POSITIVEMEDPG JIPMER NOVEMBER 2017 – EVIDENCE BASED REVIEW [An AID to  JIPMER NOVEMBER  2018 Aspirants] Type of Questions Numbers [approx] Recent Drugs [2017] 3 Genes / Chromosomes 10 Repeats [Recent&Remote;] 45-50 “HIGH END”Questions[Harrison based] 30-40 HIGH YIELD Topics – Hematology, Genetics, Infections 40 WISDOM CAPSULE  [for JIPMER November 2018 candidates] Remote Repeats are also important [in fact more important than Recent Repeats******] RECENT FDA approved drugs,to be known [HARRISON 20th NEW DRUGS-MASTER CHART can cover Most of the  QUESTIONS of recent drugs in  JIPMER November 2018 Exam]High yield Zones-Hematology,Genetics,Infections “HIGH END” questions predominatly HARRISON-20th  based Formulas of common physiological paramaters EVIDENCE BASED REVIEW OF JIPMER NOVEMBER 2017- EXAM REPEATS RECENT REPEATS [LAST 3 YEARS - arbitarily 2014-2017 ] [The choices might not be the same in the exam] 1. Marrow failure with pancreatic insufficiency and malabsorption is seen in which of the following  [JIPMER NOVEMBER 2017] [JIPMER MAY 2017] a) Dyskeratosis congenita B) Shwachman-Diamond syndrome c) Diamond blackfan Syndrome d) Fanconis syndrome Ans B 2. MYD 88 is associated with which of the following tumor [JIPMER NOVEMBER 2017] [Rpt-MAY 2017] a. Waldenstrom’s macroglobulinemia b. Hodgkins lymphoma c. Hairy cell leukemia d. Polycythemia vera Ans A 3. Which of the following antidiabetic drug can be given safely in a renal failure patient [JIPMER NOVEMBER 2017] [Rpt-2016] a)Linagliptin b)Sitagliptin c)Canagaflozasin d)Metformin Ans A 4. Which of the following disease is due to Peroxisomes defect [JIPMER NOVEMBER 2017] [Rpt-MAY 2017] a. Zellweger syndrome  b)Jamaican vomiting sickness c) Reyes sundrome d) CJD Ans A 5. Half life of Iodine 125 is  [JIPMER NOVEMBER 2017] a)13 hrs b) 8 days c) 60 Ans C 6. Cordocentesis for prenatal diagnosis  is done at which week of pregnancy [JIPMER-NOVEMBER 2017] [JIPMER- 2015] a) 10-12weeks b) 12-14 weeks c) 14-16 weeks d) 18-20 weeks Ans D 7. In Duplication of ureter ,the  upper ureter opens in the bladder [JIPMER NOVEMBER 2017] [JIPMER NOV 2016 rpt] a. Caudal and medial to lower  b. Lateral and lower to lower ureter c.  Caudal and  lateral d. medial ANS B 8. In case of double ureter the where doss the upper ureter join bladder [JIPMER NOV 2016] a. Lateral and lower to lower ureter  b. Medial and lower to lower ureter  c. Lateral and higher  d. Medial and above 9. Vascularisation of cornea seen in dificiency of [JIPMER NOVEMBER 2017] [AIIMS MAY 2014Rpt] a. B2 b. B3 c. B1 Ans A Circumcorneal vascularization is observed in deficiency of: [AIIMS May 2014] a. Vitamin D b. Thiamine c. Riboflavin d. Biotin Ans B REMOTE REPEATS [BEFORE 2014-arbitarily] 1. Schwartz formula for creatinine clearance calculation includes all of the following parameter except  [JIPMER NOVEMBER 2017] [AIIMS 2006 Rpt] a) Height b) Serum Creatinine c)age d) In SCHWARTZ formula for calculation of creatinine clearance in a child, the constant depends on the following except, [AIIMS NOV 2006] a) Age b) Method of estimation of creatinine c) Mass d) Severity of renal failure Ans (d) 2. Which of the following radiation is emitted by Phosphorus 32 [JIPMER NOVEMBER 2017] [AI-2006 Rpt] a) alfa rays b) Beta rays c) Gamma rays d) Neutrons Ans B Phosphorous – 32 emits: [AI-2006] a) Beta particles b) Alfa particles c) Neutrons d) X – rays Ans A 3. Microsatellite dna is [JIPMER NOVEMBER 2017][ AI-2006Rpt] a. 2-6 nucleotides repeats  b. 6-10 nucleotide repeats c. 10-14 nucleotide repeats Ans A Microsatellite sequence is: [AI-2006] a) Small satellite    b) Extra chromosomal DNA c) Short sequence (2-5) repeat DNA d) Looped – DNA Ans C 4. A HIV patient was admitted for evaluation of chronic diarrhea.His stool examination revealed  a cyst of 8-10 micron in size.Which of the following could be the diagnosis [JIPMER NOVEMBER 2017] [AIIMS May-2009 Rpt ] a) Cryptosporidia b) Isosporiaisis c) Cyclospora d) Giardia Ans C 25 years old male presented with diarrhea for 6 months. On examination the causative agent was found to be acid fast with 12 micro meter diameter. The most likely agent is –[AIIMS MAY 2009] a) Cryptosporidium     b) Isospora c) Cyclospora              d) Giardia Ans (C) 5. Tubes cast is applied for which bone [JIPMER NOVEMBER 2017Rpt] [AI 2007] a. patella b. Capitate c. Calcaneum d. Talus Ans A Tube cast applied in # around [AI 2007] a) Shoulder                  b) Hip c) Pelvis                       d) Knee Ans (d) 6. Lipoprotein lipase activated by which lipoprotein [JIPMER NOVEMBER 2017] [AI 2008 rpt] a. Apo c II b. Apo CI c. Apo Ai d. Apo B100 Ans-A TRUE about apoprotein-C is [AI-2008] a) b) Apoprotein C-I activates Lipoprotein lipase c) Apoprotein C-II activates Lipoprotein lipase d) Apoprotein C-III activates Lipoprotein lipase Ans (C) 7. SIRS includes all except: [JIPMER NOVEMBER 2017] [AI 2009 rpt] a. Leukocytosis            b. Thrombocytopenia  c. Hypothermia  d. Oral temperature more than 38°C Ans: b SIRS includes all except: [AI 2009] a. Leukocytosis            b. Thrombocytopenia  c. Hypothermia  d. Oral temperature more than 38°C Ans: b 8. Lubiprostone  is a [JIPMER NOVEMBER 2017][ALL INDIA-2012 Rpt] a. Chloride channel activator  b. Chloride channel inhibitor c. Sodium channel activator d. Sodium channel inhibitor Ans A Irritable Bowel Syndrome is an important disease of the modern world.  