Objective
Nocturnal hypoventilation (NH) is a complication of respiratory involvement in neuromuscular disorders (NMD) that can evolve into symptomatic daytime hypercapnia if not treated proactively ...with non-invasive ventilation. This study aimed to assess whether NH can be detected in the absence of other signs of nocturnal altered gas exchange.
Methods
We performed nocturnal transcutaneous coupled (tc) pCO2/SpO2 monitoring in 46 consecutive cases of paediatric-onset NMD with a restrictive respiratory defect (forced vital capacity < 60%). Nocturnal hypoventilation was defined as tcPCO2 > 50 mmHg for > 25% of recorded time, and hypoxemia as tcSpO2 < 88% for > 5 minutes. Daytime symptoms and bicarbonate were recorded after overnight monitoring.
Results
Twenty-nine of 46 consecutive patients showed NH. Twenty-three patients did not have nocturnal hypoxemia and 18 were clinically asymptomatic. In 20 patients, PaCO2 in daytime blood samples was normal. Finally, 13/29 patients with NH had isolated nocturnal hypercapnia without nocturnal hypoxia, clinical NH symptoms, or daytime hypercapnia.
Conclusions
Paediatric patients with NMD can develop NH in the absence of clinical symptoms or significant nocturnal desaturation. Therefore, monitoring of NH should be included among nocturnal respiratory assessments of these patients as an additional tool to determine when to commence non-invasive ventilation.
In this observational study, we report the clinical, therapeutics and outcome features of 23 patients with multisystem inflammatory syndrome (MIS-C) who have been treated in Gaslini Children Hospital ...(Genoa, Italy) with a multistep antinflammatory treatment protocol, based on disease severity at admission. Patients were initially assigned to four severity classes on admission and treated accordingly. The therapeutic options ranged from IV immunoglobulin alone to a combination of IVIG plus pulses of methylprednisolone plus anakinra for patients with marked cardiac function impairment or signs of macrophage activation syndrome, with rapid treatment escalation in case of inadequate therapeutic response. With the application of this therapeutic strategy, no patient required admission to Intensive Care Unit (ICU) or invasive mechanical ventilation, and no inotropic drugs administration was required. Early aggressive treatment of MIS-C, with therapeutic interventions modulated based on the severity of clinical manifestations may help to prevent the progression of the inflammatory process and to avoid the need of admission to the ICU. A timely intervention with anti-IL-1 blockers can play a pivotal role in very severe patients that are at risk to have an incomplete response to immunoglobulins and steroids.
Abstract
Eating epilepsy (EE) is a form of reflex epilepsy in which seizures are triggered by eating. It is a rare condition but a high prevalence has been reported in Sri Lanka. In EE, the ictal ...semiology includes focal seizures with or without secondary generalization or generalized seizures. Some cases are idiopathic while focal structural changes on imaging, if present, are often confined to the temporal lobe or perisylvian region. On the other hand, some cases support the hypothesis of a genetic aetiology. The prognosis of EE is extremely variable due to the different nature of the underlying disorder. We describe two patients with symptomatic eating epilepsy, a 13-year-old boy with a bilateral perisylvian polymicrogyria and a 2-year-old boy with a genetic cause. The presence of structural lesions or the dysfunction of specific cortical regions in the context of a germline genetic alteration might lead to a hyperexcitation fostering the epileptogenesis. We review the available literature to clarify the aetiopathogenesis and the mechanisms underlying EE to improve the diagnosis and the management of these rare conditions.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
We describe an atypical case of Whipple disease exclusively involving the spinal cord in an adolescent receiving immunosuppressive therapy for systemic lupus erythematosus. The diagnosis was ...particularly difficult since lupus and Whipple disease can present similar clinical features and the patient’s prolonged contact with sewage was initially not mentioned. A literature review of the clinical, imaging, diagnostic, and therapeutic challenges of Whipple disease is also performed.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Benign acute childhood myositis (BACM) is a self-limited syndrome associated with viral infections characterized by symmetric lower extremity pain typically affecting school-aged children. Evolution ...in rhabdomyolysis and kidney damage is rarely reported. Despite this, the acute presentation commonly concerns both parents and health care providers, often leading to unnecessary workup. The aim of the study was to determine the features and outcome of a large series of children with BACM identifying a management pathway for pediatricians in Emergency Department (ED).
We conducted a retrospective study of patients with BACM managed in 2 Italian pediatric ED during a period of 8 and a half years. Demographic data, clinical, and laboratory results were extracted from electronic medical records. Recurrence, complications, treatments, and outcomes were also recorded. Descriptive statistics were produced for first-episode patients and for those with recurrence of myositis. A comparison between groups was performed.
One hundred and thirteen patients with BACM were identified. Ninety-two children (65 males) had a single episode, while ten (nine males) had recurrence. The mean age at presentation was 6.0 years (range 2-13,2). All patients had normal neurological examination and no one developed myoglobinuria, or renal failure. At first evaluation median CK level was 1413 UI/l (normal values < 150 U/L). Median CK of "recurrent" patients was higher than "non-recurrent" (2330 vs 1150 U/L, p = 0.009). Viral studies were positive in 51/74 cases, with high prevalence of Influenza viruses. Ninety-six patients (85%) were hospitalized with a median of 4 days. No patients had any residual muscular impairment.
BACM has an excellent prognosis. Severe pathological conditions can be excluded with a complete history and clinical examination and simple blood and urine tests, avoiding unnecessary diagnostic investigations. Most patients may be discharged home from ED recommending hydration, rest, analgesics and careful follow-up.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Abstract
From March 2020 to July 2022, in Liguria region (North-West Italy) incidence of MIS-C among pediatric patients infected by SARS-CoV-2 was 38.7/100.000, which is higher than that of ...myocarditis after COVID-19 vaccination. In our opinion severity of MIS-C-related cardiac disease outweigh the risk of myocarditis after COVID-19 vaccine.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
The aim of this study was to describe the 2022-2023 bronchiolitis epidemic season (the second after COVID-19 pandemic and the first without social restriction), focusing on patients discharged home ...from a pediatric emergency department (PED) and on those revisited within 72 h. We performed a retrospective observational study in an Italian tertiary care children's hospital, reviewing PED accesses from 1 October 2022 to 31 March 2023. The number of hospitalizations for bronchiolitis was extracted from hospital discharge forms. A total of 512 patients diagnosed with bronchiolitis were admitted to PED (2.8% of total admissions). Accesses increased sharply from November to January, with a peak in December, in both admissions and hospitalizations. More than half of the patients (55.5%) were safely discharged home, while 38 (13.4%) came back to PED for a revisit. Overall PED accesses and hospitalizations for bronchiolitis increased since the previous epidemic season, and particularly compared to the pandemic and pre-pandemic eras. Empowering the collaboration between all healthcare provisioners is fundamental to suitable management of patients. Monitoring the epidemiology and seasonality of bronchiolitis is a starting point for an effective internal organization of pediatric departments and to further evaluate its socio-economic burden.
PDF
