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.

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.

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.

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.

(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.

A case of immune thrombocytopenic purpura after influenza vaccination: consequence or coincidence?

SUMMARY: The researchers describe the case of a earlier healthy 3-year-old boy, who developed immune thrombocytopenic purpura (ITP) 26 days after immunization with the second dose of seasonal influenza vaccine. He recovered quickly and uneventfully within 2 days after receiving a single dose of intravenous immunoglobulin. Review of the medical literature showed that symptomatic thrombocytopenia occurs in a substantial number of children and adults who require hospitalization for complicated natural influenza infection, particularly avian influenza. In contrast, it is exceptionally rare after influenza immunization, as only few case reports describe such an association in adults but not in children. As the risk of thrombocytopenia after natural influenza seems to be much higher than after immunization, annual influenza vaccination is advised for patients with personal history of ITP who at risk of influenza-related complications owing to underlying medical problems.

Transient hepatitis B surface antigen circulation after Infanrix-Hexa: a case report and review of the literature.

The authors describe the case of a 70-day-old boy who was mistakenly diagnosed as suffering from acute hepatitis B, when he presented with persistent unconjugated hyperbilirubinemia and transaminasemia and was found to be seropositive for the hepatitis B surface antigen. The antigenemia was transient and related to his recent immunization with Infanrix-Hexa. Caution is required during interpretation of a positive HBsAg test that is obtained within 28 days after vaccination against hepatitis B.

External re-programmation by a new radionuclidic technique of electronic cerebrospinal fluid valve in case of hydrocephalus.

Hydrocephalus is defined as an abnormal enlargement of the ventricles of the brain due to an excessive accumulation of cerebrospinal fluid (CSF) because of a disturbance of its flow, absorption and/or secretion. The usual method of CSF diversion is a ventriculo-peritoneal shunt. Complications of implanted shunt systems include mechanical failure, shunt pathway obstruction, infection, foreign body (allergic) reaction to implants and CSF leakage along the implanted shunt pathway. These problems are solved with the use of programmable ventricular-peritoneal CSF valves. In this case, we describe a radionuclidic method for the control of successful reprogramming of the CSF valve. Furthermore, we analyze some technical data of such a valve-type are essential for the application of the above technique by nuclear medicine physicians. Scintigraphic evaluation of the electronic V-P drainage valve regulation is a noninvasive, not expensive, rapid and safe method with no complications for the patient and provides a reliable proof of the patency of the V-P shunt.

Hepatobiliary scan in Alagille syndrome; arteriohepatic dysplasia.

Arteriohepatic dysplasia or congenital paucity of interlobular bile ducts - Alagille Syndrome, is a well defined syndrome characterized by five major features, including chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects, peripheral pulmonary artery hypoplasia or stenosis and facial dysmorphy. The disease is very rare. Only three cases have been reported in Greece and none with renal involvement. Hepatobiliary scan was a fundamental tool in the patients diagnosis and therefore we present the following case.

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.

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.

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.

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.

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.

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%.

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.

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.