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Kawasaki disease

Alternative Names: Kawasaki syndrome, lymph node syndrome and mucocutaneous lymph node syndrome

Definition:
Kawasaki disease (KD) is an autoimmune disease that manifests as a systemic necrotizing medium-sized vessel vasculitis and is largely seen in children under five 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 six 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 conjunctivae 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 10 days, but may range from five to 25 days. The disorder was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.

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Kawasaki disease affects boys more than girls with people of Asian ethnicity, particularly Japanese and Korean most susceptible as well as people of Afro-Caribbean ethnicity. The disease was rare in Caucasians until the last few decades and incidence rate fluctuates from country to country.

By far the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000).

However its incidence in the United States is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than five years of age. Approximately 2,000-4,000 cases are identified in the United States each year.

In the United Kingdom, estimates of incidence rate vary because of the rarity of Kawasaki disease. However Kawasaki disease is believed to affect fewer than 1 in every 25,000 people.  Incidence of the disease doubled from 1991 to 2000 however, with 4 cases in per 100,000 children in 1991 compared with a rise of 8 cases per 100,000 in 2000.

Symptoms:
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 the mucous 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. The oropharynx 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 fifth day of fever. Cutaneous exanthema may comprise macular-papular erythematous and fissure lesions, the most common type, in addition to urticariform type 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, aseptic meningitis, pneumonitis, lymphadenitis, 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

Causes:
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 reported 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

Risk Factors:
Three things are known to increase your child’s risk of developing Kawasaki disease, including:

*Age. Children under 5 years old are most at risk of Kawasaki disease.

*Sex. Boys are slightly more likely than girls are to develop Kawasaki disease.

*Ethnicity. Children of Asian descent, such as Japanese or Korean, have higher rates of Kawasaki disease.

Diagnosis:
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.

Investigations:
A physical examination will demonstrate many of the features listed above.

Blood tests:
*Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis

*Erythrocyte sedimentation rate (ESR) will be elevated

*C-reactive protein (CRP) will be elevated

*Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other optional tests
*Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis

*Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.

*Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder

*Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth

*Lumbar puncture may show evidence of aseptic meningitis

*Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

Complications :
•Inflammation of the blood vessels (vasculitis)
•Inflammation of the heart muscle (myocarditis)
•Aneurysms in arteries that increase the risk of blood clots and heart attacks

Treatment :
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 as 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[38] 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

Prognosis:
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.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://en.wikipedia.org/wiki/Kawasaki_disease
http://www.bbc.co.uk/health/physical_health/conditions/kawasaki2.shtml
http://www.vaheart.com/kawasaki-disease/
http://www.mayoclinic.com/health/kawasaki-disease/DS00576/DSECTION=risk-factors

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Kabuki Syndrome

Definition:

Kabuki syndrome is a rare disorder characterized by unusual facial features, skeletal abnormalities, and intellectual impairment. Abnormalities in different organ systems can also be present, but vary from individual to individual. There is no cure for Kabuki syndrome, and treatment centers on the specific abnormalities, as well as on strategies to improve the overall functioning and quality of life of the affected person.Kabuki syndrome appears to be found equally in males and females.

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Scientific research conducted over the past two decades suggests that Kabuki syndrome may be associated with a change in the genetic material. However, it is still not known precisely what this genetic change may be and how this change in the genetic material alters growth and development in the womb to cause Kabuki syndrome.

In Japan, it has been estimated that about one in 32,000 babies is born with Kabuki syndrome (which could mean about 50 cases a year in the UK). Although originally reported in Japan, cases have now been described around the world.

It was discovered and described in 1981 by two Japanese groups, led by the scientists Niikawa and Kuroki (hence the name). It is named Kabuki Syndrome because of the facial resemblance of affected individuals with white Kabuki makeup, a Japanese traditional theatrical form. On the Kabuki Syndrome listserv, children with this syndrome are called Kabuki Kids, or KKs.

Symptoms:
People with the syndrome have an unusual facial appearance, characterised by large eyes, long and thick eyelashes and arched eyebrows.

Infants usually have normal birth weight, but most will not grow as quickly as expected. Delay in speech and language development is very common. Many infants also have problems feeding.

Kabuki syndrome is very complex and there are many other manifestations.

