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Tuesday, July 19, 2011

Kawasaki Disease, A Dramatic Story

Posted by Dr Prahallad Panda on 2:07 PM Comments

Recently, there was a news in the MedicalXpress that a 4 years child was saved from fatal outcome due to Facebook, the social networking site.
On mother's Day. One mother found her son sick with rash and high fever; posted the picture in the Faceboook. She took the boy to the hospital where it was provisionally diagnosed as streptococcal infection and treatment started accordingly.
In the next morning the condition of her child was worse with swelling of hand and some parts of body, and that picture was also posted in the status of the mother in her Facebook page. That day the doctor reviewed the child and changed his diagnosis to Scarlet fever.
A few moments later her phone rang telling her she needed to get her child to the hospital immediately. The woman on the other end of the phone was her friend, whose son, only a few years earlier, was hospitalized with a rare disease known as Kawasaki disease that matched with the symptoms of her friend's child.
Again after some time a cousin of the mother, a pediatric cardiologist, called with the same thought. She rushed her son to the hospital where he spent the next three weeks being treated for Kawasaki disease and liver complications.
Kawasaki disease otherwise known as Mucocutaneous lymph node syndrome; Infantile polyarteritis is a rare condition in children that involves inflammation of the blood vessels.
Kawasaki disease occurs most frequently in Japan, where the disease was first discovered. In the United States, after congenital heart defects, Kawasaki disease is the leading cause of heart disease in children. Most of these patients are younger than age 5. The disease occurs more often in boys than in girls.
The exact cause of this disease is not known, but thought to be an autoimmune disease, affecting the blood vessels of body, particularly blood vessels of heart, the coronaries.
 Kawasaki diseaseImage via Wikipedia
Kawasaki disease often begins with a high and persistent fever greater than 102°F, often continuing for at least 5 days. The fever may last for up to 2 weeks and does not usually go away with normal doses of paracetamol or ibuprofen.
Other symptoms often include:
  • Extremely bloodshot or red eyes (without pus or drainage)
  • Bright red, chapped, or cracked lips
  • Red mucous membranes in the mouth
  • Strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue
  • Red palms of the hands and the soles of the feet
  • Swollen hands and feet
  • Skin rashes on the middle of the body, NOT blister-like
  • Peeling skin in the genital area, hands, and feet (especially around the nails, palms, and soles)
  • Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
  • Joint pain and swelling, frequently on both sides of the body
Additional symptoms may include:
  • Irritability
  • Diarrhea, vomiting, and abdominal pain
  • Cough and runny nose
There is no specific test to diagnose Kawasaki disease. The diagnosis is usually made based on the patient having most of the classic symptoms.
However, the following tests may be performed to exclude other causes of fever, joint pain, enlarged lymph node and rash:
X-ray showing Aneurysmal enlargement of the co...Aneurysmal Coronary Arteries
  • Chest x-ray
  • C-reactive protein (CRP)
  • Echocardiogram
  • Electrocardiogram
  • ESR
  • Serum albumin
  • Serum transaminase
  • Urinalysis - may show pus in the urine or protein in the urine
 Procedures such as ECG and echocardiography may reveal signs of myocarditis, pericarditis, arthritis, aseptic meningitis, and inflammation of the coronary arteries.
Intravenous gamma globulin is the standard treatment. It is given in high doses. The child's condition usually greatly improves within 24 hours of treatment with IV gamma globulin.
High-dose aspirin is often given along with IV gamma globulin.Even when they're treated with aspirin and IV gamma globulin, up to 25% of children may still develop problems in their coronary arteries. Some research has suggested that adding steroids to the usual treatment routine may improve a child's outcome, but more research is needed.
With early recognition and treatment, full recovery can be expected. However, about 1% of patients die from complications of coronary blood vessel inflammation. Patients who have had Kawasaki disease should have an echocardiogram every 1 - 2 years to screen for heart problems especially for development of aneurysm.
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