AIMS: To describe incidence, presentation, treatment and short term outcomes of severe neonatal hypernatraemia (SNH, sodium >/=160 mmol/l). METHODS: Prospective, population based surveillance study over 13 months using the British Paediatric Surveillance Unit. Cases were >33 weeks gestation at birth, fed breast or formula milk and <28 days of age at presentation. RESULTS: Of 62 cases of SNH reported (7, 95% CI 5.4 to 9.0 per 1 00 000 live births), 61 mothers had intended to achieve exclusive breast feeding. Infants presented at median day 6 (range 2-17) with median weight loss of 19.5% (range 8.9-30.9). 12 had jaundice and 57 weight loss as a presenting feature. 58 presented with weight loss >/=15%. 25 babies had not stooled in the 24 h prior to admission. Serum sodium fell by median 12.9 mmol/l per 24 h (range 0-30). No baby died, had seizures or coma or was treated with dialysis or a central line. At discharge, babies had regained 11% of initial birth weight after a median admission of 5 (range 2-14) days. 10 were exclusively breast fed on discharge from hospital. CONCLUSIONS: Neonatal hypernatraemia at this level, in this population, is strongly associated with weight loss. It occurs almost exclusively after attempts to initiate breast feeding, occurs uncommonly and does not appear to be associated with serious short term morbidities, beyond admission to hospital

The birth of a premature infant with haemophilia is associated with a risk of intracranial haemorrhage both on the basis of its inherited haemorrhagic diathesis and prematurity [1]. A literature review has indicated six other cases of haemophilic infants born prematurely [2][3][4]. In this report we describe the management of an infant with severe haemophilia A born at 25 weeks gestation who received prophylactic administration of recombinant factor VIII (rFVIII). An inhibitor to factor VIII (FVIII) was detected on day 23 of life; we believe this to be the first premature infant in which inhibitor development has been described.A 20 year old obligate carrier for severe haemophilia A presented at 25 weeks gestation. She had two brothers with severe haemophilia A, with an underlying FVIII intron-22 inversion, neither of whom had developed inhibitors. A healthy male infant was born by normal vaginal delivery weighing 780 g. Umbilical cord blood investigation revealed a diagnosis of severe haemophilia A, FVIII < 1 IU dL )1 (50-213), APTT 124 s (27.5-79.4), PT 16.9 s (10.6-16.2) [5]. rFVIII (ADVATE Ò Baxter Bioscience, Thousand Oaks, California, USA) was started at a dose of 50 IU (64 IU kg )1 ) twice daily. A FVIII intron22 inversion was identified.A FVIII inhibitor was first detected on day 23 of life (2.1 Bethesda units, BU), by which point, a total of 45 doses of FVIII had been administered. A course of dexamethasone (50 mcg kg )1 day )1 ) was commenced 6 days prior to inhibitor development to aid extubation from the ventilator. A higher dexamethasone (100 mcg kg )1 day )1 ) dose was required and this coincided with a peak inhibitor titre (6.2 BU).Increased FVIII doses were administered (peak 250 IU twice daily) and the Kasper inhibitor screen became consistently negative 69 days later. Frequent factor assays but no formal FVIII half life studies were performed due to the large quantities of blood that would be needed and due to the prematurity of the infant.Shortly after birth a bruise was noted at the vertex of the head which did not extend. A cranial ultrasound was performed on the day of birth which revealed a small, insignificant right sided intraventricular haemorrhage (IVH) which resolved after 24 h. On day 24 of life, a cranial ultrasound revealed irregularities of the choroid plexi suggesting small bilateral IVH. These were not deemed clinically significant and given safe FVIII levels before and after FVIII infusion no additional haemostatic agent was used. There were no clinically evident pulmonary haemorrhages noted during the admission.Intravenous access was provided initially using umbilical artery and vein catheters. At 8 weeks of age a Broviac line was inserted using FVIII continuous infusion (500 IU bolus followed by 18.5 IU h )1 for 3 days) which resulted in continuous haemostatic levels of FVIII. This central line extravasated after 4 weeks and a second Broviac line was inserted using FVIII bolus administration (500 IU pre and 500 IU post insertion). There were no bleeding complications associate...

Aims To describe incidence, presentation and short term outcomes of severe neonatal hypernatraemia (SNH) (≥160 mmol/l). Methods Prospective, population based surveillance study over 13 months using the BPSU. Cases were >33 weeks gestation at birth, fed by any means and <28 days of age at presentation. Results 62 cases of SNH were reported (66 per 100 000 livebirths; 95%CI 5.1 to 8.5). 61/62 mothers intended to achieve exclusive breastfeeding. 42 had no supplementary formula prior to admission. Infants presented at median day 6 (range 2–17) with median weight loss of 19.5% (range 8.9–30.9). 13 had jaundice and 57 weight loss as a presenting feature. 58 presented with weight loss ≥15%. Serum sodium fell by median 12.9 mmol/l per 24 h (range 0–30). No baby died, had convulsions, coma or was treated with dialysis or a central line. At discharge, babies had gained 11% of birthweight after a medium admission of 4 (range 2–14) days. 10/62 were exclusively breastfed on discharge from hospital. Conclusions Neonatal hypernatraemia at this level is strongly associated with weight loss. It occurs almost exclusively after attempts to initiate breastfeeding, occurs rarely and is not associated with serious short term morbidities. The reported incidence is higher than that in the Netherlands.1