[Therapy of severe aplastic anemia (SAA) by bone marrow transplantation (BMT) or immunosuppression?].
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Biomedical subjects
Publications and source records attributed to M Kos.
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Fifteen patients with transfusion-dependent severe aplastic anemia (SAA) were treated with combined immunosuppression consisting of horse-antithymocyte globulin (ATG; Atgam, Upjohn) and high-dose 6-methylprednisolone (MP). Oxymetholone was scheduled for 2 years but was discontinued in 7 patients after 10-385 days due to liver toxicity. Serious side effects usually seen in ATG monotherapy were rare during combined immunosuppression. Currently 12 of 15 patients are alive 110-1,275 days (median 475.5) after start of treatment. One patient has received too short treatment to be evaluated. All the others are transfusion-independent. Three patients died; two from septicemia before hemopoietic recovery could be expected and one after relapse. Our results confirm that the addition of high-dose MP abrogates the side effects of ATG monotherapy, and the addition of MP does not counteract, but rather enhances the beneficial effect of ATG in SAA. We recommend combined immunosuppressive treatment with ATG and high-dose MP as a highly feasible, safe and effectful therapy for patients with transfusion-dependent SAA.
The activity of peroxisomal enzymes was studied in human liver and cultured human skin fibroblasts in relation to the finding (Goldfischer, S. et al. (1973) Science 182, 62-64) that morphologically distinct peroxisomes are not detectable in patients with the cerebro-hepato-renal (Zellweger) syndrome. In homogenates of liver from the patients, dihydroxyacetone phosphate acyltransferase, a membrane-bound peroxisomal enzyme, is deficient (Schutgens, R.B.H., et al. (1984) Biochem. Biophys. Res. Commun. 120, 179-184). In contrast, there is no deficiency of the soluble peroxisomal matrix enzymes catalase, L-alpha-hydroxyacid oxidase and E-aminoacid oxidase. Catalase is also not deficient in homogenates of cultured skin fibroblasts from the patients. The results of digitonin titration experiments showed that in control fibroblasts at least 70% of the catalase activity is present in subcellular particles distinct from mitochondria or lysosomes. In contrast, all of the catalase activity in fibroblasts from Zellweger patients is found in the same compartment as the cytosolic marker enzyme lactate dehydrogenase.
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Projectional tests using pictures, drawings, story-telling and puppets induce children to exhibit their conflicts within the family. Among such drawing tests, "The Enchanted Family" asks the child to put the members of a family under the spell of a magician, without any limits being set on the child's imagination in the choice of objects to be drawn. The psychological content is then underlined by the child telling a story about the casting of the spell. Four thousand children and juveniles, of which 1562 had behavioural disturbances or were neurotically or psychosomatically ill, were examined with this test and 1225 of these were statistically evaluated for the formal aspects of their drawings. A group of healthy school children were evaluated as controls. In addition to the formal results, the depth-psychological results of the tests offered valuable pointers to the individual conflict situations of the test subjects, in particular in relation to the relationships within the family, the projections and identifications in "key situations" of the neurotic family behaviour patterns. This was demonstrated in individual case studies. The symbolism in the choice of animals receives specific interpretation. The use of this test in educational advisory practice and psychotherapy is recommended.
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A case control study of 59 women with subchorionic hematomas compared to 135 normally pregnant. Transvaginal ultrasound was used to image the pregnancy, and identify the site and size of the hematomas. Color flow Doppler was used to calculate velocity indices of the spiral arteries. More spontaneous abortions occurred in women with subchorionic hematomas (SCH). There was general correlation between gestational age, velocity indices, and hematoma size. There were 10 spontaneous abortions in the study group (17%) versus 9 (6.5%) in the controls (P = 0.02). Hematoma size did not affect outcome, but site did. Most hematomas associated with abortion were found in the corpus or fundus of the uterus, not in the supracervical area (P = 0.03). The presence of a hematoma did not affect the frequency of preterm delivery. In conclusion, subchorionic hematomas in early pregnancy are associated with an increased risk of spontaneous abortion. Flow disturbances are seen in the spiral arteries, but these are probably secondary effects. The critical factor is site of hematoma, not volume.
OBJECTIVE: Fetal echoic bowel can be a normal second trimester ultrasonographic finding which usually disappears by 20 weeks on serial sonograms. Recent studies have suggested a possible association of hyperechoic fetal bowel with chromosomopathies and cystic fibrosis. The aim of our study is to determine the incidence of chromosomopathies and cystic fibrosis mutations among the fetuses with isolated hyperechoic bowel. METHODS: Sixteen fetuses with isolated echoic bowel were detected: 13 fetuses < or =20 weeks gestation (group I) and 3 fetuses at 20-26 weeks gestation (group II). Cytogenetic studies were performed in all 16 cases and 11 families had deoxyribonucleic acid-based risk assessment for cystic fibrosis. The echogenity of bowel was that of surrounding bone. RESULTS: Two cases of trisomy 21 and 1 case of trisomy 13 were detected (18.7%). The other ultrasonographic markers begin to appear after 21 weeks gestation in fetuses with trisomy 13. Two of 3 pregnant women with pathological karyotype were younger than 35 years. One of 11 cases (9%) was found to be a heterozygote carrier for deltaF508 mutation. CONCLUSIONS: Isolated hyperechoic bowel in the second trimester was found to be associated with a significantly higher risk of fetal aneuploidy.
