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Antigenic functions: Complex membrane proteins within the specific layer (but not from one layer to another) discount 100 mg kamagra chewable with visa. Functions of the Lipid Bilayer Peripheral Proteins The main function of the lipid bilayer of a cell membrane Some protein molecules are inserted lightly in the outer is to create a permeability barrier between the interstitial or inner border of the membrane or are just bound to the fluid and the cytoplasm 100 mg kamagra chewable with mastercard. Such proteins are called peri­ depends on whether it is lipid­soluble or water­soluble cheap 100 mg kamagra chewable free shipping. Lipid soluble substances like oxygen and alcohol can pass They are of two types: Intrinsic and extrinsic proteins. Intrinsic proteins: They are present on the inner sur­ easily through the cell membrane, whereas water soluble face of the membrane. They usually serve as enzymes or substances like urea and glucose cannot pass easily. Thus, anchor proteins for cytoskeleton and other microfilaments lipid bilayer makes the membrane semipermeable. Membrane Proteins Extrinsic proteins: They are present on the outer sur­ face of the membrane. They serve as cell adhesion mole- The protein content of biological membrane depends cules for anchoring cells with basal lamina and with neigh­ on the function of the membrane. They can be removed without disrupting the Schwann cell, the cell membrane is concerned with insu­ membrane. Therefore, protein constitutes less than 25 percent Membrane Carbohydrates of the membrane. In membrane of mitochondria, which is The external surface of the cell membrane is loosely involved in cell metabolism, 75 percent of the membrane covered by a carbohydrate layer known as the cell coat or is protein. Thus, the outer surface of the lipid bilayer is Some membrane proteins that span the entire thickness covered by a layer of glycoproteins and glycolipids. Band-3 proteins: Band­3 protein is a dimeric protein with molecular weight 93,000 that traverses membrane about 12 times. It acts as “pore” or transport protein that exchanges bicarbonate ions in the capillaries of lungs. Note the presence of cial proteins that are linked to the cytoskeleton and are special proteins such as ankyrin, spectrin and adducin that provide essential for stabilization of membrane and biconcave plasticity (deformability) to the membrane, in addition to the pres- shape of the cell. Spectrin: Spectrin is a fibrous protein that contains an α-chain with molecular weight of 240,000 and a β-chain with molecular weight of 220,000. Some of the transmembrane glycoproteins like selec­ attached to cytoskeletal protein that maintains membrane tins recognize and bind with specific oligosaccharides integrity and cell shape. Ankyrin has molecular weight occurs between neutrophils and endothelial cells at of 200,000. Stronger adhesion between and other with N­terminal region of band­3 protein that cells is formed by integral membrane proteins such as extends into cytoskeleton (Application Box 4. Diseases due to membrane protein defects: Disorders of red cells such as hereditary spherocytosis and elliptocytosis occur due to Functions of Cell Membrane defects in the membrane proteins (for details, refer “Red Blood Cell”). Cell membrane maintains a constant and distinctive Cell Organelles intracellular environment, which is essential for func­ tioning of the organelles. For example, the intracellu­ The usually occurring organelles in animal cells are mito­ lar fluid has lower concentration of sodium and chlo­ chondria, endoplasmic reticulum, Golgi apparatus, ribo­ ride, low pH, but higher concentration of potassium, some, peroxisome, lysosome and centriole. Cell membrane maintains cell volume by actively of its organelles such as mitochondria, ribosome and lyso­ transferring ions across it, especially by pumping some during maturation. In neurons and muscle cells, it maintains a potential Mitochondria difference between intracellular and extracellular sur­ Mitochondria are the “power house” of the cell. Cell membrane helps in recognizing foreign cells or vary in different tissues of the body. Mitochondrial Membranes Electron microscopy shows that each mitochondrion has Integral Proteins two layers of membranes: the outer and inner membranes. Two special types of integral proteins are found in red cell Outer Mitochondrial Membrane: This forms a continuous membrane (Fig. The carbohy­ that permit substances with molecular weight of less than drate component is oligosaccharide and the polypeptide 10,000 to diffuse freely across the outer membrane. Enzymes in inter-membrane space membrane is folded into multiple incomplete septa like 1. Citrate synthase Number of cristae: Number of cristae is more in resting state of 3. Ornithine transcarbamylase The region enclosed by the inner membrane is called matrix. Mitochondrial diseases: The disease that affects mitochondrial energy transduction is called Luft’s disease. Therefore, it is Mitochondria are also damaged by free radicals and affected in age- abundant in cells of endocrine glands and cells secreting related degenerations. It also plays some role in the conjugation of carbohy­ rylated here, and then pass to the trans­face, where drates with proteins to form glycoproteins, a function they are packaged into the secretory vesicles (Fig. It is the site for the incorporation of carbohydrates into the newly synthesized proteins to form glycopro­ Smooth Endoplasmic Reticulum teins. Transports material to other organelles and cell sur­ surface has “smooth” or “agranular” appearance. Hence it is abundant in cells that synthesize cholesterol, steroid Lysosomes are membrane­bound spherical organelles that hormones and phospholipids. More than 40 different lysosomal is specialized for the storage of calcium ions that is enzymes (lysozymes) have been isolated. It is also the site for the detoxification or neutralization such as neutrophils and monocytes. It is present in all cells and generally is In granulocytes, the lysosomes appear as cytoplasmic located close to the nucleus. Lysosomal activity of a cell tissue can be deter­ are dilated peripherally and stacked together in a bunch mined (Application Box 4. Marker for lysosomal activity: Acid phosphatase is used as a marker for lysosomal activity. The region nearer to the nucleus is the cis face (cis Golgi) Chapter 4: Cellular Organization and Intercellular Connections 19 Table 4. Catalase During the process of phagocytosis, the phagosome that is formed by the cytoplasmic pseudopodia containing foreign body such as bacteria, viruses, etc. The interior of lysosome is acidic (pH is about 5) com­ pared to its cytosolic exterior (pH of cytoplasm is about 7. The undigestible membrane that pumps protons (H ) from cytosol into the remnants in the lysosomes are called as residual bodies. The lysosomal enzymes are acid hydolases as they func­ Functions of Lysosome tion best in acidic pH. Lysosomes contain many enzymes essential for intra- Types of Lysosomes cellular digestions. Lysosomes kill and remove infective organisms and somes (phagolysosome) (Flowchart 4.

