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Biomedical subjects

A Decristoforo

Publications and source records attributed to A Decristoforo.

At least 19 recordsLinked to original sources
PubMed1987

Pseudovaginal perineoscrotal hypospadias. Clinical, endocrinological and biochemical characterization of a patient.

A 16-year-old adolescent with perineal hypospadias, a microphallus, a bifid, labia-like scrotum, a blind-ending vagina and bilaterally undescended testicles is presented. Despite normal development of the Wolffian duct derivatives--deferentography showed that both ejaculatory ducts opened into the urethra--the patient showed severe developmental abnormality of the external genitalia. On endocrinologic investigation, gonadotropin, testosterone and 17 beta-estradiol levels were within normal limits; in a genital skin fibroblast culture testosterone was not reduced into 5 alpha-dihydrotestosterone, however, androgen receptors could be identified. Since this patient was raised as a girl up to the age of 16 and had developed female identity, the patient was further raised as a female. Bilateral orchiectomy was performed and estrogen substitution therapy was started. Clitoroplasty as well as ileal vaginoplasty were performed.

Adolescent↗
PubMed1986

Characterization of the testicular abnormality in 5 alpha-reductase deficiency.

The testes of five phenotypic women (from four families) with 5 alpha-reductase deficiency were studied. In one of the patients, the enzyme deficiency was similar in the testis and epididymis and in fibroblasts cultured from the labia majora. In testes from four of the patients, the concentrations of the 5 alpha-reduced steroids dihydrotestosterone and 3 alpha-androstanediol were less than 10% of those in normal subjects. We conclude that the testis is involved in 5 alpha-reductase deficiency. Impaired spermatogenesis was evident in testicular biopsies from all five subjects, and in two, sperm production, as estimated in testicular homogenates, was less than 10% of normal. The extent to which spermatogenic arrest is due to 5 alpha-reductase deficiency or testicular maldescent is not clear.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1978

[Postoperative fructose infusion in a case of presumed hereditary fructose intolerance (author's transl)].

Hereditary fructose intolerance (HFI) was diagnosed in a 61 year-old male patient on account of liver dysfunction followed by prolonged shock immediately after the administration of a fructose and lactose infusion postoperatively. The diagnosis of HFI was based on an increased value of fructose, hypoglycaemia, lactic acidosis and diminution of the phosphate level in combination with the typical family history. The patient's children showed a normal reaction to fructose administration. The therapy included glucose, insulin and heparin administration, balance of acidosis and partial exchange of blood, which resulted in improvement in the glucose level, coagulation factors and acidosis, but could not prevent further liver damage and uraemia with a fatal outcome.

Acidosis↗
PubMed1977

[Pre-operative renal artery occlusion with a balloon catheter (author's transl)].

Renal artery occlusion with a balloon catheter introduced by the transfemoral route makes nephrectomy for tumour much easier. Advantages of vascular occlusion are a bloodless field and shrinking of the kidney with a significant reduction of blood loss during the operation. In addition, operative displacement of tumour thrombus is largely avoided.

Catheterization↗
PubMed1977

[Urological symptoms in aneurysms of the abdominal aorta and its branches].

The clinical symptomatology of the sclerotic aneurysms of the abdominal aorta and its large branches is described, especially are described symptoms which are erroneously referred to the urinary tract. In our own clinical material of 47 patients with an aneurysm of the abdominal aorta 11 patients (23%) were primarily transferred to the urological treatment. The typical clinical picture, especially characteristic radiological changes are characterised in detail. It is referred to the early diagnosis and thus to the improvement of the prognosis of the aneurysms of the aorta.

Aged↗