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Indian Pediatr 2010;47:1070 |
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K Rajeshwari
Email:
[email protected] |
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Tetrahydrobiopterin for Phenylketonuria
(Lancet 2010; 376:1417-1427) |
The initiation of a phenylalanine-restricted diet soon after birth
prevents most of the neuropsychological complications in phenylketonuria.
However, the diet is difficult to maintain and compliance is often poor,
especially in adolescents, young adults, and pregnant women.
Tetrahydrobiopterin stimulates phenylalanine hydroxylase activity in about
20% of patients and in those patients serves as a useful adjunct to the
phenylalanine-restricted diet because it increases phenylalanine tolerance
and allows some dietary freedom.
Comments Other possible future treatments
include enzyme substitution with phenylalanine ammonia lyase,
which degrades phenylalanine, and gene therapy to restore
phenylalanine hydroxylase activity.
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Management of enuresis in children (BMJ
2010; 341:c5399) |
Bedwetting is a widespread and distressing condition that can have a deep
impact on a child or young person’s behavior, emotional wellbeing, and
social life. If the child was dry for more than six months before the
present episode started, explore possible triggers such as emotional upset
or recent illness. The management of bedwetting is not different for
secondary bedwetting, but a trigger might need treatment in its own right.
Urine analysis is not indicated unless bedwetting started in the past few
days or weeks, or in the presence of daytime urinary problems or suspected
diabetes. Further assessment is to be done for severe daytime urinary
problems, previous history of urinary infections, known or suspected
congenital physical abnormalities (posterior urethal valves or
myelome-ningocele) or neurological problems. Waking a child during the
night to take him/her to the toilet will help to keep them dry in the long
term. Offer an alarm as initial treatment for children and young people
whose bedwetting has not responded to advice on fluids, toileting, or an
appropriate reward system. If alarm treatment is not successful, offer
desmopressin (a synthetic analogue of antidiuretic hormone) as well as use
of the alarm. Alternatively, if the parents, care givers, child, or young
person no longer wants to use an alarm, offer desmopressin alone.
Comments If all the above treatments fails
then refer for a detailed assessment. After such assessment
consider offering an anticholinergic drug such as tolterodine or
oxybutynin to take with desmopressin. The effectiveness of
acupuncture and hypnotherapy for enuresis when these therapies
are used independently or in conjunction with conventional
treatments is still under exploration.
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Eating patterns and obesity (J
Pediatr 2010; 157:815-820) |
Physical activity and 24-hour dietary recall information and
anthropometric indices were collected from 1138 children (53% girls; age,
11.2 ± 0.7 years). Five lifestyle behavioral patterns were identified. The
"dinner, cooked meals and vegetables pattern," a multidimensional
lifestyle pattern including the consumption of vegetables, cooked meals,
and eating dinner, was negatively associated with all obesity indices,
even after adjustment for potential confounders.
Comments These findings are supportive of
the conditional effects of distinct lifestyle-related behaviors on
obesity. Although these results are hypothesis-generating and need
replication, they suggest potential preventive and interventional
approaches to combat childhood obesity.
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