AIIMS NOV 2007 Exam Recalls

Discussion in 'AIIMS Nov 2013' started by Guest, Nov 5, 2007.

  1. manish.

    manish. Guest

    ANSWER OF PEDIATRIC SURGERY IN AIIMS NOV 07



    A 12 yr old boy with pectus excavatum is brought for evaluation. He has no complaints of breathless on exertion however complains of intermittent wheezing. On examination mild pectus excavatum deformity of the chest is noted. which of the following findings on PFTs is an indication for surgery?
    choices:
    A)FEV1/FVC < 0.6
    B)low exercise tolerance at maximal capacity
    C)TLC 80% of predicted
    D)PEFR 60% of predicted

    Indications for surgical repair
    Operative correction should be considered in patients who present with pectus excavatum and cardiopulmonary impairment. The most common goal in operative repair of pectus excavatum is to correct the chest deformity. This is particularly important in teenagers, in whom the appearance of the chest can result in significant problems related to body image and self-esteem. Thus, the desire to improve the appearance of the chest is considered an appropriate medical indication for surgery. Images 1-3 illustrate the dramatic appearance of pectus excavatum in young male and female patients.

    Other indications include exercise and physical activity limitations, evidence of cardiac or pulmonary dysfunction, chest pain, psychological distress, and potential future need for sternotomy (open-heart surgery). Adult patients with pectus excavatum who undergo open-heart surgery typically have significant displacement and rotation of the heart to the left chest. This can make the operative approach to the heart at the time of open-heart surgery difficult and challenging. With this in mind, elective repair of the pectus deformity prior to open-heart surgery may be indicated in selected cases.

    SO ANS PROBABLY IS PES EXCAVATUM
  2. manish.

    manish. Guest

    HACEK group includes all except:

    HACEK group includes all except:
    a. Hemophilus aprophillus
    b. Acinetobacter baumanni
    c. Eikenella corrodens
    d. Cardiobacterium hominis
    Ans Haemophilus aphrophilus (and Haemophilus paraphrophilus), Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella kingae.
  3. manish.

    manish. Guest

    about the diagnosis of aspergillosis-
    DIAGNOSIS
    The repeated isolation of Aspergillus from sputum or the demonstration of hyphae in sputum or bronchoalveolar lavage fluid suggests endobronchial colonization or infection. Even a single isolation of Aspergillus from the sputum of a neutropenic patient or a hematopoietic stem-cell transplant recipient with pneumonia, particularly a child or a nonsmoker, suggests the diagnosis of invasive aspergillosis. In patients with advanced AIDS, fever, and cough, the isolation of Aspergillus from respiratory secretions raises the possibility of aspergillosis and thus should prompt bronchoscopy. Fungus ball of the lung is usually detectable by chest x-ray. IgG antibody to Aspergillus antigens is demonstrable in the serum of many colonized patients and of virtually all patients with fungus ball. Patients with allergic bronchopulmonary aspergillosis have specific serum IgE antibody to Aspergillus antigens and often have IgG antibody as well. No standardized test for these antibodies exists. Serum IgE concentrations are often >1000 ng/mL.
    Biopsy is usually required for the diagnosis of invasive aspergillosis of the lung, nose, paranasal sinus, bronchi, or sites of dissemination. Blood cultures are rarely positive, even in patients with infected cardiac valves (native or prosthetic). Detection of galactomannan antigen in serum suggests the diagnosis, but sensitivity is low early in the disease and false-positive results occur, particularly in children. Aspergillus hyphae can be identified presumptively by histology, but culture is required for confirmation and for identification of the species. Only culture can reliably distinguish aspergillosis from pseudallescheriasis; drug therapy for these two diseases differs.
  4. manish.

    manish. Guest

    which drug shud be stopped on day of surgery? is it metformin
  5. manish.

    manish. Guest

    regarding q on fluorosis? the wrong statement shud be 'it is the MC cause of dental caries' isnt it?

    a patient who had typhoid 4m back with hair loss now... ans most probably telogen effluvium

    groundnut oil(peanut) does not contain w3 FA.. I THINK
  6. Guest

    Guest Guest

    From the discussion so for...

    the answer came is

    hypervitaminosis.

    In goodman and gillman.

    Therapeutic use of bisphosphonate...is used in malignancy associated hypercalcemia .
    Zoledronate is more effective than palmidronate.

    Postmenopausal Osteoporosis - clinical trial shows an increase in bone density..

    Bisphosphonate also act as anti cancer by inactivating cancer associated proteins as Ras..

    Zolidronate has been used sucessfully as an adjuvent in treating philadephia chromosome + CML

    hence here odd one ...anwer is hypervitaminosis ..
  7. shardul.

    shardul. Guest

    18 year old girl presents with amenorrhoea, weight loss and milky discharge from the breast . Most likely diagnosis is:
    a. Anorexia nervosa
    b. Occult carcinoma
    c. Hypothyroidism
    d. HIV


    Anorexia nervosa and HIV - cant explain galactorrhoea.
    Hypothyroism - cant explain weight loss
    so ans must be occult ca
  8. shardul.

    shardul. Guest

    Subependymal giant-cell astrocytomas most often originate from the walls of the lateral ventricles in patients with tuberous sclerosis.
    Although histologically benign, SEGAs commonly arise in the lateral ventricles near the foramen of Monro and can result in obstructive hydrocephalus.
  9. shardul.

    shardul. Guest

    mustard oil contains pufa and is protective against heart diseases...ref-net
    ground nut is the answer



    regarding the q abt sexual differentiation....i think 2 options r correct.. ie; it starts by 5th week and male differentiation takes place (7th week) before female(8-9weeks).ref is dutta embroyology 3rd edition page 221
  10. aadi.

    aadi. Guest

    Q. In emergency tracheostomy the following structures are damaged except:
    a. Isthmus of the thyroid
    b. Inferior thyroid artery
    c. Thyroidea ima
    d. Inferior thyroid vein


    . A female come with complaints of chest pain.On examination she is found to have pericarditis with pericardial effusion. The pain is mediated by:
    a. Deep cardiac plexus
    b. Superficial cardiac plexus
    c. Phrenic nerve
    d. Subcostal nerve
  11. aadi.

    aadi. Guest

    A 2 year old female child developed fever, cough and respiratory distress. On chest x-ray consolidation is seen in right lower lobe. She improved with antibiotics but on follow up at 8 weeks was again found to have increasing consolidation in right lower lobe and fever. Your next investigation would be:
    a. Bronchoscopy
    b. Bacterial culture of the nasopharynx
    c. CT scan of the chest
    d. Allergen sensitivity test
  12. kavish

    kavish Guest

    female come with complaints of chest pain. On examination she is found to have pericarditis with pericardial effusion. The pain is mediated by:
    a. Deep cardiac plexus
    b. Superficial cardiac plexus
    c. Phrenic nerve
    d. Subcostal nerve

    REF:
    Anteriorly the pericardium is related to the sternum, third
    to sixth costal cartilages, lungs and the pleura. Posterior
    relations are oesophagus, descending aorta and T5–T8
    vertebrae. Laterally on either side lie the root of the lung,
    mediastinal pleura and the phrenic nerve. Innervation of the
    fibrous and the parietal layer of serous pericardium is by the
    phrenic nerves. Pericardial pain originates in the parietal
    layer and is transmitted by the phrenic nerves. The
    pericardial cavity is closest to the surface at the level of the
    xiphoid process of sternum and the sixth costal cartilages.
  13. kavish

    kavish Guest

    Regarding genital development, true is:
    a. Y chromosome is associated with ovary development
    b. Genital ridge starts developing at 5th week
    c. Male genitals develop earlier than female genitals
    d. External Genital development is complete by 10th week

    REF:

    At the end of the fifth week of development, or during the
    sixth, PGC reach the gonadal anlage, colonizing the most
    superficial areas of the developing ovary (Makabe et al.,
    1991). The first morphological evidence of the future
    gonads is represented by the appearance, in the 4-week-old
    human embryo, of two longitudinal prominences, the
    genital ridges, between the developing mesonephros and
    the dorsal mesentery root. These elongated prominences
    rapidly shorten, acquiring the aspect of hemispherical
    extrusions bulging into the coelomic cavity (Makabe et al.,
    1989, 1991). By week 5, the genital ridges are formed by
    different types of somatic cells:
    a proliferating coelomic
    epithelium covering the developing gonad and an underlying
    compartment (Figure 14). This contains
    mesenchymal cells, blood vessels, and mesonephric cells
    deriving, via the rete system, from mesonephric glomeruli
    Figure 14. Seven-week-old human embryo. The developing ovary
    is covered by the coelomic epithelial cells (CE) that, together with
    the cells of the underlying compartment (mesenchymal and
    mesonephric) form somatic cords (S). These cords surround the
    germ cells (G) and intermingle with them. Light microscopy,
    original magnification ×600.
    Figure 15. Six/seven-week-old human embryo. Gonadal PGC (G),
    closely intermingling with cells of somatic cords (S), are seen. Note
    the eccentric position of the nucleus. Transmission electron
    microscopy, original magnification ×4500.
    and tubules (Byskov, 1986). However, which of these
    components of the developing gonad has a leading role in
    the formation of the ovarian blastema is still an unresolved
    question (Wartenberg, 1989). PGC appear by light microscopy
    as large, rounded and pale elements intermingling
    with the somatic cells of the gonadal primordium
    (Figure 14). By TEM, gonadal PGC appear to be rounded
    or elliptical cells (Figure 15), sometimes with small cytoplasmic
    processes irregularly distributed over their surface
    (Fukuda, 1976). We have also occasionally observed PGC
    that had one or two amoeboid processes: presumably
    gonadal PGC still retain their motile capability, wandering
  14. nikky

