Horner's syndrome
TEE
supplemental sympathetic
cervical ganglia
=> anhydrosis, ptosis, myosis
--------------------------------------------------------------------------
tumor lysis syndrome
purine => uric acid = gout
Allopurinol = mechanism of action(MOA) = inhibit zanthine oxidase
purine => uric acid with help of ZO
gout =< wks =""> colchicine acute
gonorrhea
pharyngeal swap
pseudogout
5IAAH(?)
carcinoid tumor
like pheochromocytoma, VMA(vanillyl mandelic acid), catecholamine
rheumatoid heart disease
=> valvular defect (mitral, aortic)
Swan ganz cathetor
--------------------------------------------------------------------------
--------------------------------------------------------------------------
A pancoast tumor, also pancoast tumour (UK), is a tumor of the pulmonary apex. The growing tumor causes disruption of the sympathetic ganglion due to pressure on it.
Symptoms can include miosis (constriction of the pupils), anhidrosis on effected side(lack of sweating) and ptosis (drooping of the eyelid), in severe cases, a complete Horner's syndrome. In progressive cases, the brachial plexus is also affected, causing pain and weakness in the muscles of the arm and hand.
In superior vena cava syndrome, obstruction of the SVC by a tumor (mass effect) causes facial swelling, cyanosis and dilatation of the veins of the head and neck.
A pancoast tumor is an apical tumor that is typically found in conjunction with a smoking history. The clinical signs and symptoms can be confused with neurovascular compromise at the level of the thoracic outlet. The patient's smoking history, rapid onset of clinical signs and symptoms, and pleuritic pain can suggest an apical tumor.
Pancoast Tumor
A pancoast tumor is a superior pulmonary sulcus tumor, frequently it is a squamous cell carcinoma. The presenting symptoms may be related to an invasion of adjacent structures, with arm pain and muscular atrophy attributable to brachial plexus involvement, Horner’s syndrome occurs from involvement of the sympathetic chain, and shoulder pain from chest wall invasion. Radiographic findings include asymptomatic apical pleural thickening and mass. Soft-tissue invasion and bone destruction may be observed as well.
The case above illustrates left apical opacity with destruction of 3rd rib.--------------------------------------------------------------------------
Horner's syndrome is a clinical syndrome caused by damage to the sympathetic nervous system. It is also known by the names Bernard-Horner syndrome or oculosympathetic palsy.
Symptoms
It results in ptosis (drooping upper eyelid), miosis (constricted pupil), and occasionally apparent enophthalmos (the impression that the eye is sunk in) and anhidrosis (decreased sweating) on one side of the face, loss of ciliospinal reflex and blood shot conjunctiva.
In children Horner's syndrome sometimes leads to a difference in eye color between the two eyes (heterochromia).[1] This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.
Pathophysiology
Horner's syndrome is due to a deficiency of sympathetic activity. The site of lesion to the sympathetic outflow is on the ipsilateral side of the symptoms. The following are examples of conditions that cause the clinical appearance of Horner's syndrome:
- First-order neuron disorder: Central lesions that involve the hypothalamospinal pathway (e.g. transection of the cervical spinal cord).
- Second-order neuron disorder: Preganglionic lesions (e.g. compression of the sympathetic chain by a lung tumor).
- Third-order neuron disorder: Postganglionic lesions at the level of the internal carotid artery (e.g. a tumor in the cavernous sinus).
[edit] Diagnosis
Three tests are useful in confirming the presence and severity of Horner's syndrome:
- Cocaine drop test - Cocaine blocks the reuptake of norepinephrine resulting in the dilation of a normal pupil. The pupil will fail to dilate in Horner's syndrome.
- Paredrine test
- Dilation lag test
It is important to distinguish the ptosis caused by Horner's syndrome from the ptosis caused by a lesion to the oculomotor nerve. In the former, the ptosis occurs with a constricted pupil (due to a loss of sympathetics to the eye), whereas in the latter, the ptosis occurs with a dilated pupil (due to a loss of innervation to the sphincter pupillae).
--------------------------------------------------------------------------
| |||||
How it works
A viewing tube and small transducer are passed down the esophagus, allowing the examiner to transmit sound waves to the heart from inside the body (see figure a and figure 7b).
To visualize the back of the heart, a tiny transducer is inserted into a small tube, or catheter, and passed through the mouth into the esophagus until it is positioned immediately behind the heart.
After the transducer is positioned behind the heart, it can then be manipulated to produce images of the heart chambers and other structures.
Anhidrosis means lack of sweating.
