Published October 15, 2014 at 960 × 720 in Management of Diabetes and Hyperglycemia in Hospitalized Patients. Hyperglycemia results from increased hepatic glucose production and impaired glucose utilization in peripheral tissues. This acute illness protocol is a guideline for healthcare professionals treating the sick neonate, infant, or child who is known to have hyperammonemia. Metabolic crises in infants and children with urea cycle disorders are complex medical emergencies and must be treated as such to avoid death or serious brain injury.
Unlike fats and carbohydrates, protein is not stored in the body but rather exists in a balanced state of anabolism (formation) and catabolism (breakdown). Ammonia also circulates in the body as free ammonia or within glutamine which functions as a temporary “repository” for ammonia.
Apart from arginase deficiency, which usually presents as a subacute or chronic neurologic syndrome with spasticity and cognitive impairment rather than as an acute hyperammonemic syndrome, the other urea cycle defects often present in the newborn period with marked hyperammonemia, hepatomegaly, seizures, and coma secondary to cerebral edema. Hyperammonemic crises in neonates with a urea cycle defect mimic sepsis and can be mistaken as such. Diagnostic and therapeutic interventions in a child with hyperammonemia should be undertaken simultaneously as both the degree and duration of hyperammonemia directly correlate with prognosis. Identify potential precipitant(s) of metabolic decompensation such as infection (presence of fever) or any other physical stressor (e.g.
Plasma ammonia level is a direct index of CNS toxicity and is important to follow for acute management.
The presence of respiratory alkalosis in a sick, hyperammonemic neonate or infant is an indicator of an underlying urea cycle defect since it is an uncommon finding in an ill neonate secondary to other causes. Other amino acids, including glutamate, glycine, asparagine, aspartate and lysine, may be elevated when there is an excess in waste nitrogen burden.
Immediate treatment of hyperammonemia is crucial to prevent neurologic damage and avoid associated morbidity and mortality. Stop all protein intake (but do not withhold protein for longer than 36-48 hours as that can promote breakdown of endogenous proteins and hamper metabolic control). Prepare for probable hemodialysis by contacting the relevant renal and surgical specialists in anticipation of imminent need. There are two main ways to promote ammonia detoxification: hemodialysis and medications that facilitate ammonia excretion.
Hemodialysis is the most effective way of rapidly disposing of excess ammonia and is far superior to other methods of dialysis (hemofiltration, peritoneal dialysis). Ammonia scavenger medications include IV Ammonul® (sodium benzoate and sodium phenylacetate). Sodium should not be provided in supplemental IV fluids when IV Ammonul® is given since this solution contains sufficient amounts of sodium.
Citrulline – used in CPS1 and OTC deficiencies when the child is able to have enteral feeding. A metabolic decompensation in a patient with a UCD is often precipitated by an underlying illness such as an infection or dehydration which results in a state of catabolism. Plasma ammonia levels do not always directly correlate with the presence or severity of clinical signs and symptoms and, thus, monitoring of clinical status and changes in that is crucial. There may be a “rebound” hyperammonemia initially as stored glutamine is metabolized to glutamate and ammonia, and with the efflux of intracellular ammonia into the ‘relatively’ ammonia-depleted blood while on treatment and, thus, it is important to continue closely monitoring plasma ammonia levels until they remain stable in the normal range. Cerebral edema: Oncotic agents such as albumin will increase the overall nitrogen load but may in selected cases be considered. Neurologic status should be closely monitored for signs of CNS toxicity and cerebral edema while the patient is under treatment and in the recovery period. Avoid certain medications, such as valproic acid, as it interferes with urea cycle function and accentuates hyperammonemia.
