Continuous Glucose Monitoring in Transient Neonatal Diabetes Mellitus—2 Case Reports and Literature Review
Abstract
:1. Introduction
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- permanent neonatal diabetes mellitus (PNDM)
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- transient neonatal diabetes mellitus (TNDM)
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- moderate or severe intrauterine growth restriction
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- early development (during the first week of life)
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- rare, mild, non-ketotic hyperglycemia
2. Case Presentations
3. Discussion
4. Conclusions
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- The availability of genetic results (in a few weeks) significantly altered the short-and long-term management of these infants.
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- These babies need insulin with adequate or high caloric intake to ensure satisfactory weight gain. Insulin administration can now partially mimic the pancreas physiology.
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Patient | Case Report 1 | Case Report 2 |
---|---|---|
Gender | F | F |
Age | First day of life | First day of life |
Birth weight | 1480 g—37 weeks of gestation Severe Intrauterine growth restriction IUGR < 3rd percentile | 2100 g—35 weeks of gestation Intrauterine growth restriction (Oligohydramnios) |
Length | 43 cm | 44 cm |
Head circumference | 29 cm | 31 cm |
Feeding type | Formula-fed up to 13 days of life, then breastfed | Mixed fed—breastfed and formula fed since the first day of life |
Clinical features | facial dysmorphism epicanthus umbilical hernia | facial dysmorphism epicanthus macroglossia pectus excavatum umbilical hernia short hallux |
Glucose level at diagnosis | 275 mg/dL | 350 mg/dL |
Genetic test | Uniparental disomy at the 6q24 locus | Uniparental disomy at the 6q24 locus |
Treatment | Insulin Lispro was started continuous with a rhythm between 0.1–0.3 mL/h (0.01–0.03 units/kg/h) for the first days and constantly 0.1 mL/h after that (0.43 units/24 h); At 2 months—insulin pump with a dose 0.05 units/h | Insulin Lispro was started continuous with a rhythm between 0.026 units/kg/day and then reduced gradually up to 0.006 units/kg/day; |
Evolution | Discharge after 2 months and 2 weeks Lispro Insulin dose was reduced gradually and subsequently was stopped one week following her discharge; At the age of 12 months, the baby develops very well, normal anthropometric parameters, no infectious episodes and no hyper- or hypoglycemic events. | Discharge at 2 months when treatment with Lispro insulin was stopped; At age of 6 months, the baby develops very well, with normal anthropometric parameters, no infectious episodes and no hyper- or hypoglycemic events. |
Mechanism of β-Cell Dysfunction | Gene Mutation | Chromosome Locus | Inheritance | Additional Features | Therapy |
---|---|---|---|---|---|
Reduced β-cell development | ZAC (IPLAG1)/HYMA1 | 6q24 | imprinting; AD | macroglossia; umbilical hernia | insulin |
ZEP57 | 6p22.1 | AR | insulin | ||
HNF1B | 17q21.3 | AD | pancreatic cysts hypoplasia; renal | ||
Failure membrane to depolarize | KCNJ11 (Kir6.2) | 11p15.1 | AD; de novo | low developmental birth weight; delay; DEND | sulfonylurea |
Failure channel to close KATP | ABCC8 (SUR1) | 11p15.1 | AD; AR; de novo | low birth weight | sulfonylurea |
Abnormal β-cell function | INS (proinsulin) | 11p15.5 | AR | low birth weight | Insulin |
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Chisnoiu, T.; Balasa, A.L.; Mihai, L.; Lupu, A.; Frecus, C.E.; Ion, I.; Andrusca, A.; Pantazi, A.C.; Nicolae, M.; Lupu, V.V.; et al. Continuous Glucose Monitoring in Transient Neonatal Diabetes Mellitus—2 Case Reports and Literature Review. Diagnostics 2023, 13, 2271. https://doi.org/10.3390/diagnostics13132271
Chisnoiu T, Balasa AL, Mihai L, Lupu A, Frecus CE, Ion I, Andrusca A, Pantazi AC, Nicolae M, Lupu VV, et al. Continuous Glucose Monitoring in Transient Neonatal Diabetes Mellitus—2 Case Reports and Literature Review. Diagnostics. 2023; 13(13):2271. https://doi.org/10.3390/diagnostics13132271
Chicago/Turabian StyleChisnoiu, Tatiana, Adriana Luminita Balasa, Larisia Mihai, Ancuta Lupu, Corina Elena Frecus, Irina Ion, Antonio Andrusca, Alexandru Cosmin Pantazi, Maria Nicolae, Vasile Valeriu Lupu, and et al. 2023. "Continuous Glucose Monitoring in Transient Neonatal Diabetes Mellitus—2 Case Reports and Literature Review" Diagnostics 13, no. 13: 2271. https://doi.org/10.3390/diagnostics13132271