YDR 81-100BOF: 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.

On examination he is pigmented, there is finger clubbing and lymphadenopathy. On auscultation of the heart a pan-systolic murmur is heard.

Which of the following investigations would confirm your clinical diagnosis?

a)      ECHO

b)      Blood cultures

c)      Lymph node biopsy

d)      Small bowel biopsy

e)      Mesenteric angiogram

Answer:

d)

The patient has Whipple’s disease, which may be confirmed by small bowel biopsy. Small bowel biopsy will show large, foamy PAS positive macrophages in the lamina propria.

Whipple's Disease

BOF: 82

A 35-year-old male intravenous drug abuser presents to his GP with a complaint of

severe fatigue and a blistering eruption on the sun exposed areas of his skin. He also

abuses alcohol consuming between 30-40 units a week. The GP carries out blood test and

refers him for investigation of abnormal liver function tests.

In this patient:

a)      Combination therapy will induce a response in 90% of patients

b)      Development of cirrhosis does not preclude a good response to treatment

c)      If the patient develops cirrhosis he has an 80-90% chance of developing

hepatocellular carcinoma.

d)      Without treatment end stage liver failure will develop in 2-3 years

e)      In patients without cirrhosis, combination therapy induces a response in 45% of

patients

Answer:

e)

The patient has hepatitis C with associated porphyria cutanea tarda. Significant liver

disease develops in 20-30 % of patients who have necro-inflammatory disease. The

process takes 20-30 years. Development of cirrhosis results in a poor response to

combination therapy (Interferon and ribavarin) although the newer PEGylated interferons

yield a better virological response. Once cirrhosis is established the incidence of

hepatocellular cancer is 1.5-2 % per year

BOF: 83

A 50-year-old male has been seen by his GP who has made a diagnosis of diabetes mellitus. On routine investigation he has been noted to have abnormal liver function tests.

The GP refers him to the clinic querying non-alcoholic fatty liver disease. On examination he is pigmented, there is loss of body hair, gynaecomastia, testicular atrophy, and an arthropathy of his knee joints.

In this patient treatment of the underlying condition will:

a)      Increase the severity of the diabetes mellitus

b)      Increase the size of the liver

c)      Reverse any changes of cirrhosis

d)      Only results in decrease in malaise and decreases liver size

e)      Improves 5- year survival rate

Answer:

e)

The patient has haemochromatosis. Venesection is the best method of depleting body iron stores. It decreases the severity of diabetes mellitus and results in a general improvement in malaise and decreases liver size. The changes of cirrhosis are not reversible. Five-year survival has improved from 4.4 years in 1935 to 89% in 1969.

BOF: 84

A 40-year-old female presents with hepatitis. She also has arthralgia and autoimmune

thyroid disease. Investigations reveal high serum transaminases, increase in total globulin

and smooth muscle antibodies are positive.

Where treatment of this condition is concerned:

a)      Steroids are contraindicated as they increase the risk of osteoporosis

b)      Steroids reduce the enzyme levels and decrease jaundice but are of no long term

benefit

c)      Steroids improve survival rate, but do not prevent development of cirrhosis

d)      Steroids should be used continuously for 4 years

e)      Azathioprine is as effective as steroids and can be used instead of steroids in

older females

Answer:

c)

The patient has autoimmune hepatitis. In this condition response to steroids is excellent

and improves five-year survival but does not prevent development of cirrhosis.

Azathioprine is an useful adjunct to steroids and allows a lower dose of steroids to be

used and thus reduces the risk of osteoporosis. If the disease is inactive for 2 years

steroids may be cautiously withdrawn. Relapse is common (60-80 %) and necessitates

reintroduction of steroids.

BOF: 85 

A 50-year-old female presents with weakness of her left upper limb, which developed overnight. She says she fell asleep on a chair. On examination there is weakness of extension of her left elbow, a wrist drop and absent sensation over the first interosseus space of her left hand on the dorsal aspect.