Laboratory parameters such as plasma amino acids, urine orotic acid, blood gas, and acid-base status will guide the diagnostic workup of a patient with hyperammonemia for which a specific diagnosis is yet unclear. Helpful diagnostic clues: Plasma citrulline levels obtained before 24 hours of age may partially reflect maternal citrulline levels. Once the patient is stabilized and improving, oral diet has been established, and the plasma ammonia level is stable in an acceptable range, oral scavenger medications (sodium benzoate, sodium phenylbutyrate) and oral arginine can be provided in place of their IV forms. The dose of sodium benzoate and sodium phenylbutyrate is determined based on either body weight or body surface area. Once the diagnosis of a specific UCD is established, the Acute Illness Protocol for that disorder should be followed.
Acute fatty liver of pregnancy: Liver failure in late pregnancy, usually from unknown cause. Diabetes mellitus is a condition in which the pancreas no longer produces enough insulin or cells stop responding to the insulin that is produced, so that glucose in the blood cannot be absorbed into the cells of the body. The most common form of diabetes is Type II, It is sometimes called age-onset or adult-onset diabetes, and this form of diabetes occurs most often in people who are overweight and who do not exercise. The causes of diabetes mellitus are unclear, however, there seem to be both hereditary (genetic factors passed on in families) and environmental factors involved. In Type II diabetes, the pancreas may produce enough insulin, however, cells have become resistant to the insulin produced and it may not work as effectively.
Diabetes mellitus is a common chronic disease requiring lifelong behavioral and lifestyle changes. Several blood tests are used to measure blood glucose levels, the primary test for diagnosing diabetes. Random blood glucose test — for a random blood glucose test, blood can be drawn at any time throughout the day, regardless of when the person last ate. Fasting blood glucose test — fasting blood glucose testing involves measuring blood glucose after not eating or drinking for 8 to 12 hours (usually overnight). Oral glucose tolerance test — Oral glucose tolerance testing (OGTT) is the most sensitive test for diagnosing diabetes and pre-diabetes.
Oral glucose tolerance testing is routinely performed at 24 to 28 weeks of pregnancy to screen for gestational diabetes; this requires drinking a 50 gram glucose solution with a blood glucose level drawn one hour later. When diet, exercise and maintaining a healthy weight aren’t enough, you may need the help of medication.
The most widely used form of insulin is synthetic human insulin, which is chemically identical to human insulin but manufactured in a laboratory. Advice patient about the importance of an individualized meal plan in meeting weekly weight loss goals and assist with compliance. Assess patients for cognitive or sensory impairments, which may interfere with the ability to accurately administer insulin.
Review dosage and time of injections in relation to meals, activity, and bedtime based on patients individualized insulin regimen.
Instruct patient in the importance of accuracy of insulin preparation and meal timing to avoid hypoglycemia. Advise patient to assess blood glucose level before strenuous activity and to eat carbohydrate snack before exercising to avoid hypoglycemia. Assess feet and legs for skin temperature, sensation, soft tissues injuries, corns, calluses, dryness, hair distribution, pulses and deep tendon reflexes. Advice patient who smokes to stop smoking or reduce if possible, to reduce vasoconstriction and enhance peripheral flow.


Reduced insulin and excess counterregulatory hormones (glucagon, cortisol, catecholamines and growth hormone) increase lipolysis and protein breakdown (proteolysis), and impair glucose utilization by peripheral tissues. This protocol is only a guideline and should not be used for definitive treatment without metabolic consultation. In a patient with hyperammonemia, it is important to distinguish the underlying cause of high plasma ammonia levels. Among the six enzymes in the cycle, N-acetylglutamate synthase (NAGS), carbamyl phosphate synthetase 1 (CPS1) and ornithine transcarbamylase (OTC) are intramitochondrial enzymes whereas arginase, argininosuccinate synthetase (ASS) and argininosuccinate lyase (ASL) are cytosolic. Protein excess beyond normal bodily requirements comes from either excess dietary protein intake or from protein breakdown through various catabolic processes (stress of the newborn period, infection, dehydration, injury, or surgery).
