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Ridder LO, Balle CM, Skakkebæk A, et al. Endocrine, cardiac and neuropsychological aspects of adult congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2024 Jun. 100 (6):515-26. [QxMD MEDLINE Link]. [Full Text].
Joint LWPES/ESPE CAH Working Group. Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. J Clin Endocrinol Metab. 2002 Sep. 87(9):4048-53. [QxMD MEDLINE Link].
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Speiser PW, Arlt W, Auchus RJ, et al. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018 Nov 1. 103 (11):4043-88. [QxMD MEDLINE Link]. [Full Text].
Prednisone, prednisolone, or even dexamethasone suspensions may be used. Prednisone is available in a suspension of 1 mg/mL, and prednisolone is available in a solution of 5 or 15 mg/5 mL. The estimated equivalencies are as follows: [38]
Dangle PP, Lee A, Chaudhry R, Schneck FX. Surgical Complications Following Early Genitourinary Reconstructive Surgery for Congenital Adrenal Hyperplasia-Interim Analysis at 6 Years. Urology. 2016 Nov 23. [QxMD MEDLINE Link].
Replacement of mineralocorticoids is required in patients who have salt-wasting congenital adrenal hyperplasia. This treatment is necessary to replace the aldosterone that is insufficiently produced by the adrenal cortex.
Gunther DF, Bukowski TP, Ritzen EM, et al. Prophylactic adrenalectomy of a three-year-old girl with congenital adrenal hyperplasia: pre- and postoperative studies. J Clin Endocrinol Metab. 1997 Oct. 82(10):3324-7. [QxMD MEDLINE Link].
Plasma ACTH concentrations are of little help in adjusting doses of glucocorticoid in patients with primary adrenal insufficiency. Monitoring symptoms of salt craving and blood pressure, PRA, and electrolyte levels are helpful in adjusting the dose of fludrocortisone. High blood pressure with suppressed PRA should prompt a reduction in fludrocortisone dose.
In patients with hypotension, 0.9% (isotonic) sodium chloride solution (450 mL/m2 or 20 mL/kg IV) must be rapidly administered over the first hour. This is followed by a continuous IV infusion of 3200 mL/m2/d or 200 mL/kg per 100 cal/d of estimated resting energy expenditure as isotonic or half-isotonic sodium chloride solution to restore intravascular volume. Dextrose must also be provided.
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Aviso Importante: Aunque suministramos productos de Weishaupt, Merales SL no es un distribuidor autorizado. Todos los derechos están reservados por los fabricantes y sus socios oficiales.
Synthetic steroid with predominantly mineralocorticoid activity. Acts on renal tubule to promote sodium retention in exchange for potassium or hydrogen ion and thus maintain intravascular and extracellular volume. For patients who require parenteral mineralocorticoid therapy, high-dose hydrocortisone must be used. Available only as tab; may be crushed for infants and children.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug ReferenceDisclosure: Nothing to disclose.
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The goal of therapy for adrenal hyperplasia is the replacement of glucocorticoid and mineralocorticoid to prevent signs of adrenal insufficiency and to prevent the accumulation of precursor hormones that cause virilization. Adequate glucocorticoid replacement should prevent excessive concentrations of ACTH from stimulating the adrenal glands to produce abnormal concentrations of adrenal androgens that result in further virilization. In the growing child with adrenal insufficiency, long-term glucocorticoid replacement must be balanced to prevent symptoms of adrenal insufficiency while still allowing the child to grow at a normal rate and prevent symptoms of glucocorticoid excess. The dosage must be tailored to each patient. The recommended hydrocortisone starting dose is 8-10 mg/m2/day divided in 2-3 doses, but the general average maintenance dosage is 10-25 mg/m2/day of hydrocortisone PO divided in 2-3 doses.
Vos AA, Bruinse HW. Congenital adrenal hyperplasia: do the benefits of prenatal treatment defeat the risks?. Obstet Gynecol Surv. 2010 Mar. 65(3):196-205. [QxMD MEDLINE Link].
