Improving clinical outcomes of very low birth weight infants: Implementation of standardized management guidelines in tertiary care hospital in Haryana.

BACKGROUND: Standardized written guidelines and protocols in NICU are known to impact neonatal outcomes and improve survival. OBJECTIVE: To study and compare the morbidity and mortality outcomes of very low birth weight (VLBW) neonates before and after introduction of structured approach to standardized management guidelines on four interventions in a tertiary care hospital in North India. METHODOLOGY: Structured approach to standardized management guidelines on four interventions were implemented for VLBW infants in NICU. a) Humidified and Heated High Flow Nasal Cannula (HHHFNC) as the initial mode of ventilator support in preterm VLBW babies. b) Expressed breast milk for feeding preterm VLBW babies and absolutely no formula milk. c) Hand washing and following "Bundle Care Approach" for Central lines as the cardinal cornerstones for maintaining strict asepsis. d) Development and supportive care to be regularly followed. Data was collected prospectively from July 2015 to December 2016 (Intervention Group) and compared with retrospective matched controls from the previous year (July 2014-June 2015) (Control Group). RESULTS: There was a significant decrease in culture positive sepsis in the intervention group compared to control group (3 (2.97%) CI:0.006-0.08 vs 11 (19.64%) CI:0.10-0.32; P = .0004). There was no significant difference in the mortality (5.35% vs3.96% P = .74) amongst the two groups. CONCLUSION: Implementing structured approach to above mentioned interventions in the form of standardized management guidelines for preterm VLBW neonates was associated with significant reduction in culture proven sepsis and mechanical ventilation days without affecting mortality or other co-morbidities.

Pseudohypoparathyroidism with diabetes mellitus and hypothyroidism.

We report a 12-year-old child with pseudohypoparathyroidism (PHP) whose mother had pseudopseudohypoparathyroidism. The child had low serum calcium, high phosphorous and high parathormone (PTH) levels. PHP occurs due to a defect in the guanine nucleotide binding protein (G protein). She also had hypothyroidism which is known to utilize the G protein pathway. She developed T 1 diabetes mellitus (T1DM) while under follow-up. This is arguably the first time T1DM has been reported associated with PHP.

Cytohistological features of rhinoscleroma.

Rhinoscleroma, also known as Mikulicz disease, is a chronic progressive disease caused by Klebsiella rhinoscleromatis. The disease is uncommon in Delhi. There are no cytological references of this disease. We describe the cytological features which should alert the pathologist to rule out this entity, which needs to be treated early. Immunohistochemistry on histopathological material is a useful adjunct to the diagnosis.

Autoimmune lymphoproliferative syndrome: response to mycophenolate mofetil and pyrimethamine/sulfadoxine in a 5-year-old child.

Autoimmune Lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of disrupted lymphocyte homeostasis characterized by chronic splenomegaly and lymphadenopathy of early onset, hypergammaglobulinemia (Ig G and Ig A), autoimmune phenomena, and expanded populations of TCR-α/ß+, CD3+, CD4-, CD8-T cells (Fisher et al. Cell 81:935-946; 1995), called double negative T-cells [(DN) T cells]. We discuss a case of ALPS which showed good response to immunosuppressant drug Mycophenolate-Mofetil in combination with Pyrimethamine/Sulfadoxine.