102, FORENSIC 104. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS 204° Answer is D (It can be safely administered with any nephrotoxic drug): Katcung 9/941, 942 Toxic effects of Tacrolimus are similar to cyclosporine and include nephrotoxity. It thus cannot be safety ‘administered with any nephrotoxic drug. Tacrolimus: (FK 506) Tacrolimus isan immunosupressant macrolide antibiotic produced by streptomyces tsukubaensis. Mechanism of action: Inhibits T cel activation by inhibiting calcineurin which is required for its activation. Tacrolimus binds immunopbillin Fk binding protein (FKBP) ~» complex inhibits calcineurin + inhibits activation of T cell | Metabolism: Like cyclosporine, tacrolimus is metabolized primarily by p-450 enzymes in liver [Use: + Organ transplantation (used for same indications as cyclosporine) + Tacrolimus has been proved tobe effective therapy for preventing rejection in solid organ transplant patient even aftr failure of standard rejection therapy including anti T cell antibodies. + Topical applications is used for dermatologic diseases such as atopic dermatitis and psoriasis Toxic effect: * Neplarotoxity ‘* Hyperglycemia «* Hyperkalemia + _Neurotoxity ‘Hypertension + Gastrointestinal complaints Answer is C (Cadmium): KDT 7"/906 Bal is contraindicated in cadmium and iron poisoning because the dimercaprol — iron and dimercaprol Cd complex is it self oxic — KT. Use of different chelating agents Dimercaprol (BAL) ‘Calcium disodium edetate (Ca Na; EDTA) ~ Poisoning by As, Hg, Au, Bi, Ni, Sb ~ Asan adjuvant to EDTA in lead poisoning ~ Asan adjuvant to penicillamine in Cu = Contraindicated in iron and cadmium poisoning. = Lead poisoning + Also used in Zn, Cd, Mn, Cu, Fe poisoning + Not used in mercury poisoning Desferrioxamine (obtained from an actinomycete) ~ Acute iron poisoning Penicillamine ~ Wilson's disease (Hepatotenticular degeneration) ~ Transfusion siderosis ~ Copper/ mercury poisoning = Available only in injectable form = Chronic lead poisoning Deferiprone + Cystinuria and eystine stones = Acute iron poisoning + Scleroderma = Iron load in liver cirrhosis = Both oral and injectable preparation available. Answer is C (Anthropometry) : Parikh 5" / 52 Biological sample with jail authorities or from family members/relatives is an essential prerequisite without which, none of the three tests namely blood grouping, DNA profile on HLA typing cannot be carried out. Thus the only viable option for establishing the identity of the convict is Anthropometry. Anthropometry is system based on the fact that after the age of 21, the dimensions of the skeleton remains unchanged and also that the ratio in size of different parts to one another varies considerably among the individuals. This system includes 1. Descriptive Data: Colour of hair, eyes, complexion, shape of nose, ears, chin ete. 2. Body marks: Moles, Scars, Tattoo marks 3, Body measurements: Height, AP diameter of Head and Trunk, Span of Outstretched arms, the length of left middle finger, left little finger, left forearm, left foot, length and breadth of Right Ear and Colour of Left Iris. 4. Photographs ofa front view of the head and a profile view of the right side of the head are also taken.860 * AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 105. Answer is A (70 - 75) : Parikh 6" /226- 7; Reddy 22/48 The skull of Aryans (Indians), Aborigines and Negroes is dolicocephalic with cephalic index between 70-75. + Cephalic index or index of breadth is measured as; Max breadth of skull Max length of skull Cephalic index ; 75-799 wi Triangular (Narrow and elongated. Triangular 106. Answer is A (Grevious) : Parikh 6 /4-59 Grevious injury : ‘Grevious injury is defined as one : 1. Which i extensive or serious 2. Which docs not heal rapidly 3. Which causes a permanent deformity or disfiguraton. Scotor 320 IPC defines following injuries as grievous: Permanent privation ofthe sight of either eye Permanent privation ofthe hearing of either eye Privation of any member (pat, organ, limb) oF joint Destructive ‘or’ permanent impairing of powers of any memiber or joint Fracture or dislocation ofa bone o a toth Permanent disfiguration of head or face Any hurt which endangers life, which causes the sufferer tobe, during the space of twenty days: in severe body pain, or ‘unable to follow his daily routine. The injury 10 the patient in question should be termed grievous because : ‘The patient developed comeal opacity following injury in the only working eye. = This comeal opacity is unlikely to heal (criterion in the definition) and also = this man would have not been able to perform his daily routine because he was blinded by the injury and = loss would have been permanent if the disease is allowed to follow the natural course of events. (No surgical intervention is done). + Simple injury is one which is neither extensive nor serious and which would heal rapidly without leaving ‘any permanent deformity or disfiguration + A dangerous injury is a variety of grievous injury. It is an injury, which poses an immediate danger to life. It is either extensive or serious in relation to the organ or part wounded. The injury is fatal in the absence of surgical aid. Common examples of such injuries are gunshot wounds, compound fractures of the skull, transection ofa large or important blood vessel, and rupture of some internal organ, e.g, Spleen. 107. Answer is C (Aniline dyes) : Apurva Nandy 2" /155 Postmortem lividity (postmortem staining, hypostasis) means discolouration of skin and organs after death due to accumulation of fluid blood in the toneless capillaries and small veins of the dependent parts of the Fe me ao oe body. Colour of postmortem lividity / hypostas i. Norma ‘Biuish pink followed by Blush purple 2. Cyanide? Pink 3. Carbon monoxide® Bright cherry red (death due to burning and exposure to cold also show bright cherry red colour) 4. Chlorates Chocolate brown 5. Phosphorus Dark brown 6. Nitrtes Red brown 7. HS? Bluish green 8. Opiates? Black 9._ Aniline? Deep blue (Apurva Nandy)108, 109. 110. mi. Answer is D (Septicemia): Parikh Sih/141; Parikh 61h/3.9 There are certain conditions in which heat may be retained or even be increased in the first two hours after death. This phenomenon is known as postmortem caloricity. (After the initial rise of temperature the body begins to cool as usual). ‘onditions showit includ ‘© Sunstroke and pontine haemorrhage - the heat regulation is disturbed ‘Tetanus and strychnine poisoning - heat production is increased in muscles ‘© Acute bacterial or viral infections (septicemia) - duc to excessive bacterial or viral activity ‘Intense asphyxial conditions Answer is A (Brain) : Parikh / 346, 6t%/4,98 Contercoup or Coup injury is generally seen in the region of the head (brain). + A coup injury is one which occurs immediately subjacent to the area of impact. + A contercoup injury is one which is situated on the contralateral side of the area of impact. Itis due to sudden stoppage of the movement of the head by coming in contact with a hard substance on the opposite side causing linear and more harmful rotational strains. Answer is C (Precipitin test) : Parikh 6” / 7-21 Precipitin testis an immunological test used to determine if blood under examination is that of human being or is derived from any other source (animals) The utility is not confined to determination of species origin of blood alone, but is useful for any albuminous (protein) substance of the species in question. This testis thus useful to determine species specificity and not for settling disputed maternity. The question of disputed mat / paternity issues can sorted by 1) Blood grouping: © ABO grouping excludes paternity in 1/6" cases ‘* Addition of MN system excludes paternity in 1/3" cases ‘+ Addition of RH subgroup excludes paternity in 1/2 cases 2) HLASystem ; Can demonstrate non paternity in 90% cases 3) DNAprofiling ; _ It provides absolute certainty rather than a probable exclusion (Ginger printing) Answer is D (Teichman’s test) : Read text below + Benzedine, Phenopthaline, Orthotoludine, Leucomalachite green tests are employed as ‘screening’ tests of blood All these tests work on similar principles and detect presence of haemoglobin, a peroxidase, which in the presence of H;0; oxidises colourless bases into coloured salts. This colour change indicates the presence of blood ‘+ The principle in teichman test (haemin crystal test) is to convert haemoglobin of the stain into haemin which in presence of halogen is converted into salt and form rhombic crystals. ‘Tests in questions : ‘A. Benzedine test: Extract in Test Tube + few drops of 10% Benzidine in Glacial Acetic Acid + few drops of HO: —+ Blue Colour indicates Positive tet (similarly the stain is moistened with Normal Saline and a blotter is pressed over the area + Benzidine Solution + HO; —+ Blue Colour indicates Positive test. B. Phenophthalein test (Kastle-Meyer test: Diluted Stain Extract * Reduces Alkaline Phenophtaline + few drops of H,0; ~ Pink Colour indicates Positive tes. C. Orthototuidine test (Kohn and O'Kelly tes): Equal volume of Working Solution (4% Orthotoludine in Ethyl Aleohol + Glacial Acctic Acid + Distilled Water in equal amounts) and HO, ae mixed. Then a few drops of this mixture i added tothe stain extract in a test tube —+ Blue or Green Colour indicates Positive tes. D. Teichhmann’s test (Haemin crystal test): NaCI 2 to 3 drops of Galcial Acetic Acid is placed on the stain on a las slide. Cover slip applied + Evaporated by heating over small flame. Examined under Microscope. Faint yellowish-red to brownish black rhombic erystals of heamin or haematic chloride aranged single or in clusters are seen if blood is present.Answer is D (Dhatura poisoning) : Repeat Dhatura poisoning manifc 1. Dilatation of pupils, which are unresponsive to light 2. Dryness of mouth 3. Difficulty in taking 4. Dysphagia a 5. Dilatation of cutaneous blood vessels 6. Dry, hot skin im 7. Drunken gait 2 6 2 ie ._ Drowsiness & 2 ribed as : 3 "Dry as a bone, Red as a beat, Blind as a bat, Hot as a hare, and Mad as a wet hen". 4113. Answer is B (Arsenic) : Harrisons 15"/2594 : Parikh 6" /9.8 t0 9.12 Rain drop pigmentation of skin and Mees lines on nails are both characteristic feature of Arsenic intoxication. ‘The questions asked in Arsenic poisoning are - '* Tasteless; odourless; mixed with food, drink & paan © Associated with spasms ® Fatal dose = 120-200 mg ® Confused with cholera - Parikh Sth / 746 ‘Treatment is by freshly precipitated hydrated Ferric Chloride, © '* Manifestation of Chronic Poisoning Clinical Features = Nausea, vomiting 2 = Skin rashes, hyperkeratosis ® ~ _ Rain-drop type pigmentation ® = Mees lines (5 wks after exposure) - Tingling & Numbness 2 = Arsenic neuritis (resembles that of chronic alcoholism) ® Post-Mortem = Retardation of decomposition © - Arsenic deposited in Hair, Nails, Ends of long bones & can be detected even in charred remains} after the body is burnt. & = Marsh's test > o detect the presence of Arsenic even upto 1/1000 of a mg. - Rigor mortis lasts longer = Stomach mucosa is red velvet. 14. Answer is C (gas-liquid chromatography) : Parikh 6"/10.23, 10.27 Reddy 22/478 ‘Gas-chromatography is the most desirable method of alcohol estimation for medico legal purpose’ ~ Reddy Be chromatography ‘Alcohol dehydrogenase method (ADH method) Breath analysi a ‘© Gas chromatography: Provides both qualitative and quantitative analysis Advantages are 1, Procedure is simple, sensitive and reasonably specific 2. Only small quantity of sample is required. 3. Analysis is not interfered with by other ingredients e.g, alcohol, aldehydes, ketones ete,115, 116. Chromatogram records separate peaks for these substances. This technique is very useful when deaths are ‘due to consumption of poisonous liquor. Alcohol and other denaturants especially methyl alcohol are simultaneously recorded identified and quantified, Cavett method: Also shares advantages with gas chromatography but is less specific than chromatography and hence is not the most reliable method for estimating alcohol. Breath analysers : = These are used as preliminary screening tests = Uncertainty arises out of the important assumption that the absorption was already complete in the given case; if not over estimates of blood alcohol concentration are likely = These methods are thus certainly not the most reliable methods. Answer is D (Ethyl alcohol): Harrison 15"/2611; KDT 5! / 354 ‘The patient in question presents with features of methanol or methyl alcohol poisoning. Toxic effects of methanol are largely due to formic acid. Methanol is metabolized to formaldehyde and formic acid by alcohol and aldehyde dehydrogenases respectively. Ethanol is used in treatment of methanol poisoning as it saturates alcohol dehydrogenase and retards ‘methanol metabolism; thereby helping in reducing the rate of generation of toxic metabolites. Methyl Alcohol poisoning ‘© Methanol is used to denature ethanol, can be present in contaminated country liquor. ‘© Methanol is a CNS depressant and toxicity is caused by Formaldehyde & Formic acid Clinical Picture = Affects begin within hour of ingestion = Nausea, vomiting, abdominal pain, Headache, vertigo, = Pancreatitis = Coma, Seizures, Metabolic Acidosis & Retinal injury -»Due to formic acid © Diagnosis = By history of alcohol consumption = By measurement of serum methanol & formate levels. + Treatment ~ Gastric Aspiration is Treatment of choice - Volume Replacement, Respiratory care & TT of seizures ~ Sodium bicarbonate to correct acidosis = Ethanol or Fomepizole therapy when ~ Methanol cone > 6 mmol/L = Increased Anion Gap Acidosis = Visual symptoms Answer is D (Naloxone) : KDT 5" / 422; Harrison 15" / The patient is presenting with features suggestive of acute opioid (heroin) intoxication. "The specific antidote for such acute intoxication is Naloxone! ~ KDT The opioid antagonist eg naloxone competes with heroin and other op effects of opioid agonist. Note: + Methadone is used in rehabilitation of opioid dependent persons as maintenance therapy. It is not aimed at curing opioid dependence rather it provides a substitute drug that is legally accessible, safer and can be taken orally. The goal isto help person who have repeatedly failed in drug free programmes. It has no role in Acute opioid intoxication. + Buprenorphine is a partial opioid agonist at y receptors. It has no role in acute opioid intoxication + Flumazenil is a benzodiazepine antagonist. for receptors, and reduces the47. 119. Answer is (C) (concurrent cohort study): Park 23/76 Concurrent cohort study or prospective cohort study is one in which the outcome (disease) has not occurred at the time the investigation begins. In the given question the study began in 1970 and the outcome (occurrence of cancer) was studies between 1990 and 1995. Thus the outcome had not occurred at the time the investigation begins. The study progressed from "cause to effect” is from ‘alcohol consumption’ to the occurrence of ‘cancer' and hence is a classical prospective cohort study. Cohort studies —— ee t Prospective cohort Retrospective cohort ‘Current cohort’ ‘Historical cohort’ + Outcome has not yet occurred at the time — « — Outcome have all occurred before the start of the investigation begins investigation + Begins in present and continues into + Investigator goes back in time to select his study groups future prospectively from ‘cause to effect’ (‘cause’) but again traces them ‘forward’ through time up to the effect’ It is thus not actually a retrospective study but infact a ‘prospective study in retrospect’ ‘non concurrent prospective study. + Follow up is an integral part of study + No follow up + Attrition is a major problem + Noattrtion + Study takes a long time + Study takes short time + Study is more cost! + Study is cheaper Answer is B (Period of communicability): Park 23/100 ‘The communicable period is defined as ‘the time during which an infectious agent may be transferred directly ‘or indirectly from an infected person to another person.’ The period of communicability bears absolutely no relation to the incubation period, and merely reflects the duration when the infectious agent may be transferred. This may occur during the incubation period or during the actual illness. ‘The incubation period is defined as ‘the time interval between invasion by an infectious agent and appearance of frst signs and symptoms of disease in question’ The other three options viz ‘Latent period’, ‘serial interval’ and ‘generation time' all are approximate ‘measures of incubation period. ‘The period from ‘disease initiation "The gap in time between ‘Interval between receipt of infection bya to disease detection’? ‘onset of primary case and host and maximal infectivity’ ° 7 secondary case! 3 ‘By collecting information we In general, generation time 15 ccan guess the incubation equal to the incubation period’ jin period of disease’ ~ Park Answer is C (BCG, DPT, OPV, measles, Vitamin A): Ghai 5°/167 ‘+ While deciding the most appropriate immunization recommendation for a child who has not received any primary immunization the following guidelines need to be observed + Two live vaccines may be administered simultaneously but must be given at different sites ~ Park ‘A minimum interval of four weeks should be allowed between administration of two live vaccines.120. + Regarding pertusis component (Option between DPT and DT) /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004» 865 + Regarding Measles va = The WHO expanded programme of immunization recommends immunization at 9 months of age®. = This age can be lowered down to 6 months if there is a measles outbreak in the community. [immunization before the age of 9 months carries the risk of vaccine being rendered ineffective by the natural antibodies acquired through the mother} = Children who have not been immunized during infancy may be immunized at any age. Even adults who have not had natural measles should be immunized. = Since the child has not been immunized against measles and simultaneous administration of two or more live vaccines is not contraindicated, immunization against measles is recommended. ‘Since the severity of pertusis infection decreases with age the pertusis component in DPT vaccine is not recommended after age of 6 years. Therefore children over the age of five years who have not received DPT need, only DT’ Park. Since the child in question is 3% years of age DPT is recommended & not DT. ‘+ Regarding vitamin — A: National programme for control of blindness recommends administration of vitamin A to all preschool children in the community every 6 months. ‘The child in question is a pre school child and hence Vitamin A prophylaxis is recommended. Thus theoretically as well as logically both measles and vitamin A prophylaxis along with BCG, DPT & OPV may be given in a child coming unimmunized at 31 years of age However, what confuses the issue is the vaccination schedule for children not immunized in time as stated in a handbook on immunization.~ Immunization Digest by Parthasarthy, Lokeshwar, Shah Vaccinat An Unimmunized Child 1 visit BCG OPV.DPT.NB TrTaHB — orv pr HB rrreHe estan 4a 3 visit OPV.DPT. MMR/Measles, MMR. Typhoid (month ite) Typhoid (by ater oP per HB a Bey3 ye0r Typhoid boner ‘Typhoid booster If we were to follow these guidelines option A in the question may seem correct as well. However, considering the Indian setup where a patient coming for his first visit at 314 years might not return for a second visit at all. Option C is the answer of choice in my view. Answer is D (Presence of H. Influenza type B component increases its immunogenecity) Park 23"/162; CMDT 2003/1262; Nelson 16"/820, 837: Harrison 15/781 © ‘Adjuvants' are substances that are added to vaccines with the intent of potentiating the immune response, 50 that greater amount of antibody is produced, a lesser quantity of antigen is required and fewer does are required. ‘Aluminium phosphate is used as an adjuvant in DPT vaccine ~N1SI/781 ‘+ Pertusis component in DPT (whole killed bacteria of bordtella pertusis = 20,000/.5 ml) enhances the potency of diphtheria toxoid ~ Park (ie. it has an adjuvant effect) ‘The most serious complications following DPT immunization are neurological” and are primarily due to pertusis component of vaccine® ~ Park ‘To reduce the complications associated with whole killed bacteria of bordtella pertusis in DPT an acellular vaccine was developed. “Acellular pertusis vaccine is highly immunogenic but associated with far fewer adverse effects than the whole cell vaccine. Itis now given as DTaP’ CMDT ‘¢ H. influenza conjugate vaccine (PRD-D) is not a substitute for immunization against diphtheria. It does not increase the immunogenecity of diphtheria toxoid. ‘Diphtheria toxoid increases the immunogencity of Hib vaccine’ ~ H 15%/781 "Attempts to combine acellular pertusis vaccine with Hib conjugate vaccine have resulted in decreased PRP antibody’ — Nelson 16/8357121. 122. 123. 124, ‘> AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 Answer is A (Give a dose of Tetanus Toxoid (TT) and explain to her that it will not protect the new born and she should take the second dose after four weeks even if she delivers in the meantime): Park 23/313 ‘* Indeveloping countries the majority of pregnant women are not seen antenatally. Since pregnant women coming for an antenatal visit may infact never return again, the following guidelines are observed. = ‘Immunization should be given regardless of the month of pregnancy’ as there is no evidence to suggest that tetanus toxoid are dangerous or harmful to the foetus ~ Park = The golden rule is that no pregnant women should be denied even one dose of tetanus toxoid if she is ‘seen late in pregnancy ~ Park ‘© ‘The infants born to mothers who have not previously received 2- doses of tetanus toxoid are exposed to risk of neonatal tetanus’ ~ Park 17/241 Thus it should be explained to mother that this single dose may not protect the new born if she delivers in the ‘meantime. The second dose is recommended at least one month later (after 4 weeks) Infants born to unimmunized mother can be protected by injection of antitoxin (750 IU) if itis administered within 6 hours of birth ® Answer is D (Refer urgently to hospital after giving first dose of an antibiotic): Ghai 5/350, Park 23/170 The child in question has ‘chest indrawing’ along with fast breathing and is hence classified as suffering from ‘severe pneumonia’ (not pneumonia’) A child suffering from ‘severe pneumonia’ should be urgently referred to hospital after giving first dose of antibiotic, jon And Management Of Pneumonia In A Child Aged 2 Months To Fiv yf Protocol For: Age Criteria For Fast Breathing > 60min in child less than 2 months > 50min in child 2 months to 12 months > 40/min in child 12 months to 5 years jehest No chest (Chest Indrawing ing Indrawing Present nasal flaring Fast grunting Breathing ~eyanosis wake Present = wheezing = Stridor in calm child = Severe malnutrition 4 4 4 4 [DE No Pneumonia Pneumonia Severe Pneumonia ‘Very Severe Pneumonia il 4 J 4 care + Home care + Give first dose of + Give first dose of + Antibiotics antibiotics antibiotic + Treat fever «+ Refer urgently to + Refer urgently to + Treat wheezing hospital hospital The patient in question _~is 4 months of age ~has chest indrawing = does not have fast breathing (> SO/min, here 45/min) This would hence be classified as severe pnewmonia, and hence should be managed by first dose of antibiotic and referred to hospital. Answer is C (Tuberculin test): Repeat: Park 23/185 'The ‘tuberculin test’ is the only means of estimating the prevalence of infection in a population'-Park 17*/140 Answer is A (11%): Park 23/283 The aedes aepyptit index is kept below I percent ~ Park For surveillance of Aedes mosquitoes, the WHO uses an index known as Aedes Aegypti index. This is a ‘house index" and is defined as "the number of houses and their premises in a limited well defined area showing actual breeding of Adedes aegyptii larvae" ‘© The index should not be more than 1% in towns and seaports in endemic areas to ensure freedom from yellow fever ~ Park ‘* Airports and seaports are kept free from the breeding of insect vectors over on area extending at least ‘400m? around their perimeters.125, 126. Yellow fever: Quick review 1. Agent: ~ Group B Arbovirus® (Flavivirus®) 2. Reservior: - Monkeys in forest area? ~ Man in urban areas® 3. Insect vector: - Aedes Aegyptii ° = Person to person transmission in urban cycle 2 - Virus multiplies inside insect vector: propagative cycle? 4. Incubation: - Intrinsic 3 - 6 day (6 days recognized under international health regulations?) riod - Extrinsi: 8~12 days (mosquito becomes infective after 8 ~ 12 days) 5. Hmmunity : = One attack of yellow fever gives life long immunity? 6. Clinical : ~- Haemorthagic® fever? (black vornit®, epistaxis®, malena®) with features sever hepatic® and renal? manifestations. 7. Vaccination: —- 17 D vaccine? : Live attenuated? freeze dried? vaccine [Immunity appears on 7" day and lasts for 35 years or more] International certificate of vaccination: Validity of certificate begins 10 days after date of vaccination and extends upto 10 years? Answer is C (Transplacental spread): Park 23/318 "Transplacental spread has not been mentioned as a mode of transmission of leprosy" ‘The modes of transmission of leprosy have not been established with certainty The following theories are frequently debated : Droplet infection Contact transmission Other routes Transmission via aerosols ‘Transmission via close contact Transmission via containing M. leprae (droplet between an infectious agent Breast milk from infection) and a healthy but susceptible Jepromatous mothers host = Insect vectors = tattooing needles Direct contact? _ Indirect contact® Skin to skin Contact with soil and forites such as, ‘contaminated clothes and linen Answer is C (Defaulter is defined as a patient who has not taken treatment for six months or more): = Park 23/324 Term ‘defaulter' has primarily not been defined for Lerposy patients It is used for tubercular patients and refers 10 ‘a patient who returns sputum smear positive for tubercular bacilli after having left treatment for at least 2 ‘months." Defaulters may be seen in leprosy as well, however the period of leaving treatment of leprosy to be classifed as defaulter has not been mentioned in any of the available texts. Also as all other options are true, this is the answer of exculsion. + WHO study group on chemotherapy of leprosy for contral progromme, has recommended use of clinical ‘grounds in the form of skin lesions’ to classify leprosy. ‘The assumption is that protective immunity is inversely correlated with the number of skin lesions. Taking, into account that single lesion leprosy may be cured by regimen other than the standard MDT, the following classificaton has been proposed. Pauci ee 2-5 skin lesions Baa “> Sskin lesions + Elimination target for Leprosy is less than 1 case per 10,000127. 128. 129, 130. | ANSWERS AND EXPLANATIONS - 2004 Statistics on Leprosy that have been asked: Prevalence of leprosy in India is about: 6.77 10,000 population @ Percentage of cases in children below age of 1S years: 15% @ Percentage of infectious cases: 15% ° Percentage of cases suffering from deformities: 15% @ = India accounts for 13 of leprosy cases inthe world and has by forthe greatest number of registered cases among individual countries. © + Incidence is defined as the number of new cases occurring ina defined population during a specified period of time. New case detection rate is thus an indicator for incidence of Leprosy Answer is D (1 lakh units im, on day 0, 1, 14): Ghai 5/59; Khurana 3/416 ‘* Treatment schedule as recommended by WHO > year (except women of 2 lakh TU orally 2ilakh IU orally 2 lakh IU orally reproductive age) or or or 1 Takh TU imi 1 lakh TU inn | lakh TU imi ‘|S Lyear or any age but <8 kg 50,000 TU orally $0,000 TU orally ! © Treatment schedule applies to all stages of active xeropthalmia © Oral administration is the recommended method of treatment * Intramuscular injection should be preferred in presence of repeated vomiting and severe diarrhea The patient in question weighs 8 kg and thus can be given full adult doses, preferably 2 lakh units orally on day 0, 1 & 14, But may also be given I lakh units imi on day 0, 1 & 14. Answer is B (Exclusive breast feeding should be continued till 4 months of age followed by Supplementation with additional foods) : Ghai 5%/43 ‘Exclusive breast feeding means that except for breast milk, no other food or fluid including prelacteal feeds ‘and water should be given to a child from birth to 4 months of age" - Ghai ‘The human milk in reasonable quantities alone cannot provide all the energy and protein required for ‘maintaining an adequate velocity of growth for the infant after the age of 4 months. It is therefore necessary t0 introduce more concentrated energy dense supplements by this age’ - Ghai Answer is A (The maximum milk output is seen at 12 months) Park 23/531 ‘* Under normal conditions, Indian mothers secrete 450 ~ 600 ml of milk daily Maximum output of milk is at 5-6 months (730 ml/day) after which the output constantly declines. ‘At 12 months the output is 525 ml/day — Park/366 ‘+ Iron was supposed to be deficient in breast milk, bat recent work has shown that iron contained in human milk has a high level of bioavailiability thanks to complex phenomena, The coefficient of uptake of iron in breast milk may be as high as 70% where as itis only 30 percent for cow's milk and infant formulas’ ~ Park/374 is high so that uptake of calcium is better than cow's milk’ ~ Park/374 is 64 to 72 kcal per 100 ml — Park 372, 373, Energy value of human mil Answer is A (Multiload Cu ~375 is a third generation IUD): Park 23°/496 Multiload Cu 375 is a second generation IUD and not a third generation IUD. cea teen Lippes loop Barly devices: = Copper T © Copper 7-200 Never devices: # Variants of Copper T devices = Teu220e = Teu-380A © Nova-T © Multiload devices = ML-Cu~250 = MI-Cu~375131. