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Biomedical subjects

A Fasth

Publications and source records attributed to A Fasth.

At least 55 records · Page 3Linked to original sources
PubMed1995

Craniofacial structure in children with juvenile chronic arthritis (JCA) compared with healthy children with ideal or postnormal occlusion.

The aim of the present study was to evaluate the influence of condylar destruction on the craniofacial growth of children with juvenile chronic arthritis (JCA) and to compare their craniofacial structure with that of healthy children with ideal or postnormal occlusion. Thirty-five children (7 to 16 years) affected by JCA were compared with 136 children (7 to 16 years) with normal facial structure and occlusion (ideal group) and 62 children (7 to 12 years) with postnormal occlusion (postnormal group). Panoramic radiographs and lateral cephalograms were taken to detect condylar lesions and analyze facial structure. Multiple regression analysis was applied to test the possible relationships between the groups. The present study largely confirms earlier findings that the JCA group has a characteristic craniofacial structure. Their structure differed not only from the facial characteristics of children with ideal, but also to some extent, from children with postnormal occlusion. Furthermore, the craniofacial structure of children with JCA varied greatly, and radiographs showed that the most extreme craniofacial changes, particularly the mandibular structure, were associated with condylar lesions. In conclusion, the arthritic condylar lesions seem to be the main etiologic reason for the altered facial structure and changes in the occlusion in children with JCA. Influences of other factors, such as masticatory muscular function, are also discussed.

Adolescent↗
PubMed1995

Kawasaki disease in Sweden: incidence and clinical features.

The incidence and clinical pattern of patients with Kawasaki disease in Sweden and the outcome of treatment with i.v. immunoglobulin (Sandoglobulin) and aspirin were examined in a national prospective study over a 2-year period. Cases not referred to the study were identified by inquiry. Ninety-nine children were diagnosed as having Kawasaki disease. The annual incidence rate was calculated to be 2.9 per 100,000 in children younger than 16 years of age and 6.2 per 100,000 in children younger than 5 years of age. The median age of our patients was 2.2 years and the male-to-female ratio was 2.3:1. Cardiac ultrasonography revealed abnormalities in 33% of all patients, and 14% of cases in the prospective study had pronounced abnormalities. In most cases (91%) treatment had a prompt effect on fever and morbidity in general, and side effects were mild. Two infants, identified from the inquiry, died from rupture of an aneurysm in the coronary artery in the acute phase of the disease. The risk of cardiac involvement is obvious and emphasizes the importance of early diagnosis and treatment.

Adolescent↗
PubMed1995

A prospective, double-blind, placebo-controlled trial of i.v. immunoglobulin and trimethoprim-sulfamethoxazole in children with recurrent respiratory tract infections.

In a prospective, double-blind, placebo-controlled study of iv immunoglobulin (IVIG) and trimethoprim-sulfamethoxazole (TMS), 130 children less than 8 years of age were referred for recurrent bacterial respiratory tract infections, as judged by the referring physician. Of the 130 children referred, only 24 continued to have bacterial respiratory infections over a 4-month observation period. They were randomized and 23/24 treated for 4 months during the winter-spring season. The 7 children given placebo for both IVG adn TMS continued to have bacterial respiratory infections, while 14 of 16 children given active therapy with either IVIG or TMS became infection-free (p=0.002). No relation to IgG subclass level or between the two modalities of treatment was found. We concluded that most infection-prone children suffer from viral infections and are given antibiotics unnecessarily. Of the small group of children that have documented, repeated bacterial infections, prophylactic therapy with either IVIG or TMS can substantially diminish the number of infections.

Antibiotic Prophylaxis↗
PubMed1995

Characterization of nine novel mutations in the CD40 ligand gene in patients with X-linked hyper IgM syndrome of various ancestry.

X-linked immunodeficiency with hyper-IgM (HIGMX-1) is a rare disorder caused by defective expression of the CD40 ligand (CD40L) by activated T lymphocytes, resulting in inefficient T-B cell cooperation and failure of B cells to undergo immunoglobulin isotype switch. In the present work, we describe nine patients of various ancestry who bear different mutations in the X chromosome-specific CD40L gene. Two of the mutations were nonsense mutations, one each resulting in premature stop codons at amino acid residues 39 and 140. Three patients had single point missense mutations, one each at codons 126, 140, and 144. Another patient had a 4-bp genomic deletion in exon 2, resulting in a frameshift and premature termination. Three patients showed insertions, one each of 1, 2, and 4 nt, probably because of polymerase slippage, resulting in frameshift mutation and premature termination. Overall, these observations confirm the heterogeneity of mutations in HIGMX-1. However, the identification of two patients whose mutation involves codon 140 (previously shown to be altered in two other unrelated subjects) suggests that this may be a hotspot of mutation in HIGMX-1. In two additional patients with clinical and immunological features indistinguishable from canonical HIGMX-1, no mutation was detected in the coding sequence, in the 5' flanking region, or in the 3' UTR.

