View clinical trials related to Adrenocortical Hyperfunction.
Filter by:Classic congenital adrenal hyperplasia (CAH) is a rare genetic endocrine disorder. Its prevalence is around 1/15.000. It results from a deficiency in 21-hydroxylase, an adrenal steroidogenic enzyme involved in the biosynthesis of cortisol and aldosterone. Enzyme deficiency in the steroideogenesis pathway leads to cortisol and aldosterone deficiency of varying severity, and to the accumulation of precursors (17OHP and Progesterone), which are diverted to the production of androgens (Testosterone and D4AD). There are two clinical forms of classical CAH : the salt wasting form and the simple virilizing form, depending on the degree of aldosterone deficiency. The clinical signs are adrenal insufficiency and hyperandrogenism. Hyperandrogenism manifests itself during foetal life, and may be responsible for virilization of the external genitalia of a female foetus, of varying severity. The challenge in managing this condition is to find the right therapeutic balance. Hydrocortisone and fludrocortisone supplementation must be adapted to control adrenal insufficiency, and limit hyperandrogenism. It must be sufficient to avoid episodes of acute adrenal insufficiency, but not excessive to avoid complications secondary to hypercorticism. During childhood, this balance is necessary for growth and pubertal development. However, this balance is difficult to achieve and maintain over time. In adulthood, the fertility of patients is an important issue. This one remains poorly understood. It was only after the introduction of cortisol supplementation in the treatment of CAH in the 1950s that the first pregnancies were described. Since when, due to the rarity of the pathology, the number of pregnancies studied has remained low, and the literature has little hindsight on the subject. This pathology has long been associated with female infertility, due to many factors : biological, mechanical, psychological and sexual, among others. Biological hyperandrogenism may be responsible for chronic dysovulation, and may render the endometrium unsuitable for embryonic implantation. The virilization of the external genitalia and possible complications of pelvic surgery can be an obstacle to sexuality in these patients. They experience sexual difficulties, particularly during penetrative intercourse. The literature also shows that the majority of these patients are single and that their sexual orientation is more likely to be homosexual. Finally, the desire to have children is less prevalent in these patients than in the general population. The first published studies on the fertility of CAH patients showed a lower pregnancy rate than the general population, but did not take into account the patient' desire to become pregnant. In 2009, Casteras et al demonstrated for the first time in a cohort of CAH patients that fecundity is preserved in patients with a desire to become pregnant. It should be noted that the fertility of patients with CAH may evolve in the coming years in France thanks to the new bioethics law voted in 2021, which now allows unmarried patients and patients in homosexual couples to have access to male gamete donation. In addition, very little is known about the course of pregnancies in patients with CAH. Few pregnancies have been studied to date. Hormonal balance during pregnancy if difficult to monitor, given the absence of reliable biological makers during this period. In this context, it is complicated to know the impact of the pathology and its balance on the course of the pregnancy. The latest articles published on the subject of pregnancy complications are contradictory. Some find an increased risk of gestational diabetes. Others find a higher risk of maternal-fetal infection, low weight for gestational age, or congenital malformations than in general population. The increased risk of miscarriage is debated. On the other hand, articles are unanimous on the most frequent mode of delivery in this population : in the vast majority of cases, patients give birth by ceasarian section, due to their history of pelvic surgery. It is in context that we wish through this study to make a point of fertility and pregnancy in patients with classical CAH.
Aims and objectives: Studies on stress are generally aimed at young children and infants. However, in the neonatal period, "especially in preterm babies", this issue was not given enough attention and was almost completely ignored. Background: They are exposed to different stressors. Too much stress will increase their problems in their future lives. Design: This study was planned as a randomized study to determine the effects of Kangaroo Care and mother scent application on toxic stress in preterm infants in the Neonatal Intensive Care Unit environment and to create evidence-based recommendations regarding these applications. Methods: Research data were collected from a total of 92 preterm babies born. Babies were divided into 3 groups, those who never met their mothers, only mother scent group and KC group. The mother's undershirt was used as the maternal odor. Vital signs, blood cortisol levels and Preterm Infant Comfort Scale scores were determined and recorded each group.The data were evaluated by statistical analysis. The CONSORT checklist for reporting qualitative research was used. Results: In the group that never encountered mother and mother odor, Preterm Infant Comfort Scale, blood cortisol level and vital signs showed severe stress. It was found that maternal odor is effective in reducing this stress, but kangaroo care is much more effective in preventing stress. Conclusion: if premature babies are deprived of their mother, the stress may be exposed to reaches toxic levels. It was determined that kangaroo care application during the treatment of these babies is a more effective method in reducing stress than the maternal odor application method. Relevance to clinical practice: The results of this study will contribute to nurses' use of kangaroo care and maternal odor in the care of preterm babies to prevent stress and related complications.Therefore, it will improve the quality of care of preterm babies in the NICU.
The Smart Supplement has 20 vitamins and minerals that have strong track records of demonstrating positive health effects, both physiologically and cognitively. This twelve-week trial will examine the effectiveness and efficacy of Heights Smart Supplement on both biomarkers and subjective experiences from participants.