This increases the ameboid movement and acti- Eosinophils are granular leucocytes having the size same vity of neutrophils buy kamagra chewable 100mg mastercard. However discount kamagra chewable 100 mg, the granules are coarse phagocytic dysfunction of neutrophil kamagra chewable 100 mg line, decreases phago- and brick red in color in blood smear stained by Leishman cytic activity. Like neutrophils, eosinophils produce proinflamma- tory mediators: Eosinophil secretes various chemicals that mediate many 1. In addition, cytokines produced by eosinophil such as cytokines, and lipid-derived chemicals (Table 17. Eosinophils have short life span in circulation, whereas other cells, whereas chemicals secreted from granules are they live longer in tissue. Eosinophils are present in the epithelia of respiratory, The granules of eosinophil contain following major chemi- gastrointestinal and genitourinary tract. It is toxic against many intestinal parasites and their Cytokines larvae such as: Cytokines are synthesized outside the granules but stored 1. The important cytokines secreted from eosino- tinal parasite, blood fluke) and the larva of Ancylo- phils are: stoma duodenale (hookworm) 1. Eosinophils exhibit endothe- these worm infestations are usually associated with respiratory lial cell adhesion and chemotaxis to migrate into the tis- symptoms like bronchial asthma. Eosinophils participate in two important defense mechanisms of the body: Eosinophil Cationic Protein 1. First, eosinophils attach themselves to the Eosinophil Peroxidase larva, which activates the eosinophils. Within about 3 hours of this adherence, the activated • It has 68% homology with neutrophil myeloperoxidase eosinophils secrete proteins from their granules onto and other peroxidases. Allergic diseases Receptors for IgE, IgG IgE – Bronchial asthma – Allergic rhinitis 6. Eosinophilic leukemia The normal eosinophil count is 2–4% in differential count 5. Tropical pulmonary eosinophilia (by examining blood smear), or 40–440 per μl of blood 6. Drug-induced agranulocytosis recruit into tissues in response to immunological and inflammatory reactions, unlike eosinophils they ordi- 3. Once, the tegument is breached, eosinophils crawl narily do not reside in the tissue. Though basophils and under it and secrete toxic mediators that destroy the mast cells resemble functionally, they are not identical tissues of larva and phgocytose the larva or larval frag- (Table 17. Basophils and mast cells have high-affinity receptors play a major role in pathogenesis: for IgE on their surface. As IgE is a reagin antibody, these cells mediate many philia and airway eosinophil content. Basophils and mast cells also contribute to protect been reported to cause improvement in bronchial host responses associated with IgE production. It is not clear whether eosinophils prevent allergy and Identifying features of basophils are (Fig. They have the same diameter as of neutrophils in allergy aggravates the situation. The nucleus is usually less segmented and often allergic inflammation is accompanied by increase in appear ‘S’ shaped. However, massive release of histamine produces imme- diate hypersensitivity reactions, also known as anaphy- laxis. Anaphylaxis is acute systemic allergic reaction that occurs in conditions like injections of penicillin or xylocaine anesthesia in sensitive individuals. In anaphylaxis, histamine released from basophils and mast cells cause vasodilation and inhibition of cardiac Fig. The granules are large in size and oval or round in Role in Chronic Allergic Reactions shape, and more in number. As cell is heavily studded Basophils and mast cells also contribute to late-phase with granules, nucleus is often not visible. In chronic allergic conditions such as bronchial are surrounded by membranes and contain dense par- asthma, basophils and mast cells are recruited to the site ticles called Chracot-Lyeden crystals. Cytoplasm contains glycogen deposits, mitochondria, attract leucocytes, eosinophils and basophils, which in free-ribososmes and few lipid bodies. This mechanism of patho- Basophil granules secrete histamine, chondroitin sulphate, genesis of late-phase reaction is termed as mast cell- tryptase, carboxypeptidase A, cathepsin G, leukotrienes, leuco cyte cascade. Role in T-cell Dependent Responses Activation of mast cells in the affected tissues along with Mast Cells infiltration of basophils occurs in a variety of T-cell dependent Mast cells that remain in the tissues are round or elon- immunological responses. There are many cytoplas- mic filaments, numerous lipid bodies and no glycogen Role in Host Defense deposits. There are two types of mast cells: cells increase in conditions like chicken pox, small pox and mucosal mast cells (mast cell present in the mucosa) and tuberculosis. Normal Count Differences between Basophils and Mast Cells Normal basophil count is 0–1%, and absolute count is Though there are functional homology between basophils 20–80 per μl of blood. Mast cells are normally not found in and mast cells, there are many differences between them blood. Functions Mastocytosis Basophils and mast cells are mainly involved in allergic Secondary increase in mast cell count usually occurs in reactions. During allergy, these cells release the content allergic conditions like asthma, and connective tissue disorders like rheumatoid arthritis. Mediators such as histamine released by increase in mast cell number occurs in a group of systemic degranulation produce antimicrobial and anti-host effects. The usual stimulus for basophil and mast cell degranula- Depending on the degree of mastocytosis, the condition tion is an allergen, which should ideally cross-link IgE has been classified into 4 categories: molecule bound to the surface of basophils or mast cells Category I: Indolent mastocytosis as seen in urticaria via its high affinity Fc receptor for IgE. IgE binds to membrane of these cells and initiate denopathic mastocytosis with eosinophilia. Allergic and inflammatory conditions – Ulcerative colitis – Erythroderma – Urticaria – Drug and food hypersensitivity 2. The monocyte granules contain hydrolytic enzymes such as acid phosphatase, lysozymes, etc. In the tissues, they play an important role in nonspeci- fic defense against microbial invasion. Phagocytosis and microbial killing: Monocyte is an as reticuloendothelial system (the term has become active phagocyte: obsolete). Monocyte is the second line of defense against micro- their phagocytic activity by facilitating the recog- bial infections. Monocytes − The receptors also identify various sugar units on microbial membranes. The nucleus occupies half of the cell and remains − Monocyte is the second line of defense against eccentrically. Antigen presentation: Monocyte is an important anti- be horse-shoe shaped, round or irregular (Fig. Chronic myelomonocytic leukemia – Kupffer cell in liver – Osteoclasts in bone marrow 3. Postsplenectomy state – Red pulp macrophages in spleen – Macrophages in lymph nodes and thymus 7.

