PAPER : ALL INDIA 2005 (AIPGE 2005 January, Answers, Explanation and Notes)

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Q.251. All of the following are true about manifestations of vitamin E deficiency except:
1. Hemolytic anemia.
2. Posterior column abnormalities.
3. Cerebellar ataxia.
4. Autonomic dysfunction.

Q.252. Differential expression of same gene depending on parent of origin is referred to as:
1. Genomic imprinting.
2. Mosaicism.
3. Anticipation.
4. Nonpenetrance.

Q.253. The chances of having an unaffected baby, when both parents have achondroplasia, are:
1. 0%.
2. 25%.
3. 50%.
4. 100%.

Q.254. All of the following therapies may be required in a 1-hour-old infant with severe birth asphyxia except:
1. Glucose.
2. Dexamethasone.
3. Calcium gluconate.
4. Normal saline.

254. O.P. Ghai, Essential of Pediatrics 5th ed/153.

1) Maintain temperature
2) Provide O2/ventilation as required.
3) Ensure normal B.P: Volume expanders, dopamine, dobutamine.
4) Acidosis, hypoglycemia, hypocalcemia needs correction
5) Treat seizures.

Dexamethasone has no role in resuscitation of asphyxiated newborn.

When asphyxia occurs prenatally then high level of circulating steroids already leads to lung maturation.

Q.255. The most common leukocytoclastic vasculitis affecting children is:
1. Takayasu disease.
2. Mucocutaneous lymph node syndrome (Kawasaki disease)
3. Henoch Schonelin purpura.
4. Polyarteritis nodosa.

255. Anderson Pathology 10th ed./1433

- Small vessel vasculitis - K/a leukocytoclastic vasculitis.
- The incidence of Henoch - Schonlein Purpura Peaks around 5 yr of age and is most common type of
vasculitis of children.
- d/d of leukocytoclastic vasculitis -
1) H.S.P.
2) Microscopic polyangitis
3) Cryoglobulinemic vasculitis
4) Wegener’s granulomatosis
5) Churg strauss syndrome
6) Drug induced vasculitis
7) Neisseria induced vasculitis.
according to Harrison 15th ed. ?
- Anaphylactoid Purpura = Henoch - Schonlein purpura.

Q.256. A four year old boy was admitted with a history of abdominal pain and fever for two months, maculopapular rash for ten days, and dry cough, dyspnea and wheezing for three days. On examination, liver and spleen were enlarged 4 cm and 3 cm respectively below the costal margins.
His hemoglobin was 10.0 g/dl, platelet count 37 x 109/L, which included 80% eosinophils. Bone marrow examination revealed a cellular marrow comprising 45% blasts and 34% eosinophils and eosinophilic precursors. The blasts stained negative for myeloperoxidase and non-specific esterase and were positive for CD19, CD10, CD22 and CD20.

Which one of the following statements in not true about this disease?
1. Eosinophils are not part of ithe neoplastic clone.
2. t(5:14) rearrangement may be detected in blasts.
3. Peripheral blood eosinophilia may normalize with chemotherapy.
4. Inv (16) is often detected in the blasts and the eosinophils.

256. William’s Hematology 6th ed/1056, 1051.
CD19, CD10, CD22 and CD20 are marker of b cell origin . so it is a lymphoid leukemia not myeloid
one.

Q.257. Kidney biopsy from a child with hemolytic uremic syndrome characteristically most likely presents features of:
1. Thrombotic microangiopathy.
2. Proliferative glomerulonephritis.
3. Focal segmental glomerulosclerosis.
4. Minimal change disease.

Q.258. The classification proposed by the International Lymphoma Study Group for non-Hodgkin’s lymphoma is known as:

1. Kiel classification.
2. REAL classification.
3. WHO classification.
4. Rappaport classification.
258. William’s hematology 6th ed/1208

- In 1993 the International Lymphoma Study Group began a year long effort to establish a new
classification the REAL classification (Revised European - American lymphoma).

Q.259. One of the intestinal enzymes that is generally deficient in children following an attack of severe infectious enteritis is:
1. Lactase.
2. Trypsin.
3. Lipase.
4. Amylase.

Q.260. A newborn presented with bloated abdomen shortly after birth with passing of less meconium. A full-thickness biopsy of the rectum was carried out. Which one of the following rectal biopsy findings is most likely to be present?
1. Fibrosis of submucosal.
2. Hyalinisation of the muscular coat.
3. Thickened muscularis propria.
4. Lack of ganglion cells.

Q.261. All of the following features are seen in the viral pneumonia except:
1. Presence of interstitial inflammation.
2. Predominance of alveolar exudates.
3. Bronchiolitis.
4. Multinucleate giant cells in the bronchiolar wall.

261. Pathologic basis of diseases Robbins ed. /751.
The term atypical pneumonia denotes the moderate amount of sputum, no physical finding of
consolidation, only moderate elevation of W.B.C. lack of alveolar exudate (answer).

