Clinical Associate Professor, Department of Pharmacy Practice and Administration, School of Pharmacy, University of Missouri–Kansas City, Kansas City, Missouri
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Perform a focused physical examination of the patient with a large pupil which reacts slowly to light and accommodation (Holmes-Adie Syndrome). Perform a focused physical examination for the causes of unilateral or bilateral ptosis. Perform a focused physical examination for posterior inferior cerebellar artery thrombosis. Take a directed history and perform a focused physical examination for lateral medullary syndrome. Take a directed history and perform a focused physical examination to distinguish between pseudobalbar and bulbar palsy. Perform a focused physical examination to distinguish between total spinal cord transaction, or incomplete cord compression. Perform a focused physical examination for the causes and site of spinal cord compression 33. Perform a focused physical examination to determine the nerve roots involved in spinal cord disease (sensory dermatomes). Perform a focused physical examination for hemisection of the spinal cord (Brown-Sequard syndrome). Perform a focused physical examination to establish the neurological cause of wasting of the small muscles of the hand. Perform a focused physical examination for Charcot-Marie-Tooth disease (features of hereditary motor and sensory neuropathy). Take a directed history and perform a focused physical examination for myotonia dystrophia. Take a directed history and perform a focused physical examination for limb girdle dystrophy 60. Perform a focused physical examination to determine the location of lesions causing sensory loss. Perform a directed physical examination of the pulmonary system for tracheal deviation 3. Take a focused history and perform a directed physical examination for chronic bronchitis. Perform a directed physical examination of the pulmonary system in the patient with suspected mediastinal compression. Take a directed history and perform a focused physical examination for pulmonary hypertension. Take a directed history and perform a focused physical examination for fibrosing alveolitis. Take a directed history for the common side effects of nonsteroidal anti-inflammatory drugs. Take a directed history and perform focused physical examination to distinguish rheumatoid arthritis from osteoarthritis. Take a directed history and perform a focused physical examination for systemic vasculitis 26. Take a directed history and perform a focused physical examination for the causes of vasculitis. Perform a focused physical examination to distinguish between papilladema vs papillitis. Perform a focused physical examination for the causes of the Argyll Robertson pupil. Perform a focused physical examination for a lesion in the cavernous sinus, cerebellopontine angle, jugular foramen, pseudobulbar and bulbar palsy (multiple cranial nerve palsies), and its causes. Take a directed history and perform a focused physical examination for a lesion at the cerebellopontine angle. Take a directed history and perform a focused physical examination to determine the causes of dysarthria (disorder of articulation). Perform a focused physical examination to localize a spinal cord lesion to a specific lumbar or sacral nerve root level 30. Perform a focused physical examination for a lesion in the spinal canal at any level below T10 (cauda equina syndrome) 41. Perform a focused physical examination of the sensory dermatomes of the peripheral nervous system, and give their signature zones. Perform a focused physical examination for the cause of a carcinomatous neuropathy. Perform a focused physical examination for the causes of benign intracranial hypertension (pseudotumour cerebri). Perform a focused physical examination for meningitis (the numbers in prackets represent valves for sensivity) 80. R Thomson 8 Introduction the language of neurology Agnosia o Failure to recognize, whether visual, auditory or tactile; Related to receptive dysphagia. The Medical Society, Faculty of Medicine, University of Toronto 2005, page 156-159. Cranial nerves Remember: You need to establish where the lesion is, and what the likely lesion is. R Thomson 14 Vagal o Motor to soft palate, larynx and pharynx (from nucleus ambigus) o Sensory and motor for heart, respiratory passengers and abdominal viscera (from dorsal nucleus) Spinal accessory o Motor to sterno mastoid and trapezius o Accessory fibres to vagus Hypoglossal Motor to tongue and hyoid bone depressors Source: Burton J. R Thomson 15 V (Trigeminal) o Sensory Pain, temperature and light touch for same side of face, cornea, sinuses, nasal mucosa, teeth, tympanic membrane, anterior 2/3 of tongue The Medical Society, Faculty of Medicine, University of Toronto 2005, pages 157-158. Central scotoma Left homonymous hemianopia with macular sparing Adapted from: Talley N. A1: o the pupil react to light but not to accommodation o Seen in parkinsonism caused by encephalitis lethargic Q2: What causes miosis A2: o Old age o Pilocarpine (treatment for glaucoma) Q3: What non-neurological conditions cause an eccentric pupil R Thomson 25 Argyll Robertson pupil (distinguish) Pontine lesion Narcotics o Sympathetic Horner syndrome o Drugs Pilocarpine eye drops Adapted from: Baliga R. R Thomson 26 Benzene Tobacco o Metabolic Diabetes mellitus B12 deficiency Intestinal or uterine haemorrhage o Demyelinating disease eg. Papilladema Papillitis Optic disc o Swollen without venous o Optic disc swollen pulsation Visual acuity o Normal (early) o Poor Blind spot o Large o Large central scotoma Visual fields ofperipheral constriction o Onset usually sudden and o Usually slow onset of bilateral unilateral o Colour vision normal o Eye movement no pain o Painful Adapted from: Baliga R. Useful background: Cervical sympathetic pathway to the eye Mid brain (superior colliculus) Tectospinal tract C8, T1 and 2 ventral roots Cervical sympathetic trunk Internal carotid and cavernous nerve plexus Ophthalmic division of the trigeminal nerve Source: Burton J. Perform a focused physical examination to determine the cause of unequal pupils (anisocoria). R Thomson 29 Useful background: Useful terms Odds that a given symptom or sign is present in a person without the targeted disorder. R Thomson 32 o Lyme disease Degenerative o Multiple sclerosis o Syringobulbia Adapted from: Baliga R. Perform a focused physical examination of the patient with a large pupil which reacts slowly to light and accommodation (Holmes-Adie syndrome). At baseline, there is anisocoria with the right pupil larger than the left (first row). After instillation of dilute pilocarpine eye drops (fourth row), the pupil constricts markedly. R Thomson 34 Useful background: the relative afferent papillary defect (Marcus Gunn Pupil) Room Licat symmetric Swinging Flashlight Normal eye illuminated pupils constrict Abnormal eye illuminated pupils dilate Marcus Gunn pupil this shows a patient with an abnormal right optic nerve. R Thomson 35 Secondary to ocular disease o Glaucoma o Opthalmitis o Trauma (contusion cataract) Metabolic o Diabetes mellitus o Hypoparathyroidism (lamellar cataract) o Corticosteroid therapy Miscellaneous causes o Atopic eczema Heat and irradiation Adapted from: Burton J. Q: What is the neurological changes associated with hyperparathyroidism A: Cataracts Papilloedema Basal ganglia defects Benign intracranial hypertension Source: Burton J. R Thomson 37 Useful background: Cardinal positions of gaze Adapted from: Filate W. Useful background: Common causes of third nerve palsy Infection o Encephalitis o Basal meningitis o Carcinoma at the base of the skull Infiltration o Parasellar neoplasms o Meningioma at the wing of sphenoid o Tumors, collagen, vascular disorder, syphilis. R Thomson 41 Useful background: Causes of a red and painful eye Disease Distribution of Corneal Pupil Vision Iris Discharge redness surface Bacterial o Peripheral Normal o Normal o Normal o Normal o Muco conjunct conjunctiva o Reactive puralent tivitis o Bilateral (central sparing) Acute iritis o Around Dull o Irregular o / o Normal o Watery cornea shape blurred o Unilateral o Miotic o Photo o Slowly phobia reactive Acute o Around Dull o Oval o / o Corneal o Watery closure cornea partially blurred edema glaucoma o Unilateral dilated o Non reactive Corneal o Around Dull o Normal o o Defect o Watery/ ulcer/ cornea Fluo o Reactive shadow muco abrasion o Unilateral rescein purulent dye stains ulcer Irregul ar light reflex Disease Distribution of Corneal Pupil Vision Iris Discharge redness surface Sub o Localised Normal conjunctival hemorrhage hemorrhage o No posterior limit Conjunctival o Localised Normal hemorrhage hemorrhage o Posterior limit present Adapted from: Talley N. A: o Facial and trigeminal nerves o Corticospinal tract o Median longitudinal fasciculus o Parapontine reticular formation o Temporal bone Source: Baliga R. Useful background: Ramsay-Hunt Syndrome (herpes zoster of geniculate ganglion) Pain in ear and mastoid region Facial paresis or spasm Deafness, dizziness or hyperacusis Vesicles on auricle or anterior fauces Ipsilateral taste loss in anterior two-thirds tongue Source: Burton J. A1: oThe nervus intermedius of Wrisberg oTaste sensation from the anterio two thirds of the tongue oProbably, cutaneous impulse from the anterio wall of the external auditory canal. R Thomson 51 Useful background: Causes of multiple