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penetration into ovum). Patients with diabetes, having any of the following

features like encephalomyopathy, hypertrophic cardiomyopathy, external oph-

thalmoplegia, optic atrophy, or stroke-like episodes should also be suspected to

have mitochondrial diabetes. Mitochondrial disorders result in defective oxida-

tive phosphorylation in β-cell, and hence impaired ATP generation and glucose-

induced insulin secretion. These individuals are often initially misdiagnosed as

type 1 (although islet cell autoantibodies are negative) or type 2 diabetes.

85. What is DIDMOAD syndrome?

The term DIDMOAD refers to diabetes insipidus, diabetes mellitus, optic atro-

phy, and deafness (Wolfram syndrome). The earliest abnormality in affected

individuals is diabetes mellitus (median age 6 years), followed by optic atrophy

(median age 11 years), diabetes insipidus (median age 14 years), and deafness

(median age 16 years). DIDMOAD syndrome is inherited as an autosomal

recessive disorder and is due to mutations of Wolframin gene, which regulates

calcium homeostasis in endoplasmic reticulum. Other manifestations include

urogenital tract atonia and neurological features like ataxia, peripheral neu-

ropathy, and psychiatric manifestations.

Further Readings

1. American Diabetes Association. Diagnosis and classification of diabetes mellitus. Diabetes
   Care. 2008;31:S55–60.


2. American Diabetes Association. Standards of medical care in diabetes—2015. Diabetes Care.
   2015;38:S1–90.


3. DeGroot L, Jameson J. Endocrinology. Philadelphia: Saunders/Elsevier; 2010.


4. Fajans SS, Bell GI, Polonsky KS. Molecular mechanisms and clinical pathophysiology of
   maturity-onset diabetes of the young. N Engl J Med. 2001;345:971–80.


5. Flanagan SE, Patch AM, Mackay DJ, Edghill EL, Gloyn AL, Robinson D, et al. Mutations in
   ATP-sensitive K+ channel genes cause transient neonatal diabetes and permanent diabetes in
   childhood or adulthood. Diabetes. 2007;56:1930–7.


6. Melmed S, Williams R. Williams textbook of endocrinology. Philadelphia: Elsevier/Saunders;
   2011.


7. Stenstrom G, Gottsater A, Bakhtadze E, et al. Latent autoimmune diabetes in adults: definition,
   prevalence β-cell function, and treatment. Diabetes. 2005;54:S69–72.


8. The Diabetes Control and Complications Trial Research Group. The effect of intensive treat-
   ment of diabetes on the development and progression of long-term complications in insulin-
   dependent diabetes mellitus. N Engl J Med. 1993;329:977.


9. The Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and
   Complications Research Group. Retinopathy and nephropathy in patients with type 1 diabetes
   four years after a trial of intensive therapy. N Engl J Med. 2000;342:381–9.


10. Umpierrez GE. Ketosis-prone type 2 diabetes time to revise the classification of diabetes.
    Diabetes Care. 2006;29:2755–7.


11. Unnikrishnan R, Mohan V. Fibrocalculous pancreatic diabetes (FCPD). Acta Diabetol.
    2015;52:1–9.

12 Diabetes in the Young