Sonographic Image of Solitary Kidney in Wilms Tumour Survivors.

Background/aims: This study presents an analysis of the sonographic and laboratory parameters of solitary kidney in Wilms tumour survivors (TWs) and compares these parameters with those of healthy individuals.

Methods: Fifty-three TWs who completed treatment for Wilms tumour and 44 healthy individuals were enrolled. The study protocol consisted of completing a medical history, sonographic examination of the solitary kidney, estimation of glomerular filtration rate (eGFR) by the Schwartz or MDRD formulas, albumin urine excretion and BP measurement.

When do paediatric patients with familial hypercholesterolemia need statin therapy?

Introduction: Familial hypercholesterolemia (FH) is one of the most common autosomal dominant disorders. It is characterized by elevated LDL cholesterol levels occurring already by early childhood. Awareness of health risks in FH patients should incite health professionals to actively seek and treat children with lipid disorders to reduce their risk of myocardial infarction and stroke.

Coincidence of PTPN22 c.1858CC and FCRL3 -169CC genotypes as a biomarker of preserved residual β-cell function in children with type 1 diabetes.

Background: Genotype-phenotype studies in type 1 diabetes (T1DM) patients are needed for further development of therapy strategies.

Objective: Our aims were to investigate the distribution of selected PTPN22 and FCRL3 gene polymorphisms and their associations with clinical course of disease in children with newly diagnosed T1DM from the Pomeranian region of Poland.

Subjects/methods: The prospective, longitudinal study of 147 children with newly diagnosed T1DM-autoimmune subtype was conducted.

Increased μ-Calpain Activity in Blasts of Common B-Precursor Childhood Acute Lymphoblastic Leukemia Correlates with Their Lower Susceptibility to Apoptosis.

Childhood acute lymphoblastic leukemia (ALL) blasts are characterized by inhibited apoptosis promoting fast disease progress. It is known that in chronic lymphocytic and acute myeloid leukemias the reduced apoptosis is strongly related with the activity of calpain-calpastatin system (CCS) composed of cytoplasmic proteases--calpains--performing the modulatory proteolysis of key proteins involved in cell proliferation and apoptosis, and of their endogenous inhibitor--calpastatin. Here, the CCS protein abundance and activity was for the first time studied in childhood ALL blasts and in control bone marrow CD19+ B cells by semi-quantitative flow cytometry and western blotting of calpastatin fragments resulting from endogenous calpain activity.

First-line immunosuppressive treatment in children with aplastic anemia: rabbit antithymocyte globulin.

Immunosuppressive therapy is the treatment of choice in children with acquired severe aplastic anemia (AA) and no HLA-matched family donor. The paper presents results of a multicenter study of 63 children with AA treated with rabbit antithymocyte globulin (r-ATG) and cyclosporine A as the first line treatment in the years 1996-2012. Therapeutic effects were evaluated at Days 112, 180, and 360.

Systemic mastocytosis in children - therapeutic problems.

Systemic mastocytosis is a myeloproliferative disorder characterized by growth and accumulation of abnormal mast cells in one or more organs. The symptoms of the disease are due both to the mast cells infiltrating the organs and to the action of its degranulation products. Over 85% of adult patients exhibit point mutations of KIT at position 816 (D816V).

Serum IL-10 and IL-12 levels reflect the response to chemotherapy but are influenced by G-CSF therapy and sepsis in children with soft tissue sarcomas.

Introduction: Pre-treatment serum IL-10/IL-12 balance has been recently found deregulated in childhood soft tissue sarcomas (STS). Its role in STS monitoring and assessment of response to therapy is unknown.

Objective: To establish whether serum IL-10 and IL-12 levels and their reciprocal ratios reflect childhood STS course and actual activity and whether G-CSF therapy and central vein catheter (CVC)-related sepsis influence the interleukins levels.

Deregulated systemic IL-10/IL-12 balance in advanced and poor prognosis paediatric soft tissue sarcomas.

Context: The roles of interleukin 10 (IL-10) and IL-12 in regulation of cancer growth and Th1/Th2 immune responses towards cancer are unclear.

Objective: To establish the prognostic significance of serum IL-10 and IL-12 in paediatric soft tissue sarcomas (STS).

Materials And Methods: ELISA determinations of cytokines were performed as pre-treatment in 59 children with STS and 30 healthy controls.

Anemia secondary to valproic acid therapy in a 13-year-old boy: a case report.

Introduction: Valproic acid is a commonly used anti-epileptic drug. Hematological toxicities are among the occasionally observed adverse effects of this medication.

Case Presentation: We present the case of a 13-year-old Caucasian boy who demonstrated mild anemia 12 months after the introduction of valproic acid therapy.

Cytometric evaluation of transferrin receptor 1 (CD71) in childhood acute lymphoblastic leukemia.

Transferrin receptor 1 (CD71) is a transmembrane glycoprotein responsible for cellular iron uptake. Higher expression of CD71 has been identified as a negative prognostic marker for numerous solid tumor types and for some lymphomas. The aim of this study was to evaluate CD71 expression on acute lymphoblastic leukemia (ALL) cells and to follow its possible clinical correlations.

Renal function and low-molecular-weight proteins (cystatin C, β2-microglobulin, neutrophil gelatinase-associated lipocalin) in child and young adult cancer survivors.

Background: We sought to verify the hypothesis that children and young adults with cancer who have completed treatment differ according to the type and degree of renal damage.