Cause:
The cause is unknown – a genetic abnormality is suspected but has not yet been identified.It’s likely that if a gene is involved it’s a rare and random mutation that occurs sporadically.

Inheritance is thought to be autosomal dominant or X-linked recessive; several chromosomal abnormalities have been found, but none of them appear to be specific to Kabuki Syndrome. In August 2010, a study found that two thirds of the cases have a loss-of-function mutation in the MLL2 gene, which is coding for a histone methyltransferase; it can participate in epigenetic programming, and is thought to contribute to developmental processes.

Diagnosis
The diagnosis of Kabuki syndrome relies on physical exam by a physician familiar with the condition and by radiographic evaluation, such as the use of x rays or ultrasound to define abnormal or missing structures that are consistent with the criteria for the condition (as described above). A person can be diagnosed with Kabuki syndrome if they possess characteristics consistent with the five different groups of cardinal symptoms: typical face, skin-surface abnormalities, skeletal abnormalities, mild to moderate mental retardation, and short stature.

Although a diagnosis may be made as a newborn, most often the features do not become fully evident until early childhood. There is no laboratory blood or genetic test that can be used to identify people with Kabuki syndrome.

Treatment ;
There is no cure for Kabuki syndrome. Treatment of the syndrome is variable and centers on correcting the different manifestations of the condition and on strategies to improve the overall functioning and quality of life of the affected individual.

For children with heart defects, surgical repair is often necessary. This may take place shortly after birth if the heart abnormality is life threatening, but often physicians will prefer to attempt a repair once the child has grown older and the heart is more mature. For children who experience seizures, lifelong treatment with anti-seizure medications is often necessary.

Children with Kabuki syndrome often have difficulties feeding, either because of mouth abnormalities or because of poor digestion. In some cases, a tube that enters into the stomach is surgically placed in the abdomen, and specially designed nutritional liquids are administered through the tube directly into the stomach.

People with Kabuki syndrome are at higher risk for a variety of infections, most often involving the ears and the lungs. In cases such as these, antibiotics are given to treat the infection, and occasionally brief hospital stays are necessary. Most children recover from these infections with proper treatment.

Nearly half of people affected by Kabuki syndrome have some degree of hearing loss. In these individuals, formal hearing testing is recommended to determine if they might benefit from a hearing-aid. A hearing aid is a small mechanical device that sits behind the ear and amplifies sound into the ear of the affected individual. Occasionally, hearing loss in individuals with Kabuki syndrome is severe, approaching total hearing loss. In these cases, early and formal education using American Sign Language as well as involvement with the hearing-impaired community, schools, and enrichment programs is appropriate.

Children with Kabuki syndrome should be seen regularly by a team of health care professionals, including a primary care provider, medical geneticist familiar with the condition, gastroenterologist, and neurologist. After growth development is advanced enough (usually late adolescence or early adulthood), consultation with a reconstructive surgeon may be of use to repair physical abnormalities that are particularly debilitating.

During early development and progressing into young adulthood, children with Kabuki syndrome should be educated and trained in behavioral and mechanical methods to adapt to any disabilities. This program is usually initiated and overseen by a team of health care professionals including a pediatrician, physical therapist, and occupational therapist. A counselor specially trained to deal with issues of disabilities in children is often helpful is assessing problem areas and encouraging healthy development of self-esteem. Support groups and community organizations for people with disabilities often prove useful to the affected individuals and their families, and specially equipped enrichment programs should be sought. Further, because many children with Kabuki syndrome have poor speech development, a consultation and regular session with a speech therapist is appropriate.

Prognosis:
The abilities of children with Kabuki syndrome vary greatly. Most children with the condition have a mild to moderate intellectual impairment. Some children will be able to follow a regular education curriculum, while others will require adaptations or modifications to their schoolwork. Many older children may learn to read at a functional level.

The prognosis of children with Kabuki syndrome depends on the severity of the symptoms and the extent to which the appropriate treatments are available. Most of the medical issues regarding heart, kidney or intestinal abnormalities arise early in the child’s life and are improved with medical treatment. Since Kabuki syndrome was discovered relatively recently, very little is known regarding the average life span of individuals affected with the condition, however, present data on Kabuki syndrome does not point to a shortened life span.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/kabuki2.shtml
http://www.healthline.com/galecontent/kabuki-syndrome-1
http://en.wikipedia.org/wiki/Kabuki_syndrome

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Juvenile Idiopathic Arthritis(JIA)

Definition:
Arthritis is an inflammation of the joints, with pain or stiffness. It may be acute or chronic. Acute arthritis is also called septic arthritis and may affect one or more joints.

