| 1. |
- Bergfors, Elisabet, 1945, et al.
(author)
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Nineteen cases of persistent pruritic nodules and contact allergy to aluminium after injection of commonly used aluminium-adsorbed vaccines.
- 2005
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In: European journal of pediatrics. - : Springer Science and Business Media LLC. - 0340-6199 .- 1432-1076. ; 164:11, s. 691-7
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Journal article (peer-reviewed)abstract
- Rare cases of persistent pruritic nodules, sometimes associated with aluminium (Al) allergy, have been reported after the use of several Al adsorbed vaccines. During vaccine trials in the 1990s a high incidence of pruritic nodules (645 cases/76,000 recipients), in 77% associated with Al allergy, was observed after the administration of diphtheria-tetanus / acellular pertussis (DT/aP) vaccines from a single producer. In the present report 19 children with pruritic nodules after vaccination with Al hydroxide-adsorbed DTaP/polio+Hib (Infanrix, Pentavac) are described. The children had intensely itching nodules at the injection site, often aggravated during upper respiratory tract infections, and local skin alterations. So far, the symptoms have persisted for up to 7 years. The median time between vaccination and onset of symptoms was 1 month. 16 children were epicutaneously tested for Al, all with positive reactions indicating delayed hypersensitivity to Al. The condition is not commonly known but is important to recognise, as the child and the family may suffer considerably. Future vaccinations with Al-adsorbed vaccines may cause aggravation of the symptoms and the Al allergy. Al-containing skin products, such as antiperspirants, may cause contact dermatitis. Nodules may be mistaken for tumours. Even though the incidence of itching nodules and Al allergy after administration of Infanrix, Pentavac and other Al-adsorbed vaccines is probably low, research to replace Al adjuvants seems appropriate. We conclude that intensely itching subcutaneous nodules, lasting for many years, and hypersensitivity to aluminium may occur after DTaP/polio+Hib vaccination of infants.
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| 2. |
- Heikkinen, Terho, et al.
(author)
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Schould healthy children be vaccinated against influenza?
- 2006
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In: European Journal of Pediatrics. - : Springer Science and Business Media LLC. - 0340-6199 .- 1432-1076. ; 165:4, s. 223-228
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Journal article (peer-reviewed)abstract
- Influenza is often regarded as an illness of the elderly portion of the population because most of the excess mortality associated with influenza epidemics occurs in that age group. However, evidence derived from a large number of clinical studies carried out in different countries and various settings has clearly demonstrated that the burden of influenza is also substantial in children. The attack rates of influenza during annual epidemics are consistently highest in children, and young children are hospitalized for influenza-related illnesses at rates comparable to those for adults with high-risk conditions. Especially among children younger than 3 years of age, influenza frequently predisposes the patient to bacterial complications such as acute otitis media. Children also serve as the main transmitters of influenza in the community. A safe and effective vaccine against influenza has been available for decades, but the vaccine is rarely used even for children with high-risk conditions. Despite several existing problems related to influenza vaccination of children, the current evidence indicates that the advantages of vaccinating young children would clearly outweigh the disadvantages. Considering the total burden of influenza in children, children younger than 3 years of age should be regarded as a high-risk group for influenza, analogously with the age-based definition of high risk among persons 65 years of age or older. Annual influenza vaccination should be recommended to all children from 6 months to 3 years of age.
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| 6. |
- Manea Hedström, Minola, et al.
(author)
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ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura.
- 2007
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In: European Journal of Pediatrics. - : Springer Science and Business Media LLC. - 1432-1076 .- 0340-6199. ; 166:3, s. 249-257
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Journal article (peer-reviewed)abstract
- The activity of ADAMTS 13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n=20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS 13 was not detected in the plasma from patients with congenital TTP (n=5) by either antibody, whereas patients with acquired TTP (n=2) presented the normal phenotype. Following immunoadsorption of immunoglobulins, the ADAMTS 13 band was removed from the plasma of the patients with acquired TTP, but not from that of normal individuals. This indicates that ADAMTS13 is complexed with immunoglobulin in these patients. The lack of ADAMTS13 expression in the plasma from patients with hereditary TTP may indicate defective synthesis, impaired cellular secretion, or enhanced degradation in the circulation. This study differentiated between normal and TTP plasma, as well as between congenital and acquired TTP. This method may, therefore, be used as a complement in the diagnosis of TTP.
