Management of altered hydrocortisone pharmacokinetics in a boy with congenital adrenal hyperplasia using a continuous subcutaneous hydrocortisone infusion
Abstract
Background: Conventional hydrocortisone dosing schedules do not mimic the normal circadian rhythm of cortisol, making it difficult to optimize treatment in congenital adrenal hyperplasia (CAH). Case Details: We report a 14.5-year-old boy with CAH who had reduced bioavailability [42% (normal 80% orally and 100% by im route)] and increased clearance [half-life 50 min (normal range, 70–100 min)] of oral doses of hydrocortisone leading to ambient serum 17-hydroxyprogesterone concentrations of 400 nmol/liter (14.5 ng/ml) and androstenedione concentrations of 24.9 nmol/liter (7.1 ng/ml). Intervention: Using a continuous but variable sc hydrocortisone infusion via an insulin pump, rapid control of his CAH was attained with a normal cortisol circadian profile. Average daily hydrocortisone dose was 17.4–18.6 mg/m2, which produces on average 24-h serum cortisol and 17-hydroxyprogesterone concentrations of 316 nmol/liter (115 ng/ml) and 4.3 nmol/liter (1.4 ng/ml), respectively. Therapy has been maintained over 4 yr with suppression of normal adrenal androgen production and normal progression through puberty. Conclusions: Continuous sc infusion of hydrocortisone may prove a valuable adjunct to therapy for CAH, particularly in patients requiring high doses of oral hydrocortisone and in those with abnormal hydrocortisone pharmacokinetics. A boy is described with congenital adrenal hyperplasia and altered hydrocortisone pharmacokinetics managed by continuous subcutaneous hydrocortisone infusion.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Continuous subcutaneous hydrocortisone infusion has shown adequate ACTH suppression with lower total hydrocortisone dose compared to conventional treatment.
“Continuous subcutaneous hydrocortisone infusion via an insulin pump mimicking cortisol circadian rhythm, has similarly shown adequate ACTH suppression with lower total hydrocortisone dose compared to conventional treatment, 139-142”
From Congenital adrenal hyperplasia (2017) · cites this paper as [141]
Programmed infusion of hydrocortisone in a circadian fashion to poorly controlled CAH patients resulted in nearly normal ACTH and 17OHP.
“Programmed infusion of HC delivered in a circadian fashion to poorly controlled patients with CAH resulted in nearly normal ACTH and 17OHP (308, 309).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [308]
Cites
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency*
White, P. C., & Speiser, P. W. (2000) · Endocrine Reviews · doi:10.1210/edrv.21.3.0398 · Cited by 1,432 · see citing works
Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
White, P. C. (2000) · Endocrine Reviews · doi:10.1210/er.21.3.245 · Cited by 877 · see citing works
Modified-release hydrocortisone to provide circadian cortisol profiles
Debono, M., Ghobadi, C., Rostami-Hodjegan, A., Huatan, H., Campbell, M. J., Newell-Price, J., Darzy, K., Merke, D. P., Arlt, W., & Ross, R. J. (2009) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2008-2380 · Cited by 322 · see citing works
Mah, P. M., Jenkins, R. C., Rostami‐Hodjegan, A., Newell‐Price, J., Doane, A., Ibbotson, V., Tucker, G. T., & Ross, R. J. (2004) · Clinical Endocrinology · doi:10.1111/j.1365-2265.2004.02106.x · Cited by 243 · see citing works
Charmandari, E., Weise, M., Bornstein, S. R., Eisenhofer, G., Keil, M. F., Chrousos, G. P., & Merke, D. P. (2002) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.87.5.8456 · Cited by 164 · see citing works
