Category: Quiz

Anti-Neutrophil Cytoplasmic Antibody (Anca)

Anti-Neutrophil Cytoplasmic Antibody (Anca)

Q. 1

Which of the following types of hepatitis is associated with an immune-mediated vasculitis characterized by p-ANCA antibodies?

 A

Hepatitis A

 B

Hepatitis B

 C

Hepatitis C

 D

Hepatitis D

Q. 1

Which of the following types of hepatitis is associated with an immune-mediated vasculitis characterized by p-ANCA antibodies?

 A

Hepatitis A

 B

Hepatitis B

 C

Hepatitis C

 D

Hepatitis D

Ans. B

Explanation:

Hepatitis B is associated with polyarteritis nodosa (PAN), a necrotizing vasculitis of small- and medium-sized muscular arteries involving all organ systems.

A significant percentage of patients with PAN have hepatitis B antigenemia.

They also have circulating immune complexes containing hepatitis B antigen.

Hepatitis B antigen, IgM, and complement can be demonstrated in blood vessel walls. P-ANCA (perinuclear-anti nuclear cytoplasmic antibody) is a marker for polyarteritis nodosa.

Hepatitis A is not associated with a vasculitis. Hepatitis C accounts for 50-70% of chronic hepatitis. Chronic hepatitis C can be associated with immune-complex mediated extrahepatic complications, but is less common than hepatitis B.

Hepatitis C does have a significant association with essential mixed cryoglobulinemia, which presents with glomerulonephritis, arthralgias, hepatosplenomegaly, and lymphadenopathy, in addition to a vasculitis. However, there is no association with p-ANCA.

Hepatitis D requires that the patient be co-infected with hepatitis B. As such, it does not independently cause disease, but it can produce a worsening of the liver disease.


Q. 2

C- ANCA positivity indicates, antibody formed against which of the following?

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear Antibody

 D

Anti centromere Antibody

Q. 2

C- ANCA positivity indicates, antibody formed against which of the following?

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear Antibody

 D

Anti centromere Antibody

Ans. A

Explanation:

C-ANCAs (Cytoplasmic anti-neutrophil cytoplasmic antibodies), are a type of autoantibody formed against proteinase 3.

P-ANCA (Perinuclearanti-neutrophil cytoplasmic antibodies) are antibodies directed against myeloperoxidase.

Ref: Harrison’s Principles of Internal Medicine, 16th Edition, Page 2002; Thurlbeck’s Pathology Of The Lung, William M. Thurlbeck, 3rd Edition, Page 372; Rheumatology Secrets, Sterling G. West, 2nd Edition, Page 226.

 


Q. 3

All are TRUE about Antineutrophil Cytoplasmic Antibodies, EXCEPT:

 A

Proteinase-3, in neutrophil azurophilic granules, is the major cANCA antigen

 B

The major target for pANCA is the enzyme myeloperoxidase

 C

A pANCA pattern of staining has been associated with nonvasculitic entities such as rheumatic and nonrheumatic autoimmune diseases

 D

There are three major categories of ANCA

Q. 3

All are TRUE about Antineutrophil Cytoplasmic Antibodies, EXCEPT:

 A

Proteinase-3, in neutrophil azurophilic granules, is the major cANCA antigen

 B

The major target for pANCA is the enzyme myeloperoxidase

 C

A pANCA pattern of staining has been associated with nonvasculitic entities such as rheumatic and nonrheumatic autoimmune diseases

 D

There are three major categories of ANCA

Ans. D

Explanation:

There are two major categories of ANCA. Other statements are true.

 
Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2786

Quiz In Between


Q. 4

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Q. 4

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Ans. A

Explanation:

Ans. is ‘a’ i.e., cANCA

Antineutrophil cytoplasmic antibodies (ANCA)

o ANCA are heterogenous group of autoantibodies directed against antigens which are found within the primary granules of neutrophil and in the lysosomes of monocytes and in EC’s.

o The description of these autoantibodies is based on the immunofluorescence pattern of staining of ethanol fixed neutrophils. With immunofluorescence two principal patterns are recognized:

1.  Cytoplasmic (c-ANCA):

o This shows cytoplasmic localization of the staining and the target antigen for this type of autoantibody is — proteinase-3 (PR3), a neutrophil granule constituent.

2.  Perinuclear staining (p-ANCA)

o This shows perinuclear staining and the target antigen here is myeloperoxide (MPO).

Remember that either of these antibodies may occur in a patient with ANCA associated small vessel vasculitis but

(i) cANCA  Typically found in

  • Wegener’s granulomatosis

(ii) pANCA  Typically found in

  • Microscopic polyangiitis

o Churg-Strauss syndrome

o Idiopathic crescentic glomerulonephritis

o Goodpasteur’s syndrome.

o pANCA’s are also associated with certain non-vasculitic entities such as certain rheumatic and nonrheumatic autoimmune diseases, Inflammatory bowel diseases, certain drugs, • infections such as endocarditis and • bacterial airway infection in patients with cystic fibrosis.


Q. 5

pANCA is sensitive and specified for-

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Q. 5

pANCA is sensitive and specified for-

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., Idiopathic crecsentric glomerulonephritis


Q. 6

ANCA positive vasculitis

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

All

Q. 6

ANCA positive vasculitis

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

All

Ans. C

Explanation:

Ans. is ‘c’ i.e., Wegener’s granulomatosis

Quiz In Between


Q. 7

ANCA positive vasculitis are –

 A

Churg-strauss syndrome

 B

Polyarteritis nodosa

 C

Wegener granulomatosis

 D

a and c

Q. 7

ANCA positive vasculitis are –

 A

Churg-strauss syndrome

 B

Polyarteritis nodosa

 C

Wegener granulomatosis

 D

a and c

Ans. D

Explanation:

Ans. is ‘a’ i.e., Churg-strauss syndrome; ‘c’ i.e., Wegener granulomatosis


Q. 8

ANCA positive is –

 A

Good pasture syndrome

 B

Wegner’s granulomatosis

 C

Sjogren syndrome

 D

a and b

Q. 8

ANCA positive is –

 A

Good pasture syndrome

 B

Wegner’s granulomatosis

 C

Sjogren syndrome

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e., Good pasture syndrome; ‘b’ i.e., Wegner’s granulomatosis

Amongst the given options two are associated with ANCA: Wegner’s granulomatosis is positive for cANCA and goodpasture syndrome is positive for pANCA.


Q. 9

A patient presents with respiratory symptoms i.e. cough, hemoptysis and glomerulonephritis. His C-­ANCA levles in serum were found to be raised. The most likely diagnosis is –

 A

Goodpasteur’s syndrome

 B

Classic polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Kawasaki syndrome

Q. 9

A patient presents with respiratory symptoms i.e. cough, hemoptysis and glomerulonephritis. His C-­ANCA levles in serum were found to be raised. The most likely diagnosis is –

 A

Goodpasteur’s syndrome

 B

Classic polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Kawasaki syndrome

Ans. C

Explanation:

Ans. is ‘c’ i.e., Wegener’s granulomatosis

o Renal and pulmonary symptoms with positive c-ANCA suggest the diagnosis of wegener’s glomerulonephritis (see above explanation).

o Clinical syndrome of wegener granulomatosis may be very similar to classical PAN or microscopic polyangitis. Following facts will help you to differentiate wegner granulomatosis from other Two ‑

Wegeners granulomatosis Vs classical PAN

o Wegener’s granulomatosis involves small vessels (arteriole, venule, capillary), while PAN involves medium size vessels.

o Wegener’s granulomatosis is associated with c-ANCA, while classical PAN is not associated with ANCA. o In wegener’s granulomatosis there is lung involvement, but not in classical PAN.

o In wegener’s granulomatosis there is glomerulonephritis (hematuria) without hypertension, while in classical PAN there is hypertension without glomerulonephritis.

o In wegener’s granulomatosis, there is granuloma formation, while it is absent in classical PAN. o Classical PAN shows microaneurysms which are absent in wegener’s granulomatosis.

Wegner’s granulomatosis Vs microscopic polyangitis

o Wegener’s granulomatosis is associated with c-ANCA, while MPA is associated with p-ANCA. o Granuloma formation occurs in wegener’s granulomatosis, not in MPA.

o Lung cavities and nodules are seen in wegener’s granulomatosis, but not in MPA.

Quiz In Between


Q. 10

Which of the following condition(s) are associated with raised ANCA except –

 A

Wegener’s granulomatosis

 B

Polyarteritis Nodosa

 C

Microscopic Polyangitis

 D

Churg-Strauss syndrome

Q. 10

Which of the following condition(s) are associated with raised ANCA except –

 A

Wegener’s granulomatosis

 B

Polyarteritis Nodosa

 C

Microscopic Polyangitis

 D

Churg-Strauss syndrome

Ans. B

Explanation:

Ans. is ‘b’ i.e., Polyarteritis Nodosa


Q. 11

All are true about ANCA associated crescentic glomerulonephritis, except –

 A

Seen in Wegner’s granulomatosis

 B

Seen in microscopic polyangitis

 C

Seen in Henoch Schonlein purpura

 D

Is pauci-immue in nature

Q. 11

All are true about ANCA associated crescentic glomerulonephritis, except –

 A

Seen in Wegner’s granulomatosis

 B

Seen in microscopic polyangitis

 C

Seen in Henoch Schonlein purpura

 D

Is pauci-immue in nature

Ans. C

Explanation:

Ans. is ‘c’ i.e., Seen in Henoch Schonlein purpura


Q. 12

A 20 year old male presents with mucus and repeated gastrointestinal bleeding. Patient is positive for ANCA. The most likely diagnosis is:

 A

Ulcerative colitis

 B

Crohn’s disease

 C

Radiation colitis

 D

Ischemic bowel disease

Q. 12

A 20 year old male presents with mucus and repeated gastrointestinal bleeding. Patient is positive for ANCA. The most likely diagnosis is:

 A

Ulcerative colitis

 B

Crohn’s disease

 C

Radiation colitis

 D

Ischemic bowel disease

Ans. A

Explanation:

Answer is A (Ulcerative Colitis)

Presence of mucus and blood in stool along with positive ANCA antibodies suggests a diagnosis of ulcerative colitis

Different Clinical, Endoscopic and Radiographic Features

 Clinical

Ulcerative Colitis

Crohn’s Disease

 

Gross blood in stool

Yes

Occasionally

Occasionally

Mucus

Yes

Systemic symptoms

Occasionally

Frequently

Pain

Occasionally

Frequently.