    nikky Guest

    ground nut oil contains omega 6 pufa and it lacks omega 3 pufa mow attempts are being made to make genetically enginered ground nuts with high omega 3
    vhl has both islet cell tumor and endolymphatic cysts ...harrison ....that make hemangiendothelioma the answer cos vhl has hemangioblastoma mmr is calculated per 1000 live births but in western world where mmr is very low its calculated in 1 lakh live births to avoid fractions and decimals...... park

    among the options given fotr diagnosis of acute hepatitis ..... no option mathche the table given in harrisson. HBV DNA has been mentioned under chronis hepatitis.NOW SPRINGERLINK JOURNAL write hbv dna is positive early in infection. e medicine has given it as a marker of acute hepatitis ..... but its very costly and not readily awialble


    b-blockers are contraindicated in diabetics as they mask the symptoms of hypoglycaemia should they ever occur, so clearly glucocorticoids would be the ans to this one.
  15. nikky

    nikky Guest

    True about protease inhibitors are all except:
    a. Acts as a substrate for P-glycoprotein(P-gp) and action is mediated by mdr-1 gene
    b. Hepatic oxidative metabolism
    c. All protease inhibitors interfere with metabolism by drug interactions
    d. Saquinavir causes maximum induction of CYP3A4

    Goodman gillman 11th edition

    An important toxicity common to all approved HIV protease inhibitors is the potential for metabolic drug interactions (Tables 50-5 and 50-7). Most of these drugs inhibit CYP3A4 at clinically achieved concentrations, although the magnitude of inhibition varies greatly, with ritonavir being by far the most potentRitonavir, nelfinavir, and amprenavir are also moderate inducers of hepatic enzymes including CYP3A4 and glucuronosyl S-transferase. Concentrations of all approved HIV protease inhibitors may be reduced in the presence of other CYP inducers. Patients and care providers must be vigilant about the possibility of clinically significant pharmacokinetic drug interactions in patients receiving these drugs. Nausea, vomiting, and diarrhea are also common, although symptoms generally resolve within 4 weeks of starting treatment.

    So it is ritonavir not sequinavir which is most potent.

    Saquinavir is metabolized primarily by intestinal and hepatic CYP3A4 (Fitzsimmons and Collins, 1997). Its metabolites are not known to be active against HIV-1. Saquinavir and its metabolites are eliminated through the biliary system and feces (95% of drug), with minimal urinary excretion (3%). Saquinavir's short half-life requires administration every 8 hours

    plus kdt clearly mentions that saquinavir is least potent
  16. aayush.

    aayush. Guest

    50. Asherman’s syndrome is diagnosed by all except:
    a. HSG
    b. Hysteroscopy
    c. Endometrial culture
    e. Saline infusion USG
    Danforth obstruction and gynacology 9th edition

    The diagnosis of Asherman syndrome can be made by hysterosalpingogram, saline infusion sonohysterography, or direct visualization with diagnostic hysteroscopy
  17. aayush.

    aayush. Guest

    Hypertension with hypokalemia is seen in all except:
    a. B/L renal artery stenosis
    b. End stage renal disease
    c. Cushing’s disease
    d. Primary hyperaldosteronism


    Harrison 16th editioon page 1655
    Hypokalemia is uncommon in CRD and esrd and usually reflects markedly
    reduced dietary K_ intake, in association with excessive diuretic therapy
    or gastrointestinal losses.all other are associated with hypokalemia with hypertension.
    so answer is b end stage renal disease
  18. aayush.

    aayush. Guest

    45. Extensive involvement of deep white matter with hyperintense thalamic lesion on noncontrast ct scan of the brain is seen in:
    a. Alexander’s disease
    b. Krabbe’s ds.
    c. Canavan’s ds
    d. Metachromatic leucodystrophy

    goetz clinical neurology2007 edition
    ALEXANDER'S DISEASE
    This degenerative disorder is a primary genetic disorder of astrocytes, one of the major cell types in the CNS white matter (see Table 30-6 ). As a result, there is a global dysmyelination or demyelination of the CNS. The pathological hallmark of all forms of Alexander's disease is the presence in astrocytes of cytoplasmic eosinophilic hyaline bodies, called Rosenthal fibers. These inclusions are particularly numerous beneath the pial arachnoid membranes, beneath the ependyma, and around the cerebral blood vessels and are found throughout most of the brain. Rosenthal fibers contain the glial fibrillary acid protein (GFAP), an intermediate filament protein. Rosenthal fibers occur especially in the optic nerves, optic tracts, optic chiasm, cerebral peduncles, and spinal cord. Abnormal astrocytes are also observed in the basal ganglia, thalamus, and hypothalamus. Although the astrocytes become distended, there is no evidence of abnormal storage material within neurons. The white matter is soft and retracted and shows a variable loss of myelin, which appears more marked and diffuse in infantile cases. Axon cylinders are preserved. Rosenthal fibers in Alexander's disease result from molecular alterations and overproduction of GFAP. Alexander's disease has been shown to result from multiple mutations in the GFAP gene on chromosome 17.[73] In all patients, the GFAP mutations are dominant. The mutations usually arise de novo or from germinal mosaicism in cases with early onset. In adult forms, milder mutations are autosomal dominant.[74]
    The rare neonatal form is characterized by early, often intractable, multifocal seizures. Hydrocephalus occurs and is due to three factors: aqueductal stenosis from the propagation of astrocytes containing excessive amounts of eosinophilic cytoplasmic material, lack of developmental maturation of the aqueductal area, and elevated protein content in the cerebrospinal fluid (CSF).[75]
    The most common form of Alexander's disease is the infantile form, which has an average onset at 6 months of age. However, onset may occur at any time from shortly before birth to as late as 2 years of age. The average duration of disease is 2 to 3 years, but it can vary from a few months to several years. Psychomotor retardation predominates initially, but later progressive spasticity and seizures in the context of megalocephaly, with or without frank hydrocephalus, develop.
    In the juvenile form, which is much less common than the infantile form, onset usually occurs between 7 and 14 years of age, and the duration is approximately 8 years. Bulbar and pseudobulbar dysfunction predominates, with dysphagia, dysarthria, nystagmus, ptosis, full facial palsy, and tongue atrophy.[76] Generalized spasticity and weakness may also occur, but unlike the severe mental retardation characteristic of the infantile form, mentation tends to remain intact. The adult form of Alexander's disease has an early stuttering course mimicking multiple sclerosis and characterized clinically by blurred vision, spasticity, nystagmus, dysarthria, and dysphagia. Other reported neurological manifestations include palatal and ocular myoclonus.[77]
    CT changes include low attenuation in the deep cerebral white matter, most extensively in the frontal lobes and subependymal regions. The ventricles are variably enlarged. There is inconsistent abnormal enhancement of the caudate nuclei, anterior columns of the fornix, optic radiations, and periventricular areas.Since the types of Alexander's disease are phenotypically distinct, the differential diagnosis varies by age. If one encounters an infant with chronically developing megalocephaly or macrocephaly with mild regression in psychomotor milestones in the absence of any other obvious cause, Alexander's disease is a highly probable diagnosis. Juvenile leukoencephalopathy must be considered in children and multiple sclerosis in adults.
    No specific therapy is available for Alexander's disease. Much supportive care, however, is necessary, including good nutrition and generous use of antibiotics and antiepileptics. Despite these measures, the prognosis for infants and children with this disease at present is poor. Recent advances in positron-emission tomography scanning and single photon emission computed tomography indicate that there is an abnormal flow of spinal fluid through the blood-brain barrier. Clearer understanding of this disorder may aid in developing interventions.