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Juvenile Idiopathic Arthritis (JIA) (once called Juvenile Rheumatoid Arthritis or JRA) is a type of arthritis that affects children who are under 16 years of age. This is an autoimmune disease that causes joints to swell and become stiff, sometimes hindering a child’s mobility. It can affect any joint, and in some cases it can affect internal organs and eyes as well. Symptoms can come and go, flare-up on occassion, while others have symptoms that never go away.

There are three types of JIA, which are diagnosed according to symptoms and blood tests:-
•Oligoarticular JIA – the most common kind of childhood arthritis, which often starts at the age of two or three. The problem is limited to four joints or fewer, which become swollen and painful. Sometimes the eyes are affected, too. It is also known as pauciarticular arthritis.

•Polyarticular JIA – affects five or more joints. It can start at any age, from a few months onwards, and usually spreads quite quickly from one joint to another. Children often feel generally unwell, sometimes with a fever.

•Systemic onset JIA – affects the whole body, and causes fever and rashes as well as inflamed and painful joints. It usually starts in children under five but can affect children of any age. It used to be called Still’s disease.

About one in 1,000 children has arthritis. In many cases, the inflammation stops in late childhood, but about one-third of children affected have problems that last into their adult life.

Juvenile idiopathic arthritis affects somewhere between 8 and 150 of every 100,000 children, depending on the analysis. Of these children, 50 percent have pauciarticular JIA, 40 percent have polyarticular JIA and 10 percent have systemic JIA.

Symptoms:
Symptoms depend on the type of arthritis and joints affected . They could include:
*Pain
*Fever
*Joint Pain
*Knee Pain
*Joint Swelling
*Ankle Pain
*Hip Pain
*Swollen Lymph Nodes
*Elbow Pain
*Wrist Pain
*Limited Range of Motion
*Morning Stiffness
*Migratory Joint Pain
*Hip Stiff
*Back Pain
*Rash
*Chronic pain
*Shoulder Pain
*Foot Pain
*Dry Mouth
*Joint Stiffness
*Eye Pain
*Arm Pain
*Inflamed Joint
*Double-Vision
*Visual Disturbance
*Joint Tenderness
*Enlarged Spleen
*Eye Redness
*Visual Impairment
*Joint Erythema
*Joints Warm
*Blurred vision
*Knee Stiff
*Feels Hot to Touch
*Light Hurts Eyes
*Uneven Limb Lengths

Causes:
So far the actual cause of JIA remains a mystery. However, the disorder is autoimmune   – meaning that the body’s own immune system starts to attack and destroy cells and tissues (particularly in the joints) for no apparent reason. It is believed that the immune system gets provoked by changes in the environment or perhaps there is an error in the gene. Experimental studies have shown that certain viruses that have mutated may be able to trigger JIA. JIA appears to be more common in young girls and the disease is most common in Caucasians.   Associated factors that may worsen or have been linked to rheumatoid arthritis include the following:

*genetic predisposition; it appears that when one family member has been diagnosed with rheumatoid arthritis, the chances are higher that other family members or
*siblings may also develop arthritis

*females are more likely to develop rheumatoid arthritis than males at all ages

*there is a strong belief that psychological stress may worsen the symptoms of rheumatoid arthritis. However, when the emotional stress is under control the arthritis symptoms do not always disappear suggesting that the association is not straightforward

*even though no distinct immune factor has been isolated as a cause of arthritis, there are some experts who believe that the triggering factor may be something like a virus which then disappears from the body after permanent damage is done

*because rheumatoid arthritis is more common in women, there is a belief that perhaps sex hormones may be playing a role in causing or modulating arthritis.

Unfortunately, neither sex hormone deficiency nor replacement has been shown to improve or worsen arthritis.
The cause of JIA, as the word idiopathic suggests, is unknown and currently an area of active research. Current understanding of JIA suggests that it arises in a genetically susceptible individual due to environmental factors.