Abdominal mass

Rarely

Yes

Significant perineal disease

No

Frequently

Fistulas

No

Yes

Small-intestinal obstruction

No

Frequently

Colonic obstruction

Rarely

Frequently

Response to antibiotics

No

Yes

Recurrence after surgery

No

Yes

ANCA-positive

Frequently

Rarely

ASCA —positive

Rarely

Frequently

Endoscopic

Rectal sparing

Rarely

Frequently

Continuous disease

Yes

Occasionally

Cobblestoning

No

Yes

Granuloma on biopsy

No

Occasionally

Radiographic

Small bowel significantly abnormal

No

Yes

Abnormal terminal ileum

Occasionally

Yes

Segmental colitis

No

Yes

Asymmetric colitis

No

Yes

Stricture

Occasionally

Frequently

Epidemiology of 1BD

Age of onset

15-30 and 60-80

15-30 & 60-80

Male : Female ratio

1:1

“4.5 : 1

Smoking

May prevent disease

May cause disease

Oral contraceptives

No increased risk

Odds ratio 1.4

Appendectomy

Protective

Not protective

Monozygotic twins

6% concordance

58% concordance

Dizygotic twins

0% concordance

4% concordance

Quiz In Between


Q. 13

A patient presents with hemoptysis and hematuria few weeks after a respiratory tract infection.

ANCA antibodies are present.

Likely diagnosis is:

 A

Goodpasture’s syndrome

 B

IgA Nephropathy

 C

Nephrotic syndrome

 D

PSGN

Q. 13

A patient presents with hemoptysis and hematuria few weeks after a respiratory tract infection.

ANCA antibodies are present.

Likely diagnosis is:

 A

Goodpasture’s syndrome

 B

IgA Nephropathy

 C

Nephrotic syndrome

 D

PSGN

Ans. A

Explanation:

Answer is A (Goodpasture’s syndrome)

  • Association of hematuria and hemoptysis suggest a diagnosis of Goodpasture’s syndrome.

Goodpasture’s syndrome and ANCA

  1. ANCA is typically negative in Goodpastures syndrome and it is classified as an ANCA negative vasculitis

Nevertheless

  • ANCA seropositivity may occur in upto 30 percent of patients with Goodpasture’s.syndrome.
  • These patients with ANCA have subtle signs of a systemic vasculitis and represent an overlap between goodpastures and either Wegener’s granulomatosis (WG) or Microscopic polyangitis(MPA)-Current Diagnosis and Treatment in Pulmonary Medicine

 


Q. 14

ANCA is found in all of the following Except:

 A

A. Wegner’s granulomatosis

 B

Churg-Strauss disease

 C

Microscopic polyangitis

 D

Takayasu arteritis

Q. 14

ANCA is found in all of the following Except:

 A

A. Wegner’s granulomatosis

 B

Churg-Strauss disease

 C

Microscopic polyangitis

 D

Takayasu arteritis

Ans. D

Explanation:

Answer is D (Takayasu Arteritis):

Takayasu Arteritis is not associated with ANCA

Note:

If p-ANCA is not against Myeloperoxidase (or CANCA is not against Proteinase-3), look for causes other than Vasculitis for the positive ANCA.


Q. 15

ANCA positive vasculitis include all of the following Except:

 A

Wegner’s granulomatosis

 B

Churg strauss syndrome

 C

Microscopic PAN

 D

Good pasture’s syndrome

Q. 15

ANCA positive vasculitis include all of the following Except:

 A

Wegner’s granulomatosis

 B

Churg strauss syndrome

 C

Microscopic PAN

 D

Good pasture’s syndrome

Ans. D

Explanation:

Answer is D (Good pasture’s syndrome):

Goodpasture’s syndrome is not classified as an ANCA positive vasculitis.

Quiz In Between


Q. 16

ANCA is NOT associated with which of the following disease:

 A

Wegener’s Granulomatosis

 B

Henoch Schonlein Purpura

 C

Microscopic Polyangitis

 D

Churg Strauss syndrome

Q. 16

ANCA is NOT associated with which of the following disease:

 A

Wegener’s Granulomatosis

 B

Henoch Schonlein Purpura

 C

Microscopic Polyangitis

 D

Churg Strauss syndrome

Ans. B

Explanation:

Answer is B (Henoch Schonlein Purpura):

Henoch Schonlein Purpura is an ANCA Negative Vasculitis


Q. 17

ANCA is NOT associated with which of the following diseases :

 A

Wegener’s granulomatosis

 B

Henoch schonlein purpura

 C

Microscopic PAN

 D

Churg Strauss syndrome

Q. 17

ANCA is NOT associated with which of the following diseases :

 A

Wegener’s granulomatosis

 B

Henoch schonlein purpura

 C

Microscopic PAN

 D

Churg Strauss syndrome

Ans. B

Explanation:

Answer is B (H.S. Purpura) :

H.S. purpura is not associated with any antinuclear cytoplasmic antibody (ANCA). It is an example of ANCA negative vasculitis.

  • ANCA (Antineutrophilic cytoplasmic Antibodies) arc Antibodies directed against certain proteins in cytoplasmic granules of Neutrophil & monocytes.
  • These are two major categories of ANCA based on different targets for the antibodies.

ANCA (Antineutrophic cytoplasmic antibodies):

ANCA is of 2 types

C-ANCA

(Cytoplasmic proteinase 3q is the target antigen)

Wegeners Granulomatosisq (90-95%)

P-ANCA

(perinuclear myeloperoxidaseQ is the major target antigen)

  • Microscopic PAN (microscopic polyangitis)
  • Churg-Strauss syndrome
  • Crescenteric glomerulonephritise
  • Good pasteur’s syndrome

Q. 18

CANCA Positivity is specific for:

 A

Polyarteritis Nodosa

 B

Wegener’s granulomatosis

 C

Henoch shontein purpura

 D

Churg strouss syndrome

Q. 18

CANCA Positivity is specific for:

 A

Polyarteritis Nodosa

 B

Wegener’s granulomatosis

 C

Henoch shontein purpura

 D

Churg strouss syndrome

Ans. B

Explanation:

Answer is B (Wegener’s granulomatosis):

CANCA refers to Cytoplasmic Antineutrophilic cytoplasmic antibodies and its presence is highly specific for Wegener’s granulomatosis

Quiz In Between


Q. 19

c-ANCA is characteristic for :

 A

Polyarteritis Nodosa

 B

R.P.G.N.

 C

Henoch’s Schonlein Purpura

 D

Wegeners granulomatosis

Q. 19

c-ANCA is characteristic for :

 A

Polyarteritis Nodosa

 B

R.P.G.N.

 C

Henoch’s Schonlein Purpura

 D

Wegeners granulomatosis

Ans. D

Explanation:

Answer is D (Wegener’s granulomatosis) :

Antineutrophilic cytoplasmic antibody directed against cytoplasmic proteinase-3 (cANCA) is found in 90-95% if patients with Wegener’s granulomatosis.


Q. 20

C-ANCA is associated with:

 A

Wegener’s Granulomatosis

 B

Microscopic Polyangitis

 C

Churg- Strauss Syndrome

 D

Polyarteritis Nodosa (PAN)

Q. 20

C-ANCA is associated with:

 A

Wegener’s Granulomatosis

 B

Microscopic Polyangitis

 C

Churg- Strauss Syndrome

 D

Polyarteritis Nodosa (PAN)

Ans. A

Explanation:

Answer is A (Wegener’s Granulomatosis):

cANCA refers to Cytoplasmic Antineutrophilic Cytoplasmic Antibodies and its presence is highly specific for Wegener’s Granulomatosis. Antineutrophilic Cytoplasmic Antibody directed against cytoplasmic. proteinase-3 (cANCA) is found in 90-95% if patients with Wegener’s Granulomatosis.


Q. 21

c-ANCA positivity indicates, antibody formed against:

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear antibody

 D

Anti centromere antibody

Q. 21

c-ANCA positivity indicates, antibody formed against:

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear antibody

 D

Anti centromere antibody

Ans. A

Explanation:

Answer is A (Proteinase 3):

Proteinase – 3 (a 29 – kDA serine proteinase) present in neutrophil azurophilic granules is the major c-ANCA antigen.

Major c-ANCA antigen        Proteinase 3 (PR-3)

Major p – ANCA antigen    Myeloperoxidase (MPO)

Quiz In Between


Q. 22

p-ANCA is characteristic for:

 A

PAN

 B

Microscopic polyangitis

 C

Wegener’s granulomatosis

 D

Henoch-Schonlein purpura

Q. 22

p-ANCA is characteristic for:

 A

PAN

 B

Microscopic polyangitis

 C

Wegener’s granulomatosis

 D

Henoch-Schonlein purpura

Ans. B

Explanation:

Answer is B (Microscopic polyangitis) :

PAN can broadly be classified into

Classic PAN
(now referred to an PAN)

  • Classified as Medium Vessel Vasculitis
  • Necrotizing inflammation of small & medium sized arteries.
  • No involvement of capillaries & venules
  • Pulmonary vasculature is not involved.
  • ANCA mostly negative (Rarely p-ANCA may be seen)

Microscopic PAN
(now called microscopic polyangitis)

  • Classified as small vessel vasculitis
  • Necrotizing inflammation of small & medium sized arteries.
  • Capillaries & venules are involved
  • Pulmonary vasculature is involved.
  • ANCA positive (p-ANCA)



Q. 23

All of the following are true regarding Wegener’s granulomatosis except: 

September 2008

 A

Affects large size blood vessels

 B

May affect lungs and kidneys

 C

Necrotizing granuloma may be seen on microscopy

 D

PR3-ANCAs are present in upto 95% of cases

Q. 23

All of the following are true regarding Wegener’s granulomatosis except: 

September 2008

 A

Affects large size blood vessels

 B

May affect lungs and kidneys

 C

Necrotizing granuloma may be seen on microscopy

 D

PR3-ANCAs are present in upto 95% of cases

Ans. A

Explanation:

Ans. A: Affects large size blood vessels

Wegener’s granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs.

Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression.

Wegener’s granulomatosis is part of a larger group of vasculitic syndromes, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels.

Apart from Wegener’s, this category includes Churg-Strauss syndrome and microscopic polyangiitis.

Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils are associated with Wegener’s (upto 95% of cases).

If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from.

On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation on microscopy. These granulomas are the main reason for the appellation of “Wegener’s granulomatosis”, although it is not an essential feature.