    Krabbe's Syndrome
    In contrast to other leukodystrophies, Krabbe's syndrome appears to be purely neurological. In these patients, there is a deficiency of the lysosomal enzyme galactocerebroside β-galactosidase (GALC). The disease is inherited as an autosomal-recessive trait. The various forms of the disease result from multiple mutations in the GALC gene on chromosome 14q24.3-q32.1.[83] Assays of amniotic fluid allows prenatal diagnosis, and enzymatic assays on white blood cells or serum may detect the carrier state. A saposin A deficiency is expected to be responsible for some cases of Krabbe's syndrome with near normal GALC activity.
    In the CNS, the white matter is decreased in mass and is gliotic, firm, and rubbery to palpation. In the areas of demyelination, oligodendroglial cells are severely reduced in number. Globoid cells, found deep in the white matter around and within vessels, are distinctive. Their abundant cytoplasm stains positively with periodic acid–Schiff (PAS) and only faintly with Sudan black. No metachromasia is present. The small globoid cells are round and mononuclear, while the large one (varying from 20 to 50 μm) become irregular and multinucleated
    Electron microscopy reveals within the cytoplasm of the cells cytoplasmic inclusions containing both electron-dense linear or curved tubular profiles, a distinctive sign in Krabbe's disease.[84] Neurons in the pons, thalamus, and dentate nuclei show varying degrees of degeneration, but the neuronal processes of the cerebral cortex are reasonably well preserved. Involvement of the peripheral nervous system varies, and segmental demyelination has been reported.[85] Globoid cells are not seen in peripheral nerves, but histiocytes with foamy cytoplasm and tubular inclusions have been demonstrated. Krabbe's disease is distributed worldwide and has no gender, racial, or ethnic proclivities. There are two primary forms of Krabbe's disease, the early-onset or classic form, and the late-onset form. The early-onset type usually appears clinically between the third and eighth months of life, although biochemical and pathological changes are present in the early gestational period. Initially, the patient is irritable and has intermittent fever for no discernible reason. Episodic limb or trunk rigidity may occur, and a heightened startle response to noise, light, or touch may be apparent. Feeding problems and seizures occur frequently. Myoclonic seizures may suggest infantile spasms. Macrocephaly is uncommon, and hepatosplenomegaly is absent. There are usually no cherry-red spots. Early in the course, EEG shows a severe slowing of the background activity and abundant paroxysmal discharges. By 6 months of age, the infant develops severe hypertonus with obvious opisthotonos and decorticate-decerebrate rigidity. Reflexes may be hyperactive or hypoactive if advanced peripheral neuropathy is present. Babinski's signs are usually present along with optic atrophy. By 9 months of age, the baby is usually blind and deaf. In light of these characteristic clinical features, there is very little confusion between the diagnosis of classic Krabbe's disease and other infantile-onset degenerative conditions (Video 105, Myoclonic Seizure).
    The late-onset type of Krabbe's disease is extremely uncommon. These patients first develop difficulties later in infancy, in childhood, or even in adult life.[86] The late infantile form is characterized by the onset between 6 months and 3 years of developmental regression with ataxia and stiffness, feeding difficulties, irritability, and loss of vision. The juvenile or adult form is usually characterized by a progressive amaurosis starting in late childhood, followed years later by a progressive gait impairment with spasticity or dystonia, and finally dementia. Optic atrophy and peripheral neuropathy are consistent findings late in the course of the illness.
    Periventricular hyperdensities demonstrated on CT scans have been reported, particularly in the terminal phases of the classic form. MRI scans demonstrate white matter involvement of the cerebrum and cerebellum,[87] and electrophysiological evidence of peripheral nerve demyelination is prominent. Treatment at this time is limited to allogenic hematopoietic stem cell transplantation that appears to slow the progression of the disease and improve magnetic resonance images. Studies using stem cells and viral vectors to transduce transplantable cells are under way.[88]

    these were the facts about alexander and krabbe's disease but i choose alexander disease as the answer because in alexander disease global demylination is seen and hyperintence ct is seen in several areas while in krabbe a few reported with hypreintense ct and that too most common in terminal phase of classic subtype so i choose alxander as the asnwer.
  19. aayush.

    aayush. Guest

    45. Extensive involvement of deep white matter with hyperintense thalamic lesion on noncontrast ct scan of the brain is seen in:
    a. Alexander’s disease
    b. Krabbe’s ds.
    c. Canavan’s ds
    d. Metachromatic leucodystrophy

    goetz clinical neurology2007 edition
    ALEXANDER'S DISEASE
    This degenerative disorder is a primary genetic disorder of astrocytes, one of the major cell types in the CNS white matter (see Table 30-6 ). As a result, there is a global dysmyelination or demyelination of the CNS. The pathological hallmark of all forms of Alexander's disease is the presence in astrocytes of cytoplasmic eosinophilic hyaline bodies, called Rosenthal fibers. These inclusions are particularly numerous beneath the pial arachnoid membranes, beneath the ependyma, and around the cerebral blood vessels and are found throughout most of the brain. Rosenthal fibers contain the glial fibrillary acid protein (GFAP), an intermediate filament protein. Rosenthal fibers occur especially in the optic nerves, optic tracts, optic chiasm, cerebral peduncles, and spinal cord. Abnormal astrocytes are also observed in the basal ganglia, thalamus, and hypothalamus. Although the astrocytes become distended, there is no evidence of abnormal storage material within neurons. The white matter is soft and retracted and shows a variable loss of myelin, which appears more marked and diffuse in infantile cases. Axon cylinders are preserved. Rosenthal fibers in Alexander's disease result from molecular alterations and overproduction of GFAP. Alexander's disease has been shown to result from multiple mutations in the GFAP gene on chromosome 17.[73] In all patients, the GFAP mutations are dominant. The mutations usually arise de novo or from germinal mosaicism in cases with early onset. In adult forms, milder mutations are autosomal dominant.[74]
    The rare neonatal form is characterized by early, often intractable, multifocal seizures. Hydrocephalus occurs and is due to three factors: aqueductal stenosis from the propagation of astrocytes containing excessive amounts of eosinophilic cytoplasmic material, lack of developmental maturation of the aqueductal area, and elevated protein content in the cerebrospinal fluid (CSF).[75]
    The most common form of Alexander's disease is the infantile form, which has an average onset at 6 months of age. However, onset may occur at any time from shortly before birth to as late as 2 years of age. The average duration of disease is 2 to 3 years, but it can vary from a few months to several years. Psychomotor retardation predominates initially, but later progressive spasticity and seizures in the context of megalocephaly, with or without frank hydrocephalus, develop.
    In the juvenile form, which is much less common than the infantile form, onset usually occurs between 7 and 14 years of age, and the duration is approximately 8 years. Bulbar and pseudobulbar dysfunction predominates, with dysphagia, dysarthria, nystagmus, ptosis, full facial palsy, and tongue atrophy.[76] Generalized spasticity and weakness may also occur, but unlike the severe mental retardation characteristic of the infantile form, mentation tends to remain intact. The adult form of Alexander's disease has an early stuttering course mimicking multiple sclerosis and characterized clinically by blurred vision, spasticity, nystagmus, dysarthria, and dysphagia. Other reported neurological manifestations include palatal and ocular myoclonus.[77]
    CT changes include low attenuation in the deep cerebral white matter, most extensively in the frontal lobes and subependymal regions. The ventricles are variably enlarged. There is inconsistent abnormal enhancement of the caudate nuclei, anterior columns of the fornix, optic radiations, and periventricular areas.Since the types of Alexander's disease are phenotypically distinct, the differential diagnosis varies by age. If one encounters an infant with chronically developing megalocephaly or macrocephaly with mild regression in psychomotor milestones in the absence of any other obvious cause, Alexander's disease is a highly probable diagnosis. Juvenile leukoencephalopathy must be considered in children and multiple sclerosis in adults.
    No specific therapy is available for Alexander's disease. Much supportive care, however, is necessary, including good nutrition and generous use of antibiotics and antiepileptics. Despite these measures, the prognosis for infants and children with this disease at present is poor. Recent advances in positron-emission tomography scanning and single photon emission computed tomography indicate that there is an abnormal flow of spinal fluid through the blood-brain barrier. Clearer understanding of this disorder may aid in developing interventions.

    Krabbe's Syndrome
    In contrast to other leukodystrophies, Krabbe's syndrome appears to be purely neurological. In these patients, there is a deficiency of the lysosomal enzyme galactocerebroside β-galactosidase (GALC). The disease is inherited as an autosomal-recessive trait. The various forms of the disease result from multiple mutations in the GALC gene on chromosome 14q24.3-q32.1.[83] Assays of amniotic fluid allows prenatal diagnosis, and enzymatic assays on white blood cells or serum may detect the carrier state. A saposin A deficiency is expected to be responsible for some cases of Krabbe's syndrome with near normal GALC activity.
    In the CNS, the white matter is decreased in mass and is gliotic, firm, and rubbery to palpation. In the areas of demyelination, oligodendroglial cells are severely reduced in number. Globoid cells, found deep in the white matter around and within vessels, are distinctive. Their abundant cytoplasm stains positively with periodic acid–Schiff (PAS) and only faintly with Sudan black. No metachromasia is present. The small globoid cells are round and mononuclear, while the large one (varying from 20 to 50 μm) become irregular and multinucleated
    Electron microscopy reveals within the cytoplasm of the cells cytoplasmic inclusions containing both electron-dense linear or curved tubular profiles, a distinctive sign in Krabbe's disease.[84] Neurons in the pons, thalamus, and dentate nuclei show varying degrees of degeneration, but the neuronal processes of the cerebral cortex are reasonably well preserved. Involvement of the peripheral nervous system varies, and segmental demyelination has been reported.[85] Globoid cells are not seen in peripheral nerves, but histiocytes with foamy cytoplasm and tubular inclusions have been demonstrated. Krabbe's disease is distributed worldwide and has no gender, racial, or ethnic proclivities. There are two primary forms of Krabbe's disease, the early-onset or classic form, and the late-onset form. The early-onset type usually appears clinically between the third and eighth months of life, although biochemical and pathological changes are present in the early gestational period. Initially, the patient is irritable and has intermittent fever for no discernible reason. Episodic limb or trunk rigidity may occur, and a heightened startle response to noise, light, or touch may be apparent. Feeding problems and seizures occur frequently. Myoclonic seizures may suggest infantile spasms. Macrocephaly is uncommon, and hepatosplenomegaly is absent. There are usually no cherry-red spots. Early in the course, EEG shows a severe slowing of the background activity and abundant paroxysmal discharges. By 6 months of age, the infant develops severe hypertonus with obvious opisthotonos and decorticate-decerebrate rigidity. Reflexes may be hyperactive or hypoactive if advanced peripheral neuropathy is present. Babinski's signs are usually present along with optic atrophy. By 9 months of age, the baby is usually blind and deaf. In light of these characteristic clinical features, there is very little confusion between the diagnosis of classic Krabbe's disease and other infantile-onset degenerative conditions (Video 105, Myoclonic Seizure).
    The late-onset type of Krabbe's disease is extremely uncommon. These patients first develop difficulties later in infancy, in childhood, or even in adult life.[86] The late infantile form is characterized by the onset between 6 months and 3 years of developmental regression with ataxia and stiffness, feeding difficulties, irritability, and loss of vision. The juvenile or adult form is usually characterized by a progressive amaurosis starting in late childhood, followed years later by a progressive gait impairment with spasticity or dystonia, and finally dementia. Optic atrophy and peripheral neuropathy are consistent findings late in the course of the illness.
    Periventricular hyperdensities demonstrated on CT scans have been reported, particularly in the terminal phases of the classic form. MRI scans demonstrate white matter involvement of the cerebrum and cerebellum,[87] and electrophysiological evidence of peripheral nerve demyelination is prominent. Treatment at this time is limited to allogenic hematopoietic stem cell transplantation that appears to slow the progression of the disease and improve magnetic resonance images. Studies using stem cells and viral vectors to transduce transplantable cells are under way.[88]

