Portal vein thrombosis, livedo reticularis, polymicrobial sepsis and high antiphospholipid antibody titers in a newborn: A clinicopathological conference of antiphospholipid-associated neonatal syndrome.

Neonatal Antiphospholipid syndrome (APS) is a rare disease related to transplacental passage of antiphospholipid (aPL) antibodies from the mother or de novo production of aPL in a newborn. Neonatal aPL antibodies have rarely been associated with thrombosis. We describe a 5-week-old infant who developed fever, portal vein thrombosis and livedo reticularis like skin rash. Evaluation for thrombosis revealed high titers of antiphospholipid (aPL) antibodies (dual positive) in the child without any evidence of aPL antibodies in the mother, suggesting a de novo production in the child. Autopsy findings revealed umbilical vein sepsis with thrombosis of portal vein secondary to gram positive cocci which led to multiple liver and lung abscesses. Additionally, the baby had disseminated Cytomegalovirus (CMV) disease (acquired postnatally) involving walls of umbilical and portal vein, liver, lungs, adrenals, pancreas, thymus, and kidneys. Our case highlights the need for testing of aPL in every neonate with arterial or venous thrombosis even when the mother may have no features suggestive of an autoimmune disease.

Referrals to a pediatric emergency department of a tertiary care teaching hospital before and after introduction of a referral education module - a quality improvement study.

BACKGROUND: Provision of timely care to critically ill children is essential for good outcome. Referral from smaller peripheral hospitals to higher centers for intensive care is common. However, lack of an organized referral and feedback system compromises optimal care. We studied the quality of referral letters coming to our Emergency Department (ED) with respect to their demography, association with severity of illness and mortality before and after referral education. METHODS: Our study was completed in three phases in the Pediatric ED; Pre-intervention, Intervention and Post intervention phases. Quality of referral letter was matched with a quality checklist proforma and graded as 'good', 'fair' and 'poor' if it scored > 7, 5-7 and < 5 points respectively. A peer reviewed referral education module was prepared using case studies, expert opinions, and lacunae observed in the first phase and administered to health care providers (HCP's) of referring hospitals. Quality of referral letter was compared between pre and post intervention phases. RESULTS: Most referrals belonged to the neighboring states of Punjab (48.2%) and Haryana (22.4%). Major referring hospitals were from public sector (80.9%), of which the teaching hospitals topped the list (53.6%). Government run ambulance services (85.5%) was commonest mode of transport used and need for a PICU bed and/or mechanical ventilation (50.4%) was the commonest reason for referral. The post intervention phase saw a significant decline in the proportion of poor (93.2 vs.78.2%; p = 0.001) and a significant increase in the proportion of fair (6.1 vs 18%; p = 0.001) and good referral letters (0.7 vs 18%; p = 0.001). The proportion of children with physiological decompensation at triage had reduced significantly in the post intervention phase [513 out of 1403 (36.5%) vs. 310 out of 957 (32.3%); p = 0.001]. CONCLUSION: Referral education had significantly improved the quality of referral letters. Proportion of children with physiological decompensation at triage had decreased significantly after referral module. This change suggests sensitization of the peripheral hospitals towards a better referral process. Continued multifaceted approach will be required for sustained and increased benefits.

Acute exogenous lipoid pneumonia presenting as spontaneous air leak following accidental baby oil aspiration.

Exogenous lipoid pneumonia (ELP) is an uncommon cause of respiratory distress. The practice of oil massage and oil instillation into the nostrils is common in the Indian subcontinent. Accidental aspiration of baby oil may lead to significant chemical pneumonitis. This presentation may vary from subtle to severe respiratory distress requiring intensive care management. Spontaneous air leaks are rare in acute ELP. We successfully managed a six-month-old girl presenting with mineral oil-induced ELP and air leaks. However, these children require long-term follow-up, as a small proportion may evolve into chronic lung disease.