An Observational, Prospective, Multicenter, Registry-Based Cohort Study Comparing Conservative and Medical Management for Patent Ductus Arteriosus.

No consensus has been reached on which patent ductus arteriosus (PDAs) in preterm infants require treatment and if so, how, and when they should be treated. A prospective, multicenter, cohort study was conducted to compare the effects of conservative approaches and medical treatment options on ductal closure at discharge, surgical ligation, prematurity-related morbidities, and mortality. Infants between 240/7 and 286/7 weeks of gestation from 24 neonatal intensive care units were enrolled. Data on PDA management and patients' clinical characteristics were recorded prospectively. Patients with moderate-to-large PDA were compared. Among the 1,193 enrolled infants (26.7 ± 1.4 weeks and 926 ± 243 g), 649 (54%) had no or small PDA, whereas 544 (46%) had moderate-to-large PDA. One hundred thirty (24%) infants with moderate-to-large PDA were managed conservatively, in contrast to 414 (76%) who received medical treatment. Eighty (62%) of 130 infants who were managed conservatively did not receive any rescue treatment and the PDA closure rate was 53% at discharge. There were no differences in the rates of late-onset sepsis, necrotizing enterocolitis (NEC), retinopathy of prematurity, intraventricular hemorrhage (≥Grade 3), surgical ligation, and presence of PDA at discharge between conservatively-managed and medically-treated infants (p > 0.05). Multivariate analysis including perinatal factors showed that medical treatment was associated with increased risk for mortality (OR 1.68, 95% Cl 1.01-2.80, p = 0.046), but decreased risk for BPD or death (BPD/death) (OR 0.59, 95%Cl 0.37-0.92, p = 0.022). The preferred treatment options were ibuprofen (intravenous 36%, oral 31%), and paracetamol (intravenous 26%, oral 7%). Infants who were treated with oral paracetamol had higher rates of NEC and mortality in comparison to other treatment options. Infants treated before postnatal day 7 had higher rates of mortality and BPD/death than infants who were conservatively managed or treated beyond day 7 (p = 0.009 and 0.007, respectively). In preterm infants born at <29 weeks of gestation with moderate-to-large PDA, medical treatment did not show any reduction in the rates of open PDA at discharge, surgical or prematurity-related secondary outcomes. In addition to the high incidence of spontaneous closure of PDA in the first week of life, early treatment (<7 days) was associated with higher rates of mortality and BPD/death.

An observational, prospective, multicenter study on rescue high-frequency oscillatory ventilation in neonates failing with conventional ventilation.

BACKGROUND: To achieve gas exchange goals and mitigate lung injury, infants who fail with conventional ventilation (CV) are generally switched to high-frequency oscillatory ventilation (HFOV). Although preferred in many neonatal intensive care units (NICUs), research on this type of rescue HFOV has not been reported recently. METHODS: An online registry database for a multicenter, prospective study was set to evaluate factors affecting the response of newborn infants to rescue HFOV treatment. The study population consisted of 372 infants with CV failure after at least 4 hours of treatment in 23 participating NICUs. Patients were grouped according to their final outcome as survived (Group S) or as died or received extracorporeal membrane oxygenation (ECMO) (Group D/E). Patients' demographic characteristics and underlying diseases in addition to their ventilator settings, arterial blood gas (ABG) analysis results at 0, 1, 4, and 24 hours, type of device, ventilation duration, and complications were compared between groups. RESULTS: HFOV as rescue treatment was successful in 58.1% of patients. Demographic and treatment parameters were not different between groups, except that infants in Group D/E had lower birthweight (BW) (1655 ± 1091 vs. 1858 ± 1027 g, p = 0.006), a higher initial FiO2 setting (83% vs. 72%, p < 0.001), and a higher rate of nitric oxide exposure (21.8% vs. 11.1%, p = 0.004) in comparison to infants who survived (Group S). The initial cut-offs for a successful response on ABG were defined as pH >7.065 (OR: 19.74, 95% CI 4.83-80.6, p < 0.001), HCO3 >16.35 mmol/L (OR: 1.06, 95% CI 1.01-1.1, p = 0.006), and lactate level <3.75 mmol/L (OR: 1.09%95 CI 1.01-1.16, p = 0.006). Rescue HFOV duration was associated with retinopathy of prematurity (p = 0.005) and moderate or severe chronic lung disease (p < 0.001), but not with patent ductus arteriosus or intraventricular hemorrhage, in survivors (p > 0.05). CONCLUSION: Rescue HFOV as defined for this population was successful in more than half of the patients with CV failure. Although the response was not associated with gestational age, underlying disease, device used, or initial MV settings, it seemed to be more effective in patients with higher BW and those not requiring nitric oxide. Initial pH, HCO3, and lactate levels on ABG may be used as predictors of a response to rescue HFOV.