Morphology of atypical pneumonia -
- Predominant interstitial nature of inflammatory reaction.
- Super imposed bacterial infection causes ulcerative bronchitis and bronchiolitis.
-Epithelial giant cells with intranuclear or intra-cytoplasmic inclusion may be present in
cytomegalic inclusion diseases, other viruses produce cytopathic changes.

Hence Answer is (2).

Q.262. Aschoff’s nodules are seen in:
1. Subacute bacterial endocarditis.
2. Libman-Sacks endocarditis.
3. Rheumatic carditis.
4. Non-bacterial thrombotic endocarditis.

262. Path. Robbins /593

Aschoff bodies - These consist of foci of swollen eosinophilic collagen surrounded by lymphocytes (Primarily T cells), occasional plasma cells, and plump macrophages called Antischkow cells (Pathognomonic for RF). These disposed in a central, slender, wavy ribbon (hence the designation “Caterpillar cells”.
Some of the larger macrophages becomes multinucleated to form aschoff giant cells.

Answer Rheumatic carditis.

Q.263. Pulmonary surfactant is secreted by:
1. Type I pneumocytes.
2. Type II pneumocytes.
3. Clara cells.
4. Bronchial epithelial cells.
263. Robbin /713.
Alveolar epithelium - contains a continuous layer of 2 principal cell types:
Flattened, plate like type I pneumocyte covering 95% of surface.
Type II - Globular, only 5% of surface area.
They are the source of pulmonary surfactant. Surfactant contained in osmiophilic lamellar bodies.

Q.264. Which one of the following conditions commonly predisposes to colonic carcinoma?
1. Ulcerative colitis.
2. Crohn’s disease.
3. Diverticular disease.
4. Ischaemic colitis.
264. Robbins /848.
- incidence of cancer of git in patients with long standing progressive C.D, with a five to six -
fold increased risk over normal population. Howerver the risk of cancer in Crohn’s disease is considerably less than in patients with chronic U.C.
Hence Answer is (1).
Ulcerative colitis
Important point to be remembered -
- Crypt abscess seen in U.C.
- Lead pipe appearance of Colon - U.C
Q.265. Fibrinoid necrosis may be observed in all of the following, except:
1. Malignant hypertension.
2. Polyarteritis nodosa.
3. Diabetic glomerulosclerosis.
4. Aschoff’s nodule.
265. Robbins 593,594, 1008, 539
- The myocardial involvement - Myocarditis - takes the form of scattered Aschoff bodies, often
perivascular. Concomitant involvement of the endocardium and the left sided valves by
inflammatory foci typically result in fibrinoid necrosis within Cusps or along the cord.

- Morphology of malignant hypertension in kidney -
- Flea bitten appearance
- Fibrinoid necrosis of arterioles
- Onion skinning of intima.
- Morphology of Polyarteritis nodosa (PAN)
- transmural inflammation of arterial wall
- fibrinoid necrosis
- fibrous thickening of vessel wall
Hence Answer is (3).

Q.266. All of the following statements are true regarding reversible cell injury, except:
1. Formation of amorphous densities in the mitochondrial matrix.
2. Diminished generation of adenosine triphosphate (ATP).
3. Formation of blebs in the plasma membrane.
4. Detachment of ribosomes from the granular endoplasmic reticulum.
266. Robbins /19

- Persistent or excessive injury causes irreversible injury.
1) Extensive damage of cellular membrane
2) Swelling of liposomes
3) Vacuolization of mitochondria with reduced capacity to generate ATP.

Note - amorphous density in mitochondrial matrix is a feature of reversible injury.
Hence Answer is (3).

Q.267. Which of the following statements pertaining to leukemia is correct?
1. Blasts of acute myeloid leukemia are typically sudan black negative.
2. Blasts of acute lymphoblastic leukemia are typically myeloperoxidase positive.
3. Low leucocyte alkaline phosphatase score is characteristically seen in blastic phase of chronic myeloid leukemia.
4. Tartarate resistant acid phosphatase positivity is typically seen in hairy cell leukemia.

.267. Wiernik Neoplastic disease of blood 3rd ed/68.
- The most consistent and diagnostic abnormality of granulocytes in CML is low or absent L.A.P.
The L.A.P is elevated in Polycythemia vera and myeloid metoplasia.

Note - The LAP tends to rise during drug induced demission of CML and in blastic phase.
- Low LAP also seen in P.N.H.
- High LAP seen in - Polycythemia vera and myeloid metaplasia.
- TRAP (Tartarate resistant acid phosphatase positivity typically seen in hairly cell leukemia.
- Blasts of AML (not ALL) - Myeloperoxidase positive
- Blasts of ALL are sudan negative.
- Another frequently asked important question -
Accelerated phase CML 1) Peripheral blast > 100,000/mm3
2) Characterized by
• Progressive splenomegaly.
• Basophilia
• Platelet count aberration, including thrombocytosis.
3) This phase usually last for 3-9 months.

