Globoid cells is a diagnostic feature of

Progressive multifocal leukoencephalopathy

Association of Bombay blood group is seen with

Leukocyte adhesion deficiency type 2

Leukocyte adhesion deficiency type 1

Leukocyte adhesion deficiency type 2

Gitelman syndrome is characterised by all except

Hypokalemic metabolic alkalosis

MCQ Snacks (Free Trial) Paediatric Ss

1.

False about Treacher Collin Syndrome
- A.
  
  Autosomal dominant
- B.
  
  Mongoloid slant
- C.
  
  Micrognathia
- D.
  
  Primary affects the face
Correct Answer
B. Mongoloid slant

Explanation
Anti mogoloid slant is seen

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2.

Globoid cells is a diagnostic feature of
- A.
  
  Krabbe's disease
- B.
  
  Progressive multifocal leukoencephalopathy
- C.
  
  Tay sach's disease
- D.
  
  Metachromatic leukodystrophy
Correct Answer
A. Krabbe's disease

Explanation
Globoid cells are a diagnostic feature of Krabbe's disease. Krabbe's disease is a rare genetic disorder that affects the nervous system. It is characterized by the accumulation of certain types of cells, known as globoid cells, in the brain and other parts of the body. These cells are a hallmark of the disease and can be observed under a microscope. Krabbe's disease causes progressive damage to the nervous system, leading to symptoms such as muscle weakness, developmental delays, and vision loss. Early diagnosis and treatment are crucial for managing the symptoms and slowing down the progression of the disease.

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3.

T2 hyperintensities in frontal white matter in MRI brain is seen in
- A.
  
  Alexander disease
- B.
  
  Canavan disease
- C.
  
  Adrenoleukodystrophy
- D.
  
  Krabbe's disease
Correct Answer
A. Alexander disease

Explanation
T2 hyperintensities in frontal white matter in MRI brain are seen in Alexander disease. This is a rare and progressive genetic disorder that affects the central nervous system. It is characterized by the presence of abnormal protein deposits called Rosenthal fibers in the brain. These fibers can be visualized as T2 hyperintensities on MRI scans, particularly in the frontal white matter. Canavan disease, adrenoleukodystrophy, and Krabbe's disease are not associated with T2 hyperintensities in the frontal white matter.

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4.

Association of Bombay blood group is seen with
- A.
  
  Leukocyte adhesion deficiency type 1
- B.
  
  Leukocyte adhesion deficiency type 2
- C.
  
  Chediak higashi syndrome
- D.
  
  Myeloperoxidase deficiency
Correct Answer
B. Leukocyte adhesion deficiency type 2

Explanation
The association of the Bombay blood group is seen with Leukocyte adhesion deficiency type 2. The Bombay blood group is a rare blood type characterized by the absence of the H antigen on red blood cells. Leukocyte adhesion deficiency type 2 is a genetic disorder that affects the immune system and impairs the ability of white blood cells to migrate to sites of infection. The connection between the Bombay blood group and Leukocyte adhesion deficiency type 2 suggests a shared genetic basis or underlying mechanism between the two conditions.

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5.

Which is not the major criteria of Staphylococcal toxic shock syndrome
- A.
  
  Hypotension
- B.
  
  Acute fever
- C.
  
  Rash
- D.
  
  Mucous membrane involvement
Correct Answer
D. Mucous membrane involvement

Explanation
Staphylococcal toxic shock syndrome is a severe illness caused by toxins produced by Staphylococcus aureus bacteria. The major criteria for this syndrome include hypotension (low blood pressure), acute fever, and rash. Mucous membrane involvement is not considered a major criteria for diagnosing Staphylococcal toxic shock syndrome.

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6.

False about Ebola virus
- A.
  
  Coronavirus family
- B.
  
  Incubation period 2-21 days
- C.
  
  Diagnosed by PCR
- D.
  
  None of the above
Correct Answer
A. Coronavirus family

Explanation
Belongs to Flaviviridae family

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7.

The following skin lesion is seen in which heart disease
- A.
  
  Eissenmenger syndrome
- B.
  
  Rheumatic heart disease
- C.
  
  Infective endocarditis
- D.
  
  Heart failure
Correct Answer
B. Rheumatic heart disease

Explanation
Erythema marginatum

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8.

Complication of celiac disease are all except
- A.
  
  Intenstinal T cell lymphoma
- B.
  
  Refractory sprue
- C.
  
  Hypersplenism
- D.
  
  Osteoporosis
Correct Answer
C. Hypersplenism

Explanation
Hyposplenism is seen

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9.

Which of the following exocrine glandular ducts are not obstructed in cystic fibrosis
- A.
  
  Pancreas
- B.
  
  Lung
- C.
  
  Sweat glands
- D.
  
  All of the above
Correct Answer
C. Sweat glands

Explanation
In cystic fibrosis, the exocrine glandular ducts in the pancreas and lungs are obstructed, leading to various complications. However, the sweat glands are not obstructed in cystic fibrosis. This is because the defective CFTR protein, which is responsible for the obstruction in the pancreas and lungs, does not affect the sweat glands in the same way. As a result, individuals with cystic fibrosis often have abnormally salty sweat, which can be used as a diagnostic tool for the condition.

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10.

Gitelman syndrome is characterised by all except
- A.
  
  Hypercalciuria
- B.
  
  Hypomagnesemia
- C.
  
  Muscle cramps
- D.
  
  Hypokalemic metabolic alkalosis
Correct Answer
A. Hypercalciuria

Explanation
Hypocalciuria is seen

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MCQ Snacks (Free Trial) Paediatric Ss

False about Treacher Collin Syndrome

Globoid cells is a diagnostic feature of

T2 hyperintensities in frontal white matter in MRI brain is seen in

Association of Bombay blood group is seen with

Which is not the major criteria of Staphylococcal toxic shock syndrome

False about Ebola virus

The following skin lesion is seen in which heart disease

Complication of celiac disease are all except

Which of the following exocrine glandular ducts are not obstructed in cystic fibrosis

Gitelman syndrome is characterised by all except