    these were the facts about alexander and krabbe's disease but i choose alexander disease as the answer because in alexander disease global demylination is seen and hyperintence ct is seen in several areas while in krabbe a few reported with hypreintense ct and that too most common in terminal phase of classic subtype so i choose alxander as the asnwer.
  20. aayush.

    aayush. Guest

    Q5.Post op radiotherapy is given in all except in pt of ca endometrium-? Ans is c.Enlarged uterine cavity. Ref. Harrison556.This is repeat of may05 q.159 .Indications are-1poor histo grade 2.Deep myometrial invasion 3.Extensive involvement of lower uterine segment 4.Positive pelvic lymph node.
  21. aayush.

    aayush. Guest

    15. Nephelometry is:
    a. Lambert-Beer law
    b. Scattering of light by particulate solution
    c. Defraction of light
    d. Decreased intensity of light

    ref: Textbook of Biochemistry -2/E,Vaudevan&Sreekumari ;pg.26.

    i'm sorry guys i dont have the latest edition,but neverthless this is a standard book and Sreekumari madam is a great teacher who taught me in first yr.About NEPHELOMETRY she says

    -It is based on the measurment of turbidity or cloudiness.
    -Scattering of light by complexes formed by diphtheria toxin was forst used as a quantitaton method by Libby.The measurement is done by addition of consatnt amounts of specific antiserun to serial dilutions of antigens[the protein to be estimated.]The resultant antigen-AB complexes will form TURBIDITY of solution.A beam of light [pref. LASER beam] is passed thru the solution.The PARTICLES in the solution will SCATTER LIGHT.The light turning at 60 degree angle is collected and passed into a detector system.The emergent SCATTERED LIGHT will be PROPORTIONAL to the TURBIDITY OF THE SOLUTION which in turn will be PROPORTIONAL TO THE ANTIGEN.
    This is a very rapid method suitable for automated progrmmes.
    Proteins in biologival fluids like urine and CSF can be estimated by adding protein precipating agents[sulphosalicylic acid] and measuring the turbidity produced.
    RICHARD ZSIGMONDY had studied the scattering of light by colloids and was awarded the Nobel prize in medicine in 1925.

    also know that LAMBERT-BEER LAW the basic principle of COLOURIMETER.
    LAMBERT'S LAW states that"the amt of light absorbed by a coloured substance depends on the length of the column or the depth of the liquid thru which light passes."
    BEER'S LAW states that "the INTENSITY of the COLOR is directly proportional to the concentration of the colored particles in solution."
    So combining these 2 priciples LAMBER-BEER LAW can be expressed as ,
    Ie/Io =e['raised to the power' -kct.]
    Ie=intensity of emergent light
    Io=intensity of incident light.
    k=a constant
    c=concentrataion of cloured substance
    t=thichness or length of layer.
    from this we can equation we can calculate,
    TRANSMITTANCE [T] = ratio of Ie/Io
    OPTICAL DENSITY [OD] = -logT
    Most of the clinical chemistry estimations are done by COLOURIMETRIC METHODS.A coloured derivative of the comound to be measured is prepared and its absorbance or OD is measured using a photoelectric COLOURIMETER.This value is then compared with that of a standard of known concentration.

    so ans shud be scattering of light by particulate soln.
  22. aayush.

    aayush. Guest

    urothelium does not line-
    a. collecting duct
    b. minor calyx
    c. ureter
    d. urinary bladder


    ref: grays Anatomy p.22, p.1828, 1840

    urothelium lines much of urinary tract, extending from ends of collecting ducts of kidney thruough ureters and bladder to proximal portions of urethra.

    in males - extends upto ejaculotory duct.
    in females - extends upto urogenital membrane.
  23. aayush.

    aayush. Guest

    ureteric peristalsis depends on
    a. sympathetic tone
    b. parasympathetic tone
    c. both
    d. intrinsic muscles and pacemaker at minor calysis


    ref: grays Anatomy p. 1833

    contraction waves arising from the upper end of urinary tract are thought to propagate from muscle cell by means of intercellular gap junctions.
    this process is essentially a property of nonstriated muscle and doesnot require the direct involvment of autonomic nerves.
  24. aayush.

    aayush. Guest

    simple columnar epith with brush border is seen in gall bladder.Ref inderbir singh,r.Prasad histo.Also across pn29. Urothelium is not found in collecting duct.It is found in renal pelvis,calyces,UB,Part of urethra,ureter.
  25. aayush.

    aayush. Guest

    Q21.VHL syndrome includes all except-? Ans.A.Endolymphatic sac tumor. Ref Robins1414,harrison2236. It includes capillary hemangioblastoma of cerebellum,retina,brain stem &spinal cord.Cyst in pancreas,liver,kidney,pheochromocytoma,islet cell tumor.Increase of dev.Of RCC. Nowhere endolymphatic sac tumor is written.
  26. aayush.

    aayush. Guest

    septal haematoma interfere with vitality of nasal cartilage hence should be drained {ref..bhargav ENT
  27. adonis.

    adonis. Guest

    Cryoprecipitate contains all of the following except:
    a. Factor VIII
    b. Factor IX
    c. Von Willebrand factor
    d. Fibrinogen

    ref: Harrison's 16/E,pg.664.

    CRYOPRECIPITATE contains,
    -cold insoluble plasma proteins
    -FIBRINOGEN
    -FACTOR 8
    -VWF.
    Each unit of CRYOPRECIPITATE contains approx. 80U of FACTOR-8.
    so ans is CRYOPRECIPITATE does NOT contain factor9.

    also know FFP contains,
    -stable coagulation factors.
    -fibrinogen
    -anti thrombin
    -albumin
    -protein C&S.
    1 unit of FFP[200-250ML] increases the CF's by 2%.
  28. adonis.

    adonis. Guest

    Q21.VHL syndrome includes all except-? Ans.A.Endolymphatic sac tumor. Ref Robins1414,harrison2236. It includes capillary hemangioblastoma of cerebellum,retina,brain stem &spinal cord.Cyst in pancreas,liver,kidney,pheochromocytoma,islet cell tumor.Increase of dev.Of RCC. Nowhere endolymphatic sac tumor is written.


    check schwartz.


    The most serious sequela of VHL involves malignant degeneration of renal cysts. Renal cysts are seldom clinically significant; however, they have an appreciable rate of malignant transformation, and renal cell carcinoma (RCC) is the leading cause of death in patients with VHL disease (35-75% prevalence in one autopsy series). The average age at which patients with VHL develop RCC is 44 years. These facts reinforce the importance of obtaining renal imaging studies on a regular basis.
    The second most common cause of morbidity and mortality in patients with VHL is CNS hemangioblastomas. Approximately 70% of affected individuals develop such tumors at some point, and they typically occur below the tentorium (80% of the time in the cerebellum, 20% of the time in the spine). The mean age at diagnosis is 25 years. While the lesions are rarely malignant, enlargement of the tumors within the confines of the CNS can result in neurologic compromise and death if they are not resected. Retinal hemangioblastomas, while also not malignant, can result in considerable morbidity through retinal detachment or visual loss from an enlarging lesion.
    Via secreted catecholamines, development of pheochromocytomas can result in hypertension and its numerous deleterious sequelae. Pancreatic lesions are simple cysts and rarely cause symptoms or develop into malignant tumors. Endolymphatic sac tumors are seen in approximately 10% of patients and can cause deafness of varying severity. Epididymal tumors are present in approximately 50% of patients with VHL. They are papillary cystadenomas and rarely cause problems. When they occur bilaterally, these cystadenomas may result in infertility. For women, the equivalent lesion is a papillary cystadenoma of the ovarian broad ligament.

    these are lines from e medicine. i know you people may not be satisified with net reference. will give book reference as soon as possible.
  29. adonis.

    adonis. Guest

    Fast axonal transport is by all except:
    a. Dynenin
    b. Kinesin
    c. Microfilaments
    d. Neurofilaments


    answer d. neurofilaments

    refrence neurobiology of neuron second edition davis and morris
  30. adonis.

    adonis. Guest

    Ques from opthal section-Q.113.Transparency of cornea is maintained by all except? Ans is c.Hydration of corneal epitheliun. Ref.Basak24,khurana90-91 yanoff422-24 kanski96. Transparency is d/t relatively dehydrated state maintained by integrity of hydrophobic epithelium,endothelium,endothelmic pump& osmotic gradient because aqueous &tear are relatively hypertonic.
  31. adonis.

    adonis. Guest

    Q.177corneal endothelium ion exchange pumps are inhibited by? Ans.A.Inhibition of anaerobic glycolysis. Ref. Basak25. 60% metabolism in cornea in endothelium occurs by anaerobic glycolysis.
  32. adonis.

    adonis. Guest

    Not a constant feature of TOF:
    a)Rt. Ventricular hpertrophy b)Valvular PS
    c)Over riding aorta d)Infundibular stenosis

    Answer (b)
    Robbins 7th p-568,569.

    four features of TOF are:
    1)VSD 2)RVOT( subpulmonic stenosis ) 3)an aorta that overrides VSD 4)RV hypertrophy.

