High incidence of multisystem inflammatory syndrome and other autoimmune diseases after SARS-CoV-2 infection compared to COVID-19 vaccination in children and adolescents in south central Europe.

OBJECTIVES: To estimate the incidence and describe the spectrum of inflammatory and autoimmune diseases linked to SARS-CoV-2 infection and COVID-19 vaccination in children from two neighbouring south central European countries. METHODS: We performed a multi-centre prospective cohort study of children under 18 years diagnosed with inflammatory/autoimmune diseases linked to SARS-CoV-2 infection or COVID-19 vaccination, who were admitted to the paediatric tertiary care hospitals in Slovenia and Friuli Venezia Giulia, Italy, from January 1, 2020, to December 31, 2021. Disease incidence was calculated based on laboratory-confirmed cases only. RESULTS: Inflammatory and autoimmune diseases linked to SARS-CoV-2 were diagnosed in 192 children (127 laboratory-confirmed), of whom 112 had multisystem inflammatory syndrome (MIS-C), followed by vasculitis, neurological and cardiac diseases. Calculated risk of MIS-C was 1 in 860 children after SARS-CoV-2 infection and cumulative incidence of MIS-C was 18.3/100,000 of all children. Fifteen children had severe COVID-19. Two patients with MIS-C and a patient with myositis presented after COVID-19 vaccination. All 3 had at presentation also a serologically proven recent SARS-CoV-2 infection. After MIS-C, nine patients were vaccinated against COVID-19 and 25 patients had a SARS-CoV-2 reinfection, without recurrence of MIS-C. CONCLUSIONS: Autoimmune diseases following SARS-CoV-2 infection in children were 8.5 times as common as severe COVID-19. MIS-C was the most common manifestation and its incidence in this predominantly white population was higher than previously reported. MIS-C does not seem to recur after SARS-CoV-2 reinfection or COVID-19 vaccination. Autoimmune diseases were much more common after SARS-CoV-2 infection than after COVID-19 vaccination.

Management of the congenital solitary kidney: consensus recommendations of the Italian Society of Pediatric Nephrology.

BACKGROUND: In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), with controversial results, and a worldwide consensus on management and follow-up is lacking. In this consensus statement, the Italian Society of Pediatric Nephrology summarizes the current knowledge on CSK and presents recommendations for its management, including diagnostic approach, nutritional and lifestyle habits, and follow-up. We recommend that any antenatal suspicion/diagnosis of CSK be confirmed by neonatal ultrasound (US), avoiding the routine use of further imaging if no other anomalies of kidney/urinary tract are detected. A CSK without additional abnormalities is expected to undergo compensatory enlargement, which should be assessed by US. We recommend that urinalysis, but not blood tests or genetic analysis, be routinely performed at diagnosis in infants and children showing compensatory enlargement of the CSK. Extrarenal malformations should be searched for, particularly genital tract malformations in females. An excessive protein and salt intake should be avoided, while sport participation should not be restricted. We recommend a lifelong follow-up, which should be tailored on risk stratification, as follows: low risk: CSK with compensatory enlargement, medium risk: CSK without compensatory enlargement and/or additional CAKUT, and high risk: decreased GFR and/or proteinuria, and/or hypertension. We recommend that in children at low-risk periodic US, urinalysis and BP measurement be performed; in those at medium risk, we recommend that serum creatinine also be measured; in high-risk children, the schedule has to be tailored according to kidney function and clinical data.

How Covid-19 changed the epidemiology of febrile urinary tract infections in children in the emergency department during the first outbreak.