cranial nerve palsies inherited o Arnold Chiari malformation Infection o Guillain Barre syndrome (spares sensory nerves) o Tuberculosis o Sarcoidosis infiltration o Nasopharyngeal carcinoma o Hematological malignancy, o Brainstem tumor (eg in the cerebellopontine angle) have similar signs Vascular o Brainstem vascular disease causing crossed sensory or motor paralysis. R Thomson 52 Causes o Infection Local meningeal involvement Syphilis Tuberculosis o Infiltration Acoustic neuroma. Medical Society, Faculty of Medicine, University of Toronto, 2005, pages 153 to 154 and 157 to 158. The Medical Society, Faculty of Medicine, University of Toronto, 2005, page 162; Baliga R. Perform a focused physical examination to determine the site of defect and the causes of dysarthria (disorder of articulation). Take a directed history and perform a focused physical examination for the jugular foramen syndrome. Nystagmus and vertigo Useful background: Nystagmus Definition o A series of involuntary, rythamic oscillation of one or both eyes. R Thomson 59 Peripheral lesions o Severe vertigo + nausea/vomiting in acute phase o Lying still, fixing eyes on bright objects helps symptoms Central lesions Fast Findings with a right sided side lesion looking to the left Slow drifting phase Vestibular nystagmus Central (vestibular Peripheral nuclei) (labyrinth or vestibular nerve) Vertigo Rare Yes Auditory symptoms No Yes Lying still, fixing eyes on No Yes bright objects helpful Adapted from: Davey P. R Thomson 61 Headache and facial pain Useful background: Mechanisms of headache production Muscle o Skeletal muscle contraction. Useful background: History taking for headache Likely many aspects of history taking, a combination of pretest probability estimation, a system of inquiry, and thoughtful reiteration and probing work best. Two of the following are present: unilateral pain, pulsing or throbbing quality to pain, moderate to-severe intensity preventing daily activities, or pain provoked by routine physical activity iv. One of the following is present: nausea, vomiting, photophobia, phonophobia, or osmophobia v. R Thomson 65 and flushing of the forehead lasting minutes to hours, in bouts lasting several weeks, and coming a few times a year. The Medical Society, Faculty of Medicine, University of Toronto, 2005, page 172; Jugovic P. Perform a focused physical examination to distinguish between an intramedullary from an extramedullary cord lesion. R Thomson 71 o Chronic Alcoholic cerebellar degeneration Hypothyroidism Hydrocephalus Chronic infection (panencephalitis, rubella, prion disease) Vitamin E deficiency Paraneoplastic syndrome Alcoholic cerebellar degeneration o An ataxia that affects the trunk and gait (upper body ataxia and dysarthria are less frequent). A:o Roussy-Levy disease: hereditary spinocerebellar degeneration with atrophy of lower limb muscles and loss of deep tendon reflexes. Q: Name the three parts of the cerebellum, and perform a focused physical examination to distinguish which part is causing the ataxia. A:o Paleocerebellum Gait ataxia (inability to do tandem walking): anterior lobe o Aerchicerebellum Truncal ataxia (drunken gait, titubation): flocculonodular or posterior lobe o Neocerebellum Limb ataxia, especially upper limbs and hyponia: lateral lobes Source: Baliga R. Perform a focused physical examination to distinguish between sensory ataxia and cerebellar ataxia. Clinical Cerebellar ataxia Sensory ataxia Site of lesion o Cerebellum Posterior column or peripheral nerves Deep tendon o Unchanged or Lost or diminished reflexes pendular Deep sensation o Normal Decreased or lost Sphincter o None Decreased when disturbances posterior column involved, causing overflow incontenence Source: Baliga R. A1: o Cerebellar signs on the side opposite the third nerve palsy (which is produced by damage to the nucleus itself or to the nerve fascicle). R Thomson 75 Spinal cord and nerve roots Useful background: Spinal cord Transverse section Gracilis Cuneatus Crossed pyramidal Post. Useful background: Spinal cord disorders Paraplegia or quadriplegia due to complete transverse lesions Effect depends on level. The Medical Society, Faculty of Medicine, University of th Toronto, 2005 Filate W. The Medical Society, Faculty of Medicine, University of Toronto, 2005, page 175; Talley N. Perform a focused physical examination to localize a spinal cord lesion to a specific lumbar or sacral nerve root level. Perform a focused physical examination to distinguish between total spinal cord transection or incomplete cord compression. R Thomson 79 Useful background: Cervical spine movements and their respective myotomes Movement Myotome Neck flexion o Forward C1-C2 o Sideways C3 Shoulder o Elevation C4 o Abduction C5 Elbow o Flexion and/or wrist extension C5 o Extension and/or wrist flexion C7 Thumb o Extension and/or ulnar deviation C8 o Abduction and/or adduction of hand intrinsics T1 Adapted from: Filate W. A: o Paraplegia-flexion is seen in partial transaction of the cord where the limbs are involuntarily flexed at the hips and kees because the extensors are more paralysed than the flexors. Complete spinal cord injury Brown-sequard Central cord syndrome and anterior cord syndrome syndrome (syringomyelia). Perform a focused physical examination for the causes and site of spinal cord compression. Take a directed history and perform a focused physical examination for tabes dorsalis. R Thomson 87 Dermatomes in the lower limb Type of carcinomatous neuropathy o Dementia o Encephalomyelitis o Cerebullum xx o Cord-bone, xx, cord itself o Xx o Neuropathy o Myopathy, xx, xx o Myasthenia syndrome Adapted from: Burton J. R Thomson 91 Causes of spinal cord compression o Vertebral Spondylosis Trauma Prolapse of a disc Tumour Infection o Outside the dura Lymphoma, metastases Infection. The Medical Society, Faculty of Medicine, University of Toronto, 2005, Table 9, page 163. Remember to make side-to-side comparisons of tone, pattern of weakness and reflexes. Tendon reflexes: root level Ankle S1, 2 Knee L3, 4 Biceps C5, 6 Supinator C5, 6 Triceps C6, 7 Source: Burton J. Perform a focused physical examination for a lesion in the spinal canal at any level below T10 (cauda equina syndrome). Perform a directed physical examination to establish the neurological cause of a brachial plexus lesion, and the cervical rib syndrome. R Thomson 102 Useful background: Segmental Innervation of Muscles (Most muscles are innervated by nerves from more than one spinal root.
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Women also had a record of considered in nding the optimal transplant for the recipient allergy medicine cat dander buy generic deltasone on-line. Conjunctiva allergy testing laboratory discount 20mg deltasone visa, nasolacrimal duct and tear lm Signi cant sex-related differences have been identi ed for Signi cant sex-related differences have been identi ed in the example in the density of goblet cells and susceptibility to lacrimal gland allergy testing essex cheap 10mg deltasone mastercard, meibomian gland allergy symptoms night sweats buy cheap deltasone 20mg line, cornea food allergy testing zurich generic deltasone 5mg with mastercard, conjunctiva allergy forecast austin tx discount deltasone 5mg on-line, nasolacrimal in ammation (Table 3) allergy forecast brookfield wi purchase deltasone 40mg without prescription. These features allergy symptoms in ears buy deltasone 10mg on-line, as well as the acute angle between the bony canal and nasal oor in women, may predispose to chronic 2. Lacrimal gland in ammation of the nasolacrimal drainage system and may explain Signi cant, sex-related differences exist in the anatomy, physi why primary nasolacrimal duct obstruction is more frequent in ology and pathophysiology of the lacrimal gland (Table 3). Signi cant sex-related differences exist in the tear vestigators have speculated that the increased diffuse atrophy, and lm (Table 3). Overall, many of these sex-related differences in the orbital lobe and periductal brosis, present in the lacrimal glands of ocular surface and adnexa are likely to be due to the in uence of elderly women may decrease aqueous out ow and contribute to hormones and genetics. Sex-related differences in immunity of the ocular surface and gene in female mouse lacrimal tissue is particularly intriguing adnexa [83, 89]. In general infections are more com autoantigenic target of both B and T-cells [212]. Examples of sex-speci c differences in the innate immune response include: males have a greater percentage of pro 2. Meibomian gland in ammatory cytokine producing monocytes than females [227]; Sex-related differences have been identi ed in the morpholog females have less natural killer cell activity than males [228]; pe ical appearance, gene expression, neutral and polar lipid pro les, ripheral blood monocytes and plasmacytoid dendritic cells from and secretory output of the meibomian gland (Table 3). Examples of sex-speci c differences in the adap species are not necessarily the same. As and sebaceous glands are known have sex-associated differences alluded to with the last example, the effects of steroid hormones [213]. Signi cant sex-related differences exist in corneal anatomy and In terms of genetic effects an obvious point of discussion is the physiology (Table 3). The X chromosome has some 1100 genes (versus the Y which these alterations include variations in thickness, hydration, cur harbors less than 