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004» 869) + The pregnancy rates of both Lippes loop and CuT 200 per 100 women after 12 months of use are same - 3/100 women. “Device = recy Fae’ palit eae Firs generation ‘© Lippes loop (C) 3.0 191 [Second generation ; ‘CuT - 200 3.0 78 NovaT 07 58 * — Multiload 250 05 22 Multilaod OL 2 Third generation # Progestaset 18 31 ‘© Post coital contraception: "Introduction of UCD within a maximum of 5 days can prevent conception following an accidental unprotected exposure. This is probably through increased tubal peristalsis’ ~ Dutta ‘Effective as post coital contraceptive if inserted within 3-5 days of unprotected sex’ - Park © Effective life of Third generation 1UD ‘© Progestosert ‘© Releases 65 jg of hormone daily «Effective life of year © Levonova ‘Releases 20 pg of levonorgesterol daily Effective life of 5 years ~ Park Answer is A (To bring down Total fertility rate to replacement level by 2015) Park 23/493, ‘Objective of National Population Policy is to bring the TFR to replacement levels by 2010" — Park The objective of NPP 2000 is to bring the TFR to replacement levels by 2010. The long term objective is 10 achieve requirements of suitable economic growth, social development and environment protection. he National Socio ~ Demo; ‘Address the unmet needs for basic reproductive and child health services, supplies and infrastructure. ‘Make schoo! education up to age 14 free and compulsory, and reduce drop-outs at primary and secondary school level to below 20 percent for both boys and girls Reduce infant mortality rate to below 30 per 1000 live bith. Reduce matemal morality ratio to below 100 per 100,000 live births. Achieve universal immunization of children against all vaccine preventable diseases. Promote delayed marriage for girls, not earlier than age 18 and preferably after 20 years of age. ‘Achieve 80 percent institutional deliveries and 100 percent deliveries by trained persons. Achieve universal access to information/counseling, and services for fertility regulation and contraception with a wide basket of choices Achieve 100 per cent registration of birth, deaths, marriage and pregnancy. ‘Contain the spread of Acquired Immunodeficiency Syndrome (AIDS), and promote greater integration between the ‘management of reproductive tract infections (RTI) and sexually transmitted infections (STI) and the National AIDS ‘Control Organisation. Prevent and control communicable diseases. Integrate Indian Systems of Medicine (ISM) inthe provision of reproductive and child health services, and in reaching. ‘out to household Promote vigorously the small family norm to achieve replacement levels of TER. Bring about convergence in implementation of related social sector programmes so that family welfare becomes a people cenred programme.870 132. 133. 134, ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 Answer is C (Reduce under 5 mortality to half): Park 23/454 Essential Component of RCH programme includes : Essential Obstetric Care (antenatal, natal and postnatal care) + Early registration of pregnancy (2-16 weeks) = Minimum 3 Antenatal checkups += Provision of safe delivery at Home/Institution = Minimum 3 post natal checkups + Emergent Obstetric Care: to deal with complications associated with pregnancy #24 Hour Delivery Services at PHCs/CHCs + Service of MTP at PHCs for terminating unwanted pregnancies * Control of RTIsSTD. + Immunization against 6 vaccine preventable Disease at PHCs © Drugs & equipment Kits * Essential newbom care: = Operationalzation of distinct newborn care. = Home based neonatal care * Control of Diarhea in children through oral rehydration therapy * Control of acute respiratory disease + Control & prevention of Nutritional Blindness through vitamin A ‘+ RCH camps —to provide specialist services of Gynae obs and pediatrics in Remote Areas ‘RCH out reach scheme’ based Neonatal care through Gadchiroli model approach started in 2002 in Bihar, U.P. Orissa, Maharashtra, Rajasthan to provide neonatal care at grass root level ‘+ Hep B vaccine introduction in selective District. ‘+ Border district cluster strategy ~to provide intervention for reducing IMR and MMR Integrated management of childhood illness (MCI) Answer is D (Clean airway) : Park 23/311 Neonatal tetanus may be controlled to a large extent through clean delivery practices alone. Most programmes in developing countries are concentrating on educating traditional birth attendants and pregnant women about the ‘three cleans’ (Clean hands |Ctean delivery surface Clean cord care |Clean blade for cutting the cord (Clean tie forthe cord and no application on the cord stump Answer is (A) (A decrease in complex carbohydrate consumption): Park 23°/370 "The WHO expert committee has recommended an increase in complex carbohydrate consumption and not a decrease" -Park /276 Dietary goals prescribed by WHO expert committee prudent diet Dietary modification is the principal preventive strategy in the prevention of CHD. The WHO Expert Committee (1) considered the following dietary changes to be appropriate for high incidence populations. = reduction of fat intake to 20-30 per cent of total energy intake = consumption of saturated fats must be limited to less than 10 per cent of total energy intake; some of the reduction in saturated fat may be made up by mono and poly-unsaturated fats. - areduction of dietary cholesterol to below 100 mg per 1000 kcal per day. = an increase in complex carbohydrate consumption (ie. vegetables, fruits, whole grains and legumes) = avoidance of alcohol consumption; reduction of salt intake to 5 g daily or less. Other specific interventions as part of primordial prevention of coronary heart disease = Toachieve a smoke free society = Reduction of Blood pressure through prudent diet, Regular exercise, weight control & cessation of smoking. = Regular Physical Activity135. 136. ‘AIPGME EXAMINATION ANSWERS an Answer is C (Kartar Singh committee): Park 23°/874 Ee Srivastava committee (1975) Group on medical education and support manpower Recommended rural heath scheme Concept of village health guides was given® Recommended creation of bands of professional and semiprofessional health workers, ‘rom within the community including village health guides, multipurpose health worker and heath assistant. Iwalla ‘Covtimitee on integration of healt services” en ‘Recommended elimination of private practice by government doctors? Mukherjee committee Committee on basic health services? 1968, 66) idaliar committee ‘Health survey and planning commits’ © :) Main recommendations ‘© PHC not to serve > 40,000 ‘© Improve quality of health care by PHC e ‘© Integration of medical and health service ; Create an All India Health Service like ‘All India Administration Service.’ Bhore committee Recommendations: ¢1946) 1. Integration of preventive and curative services at all administration levels 2. Short term and long term recommendations ‘© Short term: PHC to cater to a population of 40,0002 ‘© Long term: PHC units to be set up with 75 bedded hospital for each 10,000 to 20,000 population? 3. Major charger in medical education including 3 months training in social and preventive ‘medicine to prepare ‘social physicians’ ® Answer is A (Original standard deviation x 10) Read text below ‘Standard donation’s is given by the formula Tfeach value of a given group of observations is multiplied by 10 © New individual values will be = 10%, 10 25 .-.o0 10% 10x, +10x) +10x, ‘© New, mean of values )&( will be = z The new SD= Y= ‘New S.D.=10x.S.D. 00 Let us consider the same with another example: Example "New values if each is multiplied by 10 80 9 100 110 120 ‘800 900 1000 1100 1200 S.D.inabove example = 10 New$.D.= 100 {Thus new SD = 10x SD)138. 139, Micke Mie Mien Men Men > > - Notail = Tail is towards right side - Tail is towards left side = Bell shaped curve: Gaussian - Large no of low scores, small no. - Large no of high scores, distribution of high scores small no of low scores - Mean=Median=Mode - Mean > median > mode - Mode > median > mean ‘© In this case median (140 mm Hg) is greater than the mean (130 mm of Hg) so the distribution is said to be negatively screwed (left. Handed) deviation, Answer is B (75%) : Park 23°%/139 Positive True Positive False Positive 600 400 200 a Negative False Negative ‘True Negative 700 i 100 600 ; 500 800 1300 i (A) Seasiviye ‘TRUEPOSITIVE. 400, 100-2 190-806 ‘TRUE POSITIVE +FALSENEGATIVE 500 (B) Spevifciy = RUENEGATIVE = 50° -100=79% ‘TRUENEGATIVEFALSEPOSITIVE — 800 (©) Predictive value of Positive test ‘TRUE POSITIVE 400 “TRUE POSITIVE+ FALSEPOSITIVE 800 (D) Predictive value of Negative test = TRUE NEGATIVE. = 00x 100=85.7% TRUE NEGATIVE + FALSE NEGATIVE 700 Answer is C (,001) : Mahajan 6/107 Probability can be calculated in accordance with ‘Addition law of probability’ or ‘Multiplication law of probability’ depending on whether the event/data is mutually exclusive or not, Addition law of probability Multiplication low of probability” "NI ‘© Applied when data are mutually exclusive ie. © Applied when data are not mutually ‘one event excludes the probability of exclusive occurrence of the other e.g. = On tossing a coin, getting heads excludes the ut of 100 smokers, development of possibility of getting tails cancer in one does not exclude the = Onone throw of a dice, getting 2, excludes possibility of having cancer in the other other events ie. getting, 1,3,4,5 or 6 Ifthe number of mutually exclusive events is ¢ If the number of events is n, and p, is the) ‘n' and p, isthe individual probability, then individual probably, then total probabil Jp ‘sl probability iis is Pris P= pit Paton De P=p)X 2 Xone Pe140. 141. In the given question the prevalence of diabetes is 10% is the individual probably of a person to have diabetes is 10 100 ‘Also, occurrence of diabetes in one individual does not exclude (or reduce) the (possibility) probability of developing diabetes in another i.e. events are not mutually exclusive and hence ‘multiplication law’ will give total probability © The total probability of three persons haring diabetes 'P' will be P = 0.1 x 0.1 x 0.1 = .0001 Answer is C (ordinal) High yield Biostatistics/4 ‘© There are four scales of measurement (@) Nominal (b) Ordinal (©) Interval (a) Ratio © Nomi = Nominal scale is used where data is divided into qualitative categories or group such as malerfemale black/white, urban/suburba/rural. = There is no implication of order or ratio + Ordinal = Ordinal scale is used where the data can be placed in a meaningful order (eg. Students may be ranked 1. 2" & 3" in their class) + However there is no information a bout the size of the interval no conclusion can be drawn about whether the difference between the frst and second student is same as the difference between the second and third. ~ Variables in the form of mild, moderate and severe are analyzed by ordinal scale as they can be arranged in a meaningful order but thee is no information about the size of the interval between them. © Interval + Interval scale data have the same quality as do ordinal data, in that they can be placed in a meaningful coder, in addition these data have meaningful intervals between items., which are usually measured quantities - foreg. on the celsius scale the difference between 100 degree and 90 degree is the same as the difference between 50 degree and 40 degree. However because interval scales do not have absolute O. ratios of scores ‘are not meaningful ie. 100 degree C is not twice as hot as 50 degree C because 0 degree C dos not indicate the complete absence of heat. © Rati = A ratio scale has the same properties as an interval scale: but because it has an absolute zero. meaningful ratios do exist. = eg. Weight in grams of pounds, time in seconds or days, B.P. in millimeters of mercury and pules rate are all ratio scale data The only ratio scale of temperature is the Kelvin scale in which zero degrees indicate on absolute absence of heat. Just as zero pulse rate indicate an absolute lack of pulse. Therefore, it is correct to say that a pulse rate of 120 is twice as fast as pulse rate of 60 Answer is A (Sensitivity) Park 23°/139 Screening refer to the search for unrecognized disease or defect by means of rapidly applied tests, examinations or other procedures in apparently healthy individuals’ + Forscreening a disease the most important parameter is sensitivity = Sensitivity is defined as the ability ofa test to identify correcty all those who have the disease ie. true positives as it reflects the ability to detect those who have the disease amongst the hidden cases in the community ‘© For confirming a patient as diseased most important parameter is specificity Specificity is defined as the ability of atest to identify correctly all these who do not have the disease. ie. true negatives, ‘Memo: ELISA is a sensitive test for HIV : Elisa is used as screening test for HIV Western blot is a specific test for HIV: Western blot is used as a confirmatory test for HIV.Answer is C (Decreased coagulation factor levels) : Harrison 19/737 While coagulation factors are characteristically consumed (decreased) in case of DIC their levels are essentially normal in case of TTP. DIC: Disseminated intravascular coagulation (Review) [DIC or Disseminated Intravascular Coagulation, is @ complex thrombohaemorshagie disorder characterized by follow nce of ev 1. Intravascular activation of coagulation by both intrinsic and extrinsic mechanism.& 2. A thrombotic phase 3. Consumption phase, wherin most coagulation factors and platelets are consumed. 4. Stage of secondary fibrinolysis, a site of intravascular coagulation. | Laboratory findings are in accordance with the above pathogenic mechanisms 1. Platelets count, coagulation factors and fibrinogen level : are decreased? or ‘consumed’ Asa result of above : The bleeding time as well as the coagulation time both are prolonged ° therefore PIT: increased @ PT: increased? Thrombin time : increased @ 2. Increased secondary fibrinolysis accounts for - raised plasmin levels raised levels of fibrin degradation product (FDP). © 3. The thrombotic phase accouns for features of microangiopathic haemolytic anemia, ° = presence of schistiocytes, spherocytes, burr cells, halmet cells in the peripheral film. ° |Some question asked + Most important treatment is : finding a reversible cause and treatment of the cause @ + Finding in DIC, that correlates best and most closely with bleeding is: fibrinogen level @ + Most common site for thrombin formation in DIC is : Brain? (Brain>heart=lung>kidney>adrenal >liver) + DIC is related to 2 very important endcrine manifestations : a, Adrenals - Fredrich Hausen Syndrome. b. Pituitary - Sheehan's Syndrome. © TTP: Thrombotic thrombocytopenic purpura - Revie “Thrombotic Thrombocytopenic purpura is a disorder of * vessel wall’® characterised by lesions in arteriolar walls in various organs that initiate formation of localised platlet thrombi and fibrin deposits at various sites. CLINICAL PENTAD OF TTP i 1 T [Microangiopathic Thrombocytopenia’ Decreased Renal _ Disturbed Neurological Fever ° |Hemolvtic Anemia” (due to consumption Function © function |(Coomb’s negative) of platlets) (due deposits in the Characteristically diffuse & Haemolysis” Renal Vasculature.) and non focal ° ‘eg- confusion + Fragmentation’ of RBC’s ~ aphasia - Increased LDH alteration in (clevated due to consciousness. intravascular hemolysis)” Remember :| Tests of cougulation are usually normal ° ~ NormalPT ® ~ Normal APTT® ~ Normal Fibrinogen concentration ° = Normal Fibrin degradation Products” Thus : PENTAD as mentioned above + normal coagulation tests: PATHOGNOMIC OF TTP143, 144. 