Results of a long-term, prospective study on complications of central venous catheter in pediatric patients with hematologic-oncologic diseases.

BACKGROUND: Central venous catheter (CVC)-related complications remain a significant cause of morbidity in pediatric hematology-oncology. We prospectively surveyed the incidence of CVC-related complications in children with hematologic-oncologic diseases. PROCEDURE: Five-hundred-eighty-one CVCs were inserted in 421 patients from January 2010 to June 2022 (153,731 CVC days observation; follow-up data up to December 31, 2022). RESULTS: Overall, 671 complications were recorded (4.365/1000 CVC days): 49.7% malfunctions (1.88/1000 CVC days, 4.8% of CVC early removals), 23.9% bacteremia (0.90/1000, 15.1%), 19.6% mechanical complications (0.74/1000, 70.2%), 20.1% localized infections (0.76/1000, 17.1%), 0.5% thrombosis (0.02/1000, 33.3%). At multivariate analysis, risk factors for malfunction were Broviac-Hickman type of CVC (hazard ratio [HR] 2.5) or Port-a-cath (HR 3.4) or Proline (HR 4.3), p < .0001; for bacteremia double-lumen CVC (HR 3.2, p < .0001); for mechanical complications age at CVC insertion under median (HR 4.5, p < .0001) and Broviac-Hickman (HR 1.6) or Proline (HR 2.7), p = .01; finally for localized infections Broviac-Hickman (HR 2.9) or Proline (HR 4.4), p = .0001. The 2-year cumulative incidence of premature removal was 23.5%, and risk factors were age at CVC insertion under median (HR 2.4, p < .0001), Broviac-Hickman (HR 2.3) or Proline (HR 4.2), p < .0001. CONCLUSIONS: Premature removal occurs in approximately 20%-25% of long-term CVCs. A surveillance program has a fundamental role in identifying the risk factors for CVC complications and the areas of intervention to improve CVC management.

Pros and Cons of Current Diagnostic Tools for Risk-Based Screening of Prediabetes and Type 2 Diabetes in Children and Adolescents with Overweight or Obesity.

It is still a matter of debate which is the most suitable diagnostic test for risk-based screening of prediabetes and type 2 diabetes (T2D) in children and adolescents with overweight or obesity. This review highlighted benefits and pitfalls of currently recommended screening tools (fasting plasma glucose [FPG], oral glucose tolerance test [OGTT], glycated hemoglobin A1c [HbA1c]). The greatest advantage of OGTT is the ability to detect people with impaired glucose tolerance, a subcategory at high risk of developing both T2D and cardiovascular disease. Important disadvantages are low reproducibility and inconvenience. FPG measurement is more practical, as it needs only one blood draw, although both OGTT and FPG require fasting. The reliability of HbA1c as a screening tool has been questioned, especially in children and adolescents, but its undeniable convenience can lead to increased adherence to screening. In contrast, it can be altered by several nonglycemic factors. Importantly, none of these tests have been validated in the pediatric population. Alternative tests have been recently proposed, including new OGTT-derived biomarkers and additional nonfasting glycemic markers. In conclusion, when choosing the most suitable test in clinical practice, advantages and disadvantages should be considered, as well as the possibility of performing different tests at once.

Impact of Fat Intake on Blood Glucose Control and Cardiovascular Risk Factors in Children and Adolescents with Type 1 Diabetes.

Nutrition therapy is a cornerstone of type 1 diabetes (T1D) management. Glycemic control is affected by diet composition, which can contribute to the development of diabetes complications. However, the specific role of macronutrients is still debated, particularly fat intake. This review aims at assessing the relationship between fat intake and glycemic control, cardiovascular risk factors, inflammation, and microbiota, in children and adolescents with T1D. High fat meals are followed by delayed and prolonged hyperglycemia and higher glycated hemoglobin A1c levels have been frequently reported in individuals with T1D consuming high amounts of fat. High fat intake has also been associated with increased cardiovascular risk, which is higher in people with diabetes than in healthy subjects. Finally, high fat meals lead to postprandial pro-inflammatory responses through different mechanisms, including gut microbiota modifications. Different fatty acids were proposed to have a specific role in metabolic regulation, however, further investigation is still necessary. In conclusion, available evidence suggests that a high fat intake should be avoided by children and adolescents with T1D, who should be encouraged to adhere to a healthy and balanced diet, as suggested by ISPAD and ADA recommendations. This nutritional choice might be beneficial for reducing cardiovascular risk and inflammation.

