We present the case of a 7-year-old boy who fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH). Prompt visualization of his bone marrow confirmed the diagnosis of visceral leishmaniasis (VL). He responded well to treatment with liposomal amphotericin-B. The patient had a false-negative enzyme-linked immunosorbent assay for Leishmania infantum and a false-positive immunoglobulin M test for Epstein Barr virus (EBV). Because age at presentation is similar in children with VL and familial HLH for whom EBV is the usual trigger, ruling out VL is extremely important because nonspecific serologic tests for EBV can lead to the inappropriate diagnosis of EBV-driven primary HLH and to the administration of unnecessary immunochemotherapy.

The authors describe a 13-month-old girl who presented with progressively worsening anasarca edema that developed over the last three weeks along with increasing fatigue. Over the last several months she was consuming progressively increasing amounts of fresh cow`s milk. Laboratory examinations on admission showed severe microcytic and hypochromic anemia (hemoglobin 3.8 g/dl) and hypoferritenemia indicative of iron deficiency, while urinalyses showed no proteinuria. The child was transfused with 13ml/kg packed red blood cells and approximately 2g/kg intravenous albumin. On the second and fourth hospital days, she received 100 mg of iron sucrose intravenously that she tolerated well. Eight months after the described events, she is healthy with normal hemoglobin for age, while she has no laboratory evidence of cow`s milk protein allergy. Pediatricians should be aware of the association of severe iron deficiency anemia (IDA) and anasarca edema, and should screen infants in their practice for anemia at the age of 12 months or sooner, if risk factors are present.

BACKGROUND:Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals.CASE REPORT:We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with the presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg.CONCLUSION:Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

Patients with common variable immunodeficiency (CVID) develop autoimmune hematologic manifestations. We report a 14-year-old boy with Evans syndrome, who presented at the age of 11.5 years with autoimmune hemolysis and was successfully managed with corticosteroids. Initially, the serum immunoglobulins were within the low-normal range for age, but two years after presentation he definitely fulfilled the diagnostic criteria for CVID, despite a negative history for serious infections. DNA sequencing by PCR of the TNFSRF13B gene that encodes the TACI receptor disclosed the heterozygous mutation C104R that is found in approximately 10–15% of patients with CVID. Common variable immunodeficiency should be considered in the differential diagnosis of autoimmune hematologic manifestations, since its timely diagnosis may considerably affect clinical management and patient outcome.

This article has been retracted by the authors under the agreement between the Editor-in-Chief, Masayuki Saijo and authors.

Urinary tract infections (UTIs) are common infections associated with considerable morbidity and mortality, particularly in paediatric patients. The alarmingly increasing antimicrobial resistance of contemporary uropathogens in children necessitates the re-evaluation of antibiotic treatment. We evaluated uropathogens isolated from children hospitalised due to a community-acquired UTI over a 5.5-year period in a university hospital of Northern Greece and their antibiotic susceptibility patterns. The antibiotic susceptibility of uropathogens was compared by patient sex and age. Bacterial identification and antibiotic susceptibility testing were performed by the automated VITEK (R) 2 system and the Kirby-Bauer method. Overall, 221 urinary isolates were identified from 218 children with a documented UTI, including 170 (76.9%) Escherichia coli, 17 (7.7%) Proteus spp., 15 (6.8%) Klebsiella spp., 9 (4.1%) Pseudomonas aeruginosa, 4 (1.8%) Enterococcus faecalis, 2 (0.9%) Enterobacter spp., 2 (0.9%) Morganella morganii and 2(0.9%) Serratia fonticola. Comparing antibiotic susceptibilities of E. coli isolates by age [<= 2 years vs. >2 years] and sex did not show any significant differences. Only 80(49.1%) of the 163 tested E. coli isolates were found to be susceptible to ampicillin, whereas susceptibility to amoxicillin/clavulanic acid (AMC), ampicillin/sulbactam, trimethoprim/sulfamethoxazole and nitrofurantoin was 78.3%, 78.9%, 75.3% and 96.9%, respectively. Parenteral second- and third-generation cephalosporins, aminoglycosides and carbapenems were highly active against almost all uropathogens. We conclude that ampicillin should not be used for empirical therapy of paediatric community-acquired UTIs in our region. AMC and oral second-generation cephalosporins cover ca. 80% of uropathogenic E. coli, whilst nitrofurantoin is an appealing option for UT! chemoprophylaxis. (C) 2015 International Society for Chemotherapy of Infection and Cancer. Published by Elsevier Ltd. All rights reserved.

