Match List I with List II and select the correct answer using the code given below the Lists :
List I
(Observed associations)List II
(Syndromes)A. Turcot syndrome 1. Café-au-lait spots B. Neurofibromatosis II 2. Desmoid tumours C. Neurofibromatosis I 3. Bilateral schwannomas D. Gardner syndrome 4. CNS tumours
Code :
| A | B | C | D |
| 4 | 3 | 1 | 2 |
This question requires matching specific syndromes from List I with their characteristic observed associations in List II.
The correct matching based on established medical knowledge is:
| List I (Syndromes) | List II (Observed associations) |
| A. Turcot syndrome | 4. CNS tumours |
| B. Neurofibromatosis II | 3. Bilateral schwannomas |
| C. Neurofibromatosis I | 1. Café-au-lait spots |
| D. Gardner syndrome | 2. Desmoid tumours |
Turcot syndrome (A) is a rare genetic disorder characterized by the presence of brain and spinal cord tumors (CNS tumours).
Neurofibromatosis II (B) is primarily associated with the development of tumors on nerve tissue, notably Bilateral schwannomas (tumors of the Schwann cell sheath), often affecting the auditory nerves.
Neurofibromatosis I (C) is distinguished by multiple neurofibromas and specific skin findings, including Café-au-lait spots.
Gardner syndrome, a subtype of familial adenomatous polyposis (FAP), is linked to gastrointestinal polyps, osteomas, and soft tissue tumors, including Desmoid tumours.
Therefore, the correct code representing the matches A-4, B-3, C-1, and D-2 is selected.
Which of the following are correct regarding Li-Fraumeni syndrome?
1. It has autosomal dominant inheritance and is associated with P53 gene.
2. It has autosomal recessive inheritance and is associated with P53 gene.
3. It is associated with an increased risk of sarcomas and leukaemia.
4. It is associated with an increased risk of brain tumours and osteo- sarcomas.
Select the answer using the code given below.