Quality of Life and Other Outcomes in Children Treated for Cushing Syndrome

Abstract

Journal of Clinical Endocrinology and Metabolism, 05/16/2013  Review Article  Clinical Article

Keil MF – Cushing syndrome (CS) in children is associated with residual impairment in measures of health–related quality of life, even after successful resolution of hypercortisolemia, highlighting the need for early identification of morbidities and improvements in long–term management of these patients.

A review of the literature identifies significant morbidities associated with CS of pediatric onset, which must not be treated in isolation. CS affects children and adolescents in many ways that are different than adults.

Post–treatment challenges for the child or adolescent treated for CS include: optimize growth and pubertal development, normalize body composition, and promote psychological health and cognitive maturation. All these factors impact health–related quality of life, which is an important outcome measure to assess the burden of disease as well as the effect of treatment.

Get this article here

Endoscopic transsphenoidal pituitary surgery: a good and safe primary treatment option for Cushings disease, even in case of macroadenomas or invasive adenomas.

Abstract – European Journal of Endocrinology, 06/24/2013  Clinical Article

Wagenmakers MA et al. – This study aims to gain insight in the role of endoscopic TS as a primary treatment option for CD, especially in patients with MRI negative CD and (invasive) macroadenomas.

The data suggest that in patients with non–invasive and invasive macroadenomas the endoscopic technique of TS should be the technique of choice as remission rates seem to be higher than remission rates reported for microscopic TS, although no comparative study has been performed.

Get this article here.

Have You Gone to the ER With an Adrenal Crisis?

This Survey is to gather information for the Adrenal Insufficiency Awareness Organization’s grant project to create educational materials for ER personnel.

The survey is for those of you who have been to the ER during an impending or full blown Adrenal Crisis. (you may fill it out for a child or yourself)

Your help is appreciated!

At the end of the survey you will have the chance to enter for a chance to win an Adrenal Insufficiency Awareness Pin.

https://www.surveymonkey.com/s/ERcare

Adrenal Glands

adrenal-glandsAnatomy of the adrenal glands:

Adrenal glands, which are also called suprarenal glands, are small, triangular glands located on top of both kidneys. An adrenal gland is made of two parts: the outer region is called the adrenal cortex and the inner region is called the adrenal medulla.

Function of the adrenal glands:

The adrenal glands work interactively with the hypothalamus and pituitary gland in the following process:

  • the hypothalamus produces corticotropin-releasing hormones, which stimulate the pituitary gland.
  • the pituitary gland, in turn, produces corticotropin hormones, which stimulate the adrenal glands to produce corticosteroid hormones.

Both parts of the adrenal glands — the adrenal cortex and the adrenal medulla — perform very separate functions.

What is the adrenal cortex?

The adrenal cortex, the outer portion of the adrenal gland, secretes hormones that have an effect on the body’s metabolism, on chemicals in the blood, and on certain body characteristics. The adrenal cortex secretes corticosteroids and other hormones directly into the bloodstream. The hormones produced by the adrenal cortex include:

  • corticosteroid hormones
    • hydrocortisone hormone – this hormone, also known as cortisol, controls the body’s use of fats, proteins, and carbohydrates.
    • corticosterone – this hormone, together with hydrocortisone hormones, suppresses inflammatory reactions in the body and also affects the immune system.
  • aldosterone hormone – this hormone inhibits the level of sodium excreted into the urine, maintaining blood volume and blood pressure.
  • androgenic steroids (androgen hormones) – these hormones have minimal effect on the development of male characteristics.

What is the adrenal medulla?

The adrenal medulla, the inner part of the adrenal gland, is not essential to life, but helps a person in coping with physical and emotional stress. The adrenal medulla secretes the following hormones:

  • epinephrine (also called adrenaline) – this hormone increases the heart rate and force of heart contractions, facilitates blood flow to the muscles and brain, causes relaxation of smooth muscles, helps with conversion of glycogen to glucose in the liver, and other activities.
  • norepinephrine (also called noradrenaline) – this hormone has little effect on smooth muscle, metabolic processes, and cardiac output, but has strong vasoconstrictive effects, thus increasing blood pressure.

From: University of Maryland Center for Diabetes and Endocrinology

Classifying hypertension

HYPERTENSION is classified into two categories according to its cause: essential and secondary.

The vast majority of patients have essential or primary hypertension, while only about 5-10% of patients have secondary hypertension, which are mainly caused by kidney and hormonal conditions like renal artery stenosis, hyperthyroidism, Cushing’s syndrome, and even pregnancy, among others.

The exact cause of essential hypertension is still unknown, although it is certainly the result of a combination of factors, including increasing age, having relatives with high blood pressure (ie family history), a sedentary lifestyle, a poor diet with too much salt, drinking too much alcohol, smoking and too much stress.

Says Malaysian Society of Hypertension president and Universiti Malaya Department of Primary Care Medicine senior consultant Prof Datin Dr Chia Yook Chin: “Each factor increases blood pressure by just a little, but when you add them all together little by little, it raises it by quite a lot.”

Despite not knowing the root cause of hypertension, it has been established that there is overstimulation of the sympathetic nerves in people with this condition.

This in turn increases the secretion of certain hormones involved in the regulation of sodium and fluids in the body, called renin, angiotensin, and aldosterone.

The amount of salt and water in our body affects our blood pressure – the more salt and water present, the higher our blood pressure.

These two elements are regulated by our kidneys through the three hormones mentioned above, which are produced by the adrenal glands located on top of the kidneys.

The overstimulation of the sympathetic nerves also results in increased vascular tone, which causes our arteries to become constricted, thus, also raising blood pressure.

From The Star