Osamu Nose

Effect of the Energy Source on Changes in Energy Expenditure, Respiratory Quotient, and Nitrogen Balance during Total Parenteral Nutrition in Children

Abstract: The effects of three isocaloric intravenous nutritional regimens were studied in seven infants and children, ages 2 months to 9 yr, with congenital gastrointestinal anomalies (four patients) or with prior history of malignant disease admitted in remission for bone marrow transplantation (three patients). Energy metabolism, as measured by the basal metabolic rate (BMR), and substrate utilization, as measured by the respiratory quotient (RQ), were studied to determine the effect of different levels of carbohydrate and fat on nitrogen retention in each patient. Solution A provided 8% of energy as amino acids, 87% as carbohydrate, and 5% as fat. Solution B provided 8% of energy as amino acids, 60% as carbohydrate, and 32% as fat. Solution C provided 8% of energy as amino acids, 34% as carbohydrate, and 58% as fat. Administration of solution A (high carbohydrate, low fat) was associated with moderately increased mean (±SD) BMR and RQ and with low nitrogen retention (19.1 ± 12.7%, 1.06 ± 0.14, and 98 ± 28 mg N/kg/day). Both the BMR and the RQ decreased when less carbohydrate and more lipid was given: BMR 4.3 ± 11.6% (p < 0.005), RQ 0.92 ± 0.09 (p < 0.001) for solution B; BMR 3.94 ± 10.6% (p < 0.005), RQ 0.86 ± 0.09 (p < 0.001) for solution C. Among the solutions tested, optimal nitrogen retention [163 ± 60 mg N/kg/day (p < 0.01)] was noted with solution B. Our data support the conclusion that a physiologic balance of fat and carbohydrate results in optimal nitrogen retention.

Does CSF copper level in Wilson disease reflect copper accumulation in the brain

The levels of copper and ceruloplasmin in the cerebrospinal fluid (CSF) of patients with Wilson disease were investigated. Ceruloplasmin concentrations in the CSF of all patients were almost the same but were lower than those of the controls. CSF copper concentrations in patients without neurologic signs were within the normal range, 22 +/- 6 ng/ml. In contrast, CSF copper concentrations in patients with neurologic signs (69-98 ng/ml) were significantly higher than the normal levels before and at the beginning of the treatment with D-penicillamine; it gradually decreased in response to treatment. These results suggest that the appearance of neurologic manifestations in Wilson disease is not related to the CSF ceruloplasmin concentration. The CSF copper concentration in this disease appears to reflect copper accumulation in the brain and may be useful as a marker for monitoring therapy.

Multiple associated endocrine abnormalities in a patient with pseudohypoparathyroidism type 1a

A girl with type 1a pseudohypoparathyroidism (PHP) presented several hormonal abnormalities. Although she had eluded neonatal thyroid screening, she was diagnosed as having hypothyroidism at the age of 5 months. Thereafter, a diagnosis of PHP was made on the basis of skeletal features of Albright osteodystrophy and lack of both cyclic adenosine monophosphate (c-AMP) and phosphaturic responses after parathyroid hormone (PTH) infusion. The basal levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) were higher than normal and showed exaggerated responses to luteinizing hormone-releasing hormone (LH-RH). There was no growth hormone (GH) response to arginine infusion, and the prolactin (PRL) response after thyrotropin-releasing hormone (TRH) infusion was also impaired. The stimulating guanine nucleotide-binding protein (Ns) activity of the erythrocytes was reduced to 66.9%. The skeletal age was not delayed at the age of 5 months in spite of the hypothyroid state, and it advanced following thyroxine and vitamin D treatments.

Effects of alternate and simultaneous administrations of calcium and phosphorus on calcium metabolism in children receiving total parenteral nutrition.