Continuous subcutaneous hydrocortisone infusion in Addison’s disease
Løvås, K., & Husebye, E. S. (2007) · European Journal of Endocrinology · doi:10.1530/eje-07-0052 · Cited by 155 · see citing works
Charmandari, E., Hindmarsh, P. C., Johnston, A., & Brook, C. G. D. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.6.7522 · Cited by 134 · see citing works
Self-citationRivkees, S. A., & Crawford, J. D. (2000) · PEDIATRICS · doi:10.1542/peds.106.4.767 · Cited by 130 · see citing works
Roche, E. F., Charmandari, E., Dattani, M. T., & Hindmarsh, P. C. (2003) · Clinical Endocrinology · doi:10.1046/j.1365-2265.2003.01757.x · Cited by 113 · see citing works
Self-citationCharmandari, E., Johnston, A. E., Brook, C. G. D., & Hindmarsh, P. C. (2001) · Journal of Endocrinology · doi:10.1677/joe.0.1690065 · Cited by 104 · see citing works
Self-citationMerza, Z., Rostami‐Hodjegan, A., Memmott, A., Doane, A., Ibbotson, V., Newell‐Price, J., Tucker, G. T., & Ross, R. J. (2006) · Clinical Endocrinology · doi:10.1111/j.1365-2265.2006.02544.x · Cited by 98 · see citing works
Wudy, S. A., Hartmann, M. F., & Svoboda, M. (2000) · Hormone Research in Paediatrics · doi:10.1159/000023516 · Cited by 56 · see citing works
Congenital adrenal hyperplasia: management during critical illness
Charmandari, E., Lichtarowicz-Krynska, E., Hindmarsh, P. C., Johnston, A. E., Aynsley-Green, A., & Brook, C. G. D. (2001) · Archives of Disease in Childhood · doi:10.1136/adc.85.1.26 · Cited by 31 · see citing works
Self-citation
Flagged first, then by citations
Cited by
Sorted by citations
Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Montori, V. M., Oberfield, S. E., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-2631 · Cited by 1,484 · see citing works
Speiser, P. W., Arlt, W., Auchus, R. J., Baskin, L. S., Conway, G. S., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Murad, M. H., Oberfield, S. E., et al. (2018) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2018-01865 · Cited by 1,234 · see citing works
Congenital adrenal hyperplasia
El-Maouche, D., Arlt, W., & Merke, D. P. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31431-9 · Cited by 669 · see citing works
Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management
Claahsen - van der Grinten, H. L., Speiser, P. W., Ahmed, S. F., Arlt, W., Auchus, R. J., Falhammar, H., Flück, C. E., Guasti, L., Huebner, A., Kortmann, B. B. M., et al. (2022) · Endocrine Reviews · doi:10.1210/endrev/bnab016 · Cited by 556 · see citing works
Mallappa, A., Sinaii, N., Kumar, P., Whitaker, M. J., Daley, L.-A., Digweed, D., Eckland, D. J. A., Van Ryzin, C., Nieman, L. K., Arlt, W., et al. (2015) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2014-3809 · Cited by 167 · see citing works
Whitaker, M. J., Debono, M., Huatan, H., Merke, D. P., Arlt, W., & Ross, R. J. (2014) · Clinical Endocrinology · doi:10.1111/cen.12316 · Cited by 121 · see citing works
Nermoen, I., Rørvik, J., Holmedal, S. H., Hykkerud, D. L., Fougner, K. J., Svartberg, J., Husebye, E. S., & Løvås, K. (2011) · Clinical Endocrinology · doi:10.1111/j.1365-2265.2011.04151.x · Cited by 103 · see citing works
Treatment and health outcomes in adults with congenital adrenal hyperplasia
Han, T. S., Walker, B. R., Arlt, W., & Ross, R. J. (2014) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2013.239 · Cited by 102 · see citing works
