Cases reported "Puberty, Precocious"

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1/13. Primary hypothyroidism presenting as ovarian tumor and precocious puberty in a prepubertal girl.

    We report a case of a prepubertal girl with juvenile primary hypothyroidism presenting as ovarian cysts and precocious puberty. The 7-year-old female was referred to our clinic because of a pelvic/abdominal mass and vaginal bleeding. Besides these findings, on physical examination we noticed the thyroid gland globally increased and the presence of secondary sexual characteristics. Based upon the clinical profile and investigations, the patient was diagnosed with juvenile primary hypothyroidism due to autoimmune thyroiditis. The cysts and precocious puberty resolved spontaneously after the simple replacement of thyroid hormone. It is important to bear in mind hypothyroidism in cases of girls presenting ovarian cysts and precocious puberty in order to avoid unnecessary surgery on the ovaries.
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2/13. A boy with McCune-Albright syndrome associated with GH secreting pituitary microadenoma. Clinical findings and response to treatment.

    The McCune-Albright Syndrome (MAS) is a sporadic rare disease first described in 1936 by McCune and separately by Albright. MAS is characterized by a triad of physical signs: cafe-au-lait spots, polyostotic fibrous dysplasia and autonomous endocrine hyperfunction. MAS is predominantly observed in girls and is rarely reported in males. We report the case of a 9-year old boy with gonadotropin independent precocious puberty, cafe-au-lait spots, polyostotic fibrous dysplasia and growth hormone hypersecretion.
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3/13. Unsustained central sexual precocity in four girls.

    Four girls who presented with breast enlargement at 4-5.8 years of age have been followed without specific therapy for up to 4 years. Three had normal CT brain scans, one had normal skull and sella x-rays. Stimulation of gonadotropins by LHRH was excessive in all but plasma estradiol levels were only intermittently elevated. Initially, bone age was advanced and height velocity was increased in three of the four. Ultrasound visualized an enlarged uterus in two and waxing and waning ovarian cysts in all. The clinical course was characterized by persistence of physical signs over at least 3.4 years in one patient, fluctuation in another, and marked regression in two. We propose that some patients with central precocious puberty may spontaneously have a nonprogressive course which has to be considered when evaluating the efficiency of drugs interfering with puberty.
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4/13. Viralization due to leydig cell tumor diagnosis by magnetic resonance imaging. case management report.

    A 3-year-old boy who presented with signs of virilization had a leydig cell tumor of the left testis that could not be detected by physical examination or by high resolution ultrasonography. His very small tumor was demonstrated by magnetic resonance imaging. The implications of adding this sensitive method of imaging to the evaluation of prepubertal virilization of presumed testicular etiology are discussed.
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5/13. Leprechaunism: in vitro insulin action despite genetic insulin resistance.

    We recently identified a female leprechaun infant with marked hyperinsulinemia [as high as 10,975 microU/ml (78,746 pmol/liter)], presumably secondary to insulin resistance. She had two physical findings suggestive of possible insulin action: cystic ovarian enlargement with gonadotropin-independent steroid secretion and persistent, severe myocardial hypertrophy. To examine the pathophysiology of this disorder we measured the in vitro sensitivity to insulin and other growth factors of erythroid progenitors and a T-lymphoblast cell line derived from her peripheral blood. Resistance to insulin was demonstrated by failure of her circulating erythroid progenitor cells to augment proliferation in response to physiologic concentrations of insulin (1-10 ng/ml). An immortalized T lymphoblast cell line was established by transforming the cells with the human retrovirus human T cell leukemia virus II. This cell line showed little or no response to physiologic concentrations of insulin contrary to consistently observed stimulation of colony formation by cell lines similarly derived from normals. The patient's T lymphoblasts, however, showed normal sensitivity to insulin-like growth factor i. In response to supraphysiologic insulin concentrations (25-1000 ng/ml), leprechaun T lymphoblasts showed significant augmentation of colony formation (peak 189% above baseline at 50 ng/ml); normal T lymphoblasts also showed responsiveness at these high insulin concentrations. Preincubation with a monoclonal antibody against the insulin-like growth factor i receptor (alpha IR-3 at 5000 ng/ml) blocked the in vitro effect of physiologic concentrations of insulin-like growth factor and supraphysiologic concentrations of insulin on leprechaun and control T lymphoblast colony formation, but had no clear effect upon the response to physiologic insulin concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)
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6/13. Genital examination and exposure experienced as nosocomial sexual abuse in childhood.

    Three pediatric cases of girls, one with idiopathic precocious puberty and two with a birth defect of the sex organs, exemplify the proposition that genital exposure and the physical examination of the genitals may be experienced subjectively as nosocomial sexual abuse. Negative sequelae persisted into adulthood. The dogma of the new victimology industry is that children never lie about sexual abuse. Consequently providers of pediatric and ephebiatric (pubertal and adolescent) sexual health care already are progressively at risk of being falsely accused of nosocomial (from the Greek nosokomeion, from nosos, disease, komeion, to take care of: pertaining to or originating in a hospital, as nosocomial disease) sexual abuse.
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7/13. Intrauterine growth retardation and early adolescent growth spurt in two sisters.

    Two sisters who presented with a similar growth pattern are described. They delivered with idiopathic intrauterine growth retardation and had an early adolescent growth spurt. The physical and endocrine findings suggested a potential relationship between intrauterine growth retardation and early puberty.
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8/13. Deletion short arm 18 and silver-russell syndrome.

    A 14-year-old boy presented with physical signs of the silver-russell syndrome. He further had low-set, large protruding ears, ptosis, broad nasal base, thick protruding lips, pronounced caried, malaligment of teeth, micrognathia and mental retardation. Chromosome examination showed deletion of the short arms of chromosome No. 18. It is concluded that considering the previous findings of chromosome No. 18 aberrations in some patients with silver-russell syndrome such patients should always have chromosome examination made.
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9/13. growth and development in a girl with pseudohypoparathyroidism and hypothyroidism. A longitudinal study.

    Statural growth, physical and skeletal development of a girl with pseudohypoparathyroidism and primary hypothyroidism were analysed in a longitudinal study, which lasted for 12 years from the age of 0.8 years. Growth in height, which slowed down when thyroid therapy was withheld, was within the normal range. Still, as a result of early puberty, the girl became a small adult. Skeletal age was advanced over chronological years by an average of 2.7 years. This rapid skeletal development was more pronounced in the tubular bones than in the round bones of the hand. Two of the five metacarpals of the left hand grew more slowly than the others. This became clinically apparent at the age of 5.6 years. In all five metacarpals growth ceased at the same time, indicating that the abnormally short size of the two metacarpals did not result from early epiphyseal closure.
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10/13. Primary hypothyroidism and ovarian activity evidence for an overlap in the synthesis of pituitary glycoproteins. Case report.

    A 14-year-old girl presented with precocious sexual development, galactorrhoea and symptoms and signs suggestive of hypothyroidism. On physical examination a tumour was found in the lower abdomen. serum gonadotrophins, especially luteinizing hormone, serum thyroid stimulating hormone and prolactin were elevated. Measurement of thyroid hormones and additional thyroid function tests confirmed the diagnosis of primary hypothyroidism. Ultrasound investigation revealed the mass in the lower abdomen to be of a cystic nature and to originate from the right ovary. Following the institution of thyroid substitution therapy, all symptoms disappeared, biochemical and hormonal abnormalities returned to normal and the ovarian size decreased to normal. A hypothesis is presented for non-specific pituitary glycoprotein hormone synthesis secondary to the hypothyroidism, as the cause of the syndrome.
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