Assessment of plasminogen activator inhibitor-1(PAI1) and thrombin activitable fibrinolysis inhibitor (TAFI) in Egyptian children with hemophilia A.

Patients with hemophilia A display varied bleeding phenotypes not correlated with degree of deficiency of factor VIII level. We investigated Plasminogen Activator Inhibitor 1(PAI1) level and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) also known as Carboxypeptidase B2 (CPB2) level in Patients with hemophilia A and their possible correlation with bleeding tendency. Twenty-six patients attending in hematology unit of pediatric department were included in this study. In addition, fourteen apparently healthy subjects matched ages and genders were included as control group. The International Society of Thrombosis Bleeding Assessment Tool (ISTH/BAT) was used to assess bleeding score in patients. Plasma levels of Plasminogen Activator Fibrinolysis Inhibitor (PAI1) and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) zymogen were measured by enzyme-linked immunosorbent assay (ELIZA). As compared to controls, hemophilic patients had significantly high bleeding score, low PAI 1 level and high TAFI level. There was no significant correlation between bleeding score by ISTH/BAT and patient severity. PAI 1 and TAFI level have no significant correlation with patient severity. PAI 1 level was statistically significant different between intense and non-intense hemorrhagic groups, while TAFI level has no significant correlation with bleeding phenotype. PAI 1 and TAFI levels had significantly correlation between patients and controls. PAI-1 level had statistically significant correlation with bleeding phenotype, while TAFI level failed to show any correlation between intense and non-intense hemorrhagic groups. So, PAI-1 levels may have predictive value of bleeding tendency in hemophiliacs.

Changes and correlations of T-cell coinhibitory molecule programmed death-1 and interferon-γ in pediatric immune thrombocytopenia.

BACKGROUND: Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by abnormalities of T cells subsets. Programmed death-1 (PD-1) is a co-signaling inhibitory molecule in T cells that is involved in many autoimmune diseases. PURPOSE: Here we aimed to measure changes in PD-1 expression and serum interferon-γ (IFN-γ) levels before and 1 month after treatment in pediatric patients with newly diagnosed ITP. METHODS: We measured PD-1+ CD4+ T cells percentages using flow cytometry and the serum IFN-γ levels by enzyme-linked immunosorbent assay in 40 pediatric patients with ITP and 20 healthy controls. RESULTS: Compared with healthy controls, the PD-1+ CD4+ T cells percentages and serum IFN-γ levels were significantly higher in ITP patients before and 1 month after therapy. A correlation study revealed that PD-1+ CD4+ T cells percentage was negatively associated with platelet count and positively associated with IFN-γ level in patients with ITP. Furthermore, serum IFN-γ levels were significantly decreased in patients after treatment, but no significant change was detected in the percentage of PD-1+ CD4+ T cells before or 1 month after therapy. CONCLUSION: PD-1+ CD4+ T cells expression and IFN-γ levels were increased in patients with ITP. These preliminary data suggest a potential role of PD-1+ CD4+ T cells as mediators of ITP. We also found a correlation between PD-1+ CD4+ T cells and both platelet counts and IFN-γ levels. These findings suggest a potential role of PD-1+ CD4+ T cells and IFN-γ in the pathogenesis of ITP. Further studies investigating PD-1 expression in different T-cell subsets, serum IFN-γ concentrations, and antiplatelet antibodies levels over a longer duration after therapy initiation could delineate the precise role of PD-1 in ITP pathogenesis. Consequently, novel nontraditional therapeutic strategies for ITP patients may become available.

Bone mineral density in children with idiopathic nephrotic syndrome.

OBJECTIVES: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). METHODS: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex. All of the participants were subjected to complete history taking, thorough clinical examination, laboratory investigations (serum creatinine, blood urea nitrogen [BUN], phosphorus [P], total and ionized calcium [Ca], parathyroid hormone [PTH], and alkaline phosphatase [ALP]). Bone mineral density was measured at the lumbar spinal region (L2-L4) in patients group using dual-energy X-ray absorptiometry (DXA). RESULTS: Total and ionized Ca were significantly lower while, serum P, ALP, and PTH were higher in SSNS and SRNS cases than the controls. Osteopenia was documented by DXA scan in 11 patients (44%) and osteoporosis in two patients (8%). Fracture risk was mild in six (24%), moderate in two (8%), and marked in three (12%) of patients. CONCLUSION: Bone mineralization was negatively affected by steroid treatment in children with NS.

