Acute renal failure in a child with thrombocytopenic purpura caused by acute epstein-barr virus infection after treatment with anti-D immunoglobulin

Immune thrombocytopenia (ITP) in children is usually a benign, self-limiting disorder. An acute Epstein-Barr virus (EBV) infection usually causes atypical lymphocytosis and mild decrease in platelets. Severe thrombocytopenia is an extremely rare complication. Anti-D immunoglobulin has been used for treatment of ITP in Rh(D)-positive nonsplenectomized patients. Severe hemolysis and acute renal failure are extremely rare complications that may be aggravated by the presence of an acute EBV infection. It is believed that anti-D immunoglobulin triggers an unusual virus-induced immune response causing hemolysis. We present a 4-year-old girl with ITP caused by an acute EBV infection that developed acute kidney injury following treatment with anti-D immunoglobulin. The patient recovered completely from thrombocytopenia and renal dysfunction. Intravascular hemolysis and acute kidney injury are consistent with anti-D immunoglobulin mechanism of action. Pediatric patients treated with anti-D immunoglobulin for ITP should be closely monitored for signs and symptoms of hemolysis that may be aggravated by the presence of EBV infection leading to impaired renal function. Copyright © 2013 Lippincott Williams & Wilkins

Post-transfusion changes in serum hepcidin and iron parameters in preterm infants

Background: Packed red blood cell transfusion is common in preterm neonates. Hepcidin acts as a negative feedback iron regulator. Iron parameters such as immature reticulocyte fraction (IRF) and high-light-scatter reticulocytes (HLR) are used to clarify iron metabolism. Very little is known about the regulation of hepcidin in preterm infants because most reports have evaluated prohepcidin. The aim of this study was therefore to evaluate serum hepcidin and establish hematological parameters in preterm infants after transfusion. Methods: The subjects consisted of 19 newborns (10 boys) with mean gestational age 29.1 ± 2.0 weeks, who had been transfused at the chronological age of 44.84 ± 19.61 days. Blood sample was collected before the transfusion and thereafter at 5 days and at 1 month. Serum hepcidin and other iron parameters were evaluated. Results: Mean serum hepcidin before and 5 days after transfusion was significantly different (5.5 ± 5.1 vs 10 ± 7.9 ng/mL respectively, P = 0.005). IRF and % HLR were also decreased significantly, 5 days after transfusion (0.4 ± 0.2 vs 0.2 ± 0.1, P = 0.009; 1.4 ± 1.5% vs 0.5 ± 0.4%, P = 0.012, respectively). Changes in hepcidin 5 days after transfusion were correlated significantly with changes in mean corpuscular hemoglobin (β, 0.13; SE, 0.05; P = 0.017), total iron binding capacity (β, 3.74; SE, 1.56; P = 0.016) and transferrin (β, 2.9, SE, 1.4; P = 0.039). Conclusions: Serum hepcidin concentration, along with IRF and HLR, are potentially useful in estimating pre- and post-transfusion iron status. Larger studies are needed to evaluate the sensitivity and specificity of hepcidin compared with ordinary iron parameters in premature infants. © 2017 Japan Pediatric Societ

Increased levels of Dickkopf-1 are indicative of Wnt/beta-catenin downregulation and lower osteoblast signaling in children and adolescents with type 1 diabetes mellitus, contributing to lower bone mineral density

Higher levels of Dickkopf-1, which is an inhibitor of Wnt/beta-catenin bone metabolic pathway, could be indicative of downregulated Wnt system, with possible lower osteoblast activation and higher osteoclast signaling in type 1 diabetes mellitus children and adolescents. Dickkopf-1 could significantly contribute to diabetes osteopathy. Increased fracture risk and elevated Dickkopf-1 levels, which is an inhibitor of Wnt/beta-catenin bone metabolic pathway, have been documented in adult patients with type 2 diabetes mellitus (T2D), while no relevant data exist on childhood type 1 diabetes (T1D). Our aim was to study plasma Dickkopf-1 distribution in children and adolescents with T1D and to correlate Dickkopf-1 with metabolic bone markers and bone mineral density (BMD). We evaluated 40 children and adolescents with T1D (mean +/- SD age 13.04 +/- 3.53 years, T1D duration 5.15 +/- 3.33 years) and 40 healthy age-matched and gender-matched controls (age 12.99 +/- 3.3 years). Dickkopf-1 and bone metabolic markers were measured, while total body and lumbar spine BMD were evaluated with dual-energy X-ray absorptiometry (DXA). Dickkopf-1 demonstrated a Gaussian distribution, with higher levels in T1D patients (13.56 +/- 5.34 vs 11.35 +/- 3.76 pmol/L, p = 0.024). Higher values were found in boys and in prepubertal children. Dickkopf-1 correlated positively with osteoprotegerin and fasting glucose in patients, while positive correlation with sclerostin and total soluble receptor activator of nuclear factor-kappaB ligand (s-RANKL) was found in controls. Positive correlations with C-telopeptide cross-links (CTX), osteocalcin, alkaline phosphatase, phosphate, and insulin-like growth factor 1 (IGF1) were documented in both groups. Lumbar spine Z-score was positively associated with Dickkopf-1 in controls, while a negative trend was found in patients. Higher levels of Dickkopf-1 could indicate a downregulated Wnt/beta-catenin system with possible lower osteoblast activation and higher osteoclast signaling in T1D children and adolescents. Dickkopf-1 could possibly be a significant contributor of T1D osteopathy. Future therapies could focus on Wnt/beta-catenin metabolic pathway

