Different Diseases in Different Human Body Systems
Paronychia disease is an infection of the inflammation skin and soft tissue infections that surrounds a toenail or fingernail. It can start suddenly (acute paronychia) or gradually (chronic paronychia). There are two different types of paronychia, acute and chronic depending on the amount of time the infection has been present. The etiology, infectious agent, and treatment are usually different for each form, and the 2 forms are often considered separate entities. Acute paronychia develops over a few hours when a nail fold becomes painful, red and swollen. It is the most common hand infection in the United State.
Symptoms of Paronychia
A small collection of pus forms under the skin next to the nail, or underneath the nail itself. The area around the nail is tender, red and mildly swollen the cuticle is missing; and the skin around the nail feels moist or "boggy." Often, only one nail is affected. Acute paronychia starts as a red, warm, painful swelling of the skin around the nail. In chronic paronychia, the redness and tenderness are less noticeable than the acute infection. The nail may have a green discoloration due to Pseudomonas infection. It mainly occurs in people who have constantly wet hands, such as hairdressers, nurses, bartenders and housewives.
Causes of Paronychia
The causes of acute Paronychia and chronic Paronychia are defind follow :
· Acute paronychia usually results from a traumatic event, however minor, that breaks down the physical barrier between the nail bed and the nail; this disruption allows the infiltration of infectious organisms.
· Acute paronychia can result from seemingly innocuous conditions, such as hangnails, or from activities, such as nail biting, finger sucking, manicuring, or artificial nail placement.
· Staphylococcus aureus is the most common infecting organism. Organisms, such as Streptococcus and Pseudomonas species, gram-negative bacteria, and anaerobic bacteria are other causative organisms.
· Acute (and chronic) paronychia may also occur as a manifestation of other diseases, such as pemphigus vulgaris.
· Chronic paronychia is primarily caused by the yeast fungus Candida albicans .
· Other rare causes of chronic paronychia include bacterial, mycobacterial, or viral infection; metastatic cancer; subungual melanoma; and squamous cell carcinoma.
· Chronic paronychia most often occurs in persons whose hands are repeatedly exposed to moist environments or in those who have prolonged and repeated contact with irritants such as mild acids, mild alkalis, or other chemicals
Treatment of Paronychia
The treatment of choice depends on the extent of the infection. the treatment list of acute Paronychiaare below:
· Warm water soaks of the affected finger 3-4 times per day until symptoms resolve are helpful.
· Oral antibiotics with gram-positive coverage against S aureus, such as amoxicillin and clavulanic acid (Augmentin) or clindamycin (Cleocin), are usually administered concomitantly with warm water soaks.
· Cleocin and Augmentin also have anaerobic activity; therefore, they are useful in treating patients with paronychia due to oral anaerobes contracted through nail biting or finger sucking.
· Cleocin should be used instead of Augmentin in patients who are allergic to penicillin.
· If the paronychia does not resolve or if it progresses to an abscess, it should be drained promptly.
Treatment of chronic Paronychia are
· The initial treatment of chronic paronychia consists of the avoidance of inciting factors such as exposure to moist environments or skin irritants. Keeping the affected lesion dry is essential for proper recovery. Choice of footgear may also be considered.
· Any manipulation of the nail, such as manicuring, finger sucking, or attempting to incise and drain the lesion, should be avoided; these manipulations may lead to secondary bacterial infections. Mild cases of chronic paronychia may be treated with warm soaks.
· The initial medical treatment consists of the application of topical antifungal agents. Topical miconazole may be used as the initial agent. Oral ketoconazole or fluconazole may be added in more severe cases.
· Patients with diabetes and those who are immunocompromised need more aggressive treatment because the response to therapy is slower in these patients than in others.
· In cases induced by retinoids or protease inhibitors, the paronychia usually resolves if the medication is discontinued.
· Keep your hands and feet dry and clean.
· Wear rubber gloves with an absorbent cotton lining if your hands are exposed routinely to water or harsh chemicals.
· Be gentle when you manicure your nails. Avoid cutting your cuticles or pushing them back.
· Avoid biting your nails and picking at the skin around your nails.
· If you have diabetes, keep your blood sugar levels within a normal range by following your diet and taking your medications.
Rickets is an abnormal bone formation in children resulting from inadequate calcium in their bones. This lack of calcium can result from inadequate dietary calcium, inadequate exposure to sunshine (needed to make vitamin D), or from not eating enough vitamin D - a nutrient needed for calcium absorption. Vitamin D is found in animal foods, such as egg yolks and dairy products.
Rickets is a softening of bones in children potentially leading to fractures and deformity. Rickets is among the most frequent childhood diseases in many developing countries. The predominant cause is a vitamin D deficiency, but lack of adequate calcium in the diet may also lead to rickets (cases of severe diarrhea and vomiting may be the cause of the deficiency). Although it can occur in adults, the majority of cases occur in children suffering from severe malnutrition, usually resulting from famine or starvation during the early stages of childhood.Osteomalacia is the term used to describe a similar condition occurring in adults, generally due to a deficiency of vitamin D.The origin of the word "rickets" is probably from the Old English dialect word 'wrickken', to twist. The Greek derived word "rachitis" (ραχίτις, meaning "inflammation of the spine") was later adopted as the scientific term for rickets, due chiefly to the words' similarity in sound.
Symptoms of Rickets
The symptoms of rickets include bowed legs and bowed arms. The bowed appearance is due to the softening of bones, and their bending if the bones are weight-bearing. Bone growth occurs through the creation of new cartilage, a soft substance at the ends of bones. When the mineral calcium phosphate is deposited onto the cartilage, a hard structure is created. In vitamin D deficiency, though, calcium is not available to create hardened bone, and the result is soft bone. Other symptoms of rickets include particular bony bumps on the ribs called rachitic rosary (beadlike prominences at the junction of the ribs with their cartilages) and knock-knees. Seizures may also occasionally occur in a child with rickets, because of reduced levels of dissolved calcium in the bloodstream. Hypophosphatemic rickets usually begins in the first year of life. It ranges from so mild that it produces no noticeable symptoms to so severe that it produces bowing of the legs and other bone deformities, bone pain, and a short stature. Bony outgrowth where muscles attach to bones may limit movement at those joints. A baby's skull bones may close too soon, leading to seizures.
Treatment for Rickets
The treatment goals for rickets are to relieve symptoms and correct the cause of the condition. The underlying cause mustbe treated to prevent recurrence. The replacement of deficient calcium, phosphorus, or vitamin D will eliminate most symptoms of rickets. Dietary sources of vitamin D include fish, liver, and processed milk. Exposure to moderate amounts of sunlight is encouraged. Treating rickets caused by metabolic abnormalities may require a special prescription for vitamin D. A sufficient amount of sunlight each day and adequate supplies of calcium and phosphorus in the diet can prevent rickets. Darker skinned babies need to be exposed longer to the ultraviolet rays. The replacement of Vitamin D may correct rickets using these methods of ultraviolet light and medicine. Rickets heals promptly with 4000 IU of oral vitamin D per day administered for approximately one month.. During this treatment, the doctor should monitor the levels of 25-OH-D in the plasma to make certain they are raised to a normal value. The bone abnormalities (visible by x ray) generally disappear gradually over a period of 3-9 months. Parents are instructed to take their infants outdoors for approximately 20 minutes per day with their faces exposed. Children should also be encouraged to play outside. Foods that are good sources of vitamin D include cod liver oil, egg yolks, butter, and oily fish. Some foods, including milk and breakfast cereals, are also fortified with synthetic vitamin D.