Q. 24

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Q. 24

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Ans. A

Explanation:

Ans. is ‘a’ i.e., cANCA

Quiz In Between


Q. 25

pANCA is sensitive and specified for‑

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Q. 25

pANCA is sensitive and specified for‑

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., Idiopathic cresentic glomerulonephritis


Q. 26

CANCA is associated with ‑

 A

Wegener’s granulomatosis

 B

Microscopic polyangitis

 C

Chrug strauss syndrome

 D

Good pasture syndrome

Q. 26

CANCA is associated with ‑

 A

Wegener’s granulomatosis

 B

Microscopic polyangitis

 C

Chrug strauss syndrome

 D

Good pasture syndrome

Ans. A

Explanation:

Ans. is ‘a’ i.e., Wegener’s granulomatosis

Quiz In Between



Langerhan’c cell Histiocytosis

LANGERHANS CELL HISTIOCYTOSIS (LCH)

Q. 1

Langerhans cell histiocytosis true is all except ‑

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Gonadal involvement occurs

Q. 1

Langerhans cell histiocytosis true is all except ‑

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Gonadal involvement occurs

Ans. D

Explanation:

Gonadal involvement occurs [Ref : Bobbin’s 7thle p. 701, 702]

The working group of the histiocyte society has divided histiocytic disorders into 3 groups I. Dendritic cell histiocytosis

  1. Marcophage related disorders
  2. Malignant histiocytosis
  • LCH belongs in group 1 and encompasse a number of diseases

The clinical spectrum of LCH includes 😕

  • Eosinophilic granulomas (indolent and chronic lesion of bone and other organs.
  • Hand-Schuller Christian disease (intermediate fonn, multifocal chronic involvement)
  • Letterer siewe disease

acute fulininant disseminated disease

Clainical features

Age

  • LCH affects patients from neonatal period to childhood
  • The age at onset varies according to the variant of LCH as follows

– letterer siewe disease occurs predominantly in children younger than 2 years – Hand schuller Christian disease in children (2-10 years)

– Eosinophilic granuloma (5-15 years)

The clinical features vary according to the type of LCH

Chronic unifocal LCH (eosirzophilic granuloma of bone)

  • Solitary calvarial lesion in young adults
  • Other sites involved are

– Vertebra – Rib

– Mandible – Femur

– Ilium

– Scapula

  • Lesions are usually asymptomatic but may produce following symptoms due to involvement of

bone

– Otitis media – Proptosis – Loose teeth

– Pituitary dysfunction

– Spontaneous .fracture

Classic multifocal LCH (Hand schulller Christian disease) Presents with classic triad of :-

  • Diabetes insipidus
  • Exophthalmos
  • Bony defect

Acute disseminated (LCH) litterer siewe disease

  • Cutaneous abnormalities

Present in 80% of patients – Frequently the first sign – Involve, scalp, .face, trunk, buttock

“Mimicks seborrhic dermatitis”

  • Fever
  • Anemia
  • Thrombocytopenia
  • Pulmonary infiltrates
  • Spleen and liver involvement .• Enlargement of lymph nodes

Laneherhans cells demonstrate following characteristics 😕

  • Abundant often vacuolated cytoplasm and vesicular nuclei
  • Birbeck granules

Characteristic of langherhans cells

– “Tennis racket appearance”

– Contain the protein langerin

– Typically express

– HLA — DR

– S — 100

– CD/a

Management of Histiocytosis

  • The management is based on disease severity

Single system disease

Solitary bone lesion

  • Curettage or excision
  • Intralesional steroid injection
  • Biphosphonates
  • Indomethacin (polyostic lesions)

Localized skin lesion

  • Topical steroids
  • Topical nitrogen mustards
  • Acitretin
  • Psoralen plus ultraviolet A (PUVA)

Single lymph node infiltration

  • Excision is the treatment of choice

Multisystem disease

  • Systemic chemotherapy

Combination of cytotoxic drugs and systemic steroids is generally effective.

Drugs used are:-

– Vinblastine

– Etoposide

– Prednisolone

– Mercaptopurine

Treatment of refractory disease

  • Cladirabine (2 chlorodeoxyadenosine 2 – CDA)
  • Cytosine arabinoside (Ara – c)
  • Bone marrow transplant in patients with very poor prognosis

Q. 2

A 3 year old female child presented with skinpapules. Which of the following is a marker of Langerhan’s cell histiocytosis?

 A

CD la

 B

CD 3

 C

CD 68

 D

CD 57

Q. 2

A 3 year old female child presented with skinpapules. Which of the following is a marker of Langerhan’s cell histiocytosis?

 A

CD la

 B

CD 3

 C

CD 68

 D

CD 57

Ans. A

Explanation:

CD la [Ref. Robbins Th/e p. 701, 702]

  • Histiocytosis is a disorder of the mononuclear phagocytic system.
  • Mononuclear phagocytic system consists of monoblasts, promonocytes, monocytes and tissue macrophages,
  • Macrophages are transformed monocytes i.e. monocytes present in the tissues. Moncytes emigrate continuously from peripheral blood into the tissues.

Histiocyte

  • The term histiocyte is synonymous with macrophage.
  • In diseases of mononuclear phagocytic system the terms histiocyte and histiocytosis are used.

Langern cell histiocytosis

  • Langerhans cell is macrophage and plays important role in immunity.
  • It processes antigen and present it to T cells.
  • Langerhans cells histiocytosis (LCH) is a clonal histiocytosis and the lesions are characterized by the presence of Langerhans cells, eosinophils and Lymphocytes in a variable number.

The various features of langerhans cells are :

  • Presence of Birbeck granules (E/M – HX bodies)
  • Presence of enzymes like

– a Naphthyl acetate esterase

a Naplahyl butyrate esterase

– Acid phosphatase

  • Markers like

– S-I00 positivity

– CD-la positivity’

– HCA-DR expression


Q. 3

Which of the following conditions produce a seborrheic dermatitis like lesions in an infant?

 A

Langerhan cell histiocytosis

 B

Juvenile xanthogranuloma

 C

Multicentric histiocytosis

 D

Erdheim Chester disease

Q. 3

Which of the following conditions produce a seborrheic dermatitis like lesions in an infant?

 A

Langerhan cell histiocytosis

 B

Juvenile xanthogranuloma

 C

Multicentric histiocytosis

 D

Erdheim Chester disease

Ans. A

Explanation:

All the above mentioned optionscare disorders of histiocytes.

In langerhan cell histiocytosis, bone, lymph node and skin are the most frequently affected sytems.

The most characteristic presentation is with scalp involvement.

which is erythematous with greasy scales, looking very like seborrheic dermatitis.

S100 Paraffin, peanut agglutinin, Placental alkaline phosphatase are the common used immunological markers.

Quiz In Between


Q. 4

Which of the following is a marker for Langerhans cell histiocytosis?

 A

CD 1a

 B

CD 10

 C

CD 30

 D

CD 56

Q. 4

Which of the following is a marker for Langerhans cell histiocytosis?

 A

CD 1a

 B

CD 10

 C

CD 30

 D

CD 56

Ans. A

Explanation:

The proliferating Langerhans cells in Langerhans cell histiocytosis are HLA-DR positive and express the CD1 antigen.
 
Ref: Robin’s Basic Pathology, 7th Edition, Page 441.

Q. 5

Birbeck granules are seen in which of the following?

 A

Eosinophils

 B

Langerhans cells

 C

Reed–Sternberg cells

 D

Anitschkow cells

Q. 5

Birbeck granules are seen in which of the following?

 A

Eosinophils

 B

Langerhans cells

 C

Reed–Sternberg cells

 D

Anitschkow cells

Ans. B

Explanation:

Langerhans cell inclusions are called Birbeck bodies. The Birbeck bodies have a characteristic racquet shape and are found only in Langerhans cells, making them a specific diagnostic marker for Langerhans cell histiocytosis. The current standard for identifying these cells is staining with an antibody to CD207, which is the protein, langerin, of the Birbeck granules, which is seen only by electron microscopy. Anti-CD1a staining of cells is also considered diagnostic of LCH.
 
LCH usually presents with a skin rash or painful bone lesion. Systemic symptoms of fever, weight loss, diarrhea, edema, dyspnea, polydipsia, and polyuria relate to specific organ involvement.
 
Ref: Lichtman M.A., Shafer J.A., Felgar R.E., Wang N. (2007). C. Histiocytic and Myeloid Dendritic Leukemias. In M.A. Lichtman, J.A. Shafer, R.E. Felgar, N. Wang (Eds), Lichtman’s Atlas of Hematology.

Q. 6

An 8 year old child diagnosed with Langerhans cell histiocytosis. Bone involvement is seen. All of the following are true about Langerhans cell histiocytosis, EXCEPT:

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Enophthalmos present

Q. 6

An 8 year old child diagnosed with Langerhans cell histiocytosis. Bone involvement is seen. All of the following are true about Langerhans cell histiocytosis, EXCEPT:

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Enophthalmos present

Ans. D

Explanation:

Exophthalmos is present in only 10% to 30% of cases of Langerhans cell histiocytosis.

It may be unilateral or bilateral and is due to retroocular bone infiltration by Langerhans cells.

Langerhans cell histiocytosis is a disease of childhood. Letterer–Siwe disease is the prototype of the acute, disseminated, multisystemic form that usually appears in infants or newborns. The disease can occur in individuals of any age and can also be congenital, but is most common in children aged 1–3 years. 
 
Ref: Gelmetti C. (2012). Chapter 147. Cutaneous Langerhans Cell Histiocytosis. In L.A. Goldsmith, S.I. Katz, B.A. Gilchrest, A.S. Paller, D.J. Leffell, N.A. Dallas (Eds), Fitzpatrick’s Dermatology in General Medicine, 8e.

Quiz In Between


Q. 7

The nuclei of langerhan’s giants cells –

 A

Are present at the centre

 B

Form a network

 C

Are arranged radially

 D

Are arranged around the periphery

Q. 7

The nuclei of langerhan’s giants cells –

 A

Are present at the centre

 B

Form a network

 C

Are arranged radially

 D

Are arranged around the periphery

Ans. D

Explanation:

Ans. is ‘d’ i.e., Are arranged around periphery


Q. 8

Localised langerhans cells histiocytosis affecting head & neck is –

 A

Letterer-siwe disease

 B

Pulmonary langerhans cell histiocytosis

 C

Hand-schuller-christian disease

 D

Eosinophilic granuloma

Q. 8

Localised langerhans cells histiocytosis affecting head & neck is –

 A

Letterer-siwe disease

 B

Pulmonary langerhans cell histiocytosis

 C

Hand-schuller-christian disease

 D

Eosinophilic granuloma

Ans. D

Explanation:

Ans. is ‘d’ i.e., Eosinophilic granuloma

Clinical manifestations of Langerhans cell histiocytosis (Histocvtosis-X)

Letterer Siwe disease (multifocal, multisystem LCH)

o Most frequently present before 2 years of age.

o Characterized by involvement of multiple system.

o Most common presentation is cutaneous lesions resembling seborrheic dermatitis.

o Others are hepatosplenomegaly, lymphadenopathy pulmonary lesions and destructive bone lesions. o Extensive bone marrow infiltration leads to pancytopenia.

Eosinophilic granuloma (Unifocal and multifocal unisystem LCH)

o Involvement is restricted to a single system i.e., skeletal system which may be unifocal or multifical. o Most commonly effected bones are skull, vertebrae, ribs, clavicle, and femur.

3. Hand-Schuller-Christian disease

o Characterized by triad of claviral bone defects, diabetes insipidus, and exophthalmos.


Q. 9

A 2 year old child presents with scattered lesions in the skull. Biopsy revealed Langerhans giant cells. The most commonly associated is marker with this condition will be –

 A

CD la

 B

CD 57

 C

CD 3

 D

CD 68

Q. 9

A 2 year old child presents with scattered lesions in the skull. Biopsy revealed Langerhans giant cells. The most commonly associated is marker with this condition will be –

 A

CD la

 B

CD 57

 C

CD 3

 D

CD 68

Ans. A

Explanation:

Ans. is ‘a’ i.e., CD1a

o The presence of Birbeck granules in the cytoplasm of tumor cells is characteristic of Langerhans cell histiocytosis. o As Birbeck granules are not seen in all tumor cells by election microscopy the detection of S-100 and CD1a expression by immunohistochemical techniques aids in the diagnosis.

o Other immunohistochemical markers of Langerhans cell histiocytosis are HLA-DR, Langerin (CD 207), and CD 68.

