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Biomedical subjects

L Holmberg

Publications and source records attributed to L Holmberg.

At least 235 records · Page 13Linked to original sources
PubMed1988

Frequency of different types of von Willebrand's disease in the GDR.

The frequency of different types of von Willebrand's disease (vWD) was studied in the southern part of the GDR and during investigations of relatives of already diagnosed patients. Among 111 patients diagnosed, vWD was found to be type I in 85 cases, type II in 13 cases and severe recessive type in 13 cases. The patients with type I belonged to 46 different families. Two families with type II had the II A variant and another a variant similar to II C. No patients with type II B were diagnosed. The incidence of severe recessive vWD was estimated to be lower than in Sweden but higher than in Italy and France.

Electrophoresis, Agar Gel↗
PubMed1988

Plasma concentrations of C4b-binding protein and vitamin K-dependent protein S in term and preterm infants: low levels of protein S-C4b-binding protein complexes.

We have determined the plasma concentrations of protein S and C4BP in 25 term and 26 preterm infants by radioimmunoassay. Both the total concentration and the concentration of free protein S were quantified. The concentration of C4BP was very low in preterm infants (mean 6% of the adult level). In term infants, the level had increased to a mean of 18%. Total protein S was decreased both in preterm and term infants, 4.0 mg/l and 6.8 mg/l respectively, as compared to the mean adult concentration, 20.6 mg/l. In preterm infant plasma, free protein S was the predominant (85%) form, probably due to the very low C4BP level. In plasma from term infants, free protein S represented 68% of the total protein S, the corresponding value in adult controls being 37%. The plasma concentration of free protein S in preterm and term infants was 3.3 mg/l and 4.6 mg/l, respectively (mean adult value 7.6 mg/l). These results demonstrate that, while the total protein S concentration in preterm and term infants was very low in comparison to the adult level, the difference in the concentration of the anticoagulant, active, free form of protein S between infants and adults was less pronounced.

Adult↗
PubMed1988

Prognostic significance of the Ackerman classification and other histopathological characteristics in breast cancer. An analysis of 1,349 consecutive cases with complete follow-up over seven years.

Survival rates during a follow-up period of more than seven years were analyzed in 1,349 women with breast cancer in relation to the histo-pathological classification of female breast cancer proposed by Ackerman and to other commonly used histo-pathological criteria, including the axillary node status. The information was collected prospectively during a case-control study. Major emphasis was placed on multivariate evaluation. In analyses based on the histo-pathological findings in the mastectomy specimen alone, the Ackerman grouping was found to be of prognostic value, but apart from nuclear polymorphism the other recorded characteristics (histo-pathological type, histological grading, lymphocyte infiltration, and sinus histiocytosis) did not give any prognostic information. When the axillary node status was included in the multivariate models, the presence of axillary metastases correlated well with the prognosis and the Ackerman classification provided no significant additional information. The results indicate that in cases where the histopathological axillary status is known, little additional prognostic information can be gained from traditional histo-pathological evaluation over and above this status. However, the Ackerman classification and the degree of nuclear polymorphism separate distinct prognostic groups with the same degree of difference in observed survival rates as one discriminated by the axillary node status.

Breast Neoplasms↗
PubMed1988

Diagnosis and differentiation of von Willebrand's disease type II.

Out of 113 patients with vWD 15 were of type II. The basic test programme included F VIII:C, vWF-Ag, RCF and BT (Ivy). All type II patients had relatively high vWF-Ag and low RCF values. CIEP proved increased anodal migration velocity. IRMA testing of vWF:Ag was showing narrow correlation with RCF. Thrombocyte count was unchanged after DDAVP infusion and RIPA was always diminished. Finally multimeric sizing was done in 6 patients. From the completely diagnosed patients 5 are of type IIA and one seems to have type IIC. Nobody of the 15 had the characteristics of type IIB.

Antigens↗
PubMed1988

Hereditary X-linked thrombocytopenia maps to the same chromosomal region as the Wiskott-Aldrich syndrome.

