Hypothalamic Diseases
Hypopituitarism
Encephalomalacia
Pituitary Neoplasms
Pituitary Gland
Pituitary Diseases
Subarachnoid Hemorrhage
Brain Injuries
Treatment of gonadotropin dependent precocious puberty due to hypothalamic hamartoma with gonadotropin releasing hormone agonist depot. (1/138)
The gonadotropin releasing hormone (GnRH) secreting hypothalamic hamartoma (HH) is a congenital malformation consisting of a heterotopic mass of nervous tissue that contains GnRH neurosecretory neurons attached to the tuber cinereum or the floor of the third ventricle. HH is a well recognised cause of gonadotropin dependent precocious puberty (GDPP). Long term data are presented on eight children (five boys and three girls) with GDPP due to HH. Physical signs of puberty were observed before 2 years of age in all patients. At presentation with sexual precocity, the mean height standard deviation (SD) for chronological age was +1.60 (1.27) and the mean height SD for bone age was -0.92 (1.77). Neurological symptoms were absent at presentation and follow up. The hamartoma diameter ranged from 5 to 18 mm and did not change in six patients who had magnetic resonance imaging follow up. All patients were treated clinically with GnRH agonists (GnRH-a). The duration of treatment varied from 2.66 to 8.41 years. Seven of the eight children had satisfactory responses to treatment, shown by regression of pubertal signs, suppression of hormonal levels, and improvement of height SD for bone age and predicted height. One patient had a severe local reaction to GnRH-a with failure of hormonal suppression and progression of pubertal signs. It seems that HH is benign and that GnRH-a treatment provides satisfactory and safe control for most children with GDPP due to HH. (+info)Analysis of urinary nitric oxide metabolites in healthy subjects. (2/138)
Nitric oxide (NO) has divergent actions under physiological and pathological conditions. NO is rapidly decomposed to nitrite (NO2-) and nitrate (NO3-). Since these metabolites are stable, they are good indices of NO production under various conditions. In the present study, we measured NO2- and NO3- concentrations in the urine collected from 62 hospital controls and 504 healthy subjects by means of a new HPLC system combined with Griess reaction. NOx was the sum of NO2- and NO3-. There was no considerable inter-day variation in urinary NO metabolite levels, and there was close correlation between NO2-, NO3- and NOx values in spot urine obtained in the early morning and those in 24-h stored urine in hospital controls. Urinary NO metabolite levels, which were corrected by creatinine (Cr) excretion and expressed on a logarithmic scale, showed normal distribution and were independent of sex and age in healthy subjects. The normal ranges of urinary NO2-, NO3- and NOx levels were estimated as 17-72 micromol/g Cr, 1,023-2,818 pmol/g Cr, and 1,071-2,951 micromol/g Cr, respectively. We also found that urinary NO metabolite levels were lower than normal range in patients with various diseases. (+info)Pallister-Hall syndrome: clinical and MR features. (3/138)
A 4-month-old boy with polydactyly and bifid epiglottis was found to have a large sellar and suprasellar mass. When the diagnosis of Pallister-Hall syndrome was made, conservative management was elected. When the patient was 2 years old, the tumor had grown proportionally with the patient, and he was developing appropriately. Although rare, this entity is important to recognize not only for clinical diagnosis but also for appropriate management and genetic counseling. (+info)GH deficiency in adults: an epidemiological approach. (4/138)
OBJECTIVE: The prevalence of adult onset GH deficiency (GH-D) is poorly documented. Epidemiological data are now required to estimate the financial cost of GH treatment in adults. The aim of the present study was to estimate the prevalence of GH-D, from a cohort of 1652 adult patients with hypothalamo-pituitary diseases. DESIGN: The hormonal status of all patients presenting with pituitary diseaseand observed during the year 1994 in 15 endocrine units was retrospectively analyzed, irrespective of the date of disease onset, of the nature and date of pituitary investigations, and whether or not they included specific testing of the GH axis. Of the whole population of 1652 patients, a selected group (RG2) was chosen after exclusion of patients with active acromegaly (n=1414). RESULTS: GH stimulation tests had been performed in 549 patients of the RG2 group and a documented GH-D was found in 301. A relationship between the value of the GH peak and the number of pituitary deficits was evaluated. For instance, it was shown that 93% of patients with three deficits had GH-D. These results constituted the basis for estimating the number of GH-D in the group of untested patients. The number of GH-D deduced from the number of established GH-D (n=301) and from the number of GH-D hypothesized from other pituitary deficits (n=406) was 707 cases. Prevalence and annual incidence were calculated from data recorded in a referral center with a well-defined catchment area, Marseilles (Bouches du Rhone department). We projected a prevalence of 2638 for France and an annual incidence of 12 GH-D per million of the adult population. (+info)Accelerated puberty and late-onset hypothalamic hypogonadism in female transgenic skinny mice overexpressing leptin. (5/138)