The lesion is in the:

a)      Ulnar nerve at the elbow

b)      Musculocutaneous nerve

c)      Radial nerve in the spiral groove

d)      Radial nerve in the axilla

e)      Upper brachial plexus

Answer:

d)

The weakness of extension of the elbow indicates weakness of the triceps hence the lesion should be in the axilla. Lesions of the radial nerve in the spiral groove spare the triceps.

BOF: 86

A 32-year-old female who has had surgery on her cervical spine following a car accident

presents with sudden onset of left-sided weakness. The weakness developed when she

looked up whilst bending to pick up an object. On examination she has a hemiplegia

affecting the left face arm and leg. She also has unilateral internuclear ophthalmoplegia

with failure of adduction to the left and nystagmus to the left. She also has early

papilloedema. In this patient MRI shows:

a)      Right mid brain infarct

b)      Left mid brain infarct

c)      Right pontine infarct

d)      Left pontine infarct

e)      Lateral medullary syndrome

Answer:

a)

The patient has unilateral internuclear ophthalmoplegia. The side of the lesion is the side

of the failure of adduction not the side of the nystagmus. This localises the lesion to the

right medial longitudinal fasiculus. To cause weakness of face arm and leg the lesion

must be above the pons.

BOF: 87

A 32-year-old female who has had surgery on her cervical spine following a car accident presents with sudden onset of left-sided weakness. The weakness developed when she looked up whilst bending to pick up an object. On examination she has a hemiplegia affecting the left face arm and leg. She also has unilateral internuclear ophthalmoplegia with failure of adduction to the left and nystagmus to the left. She also has early papilloedema. In this patient the papilloedema is due to:

 

a)      Obstruction of the foramen of Magendie

b)      Obstruction of the foramen of Luschka

c)      Obstruction of the aqueduct of Sylvius

d)      Obstruction of the foramen of Monro

e)      Obstruction of the foramen of Morgagni

Answer:

c)

The aqueduct of the midbrain (the aqueduct of Sylvius) runs in the tegmentum of the midbrain and joins the third and fourth ventricles. Oedema around the midbrain infarct would have compressed the aqueduct and resulted in obstructive hydrocephalus and papilloedema.

BOF 88

A 57-year-old female who is known to have rheumatoid arthritis has been on

penicillamine for treatment of her condition. She presents with oedema, proteinuria,

hypoalbuminaemia and her serum cholesterol levels are elevated. In this patient renal

biopsy:

a)      Is indicated as she may have developed a vasculitis

b)      Is indicated to decide if she needs steroids and immunosuppression

c)      Should be performed in order to demonstrate minimal change nephropathy as this

will respond to steroids

d)      Is indicated to look for amyloidosis

e)      Is not indicated

Answer:

e)

The patient has nephrotic syndrome. In patients on drugs such as penicillamine it is best

to stop the drugs and assess response first, rather than proceed to renal biopsy.

In nephrotic syndrome, renal biopsy is not indicated in:

Young children, especially males, with a highly selective protein leak, no hypertension,

no red cells or red cell casts in the urine

Long standing insulin dependent diabetes mellitus with associated retinopathy or

neuropathy as here the most likely diagnosis is diabetic nephropathy

Patients on drugs, which should be stopped first

Nephrotic Syndrome

BOF: 89

A 60-year-old male with uncomplicated stable alcoholic cirrhosis presents with severe breathlessness on exertion. On examination he was found to be anaemic and jaundiced. He has splenomegaly. His Hb is 8 gm/dl with normal MCV. WBC and platelet count are normal. There is no evidence of blood loss. Iron studies and red cell folate assay are normal. What is the most likely explanation for his anaemia?

a)      Burr cell anaemia

b)      Paroxysmal cold haemoglobinuria

c)      Autoimmune haemolytic anaemia

d)      Spur cell anaemia

e)      Hypersplenism

 

Answer:

d)

Spur cell anaemia should be suspected when the anaemia is more severe than is observed in otherwise uncomplicated cirrhosis. Splenomegaly is always present. The RBC are irregularly shaped with multiple spicules. The surface membrane of a spur cell contains 50 – 70% excess cholesterol. Spur cells are distinguished from regularly spaced crenated RBC (Burr cells), which are present in some patients with uraemia.

