A bullous rash.

A 3-year-old boy presented with a 5-day history of bullous skin lesions localised mainly in the upper and lower limbs and in the genital region (figure 1). Lesions were not pruritic nor painful and showed a central crust. There was no family history of skin disorders or autoimmune diseases. The child never had fever and his physical examination was otherwise unremarkable.

COVID-19 Pneumomediastinum: Possible Role of Transesophageal Echo in Bedside Percutaneous Bicaval Double-Lumen ECMO Cannulation in Children. A Case Report.

COVID-19 is generally uneventful in children. Only 8% of severe acute respiratory distress syndrome corona virus 2 pediatric patients require intensive care; of these, 1% may need extracorporeal membrane oxygenation. Preexisting medical conditions are an independent risk factor for pediatric intensive care unit admission. We describe the case of an 11-year-old girl with adenosine deaminase 2 deficiency who presented severe COVID-19 acute respiratory distress syndrome, complicated by a massive air leak syndrome. The respiratory failure, refractory to conventional support, required veno-venous extracorporeal membrane oxygenation. To prevent viral diffusion, bicaval double-lumen cannulation was performed percutaneously at the bedside under exclusive echo guidance. Because of pneumomediastinum, pneumothorax, and subcutaneous emphysema, ultrasound visualization of the heart was possible only with transesophageal echo. To our knowledge, this is the first description of a transesophageal echo guided bedside percutaneous bicaval double-lumen extracorporeal membrane oxygenation cannulation in a pediatric patient. Pitfalls of the technique are highlighted.

Preliminary data on prednisone effectiveness in children with Sydenham chorea.

The objective of the study is to evaluate the efficacy of corticosteroids in Sydenham chorea. This is a retrospective observational study. Clinical information of children with Sydenham chorea were collected. Outcome of Sydenham chorea was evaluated in consideration of presence or absence of corticosteroid therapy. Thirty patients were enrolled. A total of 15 were treated with prednisone, 15 received symptomatic drugs or no treatment. Patients who were treated with prednisone showed faster improvement (4 vs 16 days; p = 0.002) and shorter median time of remission (30 vs 135 days; p < 0.001).Conclusion: Our study showed that corticosteroid therapy is an effective treatment of Sydenham chorea.What is Known:• Steroid treatment in Sydenham chorea is widely used but it is not standardized.• Few manuscript report a beneficial use of steroids in Sydenham chorea if compared with no treatment.What is New:• Steroid treatment seems to be effective in both clinical remission and clinical improvement of symptoms among patients with Sydenham chorea.• Steroid treatment seems to be superior to conventional treatment.

Familial neurohypophyseal diabetes insipidus in 13 kindreds and 2 novel mutations in the vasopressin gene.

BACKGROUND: Autosomal dominant neurohypophyseal diabetes insipidus (adNDI) is caused by arginine vasopressin (AVP) deficiency resulting from mutations in the AVP-NPII gene encoding the AVP preprohormone. AIM: To describe the clinical and molecular features of Italian unrelated families with central diabetes insipidus. PATIENTS AND METHODS: We analyzed AVP-NPII gene in 13 families in whom diabetes insipidus appeared to be segregating. RESULTS: Twenty-two patients were found to carry a pathogenic AVP-NPII gene mutation. Two novel c.173 G>C (p.Cys58Ser) and c.215 C>A (p.Ala72Glu) missense mutations and additional eight different mutations previously described were identified; nine were missense and one non-sense mutation. Most mutations (eight out of ten) occurred in the region encoding for the NPII moiety; two mutations were detected in exon 1. No mutations were found in exon 3. Median age of onset was 32.5 months with a variability within the same mutation (3 to 360 months). No clear genotype-phenotype correlation has been observed, except for the c.55 G>A (p.Ala19Thr) mutation, which led to a later onset of disease (median age 120 months). Brain magnetic resonance imaging (MRI) revealed the absence of posterior pituitary hyperintensity in 8 out of 15 subjects, hypointense signal in 4 and normal signal in 2. Follow-up MRI showed the disappearance of the posterior pituitary hyperintensity after 6 years in one case. CONCLUSION: adNDI is a progressive disease with a variable age of onset. Molecular diagnosis and counseling should be provided to avoid unnecessary investigations and to ensure an early and adequate treatment.

Sedation and analgesia in children with cerebral palsy: a narrative review.

BACKGROUND: Patients with cognitive impairment due to cerebral palsy experience pain more often than healthy peers and frequently require diagnostic and therapeutic painful procedures. Analgesia and procedural sedation outside the operating room are often required, but they may not adequately be provided because of the inability to accurately recognize and classify the state of pain and for the perceived higher risk of complications. DATA SOURCES: We reviewed the available literature to highlight the specific risk factors and area of criticism, that should be further improved. We searched the Cochrane Library, Medline, Pubmed from 1987 to September 2018 using key words such as 'cerebral palsy and children and pain' or 'sedation and cerebral palsy and children'. RESULTS: While different pain scales are useful in recognizing pain expressions, anxiety scales are not available. Moreover, studies on non-pharmacological techniques do not always have comparable results. Several risk factors, from anatomic abnormalities to liver and kidney functioning, should be kept in mind before proceeding with sedation. CONCLUSIONS: Large trials are needed to assess the impact of non-pharmacological techniques and to evaluate which pain control strategy (pharmacological and non-pharmacological) should be used in different settings.

Use of ketamine by paediatricians in Italian paediatric emergency departments: a missed opportunity?

Procedural sedation and analgesia with ketamine are part of daily practice for children undergoing painful procedures in the paediatric emergency department (ED) of North America. A massive number of studies demonstrate ketamine's safety and efficacy in the hands of trained ED paediatricians, with few severe adverse events (SAEs) recorded. Since there are no data on ketamine's usage in Italian paediatric EDs, we created a survey to examine procedural sedation with ketamine in the EDs of the Italian PIPER (Pain in Paediatric Emergency Room) group, which includes 36 paediatric EDs providing 1.4 million paediatric visits each year. Results were reviewed using frequencies to describe responses. Thirty-two out of 36 centres replied to the questionnaire. In 6 (19%) out of 32 centres, ketamine is not used at all in the paediatric ED. In 6 centres (23%) of 26 which use ketamine, this drug is autonomously administered by the emergency paediatrician, whereas in 20 (77%) of them it is exclusively managed by the anaesthesiologist on call.Conclusion: ketamine is autonomously administered only by a small percentage of Italian emergency paediatricians. There is an increasing need for implementation of procedural sedation training and use of ketamine in the everyday practice outside the operating room in paediatric EDs. What is Known: • Ketamine is safely and efficaciously administered for children's procedural sedation and analgesia by trained emergency paediatricians in the everyday practice outside the operating room in North America. • In the Italian setting, there are no data at all concerning ketamine's usage by the emergency paediatricians for procedural sedation and pain control. What is New: • In this study emerged that ketamine is poorly administered by Italian emergency paediatricians for procedural sedation and analgesia outside the operating room. • A great deal of educational effort should be made to widen ketamine based procedural sedation availability in Italian emergency departments by spreading specific training tracks and guidelines.