Hereditary X-linked thrombocytopenia occurs either as isolated thrombocytopenia or as a part of the Wiskott-Aldrich syndrome (WAS). We studied X-linked thrombocytopenia in a family with eight affected male members, none of whom exhibited the increased susceptibility to infection that occurs in WAS. We found a significant linkage between thrombocytopenia and DXS 146, a marker on the proximal part of the short arm of the X-chromosome. WAS has previously been mapped to the same chromosomal region. The present findings indicate that X-linked thrombocytopenia and WAS are closely related and may even be caused by different mutations of the same gene. This view is supported by our findings of atopic symptoms and minor deviations in immunologic variables among some of the affected subjects.

Adolescent↗
PubMed1987

What is the optimum interval between mammographic screening examinations? An analysis based on the latest results of the Swedish two-county breast cancer screening trial.

Further results are presented from the Swedish two-county breast cancer screening trial. The reduction in the rate of advanced cancers and of breast cancer mortality in the group allocated to screening when compared to the control group has accelerated with a further year of follow-up. Mortality due to other causes and the rate of other cancers remains similar in the two groups. Attention has been focused on the rate at which cancers start re-emerging among women with negative mammograms. Among women over 50 years of age at entry to the study, relatively few interval cancers are seen in the first two years after a screening test; in the third year the rate rises to nearly 50% of the comparable rate in the control group. Among women aged 40-49 years at entry, by contrast, the rate of interval cancers even in the first post screening year is nearly 40% of that in the controls and in the second year nearly 70%. In older women in the group allocated to screening, much of the breast cancer mortality comes from the refusers and little from the interval cancers; in younger women the picture is reversed. The implications for screening policy, including the interscreening interval are discussed.

Adult↗
PubMed1987

Type IIB von Willebrand's disease with probable autosomal recessive inheritance and presenting as thrombocytopenia in infancy.

von Willebrand's disease (vWD) is a congenital bleeding disorder that exists in two main forms. In the classic form, type I, the concentration of the von Willebrand factor (vWF) in plasma is decreased. In type II vWD, the vWF is structurally altered. Type II can be further divided into at least six subtypes (A, B, C, D, E and F). In type IIB the vWF, in contrast to other variants of vWD, shows an increased affinity for platelets. IIB vWD is generally believed to be inherited in an autosomal dominant manner. We describe two families with three affected children in whom an autosomal recessive inheritance is more likely. Thrombocytopenia, constant or variable, was present from early infancy in all three cases. Type IIB vWD should thus be included in the differential diagnosis of congenital thrombocytopenia.

Antigens↗
PubMed1987

Corticosteroid treatment of maternal ITP and risk of neonatal thrombocytopenia.

Maternal ITP is associated with a high risk of thrombocytopenia in the fetus and newborn infant. Methods of predicting this risk include determinations of maternal platelet count and platelet-associated IgG prior to delivery. In this report we show that prednisolone treatment normalized maternal platelet count and platelet-associated IgG without eliminating circulating platelet antibodies, which thus crossed the placenta and rendered the infant severely thrombocytopenia. Determination of maternal circulating platelet antibodies may be the best way of predicting infant thrombocytopenia, especially if the mother has received corticosteroids.

Adult↗
PubMed1987

Aminolaevulinate dehydratase porphyria in infancy. A clinical and biochemical study.

Homozygous deficiency of aminolaevulinate dehydratase (porphobilinogen synthase, EC 4.2.1.24) was diagnosed in a small child. The clinical presentation was unique since severe symptoms were already present in the neonatal period. The patient, a boy, now three-years old, had recurrent attacks of pain, vomiting, hyponatraemia and symptoms of polyneuropathy engaging motor functions including respiration. The clinical course of the disease from birth on is related, as are the results of various attempts at therapy. The patient excreted large amounts of 5-aminolaevulinic acid and coproporphyrin and minor amounts of porphobilinogen in the urine. Faecal excretion of coproporphyrin and harderoporphyrin was increased as was erythrocyte porphyrin concentration. Diagnosis was established by the finding that erythrocyte aminolaevulinate dehydratase activity was less than 5 per cent of normal in the patient and between 26 and 51 per cent of normal in both the parents, the grandfathers and a sibling. The activity of the enzyme could not be restored by the addition of dithiothreitol (10(-3) mol/l) alone, or in combination with zinc or manganese in varying concentrations. The enzyme Km did not differ between affected and nonaffected members of the family.