How is rickets diagnosed?
Rickets is diagnosed by x-ray examination of leg bones. A distinct pattern of irregularities, abnormalities, and a coarse appearance can be clearly seen with rickets. A blood test may be done to measure the amounts of the minerals calcium and phosphorus. X-rays of the affected bones are obtained and a musculoskeletal examination reveals tenderness or pain of the bone itself, rather than in the joints or muscles. In some occasions, a bone biopsy, in which a small sample of bone tissue is removed for analysis, also may be needed to confirm the diagnosis.
Rhabdomyolysis is the breakdown of muscle fibers resulting in the release of muscle fiber contents (myoglobin) into the bloodstream. Some of these are harmful to the kidney and frequently result in kidney damage.
When muscle is damaged, a protein pigment called myoglobin is released into the bloodstream and filtered out of the body by the kidneys. Myoglobin breaks down into potentially harmful compounds. It may block the structures of the kidney, causing damage such as acute tubular necrosis or kidney failure.
Dead muscle tissue may cause a large amount of fluid to move from the blood into the muscle, reducing the fluid volume of the body and leading to shock and reduced blood flow to the kidneys.
The disorder may be caused by any condition that results in damage to skeletal muscle, especially trauma.
Risk factors include the following:
· Alcoholism (with subsequent muscle tremors)
· Certain inherited or genetic syndromes
· Crush Injuries
· Heat intolerance
· Ischemia or necrosis of the muscles (as may occur with arterial occlusion, deep venous thrombosis, or other conditions)
· Low phosphate levels
· Severe exertion such as marathon running or calisthenics
· Shaking chills
· Use or overdose of drugs, especially cocaine, amphetamines, statins, heroin, or PCP
· Abnormal urine color (dark, red, or cola colored)
· General weakness
· Muscle stiffness or aching (myalgia)
· Muscle tenderness
· Weakness of the affected muscles
Additional symptoms that may be associated with this disease include the following:
· Joint pain
· Weight gain (unintentional)
Exams and Tests
An examination reveals tender or damaged skeletal muscles.
· CPK is very high.
· Serum myoglobin test is positive.
· Serum potassium may be very high
· Urinalysis may reveal casts and be positive for hemoglobin without evidence of red blood cells on microscopic examination.
· Urine myoglobin test is positive.
This disease may also alter the results of the following tests:
· CPK isoenzymes
· Urine creatinine
· Serum creatinine
Early and aggressive fluids (hydration) may prevent kidney damage by rapidly flushing myoglobin out of the kidneys. Fluids may need to be given through a vein (by IV). Some patients may need kidney dialysis.
Medicines that may be prescribed include diuretics and bicarbonate (if urine output is sufficient).
Hyperkalemia and low blood calcium levels (hypocalcemia) should be treated if present. Kidney failure should be treated as appropriate.
Drink plenty of fluids after strenous exercise to dilute the urine and flush the myoglobin out of the kidney. Proper hydration is also necessary after any condition or event that may involve damage to skeletal muscle.
Choroid Plexus Papilloma
This is a rare, benign tumor most common in children younger than 12.
Choroid plexus papillomas grow slowly until they eventually block the flow of cerebrospinal fluid causing hydrocephalus and increased pressure inside the skull.
Signs and Symptoms
Signs of the tumor resulting from increased intracranial pressure are present in 91% of patients, with Vomiting, homonymous visual field defects and headache being the most common symptoms.
A neurological evaluation should be done if a patient has slowly increasing signs of mental dysfunction, new seizures, persistent headaches or evidence that there is pressure inside the skull such as vomiting or swelling or protrusion of the blind spot at the back of the eye.
A neurologist -- a doctor who has received special additional training in the diagnosis and treatment of disorders of the brain, spinal cord and nerves -- will do a complete examination.
He or she may also request that a magnetic resonance imaging (MRI) scan be done or a computed tomography (CT or CAT) scan be done as well as chest X-rays to check whether the tumor has spread from another part of the body. An MRI usually finds low-grade astrocytomas earlier than CT. Cerebral angiography is rarely used to diagnose a brain tumor, but may be done before surgery.
Depending on the patient's symptoms, specialized tests may be done. These include tests of the field of vision, the sharpness of vision and hearing.
If the results of other tests aren't conclusive, an examination of the fluid that surrounds the brain and spinal cord may be done. This is usually unnecessary.
Treatment of a brain tumor depends on the nature of the tumor, how rapidly it is growing, what symptoms it causing and where it is located. Usually several treatment approaches are used.
This type of tumor is most effectively treated with surgery. In half of the patients, surgery resolves the hydrocephalus and these patients do not require a shunt to drain the excess fluid in the brain.
Choroid plexus carcinoma is a rare, malignant form of this tumor. It is not curable with surgery alone, but requires additional therapy including chemotherapy and sometimes radiation therapy.
Cushing's syndrome is a hormone disorder caused by high levels of cortisol in the blood. This can be caused by taking glucocorticoiddrugs, or by tumors that produce cortisol or adrenocorticotropic hormone (ACTH) or CRH 
Cushing's disease refers to one specific cause of the syndrome, a tumor (adenoma) in the pituitary gland that produces large amounts ofACTH, which in turn elevates cortisol. It is the most common cause of Cushing's syndrome, responsible for 70% of cases
This pathology was described by Harvey Cushing in 1932. the syndrome is also called Itsenko-Cushing syndrome,hyperadrenocorticism or hypercorticism)
Cushing's syndrome is not confined to humans and is also a relatively common condition in domestic dogs and horses.
Signs and Symptoms
Symptoms include rapid weight gain, particularly of the trunk and face with sparing of the limbs (central obesity). A common sign is the growth of fat pads along the collar bone and on the back of the neck (buffalo hump) and a round face often referred to as a "moon face". Other symptoms include hyperhidrosis (excess sweating), telangiectasia (dilation of capillaries), thinning of the skin (which causes easy bruising and dryness, particularly the hands) and other mucous membranes, purple or red striae (the weight gain in Cushing's syndrome stretches the skin, which is thin and weakened, causing it to hemorrhage) on the trunk, buttocks, arms, legs or breasts, proximal muscle weakness (hips, shoulders), and hirsutism(facial male-pattern hair growth), baldness and/or cause hair to become extremely dry and brittle. In rare cases, Cushing's can cause hypercalcemia, which can lead to skin necrosis. The excess cortisol may also affect other endocrine systems and cause, for example, insomnia, inhibited aromatase, reduced libido, impotence, amenorrhoea/oligomenorrhea andinfertility due to elevations in androgens. Patients frequently suffer various psychological disturbances, ranging from euphoria to psychosis. Depression and anxiety are also common.