Drug for the treatment of IBS, Constipating type is: [AI-2012] a) Lubiprostone b) Ivabradine c) Cholestyramine d) Alosetron Ans (a) 9. Lisfranc amputation is done for [JIPMER NOVEMBER 2017] [AI-2011 Rpt] a) Tarsometatarsal amputation b) Midtarsal  amputation c) Ankle amputation d) Below knee amputation Ans A Lisfranc amputation is done for [AI-2011] a) Tarsometatarsal amputation b) Midtarsal  amputation c) Ankle amputation d) Below knee amputation Ans A 10. ANCA-Antineutrophilic cytoplasmic antibody is not associated with  [JIPMER NOVEMBER 2017] [AIIMS NOV 2000Rpt] a. Wegeners granulomatosis b. Chrug strauss syndrome c. Microscopic polyangitis d. PAN Ans D ANCA is NOT associated with which of the following diseases: [AIIMS NOV 2000] a) Wegener’s granulomatosis b) Henoch schonlein purpura c) Microscopic PAN d) Chrug Strauss syndrome Ans (b) 11. Which of the following is the abductor of vocal cord [JIPMER 2017][JIPMER 2013Rpt] a. Cricothyroid b. Posterior cricoartyenoid c. Interarytenoid d. Lateral Crico arytenoids Ans b Abductor of vocal cord is [JIPMER 2013] a. Posterior cricoarytenoid b. Cricothroid c. Interarytenoid d. Lateral cricoarytenoid Ans A 12. Osteoclast marker all except [JIPMER NOVEMBER 2017] (AIIMS MAY 2008 Rpt) a. Ntelopeptide   b. C-telopeptide   c. propeptide of type I procollagen  d. Acid phosphatase  Ans C Bone resorption markers are except (AIIMS MAY 2008) a) Tartarate resistant alk. Phosphatase (TRAP) b) Osteocalcin c) Cross linked-N-telopeptides d) Urine total free deoxypyridinoline Ans (b) 13. Trans-Juglar intrahepatic portosystemic shunt first complication expected is post-operative period  [JIPMER NOVEMBER 2017] [2006] a. hepatic encephalopathy  b. bleeding c. Jaundice d. Diarrhoea Ans A 14. Which of the following disease is due to defect in alfa oxidation [JIPMER NOVEMBER 2017] [AIIMS NOV 2008 Rpt] a. Refsum disease b.  Zellweger disease c. Reyes syndrome d.all Ans A Refsum’s disease is due to deficiency of which of the following enzyme? [AIIMS NOV 2008] a) Malonate dehydrogenase        b) Thiophorase c) Succinate thiokinase d) Phytanic alpha oxidase Ans (D) 15. Which of the following is not a sign of malignant gastric ulcer on radiographic studies? [JIPMER NOVEMBER 2017][AIIMS DEC 1998] a. Carmen sign b. Hampton's line c. Nodular gastric ulcer mound d. Abrupt transition between normal and abnormal mucosa several cms away from the ulcer crater. Ans B Malignant gastric ulcers are characterized by A/E: [AIIMS DEC 1998] a) ulcer extents beyound the gastric wall b) Mucosal rugae stop far of ulcers c) Eccentric crater d) Margins are raised Ans (a) 16. Youngs operation is done for  [JIPMER NOVEMBER 2017] A) Atrophic rhinitis b) Rhinophyma c) Hypertrophic Rhinitis d) Rhinitis sicca Ans A 17. Kohlers disease is due to osteonecrosis of [JIPMER NOVEMBER 2017] a) Navicular bone b) Talus c) Cuboid d) Calcaneum Ans A 18. Which of the following has a good prognosis in ALL [JIPMER NOVEMBER 2017] a) Hyperploidy b) t(4.14) c) t(8.14) d) t(9,22) Ans A 19. Which of the following is Not derived from mesoderm [JIPMER NOVEMBER 2017] a. Suprarenal medulla b. Suprarenal Cortex c. cardiac musculature d. skeletal musculature  Ans A 20. Intelligent quotient-IQ is measured by which of the following formula [JIPMER NOVEMBER 2017] a. (Mental age/chronological age)/100  b. (Chronological age/Mental age)/100  c. (Mental age/chronological age)/10  d. (Chronological age/Mental age)/10 Ans A 21. Which of the following paramater is not seen in  Restrictive lung disease [JIPMER NOVEMBER 2017] a. FRC increased   b. FEV1 decreased  c. FVC decreased  d. Ans B 22. Which of the following segment in preserved in Lumbar sympathectomy [JIPMER NOVEMBER 2017] a. L1 b. L2 c. L3 d. L4 Ans A 23. The human major histocompatibility complex (MHC), commonly called the human leukocyte antigen (HLA) complex occupies [JIPMER NOVEMBER 2017] a) short arm of chromosme 6 b) long arm of chromosme 6 c) short arm of chromosome 4 d) long arm of chromosome 4 Ans A 24. Sabin Fieldman test is used in the diagnosis for  which of the following infection [JIPMER NOVEMBER 2017] a) Toxoplasmosis b) Cysticercosis c) Leishmaniasis d) Typanosomiasis Ans A 25. Winter bottom sign is seen in which of the following infection [JIPMER NOVEMBER 2017] a) Leishmaniatropica b) Leishmaniadonovani (kala-azar) c) Leishmaniabrasiliensis (espundia) d) Trypanosomagambiense (Gambian sleeping sickness) Ans D MODIFIED REPEATS 1. 