Bickerstaff Brainstem Encephalitis (BBE) is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state of consciousness. Together with Guillan-Barr e Syndrome (GBS) and MillereFisher Syndrome, it forms a spectrum of postinfectious demyelinating diseases. Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on pediatric population. We reviewed all cases of pediatric BBE in the literature, to determine if any patient showed features suggestive for BBE/GBS. Data analysis focused on the diagnostic tests performed (e.g. anti-GQ1b antibodies), neuroimaging and nerve conduction studies (NCS). Further attention was given to the therapeutic management and to patients' outcome. We additionally present two previously unreported pediatric cases. Our review retrieved 19 cases of BBE/GBS, only 2 of which were originally and correctly diagnosed by the authors. The prevalence was higher in male subjects (ratio 3:1) and median age at diagnosis was 8 years. Anti-GQ1b were positive in 46% of the patients, while NCS were altered in 64%. Only 25% of the patients that underwent brain MRI showed abnormal findings. The incidence of BBE/GBS has been underrated in the past, mostly due to an underestimation of the PNS involvement. We therefore suggest to investigate all patients with a clinical picture suggestive of BBE/GBS through electroencephalogram, NCS, brain and spine MRI in order to promptly achieve the correct diagnosis.
Over the past 30 years, advances in antineoplastic treatment led to a significant increase in the survival of patients with childhood cancer. In Europe and the United States, 82% of children, adolescents, and young adults survive 5 years from the cancer diagnosis and the majority achieves long-term survival into adulthood. The impact of cancer therapy on fertility is related to the age of the patient and to the duration, dose/intensity, and type of treatment. Exposure to chemotherapy or to radiation to gonads or pituitary brings long-term complications of cancer-directed therapies that include effects on reproductive capacity. Different methods to preserve fertility can be offered. In prepubertal women, ovarian tissue freezing, in vitro maturation, and surgical movement of ovaries outside the field of irradiation are still experimental. In pubertal and postpubertal women, oocyte-embryo freezing is an established option. In men, the options are sperm cryopreservation, gonadal transposition, and testicular tissue or spermatogonial cryopreservation and reimplantation. Fertility risks and provision of strategies to minimize cancer treatment impact fertility include discussion of the tail of the option before cancer treatment. Having to make a decision in a limited time, while still coming to terms with a potentially life-threatening diagnosis, can cause patients to feel overwhelmed. To date, there are no uniform guidelines on how to approach this problem, so it is important to be aware of it for proper clinical practice.
Background Acute hematogenous osteomyelitis (AHOM) is an insidious infection of the bone that more frequently affects young males. The etiology, mainly bacterial, is often related to the patient’s age, but it is frequently missed, owing to the low sensitivity of microbiological cultures. Thus, the evaluation of inflammatory biomarkers and imaging usually guide the diagnosis and follow-up of the infection. The antibiotic treatment of uncomplicated AHOM, on the other hand, heavily relies upon the clinician experience, given the current lack of national guidelines for the management of this infection. Methods A systematic review of the studies on the empirical treatment of uncomplicated AHOM in children published in English or Italian between January 1, 2009, and March 31, 2020, indexed on Pubmed or Embase search engines, was carried out. All guidelines and studies reporting on non-bacterial or complicated or post-traumatic osteomyelitis affecting newborns or children older than 18 years or with comorbidities were excluded from the review. All other works were included in this study. Results Out of 4576 articles, 53 were included in the study. Data on different topics was gathered and outlined: bone penetration of antibiotics; choice of intravenous antibiotic therapy according to the isolated or suspected pathogen; choice of oral antibiotic therapy; length of treatment and switch to oral therapy; surgical treatment. Conclusions The therapeutic management of osteomyelitis is still object of controversy. This study reports the first Italian consensus on the management of uncomplicated AHOM in children of pediatric osteomyelitis, based on expert opinions and a vast literature review.
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