Other striking and distressing skin changes that may appear in Cushing's syndrome include facial acne, susceptibility to superficial dermatophyte and malassezia infections, and the characteristic purplish, atrophic striae on the abdomen.:500
Other signs include polyuria (and accompanying polydipsia), persistent hypertension (due to cortisol's enhancement of epinephrine's vasoconstrictive effect) and insulin resistance(especially common in ectopic ACTH production), leading to hyperglycemia (high blood sugar) and insulin resistance which can lead to diabetes mellitus. Insulin resistance is accompanied by skin changes such as acanthosis nigricans in the axilla and around the neck, as well as skin tags in the axilla. Untreated Cushing's syndrome can lead to heart diseaseand increased mortality. Cushing's syndrome due to excess ACTH may also result in hyperpigmentation,This is due to Melanocyte-Stimulating Hormone production as a byproduct of ACTH synthesis from Pro-opiomelanocortin (POMC). Cortisol can also exhibit mineralcorticoid activity in high concentrations, worsening the hypertension and leading to hypokalemia(common in ectopic ACTH secretion). Furthermore, gastrointestinal disturbances, opportunistic infections and impaired wound healing (cortisol is a stress hormone, so it depresses the immune and inflammatory responses). Osteoporosis is also an issue in Cushing's syndrome since, as mentioned before, cortisol evokes a stress-like response. Consequently, the body's maintenance of bone (and other tissues) becomes secondary to maintenance of the false stress response. Additionally, Cushing's may cause sore and aching joints, particularly in the hip, shoulders, and lower back.
There are several possible causes of Cushing's syndrome.
Exogenous vs. endogenous
Hormones that come from outside the body are called exogenous; hormones that come from within the body are called endogenous.
The most common cause of Cushing's syndrome is exogenous administration of glucocorticoids prescribed by a health care practitioner to treat other diseases (called iatrogenicCushing's syndrome). This can be an effect of steroid treatment of a variety of disorders such as asthma and rheumatoid arthritis, or in immunosuppression after an organ transplant. Administration of synthetic ACTH is also possible, but ACTH is less often prescribed due to cost and lesser utility. Although rare, Cushing's syndrome can also be due to the use of medroxyprogesterone  
Endogenous Cushing's syndrome results from some derangement of the body's own system of secreting cortisol. Normally, ACTH is released from the pituitary gland when necessary to stimulate the release of cortisol from the adrenal glands.
§ In pituitary Cushing's, a benign pituitary adenoma secretes ACTH. This is also known as Cushing's disease and is responsible for 70% of endogenous Cushing's syndrome.
§ In adrenal Cushing's, excess cortisol is produced by adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands with nodular adrenal hyperplasia.
§ Finally, tumors outside the normal pituitary-adrenal system can produce ACTH that affects the adrenal glands. This final etiology is called ectopic or paraneoplastic Cushing's syndrome and is seen in diseases like small cell lung cancer.
When Cushing's syndrome is suspected, either a dexamethasone suppression test (administration of dexamethasone and frequent determination of cortisol and ACTH level), or a 24-hour urinary measurement for cortisol offer equal detection rates. Dexamethasone is a glucocorticoid and simulates the effects of cortisol, including negative feedback on the pituitary gland. When dexamethasone is administered and a blood sample is tested, high cortisol would be indicative of Cushing's syndrome because there is an ectopic source of cortisol or ACTH (e.g.: adrenal adenoma) that is not inhibited by the dexamethasone. A novel approach, recently cleared by the US FDA, is sampling cortisol in saliva over 24 hours, which may be equally sensitive, as late night levels of salivary cortisol are high in Cushingoid patients. Other pituitary hormone levels may need to be ascertained. Performing a physical examination to determine any visual field defect may be necessary if a pituitary lesion is suspected, which may compress the optic chiasm causing typical bitemporal hemianopia.
When any of these tests are positive, CT scanning of the adrenal gland and MRI of the pituitary gland are performed to detect the presence of any adrenal or pituitary adenomas orincidentalomas (the incidental discovery of harmless lesions). Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Very rarely, determining the ACTH levels in various veins in the body by venous catheterization, working towards the pituitary (petrosal sinus sampling) is necessary.
Most Cushing's syndrome cases are caused by steroid medications (iatrogenic). Consequently, most patients are effectively treated by carefully tapering off (and eventually stopping) the medication that causes the symptoms.
If an adrenal adenoma is identified it may be removed by surgery. An ACTH-secreting corticotrophic pituitary adenoma should be removed after diagnosis. Regardless of the adenoma's location, most patients will require steroid replacement postoperatively at least in the interim as long-term suppression of pituitary ACTH and normal adrenal tissue does not recover immediately. Clearly, if both adrenals are removed, replacement with hydrocortisone or prednisolone is imperative.
In those patients not suitable for or unwilling to undergo surgery, several drugs have been found to inhibit cortisol synthesis (e.g. ketoconazole, metyrapone) but they are of limited efficacy.
Removal of the adrenals in the absence of a known tumor is occasionally performed to eliminate the production of excess cortisol. In some occasions, this removes negative feedback from a previously occult pituitary adenoma, which starts growing rapidly and produces extreme levels of ACTH, leading to hyperpigmentation. This clinical situation is known as Nelson's syndrome.
Testicular torsion is the twisting of the spermatic cord, which cuts off the blood supply to the testicle and surrounding structures within the scrotum. Testicular torsion is when the spermatic cord to a testicle twists, cutting off the blood supply. The most common symptom is acutetesticular pain and the most common underlying cause is a congenital malformation known as a "bell clapper deformity". The diagnosis is often made clinically but if it is in doubt an ultrasound is helpful in ruling in or out the condition. Emergent diagnosis and treatment is required in order to save the viability of the testicle.
Some men may be predisposed to testicular torsion as a result of inadequate connective tissue within the scrotum. However, the condition can result from trauma to the scrotum, particularly if significant swelling occurs. It may also occur after strenuous exercise or may not have an obvious cause.
The condition is more common during infancy (first year of life) and at the beginning of adolescence (puberty).
· Sudden onset of severe pain in one testicle, with or without a previous predisposing event
· Swelling within one side of the scrotum (scrotal swelling)
· Nausea or vomiting
Additional symptoms that may be associated with this disease:
· Testicle lump
· Blood in the semen
Exams and Tests
· Extremely tender and enlarged testicular region -- more common on the right
· The testicle on the affected side is higher
Surgery is usually required and should be performed as soon as possible after symptoms begin. If surgery is performed within 6 hours, most testicles can be saved.
During surgery, the testicle on the other (non-affected) side is usually also anchored as a preventive measure. This is because the non-affected testicle is at risk of testicular torsion in the future.
If the condition is diagnosed quickly and immediately corrected, the testicle may continue to function properly. After 6 hours of torsion (impaired blood flow), the likelihood that the testicle will need to be removed increases. However, even with less than 6 hours of torsion, the testicle may lose its ability to function.
