scholarly journals Quality of compounded hydrocortisone capsules used in the treatment of children

2017 ◽  
Vol 177 (2) ◽  
pp. 239-242 ◽  
Author(s):  
Uta Neumann ◽  
Daniela Burau ◽  
Sarah Spielmann ◽  
Martin J Whitaker ◽  
Richard J Ross ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
High Performance ◽  
Adrenal Crisis ◽  
Adrenal Hyperplasia ◽  
Web Based ◽  
Vulnerable Patients ◽  
Dose Information ◽  
Patients At Risk

Objectives Due to the lack of paediatric-licensed formulations, children are often treated with individualized pharmacy-compounded adult medication. An international web-based survey about the types of medication in children with adrenal insufficiency (AI) revealed that the majority of paediatric physicians are using pharmacy-compounded medication to treat children with AI. Observations of loss of therapy control in children with congenital adrenal hyperplasia with compounded hydrocortisone capsules and regained control after prescribing a new hydrocortisone batch led to this ‘real world’ evaluation of pharmacy-compounded paediatric hydrocortisone capsules. Methods Capsule samples were collected randomly from volunteering parents of treated children suffering from congenital adrenal hyperplasia from all over Germany. Analysis of net mass and hydrocortisone content by high-performance liquid chromatography with ultraviolet (HPLC-UV) detection method was performed based on the European Pharmacopeia. Results In a total of 61 batches that were sent, 5 batches could not be analysed because of missing dose information, insufficient number of capsules or were not possible to be evaluated. Fifty-six batches containing 1125 capsules were evaluated. 21.4% of the batches revealed insufficiency in uniformity of net mass or drug content and additional 3.6% failed because they did not contain the labelled drug. Conclusions Compounded medication is a possible cause of variation of steroid doses in children with adrenal insufficiency or congenital adrenal hyperplasia, putting these vulnerable patients at risk of poor disease control and adrenal crisis. These data may apply to other individualized compounded oral medication as well, emphasizing the need for development of licensed paediatric formulations approved by regulatory authorities.

scholarly journals Adverse Effects of Ramadan Fasting in a Girl with Salt-Losing Congenital Adrenal Hyperplasia

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Valeria Calcaterra ◽  
Francesco Bassanese ◽  
Andrea Martina Clemente ◽  
Rossella Amariti ◽  
Corrado Regalbuto ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
Treatment Plan ◽  
Management Plan ◽  
Hormonal Control ◽  
Adrenal Crisis ◽  
Adrenal Hyperplasia ◽  
Ramadan Fasting ◽  
Salt Wasting ◽  
Classic Form

Objective. Congenital adrenal hyperplasia (CAH) is the most common cause of adrenal insufficiency in pediatrics. Chronic glucocorticoid replacement is the mainstay of treatment in the classic forms of CAH, and mineralocorticoid replacement therapy is mandatory in the salt-wasting form. Fasting is a mild stressor, which can expose to dehydration, hypotension, hypoglycemia, and acute adrenal crisis in patients with adrenal insufficiency. Case. We report the case of an adolescent affected by the classic form with salt-losing CAH, who observed Ramadan for 30 days, without individualized therapeutic management plan. After Ramadan, a dramatic increase of ACTH level (1081 pg/ml, n.v. 6–57), reduced cortisolemia, tendency to hypotension, and weight loss were recorded. She experienced insomnia, intense thirst, asthenia, and headache. The symptoms disappeared restarting the previous therapy schedule and increasing the total hydrocortisone daily dose with progressive restoring of hormonal control. Conclusion. Our case confirms that patients with CAH are vulnerable, especially during fasting in Ramadan, with a higher risk of acute adrenal crisis. CAH patients should reform and individualize their treatment plan and be submitted to careful monitoring.

scholarly journals Would Cortisol Measurements Be a Better Gauge of Hydrocortisone Replacement Therapy? Congenital Adrenal Hyperplasia as an Exemplar

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Peter C Hindmarsh ◽  
John W Honour
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Plasma Cortisol ◽  
Poor Quality ◽  
Adrenal Hyperplasia ◽  
Mortality And Morbidity ◽  
Glucocorticoid Replacement Therapy

There is an increase in mortality and morbidity as well as poor quality of life in patients with congenital adrenal hyperplasia (CAH) and other causes of adrenal insufficiency. Glucocorticoid replacement therapy should aim to replace the missing cortisol as close as possible to the normal circadian rhythm using hydrocortisone. Dosing should be based on the individual’s absorption and clearance of the drug. Adequacy of dosing should be checked using 24-hour profiles of plasma cortisol with samples drawn preferably every hour or at least every 2 hours. Measurement of cortisol should be the preferred method of assessing replacement therapy as it is over- and undertreatment with hydrocortisone, both of which can occur over a 24-hour period, which leads to the problems observed in patients with CAH and adrenal insufficiency.

Psychosexual Quality of Life in Adult Intersexuality: The Example of Congenital Adrenal Hyperplasia (CAH)

2000 ◽  
pp. 200-208 ◽  
Author(s):  
Heino F. L. Meyer-Bahlburg ◽  
Sonia Gidwani ◽  
Ralf W. Dittmann ◽  
Curtis Dolezal ◽  
Susan W. Baker ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia

Metabolic profile, cardiovascular risk factors and health-related quality of life in children, adolescents and young adults with congenital adrenal hyperplasia

2019 ◽  
Vol 32 (8) ◽  
pp. 871-877 ◽  
Author(s):  
Roopa Vijayan ◽  
Nisha Bhavani ◽  
Praveen V. Pavithran ◽  
Vasantha Nair ◽  
Usha V. Menon ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Risk Factors ◽  
Cardiovascular Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Cardiovascular Risk Factors ◽  
Metabolic Profile ◽  
Good Control ◽  
Adrenal Hyperplasia ◽  
Age And Sex

Abstract Background The present study was designed to evaluate the metabolic profile, cardiovascular risk factors and quality of life in children with congenital adrenal hyperplasia (CAH) and compare it with age- and sex-matched controls. Methods Fifty-two patients aged 3–21 years with classic CAH due to 21-hydroxylase deficiency were included in the study. Metabolic profiling was done for 36 cases and compared with 28 healthy age- and sex-matched controls. Quality of life was assessed in all 52 children and their parents using a validated Pediatric Quality of Life Inventory (PedsQL) questionnaire and was compared with normative data from the same population. Results The median age was 12 years with 14 (27%) males and 38 (73%) females. Out of the total 52 patients, 35 (67%) had salt wasting and 17 (33%) had simple virilising CAH. The median height standard deviation score (SDS) of cases was similar to that of controls (−0.72 vs. −0.64, p = 0.57) and 81% of females had normal pubertal status indicating a good control of the disease. Weight SDS, body mass index (BMI) SDS, mean diastolic blood pressure and insulin resistance were significantly higher in cases when compared to controls (0.31 vs. −0.3; 0.96 vs. 0.17; 67.8 ± 10.49 vs. 61 ± 8.49 and 2.1 vs. 0.95, respectively). The quality of life was significantly reduced in all domains as per parents’ perspective, whereas the children reported reduced quality of social and school functioning. There was no significant correlation between quality of life and metabolic parameters. Conclusions Children with CAH despite a reasonably good control of the disease have a higher cardiovascular risk and reduced quality of life when compared to healthy controls.

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