Parenteral Nutrition in the Pediatric Oncologic Population: Are There Any Sex Differences?

Gender-based medicine is attracting increasing interest every day, but studies on pediatric populations are still limited. In this setting, sex differences among patients undergoing total parenteral nutrition (TPN) have not been previously reported. This study investigated the presence of sex differences in parenteral nutrition composition and outcomes among a cohort of pediatric patients admitted at the Oncohematology and Bone Marrow Transplant Unit of the Institute for Maternal and Child Health "Burlo Garofolo" of Trieste, Italy. For all 145 recruited patients (87 males, 58 females), the following data were collected: age, sex, volume and duration of TPN, macro- and micronutrient composition of TPN bags, electrolytic or blood gases imbalance, glycolipid alterations, liver damage during TPN, and the incidence of sepsis and thrombosis. The analysis showed that females required higher daily phosphate intake (p = 0.054) and essential amino acid supplementation (p = 0.07), while males had a higher incidence of hypertriglyceridemia (p < 0.05) and cholestasis. A higher incidence of sepsis was found in the non-transplanted male population (p < 0.05). No significant differences were appreciable in other analyzed variables. This study aims to create a basis for future gender-based nutritional recommendations in the pediatric field

Zero incidence of factor VIII inhibitors and successful haemostatic response in previously factor VIII-treated patients with haemophilia A switching to turoctocog alfa in a noninterventional study

Introduction: Turoctocog alfa (NovoEight®) is a B-domain-truncated recombinant factor VIII (FVIII) approved for patients with haemophilia A. Aim: To investigate the long-term safety and efficacy of turoctocog alfa in routine clinical practice. Methods: Guardian 5 was a prospective, multinational, non-interventional, post-authorisation safety study. Male previously treated patients (> 150 exposure days [EDs]) of any age with severe/moderately severe haemophilia A (FVIII ≤ 2%) and a negative inhibitor test prior to first dosing (independent of FVIII-inhibitor history) were included to receive prophylaxis or on-demand treatment. The primary endpoint was the proportion of patients developing FVIII inhibitors (≥.6 Bethesda Units [BU]) after baseline visit, measured as per routine practice of each study site during clinic visits. Secondary endpoints included haemostatic effect, annualised bleeding rate (ABR), and adverse reactions assessment. The study concluded when 50 patients reached 100 EDs/patient minimum. Results: Seventy patients were screened and 68 exposed to turoctocog alfa; 63 (92.6%) were on prophylaxis and five received on-demand treatment. Six (8.8%) patients reported a history of positive inhibitors. During the study, patients were exposed to turoctocog alfa for a mean (standard deviation) of 131.9 (99.0) days/patient. Fifty-five of 58 patients who completed the study were tested for FVIII inhibitors; no positive tests were reported. Overall success rate of turoctocog alfa for treatment of bleeds was 87.3%. Among patients receiving prophylaxis, median (range) ABR was 1.97 (.0–25.5) bleeds/year; estimated ABR (negative binomial model) was 3.65 (95% confidence interval: 2.53–5.25). Conclusion: Turoctocog alfa was safe and efficacious for haemophilia A treatment in routine clinical practice

SPINAL EPIDURAL HAEMATOMA WITHOUT SIGNIFICANT TRAUMA IN A CHILD

Spinal epidural haematoma without significant precedent trauma is an emergency medical condition, which is rarely encountered in paediatrics. The diagnosis is made by clinical examination and imaging. Treatment is generally surgical. In this paper, we present the clinical picture and treatment of spinal epidural haematoma in an eleven-year-old previously healthy boy, who complained of sudden pain in the right shoulder blade area. Clinically, we suspected spinal pathology, which was eventually proven by magnetic resonance imaging. After surgical evacuation of the haematoma, the boy made a complete recovery

SPINALNI EPIDURALNI HEMATOM BREZ POMEMBNE POŠKODBE PRI OTROKU

Spinalni epiduralni hematom brez pomembne predhodne poškodbe je nujno stanje, s katerim se v pediatriji srečamo zelo redko. Diagnozo postavimo s kliničnim pregledom in slikovno preiskavo. Zdravljenje je večinoma kirurško. V prispevku predstavljamo spinalni epiduralni hematom pri enajstletnem, do tedaj zdravem dečku, ki je nenadoma začutil bolečino v predelu desne lopatice. Po kliničnem pregledu smo sum na patološko dogajanje v predelu hrbtenice potrdili z magnetnoresonančnim slikanjem. Po kirurški evakuaciji hematoma je deček popolnoma okreval

The influence of folate pathway polymorphisms on high-dose methotrexaterelated toxicity and survival in children with non-Hodgkin malignant lymphoma

Background. We evaluated the influence of folate pathway polymorphisms on high-dose methotrexate (HD-MTX) related toxicity in paediatric patients with T-cell non-Hodgkin lymphoma (NHL). Patients and methods. In total, 30 NHL patients were genotyped for selected folate pathway polymorphisms

Association between SLC19A1 gene polymorphism and high dose methotrexate toxicity in childhood acute lymphoblastic leukaemia and non Hodgkin malignant lymphoma: introducing a haplotype based approach

We investigated the clinical relevance of SLC 19A1 genetic variability for high dose methotrexate (HD-MTX) related toxicities in children and adolescents with acute lymphoblastic leukaemia (ALL) and non Hodgkin malignant lymphoma (NHML)

HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH HAEMOPHILIA AND ITS ASSOCIATION WITH DEPRESSIVE SYMPTOMS: A STUDY IN CROATIA AND SLOVENIA

Background: There are only a few studies in patients with haemophilia (PWH) that examined both quality of life and depressive symptoms, with only few studies examining their association. Aim of this study was to examine the association between depressive symptoms and health-related quality of life (HRQoL) in PWH from Croatia and Slovenia. Subjects and methods: A total of 112 adult PWH on prophylactic (73%) or on-demand (27%) treatment were included in the study (median age 46 years, range 18-73 years). Depressive symptoms were assessed with BDI-II, HRQoL with SF-36v2, demographic and socioeconomic data were collected using a questionnaire, and clinical data were obtained from medical records. Results: All HRQoL scores were significantly negatively correlated with BDI-II in the -0.42 to -0.70 range (all p<0.05). Sociodemographic and clinical variables explained 28-51% of HRQoL variance scores. Depressive symptoms explained additional variance for six HRQoL domain scores, with incremental variance being larger for mental domain scores (ranging between 10- 27%), and for Mental Component Summary score (26%). Conclusions: This study’s findings support that having depressive symptoms is associated with HRQoL of PWH, more so in the mental health than in the physical health domains