Shiga toxin-producing Escherichia coli infection as a precipitating factor for atypical hemolytic-uremic syndrome.

BACKGROUND: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis. It can be classified as either typical, primarily caused by Shiga toxin-producing Escherichia coli (STEC) infection, or as atypical HUS (aHUS), which results from uncontrolled complement activation. METHODS: We report the case of a 9-year-old boy with aHUS due to compound heterozygous complement factor H-related genes (CFHR) 1/3 and CFHR1-CFHR4 deletions, leading to the development of anti-complement factor H (CFH) autoantibodies. The patient presented nephrological and neurological thrombotic microangiopathy with STEC positivity. Additionally, we provide an extensive literature review of aHUS cases initially classified as typical. RESULTS: A total of 11 patients were included, 73% of whom were pediatric. Kidney replacement therapy was required in 73% of patients. The recurrence rate was 55%. All cases were found positive for pathological variants of the complement system genes. The most commonly implicated gene was CFH, while the CFHR genes were involved in 36% of cases, although none exhibited anti-CFH autoantibodies. Anti-complement therapy was administered in 54% of cases, and none of the patients who received it early progressed to kidney failure. CONCLUSIONS: STEC infection does not exclude aHUS diagnosis, and early use of anti-complement therapy might be reasonable in life-threatening conditions. Genetic testing can be helpful in patients with atypical presentations and can confirm the necessity of prolonged anti-complement therapy.

Learning from the COVID-19 pandemic: IMCU as a more efficient model of pediatric critical care organization.

BACKGROUND: A significant proportion of children with SARs-CoV-2-related illnesses have been admitted to the Pediatric intensive care unit (ICU), although often for closer monitoring or concerns related to comorbidities or young age. This may have resulted in inappropriate ICU admissions, waste of resources, ICU overcrowding, and stress for young patients and caregivers. The Pediatric Intermediate Care Unit (IMCU) may represent an appropriate setting for the care of children whose monitoring and treatment needs are beyond the resources of a general pediatric ward, but who do not qualify for critical care. However, research on pediatric IMCUs and data on their performance is very limited. METHODS: We conducted a single-center retrospective study including all patients aged 0-18 with acute COVID-19 or multisystem inflammatory syndrome in children (MIS-C), admitted to a newly established stand-alone 12-bed pediatric IMCU at Gaslini Hospital, Genoa, Italy, between 1 March 2020 and 31 January 2022. Each IMCU room has a multiparameter monitor connected to a control station and can be equipped as an ICU room in case of need for escalation of care, up to ECMO support. IMCU and ICU are adjacent and located on the same floor, allowing a timely escalation from intermediate to critical care in the IMCU, with staff changes without the need for patient transfer. RESULTS: Among 550 patients hospitalized for acute COVID-19 or MIS-C, 106 (19.2%, 80 with acute COVID-19, and 26 MIS-C) were admitted to IMCU. Three of them (2.8%) required escalation to critical care due to the worsening of their conditions. Forty-seven patients (44%) were discharged home from the IMCU, while the remaining 57 (55%) were transferred to low-intensity care units after clinical improvement. CONCLUSIONS: In our study, the need for pediatric ICU admission was low for both acute COVID-19 patients (0.8%) and MIS-C patients (3.1%) compared to the literature data. The IMCU represented an adequate setting for children with COVID-19-related illness who need a higher level of care, but lack strict indications for ICU admission, thus preventing ICU overcrowding and wasting of economic and logistical resources. Further studies are needed to better assess the impact of an IMCU on hospital costs, ICU activity, and long-term psychological sequelae on children and their families.

Diabetes Insipidus Complicating Diabetes Mellitus Type 1: A Pituitary Abscess Diagnosis.

