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Methotrexate therapy has been proven to achieve remission in 69% to 71% of sufferers. At initial doses of as a lot as 5 mg kg�1 day�1, cyclosporine showed efficacy, however when the dose was lowered to 1 to 2 mg�1 kg �1day, mild disease flares occurred. Eighty-two percent of forty one patients remained in remission at 24 months, in contrast with 60% of sufferers in the placebo group. In contrast, all sufferers who obtained trimethoprimsulfamethoxazole and prednisone experienced a relapse after a median of 14. In the group that obtained trimethoprim-sulfamethoxazole with out prednisone, 58% remained in remission, compared with 86% of the sufferers who obtained methotrexate alone. Corticosteroids were famous to be ineffective if progression to serious pulmonary or renal involvement had already occurred. Azathioprine,75 chlorambucil,72,seventy six and methotrexate77 have been efficient in some instances. Oral prednisone is administered at 1 mg kg�1 day�1 until the immunosuppressive effects of the cyclophosphamide are apparent. At this level, the prednisone is switched to alternate-day remedy and is tapered. There are a quantity of medical reports of efficacy of infliximab in achieving remission or bettering disease exercise. Despite continued infliximab, 20% of the preliminary responders experienced illness flares. There are anecdotal stories of successful use of Rituximab to achieve and maintain remission. Necrotizing scleritis could occur after current local ocular trauma or surgical procedure in the absence of activation of systemic illness. Systemic cyclophosphamide and prednisone therapy are warranted in these circumstances, because this process is sight threatening. Tectonic scleral grafting is indicated in instances of impending perforation of the globe. Elective surgical procedure such as cataract extraction is contraindicated for sufferers with active disease, as a result of catastrophic woundhealing problems are likely to ensue. Of observe, 25 of the 79 patients who achieved remission suffered a relapse, normally as cyclophosphamide was tapered. In all but certainly one of these sufferers, remission was promptly reinduced with cyclophosphamide and prednisone therapy. Patients were warned of the premonitory signs of relapse similar to elevation of the erythrocyte sedimentation fee, arthralgia, malaise, and skin lesions, and relapses were famous and handled early, thus avoiding significant organ injury. One of the more recent long-term end result studies adopted one hundred fifty five patients for a median of 7 years. Significant predictors of greater mortality at prognosis had been age over 50 years, impaired renal perform, and lung involvement. Poor prognostic components had been a serum creatinine of greater than one hundred sixty mm/L, age over fifty two, and absence of ear, nostril, and throat involvement. In another study of 108 sufferers with active renal illness, the mean follow-up time was 41. Patients with orbital cellulitis or pseudotumor related to contiguous sinus illness require antibiotics and appropriate drainage procedures and must be treated in session with an otolaryngologist. Patients with proptosis may require orbital decompression to protect optic nerve operate or for the remedy of secondary diplopia. Preoperative (d) and postoperative (e) images of this similar affected person, who subsequently underwent cataract extraction in the other eye whereas receiving maintenance therapy of cyclophosphamide and prednisone. Early diagnosis and therapy with cyclophosphamide appear to be the key to avoiding irreversible end-organ injury corresponding to renal failure, ocular perforation, cardiomyopathy, and subglottal stenosis of the airway. Some of the problems of remedy, corresponding to opportunistic respiratory tract an infection and hematuria, may be manifestations of the disease itself. Deciding whether or not hematuria, for example, represents nephritis or cyclophosphamide toxicity presents a major challenge to the physician, with severe penalties for the patient. Wegener F: Uber ein eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. New elements of the disease course, immunodiagnostic procedures, and stageadapted remedy. Nolle B, Specks U, Ludemann J, et al: Anticytoplasmic autoantibodies: Their immunodiagnostic value in Wegener granulomatosis. Csernok E, Ernst M, Schmitt W, et al: Activated neutrophils categorical proteinase 3 on their plasma membrane in vitro and in vivo. Tomer Y, Gilburd B, Blank M, et al: Characterization of biologically lively antineutrophil cytoplasmic antibodies induced in mice. Harper L, Cockwell P, Adu D, et al: Neutrophil priming and apoptosis in antineutrophil cytoplasmic autoantibodyassociated vasculitis. Tuso P, Moudgil A, Hay J, et al: Treatment of antineutrophil cytoplasmic antibodypositive systemic vasculitis and glomerulonephritis with pooled intravenous gammaglobulin. Stephen Foster Scleroderma is an acquired connective tissue illness of unknown trigger. However, visceral involvement can occur at any stage of the illness and infrequently occurs independently of the pores and skin illness. The prognosis of scleroderma is made on scientific grounds, based mostly on criteria proposed by the American Rheumatism Association (Table 327. Another proposed classification divides the disease into subsets according to skin involvement, scientific associations, nail-fold capillary changes, and autoantibodies. From the Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee: Preliminary standards for the classification of systemic sclerosis (scleroderma). Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy. The disease is alleged to be more severe in young black girls and native Choctaw Indians in Oklahoma, who are probably to purchase diffuse scleroderma. The irritation and thickening predominate within the fascia however are also present in the pores and skin, subcutis, and muscle. Primary pulmonary hypertension and first biliary cirrhosis are additionally extra likely to develop in these patients. In linear scleroderma, a single extremity or the face might undergo sclerotic induration and hyperpigmentation. Initially, the skin is concerned, but the underlying subcutaneous fats, muscle, and bone could turn out to be atrophic as nicely. Linear scleroderma normally happens in children or young adults and is energetic for 2�3 years; the later scientific course is dictated by the quantity of atrophic modifications. Linear scleroderma of the face (coup de sabre) can cause facial asymmetry and disfigurement. Morphea, another type of localized scleroderma, itself represents a selection of scientific subtypes and is characterized by sclerodermatous skin induration, which may happen in discrete spots or in bigger patches.