Maternal serum concentrations of s-Endoglin and IL-6 in pregnancy complicated by preterm premature membrane rupture.

OBJECTIVE: This study aimed to investigate maternal serum concentrations of s-Endoglin and compare s-Endoglin with other inflammatory markers in prediction of time to delivery, in pregnancies complicated by preterm premature rupture of membranes (PPROM). MATERIALS AND METHODS: Fifty five patients complicated by PPROM whose gestational age were between 2433 weeks and 44 matched healthy pregnant women were included in present study. Maternal concentrations of s-Endoglin concentrations were measured by an enzyme-linked immunosorbent assay (ELISA) and compared with maternal inflammatory markers including interleukin-6 (IL-6), white blood cell (WBC) count and serum C-reactive protein (CRP). The best variable for prediction of preterm birth was computed. RESULTS: Mean s-Endoglin levels in PPROM were lower than control groups (0.24 ± 0.12 pg/ml and 0.69 ± 0.25 pg/ml, respectively, p < 0.01). Besides IL-6 (p < 0.01), WBC (p = 0.016) and CRP (p = 0.010) levels were higher in PPROM group. In PPROM group, ROC analysis results of s-Endoglin for prediction of preterm delivery <48 h, <7 days, <32 weeks were not different (p > 0.05). For predicting preterm birth before 48 h and 7 days, only IL-6 at cut off value >0.70 (pg/ml) and >0.55 (pg/ml) had area under curve (AUC); 0.871 (0.7750.965), p < 0.01, AUC; 0.925 (0.8560.993), p < 0.001, respectively. CONCLUSION: s-Endoglin as an anti-angiogenic marker seemed to have a role in pathogenesis but results of present study showed that, unlike IL-6, it was unsatisfactory for estimating time to delivery in PPROM.

Hepatitis A super infection as a cause of liver failure in a child with Wilson's disease.

Infection with hepatitis A virus can cause severe or even fatal illness in patients with chronic liver disease. Here we describe a seven-year-old girl who presented as acute liver failure and was diagnosed with Wilson's disease and later with coexistent hepatitis A infection. Wilson's disease was demonstrated on the basis of low ceruloplasmin, high urinary copper excretion, histological evidence of cirrhosis, and high biochemical estimation of liver copper concentration. Hepatitis A was diagnosed serologically. Our case suggests that acute hepatitis A may play a part in the acute decompensation seen in some cases of unrecognized Wilson's disease. We also emphasize the importance of prevention measures of hepatitis A infection in patients with chronic liver disease.

Familial syndrome of unusual triangular facies associated with cleft palate, malocclusion, midfacial hypoplasia and sensorineural hearing loss in two siblings. Is it a new autosomal recessive syndrome?

We present a previously undescribed syndrome characterized by triangular facial appearance, mid-facial hypoplasia, cleft palate and mild sensorineural hearing loss in two siblings. The parents were unrelated. The patients' stature and intelligence were normal. We suggest that the inheritance is autosomal recessive.