Excess or loss of body fat can be associated with infertility, suggesting that adequate fat mass is essential for proper reproductive function. Leptin is an adipocyte-derived hormone that is involved in the regulation of food intake and energy expenditure, and its synthesis and secretion are markedly increased in obesity. Short-term administration of leptin accelerates the onset of puberty in normal mice and corrects the sterility of leptin-deficient ob/ob mice. These findings suggest a role for leptin as an endocrine signal between fat depots and the reproductive axis, but the effect of hyperleptinemia on the initiation and maintenance of reproductive function has not been elucidated. To address this issue, we examined the reproductive phenotypes of female transgenic skinny mice with elevated plasma leptin concentrations comparable to those in obese subjects. With no apparent adipose tissue, female transgenic skinny mice exhibit accelerated puberty and intact fertility at younger ages followed by successful delivery of healthy pups. However, at older ages, they develop hypothalamic hypogonadism characterized by prolonged menstrual cycles, atrophic ovary, reduced hypothalamic gonadotropin releasing hormone contents, and poor pituitary luteinizing hormone secretion. This study has demonstrated for the first time to our knowledge that accelerated puberty and late-onset hypothalamic hypogonadism are associated with chronic hyperleptinemia, thereby leading to a better understanding of the pathophysiological and therapeutic implication of leptin. (+info)MR imaging features in hypothalamic hamartoma: a report of three cases and review of literature. (6/138)
Hypothalamic hamartomas are rare tumours of particular interest because of their unusual symptoms. Three cases of hypothalamic hamartomas are reported in children, who presented with precocious puberty and gelastic seizures. (+info)A case of hypothalamic adrenal insufficiency manifested normal ACTH response to insulin-induced hypoglycemia. (7/138)
A low plasma ACTH response to insulin-induced hypoglycemia and an exaggerated and delayed plasma ACTH response to CRH stimulation have been considered as an indicator of hypothalamic hypopituitarism. We report a case of hypothalamic adrenal insufficiency which manifested normal ACTH response to insulin-induced hypoglycemia. This case provides important information to categorize hypothalamic adrenal insufficiency caused by abnormal regulation of CRH release. (+info)Inverse correlation between baseline inhibin B and FSH after stimulation with GnRH: a study of serum levels of inhibin A and B, pro alpha-C and activin A in women with ovulatory disturbances before and after stimulation with GnRH. (8/138)
OBJECTIVE: Interest has focused recently on the influences of the polypeptide factors inhibin and activin on the selective regulation of the pituitary secretion of gonadotropins. DESIGN: Measurement of the concentrations of inhibin-related proteins in relation to the changes in pituitary gonadotropin (FSH, LH) parameters, after GnRH stimulation with a bolus injection of 100 microg gonadorelin, in 19 women with ovulatory disturbances. METHODS: Serum levels of inhibin A and B, activin A, and pro alpha-C were measured using sensitive ELISA kits. RESULTS: Within 60 min after GnRH stimulation, FSH values doubled from 5 to 10 mU/ml (P < 0.001). LH increased 12-fold from 2 to 24 mU/ml (P < 0.001). Activin A showed a significant decrease from 0.47 to 0.36 ng/ml (P < 0.001), whereas pro alpha-C increased from 127 to 156 pg/ml (P = 0.039). The median inhibin A concentration did not show a significant change between baseline and the 60 min value, whereas inhibin B was characterized by a minor, but not significant, increase in the median from 168 to 179 pg/ml (P = 0.408). A significant inverse correlation (P = 0.014) with a mean coefficient of correlation of 0.5516 was found, demonstrating a strong relationship between high inhibin B baseline levels and a small increase of FSH after 60 min. CONCLUSION: Our results show an interesting correlation between the baseline inhibin B and the change in FSH before and after GnRH stimulation. A high baseline inhibin B implies only a minor increase of FSH after 60 min. (+info)Hypothalamic diseases refer to conditions that affect the hypothalamus, a small but crucial region of the brain responsible for regulating many vital functions in the body. The hypothalamus helps control:
1. Body temperature
2. Hunger and thirst
3. Sleep cycles
4. Emotions and behavior
5. Release of hormones from the pituitary gland
Hypothalamic diseases can be caused by genetic factors, infections, tumors, trauma, or other conditions that damage the hypothalamus. Some examples of hypothalamic diseases include:
1. Hypothalamic dysfunction syndrome: A condition characterized by various symptoms such as obesity, sleep disturbances, and hormonal imbalances due to hypothalamic damage.
