Rare Hormonal Disorders

Hormones are the chemical messengers secreted from endocrine glands in your body to regulate proper functioning of it. The unique thing about hormones is that they are secreted directly into the bloodstream, but since they act only on specific target organs, no other body part is affected.^[1] However, there are certain conditions which affect the secretion of hormones. This article deals with such rare hormonal disorders.

The hormones are secreted by their respective endocrine glands only when the brain signals them to do so. When the endocrine glands do not function properly, the person experiences hormonal disorders that cause problem with the target organ that they are concerned with.^[2] Knowing about the rare hormonal disorders helps to deal with them in a better way.

Before you start discovering about rare hormonal disorders, you need to know about the major hormones in your body and their functions. So, let’s look at them first:

• The Pituitary Gland – The pituitary gland secretes majority of hormones, including tropic hormones which trigger other endocrine glands to secrete their own hormones. Almost all body factors are influenced by the pituitary hormones, which makes it the most important endocrine gland.^[3]
• Thyroid Gland – The thyroid gland functions to secrete thyroxine and calcitonin, which control body metabolism and calcium levels of the blood and the bones respectively.^[4]
• Parathyroid Gland – The parathyroid gland secretes parathyroid hormone which often acts as the opposite of calcitonin. It inhibits calcium production when the level of calcium in the body is high and enhances it when calcium is low.^[5]
• Adrenal Gland – Adrenal glands secrete adrenaline and cortisol. Its basic function is to prepare the body to cope with various situations by preparing it either to fight or flight.^[6]
• Pancreas – The pancreas secretes insulin, glucagon and somatostatin which control the blood sugar levels in our body.^[7]
• Gonads or Sex Glands – Gonads, or the reproductive parts secrete different hormones in both the sexes. In males, the testes produce testosterone for the production of sperms. In females, the ovaries secrete estrogen and progesterone to control the female reproductive cycle.^[8]

Causes of rare hormonal disorders can related to certain conditions. There are three conditions under which the endocrine glands fail to function properly, resulting in problems of the body.

Generally there are three conditions which cause these problems –

Hyposecretion. Hyposecretion of the hormones of the endocrine glands which generally cause under secretion of the hormone required. Since the hormone produced falls short of the needs required, it causes problems. Example – Hypoglycemia where the blood sugar level becomes too low.

Hypersecretion. Hypersecretion of the hormones of the endocrine glands which generally cause excessive secretion of the hormones required. Since the hormones are in abundance even after the requirements are met, the excess hormones result in causing adverse effect on the glands. Example – Hyperglycemia where the blood sugar level soars too high, this condition is commonly known as diabetes.

Tumors. Tumors of the endocrine glands are another cause of the rare hormonal disorders. The tumors can be both benign and malignant. Benign tumors are the ones which cannot invade the neighboring cells and hence are classified to the non-cancerous category of tumors. Malignant tumors are generally cancerous since they can invade the neighboring cells.^[9]

Rare hormonal disorders related to pituitary gland include the following

• Central Diabetes Insipidus. Vasopressin produced by the pituitary gland is responsible for increasing blood volume and decreasing the urine volume. Under any problem of the pituitary, if the production of vasopressin drops the person suffers from high urine volume. Desmopressin can be used to treat this condition.
• Growth Hormone Disorders. Since the pituitary controls the growth hormones, excessive secretion of the same may result in excessive growth called gigantism, and low release of the hormone may cause stunted growth called dwarfism. This is one of the important rare hormonal disorders.
• Hypopituitarism and Hyperpituitarism. This are very rare hormonal disorders but where one or more hormones of the pituitary are not secreted properly. In hypopituitarism, there is under secretion of the hormones and in hyperpituitarism there is excessive secretion of the same. Either way, the reflection of them on the target organs is very undesirable.
• Pituitary Adenoma. These are tumors in the pituitary, most of them being benign and non-invasive. Only a very small percentage of them are malignant. The growth rate of such tumors is enhanced by the hormone progesterone which is in turn enhanced in its production by tropic hormones of pituitary.^[10]

