ABSTRACT
Simulation based training (SBT) plays a pivotal role in quality improvement and patient safety. Simulation is not only for training health care professionals but also an excellent tool for systems and facility changes which will potentially improve patient safety and ultimately outcomes. SBT is already established both as a training modality, and as a quality improvement tool in high income countries. It's use in low and middle-income countries (LMIC), including India, however, is sporadic and variable because of multiple barriers. The barriers for establishment of simulation are lack of knowledge about benefits of simulation, psychological resistance, cost, and lack of trained faculty. PediSTARS (Pediatric Simulation Training and Research Society), a simulation society was founded in August 2013 to spread the simulation across India and thus improve the quality and safety of health care using SBT. In this article we discuss various barriers for healthcare simulation in India and also our attempts to overcome some of these barriers by collaborative practice.
ABSTRACT
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in neonates and infants. Medical treatment includes the use of high concentrations of glucose and combinations of diazoxide, octreotide and glucagon. We report our experience of using sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, in the treatment of CHI in seven newborns who are poorly responding to standard medical therapy. Majority (87%) of infants achieved euglycaemia using a combination of oral feeding and the addition of sirolimus to standard medical treatment. One infant who failed to achieve euglycaemia even after surgery managed successfully with sirolimus. Diagnosis was confirmed by genetics evaluation; in three infants, novel mutations were detected. Outcome and long-term follow-up of all cases are described.Conclusion: Sirolimus can be considered in treatment of CHI refractory to standard medical treatment or in cases unresponsive to surgical treatment. What is Known: ⢠Congenital hyperinsulinism (CHI) or persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) associated with mutations such as the ABBC8 or KCNJ gene known to cause hypoglycaemia refractory to standard medical treatment such as diazoxide and octreotide and may need subtotal pancreatectomy (STP). ⢠Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, was recently reported to be useful for refractory CHI cases with variable efficacy. What is New: ⢠Our case series describes efficacy and safety of sirolimus in seven genetically proven refractory CHI cases with mainly neonatal presentation. All patients' follow-ups are described. ⢠Out of seven infants, six infants responded well to sirolimus, and among these one infant who failed to respond to surgery (STP) also successfully managed with sirolimus. ⢠It highlights the right patient selection and right dose to successfully manage these cases without much adverse effects.
