Detection of 3 beta-hydroxysteroid dehydrogenase deficiency in a newborn by means of urinary steroid analysis.

A urinary steroid excretion pattern of a 3-wk-old newborn, suffering from 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency, has been produced, employing capillary gas chromatography and subsequent mass spectrometric identification of the various excreted steroids. The diagnosis could be established, apart from the clinical symptoms, on the basis of a grossly elevated excretion of 16-OH-DHEA and 16-OH-pregnenolone, combined with mass spectrometric identification of the following steroids: 17-OH-preganolone, pregnanetriol, pregnanolone, pregnenetriol and 17-OH-pregnenolone.

Cloning of a cDNA encoding the human cation-dependent mannose 6-phosphate-specific receptor.

Complementary DNA clones for the human cation-dependent mannose 6-phosphate-specific receptor have been isolated from a human placenta library in lambda gt11. The nucleotide sequence of the 2463-base-pair cDNA insert includes a 145-base-pair 5' untranslated region, an open reading frame of 831 base pairs corresponding to 277 amino acids (Mr = 30,993), and a 1487-base-pair 3' untranslated region. The deduced amino acid sequence is colinear with that determined by amino acid sequencing of the N-terminus peptide (41 residues) and nine tryptic peptides (93 additional residues).

Na+ currents generated by the purified (Na+ + K+)-ATPase on planar lipid membranes.

Purified (Na+ + K+)-ATPase from pig kidney was attached to black lipid membranes and ATP-induced electric currents were measured as described previously by Fendler et al. ((1985) EMBO J. 4, 3079-3085).

Determination of polyamines and metabolites in cerebrospinal fluid by isotope dilution mass fragmentography, and a clinical application.

Capillary gas chromatography and mass fragmentography was used to determine simultaneously 1,3-diaminopropane, putrescine, cadaverine, spermidine, spermine, isoputreanine and putreanine in cerebrospinal fluid. After addition of deuterium labelled analogs and acid hydrolysis, the compounds were isolated by adsorption onto silica and converted into their N-heptafluorobutyryl-methylesters. Quality control data and an application of the method are given.

[Effect of thymostimulin on chemotherapy-induced changes in lymphocyte subset distribution. A longitudinal study in patients with primary inoperable oropharyngeal cancers].

20 patients with primarily inoperable squamous cell carcinoma of the head and neck were treated with 2 cycles of chemotherapy prior to surgical treatment. The lymphocyte subsets were determined prior to chemotherapy, as well as directly after and 3 weeks after start of each chemotherapy cycle. For this, 2 different flow cytometric techniques were used in parallel.

Differential expression of endogenous sugar-binding proteins (lectins) in murine tumor model systems with metastatic capacity.

In order to investigate possible differences in sugar binding activities of strongly versus weakly metastatic tumors, sugar-binding molecules (endogenous lectins) of murine tumor cells differing in metastatic capacity were analyzed by affinity chromatography on supports with immobilized sugars or glycoproteins and compared. After elution with specific sugar in the absence of Ca2+-ions, the proteins were separated by sodium dodecyl sulfate-polyacrylamide slab gel electrophoresis. In comparison to a weakly metastatic subline (Eb) spontaneous strongly metastatic variants (ESb) of a murine lymphoma contained additional sugar receptors for N-acetylglucosamine (Mr 30 kDa) and maltose (Mr 64 kDa, 62 kDa, 54 kDa and 32 kDa), and lacked one sugar receptor for myoinositol (Mr 85 kDa), N-acetylglucosamine (Mr 23 kDa) and maltose (Mr 22 kDa), respectively.

Medroxyprogesterone acetate and lipid metabolic changes.

Lipid metabolic changes under oral treatment with medroxyprogesterone acetate (MPA) were investigated in four groups of patients: group I; 10 patients aged 25-45 (mean 38) years received 50 mg MPA daily for pelvic endometriosis. Group II; 21 patients aged 55-77 (mean 62) years received 200 mg MPA daily for surgically treated endometrial carcinoma stage I. Group III; 14 praemenopausal patients aged 37-52 (mean 47) years received 1000 mg MPA daily for metastasized breast cancer.

Genetic heterogeneity of steroid sulfatase deficiency revealed with cDNA for human steroid sulfatase.

Three cDNA clones with inserts of 1.2-1.6 kb that reacted both with antibodies and oligonucleotides specific for steroid sulfatase were isolated from a human placental library in lambda gt11.

In vitro adsorption of colon cancer sera over staphylococcus protein A: lymphocyte stimulation by leakage of adsorbance.

Serum factors may be responsible for reduced host-anti-tumor defence. Although there is still confusion about their origin, attempts have been made to immobilize serum components by Protein A columns as a therapeutic modality. In our study the in vitro adsorption of 90% of the IgG from cancer sera on "immobilized protein A" did not influence the inhibitory serum activity as measured in a mixed lymphocyte culture.

Intensive short-term chemotherapy in patients with advanced breast cancer.

Aiming at a high complete remission rate with an intensive induction regimen, 27 patients with advanced breast cancer were given three cycles of VAC chemotherapy consisting of vindesine 3 mg/m2 i.v. on days 1 and 12, adriamycin 40 mg/m2 i.

Abnormal urinary bile acids in a patient suffering from cerebrotendinous xanthomatosis during oral administration of ursodeoxycholic acid.

