ABSTRACT
Donohue syndrome (DS) is a severe form of congenital insulin resistance due to mutation(s) in the insulin receptor (INSR) gene. Given the similarities between insulin and insulin-like growth factor 1 (IGF-1) receptors, recombinant human IGF-1 (rhIGF-1) has been used to treat severe insulin resistance due to INSR mutation(s). Traditional subcutaneous therapy may be limited by the shortened IGF-1 half-life in these patients. We report the case of a female with molecularly confirmed DS treated with continuous rhIGF-1 therapy via an insulin pump. With treatment, the patient's hemoglobin A1c decreased from 9.8% to 8.8%, and her weight increased by 0.8 kg. Development of an ovarian tumor complicated her course, but it was unclear whether this was related to rhIGF-1 therapy. Limited treatment options exist for patients with DS. The use of continuous rhIGF-1 via an insulin pump may be a viable option, although further experience is needed to establish safety and efficacy.
Subject(s)
Donohue Syndrome/drug therapy , Infusion Pumps , Insulin-Like Growth Factor I/administration & dosage , Recombinant Proteins/administration & dosage , Antigens, CD/genetics , Donohue Syndrome/genetics , Female , Humans , Infant, Newborn , Injections, Subcutaneous , Insulin-Like Growth Factor I/pharmacology , Mutation/genetics , Prognosis , Receptor, Insulin/genetics , Recombinant Proteins/pharmacologyABSTRACT
PURPOSE: The purpose of this article is to provide nurses with an overview of the latest technologies used in the management of diabetes, as well as information about future goals of technology in this arena. Advances in technology have had a major impact on diabetes management in the inpatient and outpatient setting. Children with type 1 diabetes mellitus are increasingly utilizing continuous subcutaneous insulin infusions (insulin pumps) and continuous glucose monitoring systems (CGMS), both of which have been shown to improve glycemic control and quality of life. Insulin pumps provide users a tool to administer insulin with more efficiency, precision, and flexibility than is possible with injections. The CGMS is able to generate information on glucose values every 1 to 5 minutes, allowing for a more comprehensive analysis of glucose trends and patterns. All devices, however, have limitations, and it is therefore essential that proper education for families and providers be provided to promote successful use and reduce adverse events. Hospitals also need guidelines on how to incorporate these new technologies in an inpatient setting. Future technology is focused on the development of smaller insulin pumps, more patch pumps, multihormone pumps such as with pramlintide, greater CGM accuracy, and a more reliable system for device communication. The ultimate goal of diabetes technology is a closed-loop system where euglycemia can be achieved with minimal patient intervention.
