rdlc data matrix

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This is a familial disease of children, inherited as an autosomal recessive trait The main symptoms are postural hypotension and lability of blood pressure, faulty regulation of temperature, diminished hearing, hyperhidrosis, blotchiness of the skin, insensitivity to pain, emotional lability, and cyclic vomiting The tendon re exes are hypoactive and mild slowing of motor nerve conduction velocities is common There is denervation sensitivity of the pupils and other structures The main pathologic feature is a de ciency of neurons in the superior cervical ganglia and in the lateral horns of the spinal cord Also, the number of unmyelinated nerve bers in the sural nerve is greatly decreased according to Aguayo and to Dyck and their colleagues (see also page 1159) It is likely that this disease represents a failure of embryologic migration or formation of the rst- and second-order sympathetic neurons It is now known that this defect is the result of a mutation in the gene coding for a protein that is associated with the transcription regulator, Ikappa B (Anderson et al) Autonomic symptoms may also be a prominent feature of the small- ber neuropathy of Fabry disease ( -galactosidase de ciency) as a result of the accumulation of ceramide in hypothalamic and intermediolateral column neurons (see pages 839 and 1159) Another inherited form of peripheral dysautonomia is characterized by severe pain in the feet on exercise and an autosomal

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2 Select the Label control and open the Properties window 3 Click on the Lightning Bolt icon You will see a list of all events that can be used with

cessive cases, 63 were of central type due to brainstem strokes, 21 were preganglionic from trauma or tumors of the neck, 13 were postganglionic due to miscellaneous causes, and in 3 cases the localization could not be determined (see Chap 14 for further discussion) The pupillary disturbances associated with oculomotor nerve lesions, the Adie pupil, and other parasympathetic and sympathetic abnormalities of pupillary function are considered fully in Chap 14 The combination of segmental anhidrosis and an Adie pupil is sometimes referred to as the Ross syndrome; it may be abrupt in onset and idiopathic or may follow a viral infection

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the Label web control as shown in Figure 8-1

tactual stimuli of the limbs and abdomen, and pressure on the bladder An exaggerated vasopressor reaction also occurs in response to injected NE In such attacks, the patient experiences paresthesias of the neck, shoulders, and arms; tightness in the chest and dyspnea; pupillary dilatation; pallor followed by ushing of the face; sensation of fullness in the head and ears; and a throbbing headache Plasma NE and dopamine rise slowly during the autonomic discharge When such an attack is severe and prolonged, myocardial infarction, seizures, and visual defects have been observed Clonidine, up to 02 mg three times daily, has been useful in preventing the hypertensive crises

241 B6

4 Next to the Init event, type LabelEvent as shown in Figure 8-1

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Lesions of the C4 or C5 segments of the spinal cord, if complete, will interrupt suprasegmental control of both the sympathetic and sacral parasympathetic nervous systems Much the same effect is observed with lesions of the upper thoracic cord (above T6) Lower thoracic lesions leave much of the descending sympathetic out ow intact, only the descending sacral parasympathetic control being interrupted Traumatic necrosis of the spinal cord is the usual cause of these states, but they may be due as well to infarction, certain forms of myelitis, and tumors As discussed in greater detail in Chap 44, the initial effect of an acute cervical cord transection is abolition of all sensorimotor, re ex, and autonomic functions of the isolated spinal cord The autonomic changes include hypotension, loss of sweating and piloerection, paralytic ileus and gastric atony, and paralysis of the bladder Plasma epinephrine and NE are reduced This state, known as spinal shock, lasts for several weeks The basic mechanisms are not known, but changes in neurotransmitters (catecholamines, endorphins, substance P, and 5-hydroxytryptamine) are under investigation After spinal shock dissipates, sympathetic and parasympathetic functions return, since the afferent and efferent autonomic connections within the isolated segments of the spinal cord are intact, although no longer under the control of higher centers With cervical cord lesions, there is a loss of the sympathetically mediated cardiovascular changes in response to stimuli reaching the medulla However, cutaneous stimuli (pinprick or cold) in segments of the body below the transection will raise the blood pressure However, a fall in blood pressure is not compensated by sympathetic vasoconstriction Hence tetraplegics are almost obligatorily prone to orthostatic hypotension Pinching the skin below the lesion causes goose esh in adjacent segments Heating the body results in ushing and sweating over the face and neck but not the trunk and legs Bladder and bowel, including their sphincters, which are at rst accid, become automatic as spinal re ex control returns There may be re ex penile erection or priapism and even ejaculation With lesions in the upper thoracic cord, similar but lesser degrees of labile blood pressure are seen; in several of our patients with destructive myelitis, a viral infection of fever brought out episodes of a drop in blood pressure to approximately 80/60 mm Hg and a subsequent rapid rise to 190/110 mmHg After a time the tetraplegic may develop a mass re ex (page 1052), in which exor spasms of the legs and involuntary emptying of the bladder are associated with a marked rise in blood pressure, bradycardia, and sweating and pilomotor reactions in parts below the cervical segments (autonomic dysre exia) These reactions may also be evoked by pinprick, passive movement, con-.

When working with web controls and events, do not depend on the double-click method in the Design or Split mode Some web controls that can have events do not generate them automatically in ASPNET and C# by double-clicking them in the Design window However, working with the Properties window, as soon as you enter an event name such as LabelEvent as shown in Figure 8-1, the code for both the ASPNET and C# portions of the application are automatically coded

Acute Autonomic Crises (Sympathetic Storm)

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Figure 8-1

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