1st trimester most reliable criteria for gestational age estimation [JIPMER NOVEMBER 2017] [AIIMS NOV 2002 Rpt] a. CRL   b. femur length   c. BPD  d. Weight Ans A Best parameter for estimation of fetal age by ultrasound in third trimester is: [AIIMS NOV 2002] a) Femur length b) biparietal diameter c) Abdominal circumference d) Inter-ocular distance Ans (a) 2. Which of the following statement regarding FAP is   a False statement [JIPMER NOVEMBER 2017] a. autosomal recessive   b. 100 polyps in colon   c. duodenal polyps  d. extraintestinal manifestation are seen in all  Ans ?D Which statement is false regarding familial adenomatous polyposis: [AIIMS JUNE 1999] a) Males are usually carriers b) Autosomal dominant inheritance c) If not treated progress to malignancy in 100% of cases d) Males and females are affected equally Ans (a) 3. Loss of Y chromosome is associated with which of the following type of renal cell carcinoma [JIPMER NOVEMBER 2017] [AI 2010 RPT] a) Clear cell b) Chromophobe c) papillary d) Duct of bellini Ans C Chromophobe variant of Renal cell carcinoma is associated with (AI 2010) A. VHL gene mutations B. Trisomyof7 and 17 (+7, +17) C. 3 p deletions (3p-) D. Monosomy of 1 Ans (d) 4. Mutation  in 22q11 is associated with which of the following [JIPMER NOVEMBER 2017] a. hypercalcemia b. conotruncal anamolies c. thymic hyperplasia  d. Ans B 5. Time dependent pharmacokinetics is seen in which of the following drug [JIPMER NOVEMBER 2017] [AIIMS May 2014 Rpt] a) Amikacin b) Ciprofloxacin c) Azithromycin d) Pencillin Ans D Time dependent killing and prolonged post-antibiotics effect is seen with: [AIIMS May 2014] a. Fluoroquinolones b. Beta lactam antibiotics c. Clindamycin d. Erythromycin Ans  (C) 6. Alpha helix is formed by which of the following amino acid [JIPMER NOVEMBER 2017] [JIPMER 2011rpt] a. methionine b. glycine c. Lysine d. Histidine Alpha helix formation is inhibited by [JIPMER 2011] a. Glutamate b. Valine c. Tryptophan d. Proline Ans D An amino acid which does not participate in α helix formation is [AI 1996] a)  Leucine                   b)  Glycine c)  Proline                     d)  Lysine Ans (c) GENES/CHROMOSOMES [Better to know your GENES well, for  JIPMER NOVEMBER 2018 examinations!] 1) Early onset Alzheimer is associated with which of the following chromosome [ JIPMER NOVEMBER 2017] a. Chromosome 1 b. Chromosome 14 c. Chromosome 10 d. Chromosome 21 Ans b Gene Chromosome * APP gene 21 * Presenilin 1 14***** * Presenilin 2 1 * APO ‘Episilon’ 19 * a2 macroglobulin 10 * The POLES Team was pleased to have provided the above tabular column in POLES 2017 ,Which now looks Tailor Made  for this question**** 2) Retinoblastoma gene is true except [JIPMER NOVEMBER 2017] a. Autosomal dominant inheritance (seen in familial retinoblastoma)   b. needs both copies mutation – two hit   c. Chr 13p14 d. tumor suppressor gene  ANS C 3) MYD 88 is associated with which of the following tumor [JIPMER NOVEMBER 2017][Rpt-MAY 2017] a. Waldenstrom’s macroglobulinemia b. Hodgkins lymphoma c. Hairy cell leukemia d. Polycythemia vera Ans A 4) The human major histocompatibility complex (MHC), commonly called the human leukocyte antigen (HLA) complex occupies [JIPMER NOVEMBER 2017] a) short arm of chromosme 6 b) long arm of chromosme 6 c) short arm of chromosome 4 d) long arm of chromosome 4 Ans A 5) The chromosome associated with alfa 1 antitrypsin deficiency is [JIPMER NOVEMBER 2017] a) 10 b) 12 c) 14 d) 21 Ans  C 6) Mutation  in 22q11 is associated with which of the following [JIPMER NOVEMBER 2017] a. hypercalcemia b. conotruncal anamolies c. thymic hyperplasia  d. Ans B 7) Carcinoma breast is associated with which of the following [JIPMER NOVEMBER 2017] a) Ca 15-3 b) Ca 27-9 b) Ca 19-9 d) Ans A 8) Which of the following gene is associated with endometrial carcinoma [JIPMER NOVEMBER 2017] a) PTEN b) APC c) MLH The most heartening aspect was all the genetic questions were covered methodically in POLES 2017 9) Steven johnson syndrome due  to carbamazepine is seen in Individuals with which of the following HLA [JIPMER NOVEMBER 2017] a) HLA B 1502 b) HLA B 5701 c) HLA B 5708 d) HLA B 1505 Ans A “HIGH END Questions”[TOUGH ONES] [This list might differ from individual to individual; we have tried to provide the consensus] 1) Number of trays required