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| 7. |
- Oskarsdóttir, Sólveig, 1953, et al.
(author)
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Presenting phenotype in 100 children with the 22q11 deletion syndrome.
- 2005
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In: European journal of pediatrics. - : Springer Science and Business Media LLC. - 0340-6199 .- 1432-1076. ; 164:3, s. 146-53
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Journal article (peer-reviewed)abstract
- The aim of this study was to investigate and describe the presenting phenotype of children with the 22q11 deletion syndrome and to describe common clinical features that could serve as guidelines in the clinical diagnostic process preceding genetic testing. A hospital-based study of 100 consecutive children and adolescents with 22q11 deletion was initiated. The patients were divided into two groups according to age at diagnosis: before or after 2 years of age. Clinical features were grouped into a core set of eight features: cardiac defects, non-visible/hypoplastic thymus or infection problems, hypocalcaemia, feeding difficulties, cleft palate/speech-language impairment, developmental delay/learning difficulties, characteristic dysmorphic features and other malformations and deformities. The median age at diagnosis was 6.7 years. Of all patients, 26% were diagnosed in infancy and 92% had a congenital cardiac defect, whereas 54% of those diagnosed later had a cardiac defect. A cleft palate was present in 25 cases and 44 had some other malformation or deformity. All presented with a combination of many of the core features. Of those diagnosed after 2 years of age, the majority presented with speech-language impairment, developmental delay or learning difficulties and recurrent infections. Characteristic mild dysmorphic features were noticed in all children. Conclusion: In spite of variable clinical expression, children with 22q11 deletion share a number of major features and have a characteristic phenotype. A high proportion have no cardiac defect and hence a risk of diagnostic delay. Increased awareness and knowledge among general paediatricians and other specialists who meet these children early in life is needed to reduce the diagnostic delay.
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| 8. |
- Trollfors, Birger, 1947, et al.
(author)
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Diphtheria, tetanus and pertussis antibodies in 10-year-old children before and after a booster dose of three toxoids: implications for the timing of a booster dose.
- 2006
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In: European journal of pediatrics. - : Springer Science and Business Media LLC. - 0340-6199 .- 1432-1076. ; 165:1, s. 14-8
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Journal article (peer-reviewed)abstract
- In an open study, 502 10-year-old children, who had received primary vaccination against diphtheria and tetanus in infancy and had varying histories of pertussis disease and vaccination, were vaccinated with diphtheria-tetanus vaccine (DT) alone or with the addition of 20 microg or 40 microg of pertussis toxoid. Diphtheria toxin neutralising antibodies, pertussis toxin IgG and tetanus toxoid IgG antibodies were measured before and 1 month after the booster. All toxoids were highly immunogenic. In pertussis toxoid recipients, median levels of pertussis toxin IgG increased to 16.5 U/ml (DTaP20) and to 36 U/ml (DTaP40) in children with non-detectable (<1 U/ml) antibodies before vaccination and to >400 U/ml in children (both DTaP20 and DTaP40) with detectable antibodies before vaccination. A total of 60 children (12%) with non-detectable (<0.01 IU/ml) diphtheria antibodies and 36 children (7%) with non-detectable (<0.01 IU/ml) tetanus antibodies before the booster had lower median antibody concentrations post-vaccination than children with detectable antibodies before the booster (diphtheria: 5.12 vs. 20.48 IU/ml; tetanus: 4.0 vs. 10.0 IU/ml). There were no differences in diphtheria and tetanus antibodies after vaccination between children who did and did not receive pertussis toxoid. CONCLUSION: 10-year-old children with non-detectable diphtheria and tetanus antibodies before the booster had lower post-vaccination antibodies than those with detectable antibodies before the booster indicating a poor immunological memory. Addition of pertussis toxoid to diphtheria-tetanus vaccine did not affect the antibody responses to diphtheria and tetanus toxoids when the three toxoids were combined as a booster. Even though immunity to diphtheria and tetanus was only estimated by surrogate markers (serum antitoxin antibodies) the results indicate that a lower age for the booster dose of diphtheria-tetanus vaccine or diphtheria-tetanus acellular pertussis vaccine should be considered.
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| 9. |
- Tulinius, Mar, 1953, et al.