145, /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 » 875 Answer is A (Hb electrophoresis) : Harrison 19/638; Nelson 17" /1630 Progressive increasing pallor along with hepatosplenomegaly in a five year old girl is consistant with a diagnosis of thallasemia. (Thalassemia intermedia) “Haemoglobin electrophoresis” isthe test of choice for diagnosing this condition and hence is the option of | choice here, Clinical spectrum of Thalassemia (B thalassemia) t { + ‘Thallasemia major Thalassemia minor ‘Thalassemia intermedia (erious homozygos form) (heterozygos form) (They are also homozygos) « Presentation in carly infancy» Presents late and patient can lead a « Patent present somewhere between the with: practically normal life except for two extremes with variable clinical + Progressive pallor mild persistant anemia manifestations of ~ Hepatosplenomegaly + Compatible with norma ite ~ Progressive pallor = Bony changes ~ Hepatosplenomegaly «Invariably fatal during first few ~ Bony changes years oflifeifleft untreated - ‘These patents maintain their ie fairly ‘eequire repeated blood comfortably and are not dependent on transfusions) blood transfusions for their survival. ‘© Thallassemia is a quantitative defect in Hb characterized by decreased synthesis of Hb chains that are structurally normal, ‘+ Medullary and extramedullary erythropoisis leads to expansion of bone marrow space causing a characteristic hemolytic facies with frontoparental, occipital bossing, malar prominance and malocclusion of teeth and comipications that include distortion of ribs and vertebra, pathological fractures of the long bones, splenomegaly and its complications — hypersplenism, hepatomegaly gallstone and leg ulcers. Answer is C (Haemochromatosis) : Harrisson 19°/2517 Haemochromatosis is a disorder of iron storage in which an in appropriate increase in intestinal iron absorption results in deposition of excessive amounts of iron in parenchymal cells with eventual tissue damage and impared function of organs. Diagnosis is established after assessment of parenchymal iron stores. Serum iron Increased Decreased (PéSittation of transferrin) a 350% snion esi saturation Decreased 770 ‘Serum ferritin Normal or Increased ‘Lead poisoning presents with : - omy Seecee anemia® with basophilic stippling®. = microcytic hypochromie picture may be seen = Elevated free erythrocyte protophyrin may be seen None of the conditions other than haemochromatosis present with an increased serum iron along with an increased serum ferritin and percent saturation of transferrin. Answer is D (Haemochromatosis): Harrison 17/2432, 2433, 63; 16"/672, 2302; 15/743, 2261; CMDT 2004/647 ‘© Haemochromatosis : The treatment options for haemochromatosis involve removal of excess body iron and supportive treatment of damaged organs. ‘Treatment for haemochromatosi = Removal of excess body iron ‘Supportive treatment of damaged organs 1. Phlebotomy: Conventional management of '* Weekly phlebotomies of 500 ml of aye blood continued for up to 2-3 years to | Calis fire achieve depletion of iron stores ‘+ Maintenance phlebotomy thereafter. 2. Chelation therapy with desferoxamine = Diabetes mellitus Bone marrow transplantation is not used as a treatment modality for haemochromatosis.147. "+ AIPGMEEXAMINATION ANSWERS AND EXPLANATIONS - 2008 © Osteopetrosis: Infantile malignant osteopetrosis is due to an inability of the osteoclasts to resorb bone and sinee osteoclasts derive from the marrow, transplantation can cure this rare inherited disorder. ~ Harrison 15/743 ‘* Hurler’s syndrome and Leukodystrophy: Marrow transplantation has been used as treatment for a number ‘of storage diseases caused by enzymatic deficiencies such as Gaucher's disease, Hurler's syndrome, Hunter’s syndrome and infantile metachromatic leukodystropby-Harrson 159743 Indication of bone marrow transplantation. iat - sot © Kostmann’ssyodrome ¢ Acute leukemia AML) ‘© Severe combined Associated with» Thalessemia major Kostmann’s syndrome immunodefficiency + Paroxysmal Sickle cell anemia + Chron granulomatous Chronic leukemia (CML) # Wiskot-Aldrich—noctumal * Leukocyte adhesion (CLL-limited) use syndrome hemoglobinuria «Black fan Diamond + Myelodyspasia # Chediak-Higashi * Fanconi anemia + Lymphoma syndrome snemia + Infantile malignant = Hodgkin's osteoporosis = Non Hodgkins + Storage disease + Myeloma = Gauchers disease «Sold tumours hurler’ syndrome = Ovarian cancer + Hunters syndrome Smal ec-lung cancer + Infantile metachromatic = Neuro blastoma leukody-srophy = diate sarcomas = Severe aquired autoimmune disorders. Answer is D (Patient presents early in life before 6 months of age) : Nelson 17* / 1624; Harrison 19/634 ‘+ Affected newborn seldom exhibit clinical features of sickle cell disease. Anemia develops over the first 2-4 ‘months paralelling the replacement of much of the fetal haemoglobin by HbS. Clinical manifestations are uncommon before 5-6 months of age’ ~ Nelson 17*/ 1624 ‘* The most important factor which affects the rate and degree of sickling (polymerization) és amount of HbS and its interaction with other Haemoglobin chains ~ Robbins (ors favouring polymerization: © Hypoxia? : (2,3 diphosphoglycerate increases polymerization®) Acidosis? : decreased pH enhances polymerization? Haemoglobin concentration®: Higher concentration leads to increased polymerization? ‘Combination of HbS with other haemoglobins: This depends on the extent of homology with other hhaemoglobins. Descending order of ability to copolymerization are HbS, C, D, O, Arab, A, J & F (Least with HOF) ‘* Acute splenic sequestration has a peak incidence between 6 months and 3 years and can be rapid in onset and lead to death. The most common cause of mortality is sepsis and acute chest syndrome’ Neon 174/1626 + Chronic transfusion therapy (repeated blood transfusions) has been used to decrease the frequency of crisis and may be useful in some severely affected patients - Harrison aere sign) : Harrison 17"/ 1490, 1491; 16/1416; 15" / 1367; Repeat Answer is D (Kussmat Clinical Pulsus paradoxus Common Usually absent Rare Rare Jugular veins Prominent y descent Absent Usually present Rare Rare Prominent x descent Present Usually present Present Rare Kussmaul's sign Absent Present Absent Absent Third heart sound Absent Absent Rare May be present Pericardial knock Absent Often present Absent Absent Maybe present May be present Absent Absent Absent Absent148, 149, 150, ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS-2004 > 877. ee 3 ‘Tamponade ‘Constrictive Restrictive RYML Bees, cs: Pericarditis Cardiomyopathy Echocardiography Thickened pericardium Absent Present Absent Absent Pericardial calcification Absent Often present Absent ‘Absent Pericardial effusion Present Absent Absent ‘Absent RV si Usually small Usually normal Usually normal Enlarged Myocardial thickness Normal Normal Usually increased Normal Right atrial collapse and Present Absent Absent Absent — RYDC Increased early filling, Absent Present Present May be present mitral flow velocity 2 Exaggerated respiratory Present ‘Absent Present Absent 6 ‘variation in flow velocity — femme 3S | Thickenedicaeific Absent Present Absent Absent s | peas | pericardium a Cardiac catheterization Usually present Usually present’ Usually absent Absent or 5 Equilizaton of diastolic present Le procedures | Cardiae biopsy helpful? No 7 No Sometimes No. * RY, right ventricle; RVML, right ventricular myocardial infarction; RVDC, right ventricular diastole collapse; BOG, electrocardiograph, Answer is B (Acanthocytes) : Harrison 17"/ 652, 1786; 16°607,1681; 15"/681 Acanthocytes are erythrocytes characterized by multiple spiny cytoplasmic protections (spur cells). These are seen as a consequence of abnormal lipid metabolism such as in severe liver disease and are not a feature of glomerular (renal disease) ‘+ Hacmaturia with dysmorphic RBC’s, RBC cast are characteristic feature of glomeulonephritis (crenated RBC are a type of dysmorphic RBC’s), Answer is A (Lung) : Repeat : Harrison 16/1696; 15" / 1600 Cysts in polycystic kidney disease may be seen in : = Liver: Hepatie ysts (60-70%) + Spleen = Pancreas = Ovaries ‘© Most common extrarenal sites of eyst formation is the Liver? ‘© Hepatic cysts occur in 50 ~ 70% of cases ® ‘© Cysts are generally asymptomatic and liver function is normal.? Answer is B (Chromosome-3) : Harrison 17%/ 592; 16"/541 1(80-3):15°/607; Robbins 6/993 Presence of painless haematuria with balottable mass in right flank in this forty years old male suggests diagnosis of renal cell carcinoma, There are three major histological variants of renal call carcinom (Clear cell eareinoma (Non 9) Papitiary carsinoma ‘Chromophobe renal papillary) carcinoma ‘ Most common? type® (70-80%) * Second most common type (10-15%) «Least common (5%) + Cellshave cleat®or granular Papillary® growth patter is characteristic (lear cell not Pale eosinophilic cytoplasm and are non papillary seen) etoplasin ‘+ Psammoma bodies may be present ‘Defect lies in chromosomes 3® © Most common cytogenetic abnormality are ~ = Multiple = isomy of chromosome 7, 16 4 17 and loss of Y in chromosomal losses mae patents in sporadic form and extreme = wisomy 7 in familial form haploploidy © 2 Striking characteristic of tumor is: Tendency to invade the renal vein and grow as a solid column of cells within the vessels. Extension may produce a continuous cord of tumor in IVC and even in the right side of heart878 + AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 151, Answer is C (Doxycycline) : Harrison 14"/ 804 ‘Chlamydia trachomatis is the most common cause of non gonococcal urethritis.? ‘Treatment regimen effective for gonococeal urethritis ice daily for even days. goorally in a single dose ‘As Azithromycin has not been provided in the options, doxycycline is the answer of choice. 152. Answer is C (Cytomegalovirus) : Kanski 5" /288; Harrison 16/1119; 15"/ 1895 CMV retinitis is the most common ocular infection in AIDS patient®. CMV retinitis, Presentation: Presents as a painless progressive loss of vision, Patients may also complain of “blurred vision’. Floaters and scintillation. | Opthalmoscopic findings: (Bilateral) ° ‘Characteristic retinal appearance of perivascular haemorrhages and retinal exudates ° — Harrison Cotton wool spots ° appearing as a result of focal ischaemia of nerve fibre layer are characteristic (cystoid bodies) °- Neema Risk factors: ‘The majority of cases of CMV retinitis occur in patients with CD4 count < 50/ul. 2 Patients with CD4 + count < 100 / il should undergo an ophthalmic examination every 3 — 6 months. ‘Treatment: Therapy for CMV retinitis consists of intravenous ganciclovir? Forscamet (cidofoviris an alternative) istration of nucleoside 153. Answer is A (Following needle stick injury infectivity is reduced by adi analogues) : Harrison 16/1081, 1086-87, 1103; 15 / 1857 ‘A prospective study has indicated that use of antiretroviral drugs as postexposure prophylaxis decreases the risk of infection compared to historic controls in occupationally exposed health care workers"=Harrison 151857 © The risk of HIV transmission following skin puncture from a needle or sharp object that was contaminated with blood from a person with documented HIV infection is approximately 0.3%.° [The risk of hepatitis B infection following a similar type of exposure in a non immune person is 6-30% 2] ‘A prospective study has indicated that use of antiretroviral drugs as postexposure prophylaxis decreases ‘he risk of infection compared to historic controls in occupationally exposed health care workers" Harrison 15" / 1857 T Recommendations for axis: 1, A combination of two nucleotide analogue reverse transcriptase inhibitors should be given for 4 weeks for routine exposures. 2. combination of two nucleotide analogue reverse transcriptase inhibitors plus a protease inhil for four weeks for high risk or otherwise complicated exposures. © Five factors have been identified with an increased risk of ocupational transmission of HIV infection: Deep injury Presence of visible blood on the instrument eausing the exposure. Injuey with a device that has been placed in the vein or artery ofthe source patient ‘Terminal illness of the source patient. Lack of post exposure retroviral therapy in exposed health worker. itor should be given + The best predictor of disease progression i.e. ‘prognostic indicator’ is HIV RNA in serum or plasma and not CD4 + counts (CD4 counts indicate the current immunological status). “While CD4 + counts provide information on the current immunological status of the patient, the HIV RNA level predicts what will happen to CD4 + cell counts in near future and hence provides an important piece of prognostic information” ~ Harrison 15! / 1878154, 155. 156. /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004» 879 Set of tools for determining prognosis and monitoring response to treatmes 1. CD4+T cell count 2. HIV RNA + Microbial latency does not occur in patients with HIV infection during the period of clinical latency. ‘For most viruses other than HIV (eg. HSV) clinical latency is accompanied by microbiological latency ie viruses are not completely cleared from the body after infection and enter a latent state. This is not the case with HIV virus in which some degree of virus replications invariably occurs during the period of clinical latency' — Harrison 15" / 1863 Harrison 15" / 1863 again states that ‘I was originally thought that vry littl virus replication occurred during clinical latency. However studies of lymphoid tissue using PCR analysis for HIV RNA and in situ hybridization for individual virus expressing cells clearly demosntrated that HIV replication oceurs throughout the course of HIV infection even during clinical latency. ‘+ HIV infection is relatively unique among human viral infections. Once infection has been established the virus is virtually never cleared completely from the blood. Rather a chronic infection develops that persists with varying degree of virus replication for a median of approximately 10 years before patient becomes clinically ill. This establishment of “chronic persistant infection’ is the hallmark of HIV disease. Answer is C (Nevivapine): KDT 5"/728-731; Park 17/266, Park 18"/280; Antiretroviral agents for HIV Reverse transcriptase inhibitors Protease inhibitors L + Saquinavir Nucleoside Non nucleoside reverse Ritonavir inhibitors. transcriptase inhibitors Indinovir Ziduvudine Nevivapine Nelfinovir idanosine Ifavirenz Zalcitabine: Delavirdine| ‘Stavudine Lamivudine] Answer is C (Ritonavir) : Harrison 16"/1108; 15" / 1883 “Therapy for TB is generally the same in HIV infected patients as in HIV negative patients. Due to pharmacokinetics interactions rifabutin should be substituted for rifampicin in patients receiving HIV protease inhibitors or nonnucleoside reverse transcriptase inhibitors. Both drugs should be avoided in patients receiving ‘ritonavir’ ~ Harrison Thus, although rifampicin should be avoided with both non nucleoside reverse transcriptase inhibitors (Efavirenz) and protease inhibitors (Indinavir) a substitution with rifabutine should suffice. ‘The answer of choice here is Ritonavir as with ritonavir, both rifambutin and rifampicin should be avoided, Answer is B (Long term status of blood sugar) Harrison Harrison 17°/2296; 16/2172; 15" / 2129 ‘Measurement of pleated haemoglobin is the standard method for asesing longterm glyaemle control” - Harrison 158/2129 When plasma glucose is consistantly elevated there is an increase in non