Jan-Inge Henter

..We suggest that deferiprone not be prescribed to DBA patients unless the clinical indications are particularly strong, and that the risk of agranulocytosis in thalassemia patients be carefully considered...

..In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged...

..Prompt treatment of HLH at onset or relapse may reduce these complications...

..Conclusion: Future studies should aim to clarify the mechanisms underlying the evolution of secondary malignancies in these patients...

..Plasma levels of pro-LL-37 may thus prove useful for differential diagnosis of chronic neutropenia...

..We aimed to study care-related determinants of when parents gain awareness of their child's impending death to cancer, and whether the duration of this awareness affects the parents' long-term morbidity...

..Additionally, two novel polymorphisms, one in intron 17 of AP3B1 and another in intron 2 of SH2D1A were identified. We conclude that mutations in SRGN, ARF6, and AP3B1 are not likely to be common in patients fulfilling the FHL criteria...

..It is important to be aware of HLH in premature infants, since it is treatable...

..Patients that responded well to initial pretransplant-induction therapy fared best, but some persisting HLH activity should not automatically preclude performing SCT...

..These findings warrant further evaluation of the mechanism(s) of regulatory effects of VEGF on neuronal and non-neuronal cells, and the relation of these events to motor neuron degeneration and the onset and progression of ALS...

..Moreover, an increased degree of apoptosis of myeloid progenitor cells in the bone marrow has been shown. CONCLUSION: Future studies should aim to clarify the underlying molecular genetic defect in Kostmann syndrome...

..The 3-year probability of survival after BMT was 62% (+/- 12%). HLH-94 is very effective, allowing BMT in most patients. Survival of children with HLH has been greatly improved...

..Among multi-system patients, at least 19% suffered CNS sequelae...

..However, although they no longer experience life-threatening bacterial infections, they frequently still have recurrent gingivitis and even severe periodontitis, often starting in early childhood...

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..036). Disease monitoring is important in pulmonary LCH, and since PFT with diffusing capacity provides a measurement of the current lung function, it may be a valuable tool in monitoring pulmonary LCH, and a good complement to imaging...

..The data suggests that HLH patients with NK-cell deficiency type 3 will probably require SCT to survive. Thus, NK-cell deficiency classification may provide valuable guidance in judging whether an HLH-patient needs SCT...

..We conclude that filgrastim and lenograstim displayed equal clinical efficacy, but that ANC levels were higher during filgrastim treatment, when administered in equimolar doses...

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..Langerhans cell histiocytosis is a rare disease of unknown etiology. We wanted to assess the population-based incidence of LCH in a well-defined cohort of children...

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..We here aimed to investigate the neuropsychological consequences in more detail...

..Understanding which symptoms affect children in the terminal phase of disease is crucial to improve palliative care...

..We evaluated the complications of parvovirus B19 infection, including delays in the scheduled course of chemotherapy, in children with acute lymphoblastic leukemia (ALL)...

..To explore whether early treatment of children with idiopathic thrombocytopenic purpura (ITP) with immunoglobulin and/or corticosteroids reduces subsequent morbidity...

..With this notable improvement in survival, specific HLH treatment, including cytotoxic therapy, could be considered in patients with severe avian influenza A infection complicated by secondary HLH...

..However, none of these sequence variations were associated with the manifestations of FHL. These data do not support the notion that granulysin and granzyme B are candidate genes for FHL...

..To investigate whether pulsed high-dose dexamethasone is more efficacious than intravenous immunoglobulin (IVIg) as treatment of symptomatic chronic idiopathic thrombocytopenic purpura (ITP) in childhood...

..These observations suggest that VEGF or related compounds, might be of value in the treatment of ALS patients...

..In sum, these studies establish a role for mitochondria-dependent apoptosis in the pathogenesis of Kostmann syndrome and yield a tentative explanation for the beneficial effect of growth factor administration in these patients...

..Antibodies with anti-M specificity are detected in 10 percent of pregnant women with a positive antibody screen, but anti-M is only rarely associated with hemolytic anemia in the fetus...

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..Together, these studies indicate that resistance to BA-induced apoptosis is a common feature of BL-derived cell lines...

..These findings show, for the first time, that serum OPG levels are elevated in LCH patients, and suggest that OPG may play a role in the pathogenesis of this enigmatic disease...

..Although the numerical data cannot be directly translated to ethical conclusions, they can provide guidance for future ethical decisions...

..Further studies to confirm these findings are desired...

..The establishment of perforin deficiency as a cause of the rapidly fatal disease FHL has demonstrated the essential role of perforin in human immune homeostasis...

..We conducted a population-based, nationwide study addressing this issue, focusing on potential care-related stressors in parents losing a child to cancer...

..Overall, after a median follow-up of 43 months, 59 (75.6%) of the 78 patients are alive and well. INTERPRETATION AND CONCLUSIONS: The majority of successfully treated EBV-HLH patients have a good outcome and remain disease-free...

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..Our findings suggest that HAX1 is a major regulator of myeloid homeostasis and underline the significance of genetic control of apoptosis in neutrophil development...

..Fatal EBV infection in male infants is highly indicative of XLP. Virus-associated haemophagocytic syndrome and B-cell lymphoma can occur concomitantly and may be difficult to distinguish due to their similar histological pictures...

..We investigated parental risk of anxiety and depression 4-9 years after the loss of a child owing to a malignancy...