Saturday, December 26, 2009

41 - Differential diagnosis of Neonatal Jaundice

A. Jaundice consisting of either direct or indirect  bilirubin, that is present at birth or appears with in the first 24 hours of life causes :
*Erythroblastosis fetalis (high direct bilirubin - in infants who were given intrauterine transfusions)
*Concealed hemorrhage
*Sepsis
*Congenital infections like syphilis, CMV, rubella and toxoplasmosis
*Jaundice secondary to extensive eccymosis or blood extravasation may occur during the first day or later, especially in premature infants


B. Jaundice which first appears on the second or third day causes :
*Physiological jaundice
*Familial non-hemolytic icterus (Crigler-Najjar syndrome)
*Early onset breast feeding jaundice

C.Jaundice appearing after the third and within 1 week causes :
*Bacterial sepsis
*Urinary tract infection
*Infections like syphilis, toxoplasmosis, CMV or enterovirus

D. Jaundice first recongnised after the first week of life causes :
*Breast milk jaundice
*Septicemia
*Congenital atresia or paucity of the bile ducts
*Hepatitis
*Galactosemia
*Hypothyroidism
*Cystic fibrosis
*Congenital hemolytic anemia crises related to red cell morphology and enzyme deficiencies


E. Persistant jaundice during the first month of life causes :
*Hyperalimentation associated cholestasis
*Hepatitis
*Cytomegalic inclusion disease
*Syphilis
*Toxoplasmosis
*Familial non-hemolytic icterus
*Congenital atresia of the bile ducts
*Galactosemia
*Inspissated bile syndrome following hemolytic disease of newborn
*Rarely physiologic jaundice may be prolonged for several weeks, as in infants with hypothyroidism or pyloric stenosis.

P.S: Polycythemia may also lead to early jaundice.

Tuesday, December 22, 2009

40 - Treatment of Vesicoureteral reflux in children




Grade IReflux into a non-dilated ureter
Grade IIReflux into the upper collecting system without dilatation
Grade IIIReflux into dilater ureter and/or blunting of calyceal fornices
Grade IVReflux into a grossly dilated ureter
Grade VGross dilatation of the ureter, renal pelvis and calyces: Calyces
show loss of papillary impression



*TREATMENT RECOMENDATION FOR VUR diagnosed following a UTI :


GRADEAGE(YRS)SCARRINGINITIAL TREATMENTFOLLOW-UP
I-IIAny-Antibiotic prophylaxis-
III-IV0-5-Antibiotic prophylaxisSurgery
III-IV6-10-U/L: Antibiotic prophylaxisSurgery
III-IV6-10-B/L: Surgery
Vless than 1-Antibiotic prophylaxisSurgery
V1-5NoU/L: Antibiotic prophylaxisSurgery
V1-5NoB/L: Surgery
V1-5YesSurgery
V6-0-Surgery

Tuesday, October 13, 2009

38 - Tracheo-oesophageal fistulas

 
 

*As u can see above there are five types of Tracheo-oesophageal fistulas . They are type a, type b, type c, type d, and type e.
*Another type called type f, has been recognised which has no fistula but is characterised by oesophageal narrowing.
*TYPE C is the most common type of tracheo-oesophageal fistula, followed by TYPE A and TYPE E.
*TYPE E, is also called the H TYPE fistula, because of its resemblance to the english alphabet H.

*TYPE E ( Tracheo-oesophageal fistula without atresia or H type fistula ) is associated with refractory bronchospasm and recurrent pneumonias.


Saturday, September 5, 2009

37 - Nephrotic Syndrome Mcqs with answers part 2

18q: In a patient presenting with features of NS , diagnosis other than MCNS should be considered in the presence of ?
a. Age less than 1 year
b. A family history
c. Extrarenal findings ( arthritis, rash and anemia )
d. Hypertension
e. Pulmonary edema
f. Acute or chronic renal insufficiency
g. Hematuria


19q: all of the following are true regarding the lab findings of idiopathic nephrotic syndrome except ?
a. Microscopic hematuria may be present in 20 % of children
b. Urinalysis reveals 3+ or 4+ proteinuria
c. Spot urine protein/ creatinine ratio exceeds 2.0
d. Urinary protein excretion exceeds 3.5 gm/24 hr in adults and 40 mg/sq.m/hr in children
e. Serum creatinine value is decreased


20q: all of the following are true regarding the lab findings of idiopathic nephrotic syndrome except ?
a. Serum albumin level is generally less than 2.5 g/dL
b. Serum cholesterol and triglyceride levels are elevated
c. C3 and C4 are elevated
d. Renal biopsy is not required for diagnosis in most children


