AIIMS may 2011

1. Which of the following about atherosclerosis is true?
a. Intake of unsaturated fatty acids is associated with decreased risk
b. Thoracic aorta involvement is more common and severe than abdominal aorta involvement
c. Extent of lesion in veins is same as that in arteries
d. Hypercholesterolemia does not always increase the risk of atherosclerosis per se
Ans. A Intake of unsaturated fatty acid associated with decreased risk
Ref: Harrison 17th edition page no2429

Elementary guys considering the high prevalence of atherosclerosis in india, much has been said regarding the intake of transfats and saturated fats.
Role of PUFA
• “N-3 polyunsaturated fatty acids (n-3 PUFAs) are present in high concertration in fish and in flax seeds
• The most widely used n-3 PUFAs for the treatment of hyperlipdernias are the two active molecules in fish oil: eicosapentanoic acid (EPA) and dcohexanoic acid (DHA)
• N-3 PUFAs have been concentrated-into tablets and decrease fasting triglycerides in doses of 3—4 g/d
• Fish oils can results in an increase in plasma LDL-C levels in some patients
• Fish oils supplements can be used in combination with fibrates, niacin, or stains to treat hypertriglyceridemia. In general, fish oils are well tolerated and appear to be safe, arleast at doses up to 3—4 g
• Although fish oil administration is associated with a prolongation in the bleeding time, no increased in bleeding has been seen in clinical trails
• A lower dose of omega 3 (about 1g) has been associated with reduction in cardiovascular events in CHD patients and is used by some clinicians for this purpose.”

2. A girl presented with severe hyperkalemia and peaked T waves on ECG. Most rapid way to decreased serum potassium level?
a. Calcium gluconate IV
b. Oral resins
c. Insulin + glucose
d. Sodium bicarbonate
Ans. C. Insulin + glucose
Ref: Harrison 17th e/p. 284.
Hyperkalemia is treated by following ways.
• Injection calcium gluconate – it stabilizes cardiac membrane and thus prevent arrhythmia. It does not reduce serum potassium level. It is usually given wen serum potassium level are very high
• Injection dextrose insulin drip – it is the most widely used method to reduce serum potassium level. It is the fastest method to reduce potassium level
• Bêta 2 agonist – like salbutamol & Albuterol can also reduce serum potassium level
• Potassium chelating resin- they are used orally, usually for long term use, the prevent serum potassium level to rise. Resins are slow acting used in chronic hyper kalemia
• Dialysis
• Injection NahCO3- it is used to treat sever acidosis when PH is below 7.1 acidosis is usually associated with hyperkalemia so when we treat by NahCO3, potassium level also falls. But please note NahCO3 is not used to treated hyperkalemia as such

Treatment for urgent control of hyperkalemia.

Modality Onset Duration
Calcium Gluconate 0-5 minutes 1 hour
Sodium Bicarbonate 15-30 minutes 1-2 hour
Insulin 15-30 minutes 4-6 hours
Albuterol 15-30 minutes 2-4 hours

3. A 9 yrs old girl has difficulty in combing hairs and climbing upstairs since 6 /months. She has Gower’s sign positive and maculopapular rash over metacarphalangeal joints. What should be the next appropriate investigation to be done?
a. ESR
b. RA factor
c. Creatine kinase
d. Electromyography
Ans. C. Creatine Kinase
Ref: Harrison ed. 17th pg no: 2700
In the question, as the patient is not able to comb her hair and is unable to climb up stairs it means she is suffering most likely with proximal myopathy. She also has skin lesion. Over the metacarphalangeal joints, she probably has dermatomyositis & the skin lesion seem to be Gottron’s sign
The clinically suspected diagnosis of PM, DM, or IBM is confirmed by examination the serum muscle enzymes, EMG findings and muscle biopsy (Table 383-2)