    RVOT is most often due to narrowing of the infundibulum (subpulmonic stenosis) but is often accompanied by Pul. valve stenosis.
  33. adonis.

    adonis. Guest

    treatment for Cong. diaphragmatic hernia from any surgery or paeds surgery text book as even latest paper of Mudit Khanna tells that immediate surgery is required.

    Here's what sabistan textbook of surgery has to say abt the treatment of cong. diaphragmatic hernia...

    Treatment can be conveniently divided into three main areas: (1) stabilization and preoperative preparation, (2) operative treatment, and (3) postoperative respiratory, circulatory, metabolic, and nutritional support. Infants with diaphragmatic hernia should have direct endotracheal intubation and ventilatory support with high oxygen flow (FIO2 of 1.0) on a conventional infant ventilator, with excessive ventilatory pressure avoided. An orogastric tube should be inserted immediately, and all air should be aspirated from the stomach.
  34. adonis.

    adonis. Guest

    All of the following occur when the blood flows through the systemic capillaries except:
    a. Increase in hematocrit
    b. Hb curve shifts to the left
    c. Increased protein content
    d. Decrease in pH

    answer b. hb curve shifts to left
    guyton physiiology 11th edition page 503-508


    blood into the tissues—so rapidly that the capillary Po2
    falls almost to equal the 40 mm Hg pressure in the
    interstitium. Therefore, the Po2 of the blood leaving
    the tissue capillaries and entering the systemic veins is also about 40 mm Hg






    Increased Delivery of Oxygen to the Tissues When Carbon
    Dioxide and Hydrogen Ions Shift the Oxygen-Hemoglobin Dissociation
    Curve—The Bohr Effect. A shift of the oxygenhemoglobin
    dissociation curve to the right in response
    to increases in blood carbon dioxide and hydrogen
    ions has a significant effect by enhancing the release
    of oxygen from the blood in the tissues and enhancing
    oxygenation of the blood in the lungs.This is called the
    Bohr effect, which can be explained as follows: As the
    blood passes through the tissues, carbon dioxide diffuses
    from the tissue cells into the blood.This increases
    the blood Po2, which in turn raises the blood H2CO3
    (carbonic acid) and the hydrogen ion concentration.
    These effects shift the oxygen-hemoglobin dissociation
    curve to the right and downward, as shown in
    Figure 40–10, forcing oxygen away from the hemoglobin
    and therefore delivering increased amounts of
    oxygen to the tissues.Exactly the opposite effects occur in the lungs,
    where carbon dioxide diffuses from the blood into
    the alveoli.This reduces the blood Pco2 and decreases
    the hydrogen ion concentration, shifting the oxygen-hemoglobin dissociation curve to the left and
    upward. Therefore, the quantity of oxygen that binds
    with the hemoglobin at any given alveolar Po2
    becomes considerably increased, thus allowing greater
    oxygen transport to the tissues.
  35. anjali.

    anjali. Guest

    newborn female child, weight 3.5kg, delivered by uncomplica

    A newborn female child, weight 3.5kg, delivered by uncomplicated delivery, developed respiratory distress immediately after birth. On chest x-ray ground glass appearance was seen. Baby put on mechanical ventilation and was give surfactant but condition of baby deteriorates and increasing hypoxemia was present. A full term female ‘sibling’ died within a week with the same complaints. . Usual cultures are negative. Your diagnosis is:
    a. Total anomalous pulmonary vein connection
    b. Meconium aspiration syndrome
    c. Neonatal pulmonary alveolar proteinosis
    d. Disseminated HSV infection
  36. anjali.

    anjali. Guest

    Senile cardiac amyloidosis is due to defect in:
    a. β2 – microglobulin
    b. Transthyretin
    c. AANF
    d. Pyrin
    Answer b. transthyretin

    Harrison 16th edition page 2026

    With respect to localized amyloidosis, cardiac
    amyloidosis of the wild type or nonvariant TTR type is common
    after 80 years of age;
    there was some confusion due to Harrison table but above line I think will clear the doubts that after 80 years(senile) ttr type is common
  37. aaayush.

    aaayush. Guest

    Psychodynamic theory of mental illness is based on:
    a. Unconscious conflict
    b. Maladjusted reinforcement
    c. Organic neurological problem
    d. ?


    ahnswer a.unconscious conflict
  38. aaayush.

    aaayush. Guest

    Psychodynamic theory of mental illness is based on:
    a. Unconscious conflict
    b. Maladjusted reinforcement
    c. Organic neurological problem
    d. ..


    ahnswer a.unconscious conflict








    Refrence new oxford text book of Psychiatry 2003 edition

    Psychodynamic Psychiatry is broadly defined today. In fact, the term psychodynamic is now used almost synonymously with psychoanalytical. Freud originally used the term psychodynamic to emphasize the conflict between opposing intrapsychic forces: a wish was opposed by a defence, and different intrapsychic agencies, such as ego, id, and superego, were in conflict with one another. Indeed, for much of the twentieth century psychoanalytical theory was dominated by the drive-defence model, often referred to as ego psychology.
    In the last decades of the twentieth century, however, the hegemony of ego psychology waned, and other models of the mind gained wide acceptance. Through the influence of Melanie Klein and the British School of object relations, psychoanalytical theory expanded beyond the notion of conflict among intrapsychic agencies. Internal object relations became paramount in models deriving from these sources. In addition, a deficit model of symptomatology arose from the work of the British object-relation theorists, such as Balint and Winnicott. In the United States, Kohut's self-psychology also developed a model based on developmental deficits. In other words, disturbed patients who came to treatment were seen as suffering from absent or weakened psychic structures based on developmental failures by parents or caretakers in the early childhood environment.
    As a result of these innovations in psychoanalytical theory, psychodynamic Psychiatry is practised today in an era of pluralism. The typical psychodynamic psychiatrist then uses multiple models to assist in the understanding of a particular patient. Moreover, the diagnostic and treatment approach to an individual patient is psychodynamically informed even when a decision has been made to forego psychodynamic psychotherapy. Psychodynamic thinking provides a conceptual framework within which all treatments are prescribed, including pharmacotherapy, psychotherapy, inpatient or partial hospital treatment, and group or family modalities. Psychodynamic Psychiatry is not synonymous with psychodynamic psychotherapy.
    A comprehensive definition of current psychodynamic Psychiatry is the following1)
    Psychodynamic Psychiatry is an approach to diagnosis and treatment characterized by a way of thinking about both patient and clinician that includes unconscious conflict, deficits and distortions of intrapsychic structures, and internal object relations.In this definition emphasis is placed on the presence of two individual psychologies or subjectivities that interact in the field of treatment. Clinicians must recognize that their own unconscious beliefs, biases, and feelings will inevitably influence the way they view the patient
  39. aaayush.

    aaayush. Guest

    Corneal endothelium ion exchange pumps are inhibited by:
    a. Inhibition of anaerobic glycolysis
    b. Activation of anaerobic glycolysis
    c. Activation of cAMP phosphodiesterase inhibitors
    d. Interference with electron chain transport


    answer b.activation of anerobic glycolysis

    dunae's foundation of clinical opthalmology 2005 edition volume 2 chapter 4 Physiology of cornea and sclrea

    The endothelium utilizes the same carbohydrate metabolism pathways as the epithelium. The transport function of the cells in the endothelial monolayer requires oxidative activity that is five to six times that of the cells in the epithelium.85 Atmospheric oxygen is the primary source of oxygen to the endothelium. Interruption of this oxygen supply by low oxygen transmissibility contact lenses or a low oxygen environment will result in a shift to anaerobic metabolism, a concurrent increase in stromal lactic acid and CO2, and a drop in stromal pH.77 In addition, this hypoxia can stimulate epithelial production of 12(R)HETE, a potent inhibitor of the endothelial Na+ /K+ ATPase


    so it is hypoxia and subsequent activation of anerobic metablism inhibit corneal tranport function.

    so answer is activation of anerobic glycolysis
  40. aaayush.

    aaayush. Guest

    Cryoprecipitate contains all of the following except:
    a. Factor VIII
    b. Factor IX
    c. Von Willebrand factor
    d. Fibrinogen

    ref: Harrison's 16/E,pg.664.

