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Hypokalemic periodic paralysis (hypoKPP), also known as familial hypokalemic periodic paralysis (FHPP), [1] is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis when there is a fall in potassium levels in the blood (hypokalemia).
Potassium resides mainly inside the cells of the body, so its concentration in the blood can range anywhere from 3.5 mEq/L to 5 mEq/L. [14] The kidneys are responsible for excreting the majority of potassium from the body. [14] This means their function is crucial for maintaining a proper balance of potassium in the blood stream.
Causes of hypokalemia include vomiting, diarrhea, medications like furosemide and steroids, dialysis, diabetes insipidus, hyperaldosteronism, hypomagnesemia, and not enough intake in the diet. [1] Normal potassium levels in humans are between 3.5 and 5.0 mmol/L (3.5 and 5.0 mEq/L) with levels below 3.5 mmol/L defined as hypokalemia.
Hypokalemia leads to hyperpolarization of muscle cells, making the neuromuscular junction less responsive to normal nerve impulses and leading to decreased contractility of the muscles. [ 1 ] It is not clear how the described genetic defects increase the Na + /K + -ATPase activity, but it is suspected that the enzyme becomes more active due to ...
Hypocalcemia is a medical condition characterized by low calcium levels in the blood serum. [5] The normal range of blood calcium is typically between 2.1–2.6 mmol/L (8.8–10.7 mg/dL, 4.3–5.2 mEq/L), while levels less than 2.1 mmol/L are defined as hypocalcemic. [1] [3] [6] Mildly low levels that develop slowly often have no symptoms.
A typical TTKG in a normal person on a normal diet is 8-9. During hyperkalemia or high potassium intake, more potassium should be excreted in the urine and the TTKG should be above 10. Low levels (<7) during hyperkalemia may indicate mineralocorticoid deficiency, especially if accompanied by hyponatremia and high urine Na.
Metabolic alkalosis is an acid-base disorder in which the pH of tissue is elevated beyond the normal range (7.35–7.45). This is the result of decreased hydrogen ion concentration, leading to increased bicarbonate (HCO − 3), or alternatively a direct result of increased bicarbonate concentrations.
The most common causes of high anion gap metabolic acidosis are: ketoacidosis, lactic acidosis, kidney failure, and toxic ingestions. [3]Ketoacidosis can occur as a complication of diabetes mellitus (diabetic ketoacidosis), but can occur due to other disorders, such as chronic alcoholism and malnutrition.