BACKGROUND: The first Covid-19 pandemic affected the epidemiology of several diseases. A general reduction in the emergency department (ED) accesses was observed during this period, both in adult and pediatric contexts. METHODS: This retrospective study was conducted on the behalf of the Italian Society of Pediatric Nephrology (SINePe) in 17 Italian pediatric EDs in March and April 2020, comparing them with data from the same periods in 2018 and 2019. The total number of pediatric (age 0-18 years) ED visits, the number of febrile urinary tract infection (UTI) diagnoses, and clinical and laboratory parameters were retrospectively collected. RESULTS: The total number of febrile UTI diagnoses was 339 (73 in 2020, 140 in 2019, and 126 in 2018). During the first Covid-19 pandemic, the total number of ED visits decreased by 75.1%, the total number of febrile UTI diagnoses by 45.1%, with an increase in the UTI diagnosis rate (+ 121.7%). The data collected revealed an increased rate of patients with two or more days of fever before admission (p = 0.02), a significant increase in hospitalization rate (+ 17.5%, p = 0.008) and also in values of C reactive protein (CRP) (p = 0.006). In 2020, intravenous antibiotics use was significantly higher than in 2018 and 2019 (+ 15%, p = 0.025). Urine cultures showed higher Pseudomonas aeruginosa and Enterococcus faecalis percentages and lower rates of Escherichia coli (p = 0.02). CONCLUSIONS: The first wave of the Covid-19 pandemic had an essential impact on managing febrile UTIs in the ED, causing an absolute reduction of cases referring to the ED but with higher clinical severity. Children with febrile UTI were more severely ill than the previous two years, probably due to delayed access caused by the fear of potential hospital-acquired Sars-Cov-2 infection. The possible increase in consequent kidney scarring in this population should be considered.

Unarousable child with a short bowel.

Unarousable child with short bowelA 4-year-old boy was admitted with progressive lethargy of a few hours' duration and no other symptoms. His medical history was relevant for short bowel syndrome (SBS), following neonatal volvulus, with residual bowel length of 23 cm and intact ileocecal valve. He had similar self-limiting episodes in the past, after weaning parenteral nutrition, especially after eating large meals. The day before, he had consumed a large amount of apples.Arterial blood gas (ABG) analysis showed metabolic acidosis with normal lactacidaemia (pH 7.09, pCO2 19 mm Hg, pO2 101 mm Hg, HCO3 5.8 mmol/L, BE -24, anion gap 29.4, chloride 116 mmol/L, L-lactate level 4 mmol/L).On admission, the child could be awakened, but he was confused with slurred speech (Glasgow Coma Scale 14), with a body temperature of 37 C°, a heart rate of 125 beats/min and a respiratory rate of 38 breaths/min. The abdomen was distended, without guarding and with normal bowel sounds. Blood glucose levels were normal, as well as white blood cell count, liver and kidney function test and C reactive protein. An abdominal ultrasound ruled out an intussusception. An abdominal X-ray was performed too (see figure 1).

Is ultrasonography mandatory in all children at their first febrile urinary tract infection?

BACKGROUND: This study investigated whether performing kidney ultrasound (KUS) only in children presenting either a pathogen other than E. coli at their first febrile urinary tract infection (fUTI) or experiencing fUTI recurrence would increase missed diagnoses of kidney anomalies. METHODS: Patients aged 2-36 months with fUTI who underwent KUS evaluation from 2 January 2013 to 31 June 2018 were enrolled. Cystourethrography was performed after pathological KUS or recurring fUTIs. Thereafter, we retrospectively assessed the detection rate of kidney anomalies through performing KUS only in patients with atypical pathogen at first fUTI or with recurring fUTIs. RESULTS: In 263 patients included, the isolated pathogen was E. coli in 223 cases (84.8%) and atypical in 40 cases (15.2%). KUS detected kidney anomalies in 14/223 (6.3%) of fUTIs caused by E. coli and in 11/40 (27.5%) of fUTIs caused by an atypical pathogen (OR 5.5, 95%CI 2.5-14.5). Cystourethrography was performed in 40 patients and vesicoureteral reflux (VUR) found in 20 cases. None of the high grade VUR diagnoses or other kidney anomalies would have been lost through a different diagnostic protocol that required the presence of an atypical pathogen at the first fUTI or a fUTI recurrence to perform the KUS. CONCLUSIONS: A diagnostic protocol that requires presence of an atypical pathogen at the first fUTI or a second episode of fUTI to perform the KUS would allow a reduction in the number of negative ultrasounds with a negligible risk of missed diagnoses of kidney anomalies.

