Cushing’s syndrome is a disorder resulting from a prolonged exposure to high levels of glucocorticoids. It is subdivided into two groups based on the cause of the hypercortisolism, either adrenocorticotropic (ACTH) -dependent or –independent.1 ACTH-independent is usually caused by the use of exogenous glucocorticoid therapy 2 or from autonomous cortisol secretion by the adrenal glands.1 The most common cause of endogenous ACTH-independent Cushing’s syndrome is caused by autonomous adrenal overproduction of cortisol 2; this is usually due to a solitary adrenocortical adenoma3. Exogenous glucocorticoid therapy can also be classed as ACTH-independent, as it doesn’t require ACTH stimulation, which is similar to the adrenal gland autonomously …show more content…
ACTH-dependent Cushing’s syndrome in children is commonly caused by Cushing’s disease12.
Weight gain is one of the most common clinical features and results in accumulation of adipose tissue in the trunk, facial and cervical area. Supraclavicular fat around the neck and face is described as a ‘moon face’ and a dorsocervical fat pad is described as a ‘buffalo hump’15. In children weight gain is associated with a negative effect on growth rate and usually results in growth retardation4-. This is caused by either a removal of the pituitary gland due to a tumour16,17 or the interference of glucocorticoids with the action of growth hormone in the development of the long bones and the epiphyseal
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The first is through the direct impairment of the insulin receptor signalling pathway and the second is by modifying the lipid and protein metabolism29. The glucocorticoids interfere with the receptor pathway by causing a post-receptor defect30. This causes a decrease in the expression of molecules (such as insulin receptor substrate and protein kinase B), that are required for the functioning of the intracellular insulin receptor. This action then causes a decreased ability for the glucose transport (GLUT4) to migrate to the cell surface with the consequence of reduced glucose uptake,30,31 thus leaving high levels of glucose in the blood leading to hyperglycaemia. Glucocorticoids also influence the lipid and protein metabolism to increase lipolysis and, increase protein degradation and decrease protein synthesise, resulting in elevated levels of fatty acids and amino acids which increase gluconeogenesis and interfere with the activation of insulin
ACHONDROPLASIA is known as being undersized, or less than 50in. in height. Having short limbs, a normal sized trunk, large head with a depressed nasal bridge and small face. This is a result of a disease in the thyroid gland. It can also be caused by Down syndrome or absorption, a cartilaginous tissue during the fetal stage. Hypochondroplasia, a mild form of dwarfism. Spinal tuberculosis and the deficiency of the pituitary gland secretions. Treatment with thyroxin or thyroid extract early in childhood results in normal growth and development. Somatrophin, also known as the human growth hormone is secreted by the anterior pituitary. Respiratory problems start to occur in infants. Symptoms of problems include snoring and sleeping with neck in a hyperextended condition. The limbs have rhizometic shortening. The legs are straight in infantry but when a child. He begins walking they develop a knock-knee position. When the child continues to walk legs begin to have a bowed-leg look. Occasionally, these curvatures are fixed. As the child continues to walk the kyphosis disappears and the back assumes a lordotic posture. If a delay in child’s walking occurs, the spine should be monitored closely for signs of gibbous formation. In infancy, hypercephalus can occur. Infants head circumference should be monitored close . Monthly checks of head circumference must be monitored. Radiologic studies are indicated if head circumference raises to disproportionately, or if symptoms of hydrocephalus. Child’s pediatrician should have a copy of head circumference curves for children with achondroplasia. Radiologic procedures for dwarfism include head ultrasound, C-T scan, or MRI of the head. If intervention is necessary, a ventriculoperitoneal shunt is placed relieving the pressure. Infants should also be monitored for foramen magnum compression. It is the opening at the base of the skull in which the brain stem and cervical spinal cord exit. When you have achondroplasia the foramen magnum is compressing the brain stem and spinal cord. Symptoms of narrowing include apnea the cessation of breathing and cervical myleopathy. C-T scans and MRI scans are done to examine the size of the infectious foramen magnum. A neurosurgical procedure called a foramen magnum decompression is executed to alarge foramen and alleviate further symptoms. Adolescents are at risk of getting lumbosacral spinal stenosis. The lumber spinal cord or nerve roots become compressed producing nerosurgical symptoms. Initial symptoms including weakness, tingling, and pain of the legs. Pain usually alleviated by assuming a squatting position.