    CRYOPRECIPITATE contains,
    -cold insoluble plasma proteins
    -FIBRINOGEN
    -FACTOR 8
    -VWF.
    Each unit of CRYOPRECIPITATE contains approx. 80U of FACTOR-8.
    so ans is CRYOPRECIPITATE does NOT contain factor9.

    also know FFP contains,
    -stable coagulation factors.
    -fibrinogen
    -anti thrombin
    -albumin
    -protein C&S.
    1 unit of FFP[200-250ML] increases the CF's by 2%.
  41. ikon

    ikon Guest

    Regarding genital development, true is:
    a. Y chromosome is associated with ovary development
    b. Genital ridge starts developing at 5th week
    c. Male genitals develop earlier than female genitals
    d. Genital development is complete by 10th week
  42. aayush.

    aayush. Guest

    Violent inversion of the foot will lead to avulsion of tendon of the following muscle attched to the tuberosity of the 5th metatarsal:
    a. Peroneus brevis
    b. Peroneus longus
    c. Peroneus tertius
    d. Extensor digitorum brevis

    answer a peroneus brevis

    Snell clinical Anatomy 7th edition page 648
    Fifthe metatarsal has prominent tubercle on its base that can be easily palpated along the lateral border of the foot.The tubecle gives attachment to the peroneus brevis tendon

    The base of the fifth metatarsal bone can be fractured during forced inversion of the foot.at which time the tendon of insertion of the peroneus brevis muscle pulls off the base of the metatarsal.



    According to the new WHO criteria, all are true in a normal person except:
    a. Sperm count > 20 million
    b. Volume > 1 ml
    c. Normal morphology in > 15 % (strict criteria)
    d. Aggressive forward motility in > 25 %


    Answer b volume > 1 ml

    Campbell walsh urology 9th edition
    The reference ranges used to interpret the semen analysis are more accurately defined as minimal levels of adequacy. The finding of parameters below these levels is suggestive of infertility, whereas the finding of parameters above these levels is suggestive of fertility. There are clearly fertile patients with semen parameters below these levels and infertile patients with parameters above these levels. The World Health Organization defines the following reference values ( WHO, 1999 ):
    Volume: 2.0 mL or more
    pH: 7.2 or more
    Sperm concentration: 20 × 106 or more spermatozoa/mL
    Total sperm number: 40 × 106 or more spermatozoa per ejaculate
    Motility: 50% or more with grade “a + b†motility or 25% or more with grade “a†motility
    Morphology: 15% or more by strict criteria
    Viability: 75% or more of sperm viable
    WBCs: Less than 1 million/mL
    Motility is the percent of sperm that demonstrate flagellar motion. The evaluation is ideally performed within 1 to 2 hours of ejaculation and the specimen kept at room or body temperature to avoid a decrease in sperm motility. An assessment of the quality of forward movement of the sperm should be noted. One commonly used method rates the sperm movement on a five-point scale. A rating of zero signifies no motility; 1 denotes sluggish or nonprogressive movement; 2 refers to sperm moving with a slow, meandering forward progression; 3 signifies sperm moving in a reasonably straight line with moderate speed; and 4 indicates sperm moving in a straight line with high speed ( Amelar et al, 1973 ). The most common category of sperm movement is reported. An alternate system places the sperm into four categories. “A†signifies rapid progressive motility; “B,†slow or sluggish progressive motility; “C,†nonprogressive motility; and “D,†no motility. In this system, the percent of sperm falling into each category is reported
  43. aayush.

    aayush. Guest

    newborn female child, weight 3.5kg, delivered by uncomplicated delivery, developed respiratory distress immediately after birth. On chest x-ray ground glass appearance was seen. Baby put on mechanical ventilation and was give surfactant but condition of baby deteriorates and increasing hypoxemia was present. A full term female ‘sibling’ died within a week with the same complaints. ECHO is normal. Usual cultures are negative. Your diagnosis is:
    a. Total anomalous pulmonary vein connection
    b. Meconium aspiration syndrome
    c. Neonatal pulmonary alveolar proteinosis
    d. Disseminated HSV infection


    answer neonatal pulmonary alveolar proteinosis




    refrence nelson text book of pediatrics page 1453-1454


    Pulmonary alveolar proteinosis (PAP) is a disorder characterized by the intra-alveolar accumulation of pulmonary surfactant. On histopathologic examination, distal air spaces are filled with a granular, eosinophilic material that stains positively with periodic acid–Schiff reagent and is diastase resistant. Two clinically distinct forms of PAP have been described in children: a fulminant, usually fatal form presenting shortly after birth (termed congenital PAP) and a gradually progressive type presenting in older infants and children and similar to that observed in adults.
    Etiology and Pathophysiology.
    Although the mechanisms that lead to alveolar proteinosis are undefined, histologic findings suggest that they result in a disruption of pulmonary surfactant metabolism. The early onset of the neonatal form of PAP along with a positive family history of similarly affected newborn infants strongly suggests a genetic basis. For example, an inherited deficiency in surfactant protein-B (SP-B) has been described with some cases of neonatal alveolar proteinosis(see chapter 397)

    so neonatal pulmonary alveolar protinosis is due to surfactant protein b deficeincy.and they have described it seprately in chapter 397. now i am posting entire topic of chap 397 of nelson.because it is important



    Chapter 397 - Inherited Disorders of Surfactant Protein Metabolism

    Aaron Hamvas
    Lawrence M. Nogee
    F. Sessions Cole

    The pulmonary surfactant is a mixture of phospholipids and proteins synthesized, packaged, and secreted by type II pneumocytes that line the distal airways. This mixture forms a monolayer at the air-liquid interface that lowers surface tension at end-expiration of the respiratory cycle and thereby prevents atelectasis and ventilation-perfusion mismatch. Four surfactant-associated proteins have been described: surfactant proteins A and D (SP-A, SP-D) participate in host defense in the lung, whereas surfactant proteins B and C (SP-B, SP-C) contribute to the surface tension–lowering activity of the pulmonary surfactant. Two genes for SP-A and one gene for SP-D are located on human chromosome 10, whereas single genes located on human chromosomes 2 and 8, respectively, encode SP-B and SP-C. Although inherited deficiencies of SP-A or SP-D have not been identified in humans, genetically engineered mice deficient in these proteins are susceptible to viral and bacterial infections and lineages deficient in SP-D accumulate lipids and foamy macrophages in their lungs and develop emphysema and pulmonary fibrosis as they age. Inherited disorders of SP-B and SP-C have been identified in humans and are discussed in detail.

    DEFICIENCY OF SURFACTANT PROTEIN B
    Genetics.
    Over 20 loss-of-function mutations in the SP-B gene have been identified. The most common is a net 2 base-pair insertion in codon 121 (termed 121ins2) that generates a frameshift and interruption of SP-B protein translation. The insertion generates a restriction fragment polymorphism (RFLP) that is useful for diagnosis. This mutation accounts for 60–70% of the alleles found to date in patients identified with SP-B deficiency. Most other mutations have been family specific.
    Pathology .
    Although SP-B deficiency was first described in a patient with newborn-onset alveolar proteinosis, the histology is neither specific for SP-B deficiency nor universally present in lungs of affected infants. Several SP-B–deficient patients homozygous for the 121ins2 mutation have had histologic features more typical of desquamative interstitial pneumonitis with little detectable alveolar proteinosis at the time of lung transplantation. Other findings are nonspecific and include variable degrees of interstitial fibrosis and alveolar cell hyperplasia. Ultrastructural findings include a lack of tubular myelin, disorganized lamellar bodies, and an accumulation of abnormal-appearing multivesicular bodies, suggesting abnormal lipid packaging and secretion.

    Clinical Manifestations.


    Infants with an inherited deficiency of SP-B present in the immediate neonatal period with respiratory failure. This autosomal recessive disorder is clinically and radiographically similar to the respiratory distress syndrome of premature infants (see Chapter 90.3 ) but typically affects full-term infants and is refractory to mechanical ventilation, surfactant replacement therapy, glucocorticoid administration, and extracorporeal membrane oxygenation. SP-B deficiency has been recognized in diverse racial and ethnic groups. Almost all patients have died without lung transplantation, but prolonged survival is possible in cases with a partial deficiency of SP-B. Patients heterozygous for loss-of-function mutations in the SP-B gene are
    clinically normal as adults and have normal pulmonary function.
    Diagnosis.



    A rapid, definitive diagnosis can be established with analyses of DNA for known mutations in the SP-B gene, particularly the 121ins2 mutation (using RFLP analysis of polymerase chain reaction–amplified genomic DNA). The sensitivity of genetic diagnosis is limited, however, because assays are not readily available for all known mutations and disease may result from yet unidentified mutations. In families in which a mutation has been previously identified, antenatal diagnosis can be established by molecular assays of DNA from chorionic villus biopsy or amniocytes, which permits advanced planning of a therapeutic regimen. Other laboratory tests remain investigational and include analysis of tracheal effluent by enzyme-linked immunosorbent assay or Western blotting for the presence or absence of SP-B protein and for aberrantly processed precursor proSP-C peptides that have been found in SP-B–deficient human infants and animals. Definitive diagnosis can also be established by immunostaining of lung biopsy tissue for the surfactant proteins. The absence of SP-B protein and abundant expression of the aberrantly processed proSP-C peptide definitively establish a diagnosis of SP-B deficiency. Such studies require a lung biopsy in a critically ill child or may be performed on lung blocks acquired at the time of autopsy. Immunohistochemical assays for SP-B and SP-C are available on a research basis.


