[Myositis-specific antibodies associated with juvenile dermatomyositis]. / Myositisspezifische Antikörper bei juveniler Dermatomyositis.

BACKGROUND: Juvenile dermatomyositis (JDM) is a rare autoimmune disease associated with typical skin changes and muscle weakness. Within the framework of the diagnostics, myositis-associated (MAA) and myositis-specific antibodies (MSA) can be detected. These are important for the assessment of the course of the disease and the prognosis. METHOD: In this study we searched for MAA and MSA by means of a line immunoassay in 12 currently supervised JDM patients in the Rheumatism Center Sankt Augustin. RESULTS: In 10 of the 12 patients a total of 15 myositis antibodies were detected where 3 patients each had Mi2, SRP or NXP2 antibodies, 2 had TIF-1γ antibodies and Jo1 or Mi2ß antibodies were found in 1 patient each. Of the patients two had additional PM-Scl antibodies. In the 10 patients with detected antibodies, a good phenotype-serotype correlation was found with deviation from the phenotypes described in the literature in only 3 patients. CONCLUSION: 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.

Anticoagulation management during multivessel coronary artery bypass grafting: a randomized trial comparing individualized heparin management and conventional hemostasis management.

BACKGROUND: Individualized heparin management (IHM) uses heparin dose-response curves to improve hemostasis management during cardiac surgery as compared with activated clotting time-based methods. OBJECTIVES: IHM was compared with conventional hemostasis management (CHM) in a randomized, prospective study (ID DRKS00007580). METHODS: One-hundred and twenty patients undergoing multivessel coronary artery bypass grafting (CABG) were enrolled. Heparin and protamine consumption, blood losses, blood transfusions and administration of hemostatic agents were recorded. Time courses of platelet counts and of coagulation parameters were determined. Coagulation was analyzed at intensive care unit (ICU) arrival by thromboelastometry. RESULTS: IHM patients received significantly lower initial heparin doses (289.3IU kg(-1) [interquartile range (IQR) 221.5-376.2 IU kg(-1) ] versus 350.5 IU kg(-1) [IQR 346.8-353.7 IU kg(-1) ], P < 0.0001) but similar total heparin doses (418.5 IU kg(-1) [IQR 346.9-590.5 IU kg(-1) ] versus 435.8 IU kg(-1) [IQR 411.7-505.1 IU kg(-1) ]). IHM patients received significantly less protamine, resulting in protamine/total heparin ratios of 0.546 [IQR 0.469-0.597] versus 0.854 [IQR 0.760-0.911] in CHM patients (P < 0.0001). Activated partial thromboplastin time (50.5 s [IQR 40.0-60.0 s] versus 37.0 s [IQR 33.0-40.0 s], P < 0.0001), activated clotting time (136 s [IQR 129.0-150.5 s] versus 126.5 s [IQR 120.3-134.0 s], P = 0.0002) and INTEM clotting times (215 s [IQR 192-237] versus 201 s [IQR 191-216 s], P = 0.0397) were significantly longer in IHM patients than in CHM patients at ICU arrival, with no difference in prothrombin time (P = 0.538). IHM patients lost significantly more blood within 12 h postoperatively (420 mL [IQR 337.5-605.0 mL] versus 345 mL [IQR 230.0-482.5 mL], P = 0.0041), and required significantly more hemostatic agents to control bleeding. Red blood cell transfusion requirements and time courses of platelet counts did not differ between groups. CONCLUSIONS: Multivessel CABG patients did not benefit from IHM in comparison with our established protocol based on activated clotting time.

Intrathoracic herniation of the liver ('liver-up') is associated with predominant left heart hypoplasia in human fetuses with left diaphragmatic hernia.

OBJECTIVES: Left heart hypoplasia is commonly observed in fetuses with left diaphragmatic hernia. Because in this condition intrathoracic herniation of the liver serves as an important predictor for postnatal disease severity, we studied its potential association with left heart hypoplasia. METHODS: We prospectively assessed 32 fetuses with left diaphragmatic hernia between 19 + 6 and 38 + 6 weeks of gestation using echocardiography. The fetuses were divided into two groups: Group I exhibited an intrathoracic liver position ('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 intra-abdominal 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.