100) including several that are involved in im vature and sensitivity, endothelial pigmentation, foreign body mune function such as certain cytokine receptor subunits and Toll sensation, contact lens tolerance and visual acuity (Table 3) like receptors, E26 transformation-speci c domain-containing [214e222]. While donor transplants from males have higher which is important for Treg development [225]. The tear lm is an affect the immune response can be minimized in females whereas essential component of the ocular immune response and contains in males the effects of the altered gene will be manifest. In humans many components with antimicrobial functions [248] and a small approximately 15% of the genes on the inactive X chromosome number of studies have compared the levels of some of these actually remain active thus it is also possible for females to have antimicrobial components in males and females. Secretory IgA at increased expression of some X-linked genes if both copies have the ocular surface binds and neutralizes pathogens and facilitates remained active [235]. Although females produce higher levels of antibody contribute to enhanced immune responses. Further they have a 14-fold increase in the prevalence of tears of adult male rats than females [100, 103]. In contrast, at least in rabbits, the concentration of humans with approximately 10% being located on the X chromo lipocalin was increased in adult male rabbits in lacrimal uid and some [238]. These ndings suggest that, at least in animals, some of the that their differential expression in immune cells will also immune cells that act as a bridge between the innate and adaptive contribute to sex-related differences in immunityand susceptibility immune responses may be involved in sex-speci c responses. Most studies have demonstrated Another factor that may contribute to sex-differences in im that humans have no sex-related differences in lacrimal lympho munity are the microbial communities all humans harbor. However, it may be that the differences in immunity in males microbiome in a sex speci c manner while in turn members of the and females primarily manifest when the immune system is chal microbial community can metabolize sex hormones so in uencing lenged. Thus, male sex has been found to be risk factor for devel their effects on host immunity [242]. The conjunctiva hosts decreased conjunctival goblet cells and lower production of mucin secondary lymphoid tissue, called conjunctival associated and tears compared to male mice. To cover and quantify the on sex-related differences in pain is mostly not speci c for the multiple aspects of pain intensity and disability, multidimensional ocular surface. It is known that female sex and older ageare main factors ocular surface sensitivity are equivocal [140, 290e292]. The higher incidence of pain pausal women have been found to be more sensitive to corneal related symptoms among women compared with men has been stimulation than men of similar age, but overall there were no ascribed to sociocultural (gender-related) factors or biased report differences in mechanical and chemical thresholds between men ing. There are some common misconceptions about pain that have hindered the investigation of pain mechanisms and sex-related 2. Some of the and unpleasantness most popular incorrect beliefs include that i) pain does not exist in the current knowledge on chronic pain mechanisms involves the absence of physical or behavioral signs or detectable tissue complex brain circuits that include sensory, emotional, cognitive damage; ii) pain without an obvious physical cause is usually psy and interoceptive processing [295]. The neural networks join chogenic; and iii) patients who respond to a placebo drug are physiological systems (such as sensory, immune, endocrine, auto malingering [266]. It is dif cult to draw sations with a variety of expressions (dry/dryness, gritty, burn/ rm conclusions as to sex in uences in such complex in burning hot, red, crust, shut, discomfort, visual changes, sore terconnections. Various population-based studies suggested that irritated, gritty-scratchy, foreign body/foreign body sensation, women were more likely than men to experience a variety of burning, light sensitivity, itching, irritated, feeling of watery eyes, chronic pain syndromes [296e299], and tend to report more severe sharp, cutting, needle-like, pins and needles, pounding, pressure/ pain [300], at a higher frequency and in a greater number of body aching) [18, 274, 278e281], so it may be dif cult to correlate regions [301]. However, results from reviewed literature were not different descriptions with pain type and severity. Type of stimulus Pain thresholda Pain tolerancea Pain intensity or unpleasantness Cold pain W W < M No consistent difference Hot pain No consistent difference W < M No consistent difference Pressure pain W < W < M No consistent difference Ischemic pain W W M No consistent difference Muscle pain No consistent difference No consistent difference No consistent difference Chemical pain No consistent difference No consistent difference No consistent difference Electrical pain No consistent difference No consistent difference No consistent difference Visceral pain No consistent difference No consistent difference No consistent difference a Painthresholdreferstotheleastexperienceofpainthatcanbeidenti edbyasubject;Paintoleranceisde nedasthehighestlevelofpainthatasubjectisabletotolerate. Here it was noted provided by manufacturer is then used to convert lament length that methodologies employed to investigate the issue were not measurements to pressure. The Belmonte Gas esthesiometer [307, 308], and its conscious brain activity, brain activation imaging by positron modi ed version [309] which uses a jet of air to estimate ocular emission tomography, and brain functional magnetic resonance surface sensitivity to mechanical, chemical and thermal stimuli. Another study [310] utilized the Cochet-Bonnet esthesiometer and the role of genotype in pain is still understudied but some ev found that corneal sensitivity was higher in men than in women, idence is now emerging in terms of sex-related differences but only in superior, temporal and inferior areas. Sex differences in pain and the role of psychological factors factors Inconsistent or contradictory results were obtained with regard Bio-psycho-social factors, include hormonal factors exposure to to the direction of the association between anxiety/depressionwith sex steroid hormones (biological factors), blood pressure, heart sex and across outcome measures. Acute pain induces depressed rate, peripheral and central processing of the stimuli (physiological mood [320] and chronic pain is known to cause depression [321]. For a comprehensive review, readers are referred to males (21%) than males (13%) [322], although this might be Bartley and Fillingim [298]. Due to conceptual de cits such as small confounded by gender differences in reporting depression or sample sizes, experimental session timing across the menstrual seeking treatment. Post-traumatic stress disorders are conditions cycle and lack of biological markers to stage the cycle (such as urine that frequently coexist with chronic pain [323]. In female populations, depression, associated with reduction in discomfort symptoms [314, 315]. Of weak correlation between higher levels of androstenedione and course no sex difference could be retrieved from any of these 294 D. Sex differences in the response to and in correlation with age but not with sex [330]. Laboratory studies on sex-related differences symptoms [329, 331e335] but no sex-related differences were re in pain perception should be performed on healthy volunteers of ported in large population-based studies [336]. Taken from another various ages and on patients with painful pathologies (primary and perspective, a higher level of subjective happiness, as measured by secondary outcomes de ned beforehand, sample size estimated as a validated score [337], was inversely and signi cantly related to a function of clinical signi cance). The use of promising neuroimaging izing, a coping style which connotes negative emotional thoughts techniques is still very limited. All these points may represent the toward pain and adapting coping strategies [299]. Gender differences in pain and the role of social factors hormones include androgens, estrogens, progestins, hypothalamic Gender role broadly refers to a socially accepted set of charac pituitary hormones, glucocorticoids, insulin, insulin like growth teristics ascribed to each sex. A measure of gender-related personality traits (masculinitye femininity) is given with the Bem Sex Role Inventory [342]. Androgen regulation of the ocular surface and adnexa emotional vulnerability related to the masculinity-femininity trait and the perceived identi cation according to typical male/female Androgens are extremely important in the regulation of the stereotypes seem to alter pain tolerance, intensity, and unpleas ocular surface and adnexa [20, 348e350]. Past history may in uence pain perception in study of 390 different plasma metabolites in 1622 women with women but not in men. Table 5 Reported effects of orchiectomy or androgen treatment on the lacrimal glands of mice, rats, hamsters, guinea pigs and/or rabbits. In addition, older studies have reported no in uence of orchiectomy or androgen treatment on the growth or histological characteristics