Time filter

Source Type

Berntorp E.,Skåne University Hospital | Astermark J.,Skåne University Hospital | Baghaei F.,Sahlgrenska University Hospital | Bergqvist D.,Uppsala University Hospital | And 7 more authors.
Haemophilia | Year: 2012

In an ongoing health-technology assessment of haemophilia treatment in Sweden, performed by the governmental agency Dental and Pharmaceutical Benefits Agency (TLV; tandva°rds-och lākemedelsförma°nsverket), the Swedish Council on Health Technology Assessment (SBU; statens beredning för medicinsk utvārdering) was called upon to evaluate treatment of haemophilia A and B and von Willebrand's disease (VWD) with clotting factor concentrates. To evaluate the following questions: What are the short-term and long-term effects of different treatment strategies? What methods are available to treat haemophilia patients that have developed inhibitors against factor concentrates? Based on the questions addressed by the project, a systematic database search was conducted in PubMed, NHSEED, Cochrane Library, EMBASE and other relevant databases. The literature search covered all studies in the field published from 1985 up to the spring of 2010. In most instances, the scientific evidence is insufficient for the questions raised in the review. Concentrates of coagulation factors have good haemostatic effects on acute bleeding and surgical intervention in haemophilia A and B and VWD, but conclusions cannot be drawn about possible differences in the effects of different dosing strategies for acute bleeding and surgery. Prophylaxis initiated at a young age can prevent future joint damage in persons with haemophilia. The available treatment options for inhibitors have been insufficiently assessed. The economic consequences of various treatment regimens have been insufficiently analysed. Introduction of national and international registries is important. © 2011 Blackwell Publishing Ltd.


Schmiegelow K.,Copenhagen University | Forestier E.,Umeå University | Hellebostad M.,Ulleval Hospital | Heyman M.,Astrid Lindgrens Childrens Hospital | And 3 more authors.
Leukemia | Year: 2010

Analysis of 2668 children with acute lymphoblastic leukemia (ALL) treated in two successive Nordic clinical trials (Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-92 and ALL-2000) showed that 75% of all patients are cured by first-line therapy, and 83% are long-term survivors. Improvements in systemic and intrathecal chemotherapy have reduced the use of central nervous system (CNS) irradiation to 10% of the patients and provided a 5-year risk of isolated CNS relapse of 2.6%. Improved risk stratification and chemotherapy have eliminated the previous independent prognostic significance of gender, CNS leukemia and translocation t(1;19)(q23;p13), whereas the post-induction level of minimal residual disease (MRD) has emerged as a new risk grouping feature. Infant leukemia, high leukocyte count, T-lineage immunophenotype, translocation t(4;11)(q21;q23) and hypodiploidy persist to be associated with lower cure rates. To reduce the overall toxicity of the treatment, including the risk of therapy-related second malignant neoplasms, the current NOPHO ALL-2008 protocol does not include CNS irradiation in first remission, the dose of 6-mercaptopurine is reduced for patients with low thiopurine methyltransferase activity, and the protocol restricts the use of hematopoietic stem cell transplantation in first remission to patients without morphological remission after induction therapy or with high levels of MRD after 3 months of therapy. © 2010 Macmillan Publishers Limited All rights reserved.


Asarnoj A.,Karolinska Institutet | Asarnoj A.,Astrid Lindgrens Childrens Hospital | Moverare R.,Phadia AB | Moverare R.,Uppsala University | And 10 more authors.
Allergy: European Journal of Allergy and Clinical Immunology | Year: 2010