Quiz In Between


Q. 10

Langerhans cell histiocytosis true is all except

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Gonadal involvement occurs

Q. 10

Langerhans cell histiocytosis true is all except

 A

Peak incidence less than 3 years of age

 B

Radiosensitive

 C

Diffuse form is known as litter-sewe-disease

 D

Gonadal involvement occurs

Ans. D

Explanation:

Ans. is ‘d’ i.e., Gonadal involvement occurs

Peak incidence of langerhans cell histiocytosis is seen in children less than 5 years.

o Multifocal, multisystem langerhans cell histiocytosis is known as letter-sewe disease.

o Low dose radiotherapy is effective in single system disease.

Also know

Childhood histiocvtosis

o Three classes of childhood histiocytosis are recognized based on histopathological findings

Now histiocytosis is classified into :-

(i)         Histiocytosis I

(ii)       Histiocytosis II

(iii)    Histiocytosis III

o The most well known childhood histiocytosis previously known as histiocytosis X constitutes class I histiocytosis and includes the following three :‑

o Letterer – Siwe disease

(Acute fielminant disseminated disease)

o Eosinophilic granuloma

(Solitary or few indolent lesions of bone)

o Hand schuller christian disease

(Intermediate form characterized by multifocal chronic involvement and classically presents as triad of diabetes insipidus proptosis and lytic bone lesin)

o Class I histiocytosis is also known as langherhan’s cell histiocytosis.


Q. 11

X-bodies called Birbeck granules are characteristically seen in –

 A

Granulomatous vasculitis

 B

Pulmonary alveolar proteinosis

 C

Langerhan’s cells granulomatosis

 D

Idiopathic pulmonary alveolar proteinosis

Q. 11

X-bodies called Birbeck granules are characteristically seen in –

 A

Granulomatous vasculitis

 B

Pulmonary alveolar proteinosis

 C

Langerhan’s cells granulomatosis

 D

Idiopathic pulmonary alveolar proteinosis

Ans. C

Explanation:

Ans.is ‘c’ i.e., Langerhan’s cells granulomatosis


Q. 12

The histologic hallmark of langerhan cells is‑

 A

Dendritic cell processes

 B

Giant mitochondria

 C

Birbeck granules

 D

Eosinophilic granules

Q. 12

The histologic hallmark of langerhan cells is‑

 A

Dendritic cell processes

 B

Giant mitochondria

 C

Birbeck granules

 D

Eosinophilic granules

Ans. C

Explanation:

Ans. is ‘c’ i.e., Birbeck granules

Quiz In Between


Q. 13

A 2-year-old child comes with discharge, seborrheic dermatitis, polyuria and hepatosplenomegaly. Which of the following is the most likely diagnosis

 A

Leukemia

 B

Lymphoma

 C

Langerhan’s cell histiocytosis

 D

Germ cell tumour

Q. 13

A 2-year-old child comes with discharge, seborrheic dermatitis, polyuria and hepatosplenomegaly. Which of the following is the most likely diagnosis

 A

Leukemia

 B

Lymphoma

 C

Langerhan’s cell histiocytosis

 D

Germ cell tumour

Ans. C

Explanation:

Ans. is `c’ i.e., Lagherhan’s cell histocytosis

o Seborrhic dermatitis, polyuria, hepatosplenomegaly and discharge suggests the diagnosis of Langerhan’s cell histiocytosis.

Clinical manifestations of langerhans cell histiocytosis

o It has diverse clinical features. These are

1. Skeletal involvement

            It is seen in 80% of cases (75% cases present with solitary lytic lesion)

            Skull is the most common site of involvement (Presents with solitary lytic lesion)

            Involvement of spine leads to collapse of vertebral body and compression of spinal cord.

            In flat long bones the lesion is osteolytic

            Involement of mastoid bones leads to chronically infected ears.

2. Skin involement

            It is seen in 50% of patients.

            It usually manifests as seborrhic dermatitis of scalp or diaper region.

3. Hepatosplenomegaly –

a Seen in 20% of patients

q                 Manifests as jaundice and ascites.

  1. Localized or disseminated lymphadenopathy
  2. Exophthalmos –

            It is often B/L and is caused by retrorbital accumulation of granulomatous tissue.

6. Pulmonary infiltrates –

 Seen in 10-15% of patients

              The lesion consists of nodular infiltrates or diffitse fibrosis.

7. Endocrine Tlypopituitarism, Hypothyroidism, Diabetes insipidus

Systemic manifestations –

              Usually seen in patients affected severly

              These include fever, weight loss, malaise, irritability

o Remember these two uncommon but serious manifestations of LCH

1. Cirrhosis d/t hepatic involvement

2. Ataxia and dysarthria d/t CNS involvement


Q. 14

Baby with recurrent infection of ear & discharge & seborrheic dermatitis with hepatosplenomegaly with cystic skull lesions. Diagnosis is

 A

Hemophagocytic lymphohistiocytosis

 B

Langerhans cell histiocytosis

 C

ALL

 D

Multiple myeloma

Q. 14

Baby with recurrent infection of ear & discharge & seborrheic dermatitis with hepatosplenomegaly with cystic skull lesions. Diagnosis is

 A

Hemophagocytic lymphohistiocytosis

 B

Langerhans cell histiocytosis

 C

ALL

 D

Multiple myeloma

Ans. B

Explanation:

Ans. is ‘b’ i.e., Langerhans cell histiocytosis


Q. 15

True about Langerhan’s histocytosis

 A

Can be associated with diabetes insipidus

 B

X-ray shows pathognomonic osteosclerotic lesions

 C

Birbeck’s granules in langhan’s cell

 D

a and c

Q. 15

True about Langerhan’s histocytosis

 A

Can be associated with diabetes insipidus

 B

X-ray shows pathognomonic osteosclerotic lesions

 C

Birbeck’s granules in langhan’s cell

 D

a and c

Ans. D

Explanation:

Ans. is ‘a’ i.e., Can be associated with diabetes insipidus; ‘c’ i.e.,Birbeck’s granules in langhan’s cell

o Hand-Schuller-Christion disease (a type of histiocytosis) is characterized by triad of : –

i) Claviral bone defects                    ii) Diabetes insipidus                   iii) Exophthalmos

o There is proliferation of langerhans histiocytes (antigen presenting cells).

o LCH is associated with HLA-DR, S-100, CD la.

o Birbeck granules are present in the cytoplasm of tumor cells.

Quiz In Between


Q. 16

All are true about Langerhans’ histocytosis except?

 A

Common before 3 years of age

 B

Letterer Siwe disease is systemic manifestation

 C

Radio sensitive

 D

Testis is commonly involved

Q. 16

All are true about Langerhans’ histocytosis except?

 A

Common before 3 years of age

 B

Letterer Siwe disease is systemic manifestation

 C

Radio sensitive

 D

Testis is commonly involved

Ans. D

Explanation:

D. i.e. Testis is commonly invoved


Q. 17

Langerhan’s cells are:

 A

Phagocytic cells

 B

Antigen presenting cells

 C

Seen in auto immune conditions

 D

Seen in chronic infection

Q. 17

Langerhan’s cells are:

 A

Phagocytic cells

 B

Antigen presenting cells

 C

Seen in auto immune conditions

 D

Seen in chronic infection

Ans. B

Explanation:

Answer is B (Antigen presenting cells) :

Langerhan’s cells are characteristic Antigen presenting cells

  • Langerhan’s cells are clear dendritic Q cells situated among the cells of stratum spinosum (slun). Q
  • They are believed to be antigen processing cells. Q
  • They are S100 positive Q on immunohistochemical studies.
  • On electron microscopy: they lack melanosomesQ contain characteristic organelle, the Birbeck’s granule.Q

Q. 18

Which of the following is not a type of langerhans cell histiocytosis?

 A

Hand Schuller Christian disease

 B

Eosinophilic granuloma

 C

Letter-siwe syndrome

 D

Tones syndrome

Q. 18

Which of the following is not a type of langerhans cell histiocytosis?

 A

Hand Schuller Christian disease

 B

Eosinophilic granuloma

 C

Letter-siwe syndrome

 D

Tones syndrome

Ans. D

Explanation:

Ans. is `d.’ i.e., Torres syndrome

Langerhans cell histiocvtosis (Histiocytosis X)

  • Histiocytosis X is characterized by proliferation of Langerhans histiocytes (tissue macrophages).
  • It is Subdivided into three Categories:-
  • Letterer-siwe syndrome
  • Hand-Schuller-Christian disease
  • Eosinophilic granuloma.
  • Tumor cells in each are derived from dendritic cells and express S-100, CD la and HLA-DR.
  • The presence of Birbeck granules in the cytoplasm is characteristic.
  • Under electron microscope, Birbeck granules have a pentalaminar, rod like, tubular appearance and sometimes a dilated terminal end (tennis-rachet appearance).

Quiz In Between


Q. 19

Birbereck granules in cytoplasm are seen in‑

 A

Mast cells

 B

Langerhan’s cells

 C

Thrombocytes

 D

Myelocytes

Q. 19

Birbereck granules in cytoplasm are seen in‑

 A

Mast cells

 B

Langerhan’s cells

 C

Thrombocytes

 D

Myelocytes

Ans. B

Explanation:

Ans. is ‘b’ i.e., Langerhan’s cells


Q. 20

Birbeck granules in cytoplasm is seen in

 A

Langerhans cell histiocytosis

 B

Hodgkin’s lymphoma

 C

Non hodgkins lymphoma

 D

Gastrointestinal stromal tumor

Q. 20

Birbeck granules in cytoplasm is seen in

 A

Langerhans cell histiocytosis

 B

Hodgkin’s lymphoma

 C

Non hodgkins lymphoma

 D

Gastrointestinal stromal tumor

Ans. A

Explanation:

Ans. is ‘a’ i.e., Langerhans cell histiocytosis

Birbeck granules are characteristic of langerhans cell histiocytosis

Tumour cells in the langerhan’s cell histiocvtosis are derived from dendritic cells and express

  • S-100
  • CD1a
  • HLA-DR
  • These cells are characterized by the presence of birbeck granules in their cytoplasm unde rthe electron micro­scope, Birbeck granules have pentalaminar, rodlike tuular appearance and sometimes a dilated terminal end (tennis-racket appearance)

 

Quiz In Between



Tumor Protein 53

TUMOR PROTEIN 53 (TP53)

Q. 1

About p53 all are true except 

 A

Encodes 53k Da protein

 B

Located on Chr. 17

 C

Arrests cell cycle at GI phase

 D

Wild type p53 is associated with childhood tumors.

Q. 1

About p53 all are true except 

 A

Encodes 53k Da protein

 B

Located on Chr. 17

 C

Arrests cell cycle at GI phase

 D

Wild type p53 is associated with childhood tumors.

Ans. D

Explanation:

Wild type p53 is associated with childhood tumours [Ref: Bobbin’s 8th/e p. 290, 291, 292]

Wild type 53 is the normal (non mutated form of p53).

