RESUMO
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.
Assuntos
3-Oxo-5-alfa-Esteroide 4-Desidrogenase/deficiência , Transtornos do Desenvolvimento Sexual/enzimologia , Testículo/enzimologia , Adolescente , Adulto , Androgênios/metabolismo , Transtornos do Desenvolvimento Sexual/patologia , Epididimo/enzimologia , Feminino , Fibroblastos/enzimologia , Humanos , Masculino , Fenótipo , Receptores Androgênicos/metabolismo , Túbulos Seminíferos/patologia , Pele/enzimologia , Espermatogênese , Testículo/patologiaRESUMO
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.
Assuntos
Cateterismo , Embolização Terapêutica/métodos , Artéria Renal , Cateterismo/efeitos adversos , Hemostasia Cirúrgica/efeitos adversos , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Planejamento de Assistência ao Paciente , RadiografiaRESUMO
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.
Assuntos
Erros Inatos do Metabolismo dos Carboidratos/complicações , Intolerância à Frutose/complicações , Frutose/efeitos adversos , Encefalopatia Hepática/induzido quimicamente , Acidose/complicações , Frutose/administração & dosagem , Humanos , Infusões Parenterais , Lactatos/metabolismo , Masculino , Pessoa de Meia-Idade , Cuidados Pós-Operatórios , Hiperplasia Prostática/cirurgiaAssuntos
Prostatectomia/mortalidade , Hiperplasia Prostática/cirurgia , Idoso , Anestesia Epidural , Raquianestesia , Transfusão de Sangue , Eletrocirurgia , Hemorragia/terapia , Humanos , Masculino , Métodos , Pessoa de Meia-Idade , Cuidados Pós-Operatórios , Complicações Pós-Operatórias , Prostatectomia/efeitos adversos , Doenças Prostáticas/cirurgia , Neoplasias da Próstata/cirurgia , Instrumentos Cirúrgicos , Síndrome , Obstrução do Colo da Bexiga Urinária/cirurgiaAssuntos
Carcinoma/cirurgia , Neoplasias da Bexiga Urinária/cirurgia , Bexiga Urinária/cirurgia , Adulto , Idoso , Biópsia , Carcinoma/diagnóstico , Carcinoma/mortalidade , Carcinoma/patologia , Carcinoma/radioterapia , Cistoscopia , Seguimentos , Humanos , Masculino , Métodos , Pessoa de Meia-Idade , Metástase Neoplásica , Cuidados Paliativos , Complicações Pós-Operatórias , Prognóstico , Radioterapia de Alta Energia , Uretra , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/mortalidade , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/radioterapiaRESUMO
Despite European unification regarding a common legal framework for many aspects of pharmaceutical production including industrial manufacture of pharmaceuticals, the practice of pharmacy in general, and of radiopharmacy in particular, differs substantially and are mainly regulated at the national level. Herein the authors discuss major European documents relevant for radiopharmacy practice in Europe and recent developments on the national level especially regarding the small-scale preparation of radiopharmaceuticals (RP). Issues related to marketing authorization (and exemptions from it), standards of preparation, quality requirements, regulations of clinical trials and education will be outlined. Standards for the industrial preparation of pharmaceuticals are defined in "Good Manufacturing Practice" (GMP), not taking into account specific requirements for the small scale, extemporaneous preparation of RP. The European Association of Nuclear Medicine EANM has published several documents based on GMP and called "Good Radiopharmaceutical Practice" (cGRPP) to specifically address this in an attempt to harmonize RP preparation across Europe. Clinical trials have been hampered by the introduction of directive 2001/20/EC again aimed at the marketing track of industrial production and currently a number of activities are ongoing to counterbalance this problem in radiopharmaceutical research. Additionally, the role of the European Pharmacopoeia in regulating quality requirements and the need for specific education and training in the small scale radiopharmaceutical preparation are also discussed.
Assuntos
Aprovação de Drogas/legislação & jurisprudência , Regulamentação Governamental , Marcação por Isótopo/normas , Medicina Nuclear/normas , Compostos Radiofarmacêuticos/normas , Europa (Continente)RESUMO
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.
Assuntos
Transtornos do Desenvolvimento Sexual/complicações , Hipospadia/diagnóstico , Adolescente , Humanos , Hipospadia/genética , Hipospadia/cirurgia , Masculino , Orquiectomia , Testosterona/metabolismoRESUMO
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.
Assuntos
Aorta Abdominal/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Doenças Urológicas/diagnóstico por imagem , Idoso , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Ruptura , Urografia/métodosRESUMO
Based on the practice of the Browne technique for hypospadias, epithelial skin strips were splinted with polyvinyl tubes. In an experimental study the well-known excellent tissue tolerance of the polyvinyl material was demonstrated. Definite enlarged diameter of the splinted epithelialized tubes was observed in comparing it with unsplinted controls.