If the blood supply is cut off to the testicle for a prolonged period of time, it may atrophy (shrink) and need to be surgically removed. Atrophy of the testicle may occur days-to-months after the torsion has been corrected. Severe infection of the testicle and scrotum is also possible if the blood flow is restricted for a prolonged period.
When to Contact a Medical Professional
Go to the emergency room or call the local emergency number (such as 911) if testicular torsion symptoms occur.
Use precautions to avoid trauma to the scrotum. Many cases are not preventable.
Torsion of the testis; Testicular ischemia; Testicular twisting.
Kawasaki disease (KD), also known as Kawasaki syndrome, lymph node syndrome and Mucocutaneous lymph node syndrome, is an autoimmune disease that manifests as a systemic necrotizing medium-sized vessel vasculitis and is largely seen in children under 5 years of age. It affects many organ systems, mainly those including the blood vessels, skin, mucous membranes and lymph nodes; however, its most serious effect is on the heart where it can cause severe coronary artery aneurysms in untreated children. Without treatment, mortality may approach 1%, usually within 6 weeks of onset. With treatment, the mortality rate is less than 0.01% in the U.S. There is often a pre-existing viral infection that may play a role in its pathogenesis. The conjunctival and oral mucosa, along with the epidermis (skin), become erythematous (red and inflamed). Edema is often seen in the hands and feet and one or both of the cervical lymph nodes are often enlarged. Also, a remittant fever, often 40℃ (104°F) or higher, is characteristic of the acute phase of the disease. In untreated children, the febrile period lasts on average approximately ten days, but may range from 5 to 25 days. The disorder was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.
Systemic vasculitis is an inflammatory condition affecting both veins and arteries throughout the body, and is usually caused by a proliferation of cells associated with an immuneresponse to a pathogen, or autoimmunity. Systemic vasculitides may be classified according to the type of cells involved in the proliferation, as well as the specific type of tissue damage occurring within the vein or arterial walls. Under this classification scheme for systemic vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis (also called necrotizing angeititis), which may be identified histologically by the occurrence of necrosis (tissue death), fibrosis, and proliferation of cells associated with inflammation in the inner layerof the vascular wall. Other diseases featuring necrotizing vasculitis include Polyarteritis nodosa, Wegener's granulomatosis, Henoch-Schönlein purpura and Churg-Strauss syndrome. Kawasaki disease may be further classified as a medium-sized-vessel vasculitis, affecting medium and small sized blood vessels, such as the smaller cutaneous vasculature (veins and arteries in the skin) that range from 50 to 100µm in diameter. KD is also considered to be a primary childhood vasculitis, a disorder associated with vasculitis that mainly affects children under the age of 18. A recent, consensus-based evaluation of vasculitides occurring primarily in children resulted in a classification scheme for these disorders, to both distinguish them and suggest a more concrete set of diagnostic criteria for each. Within this classification of childhood vasculitides, Kawasaki disease is, again, a predominantly medium-sized vessel vasculitis. It is also an autoimmune form of vasculitis, and is not associated with ANCA antibodies, unlike other vasculitic disorders associated with them, such as wegener's granulomatosis,microscopic polyangiitis, and Churg-Strauss syndrome. This categorization is considered essential for appropriate treatment.
Signs and Symptoms
(A) Bilateral, non-exudative conjunctivitis with perilimbal sparing - "conjunctival injection". (B) Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding. (C)Erythematous rash involving perineum. (D) Erythema of the palms, which is often accompanied by painful, brawny edema of the dorsa of the hands. (E) Erythema of the soles, and swelling dorsa of the feet. (F) Desquamation of the fingers. (G) Erythema and induration at the site of a previous vaccination with Bacille Calmette-Gurin (BCG). (H) Perianal erythematous desquamation.
Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes because of non-suppurative conjunctivitis, iritis and bilateral anterior uveitis. Inflammation of themucous membranes in the mouth, along with erythema (redness), edema (swelling) with fissures(cracks in the lip surface), desquamation (peeling) and exsudation of the lips are also evident. Theoropharynx mucosa has enanthema and the tongue maintains an unusual red appearance termed "strawberry tongue" (marked erythema with prominent gustative papillae). Keratic precipitates(detectable by a slit lamp but usually too small to be seen by the unaided eye), and swollen lymph nodes may also be present and can be the first manifestation of the disease. Rashes occur early in the disease, and the cutaneous rash observed in patients with KD is non-specific, polymorphic, non-itchy and normally observed up to the 5th day of fever. Cutaneous exanthema may comprisemacular-papular erythematous and fissure lesions, the most common type, in addition to urticariformtype rash, purpuric, multiform-like erythema. and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. It is a syndrome affecting multiple organ systems, and in the acute stage of KD, systemic inflammatory changes are evident in many organs.
Myocarditis,pericarditis, valvulitis,asepticmeningitis, pneumonitis, lymphadenis, and hepatitis may be present and are manifested by the presence of inflammatory cells in the affected tissues. If left untreated, some symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short. High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F), The duration of fever is on average one to two weeks; in the absence of treatment, it may extend for three to four weeks. However, when appropriate therapy is started the fever is gone after two days. Red eyes (conjunctivitis) bilateral without pus or drainage, also known as "conjunctival injection".Anterior uveitis. Bright red, chapped, or cracked lips. Red mucous membranes in the mouth. Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue. Red palms of the hands and the soles of the feet. Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails. Rash which may take many forms, non-specific, polymorphic, non-itchy, but not vesicle-bullous lesions, and appears on the trunk. Swollen lymph nodes (frequently only one lymph node is swollen, and is usually on one side), particularly in the neck area. Joint pain (arthralgia) and swelling, frequently symmetrical, Also arthritis can occur. Irritability. Tachycardia (rapid heart beat). Beau's lines (transverse grooves on nails). May find breathing difficult.
X-ray showing Aneurysmal enlargement of the coronary arteries, which is a complication in a Kawasaki syndrome
The cardiac complications are the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children. Overall, about 10–18% of children with Kawasaki disease develop coronary artery aneurysms with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease and rheumatic fever are the most common causes of acquired heart disease among children in the United States.
Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown, but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology, but debate continues on whether the cause is a conventional antigenic substance or a superantigen. Children's Hospital Boston reports that "[s]ome studies have found associations between the occurrence of Kawasaki disease and recent exposure to carpet cleaning or residence near a body of stagnant water; however, cause and effect have not been established."
An association has been identified with a SNP in the ITPKC gene, which codes an enzyme that negatively regulates T-cell activation. An additional factor that suggests genetic susceptibility is the fact that regardless of where they are living, Japanese children are more likely than other children to contract the disease. The HLA-B51 serotype has been found to be associated with endemic instances of the disease.
Criteria for Diagnosis of Kawasaki Disease
Fever of ≥5 days' duration associated with at least 4† of the following 5 changes
Bilateral nonsuppurative conjunctivitis
One of more changes of the mucous membranes of the upper respiratory tract, including pharyngeal injection, dry fissured lips, injected lips, and "strawberry" tongue
One or more changes of the extremities, including peripheralerythema, peripheral edema, periungual desquamation, and generalized desquamation
Polymorphous rash, primarily truncal
Cervical lymphadenopathy >1.5 cm in diameter
Disease cannot be explained by some other known disease process
†A diagnosis of Kawasaki disease can be made if fever and only 3 changes are present in conjunction with coronary arterydisease documented by two-dimensional echocardiography orcoronary angiography.
