The insulin-like growth factors (IGFs), insulin-like growth factors binding proteins (IGFBPs), and IGFBP proteases regulate somatic growth and cellular proliferation both in vivo and in vitro. IGFs are potent mitogens whose actions are determined by the availability of free IGFs to interact with IGF receptors. IGFBPs comprise a family of proteins that bind IGFs with high affinity and high specificity and thereby regulate IGF-dependent actions. IGFBPs have recently emerged as IGF-independent regulators of cell growth. This review covers clinical aspects of the IGF axis with particular attention to its diagnostic and therapeutic roles in pediatrics. The ubiquity and complexity of the IGF axis promise exciting discoveries and applications for the future.

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We studied the change of size and shape of the ovaries after administration of nasal GnRH analogue by pelvic sonography and the serum levels of LH, FSH, and estradiol in two girls with central precocious puberty. The ovaries enlarged gradually after the administration of GnRH analogue. And after 2 weeks, one follicle of the right ovary became larger rapidly in both cases; the ovarian volumes increased 21.8- and 6.6-fold respectively, compared to volumes before medication, while serum estradiol concentrations peaked at 2 and 3 weeks after GnRH analogue respectively. The authors thought that the massive enlargement of the ovarian follicles was caused by the stimulation of LH elevated by GnRH analogue, and possibly direct action of GnRH analogue on the gonads. The authors believe that the agonistic potency of nasal administration of GnRH analogue remains for at least several weeks. Attention must be paid to changes of the ovaries during administration of nasal GnRH analogue, at least for the initial period of treatment for central precocious puberty.

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A Japanese female who had remitted Langerhans cell histiocytosis with major involvement of the suprasellar region, exhibited sustained linear growth despite apparently impaired growth hormone secretion. This phenomenon, usually referred to as “growth without GH”, has been described mainly in patients with craniopharyngioma who have undergone surgery. This is the first detailed description of “growth without GH” accompanied by Langerhans cell histiocytosis, which implies that the pathological nature of the initial brain involvement is not critically important for the development of “growth without GH”.

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To study the initial stimulative and subsequent suppressive effects of Luteinizing hormone releasing hormone analog (LHRH-a) therapy on the hypothalamic-pituitary-ovarian axis, serial urinary excretion of gonadotropin and ovarian steroids were investigated in 23 female patients with central precocious puberty. Among them, seven were treated with intranasal administration of buserelin (G1), seven with daily subcutaneous injection of buserelin (G2) and nine with subcutaneous injection of super-long-acting LHRH-a every 28 days (G3). After the initial therapy in G1, the levels of urinary gonadotropin excretion increased greatly with a high level persisting for a longer period. Responding to these increases, the levels of urinary total estrogen excretion increased to an excessively high maximum level. After the initial injection in G3, the levels of urinary gonadotropin and total estrogen excretion were augmented at the same time for a shorter period with a lesser excretion of urinary total estrogen. Ovarian cysts developed after urinary total estrogen attained a maximum and tended to grow depending on the duration and degree of high urinary total estrogen excretion.The size of ovarian cysts was greater in G1 than in G3. During therapy in all groups, the mean urinary gonadotropins were significantly suppressed compared with pretreatment. The mean urinary total estrogen excretory level during treatment in G1 was not significantly suppressed compared with before treatment, but on the other hand, with significant suppression in G3. During therapy, urinary FSH excretion increased within 24 hours of every injection of super-long-acting LHRH-a with a slight rise in LH only in all patients in G3 studied. Studies in many more patients are required because the number of subjects was limited in this study.

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We report sisters with congenital hypothyroidism due to iodide transport defect (ITD) not detected in neonatal mass screening for cretinism. The symptoms of hypothyroidism appeared at the age of about 3 years (elder sister) and 2 years (younger sister), respectively. Since a thyroidal function test revealed severe hypothyroidism, they have been treated with l-T₄ and have remained euthyroid. Thyroidal ¹²³I uptake and the saliva/serum radioactive iodide ratio confirmed ITD. Moreover, homozygous sodium/iodide (Na⁺/I^-) symporter (NIS) gene mutation of G543E was seen as a result of genetic analysis. The reason why their hypothyroidism was not found in neonatal mass screening for cretinism was because Japanese ingest large amounts of iodine, and therefore the sisters could obtain enough iodine throuth breast feeding. As a result, we suspect that they could produce thyroid hormones to some extent without active iodine transport to their thyroid. It might be difficult for the neonatal mass screening for cretinism in Japan to discover hypothyroidism due to ITD.

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We followed up 5 patients with transient neonatal thyroid dysfunction born to mothers with Graves’ disease from 5 to 10 years and assessed their outcome in physical growth and intellectual development as well as thyroid function. The patients consisted of 3 girls, including a pair of siblings, and 2 boys. Four of them had transient neonatal hyperthyroidism and the other boy had transient neonatal hypothyroidism with normal thyrotropin. The mother of the latter patient developed hyperthyroidism during the second trimester of her pregnancy. The mothers of the former patients were found hyperthyroid before bearing the children. We analyzed physical findings, growth and intellectual development as well as thyroid function including anti-thyroid antibodies and anti-TSH receptor antibody. Mild thyromegaly developed in 2 patients with transient neonatal hyperthyroidism. Short stature was seen in a patient with neonatal hyperthyroidism in whom an excess band on the X chromosome was found. One patient had only a borderline IQ but the other three with neonatal hyperthyroidism had above average ability, whereas moderate delay in psychomotor development was seen in the patient with neonatal hypothyroidism. In the sibling patients, anti-thyroid antibodies have been positive since 6 years of age. Further follow-up is required to know the final outcome of these patients.

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