in thanatorium 2)  'Pie in the sky' appearance 1. The recent drug for Acute Myeloid Leukemia [JIPMER NOVEMBER 2O17] a. Midostaurin b. Imatinib c. Ponitunib Ans A 2. In Hereditary Haemorrhagic telangiectasia, ALK  1 is positive in which of the following type  [JIPMER NOVEMBER 2017 ] a. type 1   b. type 2   c. type 3   d. type 4  ANS A 3. PCSK9 inhibitor drug [JIPMER NOVEMBER 2017] A Alirocumab B Linotinib C Abxcicimab D Ans A HARRISON UPDATES-20th EDITION[NOT PRESENT IN 19th edition] PCSK9 inhibitorsEvolocumabAlirocumab****** 4. The lattest drug approved for advanced metastatic Carcinoma breast  [JIPMER NOVEMBER 2017] a) Abemaciclib b) Ixabipilone c) Ribociclib d) Ans A 5. Which of the following is not true regarding Plasmodium Knowlesi [JIPMER NOVEMBER 2017] a) also called monkey malaria b) common in africa c) d) Ans B 6. Early onset Alzheimer is associated with which of the following chromosome [JIPMER NOVEMBER 2017] a. Chromosome 1 b. Chromosome 14 c. Chromosome 10 d. Chromosome 21 Ans B 7. Severe noise induced hearing is seen in exposure of which of the following? [JIPMER NOVEMBER 2017] [options are elusive] a. Car engine idle at 10 feet away  b. Power mower  c. Niagra falls   d. noisy night street  Ans ?D 8. Which of the following statement regarding Severe Aplastic anemia is false [JIPMER NOVEMBER 2017] a) Neuutrophil count less than 1500 b) platelet count less than 1Lakh c) Corrected Retic count less than 1% d) Absolute retic count less than 60000 Ans A 9. All of the following are true regarding prone position ventilation except  [JIPMER NOVEMBER 2017] a) b) c) d) 10. Klippel-Trenaunay syndrome is associated with all of the following  except [JIPMER NOVEMBER 2017 a. gigantism  b. c. d. Ans A Questions BASED ON HARRISON  EMBRYOGENESIS of JIPMER NOVEMBER 2017 Exam “HIGH END”questions  in reference to HARRISON                                                              [Choices might not be the same in the exam] 1) Which of the following  is false regarding Leflunomide [JIPMER NOVEMBER 2017]  a) Is used in treatment of Rheumatoid arthritis b) acts by inhibiting IMPDH enzyme c) inhibits  proliferation of T and B cells d) it is contraindicated in pregnancy Ans B Lead from HARRISON * Teriflunomide inhibits the mitochondrial enzyme dihydro-orotate dehydrogenase******, which is a key part of the pathway for de novo pyrimidine biosynthesis from carbamoyl phosphate and aspartate. *It is the active metabolite of the drug leflunomide (FDA-approved for rheumatoid arthritis), 2) Severe noise induced hearing is seen in exposure of which of the following? [JIPMER NOVEMBER 2017] [options are elusive] a. Car engine idle at 10 feet away  b. Power mower  c. Niagra falls   d. noisy night street  Ans ?D Lead from HARRISON Decibel (Loudness) Level of Common Environmental Noise Source Decibel (dB) Weakest sound heard 0 Whisper 30 Normal conversation 55-65 City traffic inside car 85****** OSHA monitoring requirement begins 90******* Jackhammer 95 Subway train at 200 ft 95 Power mower 107***** Power saw 110 Painful sound 125 Jet engine at 100 feet 140 12-gauge shotgun blast 165 Loudest sound that can occur 194**** 3) The recent drug for Acute Myeloid Leukemia [JIPMER NOVEMBER 2O17] a. Midostaurin b. Imatinib c. Ponitunib d. Ans A Lead from HARRISON Selected Agents Under Study for the Treatment of Acute Myeloid Leukemia Class of Drugs                                      Examples of Agents in Class Inhibitors of Mutant proteins Tyrosine kinase inhibitors IDH2 mutation inhibitor Dasatinib, midostaurin*****, quizartinib, sorafenib AG-221 Aminopeptide inhibitors HSP-90 antagonists Nedd8 activating enzyme (NAE) inhibitors Tosedostat******* 17-Allylaminogeldanamycin (17-AAG), DMAG, or derivatives MLN4924 4) Which of the following is not true regarding Plasmodium Knowlesi [JIPMER NOVEMBER 2017] a) also called monkey malaria b) common in africa c) Young ring forms resemble those of P. falciparum d) Ans B Lead from HARRISON Plasmodim  knowlesi-Young ring forms resemble those of P. falciparum, while older trophozoites resemble those of  P. malariae. Reliable identification requires molecular genotyping. 5) The latest drug approved for advanced metastatic Carcinoma breast [JIPMER NOVEMBER 2017] a) Abemaciclib b) Ixabipilone c) Ribociclib d) Cetuximab Abemaciclib is a cyclin dependent kinase inhibitor was approved by FDA in September 2017 for Advanced metastatic breast carcinoma 6) MYD 88 is associated with which of the following tumor [JIPMER NOVEMBER 2017][Rpt-MAY 2017] a. Waldenstrom’s macroglobulinemia b. Hodgkins lymphoma c. Hairy cell leukemia d. Polycythemia vera Ans A Lead from HARRISON * Lymphoplasmacytic lymphoma is the tissue manifestation of Waldenstrom’s macroglobulinemia . Many of these tumors harbor a specific mutation, L265P, in MYD88*****, a change that Leads to NF-κB activation 7) Guanylate Cyclase-C Agonist used in the management of   Irritable bowel syndrome is [JIPMER NOVEMBER 2017] a) Linaclotide b) Lubiprostine c) Tegaserod d) all Ans A Lead from HARRISON Guanylate Cyclase-C Agonist * Linaclotide****** is a minimally absorbed 14-amino-acid peptide guanylate cyclase-C (GC-C) agonist that binds to and activates GC-C on the luminal surface of intestinal epithelium. 