The hypothalamus releases corticotrophin (CHR), which is a chemical messenger. CHR stimulates the pituitary gland to release adrenal corticotrophin (ACTH). ACTH, which is released into the blood, then signals the adrenal gland. When the adrenal gland receives ACTH, it is the chemical signal for cortisol production. In a normal system, cortisol (an anti-inflammatory) production would stop after being released, but if there is a break down in the process, cortisol production continues, leading to Cushing’s disease....
Our body obtains the energy by digesting the carbohydrates into glucose. Volumes of glucose are required by the body to create ATP. ATP is short for 'Adenosine Triphosphate ' and is an energy carrier. When we consume too many carbohydrates our body produces a lot of glucose and as a result blood glucose levels rise and sometimes they may rise over the normal range of blood glucose concentration. To bring it back within the healthy range, the homeostatic system of blood glucose regulation is used. The blood flows through the pancreas where the beta cells, receptors, detect the high blood glucose level. To counteract this stimuli beta cells alert the control centre, which are also the beta cells located in the islets of Langerhans in the pancreas. The secretion of insulin has to be done quickly but can only be carried out when insulin gene is switched on. Turning on the insulin gene switch can take 30 minutes to an hour therefore, the production of insulin by beta cells are done in advance and are packaged in vesicles right until blood glucose rises. Glucose comes into the beta cell to trigger the vesicle that contains the insulin to move towards the plasma membrane and fuse. This releases the insulin into the bloodstream where they are distributed throughout the body and only affect specific target cells. The receptor, a protein, on the target cell’s plasma membrane recognises and connects
The pathophysiology of diabetes mellitus in is related to the insulin hormone. Insulin is secreted by cells in the pancreas and is responsible for regulating the level of glucose in the bloodstream. It also aids the body in breaking down the glucose to be used as energy. When someone suffers from diabetes, however, the body does not break down the glucose in the blood as a result of abnormal insulin metabolism. When there are elevated levels of glucose in the blood, it is known as hyperglycemia. If the levels continue to remain high over an extended period of time, damage can be done to the kidneys, cardiovascular systems; you can get eye disorders, or even cause nerve damage. When the glucose levels are low in one’s body, it is called hypoglycemia. A person begins to feel very jittery, and possibly dizzy. If that occurs over a period of time, the person can possibly faint. Diabetes mellitus occurs in three different forms - type 1, type 2, and gestational.
CAH is an autosomal recessive genetic disorder and males and females are affected in equal numbers. Chromosome 6 is where the group of genes lie that causes the most common forms of CAH. Adrenocorticotropic hormones (ACTH) are released by the anterior lobe in the pituitary gland. Its role is to act on cells of the adrenal cortex which then synthesizes corticosteroids and cortisol. However, those with CAH have insufficient amounts of the enzyme 21-hydroxylase, needed to convert 17-hydroxyprogesterone (17-OHP) into cortisol.
Addison’s disease is a disorder of the endocrine system. It is a hormonal disorder that can strike anyone, any gender at any age. Addison’s disease has also been called Adrenal Insufficiency (hypocortisolism) because the root of the disease is in the adrenal gland not producing enough of the hormone cortisol, or sometimes not enough of the hormone aldosterone to satisfy the body’s needs.
Cushing syndrome was named after Harvey Cushing in 1912. “Harvey Cushing first described the symptom complex of obesity, diabetes, hirsutism, and adrenal hyperplasia. He gave a vivid description of his first patient who was 23 years old and presented with the “most extraordinary appearance”” (“Osler’s phenomenon”: misdiagnosing Cushing’s syndrome; Postgraduate Medical Journal; October 2003). Although, Sir William Osier was the first to describe the symptoms of Cushing’s, but diagnosed his patient with myxoedema by mistake. Unfortunately many people were misdiagnosed, therefore, leading to mortality. In 1933, Cushing performed his first neurosurgery on his patient, which did not gain acceptance until much later. “Adrenal surgery was for many years the treatment of hypercorticism but prior to the availability of glucocorticosteroids substitution an extremely perilous undertaking” (Cushing’s syndrome: Historical Aspects, Lindholm J., October 2000).
...ll gland at the base of the brain, under the control of the hypothalamus, can signal the adrenal glands to increase the production of stress hormones such as cortical and adrenaline even further.
