OBJECTIVE: This study aimed to investigate the effectiveness and safety of sulfonylurea therapy in Chinese NDM patients during infancy before genetic testing results were available.
METHODS: The medical records of NDM patients with their follow-up details were reviewed and molecular genetic analysis was performed. Sulfonylurea transfer regimens were applied in patients diagnosed after May 2010, and glycemic status and side effects were evaluated in each patient.
RESULTS: There were 23 NDM patients from 22 unrelated families, 10 had KCNJ11 mutations, 3 harbored ABCC8 mutations, 1 had INS mutations, 4 had chromosome 6q24 abnormalities, 1 had a deletion at chromosome 1p36.23p36.12, and 4 had no genetic abnormality identified. Sixteen NDM infants were treated with glyburide at an average age of 49 days (range 14-120 days) before genetic confirmation. A total of 11 of 16 (69%) were able to successfully switch to glyburide with a more stable glucose profile. The responsive glyburide dose was 0.51 ± 0.16 mg/kg/d (0.3-0.8 mg/kg/d), while the maintenance dose was 0.30 ± 0.07 mg/kg/d (0.2-0.4 mg/kg/d). No serious adverse events were reported.
CONCLUSIONS: Molecular genetic diagnosis is recommended in all patients with NDM. However, if genetic testing results are delayed, sulfonylurea therapy should be considered before such results are received, even in infants with newly diagnosed NDM.
SUBJECTS AND METHODS: The package known as "Preemie Mom: A Guide for You" was designed based on Stufflebeam's model and has four phases: (1) content evaluation from available sources of information, (2) input evaluation based on mothers' need related to premature baby care, (3) process evaluation for package designing and content drafting, and (4) product evaluation to determine its feasibility. The contents were extracted and collated for validation by consulting various specialists in related fields. A final draft was drawn based on comments given by experts. Comments from the mothers were taken for formatting, visual appearance, and content flow for easy understanding and usage.
RESULTS: All ten existing articles and eight relevant documents were gathered and critically appraised. The package was designed based on 11 main components related to the care of premature baby after discharge. The content validation was accepted at a minimum score of 0.85 for the item-level content validity index analysis. Both experts and mothers were agreed that the package is easy to use and well accepted as a guide after discharge. The agreement rate by the mothers was at 93.33% and greater for the front page, writing style, structure, presentation, and motives of the package.
CONCLUSIONS: "Preemie Mom: A Guide for You" is a validated health educational package and ready to be used to meet the needs of the mother for premature baby care at home.
METHODS: Retrospective review of all neonates with clinical and radiological evidence of non-perforated NEC that were treated in a tertiary-level referral hospital between 2009 and 2018. General patient demographics, laboratory parameters and outcomes were recorded. Receiver operating characteristics analysis was performed to evaluated optimal cut-offs and area under the curve (AUC) with 95% confidence intervals (CI).
RESULTS: A total of 191 neonates were identified. Of these, 103 (53.9%) were born at ≤ 28 weeks of gestation and 101 (52.9%) had a birth weight of ≤ 1000 g. Eighty-four (44.0%) patients underwent surgical intervention for NEC. The overall survival rate was 161/191 (84.3%). A CRP/ALB ratio of ≥ 3 on day 2 of NEC diagnosis was associated with a statistically significant higher likelihood for surgery [AUC 0.71 (95% CI 0.63-0.79); p