Further characterization of [3H]ifenprodil binding in rat brain.

The present study was undertaken to characterize [3H]ifenprodil binding in rat brain. [3H]Ifenprodil showed saturable, high-affinity binding at 4 degrees C. Specific binding, defined with 10 microM ifenprodil as a competitor, was inhibited biphasically by the s receptor ligands, GBR 12909, 1,3-di-o-tolylguanidine (DTG), and (+)-3-(3-hydroxyphenyl)-N-propylpiperidine ((+)-3-PPP).

Differential diagnosis of hyperphenylalaninaemia by a combined phenylalanine-tetrahydrobiopterin loading test.

We describe a new fully reliable method for the differential diagnosis of tetrahydrobiopterin-dependent hyperphenylalaninaemia (HPA). The method comprises the combined phenylalanine (Phe) plus tetrahydrobiopterin (BH4) oral loading test and enables the selective screening of BH4 deficiency when pterin analysis is not available or when a clear diagnosis has not been previously made. It should be performed together with the measurement of dihydropteridine reductase (DHPR) activity in blood.

Hyperphenylalaninemia and pterin metabolism in serum and erythrocytes.

The relationship between blood phenylalanine concentrations and serum and erythrocyte biopterin and neopterin concentrations was investigated in 20 phenylketonuric patients with different dietary compliance. At serum phenylalanine concentrations ranging from 43 to 1004 mumol/l, a good correlation was found with serum biopterin (r = 0.76, P < 0.

Catalytic activity of tetrahydrobiopterin in dihydropteridine reductase deficiency and indications for treatment.

It is now widely accepted that tetrahydrobiopterin (BH4), the natural cofactor of aromatic amino acid hydroxylases, in the absence of its regenerating enzyme dihydropteridine reductase (DHPR), will function only stoichiometrically in the phenylalanine (Phe) hydroxylating system. This has limited the use of pterin cofactor in diagnosis and treatment of patients suffering from inherited DHPR deficiency, one of the most common forms of hyperphenylalaninemia caused by BH4 deficiency. This is despite the observation of a dramatic fall in serum Phe concentration after BH4 loading in such patients.

[Hemodynamic changes in liver and multi-organ transplantation].

Multivisceral transplantation is a surgical technique developed as treatment for abdominal metastatic and/or multifocal malignancies. At present its clinical employment is reduced by our fragmentary knowledge of the intraoperative and postoperative outcome. The aim of this study is to compare intraoperative hemodynamic and respiratory changes during multivisceral transplantation (MTV, n = 12) and liver transplantation (OLTX, n = 14).

[Anhidrotic ectodermal dysplasia or Christ-Siemens-Touraine syndrome. A case report].

A case of anhydrotic dysplasia is reported. The history of the syndrome is reviewed and the possibility of identifying carriers of the gene that causes it discussed.

Effects of alpha 1-adrenergic blockade on pulsatile luteinizing hormone, follicle-stimulating hormone, and prolactin secretion in polycystic ovary syndrome.

Central noradrenergic mechanisms may participate in the regulation of pulsatile gonadotropin secretion in women with the polycystic ovary syndrome (PCO). To examine this possibility we measured serum LH, FSH, and PRL concentrations at 10-min intervals and total testosterone and 17 beta-estradiol at 60-min intervals for 8 h basally and during the infusion of the alpha 1-adrenoceptor antagonist thymoxamine (10 micrograms/kg X min) in 10 young women with PCO. Mean and integrated serum LH concentrations as well as LH pulse frequency were not significantly altered (P = NS) during the thymoxamine infusion.

[Presacrococcygeal congenital cyst in the adult].

Two cases of congenital cyst of the presacral space in the adult are presented. The unusualness of the lesion is stressed, and reference is made to the usefulness of a classification covering all retrorectal neoformations, including congenital forms. Attention is given to the manner of diagnosis.

[Actinic lesions of the ileum].

After an examination of the literature regarding the causes of lesions from rays, their most frequent sites, the diagnostic difficulties and the various types of treatment, the paper draws attention to the increase in such lesions in relation to the use of megavoltages, and the advisability of exploratory laparotomy in previously irradiated subjects even in cases apparently labelled as neoplastic relapses.