Q.268. In which of the following conditions bilateral contracted kidneys are characteristically seen?
1. Amyloidosis.
2. Diabetes mellitus.
3. Rapidly progressive (crescentic) glomerulonephritis.
4. Benign nephrosclerosis.

Q.269. All of the following CSF findings are present in tuberculous meningitis, except:
1. Raised protein levels.
2. Low chloride levels
3. Cob web formation.
4. Raised sugar levels.
269. O.P. Ghai Pediatrics 5th ed/395

- Sugar level is normal or decreased in T.B.M. it is not raised in any bacterial or fungal
meningitis.
- Cob web formation in CSF is feature of T.B.M. due to excess protein within it.
- Cl decreased in csf (<600 MG/Dl)
Hence Answer is (4).

Q.270. All of the following vascular changes are observed in acute inflammation, except:
1. Vasodilation.
2. Stasis of blood.
3. Increased vascular permeability.
4. Decreased hydrostatic pressure.
270. Robbins /50

Vascular changes in acute inflammation -
1) Vasodilation is one of earliest manifestation.
2) It followed by increased vascular permeability
3) Loss of fluid result in concentration of red cells in small vessels and increased viscosity of
blood - blood flow slower - a condition term as stasis.
4) Colloid osmotic pressure is decreased.
5) While hydrostatic pressure is increased.
Hence Answer is (4).

Q.271. The subtype of Hodgkin’s disease, which is histogenetically distinct from all the other subtypes, is:
1. Lymphocyte predominant.
2. Nodular sclerosis.
3. Mixed cellularity.
4. Lymphocyte depleted.
271. Robbins /686

Now WHO Classification recognizes 5 subtype of H.L. In first 4 type (nodular sclerosis, mixed
cellularity, lymphocyte rich, and lymphocyte depletion) the Reed Sternberg Cells have a similar
immunophenotype; as these 4 are often clumped as classical form of H.L.
In lymphocyte predominance HL, Reed sternberg cells have a characteristic B cell -
immunophenotype distinct from that of ithe classical HL subtype.

Hence Answer is (1).

Q.272. In apoptosis, Apaf-1 is activated by release of which of the following substances from the mitochondria?
1. Bcl-2.
2. Bax.
3. Bcl-XI.
4. Cytochrome C.
272. Robbins /30

Cell under stress or deprived of survival signals
¯.
Release of Bcl-2 and/or Bcl - x from mitochondria
¯
Now increased permeability of mitochondria
¯
Cytochrome - C comes out of mitochondria binds to ApaF-I in cytosol.
¯
This activated complex activate the Caspase - 9 and apoptosis starts.
Hence Answer is (4).

Q.273. Which type of amyloidosis is caused by mutation of the transthyretin protein?
1. Familial Mediterranean fever.
2. Familial amyloidotic polyneuropathy.
3. Dialysis associated amyloidosis.
4. Prion protein associated amyloidosis.

Q.274. In familial Mediterranean fever, the gene encoding the following protein undergoes mutation:
1. Pyrin.
2. Perforin.
3. Atrial natriuretic factor.
4. Immunoglobulin light chain.

Q.275. Which of the following statements is not true?
1. Patients with IgD myeloma may present with no evident M-spike on serum electrophoresis.
2. A diagnosis of plasma cell leukemia can be made if circulating peripheral blood plasmablasts comprise 14% of peripheral blood white cells in a patient with white blood cell count of 11 x 109/L and platelet count of 88 x 109/L.
3. In smoldering myeloma plasma cells constitute 10-30% of total bone marrow cellularity.
4. In a patient with multiple myeloma, a monoclonal light chain may be detected in both serum and urine.
Neoplastic disease of blood 3rd ed/497-498.
Peter H, Wiernip, George P, Canellos

PLASMA CELL LEUKEMIA
Diagnosis is made when atypical plasma cells in peripheral blood at least 20% of the
differential count.

SMOLDERING MULTIPLE MYELOMA
Diagnosis depends on presence of serum M protein <30 g/L.
< 10% atypical plasma cells in bone marrow smears.
Absent of symptom, anemia and skeletal lesion.

MONOCLONAL GAMMOPATHY OF UNKNOWN SIGNIFICANCE

Circulating atypical plasma cell also < 10%.

Definition of leukemia (my) ? >20% of blast cell must be there.

Myelodysplastic syndrome - also K/a smouldering leukemia because it is having blast cells < 30%
but > 10%. So it is also K/a preleukemic leukemia.

Like smouldering leukemia the smouldering myeloma may have 10-20% plasma cells. But iwhen the
ward leukemia is used, it always means that cells are > 20% of circulating W.B.C.
- Williams hematology 6th ed/1284 ? a small proportion of patient ihave non-secretory myeloma in
which the neoplastic cells do not produce significant amount of monoclonal immunoglobulins.

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