YDR 81-100

BOF: 81
A 55-year-old Caucasian male presents with a 2-year history of arthritis, fever, recurrent
cough and chest pain. He has been feeling generally unwell. Recently he has developed
diarrhoea steatorrhoea!, abdominal pain and weight loss.
"n e#amination he is pigmented, there is finger clubbing and lymphadenopathy. "n
auscultation of the heart a pan-systolic murmur is heard.
$hich of the following investigations would confirm your clinical diagnosis%
a! &CH"
b! 'lood cultures
c! (ymph node biopsy
d! )mall bowel biopsy
e! *esenteric angiogram
Answer+
d!
,he patient has $hipple-s disease, which may be confirmed by small bowel biopsy.
)mall bowel biopsy will show large, foamy .A) positive macrophages in the lamina
propria.
Whipple's Disease
'"/+ 02
A 15-year-old male intravenous drug abuser presents to his 2. with a complaint of severe
fatigue and a blistering eruption on the sun e#posed areas of his s3in. He also abuses
alcohol consuming between 14-54 units a wee3. ,he 2. carries out blood test and refers
him for investigation of abnormal liver function tests.
6n this patient+
a! Combination therapy will induce a response in 748 of patients
b! 9evelopment of cirrhosis does not preclude a good response to treatment
c! 6f the patient develops cirrhosis he has an 04-748 chance of developing
hepatocellular carcinoma.
d! $ithout treatment end stage liver failure will develop in 2-1 years
e! 6n patients without cirrhosis, combination therapy induces a response in 558 of
patients
Answer+
e!
,he patient has hepatitis C with associated porphyria cutanea tarda. )ignificant liver
disease develops in 24-14 8 of patients who have necro-inflammatory disease. ,he
process ta3es 24-14 years. 9evelopment of cirrhosis results in a poor response to
combination therapy 6nterferon and ribavarin! although the newer .&2ylated interferons
yield a better virological response. "nce cirrhosis is established the incidence of
hepatocellular cancer is :.5-2 8 per year
'"/+ 01
A 54-year-old male has been seen by his 2. who has made a diagnosis of diabetes
mellitus. "n routine investigation he has been noted to have abnormal liver function tests.
,he 2. refers him to the clinic ;uerying non-alcoholic fatty liver disease. "n
e#amination he is pigmented, there is loss of body hair, gynaecomastia, testicular atrophy,
and an arthropathy of his 3nee <oints.
6n this patient treatment of the underlying condition will+
a! 6ncrease the severity of the diabetes mellitus
b! 6ncrease the si=e of the liver
c! Reverse any changes of cirrhosis
d! "nly results in decrease in malaise and decreases liver si=e
e! 6mproves 5- year survival rate
Answer+
e!
,he patient has haemochromatosis. >enesection is the best method of depleting body iron
stores. 6t decreases the severity of diabetes mellitus and results in a general improvement
in malaise and decreases liver si=e. ,he changes of cirrhosis are not reversible. /ive-year
survival has improved from 5.5 years in :715 to 078 in :7?7.
BOF: 84
A 54-year-old female presents with hepatitis. )he also has arthralgia and autoimmune
thyroid disease. 6nvestigations reveal high serum transaminases, increase in total globulin
and smooth muscle antibodies are positive.
$here treatment of this condition is concerned+
a! )teroids are contraindicated as they increase the ris3 of osteoporosis
b! )teroids reduce the en=yme levels and decrease <aundice but are of no long term
benefit
c! )teroids improve survival rate, but do not prevent development of cirrhosis
d! )teroids should be used continuously for 5 years
e! A=athioprine is as effective as steroids and can be used instead of steroids in
older females
Answer+
c!
,he patient has autoimmune hepatitis. 6n this condition response to steroids is e#cellent
and improves five-year survival but does not prevent development of cirrhosis.
A=athioprine is an useful ad<unct to steroids and allows a lower dose of steroids to be
used and thus reduces the ris3 of osteoporosis. 6f the disease is inactive for 2 years
steroids may be cautiously withdrawn. Relapse is common ?4-04 8! and necessitates
reintroduction of steroids.
BOF: 85

