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The typical child with growth hormone (GH) deficiency is one who may have had low-blood sugar as a newborn or later; one who continues to show failure to grow; or if he is a boy, he may have small genitals. These children may be identified by a test that stimulates GH formation and then measures the resulting GH levels. The usefulness of this test has been questioned, because the child may not be spontaneously secreting a normal level of GH, and might not be identified. In an article in the Journal of Pediatrics, Costin, et al., reported that testing levels of growth hormone spontaneously secreted over a 24-hour period produced a wide variability of levels. They concluded that measurement of 24-hour GH levels to evaluate children for possible GH deficiency is of limited value. Children with malfunctioning of the hormone- producing organs such as the pituitary or hypothalamus can usually be identified by the GH stimulating test. However, children with leukemia who have been treated by radiation therapy to the head do not test accurately using this GH stimulating test. This is a screening test and does identify those children who need to be treated for GH deficiency, but it does not prove that a child does not have GH deficiency. Children without a proven GH deficiency, but with severely short stature and abnormal rate of growth should be evaluated by a pediatric endocrinologist. Long-term treatment is necessary to produce any significant height increase and treatment should be begun as early as possible.

Abstract: In an open-label study, 69 children with organic or idiopathic growth hormone deficiency (GHD) were treated with recombinant human growth hormone (Saizen®) for an average of 64.4 mo, with treatment periods as long as 140.9 mo. Auxologic measurements, including height velocity, height standard deviation score, and bone age, were made on a regular basis. The data suggest that long-term treatment with Saizen in children with GHD results in a positive catch-up growth response and proportionate changes in bone age vs height age during treatment. In addition, long-term Saizen therapy was well tolerated, with the majority of adverse events related to common childhood disorders or existing baseline medical conditions and not to study treatment. There were no significant changes in laboratory safety data or vital signs, and no positive antibody tests for Saizen.

Insulin-dependent juvenile diabetes mellitus (Type I diabetes) is a chronic disorder resulting from the inadequate production or utilization of insulin. The ongoing chronic strains of juvenile diabetes, such as the complex daily medical management requirements, acute exacerbations of the disease, and physical complications interact synergistically to produce a negative impact on psychological adjustment. In the present study, family and peer social support and self-esteem were investigates as mediating variables in the chronic strain-psychological adjustment relationship. Hierarchical multiple regression analyses were utilized to test the model. For children, the model accounted for 46 percent of the variance in internalizing behavior problems and 35 percent of the variance in externalizing behavior problems. For adolescents, the model accounted for 54 percent of the variance in internalizing behavior problems and 25 percent of the variance in externalizing behavior problems. The results are discussed in terms of their implications for primary and secondary prevention efforts for chronically ill children and adolescents who are at increased risk for psychological adjustment problems.

Before 1985 the use of growth hormone (GH) was governed by a philosophy of scarcity and conservation of resources. Between 1956 and 1959 human pituitary GH was shown to be effective. The competition for gland collection and extraction that followed benefited only certain patients with motivated parents and only a few investigators. To maximize gland collection, the distribution of GH for clinical investigation, and the number of patients who could be treated, the National Institutes of Health and the College of American Pathologists formed the National Pituitary Agency (NPA). In Canada a similar program was developed by the Canadian Medical Research Council. For more than 20 years the NPA supervised most of the GH treatment in the United States. Commercial pituitary GH entered the U.S. market in 1976, and competition soon appeared. Patients treated through the NPA were subjects in clinical studies for part of the first year of treatment, after which the limited availability of GH dictated treatment for only part of the year and caps on final heights. By 1984 treatment was year round and the height caps largely unenforced. In the last year of its distribution NPA GH was used in 2450 patients in the United States and commercial pituitary GH was used in 600 to 800; slightly more than 300 patients were being treated in Canada. And then, in 1985, came Creutzfeldt-Jakob disease. While the not-so-good old days are gone and need not be lamented, there remains virtue in a conservative therapeutic philosophy. If anything can be learned from the use of pituitary GH in children, it is a healthy respect for the law of unintended consequences. (J Pediatr 1997;131:S1-4)

The assessment of growth patterns in children has become an important parameter to consider in the detection of endocrine abnormalities. Two such disorders, short and tall stature, are discussed, and emphasis is placed on their etiology, clinical manifestations, diagnosis, and treatment.