Table 383-2 (Harrison ed. 17th) Criteria for Diagnosis of inflammatory Myopathies
Criterion Definite Probable Dermatomyositis Inclusion Body Myositis
Myopathic muscle weaknessa Yes Yes Yesb Yes; slow onset, early involvement of distal muscle, frequent falls
Electromyographic findings Myopathic Myopathic Myopathic Myopathic with mixed potentials
Muscle enzymes Elevated (up to 50-fold) Elevated (up to 50-fold) Elevated (up to 50-fold) Elevated (up to 10-fold) or normal
Muscle biopsy findings “Primary” inflammation with the CD8/MHC-1 Ubiquitous MCH-I expression but minimal inflammation and no vacuoles Perifascicular perimysial, or pervascualr infiltrates, perifascicular atrophy Primary inflammation with CD8/MHC-1
Complex; vacuolated fibres with –amyloid deposits; cytochrome oxygenase-negative fibers; sings of chronic myopathy
Rash or cacinosis Absent Absent Present Absent

• ESR & RA factor would not directly help in establishing the diagnosis of inflammatory myopathy Needle
• EMG shows myopathic potentials characterized by short-duration, low-amplitude polyphasic units on voluntary activation and increased spontaneous activity with fibrillations, complex repetitive discharges, and positive sharp waves.
• These EMG findings are not diagnosis of an inflammatory myopathy but are useful to identify be presence of active or chronic myopathy and to exclude neurogenic disorders
• Muscle biopsy is the definitive test for establishing the diagnosis of inflammatory myopathy and for excluding other neuromuscular disease

Hereditary Myopathies
Muscular dystrophy refers to a group of hereditary progressive disease each within unique phenotypic and genetic features. (Table 382-5, 382-7)

Type Inheritance Onset Age Clinical Features Other Organ Systems Involved
Duchenne XR Before 5 years Progressive weakness of girdle muscle
Unable to walk after age 12 Progressive kyphoscoliosis
Respiratory failure in 2d or 3d decade Cardiomyopathy
Metal impairment

Becker XR Early childhood to early adult Progressive weakness of girdle muscles
Able to walk after age 15
Respiratory failure may develop by 4th decade Cardiomyopathy
Limb-girdle AD/AR Early childhood to early adult Slow progressive weakness of shoulder and hip girdle muscle + Cariomyopathy
Emery –Dreifuss XR/AD Childhood to adult Elbow contractures, humeral and peroneal weakness Cardiomyopathy
Congenital AR At birth or within first few months Hypotonia, contracrures delayed milestones
Progression to respiratory failure in some, static course in others CNS abnormalities (hypomyelination, malformation)
Eye abnormalities
Myotonica (DM1, DM2) AD Usually 2d decade
May be infancy if mother affected (DM1only)
Slowly progressive, weakness of face, shoulder girdle, and foot diorsiflexion
Preferential proximal weakness in DM2 Cardiac conduction defects
Mental impairment
Cataracts
Frontal baldness
Gonadal atrophy
Facisoscapulohumeral AD Before age 20 Slowly progressive weakness of face, Shoulder girdle, and foot dorsiflexion Deafness Coats (eye) disease
Oculopharyngeal AD 5th to 6th decade Slowly progressive weakness of extraocular, pharyngeal, and limb muscles s


aTwo forms of myotonic dystrophy, DM1 and DM2, have been identified. Many features overlap (see text)
Abbreviations: XR, X-linked recessive; AD, autosomal dominant; AR, autosomal recessive; CNS, central nervous system

The most sensitive enzyme is CK, which in active disease can be elevated as much as 50-fold. Although the CK level usually parallels disease activity, it is can be normal in some patients with active IBM or DM, especially when associated with a connective tissue disease. The CK is always elevated in patients with active PM. Along with the CK, the serum glutamic-oxaloacetic and glutamate pyruvate transaminases, lactate dehydrogenase, and aldolase may be elevated
EMG findings are not diagnostic of an inflammatory myopathy but are useful to identify the presence of active or chronic myopathy and to exclude neurogenic disorders:

4. 14 yrs old girl on exposure to cold has pallor of extremities followed by pain and cyanosis. In later ages of life she is prone to develop?
a. SLE
b. Scleroderma
c. Rheumatoid arthritis
d. Histiocytosis

Ans. B. Scleroderma
Ref- Harrison ed. 17th pg no. 2101
• “Raynaud’s phenomenon, defined as episodic vasoconstriction in the fingers and toes, develops in virtually every patients with Systemic sclerosis. In some, episodes may also affect the tip of the nose and earlobes
• Attacks are triggered by
o Exposure to cold
o Decrease in temperature
o Emotional stress
o Using vibration tools
• In colder climates, patients commonly experience an increase in the frequency and severity of episodes during the winter months

Table 243-1 Classification of Raynaud’s Phenomenon
Primary or idiopathic Raynaud’s phenomenon” Raynaud’s disease
Secondary Raynaud’s phenomenon
1. Collagen vascular disease : scleroderma, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, polymyositis
2. Arterial occlusive disease: atherosclerosis of the extremities, thromboangitis oblietrans, acute arterial occlusion, thoracic outlet syndrome
3. Pulmonary hypertension
4. Neurologic disorders: intervertebral disk disease, syringomyelia, spinal cord tumors, stroke, poliomyelitis, carpal tunnel syndrome
5. Blood dyscrasias: cold agglutinins, cryoglobulinemia, myeloproliferative disorders, Waldenstrom’s macroglobulinemia
6. Trauma: vibration injury, hammer hand syndrome, electric shock, cold injury, typing, piano playing
7. Drugs: ergot derivatives, methysers, Beta-adrenergic receptor blocker, bleomycin, vinblastine, cisplatin

• Typical attacks start with pallor, followed by cyanosis of variable duration. Eventually erythema develops spontaneously or with rewarming of the digit.
• The progression of the three color phases reflects the underlying path mechanisms of vasoconstriction, ischemia, and reperfusion
• Some patients with Raynaud’s phenomenon may experience only pallor or cyanosis
• In a patient of Raynaud's phenomena all arteries (like radial, brachial, popliteal, dorsalis peats, are normally palpaoie.

• Recently a new drug, Bosentan, which is a endothelin receptor blocking drug has been approve for the treatment of Raynaud's phenomenon.
• Bosentan has also approved for the treatment of primary pulmonary arterial hypertension


5. Berry aneurysm –primary Defect lies in
a. Degeneration of internal elastic lamina
b. Deposition of mucoid material in media
c. Defect in muscular layer
d. Disturbance in vessel wall
Ans. a. Degeneration of internal elastic lamina
Ref- Harrison 17h, Page 1727
• Saccular (Berry) aneurysms occur at the bifurcations of the large to medium-sized intracranial arteries; rupture is into the subarachnoid space in the basal cisterns and often into the parenchyma of the adjacent brain.
• Approximately 85% of aneurysms occur in the anterior circulation, mostly on the circle of Willis. About 20% of patients have multiple aneurysms, many at mirror sites bilaterally.
• As an aneurysm develops, it typically forms a neck with a dome. The length of the neck and the size of the dome vary greatly and are factors that are important in planning neurosurgical obliteration or endovascular embolization.
• The arterial internal elastic lamina disappears at the base of the neck.
• The media thins, and connective tissue replaces smooth-muscle cells. At the site of rupture (most often the dome) the wall thins, and the tear that allows bleeding is often <0.5 mm long.
• Aneurysm size and site are important in predicting risk of rupture.
• Those >7 mm in diameter and those at the top of the basilar artery and at the origin of the posterior communicating artery are at greater risk of rupture.

6. In which cotrimoxazole not use in treatment?
a. UTI
b. Prostatitis
c. Chancroid
d. Typhoid
Ans. C. Chancroid

Uses of cotrimoxazole
• Pneumocystis pneumonia
• Toxoplasmosis and nocardiosis .
• Acute exacerbations of chronic bronchitis and infections of the urinary tract where there is good rationale for use
• Acute otitis media in children where there is good rationale Specific indications for its use include:
HIV
Being an antibiotic, co-trimoxazole does not have any activity against HIV itself, but it is often prescribed to immunocompromised patients as Pneumocystis jirovecii'pneumonia prophylaxis.