Diagnosis:
Diagnosis of JIA is difficult because joint pain in children can be from many other causes. There is no single test that can confirm the diagnosis and most physicians use a combination of blood tests, x rays and the clinical presentation to make an initial diagnosis of JIA. The blood tests measure antibodies and the rheumatoid factor. Unfortunately, the rheumatoid factor is not present in all children with JIA. Moreover in most children the blood work is usually normal. X rays are obtained to ensure that the joint pain is not from a fracture, cancer, infection or a congenital abnormality.

In most cases, fluid from the joint is aspirated and analyzed. This test often helps in making a diagnosis of JIA by ruling out other causes of joint pain

Treatment :
The treatment of JIA is best undertaken by an experienced team of health professionals, including pediatric rheumatologists, nurse specialists, physiotherapists, and occupational therapists. Many others in the wider health and school communities also have valuable roles to play, such as ophthalmologists, dentists, orthopaedic surgeons, school nurses and teachers, careers advisors and, of course local general practitioners, paediatricians and rheumatologists. It is essential that every effort is made to involve the affected child and their family in disease education and balanced treatment decisions.

The major emphasis of treatment for JIA is to help the child regain normal level of physical and social activities. This is accomplished with the use of physical therapy, pain management strategies and social support.

There have been very beneficial advances in drug treatment over the last 20 years. Most children are treated with non-steroidal anti-inflammatory drugs and intra-articular corticosteroid injections. Methotrexate is a powerful drug which helps suppress joint inflammation in the majority of JIA patients with polyarthritis  (though less useful in systemic arthritis). Newer drugs have been developed recently, such as TNF alpha blockers, such as etanercept.  There is no controlled evidence to support the use of alternative remedies such as specific dietary exclusions, homeopathic treatment or acupuncture. However, an increased consumption of omega-3 fatty acids proved to be beneficial in two small studies.

Celecoxib has been found effective in one study.

Other aspects of managing JIA include physical and occupational therapy. Therapists can recommend the best exercise and also make protective equipment. Moreover, the child may require the use of special supports, ambulatory devices or splints to help them ambulate and function normally.

Surgery is only used to treat the most severe cases of JIA. In all cases, surgery is used to remove scars and improve joint function.

Home remedies that may help JIA includes getting regular exercises to increase muscle strength and joint flexibility. Swimming is perhaps the best activity for all children with JIA. Stiffness and swelling can also be reduced with application of cold packs but a nice warm bath or shower can also improve joint mobility

Complications:
JIA is a chronic disorder which if neglected can lead to serious complications. Proper follow up with health professionals can significantly reduce the chance of developing complications.

Eyes can be affected in some types of JIA. The inflamed eyes if left untreated can result in glaucoma, scars, cataracts and even blindness. Often the eye inflammation occurs without symptoms and thus it is important for all children to get regular eye checkups from an eye physician.

Growth retardation is common in children with JIA. Moreover, the medications (corticosteroids) used to treat JIA have potent side effects that can limit growth.

Children who delay treatment or do not participate in physical therapy can often develop joint deformities of the hand and fingers. Over time hand function is lost and almost impossible to recover.

Occupational therapy:
The best approach to treating a child with JIA involves a team of medical professionals including a rheumatologist, occupational therapist (OT), physical therapist, nurse and social worker.

The role of the OT is to help children participate as fully and independently as possible in their daily activities or “occupations”,  by preventing psychological and physical dependency. The aim is to maximize quality of life, and minimize disruption to the child’s and family’s life. OTs work with children, their families and schools, to come up with an individualized plan which is based on the child’s condition, limitations, strengths and goals. This is accomplished by ongoing assessments of a child’s abilities and social functioning. The plan may include the use of a variety of assistive devices, such as splints, that help a person perform tasks. The plan may also involve changes to the home, encouraging use of uninvolved joints, as well as providing the child and their family with support and education about the disease and strategies for managing it.  OT interventions will be changed depending on the progression and remission of JIA, in order to promote age-appropriate self-sufficiency. Early OT involvement is essential.

Self-care:
OTs can provide many strategies to assist children in their dressing routine. Clothes with easy openings and Velcro, as well as devices, such as buttonhooks and zipper pulls can be used. For children who have difficulty bending, a long handled reacher and sock aid is recommended. OTs may also show children how to sit during dressing so less strain is put on their joints.