In this report we present a case of a 14-year-old girl with type 1 diabetes mellitus (T1DM) who experienced glycemic instability and multiple hormonal deficits, including diabetes insipidus, central hypothyroidism, and central adrenal insufficiency. Brain and sellar magnetic resonance imaging revealed a mass in the suprasellar region, which was confirmed to be a pituitary abscess through transsphenoidal biopsy. T1DM is a chronic systemic disease that can lead to suboptimal glycemic control and increased susceptibility to infections. Pituitary abscess is a rare and serious infection that can manifest with nonspecific signs and symptoms, as well as pituitary hormonal deficiencies. Currently, after a 6-year follow-up the pituitary hormone deficiencies have resolved apart from persistent partial diabetes insipidus. Through a review of the current literature, we discuss the clinical characteristics of pituitary abscess, the challenges in diagnosing it, and speculate on the potential clinical and pathophysiological relationship between this uncommon infection and T1DM in our patient.

Advanced Hybrid Closed Loop users' satisfaction of telemedicine and telenursing in pediatric and young adult type 1 diabetes.

Background and aims: The aim of the study was to evaluate the satisfaction of the use of telemedicine and telenursing in children and young adults with Type 1 Diabetes (T1D) using Advanced Hybrid Closed Loop systems (AHCL) with a focus on the role of connectivity, data download and the ease of technical steps in the set and sensor change procedures. Methods: An online anonymous survey was administered to AHCL users. The questionnaire consisted of five Clusters: Cluster A-B-C included questions related to the general satisfaction in the use of telemedicine, Cluster D was focused on the role of data download and connectivity, Cluster E was related to satisfaction in telenursing and Cluster F to the perception of ease of execution of the technical steps like changing the infusion set and the sensor. Results: We collected 136 completed questionnaires. 83.8% of AHCL users were overall satisfied with the quality of the telemedicine service. 88.2% of patients downloaded AHCL data before visits and the overall quality of televisits (data sharing, connectivity, ease of use) was satisfactory for 85.3% of users. Telenursing support during set and sensor change procedures was considered effective by 98% of AHCL users. The sensor and insulin infusion set change procedure is perceived as different for the two systems: set change simpler for Medtronic (p = 0.011) users, while sensor change was simpler for Tandem users (p = 0.009). Conclusion: Telemedicine and telenursing have an essential role in diabetology and are highly appreciated in AHCL users. The nurse support in the education of the use of AHCL systems is effective and must be implemented. Unfortunately, not all patients have the technological tools needed for downloading data at home and using telemedicine services; this represents an important challenge for the future of diabetology and for the equity in accessibility to care.

Management of Type 1 Diabetes in a school setting: effectiveness of an online training program for school staff.

Background and aims: Since Type 1 Diabetes (T1D) onset usually occurs at a young age, a relevant number of affected people attend school for most of their time; it is necessary that school personnel receive appropriate education and training. We aimed to evaluate the effectiveness of the online training program offered by IRCCS Istituto Giannina Gaslini during and after COVID-19 pandemic. Methods: The Institute's Diabetes team offered an online training program to school staff of the Region during COVID-19 pandemic. A validated questionnaire was proposed to all the schools in which training meetings were held in the previous 2 years (2020-2021 and 2021-2022). The questionnaire consisted of four sections: Section 1 (Socio-demographical data), Section 2 (Theoretical knowledge on T1D), Section 3 (Confidence in handling T1D), and Section 4 (Practical skills and Glucagon Administration). To evaluate the effectiveness of the online training program, the answers between participants (Group A) and non-participants (Group B) were then compared. Results: 225 subjects from 19 schools participated in the survey. People who participated to the training (Group A, n = 53) demonstrated better T1D theoretical knowledge compared to non-participants (Group B, n = 154; p < 0.001). Group A revealed to feel more confident in the management of children with T1D during scholastic (p = 0.006) and extra-scholastic activities (p = 0.01), in supporting the children in the administration of insulin (p < 0.001) and in recognizing hypoglycaemia (p = 0.006). Moreover, results confirmed good levels of confidence among scholastic personnel who participated in the training of administration of glucagon in case of severe hypoglycaemia. Conclusion: School staff who took part in the online training program on management of T1D showed better theoretical knowledge and better confidence in the management of daily needs and possible emergencies of students with T1D. It appears essential to offer educational programs on T1D for school staff by implementing the use of technological tools to reach a wider population. Moreover, it is advisable to offer a more practical approach, involving educational nurses.