Outbreak of bloodstream infections because of Serratia marcescens in a pediatric department.

Background: Serratia marcescens can cause health care-associated infections. We herewith report the investigation and control of an outbreak of S marcescens bloodstream infections (BSI) in a general pediatric department.

Methods: From April to May 2009, temporally related cases of S marcescens BSI occurred in a 40-bed general pediatric department of a tertiary care hospital.

Standard-dose intravenous anti-D immunoglobulin versus intravenous immunoglobulin in the treatment of newly diagnosed childhood primary immune thrombocytopenia.

Background: We conducted a study to evaluate the efficacy of intravenous (IV) anti-D against IV immunoglobulin (IVIG) in newly diagnosed immune thrombocytopenia (ITP) in children and to identify the clinical characteristics of the children most likely to benefit from one or the other treatment.

Procedure: Children (6 mo to 14 y) with newly diagnosed ITP and a platelet count <20,000/μL were treated either with a single bolus dose of 50 μg/kg IV anti-D or with 0.8 to 1 g/kg IVIG in a randomized manner.

Successful treatment of a spontaneous haemothorax with recombinant factor VIIa in a haemophilic child with inhibitors.

Haemophilia A is an X-linked disorder caused by a deficiency of factor VIII. Haemorrhage in various sites may occur spontaneously or secondary to trauma depending on the severity of the deficiency. Common manifestations include haemarthrosis, epistaxis, gastrointestinal haemorrhage and haematuria.

A patient with Lemierre syndrome.

Lemierre syndrome, also known as postanginal sepsis, is a severe complication of an acute oropharyngeal infection that results in septic thrombophlebitis of the ipsilateral internal jugular vein with subsequent septicemia, often complicated by metastatic infections (Syed et al., Laryngoscope 117:1605-1610, 2007). We present the case of a previously healthy 12-year-old boy with Lemierre syndrome, caused by streptococci (Abiotrophia defectiva), complicating a subcutaneous neck abscess.

Hemolytic anemia presenting with idiopathic intracranial hypertension.

We report on an 8-year-old girl with hemolytic anemia because of infection with parvovirus B19 and increased intracranial pressure. She presented acutely with headache, vomiting, and mild scleral and mucosal icterus. Upon evaluation, the patient exhibited profound hemolytic anemia, papilledema, and increased intracranial pressure.

The effect of dietary habits and socioeconomic status on the prevalence of iron deficiency in children of northern Greece.

Background: Despite encouraging reports concerning the declining prevalence of iron deficiency, this easily preventable disorder is still an existing problem in presumably developed regions.

Objective: To evaluate the prevalence of iron deficiency and relevant anemia in children residing in Northern Greece and to study possible associations.

Design: 3,100 children aged 8 months to 15 years were evaluated.

Neurological complications in beta-thalassemia.

Over the years, several reports have demonstrated involvement of the nervous system in beta-thalassemia patients. Neurological complications have been attributed to various factors such as chronic hypoxia, bone marrow expansion, iron overload, and desferrioxamine neurotoxicity. In most cases, neurological involvement does not initially present with relevant signs or symptoms (i.

Rare thalassemic syndrome caused by interaction of Hb Questembert (alpha1 codon 131, TCT>CCT, Ser>Pro) with an alpha-thalassemia-2 deletion: implications for diagnosis and management.

Abnormal globin chain biosynthesis may result in deficient quantity (thalassemia) or structural variation (abnormal hemoglobins) and traditionally, they represent two phenotypically distinct groups of disorders. However, the phenotypic expression of unstable hemoglobin variants often combine features of thalassemia together with variable peripheral hemolysis. To achieve definitive diagnosis in a child presenting with hemolytic anemia along with features associated with thalassemia intermedia, we evaluated clinical, hematological, biochemical, globin biosynthetic and molecular data.

Congenital erythrocytosis with increased erythropoietin level.

The term "absolute erythrocytosis" denotes a heterogeneous group of disorders characterized by an increased red blood cell mass. The authors describe a 20-month-old girl with absolute erythrocytosis. Erythropoietin levels were found to be extremely increased, although extensive evaluation failed to reveal a cause for such an inappropriate increase.

Growth hormone treatment in short children with beta-thalassemia major.

The effect of one year recombinant human growth hormone (rhGH) treatment on growth rate and bone age was studied in ten short prepubertal children with beta-thalassemia major (age range 7.10-12.03 yr) with normal GH response to provocative stimuli.

Intragenic interspecific complementation of glucose 6-phosphate dehydrogenase in human-hamster cell hybrids.

A new variant of human glucose 6-phosphate dehydrogenase (G6PD), provisionally designated G6PD Harilaou, was observed in a Greek boy affected by severe hemolytic anemia. G6PD Harilaou was associated with very severe deficiency of enzyme activity, which measured about 1% of normal in the patient's fibroblasts. By fusion of Harilaou fibroblasts with a similarly enzyme-deficient mutant CHO cell line, we have isolated a hybrid cell line that has a G6PD activity 5-10 times higher than either of the parental cells.

Iron-related disturbances of cell-mediated immunity in multitransfused children with thalassemia major.

Immunological abnormalities have been observed in many haemophiliacs receiving clotting factor concentrates. To determine whether similar changes also occur after repeated blood transfusions we estimated T cell subsets and cutaneous delayed hypersensitivity (CDH) in 50 multitransfused children with beta-thalassemia major (beta-TM). All patients were also tested for anti-HTLV-III/LAV antibodies.