Cystic fibrosis-related liver disease is an independent risk factor for mortality and increased health care resource utilization in hospitalized pediatric patients with cystic fibrosis.

BACKGROUND: Cystic fibrosis-related liver disease (CFLD) is more prevalent in recent decades due to the increasing life expectancy of patients with cystic fibrosis (CF). There is paucity of population-level data on the impact of CFLD on hospital outcomes. METHODS: We interrogated nonoverlapping years (2003-2016) of the National Inpatient Sample and Kids' Inpatient Database to include all hospitalized patients <21 years of age with a primary diagnosis of CF within the United States. A concomitant diagnosis of cirrhosis, liver fibrosis, chronic liver disease, portal hypertension, hepatomegaly, splenomegaly, hypersplenism, and liver transplant status was considered as surrogates for the diagnosis of CFLD and was compared with CF-related hospitalizations without these diagnoses (controls) for demographics, comorbid conditions, in-hospital mortality, length-of-stay, and hospital charges. RESULTS: We evaluated 94,374 CF-related hospitalizations. The prevalence of CFLD was 5.8%. The prevalence increased from 3.1% (2003) to a peak of 7.3% (2014) with an overall increasing trend, p < 0.001. Hospitalizations with CFLD had an increased prevalence of significant comorbidities: respiratory failure,lung transplant, pulmonary hypertension, diabetes mellitus, malnutrition, Clostridioides difficile infection, cholelithiasis, anemia, and need for parenteral nutrition, p < 0.001. Multivariate regression models showed CFLD as independently associated with 2.1 (95% confidence interval [CI]: 1.5 to 2.8) times increased risk of inpatient mortality, contributed to 1.1 (95% CI: 0.89 to 1.37) additional days of hospitalization, and incurring $14,852 (95% CI: 12,204 to 17,501) excess hospital charges, p < 0.001. CONCLUSION: CFLD is associated with multiple comorbidities and is independently associated with increased risk of mortality and increased health care resource utilization in pediatric CF-related hospitalizations.

Feasibility of P wave Centric Ambulatory Electrocardiogram Monitoring in Infants and Young Children.

P wave centric ambulatory electrocardiographic (ECG) monitoring has emerged as an important tool aiding the diagnosis of arrhythmias. However, their efficacy and user experience in infants and young children are not well established. A retrospective study was performed to detail clinical and user experience of ECG monitoring using the Carnation Ambulatory Monitor (CAM) patch (Bardy Diagnostics Inc., Seattle, WA) in patients less than 10 kg. Additionally, 2 different monitor locations (over the sternum and horizontal over the left axilla) were assessed to address the optimal placement in these patients. A total of 33 CAM reports from 25 patients, aged 0 to15 months were included in the study. Mean patient age was 4.2 months ± 5.0 and mean weight was 5.3 kg ± 2.4. Thirty-six percent of patients (9/25) had known congenital heart disease. Indications for monitoring included: tachyarrhythmia (15/33, 45%), bradyarrhythmia (6/33, 18%), ectopic rhythm (9/33, 27%), cardiac tumor (1/33, 1%), and prolonged QT interval (1/33, 1%). All CAM reports showed clear, identifiable P waves which were diagnostic and lead to changes in medical management for 30% of patients (e.g., medication adjustments or discharge from cardiology care). We found the upright placement over the sternum performed better than the horizontal placement over the left axilla for small infants and children less than 10 kg. A P wave centric single-lead ECG monitor is helpful in providing accurate diagnostics tracings in infants and small children aiding in their clinical management.

The increasing prevalence and adverse impact of morbid obesity in paediatric acute pancreatitis.

OBJECTIVE: Morbid obesity is proinflammatory has been associated with adverse clinical outcomes in acute pancreatitis (AP) among adults. Obesity is increasingly prevalent in children and hence, we sought to evaluate the impact of morbid obesity on the clinical outcomes of AP using a large paediatric population-based cohort. METHODS: We analysed the US Kids' Inpatient Database between years 2003 and 2016 to include all patients (age ≤ 21 years) with a primary diagnosis of AP using specific ICD-9-CM and ICD-10-CM codes. We compared clinical outcomes between children without obesity (controls) and those with morbid obesity (cases). The primary outcome was severe AP (2012 revised Atlanta classification) and secondary outcomes included healthcare resource utilization (length of stay and hospital costs). RESULTS: Among 36 698 paediatric AP hospitalizations, 1275 (3.5%) were found to have morbid obesity. From 2003 to 2016, the proportion of children with morbid obesity among AP patients increased from 1.3% to 5.5% (P < .001). More than half (54%) of the children with morbid obesity were presented with cholelithiasis as contributive aetiology. The prevalence of severe AP was significantly higher in morbid obesity (7.3% vs 3.8%, P < .001). Multivariate analysis revealed that morbid obesity was increasingly associated with severe AP (OR 1.79, 95% CI 1.4, 2.26), prolonged hospitalization (by 0.41 days, P = .03), and higher hospital costs (by $1596, P < .001). CONCLUSION: Unlike adult population, there is a lack of other confounding comorbidities in children and this national-level analysis shows that morbid obesity independently prognosticates adverse clinical outcomes in paediatric AP.

Congenital right ventricular aneurysm with characteristics of a pseudoaneurysm.

We report a case of an isolated congenital right ventricular outpouching detected incidentally on foetal echocardiogram that was performed due to suspicion of CHD. Subsequent echocardiogram after birth revealed an aneurysm with features of a pseudoaneurysm having a thin and hypokinetic wall connected to the ventricle's cavity via a narrow neck. This pseudoaneurysm appears to be stable in size and of no clinical significance during the short-term follow-up.