What is Hyperosmolar Hyperglycemic Syndrome?
Hyperosmolar hyperglycemic syndrome (HHS) is a medical issue that arises from complications related to diabetes. It’s most commonly associated with Type 2 diabetes. Around 1880, medical professionals Won Frerichs and Dreschfeld first discovered this condition in patients who had diabetes with high levels of blood sugar and sugar in their urine. However, these patients lacked specific symptoms often seen in diabetic ketoacidosis such as heavy breathing or acetone in the urine. Earlier, this condition was referred to by different names like non-ketotic hyperglycemic coma or hyperosmolar non-ketotic coma (HONK).
Diabetes is a medical condition where the main issue is a high level of blood sugar, known as hyperglycemia. This can result from either a lack or not enough insulin in the body. Insulin is a hormone produced by specific cells in the pancreas. It helps to lower the level of glucose in the blood by promoting the storage of glucose in fat tissues and muscles. Insulin also slows down the breakdown of fat in body fat tissues. However, other hormones like glucagon and catecholamines can counter insulin’s metabolic effects.
Type 1 diabetes, affecting about 5-10% of diabetic patients, comes from the body’s immune system attacking insulin-producing cells in the pancreas. A common complication for this type is diabetic ketoacidosis (DKA), a serious condition marked by very high blood sugar levels.
Type 2 diabetes, accounting for about 90% to 95% of diabetes cases, is most commonly found in individuals who are obese. Obesity and a high body mass index (BMI) can cause resistance to insulin in the body’s tissues. Although the pancreas continues to produce insulin in these cases, it is not enough to counteract the body’s resistance.
HHS is a severe and possibly fatal complication of type 2 diabetes. The death rate due to HHS can be as high as 20%, which is roughly ten times greater than the mortality rate seen in diabetic ketoacidosis. Factors determining outcomes and prognosis in HHS include patients’ age, the degree of dehydration, and the presence of other health issues.
What Causes Hyperosmolar Hyperglycemic Syndrome?
In kids and young adults who have type 1 or type 2 diabetes, illnesses, particularly those affecting the respiratory system (like the lungs), circulatory system (like the heart and blood vessels), and genitourinary system (like the kidneys and bladder), can lead to a condition known as Hyperosmolar Hyperglycemic State (HHS). Being overweight and drinking too many sugary beverages can also increase the risk of developing HHS. It’s important to note that more children are now being diagnosed with type 2 diabetes, which also increases the likelihood of developing HHS.
Generally speaking, HHS is most common in people with type 2 diabetes. If someone with diabetes manages their blood sugar well, they’re less likely to develop HHS. However, certain conditions and factors can trigger its onset. Infections are the leading cause, specifically those affecting the respiratory, digestive, and genitourinary systems. This happens because of water loss and the release of certain hormones (known as endogenous catecholamines) that can affect the body’s response to disease.
Medications used to treat different conditions in elderly patients with type 2 diabetes could also trigger HHS. These medications include thiazide diuretics (used to reduce blood pressure), beta-blockers (drugs that manage heart disease and hypertension), glucocorticoids (used to treat inflammation), and some types of antipsychotics.
Lastly, serious heart conditions like strokes, angina (chest pain due to reduced blood flow), and heart attacks can also trigger HHS. These conditions stress the body, releasing hormones that increase blood sugar levels. This can lead to osmotic diuresis (where the body gets rid of sugar through urine, causing dehydration) and result in HHS.
Risk Factors and Frequency for Hyperosmolar Hyperglycemic Syndrome
There’s not a lot of data on the rates of a condition called HHS. However, some studies suggest that about 1% of all hospital admissions for diabetes are due to this condition.
HHS tends to affect patients in their 50’s and 60’s. Meanwhile, another condition, DKA, is more common in younger patients, with the most cases seen in people in their 40’s.
In the US, we’re seeing more cases of type 2 diabetes because of increased rates of childhood obesity, which is often linked to a diet high in carbohydrates. This might also mean an increasing number of HHS cases among children.
HHS also disproportionately affects African Americans, Native Americans, and Hispanics. This could be because type 2 diabetes is also more common in these groups. Particularly, overweight African American men may be at a higher risk.
Signs and Symptoms of Hyperosmolar Hyperglycemic Syndrome
To diagnose Hyperglycemic Hyperosmolar Syndrome (HHS), doctors place a lot of importance on your medical history and a physical examination. There are often similarities in the signs and symptoms of HHS and diabetic ketoacidosis (DKA). During the initial assessment, the doctor will pay careful attention to aspects like your insulin routine, missed medication, high carbohydrate consumption, and usage of certain medications that can trigger high blood sugar levels or cause dehydration.
