Efficacy and safety of sirolimus in a neonate with persistent hypoglycaemia following near-total pancreatectomy for hyperinsulinaemic hypoglycaemia.

Hyperinsulinaemic hypoglycaemia (HH) is characterised by inappropriate insulin secretion and is the most common cause for persistent neonatal hypoglycaemia. The only treatment available for medically unresponsive hypoglycaemia is a near-total pancreatectomy. A neonate with severe HH, due to a homozygous ABCC8 mutation, was not responsive to treatment with maximal doses of diazoxide and subcutaneous daily octreotide, and underwent a near-total pancreatectomy; however, hypoglycaemia persisted. Introduction of sirolimus, an mTOR (mammalian target of rapamycin) inhibitor, obviated the requirement for glucose infusion. Euglycaemia was achieved with no significant adverse events from the drug. Sirolimus therapy was ceased at 13 months of age. No episodes of persistent hypoglycaemia were observed after cessation of sirolimus. This report demonstrates the successful use of sirolimus for persistent hypoglycaemia in the critically ill infant post pancreatectomy. Sirolimus could be considered in patients with severe HH not responsive to conventional medical and surgical therapy. However, the long-term efficacy and safety with this immunosuppressive drug in very young patients are not assured.

Review of the radiation exposure during screening of surgically implanted central venous access devices.

PURPOSE: Ionizing radiation is used for the insertion of surgically implanted venous access devices (SIVADs) with children at the highest risk of cumulative radiation effects from these procedures. This study examines the radiation dose in a pediatric population during intraoperative radiological screening. METHODS: A retrospective study looked at all pediatric patients in a tertiary hospital between January 2008 and January 2014 who had a surgically implanted venous access device inserted using intraoperative fluoroscopy. Patient demographics, reason for SIVAD insertion, the type and method of insertion, fluoroscopy time and radiation dose area product were determined. RESULTS: A total of 505 patients had 682 SIVADs inserted, with 123 patients receiving multiple SIVAD over the six year period. There were two types of SIVAD inserted, 492 were totally implanted venous access devices (TIVAD) and 190 were tunneled central venous catheters (cuffed central line). Five hundred seven of the SIVAD inserted recorded the dose area product and fluoroscopy time. The median time for screening was 5seconds (range 1 to 275seconds) and the median dose area product was 0.00352mGym(2) (range 0.000001mGym(2) to 0.28mGym(2)). Of the 507 SIVAD that recorded the radiation data, 479 were open surgical cut-down insertion and 27 were percutaneous insertion. Percutaneously inserted surgically implanted venous access devices (mean 0.0060mGym(2)) had a longer dose area product than open insertion (mean 0.0034mGym(2); p=0.05). CONCLUSION: Screening of SIVAD involves low levels of radiation exposure and is comparable to a chest x-ray or a transatlantic flight. The excess lifetime cancer risk to patients is estimated to be very low and is considered to be outweighed by the benefits of insertion. Open surgical cut-down insertion has a significantly reduced radiation exposure compared to percutaneous techniques. Although radiation dose is higher with percutaneous procedures, the clinical effects are considered minimal, and the resultant radiation risk is estimated to be very low. Radiation dose should not determine technique of insertion of SIVAD.

Evidence of improved milk intake after frenotomy: a case report.

Ankyloglossia (tongue tie) is a well-recognized cause of breastfeeding difficulties and, if untreated, can cause maternal nipple pain and trauma, ineffective feeding, and poor infant weight gain. In some cases, this condition will result in a downregulation of the maternal milk supply. Milk-production measurements (24-hour) for a breastfeeding infant with ankyloglossia revealed the ineffective feeding of the infant (78 mL/24 hours), and a low milk supply (350 mL/24 hours) was diagnosed. Appropriate management increased milk supply (1254 mL/24 hours) but not infant milk intake (190 mL/24 hours). Test weighing convincingly revealed the efficacy of frenotomy, increasing breastfeeding milk transfer from 190 to 810 mL/24 hours. Postfrenotomy, breastfeeding almost completely replaced bottle-feeding of expressed breast milk. This case study confirms that ankyloglossia may reduce maternal milk supply and that frenotomy can improve milk removal by the infant. Milk-production measurements (24-hour) provided the evidence to confirm these findings.