Bacterial
• Infections caused by Listeria monocytogenes. Nocardia spp., Stenotrophomonas maitophiiia (Zanthomonas maltophilia)
• Staphylococcus saprophytics infections presenting as urinary tract infection or cystitis
• Melioidosis
• Shigellosis
• Whipple's Disease
• Traveler's Diarrhea
• Typhoid

Protozoan
• Isosporiasis
• Prophylaxis of cerebral toxoplasmosis in HIV patients
• Cyclospora cayetanensis

Chronic bacterial prostatitis treatment
• Chronic bacterial prostatitis treatment is with long-term antibiotics, *up to eight weeks, with ciprofloxacin, sulfa drugs [for example, sulfamethoxazole and trimethoprim or erythromycin
• Chancroid - Treatment regimens may include the following azithromycin, ceftriaxone, ciprofloxacin and erythromycin bas
• Typhoid fever-
1. Ciprofloxacin is the most frequently used drug.
2. Ceftriaxone, an intramuscular injection medication, is an alternative s
3. Ampicillin and trimethoprim-sulfamethoxazole are frequently prescribed antibiotics.

7. Thrombotic event is seen in all of following except
a. PNH
b. DIC
c. ITP
d. Heparin induced thrombocytopenia
Ans. C.ITP
Ref- .Harrison 17th edition Page 367
Table 59-3 Risk Factors for Thrombosis
Venous Venous and Arterial
Inherited Inherited
Factor V Leiden Homocystinuria
Prothrombin G20210A Dysfibrinogenemia
Antithrombin deficiency
Protein C deficiency Mixed (Inherited and acquired)
Protein S deficiency Hyperhomocysteinemia
Elevated FVIII
Acquired
Acquired Malignancy
Age Antiphospholipid antibody syndrome
Previous thrombosis Hormonal therapy
Immobilization Polycythemia vera
Major surgery Essential thrombocythemia
Pregnancy & puerperium Paroxysmal nocturnal hemoglobinuria
Hospitalization Thrombotic thrombocytopenic purpura
Obesity Heparin-induced thrombocytopenia
Infection Disseminated intravascular coagulation
APC resistance, nongenetic
Unknown3
Elevated factor II, IX, XI
Elevated TAFI levels
Low levels of TFPi
aUnknown whether risk is inherited or acquired. Note: APC, activated protein C; TAH. ihrombin-aciivatable fibrinolysis inhibitor; TFPI, tissue facior pathway inhibitor

8. A patient develops sudden palpitation with HR 150 / min, regular. What could be the cause?
a. PSVT
b. Sinus tachycardia
c. Ventricular tachycardia
d. Atrial flutter with block

Ans. A. PSVT

• An arrthymia in a structurally normal heart is usually a PSVT.
• Atrial flutter with block will have irregular pulse.
• VT occurs in setting of myocardial ischemia mostly and if it occurs the rates are so fast that most patients are pulseless. Even if you argue for slow VT / AIVR then the textbook states rates of 60-120bpm.
Paroxysmal supraventricular tachycardia (PSVT) is an occasional rapid heart rate.

Causes:
i. PSVT can be initiated in the SA node. In the atria, in the atrial conduction pathways, or other areas.
ii. PSVT can occur with digitalis toxicity and conditions such as Wolff-Parkinson-White syndrome.
iii. The condition occurs most often in young people and infants.

The following increase risk for PSVT:
i. Alcohol use ii. Caffeine use iii. Illicit drug use iv. Smoking

Symptoms
i. Anxiety ii. Chest tightness iii. Palpitations (a sensation of feeling the heart beat)
iv. Rapid pulse v. Shortness of breath vi. Polyuric vii. Dizziness viii. Fainting

Signs and tests
• A physical examination during a PSVT episode will show a rapid heart rate.
• The heart rate may be 150 to 250 beats per minute (bpm). In children, the heart rate tends to be very high. There may be signs of poor blood circulation such as lightheadedness. Between episodes of PSVT, the heart rate is normal (60 to 100 bpm).
• An ECG during symptoms shows PSVT.
• An electrophysiology study (EPS) is often necessary for an accurate diagnosis and to recommend the best treatment.