OTs can help children maintain cleanliness through recommending assistive devices. For children who have trouble reaching all areas of their body, a long handled sponge with a soft grip can be provided. If children find it difficult to sit in a bath or stand in a shower, an OT can prescribe a bath bench or bath seat to be installed to help the child remain in a pain free position. If tooth brushing is challenging, a toothbrush with a larger, soft grip or an electric toothbrush may be recommended. For flossing, a flosser with an adapted handle may be provided.  Long handled hairbrushes may be used by children who have difficulty reaching the back of their head. Razors handles can be adapted for easier grip, or an electric razor may be used for shaving. The OT can also show girls wishing to use make-up, ways of increasing the sizes of the handles of make-up application tools for easier grip.

For children with pain in their hands and wrists, utensils and devices that are lightweight with large handles as well as other devices (such as angled knives, strap-on utensils, jar and bottle openers, turning handles, door knob extensions, etc.) can be provided to make the task easier, less painful and more enjoyable.[32] Tilted glasses can be used for children who have neck stiffness. Education can be provided about good eating habits that help control bone loss caused by inactivity and drug side effects. Occupational therapists provide a myriad of strategies to assist children with JIA in performing self-care tasks.

Leisure:
One of the best ways OTs can help children with JIA participate in activities with their friends is by helping them make their home exercise programs into play. Exercises are prescribed by both physiotherapists and OTs to increase the amount a child can move a joint and strengthen the joint to decrease pain and stiffness and prevent further limitations in their joint movements. OTs can provide children with age appropriate games and activities to allow the children to practice their exercises while playing and socializing with friends. Examples are crafts, swimming and non-competitive sports.

OTs will often prescribe custom made orthotics which are devices that support and correct body position and function. Orthotics help keep the child’s body in good alignment. Orthotics reduce discomfort in the legs and back when the child participates in physical activities such as sports. Splints can be used to support the joints during activity, to reduce the child’s pain and increase participation in their preferred leisure activities. Resting splints may be prescribed for children to wear during the night to reduce swelling and stiffness in joints, allowing children to have less pain and stiffness while participating in play activities.Furthermore, working splints are used to support the joint and relieve pain while working the with hands such as during crafts. A series of casts might be used to gradually extend shortened muscles allowing for increased participation in leisure activities.

OTs can help a child learn how to interact with their classmates and friends by collaboratively brainstorming strategies, role playing and modeling. OTs also help children see what activities they are good at and which ones give them difficulty. Furthermore, OTs can help children learn to communicate their pain to others. Benefits of OT treatment include: improved social interaction, improved self-confidence and a positive self-image. OTs can help children build friendships with other children suffering from similar diseases to help them feel less alone or less different from others. Many OTs run summer camps for children with similar diseases so children can get to know others with their disease. Education sessions on JIA and leisure, and activities such as swimming, canoeing and nature trails are common.

For children who find that cool or damp weather make it hard to play with friends outside, OTs can give ideas for clothing that will keep the child warm and dry without limiting movement. An example of this is biking gloves which allow children to move their fingers while still keeping their hands warm, as opposed to large winter gloves which limit hand function. Warm pajamas and electric blankets can reduce pain and improve sleep.

Prognosis:
With proper therapy, some children do improve with time and lead normal lives. However, severe cases of JIA which are not treated promptly can lead to poor growth and worsening of joint function. In the last two decades, significant improvements have been made in treatment of JIA and most children can lead a decent quality of life. The prognosis of JIA depends on prompt recognition and treatment. Finally, it is important for both the child and family member to be educated about the disorder. The more educated the person, the better the care you can receive. Chronic JIA is no longer the dreaded disease where one remains home bound. Many children with JIA have gone on to play professional sports and have a variety of successful careers

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/in_depth/arthritis/aboutarthritis_children.shtml
http://en.wikipedia.org/wiki/Juvenile_idiopathic_arthritis
http://www.healthline.com/channel/juvenile-rheumatoid-arthritis_symptoms
http://www.seattlecca.org/diseases/juvenileidiopathicarthritis-overview.cfm?gclid=CLCm7aSepakCFcO8Kgodl2kxyQ

http://apps.ashland.edu/index.php/File:Arthritic_joints.jpg

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Infant jaundice

Definition:
Infant jaundice is a yellow discoloration in a newborn baby’s skin and eyes. Infant jaundice occurs because the baby’s blood contains an excess of bilirubin (bil-ih-ROO-bin), a yellow-colored pigment of red blood cells. Jaundice isn’t a disease itself but the name given to the yellow appearance of skin and the conjunctiva (whites) of the eyes.