Background Intravenous iron sucrose is not recommended by its manufacturers for use in children despite extensive safety and efficacy data in adults. Methods We reviewed the experience of our department between January, 2011 and February, 2014 with the use of intravenous iron sucrose in children ≤14 years of age who failed in oral iron therapy for iron deficiency anemia (IDA). Results Twelve children (6 females) aged 1.2-14 years (median age 8.9 years) received at least one dose of intravenous iron sucrose. Ten patients had IDA inadequately treated or non-responsive to oral iron therapy. One patient received therapy for blood transfusion avoidance and one for presumed iron refractory iron deficiency anemia (IRIDA). Iron sucrose infusions were given on alternate days up to three times per week. The number of infusions per patient ranged from 2 to 6 (median, 3), the individual doses from 100 mg to 200 mg (median, 200 mg), and the total doses from 200 mg to 1200 mg (median, 400 mg). Iron sucrose was effective in raising the hemoglobin concentration to normal in all patients with IDA, i.e., from 7.6±2.38 g/dL to 12.4±0.64 g/dL, within 31-42 days after the first infusion. The single patient with IRIDA demonstrated a 1.8 g/dL rise. Injection site disorders in three cases and transient taste perversion in one case were the only side effects. Conclusion Intravenous iron sucrose appears to be safe and very effective in children with IDA who do not respond or cannot tolerate oral iron therapy.

A 9-year-old boy with multiple decayed teeth developed right eye preseptal cellulitis and left subdural empyema due to Streptococcus constellatus. He recovered after neurosurgical evacuation of the empyema and antibiotic treatment with highdose intravenous ampicillin for three weeks. Poor oral hygiene predisposes to hematogenous and possibly fatal dissemination of rare oral commensals to the subdural space.

The authors describe two young brothers and a 12‐year‐old male with long‐standing thrombocytopenia with normal sized platelets, in whom novel mutations of the WAS gene were identified. Their clinical picture and the in vitro assessment of the T‐cell function were consistent with X‐linked thrombocytopenia (XLT). A high index of suspicion for XLT is required, even in the setting of normal sized platelets for males with affected maternally‐related male family members, and males with moderately severe chronic thrombocytopenia that have failed to respond to treatments that are usually effective for immune thrombocytopenia. Pediatr Blood Cancer 2014;61:2305–2306. © 2014 Wiley Periodicals, Inc.

Background Streptococcus pneumoniae (SP) strains carried in the nasopharynx of healthy children reflect the circulating strains implicated in respiratory and invasive infections. Methods We prospectively analyzed the serotype distribution of SP nasopharyngeal isolates in healthy toddlers aged 21 to 78 months (median age, 63 months) attending day care centers during the winters of 2010–2012. All children were sampled once during the study period. Serotyping was performed by the Quellung reaction using the 12 pooled antisera Pneumotest panel and specific factor sera. We also studied the in vitro antimicrobial susceptibilities of the isolated pneumococci to several antibiotics by E-test, and for macrolide-resistant isolates of 2010 only, the resistance genes mef(A) and erm(B). Results Among the 543 toddlers studied, 98.8% had received 1 or more doses of 7-valent pneumococcal conjugate vaccine (PCV7), whereas none was immunized with PCV10 or PCV13. Overall, 27.4% of the children tested were colonized with SP, whereas 22.8% and 52.3% of the isolates belonged to PCV7 and PCV13 serotypes, respectively. Overall, 25.5% of the isolates had intermediate resistance to penicillin (minimal inhibitory concentration, 0.12–1 μg/mL) and 3.4% were resistant to penicillin (minimal inhibitory concentration, 2 μg/mL). All isolates were susceptible to amoxicillin, whereas 28.2% were resistant to macrolides, with the mef(A) gene–mediated M phenotype being the most common in 2010, the year that macrolide resistance genes were studied (93.4% of macrolide-resistant isolates). Conclusions Implementation of PCV13 will increase the coverage of children in our area for invasive pneumococcal serotypes by approximately 30%. Postlicensure serotyping and antimicrobial susceptibility surveillance are essential for designing rational immunization strategies.

Between late May and July 2012, 105 children (62 boys) originating from 2 cities of Thrace were examined because of fever, headache and abdominal pain. Thirty-three of them were hospitalized. They had normal hemograms, and mild to moderate cerebrospinal fluid pleocytosis. Echovirus 30 was isolated from fecal and cerebrospinal fluid samples. Among confirmed cases of echoviral illness, the meningitis attack rate was 51.9%.

Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancreatitis should be ruled out in the context of vomiting in any patient with isovaleric acidemia. Conversely, branched-chain organic acidemias should be included in the differential diagnosis of any child with pancreatitis of unknown origin.

Objective. Childhood asthma is a frequent cause of absenteeism that affects school performance. We aimed to investigate the impact of asthma on absenteeism and school performance level of elementary and high school students. Methods. Data about sociodemographics, absenteeism, and academic achievement were obtained from 1539 students attending 98 schools in Greece. School performance was assessed for the last two years of school attendance using parents’ and teachers’ reports and grade point average promotion. Results. The mean of the days of absence of students with asthma was higher compared to the healthy students (6.2 ± 11.7 versus 0.3 ± 3.1, resp., P<0.001). Students with reduced healthcare use presented less absenteeism than those with increased healthcare use for asthma (4.3 ± 8.6 versus 12.4 ± 17.0 days, resp., P<0.001). Asthma and healthcare use for asthma accounted for an overall estimated variability in absence days of 13.8% and 9%, respectively. Absenteeism was associated with poor school performance for the last two years of school (P=0.002) and with lower grade point promotion in elementary school students (P=0.001) but not in high school students (P=0.316). Higher level of parental education was associated with better school performance (P<0.001). Asthma was associated with a decreased possibility for excellent performance (OR = 0.64, P=0.049, 95%CI = 0.41–1.00) in elementary students. Students with asthma using inhalers were four times more likely to perform excellently in elementary school (OR = 4.3, P=0.028, 95%CI = 1.17–15.95) than their asthmatic peers with alternative asthma treatments. Conclusions. Asthma and increased healthcare use enhance school absenteeism. Inhaled steroid use and the higher parental education level were the most important predicting factors for good school performance in elementary school asthmatic children.

