PACES MRCP UK - Where MRCPians Meet Since 2006

MRCP is well establised as an entry exam for advanced specialist training in many countries including Malaysia. It consists of 3 paper i.e. Part1, Part2(written tests) and PACES. PACES in full means Practical Assessment of Clinical Examination Skills. It is the third part and the candidate is assessed by fellows of RCP. I passed my PACES in 2005. I am glad that many seniors had guided me throughout my preparation for PACES and I wish to share my experiences with PACES candidates via this blog.

Sunday, June 03, 2007

Abdomen: Thalassemia

This 20 year old young man was referred from our paediatric colleagues for further follow up and management. Please examine his abdominal system.

Suggested presentation
(When turning to the examiner)

I would like to complete my examination by examining his genitalia, doing a per rectum examination.

(If asked to present your findings)

This thin young man is pale, jaundiced, hyperpigmented and short for his age. He has frontal bossing, prominent maxillaries and chipmunk teeth which are maloccluded. There is also sparse axillary and pubic hair

The abdomen is distended and there is a left lower quadrant scar. There is hepatomegaly, extending 4 finger breadths beneath the costal margin. The liver has smooth edges and non tender on palpation. The kidneys are not ballotable. There is no shifting dullness to suggest the presence of ascites.

I did not find any lymph nodes in him. There were no features to suggest chronic liver failure. I also note he does not have any abdominal puncture wounds to suggest the use of desferral

In summary, this is short young man with jaundice, pallor, hepatomegaly, a previous splenectomy done and has thalaessaemic facies and possible underlying hypogonadism and haematochromatosis. He has chronic hemolytic aneamia. He is transfusion dependent.

I would like to offer the differential of a haemoglobinopathy. The most common haemoglobinopathy in this part of the world is Thalaessaemia.

1.What investigations would you like to do?
I would like to confirm the underlying cause of hemolytic anemia by doing serum electrophoresis. I would like assess the complications of treatment and patient’s disease by doing LFT, coagulation profile, hormonal assay, hepatitis serology and echocardiogram. I will assess his functional status using FBC.

2.How would you like to manage this young man?
a. Treatment of Disease- hypertransfusion, Splenectomy, Pneumococal vaccination, bone marrow transplant in some centres
b. Treatment of its complications-hormone replacement therapy, iron chelation therapy, control heart failure
c. Patient and family education, genetic counseling is also important so that family members can be screened and treated early.
Contributed by Giant Eagle



At 6/03/2007 09:44:00 PM, Blogger Dr. David Teoh (eMRCPian) said...

Scars in Thalassemia may be due to previous cholecystectomy or splenectomy.
Laparascopic scars may give you a small scars (Look for it carefully!!)


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