Source: Nelson's essentials of pediatrics, Review
Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms). There exists no specific laboratory test for this condition. It is difficult to establish the diagnosis, especially early in the course of the illness, and frequently children are not diagnosed until they have seen several health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever,toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (infantile acrodynia). Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters.
Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.
Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology and pediatric infectious disease specialists (although no specific infectious agent has been identified yet). It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries.
Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. In rare cases, a third dose may be given to the child. IVIG by itself is most useful within the first seven days of onset of fever, in terms of preventing coronary artery aneurysm.
Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as IVIG. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for two months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye's syndrome. Because children with Kawasaki disease will be taking aspirin for up to several months, vaccination against varicella and influenza is required, as these infections are most likely to cause Reye's syndrome. Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. Additionally, corticosteroid use in the setting of Kawasaki disease is associated with increased risk of coronary artery aneurysm, and so its use is generally contraindicated in this setting. In cases of kawasaki disease refractory to IVIG, cyclophosphamide and plasma exchange have been investigated as possible treatments, with variable outcomes.
There are also treatments for iritis and other eye symptoms. Another treatment may include the use of Infliximab (Remicade). Infliximab works by binding tumour necrosis factor alpha.
With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement.
It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires re-hospitalization and re-treatment. Treatment with IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment. There is also evidence that Kawasaki disease produces altered lipid metabolism that persists beyond clinical resolution of the disease.
Pleurisy (also known as pleuritis) is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs. Among other things, infections are the most common cause of pleurisy.
The inflamed pleural layers rub against each other every time the lungs expand to breathe in air. This can cause severe sharp pain with inhalation (also called pleuritic chest pain).
The main symptom of pleurisy is a sharp or stabbing pain in the chest that gets worse with deep breathing, coughing, sneezing or laughing. The pain may stay in one place, or it may spread to the shoulder or back. Sometimes it becomes a fairly constant dull ache.
Depending on what's causing the pleurisy, one may have other symptoms:
§ Shortness of breath
§ Fever and chills
§ Rapid, shallow breathing
§ Unexplained weight loss
§ Sore throat followed by pain and swelling in the joints
Viral infection is the most common cause of pleurisy. However, many different conditions can cause pleurisy:
§ Bacterial infections like pneumonia and tuberculosis
§ Autoimmune disorders like systemic lupus erythematosus (or drug-induced lupus erythematosus) and rheumatoid arthritis
§ Lung cancer and lymphoma
§ Other lung diseases like Cystic Fibrosis, sarcoidosis, asbestosis, lymphangioleiomyomatosis, and mesothelioma
§ Pulmonary embolism, a blood clot in the blood vessels that go into the lungs
§ Inflammatory bowel disease
§ Familial Mediterranean fever, an inherited condition that often causes fever and swelling in the abdomen or lung
§ Infection from a fungus or parasite
§ Heart surgery, especially coronary artery bypass grafting
§ High blood pressure
§ Chest injuries
§ Aortic dissection
§ Can occur with no illness or infection
Some cases of pleurisy are idiopathic, meaning the cause cannot be determined.
A diagnosis of pleurisy or another pleural condition is based on medical histories, physical exams, and diagnostic tests. The goals are to rule out other sources of the symptoms and to find the cause of the pleurisy so the underlying disorder can be treated.
A doctor uses a stethoscope to listen to the breathing. This detects any unusual sounds in the lungs. A person with pleurisy will have inflamed layers of the pleura that make a rough, scratchy sound as they rub against each other during breathing. This is called pleural friction rub, and it is a likely sign of pleurisy.
Depending on the results of the physical exam, diagnostic tests are sometimes performed.
A chest x-ray takes a picture of the heart and lungs. It may show air or fluid in the pleural space. It also may show what's causing the pleurisy –for example; pneumonia, a fractured rib, or a lung tumor.
Sometimes an x-ray is taken while lying on the painful side. This may show fluid that did not appear on the standard x-ray taken while standing.
Computed tomography (CT) scan
A CT scan provides a computer-generated picture of the lungs that can show pockets of fluid. It also may show signs of pneumonia, a lung abscess, or a tumor.
Ultrasonography uses sound waves to create pictures of the lungs. It may show where fluid is located in the chest. It also can show some tumors.
Magnetic resonance imaging (MRI)
Magnetic resonance imaging (MRI), also called nuclear magnetic resonance (NMR) scanning, uses powerful magnets to show pleural effusions and tumors.
Blood tests can detect bacterial or viral infection, pneumonia, rheumatic fever, a pulmonary embolism, or lupus.
Arterial blood gas
In arterial blood gas sampling, a small amount of blood is taken from an artery, usually in the wrist. The blood is then checked for oxygen and carbon dioxide levels. This test shows how well the lungs are taking in oxygen.
Treatment has several goals:
§ Remove the fluid, air, or blood from the pleural space
§ Relieve symptoms
§ Treat the underlying condition
Treating the cause
Ideally, the treatment of pleurisy is aimed at eliminating the underlying cause of the disease.
§ If the pleural fluid is infected, treatment involves antibiotics and draining the fluid. If the infection is tuberculosis or from a fungus, treatment involves long-term use of antibiotics or antifungal medicines.
§ If the fluid is caused by tumors of the pleura, it may build up again quickly after it is drained. Sometimes antitumor medicines will prevent further fluid buildup. If they don't, the doctor may seal the pleural space. This is called pleurodesis. Pleurodesis involves the drainage of all the fluid out of the chest through a chest tube. A substance is inserted through the chest tube into the pleural space. This substance irritates the surface of the pleura. This causes the two layers of the pleura to squeeze shut so there is no room for more fluid to build up.
§ Chemotherapy or radiation treatment also may be used to reduce the size of the tumors.
§ If congestive heart failure is causing the fluid buildup, treatment usually includes diuretics and other medicines.
The most common and known treatment for pleurisy is generally to carry on as normal, ibuprofen and amoxicilin being common treatments prescribed by doctors. Milder forms of Pleurisy can be noticed by less inflammatres of the arms and legs. If this is the case Pleurisy will clear of all symptoms within two weeks.
A number of alternative or complementary medicines are being investigated for their anti-inflammatory properties, and their use in pleurisy. At this time, clinical trials of these compounds have not been performed.
Extracts from the Brazilian folk remedy Wilbrandia ebracteata ("Taiuia") have been shown to reduce inflammation in the pleural cavity of mice. The extract is thought to inhibit the same enzyme, cyclooxygenase-2 (COX-2), as the non-steroidal anti-inflammatory drugs. Similarly, an extract from the roots of the Brazilian Petiveria alliacea plant reduced inflammation in a rat model of pleurisy. The extract also reduced pain sensations in the rats. An aqueous extract from Solidago chilensis has been shown to reduce inflammation in a mouse model of pleurisy.
