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Introduction: Some patients with hyperphenylalaninemia due to phenylalanine hydroxylase deficiency respond with a variable decrease in plasma phenylalanine levels after oral tetrahydrobiopterin (BH4) administration and are then able to tolerate higher dietary phenylalanine intake or even to discontinue a phenylalanine-restricted diet. BH4-sensitive patients are usually identified by means of a BH4 loading test, but consensus on the methodology of this test and the interpretation of its results is lacking. Consequently, a simple tool to identify which patients are likely candidates for this treatment and how they will progress in the long-term is required., Material and Methods: A combined oral BH4 loading test with phenylalanine (100 mg/kg) and BH4 (20 mg/kg) was performed in 20 patients with hyperphenylalaninemia under dietary phenylalanine restriction., Results: Independently of the genotype, the result was positive in all the 9 patients whose maximum phenylalanine level at diagnosis was below 815 nmol/ml. Currently, they are under treatment with tetrahydrobiopterin doses of 7-15 mg/kg/day. All these patients have been able to increase their oral phenylalanine intake. Six are currently following a normal diet and the remaining three are close to reaching this goal. None of the patients with a maximum phenylalanine level at diagnosis higher than 938 nmol/ml responded to the BH4 loading test., Conclusions: The maximum phenylalanine level at diagnosis seems to be a simple and reliable method to predict response to BH4 treatment. A high percentage of BH4-sensitive patients are able to discontinue a phenylalanine-restricted diet after long-term tetrahydrobiopterin treatment.

Several anthropometrical, immunological and biochemical parameters have been utilized for the detection of chronic undernourishment and inflammation. The present work proposes a comparative study between a control group and other of 24 cardiac patients, with the intention of valuating their nutritional and inflammatory state. We have used the index proposed by Ingenbleek (PINI = prognostic inflammatory and nutritional index) in which nutritional scoreboards and biochemical inflammatories correlate. We find some statistically significant differences between cases and controls, both with regard to the value of the PINI, and with regard to each of this variables. In the same way, we do not find any important correlations within each group among the nutritional biochemical, inflammatory and anthropometrical parameters. The clinical impression shows that the classification by groups of risk obtained according to the value of the PINI is very near to reality. We consider that the PINI index is useful as a predictive and evolutive parameter, and it can be used in children with a chronic illness.

We analyze the therapeutical value of an oral glucoelectrolytic solution when treating 132 hospitalized patients ranging from 1 to 36 months of age, with acute diarrhea and/or dehydration. These children were offered ad libitum a solution containing: Na 50 mEq/l, K 30 mEq/l, CO3H 24 mMol/l, glucose 111 mMol/l, and saccharose 55 mMol/l, with an osmolarity of 300 mOsm/1. We observed a simultaneous clinical recovery and normalization of the hydroelectrolytic disturbances (p less than 0.05). Furthermore the differences in the ingested quantities by dehydrated and well hydrated children (p less than 0.05), or as expressed in ml/kg/hour during the first six hours compared with the following hours in the whole group (p less than 0.05), demonstrate a physiological recovers of the patient. We conclude that the use of these solutions should have priority over other treatment even in hospitalized patients.