21q: What is the most frequent type of infection which occurs as a complication of Nephrotic syndrome?
a. Pneumonia
b. Spontaneous bacterial peritonitis
c. Cellulitis
d. Urinary tract infections


22q: Which is the most common organism causing peritonitis in a nephrotic syndrome patient?
a. E.Coli
b. Streptococcus pneumoniae
c. Staphylococcus
d. Mycoplasma


23q: All of the following are true about nephrotic syndrome except ?
a. All children with nephrotic syndrome should receive polyvalent pneumococcal vaccine
b. Influenza vaccine should be given on a yearly basis
c. Children with NS are at increased risk for thromboembolic events
d. Prophylactic anticoagulation is recommended in all children with NS


24q: Example of a malignancy in which the renal pathology most often resembles Minimal change nephrotic syndrome ( MCNS ) ?
a. Lung carcinoma
b. Hodgkin lymphoma
c. Non-hodgkin lymphoma
d. GIT cancers


25q: Drugs which cause nephrotic syndrome with a histological picture of MCNS are ?
a. Probenecid
b. Ethosuximide
c. Methimazole
d. Lithium


26q: Drugs which cause nephrotic syndrome with a histological picture of membranous glomerulopathy are all except ?
a. Pencillamine
b. Captopril
c. Gold
d. NSAIDs
e. Mercury compounds
f. procainamide


27q: Drugs which cause nephrotic syndrome with a histological picture of proliferative glomerulonephritis are
a. procainamide
b. chlorpropamide
c. phenytoin
d. trimethadone
e. paramethadione


28q: Infants who develop nephrotic syndrome within the first ----- months of life are considered to have congenital nephrotic syndrome ?
a. first 3 months
b. first 6 months
c. first 9 months
d. first 12 months


29q: what is the most common cause of congenital nephrotic syndrome ?
a. Denys-Drash syndrome
b. Finnish type of congenital NS
c. Nail patella syndrome
d. Frasier’s syndrome


30q: Major features of the finnish type of congenital nephrotic syndrome are all except ?
a. Dilatation of the proximal tubules
b. Dilatation of the distal tubules
c. Mesangial hypercellularity
d. Glomerular sclerosis


31q: All of the following are true about finnish type of congenital NS except ?
a. Infants present with large placenta and marked edema
b. Prematurity and respiratory distress are seen in these infants
c. Separation of cranial sutures and massive proteinuria is seen in these infants
d. Corticosteroids are very helpful in the treatment


32q: All of the following are true about finnish type of congenital NS except ?
a. Progressive renal failure with death by the age of 5 years
b. This disease is detectable in utero by increased alpha fetoprotein levels
c. Inheritance is autosomal dominant type
d. Most common in populations of Scandinavian descent ( 1: 8000 incidence )
e. Mutation in the NPHS1 gene which produces nephrin



33q: all of the following are true about Denys-Drash syndrome except ?
a. Mutation in WT1 gene
b. Wilm’s tumor is a feature of it
c. Male pseudohermaphroditism is seen in it
d. Diffuse mesangial sclerosis is characteristic
e. WT1 gene is located on chromosome 13


34q: Which of the following drugs are used in the treatment of Idiopathic nephrotic syndrome ?
a. Prednisone
b. Cyclophosphamide
c. Methylprednisolone
d. Cyclosporine
e. Tacrolimus
f. Mycophenolate


35q: In the presence of which of the following features do u suspect the presence of secondary nephrotic syndrome ?
a. Age greater than 8 years
b. Hypertension and hematuria
c. Renal dysfunction
d. Extrarenal symptomatology ( rash, arthralgias, fever )
e. Raised serum complement levels


36q: Drugs causing nephrotic syndrome are ?
a. Gold
b. Pamidronate
c. Interferon
d. Heroin


37q: Steps taken to ameliorate nephrotic state in a patient with congenital nephrotic syndrome are all except ?
a. ACE inhibitors
b. Indomethacin
c. Unilateral nephrectomy
d. Corticosteroids



36 - Nephrotic Syndrome Mcqs with answers part 1

1q: Nephrotic syndrome is how many times more common in children than adults ?
a. 5 times
b. 10 times
c. 15 times
d. 20 times


2q: what is the incidence of nephrotic syndrome ?
a. 2-3/100000 children per year
b. 20-30/100000 children per year
c. 200-300/100000 children per year
d. 2000-3000/100000 children per year


3q: The characteristic features of nephrotic syndrome are ?
a. Heavy proteinuria ( greater than 3.5 g/24 hr in adults or 40 mg/sq.m/hr in children )
b. Hypoalbunemia ( less than 2.5 g/dL )
c. Edema
d. Hyperlipidemia
e. All the above


