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Indian Pediatr 2010;47:1070

 

K Rajeshwari

Email: [email protected]  

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.
 

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.
 

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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