Effect of growth hormone and oxandrolone singly and together on growth rate in girls with X chromosome abnormalities.

The linear growth effects of oxandrolone, human growth hormone, and Ox + hGH were compared in six girls, age 11 to 15 years, with X chromosome abnormalities (three with X,O karyotype, three with Turner syndrome variants). Over six successive three-month periods, the patient received either no treatment, Ox, hGH, or Ox + hGH. The data demonstrate a significant (P less than 0.001) synergistic effect between Ox and hGH in stimulating linear growth in Turner syndrome and its variants.

Prevalence of growth hormone deficiency in children with cleft lip or palate.

Two hundred children 7 to 14 years of age with isolated cleft defects of the lip, palate, or both were surveyed for stature. Twelve percent were less than the third percentile in height and were designated "short." All of the short children received an endocrine evaluation. Endogenous growth hormone was examined after two days' pretreatment with stilbesteral. Four of the 25 short children with CLP had total, and four had partial, GH deficiency. Three of the GH-deficient patients were also deficient in ACTH or TSH. In contrast, in a series of 75 short (less than third percentile) children 7 to 14 years of age without cleft defect or other apparent congenital abnormality, only two had total and two had partial GH deficiency. The data suggest that children with isolated CLP have short stature about four times more often, and GH-deficiency about 40 times more often, than children without CLP. The increased prevalence of GH-deficiency may stem from the embryologic relation of adenohypophysis and oral ectoderm.

Hyperdibasicaminoaciduria, hyperammonemia, and growth retardation: Treatment with arginine, lysine, and citrulline.

A 9-year-old girl with hereditary dibasicaminoaciduria has been studied for three years. Initially, clinical features were: growth failure; anorexia and aversion to protein, spontaneous daily protein intake averaging only 10 gm; fasting and postprandial venous hyperammonemia; subnormal plasma concentrations of lysine, arginine, ornithine, and citrulline, with generalized hypermonobasicaminoacidemia; abnormally high renal clearances of lysine, arginine, and ornithine; and intestinal malabsorption of lysine and arginine. Intestinal absorption of citrulline, a precursor of arginine and ornithine, was normal. The patient was observed during four sequential 6-month periods as follows: no treatment (Period I); dietary supplement of arginine and lysine (Period II); dietary supplement of citrulline and lysine (Period III); no treatment (Period IV). During Periods II and III growth rate increased 3- to 4-fold, spontaneous protein intake increased 2- to 3-fold, and abnormalities in blood NH3 and the plasma aminogram were partially corrected. In most respects the citrulline plus lysine supplement was more beneficial than that of arginine plus lysine.

Plasma amino acid patterns during supplemental intravenous nutrition of low-birth-weight infants.

In 42 low-birth-weight infants (smaller than 1,200 gm), we have compared the effects of intravenous nutrition supplement versus conventional feedings on growth, morbidity, mortality, and plasma amino acid patterns. Despite similar total caloric intake in INS and control groups, weight gain was greater in the INS infants. The overall mortality rate did not differ in the two groups. Nonsurviving infants receiving INS lived longer (mean equal to 30 days) than nonsurviving CON infants (mean equal to 5 days). Complications were equally frequent in both groups except that hyperglycemia occurred more often in infants receiving INS. The plasma aminogram of the LBW infant is described and compared to those of the full-term infant and adult. Hypoaminoacidemia was present at birth in the LBW infants, concentrations of glutamine, alamine, glycine, histidine, and ornithine being significantly (P smaller than 0.05) below FT values. During INS, elevations of threonine, serine, and methionine above FT values occurred. Glutamine remained subnormal despite INS. Recommendations for an INS solution more suitable for use in LBW infants are presented.