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Pancreas/GI Tract: Diabetes Mellitus
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Patients with pancreatic insufficiency often develop diabetes mellitus because pancreatic duct blockages and digestive enzyme damage lead to fibrosis or scarring of the pancreas. This scarring impairs the endocrine functions of the pancreas, such as regulating insulin secretion. Impaired production of insulin results in diabetes mellitus.

Diabetes or impaired glucose regulation occurs in 15 percent of CF patients over the age of 35. The CF-related diabetes (CFRD) has Type I and Type II characteristics; CFRD is more similar to Type I diabetes because the pancreatic tissue that makes insulin is damaged by recurrent blockages of the ducts and the development of fibrosis. 

Type 1Type 2CFRD
No insulin secretionSlight insulin secretionSlight insulin secretion
Pancreatic cells are damagedPancreatic cells are damagedPancreatic cells are damaged
Rapid onset presenting in childhoodSlow onset later in lifePresents between ages 16-20
Excessive thirstExcessive thirstExcessive thirst
Increased urinationIncreased urinationIncreased urination
Weight lossWeight lossWeight loss

Once diagnosed, strict blood glucose control is required by frequent monitoring and insulin injections. A decline in pulmonary symptoms may be related to uncontrolled diabetes. Other complications, including kidney, neurologic and eye disease can also occur. 

Eye Disease
CFRD can damage the retina, resulting in diabetic retinopathy. Progressive damage to the small blood vessels that supply the retina, which can lead to diminished vision, occurs in up to 15 percent of people with CFRD. Untreated, this can cause scarring, bleeding, and detachment of the retina, resulting in vision loss. Strict blood glucose control is essential. An annual evaluation by an ophthalmologist is recommended.

Neurologic Disease
CFRD can damage the peripheral nervous system that controls sensation. Similar to the damage caused in the eyes, blood supply to nerves that control sensation in the hands and feet is impaired, resulting in numbness in about 20 percent of people with CFDR. Strict blood sugar control is the best prevention. 

Kidney Disease
A type of kidney damage called diabetic nephropathy occurs in about 10 percent of CFRD patients. Developing later in the course of the disease, symptoms include headaches, confusion, swelling of the legs, and decreased urination. Untreated, it can cause kidney failure. Prevention requires strict blood sugar control and monitoring of kidney function by blood and urine tests. Medications and lifestyle modifications can also slow disease progression. 

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