METHODS: A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed.
RESULTS: A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6-9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0-12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%.
CONCLUSION: Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.
BACKGROUND: Roux-en-Y gastric bypass (RYGB) and sleeve gastrectomy (SG) account for >95% of bariatric procedures in United States in patients with T2DM. To date, there is no validated model to guide procedure selection based on long-term glucose control in patients with T2DM.
METHODS: A total of 659 patients with T2DM who underwent RYGB and SG at an academic center in the United States and had a minimum 5-year follow-up (2005-2011) were analyzed to generate the model. The validation dataset consisted of 241 patients from an academic center in Spain where similar criteria were applied.
RESULTS: At median postoperative follow-up of 7 years (range 5-12), diabetes remission (HbA1C <6.5% off medications) was observed in 49% after RYGB and 28% after SG (P < 0.001). Four independent predictors of long-term remission including preoperative duration of T2DM (P < 0.0001), preoperative number of diabetes medications (P < 0.0001), insulin use (P = 0.002), and glycemic control (HbA1C < 7%) (P = 0.002) were used to develop the Individualized Metabolic Surgery (IMS) score using a nomogram. Patients were then categorized into 3 stages of diabetes severity. In mild T2DM (IMS score ≤25), both procedures significantly improved T2DM. In severe T2DM (IMS score >95), when clinical features suggest limited functional β-cell reserve, both procedures had similarly low efficacy for diabetes remission. There was an intermediate group, however, in which RYGB was significantly more effective than SG, likely related to its more pronounced neurohormonal effects. Findings were externally validated and procedure recommendations for each severity stage were provided.
CONCLUSIONS: This is the largest reported cohort (n = 900) with long-term postoperative glycemic follow-up, which, for the first time, categorizes T2DM into 3 validated severity stages for evidence-based procedure selection.
METHODS: This is a retrospective review of consecutive patients who underwent argon plasma coagulation for haemorrhagic radiation proctitis between January 2003 and December 2013. The patients were followed up using a prospectively maintained database.
RESULTS: Ninety-one patients were included with a mean follow-up of 13.1 months. Majoity (n = 85, 93.4 %) of the patients were female. Mean age at the time of treatment was 58.2 (range 23-87) years old. Majority of the patients (n = 73, 80.2 %) received radiotherapy for gynaecological malignancies followed by colorectal (n = 13, 14.3 %) and urological (n = 5, 5.5 %) malignancies. Mean interval between radiation and proctitis was 13.8 (range 3-40) months. Seventy-nine percent of patients were successfully treated after 1-2 sessions. Seventeen (18.7 %) patients experienced self-limiting early complications, and three (3.3 %) had late complications of rectal stenosis which was managed conservatively. Severity of bleeding during the initial presentation is an independent factor that predicts the number of sessions required for successful haemostasis (p = 0.002).
CONCLUSIONS: Argon plasma coagulation is a reasonable treatment option in patients with haemorrhagic radiation proctitis with good safety profile. Our study suggests that the number of APC sessions required to arrest bleeding correlates with the severity of bleeding on initial presentation.
OBJECTIVES: The aim of this study was to look at our intermediate outcomes after LDJB-SG.
SETTING: An academic medical center.
METHODS: A prospective analysis of T2D patients who underwent LDJB-SG between October 2011 and October 2014 was performed. Data collected included baseline demographic, body mass index, fasting blood glucose, glycosylated hemoglobin, C-peptide, resolution of co-morbidities, and postoperative complications.
RESULTS: A total of 163 patients with minimum of follow-up >1 year were enrolled in this study (57 men and 106 women). The mean age and body mass index were 47.7 (±10.7) years and a 30.2 (±5.1) kg/m2, respectively. There were 119 patients on oral hypoglycemic agents only, 29 patients were on oral hypoglycemic agents and insulin, 3 patients were on insulin only, and the other 12 patients were not on diabetic medication. Mean operation time and length of hospital stay were 144.7 (± 45.1) minutes and 2.4 (± 1.0) days, respectively. Seven patients (3.6%) needed reoperation due to bleeding (n = 1), anastomotic leak (n = 2), sleeve strictures (n = 2), and incisional hernia (n = 2). At 2 years of follow-up, there were 56 patients. None of the patients were on insulin and only 20% of patients were on oral hypoglycemic agents. Mean body mass index significantly dropped to 22.9 (±5.6) kg/m2 at 2 years. The mean preoperative fasting blood glucose, glycosylated hemoglobin, and C-peptide levels were 174.7 mg/dL (± 61.0), 8.8% (±1.8), and 2.6 (±1.7) ng/mL, respectively. The mean fasting blood glucose, glycosylated hemoglobin, and C-peptide at 2 years were 112.5 (±60.7) mg/dL, 6.4% (±2.0), and 1.5 (±0.6) ng/mL, respectively. No patient needed revisional surgery because of dumping syndrome, marginal ulcer, or gastroesophageal reflux disease at the last follow up period.