of the lacrimal gland [416, 417]. These latter ndings may be attributed in part to differences in experimental design, variations in the age, sex, and endocrine status of animals, the dosage and time course of androgen administration, and the methods of analysis. The lacrimal Androgen effects on the lacrimal gland may be enhanced or gland is an androgen target organ. Androgens exert a considerable attenuated by a variety of neurotransmitters, cytokines, secreta impact on the structure and function of this tissue, including gogues, autocoids, hormones, factors and viruses. Modulatory fac its cellular architecture, gene expression, protein synthesis, tors include vasoactive intestinal peptide, b-adrenergic agonists. Alter the primary mechanismby which androgens act on the lacrimal ations include degenerative changes, such as reduced growth and gland appears to involve binding to saturable, high-af nity and activity, loss of glandular elements, an attenuation in acinar cell steroid-speci c receptors in acinar and ductal epithelial cells. As shown in studies with mice, androgens modulate mice, rats, hamsters, guinea pigs, rabbits and humans the expression of thousands of lacrimal gland genes involved in [352, 389, 414, 425, 426]; (b) androgen receptor protein is present biological processes, molecular functions and cellular components predominantly within epithelial cell nuclei of lacrimal tissues of [90, 351]. Gene ontologies most affected by testosterone include mice, rats, hamsters and humans [97, 372, 385, 389, 427, 428]; (c) those associated with cell growth, proliferation and metabolism, lacrimal glands feature a single class of saturable, high-af nity and cell communication and transport, nucleic acid binding, signal steroid-speci c androgen binding sites, which have a dissociation transduction and receptor activities [90, 351]. Androgens also modulate the expression of their own include: [a] testosterone derivatives. Androgen tains a nitrogen derivative substitution for the 3-ketone function in binding proteins have also been identi ed in lacrimal glands and dihydrotestosterone); [g] adrenal cortical androgens. Consequently, although androgen action on may primarily be from local, intracrine synthesis (Fig. As lacrimal gland structure is considerable, it is unlike that of the demonstrated in the eld of intracrinology, the vast majority of ventral prostate, which in most species is completely dependent androgens in women. Instead, androgens induce time-, strain and to adjust the formation and metabolism of sex steroids to local species-dependent effects, leading to a non-uniform increase, requirements [449, 453]. Of particular in 17b-hydroxysteroid dehydrogenase types 1 and 3, aromatase, glu terest is the observation that androgens upregulate the expression curonosyltransferase and sulfotransferase [459]. Aromatase is an enzyme that catalyzes the transformation of testosterone and androstenedione to 17b-estra 3. Clinical relevance of androgen in uence on the lacrimal gland diol and estrone, respectively. Women with Sjogren syndrome are androgen-de cient testosterone and 17b-estradiol in lacrimal glands of castrated fe [391, 467e472]. Thus, androgen action on the lacrimal gland is not 3a, 17b-diol glucuronide (3a-diol-G) are signi cantly reduced in mediated primarily through a conversion to estrogens. This related to in ammatory responses, immune cell chemotaxis and decrease in testosterone would enhance in ammation, given that antigen presentation [510]. This androgen de ciency would interaction may then induce the altered activity of speci c genes compromise the positive regulatory in uence of androgens in and proteins in lacrimal tissue. In addition, this anti-in ammatory effect seems to be site administration, in turn, may prevent lacrimal gland regression speci c: androgens decrease lymphocyte accumulation in lacrimal, [361, 362, 387], thereby suggesting that these hormones are essen as well as salivary, glands, but do not diminish the extent of tial for maintaining uid secretion by lacrimal tissue. Moreover, androgen synthesis in Tfm mice is severely reduced these ndings support the hypothesis that androgen de ciency is a [516]. Similarly, investigators have found that androgen ciency may contribute not only to the prevalence of Sjogren syn- de ciency caused by castration and/or interruption of the drome, but also to the sex-speci c expression of other autoimmune hypothalamic-pituitary axis does not induce any lymphocyte diseases. This sexual dichotomy, in turn, has been linked to the accumulation in, or regression of, the lacrimal glands in male and differential actions of sex steroid hormones on the immune system female rats, guinea pigs and/or rabbits [204, 353]. Estrogens often enhance, whereas androgens also demonstrated that androgen receptor dysfunction. Tfm frequently attenuate, the progression of autoimmune sequelae mice) and androgen insuf ciency. In effect, androgen de ciency appears to medications) do not cause aqueous tear de ciency [353]. Overall, it appears that androgen de ciency erythematosus, thyroiditis, polyarthritis, autoimmune hemolytic promotes, but does not cause, the lacrimal gland in ammation in anemia, and myasthenia gravis, as well as in humans with Sjogren syndrome. Further, androgen de ciency may impair rheumatoid arthritis and systemic lupus erythematosus lacrimal gland function, but does not seem to induce aqueous [348, 394, 396, 397, 487, 498, 499, 505e508]. The mechanism(s) by which androgens suppress lacrimal gland Lastly, it should be noted that androgens induce lymphocyte in ammation in Sjogren syndrome has yet to be clari ed. For example, androgens suppress the develop lacrimal gland immunopathology [521e523]. Researchers have reported gland activity and secretion, in turn, may be antagonized by or that low serum concentrations of testosterone are also more chiectomy or topical anti-androgen treatment [550e554]. However, the reason for this at least in part, through binding to classical nuclear receptors. In fact, investigators have proposed that the measure within acinar epithelial cell nuclei [426, 427, 543]. In addition, an ment of serum testosterone in women may have little or no value drogens regulate the expression of numerous genes in mouse, except as an index of ovarian activity [447, 449, 451]. These effects synthesis in peripheral tissues, and not the ovary, is the primary appear to depend on the presence of functional androgen receptors source of androgens (or estrogens) in human females [556, 557]. It is possible that androgen action may also involve androgen levels that occurs during menopause, pregnancy, lacta binding to membrane receptors, triggering of signal transduction tion, or the use of estrogen-containing oral contraceptives may cascades and associated changes in gene transcription [561, 562]. The reason is that extended matase, glucuronosyl-transferase and sulfotransferase [427, 459]. Androgens exert a signi cant impact on gene expression in the Consequently, androgen de ciency in males who showno evidence human meibomian gland. Androgens stimulate 25 gens have been demonstrated to stimulate the lacrimal gland different ontologies (with! This immune function is mediated primarily through this hormone response is similar to the androgen in uence on secretory IgA (sIgA), which originates from plasma cells in the mouse meibomian glands in vivo [90, 547, 558, 559] wherein lacrimal gland [533, 534], is transported across epithelial cells into testosterone upregulates many genes linked to lipid metabolic tears by the polymeric Ig receptor. The meibo ciated with steroidogenesis, microbial protection, tissue develop mian gland, a large sebaceous gland, is an androgen target organ. Clinical relevance of androgen in uence on the meibomian epithelial surface resistance to microbial colonization [565], aswell gland. Sjogren syndrome, systemic lupus erythematosus, scription factor that mediates cell responses to oxidative stress rheumatoid arthritis) [391, 430, 585]. Aging is also for the metabolism of all active androgens and estrogens [576] and accompanied by marked alterations in the polar and neutral lipid may mediate the local, intracrine synthesis of androgens from ad pro les of meibomian gland secretions [112, 588]. Testosterone stimulates tions were made by comparing 37 and 70 year-old people, and this insulin-like growth factor 1, a pleiotropic protein that stimulates time period between the 4th and 8th decades coincides with a sebocyte proliferation, differentiation and signaling [577], Estrogen dramatic decline in androgen levels in both sexes [447]. In fact, the age-related cellular shrinkage in certain Androgens also in uence the lipid, and possibly protein, sebaceous glands has been directly correlated with attenuation in composition within the meibomian gland. Furthermore, investigators have re released and possess a lipocalin-like function in the tear lm ported that decreased serum concentrations of testosterone are 300 D. This latter correlation is of interest, rose bengal staining and inferior bulbar conjunctival rose bengal given that serum testosterone levels represent < 0. Androgen regulation of the cornea and conjunctiva found in rabbits following orchiectomy, including increased rose 3. Conversely, androgen de ciency has been linked to in women with polycystic ovary syndrome [608, 609]. However, the the mechanism of androgen action in the cornea and conjunc