Consequently, in a urea cycle defect not only does free ammonia rise (hyperammonemia) but glutamine is also elevated.
Typically, OTC and CPS1 deficiencies have the most severe neonatal presentation but citrullinemia and argininosuccinic acidemia (ASA) may also present with severe illness. Hyperammonemia with metabolic acidosis is more likely to be due to an organic acid disorder in which the hyperammonemia is secondary. Glutamine, as an ammonia buffer, reflects the direction of control of the hyperammonemia and, therefore, it is a useful marker for monitoring of ammonia status. Cognitive outcome is inversely related to the number of days of neonatal coma in the urea cycle disorders.
Hemodialysis has the added benefit of removing amino acids such as glutamine and, in that way, disposing of additional waste nitrogen from the body. Sodium benzoate conjugates with glycine to form hippuric acid and sodium phenylacetate conjugates with glutamine to form phenylacetylglutamine; both compounds are excreted in the urine, thereby removing the nitrogen (N) in glycine and glutamine which contribute to the hyperammonemia. This may be controlled with antiemetic medications such as ondansetron, either prior to or during the infusion.
However, do not use if arginase deficiency has been diagnosed, in which the arginine level is elevated.
Citrulline (by mouth, NG, or g-tube) may help pull aspartate into the urea cycle and, thus, increase nitrogen clearance.
Diagnostic investigation and treatment aimed at this underlying precipitant is extremely important to optimize metabolic control of the decompensation and should be undertaken at the time of initial presentation and continued throughout the management phase of hyperammonemia. Clinical decisions on appropriate treatments should be based on the combination of clinical assessment and plasma ammonia levels. On rare occasions it may be necessary to assess the magnitude of glutamine excess in brain tissue by performing brain magnetic resonance spectroscopy (MRS). Mannitol has been used but may not be as effective as hypertonic saline in alleviating cerebral edema due to hyperammonemia. Close monitoring of the intake of calories is an essential part of treatment and monitoring of a patient with a urea cycle defect in crisis. A specific urea cycle defect, an organic acid disorder, or a fatty acid oxidation defect are diagnostic considerations and a thorough diagnostic evaluation should be undertaken simultaneously with treatment of hyperammonemia. The dose should be decided in conjunction with a metabolic physician if the patient does not have an up to date regimen. Consensus statement from a conference for the management of patients with urea cycle disorders. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose.
Type II is considered a milder form of diabetes because of its slow onset (sometimes developing over the course of several years) and because it usually can be controlled with diet and oral medication. Symptoms of Type II diabetes can begin so gradually that a person may not know that he or she has it.
It is best managed with a team approach to empower the client to successfully manage the disease. The body’s primary energy source is glucose, a simple sugar resulting from the digestion of foods containing carbohydrates (sugars and starches).
It is used to monitor blood glucose control in people with known diabetes, but is not normally used to diagnose diabetes. However, the OGTT is not routinely recommended because it is inconvenient compared to a fasting blood glucose test. The person then drinks a 75 gram liquid glucose solution (which tastes very sweet, and is usually cola or orange-flavored). For women who have an abnormally elevated blood glucose level, a second OGTT is performed on another day after drinking a 100 gram glucose solution. These medications, such as repaglinide (Prandin), have effects similar to sulfonylureas, but you’re not as likely to develop low blood sugar. Metformin (Glucophage, Glucophage XR) is the only drug in this class available in the United States. These drugs block the action of enzymes in your digestive tract that break down carbohydrates. These drugs make your body tissues more sensitive to insulin and keep your liver from overproducing glucose.
By combining drugs from different classes, you may be able to control your blood sugar in several different ways. Hyperglycemia causes osmotic diuresis that leads to hypovolemia, decreased glomerular filtration rate, and worsening hyperglycemia.
In urea cycle defects, the high ammonia level is the primary metabolic abnormality and is due to an enzymatic block in ammonia metabolism within the urea cycle.