[edit] Causes
It may be caused by underactivity of the sympathetic nervous system. Eccrine sweat glands are innervated via muscarinic acetylcholine receptors. Hence antimuscarinic drugs cause anhidrosis.
--------------------------------------------------------------------------
Ptosis (πτωσις "falling, a fall") refers to droopiness of any body part. Specifically, it can refer to:
- Ptosis (eyelid)
- Ptosis (breasts)
- Nephroptosis (kidney)
- Gastroptosis (stomach)
In ophthalmology, ptosis is an abnormally low position (drooping) of the upper eyelid.
Depending upon the cause it can be classified into:
- Neurogenic ptosis which includes IIIrd cranial nerve palsy, Horner's Syndrome, Marcus Gunn jaw winking syndrome, IIIrd cranial nerve misdirection.
- Myogenic ptosis which includes myasthenia gravis, myotonic dystrophy, ocular myopathy, simple congenital ptosis, blepharophimosis syndrome
- Aponeurotic ptosis which may be involutional or post-operative.
- Mechanical ptosis which occurs due to edema or tumors of the upper lid
[edit] Treatment
One of the surgical procedures that can partially correct ptosis is called a frontalis lift, where the eyelids are connected by fascia ties to the frontalis muscles (the muscles in the forehead) This can be done by using synthetic mesh materials, or, for more advanced patients, a length of tissue from the tendon in the upper leg can be used. Surgery usually requires being modified after about ten years.
Miosis is a medical term for constriction of the pupil. It is the opposite of mydriasis. It is seen in a variety of medical conditions, and can also be caused by certain drugs and chemicals. Eye drops used to intentionally cause miosis are known as "miotics". Extreme miosis is commonly called "pinpoint pupils".
Physiology of the photomotor reflex
Visual stimuli enters the eye, and through the retinal photoceptors is converted into an electric impulse, carried through the optic nerve (cranial nerve II) to the brain, where it connects to the pretectal nucleus of the high midbrain. It bypasses the lateral geniculate nucleus and the primary visual cortex. From the pretectal nucleus neurons send axons to neurons of the Edinger-Westphal nucleus whose visceromotor axons run along both the left and right oculomotor nerves. Visceromotor nerve axons (which constitute a portion of the III cranial nerve, along with the somatomotor portion derived from the Edinger-Westphal nucleus) synapse on ciliary ganglion neurons, whose parasympathetic axons innervate the constrictor muscle of the iris, producing miosis. [1]
[edit] Causes
[edit] Diseases
- Horner syndrome (a set of abnormalities in the nervous supply of the face due to damage to the sympathetic nervous system).
- Pancoast tumor (a tumor of the apical lung), due to damage to the ascending sympathetic tract that would normally cause the pupil to dilate.
- Hemorrhage into pons (intracranial hemorrhage)
[edit] Drugs
- Opioids such as codeine, morphine, heroin and methadone
- Antipsychotics, including haloperidol, thorazine, and others
- Cholinergic agents such as those used to treat Alzheimer's Disease and nerve gasses; examples of Cholinergic Drugs: carbachol (Miostat) and neostigmine
- Some cancer chemotherapy drugs, including camptothecin derivatives
- Trazadone
- Alcohol
In medicine (oncology and hematology), tumor lysis syndrome (TLS) is a term given to a group of metabolic complications that occur after treatment of neoplasms, usually lymphomas and leukemias. These disorders include hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, and acute renal failure.
Epidemiology
The introduction of allopurinol and uricase prior to anti-neoplastic therapy has decreased the incidence of acute renal failure from uric acid nephropathy. Currently, acute renal failure is more frequently due to hyperphosphatemia. Acute renal failure can still occur spontaneously prior to anti-cancer therapy, but this is due to an increased tumor cell turn-over with overproduction of uric acid (not due to cancer therapy).
[edit] Cause and risk factors
The most common tumors associated with this syndrome are poorly differentiated lymphomas, such as Burkitt's lymphoma, and leukemias, such as acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). Other cancers have also been associated with TLS but are less common. Usually, the precipitating medication regimen includes combination chemotherapy, but those patients with lymphoma and ALL can be affected with steroid treatment alone.
[edit] Symptoms and pathogenesis
Hyperkalemia. Potassium is mainly an intracellular ion. High turnover of tumor cells leads to spill of potassium into the blood. Symptoms usually do not manifest until levels are high (> 7mmol/dL) [normal 3.5-5.0 mmol/dL] and they include
- severe muscle weakness or paralysis
- cardiac conduction abnormalities (can be fatal)
Hyperphosphatemia. Like potassium, phosphates are also predominantly intracellular. With the advent of allopurinol and uricase, hyperphosphatemia is the main cause of acute renal failure in tumor lysis syndrome. The reason is due to deposition of calcium phosphate crystals in the renal parenchyma.