Treatment

The Valsalva maneuver can be used to interrupt the fast heartbeat.
Emergency treatment of PSVT may include:

1. Medicines through a vein, including adenosine and verapamil.
2. Other medications may be used, such as procainamide, beta-blockers, and propafenone.
3. Electrical cardioversion, the use of electric shock to restore a rapid heartbeat back to normal.

Long-term treatment of PSVT may include:
1. Daily medications such as propafenone, flecainide, moricizine, sotalol, and amiodarone.
2. Pacemakers to override the fast heartbeat; very occasionally used in children with PSVT who have not responded to any other treatment.
3. Radiofrequency catheter ablation; currently the treatment of choice for most PSVTs.

9. Where pulsatile liver and ascites is found
a. TR
b. Critical pulmonary stenosis
c. MR
d. MS

Ans. A. TR
Ref: Harrison, 17th edition page 1479

• For pulsatile liver, the pathology has to be in mainly the right side of the heart. So we can easily eliminate choices c and choice d.
• In pulmonic stenosis of critical levels, the pulmonary blood flow would be so compromised that cyanosis would ensue

• The clinical features of TR result primarily from systemic venous congestion and reduction of CO.
• With the onset of TR in patients with pulmonary hypertension, symptoms of pulmonary congestion diminish, but the clinical manifestations of right-sided heart failure become intensified.
• The neck veins are distended with prominent v waves and rapid y descents, marked hepatomegaly, ascites, pleural effusions, edema, systolic pulsations of the liver, and a positive hepatojugular reflux.
• A prominent RV pulsation along the left parasternal region and a blowing holosystolic murmur along the lower left sternal margin, which may be intensified during inspiration and reduced during expiration or the strain of the Valsalva maneuver (Carvallo's sign), are characteristic findings;
• AF is usually present.

10. In renal cell carcinoma which is not associated :
a. Polycythemia
b. Amyloidosis
c. Cushing syndrome
d. Malignant hypertension

Ans. C. Cushing syndrome Ref-Harrison 17th/e p.2254, 592.

• RCC para-neoplastic manifestations secondary to production of renin and erythropoietin explains polycyathemia and hypertension.
• The presenting signs and symptoms include hematuria, abdominal pain, and a flank or abdominal mass.
• This classic triad occurs in 10-20% of patients. Other symptoms are fever, weight loss, anemia, and a varicocele (Tabie 90-4).
• The tumor can also be found incidentally on a radiograph. Widespread use of radiologic cross-sectional imaging procedures (CT, ultrasound, MRI) contributes to earlier detection, including incidental renal masses detected during evaluation for other medical conditions.
• The increasing number of incidentally discovered low-stage tumors has contributed to an improved 5-year survival for patients with renal cell carcinoma and increased use of nephron-sparing surgery (partial nephrectomy).
• A spectrum of paraneoplastic syndromes has been associated with these malignancies, including erythrocytosis, hypercalcemia, non-metastatic hepatic dysfunction (Stauffer syndrome), and acquired dysfibrinogenemia.
• Erythrocytosis is noted at presentation in only about 3% of patients. Anemia, a sign of advanced disease, is more common.