Descubra la calidad superior y el rendimiento del Weishaupt Fan 24 00 m3, 75 mb con Merales SL en España . Ya sea que busque confiabilidad o eficiencia, este producto satisface todas sus necesidades industriales. El Fan 24 00 m3, 75 mb está diseñado para proporcionar un rendimiento óptimo y durabilidad en diversas aplicaciones. Conocido por su construcción de alta calidad y características innovadoras, es la elección preferida por los profesionales. Características principales de Fan 24 00 m3, 75 mb : Alto rendimiento: Diseñado para ofrecer resultados excepcionales. Construcción duradera: Fabricado con materiales de alta calidad para un uso prolongado. Diseño innovador: Incorpora la última tecnología para una mayor eficiencia. Costo efectivo: Ofrece un gran valor por su dinero con precios competitivos. En Merales SL , ofrecemos el Fan 24 00 m3, 75 mb a precios competitivos y con los tiempos de entrega más rápidos en España . Nuestro equipo de expertos está listo para ayudarlo con todas sus consultas y proporcionar soluciones personalizadas que satisfagan sus necesidades específicas. ¿Por qué elegir Merales SL ? Asistencia experta: Obtenga ayuda de nuestro equipo conocedor para todas sus preguntas relacionadas con el producto. Precios competitivos: Ofrecemos algunos de los mejores precios del mercado. Entrega rápida: Aproveche nuestra red de entrega eficiente para tiempos de respuesta rápidos. Satisfacción del cliente: Nuestro compromiso con el servicio al cliente garantiza una excelente experiencia de compra. ¡Obtenga su cotización hoy! ¿Listo para mejorar sus operaciones con el Fan 24 00 m3, 75 mb ? Contáctenos ahora para una cotización personalizada. Complete el formulario a continuación o envíenos un correo electrónico con su consulta. Permítanos ayudarle a tomar una decisión rentable y de calidad para su negocio.
[Guideline] Torre JJ, Bloomgarden ZT, Dickey RA, et al. American Association of Clinical Endocrinologists Medical Guidelines for Clinical Practice for the diagnosis and treatment of hypertension. Endocr Pract. 2006 Mar-Apr. 12(2):193-222. [QxMD MEDLINE Link].
Auchus RJ. The backdoor pathway to dihydrotestosterone. Trends Endocrinol Metab. 2004 Nov. 15(9):432-8. [QxMD MEDLINE Link].
Sasigarn A Bowden, MD, FAAP Professor of Pediatrics, Section of Pediatric Endocrinology, Metabolism and Diabetes, Department of Pediatrics, Ohio State University College of Medicine; Pediatric Endocrinologist, Division of Endocrinology, Nationwide Children’s Hospital; Affiliate Faculty/Principal Investigator, Center for Clinical Translational Research, Research Institute at Nationwide Children’s Hospital Sasigarn A Bowden, MD, FAAP is a member of the following medical societies: American Society for Bone and Mineral Research, Central Ohio Pediatric Society, Endocrine Society, International Society for Pediatric and Adolescent Diabetes, Pediatric Endocrine Society, Society for Pediatric ResearchDisclosure: Nothing to disclose.
Longui CA, Kochi C, Calliari LE, Modkovski MB, Soares M, Alves EF, et al. Near-final height in patients with congenital adrenal hyperplasia treated with combined therapy using GH and GnRHa. Arq Bras Endocrinol Metabol. 2011 Nov. 55(8):661-4. [QxMD MEDLINE Link].
Fluck CE, Tajima T, Pandey AV, et al. Mutant P450 oxidoreductase causes disordered steroidogenesis with and without Antley-Bixler syndrome. Nat Genet. 2004 Mar. 36(3):228-30. [QxMD MEDLINE Link].
