Fabry's disease: alpha-galactosidase deficiency.

The leukocytes of male patients with Fabry's disease are deficient in alpha-galactosidase. The alpha-galactosidase activity in the leukocytes of female carriers of the disease is 15 to 40 percent of the amount present in normal leukocytes. The activities of beta-galactosidase, beta-acetylgalactosaminidase, and beta-acetylglucosaminidase in the leukocytes of affected individuals are normal.

Mucopolysaccharidosis: secondarily induced abnormal distribution of lysosomal isoenzymes.

Total activities of acid hydrolases in liver of two patients with mucopolysaccharidosis are decreased for beta-galactosidase, alpha-galactosidase, and arylsulfatase A; total activities of four other hydrolases are normal or increased. The isoenzyme distribution of five hydrolases (beta-glucuronidase, alpha-glucosidase, beta- galactosidase, N-acetyl-beta-glucosaminidase, and alpha-galactosidase) is ábnormal in that the isoelectric points (by isoelectric focusing) of these enzymes are more acid than in control liver. Along with the isoenzyme abnormalities different kinds of glycolipids were stored in kidney, liver, and brain. The isoenzyme abnormalities can be reproduced in vitro by addition of chondroitin sulfate to a homogenate of normal liver, suggesting that stable binding occurs between mucopolysaccharides and the hydrolase molecules. After the addition of chondroitin sulfate, the total activity of beta-galactosidase is inhibited, whereas other hydrolases are affected only slightly or not at all.

Production, purification and characterization of antibodies to 1,25-dihydroxyvitamin D raised in chicken egg yolk.

For this sensitive RIA for 1 alpha,25-dihydroxyvitamin D, we used antibodies to 1 alpha,25-dihydroxycholecalciferol-3-hemisuccinate conjugated to bovine serum albumin, raised in eggs by immunization of chickens. We describe an efficient method for purification of IgG from egg yolk. We characterized these antibodies with immunoelectrophoresis and by radioimmunoassay. These antibodies show a high affinity for 1 alpha,25-dihydroxyvitamin D3 but cross react with other vitamin D metabolites as well. Extraction and liquid chromatography are necessary to isolate the 1 alpha,25-dihydroxyvitamin D from human serum or plasma before determination by RIA. The sensitivity of the assay is estimated at 5 pg/tube.

Quality control of albumin solutions by size-exclusion high-performance liquid chromatography, isoelectric focusing, and two-dimensional immunoelectrophoresis.

Quality control of albumin solutions for human use is of utmost importance because the presence of impurities can provoke adverse reactions in treated patients. Three different techniques were used to detect the presence of albumin oligomers and polymers as well as foreign proteins in commercial solutions. The relative concentrations of the former two were estimated using size-exclusion high-performance liquid chromatography. Isoelectric focusing and two-dimensional immunoelectrophoresis allowed detection of other contaminants of proteinaceous nature. The application of this combination of techniques supersedes the traditional approaches (gel filtration on polydextran gels, electrophoresis) in specificity and speed. Analysis of 34 lots of commercial albumin solutions from 22 manufacturers revealed the superior quality of preparations of placental rather than plasmatic origin.

Mannosidosis in a litter of Persian cats.

Three kittens in a litter of Persian cats showed, from the age of eight weeks, tremor, ataxia, dysmetria, progressive weakness and emaciation. Cytoplasmic vacuolation was observed in neurons, mesenchymal and epithelial cells of tissues taken post mortem. The alpha-mannosidase activity of brain tissue of one cat tested was 4.8 per cent of control values and the urine of two cats contained large amounts of mannose-rich oligosaccharides.

[Prevalence and risk factors of Chlamydia trachomatis infection in clients of a venereal disease clinic in Amsterdam]. / Vóórkomen en risicofactoren van infectie met Chlamydia trachomatis bij bezoekers van een geslachtsziektenpolikliniek in Amsterdam.

