Clinical_Rounds_in_Endocrinology_Volume_II_-_Pediatric_Endocrinology

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has also been implicated in worsening of β-cell function due to failure of
β-cell to combat oxidative stress. With intensive insulin therapy, glucotoxic-
ity and oxidative stress are ameliorated which result in restoration of β-cell
function and consequent insulin independence in majority of these patients
during follow-up.


  1. What is the natural history of Flatbush diabetes?


After resolution of DKA, approximately 50 % of the patients are able to discon-
tinue insulin therapy within 6 months; however, 80 % of these patients require
oral antidiabetic drugs (sulfonylurea or metformin) and the rest 20 % could
maintain euglycemia only on lifestyle modification at 1 year of follow-up.
Recurrence of DKA is rare (2 %) in these patients.


  1. What is neonatal diabetes mellitus?


The onset of diabetes mellitus before 6 months of age is termed as neonatal
diabetes (NDM). However, 5–10 % of children with NDM may present between
6 and 12 months of age. Neonatal diabetes is classified as transient NDM
(TNDM), permanent NDM (PNDM), or syndromic NDM. Forty-five percent of
infants with NDM have transient diabetes, 45 % have permanent NDM, and
10 % have syndromic NDM. NDM is an uncommon disorder with prevalence
of 1 case per 300,000–500,000 live births.


  1. Why is T1DM uncommon before 6 month of age?


T1DM is uncommon before the age of 6 months possibly because immune sys-
tem is immature to elicit an immune response against environmental antigens.
However, infants with IPEX syndrome (immune dysfunction, polyendocrinop-
athy, enteropathy, X-linked syndrome), a form of neonatal diabetes, can present
with immune-mediated diabetes within 6 months of age.


  1. What is transient neonatal diabetes mellitus?


Transient neonatal diabetes mellitus (TNDM) usually manifests within first
several days or weeks of life and resolves by 12 weeks of life. Children with
TNDM present with failure to thrive, increased thirst, and frequent urination.
Although the blood glucose levels are very high, ketonemia is extremely rare.
Half of the infants with TNDM may have relapse of diabetes during adoles-
cence and early adulthood.


  1. What are the causes of transient neonatal diabetes mellitus?


Defective growth and development of β-cell during embryogenesis and in
fetal life results in decreased insulin secretion and consequently hyperglyce-

12 Diabetes in the Young
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