2. Kallmann syndrome: A genetic disorder that affects the development of the hypothalamus and results in a lack of sexual maturation and a decreased sense of smell.
3. Prader-Willi syndrome: A genetic disorder that causes obesity, developmental delays, and hormonal imbalances due to hypothalamic dysfunction.
4. Craniopharyngiomas: Tumors that develop near the pituitary gland and hypothalamus, often causing visual impairment, hormonal imbalances, and growth problems.
5. Infiltrative diseases: Conditions such as sarcoidosis or histiocytosis can infiltrate the hypothalamus, leading to various symptoms related to hormonal imbalances and neurological dysfunction.
6. Traumatic brain injury: Damage to the hypothalamus due to head trauma can result in various hormonal and neurological issues.
7. Infections: Bacterial or viral infections that affect the hypothalamus, such as encephalitis or meningitis, can cause damage and lead to hypothalamic dysfunction.
Treatment for hypothalamic diseases depends on the underlying cause and may involve medications, surgery, hormone replacement therapy, or other interventions to manage symptoms and improve quality of life.
Hypopituitarism is a medical condition characterized by deficient secretion of one or more hormones produced by the pituitary gland, a small endocrine gland located at the base of the brain. The pituitary gland controls several other endocrine glands in the body, including the thyroid, adrenals, and sex glands (ovaries and testes).
Hypopituitarism can result from damage to the pituitary gland due to various causes such as tumors, surgery, radiation therapy, trauma, or inflammation. In some cases, hypopituitarism may also be caused by a dysfunction of the hypothalamus, a region in the brain that regulates the pituitary gland's function.
The symptoms and signs of hypopituitarism depend on which hormones are deficient and can include fatigue, weakness, decreased appetite, weight loss, low blood pressure, decreased sex drive, infertility, irregular menstrual periods, intolerance to cold, constipation, thinning hair, dry skin, and depression.
Treatment of hypopituitarism typically involves hormone replacement therapy to restore the deficient hormones' normal levels. The type and dosage of hormones used will depend on which hormones are deficient and may require regular monitoring and adjustments over time.
Encephalomalacia is a medical term that refers to the softening and degeneration of brain tissue. It is typically caused by an injury, infection, or lack of oxygen supply to the brain. This condition can lead to various neurological symptoms depending on the location and extent of the damage in the brain. Encephalomalacia may result in cognitive impairments, motor function loss, speech difficulties, and other long-term disabilities. Treatment options vary based on the underlying cause and severity of the condition but often include rehabilitation therapies to help manage symptoms and improve quality of life.
Pituitary neoplasms refer to abnormal growths or tumors in the pituitary gland, a small endocrine gland located at the base of the brain. These neoplasms can be benign (non-cancerous) or malignant (cancerous), with most being benign. They can vary in size and may cause various symptoms depending on their location, size, and hormonal activity.
Pituitary neoplasms can produce and secrete excess hormones, leading to a variety of endocrine disorders such as Cushing's disease (caused by excessive ACTH production), acromegaly (caused by excessive GH production), or prolactinoma (caused by excessive PRL production). They can also cause local compression symptoms due to their size, leading to headaches, vision problems, and cranial nerve palsies.
The exact causes of pituitary neoplasms are not fully understood, but genetic factors, radiation exposure, and certain inherited conditions may increase the risk of developing these tumors. Treatment options for pituitary neoplasms include surgical removal, radiation therapy, and medical management with drugs that can help control hormonal imbalances.
The pituitary gland is a small, endocrine gland located at the base of the brain, in the sella turcica of the sphenoid bone. It is often called the "master gland" because it controls other glands and makes the hormones that trigger many body functions. The pituitary gland measures about 0.5 cm in height and 1 cm in width, and it weighs approximately 0.5 grams.