Table 90-4 Signs and Symptoms in Patients with Renal Cell Cancer

Presenting Sign or Symptom Incidence %
Classic triad: hematuria, flank pain, flank mass 10-20
Hematuria 40
Flank pain 40
Palpable mass 25
Weight loss 33
Anemia 33
Fever 20
Hypertension 20
Abnormal liver function 15
Hypercalcemia 5
Erythrocytosis 3
Neuromyopathy 3
Amyloidosis 2
Increased erythrocyte sedimentation rate 55

11. Cause of alpha thalassemia
a. Deletion of alpha genes
B. Deletion of beta genes
C. Excess of alpha gene
D. Single amino acid substitution in alpha chain
Ans A. Deletion of alpha genes
Ref: Harrison 17th e/p.641.
The four classic alpha thalassemias, most common in Asians, are thalassemia alpha -2 trait, in which one of the four -globin loci is deleted; alpha -thalassemia-1 trait, with two deleted loci; HbH disease, with three loci deleted; and hydrops fetalis with Hb Bart's, with all four loci deleted.

Table 99-4 The alpha Thalassemias
Condition Hemoglobin A,
% Hemoglobin H (p4), % Hemoglobin Level, g/L (g/dL) MCV, fL
Normal 97 0 150(15) 90
Silent thalassemia: -a/aa 98-100 0 150(15) 90
Thalassemia trait: -a/-ochomozygous a-thal-2* or - -/aaheterozygous a-thal-1a 85-95 Rare red blood cell inclusions 120-130(12-13) 70-80
Hemoglobin H disease: --/-aheterozygous a-thal-1/a-thai-2 70-95
* 5-30 60-100 (6-10) 60-70
Hydrops fetalis: - -/- - homozygous a-thal-1 0 S-10D Fatal in utero or at birth

12. Reperfusion is useful for
a. Stunt myocardium
c. Non ischemic viable myocardium
b. Hibernating myocardium
d. Mixed ischemic myocardium

Ans. B. Hibernating myocardium
• "LV dysfunction can be due to non contractile or hypo contractile segments that are viable but are chronically ischemic (hibernating myocardium).
• As a consequence of chronic reduction in myocardial blood flow these segments down regulate their contractile function.
• These can be detected by using consequence of chronic reduction in myocardial blood flow these segments down regulate their contractile function.
• These can be detected by using radionuclide scans of myocardial perfusion and metabolism, PET, CMR imaging, or delayed scanning with thallium-20 I; or by improvement of regional functional impairment, provoked by low-dose dobutamine.
• " In such patients, revascularization improves myocardial blood flow, can return function., and can improve survival.,:
• Stunned myocardium can be differentiated from hibernating myocardium by three clinical parameters, namely, LV wall motion, myocardial perfusion, and myocardial metabolism. b
• Stunned myocardium has abnormal wall motion that tends to normalize in response to inotrope and postextrasystolic potentiation.
• Perfusion is adequate and metabolism is alsQ adequate. Hibernating myocardium also has abnormal wall motion, which normalizes after nitrates, inotrope, post extrasystolic potentiation (PESP), PTCA, or CABG.
• Myocardial perfusion is reduced but can be reversed with PTCA or CABG and metabolism is adequate.
e: Recent Advances:
Following are the methods to reperfuse the myocardium
a. Thrombolytic therapy
b. Mechanical method:
i. PTCA
ii. Stent:
iii. Rotablator
iv. LASER
c. Coronary artery bypass graft (CABG)

13. Positive hepatojugular reflux is found in all of the following conditions except?
a. Tricuspid regurgitation
b. Right heart failure
c. Decreased after load
d. Increased pulmonary capillary bed pressure
Ans. C. Decreased after load
Ret: Harrison, 17th edition, page 1384
• In patients suspected of having right ventricular failure who have a normal CVP at rest, the abdominojugular reflux test may be helpful.
• The palm of the examiner's hand is placed over the abdomen, and firm pressure is applied for 10 s or more. In normal persons, this maneuver does not alter the jugular venous pressure significantly but when right heart function is impaired, the upper level of venous pulsation usually increases.
• A positive abdominojugular test is best defined as an increase in JVP during 10 s of firm midabdominal compression followed by a rapid drop in pressure of 4 cm blood on release of the compression.

a. The most common cause of a positive test is right-sided heart failure secondary to elevated left heart filling pressures.
b. Increase after load is associated with LVF. That is why ACEI are used in the treatment of LVF (To reduce after load)