Pleurisy root, or butterfly weed, was so named because it was used by Native Americans to treat pleurisy. The root was said to encourage coughing by thinning the mucus in the lungs. Pleurisy root is not used much today because more effective medicines are available.
Pleurisy is often associated with complications that affect the pleural space.
In some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. The buildup of fluid usually forces the two layers of the pleura apart so they don't rub against each other when breathing. This can relieve the pain of pleurisy. A large amount of extra fluid can push the pleura against the lung until the lung, or a part of it, collapses. This can make it hard to breathe.
In some cases of pleural effusion, the extra fluid gets infected and turns into an abscess. This is called an empyema.
Pleural effusion involving fibrinous exudates in the fluid may be called fibrous pleurisy. It sometimes occurs as a later stage of pleurisy.
A person can develop a pleural effusion in the absence of pleurisy. For example, pneumonia, heart failure, cancer, or a pulmonary embolism can lead to a pleural effusion.
Air or gas also can build up in the pleural space. This is called a pneumothorax. It can result from acute lung injury or a lung disease like emphysema. Lung procedures, like surgery, drainage of fluid with a needle, examination of the lung from the inside with a light and a camera, or mechanical ventilation, also can cause a pneumothorax.
The most common symptom is sudden pain in one side of the lung and shortness of breath. A pneumothorax also can put pressure on the lung and cause it to collapse.
If the pneumothorax is small, it may go away on its own. If large, a chest tube is placed through the skin and chest wall into the pleural space to remove the air.
Blood also can collect in the pleural space. This is called hemothorax. The most common cause is injury to the chest from blunt force or surgery on the heart or chest. Hemothorax also can occur in people with lung or pleural cancer.
Hemothorax can put pressure on the lung and force it to collapse. It also can cause shock, a state of hypoperfusion in which an insufficient amount of blood is able to reach the organs.
Pleurisy and other disorders of the pleura can be serious, depending on what caused the inflammation in the pleura.
If the condition that caused the pleurisy or other pleural disorders isn't too serious and is diagnosed and treated early, one usually can expect a full recovery.
Radiation-induced esophagitis is an injury to the esophagus caused by radiation therapy designed to treat cancer.
The injury can take the forms of:
§ Stricture or narrowing of the esophagus
§ Perforation or rupture
§ Fistula or a hole in the tissue
In some cases, radiation-sensitizing drugs designed to make cancer cells more susceptible to the radiation therapy, can also worsen the radiation damage to the esophagus.
Symptoms of Radiation-Induced Esophagitis
The symptoms of radiation-induced esophagitis are:
§ Difficulty in swallowing
§ Pain during swallowing
§ Vomiting food or blood
§ Chest pain
§ Inflammation of the esophageal lining
Your doctor may perform the following tests to diagnose radiation-induced esophagitis:
§ Barium swallow test
The tell-tale sign of this form of esophagitis is inflammation.
Treatment of Radiation-Induced Esophagitis
If eating or drinking difficulty is present, then a temporary feeding tube to the stomach or an intravenous line may be inserted until the injury heals.
Otherwise, treatment for radiation-induced esophagitis depends on the type of injury:
This abnormal narrowing of the esophagus can be treated by manual dilation or enlargement by:
A bougie (“boojie”) is rubber device used to flatten the lining of the esophagus and to enlarge its opening.
§ Pneumatic dilator
A balloon is placed and inflated to enlarge the narrowed portion of the esophagus.
§ Guided wire dilator
During an endoscopic procedure, a tapered device is threaded through the esophagus by using a guided wire
§ Perforation and fistula
Surgery may be required to treat these injuries
Excretory System Gout
Gout presenting in the metatarsal-phalangeal joint of the big toe. Note the slight redness of the skin overlying the joint.
Gout (also known as podagra when it involves the big toe is a medical condition usually characterized by recurrent attacks of acuteinflammatory arthritis—a red, tender, hot, swollen joint. The metatarsal-phalangeal joint at the base of the big toe is the most commonly affected (~50% of cases). However, it may also present as tophi, kidney stones, or urate nephropathy. It is caused by elevated levels of uric acid in the blood which crystallize and are deposited in joints, tendons, and surrounding tissues.
Diagnosis is confirmed clinically by the visualization of the characteristic crystals in joint fluid. Treatment with nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, or colchicine improves symptoms. Once the acute attack has subsided, levels of uric acid are usually lowered via lifestyle changes, and in those with frequent attacks allopurinol or probenicid provide long-term prevention.
Gout has increased in frequency in recent decades affecting approximately 1–2% of the Western population at some point in their lives. The increase is believed to be due to increasing risk factors in the population, such as metabolic syndrome, longer life expectancy and changes in diet. Gout was historically known as "the disease of kings" or "rich man's disease".
Signs and Symptoms
Gout can present in a number of ways, although the most usual is a recurrent attack of acute inflammatory arthritis (a red, tender, hot, swollen joint). The metatarsal-phalangeal joint at the base of the big toe is affected most often, accounting for half of cases. Other joints, such as the heels, knees, wrists and fingers, may also be affected. Joint pain usually begins over 2–4 hours and during the night. The reason for onset at night is due to the lower body temperature then. Other symptoms that may occur along with the joint pain include fatigue and a highfever. Long-standing elevated uric acid levels (hyperuricemia) may result in other symptomatology, including hard, painless deposits of uric acid crystals known as tophi. Extensive tophi may lead to chronic arthritis due to bone erosion. Elevated levels of uric acid may also lead to crystals precipitating in the kidneys, resulting in stone formation and subsequent urate nephropathy.
Hyperuricemia is the underlying cause of gout. This can occur for a number of reasons, including diet, genetic predisposition, or underexcretion of urate, the salts of uric acid. Renal underexcretion of uric acid is the primary cause of hyperuricemia in about 90% of cases, while overproduction is the cause in less than 10%.About 10% of people with hyperuricemia develop gout at some point in their lifetimes. The risk, however, varies depending on the degree of hyperuricemia. When levels are between 415 and 530 μmol/L (7 and 8.9 mg/dL), the risk is 0.5% per year, while in those with a level greater than 535 μmol/L (9 mg/dL), the risk is 4.5% per year.
Gout frequently occurs in combination with other medical problems. Metabolic syndrome, a combination of abdominal obesity, hypertension, insulin resistance and abnormal lipid levelsoccurs in nearly 75% of cases. Other conditions which are commonly complicated by gout include: polycythemia, lead poisoning, renal failure, hemolytic anemia, psoriasis, and solid organ transplants. A body mass index greater than or equal to 35 increases a male's risk of gout threefold. Chronic lead exposure and lead-contaminated alcohol are risk factors for gout due to the harmful effect of lead on kidney function.. Lesch-Nyhan syndrome is often associated with gouty arthritis.
Diuretics have been associated with attacks of gout. However, a low dose of hydrochlorothiazide does not seem to increase the risk. Other medicines that have been associated include niacin and aspirin (acetylsalicylic acid). Cyclosporine is also associated with gout, particularly when used in combination with hydrochlorothiazide, as are theimmunosuppressive drugs ciclosporin and tacrolimus.