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History the presence of neurological disorder, developmental delay, and hereditary metabolic ailments ought to be elicited. A household historical past of nystagmus, and the timing of onset must be sought from the parents. Any history of refractive treatments, and medical or surgical interventions ought to be elicited. In an older child, questions concerning the presence of oscillopsia, worsening of nystagmus in any particular gaze, and the presence of dizziness should be asked. The household must also be questioned whether or not the kid turns or tilts his or her head. Measurement of Nystagmus Waveform In order to characterize nystagmus it may be very important measure eye position and velocity as nicely as target position during tried fixation in different gaze angles. Anterior Segment Examination Clinical Examination Any abnormality of head posture ought to be noted. Visual acuity, colour vision, stereopsis, and visible fields ought to be examined, and dilated funduscopic examination should be carried out. Examination of the pupils is a crucial a half of the work-up for each nystagmus patient. Bilaterally sluggish pupils or presence of relative afferent pupillary defect suggests anterior visual pathway illness. Iris transillumination defects are universally current in oculocutaneous albinism. The macula ought to be assessed for normal pigment and vascular pattern surrounding a well-delineated fovea. Lack of a traditional foveal reflex is indicative of macular hypoplasia, as in ocular albinism. Different strategies for recording eye movements are described in great element elsewhere. Once optical management is maximized, the key precept of management of nystagmus patients is to optimize visible potential by stabilizing the retinal picture, which will result in enchancment in visible acuity via increase in the foveation time. Visionenhancing changes at college embrace orienting the chalkboard, trainer, and television within the favored course of gaze for the child. Neuroimaging Infants and children with nystagmus and reduced imaginative and prescient with irregular optic nerves will need neuroimaging. Atypical congenital waveforms corresponding to seesaw nystagmus and periodic alternating nystagmus also needs to bear neuroimaging. Children in whom the timing of onset of nystagmus is uncertain should have neuroimaging carried out to exclude any structural lesion in the mind. Examination beneath anesthesia could also be required in some patients to adequately evaluate ocular constructions within the work-up of childish nystagmus. Careful cycloplegic refraction and prescribing glasses to maximize visible potential is the initial step in the management of kids with congenital nystagmus. In kids in whom the nystagmus damps with convergence, prescribing an overminus correction will stimulate accommodation and convergence and will cut back the amplitude of nystagmus and improve visual operate. In patients with latent nystagmus, binocular refraction with fogging of 1 eye could also be employed to optimize the subjective visible acuity refinement procedure. Contact Lenses Some patients with congenital nystagmus experience damping of nystagmus and consequent improvement in visual acuity when utilizing contact lenses. This concept is supported by findings that topical anesthesia abolishes this phenomena. Base-out/base-in prisms to improve visible acuity by stimulating vergence eye movements. Eye Movement Recordings As a research device, eye motion recordings are invaluable. Recordings performed over a quantity of decades have led to outstanding advances in the understanding of the physiology of regular and irregular eye movements. In congenital nystag- Correction of Head Posture Prisms minimize head flip by reorienting the road of sight toward the first gaze. Prisms help to redirect the road of sight so that the head can assume a more natural relationship with the surroundings. So for example, in a affected person who has a head turn to the left and most well-liked gaze to the proper the prisms are oriented with apices within the path of the popular gaze. Similarly in patients with downbeat nystagmus, the prism is oriented base down which shifts the picture up and due to this fact decreases the oscillopsia. The disadvantage is that they blur the image, are troublesome to clear, and additional degrade the standard of the imaginative and prescient. They are, nonetheless, inexpensive and may be suitable for older people and in patients as a therapeutic trial. In the adducted eye, a recession of the medial rectus muscle is coupled with a resection of the lateral rectus muscle. After the surgical procedure, in addition to the anticipated shift of the null zone toward major place, the breadth of the null region increased and the overall nystagmus intensity is decreased. It is believed that there are two mechanisms that contribute to the effectiveness of this surgical procedure. The expected null-shift toward main position is mechanical as a result of the efficient rotation of the globe opposite the null angle and the next innervation required to transfer the eyes again to major place. In addition to the resections and recessions it was hypothesized that the accompanying tenotomies of the muscle tissue resulted in damping of congenital nystagmus in any respect gaze angles. Improvement of Visual Acuity Convergence prisms present an optical strategy for patients with congenital or acquired nystagmus in whom the nystagmus damps after they view a near target. Kestenbaum Anderson procedure (classic maximum) the surgical dosage tips for this process were put forth by Parks and are the most accepted pointers today. A 5 mm recession of medial rectus muscle was coupled with an 8 mm resection of the ipsilateral lateral rectus muscle, the man eye undergoes 7 mm recession of the lateral rectus muscle coupled with a 6 mm resection of the medial rectus. One drawback of the procedure is that undercorrections generally happen in patients with more than 30� of head flip. In 56% of the eyes, the postoperative uncorrected visual acuity was higher than one of the best spectacle-corrected visible acuity. This technique has been used with restricted success in patients with acquired nystagmus such as oculopalatal myoclonus. Ptosis is discovered to be much less common with retrobulbar injections as in comparability with intramuscular injections since the intraconal injection is directed away from the muscle cone. These include 10 mm surgical procedure on all horizontal muscles advocated for higher than 35� of head turn. The common consensus in the type of procedure to carry out appears to be to do a traditional procedure for head turns of 20� or less and to use 40% augmentation for most sufferers with larger head turns.