14. Gold standard test for diagnosis of insulinoma is
A. 72 hour fast test
B. Plasma glucose levels < 3 mmol/l
C. Plasma insulin levels > 6µU/ml ]
D. C-peptide levels < 50 p/mol/lit
Answer is A (72 hour' fast test)-
Ref: Harrison's 17h/2354. 2355

Diagnosis
• The diagnosis of insulinoma requires the demonstration of an elevated. Plasma insulin level at the time of hypoglycemia.
• A number of other conditions may cause fasting hypoglycemia, such as the inadvertent or surreptitious use of insulin or oral hypoglycemic agents, severe liver disease, alcoholism, poor nutrition, or other extrapancreatic tumors.
• The most reliable test to diagnose insulinoma is a fast up to 72 h with serum glucose, C-peptide, and insulin measurements every 4-8 h. If at any point the patient becomes symptomatic or glucose levels are persistently <2.2 mrnol/L (40 mg/dL), the test should be terminated and repeat samples for the above studies obtained before glucose is given.
• Some 70-80% of patients will develop hypoglycemia during the first 24 h and 98% by 48 h. In nonobese normal subjects, serum insulin levels should decrease to <43 pmol/L (<6 uU/mL) when blood glucose decreases to <2.2 mmol/L (<40 mg/dL) and the ratio of insulin to glucose is <0.3 (in mg/dL).
• In addition to having an insulin level >6 uU/mL when blood glucose is -:-40 mgydL, some investigators also require an elevated C-peptide and serum proinsulin level an insulin/glucose ratio >0.3: and a decreased plasma 8-hydroxybtityrate level for the diagnosis of insulinomas.
• Surreptitious use of insulin or hypoglycemic agents may be difficult to distinguish from insulinomas.
• The combination of proinsulin levels (normal in exogenous insulin/hypogiycemic agent users), C-peptide levels (low in exogenous insulin users), antibodies to insulin (positive in exogenous insulin users), and measurement of sulfonylurea levels in serum or plasma will allow the correct diagnosis to be made.
• The diagnosis of insulinoma has been complicated by the introduction of specific insulin assays that do not also interact with proinsulin. as do many of the older radioimmunoassays (RIAs), and therefore give lower plasma insulin levels.
• The increased use of these specific insulin assays has resulted in increased numbers of patients with insulinomas having lower plasma insulin values than the 43 pmol/L (6 uU/mL) levels proposed to be characteristic of insulinomas by RIA.
• In these patients the assessment of proinsulin and C-peptide levels at the time of hypoglycemia are particularly helpful for establishing the correct diagnosis.

15. Insuiin + acarbose given to patient and hypoglycemia seen. What is the treatment?
A. Sucrose
B. Maltase
C. Glucose
D. Galactose

Ans. C. Glucose
Ref: Harrison 17th e/p.2310.
When acarbose used in combination with insulin or other insulin secretagogues (Sulfonylureas or repaglinide), hypoglycemia may occur.
When used as monotherapy, acarbose and miglitol do not enhance insulin secretion and hence in overdose do not cause hypoglycemia .

Treatment
• Hypoglycemia associated with the use of acarbose or miglitol plus insulin or a insulin secretagogue should be treated with oral glucose (dextrose) and not sucrose or other complex carbohydrates, which may be ineffective.
• The hydrolysis of sucrose (can sugar) to fructose and glucose is inhibited by acarbose and thus products containing sucrose are unsuitable for the rapid correction of hypoglycemia.
• Patient should be aware of the need to have a readily available source of glucose (dextrose^ d-glucose) to treat hypoglycemic episodes.
• In server hypoglycemia, intravenous dextrose or glucagon injections may be required.

16. In a child with respiratory distress, failure to thrive. His sweat chloride levels were estimated 35 & 41. What is next best test to do cystic fibrosis for diagnosis?
a. Nasal transmembrane potential
b. DNA analysis A 508 mutation
c. CT chest
d. 72 hour fecal fat estimation
Ans. A. Nasal transmembrane potential
Ref: Nelson 1&h edition, chapter 400
Diagnosis And Assessment.