Study of CD4+, CD8+, and natural killer cells (CD16+, CD56+) in children with immune thrombocytopenic purpura.

OBJECTIVE/BACKGROUND: To assess the percentage of CD4+, CD8+, and natural killer cells (CD16+, CD56+) in children with immune thrombocytopenic purpura (ITP) at presentation and study their impact on disease chronicity. METHODS: This case-control study was conducted at the Pediatric Hematology and Oncology Unit, Menoufia University Hospital (tertiary care center in Egypt). The study was held on 30 children presenting with ITP; they were followed-up and classified into two groups: 15 children with acute ITP; and 15 children with chronic ITP. Patients were compared to a group of 15 healthy children of matched age and sex. Measurements of CD4+, CD8+, and natural killer cells (CD16+, CD56+) by flow cytometry were assessed and compared in these groups. RESULTS: CD4+ and CD4+/CD8+ were significantly lower in acute and chronic patients than the control group (p<0.05 and p<0.001, respectively), with no significant difference between acute and chronic patients (p>0.05). However, CD8+ was significantly higher in acute and chronic patients than the control group (p<0.05), with no significant difference between acute and chronic patients (p>0.05). Natural killer cell percent was significantly lower in acute patients than the control group (p<0.001), with no significant difference between chronic and control groups (p>0.05). CONCLUSION: ITP is associated with immunity dysfunction denoted by the increase in cytotoxic T lymphocytes and the decrease in natural killer cells.

Bone mineral density in children with idiopathic nephrotic syndrome / Densidade mineral óssea em crianças com síndrome nefrótica idiopática

Abstract Objectives: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). Methods: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex. All of the participants were subjected to complete history taking, thorough clinical examination, laboratory investigations (serum creatinine, blood urea nitrogen [BUN], phosphorus [P], total and ionized calcium [Ca], parathyroid hormone [PTH], and alkaline phosphatase [ALP]). Bone mineral density was measured at the lumbar spinal region (L2-L4) in patients group using dual-energy X-ray absorptiometry (DXA). Results: Total and ionized Ca were significantly lower while, serum P, ALP, and PTH were higher in SSNS and SRNS cases than the controls. Osteopenia was documented by DXA scan in 11 patients (44%) and osteoporosis in two patients (8%). Fracture risk was mild in six (24%), moderate in two (8%), and marked in three (12%) of patients. Conclusion: Bone mineralization was negatively affected by steroid treatment in children with NS.

Resumo Objetivos: Avaliar a densidade mineral óssea (DMO) em crianças com síndrome nefrótica idiopática (SNI) e com taxa de filtração glomerular (TFG) normal. Métodos: O estudo transversal de caso-controle foi feito com 50 crianças: 25 casos de SNI [16 sensíveis a esteroides (SNSE) e nove resistentes a esteroides (SNRE) com acompanhamento na unidade de nefrologia pediátrica do hospital da Menoufia University, centro de cuidados terciário] foram comparados com 25 controles saudáveis do grupo de controle com idade e sexo equivalentes. Todos os participantes foram submetidos a anamnese completa, exame clínico completo, exames laboratoriais [creatinina sérica, nitrogênio ureico no sangue (BUN), fósforo (P), cálcio (Ca) total e ionizado, paratormônio (PTH) e fosfatase alcalina (ALP)]. A densidade mineral óssea foi mensurada na região da coluna lombar (L2-L4) no grupo de pacientes com a absorciometria por raios X de dupla energia (DXA). Resultados: Os níveis de cálcio total e ionizado eram significativamente menores, ao passo que o fósforo sérico, a FA e o PTH eram maiores em casos de SNSE e SNRE do que nos controles. A osteopenia foi documentada pelo exame DXA em 11 pacientes (44%) e a osteoporose em dois (8%). O risco de fratura era leve em seis (24%), moderado em dois (8%) e acentuado em três (12%). Conclusão: A mineralização dos ossos foi afetada negativamente pelo tratamento com esteroides em crianças com SNI.