If an infection triggers HHS, you might experience symptoms like fever, fatigue, general weakness, rapid breathing, and an increased heart rate. Alternatively, if a heart or blood vessel problem signals the onset of HHS, symptoms can include chest pain, chest tightness, headache, dizziness, and heart palpitations.
Patients with HHS typically present with symptoms like increased urination (polyuria) and excessive thirst (polydipsia). This is due to severe dehydration and high blood sugar stimulating the thirst center in the brain. Other common symptoms include weakness, fatigue, and feeling sluggish.
Severe dehydration associated with HHS could also affect your skin and mouth. Generally, the skin and the interior of the mouth are dry with a slow capillary refill.
Another important distinguishing characteristic of HHS is neurological signs. Decreased blood flow to the brain resulting from extreme dehydration can lead to issues with motor functions, disturbed vision, confusion, and even unconsciousness in severe cases.
The doctor will also conduct a system-based physical assessment, looking at:
- General appearance: Patients with HHS usually appear unwell with altered mental status
- Cardiovascular: Increased heart rate, fainting when standing up, weak and threadlike pulse
- Respiratory: Breathing rate could be normal, but may be rapid if the body has excessive acid
- Skin: Delayed blood refill in your skin, poor skin elasticity, skin clinging to your body; the symptoms may not be present in extreme dehydration due to obesity
- Genital and urinary: Reduced urine output
- Central Nervous System (CNS): Dysfunction in motor skills, drowsiness with a low consciousness score, patients may even be unconscious in serious cases of HHS.
During the physical examination, the doctor would also focus on other conditions related to diabetes. The existence of disorders like Acanthosis nigricans, oral thrush, vulvovaginitis, and multiple pimple-like skin sores might indicate poor blood sugar management, especially if HHS is the first sign of type 2 diabetes.
Testing for Hyperosmolar Hyperglycemic Syndrome
Hyperosmolar Hyperglycemic Syndrome (HHS) is a health condition typically diagnosed based on a series of specific diagnostic criteria. This standard was established based on the framework from studies published in 1971. According to the guidelines from the American Diabetic Association and other international health associations, doctors identify HHS through a blood sugar level greater than 600 mg/dL, a plasma osmolarity (a measure of how concentrated substances are in your blood) above 320 mOsm/L, and no significant presence of ketoacidosis, which is a high level of acids in the blood.
The term, Hyperosmolar Hyperglycemic Non-ketotic coma, used to be applied to this condition, but it’s no longer accepted because all patients with HHS do not necessarily experience coma, despite high blood sugar levels and hyperosmolarity.
Doctors use various methods to check for HHS. Initially, they’ll prick your finger to check your blood glucose levels, which for HHS are usually between 600 to 1200 mg/dL. High levels of glucose increase your osmolarity and lead to dehydration. As part of your treatment, your doctor will monitor your glucose level every hour to ensure it doesn’t drop too quickly, as this could lead to a life-threatening condition called cerebral edema, or brain swelling. This is particularly a higher risk in HHS.
Other tests include a hemoglobin A1C test, which measures long-term glycemic control and helps in diagnosing new-onset diabetes. The doctor might also check your serum osmolarity, which is usually very high in HHS patients, and it might lead to a coma when the levels are too high. Your doctor would also check your electrolyte levels using a comprehensive metabolic panel.
In HHS, sodium can appear falsely low because of the presence of excess glucose in your blood. Your doctor will calculate your actual sodium level. The potassium level may be high or low depending on insulin and urinary fluid loss. Keep in mind that aggressive correction of a low potassium level isn’t advisable because of dehydration-related issues.
In HHS, the bicarbonate level will usually be normal, unlike “Diabetic Ketoacidosis” (DKA). The anion gap, which is a measure of the difference between positive and negative charged ions in your blood, is usually normal in HHS but high in DKA. An abnormally high anion gap, when present in HHS, might be due to lactic acid, produced by decreased blood flow to the tissues.
Magnesium and phosphorus levels may be low in HHS, while ketones in the blood are usually minimal. Electrolytes should be checked every 2 to 3 hours for those being treated for HHS.
To assess the level of acidosis, or the acidity of your blood, your doctor may use arterial blood gases. In HHS, the level of acids in the blood is usually higher than normal due to dehydration and inadequate blood supply to your organs. These blood gas measurements are usually checked every 2 to 3 hours in HHS.
