Growth and Maturation in Children With Type 1 Diabetes

José F. Cara, MD

Disclosures

March 22, 2001

Question

I encountered a 19-year-old man with type 1 diabetes of 10-11 years' duration. In recent years, he exhibited diabetic nephropathy with impaired renal function and high blood pressure (serum creatinine = 3.4 mg/dL with no obstructive uropathy or congenital genitourinary tract abnormality).

The interesting point about this patient was the evidence of delayed puberty: small testes (about 2-3 mL for both), scanty pubic hair (Tanner Stage II-III), short stature (145 cm and 47 kg), and bone age 14-15 years. Hormone analysis revealed normal growth hormone levels, normal thyroid function, and normal basal levels of luteinizing hormone, follicle-stimulating hormone, and testosterone. Because this patient had type 1 diabetes for more than 10 years, resultant end-stage renal disease (ESRD) and hemodialysis is indicated in the future.

How do I treat this patient's short stature and delayed puberty (delayed bone age 4-5 years)? Are there many cases of type 1 diabetes associated with delayed puberty and delayed bone age?

Chung-Jung Wu, MD

Response from José F. Cara, MD

Growth and maturation of children with diabetes depends on the adequacy of insulin administration and degree of metabolic control.[1,2] Children who are on appropriate doses of insulin and, as a result, have well-controlled diabetes typically have patterns of growth and development that are identical to those of otherwise healthy children. Underinsulinization and poor diabetes control can result in growth delay and growth attenuation. When severe, chronic insulin deficiency results in Mauriac syndrome, also known as "diabetic dwarfism." Children with Mauriac syndrome have decreased growth velocity, short stature, and delayed puberty.[1,2,3,4] They are often pale, have thickened skin, and have a protuberant abdomen with hepatomegaly. Improved insulin delivery usually results in normalization of growth but can produce rapid deterioration of retinopathy and nephropathy, if undertaken too aggressively.[4,5,6] Also, rapid skeletal maturation may occur in older teens after appropriate insulin therapy, resulting in short stature in adulthood.

The presence of nephropathy in a 19-year-old youngster with type 1 diabetes of 10-11 years' duration suggests long-standing underinsulinization and poor diabetes control. It is likely that his chronic insulin deficiency has resulted in his short stature, delayed puberty, and delayed bone age, as seen in Mauriac syndrome. It would be helpful to know the values of his hemoglobin A1C and insulin-like growth factor (IGF-1) levels (which are typically depressed despite normal growth hormone concentrations) to confirm the diagnosis. Whereas it is likely that improved insulin administration will help restore normal growth and maturation, it is likely that his skeletal maturation will advance too rapidly for him to reach a normal adult height. Also, care must be taken to avoid further exacerbation of his nephropathy and/or retinopathy following appropriate treatment of his diabetes.

Other diagnoses that may be considered in this young man include severe constitutional delay of adolescent growth and development, partial hypogonadotropic hypogonadism, and primary gonadal failure (hypergonadotropic hypogonadism).[6] These possibilities appear less likely, however, in view of the above considerations.

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