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The impact of marrow transplant preparative regimens on subsequent growth and development. The Seattle Marrow Transplant Team.

These data demonstrate that endocrine function abnormalities influencing subsequent growth and development in children after marrow transplantation rarely occur after preparation with CY alone. However, after administration of regimens containing TBI, multiple endocrine function abnormalities can occur. Although longer follow-up is needed, it appears that the addition of BU to CY does not result in thyroid function or growth abnormalities. Pubertal development and gonadal function, however, may be adversely influenced by BU and patients may require supplementation with appropriate gonadal hormones.

Adolescent↗

[The role of echography in the complex dynamic examination of patients with retardation of sexual development].

Seventy-nine patients with sexual development retardation were examined, 24 of these suffered from ovarian genesis condition and 55 from central genesis condition. The findings evidence that detection of the uterus and gonads presenting as cords is one of the diagnostic criteria indicating gonadal dysgenesis. Echographic examinations carried out over the course of therapy yield a more accurate picture of ovarian function. No increase in uterine size on the echogram after discontinuation of hormonal therapy and the appearance of follicles in the ovaries after treatment point to normally functioning ovaries and helps specify the origin of sexual development retardation. In sexual development retardation of a central origin ultrasonic scanning helps assess the therapy efficacy and predict its outcome.

Adolescent↗

[Transient complete somatotropin insufficiency. A retrospective study].

Two groups of patients with short stature and a limitation in the growth hormone (GH) response (peaks of 10 mU/l or below in response to 2 stimuli) differed by their GH responses at puberty: normalisation in group 1 (12 patients) and lack of normalisation in group 2 (13 patients). The condition of the patients of group 1 appeared to be less severe as judged by their initial height deficiency, their growth velocity and their GH peaks. Sex steroid hormone priming tests were highly discriminative, especially for the GH response to insulin stimulation. Other pituirary hormone deficiencies were never found in group 1, but did occur in group 2. Finally the beneficial long term effect of hGH therapy was questionable in group 1 in that there was no difference between the final heights of the treated (n = 7) and the untreated (n = 5) patients.

Adolescent↗

[Complex assessment of sexual development of pubertal girls with diabetes mellitus].

Abnormal sexual development of girls suffering from diabetes mellitus was shown to depend on the diabetes gravity and degree of its compensation. The patients with a history of multiple diabetic comas were characterized by dramatic retardation of sexual development. In view of this fact it is assumed that deep disorders of metabolism may influence the central brain structures responsible for reproductive function.

Adolescent↗

Ultrasensitive two-site immunometric assay of human lutropin by time-resolved fluorometry.

A rapid, sensitive two-site immunometric assay for human lutropin (LH), with two monoclonal antibodies directed against the beta subunit, has been developed with time-resolved fluorescence as the detection principle. The cross-reactivities with human thyrotropin and human follitropin are negligible and that with human choriogonadotropin is 1% in the standard two-step protocol. Free beta subunits of LH are also detected. Besides the standard procedure (45 + 15 min), more rapid alternative protocols (15 + 15 min or 30 min in one step) have also been evaluated. The minimum detectable dose per well is approximately 1 amol (10(-18) mol), corresponding to less than 0.01 int. unit/L, for a sample volume of 25 microL and less than 0.001 int. unit/L for 200-microL samples. The intra- and interassay CV is less than 6% for LH concentrations from 0.2 to 250 int. units/L. With 200-microL sample volumes, intra-assay variation is less than 6% at 0.04 int. unit/L and interassay variation is 7% at 0.05 int. unit/L. The very high sensitivity and reproducibility of the presented method enables assay of low prepubertal and of suppressed LH concentrations, and represents a clear improvement over currently available immunoassays.

Adult↗

Hypothyroidism in children/adolescents. Clinical and hormonal profiles.

This paper describes the clinical, hormonal and radiologic profiles in 282 children evaluated for hypothyroidism. Short stature, mental retardation or puberal disturbances were often the presenting features in the older age group, whereas in the 1-5 years age group medical opinion was usually sought for symptomatology suggestive of thyroid hypofunction. Children in the 0-1 year group were suspected on the basis of psychomotor dysfunction. Skeletal immaturity was found in 93.0% of patients with overt hypothyroidism and in 36.6% cases with normal thyroid profiles but associated with malnutrition. High TSH levels were noted in 70.9% of the cases studied. 4.9% and 7.3% patients with normal TSH had low T3, and T4 levels respectively. FSH, testosterone and PRL levels were also affected in some patients with overt hypothyroidism. Therapeutic responses based on at least 1 year follow up were available in 170 cases. The results are discussed.

Adolescent↗

[Pituitary reserve of growth hormone in adolescent patients with cerebral palsy and mental retardation].

The pituitary reserve of growth hormone (GH) was studied in 11 severely handicapped boys (14 approximately 18 years old) with mental retardation and cerebral palsy (CP); athetotic type 4 cases, and spastic type 7 cases. Serum GH was determined before and after intravenous injection of 1 ng/kg growth hormone releasing factor (GRF) or oral administration of 0.1 mg/m2 clonidine. The athetotic type patients showed low somatomedin C (Sm C) concentrations (0.31 +/- 0.11 U/ml), poor responses to clonidine, and good responses to GRF. The spastic type patients showed subnormal responses to GRF although their Sm C was normal (0.91 +/- 0.43 U/ml). Clinically, the stature was small, and the puberty was delayed in the former. The levels of plasma testosterone were significantly lower than those in the latter. It is concluded that athetotic type CP was associated with hypothalamic GRF deficiency and spastic type CP was associated with reduced pituitary reserve of GH.

Adolescent↗