  • The normal p53 gene is a tumour suppressor gene that prevents the development of tumours.
  • When wild/normal p53 gene undergoes some mutation, it is called the mutant p53 gene.
  • Mutant p53 gene is associated with various human cancers.

– A little over 50% human tumours contain mutation in this gene.

– Homozygous loss of p53 occurs in virtually every type of cancer including carcinomas of the lung, colon and breast.

p53 Guardian of genome

  • p53 gene is located on chromosome 17Q.
  • It is a tumour suppressor gene.
  • “p53 acts as a molecular policemean that prevents the propagation of genetically damaged cell”.

p53 gene product i.e., p53 protein is a DNA binding protein in the nucleus, when called into action, it controls the transcription of several other genes.

  • The major functional activities of the p53 protein are: ?

Activation of temporary cell cycle arrest (quiescence)

p53 induces transcription of p21°, a CDK inhibitor.

– p21 inhibit cyclin D-CDK-4 e complex leading to arrest of cell cycle late in G1 phase. – This allows time for DNA repair (1.

DNA repair

p53 helps in DNA repair not only by allowing time for DNA repair but also by directly inducing the transcription of GADD 45Q (growth arrest and DNA damage).

GADD 45 encodes a protein that is involved in DNA repair.

– If DNA damage is repaired successfully, p53 activates MDM 2 which in turn causes degradation of p53. This MDM-2 induced degradation of p53 causes relieve in cell cycle block.

Triggering of programmed cell death

p53 directs the transcription of several pro-apoptotic genes such as BAX and PUMA (approved name BBC3) and induces apoptosis.

Induction of pennanent cell cycle arrest (quiescent)

p53 can also cause permanent cell cycle arrest.

It is characterized by specific changes in morphology and the exact mechanism is not known.


Q. 2

In a Teritary level Lab it is observed that chronic, increased exposure to ionizing radiation results in damage to cellular DNA. As a consequence, a protein is now absent that would arrest the cell in the G1 phase of the cell cycle. Subsequent to this, the cell is transformed to acquire the property of unregulated growth. The absent protein is most likely the product of which of the following genes?

 A RAS
 B VHL
 C p53
 D MYC
Q. 2

In a Teritary level Lab it is observed that chronic, increased exposure to ionizing radiation results in damage to cellular DNA. As a consequence, a protein is now absent that would arrest the cell in the G1 phase of the cell cycle. Subsequent to this, the cell is transformed to acquire the property of unregulated growth. The absent protein is most likely the product of which of the following genes?

 A RAS
 B VHL
 C p53
 D MYC
Ans. C

Explanation:

p53


Q. 3 False about p53 is:
 A It is present on chromosomes 17
 B It causes cell cycle arrest in G 1
 C 53 KDa
 D Non mutated wild p53 is asssociated with neoplasm in childhood
Q. 3 False about p53 is:
 A It is present on chromosomes 17
 B It causes cell cycle arrest in G 1
 C 53 KDa
 D Non mutated wild p53 is asssociated with neoplasm in childhood
Ans. D

Explanation:

Non mutated wild p53 is asssociated with neoplasm in childhood

Quiz In Between


Q. 4

What is the half-life of p53 protein in normal cells?

 A

20 minutes

 B

60 minutes

 C

12 hours

 D

1 day

Q. 4

What is the half-life of p53 protein in normal cells?

 A

20 minutes

 B

60 minutes

 C

12 hours

 D

1 day

Ans. A

Explanation:

In non stressed, healthy cells, p53 has a short half-life (20 minutes), because of its association with MDM2, a protein that targets it for destruction.
When the cell is stressed, for example by an assault on its DNA, p53 undergoes post-transcriptional modifications that release it from MDM2 and increase its half-life. Unshackled from MDM2, p53 also becomes activated as a transcription factor.
Ref: Robbins 8th edition Chapter 7.

 


Q. 5

Which of the following statements is not correct regarding p53 gene?

 A

Located on chromosome 17

 B

Arrests cell cycle at Gl phase

 C

Is a 53 kDa protein

 D

Wild type is associated with tumors

Q. 5

Which of the following statements is not correct regarding p53 gene?

 A

Located on chromosome 17

 B

Arrests cell cycle at Gl phase

 C

Is a 53 kDa protein

 D

Wild type is associated with tumors

Ans. D

Explanation:

Wild type of p53 gene refers to the Normal (Non-mutated) form of p53 gene.
The wild type of p53 gene (Normal/Non-mutated) is a tumor suppressor gene that maintains the genetic integrity of cells and prevents the development of tumors.
It is the mutated form of p53 gene (and not the wild type) that is associated with tumors.
 
Ref: Harrison’s Principles of Internal Medicine, 17th Edition, Pages 499, 500; Cancer: Principles and Practice of Oncology By De vita, 6th Edition, Page 19; Robbin’s Illustrated Pathology, 7th Edition, Page 302; Textbook of Pathology By Harsh Mohan, 5th Edition, Pages 2216-17

Q. 6

‘Policemen gene’ or ‘Guardian gene’ is the name given to?

 A

Myc

 B

Meu

 C

P53

 D

Abl

Q. 6

‘Policemen gene’ or ‘Guardian gene’ is the name given to?

 A

Myc

 B

Meu

 C

P53

 D

Abl

Ans. C

Explanation:

p53 acts as a ‘molecular policemen’ that prevents the propagation of genetically damaged cells. p53 is crucial in multicellular organisms, where it regulates the cell cycle and, thus, functions as a tumor suppressor that is involved in preventing cancer.

As such, p53 has been described as “the guardian of the genome” because of its role in conserving stability by preventing genome mutation.

Ref: Robbins Pathology, 7th Ed, page 302

Quiz In Between


Q. 7

Which of the following statement is NOT true about p53 protein?

 A

It is present on chromosomes 17

 B

It causes cell cycle arrest in G1

 C

53 KDa

 D

Non mutated wild p53 is associated with neoplasm in childhood

Q. 7

Which of the following statement is NOT true about p53 protein?

 A

It is present on chromosomes 17

 B

It causes cell cycle arrest in G1

 C

53 KDa

 D

Non mutated wild p53 is associated with neoplasm in childhood

Ans. D

Explanation:

The tumor suppressor p53, a protein of apparent MW 53 kDa.

The gene for human p53 cellular tumor antigen is located on chromosome 17 short arm (17p13).

When DNA Is damaged, the p53 protein accumulates in cells.

It first arrests the cell cycle (at the G1 phase) to allow the DNA to be repaired before it is replicated.

A cell with damaged DNA that cannot be repaired is directed by p53 to either enter senescence or undergo apoptosis.

In view of these activities, p53 has been called the ‘guardian of the genome’. 

With homozygous loss of the TP53 gene, DNA damage goes unrepaired, mutations become fixed in dividing cells, and the cell turns to malignant transformation.
 
Ref: Robbins Basic Pathology By Vinay Kumar, Abul K. Abbas, Nelson Fausto, Richard Mitchell, 2012, Page 20, 185-187

Q. 8

Which of the following is known as the guardian of the genome?

 A

VEGFR-2

 B

p53

 C

Mdm2

 D

4ATM

Q. 8

Which of the following is known as the guardian of the genome?

 A

VEGFR-2

 B

p53

 C

Mdm2

 D

4ATM

Ans. B

Explanation:

Due to its critical importance in maintaining genetic stability p53 is called the “gatekeeper” or “guardian” of the genome.
 
The tumor suppressor protein p53 is a key regulator, of the cellular response to geno-toxic damage, and thus plays a pivotal role in preventing cancer formation. Once DNA damage has been incurred, p53 can elicit several different responses to either correct the errors or destroy the damaged cell. 
 
3 Important actions: 
 
1. p53 can induce G1 cell cycle arrest, which stops the cell from dividing and allows time to repair the damage before the DNA is replicated. 
 
2. p53 can activate DNA repair proteins to drive the repair of damaged DNA. 
 
3. As a last resort, p53 can induce damaged cells to undergo programmed cell death (apoptosis), thereby eliminating damaged and potentially dangerous cells at risk for neoplastic transformation. 
 
Ref: Cancer Genome and Tumor Microenvironment, By Andrei Thomas-Tikhonenko, 2010, Page, 190

Q. 9

Regarding oncogenesis –

 A

Topoisomerase causes breaks in strands

 B

P53 is the most common oncogene mutation causing malignancy in humans

 C

At G2-M-phase there is loss of inhibitors controlling cell cycle

 D

All options are correct

Q. 9

Regarding oncogenesis –

 A

Topoisomerase causes breaks in strands

 B

P53 is the most common oncogene mutation causing malignancy in humans

 C

At G2-M-phase there is loss of inhibitors controlling cell cycle

 D

All options are correct

Ans. D

Explanation:

 

o p53 gene is located on chromosome 17p13 and it is the most common target for genetic alteration in human tumors. A little over 50% human tumors contain mutation in this gene.

o DNA topoisomerase bind tightly to DNA double helix and make transient breaks in both strands.

o Telomerase activity and maintenance of Telomer length are essential for maintenance of relicative potential in tumor cells —> Decrease of telomerase activity cause antitumor effects

o GIS and G2M are cell cycle check point and defect in cell cycle check point component is a major cause of genetic instability in cancer cells.

Quiz In Between


Q. 10

Increased susceptibility to breast cancer is likely to be associated with a mutation in the following gene-      

 A

p53

 B

BRCA-1

 C

Retinoblastoma

 D

a and b

Q. 10

Increased susceptibility to breast cancer is likely to be associated with a mutation in the following gene-      

 A

p53

 B

BRCA-1

 C

Retinoblastoma

 D

a and b

Ans. D

Explanation:

 

o Genetic mutations associated with breast cancer are of two types‑

(i)      Germline mutations (inherited mutations)

o Involved in familial cases of breast cancer

(ii)    Somatic mutations (acquired mutation)

o Involved in sporadic cases of breast cancer

o Tumour suppressor genes involved in Breast cancer

(i) Genes involved in germline mutations (cause familial Breast Ca)

(a)  BRCA-1 and BRCA-2 –> Causes familial breast and ovary Ca

(b)  p53 —> (Li Fraumeni syndrome) — There is increased susceptibility to Ca breast, colon, leukemia, Sarcomas, brain tumours.

(ii) Genes involved in Somatic mutation (cause spordic breast cancer or primary breast cancer) —-> p53

o So p53 gene is involved in both germline mutation and somatic mutation causing breast cancer. Where as BRCA-1 is involved only in germline mutation


Q. 11

Which of the following mutations in a tumour suppressor agent causes breast carcinoma?

 A

p43

 B

p53

 C

p73

 D

p83

Q. 11

Which of the following mutations in a tumour suppressor agent causes breast carcinoma?

 A

p43

 B

p53

 C

p73

 D

p83

Ans. B

Explanation:

Q. 12

Which of the following is known as the “guardian of the genome”?

 A

p53

 B

Mdm2

 C

p14

 D

ATM

Q. 12

Which of the following is known as the “guardian of the genome”?

