Early anakinra treatment improves cardiac outcome of multisystem inflammatory syndrome in children, regardless of disease severity.

OBJECTIVE: The main aim of this study was to define the best treatment option for multisystem inflammatory syndrome in children (MIS-C) and to analyse the role of anakinra. METHODS: This is a multicentre retrospective cohort study. Patients were treated according to the attending physician's decision. The patients were divided into four groups on the basis of the first treatment at time of admittance: (i) IVIG, (ii) IVIG and methylprednisolone (≤2 mg/kg/day), (iii) IVIG with high-dose methylprednisolone (>2 mg/kg/day) and (iv) anakinra with or without IVIG and/or methylprednisolone. Primary outcomes were defined as the presence of at least one of the following features: death, the failure of initial treatment, meaning the need for additional treatment for clinical worsening and cardiac involvement at the end of follow-up. RESULTS: Two hundred thirty-nine patients were recruited. At univariate analysis, persistent heart involvement at discharge was more frequent in those not receiving anakinra as initial treatment (3/21 vs 66/189; P = 0.047). After comparisons between the four treatment regimens, adjusting for the propensity score, we observed that early treatment with anakinra was associated with a lower probability of developing persistent heart disease at the end of follow-up (odds ratio: 0.6; 95% CI: 0.4-1.0). CONCLUSION: We report that early treatment with anakinra is safe and very effective in patients with severe MIS-C. In addition, our study suggests that early treatment with anakinra is the most favourable option for patients with a higher risk of developing a severe disease outcome.

[Guillain-Barré syndrome and its variants: Miller-Fisher syndrome. Description of a clinical case in pediatric age.] / La sindrome di Guillain-Barré e le sue varianti: la sindrome di Miller-Fisher. Descrizione di un caso clinico in età pediatrica.

Miller-Fisher syndrome is a rare acquired nerve disease related to Guillain-Barré syndrome. Clinical features include asthenia, ocular muscle weakness with ophthalmoplegia, impaired limb coordination with instability, and absence of tendon reflexes. Swallowing disorders and rarely respiratory failure may be associated. The article aims to summarize, starting from the presentation of a clinical case, the latest updates which, in clinical practice, can be useful for a correct diagnosis and treatment of this condition which concerns both adult and pediatric patients.

Multi-System Inflammatory Syndrome in Children (MIS-C) with Retropharyngeal Involvement Mimicking Abscess: the Therapeutic Role of Anakinra.

Multi-System Inflammatory Syndrome in Children (MIS-C) is a new diagnostic entity that appeared with the Covid-19 pandemic, and that only concerns the pediatric age. Early diagnosis and early immunomodulatory treatment lead most of the time to a complete resolution of the pathology. We present a case of MIS-C, whose main clinical feature was neck pain due to inflammation of retropharyngeal tissues. The use of anakinra was critical for healing.

[Unicentric Castleman disease: a blind lymph node.] / Malattia di Castleman unicentrica: un linfonodo nascosto.

The paper reports the case of a 13-year-old female adolescent presenting with persistent fever. She had no other significant symptoms or signs. Laboratory examinations showed mild anemia and elevated C-reactive protein (CRP) and erythrosedimentation rate (ERS). The abdominal ultrasonography revealed para-aortic lymphadenopathy that was confirmed by magnetic resonance imaging (MRI) and positron emission tomography-computed tomography (PET/CT) that showed no other locations. The patient underwent laparoscopic excision but complete removal was not possible due to the position of the mass. The histological exam documented unicentric Castleman's disease. After surgery a clinical improvement was assisted but with persistence of very high CPR, ERS and serum amyloid. According to guidelines, she was treated with tocilizumab achieving complete remission of indices of inflammation. In the case with symptomatic unresectable unicentric Castleman's disease treatment with anti-IL-6 agents should be considered.

Methicillin Resistant Staphylococcus Aureus Shoulder Pyomyositis with Multifocal Lung Infiltrations.

Pyomyositis is a rare condition in temperate climates. We present a case of Methicillin Resistant Staphylococcus aureus pyomyositis of the shoulder complicated by multifocal lung infiltrations, treated successfully with antibiotic therapy. After excluding shoulder septic arthritis, a low threshold of suspicion for the diagnosis of shoulder pyomyositis should be applied to patients with persistent fever, pain, and decreased range of shoulder motion. A prompt diagnosis and a rapid rise in antibiotic therapy are important to avoid local and systemic complications.