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At the height of cycloplegia, the kid is introduced, with the mother or father, into a darkened room. Having the child fixate a distant goal may be the greatest way to remove any lodging, however can cause errors in studying astigmatism axis. Therefore many practitioners choose to make the room as darkish as potential to give the child just one possible target to fixate: the retinoscope mild. If the child has both latent or manifest strabismus, you will want to cowl one eye while retinoscoping the opposite to keep away from having the child switch fixation, creating an off-axis studying. They could also be scared of it, but extra commonly, they squirm and turn their head and eyes whereas sitting behind it, making it tough for the examiner to tell whether or not the child is basically fixating centrally. For most young kids, the most correct measurements will be obtained using loose lenses with the retinoscope. This methodology theoretically allows the child an unobstructed view to a goal on the finish of the room for fixation. An alternate methodology which insures that the child is fixating on axis, even when strabismus is current, is for the examiner to maintain the retinoscope over the proper eye with the right hand, holding the trial lens with the left hand, to retinoscope each eye. It has been proven that even when the optic nerve is cut, lid suture myopia will happen. This points to native factors within the retina as being concerned, elements triggered by light and kind. Atropine has been famous to retard experimental myopia, once more even when the optic nerve is severed. Thus a complex system of chemical mediators and receptors is concerned in each regular and disordered axial elongation. Because of their massive accommodative capacity, noncycloplegic retinoscopy and/or manifest refraction in kids typically measures refractive error combined with accommodative reserve, resulting within the prescription of incorrect lenses. Noncycloplegic refraction in kids tends to end in undercorrection of hyperopia and astigmatism, and overcorrection of myopia. In addition, even very brilliant cooperative youngsters could not have the power to adequately participate in manifest refraction, requiring an objective measurement by retinoscopy. In common, a cycloplegic refraction must be carried out as a half of the preliminary ophthalmologic examination on each youngster 9 years of age or much less. Because of the rapid modifications in anatomy throughout this stage, children followed for continual issues ought to have cycloplegic refraction no much less than annually. Even youngsters and adults lower than forty five years of age may be over- or undercorrected with manifest refraction alone because of uncontrolled accommodation. Therefore, cycloplegic refraction on all sufferers at initial go to (which could also be delayed if presenting for trauma, iritis, etc), then a minimal of yearly for youngsters less than 9 years, and on a case-bycase basis for all others depending on diagnosis, signs, and signs is beneficial. In youngsters already carrying spectacles or contact lenses, especially if the prescription is of excessive power, an overrefraction could also be carried out. This technique eliminates the error introduced by vertex distance with high prescriptions. If further cylinder at an axis completely different from the original is discovered, confirmation with unfastened lenses alone is really helpful. When attainable, a subjective refinement ought to be carried out, however that is hardly ever useful in kids lower than 7or 8 years old, and is type of never useful at the first visit if amblyopia is current. After dilation and retinoscopy if the child is cooperative a subjective refinement can be tried, and repeated on subsequent visits under noncycloplegic situations. Most of the time younger children must be prescribed spectacles based on retinoscopy alone. Some kids, for example those with developmental delay, could additionally be unable to cooperate with an entire eye examination and may require examination beneath anesthesia. Children break their glasses incessantly, and parents might not remember whether this has occurred since the final go to. Although refractive errors rarely trigger asthenopia or headache, treatment of gentle refractive points may typically be recommended to rule this out as a cause. Thus, if an toddler has a very massive refractive error that would preclude useful macular vision, optical correction should be supplied promptly, to be in place during the crucial period of vision improvement from start to 3�4 months of age. Some infants have very giant refractive errors in the aphakic vary even without aphakia. Nanophthalmic eyes, eyes that had extreme retinopathy of prematurity, eyes with pathologic myopia because of Stickler syndrome or other connective tissue disorders, could have hyperopia or myopia of such giant degree that optical correction is mandatory within the first three months of life. In basic, if hyperopia or myopia is greater than 10 D, immediate correction ought to be considered in the neonatal period to prevent irreversible amblyopia. Nystagmus is often seen if myopia is within the 15�20 D range, and bilateral amblyopia has been reported with hyperopia of more than four. Hyperopia might quickly enhance in neonates; very high myopia above 10 D is unlikely to improve much. However for a newborn, whose main visible stimulus is a face close to its own, and meals close to its mouth, even pretty excessive amounts of myopia permit normal visual growth. Refractive Errors in Children the commonest signal caused by hyperopia is esotropia, or crossed eyes. The effort of accommodating triggers the close to synkinesis response of lodging and convergence, causing the eyes to deviate inward as if viewing a near target. The esotropia is commonly intermittent at first, mainly when drained or unwell, then turns into extra fixed. It may be more evident with near duties, where more lodging is required, than for distance, or it might be equal distance and near. This ratio describes the amount of convergence triggered by a given quantity of accommodation. In some youngsters, even a small quantity of hyperopia with its attendant extra accommodation induces crossing. If the crossing persists at close to regardless of full cycloplegic refraction, an add ought to be given. The aim is to reduce the power of the add to the point that a small, well-controlled esophoria exists. This is prescribed, then rechecked 6 months later to see if additional discount is possible. If diplopia or crossing is famous after prescription of the decrease add, the previous energy must be reinstituted. It is often useful to ask the parents or optical shop not to discard any lenses throughout this course of, since some children may must go again and forth. Many kids can be weaned from an add and obtain straight eyes with good stereopsis by age 9 years. One research discovered that the best predictor of a kid with hyperopia and accommodative esotropia efficiently weaning out of spectacles was a refractive error spherical equal of lower than three D at presentation. Eyes with very high hyperopia could not accommodate absolutely, so, paradoxically, kids with severe hyperopia may cross less than those with gentle amounts. Even young children can respect improved imaginative and prescient and can readily maintain spectacles on if they assist. If the kid refuses to put on the glasses after every week or so of repeated makes an attempt, it may help to cut back the prescription, allowing the child to accommodate an quantity acceptable for age, often 1 or 2 D.