Base Sequence↗
PubMed1995

The natural history of juvenile chronic arthritis: a population based cohort study. I. Onset and disease process.

OBJECTIVE: To study the natural history and the disease process in a population based cohort of patients with juvenile chronic arthritis (JCA) who were in the process of being transferred from pediatric to adult rheumatology care. METHODS: From a prospective population based epidemiological study in southwestern Sweden the cohort of patients with JCA born from 1968 through 1972 were investigated after a median disease duration of 7.1 years. The study cohort constituted of 124 patients with median age 17.7 years. RESULTS: At followup 49.2% patients still required medication, 20.2% had inactive disease and 30.6% were in remission. The highest risk of continuing disease activity was observed in patients with short disease duration (RR = 9.0) or very long duration (RR = 2.5) compared with those having medium long duration. Girls were 5 times more likely than boys to have continuing disease activity. Based on incidence data from the total population of the epidemiological study it can be estimated that an additional 100 patients with JCA, who were in remission in the beginning of the study, should be included in the present cohort. Thus a total of 60% of the patients with JCA had disease in remission and 70% were rid of disease activity when reaching adulthood in a population based setting. CONCLUSION: The selection of patients in studies of the natural history of JCA markedly influences the results, which may be one explanation for the divergent views of JCA as both a mild and a progressive disease.

Adolescent↗
PubMed1995

The natural history of juvenile chronic arthritis: a population based cohort study. II. Outcome.

OBJECTIVE: To investigate the outcome in a population based cohort of patients with juvenile chronic arthritis (JCA) who were in the process of being transferred from pediatric to adult rheumatology care. METHODS: The cohort of patients born in 1968 through 1972, recruited from a population based epidemiological study in southwestern Sweden, were called to a followup after a median disease duration of 7.1 years. The study group consisted of 124 patients with a median age of 17.7 years. The disability and discomfort dimensions were evaluated using the Childhood Health Assessment Questionnaire (C-HAQ). The impact of the disease on social life was evaluated by patients and parents. RESULTS: The median C-HAQ disability index was 0.19 with a range from 0 to 2.75 (maximum possible score = 3). Sixty percent of the patients indicated some difficulty in daily activities. Female sex and a polyarticular disease course were risk factors for disability. The strongest determinants for disability were continuing disease activity and a positive IgM rheumatoid factor. Social impact of the disease was strongly linked to a raised C-HAQ disability index. CONCLUSION: Even in a population based study of JCA, which includes many mild cases, the majority of patients experienced some difficulty in daily activities when judged by themselves. This underlines the necessity to use the patient's own values in outcome studies, rather than the physician's. Further development of internationally accepted, standardized instruments to evaluate the handicap dimension of childhood arthritis is called for.

Activities of Daily Living↗
PubMed1995

[Bone marrow transplantation in primary immunodeficiency syndrome and in osteopetrosis].

The designation primary immunodeficiency embraces a multiplicity of diseases of which only the more severe constitute indications for BMT (bone marrow transplantation)--e.g. severe combined immunodeficiency (SCID), Wiskott-Aldrich syndrome, familial haemophagocytic lymphohistiocytosis and malignant osteopetrosis. In cases of immunodeficiency, the outcome of BMT is strongly dependent on the patient's age, clinical status at transplantation and the type of immunodeficiency. In children with SCID who undergo BMT during the first few months of life, lasting cures can be obtained in almost 100 percent of the cases, whereas there is only a 15 percent probability of success if the child is older, infected, cannot undergo cytostatic preconditioning or cannot be given T-cell depleted bone marrow.

Bone Marrow Transplantation↗
PubMed1994

Serum procollagen type III is an early and sensitive marker for veno-occlusive disease of the liver in children undergoing bone marrow transplantation.