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However buy generic kamagra chewable 100mg on line, these symptoms are nonspeci c buy 100mg kamagra chewable overnight delivery, and the de nitive diagnosis of History of any of the following: asthma requires the presence of both typical respiratory – Cough buy generic kamagra chewable 100 mg on line, worse at night symptoms and signs and the clinical demonstration of – Recurrent wheeze variable expiratory air ow obstruction4 (Table 13. Symptoms occur or worsen in the presence of When assessing an individual with suspected asthma one – Exercise should weigh the clinical features that heighten or reduce the 4 – Viral infection probability of asthma. Conversely, – Smoke (tobacco, wood) features that reduce the probability that the symptoms are – Pollen due to asthma include the presence of an isolated cough – Changes in weather in the absence of other respiratory symptoms, a lack of – Strong emotional expression (laughing or crying improvement in symptoms following the administration of hard) antiasthma medications, such as inhaled bronchodilators – Airborne chemicals or dust or oral glucocorticoids, age of onset over the age of 50, – Menstrual cycles a signi cant smoking history (>20 pack years), chronic sputum production, dyspnea associated with dizziness, light- Symptoms occur or worsen at night, awakening the headedness and peripheral tingling, chest pain, syncope, and patient. Notably, symptoms brought on by is important to evaluate the current level of asthma control irritant-type exposures (e. It is important to empha- and changes in weather and airborne chemicals/dusts are size that determination of asthma control requires careful not speci c to asthma). Examination should focus severe asthma exacerbation) is seen only in a minority (7%) on the chest; however, evidence of chronic rhinitis (pale or of asthmatic individuals. Exercise-induced symptoms in asthma usually occur In atopic patients with asthma, eczema suggestive of atopic 5–15 minutes a er a short period of exertion or 15 minutes dermatitis is more commonly identi ed and may be evident, into exercise, and typically resolve with rest within an hour. To help con rm the diagnosis of asthma, it is impor- Inspection, palpation, and percussion of the chest is usu- tant to determine an individual’s predisposing risk factors. However, e medical history should therefore include questions to in individuals with long-standing, severe asthma, signs of determine whether the patient has a personal or family his- hyperin ation (e. Furthermore, enquiry evident and the expiratory phase of the respiratory cycle into childhood asthmatic symptoms should be made (e. Using the Asthma auscultation of the chest is wheeze, an adventitious, contin- Predictive Index, a combination of three or more episodes uous musical sound thought to be produced by oscillation of recurrent wheeze before the age of three, either phy- of opposing walls of an airway that is narrowed almost to sician-diagnosed eczema or a family history of parental the point of closure. It is important to document thoracic or intrathoracic), and the timbre (polyphonic or a thorough occupational history at the initial consultation, monophonic), as these features may help narrow the dif- as approximately 10% of adult-onset new cases are employ- ferential. However, the sensitivity and speci city of physi- ment related; in occupational asthma, the characteristic cal examination in determining the location and severity of symptoms in most cases are temporally associated with air ow obstruction is limited. However, wheeze may be heard during both the burden, poor asthma control, impaired quality of life, and inspiratory and expiratory phases, and this may suggest polypharmacy, which can lead to medication interactions. Furthermore, a detailed Indeed, the absence of wheezing in an asthma patient may medication and allergy history should be obtained, as indicate either improvement of the bronchoconstriction or asthma may be exacerbated by certain medications. Polyphonic Speci c enquiry into the frequency of asthma symptoms wheezing is con ned to the expiratory phase and com- (days per week), night waking, exercise limitation, and fre- prises multiple musical notes/pitches that start and end at quency of reliever use (excluding prior to exercise) should the same time (like a musical chord); it is typically pro- be determined. If the obstruction is rigid, then wheeze may be heard can be used to identify individuals who require more throughout the respiratory cycle, whereas if it is exible, it detailed assessment (Table 13. Direct question- Note: The “yes/no” responses are scored with 1 for each positive ing about asthma symptoms for 1 to 4 weeks prior to the answer giving a total score between 0 and 3. Obesity confers a significant the current therapeutic regimen, and the patient’s compre- negative impact upon the health status of individuals hension of it should be reviewed, including adherence, dos- with asthma and has been reported to be associated with ages, side e ects, inhaler technique, and e ectiveness of any poorer asthma control, impaired response to inhaled changes in the regimen at controlling symptoms. It should corticosteroid therapy, increased exacerbation fre- be noted that poor inhaler technique leads to poor asthma quency, increased healthcare utilization, and diminished control, increased risk of exacerbations, and increased asthma-specific quality of life relative to normal-weight adverse e ects, and that the majority of patients (up to 70%– asthmatics. Since, psychiatric disorders, treatment to be taken correctly as agreed upon by the patient particularly depression and anxiety disorders, are more and the health-care provider, and it is a common reason for prevalent among individuals with asthma, it is important poor asthma control. Approximately, 50% of individuals on to be alert to these conditions, especially where there has long-term therapy for asthma fail to take medications as been a previous history. In clinical practice, poor quently coexist with asthma and may contribute to poor adherence may be identi ed by an empathetic question that asthma control. Therefore, these conditions should be acknowledges the likelihood of incomplete adherence and assessed at each outpatient consultation with specific encourages an open discussion to address social, mental, questions that address nasal blockage, obstruction, ante- cultural, and economic barriers that may contribute to poor rior and posterior discharge, facial pain/pressure, and/or compliance. Respiratory viruses cause approximately 90% of asthma Following the initial assessment and determination of exacerbations, with rhinovirus being the most common asthma severity, the focused history performed in conjunc- culprit. Exacerbations are an important cause of asthma tion with initiation of appropriate therapy should prioritize mortality, and they result in frequent hospitalizations and the timing of onset and precipitating cause of the present considerable socioeconomic cost. It should be noted that an brief focused history and relevant physical examination asthma