8) Which of the following is a PCSK9 inhibitor drug [JIPMER NOVEMBER 2017]  a)alirucumab b)Cetuximab c)Abxicimab d) Ans A RELEVANCE OF HARRISON 20th UPDATES for  JIPMER NOVEMBER 2018 [ Questions in JIPMER NOVEMBER 2017 ,for which answers were not present in HARRISON 19th,but Present in HARRISON 20th ] 1) PCSK9 inhibitor drug [JIPMER NOVEMBER 2017] A Alirocumab B certuximab CBotezumab D Tocilizumab ANS A REF-HARRISON 20th-Chapter-400PCSK9 inhibitorsEvolocumab,Alirocumab 2. The lattest drug approved for advanced metastatic Carcinoma breast  [JIPMER NOVEMBER 2017] a) Abemaciclib b) Ixabipilone c) Ribociclib d) Ans A REF-HARRISON 20th chapter-68 Palbociclib,Ribociclib,Abemaciclib 3 The latest FDA Approved drug for Tardive dyskinesia a)Valbenazine b)tetrabenazine c)valproic acid d) ansA Ref-HARRISON 20th Chapter 428 Valbenazine is an ester oftetrabenazine that has recently been approved for the treatment of tardive dyskinesia based on results ofefficacy in double blind trials, but it is associated with sleepiness and QT prolongation. It acts as a vesicularmonoamine transporter type 2 (VMAT-2) inhibitor and blocks storage of dopamine BEST WISHES FOR JIPMER NOVEMBER 2018                                                                                   


Dr.R.S.Manoraajan's Exam Potentials

NIMHANS 2018-GUIDANCE SERIES-  APHASIAS WERNICKE's APHASIA[AIIMS MAY-1998***] *    Here comprehension is  impaired.  *Fluency is preserved. *It is also called as Jargon Aphasia and is associated with Neologisms. * Repetition,   Naming,   reading,   writing is also impaired. *The common cause is and emboli to Inferior Division of MCA. *This involves the wernickes area in the posterior.  1/3 of superoir. Temporal sulcus. (sensory speech area). *Intracerebral hemorrhage,   severe head trauma,   or neoplasm are other causes. Insight is typically  lost* BROCA'S APHASIA[AI-2007***] *    In this condition comprehension is preserved. *Fluency is decreased. *It is called as ‘Bound morpheme’- agrammatism. *Speech is telegraphic  but  informative. *Insight  is preserved*. *The common cause is infarction in Broca’s area and is  due to occlusion of the superior division of the middle cerebral artery,  which involves posterior part of Inferior Frontal Gyrus CONDUCTION APHASIA *    It is due to functional disconnection between Brocas,   wernicke area. * Speech is Fluent but paraphasic. * Comprehension is intact. *Reading aloud is impaired but reading comprehension is preserved. ANOMIC APHASIA [AIIMS MAY-1999***] *    Articulation,   comprehension,   repetition are  intact. * Confrontation naming,   word finding and spelling  are  impaired.  The speech is fluent but uninformative. *The site of lesion is  the  left hemisphere language network. *It is the commonest language disturbance following head injury,   metabolic encephalopathy and  Alzheimer. ** GLOBAL APHASIA *    The speech output is nonfluent,   and comprehension of spoken language is severely impaired. * Naming,   repetition,   reading,   and writing are also impaired. *It is due to a combined dysfunction of Broca's and Wernicke's areas and results from strokes that involve the entire middle cerebral artery distribution in the left hemisphere. * It is associated with  right hemiplegia,   hemisensory loss,   and homonymous hemianopia.  NON FLUENT TRANS CORTICAL APHASIA (Transcortical Motor Aphasia) *    The features are similar to Broca's aphasia,   but repetition is intact and agrammatism may be less severe. *The lesion site  involves the anterior watershed zone between anterior and middle cerebral artery territories or the supplementary motor cortex in the territory of the anterior cerebral artery. Fluent Transcortical Aphasia (Transcortical Sensory Aphasia) *    It is similar to those of Wernicke's aphasia,   but repetition is intact. *    There is  disconnection of the intact core of the language network from other temporoparietal association areas. *Associated neurologic findings may include hemianopia. *Cerebrovascular lesions (infarctions in the posterior watershed zone) or neoplasms that involve the temporoparietal cortex posterior to Wernicke's area are the most common causes.


