Impact of Physician Characteristics on Late-Onset Sepsis (LOS) Evaluation in the NICU.

The threshold for a late-onset sepsis (LOS) evaluation varies considerably across NICUs. This unexplained variability is probably related in part to physician bias regarding when sepsis should be "ruled out". The aim of this study is to determine if physician characteristics (race, gender, immigration status, years of experience and academic rank) effect LOS evaluation in the NICU. This study includes a retrospective chart review of all Level III NICU infants who had a LOS evaluation over 54 months. Physician characteristics were compared between positive and negative blood culture groups and whether CBC and CRP were obtained at LOS evaluations. There were 341 LOS evaluations performed during the study period. Two patients were excluded due to a contaminant. Patients in this study had a birth weight of [median (Q1, Q3)]+ 992 (720, 1820) grams and birth gestation of [median (Q1, Q3)] 276/7 (252/7, 330/7) weeks. There are 10 neonatologists in the group, 5/10 being female and 6/10 being immigrant physicians. Experienced physicians were more likely to obtain a CBC at the time of LOS evaluation. Physician characteristics of race, gender and immigration status impacted whether to include a CRP as part of a LOS evaluation but otherwise did not influence LOS evaluation, including the likelihood of bacteremia.

Congenital hyperinsulinism and panhypopituitarism: a rare combination.

Summary: Neonatal hypoglycemia is a serious condition that can have a major impact on the growing neonatal brain. The differential diagnosis of neonatal hypoglycemia is broad and includes hyperinsulinism as well as panhypopituitarism. The FOXA2 gene has been involved in the development of the pancreas as well as the pituitary gland. Six cases have been reported thus far with FOXA2 mutations presenting with variable degrees of hypopituitarism, and only two patients had permanent hyperinsulinism; other cases have been reported with microdeletions in 20p11, the location that encompasses FOXA2, and those patients presented with a wider phenotype. A full-term female infant presented with severe hypoglycemia. Critical sampling showed an insulin of 1 mIU/mL, suppressed beta-hydroxybutyric acids, and suppressed free fatty acids. Blood glucose responded to glucagon administration. Growth hormone (GH) stimulation test later showed undetectable GH in all samples, and cortisol failed to respond appropriately to stimulation. Gonadotropins were undetectable at 1 month of life, and MRI showed ectopic posterior pituitary, interrupted stalk, hypoplastic anterior pituitary, cavum septum pellucidum, and diminutive appearance of optic nerves. Whole-exome sequencing revealed a likely pathogenic de novo c.604 T>C, p.Tyr202His FOXA2 mutation. We expand the known phenotype on FOXA2 mutations and report a likely pathogenic, novel mutation associated with hyperinsulinism and panhypopituitarism. Learning points: FOXA2 has been shown to play an important role in the neuroectodermal and endodermal development. FOXA2 mutation may lead to the rare combination of hyperinsulinism and panhypopituitarism. Patients reported so far all responded well to diazoxide. Dysmorphology may be subtle, and liver functions should be monitored.

Fresh frozen plasma and recombinant factor VIIa use in neonates.

BACKGROUND: Little recent data are available describing fresh frozen plasma (FFP) use in neonates. The purpose of this study was to determine the outcomes of FFP transfusions in neonates. PATIENTS AND METHODS: A single institution, observational, and retrospective review of each transfusion of FFP given to neonates admitted to a neonatal intensive care unit over a 2-year period. RESULTS: One hundred and seventy-three neonates were identified as having received FFP, giving a prevalence of FFP use at 12%. By far the most common determining factor for FFP use was an association with an abnormal activated partial thromboplastin time or prothrombin time (52%). Other factors included bleeding, invasive procedures, volume expansion, necrotizing enterocolitis, cardiopulmonary bypass, and hydrops fetalis. Of objectively accessible responses, FFP was able to correct abnormal coagulation tests into the normal range only 40% of the time. Twenty-four neonates received recombinant factor VIIa (rFVIIa) after first receiving FFP. The prevalence of thrombotic events was not higher in neonates receiving rFVIIa than those receiving FFP alone. CONCLUSIONS: FFP was widely used in this neonatal unit. As data showing the predictive value of coagulation tests in neonates are discrepant, it is unclear if FFP was being appropriately used. Prospective, controlled data are required.

Thrombotic events in neonates receiving recombinant factor VIIa or fresh frozen plasma.

BACKGROUND: Numerous recent reports have described the use of recombinant factor VIIa (rFVIIa) in non-hemophilia bleeding situations for achievement of hemostasis. However, its use in clinical situations other than hemophilia patients with inhibitors has been complicated by some reports of thrombotic events. rFVIIa has been used successfully to treat coagulopathic and/or bleeding neonates. The prevalence of thrombotic events in these neonates is completely unknown. This study was initiated to determine the risk of thrombotic events associated with rFVIIa use in neonates. PROCEDURE: All published literature in non-hemophilic, non-congenital factor VII deficient neonates receiving rFVIIa was reviewed. In addition, all data submitted to the SeveN Bleep Registry, a web-based registry of rFVIIa uses in non-hemophilic children was analyzed. As the baseline risk of thrombotic events in bleeding and/or coagulopathic neonates is not known, we also reviewed the records of 100 consecutive neonates from a single institution who received fresh frozen plasma (FFP) alone to treat their coagulopathy and/or bleeding episode. RESULTS: A total of 134 neonates received rFVIIa. Of these, 10 (7.5%) had a thrombotic event. The baseline risk of thrombotic events in neonates receiving FFP was 7%. CONCLUSIONS: Overall the prevalence of thrombotic events in bleeding and/or coagulopathic neonates appears to be around 7%, whether or not they receive rFVIIa.