exacerbation thought to be occurring as part of an should be conducted concurrently with the prompt initia- anaphylaxis event requires prompt administration of intra- tion of therapy. It is important to note that in a minority of muscular epinephrine in conjunction with other therapies patients (predominantly male or patients with a history of being used to manage the asthma exacerbation. An asthma patient exacerbation should include assessment of vital signs (level who is experiencing a mild/moderate exacerbation is able of consciousness, temperature, pulse, heart rate, respira- to talk in sentences, prefers sitting to lying, and is not tory rate, blood pressure), signs of exacerbation severity agitated. However, this is o en di cult to evaluate plan as patients frequently swallow sputum rather than expec- – Food allergy in a patient with asthma torate it. Systolic paradox (pulsus paradoxus) is an dyspnea scale inadequate indicator of asthma severity and should not be assessed. It is important to note, as elbows (calluses or swollen bursas on the extensor surfaces with asthma, their absence does not exclude a diagnosis of of the forearms may even be seen). Use of the accessory ity of alternative diagnoses, such as ischemic heart disease, muscles of respiration, especially the sternocleidomastoids, pulmonary embolism, or bronchogenic carcinomas. Cigarette smoking is the major risk fac- spaces during inspiration (Hoover’s sign) may be present. As discussed, inspection of the chest may reveal an Following clinical assessment of the presenting com- increased antero-posterior diameter and Hoover’s sign. Hyperresonance may be observed upon in severe disease, but may also herald the development of percussion. Symptoms suggestive of the devel- sounds and di use, multiple monophonic wheezes due to opment of secondary pulmonary hypertension, such as small airways obstruction. Crackles or rales suggest con- ankle swelling, fatigue, dizziness, syncope, chest pain, and current infection. Finally, it is important for future planning that the social and family support networks available to the patient 13. It is important to note the frequency, severity, and likely causes of any exacerbations that may have occurred between consultations. Speci c inquiry into unscheduled dyspnea, and the presence of purulent sputum revealed dur- visits to providers, telephone calls for assistance, and use ing the course of an outpatient consultation should alert one of urgent or emergency care facilities is important. Naturally, increased sputum volume, worsening therefore, changes in social isolation and social support 13. Global stategy for the diagnosis, management, and prevention of chronic obstructive pul- right ventricular heave and pansystolic murmur may still be monary disease; 2016. Pattern of respiratory Symptoms may vary over Chronic usually continuous Respiratory symptoms including symptoms time (day to day, or over symptoms, particularly exertional dyspnea are longer periods), often during exercise, with persistent but variability may be limiting activity. History or family history Many patients have allergies History of exposure to Frequently a history of doctor- and a personal history of noxious particles and diagnosed asthma (current or asthma in childhood, and/ gases (mainly tobacco previous), allergies, and a family or family history of smoking and biomass history of asthma, and/or a asthma. Time course Often improves Generally, slowly Symptoms are partly but spontaneously or with progressive over years, signi cantly reduced by treatment, but may result despite treatment. Exacerbations Exacerbations occur, but the Exacerbations can be Exacerbations may be more risk of exacerbations can reduced by treatment. Aspirin mass fuel exposure, progressive deterioration, limited relief or other nonsteroidal in ammatory agent exac- from fast-acting bronchodilators, and severe hyperin ation erbated asthma. Near- fatal bronchospasm after oral nadolol in a young asthmatic and response to ventilation 13. Evaluation of wheezing illnesses other than for each condition should be evaluated. Targeting the interleukin path- Development and validation of a question- way in the treatment of asthma. Inhaler Characteristics and prevalence of asthma/chronic competence in asthma: Common errors, barriers obstructive pulmonary disease overlap in the United to use and recommended solutions.

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Downes and colleagues evaluated 12 families with codon 172 mutations; all demonstrated strik­ ing inter- and intrafamilial consistency in presentation and prognosis purchase 100mg kamagra chewable with visa. Finally order 100mg kamagra chewable with mastercard, van Lith-Verhoeven and coworkers evaluated families with autosomal dominant butterfly macular dys­ trophy and found evidence of genetic heterogeneity generic 100 mg kamagra chewable with visa. He is part of an one pedigree in which no atfccted patients reported vision extended pedigree carrying the diagnosis of pattern dystrophy. Large-caliber choroidal vessels arc visible in the region of atrophy, while fluorescence from the choriocapillaris is still present along the edges of the clinically apparent atrophy (bottom row). Note the sharply dem arcated lipofuscin figures in the m acula, more so in the left eye than the right eye. The fluorescein angiogram shows blockage of fluorescence centrally due to the lipofuscin accum ulation a s well a s stammg of som e of the lipofuscin deposits. When consid­ ering patients with reticular pigmentary changes, though, other clinically more common syndromes such as mito­ chondrial macular dystrophies associated with maternal diabetes and deafness should also be considered, especially from the standpoint of systemic disease intervention. Ann Intern atrophic peripheral changes in addition to the macular Med 2001;131:721-8. A pcriphcrin/rctinal degen­ eration slow m utation (Pro-210-Arg) associated with m acular and detachments and deposition of lipofuscin. Arch Using autofluorescence, Spaide described pathophysiologic Ophthalm ol 1996;114:448-56. Br J locus on chromosome 5 indicates that there must be geno­ O phthalm ol 2007;91:1504-11. Arch even within a single extended family with the same disease- O phthalm ol 1999; 117:1373-83. While the overall prognosis is fairly the norm al product o f the gene responsible for retinal degeneration in the rds mouse. Prog subfoveal choroidal neovascularization secondary to reticular pattern Retin Eye Res 2008;27:213-35. Acta O phthalm ol Scand 1997; m utations in peripherin/R D S in autosom al dom inant m acular 75:22-7. Visual outcom e following sub­ m aculatus in a single family with a deletion of codon 153 or 154 of retinal hem orrhage in Best disease. Arch Ophthalm ol ity associated with a novel m utation of the retinal degeneration slow/ 2001;119:359-69. Intrafamilial phenotypic in patients with Stargardt disease and fundus flavimaculatus. Br J Ophthalm ol heterogeneity of butterfly-shaped pigm ent dystrophy of the lovea. Clinical features of diabetes mel- phy: a gcnctic, clinical, and histopathological report. Deposition of yellow subm acular m aterial