BACKGROUND: Our study was designed to determine bone mineral density (BMD) in patients beginning hemodialysis (HD) treatment, a possible correlation with the duration of renal failure prior to treatment, a possible correlation with the basic disease and the association with the concentration of intact parathormone (iPTH). METHODS: Our prospective clinical trial included 50 patients beginning HD treatment. Cortical bone mineral density (BMDc) was measured at the left femoral neck and trabecular bone mineral density (BMDt) in the region of the lumbosacral spine. Bone mineral density (BMD) was measured by quantitative digital radiography using a Hologic 2000 plus device belonging to the third generation of densitometers based on dual-energy X-ray absorptiometry. RESULTS: In patients (PTS) beginning HD, the average BMDc was 82 +/- 15% of BMDc in a healthy population of corresponding age and sex. The average BMDt was 91 +/- 16% of BMDt in a healthy population of corresponding age and sex. The difference was statistically significant (p < 0.05). There is a negative correlation between iPTH and BMDc r = -0.34 (p < 0.02). Patients with chronic glomerulonephritis (GN) had a statistically significantly higher BMDc (g/cm(2)) (p < 0.01) than those with analgetic nephropathy (AN). PTS with AN have lower BMDc (g/cm(2), %) (p < 0.02) and BMDt (p < 0.005) than the rest of the PTS, iPTH in PTS with AN is higher than in the rest of the PTS (p < 0.05). CONCLUSIONS: In PTS at the beginning of HD, BMD is lower than in healthy people of corresponding age and sex. This means that BMD already decreases prior to HD. BMDc was statistically significantly lower than BMDt (p < 0.00005). PTS with AN have lower BMD than those with GN and all remaining PTS. A negative correlation between iPTH and BMDc was found.
OBJECTIVE: Our purpose was to determine if the frequency of confined placental mosaicism in newborns with unexplained intrauterine growth retardation (IUGR) was higher compared with infants with appropriate growth in utero and the outcome of these pregnancies. STUDY DESIGN: A total of 20 cases with unexplained IUGR and 20 cases with appropriate growth for gestational age has been studied. Amnion, chorion and villi biopsy specimens were obtained from growth-retarded cases and controls at delivery. Cord blood specimens for 48-hour lymphocyte cultures were obtained from all infants with IUGR. RESULTS: Karyotype analysis revealed confined placental mosaicism in two of 20 (10%) cases with IUGR. In one growth retarded case and one appropriate growth for gestational age case, mosaicism was also confirmed in the amnion. Cytogenetic analysis from peripheral blood of newborns showed normal karyotype in all cases. Three pregnancies in the group of fetuses with IUGR (15%) ended with fetal death compared with normal fetal surveillance of all cases from the control group. CONCLUSION: Confined placental mosaicism was detected two times more frequently from placentas of growth- retarded infants compared with those of newborns with appropriate growth. The fetal loss was significantly higher in the group of cases with IUGR compared with the control group.
OBJECTIVE: To analyze the potential of three-dimensional power Doppler sonography in morphologic and functional assessment of the fetus and placenta. METHODS: Review of the recent literature on three-dimensional sonography in early pregnancy and in the second and third trimester. RESULTS: Three-dimensional sonography plays an important role in obstetrics predominantly for assessing fetal anatomy. Multiplanar images and rotation of the object allow systematic review of anatomic structures, such as limb buds, cerebral cavities, cord insertion, stomach, and bladder. Using this modality, volumes of the gestational sac, yolk sac, and fetal organs can be obtained easily. Three-dimensional power Doppler sonography has the potential to study the intervillous and placental circulation and evaluate the development of the embryonic and fetal cardiovascular system. CONCLUSIONS: Three-dimensional ultrasound imaging complements pathologic and histologic evaluation of the developing embryo, giving rise to the new term "three-dimensional sonoembryology." It is evident that three-dimensional ultrasonography improves the visualization of the normal and abnormal fetal anatomy giving a realistic impression of the extent of the defects. These data are useful not only for obstetricians but also for pediatricians and pediatric surgeons.
From 1980 to 1989 clear-cell carcinoma was diagnosed in 66(4.2%) out of 1562 adenocarcinomas of the female genital tract: in the ovary, 2.9% in the endometrium, 9.8% in the uterine cervix; 4.5% in the vagina, and 1.9% in the Fallopian tube. The diagnosis was made at clinical stage I in 26.9% of cases located in the ovary, 62.5% in the endometrium, and 42.8% in the uterine cervix. At stages III and IV the highest percentage of cases was diagnosed in the ovary -42.3% and 26.9%, respectively. The material was divided into the histological subtypes, and the glandular papillary was found in 54.5%, the tubulocystic in 31.8% and the solid in 13.6% of cases. The tubulocystic subtype showed the best prognosis, with the 50% five-year survival rate, while the glandular papillary subtype had 38.1%, and the solid subtype a 14.3 five-year survival rate was 66.6% for the ovary, 85% for the endometrium and 75% for the uterine cervix. For all other stages together (II, III, IV), the five-year survival rate was 0% for the ovary, 20% for the endometrium and 0% for the uterine cervix.
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