Hypocalcemia. Because of the hyperphosphatemia, calcium is precipitated to form calcium phosphate, which is the main cause of acute renal failure in this syndrome. Symptoms of hypocalcemia include (but not limited to):
- tetany
- seizures
- mental retardation / dementia
- parkinsonian (extrapyramidal) movement disorders
- papilledema
- emotional instability / agitation / anxiety
- myopathy
Hyperuricemia. Uric acid is a breakdown product of DNA, converted by xanthine oxidase from xanthine and hypoxanthine, which are in turn purine breakdown products. Uric acid is more toxic to tissues than (hypo)xanthine. Uric acid nephropathy has been a dominant cause of acute renal failure but with allopurinol and uricase, hyperphosphatemia as replaced this as the main cause of acute renal failure. Oddly, gout is not a feature of tumor lysis; the high uric acid levels that cause gout probably need to be present for an extended period of time before the putative crystals can develop.
Pretreatment spontaneous tumor lysis syndrome. This entity is associated with acute renal failure due to uric acid nephropathy prior to the institution of chemotherapy and is largely associated with lymphomas and leukemias. The important distinction between this syndrome and the post-chemotherapy syndrome is that spontaneous TLS is not associated with hyperphosphatemia. One suggestion for this is that the high cell turnover rate leads to high uric acid levels through nucleoprotein turnover but the tumor reuses the released phosphate for resynthesis of new tumor cells. In post-chemotherapy TLS, tumor cells are destroyed and no new tumor cells are being synthesized.
[edit] Diagnosis
TLS should be suspected in patients with large tumor burden who develops acute renal failure along with hyperuricemia (> 15 mg/dL) or hyperphosphatemia (> 8 mg/dL). (Most other acute renal failure occurs with uric acid <> 1.0, compared to a value of 0.6-0.7 for most other causes of acute renal failure.
--------------------------------------------------------------------------Gout (also called metabolic arthritis) is a disease due to an inborn uric acid metabolism. In this condition sodium urate crystals are deposited on the articular cartilage of joints and in the particular tissue like tendons. This provokes an inflammatory reaction of these tissues. These deposits often increase in size and burst through the skin to form sinuses discharging a chalky white material.
Normally, the human bloodstream only carries small amounts of uric acid. However, if the blood has an elevated concentration of uric acid, uric acid crystals are deposited in the cartilage and tissue surrounding joints. Elevated blood levels of uric acid can also result in uric kidney stones.
Signs and symptoms
The classic picture is of excruciating and sudden pain, swelling, redness, warmness and stiffness in the joint. Low-grade fever may also be present. The patient usually suffers from two sources of pain. The crystals inside the joint cause intense pain whenever the affected area is moved. The inflammation of the tissues around the joint also causes the skin to be swollen, tender and sore if it is even slightly touched. For example, a blanket draping over the affected area would cause extreme pain.
Gout usually attacks the big toe (approximately 75% of first attacks), however it can also affect other joints such as the ankle, heel, instep, knee, wrist, elbow, fingers, and spine. In some cases the condition may appear in the joints of the small toes which have become immobile due to impact injury earlier in life, causing poor blood circulation that leads to gout.
Patients with longstanding hyperuricemia (see below) can have uric acid crystal deposits called tophi (singular: tophus) in other tissues e.g. the helix of the ear. Uric acid stones can form as one kind of kidney stone in some occasions.
Pathogenesis
Although the exact cause of gout is not known, it is thought to be linked to defects in purine metabolism. Purine is an organic compound commonly found in the body and is metabolized by the body into uric acid. People with primary gout have either an increased production of uric acid or an impaired excretion of uric acid, or a combination of both.
There are also different racial propensities to develop gout. The prevalence of gout is high among the peoples of the Pacific Islands, and the Māori of New Zealand, but rare in the Australian aborigine despite the latter's higher mean concentration of serum uric acid.[3]
Hyperuricemia is considered an aspect of the metabolic syndrome, although its prominence has been reduced in recent classifications. This explains the increased prevalence of gout among obese individuals.