    Treatment.

    Because virtually all patients with SP-B deficiency die within the first year of life, prompt recognition is critical. Conventional neonatal intensive care interventions can maintain extrapulmonary organ function for a limited time (weeks to months). Replacement therapy with commercially available surfactants is ineffective. Lung transplantation has been successful, but the pre-transplant, transplant, and post-transplant medical and surgical care is highly specialized and only available at pediatric pulmonary transplant centers. The oldest living SP-B–deficient survivors after lung transplantation were born in 1994. The relative scarcity of available infants' lungs for transplantation suggests that gene therapy may be the treatment of choice in the future. Genetic counseling is also important to convey the risks for future pregnancies and the availability of antenatal diagnosis and therapeutic options. Palliative care consultation is also helpful.
  44. aayush.

    aayush. Guest

    18 year old male presents with pectus excavatum. He denies history of any dyspnoea or chest pain. On examination there is mild pectus excavatum and intermittent wheezing on exertion. Surgery in this patient is indicated if he has:
    a. FEV1 / FVC less than 0.60
    b. Limiation of maximum inspiration during exercise
    c. Peak work capacity 60% of expected
    d. Functional work capacity 80% of expected


    answer c.peak work capacity 60% of expected



    nelson 17th edition page 1467 chapter 409.1

    Pectus Excavatum (Funnel Chest)
    Pathogenesis.
    Midline narrowing of the thoracic cavity is usually an isolated skeletal abnormality. The cause is unknown. It may be associated with a connective tissue disorder (i.e., Marfan syndrome) or rickets, or it may be acquired.

    Clinical Manifestations.

    The deformity is present at or shortly after birth but is usually not associated with any symptoms at that time. Over time, decreased exercise tolerance, chest pain, palpitations, recurrent respiratory infections, wheezing, stridor, and cough may be present. Because of the cosmetic nature of this deformity, many children experience significant psychological stress. Physical examination may reveal a narrowed anteroposterior diameter, rounded shoulders, kyphoscoliosis, protuberant abdomen, left shift of the cardiac impulse, and an innocent systolic murmur.

    Diagnosis.

    Lateral chest radiograms demonstrate the depression. An electrocardiogram may show a right-axis deviation. Mitral valve prolapse, Wolff-Parkinson-White syndrome, bronchial atresia, and bronchomalacia are associated. Results of pulmonary function tests may be normal or may show a restrictive defect, if the pectus deformity is severe. Exercise testing may demonstrate either normal tolerance or limitations from underlying limited habitual activity or cardiopulmonary dysfunction. Lowered ventilatory reserves at peak exercise are common, although the clinical significance of these findings remains unclear.

    Treatment.
    Corrective surgery is beneficial for individuals with restrictive lung disease. Although surgery itself does not reverse the lung restriction, it may halt the progression of cardiopulmonary compromise. For teenagers with exercise limitations, surgical repair may result in improved exercise tolerance. Normalization of lung perfusion scans and maximal voluntary ventilation have also been seen after surgery. Surgery is often performed for psychological or cosmetic reasons.


    now ou can see that they have given exercise intolrence that means peak exercise capacity and not functional.

    they have restrictive lung disease so a. is ruled out.

    now las two lines creates some confuion regarding b but maxiumum inspiration does not mean voluntary(during exrcise we do not voluntary increase our ventialtion) and other thing is that it is stated no improvement in lung function is seen after surgery.

    i have looked into thoracic surgery,pediatric surgery books but could not find better refrence than this.

    so my bet is answer is c.
  45. aayush.

    aayush. Guest

    Violent inversion of the foot will lead to avulsion of tendon of the following muscle attched to the tuberosity of the 5th metatarsal:
    a. Peroneus brevis
    b. Peroneus longus
    c. Peroneus tertius
    d. Extensor digitorum brevis

    answer a peroneus brevis

    Snell clinical Anatomy 7th edition page 648
    Fifthe metatarsal has prominent tubercle on its base that can be easily palpated along the lateral border of the foot.The tubecle gives attachment to the peroneus brevis tendon

    The base of the fifth metatarsal bone can be fractured during forced inversion of the foot.at which time the tendon of insertion of the peroneus brevis muscle pulls off the base of the metatarsal.



    According to the new WHO criteria, all are true in a normal person except:
    a. Sperm count > 20 million
    b. Volume > 1 ml
    c. Normal morphology in > 15 % (strict criteria)
    d. Aggressive forward motility in > 25 %


    Answer b volume > 1 ml

    Campbell walsh urology 9th edition
    The reference ranges used to interpret the semen analysis are more accurately defined as minimal levels of adequacy. The finding of parameters below these levels is suggestive of infertility, whereas the finding of parameters above these levels is suggestive of fertility. There are clearly fertile patients with semen parameters below these levels and infertile patients with parameters above these levels. The World Health Organization defines the following reference values ( WHO, 1999 ):
    Volume: 2.0 mL or more
    pH: 7.2 or more
    Sperm concentration: 20 × 106 or more spermatozoa/mL
    Total sperm number: 40 × 106 or more spermatozoa per ejaculate
    Motility: 50% or more with grade “a + b†motility or 25% or more with grade “a†motility
    Morphology: 15% or more by strict criteria
    Viability: 75% or more of sperm viable
    WBCs: Less than 1 million/mL
    Motility is the percent of sperm that demonstrate flagellar motion. The evaluation is ideally performed within 1 to 2 hours of ejaculation and the specimen kept at room or body temperature to avoid a decrease in sperm motility. An assessment of the quality of forward movement of the sperm should be noted. One commonly used method rates the sperm movement on a five-point scale. A rating of zero signifies no motility; 1 denotes sluggish or nonprogressive movement; 2 refers to sperm moving with a slow, meandering forward progression; 3 signifies sperm moving in a reasonably straight line with moderate speed; and 4 indicates sperm moving in a straight line with high speed ( Amelar et al, 1973 ). The most common category of sperm movement is reported. An alternate system places the sperm into four categories. “A†signifies rapid progressive motility; “B,†slow or sluggish progressive motility; “C,†nonprogressive motility; and “D,†no motility. In this system, the percent of sperm falling into each category is reported
  46. aayush.

    aayush. Guest

    In post-ductal coarctation of the aorta, blood flow to the lower limbs in maintained by increased blood flow through:
    a. Inferior Phrenic and pericardiophrenic vessels
    b. Intercostal and Superior epigastric
    c. Subcostal and Umbilical
    d. …


    answer c. intercostal and superior epigastric

    refrence gray Anatomy

    The postductal type of coarctation has been attributed to abnormal extension of the ductal tissue into the aortic wall, stenosing both vessels as the duct contracts after birth. This form can permit many years of normal life, allowing the development of an extensive collateral circulation to the aorta distal to the stenosis. High vascularity of the thoracic wall is important and clinically characteristic; many arteries arising indirectly from the aorta, proximal to the coarctation segment, anastomose with vessels connected with it distal to the block; these become greatly enlarged. In the anterior thoracic wall the thoraco-acromial, lateral thoracic and subscapular arteries from the axillary, the suprascapular from the subclavian and the first and second posterior intercostal arteries from the costocervical trunk anastomose with other posterior intercostal arteries; the internal thoracic artery and its terminal branches anastomose with the lower posterior intercostal and inferior epigastric arteries. Posterior intercostal arteries are always involved, and enlargement of their dorsal branches may eventually groove ('notch') the inferior margins of the ribs. The radiograph shadow of the enlarged left subclavian artery is also increased. Enlargement of the scapular vessels and anastomoses may lead to widespread interscapular pulsation (easily appreciated with the palm of the hand, and sometimes heard on auscultation).


    so it says internal thoracic and its terminal branches anastomoise with posterior intercosal and inferior epigastric.

    now terminal branch of internal thorcic artery is nothing but the superior epigastric artery.

    so we are clear that supiroir epigastric and intercosal take part in anastomosis.now we have to see that this anastomosis supply lower extremey or not.(question is about collateral for leg) forthat see folloing lines and figure from gran Anatomy atlas

    grant atlas of Anatomy says

    Epigastric Anastomoses
    The superior epigastric vessels anastomose with the inferior
    epigastric vessels within the rectus sheath (Fig. 4.14). If the inferior
    vena cava becomes obstructed, the anastomosis between
    the inferior epigastric and superior epigastric veins provides a
    collateral venous channel that drains into the superior vena
    cava. If the aorta is occluded, collateral arterial circulation to
    the lower part of the body occurs through the superior and inferior
    epigastric arteries

  47. alam.

    alam. Guest

    145. Regarding type A personality, false is:
    a. Hostility
    b. Time pressure
    c. Competitiveness
    d. Mood fluctuations

    Answer d. mood fluctuations

    Neeraj ahuja 5th edition page 155

    Type a personality includes time urgency,excessive competitiveness and hostility
  48. aayush.

    aayush. Guest

    130. Which muscle is not punctured while doing a thoracic procedure in the mid-axillary line:
    a. Innermost intercostals
    b. Transverses thoracis
    c. External intercostals
    d. Internal intercostals

    Answer b.transverces thoracis


    refrence gray 38th edition. just read Anatomy from gray and you will understand why this is the answer.