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Infant jaundice is a common condition, particularly in babies born before 38 weeks gestation (preterm babies) and breast-fed babies. Infant jaundice usually occurs because a baby’s liver isn’t mature enough to get rid of bilirubin in the bloodstream. In some cases, an underlying disease may cause jaundice.

Infant jaundice can be concerning as although the majority of causes are easily treated, some rarer causes are very serious. Also, high levels of unconjugated bilirubin can cause brain damage. This is virtually never seen now due to treatment with UVB light, but it means that it is very important that the baby receives proper treatment.

Types of Infant jaundice:
The most common types of jaundice are:

Physiological (normal) jaundice: occurring in most newborns, this mild jaundice is due to the immaturity of the baby’s liver, which leads to a slow processing of bilirubin. It generally appears at 2 to 4 days of age and disappears by 1 to 2 weeks of age.

Jaundice of prematurity: occurs frequently in premature babies since they are even less ready to excrete bilirubin effectively. Jaundice in premature babies needs to be treated at a lower bilirubin level than in full term babies in order to avoid complications.

Breastfeeding jaundice: jaundice can occur when a breastfeeding baby is not getting enough breast milk because of difficulty with breastfeeding or because the mother’s milk isn’t in yet. This is not caused by a problem with the breast milk itself, but by the baby not getting enough to drink.

Breast milk jaundice: in 1% to 2% of breastfed babies, jaundice may be caused by substances produced in their mother’s breast milk that can cause the bilirubin level to rise. These can prevent the excretion of bilirubin through the intestines. It starts after the first 3 to 5 days and slowly improves over 3 to 12 weeks.

Symptoms:
The main symptom of jaundice is yellow colouring of the skin and conjunctiva of the eyes. Jaundice can also make babies sleepy which can lead to poor feeding. Poor feeding can make jaundice worse as the baby can become dehydrated.

If a baby has conjugated jaundice, it may have white chalky stool (poo) and urine that is darker than normal. (The bilirubin that normally colours the stool is excreted in the urine.)

Medical advise should be sought urgently if:
•Jaundice is present in the first 24 hours of life
•Jaundice is present when the baby is 10 days old
•The baby has problems feeding or is very sleepy
•The stools are pale or the urine is very dark

Causes:
The main cause of jaundice is:
Excess bilirubin (hyperbilirubinemia). Bilirubin is the substance that causes the yellow color of jaundice. It’s a normal part of the waste produced when “used” red blood cells are broken down. Normally, the liver filters bilirubin from the bloodstream and releases it into the intestinal tract. Before birth, a mother’s liver removes bilirubin from the baby’s blood. The liver of a newborn is immature and often can’t remove bilirubin quickly enough, causing an excess of bilirubin. Jaundice due to these normal newborn conditions is called physiologic jaundice, and it typically appears on the second or third day of life.Other causes

A baby may have an underlying disorder that is causing jaundice. In these cases, jaundice often appears much earlier or much later than physiologic jaundice.

Diseases or conditions that can cause jaundice include:
*Internal bleeding (hemorrhage)
*An infection in your baby’s blood (sepsis)
*Other viral or bacterial infections
*An incompatibility between the mother’s blood and the baby’s blood
*A liver malfunction
*An enzyme deficiency
*An abnormality of your baby’s red blood cells

Risk Factors:
Problems with the blood may lead to a rapid breakdown of cells (haemolysis) – if the mother’s blood type isn’t compatible with her baby’s. For example, she may make antibodies that attack and destroy her baby’s red blood cells.

Hormone deficiencies such as low levels of thyroid hormone (hypothyroidism) or pituitary gland hormones (hypopituitarism) can trigger jaundice.

There may be inherited genetic problems with the enzymes that convert or break down bilirubin – these include rare conditions such as Crigler-Najjar syndrome, Gilbert’s syndrome, galactosaemia and tyrosinaemia.