Rare hormonal disorders related to thyroid gland include the following

• Hashimoto’s Disease. It is caused due to an undesirable reaction of the immune system towards the thyroid gland. Since the disease develops slowly, it may take months to get diagnosed. Several symptoms of this disease include weight gain, enlargement of the neck, irregular periods etc.
• Myxedema. This is one of the rare hormonal disorders. Myxedema coma is caused due to the under secretion of the thyroxine hormone, for long course of time. Myxedema generally results in an undesirable appearance, making the skin coarse, thick and swollen. However in myxedema coma, there is threat to the patient’s life due to heart failure, cerebrovascular disease, trauma etc. Most of the time, such patients do not live long unless diagnosed really early.
• Pendred Syndrome. It is caused due to the enlargement of the thyroid gland, resulting in goiter and hearing loss. Generally, it’s a hereditary factor in most of the cases. This rare hormonal disorder is responsible for 10% of the world’s deaf population.^[11]

Rare hormonal disorders related to adrenal gland include the following

Addison’s Disease. Addison’s disease results due to under or almost no secretion of the adrenal hormone cortisol. The symptoms begin to appear slowly, one by one, beginning with weight loss and pigmentation of the skin. One can also have vomiting, fainting without any cause and back pain as other symptoms. Generally, this rare hormonal disorder is managed with hydrocortisone hormone.

Cushing’s Syndrome. This is generally experienced when there is excessive production of the cortisol hormone by the adrenal glands. This is generally caused due to formation of tumors in the pituitary as well, as a side effect and is also one of the rare hormonal disorders. The symptoms include extreme obesity around the central body part, hump formation on the back of the neck and the face becoming moon like. Patients also complain of excess sweating.^[12]

Here are some of the rare hormonal disorders related to the gonads.

• Kallmann Syndrome. Kallmann syndrome occurs due to the failure of the body hormones to attain puberty due to under secretion of the gonadotropic hormone. It is a very rare hormone disorder but the majority of the cases are reported by men.
• Gonadal Dysgenesis. This causes the slow loss of germ cells, resulting in underdeveloped gonads which ultimately lead to infertility and infant like looks. It can be the side effect of other disorders of the gonads or may be caused due to any endocrinal disruption.^[13]

The treatment of rare hormonal disorders mainly includes symptomatic management. Most conditions can be kept in control with medications to replace the hormones produced in abundance, or add more hormones in the cases of under secretion. It is necessary to get yourself evaluated medically so as to begin appropriate and timely treatment. Following medical advice can ensure proper management of the rare hormonal disorders.

Hormonal problems or any of the rare hormonal disorders can be diagnosed only if the symptoms or relevant history are noted in time and further evaluation is done. Here are some tips to keep your endocrine system and hormone function healthy.

Look at Your Family History. Try to keep an eye and dig up your family history since many endocrine disorders run in families. If someone in your family suffered from thyroid disorder or any of the rare hormonal disorders, consult your doctor to evaluate your risk. It will help to follow up regularly and plan a diet and lifestyle that may help prevent or manage the condition.

Healthy Diet. Having a healthy diet will help in every way to keep the hormone system function well. It not only helps to maintain an ideal weight but also prevents sudden fluctuations in weight and also in chemical secretions of the endocrine glands. Incorporate proteins, vitamins, iron, minerals etc. in proper amounts in your diet. Having a fruit every day, green vegetables and whole grains can help you achieve your goals. Never skip meals, in fact, have your favorite meals but in smaller amounts to keep those calories in check.

Reduce Stress Levels. It is important to reduce stress levels since it results in excess secretion of many hormones which increases your risk of encountering any hormone related disorders. You can devote fifteen minutes to mediation and exercise every day to help you relieve stress in releasing endorphins in your body.^[14]

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