Subclinical Leber's hereditary optic neuropathy with pediatric acute spinal cord onset: more than meets the eye.

BACKGROUND: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by visual loss consequent to optic nerve atrophy. In some cases, LHON is associated with heterogeneous neurological extraocular manifestations and is referred to as "Leber plus disease"; rarely it is associated with a multiple sclerosis (MS)-like syndrome known as Harding disease, but no pediatric extraocular acute spinal onset is reported. CASE PRESENTATION: We describe the case of a 5-year-old girl carrying the G3460A mtDNA mutation who was referred to clinical examination for bilateral upper and lower limb weakness with no sign of optic neuropathy. Spinal cord MRI showed hyperintense signal alterations in T2-weighted and restricted diffusion in DWI sequences in the anterior portion of the cervical and dorsal spinal cord resembling a spinal cord vascular injury. No association between this mutation and pediatric spinal cord lesions has previously been reported. Alternative diagnostic hypotheses, including infective, ischemic and inflammatory disorders, were not substantiated by clinical and instrumental investigations. CONCLUSIONS: Our case reports a novel pediatric clinical manifestation associated with the m.3460G > A mtDNA mutation, broadening the clinical spectrum of this disease. Early identification of new cases and monitoring of carriers beginning in childhood is important to prevent neurological deterioration and preserve long-term function.

Epidemiology of bacterial meningitis in Lombardy Region in the period 2000-2006.

Epidemiological study of bacterial meningitis, particularly those forms susceptible to vaccination, is an indispensable tool in choosing vaccination strategies. Lombardy Region, where approximately 20% of the Italian population resides, has conducted an in-depth study on invasive meningococcal diseases and pneumococcal meningitis using available health statistics and performing a control of single cases to achieve complete knowledge of the phenomenon. The results, relating to the period 2000-2006, indicate limited incidence rates both for the general population and childhood age groups that do not justify hypotheses of a universal offering of the specific vaccines. However, the healthcare system must equip itself with additional tools for the identification of the serogroups and serotypes responsible for disease as a further support for possible choices modifying the current vaccination policies.

Decreased Antibiotic Prescription in an Italian Pediatric Population With Nonspecific and Persistent Upper Respiratory Tract Infections by Use of a Point-of-Care White Blood Cell Count, in Addition to Antibiotic Delayed Prescription Strategy.

The aim of this study was to test, in delayed antibiotic strategy, if the usages of a point-of-care leukocyte count would significantly decrease the prescription rate of antibiotics for children with nonspecific upper respiratory tract infections. A prospective clinical trial was performed in 23 primary care pediatric doctors' offices on children with nonspecific upper respiratory tract infection with fever for at least 48 hours. The children were randomized into 2 groups: one using a point-of-care white blood cell (WBC) count as guidance and the other prescribing antibiotics to all children, according to delayed antibiotics prescription strategy. A total of 792 patients participated. In the WBC group (n = 437), 56 patients had WBC >15 000/mm(3) and received antibiotics. At follow-up, an additional 44 children received antibiotics. In the control group (n = 355), antibiotics were prescribed to all children. The reduction of antibiotic usage was 77% between the groups. The decrease in antibiotic usage gave no influence on recovery, complications, or other medical outcome.

The decision-making process in antibacterial treatment of pediatric upper respiratory infections: a national prospective office-based observational study.

BACKGROUND: The identification of patient management practices and the sources of medical information is crucial for rationalizing the treatment of respiratory tract infections, whose high incidence, especially in children, makes them one of the maior areas of unnecessary health expenditure. MATERIALS AND METHODS: This national prospective study was designed to investigate the diagnostic and prescribing habits of 100 office-based pediatricians managing upper respiratory tract infections in 1111 pediatric patients (604 males, mean age 6.7962.77 years; 507 females, mean age 6.7362.8 years) sequentially enrolled when an antibiotic treatment was deemed necessary. RESULTS: The most frequently diagnosed diseases were acute tonsillopharyngitis (56.2%) and acute otitis media (18.1%). Penicillins were prescribed in 34.3% of the cases, cephalosporins in 38.1%, and macrolides in 26.1%: oral drugs accounted for 92.2% of the prescriptions. The treatments were administered once or twice daily in 75.8% of the patients, and prescribed for 8 days in more than 80%; 76.7% also received supportive or symptomatic treatment (antipyretics, corticosteroids, cough suppressants and non-steroidal anti-inflammatory drugs). Laboratory or radiologic investigations were rarely requested. The main sources of medical information indicated by the participating pediatricians were pharmaceutical companies (35.6%) and meeting or congress reports (27.3%). CONCLUSIONS: The results indicate that more active education is still needed to improve the decision-making processes of office-based pediatricians.

Structural and immunological characteristics of chronically inflamed adenotonsillar tissue in childhood.

Recurrent or chronic adenotonsillar infections mainly affect children and frequently involve otherwise healthy subjects. Therefore, having excluded systemic immunological deficiencies, this disease may be due to a local dysfunction of the epithelial structures at either the rhino or oropharyngeal level. The aim of the present investigation was to analyze structural and immunological aspects of tonsils and adenoids in subjects who underwent adenotonsillectomy because of recurrent inflammatory episodes with fever. Histological studies and analyses of the cytokine patterns were carried out in palatine tonsils and adenoid samples from 105 patients who underwent adenoidectomy and bilateral extracapsular tonsillectomy for chronic inflammatory hypertrophy of these organs; 46 of the 105 cases examined presented hyperkeratosis of the crypt epithelium; in the remaining 59, the epithelium was hyperplastic with no signs of keratosis. Scanning electron microscopy revealed a continuous epithelial surface of polygon-shaped flattened cells with fissures towards the cryptic depressions. Titration of interleukin-1beta and tumor necrosis factor alpha in serum and tissues demonstrated higher concentrations in the adenotonsillar specimens, whereas the rise in interleukin-6 was more modest.

Exploring the variability in antibiotic prescribing profiles among paediatricians from two different areas of Italy.

We carried out a multicentre community-based study in order to describe the antibiotic therapeutic approach of paediatricians from two different areas of Italy in the treatment of respiratory tract infection (RTIs), and to assess which factors are involved in a possible variability of prescribing habits. Forty paediatricians participated in the study between October 1998 and April 1999. They had to complete a questionnaire for each therapeutic intervention resulting in an antibiotic prescription. A logistic regression model was used to identify possible predictors in choosing parenteral antibiotics for the treatment of RTIs. In 2 975 questionnaires of antibiotic treatment, RTIs represented 90.2% of the total antibiotics used. Upper respiratory tract infections were the most commonly treated diagnostic group (59.6%), followed by lower respiratory tract infections (20.4%), and middle ear infections (19.8%). Statistically significant differences between northern and southern Italy were reported in the antibiotic prescription profile and the duration of the therapy. Another marked difference was reported in the frequency of laboratory analysis requests. The logistic regression model indicated that the use of parenteral antibiotics appears significantly related to the type of infections [lower RTIs: (OR: 3.99; 95% CI: 2.49-6.37)], the geographic location [northern Italy: (OR: 0.20; 95% CI: 0.20-0.39)], and the presence of concurrent diseases (OR: 3.21; 95% CI: 1.46-7.02). The lack of adherence to clinical guidelines and the marked variability of antibiotic prescription rates between different areas of the country appear to be related to factors other than bacterial resistance, and highlight the importance of carrying out educational programmes targeted at the national level for improving the antibiotic prescription habits for the treatment of RTIs.