A Novel Approach to the Management of Neonatal Diabetes Using Sensor-Augmented Insulin Pump Therapy With Threshold Suspend Technology at Diagnosis

Marin, Monica T.; Coffey, Michael L.; Beck, Joni; Dasari, Paul S.; Allen, Rebecca A.; Krishnan, Sowmya

doi:10.2337/diaspect.29.3.176

Cited by 11 publications

(8 citation statements)

References 14 publications

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“…Among the cohort of paediatric diabetes, NDM constitutes a minor percentage of 0.17%. [ 8 ] Permanent NDM is caused by monogenic disorders affecting a number of genes that have been comprehensively reported. [ 3 ] All infants with NDM are treated with insulin therapy initially and those with positive KCNJ11 or ABCC8 mutations are changed over to oral glibenclamide therapy.…”

Section: Discussionmentioning

confidence: 99%

“…This is very different from the data published earlier where 46% of cases from consanguineous families had one of the potassium channel mutations[ 3 ] and other Indian studies which report at least 30% to have potassium channel mutations. [ 7 8 ] This could be attributed to a high degree of consanguinity in the south Indian cohort and referral bias. Perhaps with more prospective data, a better understanding of the same can be elicited.…”

Section: Discussionmentioning

confidence: 99%

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Genetic Heterogeneity and Challenges in the Management of Permanent Neonatal Diabetes Mellitus: A Single-Centre Study from South India

Korula

Ravichandran

Paul

et al. 2022

Indian Journal of Endocrinology and Metabolism

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Aim and Objectives: 1. To study the clinical outcome, growth and glycaemic control, 2. To study the frequency and type of genetic mutations. Methods: This is a retrospective study with a review of data of medical records from 2008 till date. Results: Twelve patients (six males) with neonatal diabetes mellitus (NDM) were identified. Median (interquartile range – (IQR)) age at diagnosis was 72 (31–95) days with a history of consanguinity in 75%. The median birth weight (range) was 2345 (900–3300) g. Follow-up data were available for eight patients with a median age at (IQR) follow-up of 3.3 (3–5.3) years. At follow-up, the mean annual HbA1c was 8.2% at a mean insulin dose of 1.1 U/kg/d. One patient with Wolcott-Rallison syndrome (WRS) and 21α-hydroxylase deficiency had poor growth and intellectual difficulty. The rest demonstrated satisfactory growth with an increase of mean weight centile from 2 nd to 13 th , height centile from 6.5 th to 20 th and normal neuro-cognitive development. Eleven patients underwent genetic testing with a molecular diagnosis in 54% (6/11): EIF2AK3 ( n = 2) and one each in INS , PDX1 , IL2RA and FOXP3 . None had variants in ABCC8 or KCNJ11 . One with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome underwent haematopoietic stem cell transplant (HSCT) and later succumbed. Conclusion: Our study demonstrates good clinical outcomes among NDM patients without immune dysfunction. Molecular diagnosis was attained only in around half of the patients (54%) with a great genetic heterogeneity.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Genetic Heterogeneity and Challenges in the Management of Permanent Neonatal Diabetes Mellitus: A Single-Centre Study from South India

Korula

Ravichandran

Paul

et al. 2022

Indian Journal of Endocrinology and Metabolism

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“…A sensor-augmented pump with threshold-suspend features was successfully used in a 3 month old ( INS : p.Tyr108Cys). Importantly, this case also describes the difficulties in obtaining insurance approval for new diabetes technologies, and how the authors overcame these obstacles [26].…”

Section: Updates On Existing Gene Causesmentioning

confidence: 99%

Congenital forms of diabetes: the beta-cell and beyond

Letourneau

Greeley

2018

Current Opinion in Genetics & Development

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The majority of patients diagnosed with diabetes less than 6 months of age, and many cases diagnosed between 6 and 12 months of age, have a gene mutation that causes permanent or transient hyperglycemia. Recent research advances have allowed for the discovery of new causes of congenital diabetes, including genes involved in pancreatic development (GATA4, NKX2-2, MNX1) and monogenic causes of autoimmune dysregulation (STAT3, LRBA). Ongoing follow-up of patients with KCNJ11 and ABCC8 mutations has supported the safety and efficacy of sulfonylureas, as well as the use of insulin pumps and continuous glucose monitors in infants with insulin-requiring forms of monogenic diabetes. Future studies are needed to improve clinical care and outcomes for these patients and their families.

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“…Sensor-augmented pump (SAP) therapy is a treatment method that combines continuous glucose monitoring (CGM) with continuous subcutaneous insulin infusion (CSII) ( 3 ). SAP therapy has been widely used in children with type 1 diabetes mellitus (DM), but its use in patients with NDM is limited ( 4 , 5 , 6 ). To the best of our knowledge, there are very few reports of patients with NDM who received SAP therapy in the neonatal period for more than several months.…”

Section: Introductionmentioning

confidence: 99%

“…SAP therapy has been widely used in children with type 1 diabetes mellitus (DM), but its use in patients with NDM is limited [4][5][6] . To the best of our knowledge, there are very few reports of patients with NDM who received SAP therapy in the neonatal period for more than several months.…”

Section: Introductionmentioning

confidence: 99%

Long-term sensor-augmented pump therapy for neonatal diabetes mellitus: a case series

Fukuda

Ishii

Kamasaki

et al. 2022

Clin Pediatr Endocrinol

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. Neonatal diabetes mellitus (NDM) is a rare metabolic disorder that is mainly present in the first 6 months of life and necessitates insulin treatment. Sensor-augmented pump (SAP) therapy has been widely used in children with type 1 diabetes mellitus, but its use in patients with NDM is limited. We report three patients with NDM who received SAP therapy using the MiniMed™ 640G system starting in the neonatal period. Two patients were treated for 3 months, and one patient continued treatment up to an age of 22 mo. The MiniMed 640G system can automatically suspend insulin delivery (SmartGuard™ Technology) to avoid hypoglycemia when the sensor glucose level is predicted to approach the predefined threshold. We suggest that SmartGuard Technology is particularly useful for infants in whom hypoglycemia cannot be identified. The MiniMed 640G system automatically records the trends of sensor glucose levels and the total daily dose of insulin, which can make the management more accurate and reduce the family’s effort. SAP therapy for patients with NDM automatically prevents severe hypoglycemia and is useful for long-term management; however, attention should be paid to its application.

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A Novel Approach to the Management of Neonatal Diabetes Using Sensor-Augmented Insulin Pump Therapy With Threshold Suspend Technology at Diagnosis

Cited by 11 publications

References 14 publications

Genetic Heterogeneity and Challenges in the Management of Permanent Neonatal Diabetes Mellitus: A Single-Centre Study from South India

Genetic Heterogeneity and Challenges in the Management of Permanent Neonatal Diabetes Mellitus: A Single-Centre Study from South India

Congenital forms of diabetes: the beta-cell and beyond

Long-term sensor-augmented pump therapy for neonatal diabetes mellitus: a case series

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