Immunomodulatory therapy in recurrent acute necrotizing encephalopathy ANE1: is it useful?

Acute Necrotizing Encephalopathy (ANE) is a rare disorder characterized by fever, seizures and rapid progression to coma after the onset of a viral infection. Most cases are sporadic, however the observation of multiple cases in the same family with recurrent episodes of ANE led to the identification of a genetic form of the disorder, called ANE1, and to the discover of the causative mutation in RANBP2 gene. We report the first Italian child with ANE1 carrying the common c.1880C>T mutation in the RANBP2 gene, who presented three episodes of acute encephalopathy in the first two years of life. The child showed a less severe clinical and neuroradiological course with respect to the previously reported patients. During the acute encephalopathy episodes he was treated with steroids and immunoglobulin. A very low steroid maintenance therapy was administered after the second episode until the onset of the third. Thirty days after the last episode he started monthly intravenous immunoglobulin that might be used for prevention of viral infections. At the moment he is still continuing a low steroid maintenance therapy and monthly IVIG. We could hypothesize that the less severe clinical presentation of the third episode might be correlated to the steroid treatment or that the patient grew older. Despite there is no evidence to support that ANE1 is an immune-mediated disease, immunomodulatory therapy might be considered in the management of ANE1 cases especially in early childhood, in which a fatal course has been frequently reported. Further studies will be necessary to define the clinical, immunological and genetic aspects, as well as the outcome of immunomodulatory therapy in patients with ANE1.

Epidemiology of febrile neutropenia in children with central nervous system tumor: results from a single center prospective study.

Data regarding the epidemiology febrile neutropenia during chemotherapy for pediatric central nervous system neoplasia are scarce. Data retrieved from a prospective study performed from January 2002 to December 2004 at G.Gaslini Children Hospital, Genoa, Italy, where analyzed to evaluate proportions, rate for 1000 neutropenic days and etiology of fever in neutropenic children receiving gentle, standard, or peripheral blood stem cell transplant (PBSCT) therapy for central nervous system tumor. During the study duration, 243 periods of neutropenia (granulocyte count <1000/cmm), accounting for 3544 patient-days at risk, were documented in 62 children. A total of 72 febrile episodes were observed in 66 (27%) neutropenic periods, for a rate of 20.31. A primary febrile episode was observed in 10% of neutropenic periods after gentle chemotherapy, in 30% after standard chemotherapy, and in 48% after PBSCT (P<0.0001). The rate of primary febrile episodes was 6.19 after a gentle chemotherapy, 27.02 after standard treatment, and 31.02 after PBSCT (P<0.0001). In a multivariable regression model, the type of chemotherapy (gentle vs. standard and PBSCT) and the thresholds of granulocyte count at neutropenia onset (999-501/cmm and 500-101/cmm vs. ≤100/cmm) were the only factors significantly associated with the development of febrile neutropenia.

Catheter-related bacteremia caused by methicillin-resistant coagulase negative staphylococci with elevated minimal inhibitory concentration for vancomycin.

Treatment of bacteremia caused by methicillin-resistant coagulase-negative staphylococci with vancomycin minimum inhibitory concentration ≥2 mg/L frequently requires central venous catheter removal in children with cancer. There are few data supporting efficacy and safety of antibiotic catheter lock or use of daptomycin or linezolid for this indication in children.

Epidemiology and clinical features of Mycoplasma pneumoniae infection in children.

Mycoplasma pneumoniae (MP) is, considered to affect rarely children less than 5 yrs of age. This study was performed to describe the epidemiology and the clinical features of MP lower respiratory tract infection (LRTI) in children, presenting to a tertiary children hospital. Eleven month-longitudinal study of LRTI due to MP, diagnosed by polymerase chain reaction (PCR) on throat swab specimen, was performed. Out of 866 children with LRTI admitted to the Gaslini Pediatric Institute in Genoa, 102 had a positive PCR for MP. We found 39 preschool-aged children, 42 school-aged children and 21 young adolescent [6.20 (3.81) yrs old]. Interestingly, eight MP+ infants had <8 months of age. The commonest presentations were cough and/or fever (76.5%). Tachypnoea, upper respiratory tract involvement, diarrhoea and vomiting were more common in the <5 yr Gr as compared to the other groups. Chest X-ray was found abnormal in 76 children: consolidations were the commonest finding. Laboratory test showed that the preschool-aged children had a higher number of lymphocytes (p<0.0001) and monocytes (p=0.009). Thrombocytosis was found in 35.7% of children and was more frequent in the preschool-aged children (p=0.013). MP infection is common in preschool-aged children, including young infants, and may have different clinical presentation, as compared to older children.

Unusual manifestations of infections due to Mycoplasma pneumoniae in children.

Mycoplasma pneumoniae (Mp) is an important cause of pneumonia in paediatric age, but also other organs or systems can be affected even without pulmonary involvement. The purpose of this study is to stress the unusual clinical features of Mp infection in children. A review of children affected with Mp infection with peculiar pulmonary and/or extra-pulmonary forms is reported. Diagnosis of Mp infection was always confirmed by serum anti-Mp antibody assay. Two patients with infection of the lower airways showed severe respiratory distress; nine cases with only extra-pulmonary manifestations presented urticaria and arthralgia; three patients had severe neuromuscular impairment, one of these resulting in flaccid tetraparesis; one 2-year-old child had anicteric hepatitis, without any sequelae; one case of a 6-year-old child presented severe haemolytic anaemia, and a 5-year-old child with Schonlein-Henoch purpura. In conclusion, Mp infection, a frequent cause of pneumonia at all paediatric ages, may also give rise to extrapulmonary manifestations. Frequently, muscular-articular or neurological systems, skin or other organs are involved. Clinical suspicion of Mp infection is essential in severe cases and the outcome of all pulmonary and/or extra-pulmonary manifestations depends on early diagnosis and specific therapy.