The effects of alternate and simultaneous administrations of calcium (Ca) and phosphorus (P) on Ca metabolism in children receiving total parenteral nutrition (TPN) were examined. Eight children, aged 2 to 36 months, were studied. The following three solutions were administered: solution 1 contains Ca (533 mg/liter); solution 2 contains P (413 mg/liter); and solution 3 contains Ca (267 mg/liter) and P (207 mg/liter). Solutions 1 and 2 were administered alternately for 24-hr periods. (Results) I. During administration of solution 1, significant hypophosphatemia (4.39 +/- 0.26 mg/dl) and hypercalcemia (9.96 +/- 0.15 mg/dl) were observed and, conversely, during administration of solution 2, significant hypocalcemia (8.36 +/- 0.18 mg/dl) and hyperphosphatemia (6.16 +/- 0.27 mg/dl) were observed. During administration of solution 3, the serum levels of both minerals were maintained within the normal ranges (Ca 9.46 +/- 0.12 mg/dl, P 5.65 +/- 0.21 mg/dl). II. The urinary excretion of cyclic AMP was significantly lower during administration of solution 1 (6.67 +/- 0.45 nmol/mg creatinine (Cr] as compared with solution 3 (7.50 +/- 0.61 nmol/mg of Cr). On the other hand, the excretion was significantly higher during administration of solution 2 (11.55 +/- 1.58 nmol/mg of Cr) as compared with solution 3, indicating the existence of secondary hyperparathyroidism. III. The Ca and P retention rates were significantly higher with solution 3 (Ca 79.0 +/- 5.5%, P 73.2 +/- 7.2% of the intake) than with solutions 1 and 2 alternately (Ca 62.7 +/- 4.5%, P 49.2 +/- 9.3%). (Conclusions) Simultaneous administrations of Ca and P are preferable to their alternate administrations for Ca metabolism in children receiving TPN.

Transient neonatal hypothyroidism probably related to immaturity of thyroidal iodine organification

roid hormone replacement then may cause thyrotroph regression and expose the pituitary gland and sella to CSF pressure or arachnoid pulsations, which may produce an empty sella. 13 In general, only a minority of patients with empty sella develop pituitary hormone deficiencies, 14 so additional damage to the pituitary gland must occur in some individuals. One might wonder whether slower thyroxine treatment (e.g., less than full replacement dosage) might have altered the development of an empty sella and hypopituitarism. This has been suggested by McVie, ~3 although we know of no studies to support this recommendation. Our patient was initially given a relatively 10w dose of thyroxine, 1.67 /~g/kg body weight, and subsequently required increased dosage. Evidence is accumulating that pituitary enlargement and subtle visual field defects and pituitary alterations occur more commonly with primary hypothyroidism than previously appreciated. Our case report outlines the full spectrum of these changes during childhood, when the development of hGH deficiency has more impact. The initial CT scan was obtained somewhat serendipitously; with the diagnosis of primary hypothyroidism, we would recommend CT scan of the brain only if the patient has signs of increased intracranial pressure or visual field deficits. With pituitary enlargement or mild visual field defects, we believe a conservative approach with thyroxine therapy and close observation is warranted. If the expected clinical response is not seen (in particular, failure to return to normal or catch-up growth rate), the clinician must consider the development of hypopituitarism, which may be associated with an empty sella. We thank Douglas Livermore, M.D., for referring the

Metabolic changes following gastroplasty in Prader-Willi syndrome--a case report.

A vertical banded gastroplasty was performed in an adult female patient with Prader-Willi syndrome in an attempt to prevent the metabolic deterioration caused by polyphagia. After her operation, the patient felt satiated with the scheduled amount of food and one month later, her fasting blood sugar concentration (FBS) decreased from 521 to 125 mg/dl, and her urinary sugar excretion (US) from 257 to 9g/day. Both glucose tolerance and insulin secretion were also improved. However, these parameters subsequently became worse after dietary control was lost since the surgical procedure alone was unable to continue to suppress the insatiable desire to eat food. Both her glucose tolerance and insulin secretion by the 31st postoperative month were better than before the surgery, but worse than at one month after the surgery. At the end of the 34th postoperative month, even under the temporary administration of 0.625 mg/day of glibenclamide, her FBS was 158 mg/dl and US, 38.1 g/day. Her body weight had also increased to over her preoperative value. Based on these results, we conclude that the effect of gastroplasty to prevent metabolic deterioration in our patient with Prader-Willi syndrome gradually diminishes.

