There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population.

A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation.

Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measuroptimal timing and fixation constructs for those with MFS and other connective tissue diseases.

This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.Chylothorax is an unusual complication of childhood cancer. It causes to additional morbidity and mortality during management. It should be kept in mind that chylothorax may occur due to mass shrinkage during treatment in patients with mediastinal mass and ductus thoracicus invasion at the initial diagnosis and necessary measures should be taken. This case was presented because of the rarity in pediatric oncology practice.Implementation and adherence to consensus statement criteria for referral of pediatric cancer patients for genetic evaluation are critical to identify the 5% to 10% with a genetic cancer predisposition syndrome. The authors implemented a Plan-Do-Study-Act quality improvement initiative aimed at increasing referrals of at-risk patients. Retrospective chart review was followed by educational intervention-with impact assessed over a 9-month prospective chart review. Referral rate improved >2-fold and there was an improvement in documented oncologic history to at least a third-degree relative. The integration of quality improvement can be an effective tool to improve the referral of patients with an elevated risk for a cancer predisposition syndrome.

Low-molecular-weight heparin is cleared through the kidneys and is commonly used for anticoagulation in the pediatric population.

We present 3 challenging cases of children requiring anticoagulation in the context of acute kidney injury, nephrotic syndrome, and hemodialysis.

A significant change in anti-factor Xa (anti-Xa) levels-used for drug monitoring-should prompt an assessment of renal function. In nephrotic syndrome, anti-Xa levels should be closely monitored when there is a change in the status of nephrotic disease activity. In hemodialysis patients, enoxaparin at once daily reduced dosing should be considered with trough and peak anti-Xa levels monitoring.

A significant change in anti-factor Xa (anti-Xa) levels-used for drug monitoring-should prompt an assessment of renal function. In nephrotic syndrome, anti-Xa levels should be closely monitored when there is a change in the status of nephrotic disease activity. BC-2059 datasheet In hemodialysis patients, enoxaparin at once daily reduced dosing should be considered with trough and peak anti-Xa levels monitoring.

This study was concerned with whether vWF (von Willebrand factor) and a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 (ADAMTS13) has altered in patients with cirrhosis and extrahepatic portal hypertension (EPH). We aimed to investigate changes to vWF and ADAMTS13 in children with cirrhosis and EPH.

This study was conducted between January and October 2019 with both cirrhosis and EPH patients and with healthy volunteers. The von Willebrand factor antigen (vWFAg), von Willebrand Ristocetin cofactor (vWFRCo), and ADAMTS13 antigen and activity were studied.

Twenty-eight children with cirrhosis, 16 children with EPH, and 20 healthy controls were included in the study. vWFAg and vWFRCo levels were higher in patients with cirrhosis than in healthy controls (171.65±101.67 vs. 85.86±30.58, P<0.01 and 121.62±55.83 vs. 61.52±27.03, P<0.01, respectively). vWFAg and vWFRCo levels were higher in patients with EPH than in healthy controls (133.93±80.13 vs. 85.86±30.58, P<0.01 a cirrhosis.Influenza virus can trigger atypical hemolytic uremic syndrome and present with complement-driven thrombotic microangiopathy (TMA). When administered promptly, complement-blocking therapies can spare organ injury and be lifesaving. However, diagnosing TMA in the setting of a severe viral infection can be challenging, as a significant overlap of symptoms and disease complications exists. This is particularly true in influenza virus infections and more recently, Coronavirus disease 2019 (COVID-19) infections. We present a 16-year-old male with H1N1 influenza-induced atypical hemolytic uremic syndrome who quickly improved with complement-blocking therapy, highlighting an urgent need to include TMA in the differential diagnosis of severe viral infections.The Coronavirus Disease 2019 (COVID-19) pandemic has become the worst pandemic in modern history. The lack of prior immunity to the virus has resulted in a high mortality rate, though children have fared better than adults, overall. We present a case of a child who developed B-cell acute lymphoblastic leukemia 1 week following a symptomatic COVID-19 infection. It is possible that this viral infection provided the “second hit” posited to occur in pediatric leukemogenesis as proposed by Dr Greaves, with his initial viral exposure occurring several weeks earlier.