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Results for “ADRENOGENITAL SYNDROME”

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At least 73 records · Page 4Linked to original sources
PubMed1989

Bilateral renal malacoplakia in systemic lupus erythematosus and adrenogenital syndrome.

We report a case of an 8-year-old girl with adrenogenital syndrome secondary to adrenocortical hyperplasia. Thirteen years later systemic lupus erythematosus developed with lupus nephritis. In spite of complex continuous immunosuppressive therapy, she died from terminal renal failure. At autopsy, extensive bilateral renal malacoplakia was discovered. The role of recurrent urinary tract infections and of immunological disturbances in the pathogenesis of malacoplakia is discussed.

Adrenal Hyperplasia, Congenital↗
PubMed1976

Our experience with the adrenogenital syndrome: a review of 16 cases.

An analysis of 16 children with the adrenogenital syndrome illustrates the diversity of the clinical and laboratory features of the disorder. The need for critical periodic evaluation of individual assessment of each child and the dosage of corticosteroids being used is emphasized.

Adolescent↗
PubMed1989

[Problems and characteristics of the orthodontic treatment of a patient with adrenogenital syndrome].

This article describes biochemical relationships accompanying pathological symptoms of C-21-hydroxylase deficiency (adrenogenital syndrome). Disturbances of bone growth and development have been observed, even during medical treatment. A discrepancy of 2 1/2 years was noted between the patients chronological age and his bone development. The patient reaches a maximum height of 155 to 160 cm. Supernumeraries and delayed mineralisation are to be expected. Surgical procedures require an increase in cortisone dosage. When considering orthodontic treatment, one must determine the most appropriate time to start, take into account the long retention period and pay particular attention to oral hygiene.

Adolescent↗
PubMed1989

Feminizing genitoplasty: one-stage genital reconstruction in congenital adrenogenital syndrome.

Complete one-stage genital reconstruction was made in five children with congenital adrenogenital syndrome. Removal of the cavernous body of the clitoris was made by preservation of the glans with its vascular and neural supply. The lesser lips of the pudendum were fashioned from the skin of the penis and the prepuce. During the same operation, the vaginal orifice was created by the inversion of an U-shaped flap taken from the perineum into the introitus vaginae. There were no surgical complications. The patients recovered with good cosmetic results. It is necessary to regularly dilate the new vaginal orifice.

Adrenal Hyperplasia, Congenital↗
PubMedDate not supplied

[Seminoma and adrenogenital syndrome].

The Authors report a case of a 26 year old patient affected by adrenogenital syndrome, likely due to 21 hydroxylase defect, hermaphroditism (46XX genotype and female phenotype) and worked hyperandrogenism; moreover a hidden testis neoplasm (seminoma) was associated.

Adrenal Hyperplasia, Congenital↗
PubMed1986

[Pseudo- and central precocious puberty due to adrenogenital syndrome].

We describe three patients in whom at the age of 4-7 yrs the diagnosis adrenogenital syndrome (21-hydroxylase deficiency) was made. There was no salt-loss. Two boys presented with precocious pubic hair development and increased growth velocity and bone maturation. The third patient was a severely virilized girl raised as a boy until she was 4 yrs old. Following an extensive psychiatric evaluation, it was decided to raise her as a girl and feminoplasty was performed. Clinical and biochemical evidence of central precocious puberty was present in one boy at the time of diagnosis at age 7 and developed under hydrocortisone substitution therapy in the two other patients. The precocious puberty was treated in two patients with the anti-androgen cyproterone acetate. In one boy suppression of pituitary gonadotropin secretion was obtained by LH-RH analogue (Buserelin) treatment.

Adrenal Hyperplasia, Congenital↗