Seasonality of birth in sporadic cretinism

The seasonal distribution of birth dates of 31 patients with sporadic cretinism due to thyroid dysgenesis was analyzed in Osaka area for 14 years. The incidence was statistically high in the summer months. A hypothesis that some environmental factors such as viral infection may cause the disease is proposed.

Vitamin D Metabolism in Biliary Atresia: Intestinal Absorptions of 25-Hydroxyvitamin D3 and 1,25-Dihydroxyvitamin D3

In children with biliary atresia, defective intestinal absorption of vitamin D and impaired hepatic uptake and 25-hydroxylation of vitamin D lead to a deficiency of vitamin D and rickets. We recently observed severe rickets in a 3-year-old boy with corrected biliary atresia resulting in jaundice, despite oral treatment with 1 alpha-hydroxyvitamin D3 (1 alpha-OHD3) or 1,25-dihydroxyvitamin D3 [1,25-(OH)2D3]. He had low 25-hydroxyvitamin D (25-OHD) and high 1,25-(OH)2D serum levels. Intramuscular vitamin D2 administration produced radiological and biochemical evidence of recovery. Oral 1,25-(OH)2D3 (0.1 microgram/kg) and 25-OHD3 (10 micrograms/kg) tolerance tests were done to assess the ability to absorb vitamin D and the effectiveness of using these drugs orally. Eleven children with corrected biliary atresia, aged 9 months to 7 years, were studied. In oral 1,25-(OH)2D3 tolerance tests, the increments above the baseline serum levels of 1,25-(OH)2D were 140.7 +/- 27.4 pg/ml in nonjaundiced patients (n = 5). In jaundiced patients (n = 3), 1,25-(OH)2D3 absorption in two patients with high basal 1,25-(OH)2D values was lower than that of nonjaundiced patients; however, the absorption in the third patient with a low basal value was similar to that of nonjaundiced patients. In oral 25-OHD3 tolerance tests, the mean increase of serum 25-OHD was 48.9 +/- 30.6 ng/ml in nonjaundiced patients (n = 5) and 23.7 +/- 9.5 ng/ml in jaundiced patients (n = 4), the peak serum 25-OHD levels being reached 6-12 h after 25-OHD3 loading.(ABSTRACT TRUNCATED AT 250 WORDS)

Semiautomated Enzyme Immunoassay of Thyrotropin as a Mass Screening Test for Neonatal Hypothyroidism

ABSTRACT: A sensitive, simple, and rapid semiautomated sandwich enzyme immunoassay (EIA) was developed for measuring thyrotropin in dried blood samples on filter paper for use in screening for neonatal hypothyroidism. Good correlation was found between values for thyrotropin determined by this method and those determined by radioimmunoassay (RIA) (r = 0.94). In pilot tests on 17,160 newborn infants in the general population, five cases of primary hypothyroidism were detected by both EIA and RIA. The recall rate was slightly higher in EIA than in RIA.

Total parenteral nutrition with a new amino acid solution for infants.

The currently available, commercially prepared amino acid mixtures for intravenous usage do not result in completely normal plasma amino acid patterns. Taking into consideration the known imbalances that occur with the commercial preparations, we designed a new amino acid solution (AF). This solution was prepared to contain a low concentration of those amino acids usually found in increased concentrations in plasma during the course of total parenteral nutrition (TPN) with a commercially prepared product (CF) and a high concentration of branched-chain amino acids and arginine. Plasma amino acid concentrations were determined for nine infants receiving CF (Group A) and eight infants receiving AF (Group B) as the protein source while on TPN for 5 days. These data were compared to 2-h postprandial values obtained from breast-fed, growing infants serving as controls. In comparison with controls, the plasma concentrations of threonine, glycine, methionine, phenylalanine, tryptophan, and lysine were higher in infants in group A, while those of taurine, alanine, valine, cystine, isoleucine, and proline were lower. In group B, the levels of valine and leucine were slightly higher, while those of alanine, cystine, tyrosine, histidine, and proline were lower. Mean nitrogen balances in groups A and B were + 112 and + 170 mg/kg/day, respectively. Therefore, our new amino acid solution may be better for infants receiving TPN.