OBJECTIVE:Breastfeeding is recognized as an important public health issue with substantial social and economic implications. Moreover, the growth of exclusively breastfed babies differs from that of their formula-fed counterparts. The purpose of this study was to evaluate the physical growth of exclusively breastfed and self-weaned boys and girls of Greece 0-36 months of age.SUBJECTS AND METHODS:The physical growth of children was monitored from birth up to 36 months of age. Body weight, length/height, and head circumference were recorded. The study population included 101 boys and 105 girls who were recruited consecutively from a private breastfeeding clinic in the second largest city of Greece and through La Leche League groups throughout the country during 2000 to 2005. All infants were exclusively breastfed for ≥ 6 months. Anthro software ( www.who.int/childgrowth/software/en/index.html ) was used to compare the data of our study population and the World Health Organization standards for weight, length/height, and head circumference for age.RESULTS:Male and female infants at 12 months had almost tripled their weight (192% and 190% increase, respectively) and had increased their length (height) by 48% and 47%, respectively, and head circumference had increased by 35% and 33%, respectively. In both sexes the relative length/height and the head circumference-for-age increase rates were higher from the first to the second month of life than at any other period.CONCLUSIONS:Long-term exclusively breastfed infants grow normally. Hence, no recommendations for the interruption of lactation and/or supplementation with formula are justified.

Despite the fact that sunlight-dependent skin synthesis is the major mechanism for vitamin D synthesis in vivo, vitamin D-deficiency rickets continues to occur in exclusively breastfed infants in Greece. We present such a case in a 5-month-old infant who presented with afebrile seizures and whose mother was underexposed to sunlight due to veiling for religious reasons. Additionally, we briefly but thoroughly review the relevant medical literature. A high index of suspicion is required for nutritional rickets, when seizures occur in exclusively breastfed infants whose mothers have inadequate exposure to sunlight.

Clinical manifestations of deep venous thrombosis (DVT) tend to overlap with those of deep-seated musculoskeletal infections (MSIs). Consequently, the incidence of DVT as a complication of MSI may be underestimated. The objective of this study was to evaluate the incidence, clinical features, and outcomes of MSI-related DVT in children.We systematically reviewed relevant studies retrieved from PubMed and Scopus databases.Overall, 93 children with MSIs who developed DVT were identified from 28 retrospective studies. The majority were boys. Osteomyelitis was the most frequent MSI (69/74, 93%). Staphylococcus aureus was the predominant pathogen (83/93, 89%); 61% of these isolates were methicillin-resistant S. aureus (MRSA). Pulmonary involvement, presumably due to septic emboli, was observed in 65% of the included children. Four children died due to multiple organ failure and two due to respiratory distress. In two of the three studies providing comparative data, MRSA infections were observed significantly more frequently in children who developed DVT compared to those who did not. Yet, the respective differences observed for methicillin-susceptible S. aureus (MSSA) infections were non-significant in these three studies.Despite the inclusion of many case reports and the retrospective design of the evaluated studies, our findings suggest that boys seem to be more frequently affected by MSIs complicated by DVT. Moreover, MRSA seems to be more frequently associated with DVT compared to MSSA. Pulmonary involvement appears to be a frequent complication. Prospective studies are needed in order to further clarify this issue.

Propranolol has emerged as a new treatment option for infantile hemangiomas. We describe a 20-month-old boy with a large right parotid hemangioma diagnosed at the age of 37 days. Starting at the age of 2.5 months, he received oral propranolol for 6.5 months. Although the mass regressed, it recurred when propranolol was discontinued. He was successfully retreated at the age of 11 months with propranolol for another 5.5 months without further recurrences. Treatment was tolerated well. Our paper and a review of the literature demonstrate that propranolol appears to be safe and effective for symptomatic infantile parotid gland hemangiomas.

Introduction: Macrodystrophia lipomatosa (MDL) is a non-hereditary congenital developmental anomaly characterized by localized gigantism due to overgrowth of all mesenchymal elements. Case Report: The authors describe a case of macrodystrophia lipomatosa (MDL) with involvement of the median nerve in a 10-year-old girl and review the pathologic, radiographic, ultrasonographic and MR imaging characteristics. Conclusion: Ultrasonography and MR imaging are pathognomonic for MDL and reliably distinguish it from other forms of localized congenital gigantism. However, since ultrasonography has the advantages of much lower cost, portability and easy accessibility, we propose its use as the imaging modality of choice in the initial diagnostic approach of congenital localized gigantism.