Veno-occlusive disease of the liver (VOD) is a life-threatening complication occurring in patients undergoing bone marrow transplantation (BMT). Although clinical signs and laboratory parameters such as elevation of serum bilirubin often suggest this condition, it would be useful to identify early biochemical markers for VOD. Fibrous alterations in the hepatic venules and small lobular veins occur during development of VOD; these changes are accompanied by the deposition of types I and III collagen in the liver tissue. Since the N-terminal propeptide of type III procollagen (PIIINP) is a sensitive marker of liver and lung fibrosis, we undertook a study to evaluate the usefulness of measurements of serum PIIINP in children with VOD. Seven of the 28 children who underwent BMT, both allogenic and autologous, developed VOD. All seven had an increase of more than 100 ng/mL in the serum PIIINP level, whereas only one of the remaining 21 children not affected by VOD had an increment of PIIINP more than 100 ng/mL (P = .0001). The levels of serum PIIINP were higher in the VOD group during the follow-up period of up to 91 days after BMT. The elevation of PIIINP also occurred at a stage of the disease usually preceding any other laboratory or clinical signs of VOD. Serum concentration of PIIINP thus seems to be of value as an early marker for VOD in children undergoing BMT.

Adolescent↗
PubMed1994

Bone marrow transplantation (BMT) in Europe for primary immunodeficiencies other than severe combined immunodeficiency: a report from the European Group for BMT and the European Group for Immunodeficiency.

Bone marrow (BM) transplantations performed between 1977 and 1991 at 13 European centers in 149 patients with 11 different primary immunodeficiency (ID) diseases (excluding severe combined immunodeficiency) were analyzed retrospectively. Overall survival among recipients of HLA genetically identical BM (n = 56) was 66%. Since October 1985, the date of a previous survey, a significant improvement in survival has been achieved in most ID diseases (overall survival, 81.5% v 51.7%; P < .01), primarily because of a decrease in the frequency of infectious complications. In long-term survivors, disease correction is excellent, with minimal sequelae in most patients. In 22 patients who received closely matched BM (ie, from phenotypically identical related donors, matched unrelated donors, or one HLA-ag-mismatched related donors), the survival rate (45.5%) was not significantly better than among 71 recipients of BM with 2 or 3 mismatched HLA antigens (38%). In the latter group, favorable outcome was associated with younger age, with transplantation since October 1985 (47% v 25%; P < .0001), and with a diagnosis of leukocyte adhesion deficiency. The improvement in outcome was mainly because of a higher engraftment rate and a decrease in the frequency of infections, although Epstein-Barr virus-induced B-lymphocyte proliferative disorders occurred in 16 patients (mainly those with Wiskott-Aldrich syndrome), 10 of whom died. The improvement in engraftment corresponded to the introduction of treatment in vivo with anti-LFA-1 antibody to prevent rejection of T-cell-depleted grafts (74% engraftment and 45% survival in 38 treated patients versus 37.5% and 21%, respectively, in 24 untreated patients.

Adult↗
PubMed1994

Bone marrow transplantation for autosomal recessive osteopetrosis. A report from the Working Party on Inborn Errors of the European Bone Marrow Transplantation Group.

The outcomes of 69 patients who received allogeneic bone marrow grafts for autosomal recessive osteopetrosis in the period between 1976 and 1994 were analyzed retrospectively. Four patients received bone marrow transplants (BMT) without prior myeloablative conditioning; transient osteoclast function was demonstrated in one of them. Sixty-five patients received myeloablative pretreatment. Recipients of a genotypically human leukocyte antigen (HLA)-identical BMT had an actuarial probability for 5-year survival, with osteoclast function, of 79%; recipients of a phenotypically HLA-identical bone marrow graft from a related or unrelated donor, or one HLA-mismatched graft from a related donor, had an actuarial probability for 5-year survival, with osteoclast function, of 38%; patients who received a graft from an HLA-haplotype mismatched related donor had a probability for 5-year survival of only 13%. The main problems in haplotype-nonidentical BMT were graft failure and BMT-related complications such as sepsis, bleeding, and interstitial pneumonia. Osteoclast function developed in all patients with full engraftment. Recovery of osteoclast function was associated with severe hypercalcemia in 24% of the patients with engraftment, especially those older than 2 years of age. At the time of BMT, severe visual impairment was present in 35% of the patients; of the 15 patients who had visual impairment at the time that a successful BMT was performed, two had improvement after BMT (13%). Within the total group, one patient had neurodegeneration. Engraftment of healthy donor cells had no influence on the progression of that abnormality and BMT thus had no beneficial effect on this phenotype of osteopetrosis. In general, however, early BMT remains the only curative treatment for autosomal recessive osteopetrosis.

