@article {8052, title = {Life-threatening unilateral hearing impairments. Review of the literature on the association between inner ear malformations and meningitis.}, journal = {Int J Pediatr Otorhinolaryngol}, volume = {79}, year = {2015}, month = {2015 Dec}, pages = {1969-74}, abstract = {

BACKGROUND: Bacterial meningitis is a life threatening disease that can be triggered by a CSF leak through an inner ear malformation. Early identification of the specific type of cochleovestibular dysplasia and the associated risk of meningitis is of vital importance.

OBJECTIVES: The objective of this review is to collect and discuss available data on the association between inner ear malformations and meningitis in children.

METHODS: Electronic databases were crosschecked for obtaining relevant papers published in the last 20 years, and further cases were identified by hand searching through the references. Demographic data were extracted from full texts, together with information on the severity of hearing impairment, the type of inner ear anomaly, the site of cerebrospinal fluid leak, the number of recurrent meningitis episodes.

RESULTS: Sixty-seven cases of meningitis related to inner ear malformation have been identified among 45 papers. Mean age at presentation is 3.60{\textpm}3.00 (range 0.1-14) years. Average diagnostic delay from the first episode of meningitis is 3.44{\textpm}3.41 (range 0.00-10.00) years. The number of meningitis episodes that occurred before the correct diagnosis and definitive surgical treatment is 3.27{\textpm}1.81 (range 1.00-10.00). Unilateral hearing impairment affects 70\% of patients. Six patients had normal hearing at presentation. Two children are dead from inner-ear-malformation-related meningitis among reviewed reports.

CONCLUSION: A high number of paediatric patients carrying inner ear malformations, especially when associated with unilateral hearing impairment, could be at risk to develop recurrent bacterial meningitis. Universal newborn hearing screening programs should prompt a diagnostic work-up even in the case of unilateral hearing impairment, in order to prevent inner ear malformation-related meningitis.

}, issn = {1872-8464}, doi = {10.1016/j.ijporl.2015.09.028}, author = {Muzzi, E and Battelino, S and Gregori, M and Pellegrin, A and Orzan, E} } @article {3600, title = {Experience from a single paediatric transplant centre with identification of some protective and risk factors concerning the development of hepatic veno-occlusive disease in children after allogeneic hematopoietic stem cell transplant.}, journal = {Int J Hematol}, volume = {99}, year = {2014}, month = {2014 Jun}, pages = {766-72}, abstract = {

Hepatic veno-occlusive disease (VOD) is a frequent and severe complication of hematopoietic stem cell transplantation (HSCT) affecting 9.6-17.3 \% of cases. 200 HSCT, performed between January 1995 and March 2013 in our Paediatric HSCT Centre in Trieste, were retrospectively analysed to evaluate the frequency of VOD and to identify the associated risk factors. The frequency of VOD according to the Seattle criteria was 17 \%, within the range reported in literature. The mortality rate was 37.5 \% (75 out of 200 transplantations) in the general population and 73.5 \% (25 out of 34) in VOD patients (p < 0.05). Veno-occlusive disease significantly decreased from 38 \% (1995-2000) to 8 \% (2007-2013) p < 0.05. Univariate and multivariate analyses identified sepsis and pre-transplant ferritin levels above 1000 ng/ml as two significant risk factors for VOD, while the use of tacrolimus appeared to be associated with a lower VOD risk. Veno-occlusive disease still remains an important cause of transplant-related mortality even if it appears to have decreased over the last few years.

}, keywords = {Adolescent, Child, Child, Preschool, Female, Hematopoietic Stem Cell Transplantation, Hepatic Veno-Occlusive Disease, Humans, Infant, Infant, Newborn, Male, Mortality, Retrospective Studies, Risk Factors, Transplantation, Homologous, Young Adult}, issn = {1865-3774}, doi = {10.1007/s12185-014-1578-y}, author = {Maximova, N and Ferrara, G and Minute, M and Pizzol, A and Kiren, V and Montico, M and Gregori, M and Tamaro, P} } @article {1772, title = {Atlanto-axial joint involvement as exclusive manifestation of juvenile idiopathic arthritis (JIA).}, journal = {Clin Exp Rheumatol}, volume = {29}, year = {2011}, month = {2011 Jul-Aug}, pages = {755}, keywords = {Antirheumatic Agents, Arthritis, Juvenile, Atlanto-Axial Joint, Child, Dislocations, Female, Glucocorticoids, Humans, Magnetic Resonance Imaging, Pulse Therapy, Drug, Torticollis, Treatment Outcome, Uveitis}, issn = {0392-856X}, author = {Taddio, A and Pellegrin, M C and Gregori, M and Wientroub, S and Padeh, S and Lepore, L} }