Returning to dialysis after kidney allograft failure: the experience of the Italian Registry of Paediatric Chronic Dialysis.

BACKGROUND: The need for dialysis after kidney allograft failure (DAGF) is among the top five reasons for dialysis initiation, making this an important topic in clinical nephrology. However, data are scarce on dialysis choice after transplantation and clinical outcomes for DAGF in children. METHODS: Patients receiving chronic dialysis < 18 years were recorded from January 1991 to January 2019 by the Italian Registry of Pediatric Chronic Dialysis (IRPCD). We investigated factors influencing choice of dialysis modality, patient outcome in terms of mortality, switching dialysis modality, and kidney transplantation. RESULTS: Among 118 patients receiving DAGF, 41 (35%) were treated with peritoneal dialysis (PD), and 77 (65%) with haemodialysis (HD). Significant predictors for treatment with PD were younger age at dialysis start (OR 0.85 per year increase [95%CI 0.72-1.00]) and PD use before kidney transplantation (OR 8.20 [95%CI 1.82-37.01]). Patients entering DAGF in more recent eras (OR 0.87 per year increase [95%CI 0.80-0.94]) and with more than one dialysis modality before kidney transplantation (OR 0.56 for being treated with PD [0.12-2.59]) were more likely to be initiated on HD. As compared to patients on HD, those treated with PD exhibited increased but non-significant mortality risk (HR 2.15 [95%CI 0.54-8.6]; p = 0.28) and higher prevalence of dialysis-related complications during DAGF (p = 0.002) CONCLUSIONS: Patients entering DAGF in more recent years are more likely to be initiated on HD. In this specific population of children, use of PD seems associated with a more complicated course. A higher resolution version of the Graphical abstract is available as Supplementary information.

Mental health problems in children admitted with physical symptoms.

The aim of the study was to investigate the burden of mental health problems in children and adolescents admitted to a pediatric ward with physical complaints. We conducted a retrospective study, considering all patients admitted to the pediatric ward of the IRCCS Burlo Garofolo, Trieste, Italy, between January 2015 and September 2016. We selected all patients, from 5 to 17 years old, who were admitted with physical complaints and were discharged with a diagnosis suggestive of a mental health problem: somatic symptom disorder, anxiety disorder, depressive disorder, factious disorders. For every patient, we collected demographic features, medical characteristics, health care services utilization, length of hospital stay, and after discharge referral. We selected 1456 patients; of these, 101 (6.9%) revealed a mental health problem. The median duration of symptoms was 5 months (IQR 1.5-12), and pain was the main reported symptom (69%). Of the 101 patients, 23 (23%) were affected by a previously documented chronic organic disease. Somatic symptom disorder was the most common diagnosis. In 69/78 patients (88%), a loss of social contacts emerged; 49/95 patients frequenting school (51%) had chronic school absenteeism in the previous school year.Conclusion: A considerable proportion of patients admitted to a pediatric ward with physical complaints have mental health problems. What is Known: • It has been suggested that mental health problems in children and adolescents are increasing and frequently burden on pediatric healthcare services, but how they impact on a general pediatric ward is not clear. What is New: • Among 1456 patients admitted to a general pediatric ward with physical symptoms, 101 patients (6.9%) had a mental health problem. Among them, pain was the most commonly reported symptom and somatic symptom disorder was the commonly reported diagnosis.

A toddler with a persisting limp after a minor trauma.

