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Biomedical subjects

C de Sanctis

Publications and source records attributed to C de Sanctis.

32 records · Page 2Linked to original sources
PubMed1993

Combined therapy with LHRH and HCG in cryptorchid infants.

In 238 boys with cryptorchidism, between the ages of 4 and 48 months, luteinizing hormone releasing hormone (LHRH) was administered as nasal spray 1.2 mg/day for 4 weeks. The nonresponders received human chorionic gonadotropin (HCG) 500 I.U. i.m. three times a week for 3 weeks. With the combined treatment 37.8% of testes descended into the scrotum. Testicular descent occurred more often in patients whose testes were located in a lower position. Histological findings indicated a reduction in the number and maturation of germ cells. A clear improvement of germ cells trophism was observed in boys hormonally treated and operated on before the 12th month of life. Early administered combined treatment with LHRH and HCG can be considered as a substitution of the gonadotropins insufficiency manifested by most cryptorchid infants in the first months of life.

Administration, Intranasal↗
PubMed1990

[Diagnosis of ambiguous genitalia].

Diagnosis in patients with ambiguous genitalia is based on various investigations. Simple genital examination is not sufficient to attribute sex. Scarce androgenization in a male patient or marked androgenization in a female may both lead to the same stages of genital ambiguity according to Prader. It is important to get information about genital ambiguity in the family, drug consumption during gestation and signs of virilization in pregnant mothers. External genital findings must be integrated by visualization of urogenital sinus by X-rays, ultrasounds and endoscopy. Furthermore, hormonal evaluations on plasma and 24 hours urines allow diagnosis of some disorders of adrenal and gonadal steroidogenesis. To define genetic sex, chromosomal examination is required to integrate X chromatine investigation and fluorescent staining of Y chromosome. Evaluation of psychosexuality in patients who have already got gender identity is mandatory.

Disorders of Sex Development↗
PubMedDate not supplied

Growth hormone treatment in irradiated children with brain tumors.

We assessed the efficacy of GH treatment in 25 GH deficient patients irradiated for brain tumors (eight with glioma cranio-irradiated, eleven with medulloblastoma and six with ependymoma craniospinal-irradiated). We administered GH at doses of 0.6-0.9 IU/kg/week for one to three years at least two years after diagnosis of the tumor. We assessed the efficacy of the treatment each year by comparing the values of height velocity over bone age and change in the ratios progression of chronological age/progression of bone age and progression of statural age/progression of bone age. The treatment promoted satisfactory growth; better results were obtained in patients with glioma, who received cranial irradiation only, than in those with medulloblastoma or ependymoma, who received spinal irradiation as well. Moreover, the growth prognosis improved, especially in the cranio-irradiated patients. In our series of patients four presented tumor recurrence; these results did not differ significantly from those in irradiated patients with cerebral tumors who were not treated with GH.

Adolescent↗
PubMedDate not supplied

McCune-Albright syndrome: a longitudinal clinical study of 32 patients.

We report the diagnostic clinical features and their long term evolution in 32 patients with McCune-Albright syndrome. Patient data are made up of two periods: the first, classified as personal history, is from birth until the time when the diagnosis of McCune-Albright syndrome was made; the second, classified as clinical observation, is from the first observation until the end of follow up. The total duration of these two periods was 9.6+/-2.9 yr; mean age at first observation was 5.7 yr (range 0.7-11 yr). The probability of manifesting main clinical signs according to age was calculated: almost all had skin dysplasia at birth, 50% probability of peripheral precocious puberty in females at 4 years and 50% of bone dysplasia at 8 years of age were found. Other clinical signs had diagnostic relevance when preceding the main signs leading to diagnosis of McCune-Albright syndrome even without specific genetic investigation. The most important clinical manifestations have different evolutions: skin lesions increase in dimensions according to body growth; precocious puberty in females evolves rapidly but periods of regression can be seen in some patients; bone dysplasia in most patients evolves with an increase both in the number of affected bones and in the severity of lesions.

Child↗
PubMedDate not supplied

[Cryptorchidism].

Cryptorchidism is the most frequent anomaly of an endocrine gland; it entails risks of infertility and testicular cancer. Its pathogenesis is thought to be multifactorial including anatomical and mechanical together with endocrine causes. In the last decades most Authors favoured the hypothesis that some degree of androgen insufficiency during fetal life may play a role in determining testicular maldescent. Morphological alterations have been demonstrated in cryptorchidism testes since birth; both tubular and interstitial damage already can be found in the first months of life. A critical phase in germinal development occurs during the third month when gonadotropin and testosterone postnatal surge induces the first maturational step in germ cells development. In cryptorchid infants, gonadotropin insufficiency at this time reduces germ cells differentiation, leading to progressive germinal decline, which becomes manifest after 12 months of life. In the opinion of some Authors, germinal alteration can be partially reversed by early scrotal reposition of the cryptorchid testes. Hormonal therapy with human chorionic gonadotropin (HCG) and luteinizing hormone releasing hormone (LHRH) has been used in the past decades with variable results. A combination of the two hormones has had a greater effect in inducing testicular descent compared to single hormone treatment. Although some surgeons already treat cryptorchid patients during childhood, an increasing number of pediatric surgeons believe that early treatment, performed during infancy is preferable, when considering the early and progressive histological damage seen in cryptorchid gonads. In our opinion, also hormonal treatment performed during the first year of life seems preferable since it can at the same time induce scrotal descent of cryptorchid testes and substitute postnatal gonadotropin insufficiency.

Adolescent↗
PubMedDate not supplied

Auxological and biochemical parameters in assessing treatment of infants and toddlers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

We studied height velocity (HV), bone age progression (delta BA/delta CA), urinary pregnanetriol (PT) and plasma 17-hydroxyprogesterone (17-OH-P) during the first years of life in 12 patients with 21-hydroxylase deficiency, treated by cortisone acetate. In the well-controlled phases normal growth rate (SDS between -1 and +1), satisfactory bone age progression (delta BA/delta CA < or = 1) and no clinical sign of poor treatment were found; in the undertreatment phases enhanced growth rate, rapid bone age progression and, in some instances, signs of virilization were found; in the overtreatment phases, reduced growth rate was the only sign of poor treatment. Hormonal values were only weakly correlated to therapeutic control. Therefore, growth rate evaluation can represent the best method of monitoring treatment in very young patients with 21-hydroxylase deficiency.

17-alpha-Hydroxyprogesterone↗