enzymatic glycation of haemoglobin — stnall amount of HbA are nonenzymatically glycated to form HbA. This alteration reflects the glycemic history over previous 2-3 months (since erythrocytes have an average life span of 120 days) Remember: HbA c measurement becomes unreliable in conditions like - Haemoglobinopathies” ~ Hemolytic anemia? = Uremia? An alternative indicator of glycemic control when HbA,c is inaccurate is: glycated albumin 2 (glycation of other proteins such as albumin)‘880 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 157. Answer is A (She was suffering from insulin dependant diabetes mellitus) : Harrison 16/2156; 15"/ 2114; 17°17°/2281; CMDT 2004 / 1149, 1150; API7"/ 1105 Q ion through the conceptual differences between type I & type II diabetes will help us in solving this, particular problem. Patho- « Results from complete or absolute lack of # Results from insufficient insulin seretion relative to physiological insulin caused by a reduction in B cell slucose load ‘or “insulin resistance’. inability of difference mass.2 ‘peripheral tisues to respond to insulin '* Autoimmunity @ is the major culprit ‘© Genetic factor are the major culprit + Autoantibodies are present ® in blood ~ No autoantibodies ° (gains islet cells) = NoHLA association ® ~ HLA DR3 / DR¢4 are ° expressed in 95% of patients = (Genetic factors are of low significance) ‘Age <40 years? > 40 years? Habitus Non obese Obese? Presentation Patient present with sudden onset of Patients present with a more gradual /insiduous onset 2 ‘osmotic symptoms inthe form of polydipsia, of esmotic symptoms (may not be present) polyuria or symptoms of ketoacidosis Many of them may be asymptomatic ? and are recognized when they develop one of the macrovascular ‘or microvascular symptoms of diabetes like delayed healing of wounds with atherosclerosis or gangrene or peripheral neuropathy etc. *# Ketoacidosis prone? ‘* Ketoacidosis not prone ° (Non ketotic hyperglycemic coma) Laboratory» Blood insulin is decreased «+ Insulin Levels are normal or may be increased parameters {explained by insulin resistance) + Blood glucagon + Blood glucagon High /supresible High resistant ‘* Ketonuria: often present + Not present Pathology « Early insulitis * No insulitis ‘© Marked atrophy and fibrous + Focal atrophy and amyloid '* Beta cell depletion * Mild B cell depletion ‘Treatment | Nutrition therapy + Exercise Nutrition therapy + Exercise Protocol | (to coordinate and match the calorie | (to reduce calorie intake, reduce hyper- lipidemia intake with appropriate amount of |__| and hypertension and promote weight loss) insulin) Oral hypoglycemia agents (often sufficient) foot controlled | Development of Complication (Pregnancy infection, surgery) Insulin Insulin (usually in combination) Points which favour a diagnosis of non insulin dependant diabetes mellitus (type 2) in this question: 1, Age> 40 yrs (45 yrs) 2. Complication in the form of impalpable pulses in feet, gangrene at presentation along with osmotic symptoms (polyuria, polydipsia) hint to the possibility of occult disease which is likely to have been present for some time prior to diagnosis. Note: This type of diabetes goes unrecognised for many years because the hyperglycemia develops gradually ‘and in the early stages it is often not severe enough for the patient to notice any of the microvascular complications of diabetes at presentation e.g. peripheral neuropathy hypertension, atherosclerosis, dyslipidemia, 3. Absence of Ketonuria: Ketonuria is a feature of type —I diabetes. As depicted in the comparitive analvsi ‘* Family history (genetic tendency) is more important in type II disease and is useful in predicting the nature of diabetes ‘© Protocol for management of type Il diabetes involves diet control and exercise as the primary management, followed by oral hypoglycemics and still further by insulin if pathological situations can not be controlled.158, 159, 160. Answer is C (TSH) : Harrison 16/2108; 15"/ 2064 * The enhanced sensitivity and specificity of TSH assays have greatly improved laboratory assessment of ‘thyroid function — Harrison + With rare exceptions a normal TSH level excludes a primary abnormality of thyroid function. ~ Harrison ‘Approach to thyroid testing]: Because TSH change dramatically in response to alteration of free T, and Ty, a logical approach to thyroid testing isto determine first whether TSH is supressed, normal or elevated — Harrison Determine levels of TSH as first step Euth Hypothyroids Measurement of circulating thyroid hormone levels {should be done as a second step on finding an abnormal TSH level) Radio immune assay for serum total T3 and serum total T4 to confirm the diagnosis of hypothyroidism (1 TSH) or hyperthyroidism (TSH) Answer is A (Jod-Basedow effect) : Harrison 17/2241; 15" / 2077; CMDT 2004 / 1093 Jod Basedow disease is synonymous with iodine induced hyper thyroidism and may occur in patients with multinodular goitre after intake of large amount of iodine = inthe diet = inthe form of radiographic contrast material = _ inthe form of drugs especially amiadarone Contrast agents and other iodine containing substance should be avoided because of risk of inducing the ‘Jod ~ Basedow effect’ characterized by enhanced production of thyroid hormone by autonomous nodules’ — Harrison ‘Thyrotoxicosis factitia: Thyrotoxicosis produced due to ingestion of excessive amount of exogenous thyroid hormone is called Thytotoxicosis facttia. Epidemics of thyrotoxicosis have been caused by consumption of ground beef ‘contaminated with bovine thyroid gland. Presentation is one of thyrotoxicosis with low or absent thyroid nucleotide uptake. Wolff — Chaikoff effect: Harrison 15" / 2062, 2073, 2076 Excess iodine transiently inhibits thyroid iodide organification. This phenomenon of iodine dependent transient supression of thyroid is called Wolff-Chaikoff effect. De Quervon’s thyroiditis is same as subacute thyroiditis, granulomatosus thyroiditis or viral thyroiditis Answer is B (Hypothyroidism is more common in men) : Harrison 17°/2239, 2240; 16"/2119; 15" / 2076 ‘Amiadarone associated hypothyroidism is more common in women and individual with positive TPO antibodies? ~ Harrison 15" / 2076 Amiadarone is a class III antiarrythmic agent structurally related to thyroid hormone and containing very high amount of iodine, Facts: * Typical dose of amiadarone (200 mg/d) are associated with very high iodine intake, leading to > 40 fold inerease in plasma and urinary iodine levels. Amiadarone is stored in adipose tissue and high iodine levels therefore persists for > 6 months after discontinuation of drug Mechanism of action®: Amiadorone inhibits deiodinase activity.° Initiation of Amiadarone treatment | = Inhibitory effect of iodine on T, release = Wolff Choikoff effect: Excess iodine transiently inhibit thyroid iodide organification ‘Transient decrease in T4 levels (Hypothyroidism) | = Escape from Wolff Choikoff effect (most individuals escape from iodide dependent supression of thyroid) = Inhibition of deiodination (deiodination is required for conversion of T to Ts.) © Enhanced T, levels © Decreased 7; levels / increased Ts © Elevated TSH levels (TSH levels normalize or are slightly supressed after about 1-3 months)161. ° Possible effects of Amiodorone on thyroid dysfunction: Almost any change in thyroid status can be produced by amiodarone ——+——, Acute transient Hypothyroidism Hyperthyroidism changes in thyroid In patients susceptible to May be caused by three mechanism function inhibitory effect of a 1, Jod basedow effect: from iodine load in setting high iodine intake of multinodular goitre 2. Thyroiditis like condition 3. Autoimmune Grave's disease Amiadarone associ thy roidism: Incidence: Correlates with iodine intake and varies from 13% in iodine replete countries to 6% in iodine depleted countries. Pathogenesis : Involves an inability of thyroid to escape from high iodine load, Consequently amiadarone associated hypothyroidism is more common in women and in individals with positive TPO®. Treatment: Levothyroxine can be used to normalize thyroid function. It is usually unnecessary to discontinue amiadarone for this side effect. Amiadarone associated hyperthyroidism: Incidence: Coorelates with iodine intake and varies from 10% in iodine depleted countries to 2% in iodine replete countries (high iodine intake) Pathogenesis: Two types of Amiadarone induces thyrotoxicosis (AIT) are seen — ‘Associated with an underlying thyroid abnormality Associated with individuals with no intrinsic thyroid = preclinical gravis abnormality = multinodular goitre ‘Thyroid hormone synthesis becomes excessive as a Thyrotoxicosis results from drug indused lysosomal result of increased iodine exposure - Jod basedow activation leading to destruction thyroiditis. ‘phenomenon This can resolve spontaneously or can occasionally lead to hypothyroidism Increased vascularity is seen on color-flow dopler Decreased vascularity is seen on color flow dopler scan - ‘© Amiadarone should be stopped if possible = This is often not possible because of underlying cardiac disorder = Discontinuation will not have any acute effect because of its storage and prolonged half life. # Potassium perchlorate: has been used to reduce thyroidal iodine content * Lithium : blocks thyroid hormone release and ean provide modest benefits. ‘High doses of Antithyroid drugs may be tried in Type I - AIT (often ineffective) * Glucocorticoids may be used for subacute thyroiditis in Type Il - AIT + Near total thyroidectomy rapidly decreases thyroid hormone levels and may be the most effective longterm ‘treatment. Answer is B (Plasma cortisol) : Harrison 16/2110, 2134; 15" /2067, 2091; API Medicine 7" / 1058 ds ‘The clinical presentation of patient in question gives us the followin; 1. Weight gain (10 kg) 2. Hypertension (160/100) (both systolic and diastolic hypertension for a 28 year old lady) 3. Menstrual irregularities inthe form of oligomenorthea followed by amenorthea.162. [AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 » 883 Cushing's syndrome b. Hypothyroidism Cushing's Syndrome: Signs and symptoms of presentation in decreasing order T. Typical habitus (centripetal obesity)? 8. Personality changes? 2, Increased body weight : Weight gain® | 9. Echymosis? 3. Fatiguability and weakness? 10. Proximal myopathy? 4, Hypertension® > 150/90 mm Hg) 1, Edema’ 5S. Hiruitism? 12. Polyuria / Polydipsia 6. Amenorrhea? 13. Hypertrophy of clitoris 7._Broad violoceous cutaneous stria? As all features in the presentation are consistent with a diagnosis of Cushing's Syndrome and there are no points to doubt / disfavour our diagnosis, plasma cortisol would be the most appropriate investigation which ‘would confirm our diagnosis. Hypothyroidism: Although hypothyroidism may present with all features in the question the points which go ‘against probable diagnosis are: 1. Presence of both systolic and diastolic hypertension as against mild diastolic hypertension expected in hypothyroidism, 2. Presence of oligomenorrhea followed by amenorrheo as against menorrhagia initially which is a much ‘more common form of menstrual irregularity in hypothyroidism. ‘Hypothyroidism: Clinical Manifestation 1. Skin Day scaly skin 2 Loss of hair® from sealp and body Generalized oedema ° intially pitting Britle nails (ent ‘Hoarseness of voice? and slowness of speech? : impaired hearing? Constipation? = Weight gain® & loss of appetite? 3. Gastrointestinal tract = Bradyeardia? Ai Cardiovascular system Mild diastole hypertension? é + Breathlessness? 5. Newological Physical and mental slowness®, lethargy and somnalence® Delayed tendon reflexes? Paraesthe [lFeitale reproductive Menorthagia® (Later oligomenorrheo and amenorthco) a system Galactorthea® (@/t hyperprolactinemia® due to high TRH) a 7. Miscellaneous (Carpal tunnel syndrome” Serous cavity effusion? Answer is A (Loss of Diurnal variation) : Harrison 17"/2256; 16/3135; 15" /2092; AP 17/1072 Owing to circadian variability plasma cortisol and to a certain extent ACTH determinations are not ‘meaningfull when determined in isolation, but the absence of normal fall of plasma cortisol at midnight (Loss of diurnal variation) is consistent with Cushing’s syndrome” - Harrison 15* /2092 From the statement above, options of increased ACTH and increased plasma cortisol are ruled out as desirable early manifestation of Cushing's syndrome. We are then left with the options of loss of diurnal variation and increased urinary metabolites of cortisol. Although ‘ree cortisol’ in urine is used as a screening test for Cushing's syndrome, no mention has been made about use of ‘increased metabolites of cortisol in urine’ for the same purpose. Secondly, manifestation in serum level of cortisol are certain to precede changes in urine and hence loss of diurnal variation in serum cortisol is certain to be the earliest manifestation of Cushing's syndrome amongst the options provided.884 163. ‘» AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 Diagonastic Protocol Signs + Symptom: + jion rhythym — Loss of diurnal variation (8.00 AM & Mid night) : API 7®/ 1072] Innormal individuals the serum levels of cortisol reach their lowest levels at midnight. Inpatients with Cushings this diurnal variation is lost and serum levels of cortisol remain same throughout 4 hours. Y Screening Test dF Low dose dexamethasone supression test 24 hours urinary free cortisol levels ‘* Img dexamethasone at midnight and Plasma cortisol level determined next day 8.00 AM Elevated > 140 n mol / day > 140 nmol / day ot >Sug/dl > 50 ne/ day ‘© 0.5 mg dexamethasone 6 hourly for 48 hours and plasma cortisol level determined after 45 hours > 140 nmol / day >Sug/dl + High dose dexamethasone supression test (If abnormal findings are recorded a dexamethasone supression test with high doses is ‘carried out) (2 mg 6 hourly) | (Supression No response + Adrenal hyperplasia? ‘+ Adrenal hyperplasia secondary to ectopic ACTH producing tumor® (high ACTH) ‘+ Secondary to pituitary ACTH secretion + Adrenal neoplasia? (low ACTH) Further distinction between adrenal neoplasia and adrenal hyperplasia secondary to ectopic ACTH producing tumor and their causes can be carried out as follows- >————41——_ High ACTH Low ACTH Adrenal hyperplasia secondary to ACTH Adrenal neoplasia producing tumor [Abdominal CT scan Pituitary imagi Selective venous sampling Urinary, 17 kS or DHEA sulphate Lv Positive Negative High (> 6em) Normal — Low (<3 em) Pituitary tumor Ectopic tumor Adrenal carcinoma Adrenal adenoma Answer is D (Congenital Adrenal hyperplasia) : Harrison 17"/2345; 16/2088, 2134; 15" / 2042, 2178 Congenital adrenal hyperplasia is acompanied by increased levels of sex steroids and is thus associated with accelerated bone maturation. ‘Several pathological conditions accompanied by increased levels of sex steroids including precocious puberty, androgen exposure (exogenous or endogenous), congenital adrenal hyperplasia and obesity are associated with accelerated bone maturation.’ - Harrison 15! / 2042 ‘In both sexes rapid somatic maturation results in premature epiphyseal closure and a short adult height’ = Harrison 15* / 2179.164. 