Emapalumab for adult and pediatric patients with hemophagocytic lymphohistiocytosis.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome. Standard treatment is based on immunosuppressive, cytotoxic drugs and hematopoietic stem cell transplantation (HSCT) in primary HLH. Interferon-gamma (IFN-γ) plays a key pathogenic role. Emapalumab, a monoclonal antibody directed against IFN-γ, is the first target therapy approved for primary HLH with refractory, recurrent or progressive disease or intolerance to conventional therapy. AREAS COVERED: We reviewed the pharmacological characteristics, safety, efficacy and clinical uses of emapalumab. We summarized the results of current standard treatment based on chemo-immunosuppressive protocols and outlined the alternative options available. EXPERT OPINION: Emapalumab is an effective treatment for HLH with a good safety profile. Its efficacy was demonstrated in a phase II/III study on primary HLH pediatric patients with refractory, relapsing HLH or intolerance to first-line treatment. The use of emapalumab allowed most patients to proceed to HSCT, with a high estimated probability of survival 12 months after transplantation. The outcomes in patients who underwent transplantation compare favorably with those reported previously with either myeloablative or reduced-intensity conditioning regimens. The potential role of emapalumab in the treatment of secondary HLH and as a prevention of graft failure after HSCT deserves to be further assessed.

Long-term and quality of survival in patients treated for acute lymphoblastic leukemia during the pediatric age.

Long-term survival for acute lymphoblastic leukemia (ALL) in children improved over the last three decades up to 80-90% of affected patients. Consequently, the quality of life of survivors has become increasingly important. This study analyses the clinical features and outcome of 119 children with ALL, focusing on the quality of long-term survival in a subset of 22 patients over 18 years of age. Among this group, the 10-year event-free survival and overall survival were 83.1% (C.I. 74.0-89.2) and 88.4% (C.I. 80.9-93.1), respectively. Treatment related long-term medical complications were reported only in 2 patients (9.1%). Secondary school was completed successfully in 20 of 22 patients (89.9%). The remaining 2 patients were still attending at the time of the analysis. In conclusion, current treatment for ALL is well tolerated and does not compromise significantly the quality of life of survivors.

The Impact of Chemotherapy after Pediatric Malignancy on Humoral Immunity to Vaccine-Preventable Diseases.

BACKGROUND/AIM: The antibody titer of vaccine-preventable diseases in pediatric patients who underwent chemotherapy was assessed in order to evaluate the seroprotection after treatment and the feasibility and the efficacy of a policy of revaccination. METHODS: Serum antibody titers of 55 patients for hepatitis B (HBV), rubella, varicella-zoster (VZV), measles, mumps, polio viruses, Clostridium tetani (C. tetani) and Streptococcus pneumoniae (S. pneumoniae) were analysed.Results: After chemotherapy, a lack of protective antibody titers against HBV, rubella, VZV, measles, mumps, polio viruses, C. tetani, and S. pneumoniae was found in 53%, 45%, 46%, 46%, 43%, 21-26%, 88% and 55% of patients, respectively. In 49 of 55 patients who were tested both before and after chemotherapy for at least a pathogen, the loss of immunity for HBV, rubella, VZV, measles, mumps, polio viruses and C. tetani was respectively 39%, 43%, 38%, 42%, 32%, 33%, and 80%. A low number of B-lymphocytes was associated with the loss of immunity against measles (p=0.04) whereas a high number of CD8+ T-lymphocytes was associated with the loss of immunity against VZV (p=0.03). A single booster of vaccine dose resulted in a seroprotection for HBV, rubella, VZV, measles, mumps, polio viruses, C. tetani and S. pneumoniae in 67%, 83%, 80%, 67%, 33%, 100%, 88% and 67% of patients, respectively. CONCLUSIONS: We confirm that seroprotection for vaccine-preventable diseases is affected by treatment for pediatric malignancy. A single booster dose of vaccine might be a practical way to restore vaccine immunity in patients after chemotherapy.