Background: Allergen-specific IgE testing is often performed with crude peanut extract, but the results may be difficult to interpret because of cross-reactions between peanut and other plant allergens. The aim was to investigate IgE reactivity to peanut allergen components in children from a birch-rich region in relation to pollen sensitization and peanut symptoms. Methods: From a birth cohort, clinical parameters were obtained through questionnaires and IgE antibody levels to peanut and birch pollen were measured. Different peanut/birch sensitization phenotypes were defined among 200 selected children. IgE reactivity to peanut and pollen allergen components was analysed using microarray technique. Results: Peanut symptoms were reported in 87% of the children with IgE reactivity to any of the peanut allergens Ara h 1, 2 or 3 but not to Ara h 8 (n = 46) vs 17% of children with IgE reactivity to Ara h 8 but not to Ara h 1, 2 or 3 (n = 23), P < 0.001. Furthermore, symptoms were more severe in children with Ara h 1, 2 or 3 reactivity. Children with IgE reactivity both to Ara h 2 and to Ara h 1 or 3 more often reported peanut symptoms than children with IgE only to Ara h 2 (97%vs 70%, P = 0.016), particularly respiratory symptoms (50%vs 9%, P = 0.002). Conclusions: IgE analysis to peanut allergen components may be used to distinguish between peanut-sensitized individuals at risk of severe symptoms and those likely to have milder or no symptoms to peanut if sensitized to pollen allergens and their peanut homologue allergens. © 2010 John Wiley & Sons A/S.


Asarnoj A.,Karolinska Institutet | Asarnoj A.,Astrid Lindgrens Childrens Hospital | Glaumann S.,Sachs Childrens Hospital | Glaumann S.,Karolinska Institutet | And 8 more authors.
International Archives of Allergy and Immunology | Year: 2012

Diagnosis of peanut allergy has improved thanks to component-resolved diagnostics. Peanut allergen component Ara h 2 is considered to indicate true peanut allergy. The component Ara h 6 is structurally similar to Ara h 2, but the diagnostic value of analyzing IgE antibodies to Ara h 6 is unclear. A boy sensitized (≥0.35 kUA/l) to Ara h 8 but not to Ara h 1, Ara h 2 and Ara h 3 was challenged with peanut and developed grade II anaphylaxis. In serum collected at the time of challenge a doubling of IgE to the peanut allergen extract was observed compared to allergy testing 9 months earlier. In contrast, IgE levels to Ara h 1, Ara h 2, Ara h 3 and to Ara h 8 were rather unchanged. After another 2 months, Ara h 6 was analyzed and revealed a level of 24 kUA/l whilst Ara h 2 was 0.12 kUA/l. We suggest that IgE sensitization to Ara h 6 caused the reaction and conclude that analyses of IgE levels to peanut and peanut components should be performed in connection with a challenge. Furthermore, levels to Ara h 2 below 0.35 kUA/l may still indicate a risk of severe reaction at the time of challenge since in rare cases, Ara h 6 IgE antibodies may be present without occurrence of IgE antibodies to Ara h 2. Copyright © 2012 S. Karger AG, Basel.


Mesas Burgos C.,Astrid Lindgrens Childrens Hospital | Mesas Burgos C.,Karolinska Institutet | Ghaffarpour N.,Astrid Lindgrens Childrens Hospital | Ghaffarpour N.,Karolinska Institutet | And 2 more authors.
Journal of Pediatric Surgery | Year: 2011

Laparoscopic cholecystectomy is the standard approach in most pediatric surgical centers. In an attempt to further minimize the surgical trauma and improve cosmetic outcome, new techniques with a single incision through the umbilicus have been proposed. There are still few reports concerning this technique in the pediatric population. We evaluated the feasibility of the single incision for laparoscopic cholecystectomy in children. We performed the operation in 10 patients, with a mean age of 12 years, mean operating time of 122 minutes, and mean hospital stay of 2 days. No complications occurred, and no conversion to open surgery was needed. In 1 patient, an extra 5-mm port was necessary. The cosmetic results were very satisfactory. In our experience, despite its technical difficulty and initial learning curve, single-incision laparoscopic cholecystectomy in the pediatric population is a safe and feasible method. © 2011 Elsevier Inc.