In patients who are sick and who have signs of adrenal insufficiency, therapy should consist of stress dosages of hydrocortisone (50-100 mg/m2 or 1-2 mg/kg IV administered as an initial dose), followed by 50-100 mg/m2/d IV divided every 6 hours. Comparable stress dosages include 10-20 mg/m2 of methylprednisolone administered IV or intramuscularly (IM) and 1-2 mg/m2 of dexamethasone. Methylprednisolone and dexamethasone have negligible mineralocorticoid effects. Therefore, if the patient is hypovolemic, hyponatremic, or hyperkalemic, large dosages of hydrocortisone (double or triple the stress dosages mentioned above) are preferred because of its mineralocorticoid effect.
McKusick VA. Online Mendelian Inheritance in Man. National Center for Biotechnology Information. Available at https://www.ncbi.nlm.nih.gov/sites/entrez?db=omim.
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Hirvikoski T, Nordenstrom A, Wedell A, Ritzen M, Lajic S. Prenatal dexamethasone treatment of children at risk for congenital adrenal hyperplasia: the Swedish experience and standpoint. J Clin Endocrinol Metab. 2012 Jun. 97 (6):1881-3. [QxMD MEDLINE Link].
Arlan L Rosenbloom, MD Adjunct Distinguished Service Professor Emeritus of Pediatrics, University of Florida College of Medicine; Fellow of the American Academy of Pediatrics; Fellow of the American College of Epidemiology Arlan L Rosenbloom, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Epidemiology, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, Florida Chapter of The American Academy of Pediatrics, Florida Pediatric Society, International Society for Pediatric and Adolescent DiabetesDisclosure: Nothing to disclose.
White PC. Neonatal screening for congenital adrenal hyperplasia. Nat Rev Endocrinol. 2009 Sep. 5(9):490-8. [QxMD MEDLINE Link].
Lin-Su K, Harbison MD, Lekarev O, Vogiatzi MG, New MI. Final adult height in children with congenital adrenal hyperplasia treated with growth hormone. J Clin Endocrinol Metab. 2011 Jun. 96(6):1710-7. [QxMD MEDLINE Link]. [Full Text].
En Merales SL , ofrecemos el Fan 24 00 m3, 75 mb a precios competitivos y con los tiempos de entrega más rápidos en España . Nuestro equipo de expertos está listo para ayudarlo con todas sus consultas y proporcionar soluciones personalizadas que satisfagan sus necesidades específicas.
Janzen N, Riepe FG, Peter M, Sander S, Steuerwald U, Korsch E, et al. Neonatal screening: identification of children with 11ß-hydroxylase deficiency by second-tier testing. Horm Res Paediatr. 2012. 77(3):195-9. [QxMD MEDLINE Link].
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Turcu AF, Auchus RJ. The next 150 years of congenital adrenal hyperplasia. J Steroid Biochem Mol Biol. 2015 Sep. 153:63-71. [QxMD MEDLINE Link]. [Full Text].
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Auchus RJ, Miller WL. Congenital adrenal hyperplasia--more dogma bites the dust. J Clin Endocrinol Metab. 2012 Mar. 97(3):772-5. [QxMD MEDLINE Link].
Meyer-Bahlburg HF, Dolezal C, Haggerty R, Silverman M, New M. Cognitive Outcome of Offspring from Dexamethasone-Treated Pregnancies at Risk for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency. Eur J Endocrinol. 2012 May 1. [QxMD MEDLINE Link].
Aycan Z, Akbuga S, Cetinkaya E, et al. Final height of patients with classical congenital adrenal hyperplasia. Turk J Pediatr. 2009 Nov-Dec. 51(6):539-44. [QxMD MEDLINE Link].
Roth JD, Casey JT, Whittam BM, et al. Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large Scale Administrative Database. Urology. 2018 Mar 2. [QxMD MEDLINE Link].
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Turcu AF, Auchus RJ. Adrenal steroidogenesis and congenital adrenal hyperplasia. Endocrinol Metab Clin North Am. 2015 Jun. 44 (2):275-96. [QxMD MEDLINE Link]. [Full Text].
