month baby, seizure with chorioretinitis is suggestive of-1.Congenital toxoplasmosis------ans2.Congenital CMV3.Congenital Rubella4.Congenital syphilisDiscussion- M/C with toxoplasma-

• Chorioretinitis

• Calcification(stipple diffuse)• Hydrocephalous

99. A 4 mo boy develops a temperature of 101f and is irritable for 2 hrs after immunization with DPT vaccine.What is the most appropriate step when this boy is seen at 6 months of age?1.Defer immunization with the pertussis vaccine,and instead administer diphtheria and tetanus toxoid vaccine2.Defer immunization with the pertussis vaccine,and instead administer tetanus toxoid and reduced-dose diphtheria toxoid vaccine3.Administer the DPT vaccine with instructions for fever control-----ans4.Defer all immunizations until the infant is 12 months oldDiscussion- 2nd dose contraindicated when-

• Anaphyllaxis after 1st dose• Progressive encephalitis after 1st dose

100. Recurrence risk of Down’s syndrome. If previous sib had 14-21 translocation with mother +ve for carrier state is-1.2-3%2.100%3.12-15%----------ans4.1%Discussion-

• Chr. 13/14/15/22 translocation occurs with 21(m/c)-->10-15% risk in 2nd pregnancy if mother is carrier(but if father is carrier risk is 2-3%)

• Chr. 21 to 21 translocation then-->100% risk in 2nd child

101. In Down’s syndrome, false is1.Increased PAPP-A------ans2.Increased b-HCG3.absent nasal bone4.Reversal of diastolic blood flowDiscussion- (P=107)Down's Syndrome-->HCG high(only one among the triple markers)3rd option-->may be found in any aneuploidy

102. 1st Phenotypic change in Heredietary hemochromatosis is1.Post parandial increase in S.iron-----ans2.Increased S.Ferritin3.Increased transferring saturation4.Slate grey pigmentationDiscussion-Phenotypic-->Clinical+BodyIf Option 1 is not given then go for-->3

103. Fetal to adult hemoglobin switch over occurs in utero at1.16 week2.20 week3.30 week-----------ans4.14 weekDiscussion-

• At 8 wks erythropoiesis shifts to liver• At birth-->70% fetal blood Hb-->at 2 mth-->equal to adult

104. The best drug used for chelation therapy in beta thalassemia major is-

1.i.v. desferrioamine2.s.c. desferroxamine------ans3.Oral deferiprone4.Oral succimerDiscussion- if no 2 then go for option-->3Therapy for thallasemia should start at---->3 yrsS/E of desferriooxamine-

• Vision weakening• Bony dysplasia• Deafness• Short stature• Sepsis(yersinia group of organisms)

S/E of deferiprone-• GI upset• Arthritis*• Neutropenia/Agranulocytosis

105. A 3 year girl with sickle cell anemia presents with pallor, tachycardia, hypotension, and massive splenomegaly .Most likely explanation is1.Hemorrhagic shock2.Splenic sequestration----------ans3.Septic shock4.Carcinogenic shockDiscussion- Osteomyelitis in SCD-->SalmonellaEarliest menifestation of SCD in children-->Hand foot syndrome(also called dactylitis)--> Painful swelling of the hands and feetHighest success rate in thalassemia-->BM transplantation

106. 4yr child presented with severe pallor petechiae weakness, ecchymotic rash on lower legs but no heopatosplenomegaly. He has abdominal pain 2 weeks back.Probable diagnosis is1.ITP2.Aplastic anemia-----ans3.Leukemia4.Ac viral illnessDiscussion- (P=323)Severe pallor-->ITP ruled out-->also spleen usually not palpable

107. Which factor deficiency is the cause of episatxis & echymosis when h/o umbilical stump bleeding is present?1.X2.V3.XIII-----ans4.VIIDiscussion-

• Infertility in males-->since takes part in spermatogenesis• Recurrent abortions in females-->takes part in placental adhesions• Test is done in bipolar urea solution-

▪ clot dissolves-->in this disease▪ N-->clot does not dissolve

109. M.C. presentation of neuroblastoma in children is-1.Lytic lesion in skull with suture diaschisis2.Abdominal lump----ans3.Renal invasion

4.Secondaries in the brainDiscussion- lobulated lump-->crosses midline--> secondaries in bone

110. Wilm’s tumor is associated with all except-1.Aniridia2.Beckwith syndrome3.Polycystic kidney----------ans4.HemihypertrophyDiscussion-(P=574)

• Wilm's secondary-->lungs• WAGR(Wilm's, Aniridia, Genital anomalies, Retardation mental)• Dany-Drash-MM

▪ Male pseudohermaphroditism▪ Mesangial Sclerosis of kidney

• Favouable histology-▪ Blastemal cell▪ Epithelial cell▪ Stromal cell

• Unfavourable histology-▪ Clear cell sarcoma(give cyclophosphamide)-->secondary bone