Variant of acute intermittent porphyria with normal erythrocyte uroporphyrinogen-I-synthase activity

A kindred in which several members have otherwise typical acute intermittent porphyria but normal erythrocyte uroporphyrinogen-I-synthase activity has been described from Finland. We studied two porphyric members of this kindred, two patients with typical acute intermittent porphyria, and two healthy controls using the delta-aminolaevulinic acid loading test and by measuring the erythrocyte enzymes of haem biosynthesis. The excretion pattern of haem precursors after the delta-aminolaevulinic loading test in the members of the kindred studied, was similar to that in typical acute intermittent porphyria suggesting an identical enzyme defect in the liver. The activity of all red cell enzymes studied was normal in the members of the kindred. The results suggest that porphyria in the kindred studied is a variant of acute intermittent porphyria, where the uroporphyrinogen-I-synthase defect is manifested in the liver but not in red cells.     

Effects of a chlorhexidine-fluoride-strontium rinsing program on caries, gingivitis and some salivary bacteria among Finnish school children

Abstract – In order to find out if it is possible to prevent caries and gingivitis by periodical use of chlorhexidine-fluoride mouthrinses with or without strontium, and to find out what effects they have on salivary mutans streptococci and lactobacilli counts, a total of 243 schoolchildren aged 11 yr with high DMFS scores were randomly divided into four groups. One group (C) served as a basic control. Subjects in the second group (GXF) rinsed their mouths twice a day every third week with a rinsing solution containing 0.05% chlorhexidine gluconate and 0.04% NaF. In the third group (CXFS) the rinsing solution contained 500 ppm Sr during the first and second year and 15 ppm during the last 6 months, in addition to chlorhexidine and fluoride. In the fourth group (CX) the solution contained only 0.05% chlorhexidine gluconate. All the rinsing solutions had pH 5.8 buffered with succinic acid-NaOH buffer. After 2 yr and 9 months, the mean DMFS (SD) increments in the C, GXF, GXFS, and GX groups were 3.8 (5.7), 2.5 (3.2), 3.5 (4.8), and 3.4 (5.5), respectively. The percentage of subjects with bleeding gingival units had decreased from initial to final values as follows: C, 81–38; GXF, 88–42; GXFS, 89–56; GX, 89–37. The number of lactobacilli and mutans streptococci in saliva remained virtually unchanged throughout the study. For caries increment and gingival bleeding, the differences between groups were not statistically significant. The chlorhexidine-fluoride combination tended to prevent caries, but the effect on gingival bleeding and salivary counts of mutans streptococci and lactobacilli was negligible.     

Morbid Obesity,Gastric Plication and a Severe Neurological Deficit

ABSTRACT. SomerH, BergstrÖm L, Mustajoki P, Rovamo L. (Department of Neurology, Third Department of Internal Medicine and Children's Hospital, University of Helsinki, Helsinki, Finland.) Morbid obesity, gastric plication and a severe neurological deficit. A 39-year-old man had protracted vomiting after gastric plication for morbid obesity. Within three months he lost 53 kg in weight and developed neuromuscular weakness, especially in the lower extremities. Clinical and laboratory studies suggested both radicular and peripheral neuropathy. One year later the condition was only marginally improved: he took only few steps unsupported. The apparent etiology is malnutrition but the primary cause remained unknown.     

Labetalol does not alter the placental and fetal blood flow or maternal prostanoids in pre-eclampsia

Summary. The effect of intravenously administered labetalol (1 mg/kg) on placental and fetal blood flow was studied in 13 pre-eclamptic women. Although the maternal blood pressure decreased, no changes occurred in the blood flows in the intervillous space, the umbilical vein or the fetal descending aorta, nor did the indices of peripheral vascular resistance in the fetal aorta change, but the placental vascular resistance did decrease. Labetalol had no effect on prostacyclin or thromboxane A₂ as measured by urinary 6-keto-prostaglandin F_1α and serum thromboxane B₂ respectively. These findings are clinically relevant since they suggest that labetalol reduces maternal blood pressure without interfering with the placental or fetal blood flow.     

Glucose Intolerance and Insulin Resistance Accompany Immobilization

ABSTRACT. Physical activity is known to increase glucose tolerance and insulin sensitivity. To examine the influence of physical inactivity on insulin sensitivity, we measured oral glucose tolerance (OGTT) and insulin response to glucose in 18 patients immobilized to bed for six weeks after acute spine fracture. The results were compared to those of nine chronically immobilized spinal cord injury patients and to eight healthy mobile controls. During the first week after trauma both glucose and insulin responses in the OGTT were two- to three-fold above normal (p<0.01). The index of insulin resistance (glucose area × insulin area) was seven-fold greater than in healthy controls (p<0.001). After three weeks' immobilization insulin resistance had declined by 30–35% (p<0.05) being then at the level observed in chronically immobilized subjects. After remobilization the insulin resistance was further decreased but remained still 2.3 times higher than in controls. Thus, trauma causes a manifold increase in insulin resistance, which is reduced but not normalized during the subsequent immobilization and remobilization.     