We studied the prevalence of Chlamydia trachomatis (CT) infection as well as its predictors among a sample (1000) of visitors (651 male, 349 female) of a clinic for sexually transmitted diseases (STD) in Amsterdam. The prevalence of CT was higher than that of gonorrhoea: among men, 14.3% and 11.5% and among women, 12.9% and 6.3% respectively. Independent predictors for CT infections were for men: number of leucocytes in discharge or urine, nature of urethral discharge, age and sex behavior, and in women: being warned as source or contact of a STD, age, nature of cervical discharge and bleeding from the cervical ostium after insertion of a swab. As many as 92% of the male and 81% of the female visitors of our STD clinic should be examined for CT if the presence of anyone or more of these predictors is taken as a criterion. Even then, 5% of the CT infections in women would still be missed. Based on our study results, material for the diagnosis of CT will from now on be collected routinely from all female patients of our STD clinic as well as from all males with urethritis. We advocate the expansion in the Netherlands of facilities for laboratory CT detection and its standard inclusion in STD examination.

[Periodontal inflammation. Biochemical markers in crevicular fluid for diagnosis of periodontal disease]. / Pardontale onsteking. Biochemische merkers voor parodontale onsteking in sulcusvloeistof.

Based on a review of the literature the possible application of biochemical markers in the early detection of periodontal disease is discussed, using crevicular fluid.

Dog alpha-1-acid glycoprotein: purification and biochemical characterization.

Dog alpha-1-acid glycoprotein was purified to homogeneity from dog serum in a three-step procedure involving precipitation with sulphosalicylic acid, isoelectric focusing and size exclusion chromatography. The molecular heterogeneity in the peptide part and in the carbohydrate part of the molecule was investigated with analytical isoelectric focusing in a narrow pH range and crossed immunoaffinity electrophoresis with concanavalin A (con A) in the first-dimension gel. Up to seven molecular forms with different isoelectric points were found, whereas only a single con A-dependent molecular form was detected.

Galactosialidosis: low beta-galactosidase activity in serum after long-term clotting.

Serum beta-galactosidase activity was found to be markedly increased in clotting blood from normal subjects, but patients with galactosialidosis showed only a slight increase of this enzyme activity. Consequently, the beta-galactosidase activity was low in serum after long-term clotting in patients with this disease. The mechanism of the enzyme activation is unknown. The use of anticoagulants completely inhibited the activation.

HIV prevalence and risk behaviour among prostitutes and clients in Amsterdam: migrants at increased risk for HIV infection.

OBJECTIVES: To study groups of prostitutes and clients of prostitutes in order (i) to determine HIV prevalence and sexual risk behaviour, (ii) to determine differences between samples recruited within and outside a clinic for sexually transmitted diseases (STD) and (iii) to determine correlates of inconsistent condom use (ICU) among both groups. DESIGN: Participants were interviewed and anonymously tested for HIV-antibody; approximately half were recruited at a clinic for sexually transmitted diseases (STD) and half at prostitute working places. SETTING: An STD clinic and prostitute working places in Amsterdam in 1991. SUBJECTS: 201 female prostitutes without a history of injecting drugs and 213 male clients of female prostitutes. MAIN OUTCOME MEASURES: antibodies to HIV, consistency of condom use in commercial vaginal contacts in the preceding 6 months. RESULTS: HIV prevalence was low: three prostitutes (1.5%; 95% CI 0.5-4.6%) and one client (0.5%; 95% CI 0.1-3.3%) were infected. All three HIV positive prostitutes originated from AIDS-endemic countries, came to the Netherlands only recently and were recruited outside the STD clinic. Large differences between subgroups resulted from the two recruitment methods: while clients of prostitutes with relatively high risk behaviour were strongly represented among the STD clinic sample, high risk prostitutes were underrepresented in this sample. Consistent condom use (with 100% of contacts) was reported by 66% of prostitutes and 56% of clients of prostitutes. Inconsistent condom use was found to be high among prostitutes who had migrated from Latin America and among migrant clients of prostitutes. CONCLUSIONS: When monitoring HIV infection one must take into account imported cases. HIV prevention efforts should be particularly focused at prostitutes from Latin America and at clients of prostitutes who migrated to the Netherlands.