The diagnosis of CF has been based on a positive quantitative sweat test (CI- > 60 mEq/L) in conjunction with 1 or more of the following: typical chronic obstructive pulmonary disease, documented exocrine pancreatic insufficiency, or a positive family history. Diagnostic criteria have been recommended to include additional testing procedures (Table 400-3).

TABLE 400 - 3. Diagnostic Criteria for Cystic Fibrosis (CF)
Presence of typical clinical features (respiratory, gastrointestinal, or genitourinary)
OR
A history of CF in a sibling
OR
A positive newborn screening test
PLUS
Laboratory evidence for CFTR dysfunction:
Two elevated sweat chloride concentrations obtained on separate days
OR
Identification of two CF mutations
OR
An abnormal nasal potential difference measurement.

CFTR, CF transmembrane regulator

Abnormal nasal potential difference measurement
The finding of increased potential differences across nasal epithelium, the loss of this difference with topical amiloride application, and the absence of a voltage response to a ^-adrenergic agonist have been used to confirm the diagnosis in patients with equivocal or frankly normal sweat chloride values. Failure to sweat when a combination of isoproterenol and atropine is injected into the skin has also been used to characterize CF variants.

Radiology:
Pulmonary radiologic findings suggest the diagnosis but are not specific.

DNA analysis A 508 mutation
Several commercial laboratories test for 30-80 of the most common CFTR mutations. This testing identifies >90% individuals who carry 2 CF mutations.

Pancreatic Function.
Exocrine pancreatic dysfunction is clinically apparent in many patients. Documentation is desirable if there are questions about the functional status of the pancreas. Measurement of fat balances with a 3 day stool collection or direct documentation of enzyme secretion after duodenal intubation and pancreo-zymin-secretin stimulation provides a reliable measure, but these methods are cumbersome or invasive for children and are not routinely used.

17. Death in acute Ml with thrombolytic therapy is due to?
A. Intracranial hemorrage
B. Vasospasm
C. Hydrocephalus
D. Hyponateremia
Ans. A. Intracranial hemorrage
Ref: Harrison, 17th edition, chapter no 1538

Complications of Thrombolytic therapy
• Allergic reactions to streptokinase occur in -2% of patients who receive it.
• Hypotension While a minor degree of hypotension occurs in 4-10% of patients given this agent, marked hypotension occurs, although rarely, in association with severe allergic reactions.
• Hemorrhage is the most frequent and potentially the most serious complication. Because bleeding episodes that - require transfusion are more common when patients require invasive procedures, unnecessary venous or arterial interventions should be avoided in patients receiving fibrinolytic agents.
• Hemorrhagic stroke is the most serious complication and occurs in -0.5-0.9% of patients being treated with these agents.
• This rate increases with advancing age, with patients >70 years experiencing roughly twice the rate of intracranial hemorrhage as those <65 years.

18. Octreotide is not useful for?
A. Insulinoma
B. Glucagonoma
C. Carcinoid tumor
D. Glioma
Ans. D. Glioma
Octreotide is an octapeptide that mimics natural somatostatin pharmacologically, though it is a more potent inhibitor fo growth hormone, glucagon, and insulin than the natural hormone.

Uses of Octreotide:
1. Acromegaly /. Carcinoid/Carcinoid syndrome/
3. Diarrhea secretory
4. Dumping syndrome,
5. Glucagonoma
6. Hepatorenal syndrome
7. insulinoma
8. Intestinal obstructive
9. Metastatic neuroendocrine tumor
10. Nausea and vomiting
11. Nonfunctioning pituitary tumor
12. Oncogenic osteomalacia
13. Orthostatic hypotension
14. Pancreatitis
15. Thymoma,
16. TSH-secreting adenomas
17. Variceal bleeding
18. VIPoma
19. Zollinger-Ellison syndrome