Clinical and morphological features of paediatric myelodysplastic syndromes: a review of 34 cases

Background: The clinical and morphological spectrum of myelodysplastic syndromes (MDS) during childhood has not yet been completely documented. We herein present the clinical features and morphological data from peripheral blood (PB), bone marrow aspirates (BMA) and bone marrow biopsies (BMB) of a series of paediatric MDS patients, with particular emphasis on their specific morphological characteristics and their diverse underlying genetic background. Patients and methods: Thirty-four patients with MDS (median age 8.45 y) were consecutively diagnosed and treated during a period of 15 y (1988-2002). Diagnosis was based on clinical manifestations, morphology of PB, BMA and BMB, and cytogenetic analysis of BM cells. Clonogenic methylcellulose cell cultures were performed in 23/34 patients. Patients were categorized into group A [26 primary/de novo MDS, i.e. refractory anaemia (RA) 18, RA with excess of blasts (RAEB) 2, RAEB in transformation (RAEB-t) 6] and group B (8 secondary MDS, i.e. RA 4, RAEB 1, RAEB-t 3). Treatment options varied according to protocols active during the period of the study and the availability of a suitable BM donor. Survival probabilities were estimated using the Kaplan-Meier method. Results: Dysplastic features of the erythroid, myeloid and megakaryocytic lineage were detected at BMA in 85%, 50% and 90% of the patients, respectively, while decreased cellularity was found at BMB in 21/34 patients (60%). RA patients of group A presented at BMB significant hypocellularity (14/18) as a prominent finding due to decrease of the myeloid (13/18 patients) and/or the megakaryocytic (14/18 patients) lineage. Hypocellularity in RA was accompanied by dysplasia of the erythroid (17/18 patients) and megakaryocytic (16/18 patients) lineage, the presence of abnormal localization of immature precursors (ALIP, 8/18 patients), fibrosis (5/18) and stromal changes (11/18). Chromosomal aberrations were revealed in 17/34 patients, of which monosomy 7 was present in seven. Cell cultures demonstrated abnormal myeloid and/or erythroid in vitro clonal growth pattern in all the examined patients. An associated disorder or inherited disease, was identified in 14/26 patients (54%) with primary MDS. Cumulative survival of group A patients was 44.2% (RA 66.6%, RAEB/RAEBt 14.6%; p = 0.001), and of the whole group 42.4%, at 14 y. Conclusions: Hypocellularity of significant degree is a constant and prominent feature among paediatric MDS, especially those with RA. A large variety of associated disorders underlies the clinical appearance of paediatric MDS, reflecting their marked heterogeneity. RA represents the prominent subtype during childhood (69% in this study), and it appears to have the best prognosis, while prognosis of RAEB/RAEBt remains extremely poor

Plant sterols-enriched diet decreases small, dense LDL-cholesterol levels in children with hypercholesterolemia: A prospective study