 A

p53

 B

Mdm2

 C

p14

 D

ATM

Ans. A

Explanation:

 

p53; Guardian of genome

o p53 is a tumor suppressor gene.

o p53 gene is located on chromosome 17.

o p53 acts as molecular policeman that prevents the propagation of genetically damage cell.

o p53 gene product, i.e. p53 protein is a DNA binding protein in the nucleus, when called into action, it controls the transcription of several other genes.

o The major functional activities of the p53 protein are cell cycle arrest and initiation of apoptosis in response to DNA damage.

o When there is DNA damage due to irradiation, UV light or mutagenic chemicals, there is rapid increase in p53

levels.

Quiz In Between


Q. 13

The following statements are true about Tumour Suppressor Gene p53 except –

 A

It regulates cetain genes involved in cell cycle regulation

 B

Its increased levels can induce apoptosis

 C

Its activity in the cells decreases following UV irradiation and stimulates cell cycle

 D

Mutations of the p53 gene are the most common genetic alteration seen in human cancer

Q. 13

The following statements are true about Tumour Suppressor Gene p53 except –

 A

It regulates cetain genes involved in cell cycle regulation

 B

Its increased levels can induce apoptosis

 C

Its activity in the cells decreases following UV irradiation and stimulates cell cycle

 D

Mutations of the p53 gene are the most common genetic alteration seen in human cancer

Ans. C

Explanation:

Q. 14

True statements about P53 gene are all except

 A

Arrest cell cycle at GI Phase

 B

Product is 53 KD protein

 C

Located on chromose 17

 D

Wild/non-mutated form is associated with in­creased risk of childhood tumors.

Q. 14

True statements about P53 gene are all except

 A

Arrest cell cycle at GI Phase

 B

Product is 53 KD protein

 C

Located on chromose 17

 D

Wild/non-mutated form is associated with in­creased risk of childhood tumors.

Ans. D

Explanation:

 

It is mutated form (not non mutated form) of p53 which is associated with increased risk of tumors.

o p53 gene is a tumor suppressor gene and non-mutated form of this gene prevent development of malignancy by :

(i)   Causing cell cycle arrest in late GI phase

(ii)   Inducing apoptosis

(iii)  Helping in DNA repair

o Mutation in p53 gene causes inactivation of p53 gene and abolishen of above function that results in uncontrolled proliferation of cells and malignant transformation.

o Mutation in p53 gene is the most common genetic alteration found in human Cancer.

o The name p53 is in reference to its apparent molecular mass; it runs as a 53 kilodalton (Kda) protein on SDS-page. But based on calculations from its amino acid residues, p53 ‘s mass is actually only 43.7 K Da.

 

o p53 prevents neoplastic transformation by three interlocking mechanisms :

1.  Activation of temporary cell cycle arrest (quiescence)

o It is considered as the primordial response to DNA damage.

o p53 causes arrest in late GI through p2 I .

o This is temporary arrest that gives the cell “breathing time” to repair DNA damage.

o After DNA repair, cell cycle block is relieved by MDM-3 which degrades p53.

2.  Induction of permanent cell cycle arrest (senescence)

o p53 induced sencence is a permanent cell cycle arrest characterized by specific changes in morphology and gene expression that differentiate it from quiescence (temporary or reversible cycle arrest).

o The mechanisms of senscence is unknown, but involve epigenetic changes that result in the formation of heterochromatin at different loci throughout the genome.

3.  Triggering of programmed cell death

o p53 directs the transcription of several pro-apoptotic genes such as BAX and PUMA (approved name BBC3) and induces apoptosis.

o It has been shown that p53 activates transcription of the mir 34 family of micro RNAs (miRNAs),mir 34a.

o mir34 inhibits translation of anti-apoptotic genes such as BCL2 (there by induce apoptosis) and pro-proliferative genes such as cyclins (there by prevent proliferation) p53 induce apoptosis and prevent proliferation through mir34.


Q. 15

True about p53 –

 A

Tumor suppressor gene

 B

Protooncogene

 C

Proapoptotic

 D

a and c

Q. 15

True about p53 –

 A

Tumor suppressor gene

 B

Protooncogene

 C

Proapoptotic

 D

a and c

Ans. D

Explanation:

 

o p 53 is a tumor suppressor gene and it is a proapoptotic factor, i.e. it promotes apoptosis if repair of DNA damage is unsuccessful at G1 arrest.

o The name p53 is in reference to its apparent molecular mass; it runs as a 53 kilodalton (Kda) protein on SDS-page. But based on calculations from its amino acid residues, p53 ‘s mass is actually only 43.7 K Da.

Quiz In Between


Q. 16

False about p53 is –

 A

It is present on chromosomes 17

 B

It cvauses cell cylce arrest in G1

 C

53 KDa

 D

Non mutated wild p53 is associated with neoplasm in childhood

Q. 16

False about p53 is –

 A

It is present on chromosomes 17

 B

It cvauses cell cylce arrest in G1

 C

53 KDa

 D

Non mutated wild p53 is associated with neoplasm in childhood

Ans. D

Explanation:

Ans. is ‘d’ i.e., Non mutated wild p53 is associated with neoplasm in childhood

It is mutated form (not non mutated form) of p53 which is associated with increased risk of tumors.

o p53 gene is a tumor suppressor gene and non-mutated form of this gene prevent development of malignancy by :

(i)       Causing cell cycle arrest in late GI phase

(ii)     Inducing apoptosis

(iii)      Helping in DNA repair

o Mutation in p53 gene causes inactivation of p53 gene and abolishen of above function that results in uncontrolled proliferation of cells and malignant transformation.

o Mutation in p53 gene is the most common genetic alteration found in human Cancer.

o The name p53 is in reference to its apparent molecular mass; it runs as a 53 kilodalton (Kda) protein on SDS-page. But based on calculations from its amino acid residues, p53’s mass is actually only 43.7 K Da.


Q. 17

The tumor suppressor gene P53 induces cell arrest at-

 A

M phase

 B

S – G2 phase

 C

G1 – S phase

 D

Go – phase

Q. 17

The tumor suppressor gene P53 induces cell arrest at-

 A

M phase

 B

S – G2 phase

 C

G1 – S phase

 D

Go – phase

Ans. C

Explanation:

Ans. is ‘c’ i.e., G1 – S phase


Q. 18

In Breast cancer following are expressed: 

 A

HER2/neu

 B

P53

 C

B RCA1

 D

All

Q. 18

In Breast cancer following are expressed: 

 A

HER2/neu

 B

P53

 C

B RCA1

 D

All

Ans. D

Explanation:

Ans. is ‘a’, ‘b’ & ‘c’ i.e. HER2/neu, P53 & BRCA1

Quiz In Between



Medical Termination Of Pregnancy (Mtp) Act

MEDICAL TERMINATION OF PREGNANCY (MTP) ACT

Q. 1

For MTP consent is taken from

 A

Wife only

 B

Husband only

 C

Both

 D

Neither

Q. 1

For MTP consent is taken from

 A

Wife only

 B

Husband only

 C

Both

 D

Neither

Ans. A

Explanation:

A i.e. Wife only

– MTP Act 1971 allows registered medical practioner with certified experience (by CMO, of assisting  25 MTP cases) to perform abortion in a licenced setup (Governement or private). The consent of only women is required before conducting abortion; written consent of guardian is required if the women is a minor or mentally ill. Consent of husband is not necessaryQ.

MTP Act 1971 does not allow abortion on poor socioeconomic grounds and if only husband (not mother) is willingQ. Rape; contraceptive failure, use of cytotoxic drugs, risk of delivering seriously handicapped baby, and injury to physical & mental health of mother are valid grounds for MTP.


Q. 2

The MTP act defines the following –

 A

Who should do termination of pregnancy

 B

Where it should be done

 C

When it should be done

 D

All of the above

Q. 2

The MTP act defines the following –

 A

Who should do termination of pregnancy

 B

Where it should be done

 C

When it should be done

 D

All of the above

Ans. D

Explanation:

Ans. is ‘d’ i.e., All of the above 

Medical termination of pregnancy act (1971)

o The MTP act, 1971 lays down

1.       The conditions under which a pregnancy can be terminated.

2.       The person or persons who can perform such terminations.

3.       The place where such terminations can be performed.


Q. 3

Consent from guardian/husband for MTP is required if –

 A

Female is below 28 years of age

 B

Female is below 23 years of age

 C

Female is below 17 years of age

 D

All

Q. 3

Consent from guardian/husband for MTP is required if –

 A

Female is below 28 years of age

 B

Female is below 23 years of age

 C

Female is below 17 years of age

 D

All

Ans. C

Explanation:

Ans. is ‘c’ i.e., Female is below 17 years of age 

The conditions under which a pregnancy can be terminated under the MTP Act. 1971 :

There are 5 conditions that have been identified in the Act :

a.       Medical – where continuation of the pregnancy might endanger the mother’s life or cause grave injury to her physical or mental health.

b.       Eugenic – where there is substantial risk of the child being born with serious handicaps due to physical or mental abnormalities.

c.        Humanitarian – where pregnancy is the result of rape

d.       Socio-economic – where actual or reasonably foreseeable environments (whether social or economic could lead to risk of injury to the health of the mother).

e.        Failure of contraceptive devices – The anguish caused by an unwanted pregnancy resulting from a failure of any contraceptive device or method can be presumed to constitute a grave mental injury to the health of the mother. This condition is a unique feature of the Indian law and virtually allows abortion on request, in view of the difficulty of proving that a pregnancy was not caused by failure of contraception.

The written consent of the guardian is necessary before performing abortion in women under 18 years of age, and in lunatics even if they are older than 18 years.