It is not hypersplenism because his white cells and platelets are normal.

BOF: 90

Which of the following conditions is most likely to be associated with Paroxysmal Cold

Haemoglobinuria

a)      Mycoplasma infection

b)      Lymphoma

c)      Systemic lupus erythematosus

d)      Tertiary syphilis

e)      Chronic lymphocytic leukaemia

Answer:

d)

This is a rare disorder now. It was more frequent when tertiary syphilis was prevalent.

Now most cases are either secondary to a viral infection (measles and mumps in children)

or are autoimmune. Paroxysmal Cold Haemoglobinuria results from the formation of the

Donath-Landsteiner antibody, an IgG antibody that is directed against the P antigen. This

can induce complement-mediated lysis. Attacks are precipitated by exposure to cold and

are associated with haemoglobinemia and haemoglobinuria.

BOF: 90

Which of the following conditions is most likely to be associated with Paroxysmal Cold

Haemoglobinuria

a)      Mycoplasma infection

b)      Lymphoma

c)      Systemic lupus erythematosus

d)      Tertiary syphilis

e)      Chronic lymphocytic leukaemia

Answer:

d)

This is a rare disorder now. It was more frequent when tertiary syphilis was prevalent.

Now most cases are either secondary to a viral infection (measles and mumps in children)

or are autoimmune. Paroxysmal Cold Haemoglobinuria results from the formation of the

Donath-Landsteiner antibody, an IgG antibody that is directed against the P antigen. This

can induce complement-mediated lysis. Attacks are precipitated by exposure to cold and

are associated with haemoglobinemia and haemoglobinuria.

BOF: 91

A 40-year-old male was admitted with sudden onset headache and generalised tonic-clonic convulsion. MRI scan and subsequent MRI venography revealed sagittal sinus thrombosis. He recently recovered from an episode of aplastic anaemia. Investigation reveals anaemia with reticulocytosis. What is the most likely diagnosis?

 

a)      Homocystinuria

b)      Thrombotic thrombocytopaenic purpura

c)      Paroxysmal Cold Haemoglobinuria

d)      Paroxysmal Nocturnal Haemoglobinuria

e)      Thalassaemia

 

Answer:

d)

Paroxysmal Nocturnal Haemoglobinuria (PNH) is an intracorpuscular defect acquired at the stem cell level. Three common manifestations are haemolytic anaemia, venous thrombosis and deficient haematopoiesis. Granulocytopaenia 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. Venous thrombosis is a common complication of patients of European origin. Thrombosis can occur in cerebral venous sinuses and is a common cause of death in a patient with PNH.

BOF: 92

Thrombocytosis does not occur in:

a)      Essential Thrombocytosis

b)      Systemic Lupus Erythematosus

c)      Inflammatory Bowel Disease

d)      Acute Myeloid Leukaemia

e)      Hyposplenism

Answer:

d)

Acute Myeloid Leukaemia (AML) causes thrombocytopaenia. Chronic Myeloid

Leukaemia can cause thrombocytosis. Idiopathic sideroblastic anaemia and

Myelodysplasia can cause thrombocytosis. Essential thrombocytosis is a clonal disorder

of unknown aetiology and manifests clinically by the overproduction of platelets. It is

often identified incidentally. Patients with Essential Thrombocytosis do have

haemorrhagic and thrombotic tendencies

BOF: 93

Which of the following statements is not true about Waldenstrom’s Macroglobulinaemia?

a)      Hypercalcaemia is common

b)      Renal disease is not common

c)      Peripheral neuropathy is not uncommon

d)      Hepatosplenomegaly can occur

e)      Lymphadenopathy is not uncommon

 

Answer:

a)

Waldenstrom’s Macroglobulinaemia is a malignancy of lymphoplasmacytoid cells. The disease is associated with lymphadenopathy, hepatosplenomegaly and the hyperviscosity syndrome. The disease involves the bone marrow, but doesn’t cause lytic bone lesions or hypercalcaemia. IgM paraprotein has got very little excretion through urine because of its size. Therefore renal disease is not common.

