For the following disorders, select the serum concentrations (mEq/L) of sodium (Na+) and potassium (K+) with which it is most likely to be associated in a dehydrated patient.A 1-year-old girl has had since about 3 months of age repeated episodes of hypoglycemic seizures and bouts of lactacidosis. She has a protuberant abdomen due to her massive hepatomegaly, a doll-like facies, thin extremities, and is short for her age. Upon drawing her blood, you are struck by the “milky” appearance.
For the following disorders, select the serum concentrations (mEq/L) of sodium (Na+) and potassium (K+) with which it is most likely to be associated in a dehydrated patient.A 1-year-old girl has had since about 3 months of age repeated episodes of hypoglycemic seizures and bouts of lactacidosis. She has a protuberant abdomen due to her massive hepatomegaly, a doll-like facies, thin extremities, and is short for her age. Upon drawing her blood, you are struck by the “milky” appearance.
π‘ Explanation
**Core Concept**
The underlying condition described is likely a glycogen storage disease, given the symptoms of hypoglycemic seizures, lactacidosis, hepatomegaly, and the "milky" appearance of the blood, which suggests hypertriglyceridemia. This condition affects the body's ability to regulate blood sugar levels and can lead to electrolyte imbalances.
**Why the Correct Answer is Right**
In glycogen storage diseases, especially type I (von Gierke's disease), the inability to break down glycogen to glucose leads to severe hypoglycemia, lactic acidosis, and hypertriglyceridemia. The hypoglycemia and metabolic acidosis can lead to increased levels of potassium (due to acidosis-induced shift of potassium out of cells) and decreased levels of sodium, although sodium levels can be more variable. The described "milky" blood appearance is due to hypertriglyceridemia, a common finding in type I glycogen storage disease.
**Why Each Wrong Option is Incorrect**
**Option A:** Would not typically be associated with the described metabolic derangements.
**Option B:** Does not align with the expected electrolyte imbalances seen in glycogen storage disease.
**Option C:** Incorrect due to the expected hyperkalemia in the context of acidosis.
**Clinical Pearl / High-Yield Fact**
Glycogen storage diseases, particularly type I, are characterized by the inability to convert glycogen to glucose, leading to severe hypoglycemia, lactic acidosis, and hyperlipidemia. Recognizing the clinical presentation, including hepatomegaly and "milky" blood due to hypertriglyceridemia, is crucial for diagnosis.
**Correct Answer:** D. 130 mEq/L sodium, 6.0 mEq/L potassium
β Correct Answer: B. Na+ 125, K+ 3.0
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