Positive Potentials for JIPMER

1) Healthy kidneys receive 20% of the cardiac output and account for 10% of resting oxygen consumption. 2) Amphotericin B -causes direct tubular toxicity mediated by reactive oxygen species. Presents with polyuria, hypomagnesemia, hypocalcemia, and nongap metabolic acidosis. 3) The kidney is one of the most radiosensitive organs, and injury can result with as little as 4–5 Gy exposure4) Renal Vein Thrombosis- present with flank pain, tenderness, hematuria, rapid decline in renal function, The most sensitive test is CT angiography, which is nearly 100% sensitive5) Painful Bladder Syndrome-Interstitial cystitis/painful bladder syndrome (IC/PBS) is characterized by pain from the urinary bladder, urinary urgency and frequency, and nocturia, in the absence of urinary infection or other obvious pathology. It is more common in women. 10% of patients with IC/PBS have a Hunner's ulcer. It can be associated with fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, vulvodynia, and migraine. These syndromes collectively are known as functional somatic syndromes (FSSs) (laboratory tests and histologic findings are normal. )


Harrison's Daily Dose - Haematology

xerocytosis Disorder of cation transport system in RBC membrane with dehydrated RBC (high MCHC)- autosomal dominant mode of transmission Familial Hemolytic Uremic Syndrome (HUS) Microangiopathic hemolytic anemia with fragmented erythrocytes in the peripheral blood smear, thrombocytopenia (usually mild), and acute renal failure.Mutations in any one of several genes encoding complement regulatory proteins: complement factor H (CFH), CD46 or membrane cofactor protein (MCP), complement factor I (CFI), complement component C3, complement factor B (CFB), and thrombomodulin. Myelodysplasia Treatment-anti-CD52 monoclonal antibody Campath - especially effective in younger MDS patients (younger) ,who bear the histocompatability antigen HLA-DR15. Th commonest type with more blasts-Refractory anemia with excess blasts, type 2 (RAEB-2) - (blast 5-19%)The commonest type of MDS is Refractory anemia with excess blasts, type 1 (RAEB-1) WHO Classification of Chronic Myeloproliferative Disorders Chronic myelogenous leukemia, bcr-abl–positive Chronic neutrophilic leukemiaChronic eosinophilic leukemia, not otherwise specified Polycythemia vera Primary myelofibrosis Essential thrombocytosis Mastocytosis Myeloproliferative neoplasms, unclassifiable Renal Disease- with POLYCYTHEMIA Renal artery stenosis Focal sclerosing or membranous glomerulonephritis Postrenal transplantation Renal cysts Bartter's syndrome Erythropoietin receptor mutation VHL mutations- (Chuvash polycythemia) TREATMENT-Primary myelofibrosis Erythropoietin may worsen splenomegaly for unexplained reasons, splenectomy increases the risk of blastic transformation IFN-Alfa Glucocorticoids Thalidomide Allogeneic bone marrow transplantation is the only curative treatment JAK2 inhibitors -phase III clinical trials.(18th) New drug for CMLBosutinib - Src and Abl TK inhibitor. MANTLE CELL LYMPHOMA Overexpression of the ALK protein is an important prognostic factor, with patients over expressing this protein having a superior treatment outcome. The ALK inhibitor crizotinib appears highly active. Splenic Marginal Zone Lymphoma Mainly small lymphocytes. Splenic hilar nodes, bone marrow, and peripheral blood may be involved. The circulating tumor cells have short surface villi and are calledvillous lymphocytes. Express surface immunoglobulin and CD20, but are negative for CD5, CD10, and CD103. Mid-fifties and men and women are equally represented. Autoimmune anemia or thrombocytopenia may be present. About 40% of patients have either deletions or translocations involving 7q21, the site of the CDK6 gene. The genetic lesions typically found in extranodal marginal zone lymphomas [e.g. trisomy 3 and t(11;18)] are uncommon in SMZL. The clinical course of disease is generally indolent. -undergo histologic progression to diffuse large B cell lymphoma.


Secret immunology points for AIPG

Neutrophil disorders Pelger Heut anomaly is a autosomalDominant concition with Bililobednuclei.Acquired bilobed nuclei, pseudoPelger-heut anomaly occurs with acute infections or in myelodysplasticsyndromes.Toxic granulation occursin sepsis. Dohle bodies are cytoplasmicinclusions,which are fragments of ribosomerich endoplasmic reticulum.Kostman’ssyndrome.(AIIMS-NOV-2009***) is dueto mutations in the anti-apoptosis gene HAX-1. Shwachman- Diamond –- Bodian (SDBS)syndrome is Neutropenia + Pancreaticinsufficiency and is due to mutation in SDBS gene.Hereditary cyclic neutropenia is due to mutations in neutrophilelastase (ELA-2); cartilage-hair hypoplasiasyndrome is due to mutations in themitochondrial RNA-processing endoribonuclease RMRP. WHIM [warts, hypogammaglobulinemia,infections,myelokathexis (retention of WBCs in the marrow)] syndrome,ischaracterized by neutrophil hypersegmentation and bone marrow myeloidarrest and is due to mutations in thechemokine receptor CXCR4.Absence of both myeloid and lymphoid cells is seen in reticular dysgenesis,which is due to mutations in the nucleargenome-encoded mitochondrial enzyme adenylate kinase-2 (AK2).Disorders ofChemokinesis-chemotaxis Chédiak-Higashi syndrome, neutrophil-specificgranule deficiency, hyper IgE–recurrent infection (Job’s) syndrome, Downsyndrome, -mannosidase deficiency, severe combined immunodeficiency,Wiskott-Aldrich syndrome.Disorders of MicrobicidalactivityChédiak-Higashi syndrome, neutrophil-specific granule deficiency,chronic granulomatous disease, defects in IFN-gamma/IL-12 axis. Leukocyte AdhesionDeficiency disorders Type 1: Delayed separation of umbilical cord,sustained neutrophiliaType 2: Mental retardation, short stature,Bombay (hh) blood phenotype, recurrent infections, neutrophilia-antibodiesagainst CD15sType 3: Petechial hemorrhage, recurrentinfections-Mutation in FERMT3.**Chediak – Higashi syndrome is an autosomal Recessivedisorder due to defect in lysosomal transport proteinLYST, encoded by gene CHSI at Chr 1q. There is defect in packaging and distributing granules.So all cells which have granules will be affected,namelyneutrophis ,melanocytes,platelets and schwann cell(myelin).NK cellfunction is also impaired.Defects are decreased chemotaxis and phagylosome fusion,decreased egress from marrow,abnormal skin window and increased respiratory burst activity.The common organism is staph. Aureus, Catalase positiveorganisms are S aureus, Burkholderia cepacia, Aspergillus, and Chromobacterium violaceum. Autosomal recessive defectsin dedicator of cytokinesis 8 (DOCK8). In DOCK8 deficiency, IgE elevation is associated with severe allergy, viral susceptibility, and increased rates of cancer.Recombinant human IFN-gamma-uses are patients with CGD , leprosy, nontuberculousmycobacteria, and visceral leishmaniasis.