Ballardini N.,Karolinska Institutet | Ballardini N.,Sachs Childrens Hospital Sodersjukhuset | Kull I.,Karolinska Institutet | Lind T.,Karolinska Institutet | And 13 more authors.
Allergy: European Journal of Allergy and Clinical Immunology | Year: 2012

Background Allergy-related diseases are a public health issue, but knowledge on development and comorbidity among children is scarce. The aim was to study the development of eczema, asthma and rhinitis in relation to sex and parental allergy, in a population-based cohort, during childhood. Methods At 1, 2, 4, 8 and 12 years, parental questionnaires were used to obtain data on allergy-related diseases. Complete data for all five follow-up occasions were available from 2916 children. Odds ratios for the risk of any allergy-related disease in relation to heredity and sex were calculated using generalized estimating equations. Results At 12 years, 58% of the children had had eczema, asthma and/or rhinitis at some time. Disease turnover was high for all three diseases throughout the study. Comorbidity increased with age, and at 12 years, 7.5% of all the children were affected by at least two allergy-related diseases. Parental allergy was associated with increased comorbidity and more persistent disease and increased the risk of having any allergy-related disease (adjusted OR 1.76; 95% CI 1.57-1.97) up to 12 years. Male sex was associated with an increased risk throughout childhood. Boys and girls did not differ in disease persistence, and for comorbidity, the differences were minor. Conclusions Allergy-related diseases may affect a majority of children. Eczema, asthma and rhinitis develop dynamically throughout childhood, and allergic comorbidity is common. These findings indicate that allergy-related diseases should be neither seen nor studied as isolated entities. © 2012 John Wiley & Sons A/S.


Marild K.,Astrid Lindgrens Childrens Hospital | Marild K.,Örebro University | Stephansson O.,Karolinska University Hospital | Montgomery S.,Örebro University | And 4 more authors.
Gastroenterology | Year: 2012

Studies on pregnancy characteristics and mode of delivery and risk of later celiac disease in offspring are inconsistent. In recent decades rates of cesarean delivery and preterm birth survival have increased while at the same time the prevalence of celiac disease has doubled. In this population-based case-control study we examined the risk of celiac disease in individuals exposed to cesarean delivery and adverse fetal events (ie, low Apgar score, small for gestational age, low birth weight, preterm birth, and neonatal infections). Prospectively recorded pregnancy data were obtained from the Swedish Medical Birth Register between 1973 and 2008. Study participants consisted of 11,749 offspring with biopsy-verified celiac disease identified through histopathology reports from Sweden's 28 pathology departments, and 53,887 age- and sex-matched controls from the general population. We found a positive association between elective cesarean delivery and later celiac disease (adjusted odds ratio [OR], 1.15; 95% confidence interval [CI], 1.041.26), but no increased risk of celiac disease after emergency (adjusted OR, 1.02; 95% CI, 0.921.13) or any cesarean delivery (adjusted OR, 1.06; 95% CI, 0.991.13). Infants born small for gestational age were at a 21% increased risk of celiac disease (95% CI, 1.091.35), whereas other pregnancy exposures did not increase the risk of future celiac disease. The positive association with elective, but not emergency, cesarean delivery is consistent with the hypothesis that the bacterial flora of the newborn plays a role in the development of celiac disease. © 2012 AGA Institute.


Mesas Burgos C.,Astrid Lindgrens Childrens Hospital | Svenningsson A.,Astrid Lindgrens Childrens Hospital | Vejde J.H.,Karolinska University Hospital | Granholm T.,Astrid Lindgrens Childrens Hospital | Conner P.,Karolinska University Hospital
Pediatric Surgery International | Year: 2015