Procedure: This study included 144 children and young adults (73 female) who had completed treatment for leukemias and lymphomas (group L, n=45), Wilms tumor (group W, n=52) and other solid tumors (group S, n=47). The following parameters were evaluated: serum concentrations of creatinine, cystatin C, β2-microglobulin, neutrophil gelatinase-associated lipocalin and urine excretion of albumin, and urinalysis with sediment.

Administration of CD4+CD25highCD127- regulatory T cells preserves β-cell function in type 1 diabetes in children.

Objective: Type 1 diabetes is a condition in which pancreatic islets are destroyed by self-reactive T cells. The process is facilitated by deficits in the number and suppressive activity of regulatory T cells (Tregs). Here, we show for the first time that the infusion of autologous Tregs prolongs remission in recently diagnosed type 1 diabetes in children.

Topotecan exposure estimation in pediatric acute myeloid leukemia supported by LC-MS-based drug monitoring and pharmacokinetic analysis.

Individualization of the topotecan dosing can reduce inter-patient variability, toxicity, and at the same time increases chemotherapy efficacy. Topotecan dosing based on simultaneous drug monitoring and pharmacokinetic analysis can yield more accurate and precise estimation of the topotecan systemic exposure than that attainable with the fixed dosing approach. Therefore, a combined approach could provide a tool assisting the clinicians in individualization of the topotecan dosing.

Association between intestinal and antioxidant barriers in children with cancer.

Objective: Reactive oxygen species (ROS) play a role in cancerogenesis processing and damage tissues. Furthermore, oncological treatment may impair proper function of the gut barrier. The aim of this study was to measure intestinal permeability in children in clinical remission for solid tumours and to search for a possible relationship between free radicals and the intestinal barrier.

Analysis of the impact of environmental and social factors, with a particular emphasis on education, on the level of metabolic control in type 1 diabetes in children.

Background: Type 1 diabetes is a chronic, incurable childhood disease. Chronically uncontrolled diabetes is associated with eye, kidney, nerve, heart and blood vessel damage and function impairment. The aim of this study was to evaluate the impact of various social and environmental factors, with a particular emphasis on education, on the level of metabolic control in diabetes.

Parameters of antioxidant barrier in different histopathologic types of pediatric cancers.

Aim Of The Study: The goal of this study was to evaluate the activities of erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px), and the levels of glutathione (GSH) and ischemia-modified albumin (IMA), as potential markers in different histopathologic types of pediatric neoplasms. No studies on this subject have been reported to date.

Material And Methods: SOD, GSH-Px, GSH, and IMA were measured before oncologic treatment in 129 children with neuroblastoma (NB), soft tissue sarcomas (STS), brain tumors, Hodgkin's disease (HD), and acute leukemias, and in 30 healthy controls.

Renal function and solitary kidney disease: Wilms tumour survivors versus patients with unilateral renal agenesis.

Aims: To test the hypothesis that Wilms tumour survivors (WTs) experience increased disturbance in renal function, even after prompt treatment, compared to patients with unilateral renal agenesis (URA).

Methods: To assess the renal function of 30 WTs and 17 individuals with URA, the estimated glomerular filtration rate (eGFR) was calculated using the Schwartz and Filler formulas as well as the new Schwartz equation for chronic kidney disease. To measure kidney damage, serum levels and urine excretion of β(2)-microglobulin (B2M), cystatin C (Cys C), neutrophil gelatinase-associated lipocalin (NGAL) were tested, N-acetyl-β-glucosaminidase (NAG), and albumin urine excretion and urine sediment were examined.

[Budd-Chiari syndrome in 16 year old girl with essential thrombocytosis].

Vein thrombosis is a rare disorder diagnosed in childhood. The principal causes of this illness are inborn thrombophilias, essential thrombocytosis, neoplasms, autoimmunologic diseases and pathologic reaction to certain drugs. Budd-Chiari syndrome, a hepatic vein occlusion is an atypical manifestation of vein thrombosis.

Methemoglobinemia in postchemotherapy stomatitis topical treatment: 2 pediatric cases.

Methemoglobinemia is a rare congenital or acquired disease of increased blood methemoglobin concentration. We documented 2 cases of children suffering from neuroblastoma whose postchemotherapy anemia, leucopenia, and stomatitis were complicated by methemoglobinemia after using a formulary oral gel (7.5% benzocaine, doxycycline, nystatin, glycerin).

[Characteristics of extracranial malignant germ cell tumours in two age groups of children (0-10 and 10-18 years). Multicentre experiences].

Unlabelled: In order to assess if any differences exist in children germ cell tumours depending on age, we compared some features of germ cell tumours in two age groups:younger than 10 and between 11 and 18 years.

Material And Methods: Data of 146 patients with germ cell tumours treated in 15 Polish paediatric oncology departments between 1995 and 2005 were evaluated. They were divided into two groups: 76 children 0-10 years old (group I) and 70 patients 11-18 years old (group II).

Ischemia-modified albumin as a biochemical marker in children with neuroblastoma and soft tissue sarcomas.

In this study, the levels of ischemia-modified albumin (IMA) in pediatric oncology patients with soft tissue sarcomas (STSs) and neuroblastoma (NB) were analyzed. To date, there have been no studies concerning IMA in these groups of patients. Ninety-nine children with STSs and NB were analyzed from 2006 to 2009, and 30 healthy children were also enrolled in the study.