2025 – PAGE 87 – ENDOCRINOLOGY

(DOUBLE TAKE) KLINEFELTER SYNDROME (AKA KLINEFELTER’S)

Klinefelter Syndrome (AKA Klinefelter’s) = XXY. It presents with gynecomastia, small testicles, infertility, and normal intelligence to MILD intellectual disability. Patients may have mild motor or speech delay, tall stature with long arms and legs, and a low upper-to-lower segment ratio. For workup, REFER FOR CHROMOSOMAL ANALYSIS!

MNEMONIC: Gynecomastia = Kalvin Klein FELT HER BREASTS. (Yes, I know Kalvin is misspelled > Klinefelter.)

PEARL: Mild intellectual disability may be described as a patient who is “awkward,” “below average in school,” or even just “shy.” Generally, though, these patients have some learning disabilities, but their IQ can be normal.

PEARL: Someone who is tall with long arms and legs could also be described as having a LOW “upper-to-lower segment ratio.”

DIABETES MELLITUS

HONEYMOON PERIOD

In diabetes mellitus, the “honeymoon period” usually occurs soon after the diagnosis of type 1 diabetes mellitus (T1DM). A lower insulin requirement occurs as the remaining islet cells begin to function again after the glucose toxicity is relieved via exogenous insulin injections. These remaining islet cells begin to produce insulin again and relieve the patient from having such a high insulin requirement.

MNEMONIC: After the beginning of any budding relationship comes a short time referred to as the “honeymoon period.” Why should it be any different for patients in their relationships with DM?

HEMOGLOBIN A1C

A hemoglobin A1C of 8 is OKAY in kids. Tighter control is dangerous. An A1C of 10 or higher is BAD and can result in poor growth and hepatomegaly.

SOMOGYI EFFECT & DAWN PHENOMENA

The Somogyi effect (AKA Somogyi phenomenon) and the Dawn phenomenon (AKA Dawn effect) have been tested less in recent years. Both cause early morning HYPERglycemia.

• ­SOMOGYI EFFECT: This has recently been discredited and is unlikely to be tested. The pattern USED to include hypoglycemia around 2–3 AM (diaphoresis or nightmares in the middle of the night) followed by REBOUND HYPERGLYCEMIA at 6–8 AM. The treatment is DECREASED nighttime insulin.
  • MNEMONIC: Imagine, “Mr. Miyagi from that karate movie is having trouble reading his insulin vial. What’s the worst thing he could do? He gives himself TOO MUCH insulin every night (resulting in low glucose levels around 2 AM). Treatment? Get the old man some glasses!”
• DAWN PHENOMENON: Hyperglycemiaoccurs at 6–8 AM. While there are several theories, most believe it’s due to a PHYSIOLOGIC RELEASE OF GROWTH HORMONE or other hormones that can signal the liver to release glucose. Treat by decreasing nighttime carbohydrates, giving an early AM insulin dose (by pump), or changing the injection site.

HYPOGLYCEMIA

Hypoglycemia is generally considered to be a glucose level < 60 mg/dL. Give 15 grams of fast-acting carbohydrates (corn syrup, crackers, juice, sugar, soda), and recheck in 15 minutes if minimally symptomatic. If still < 60 mg/dL, repeat carbohydrate bolus. If more emergent, give a dextrose bolus via IV and consider a drip. For infants and young children give 2 ml/kg of D10 (max dose is 500 ml, which equals 50 grams of dextrose). For adolescents, simply give ½ –1 ampule of D50 (12.5 – 25 grams of dextrose).

PEARL: Percent solutions refer to grams/100 ml. For example, D50 refers to 50 grams of dextrose per 100 ml water. So, a 50 ml “ampule” of D50 contains 25 grams of dextrose.

PEARL: Fifteen grams of sugar can be found in one tablespoon of sugar or honey, four ounces of fruit juice, or six ounces of soda.