A Brief Report and Mini-Review of Visceral Leishmaniasis-associated Hemophagocytic Lymphohistiocytosis in Children.

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.

Severe iron deficiency anemia and anasarca edema due to excessive cow`s milk intake.

Mantadakis E, Zikidou P, Tsouvala E, Thomaidis S, Chatzimichael A. Severe iron deficiency anemia and anasarca edema due to excessive cow`s milk intake. Turk J Pediatr 2019; 61: 102-106. 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.

Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.

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.

Retraction: First human case of Rickettsia sibirica mongolotimonae infection in Northern Greece in a teenager from Thrace.

This article has been retracted by the authors under the agreement between the Editor-in-Chief, Masayuki Saijo and authors.

Intravenous iron sucrose for children with iron deficiency anemia: a single institution study.

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.

Susceptibility patterns of uropathogens identified in hospitalised children with community-acquired urinary tract infections in Thrace, Greece.

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® 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 UTI chemoprophylaxis.

X-linked thrombocytopenia in three males with normal sized platelets due to novel WAS gene mutations.

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.

Echovirus 30 outbreak associated with a high meningitis attack rate in Thrace, Greece.

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%.

Acute pancreatitis with rapid clinical improvement in a child with isovaleric acidemia.

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.

Involution of a large parotid hemangioma with oral propranolol: an illustrative report and review of the literature.

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.

Growth of exclusively breastfed and self-weaned children of Greece aged 0-36 months.

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.

Deep venous thrombosis in children with musculoskeletal infections: the clinical evidence.

BACKGROUND: 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. METHODS: We systematically reviewed relevant studies retrieved from PubMed and Scopus databases. RESULTS: 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. CONCLUSIONS: 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.

Seizures as initial manifestation of vitamin D-deficiency rickets in a 5-month-old exclusively breastfed infant.

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.

(99m)Tc-DMSA scintigraphy diagnosing crossed renal ectopia with fusion in a three years old boy.

A 3 years old boy with a history of surgery for orchidopexy was admitted to our hospital with fever and abdominal pain. Clinical examination and laboratory investigations revealed urinary tract infection with renal involvement. Ultrasonography demonstrated a solitary left kidney and raised the suspicion of a fusion anomaly. Voiding cystography disclosed grade III vesicoureteral reflux and technetium-99m dimercaptosuccinic acid scintigraphy revealed right to left crossed renal ectopia with fusion (L-shaped kidney). The patient is undergoing standard follow-up for the early detection of possible renal complications. In conclusion, L-shaped kidney is a rare entity and the (99m)Tc-DMSA scintigraphy played an important role on timely diagnosis.

Acute Tc-99m DMSA scan for identifying dilating vesicoureteral reflux in children: a meta-analysis.

Controversy exists regarding the type and/or sequence of imaging studies needed during the first febrile urinary tract infection (UTI) in young children. Several investigators have claimed that because acute-phase Tc-99m dimercaptosuccinic acid (DMSA) renal-scan results are abnormal in the presence of dilating vesicoureteral reflux, a normal DMSA-scan result makes voiding cystourethrography (VCUG) unnecessary in the primary examination of infants with UTI. To evaluate the accuracy of acute-phase DMSA scanning in identifying dilating (grades III through V) vesicoureteral reflux documented by VCUG in children with a first febrile UTI, we performed a meta-analysis of the accuracy of diagnostic tests as reported from relevant studies identified through the PubMed and Scopus databases. Patient-based and renal unit-based analyses were performed. Overall, 13 cohort studies were identified. Nine studies involved patients younger than 2 years, 3 involved children aged 16 years or younger, and 1 involved exclusively neonates. Girls constituted 22% to 85% of the involved children. Pooled (95% confidence intervals) sensitivity and specificity rates of DMSA scanning were 79% and 53%, respectively, for the patient-based analysis (8 studies) and 60% and 65% for the renal unit-based analysis (5 studies). The respective areas under the hierarchical summary receiver operating curves were 0.71 and 0.67. Marked statistical heterogeneity was observed in both analyses, as indicated by I(2) test values of 91% and 87%, respectively. Acute-phase DMSA renal scanning cannot be recommended as replacement for VCUG in the evaluation of young children with a first febrile UTI.

Spinal epidural abscess in a young girl without risk factors.