Nella, A. A., Mallappa, A., Perritt, A. F., Gounden, V., Kumar, P., Sinaii, N., Daley, L.-A., Ling, A., Liu, C.-Y., Soldin, S. J., et al. (2016) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2016-1916 · Cited by 91 · see citing works
Whittle, E., & Falhammar, H. (2019) · Journal of the Endocrine Society · doi:10.1210/js.2019-00136 · Cited by 87 · see citing works
Monogenic disorders of adrenal steroidogenesis
Baranowski, E. S., Arlt, W., & Idkowiak, J. (2018) · Hormone Research in Paediatrics · doi:10.1159/000488034 · Cited by 79 · see citing works
Subbarayan, A., Dattani, M. T., Peters, C. J., & Hindmarsh, P. C. (2014) · Clinical Endocrinology · doi:10.1111/cen.12265 · Cited by 73 · see citing works
Self-citationManagement considerations for the adult with congenital adrenal hyperplasia
Auchus, R. J. (2015) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2015.01.039 · Cited by 65 · see citing works
Turcu, A. F., Spencer-Segal, J. L., Farber, R. H., Luo, R., Grigoriadis, D. E., Ramm, C. A., Madrigal, D., Muth, T., O'Brien, C. F., & Auchus, R. J. (2016) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2015-3574 · Cited by 61 · see citing works
Congenital adrenal hyperplasia – pharmacologic interventions from the prenatal phase to adulthood
Claahsen-van der Grinten, H. L., Stikkelbroeck, N. M. M. L., Otten, B. J., & Hermus, A. R. M. M. (2011) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2011.05.004 · Cited by 53 · see citing works
Mallappa, A., Nella, A. A., Sinaii, N., Rao, H., Gounden, V., Perritt, A. F., Kumar, P., Ling, A., Liu, C., Soldin, S. J., et al. (2018) · Clinical Endocrinology · doi:10.1111/cen.13813 · Cited by 45 · see citing works
Markmann, S., De, B. P., Reid, J., Jose, C. L., Rosenberg, J. B., Leopold, P. L., Kaminsky, S. M., Sondhi, D., Pagovich, O., & Crystal, R. G. (2018) · Human Gene Therapy · doi:10.1089/hum.2017.203 · Cited by 42 · see citing works
Peters, C. J., Hill, N., Dattani, M. T., Charmandari, E., Matthews, D. R., & Hindmarsh, P. C. (2013) · Clinical Endocrinology · doi:10.1111/j.1365-2265.2012.04502.x · Cited by 34 · see citing works
Self-citationThe Urinary Steroidome of Treated Children With Classic 21-Hydroxylase Deficiency
Kamrath, C., Wettstaedt, L., Boettcher, C., Hartmann, M. F., & Wudy, S. A. (2017) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2016.08.006 · Cited by 30 · see citing works
Mallappa, A., Nella, A. A., Kumar, P., Brooks, K. M., Perritt, A. F., Ling, A., Liu, C.-Y., & Merke, D. P. (2017) · Journal of the Endocrine Society · doi:10.1210/js.2017-00215 · Cited by 16 · see citing works
- Authors
- Bryan, S. M., Honour, J. W., & Hindmarsh, P. C.
- Affiliations
- University College London (GB)
- Corresponding author
- Hindmarsh, Peter C.
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2009-09-01
- Volume
- 94
- Issue
- 9
- Pages
- 3477-3480
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2032490034
- Cited by
- 75
- References
- 13
- Citations by year
- 2026: 6 · 2024: 5 · 2023: 3 · 2022: 5 · 2021: 1 · 2019: 4 · 2018: 6 · 2017: 3 · 2016: 7 · 2015: 4 · 2014: 7 · 2013: 6 · 2012: 2
- FWCI
- 4.35
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal and reproductive studies
- Keywords
- congenital adrenal hyperplasia, cortisol, continuous subcutaneous infusion, 17-hydroxyprogesterone, androstenedione, diurnal cortisol rhythm, insulin pump, puberty, pediatric endocrinology
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Humans, Hydrocortisone, Male, 17-alpha-Hydroxyprogesterone, Infusions, Subcutaneous
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