BOF: 94

Alopecia is not a feature of:

a)      Secondary Syphilis

b)      Hypothyroidism

c)      Hypopituitarism 

d)      Hyperthyroidism 

e)      Hypoparathyroidism

Answer:

e)

Other systemic diseases that cause alopecia are lupus erythematosus, deficiencies of protein/iron/zinc/biotin, HIV infection

BOF: 95

Hyperpigmentation is not a manifestation of:

a)      Addison’s disease

b)      Vitamin B12 deficiency

c)      Pellagra

d)      Whipple’s disease

e)      Systemic Lupus Erythematosus

 

 

Answer:

e)

 

Scleroderma can cause hyperpigmentation. Other conditions causing pigmentation are Nelson syndrome, Porphyria Cutanea Tarda, Haemochromatosis, Folate deficiency, Malabsorption, Biliary Cirrhosis, Eosinophilia-myalgia syndrome and POEMS syndrome.

BOF: 96

The following statements about Dermatitis Herpetiformis is false:

a)      Papulovesicular lesions over the extensor surfaces are common

b)      The rash is non-pruritic

c)      Almost all patients have associated sub-clinical gluten-sensitive enteropathy

d)      IgA is deposited in the skin

e)      Increased incidence of thyroid abnormalities are found

Answer:

b)

Dermatitis Herpetiformis is intensely pruritic. Most patients with dermatitis

herpetiformis do not report overt gastrointestinal symptoms or laboratory evidence of

malabsorption. They also have increased incidence of thyroid abnormalities,

achlorhydria, atrophic gastritis and antigastric parietal cell antibody. Dapsone is the

initial treatment with institution of a gluten free diet.

BOF: 97

A 55-year-old male presents with acute bilateral visual loss. Both

the optic discs are swollen and a central scotoma is detected. In

further investigation of the cause of this condition the following

blood levels are not required:

a)      Ethylene glycol

b)      Thiamine

c)      Vit.B12

d)      Folate

e)      Vitamin A

Answer:

e)

The diagnosis is Toxic Optic Neuropathy. This can happen from exposure to ethambutol, methyl alcohol, ethylene glycol or carbon monoxide. Deficiency states induced either by starvation, malabsorption or alcoholism can lead to insidious visual loss. Thiamine, vitamin B12 and folate level should be checked in any patient with unexplained bilateral central scotoma and optic pallor

BOF: 98

Which of the following is not a clinical sequel of rhabdomyolysis:

a)      Hypovolaemia

b)      Metabolic alkalosis

c)      Hyperkalaemia

d)      Acute renal failure

e)      Disseminated intravascular coagulation

Answer:

b)

Metabolic acidosis occurs in rhabdomyolysis due to release of cellular phosphate and

sulphate

BOF 99

Which one of the following tumours, in advanced stages, is not poorly responsive to

chemotherapy?

a)      Pancreatic carcinoma

b)    Hypernephroma

c)      Gall Bladder carcinoma

d)      Ovarian carcinoma

e)      Non-small cell lung carcinoma

Answer

d)

 Other cancers that are poorly responsive to chemotherapy are thyroid carcinoma,

carcinoma of vulva, colorectal carcinoma, prostate carcinoma, melanoma and

hepatocellular carcinoma

BOF: 100

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 3 months back. Ophthalmoscopy reveals bilateral optic atrophy. What is the possible diagnosis?

 

a)      Leber’s Optic Neuropathy

b)      Central Retinal Artery Occlusion

c)      Glaucoma

d)      Central Retinal Vein Occlusion

e)      Sarcoidosis

Answer:

a) This is a disease of young men. Visual loss is painless and gradual onset. There is no treatment. Curious male predilection is a mystery