4q: Which of the following statements about nephrotic syndrome are true ?
a. Most children ( 90 % ) with nephrotic syndrome have a form of idiopathic nephrotic syndrome
b. Minimal change disease ( 85 % ) is the most common cause of idiopathic nephrotic syndrome
c. Focal segmental glomerulo sclerosis ( 10 % ) and mesangial proliferation ( 5 % ) are the next common causes of idiopathic nephrotic syndrome
d. The remaining 10 % who do not have idiopathic NS have secondary NS related to systemic or glomerular diseases such as membranous nephropathy or membranoproliferative glomerulonephritis
 e. All the above statements are true


5q: Which of the following causes nephrotic syndrome which is steroid responsive ?
a. Minimal change nephrotic syndrome
b. FSGS
c. Denys-drash syndrome with diffuse mesangial sclerosis
d. Nail-patella syndrome
e. Finnish type of congenital NS


6q: All of the following are true about nephrin except ?
a. It is a product of the gene NPHS1
b. Location of the gene is on 1q25
c. The inheritance is of recessive type
d. A defect in the production of nephrin causes finnish type of congenital nephrotic syndrome
e. The NS produced by nephrin defect is steroid resistant


7q: Alpha actinin-4 is a product of which gene ?
a. NPHS1
b. NPHS2
c. FSGS1
d. FSGS2
e. WT1


8q: All of the following are true about podocin except ?
a. It is product of the gene LMX1B
b. Inheritance is of recessive type
c. A defect in podocin production is associated with FSGS
d. Gene location is on chromosome 1q25


9q: gene for alpha-actinin-4 ( FSGS1 ) is located on which chromosome ?
a. 19q13
b. 1q25
c. 2q35
d. 9q34


10q: What is the renal disease causing steroid resistant nephrotic syndrome associated with the defect in gene LMX1B which produces the protein LIM-homeodomain ?
a. Denys-Drash syndrome with diffuse mesangial sclerosis
b. Nail patella syndrome
c. FSGS
d. MCNS


11q: In the nephrotic state , serum lipid levels ( cholesterol, triglycerides ) are elevated for the following reason ?
a. Hypoalbuminemia stimulates generalized hepatic protein synthesis, including synthesis of lipoproteins
b. Lipid catabolism is diminished, as a result of reduced plasma levels of lipoprotein lipase, related to increased urinary losses of this enzyme
c. Both a and b
d. None


12q: How much percentage of children with Minimal Change Nephrotic Syndrome respond to corticosteroid therapy ?
a. 95 %
b. 50 %
c. 20 %
d. 5 %


13q: Electron microscopy of a patient with FSGS shows segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen . A similar lesion may be seen with ?
a. HIV infection
b. Vesicoureteric reflux
c. Intravenous heroin abuse
d. All the above


14q: All of the following are true about FSGS associated nephrotic syndrome except ?
a. FSGS involves 85 % of all the cases presenting with idiopathic NS
b. 20 % of these cases respond to prednisone
c. Immunofluorence microscopy shows IgM and C3 staining in areas of segmental sclerosis
d. Electron microscopy shows segmental scarring of the glomerula tuft with obliteration of the glomerular capillary lumen
e. FSGS is frequently progressive, ultimately involving all glomeruli , and leads to end-stage renal disease in most patients


15q: Which of the following are uncommon findings in a patient with idiopathic nephrotic syndrome ?
a. Abdominal pain
b. Diarrhea
c. Anorexia and Irritability
d. Hypertension
e. Gross hematuria


16q: what is the differential diagnosis of a child with marked edema ?
a. Protein-losing enteropathy
b. Hepatic failure
c. Congestive heart failure
d. Acute or chronic glomerulonephritis
e. Protein malnutrition
f. All the above


17q: All of the following are true about idiopathic nephrotic syndrome except ?
a. More common in females than in males ( 2:1 )
b. Most commonly appears between the ages of 2 and 6 years
c. MCNS is present in 85-90 % of patients of less than 6 years of age
d. FSGS develops in older children
e. 20 -30 % of adolescents have MCNS



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

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



Thursday, March 12, 2009

30 - Nadas criteria



The assessment of a child for the presence or absence of heart disease can be done with the help of some guidelines suggested by nadas and are called "nadas criteria" . the criteria are divided into major and minor criteria. Presence of one major and two minor criteria are essential for indicating the presence of heart disease.

The major criteria are :

1. systolic murmur grade 3 or more specially with a thrill
2. diastolic murmur
3. cyanosis
4. congestive cardiac failure

The minor criteria are :

1. systolic murmur less than grade 3 in intensity
2. abnormal second heart sound ( S2)
3. abnormal ECG
4. abnormal X-ray
5. abnormal blood pressure



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