Prognostic Factors in High-Grade Osteosarcoma of theExtremities or Trunk: An Analysis of 1,702 Patients Treatedon Neoadjuvant Cooperative Osteosarcoma Study GroupProtocols.

Purpose: To define prognostic factors for response and long-term outcome for a wide spectrum of osteosarcomas, extending well beyond those of the typical young patient with seemingly localized extremity disease.

Patients And Methods: A total of 1,702 consecutive newly diagnosed patients with high-grade osteosarcoma of the trunk or limbs registered into the neoadjuvant studies of the Cooperative Osteosarcoma Study Group before July 1998 were entered into an analysis of demographic, tumor-related, and treatment-related variables, response, and survival. The intended therapeutic strategy included preoperative and postoperative chemotherapy with multiple agents as well as surgery of all operable lesions.

Time course of optic nerve sheath dilation: In vitro response characteristics to controlled pressure elevations.

Background: Optic nerve sheath (ONS) dilation indicates intracranial pressure elevation under clinical conditions but limited data exist with regard to the dynamics of sheath expansion.

Objective: To assess the time course of ONS widening and its stability under controlled pressure conditions in vitro.

Methods: Pre-defined pressure steps up to 65 mmHg were applied to the perineural space of ex-vivo human optic nerves (n = 16).

Optic Nerve Sheath Viscoelastic Properties: Re-Examination of Biomechanical Behavior and Clinical Implications.

Background: Meta-analyses show a variable relationship between optic nerve sheath diameter (ONSD) and the presence of raised intracranial pressure (ICP). Because optic nerve sheath (ONS) tissue can be deformed, it is possible that ONSD reflects not only the current ICP but also prior deforming biomechanical exposures. In this post hoc analysis of two published data sets, we characterize ONS Young's modulus (E, mechanical stress per unit of strain) and calculate threshold pressure for plastic deformation.

Persistence of post-operative color Doppler abnormalities is linked to reduced graft survival in pediatric patients after liver transplantation.

Color Doppler US is a readily available imaging modality for the evaluation of liver transplants. The aim of our study was to evaluate the temporal course of color Doppler US findings in children after LTX and to investigate the effect of resolving and persisting abnormalities during follow-up on long-term outcome. All children who underwent LTX during January 2000 until December 2003 (155 LTX in 137 patients, 75 male and 62 female; mean age at LTX 4.

Impact on the hepatic flow velocity after pediatric combined liver-kidney transplantation compared to isolated pediatric liver transplantation-A matched-pair analysis.

Background: Combined liver-kidney transplantation (CLKT) in children is still a rarely performed procedure. Our aim was to analyze the effect of the simultaneous transplantation of the kidney in pediatric CLKT on the liver graft flow velocity, and vascular complications compared to singular liver transplantation (LTX) in children.

Methods: All pediatric CLKT performed at our institution from 1998 to 2016 were matched with singular LTX and retrospectively analyzed.

Comparison of MAPIE versus MAP in patients with a poor response to preoperative chemotherapy for newly diagnosed high-grade osteosarcoma (EURAMOS-1): an open-label, international, randomised controlled trial.

Background: We designed the EURAMOS-1 trial to investigate whether intensified postoperative chemotherapy for patients whose tumour showed a poor response to preoperative chemotherapy (≥10% viable tumour) improved event-free survival in patients with high-grade osteosarcoma.

Methods: EURAMOS-1 was an open-label, international, phase 3 randomised, controlled trial. Consenting patients with newly diagnosed, resectable, high-grade osteosarcoma aged 40 years or younger were eligible for randomisation.

Methotrexate, Doxorubicin, and Cisplatin (MAP) Plus Maintenance Pegylated Interferon Alfa-2b Versus MAP Alone in Patients With Resectable High-Grade Osteosarcoma and Good Histologic Response to Preoperative MAP: First Results of the EURAMOS-1 Good Response Randomized Controlled Trial.

Purpose: EURAMOS-1, an international randomized controlled trial, investigated maintenance therapy with pegylated interferon alfa-2b (IFN-α-2b) in patients whose osteosarcoma showed good histologic response (good response) to induction chemotherapy.

Patients And Methods: At diagnosis, patients age ≤ 40 years with resectable high-grade osteosarcoma were registered. Eligibility after surgery for good response random assignment included ≥ two cycles of preoperative MAP (methotrexate, doxorubicin, and cisplatin), macroscopically complete surgery of primary tumor, < 10% viable tumor, and no disease progression.

Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.

In ARPKD, mutations in the PKHD1 gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long-term outcomes are scarce.

Transcapsular arterial neovascularization of liver transplants increases the risk of intraoperative bleeding during retransplantation.

Arterial neovascularization of liver grafts can be a source of significant blood loss during retransplantation. This study evaluated the effect of transcapsular arterial neovascularization on intraoperative blood loss during retransplantation and long-term follow-up. Eleven consecutive patients with transcapsular arterial neovascularization (seven male, four female; nine children, two adults; mean age 12.

Transcapsular arterial neovascularization after liver transplantation in pediatric patients indicates transplant failure.

Purpose: To identify transcapsular arterial neovascularization with Doppler ultrasonography (US) in pediatric patients after liver transplantation and to assess the frequency of the finding, its underlying causes, and its relevance in terms of clinical outcome.

Materials And Methods: The study was approved by the local ethics committee, with waived informed consent. All pediatric patients who underwent liver transplantation between January 2000 and December 2003 were retrospectively evaluated.

Dependence of the optic nerve sheath diameter on acutely applied subarachnoidal pressure - an experimental ultrasound study.

Purpose: To determine the distensibility and elastic characteristics of the optic nerve sheath for development of a basic understanding of ultrasound studies aimed to measure optic nerve sheath diameter (ONSD) for detection of acutely elevated intracranial pressure (ICP).