in central serous study of three generations. Although glaucoma is an optic neuropathy and hereditary optic neuropathy cases from across the globe. The energy demands of the photo receptors arc greater tumor compressing the optic nerve. Thus, in most cases, than the retinal ganglion cell, but some feature of the mito­ neuroimaging is necessary. It is particularly important to chondria at the lamina cribrosa makes them susceptible. A thorough family history is vital and does not end with the first clinical consultation. Patients (or par­ Molecular diagnosis and extended-family studies have ents) should be asked to find out whether anyone else in the increased the number of individuals with optic atrophy extended family had or has any form of visual loss. A history of 1/50,000 in the French' and the Finnish populations,7 prematurity, or problems in pregnancy or the neonatal period 1/39,000 in the Netherlands,8 1/31,000 in northeast may suggest perinatal optic atrophy, which is often not England,9 and 1/12,500 in Tasmania. Even when the cause of optic atrophy appears obvious, it is wise to perform neuroimaging studies on virtually all patients to exclude a compressive lesion. Numerous other authors described cases and L e b e r stellate neuroretinitis pedigrees, with the first major review by Bell (1931),26 in which she provided details on over 600 patients. Several changes were proposed as primary muta­ tions but later found to be related to mitochondrial haplotypes—for example the 15257 mutation, which was not 3460 3:1 3. The addi­ population of 1 million children under 18 years) found no tional feature of diabetes insipidus was reported by Raiti cases. Barbet and associates1'’ reported a consanguineous and colleagues,169 and nerve deafness by Shaw and family of French origin in which four sibs were affected Duncan. Examination showed optic atrophy ates172 proposed that an inherited abnormality of thiamine with adult visual acuity ranging from 6/30 to 6/60. Lcsscll and Rosma175 described anosmia, 311050) tonic pupils, and disc cupping (Fig. Swift and collcagucs,7,; 177also associated urinary tract atony, periph­ X-linkcd optic atrophy, ОРЛ2 has been mapped to Xpl 1. A Dutch kindred with eight males in seven sibships atric illness, while Rando and coworkers178 added ataxia, o f three generations (connected through females) had optic nystagmus, and seizures. Page and associates179emphasized atrophy of early onset, perhaps present at birth. Swift plantar reflexes, dysarthria, tremor, dysdiadochokincsia, and colleagues176,1 reported that 25% of homozygous and difficulty with tandem gait. Optic discs were sharply outlined and com ­ greater risk of severe psychiatric disturbance. Multipoint link­ diabetes mellitus and optic atrophy should be investigated age analysis placed the gene in the Xpl 1. Diabetes insipidus may be An American family with six males affected with optic confirmed by using a water deprivation test for 8 hours and atrophy in three generations was reported. Intravenous pyel­ individuals had decreased visual acuity from early ography, voiding cystourethrography, and ultrasound may childhood; visual acuities ranged from 6/9 to 6/30, and all reveal dilatation o f the urinary tract. Female carriers were clin­ widespread atrophic changes throughout the brain, some ically unaffected. No other neurologic abnormalities o f which correlate with the major neurologic features of the were observed in any family members. Optic atrophy in children with diabetes may also occur The extended disease haplotype of this family was distinct because o f the random overlap of two disorders, or may be from that of the family described by Assink and secondary to papilledema. Invest O phthalm ol Vis Sci with spccial rcfcrcncc to the associated color-sense disorder). J Neurol Sci m utations on m itochondrial m orphology and apoptosis: relevance 1978;38:11-21. Am J O ptom Arch Am responsible for autosom al dom inant optic atrophy and cataract.

Abramson reviewed 1 order kamagra chewable 100mg overnight delivery,265 patients cleft lip and/or palate in 60% to 80% purchase 100 mg kamagra chewable with amex, and abnormal exter­ with retinoblastoma 100mg kamagra chewable mastercard, and only 0. Capillary hemangiomas, in particular involving oplasia, and depigmentation of retinal pigment epithelium the forehead, as well as localized scalp dcfects in the parie- adjacent to the optic nerve extending toward the fovea in to-occipital area may occur. Polycystic kidneys occur in one third of patients, along with other Trisom y 13 (Patau Syndrom e) less common genitourinary abnormalities. Ninety percent of patients have abnormalities that can be detected on prena­ Trisomy 13 as discussed here refers to a complete or near- tal ultrasound. One study showed that 28% of surviving newborn infants die in the History first week of life, 44% within one month, and 86% in infan­ cy. Although there Bartholin described what was probably trisomy 13 in 1657, is a very high incidence of congenital heart disease, these but Patau and coworkers in 1960 were the first to recognize lesions are usually not lethal. One study suggested that pri­ the relationship of the clinical syndrome to trisomy 13. Epidemiology Ophthalmic Manifestations Trisomy 13 is the most common chromosomal abnormali­ ties associated with congenital ocular malformations at Ocular malformations are one of the cardinal signs of birth. Three anomalies are frequently severe and incompatible with recent studies suggest that the incidence of trisomy 13 in vision. Males and females arc affected with equal microphthalmia, as colobomatous microphthalmia is a well-recognized feature of trisomy 13. Dysembryogenesis of the anterior seg­ the phenotype of patients with full trisomy, and most ment is common, sometimes phenotypically consistent patients survive. Individuals with partial trisomy for the with Rieger anomaly or less commonly Peters’ anomaly. Cataract persist/4’ Hsu reported microphthalmia, coloboma, cata­ is common, with retention of the cell nuclei within the ract, glaucoma, and optic disc hypoplasia. Optic nerve hypoplasia bital ridges, slanting palpebral fissures, absent eyebrows, m a v also o c c u r. Congenital unilateral facial paralysis may rarely nal dysplasia with or without intraocular cartilage may occur. Schlade-Bartusiak and coworkers reported hearing loss, congenital inguinal hernia, joint Ring 13 contractures, cardiorespiratory anomalies, and gastroin­ Systemic Manifestations testinal and genitourinary abnormalities in patients with delL4q32. Deletion 14q However, the presence of hypotonia, choreoathetosis, sloping forehead, deeply set eyes, upturned nose, and Incidence everted upper lid in her affected child all suggest the effect Approximately 20 patients have been reported through of her dupl4p. More patients are described with terminal deletions as small with microcephaly, features known to be associated a result of ring formation. Systemic Manifestations Duplication 14q Pituitary hypoplasia, hypogonadism, and hypothyroidism Systemic Manifestations are features of patients with dell4q22-23. There was a prominent forehead, anteverted nares, bulbous