extent to which the ocular ndings in polycystic ovary syndrome tiva appears to involve the local, intracrine synthesis of androgens relate to excess androgen levels is unclear. Androgens and estrogens may often induce opposite re tival epithelial cells [575]. Hence, in postmenopausal women and in men, local rather mediate almost 30% of the sex-associated differences in gene than systemic estrogen levels would be expected to direct estrogen expression of the mouse meibomian gland [8]. In addition, andro action in peripheral tissue, including the ocular surface [639]. Serum estrogen levels may be more relevant to the and these have been linked to the presence of functional androgen ocular surface of women prior to menopause. For example, several genes are up or of serum testosterone are an order of magnitude greater than down-regulated by testosterone in the female, but not male, mouse circulating estrogen levels in postmenopausal women and thus meibomian gland [90]. In addition, a number of the androgen probably an important source of estrogen in peripheral tissues regulated genes in female glands are altered in the opposite di [639]. Thus, further to its direct androgen action discussed in the rection by 17b-estradiol and/or progesterone [90]. These genes section above, testosterone has an important in uence on estrogen could be involved in cell maturation, migration and holocrine action through its aromatization to estrogen in target tissues, secretion in the meibomian gland. In men, serum levels of testosterone with a pro-sebaceous action of androgens and an anti-sebaceous are another order of magnitude higher than those in post effect of estrogens. These sex-associated differences may be due to the in uence of androgens, given that these hormones are known to stimulate mitosis in the corneal epithelium [155]. Estrogen and progesterone regulation of the ocular surface and adnexa In contrast to androgen, the role of estrogenat the ocular surface is less well de ned, with effects that appear to be tissue-, sex-, and dose-speci c. Estrogen and progesterone presence at the ocular surface Analysis of intra-tissue estrogen or progesterone levels in ocular tissue has not as yet been undertaken. Both estrogen and proges terone have been detected in human tears, and are reported to be correlated with levels in serum of premenopausal females [636]. Other than these two conference abstracts, no investigation of sex hormones in tears has been published, re ecting the dif culties in detecting low concentration com pounds, such as sex hormones, in small volumes of tears. As is the case for testosterone, there is substantial evidence for biosynthesis and metabolism of estrogen at the ocular surface, which implies that it exerts a biological in uence here. Intracellular syn thesis and metabolism of sex hormones is a process unique to Fig. Immunohistochemical staining shows nuclear positivity for estrogen receptors in basal primates, and as such suggests caution for extrapolation of rodent and parabasal cells of acini (arrow). Progesterone, in nuclear signaling pathways or via control of other pituitary contrast, is primarily formed from cholesterol within the adrenal hormones [647].
Dermatology is important for the identification of skin lesions and the treatment of benign and malignant skin lesions allergy treatment providers generic deltasone 20 mg. The first year residents begin by assisting the faculty on their clinical rotations allergy symptoms of flu buy 20mg deltasone fast delivery. When the faculty feels that they are capable allergy symptoms wheezing discount deltasone 40 mg without a prescription, they begin performing simple and then moderately complex procedures allergy testing and zantac buy generic deltasone from india. The first year rotations are experiences in general hospital plastic surgery allergy forecast nj mold buy deltasone 5mg overnight delivery, (West Jefferson and Our Lady of the Lake) which provide opportunities to do consultations allergy forecast georgetown tx deltasone 40 mg with amex, workup allergy treatment for babies generic deltasone 10 mg on-line, surgery and follow up on general hospital based patients allergy medicine build up cheap deltasone generic. We also provide a hand rotation at this level so that residents can gain experience in the diagnosis and treatment of hand problems. Second Year: the goal for the second year is to increase their knowledge of the core curriculum by repeating some of the core and adding additional parts not covered during the first year. They are also responsible for helping organize the presentations for core curriculum. They participated in the anatomy labs and the microsurgery courses as a team with the first year residents. The second year residents are expected to help with the instruction of the first year residents. The second year residents will be introduced to aesthetic surgery and begin to learn about these patients and their specific problems the second year residents also have ancillary rotations in Oral Surgery, Orthopedics, and Ophthalmology. During these rotations they are expected to learn surgery of the mandible, occlusion, and orthognathic techniques. Orthopedics will provide opportunities to deal with trauma patients and bone healing. Ophthalmology will be helpful in learning preoperative issues with orbital surgery and how to deal with topical problems in the eye. They will begin rotations at University Hospital to be involved in more complex cases. They will begin to improve their microsurgery skills and will be involved in teaching junior residents and medical students, after they have been judged capable by the faculty. They will help to administer the program and run the service at University Hospital. They will be expected to finalize their work on papers which they have begun during their first two years. They will be expected to have the skills necessary for the independent practice of plastic surgery upon graduation. They will be exposed to complex craniofacial surgery, and complex head and neck reconstructive surgery. They will be expected to develop skills in aesthetic surgery and become capable of independent practice in aesthetic surgery. Integrated: Residents will have completed medical school and will have had varied exposure to clinical practice. It is our goal to begin at that entry level and progress through a training program that will produce surgeons who are able to operate independently. Residents will rotate through general surgery where they will learn to evaluate the surgical patient, perform history and physical examinations, assist in surgery and begin to care for surgical patients on the floor. They will rotate through Emergency Medicine, learning to evaluate and treat patients who present with both acute medical and surgical problems. They will rotate through Orthopedics and learn to evaluate patients with musculoskeletal injuries. They will rotate through the trauma service and learn complex resuscitation and assist in emergent surgery. They will rotate through the dermatology service to become exposed to the diagnosis of skin lesions. Tissue dissection and avoidance of complication are goals, as well as progressive responsibility for care on the floor. They will learn to place central lines, chest tube, tracheostomy and other invasive procedures. They will rotate through Neurosurgery to learn how to evaluate patients with neurosurgical injures, frequently associated with craniofacial trauma. They will learn to evaluate burn patients and their resuscitation on the burn service as well as complex nutritional problems. They will rotate on the vascular service to be exposed to vasculopathies and vascular surgical repair. They will learn the evaluation of patients for anesthesia on that service and learn basic anesthesia drugs and techniques. They will have two months of introductory plastic surgery in this year to begin to understand the plastic surgery patient and to assist on plastic surgical procedures. They will rotate on the head and neck service for exposure to patients with head and neck cancer and the surgery for ablation in order to appreciate the reconstructive demands in these patients. They will rotate on a transplant service to learn to appreciate the process of evaluation, matching and avoidance of rejection. They will have upper level general surgical experience, performing more complex procedures and managing complex patients on the floor and in the clinics. They will have additional months of basic plastic surgery and attend conferences while on that service. During their next years, they will assume greater responsibility for hand trauma, especially at the Academic Medical Center. They will rotate on an oral surgical service to be introduced to lower facial trauma, application of splints and intermaxillofacial fixation and other oral surgical skills. They will be exposed to ophthalmology and learn to examine the eye and to understand the possible complications which may occur after eyelid surgery. They will have advanced rotations in plastic surgery and begin to perform plastic surgical procedures and care for complex plastic patients in the clinics and wards. They will take plastic surgery call and attend all conferences and laboratories that the division holds. A rotation specific to breast reconstruction will be available and the residents will rotate to Our Lady of the Lake in Baton Rouge for a very varied