Aminolevulinic Acid↗
PubMed1987

Enzymatic defect in a child with hereditary hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency: immunochemical studies.

Immunochemical studies of the enzyme defect in the first reported child with acute hepatic porphyria due to homozygous delta-aminolevulinic acid dehydratase deficiency are described. This enzyme activity was markedly decreased (approximately 2% of the normal control level) in the proband, a 3-year-old boy, and intermediately decreased (23% to 57%) in both parents, in both grandfathers, and in a sister, but it was normal in two siblings and in both grandmothers. In contrast to the profound decrease in delta-aminolevulinic acid dehydratase activity, the immunoreactive enzyme protein in the child's erythrocytes was decreased to only 28% of the normal control level, suggesting the presence of positive cross-reactive material. In other family members with abnormally decreased delta-aminolevulinic acid dehydratase activity, and in the proband immediately after transfusion of normal RBCs, the positive cross-reactive material was not detectable. The immunochemical and enzyme activity data support the idea that delta-aminolevulinic acid dehydratase deficiency in this porphyric child is associated with the production of a catalytically abnormal enzyme protein.

Blood Proteins↗
PubMed1986

Demands on surgical inpatient services after mass mammographic screening.

The changes in the demand for surgical inpatient care created by mammographic screening for breast cancer were analysed by comparing two counties, one with and one without a mass screening campaign. A comprehensive computerised register of inpatient care in the region was used. The results indicate that population based screening offered to women above 40 years and repeated every two to three years will increase the number of operations required for breast cancer and inpatient days by at least 150% during the initial screening round. During the second round the figures tend to return to previous levels. Of decisive importance for the demands on health service resources are the specificity of screening, the duration of the first screening round, and the age groups included.

Adolescent↗
PubMed1986

The relation between survival and age at diagnosis in breast cancer.

We analyzed the relation between age at diagnosis and relative survival (ratio of observed to expected survival) in 57,068 women in Sweden in whom breast cancer was diagnosed in 1960 to 1978 (about 98 percent of all cases). Women who were 45 to 49 years old had the best prognosis, with a relative survival exceeding that of the youngest patients (less than 30 years) by 7.6 to 12.9 percent at different periods of observation. Relative survival declined markedly after the age of 49--particularly in women aged 50 to 59--and the oldest women (greater than 75) had the worst rate. The difference in relative survival between those older than 75 and those 45 to 49 increased from 8.6 percent at 2 years to 12.2, 20.3, and 27.5 percent after 5, 10, and 15 years of follow-up, respectively. The long-term annual mortality rate due to breast cancer approached 1 to 2 percent at the premenopausal ages but exceeded 5 percent throughout the period of observation in the oldest age group. An understanding of the biologic basis for the complex relation between age and prognosis might provide a better understanding of the natural history of breast cancer in women.

Adult↗
PubMed1986

Platelet-associated IgG in childhood idiopathic thrombocytopenic purpura: measurements on intact and solubilized platelets and after gammaglobulin treatment.

An immunoradiometric assay was developed for determining platelet-associated IgG (PAIgG) both on intact and solubilized platelets. In 20 healthy subjects PAIgG was 0.28 +/- 0.20 ng/10(6) platelets and 2.2 +/- 1.1 ng/10(6) platelets on intact and on solubilized platelets, respectively. 13 children with acute ITP all had increased concentrations of PAIgG, but no correlation was found between the severity of thrombocytopenia and PAIgG concentrations, either on intact or on solubilized platelets. Neither was there any correlation of the increase in PAIgG between intact and solubilized preparations. 3 out of 4 children who received high-dose i.v. gammaglobulin showed a concomitant normalization of the platelet count and of the PAIgG concentration both on intact and lyzed platelets. The remaining child did not respond to gammaglobulin and continued to have abnormal PAIgG.

Blood Platelets↗