The pituitary gland is divided into two main parts: the anterior lobe (adenohypophysis) and the posterior lobe (neurohypophysis). The anterior lobe is further divided into three zones: the pars distalis, pars intermedia, and pars tuberalis. Each part of the pituitary gland has distinct functions and produces different hormones.
The anterior pituitary gland produces and releases several important hormones, including:
* Growth hormone (GH), which regulates growth and development in children and helps maintain muscle mass and bone strength in adults.
* Thyroid-stimulating hormone (TSH), which controls the production of thyroid hormones by the thyroid gland.
* Adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to produce cortisol and other steroid hormones.
* Follicle-stimulating hormone (FSH) and luteinizing hormone (LH), which regulate reproductive function in both males and females.
* Prolactin, which stimulates milk production in pregnant and lactating women.
The posterior pituitary gland stores and releases two hormones that are produced by the hypothalamus:
* Antidiuretic hormone (ADH), which helps regulate water balance in the body by controlling urine production.
* Oxytocin, which stimulates uterine contractions during childbirth and milk release during breastfeeding.
Overall, the pituitary gland plays a critical role in maintaining homeostasis and regulating various bodily functions, including growth, development, metabolism, and reproductive function.
Pituitary diseases refer to a group of conditions that affect the pituitary gland, a small endocrine gland located at the base of the brain. The pituitary gland is responsible for producing and secreting several important hormones that regulate various bodily functions, including growth and development, metabolism, stress response, and reproduction.
Pituitary diseases can be classified into two main categories:
1. Pituitary tumors: These are abnormal growths in or around the pituitary gland that can affect its function. Pituitary tumors can be benign (non-cancerous) or malignant (cancerous), and they can vary in size. Some pituitary tumors produce excess hormones, leading to a variety of symptoms, while others may not produce any hormones but can still cause problems by compressing nearby structures in the brain.
2. Pituitary gland dysfunction: This refers to conditions that affect the normal function of the pituitary gland without the presence of a tumor. Examples include hypopituitarism, which is a condition characterized by decreased production of one or more pituitary hormones, and Sheehan's syndrome, which occurs when the pituitary gland is damaged due to severe blood loss during childbirth.
Symptoms of pituitary diseases can vary widely depending on the specific condition and the hormones that are affected. Treatment options may include surgery, radiation therapy, medication, or a combination of these approaches.
A subarachnoid hemorrhage is a type of stroke that results from bleeding into the space surrounding the brain, specifically within the subarachnoid space which contains cerebrospinal fluid (CSF). This space is located between the arachnoid membrane and the pia mater, two of the three layers that make up the meninges, the protective covering of the brain and spinal cord.
The bleeding typically originates from a ruptured aneurysm, a weakened area in the wall of a cerebral artery, or less commonly from arteriovenous malformations (AVMs) or head trauma. The sudden influx of blood into the CSF-filled space can cause increased intracranial pressure, irritation to the brain, and vasospasms, leading to further ischemia and potential additional neurological damage.
Symptoms of a subarachnoid hemorrhage may include sudden onset of severe headache (often described as "the worst headache of my life"), neck stiffness, altered mental status, nausea, vomiting, photophobia, and focal neurological deficits. Rapid diagnosis and treatment are crucial to prevent further complications and improve the chances of recovery.
A brain injury is defined as damage to the brain that occurs following an external force or trauma, such as a blow to the head, a fall, or a motor vehicle accident. Brain injuries can also result from internal conditions, such as lack of oxygen or a stroke. There are two main types of brain injuries: traumatic and acquired.
Traumatic brain injury (TBI) is caused by an external force that results in the brain moving within the skull or the skull being fractured. Mild TBIs may result in temporary symptoms such as headaches, confusion, and memory loss, while severe TBIs can cause long-term complications, including physical, cognitive, and emotional impairments.
Acquired brain injury (ABI) is any injury to the brain that occurs after birth and is not hereditary, congenital, or degenerative. ABIs are often caused by medical conditions such as strokes, tumors, anoxia (lack of oxygen), or infections.
Both TBIs and ABIs can range from mild to severe and may result in a variety of physical, cognitive, and emotional symptoms that can impact a person's ability to perform daily activities and function independently. Treatment for brain injuries typically involves a multidisciplinary approach, including medical management, rehabilitation, and supportive care.