There may be problems with the liver, such as biliary atresia, in which the tubes that drain bile from the liver are blocked. If spotted early, an operation can prevent long-term damage (which is why it is important to investigate jaundice that is still there at 10 days).

Diagnosis:
Doctors, nurses, and family members will watch for signs of jaundice at the hospital, and after the newborn goes home.

Any infant who appears jaundiced should have bilirubin levels measured right away. This can be done with a blood test.

Many hospitals check total bilirubin levels on all babies at about 24 hours of age. Hospitals use probes that can estimate the bilirubin level just by touching the skin. High readings need to be confirmed with blood tests.

Tests that will likely be done include:
•Complete blood count
•Coomb’s test
•Reticulocyte count
Further testing may be needed for babies who need treatment or whose total bilirubin levels are rising more quickly than expected.

Treatment:
Treatment is usually not needed.

When determining treatment, the doctor must consider:

•The baby’s bilirubin level
•How fast the level has been rising
•Whether the baby was born early (babies born early are more likely to be treated at lower bilirubin levels)
•How old the baby is now
Your child will need treatment if the bilirubin level is too high or is rising too quickly.

Keep the baby well hydrated with breast milk or formula. Frequent feedings (up to 12 times a day) encourage frequent bowel movements, which help remove bilirubin through the stools. Ask your doctor before giving your newborn extra formula.

Some newborns need to be treated before they leave the hospital. Others may need to go back to the hospital when they are a few days old. Treatment in the hospital usually lasts 1 to 2 days.

Sometimes special blue lights are used on infants whose levels are very high. This is called phototherapy. These lights work by helping to break down bilirubin in the skin.

The infant is placed under artificial light in a warm, enclosed bed to maintain constant temperature. The baby will wear only a diaper and special eye shades to protect the eyes. The American Academy of Pediatrics recommends that breastfeeding be continued through phototherapy, if possible. Rarely, the baby may have an intravenous (IV) line to deliver fluids.

If the bilirubin level is not too high or is not rising quickly, you can do phototherapy at home with a fiberoptic blanket, which has tiny bright lights in it. You may also use a bed that shines light up from the mattress.

•You must keep the light therapy on your child’s skin and feed your child every 2 to 3 hours (10 to 12 times a day).
•A nurse will come to your home to teach you how to use the blanket or bed, and to check on your child.
•The nurse will return daily to check your child’s weight, feedings, skin, and bilirubin levels.
•You will be asked to count the number of wet and dirty diapers.
In the most severe cases of jaundice, an exchange transfusion is required. In this procedure, the baby’s blood is replaced with fresh blood. Treating severely jaundiced babies with intravenous immunoglobulin may also be very effective at reducing bilirubin levels.

Prognosis:
Usually newborn jaundice is not harmful. For most babies, jaundice usually gets better without treatment within 1 to 2 weeks.

Very high levels of bilirubin can damage the brain. This is called kernicterus. However, the condition is almost always diagnosed before levels become high enough to cause this damage.

For babies who need treatment, the treatment is usually effective

Possible Complications:–
Rare, but serious, complications from high bilirubin levels include:

•Cerebral palsy
•Deafness
•Kernicterus — brain damage from very high bilirubin levels

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.nlm.nih.gov/medlineplus/ency/article/001559.htm
http://www.mayoclinic.com/health/infant-jaundice/DS00107
http://www.bbc.co.uk/health/physical_health/conditions/jaundice2.shtml

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Ailmemts & Remedies Pediatric

Impetigo

Definition:–

Impetigo is a highly contagious skin infection which is most commonly seen in babies as well as small children. It causes red sores that can break open, ooze fluid, and develop a yellow-brown crust. These sores can occur anywhere on the body but most often appear around the mouth and nose.

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It can occur in adults but is seen far more often in children. Impetigo is contagious and can be spread to others through close contact or by sharing towels, sheets, clothing, toys, or other items. Scratching can also spread the sores to other parts of the body.

Symptoms:–
You or your child may have impetigo if you have sores:

*Small red spots typically appear on the skin of the face (especially around the mouth and nose), neck or hands, although any part of the body may be affected.The spots may be clustered or merge together. The centre of each one rapidly becomes a blister, which then bursts, oozing a typical golden fluid. Crusts form over the red spots, which may be itchy or slightly sore.