CONCLUSION: At 2 years, LDJB-SG is a relatively safe and effective metabolic surgery with significant weight loss and resolution of co-morbidities.
METHODS: We herein report, to our knowledge, the first observation on the frequency and nature of GATA1 gene mutations in a cohort of Malaysian children with DS-associated TAM (n = 9) and ML-DS (n = 24) encountered successively over a period of five years at a national referral centre.
RESULTS: Of the 29 patients who underwent GATA1 analysis, GATA1 mutations were observed in 15 (51.7%) patients, including 6 (75.0%) out of 8 patients with TAM, and 9 (42.9%) of 21 patients with ML-DS. All identified mutations were located in exon 2 and the majority were sequence-terminating insertions or deletions (66.7%), including several hitherto unreported mutations (12 out of 15).
CONCLUSION: The low frequency of GATA1 mutations in ML-DS patients is unusual and potentially indicates distinctive genomic events in our patient cohort.
METHOD: A meta-analysis was performed on data from three genome-wide pharmacogenetic studies (the Genome-Based Therapeutic Drugs for Depression [GENDEP] project, the Munich Antidepressant Response Signature [MARS] project, and the Sequenced Treatment Alternatives to Relieve Depression [STAR*D] study), which included 2,256 individuals of Northern European descent with major depressive disorder, and antidepressant treatment outcomes were prospectively collected. After imputation, 1.2 million single-nucleotide polymorphisms were tested, capturing common variation for association with symptomatic improvement and remission after up to 12 weeks of antidepressant treatment.
RESULTS: No individual association met a genome-wide threshold for statistical significance in the primary analyses. A polygenic score derived from a meta-analysis of GENDEP and MARS participants accounted for up to approximately 1.2% of the variance in outcomes in STAR*D, suggesting a weakly concordant signal distributed over many polymorphisms. An analysis restricted to 1,354 individuals treated with citalopram (STAR*D) or escitalopram (GENDEP) identified an intergenic region on chromosome 5 associated with early improvement after 2 weeks of treatment.
CONCLUSIONS: Despite increased statistical power accorded by meta-analysis, the authors identified no reliable predictors of antidepressant treatment outcome, although they did identify modest, direct evidence that common genetic variation contributes to individual differences in antidepressant response.
AIM AND OBJECTIVES: The aim of this study was to evaluate the clinical significance of serum CD26 in patients with acute lymphoblastic leukaemia patients in the post remission induction phase, as well as the relationship between CD26 activity and the oxidative stress status.
MATERIALS AND METHODS: CD26, total antioxidant status (TAS), total oxidant status (TOS), and oxidative stress index (OSI), in addition to activity of related enzymes myeloperoxidase, glutathione- s-transferase and xanthine oxidase, were analysed in sixty children with acute lymphoblastic leukaemia in the post remission induction phase.
RESULTS: The study showed significant elevation in CD26, TOS and OSI levels in patients with acute lymphoblastic leukaemia in the post remission induction phase in comparison to healthy control samples. In contrast, myeloperoxidase, glutathione-s-transferase and xanthine oxidase activities were decreased significantly. A significant correlation between CD26 concentration and some oxidative stress parameters was evident in ALL patients.
CONCLUSIONS: Serum levels of CD26 appear to be useful as a new biomarker of oxidative stress in children with acute lymphoblastic leukaemia in the post remission induction phase, and levels of antioxidants must be regularly estimated during the treatment of children with ALL.
OBJECTIVE: To investigate prevalence and predictors of PREM defined by IDAA1c.
METHODS: Six hundred fourteen of 678 children (aged <15 years) with new-onset T1D (2000-2013) from a regional pediatric diabetes service (Auckland, New Zealand).
RESULTS: Overall rate of PREM at 3 months was 42.4%, and lower in Māori/Pacific children (28.6%; P = .006) and those of other ethnicities (28.8%; P = .030) compared with New Zealand Europeans (50.4%). Comparing the most and least deprived socioeconomic quintiles, the odds of PREM were lower among the most deprived (adjusted odds ratio [aOR] 0.44; P = .019). Lower rates of PREM were seen in children aged 0 to 4.9 years (23.8%) and 10 to 14 years (40.9%) than in children aged 5 to 9.9 years (57.4%; P