nasal tip, Prader-Willi syndrome is characterized by neonatal hypoto­ low-set posteriorly rotated ears, micrognathia, and brachy- nia and feeding difficulties followed by obesity, develop­ dactyly with syndactyly of the second and third toes. The mental delay, and in some children hyperphagia that may be clinical findings in distal trisomy 14q22 qter are variable. Other features include They include growth retardation (77%), micrognathia small hands and feet, narrow bifrontal skull, and primary (72%), ear anomalies (63%), facial anomalies with prom i­ hypogonadism with cryptorchidism and small genitals. They do not demonstrate the obese pheno­ Ophthalmic Manifestations type seen in Prader-Willi syndrome. The child reported by North and colleagues had hyperopic astigmatism and bilateral iris colobomas. Hypertelorism Ophthalmic Manifestations was seen in 72% of patients with trisomy 14q22-qter. In Prader-Willi syndrome the palpebral fissures phenotype has not yet been identified. The typical manif­ maternal age, with the trisoinic chromosomc usually being estations include mental retardation, seizures, retinal of maternal origin. The syndromes may also result from uniparental disomy in children with Definition normal karyotypes. There may 141750) is a contiguous gene microdeletion syndrome due be intrachromosomal aberrations in the parental alleles to the del 16p 13. Other features Fewer than 20 cases have been reported, mostly secondary may include midfacial hypoplasia, capillary hemangioma, to familial balanced translocation. Scant eyebrows with sions lasting several seconds without loss of consciousness” hypoplastic superior orbital ridges and shallow orbits have that, in the absence of seizure activity on electroencepha­ also been reported. Narrow palpebral fissures that give the Ballif reported blepharophimosis with del 16p 11. One patient with a duplication of the whole (derived from a paternal inversion of 17p) or an unrecognized short arm of chromosome 17 exhibited respiratory dys­ mutation on the other allele. A slight upward slant of the palpebral fissures and growth retardation, club feet, delays in expressive speech, exotropia may also occur. High myopia with retinal detachment has been observed in non­ Ophthalmic Manifestations mosaic patients. Characteristics in the chil­ upper lip, dental anomalies, microcephaly, round face, dren included short stature, microcephaly, thin upper lip, hypertelorism, macrognathia, symphalangism, and low- highly arched palate, malaligned teeth, and mild mental set simple ears. A striking anomaly found in all three patients was the shortening of the metacarpal and metatarsal bones. Shimizu and coworkers Ophthalmic Manifestations described an infant with dup 17q24. The Almond-shaped palpebral fissures arc one of the cardinal patient had similar external abnormalities as the patients phenotypic features of the Hunter-McAlpine phenotype. At 8 years old the patients visual evoked potentials were consistent with a right optic nerve lesion. However, de Grouchy and colleagues congenital heart disease,119 club feet,150 and minor distal were the first to suggest that a locus for tapetoretinal degen skeletal anomalies,11^15" in particular cutaneous dimples in eration may be located on 18q. Warburg presence of a retinal dystrophy, traction retinal detachment is and associates felt that the retinal abnormalities were not not usually associated with del 18q. It must also be recognized attributable to pathologic myopia, which might indepen­ that Smith and coworkers326were unable to demonstrate the dently alter the clectrorctinogram. Ihe patient had Izquierdo and coworkers reported a child with esotropia, dcI18q21. An clectrorctinogram was of patients had fundus abnormalities, but cytogenetic not done. Warburg reported a patient who also had micro­ correlation and clinical details were not provided. Wilson visual loss may occur as a consequence of abnormalities and colleagues reported six children with terminal or subter­ in visual pathway myelination or gliosis when 18q22 is minal deletions sharing a dcll8q21. In four of the six seen in some patients with breakpoints for deletion in the patients, who ranged in age from 1 to 13 years, the fundi arc 18q21 region. One child had optic atrophy associated with 6 years this patient’s eye examination was normal following nystagmus. Choroidal retinal detachmcnt with tortuous retinal vessels and severe coloboma was present in one child with del 18q21 -qtcr. The felloweye had a “misshapen” Although the interpretation of many of the foregoing disc with nasal elevation and, on the provided figure, some eases may be limited by the accuracy of cytogenetic studies abnormalities of the nerve fiber layer and/or internal limit­ at the time of their publication,119 it appears reasonable to ing membrane of the macula arc apparent.

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Furthermore kamagra chewable 100mg sale, the term has even been extended to cipitant’ is simply one of degree generic kamagra chewable 100mg mastercard. In a recent survey buy kamagra chewable 100mg cheap, it was found include seizures that lead to the diagnosis of progressive conditions that 97% of patients with epilepsy believe that there is at least one such as tumours (primary and secondary) which are in fact ‘remote’ precipitant for some of their seizures, and 28% believe that there is symptomatic seizures’. In the metabolic conditions, arbitrary cut-of a precipitant for all of their seizures [44]. Te line between the two is For these reasons, in the author’s opinion, the classifcation of ep- not easy to defne, and to do so is to apply largely arbitrary criteria. If ring or artifcial stimulation of a certain receptor or group of recep- the term ‘acute symptomatic’ is to be retained, it should be restrict- tors, and a similar formulation was given by the 2001 glossary. In ed to the physiologically distinct ‘early seizures’ afer acute brain a recent textbook, the working defnition of refex epilepsy was ‘an injury. Acute seizures caused by metabolic disturbance or toxins epilepsy in which seizures are reliably provoked by a specifc identi- should be simply referred to as ‘provoked seizures’ [43]. Whatever term is used, it is important to point out that the ear- Currently, the refex epilepsies are commonly subdivided into ly seizures afer acute brain injury are quite diferent from the late two categories. In early seizures, the epilepsy may be caused 1 Simple refex epilepsies – where the seizures are precipitated by by contusions, haemorrhage, metabolic change, endocrine change, simple sensory stimuli (e. Tese are mechanisms that have nothing in tive epilepsy is by far the most common type and has been ex- common with the late seizures of post-traumatic epilepsy. Epilepsy in remission 2 Complex refex epilepsy – where the stimuli are more integrative Another important distinction for clinical practice is the diference and complicated. Examples include musicogenic epilepsy, in between epilepsy in which seizures are controlled on or of treat- which sometimes a highly specifc piece of music triggers the sei- ment, and epilepsy in which seizures