415should be carefully examined for other features of insulin resistance (double
chin, skin tags, and central obesity), hypertension, and xanthelasmas. He should
also be evaluated for dyslipidemia and microvascular complications. Female
patients should be looked for features of PCOS. The index patient was advised
to follow lifestyle modification and was initiated on metformin 1 g twice a day
after meals. At 3 months of follow-up, he lost 5 Kg weight and his HbA1c was
reduced to 6.8 % (Fig. 12.4).- What are the monogenic forms of diabetes?
Monogenic disorders are characterized by mutation of a single gene. The
monogenic forms of diabetes are rare and contribute only 1–2 % of individuals
with diabetes. Monogenic forms of diabetes could be due to defects in β-cell
function (MODY, neonatal diabetes, mitochondrial diabetes, and mutant insulin
syndromes) or defects in insulin action (congenital lipodystrophic diabetes,
type A insulin resistance syndrome, leprechaunism, and Rabson–Mendenhall
syndrome).- What is maturity onset diabetes of the young?
Maturity onset diabetes of the young (MODY) includes a heterogeneous
group of monogenic disorders and is clinically characterized by the early
onset of diabetes (<25 years of age), vertical transmission of disease in three
generations (autosomal dominant mode of inheritance) with at least one fam-
ily member with onset of disease <25 years of age, and not requiring insulin
for at least initial 5 years. Patients with MODY have a primary defect in
β-cell function and are usually nonobese and are negative for islet
autoantibodies.Fig 12.4 Grade IV
acanthosis nigricans in an
adolescent with T2DM
12 Diabetes in the Young