165, /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS -2004 * 885 Answer is D (Complete androgen insensitivity syndrome) : Harrisson 17"/ 2344; 16/2218; 15" /2182 [A 46 XY karyotypic male presents witha truly feminine appearance with complaints of primary amenorrhea (third most common cause of primary amenorrhea after gonadal dysgenesis and congenital absence of vagina) Mature breast development - Habitus and distribution of body fat are female in character = Scalp hair is that of normal women and facial hair is absent (no hiruitism) External genitalia is unambiguously female and clitoris is normal - Internal genitalia : All intemal genitalia are absent except testes that contain normal leydig cells and seminiferous tubules but without spermatogenesis. The patient in question presents as a 21 year old women (truly feminine appearance) with well developed breasts, no hiruitism, no pubic or axillary hair with primary amenorrhea. All features thus mentioned lead to «a diagnosis of complete androgen insensit Also remembs * The testes may be located in the abdomen, along the course of inguinal canal or in he labia majora (undescended testes)? ‘© The major complication of undescended testes in this disorder is the development of tumors. ° ‘Turner's Syndrome : Gonadal dysgenesis ‘A phenotypic female presents with primary amenortheo. Pubic and axillary hair remain sparse and breasts are infantile (patient in question has mature breasts) Stein Leventhal syndrome: Polycystic ovarian disease: Harrison 15" / 2163 This is characterized by Hirsuitism (patient in question has no hiruitism) along with amenorrhea / oligomenorthea, obesity and infertility. Premature ovarian failure: Presents with secondary amenorrhea and not primary amenorthea. Answer is B (prolactinoma) : Harrison 17"/ 2206; 16/2086; 15" / 2041 % ‘Menstrual eye disturbance in the form of amenorrhea along with galactorrhea suggests a diagnosis of Iyperprolactinemia. Amongst the options provided the tumor most likely to produce above endocrine manifestation is a prolactinoma. Prolactinomas:) * Most common functional pituitary adenomas? '» Microprolactinomas are more common than macroprolactinomas® [Clinical presentation: ‘Symptoms due to hyperprotactinemia ‘Symptoms due to mass effeet (more common seen with both microadenomas Less common ‘and macroadenomas) Seen with macroadenomas + Galactorthea® © Headache ‘© Amenorrhea? © Visual field defects ‘+ Inferitity © © Other mass effects (Hyperprolactinemia from any cause produces Hypogonadotrophic hypogonadism) ‘Treatment: Bromocriptine” is the therapy of choice It is a dopamine agonist® agent and is an effective prolactin lowering agent. ° [Craniopharyngiomas}; these tumors arise from remnants of Rathke’s pouch. ° Although craniopharyngiomas ‘ay also produce hyper prolactinemia, purely endocrinal manifestations in the form of galactorrhea and amenorthea are unlikely with craniopharyngiomas. These tumors: 1. Present mostly in childhood 2. Most common presentation features are: + Features of raised ICT ~ Visual abnormalities * (hypogonadism is seen in about 35% of cases) and accompanies the above manifestations) jeningiomas and SAH] will not produce symptoms of hyperprolactinemia alone.& a x 3 8 8 mm 2 & = = 166. 167. | ANSWERS AND EXPLANATIONS - 2004 Answer is A (Lung perfusion - ventilation sean) : Harrison's 17"/ 1652, 1663; 16"/1562-63; 15th/1510, 1511 The clinical picture depicted in the question is suggestive of a case of pulmonary thromboembolism. ~ An elderly man on bed rest for past 10 days: predisposition for deep vein thrombosis. = Complaints of breathlessness and chest pain: indicative of pulmonary embolisation. (A chest X-Ray in patient with PTE may well be normal or near normal - Harrison's 1Stv510) Diagnostic Strategy in eases of suspected Pulmonary Thromboembolism NON IMAGING DIAGNOSTIC MODALITY (Blood test) ‘Quantitative Plasma-D dimer ELISA (Reflects plasmin breakdown of fibrin and indicates endogenous thrombolysis) + HIGH + NON INVASIVE IMAGING MODALITIES Lung Ventilation Perfusion Sean Normal or Near Normal ‘Non Diagnostic High Probability + + + Stop workup Leg ultrasonography Treat Definite DVT: Treat Normal + INVASIVE DIAGNOSTIC MODALITY Pulmonary Arteriogram + In accordance with the diagnostic strategy for PTE outlined in Harrison's textbook the frst investigation would have been a blood test for estimating D-Dimer by ELISA technique. + As this is not provided amongst the options we move to the next step i.e. Lung Ventilation Perfusion Scan. Remember — + Most specific test for establishing a diagnosis of pulmonary thromboembolism is selective pulmonary angiography.® + Most specific non invasive test for establishing a diagnosis of Pulmonary thromboembolism is Lung ventilation - perfusion seant. Answer is A (Wegener’s granulomatosis) : Harrison 16"/2005; 15" / 1961, 1962; CMDT 2004 /816 Wegener's granulomatosis in its classical form presents as characteristic triad of ~ = Upper respiratory infection (ENT manifestations) : includes otitis media (conductive deafness) = Lower respiratory tract infection (Lung) : includes haemoptysis and radiological cavity. ~ Renal involvement: includes red cell casts and raised serum creatinine Skin lesions are seen in 46% of patients and may take the form of palpable purpura. ‘The patient in question has been depicted to have one manifestation each of the classical triad along with skin lesion in the form of palpable purpura and hence the answer is clearly Wegener’s granulomatosis. Wegener’s granulomatosis ‘Wegener's granulomatosis is a distinct clinicopathological entity characterised Glinically * Triad of Upper respiratory tract Lower respiratory tract” Renal manifestations? manifestation (ENT) manifestations (Lung) = Otitis media (condastve = Cough, haemopiyais, dyspnea” | Rapidly progressive renal failure” seatness) = Multiple bilateral cavitatory | Crescentrc glomerulonephrits? = Paranasal sinus pain & nodular infiltrates drainage = Septal perforation? 2 Other manifestations + Fever®, skin manifestation? in the form of papules, vesicles, palpable purpura ete eye manifestations®, joint manifestations” cardiac manifestations” and nervous system manifestations.168. AIPGME EXAMINATION ANSWERS AND EXPLANATIONS 2004» 887 Pathologically by © Necrotizing vasculitis” of small? arteries and veins © _ Intravascular and extravascular granuloma formation 2Granulomas contain multiple well defined multinucleated giant cells Bronchoalveolar lavage fluid contains high percentage of nutrophills compared to other granulomatous diseases which contains increased numberof Lymphocytes "ANCA is the predominant ANCA in the disease. Characteristic laboratory findings include: 1. Positive e-ANCA‘ 2, Elevated ESR, RF? (mild elevation) 3. Hypergammaglobulinemia (particularly IgA class) ° 4. Hypocomplamentemia is not seen despite presence of circulating immune complexes? ‘Treatment of choice is : cyclophosphamide? Answer is B (Polyarteritis Nodosa) : Harrison 16%/2007-08; 15" / 1959 ‘The clinical presentation of patient in question gives us the following ‘Ieads’ in approaching the diagnosis. = Digital gangrene in the setting of normal peripheral pulses. + Presence of hypertension + Negative antinuclear antibodies + Negative anti dsDNA + Negative ANCA Polyarteritis Nodosa: PAN ‘Classic Polyarteritis nodosa is characterized by necrotizing inflammation of medium sized muscular arteries. * Inclassic Polyartertis Nodosa, skin findings include livido reticularis, subcutaneous nodules and skin ulcers. Digital gangrene? is not an unusual occurance ~ CMDT/ 2004/813 ‘* Involvement of renal arteries leads to renin mediated hypertension, ‘Patients with classic PAN are ANCA negative — CMDY 2004/814 ‘© Positive ANCA (pANCA) are found in low percentage (< 20%) of patients with classical PAN iris [ANCAS that produce a p-ANCA pattem on immunofluorescence testing are frequently associated with microscopic polyangitis and not classical PAN. ‘© Polyarteritis nodosa is a term given to necrotizing arteritis of medium sized vessels. - CMDT 2004 Microscopic polyangitis may involve medium sized as well as small blood vessels and its specrtrum overlaps that of PAN. ‘The clinical presentation of patient in question is consistant with a diagnosis of PAN. ‘Takayasu's disease is also known as ‘pulseless disease’ and absence of peripheral pulses is a clinical hallmark of disease. This should be suspected strongly ina - [Young women with - | decrease or absence of peripheral pulses? ~ | discrepancy in arterial pressure ~ | arterial bruits The presence of all peripheral pulses makes this diagnosis unlikely. SLE ‘Antinuclear antibodies 98% ae sensitive test (AntiDSDNA 170% 0) Absence of ANA and Anti ds DNA almost rules out the possibility of SLE as a probable diagnosis. Wegener's granulomatosis is a necrotizing granulomatous vasculitis of small arteries and skin manifestations does not include digital gangrene. Antineutrophil eytoplasmic antibodies (ANCA) occur is about 90% of patients with active Wegner’s ‘granulomatosis. ‘Absence of ANCA in blood strongly points against a diagnosis of Wegner's granulomatosis.170. Answer is A (CD4/CD8 counts in the blood) : Harrison 16"/2019; 15"/1970; Not in Harrison 17 The patient in question is suffering from sarcoidosis, “While there is marked enhancement of no. of Ty cells atthe sites of disease the number of Ty cells in the blood are normal’ — Harrison ‘Where as Ty, cells in the involved organs are proliferating at an enhanced rate the T cells in other sites such as blood are quiescent’ - Harrison Itis the CD4/ CD8 counts in the affected organs which is an important ancillary aid in established the diagnosis. of sarcoidosis and not the CD4 / CD8 count in the blood as specifically asked in the question. Active sarcoidosis results from an exaggerated cellular immune response resulting in accumulation of large no. of T4 cells in the affected organs, where the ratio of CD4 to CDB cells may be as high as 10:1 Although CT of chest is also only rarely helpful, it may identify early fibrosis and active alveolitis and thus contribute to a diagnosis of sarcoidosis. Diagnostic tests for sarcoidosis have often been asked Pees © Blood: ACE® BIOPSY © Xray chest? ‘© Skin test for anergy? © Gallium 67 etc. 1, X-Ray chest: Bilateral hilar lymphadenopathy is the hallmark of disease Parenchymal involvement may or may not be present (clear lung fields) ° Stage T jateral hilar lymphadenopathy alone (no parenchymal involvementiclear lung fileds) Stage Tl Bilateral hilar lymphadenopathy + parenchymal involvement Stage II Parenchymal involvement alone Egg shell calcification of hilar nodes may be seen® 2. ‘Kveim silcbach test: Skin tes? intradermal injection ofa heat treated suspension ofa sarcoidesssplen extract. 3. Angie coneringengme (ACE) Bod The cls in rama ees egies comering ezine ACE is elevated in 2/3 of patients with sarcoidosis? (60%)? ACE levels indicate activity of disease? ACE levels provide an imporatnt method of monitoring the course of disease. 4. Gallium 67 can_ Gallium 67 lng scan is usually abnormal showing a pater of defuse uptake 5. Bronchoalveolar large: Lavage Mud is characterized by = increase in Lymphocytes? = high CD4 / CD8 cell ratio® iopsy : Biopsy is mandatory to make a difinitive diagnosis of sarcoidosis Characteristic finding is atypical non casseating granuloma? Lung function abnormaies : Typical fr interstitial lung disease. Answer is C (Monoclonal gammopathy of unknown significance) : Harrison 19°V/714 ‘Patients with monoclonal gammopathy of uncertain significance have < 10% bone marrow plasma cells, < 3g/ dL of M component’, - Harrison 15" /730 These are monoclonal neoplasms characterisized by expansion of a single clone of immunoglobulin secreting cells (plasma cells) and result in increase in serum levels of a single homogenous ‘immunoglobulin’ or ~ its fragments (light chains or heavy chains) ‘The most important differential diagnosis in patients with multiple myeloma involves their distinction from individuals with MGUS or Benign monoclonal gammopathy:im. [AIPGME EXAMINATION ANSWERS AND EXPLANATIONS -2004 889 Lee $Y /Mattipte myeloma Monoclonal gammopathy of Sh undetermined significance 1. Age > 30 years > 50 years firincidence 2 Less common More common 3. Plasma cells ® (age) > 10% < 10% [BEMeomponent® (concentration: g/dl) > 3g/dl <3yidl 5. Benze Jones ® proteins Present [No urinary Bence Jones proteins cal features? ~ Lytic bones lesions No Lytic bony lesions? ~ Hypercalcemia No hypercalcemia’ E = Renal failure No renal failure? = Anemia No anemia? 7. Labelling index (Thymidit >I =1% Ti = i High Low 9. Treatment Required No therapy required Woldenstorm’s macroglobulinemia: is also a monoclonal gammopathy and is characterized by neoplasm of {lympho plasma cytoid cells that secrete IgM (M compartment is only IgM and not IgG) and is characterized clinically by = Lymphadenopathy = Hepatosplenomegaly = _ Hyperviscosity syndrome Indolent multiple myeloma (IMM): © Lies in the spectrum between MM and MGUS ‘+ Unlike MGUS, patients with IMM have: [ > 10% plasma cells > 3g/ dl of M protein| but clinically unlike patients with MM these are mostly = asymptomatic - patients have mild non progressive anemia but no bone lesions, hypercalcemia or renal failure ‘¢ Most common patients progress to overt MM in < I years Answer is B (Wilson’s disease) : Harrison 16/2314; 15/2274 Psychiatric manifestations in the form of emotional lability along with neurological manifestations in the ‘form of tremors and the characteristic ‘Kayser Fleisher Rings’ (golden brown discolouration of descemet’s ‘membrane) are diagnostic of Wilsons disease. Presented below is a detailed note on Wilson’s disease, encompassing most questions formed on this important topic: Basie defect in Wilson's disease is reduced biliary excretion of Cu (anutation of a gene on chromosome 13° which promotes Cu excretion) (ATPTB gene) V Accumulation of Cu in hepatocytes? Coonibs negative hemolyiie All forms of hepatitis including fulminant type® anemia seen = Acute hepatitis © Renal tubular acidosis + Fulminant hepatitis though rare, may be seen = Chronic active hepatitis = Cirthosis Cu saturates liver, spills in plasma and spread @ to other systems (serum ceruloplasmin is 14) Copper deposit in basal ganglion (CNS) Eyes Free copper in Kidney + (Neurological manifestations) + Copper gets deposited in intrauterine secretions - Microscopic + Tremors resting / intention? Descemet’s? membrane of ~ primary secondary haematuria ~ Spastcty rigidity comea.2 amenorthea ~ Nephoealeemia + Chores - Kayser leischner ring Recurrent abortions ~ Renal calculi + Drooling / dysphagia / dysarthria (KF rings do not interfere [Sensory Changes Never Occur] abel « Sunilower cataract? Psychiatric manifestations Diagnosis is confirmed by demonstration of ether. © A serum ceruloplamsin level <200 mg/L. + KF ring .A serum ceruloplasmin evel < 200 mg/L_+ Cun liver biopsy > 250 uw/g dry weightAIPGMEE 2004 - EXPLANATIO 173. ‘Answer is D (Lewy bodies) : Harrison 174/ 2540, 2541, 2542; 16/2403; 15* /2396; Robbins 6 ‘Lewy bodies" are intraneuronal, intracytoplasmic, eosinophilic inclusions that have a dense core surrounded by a pale halo. They are composed by Neurofilaments and contain epitopes recognized by antibodies against neurofilament proteins, Ubiqutin and a-synuclein (presynaptic protein) They are characteristically seen in idiopathic parkinsons disease (and notin Alzheimer's disease) Alcheimer’s disease is the most common cause of dementia in the Wester world and is characterized by the ical and biochemical triad ‘© Subtle onset memory Gross? Microscopic * Decrease in cerebral cortical loss followed by « Diffuse atrophy © Neuritic plaques levels of acetyl choline? « Slowly progressive of cerebral containing f amyloid (Choline acety transferase (CAT) dementia over several cortex « Neurofibrillary° ‘and nicotinic cholinergic years * Secondary tangles receptors are also reduced) enlargement of « Amyloid angiopathy (Reduction in ACH is related ventricular ‘accumulation of B to degeneration of nucleus system amyloid in arterial besalis of walls of cerebral blood vessels. Lewy bodies are not mentioned as feature of Alzheimer’s disease. Diffuse Lewy body disease Dementia + Large number of lewy bodies in cortical neurons + ‘No other pathological findings Diffuse Lewy body variant of Alzheimer's | Dementia > Large number of Lewy bodies in cortical neurons + disease (and not in Alzheimer's disease) _| Neurofibrillary tangles and amyloid plaques. itoxication) : Harrison 17/2653 (379-3); 16"/2505, 2509, 2577; 15/2503, 2506, 2594 “Arsenic produces a sensory and motor polyneuritis manifestating as numbness and tingling in a ‘stocking ~ glove’ distribution, distal weakness and quadriplegia” ~ Harrison 15%/ 2594, 2503 Pure motor neuropathy: Disorders affecting any level of ‘the motor unit’ can result in purely motor neuropathy ‘without sensory disturbance. ‘The affections include- + anterior horn cell = motor axon = neuromuscular junction Examples of pure motor neuropathies include: Answer is D (Arseni Lower motor neuron form of amyotrophic lateral sclerosis” 1. 