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Hypothalamic-pituitary functions in patients with idiopathic pituitary dwarfism.

To investigate hypothalamic-pituitary functions and the primary site of the lesion in idiopathic pituitary dwarfism, various pituitary function tests, especially the pituitary hormone responses to the hypophysiotropic hormones were studied in 23 patients with idiopathic pituitary dwarfism. A few cases showed slight responses of GH to GH stimulation tests. Gonadotropin deficiencies were most frequently noted among pituitary hormones. The basal levels and the responses of plasma LH and FSH to LH-RH test were diminished markedly in all of the cases except in 5 cases with isolated GH deficiency. Responses of LH and FSH to LH-RH improved markedly after a long term administration of LH-RH for a period of one month in 2 patients with gonadotropin deficiency. As to TSH axis, half of the cases accompanied hypothyroidism. However, the responses of TSH to TRH were normal in all of the cases regardless of the thyroid function. The basal levels and the responses of plasma cortisol and 11-deoxycortisol to the rapid metopirone test were also impaired in about half of the cases. Basal levels of plasma prolactin were normal in all of the cases and the responses of prolactin to TRH were normal in cases with normal thyroid function, but slightly delayed in cases with hypothyroidism. It is concluded from the above observations that the incidences of various pituitary hormone deficiencies were quite high in this disorder and hypophysiotropic hormone deficiencies may cause pituitary hormone deficiencies. Therefore, it is suggested that the primary site of the lesion in this disorder might be at the hypothalamus.

Adolescent

Serum levels of somatomedin A and growth during long-term treatment of patients with pituitary dwarfism with human growth hormone.

Eighteen patients with pituitary dwarfism were treated for 1 7/12 to 6 years with human growth hormone (hGH) at a dose of 0.19--0.62 unit (U) per kg of body weight per week. The mean increment in height was 2.0 +/- 0.4 and 8.8 +/- 0.5 cm/year before and the first year after treatment of hGH, respectively. A significant positive correlation was observed between serum levels of somatomedin A and growth rate, espcially in children with bone age below 10 and a duration of treatment of less than one year (r = 0.66, P less than 0.005). Long-term treatment with hGH was accompanied by a decreasing response. However, the serum levels of somatomedin A did not decrease significantly. Therefore, decreased growth increment in these situations was not due to decreased serum levels of somatomedin A.

Adolescent

The electroencephalogram in a case of pituitary dwarfism before and after eight years of treatment with human growth hormone.

The EEG of an 11-year-old hypopituitary dwarf was investigated to determine whether the disorder was accompanied by a disturbance in cortical maturation. By means of a previously derived equation relating age and the first four central moments of the EEG interval histogram in normal children, the dwarf's developmental age was estimated from his EEG to be only about 6 1/2 years. After 8 years of treatment with human growth hormone, age estimated from the EEG was 8 2/3 years - an increase of barely more than 2 years.

Body Height

[Etiology and associations of pituitary dwarfism. Study of a series of 135 cases].

Among 135 consecutive cases of hypopituitary dwarfism, 42 related to detectable intracranial tumour or defect. In 13 cases the tumour had been previously operated and/or irradiated. In 29 others the defect was suggested by neurological abnormalities or headache, skull radiographs, ocular examination or by associated post-hypophyseal deficiency and was demonstrated by pneumoencephalography. When none of these associated abnormalities was found, pneumoencephalography failed to demonstrate any intracranial lesion. Among the 93 so-called idiopathic cases there was a large majority of males (60/93) with a history of birth difficulties (34/60) and especially of breech delivery (23/60). TSH, FSH/LH and ACTH deficiencies were associated to GH deficiency in 81% of patients with detectable intracranial lesions, 57% of male and 39% of female idiopathic cases. The number of patients with idiopathic isolated GH deficiency was similar in boys and girls, suggesting in them the hypothesis of a recessive autosomic genetic defect in spite of the scarcity of familial cases. Peculiar clinical associations may contribute to the diagnosis.

Adolescent