    it never reach upto mid-axillary line








    transversus Thoracis
    Transversus thoracis (also called triangularis sternae and sternocostalis) spreads over the internal surface of the anterior thoracic wall (7.73). It arises from the lower third of the posterior surface of the sternum, the xiphoid process, and the costal cartilages of the lower three or four true ribs near their sternal ends. Its fibres diverge and ascend laterally as slips which pass into the lower borders and inner surfaces of the costal cartilages of the second, third, fourth, fifth and sixth ribs. The lowest fibres of this muscle are horizontal, and are contiguous with the highest fibres of transversus abdominis; the intermediate fibres are oblique; the highest are almost vertical. This muscle varies in its attachments, not only between individuals but even on opposite sides of the same individual. Like intercostales intimi and subcostales, transversus thoracis separates the intercostal nerves from the pleura.
    Actions
    The muscle draws down the costal cartilages to which it is attached.
    Nerve Supply
    All the above muscles are supplied by the adjacent intercostal nerves.
  49. aayush.

    aayush. Guest

    Basement membrane consists of all except:
    a. Laminin
    b. Nidogenin
    c. Entactin
    d. Rhodopsin

    Answer d. rhodopson

    Refrence rubin’s Pathology 5th edition

    Basement Membranes
    Basement membranes, also called basal lamina, are thin, well-defined layers of specialized extracellular matrix that separate the cells that synthesize it from connective tissue (Fig. 3-2). By light microscopy, a basement membrane appears as a thin lamina that is stained by the periodic acid-Schiff stain (PAS). Epithelium, adipocytes, muscle cells, Schwann cells, and capillary endothelium produce basement membranes.
    • Basement membranes are constructed from extracellular matrix molecules, including collagen IV, laminin, entactin/

    nidogen, and perlecan, a heparan sulfate proteoglycan (Table 3-1). They self-assemble into a sandwich-like structure composed of two interacting networks.

    • Within different tissues and during development, the expression of unique members of the collagen IV and laminin families imparts diversity to the basement membrane and the many structures and functions it supports.
    • Basement membranes act as filters, cellular anchors, and a surface for migrating epidermal cells after injury. They also serve to reestablish the neuromuscular junction after nerve damage. Basement membranes also determine cell shape, contribute to developmental morphogenesis, and, notably, provide a repository for growth factors and chemotactic peptides
  50. aayush.

    aayush. Guest

    105. Peroxidase is used to detect:
    a. Glucose
    b. Ammonia
    c. Hemoglobin
    d. Creatinine

    Henry’s clinical diagnosis and management by laboratory methods 21st edition

    Glucose Measurement Methods
    Most measurements of glucose employ enzymatic methods. These enzymatic methods provide specificity and can be packaged to furnish point of care determinations. Three enzyme systems are currently used to measure glucose: glucose dehydrogenase, glucose oxidase and hexokinase. These reactions produce an electrical current that is proportional to the initial glucose concentration or a product that measured spectrophotometrically is proportional to the initial glucose concentration. The assays can be initial rate of change assays, where the velocity of the reaction is dependent on the initial glucose, or end-point assays.
    When glucose is measured using a glucose dehydrogenase method, glucose is reduced to produce either a chromophore that is measured spectrophotometrically ( Equation 16-1 ) or by an electrical current (equation 16-2)

    Glucose oxidase, a flavoenzyme, catalyzes the reactions shown below. The peroxidase reaction can be measured spectrophotometrically and can be inhibited by high concentrations of uric acid, ascorbic acid, bilirubin, glutathione, creatinine, l-cysteine, l-dopa, dopamine, methyldopa and citric acid ( Zaloga, 1997 ). In addition, the glucose oxidase reaction can be coupled to ferricyanide/ferricyanide couple to produce an electrical current, as shown below ( Equation 16-4 ).
    This system is dependent on the partial pressure of O2 since oxygen will compete in the reaction to form hydrogen peroxide, so that the higher the partial pressure of O2the lower the electrically measured glucose ( Kurahashi, 1997 ). Glucose oxidase can also be used in another electrical system, shown below in Equation 16-5 .
    Equation 16.1
    Alpha-d glucose (muta rotase) beta d glucose
    Beta d glucose + nad (glucose dehydrogenase) d-glucolactone +nadh
    Mtt+nadh mtth(blue colour)+nad


    Equation 16.2
    Glucose+pyroliqinoline quinine(pqq) (glucose dehydrogenase) glucolactone+pqqh2
    Pqqh2+2[fe(cn)6]3- pqq+2{fe(cn)6}4-+2h+
    2{fe(cn)6}4-2[fe(cn)6]3-+2e-


    Equation 16.3
    Beta d glucose+o2(glucose oxidase) d glucolactone +h202
    Glucolactone+h2ogluconic acid
    H202+chromogenic oxygen acceptor( o r t h o t o l u d i n e diansidine,4-aminophenazone,orthotoludine) peroxidasecolour chromogen+h2o

    Equation 16.4
    Beta d glucose+2[fe(cn)6]3-+h20(glucose oxidase) d gluconic acid+2{fe(cn)6}4-+2h+
    2{fe(cn)6}4-2[fe(cn)6]3-+2e-
    Equation 16.5
    Beta d glucose+02 (glucose oxidase) d glucolactone+h202
    H202 2h+o2+2e-
    Equation 16.6
    Glucoase+mgatp (hexokinase)glucaose 6 phosphate+mgadp
    G6p + na(P+) (glucose 6 phosphate dehydrogenase) 6 phosphoglucolactone+nadph+h+


    The hexokinase system assay is the generally accepted reference method for measuring glucose. The reaction is shown below ( Equation 16-6 ). The glucose concentration is proportional to the rate of production of NAD(P)H, which is followed spectrophotometrically. Depending on the source of the glucose-6-phosphate dehydrogenase, the enzyme can require specificity for NADP or from some sources it can use NAD as well. Hemolyzed samples can be problematic since the contents released from the erythrocytes may interfere with the stoichiometric relationship between glucose and NAD(P)H accumulation.

    Whole blood glucose specimens can be analyzed with point of care devices. These monitoring devices are used in the home, in the physician's office, or at the bedside in the hospital to monitor for hypoglycemia and hyperglycemia. Most of these devices have been calibrated to give results similar to plasma levels and can report either plasma or whole blood readings. Whole blood tends to give approximately 10-15% lower glucose readings than plasma, but the percentage varies based on hematocrit, analysis technique and sample timing (fasting versus post-glucose load). Capillary blood is the source for most of these whole blood glucose measuring devices. Capillary blood glucose is similar to arterial glucose, but can vary markedly from venous samples, depending upon timing relative to food ingestion. For example, a postprandial specimen is higher in the capillary sample than in the venous sample. Capillary glucose tests, using point of care devices, should not be used to diagnose diabetes or hypoglycemic disorders. To establish these diagnoses, confirmation with laboratory measurements of plasma glucose is essential because of their greater accuracy.
    Home blood glucose monitoring devices help people with diabetes better self-manage their disease. A wide variety of devices are available for home measurements. Proper training of patients in the use of individual meters is critical in avoiding operator errors, which, in one study, were reported in 12% of users ( Schrot, 1999 ). Errors that may contribute to inaccurate readings in certain devices include the application of an insufficient volume of blood, milking the finger to acquire sufficient blood, the use of outdated test strips, environmental factors (humidity, heat, altitude), the use of a malfunctioning meter, the use of a dirty meter, hypertriglyceridemia, hypotension, and measurements outside of the hematocrit or temperature range. Some blood glucose monitoring devices are influenced by high levels of salicylate, acetaminophen, levodopa, uric acid, bilirubin, lipids or low oxygen levels, and others are altered by touching the reaction area. Most are inaccurate at very high and low glucose values.

    Desirable features of home blood glucose monitoring devices, aside from performance characteristics (precision and accuracy) include the following: ease of use, requirement of a small volume of blood, low maintenance, large print readout, rapid testing, appropriate alarms, minimal interfering substances, and memory storage and download capabilities. Some meters now permit alternative site testing (such as forearm, upper arm, thigh), but results from alternative sites may be less accurate when there are rapid changes in glucose levels. Others incorporate electronic logbooks for recording events, insulin doses and carbohydrate intake. This information can be downloaded to a computer to present the data in several displays such as logbook, graphs, charts, and summary statistics.

    Interstitial glucose measuring devices have been developed for monitoring glucose levels in people with diabetes. Most of these devices use electrochemical methods to automatically and frequently measure glucose levels in the interstitial fluid of dermis or subcutaneous fat tissue, and require repeated calibration to plasma or whole blood glucose levels. Examinations of these data provide information about glucose patterns over hours to days. This glucose ‘trend analyses' can reveal useful findings for modifying treatment, such as unsuspected nocturnal hypoglycemia or postprandial hyperglycemia ( Kaufman, 2002 ).
    Interstitial glucose is in slow (5-30 min) equilibrium with capillary blood glucose, and therefore not equal to blood glucose except in stable systems ( Zierler, 1999 ; Cheyne, 2002 ). Particularly during times when glucose levels are rapidly changing, such as after meal ingestion or recovery from hypoglycemia, interstitial fluid readings will lag behind fingerstick glucose levels. Because the precision and accuracy of the currently available portable continuous glucose monitors are not as high as for the conventional home blood

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