A 54-year-old female presents with wea3ness of her left upper limb, which developed
overnight. )he says she fell asleep on a chair. "n e#amination there is wea3ness of
e#tension of her left elbow, a wrist drop and absent sensation over the first interosseus
space of her left hand on the dorsal aspect.
,he lesion is in the+
a! @lnar nerve at the elbow
b! *usculocutaneous nerve
c! Radial nerve in the spiral groove
d! Radial nerve in the a#illa
e! @pper brachial ple#us
Answer+
d!
,he wea3ness of e#tension of the elbow indicates wea3ness of the triceps hence the
lesion should be in the a#illa. (esions of the radial nerve in the spiral groove spare the
triceps.
'"/+ 0?
A 12-year-old female who has had surgery on her cervical spine following a car accident
presents with sudden onset of left-sided wea3ness. ,he wea3ness developed when she
loo3ed up whilst bending to pic3 up an ob<ect. "n e#amination she has a hemiplegia
affecting the left face arm and leg. )he also has unilateral internuclear ophthalmoplegia
with failure of adduction to the left and nystagmus to the left. )he also has early
papilloedema. 6n this patient *R6 shows+
a! Right mid brain infarct
b! (eft mid brain infarct
c! Right pontine infarct
d! (eft pontine infarct
e! (ateral medullary syndrome
Answer+
a!
,he patient has unilateral internuclear ophthalmoplegia. ,he side of the lesion is the side
of the failure of adduction not the side of the nystagmus. ,his localises the lesion to the
right medial longitudinal fasiculus. ,o cause wea3ness of face arm and leg the lesion
must be above the pons.
BOF: 87
A 12-year-old female who has had surgery on her cervical spine following a car accident
presents with sudden onset of left-sided wea3ness. ,he wea3ness developed when she
loo3ed up whilst bending to pic3 up an ob<ect. "n e#amination she has a hemiplegia
affecting the left face arm and leg. )he also has unilateral internuclear ophthalmoplegia
with failure of adduction to the left and nystagmus to the left. )he also has early
papilloedema. 6n this patient the papilloedema is due to+

a! "bstruction of the foramen of *agendie
b! "bstruction of the foramen of (usch3a
c! "bstruction of the a;ueduct of )ylvius
d! "bstruction of the foramen of *onro
e! "bstruction of the foramen of *orgagni
Answer+
c!
,he a;ueduct of the midbrain the a;ueduct of )ylvius! runs in the tegmentum of the
midbrain and <oins the third and fourth ventricles. "edema around the midbrain infarct
would have compressed the a;ueduct and resulted in obstructive hydrocephalus and
papilloedema.
'"/ 00
A 5A-year-old female who is 3nown to have rheumatoid arthritis has been on
penicillamine for treatment of her condition. )he presents with oedema, proteinuria,
hypoalbuminaemia and her serum cholesterol levels are elevated. 6n this patient renal
biopsy+
a! 6s indicated as she may have developed a vasculitis
b! 6s indicated to decide if she needs steroids and immunosuppression
c! )hould be performed in order to demonstrate minimal change nephropathy as this
will respond to steroids
d! 6s indicated to loo3 for amyloidosis
e! 6s not indicated
Answer+
e!
,he patient has nephrotic syndrome. 6n patients on drugs such as penicillamine it is best
to stop the drugs and assess response first, rather than proceed to renal biopsy.
6n nephrotic syndrome, renal biopsy is not indicated in+
Boung children, especially males, with a highly selective protein lea3, no hypertension,
no red cells or red cell casts in the urine
(ong standing insulin dependent diabetes mellitus with associated retinopathy or
neuropathy as here the most li3ely diagnosis is diabetic nephropathy
.atients on drugs, which should be stopped first
Cephrotic )yndrome
'"/+ 07
A ?4-year-old male with uncomplicated stable alcoholic cirrhosis presents with severe
breathlessness on e#ertion. "n e#amination he was found to be anaemic and <aundiced.
He has splenomegaly. His Hb is 0 gmDdl with normal *C>. $'C and platelet count are
normal. ,here is no evidence of blood loss. 6ron studies and red cell folate assay are
normal. $hat is the most li3ely e#planation for his anaemia%
a! 'urr cell anaemia
b! .aro#ysmal cold haemoglobinuria
c! Autoimmune haemolytic anaemia
d! )pur cell anaemia
e! Hypersplenism