continue despite treatment. Internal trig- Of course, the actual number of cases in remission depends on how gers, such as the efects of menstruation of fatigue, are not usually long the seizure-free period must be to qualify as a remission. All included in the category, nor are more indirect external triggers studies in this feld have shown that the longer the period of seizure such as alcohol intake. For most studies, remission has been defned as a 2 or 5-year period Defnition and classifcation – status without seizures. Te term ‘état de had an age-dependent epilepsy syndrome but are now past the mal’ though was coined by Calmeil in 1824 in his doctoral thesis, applicable age; or (ii) those who have remained seizure free for at where he notes it was used by patients in the Parisian asylums. Te least 10 years of antiseizure medicines, provided that there are no frst detailed modern medical description was by Bourneville in known risk factors associated with a high probability (>75%) of fu- 1869. However, as all three terms can be only demonstrated ret- Te frst major conference devoted solely to the topic of sta- rospectively, there is no practical diference between them. Te tus epilepticus was the Xth Marseille Colloquium, held in 1962 taskforce also recognized that diferent practical defnitions (e. A new defnition of status where a defnition was proposed: ‘Status epilepticus is a condition was proposed: ‘status epilepticus is a term used whenever a sei- in which epileptic activity persists for 30 minutes or more, caus- zure persists for a sufcient length of time or is repeated frequent- ing a wide spectrum of clinical symptoms, and with a highly var- ly enough to produce a fxed or enduring epileptic condition’. Although no duration was specifed in the defnition, Gastaut A detailed hybrid classifcation was proposed, which attempted later specifed a duration of 60 minutes to defne status epilepti- comprehensively to incorporate all types of status epilepticus, sub- cus. Another development of great importance coming from the divided by age, and also a group of conditions, termed ‘boundary colloquium was the concept, as Gastaut put it, that ‘there were as syndromes’ in which it was not clear to what extent these conditions many types of status as there were types of epileptic seizure’ [49]. Jacksonian) or general- stage of established status epilepticus (30–60/90 minutes: treated ized (e. Defnition (Terminology) and Classifcation in Epilepsy 21 was formulated so that there was no delay in initiating emergency are based on some evidence from animal experiments and (albeit therapy in patients with prolonged seizures [50]. Axis 1 (semiology) lists diferent forms of status Core Group of the Commission on Terminology and Classifcation epilepticus divided into those with prominent motor systems, those [25] in 2006 included status epilepticus in its listing of ‘seizure type’, without prominent motor systems, and currently indeterminate albeit in a rather incomplete fashion Table 1. Terminology produced a proposal for a new defnition of status epilep- without prominent motor features) was divided into those cases in ticus [51]: in which two time points are mentioned – the time point at coma and those not in coma. Axis 2 (aetiology) is divided into sub- which seizure activity can be considered continuous (t1) and the time categories of known and unknown causes. Finally, Axis 4 divides age groups into neonatal, infancy, child- epilepticus, both time points (t1 at 5 minutes and t2 at 30 minutes) hood, adolescent and adulthood, and elderly (Table 1. Known (synonymous: symptomatic) (c) Unknown whether focal or generalized (a) Acute (e. Location: generalized (including bilateral synchronous patterns), (a) Generalized lateralized, bilateral independent, multifocal i. Atypical absence status pattern duration and index, onset (sudden or gradual) and iii. Adolescence and adulthood (13–60 years) Boundary syndromes (currently indeterminate conditions) 5. Tis is perhaps because at one level it is and 1989 are amongst the organization’s greatest achievements. A the work of gardeners not botanists, and thus has been usually a number of general observations concerning classifcation and ter- matter of opinion (assertion) and not of fact. Te fare-up of pas- minology in epilepsy become apparent when an historical approach sions in 1969/1970, in 1989 and now in 2010–2014 are evidence is taken, and I end this chapter with a brief consideration of these. Everyone can have an opinion, and it seems that everyone What is abundantly clear from the historical perspective is that does, ofen from a limited viewpoint (the current author is guilty the state of knowledge in the feld of epilepsy is such that our cur- of this), and it is sad to see the intrusion of politics and personal rent and past classifcations schemes have been by necessity utilitar- opinions and vanities into a feld that should be dry and academic. Although much efort has been expended, ture, must win the approval of the community at large and cannot and with boring regularity unsubstantiated claims to the contrary be forced through on to unwilling recipients. To try to do so sim- have been made, we are as far as ever from being able to devise a ply causes confict, as has been evident on several occasions. First, there is a danger that, through a desire to be too However, this only works if the results of the consultation are heed- all-inclusive, the schemes become too complex and unwieldy, there- ed, as Gastaut found to his cost in 1969/1970. Given that they are gardening in tion schemes have also depended on an acceptance of the authority character and thus derive their worth entirely from their utility in of their authors, and the widespread respect that Jackson, Gastaut common practice, complexity is an enemy. Te failure of uptake and Dreifuss commanded was instrumental in the success of their of the 1989 Classifcation of the Epilepsies and Epileptic Syndromes schema. Let us hope a botanical scheme, fully sci- ity (indeed, on the contrary, it is of excellent quality) but due to entifcally justifed, based on such aspects as pathophysiology, neu- its complexity. Similarly, Gastaut’s summary classifcation of 1964 rochemical systems, or physiological or anatomical networks, and (Table 1. As knowledge advances, the feld becomes more complex, but the difcult trick for classifcationists is to maintain a balance between the Scylla of superfciality and the Charybdis of Acknowledgement intricacy. Of course, these ‘axes’, known then as ‘criteria’ or ‘parameters’, were used in all the previ- ous schemes, but were not there separated so conclusively as is the References current trend. On the anatomical, physiological and pathological investigation of are difcult to beat in this regard. London: Hodder and spuriously justifed as being needed by advances in science, should Stoughton, 1930: 162–172. International League Against Epilepsy – the second peri- Neurol 2011; 52: 541–547. Modern technology calls for a modern Against Epilepsy 1909–2009: A Centenary History. Oxford: Wiley Blackwell, 2009: approach to classifcation of epileptic seizures and the epilepsies.