Many still believe that gout is caused by a combination of dietary factors and "laziness". In particular, many believe that gout develops following several years of excessive alcohol consumption combined with an ongoing lack of physical activity and a diet completely lacking in purine-neutralising foods, such as berries, as well as other specific fruit and vegetables (see below). Others have refined this theory, saying that some are genetically predisposed to gout and some are not. As a result, people who are not predisposed can live over-indulgent lifestyles and not develop gout, while others who are predisposed can develop gout, despite being physically active and having a well-rounded diet. However, most in the "genetic predisposition" school of thought nonetheless believe that the condition is much more likely to develop in the predisposed if the other factors are present over several years (excess alcohol, inactivity and failure to eat purine-neutralising foods). It is known that lead sugar was used to sweeten wine, and that chronic lead poisoning is a cause of gout,[4][5] which condition is then known as saturnine gout, because of its association with alcohol and excess.[6]
Gout can also develop as co-morbidity of other diseases, including polycythaemia, leukaemia, intake of cytotoxics, obesity, diabetes, hypertension, renal disorders, and hemolytic anemia. This form of gout is often called secondary gout. Diuretics (particularly thiazide diuretics) have traditionally been blamed for precipitating attacks of gout, but a Dutch case-control study from 2006 appears to cast doubt on this.[7]
--------------------------------------------------------------------------------------------------------
Gonorrhoea (gonorrhea in American English; formally acute gonococcal perihepatitis), is among the most common sexually-transmitted diseases in the world and is caused by Gram-negative bacterium Neisseria gonorrhoeae. The term comes from Greek γονόρροια (gonórrhoia), literally "flow of seed"; in ancient times it was thought that the pus discharge associated with the disease contained semen. So, don't let strangers penetrate your anus.
Infection with gonorrhoea increases the risk of passing on or becoming infected with HIV (the virus that causes AIDS). This is likely due to weakening of the mucosal surface secondary to the gonorrhoea infection. Gonorrhoea might also increase the amount of HIV present in semen and other genital secretions. Note, however, that this effect is by no means limited to gonorrhoea and there is increased risk of HIV transmission with co-infection of most sexually-transmitted infections.
The first place this bacterium infects is usually the columnar epithelium of the urethra and endocervix. Non-genital sites in which it thrives are the rectum, the oropharynx and the conjunctivae of the eyes. The vulva and vagina in women are usually spared because they are lined by stratified epithelial cells — in women the cervix is the usual first site of infection.
Gonorrhoea spreads during sexual intercourse, whereby the infective partner does not need to be human.[1] Infected women also can pass gonorrhoea to their newborn infants during delivery, causing eye infections (conjunctivitis) in their babies. When the infection occurs in the genital tract, mouth, or rectum of a child, it is most commonly due to sexual abuse. Gonorrhoea among females can also be transmitted from one individual to another via contact to surfaces that may still be damp from prior contact.
What pseudogout is
Pseudogout is a type of arthritis that, as the name implies, can cause symptoms similar to gout, but in reaction to a different type of crystal deposit. Pseudogout, sometimes referred to as calcium pyrophosphate deposition disease, can cause severe episodes of localized pain and swelling resulting in incapacitation for days or weeks. It also can cause more chronic arthritis that mimics osteoarthritis or rheumatoid arthritis. Knees are most often involved but wrists, shoulders, ankles, elbows or hands can be affected.
What causes pseudogout
Pseudogout develops when deposits of calcium pyrophosphate crystals accumulate in a joint. Crystals deposit first in the cartilage and can damage the cartilage. The crystals also can cause a reaction with inflammation that leads to joint pain and swelling. In most cases it is not known why the crystals form, although crystal deposits clearly increase with age. Because the condition sometimes runs in families, genetic factors are suspected of contributing to the disorder as can a severely underactive thyroid (hypothyroidism), excess iron storage (hemochromatosis), low magnesium levels in blood, an overactive parathyroid gland, and other causes of excessive calcium in the blood (hypercalcemia).
Who gets pseudogout
The calcium crystal deposits seen in pseudogout affect about 3 percent of people in their 60s and as many as 50 percent of people in their 90s. Any kind of insult to the joint can trigger the release of the calcium crystals, inducing a painful inflammatory response. Attacks of pseudogout also can develop following joint surgery or other surgery. However, not everyone will experience severe attacks.