Answer+
d!
)pur cell anaemia should be suspected when the anaemia is more severe than is observed
in otherwise uncomplicated cirrhosis. )plenomegaly is always present. ,he R'C are
irregularly shaped with multiple spicules. ,he surface membrane of a spur cell contains
54 E A48 e#cess cholesterol. )pur cells are distinguished from regularly spaced crenated
R'C 'urr cells!, which are present in some patients with uraemia.
6t is not hypersplenism because his white cells and platelets are normal.
'"/+ 74
$hich of the following conditions is most li3ely to be associated with .aro#ysmal Cold
Haemoglobinuria
a! *ycoplasma infection
b! (ymphoma
c! )ystemic lupus erythematosus
d! ,ertiary syphilis
e! Chronic lymphocytic leu3aemia
Answer:
d!
,his is a rare disorder now. 6t was more fre;uent when tertiary syphilis was prevalent.
Cow most cases are either secondary to a viral infection measles and mumps in children!
or are autoimmune. .aro#ysmal Cold Haemoglobinuria results from the formation of the
9onath-(andsteiner antibody, an 6g2 antibody that is directed against the . antigen. ,his
can induce complement-mediated lysis. Attac3s are precipitated by e#posure to cold and
are associated with haemoglobinemia and haemoglobinuria.
'"/+ 74
$hich of the following conditions is most li3ely to be associated with .aro#ysmal Cold
Haemoglobinuria
a! *ycoplasma infection
b! (ymphoma
c! )ystemic lupus erythematosus
d! ,ertiary syphilis
e! Chronic lymphocytic leu3aemia
Answer:
d!
,his is a rare disorder now. 6t was more fre;uent when tertiary syphilis was prevalent.
Cow most cases are either secondary to a viral infection measles and mumps in children!
or are autoimmune. .aro#ysmal Cold Haemoglobinuria results from the formation of the
9onath-(andsteiner antibody, an 6g2 antibody that is directed against the . antigen. ,his
can induce complement-mediated lysis. Attac3s are precipitated by e#posure to cold and
are associated with haemoglobinemia and haemoglobinuria.
'"/+ 7:
A 54-year-old male was admitted with sudden onset headache and generalised tonic-
clonic convulsion. *R6 scan and subse;uent *R6 venography revealed sagittal sinus
thrombosis. He recently recovered from an episode of aplastic anaemia. 6nvestigation
reveals anaemia with reticulocytosis. $hat is the most li3ely diagnosis%

a! Homocystinuria
b! ,hrombotic thrombocytopaenic purpura
c! .aro#ysmal Cold Haemoglobinuria
d! .aro#ysmal Cocturnal Haemoglobinuria
e! ,halassaemia

Answer+
d!
.aro#ysmal Cocturnal Haemoglobinuria .CH! is an intracorpuscular defect ac;uired at
the stem cell level. ,hree common manifestations are haemolytic anaemia, venous
thrombosis and deficient haematopoiesis. 2ranulocytopaenia and thrombocytopaenia are
common and reflect deficient haematopoiesis. Clinical haemoglobinuria is intermittent in
most patients and never occurs in some, but haemosiderinuria is usually present. >enous
thrombosis is a common complication of patients of &uropean origin. ,hrombosis can
occur in cerebral venous sinuses and is a common cause of death in a patient with .CH.
'"/+ 72
,hrombocytosis does not occur in+
a! &ssential ,hrombocytosis
b! )ystemic (upus &rythematosus
c! 6nflammatory 'owel 9isease
d! Acute *yeloid (eu3aemia
e! Hyposplenism
Answer+
d!
Acute *yeloid (eu3aemia A*(! causes thrombocytopaenia. Chronic *yeloid
(eu3aemia can cause thrombocytosis. 6diopathic sideroblastic anaemia and
*yelodysplasia can cause thrombocytosis. &ssential thrombocytosis is a clonal disorder
of un3nown aetiology and manifests clinically by the overproduction of platelets. 6t is
often identified incidentally. .atients with &ssential ,hrombocytosis do have
haemorrhagic and thrombotic tendencies
BOF: 93
Which ! "he !llwin# s"a"e$en"s is n" "r%e a&%" Wal'ens"r$(s
)acr#l&%linae$ia*
a! Hypercalcaemia is common
b! Renal disease is not common
c! .eripheral neuropathy is not uncommon
d! Hepatosplenomegaly can occur
e! (ymphadenopathy is not uncommon

