Pulmonary haemorrhage in neonates: Systematic review of management.

AIM: Pulmonary haemorrhage (PH) is an acute catastrophic event with low incidence yet high mortality among neonates. We aimed to systematically review the management of PH. METHODS: A search was carried out of the PubMed, EMBASE and Cochrane databases according to the PRISMA guidelines. Data were extracted on study design and size, patient demographics, primary and adjunctive treatment methods, and treatment outcomes. RESULTS: Sixteen studies with 385 newborn infants were included and were significantly heterogeneous regarding treatment methods. Primary treatments included surfactant, high-frequency oscillatory ventilation (HFOV), epinephrine, coagulopathy management, intermittent positive pressure ventilation, cocaine and tolazoline. Adjunctive treatment methods included blood products, HFOV, increased positive end-expiratory pressure, vitamin K, surfactant, adrenaline, vasopressors and inotropes. All five studies using surfactant as primary treatment were effective in improving oxygenation index measures and preventing recurrence of PH, and three studies found no association between surfactant and death or long-term disability. Ventilatory support, epinephrine, management of coagulopathy and tolazoline were all found to be effective primary treatments for PH. CONCLUSION: There are several effective methods of managing PH in neonates. Further understanding of the aetiology of PH and ongoing research will allow future prevention and improvements in management of PH.

The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.

There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long-term outcome. We identified 781 patients, median age 12 years, with 4,277 person-years of follow-up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event-free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC-inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5-0.9, and 0.7, 95% confidence interval 0.5-0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long-term outcome. CONCLUSION: PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC-inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518-527).

Comparison of Two Techniques of Superior Vena Cava Flow Measurement in Preterm Infants With Birth Weight <1,250 g in the Transitional Period-Prospective Observational Cohort Study.

Objectives: Superior Vena Cava (SVC) flow in neonates measured by the standard approach has been validated by different groups around the world. The modified SVC flow measurement technique was recently suggested. The aim of our study was to evaluate standard and modified technique of echocardiography SVC flow measurement in a cohort of extremely preterm neonates in the immediate postnatal period. Methods: Prospective, observational cohort study in a level III neonatal center. Infants with birth weight <1,250 g were eligible for enrolment. SVC flow was measured by echocardiography using standard and modified methods at 6, 18 and 36 h of age. Our primary outcome was equivalency (using raw bounds of -20 to +20 mL/kg/min difference between the paired measurements), agreement and correlation between standard and modified methods of the SVC flow measurements. Results: Thirty-nine infants were enrolled. The mean gestational age of the cohort was 27.4 (SD 2.1) weeks of postmenstrual age, the mean birth weight was 0.95 kg (SD 0.2). The measurements at 6 and 36 h of age were equivalent as defined in the design of the study (p = 0.003 and p = 0.004 respectively; raw bounds -20 to +20 mL/kg/min). At 6 h of age the mean difference (bias) between the measurements was -0.8 mL/kg/min with 95% limits of agreement -65.0 to 63.4 mL/kg/min. At 18 h of age, the mean difference (bias) between the measurements was +9.5 mL/kg/min, with 95% limits of agreement -79.6 to 98.7 mL/kg/min. At 36 h of age the mean difference (bias) between the measurements was -2.2 mL/kg/min with 95% limits of agreement -73.4 to 69.1 mL/kg/min. There was a weak, but statistically significant correlation between the standard and modified method at 6 h of age (r = 0.39, p = 0.04). Conclusion: Both SVC flow echocardiography measurement techniques yielded clinically equivalent results, however due to wide limits of agreement and poor correlation they do not seem to be interchangeable.