The most common urea cycle defect is OTC deficiency followed by argininosuccinic acidemia (ASL deficiency) and citrullinemia (ASS deficiency).
Amino acids liberated from excess protein are broken down, releasing nitrogen which circulates in the body as ammonia (NH3). Alanine (Ala) is another amino acid that accumulates as a result of hyperammonemia due to a urea cycle defect.
Of note, all the urea cycle disorders may also present later in childhood with a severe metabolic crisis or a more chronic neurobehavioral course.
Rapid control of the hyperammonemia is crucial in preventing or lessening the degree of mental retardation. Overdoses (3-5x the recommended dose) of IV Ammonul® can lead to agitation, confusion and hyperventilation. If a urea cycle defect is suspected, the following table can help distinguish the different urea cycle disorders based on the corresponding abnormalities in the plasma amino acid levels and urine orotic acid level.
The treatment includes changes in diet, oral medications, and in some cases, daily injections of insulin. The consequences of uncontrolled and untreated Type II diabetes, however, are the just as serious as those for Type I.
In Type I diabetes, the immune system, the body’s defense system against infection, is believed to be triggered by a virus or another microorganism that destroys cells in the pancreas that produce insulin. As part of the team the, the nurse plans, organizes, and coordinates care among the various health disciplines involved; provides care and education and promotes the client’s health and well being.
Glucose from the digested food circulates in the blood as a ready energy source for any cells that need it. The blood glucose level is measured before, and at one, two, and three hours after drinking the solution.


Everyone with type 1 diabetes and some people with type 2 diabetes must take insulin every day to replace what their pancreas is unable to produce. One of its chief failings is that it doesn’t mimic the way natural insulin is secreted. It works by inhibiting the production and release of glucose from your liver, which means you need less insulin to transport blood sugar into your cells. That means sugar is absorbed into your bloodstream more slowly, which helps prevent the rapid rise in blood sugar that usually occurs right after a meal. Side effects of thiazolidinediones, such as rosiglitazone (Avandia) and pioglitazone hydrochloride (Actos), include swelling, weight gain and fatigue. At the cellular level, increased blood glucose levels result in mitochondrial injury by generating reactive oxygen species, and endothelial dysfunction by inhibiting nitric oxide production. Secondary hyperammonemia can be due to metabolic defects such as organic acid disorders and fatty acid oxidation disorders, drugs or other metabolites that may interfere with urea cycle function, or severe liver disease. All urea cycle defects are autosomal recessive in inheritance except OTC deficiency which is X-linked. Ammonia is then converted into urea via the urea cycle and disposed of in the urine.  An enzymatic block in the urea cycle results in the accumulation of excess ammonia which has toxic effects most severe in the central nervous system causing cerebral edema. These two amino acid elevations (glutamine, alanine) may precede hyperammonemia and the onset of clinical symptoms and can serve as useful biochemical markers of decompensation in a patient with a urea cycle defect.
Central venous catheters should be placed in a critically ill patient in hyperammonemic crisis in anticipation of the potential for hemodialysis and the appropriate nephrology and surgical specialists should be alerted in advance for this potential need. Supplemental calories are added from a non-nitrogenous formula with vitamins and minerals (Ross formula Pro-Phree® or equivalent). Please note that as the patient’s condition improves and anabolic homeostasis is restored, it may be necessary to rapidly eliminate or reduce the rate of the insulin infusion as hypoglycemia may develop. This form is also called noninsulin-dependent diabetes, a term that is somewhat misleading.
Other symptoms may include sudden weight loss, slow wound healing, urinary tract infections, gum disease, or blurred vision. Insulin is a hormone or chemical produced by cells in the pancreas, an organ located behind the stomach. Unfortunately, insulin can’t be taken in pill form because enzymes in your stomach break it down so that it becomes ineffective. But newer types of insulin, known as insulin analogs, more closely resemble the way natural insulin acts in your body. Second-generation sulfonylureas such as glipizide (Glucotrol, Glucotrol XL), glyburide (DiaBeta, Glynase PresTab, Micronase) and glimepiride (Amaryl) are prescribed most often. One advantage of metformin is that is tends to cause less weight gain than do other diabetes medications. Most doctors prescribe two drugs in combination, although sometimes three drugs may be prescribed.
