https://ogma.newcastle.edu.au/vital/access/ /manager/Index ${session.getAttribute("locale")} 5 https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:31519 F508del) or NBS-positive but discharged following sweat chloride < 60 mmol/L. Cases of LD-CF were each matched 1:2 with patients with NBS-CF for age, sex, hospital, and exocrine pancreatic status. Results: A total of 45 LD-CF cases were identified (39 NBS-negative and 6 NBS-positive) with 90 NBS-CF matched controls. Median age (IQR) of diagnosis for LD-CF and NBS-CF was 1.35 (0.4-2.8) and 0.12 (0.03-0.2) years, respectively (P <.0001). Estimated incidence of LD-CF was 1 in 45 000 live births. Compared with NBS-CF, LD-CF had more respiratory manifestations at time of diagnosis (66% vs 4%; P <.0001), a higher rate of hospital admission per year for respiratory illness (0.49 vs 0.2; P = .0004), worse lung function (forced expiratory volume in 1 second percentage of predicted, 0.88 vs 0.97; P = .007), and higher rates of chronic colonization with Pseudomonas aeruginosa (47% vs 24%; P = .01). The LD-CF cohort also appeared to be shorter than NBS-CF controls (mean height z-score −0.65 vs −0.03; P = .02). Conclusions: LD-CF, despite NBS, seems to be associated with worse health before diagnosis and worse later growth and respiratory outcomes, thus providing further support for NBS programs for CF.]]> Wed 24 Nov 2021 15:52:38 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:37433 Wed 24 Nov 2021 15:50:40 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:37724 Wed 19 Apr 2023 13:53:21 AEST ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:29219 Thu 28 Oct 2021 12:35:47 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:20760 Sat 24 Mar 2018 08:00:26 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:28742 Sat 24 Mar 2018 07:37:37 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:25615 Sat 24 Mar 2018 07:28:13 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:31059 45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P=0.02) at 3 months post-HPE, and center (P=0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB < 74 µmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level > 35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB =74 µmol/L, albumin > 35 g/L; 2: TB =74 µmol/L, albumin =35 g/L; 3: TB > 74 µmol/L) with distinct short-and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs-0.30, P=0.0217) with similar rates of coagulopathy. Conclusions: Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level < 35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.]]> Sat 24 Mar 2018 07:25:47 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:27790 Sat 24 Mar 2018 07:23:23 AEDT ]]>