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Although not particularly stained for elastin, the brightly eosinophilic fragmented elastic lamina may be recognized across the giant cell. In these circumstances, the ophthalmologist could be the physician to provoke and monitor corticosteroid therapy. These sufferers should be adopted intently at the side of an internist or rheumatologist. Although mortality charges of big cell arteritis patients are comparable to these of the overall population, the morbidity of the illness (and its treatment) is important. In spite of the quantity of literature reported on the disease, it stays enigmatic. Family physicians, rheumatologists, and ophthalmologists should be aware of its myriad, and often insidious, displays. Ancillary tests, including temporal artery biopsy, are weighed in the decision-making process, however none should override a robust clinical suspicion. Nordborg C, Johansson H, Petursdottir V, et al: the epidemiology of biopsy-positive big cell arteritis: particular reference to changes in the age of the inhabitants. Salvarani C, Cantini F, Boiardi L, et al: Polymyalgia rheumatica and giant-cell arteritis. Dummer W, Zillikens D, Schulz A, et al: Scalp necrosis in temporal (giant cell) arteritis:implications for the dermatologic surgeon. Petursdottir V, Nordborg E, Nordborg C: Atrophy of the aortic media in big cell arteritis. Imakita M, Yutani C, Ishibashi-Ueda H: Giant cell arteritis involving the cerebral artery. Pego-Reigosa R, Garcia-Porrua C, Pineiro A, et al: Predictors of cerebrovascular accidents in big cell arteritis in an outlined population. Liozon E, Herrmann F, Ly K, et al: Risk components for visual loss in big cell (temporal) arteritis: a prospective examine of 174 sufferers. Valmaggia C, Speiser P, Bischoff P, et al: Indocyanine green versus fluorescein angiography within the differential diagnosis of arteritic and nonarteritic anterior ischemic optic neuropathy. Ahmad I, Zaman M: Bilateral internuclear ophthalmoplegia: an preliminary presenting signal of big cell arteritis. Bandini F, Benedetti L, Ceppa P, et al: Uveitis as a presenting sign of big cell arteritis. Miller A, Green M, Robinson D: Simple rule for calculating regular erythrocyte sedimentation rate. Liozon E, Roblot P, Paire D, et al: Anticardiolipin antibody ranges predict flares and relapses in patients with giant-cell (temporal) arteritis. Taylor-Gjevre R, Vo M, Shukla D, et al: Temporal artery biopsy for big cell arteritis. Stephen Foster and Jean Yang otitis, ulcerations in the oral cavity, nasolacrimal duct obstruction, orbitopathy, conjunctivitis, keratitis, scleritis, or uveitis. Klinger first described this disease as a particular form of polyarteritis nodosa in 1931. It is considered a hypersensitivity disorder as a end result of granulomatous inflammation, tissue eosinophilia, vasculitis, and glomerulonephritis are all characteristic of hypersensitivity states. This idea of hypersensitivity as a pathogenic issue is supported by a study of prevalence of allergic reactions in sufferers with systemic vasculitis, which revealed that forty five of 60 patients (73%) had a history of at least one sort of allergy, which was significantly higher than in control topics. Hypersensitivity to inhaled, maybe microbial, pathogens has been postulated as the trigger of the illness. The latter form correlates with the classically defined disease of Godman and Churg whose traditional diagnostic triad of morbid pathologic findings4 are listed in Table 326. Necrotizing granulomatous irritation and vasculitis are seen in the concerned tissue. Vasculitis Involves each arteries and veins and is of a focal, necrotizing variety; is granulomatous in nature, featuring multinucleated giant cells; usually seen in lung tissue and variably present elsewhere three. Most patients initially presented to their physicians with upper airway sicknesses. They identified that both of these findings would strongly point out one other localized midline destructive disease such as midline granuloma or a midline neoplasm. Secondary infection was extremely common in sufferers with upper airway illness, Staphylococcus aureus being the predominant organism in every case. Renal disease, as quickly as current, may progress rapidly and is associated with poorer prognosis regardless of cytotoxic therapy. Some of these findings may be triggered by seemingly trivial local trauma, as can the rheumatoid nodules found on extensor surfaces in rheumatoid arthritis. Acute pericarditis was the most common form of cardiac illness, with carditis, dilated congestive cardiomyopathy, cardiac valve involvement, and heart block additionally seen. Arthralgia or arthritis, fever, otitis (usually associated to eustachian tube obstruction), and cough were a presenting sign in at least 25�50% of patients. There was ocular inflammation in 16% of patients at presentation; 7% of sufferers had proptosis as a presenting sign. Computed tomographic scan displaying a mass at the left orbital apex in a sixty nine 12 months old girl who introduced with progressive left-sided proptosis, ophthalmoplegia, optic neuropathy, and malaise. The mass prolonged to the cavernous sinus, and there was opacification of the sphenoid sinus. Orbital socket contracture could be a complication of inflammatory orbital illness, which can result in enophthalmos, restrictive ophthalmopathy, continual orbital ache, and optic nerve illness. An association with sublgottic stenosis was found with tarsal-conjunctival illness. Localized conjunctivitis or episcleritis usually occurs earlier than the onset of peripheral ulcerative keratitis or scleritis. The corneal component begins with the event of intrastromal, peripheral, corneal inflammatory infiltrates. The crescentic peripheral corneal ulcer progresses each centrally and circumferentially. The affected person had a history of scleritis and renal, sinus, and central nervous system involvement but had been in remission for 8 years after cyclophosphamide remedy. At the time of therapeutic conjunctival resection, necrotizing scleritis was noted. Systemic cyclophosphamide therapy was initiated for this sight-threatening course of. A research of 47 sufferers treated at the Massachusetts Eye and Ear Infirmary revealed that 57. Abnormal urinary sediment (red-cell casts or 5 red blood cells per high-power field). Abnormal findings on a chest radiograph (nodules, cavities, or fixed infiltrates). Using classification-tree analysis, they found that granulomatous irritation seen on biopsy samples separated the cases from the management subjects higher than another criterion; they cautioned that cultures and special stains must be used to rule out infectious causes of granulomatous irritation, similar to acid-fast bacilli and fungi. By utilizing immunohistochemical analysis, markers for eosinophil granule proteins (eosinophil major fundamental protein and eosinophil cationic protein) were tested. From this study, one might hypothesize that activated eosinophils in conjunctiva or sclera could additionally be a harbinger of illness progression.

