[Dietary treatment of maple syrup urine diseases]. / Le traitement nutritionnel des leucinoses.

Principles of dietary management and needs in maple syrup urine disease are well defined. However, long-term results show that there is a need for an improvement of the maintenance of a low plasma leucine level and in monitoring episodes of metabolic decompensation in order to ameliorate the survival and psycho-intellectual outcome.

History and current status of infant formulas.

Formulas that approximate the composition of human milk are widely used as the sole source of nutrients for infants during the first months of life. Such formulas contain milk, soy, or meal proteins, or protein hydrolysates together with those forms of fat carbohydrate, vitamins, and minerals shown to be bioavailable to the infant. Legislation concerning nutrient composition and quality assurance of products labeled as infant formulas has been enacted by Congress. Governmental and industrial standards also help to assure microbiological safety and stability. Problems of nutrient composition and adverse reactions have occurred during the evolution and development of infant formulas. Problems concerning nutrient deficiency are now almost unknown, but adverse reactions of individuals to specific formula ingredients continue. Accurate information on composition and related nutritional properties of infant formulas is essential for their effective use and for the avoidance of undesirable complications.

Medium chain triglycerides for treatment of spontaneous, neonatal chylothorax. Lipid analysis of the chyle.

Volume and contents of lipid and protein in the pleural fluid from a three weeks old girl with spontaneous chylothorax were studied (a) during parenteral, nonfatty nutrition and later (b) during administration of a formula (Biosorbin) containing medium chain triglycerides (MCT). The pleural fluid production could not be correlated to the treatment employed but suddenly ceased after 20 days management. Triglyceride and total esterified fatty acid concentrations in pleural fluid were high on admission when feeded with human milk, and chylomicrons and other lipoproteins were present in the chyle. During parenteral treatment a pronounced decrease in pleural fluid concentrations of triglyceride and total fatty acids occurred concomitant with a disappearance of the chylomicrons. During the following MCT diet a pronounced increase in triglyceride and total fatty acids concentrations appeared and the chylomicrons reappeared in the chyle. The cholesterol and phospholipid concentrations in the pleural fluid showed only small changes during the different treatments. No significant changes in protein and albumin concentrations of chyle were observed. It is concluded, that administration of the Biosorbin MCT formula, containing 871/2% of the fat as MCT, seems without value ih the treatment of spontaneous, neonatal chylothorax.

[Idiopathic intestinal lymphangiectasis. Evolution with M.C.T. (author's transl)]. / Linfangiectasia intestinal idiopática. Evolución con triglicéridos de cadena media

A case of idiopathic intestinal lymphangiectasis is reported in a three month old child. Clinical course and laboratory findings are given in relation to administration of three diets containing different concentrations and types of fat. Short term improvement was only noticed with diets containing low concentrations of long chain triglycerides supplement with medium chain triglycerides. Clinical manifestations related to fat malabsortion improved greatly but there was no relationship with serum protein level. No effect on low level of gamma-globulins and lymphatic displasia was found as sawn in an intestinal biopsy performed after three months of treatment. Nevertheless, long-term results were poor and only were evident in a diminution of steatorrhea and normalization of stools.

[Different caloric intake in 75 "low birth weights": effect on weight gain, blood sugar, serum protein, and serum bilirubin (author's transl)]. / Unterschiedliche Calorienzulfuhr bei 75 "low birth weights": Einfluss auf Gewichtszunahme, Serumeiweiss, Blutzucker und Serumbilirubin

A prospective study on oral feeding was started in 75 low birth weights (below 2500 g) with adapted milk. Early feeding was given in two groups with different feeding volume and caloric intake. The infants were grouped alternately. Both groups were comparable concerning birth weight, gestational age, and intrauterine growth. Due to partially different variances, covariate correction was applied to analysis of the data. The high caloric group had excellent weight gain, the maximum weight loss was less, and birth weight was regained earlier than in the control group. Blood sugar and serum protein were similar in both groups. Mean serum bilirubin levels were lower in the group with high caloric intake, but differences failed to be significant. Early high caloric feeding was well tolerated and is preferable to parenteral nutrition in low birth weights.

Nutritional considerations for the newborn requiring intensive care.

The importance of providing nutrition for the critically ill or low-birth-weight baby is frequently over-looked in the zeal to preserve life. Unfortunately, because of this oversight, a significant factor influencing the quality of that salvaged life may be neglected. Common nutritional problems requiring a team effort have been described to emphasize the need for support by the dietitian. There is increasing evidence that what is appropriate for the term baby may not be ideal for the premature infant. Some of the recognized discrepancies are discussed, and an approach to management outlined. Through prospective evaluation, we hope to gain insight into many of the unanswered and perhaps unrecognized questions regarding nutrition for the intensive care newborn.

Use of an oral elemental diet in infants with severe intractable diarrhea.

Twenty-seven infants from 1 day to 9 months of age with severe intractable diarrhea were fed an oral elemental diet (Vivonex) consisting of crystalline amino acids, glucose, electrolytes, and vitamins by continuous nasogastric drip. Complete control of diarrhea was achieved in 24 patients (89 percent) who had an average weight gain of 28 gm/day. Nitrogen balance and plasma amino acids were measured in five patients while they received 2.25 gm of amino acid/kd/day for two weeks and 4.58 gm of amino acid/kg/day for two weeks; the nitrogen balance and weight gain in three patients was proportional to the amino acid intake. When compared to normal levels, plasma amino acids were not appreciably increased with the lower amino acid intake. With the higher amino acid intake, there were significant increases in plasma values for 11 amino acids.