The deep infrapatellar bursa is held in place by patellar tendon which is an extension of the common tendon of the quadriceps tendon (Fig cheap kamagra chewable 100mg line. Both the quadriceps tendon and its expansions as well as the patellar tendon and the deep infrapatellar bursa are subject to the development of inflammation caused by overuse 100mg kamagra chewable free shipping, misuse kamagra chewable 100 mg amex, or direct trauma. The quadriceps tendon is made up of fibers from the four muscles that comprise the quadriceps muscle: the vastus lateralis, the vastus intermedius, the vastus medialis, and the rectus femoris (Fig. The tendons of these muscles converge and unite to form a single, exceedingly strong tendon. The patella functions as a sesamoid bone within the quadriceps tendon, with fibers of the tendon expanding around the patella and forming the medial and lateral patella retinacula, which help strengthen the knee joint. These fibers are called expansions and are subject to strain; the tendon proper is subject to the development of tendinitis. The deep infrapatellar and prepatellar bursae also may concurrently become inflamed with dysfunction of the quadriceps tendon. The deep infrapatellar bursa is held in place by patellar tendon which is an extension of the common tendon of the quadriceps tendon. The quadriceps tendon is made up of fibers from the four muscles that comprise the quadriceps muscle: the vastus lateralis, the vastus intermedius, the vastus medialis, and the rectus femoris. The bursa serves to cushion and facilitate sliding of the patellar tendon over the tibia. The bursa is subject to inflammation from a variety of causes with acute trauma to the knee and repetitive microtrauma being the most common. Acute injuries to the bursa can occur from direct blunt trauma to the anterior knee from falls onto the knee as well as from overuse injuries including running on uneven or soft surfaces or 937 jobs that require kneeling or crawling on the knees like carpet laying and scrubbing floors. If the inflammation of the bursa is not treated and the condition becomes chronic, calcification of the bursa with further functional disability may occur (Fig. Gout and other crystal arthropathies may also precipitate acute deep infrapatellar bursitis as may bacterial, tubercular, or fungal infections. Lateral radiograph of the right knee showing an osseous mass attached to the tibial tubercle. The mass extends to the inferior aspect of the patella and is surrounded by several ossified fragments. Infrapatellar bursal osteochondromatosis associated with unresolved Osgood-Schlatter disease. Physical examination of the patient suffering from deep infrapatellar bursitis will reveal point tenderness over the anterior knee. If there is significant inflammation, rubor and calor may be present and the entire area may feel boggy or edematous to palpation. At times, massive effusion may be present which can be quite distressing to the patient (Fig. Active resisted extension and passive flexion of the affected knee will often reproduce the patient’s pain. If calcification or gouty tophi of the bursa and surrounding tendons are present, the examiner may appreciate crepitus with active extension of the knee and the patient may complain of a catching sensation when moving the affected knee, especially on awaking. Occasionally, the deep infrapatellar bursa may become infected, with systemic symptoms, including fever and malaise, as well as local symptoms, with rubor, color, and dolor being present. Effusion associated with deep infrapatellar bursitis can be appreciated by displacing the patella. Based on the patient’s clinical presentation, additional testing may be indicated, including complete blood cell count, sedimentation rate, and antinuclear antibody testing magnetic resonance imaging or ultrasound imaging of the affected area may also confirm the diagnosis and help delineate the presence of other knee bursitis, calcific tendinitis, tendinopathy, triceps tendinitis, or other 938 knee pathology (Fig. Rarely, the inflamed bursa may become infected and failure to diagnosis and treat the acute infection can lead to dire consequences. Radiograph demonstrating high-energy tibial plateau fractures include primary fracture lines that involve both tibial condyles, severe impaction and comminution of one or both articular surfaces, and fracture extension into the shaft. A,B: Magnetic resonance images of the knee showing abnormal mass in the infrapatellar region consistent with deep infrapatellar bursitis. A linear high frequency ultrasound transducer is placed over the previously identified patella in a longitudinal orientation (Fig. A survey scan is taken which demonstrates the hyperechoic margin of the skin and subcutaneous tissues, the superficial infrapatellar bursa, the patellar tendon, and the deep infrapatellar bursa beneath it (Fig. After the skin and subcutaneous tissues, the patellar tendon, and the deep infrapatellar bursa are identified, the bursa is evaluated for enlargement, inflammation, crystals, rice bodies, hemorrhage, and infection (Figs. The patella and patellar tendon are then evaluated for abnormalities including infections, anatomic abnormalities, and fracture (Fig. Color Doppler may help identify neovascularization and hyperemia associated with patellar tendinopathy. Correct longitudinal position for ultrasound transducer for ultrasound evaluation of the deep infrapatellar bursa. Longitudinal ultrasound image of the knee joint demonstrating the deep infrapatellar bursa lying beneath the patellar tendon. Longitudinal ultrasound image of the knee joint demonstrating deep infrapatellar bursitis. Longitudinal ultrasound image demonstrating bursitis of the deep infrapatellar bursa. The osteophyte just distal to the bursa may be serving as the nidus for the inflammatory process. Longitudinal ultrasound image demonstrating a moderately large deep infrapatellar bursitis. The use of ultrasound guidance can simplify needle placement when injecting or aspirating the deep infrapatellar bursa. The pes anserine bursa lies between the combined tendinous insertion of the sartorius, gracilis, and semitendinosus muscles and the medial tibia (Fig. The bursa is subject to the development of inflammation after overuse, misuse, or direct trauma (Fig. The medial collateral ligament often also is involved if the medial knee has been subjected to trauma. The medial collateral ligament is a broad, flat, band-like ligament that runs from the medial condyle of the femur to the medial aspect of the shaft of the tibia, where it attaches just above the groove of the semimembranosus muscle (Fig. The medial collateral ligament is crossed at its lower part by the tendons of the sartorius, gracilis, and semitendinosus muscles. Axial (A) and sagittal (B) fat-suppressed proton density-weighted images demonstrating an inflamed pes anserine bursa (arrows) along the medial tendons. The medial collateral ligament is a broad, flat, band-like ligament that runs from the medial condyle of the femur to the medial aspect of the shaft of the tibia, where it attaches just above the groove of the semimembranosus muscle. The pes anserine bursa lies beneath the pes anserine tendon, which is the insertional tendon of the sartorius, gracilis, and semitendinosus muscles to the medial side of the tibia (Fig. The bursa serves to cushion and facilitate sliding of pes anserine tendon over the tibia. The bursa is subject to inflammation from a variety of causes with acute trauma to the knee and repetitive microtrauma being the most common. Acute injuries to the bursa can occur from direct blunt trauma to the medial knee as well as from overuse injuries including running on hills or sudden increases in the distance that one runs. If the inflammation of the bursa is not treated and the condition becomes chronic, calcification of the bursa with further functional disability may occur (Fig.

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