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If the lesion is partial, the resultant hemianopia is incongruous because of unequal involvement of the alternating layers of neurons subserving the two visible fields. Superior quadrantic visual-field defects occur in patients with temporal lobe illness as a outcome of fibers within the temporal lobe carry data from the inferior retina. The visual-field defects are characteristically incongruous as a result of fibers carrying information from identical factors within the two visible fields are spatially separated. Incongruous, homonymous, wedge-shaped defects within the higher visible fields virtually always indicate involvement of the temporal lobe. Modified from Frisen L, Holmegaard L, Rosencrantz M: Sectorial optic atrophy and homonymous, horizontal sectoranopia: a lateral choroidal artery syndrome J Neurol Neurosurg Psychiatry 1978; 41:374; and Frisen L: Quadruple sectoranopia and sectorial optic atrophy: a syndrome of the distal anterior choroidal artery. Involvement of either temporal lobe could cause uncinate seizures characterized by an aura of surprising style or smell along with involuntary movements of the mouth and auditory or fashioned visual hallucinations. Usually, a significant quantity of temporal lobe should be removed before vision is affected. However, in patients with incomplete area defects, inattention may be distinguished from visual-field loss by use of double simultaneous stimulation. A useful oculomotor signal of parietal lobe dysfunction is loss of the traditional optokinetic responses. On event, sufferers with parietal lobe disease have normal visible fields but asymmetric optokinetic responses. Another oculomotor sign seen in sufferers with parietal illness is conjugate deviation of the eyes. The first is sustained, involuntary gaze deviation occurring spontaneously towards the facet of frontoparietal lesions. Most occipital lesions, including those affecting the most posterior parts of the occipital lobe, are inclined to spare the central 5� of the visible area (macular sparing). With bigger occipital lesions, a quadrantic defect may be detected, typically respecting the horizontal meridian as well as the vertical meridian. However, the phenomenon of statokinetic dissociation has subsequently been found to occur in lesions situated elsewhere in the visible pathways. Although most affected patients are conscious of an issue with their visual-field loss, some sufferers could also be unaware of the defect till they begin bumping into objects or having car accidents. Others could only turn out to be conscious of preexisting visible loss when they lose imaginative and prescient within the contralateral visible subject. Visual acuity may appear to be lowered in some patients with unilateral occipital illness in whom splitting of fixation occurs or in whom a quadrantic defect encroaches on fixation, however even with full, maculasplitting hemianopias these sufferers have preserved Snellen acuities. Some sufferers also have issue with reading, although their visible acuity and their ability to write are preserved. Tumors Affecting the Posterior Visual Pathways102,103 Astrocytomas � Affect sub-cortical white matter of the cerebral hemisphere is more generally in adults (as opposed to optic nerve, cerebellum and brain stem astrocytomas in children). Oligodendrogliomas � Affect retrochiasmal visual pathways more usually than astrocytomas. Meningiomas � Occasionally affect the posterior visual pathways in center aged and aged patients. Metastatic Carcinoma � 40% of cerebral metastases may occur without a pre-existing diagnosis of systemic most cancers. In sufferers with vascular occlusion of the posterior cerebral artery or its branches, the visual loss may improve slightly and then stay secure without evidence of progression. This characteristic may help to differentiate stroke from other kinds of lesions. In patients with occipital infarcts, it may be very important rule out an embolic supply,forty nine such as valvular heart illness and atrial fibrillation. Although localized disease (thrombosis) within the posterior cerebral artery can happen,64 that is unusual. Rarely, posterior cerebral artery occlusion could develop in a patient with difficult migraine, however in these circumstances, an related abnormality, particularly one thing that will end in hyperviscosity. Giant cell arteritis could have an result on the vertebrobasilar artery system and rarely might trigger occipital infarction. Other vascular issues, including systemic lupus Retrochiasmal Disorders erythematosus, could lead to visual loss from occipital lobe involvement. Such patients with lupus might have optimistic visible experiences, corresponding to flashing lights and visual hallucinations. Occipital hemorrhage results from hypertension, amyloid angiopathy, or coagulopathy, or it happens within a tumor, notably glioblastomas and metastatic melanomas. Occipital area defects may happen in isolation secondary to occipital lobe tumors,76 however headache and different neurologic signs, including papilledema, are often seen. Positive visual phenomena, such as transient flashes of colored shapes or fashioned pictures, may happen in such individuals, and the symptom of palinopsia (recurring images) is extremely suggestive of a tumor, though it could observe an infarct. Other problems that affect the occipital lobe embrace multiple sclerosis,14 abscess, and poisonous results of immunosuppressive remedy. Rarely, bilateral infarcts of either each superior or both inferior calcarine cortices end in bilateral altitudinal visual field defects that may be misinterpreted as being as a outcome of bilateral optic nerve head illness. The time period cortical blindness is used if the disorder may be extra discretely localized to the occipital cortex. Left hemianopia because of an oligodentroglioma involving the occipital area (a and c) in a 12-year-old boy who presented with complications, papilledema and a sixth nerve palsy. Therefore, the excellence between cerebral and cortical blindness could also be assumed on the basis of trigger. It is sometimes difficult to distinguish acute bilateral optic nerve lesions from cortical blindness. Optic nerve lesions are accompanied by sluggish pupillary responses to light and, with time, optic atrophy. This phenomenon, which is usually momentary, can make it troublesome to determine the date of onset of visual loss. It rarely happens in sufferers with lesions involving other areas of the visual pathways. Cortical injury that follows hypoxia, trauma, or drowning is associated with laminar necrosis. After hypoxia or after important head harm, particularly if there was trans-tentorial herniation and compresssion of the posterior cerebral arteries, irrevocable cortical blindness could result seventy one Cortical blindness and homonymous hemianopia seem in some ladies within the postpartum period. This phenomenon, additionally referred to as reversible leukomalacia, may be transient and has been thought to be as a outcome of cerebral venous thrombosis in many cases73,seventy four or to multifocal capillary and precapillary endothelial breakdown with microvascular occlusion from spasm and/or microthrombi within the occipital subcortex. This disorder has been divided into three clinical syndromes: (1) juvenile (8 years of age), with blindness lasting for hours and an excellent prognosis for restoration; (2) adolescent (8 years of age by way of the teens), with a delay of onset of blindness lasting minutes to hours and an excellent prognosis for recovery; and (3) grownup, from severe head damage, with blindness that may be protracted and with a variable visible consequence. Reversible posterior leukoencephalopathy caused by immunosuppressive therapy, eclampsia, or acute hypertension might take days to weeks to resolve. Contrast brokers are helpful, especially within the acute part of an infarct, throughout which the lesion could seem isointense with a standard brain. Frisen L, Holmegaard L, Rosencrantz M: Sectorial optic atrophy and homonymous, horizontal sectoranopia: a lateral choroidal artery syndrome