The urinary bile acid profile, obtained by capillary gas chromatography, of a patient suffering from cerebrotendinous xanthomatosis and treated with ursodeoxycholic acid demonstrated, besides the occurrence of 23-norcholic acid and (23R)-hydroxycholic acid (as a consequence of this disease), six additional unknown bile acids and three known bile acids, viz. ursodeoxycholic acid, hyocholic acid and omega-muricholic acid. The structure of two of the unknown bile acids were elucidated and proven by organic syntheses.

Sensitivity of leukemia cell lines to cytotoxic alkyl-lysophospholipids in relation to O-alkyl cleavage enzyme activities.

The human leukemia cell lines K562, HL60, and Raji and the mouse leukemia cell line L1210 showed a differential susceptibility to the action of the alkyl-lysophospholipid (ALP) 1-octadecyl-2-methyl-rac-glycero-3-phosphocholine (ET-18-OCH3). After 48 hours, the 50% growth-inhibition doses (ID50) of ET-18-OCH3 were found to be 0.78 microgram/ml (HL60), 1.

Determination of the urinary cortisol production rate using (1,2,3,4-13C)cortisol. Isotope dilution analyses at very small enrichments.

An isotope dilution mass spectrometric method to determine the urinary cortisol production rate (CPR) in babies and children is described. The method uses stable isotopically labelled (1,2,3,4-13C)cortisol. The tracer is intravenously administered to the patient and urine is collected for the following three days.

Calcium content in some organs of rats treated with a toxic calciol dosis.

Administration of 100,000 IU of calciol for 3 consecutive days to female rats increased calcium levels in serum, kidney, duodenum and cardiac muscle, but was without effect on calcium content in skeletal muscle, liver and brain.

Characterization of membrane lectins in human colon carcinoma cells by flow cytofluorometry, drug targeting and affinity chromatography.

Flow cytofluorometry and drug targeting with labelled neoglycoproteins are used as tools to probe for membrane lectins in two human adenocarcinoma cell lines. Both cell lines express activities for galactosides, glucosides and fucosides. Affinity chromatography on gels with immobilized sugar leads to purification of an alpha-galactoside-binding protein at an apparent molecular weight of 64 kDa that also binds to lactose, maltose and fucose and exhibits Ca2+-requirement for binding, a beta-galactoside-binding protein without Ca2+-requirement at an apparent molecular weight of 14 kDa, and an alpha-glucosyl-binding protein without Ca2+-requirement at an apparent molecular weight of 34 kDa from both cell lines.

Aminoglutethimide in the treatment of premenopausal patients with metastatic breast cancer.

Eighteen premenopausal patients with progressive metastatic breast cancer were treated with aminoglutethimide (AG)/cortisone. All patients received 1000 mg AG per day in combination with 2 X 25 mg cortisone acetate. Complete (CR) and partial remissions (PR) were achieved in 27.

Measurements of prednisolone and some of its metabolites, in urine of patients after orthotopic liver transplantation, as a means of monitoring prednisolone absorption.

In patients who had undergone orthotopic liver transplantation, malabsorption of prednisolone or increased metabolism of prednisolone was suspected. In order to rule out this possibility, urinary prednisolone, and some of its metabolites, viz prednisone and 6 beta-hydroxyprednisolone, were determined by means of a gas chromatographic assay. To evaluate this assay aliquots of a pooled urine from several of our patients were analysed in multiplicate (n = 10).

Aminoglutethimide and medroxyprogesterone acetate in the treatment of patients with advanced breast cancer. A phase II study of the Association of Medical Oncology of the German Cancer Society (AIO).

One hundred twenty-eight women with advanced metastatic breast cancer were treated with a combination of aminoglutethimide (AG) (1000 mg orally, daily) and medroxyprogesterone acetate (MPA) (1500 mg orally, daily for six weeks and thereafter 500 mg orally, daily; omitting cortisone substitution). AG/MPA did not lead to side effects other than those described under AG or MPA monotherapy. Mental and personality changes seem to be more severe and frequent under combined therapy than under monotherapy.

Suppressed colony formation of peripheral blood lymphocytes in a patient with somatostatinoma.

The in vitro clonal growth of T lymphocytes was examined in a patient with somatostatinoma over a period of 8 months. After phytohemagglutinin stimulation colony-forming unit T lymphocytes could not be detected as compared to healthy controls and patients with metastatic colon tumors. The somatostatin produced by this tumor was identified as somatostatin-14.

Increased (23R)-hydroxylase activity in patients suffering from cerebrotendinous xanthomatosis, resulting in (23R)-hydroxylation of bile acids.

Patients suffering from cerebrotendinous xanthomatosis, an inborn error of metabolism in bile acid synthesis, excrete excessive amounts of 23-hydroxylated bile alcohols, 23-norcholic acid and 23-hydroxycholic acid into urine. In this study the configuration of this excreted 23-hydroxycholic acid was established as (23R)-hydroxycholic acid. Urine samples of two treated patients, receiving chenodeoxycholic acid, were investigated to see whether this administered bile acid was partly converted into 23-hydroxychenodeoxycholic acid.

[Metergoline in hyperprolactinemic breast cancer: a phase II study].

The prolactin release inhibiting action of the dopamine receptor agonist metergoline was investigated in 16 patients with metastatic breast cancer associated with hyperprolactinemia. At a daily dose of 12 mg p.o.