(author)
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A family with pyruvate dehydrogenase complex deficiency due to a novel C>T substitution at nucleotide position 407 in exon 4 of the X-linked Epsilon1alpha gene.
- 2005
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In: European journal of pediatrics. - : Springer Science and Business Media LLC. - 0340-6199 .- 1432-1076. ; 164:2, s. 99-103
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Journal article (peer-reviewed)abstract
- The pyruvate dehydrogenase complex (PDHc; McKusick 312170), localised in the mitochondrial matrix, is a multienzyme complex which converts pyruvate to acetyl-CoA. A deficiency of PDHc leads to inadequate removal of pyruvate and lactate resulting in lactic acidaemia and insufficient energy production. The major cause of PDHc deficiency is a defect in the E1alpha component. The gene of this component is localised to Xp22.1. We describe two brothers with a relatively mild clinical phenotype of PDHc deficiency. Onset of disease was associated with muscle weakness and swallowing difficulties in both. At follow-up, the older brother developed encephalopathic features consistent with Leigh syndrome. Lactate to pyruvate ratios were low, consistent with a PDHc deficiency which was confirmed by measurements of PDHc activity in thrombocytes. A 407C>T change in exon 4 of the E1alpha gene was found in both brothers and their mother. This substitution predicts a replacement of a conserved alanine at position 136 by valine. CONCLUSION: Due to the X-linked inheritance pattern combined with the overall results of clinical investigations, molecular genetic findings and a corresponding functional deficiency of the gene product we believe that this substitution in the pyruvate dehydrogenase E1alpha gene is a mutation leading to pyruvate dehydrogenase complex deficiency in this family.
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| 11. |
- Andersson, Ingvar, et al.
(author)
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Breast tomosynthesis and digital mammography: a comparison of breast cancer visibility and BIRADS classification in a population of cancers with subtle mammographic findings.
- 2008
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In: European Radiology. - : Springer Science and Business Media LLC. - 0938-7994 .- 1432-1084. ; 18, s. 2817-2825
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Journal article (peer-reviewed)abstract
- The main purpose was to compare breast cancer visibility in one-view breast tomosynthesis (BT) to cancer visibility in one- or two-view digital mammography (DM). Thirty-six patients were selected on the basis of subtle signs of breast cancer on DM. One-view BT was performed with the same compression angle as the DM image in which the finding was least/not visible. On BT, 25 projections images were acquired over an angular range of 50 degrees, with double the dose of one-view DM. Two expert breast imagers classified one- and two-view DM, and BT findings for cancer visibility and BIRADS cancer probability in a non-blinded consensus study. Forty breast cancers were found in 37 breasts. The cancers were rated more visible on BT compared to one-view and two-view DM in 22 and 11 cases, respectively, (p < 0.01 for both comparisons). Comparing one-view DM to one-view BT, 21 patients were upgraded on BIRADS classification (p < 0.01). Comparing two-view DM to one-view BT, 12 patients were upgraded on BIRADS classification (p < 0.01). The results indicate that the cancer visibility on BT is superior to DM, which suggests that BT may have a higher sensitivity for breast cancer detection.
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| 12. |
- Mellgren, Karin, 1962, et al.
(author)
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Liver transplantation after stem cell transplantation with the same living donor in a monozygotic twin with acute myeloid leukemia
- 2005
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In: Ann Hematol. - : Springer Science and Business Media LLC. - 0939-5555. ; 84:11, s. 755-7
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Journal article (peer-reviewed)abstract
- Two monozygotic twins from a Swedish, nonconsanguine family-with concordant acute myeloid leukemia and similar morphological and cytogenetic changes, but with additional changes in one twin, suggestive of clonal evolution-are described. Twin I relapsed 4 months after completion of treatment, while twin II was still on treatment and was transplanted with stem cells from the human leukocyte antigen-identical father. An early relapse after transplantation was treated with donor lymphocyte infusions, but twin I relapsed again and died 8 months after stem cell transplantation (SCT). On relapse of twin I, treatment of twin II was reconsidered and consolidation was intensified with SCT in CR1 with peripheral blood stem cells from the father. Due to irreversible liver failure caused by severe venoocclusive disease, a living, related liver transplantation from the father was performed on day +84 post-SCT. Minimal immunosuppression was required, and graft rejection did not occur. The patient was in complete remission 29 months after SCT and 25 months after liver transplantation.
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| 13. |
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