Abstract

Keywords
There is an increasing recognition of the importance of Sensor Augmented Pump Therapy (SAP) in the management of Neonatal Diabetes.1,2
Neonatal diabetes mellitus (NDM) is a rare disorder, predominantly monogenic in origin. Generally, NDM presents as persistent hyperglycemia within the first 6 months of life. 3 Mutations involving the KCNJ11, ABCC8 and INS genes accounts for nearly 70% of monogenic causes of permanent neonatal diabetes mellitus (PNDM).
Mutations in the PDX1 gene are a rare cause of NDM associated with pancreatic agenesis.4,5 In these infants, hyperglycemia is combined with severe intrauterine growth restriction (IUGR) and exocrine pancreas insufficiency. All these conditions lead to a very low birth weight.
We present a case of permanent neonatal diabetes mellitus due to compound heterozygosity on PDX1 gene managed with a SAP therapy (MiniMed 640G™ insulin pump with SmartGuard®) and Predictive Low Glucose Suspend function (PLGS). She was born at 34 weeks of gestation with birth weight of 1180 g. We want to share our experience with this rare case of diabetes because of the peculiar choice of the insulin infusion site (arm skin). In fact, we found a lot of difficulties because of the poor subcutaneous tissue in the usual sites of infusion (abdomen, buttocks) 6 and because we reserved the skin of the thighs for the glucose sensor application (the transmitter needed more space).
In the video it is shown the application of the cannula needle (Minimed Silhouette 13 mm®). We preferred an oblique cannula of 13 mm for a better fixing in subcutaneous tissue, for supporting against needle dislodging and to control application site (this set had a window on the top). The procedure was simple and repeatable. Pain level (NIPS) was 2.
We used this video to show to all the medical and nursing staff of Neonatal Intensive Care Unit this unusual procedure, that we repeated every 3 days for 4 months, period of patient’s hospitalization. We believe that our experience could help others who have to take in care newborns with diabetes mellitus with these skin conditions.
No cannula occlusions occurred during the period of hospitalization. About glycemic control, we were able to obtain a good result given the critical condition of this patient. The Time in Range (80-180 mg/dl), during the first 4 months of life, was of 62% and Time Below Range (<70 mg/dl) was 4%.
Obtaining a good glycemic control, through insulin administration, is fundamental in NDM to assure a good weight gain and to prevent hyperglycemia, as well as hypoglycemia, for their association with adverse neurological outcomes. Moreover, the intravenous administration of insulin would be complex, and the multiple subcutaneous injections of insulin give the same difficulties in finding an adequate skin site, even if not every 3 days (as with insulin pump infusion sets) but more times in a day.
Therefore, using a SAP, especially with PLGS, is a priority in the treatment of these critical newborns even when skin conditions are not optimal.
Supplemental Material
sj-pdf-1-dst-10.1177_19322968211043112 – Supplemental material for Sensor Augmented Pump Therapy is Safe and Effective in Very Low Birth Weight Newborns Affected by Neonatal Diabetes Mellitus, with Poor Subcutaneous Tissue: Replacement of the Insulin Pump Infusion Set on the Arm, a Video Case Report
Supplemental material, sj-pdf-1-dst-10.1177_19322968211043112 for Sensor Augmented Pump Therapy is Safe and Effective in Very Low Birth Weight Newborns Affected by Neonatal Diabetes Mellitus, with Poor Subcutaneous Tissue: Replacement of the Insulin Pump Infusion Set on the Arm, a Video Case Report by Angela Zanfardino, Mauro Carpentieri, Alessia Piscopo, Stefano Curto, Emanuele Miraglia del Giudice, Alessia Inverardi, Mario Diplomatico, Sabino Moschella, Ferdinando Spagnuolo, Elisabetta Caredda, Paolo Montaldo and Dario Iafusco in Journal of Diabetes Science and Technology
Footnotes
Acknowledgements
None
Abbreviations
IUGR, Intrauterine Growth Restriction; NDM, Neonatal Diabetes Mellitus; NIPS, Neonatal Infant Pain Scale; PLGS, Predictive Low Glucose Suspend Function; PNDM, Permanent Neonatal Diabetes Mellitus; SAP, Sensor Augmented Pump; VLBW, Very Low Birth Weight.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Supplemental Material
Supplemental material for this article is available online.
References
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