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The first renal changes in diabetes are increased kidney dimension and elevated glomerular filtration fee, leading to microalbuminuria. Later, diffuse thickening of the glomerular basement membrane and mesangial thickening, leading to glomerular occlusion and medical renal insufficiency, happen. Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers gradual this progression. Patients with diabetes receiving dialysis have a 1year survival fee of 80% and a 3-year survival price of 60%. With kidney transplantation, the survival rate with either cadaver or related-donor kidneys is just like that among nondiabetic sufferers present process transplantation. Neurologic Disease Diabetic neuropathy is a typical reason for peripheral neuropathy and a big source of disability. The incidence of symmetric neuropathy is analogous in kind 1 diabetes and kind 2 diabetes, though the focal neuropathies are more widespread amongst those who have had type 2 diabetes longer. The etiology of diabetic neuropathy is 4390 Diabetes Mellitus caring for these compounded lesions adequately. Podiatrists trained in corrective surgery, in concert with the vascular surgeon and with internist help, are attaining commendable results. Rationale for Normalization of Glucose Hyperglycemia is the most obvious manifestation of diabetes and is presently acknowledged as a main cause of diabetic issues. There is rising clinical and biochemical evidence that uncontrolled hyperglycemia results in issues. In secondary diabetes, when genetic associations are absent, problems additionally arise, suggesting that hyperglycemia alone is a significant pathogenic factor. Healthy kidneys from nondiabetic persons transplanted into diabetic sufferers ultimately show diabetic modifications. Prior to the discovery of insulin, sort 1 diabetes was a deadly disease and kind 2 diabetes was uncommon. When life span of those with diabetes was prolonged by way of insulin therapy, a heated debate raged on for nearly all of the 20 th century over the connection of glycemic management and the event of continual problems. Whether or not the goal of physiologic management in a clinical setting is definitely price the often nice effort to prevent the issues of diabetes, has been addressed as latest as the 1990s in landmark nationwide research in the United States and United Kingdom. Macrovascular Disease Macrovascular illness involves two main issues � lower extremity ischemic illness (peripheral vascular disease) and coronary artery illness. Peripheral vascular illness of the decrease extremities is about 15�20 times as common in the diabetic population as in the nondiabetic inhabitants. Type 2 sufferers seem to have a greater proclivity for this manifestation than sufferers with type 1 diabetes. The risk elements of smoking and first hyperlipidemia accentuate the manifestation of this disease. This illness underlines the majority of the extra complicated diabetic foot problems. The onset of the illness is earlier, the manifestations are more extreme, and the mortality is greater. Atypical chest ache, including extreme tiredness, is usually a representation of this downside. Risk factors for creating coronary artery illness embrace smoking, age, hypertension, microalbuminuria, and ldl cholesterol and triglyceride ranges. In addition, diabetes has been firmly established as a cardiovascular threat equivalent. In different phrases, the danger for main coronary events is comparable in diabetic persons without preexisting cardiovascular disease as in nondiabetic persons with heart problems. In addition, the increased prevalence of hyperlipoproteinemia and arterial hypertension in diabetic persons may contribute to the increased atherogenesis. Finally, there are extra issues from myocardial infarction, and the prognosis is poorer. In June 1993 a 10-year report on almost regular glucose management arresting or slowing the onset and progression of issues in type 1 diabetes became available. The addition of C peptide determination, the availability of glucose monitoring, and the measurement of glycosylated hemoglobin had been welcome dimensions of the examine design. The research evaluated whether near-normalization of blood glucose levels resulted in a decreased risk of retinopathy, nephropathy, and neuropathy for sufferers with no retinopathy and decreased development for patients with minimal retinopathy (Table 321. Urine and/or blood glucose testing was carried out once per day and sufferers followed a standard kind 1 diabetes food regimen. No specific blood glucose target was given, and insulin dose adjustment was infrequent. There was frequent insulin dose adjustment aided by blood glucose testing 4 times per day (Table 321. In addition to a quantity of injections of insulin and frequent self-monitoring of blood glucose ranges, intensive therapy involved frequent visits with a multidisciplinary diabetes care group, intensive training, and glycosylated hemoglobin testing every 3 months. Meals and snacking should be measured with accuracy, and activity should be deliberate in consultation with an train physiologist to keep away from extreme hypoglycemia. The fundamental problems in sustaining the therapy on a more physiologic degree are motivation and availability of a support staff. Nearly every side of remedy has undergone a model new alignment since the last decade of the 20th century, together with obtainable oral and noninsulin agents, insulin preparation, needle gauge, residence monitoring of blood glucose, in-office blood glucose assays, and philosophy of administration. The minimal requirement general would be to have out there a diabetes educator as part of the health group. Also, the individual with diabetes must be keen to adjust the remedy three or extra times a day. Ability to afford the extra cost of monitoring and insulin gear is crucial. Specifically, it found that intensive therapy in sort 2 diabetes was useful in reducing combination diabetes outcomes by 12% even with a modest 1% absolute reduction of A1c. Ironically, the research revealed that it could be harder to preserve glycemic control in type 2 diabetic population. On the basis of research findings, patients with intensive remedy may expect a further 7. The results of the research offered the unequivocal reply to the debate concerning the significance of glycemic management. In the study, every point lower in A1c was associated with 30�35% reduction in threat for microvascular complication. The perfect goal for insulin replacement is to simulate physiologic insulin secretion. The combination bolus/basal use of ultra-short-acting insulin, corresponding to aspart or lispro, with longacting insulin, similar to detemir or glargine, brings us a step nearer to covering supper time glucose excursions whereas at the identical time assembly the basal metabolic insulin needs of the body; nonetheless, this regimen translates into four or extra insulin injections per day, a task too demanding for many. Continuous subcutaneous insulin infusion through a pump is an alternate option that eliminates multiple insulin injections.