Quiz In Between


Q. 4

MTP Act in India does not permit termination of pregnancy after:       

Delhi 06; AP 06; JIPMER 14

 A

12 weeks

 B

16 weeks

 C

20 weeks

 D

24 weeks

Q. 4

MTP Act in India does not permit termination of pregnancy after:       

Delhi 06; AP 06; JIPMER 14

 A

12 weeks

 B

16 weeks

 C

20 weeks

 D

24 weeks

Ans. C

Explanation:

Ans. 20 weeks


Q. 5

According to MTP Act, 2 doctors opinion is required when pregnancy is:        

Maharashtra 09

 A

6 weeks

 B

10 weeks

 C

> 12 weeks

 D

> 20 weeks

Q. 5

According to MTP Act, 2 doctors opinion is required when pregnancy is:        

Maharashtra 09

 A

6 weeks

 B

10 weeks

 C

> 12 weeks

 D

> 20 weeks

Ans. C

Explanation:

Ans. > 12 weeks


Q. 6

Under the MTP Act, termination of pregnancy can be done by:          

Rohtak 08

 A

Any registered medical practitioner (RMP)

 B

Any RMP with MD/MS degree in Obs and Gynae

 C

Any RMP with 6 months residency in any depar intent

 D

Any RMP with a minimum of 3 months residency in Obs and Gynae

Q. 6

Under the MTP Act, termination of pregnancy can be done by:          

Rohtak 08

 A

Any registered medical practitioner (RMP)

 B

Any RMP with MD/MS degree in Obs and Gynae

 C

Any RMP with 6 months residency in any depar intent

 D

Any RMP with a minimum of 3 months residency in Obs and Gynae

Ans. B

Explanation:

Ans. Any RMP with MD/MS degree in Obs and Gynae

Quiz In Between


Q. 7

Indication of MTP-

 A

Pregnancy due to rape

 B

Contraceptive failure

 C

Pregnancy endangering mother’s life

 D

All of the above

Q. 7

Indication of MTP-

 A

Pregnancy due to rape

 B

Contraceptive failure

 C

Pregnancy endangering mother’s life

 D

All of the above

Ans. D

Explanation:

Ans. is ‘d’ i.e., All of the above

The conditions under which pregnancy can be terminated under the MTPAct

  • There are 4 conditions that have been identified in the Act :
  1. Medical – Where continuation of the pregnancy might endanger the mother’s life or cause grave injury to her physical or mental health.
  2. Eugenic – Where there is substantial risk of the child being born with serious handicaps due to physical or mental abnormalities.
  3. Humanitarian – Where pregnancy is the result of rape.
  4. Failure of contraceptive devices – The anguish caused by an unwanted pregnancy resulting from a failure of any contraceptive device or method can be presumed to constitute a grave mental injury to the health of the mother. This condition is a unique feature of the Indian law and virtually allows abortion on request, in view of the difficulty of proving that a pregnancy was not caused by failure of contraception

Q. 8

MTP Act of 1971 provides for termination of pregnancy till how many weeks of pregnancy ‑

 A

12 weeks

 B

16 weeks

 C

20 weeks

 D

24 weeks

Q. 8

MTP Act of 1971 provides for termination of pregnancy till how many weeks of pregnancy ‑

 A

12 weeks

 B

16 weeks

 C

20 weeks

 D

24 weeks

Ans. C

Explanation:

Ans. is ‘c’ i.e., 20 weeks 

Medical termination of pregnancy act (19711

  • The MTP act, 1971 lays down
  1. The conditions under which a pregnancy can be terminated.
  2. The person or persons who can perform such terminations.
  3. The place where such terminations can be performed.
  4. The conditions under which a pregnancy can be terminated under the MTPAct. 1971 :
  5. There are 5 conditions that have been identified in the Act :
  • Medical – where continuation of the pregnancy might endanger the mother’s life or cause grave injury to her physical or mental health.
  • Eugenic – where there is substantial risk of the child being born with serious handicaps due to physical or mental abnormalities.
  • Humanitarian – where pregnancy is the result of rape
  • Socio-economic – where actual or reasonably foreseeable environments (whether social or economic could lead to risk of injury to the health of the mother).
  • Failure of contraceptive devices – The anguish caused by an unwanted pregnancy resulting from a failure of any contraceptive device or method can be presumed to constitute a grave mental injury to the health of the mother. This condition is a unique feature of the Indian law and virtually allows abortion on request, in view of the difficulty of proving that a pregnancy was not caused by failure of contraception.
  • The written consent of the guardian is necessary before performing abortion in women under 18 years of age, and in lunatics even if they are older than 18 years.
  • The person or persons who can perform abortion The Act provides safeguards to the mother by authorising only a Registered Medical Practitioner having experience in gynaecology and obstetrics to perform abortion where the length of pregnancy does not exceed 12 weeks. o However, where the pregnancy exceeds 12 weeks and is not more than 20 weeks, the opinion of two Ragistered o Medical Practitioners is necessary to terminate the pregnancy.
  • Where abortion can be done
  • The Act stipulates that no termination of pregnancy shall be made at any place other than a hospital established or maintained by Government or a place approved for the purpose of this Act by Government.
  • Abortion services are provided in hospitals in strict confidence. The name of the abortion seeker is kept confidential, since abortion has been treated statutorily as a personal matter.

Quiz In Between



Undertaker Fracture,Stalking,para-suicide, heat and cold stoke

Undertaker Fracture,Stalking,para-suicide, heat and cold stroke

Q. 1

Which among the following sites are involved in “undertakers fracture”?

 A

Skull

 B

Cervical spine

 C

Pelvis

 D

Mandible

Q. 1

Which among the following sites are involved in “undertakers fracture”?

 A

Skull

 B

Cervical spine

 C

Pelvis

 D

Mandible

Ans. B

Explanation:

The so called undertaker fracture is caused due to the head falling backward forcibly after death.

It usually causes one of intervertebral discs (usually around C6 or C7) to tear open and results in the subluxation of the lower cervical spine.

Ref: Textbook of Forensic Medicine and Toxicology by Narayan Reddy, Edition 21, Page – 92


Q. 2

In heat stroke, skin will be:               

September 2012

 A

Warm

 B

Normal

 C

Cold

 D

Cyanosis

Q. 2

In heat stroke, skin will be:               

September 2012

 A

Warm

 B

Normal

 C

Cold

 D

Cyanosis

Ans. A

Explanation:

Ans. A i.e. Warm

Heat stroke presents with a hyperthermia of greater than 40.6 °C (105.1 °F) in combination with confusion and a lack of sweating.


Q. 3

NOT seen in heat stroke: 

Kerala 06

 A

Hypovolemic shock

 B

Rhabdomyolysis

 C

Pancreatitis

 D

Cerebral edema

Q. 3

NOT seen in heat stroke: 

Kerala 06

 A

Hypovolemic shock

 B

Rhabdomyolysis

 C

Pancreatitis

 D

Cerebral edema

Ans. A

Explanation:

Ans. Hypovolemic shock

Quiz In Between


Q. 4

Undertaker’s fracture is seen at the level of cervical vertebra:

 A

C1-C2

 B

C3-C4

 C

C5-C6

 D

C6-C7

Q. 4

Undertaker’s fracture is seen at the level of cervical vertebra:

 A

C1-C2

 B

C3-C4

 C

C5-C6

 D

C6-C7

Ans. D

Explanation:

Ans. C6-C7

  • It is caused clue to the head falling backwards forcibly after death, which tears open one of the intervertebral discs, usually around C6 and C7.
  • Due to the prolapse of the cervical intervertebral disc, there is increased tendency of subluxation of the lower cervical spine.

Q. 5

What is the most common cause of parasuicide?

 A

Drug ingestion

 B

Hanging

 C

Cutting wrist

 D

Firearms

Q. 5

What is the most common cause of parasuicide?

 A

Drug ingestion

 B

Hanging

 C

Cutting wrist

 D

Firearms

Ans. A

Explanation:

Ans. a. Drug ingestion

Parasuicide is a suicide attempt or gesture and self-harm where there is no result in death. It is a non7fatal act in which a person deliberately causes injury to him or herself or ingests any prescribed or generally recognized therapeutic dose in excess. It is considered to be a serious public health issue. Parasuicide is the strongest known indicator for a future successful suicide attempt. Examples of suicidal gestures include cutting, where the cut is not deep enough to cause significant blood loss, or taking a non-lethal overdose of medication.’- http://en.wikipedia.org/wiki/Parasuicide

`Maximum cases of near-suicides are due to drug overdose. In India, wrist cutting is equally common, but drug overdose is by far the most common cause of parasuicide in the world.’- Methods used for parasuicide: results of the WHO/EURO Multicentre Study on Parasuicide.

Quiz In Between



Ethylene glycol & Boric acid poisoning

Ethylene glycol & Boric acid poisoning

Q. 1

Which of the properties accounts for ethanol’s use in ethylene glycol poisoning?

 A

Competitive inhibitor of NADPH oxidase

 B

Competitive inhibitor of alcohol dehydrogenase

 C

Competitive inhibitor of aldehyde dehydrogenase

 D

Non-competitive inhibitor of aldehyde dehydrogenase

Q. 1

Which of the properties accounts for ethanol’s use in ethylene glycol poisoning?

 A

Competitive inhibitor of NADPH oxidase

 B

Competitive inhibitor of alcohol dehydrogenase

 C

Competitive inhibitor of aldehyde dehydrogenase

 D

Non-competitive inhibitor of aldehyde dehydrogenase

Ans. B

Explanation:

Ethanol acts by competing with ethylene glycol for alcohol dehydrogenase, the first enzyme in the degradation pathway.

Because ethanol has a much higher affinity for alcohol dehydrogenase, about a 100-times greater affinity, it successfully blocks the breakdown of ethylene glycol into glycoaldehyde, which prevents the further degradation.

Ref: Lehninger Principles of Biochemistry, 4th Edition, Page 336; Updates in Emergency Medicine By John Cahill, 2002, Page 115


Q. 2

Antidote for Ethylene glycol poisoning-

 A

Methyl violet

 B

Methyl violet

 C

Fomepizole

 D

All

Q. 2

Antidote for Ethylene glycol poisoning-

 A

Methyl violet

 B

Methyl violet

 C

Fomepizole

 D

All

Ans. C

Explanation:

Ans. is ‘c’ i.e., Fomepizole

o I.V. fomepizole or ethanol is given in ethylene glycol poisoning.

o Fomepizole (an alcohol dehydrogenase inhibitor) is the DOC.


Q. 3

Ethanol is used for ethylene glycol poisoning because it is a: 

JIPMER 13

 A

Competitive inhibitor of aldehyde dehydrogenase

 B

Higher affinity for alcohol dehydrogenase

 C

Chemically combines and neutralizes ethylene glycol

 D

A and B

Q. 3

Ethanol is used for ethylene glycol poisoning because it is a: 

JIPMER 13

 A

Competitive inhibitor of aldehyde dehydrogenase

 B

Higher affinity for alcohol dehydrogenase

 C

Chemically combines and neutralizes ethylene glycol

 D

A and B

Ans. B

Explanation:

Ans. Higher affinity for alcohol dehydrogenase

Competitive inhibitor of alcohol dehydrogenase [Ref Lehninger Principles of Biochemistry 4th/e p. 336]


Q. 4

Ethylene glycol when ingested affects kidney by forming:      

NEET 13

 A

Formaldehyde

 B

Oxalates

 C

Phytates

 D

Phosphates

Q. 4

Ethylene glycol when ingested affects kidney by forming:      

NEET 13

 A

Formaldehyde

 B

Oxalates

 C

Phytates

 D

Phosphates

Ans. B

Explanation:

Ans. Oxalates

Quiz In Between



Miscellaneous Poisoning

MISCELLANEOUS POISONING

Q. 1

Sewer gas is:

 A

Phosgene

 B

H2S

 C

CO2

 D

b and c

Q. 1

Sewer gas is:

 A

Phosgene

 B

H2S

 C

CO2

 D

b and c

Ans. D

Explanation:

B i.e. H2S > C i.e. CO2

Name

Composition

Sewer gas

– H2S, with CO2 &

MethaneQ

Marsh gas

– Methane

War gases

 

– Blistering

– Mustard gas &

gas

B.A.L (lewisite

gas)

– Tear gas

– BBC (Bromo

 

Benzyl Cyanide)

 

– CAP (Chlor Aceto

 

Phenone)

 

– KSK (Ethyl lido

acetate)

– Sickening

– Diphenyl amine

gas

chlor arsine


Q. 2

Orange-skin cornea results due to:

 A

Chalcosis

 B

Siderosis

 C

Ammonia burn

 D

Mustard gas

Q. 2

Orange-skin cornea results due to:

 A

Chalcosis

 B

Siderosis

 C

Ammonia burn

 D

Mustard gas

Ans. D

Explanation:

Ans. Mustard gas


Q. 3

Blistering war gas is:

Maharashtra 09

 A

Chlorine gas

 B

Mustard gas

 C

HCN gas

 D

Tabun

Q. 3

Blistering war gas is:

Maharashtra 09

 A

Chlorine gas

 B

Mustard gas

 C

HCN gas

 D

Tabun

Ans. B

Explanation:

Ans. Mustard gas

Quiz In Between



DELIRIANT POISONS: Dhatura,Strychnine

DELIRIANT POISONS: Dhatura,Strychnine

Q. 1

Signs and symptoms usually develop within 15–30 minutes of strychnine ingestion. What is the fatal dose of strychnine?