Background: The timing and mode of delivery of pregnancies with prenatally diagnosed gastroschisis remains controversial. Aim: To evaluate the outcome of patients with gastroschisis managed during two time periods: 2006–2009 and 2010–2014, with planned elective cesarean delivery at 37 versus 35 gestational weeks (gw). A secondary aim was to analyze the outcome in relation to the gestational age at birth. Material and methods: Retrospective review of all cases with gastroschisis managed at our institution between 2006 and 2014. Results: Fifty-two patients were identified, 24 during the initial period, and 28 during the second. There were a significantly higher number of emergency cesarean deliveries in the first period. There were no differences between groups with regard to the use of preformed silo, need of parenteral nutrition or length of hospital stay. When analyzing the outcome in relation to the gw the patients actually were born, we observed that patients delivered between 35 and 36.9 gw were primary closed in 88.5 % of cases, with shorter time on mechanical ventilation, parenteral nutrition and hospital stay. Conclusion: Planned caesarian section at 35 completed gestational weeks for fetuses with prenatally diagnosed gastroschisis is safe. We observe the best outcome for patients born between 35 and 36.9 gw. © 2015, Springer-Verlag Berlin Heidelberg.


Mesas Burgos C.,Astrid Lindgrens Childrens Hospital | Hammarqvist-Vejde J.,Karolinska University Hospital | Frenckner B.,Astrid Lindgrens Childrens Hospital | Conner P.,Karolinska University Hospital
Fetal Diagnosis and Therapy | Year: 2016

Objectives: To compare outcomes in pregnancies with a prenatal detection of congenital diaphragmatic hernia (CDH) with children diagnosed after birth, treated at the same institution, and to determine the ability to predict prognosis through measurements of the observed to expected lung-to-head ratio (O/E LHR). Methods: This is a retrospective review of all children with CDH treated at our institution during 2006-2014. We compared outcomes of infants referred for surgery after postnatal diagnosis with outcomes of infants with prenatally diagnosed CDH. Results: In the prenatal group, O/E LHR was significantly different between survivors and deceased patients, with a cutoff at 35% O/E LHR. Survival to discharge and 1-year survival were significantly higher in the postnatal group that required intubation within 24 h; i.e., 92 and 89% versus 85 and 73% in the prenatal group (p < 0.05). There was less need for extracorporeal membrane oxygenation (ECMO), 41 versus 60%, and patch, 41 versus 75% (p < 0.001), in the postnatal group with early diagnosis compared with the prenatal group, respectively. Conclusion: Children with prenatally diagnosed CDH represent a population with a more severe condition compared to infants diagnosed after birth. They have poorer outcomes with higher needs for ECMO or use of patch, and lower survival rates were observed at an O/E LHR below 35%. © 2015 S. Karger AG, Basel.


PubMed | Astrid Lindgrens Childrens Hospital
Type: Journal Article | Journal: Fetal diagnosis and therapy | Year: 2016

To compare outcomes in pregnancies with a prenatal detection of congenital diaphragmatic hernia (CDH) with children diagnosed after birth, treated at the same institution, and to determine the ability to predict prognosis through measurements of the observed to expected lung-to-head ratio (O/E LHR).This is a retrospective review of all children with CDH treated at our institution during 2006-2014. We compared outcomes of infants referred for surgery after postnatal diagnosis with outcomes of infants with prenatally diagnosed CDH.In the prenatal group, O/E LHR was significantly different between survivors and deceased patients, with a cutoff at 35% O/E LHR. Survival to discharge and 1-year survival were significantly higher in the postnatal group that required intubation within 24 h; i.e., 92 and 89% versus 85 and 73% in the prenatal group (p < 0.05). There was less need for extracorporeal membrane oxygenation (ECMO), 41 versus 60%, and patch, 41 versus 75% (p < 0.001), in the postnatal group with early diagnosis compared with the prenatal group, respectively.Children with prenatally diagnosed CDH represent a population with a more severe condition compared to infants diagnosed after birth. They have poorer outcomes with higher needs for ECMO or use of patch, and lower survival rates were observed at an O/E LHR below 35%.

Loading Astrid Lindgrens Childrens Hospital collaborators
Loading Astrid Lindgrens Childrens Hospital collaborators