Serum protein turnover in the congenital nephrotic syndrome. Experimental studies with 3H-tyrosine and 131-I-proteins.
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Albumins↗
Biomedical subjects
J Perheentupa
Publications and source records attributed to J Perheentupa.
Protein intolerance with deficient transport of basic aminoacids. Another inborn error of metabolism.
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Amino Acid Metabolism, Inborn Errors↗
West syndrome: individualized ACTH therapy.
Individualized ACTH treatment of the West syndrome (WS) was assessed in a prospective multicenter study, in which each patient's dosage was increased stepwise according to response. Our series included six patients with cryptogenic and 24 with symptomatic infantile spasms. During the treatment period the total ACTH dose ranged from 58 to 373 i.u./kg. In the cryptogenic group one patient responded to pre-ACTH pyridoxine and four to the lowest dosage of ACTH (3 i.u./kg daily) with cessation of spasms and good outcome; one patient needed the highest dosage (12 i.u./kg daily) for cessation of seizures and became developmentally retarded. In the symptomatic group, 21 of the 24 patients needed 6-12 i.u./kg daily; 12 became seizure-free or having infrequent non-IS fits. Complications such as arterial hypertension, cerebral ventricle dilatation, cardiac hypertrophy, and prolonged adrenocortical hyporesponsiveness were related to the dose. The individualization provides all the benefits of ACTH treatment with minimal side effects and cost.
Adrenocorticotropic Hormone↗
Effects of feeding regimen on blood glucose levels and plasma concentrations of pancreatic hormones and gut regulatory peptides at 9 months of age: comparison between infants fed with milk formula and infants exclusively breast-fed from birth.
Little is known about the development of gut endocrine responses to food intake in infants after the first postnatal month. To examine this question and to ascertain whether the mode of feeding from birth affects postprandial endocrine changes, blood glucose levels and the plasma concentrations of 11 regulatory peptides were measured at 9 months of age before and after a breast feeding in 13 exclusively breast-fed infants and before and after a formula feeding in 7 infants weaned during the first 3 months of life. In the prefeeding concentrations of these substances, no significant differences were found between the two groups, with the possible exception of the plasma concentration of pancreatic polypeptide (p = 0.06). Postprandially, the responses were significantly smaller in the breast-fed infants, whose plasma concentrations of insulin, gastric inhibitory polypeptide, pancreatic polypeptide, and cholecystokinin were lower than in the formula-fed infants. In addition, the overall level of the insulin-glucagon ratio was lower (p = 0.03) in the breast-fed infants. A difference in the opposite direction was observed for plasma gastrin levels. No significant differences appeared between the groups for blood glucose, or plasma glucagon, vasoactive intestinal polypeptide, motilin, enteroglucagon, secretin, or neurotensin concentrations after feeding. It is concluded that at 9 months of age, the gut regulatory responses to milk feeding are of lower magnitude than during the neonatal period, but even at this age the response patterns still depend on the mode of feeding.
Animals↗
Oestrogen treatment of tall girls: effect decreases with age.
Fifty-nine tall girls were treated with oestrogen to reduce final height, starting at the ages of 9.1 to 16.2 years. We assessed the result of this treatment by comparison with matched controls. The epiphyseal bone age at the start of therapy, the final height, the Bayley-Pinneau (BP) and Roche-Wainer-Thissen (RWT) predictions of final height, and the errors in both predictions were evaluated. The matched pairs were divided into three groups according to bone age at the start of treatment; I less than 10.5 (n = 16), II 10.5-12.0 (n = 22) and III greater than 12.0 years (n = 21). The mean (SD) intrapair reduction of height for these groups was 9.7 (4.0) cm, 4.3 (4.3) cm and 3.5 (3.2) cm, respectively, according to BP predictions and 6.3 (4.3) cm, 3.4 (3.0) cm and 1.2 (3.3) cm according to RWT predictions. No method of predicting height is accurate for tall girls and simultaneous predictions may differ greatly. Close agreement between the BP and RWT predictions does not indicate greater accuracy. The earlier therapy is started, the greater is the effect. Young girls need psychosocial support with therapy.
Adolescent↗
Folate nutrition is optimal in exclusively breast-fed infants but inadequate in some of their mothers and in formula-fed infants.
Plasma concentrations of folate were studied in a group of exclusively breast-fed infants and their mothers (their numbers gradually decreased from 200 at birth to 7 at 12 months) and in infants completely weaned to a cow's milk formula (containing 35 micrograms of folate/L) and solid foods. The exclusively breast-fed infants were in no danger of folate deficiency; their plasma levels were elevated after the age of 2 months and, on average, were 2.0-3.3-fold higher than maternal levels throughout the study. None of these infants had an inadequate plasma concentration, whereas up to 5% of the mothers had values less than or equal to 3 micrograms/L, despite supplementation during lactation with 0.1 mg folate/day. In the formula-fed infants, 69-94% of the plasma folate concentrations lay below the lowest concentration for the breast-fed infants. Although no infant had signs of anemia or macrocytosis in red cell indices, the infants weaned earliest had the lowest hemoglobin concentrations (p = 0.09) and the highest mean corpuscular volume (MCV) values (p = 0.06) at 9 months of age. Thus, an infant fed a formula containing the recommended amount of folate runs a risk of folate deficiency.
Age Factors↗