Gout on X-ray of a left foot. Typical location at the big toe joint. Note also the soft tissue swelling at the lateral border of the foot.
Gout may be diagnosed and treated without further investigations in someone with hyperuricemia and the classic podagra. Synovial fluid analysis should be done, however, if the diagnosis is in doubt. X-rays, while useful for identifying chronic gout, have little utility in acute attacks.
Spiked rods of uric acid (MSU) crystals from a synovial fluid sample photographed under a microscope with polarized light. Formation of uric acid crystals in the joints is associated with gout.
A definitive diagnosis of gout is based upon the identification of monosodium urate (MSU) crystals in synovial fluid or a tophus. All synovial fluid samples obtained from undiagnosed inflamed joints should be examined for these crystals. Under polarized light microscopy, they have a needle-like morphology and strong negative birefringence. This test is difficult to perform, and often requires a trained observer. The fluid must also be examined relatively quickly after aspiration, as temperature and pH affect their solubility.
Hyperuricemia is a classic feature of gout; gout occurs, however, nearly half of the time without hyperuricemia, and most people with raised uric acid levels never develop gout. Thus, the diagnostic utility of measuring uric acid level is limited. Hyperuricemia is defined as aplasma urate level greater than 420 μmol/L (7.0 mg/dL) in males and 360 μmol/L (6.0 mg/dL) in females. Other blood tests commonly performed are white blood cell count, electrolytes, renal function, and erythrocyte sedimentation rate (ESR). However, both the white blood cells and ESR may be elevated due to gout in the absence of infection. A white blood cell count as high as 4.0×109/L (40,000/mm3) has been documented.
The most important differential diagnosis in gout is septic arthritis. This should be considered in those with signs of infection or those who do not improve with treatment. To help with diagnosis, a synovial fluid Gram stain and culture may be performed. Other conditions which present similarly include pseudogout and rheumatoid arthritis. Gouty tophi, in particular when not located in a joint, can be mistaken forbasal cell carcinoma, or other neoplasms.
Both lifestyle changes and medications can decrease uric acid levels. Dietary and lifestyle choices that are effective include reducing intake of food such as meat and seafood, consuming adequate vitamin C, limiting alcohol and fructose consumption, and avoiding obesity. A low-calorie diet in obese men decreased uric acid levels by 100 µmol/L (1.7 mg/dL). Vitamin C intake of 1,500 mg per day decreases the risk of gout by 45% compared to 250 mg per day. Coffee, but not tea, consumption is associated with a lower risk of gout. Gout may be secondary to sleep apnea via the release of purines from oxygen-starved cells. Treatment of apnea can lessen the occurrence of attacks.
The initial aim of treatment is to settle the symptoms of an acute attack. Repeated attacks can be prevented by different drugs used to reduce the serum uric acid levels. Ice applied for 20 to 30 minutes several times a day decreases pain. Options for acute treatment include nonsteroidal anti-inflammatory drugs (NSAIDs), colchicine and steroids, while options for prevention include allopurinol, probenecid and febuxostat. Lowering uric acid levels can cure the disease. Treatment of comorbidities is also important.
NSAIDs are the usual first-line treatment for gout, and no specific agent is significantly more or less effective than any other. Improvement may be seen within 4 hours, and treatment is recommended for 1–2 weeks. They are not recommended, however in those with certain other health problems, such as gastrointestinal bleeding, renal failure, or heart failure. While indomethacin has historically been the most commonly used NSAID, an alternative, such as ibuprofen, may be preferred due to its better side effect profile in the absence of superior effectiveness. For those at risk of gastric side effects from NSAIDs, an additional proton pump inhibitor may be given.
Colchicine is an alternative for those unable to tolerate NSAIDs. Its side effects (primarily gastrointestinal upset) limit its usage. Gastrointestinal upset, however, depends on the dose, and the risk can be decreased by using smaller yet still effective doses. Colchicine may interact with other commonly prescribed drugs, such as atorvastatin and erythromycin, among others.
Glucocorticoids have been found to be as effective as NSAIDs and may be used if contraindications exist for NSAIDs. They also lead to improvement when injected into the joint; the risk of a joint infection must be excluded, however, as they worsen this condition.
Pegloticase (Krystexxa) was approved in the USA to treat gout in 2010. It will be an option for the 3% of people who are not adequately treated with other medications due to their association with severe allergic reactions. Pegloticase is administered as an intravenous infusion every two weeks. As of March 2010, however, no double blind, placebo controlled trials have been completed.
A number of medications are useful for preventing further episodes of gout, including allopurinol, probenecid, and febuxostat. They are not usually commenced until one to two weeks after an acute attack has resolved, due to theoretical concerns of worsening the attack, and are often used in combination with either an NSAID or colchicine for the first 3–6 months. They are not recommended until a person has suffered two attacks of gout, unless destructive joint changes, tophi, or urate nephropathy exist, as it is not until this point that medications have been found to be cost effective. Urate-lowering measures should be increased until serum uric acid levels are below 300-360 µmol/L (5.0-6.0 mg/dL) and are continued indefinitely. If these medications are being used chronically at the time of an attack, it is recommended they be continued.
Allopurinol blocks uric acid production, and is the most commonly used hypourecemic agent. Long term therapy is safe and well tolerated, and can be used in people with renal impairment or urate stones, although hypersensitivity occurs in a small number of individuals. Probenecid is effective for treating hyperuricemia, but has been found to be less effective than allopurinol. Febuxostat, a nonpurine inhibitor of xanthine oxidase, is now available as an alternative to allopurinol. It is approved in both Europe and the United States.
Without treatment, an acute attack of gout will usually resolve in 5 to 7 days. However, 60% of people will have a second attack within one year. Those with gout are at increased risk of hypertension, diabetes mellitus, metabolic syndrome, and renal and cardiovascular disease and thus at increased risk of death. This may be partly due to its association withinsulin resistance and obesity, but some of the increased risk appears to be independent.
Without treatment, episodes of acute gout may develop into chronic gout with destruction of joint surfaces, joint deformity, and painless tophi. These tophi occur in 30% of those who are untreated for five years, often in the helix of the ear, over the olecranon processes, or on the Achilles tendons. With aggressive treatment, they may dissolve. Kidney stones also frequently complicate gout, affecting between 10 and 40% of people, and occur due to low urine pH promoting the precipitation of uric acid. Other forms of chronic renal dysfunctionmay occur.
Priapism (Ancient Greek: πριαπισμός), known also as Hulseyism, is a potentially harmful and painful medical condition in which the erectpenis or clitoris does not return to its flaccid state, despite the absence of both physical and psychological stimulation, within four hours. There are two types of priapism: low-flow and high-flow. Low-flow involves the blood not adequately returning to the body from the organ. High-flow involves a short-circuit of the vascular system partway along the organ. Treatment is different for each type. Priapism is considered amedical emergency, which should receive proper treatment by a qualified medical practitioner. Early treatment can be beneficial for a functional recovery.
The name comes from the Greek god Priapus, who was noted for his disproportionately large and permanent erection.