experience in general plastic surgery and surgery of the hand. They will continue their research activities and should produce at least one paper or presentation during this year. Residents will work with faculty both in the private setting and in a clinic setting. Residents will perform their own surgery on clinic patients under supervision of the faculty. They will be exposed to lipo-injection and will be shown office management and ethical aesthetic surgery. The will also assume the role of chief resident on the plastic surgery service and assist with management of the service. Clinical Faculty Baton Rouge Rotation *Educational directors # Core faculty Sponsoring Institutions Louisiana State University is the major educational institution in Louisiana. Medical training is based in two campuses, one in New Orleans and one in Shreveport, Louisiana. The New Orleans campus is called the Louisiana State University Health Sciences Center. It includes a four year medical school and postgraduate training programs in virtually all specialties and administers University campus of the Medical Center of Louisiana. The new state of the art Privately owned and operated 450 bed University Medical Center is the only Level 1 Trauma Hospital in the city. West Jefferson Medical Center is a large, (licensed for 450 beds) fully equipped non-profit state hospital on the West Bank. This hospital provides a large, varied experience in both elective plastic surgery and trauma. The Lake also operates two nursing homes, has an affiliated cancer facility adjacent to the main hospital, and operates a number of outpatient services on its campus as well as in outlying locations. East Jefferson General Hospital serves as the primary site for the dedicated hand rotation. The hospital provides funding for this rotation and the hand faculty uses it as their primary hospital. Ochsner Medical Center-Westbank is a 250 bed hospital in Jefferson parish on the west bank of the river. Touro Infirmary is a large hospital in Orleans Parrish utilized by academic and clinical faculty for general plastic surgery cases. Baton Rouge General Hospital located in Baton Rouge, Louisiana it has a burn unit through which residents rotate. The following is the projected rotation schedule for each academic year and the Goal and Objectives that correspond to each rotation. There may be some changes in the schedule due to circumstances beyond control of the division of plastic surgery. The plastic surgeon has an enormous amount of competition for these cases, and the number of non plastic surgeons in the market is increasing. Residents will attend private clinic and surgery and will also attend their own senior resident aesthetic surgery clinic. At the end of the rotation, the resident will demonstrate that he/she has developed the necessary knowledge and surgical skills to evaluate and treat patients with aesthetic problems. Demonstrate a satisfactory level of competency in the following general categories of aesthetic surgery: a. Recognize the effects of aging, photo-damage, lipodystrophy and other causes of aesthetic deformity 36 3. Anatomy of the eyelids, muscles, fascia, cartilage support and function of components b. Anatomy of the facial muscles, facial nerve and fascia and their relationship to the facelift d. Be familiar with aesthetic problems of the breast, include ptosis, tubular breast and asymmetry 5. Demonstrate that he/she can compare the preoperative condition and postoperative results in a critical manner 2. Develop knowledge of the aesthetic literature and apply the information to specific cases 4. Evaluation of nutritional status prior to surgery for massive weight loss this rotation includes aesthetic surgery of the head and neck, trunk and extremities and the breast. All clinic cases scheduled by the residents at the outpatient facility must fulfill the certification requirement. In order to be certified as competent to perform any aesthetic case, the resident must have observed/assisted a staff member in performing that type of case and demonstrate adequate knowledge about techniques, complications and follow-up to ensure that he/she can perform the surgery. They will see the team approach to congenital deformities of the head and neck and also will see adult craniofacial surgery as well. At the end of the rotation, the resident should demonstrate understanding of special plastic surgical problems and that he/she has reasonable experience and capabilities to care for these children. Obtain sufficient historical data and perform an adequate physical examination to formulate a treatment plan. Postoperative care for children, including pain control, fluid and electrolyte balance, wound care and antibiotic therapy 6. Etiology, embryology and anatomy of congenital abnormalities seen in children, including: a. Understanding the classification of facial fractures in children and how their treatment is different than in adults. Be able to obtain informed consents to parents regarding risks and expectations prior to surgery 3. Prevention of decubitus ulcer Hand and Upper Extremity Rotation Rotation Goals: At the end of the rotation the resident should demonstrate that he/she is familiar with both traumatic and elective surgery of the hand and upper extremity. Manage post-operative care and participate in rehabilitation of the hand as a member of the medical team. Surgery of traumatic injuries including primary and secondary reconstruction of tendons and nerves b. Professional demeanor, punctuality and reliability in regard to the performance of his/her duties 2. Use the library and online resources to access literature and apply that literature to his/her cases F. Systems Based Practice By the end of the rotation, the resident is expected to be: 1. Aware of malingering and dystrophic conditions and be able to differentiate them 2. Importance of periods of immobilization to prevent stiffness Ochsner Baptist: Rotation Goals: the resident will attend microsurgical cases done at Ochsner Baptist as well as craniofacial cases done at the Ochsner Main Campus. They should also be competent to perform other types of breast surgery, including reconstruction and aesthetic breast surgery. At the end of the rotation the resident should be familiar with all flaps performed for breast reconstruction and should be able to outline a plan for craniofacial reconstruction including virtual planning procedures and multiple flap procedures. Demonstrate a satisfactory level of competency treating patients in these general categories of breast surgery a. Recognize the deformities resulting from mastectomy, failed reconstruction and radiation. Demonstrate punctuality, professional demeanor and reliable performance of his duties. The resident should be attentive to the special needs of breast cancer patients, respect their privacy and confidentiality. Practice Based Learning and Improvement: During the rotation, the resident should: a. Understand the need for multidisciplinary teamwork, for example pathology surgery oncology radiation therapy. Position of patient in long cases to prevent neuropathy Our Lady of the Lake: Rotation Goals this rotation in Baton Rouge is based at Our Lady of the Lake. At the end of the rotation, the resident should demonstrate the ability to care for patients in both in and outpatient settings. The resident should be able to use plastic surgery principles to solve a variety of problems including extremity trauma, complex wounds, facial fractures, oncologic reconstruction, facial deformities, breast deformities, etc. Obtain sufficient historical data and perform a physical examination adequate to formulate a treatment plan 3. Demonstrate ability to manage postoperative complications in adults, including infections, nutrition and wound healing problems 5. Exhibit operative exposure and appropriate technical skills in general plastic surgery 6. Oncologic reconstruction after resection of breast, head and neck, and skin cancers h. Demonstrate adequate knowledge experience in skin grafts, local flaps, free flap tissue transfer and complex wound closure 2. Demonstrate adequate knowledge of head and neck, hand, trunk and extremity anatomy 2. Have an adequate fund of knowledge to treat problems such as difficult wounds, extremity trauma, hand trauma, facial trauma and oncologic problems 3. Interpersonal and Communication Skills During the rotation, the resident must demonstrate: 1. Respect to all healthcare professionals, including staff, faculty, fellows, residents, nurses, office staff, etc. Practice Based Learning and Improvement During the rotation, the resident should have: 1. Surgical knowledge to compare treatment approaches suggested in professional literature 2. Surgical knowledge to identify complications and formulate a plan to correct them, as well as prevent them in the future 45 3. Systems Based Practice During the rotation, the resident must demonstrate understanding of: 1. Touro Infirmary: Rotation Goals: Touro is a large community hospital in downtown New Orleans. Residents will work with a young well trained plastic surgeon and will be involved with referrals from the hospital as well as the community. At the end of the rotation they will have gained considerable experience in community based plastic surgery. The educational director is a highly innovative micro surgeon and performs state of the art inventive procedures for microsurgical reconstruction of the breast.