 A

10-15 mg

 B

20-40 mg

 C

60-100 mg

 D

100-120 mg

Q. 1

Signs and symptoms usually develop within 15–30 minutes of strychnine ingestion. What is the fatal dose of strychnine?

 A

10-15 mg

 B

20-40 mg

 C

60-100 mg

 D

100-120 mg

Ans. C

Explanation:

Strychnine poisoning:

  • It is an alkaloid derived from the seeds of the tree Strychnos nux-vomica.
  • The potentially fatal dose of strychnine is approximately 50–100 mg (1 mg/kg) and fatal period is 1-2 hours.
  • Strychnine competitively antagonizes glycine, an inhibitory neurotransmitter released by postsynaptic inhibitory neurons in the spinal cord.
  • Muscular stiffness and painful cramps precede generalized muscle contractions, extensor muscle spasms, and opisthotonus. 
  • Death usually is caused by respiratory arrest that results from intense contraction of the respiratory muscles.
 
Ref: Nordt S.P. (2012). Chapter 145. Strychnine. In K.R. Olson (Ed), Poisoning & Drug Overdose, 6e.

Q. 2

Which of the following is the site of action of strychnine poison for its toxicity?

 A

Heart

 B

Anterior horn cells

 C

Posterior horn cells

 D

All of the above

Q. 2

Which of the following is the site of action of strychnine poison for its toxicity?

 A

Heart

 B

Anterior horn cells

 C

Posterior horn cells

 D

All of the above

Ans. B

Explanation:

Strychnine competitively blocks ventral horn motor neuron postganglionic receptor sites in the spinal cord and prevents the effects of glycine, an inhibitory neurotransmitter released by postsynaptic inhibitory neurons in the spinal cord.
Strychnine binds to the chloride ion channel, causing increased neuronal excitability and exaggerated reflex arcs.
This results in generalized seizure-like contraction of skeletal muscles.
Simultaneous contraction of opposing flexor and extensor muscles causes severe muscle injury, with rhabdomyolysis, myoglobinuria, and, in some cases, acute renal failure.
 
Ref: Nordt S.P. (2012). Chapter 145. Strychnine. In K.R. Olson (Ed), Poisoning & Drug Overdose, 6e.

Q. 3

True about strychnine poisoning is :

 A

All muscles affected at the same time

 B

Shoulder girdle affected first

 C

Pelvic girdle affected first

 D

None of the above

Q. 3

True about strychnine poisoning is :

 A

All muscles affected at the same time

 B

Shoulder girdle affected first

 C

Pelvic girdle affected first

 D

None of the above

Ans. A

Explanation:

A i.e. All muscles affected at same time

Quiz In Between


Q. 4

Nux vomica seeds contain 2 alkaloids, strychnine and :

 A

Hyoscine

 B

Hyoscyamine

 C

Brucine

 D

Atropine

Q. 4

Nux vomica seeds contain 2 alkaloids, strychnine and :

 A

Hyoscine

 B

Hyoscyamine

 C

Brucine

 D

Atropine

Ans. C

Explanation:

C i.e. Brucine


Q. 5

The active principles of Dhatura are all of the following except :

 A

Pyricatachol

 B

Hyoscyamine

 C

Atropine

 D

Hyoscine

Q. 5

The active principles of Dhatura are all of the following except :

 A

Pyricatachol

 B

Hyoscyamine

 C

Atropine

 D

Hyoscine

Ans. A

Explanation:

A i.e. Pyricatachol


Q. 6

All the following are characteristic of Dhatura poisoning except:

 A

Delirium

 B

Diplopia

 C

Pin-point pupils

 D

Dysphagia

Q. 6

All the following are characteristic of Dhatura poisoning except:

 A

Delirium

 B

Diplopia

 C

Pin-point pupils

 D

Dysphagia

Ans. C

Explanation:

C i.e. Pinpoint pupil

Quiz In Between


Q. 7

Following is not present in dhatura:           

JIPMER 11

 A

Hyoscine

 B

Hyoscyamine

 C

Muscarine

 D

Atropine

Q. 7

Following is not present in dhatura:           

JIPMER 11

 A

Hyoscine

 B

Hyoscyamine

 C

Muscarine

 D

Atropine

Ans. C

Explanation:

Ans. Muscarine


Q. 8

Treatment of dhatura poisoning is done with:

NEET 15

 A

Pilocarpine

 B

Naloxone

 C

Physostigmine

 D

Neostigmine

Q. 8

Treatment of dhatura poisoning is done with:

NEET 15

 A

Pilocarpine

 B

Naloxone

 C

Physostigmine

 D

Neostigmine

Ans. C

Explanation:

Ans. Physostigmine


Q. 9

Strychnine acts by inhibiting:       

COMEDK 15

 A

GABA

 B

Glycine

 C

Acetylcholine

 D

Dopamine

Q. 9

Strychnine acts by inhibiting:       

COMEDK 15

 A

GABA

 B

Glycine

 C

Acetylcholine

 D

Dopamine

Ans. B

Explanation:

Ans. Glycine

Quiz In Between


Q. 10

Antidote for strychnine poisoning is:          

NEET 13

 A

Fomepizole

 B

Physotigmine

 C

Barbiturates

 D

Naloxone

Q. 10

Antidote for strychnine poisoning is:          

NEET 13

 A

Fomepizole

 B

Physotigmine

 C

Barbiturates

 D

Naloxone

Ans. C

Explanation:

Ans. Barbiturates


Q. 11

In dhatura poisoning 9 ‘Ds’ include all except ‑

 A

Diarrhea

 B

Dysphagia

 C

Dilated pupil

 D

Drowsiners

Q. 11

In dhatura poisoning 9 ‘Ds’ include all except ‑

 A

Diarrhea

 B

Dysphagia

 C

Dilated pupil

 D

Drowsiners

Ans. A

Explanation:

Ans. is ‘a’ i.e., Diarrhea

9-D’ manifestations of Dhatura (anticholinergic) poisoning

  1. Dry hot skin i.e. ‘Hot as a hare’.
  2. Dialation of cutaneous blood vessels 1/t facial i.e. ‘Red as a beet’.
  3. Dialation of pupils with loss of accomodation and unresponsiveness to light i.e. ‘Blind as a bat’.
  4. Dryness of mouth and throat i.e. ‘Dry as a bone’.
  5. Difficulty in talking.
  6. Dysphagia (difficulty in swallowing)
  7. Drunken gait
  8. Drowsiness
  9. Delirium i.e. ‘Mad as wet hen’
  • So symptoms are described as ‘Dry as a bone, Red as a beet, Hot as hare, Blind as a bat and Mad as a wet hen’.

Quiz In Between



CNS DEPRESSANTS: Barbiturate poisoning & Chloral hydrate

CNS DEPRESSANTS: Barbiturate poisoning & Chloral hydrate


Barbiturate  poisoning

  • It  is  mostly  suicidal, sometimes  accidental.
  • Manifestations  are due  to  excessive  CNS  depression.
  • patient  is  flabby  and  comatose  with shallow  and failing  respiration  cyanosis.
  • fall  in  BP and  cardiovascular  collapse.
  • Renal shut  down,  pulmonary complications,  bullous eruption  (barbiturate  blisters).
  • Hypothermia ,  Finally  there  is  coma.
  • Individuals treated with drugs such as barbiturates show hypertrophy of the smooth endoplamic reticulum (ER) in hepatocytes

Important  specific  features  are

  • constricted  pupil (there  may  be  alternate  constriction and dilatation,  i.e. happus reaction).
  • liquid  gold  urine.

 Treatment  includes  :

  • Gastric  lavage
  • Haemodialsis
  • Supportive  measures (O2, assissed  respiration,  maintenance  of BP).
  • Forced  alkaline  diuresis.

Chloral Hydrate

  • Chloral  hydrate  is  a colourless,  crystalline  substance  having  peculiar  pungent  odor  and pungent bitter test.
  • It  is  a powerful  hypnotic in doses  produces  natural  sleep,  but in  larger  doses  depresses  CNS  and paralyses  vital centers.
  • It  is  given  in  food  or drink  to  make person  helpless,  called  knock out drops.
  • Mickey finn is  a  combination  of alcohol and chloral  hydrate.
  • Chloral  hydrate  is  also  called  dry  wine.

Exam Important

Barbiturate  poisoning:

  • patient  is  flabby  and  comatose  with shallow  and failing  respiration  cyanosis.
  • fall  in  BP and  cardiovascular  collapse
  • Hypothermia ,  Finally  there  is  coma.
  • Individuals treated with drugs such as barbiturates show hypertrophy of the smooth endoplamic reticulum (ER) in hepatocytes.

Chloral Hydrate:

  • crystalline  substance  having  peculiar  pungent  odor  and pungent bitter test.
  • It  is  given  in  food  or drink  to  make person  helpless,  called  knock out drops.
  • Mickey finn is  a  combination  of alcohol and chloral  hydrate.
  • Chloral  hydrate  is  also  called  dry  wine
Don’t Forget to Solve all the previous Year Question asked on CNS DEPRESSANTS: Barbiturate poisoning & Chloral hydrate

Module Below Start Quiz

Phosphorus Poisioning

phosphorous poisoning

Q. 1

CuSO4 was used as an antidote for:

Maharashtra 11

 A

Dhatura poisoning

 B

Cocaine poisoning

 C

Phosphorus poisoning

 D

Opium poisoning

Q. 1

CuSO4 was used as an antidote for:

Maharashtra 11

 A

Dhatura poisoning

 B

Cocaine poisoning

 C

Phosphorus poisoning

 D

Opium poisoning

Ans. C

Explanation:

Ans. Phosphorus poisoning


Q. 2

Yellow/fatty liver is characteristically seen in:

Jharkhand 11

 A

Datura poisoning

 B

Cocaine poisoning

 C

Phosphorus poisoning

 D

Opium poisoning

Q. 2

Yellow/fatty liver is characteristically seen in:

Jharkhand 11

 A

Datura poisoning

 B

Cocaine poisoning

 C

Phosphorus poisoning

 D

Opium poisoning

Ans. C

Explanation:

Ans. Phosphorus poisoning

Quiz In Between



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