Your doctor might also measure your kidney function by checking BUN and creatinine levels, which usually decrease with treatment. Your doctor may also test your blood for levels of specific enzymes such as creatinine kinase and aldolase, which can be elevated due to dehydration. A complete blood count can reveal an elevated white blood cell count, an indicator of stress response or infection, as well as elevated hemoglobin and hematocrit levels, a sign of dehydration.
Finally, through urine analysis, your doctor will look for glycosuria (sugar in urine), ketonuria (ketones in urine), and check the specific gravity of your urine, which could be high in HHS.
Treatment Options for Hyperosmolar Hyperglycemic Syndrome
The treatment of Hyperglycemic Hyperosmolar Syndrome (HHS), a serious condition that can occur in people with diabetes, requires collaboration from a team of medical professionals. You will probably be seen by an endocrinologist, who is a specialist in conditions like diabetes, and an intensive care expert.
When someone comes in with signs of HHS, the first thing doctors do aligns with the principle of Airway, Breathing, Circulation (ABC). This basically means they will first ensure your airway is open, you’re breathing properly, and your blood has the right circulation levels. This is crucial as HHS can cause alterations in mental status due to severe fluid loss and lower levels of blood flow to the brain. If the patient’s brain function score (Glasgow coma score) is less than 8, they will secure the airway.
The main treatment for HHS is aggressive hydration with a type of fluid that contains various essential body salts (isotonic fluid) and the replacement of lost electrolytes. Adults usually receive an initial large amount (bolus) of fluid, which is calculated based on their body weight. This is then followed by steady drips of fluid. For children, the fluid drips are given at about double the usual maintenance rate. Hydrating with this kind of fluid helps because it reduces the amount of hormones produced during HHS that work against insulin. Just this treatment alone can lower sugar levels in the blood.
While the initial blood test might show high levels of potassium, in reality, the body is low on potassium because of an imbalance caused by lack of insulin. So, potassium replacement is also part of the treatment when the blood potassium levels drop to a certain point.
One thing doctors will be cautious about is starting an insulin drip too early. This is because if blood sugar levels drop too quickly, it can cause brain swelling (cerebral edema). So, they’ll aim to keep your blood sugar levels stable to prevent this from happening.
In children, slowly correcting dehydration and replacing lost salts over a more extended period, like 48 hours, can help prevent the chance of brain swelling.
What else can Hyperosmolar Hyperglycemic Syndrome be?
When trying to understand Hyperosmolar Hyperglycemic State (HHS), doctors divide their investigations into two key groups: conditions that change a person’s mental status and conditions that cause high blood sugar levels.
The mental status of a person can be altered due to conditions such as:
- Low blood sugar (Hypoglycemia)
- Low sodium level in blood (Hyponatremia)
- High sodium level in blood (Hypernatremia)
- High ammonia level in blood (Hyperammonemia)
- Severe effects of drugs or alcohol
- Brain issues due to kidney failure (Uremic encephalopathy)
Hyperglycemia, or high blood sugar, can occur with conditions like diabetic ketoacidosis and diabetes insipidus.
Through careful scrutiny of the patient’s history, a thorough physical examination, and the use of additional studies, doctors can quickly determine the diagnosis.
HHS is a potentially deadly medical condition. It’s crucial to have a thorough understanding of its causes and how it presents in patients for the best outcomes in managing the condition.
What to expect with Hyperosmolar Hyperglycemic Syndrome
The overall death rate associated with a hyperglycemic crisis is generally low, and more often than not, it’s caused by the illness that triggered the crisis in the first place. Older patients who experience a severe coma and low blood pressure as a result of the crisis are more likely to have a worse outcome compared to younger patients.
Possible Complications When Diagnosed with Hyperosmolar Hyperglycemic Syndrome
Complications due to the treatment of HHS, a condition that impacts the body’s electrolytes, are quite common. It is important to closely monitor these complications to prevent harmful outcomes. The most frequent problems happen when people have low potassium (hypokalemia) and low blood sugar (hypoglycemia).
Another complication, which is rare but serious, is called cerebral edema, essentially swelling in the brain. This generally happens more in kids than adults and is often caused by a rapid decrease in glucose levels.
Common Complications:
- Low potassium levels (Hypokalemia)
- Low blood sugar (Hypoglycemia)
- Cerebral edema (swelling in the brain)
Preventing Hyperosmolar Hyperglycemic Syndrome
It’s very important for people with diabetes to learn about their condition, including the need to stay well hydrated. This knowledge can help prevent the condition from coming back.