Red meat consumption and cancer: reasons to suspect involvement of bovine infectious factors in colorectal cancer allergy testing indianapolis order generic deltasone on line. Prevalence of Birth Defects by Plurality of Live Births and Stillbirths 37 Table 6 allergy testing madison wi cheap deltasone online american express. Plurality of All Live Births and Births Defect Cases allergy gainesville band order discount deltasone line, Live Births Only 44 Figure 4 allergy medicine 013 cheap deltasone on line. Prevalence of Selected Birth Defects by Plurality among Live Births and Stillbirths 45 Table 7 allergy shots for ragweed discount deltasone 5 mg free shipping. Prevalence of Birth Defects by Maternal Race / Hispanic Ethnicity for Live Births 76 Table 12 allergy medicine zyxel deltasone 20 mg cheap. These figures include direct costs of medical treatment allergy medicine and erectile dysfunction discount 20mg deltasone mastercard, developmental services and special education allergy gold filter cleaning buy generic deltasone 40mg on-line, as well as indirect costs to society for lost wages due to early death or occupational limitations. Inborn errors of metabolism are monitored separately by the state newborn screening program. The first annual report presented Massachusetts birth defects data for the year 1999. Common non cardiovascular defects included Trisomy 21, Polydactyly/Syndactyly, Hypospadias, Clubfoot, Cleft Lip with and without Cleft Palate, Cleft Palate alone, and Obstructive Genitourinary Defects. Selected Pregnancy Outcomes We compared selected pregnancy outcomes (C-sections, birthweight, gestational age, multiple birth and infant death) among infants born with birth defects to those born without birth defects in 2002-2003. Birth defects that more commonly occurred in multiple births included Esophageal Atresia/Tracheoesophageal Fistula, Hypospadias, Coarctation of Aorta, Diaphragmatic Hernia and Polydactyly/Syndactyly. The most common defects in Hispanics included Septal Defects, Polydactyly/Syndactyly, Clubfoot and Down Syndrome. In Asians, the most common defects included Septal Defects, Clubfoot, Cleft Lip, Cleft Palate, and Down Syndrome. These cases needed intensive medical care and planning for continuing care and long-term disability. Birth defects can lead to lifelong disability, require costly medical care and cause great distress in families. Researchers are looking at a wide variety of environmental exposures and risk factors as causes. For the developing pregnancy, the environment includes any exposure to the fetus as well as any exposure to the mother. Mandatory fortification of cereal grains with folic acid has resulted in a 26% reduction in the number of babies born with these neural tube defects (Mills, 2004). Birth Defects Surveillance in Massachusetts Over the past ten years, the Center for Birth Defects Research and Prevention has developed and refined its surveillance program. Abstractors have specialized training and ongoing education to abstract medical records of potential cases. Economic Impact on Massachusetts Estimating the economic impact of birth defects on the state of Massachusetts is challenging. Legislative Changes Regarding Birth Defects Surveillance In March 2002, the Massachusetts Legislature amended the state birth defects monitoring statute (Chapter 111, section 67E) to allow expansion of the surveillance system to capture diagnoses through age three. The data are presented in combined form since the numbers are relatively small for individual defects. Interpretations of these data must be made with caution until a multi-year estimate establishes a stable, baseline rate. Cases met the following criteria: the infant was live born or, the fetus was stillborn with a gestational age greater than or equal to 20 weeks or with a weight of at least 350 grams. The Center has developed extensive procedures to guarantee the confidentiality of data and protect the privacy of families. If the case had more than one defect within the same defect category, only one of these defects was counted in the category total. Prevalence is calculated as the number of birth defect cases born during the period 2002-2003 per 10, 000 live births born during the same period. Prevalence tables include the number of cases found, the estimated prevalence rate per 10, 000 live births, and the 95% confidence interval for that rate. Wide confidence intervals reflect the large variation due to small numbers (see Technical Notes). For example, it is estimated that up to 50% of all pregnancies affected with a neural tube defect may be discontinued and would thus not be included in hospital records (Cragan 2000). Spontaneous abortions that are delivered prior to 20 weeks of gestation and less than 350 grams are not included in the case definition. Another example, Fetal Alcohol Syndrome, may not be detected until developmental delays become evident when a child is much older. Glossary A glossary of selected birth defect terms is included in the appendices of this report. For the two years 1994 and 1999, 40-80% of pregnancies with either lethal or very severe defects were terminated (Peller 2004). The overall prevalence of reported birth defects in Massachusetts in 2002-2003 was 157. Cardiovascular Defects were the most commonly occurring birth defects in both Massachusetts and in the nation. They also contribute more to infant deaths than any other defect category (Petrini 1998). Selected Pregnancy Outcomes Figure 2 compares selected pregnancy outcomes (C-sections, birthweight, gestational age, multiple birth and infant death) among infants born with birth defects to those born without birth defects in 2002-2003 by percentage. These rates are from surveillance systems that include prenatally diagnosed and terminated pregnancies. As expected, there was a strong association of Down Syndrome with advanced maternal age (see Figure 6). Older mothers had higher rates for many defects including Esophageal Atresia/Tracheoesophageal Fistula, Hypospadias, Tetratology of Fallot, and many Syndromes. While results for other defects also differed by age group, the small numbers from two years of surveillance were not sufficient for interpretation. Atrial Septal Defects and Ventricular Septal Defects were common to all maternal age groups. Polydactyly/Syndactyly and Club Foot (except for mothers 25-29 years) were among the top five most common in every age group. Monitoring birth defects by maternal age is important since the number of births to older mothers has been increasing over time in Massachusetts. The most common defects in Whites included Septal Defects, Hypospadias, Down Syndrome, Polydactyly/Syndactyly and Clubfoot. More years of data and in-depth studies are needed to affirm the stability of these rates and to understand racial and ethnic patterns. The severity scale was developed by the Center in collaboration with our partners at Boston University and the Massachusetts General Hospital. If a case had multiple defects with equal severity, it was reviewed in detail by the Center Clinical Geneticist. In contrast, babies with isolated Dextrocardia (heart in the right side of the chest instead of the left) and no other heart defect have no clinical consequence. The width of the interval reflects the size of the subpopulation and the number of cases of birth defects. Excludes: aplasia of or absent lower limb 755685 7 Lower limb: other specified anomalies / hyperextended legs / shortening of legs 755680 8 Other absent or hypoplastic muscle / absent pectoralis major. Excludes: with Turner phenotype (758610) 758800 2 Nail-patella syndrome 756830 1 Other craniofacial syndromes / Hallermann-Streiff syndrome 756046 2 Other specified acrocephalosyndactylies 756057 1 Sex chromosome: Other specified anomaly / fragile X 758880 4 Specified syndromes, not elsewhere classified, involving skin anomalies 757300 1 Treacher-Collins syndrome / Mandibulofacial dysostosis 756045 2 Triploidy 758586 6 Trisomy 13: Patau syndrome 758100 8 Trisomy 13: translocation trisomy with duplication of a 13q 758120 1 Trisomy 18: Edwards syndrome 758200 21 Trisomy 18: Translocation trisomy with duplication of 18q 758220 1 Trisomy, partial / 8/02 "partial trisomy" = "duplication". For specified anomalies of nails 757580 757800 3 Spleen: Absence / asplenia 759000 3 Spleen: Accessory / 8/02 Use for polysplenia, though not exactly the same 759040 4 Spleen: Hypoplasia 759010 1 Thyroglossal duct anomalies / thyroglossal cyst 759220 2 110 1 Glossary of Selected Birth Defects Terms Agenesis, aplasia: Congenital absence of a body part or organ, implying that the structure never formed. Anophthalmia: Congenital complete (or essentially complete) absence of the eye globe. Cleft palate: Congenital defect in the closure of the palate; the structure which separates the nasal cavities and the back of the mouth. Usually occurs as an indentation at a specific location, less commonly diffuse narrowing. Fistula: Abnormal connection between an internal organ and the body surface, or between two internal organs or structures. Hirschsprung disease: Congenital aganglionic megacolon (enlarged colon) due to absent nerves in the wall of the colon. Holoprosencephaly: Spectrum of congenital defects of the forebrain due to failure of the brain to develop into two equal halves. Hydronephrosis: Enlargement of the urine-filled chambers (pelves, calyces) of the kidney Hyperplasia: Overgrowth