Spinal epidural abscess (SEA) is a rare infection associated with well-established risk factors mainly in adults. We describe an 11-year-old girl without any known risk factors who presented with fever and localized spinal tenderness in the lumbar area and was diagnosed with spinal MRI as suffering from a posterior SEA extending between T11 and L4. She was successfully managed with sequential intravenous and oral antibiotics along with minimally invasive surgery without laminectomy. Methicillin-sensitive Staphylococcus aureus was the responsible pathogen isolated at surgery. Immediate institution of antibiotics, spinal MRI, and well-timed neurosurgical consultation are mandatory for a favorable outcome in cases of SEA in children.

Recurrence of paroxysmal cold hemoglobinuria in a boy after physical cooling for fever.

Hemolysis and hemoglobinuria after direct exposure to cold has rarely been reported in paroxysmal cold hemoglobinuria (PCH). The authors describe a 2.5-year-old boy with PCH (Donath-Landsteiner autoimmune hemolytic anemia), in whom 16 days after presentation, the hemoglobinuria and hemolysis recurred, when he was subjected to physical cooling, as a means to control fever associated with hospital-acquired croup. The hemolysis resolved with warmth, and administration of dexamethasone. PCH should be suspected in children with hemolytic anemia and positive direct antiglobulin test for complement. Avoidance of cold in the recovery period is imperative to prevent recurrences, whereas a short course of corticosteroids may be of benefit in suppressing the antibody production.

Antimicrobial susceptibility of pediatric uropathogens in Thrace, Greece.

The aim of this study was to investigate the bacterial pathogens involved in pediatric urinary tract infections (UTIs) in a tertiary general hospital located in the Thrace province of Northern Greece over a 69-month period (1/2003 to 9/2008), and their antibiotic susceptibility patterns. A total of 622 episodes of UTIs in 508 children were identified. Median age of all children was 16 months (range 1 month to 14 years). Boys were significantly younger than girls (9 months vs. 24 months). Escherichia coli was the most common uropathogen and responsible for 69.1% of UTIs. Approximately half of E. coli isolates were resistant to ampicillin and 20.5% to trimethoprim/sulfamethoxazole (TMP/SMX). E. coli resistance to second-generation and third-generation cephalosporins was <4%, to aminoglycosides <8%, and to nitrofurantoin 4.4%. Pediatric E. coli urine isolates were significantly more resistant to ampicillin and ticarcillin and more sensitive to quinolones compared to adult E. coli uropathogens identified in the same hospital. E. coli resistance to ampicillin and amoxicillin/clavulanic acid was significantly higher in boys 12-23 months-old compared to girls of the same age. In conclusion, nitrofurantoin is a very good choice for chemoprophylaxis. Amoxicillin/clavulanic acid, second-generation cephalosporins, and TMP/SMX are appropriate choices for oral empirical treatment of UTIs. Parenteral aminoglycosides and second and third-generation cephalosporins are excellent treatment choices for inpatient therapy. Finally, sex and age are additional factors that should be taken into account when choosing empirical therapy for children with UTIs.

Knowledge on pulse oximetry among pediatric health care professionals: a multicenter survey.

OBJECTIVES: The objective of this study was to assess the knowledge on pulse oximetry among health care professionals involved in pediatric care. METHODS: A multiple-choice questionnaire was distributed to 505 pediatric health care professionals from 19 hospitals and health centers throughout Greece. Exploratory factor analysis was performed to identify underlying factors that could explain most of the variance of the responses. The mean test and factor scores were calculated and compared between clinical settings. RESULTS: The mean test score was 61.9+/-18.1%. After factor analysis, 2 distinct groups of deficits in knowledge regarding pulse oximetry were identified: 1 was interpreted as relating to practical knowledge and the other to theoretical knowledge. The mean score of the items that assessed practical knowledge was 82.7+/-12.5% and of those that assessed theoretical knowledge was 44.2+/-21.7%. Pediatricians and family practitioners, participants from level 3 institutions, and health care professionals working in ICUs scored better, particularly on the items that assessed theoretical knowledge. Logistic regression analysis revealed that only participants from level 3 institutions and those from ICUs had a greater likelihood of achieving a higher score (total score odds ratio: level 3, 2.89, ICU, 8.13; theoretical knowledge odds ratio: level 3, 3.40, ICU, 10.95). CONCLUSIONS: Pediatric health care professionals have marked deficiencies in their knowledge on pulse oximetry, particularly in regard to the principles underlying the method and its limitations. Strategies that are directed at improving knowledge on pulse oximetry are urgently needed at all levels of experience in pediatric care.