Background: Small dense low density lipoprotein-cholesterol (sdLDL-C) molecules are more atherogenic compared with large buoyant ones. Phytosterols-enriched diets are effective in decreasing total cholesterol (TC) and low density lipoprotein-cholesterol (LDL-C) concentrations in hyperlipidemic children without significant adverse effects. Limited data on the impact of such a diet on sdLDL-C levels is available in adults while there are no reports concerning children. The purpose of this study is to prospectively evaluate the effect of the daily consumption of 2 g of plant sterols on sdLDL-C levels in children with hypercholesterolemia. Methods. Fifty-nine children, 25 with LDL-C ≥ 3.4 mmol/l (130 mg/dl) and 34 with LDL-C < 3.4 mmol/l, aged 4.5-15.9 years, were included in the study. A yogurt-drink enriched with 2 g of plant sterols was added to the daily diet of hypercholesterolemic children and 6-12 months later lipid profiles were reassessed. Direct quantitative methods were used to measure LDL-C and sdLDL-C levels. Results: The consumption of plant sterols reduced sdLDL-C significantly (p < 0.001), but levels remained higher compared with controls (p < 0.001). TC, LDL-C, non high density lipoprotein-cholesterol (NonHDL-C) and apolipoprotein B (ApoB) levels also decreased significantly (p < 0.05). The median reduction of sdLDL-C and LDL-C was 16.6% and 13%, respectively. These variables decreased >10% in sixteen children (64%), independently from baseline levels, sex, age and body mass index (BMI). High density lipoprotein-cholesterol (HDL-C), lipoprotein a [Lp(a)], and triglycerides (TGs) levels remained unaffected. Conclusions: Plant sterols decrease sdLDL-C significantly and may be beneficial for children with hypercholesterolemia. © 2014 Garoufi et al.; licensee BioMed Central Ltd

Infections in Children With Cancer: The Role of the Presence or Absence of Neutropenia

BACKGROUND: Infections in patients with cancer are a major cause of morbidity and mortality. In most cases, the presence of neutropenia renders them prone to infections to either common or opportunistic pathogens. A wide spectrum of bacterial, viral, or fungal agents is encountered in these patients. AIM: The aim of this study was to evaluate infection types and pathogens in pediatric patients with cancer with and without neutropenia. METHODS: A total of 37 pediatric patients with cancer (median age ± 25% quartile, 6.0 ± 2.0% years) with 70 febrile episodes were evaluated at fever's onset and 48 hours later with complete blood count, C-reactive protein, cultures of biological fluids, polymerase chain reaction, and antibody titers. RESULTS: Of 70 infections, 30 (42.85%) were bacterial, 13 (18.57%) were viral, 3 (4.28%) were fungal, 16 (22.85%) were fever of unknown origin, 18 (25.71%) were opportunistic, and 12 (17.14%) were mixed infections. Neutropenia was detected in 42 (60.0%) of 70 febrile episodes, mainly in patients with hematological malignancies [odds ratio, 2.81 (0.96-8.22); P = 0.059]. Neutropenic patients had higher prevalence of mucocutaneous infections (47.6% vs 7.14%; P = 0.004). Herpes simplex virus 1 infections occurred only in the neutropenic group (14.3%). CONCLUSIONS: Patients with cancer exhibited a high prevalence of bacterial (42.85%), opportunistic (25.7%), and mixed infections (17.14%). Patients with hematological malignancies and neutropenia presented higher frequency of mucocutaneous and herpes simplex virus 1 infections than the nonneutropenic ones. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved

Variation of serum C-reactive protein (CRP) over time in pediatric cancer patients with febrile illness and its relevance to identified pathogen

Objective: To evaluate the correlation of serum CRP with clinical and laboratory parameters proven to be related to the cause of infection in pediatric cancer patients. Methods: We studied prospectively for a 12-month period, 37 pediatric cancer patients, who presented with 70 episodes of febrile illness (38 bacterial and 13 viral infections). At fever's onset and 48. h later, infection indices, such as CRP, WBC, ANC were measured in the peripheral blood. Moreover we calculated the change rate of CRP over 48. h [CRP/t = (CRP48h - initial CRP)/t (t = 2 days)]. Cultures of biological fluids, PCR and antibody detection of infectious agents were also obtained. Results: When comparing patients with viral vs. bacterial infections, mean CRP levels on admission (11.0 vs. 33.1 mg/L, p = 0.005) and at 48. h (13.4 vs. 71.9 mg/L, p = 0.0007), and CRP/t (0.9 vs. 18.8 mg/L/day, p = 0.030) were significantly lower in the group with viral infection. At 48. h - follow-up, patients with positive culture had higher CRP levels (57.3 vs. 43.3 mg/L, p = 0.048) and higher CRP/t (15.9 vs. 7.7 mg/L/day, p = 0.025), compared to those without proven infection. CRP/t at 48. h was correlated with both the fever duration (r = 0.27, p = 0.027) and maximum temperature (Tmax) during the febrile episode (r = 0.30, p = 0.013). Conclusions: Single CRP values on fever initiation can differentiate between viral and bacterial infections in febrile pediatric cancer patients. Moreover the change rate of CRP over time (CRP/t) is offered as a prognostic index of bacterial infection and a marker of the total duration of fever and Tmax. © 2012 The Canadian Society of Clinical Chemists