This study is a randomized, double-blind, active-controlled, phase III study of Chronocort® compared with immediate-release hydrocortisone replacement therapy in participants aged 16 years and over with Congenital Adrenal Hyperplasia.
This is a Phase 2, open-label, multiple-dose, dose-escalation study to assess the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of NBI-74788 in approximately 12 pediatric female and male subjects (14 to 17 years of age) with a documented medical diagnosis of classic 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH).
Benign enlargements of the adrenal glands (adrenal adenomas) are frequent in adults. In the general population these adenomas are rare in subjects below 40 years of age but at the age of 60 and 80 years the prevalence is 6 and 8-10 % respectively. Since these adenomas do not causes obvious symptoms they are almost exclusively found incidentally in patients examined radiologically for other reasons than suspected adrenal disease. These enlargements are thus termed adrenal incidentalomas (AI). AI may secrete cortisol and more than 25 percent of patients with an AI have increased cortisol levels called autonomous cortisol secretion (ACS). Such increased secretion of cortisol may cause metabolic complications such as hypertension, high cholesterol, diabetes and cardiovascular disease. Studies have shown that ACS may cause increased mortality. These studies are however small and have not adequately taking other conditions into account which most likely influences the result. The investigators hypothesis is that ACS is linked to increased mortality as the previous studies have shown. The aim is to perform a larger study on patients with adrenal incidentalomas, both with and without ACS, and compare the mortality rates with a control group matched for age and sex. This study may more precisely describe the cardiovascular risk for ACS and define the risk at different levels of ACS.
According to scientific revisions, salivary cortisol is a valid method for measuring adrenocortical activity in newborns because it is non-invasive in fact the measurement of salivary cortisol has been used as a stress marker in newborns since 1992. The mechanism by which cortisol induces stress, concerns the hypothalamic-pituitary-adrenal axis. The hypothalamus produces the corticotropin-releasing hormone, which stimulates the production of adrenocorticotropin by hypophysis. Adrenocorticotropin induces the secretion of glucocorticoids such as cortisol by stimulating the cortex of the adrenal gland. An appropriate release of cortisol due to stress factor is vital for surviving and the loss of this mechanism increase the risk of morbidity and mortality for newborns. However, prolonged exposure to a high level of cortisol may increase the risk of cognitive and behavioural disorders, hypertension, hyperlipidaemia, insulin resistance, immune deficiency and hippocampal impairment. The separation from the mother, the exposure to painful procedures but also routine interventions such as physical examination, diaper changes and bathing increase the salivary cortisol level of the newborn compared to the basal state. The measurement of salivary cortisol in the newborn therefore has the aim of understanding and preventing those stressful conditions that may have long-term side effects. For this reason, the lose weight in the first days and double weighing should be a stress practice for the newborn.
This is an open-label, non-randomized crossover design feasibility trial comparing oral hydrocortisone treatment with interval bolus delivery (pulsatile) of subcutaneous hydrocortisone via infusion pump in children with congenital adrenal hyperplasia. Eight children, ages 4-18 yrs, will have 24-hr pharmacokinetic and pharmacodynamic profiles of cortisol, 17-hydroxyprogesterone and androstenedione concentrations while on oral hydrocortisone therapy (admission 1), during an initial trial of the subcutaneous hydrocortisone pump (admission 2), and after 6 weeks of subcutaneous hydrocortisone pump treatment (admission 3). An integrated pharmacokinetic and pharmacodynamic model will be used to determine cortisol, 17-hydroxyprogesterone and androstenedione parameters to compare the duration of time subjects have these concentrations outside acceptable ranges. Funding Source - FDA OOPD
This is a Phase 2 study of SPR001 for the treatment of classic CAH that will provide 12 weeks of open-label treatment to eligible subjects.
Hypercortisolism leads to long term physical and cognitive sequelae. This also holds true for quality of life, even several years after remission. This altered quality of life, highly subjective, is however, badly evaluated by the family of the patient. This could lead to misunderstanding, avec worsen the general physical and mental health of the patient. To our knowledge, this theoretical difference of perception has never been evaluated up to now. The aim of our study is to evaluate the difference of perception of the quality of life and body image between patients in remission of hypercortisolism, and their caregivers. Secondary objectives will be to evaluate the quality of life of the caregivers, the coping strategies, and the depression/anxiety parameters of both the patient and the caregiver. Patients and methods: the study is Observative, prospective and non-randomized. Inclusion criteria will be patients, aged more than 18, with hypercortisolism in remission for at least 1 year. Self-questionnaires on quality of life, body image, coping, depression and anxiety will be provided to the patient and his/her caregiver. Fulfilling will be blind between the patient and his/her caregiver. Inclusion period will be 12 months. Results will be compared between the patient and his/her caregiver. Expected results: investigators anticipate that some caregivers will have a different perception of the general physical and mental condition as stated by the patient. The first time that the quality of life of the caregiver would be also altered. This original project might lead to modify the management of Cushing's syndrome, by considering both the patient and his/her caregiver on a long term basis after remission.