2. Poliomyelitis? 3. Hereditary spinal muscular atrophy 4. Adult variant of Hexosaminidase deficiency 5. Lead intoxication? 6. Dapsone intoxication® 7. Acute intermittent porphyria (occasionally) 8. Neuromuscular junction disorder : Lambert Eaton Myaesthenic syndrome? Tick bite paralysis? Some motor sensory neuropathies have predominant motor symptoms and si ser nent is subclinical (readily demonstrable electrodiagnosticall 1. Hereditary motor sensory neuropathy 2. GBS or Acute inflammatory demyelinating polyneuropathy? 3._ CIPD?174. 175. 176. 177. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 = 891 Answer is A (Herpes simplex virus type 2) : Harrison 16/2477 (360-4), 2480; 15! /2475 HSV pe 2 is a common cause of aseptic meningitis, however encephalitis following HSV type 2 has not been ‘mentioned. Viruses causing encephalitis: aa A SRT PE EE 1. Atboviruses + CMY ‘Adenovirus (Gapanese—B encephalitis + EBV + Influenza Virus isan arbo virus) + HIV «+ Parainfluenza 2. Enterovirus + Measles + Rabies 3. HSV-1 + Varicella Zoster + Rubella 4. Mumps + CTFV (Colarado tick fever virus) + LCMV: Lymphocytic choriomeningitis virus. ‘* Japanese encephalitis virus is an atbovirus and is one of the most common causes of encephalitis outbreaks. ‘* Nipah virus: is a new member of the paramyxo virus family. ‘New cases of viral encephalitis are constantly appearing as evidenced by the recent outbreaks of 300 cases of encephalitis with 40% mortality rate in Malaysia caused by Nipah virus (paramyxovirus family). Answer is B (Herpes simplex encephalitis) : Harrison 16"/2480-81; 15" / 2476 The patient in question is presenting with two groups of clinical manifestations a= sneer ESOS v v Clinical manifestations common to all viral encephalitis Focal findings © Fever ‘© Focal seizures * Altered level of consciousness/ confusional state ‘© Right sided hemiparesis © Headache ‘© Frontoparital hyperintense lesion on MRI According to Harrison 15% /2476: Focal findins in a pateint with encephalitis should always raise the possibility of HSV encephalitis. Examples of focal findings include: 7 | Areas of increased signal intensity in the onto temporal, cingulate or insular region of brain on MRT (72 weighted spinecho MRI) “Temporoparietal areas of low absorption, mass effect and control enhancement [1 Periodic temporal lobe spikes on a background of slow of low amplitude activity on BEG ‘Since the patient in question is presenting with focal findings, HSV encephalitis is the most likely clinical diagnosis MRI fin other options provided in question: Ta “Neurocysticercosis ‘Multiple cystic lesions with enhancement on MRL (Aaa procenic meningitis REC | Carcinoma meningitis ~ Nodular tumor deposits in the meninges or diffuse meningeal enhancement on MRI, Answer is D (Craniopharyngioma) : Gunderson and Tepper, Clinical and radiation Oncology /317 "Certain brain tumors such as medulloblastomas, primitive neurectodermal tumors, anaplastic ependymomas, ‘choroid plexus carcinoma, pineoblastomas germ cell tumors and lymphomas may involve the CSF, leptomeningis of the brain or spinal cord". Thus all tumors except craniopharyngiomas mentioned as options may involve CSF. Answer is D (Lymphadenopathy): Harrison 16/23 Lymphadenopathy Pseudolymphoma’ isa complication associated with use of phenytoin & is not seen with valproate 1. Hepatotoxicity : - asymptomatic rise in serum. 1, Hyperplasia of gums iacianmnintoe KOT 2. Rypagiyounis -Fiomeatiogciiocoy ine: 5 prawn 3 eee = 4. Hypersensitivity reaction amano 7 antoin syndrome is foetus : during pres Bsc mao ore vesting 5. Radmin and , eenney 5. Thrombocytopenia “al 6. Transient alopecia Fes econo) ch 7. Ataxia, tremors and sedation may be seen 8. Lymphadenopathy - (Harrison 15" /2364) 8. Use during pregnancy may cause neural tube defects (Pseudolymphoma) Drugs causing Lymphadenopathy - Harrisons Phenytoin Primidone Neurological symptoms like dizziness, ataxia, incoordination, confusion, diplopia392 178. 179. 180. © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 Answer is B or C (Aci Persistent vomiting results in chloride responsive hypochloremic, hypokalemic metabolic alkalosis with paradoxical aciduria. Thus both hypochloremia and acid urine excretion are correct alternatives. ic urine excretion or Hypochloremia): Read fext below ectrolyte disturbance in vor ‘+ Persistant vomiting produces loss of fluid with high chloride and hydrogen ion concentration, = Hypochloremia in blood = Excess HCOy accumulates (metabolic alkalosis) + Accompanying contraction of ECF volume stimulates maximal reabsorption of sodium to maintain volume. Since there is less chloride in glomerular filtrate for reabsorption of sodium in the proximal tubule, more sodium must be absorbed in the distal tubule, where sodium reabsorption occurs in exchange of potassium (initially) and hydrogen (if K" is depleted) + Developing hypokalemia from continued vomiting and above exchange, subsequently encourages exchange of H’ for Na’, since less K" is available. If hypokalemia is severe enough the initially alkaline urine becomes acidic with excretion of H” ions in urine ~ Paradoxical aciduria Thus = Hypochloremia is a feature in both the initial developmental phase as well as during the compensatory phase following persistant vomiting. + Acidic urine excretion or paradoxical aciduria occurs only after persistant vomiting when potassium depletion becomes severe. ‘Since the question specifically talks about the most likely event following ‘persistant vomiting’ (compensatory hase), ‘acidic urine excretion’ may carry a slightly higher weightage in terms of the likely answer that the examiner desires. Answer is A (It is due to estrogen mediated sodium retention) : Harrisons 16"/214-215; 15" /220 Idiopathic edema should be distinguished from premenstrual or cyclical edema in which the sodium retention and water retention may be secondary to excessive estrogen stimulation - Harrison “Tiopathic edema’ is a syndrome exclusive to women, characterized by periodic episodes of edema unrelated to menstrual] ‘egele and frequently accompanied by adbominal distension. Etiology : Although exact etiology is not known the suggested causes include = increase in capillary permeability that fluctuates inseverty and may be aggrevated by hot weather = secondary activation of RAA (Renin angiotensin system) = improved supression of AVP release = ‘Diuretic induced in some cases = Decreased dopaminergic activity and urinary Kallikrein and kinin excretion have been reported in this condition which ‘may be of pathogenic importance Idiopathic edema should be distinguished from premenstrual or cyclical edema in which the sodium retention and water retention may be secondary to excessive estrogen stimulation - Harrison Presentation : + Periodic episodes of edema ‘© Diurinal alteration in weight with orthostatic retention of sodium and water. Patient weighs several pounds more after having been in upright posture for several hours. + ‘Abdominal distension’ ‘Treatment : supportive Reduction in salt intake ACE inhibitors Rest in supine positon for several hours Progesterone Elastic stockings Dextroamphetamine (Sympathomsmetic amine) Understanding underlying emotional problem Diuretics: helpful initially and should be used sparingly. larvison's 16/1863; 15/1759 ‘When cirrhosis is complicated by portal hypertension the increased resistance is usually sinusoidal’ - Harrison's 15°/1759 Portal hypertension (> 100 mm Hg: Normal portal pressure = 5 to 10 mmHg) most commonly results from increased resistance to portal blood flow.181. 182, 183. Increased resistance can occur at three levels 1, Anatomically outside the Cirrhosis 1. Outside iver, at level of hepatic veins liver eg. Portal vein When cirrhosis is complicated ~ Budd chiarri syndrome thrombosis by portal hypertension, the 2. Inthe inferior vana cava 2. Within the liver but increased resistance is usually + Thrombosis proximal to sinusoids eg. sinusoidal 3. Inthe liver but distal to sinusoid Schistosomiasis = Venoocclusive disease ‘© Cirrhosis is the most common cause of portal hypertension in the United States. ‘© Portal vein obstruction is the second most common cause. Answer is C (hepatie vein): Harrison's 16/1863, 15/1759: See previous question Budd chiari syndrome is characterized by post sinusoidal obstruction outside the liver atthe level of hepatic veins ~ Harrison's 15"/1759 Answer is B (Hypogastrinemia): Harrison's 17/1882, 1883; 16/1773; 15"/1676 Short bowel syndrome is characterized by increased gastrin levels (hypergastrinemia) due to reduced small intestinal catabolism of gastrin — Harrison's Short bowel syndrome (Myriad of clinical problems that follow resection of warying length of small intestine) Causes: ‘Mesentric vascular disease: mesenteric ischaemia® 2. Primary mucosal and submucosal disease — Crohn's disease® 3. Operations without preexisting small. po disease — jejunoilial bypass for obesity® jfestation resulting from resection of th of small intestine ‘Multiple factors contribute to diarhea and There occurs increased Due sseatorhea such as: decrease Related to reduced absorption of oxalate by large imthebile acid hormonal inhibition of intestine inpatients with a small pool sizeand _avi secretion or increased ‘+ Absence of intestinal mucosa required for intestinal revestion with inact fesuling super gastin levels due to. pfeorrion of pid and Dak ond ‘colon saturation of | reduced small intestine srolytes ypuvalria andrea cae SESS all- catabolism of asin + Stimulation of colonic uid and ue ium blader bile. | electrolyte secretion by increased bile a cid that ener colon ‘Treatment: ‘+ Decreased intestinal transit nd bactorial © Dietry restriction of oxalate overgrowth from absence of lio caseal containing feed (spinach, tea) ee, + Cholesyramine an anion binding '* Reduced pH in duodenum from gastric resin and caleum pemcaetetn Answer is B (Prolactin): Harrison 17"/ 2318; 16/2193; 15"/ 2170, 2171 Gynaecomastia may result from deficienet testosterone or increased estrogen. ‘Prolactin’ causes synthesis of milk in the female breast, and its excess results in galactorrhea. It also opposes the action of pituitary gonadotrophins on the gonads and may thus result in hypogonadism and amenorrhea. Gynoaccomastia however does not result from either excess or deficiency of prolactin. Endocrine work up for Gynecomastia includ: 1. Measurement of serum androstenedione or 24hr urinary 17 ketosteroids (sualy elevated in feminizng adrenal states) ‘Measurement of plasma estradiol and heG. 3. Mesurement of plasma LH and testesterone Testicular fuilure Ted primary estrogen production eg. sertol ell tumors Androgen resistant state oF j (provided hypogonadotrophic hypogonadism has been gonadotrophin seeting tumor. excluded)‘894 AIPGMEEXAMINATION ANSWERS AND EXPLANATIONS - 2004 sata SURGERY 184. 185, 186. Answer is A (Melanoma, Clarks level IV): Harrison's 15"/555; Chadrasoma Taylor 3/895 Mole on the foot with atypia of melanocytes and diffuse infiltration of anaplastic cells into the papillary and reticular dermis is diagnostic of clark level IV of malignant melanoma, ‘lark val [—-— | - Restice o epiermie ans (toon 1s tevang papitary ‘eens oat lng k ‘treachingrescuar ‘Breslow’ depth of avasion ‘— it + Fiogpapitary doris Fig: Two common methods of estimating the prognosis of malignant melanoma based on the degree of vertical invasion, Answer is C (Marjolin's ulcer): Bailey 24/978 Clinical appearance of lymphoedema is variable and depends on severity and duration of syndrom Initially ‘Initially swelling is soft and pits on pressure (collection of protein rich fluid) ‘* Swelling decreases or may disappear on clevation of Limb Later (During a period of years, protein rich fluid incites an inflammatory reaction and consequently: ‘© Edema becomes woody in texture Skin becomes thickened, hypertrophic and hyperkeratotic Pitting becomes less prominent Limb elevation and compression become increasingly unsuccessful at reducing extremity volume. Patients with lymphaedema have increased susceptibility to bacterial infections believed to be due to loss of local immune defences as a result of diminished lymphatic function. ‘Approximately half of patient with lymphaedema have recurrent spontaneous attacks of bacterial cellulites. ‘These attacks are characterized by increased swelling due to local inflammation, pain and high fever. Sabiston 16%/1447, 1448, ‘© Lymphangiosarcoma is a rare lesion that may develop in a lymphedematous extremity regardless of the cause of lymphoedema. Morjolin's ulcer: - Refers to development of malignant ulcers over chronic scars most commonly those from ‘burs. Malignancy is usually a squammous cell carcinoma and has not been mentioned as a complication of Iymphoedema, Answer is D (Direct pressure and elevation): Oxford text book of surgery 2/1000 The question clearly asks about the 'first treatment" of rupture of a varicose vein near the ankle which is definitely direct digital pressure and elevation of foot with patient lying down. (Inference from oxford text 24/1000) smorrhage: A complication of varicose v ‘* Varicose veins around the ankle foot and lower leg specially in the elderly are highly vulnerable to haemorrhage which may oceur with minor trauma or a spontaneously when patient is up and about. ‘© The ensuing haemorrhage may be copious but is easily stopped by finger pressure or when patient lies down with foot elevated and a firm pad and bandage is applied’. - ‘Oxford text’ first initial management) ‘© However the procedure plugs the aperture only temporarily with litle tendency to heal as underlying veins remain open and unsupported. ‘© Treatment must be completed by elimination of affected varicosity which may be achieved by compression sclerotherapy.187. 