Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood

Purpose. The aim of this study was to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period. Materials and Methods. A retrospective review was conducted of all pediatric patients treated for SC-GCTs at our hospital from 1998 to 2012. Results. Fifty-seven patients were treated for SC-GCTs with the most common in Altman’s classification type I. Age at surgery ranged from one day to 5.6 years. Tumor resection and coccygectomy were primarily performed in about 84% of the cases. Pathology revealed mature, immature, malignant sacrococcygeal teratomas (SCTs), and endodermal sinus tumors (ESTs) in 41 (72%), 4 (77%), 6 (10.5%), and 6 (10.5%), respectively. Recurrence of discase occurred in 3 of 41 patients with mature teratomas (7.3%); 2 recurrences with mature teratomas and one recurrence with EST. Five of 6 malignant SCTs and 3 of 6 ESTs responded well to the treatment. Alpha-fetoprotein (AFP) level was elevated in both malignant teratomas and ESTs. No immediate patient death was noted in any of the 57 cases, but 4 patients with malignant tumors and distant metastasis succumbed at home within 2 years of the initial treatment. Conclusion. Benign SCTs have a significant recurrence rate of approximately 7%. Close follow-up with serial AFP level monitoring should be done for 5 years after initial tumor resection and coccygectomy. The survival rate for malignant SC-GCTs with distant metastasis was unfavorable in the present study

Clinical Outcomes of Esophageal Atresia: Comparison Between the Waterston and the Spitz Classifi cations

Abstract Introduction: Preoperative prognostic predictors are important for surgeons and parents to estimate the survival of patients with esophageal atresia (EA). The aim of this study was to update the clinical outcomes of EA treatment by comparing between the Waterston and the Spitz classifi cation. Materials and Methods: Medical records of the patients with EA treated at Queen Sirikit National Institute of Child Heath from 2003 to 2010 were reviewed. All of the patients were categorised into 3 groups of the Waterston and 3 groups of the Spitz risk factor criteria for comparing of the differences in each group and each classifi cation. Results: One hundred and thirty-two patients (81 males and 61 females) were treated for EA during the study period. Applying the Waterston classifi cation, survival rate was 100% in group A, 91.5% in group B and 48.8% in group C. There was no statistical difference between the survival rate in group A and group B (P = 0.119) but signifi cant difference between group B and group C (P = 0.000). Using the Spitz classifi cation, survival rate was 97.4% in group I, 64.4% in group II and 27.3% in group III. There was obviously statistical difference of the survival rate between each group (group I vs group II, P = 0.000; group II vs group III, P = 0.041). Conclusion: Comparing with the prognostic predictors, the Spitz classifi cation was more valid than the Waterston criteria. The Spitz classifi cation is suitable to use for preoperative predictor to parental counselling and comparing of treatment outcomes of EA among paediatric tertiary care centres

Effects of sevuparin on rosette formation and cytoadherence of Plasmodium falciparum infected erythrocytes.

In severe falciparum malaria cytoadherence of parasitised red blood cells (PRBCs) to vascular endothelium (causing sequestration) and to uninfected red cells (causing rosette formation) contribute to microcirculatory flow obstruction in vital organs. Heparin can reverse the underlying ligand-receptor interactions, but may increase the bleeding risks. As a heparin-derived polysaccharide, sevuparin has been designed to retain anti-adhesive properties, while the antithrombin-binding domains have been eliminated, substantially diminishing its anticoagulant activity. Sevuparin has been evaluated recently in patients with uncomplicated falciparum malaria, and is currently investigated in a clinical trial for sickle cell disease. The effects of sevuparin on rosette formation and cytoadherence of Plasmodium falciparum isolates from Thailand were investigated. Trophozoite stages of P. falciparum-infected RBCs (Pf-iRBCs) were cultured from 49 patients with malaria. Pf-iRBCs were treated with sevuparin at 37°C and assessed in rosetting and in cytoadhesion assays with human dermal microvascular endothelial cells (HDMECs) under static and flow conditions. The proportion of Pf-iRBCs forming rosettes ranged from 6.5% to 26.0% (median = 12.2%). Rosetting was dose dependently disrupted by sevuparin (50% disruption by 250 μg/mL). Overall 57% of P. falciparum isolates bound to HDMECs under static conditions; median (interquartile range) Pf-iRBC binding was 8.5 (3.0-38.0) Pf-iRBCs/1000 HDMECs. Sevuparin in concentrations ≥ 100 μg/mL inhibited cytoadherence. Sevuparin disrupts P. falciparum rosette formation in a dose dependent manner and inhibits cytoadherence to endothelial cells. The data support assessment of sevuparin as an adjunctive treatment to the standard therapy in severe falciparum malaria

