Clinical features and outcomes from the Singapore Sjögren's syndrome study.

OBJECTIVE: To study the clinical features, treatment and outcomes of primary Sjögren's Syndrome (pSS) in a Singapore cohort from an outpatient rheumatology clinic. METHODS: Computerised Physician Order entry records of patients who fulfilled the 2016 ACR-EULAR classification criteria for pSS between 1993 and 2013 were retrospectively analysed. RESULTS: There were 102 patients, of which 96 (94.1%) were females, and 91 (89.2%) Chinese. Mean age at diagnosis was 49.3 ± 11.8 years, mean disease duration was 9.0 ± 4.6 years. The most common manifestations were keratoconjunctivitis sicca (99.0%), xerostomia (96.1%), arthralgia/arthritis (56.9%). Exocrine glandular enlargement comprised parotidomegaly (28, 27.5%), with concurrent submandibular and lacrimal gland enlargement in one. The nervous system (15.7%) was the most commonly affected internal organ, with peripheral nervous system (peripheral neuropathy, mononeuritis multiplex) involvement more common than central. Hydroxychloroquine was most frequently used (88.2%), followed by methotrexate (7.8%) and azathioprine (6.9%). Pulsed intravenous (IV) methylprednisolone 500 mg/day for 3 days was used in 5 patients followed by oral (4) or IV cyclophosphamide (1) for cardiomyopathy and interstitial lung disease (1), and neurological involvement (4). These comprised neuromyelitis optica, transverse myelopathy, cranial neuropathy, mononeuritis multiplex and/or peripheral neuropathy alone or in combination. Intravenous immunoglobulins (2.0%) was used for sensory neuropathy and mononeuritis multiplex; rituximab (1.0%) in 1 patient for treatment of non-Hodgkin's B-cell lymphoma. There were no deaths. CONCLUSION: Musculoskeletal manifestations were common, with the nervous system (peripheral more than central) the most common internal organ involved. Lymphoma was uncommon despite up to one-third of the cohort developing glandular enlargement.

Creation and validation of the Singapore birth nomograms for birth weight, length and head circumference based on a 12-year birth cohort.

INTRODUCTION: Both gestation and birth weight have significant impact on mortality and morbidity in newborn infants. Nomograms at birth allow classification of infants into small for gestational age (SGA) and large for gestational age (LGA) categories, for risk stratification and more intensive monitoring. To date, the growth charts for preterm newborn infants in Singapore are based on the Fenton growth charts, which are constructed based on combining data from various Western growth cohorts. Hence, we aim to create Singapore nomograms for birth weight, length and head circumference at birth, which would reflect the norms and challenges faced by local infants. MATERIALS AND METHODS: Growth parameters of all babies born or admitted to our unit from 2001 to 2012 were retrieved. Following exclusion of outliers, nomograms for 3 percentiles of 10th, 50th, and 90th were generated for the gestational age (GA) ranges of 25 to 42 weeks using quantile regression (QR) combined with the use of restricted cubic splines. Various polynomial models (second to third degrees) were investigated for suitability of fit. The optimum QR model was found to be a third degree polynomial with a single knotted cubic spline in the mid-point of the GA range, at 33.5 weeks. Check for goodness of fit was done by visual inspection first. Next, check was performed to ensure the correct proportion: 10% of all cases fall above the upper 90th percentile and 10% fall below the lower 10th percentile. Furthermore, an alternative formula-based method of nomogram construction, using mean, standard deviation (SD) and assumption of normality at each gestational age, was used for counterchecking. RESULTS: A total of 13,403 newborns were included in the analysis. The new infant-foetal growth charts with respect to birth weight, heel-crown length and occipitofrontal circumference from 25 to 42 weeks gestations with the 10th, 50th and 90th were presented. CONCLUSION: Nomograms for birth weight, length and head circumference at birth had significant impact on neonatal practice and validation of the Singapore birth nomograms against Fenton growth charts showed better sensitivity and comparable specificity, positive and negative predictive values.