Preferential ductus venosus and inferior caval vein streaming towards the fetal right heart offers another haemodynamic mechanism for left heart underdevelopment in fetuses with left diaphragmatic hernia. The pathoanatomical basis of this abnormal flow pattern results from intrathoracic abdominal organ herniation and rightward displacement of the heart.
K E Y W O R D S: ('liver-up') and Group II an intra-abdominal liver position ('liver-down'). Cardiac inflow and outflow diameter ratios and cardiac Z-scores were compared between the two groups.
Results
Eleven of the 15 Group I (liver-up) fetuses, but only three of the 17 Group II (liver-down) fetuses with left diaphragmatic hernia exhibited predominant left heart hypoplasia with disproportionately smaller left than right heart dimensions (P = 0.0036). In addition, 14 of the 15 Group I fetuses, but only five of the 17 Group II fetuses exhibited preferential streaming of the ductus venosus towards the right heart (P = 0.0003).Conclusions In fetuses with left diaphragmatic hernia, intrathoracic liver herniation is commonly associated with predominant left heart hypoplasia, whereas an intraabdominal liver position is not. This observation may be explained by preferential ductus venosus streaming towards the right heart from elevation and leftward distortion of the normal course of the ductus venosus and inferior vena cava.
The frequent detection of certain antibodies and the good correlation with those phenotypes described in the literature, show that the determination of MSA is an important diagnostic tool to assess the course, complications and outcome and to initiate adequate therapy at an early stage.
BackgroundSystemic juvenile idiopathic arthritis (sJIA) is characterised by arthritis accompanied or preceded by systemic autoinflammation. High-dose steroids has been the mainstay of therapy with proven effectiveness but also with side-effects. In many patients a chronic course with destructive arthritis long-term cannot be prevented.ObjectivesIn patients naïve for steroids, a steroid-free treatment may allow reconstitution of an impaired NK-cell function and probably remission of sJIA.MethodsFirst experience with first line Anakinra without steroids in 9 consecutive patients is reported.ResultsAll patients presented with ongoing spiking fever and rash and further features of sJIA, high CRP, S100 and IL18 (table 1). Daily sq. injections of Anakinra 2 mg/kg for 3 months resulted in complete remission in 4 and partial response in two children presenting with an oligoarticular involvement. One patient with typical sJIA and very high S100 (MRP8/9) levels did not respond to Anakinra nor to Canakinumab. Two patients presented with polyarthritis. One had no response, the other showed a minor response but improved on steroids and was later treated with tocilizumab. One of the oligoarticular patients with an initial partial response had a flare upon anakinra captured by increased dosing (4 mg/kg) but finally developed macrophage activation syndrome. Anakinra was discontinued after 3 months in 3 of the 4 responders. Two remained in drug free remission while the remainder flared several months later and retreatment was instituted. The patients with polyarticluar involvement first received corticosteroids and were later both treated successfully with the IL-6 inhibitor.ConclusionsExperience with first line steroid free treatment with Anakinra for sJIA is presented. A complete remission was reached in 4 cases with oligoarticular involvement. In 3 further cases improvement was observed and 2 had no response including one who also failed Canakinumab. A toddler with a particular response to Anakinra later on developed MAS. One patient did not respond to both IL-1 inhibitors. Thus, steroid free treatment regimen with Anakinra is feasible and resulted into remission in most but not all patients. Aside, unwarranted effects of long lasting steroid application were avoided.Disclosure of InterestNone declared
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