<![CDATA[Mock 24 Sickle cell]]>Stem

A 58-year-old lady with sickle cell disease fell and sustained an injury to the parietal area. CT scan was done, SOL was found.

What is sickle cell disease?

Common hereditary hemoglobinopathy.
Caused by a point mutation in β-globin → that promotes the polymerization of deoxygenated haemoglobin.
Leading to red cell distortion, haemolytic anaemia, microvascular obstruction, and ischaemic tissue damage.
HbS is produced by the substitution of valine for glutamic acid.

How is it inherited? Name of gene responsible?

Autosomal recessive.
β-globin gene on chromosome 11.

What is the surgical concerns of sickle cell disease?

Gallstones and cholecystitis.
Avascular bone necrosis.
Bowel ischaemia.
Osteomyelitis.
Poor wound healing.
Heart failure.
Anaesthesia.

Why bone pain?

Vaso-occlusive crises triggered by infection, dehydration, acidosis, affecting the bones.

Why immunocompromised?

Because of auto-splenectomy with increased susceptibility to infection with encapsulated organisms.

What are the most common organisms causing infection after splenectomy?

Streptococcus pneumoniae
Haemophilus influenzae
Neisseria meningitidis

What do you think is this Space Occupying lesion (SOL)?

Brain tumour.

What is the most common primary brain tumour of parenchymal origin?

Gliomas and glioblastoma multiforme.
Medulloblastoma.

What other brain tumours do you know?

Meningiomas.
Acoustic neuromas.
Neurofibroma.
Pituitary tumours.
Pineal tumours.
Craniopharyngiomas.
Secondaries.

Natural history of brain tumours if left untreated

Increase ICP -> herniation of brainstem -> compression of respiratory centre -> death.

Biopsy done, shows squamous cells. What do you think?

Metastatic SCC

Where might be the primary site of SCC?

Skin
Lung
Oesophagus
Nasopharynx
Cervix

Post-op craniotomy wound is infected. What is the common causal agent?

Staph aureus

Fluid from the wound shows glucose 3.2mmol/l. What does it mean?

CSF communication

What are the functions of spleen?

Extramedullary hemopoiesis.
Removal of abnormal RBCs
Defence mechanism against incapsulated organisms
Synthesis of IG

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