Merke DP. Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2008 Mar. 93(3):653-60. [QxMD MEDLINE Link].
No parenteral form of mineralocorticoid is currently available in the United States; however, if the patient has good GI function, administer 0.05-0.2 mg of fludrocortisone by mouth (PO).
Purandare A, Godil MA, Prakash D, et al. Spontaneous adrenal hemorrhage associated with transient antiphospholipid antibody in a child. Clin Pediatr (Phila). 2001 Jun. 40(6):347-50. [QxMD MEDLINE Link].
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Mimics actions of cortisol, which is the primary steroid hormone secreted by adrenal zona fasciculata and reticularis. DOC in children owing to short half-life and decreased potential for growth suppression. Mineralocorticoid effect occurs with large doses.
These forms of glucocorticoid have the advantage of half-lives longer than those of hydrocortisone, permitting twice-daily or even once-daily dosing (dexamethasone), which often aids compliance. However, because of their increased potency, growth suppression and other signs of glucocorticoid excess are common.
All patients with adrenal insufficiency must have injectable glucocorticoid available, and the caretaker must be instructed in its use and importance. Glucocorticoid or mineralocorticoid replacement has no contraindications when it is needed, and it has few drug-drug interactions.
Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta KappaDisclosure: Nothing to disclose.
Hydrocortisone is available in 5-mg, 10-mg, and 20-mg tablets. Also, an immediate-release oral granule product (Alkindi Sprinkle) is available in 0.5-mg, 1-mg, 2-mg, and 5-mg dosage sizes to assist with dosing in young children. Hydrocortisone is recommended in the pediatric population because of its lower potency, which permits easier titration of appropriate doses. Unfortunately, hydrocortisone suspension (Cortef solution) is no longer available in the United States.
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Kamrath C, Hochberg Z, Hartmann MF, Remer T, Wudy SA. Increased activation of the alternative "backdoor" pathway in patients with 21-hydroxylase deficiency: evidence from urinary steroid hormone analysis. J Clin Endocrinol Metab. 2012 Mar. 97(3):E367-75. [QxMD MEDLINE Link].
Halper A, Sanchez B, Hodges JS, et al. Bone mineral density and body composition in children with congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2018 Feb 20. [QxMD MEDLINE Link].
Carvalho LC, Brito VN, Martin RM, et al. Clinical, hormonal, ovarian, and genetic aspects of 46,XX patients with congenital adrenal hyperplasia due to CYP17A1 defects. Fertil Steril. 2016 Jun. 105 (6):1612-9. [QxMD MEDLINE Link].
Witchel SF. Nonclassic congenital adrenal hyperplasia. Curr Opin Endocrinol Diabetes Obes. 2012 Jun. 19(3):151-8. [QxMD MEDLINE Link].
Barry B Bercu, MD Professor, Departments of Pediatrics, Molecular Pharmacology and Physiology, University of South Florida College of Medicine, All Children's Hospital Barry B Bercu, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinology, American Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Society for Pediatric Research, Southern Society for Pediatric Research, Society for the Study of Reproduction, American Federation for Clinical Research, Pituitary SocietyDisclosure: Nothing to disclose.
Yang M, White PC. Risk factors for hospitalization of children with congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2017 Feb 13. [QxMD MEDLINE Link].
One of the important physiologic responses to stress is an increase in the cortisol production that ACTH mediates. Patients with adrenal insufficiency of any etiology cannot mount this response and must be given stress doses of glucocorticoid. In the patient with a minor illness (temperature of < 38°C), the dosage of hydrocortisone should be at least doubled. For patients with relatively severe illness (temperature of >38°C), the dosage of glucocorticoid should be tripled. If the patient is vomiting or listless, administer parenteral glucocorticoid (50-75 mg/m2 of hydrocortisone IM or IV or an equivalent dosage of methylprednisolone or dexamethasone). Because hydrocortisone succinate has a short duration of action, the dose must be repeated every 6-8 hours at a dosage of 50-100 mg/m2/d until the patient is well.