Obesity is a medical condition in which excess body fat has accumulated to the extent that it may have an adverse effect on health, leading to reduced life expectancy and/or increased health problems. Obesity has been a health problem ever since infectious disease began in the first half of the 20th Century. A person with obesity is not the only person who is affected by their disease. In the case of childhood obesity, it can affect the parents because they might be the cause of the child’s issues. It can also lead to many different health problems such as cancer, diabetes, heart disease, and respiratory problems, and it can also even lead to death.
Other hormones (glucagons, epinephrine, growth hormone, and cortisol) work to oppose the effects of insulin and are often referred to as counterregulatory hormones. These hormones work to increase blood glucose levels by stimulating glucose production and output by the liver and by decreasing the movement of glucose into the cells. Insulin and the these counterregulatory hormones provide a sustained but regulated release of glucose for energy during food intake and periods of fasting and usually maintain blood glucose levels within the normal range. An abnormal production of any or all of these hormones may be present in diabetes.
The thyroid gland is found in the front of the neck and produces two main hormones. The hormones are called thuroxine (T4) and Triiodothyronine (T3). Together these hormones regulate the body’s metabolism by increasing energy use in cells, regulate growth and development, help to maintain body temperature and aid in oxygen consumption. These two hormones are regulated by hormones produced by the hypothalamus and pituitary gland. The hypothalamus senses changes in body’s metabolic rate and releases a hormone known as thyropin-releasing hormone (TRH). This hormone then flows through connecting vessels to the pituitary gland which signals it to release another hormone. This hormone is known as thyroid-stimulating hormone (TSH). TSH then makes its way to the bloodstream until it reaches the thyroid where it is then signaled to activate T3 and T4 production [1]. This mechanism is controlled by a negative feedback loop meaning that when there is a sufficient amount of thyroid hormones in the blood stream, this will signal back to stop production of thyroid stimulating hormones. Complications occur when the thyroid hormones keep increasing even though there is already a sufficient amount of T3 and T4 in the blood stream. This process of over expression of thryroid hormones is known as hyperthyroidism. Hyperthyroidism is a general term that includes any disease that has a consequence of an overabundance of thyroid hormones. Hyperthyroidism is a general term but there are many variant diseases that are in the hyperthyroidism category. These diseases include diffuse toxic goiter, Basedow’s disease, thyrotoxicosis, Parry’s and Graves’ disease.
A. One condition is known as hyperglycemia, which means that the blood glucose gets too
Type 1 diabetes has a genetic onset that often occurs in adolescence (Porth, 2005). It is an autoimmune disease in which the insulin-producing beta cells within the liver are destroyed (Dorman, 1993). This causes a deficiency in insulin secretion, which ultimately leads to high blood glucose levels, also referred to as hyperglycemia (Guthrie & Guthrie, 2004). The mechanism for insulin deficiency leading to hyperglycemia is described in more detail in the following section and in Figure 1.
According to the 1999-2000 National Health and Nutrition Examination survey says that, 16 percent of children around the ages of 6-19 years old are at risk of being obese. Obesity has increased in the last twenty years now. Obesity is like other disease, it impairs the normal body functioning. Children who are overweight have excess fat tissue that caus...
The pancreas uses these two hormones in order to monitor blood glucose levels. After a meal, blood glucose usually rises. This is when insulin secretion will start (Nussey S, Whitehead S. “Endocrinology: An Integrated Approach”). Consequently, blood glucose decrease to the normal range. This is how insulin maintains blood levels when is high. However, when blood level falls below normal range, glucagon comes into play. Low blood glucose occurs usually when hungry and during exercise. This will then triggers glucagon secretion. When blood level falls, the body goes into imbalance. Hence is why in order to maintain homeostasis glucagon is crucial. The body will tell the pancreas to increase more glucose and the pancreas will secrete glucagon by taking glycogen from the liver to produce glucose. The glucose will produce energy and will make blood glucose concentration increase (Homeostasis of Insulin and Glucose, Abpischools.org). When the pancreas cannot maintain homeostasis, many problems will arise in the body. When the pancreas fails to produce insulin, type 1 and 2 diabetes can occur. For those with type 1 diabetes, insulin injections will be needed in order to regulate blood glucose level, otherwise, glucose levels will be out of control. For type 2 diabetes, they are not insulin dependent like type 1, however, the body does not create enough in the body. When blood glucose