Hyperglycemia increases levels of pro-inflammatory cytokines such as TNF-? and IL-6 leading to immune system dysfunction. Laboratory studies can help distinguish the underlying primary defect and cause of hyperammonemia and guide appropriate treatment. The decision to hemodialyze is critical in preventing or minimizing irreversible CNS damage; when in doubt in the face of a markedly elevated ammonia level, the decision should be to hemodialyze as soon as possible. High IV dextrose solutions should not be decreased or stopped in the face of hyperglycemia. Many people with Type II diabetes can control the condition with diet and oral medications, however, insulin injections are sometimes necessary if treatment with diet and oral medication is not working.
It is not unusual for Type II diabetes to be detected while a patient is seeing a doctor about another health concern that is actually being caused by the yet undiagnosed diabetes. Insulin bonds to a receptor site on the outside of cell and acts like a key to open a doorway into the cell through which glucose can enter. For that reason, many people inject themselves with insulin using a syringe or an insulin pen injector,a device that looks like a pen, except the cartridge is filled with insulin. The most common side effect of sulfonylureas is low blood sugar, especially during the first four months of therapy. Possible side effects include a metallic taste in your mouth, loss of appetite, nausea or vomiting, abdominal bloating, or pain, gas and diarrhea.
Although safe and effective, alpha-glucosidase inhibitors can cause abdominal bloating, gas and diarrhea.
The thiazolidinedione troglitzeone (Rezulin) was taken off the market in March 2000 because it caused liver failure. Newer medications, such as Glucovance, which contains both glyburide and metformin, combine different oral drugs in a single tablet. These changes can eventually lead to increased risk of infection, impaired wound healing, multiple organ failure, prolonged hospital stay and death. Some of the glucose can be converted to concentrated energy sources like glycogen or fatty acids and saved for later use. Others may use an insulin pump, which provides a continuous supply of insulin, eliminating the need for daily shots. You’re at much greater risk of low blood sugar if you have impaired liver or kidney function. These effects usually decrease over time and are less likely to occur if you take the medication with food. If your doctor prescribes these drugs, it’s important to have your liver checked every two months during the first year of therapy. Wide swings in glucose levels are not ideal and so plasma glucose should be kept within the above as best as possible. When there is not enough insulin produced or when the doorway no longer recognizes the insulin key, glucose stays in the blood rather entering the cells. A rare but serious side effect is lactic acidosis, which results when lactic acid builds up in your body.
Contact your doctor immediately if you experience any of the signs and symptoms of liver damage, such as nausea and vomiting, abdominal pain, loss of appetite, dark urine, or yellowing of your skin and the whites of your eyes (jaundice).
Early diagnosis of AFLP and prompt delivery dramatically improve the outcome and the once-bleak outlook. These may not always be related to diabetes medications, but your doctor will need to investigate all possible causes. Lactic acidosis is especially likely to occur if you mix this medication with alcohol or have impaired kidney function. This abnormality is a deficiency of the enzyme long-chain-3-hydroxyacyl-CoA dehydrogenease (LCHAD). The mother (and father) have 50% of normal LCHAD activity and the fetus has no LCHAD activity. The metabolic disease in the baby's liver apparently causes the fatty liver disease in the mother.



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Comments

  1. 29.05.2015 at 23:31:39


    Can monitor their own levels kidney diseases often cause.

    Author: PIONERKA
  2. 29.05.2015 at 21:53:44


    Emergency department hours must be equal to or higher than 126 capillary.

    Author: ELSAN