Question 6: Susie is now 8 months pregnant and amniocentesis results confirm tha

Question

Question 6: Susie is now 8 months pregnant and amniocentesis results confirm that her baby carries the same PNDM causing mutation that she has. Susie and Carlos are looking into possible treatments for PNDM. Evaluate the two treatments below and determine which would be the best option for their baby. For full credit discuss why a particular treatment would or would not work.

Treatment 1:

Oral Sulfonylurea: Sulfonylureas, a class of drugs used to treat type 2 diabetes mellitus, have been recently approved for the treatment of PNDM. They close KATP channels by an ATP-independent route, thereby causing insulin secretion.

Treatment 2:

Recombinant Insulin: An appropriate regimen of subcutaneous insulin administration should be established when the infant is stable and tolerating oral feedings. The longer-acting preparations with no significant peak-of-action effect such as Lantus

Explanation / Answer

Permanent neonatal diabetes mellitus (PNDM) is due to KCNJ11 gene mutation occurs in 6 months of birth

1. KCNJ11 mutation is treated withoral hypoglycemic agent sulfonylurea. the treatment has now switched from insulin therapy to sulfonylurea. so it can be taken up with infants.treatment with oral sulfonylureahas shown dramatic improvements in hyperglycemia control

2.Recombinant Insulin: proinsulin disulfide isomers exhibited substantially decreased recovery, cannot be recommended

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