Actuarial Analysis↗
PubMed1994

Serum concentration of hyaluronan, IgM and IgA rheumatoid factors in a population based study of juvenile chronic arthritis.

Increased serum levels of hyaluronan (HA) have been found in patients with liver diseases, psoriasis, malignancies as well as in rheumatoid arthritis and osteoarthritis. In the two latter groups serum HA levels may reflect the extent of synovial involvement and inflammation, rather than only unspecific information. This study was performed to elucidate the value of measuring HA in children with juvenile chronic arthritis (JCA) and to study its relation to class specific rheumatoid factors (IgM Rf and IgA Rf). HA was analysed in sera from 271 patients with JCA and 130 controls using a radiometric assay and IgM Rf and IgA Rf were analysed using an enzyme immunoassay. The results were analysed in relation to JCA subgroup, disease activity and functional outcome. In patients and controls higher levels of HA were found in young children than in older. Raised levels of HA and IgM Rf were mainly found in children with polyarticular disease. Impaired functional outcome was related to raised HA levels. No correlation was found between HA levels and IgM Rf or IgA Rf. Measurement of HA levels in JCA is of no diagnostic value because of low sensitivity. Quantification of HA may, however, have prognostic value in a subgroup of patients belonging to the polyarticular subgroup.

Adolescent↗
PubMed1993

Adverse reactions in healthy and immunocompromised children under six years of age vaccinated with the Danish BCG vaccine, strain Copenhagen 1331: implications for the vaccination policy in Sweden.

A retrospective analysis of the adverse reactions reported between 1979 and 1991, in the 139,000 children under six years of age vaccinated in Sweden with the Danish BCG vaccine, strain Copenhagen 1331, showed an incidence of 1.9 per 1000 vaccinated children. Regional lymphoglandular swellings and/or abscesses were most commonly reported in 1.4 per 1000. Serious, disseminated, BCG infections developed in four infants vaccinated neonatally. Three of the infants suffered from severe, combined, immunodeficiency syndrome, undiagnosed at the time of vaccination. The incidence of severe, combined, immunodeficiency syndrome was higher in the BCG-vaccinated population (4 per 100,000 infants vaccinated within a year of their births), compared with all newborns in Sweden (1 per 100,000). The mean age at the onset of symptoms was 2.4 months for the seven non-BCG-vaccinated infants versus 1.3 months for the four BCG-vaccinated ones, while the immunodeficiency syndrome was diagnosed at an average age of 7.6 months in those who were not vaccinated versus 5.3 months in those BCG-vaccinated. It is recommended that the selective BCG vaccination of infants at high risk of exposure to tuberculosis should be postponed to six months of age to reduce the risk of inoculating infants suffering from immunodeficiency syndromes.

Age Factors↗
PubMed1992

Epidemiology of juvenile chronic arthritis in southwestern Sweden: a 5-year prospective population study.

Previous epidemiological studies of juvenile chronic arthritis (JCA) report divergent results owing to differences in diagnostic criteria, patient retrieval, and study designs. To investigate incidence and prevalence of JCA in a total population, this prospective survey was performed in southwestern Sweden between 1984 and 1988. Cases were identified using the European League Against Rheumatism criteria for JCA and were reported annually from eight pediatric departments and local pediatricians in the studied area. During the 5 years, 213 new cases of JCA were found, corresponding to an incidence of 54.6 per 100,000 children younger than 16 years of age. The average annual incidence was 10.9 per 100,000. The peak incidence rate, 18.3 per 100,000 was found in girls 0 through 3 years old. The lowest incidence rate, 6.4 per 100,000, was found among boys 12 through 15 years old. In December 1988, 334 cases of JCA were recorded, giving a prevalence of 86.3 per 100,000. When patients in remission were omitted the prevalence was 64.1 per 100,000. The monoarticular+pauciarticular onset type constituted 68.3% of the prevalence cases, while 21.9 were polyarticular and 6.6% had systemic onset. To avoid underestimation of incidence and prevalence, and to get a correct picture of disease patterns, epidemiological surveys of JCA should be population-based rather than referral center-based. Further descriptive studies of JCA in different well-defined geographic areas are important to make valid comparisons. Such comparisons could give clues to etiological factors, both genetic and environmental.

Adolescent↗