4th Pediatric Infectious Diseases Conference
 
 
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Pedi Poll
Today's Poll
Should teicoplannin, colistin be used in case of neonatal sepsis where culture does not reveal any organism_?
No, it should be used only after drug sensitivity report
Yes, under guidance of an infectious disease expert
TUBEROUS SCLEROSIS
TUBEROUS SCLEROSIS
Tuberous Sclerosis Management Prognosis and Cure
Tuberous Sclerosis Management Prognosis and Cure
Dr. Ira Shah
M.D, DCH(Gold Medalist), FCPS, DNB

Q: How is a child with Tuberous
sclerosis managed?



A. For a child below 5 years, the following should be especially taken care of :-

  • Epilepsy :-
    It may not always be possible to stop fits altogether, however, best possible control with minimum side effects should be the goal. Infantile spasms can be well controlled with Vigabartin.

  • Developmental delay :-
    The speech and communication is usually delayed rather than motor delay.

  • Sleep problems :-
    Blood pressure and blood test for renal functions should be checked annually.

    For children between 5 - 10 years,
    Standard eye and hearing check - ups should be carried out. These are especially important in a child with a learning disability, so that these problems can be corrected to maximize the learning capacity. Regular dental checkup is also important as certain antiepileptic medication may cause teeth problems.

    For children above 10 years,
    Kidney problems should be monitored as they may turn up later. Facial rash ( adenoma sebaceum) may be seen and can be disguised with special make - up or treated with laser therapy. Signs for hydrocephalus should always be looked for.

Q. What is the prognosis of a child with tuberous sclerosis ?

A. Most people affected by tuberous sclerosis have a normal life span. Over 50% of people with tuberous sclerosis are intellectually normal. Remainder have learning disabilities to a greater or lesser extent.

Q. Is there a cure for tuberous sclerosis?

A. Unfortunately, there is no cure for tuberous sclerosis but treatment is available for various related symptoms.

Q: Why does tuberous sclerosis affect so many body organs?

A. In tuberous sclerosis, there is a growth of normal body tissue in a disorganized way (hamartomas). They interfere with the functioning of the organ in which they are growing. When the person becomes an adult, the growth of the organ and the hamartoma stops. As a result, most patients have a normal life span. However, there may be occasional serious problems (kidney, lung or brain tumours) that need to be dealt with later in life.

Q. What are the chances that my next child will have tuberous sclerosis?


A. In 2/3rd of the cases, tuberous sclerosis is a spontaneous mutation and no one else in the family is affected. There are no definitive tests to diagnose tuberous sclerosis in the unborn. Sometimes echocardiography of the baby's heart during mid - pregnancy may provide evidence of lesions but gives no indication of how severely the baby is affected.

If one of the parents have Tuberous sclerosis , the chances of Tuberous sclerosis in the next pregnancy is 50%.

References:
    National Tuberous Sclerosis Association (Tuberous Sclerosis Alliance)
    The Tuberous Sclerosis Association online

Last created on 14-05-2001
Last updated on 28-04-2007






 
 
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