--------------------------------------------------------------------------
Pathophysiology
Carcinoid tumors produce the vasoactive substance, serotonin; it is commonly but incorrectly thought that serotonin is the cause of the flushing. The flushing results from secretion of kallekrein, the enzyme that catalyzes the conversion of kininogen to lysyl-bradykinin. The latter is further converted to bradykinin, one of the most powerful vasodilators known. Other components of the carcinoid syndrome are diarrhea (probably caused by serotonin), a pellagra-like syndrome (probably caused by diversion of large amounts of tryptophan from synthesis of the vitamin, niacin, to the synthesis of 5-hydroxyindoles including serotonin), fibrotic lesions of the endocardium, particularly on the right side of the heart resulting in insufficiency of the tricuspid valve and, less frequently, the pulmonary valve and, uncommonly, bronchoconstriction. The pathogenesis of the cardiac lesions and the bronchoconstriction is unknown. When the primary tumor is in the gastrointestinal tract, as it is in the great majority of cases, the serotonin and kallekriein are inactivated in the liver; manifestations of carcinoid system do not occur until there are metastases to the liver. Carcinoid tumors arising in the bronchi, because their biologically active products reach the systemic circulation before passing through the liver, may be associated with manifestations of carcinoid syndrome without liver metastases.
--------------------------------------------------------------------------Vanillyl mandelic acid (VMA) is a chemical end product of catecholamine metabolism. It is found in urine together with homovanillic acid (HVA). In timed urine tests its quantity (concentration μg /24 h) is assessed, along with creatinine clearance, and the concentration of cortisols, catecholamines, and metanephrines.
Urinary levels of vanillyl mandelic acid are raised in pheochromocytoma, a tumor of catecholamine-secreting chromaffin cells.
--------------------------------------------------------------------------
Chromaffin cells are neuroendocrine cells found in the medulla of the adrenal gland and in other ganglia of the sympathetic nervous system. They are derived from the embryonic neural crest.
[edit] Function
Chromaffin cells of the adrenal medulla are innervated by the splanchnic nerve and secrete adrenaline, noradrenaline, and enkephalin into the bloodstream. As such, they play an important role in the fight-or-flight response. They are also referred to as granules and this is where the enzyme dopamine-hydroxylase catalyzes th conversion of dopamine to norepinephrine.
--------------------------------------------------------------------------
Rheumatic fever is an inflammatory disease which may develop after a Group A streptococcal infection (such as strep throat or scarlet fever) and can involve the heart, joints, skin, and brain.
General information
Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. In the Western countries, it became fairly rare since the 1950s, possibly due to higher hygienic standards. While it is far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a mortality of 2–5%.
Rheumatic fever primarily affects children between ages six and 15 and occurs approximately 20 days after strep throat or scarlet fever. In up to a third of cases, the underlying strep infection may not have caused any symptoms.
The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.
The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode of rheumatic fever. Heart complications may be long-term and severe, particularly if the heart valves are involved.
--------------------------------------------------------------------------
In medicine pulmonary artery catheterization is the insertion of a catheter into a pulmonary artery. Its purpose is diagnostic; it is used to detect heart failure or sepsis, monitor therapy, and evaluate the effects of drugs. The pulmonary artery catheter allows direct, simultaneous measurement of pressures in the right atrium, right ventricle, pulmonary artery, and the filling pressure ("wedge" pressure) of the left atrium.
The pulmonary artery catheter is frequently referred to as a Swan-Ganz catheterGeneral indications are
- Management of complicated myocardial infarction
- Hypovolemia vs cardiogenic shock
- Ventricular septal rupture (VSR) vs acute mitral regurgitation
- Severe left ventricular failure
- Right ventricular infarction
- Unstable angina
- Refractory ventricular tachycardia
- Assessment of respiratory distress
- Cardiogenic vs non-cardiogenic pulmonary edema
- Primary vs secondary pulmonary hypertension
- Assessment of type of shock
- Assessment of therapy
- Afterload reduction
- Vasopressors
- Beta blockers
- Intra-aortic balloon counterpulsation
- Assessment of fluid requirement in critically ill patients
- Hemorrhage
- Sepsis
- Acute renal failure
- Burns
- Management of postoperative open heart surgical patients
- Assessment of valvular heart disease
- Assessment of cardiac tamponade/constriction
[edit] Procedure
The catheter is introduced through a large vein—often the internal jugular, subclavian, or femoral veins. From this entry site, it is threaded, often with the aid of fluoroscopy, through the right atrium of the heart, the right ventricle, and subsequently into the pulmonary artery.
The standard pulmonary artery catheter is equipped with an inflatable balloon at the tip, which facilitates its placement into the pulmonary artery through the flow of blood. The balloon, when inflated, causes the catheter to "wedge" in a small pulmonary blood vessel. So wedged, the catheter can provide a direct measurement of the "filling pressure" of the left ventricle of the heart.
![[1]](http://www.coldbacon.com/mdtruth/pics/netter/fn-oculomotor.jpg){kind=link}