(???AIPG14/15***)CD classification of human lymphocyte was started on 1982Co-stimulatorymolecules are molecules of antigen-presenting cells (such as B7-1 andB7-2 or CD40) that lead to T cell activation when bound by ligands on activatedT cells (such as CD28 or CD40 ligand).Absence of costimulatory molecule willlead toanergy or tolerance.Chronic granulomatous disease-It is a disorder of monocyte andgranulocyte oxidative metabolism. It is rare, 1 in ½ lakh.70% are X linked recessive and 30% are Autosomalrecessive.There is severely decreasedH202 production.Infections with catalase positive organism are common.(They destroy their own H202production).There is extensive inflammation and granuloma formation.There is norespiratory burst due to lack of 1 of 4 NADPH oxidase in neutrophils,monocytes, eosinophils.Inflammasome are largecytoplasmic complexes of intracellular proteins that link the sensing ofmicrobial products to the proteolytic activation of interleukin (IL)-1 andIL-18 inflammatory cytokines.NK(15%) cells are nonadherent, nonphagocytic cells with largeazurophilic cytoplasmic granules. NK cells express surface receptors for CD16 , CD56, CD8, CD3 and proliferate inresponse to IL-2.NK cell cytotoxicity is the nonimmune(effector cell never having had previous contact with the target), MHC-unrestricted,(AIIMS***) non-antibody-mediatedkilling .Mutations of JAK3result in a disorder identical to X-SCID.MHC class I–or class II–restricted means that T cells recognize antigen peptidefragments only when they are presented in the antigen-recognition site of aclass I or class II MHC molecule, respectively. Conventional antigens bind to MHC class I or II molecules in the groove of the heterodimer and bind to Tcells via the V regions of the TCR-alfa and -beta chains.(Fig.93.11).Superantigensbind directly to the lateral portion of the TCR-beta chain and MHC class IIchain.Superantigens are protein molecules capable of activating 20% Tcell, whereas conventional antigens activate Tcell superantigens include staphylococcal enterotoxins. TH1-typehelper T cells mediated cytokines are--IL-2, IFN-, IL-3, TNF-alfa, GM-CSF, andTNF-betaTH2-typehelper T cells mediated cytokines are -IL-3, -4, -5, -6, -10, and -13Th17-type helper T cells(Recent) mediated cytokines are -IL-17, -22, and -26The method todetect apoptosis are Annexin V serology and TUNEL . Terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL)The HLA class III region of HLA is between the class I and class II complexes, which includes genes forTNFalfa- and lymphotoxin (TNF-); the complement components C2, C4, and Bf; heatshock protein (HSP)70; and the enzyme 21-hydroxylase.The alternate pathway C3 convertase is C3bBb.The Classical pathway C3convertase is C4b2aAutosomal DominantHyper-IgE Syndrome (HIES)-Presentation- facialdysmorphy, defective loss of primary teeth, hyperextensibility, scoliosis, andosteoporosis. Elevated serum IgE levels are typical of this syndrome.Wiskott-Aldrich Syndrome-It is X-linked recessive disease caused bymutations in the WASP gene .Triad:recurrent bacterial infections, eczema, and thrombocytopenia. It is prone for lymphoma IgA deficiency is the mostcommon primary immunodeficiency-1 in 600 individuals.Immunodysregulation polyendocrinopathy enteropathy X-linkedsyndrome (IPEX), has food intolerance, skin rashes, autoimmune cytopenias,and diabetes.It is due to loss-of-function mutations in the FOXP3.X-linked NF-kappa-Bessential modulator (NEMO) deficiency– also has mild osteopetrosis,lymphedema, and, anhydrotic ectodermaldysplasia, dysmorphic facies, and abnormal conical teeth.Hereditary angioedema-An autosomal dominant disease due toa deficiency of C1INH (type 1- 85%) and to a dysfunctional protein (type2-15%).Clinical presentation- lacks pruritus and of urticarial lesions;there is nonpitting edema andperiorbital edema; there is prominence of recurrent gastrointestinal attacks ofcolic, and episodes of laryngeal edema.It can mimic an acute abdomen A bradykinin 2 receptor antagonist and ecallantide, kallikreininhibitor, are tried for acute attacks.. The recent C1 inhibitor proteins are cinryze and Berinert. The other C1INH is rhucin, still not approved inU.S.. Icatibant is a bradykinin antagonist recently approved in the U.S. andadministered SC via a single injection


Pearls for AIIMS/JIPMER/PGI/AIPG/DNB - Infections

Following pearls are part of OHC7 classroom crash course for AIIMS/AIPG/DNB/JIPMER and comes from Infection Section of Operation Harri books. The cells which take part in adaptive immunity are T cells. B cells and monocyte - Macrophage system.Treg cells are a subgroup of CD4 + T cells used to prevent autoimmune responses. Mechanism used by Treg cells to down regulate inflammation is the production of the anti-inflammatory cytokine IL - 10.The elevation in body temperature in fever is due to resetting of the hypothalamic set point mediated by cytokines.The term "complement" was introduced by Paul Ehrilich. Complements account for 5% of the globulin fraction of blood serum and can serve as opsinins. C3b can act as opsonins. "Terminal" componenets (C5b, C6, C7, C8 and C9) is called as membrane attack complex. C5a, act as chemoattractants for PMNs.The host receptor for P. Vivax is Duffy Fy antigen. Receptor for P. Falciparum is Glycophorin A and the receptor for Entamoeba histolytica is N-Acetyl glucosamine [APPG-1-2014]Rabies virus grows in striated muscle cells at the site of inoculation.H1-subtype strains of influenza bind to a receptor composed of two sugar molecules: sialic aced linked alfa-2-6 to galactose. This is highly expressed in the airway epithelium. (Since it attaches to the upper airway, it spreads commonly but it is mild).H5N1 avian influenza virus binds to sialic acid linked alfa-2-3 to galactose, and this receptor is highly expressed in pneumocytes in the alveloli. This leds to high mortality rate and also the low human-to-human transmissibility rate.Most capsules are carbohydrates, exception is B. anthracis, which contains polypeptide capsule.LPS from gram negative bacteria attaches with GPI anchored membrane protein - CD14 on the surface of phagocytes.Toll-like receptors (TLRs) are expressed in macrophages and dendritic cells, that recognize microbes. TLRs initiate cellular activation through nuclear factor kappa B (NF-kB), a master - switch for production of inflammatory cytokines. Bacterial flagella binds to TLR5. Pathogens that do not bind to TLR5 are Campylobacter jejuni, Helicobacter pylori, and Bartonella bacilliformis.Inflammasome are the site where inflammatory cytokines IL-1beta and IL-18 are changed from their precursor to active forms prior to secretion by the cysteineprotease caspase-1. Four inflammasomes with different components are formed: the IPAF inflammasome, the NALP1 inflammasome, the cryoprin/NALP3 inflammasome, and an inflammasome triggered by Francisella tularensis infection.Adenovirus inhibits host defense by decreasing production of major histocompatibility complex molecules. Epstein-Barr virus and cytomegalovirus inhibits host defense by diminishing cytotoxic T cell recognition of virus-infected cells.Organisms that act by inhibiting ADP-ribotransferase activity are cholera, Diphtheria Pertussis, E.Coli - heat labile toxin and P. Aeuroginosa exotoxin (A, S and T). Pseudomonas inject toxins directly into host Target cells by means of a complex set of proteins leading to stimulation of Type III system.