Herting MM, Azad A, Kim R, Tyszka JM, Geffner ME, Kim MS. Brain Differences in the Prefrontal Cortex, Amygdala, and Hippocampus in Youth with Congenital Adrenal Hyperplasia. J Clin Endocrinol Metab. 2020 Apr 1. 105 (4):[QxMD MEDLINE Link].
Maccabee-Ryaboy N, Thomas W, Kyllo J, et al. Hypertension in children with congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2016 Apr 22. [QxMD MEDLINE Link].
Lim SG, Lee YA, Jang HN, et al. Long-Term Health Outcomes of Korean Adults With Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency. Front Endocrinol (Lausanne). 2021. 12:761258. [QxMD MEDLINE Link]. [Full Text].
If the patient is hypoglycemic, 2-4 mL of dextrose 10% in water (D10W) should be administered to increase the blood sugar, followed by a continuous infusion of dextrose 5% in water (D5W). If the patient is not hypoglycemic, D5W should be administered to prevent hypoglycemia. Patients with salt-wasting forms of adrenal hyperplasia do not need potassium supplementation because they are usually hyperkalemic. However, patients with 11-hydroxylase and 17-alpha-hydroxylase deficiency may be hypokalemic and may require potassium. After appropriate diagnostic studies are performed or after the results are known, glucocorticoid therapy, mineralocorticoid therapy, or both may be started.
Administer fludrocortisone (0.05-0.2 mg/d PO) to patients with mineralocorticoid deficiency. Administer NaCl (2-5 g/d) to infants to counteract salt wasting. Older children can usually scavenge adequate salt to provide for their needs and may lose their salt-wasting tendencies as they mature. The dose of glucocorticoid is adjusted by clinically evaluating the patient (for an absence of symptoms of glucocorticoid deficiency and normal growth) and by periodically measuring the concentrations of precursor hormones. For example, in 21-hydroxylase deficiency, keeping plasma concentrations of 17-hydroxyprogesterone in the 200- to 500-ng/dL range and keeping androstenedione in the normal physiologic range is desirable.
Poppas DP. Clitoroplasty in congenital adrenal hyperplasia: description of technique. Adv Exp Med Biol. 2011. 707:49-50. [QxMD MEDLINE Link].
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The purpose of glucocorticoid therapy in congenital adrenal hyperplasia is (1) to replace the body's requirement for glucocorticoids under normal conditions and during stress and (2) to suppress ACTH secretion, thereby reducing the stimulus for the adrenal glands to overproduce adrenal androgens in virilizing forms of congenital adrenal hyperplasia. Unfortunately, no currently available preparation is able to mimic the diurnal rhythm of physiologic cortisol secretion. Thus, in an attempt to suppress androgen secretion from the adrenal glands in response to early morning rises in ACTH, overtreatment often occurs, resulting in inhibition of linear growth and Cushingoid features.
Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2010 Sep. 95(9):4133-60. [QxMD MEDLINE Link].
El Fan 24 00 m3, 75 mb está diseñado para proporcionar un rendimiento óptimo y durabilidad en diversas aplicaciones. Conocido por su construcción de alta calidad y características innovadoras, es la elección preferida por los profesionales.
Carlson AD, Obeid JS, Kanellopoulou N, et al. Congenital adrenal hyperplasia: update on prenatal diagnosis and treatment. J Steroid Biochem Mol Biol. 1999 Apr-Jun. 69(1-6):19-29. [QxMD MEDLINE Link].
Riehl G, Reisch N, Roehle R, Claahsen van der Grinten H, Falhammar H, Quinkler M. Bone mineral density and fractures in congenital adrenal hyperplasia: Findings from the dsd-LIFE study. Clin Endocrinol (Oxf). 2020 Apr. 92 (4):284-94. [QxMD MEDLINE Link]. [Full Text].