The causative mechanisms are poorly understood but involve complex neurological and vascular factors. Priapism may be associated with haematological disorders, especially sickle-cell disease, and other conditions such as leukemia, thalassemia, and Fabry's disease, and neurologic disorders such as spinal cord lesions and spinal cord trauma (priapism has been reported in hanging victims; see death erection). Priapism may also be associated with glucose-6-phosphate dehydrogenase deficiency, which leads to decreased NADPH. NADPH is a co-factor involved in the formation of nitric oxide, therefore glucose-6-phosphate dehydrogenase deficiency will lower nitric oxide levels, which may result in priapism. Recent breakthroughs in research of the disease have pointed to a raised level of the biochemical adenosine being the cause of the condition. This seems to cause blood vessels to dilate and has the potential to influence blood flow into the penis.
Sickle cell disease often presents special treatment obstacles. Hyperbaric oxygen therapy has also been used with success in some patients.
Priapism can also be caused by reactions to medications. The most common medications that cause priapism are intra-cavernous injections for treatment of erectile dysfunction(papaverine, alprostadil). Other groups reported are antihypertensives, antipsychotics (e.g., chlorpromazine, clozapine), antidepressants (most notably trazodone), anticoagulants,cantharides (Spanish Fly) and recreational drugs (alcohol and cocaine). Priapism has also been linked to achalasia. Priapism is also known to occur from bites of theBrazilian wandering spider. PDE-5 inhibitors have been evaluated as preventive treatment for recurrent priapism.
Potential complications include ischemia, clotting of the blood retained in the penis (thrombosis), and damage to the blood vessels of the penis which may result in an impaired erectile function or impotence. In serious cases, the ischemia may result in gangrene, which could necessitate penis removal.
Medical advice should be sought immediately for cases of erection beyond four hours. Generally, this is done at an emergency department. In sickle cell patients with priapism, the first step in management is a blood exchange transfusion, not a surgical intervention. For other patients, orally administered pseudoephedrine may be effective. Likewise, other sympathomimetic drugs of the amphetamine class have been observed to induce erectile dysfunction, although in a small number of cases they may have the opposite effect. Otherwise, the therapy at this stage is to aspirate blood from the corpus cavernosum under local anaesthetic. If this is still insufficient, then intracavernosal injections of phenylephrine are administered. This should only be performed by a specialist trained in the procedure, with the patient under constant hemodynamic monitoring, as phenylephrine can cause severehypertension, bradycardia, tachycardia, and arrhythmia.
If aspiration fails and tumescence recurs, surgical shunts are next attempted. These attempt to reverse the priapic state by shunting blood from the rigid corpora cavernosa into thecorpus spongiosum (which contains the glans and the urethra). Distal shunts are the first step, followed by more proximal shunts.
Distal shunts, such as the Winter's, involve puncturing the glans (the distal part of the penis) into one of the cavernosa, where the old, stagnant blood is held. This causes the blood to leave the penis and return to the circulation. This procedure can be performed by a urologist at the bedside. Winter's shunts are often the first invasive technique used, especially in hematologic induced priapism, as it is relatively simple and repeatable over time.
Proximal shunts, such as the Quackel's,[clarification needed] are more involved and entail operative dissection in the perineum to where the corpora meet the spongiosum, making an incision in both, and suturing both openings together.
Shunts created between corpora cavernosa and saphenous vein called Grayhack shunt can be done though rarely.
Priapism in females (continued, painful erection of the clitoris) is also known as clitorism.
Priapism is a persistent, usually painful, erection that lasts for more than four hours and occurs without sexual stimulation. The condition develops when blood in the penis becomes trapped and unable to drain. If the condition is not treated immediately, it can lead to scarring and permanent erectile dysfunction.
It can occur in all age groups, including newborns. However, it usually affects men between the ages of 5 to 10 years and 20 to 50 years.
There are two categories of priapism: low-flow and high-flow.
· Low flow: This type of priapism is the result of blood being trapped in the erection chambers. It often occurs without a known cause in men who are otherwise healthy, but also affects men with sickle-cell disease, leukemia (cancer of the blood) or malaria.
· High flow: High flow priapism is more rare than low-flow and usually less painful. It is the result of a ruptured artery from an injury to the penis or the perineum (area between the scrotum and anus), which prevents blood in the penis from circulating normally.
What causes priapism?
Sickle cell anemia
Some adult cases of priapism are the result of sickle-cell disease and approximately 42% of all adults with sickle-cell will eventually develop priapism.
A common cause of priapism is the use and/or misuse of medications. Drug-related priapism includes drugs such as Desyrel used to treat depression or Thorazine, used to treat certain mental illnesses. For people who have erectile dysfunction, injection therapy medications to treat the condition may also cause priapism.
Other causes of priapism include:
· Trauma to the spinal cord or to the genital area
· Black widow spider bites
· Carbon monoxide poisoning
· Illicit drug use, such as marijuana and cocaine
In rare cases, priapism may be related to cancers that can affect the penis and prevent the outflow of blood.
How is priapism diagnosed?
If you experience priapism, it is important that you seek medical care immediately. Tell your doctor:
· The length of time you have had the erection
· How long your erection usually lasts
· Any medication or drugs, legal or illegal, which you have used. Be honest with your doctor, illegal drug use is particularly relevant since both marijuana and cocaine have been linked to priapism.
· Whether or not priapism followed trauma to that area of the body.
Your doctor will review your medical history and perform a thorough physical examination to determine the cause of priapism. This will include checking the rectum and the abdomen for evidence of unusual growths or abnormalities that may indicate the presence of cancer.
After the physical exam is complete, the doctor will take a blood-gas measurement of the blood from the penis. During this test, a small needle is placed in the penis, some of the blood is drawn and then it is sent to a lab for analysis. This provides a clue as to how long the condition has been present and how much damage has occurred.
How is priapism treated?
The goal of all treatment is to make the erection go away and preserve future erectile function. If a person receives treatment within four to six hours, the erection can almost always be reduced with medication. If the erection has lasted less than four hours, decongestant medications, which may act to decease blood flow to the penis, may be very helpful. Other treatment options include:
· Ice packs: Ice applied to the penis and perineum may reduce swelling.
· Surgical ligation: Used in cases where an artery has been ruptured, the doctor will ligate (tie off) the artery that is causing the priapism in order to restore normal blood flow.
· Intracavernous injection: Used for low-flow priapism, during this treatment drugs known as alpha-agonists are injected into the penis that cause the veins to narrow reducing blood flow to the penis causing the swelling to subside.
· Surgical shunt: Also used for low-flow priapism, a shunt is a passageway that is surgically inserted into the penis to divert the blood flow and allow circulation to return to normal.
· Aspiration: After numbing the penis, doctors will insert a needle and drain blood from the penis to reduce pressure and swelling.
If you suspect that you are experiencing priapism, you should not attempt to treat it yourself. Instead seek emergency as soon as possible.
What is the outlook for people with priapism?
As long as treatment is prompt, the outlook for most people is very good. However, the longer medical attention is delayed, the greater the risk of permanent erectile dysfunction.
Mayville A. Visitacion