Methods: Isolated human optic nerves preparations obtained from autopsies were submitted to predefined pressure alterations, and consecutive changes in ONSD were measured by B-scan ultrasound under defined conditions.

Results: Following submission to pressure, the diameter of the nerve sheath increased up to 140% of its baseline value.

Liver transplantation in children using organs from young paediatric donors.

Nowadays, most paediatric liver transplant recipients receive a split or other technical variant graft from adult deceased or live donors, because of a lack of available age- and size matched paediatric donors. Few data are available, especially for liver grafts obtained from very young children (<6 years). We analysed all paediatric liver transplantations between 1989 and 2009.

Solitary skeletal osteosarcoma recurrence. Findings from the Cooperative Osteosarcoma Study Group.

Background: Solitary skeletal osteosarcoma (OS) manifestations distant from the site of the primary tumor rarely arise as only sign of disease recurrence.

Methods: This report reviews 38 patients with high-grade central OS of the limbs or axial skeleton and initial complete surgical remission (CR) who developed first recurrences as solitary osseous lesions distant from the primary tumor. The Cooperative Osteosarcoma Study Group (COSS) database was used to evaluate patient-, tumor-, and treatment-related variables and outcomes.

Value of ultrasound-guided percutaneous liver biopsy in children following liver transplantation.

Objectives: Pediatric liver transplant recipients often need to undergo liver biopsies for the detection and specification of complications such as acute or chronic graft rejection, infection, or drug toxicity. Complications resulting from liver biopsy are rare. The aim of our single-center retrospective study was to report on liver biopsy-related complications and, moreover, to assess the significance of histological findings in correlation with the suspected diagnosis.

Schimke immunoosseous dysplasia: defining skeletal features.

Schimke immunoosseous dysplasia (SIOD) is an autosomal recessive multisystem disorder characterized by prominent spondyloepiphyseal dysplasia, T cell deficiency, and focal segmental glomerulosclerosis. Biallelic mutations in swi/snf-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) are the only identified cause of SIOD, but approximately half of patients referred for molecular studies do not have detectable mutations in SMARCAL1. We hypothesized that skeletal features distinguish between those with or without SMARCAL1 mutations.

Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.

Background: Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment.

Second and subsequent recurrences of osteosarcoma: presentation, treatment, and outcomes of 249 consecutive cooperative osteosarcoma study group patients.

Purpose: To evaluate patient and tumor characteristics, treatment, and outcomes in a large cohort of unselected patients with second and subsequent recurrences of osteosarcoma.

Patients And Methods: Two hundred forty-nine consecutive patients who had originally received combined-modality therapy on neoadjuvant Cooperative Osteosarcoma Study Group protocols and went on to develop a total of 409 second and subsequent osteosarcoma recurrences were analyzed for patient-, tumor-, and treatment-related factors and outcomes.

Results: Five-year overall and event-free survival rates were 16% and 9% for 249 second, 14% and 0% for 93 third, 13% and 6% for 38 fourth, and 18% and 0% for 14 fifth recurrences, respectively.

Comparative study of portal vein embolization versus portal vein ligation for induction of hypertrophy of the future liver remnant using a mini-pig model.

Summary Background Data: The extent of hepatectomies is limited by the functional reserve of the remnant liver. The introduction of preoperative portal vein occlusion techniques to induce a preoperative hyperplasia of the future liver remnant has reduced the risk of postoperative liver failure. However, it has remained a matter of debate whether partial portal vein embolization (PVE) or suture ligation of the portal branches during exploration is the preferred technique.

Clinical relevance of autoantibodies after pediatric liver transplantation.

Background: The presence of autoantibodies and development of autoimmune hepatitis after liver transplantation has recently been reported as one of the causes for chronic graft dysfunction. The pathogenesis and clinical significance of this disease still remains unclear.

Methods: We evaluate 96 patients for the prevalence of autoantibodies and autoimmune hepatitis after pediatric liver transplantation and review their clinical follow-up including virus serologies, ultrasound examination and liver biopsies.

Diaphragmatic hernia resulting in enterothorax following pediatric liver transplantation: a rare complication.

Diaphragmatic hernia is a rare complication following solid organ transplantation. We here report three pediatric patients suffering from posttransplant enterothorax. One patient with biliary atresia presented with clinical signs of peritonitis without showing pulmonary symptoms four weeks following liver transplantation.

Technical refinements and results in full-right full-left splitting of the deceased donor liver.

Objective: Splitting of the liver at the line of Cantlie of otherwise healthy people is accepted worldwide as a reasonable procedure for the donors in adult living donor liver transplantation. A similar operation is still considered as experimental if performed in the deceased donor liver. The aim of this study is to evaluate the technical evolution and the results of this variant splitting technique.

Pediatric transplantation: the Hamburg experience.

Background: Since starting our program in 1989, 455 pediatric orthotopic liver transplantations have been performed using all techniques. In April 2001, we experienced our last in-hospital death of a pediatric liver-transplant recipient. Since then, all our liver-transplant children (n=170) were able to be discharged from the hospital.

Osteosarcoma relapse after combined modality therapy: an analysis of unselected patients in the Cooperative Osteosarcoma Study Group (COSS).

Purpose: To evaluate the impact of patient, tumor, and treatment-related factors on outcome in unselected patients with recurrent osteosarcoma.

Patients And Methods: Five hundred seventy-six consecutive patients who had achieved a first complete surgical remission (CR) during combined-modality therapy on neoadjuvant Cooperative Osteosarcoma Study Group (COSS) protocols and then developed recurrent osteosarcoma were analyzed (median time from biopsy to relapse, 1.6 years; range, 0.