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The palsy can affect all forms of eye movement within the one direction or solely a selective saccadic palsy could also be current. All ipsilesional saccadic eye movements, including the quick phases of vestibular and optokinetic nystagmus, are both gradual or absent, whereas contralesional saccades and vestibular-evoked, easy pursuit, and optokinetic-evoked eye movements are regular. In addition, there could also be bilateral facial and hypoglossal palsies because of aplasia of these nuclei. All ipsiversive saccadic eye actions are hypermetric, whereas all contraversive saccadic eye movements are hypometric. Lateropulsion probably happens because of disruption of olivocerebellar climbing fibers passing by way of the lateral medulla to produce, indirectly, elevated inhibition of the ipsilateral fastigial nucleus. There was also main position esotropia and bilateral whole facial palsy however no disturbance of consciousness and no long-tract indicators. The saccade in response to a 20� rightward goal change undershot the goal, in order that additional rightward saccades had been required to fixate the target. Vertical saccades in response to a 20� upward and a 20� downward goal change additionally had an unintended leftward part, so that several small saccades with rightward parts were required to fixate the target. Unilateral peripheral trochlear nerve lesions trigger isolated weakness of the superior oblique muscle, leading to vertical and torsional diplopia. Bilateral peripheral trochlear nerve lesions, if symmetric, may produce esotropia and bilateral excyclotropia82 with hypertropia of each eye during adduction. Bilateral superior indirect paralysis should be differentiated from restrictive ophthalmopathies, overaction of the inferior obliques,83 from physiological hyperdeviation in lateral gaze,84 and from alternating skew deviation. Skew deviation is a vertical strabismus ensuing from a supranuclear lesion affecting central otolithic pathways. It may be unilateral or bilateral, and has been reported with lesions all through the brainstem and cerebellum. The ocular tilt reaction is a head�eye postural synkinesis characterized by skew deviation with head tilt and conjugate eye torsion toward the facet of the hypotropic eye. It appears to be an otolithic righting reflex and occurs in patients with lesions of each the peripheral90 and central vestibular systems. A unilateral midbrain lesion that involves the trochlear nucleus or fascicles ends in a typical unilateral superior oblique palsy on the other aspect. A midbrain lesion, similar to an infarct, involving the oculomotor nucleus or fascicles can even lead to a paralytic vertical strabismus, typically with related ptosis. Tonic contraversive ocular tilt reaction brought on by a unilateral midbrain-thalamic lesion. Unilateral medullary lesions that involve the vestibular nucleus produce a tonic ipsilesional ocular tilt response. Acute unilateral lesions of the labyrinth or vestibular nerve invariably produce asymptomatic ipsilesional conjugate ocular torsion. Tethering of the extraocular muscle tissue,103 infiltration of the extraocular muscles,104 and myasthenia gravis are different widespread causes of vertical diplopia. Vertical Gaze Deviation A tonic vertical gaze deviation may result from both underactivity of a contraversive gaze mechanism or overactivity of an ipsiversive gaze mechanism. Examples of an overactive gaze mechanism embrace spasms of upward gaze resulting from oculogyric crises105,106 or from epileptic seizures. He additionally had loss of ache and temperature sensation on the left facet of the face and on the proper aspect of the physique. The vertical gaze palsies that happen with midbrain lesions can assume several completely different patterns with respect to the course and the type of eye movement affected. A vertical gaze palsy can selectively impair upgaze or downgaze, or it could possibly impair both; it might possibly selectively impair only saccadic eye actions or vestibular-evoked eye movements, or it can impair both. In focal midbrain lesions that selectively impair solely saccades, it may be upward saccades, downward saccades,110 or saccades in each directions which are impaired. An isolated upward gaze palsy may be congenital116 and can also happen as an incidental finding in the aged. A vertical gaze palsy can occur in isolation or in association with eyelid, pupillary, and different ocular motor abnormalities. In the highest row, the patient is trying to look straight forward; observe the lid retraction. Once the shunt was changed, the ventricles reduced in size and her eye movements returned to regular. In this affected person, compelled downward gaze was the only real indication of shunt malfunction. The arrows present a small space of edema on the best side of the midbrain ensuing from an embolic infarct. The pupillary responses were spared as a end result of the Edinger�Westphal nucleus is caudal to the lesion. Convergence was spared, and the affected person skilled convergence�retraction nystagmus on attempted upgaze. Convergence�retraction nystagmus has been reported with unilateral lesions of the nucleus of Darkeschewitsch, which was also affected by the infarct. The time period crossed vertical gaze paresis refers to faulty elevation of the ipsilesional eye and defective despair of the contralesional eye in an orthotropic affected person with a unilateral midbrain lesion. The patient had mitral stenosis with atrial fibrillation and had presented 15 years beforehand with transient confusion and permanent downgaze palsy (case 1327). A so-called optokinetic drum or optokinetic tape can be used to reveal pursuit deficits in patients with cerebral or cerebellar lesions, to elicit pursuit-induced congenital nystagmus, or to elicit reflex saccadic eye actions. Unilateral thalamic,one hundred thirty five brainstem,58,seventy five,136�138 and cerebellar139 lesions can every produce ipsiversive, contraversive, or bidirectional horizontal pursuit deficits. Optokinetic Palsies True loss of optokinetic-evoked eye movements could additionally be seen with lesions of the parietal lobe. Bilateral horizontal vestibuloocular reflex suppression deficits happen in sufferers with congenital nystagmus. Global deficits of vestibuloocular reflex suppression occur in diffuse cerebellar degeneration and with drug intoxication. The goal moved to the left and then to the proper at a relentless velocity of 30�/s. The target (t) moved to the left and then to the best at a constant velocity of 25�/s. Rightward clean pursuit was defective (eye velocity <8�/s, shown because the slope of line 2), whereas leftward clean pursuit was regular (>15�/s, slope of line 1). Similarly, convergence spasm is unlikely to be due to neurological disease unless it occurs in affiliation with other signs of a lesion at the midbrain�thalamic junction. Oculographic recordings of the left eye exhibiting that tried pursuit of a target (not shown) shifting to the left and then to the right at a relentless velocity of 10�/s induces a jerk nystagmus with the attribute increasing-velocity, slow-phase waveform of congenital nystagmus. The patient had offered at age 28 years with headache and was discovered to have first-degree left-beating and first-degree right-beating nystagmus, but no primary place nystagmus. Unilateral failure of vestibuloocular reflex suppression due to unilateral cerebrellar lesion.