Effects of sevuparin on rosette formation and cytoadherence of <i>Plasmodium falciparum</i> infected erythrocytes - Fig 1

<p>(A) Effect of sevuparin on rosetting (%) of <i>P</i>. <i>falciparum</i> (n = 47) is dose dependent. The data show the median with interquartile range of rosettes formed at each concentration of sevuparin. (B) Median (interquartile range) % disruption of rosetting at each concentration of sevuparin. Sevuparin significantly disrupted rosette formation, p < 0.001.</p

Effects of sevuparin on rosette formation and cytoadherence of <i>Plasmodium falciparum</i> infected erythrocytes

<div><p>In severe <i>falciparum</i> malaria cytoadherence of parasitised red blood cells (PRBCs) to vascular endothelium (causing sequestration) and to uninfected red cells (causing rosette formation) contribute to microcirculatory flow obstruction in vital organs. Heparin can reverse the underlying ligand-receptor interactions, but may increase the bleeding risks. As a heparin-derived polysaccharide, sevuparin has been designed to retain anti-adhesive properties, while the antithrombin-binding domains have been eliminated, substantially diminishing its anticoagulant activity. Sevuparin has been evaluated recently in patients with uncomplicated <i>falciparum</i> malaria, and is currently investigated in a clinical trial for sickle cell disease. The effects of sevuparin on rosette formation and cytoadherence of <i>Plasmodium falciparum</i> isolates from Thailand were investigated. Trophozoite stages of <i>P</i>. <i>falciparum</i>-infected RBCs (Pf-iRBCs) were cultured from 49 patients with malaria. Pf-iRBCs were treated with sevuparin at 37°C and assessed in rosetting and in cytoadhesion assays with human dermal microvascular endothelial cells (HDMECs) under static and flow conditions. The proportion of Pf-iRBCs forming rosettes ranged from 6.5% to 26.0% (median = 12.2%). Rosetting was dose dependently disrupted by sevuparin (50% disruption by 250 μg/mL). Overall 57% of <i>P</i>. <i>falciparum</i> isolates bound to HDMECs under static conditions; median (interquartile range) Pf-iRBC binding was 8.5 (3.0–38.0) Pf-iRBCs/1000 HDMECs. Sevuparin in concentrations ≥ 100 μg/mL inhibited cytoadherence. Sevuparin disrupts <i>P</i>. <i>falciparum</i> rosette formation in a dose dependent manner and inhibits cytoadherence to endothelial cells. The data support assessment of sevuparin as an adjunctive treatment to the standard therapy in severe <i>falciparum</i> malaria.</p></div

Median (interquartile range) Pf-iRBCs adhering to HDMECs under shear stress at 0.05 Pa, 0.5% parasitaemia and 1% haematocrit at different concentrations of sevuparin (n = 10).

<p>Median (interquartile range) Pf-iRBCs adhering to HDMECs under shear stress at 0.05 Pa, 0.5% parasitaemia and 1% haematocrit at different concentrations of sevuparin (n = 10).</p

Effects of sevuparin on rosette formation and cytoadherence of <i>Plasmodium falciparum</i> infected erythrocytes - Fig 2

<p>(A) The effects of sevuparin on Pf-iRBC adherence (n = 28). Median (interquartile range) adherence number of Pf-iRBCs binding per 1000 HDMECs at each concentration of sevuparin. (B) The inhibition of sevuparin on cytoadherence of <i>P</i>. <i>falciparum</i> (n = 28). Median (interquartile range) inhibition effect on cytoadhesion. The cytoadherence of patient isolates was significantly inhibited by sevuparin at concentrations ≥ 100 μg/mL (all p < 0.05).</p

The correlation between the adherent number of Pf-iRBCs binding per 1000 HDMECs and rosetting number (%) of <i>P</i>. <i>falciparum</i> (n = 28), (r_s = 0.152, p = 0.439).

<p>The correlation between the adherent number of Pf-iRBCs binding per 1000 HDMECs and rosetting number (%) of <i>P</i>. <i>falciparum</i> (n = 28), (r_s = 0.152, p = 0.439).</p