* The sores begin as small red spots, then change to blisters that eventually break open. The sores are generally not painful, but they may be itchy.

*That ooze fluid and look crusty. Sores often look like they have been coated with honey or brown sugar.

*That increase in size and number. Sores may be as small as a pimple or larger than a coin.

*In babies, impetigo often occurs in the nappy area.

*New crops of spots may appear over several days or weeks (if untreated) and the infection is easily spread to other parts of the body.

Causes:–
Impetigo is caused by one of two kinds of bacteria—strep (streptococcus) or staph (staphylococcus). Often these bacteria enter the body when the skin has already been irritated or injured because of other skin problems such as eczema, poison ivy, insect bites or scratches, or nappy rash (in babies), chickenpox, burns, or cuts. Children may get impetigo after they have had a cold or allergies that have made the skin under the nose raw.In these situations the bacteria can more easily penetrate the skin’s defences and establish an infection. However, impetigo can also develop in completely healthy skin.
Risk Factors
Factors that increase your chance for impetigo include:

*Age: preschool and school-aged children
*Touching a person with impetigo
*Touching the clothing, towels, sheets, or other items of a person with impetigo
*Poor hygiene, particularly unwashed hands and dirty fingernails
*Crowded settings where there is direct person-to-person contact, such as schools and the military
*Warm, humid environment
*Seasonal: Summer
*Poor health or weakened immune system
*Tendency to have skin problems such as eczema , poison ivy , or skin allergy
*Cuts, scratches, insect bites , or other injury or trauma to the skin
*Chickenpox
*Lice infections (like scabies , head lice , or public lice ), which cause scratching

Diagnosis:–
Doctors can usually diagnose impetigo just by looking at  child’s skin. Sometimes doctor gently remove a small piece of a sore to send to a lab in order to identify the bacteria. If you or your child have other signs of illness,  the doctor may order blood or urine tests

Treatment:–
Impetigo is treated with antibiotics. For cases of mild impetigo, a doctor will prescribe an antibiotic ointment or cream to put on the sores. For cases of more serious impetigo, a doctor may also prescribe antibiotic pills.

After 3 days of treatment, you or your child should begin to get better. A child can usually return to school or daycare after 48 hours of treatment. If you apply the ointment or take the pills exactly as prescribed, most sores will be completely healed in 1 week.

At home, you should gently wash the sores with soap and water before you apply the medicine. If the sores are crusty, soak them in warm water for 15 minutes, scrub the crusts with a washcloth to remove them, and pat the sores dry. Do not share washcloths, towels, pillows, sheets, or clothes with others and be sure to wash these items in hot water before you use them again.

Try not to scratch the sores because scratching can spread the infection to other parts of the body. You can help prevent scratching by keeping your child’s fingernails short and covering sores with gauze or bandages.

Call your doctor if an impetigo infection does not improve after 3 or 4 days or if you notice any signs that the infection is getting worse such as fever, increased pain, swelling, warmth, redness, or pus.

Prevention:–
f you know someone who has impetigo, try to avoid close contact with that person until his or her infection has gone away. You should also avoid sharing towels, pillows, sheets, clothes, toys, or other items with an infected person. If possible, wash any shared items in hot water before you use them again.

If you or your child has impetigo, scratching the sores can spread the infection to other areas of your body and to other people. Keeping the sores covered can help you or your child resist scratching them. Washing your or your child’s hands with soap can also prevent spreading the infection.

If your child has a cut or insect bite, covering it with antibiotic ointment or cream can help prevent impetigo.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
*http://www.bing.com/images/search?q=pictures+of+impetigo&id=66954188E428748C5C3FE74DC0B51F5FB5506A49&FORM=IGRE2
*http://www.everydayhealth.com/health-center/impetigo.aspx
*http://www.bbc.co.uk/health/physical_health/conditions/impetigo2.shtml

*http://www.lifescript.com/Health/A-Z/Conditions_A-Z/Conditions/I/Impetigo.aspx?gclid=CK-inOjLmKkCFcW8KgodIhWnwQ&trans=1&du=1&ef_id=S81NxKI5DCoAAIRd:20110603013319:s

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