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Spontaneous feminization in a 46,XX female patient with congenital lipoid adrenal hyperplasia due to a homozygous frameshift mutation in the steroidogenic acute regulatory protein.

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Prijava / prijava

The most severe form of congenital adrenal hyperplasia (CAH) is lipoid CAH. It was once thought that this disease was due to mutations in the cholesterol side-chain cleavage enzyme system, thus eliminating the ability to convert cholesterol to pregnenolone, causing a complete absence of steroid

Haemophilia A in a patient with testicular feminization.

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Prijava / prijava

A case of typical haemophilia A in phenotypically "hairless" women aged 18 with complete testicular feminization (primary amenorrhea, absence of palpated gonads, negative sex chromatin, fluorescence of Y chromosome in interphase nuclei, caryotype 46, XY by common and fluorescent methods) is reported

Feminization in a galactosemic girl in the presence of hypergonadotropic hypogonadism.

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Prijava / prijava

A galactosemic girl has been followed in our clinic since her 8th day of life when the diagnosis of transferase-deficiency galactosemia was made until her present age of 21 years. Although she presented with direct hyperbilirubinemia and severe liver dysfunction, her subsequent somatic and

Spontaneous feminization and menstrual function developing during puberty in Turner's syndrome.

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Prijava / prijava

An 18-year-old patient with Turner's syndrome presented with cyclical vaginal bleeding and spontaneous development of secondary sexual characteristics. She demonstrated classic features of Turner's phenotype, and a culture of blood lymphocytes revealed a 45,XO karyotype. The patient's plasma and

Histological alternation and vitellogenin induction in adult rare minnow (Gobiocypris rarus) after exposure to ethynylestradiol and nonylphenol.

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Prijava / prijava

Adult rare minnow (Gobiocypris rarus) were exposed to 0, 1, 5, and 25 ng/l (nominal concentrations) of 17alpha-ethynylestradiol (EE2) and 3, 10, and 30 microg/l (nominal concentrations) of 4-nonylphenol (NP) under flow-through conditions for a period of 28 d. Low mortality was observed at 5 and 25

Management of ambiguous genitalia in pseudohermaphrodites: new perspectives on vaginal dilation.

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Prijava / prijava

OBJECTIVE To evaluate vaginal size and sexual activity after different techniques of feminization of external genitalia in patients with pseudohermaphroditism. METHODS Retrospective clinical study. METHODS Pseudohermaphrodite patients seen at our institution. METHODS Three female and 20 male

Molecular pathogenesis of lipoid adrenal hyperplasia and adrenal hypoplasia congenita.

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Prijava / prijava

Congenital lipoid adrenal hyperplasia (lipoid CAH) is the most severe form of CAH in which the synthesis of all gonadal and adrenal cortical steroids is markedly impaired. Lipoid CAH may be caused by the defect in either the steroidogenic acute regulatory (StAR) protein or the P450scc. More than 34

Prenatal stress disrupts reproductive behavior and physiology in offspring.

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Prijava / prijava

Prenatal maternal stress has been related to neonatal activity and irritability in both lower animals and humans in documented research for at least the past 30 years. Contemporary animal research demonstrates that prenatal stress feminizes and demasculinizes the sexual behavior of males and reduces

Hemophilia A in a phenotypic female with normal male karyotype associated with a low factor XII level.

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Prijava / prijava

Hemophilia A was detected in a 40-year-old black Gabonese female prior to thoracic surgery for empyema. The diagnosis of mild hemophilia A was supported by the findings of low factor VIII coagulant activity (VIII:C 4%), normal levels of factors VIII related antigen (VIIIR:Ag) and VIII von Willebrand

Hemostasis by premedication with estrogen in hair-transplant surgery.

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Prijava / prijava

Premedication with water-soluble conjugated estrogens administered the previous day and a half-hour before operation diminished bleeding and allows for a drier field, better vision and quicker work. Post-operative bleeding is negligible. Their short-term use has not led to feminization nor presented

Ovarian resistance to luteinizing hormone: a novel cause of amenorrhea and infertility.

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Prijava / prijava

OBJECTIVE To report the clinical, hormonal, and histopathological features of a woman with ovarian resistance to LH. METHODS Clinical study. METHODS University hospital. METHODS A woman with amenorrhea, sister of a patient with male pseudohermaphroditism due to Leydig cell hypoplasia. METHODS Blood

Bone marrow hypoplasia in eight dogs with Sertoli cell tumor.

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Prijava / prijava

Eight male dogs with Sertoli cell tumor had pancytopenia and bone marrow hypoplasia attributed to endogenous estrogen myelotoxicosis. Clinical signs were hemorrhage caused by thrombocytopenia, anemia caused by blood loss or diminished erythrocyte production, and infection and fever associated with

Refractory hypertension and isosexual pseudoprecocious puberty associated with renin-secreting ovarian steroid cell tumor in a girl.

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Prijava / prijava

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore,

Clinical and laboratory evaluation of patients with primary amenorrhea.

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Prijava / prijava

Sixty-two patients with primary amenorrhea were retrospectively categorized into 4 groups: 1) breast development absent and uterus present (29 patients), 2) breast development present and uterus absent (9 patients), 3) both breast development and uterus absent (2 patients), and 4) both breast

Molecular pathology and mechanism of action of the steroidogenic acute regulatory protein, StAR.

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Prijava / prijava

The first and rate-limiting step in the synthesis of all steroid hormones is the conversion of cholesterol to pregnenolone by the mitochondrial enzyme, P450scc. Tropic hormones such ACTH and gonadotropins induce steroidogenesis via cAMP by elaborating intracellular cAMP which stimulates P450scc

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