HARRISON's Secrets

5 high yielding topics to boost you PG entrance exam preparation

Liver transplantLithium induced nephrogenic DIRefeeding syndromeMitochondrial DNAStemcells 1) Liver transplant It is an orthotopic transplantation, in which the native organ is removed and the donor organ is inserted in the same anatomic location. It was done 1st by Thomas Starzl, England in 1963. ). The first report of successful Living donor LT was by Dr. Christoph Broelsch in November 1989Cold ischemic time-ideally is less than 12 hours. Perfusion fluid- use of University of Wisconsin (UW) solution, rich in lactobionate and raffinose Hepatitis C, is the most frequent indication for liver transplantation in adults. (AIPG-2010***) and in children ,it is biliary atresiaModel for end-stage liver disease (MELD) (TN-2011***)- it is the score which is currently considered for patients awaiting liver transplant. It has 3 variables-bilirubin, sr. Creatinine and prothrombin timeFor children <18 years of age, the Pediatric End-Stage Liver Disease (PELD) scale is used. This scale is based on albumin, bilirubin, INR, growth failure, and age.Living donor liver transplantation for pediatric recipients involves removal of approximately 20% of the liver (Couinaud segments 2 and 3*In amyloidosis ATTR type - transthyretin [AIIMS N0V - 2007***] is the primary protein component and this presents with orthostatic hypotensison. Treatment of familial cases with liver transplantation are tried. Liver Transplantation- OLTX is done for patients with a single lesion 5 cm or three or fewer nodules, each 3 cm (Milan criteria).This resulted in excellent tumor-free survival (70% at 5 years).Crigler-Najjar type I - complete absence of bilirubin UDPGT activity- the only effective treatment is orthotopic liver transplantation.The vanishing bile duct syndrome and adult bile ductopenia - histologic picture is similar to that found in primary biliary cirrhosis. It is seen in chronic rejection after liver transplantation, in those who develop graft-versus-host disease after bone marrow transplantation, sarcoidosis, chlorpromazine. Child-Pugh class B-indicates need for liver transplantParacetomol toxicity- Lactate levels >3. 5 mmol / L might need liver replacement. Among children listed as candidates for liver transplantation, valproate is the most common antiepileptic drug implicated.(In children the commonest indication is biliary atresia) 2) Lithium induced nephrogenic DI Lithium causes NDI by multiple mechanisms, including direct inhibition of renal glycogen synthase kinase-3 (GSK3), GSK3 is required for the response of principal cells to AVP.The entry of lithium through the amiloride-sensitive Na+ channel ENaC is required for the effect of the drug on principal cells; thus, combined therapy with lithium and amiloride can stop lithium-associated NDI. 3) Refeeding syndrome In the early stages of treatment, severely malnourished patients may develop a "refeeding syndrome" characterized by hypophosphatemia, hypomagnesemia, and cardiovascular instability. 4) Mitochondrial DNA Mitochondria are cytoplasmic organelles whose major function is - oxidative phosphorylation in aerobic conditions by the respiratory electron transport chain (ETC) multiprotein enzyme complexes I–V and the two electron carriers, coenzyme Q (CoQ) and cytochrome c. Complex II, CoQ, and cytochrome c are encoded by nuclear DNA. In contrast, complexes I, III, IV, and V contain at least some subunits encoded by mtDNAmtDNA molecules do not undergo recombination.Mothers transmit their mtDNA to both their sons and their daughters"Threshold" effect in which the actual expression of disease depends on the relative percentage of mitochondria whose function is disrupted by mtDNA mutations. The most striking overall characteristic of mitochondrial genetic disease is the phenotypic heterogeneity associated with mtDNA mutations. Mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) is the most common mtDNA disease, consisting of a progressive encephalomyopathy characterized by repeated strokelike events involving mainly posterior cerebral areas 5) Stem cells The term potency is used to indicate a cell's ability to differentiate into specialized cell types. Cultured stem cells are grouped according to their potency. Potency- The hierarchy of stem cells are given below.Totipotency> Pluripotent> Multipotent cells> Oligopotent cells> Unipotent cells> Terminally differentiated cells.Three genes,namely Pou5f1 (Oct3/4), Nanog, and Sox2 are the key gene regulatory pathways of self-renewalUmbilical Cord Stem Cells are associated with less graft-versus-host disease.Hematopoietic stem cells express the following cell surface molecules - CD34, Thy-1 (CD90), c-Kit receptor (CD117), CD133, CD164, and c-Mpl (CD110, also known as the thrombopoietin receptor).