NUCLEAR MORPHOMETRY AS A PREDICTOR OF DISEASE OUTCOME IN GASTRIC CANCER

Nuclear morphometric features including nuclear area (NA), nuclear perimeter (PE), their variation (SDNA, SDPE), and mean largest and smallest nuclear diameters (Dmax, Dmin) were examined in 116 patients with stage I–II gastric adenocarcinoma. Morphometric measurements were compared with tumour differentiation, depth of invasion (pT), lymph node status (pN), and Lauren classification. All morphonuclear features were significantly larger in the intestinal type than in the diffuse type of cancer. None of the morphometric variables was related to TNM status or tumour size. Nuclear area and perimeter and their variation were closely related to survival in univariate analysis, patients with small and regular nuclei surviving longer. In the multivariate analysis, pT, pN, perineural invasion, and the standard deviation of nuclear perimeter (SDPE) were independent predictors of survival. Nuclear morphometry is a quantitative, objective, and highly reproducible method of revealing malignant features in several neoplasms. The results of the present study suggest that nuclear morphometric data may help in defining prognosis in gastric cancer. © 1997 by John Wiley & Sons, Ltd.     

Comparison of polyclonal and monoclonal antibodies to Actinomyces and Arachnia species

Abstract – Polyclonal (PoAbs) and monoclonal (MoAbs) antibodies were produced to Actinomyces israelii serotypes 1 and 2, to Actinomyces naeslundii, and to Arachnia propionica, and their specificities were studied by an enzyme immunoassay (EIA). All PoAbs except those to A. propionica reacted also with at least one other Actinomyces species. Only the MoAb to A. naeslundii proved to be more specific than the corresponding PoAbs. This MoAb did not cross-react with other Actinomyces or Arachnia species, nor with any other anaerobic or aerobic bacteria studied by inhibition EIA. Immunoblotting studies indicated that the antibody specific to A. naeslundii is directed against a large molecular weight antigen (>150 kd), probably polysaccharide in nature. The produced PoAbs and MoAbs can be used for further analyses of the antigenic determinants of different Actinomyces and Arachnia species.     

Skin morphology in porphyria cutanea tarda does not improve despite clinical remission

Five patients with porphyria cutanea tarda (PCT) were treated with a prolonged, low-dose chloroquine regimen (250 mg twice weekly). Their skin symptoms disappeared, and the abnormal urinary porphyrin excretion normalized during treatment periods ranging from 6 to 17 (mean 11.8) months. The morphology of the skin was studied by light and electron microscopy methods before treatment, at the onset of remission, and 6-12 months later. In samples taken during remission, the PAS-positive thickening of the superficial dermal vessels was comparable to that before treatment. In electron microscopy, the vessels showed thickening because of reduplication of the basal lamina and perivascular deposition of amorphous material; no consistent changes were found during remission. The results show that the histopathological changes of the skin in PCT are of chronic nature, and are probably irreversible.     

Effect of fluconazole dose on the extent of fluconazole-triazolam interaction

1 Azole antimycotics interact with the short acting hypnotic triazolam. The effect of fluconazole dose on the extent of fluconazole-triazolam interaction was investigated in a double-blind, randomized cross-over study of four phases.
2 Eight healthy volunteers received either 50  mg, 100  mg or 200  mg (400  mg on day 1) of fluconazole or placebo orally once a day for 4 days. On day 4, they took a 0.25  mg oral dose of triazolam, after which plasma samples were collected and pharmacodynamic effects measured for 18  h.
3 The mean area under the triazolam concentration-time curve (AUC) was increased 1.6-, 2.1- and 4.4-fold ( P <0.001) by fluconazole 50  mg, 100  mg and 200  mg, respectively. The increase in the elimination half-life of triazolam ( t _½,z) varied from 1.3-fold (fluconazole 50  mg, P <0.05) to 2.3-fold (fluconazole 200  mg, P <0.001). The peak concentration of triazolam was also increased significantly during all fluconazole phases; more than twofold by fluconazole 200  mg ( P <0.001).
4 The pharmacodynamic effects of triazolam were increased significantly ( P <0.05) by fluconazole 100  mg and 200  mg.
5 Even a small 50  mg daily dose of fluconazole can interact with triazolam and the extent of the interaction increases with increasing fluconazole dose. When triazolam is used concomitantly with fluconazole 50–200  mg, the dose of triazolam should be reduced, accordingly. Simultaneous use of triazolam with higher fluconazole doses should be avoided.