BACKGROUND: The absence of high quality evidence for basic clinical dilemmas in immune thrombocytopenic purpura (ITP) underlines the need for contemporary guidelines relevant to the local treatment context. ITP is diagnosed by exclusions, with a hallmark laboratory finding of isolated thrombocytopenia.
UNASSIGNED: Bleeding, family and medication histories and a review of historical investigations are required to gauge the bleeding risk and possible hereditary syndromes. Beyond the platelet count, the decision to treat is affected by individual bleeding risk, disease stage, side effects of treatment, concomitant medications, and patient preference. Treatment is aimed at achieving a platelet count > 20 × 109 /L, and avoidance of severe bleeding. Steroids are the standard first line treatment, with either 6-week courses of tapering prednisone or repeated courses of high dose dexamethasone providing equivalent efficacy. Intravenous immunoglobulin can be used periprocedurally or as first line therapy in combination with steroids.
UNASSIGNED: There is no consensus on choice of second line treatments. Options with the most robust evidence include splenectomy, rituximab and thrombopoietin receptor agonists. Other therapies include azathioprine, mycophenolate mofetil, dapsone and vinca alkaloids. Given that up to one-third of patients achieve a satisfactory haemostatic response, splenectomy should be delayed for at least 12 months if possible. In life-threatening bleeding, we recommend platelet transfusions to achieve haemostasis, along with intravenous immunoglobulin and high dose steroids.

BACKGROUND: Thrombopoietin receptor agonists (TPO-RAs) are used to treat primary immune thrombocytopenia (ITP). Some patients have discontinued treatment while maintaining a hemostatic platelet count.
OBJECTIVE: To develop expert consensus on when it is appropriate to consider tapering TPO-RAs in ITP, how to taper patients off therapy, how to monitor patients after discontinuation, and how to restart therapy.
METHODS: We used a RAND/UCLA modified Delphi panel method. Ratings were completed independently by each expert before and after a meeting. Second-round ratings were used to develop the panel\'s guidance. The panel was double-blinded: The sponsor and nonchair experts did not know each other\'s identities.
RESULTS: Guidance on when it is appropriate to taper TPO-RAs in children and adults was developed based on patient platelet count, history of bleeding, intensification of treatment, trauma risk, and use of anticoagulants/platelet inhibitors. For example, it is appropriate to taper TPO-RAs in patients who have normal/above-normal platelet counts, have no history of major bleeding, and have not required an intensification of treatment in the past 6 months; it is inappropriate to taper TPO-RAs in patients with low platelet counts. Duration of ITP, months on TPO-RA, or timing of platelet response to TPO-RA did not have an impact on the panel\'s guidance on appropriateness to taper. Guidance on how to taper patients off therapy, how to monitor patients after discontinuation, and how to restart therapy is also provided.
CONCLUSIONS: This guidance could support clinical decision making and the development of clinical trials that prospectively test the safety of tapering TPO-RAs.

暂无摘要。

Atopic eczema is a chronic inflammatory disease affecting about 30% of Australian and New Zealand children. Severe eczema costs over AUD 6000/year per child in direct medical, hospital and treatment costs as well as time off work for caregivers and untold distress for the family unit. In addition, it has a negative impact on a child\'s sleep, education, development and self-esteem. The treatment of atopic eczema is complex and multifaceted but a core component of therapy is to manage the inflammation with topical corticosteroids (TCS). Despite this, TCS are often underutilised by many parents due to corticosteroid phobia and unfounded concerns about their adverse effects. This has led to extended and unnecessary exacerbations of eczema for children. Contrary to popular perceptions, (TCS) use in paediatric eczema does not cause atrophy, hypopigmentation, hypertrichosis, osteoporosis, purpura or telangiectasia when used appropriately as per guidelines. In rare cases, prolonged and excessive use of potent TCS has contributed to striae, short-term hypothalamic-pituitary-adrenal axis alteration and ophthalmological disease. TCS use can also exacerbate periorificial rosacea. TCS are very effective treatments for eczema. When they are used to treat active eczema and stopped once the active inflammation has resolved, adverse effects are minimal. TCS should be the cornerstone treatment of atopic eczema in children.

暂无摘要。

BACKGROUND: The evaluation of febrile children with petechial rashes evokes controversy. Although many of these children have viral infections, on occasion such patients may be infected with Neisseria meningitidis.
OBJECTIVE: To investigate differences in practice trends for the evaluation and management of non-toxic-appearing febrile children with petechial rashes among pediatric specialty groups.
METHODS: We surveyed 833 pediatricians in 4 specialties [community (CGP) and academic (AGP) general pediatrics, emergency medicine (EM) and infectious diseases] regarding 4 hypothetical non-toxic-appearing febrile children ages 1, 2, 5 and 7 years. The patients differed with regard to clinical appearance, distribution of petechiae and complete blood count results. We compared specialty group responses, adjusting for practice setting, population size and years in practice using multiple logistic regression analysis.
RESULTS: The survey was completed and returned by 416 (50%) pediatricians. There was substantial variation in the evaluation of the 2 younger febrile children without clear sources for their petechiae. For the 1-year-old the overall blood culture (BCx) rate was 82%, with the EM group (91%) more often requesting BCx than either the CGP (76%) or AGP (73%, P=0.001) groups. The overall hospital admission rate was 31%, with CGP less often requesting admission than infectious disease pediatricians (22% vs. 40%, P=0.007). In the regression analysis the only significant difference between groups was in BCx rate between the EM and AGP groups. For the 2-year-old the overall rate of BCx was 95%, lumbar puncture was 41% and admission was 44%, with no significant differences among groups. For the scenarios involving the 2 older febrile children with sources for their petechiae, the majority of respondents chose neither lumbar puncture nor admission. There was disagreement regarding BCx, both within and between groups, although most of the between group differences did not persist in the regression analysis.
CONCLUSIONS: There are substantial differences among pediatricians in the evaluation of young non-toxic-appearing febrile children with petechial rashes. Although there are some differences between pediatric subspecialties, most of these differences do not persist after adjusting for practice setting, population size and physician experience.