Consecutive daily serum samples were obtained over 25–30 days from 2 pre-pubertal girls (Stage I), 2 girls with breast development alone (Stage II); one girl with both breast development and pubic hair (Stage III), and 2 girls who were six months and three years post-menarche, respectively (Stage IV). All serum samples were assayed for LH, FSH, progesterone and 17-hydroxyprogesterone. Sera from 6 girls were assayed for testosterone, and sera from 3 were assayed for oestradiol.The patterns of gonadotrophins and sex steroid concentrations in both Stage I and Stage II girls were characterized by day to day stability. LH and FSH concentration was increased in Stage II subjects, while steroid concentration were similar to those in Stage I. The Stage III subject showed marked, apparently random, fluctuations in the concentrations of LH, FSH, oestradiol and 17-hydroxyprogesterone. In Stage IV, as early as six months post-menarche, there was a definite peak in the serum concentration of gonadotrophins, 17-hydroxyprogesterone and oestradiol. However, luteal phase length and maximal progesterone levels of these post-menarchial girls were less than those observed for normal adult females.These data are consistent with evolution of the adult menstrual pattern of hormone secretion through a series of developmental stages.

CRANIOPHARYNGIOMA is frequently associated with dwarfism secondary to interference with hypothalamic or pituitary function or both. Persistence of pituitary insufficiency would be expected following removal of the tumor, whether or not hypophysectomy were a part of the surgical procedure. However, the maintenance or return of normal growth following surgery for craniopharyngioma has been suggested by several preliminary reports.1-4We have observed a postoperative growth spurt followed by normal growth in a patient with preoperative growth retardation secondary to this lesion. The serum growth hormone response to hypoglycemia has been investigated preoperatively and postoperatively. REPORT OF A CASE The patient was admitted to the UCLA hospital at age 13 9/12 years (Oct 19, 1964) because of short stature, polyuria, polydipsia, and impaired vision.He had been noted to be short at age 7, but evaluation was not undertaken at that time. Beginning at age 8, the patient had the onset

THE SEPARATION of patients with diabetes insipidus from individuals with normal ability to form concentrated urine has not always been satisfactory. Three diagnostic procedures have been utilized to make this differentiation. The Carter-Robbins test1 based on the observations of Hickey and Hare2 has been the procedure most frequently employed. The test is preceded by an overnight period of dehydration which in itself is dangerous in the small child with diabetes insipidus. The intravenous infusion is uncomfortable and catheterization of the bladder is frequently necessary to collect accurately timed urine samples. A second diagnostic procedure has been the parenteral administration of nicotine to stimulate antidiuretic hormone release.3 This has proven to be unreliable and uncomfortable for the patient. A water deprivation test employing the measurement of urinary specific gravity4 has been largely abandoned because the 24-hour period of dehydration required was hazardous in patients with diabetes insipidus.

The development of antibodies to human growth hormone (HGH) was studied in three growth hormone deficient patients utilizing specific antisera to Ig G and Ig M immunoglobulins. At 2½ weeks after beginning growth hormone administration binding of HGH131I to Ig M (12.1–16.3 %) and Ig G (8.8–11.2 %) immunoglobulins was demonstrated. At 13 weeks after the onset of therapy binding to Ig M had decreased (7.2-9.4 %) and the binding to Ig G had increased (11.2–15.7 %). The binding of HGH131I to immunoglobulins was inhibited by unlabelled HGH. These findings, which are typical of the sequential development of antibodies of different immunoglobulin classes seen in the early antibody response, strengthen the concept that the binding of HGH131I to plasma proteins observed in response to HGH therapy represents the development of specific antibody.

GLYCOGEN-storage disease (GSD) type 1 is an inborn error of carbohydrate metabolism resulting in diminished activity of the enzyme glucose-6-phosphatase in the liver,1 kidney,2 and small intestine.3 Clinically, the disease is characterized by hypoglycemic seizures, lactic acidosis, hyperuricemia,4 hyperlipidemia,5 hepatomegaly, short stature, and retarded development of secondary sex characteristics.The pattern of growth and sexual development in patients with GSD type 1 is suggestive of a deficiency of one or more of the pituitary trophic hormones. Therefore we have investigated those functions which influence growth and sexual development in patients with this disorder. genital defect may be strengthened. REPORT OF CASES Four patients (patient 1, a 21-year-old white man; patient 2, a 17-year-old oriental girl; pa tient 3, a 14-year-old white girl; and patient 4, a 13-year-old white boy) with GSD, type 1, who met the following biochemical criteria were studied: (1) no increase in the

THE DETECTION of antibodies to human growth hormone (HGH) developing during long-term therapy with HGH has recently been reported by several investigators. Antibodies did not appear to interfere with the response to HGH in two patients reported by Trafford et al1 or in three patients reported by Roth et al.2 The growth-promoting effect appeared to be neutralized in the three patients of Prader et al3 and in the patient described by Parker et al.4 The short-term metabolic effects of HGH were inhibited in two of Prader's patients as measured by nitrogen balance studies. These findings suggest that antigenicity of HGH preparations in man may limit their therapeutic usefulness.We have observed the development of antibodies to HGH in one of three patients receiving long-term therapy with Li HGH. This patient failed to show a growth response to HGH therapy. Immunological and biological studies of these anti-HGH