Answer:
a!
Wal'ens"r$(s )acr#l&%linae$ia is a $ali#nanc+ ! l+$phplas$ac+"i'
cells, -he 'isease is asscia"e' wi"h l+$pha'enpa"h+. hepa"splen$e#al+
an' "he h+per/iscsi"+ s+n'r$e, -he 'isease in/l/es "he &ne $arrw. &%"
'esn(" ca%se l+"ic &ne lesins r h+percalcae$ia, 0#) parapr"ein has #"
/er+ li""le e1cre"in "hr%#h %rine &eca%se ! i"s si2e, -here!re renal 'isease
is n" c$$n,
'"/+ 75
Alpecia is n" a !ea"%re !:
a3 4ecn'ar+ 4+philis
&3 5+p"h+ri'is$
c3 5+ppi"%i"aris$
'3 5+per"h+ri'is$
e3 5+ppara"h+ri'is$
Answer+
e!
"ther systemic diseases that cause alopecia are lupus erythematosus, deficiencies of
proteinDironD=incDbiotin, H6> infection
'"/+ 75
Hyperpigmentation is not a manifestation of+
a! Addison-s disease
b! >itamin ':2 deficiency
c! .ellagra
d! $hipple-s disease
e! )ystemic (upus &rythematosus


Answer:
e)

)cleroderma can cause hyperpigmentation. "ther conditions causing pigmentation are
Celson syndrome, .orphyria Cutanea ,arda, Haemochromatosis, /olate deficiency,
*alabsorption, 'iliary Cirrhosis, &osinophilia-myalgia syndrome and ."&*)
syndrome.
BOF: 96
,he following statements about 9ermatitis Herpetiformis is false+
a! .apulovesicular lesions over the e#tensor surfaces are common
b! ,he rash is non-pruritic
c! Almost all patients have associated sub-clinical gluten-sensitive enteropathy
d! 6gA is deposited in the s3in
e! 6ncreased incidence of thyroid abnormalities are found
Answer+
b!
9ermatitis Herpetiformis is intensely pruritic. *ost patients with dermatitis
herpetiformis do not report overt gastrointestinal symptoms or laboratory evidence of
malabsorption. ,hey also have increased incidence of thyroid abnormalities,
achlorhydria, atrophic gastritis and antigastric parietal cell antibody. 9apsone is the
initial treatment with institution of a gluten free diet.
BOF: 97
A 55-+ear-l' $ale presen"s wi"h ac%"e &ila"eral /is%al lss, B"h "he p"ic
'iscs are swllen an' a cen"ral sc"$a is 'e"ec"e', 0n !%r"her in/es"i#a"in
! "he ca%se ! "his cn'i"in "he !llwin# &l' le/els are n" re7%ire':
a! &thylene glycol
b! ,hiamine
c! >it.':2
d! /olate
e! >itamin A
Answer+
e!
-he 'ia#nsis is -1ic Op"ic 8e%rpa"h+, -his can happen !r$ e1ps%re "
e"ha$&%"l. $e"h+l alchl. e"h+lene #l+cl r car&n $n1i'e, De!icienc+
s"a"es in'%ce' ei"her &+ s"ar/a"in. $ala&srp"in r alchlis$ can lea' "
insi'i%s /is%al lss, -hia$ine. /i"a$in B
19
an' !la"e le/el sh%l' &e chec:e'
in an+ pa"ien" wi"h %ne1plaine' &ila"eral cen"ral sc"$a an' p"ic pallr
'"/+ 70
$hich of the following is not a clinical se;uel of rhabdomyolysis+
a) Hypovolaemia
b) Metabolic alkalosis
c) Hyperkalaemia
d) Acute renal failure
e) Disseminated intravascular coagulation
Answer+
b!
*etabolic acidosis occurs in rhabdomyolysis due to release of cellular phosphate and
sulphate
'"/ 77
$hich one of the following tumours, in advanced stages, is not poorly responsive to
chemotherapy%
a! .ancreatic carcinoma
b! Hypernephroma
c! 2all 'ladder carcinoma
d! "varian carcinoma
e! Con-small cell lung carcinoma
Answer
d!
"ther cancers that are poorly responsive to chemotherapy are thyroid carcinoma,
carcinoma of vulva, colorectal carcinoma, prostate carcinoma, melanoma and
hepatocellular carcinoma
'"/+ :44
A 22-year-old male presents with gradual onset loss of central visual field in the right
eye. He also had the same process in the left eye 1 months bac3. "phthalmoscopy reveals
bilateral optic atrophy. $hat is the possible diagnosis%

a! (eber-s "ptic Ceuropathy
b! Central Retinal Artery "cclusion
c! 2laucoma
d! Central Retinal >ein "cclusion
e! )arcoidosis
Answer:
a)
-his is a 'isease ! +%n# $en, ;is%al lss is painless an' #ra'%al nse", -here
is n "rea"$en", <%ri%s $ale pre'ilec"in is a $+s"er+