188, [AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 © 895 Answer is B (Bronchial artery): « Grainger & Allison's ~ Diagnostic Radiology 4/e P-609 In 95% of cases of massive recurrent hemoptysis the source of blood are systemic bronchial artery® rather than pulmonary artery; so these are the vascular structures which should be evaluated first. Answer is C (surgery): Harrison's 15/565; Oxford text book of surgery I"/2480, Schwartz 7°/753 The bafiling aspect of the quest cell carcinoma as this histologi is that it does not mention whether it i a small cell carcinoma or a non small distinction is the bases of the primary modality of treatment. Small cell eareinomas For non small cell carcinomas the primary modality depends on the — Small cell lung carcinomas behave as extent of disease systemic disease. ‘© Localized diseases: (Stage 1 & II) © — Most small cell carcinomas have Primary modality of treatment is surgery ~ pulmonary already spread atthe time of resection, presentation beyond the bounds of ‘+ Advanced disease: surgery and they are managed Radiotherapy with curative intent is advocated primarily by chemotherapy with or (& chemotherapy) ‘without radiotherapy. ‘+ Metastatic disease (Stage IV) symptomatie/supportive Ifwe were to go by the inform: ‘© The maximum tumor size (T) is ‘+ There is no nodal involvement (No) ‘© There is no distal metastases (Mo) avail em (2 3), ie. Ta ‘Thus it is T; Np My disease or stage I disease The treatment of choice for stage I disease is surgery ie. Pulmonary resection ‘© The tumor in question has no nodal involvement and no metastases and hence is likely to be a non small cell carcinoma. Further, according to oxford textbook of surgery 2™/2480 "Recent data suggest that staging small cell lung cancer using TNM system employed for non small cell carcinomas may be helpful. For patients with stage I disease surgical resection and postoperative adjuvant chemotherapy provides a better prognoses than non surgical therapy". Thus within the limits of information provided the primary modality of treatment here should be surgery. Stage “TNM Deseriptors nical Stage Surgical Pathologie Stage ma TINOMO “i @ an B TNOMO 8 3 aw TINIMO M 55 a 13 PNINO x » a TNOMO 22 38 = IA TSNLMO o 35 Th23NMO B B T4N0-12MO z 3 E TI23-4N3MO 3 3 Ww ‘any Tany NM 1 < [Eamor (1) sats deseriptor 10 ‘No eidens of primary unr Primary tur amor be assed, omar ren bythe presse of malignant cls in pum or rons vashings but not vai by imaging or ronchoscony TIS Caring ‘Tumor > 3 em in greatest dimension, surrounded by lung or visceral pleura, without bronchoscopic evidence of invasion more proximal than ler bronchus (Le. not in main bronchus) “Tumor with any of following: > 3 em in greatest dimension: iolves main broachus, >= 2 cm distal to the carina: invades visceral pleura ssocated with atelectasis or obstructive pneumonitis extending to hilum but does not involve entire lung. ‘Tumor of any size that diretly invades any ofthe following: chest wall (nchudng superior sulcus tumors), iaphragm, mediastinal pleura, parietal pericardium; or tumor in main bronchus <2 cm distal to carina but without involvement of carne, or associated atelectasis or obstructive pneumoaitis of entire lng, “Tumor of any size that invades any ofthe following: mediastinum, hea, great vesels, trachea, esophagus, vertebral body, carina or tumor with malignant pleural or paercardia effusion, or with satellite tumor 5 ‘nodule(s) within the ipsilateral primary-tumor lobe of the lung.189. 191. SRB Regional Imp nodes canot be asessod [0 Nair ‘Metastasis tipster! peribronchial andor psilatral hilar lymph nodes, and intrapulmonary nodes involved by diret extension of the primary tumor. ae - Metastasis to ipsilateral medintinal, contralateral Iymph node(s) ‘Metastasis to contralateral modiastial, contralateral hil, pst or contralateral salene, or sina & Aymph nodes) (Distant metastasis (M) descriptor ans moet No distant metstasis (UL ot i Answer is A (Neck): Bailey 24"/788 The question talks about the most uswal approach to removal of a retrosternal goiter ‘© ‘Resection can almost always be carried out form the neck and a midline sternotomy is hardly even necessary’ - Bailey 24°/788 The cervical part of goiter is mobilized first and the retrosternal goiter is then delivered by traction which may be facilitated by inserting a series of sutures fingers or traditionally a spoon. * ‘Nearly all retrosternal goiters can be removed via a cervical incision which should be placed higher than the conventional incision." - Oxford texthook of surgery 2/1117 = If preoperative symptoms and investigations indicate that the chest may need to be opened, then the anterior chest wall is draped and prepared accordingly. = Ifretrostemnal thyroid malignancy is suspected or surgery is being performed for a recurrent goiter, formal splitting of sternum is appropriate. To conclude the ‘usual approach’ as asked in the question for retrosternal extension of a cervical goiter is, undoubtedly through ‘the neck’ via a ‘cervical incision’. Answer is A (Medullar Carcinoma Thyroid): Harrison's 15/2187 Out of all the histological variants of carcinoma thyroid itis the medullary variant that is associated with ‘multiple endocrinal neoplastic syndromes and hence is the answer here. (No confusion regarding the answer) What puzzles the situation? is the fact thatthe combination of pancreatitis, pituitary tumor and pheochromocytoma, suggests a diagnosis of MENI syndrome. MEN I has been mentioned to be associated with C cell hyperplasia, and not Medullary carcinoma thyroid. Medullary thyroid carcinomas is a feature of MEN II syndromes. Justification: Medullary carcinoma thyroid arises from parafollicular C cells. C cell hyperplasia thus, may be regarded as a precursor of Medullary carcinoma thyroid Ifpresent in the options, C cell hyperplasia would have been a more correct answer, however in its absence, Medullary thyroid carcinoma is certainly the option of choice MEN SYNDROMES (Broad Perspective) Q _MEN Ti ‘Characterized by tumors of Characterized by association of 1. Parathyroid = Medullary carcinoma thyroid & 2. Pituitary = Pheochromocytoma 3. Pancreatic islet cells = Zollinger Ellison syndrome (gastrin) WA UB = Insulinoma (Insulin) eer When associated with | When associated with veel tency diantnea | /Per-parathyroidism — | mucosal neuromas and ‘marfinoid habitus syndrome (pancreatic cholera) Answer is D (MEN IIB): Harrison's 15%/2185 The association of medullary carcinoma thyroid & pheochromocytoma, with mucosal neuromas and ‘marfinoid habitus is designated 'MEN IIB" — Harrison's 15%/2187.192. 193. 194, 195. [AIPGME EXAMINATION ANSWERS AND EXPLANATIONS -2004 = 897. © Mucosal neuromas and marfinoid habitus are the most distinctive features of MEN IB and are recognized in childhood. ~ Harrison's © True Neuromas are present on the tip of tongue, under the eyelids and throughout the gastrointestinal tract ‘and are tree neuromas distinct from neurofibromas. Parathyroid hyperplasia or denoma MIC MTC Islet cell hyperplasia, adenoma, or carcinoma Pheochromocytoma Pheochromocytoma Pituitary hyperplasia or adenoma Parathyroid hyperplasia or Mucosal and gastrointestinal Other ess common manifestations; adenoma neuromas foregut carcinoid, pheochromocytoma, ‘Cutaneous lichen “Marfanoid features subcutaneous or visceral lpomas, dermal amyloidosis angiofibromas or collagenomas Hischsprung disease MTC Answer is C (Papillary): Repeat: Robbins 5/1136 ‘The morphological variants of thyroid carcinoma and their approximate frequencies are: Papillary carcinoma 75-85% Follicular carcinoma 10-20% Medullary carcinoma 5% Anaplastic carcinoma Rare The question has been dealt in greater detail earlier and hence has not been elaborated here. Answer is C (Alveobuceal complex): 451/348 According to textbook of surgery by ‘Association of surgeons of India’, various cancers of oral cavity are reported with the following frequency: Buccal mucosa —«=—=38 percent Anterior tongue 3 ‘16 percent Loweralveolus —-__15,7 peroent The alveobuccal complex thus accounts for 53.7 percent of oral cancers and thus constitutes the commonest site in the Indian population, ‘ote Below According to Ackerman's textbook on surgical pathology 8/234 the incidence of oral cancers is as follows: Lip B% Tongue 16% Floor of mouth Lower Gingiva Upper Gingiva Alveobuecal Complex Hard palate 05% Thus, ifone chooses o go by foreign incidence, the answer is definitely 'Lip' however if we rely on the ‘associations of surgeons of India’, buccoalveolar complex is the answer. I have personally chosen the latter because the question specifically asks about the incidence in the Indian population. Answer is A (oropharyngeal carcinoma): Park 17"/290; Bailey 24°/7.2 ‘In Global terms, oral/oropharyngeal carcinoma is the sixth most common malignancy. In western world it accounts for only 2-4% of all malignant tumors. In contrast, in Asia oraVoropharyngeal carcinoma is the most ‘malignant tumor, accounting in parts of India, for no less than 40% of all malignancy.- Bailey ‘Oral cancer is a major problem in India and accounts for $0 t0 70% of all cancers diagnosed! - Park Answer is B (Ileum): Harrison's 15/1783: Repeat ‘Mechanical intestinal obstruction resulting from the passage of large gall stones into the bowel lumen is called ‘Gall stone ileus’ The most common site of obstruction is the ileocoecal valve (provided that the more proximal level of small intestine is normal in calibre) - Harrison898 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2004 196. Answer is B (Gall stone pancreatitis): Oxford textbooks of surgery 2/1766 ‘The prognosis is best in patient where pancreatitis is caused by a remediable cause such as cholelithiasis. ‘Eradication of gall stone disease’ prevents further attacks of pancreatitis. -Oxford tex. The etiological mechanism probably involves the transient obstruction of pancreatic duct by a gall stone in the common bile duct at the Ampulla of Vater. Good prognosis is also evidenced by the fact that gall stones are recoverable in the faeces of over 90% of patients within 10 days of an attack, suggesting that the obstruction is brief and that most such stones pass into the duodenum. Bailey 24°/1258 Injection sclerotherapy is ideal for first degree internal haemorrhoids which bleed. Early second degree internal haemorrhoids may also be treated but a proportion relapses ~ Bailey 197. Answer is B (Internal haemorrhoi Haemorrhoids or piles refer to dilated veins occurring in relation to the anus. ‘They may be classified as ‘© Internal haemorrhoids: Arise internal to the anal orifice and therefore are covered by anal mucous membrane ‘+ Extemal haemorthoid: Arise extemal to the anal orifice and therefore are covered by skin. ble for t iment of internal haemor ‘Sclerosant is introduced Tight elastic bands are Infrared irradiation is insubmucosa which slipped on the base of applied to mucosal produces inflammatory pedicle of each surface of a pile with eactionand ether _haemomthoid witha special the help of a probe thromboses vessels or instrument. Bands cause I compresses them by ischami nerses ofthe kee fibrosis pile which sloughs off ee within af days operative methods ations: Indications: Indications: « Fibrosed « First degree Second degree First and second degree haemorthoids hhaemorrhoids hacmorthoids which ae too Haemorthoids « Interoexiernal # May be tied ia larg for injetion treatment ear second degree piles ‘external is well also defined Remember: Three degree of internal haemorrhoids: First degree Bleed only No prolapse Second degree Prolapose but will reduce spontaneously or can be reduced digitally and will remain reduced ‘Third degree Continuously remain prolapsed 198. Answer is B (Abdominoperenial resection): Subiston 16/966; ASI/S2' Bailey 24/1235 A ‘rectal carcinoma’ located at 5 cm from the anal verge lies in the lower rectum. ‘Considering the conventional methods of treatments * Anterior resection is possible for tumors of upper 2/3" of rectum © Abdominoperenial resection is indicated for tumors of lower 1/3" of rectum General site specific plan (ASI/521) Anterior Resection Low Anterior resection Abdominoperenial resection Note: Length of rectum is about 12 10 15 cm and the anal canal is about 4 em any tumor 5 cm from anal verge would thus lie at lower rectum.199, 200. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS -2004 @. General considerations for tumors in lower Rectum: The Abdominoperenial resection (APR) is the gold standard for the treatment of distal rectal cancer. ~Sabiston 16°/966 ‘+ ‘APR’ involves complete excision of rectum and anus, by concommitant dissection through abdomen and perenium, with permanent closure of perenial rafe and creation of an end colostomy. The most significant disadvantage of this procedure is that it sacrifices the sphincter mechanism and hence now focus is shifting towards a number of sphincter sparing procedures. Itis important however to note here that the information provided is too limited to comment upon the possibility of performing sphincter saving procedures such as local resection or a posterior proctotomy. ‘+ Young patients who have a favourable body habitus Coloonal anastomosis 1 « Patients with good preoperative sphincter function 2. Local excision * Lack of sphincter involvement by tumor 3. Transanal excision « Limited extension of tumor into rectal wall (T oF Ty lesions) 4 Trans sphincteric excision © Low grade tumors 5. Posterior proctotomy (Kraske procedure) Answer is A (Pedunculated polyp): Harrison's 15/582, Schwartz 7/1342 Adenomatous polyps may be sessile or pedunculated. Cancers develop more frequently in sessile polyps. A'polyp' is a grossly visible protrusion from the mucosal surface and may be classified pathologically as. 11. Hamortoma ‘(Nonneoplastic? eg. Juvenile polyp®) |2. Hyperplastic polyp (hyperplastic mucosal proliferation, non neoplastic®) |3. Adenomatous polyp: (clearly premalignant?) The probability of an adenomatous polyp becoming a cancer depends on A. Gross appearance? B. Histological features® C. Size® 1, Gross appearance Cancer develop more frequently in sessile polyps (Pedunculated vs sessile) Histological features Villous or papillary adenomas (most are sessile) become malignant (tubular vs villous (or papillary) more than three times as often as tubular adenomas (Atypia isa risk factor for cancer, wherever it appears) 3, Size <15em Negligible (< 2%) 1.5 1025em Intermediate (210 10%) > 250m Substantial (10%) Answer is C (Intravesical BCG): Oxford textbook of surgery 2/2137, Harrison's 15"/605; Schwartz 7"/1793 he jrovides the following informat ‘+ The patient has a transitional cell carcinoma of urinary bladder with no invasion into the detrusor muscle ~ Superficial bladder cancer. He thus represents a superficial bladder cancer with either stage T, or T; disease. a. The tumor does not invade muscle and does not cross the lamina T, disease (35%) propria of bladder mucosa ’. The tumor extends beyond the lamina propria of mucosa into T; disease (30%) subepethelial tissue but does not infiltrate detrusor muscle ‘+ The patient has a poorly differentiated tumor (Grade III disease) ¢ The lesion is solitary