due to an increase in the number of cells of tissue. Spina bifida: Neural tube defect with protrusion of the spinal cord and/or meninges. Includes myelomeningocele (involving both spinal cord and meninges) and meningocele (involving just the meninges). Stenosis: Narrowing or constriction of the diameter of a bodily passage or orifice. Translocation: Chromosome rearrangement in which a piece of genetic material is transferred from one segment to another. Truncus arteriosus: Congenital heart defect characterized by a single great arterial trunk, instead of a separate aorta and pulmonary artery. Impact of prenatal diagnosis and elective termination on the prevalence of selected birth defects in Hawaii. Bureau of Health Statistics, Research and Evaluation, Bureau of Family Health and Nutrition, Massachusetts Department of Public Health. Impact of including induced pregnancy terminations before 20 weeks gestation on birth defect rates. Needless to say, I would like to express my deeply thank to the editors, Professor Rungsun Rerknimitr, Dr. Linda Pantongrag-Brown, Associated Professor Sombat Treeprasertsuk, and all contributors for their great efforts to create this fascinating book. Last but not least, please do not forget to visit us and download all previous issues from our website. Nuttaporn Norrasetwanich Division of Gastroenterology, Department of Medicine, Division of Gastroenterology, Department of Medicine, Chulalongkorn University, Bangkok, Thailand Chulalongkorn University, Bangkok, Thailand 2. Phonthep Angsuwatcharakon Division of Gastroenterology, Department of Medicine, Department of Anatomy, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Chulalongkorn University, Bangkok, Thailand 3. Pornphan Thienchanachaiya Division of Gastroenterology, Department of Medicine, Division of Gastroenterology, Department of Medicine, Chulalongkorn University, Bangkok, Thailand Chulalongkorn University, Bangkok, Thailand 5. Nopavut Geratikornsupuk Division of Gastroenterology, Department of Medicine, 17. Sasipim Sallapant Chulalongkorn University, Bangkok, Thailand Division of Gastroenterology, Department of Medicine, Chulalongkorn Unibersity, Bangkok, Thailand 9. Satimai Aniwan Chulalongkorn University, Bangkok, Thailand Division of Gastroenterology, Department of Medicine, Chulalongkorn University, Bangkok, Thailand Contributors 19. Tanassanee Soontornmanokul Chulalongkorn University, Bangkok, Thailand Division of Gastroenterology, Department of Medicine, Chulalongkorn University, Bangkok, Thailand 20. Vichai Viriyautsahakul Division of Gastroenterology, Department of Medicine, Department of Medicine, King Chulalongkorn Chulalongkorn University, Bangkok, Thailand Memorial Hospital, Thai Red Cross Society 21. Progressive scarring disease may lead to blistering diseases that predominately affects the 4 esophageal stenosis requiring dilatation procedures. The oral mucosa is involved in 89-100% of cases pemphigoid (cicatricial pemphigoid). Clin and the rate of conjunctival involvement is 61-71% with Dermatol 2012;30:34-7. The first contour, commonly in the upper esophagus, and international consensus on mucous membrane 3 representing the advanced stage of the disease. Her symptoms were partially improved increased number esophageal capillary loops (Figure 1 with proton pump inhibitors. A 24-hr esophageal pH monitoring was compatible revealed minimal change of distal esophageal mucosa. World J disease that the novel technology such as magnifying or Gastrointest Endosc 2010;2:121-9. A 64-year-old woman was scheduled for a Sodium sulfate, Sodium hydrogen carbonate, Sodium colonoscopy as a part of her colon cancer screening chloride and Potassium chloride). He underwent smooth narrowing segment of mid esophagus with surgery, radiation and chemotherapy 6 months ago. Microscopic Esophagoscopy showed narrowing esophageal lumen examination showed organizing inflammation with with smooth surface at 33 cm from the incisor (Figure 1 granulation tissue (Figure 5). The median duration between the end of radiation therapy and the time for diagnosis of References esophageal stricture was 8 months (1-132 months). Ingestion of alkali (such as ammonia or sodium hydroxide) acutely results in a penetrating injury called liquefactive necrosis. Up to one-third of patients who suffer caustic esophageal injury develop esophageal strictures. Dilation usually relieves symptoms of dysphagia; however, recurrent strictures occur in selective cases. A 25-year-old male was admitted to the erythematous mucosa were observed in the mouth emergency department with a sore throat, dysphagia, and on the tongue. These may result in perforation, Esophageal corrosive injury from paraquat mediastinitis and/or pneumomediastinum. The ingestion contribution of this direct caustic effect to mortality 2 is probably underestimated. The very endoscopies at Chang Gung Memorial Hospital between high case fatality of paraquat is due to inherent toxicity 1980 and 2007. Their findings showed a potential relationship failure, pulmonary hemorrhage, and late pulmonary between the degree of hypoxia, mortality, and degree of 3 fibrosis. Mucosal lesions in the corrosive esophageal injury after intentional pharynx, esophagus and stomach are also very common paraquat ingestion. Self covered stent placement expandable metal stenting of refractory upper gut corrosive strictures: a new role for endoscopy A 61-year-old man underwent esophageal a well-defined erythematous flat mucosa, 1. Figure 4: Microscopic examination showed disorganization and disorientation of esophageal mucosa with scatter pleomorphism of nuclei. Histological Vienna consensus for diagnosis of early esophageal precursors of oesophageal squamous cell neoplasia. The relative risk of high grade esophageal carcinoma: results from a 13 year prospective dysplasia patient to develop esophageal squamous cell follow up study in a high risk population. A 61-year-old man with a history of squamous dysplasia, at 32 cm from the incisor (Figure 1-2). Feasibility of 1 endoscopic resection in superficial esophageal develop esophageal squamous cell carcinoma. Gastrointest Endosc 2011; node metastasis, thus forming the basis for endoscopic 73:881-9. A 41-year-old man presented with dyspepsia demonstrated papillary projection of the esophageal and gastroesophageal reflux symptoms for 3 months. Esophageal squamous papilloma Morphologically, it is a benign lesion, but there is much debate as to whether it is a premalignant lesion. Discussion: At present, there is no evidence for this, and malignant Esophageal papilloma is rare benign epithelial 2, 3 changes have not been reported in humans. The etiology of esophageal squamous papilloma of the esophagus: long-term follow up. Most etiology in humans is chronic cell papillomas of the esophagus: report of 20 irritation from reflux esophagitis; two-thirds of reported cases and literature review. Am J Gastroenterol cases of esophageal papillomas are found in the distal 1994;89:434-7. The Esophageal Inlet patch inlet patch found in 10% of the population with careful searching at endoscopy but it is often overlooked by Discussion: endoscopists and radiologists and studies frequently Inlet patch is a congenital anomaly of cervical 1 report prevalence between 0. It occurs most Most inlet patches are largely asymptomatic, frequently in the postcricoid portion of the esophagus at but in problematic cases complications related to acid secretion such as esophagitis, ulcer, web, and stricture References may produce symptoms such as chest and throat pain, 1. Adenocarcinoma endoscopic prevalence, histopathological, may arise in the ectopic gastric mucosa but this is demographical and clinical characteristics. Frequency, 1-3 histopathological findings, and clinical significance associated with inlet patches as they are not metaplastic. Heterotopic gastric mucosa in only part of the circumference, but some are annular the upper esophagus: a prospective study of 33 and multiple lesions are not uncommon. Visible columns of red tortuous ectatic about 4% of all non-variceal upper gastrointestinal vessels along the longitudinal folds of the antrum are 1 6 bleeding. This is supported by findings that there is no significant Endoscopy 2004;36:68-72. Gastric (mean mucosal capillary cross-sectional area) with the antral vascular ectasia in cirrhotic patients: absence degree of portal hypertension and lack of response to of relation with portal hypertension. There are from severe portal hypertensive gastropathy in distinct entities that require different treatments. Liver disease in hereditary hemorrhagic this disease is diagnosed by the Curacao criteria which is telangiectasia. Hepatic vascular clinical features: nose bleeding history, mucocutaneous malformations in hereditary hemorrhagic telangiectasia, visceral involvement (pulmonary, cerebral, telangiectasia. Figure 7 Endoscopically, adenomatous polyps are typically velvety, lobulated solitary (82%), located in the antrum, typically with size less than 2 centimeters (cm) in diameter. Histology reveals dysplastic another area of the stomach has been found in up to epithelium without detectable invasion of the lamina 30% of patients with an adenomatous polyp, and up propia. Both conditions the risk of association between adenomatous 1, 2 are often found in patients with chronic, atrophic, polyps and cancer increases with age.
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