A 3-year-old toddler was admitted for a 5-day history of worsening painful limping on his left leg. History was remarkable only for a minor trauma 2 days before the onset of symptoms; the boy fell on his buttocks but was walking normally in the following days. No fever was reported. Pain was also present at night, with no response to oral ibuprofen.On physical examination, the patient refused to stand on his left leg, palpation of the left buttock evoked pain, and exorotation and abduction of the left hip were only moderately limited, without local signs of inflammation such as redness, swelling or skin warming. Blood tests showed elevated erythrocyte sedimentation rate (ESR) (98 mm/hour, normal value <20 mm/hour) with normal C reactive protein (CRP) level (0.5 mg/dL, normal value <0.5 mg/dL). His white cell count was 12 110 x 109/L, haemoglobin was 127 g/L and PLT was 430 x 109/L. Creatine kinase values were within the normal range.An X-ray of the pelvis was unremarkable. An ultrasound of the left hip showed a 2 mm articular effusion.

Emergency department attendance for injury and behaviours suggestive of attention deficit hyperactivity disorder (ADHD): a cross-sectional study.

BACKGROUND: The study aimed to investigate if the behaviours suggestive of ADHD were more frequent in a population of children attending the Emergency Department (ED) for injuries, rather than for other causes. METHODS: A cross-sectional study was carried out. Patients, aged 6 to 17 years, attending the ED for acute injuries and other causes were considered cases and controls, respectively. We used a questionnaire, which investigates the presence in the child of inattention, hyperactivity, and impulsivity. The primary outcome was the number of children with behaviours suggestive of ADHD in cases and controls. RESULTS: Five hundred forty-five children were enrolled, 251 with injuries and 294 with other complains. Twenty two out of two hundred fifty one (9%) children visited for injuries, and 30 out of 294 (10%) visited for other causes had behaviours suggestive of ADHD (p = 0.661). Among these cases, children with evocative ADHD scores had a higher probability (OR 4.52; 95% CI 1.45-14.04; p = 0.009) of having had more than five previous ED accesses due to injury, compared to the others. CONCLUSIONS: This study did non shown a difference in behaviours suggestive of ADHD between cases and controls, but identified a population of children with behaviours suggestive of ADHD who more frequently access the ED for injuries.

Familial hypogammaglobulinemia with high RTE and naïve T lymphocytes.

Most of primary immunodeficiencies with hypogammaglobulinemia are associated with reduced memory B cells. T cell development may be interesting as well, but increased recent thymic emigrants are rarely reported in these patients. We report the case of a family (mother and her two sons) diagnosed with common variable immunodeficiency 10 due to a mutation in the NFKB2 gene. Laboratory findings showed that all three patients presented hypogammaglobulinemia, reduced memory B cells and elevated naïve T lymphocytes and recent thymic emigrants. This feature, in the absence of glucocorticoid deficiency, may suggest a primary thymic dysfunction. Interestingly, the mother presented the worst immune phenotype, as regards both antibody production and NK function, indicating that immune function may deteriorate in the course of time. We conclude that close monitoring of immune functions may widen the knowledge on the CVID10 and improve the patients' care.

Administering analgesia sublingually is a suitable option for children with acute abdominal pain in the emergency department.

AIM: Acute abdominal pain is a frequent complaint in children attending emergency departments. The aim of this study was to investigate the pain score reductions when children with acute abdominal pain received medication sublingually. METHODS: We carried out a multicentre randomised controlled trial in three children's hospitals in Italy between March 2015 and June 2017. Children from four to 18 years of age with acute abdominal pain were recruited if their self-reported pain was at least six on a scale from 0-10. The children were randomised to receive ketorolac 0.5 mg/kg (n = 70) or tramadol 2 mg/kg (n = 70) sublingually or a melt in the mouth powder of 20 mg/kg paracetamol (n = 70). The main study outcome was the pain scores for the three drugs after two hours. RESULTS: The 210 children (58.6% girls) had a median age of 12 years with an interquartile range of 9-14.3. The median pain scores at two hours were not significantly different between ketorolac 2.0 (interquartile ranges, IQR 0.0-4.3) and tramadol 3.0 (IQR 1.0-5.0) vs paracetamol 3.0 (IQR 0.8-5.0). The median pain reductions were all 5.0 points. CONCLUSION: Delivering analgesia sublingually was a suitable option for pain relief in children with acute abdominal pain in the emergency department.