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It is common for organic and nonorganic diseases to coexist (the so-called useful overlay), and the confusion generated might mask treatable organic problems. Third, the physician should meticulously record within the chart the options that support these conclusions. Statements such as "The affected person reads no better than 20/80 with the right eye viewing monocularly, but throughout binocular viewing with the left eye surreptitiously fogged by crossed �4. Before performing any subtle exams, simple measures might uncover the practical nature of the illness. For example, when a truly blind person is requested to take a glance at his/her left hand, or contact the tips of proper and left index fingers, he/she will immediately give the correct response, without hesitation because of place and joint sense or kinesthetic and spatial orientations. Another task is to observe the affected person whereas he/she is asked to signal his/her name on a chunk of paper. The blind affected person will be able to sign the name whereas the functional affected person makes meaningless scratches or demonstrates hesitation to do it. Malingerers are often anxious and could also be hostile and uncooperative throughout examination, questioning every section and refusing or delaying responses to testing; occasionally, nonetheless, they show overly solicitous conduct in an attempt to persuade the examiner of their sincerity in making an attempt to adjust to directions, whereas avoiding responses and checks which may reveal the dearth of goal proof of illness. Patients with hysteria, on the other hand, are usually fairly cooperative in testing and may paradoxically be unconcerned by their signs. A blind individual usually accepts blindness if it is long-standing; and the person is appropriately anxious if the visible loss is of recent onset. Visual acuity and visual-field loss are considered afferent, whereas pupil abnormalities and extraocular motility disturbances are thought-about efferent visible problems. The visual acuity loss could also be monocular or binocular and will current with a mildmoderate or extreme visual acuity loss. The most typical visual-field criticism with functional visible loss is a concentric lack of peripheral vision or a tunnel imaginative and prescient. Even when the patient complains of impaired acuity, central scotomas are hardly ever discovered. Regardless of the sort of nonorganic complaint, patients tend to attribute it to a sure incident, such as an accident, damage, or sickness, and to date its onset accordingly. The signs may be nonphysiologic in nature, similar to transient binasal visual-field defects, or may be inconsistent with the bodily findings, corresponding to markedly 4018 Nonorganic Visual Disorders functional vision loss could current with monocular or binocular diplopia or triplopia. If the affected person acknowledges that the diplopia stays with closure of one eye, the origin of the symptom is a monocular drawback (which could be bilateral). Monocular diplopia is usually a results of a problem within the refractive standing or the ocular media. Viewing by way of a pinhole ought to remove the monocular diplopia consequent to ocular pathology. Its persistence with a pinhole is suggestive of a functional dysfunction, although it not often may be seen with macular problems, cortical polyopia, or following strabismus surgery. Binocular diplopia usually signifies a disturbance in the ocular motility, however, it too could additionally be a presentation of a functional disorder. It is in the cases by which the patient claims comparatively mild, bilateral impairment of acuity that the excellence could turn out to be tough. The first objective of the evaluation in either state of affairs is to verify or refute any part of organic visible pathway harm. A thorough neuroophthalmological examination including a dilated fundus analysis is essential, as some disorders may be fairly refined. If a patient claims to be unable to understand light, the presence of pupillary constriction to mild stimulation indicates that the vision is best than the patient alleges. With severe bilateral visible loss, the pupillary responses to gentle may be abnormally sluggish bilaterally. When gentle or reasonable bilateral visual loss is claimed, it may be inconceivable to recognize an abnormality within the pupillary mild responses. In these instances and others during which the examination fails to establish the trigger, electroretinography and neuroimaging of the mind must be considered to rule out occult harm to the retina or visual pathways. Such responses show a certain stage of acuity corresponding to the stimulus measurement offered and will contradict claims of poor acuity, suggesting a nonorganic disorder. As noted earlier, the first and significant step is to make sure that no seen evidence of refractive, media, macular, or optic nerve abnormality is present. It is extraordinarily rare for an organic disorder to produce a large disparity in visible acuity between the two eyes. Some techniques that are very useful for evaluation of monocular functional visual loss could not apply in evaluating patients with useful binocular or symmetrical visual acuity loss. In suspected instances of nonorganic extreme bilateral loss, several exams and observations may help confirm better visible operate than is represented with commonplace strategies. Confirmatory testing may embrace involuntary responses to visual stimuli such as an optokinetic tape or a mirror. If the patient claims imaginative and prescient of hand movement or worse, a horizontal jerk nystagmus induced by the usual optokinetic stimulus signifies at least 20/400 imaginative and prescient. Additional testing that will seem to the patient to check imaginative and prescient but actually checks one other operate, similar to proprioception, may be performed. For example, a patient claiming blindness often demonstrates an incapability to touch the ideas of the 2 index fingers collectively or to sign his or her name, whereas patients blind from natural causes accomplish that with out difficulty. With a 15� to 20� axis disparity, the cylindrical lenses will induce substantial blur within the good eye whereas the spherical lenses will impact no change in the dangerous eye; any continued reading is being carried out with the bad eye. Under these circumstances, the patient sees both halves of the screen while viewing via the pink lens and solely the letters on the green half of the screen by way of the green lens. One must be cautious of the subtle patient who both closes one eye or shortly covers one eye with the palm of his/her hand. After establishing good rapport with the kid, the examiner signifies that she or he is going to use some special eye drops. The patient is advised to slowly open the eyes after 30 seconds and appears by way of his/her corrective glasses and a pinhole. Similar exams utilizing polarizing filters and projected acuity targets selectively permit vision by way of only one eye whereas the affected person views binocularly; the letters seen while the pictures are selectively projected to the unhealthy eye confirm a nonorganic element. The prism is placed base up in entrance of the nice eye while the unhealthy eye is occluded. With the prism edge bisecting the pupil, the patient is asked to view the imaginative and prescient chart and to note the (monocular) diplopia induced. The occluder is unobtrusively moved out of the road of vision of the dangerous eye while the prism is simultaneously moved to cover the good eye totally in order that the patient continues to note diplopia (now binocular). The affected person is then requested to read letters on each of the two charts seen, certainly one of which, unknown to the affected person, is being seen via the unhealthy eye. Further, the prism shift check makes use of a 4-D prism base out and depends on normal refixation actions to keep away from diplopia. Thus the patient who claims severe monocular visible loss and is discovered to have regular stereopsis is most probably to be functional. Stereoacuity testing is useful for evaluation of sufferers claiming mild to reasonable bilateral visual loss, within the range of 20/40 to 20/400.

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